Category Archives: Uncategorized

  Well, I totally misunderstood Dr. Tolar’s email from Saturday, but I guess it let me have a couple good days while we prepared for our return to Minnesota. Kiira still does not need chemo before her boost on Wednesday as he wants to preserve her organs, but here are the numbers as they relate to types of T cells: 

CD3: 48% donor (down from 74%); 

CD15: 0% donor (down from 5%). 

While she is on the way to losing her transplant, the hope is that the boost will add cd15 cells and they’ll increase because the cd3 is present and cd3 helps the cd15 in our immune systems. He said it’s unusual for the transplant to act like this–usually CD15 is higher than CD3, but by 6 months the numbers even out, so usually they’re either both around 100 or 0. At this point, the lack of cd15 is dragging down the cd3 and this scenario may be because she developed an antigen to the donor. This boost will use up the last of the donor blood, but if she has developed an antigen and therefore, antibodies, we could not use the same donor if she needs a second transplant. Keep in mind, she only had one perfectly matched donor. This was/is our best chance in my mind, but God has His own ways sometimes. 

They will give Kiira the boost on Wednesday, test her in 30 and 90 days and decide where to go from there. She will remain on CSA, the immunosuppressant, so that means she will continue to be in her bubble (in a mask and not in crowded places) for at least 3 more months. Thanks to El Niño, it’s probably better off we stay inside anyway. 

I can’t even fathom going through another transplant again, uprooting my family, hoping the second go is as easy on Kiira as before, but successful. I keep reminding myself, one day at a time, so today I got answers. Not what I wanted to hear, but I got them and now it’s just more waiting, and trusting. 

Tomorrow she goes in the OR at 11:30 am CST, so please pray that she does well under anesthesia, she has no strictures in her esophagus, her biopsy heals quickly, and the central line goes in without a hitch and Kiira doesn’t pull at it. And for my girls at home, who said they are trying to be brave and not cry because they miss me.

  We made it to Minnesota! The flight was on time and smooth, but Kiira was not a fan of sitting still for the 2 flights totaling 5 hours plus a layover and she made it well-known. One woman even told me after the flight that she was a pediatric nurse and thought about giving me tips for flying with children. Trust me, I tried just about everything–all my tricks with my other 2 don’t work for Kiira! If someone has tips for a 15-month old who wants to be on the move, doesn’t know how to talk well so she screeches to tell us, doesn’t eat much or take a bottle and only likes a paci for nighttime, and Benadryl does not knock her out, plus needs to be contained from germs and difficult to handle with her skin, please do give me advice! Anyway, this picture is from the beginning of the flight when we were both happy 🙂

Our dear friend Stacy picked us up in the freezing cold and had snacks, waters, our favorite Finnish breads, a toy for Kiira and other necessities that were such a blessing! Thank you!! 

We are off to bed to start out a busy week of doctor appointments. I’ll admit, I am so, so sad I won’t be seeing Vanessa and Help Anton this week. I loved stopping in to chat with them each week, and it feels like something is missing here. But I hope to see a couple other kiddos who are here for their BMTs. 

Please pray for good reports tomorrow when we meet with Dr. Tolar.

Today is a day of good news! After an amazing worship night last night with friends, many of them prayed over us and for Kiira with the hope that Kiira’s donor numbers would increase enough that she wouldn’t need chemo. Today I got an email from Dr. Tolar to tell us that the majority of her T cells are still donor (CD15 are all recipient), and the plan now is to give her a boost with no conditioning (no chemo or immunosuppression). We are so happy and I can’t wait to find out exact numbers on Monday, but going into this trip, this news made me feel so much better and all I can do is praise God for it as we witness the power of answered prayer once again. 

Also, after about 4 days of favoring one foot and acting grumpy and in pain, yesterday Kiira was back to her cheerful self and walking on her own with a push toy. I know most kids go through this and it is probably exciting mostly to only the parents, but I am so happy to see Kiira reach this milestone, because I honestly wondered if she would ever walk. She never ceases to amaze me! 

We fly out tomorrow and hope that Kiira does well on the flight and somehow we avoid any sicknesses that could be lurking on the flight. I am so glad my mom is joining me and can help me with ton of luggage we need to carry bandages, feeding tube and pump supplies, toys, and clothes for 3 degree weather we will arrive to tomorrow. 

We will keep you updated about our doctor visits in Minnesota and appreciate your continued prayers!

