James' Story

In Loving Memory

2007-12-15T06:57:00.000-05:00

James Robert

Born

Wednesday, December 15, 2004

London Health Sciences Centre

London, Ontario

Passed Away

Friday, November 23, 2007

Hospital for Sick Children

Toronto, Ontario

Age

35 months

On Wednesday, December 15, 2004 at 9:58am the Lord blessed our family with what was deemed to be healthy baby boy. This was a great relief after losing James older brother Justin, due to pregnancy complication and prematurity (born at 27 weeks) and after what had felt like an extra long pregnancy due to being on partial bed-rest, developing gestational diabetes, having slightly raised blood pressure levels, and some concern over the placenta's defficiency. But all appeared well at that time for which we were very thankful.

PROVIDENTIAL MOVE

Two weeks after James was born we moved from our big old farmhouse out in the country to smack-dab in the middle of the city. At that time the reason for moving was so Rob could begin his studies at McMaster University, but as time went on we could see the Lord's timing in Rob's decision to return to school and our decision to move the family. If we thought the 20 months of James' illness were rough where we are now, we do not want to begin to imagine what it would have been like living out in the country, an hours drive from the nearest children's hopsital, where every appointment becomes a day-long event, and with often 1-3 appointments a week ... well you can figure out the rest.

ONSET OF SYMPTOMS

Many times we are asked how we knew something was wrong with James and at what age did we start to see signs of trouble. For the first 13 months of his life, James appeared to be mostly a normal, but colicky, child. There were signs of trouble already, unfortunately we didn't read them the way we could have had we known. James was a fussy baby, but after an extremely colicky sister before him, we did not think too much of this. Comfort also went a long ways with James and if he was held he was often happier, and so we wrote him off as another colicky baby. Often in the evenings James was awake, fussy and crying until 10-11pm, even holding him did not always make him happy, and this went on until he was about six months, when he finally started going to bed a bit earlier (8-9pm), but remained a fussy child. We labeled James as a high maintance child and carried on. Rebecca had been extremely colicky and at a year things finally settled for her, so we hoped the same for James.

BREAST-FEEDING ISSUES

Nursing was also a battle with James, who wanted to eat every 1.5-2 hours and I'm still surprised that I lasted until seven months before I finally gave up. Weaning James was a big challenge. Both the girls had been breastfed only babies, who refused to take bottles, the same as James. So I was prepared for a battle of starving James to get him to take a bottle, but not totally prepared as I forgot to factor in that the girls were both eating solids just fine by time I weaned them (at around 8.5 months) and so even though they were stubborn they weren't totally starving. James was not eating solids and yet he still refused to touch the bottle for over 17 hours. He did not look well by time he did take it and I was beginning to worry, but refused to give up - he had to be bottlefed and starving him was the only way to get him to do it.

FEEDING ISSUES

From 2-4 months James slept through the night (going to bed late meant this was really only around an 8 hour stretch) and then it stopped until he did it again from 26-28 months old (when we put him on reflux medication). Thinking pablum might help at four months I tried to introduce solids in the hope that he would sleep longer again. Here began our first and most obvious sign that something was wrong. He absolutely refused to take it ... and so the battle went for the next months as I tried to get him to eat solids, but he refused. He would clamp his mouth shut so tight you couldn't get anything in it. Or on other occasions, he would allow us to put it in his mouth, but then dribbled it all back out again. Off and on we tried solids, but the battle never ended, occasionally we would have a good few days, where he would gobble up a new food we had tried, and then he quit eating again for a few weeks before we discovered a new food that he would eat. We nicknamed him Bear, for he seemed to eat and then hibernate. The only thing we could pretty consistently get into him was Baby Mum-Mum rice crackers (low in protein), for the rest I was happy to get 1 tsp in him during a meal (and 3/4 of that would usually come back out again). And then there was the fact that he seemed to like pudding, ice cream and all those sweet yummy deserts, making us believe he was just an extremely fussy and extremely stubborn child, and so we didn't bother to pursue what would make a child refuse to eat. It didn't help us that he had a belly the size of a whale and anytime I ever complained people would always say "well you must be doing something right, he looks fat and chubby to me". Later we would find out the reason for this large belly (enlarged liver and spleen).

The introduction of formula brought some problems as well, hindsight we realize why. At the time we thought that he did not know how to suck and swallow properly from a bottle, and so we tried many different nipples. It took a couple weeks before he settled down and so we thought it was all a bottle issue. He would gag and sputter, was full of gas and often cried when done the bottle, as well as vomitted at times. We had evenings where he would cry and cry and refused to come near the bottle, we would walk and rock him until he finally slept. What we didn't realize at the time was that his body was having a major protein jolt, and this obviously wasn't going over very well. LPI does not show up when nursing because the mother break downs the protein and so baby only gets already "processed" protein. It took James' body a couple weeks, but surprisingly he did settle and began to enjoy his bottles.

RESPIRATORY ISSUES

Looking back through James journal when Matthew was born I began to see there were other signs of trouble earlier on. At two months old I wrote about how James always seemed congested, especially at nights. At first I thought he was coming down with a cold, but the congestion was only at nights, and he never got worse and never had a runny nose during the days. I didn't think much of this either, since Rebecca had asthma from a very young age, which she only gets at nights, so it just seemed he had the same thing, and since it wasn't bad enough to keep him awake at nights I didn't bother trying puffers on him ... until he was about a year old. At 11 months he developed a cold that never went away ... except the symptoms only remained at nights, with an awful cough during the nights. So we tried puffers for a bit to see if it was a form of asthma, but this did not help and we eventually took him to the doctor who prescribed antibiotics, which helped ... and so began our respiratory battles of treating the symptom (a secondary infection) by not the cause ... of course at that time we had no idea that this was what we were doing. At about seven months old we began to notice that James would have these bouts where he would have a clear runny nose. We could not figure out what woud cause this, as he did not have a cold (which would have been green and not clear) and appeared well otherwise. We thought it was an allergy of some sort. It would come and go and we never were able to figure it out ... but time would tell us this was part of his lungs/PAP, as in the next years we would see this symptom come and go often. Also already at a young age we noticed that James sweat excessively when he slept (as in he woke up looking like he just took a bath). This also we would later find out to be a symptom of PAP.

FAILURE TO THRIVE

James' 13 months doctors appointment it became apparent that he was not gaining weight as he should. Considering the fact that he was not eating solids, this made sense. Although the doctor noticed it, she was not extremely concerned at this point yet, but did send us to a dietician. Since you usually only see the doctor at 6 months and 13 months (9 months is optional, I opted out of it) she did not have weights for the inbetween period. I did have weights for each month inbetween 6-13 months and it showed that he dropped around 7 months (when we weaned him and his body had to struggle to process the protein) and he struggled from then on. slowly dropping from the 10th percentile to the 5th. Since it was not an immediate or extreme drop we did were not immediately overly concerned. What the doctor did point out at that time was that even though he had a belly the size of a whale, he didn't have any fat on the rest of his body.

SEIZURES

As James neared a year in age he began to have what we thought were night terr ors. He would wake with a specific cry, almost a scream. When we would get to him he would lay there and would not stop crying, he would not recognize you or respond to you. If you picked him up he would be like a rag doll one minute and suddenly be completely tense, going back and forth between these two modes. These started quite mild and not very often, but as we got nearer to his diagnosis at 18 months, they were happening a bit more often and each time it would get worse. Near the end they would last longer and he would end up vomitting. It became obvious these were not night terrors and so they were thought to be seizures but we did not know what from. An EEG was done to see if they could find anything abnormal, but this came back fine. Later we realized this was because of the excess protein; once we had him on the proper diet we never seen another one of these episodes

PROGRESSION OF SYMPTOMS

And so, yes, there were signs that something was wrong during James' first months of life, but we did not put all the signs together to read them as a real problem. The past couple days as I read his journal and look back I wonder how I put up with such a fussy baby who took so much of my time, needing to be nursed every 1.5-2 hours, sleeping very little throughout the day, wanting to be held all the time, crying most of the evenings, and usually only sleeping 5-6 hours for the longest stretch at night, meaning I was often up twice a night. Although the obvious medical challenges began when he was 15 months old, right from the beginning he was a child that took a lot of our time, and considering we had two young girls life, was certainly busy. At 15 months of age we were awakened to the realization that there was something indeed wrong with our little boy.

On Tuesday, March 28 2006 James woke up miserable and looking sick, as the day progressed things got worse. By mid afternoon he would just sit there and cry. If you did not hold him he would just let himself fall backwards and lay there crying. I had booked an appointment with our family doctor earlier thinking he had an ear infection, but as he became more and more miserable I realize there was something else going on. That night James was first admitted to St. Joeseph's Hospital and later transferred to McMaster Children's Hospital ... and this is where the blog picks up on James' story. I will never forget that first night in hospital where he cried for hour and hours and nothing would quiet him and eventually I just cried with him for I was so tired I didn't know what else to do. For whatever reason he would not allow me to sit, but I had to stand or walk, should I sit he would cry even harder, throwing himself around in my arms. During those first hospitalizations I found myself humming Hymn 48 all the time to him, I'm not sure where or why I picked that hymn, but it stuck and for the next 20 months I would find myself humming and singing it often. We wonder how much pain he was in at that time to cause him to act this way, for over the next year and half he went through a lot of pain, but was always tough and brave and never cried like he did those first two hospitalizations.

The next three months were difficult as James' health continued to show signs of trouble and we dealt with the frustrations of our medical system. Looking back we realize that we caused James' extra discomforts during those three months by attempting to feed him high calorie formulas in order to get him to grow. High calorie = high protein. His poor little body. It was at that time we began to also see the signs of colitis and we feel that we may have caused this colitis by the excessive protein we tried to give him. But we had no idea and we thought we were doing what was best for him ... just as later we would realize that GM-CSF treatment likely made things worse instead of better. He was finally re-admitted into hospital in order to do investigation and thankfully the answer came a week later.

DIAGNOSIS
What joy we had on July 5, 2006 when we received the diagnosis of LPI. Although it seemed overwhelming and difficult at the time, we were excited that we had an answer and could move foward, realizing that with some adjustment we could be handled this change in his diet. What disappointment it was to only five days later end up in hospital again, our excitement was quickly dashed. Here began the first of several hospilizations that were a result of his PAP, but it would be another year before we would get this diagnosis.

SECOND HOME

And so began James short but long journey. The hospital became like a second home. He even called it "home" and this past summer he rather enjoyed it there. At that time he was still relatively well and had plenty of time free of "leashes" as he was not on oxygen during the day yet. To him the hospital was a lovely playground where he always got one on one attention. This seemed to overpower the bad things that happened to him there. When we received LOA's to go home for a few hours in the afternoon he was alway eager to go back to the hospital and as soon as supper was over he would sit on the stairs by the door waiting for someone to put his sandals on and would yell "Goooo Hoooomme!!" Getting louder and longer each time he said it, and it took much to distract him and tell him that I was not ready to go just yet.

TEDDY
Previous we have talked about dear Teddy on the blog (see here) and as you look through the various pictures on the blog you will often see Teddy hanging out somewhere in the background, or maybe even in the foreground. Teddy was indeed James' best friend. James and Teddy had their good time and bad times, their close times and distant, but through thick or thin Teddy was there and there was nothing worse then losing him when visiting at someones house ... 40 minutes later and we're still looking for Teddy (remember doing this Dave?). The odd thing was that usually James knew exactly where he was even if they hadn't played all day. But when ready to go home from visiting, he would be asleep already and we were on our own to find Teddy.

What a comfort Teddy was to James, who took him to bed with him all the time. During the earlier days of their friendship it was hard to tell how real teddy really was in James' mind, for he took him everywhere, had him partake in everything, "talked" to him, fed him, took him for rides in his truck, etc. There was a stage where James was slowly going his seperate way from his dear friend and would even go to bed without realizing he did not have Teddy. But when he became sick again it became apparent how important Teddy still was. Especially on the pictures of his last hopsital stay you can see Teddy in a lot of the pictures and even though he took a liking to his new SickKids Teddy Bear, when the going got really rough it was dear old Teddy he depended on. Teddy and Blankie, what a comfort those were to James.

TRUCKS

"I like trucks, I always will, big or small I like them still. I like trucks I always will. I liiiikkkkkeeeee trucks!!" Anyone who's spent a bit of time with James will know this song.

And what boy does not love his trucks?

Already as a little boy James loved trucks for when we drove down the highway he would point each of them out to us. He was thrilled whenever Rob brought the truck home to wash it and would joyfully sit in the drivers seat watching Rob and the girls at work. During the spring, James' true boy colours shone out even more clearly as they began to do work on the next road over. It was our road that all the trucks and equipment would travel up and down to get to the construction site. All day long gravel trucks, loaders, backhoes, dump trucks, etc travelled by our house and pretty much all day long James stood by the front window, out on the porch or in the driveway watching them. We found some imitation plastic equipment to match each of the vehicles that would go by our house and James spent many hours playing with these ... or instructing others on what their proper names were.

James also found a great use for these toys ... they became his mealtime entertainment. Once he stopped eating any sort of solid food we had to work a bit harder to keep him entertained during mealtimes, but it wasn't long before we discovered the trick. Give him his excavator and dump truck, along with his bowl of food and he was content. He would scoop the food up with his excavator, dump it into the dump truck, back the dump truck up to his bowl and unload ... and so the process would go for the duration of the meal. Now talk about breaking the rules!!

In hospital James spent a lot more time watching TV ... Treehouse to be exact ... so much time that he could sing the Treehouse "song" and say that proper things are the proper time when watching Bob (or many of the other shows). He loved to watch Bob the Builder and Mighty Machines. The great thing was that mom and dad loved to watch those shows too ... especially dad!! :)

When well enough James liked to sit up in his hospital window watching the cars and trucks down below. He had excellent ears and could hear the firetrucks coming (code Reds weren't all that uncommon) and then I'd have to rush to get him to the window so he could watch all the firetrucks that parked on the street below.

LIFE IN GENERAL

We already talked about James' early days of crying and stubborness, but even at a young age he still was an enjoyable fellow ...as are most all babies. We had are fun with him. And just like all children he slowly developed a character. There is no way to explain James' character. A nurse recently wrote in a card that there was no good way to describe James but to simply say he was "James". He had an ability to accept all that was put before him and still enjoy life. Even when ill he managed to carry on, mostly happy and content. Each time we seen him head on a downhill slope it took us a long time to feel that he was truly sick enough to take him to Emerge because he would tolerate everything so well. He's walked around with fevers as if nothing was wrong, playing and enjoying himself. Several times I arrived in Emerge embarrassed because once we got there he perked up enough to go play and appear well again. We often ended up in Emerge during the middle of the night, which was always his worst times. He always knew when it was dark out and mom and dad were packing him into the van that he was heading off to the hospital, but he never complained. It was like he knew that he was sick enough and needed the help and this gave him some sort of extra boost to appear well when he arrived. As time went on the doctors began to realize that he was indeed sick and it was only a matter of time before he'd "crash" again and they'd see the true symptoms.

And that was how his character was throughout his short life. He took challenges in a strides, adjusting to each one of them as they came. He of course did not enjoy pain or hurt anymore then the next person does, but he rarely bucked the system. He quickly knew who the IV nurses were and would repeat over and over "all done, all done" but at the same time he would put out his arm and lay perfectly still while they did what they had to do. In later days he knew the x-ray department and for each trip we went down he would repeat the whole way down "no picture, no picture" almost working himself into a state of panic. But when he arrived he did exactly what he was told, and after a while even told them how it should be done, in typical James mannerism which everyone loved. Then he would have a pleasant ride back to his room. And after he completed each procedure, test, poke, prod, etc he returned to his happy cheerful self again, waving a friendly good-bye, never holding a grudge.

At 18 months James began to be tube feed and he accepted this attachment without much issue. Instead of seeing it as a restriction, already at that age he learned how to push the IV pole around to get to where he wanted to go, and calling for help if it was somewhere he couldn't push the pole by himself. He knew the limits on the length of his tube and how to watch that the tube wouldn't get stuck under the wheels of the IV pole. As he got older, he did get a bit lazier, realizing that if he called for help it was easier and faster then if he did it himself, but when really determined he would do it himself. Near the end this was no longer possible as he also had an oyxgen tank but by that point he didn't spend much time wandering around anymore.

We remain so thankful for the beautiful character that James had. Although he was a large burden to our household, we never seen it as a burden at the time. He was so easy to love and so easy to work with that it was just simply the way life was. I suppose he rubbed off on us as well. He took a lot of our time, especially time away from his sisters, but they never complained, for they too seemed to warm up to this loveable character who never complained and always enjoyed life.

Sleep was something James never put much stock into. As a baby he often was awake until 10pm (and sometimes later). He almost never slept through the night and was used to a disturbed sleep. Naps were not something he believed in doing as a baby. Eventually he did begin to have short naps. So when it came to hospital life James didn't think much of the idea of sleep either. Usually he went to bed between 10-11pm. He eventually learned how to take naps at the hospital. As his health grew worse the sleep he did get at night was very broken up and far from peaceful. We spent plenty of nights sleeping in the chair with him, or him in the chair and us on the floor beside it (as in the pictures). There were also the many, many nights where we slept on the floor in his room so that we could hold his hand for all he wanted was the comfort of knowing we were there ... and then the occasional time where he slept on the floor with us. While home, many times a night we were in his room because he was coughing, moaning, deSATing etc. He seemed to sleep through alot of it, but you really wonder how much true sleep he was getting. Often we had to wake him up to get him to breath proper or readjust him. As his nighttimes became more restless he did end up taking a bit longer naps, but in general he was a child who simply did not take much time out for sleep.

The last months of James' life were mostly spent at home. He became homebound because of the humid weather and the large amount of time and effort it took just to take him out on a small trip. It was amazing to see the joy that lit his face when he was allowed to go with somewhere, even if it meant he stayed in the van the whole time and just went along for the drive.

It's so hard to think of the last time we pulled him out of bed at 5am the morning and took him to Emerge and how he never got to see home again. Since then, when he woke feeling bad during the middle of the night the first thing he would say was "go home". During the day if something was happening that he didn't like his first words were usually "go home, all done". As time wore on he seemed to accept the hospital as his new home and asked less often, but it still broke my heart.

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During his last year James took a liking to the telephone. Spending so much time away from his daddy and sisters he discovered this as a way of staying connect with them and eventually also learned to talk to Grandma and Joyce too. Each time I spoke on the phone to them he would ask to also speak to them ... exce pt he would never speak. He would hold the phone to his ear, his eyes would shine his glee and there was the occasional nod of the head. He was content to do this for quite some time and I would have to take the phone back from him and rescue the person trying to keep a one sided conversation with him. During his last hospitalization James figured out how to actually speak into the phone. It started with a simple "hi", slowly progressing to his famous "uh-huh" and "yep". As time went on he learned how to answer basic questions about what he was doing or watching on TV and how to start and end a conversation. Our last peace of video tape about James is with him speaking to daddy on the phone ending with his precious "Bye Daddy-O". We have no idea where the Daddy-o came from, but for some reason James took a liking to adding "O" to names ending with 'y'... It started with "Hi Daddy-O", but slowly progressed to "Hi Mommy-O" and then Teddy-O, Barney-O .

James had a great love for ride-on toys. He did not walk until he was 20 months old, but as soon as his feet were long enough to touch the ground he used ride-on toys as his mode of transportation. As he got older he continued to enjoy these toys, and if it had a spot to store things you would often find Teddy and a few of his favourite dinky cars in the 'glove compartment' under the seat.

They also came in handy as a way of allowing him some freedom when hooked up to his feeding pump.

And what more fun than to fix your car ... just like daddy does.

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Camping at 6 and 7 months old

Daddy's Boy

We will never look at a fooseball table in the same way anymore. James absolutely loved foose

ball and whenever well enough to be out of his room when staying at Mac you would find him at the fooseball table. He became very impressive at it and by time we left Mac he has master the idea of the game and was a challange for me to play against (yes I'll admit I suck at the game - but he was still talented).

Treatment time:

Two weeks before James ended up back in hospital we finally moved him from his crib to a toddler bed. Something we should have done long before that but were always afraid of what might happen if he decided to get out of bed when hooked to all his wires and tubes. He loved the toddler bed and never once crawled out of it.

I didn't have much time to think about how James was going to take to the idea of a baby in the house who would still the never-ending attention that he seemed to get. Good thing I didn't think about it or worry about it because true to the rest of James' character this too he accepted, adjusted and moved on. He loved little "Matt-two", always asking to hold him, helping rock him, watching him play. He never once showed any sign that he thought Matthew was interferring with his life, he found this change entertaining and simply accepted it and carried on. He was a proud big brother.

These pictures were taken a couple hours before James passed away. In James hand you will see his absolute favourite truck. Some of you may remember this truck from various visits, or our previous posting on his 2nd birthday (see here). We had lost/misplaced this precious truck in the spring. The night before James passed away Rob put his winter coat on for the first time for the season and in there he found the Leon's truck. I was not there when Rob showed it to James but I hear that they have never see his eye light up so big or his hand shot out so fast. He did not let go of that truck again until he passed away.

And so there are so many more stories I can write about and pictures I could tell about. Memories that we hope we will forever be able to remember. Although James did not make it to his third birthday and will never be able to celebrate this birthday we take this day to remember that three years ago the Lord entrusted us with a precious gift for which we shall forver remain thankful and grateful for. The Lord in His infinite wisdon decided to take this gift from us and bring him home to a place free of pain and suffering, for this we can rejoice and be glad. Our hearts will forever miss our special boy, but as Life Goes On we can look forward to the day when we too will be together with our Heavenly Father free from the pain and sufferings of this world. And so we say ...

Good Night, Sleep Tight James

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Blog Printing

2007-12-14T14:02:00.000-05:00

Well it seems the first comment on our post Thank You All has already mentioned what I had hoped to asking in this next post.

We would like to have James' Story put into a printed format and have heard that it is possible to have your blog professionally printed, but we are not sure where this is done and we wonder if any of our readers have had this done and could guide us where to go ... or even if you haven't had it done but still know a place that prints blogs. The one place I did ask told us they only print things that they receive in "hard" format on a disk and as far as I know there is no way to back-up a blog onto a disk. Since then I haven't had a chance to look around any further.

If anyone can help us out that would be greatly appreciated

Thank-you All

2007-12-14T08:40:00.000-05:00

It's hard to believe that it's only a little over a year ago that I began this blog. But as we read the purpose on the side of the blog (and here) we realize that our purpose has been completed. There are no more updates to provide about James health, for he is now completely healthy and happy. With that in mind we feel that it is time to end our blogging on James' Story and so tomorrow, on what would have been James' 3rd birthday, we will post our last entry.

We had no idea when we started writing that our blog that it would end up being so widely read, nor that so many people, although they never really met James, would treasure him from what was posted on the blog. I think that had we known this reaction, we likely would not have posted, not wishing to draw affectionate attention to ourselves, or to any of our children. We simply wished to project information. And for the most part, I do think we remained within that scope. Some people let us know that they skimmed through the blog because often there was too much medical information. Even though we were not writing to entertain readers, we became mindful of this, and posted relevant links from which anybody read up on if so inclined. At times we summarized the big picture or made analogies so that all our readers could understand what was going on. In spite of this objective approach, James touched so many hearts.

When many people began encouraging us with their comments, we realized that many were praying with and for us, and so found it necessary to update in a timely fashion so that our supporters knew what to petition for. It is without a doubt that God did answer our prayers. Even though we prayed for the restoration of health, God said no, but then gave us the assurance that James is so much better off. He gave us the strength, courage, persistence to carry this burden already from James' birth, and continues to do so as we cope with our loss. Many see this as a testimony of our faith. This is a slanted view. We see this assurance as an answer to all your prayers. This is not in ourselves, but a gift of God, a benefit of belonging in Christ.

And so, since our readers were and are an important role in "James' Story," we feel that it is only proper to thank you all for all that you have done. Many in our vicinity, and even further abroad have been able to support us with gifts besides their prayers. These were gifts of love responding out of faith. We are very grateful for these. But primarily, your prayers were coveted, and the fruit of these is immeasurable. We have been warned by others who can relate, that the grieving process takes along time to work through. And for some, it hasn't finished. Let us not forget them either. "Rejoice in hope, be patient in tribulation, be constant in prayer" (Romans 12:12).

As parents, we have had our faith tested and refined. As husband and wife we have been drawn closer together, realizing that it is not by chance that we are one, but by God's providential hand that we have each other and our children.

Life Goes On and so our story does as well ... just a little less detailed and a little less often. But just as long winded. :)

Why

2007-12-10T21:05:00.000-05:00

Yesterday we heard a wonderful sermon on Matthew 27:46b "My God, my God, why have you forsaken me?" in which Christ quotes David from Psalm 22:1. The "why me" question! Through all we been through I cannot say that my mind has dealt greatly on the "why me" question. Yes, of course there have been the times when it crosses my mind, but probably more in a "why" question, then a "why me". A more general question of "why" does the Lord allow such things, "why" must we see James suffer so, etc. And this "why" question can simply be answered by "sin and Satan", not always simply accepted, but the answer remains simple ... we live in a broken and sinful world. Although the sermon in itself did not reveal anything earth shattering or new, it served as a gentle awakening, a reminder of what we already know, that God will never forsake us. The minister ended his sermon with a quote from Hebrews 13:5 "Never will I leave you; never will I forsake you." What a comfort we have.

And today to add to the many blessing we have received from the communion of saints, we received a letter. It was almost like the person had read my blog entry from Saturday and responded, except it was dated for the Thursday before I wrote my blog entry. This letter spoke clearly about our conflicting emotions ... grief as well as peace. Grief because we miss James; peace because we know he is better of, but that we can do both, in Christ our Lord. The writer acknowledged how this at times will also feel like a conflict in our mind but that we greive, as those who have hope; and we have peace in the promises of redemption and eternal life ... we can indeed do both. How comforting it is to hear that our feelings are normal and to receive words of encouragement from so many members.

And so we can go on each day again, knowing that God is there. Just as we read in the poem Footprints in the Sand ... the Lord carries us through the difficult times.

Heart vs. Mind

2007-12-08T15:10:00.000-05:00

Yesterday marked two weeks since James passed away. In some ways it feels much longer then that as we have been so busy and doing so much since that day, but in other ways it feels like it was just yesterday that we looked into those big hazel eyes for the last time and said our good-byes.

These past few weeks have definitely had their ups and downs, something we fully expected. Some days I start to wonder when things will get better, and other days I realize that they are probably going to get worse yet before they get better.

There are days I wish I wouldn't feel like crying about everything, and there are other days where I wish I would just cry and get it over with. When I packed up James' room and other items no tears would come, instead I had an overwhelming feeling, as if I were going to be sick to my stomach any moment ... I sure wished for tears that day, for tears seem to heal. Crying may dry out the eyes and give a headache, but I often feel that after a good cry I am ready to go on again ... altho there are also times when the good cry doesn't stop as quickly as I would like it to. And then there are times I'm happy the tears won't come, for it'd be awful to stand in the middle of a store looking at Bob the Builder and be crying ... then a upset stomach seems the better option, if an choice must be made.

Now this may make it sound like this is all we do, but fear not, I do feel we are coping well, for it would be worse if we did not have the tears or the sadness. Most of the time we are able to go on and function to the point that it seems like nothing has happened recently. Especially during the day, when the kids are around to keep us busy, the house flows smoothly. We are able to freely talk about James, to enjoy the memories we have, to laugh at the things he used to do. There are moments throughout the day, for we cannot avoid the memories and the constant thoughts, but usually it is not unless left alone to think too much that there seems to be less control of the emotions. This often happens in the evening, when the daily pace slows down and there is more time to get sidetracked in our thoughts, or even just simply time for Rob and I to talk together without kids around.

