Pictures - It's been a while!

This was James a few minutes before he pulled out his first J-tube. He was having great fun under the car fixing it ... just like daddy does.

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Thursday at his appointment. Althought feeling awful after his nap he so badly wanted to play with the toys ... such a trooper!!
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James new bed and room. Behind me is the bathroom (a full bathroom - tub and shower) and my sleeping area, as well as some proper storage area too.

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GM-CSF Treatment inhaled by mouth. As you can see he's quite cooperative ... altho looking very sad and will say/ask every minute or so "all done??"

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Pretty ironic that we make him breath the stuff in and all those around him wear masks just in case it's toxic to us ... the precaution you have to take with unknown products. We remind ourselves that our options are limited and we reallyhave no choice ... if no one dares take the risk we will never know if it's safe.

Please excuse my extra .'s ... I can't get blogger to cooperate and put my pictures to the left as requests ... and really don't have time to try figure it out ... this works for now! :)

PAP Treatment

Most of Friday was spent quietly in our room, wondering where the doctors were, but the answers came late in the afternoon. She had not forgotten about us but was actually very hard at work on our case. Our “new “ Lung Specialist is going on holidays (just our luck) and wanted to get everything together before she left, as well, she is the one who knows most about PAP here and so she felt it was easier to do all the paper work, etc herself then get someone else to do it. James’ medication is VERY expensive, as well it is still on trial, especially the way that they will be giving it to him. So first they have to receive approval from Health Canada to actually go forward with the treatment. Proof that it is needed and should be effective is required, lots of paperwork etc in order to get it approved and then she had to actually order the medication, which comes from Seattle. But she got it all done, approval was made, medication order and it arrived by the evening … amazing really!!! Approval also means that we’ve been approved for coverage and can take the medication (and equipment to run it) home if it shows to be effective after a week. Originally she had said that we would need to prove after one week that it was effective to get home coverage, but now she said that it’s all covered for us to take it home … if we do indeed see positive results How long have we worked to get oxygen approval and monitors (which we still haven’t gotten a fixed price on)? And in one day such a rare and expensive medication is approved.

The doctor sat down with me today to talk in more detail. The meeting brought reality and new light to James’ disorder. Much of the information we have read on PAP is with regards to auto-immune PAP and this is more treatable with better outcome then the PAP that James is thought to have …since most of our research was based on this as it is more common, it showed a pretty good prognosis for patients with PAP, with it not being uncommon to go into remission. So far records of the LPI related PAP show that it does not go into remission and treatment effectiveness is not well known yet. I was once asked if people die from LPI and I had responded that if diagnosed and treated it is manageable and that the only deaths I have read about related to LPI have all been because of PAP … at the time I had no idea that James had lung issues to this extent. They cannot give us any idea of the prognosis for James. We are a trial and only the Lord knows what direction things will go.

As for James condition she classified it as moderately severe. I think here we can see the difference of someone who is looking at the test results versus someone who looks at James’ clinically. This is the issue we ran into at Mac, we always heard “clinically he looks well” and they seemed to focus on this more then what the tests were actually showing, always expressing surprise at what they found when they did tests. We were also advised that not treating James is not an option (technically it’s always an option), she estimated he would have 1-3 years if we went this route. So although James is a trial and we do not know the long term effects of the medication we are using … we have no other option. The doctors may be in control of the medication we administer, but we know that there is no better medicine then prayer and faith and trust in our Lord who is ultimately in control.

Surfactant produced in the lungs is normally either recycled by lung cells or destroyed (catabolized) by alveolar macrophages in the lungs. GM-CSF (Granulocyte-Macrophage Colony-Stimulating Factor) is required to stimulate surfactant catabolism and other functions in these cells. In PAP, very high levels of antibodies against GM-CSF are present in the blood and lungs and completely neutralize the activity of GM-CSF. Without GM-CSF-stimulation, alveolar macrophages are unable to breakdown surfactant. Consequently, surfactant accumulates progressively leading to impaired oxygen absorption in the lungs and symptoms.

I talked about treatments before , but more specifically the Whole Lung Lavage as this is the most common treatment. We had read about the experimental GM-CSF treament but did not really think this was something that would be used ... I guess we should have realized in a hospital like Sick Kids that's where you would use these new treatments.

The current treatment plan is to try GM-CSF Treatment (also know as sargramostim or leukine) for a week and see if James improves. By improvement we mean his breathing and SATs and they will look at specific blood work related to your oxygen levels. If James improves they will allow him to go home and we will continue the treatment at home. After about a month they will do a repeat CT Scan to get a visual idea of the improvement and a better picture of whether he is indeed getting better. Long term if it works they will likely keep him on the treatment for about a year and then test him to see how he does off of it.

All sounds simple ... but this is a trial. They have never done this in an LPI case and have only used the inhaled method they are using on James once before in a child. We have no idea if the treatment will work. GM-CSF is used in other disorders as well as in cancer, but this is done by IV. The method we are using is new, making James an even bigger guinea pig. Trials have shown inhaling the medication is more effective as it gets to the source quicker.

Originally they said he would use a mask to inhale it, but when he actually had the treatment they used a mouth piece. He had to hold the mouth piece between his lips for about 15 minutes. The doctor explain that when using a mask they get about 5% of the GM-CSF because our nose acts as a filter, when breathing in it by the mouth he gets about 20% ... much more effective. When they came with the mouth peice I thought they were nuts to expect him to keep it in his mouth, but our little James often suprises us and he is much more cooperative with this then he ever would have been with a mask (he goes beserk when he sees masks ... especially since being on BiPaP). He did not like the idea at first and it took a minute or two to get him to cooperate, then he started gagging and vomitting, likely because he was so worked up. But after throwing up he settled down and sat good with it. He has had two treatments so far - Friday night and this morning. Again this morning he got a bit worked up at first, but settled down quicker and sat there nicely. The doctor came during his treatment and was impressed with how good he was.

What is 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 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 long term prognisis? Absolutely no idea. She explained that James is very sick and wants us to know ahead of time that there is just as much chance they cannot make him better as there is that they can make him better ... they really don't know what to tell us, but only that we can be thankful there are treatments available. She said if we had come to them about 6 years ago they probably wouldn't have known what to do with him ...we have options now and all we can do is pray that they will work.

A few other factors which remind us of our "wasted" time at Mac. The Lung Specialist says that James does not have aspiration pneumonia (which our old specialist insisted he did) and that the lipids they seen to give this diagnosis are PAP related. She explained that this is the difference on having a adult specialist (as ours was) and a child specialist. She said children who are mentally and physically fine, as James is, do not get aspiration pneumonia - she has never seen it - because they are able to swallow and function properly ... as we know James can ... and therefore know how to use their coughing reflux etc to control any reflux. James likely does have reflux but it is probably mild. This is not uncommon in children, especially those with lung problems. She explained that when the lungs are under stress they also put extra stress on the esophagis and food track therefore causing some reflux. So if we get the lungs relaxed and under control his reflux should also settle. But ... the reflux that he has is likely mild and not really a factor. So ... in other words ... all we went through to put a J-tube in was not necessary, and we are extremely thankful that they were not able to do the fundoplication surgery right away. There is no reason to remove the J-tube since it's in now and won't hurt, but should it come out we could go back to a g-tube and likely just using reflux medicine (Losec) will keep the amount of reflux he probably has under control. Those are things to worry about in the future, one thing at a time. First we try get the lungs under control then we worry about how extensive his reflux really is.

Although I say "wasted" time at Mac, it wasn't all wasted and I do realize they were doing the best they could. Our frustration remains mostly with the Lung Specialist who never showed up and never took the time to really listen and for some reason with all the proof in front of him refused to see that this was PAP and James needed further help. He had said we would take a wait and see approach ... we are now told that waiting could mean James' life. Our time was not completely wasted either as no further tests were needed to be done at Sick Kids, we could move straight to treatment. We still love Mac, we just don't care for the Lung Specialist (no matter how positive the nurses talk about him) ... we will gladly drive the distance to Toronto each time again to have a doctor who listens and talks to us at our level!!

As for James himself ... the last couple days he has not been happy. There has not been the shine in his eyes that there usually is. He badly wants to go home, more so then I've ever seen him ask at Mac. I have to remind myself that just because I think of it as just another hospital room, to him it is a new surround and usually it takes him a few days to adapt with each admission to Mac, so he needs to adapt here too. Today he started to perk up again and we were able to wean him down a bit more on his oxygen ... he is now okay at just under .5L and they were attempting .25L this afternoon.

