From One Holiday to the Next

Well, it’s been six weeks. James first was admitted on Victoria Day … and he’s still admitted after Canada Day. Hard to believe. I’m still stuck in May, even though I know school is finished and the girls are off camping … it just doesn’t seem like July to me. But reality says I better catch up because babies coming whether I think it’s July or not!! :)

I did not think I’d be going home all that often, but then we realized it was a long weekend, and so I took the opportunity to go home a couple times over the weekend and get some relief from my “hotel” room and a chance to get a few things done at home. Previously the girls were home on the weekend, so besides doing the basics of laundry and organizing I did not have much time for anything else … it was nice to be able to tackle a few projects without any interruptions. This weekend the girls went off camping with Grandpa and Grandma, as well as some of the rest of my family. They were excited to find out there were more people they know camping there as well. I miss the girls, but I’m glad that they’ve had a chance to get out and have a nice time since it’s once again unlikely we’ll be having much of any sort of holidays this summer. The girls on the other hand are having so much fun gallivanting from one place to the next that they don’t seem all too bothered, especially Rebecca. Marietta has her ups and downs, she is more in tune with what is going on and more emotional. Rebecca on the other hand doesn’t seem to fizzled by what’s going on and just asks where she’s going next.

James has perked up back to his normal self … chipper and playing, along with an attitude developed from having one on one for so many weeks. He’s gotten used to the routine here and is more relaxed, sleeping better and cooperative with the nurses and doctors. He’s doing good on his treatment. He knows approximately when it’s treatment time and gets worked up the minute the nurse walks in the room at that time. He cries and is upset for the first minute or so of treatment and then settles down. We are happy that he is cooperative with the mouth piece. Treatment could also be done with a mask, but just the sight of a mask makes him go somewhat beserk. When using a mask they actually inhale only about 5% of the GM-CSF, this is because our nose has filters and actually filters out a lot of the material. When using a mouth piece he is getting closer to 20% of the treatment, so this is a much more effective way to treat him. The problem is that he is breathing with his nose while the mouth piece is in, therefore not getting the treatment as he should. The last couple times we have been plugging his nose while we do it … not exactly making it more pleasant, but he was more calm with the idea this morning. Now we just have to find a gentle way to plug his nose each time so we don’t have to hold it every time. We figure it’d be more effective to fight with him to accept a nose plug then fighting to accept a mask, which isn’t as effective. The doctors are very happy and very impressed that he is taking the mouthpiece.

Yesterday Rob stayed with James for the day. James had been on about 100-150ml of oxygen. After giving his morning treatment he forgot to turn the oxygen back on, but James levels stayed good and he didn’t notice until he went to take James for a bath. After attempting a bath James was playing on our bed and suddenly Rob realize his face and hands were turning purple and blue. A quick check showed his oxygen levels had dropped in the 80’s again and made us realize how much that little bit of oxygen helps him. We’ve never actually seen him turn colour before.

Today’s news is that they are working on discharge … yup we may just get home this week!! Tomorrow they will do an x-ray to see if any changes have occurred. They do not expect the GM-CSF to have actually done any changes yet, but wonder if he had a virus or was irritated from the procedure he had last week and whether that will change the look of his x-ray. At this point they figure they aren’t doing anything here that we can’t do at home. This means James will likely be on oxygen at home since he still requires it around the clock… a new thing for him to adjust to … and us.

They have set James oxygen limit at 92%. This means that he needs to be 92% or above and if he’s not he needs oxygen. At this point he is not able to hold that on his own, so they are working to get us a low flow oxygen meter as most home oxygen starts at 500ml and he doesn’t need this much. Hopefully it’s just a matter of a few more days and he’ll be able to tolerate being off oxygen …although so far he hasn’t indicated that this is the case. Again today I took him off for a bit to have a bath, but by time bath was over he was in the 80’s again … all he’s getting is a whiff of oxygen thru the nose prongs, but he needs it.

The initial plan is that we come back in the next 2-4 weeks (whenever they can get us an appointment) and have another x-ray done. At that appointment they will make further plans. Since this is all trial and experimental this means that they can’t really speculate on what we will be doing in the future … we tackle each bump as it comes. Today’s speculation is that after that they’ll do another CT Scan to get a clear picture and then review whether a Whole Lung Lavage (WLL) will still be necessary. We cross that bridge when we get there.

How long it will take for discharge will depend on how quickly they can get everything organized … one thing is for sure … they can do it quicker then Mac. Tonight he has to be retested for oxygen coverage since we’re in a new hospital and also the last one was already two weeks ago, which means many things could have (and have) changed since then … they need something closer to discharge date. If we have trouble getting the full strip she did not seem concerned and said they would just write a letter and get the coverage that way. He will also be getting nebulizer equipment (a machine that turns liquid medicine into fine droplets that are inhaled through a mask/mouthpiece) to run his treatments … this is all government funded through the same programs we are already registered with for James’ tube feeding equipment. With regards to SATs monitors … they are advising us to try our best to hang onto the loaner one we currently have …unfortunately we probably won’t be able to hang onto it long enough as we were only suppose to get it for two weeks, and two weeks is already up. They do not have one available for loan, but they are hoping that by the end of this summer a government program will be in place which will fund these as well. If we run into trouble we’ll still have to purchase, but it seems they have better vendor connections and can get the machine at the same “lower price” we were originally quoted but are having so much trouble actually getting at Mac due to sales person changes. The other thing to be organized is to actually have enough medicine for us to go home on before our full supply comes in. So we’ll see how long it takes to get everything together.

And that brings us to the end of another long update.

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.

.

.

.

.

.

.

Thursday at his appointment. Althought feeling awful after his nap he so badly wanted to play with the toys ... such a trooper!!
.

.

.

.

.

.

.

.

.

.

.

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.

.

.

.

.

.

.

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??"

.

.

.

.

.

.

.

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.