I think I've swallowed so many tears that there is an ocean in my stomach.
I started this blog in large part to reach out to other parents of sons with ALD. It's so hard to find information out there. Old hypothesises conflict with fresh studies and it's hard to make head or tails out the information. For instance: diet. To follow a special ALD diet or not? I read a 15+ page report that leaned toward following a low very-long-chain-fatty-acid diet, then other pages that said such a diet is basically useless. I took it to the experts and emailed Dr. Tim. He says that there's no benefit to following any special diet. It's things like this that make me want to write this blog. That and the fact that so much of what I read on-line about ALD is starkly depressing and bleak. I want to give other parents hope.... That's my mission, but it's also my challenge. Because some days, it feels like the weight of the world is bruising the butterfly's wings.
We took Gabe to Universal Studios last week and he had a blast. He got to ride some big rollercoasters and we all got to forget for a little while about the real world. We rode the Monster Bus Eco-tour at Showcase of Citrus- one of Gabe and his dad's favorite things to do in Florida. Not only does he like the giant bus, but get this: he has a huge thing for Florida Natural Orange Juice after learning something about how orange juice is made in school. It turns out that Showcase of Citrus is one of the major providers of oranges for Florida Natural and Gabe got to pick his own oranges in their grove. Best of all, he got to UP in the air in a helicopter! I'm so glad that he got the chance to fly! He didn't want me mentioning "his disease" while we were on vacation and the few times that I tried to bring it up, sensing a teaching moment or opportunity to talk, he got irritated and asked me to drop it. He hasn't wanted to talk about it almost at all lately. I asked him why today and it broke my heart when he said "because I'll say it'll be alright, but what if it isn't?" I struggled to answer that. I said something about bravery and faith and him being in the best hands and remember when daddy had cancer and we were afraid and look how great he's doing now.... Last night he walked up to me in the kitchen and gave me a hug. Then he just started to cry.
I'm afraid of the transplant. Admittedly, I don't know very much about bone marrow transplants - I've never had much reason to. I tried to become a donor once, but because I have some conditions on the medical restrictions list, I couldn't do it. My search for information stopped at "sorry, you can't become a donor." So, my first reaction to hearing that he might need a bone marrow transplant was "Oh. Well that's common. No big deal, right?" I've assisted in blood transfusions, I've run down to the blood bank to pick up bags of blood....This isn't THAT much different, is it? Well...yeah. It kind of is in some crucial ways. The more I read, the more it frightened me. Gabe will have to have chemotherapy and radiation to kill off his own bone marrow. He has to find the closest possible match - preferably a relative - because kids with related donors have the best chance for survival and kids with a very close match have the next best chance of survival. Wait a minute...survival rates?! With no immune system to speak of for a while, bone marrow patients are at risk for developing infection and disease that puts them in a crisis. Their new bone marrow can perceive their own cells as foreign and attack in "graft vs host disease," the adrenoleukodystrophy continues to progress for about 6 months until the new bone marrow is established and, depending on the individual progression rate, could cause a great deal of damage or even prove fatal in that time frame. I want to sugar coat this...As I read stuff online, I find over and over that the uncertainty and bleakness of everything that talks about ALD is frightening and depressing. I want to put on a nice happy, positive spin so any ALD parents out there reading this as they search for answers will feel less frightened. What can I say? I'm pretty scared, myself.
The qualifier in all of this is that I have supreme confidence in the capabilities of Duke Hospital. Maybe that's naive of me and maybe that smacks of the same superstition in doctors that usually annoys me when it comes from other people, but he really does have a team of experts. They aren't God(s), but he's in good hands. For goodness sake, Dr. Loes himself evaluated Gabe's MRI (and gave it a 0, in case you were wondering). Just today, a world renowned immunologist walked into Gabe's room to borrow his sink to rinse her cup. There you go....routine brushes with scientific greatness at ol' Duke Hospital.
I wasn't really prepared for what to expect from the initialization of bone marrow transplant procedures, so I want to share a little about the steps. His first visits have been for evaluations: meeting the team, MRI, EEGs, nerve testing... All of this information is to give the doctors a picture of exactly where Gabe is at in disease progress. The MRI was about an hour long, the meetings with the bone marrow team comparatively short, EEGs and nerve studies took about 6 hours. Nothing was painful. Some tests were incredibly boring - like when he had to watch a screen of flashing checkerboard patters for 2 hours - but not so bad. During the nerve conduction portion, he did have to have thin needles inserted into his arm and leg on one side, which he said was uncomfortable. The electricity that they used to test his peripheral nerves felt weird, but his reaction showed that he thought it was kind of cool. Here he is during the visual portion.
