Family Reunion

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

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

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

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

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

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).

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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

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

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

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

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

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

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

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.