Day +107: Yesterday we drove through Kansas to Colorado and hit a snow storm. Accidents were happening all over and sometimes it was hard to see, but thankfully we made it safely to our hotel and had a restful night.
Today we wiped the snow down from the car and headed through Colorado to Wyoming and finally Utah. It was sunny, but temperatures were in the 20s and we are not very prepared for such cold conditions.
We are taking a driving break tomorrow, but Kiira is so good in the car that it makes these drives much easier. We love exploring the beautiful country and glad that this road leads us home.
Day +105: Today we left Minneapolis behind. It was hard to say goodbye, although I know I have so many great things back home, including my other two daughters. Also, a road trip is never complete without some bumps along the way.
Getting out the door took longer than expected and then in the 10 minute drive to the hospital for a quick lab draw, I got a call from our credit card company that there was fraudulent activity so we don’t access to that card this week. As I was finishing the call, Kiira’s gtube got stuck in her seatbelt and completely pulled it out of her stomach. It’s the first time in nearly 2 years that it’s ever happened and we all kind of freaked out, especially since it hurt Kiira. In the next 10 minutes that it took to be seen by the nurses, the hole had closed up too much to put it back in. They discussed sending us to the OR, which meant we would not be able to leave today. Fortunately, two specialists used dilators to make the hole big enough to fit the gtube back in and an hour later, we were finally on the road. It was a bit traumatic so Kiira fell asleep almost immediately. We made it to Kansas, hours later than planned. However, our dear friends had a warm meal ready for us, and a fun place with 3 girls for Kiira to run around with after being in the car for 7 hours.
Tomorrow we have a 9 hour drive to Denver, hoping to avoid the impending storm coming in.
Thank you for all your prayers over the past several months, or even years. We couldn’t have gotten through this without them!
Day +103: Kiira’s 100 day engraftment came in. She is 100% CD3 and 92% CD33/66 (types of T cells). She is also CMV (virus) negative again. All of this is great news!
Last night we picked up Jason from the airport and Kiira was thrilled to see her daddy. She wanted him to read to her and do everything for her. I’m glad she fell back into routine with him so easily. Aside from a couple day visit in October, it’s been over 3 months since he and the girls went home and it’s wonderful to be together again.
Today we had our final appointment in the clinic, a full 9 hours, and we had a mini reunion with EB families in the area. People from around the world come to Mn for treatment and we all have an incredible bond because of EB. I wish Kiira could’ve been down playing with the other girls, but she was getting MSC cells from Miia (2 of 3 sets), inhaled antibiotic, and a flu shot. We said our goodbyes to the doctors who have been an enormous help to us over the last 2 years. We will see them again in January, but this has been our home and Kiira gets so excited to see them. When we told her we weren’t going to the hospital anymore she said, “awwww” in a tone of disappointment. The day wouldn’t have been complete without more music therapy too.
I wasn’t sure about going through a BMT a second time, and how it helps her skin is still to be determined, but now it’s coming to an end and I’m confident we made the right decision. I’m thankful for the experience and God’s provision for our family as we navigated through this challenge. We head west on Wednesday to rejoin our family and friends early next week!
Day +96: Today is engraftment study day! The clinic drew 21 vials of blood for a variety of tests, including the 100 day engraftment test to see how many donor cells Kiira has. We are hoping it’s still 100% donor! We also found out that Kiira has 7% of Miia’s cells in her skin. This amount isn’t significant, but it’s good to see that some is present.
We spent most of today’s appointment discussing our plans to go home next week. After we get results from today’s test (late this week), we’ll plan on weaning off the immunosuppressant and the IV magnesium. We can also drop off the anti-fungal and anti-viral drugs in a couple of weeks. However, they decided to give Kiira the inhaled antibiotic that requires us to go in once a month for treatment and we start that on Wednesday, along with our flu shots. It’s possible we can switch back to an oral version in the future, but the team is being extra-cautious with Kiira’s immune system, which I appreciate.
Kiira continues to do well. Her favorite new saying is, “That’s silly mama!” and she runs around, purposely standing precariously on things like books. It makes me anxious, but she gives a sly smile, like a typical 2-year old who knows they’re not supposed to do what they’re doing. Let’s just hope the BMT is working on her skin fast enough to keep up with this dare devil.
Day +91: All went well today in the OR. Kiira had a biopsy on her leg and they ended up doing an echocardiogram in recovery, which was planned for Monday so it was nice to get it out of the way. After a short nap today, she ran around nonstop for hours, as though nothing happened today. She wore herself out and was in good spirits.
Thank you for your prayers!
