CT Results

Last Thursday James and I went off to see the Lung Specialist once again, this time for the results of his CT Scan.

Although we were not suprised to hear the results, we were still disappointed that it did actually come back as we expected. The CT Scan showed that James still has a lot of congestion involving the bottom part of his left and right lung. The radiology report states that it is worse then the CT that was done last year. (At that time he had a more general CT because we did not know what was wrong with him, they also thought he had some sort of pneumonia or croup and so did not pay closer attention to this problem.) The Specialist stated that if this is the first time they were seeing this they would think that James had a bad case of pneumonia, but we have now been seeing this for a year and it is not clearing up on it's own. We can only wonder if the recent antibiotic treatment was of any help as we do not have a directly before picture to compare with ... all we know is that it's been getting worse over the past year and all the more "mild" treatment options have been used.

At this time he has simply classified James as having chronic congestion. Where has this come from? It is believed that the majority of it stems from the chronic aspiration, which we now seem (key word) to have under control with the Losec medication that he is taking. As well the congestion comes from previous and/or current infections. These infections likely would have come from the aspiration/food particles left in his lungs. We are not sure if the antibiotics cured the infection, but he figures it is now more likely he has chronic congestion then an ongoing infection as clinically he appears to be doing better ... unfortunately radiologically he does not. We also do not know how much inflammation versus scarring there is in James' lungs. Once you have scarring there is no recourse and the lung function has been reduced forever.

What is the next step? James has been put on a new drug called Prednisolone which is a form of Prednisone. Many of you have probably heard of this drug - it is commonly used for asthma. Used short-term this drug can have side effects, but long-term it can REALLY have side effects. Sometime we wonder if it's better not to know too much and to be left in the dark ... but yet we realize it's wise to be informed. What is considered short term? that depends on who you ask I guess ... according to most internet sites it's around 7 days of use, according to our doctor the long term effects come after 2-3 weeks of use ... either way it doesn't take long to reach the long-term usage point and James has been prescribe to start with one months usage, so either way he falls into long-term use.

Our doctor advised that most tolerate this drug okay for short term use, and so far James is tolerating it okay. The most common short term effects are upset stomach, increased appetite/weight gain, insomnia, mood swings/depression, a round/swollen face and excess sweating. Reading up on it I found many people complained about these symptoms, that they simply felt awful on the drug. Not very comforting, so we will continue to watch James carefully.

Prednisone is a steroid ... it is a corticosteroid or cortisonelike medication. Our body naturally produces corticosteroid. These chemicals are involved in various regulatory processes in the body (such as those involving fluid balance, temperature, and reaction to inflammation). Long term use of this drug depresses the ability of your body to produce corticosteroid. A drug like Prednisone suppresses inflammation, but this means that is also impairs the body's natural immune response to infection and makes you more susceptible to infeciton. As well it impairs calcium absportion and new bone formation which means that is causes or increases osteoporisis risks. It also causes thinning of the skin which in turn makes for easy bruising. This on top of all the short-term effects which often don't disappear but only lessen with time. Prednisone is also not something that you stop whenever you feel like. Your body stops producing on it's own and so it needs to be weaned off the medication or you could find yourself in even more trouble

So why put him on such a drug? How true are all these symtpoms? Are the benefits worth the risk? What are our other options? Of course we always hear more about the bad side of things then the good, so we like to think that these side effects are not as real as the information provided, but yet it seems many webpages with personal testimony testify that the symptoms do indeed happen. We are sort of feeling that at this point we have no other options but to try this drug. Our Specialist has stated this as well ... he said he treated James in a very aggressive manor with regards to the antibiotics and did not see a positive result. Our only other options are to wait and see if it clears up on it's own, which is highly unlikely given the way it has gone so far or try long-term antibiotics, which have not seemed to help and can do more damage to James' colitis. Prednisone is the last attempt before having to do an Open Lung Biopsy and some brief reading on this type of biopsy tells us we should give the Prednisone a chance before subjecting him to such a test.

If we are able to clear up the congestion will it be permanantly gone? This question is impossible to answer as we are not 100% sure of the cause but are assuming it is from aspiration and infection. If we can clear the congestion, treat the reflux and aggressively treat any future infection we can only hope that the congestion will not come back. But for the time being we have to first get rid of the congestion.