It's been an emotional few days, especially Friday when we realized that just because we got the diagnosis didn't mean we were on the road to recovery ...our reading of PAP did not prepare us for this prognosis. We pray that recovery is the case, but know that ultimately the Lord will decide ... and so we pray for strength to accept whatever is put before us in the days, weeks, months or years ahead. I look at James in a new manner now ... our happy, cheerful, energetic little guy who is such a little charmer ... oh how much he has gone through and still has to go through ... and all the what-if's that automatically come as a parent. I hope that he continues to improve the next couple days so that I too may feel more relax and happy ... happiness is contagious, James has a way of bringing that out in people.

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

Thursday - The Detailed Version :)

Rob has taken the time to give a quick update on what has happened with our much anticipated visit to Toronto on Thursday. We all know how long winded I can get, (more for my own benefit in the future, then for everyone else to know all the details), so some of what I will have to say will be a repeat of Rob’s update.

Wednesday night I went home for a lovely sleep in my own bed, at that time thinking we would be coming home for good Friday … little did I know it would be the last time I would see home and my bed for some time.

Thursday morning I had to be up and back at the hospital early for an OB appointment … the third one since our admission to Mac, been rather handy being so close … as I am quickly finding out now that I am further away. As is usual for this time of my pregnancy I was measuring small for my dates and so I will need an ultrasound done next week. With two of my pregnancies this has been due to placenta insufficiency, showing up around 36 weeks, one other pregnancy it was questionable but I went into labour before they ever got around to an ultrasound, and with one pregnancy I did not even make it this far before placenta insufficiency and severe toxemia appeared. At the same time I have been experiencing some higher blood pressure measurements, although not consistently and not in the concerning levels yet … just higher then normal for me. So we know there is a concern and pray that at this time it is just because I carry small and stress has created these higher levels … relax Steph :)

After my appointment I went to find James and Rob. James was getting ready to go and was very excited to go in the van, but yet seemed more mellow and different then normal. His SAT levels were low, dropping into the 80’s. We worked with several different probes and positions until we got it to read low 90’s, they charted that amount and then we got ready to go. We realized upon leaving that James was not James. He was SATing low for daytime, his personality was not himself, he didn’t even want to go walking or play in the child-height sink in the family bathroom (a place he’d love to hang out for hours if mom let him) … definitely something was off. But at the same time, we desperately wanted to get to Toronto, and knowing that we could get oxygen and all the help we needed there we proceed to go.

We did not put his monitor on during the ride over, but he was very quiet, not even pointing out the trucks. Upon arriving we found his SATs in the mid 80’s and not picking up. We registered and then went for x-rays, when we arrived back at the clinic he was SATing low 80’s sometimes even 70’s with his heart rate up between 170-190 (normal is between 70-110), plus he had a fever (something we have not seen since his admission in May). We asked at the desk if they had a spot checking machine that we could check how accurate our machine was. From there we were brought in and checked and their machine was saying the same as ours. He was moved to a room with oxygen and quickly started on 1.5 L (normally he only requires 100 ml). He had not required this much oxygen since his weaning days afer coming out of PICU.

So that is how James was introduced to the Resident. He went from plain miserable to falling asleep on my lap while they examined him … definitely not James. The lack of oxygen had exhausted him. By time the resident went through everything the oxygen had helped enough that James woke again and played quietly, but still very mellow for James.

The doctor arrived and without hesitation told us she believed James has PAP (Pulmonary Alveolar Proteinosis). She said all evidence – symptoms, CT Scan and bronchoscopy results – point to this. The reason the bronchoscopy results were not conclusive was because they claimed the substance removed from James lungs was not the classic appearance of PAP. She spoke to our Lung Specialist before seeing us and he told her it was a milky substance, which is exactly what PAP looks like. She said she didn’t know why they said it wasn’t classic appearance but she didn’t know of anything else that appears like this. (As a side note, we brought the records and information with us from Mac, before leaving Mac we took the time to photocopy the written information and had already read exactly what she told us – the stains were positive except it didn’t look classic … which already frustrated us … knowing that the results had been positive and it was only the look that made it inconclusive and yet our Lung Specialist had insisted it was not PAP but Aspiration Pneumonia.) Our “new” Lung Specialist proceed to outline our treatment options, advising that they were arranging for a bed for James as he was in no condition to go back. She did say that treatment could be done in Hamilton, and she had already spoken to our “old” Lung Specialist who said he was willing to do it … but we’ve been there long enough and know him well enough that there was no way we were going back, we struggled to get here and here we were staying.

Our visit with the actual Lung Specialist was not very long, we had already taken a couple hours of their time and were going to be checked in so more details and questions could be answered later. She made sure she went over the basics and explain our treatment options and then proceed with her clinic appointments and left the rest to her staff to get us set up in a room, which actually did not take very long.

Thursday brought some emotional up and downs. We’ve got what we wanted … a visit to Sick Kids, a second opinion, clearer answers. The hard part comes once again when we thought we were at the end the road and here we’re only at the beginning again. The realization of how much time was “wasted” (not all, but lots) at Mac … and the frustration of the fact that if they had just listened when we first asked to come here we could have been a whole lot further a whole lot faster. But we can’t undo the past and so we must deal with the future. It was a day where I found out that I will only have 4 more weeks before a new arrival in the family due to them deciding another c-section is necessary (I was actually hoping to go overdue for once, although not really expecting it)… and at the same time finding our son in a hospital further from home for a minimum of one week possibly weeks. How I miss home and the girls and just the regular ups and downs of everyday life. I am thankful the girls are handling things so well, but how much longer can they carry on not really having a home?? And when they finally arrive home will anything be the same?

Sick Kids

Today's visit to Sick Kids turned out a little different than we anticipated. James went in as an outpatient, and is currently an in-patient. He picked up a mild fever, and de-SAT-ed into the low 80's as the morning progressed. This warranted Sick Kids to admit him to hospital, realizing that he was in no shape for a ride back home. However, the lung specialists had seen different cases of PAP before, and could unequivocally declare that James had PAP, and a lung biopsy was not required. She laid out two treatment plans. The first is "simply" inhaling a vapour which will cause the excessive surfactant to metabolize, or dissolve. The 2nd is to do a complete lung lavage. Although she has done complete lung lavages, and has all the equipment and skills to do so again, she admitted that this has never been done on a child James' age. Any complete lung lavage is a risky procedure, threatening the lung to collapse. The younger the patient, the greater the risk. So James needs to be on this vapour 2 times a day for 15 minute intervals, after which a week should show signs of improvement. If he shows improvement on this treatment plan, we may qualify to have a Government coverage for this expensive treatement, and we can treat him at home. As an in-patient, this is covered. We begin treatment on that note, crossing bridges as we encounter them. Whatever is in store for us, we are grateful that James is in gifted hands. Even though this brings on new trials of being further from home, our primary concern is get James onto the road of recovery. As much as we depend on the abilities of Doctors to help James, we must remind ourselves not to boast in "chariots or horses," but only in the LORD. (Psalm 20). Indeed, our help is in His Name. And that by Grace.

J-tube

Well it's been a few days since I've made it to the computer. There wasn't much to report. As we had expected, the j-tube and lung flush were NOT done on Monday. We did find it rather odd that they would last minute on a Friday say they were going to do it, especially when they had no time or doctor book and no room in PICU books. All this was left to be scheduled for Monday morning ... and we knew full well it would never happen, I would have been more suprised if it did then that it didn't. We were somewhat happy for the delay as there were too many unanswered questions.

The J-tube change was scheduled for Tuesday at 1pm and the Lung Specialist could not be there that day, so another bronchoscopy and flush were out of the question. Instead he ordered that the anesthetis do a small flush to get a sample from his lungs so we can see if there is any improvement in the bacteria we are treating ... or if anything new has surfaced.

We were not totally upset about not getting another lung flush done as we had a chance to speak to the Lung Specialist and found that he wasn't doing a more thorough flush, but was just taking another peek so see what had happened with his lungs in the last two weeks and reattempt another stain. Nice to know, but not necessary and we wonder how much we can keep subjecting his lungs to. Although the Lung Specialist did say that the stains "suggest" PAP he came back singing a bit of a different tune on Monday ... still insisting that he thinks James has aspiration pneumonia (inflammation in the lungs due to reflux/aspiration) and not PAP ... and here we thought we were finally make progress with him. We were begining to think our 2nd opinion from SickKids wasn't as necessary anymore, but now we're glad we're going. If anything, hopefully this specialist will talk "english" so we can understand everything, or more anyways.

Our Lung Specialists also said that even if James does have PAP he would not do a Whole Lung Lavage (WLL) at this time as he does not think his lungs are bad enough to do this. We also requested having a biopsy done via a bronchoscopy (called a transbronchial biopsy) as further research told us that PAP is usually confirmed this way and not through a Open Lung Biopsy. But he told us these biopsy usually come back insufficient and still insists on an Open Lung Biopsy. So for now we wait ... wait to see what Toronto says, wait to see how James does.

