Category Archives: Uncategorized

Day +163: I finally got the dates to travel to Minnesota in January (brrrr!). Instead of 2 days, we’ll be there for 7 so Kiira can get a temporary central line put in to receive the prep and boost of donor cells. She will still get her biopsy and endoscopy as well. I hope they only have to put her under one time, but I don’t know the exact plan and they still haven’t decided in the pretreatment of either chemo or immunosuppression drugs.

Although the unknown of this trip can be frightening, I try not to think about it. We’ve been enjoying a few days in the snow and got dumped on last night so we enjoyed fresh powder for skiing and sled runs today. Even Kiira went in a sled and loved it. When we stopped, she said “Go! Go!” It’s been so fun spending time with family and letting all the cousins play together.

Please continue to pray for Anton and his family. There is nothing more they can do for him, but today he had a decent day, making more memories with his family. I kept waking last night thinking of him and his sweet smile, his singing, him waving hi to me from his room as I visited with Vanessa… It isn’t my child, but my heart hurts for him and his family and it’s hard not to think that Kiira could be at that point sooner than she should be. We will try to make the most of every day we have with her and whether God grants us a full life-time or a shortened one, we will praise Him for the beautiful gift she is.

Day +160: Kiira may not take a bottle herself, but she loves to feed her baby doll. She stays busy reading books, playing instruments, and standing anywhere she can, even when it means another bonk in the head. She may not say a lot of words but she knows how to say Elsa and Ariel–the important princesses in my kids’ lives.

Kiira had labs Monday and they weren’t as treacherous as last time. Her labs came back great, but the normal ANC and white blood cell counts make me wonder if she has any donor cells left. We should find out in about a month. Unfortunately her hands, fingers and tops of her feet have been blistering a lot. I wish there was more I could do to prevent it. Now she not only points out the owies, but she pushes on the blisters to figure out what they are. It’s funny she is just now exploring these things that have been a part of her for nearly 14 months. With her being more aware, we keep telling her that we are just trying to help her as we poke her with the needle so that hopefully she gets used to that too and doesn’t fear the necessary tool.

Overall we are good and taking a little trip to the snow with my brother’s family and my parents. We haven’t all been together in probably about a year, so it will be a wonderful outing and give us a chance to spend time together.

We are so thankful for a generous gift we received from a local mom’s group and their families. Also for Flip2It Sports for offering a safe place for Kiira to play and explore.

Please pray for Kiira and Anton and Raul and so many others who need God’s healing. Thank you all so much for your love and support!

Day +157: We had quite a few blisters to deal with today and now Kiira points them out on her fingers and knows the word “owie”. However, she gets over it quickly. I’ve had a cut on my finger for days and it bothers me constantly. It amazes me how she can just move on.

The past couple of days have been so fun. I took Kiira to the store in her covered stroller for the first time. She loved it and we even ran into one of her Facebook followers. The conversation that ensued was so uplifting and sweet. I love that she is so loved. I also had the opportunity to get a girls’ night out and then took the big sisters to a birthday party on a farm today. It was something I really needed since I’ve had so little time with friends since being back from Minnesota. I love Kiira, but sometimes I just need to experience life outside of her bubble-protected-world for awhile.

Overall she is doing great and we keep praying for the donor cells to grow, infections to stay away, and skin to be healed!

Day +153: After all of Kiira’s experience at the doctors’, she is trying her hand at playing doctor too. This morning I was thinking about how great she is doing. Her cold was so mild and seems to be gone already. Her skin is good, but I’m wondering if I am becoming more immune to her owies, because many people look at her with pity, but I know it could be so much worse! She went a few days with no blisters which is such a welcome change, but today I did pop 5. We had her feeding therapy yesterday and she ate more than I’ve ever seen, which is about 1 oz of yogurt and 1-2 Tb of avocado. It’s not much, but it’s progress. We don’t have therapy next week, but we will be going every week after that. We are still waiting on our plan for Minnesota in January. I have no idea if I’ll be there for 3 days or 3 weeks, but this time our family will be separated. Today it’s been 5 months since Kiira’s bone marrow transplant. 5 months that she has been in isolation, which means one of us has been too for the most part. I was hoping in a month she could taper off CSA, her immunosuppressant, so that she can finally experience life outside of the house and a doctors office, but with her counts, I don’t know. We are pretty much in a constant state of the unknown, but we are just taking things as they come.

If you missed it, check out the video I posted about Michael, a child with RDEB. If you’re looking for a place to donate to before the end of the year, the efforts of the EB Partnership Foundation are getting us closer to a cure for EB and they need funding for some critical research. The news just announced the first EB child will get localized gene editing, which is scientifically complicated, but so amazing! We are so close, but these things take years to develop and approve, so keep praying for a cure!

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. 

  Day +141:

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.