I find the difficult part of the whole situation is the constant battle between the heart and the mind. My mind knows that we did all we could for James, and most of all my mind knows that he is in a much better place now. But my heart aches because it misses him and so it wants him back, thinking he'd enjoy being here, seeing as he was always such a happy fellow. My mind has to constantly remind my heart that he would not enjoy it here, for he is in the best place he could possibly be, and if anything I should be longing to go to him, not longing for him to come to us. And although my mind knows that we tried all we could, my heart keeps asking these what ifs. What if we had just a bit more time, would they have found an answer? What if we had just not done the last WLL and instead a biopsy? What if? What if? And yet, my mind knows that we did all we could humanly do, and the Lord decided this is the course of things and so James' time would end now and not later. For in reality, the chances of avoiding death were slim ... and so all the what ifs don't change the fact that the Lord at made a room ready for him and it was his time to go. If only my heart could accept what my mind already knows.

If my heart could agree with my mind then maybe I wouldn't feel guilty for enjoying some of the freedom I now have. On Monday I was able to go out shopping for the whole day, from the moment the kids left for school to the moment they came home. And throughout the week I've been in and out of the house without the constant worries of what is going on at home, for seldom did I leave for more then an hour or so before. Of course this is all made even easier by the fact that Rob is home right now, but even when I do take the kids with it's not so hard to quickly bundle up Matthew and away we go. We were able to stay over at my parents last night, when in the past year we've only visited a few times because James was too unstable and it was so much work to pack everything just to go visiting. And although we enjoy this new freedom, it also comes with a constant feeling of guilt. James was definitely a burden, but we never really seen it that way, for he was always so cooperative and cheerful, he made it a happy burden to carry, to the point where we no longer seen it as a burden, just simply our task as parents. How can we be happy to have freedom when it means we've lost a child to get it? And there my mind has to remember the freedom James now has ... freedom from all pain and suffering, freedom from sin and the evils of this world. He has more freedom then us, so heart please stop making me feel guilty and listen to my mind.

And so the heart vs mind battle goes, I pray my mind will continue to stay in control!!

Common Questions

2007-12-04T21:10:00.000-05:00

Over the past week we've been hearing a few of the same questions. So I thought I'd take a few minutes to answer them for those of you who have not asked us (yet).

Starting in January Rob will be going back to school full-time. There was some concern about him being able to go back since one of the courses he dropped was a full-year course and isrequired in order for him to get his B.A. You must complete the entire year in order to get the credits for a full-year course and of course you cannot start the course in January when it is half-way through. This course was one of the reason we had such a struggle deciding whether Rob should drop out of school since we knew he would not be able to go back in January as he needed this course to get his B.A. Last week Rob spoke to the Dean and the Professor of this course and they have allowed him back in the course. This means he has an exam to write on December 19 and at that time he also has to hand in an essay proposal which was due while he was gone. So that means lots of studying required the next couple weeks to catch up on what he missed, but all he needs to do is pass so he can carry on. If all goes well Rob will need to take one summer course next year and then he will have his B.A. ... and on to the College, the Lord willing.

In the meantime Rob has decided not to work this December. Usually December is a mad rush to work as many hours as possible to earn those few extra bucks as well as trying to catch up at home on all that was put on the backburner during the months in school. We felt that family was more important at this time and so even though we're both rather busy, we're enjoying some time together as a family again.

The past week has been busy as we try to catch up on many things. For myself it means reorganizing the house again. When we left in September I was just in the process of rearranging things as we tried to figure out how to fit Matthew in our house. Matthew had outgrown his Moses basket and could no longer just float wherever we needed, but required his own space. It became apparant that James was not able to share a room with anyone (much to Rebecca's disappointment) due to the continuous alarms and attention he needed at nights. So we were attempting to squeeze Matthew into the girls room. At the same time it was the time of year where you get out the winter clothes, etc. So I left the house half torn apart, and it didn't get any neater while we were gone. Upon returning home we had to "move" back in and then I began to the process of cleaning up James' stuff and rearranging the bedrooms again. James' room has been transferred into Matthew's room. Wow, amazing how spacious the room is without all that medical equipment. It seems James effected many rooms in the house. The girls room was transferred back into just the girls room. I now have an entire empty pantry in the kitchen as we no longer need all his special foods. James' toys are no longer needed in the dining room and so we make room for all the baby equipment that Matthew has. Packing away James clothes brought me to reorganizing the storage room again ..etc, etc. I'm sure many of you know how it is when you start in one spot and suddenly the whole house is upsidedown, but it's slowly coming back together.

And the most common question we're ask is how we're really doing ... and how the girls are doing. It appears the girls have mostly moved on as if nothing has really happened, but every now and then something occurs to make us realize they are still thinking and dealing with things. The other day I seen Rebecca sitting at my desk watching the computer screensaver which plays pictures from the last few months. Not long after she came to the kitchen crying "I miss James". For the next hour or so every little thing set her off crying again. Marietta continues to remain quiet and reserved, but appears to be doing fine. We also know her mind is still thinking. Before the funeral one night she suddenly blurt out during the middle of supper "how do you know James is really dead?" We explained how the heart stops beating, that you can feel it's no longer beating as well as hear it with a stethescope (she's used ours occasionally). One of the things she needed to do before James was buried was to put her hand on his chest to confirm for herself that his heart was no longer beating. Since the funeral the girls have mostly been themselves and have shown no reason for us to be concerned. They talk freely about James and like to watch homevideos of him.

I will not say that things have been easy, but all considering I feel we are doing relatively well. I am glad for the business and even for once thankful for my upsidedown house. I'm sure I would find plenty to do even if we had come home to it completely organized, but for the time being it's good to be busy. I am thankful for the children who keep us busy also. I can easily see how someone who does not have distractions can become swallowed up in their sorrow. We realize that our strength each day again comes from the Lord, who is there to help each and everyone of us in our individual circumstances.

Psalm 105:4 "Look to the Lord and his strength; seek his face always."

The Lord's Blessings

2007-11-28T20:45:00.000-05:00

This morning we went to pick up Matthew, after being away from us for 9 weeks. Although we had opportunities to see him and spend time with him during these past 9 weeks, we usually felt more like babysitters then parents. We weren't always sure exactly what his schedule was, or how he liked things. We would spoil him for a day or so and then hand him back over to be "retrained". The time we spent with him was often hard on us because it reminded us of what we were missing ... our boy was growing up and we weren't there to enjoy it. But each time we had to hand him back we took great comfort in the fact that he was young enough not to realize all that was going on and that he was in great hands that were taking excellent care of him. It's a strange feeling to realize that he is back with us for good; that we will not have to hand him over again anytime soon; that we can bond with him and cherish his growing moments.

Over the past few days many have commented on what a blessing Matthew is to us, and indeed he is. Through this small child we are remind again and again of how all things are in God's control, how He has a purpose for everything that happens, how He holds the Master Plan. Right from the moment of concept God knew what He was doing. Many will remember my blog from almost a year ago where I shared our shock and anxieties over the idea of having another child. But the Lord knew what the future held and that without Matthew at this designed time there would never have been another child. The Lord blessed the pregnancy and all went smoothly. He allowed James to come out of hospital with just enough time to prepare for Matthew's birth. He gave us a small amount of time to allow us to welcome Matthew into our family before He decided that Matthew would need to be a blessing in another family for a short period of time. And the biggest blessing of all is that Matthew does not have LPI.

I still remember as clear as day the night I came to fully realize what a blessing Matthew was to us. Matthew was a little over a month old. It was a sleepless night, where James alarms, deSATs, coughing, etc seemed to be getting me out of bed every few minutes. As I fed Matthew that night it hit me like a brick wall that the Lord had likely sent us Matthew to fill the hole in our lives after James left us. When I crawled back into bed my crying woke Rob, and as I explained to him my gut feeling I began to realize that he also had this same feeling. I prayed often over the next couple months that my gut feeling was wrong, but when the second WLL failed this feeling began to speak louder. As time went on we realized that only a miracle would save our boy and we thanked the Lord each time again for sending us Matthew at a time that we thought was so wrong, but yet He knew was so right.

So when everyone comments on what a blessing Matthew is I cannot agree more. But at the same time I more and more realize what a blessing it is that we have THREE healthy children. LPI is a genetic disease, meaning that with each conception there is a 25% chance that the child will have LPI, and the Lord chose to bless us with three children who do not have LPI and we cherish each day we have with them. We have an awesome task of raising them up in the Lord. Being away so much from each other will have lasting impressions on these young, sharp minds. Although this experience can shape character, we cannot expect another such experience to build character.

Funeral Information

2007-11-24T20:24:00.000-05:00

The Lord Willing on Tuesday, November 27 at 11am we will have the funeral service for James at the Hamilton Cornerstone Canadian Reformed Chuch located at 353 Stonechurch Road East (map). There will be a reception at the church after the interment.

Visiting hours will be from 6pm-9pm on Monday, November 26 at P.X. Dermody Funeral Homes located at 796 Upper Gage (map).

Good Night James

2007-11-23T15:31:00.000-05:00

James Robert

December 15, 2004 to November 23, 2007

Psalm 139: 1-18

O Lord, You have searched me and You know me.
You know when I sit and when I rise; You perceive my thoughts from afar.
You discern my going out and my lying down; You are familiar with all my ways.
Before a word is on my tongue You know it completely, O LORD.
You hem me in - behind and before; You have laid Your hand upon me.
Such knowledge is too wonderful for me, too lofty for me to attain.
Where can I go from Your Spirit? Where can I flee from Your presence?
If I go up to the heavens, You are there; if I make my bed in the depths, You are there.
If I rise on the wings of the dawn, if I settle on the far side of the sea, even there Your hand will guide me, Your right hand will hold me fast.
If I say, "Surely the darkness will hide me and the light become night around me," even the darkness will not be dark to You; the night will shine like the day, for darkness is as light to You.
For You created my inmost being; You knit me together in my mother's womb.
I praise You because I am fearfully and wonderfully made; Your works are wonderful, I know that full well.
My frame was not hidden from You when I was made in the secret place. When I was woven together in the depths of the earth,
Your eyes saw my unformed body. All the days ordained for me were written in Your book before one of them came to be.
How precious to me are Your thoughts, O God! How vast is the sum of them!
Were I to count them, they would outnumber the grains of sand. When I awake, I am still with You.

The day has come for James to finish his bath and to be tucked into his final bed. Our Almighty God has relieved James from his struggles and sickness, and called him to further Glory. And so in the next few days we will prepare to lay his body to rest, knowing that he already has eternal rest. Our comfort is knowing that "When I awake, I am still with You."

In a few days we will plant a seed...

Eight Weeks!

2007-11-22T20:17:00.000-05:00

After five doses of MethylPrednisolone James has not shown any significant improvement. Last time they gave him a Pulse Dose they gave him 10mg/kg once a day, this time they doubled the dose to 10mg/kg twice a day. So at this point he has already had more then the last Pulse Dose and the only real improvement we seen is that he is no longer having fevers and there’s a slight decline in his heartrate.

Our doctors has been in discussion for the last weeks with the doctors in Finland who are more familiar with LPI and LPI PAP due to the fact that 50% of the LPI cases are in Finland (about 50 LPI cases in Finland). Unfortunately even though they've tried many things they have not had any more sucess then here when it's children that have PAP. It seems that when developed as adults PAP is more of a minor "side-effect" of LPI, but with children it's progresses quickly, effecting the inflammation system and there seems to be no answers.

Over the last few days James has spent most of his time sleeping, or resting. A lot of the time he sleeps with his eye part open in order to watch in his sleep what is going on and wakes as soon as a nurse or doctors gets too close. This is a habit he developed right from his first hospitalizations. Eventually he does fall into a deep enough sleep that then you are able to examine him and he doesn't even wake.

It seems this excessive sleeping is simply because he takes so much energy when he is awake. We have tried to play with him or entertain him, or even just "chat" with him, but this can only be very draining and he really rather us just sit in the chair beside the bed and be there. The other day the clown came to visit him, and even though he enjoyed it, it drained him and made him deSAT. He is most content just to be left in his bed watching TV with the occasional chat.

Realizing what lies ahead the doctors have now removed the ICU restrictions. We are now allowed more then two "visitors" (we are classify as visitors, so that meant whenever someone visited one of us stayed in the waiting room and the other took visitors in one at a time) in the room at a time. The girls are also now allowed to come and visit whenever they want. As well we no longer have to call in to ask permission to come in each time and are allowed to come and go as we want, even during shift changes.

Yesterday was the girls came to visit for the first time. They are aware of what is to come, or at least we have discussed it, although we're not always sure how much is truly sinking in or how they are cooping. Rebecca seems to be doing fine. Before coming in her most important question was whether she would be allowed to give James a kiss and hug. Words cannot explain the smile on her face when we said she could. True to her question, the first thing she did was climb up on the bed and give him a great big kiss, or two, or three :) James and Rebecca have always had a special bond and we could see that yesterday when he allowed her to lay on the bed beside him, no one else is allow on the bed. Marietta is much more reserved, not saying anything and not asking anything .... and for anyone who knows Marietta you realize that this is not normal and does cause us some concern. We hope we can break through this and she will feel free to speak about what is going on in her head. Today the girls had an enjoyable time again, this time together with James they attempted to make a mold of their hands. It is currently waiting to dry and we’ll see how it turns out tomorrow.

For the past three weeks they have been restricting James fluid intake as well as medicating him in order to make him pee. The idea behind this is to keep him as dry as possible in order to keep his lungs dry. Realizing that he does not have a pneumonia or anything of that assort we have not really been in agreement with this continual treatment as we can see how much he wants water. Because he is intubated he cannot drink as it could end up in his lungs. He has become dependent on sponge suckers, which we are only suppose to moisten enough to wet his lips or mouth. We have been wetting them generously so that he can get some fluid out of them and he keeps them in his mouth pretty much around the clock. Today they stopped medicating him to pee and allowed him to have freezies. He was too tired to complete a whole one, but quite excited to have something moist and flavourful in his mouth.

We have been bombarded by doctors, child life specialists, therapists, whatever you want to call them all, in the last few days. They mean well, but I think we've gone over everything often enough now. On the other hand they are very good at making sure the siblings are kept in mind. They let us know of activities going on, even take them to the activities if we are busy with doctors meetings. They bring things for them to do when they come to see James and also try to help with any questions that we may have with regards to helping the girls through this. I think we have this all under control (as best we can given the circumstance), we're just a bit concerned about Marietta's quietness, but she may just need time and pouncing on her is not going to help things. Although she is quiet she is still enjoying herself immensely and both girls are loving all the attention, craft things, baking, games, etc that they get to do now.

Family Meeting

2007-11-20T23:59:00.000-05:00

James has recuperated from the pneuomothorax that he had on Sunday. They clamped the chest drain tube yesterday and this morning removed the tube. So far all looks well in that area.

It appears that James' battle is getting more difficult. The amount of oxygen assistance he needs continues to increase (now around 75-80%) and his x-rays are not showing improvment. Once again we are faced with the reality of the situation - James is not getting any better, there are no further options, and the technology we are currently using is not helping him to get better.

In light of this we had a "Family Meeting" (or so they call it) today with various doctors, the social worker, therapists, etc in order to decide how best to proceed.

They have started James on another three day Pulse Dose of MethylPrednisolone as it is felt that the last time we did this he did improve. The aim is once again to give him enough edge in order to extubate him. At the end of this Pulse Dose if his numbers are good they will attempt to extubate again.

We continue to pray the Lord will guide us in the right direction during these difficult days.

Ronald MacDonald House

2007-11-18T22:18:00.001-05:00

I started this post a while ago, but more pressing things have come up in between and it’s never gotten posted, so I try complete it tonight.

We've been at the Ronald MacDonald House for over week now and I thought I would take a few minutes to introduce our new home. We were quite impressed with the home, as have those who have come to visit us, so I thought I’d take a few minutes to let others out there know what a great service the Ronald MacDonald House is, which only costs $15 per night to stay at. Fortunately it doesn’t need advertising, seeing as it’s so hard to get into in the first place … but hey, if you’re ever looking for a cause to support, we’re all for this one.

The home is six stories. The first two floors are common areas and the top 4 floors are where the rooms are. In total there are 27 families at the RMH. Our room is like a hotel room with two double beds and a bathroom ... a less frilly version of the room on the Ronald MacDonald Website. In our room we also have a small bar fridge since there is very little space in the kitchen fridge for each person. Each room also has a desk and wireless internet connection (when it's working :)

The second floor has the common living areas, with a kitchen, dining area, playroom, lounge, computer room and quiet room.

There are three full kitchens in the big kitchen/dining area. Each room is assigned a kitchen that is theirs mainly to work in. This is a completely workable normal kitchen just like you find at home, and if you can't find something in your kitchen you can search through the other kitchens to see if you can find it. Each room is given three shelves of cupboard space and 1/3 of a fridge shelf. In the kitchen is also two common fridges for everyone to use; in there you'll find donated items (such as milk which is donated weekly) or items leftover from other residents or meals that have been served at the home. All residents are free to use what they want/need from these fridges. As well coffee, tea, sugar and cream are supplied to get us through the “staples”.

Three to four nights of the week groups come in to prepare supper. We have enjoyed several of these nights so far ... lots of good eating!! At the end of the night the leftovers are put in the common fridge, and it doesn't seem that many people eat from there, so there's always plenty of salads and foods in there for us frugal folks to enjoy the next day. A lot of these evenings they also have some sort of entertainment or gift for the kids. The pictures is from one of the evenings they had a pinata ... Rebecca giving it a try.

Almost every day there is some volunteers in the playroom to do crafts or cooking or play with the kids. We are allowed to leave the girls with them, but we must remain in the building. So this does not give us free babysitting time to spend time together at the hospital, but it does allow some quiet time to do some laundry, e-mailing, blogging, research etc ... or if necessary a snooze.

Each floor has a laundry room where we can do our laundry for free, with detergent provided. There is no cleaning service; we must clean our own rooms. We also each have a daily task in the kitchen (e.g. clean stove, clean fridge, wash floors, etc). The quiet room is a place to sit and read or make phone calls. In the quiet room is one of two phones in the home where we can make free unlimited long distance calls (with consideration for others needing to use the phone).

For those who are without a vehicle, there is also a common vehicle for the residents to use for around town only. I guess that stipulation prevents anybody from putting the vehicle with the best 4-wheel system to the test (Rob’s comment, not mine).

As mentioned in a previous blog the home also has a school, which the girls are now attending. There are only six students right now and only 3 students from K-3. Rebecca is the only Kindergarten student and Marietta the only Grade 2 student at this time. We were told originally that the teacher follows her own curriculum, but thanks to the wonderful job the girls teachers did on putting together a work package, and the fact that there is no one else taking the same grades as the girls, the teacher is following the girls current curriculum. We are really happy about this and this leaves us to do their Bible Studies.

The girls are thoroughly enjoying it here. Eventually the novelty will wear off but for now they think its one big party. We have set up a bit of a routine and that routine became a bit easier when we were finally allowed a cot in our ICU room.

Previosuly whoever went home for supper with the girls also was suppose to go to bed at the same time as the girls around 8pm (okay I admit it, I never actually made it by that time, it was usually closer to 10) and slept until around 1:30am, at which time whoever was at the hospital came back to the house to switch and get a few hours of sleep. So most nights we were getting about 4-5 hours sleep. Our bodies had adjusted to this and it was going okay, the bigger pressure was the fact that you always felt when you were at the house you needed to sleep, leaving little time to do anything else and always feeling you were rushing against time.

Thankfully ICU finally decided to be nice and allow us a cot. This has made life much more “normal” … or at least the sleeping part of life. Now we take turns staying at the hospital for the night. If a night were to go really badly then we can use the time the girls are in school or doing crafts to snooze. This also means that whoever is at the house with the girls for the evening can actually take time to return phone calls, e-mails, do blogs, laundry, etc without feeling majorly pressured to get to bed.

As much as I say we’ve set a routine, in reality I suppose it hasn’t always worked that way. There are many things in a day that can throw it off, whether it be something going on immediately with James, waiting to talk to doctors, visitors (not that we’re complaining), etc. But I suppose that’s much like normal life, routine can only work to a certain extent. Whatever the case, the Ronald MacDonald House has helped us to bring part of the family together again. It has taken several reminders to get the girls to understand that this is home now, it’s very different then a normal home, but there are still rules, bedtimes, school, etc and they have to adjust to this new concept of “home”.

We are thankful to have the girls here, and we thoroughly enjoyed our time with Matthew this weekend, except it made us miss him more and wish all the more there was some way we could fit him into our lives as well.

Pneumothorax

2007-11-18T19:40:00.000-05:00

So I know a lot of you get confused and can't keep up with all these medical terms and here I throw out another new one.

Early (5:30) each morning they come around and do an x-ray. This mornings x-ray came back showing that James had a pneumothorax on his left side. What this really means is a collapsed lung or more specifically it means there is air in the pleural space, which is the membrane between the chest wall and the lung. When air gets into the pleural space it deflates the lung, or gives the lung less room to function and can eventually cause the lung to collapse. The first picture below shows what normal lung and pleural space should look like. The second one shows what a pneumothorax looks like, in this picture the pleural space is filled with air leaving little room for the lung.

The initial x-ray this morning showed a very small amount of air in the upper left pleural space of the left lung. They decided to decrease the pressure they were giving James through the ventilator and increase the amount of oxygen they were giving him to 100%. Hoping with less pressure and lots of air coming in the lung would reinflate and stabalize on it's own.

Just after lunch time they did another chest x-ray. Unfortunately this approach had not worked and instead James' lung had collapsed half way ... too look almost identical to the picture above (except on the left lung). A few more hours and it would have completely collapsed.

How did this happen? It's hard to say for sure, but it likely is a hole in the lung at the biospy spot which is allowing air out of the lung into the pleural space. Similar to a bicycle tire ... if you pump to much air in the tube it will burst. The lung will also burst or open if you pump air too hard into it. It is possible that the higher pressures they had to use when they reintubated James caused a hole in his lung. Considering he just had a biopsy the most likely spot would be the biopsy spot as it probably had not completely healed. That being said, the actual spot cannot be confirm and it is also possible that the hole developed in a completely new spot, that we don't know, we just know the problem needs to be fixed.

Fixing the problem means inserting another chest drain tube. This new tube was inserted at the upper left side of his lung, where the air space was found on the first x-ray. Suction is applied to the tube once it is inserted. The idea behind this is that they suck the air out at a greater speed then it is coming in, therefore drawing/sucking the lung back to it's full size and hopefully allowing it to heal again.

So tonight James is recovering for another mini-surgery and aneasthetic. This procedure was done right in his ICU room and took them about 45 minutes to do. At this time it appears all went well and they talk about clamping the tube tomorrow and removing it the next day. But they also told us the last tube was going to come out the right away and it ended up staying in for 5 days ... so we know not to count on it out that quickly.

Earlier this week we were concerned about James' "attitude", or lack thereof. We would try communicate with him, talk to him, read, play, etc. but he would just stare past us and was unresponsive, except maybe to tell us to move because we were blocking the TV. We discussed this with the doctors and they said this is not uncommon and considered ICU Depression. At that time we had hoped he'd be out of ICU soon and so we were not overly concerned as they said usually children snap out of it within a week of getting out of ICU. It's funny how the recent days seemed to have snapped him out of it. Ever since they re-intubated him he's had more life to him, been more responsive and more fiesty when he doesn't want something done. It's hard to have to fight to do things, because then we know what's really going on in his head and how much he dislikes everything, but we're glad to see it as then we don't feel like he's already given up on us.

I left off my last post with a very big question: if they can't extubate now, just after having MethylPrednisolone, will they ever be able to?? We of course can't know the answer to this until we try again, but the doctors were very honest and upfront with us, confirming that our fears are not unwarranted.

Tomorrow the Respiratory Team has it's usual Monday morning meeting at which time they will go over all that has happened and at some point then sit down with us to discuss what they feel is the best plan and to find out how we would like to proceed. Whatever the plan becomes, the biggest decision next time will be whether we re-intubate if he cannot do it on his own. We had (and still do) hoped and prayed that this decision would not fall into our hands once again. If we thought it was a hard decision the last time we had to make it, we definitely realize that this time will be 10 times harder. Our heart breaks for our boy who is suffering so, and yet we must always remember it is not our decision to take a life. God will bring him home when the time is right!! We pray for strength when that time comes.

We have been asked several times and I keep forgetting to address this question. So before I forget again, the question often comes to us is if they could do a lung transplant. This has been attempted in one other case that they know of, unfortunately that lung also developed PAP, and eventually that patient passed away. The problem with LPI PAP is that it is the underlying LPI that is causing the problem, and until they can find out how to prevent the LPI from causing the problem to reoccur a lung transplant will not help.

Intubated

2007-11-17T10:02:00.000-05:00

James was extubated at 2pm yesterday, he was re-intubated at 6am this morning.

There was some argument about whether he was ready to be extubated; the RT's (Respiratory Therapists) looking at the numbers and saying he was not ready and finally the doctor stepping in and saying he does not follow numbers, and so we were going to try it. When discussing with the RT's, I had a hard time getting clear enough answers to understand if they were aiming for the impossible with James. All the numbers on the ventilator were where they said they should be for extubation but still they said he was not ready. In their opinion he was breathing too fast, he was dropping his SATs too quickly when suctioned, and he was needing too much suctioning. If we wait for those things to change they would never extubate. He was breathing slower then his regular breathing rate and he always drops his SATs immediately when taken off oxygen. While intubated, James will always need suctioning because his lungs continue to produce surfactant that he cannot couph out, plus being intubated by itself creates extra secretion.

The doctor ended up stepping in to say they were going to extubate; he agreed with us that James was about as ready as he could be. He also put it clear that although the RT's have their input, it was not their decision to make, but his. The worst that could happen is we would have to re-intubate. Unfortunately that worst happened.

James was put on a regular mask after extubation. This means he just had air flowing to him, similar to nose prongs, but they can provide greater volumes with a mask. Surprisingly he tolerated this fine after his initial few minutes of fighting. He was having deSATs, not uncommon to James but more frequent then usual. We had hoped that once he went to sleep and relax he would get to better breathing going as well as time to recuperate from the extubation and be better off tomorrow. Considering James was on C-PAP when on the ventilator we did not think he would need to be re-intubated.

Unfortunately we were not with James last night, so I cannot say what really happened to bring the decision to re-intubate and we'll have to discuss it further with the doctors. They did try to put him on BiPAP twice, but he got so worked up that it just made things worse and so they re-intubated. We wonder if they could not have further sedated him in order to calm him. If he woke up with the BiPAP on afterwards he would likely have accepted it much better then having a crowd of people surrounding him and doing things to him which just make him panic. We are reminded of why we like to be with James at all times.

But whatever the case he has been re-intubated and we now have to emotionally accept this and deal with it. The biggest question is: if they can't extubate now, just after having MethylPrednisolone, will they ever be able to??

Pictures of the mask he was on.

Some day I'll have a chance to upload more pictures, but right now we're having internet issues that make it a bit time consuming to add pictures.

Extubate??

2007-11-15T21:46:00.000-05:00

The doctors have ordered to stop James' feeds at 4am with the hope of extubating him sometime tomorrow morning.

Over the last two days they have slowly been decreasing the ventilator settings and by the end of today he was almost at the levels needed to be extubated. His blood gas levels showed that he is doing well on these settings and so they're hoping to be able to extubate tomorrow.