I asked our Genetic Doctor today what his opinion was, as I know he feels this is PAP. He said he has not had a chance to sit down with a pathologist to go over it, but had read that the results suggest but do not confirm PAP. He also said that the tests have been sent on to Toronto for a second evaluation ... hopefully something will come of this. He also questions how much is reflux versus PAP ... he's on the same track as us, but unfortunately not being a lung specialist he can't do much to change things.

So Tuesday was J-tube day ... but first Monday I had to chase down a few people regarding what exactly they were putting in. We had waiting around all day Thursday and Friday for Radiology to come talk to us, but it never happened. In the end they never did Monday either, I instead asked to have the g-tube nurse I knew contacted. She did come and although she didn't know a lot about J-tubes as there are not very common and usually done by radiology she was able to help. She spoke with radiology and came to tell us there was no tube the proper size for James except the same tube we had before. We had heard of having the tube stitched down, but also that this is not a very reliable answer either, unless it is a special tube designed to be stitched, and these aren't. We refused to have the same tube again, they would have to come up with another solution. So she went back to radiology and came back to say they had found a MicKey Button tube that was a bit bigger then his last MicKey, but should work. The next day 5 minutes before the procedure the radiologist came to talk to me and informed me that he did not think this tube would work. It would be fine for going into his intestine, but the little bit of extra size at the top could mean leakage problems. I had to make a decision, give it a try or have the original type put in, they had nothing else available.. Since the original was not an option I felt we would have to give this one a try and hope by filling the balloon a bit more it would not have leakage problems ... time will tell.

I am happy to report that all went very well for the procedure. They still had to use a lot of anesthetic to keep him under, but no problems like last time and he was done and awake within an hour. He woke up calm this time and remained calm, no screaming and crying and confusion. He spent the rest of the day staring at the TV and slept through the night. He did require to stay on oxygen afterwards, but we were able to take him off this morning. He remains mellow and so far has spent most of the day in bed watching TV, only spending 15 minutes in the playroom ... after a slow walk there ... definitely different then the active boy everyone is used to. He is slowly progressing towards full feeds and then will likely be on 24 hours feeds for a bit before we start seeing if he can tolerate less hours and have more daytime freedom. So far he is tolerating feeds well and things are going good.

After 5 1/2 weeks we are hoping to be discharged on Friday. Tomorrow is our big day to Toronto. We don't expect a whole lot to come of this visit but it is a foot in the door. The doctor made room for us to get in this quickly and has expressed interest in the case, knowing only the basics. We will be bring all the files and images with us, so the doctor will not have had time to review everything before hand and give us a clear idea of what he/she thinks. At the time of setting things up we also did not have the results from the bronchoscopy, altho the doctor was informed that it is believe James has PAP, so hopefully this means he/she has done the research or already knows something about PAP and will be able to provide us with some answers/ideas. James seems to be doing well enough that we will have a day pass to go to Toronto, if anything happens that he requires oxygen then they will do a OPT (Ontario Patient Transfer) via ambulance, but would prefer not to as they are not reliable on getting you there on time.

There have been several times where we thought we were almost ready to go home and something happened to change this. Please pray that all will continue to go well and this time we will truly find ourselves home as a family again soon.

Pulmonary Alveolar Proteinosis (PAP)

This afternoon we received the results of the bronchoscopy. The stains "suggest" that James does have PAP. By suggest we mean that there is evidence of PAP but it is not clear enough to give a confirmed diagnosis of PAP. To us this is a good as saying he has PAP, the symptoms lean towards it and now the evidence leans towards it. It is sort of like when he was diagnosed with LPI, the symptoms agreed with the findings in the urine but until the DNA results came back it could only be "assumed" he had LPI but not confirmed. In the case of his LPI it was obvious enough that they didn't doubt they had the right diagnosis ... in this case ... time will tell, but we see how the doctors all seemed to have thought PAP was a factor and now we have more proof that it is. From a doctors perspective we can under stand that he cannot truly label James as having PAP without having a very clear confirmed result.

Once again standing our ground has paid off, once again we were able to find something the doctors did not. I feel for those parents or even adults who believe that if the doctor says the doctor knows and so the doctor is right. We have learned over and over that if we pay close attention, evaluate things ourselves, do our own research, we can be just as helpful or more helpful in the treatment of our child. Sometimes it is hard to stand our ground when the doctors oppose what we say, but after several experiences of being right in the end we now are more confident and do not let the doctors intimidate us. If they truly believe we are wrong in our opinion then they will need to prove we are wrong, until they do we will not give up.

So what is Pulmonary Alveolar Proteinosis (PAP)?

PAP is a rare lung disorder in which the tiny air sacs of the lungs (alveoli) fill up with a secreted mateiral known as surfactant. Surfactant is normally necessary for the air sacs to remain open, however, patients with PAP accumulate excessive surfactant lipids (fat molecules) and proteins in the lungs, which impairs absorption of oxygen from the air and causes a feeling of breathlessness.

Treatment of PAP is whole lung lavage. In this procedure they wash the excess surfactant with large volumes of salt water. Usually this is done in two steps, first they wash the one lung, then allow a few days of recuperation, then they wash the other lung. How often this needs to be done is very individual to each case, but it usually has to be done repeatedly. James has already had two lavages, these were not the same as a whole lung lavage, but they were a washing of the lungs ... the second one more then the first. This was in a three month span and is probably why the doctor suggested that he would need it done every three months, considering the amount of fluid they found in each lung the second time. Research tells us everyone is different and some only need it a few times and then for no explained reason are okay, while other need it repeatly and can still progress and develop respitory failure or go as far as needing a lung transplant. With treatment the survival rate is approximately 95%.

Although our Lung Specialist has not been very useful at showing his face during this admission, I must admit that he does seem to be "coming around", being a bit more down to earth and more willing to listen and answer our questions ... he's learning :) Today he showed up for the first time since the bronchoscopy to see how things were going, he had not looked if there were any results yet, so our discussion was more "what if's" The one thing he said that was opposite to others we have talked to is that if we see no improvement with the J tube it was more reason to have the fundoplication done ... whereas other see it as reason not to have it done since it gives evidence that food is not coming back up from his stomach since the J doesn't put food in the stomach. I figured I wouldn't argue that one out all too much at this point, but deal with it as we see what the J tube does ... just another question we can ask in Toronto.

The other thing I questioned is if he were to show PAP would we need to do a whole lung lavage right away ... and if so, we want that coordinated with the J tube insertion. He said he could certainly arrange another lavage but the impression I get is that it would not be to the extent of what I read about the whole lung lavages. He feels that a whole lung lavage can also be risky because you are cleaning out all the surfactant, and surfactant isn't a bad thing, it just is when you have too much. In James' case we see trouble in the bottom of his lungs, but the tops areas are okay, so he would focus on just those parts, and by the sounds of it do both lungs at the same time. But he had no issue with doing a lavage again, even though we just did two weeks ago and also said they would send stains away again hoping to get a more definite answer ... we're seeing more cooperation on his part!!

After our conversation he left to check if there were any results and came back a few minutes later to tell us what they had found. Interesting enough he did say that the only way to get a definite answer is by a lung biopsy, but this time he did not push to have it done, instead he suggested a watch and see approach.

At 5:30 this afternoon we found out they hope to put the J tube in on Monday, but yet they have no time or confirmation of that ... this just before a weekend. So James has to be prepared for this (no food again) Monday morning. It's nice to have things done, but we're feeling a little rushed now. You can't get answers on the weekend and they haven't told us what time Monday ... are we talking 9am or 3pm kind of idea ... 9am does not allow time for answers, 3pm does. Radiology did not show up to discuss tube options today ... nobodies doing anything until we've had a clear discussion about the options. The nurse who specializes in the tubes was not in today and was asked to speak to us Monday ... will she be in before we go down? So I could be left trying to make a last minute decision or not sure that I have gotten all the options because I haven't talked to everyone.

Then there is the PAP issue, so the nurse went back to find out about that and came back to say that they'd make it a go ... all sounds like too many promising words last minute. Once again this will require a transfer under anesthetic, and the Lung Specialist had said he'd likely do it in PICU again. He also said it's possible James will be required to stay in PICU afterwards, both meaning a bed needs to be available. We have a better answer now and our research shows that the only treatment for PAP is lavage, so we don't have too many issues with this, but at the same time it would have been nice to have gone to Toronto firs ... just in case they bring any more insight. We're also not sure if too many lavages is in a row is good either. Just lots of questions that we hope to have answers in a "short" period of time (short - when we're talking the way hospitals operate).