So at this point we have given it a go with the Prednisolone for one month, he will have a repeat x-ray at that time to see if we have made any progress. James has been given anti-imflammatory dosage versus an immune suppressant dosage, but yet the doctor did state that this dosage would also calm the immune system and make him more suseptible to infection once used over a period of time. The question is a matter of when to become worried about such side effects and so we felt it is just easier to try and "isolate" James (if such a thing is possible when you have a sibling in school and spend lots of time at the hospital) sooner then later. We will reevaluate this at the end of the month also, when we know if he'll have to stay on the drug and also by that time the weather will be nicer and hopefully less bugs floating around. So ... don't be surprised if you hear me ask "does anyone have any sort of cold or illness in your house?"

On the bright side ... prednisone will be helpful for James' colitis as it works again all inflammation in the body ... and ... an increased appetite and weight gain works in James' favour. The Lung Specialist also stated again that he is amazed at how James' lungs always sound clear when he listens to them.

Congrats to my Dear Hubby!!

Well congratulations are in order ... Rob wrote his last exam and can now say he made it through Year #2. I know the marks aren't in yet, but I'm confident he did fine. It wasn't always the easiest, and he was ready to give up at one point ... but his wife helped him pull through ... even if he didn't like her non-sympathetic ways at the time. If I had been sympathetic he would have dropped some courses, so I had to be tough :) Two years down, five more to go ... argh ... but we're looking at the positive side today. Can't wait until next year when he can say he has completed University and gotten his B.A. .... but that's a whole year away and who knows what will happen between now and then. So for today we're celebrating Year #2 being completed.


"Celebration" was very mild, but the kids thought it was great. We had candlelight supper which they helped set up. James especially loved the candles and spent half the supper trying to blow out the one nearest to him. After supper came a game of "hide the presents". The girls had "wrapped" and hid the presents ... which simply consisted of a large supply of Rob's much loved droopies and other favourite dutch candies (that will probably disappear mighty quickly) ... and Rob had to find them. Of course they made sure to put each variety in a seperate gift bag so they could prolong the hide and seek game.

And now we can kiss Rob good-bye as he eagerly goes off to work tomorrow ... we look forward to September when we will have a hubby and daddy again. Everyone knows how much I dislike his job, and everyone knows how much Rob loves his job (and for those of you who didn't know ... you do now :) ... but whatever the case it brings in the money and it's only for four months ... and really, it won't be so bad this year ... I hope :) Rob has decided to work only 4 days a week so that he will actually have time to open his books and do some studying and reading over the summer. The long hours previously never allowed him to do much more then work and sleep, especially when last year involved countless hospital visits at the end of a long day (we pray this summer will not be the same). So this year shouldn't be so bad ... I just have to adjust again to not having him around and not being able to count on when he'll be home. That adjustment will start first thing tomorrow morning when I no longer have the luxury of a hubby who brings Marietta off to school and who often picked her up too. But that's okay, most others don't have that luxury either .. and the weathers not so bad anymore :)

So congratulation dear hubby ... I'm glad you stuck it out and made it through your second year ... enjoy those days in the truck because September will be here before you know it!!

Old versus New

For those of you who are curious as to the difference between James old and new tube ...

this pictures is from October 2006, a couple months after his first tube was put in.

I am not sure as to the technical name of this tube. It has been referred to as a PEG tube (percutaneous endoscopic gastrostomy) but most commonly just called a G-tube (gastrostomy) - which is a broad term for any tube placed into the stomach (including his new one). This tube was pretty permanant ... in that it won't just fall out and could last years - or so they claimed. It is recommended to have it changed after 6 months because you cannot just take the tube off and soak it to clean it out properly ... slowly things begin to build up in it ... if you look closely at the picture you will see a cross-bar by his stomach, it is more of a clear colour, then look at the tube itself, it is yellowish ... but it was at one time the same colour as the cross bar. This tube was held in by a small disk behind the abdomen wall, which they had to literally yank out. Considering how James has yanked on this and caused some good bleeding, but yet it didn't come out, I can only imagine the kind of yank they had to do to get it out ... hence the reason he need to be sedated. So it is suprising the the site is not damaged after the change, but besides some slight pussiness it looks pretty good.

