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Monthly Archives: November 2015
Day +150: Kiira is doing great. She mimics so many of our words and says “walk, walk” as she has us help her walk around the room. She has a bit of a cold but no fever, so hopefully it doesn’t turn into anything worse with her lowered immune system.
We have so much to be thankful for. We got a package from KicKee Pants with about 10 new outfits for Kiira. Thank you so much to those who purchased them for her. We are thankful for our family and how they support us and are willing to watch Kiira so I can do things like grocery shopping or go to appointments. We are thankful for health insurance: a $318,000 hospital stay and we only had to pay $500. We are thankful for Kiira’s health. She is still healthy and her skin is doing well. And we are thankful for the prayers and support from all of you.
While we are so thankful for so many things, our hearts go out to kiddos like Anton. He is doing better than last night, but still in critical condition. Being in the EB community is not often easy, as there is a lot of heartbreak, but it’s amazing to see God’s faithfulness time and time again.
Please continue to pray for Anton and Kiira’s donor numbers to increase. Thank you!
Day +145: Today we had labs. 90 minutes, 5 pokes and 2 blisters/cuts later, they got Kiira’s blood and a flu shot. I had all 3 girls with me in the lab and Kiira’s sisters were angels. I don’t spend a lot of time talking about them but I am amazed by how they have adapted to having a time-consuming sister–actually more than adapted. They love her so much and would do anything for her. They were saying how scared they were for her to get poked and I told them they can be scared, but they need to be strong for Kiira and do things to make it fun or make her laugh so that she won’t be scared. So they sang “Jesus loves me” to Kiira during the pokes and it was the sweetest thing. I couldn’t be more in love with my girls than in these moments that capture my heart so completely.
Day +142: today we our visit to the Stanford EB clinic. The visits with the doctors were fairly brief, as they thought Kiira looked great. To show off for PT she even decided to start cruising today (walking while holding on to things) and wants to do it all the time now. They had some questions about the BMT, wondering if it has helped her, and though we don’t have the answer to that, we can say it hasn’t really hurt her, as far as know. The outcome of the visit: we will need start occupational and physical therapy every week and see a dentist at UCSF when she gets more teeth. The mouth is so sensitive due to EB and somehow it affects the enamel, causing rapid tooth decay so UCSF specializes in EB dentistry and hopefully we an try to save Kiira’s teeth. Going forward, we will visit the Stanford EB clinic once a year, since we will also be going to Minnesota once a year. However, we might get to do Scioderm Phase III trial at Stanford early next year so then we have to come every month for evaluation. Scioderm created, Zorblisa, a topical cream to help with healing and itching. We are excited to be a part of research and another opportunity to get Kiira some help, if she can get in the trial.
On another note, we heard from Dr. Tolar today that Kiira will get a boost of donor cells in January but he is trying to decide on the pretreatment regiment, meaning either more chemo or increased immunosuppressants. Her BMT is a part of a trial and so few people have had a BMT for EB that there isn’t a set protocol for boosts. I have so many questions but will wait to hear from them as I know it truly is trial and error to see what works best. We don’t want her to lose her transplant, so we pray that Dr. Tolar can make the best decision for Kiira to give her the best outcome possible.
Tonight we left for Stanford because of an appointment with the EB clinic tomorrow at 8:30am and it’s a nearly 3-hour drive without traffic, about 6 hours with traffic. Another doctors appointment, another missed birthday dinner, another drive and more coordinating care for the other 2 kids, but we made it! The trip brings back so many memories of our Stanford trip from last December. We were so new to dealing with EB and the trip was filled with anxiety and hope. We left there with new knowledge of hand wrapping and what to expect down the road from EB, but also in tears, feeling our only hope would be a bone marrow transplant, despite their misgivings about it. Stanford has its own trial, and its eventual application should significantly benefit chronic wounds, but EB affects every body system in some way. A skin graft for Kiira’s hand wouldn’t fix her almost inevitable esophageal strictures, or inability to absorb nutrients, or keep her teeth from falling out. It’s not a systemic treatment, but EB is a systemic condition. The only hope at this point is a bone marrow transplant. I remember thinking how scary and drastic that seemed– to put my otherwise healthy child through chemo and radiation? To wipe out her immune system? To move to Minnesota for a minimum of 4 months? — now we face this Stanford visit being on the other side of all that scariness of the unknown and all the planning. No, Kiira’s EB is not gone, but I feel a peace knowing we did all we could for her up to this point and she is happy and thriving. I hope it can slow the progression enough that someone can find a cure. I don’t care whether it’s Stanford, Minnesota, Cincinnati, or whoever, as long as someone is working on it. As long as someone cares. Last weekend David Spade hosted Rock4EB, a concert by Ed Sheeran with a ton of celebrities and EB families in attendance. I don’t usually get caught up in celebrity life, but they pull some weight and funding that the average person can’t and I love that EB has caught their eye.