So Monday could possibly be a busy day and I will admit I'm much more nervous about this anesthetic then ever in the past ... also knowing his lungs have not been great lately and wondering how long it will take to recuperate. We leave these things in the Lord's hand and pray for that he gives the doctors and James all they stand in need of. In the meantime we hope to enjoy a weekend at home as a family as James has been given day passes ... meaning he can leave when he wakes and come back at bedtime ... no waiting around for doctors rounds. It will be challenging for James tho as he will have to be hooked up to his feeds all day, but we'll make the best of it.

Two long updates in one day ... hopefully I'll have nothing more to report until Monday (or likely Tuesday by time I get around to it). We wish you all a good weekend.

Decisions

Yesterday was a day of much thought and prayer to come up with the most appropriate and ideal decisions and treatment. We've sort of been left stuck between a rock and a hard place, but after much evaluation seem to have come to some sort of decision.

After the Team Meeting the ball was put forward to start organizing things. The first thing they found was that they were not able to do an inpatient tranfer to Toronto as they had hoped. However we were able to get a outpatient appointment which is tenatively for next Thursday, but not yet written in stone. The advantage of an inpatient transfer would be that James' would actually become an inpatient in Toronto and would probably stay 1-2 days. At that time he would see the Lung Specialist and the Genetic Doctor. Then if they felt it would be beneficial to see any other doctors that would all be done at the same time ... and then he would be sent back to Mac for the different doctors to work together. Since this is not possible we are thankful that they were able to get us an outpatient appointment with the most important doctor, the Lung Specialist, so quickly. This appointment would be no different then a regular specialist consultation.

Surgery came to see us after the Team Meeting to inform us that a Fundoplication would not be able to be done immediately as they do not feel it qualifies for the add-on list. Right now they are booking into September. Their solution to the wait was to put another J tube in to tie us over in the meantime. Our first reaction was no way and so we sent them back to reevaluate their time line and also spoke with some of the other doctors ... but it seems there have been no beds in the hospital lately, many surgeries have had to be cancelled and so there is a major backlog of surgeries and this one just isn't important enough.

As we spend time evaluating this we realize that it may not be such a bad thing afterall. It would be great to have it done quickly, but we began to question the what if's ... mostly ... what if this surgery isn't really the answer. It is irreversible and sometimes has some irreversible side effects ... that would be lifelong. When tighting the esophagus you prevent food from coming up ... which can also prevent you from being able to burp and allow simple air out, causing bloating and other major discomforts. As well, if the esophagus is too tight people can have trouble swallowing. Those we have spoken to say they haven't seen these side effects in children and it may be more adult related, but regardless it's a risk.

We know that James has reflux, treatment of Losec helped this reflux, but we do not know that this is the biggest problem he has. What if we are trying to maximum the reflux treatment when it is really only a minimal problema nd something else is doing the damage? The endoscopy showed James only has mild esophagitis (burning of the esophagis) which suggest only mild reflux. The bronchoscopy shows lipids (fats) in his lungs which suggest food is getting into them, but your body also normally has some lipids in the lungs ... so what if it's more because his body isn't able to process the lipids that are in there? We do not doubt James has reflux, we're just not sure it's the only or the biggest problem ... so we want to be careful before we do a irreversible surgery.

So our decision? We are going to make another attempt at the J tube. We struggle with this decision because we wonder how many J tubes we'd have to put in before he reached the surgery date. The only way they can put another J tube in is if they make sure this one is anchored properly. We are suppose to see a couple different people about this today to review the options. So far we are told that there are some with balloons as well they can actually stitch the tube down on the side of his stomach. No matter what solution they come up there we could still run into trouble as J tubes clog easier then G-tubes ... not to point out we don't even know if James' body will tolerate the J tube as he didn't have it in long enough last time to give us a clear indication ... but we won't know until we try.

On the bright side we feel that maybe it is wiser to go this route because we can better evaluate how James' lungs do with the J tube. If we put a J tube in and his lungs get no better, or worse, we also know that a Fundoplication is not going to help ... thus saving an unnecessary surgery. If we see great improvement or stability ... we will feel more confident about doing the surgery. It will buy us some time to make a more informed decision ... we just pray the tube will stay in this time ... and for a long time.

We're still waiting for the bronchoscopy results which really hold us up in many ways. They keep saying they should be in soon and this will really help ... if something comes of the results. We've asked to proceed with the J but at the same time question whether we need to wait for the bronchoscopy results just in case we get a positive result for PAP. If it comes back saying he has PAP we question whether a whole lung lavage will be needed immediately to get his lungs on track ... therefore another sedation ... and can we coordinate the two sedation. A whole lung lavage is a very thorough washing of the lungs, doing one lung at time and taking a couple hours for each one. They usually do one lung and then wait several days (until stable) to do the other ... not a minor procedure either. So ... we'll see what they come up with, but unfortunately we can't keep sitting and sitting here either ... yet if we leave we know it will take forever to get a bed back ...and with a baby due soon we need some things done sooner then later.

This means another weekend here and likely another week ...altho we've stopped speculating on time but take it one day at a time. Thankfully they say we can go home all day Saturday and Sunday and do not need to stick around for rounds and doctors since nothing gets done on the weekends. We will need to come back at nights because James continues to require oxygen at nights and we haven't gotten our own for at home yet.

Just to clear up, since several people have asked. We qualify for home oxygen ... this is just the oxygen ... the monitor is not covered, we still have to purchase that and I believe we're also responsible for the "smaller" equipment (oxygen probes, nose prongs. etc). There is no way to get monitor coverage and very strict quidelines for oxygen coverage. The oxygen coverage is only valid for 3 months and then he will have to be retested again ... so we are waiting until we're sure we're discharge before getting it at home.

The Big Meeting

This morning James doctors' met together at 9am. They managed to get all the key doctors together, although not all could stay the entire meeting. So they had his Pediatrician, Lung Specialist, GI doctor, Genetic Doctor, Dietician, OT (Occupation Therapist) as well as the Team Doctor and Resident (those are the ones that he is under while an inpatient).

After they met I was able to meet with them once they had done their diiscussing and come to some sort of conclusion. The biggest problem is that we still do not have all the results of the bronchoscopy, thus leaving us to speculate on some important issues. It is hoped that these results will be in by the end of the week ... we won't hold our breath.

At this time is seems that the doctors are sort of expecting (or at least seem to be leaning toward the idea) that James does indeed have Pulmonary Alveolar Proteinosis (PAP), but yet they (as well as we) believe that aspiration/reflux is still a contributing factor to his problems. It is felt that maximum treatment needs to be done in order to stop this and help to restore/heal the lungs before they are damaged beyond repair.

It is felt that there is no point going back to a GJ tube (actually techincally James just had a J tube, altho everyone has been calling it a GJ - a GJ allows you to feed into the stomach and the intestines, a J only allows into the intestines). Although they said it is still our choice to go this route, they did not feel it would be a long term solution. This is partly because the J tube is hard to keep in place, altho there are better tubes then the one we had (why they didn't put a better one in remains an unanswered question), is known to clog more often or to coil up and come out of the intestine. So it's unpredictable how often we would need to replace it ... and considering James' track record with anesthetics we can't be doing this all the time. At the same time James did not have the J tube long enough to see if it was any benefit to him. What we do know is that it is not helping with his deSATing issues, yet it can still be useful to control reflux. We had just reached full feed requirements when the tube came out, what we noticed that day was more mucasy diapers and he got a rashy bum ... something he hasn't had since coming into hospital. This would not mean we'd rule it out as with James' mucas and red bums are part of his life ... but it's questionable when it's been going good for so long. The overall decision was to look at a more long term solution.

And what is a more long term solution? Fundoplication surgery. In this surgery they wrap a part of the stomach around the esophagus to strength the valve and help stop reflux. I was not surprised this came up as it had been suggested before as the last step ... but unfortunately I also have not had time to do any research on it, so I was not able to come with full and complete questions on this decision. The questions I did have they could not answer and said a surgeon will be better able to explain and answer my questions, all they really knew about was the results of the operation and claim that it is not an uncommon survery and is usually effect as well as long lasting ... altho it can loosen and be required to be done again. We will have to do some further research before making a clear decision ... altho it is apparent something needs to be done and our options are limited.

Together they are in the works of trying to arrange a patient transfer to Toronto Sick Kids for a second opinion. Didn't we ask for this about 3 weeks ago? At that time we were told it was not urgent enough to do as a patient transfer ... now there seems a change of opinion. So they were on the phone with Toronto this morning hoping to get an answer to if this is possible and when ... so far we haven't heard any answers. If they do manage to arrange it as an inpatient transfer then they hope to also arrange a visit with the Genetics Team there as well. Hopefully they'll be able to get something together so that we don't have to wait months for another opinion.

