They call the week before Kiira gets admitted, “Work-up Week”. They do a slew of tests to make sure all of her systems are functioning adequately enough to withstand treatment and to get a baseline so they know if something starts failing during chemo, radiation or after transplant. It’s an exhausting week of meeting with doctors, being inundated with information, signing forms to give our consent to all the awful things chemo and radiation do to a person, but we made it through–more importantly, Kiira made it through with flying colors! Here is a recap of the week:
Day -18: Today I had the pleasure of meeting Help Anton ‘s mom and Christie Zink from I Refuse EB. Both of these beautiful women have been an encouragement to me since Kiira was born and I felt privileged to meet them in person.
Plus, we received an amazing gift from my extended family to decorate our home. These family photos will be cherished for years to come!
Kiira is doing well and we hope she will pass all of her tests in this coming week, as they check all of her major organs. It should be an enlightening week!
Day -16: Kiira started her work-up today, which tells the BMT team about her overall health so they can be fully prepared for the BMT. She had a physical, EKG, echocardiogram, chest X-Ray, and blood drawn for labs, all without tears–even the needle poke! We also met with Integrated Health, nutrition, and a PA and Dr on the work up team. Her weight is back up so she is following a 30% curve, which was a relief, since she dropped to 10% in April after her gtube surgery.
We felt Kiira was handled so much that we gave her a full bath in the bathtub without wrapped hands for the first time ever. Kiira loved it! Doing bandages at the end of a day of testing was not easy though and Kiira was ecstatic to get on the floor to play finally at 5:30.
Everyone commented on how good Kiira looks, so hopefully the healing after BMT will be quick. At least that’s my prayer!
Plus, Kiira got to meet Anton (Help Anton) and his mama today, which was such a treat!
Back at the apartment, my mom and Kiira’s sisters, played, walked and swam. Tomorrow is another busy day of appointments so keep us all in your prayers!
Day -15: Although we were at the hospital for 6 hours today, it wasn’t as busy. We started with a radiation consult, where we learned about the risks of total body irradiation, including possible sterilization. At first I was upset, but I have to remind myself that with RDEB, most of the these things will be an issue anyway. Hopefully the low dose at her age won’t leave her with as many side effects.
The next appointment was with Occupational Therapy for a hand wrapping consult. She was impressed with the technique we use and Kiira’s use of her hands, which made me happy since I was so worried about it with our over-wrapping for the first 6 months of her life. We are wrapping in a way that helps prevent or slow webbing, but still give her the ability to grasp and touch things. It’s nice to be reassured that we are doing something right!
After that, we had time with our social worker, who talked to us about stress relief, among other things.
Then we had our GI consult. Tomorrow Kiira has an endoscopy, and much to my relief, he explained that he will go through her gtube to prevent injury to Kiira’s mouth and upper esophagus. They are looking for strictures and webbing of the esophagus. If she has any, they will do a dilation at the same time.
Since we walked to the hospital, we enjoyed a nice walk home as well. Kiira loves being outside so we don’t hear a peep from her while we walk.
We can definitely use prayers for tomorrow. Kiira will be admitted around 8:30am and put under for her central line placement, 5-7 biopsies, and the endoscopy. Please pray that the doctors work carefully on Kiira, don’t find esophageal strictures, and we have no issues with the central line.
On a side note, we also saw Prayers for Cole’s mom. This little guy was getting his BMT today, so please pray for him too!
Day -14: Kiira’s surgery went well. Her central line is in place and ready to use for the very frequent blood draws they’ll do and to administer medication. Then she had her endoscopy. They found inflammation in her upper esophagus, but no strictures or webbing. She is on anti-reflux meds to reduce inflammation and hopefully stop further damage (plus the BMT will help with that). Then they did 5 skin biopsies and a skin integrity test. It took7 minutes for Kiira to get a blister, which apparently is average for EB at her age. We will get tests too to compare, but a non-EB carrier was tested at 45 minutes and carriers, like us, will likely be somewhere in between. Then we went in the OR to do her bandages while Kiira was still sedated. They handled her very carefully, so besides the necessary inflictions, she looked good. Kiira is now home and resting. Thank you for your prayers!
Kiira also got to see Anton (Help Anton) again today! Having others here, going through what we are, makes us feel like instant family, which is such a blessing.
Day -12: Kiira is back to her normal, happy, active self. Yesterday we met with the BMT coordinator to discuss the BMT process, including the types of chemo and medications she will be on. It’s quite a list, but necessary for the new cells to make their home in Kiira’s bone marrow. We also met with infectious diseases and got Kiira cleared by them–she is healthy enough from an infections stand point to go through this process. He reminded us that there will be bumps in the road, but there is a light at the end of the long tunnel and we look forward to reaching that light!
Last night we went to the Ronald McDonald House for dinner and Bingo with Help Anton and Reid Davis Underwood. That is an amazing facility for kids going through medical issues. They really make life easier for the families, so save your soda can tabs and donate to RMH!
Today Kiira had GFR, a 4 hour test that checks her kidney function, and we learned how to flush Kiira’s central line. It’s a little intimidating because you have to do it in a sterile environment, and we aren’t sterile with her skin wound care, but I’m sure we’ll learn how to do this as easily as we do bandages now. We also met with pharmacy to learn more about Kiira’s upcoming medicines and side effects. It’s a long, uncomfortable list, but many EB sufferers live on pain and anti-itch meds, antibiotics, sedatives, among others, so hopefully this year of craziness will prevent us from a lifelong prescription pad.
We got to say hi to Dr. Tolar, one of the genius doctors behind EB research that’s led us to this point. I’m so thankful that God gives some people the ability to enjoy science and dedicate their lives to research that helps so many people.
We get the weekend off, have our exit consult on Monday and if all goes well, Kiira gets admitted next Wednesday. It’s been a long week, but we are excited the day leading to recovery is almost here!
God is good!
Day -9: We are finally below double digits! We had a great weekend off and celebrated Father’s Day at Minihaha Falls. Today’s exit meeting went well. Kiira is in good shape except she has slightly slow kidney function. Since some of the meds slow the kidney even more and trigger high blood pressure, they’ll keep a close eye on that. Kiira and the donor also tested positive for CMV, a common cold-like virus, but chemo and radiation don’t always kill it off, which can cause major issues when you have no immune system, so Kiira will get anti-virals for that. The positive side of it, if there is one, is that I can keep giving her my milk. If I was positive and she was negative, I would not be able to. We signed off on all the crazy side effects and we are moving forward to have Kiira admitted at 11am on Wednesday. She will get connected to an IV for lots of fluids and start chemo Thursday.
It’s been a long road and I know it’s not over, but with the amount of blistering Kiira has had the last couple of weeks (bigger and more than ever), I am reaffirmed in this decision. I would appreciate prayers as we go forward: for the donor; that Kiira will fly through this without complications and God will use this to heal her; for us, as parents, to have faith and strength; for my mom’s strength and energy as she watches Kiira’s sisters everyday; and Kiira’s sisters–that they will understand why we aren’t around much and be strong through this too.
Thank you!
Day -8: As I sit here putting Kiira to bed for her last night at home until after BMT, I have this bittersweet feeling. We’ve waited so long for this day to come… Today we spent our last day of freedom at Nickelodeon Universe. Kiira enjoyed watching her sisters on rides and when we got home, I observed Kiira rolling, sitting up, and laughing with her sisters. I wonder if she will be the same, happy baby when she returns. I wonder if the BMT will allow her to play with her sisters as actively as they do. I wonder if the BMT results will be enough to make me happy and I wonder “What is enough?” I don’t know yet, but so far Kiira exhibits amazing strength and resilience and as she watches my reaction with curious, soul-searching eyes, I only hope I can be strong for her.
Goodnight world.
In the midst of our own journey, this article popped up. As much I hate that other families have to deal with EB too, it is encouraging to see how other families are using their experience to help others, like this father, who the co-founder of EB Research Partnership, a non-profit aimed at finding a cure for Epidermolysis Bullosa. Father to ‘butterfly’ child: ‘I would give anything to take away the pain’
