Monthly Archives: October 2015

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

After 7 days of driving and staying in a hotel, we made it to our new home to find a freshly cleaned house, a welcome package from a sweet family in our bible study group, and friends and family ready to help us unload. We love our new home and are busy unpacking and settling in. Unfortunately our TV cracked in the move, but we all got here in one piece, thankfully!

The girls were so excited to be back and explored the entire house, and they scattered their toys in each room faster than we could put things away. It’s so hot here, so poor Kiira already has a face rash and we have to figure out a way to keep her cool with all the bandages and long sleeved footies. I wish her skin was tough enough to crawl in a onsie, but it’s just not. At least winter is around the corner, maybe.

It was so great to see familiar faces and we look forward to seeing more over the coming week. Kiira goes in for labs on Monday and has her first doctor appointment on Tuesday.

Our journey in Minnesota feels surreal, already like a distant memory. We don’t know what the next season in life holds for us, but for now we are just enjoying being home.

Today is also Kiira’s first birthday. I wish we were having a huge party and she was smashing her face in cake, but instead she gets to ride in the car for hours as we drive from Minnesota to California over the next week. We went to see Mount Rushmore, but since it’s windy here and wind stirs up spores that can make her very sick, she had to stay in the car and we took turns looking at it. Yesterday, Kiira scratched her eye. It was horrifying, because we could see the skin on the eyeball was peeled back and she wouldn’t open her eye as she cried in pain. I’ve read it can take days to heal, but miraculously, when she woke up, she was able to open her eye today without pain. She has a little redness on the outer corner, but even the pulled skin we saw on the eyeball last night is gone or healed. Thank you for your prayers, because God answered them!

So a one year old–I wasn’t sure she would live to see her first birthday since Epidermolysis Bullosa can lead to death especially in the first year, but she did and she is thriving. She weighs 21lbs and is 28″ tall. She shakes her head no, signs “thank you”, says several words, stands holding on to things, and adores her sisters. She has an infectious laugh and strong temper. Despite having EB, she doesn’t let it slow her down. I am so blessed by God’s faithfulness in providing for her and our family as we adjusted to her medical needs. We pray her second year leads to continued healing and instead of being riddled with fear, that we can celebrate all the joys of toddlerhood.

Day +100: This day is so significant in the BMT world. It marks the earliest possible day you can go home and we got the final word that Kiira can go home!

With that said, we are wasting no time and packing like maniacs to leave tomorrow. We can no longer take our scenic southern route, because Kiira needs to be back home to see a doctor as soon as we get back in town. We did not get the engraftment numbers back yet (tells us how much is her cells vs the donor’s), but we know she has a very good T-cell count and no B cells because of the medication she took to get rid of the Epstein-Barr Virus. It will take around 6 months for those cells to return. Both T and B cells are a vital part of your immune system, so we still have to keep Kiira safe from germs.

Though our journey here is done, at home she will still go in for weekly labs and doctor visits and she will be on an immunosuppressant that will keep us from taking her to gatherings with kids and large crowds, including things like church and even big family gatherings at holidays. While that is disheartening, Kiira’s health, especially in the midst of flu season (flu has a high risk of death in immunocompromised people), is of utmost importance to us and we are so beyond blessed to be able to go home at day 100. We will come back to Minnesota for a week in early January to do another biopsy and round of tests.

We can’t say enough good things about the doctors and nurses at the University of Minnesota Children’s Hospital. They took care of Kiira like she was their own and we are so happy we came here to get her treated. We continue to pray that her donor cells are growing and improving her skin every day!

Day +96: Just to update everyone, Kiira is back home and sleeping after a long day at the hospital. The procedures all went well. She has another 5 holes on her leg where they took the biopsy, but it is close enough to her previous biopsy site that she will only have one set of scars. They tried to do the endoscopy through her gtube site, but the hole had healed up around the tube too well and they couldn’t fit the scope through so they went in through her mouth, which is a little scarier due to the possibility of damaging her airway. Everything went great though–there were no strictures and the inflammation that they saw in her initial scope is gone too! In preparation for her 6 month visit we will have to get her fitted with a bigger gtube, but I think that is a pretty simple procedure. The central line was removed without any issues and it is already so nice not to have those ports dangling from her chest.

We’re hoping to get the engraftment results during Kiira’s final clinic appointment on Friday. Praying for high numbers meaning no boosts!