And here we have James today ... sporting his new cadillac MIC-KEY Low-Profile Gastrostomy Feeding Tube ... simply referred to as a Mickey or Mickey Button, and still can also be referred to as a G-tube. Unlike his other tube this one is not so permanant and can fall out on us, either by a really good tug or because the balloon has worn down, gotten a hole or deflated. This tube is held in by a balloon behind the abdomen wall, which is inflated with water. Once a week we have to check to make sure there is still the right amount of water in the balloon. On the right you see a picture of what the Mickey looks like when not inserted. The balloon is blown up with air on the picture, but air would not hold once inside the stomach so water is used. The Mickey has two wonderful features ... first, the obvious, it doesn't have the extension of tube hanging off the belly all the time (which we always had to tape to his stomach); second, it has a check valve that prevents backflow ... so if the cap accidently opens (and yes that has happened) we don't have James stomach contents all over the place. In order to feed James we have to hook up a special extension tube to the Mickey button, which sort of functions like a key to open up the valve.

That being said, so far James isn't all to impressed with his fancy new tube. He cries when we try to feed him and says "no, no". He has even pushed my hand away in the middle of his sleep when I tried to hook him up for his night feed. We do not see any signs to be concerned about and are assuming that he is experiencing some discomfort and pain with the healing from the change over and the adjustment of how the new tube hangs and puts pressure on his stomach when being fed. He is completely happy when he is not hooked up and plays as if nothing is wrong, so it seems his stomach just has to toughen up and adjust to this change. Our little trooper keeps trooping!! :)

CT Scan and Mic-Key

We had a good day today and all went well with James' sedation, CT Scan and G-tube change. We are happy to say that James is now sporting the "cadillac of g-tubes" (or so we were told) called a Mic-Key. The tube is actually not as flat as we had thought, but regardless it doesn't have the extension on ... no more taping stuff to his tummy.

I must say things went really smoothly today and didn't take a whole lot longer then a regular Mac appointment takes. We arrived at the hospital shortly before 10:30 and left around 1:30, I had expected to stay much longer. These procedures get harder and harder each time as James gets a bit older and wiser. When we arrived we didn't even get to sit in the waiting room and they called us in, all happy that we were early because they were ahead of schedule. We had to go to the same room for set-up as the last time and James stopped dead in his tracks before we even entered the room and there he stood crying and calling for daddy, and saying over and over "all done, all done". So I had to go through all the questions etc with a rather upset, crying little guy who knew full well what was ahead. And when I finally get him settle down a bit they realized that they had their scheduling a bit out of order and say we could go back out to the waiting room ... lovely, now I would have to go through this all again. James quickly relaxed, probably thinking he had escaped the torture session.

When they called us back in they brought us directly to the CT Scan room, but James is no dummy, one look at the bed and all the equipment and he knew there was trouble ahead ... there starts the crying and pointing at the door, calling for daddy and saying "all done, all done" again. We have been told that James will always need to be sedated by IV as he has been diagnosed with reflux and it is safer to do it this way. Whenever sedated he will also be intubated (insertion of a tube into his airway) to keep his airway clear since he may aspirate once his muscles relax. They decided to give him some gas "just to relax him" so they could put the IV in ... or so they said. Well I'm not sure what the difference between relax and sleep is because he looked pretty dead to the world to me by time they took the mask off. I prefer the gas as it is so much faster and I don't have to look into those pitiful eyes that are yelling help for more then half a minutes.

Everything went quickly and smoothly and James woke up for the anesthetic nicely this time ... none of this screaming and crying and crawling out of my arms for a couple hours. He woke up like he had been woken from a nap - grumpy, but responsive, and very shortly after they said we could go. He was miserable and irritable, crying and getting upset at the funniest things, but he was also hungry and asking for food. By 5pm he had passed the "test" of his stomach and we allowed him to eat what he wanted, which happened to be Pringles chips.

