24 Apr Finally what you’ve been waiting for…
Have I put off a substantive update about Keegan long enough yet? Yes? Ok. I apologize for the delay, but it is for the most part a no-news-is-at-least-no-bad-news kinda thing.
To start off with, Keegan’s heart is A-Ok, as usual. We had a quarterly transplant clinic appointment last week with echo, labs, and doctor visit. It’s always good to hear that his heart function is great and stable. No concerns at all there. It’s the rest of his little body that always throws us for a loop.
We have successfully weaned down another milligram of steroids. Keegan will only have about four more months of them if he holds on this pattern without incident. Starting with the next wean, we will likely increase his anakinra to give him extra coverage from a flare with the reduced amount of steroids. That will be the equivalent of two full adult doses per day. Luckily, there are few side effects of anakinra. However, it locks us into using a central IV for the foreseeable future.
We met with the immunologist last month. She gave him some vaccination boosters to try to increase his protection from certain strains that he didn’t respond to initially, and we also gave him a dose of a special pneumonia vaccine that children don’t usually get. This was two-fold. This vaccine is for a saccharide-based bacteria. By giving him the vaccine and then testing his response a month later, we were able to (1) protect him from this bad strain of pneumonia and (2) see if his immune system can respond to especially dangerous saccharide-based bacterias. Thankfully, we got good news on both fronts! He had a good response to the vaccine, which gives us such peace of mind.
We have a follow-up appointment with the neurologist in two weeks. We’re anticipating that will be another good report with stable news. His teacher recently did some informal standardized testing in order to prepare for a meeting with the school district to plan for kindergarten (yikes!). Keegan placed in the 32-40 month age range in social/emotional and communication, and on the low side of average for his real age range in cognitive skills! That means that while he has difficulty expressing himself and engaging with others or attending to self-help needs, he understands things on a level consistent with other five year-olds. This is a full year better than where he placed in January of 2012. He will have a formal evaluation by the neuropsychologist again in July to get more solid numbers, but we couldn’t be more thrilled. Keegan has worked hard for that progress, and it shows. As for kindergarten, the medical team, the school, and Gray and I all agree that he needs to be home-bound for at least one more year due to his immune suppression. He would have been in a resource room alone for all core subjects anyway, so keeping him home makes even more sense. Next year, he will continue with his current special education teacher (yay!), but he will have 4 hours of in-home teaching, instead of just 2 hours. Hopefully, we will learn more about his genetic immune deficiency and get him off steroids during the coming year, which will help him feel better, learn better, and have a better chance of leaving home for school by first grade.
We have a follow-up appointment with the neurologist in two weeks. We’re anticipating that will be another good report with stable news. His teacher recently did some informal standardized testing in order to prepare for a meeting with the school district to plan for kindergarten (yikes!). Keegan placed in the 32-40 month age range in social/emotional and communication, and on the low side of average for his real age range in cognitive skills! That means that while he has difficulty expressing himself and engaging with others or attending to self-help needs, he understands things on a level consistent with other five year-olds. This is a full year better than where he placed in January of 2012. He will have a formal evaluation by the neuropsychologist again in July to get more solid numbers, but we couldn’t be more thrilled. Keegan has worked hard for that progress, and it shows. As for kindergarten, the medical team, the school, and Gray and I all agree that he needs to be home-bound for at least one more year due to his immune suppression. He would have been in a resource room alone for all core subjects anyway, so keeping him home makes even more sense. Next year, he will continue with his current special education teacher (yay!), but he will have 4 hours of in-home teaching, instead of just 2 hours. Hopefully, we will learn more about his genetic immune deficiency and get him off steroids during the coming year, which will help him feel better, learn better, and have a better chance of leaving home for school by first grade.
Our biggest problem lately has been getting his electrolytes stable, mainly his magnesium levels. His potassium and sodium have been ok as of late, but his mag and phosphorous are chronically low. The mag is the one of most concern, as low magnesium can cause heart arrhythmias. In a denervated heart (the electrical pathways of the heart are cut during transplantation), arrhythmias can be dangerous and difficult to correct. Unfortunately, the main anti-rejection medication, tacrolimus, inhibits the absorption of magnesium in almost all patients. Keegan has always had low levels, and for the most part, we’ve been ok with it, as he didn’t have too many ill side effects of the condition. At least, so we thought. The few days that we were able to get his mag levels even a touch higher than his normal levels (nowhere near normal), his teacher and therapists all consistently noted that he behaved better, was more cooperative, and concentrated better in sessions. This prompted us to believe that even though he seemed “ok” at these chronically low levels, he would be better with higher ones.
However, lately his levels have routinely dipped to “critical” levels. The doctors first increased his TPN to three nights per week. Then, the TPN was stretched from 10 hours to 12 hours. We completely maxed out his oral dose of supplemental magnesium. Finally, we gave him a few doses of IV magnesium here and there. We checked his kidney function, but it didn’t seem he was wasting an excess amount via his urine. When nothing else worked, the team decided to bring him into the hospital for IV mag every Tuesday and Thursday. After the first week, his levels were still low, so know we’re increasing his dose…again. Our goal is to get him at least close to normal. So, it looks like this will be our new routine for a few months. If it works long term and he tolerates the infusions well, we will eventually be allowed to administer them at home.
Of course all this fluid has him a little on the puffy side, but that’s a small price to pay for now. Regular labs will be drawn on Friday, so we’ll see if all the work is even helping him at all. He has been sick with a cold that his sister brought home. It’s been a little hard to tell how he’s feeling other than that, but the snot seems to be subsiding now. We are hopeful for a break for our little buddy soon.
Of course all this fluid has him a little on the puffy side, but that’s a small price to pay for now. Regular labs will be drawn on Friday, so we’ll see if all the work is even helping him at all. He has been sick with a cold that his sister brought home. It’s been a little hard to tell how he’s feeling other than that, but the snot seems to be subsiding now. We are hopeful for a break for our little buddy soon.
I again apologize for the delay in posts. Sometimes things aren’t really black-and-white, and I’m not sure what to update about, if anything. Sometimes I don’t want to jinx a good stretch. More often than not, I’m exhausted by the time I sit down, and I really just don’t want to dwell on it all. I’ve said before that it seems hardest to grapple with everything when things are stable, when you have time to actually think and react. I have a million excuses to throw out. At any rate, thank you for sticking with us through it all, thick and thin, stable and not. It means the world to us.
And to show our appreciation, a little entertainment for you:
