Kiira Kinkle

Day +1: Kiira and Miia are both doing great today. We had a few hours where we unhooked Kiira from the IV so she could walk around freely. She gave out a lot of hugs and thrives with her sisters around. She took a tumble though and sheared the skin off her cheek and then hit her lip on the crib and injured it too. She looks very beat up right now but still smiling (pics are from before the fall). 
Reality is setting in that Jason and the girls leave Saturday. I don’t think either of us have in it us to say more at this point, but I’ll spend tomorrow with the older girls and Jason will be with Kiira. Then we will all try to go to dinner while my mom stays with Kiira. 
Catt is also doing well so far, although a little nauseous. 
Thank you for your continued prayers!

Day 0: Today is transplant day!! Miia, Kiira’s sister went in to the OR around 8:30 AM. I was able to be with her while they put her to sleep and though she was crying in that moment, she has been incredibly brave and goes along with whatever we ask of her (except drinking her Tylenol this morning). The procedure took about an hour.  She came out of sedation well, but because they had to intubate her, her voice is hoarse and they knocked out her loose front tooth. She was up walking around, eating and drinking within an hour. Her recovery is quick and fun with a movie, Popsicles and a new toy. She said she had no pain either and didn’t need a blood transfusion later today, so she is now home. We are beyond proud of Miia and so happy she was able to sacrifice herself in this way for Kiira.

The other positive is that her blood was so rich with stem cells–about 4x the amount they hope for. The doctors were pleasantly surprised and we hope that helps Kiira even more. 
Kiira received those cells at 2:30 pm today. Miia was here to watch and got to unclamp the line to start the flow. It was…beautiful. I don’t know how to describe it, but we are so happy to be at this point. Kiira slept through it all, but has been so happy and energetic tonight. Last time, she started getting sick at this point, so it’s really fun to see both girls having fun, celebrating such a monumental day. 
The point of chemo and radiation is to lower Kiira’s own immune system, so now her white blood cell (wbc) counts are very low. She is extremely susceptible to infection until Miia’s cells start to grow. Kiira still has 2 doses of chemo over this weekend too. 
As Miia left tonight, she asked if Kiira’s owies will be gone tomorrow. Such a sweet, innocent question, and one that comes up often as we talk about doing a BMT for EB. A BMT is not a cure and is a very long term, complicated process. Over the upcoming YEARS it should help Kiira to not get injured as easily and help her heal faster, but it is not designed to completely take away EB–only gene editing can do that. However, we are hopeful that as Miia’s cells grow in Kiira, they will go to injured areas of skin and heal them with the corrected gene, which would then give her Collagen VII in those areas and make her skin stronger. The outcome would be to slow the progression of EB while we wait for a cure. Damage to the skin is irreversible and scars are deep, so minimizing the damage now will help Kiira in the long-run. 
So in short, we need Miia’s cells to grow steadily and become stronger than Kiira’s. 
Thank you so much for all your prayers. We know today went so well as a result.

Day -1: Today Kiira was sedated for her total body irradiation. It will make her sick eventually and lead to mucositis, but we didn’t have too many side effects last time and hoping for the same this time. I did Kiira’s bandages in the recovery room, but we had to do her central line bandage change in her room. My sweet friend Gabriella came to help, but Kiira’s skin was so damaged from the last one. The adhesive remover and cleaner stung her wounds and some bandages were stuck to the torn skin. Nothing has even started healing in 2 days, so we tried to keep anything sticky from her the open wounds this time. Kiira screamed and writhed in pain. I do not know what I’ll do if we keep going through this–if EB gets to the point where this is a daily thing, central line or not. Admittedly, I felt like I was going to pass out. I’ve dealt with a lot of her wounds and pop blisters daily, but something about this tore at my heart as she looked into my eyes, tears streaming down her cheeks. God, I pray this BMT helps her! 
Kiira was very sleepy after and fell asleep to the strumming of a guitar. Tonight she had a slight temperature and red cheeks–almost like she was sunburned, but full of energy and joy. 
Miia also had her physical and blood test today. Tomorrow we arrive at 6am for her sedation and harvest of bone marrow, as they call it. She will get a needle in each hip bone from the back. When she wakes, she will be admitted to the BMT floor while her blood/stem cells are processed. Then she will get to watch Kiira get her stem cells tomorrow afternoon. I cannot believe this day is here! 
Love for Baby Catt and Kiira are on the same track. Catt is doing well so far too and getting her BMT tomorrow too. Please keep them both in your prayers!

Day -2: Today was Kiira’s last dose of chemo for several days. Tomorrow she has total body radiation. The dose, like last year, is far less than leukemia patients receive, but it’s goal is still to kill off Kiira’s bone marrow, which can cause numerous other issues in her body too. This is part of the challenge of deciding how much to put her through vs what EB will put her through. I don’t think there’s a right answer, but we continue to hold on to the hope that it will help more than it will hurt.