Considering the lack of food, the lack of a true nap, and the effects of an anesthetic you would never have guessed anything by the way James was playing after supper time. We seen him rolling around, horseplaying and everything as if nothing had happened today. So it would seem that the yanking out of the old g-tube did not hurt him all too much. He isn't all too pleased the this new object in his stomach, getting upset whenever we look at it or try to feed him and pointing at it like he wants it out ... but he'll adjust.

Hopefully this will be the last of the procedures and tests for a bit. The Mickey g-tube is something that we can change ourselves at home, so James will not need to be sedated again when a new one is needed. They informed us that if this tube falls out we need to get a replacement into him within the hour as within 1-2 hours the hole begins to get smaller and by 24-48 hours it's gone. This means we have to carry a extra Mickey with us at ALL times. So I made sure I got my prescription filled this afternoon (each Mickey is specially measured to fit, so a prescription is needed) so that we are all set.

Just Stuff

Wow, two whole weeks with no James appointments ... I think it's a record!! We've been enjoying a relatively quiet time. Last week I was able to actually enjoy several days in a row without having to be gone half the time for appointments ... what a nice change ... I could get used to this :)

Rob is in the middle of exams right now. He's itching to get back into the truck and out on the road, but still has two more exams to write next week. In the meantime he'll have to settle for a one day of trucking, which he's planning to do tomorrow. Hopefully this will calm the itching a bit, instead of making it worse since he finds it hard enough to concentrate on studying as it is. At least the cool weather has kept him from getting sidetracked outside, and suprisingly the honey-do list is actually quite short right now.

This past weekend we enjoyed a lovely trip back to Chatham to catch up with friends and family in the area. The kids had a great time and James did really well sleeping away from home (something we usually have a struggle with). The girls, especially Marietta, struggled with very bad chest colds, mostly at nights, but were still able to enjoy themselves considering the lack of sleep from coughing and gagging.

As for James' health, he has been doing relatively well but unfortunately their are signs of trouble ahead. He is happy, content and energetic, but runs around with a constant clear runny nose again. Two days after we took James off the antibiotics that he had the allergic reaction to we seen his runny nose come back. The second attempt at antibitotics they put him on two antibiotics at the same time, one for 7 days, one for 14 days. Once back on antibiotics the runny nose disappeared, but two days after he completed the 7 day antibiotics the runny nose was back. We were afraid of what would happen once he came off the 14 day antibiotic, but we didn't see any immediate change until last night (6 days off antibiotics), where we seen the first fever again. As well, over the weekend we seen the ear tugging thing happening again.

It all seems so clear and obvious to us ... the problem is not cured, once again the antibiotics hid the symptoms for a period and now they're back. But of course, we have to prove to the doctor. First we're told it's likely just a cold .... I think we've done this enough to know the difference, and since we know that they will tell us that we waited and watched the symptoms long enough to be confident it's not a cold. Of course, our fear is that in the meantime he will pick up a cold since the girls are roughing their way through one tough chest cold.

Today I called back again to say the symptoms are still the same, no sign of a cold, and first fever last night. The lung specialist called me back and said we can be looking at two things, infection or irritation. Infection can be viral (meaning cold) or another cause (bacteria). Irritation would mean that their is an inflammation as opposed to an infection. What he is referring to is called rhinitis (more indepth explanation here) which is an inflammation of the nose causing excess mucus production. (A form of rhinitis, called allergic rhinitis is commonly known as hay fever - so fancy word for a common problem. In James case it would likely be vasomotor rhinitis which means it's has non-allergic cause or unknown cause) If we're looking at irritation then we shouldn't see fevers, so we'll be watching the fever activity for the next bit. Irritation can be treated by a steroid nasal spray, similar to those used for people with hayfever. Unfortunately if we're looking at infection, which is seems we are due to the fevers (which he had again today) then another round of antibiotics is needed. He has mentioned that he's already been more aggressive then normal with James and would rather not do this ... we really do not want to do either as we do not feel it's helping, but what other options do we have?? We are to watch his symptoms for the next week, until we have an appointment with the specialist again and then we'll have to review our options. In the meantime we're praying he won't pick up the girls cold as we're afraid he won't be able to handle this one. But he amazes us so often and has an excellent immune system, so hopefully he can get past this one as well.