We have no idea what type of oxygen support James will need once the ventilator is removed. He could go back to nasal prongs or he may need BiPAP or C-PAP (these provide air pressure to the lungs helping to get more air in and out of the lungs), or there's a possibility that they'll have to intubate him again ... we won't know until we try.

This is so exciting, but at the same time we know not to put our trust in "plans" as James' likes to throw loops in the doctors plans and we end up even more disappointed in the end. It will be
so nice to hear his voice again, to give him that water he's been asking to drink for two weeks now, but most of all to pick him up and hug him.

We have cannot say for sure whether the MethylPrednisolone had anything to do with the fact that they've been able to wean James. Before the lung biopsy James' was getting close to extubation, so it's possible that he just recuperated from the lung biopsy and was able to slowly be weaned. It is more likely that it was a combination of the two, healing from the biopsy and reducing some of the inflammation with the MethylPrednisolone. Whatever the case, the last couple days have been more positive.

They continue to slowly wean down the morphine James is on. Today his chest drain tube came out and seeing as we didn't hear anything back about the x-ray we'll assume all looks well in that area also. Results from Immunology came back today as well. They have looked at his B Cells and T Cells, which are white cells that create antibiodies to fight infection. It seems that they are a bit slow at reacting, but they are working fine. So there is not any real concern here, but they would like to follow up again when he is "healthier" to see how he does then. Other then that our last few days have been quiet as we just wait for the slow ventilator weaning process to take place.

If all goes well tomorrow will also be an exciting day because Matthew is coming to visit for a couple days. We haven't seen Matthew for over two weeks now so we are very excited to spend some time with our little guy.

Peliminary Results

2007-11-13T23:34:00.000-05:00

Monday afternoon we received the peliminary results of James' Lung Biopsy. The doctors took time to sit down with us and explain what was found and what to expect. We did not really learn anything new from these results; as we expected it just confirmed the diagnosis.

The current diagnosis:
1. LPI PAP (Pulmonary Alveolar Proteinosis)
The macrophages in James lungs do not function properly. There job is to get rid of the surfactant in his lungs, and since they cannot do this the surfactant builds up and reduces his ability to breath. Although the fluid in James' lungs is not what they call Classic PAP, it is still PAP since it is a protein based fluid building up in the air sacs of his lungs. What makes it "unclassical" is the excessive lipids mixed in the fluid and hence the reason it is called LPI PAP as they feel these lipids are cause by his LPI
2. Lipoid Pneumonia
Although they mention this as a separate item, it is really one in the same as PAP. This has to do with the excessive lipids in his lungs, which is in line with LPI PAP. Other individuals can also have this type of pneumonia which is most common in aspiration cases (hence the reason we were always fighting the battle of whether he had aspiration), but in James’ case it’s part of his LPI PAP
3. Interstitial Lung Disease/ Pneumenitis
This is inflammation of the actual lung air sacs or lung tissue.

The one good thing they were able to report was that there is no fibrosis or scarring on the tissue they took. Scarring is irreversible, so as long as scarring is not present then if we are able to keep the PAP under control, the lung will be healthy.

What does this diagnosis mean? Really it doesn't tell us anything new and it doesn't help us to treat James. There still remains no treatment, and as long as that remains, James has a terminal illness and it's only a matter of time before his lungs give up.

I must say the doctors are more optimistic then we are as they gave James a prognosis of up to two years ... of course advising that it could be more or less. Given how James was doing before all this, we have a hard time seeing this as possible. We cherish each day, knowing that any day he can leave us for a better place. If the Lord should grant James another year or so we can still pray that through new research an answer may be found during that time.

One thing that may have made him go downhill so fast is the GM-CSF treatment. We really do not know if it did or not, but there is a possibility that is actually did more damage then good. In the labs they studied his macrophages being treated with GM-CSF and without GM-CSF. What they saw was that initially the GM-CSF did help the macrophages to function, but then they quickly pooled together and created a "clump" of lipids. The macrophages that did not receive the treatment did the same thing but at a much slower pace. So they stopped the GM-CSF treatment last week, thinking that it may have been the reason for his quick decrease in lung function as it was making the lipid levels in his lung increase. This is obviously unfortunate, but that is the risk of being a trial. This too is from the Lord.

As we have seen, the WLL's do not work on James and almost seem to have the opposite effect on him. Why? We do not know.

Those are the two main treatments for common PAP, but James does not have common PAP and these treatments are not working for him. This leaves us with no known treatment and nothing we can do to help our boy get better.

There is one new treatment the doctors have implemented in order to help the inflammation of his lung air sacs (Interstitial Lung Disease). This treatment is to give him high doses of a steroid called MethylPrednisolone (10 mg/kg in comparison to the 1 mg/kg he had when on prednisone) for 3 days. This is called a Pulse Dose. For three days they "sock" the inflammation in his lungs in an attempt to shut everything down and reduce what is already there. They started this new treatment last night with the hopes that this may bring him back to his baseline breathing and allow him off the ventilator. If this treatment seems to have some effect (and no major side effects) then they will consider doing this once a month to slow things down and keep the inflammation in check.

Regardless this is not a cure. They knew he had Interstitial Lung Disease but they also know that it is not his main problem. They just feel that this may just give him that little edge he needs to improve a bit and get him out of ICU. They have told us we should see pretty instant results from this Pulse Dose if it works, so the next few days will be the tell-tale.

Post Lung Biopsy

2007-11-13T17:33:00.000-05:00

1. PICC Line. This is a central line that runs up his arm and sits right above his heart. This line is in place of an IV line and can run more fluids at a time and stay in for longer periods of time, usually 30 days, sometimes for months. This line can also be used to draw blood so that he does not need to be pricked all the time.

2. Endotracheal Tube. This is James' ventilating tube which brings the air to his lungs. This tube goes through his vocal cords and so he is unable to talk. He is also not allowed to eat or drink because this will cause choking. The most he is allow is to suck on a sponge that has been dipped in water. They can remove the orange and blue tubes which bring the air to him and then insert a small suctioning tube into the Endotracheal Tube in order to remove mucas from his lungs.

3. Arterial Line. This is like an IV line except it goes into his artery instead of his vein. Throught his line they are able to constantly monitor James' blood pressure from second to second. These lines are also used to closely monitor a patients blood gas levels to make sure they are getting enough oxygen.

4. Arterial Line Ports. Through these ports they are able to draw blood. They could also use a PICC Line to draw blood, but since James' has the arterial line they use it. They also need to use this line because they need to watch his blood gas levels closely, which is best taken from an arterial line.

5. Chest Tube Drain. This is the drain tube that they needed to insert after the biopsy in order to drain blood, fluid or air from the space between the lungs and the wall of the chest in order to allow the lungs to fully expand again. The tube is placed between the ribs and into the space between the inner lining and the outer lining of the lung. As you can well imagine this is very painful.

6. This is the two inch incision they made in order to go in and take the piece of James' lung.

7. James' g-tube. This tube goes directly into James' stomach. It was insert last August (2006) and it is through this tube that we feed James his formula. At this time this is the only way that James gets food, he does not eat anything orally (not because he's ventilated, he hasn't really eaten anything since June)

8. There are three of these purple lines. You cannot see the actual place where they are connect to James, usually they are placed on the chest area, but since he just came out of surgery they have moved them to his arm and leg. These lines monitor James' heartrate and respiratory rate. There are three stickers placed on his body and the lines are connect to the stickers in order to monitor these. The other end of the lines are connect to the computer monitor so it can "beep off" if his rates go to high or low.

9. Blood Pressure Cuff. This is just a regular blood pressure cuff that they use post-surgery or when things are not going well to confirm that the blood pressure results from the artery line are correct. The machine is programmed to automactically check every 5 or 15 minutes (or whatever they set if for at that time) so the cuff has to remain on until they feel he is stable enough to remove it.

10. SATs Probe. It's a bit hard to see the probe on the picture since it is flesh colour. This is the probe that James wears day in and day out when in hospital in order to monitor what his oxygen levels are and make sure he is getting the amount that he needs

11. Peripheral IV Line. This is just a regular IV line which usually lasts anywhere from 1-5 days. They had this IV so that they could give him medications through it. Some medications do not mix well with TPN and so they liked to have this IV to keep them seperate. Eventually the line came out and after three pokes to try put a new one in they gave up and are now just using his PICC Line.

Update Finally

2007-11-13T17:30:00.000-05:00

After a few busy days, things seem to have settled down and once again we are working towards extubation (removal of ventilating tube). Prior to the biopsy he was getting close, but since he needed the biopsy they were not going to attempt to take him off anyways. Now that everything is done our goal is to get him back to his baseline and off the ventilator.

After the biopsy James once again had a rough couple days. We were not surprised by this as he seems to have trouble anytime they monkey with his lungs. This does not mean that it was easy to once again watch him suffer so. Our biggest challenge was once again fevers, hitting 41C (105.8F) at one point, as well as dealing with high blood pressure. The fevers were further complicated by the fact that he could not have tylenol or ibprophen. James' liver function had decreased to such a point that he could no longer have tylenol and since he just came out of surgery he could not have ibprophen, which thins the blood, They had already sedated him relatively well because the drain tube that was left in after the biopsy is very painful. In the end they gave him ibprophen 12 hours after his surgery and had to put him on a cooling blanket to bring the temperature down. This did work, but in order to use it he had to be even further sedated and to keep his body from shivering (and thus rising the blood pressure more) they had to give muscle relaxants so he could not move. On top of that they had to insert two temperature probes down his nose. One would go to the monitor and the other to regulate the cooling blanket. Although this blanket did work I must say it's a pretty eerie feeling to hold a hand that is dead cold and has no muscle function. He could still hear us but had no muscles to even shake or nod his head at questions.

Saturday night they had to put in a new ventilating tube since the swelling in his throat had decrease enough that air was leaking around the tube, and his blood gas levels were not doing very good anymore. This requires lots of sedation too. So all in all he spent the weekend pumped full of sedatations.

At this time he continues to be on a high doze of morphine to diminish the discomfort of the chest tube, which they were not able to pull out as quickly as they hoped because of an air pocket that developed. Today they were finally able to turn the vacuum off the tube, and hopefully tomorrow take the tube out, and then back off the morphine significantly. It is invigorating to watch that even through this dopiness, James still makes the effort to wave goodbye to the nurses, and to point behind him and shake his head to tell us he doesn't want his breathing tube suctioned out. Every new nurse's reaction when they see James is to want to tie his hands down to prevent him from pulling out his breathing tube out, but stand amazed that even while being suctioned he does not grab at it. They say that even adults have the natural reaction to rip that tube out when suctioned, for this process induces the suffocation panic reaction.

Yesterday they finally got around to doing the calorimetry test. This was easy to do since they could just hook the machine up to his ventilating machine. From there they could get an idea of how many calories he's burning in his current state of "rest". This told them that he has already reached the maximum for his feeds and they do not need to try pump more into him. His current rate is significantly lower then when he was well. He now requires 30ml/hr around the clock, previously he would have taken 50ml/hr if fed around the clock. Since he has reached full feeds that means that he is now off of TPN.

James liver function is slowly getting better, which could very likely be because he stopped the TPN, but it that is also hard to say as stress can also cause the liver function to decrease. Even though it’s above normal we’re just thankful that each day it’s slowing going down

Reality

2007-11-11T01:59:00.001-05:00

Through all of our trials one thing we always want is for the doctors to be up front, to tell us the truth and not try to hide what's really going on. Often we have to draw our own conclusions, sometimes hoping that these conclusion are wrong, other times drawing hope from these conclusion. Several weeks ago we had come to one such conclusion, this time hoping we were wrong, but strongly feeling we were right. This past week the doctors confirmed our conclusion.

Just as we like the doctors to be upfront with us, so also we like to be up front with our readers. We've been so busy reporting on what's been happening each day and trying to keep up this past week that I haven't had time to sit down and sum things up. So I will try to do that now.

From our discussion and conversations with many of you, we realize that the tub analagy really hit home (see here). As the days progress, it is becoming more evident that James' time in the tub is coming to a close. When things went bad after the second WLL and antibiotics did not get rid of the fevers, we began to realize that there were no other options. We had tried the known options and now we were left with the unknown. The third WLL was done in hope that they would find something new in his lungs that could easily be treated, but in reality we knew it wasn't likely to happen. At the same time we realized not attempting to look again would definitely not give any further information. So we plodded ahead. While we were not expecting a cure from the third WLL, we were also not expecting him to take such a bad turn afterwards.

Along with the doctors we realized that a lung biopsy was very much necessary now. We even wonder why we didn't question to do this instead of the third WLL. Hindsight. What are we truly expecting from this biopsy? To be honest ... nothing. We are expecting a miracle if we truly believe this was going to give us something new. The doctors are quite confident that James has secondary PAP. His PAP is secondary to his LPI. Normal PAP is much easier to treat, LPI PAP is a whole new department. So in essence it is really James' LPI that is the problem here. We can speculate that James not only has PAP but also another disease which is throwing off his PAP and making it difficult to treat. But if he does have another disease it too will be rare, and likely hard to treat.

So what this lung biopsy does, is it confirms the diagnosis of LPI PAP, of which there is no cure. With it, the doctors document they have done all that they can do. Once we get the results we will know if there is anything else that we can try, or we will have to make James as comfortable as possible in his final days. If it is the latter, we pray that this will be but a short term, foremost for the sake of his suffering, but also for the burden on us as parents.

"I consider that our present sufferings are not worth comparing with the glory that will be revealed to us." Romans 8: 18

Pre-ICU Days

2007-11-09T22:23:00.001-05:00

Left: James knows how to relax. He is often found laying like this while watching TV or playing, and also when he sleeps.
Right: No nasal prongs ... we've gotten so used to him with them on he looks funny without them

Left: The daily routine of talking to Daddy, Marietta and Rebecca each evening. This time they caught him just as he was getting ready to go in the tub.
Right: James watching TV on my bed. He is holding the Teddy Bear that he received as part of the Sick Kids Foundation video they were making. They let him keep it even though he didn't cooperate

James enjoyed visitors, although during these times he did have to relearn the concept of sharing, especially with his sister where he could get rather fiesty at times.

Sunday Visit. Rob and the kids would come to visit between services on Sunday.

Lung Biopsy

2007-11-09T21:22:00.000-05:00

James went for his lung biopsy today around 1pm. So far it appears all went well from this procedure. They were able to do the procedure thorascopically, which means they did not have to cut his entire side open, but instead made a two inch incision on the left side and used a tube. They had hoped they would not need to leave a chest drain tube in since they are painful, but unfortunately it was necessary in order to prevent an air leakage and the lung from collapsing. Tomorrow they will do an x-ray (they do daily around 6am) and see how things are looking. If the lung is good and expanded they will likely remove the tube since he was not getting much drainage from it, nor was their air coming from it. Although he was not complaining, if we asked him if he hurt he would say yes and his blood pressure was high indicating he was stress ... likely from the pain. They have given him extra morphine for the night to help him through the pain, so hopefully he sleeps well. He continues to have fevers but they are unable to give him anything for it, except to pack him in ice. Due to the current situation of his liver (the levels were higher again today) he cannot have tylenol. He cannot have ibprophen because it thins the blood and he is trying to heal right now. Hopefully we don't run into the same fever problems we did after his last procedure.

As we expect James needed to have a blood transfusion last night. His levels dropped to the point where they would normally need to do a transfusion, on top of that the surgeon wanted his levels higher before he went to O.R.

Yesterday afternoon someone from the Respiratory Team came to us to find out if we would consent to them doing a BAL (also called a bronscoscopy) today. This is basically an extremely small lavage. Their reason for requesting this was to gather more fluid from his lungs for further testing. Once removed from the lungs the macrophages last about 2 weeks, so they quickly get old and any tests that they want to do should be done as soon as possible. Also the fluid they got from the last WLL had traces of fungi in it, which means that the testing could give false results. They felt that he was strong enough for this and it would work fine to do this with the biopsy. Realizing we need these in order to continue the research we consented to this procedure. So today he also had a BAL where they inserted 70ml into his lungs (in comparison to the 1-2L for the WLL's) and removed it again. He did begin to deSAT so they decide that was enough for the time being and should suffice for the current research plan.

So now we sit and wait for the results of all the various tests that have been done over the last couple days.

Last night Rob picked up the girls and brought them to our new home. They were extremely excited about this. We have not made any firm decisions on what we will do, but will likely keep them with us for the next week and see how it goes. We'll be looking into the school provided here to see how it functions. We hope to start them in the school on Monday, even if they're not learning what they should be, at least it will keep them busy and give us some free time without them. The home also has various activities at times that can keep them busy and allow us a breather. As well the hospital provides a "babysitting" service where you can drop your kids of for a couple hours and volunteers watch them, do crafts etc with them. We'll give this a try and see how we all survive. The biggest challenge is for Rob and I to get sleep somewhere inbetween all this.

Rob also picked up my computer for me yesterday ... yahh!!! So among other things, I will now be able to upload pictures ... if I can get this wireless connection to cooperate with me ... soon anyways.

Another Day

2007-11-08T13:56:00.000-05:00

We keep thinking we're at the end of all the rounds of test the doctors would like to perform, but it seems to be neverending list that keeps growing. The doctors continue to probe from all different angles in order to try narrow down and eliminate all possibilities of James' fevers and current condition.

We have not gotten the official word yet, but it appears they will be doing the lung biopsy tomorrow. His file has been marked that he cannot eat tomorrow morning, but there is no set time yet. Maybe we'll know better later on today.

Today we were introduced to the Immunology Department who is not carefully evaluating James immune system to make sure that it is fuction properly and not a problem in the equation. They are doing this by testing the B Cells and T Cells. These are the white cells that create antibodies to fight infections. They are hoping to see if James immune system is working up to par, or if this might explain the persistent fevers. Their tests require a wack of blood to be taken, not a good thing when James' hemoglobin levels are already low. If James' hemoglobin levels drop much lower they will need to do a blood transfusion, part of the blood work they order was also prep work for such a transfusion ... just in case.

After Immunology came by GI came by. They had been called back on the case becuase James' liver size has increased as well his LFT (Liver Function Test) results are high, indicating his liver is having troubles. They need to evaluate closer ... and how do they do that? You guessed it ... more bloodwork.

These are all on top of the fact that they changed the regular bloodwork from every 24 hours to every 12 hours. This change was made because James potassium levels are low, among other issues low potassium can put stress on the heart. At the rate we're going it's suprising he's lasted this long without a transfusion, and it definitely won't be surprising if he needs one after today ... even though the nurse is spacing out all the bloodwork, taking small amounts every couple hours to try prevent shocking his body.

James was suppose to have a calorimetry tests today, but when I left at noon it still had not been done. This was suppose to be done the day after he arrived in ICU, but they don't seem to be in any big hurry. This test helps them to evaluate what he needs to be fed, especially now when he does nothing all day long but lie there.

They restart James' g-tube feeds a couple days ago and we are very surprised that this is going quite well. He is only receiving 10ml/hr, but suprisingly his stomach is not extremely distended. We still need to vent him at times to ease some pressure, but he's doing pretty good.

Okay, I think that covers the main gists of the medical stuff. As many of you have probably figured out by now ... considering the fact that Rob has been here for over a week ... he has decided to drop most of his courses. He kept one course because it only requires him to be at school one day of the week and he was already done almost all the assignments, so it didn't make sense to drop it. For the time being Rob will continue to help with balancing the family life. When things settle down a bit he will consider his employment options. We settled into the Ronald MacDonald Home (RMH) last night, I will write more about that later, but we're happy with the arrangement and are considering whether it would work to have the girls stay with us ... this of course takes much evaluation since they are both in school. The RMH does have school for the children staying here, we're not sure yet if this is a option we want to take. We have to evaluate everything, also considering the high school bus the leaves this area (although we're not sure how close from here) and whether it is a reasonable way of getting the kids to school without completely wearing them out. Bringing the girls here would bring some of the family back together, but we have to consider how we'll get any sleep if we take that option. Sadly, no matter how we evaluate things we cannot fit Matthew into the picture at this time. We have now been apart from our little guy for 6 weeks. Our "lifestyle" is hardest on the girls who realize what is going on and are moved from one place to the next, but the harder part for me is not seeing my little Matthew grow up. At least I can talk to the girls everynight, and they don't change so drastically in a couple months, but Matthew is almost not my child anymore, he has settled into his home and has no idea that we are his parents. This is definitely hard for us to accept, but at the same time we take comfort in the fact that he is in good hands and doesn't know any different and is content where he is.

Staying at the RMH means that we have to spend more time apart from James. Since he cannot speak we have no idea how he is truly handling this, but when I arrived back today I seen little tears on the side of his face. I'm not sure if it's the fact that we were gone or that something happened while we were gone. He has been very sad all day, more sad then "normal". We hate leaving him as it's hard enough to stay on top of all that is going on as it is and we hate missing seeing the doctors etc (especially when it seems there are always new one showing up) and having no idea what decisions have been made. That being said I must get back to him.

Many of you have mentioned that pictures are nice to see. I have lots, but unfortunately have not been able to upload them since I have not been home for almost three weeks. When I finally brought the stuff here it somehow got lost in the packing when we moved out of the room ... hopefully soon.

Busy Days

2007-11-08T13:23:00.000-05:00

Wednesday, November 7, 2007 (for some reason this is registering as Nov 8, when it's only the 7th)

Where to start?

The last couple days have been unbelievably busy. It seemed everytime we turned around there was a new doctor, new idea, new procedure. The advantage of these days is that they do not seem near as long as just sitting in a chair, and so it's much easier to stay awake that way.

The basic rundown. On Monday they put a new ventilating tube in since they will not be extubating him anytime soon. The original tube went through his mouth and the new one goes through his nose. We're told this should be more comfortable. Neither look comfortable, but at least he can close his mouth now and keep it a bit moist.

Monday was the turning point where they stopped talking on a day to day basis and started talking more long term, admitting that he wouldn't be extubated anytime soon and that certain things had to be done first, the biggest being an Open Lung Biopsy.

They did an ultrasound of his abdomen area to make sure that they are not missing anything with regards to an infection or growths in that area that could be causing his fevers. Everything came back normal on that, except for the enlarged liver, which we already knew he had. No comments were made about an odd shaped stomach which the GI doctor seen when doing the endoscopy.

Yesterday he had a CT Scan done to prepare for the biopsy. The scan did not show anything new, just more of the same stuff. They will use this to determine the best area to take tissue from during the biopsy. The general surgeon said he thought they could do the biopsy thoroscopically by making three small incision and using small tubes to view and cut. This is much better and quicker healing then having to actual cut open his entire side.

The lab results of the fluid that came from James' lungs discovered a mild fungi in his lungs. This is not uncommon with people who have immune problems which James is said to have since his macrophages (amazing cells which literally attack foreign particles) don't function properly. Because James is ventilated they are able to do tiny (or what would be considered normal) lavages to evaluate the fluid in his lungs on a more regular basis. They are waiting for the results of the lavage they did yesterday to compare and decide whether to treat this fungi since it is showing up so mildly.

James fevers are more stable now. He has only been getting mild ones which we are able to control by medication. Whether this is because of the antibiotics or simply because his lungs have had a chance to settle down after being disturbed so badly we do not know. He has been on the antibiotics long enough that if the fevers came for the bacteria in his lungs then they should have been completely gone by now. Since they have been reduced it's possible the bacteria was creating greater fevers, but still is not the underlying cause of the fevers.

They have stopped the GM-CSF treatment feeling that this may be hindering more then helping James. GM-CSF is shown to be more effective on mild cases, and James' case is definitely not mild.

The previous discussion about lowering the cholestrol levels are on hold. The scientist had not found any evidence that this works and they do not want to start any new treatments until after they receive the results of the biopsy. We understood this to be a method of turning down the tap on the surfactant discussion. Further reading we have done shows that this could be more in line with another rare problem with the transportation of lipids (which cholestrol is) causing lipids to be "dumped" into the lungs. To build on the previous analogy of the being in the tub, this would amount to someone sneaking a garden hose into the tub, and no matter how low you turn the water down, it water level will still rise.

We still have our moments with staff, but never as bad as the one night. You can tell that we have been labelled as interferring parents and this gets past down from one nurse to the next. Thankfully they decided to move James back into isolation, (why the change of heart we don't know and really don't care). The care we received in the ward room was not one-on-one care as should be in ICU. Never mind that fact that we rather not see the nurses putting more priority in socializing and watching the basketball game on TV than their patient. At least in isolation when they leave the room I have no idea if they're doing work or personal things while gone (okay I do a bit as I can see them surfing the net etc, but even so they are better tuned to their alarms then in a noisy ward room). Pretty soon we should be through the shift of nurses we had to deal with in the ward room and hopefully have better luck with the next shift. The big thing with ICU is that they are used to dealing with the emergency at hand, which in James' case is ventilation, and then they send you off to the upstairs wards to deal with the actual medical issues. But James is not going anywhere soon and so they have to deal with the issues also. It is quite obvious that some staff are not used to parents taking part in their childs care, and see our involvement as an intrusion into their terrain. Their characters reak of insecurity. Why is it that on the upstair ward you can have say but suddenly in ICU you just have to sit by and hold your childs hand while they make decisions not always fully taking in the situation or the history?? We are glad to say that not all staff are like that, and can thank one nurse in particular for including us in the discussion when the Dr.'s did their rounds. She valued our role, and often jokingly said when we changed his diaper, or gave him his oral medication: "You're taking some of my salary"

As you may have realized we lost our room on the Ward, this happened on Monday. Thankfully we have been able to get a parent room the last couple nights. This goes on a day to day basis. Each morning we ask for our name to be put on the list and then around 7pm you can go to find out if you may stay or not. This makes it rather hard to settle in, but at least we have a quiet place to sleep and it's still better then living out of a locker (a small locker at that). We've been in touch with a social worker who helped to get us on the short list at the Ronald MacDonald house, each day we call to see if there is room available, they tell you to call as many time as you want throughout the day to keep checking ... and this just reminded me to call .... wow, that was fast, they have a room for us. We have not been there yet, but it's about a 15 minutes walk from here. We were told that it much like a regular home, we have our own room and bathroom and share the kitchen, laundry area etc. We each get our space for storing food and we can do our own cooking, as well they say it's not uncommon for people to come in and do cooking for the residents. Once we have a room it is ours until we need to leave. So we will have to pack up again and go settle in there this afternoon. It will be nice to have a place to settle into, even if it's a bit further from the hospital and makes it harder to switch at any hour so that James has one of us with him almost all of the time ... especially considering he has no concept of day and night anymore.

Good-bye Room

2007-11-05T02:06:00.000-05:00

Today we seen James' name wiped off the front board on the Ward indicating that the room is no longer his. We were told that tomorrow they will have enough staff to fill all the rooms and so they are required to fill it and we will likely have to move out in the next day or so.

We knew this would come and are surprised we managed to hang on to the room this long but that doesn't make the idea any easier.

So good-bye bed, good-bye bathroom and shower, good-bye phone line, good-bye computer (the battery only lasts 5 minutes and I have nowhere to charge it anyways ... nevermind I won't have a window to pick up the occasional wireless signal) ... most of all good-bye nice private place away from the eyes of everyone else.

It's funny how hospitals make the most stressful time of your hospital stay even more stressful by having either find accomadations ($$ in TO) or live in a waiting room, where they kindly provide you with a small locker and blankets so you can crash out on the couch ... and somehow tune out all the noise around you (definitely not going to happen during the day when it's full of people). But that's how all hospitals work and so we I guess we just have to remain thankful for the days that we managed to have some privacy and get some sleep. If we lose our we will talk to the appropriate people to first find out if there is any parent rooms available (we've seen a few, but not very many, and always with names on the door) before we decide what we'll do if not. I'm told the Ronald MacDonald Houses are also hard to get into, plus they are not exactly right next door to the hospital.