They have also told us that there is still a possibility we will be looking at an Open Lung Biopsy, but nothing further will be decided or pushed that way until we get the bronchoscopy results and a second opinion. If the bronchoscopy shows PAP then it will not be needed, but since the special "stain" they sent away to get the PAP result is very sensitive a negative result will not necessarily mean he does not have it ... and so they will want to do a biopsy to get clearer answers. We are praying the bronchoscopy will come through for us as this is no minor surgery, especially when factoring in James' anesthetic track record. They bring this to our attention now as they would like to combine this is a Fundoplication surgery if that is the route we have to go ... therefore one anesthetic and one surgery healing.

Treatment for PAP was briefly discussed as well, but obviously we'll get into that further if that is indeed what he has. The main treatment for this is called a whole lung lavage, which is basically a very, very thorough cleaning of the lungs ... and what does this mean ... more anesthetics. The Lung Specialists was suggesting he'd need this every 3 months ...altho our research showed every 6-12 months, sometimes less often, it's very hard to estimate. And hopefully if his reflux is under control the lungs have a better chance to fight this. But since nothing is confirmed yet we will not spend too much time dwelling on it.

It was also agreed that the prednisone does not seem to be helping and so we will be weaning James off of this medication ...yahoo!!! Since this is steroid you cannot just stop it immediately as it can have very adverse effects on the body, so it will be a slow process to get him off of it, but at least we're heading that way.

So ... I think I've covered it, or at least tried to in the little bit of time I have. Yesterday we did manage to go home for an LOA ... I was so tired my only goal was to sleep, but James had quite the opposite idea ... and so instead I wasted my time trying to get him to sleep so I could sleep and in the end neither of us slept!! James and I had an enjoyable visit with friends from the Chatham area in the evening ... thanks guys, the visit brightened our day ... to bad Rob wasn't home!!

Oxygen Coverage

Monday night I got smart and didn't bother fighting the deSATing issues for the night, he had deSATed enough by 11:30pm that we knew it wasn't worth readjusting him all night ... and so we stuffed the oxygen on and got some sleep for the night ... as much sleep as a person can get on a pull out bed in a hospital.

Tuesday we addressed the issues of oxygen again. We had thought the recent deSAT problems were only due to the anesthetic, but an x-ray done Tuesday morning showed his lungs were back to the same condition they were on June 6 (the most recent x-ray prior to the anesthetic) ... so the anesthetic does not explain why he is deSATing again after having a period of doing okay. There is no point in leaving the hospital to go home and have to move him all night long to try keep him in proper saturation ranges, so we requested to try again to qualify for oxygen coverage ... thus meaning we could go home since we have a loaner SATs monitor until they get straighted out the quotes for our own monitor.

In order to have coverage for home oxygen you have to deSAT below 88% for a full 2 minutes. The computer prints out the deSAT levels and then you have to continue to print out 3 full minutes of proper SATs using oxygen in order to prove that oxygen actually helps the situation. Previously we had gotten a record of him deSATing but had not realized we need the extra printout of him SATing properly. So our task for last night was to get a proper printout.

I knew it would be a bit of a challenge because he bounces back and forth pretty quickly. Well just our luck he spent the whole night SATing at 88% ... normally under 90% and we put him on oxygen ... but this time we needed lower yet. He would hang out in the 85% -88% area, but each time the level hit 88% that meant the printer stopped. Finally at 6:30 am I was ready to give up since I had gotten no sleep listening to the machine beep off all night and watching it whenever it beeped long enough. He had SATed low all night which meant he wouldn't be much good for the day, so I thought I'd just turn the oxygen on for the last hour or so of his sleep ... and wouldn't you know, just at that point he managed to stay low enough for the full 2 minutes, turn on the oxygen and finish printing the rest and we were done. Boy am I glad we don't have to go through another sleepless night trying to get that done.

So the paperwork is filled out and within the next day or so we should have our own oxygen for at home ... yahoo ... one more hurdle overcome!!

Now if they could just get things straighted out with ordering a monitor. The rep. advised that she sent an urgent message out to have this taken care of yesterday, so hopefully soon. It seems that the lower quote we got was directly from the monitor company, but in the meantime the sales person has changed and there seems to be some trouble getting the same quote back. Normally monitors are purchased through a Home Care company ... and they mark them up double the price, thus the difference in prices we were quoted ... all the same montiors, just depends on where you go. So patience will hopefully mean cheaper.

Two Days!!!

The GJ tube lasted TWO DAYS!! Two whole days ... that's it ... after all James went through!!

Sunday James was back to himself, happy and cheerful, playing nicely, waving and smiling to everyone ... it was so wonderful to see. Sunday evening as I chatted on the phone James decided he was going to do some mechanic repairs on his Little Tikes car .... he lay under the car and banged and clanged and was having a hay day ... then he decided he was done, and his tube happened to be stuck in the door ... out came James, out came the tube. James didn't flinch or realize what had happened, but I just took one looked and started to cry. He's been through a lot and I haven't cried since his first days in ICU ... but I cried this time. When will it ever end??

Why do we always learn lessons the hard way?? One look at the tube told me why it came out and that it would never have lasted ... I would never have agreed to a GJ tube if I had known there was no balloon, disk or bar to hold it in place. It was quite literally a tube and the only thing holding it in place was the tape that you can see on the picture I posted the other day. Nobody told us this ... they told us it was anchoring tape, but we were given the impression it was just for the healing process and that it would come off shortly. The tape had been a bit lose, James had been pulling at it because he was sweating, but I had no idea how important it was that it remain firmly fastened. No matter .... even if I had known I can tell you right now the tube wouldn't have lasted a whole lot longer. James is active and his g-tube often got tugs ... that's the whole point of having a balloon or disk in the inside ...to keep it in place.

Since then I've gone from being upset to frustrated that the radiologist actually thought that something like that was going to stay in the child. And it seems nobody knows anything about these tubes because the doctors and people we've talked to today had no idea that it was simply held in by tape and said they would go speak to the surgeon to see if anything else is available as surgeons often think different the radiologist. Everyone with their bright ideas but nobody making sure it's done right or that it will truly work.

Whatever the case, if they are going to put another GJ tube in it has to have a balloon or disk that will keep it firmly in place and it will have to be done without sedation or anesthestic. We refuse to allow them to put him under again ... pinning him down for ten minutes may be traumatizing, but not near as traumatizing 0r dangerous as all he went through for the sedation and anesthetic. Once again we've learned never to take things for granted, never to assume the doctors know what they're doing and to always asking every question, even when we think it's the simpliest and stupidest question.

So for the time being they've put an ugly g-tube back in James' hole (these tubes get uglier and longer every time) to keep it open and we've gone back to feeding him just during the day. He gets upset that he can never be disconnected, but even having him hooked up just when he is awake means he isn't getting enough formula in a day ... and his weight shows it as it was down again today. We've also come to realize that no nighttime feeds does not seem to help his deSATing issue, although with the sedation and anestetic throwing things off we do not know what's contributing to the worsening of this situation.

Saturday night he had to be moved quite a few times to keep from deSATing ... last night it seemed like I was readjusting him every 15-30 minutes ... needless to say I didn't get any sleep and he was awake often, altho he didn't seem none the worse come morning time ... bright and chipper like normal. Moving him makes his SATs come back up, but not long after they're down again, even if he's still laying in the same position that brought them back up ... so that trick no longer seems to be doing the trick ... and now we're questioning oxygen again. But this could all be sedation related also as we have not had a repeat x-ray done since Friday (we were suppose to today, but they never showed up) and every doctor I see says that the x-ray was awful and it's unbelievable that he doesn't need complete oxygen. But it's also been suggested that when it was taking he was having a spasm and it may not be near as bad as we think. Whatever the case he's having trouble with his SATs at night and his daytime SATs are not anything to write home about either.

And now we sit around with nothing happening again. The Team Meeting is Wednesday morning at 9am and at that time hopefully some decisions will be made. They are not doing anything about the tube right now, except researching what options are available for it ... so it looks like another week will be shot ... by time they have the meeting and then implement any bright ideas they come up with. We've also sent them back to question whether we need to keep James on prednisone ... it hasn't helped thus far and we would really like him off the drug if it isn't doing anything ... another question to be discussed at the meeting. The sad thing is that James actually called the hospital "home" the other day. And the times we took him home he actually wanted to go back ... I wasn't fast enough at getting ready to go. It's sad, but I suppose it's good too ... at least he doesn't hate it here.

I better go rescue James from the volunteer who's playing with him ... he doesn't like to be left for long, even tho they do the exact same things with him as I do.