Day -3: Kiira had physical therapy this morning. She went down the slide over and over and we played ball. The therapist feels she is right on track for any kid her age, but it’s important to keep her muscles strong while spending so much time in bed. Later, she had fun playing with her sisters and had another bath and bandage change. My mom bandaged her first leg and arm ever. It’s about time we teach someone else, just in case. 
Her skin is looking decent–just a few blisters here and there and the eczema has cleared up. She has some scratches from the nurses doing night diaper changes, but they don’t bother her and we need our sleep at night, so we let them do it. And obviously the scab on her head from falling in the crib. 
Kiira had to get her central line bandage changed again because it’s either getting wet during the bath despite trying to be careful with it, or moist from sweat. Mistakenly, we switched to a more sticky bandage because the other type kept coming off. It’s important to keep it covered because if bacteria gets in, it can lead to a blood infection–the catheter is inches from her heart. I wasn’t there, but Jason told me he’s never seen Kiira in so much pain as her skin tore off while they removed the bandage, despite using adhesive remover. She fell asleep right after from screaming for 30 minutes. We didn’t have these issues last year, but bandaging is always trial and error and we need to figure out a better method.
After a long nap, she had fun playing with daddy and is down for the night. Tomorrow is her last dose of the chemo Fludarabine. We are getting so close!

Day -4: Today I took the older girls to visit our old neighbor. It was so nice for the kids to see each other again and run around outside. So many memories from last year came flooding back. When I got to the hospital, Kiira was in a great mood, although she threw up once this morning. She took a long nap after her bath and bandages and then she and I played all over the room, as I followed behind with her IV pole. Her favorite spots are the window sill to look at the elephant on the playground below and the closet. She even attempted to feed her baby while leaning over from the closet. We read books, sang songs, and played with her new toys. Since she only had one of her chemo drugs today, tonight she doesn’t have to get diapers changed every 2 hours, so hopefully she will sleep more soundly. 
I said a prayer with her tonight before bed and then asked her to say one. She said “Dear God, Amen!” So simple, but so sweet. I love knowing that God hears her calling him and I pray He heals this precious child.

Day -5: Things continue to go well. Kiira is loving the new toys and I’m so thankful for them, because I brought almost nothing here for her. The fun gifts really help while she is stuck in a small room for 24 hours a day, hooked up to IV lines. Today we continued with 2 chemo drugs and dealt with a few falls in the crib. I can’t remember when the sickness will hit, but we enjoy these days of energy, so many hugs and kisses from her, and laughing hysterically about the silliest things. This girl is so full of joy–I love it! 

Jason and the girls return home August 6th, so I just have one week left with them. It will be hard on both of us, but we both have a lot of support, which helps immensely. We are swapping nights with Kiira for now, so we see each other for a couple hours a day at the hospital while we give her a bath and do bandages. Then we swap kids/homes for the night. It’s only temporary though, and I love having some time with the other girls before they go home. 
We appreciate your continued prayers!

Day -6: Aside from Kiira’s frustration with being hooked up to her lines all day and required diaper changes every 2 hours, you wouldn’t even know Kiira was on chemo yet. She started her doses of 2 new chemo drugs today, but typically the effects take awhile to kick in, so I enjoyed watching her play with a ton of energy. Today music therapy came in to relax her for a nap after her bath and bandage change, but she was so intrigued by the guitar and wanted to play it too. 
Kiira is also enjoying her new doll and bear, so thank you for those! I haven’t been home today, but heard a ton of stuff came in her for her. Thank you so much for showering her and my other daughters with gifts! 
 Thank you also for your continued prayers.

Day -7: Today went great! Kiira woke in a good mood and she had no fevers today. In fact, she was still full of energy and asking for veggie straws and gloves, 2 hours past bed time. 
Last night I was able to chat with a couple of EB moms and today a dear friend we met last year during transplant (Help Anton’s mom, Vanessa), came to see Kiira. I loved catching up with her and Kiira had to show off by climbing dangerously on everything. 
Being a part of a community with kids who have such extreme medical needs isn’t something I dreamt about, but the moms of these kiddos are amazing and I’m so blessed to have them in my life. Today I met a caretaker of another child here for a BMT for a different condition. Everyone has a story and all involve heartbreak of some kind. I am so thankful for our days with Kiira and how it has opened my eyes to so much need in this world. 
We are so thankful for the prayers and support, the cards and messages we’ve received. Thank you for helping to meet our needs!

Day -8: All has been going great until a slight fever this evening. We know it’s not a big deal–just a side effect of ATG to feel flu-like, but Kiira’s high energy took a turn at dinner time. She is still so cute and sweet, but very subdued. I even got to rock her like a baby for awhile. 
Kiira’s sisters visited today, which was a huge help. Kiira loved the company and we loved the entertainment for her. Plus, she got to be off her IV for a few hours so she could roam around the room free of her lines and practice her yoga moves. 
We know feeling crummy is a part of this process, but we hope it’s short-lived and not severe. 
Some of you have asked about sending things to Kiira. You can message me for her address. One benefit to being in Minnesota is that we get bandages covered so we are taking advantage of that. She doesn’t NEED anything, but some have asked, and she will love it. Thank you!