That being said our blog entries may become more sparadic as the parent resource centre where you can access the internet has terrible hours and only a few computers, but we'll try our best to remain faithful ... seeing as we also won't have a phone to be reached at anymore. The head nurse told us to cross our fingers that someone calls in sick tomorrow and they can't fill their rooms ... so our fingers are crossed.

James continues to have fevers. He had a restful morning where his numbers all looked good and his fever was gone, but as the day progressed so did the fever and as I left at midnight he was again spiking a 40C. We are now 24 hours into the antibiotics, if no change in the next 24 hours we confirm that the bacteria is not the source of the fevers ... which is our feeling already, just knowing James, but we still pray that it's simply an infection. Over the last months James oxygen requirements slowly increased and so did his temperatures ... we begin to wonder ... is this the new "norm" for James? It's a scary thought!! We pray it is not. The one positive thing about today is he required less suctioning, but I'm not sure how positive that is in light of the fact that they had to increase the amount of oxygen he's getting and the assistance they give to his lungs to keep functioning as they should.

Good night all ... zzzz

Long Night

2007-11-04T14:44:00.000-05:00

Last night was a long night for more then the reason that time changed. James deteriorated to a point we have never seen him before. He had "somewhat" (explain that later) uncontrollable fevers. We had him packed in ice but couldn't bring it down. With the rising temperature (40.9) came extremely high heart rate and high blood pressure. His respiratory rate was high, but not too extreme for James. He was working hard to breath and needed more ventilation and assistance and would often deSAT. They spent a lot of time suctioning him, but this didn't seem to help. They had to medicate him a couple times to bring his blood pressure down and finally sedated him further in order to make him rest and relax better. Lots of blood work was done and an extra x-ray, but in the end there were no answers. Eventually a shot of tylenol slowly helped to bring the temperature into a more reasonable level.

Looking at numbers is sometimes easier then looking at your child. A nurse looks at numbers and doesn't have the same special bond with the child. I found as long as I looked at the numbers I was okay, but looking at James was very difficult. He just looked completely awful, we had never seen him look so bad before. We had been telling them all day that he didn't look good, but until the actual clinical signs begin to show there's nothing to be done. And even then, what can be done when you have no idea what is going on. Yesterday they did start him on antibiotics. The lavage showed a bacteria and since it was not very strong they felt it was likely just an unsterile culture or too mild to treat. But given the current situation we have nothing to lose but to try. This is a bacteria that should have been killed by the previous antibiotics, so I'm not sure how to explain why it was found.

As if dealing with James last night wasn't enough we also had to deal with the most awful nursing staff we've ever seen. I think this in itself is what made the night so long and awful. Right from the beginning of the shift it was battles with a nurse who thought she knew everything and within two hours could tell me more about my child then I had learned in the last 2 3/4 years. I'm not sure what I would have done if I heard the words "hospital policy" one more time. After a few smaller battles she then basically refusing to give him tylenol because hospital policy and pharmacy says you should have one or the other and ibprophen seems more effective for him. This after the child has been receiving both for days (and even prior to his ICU admission) in order to attempt to control his high fevers. I figured I had to chose my battles and it almost being time for his ibprophen I'd bid the time and pack him with ice instead. And this is why I say he had "somewhat" uncontrollable fevers. Finally at 3 am everyone was in a panic because they couldn't get him stabalized so they decided to try some tylenol ... and wouldn't you know, in the next hour his fever slowly came down. Still a fever, but much more reasonable. It's one thing if it's one nurse you're doing battle with, but there were two, the nurse and the head nurse. I felt sorry for the training nurse who felt stuck in the middle. And then you can see them whispering away everytime they leave the bedside. There were several other issues we had to deal with, plus their attitude of thinking they needed to teach me how to do everything. I won't bother getting into them because I still get rather worked up talking about it and after several almost yelling matches last night I rather put it behind me. This morning we talked to the charge doctor about what had happened overnight, not surprising he knew immediately who we were talking about when we told him who are chief complaint was against and said he would deal with the issues immediately. Oh ... and there's no hospital policy about tylenol and ibprophen, it's the doctors call ... like I didn't know that!

Thankfully by this morning James fever was gone and with the extra sedation he was able to rest nicely finally. He looks better, but his eyes tell us alot and considering he's had them closed all morning sleeping it's hard to really say. Either way it doesn't mean we're at the end of this road as we've had the fever come down before just to respike, and as I left for lunch his temp was climbing again. We have no idea in the end why the temp finally came down, we just pray it won't skyrocket again.

Needless to say I got little to no sleep last night so I will try now.

Still in ICU

2007-11-03T15:03:00.000-05:00

James x-ray looked a little better again this morning and his oxygen requirements remain the same, but he continues to need an awful lot of suctioning. With that in mind they will be leaving him on the ventilator another day and will reevaluate tomorrow.

James is looking worse today then he has the last few days. We are uncertain why this is. It could simply be that his body can no longer take the toll of the continous high fevers that we are fighting to control. It could also be that he is feeling unwell because they are sort of trying to dehydrate him. Usually they calculate what goes in and what comes out of him each day and want to see a positive balance. They are now looking for a negative balance, where more goes out then comes in. Since he is not doing this well on his own he has been receiving medication to help him pee more. By doing this they hope to remove as much fluid as possible from his body, especially his lungs, but at the same time monitor closely to ensure that he does not actually become really dehydrated.

James almost has a look like he's given up. He is so patient and good as they suction him, enduring everything without a fuss, but to some point it's like there's no fight left in him. I noticed this yesterday when they got him ready for transport to have the PICC Line put in. Usually once they start getting all the portable stuff out he gets worked up and anxious about where he's going and what's going to happen. Of course now he cannot talk to express himself, but he didn't even shake his head no or show any concern. Again today when we were moved to a new room he just sat there with no indication that he cared what was going on. He's a real trooper and everyone has told us how they've never had such a good kid before, especially for suctions. We just hope that he can soon have the freedom to talk and breath on his own again.

We lost our nice private ICU room today. James was in an isolation room, but someone else with a greater isolation need required his room and so they transferred him to a ward as his isolation is just contact isolation due to his previous c.diff. This means that they have to wear gowns and gloves (which they don't do anyways) when working with him, but that he does not have an airborne issue that could spread to others, therefore making it okay to put him with others. I suppose we've enjoyed quite the luxury so far, so we can't complain, especially considering we still have our room on the floor and a nice private place to sleep and shower. The biggest downside to being in a ICU Ward Room is that when the nursees do shift change we have to leave the room for patient confidentiality reasons. Thankfully James does not get too worked up if we leave him, so hopefully this won't stress him out too much.

A Bit of Light

2007-11-02T17:44:00.000-05:00

James will be remaining in ICU overnight, possibly for the weekend. His x-ray is looking better and his oxygen requirements are back to where they were before the procedure but he is still working hard at these levels and needs frequent suctioning. They could take him off the ventilator, but then they would not be able to suction out the secretions and there is fear that he may not cough it out well enough on his own. They also would not be surprised if he had to go on BiPAP (see here) if they took him off now. So it only makes sense to keep him on as long as possible to help clear out his lungs as best as we can or we may end up dealing with pneumonia as well.

The ICU Team strongly discouraged the idea of doing a Lung Biopsy at this time and the Respiratory Team has listened and decided to wait until James is back to "normal" - as in, how he was before this procedure. We were glad to hear this, but still wondered why the sudden rush and change in plans. The doctor came to speak to us this morning and helped to clarify some of this. They thought that since he was already intubated it might as well be done right away as it is going to be needed. I'm guessing they also thought that he would have recuperated more by now. Their plan had been to do a CT Scan and the Biopsy so they could take advantage of his intubation. They did not realize that he does his CT Scans unsedated. They CT Scan has also been put on hold until he is back to "normal". If done today it would have given false indications as he still has far too much fluid in his lungs that is still related to the procedure and the fact that he is retaining his fluids and not peeing it out on his own. So it seems the biggest rush was for convenienced but they realized their mistake in this area.

We were still wondering why the sudden change in plans as there had been no talk of a Biopsy before. It seems that Wednesdays procedures did at least help us to end their ideas of aspiration pneumonia. Given the amount of substance in his lungs, if this was aspiration there should have been more then just mild esophagitis, what they saw did not support a case of aspiration. This combined with our argument that we seen no improvement in James' condition when he was last on a J-tube (feeding tube inserted into the intestines and so avoids the use of the stomach, therefore preventing food from coming back up from the stomach) seems to have made the realize that a simple explanation of aspiration is not the answer. We are thankful that we do not have to go through the same battles again of convincing that it is something else. It would be so nice if it were just a simple answer like aspiration and all that was needed was a j-tube, but we have no desire to waste time on what we are confident is a dead-end road.

So unfortunately the question remains: what are we dealing with? And even more unfortunate is the fact that there is no answer and no idea as to what the answer is. We are even back to questioning if this is indeed PAP. Previous lavages indicated that this is PAP but the last lavage test came back as a weak indication of PAP. So does that mean his PAP has improved with treatment and there is something else ALSO effecting his lungs? or are we dealing with some completely new rare disease that has similarities to PAP but is not actually PAP? So it seems we are back at square one and are starting all over again and they feel that the only way we're going to get some clearer answers is to attempt a Biopsy. This will not necessarily give THE answer, but it will help to better indicate if we are dealing with PAP or not and gives a better indication of the cell fuctions.

When it comes to our current battles of James' feeds we have also won that battle, although it did take some time and several vomits before we got through to them that they could not feed him at this time. He is currently in procedure of having PICC Line (a central IV line that goes in the arm and up to the heart which can stay in for about a month sometimes longer) put in. Once that has been put in they will be putting him on TPN (IV nutrition). We are glad that he is finally getting the PICC and we don't have ot keep fighting for it. He was scheduled to go in at 3:30 and we kissed him good-bye as they took him in and then head outside for a walk and to get a few groceries etc. We came back an hour later when they were suppose to be done to be told by the nurses on his old floor that they have been looking for us as they forgot to get us to sign the consent form beforehand and could not do the procedure without it. We rushed downstairs just in time to find them coming out of the unit and heading back to the room with him. Boy were they glad to see us and boy were we glad when we heard that they were still able to proceed ... pretty good considering they had to beg to get him in today in the first place. They had even called us on the overhead speakers, but that didn't do a whole lot of good since we were not in the building. Just a reminder of why I never leave when they are doing a procedure, no matter how small it may be considered.

That being said I must get back as they should be done by now.

Darkness

2007-11-02T07:14:00.000-05:00

It seems we are once again being left in the dark ... for some reason doctors seem to like to do this when they're dealing with unknowns and uncertainties

We had a really good nurse yesterday who made sure Rob was able to listen and ask questions during rounds (they do it outside the room and since the door is closed you may miss that they are there).

During rounds it came up that they may not extubate (remove the ventilation tubes) todoay as there is talk of doing an Open Lung Biopsy today. This is news to us and had Rob not been there it would appear as though they weren't planning to tell us anytime soon ... maybe like two minutes before the procedure so we could sign the consent forms!?!?

Unfortunately I do not have internet service right now (this will just be left in the window until a connection picks up and posts this). This means I am unable to provide a link to further explain what this is. This also means that I am unable to do my own research at this time, and since it has been months since this was last brought up it would be really nice to read on it. Hopefully we'll get a chance before they make up their mind. If you are interested you can read more in it by typing Open Lung Biopsy in the search line of the blog as I know this has previous been discussed on the blog.

Off the top of my head: This is not a small procedure when it comes to talking about biopsies, otherwise this would have been done long ago. The procedure involved cutting through the side of his chest to access the lung from the outside. I do not remember why it is that they need outside access, I just remember the procedure was not nice and carried risks, the biggest including lung collapse.

We are not against having this done and recently I've been thinking we should start asking why this is not being considered, given the complexity of his sutation. At the same time we are thankful to see this has not been quickly push for. A biopsy can give much better information and answers. But I'm guessing it's like most tests - if you don't know what you're looking for and you don't order the exact tests to find what you are looking for the answer could still be missed.

Our issue now is the fact that we aren't being told what's going on and the fact that we don't feel he is strong enough for this at this point. The ICU doctor seemed somewhat in agreement with us on this one and said they (the Respiratory Doctors) would have to have some pretty strong reason before they would allow it to go ahead. Our biggest questions are why now? why suddenly two days after his procedure? why was this not done two days ago instead of another lavage? have they found something to warrant this? or did they not find anything and the procedure left them so confused they feel it nessecary? being Lung Specialist they should realize the state of his lungs right now, so why do they think this is necessary at this point instead of letting him recuperate better?

We have a feeling this won't happen today seeing as ICU wasn't totally in favour of the idea, but it has kept our senses up and made us wonder what the talk is behind the scenes. Why do they keep trying to give us such positive information and not tell us what they're really thinking?

Well I'm just on my way down after my shift of snoozing, so we'll see what the morning brings. James is doing as well as can be expected. Thankfully we are able to get his fever under control last night. Tylenol and Ibprophen were not working and so they had him packed in ice most of yesterday. I was freezing just sitting in the room because of the vent above the bed, and here I had to look at this little guy surrounded by ice ... poor thing.

I will leave of at that and will update whenever possible. If the Open Lung Biopsy is not done the hope still is that he will be extubated today.

Another Night

2007-11-01T13:19:00.000-05:00

Yesterday afternoon they took James off the aneasthetic and put him on morphine to relieve any pain and keep him somewhat sedated. We were a bit concerned about how he would react to the tubes once he was aware of his surrounds, but typical James style ... he is doing really well.

Most of yesterday they kept his hands restrained but at night I was able to release one hand and he did not pull at the tube. Today he has both hands free and is being very good. Because of the excessive amount of fluid and secretion in his lungs he is needing frequent suctions (where they put a suction tube down his ventilating tube in order to remove any blockage and lose secretion from coughing). Yesterday he was quite upset each time they did this and would bite on his ventilating tube to try stop them from putting the suction tube down. Today he has come to accept this procedure but his eye tell us he does not like it any better then before. As he cannot talk it is so very hard for us to know what is going through his mind. His eyes look so sad it is painful to look at him at times, but yet he lays there so patiently and is so good. I want so badly to just pick him up and hold him, but we cannot.

We took turns sitting with him last night so that someone was always there to comfort him. Once again we are thankful for the room we have on the ward where we can go for quiet and sleep. They have decided to keep James in ICU at least one more night, so we're not sure how much longer that room will be available to us.

Because of James' metabolic issue they are pushing to have him back on his feeding tube again and started it very slowly this morning. We are not impressed with this idea and will fight a bit harder to have it stopped. His belly is so distended from all the air that is being pumped into him and we have had his tube open most of the time and mucas and phlegm continues to flow out of the tube, especially with each coughing fit. Now he will not be able to release that air and at the same time we are pumping food in. If the stomach is distended it puts pressure on the lungs, making it more difficult to breath ... why they can't see that simple fact is beyond me. It's funny how they feel just because they have a way to push food in they need to. Any normal person would have been put on TPN (IV nutrition) as they would not be able to stuff food into them when they have a ventilator.

This mornings x-ray looked worse then yesterdays (they took several throughtout the day). They tell us this is normal, that x-rays are usually behind in showing the inflammation so they are not suprised by this. Clinically he is improving and they have been able to wean him slowly. James is also not peeing and needs medication to do so, which means he is retaining fluid and this can also effect the lungs and what the x-ray looks like. This on top of how much was left in his lungs yesterday. Nurses can sometimes help you to get a clearer picture of things. For example yesterdays nurse was interested in what exactly they were trying to do with James. When I told her they had left 440ml of saline water in his lungs she was absolutely stunned, she had no idea that much had been left in and suddenly began to understand why he needed suction so often. She then explained that when they do a small BAL (lavage) in the ICU they put in 1ml per kg. This would mean in James they would only put in 11ml and then withdraw what they can ... and here were left 440ml in.

They think that James could possibly come of the ventilator tonight, but will be leaving him until tomorrow morning. They do not want to remove it too soon as they would then have to put him on BiPAP (see here for pictures) ... and we definitely don't want to put him through that again. As well once they remove the ventilator they can no longer suction him, and the more we can suction the better. So as it stands nwo we will be spending another night in ICU.

We got the results of James' weekend stool test today ... one day too late. He no longer has C.Diff. If we had had those results yesterday they would have been able to do a colonoscopy and better look at his colitis issues.

Thanks you all for your continued support.

Philippians 4:13 "I can do everything through him who gives me strength"

ICU

2007-10-31T15:05:00.000-05:00

As I know you are waiting to hear how things went today I am hear to convey only what we know at this time, which doesn't seem to be a whole lot.

We went into this Lavage with a different perspective then the last one. Last time we were hoping for a cure, this time we were hoping for some answers. So with that in mind it's hard to say whether there was any sucess. Only time will tell us whether there are any answers, right now we just pray for stability in James' condition.

As we were told, this Lavage involved more risk then the last ones. In the end they had to abort the procedure because James was deSATing and no longer stable. They put 1100 ml in and were only able to get 60% of it out, leaving 440ml in his lungs. From what we were told they got the same milky substance out of his lungs as previously, but it was thicker then the last lavages. It's hard to say if this is for medical reasons or because of the technique they were using this time. What they did remove from his lungs has been sent off for further testing, this time also looking at the lipids (fats) to try help determine the source of these. Once again we are told that the substance removed from his lungs does not have the "classic" appearance of PAP. This is not to say they do not believe he has PAP, we are confident he does, we are just unsure what else is going on in his lungs and how it all works together, if it does.

The endoscopy was also done and we were disappointed to hear that they did not find anything abnormal. I suppose that's not the right thing to say. It's good to hear that things appear normal, but unfortunately there was not an excess amount of mucas as we have been seeing, so we may not be getting any answers. They did do biopsies and there is always the hope that this will provide some answers.. We were told that he only appeared to have very mild esophagitis (inflammation in the esophagus) which we would expect to be worse if he indeed had aspiration or even given his daily vomitting episodes. The one thing they did note was that his stomach was oddly shaped and so they would like an ultrasound done to look at the surrounding body parts to see if that explains the different shape. This could possibly just be because of his enlarged liver and spleen and they don't really see it as a concern.

They were unable to extubate James and so he was moved to ICU to be closer monitored and extubated (remove the ventilation tubes) when ready. Upon first seeing him he appeared very relaxed, calm and comfortable ... much better then the time I seen him at Mac that way. This did not last ...we were just going to head out to get some lunch when all that changed. He began to cough and in turned deSATed badly and he would wake and fight the tubes. It seemed something was blocking his tube and they worked hard to suction and stabalize him for some time, but his CO2 levels remained unstable. It's amazing to see the quick response when needed. At one point the nurse simply called out one name "Anne-Marie" and within seconds five people came flooding in the room, and then one says "Anne-Marie's on her way" Two more people arrived before Anne-Marie (the doctor) did. You get this feeling like you're watching a TV show as they all surround the bed and you're off to the side watching and unable to do anything. But with time they were able to clear his airway and stabalize him again and so far he's remained that way. Once again they were shocked at how much profofol (aneasthetic) was needed to keep James asleep, he kept waking and they kept giving him shots of it and slowly increasing the consistant dosage. The doctor said we (as in normal adults) would be sleeping sounding on this amount. That's our James.

So at this time he is once again "stable" and we have to play the waiting game again. In situations like this there is no way to estimate how long it will take before they can extubate him. Patience is required!

Mind Overload

2007-10-30T21:11:00.000-05:00

Well it seems my mind isn't functioning properly anymore after the doctors threw a new loop into things this morning, the sum of which brings no new information; it just revisits old information and brings back those old feelings of this summer ... of having to review and review all that happens and research and research and question and question, not simply because there are no answers, but more the issue of not being confident in the answers the doctors are giving ... and maybe things just won’t add up. Nothing makes any sense, everyone is stumped ... I feel like we're reliving the 5 weeks we spent at Mac this summer. There was such relief when we got answers when we first came here, like we were finally moving forward and could finally focus on the issue at hand. That has all been taken away again. I suppose it's been obvious for the last two weeks that there's more to this than we original thought, but there was always that small hope of the last few setbacks being resolvable.

This morning the Lung Specialist doing the procedure came in to say that after much thought yesterday he's not completely confident that PAP is the issue here. Further results from the previous lavage have come in, wrenching uncertainty into everything since these results are not supporting PAP to be the sole issue. This new information points to aspiration pneumonia (when food comes up and then goes into the lungs…see here and here for past discussions on this). We've been down this road before when they insisted James' problems were aspiration pneumonia and would not acknowledge results supporting PAP. (Philosophy buffs will note the fallacy of a false dichotomy). Although they are prepared to do another cleaning tomorrow, there is a possibility that after first viewing the lung with their camera, they may decide a lavage is not necessary and just take samples for further testing instead. This suspicion of aspiration has induced the talk of once again putting a j-tube back in, since this can help eliminate aspiration and help determine if this is an issue. Our notes on when the last one was in do not support their ideas at this time, but we'll deal more with that if the issue seriously arises.

The biggest issue that arises from this new thought is that since there isn’t a clear way to diagnose aspiration, it's mostly a guessing game involving trial and error. There are several tests that can be performed:
PH Probe:
o This involves a probe sitting in his esophagus for 24 hours, recording acid levels.
o previously done showing some reflux
Upper GI Series:
o a series of x-rays on stomach, capturing the operation of the stomach and its contents when food enters it
o this test has not been done
Swallow Test:
o A series of x-rays on his throat, capturing the operation of that little valve on the windpipe, to see if food leaks into the windpipe while swallowing.
o This was previously done, but showed nothing abnormal).

These can give an indication but are not very accurate at diagnosing, because he may not actually aspirate during the time the test is running. It’s like taking a picture of a family. They are all smiling when the shot is taken, but those smiles can be long gone before they leave the studio. We do know that James has at least a mild form of reflux (when food comes back up…think of persistent heart burn) or even possibly aspiration, but there have never been enough signs to indicated that it is a serious problem. I will not bother going any further in depth about all the arguments that can support or not support their thoughts; we'll wait to see what tomorrow brings.

Once again the doctors are thoroughly stumped and have no idea what to do or how to proceed. I think the big hope is that some magical answer will come from tomorrow’s procedures. The GI department heeded the final boarding call and is now adrift with everyone else, and will be performing an endoscopy tomorrow. However, due to James' C.Diff they do not feel a colonoscopy will give accurate information at this time, so it will have to wait until another time. With regards to the PICC Line, we are told it cannot be coordinated with the other procedures, likely it is performed in another section of the hospital, whatever the case at this time we're no longer sure if it's needed as we have stopped antibiotics and have no idea what we're dealing with anymore.

So although these are just their thoughts it gets our minds running again, going back over all the information we went through before. All the time we were at Mac and they tried to convince us this was his problem we could not agree with them and felt like we were constantly fighting with their one track thoughts. I hope that we don't find ourselves stuck in such a position again as there's no place to go anymore to ask for a second opinion. At the same time, we take great comfort knowing that we are at the best place we can be, and we can truly see the difference of Mac and Sick Kids. Sick Kids is a place where they truly want to learn, to understand, to research and try put the puzzles together. We did not get that feeling at Mac; there we felt like we were the ones who had to do the research and pushing for tests.

Today was a busy day, right from the moment I woke up ... boy do days like that fly by so much faster. I had a pleasant visit today with friends, but especially with a little guy named Matthew. James' home-nurse was one of the ones who came today and since she knows him fairly well I could leave James in her capable hands for awhile and go out for a walk and shopping with Matthew. Unfortunately I couldn't be gone long as we had a flood of doctors all morning and I was waiting for more for the afternoon. On top of that my mind was also busy thinking on a phone call Rob received this morning from the Faculty Office. It seems they have taken note of his situation and have offered that should he need to drop out, and with the proper medical documentation, they will refund him (usually you can only be refunded if you drop out within the first two weeks) with no consequences on his transcript. This leaves much to think about. He is 2/3 of the way through the semester, but is unable to fully concentrate and so his marks are suffering. It becomes a question of muddling through the next month or dropping out for this term, or this year and coming back next September. (Rob's editorial note here: If in September, when we debated what direction to go, if we knew then what we know now, the answer would have been obvious. But now, after putting all this work and effort into it, the scale isn't so clearly off balanced. Is it pride, or ambition, or the opposite; stewardship that desires to plod on. This reminds me of what a pilot told me when I asked if a twin engine plane could fly on a single engine. His reply was clear: "The remaining engine will take the plane to the crash site." If it is pride, I plod on to my own undoing. If it is stewardship, is the eject button ever not an option?)

And so I end another long-winded entry. We pray the Lord will guide the hands of the doctors tomorrow and most of all we continue to pray for answers. Maybe someday our minds will be able to rest from the constant medical information flowing through it, and find the mundane details of life refreshing.

O.R. Time

2007-10-29T19:46:00.000-05:00

We have gotten O.R. time for Wednesday morning at 8 a.m. The plan is to do the right lung on that day.

GI did not show up today ... what a surprise. The nurse even paged and pestered them at the end of the day, but still no luck. At this rate there is not much hope of having the endscopy done on Wednesday.

The Revised Plan

2007-10-29T12:44:00.000-05:00

Monday mornings the entire team of Lung Specialists gather together to dicuss all inpatients so that all the doctors know what is going on. From what I'm told there was a rather lengthy discussion as to what to do with Mr. James.

48 Hours after starting back on the Gentamicin James is still having fevers. Last night and this morning he spiked high ones (40.6C/105.1F) immediately after vomitting. Unfortunately there's still no answers. The Gentamicin isn't working so that could mean these fevers are not infection related. At the same time there's a possibility he's built some resistance to the Gentamicin because he was just on it. So we still have are no further on putting the puzzle together.

The new plan is to stop all antibiotics. James technically should have been done the other antibiotic (Tazocin) yesterday but they continued it given the fevers and restart of Gentamicin. Since we're still dealing with fevers they've decided to just stop all antibiotics. The next move is a WLL. They hope to gain some insight from doing this as well as assist James by once again cleaning his lungs. They feel their tests will be better if he is not on antibiotics which many cover up things that are going on in the lungs.

This time when they do the WLL they plan to do it differently, going back to their original plan when he first arrived in Emerge over a month ago. (Click here to read the difference betweent he two techniques). All the talk we have heard has always indicated that they were quite happy and excited about the technnique they used last time, although as time went on they did admit that they didn't get a full seal when they went in to do the right lung. I have no idea if this could be a factor in todays problems as we aren't always told everything (information tends to slowly leak out here and there). When they came up with the technique they used last time they told us it would be less risky then their original plan. Since we're now going back to the original plan that also means there are more risks to this procedure as this technique makes it harder to keep the lung stable while washing. The only good side to this procedure is that it has been done several times by a collegue in France so they have more literature to support their plan. We realize that often we are kept in the dark on the greater details of these procedures. Before there was any plans for such a procedure they quite openly told us how risky it was, but when the time came to actually do the procedure they tried as best as possible to downplay everything.

Since we are stopping the antibiotics the PICC Line has been put on hold for the time being. They would like to get the results from the lavage before they decide if it is necessary, although there is feeling that it will still be ... likely to give long term antibiotics. Someone still came by today to go over the details of the PICC so that we are ready to go if one is needed.