This Weeks Pictures

The old Mic-Key button tube

The new GJ tube. I don't know the official name of this tube. This is also not what it will always look like. From what I understand the tape and small "box" are to anchor it for the time being. I haven't a change to talk to the right person to find out when we take that off.

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Thanks Dad for the use of your DVD player ... Thanks Laura for the use of your "truck" DVD. After we lost our private room this was a much better way to go then dragging one of the big huge TV's and VCR's into our tiny room each time he wanted to watch TV.

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Although he doesn't really eat much anymore ... at least not off the food cart ... James still can't wait for the cart to come. He asks for is by saying "coming, coming?" or stands at the door waiting. Then often doesn't even touch what they bring or wants my food instead.

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Lunch today! Usually by time the girls and I get home for lunch on Saturdays it's late and we're hungry and miserable, so this time I scrounged up some things and we had lunch together in the lounge area. Notice James who is busy stuffing his face and hardly could take the time to look up at the camera. This is the first he's actually eaten since his procedures (altho he's been offered lots) ... and he chowed it down ... guess he's rather sick of the lack of variety he gets off the cart ... I don't blame him.

Good-bye Beautiful Mic-Key G-Tube

After waiting all of 8 months for James' nice button Mic-Key G-tube we got to enjoy it for all of 8 weeks and now he has an even uglier long tube hanging off his belly again. It suddenly dawned on me that if I didn't show up with a Mic-Key tube on Thursday for his tube change then we would have to wait until the next time they changed the tube as this is not a tube that can be changed at home ... I'm usually more on the ball. I spent a good part of my time at home on Wednesday trying to see if I could get him a Mic-Key GJ tube, but there are only a couple places that deal in this type of medical equipment/supplies and a GJ tube is even less common then a G Tube, so nobody stocks them. In the end it didn't matter as the doctor who put the GJ tube in recommended that we wait another year before putting a Mic-Key. He said due to the design and size of James' bowel we would likely have clogging problems with the Mic-Key GJ. So ... at least whole year with big ugly tubes ... but, if this is going to help everything else it's not so bad afterall! :)

Thursday night James slept well ... except he needed oxygen again. By 11:30pm we had adjusted and moved him often enough to realize it wasn't helping and so we put the nose prongs back on. Before he was only taking 50-75ml of oxygen when hooked up ... this is really just a whiff of air and what they use on neonatal babies ... but it works for James. This time he needed 160ml ... still not really much, but more then what we've been getting by on. He slept after that and unfortunately woke up earlier then I had hoped ... but at least he had to go down early this time so I didn't have to distract him from food too long ... it being over 24 hours since he had eaten and absorbed only a small amount of formula (he threw up everything he ate yesterday afternoon, to the point of drive heaves) he was rather hungry.

They came for him just before 9 and of course he knew something was up, but remained relatively calm, although constantly asking me to confirm "no hurt". It's hard to answer that one honestly ... really he won't be hurt, he'll be asleep ... but since hurt to him seems to means any trauma then I'm lying to him. I held him while they gave him the initial sleep medicine through the IV, I like this way as he's still comfortable in my arms and not all worked up because they've taken him or made him lie somewhere. I left the room as they tried to stabalize him since he was kicken and squirming and trying to get off the bed ... doesn't this sound like what we went through a week ago.

The procedure is actually quick ... I feel asleep while waiting, but it took them about 45 minutes before they came out to confirm it was done. I didn't think too much of this timing, even though I knew that the actual procedure only takes about 10 minutes ... but they had to put him under so I expected it longer then just 10 minutes. The nurse came out to let me know that he was done, she told me there was absolutely no way we would even have been able to sedate him the day before as it took almost adult dose to keep him asleep. She said the anesthesist couldn't believe how much they had to give him. But then of course she rushed on to tell me that it was safe and they knew what they were doing, etc. etc. Regardless three times in a one week period of such high dose we remain completely thankful that our Heavenly Father watch over him and nothing happened to him (especially considering we know of a child who went for a simple procedure and because of the anesthetic never woke the same). I was then told I could go wait in the Post Anesthetic Unit until they came to get me, which shouldn't be long.

An hour and forty-five minutes later I was still waiting and becoming rather concerned ... maybe I misunderstood where I was suppose to wait ... but then I've waited here enough times I really knew that that's where I was suppose to be. They finally called and I found him sitting in bed quite calmly with oxygen on. They put a mask type on ... don't know how they managed that without him ripping it off. The first thing he asked when he seen me is if he could take it off and then proceeded to rip it off ... which they replaced with nasal prongs and he was fine with that. He kept asking to go and seemed completely fine ... boy was I glad to skip the screaming and kicking that he often does and what we had gone thru the day before.

I was then informed as to why it took them so long to call me, as well as why the actual procedure took long. When they first sedated James his SATs dropped into the 60's and so they had to work to get him back into line. Then when they went to bring him out of sedation and take his ventilator out he went into what seemed like a bronchial spasm (like an asthma attack) and so they had to resedate and ventilate him. His breathing went up, she said high, but I found out it was only in the 60's. When I told her that was normal for him she said "oh, wish you had been there to tell us" ... well ya, but you won't let us be there. I guess his chart says his normal breathing is 45 ... he does breath that, but normal is anywhere from 45-60 for him. Regardless they were concerned about his breathing and sent him for an x-ray which showed his left lung to be in rough shape. I'm not sure what made them decide to try again, but eventually they brought him out of sedation and he was fine with a bit of oxygen ... and that is how I found him.

I can see why they wouldn't want parents around for the entire procedure. I like to think I wouldn't panic too quickly, but dipping into the 60's and knowing they had trouble with the sedation makes me even more concerned about future sedations, which we know he can't avoid. A two year old receiving almost adult doses concerns us. Sometimes you rather not know things, but yet we realize that this is important to know so that we don't just take anesthetics lightly.

He made it back up to the ward as a "happy" child ... and then the fun began ... AGAIN. He didn't know whether he was coming or going, what he wanted, how he wanted it, why he wanted it, where he wanted it. He'd be crying and crying to eat but then would throw anything and everything we brought him ... and we brought lots of options. Once again he was wobbly on his feet, but he wanted to walk and play ... he would walk like a drunk and trip and fall, making me worried we'd have another accident ... but get oh so mad if he didn't get his way. Nothing made him happy ... especially because this time I refused to walk him for hours in the stroller and since he refused to go with a volunteer this time he just wasn't getting a ride. I finally got him to fall alseep after screaming and crying and throwing himself around. Unfortunately he didn't wake up a whole lot better ... for a bit he wasn't too bad, but as the night wore on he became worse again. Thankfully I had company and therefore an assistant later on and so together we walked and talked ... didn't make him sleep, but kept him quiet ... until we got back to the room. After some kicking, crying and throwing himself around he finally fell asleep.

Once again by 11:30 he was on oxygen, about 100ml this time. He didn't sleep as deeply and soundly either ... waking often, wanting me to hold his hand, or suddenly sitting up and asking for a drink, and even deSATed once while on oxygen, but readjusting helped that time. This morning he woke up more himself, altho after an hour or so I realized he wasn't completely back to himself, but this time somewhat more toleratable. I was getting to the end of my rope again because he was so tired but refused to sleep and was miserable ... but then Daddy, Marietta and Rebecca showed up and he perked right up and was more himself. We went for a walk together, played in the playroom and had lunch together. Lucky daddy, or should I say talented daddy ... after we left James fell asleep in a couple minutes without a fuss.

I did run into the doctor yesterday who told me James' x-ray looked "horrible" and so they will need to repeat it again in a few days. Clinically he's doing okay, so likely it was just a bronchial spasm that made it look so bad. We're hoping that's the reason and that all the drugs is the reason for the need for oxygen again and this will clear up in a few days ... please, we really want to go home.

The "Odd Child"

Our poor little boy had a very rough day today. Yesterday, on the other hand, was a nice day. With nothing that needed to be done, since we were just waiting for the GJ tube and a SAT monitor, we were able to take off for home at 10 a.m. ... only to be told we had to be back by 6-6:30 so they could put an IV in ... a nice welcome back. Usually we don't go back until 8:30-9pm ... but regardless we had a nice stay home.

Today we got to the day for the GJ tube. James had to be off feeds for 6 hours prior to the procedure and was scheduled for 1pm ... another long morning of distracting him from food. Since we do not want to feed him at night, this means he's gone quite a while without food ... IV fluids do not help an empty stomach. Twenty minutes before 1 they came in to give him his "sleepy" medicine. He would only be sedated (or mellowed) for the procedure to avoid a whole general anesthetic and another intubation (tube down throat for breathing). Only a couple minutes later they came back to say that they were behind schedule and he would not be going until 2pm. But ... he should get sleepy so it shouldn't be too bad and hopefully he'd go for a nap and stop asking for food!!! YA RIGHT!!