Day +89: Last week Kiira had a swallow study. It did not show any strictures in the throat or upper esophagus, which is great, but she is still choking on soft solids such as avocado. Our next step is to resume feeding therapy when we get home and hopefully she will pick up the proper eating technique quickly.
Today’s clinic appointment was quick and uneventful. Kiira’s white blood cell count’s are back to normal and CMV (virus) is still detectable but below the treatable amount. We are staying off IV meds and can plan to go home in two weeks.
On Wednesday Kiira will go into the OR for a skin biopsy to determine if she is producing any collagen VII yet. It is a routine procedure but prayers are still appreciated.
My aunt left yesterday after a 10 day visit. It was so nice to have an extra set of hands to entertain Kiira. I even got a night out with a friend which was so nice. Thank you for your help! Jason will arrive in two weeks to drive home with us. It is hard to believe we are wrapping up everything here and we may have had to do the BMT twice, but it could not have gone any better both times and I’m so thankful for that!
Bandages: Kiira is still using bandages donated 2 years ago by many of you (and still have plenty of most things). We cannot express our appreciation adequately in words. Our insurance still doesn’t cover them and they can cost thousands of dollars a month. When Kiira was born 2 years ago, I was so stressed about providing her with what she needs to reduce wounds and infections, but God used a number of people who came to Kiira’s aid and subdued my worries for over 2 years! We learned how to reduce waste and reuse bandages when we can, but I felt like we witnessed a miracle of never-ending supplies. We have even sent some overseas to help others in need of bandages, because unfortunately not everyone has those resources. Without a cure, bandaging the skin is the only protection we can provide to those with EB. Several organizations and fundraisers rise up to help support those families, like debra.org and IrefuseEB.org, but many families still have to do fundraising on their own to cover supplies, copays, lost wages due to so much time off to care for a child with EB, special clothing, and so much more.
Thank you for blessing us. It helped Kiira be as healthy as possible and relieved so much stress for our family. Thank you!
Sometimes waiting for a cure takes too long. It requires too much money, time, trial and error (and patients to be a part of those trials but when there’s only one in a million, that doesn’t leave many to try it), and red tape. Plus, the only way to cure EB is genetic modification, which is very controversial and extremely difficult to do. So, in the meantime, there are organizations like debra.org who help patients with bandages, tips, and other resources to make life with EB easier. We are thankful to them and the EB community for keeping us up-to-date on bandaging techniques, creams, medications, clinical trials and other things we can do to help Kiira. However, it couldn’t be possible without funding, of course. Consider donating to debra.org to help those with EB.
We may have done a bone marrow transplant to keep Kiira from blistering as much and help her heal faster, but EB is still very much a reality in our lives. Just a few months ago (pre-BMT), this is what her feet and knees endured. Though we see less of this after the BMT, yesterday she stepped on something, blistered the bottom of her foot and could not walk. I carried her wherever she wanted to go and it broke my heart watching her attempt to push her stroller when the pain was too much. EB is harsh, but Kiira pushes through with unimaginable strength and despite the pain in her foot, she made herself walk around today.
EB affects about 1 in 20,000 people and Kiira’s subtype, RDEB, affects 1-2 in a million. Though research has made enormous strides despite being so rare, we have a long way to go to find a cure. We support the work of Dr. Tolar and the team at the University of Minnesota who actively seek a cure for EB, and if you want to help find a cure for Kiira, 100% of proceeds to irefuseEB.org go toward that research.
Day +82: Kiira’s clinic appointment went well today. Her WBC count has gone up some and while we wait for her CMV (virus) results, we’re sticking to our plan to stay off the IV antiviral and restarting an oral one to keep her immune system up. I’m already making preparations to head home in about 3 weeks. We have several more things to get through, including a swallow study on Wednesday. Kiira has been choking on the tiniest bits of food. She isn’t drooling and I’m not seeing blood in her mouth, but I’m wondering if there’s some narrowing in her throat or upper esophagus that can’t be seen by an endoscope. We haven’t done one since she was 4 months old and I’m hoping her throat is clear, but we want to be sure before we head home. Unfortunately this is extremely common in EB due to blistering and narrowing of the digestive tract and I’m beyond thankful it hasn’t been an issue for us yet. Stopping the internal damage was an enormous reason for us to do a BMT so early for Kiira. Once the damage is done, its irreversible.
My aunt is here visiting so we enjoyed an outing to Minnehaha Falls. Kiira wanted to see the waterfall so much that she actually wore her mask for about 20 minutes. I was shocked! Thank goodness for the stroller rain cover, which gives us the freedom to go for walks without the fight or discomfort of a mask.
We appreciate your continued prayers as we know they’re working. Thank you!