We are waiting for GI to show their face. Since our original visit with them we have not seen them back ... maybe this is just the way GI people work since we had the same issues at Mac. We have finally gotten the Respiratory Team to realize that we have greater issues in James stomach then just swallowing a bit of phlegm from his lungs. James continues to ask to have the bubbles (air/gas) out of his stomach and has been quite uncomfortable the last few days. I am unable to get air out because all that comes out of his stomach is phlegm ... usually more phlegm them formula. Where did the formula go if all I'm getting out is phlegm? Yesterday I showed them what I had withdrawn from his G-tube just before they arrived. I had about 20ml of phlegm with a few streaks of formula in it. They were quite in agreement that he couldn't be swallowing that much from his lungs and something else is going on, but they have absolutely no idea what. PAP is an issue of the macrophages not working in the lungs ... but marcrophages are in other parts of the body as well and we wonder how they are functioning in those parts. We have requested an endoscopy (when they look inside the stomach) done while James has the lavage. We're not sure if this will really give any answers, but feel we won't get anywhere until they have a look. The Team is in agreement but GI is the one who has to put in the orders and do it, so we're waiting!!

So for the time being we try to control the fevers and wait for OR time, hoping that both the lavage and endoscopy can be coordinated for the same time in order to avoid additional aneasthetics.

Miscommunication

2007-10-27T12:54:00.000-05:00

Well it seems there was some miscommunication between doctors and nurses yesterday. The orders were suppose to be that if James spiked another fever, which he did regularly each time the ibprophen wore off, they were suppose to order a blood culture and start him back on the Gentamicin antibiotic. Since I was not aware that this was the plan I couldn't remind them of it either. So these things did not happen until this morning. Early this morning James spiked a high fever of 40.2 (104.4) which meant they needed to call the resident. Thankfully we were able to get his fever down without any seizures (James has had temps in the the 41's/106 area before and has also had febrile seizures several times).

There is much regret from us as well as the doctors that we took James off the Gentamicin before the full 10 days that they originally planned. The doctors explained that 5 days is normal and when Infectious Control became in involved they felt we should just go the 5 days, whereas the Respiratory Team felt that the 10 days would be better due to the troubles they were having getting the infection under control and given the experience they have with other children who have respiratory problems. Whatever the arguments were, in the end Infectious Control won out ... likely due to the dangerous side effects that Gentamicin can have if given for a longer period of time. Had we just waited out the 10 days originally we would have given the medication ample time to prove itself. But now we're worse off because we have to start over and this time they want to give it for a longer term, likey 14 days. So in the end we haven't avoid the risks that come from longer use of the medication ... unless of course, stopping the Gentamicin is not the reason for the return of the fevers.

This plan is not written in stone though. Since the fever has resurfaced they have to once again go through the ruling out process of doing urine and stool tests, nose swabs and blood work. We're not really expecting anything from these, but they have to be done anyways. There is also the fact that the bigger plan still is to move forward to doing WLL's again with the hope this will provide some better answers, which in turn can change the antibiotic plan.

But with two more weeks of antibiotics in the plan we can expect we won't being going anywhere for at least two weeks, not that we really were expecting to anyways. We are now pushing to have PICC (a central line that runs up his arm and sits by his heart) put in, which the doctors are in agreement with now. Last time they said to wait as we could be done and home in a week. We weren't all to confident of this at that time, but the doctors get the final say. This time they agree with us ... altho techincally we'd like a PORT, but that comes with more chances of complication. We knew they wouldn't agree to a PORT, so we expected we'd have to settle for a PICC. Since this procedure involved having an aneasthetic it cannot be done on the weekend, but they will start the ball rolling on Monday ... hopefully it can be done before his IV needs to be replaced again and we have to try find a vein again. Not only does a PICC mean we won't have to search for places to put IV lines all the time (several of them have ended up in his feet, meaning he can't walk during those times), but they can also do bloodwork from the PICC Line so he won't have to be poked and prodded each time they have to do bloodwork, which they seem to love doing ... it a wonder the guy has any blood left. A PICC line lasts around 30 days, although there are cases where it lasts longer. The downside of a PICC is the fact that it comes with the risks of getting infected and whenever a fever is present they then have to wonder if it's the PICC and whether it needs to be pulled ... this is there biggest reason for not wanting to put a PORT in, the longer a central line is in the more risk of infection and James' has too many unexplained fevers. So these can both make life easier and more difficult.

Today James seems more effected by the fevers ... or whatever it is that is bothering him. Yesterday he was fine as long as he had the ibprophen in him, but today he's more miserable, has less energy and he has to work harder on his breathing. They did an x-ray yesterday but it didn't show any specific or obvious difference from the last x-ray taken over a week ago. Our poor strong little guy who keeps asking to go home. Whenever we have to give him something or do something we explain it's to help him get better and his reply is "doctors help me get better, doctors help me go home". If only we could have as much faith as he does in the doctors, but we know who to put our faith in truly and fully, the words would have so much more meaning and depth if they were "God help me get better, God help me go home".

What now?

2007-10-26T18:15:00.000-05:00

6:00 a.m. James spiked a fever…

"Do not be anxious about anything, but in everything, by prayer and petition, with thanksgiving, present your request to God. And the peace of God, which transcends all understanding, will guard your hearts and your minds in Christ Jesus. Philippians 4: 6, 7

After six days of being fever free, we have taken another step backwards…a very big, heart aching step backwards. After a couple days of being able to tolerate slightly lower oxygen levels of around 1.5L, we're back up to the 2-2.5L area.

The question if whether this came because we dropped the Gentamicin medication on Wednesday arises. It's very well possible, but we won't ever know. The initial plan had been to administer Gentamicin for 10 days, but after some discussion between the Team and Infectious Control it was decided to change this to 5 days, which they say is still a normal treatment length for this medication. I don't know all the reasoning behind why they dropped it, for they did blood work often enough that there shouldn't have been an issue with the levels (Gentamicin is difficult on the kidney's). Maybe five more days would have done the trick, maybe the same thing would have happened at the end of ten days, maybe the fevers aren't from infection after all ... we really have no idea what we're dealing with. If only they had kept him on the Gentamicin, we wouldn’t face the as many question marks as we do now.

If only….

Whatever the reason, it's done now and we're back to fevers.

Yesterday we were told we would wait for the full 10 days of his remaining antibiotic to be completed (Sunday) and then wait to see if the fevers reappear. The idea was to be confident that we had gotten rid of the infection we assume he has. Once they’re confident that we're rid of that, they would be better able to evaluate and determine what's going on in his lungs to cause the increased oxygen levels again. So they admitted that they don't really think an infection is the issue here, they just want that out of the way before they try get to the heart of the issue. With this morning’s fever, everything changes again and now they have decided to go ahead and have a closer look at what's going on in his lungs. Originally I had thought they were thinking along the lines of doing a bronchoscopy – (a common procedure used to look at the lungs, do small biopsies, test fluids in the lungs, etc). But they have advised they are thinking of doing a WLL lavage again. They want to get more samples to test for infections again, and they feel that if they are going to go in, they might as well go in completely and do a “therapeutic" treatment as they called it. In some ways this seems logical, as a bronchoscopy isn't really going to help, it's just going to give them a chance to look, whereas they in the WLL they can look and treat at the same time. But we can't ignore the risks involved with a WLL. We've had two "successful" lavages in that we had no unexpected emergencies, but that doesn't mean the dangers are not there. At the same time since we're again at a (never ending) dead end road, there really isn't any other choices.

The words of Team Doctor when he left today were "we're trying". Those words were probably meant to encourage, but it also was said in such a way that it felt like they were admitting that we're really out of options, but we'll keep going as long as we have the opportunity to. More and more so lately I've been feeling the same way. Is this a losing battle? The WLL did seem to work, but hours later everything turned around. So maybe it's possible a WLL will work, but if his PAP is going to return so quickly and drastically anytime he has an infection then what hope do we have? I know the LORD can make anything possible, and we, with many others, keep praying that some day James will come home with healthier lungs. But at the same time we are fully aware that the LORD’s ways are higher than our ways, and He will answer our prayers, even if it is not how we would like them to be answered. We are prepared to accept that James may never come home, or if he does it may only be after they have said there is no more they can do for him. These are not easy things to accept, but they are not unrealistic.

This is not to say the doctors have truly given up though. I spoke to my Genetic Doctor yesterday who was very impressed with the work that has been going here, and with what the scientists are trying to do to learn more about PAP and how to treat it. They are currently experimenting in the lab how a cholesterol reducing medicine might inhibit the production of surfactant (the fluid which they washed out of James’ lungs), and from there they will decide whether to try it on James or not. How this will be administered, how long it will take, side effects, etc. we don’t know yet, since we have not sat down to thoroughly discuss it.

To understand what they are trying to achieve by the GM-CSF treatment and the cholesterol reducing treatment, think of what happens when taking a shower. You place a strainer in the drain so that a little bit of water stays in the bottom of the tub to keep your feet warm, but no more than that. But after a while, hair clogs the drain, and the water level rises. So you administer drain-o (GM-CSF) to help get rid of the water. This doesn’t work, so you reach down and lift the strainer so that all the water flushes away (WLL). But now the water is rising again, so you close the tap a little (cholesterol reducing medicine), and hopefully you’ll reach homeostasis. Whether James will ever reach that or not, well, that has yet to be seen. If it doesn’t, God will take him out of the tub, safely in His arms.

After all, He will take all of us out eventually.

However, for the majority it’s not until their skin is all wrinkly.

"So do not fear, for I am with you; do not be dismayed, for I am your God. I will strengthen you and help you; I will uphold you with my righteous right hand." Isaiah 41:10

Same Old

2007-10-24T11:04:00.000-05:00

So it seems that I'm due for an update, but there's really nothing new to tell.

There hasn't really been any change since my last report. James is still fever free but we still are not seeing any changes in his oxygen levels. He has started on antibiotics for his c.diff. He is having some pain and discomfort from the c.diff ... or I'm assuming that's what's causing it. He had similar discomfort last time when they discovered he had c.diff and after a couple days of medication it settled down, so we'll hope for the same effect this time.

Monday night we started the GM-CSF inhaled treatment again. So far the reports from the lab are saying that this seems to be the only effective medication and so we hope this will encourage his macrophages to work again and in turn decrease his current oxygen needs.

Other then that it's just a waiting game. Originally they said they wanted to give the medication a week, but now it seems like they're going to wait out the full 10 days before making any decisions. Sometimes it is frustrating to sit around and wait for something that does not seem to be working anyways, but at the same time we remind ourselves of how different James is, how he never follows the books and how unknown his disease is. This reminds us to try and be patient as we would hate to rush into doing unnecessary procedures if time and patience is all that is needed.

Oh, and in case you noticed that the posts are being done in my name afterall and not Rob's ... I had a chance to look around blogger and found that I could set up it up so I could just send the post directly to the blog via e-mail. I remembered seeing it before and thinking "Why would I need that? If I can send e-mail then I have internet connection and can just post directly on the blog." Well I discovered that's not always the case and it's come in handy.

Half Minute of Fame

2007-10-22T16:42:00.000-05:00

James decided to turn down his opportunity for 1/2 a minute of fame today.

James was referred to Public Affairs as a child for their current SickKids Foundation video, which can also be used for other public affairs, including on posters, webpages, etc. They were just doing short little clips of kids smiling while receiving a SickKids Teddy. Unfortunately James paniced when they came in the room ... "no pictures, no pictures" ... which turned into deSATing and then almost throwing up. I couldn't get him to settle or convince him it was okay, so that was it for his chance of fame.

I asked the nurse how a child was "referred' and she said they probably just asked the head nurse for a cute kid. There was one other one done on our Ward who was also considered "cute". Compliment for our child, unfair for the not so cute children who spend just as much time here.

Over 72 Hours

2007-10-22T14:14:00.001-05:00

Well it seems that the new antibiotics have been effective in getting rid of James' fevers. We of course are excited and hopeful about this, but as time keeps ticking our excitement lessens as we are not seeing any difference in his oxygen needs. Occasionally we can get him down to 1.5L, but most of the time he's at 2-2.5L

Sometimes it makes things more confusing when there is more then one department offering their opinion, other times it opens your eyes to reality and helps you to be more prepared. Infectious Control stopped by this morning. There were a couple things they had different opinions on, but the one that sticks out is the fact that they aren't so confident that an infection is James' problem. The fever has been controlled, so there may have been an infection, but by this time we should be seeing a decrease in oxygen levels. They also feel he looks to good to have an infection. So in their opinion there's probably two things going on, we've gotten the infection under control but the PAP is not under control.

This is a hard call to make and the Team feels given James' situation they think we should give the antibiotics a full week before we make any further decisions. Given that James' macrophages don't work well this makes sense as he could simply need more time for his lungs to recuperate. So Infectious Contrl makes us realize that a normal child would have improved by now, and the Team reminds us that James is not normal.

James' stool came back positive for C.Diff. Since C.Diff can come from antibiotics or if already there can be aggrevated by antibiotics, we have no idea if it was ever gone, although it's not likely. So he will be restarted on medication for that as well, hoping to tame down the current diarrhea due to the antibiotics he's on. This time he'll probably have to carry on the medication for about 10 days after the antibiotics are done, so this leaves use somewhat unable to further investigate his colitis and stomach issues at this time.

Well I enjoyed my time home and made sure I packed a big box with lots of projects to keep me busy over the next couple weeks so I don't have too much time to think about what I'm all missing. Rob has decided to have the girls home this week and see how that works out. (When he made this decision he didn't realize they have 3 days off this week). Since he dropped his Hebrew course he is now home by 3pm each day for when they come home from school. He'll give it a try and see how much it effects his study time. Even Rebecca, who's always asking who's house she gets to go to next, was excited when I told her she was staying home this week. Marietta has her ups and downs. She's usually okay when she's away, but has a hard time saying good-bye again at the end of the weekend. And Matthew doesn't even seem to realize what's going on, which I suppose is a good thing.

Almost 3 Months

2007-10-21T11:07:00.000-05:00

.
Since I won't be home when Matthew turns 3 months old (Thursday) I figured I'd better take a few pictures today. Usually I take them in to have their portraits done on their 3 month "birthday", but that won't be happening this week. So here's the latest on our little man.

The Last Two Weeks in Photo's

2007-10-20T22:41:00.001-05:00

Enjoying a freezie after his first procedure. Once he had his fill and was no longer interested he decided to share his second one with Teddy.

Dr. James

One of his favourite toys is this backhoe which he loves to use to scoop up mega blocks, he now has an equally big dump truck which he likes to put the blocks in. Needless to say when he plays with these in his bed there isn't a whole lot of room left once they're both on the bed.

Family Time:

The window remains a favourite spot. The days he does get out of his bed he usaully spends some time looking down at the traffic below.

Colouring Time

Checking out the Photo Albums mom's been spending all her time on

James loves to play these two games - fishies and Bob the Builder. Bob the Builder is actually a memory game, but he's quite content to look at the pictures and put the pieces in the container.

Keeping Afloat

2007-10-20T22:19:00.000-05:00

Rob has often regretted his decision to continue on with school. He immediatley fell behind at the beginning of the year while he was trying to help out extra at home. Once he decided to carry on with school he ceased with the extra help and began to focus fully on his school work. But once behind always behind and so it was a challange in a good situation to get caught up again, and then things took a worse turn. Missing several days of school and trying to concentrate when so many other things are going on has not worked well and so Rob has decided that in order to keep afloat he is going to need to drop his Hebrew course. If he can survive through the semester with only dropping this course he can either try to make up the lost credit next semester or during summer school, and therefore save from losing a whole school year. Neither of these are a favourable decision as he'll only be able to manage this next semester if everything goes completely smoothly and summer school is difficult on the work and family situation, him being home so little as it is when he's just working (never mind that James has spent the last two summers in the hospital). But we will have to cross those bridges when they come, for the time being he needs to keep his sanity right now. He is fortunate that the University has been understanding and has agreed to allow him to take Hebrew next semester, even though he will not be completing this semester.

As for James. He is doing quite well. We have now gone over 24 hours without a fever. Yesterday morning he still had a low grade fever, which he managed to fight off without any medication and since then he's been without fevers, although sometimes still borderline low-grade. This is exciting as it's an indication the medication is working, unfortunatley his oxygen needs remain at 2.5L, we haven't seen any improvement in this area, but we did not expect to so quickly either, so we are still optimistic.

Since I now enjoy going for a walk each day instead of spending time in the computer lab that means you see Rob's name at the end of more blog entries. Several times a day I manage to get a strong enough wireless connection from our room in order to send and receive e-mails, but usually not strong enough to be surfing and posting blogs (altho I've managed a few times). So if I can't post the blog myself I leave it in the outbox and hope they make it to Rob who in turn posts them for me. So in case you all thought my writing style had rubbed off on Rob, I'm can assure you they haven't ... you may even notice that they are more grammatically correct now since he now gets a chance to edit my babble before it hits the screen! :)

As for the comments I received on walking. Yes, that is Queen's Park north of the hospital, and it's only a few minutes walk. I have not yet taken a closer walk by it but just a walk-by as I try to see what's all around us. And thank's Amy for telling me the Eaton's Centre was that close. I knew it was a few blocks over, but thought it was a bit further of a walk. Yesterday I ventured that way, but I've never been too fond of shopping and since I'm trying to get exercise speeding walking in the mall or around Younge Street doesn't work very well. But at least I know it's not that far if I ever need anything. My dear hubby showed up for a surprise visit on Thursday night, we had an enjoyable time and he brought my jacket ... not that I've needed it so far, but now I have one should it be cold again.

After being gone for two weeks I get to sleep in my own bed again tonight and enjoy the company of my children tomorrow, especially Matthew who I have not seen for two weeks now. It's a strange feeling when it feels more like you are babysitting someones child then that it's actually your own child. I look forward to the day when we can get to know each other again.

Infection? PAP? or Both?

2007-10-19T15:38:00.000-05:00

Well just as I was about to head downstairs to post the last entry, our Lung Specialist stopped by. We have not seen her since the last lavage. As an inpatient you are followed by the "Team", who consults with your doctor if necessary. Today she stopped by to discuss a few things, mostly when we should restart his GM-CSF treatment as he has now been off of it for over a week. They have decided to wait until Monday as we just started new antibiotics and don't want to throw too much into the equation.

She did not have a huge update on the work the scientist is doing, but what she did bring was not the most encouraging. So far, they have determined that the macrophages (which are cells) in James' lung show to either not work or not work to capacity. (Check here for how macrophages work. If you can, watch the video that's near the bottom. Amazing!)So, some do work, but not as good as they should. This in itself is nothing new and just confirms and supports the PAP diagnosis. The problem is that you need macrophages to fight infection. Which brings us full circle to todays problem. Is this problem indeed more a PAP issue then an infection issue? Are we unable to get rid of the infection because his macrophages are not doing their job? Is the PAP progressivley out of control because of this? This theory is supported by the increasing amount of foamy phelgm we are getting from his stomach, something which the doctors are no longer ignoring, but have come around to see it as a factor in the equation.

How do we get the macrophages to work? Sometimes a WLL is enough to get them up and going again ... so far we have been shown no reason to believe that this has been the case for James. The only other known way is to provide GM-CSF to the macrophages, but it is such a challenge to do this. We have been using the inhaled treatment. The other method is done by injection into the blood stream, which then works through the bone marrow, back to the blood stream, and delivered to the lungs. But this also causes more white cells to be produced, which in turns causes more problems in the lungs. So this way usually carries too many negative side effects for PAP. The question still remains whether the inhalation method will do anything, since it did not help before. But at the same time we can't give up on it just yet as we hope by removing the worst of the buildup we will create an environment where the GM-CSF can stay on top of whatever new build-up develops. Therefore we will restart GM-CSF on Monday, hoping it will indeed work better this time around.

A quick summary:

GM-CSF treatment is needed to to get the macrophages to function properly.
Macrophages are needed to fight infection
Infection of some sort is present.
Will GM-CSF treatment work?

The scary question is: If the GM-SCF treatment is unsuccesfull.... are we fighting a losing battle? There is no answer to this question right now. But we do know that if we remain focused, with our blinders on, regarding the achievements and limitations of man and his science, the question can be haunting. But we can lift up our hearts where Christ is seated at the right hand and majesty of God. It is here that we find rest, and hope.

What's another 10 days?

2007-10-19T15:17:00.001-05:00

*sigh* :(

Today they came by to take bloodwork before and after they administer James' Gentamicin antibiotic (he's on two new ones) to determine if the dose is appropriate. This caused a little lightbulb to go on.

If they need to watch the antibiotic that closely can it be given orally so we can be home??
The answer: NO.
And how many days do they administer these antibiotics?
10 days.

Given James does not have a PICC or PORT (something we are reconsidering ... see here,and here for previous thoughts on this) he can't go home with an IV. A PICC could be put in, but they basically said they did not think he was a candidate for going home. The nurse said they don't usually send home people on Gentamicin. The doctors said they would discuss these things further next week if these new antibiotics actually work, but at this time it's quite apparent that whether they work or not, we will not be going home anytime soon.

The stool test for c.diff did not work and they have to redo it. So we'll have to wait a couple more days for that. In itself we're not too majorly concerned, although the result does stand in the way of some of our other concerns. As I've mentioned before we find ourselves venting James excessively; we're also finding we get mucas or foamy phlegm (similar to what's in his lungs) out of his g-tube when he vents. At night or first thing in the morning I open his tube for a period of time to see what we get out on an empty stomach. In a 10 minute period this morning I got over 15mls of foamy phlegm. This leaves several questions. It's possible that he is swallowing some of the stuff in his lungs, which is not uncommon ... but if his lungs are supposedly relatively clean of the PAP why are we still getting so much "foam" out of his stomach? Is it possible that he's not actually swallowing this stuff but that because of the inbalance in his body he's producing it in his stomach?(???) The doctors have said this doesn't happen, so it's from the lungs. So why does James have so much mucas in his diapers? All this time we've thought his colitis isn't under control and have been working with his formula to try help. So what is it, his colitis out of control, or is this mucas coming from his stomach which is coming from his lungs?

How can we hopefully find some answers? By doing an endoscopy and/or colonoscopy. The problem is that c.diff causes colitis to flare up, so if we were to do such a test now and his colitis is flared up we would not know if this is from the c.diff or if it's been that way for a long time. So we have to wait until we have a clean stool that clears him of having c.diff and then we can consider doing such tests. At the same time we're not all that eager to put James out again just for the sake of these tests. It would be good to have the answers, but not really going to make a huge difference in his treatment. This is something we'd be more interested in lining up so that if he requires an aneasthetic for any reason in the future we can coordinate it all to be done at the same time.

New Medication

2007-10-18T13:46:00.000-05:00

I once again do not really have anything new to report. The Infection Control peoples decided that we needed to wait a full three days to give the medication a try, even though it was obvious that it wasn't working. Yesterday afternoon made three full days, but do you think they bother to reevalute at the end of the day? Nope, what's another 12 hours, obviously they aren't the one sick or waiting to get out of this place.

Last night at 6pm, 1am and this morning at 7am he again had high fevers. His oxygen requirements were up to 3L at some points during the night since coughing fits made him deSAT. So they could no longer argue to give the medication a chance, if it wasn't obvious enough before it certainly was now that it was not working. This morning they ordered to have his antibiotics changed, unfortunately his IV came out during the night and by time they actually got around to putting a new IV in it was late morning. So he is now running on new antibiotics which they say they have to give 3-4 days chance before making any further decisions. Let us pray no further decisions are needed but that this is the answer. Whatever the case we can expect to be here for the weekend.

James himself continue to do amazing through everything. Ibprophen does the trick to bring the temperatures down. Although awake quite a few times last night he quickly fell asleep when he realized mom was there to hold his hand. During the day he lays around when not feeling well and enjoys life when feeling better. Most days he spends some time sitting in the window watching the cars, or finds it a thrill just to sit on mommy's bed instead of his own. Just give him a new toy every now and then and he's happy and content. What a blessing to have such a easy, adaptable, loving child.

I discovered that I picked a bad day to try the great outdoors, yesterday and today have been much better. After two weeks of hanging around here I'm beginning to feel the effects of doing nothing all day and since I have no idea how much longer this is going to be I've begun to make a point of getting a bit more exercie (yes, walk the stairs instead of the elevators, it's only seven floors :) and am trying to get outside for a view of the city one direction or another each day. So far all I've discovered is that there's no interesting or useful places around here! :)

Still the Same

2007-10-17T12:53:00.000-05:00

It appears we are not making any progress on James' current situation. James himself has perked up a bit over the last couple days, appearing more energetic and himself, but as soon as the ibprophen wears off the fever seems to set in again. Over the last couple days we have not seen high fevers, but just mediocre ones, but he continues to be on at least 2L of oxgyen. So although he himself looks better he is not actually getting better.

This mornings x-ray showed the right lung to be about the same and the left lung to be worse. So it seems the infection has spread from the right to the left, which is to be expected. James is currently on a broad spectrum antibiotic which is meant to kill the bacteria that was found during his lavage as well as hopefully kill anything else in his lungs. Since this antibiotic is not working it is assumed that it is not this bacteria that is causing the infection but something else must be going on. The Respiratory Team's plan is to change to an even broader spectrum antibiotic to hopefully kill whatever could be in the lungs. At the same time they have called Infectious Control in to evaluate what they are doing and offer any adviced on what to give him now. We meet with them this morning to go over things and they have the same plan as the Respiratory Team. So we will be starting with a new antibiotic and if this doesn't work then we will need to do another bronchoscopy to once again clean his lungs and test what is in it to help them understand how to treat him. They have mentioned that there is a slight chance that this is his PAP at work, although nobody really seems to believe that this would be the case as it has never been seen to progress so rapidly. So, we are praying that this new antibiotic does the trick.

We still have not received the results of his stool test checking for C.Diff, but his stools have been good the last few days, so we no longer think that this is an issue. Whatever the case, it would not be causing the current problem.

Yesterday I decided to take a walk outside and get some fresh air while James napped. Usually I spend that time at the computer, but did not need to yesterday. My bright and fresh idea was cut short when I stepped outside and was reminded of how the world and the seasons keep on ticking even when we live in our small little world. Apparently a person needs to wear socks and shoes now, and long sleeves would be good ... and a jacket definitely helps!! :) Funny how I write the date every day, but it doesn't seem to mean anything. As I looked around and seen the flowers still blooming I realized that it's warmer then normal and here I was suprised as the last time I was outside it was shorts weather. And seeing as I don't have a jacket here, looks like I won't be taking any walks afterall ... maybe if I jog I won't need a jacket! :) So I hope you're all enjoying the lovely weather out there ... fall has always been my favourite time of year ... I can't wait to get out of here and have my opportunity to dress appropriate and go out and enjoy the refreshing air.

No Real Change

2007-10-15T20:52:00.000-05:00

Today hasn't brought a whole lot of change to James' condition, but since we have many faithful readers who are as anxious as we are about progress, we provide this update. James continues to be on about 2L or oxygen and spikes fevers throughout the day and night. Tylenol and Ibprophen give him some perkier times where he feels up to playing and chattering.

They did a nose swab today which came back negative, indicating that he does not have an upper respiratory infection/virus. When he had another fever spike this afternoon they ordered a blood culture to be done to look for other types of viruses or issues that could be causing the fevers. We have not received the results from this yet (and don't really expect it will tell us much more). He has been on IV antibiotics for 24 hours now but has not really improved, so the results from this blood work may determine whether to increase the amount of antibiotic they are giving him or not.