Fifteen minutes later he's wired and irritable, twenty minutes later her starts crying, and cries for the next twenty minutes. Meanwhile his SATs keep dropping and then he starts gagging and coughing. Finally he has to go on oxygen, which just upsets him more, but the gagging settles down. It seems to me that this medicine is not working, but nobody seems all that concerned, they just get an extra dose and we're on our way! They give him a shot of morphine to help with the sedation and finally at 2:20 we arrive in the room where he, of course, realizes something is up and becomes even more frantic. After waiting for the doctors approval they give another shot of "sleepy" medicine ... and another, and another ... plus an extra morphine. You would think after the second shot they would clue in that this is definitely not working for the poor child, who is not in the least bit settling down, and besides having slightly heavy eyelids is wide awake, fully alert and beyond irritable. But they push it to the limit of what they can give him ... and the poor child has to deal with the consequences afterwards.

It is finally decided that this is not working and he needs a general anesthetic afterall. A call to anesthesia says there is no one available at that time and so we much go back to our room and have it booked when there is someone available. I asked if this happens more often and the nurse told me that "the odd child" does not respond or it has the opposite effect. That's our James.

All James wanted to do is eat ... he is irritable and crying and we think food will at least settle him down. So we get him some food and he eats, then he's sort of dozing off in the chair but only closes his eyes for two seconds before he starts crying up a storm. Then he starts to vomit, and cry, and babble about anything and everything, and ask for this and that and everything when wanting nothing .... and did I mention cry, not just wimper, but a nice loud upset crying. The boy is totally confused and totally unhappy ... and totally driving his mom nuts as nothing helps. Although the medicine didn't mellow him to sleep, it did mellow his muscles/body as he couldn't stand well and seemed "relaxed" if held. Unfortunately his mother was not very bright and forgot this point and when she took the tray off his high chair and turned for two seconds: he fell out, hitting his head on the floor ... of course with witnesses ... and then shortly afterwards vomits again (this was not the first time) .... and so more torturing as they had to access him and then call the doctor and reassess, etc. It seems the fall did not do damage, the vomitting and irritability began before hand and he seemed fine when assessed.

After almost two hours of trying to figure out how to stop him from crying and trying to get him to sleep I discover that only thing that will quiet him is a ride in a stroller. And so begins a couple hours of walking around the hospital. He is tired, but does not sleep. The ride keeps him quiet, if I stop, he cries ... my arms are about to fall off from pushing a stroller and IV pole, nevermind I'm just tired and hungry. Thankfully I find a volunteer to relieve me for 1/2 hour while I eat and call my daughters ... and more thankfully when the volunteer comes back he actually asks to come out ... 15 minutes later he is sound asleep on my lap ... and like they predicted, once asleep he is asleep, his body is limp and he doesn't react is you readjust him ... he's definitely sleeping ... hopefully for the whole night because tomorow morning he has to be traumatized again as we are booked for 9am to have his GJ tube changed under general anesthetic this time. I sure hope his body can handle this ... once again he can't be feed and so we sent the nurse off to find out what to do with him as he vomits if I feed him and since I cannot feed him he will now be going over 24 hours without protein. When they restart his feeds tomorrow it will only be at 10ml/hour ... so it will be 48 hours before he's getting proper protein again.

But although he's sleeping I should not leave him long as his SATs were not doing the greatest and I hate for him to wake up in his current state and not have mommy there.

On a good note. They managed to find us a loaner monitor which they brought in today and we're trying it out to see how it works in comparison to the hospital monitor. We can use this until we get our own monitor ... which we're STILL waiting for quotes on ... but now that we have a loaner it's their problem if things go so slow. James continues to drop his SATs 2-4 times a night and repositioning him brings them back up, so we don't need oxygen, but we do need the monitor to know when to move him around. This loaner will be identical to the one they are looking for quotes on, so it will give us a chance to try it out ... not like we know what else is out there or what other options are available.

Another good note. I had an ultrasound and OB appointment today. All is going well with the pregnancy, which I continue to be sooo very thankful for. Baby is growing, fluid is good, placenta is doing good, blood pressure is still nice and low, sugars are doing good ... yes, there is much to be thankful for. James has not allowed me the opportunity to worry about the pregnancy and things continue to go well ... I just hope my lack of focus on baby will not make me miss any signs. I will admit these last weeks have been very hard on accepting the idea of having a baby in the house again ... I really can't imagine what we would have done if we had a little one ... but I know the Lord helps us through all trials, just as he has helped us to keep our sanity the past 3 1/2 weeks ... there is no point in worry about something that hasn't happened, we'll cross that bridge if and when we arrive at it.

One more note ... the Team Meeting is scheduled for next week Wednesday ... I will have to wait another week to hear what comes of it as that's when I have my next pediatrician appointmnet. Hopefully results are in on the special "stains"/tests they did by that time. A lot of items have already been dealt with, but it's still wise to get everyone together so that they're all working towards the same goals.

Sigh!

Yesterday James began to cough and have a clear runny nose. This was of no concern, as he did this last time after his bronchoscopy as well, so we are assuming it is from that. But unfortunately it seems to be having an effect on his SAT levels. At nap yesterday he only SATed 90-91% and after a short LOA yesterday afternoon he came back SATing the same. Overnight he hung in that area, continously dropping into the 80's. At one point they were going to put him back on oxygen, but I asked to just try respositioning a few more times ... we made it through the night, but his levels were not good first thing this morning. They managed to pick up after an hour or so of being awake and off we went to play. Unfortunately playing drained him. I was getting ready to pack up for a nice long LOA since most of our doctor stuff got finalized quickly this morning ... and thought I should do a quick SAT check before we left. He was SATing 88/89% ... so much for going home. James has come to expect these trips home as well, so it's difficult to explain to him that he needs to stay. Possibly if he picks up enough after his nap we might be able to leave, but probably not a good idea since we know activity is effecting him too much today ... sigh ... mom has come to expect those LOA's too!! :(

Things seem to move so slow around here. A few quotes came back on the monitors we are waiting for ... but there was quite a range in the prices, so the quotes have been sent to a Tech to review and let us know if the cheaper one is adviseable ... otherwise we're looking at quite a bit more then we originally thought they would be! :( If we ever get to the actual ordering part it still takes two weeks for the machine to come in ... and we can't leave hospital until James is SATing regularly in the 90's .... sigh ... I want to go home!! :(

We are scheduled for a GJ tube on Thursday at 1pm. Considering it took 3 days to get that appointment I'm sure hoping that this thing is going to stay in long as we can expect hospital stays whenever it comes out ... sigh ... :( Since it's rather iffy whether James would actually lay still for the procedure we now have to sedate him again .... just lightly this time but still another IV line into his poor scarred veins ... sigh ... poor guy!! :(

They have decided to put James on antibiotics for the bacterial infection in his lungs. This will be a three week long treatment ... sigh ... hope his bowels, diarhea and bum can handle that!! :(

So ... if everything went really well and suddenly we had really good consistent SATs the earliest we will get out of here is Friday .... sigh ... did I say I want to go home!! :(

But after all those sighs ... we're managing ... just tired of it all and really wish we could go back to "normal" again. And I used to complain about 2-3 appointments a week. But as I spend more time here I see more people who have spent longer periods here then we ... we each have our ways of coping. As James is so complicated it is hard for us to leave him when we need to be there ourselves to discuss each thing with the doctors ... when a parent is not there or on top of things it takes even longer yet as there is no proper guidance. For some parents they are fine to let the doctors and nurses make all the decision ... James is complicated and nobody knows him like we do ... so it is hard to leave him in the hands of those who do not understand how everything ties together. But he's a trooper ...and really doesn't mind the hospital ... each night he is quite eager to go in the van back to the hospital ... for this we are thankful!!

Three Weeks Later

Well it's been three weeks now, and we still don't know when we'll be coming home permanantly. We've been enjoying afternoon LOA's which help us to keep our sanity, even if it's just for a few hours each day.

James recuperated quickly from the bronchoscopy. We had thought that this would set him back, but by Saturday morning he was off oxygen again and back to what is now considered normal. As usual, over the weekend nothing gets done, so we didn't get any answers on what to do with regards to feeding James or where to go from here. We stuck with our decision not to feed him through the night, although Saturday night we did feed him until midnight. We seen that he deSATed before midnight as well as several time throughout the night. He just needed to be repositioned and then was okay again. Sunday night we stopped his feeds two hours before he went to sleep, as it is normally recommended that people with reflux do not eat 2-3 hours before sleeping. He SATed at 95% the entire night. We will be trying this again tonight to see if it's concidence. As well we are happy to see that repositioning him now works instead of having to put him back on oxygen. We have watched him to see if he can figure out on his own to roll over, but so far no luck in that area. This still makes us a bit leary about what to do at home as I can't always hear by his breathing that he's deSATing. We have advised that we would like to purchase a monitor but are still waiting for quotes ... nevermind getting the actual machine, which takes a couple weeks to come in.