Day +188: As a parent, it’s awful to feel helpless. Right now all we can do is wait for a cure, but in the meantime Kiira has another huge blister covering the top of her foot by her ankle, wrapping around to the bottom. The bottoms of her feet have never been affected before and I somehow hoped they never would be so that she could walk with more ease, but now that it’s been injured, it’s that much more susceptible to injury again. This one is painful to her, so anytime she tries to stand, which is about every minute, she winces in pain and either stands on only one foot or falls down. All we can do is try to relieve the pain by popping the blister every time it fills, applying cream, and rewrapping her with ever-increasing bandages. Tonight, during a second bandage change today, the foot even looked swollen. Plus she now points to her fingers and does a half cry whenever they hurt, multiple times a day and deals with mouth blisters frequently. (Don’t worry–she is still overall happy). I’ve come to grips that Kiira has EB and will always have it, but seeing this new level of pain, where it’s starting to inhibit her, breaks my heart and realistically, it’s just the beginning…

There’s been a lot of discussion in the EB community about pain management. Most kids are on morphine or much stronger drugs and the push for marijuana use for EB (a more natural remedy) is prevalent. Kiira has never been on pain meds, besides a couple days after her gtube surgery and BMT. I’ve heard the drugs make the skin worse, cause personality and emotional changes including hallucinations and I have to imagine affects learning as well. I haven’t felt like she has needed them because her her pain is acute and temporary and things like distraction work so well during bandages changes. I wouldn’t give my other girls morphine for a cut on their knee, but when it becomes chronic pain, the need for better pain management will arise and we’ll cross that bridge when it comes.

We heard from Dr. Tolar about her biopsy report and it shows she *might* have very minimal collagen VII present from the October biopsy. His feeling is that you don’t need much, so it might be enough to make her skin stronger. Maybe it’s helping parts of her but not her fingers or feet, which are at their worst. It has only been 6 months and hopefully the donor cells are increasing and we will see better results in the next 6 months, but watching a lot of other stories confirms that a BMT is not even close to a cure–it just buys us time. I haven’t lost all hope–I know doctors are working so hard and gene editing trials on animals is getting further and more successful, but it’s still a long way off. Kiira was given to us for a reason, and it’s our job to not just care for her, but also show her the unconditional love of God until her cure comes.

On a note of thanksgiving, thank you to our bible study group for planning to bring Jason and the kids meals while Kiira and I are in Minnesota. In the midst of your own business and hardships, you are blessing our family and we are so thankful! Also to the girls’ school for promising to love on them while I am gone–I know it’s only a week, but it’s comforting to know they’re in good hands while I focus on Kiira.
Thank you for your continued prayers for our sweet girl!

Day +184 / 6 Months: Happy New Year! 2015 was by far the craziest year for us. I don’t have a specific set of resolutions, but I’m sure Kiira will still take up much of time and thoughts and I’ll try to integrate improvements to my own overall well-being in between. We are hopeful and holding on to our faith that Kiira will continue to thrive and we will do our best to love on her and help her flourish.  

It’s been 6 months since Kiira’s Bone Marrow Transplant, a day that is kind of like a new birthday for her, a new chance at a life with less severe EB. It feels like a lifetime ago that we packed up our house and moved to Minnesota for the BMT and have since returned home. We had a great Christmas with family and fun New Year’s Eve with friends like old times, so we are slowly putting our lives back together. We face many unknowns about the success of her BMT, but we hope for many answers when we return to Minnesota in about a week. 

Kiira is getting 4 teeth at once and has been biting her fingers and been grumpier than usual as a result, but it will be fun to see her with more teeth. Today we gave her a full bath, bandage free, which are few and far between, but every time we do, I’m reminded how much she loves them at this age, so I should do it more often. Her feet are healing slowly but they look much better than last week and the extra padding is helping so far. 

We also got her biopsy images back today from early October, but I have no idea what they mean. The hope is that she is creating Collagen VII and they are producing anchoring fibrils, which is like the glue between our layers of skin, and necessary for her to stop blistering and peeling back skin with the slight friction. I imagine the images will be explained to me in Minnesota. 

I’m so unbelievably thankful for your prayers and support. I don’t know how I would have made it through the last year without them and I know we will continue to need them for a long time to come. So thank you and wishing you a very blessed 2016!

   Day +182: Not everything with EB is fun, but somehow Kiira brings joy to most situations. Her newest thing is to help us with her gtube feedings as she pretends to put the syringe of water in to flush it and she thinks it’s just hilarious. I love her laugh! 
After torturing her 45 minutes to get her blood, we had her labs drawn on Monday and shipped to Minnesota for testing. Please pray that the levels show increased donor cells so that she doesn’t need more chemo or even a boost and that it can start healing her skin inside and out. 
Lately I’ve been questioning why we were led down the path of a BMT for it to just fail (possibly), but I was reminded that if we didn’t try it, I would always wonder if I should have. I have to remember at this point in time, a BMT is the best option for RDEB with no collagen production and I had to give Kiira the best option. Whatever may come from it is in God’s hands and I’m beyond thankful for the hope He provides, not just in this life, but for all eternity.

Day +178: I have a long overdue update, but I needed time to process Anton’s death and what it means for our future. I am still so sad that the world lost an incredible little boy, but realized I was going into a dark hole, where I was a year ago, and for my family, especially Kiira, I couldn’t do that again. From day one we were told to take one day at a time and I had to remind myself of that. I’m sure it won’t be the last time and there are days or moments when I struggle, but I try to be strong for Kiira, because she is strong. 