James' sore throat seems to be gone, or at least it doesn't sound bad anymore. Instead he now has a very chesty cough, which is in line with the inflammation which the x-rays show. He continues to vomit at least once a day and we don't know why this is. It could simply be because of phlegm when coughing, causing him to gag and then vomit. He has not had his GM-CSF treatment since last Tuesday, which suggests that the treatment itself is not the direct cause of his vomiting; we have always assumed that the loose phlegm (due to the treatment) was the reason for his vomiting.

James also continues to require a lot of venting to get the air out of his stomach. This again could be because of his increased oxygen needs, but there may be other reason for this. We have been getting a lot of phlegm out of his g-tube when we vent him, so we wonder how much this is backing up his system. Since we are still waiting for the C. Diff results we don't know if that's still a factor. GI has also suggested that sometimes the digestion of food can cause gas (e.g. a person who is allergic/intolerant to milk can become bloated) ... so if it continues we'll have to take a closer look at his formula. But yet, he never had this before, and he’s been on this formula for a while now. At nights we leave his g-tube open to let his stomach drain and we've been seeing huge chunks of phlegm coming out of his stomach. We always knew that he was getting stringy phlegm similar to what is in his lungs coming out of his stomach, but we've never really seen chunks before. Hopefully this is just because of the procedures and will settle within a few days.

They have decided to hold off on the CT scan for a couple weeks until his right lung has fully recuperated. Doing a scan now would give false results of how effective the lavage was.

Grrrr: They took blood (again) while he spiked a fever, and tested all other things but what they specifically took blood for in his fever. So now they're back again for more!! (Did I say Grrrr?? Let me clear my throat......GRRRR. Thats better.)

So for the time being we’re still bumming around here, waiting for things to settle down and improve.

At Bedtime

2007-10-14T20:17:00.001-05:00

Tylenol and Ibprophen has helped James perk up a bit. He was more himself when Daddy and his sisters came to visit this afternoon. At least he was able to enjoy the visit. As soon as they left he had to go down for another x-ray. By time he was done with that you could tell he was drained. He took a nap until they woke him up to do blood work again and replace the IV line he had lost a few hours earlier.

The doctors came just after lunch and said they were hearing crackles in his right lung which they had not heard yesterday plus they were concerned about his increased O2 needs again and therefore they sent him for another x-ray. The x-ray came back looking worse then yesterday indicating that there is definitely something going on. This doesn’t tell them whether it’s viral or bacterial, but he has been started on an antibiotic and if that helps we’ll have to assume it was bacterial. They had wanted to also give him a medication which would assist in getting rid of any fluid in the lungs, but after looking at the x-ray they feel the problem is more a virus or infection then fluid. We are still waiting for the results of the stool we sent off testing for C.Diff (and other bacterias as well)

This evening I had to again increase his O2 up to 2L for a period of time, but thankfully we’ve since be able to get it back down to 1L . His heartrate was also hanging in the 170-180’s (110 is a high normal for a child his age, 140 is normal for James), hopefully this is only because of the fevers. Once James seems to be stable and improving they will consider doing a CT Scan, doing a scan now would give us a false indication of the lavage results, so they may want to wait a bit before they go ahead with that plan now.

The bright side of all of this is that it looks to be some sort of virus or infection causing the problems and we can still pray that the lavage truly did a good job and his O2 needs will decrease drastically once this is under control. His cough and hoarse throat have also improved greatly today.

More Detailed Update

2007-10-14T10:11:00.000-05:00

When Rob said that James is not doing any better then yesterday (Friday) he did not explain really what that meant. Friday evening after the procedure James woke up from a short nap and started a “coughing fit” because he couldn’t breath. Although he did not present the normal sign/sound they are looking for when your airway is restricted they proceeded to give him a dose of Epinephrine via mask and a dose of Dexamethasone via IV. Since both were given at the same time it’s hard to say which one did the trick. James absolutely hates to have a mask put on his face and goes somewhat beserk when he sees one coming. Rob had to put some effort into restraining his hands while I held the mask in place. It took a bit for him to just calm down from the whole mask procedure, so by that time we don’t know if the Dexamethasone had kicked in or the mask treatment had done the trick. Regardless he did better the rest of the night, just having a few smaller coughing fits. He did not sleep well and could be heard saying “no mask, no mask” as he rolled around in his sleep.

Yesterday he was definitely under the weather, fighting a fever and have occasional coughing fits again. He would get to the point where they would call the doctors in and then by time the doctor actually arrived each time he had settled down enough to wait it out. At one point we did try a new mask treatment called Atrovent, but it had no effect whatsoever. This time I gave it through his mouthpiece and told him it was treatment time. He wasn’t all too happy about this but did cooperate and we did get it in him. Blood work yesterday showed his white cell count to be fine, so there is no indication of an infection. It also showed his potassium levels low and borderline of greater concern (and from what I understand if these levels are low it causes troubles with your heart). So they readjusted his IV. He also was not able to get much of his feed in so they had to readjust his IV again to make sure he wasn’t going to run into any problems metabolically.

Last night we had to turn his O2 up to 1L and even on that he is only SATing 93/94%. This is discouraging as yesterday he was on 0.5L all day and throughout his coughing fits and/or rough breathing he was still able to hold good SATs most of the time.

He continues to have fevers. Also his belly has been hard and distended. We’re trying a new venting system called a Farrell Valve which is set up with his feeding bag and suppose to relieve pressure during feeding. So far I can’t see any real results from it and I’m still venting him myself throughout the day. We’ve also been leaving his G-tube open during the middle of the night after his system has absorbed his feed and we’re getting chunks of phlegm out of his system that way. We’re not sure how much this phlegm is affecting his gut and how he feels overall. Even though we’ve been faithfully venting and using this pressure relief system he still passes some pretty strong gas. They sent away for a retest of his c. diff yesterday, as that also can cause excessive gas. We should get the results today or tomorrow.

The doctors had hoped he would look better today. It is not uncommon to have fevers for the first 24 hours after an anaesthetic, but he is past that stage now and they have to start wondering if something else is going on. The famous suggestion: he picked up a virus. So today they’ll do a nose swab for viruses. They’ll be giving him a shot of potassium and then later today checking his blood again. They want him to be feeling better before they do a CT Scan, so they’ll put in a request for one but likely it won’t be tomorrow unless he makes some major changes today. At the same time it’s highly unlike he’ll go home tomorrow unless there is major improvement and stability by the end of today. Tuesday will be the earliest and even that is somewhat questionable. *sigh*

Downhill

2007-10-13T21:25:00.000-05:00

James has not improved much since yesterday. His cough is oh so ragged! It seems that there is still a fair amount of swelling in his wind pipe, for his breathing is still quite laboured. He has had a few doses of Dexamethasone to counter the swelling, but he may need some more tonight yet. (As an aside, I had the privilege of administering Dexamethasone to a sow back on the farm with fantastic results: she went from lame to running overnight...just too bad for me that I left her loose in the aisle). The X-ray he had this morning did not show as wonderful of a cleaning as on his left lung, but this may be to the extra fluid that couldn't be extracted. He's still SATing 95-96% on 0.5L of O2, but is not able to go without yet. We are still waiting to get a Low-Flow valve for his O2, since the one he is on only goes as low as 0.5L, but it seems to be a rare commodity. Although they wanted to do a CT scan in a couple weeks to get a "clean" picture on file, they are trying to get this done on Monday already, and we're thinking because the X-ray wasn't that great. James has had higher fevers today (over 39 C), and did not subside significantly with Tylenol nor Advil.

So all in all, James isn't out of the woods yet. This is somewhat of a let down, but after all he went through this week, it is to be expected. It's just that he did so well after the 1st one...

I sign of wishing you all a blessed Sunday. May we all be nourished by God's Word, that we may continue to examine our lives by it, and more and more realize what an unmerrited gift of grace we have received.

WLL # 2

2007-10-12T13:54:00.000-05:00

Hi all

With thankfulness and awe of our Master Physician I may report that they were able to do the lavage on James' right lung today, and that he is out of the O.R. recovery room and is in the Observation room on 7th floor for the night. We were not able to speak with the Dr or Anethesist (sp?) since they were whisked away somewhere else. However, the nurse read the report, and said they only needed 1.8L of Saline to do the job (as opposed to 2.2 for the left). This adds up, since the right was not as bad as the left and would not require as many rinses. However, they were not able to extract 300 mL of the Saline. They say that this will be absorbed into the body within 24-48 hours, and is not really a great concern. He will have an X-ray done tomorrow to view the success of the procedure.

James is doing relatively well at the moment, (even though he did not come out of recovery as cheerful as on Wednesday) and is only on 1L of O2 and SATing 96%. This is really good. On Wednesday at this time he need 1.5, and was 0.5 by the next day. So if this holds up, we hope to see him at 0.0L of O2 by tomorrow some time. This is optimistic on our end, but I don't think unrealistic.

It appears that the C-Diff is not under control, but we cant know if this is all because of being NPO (off feed), missing a couple of doses and being sedated, or what. By Monday he should be settled enough and they will re-test.

We are indebted to all of you who have pulled together with us and for us in prayer, encouragement, and support. In all this it is humbling to experience that neither God nor His people have forgotten us. This too is a witness of your faith.

Until next time....

Tomorrow

2007-10-11T13:29:00.000-05:00

Psalm 89:1 "I will sing of the LORD's great love forever; with my mouth I will make your faithfulness known through all generations"

How gracious our God had been!

Ever since July when James was diagnosed and we were made aware of the risks of doing a WLL on an adult, and the greater risk of doing it on such a small child we have dreaded the day we would hear that James needed a WLL. As time went on and we realized that we had no other choice we began to accept this as we could not bear to see him slowly suffering more and more. I cannot even begin to express what a sense of relief it was yesterday for everything to go well. Not just well, but SO well. We of course don't know all that has gone on behind the scenes or what they are not telling us, but the doctors almost make it sound like it was all a breeze. We know it wasn't, for we also know that they had to work hard to get a Team together who was willing to attempt the procedure.

James is doing wonderful. We cannot get over how he woke up from the procedure, it was just like he took a little nap. I am used to a screaming, upset, cranky, irritable child after anesthetic so this was totally unexpected but very welcomed. James spent the night in the observation room and did wonderful. He slept well with no deSATs. This morning he was moved back to his room again. He is currently running on about 0.5L of oxygen and SATing around 96% on that ... a definite improvement. They were extremely happy with how his x-ray looked this morning. We have held off on doing James' GM-CSF treatment until he is feeling better, he has a really sore throat now from the tubes being put down it. They expect to get some result back today on fluids that were taken out of his lungs to be tested for infection. It will probably be next week before we get results on the surfactant that was sent to the scientist to evaluate how it reacts to the treatment he is currently on.

So what does the title mean. No, it does not mean we're going home tomorrow. Tomorrow we will head back down to the O.R. to clean out his right lung. Our doctor put in a requisition for O.R. time and was told there is a cancellation tomorrow morning and it's ours if we want it. They feel he is doing well enough to go, clinically he is doing better then when he went down yesterday. The only thing I was concered about was his throat. It sounds awful and he has an extremely hoarse cough, I was concerned the swelling would make it difficult for them to put the tubes back down. They advised that they can give him a medication to reduce the inflammation and if they really felt it was too bad to proceed they would stop. Getting O.R. so quickly is amazing and having the entire special team available again tomorrow is even more amazing so there doesn't seem to be any reason to hold us back.

I pray that they, as well as us, are not getting overconfident. They have decided not to book ICU this time, so we won't have to worry about a cancellation due to that. I keep reminding myself that just because yesterday went so well doesn't mean tomorrow will be the same. They have advised us tomorrow should be better and easier then yesterday. For one, he is that much healthier. His left lung will have to do the work and it's in much better shape. It is also easier to put the tube into the right lung to clean it, so the procedure itself should be easier as well. We pray that our Heavenly Father will once again guide the work they are doing and that we do not become confident in the hands of man but remember who is in control. We know that we will be more relaxed and less anxious tomorrow then yesterday, but we continue to remind ourselves that things can still go wrong, just like they could have last time. But oh, how nice it will be to have it all done.

If things go as well tomorrow as they did yesterday then they hope to discharge us on Monday. Yah!! I am thankful for this going so quickly as I was dreading having to hang around here until next week, or being discharged and having to come back ... that would have been worse. At least we are settle here now, back and forth is harder on the family and on James. There are great advantages to having this done as inpatient also. If he had to come back as an outpatient who was later admitted we would not have a room here anymore. That would mean hanging out in the O.R. waiting room before hand (you have to be there two hours before your procedure and who knows how much it will be delayed). It would mean repacking and having to drag everything in again. It would mean that I would not have a room to sleep in if he is in Observation. Last night I had my own private room since they left it for James to come back to today. The C.Diff has been handy also (if you want to look at it that way). Because James has C.Diff he goes into isolations rooms before going to OR and after in the recovery room. Normally only one parent goes into the recovery room but since we have our own private room both Rob and I were able to go yesterday. Not that it really matter as he was so calm and collected anyways.

So, that's my story for today. More again tomorrow!! :) We continue to covet your prayers.

Galatian 6:2 "Carry each other's burdens, and in this way you will fulfill the law of Christ."

Success!?!?

2007-10-10T13:56:00.001-05:00

Only time will tell us whether today was completely successful but at this point everything went amazing well. I am so thankful to be able to pass along a "happy report".

Our doctor came out of O.R. with a huge smile on her face, a smile that told us she was happy with how things went ... and indeed she was. She said things could not have gone better. It took them an hour to get the tubes into place, but once they were the lavage went well. They put 2190ml in his left lung and were able to suck back out 2100ml, the lung will absorb the remaining 90ml. The initial stuff they sucked out with thick and white, like we have been seeing him throw up and coming out of his g-tube, but by the end they were getting pretty clear liquid back out of his lungs.

Whether this will cure James we obvious don't know, but it will definitely helped his lung and we have seen that already. They were able to take him off the ventilator and by time we got to the recovery room he was calm and collect and asking over and over for juice. Unfortunately he had to wait another two hours, at which point he goobled up an entire freezie and asked for another. Not a major accomplishment for most children, but for James who never takes more then one small zip out of cup this is a lot for him to take in at one time.

By time we left recovery he was SATing 98% on 1.5L, he was chipper and happy, his colour had come back and he looked great. He came back up to the ward doing better then when he went down, but they have put him in the Observation Room for the night. I'm not sure if that plan will last or whether they will change their mind once they see how well he is doing, we'll see as time goes on.

We are so thankful that all went so well today, we know who we need to thank for this and we continue to stand in awe at our Heavenly Father's loving care.

At this time we do not know what the next days will bring, we pray this good is not the calm before the storm, but things look extremely positive right now. As we see how he recuperates they will decide when to do the next lung. They have suggested next week, but if he needs more time probably in two weeks time.

O.R.

2007-10-10T09:37:00.000-05:00

James left us for the OR room at 8:10 this morning. They told us they thought this new technique would take less time. They had the OR booked from 8am-11am, but figured it would only take two hours. At 10:10am we received a brief update that all is going well so far, but it sounds like they had only started to actually do the cleaning. They were just taking the first sample over to the scientist who will be studying it. They advised they figured it would probably take another two hours.

After he comes out of OR he will be in recovery for 2-4 hours. They will determine when they are done whether it is safe to take him off of ventilation on whether he will need to stay intubated and go to the ICU.

We will update when opportunity arises again. Thank you for all your prayers.

James and Family

2007-10-09T13:43:00.000-05:00

After our Lung Specialist appointment on September 14 we realized that James was going to need to have a WLL done at some point. At that time we did not realize how soon it would be. One thing we decided was that we wanted to have photos done before the procedure, updated photos of James and updated family photos. I was still thinking about when and where when we received a lovely offer from Tobi Bos Photography (and her blog). To follow are some of the pictures she did for us. As you can see a couple of them have already been displayed on the new and updated blog look (which I must give credit to Rob for).

And since this computer is soooooooooooooo slow and my time limited ... I will have to save more for another day.

Thank you Tobi!!

A New Technique

2007-10-09T13:07:00.000-05:00

I didn't think I would bother with an entry today as there was not much new to say, but on my way downstairs I ran into our Lung Specialist. I guess the past week gave her some more time to think and do research and they have now decided to do a different approach then what they would have done had they done the procedure a week ago.

The original plan was to do a very extensive broncoscopy. Using a bronchial tube they would go down into the lung and wash section by section. There are some reports of sucess doing this and others have been unsuccessful in getting the lung completely clean. She was concern about this and also felt this partial washing would be harder on the blood circulation in the lung then the new technique she hopes to use. In an adult Whole Lung Lavage (WLL) they use what's called a double lumen endotracheal tube. I won't pretend that I completely understand how it works, but I do know that this is when two tubes are connected together allowing them to use one ventilation system while going into each lung at the same time. These tubes have cuffs at the end allowing it to block off a lung if necessary. How the washing is done using this I am not completely sure, but I think it means that they would pour the saline water down one side and ventilate the other side. Whatever the case these are too large for children.

I guess our doctor began to think about how we were going to be putting two tubes down, one to ventilate and then the broncoscopy tube, so she wondered why we could put two single lumen tubes down instead. This would mean that they have to have two ventilation systems running. So over the past week she did lots of research and feels that they could do this. The advantage of the lumen tubes is that they have cuffs on the end, allowing them to completely block off one or another lung. Using this technique they will test which lung seems strongest and proceed to clean out the weaker on, which at this time they believe is the left side judging by the CT Scan. The main advantage of taking this approach is that we will then be able to actually do a WLL instead of just a Partial Lavage as was planned last week. Because of how the lungs function (which I also don't completely understand) she feels that this would be an even safer route as it will prevent the lung from having to go through big blood circulation issues with each partial wash. So she is confident that this a better route.

Of course my question was ... if this seems to make so much more sense and seems to be a safer route then why have they not done it before? She could not answer this, but felt part of it could be the technology advances we have in recent years where they have smaller tubes that have cuffs on them, therefore now allowing such a technique.

In the end there is not much choice to make. We fully realize that we have no real choice and that we have to put our faith and trust in the Lord to guide the doctors in the right direction. On the other hand, can we be thankful for this extra week of waiting? this extra week to make the doctors think and possibly come to a better result? Only time will tell and we pray that that time will come tomorrow as schedule.

When I ran into our Lung Specialist she was just on her way back up from the ICU. She had scoped out the place and feels that unless something major happens we should be able to move ahead tomorrow. She advised that she has also spoken to the ICU Centre about using an ECMO (extracorporeal membrane oxygenation) system if this technique does not work. This is when they put both the heart and the lungs on by-pass. We often hear of heart by-pass, but this is a step further and this means it involves even further risks. I have not had much time to read on it yet and hope that I will not need to. This is not something that would happen immediately but only after much consideration if we find out that tomorrows treatment is not effective.

We thank you all for your continual prayers. Please pray that the procedure may go ahead tomorrow. Please pray for the doctors and the work that they will be doing that it may be safe and effective. And once again, please pray that whatever the outcome we may accept this as the Lord's will.

Wednesday Morning ... Maybe

2007-10-07T12:26:00.000-05:00

At this time we have been informed that James is scheduled in the OR on Wednesday morning at 8a.m. This of course once again hinges on whether there is a bed available in the ICU. So as it stands now we will be in hospital almost two weeks before the possibility of the lavage taking place.

On the positive side: this time in hospital may not be good for the family, but it is good for James. The low activity level is what is keeping him stable. We definitely seen this on Friday when Rob and the girls came again with my parents as well. James wanted to join the rest of us on the couch and looking out the window. Just the simple move from his bed to the couch made him deSAT and his heart rate sour. We also allowed him to sit in the window in his room for a bit before we forced him back into bed since his oxygen requirements had gone up a litre and his heart rate wasn't settling. During this time he didn't even do any walking, he was simply sitting, fooling around a bit and getting more excited because of the change in the scenery and entertainment.

James' "owee's" seem to have improved, so if it stays that way then we're figuring it was likely some discomfort due to the C. Diff. It looks like he is slowly on the mend in the C. Diff area as his stools are beginning to head back in the "normal" direction. GI did a change to his formula yesterday, just adjusting the amounts of the three formulas he's already getting. It will be hard to gage any initial effects of this change as we're still dealing with the C. Diff right now. GI has also talked about doing a Calorimetery test. In this test they measure the calories your body burns when resting and then use that figure to estimate what you would burn doing other activities. When I read about the test I get a pretty strong feeling that James is not going to cooperate for this one ... and he definitely won't be resting when it's done, so I'm not sure it will be of any use.

So for now it looks like we're just "hanging out" for the next few days, waiting, hoping and praying that everything will go through on Wednesday. Rob has taken over my post for the day, and I'm home with the kids. Later today we'll spend some time together again before I take up my position again and the others go back home and from there get "disbursed" to their new homes for the week.

The Week in Pictures

2007-10-06T22:46:00.000-05:00

Hanging out in Emerge:

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From litle bed to big bed:

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Family Reunion on Sunday:

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Finally well enough to venture out of his room and the next day put into isolation:

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GM-CSF Inhalation Treatment and "Cheese" (James way of telling us he wants us to take a picture of him):

Today's Story

2007-10-05T13:02:00.000-05:00

Much to our surprise this morning when they did rounds they told us that not only could we have an overnight pass, they figured we could have a 48 hr pass. Considering they haven't gotten to the bottom of this "owee" James has, which has been getting worse instead of better, we were suprised that they thought we could still go home.

That plan changed quickly when they went to arrange our pass. Supposedly 48 hour passes are no longer allowed, nor are overnight passes. Apparently if you are well enough to go home overnight you should be discharged. Funny how the doctors know nothing about this change ... makes you wonder, but we won't get into that. We were given a choice: be discharged today and come back Monday afternoon or stay. Well that was a pretty easy decision ... we're staying. We know all to well how easy it would be to get our bed back on Monday ... never mind the whole hassle of packing up, emptying our room, going thru discharge routine and then having to unpack and go thru admission routine again. It's nice to be home, but not that necessary. At the same time they warned us that now that the request was made to go home it could be that they may want to force a discharge if they need the bed seeing as we were apparently well enough to go home for a couple days. Well ... force they will have to do because we won't be leaving on our own. From a paper perspective James is stable respiratory wise. We can't argue with that, since we arrive he has done better, altho still required 1.5 to 2.5L of oxygen ... but he hasn't gotten worse. The reason for that ... he sits in a bed all day and does nothing. Each time he has gotten too excited or active he's thrown up and/or deSATed. It would be interesting to keep him in bed all day at home ... we would be back in Emerge pretty quickly. So we're not going home.

At the same time they're doing there best not to promise anything for Tuesday. Basically it will be the same as on Monday. They've requested and somewhat lined up OR time as well as "booked" a ICU bed ... but James is not considered an Emergency yet, just High Priority, so we can still get bumped. The joys of our healthcare system.

GI came by yesterday and went over James' situation in great detail, so far I was impressed. The Team told me today they are looking to change his formula. They did not come by to talk to us today yet, so I haven't heard the official word yet. We haven't gotten any more word, help or info on why James' keeps complaining about owee's. The first urine test was fine, but we did another one today, just in case. The blood culture they did the other day isn't back yet, but they don't really expect an answer in that. So we're at a standstill in that area ... and it being the weekend ... well we know how that goes.

But I must head back to the room. Usually I leave once James is asleep, but my little visitor - Matthew - was not all too quiet, so I left James with the instructions to take a nap while I tried to walk Matthew to sleep ... which hasn't been successful yet. As much as I miss Matthew (and the girls of course), I'm so glad he's NOT a nursing baby. I think I would go nuts if I was trying to take care of both of them every day. But a one day visit is quite nice ... I'm allowed to spoil him then :) It's almost like being a Grandma, I can spoil him for the day and then hand him back over at the end of the day :) Oh and look ... he just fell alseep in my arms.

C.Diff and Venting

2007-10-04T12:39:00.000-05:00

A little about C. Diff: C. Diff is a bacterial infection, so this means it can be treated by an antibiotic, which is what they are doing with James. Antibiotics are usually very hard on James’ colitis, but they’ve told us that this antibiotic is usually good for colitis and may even be helpful … unfortunately not long term tho. Other then diarrhea, some of the symptoms of C. Diff are fevers, vomiting and abdominal pain. So … it’s possible that James’ fevers are from the C. Diff. At the same time, fevers also occur with the GM-CSF treatment. And when we talk about James … he’s been known to get unexplained fevers for the past year. So it’s hard to say if his fevers are related to the C. Diff … if they go away in the next few days then we’ll have our answer. We are pretty sure that James’ vomiting is related to his GM-CSF treatment since he is vomiting up phlegm most of the time and often a vomiting episode comes during or after a moaning/grunting episode (which are terms for describing a type of breathing where you are exhaling very loudly and in turn not inhaling very well). C. Diff is also common in people with colitis and they have advised us that a lot of times if a person has serious colitis they automatically put them on the antibiotics for C.Diff if they are hospitlized.

Where did James pick up the C. Diff? This is a harder question to answer. C. Diff is often caused by antibiotics. It is possible that this is still from James’ last dose of antibiotics since C. Diff has the ability to live in a body without symptoms for 8-10 weeks. But it has been over 10 weeks since a James was last on last on antibiotics. The same goes for picking it up in hospital. He has been out of hospital more then 10 weeks, and his symptoms appear the day before he came into hospital.

At this time we are unsure if this is the reason for the oweee he keeps complaining about and then grabbing his diaper. We’ll find out in the next couple days. They did send a urine test away, but initial results do not show anything.

C. Diff does also cause extra gas in a persons system. Last week we started a new “procedure” on James, called Venting. The advantage of having a g-tube is that we can take gas out of the system before it reaches the intestines. When venting we open up the tube, attach a large syringe with the plunger out of it and then press on James’ stomach to get the air out. James calls it “bubbles out” because as the air comes out it makes little bubbles in the bits of milk that also are in the syringe. The reason we started this was to see if it would help settle his moaning/grunting episodes during the day. If your stomach is full of air it presses up on the lungs (sort of like being pregnant) therefore limiting the lung function. We did notice that venting seemed to help during the day with the moaning/grunting episodes but it did not seem to help with the worst episodes at nap time or night time. For a bit we withdrew the air into a syringe so we could calculate how much he had and we were drawing back about 100ml of air several times a day, along with smaller amounts here and there.

It’s hard to say whether he has a lot of gas in his stomach because of the C. Diff or whether this has always been there since we have only been doing this for a weeks time and have nothing to gage by. James has come to realize what it feels like to have “bubbles” in his tummy and will tell us when he wants “bubbles out”. I’ve learned that even if I just did it 15 minutes earlier, if he says “bubbles out” there’s probably more there.

The C. Diff should not effect James’ procedure, unless he suddenly starts spiking some pretty bad fevers. And if he were to do that we would likely not be from the C. Diff because he’s on antibiotics now. Tuesday night we got them to pull out James IV as we seen no use for having it in. Well poor little guy only got one days break and yesterday they put one back in and hooked him up to IV fluids just to make sure he doesn’t get dehydrated. They insisted on doing this, even though I told them that he is having less bowel movements now then what is normal and he is still tolerating all his formula. They want it as a preventative. Today they lowered the amount that they are giving him.

And I spoke too soon about night times … should know better by now. James is more back to his normal night time moanings and deSATs … not his extreme bad version yet, but up often enough anyways. So much for getting rest.

We seen the GI resident this morning, who went over James’ history. They will be getting in touch with MAC to get the results of some of his previous tests and then do a review as a Team. We should hear back from them once they have gone over things. They are also helping to keep an eye on and be on top of the “oweee” James keeps complaining about.

Short Lived Freedom

2007-10-03T13:27:00.000-05:00

Well, only about two minutes after I published my last blog the nurse shows up to tell me that they received James' stool test back and he has tested postitive for C. Diff (or Clostridium difficile cytotoxin).