Last year we also spoke to the retiring professor of dermatology and pediatrics at Stanford, Alfred Lane, who spent the past 20 years trying to find a cure. He came to see the “famous Kiira” because her local news story aired that day and had circled around the world and Yahoo! News called him for an interview about it. That news story changed our life and I’m so grateful for the love, support, donations and prayers we were (and still are) shown because of it.
Meanwhile, we are still waiting to hear about our Minnesota trip and whether or not Kiira will get a boost of donor cells. I’m still not even sure a boost will help increase her CD15. The process over the past year sure has tested (and hopefully grown) my patience and I know we will always be waiting for the next visit, the next blood test results, the next day to see how much EB will affect Kiira’s life going forward. I have to keep reminding myself to have faith as we wait patiently.
To end on a note of thanksgiving, we were able to stop by my aunt and uncle’s house on the way here to see my cousins, their kids and the newest addition to our family. I am so thankful for my amazing family!
Day +138: We’ve been living life semi-normally the past week. We didn’t have to do labs this week and had no other doctor appointments, which was so nice. Kiira and I even took a walk one day and she loved being outside, although she still has to be covered in the stroller with the rain cover. Thankfully, Kiira’s hand is still healing, as is the bump she got on her head.
My sister-in-law graciously planned a girls-only welcome home party for me over the weekend. It was a great night with friends and family without the kids. I felt very loved, so thank you to everyone who came and for thinking of putting it together for me!
This week we have her speech/feeding appointment. I know we have made so much progress in the past month, but we still have a long way to go. We also head to Stanford Thursday night for an early appointment on Friday morning at their EB clinic. We will see multiple specialists again and I’m very curious as to what the day will hold for us, but we’ve been before so at least I somewhat know what to expect.
Thank you for your continued prayers. We still don’t know what Kiira’s donor cells are doing, but we pray the numbers are going up and creating useful Collagen VII so that her skin get stronger.
Day +132: The girls are feeling better and Kiira is still healthy, thankfully. Plus her hand that I stepped on is still healing so well. We were talking last night about the fact that we don’t think Kiira doesn’t have fingerprints because she already has so much scarring on her fingers, but she still uses her hands so well. Nothing slows her down!
Today we worked on our travel arrangements for our return trip to Minnesota in January. It’s kind of crazy to think we’ll be heading back there so soon, but it’s also oddly comforting as it gets us more information about Kiira’s bone marrow transplant results. Now we only have to go in for labs every 2 weeks, so I’m sure Kiira will be happy about that!
Overall the past few days have been good, so I’m just feeling thankful for the reprieve and enjoying the endless giggles from my girls today.
Day +130: It’s been an eventful weekend here. On Friday we put Kiira to bed before my aunt and uncle came to babysit so we could join friends to celebrate their birthday. Later that evening we got a text that Kiira had been crying for 40 minutes and was gagging and we didn’t know if it was because she was worked up or if she had a blister in her throat or what, but we were about 40 minutes away and terrified as we drove home, because usually Kiira sleeps well through the night and we haven’t heard her gag/choke in a very long time. Of course by the time we got home, she was back to sleep and appeared to be ok. The next morning, we could see a lot of mouth sores, so it’s possible a blister was further back, causing her to gag, but we’ll never know.
Later yesterday, the older girls got a fever. We kept Kiira in a separate room because if she gets a fever, she will be hospitalized for at least a couple days. It’s only been the first week back at school and already the kids are sick. I keep trying to make the right decisions for our family and no decision is perfect–I knew getting sick was very possible as the kids got back to their activities and we can all fight it off, but Kiira’s lowered immune system may not be able to. I hope Kiira can stay healthy, so please pray for her.
As for her hand injury–the layer of skin that detached on her thumb and pointer fingers are drying up. Eventually the skin will fall off and hopefully we’ll find stronger skin underneath if the donor cells went to that area. Kiira is back to using her hand normally, so we are so happy about that!
Kiira is eating more by mouth, which is really great. It does seem to be causing more mouth sores, but hopefully we’ll figure out what works best for her. She has 5 teeth coming in–one just popped through, so she wants to chew on everything, which doesn’t help.
We got word that we’ll be going to the Stanford EB clinic on November 20th, where we’ll see numerous specialties. We haven’t been since last December (such vivid memories of that day!) and they weren’t supportive of Kiira’s BMT, but the team is very knowledgeable about EB, so I’m anxious to go and show them a BMT isn’t such a terrible thing. We still don’t know if the BMT helped Kiira or if she is losing her transplant, but we’re trying to take one thing at a time.