And again during the EEG and auditory stimulation.
His ACTH levels (the hormone that the pituitary produces to tell the adrenals to work) is still high, so his hydrocortisone dose will be adjusted until they find the right dose for him. They ran another blood test to be sure that Gabriel really has ALD and it came back consistent with the diagnosis. At that point, Dr Tim said that he would recommend that Gabriel proceed with the bone marrow transplant because it is very risky to let him go untreated given what this disease does and the uncertainty of how fast it could progress. I guess some part of me was expecting them to say that since his brain looks good, they didn't want to do the transplant. I swallowed around a lump in my throat and kept talking to the team. I forgot to ask him when we would get started with the transplant, so as we were leaving I posed the question to Gabe's wonderful nurse. She said that they would probably begin when our daughter's tests come back indicating whether she could be his donor or not. There will be a few more tests, like making sure his organs are all okay, then they will probably get started in the next few weeks. We can expect the transplant to start in April.
Gabe didn't take the news well. I think he's in the anger and denial stages of grief. He wanted to leave the hospital after he found out that they want to proceed with the bone marrow transplant and said, as he walked away, that he is "not doing this." He's just a child, so I think that his rational is that if he doesn't do the procedures, he won't have to deal with the disease. I think his reaction is normal. I caught up to him and gave him space to be angry. Then, when he was ready, I hugged him and let him cry, trying to be brave for him and not cry right along with him. He went in to the next round of tests willingly and was very brave even though he was afraid of the little needles.
Our twelve year old daughter, Cai went in this week to get some blood tests done to find out if she is a carrier and also if she is a bone marrow match for her brother. Cai has a 50/50 chance of being a carrier and a 1 in 4 chance of being a match. If she is a carrier she can't be his donor even if she is a match. The process for his bone marrow transplant will begin when we find out whether she is able to be his donor.
There is a bone marrow drive today just a few minutes from my house. It's being hosted by ABC 11 in honor of Robin Roberts and ABC 11 family member Joshua Chavis. I can't donate, but I plan to stop by with a thank you card. Who knows - maybe one of the donors that they register today will save my son's life.
I wasn't really prepared for what to expect from the initialization of bone marrow transplant procedures, so I want to share a little about the steps. His first visits have been for evaluations: meeting the team, MRI, EEGs, nerve testing... All of this information is to give the doctors a picture of exactly where Gabe is at in disease progress. The MRI was about an hour long, the meetings with the bone marrow team comparatively short, EEGs and nerve studies took about 6 hours. Nothing was painful. Some tests were incredibly boring - like when he had to watch a screen of flashing checkerboard patters for 2 hours - but not so bad. During the nerve conduction portion, he did have to have thin needles inserted into his arm and leg on one side, which he said was uncomfortable. The electricity that they used to test his peripheral nerves felt weird, but his reaction showed that he thought it was kind of cool. Here he is during the visual portion.His ACTH levels (the hormone that the pituitary produces to tell the adrenals to work) is still high, so his hydrocortisone dose will be adjusted until they find the right dose for him. They ran another blood test to be sure that Gabriel really has ALD and it came back consistent with the diagnosis. At that point, Dr Tim said that he would recommend that Gabriel proceed with the bone marrow transplant because it is very risky to let him go untreated given what this disease does and the uncertainty of how fast it could progress. I guess some part of me was expecting them to say that since his brain looks good, they didn't want to do the transplant. I swallowed around a lump in my throat and kept talking to the team. I forgot to ask him when we would get started with the transplant, so as we were leaving I posed the question to Gabe's wonderful nurse. She said that they would probably begin when our daughter's tests come back indicating whether she could be his donor or not. There will be a few more tests, like making sure his organs are all okay, then they will probably get started in the next few weeks. We can expect the transplant to start in April.
Gabe didn't take the news well. I think he's in the anger and denial stages of grief. He wanted to leave the hospital after he found out that they want to proceed with the bone marrow transplant and said, as he walked away, that he is "not doing this." He's just a child, so I think that his rational is that if he doesn't do the procedures, he won't have to deal with the disease. I think his reaction is normal. I caught up to him and gave him space to be angry. Then, when he was ready, I hugged him and let him cry, trying to be brave for him and not cry right along with him. He went in to the next round of tests willingly and was very brave even though he was afraid of the little needles.
There is a bone marrow drive today just a few minutes from my house. It's being hosted by ABC 11 in honor of Robin Roberts and ABC 11 family member Joshua Chavis. I can't donate, but I plan to stop by with a thank you card. Who knows - maybe one of the donors that they register today will save my son's life.
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