Day +236: So what does a loss of a bone marrow transplant mean for Kiira? It means we are back at square one. It’s as though we just learned she has severe RDEB (based on genetics and biopsy–if anyone out there has no collagen VII production and doing well, please let me know, because I can’t find that in research!) and there’s nothing we can do for it but maintain good nutrition to aid her body in healing the constant wounds on her body and keeping up with bandage changes and wound care to avoid injuries and infection.
The loss of a transplant does NOT mean Kiira is in bone marrow failure, which definitively requires a second transplant and is very serious. Kiira’s own bone marrow seems to be functioning fine, as are her other systems, despite low doses of chemo and radiation prior to her bone marrow transplant. We don’t know if a second transplant is an option for her. First, she only had one complete match. So any other match would be inferior and maybe less likely to work. Second, a second transplant means putting her through that much more chemo and radiation. My guess is that it would be even more dangerous the second time, but it has been done many times in the history of BMTs and 3 times in EB patients. Third, despite her diagnosis, she is doing quite well–her wounds are not life threatening at this time.
The BMT is the only systemic approach at this time to help stop the progression of EB. We still believe it’s a good choice for severe EB cases. Patients have been told for over 20 years that a cure is on the horizon. While they’re closer than ever, the research on animals and then human trials take years to complete. So while you wait, to stop the progression as early as possible, is a good thing. It really sucks that the BMT didn’t work for Kiira, but that’s not a reflection of all BMTs, EB or the BMTs ability to help other patients. I get that it’s not for everyone, but the research and theory behind the BMT to help with numerous genetic conditions like SCID, EB, and Osteogenesis, to name a few, is valid and we do not regret our decision to do the ONLY thing available to help Kiira.
Now, all we can do is wait while this horrendous condition takes over our beautiful baby girl. RDEB is progressive. It doesn’t go away. It doesn’t get better with age. So we pray that she doesn’t suffer too much pain, debilitation, or the many other things to come. And pray for a cure.
Day +235: We received news we prayed we wouldn’t hear, but we heard it. The boost did not help Kiira and her numbers are lower than ever. The CD3 (T cells) are 36% donor and the other cells, CD15, remained 0% donor (ideally, these numbers would be 100%). The biopsies are still in progress, but with so little donor cells I don’t expect to see much, if any Collagen VII. I don’t know what the next steps are, if there are any. To say I’m disappointed that she is losing her bone marrow transplant, my only hope medically, is an understatement, and there’s so much more to say, but for now, I need to have a long talk with God.
Day +234: Kiira hasn’t done much more walking over the past few days, but we had another morning of fun and exercise at gymnastics on Thursday. We spent the day yesterday at Jason’s parents to celebrate grandpa’s birthday and Kiira loved sitting in their grass. I don’t think she has done it before–or at least she doesn’t recall, so she was very interested in it and got so mad when we had to go.
Today I took her on a long walk and she is so content in her stroller taking it all in. She loves getting outside anytime she can and crawls to the door and says “bye?” because she wants to go out. I’m really hoping those donor cells are growing so we can come off the immunosuppressants in April and she can be out without her rain cover. Though we venture out a little more, we are still very cautious about taking Kiira in crowds or with a lot of kids, especially with all the illnesses going around right now.
Later today, the girls gave me an early birthday present since they couldn’t hold back the surprise. It was a butterfly necklace with Kiira’s birthstone. EB identifies with a butterfly because the fragility of the skin is like the fragility of the wing, so they’ve become very symbolic to us.
Kiira also got a full bath today, which is still rare. She loves splashing in the water, but it’s so hard to completely unbandage her all at once. Her knee is healing amazingly fast. It has another small blister extended out from it, but we are amazed by the healing of the main wound, as well as her wrist. If the BMT helps even this much, so that we avoid infections and chronic wounds, I would say it’s worth it.
Tomorrow we get labs and our evaluation for physical and occupational therapy. Although Kiira is doing well, we notice some delays and her right foot is turned in significantly so we hope the tips they provide in therapy, will get her where she needs to be.
Thank you for your prayers and comments. The path isn’t always easy, but hearing your words of encouragement and knowing you’re praying, is soothing to our souls. So thank you.
Day +231: Kiira took 2 unassisted steps yesterday and 4 today. She was so proud of herself, that you can see her determination to try again and again in this video. With EB, we hear of many kids who aren’t walking for years, so to make it this far at 16 months, we are so proud of her too!
Kiira Walking Video
Kiira suffers from Recessive Dystrophic Epidermolysis Bullosa and this article, written by another mom with a child with EB, helps sum up the condition, its daily requirements and toll it can take on our children and families.
Day +229: Today, Kiira had a good day. We got labs done nearly painlessly to test for Kiira’s donor cell levels. Pray they are higher than ever and stay up!
Then she got to play with her aunt Kiki while I worked and later had a tear-free bandage change full of laughs and kisses. Love this girl so much!
Thank you so much for your continued prayers. We know God is faithful and able to do more than we can imagine.
Day +228: The painful side of EB.
But nothing a balloon couldn’t fix when we told her we were all done relieving the blisters and cleaning the wounds. Our sweet Kiira…
Day +227: Happy Valentine’s Day! We got to spend the beautiful day with our 3 loves. The girls and I went to church, and then we had a picnic lunch at the park, came home for naps, and spent the evening at my parents, enjoying their hot tub. Plus, Jason knows I’m sentimental and he got me a book made with all of Kiira’s posts from the last year. It’s amazing to see how far she has come and how many blessings we have received since she was born. My heart is full of love tonight! ❤️
Kiira’s knee is still a bit raw, but looks better than yesterday. And for my parents sake, I am in no way blaming them for her injury yesterday. The bandages slide down and her knee was unprotected. It’s frustrating, but it happens on her feet all the time and is probably one of our biggest issues.
After Colton’s passing last night (he didn’t have EB, but was next door to Kiira in the BMT floor), we are reminded of how fragile this process can be, and how truly fortunate we were that Kiira is doing so well. I try to push back the survivor’s guilt that peeks through and just be thankful. By the way, there are others doing very well too and others in the midst of their BMTs for EB, and I know I haven’t spent any time talking about them, but because of the positive stories, we felt a bone marrow transplant was successful enough to take the risk for Kiira. We try to enjoy every moment with her, while holding onto the hope we have in Him.
Day +226: We have had some great days with Kiira’s skin. We had no blisters at all yesterday and we want to celebrate those moments, but this world makes me think I’m ” jinxing” it by celebrating and being thankful, because it means the next day will be bad (I know, not very positive).
Similarly, today we had a family outing. We started with a walk and it’s not even 70 degrees, but Kiira got so sweaty that it made her face rashy and itchy. (I don’t know what I’ll do when it’s 110 this summer!) Then we took the older girls to lunch and to see Disney on Ice while my parents watched Kiira. We had such a wonderful time, not thinking of any of our cares and enjoying one of the rare times the 4 of us go out together. When we returned, we found Kiira’s knee bandages covered in blood, with the largest, deepest knee wound to date, because her bandages slid down. I read about other kids who knees never heal and the bone is showing and I pray my child never gets there, but it’s the beast of RDEB. She cried “mommy” and some gibberish as we washed and rebandaged it, and I wanted to cry, but within a minute, she was smiling and being her goofy self. EB sucks, and I want to get down and think about all the things it’s taken from us, but I can’t, because Kiira doesn’t. And we are home, with our baby girl and get to go on outings, and have many good days, and not everyone has that opportunity. We have so much to be thankful for and we have to keep fighting for all those who have lost their battles.