To end with thanksgiving, it was so wonderful seeing friends on Friday night. With the girls sick today, we got to enjoy a rainy day inside, relaxing and my brother and dad came by for a visit and then my aunt and uncle brought us dinner. We love our friends and family and are so thankful for their presence in our lives!
Day +127: The engraftment is back: CD3+ 74% from hematopoietic graft plus 1% from MSC graft, CD15+ 5%, and skin 2% (this may not seem like much but it is important, according to Dr. Tolar). So none of that probably means much to you, but CD3 and CD15 are T cells in the immune system that are evaluated to see how many are donor cells vs. Kiira’s cells. Unfortunately these numbers have decreased since the 30 day and 60 day tests. The most alarming number to me is the 5%. While Kiira could benefit from a boost of donor cells (same donor, just an additional dose of her cells) when we go in January, I’m afraid if it hits 0, we may lose the graft completely. So we need some prayers for these numbers to increase. Thinking about putting her through another bone marrow transplant, if that would even be a possibility, is not even an idea I can fathom right now.
We are waiting on the biopsy results to know if she is making any collagen VII yet. That’s probably the more important aspect of this all, because that’s what we need to make her skin stronger.
Tonight’s bandage change was rough and I wish I could take it all away from Kiira.
On a note of thanksgiving, the girls returned to gymnastics tonight. The gym has been so incredibly supportive of our family this year and having gone there for nearly 4 years, it felt like returning home. It really made my heart happy to see the girls do something they love so much. My in-laws were also here all day to spend time with Kiira so I could work and take the girls. It was really nice to visit with them.
Day +126: Update about Kiira’s hand: she didn’t use her hand much today to play or eat, but she did crawl around. When I did bandages late this afternoon, I found her entire thumb and pointer finger had skin floating/detached from the skin below it, but the skin was still there, which is good because it heals faster and usually remains free of infection. Her third finger just had a little blisters, but nothing more than usual. So it all looked better than expected and we’ll just pray for faster healing. I’m so thankful it wasn’t worse and I appreciate all your prayers!
I’m also thankful today I was able to visit a moms’ group. It is nice to get back into having a social life, and be encouraged by women in my church. It made me wonder if I’ll ever be able to put Kiira in a childcare class at church because of her skin fragility. I just feel like that’s a lot of pressure to put on a child care worker (having been one), or a teacher in school, or just any babysitter. At this point, I’m fortunate to have family watching over Kiira with care and diligence so we can go to church or on a date or to an appointment, but I know that can’t go on forever. I hope Kiira experiences enough healing that someday I can let go and let her experience life.
Day +125: With November being a month to focus on thanksgiving, I have been contemplating all the things I have to be so thankful for: my family who is so helpful to me and with my kids–especially this week with my aunt and uncle being here so early to care for Kiira in the mornings, for my girls and the past 6 months we had together to bond, but also for their school where they started today, and for the prayers and support of so many friends and strangers who have helped us through the past year. I look back to last November and I couldn’t find much to be thankful about in the midst of learning how to care for Kiira, so I have a lot of making up to do. God has worked in amazing ways to show me that no matter what we go through, He is there.
The past few days have been good. Relatively uneventful and Kiira has been doing great. Today she had labs drawn and she didn’t fight it when they held her to draw blood. They got it in one poke and she cried for only a few seconds. Such a difference from last week! She also had her 1-year well checkup and everything looks good.
The girls had a good first day of school. It was more sad for me than I expected. Last year I couldn’t wait to have them out of the house, but all 3 girls have bonded so much and I’ve loved my time with them. Even though Miia would fight me about homeschooling, it was amazing to see her read a book or figure out math problems on her own. However, because I do work part-time, the time had to come when I couldn’t be with them 24/7.
All was good until this evening. Kiira was playing by the fridge until I turned around, took a step and felt her hand beneath my foot. My heart sunk, she screamed as 3 of her fingers filled with blood because I tore the layers of skin apart on them. I knew I had to poke them immediately or the blisters would grow. I don’t know how many pokes I did, but more and more blood and more pokes and squeezing and tears… It was awful. I remember reading a book or blog about someone who did that to their EB child and degloved their entire hand. I was horrified and try to never to take a step unless I know where she is. I get on the girls all the time about it, but I was flying solo tonight and in too big of a hurry to get things done. I have no idea what I’ll see tomorrow but I pray, pray, pray, for quick healing and that the blisters won’t have grown or cause fusing. To be thankful for something in the situation–she was incredibly subdued for bandage changes so I got them all done by myself without any further issues.