So her update… Her hands and feet were in terrible shape over the past couple of weeks–maybe the worst ever. We (meaning Jason) had to cut the skin between the toes because the skin was fusing them together. We’ve had to change our wrapping technique again to include yet another layer of padding on her feet. However, we are still struggling with what to do with her hands to allow her fine motor skills and tactile perception, but protect them from the extensive scarring she has incurred in one short year of her life. I’m thankful the rest of her body has been in good shape though. 

Kiira is eating more these days, but still taking gtube feedings during the day and at night until about 2am. We go to feeding therapy every week and the tips have been so valuable, so though it’s slow, we are seeing progress. Kiira loves soup, refried beans, avocado and kiwi. 

This week we received our schedule for Minnesota. My mom, Kiira and I are going on January 10th for a week. She will get her blood test to check her chimerism (amount of donor cells) on December 28th. They’ll ship the blood to Minnesota for testing and I hope the results are available by January 11th so that they can make the final plan for her boost. Her current schedule has no plan for pretreatment of chemo or immunosuppressants, but I hope her blood test will help them make the best decision for that. They still plan to do the endoscopy, biopsy, and put in a temporary central line. Jason will stay home with the other 2 girls, juggling work and their schedules. 

Kiira is so active and a little show off. We had some visitors who follow Kiira and she knows just how to get people to smile and laugh. She will stand for a few seconds without holding on to anything, but I think we are a long way off from her walking. She repeats sounds and words you say and she is in the phase of asking “What’s that?” about everything. 

We had an amazing Christmas. It was wonderful to celebrate with our family, most of whom we haven’t seen since returning from Minnesota. We hosted Christmas Eve, but decided not to have Kiira attend our big family gathering on Christmas Day so Jason stayed with her. Not only did he miss out, but he did her bandages by himself–I told him that’s the most wonderful Christmas gift he could have given me. Honestly, I dread bandages every day, but I know it’s necessary for Kiira’s well-being. 
Some have asked how you can pray for us: 

1. For Kiira’s donor cells to grow and create Collagen VII to make her skin stronger
2. For Kiira to develop normally–to eat by mouth and not rely so much on the gtube, to learn to walk and do all the other things kids can do
3. For Kiira’s big sisters to love her and be gentle and protect her always
4. For us, as parents, to be patient, remain faithful and hopeful, and to be a light in all circumstances. 

Thank you for your love and support. We hope your Christmas was filled with joy and hope for this coming year.

Day +177: A very merry Christmas from our family to yours.

Day +171: We are overwhelmed, in a good way, by the love and support you want to show Kiira. I want to say a big thank you to The Butterfly Fund and Fifth Stocking Project they put on. People generously donated to our kids and Laurie delivered the gifts to us in person yesterday. It is so wonderful to meet the people behind these organizations who dedicate their lives to blessing others.

I know some of you asked how you can help with a medical table. You are amazing to jump in and offer to help–I was just trying to tell you about some of the things we need to keep Kiira safe and healthy and you jumped in with offers to help. Thank you! We are researching options and will let you know what we decide.

Thank you also for helping to keep me afloat on the hard days with your prayers and comments. It really does help! Today was better for me, and despite a couple falls and face injuries, Kiira had a good day too.

  
Day +169: The death of Anton has sent a flurry of thoughts through my mind and I am not even sure where to start. I don’t even feel like updating about Kiira but I know some of you have asked. She is fine–she is busy as usual and despite a blister that spans half of her foot and another across the back of her hand, she keeps moving and remains happy. We are just trying to keep the fingers and toes from fusing from these injuries. She yearns to be outside and cries at the front door to let her out. Her big outing, besides the days we had in the snow last week, is to get the mail with daddy when he gets home from work. Something so simple brings her so much joy. 
I wish and I try to share her joy, but all 3 kids with EB who were in the hospital while Kiira was there, are now in the arms of Jesus. I’m losing hope. It’s a race against time because RDEB is a progressive condition. And the cure isn’t here and comes with so much red tape (genetic alteration isn’t an easy sell) and although it’s in the works, could take years to develop and approve. 
I’ve been trying to push for another chimerism test before we go to Minnesota on Jan 10th because it can tell us if she dropped to 0 donor cells or hopefully it’s increasing on its own, as we’ve prayed for. Then maybe she wouldn’t need chemo and a boost in either case. She has done so well post-transplant and I don’t want to destroy more of her body unless we absolutely have to. Plus, I think it will help them make a more informed decision about what to do. 
I also inquired about getting a medical exam table for Kiira. Currently we do her bandages on the floor in a beanbag chair so she can’t roll away, but even that can’t stop her anymore. We are far from sterile and between the germs, dog hair and the ant that fell in Kiira’s eye during tonight’s dressing change, it’s time we figure out our next plan, especially if she continues to get big wounds that can become infected. Unfortunately insurance doesn’t provide anything like that, but it’s on my to-do list, along with calling about setting up physical and occupational therapy and organizing Kiira’s bandages so I know what we have and can find them when needed. 
There’s so much more I can say but please keep the Delgado family in your prayers as they mourn the death of their son, prepare for his memorial and their return to their home. 
Thank you for your prayers and the love you show us, and especially Kiira.