Since James was doing well and nothing happens on the weekends he was able to escape earlier then normal for an LOA on Sunday and was home for lunch ... what a nice change!!

Today we tackled the Team on the feeding issues, but didn't really get the help we expected. The doctors basically said that we could look at trying to get all his feed into him during the 12 hours we have that he's awake during the day. She also stated that kids adjust, he would get used to be hooked up all day. Let's be realistic here ... James really functions like a typical two year old, he is not delayed, he is not tied down to other medical equipment ... when disconnected from his feeds nobody would know there is anything wrong with him. If there were no other options we would resign to this and work with it ... but other options are available.

We know our choices are to put him on domperidone or to go to a GJ tube. Domperidone is a medication that increases the movement of the digestive system, therefore making foods go through you system faster and keeping the contents in your stomach low so they can't come back up. This is used in combination with Losec which keeps the acid levels down in the stomach. James' system already works rather quickly, as we often see food go through him in a couple hours, so I'm not sure how much more effective this would be. As well ... you need to trial this for a few months, see how he handles it and how his lungs are doing to gage if it's working. So if it doesn't work we'll know because his lungs will fill up again. I'm not so sure this is the answer.

So that leaves us with the GJ Tube. This is similar to a G-tube. The G tube is placed in the stomach and the GJ Tube goes into the small bowel (jejunum). No further surgery is need to change to a GJ tube. They use the same hole as his current G-tube but change the tube so that it has an extension inside which they guide into place in Radiology ... as long as he cooperates, unsedated. This has to be done each and everytime a new tube is needed. GJ tubes tend to block up easier as they are smaller tubes so more care needs to be taken to flush the line and keep it clean. They also tend to come out a bit easier the G-tubes. Since you are feeding directly into the intestine instead of the stomach you can only use continous feeds as the intestines cannot handle a large volume at one time ... we would have to start over to see how fast he can handle these feeds and how many hours he can have tube free each day.

Since I didn't get a straight answer out of the Team and their response was somewhat unrealistic I waited to talk to our Dietician. We discussed our options and she felt that we had nothing to lose by trying the GJ tube ... if it doesn't work we just pull it out and put a G tube back in. At this time we know that James' nutrition levels came up to normal levels once we put him on his full daily formula requirement, so we cannot afford to lose hours and feed and need to get the maximum amount in him each day. This cannot be done in the hours he is awake, and she agreed that it was unrealist to expect James to stay hooked up all day. She consulted with the Team and they are in the process of arranging the GJ tube, hopefully soon.

The Team also advised us that intitial tests coming back from the bronchoscopy indicate that he has a bacterial infection again. They will wait until the Lung Specialist is in tomorrow to decide whether to treat with more antibiotics or to watch clinically and see how he handles it. The Team also advised us that the CT Scan shoes that James' lungs look worse now then they did at the end of April on his last CT Scan. It is hard to say if this is because he's been ill or because of his unlying lung issues. The CT Scan also suggests that James does have what looks like PAP (Pulmonary Alveolar Proteinosis) but they will not know until we get the special tests back from the bronchoscopy, which will likely take a couple weeks.

I also got a chance to view the Swallow Video Study today. We didn't really determine much from this test. We did not see anything go into his lungs (actually I couldn't really tell much of anything from the video/pictures, gotta have a good eye to read those). James' swallowing technique is not quite "normal" but not really considered "abnormal" either. For the time being we will have to thicken James' liquids to prevent him from coughing, gagging and dropping his SATs, as times goes we can make it less thick and see how he handles it. The question is, why did he suddenly start coughing and gagging when drinking? we never had the problem before. Just another James puzzle.

Bronchoscopy and CT Scan

After a long morning of distracting James from food, at 1pm yesterday they were ready and came to get him for his Bronchoscopy and CT Scan. It doesn't matter what room it's done in ... he knows the site of trouble. One look at the room, all the people, the bed, the equipment and he started crying "no hurt, no hurt". I sat with him on the bed for a while while they finished getting everything ready as he continued to say "no hurt, no ow". Thankfully I could tell him it wouldn't hurt (at least not that part) because he already had his IV in and all they had to do is insert the med into the line. I held him until he dropped off and then watch as they tried to stabalize his sedation. After a bit the Lung Specialist dragged me out to sign some papers saying I didn't need to watch the detail ... probably scared that they would have a panicky parent on their hands since it was taking a bit to get him stable.

The bronchoscopy was completed in the PICU unit (technically now called PCCU - Pediatric Critical Care Unit ... altho everyone still refers to it as PICU). The Lung Specialist came after to say that he found a lot of mucas in his lungs as well as a "milky" substance. He did admit in the many bronchoscopies he's done he's never seen such a substance. Milk usually has a fatty look, but James' formula is not normal, so it could still be formula. The Specialist still hold that we're likely looking at aspiration, which we know we are, but all the normal tests have been sent out, as well as the special tests. Some will come back next week, some will take a couple weeks.

From the PICU Unit (for those of you who know the hospital, what used to be Ward 3B is now PICU) James had to travel down to the Radiology to have his CT Scan. We had a bit of time to wait, so I got to sit with James for a bit while we wait. This time I got to have a look at what he looks like ventilated ...and boy am I thankful we avoided that in ICU as it was not nice to look at, and I only had to for a short period. I think what made it look the worst is that he was sleeping with his eyes more then half open. They were surpised at how often they had to give him more propofol (sedation drug) to keep him from moving around. At one point he started swinging around, kicking and squirming, but settled upon finding my hand.

We have not received any results from the CT Scan yet, but should hear on that today. It's pretty ironic that the last time we needed these tests done we couldn't get them done on the same day due to having to move him while sedated ... and they only had to move him a couple rooms down that time ... here they took him half way across the hospital and down one floor ... talk about politics!! Regardless they both got done at the same time this time and I was able to stay in long enough this time to actually get some pictures, but had to leave because I am pregnant. It's also amazing the difference on what they allow when an inpatient versus an outpatient, normally you get to see them drift off and are quickly asked to leave, this time they didn't care whether I stayed or not ... only the pregnancy thing got in the way.

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After the CT Scan they kept him sedated until they got him back up to PICU and then slowly let the sedation wear off ... once again I was allowed to sit with him, whereas when an outpatient they do not let you in until they've taken all the tubes out and he's fully awake (and usually screaming for mom). He arrived in his room shortly after 6:30pm, on 75ml of oxygen, just a small puff, but still needed to keep his SATs up. While gone we also lost our lovely private room with a bathroom ...and are now stuck in the smaller size semi's with no bathroom :( still in the step-down unit. I was surprised we hung onto our nice room so long so I shouldn't complain, but it really sucks downsizing, talk about absolutely no space in our new room ... never mind how many times I go to the bathroom in a day :) and my cot doesn't fit either :( oh, I want to go home!!

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The night went well, James stayed on oxygen and only dropped his SATs once, likely due to how he was sleeping as once he rolled over he was good again. We ran into a bit of a dilema that we should have asked about before the procedure as it was now evening and the weekend and no doctors around ... should we feed him at night?

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We had just cleaned out his lungs ... we believe he aspirates, especially at night. We now have clean lungs, it would not be wise to start refilling them by feeding him at night. On top of that he had not had his Losec (reflux) med that day due to sedation. But ... we could not withhold his feeds as he is not allowed to go without protein for more then 24 hours. The nurse called the resident who was of no help, saying that the bronchoscopy team said to carry on feeds as normal ... not like the had ever asked how we normally feed him. When the RT (Respitory Therapist) came around he agreed with us that it was not wise to feed him until we got things sort out ... but not being able to make that decision he did say that 12 hours of feed should not do much damage and then to make sure we get it sorted out in the morning ... as well, if his SATs dropped at night to stop the feeds. Considering he was on oxygen I doubted we'd see him deSATing all that much ... which we didn't.

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Today we are feeding him most of the day (short break at naptime) and then we will not feed him at night. This was just our decision, when I left at lunch time the Team had not come back to say what they thought would be best, but the Resident had been in earlier and agreed with our concern, saying the Team would review it. It looks like we'll be heading for a GJ tube, it's just a matter of how quickly this can be done. And then we'll have to see how he tolerates it.