On Friday James will be sedated to have his High Resolution CT Scan done on his lungs. We're also hoping to have his current g-tube changed that day, but we're not holding our breath on that one. Currently his tube has a permanant extension which we have to tape to his stomach to keep out of the way. We hope to have him changed over to a Mickey button tube, which will basically look like a botton in his stomach.

Tomorrow James is going to be reassessed by the Speech Pathologist, which means I should get to completing his word list. James chatters up a storm now and we see no problem with his speech. We know they will still not give him good "marks" as he still has not reach the 200 word mark, at least not in words that I can remember, although everyday it seems he adds more words to his vocabulary. He continues to learn like a typical child does he's just a few months behind. His understanding is excellent and well on-track. The past weeks/month we have really seen our boy start to grow up in his actions and play. Rebecca and James spend a lot of time playing together now and seem to have a special bond, it is so neat to watch them.

Some time ago I mentioned Marietta's finger and how she had some bumps on it which they thought might be ganglion cysts. The bumps suddenly disappeared after a month or so, but her finger remained swollen so I still took her for the x-rays and an ultrasound during the March Break. The results came back that everything was fine and to keep an eye on it. Which we did and one of the bumps suddenly reappeared a couple weeks ago. So after waiting almost a week to make sure it wasn't going to play the disappearing act again I called and asked for another ultrasound, which they booked for today ... but alas the bump is scared of having an ultrasound and disappeared on us again. This time the swelling is gone as well ... so hopefully we've seen the last of it.

Meeting of the Minds

This week has been pretty quiet medical wise - always a welcome change. James had one appointment this week. This appointment was with the pediatrician and wasn't really necessary for any new medical reasons, the only reason we went was to see how the Meeting of the Minds went and sort of tie all information together.

Our pediatrician said that each doctor had their chance to go over their treatment of James and what they forsee as future treatment. It is ironic to note that the Genetic Doctor had the least to say in the situation - James' LPI has remained under control, his ammonia levels have always held out well under each circumstance ... LPI wise he's doing good ... it's all the other factors that have kepts us so confused and busy. I didn't reap any new information from the visit ... as often happens when our pediatrician tells us some of the "behind the scenes" thoughts or comments.

So it stands to say that in addition to LPI James has colitis, esphogitis, reflux/aspiration and chronic bronchitis. The esphogitis is reflux related, which they are controlling by Losec (acid reducing medication). James' biggest challenge right now is his interstitial lung disease, which has been narrowed down and specified as being chronic bronchitis primarily due to aspiration (or reflux). James is struggling with his stools and yeast bum-rash right now due to the antibiotics he's been on for the last weeks and it will continue to be the challenge of weighing the benefits of one treatment against the effects it does on other parts of his body ... namely at this time, irritating his colitis. We need to question if this continual irritation will cause more colitis damage, and the only way to answer that is another colonoscopy, which we do not want to do. We pray that this round of antibiotics will indeed do the trick.

The only advantage of this round of antibiotics is that we now know he has reflux and have him on Losec. Previous antibiotic treatments were considered ineffective because he continued to aspirate into his lungs, which means food continued to land in the lungs and cause infection. So if the Losec is indeed working it should be holding the food out of his lungs this time and allowing the antibiotics to truly do their job of cleaning up the bacteria in the lungs. By the end of next week we should begin to get our answer.

A referal has been made to the Ears Nose Throat (ENT) Specialist to get the ball rolling on his continual fluid behind the ears. We're hoping that the fluid will clear up before any treatment is necessary, but at least for the time being the ball is rolling.

Other then that, if the lung treatment goes well we hope to be able to start spacing out our doctors visits a bit more ... anything is better then 2-3 appointments a week :)

We focus much effort on minimizing pain and suffering in life, but Easter always brings us to the reality that nothing compares to what our Lord and Saviour did for us. He willfully suffered unimaginable pain and anguish, not only by the scourings and ridicule by men, but especially by being forsaken by God. He endured God's wrath against sin so that we would not have to; indeed, we could not. Therefore we have confidence that whatever pain we may endure now will never separate us from His love. To Him be all glory, laud, and honour, for death could not contain Him, but He has risen, and has been seated at God' right hand.