Mar 28, 2015

{cincinnati & a change of plans}

one of the things i wanted to do more of this year was updating my blog again. its been on my list of things to sit down and do. more little, fun updates. well this one isn't that. daniel and i actually tag teamed writing this one, so if there are bits that don't sound quite like me - its probably him! he did most of the actual medical update pieces. he's better at simplifying those things. 

this past week we drove up to cincinnati - they have a really incredible childrens hospital and we've started transferring some of isaacs care there this past december. the original plan was to knock out a bunch of annual testing at once to cut down on some of the frequent specialist follow-ups we have at home. so while that did happen, we also just about doubled our expected time in the hospital as we kept finding out things that were unexpected. and just as a disclaimer, each of the doctors we met with were absolutely fantastic. they took their time with us and were kind and concerned and they all worked together to help us decide what steps we need to take for isaac, which meant the world to us. so all that said. all of the x-rays, mri's, blood draws and talks we had this week led us to some of the following, sort of in order of when we learned them:

  • Isaac's pituitary gland is irregular. This was no surprise for us, as this gland is responsible for growth and Isaac is off-the-charts small for his age (even for FA). But an MRI showed amorphous tissue formations where the gland should be. Testing is underway to see just how dysfunctional it is so we'll know how much to counteract it with medicine/treatment.
  • Isaac needs to get more calories in him. Even though his small size may be largely due to growth hormone deficiency, he still needs more nutrition for sustained growth. And with all of the eating issues inherent to FA, it'd be almost impossible to get him to eat enough orally. Sooooo, we're going down the path of a feeding tube. He's got a temporary NG-tube going into his nose now, which will be testing his body's absorption of supplemental feeding over the next few weeks. Once that's proven, he'll likely have a G-tube surgically installed in his tummy. As much as we'd hoped to NOT have to go this route, I think it's going to relieve pressure on Isaac's eating and make his nutrition more consistent and trackable.  
  • Isaac's ability to empty his bladder isn't great. Instead of pushing urine OUT of his body, some urine is taking the path of least resistance & going back UP to the kidney (called reflux). May not sound like a huge deal, but...
  • Isaac's kidneys are in worse shape than we thought. He was born with 2 fused kidneys on the left side & they're only functioning at about 40% right now between both of them. The reflux that's occurring is dangerous because it can bring bacteria up to the kidneys and damage them with infection. Kidney preservation is key to a successful bone marrow transplant, so we're considering surgery options that can help reduce the reflux and protect the kidneys. But, surgery would be risky right now because...
  • Isaac's blood counts are not looking good. His platelets have dropped significantly in just the last couple months, and his other blood counts are trending downward as well. Our FA team is very concerned and want to monitor him closely over the next couple months to determine if a bone marrow transplant will be necessary sooner than later. We can never exactly predict timing with this, but the time frame we are bracing for at this point is any time in the next month to a year. Because of those concerns...
  • We conducted HLA-typing tests & are currently searching the registry for a genetic match for Isaac. Bone marrow transplants have a much higher chance of success when the donor has very similar genetics to the recipient. We did a preliminary search in Minnesota in 2013 that didn't show any GREAT matches, so we're hoping for some better options this time. We should know more in the next day or two!
so thats some of the nitty gritty. i don't know that we've ever talked on here - for real, about what a bone marrow transplant means for our family. and its all still so fresh, i'm not really feeling like i can give a statement just yet on how we're handling things or what the next few months are going to look like, because it changes all the time. the night we got his results from his blood test we cried ourselves to sleep and the next day it was back to business mode, and trying to keep isaac from being too overwhelmed by all of the tests we had to do. so more on that later. the last year and a half though, have been incredibly peaceful, so i'm grateful we had so much time without having to think about a lot of this. and in the midst of all of the crazy - we still managed to make the time outside of the hospital fun for isaac. we watched movies and saw the aquarium and the zoo and visited cousins. so with that said...more to come soon.