Wednesday, February 24, 2016

Facebook update from Help Anton

I think as a whole the EB community seems to be afraid of bone marrow transplants for EB. I think the majority of people's fears are based off of rumors and not the truth. I was terrified of transplants when we brought Anton home but it was mostly from hearsay and not based off of any facts. In 2014 doctor Tolar spoke at the Debra Patient Care Conference. He shared the FACTS about his research and shared that 9 out of the last 10 patients with EB had survived transplant. At that point is was safer to have a bone marrow transplant for EB than for leukemia. I HIGHLY recommend anyone considering transplant to listen to the recordings from that conference (, or attend the conference this year where Dr. Tolar will be speaking. I also HIGHLY encourage anyone considering transplant to talk to other parents who have recently gone through it. The protocol has changed so much from where it started. The other thing I would say is contact Dr. Tolar's team directly with any questions. Find out from the source instead of going to people who may have outdated information. With all that said... Last year was a hard year as far as BMT's for EB goes but I think it's important to look at the facts. Two of the sweet babies who passed away had junctional herlitz. Anton's BMT went amazing. He was 100% engrafted with his donors cells, and over 50% engrafted in his skin (which is a huge number). Anton hardly ever got blisters and hardly ever got new wounds (after transplant). His transplant worked and it worked beautifully. He died of intussusception which is NOT a complication of transplant or EB. Kiira didn't fully engrafted with her donor's cells, which is heartbreaking but unfortunately does happen sometimes with transplants (not just transplant for EB). The thing about BMT for EB is it isn't a cure and is a trial so no two patients are going to have the same results. Although I do think it is the best chance people with EB have at this point. I always tell families considering transplant to make the best decision they can at the time. Well really that is my advice about all things EB. You pick the lesser of two evils, there is hardly ever a good and super easy choice when it comes to treatment with EB. It took us a really long time (about 3 years) to decide we were ready to move forward with transplant. The reasons we decided to move forward are... We saw so many people will RDEB die before their 20th birthday and that scared us. Part of the reason we chose to move forward was in 2014 the last 9 out of 10 patients who had a BMT for EB had survived. The right ventricle of Anton's heart was dilated, this almost always turns into cardiomyopathy in kids with RDEB. We knew if Anton developed cardiomyopathy he could be disqualified from transplant. Anton's quality of life had also gone down in the few months before we made our choice. He was in pain most of the time (despite some pretty strong meds), he was getting corneal abrasions all the time, and he was just having more bad days than good. All of those thing (plus so many more things) played into why we chose transplant. I will say without a doubt given the choice we would have chosen transplant all over again. It GREATLY improved Anton's quality of life (which I know was hard to tell from FB). He hardly ever got blisters (maybe 5 totally in 5 months), his skin was so much stronger, he hardly ever got wounds, and went from having corneal abrasions ALL THE TIME to never having another one post transplant. He went 8 months without a corneal abrasion! Tolar said the eyes are the only place that transplant doesn't go to but several of his patients stopped having abrasions after transplant. Yes transplant is scary (it is a MAJOR medical procedure) and there are no guarantees with it at all but there are also no guarantees with doing nothing. I have so much love for our EB community and this isn't a push at all to get anyone to consider transplant. Instead this is an encouragement to anyone considering transplant to get the facts from a reliable source. Big Hugs! - February 24, 2016 at 09:11AM

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