What does this mean? I don't know you yet, but I do know that it means that we are now on contact restrictions ... or isolation. Anyone who comes in the room has to wear gowns and gloves and James will no longer be allowed out of his room ... now who wants to explain that to him after nap?? Not me!!

The doctor has been informed and we're waiting to see what they plan to do. In the meantime I better go search the playroom for something to keep this boy busy ... now that he's been having a bit more freedom he's a bit harder to keep entertained.

Nothing Happening

2007-10-03T12:58:00.001-05:00

Well there isn’t anything new to report. At this time things are being lined up for Tuesday. We will actually be able to get an OR slot in advance this time, but that doesn’t really matter as we can always get bumped. And then there’s always the ICU issue, which means if there isn’t a bed again we would have to wait again. So nothings ever a guarantee as we have learned over the past couple years.

James is doing better spirit wise. He has more desire to get up and move around. He still likes the TV and will now actually sit up and bed and play with his toys (before he would only do so laying down and refused to sit). He also asks to go for a walk each day. Thankfully we were smart and brought a really long oxygen extension hose (which the hospital does not have), so we are able to hook him up and let him wander a bit more freely in the room and the hallway. This allows him to sit in the bay window at the end of the hall and watch the traffic … and being a true boy this is quite thrilling to him. He takes along his dinky cars and rides them up and down the window while he watches what goes on seven floors below him. After nap the new routine seems to be that I take him for a walk in the stroller. It being a bit of work to load everything and switch him over I’ve made a point of getting him used to the idea that this only happens once a day. He likes to sit down in the cafeteria area and watch the waterfalls and elevators. Once bored of that he enjoys to sit outside (weather permitting) and watch traffic again. Yesterday he asked to go back to his bed after an hour, he seems to know his limits.

Thankfully James oxygen requirements have not gotten any worse, although I believe a lot of this is due the the lesser amount of energy he uses. Whenever he does get somewhat active – even just walking around or having a bath – his SATs drop. We have been able to get him below 2L at times, usually at night, but once active he’s usually back up to 2.5L and even then his SATs are borderline.

And just in case we get bored, he thought he’d throw a new loop into things, developing some nasty diarrhea even for James who has always had loose stools. On top of that he complains his bum/bottom hurts all the time, but he actually doesn’t have a rash for once. A urine test today showed high protein and ph levels … so further testing has to be done to see if he’s brewing an infection or why there is such high acid levels. Since we are sitting here doing nothing we asked to see the GI Team. We have never been happy with the doctor we have at Mac, but knowing how hospital politics work realized we had to stick with him or our lives would just be more miserable. Now that we spend most of our hospital time in Toronto and not at Mac I’ve gone ahead and requested our GI Doctor be changed . So we’re hoping to have the team here evaluate him and maybe give us some input or advise on James’ colitis, continual diaper rash and yeast infection, and continual loose and mucasy stools. If we are happy with the team here we may just decide to stick it out. The more we can have tackled in Hamilton the better, but if we’re not getting the care that is needed there we must deal with the hassles of going to Toronto.

Well for nothing going on, I’ve manage to babble plenty. Until next time,

Not Today ... and Happy Birthday Dear!

2007-10-01T13:09:00.000-05:00

So you're all waiting for the update ... here it's goes:

The never-ending story. Things never seem to work out as planned, we should know better by now. We did somewhat expect that they might not be able to do it today, as it all hinged on getting OR time after a weekend, something that at times can be difficult. In the end it was not OR that was the problem, they had a spot for us later this afternoon, it was ICU that was full and could not provide a bed for afterwards. I guess part of us just tried to ignore the possibility it wouldn’t happen, so it hasn’t gone over easy that we have to wait.

Waiting at this time does not mean a day either. Part of the reason there was such a rush to get us here and get this done is because the majority of the Respiratory Doctors are leaving for an important conference tomorrow and won’t be back until next Tuesday (I imagine they themselves will be back before that, but it being a long weekend …). That being said, they are now looking at the earliest possible date being next Tuesday, October 9. And you can well imagine how that will work out if they are all just coming back after being gone a week. Of course there are some Respiratory doctors still left at the hospital, but the issues comes in the fact that not just anyone will tackle this procedure. It being a delicate and risky procedure it needs to be someone with a lot of experience in Bronchoscopies. There were two doctors willing to do it, our doctor and the head doctor, who are both going to the conference. Our doctor came to see us this morning and advised that there is one other doctor who is experienced with lung transplants and bronchoscopies that just arrived back from vacation, who might be willing to do it while she is gone. We will find out by the end of today, otherwise we have to wait.

It’s funny how what was considered a high priority case can all of sudden be put off for another week. Once again we’re involved in hospital politics. It seems they have tried what they can tho, as they even looked into getting an ICU bed at McMaster, but McMaster was also full. They actually do not expect he will need the ICU bed, but due to the procedure it would be foolish of them to go forward without having it available. Our biggest concern is that two weeks ago James required 0.5L oxygen, one week ago 1.5L and now often 2.5L … what will another week bring.

James is not able to go home. He has been relatively stable the last few days, but his oxygen requirements have increase drastically in the last couple weeks. We also believe part of the reason he’s a bit more stable is because he isn’t doing anything anymore. He spends all day sitting in his bed - mostly watching TV, sometimes playing with a truck or car, and occasionally reading a book. The few times he has gotten out of bed, for a bath or to play on my bed have made his SATs drop or he vomited. This morning he got upset because he couldn’t have what he wanted and immediately he deSATed and his monitor started beeping off, which in turn brings a nurse to the room … talk about a good way to get what you want!! (One doctor did say we would be going home, but as much as I would like to I think we would just end up back in Emerge within a day or two.)

They have said that he may be able to get some passes if his requirements stay stable, possibility an overnight pass for Thanksgiving. Even if he remains stable they don’t want to lose the bed, so he will only be able to go home for short periods. Considering the distance and the hassle that comes with this (we are not well equipped to administer this much oxygen at home, never mind the fact that our tank is currently empty because we knew we wouldn’t be home for a bit), I’m not sure how much we’ll do this. We’ll probably take the overnight pass, but not the shorter passes.

We are also going to try a new nebulizer (in the picture you can see the mouth piece that James is currently using, we use a different compressor machine, but same basic idea) for doing James’ treatment. The CT Scan showed that the center of his lungs cleared, but the peripheral parts did not. They are hoping this new nebulizer (basically looks the same, it's the parts inside that are different), which makes the droplets smaller then the one we had will reach the peripheral parts. The nebulizer they gave me was the sample they had in the lab, it is not released in Canada and is only being used in German at this time. It has a nice big sticker on it NOT FOR HUMAN USE. He told me not to start using it until we got home … more hospital politics.

So here we sit for another week or two. Previously we had wanted a situation where the kids would be home when James was in hospital so that our household continued to run somewhat “normal”, but in the past weeks Rob has already fallen so far behind in his school work while trying to help around the house that now we feel it’s better that the kids are gone and he just has quiet to focus on the school work when he has a chance.

All rooms are private rooms here and that makes our stays much nicer and more endurable. Thankfully I have a laptop that I borrowed which helps me to keep my sanity. I took some photo albums along which I am only a few years behind on and am slowing working on getting them caught up. Surprisingly I can actually say that I might feel well rested by time we get home, or at least well rested by time he has his procedure. James has been doing better here at nights (can this also be attributed to the less activity during the day?), which means I’m not getting up every hour to tend to him and can actually get several hours of sleep at a time.

If anyone decides to come for a visit, please call first as I am not sure if or when we might go home for a bit. Also, if you’re from our area, maybe check with Rob if there is anything that I need brought up since he will not be coming to visit again until the weekend. So far I think we’re good, but you never know what I might think of.

And as some of you have already commented on this, I figure I’ll take a second to as well. Today is Rob’s birthday … congratulations dear hubby on another year!!! Looks like we’re going to have to cancel our plans for tonight and celebrate another day!!

Family Reunion

2007-09-30T20:57:00.000-05:00

So today the family was together for a few hours, albeit in James' room. The room was a little smaller than on Friday, since James received a real bed instead of a crib. We had requested that he have a bed, since he sleeps in a toddler bed at home, and a real bed would make his play area a little bigger. And bigger means less floor space in his room. So when I arrived with the girls, we found a table and benches in one of the hallways, and had a "picnic" style supper. James was content to stay in the stroller and look out the big glass window at cars and buses and people 7 stories down. After dinner, we were about to go for an elevator ride (which are really cool because they're glass, and you look down on the cafeteria below), when Matthew arrived. With the family complete, we went down to the waterfall, but again James was content to stay in his stroller. Soon after we went back up, the kids were playing in the hall while Steph and I visited with our guests, James asked to go back to his bed. So all in all, although his O2 levels were fine (on 2L), his energy level is down, and he does not have much get-up-and-go like he used to. What was interesting was that although sitting, he would laugh at the girls goofing around. Often times it is James' cheerful soul which takes the edge off his harsh world, and certainly assists us in coping with his troubles. I have often said, and will say it again, for all the trouble that James has been, he really is no trouble at all. And that is a gift from God. How gracious He is!

CT Completed

2007-09-28T19:05:00.000-05:00

On Wednesday when the doctor said they did not want to wait until Monday to do a CT Scan, but wanted us to come to Emerge, we spent some time debating whether it was really that necessary to come right away, finding that his condition did not warrant an Emerge visit and we would feel silly coming to Emerge with a relatively stable child. Well it seems our decision to follow Doc’s instruction was wise.

James’ oxygen requirements have again increased and he is now requiring 2-2½ L; at one point yesterday they had him on 3 L. Emerge did not think at all that we were out of place coming and we almost felt like they were overreacting, mostly because we are used to dealing with him and know how to react to his different health states. James himself is much more mellow, not having a real desire to get up and play. He has spent the last two days in his bed and is completely content there with a few toys and the TV. Although he usually likes baths we, couldn’t convince him to get out of bed to take a bath this morning....he just wants to lay in his bed. Although lacking energy he isn’t so sick that he complains or wants to be entertained, but plays well on his own.

The CT Scan was done this afternoon. We’re so thankful that they have good enough equipment here to do unsedated CT’s and we don’t have to go through the whole process we used to with sedations each time. Radiology isn’t always happy about it as he cries during the process making it hard to get a good picture, but each time they’ve managed to get a good enough picture in the end.

By the end of yesterday it became obvious that they were planning to do a lavage regardless of what the CT showed. Clinically, James is deteriorating and is bad enough that they can no longer allow him to go home. Yesterdays x-ray showed that James looked worse, and so were actually surprised at his CT Scan today. They reviewed it with two radiologist: one felt today’s looked the same as last time, the other felt it looked worse. The Lung Specialist felt it looked a bit worse, but not as bad as she expected. James’ LDH levels, which had been getting better, have suddenly gone back in the other direction, and yesterdays blood gas levels were not good either. All this indicates that we are not making progress in the right direction with his current treatment and a new approach is needed. If we wait too long he will be at a much greater risk of not tolerating the procedure, and so the wheels are rolling to line up a lavage on Monday.

Yesterday we met with anesthetics and genetics to prepare for Monday. At this point the doctors have their team organized and an ICU bed reserved (just in case) so that everything is ready to go ahead with the procedure. O.R. has James on the list but we won’t get an actual time until Monday. With the priority they placed on the order, they are pretty confident it will go through Monday, but there’s still the chance that greater emergencies will come up that will bump us to another day.

While doing the Lavage, they sending some of the surfactant that is taken from James’ lungs to a scientist who has been doing research on PAP and different treatment options for this disease. They will use the macrophages (cells) from this and do various tests to see how they react to GM-CSF and other treatments. From this they hope to get some indication of whether the GM-CSF treatment was actually effecting the cells and working or whether another treatment might work better (more trials). Once again these are trials and they won’t know for sure but are hoping to get some indication on how to carry on with treatment after the Lavage.

Although there still remain risks with doing the Partial Lavage, the doctors are confident that things will go well and that James will make it through okay and recuperate quickly. They are still saying that he will likely be able to go home after a couple days, and we hope they are right.

No Further Ahead

2007-09-27T12:54:00.000-05:00

So we awoke at 4am, left at 5:20 am, arrived at 6:30 am, got to Emerg by 7am (had to do James' treatment first), had a bed in Emerg by 7:20am, had blood work at 10am, sat around until 12:30pm when we chatted with our doctor, had an x-ray done at 1:30pm and that's pretty much the sum of things.

There are so many emergencies that need a CT Scan today that we're not further ahead, unless your dying you won't get one today. We did get a bed though, we're waiting for a patient to be discharged so we can be moved to our room.

Although nothing has really changed the doctors are still very busy and likely we will be busy later today or tomorrow. The CT is now in the works for tomorrow and all further plans hinge on the results on this scan. Todays blood work looking at James' the blood gas levels did show sugnificant lung disease/damage. Even though they cannot get the CT Scan done today they are still working on getting everything together. We will have consultations with the anesthesis and genetic team in order to have everything prepared if we need to proceed. At this point they are aiming to have the Lavage done on Monday if it is necessary. But once again it's the battle of hospital politics as they will need OR time and a bed in ICU. They cannot book any of these until we have results and have made a decision.

We did have a chance to discuss the procedure. Our doctor has been working on gathering information and had us scheduled for the 3rd week of October had James' health not deteriorated. I have previously talked about a Whole Lung Lavage (WLL) (here and here), this is the procedure used for adults, but it is impossible to do on children under the age of 8 because of the size of their lungs and vocal box. They have researched different ways that they can do the procedure for James and have decided to do what would almost be called a Partial Lung Lavage. When they do a WLL they block off one lung and continue to feed it O2 so it can do all the lung worked needed in the body and proceed to clean the other lung out with saline water. They pour a litre at a time into the lung and then drain it out again, doing this about 20 times. This gets at the entire lung at the same time. Unfortunately with young children they cannot put this larger special tube down their throat in order to block off one lung and at the same time access the other lung. There are several different attempts that have been made on the one they feel will be our best chance is to use somewhat of the same concept but because they have to use different smaller tubing - the same as they use when doing a bronchoscopy (also explained here) (which James has had done a couple times already) - they will only be able to put small amounts of saline water in at a time and cannot do the whole lung this way, but instead have to slowly focus on each area. This means the procedure will take much longer. It has been reported to be effective with some and not with others. All we can do is try it and find out. The only good side to this procedure is that it will be somewhat less risky then a WLL, but it doesn't change the fact that while the clean the bad stuff out of his lungs they will also be cleaning the good stuff out and this can mean chances of lung collapse or failure. As mentioned before, they would do one lung, allow him to recupperate for a couple weeks and then do the other lung.

We are not at the end of the road if this does not work, they have learned about several different procedures and the last option would be to put his body on bypass, let the machines do the heart and lung work and then proceed to cleaning out the lungs. This is a last resort as it carries very high risks.

So at this point we have no further idea of what lies ahead of us. We continue to wait for the CT Scan which will help to guide us in the right direction. Since the Family Resource Room (where there is internet access) closes at 3pm that means that if anything changes I will not be able to give a further update until tomorrow. Thank you all for your continued thoughts, prayers and support.

Hospital Politics

2007-09-26T17:34:00.000-05:00

James will be going in to Toronto tomorrow morning via Emerg.

This morning I received a called from the booking clerk that they had an appointment for James for a CT Scan on Monday. When I questioned her about him being admitted to hospital afterwards, or what happened to it being in the next couple days she had no clue what I was talking about. I asked her to please look into things for me.

Half hour later the Lung Specialists nurse called me to tell me they were still working on things. When I asked about the CT for Monday she had no idea I had been called about that and told me I should not have received a call as they felt Monday was too late and were working to try get it done tomorrow. They were still trying to get things organized but told me to expect to come in tomorrow, so we carried on with that expectation.

At the end of the day they called to say they were running into a few problems. CT could not get us an outpatient appointment for tomorrow (we would arrive as outpatients and be admitted once the results had been reviewed). The only way to get a CT Scan done immediately is as an inpatient. That meant getting us a bed, but there are no beds available. After several different attempts they were still getting nowhere.

That left one other options, for us to come to Emerg. Emergency patients have precedence and so we could get a bed that way. So the plan is to arrive at Emerg early tomorrow morning (we need to beat traffic). Our Lung Specialist is the Chest Doctor on call, so once we get thru the initial Emerg rig-a-ma-roll we'll be dealing with her. They will order the CT Scan and required blood work and we go from there. This is definitely the longer route but they did not feel they wanted to wait until Monday. We've never done the Toronto Emerg thing, so we'll see how long this takes in comparison to Mac. Mac usually took 12-16 hours to get a bed (with a few exceptions), so it will be a long day, especially considering we'll need to leave here at 5am.

We have no idea what tomorrow brings. We thank you for all your prayers, well-wishes, calls and thoughts. What a blessing it is to be surrounded by the Communion of Saints. We will update as soon as opportunity arises.

Please Pray

2007-09-25T14:53:00.000-05:00

Early today I thought I should update the blog since it has been over a week, but there wasn't really much to write about. Our biggest news was that after 7 months of not gaining any weight last week when I took James in he weighed 11kg (24lbs 4oz).

For 7 months James weighed 10.75 kg (23lbs 11oz), with a few variations, but sticking in that area. In one week he went from 10.75 kg to 11kg. This was not without lots of hard work, for us and James. We increased his daily requirement by 10%, but the biggest reason for this increase finally was that we were making sure he was actually getting the correct volume each day ... this after two months of using a pump that was completely inaccurate. James is once again hooked up almost 24 hours of the day in order to get his daily requirements. He has no time off during the day and at night he's finally disconnected when the last amount of the pre-measured volume is in him. Since he does not tolerate nighttime feeds well at all, this has meant sleepless nights as we turn the pump on and off, giving him breaks whenever he starts moaning and once he stops moaning starting it up again so we can get a bit more into him before he starts moaning again. We would love to not feed him at night, but in only a few days of getting the correct volume into him we seen little round cheeks and a healthier looking face ... and this time not because of prednisone. What a lovely and encouraging sight. As we look at the rest of him we are reminded that we have a long ways to go, as he still is far below the 5th percentile on the charts and his scrawny little body shows it. Although we're happy with this improvement we realize that we need to see it stay steadily (and hopefully increase) there before we'll be really excited.

Our lives have made a sudden turn again. We had thought that we would be more prepared for the next hospitalization, but it does not seem that James functions the way we would like. You would think we would learn....

Today I called Toronto to review with them how James is currently doing. A little over a week ago we visited the office and reported that he's usually on 0.5 to 1L of oxygen. In the last week he has jumped to 1-2L, often requiring 1.5 litres. We wondered at what point do we need to stop just maintaining him and start taking a closer look at what's going on. James' temperature remains higher then normal, with occasional, but not consistent fevers. His heart rate is more often in the 150's (normal is 70-110, for James normal is around 140) and he continues to vomit daily, usually 2-3 times. Personality wise James is still James, he has his low times and once he gets through it (usually after a vomit) he's happy and chipper, enjoying life as it has been set before him ... such a loveable child!!

The response we received from Toronto was that it's time to bring him in. They no longer want to wait the three months to evaluate if the treatment is working. We are not really sure what their plan is, but they will do some testing, including a CT Scan and from there a decision will have to be made, but the feeling is we'll have to prepare for a Whole Lung Lavage (WLL). Since they are admitting him and not doing this as outpatient I get the impression that if this is needed, they will proceed with it immediately.

We are waiting to here back about when to bring him in. They originally said tomorrow, but upon realizing how hectic this would be for us they said likely the next day. Regardless we have to be prepared to go once a bed is available. And so I must go prepare.

Please pray for us as we will likely be encountered with decisions in the next few days. Pray for the doctors and the work that they are doing. Pray for James that he may continue to endure all that is set before him and if it is His will may return home a healthier child. Pray that we may remember to put our full faith and trust in the Lord and not in the doctors, but see them only as instruments set before us by the Lord. Most of all pray that we may accept whatever happens as being the Lord's will.

Photo Time

2007-09-17T21:11:00.000-05:00

These pictures are not very clear. They were not taken on my good camera and for some reason although they view nice on the camera they are fuzzy once loaded into the computer.

These pictures are from Friday after our doctors visit in TO. James was in quite the mood. The first showing off his idea of food - seefood (admire food, but never mind eating food). Then suddenly he was in a very smoochy mood. He has spent much time in front of the camera and definitely knows how to ham it up. It was an enjoyable time with our precious boy. It is nice to have these times with just him away from the house (or hospital) and routine (if you can call it that).

Dressed up for Sunday. He would love to go to church with us and asks each week. Unfortunately that is just not an option right now.

A Decision Made

2007-09-17T19:41:00.000-05:00

Well the McMaster Registrars Office will close today without a visit from Rob.

After much weighing and praying we have decided that Rob will carry on with his schooling. It was not an easy decision and I must say we're not totally confident we made the right decision, but it's still the decision we've made. I'm sure there are going to be days we'll regret the decision but we continue to pray that he may indeed be able to complete at least this term and hopefully the entire year.

This means a few adjustments. Rob has been trying to help out around the house and quickly found himself fall behind already in the first week. This is the reason he felt he needed to either work for a year or drop a course. I was definitely not in favour of dropping a course. This would mean making that course up next semester or during the summer ... both only prolonging the agony. We've done summer school before and when he was done with it he said he'd never do it again. He works long enough hours, and then to have to go to school and find time to study inbetween there ... it just doesn't work. Rob's study hours are longer then his works hours, (but at least we get to see him at breakfast and supper time), now that we have decided to carry on with school it will mean he will no longer have time to help me out. Since I now have helping coming during the day and we are working on getting our night shift nursing figured out we felt that this was somewhat of an answer to our prayers. These two should help me to better balance the household. As well, Matthew is also getting older and less fussy. Life is always going to be busy and there will be many things we'd like to do but cannot, but as long as we can keep the basics running we'll manage.

With James being such a rare case we having nothing to go by when it comes to complications, hospitalizations, procedures etc. It would be hard to see Rob lose a year of school and him only be in hospital a short time. On the other hand, it will become very difficult if James does end up in hospital long-term again as Rob will not have time to visit and the extra stress and decisions will make it hard to focus on his studies. Although we can no longer be refunded, Rob can still drop out any time that he feels it is too much. We will cross that bridge if we encounter it.

More Decisions

2007-09-15T12:43:00.000-05:00

So some of you are wondering how our appointment went yesterday. Never have we looked forward to appointments as much as we do these Lung Specialist appointments and I'm so thankful that Rob comes with each time.

Yesterdays appointment did not really bring much in the way of surprises. What we heard is somewhat what we expected, although not necessarily what we wanted to hear. The doctor does not feel that the treatment is helping James as it should; She had expected better improvement by this stage. The only encouragement is that James' LDH levels continue to drop: July 16 - 3700 units per litre; August 20 - 1537; September 5 - 1220. We are not in the normal range yet (500-900), but getting much closer. The puzzle is: why are the levels dropping if the treatment doesn't seem to be working? Just because James is clinically struggling, does this mean the treatment is not working? Does he have to struggle through this to reach the better stage? Can we expect him to all of a sudden "pop out of the water"? How long should he struggle before it's too late and his body isn't able to go through the lavage? That and so many other questions remain looming.

The doctor still hopes that there will be a sudden breaththrough and turn-around stage. The other PAP case they had also had struggles with coughing up phelgm at first and then suddenly turned around. This child was older and did not have the vomitting issues, but likely this is because he/she was old enough to control this and just cough out the phelgm. By three months they seen a remarkable difference in this child. So far James does not look like he's heading in this direction. It becomes a questions of how much do you compare? This child turned around in three months ... what if James was to turn around in three and a half months and we don't wait long enough to find out?

Although the doctor says she hopes there will be a break through, she is not completely happy with how he has been doing and has decide to prepare for the next step.... a Whole Lung Lavage. I have previously written about this (click here to read full version) and will just copy and paste so you can get the general idea:

What if GM-CSF doesn't work?? Then we move to the more common treatment of Whole Lung Lavage (WLL). This is where they literally wash out the lungs. When they do this they keep one lung going while they wash the other one out ... with up to 50L (compared to 100ml in a bronchoscopy) of saline water. They allow you to recuperate for several days and then do the other lung. When reading on the internet it all sounds so simple but we are told there are great risks to doing this treatment as you wash the good with the bad and put the lungs under great strain for the process. Even anesthetics are difficult and require a anesthesis who is specifically trained to use double tubes to keep the lungs going seperately. For a normal adult this is a critical procedure and for James ... well it's very critical. They have never done this on someone so young and his lungs are not large enough for the equipment to even go down as they normally would. All she said is that if it came to that point, they would find a way to do it ... but this would be no small procedure. There is also a possibility that even if the GM-CSF treatment is working they will still need to do the WLL in order to get out the build-up that he has accumulated in the past year.

The current plan is to continue with the GM-CSF treatment for another month and then do another CT Scan and some specific blood work. On that day if we see that there is little to no improvement (or degression) then we need to proceed to the WLL. As the doctor believes that this is where we are heading, she is already in the process of getting this booked and it will likely happen within a couple weeks after the decision is made. In this we she that she is very pro-active. On initial diagnosis, she said that without treatment, James had a one year life expectancy. We are now two months into this year with little obvious progress. To give this treatment a fair trial, we should be administering the GM-CSF for three months. Her approach is that if after three months we do not see any improvement, we can immediately proceed with the WLL. NO messing around!

We did not get into great detail of what to expect if they do a WLL. She has already advised us of the risks involved in this procedure and that there is a chance of death. This is another reason why she is already getting prepared for the procedure. It is rare and has great risks. She will be contacting the Doctor in France who has done the procedure on a young child (the only other time that we're aware of) to get guidance. As well she has to find an anethesis who is able to meet the challanges of double intubation. She advised that they will do one lung and then if he is able the other a couple weeks later. She also told us that she expected he would be in hospital for a day after the procedure. We were surprised by this as almost all the information we have read about this have said it's about a five day hospital stay. That being said, we'll be expecting five days (or more) even if they say only a day ... they do not know James as well as we do!!

I have often said I did not know what I would do if confront with having to go through this procedure. Surprisingly at this time I am much more calm then I thought, but that is likely because a final decision and date has not been set. James has been through many procedures already and each time we realized that there was some risk to the anesthetics (more so as time went on), but never have we truly had to kiss him good-bye with the realization that there was a great chance it may be the last time we do so. We remind ourselves that if treatment is not working we have no other choice but to go forward. As hard is it will be that day we know that without the WLL, humanly speaking, his prognosis of surviving is slim, and we are just prolonging the inevitable. We can do nothing more then lean on our Heavenly Father for strength, and submitting to His plan.

With regards to the fevers James is having the Lung Specialist believes they are related to the treatment. Fevers have been reported in relation to the treatment and since James is going in and out of fevers (daily), and not having consistent fevers with high spikes, it is likely the treatment. They would still like blood work done to check his white cell count and see if it's up. If it is they may decide to give him a round of antibiotics.

We have been asked several times about a lung transplant. Although a transplant could be done it would likely not be an answer to the problem so it would only be a very last resort if they thought there was any chance it would help. They have tried this on one other LPI patient and the new lung developed PAP as well. They know little about PAP, what causes it or how it is related to LPI, but this shows that it develops because of some other issue(s) within the body.

So we will be confronted with a major decision next month. Likely the answer will easily come out of the tests, but if it is not an obvious answer we pray the Lord will guide us when the time comes to think clearly. At the same time we are confront with another major decision this weekend. Is it wise for Rob to continue with school with all this uncertainty, or postpone it for a year?

