Category Archives: Uncategorized

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.

Today is the last day of EB Awareness week, but as you know, people like Kiira deal with it every day of the year. Please feel free to ask us questions about EB at any time.

Maybe Kiira didn’t wear a costume today, but she is wearing a shirt with an iron on backing (which used to blister her chest) for the first time and she was ok! Happy Halloween from our little pumpkin!

Day +121: Yesterday Kiira had IVIG to replace IgG, an antibody. It was a nightmare trying to get an IV in her. We tried 5 sites with multiple pokes at each one and I had to unwrap both hands to try the backs of her hands. She is so strong that we had 3 of us holding her down, 1 doing the IV and 1 distracting her but she just screamed for the hour that it took us to get the IV in. I don’t regret taking out the central line, but it certainly made things like this easier! Once we got the IV in, Kiira calmed down but cried every time she tried to lay on or use her arm. I was able to bring her pack n play so she took a little nap. The infusion itself only took about 3 hours instead of 8 like last time, but when we were done, we realized her arm was swollen, so the IV did pull out of the vein. That’s what happens when you can’t use tape on EB skin. Apparently the IgG can still get into her system subcutaneously, so it’s not too big of a deal, but her arm was sore until the swelling went down.

Other than the IV issues, the infusion went well. I also met with a GI doctor and nutritionist. They are on board with reducing Kiira’s calories because her growth curve jumped so high, but her weight to height ratio is low (basically she weighs too much for her height). It’s a good problem to have when it comes to EB though. In addition, they support blended food diets (blending real foods like meat, veggies, etc) rather than just giving her formula and eventually Pediasure, so I’m happy to have their support and guidance. Kiira is eating more by mouth, but not nearly enough to sustain her, so I’m very grateful for the g-tube.

Today Kiira is still doing great. Her arm swelling is gone and she was her normal, happy self, except when I did bandages. She is really showing her strength to fight me, but I was so relieved to find no injuries from yesterday when we held her down for the IV.

She won’t be trick-or-treating tomorrow night, or even dressing up, but Kiira gets a kick out of her sisters’ superhero costumes. Hopefully next year she can participate.

Thank you for your continued prayers. We really appreciate the support!

Day +119: I’ve been focusing on EB Awareness week because it’s so important to me to help everyone understand EB. It will be a part of our family for the rest of our lives. But tonight I want to give an update on Kiira.

Unfortunately one of Kiira’s labs came back with a low reading so tomorrow Kiira gets to have an IVIG infusion to replace IGG, an antibody. She had this once before in Minnesota, but she had a central line and a crib to sit in. Tomorrow they’ll try to keep an IV in without tape while a one-year-old active child sits in a stroller or on a stretcher for 4-8 hours. It should be interesting!

I wasn’t sure having a dog would be safe with Kiira’s lowered immune system, but apparently it’s fine, so our dog Lexi returned home over the weekend. Kiira loves Lexi and says “dog” or makes a barking sound while trying to pull her tail or her face. Lexi does not care for it, but hopefully they’ll learn to get along.

Kiira’s face is getting better. An unfortunate side effect of EB is seeing drops of blood and scabs all over the sheets. It’s from any number of wounds that she has, and oddly, something I’ve gotten used to. As the scabs fall off, I see fresh skin underneath, so I’m sure her face will look better soon.

We’ve also had the opportunity to return to church. Kiira stays home with my aunt and uncle, but we get to go as a family. While watching it online in Minnesota was fine, there’s something beautiful and renewing about worshipping together with other believers. We know we couldn’t have faced the circumstances with Kiira without God and our church, and the many churches around the nation, who are praying for us.

A diagnosis of Epidermolysis Bullosa is life changing. I hadn’t heard of EB before Kiira was born, as most people haven’t. Due to the lack of awareness, there is a lack of resources, medical attention, and funding for research. We were fortunate to be at a hospital with doctors who had at least heard of EB and nurses were so diligent about learning about EB and how to wrap a baby to prevent infection and protect Kiira from further injury. Unfortunately, the bandages needed to keep Kiira safe are not covered by insurance so we went home with bags filled with bandages from the nurses to last a couple weeks and there are amazing organizations and generous donors who help families like us fill the gap, but in some countries, getting access to the bandages are nearly impossible. Not to mention, they cost thousands of dollars per month. In addition, we had to go from 3-4 bandagers (1-2 nurses and us) to 2, one being me and the other a family member or friend whom I trained. It is our responsibility to know if she has an infection, how and when to pop blisters, how to bandage new areas of injury, how to incorporate 2 hours of bandaging into our day on top of regular newborn responsibilities that actually take at least twice the amount of time because of how careful we have to be with Kiira. No decision is easy–what bottle do I use? Do I coat the nipple with Aquaphor or Coconut oil so it doesn’t blister her mouth? What clothes can she wear so that seams don’t cause blisters? How much can I touch her, how do I pick her up, how do I get her in and out of a car seat? How do I bathe her? What toys can she play with? These are just a few of the questions we’ve had to answer and as she gets older, my list grows.

Over the past year, Kiira spent a month of it in a hospital, which isn’t much compared to many others with EB. She has seen numerous specialists, including a dermatologist, hematologist, physical therapist, occupational therapist, speech/feeding therapist, gastroenterologist, ophthalmologist, bone marrow transplant team, radiologist, geneticist and pharmacist. As she gets older, more specialists may be needed because EB doesn’t just affect the skin. It affects the interior path from the mouth to colon, ability to absorb nutrition, major organs like the heart and kidneys, eyesight, muscle and bone deterioration, skin cancer… The list goes on. Like I said, EB is life changing.

Awareness–spreading awareness is crucial. It could mean more support, better skin care products (creams and bandages), better medical procedures, a cure.

Understanding EB is not easy. There are 3 subtypes and Kiira has one called Recessive Dystrophic Epidermolysis Bullosa. Since it’s a recessive gene, as parents, we each carry one gene, but have no symptoms ourselves. Kiira’s sisters are unaffected, but could be carriers. Within RDEB, there are over 500 mutations alone. The combinations are complicated and make every EB patient present differently. Kiira’s 2 mutations have been reported in one other person in the 90s and was classified severe. However, there are modifier genes that can make Kiira’s skin stronger or weaker and based on her current condition, in combination with the bone marrow transplant, we hope she is less severe. We really won’t know and trying to predict the future is impossible. We take one day at a time. Some days are great. We celebrate a day like today where Kiira had no blisters to pop. Some days are hard. Some days I’m sad or angry, but I keep holding onto my faith that God has a plan for all this. I wouldn’t wish EB on anyone, but I do wish everyone knew of EB and could donate an hour of time, a $1 to research for a cure and/or making lives easier, or a bandage to families who need them, or prayers that God will provide families with strength and comfort and doctors with the knowledge and resources to come up with a cure.
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The last week of October is dedicated to bring awareness about EB. There is so much to know, so much we’ve learned, and I’ll try to share some of those things during this week.

There are some great organizations who support families with EB. They provide numerous resources and education about EB so we can be prepared to help our children. So if you’re wondering what EB is or how you can help kids like Kiira, click one of the links below to learn more (there are other organizations too):

http://debra.org
https://ebresearch.org/
http://irefuseeb.org/
https://www.facebook.com/sonomabutterflyfund