The Lord's Blessings

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

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

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!

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

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

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

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

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??

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

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

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

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

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

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

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

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

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

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

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

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

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.