This decision has been laying heavingly on us the past week with this coming Monday being the deadline to drop out and be refunded. Rob has already felt the effects of the extra work involved in taking care of James. Until now Rob has been in charged of James at nights; this means little to no sleep as he is up almost hourly (and often more) with him throughout the night. This week we started with night nursing three nights a week to allow some rest. We hope to get more hours to allow for a couple more nights a week of assistance (won't get into the struggles we are having with that right now). Since I am getting little to no sleep just listen to the constant alarms going off I will likely start taking over James for the nights as Rob just ends up falling asleep at his desk right now. Unlike me, Rob is able to sleep through the monitor and alarms if he knows it's not his responsibilitiy (and sometimes when it is his) :) Matthew is at the stage where I can hopefully balance this, and if we move him into our room at night then hopefully Rob will wake up if he cries too loud because I'm busy with James. Needless to say I have been functioning on survival mode for almost 4 months now and this is only a temporary solution until we can get our nursing battles sorted out. This past week's nursing was nice, but it only made me more tired because my body suddenly realized what it's been missing out on.

Anyways, I was talking about school. James takes up a lot of our time and this has left Rob with little study time and a lot of responsibilities. How will he balance school and assisting with the care of James? We have introduced another change in our house this week to help in this area. We now have someone come in almost daily to help me out with anything and everything. This allows me to focus on taking care of James and hopefully also giving me more time with the other kids, not to point out trying to get some of those things done in the house that only I can do. So if James were to stay home and we have nursing and the extra help would Rob then be able to manage? Likely .. but what if James were in the hospital? Before he was at Mac and it was easy to pop in and out, but now James will be in Toronto. It seems inevitable that he will end up in hospital, it just a matter of how often, how long, how serious ...etc. There are so many factors to weigh out and it's always hard when you have a deadline. Should he just drop out, take an 8-5 job, and post-pone a year? What if that year turns into 2, 3, 4, etc? Should he forge ahead and hope for the best? Should he go part-time and do summer school in order to have his degree before next fall? How much of his responsibility as husband and father can he delegate out, in order to finish pre-seminary education? Sometimes it seems like the answer seems obvious and then it's not so clear.

Through all this, several people have offered to help in many ways, for which we are greatful. We also realize that consistantly having different helpers is in the long run more work and time consuming than if we do it ourself. Any employer can vouch for the expense training new employees can entail, due to inefficient productivity and screw ups. Because it is near impossible to establish a dependable schedule, its also unrealistic to spend all morning on the phone trying to find babysitters and what not else. For this we are optomistic that our new helper will take all the drudgery items off our agenda.

Well this blog entry has become plenty long and it is time for me to go back to work, and muddle through the many thoughts that continue to go through my head.

Orthopedics

2007-09-12T21:38:00.000-05:00

Todays visit was to the Orthopedic Surgeon. In March we seen Orthopedics regarding James' feet and the way he walks (see here for info on that appointment). At that time we were advised that James has Flexible Flatfeet and that they would monitor him every six months to see if it was improving.

So here it is six months later and we went back for another checkup. I had felt that James' feet were not getting better and to me it seemed his left foot was getting worse. She also picked up on this. She advised that if both feet were like his right foot she likely would tell us we didn't need to come back for another year, but since his left foot is worse and he seems to be doing more damage on it she would like to see us in six months again. At this time we have two options: do nothing or get an SMO brace. She said that at this time she did not think the brace was necessary, there is no proof that using the brace at an early age helps and there does not seem to be any disadvantage to waiting until a child is 3 or 4. She thought that since he has lots of other things going on we could leave it and have him rechecked in six months. At the same time, she said that if we have coverage (which I'm pretty sure we do through James' disability) that we could go ahead with a brace if we wanted and just have him wear it part-time.

With all that's going on with James I think we'll be waiting before we act on this one.

Genetics

2007-09-11T14:53:00.000-05:00

We have a few appointments this week, the most important being at Sick Kids with the Lung Specialist on Friday. Last week we did not think we would make it until this Friday and thought we would be seeing him back in hospital by the time the weekend came around. On Sunday we did a major change to his feeding and this seems to be helping.

We had been working James up to bolus feeds (meaning he'd be fed "meals" instead of continiously around the clock). The most obvious advantage to this would be he wouldn't be hooked up all the time. The other reason we preferred this is because it's a more normal way for the stomach to function, as well this would mean we wouldn't be feeding him at night, which his body does not seem tolerate all that well. We were at the point where James was getting 120ml/hr, which is closer to James' old bolus rate from before his hospitalization ... but James started vomitting more often. Slowly he increase to three vomits per day, each vomit still started with gagging and throwing up phlegm, but then proceeded to throwing up formula as well. We were measuring 110-120ml each time he threw up. So here he was losing 200-300ml each day of the precious food we were putting into him. This on top of his 6-8 diarrhea diapers. And to make matters worse we discovered that our feeding pump was completely inaccurate all this time, meaning James could be losing anywhere from 100-250 ml of feed each day that way. So it's no wonder we were seeing our boy waste away.

When being fed at such a slow rate his body should be digesting the food about as fast as he gets it, but each vomit showed that he had at least 1 hours worth of feed in his stomach, probably more since he never reached the dry heaving stage. This concerned us and made us think that were were over-pumping him - what he used to tolerate he no longer can. So we closed the idea of bolus feeds and dropped him back down to slow 65 ml/hr continous 24 hour feeding. Day #1 - 3 vomits but only about 10-15 ml each; Day #2 - no vomits; Day #3 - 1 vomit of 30ml. Day #1 - wow ... new kids, the shine in his eyes the happiness, playing and chipper again; Day #2 - not quite as energetic, but still much better; Day #3 - not as bad as before, but not the greatest day (other factors also). Whatever the case we know that this is the better way to go. This unfortunately means nights are much rougher for us all. James does not tolerate nighttime feeding, it makes him "moan" (which is actually a funny way of breathing, we do not think it that he's in any major pain, altho could be some discomfort, it's very hard to tell) and causes him to deSAT continously throughout the night. He seems to be able to sleep through a fair amount of it ... unfortunately we are not so lucky, especially Rob who is mostly in charge of James at nights. Since he needs every ml we can get in him we spend the entire night turning the pump on and off, soon as he settles for a bit we try pumping again for a while until the next moaning, groaning, deSAT or coughing fit. But, our efforts may pay off, after only a few days we see James face fill out, he has cheeks again, and hopefully more weight will add on as we keep struggling along.

Although this change has eased some issues, others still remain, the biggest being fevers once again. Likely a sign of a secondary infection, I'm guessing they'll be putting him on a round of antibiotics to see if that helps ... more diarrhea, more sore bum ... poor guy. We also continue to see his oxygen levels swing all over and in general increase. These increased oxygen levels means he has a decreased activity level, not being very active. At the same time he's a bit more unhappy then he was a couple weeks ago. Everything just seems to be wearing him down.

But my title was Genetics, relating to our Genetics appointment day. Genetics deals with James actual disorder and mostly focuses on the protein end of James disorder. Since that is mostly controlled by diet they are the ones we go to for feeding issues. Much of the above issues were discussed today, but really there was no answer ... we have already tried all that can be thought of. Since we now know our pump is inaccuarte and seem to have minimized his vomitting, both working to make sure James gets the right amount of feed each day we hope to see some improvement in his blood work and weight (which wasn't all that great). They want to re-evaluate in a couple weeks and then go from there ... not that there are really a whole lot more options.

School Starts Again

2007-09-07T23:15:00.000-05:00

This week brings back routine for most households. Although I'm not so sure there will ever be a routine in our house anymore, there will be a bit more structure ... there has to be.

This year we have 3 students in our house. Marietta had her first day of Grade 2 on Tuesday, Rebecca her first day of Kindergarten on Wednesday, and Rob his first day of his third and last year of University on Thursday. Rebecca of course was the most excited. After two years of hearing Marietta talk about school she finally gets to go herself. She had so much fun on Wednesday that she complain all evening and the next day because she wants to go every day ... if only it would stay like that!!

Yes, as the picture shows, even though we live the closest to the school, this year we are receiving bus service, for which we are very thankful. There are days that James should not be outside unless absolutely necessar, even for the 15-20 minutes it takes to go to and from school. So it made it hard for me to pick up the kids from school and instead we are receiving bus service. The girls are thrilled about there little two minute drive, especially Rebecca!!

Little Matthew - 6 Weeks Old

2007-09-07T22:47:00.001-05:00

It won't be long and Matthew will be bigger then James :) Last week Matthew weighed 10lbs 4oz. Over the past weeks he's become an easier and more predicable baby. He has his fussy, I want to be held times in the morning and evening ... unfortunately the busiest and most hectic times of day, but these times are getting shorter, and since we expect them we've just gotten used to it and learn to work with them. He continues to be a frequent eater, especially during fussy times, often eating hourly, it's amazing how much he can pack away during those hours.

Unlike James, who has diarrhea, Matthew suffers from constipation ... the joys of being a formula fed baby. This means we have the occasional day where he's quiet miserable. When I seen James' pediatrician this week I asked her what to do and she suggested prune juice. I gave him a decent amount the first day and it worked, then I lowered it to more of a maintenance amount, but it looks like I have to increase the amount again as he seems backed up again. James' dietician suggested giving him low iron formula, which I had already started before she suggested even though most of what I read said it won't help. Hopefully we can get it under control so he's not in pain all the time. All considering he's a good baby!!

James (proud and loving big brother) and Matthew ... all dressed up for my brothers wedding.

1 Month Old

James' Current Health State

2007-09-07T21:57:00.000-05:00

Okay, so I get the hint ... I haven't updated for a while!! :) Before someone else asks, I figured I better try post something ... and believe me I've tried several times since my last entry, but have not been given the opportunity.

James has been home from hospital for 2 months now and it seems with each passing week things seem more complicated and he requires more and more care. We wish that James appeared as well as his blood work showed (by the way I got the correct # for his LDH levels and it was 1537 ... so bit higher then we were orginally told, but still half what it was before), but each day leaves us more discouraged. We continue to pray that all this is short-term and necessary in order to get his lungs back in shape. More and more we get this deep down feeling that he needs to have the Whole Lung Lavage (WLL) in order for us to make any progress. But he has a great risk of not making it through this procedure and so we remind ourselves to be patient. It's a matter of weighing out how long to be patient, if we wait too long his health will deteriorate more and more and make the procedure that much more risky. We are in constant pray as to what to do and we covet your prayers as well.

We look forward to next Friday when we see the Lung Specialist again, although we are beginning to wonder if we'll make it to next Friday. James' vomitting episodes have returned, but worse then before. Previously he would vomit in the mornings when waking, sort of like clockwork. It's not so simple anymore as he can vomit at any point and time. He is a very bright 2 year old and knows when it's coming, he is even able to hold it until a bowl arrives. If you visit our house you will find ice cream buckets pretty much in all rooms .. so far he has never missed the bowl, for which I am so very thankful. Sometimes it's a coughing episode that sets him off, other times it's because he's crying to hard, or even laughing to hard, and then there are times where it just comes. The last four days he has vomitted at least twice a day, each time vomitting up a fair amount of the precious food we put into him. Already a few weeks ago we realized that James had really become skin and bones and now this is definitely not helping the sitatuion. The only good thing about the vomits is that usually afterwards he perks up and is much more chipper, but that's after sitting with him for an hour or two because he's miserable and feeling awful.

Along with the return of the vomitting episodes we have begun to see fevers more often, they come and go and tylenol actually seems to help (unlike in the past) but they are a sign of more troubles. At the same time James' colitis is acting up and it seems we are constantly changing his diapers ... never mind his poor sore little bum. Night sweating (or nap sweating :) is a symptom of PAP, but lately he's even sweating when awake. Usually several times a night James will deSAT, often we find him "moaning" when this happens. It took us a bit to realize that he's not really moaning ... it sounds like moaning but it's actually the way he's breathing ... thus causing him to deSAT. He's done this moaning for a long time at nights, but lately it's getting much worse and now we're even hearing it during the day (which usually means a vomit is coming, but sometimes it takes an hour or two to come). A cough during the middle of the night sends us running because we never know if it's going to result in a vomit, a deSAT or nothing. James' oxygen requirements continue to bounce all over the place, leaving us checking him often to see how he's doing. We so far see no rhyme or reason to these ups and downs, each time we begin to see a pattern it suddenly changes again.

It's not good to write when you're discouraged, as this blog shows. So let me try to be more positive. When not moaning or asking to sit with us James continues to be a cheerful and easy going child, excepting everything as it is put before him. I stand in awe as I watch how well he tolerates everything, he truly amazes us. He is a child that is so easy to love for even when sick he is cooperative, it's like he truly understand that all that we do to him is for his good and not because we want to torture him. The simple things in life thrill him and make us appreciate all that we have.

These past weeks I have spent a lot of time on my butt, either sitting with Matthew or James, but I've learned to relax and not worry about the disasterous state my house is in or the work that didn't get done for the day because I know those moments are things I will never forget. Matthew is growing so fast and we don't know what the future holds for James, life would be absolutely miserable if I were alway uptight about that things that are not getting done. I've learn to be thankful for the few basic chores that get done each day. Not to say there aren't moments where I get so frustrate because there is so much to do and no time. Often we are up late at night because it's the only child-free time we have ... and often I don't know where to start because there is so much to be done ... (even a "normal" household can have these issues) ... it's learning not to let it get to me that has been the challenge. We continue to thank our Heavenly Father for each day He helps us get through, knowing that He is there helping us along the way, reminding ourselves that He does not set anything before us that we cannot handle and realizing that in order to handle these things we much seek and find our strength in Him alone.

Encouraging News

2007-08-27T20:30:00.000-05:00

Last Thursday I arrived home to find a positive message left on our answering machine by James' Lung Specialist.

Last Monday I had taken James in for blood work before he went for his J tube change. The Specialist called to say that the blood work looked very positive and so she was wondering how James was actually doing clinically. In the blood work they evaluated whether is seems that James' body is having any sort of reaction or negative side effect from the treatment. This all came back looking fine and so they feel it safe to continue with the treatment.

The part of the blood work that they were most excited about was James' LDH (Lactic acid dehydrogenase) levels. This is an enzyme that is important in energy production in cells. LDH levels are monitored with PAP, these levels are often high when a person has PAP. LDH levels can be high for many reason and in many diseases and therefore do not give any sort of diagnosis, but are monitored once a diagnosis is made and treatment is being done. James' LDH levels were high already when we were trying to diagnois him last year and this is why they did a bone marrow biopsy as that time. After he was diagnosed with LPI these high levels seemed to have been forgotten ... until I started reading about PAP and a couple sites said how these levels were usually elevated.

So the phone message said James' LDH levels had dropped and they were happy with what they were seeing, at the same time they were hoping that my report would be just as positive. Unfortunately I was not able to call back with a very positive report, as clinically James seems to be going downhill. When James came home from Sick Kids he was on 1/8L oxygen. We had a short period where he was able to go without oxygen but then he once again required it 24 hours. Slowly his requirement went up to 1/4, next to 1/2 and now we're seeing him on 3/4L more often (and since Thursday when I called to give my report we've had to put him on 1L a few times).

As I mentioned in my last post James' vomitting had pretty much stopped, but as I evaluated my notes closely I came to see that as his vomitting amounts decrease his oxgyen requirements increased, and since the vomitting had stopped it increased even more. So although it was very difficult to watch him choke and gag each day, this did seem to be doing something good for him. Since I called on Thursday we've begun to see small vomitting episodes in the morning again ... but his oxygen requirements have not been better on those days.

The other not so positive things were the fact that we've been seeing a rise in his temperature lately. Occasionally in fever area, but usually hanging just below that area ... but stil higher then his norm. Along with this we're seeing his BPM (breath per minute) go up from 60 to the 70-80 area. James is basically housebound as even a short trip outside seems to deSAT him and once he deSATs we have to sock the oyxgen to him for the rest of the day.

So from our prespective the last weeks have been discouraging, yet the doctor comes with encouraging news. When looking at Jame's LDH levels in July he was 3700. For his age the normal woudl be between 500-900. She did not have the results in front of her when I called but she was pretty sure that they were below 1000. This is pretty much back into the normal range and this was why they were so excited and gave us a call. We like to see these discouraging times as temporary. One theory can be that the treatment is working but since it is loosening everything up it is making it difficult for him to breath at this time .... but with time it will improve again as we get rid of all that sits in his lungs. We really have no idea, it's all a guessing came. This is just a theory and unfortunately we have no idea and so we just keep working with the situation and praying and praying that indeed things will improve.

The higher oxygen requirements mean another change in our house as we will need to change the way we get our oxygen. On low amounts we can just use smaller more portable tanks, but now we are going thru far too many and need to get a larger non-portable tank or an actual machine that converts the oxygen (not sure how that works, we'll see). Of course we'll still need the small tanks for when we go out etc, so we also have to continue to store these somewhere. We're left scratching our heads again wondering where we're gonna fit this change in our already congested house. We're often left wondering if it's time to move (as must as we rather not). We must be hitting the three year mark ... seems every three years we find ourselves dealing with this question.

I should just update on the G-tube situation. It's going well and we're glad we made the change. James continues to pick at foods. He is not eating much, but he's showing interest, trying foods, eating a few bites here and there ... all very postive after not touching a thing for two months. At this time we don't expect him to eat much as we are still "stretching" his stomach (not that we really ever expect him to eat much as he never did before either). After two months of not using his stomach it would have shrunk and so as we slowly increase the rate we feed him at we also increase his stomach size back to what it was. As we increase the rate we give him more time off the pump, which right now we mostly do at night so that his stomach is empty in the morning in case he vomits. As we suspected the vomitting is related to his lungs and was not due to the j-tube.

Coincidence??

2007-08-20T22:29:00.000-05:00

Today James went in to have his J-tube removed and a G-tube put back in. I must say I was dreading this appointment but it went much better then expected.

After having tried to pull the J-tube ourselves and being unsuccessful I was worried about how much it would hurt James to get it out. It seems that we just needed to be a bit braver and tug a bit harder and we would have had it out on our own. Once the balloon was deflated it bunched up a bit at the bottom and that's why we had trouble getting it out, a good tug and it was through. Radiology did use a guide wire when taking it out, we're guessing this was to help prevent any sort of shifting of his stomach while it was being done. Whatever the case we're now back to a g-tube ... again our nice Mic-Key Button kind. Upon looking at the size of the tube that they withdrew from James' stomach it's no wonder the child was moaning and complaning, it was much larger then the first j-tube they put in ... quite large for such a little guy.

A couple hours later James asked for a cracker from his diaper bag and then proceeded to actually put it in his mouth ... not just a lick, not just one bite ... he ate half the cracker. A hour later he sat down for snack with the girls and ate a few rice krispies and drank some juice. At supper time he asked for Ranch Salad dressing, licking the lid when finished pouring it and judging from what was on his face he also licked up some of what he put on his plate. He had some rice, eating one piece at a time ... only amounting to maybe 10 little pieces, but they went in his mouth and he didn't spit them out again. To the average person this means nothing, but to us it's a big step for a boy who we flood with praise when he takes a tiny nibble or lick of something. All this in one afternoon after two months of refusing to touch food, only occasionally licking something and seldom swallowing it, with the occasional sips of juice. Coincidence?? Only time will tell.

We continue to remain thankful for the surgical backlog when we were in hospital. If there had not been a backlog James would of ended up with a fundoplication instead of just a j-tube. He went through alot to have the j-tube placed, but it was a reversible treatment ... the fundoplication would not have been. Time and again we are reminded of how good it is that this surgery did not happen ... especially in light of James' daily vomitting, which a fundo would make very difficult to almost impossible, never mind how painful. Only with time will we be able to tell if the move back to a g-tube was a good move, but we are quite confident that we've made the right decision.

Speaking of vomitting ... for over a week now James is no longer vomitting daily in the mornings. We have had several vomitting episodes. A couple in the late evening after he receives his last medication for the day (on a bright note when given it tonight he didn't even moan, cry or move ... something he always did before). We're not sure what brings on the late night episodes. A few other episodes happened after he got overly worked up, from being hurt or disciplined. But it seems that this situation is getting better ... of course we were never really able to determine if it was a good or bad thing ... good because he's getting rid of the stuff in his lungs ... bad because, well, why was it happening?

And now I must get to bed for some of that much desired and often lacking sleep.

Nothing New!

2007-08-15T22:43:00.000-05:00

Not much new to report from here. We packed in five appointments in three days so far this week ... so hopefully we can enjoy the next two days off! :)

Only one of those appointments was for James and it was nothing major, just a pediatrician appointment, mostly just to review what's been going on as she doesn't get the information as quickly now that it has to come from Toronto. We have decided to pull the J-tube and go back to a G-tube and she was in agreement with our decision. For three nights we did not feed James and he slept peacefully. He did call us once or twice, but for no reason other then to make sure we're still around. The J-tube is not sitting well with him and by stopping the night feeds we are losing more feeds then what he might vomit up if on G-tube feeds. At the same time we will likely go back to bolus feeding instead of continous feeding. This means that we would give James four "meals" a day instead of having the pump run most of the day. Since James cannot tolerate his feeds too quickly that would mean that each "meal" will take approximately 1 hour and 45 minutes to pump into him. Previously we were unable to get the full volume that James required into him by doing bolus feeds, but at one point he was growing okay on what we were giving him so there wasn't much concern to meet the required volume. Since James is no longer growing full volume has now become a priority and we have worked to concentrate his formula so we can get the proper nutrition into him and at the same time keep him properly hydrated. We hope that he can go back to the bolus feeds as he had as this in our opinion is a more normal and natural way for a persons body to function. We have never cared for the idea of continous feeds because it just didn't seem proper to feed a person around the clock ... but then nothing with James (or most g-tube people) is normal so we had to give in when continous feeds seemed the only way to help him grow.

So that being said James is scheduled to have the tube change done on Monday. From there we will see what his stomach can handle and work on his feeding schedule so we feed him as minimal as possible at nights ... allowing his gut to rest and hopefully preventing him from vomitting up feeds in the mornings. We will have to start on continuous feeds but hope to work towards bolus feeds over time.

For the past week James' vomitting has been somewhat better. He is either not vomitting or vomitting less, especially less bile. He wakes up with quite the froggy in his throat and sometimes vomits up phlegm, other time the froggy just goes away with time. We try to teach him to cough it out of the way, but he won't cough for us ... likely fear that it will cause him to vomit. The discouraging news is that he's been requiring more oxygen lately to keep him stable. It also seems that he really can't handle the outside air. We had been looking forward to the heat leaving and to fall coming ... but even yesterday on a rather cool and breezy day he still would deSAT on us if we didn't crank up the oxygen while he was out there.

But yes, didn't I say not much happening here ... and really not much, just appointments and regular hectic life of having four kids, one being a (sort-of) newborn. Matthew is now three weeks old and weighed in at 8lbs 10oz on Monday. Matthew is a medicore kind of baby. He does sleep throughout the day so we do have peaceful times, but when awake he's either eating or being held. He goes into panic mode and screams up a storm about 30 seconds after being put down ... we've got some training to do in that area ... for now his adoring sisters are quite happy to hold him and keep him quiet. Thankfully I have these helpers, otherwise I'd would be complaining more about him ...or just simply going crazy listening to his screaming. He continues to like to feed frequently, altho this has gotten somewhat better ... he has cluster feeds and then goes for 3 hours ... and we're even gotten some longer 7-8 hour nighttime stretches a few times. They grow up so fast so we're trying to remember to cherish this time and not let our upsidedown lives get in the way.

Not an uncommon sight in the evenings. Matthew loves his tummy and loves to snuggle on a persons chest or shoulder, especially daddy's. Rob likes it too .... it's an excuse to snooze away the evening without feeling like he wasn't doing anything :)

CT Scan and Lung Specialist Visit

2007-08-09T21:29:00.000-05:00

It's hard to say whether todays visit brought favourable results. I guess it's easier to say that they didn't bring unfavourable results.

James did relatively well for the CT Scan. He lay nice and still. He had to have his hands up over his head, but he did okay with this also. He cried throughout the scan, which made it a bit harder to get a good pictures, but they were happy with what they were able to get. We were very thankful that he was so cooperative and hopefully after a few times of doing this he will be more calm knowing that it won't hurt.

Because James' last CT Scan was done at Mac on a different system our doctor was not able to pull up the pictures of both scans and look at them side by side, instead she had to go by memory when reviewing them. She felt that todays imagine was about the same or maybe slightly better. Of course we would have liked to hear that there was an obvious improvement, but what we did hear today can still be considered positive. In March and June James had CT Scans done and the June CT showed significant progression of the PAP. This was over a three month period. So that fact that todays scan was not worse is considered positive. Another thing to consider is that previous CT's were done sedated and todays was done unsedated. Unsedated makes the pictures less clear because he will still have slight movements, and the fact that he was crying makes it even worse. So todays pictures may not be as clear and with that in mind could be better then we think.

Based on this we cannot say for sure that the GM-CSF Treatment is actually working, but at the same time we cannot say it is not working either. She felt it was still early and there is no reason why we can't carry on with the treatment. At the same time she felt it was too early to make a call to move on to doing a Whole Lung Lavage (this is where they wash out the lungs), especially considering the risks of this procedure, which she was clear in pointing out that these risks include death. If successful this treatment could be very beneficial, but at this time we are not out of time and options and so we do not need to move to such a dangerous procedure. The plan is to carry on with the treatment for another two months. They will re-evaluate each month, doing another CT Scan at the end of two months.

It is felt that James' vomitting is indeed related to his treatment. This in light of the fact that recent vomits have been more of a white foamy phelgm or phelgm chuncks and less bile. The foamy phlegm is characteristic to PAP. Likely he is throwing out the bile because he is heaving so hard. We discussed the G vs J tube. She still maintains that James does not need a J tube ... but (yes, there always seems to be a but to make decisions so much harder) ... our concern is that if we go back to a G tube he may start hurling out his feeds, thus losing precious calaries. On that note, I weighed James yesterday and much to our disappointment he has not gained any weight but is still 10.7kg (23lbs 9oz). Today we measured James and found he has growned 3 cm and is 86.5cm. So in light of the fact that he is still not gaining weight we really can't afford to lose any of his feeds from him throwing up. As well, throwing up formula each day can get a bit hard on the esphogis. She felt that this concern about vomitting was valid but at the same time we have to bear in mind the moaning and groaning he does and how the tube is effecting him. She left it in our hands to decide what we would like to do, suggesting that it may be a good idea to wait another month before we pull the j-tube and hopefully by then the vomitting has settled a bit.

The J-tube can come out but we could end up with bigger vomits then. At the same time, each night we turn off James' pump for a period of time because he is moaning and so we are losing feeding time each day because of this. We will have to do some thinking and evaluating to decide if there is a way we change James' current feeding pattern to allow us to stop feeding him part of the night and so allow his stomach to digest the food in it before he wakes in the morning and throws up. The G-tube has the advantage of allowing more feeding flexibility, as well we were able to feed him at a faster rate allowing him more time off his feeds each day, which would mean we'd have more flexibility for taking him off feeds at night. Previously we strived to give him as many hours as possible off his feeds during the day because then he could run and play, but now that his is hooked up to oxgyen pretty much 24 hours of the day it really makes no difference if we feed him all day as well. We'll have to do some thinking over the next few day ... just never seems to be any straight forward answers around here.

We remain thankful that we did not receive a negative report today and continue to pray that the next couple months will bring a clearer and even more positive result. We continue to look to Him who is in control of all things, knowing that James is in His care.