Day +49: Yesterday I posted the picture of Kiira without bandages in the midst of me changing them and her skin is looking great! I hope someday that she can go without bandages for more than a few minutes at a time, but for now, I still bandage everything as before the BMT to keep her skin protected.
I don’t really have an update about the guy who is in the hospital, but I know he is still struggling and one of many in the hospital due to the toll of EB. Awhile ago I also posted about a little boy with EB in an orphanage who needed his forever home. I’m so happy that a friend in the EB community is adopting him. He isn’t doing well and she needs to get to him as soon as possible. If you have the means, please consider donating to help fund his adoption. http://reecesrainbow.org/109644/sponsortravis.
Thank you for the prayers and support for Kiira. Other kids aren’t so fortunate and I’d like to share the love with them tonight.
Day +48: We had Kiira’s checkup yesterday and everything is looking good, so we’re just staying the course on treatment of CMV and recovering from the BMT. Her skin still looks really good too.
However, tonight my heart is heavy. A sweet baby boy, Nate Tough, passed away last week as he was getting ready to start the BMT process. He had junctional herlitz EB. Another, one of the “older” people (31) with RDEB, is alone and fighting for his life in the hospital. I hate EB for so many reasons. Yes it is a very physically painful and debilitating condition that takes lives too early, but it also leaves a wake of destruction among families, other relationships, mental health, financial stability, and every aspect of life you can think of. I’m thankful Kiira is doing well for now, but until EB is cured, no one who has EB or knows someone with EB, will go unscathed. Please keep these families in your prayers and pray for a cure.
Day +44: Thank you all for your prayers, because they worked! Kiira’s CMV level dropped from 2200 to 600! Yay–no hospital at this point! Also her IgG level is good so Kiira doesn’t need an infusion on Monday. So I’ll take this weekend to be thankful for our freedom from the hospital and for Kiira’s improved health.
Thank you also to those who have sent cards or packages. It’s really nice to see the love and support you all have for Kiira and our family. We are beyond grateful for all of your thoughts and prayers!
Day +43: There isn’t much new to tell, but Kiira’s CMV numbers are still going up, despite staying on the IV anti-viral medication twice a day. If today’s level increases even more, which I’ll find out late tomorrow, Kiira will be readmitted to hospital this weekend for a minimum of 48 hours to get her electrolytes stabilized as she starts a new, more intense treatment. She will be hooked up to an IV nearly all day, between the 4 IV meds I need to give her, but I know it’s temporary, and I’d rather fight off CMV quickly than let it take Kiira down. Let’s just pray her CMV level starts going down!
Day +39: Things are going well for Kiira for the most part. We had to add another IV infusion each day to supply her with magnesium, but we made a little backpack for her to carry it around in and she is actually proud of it, so it’s not a big deal. Her WBC, hemoglobin, platelets and other electrolyte levels are good, but CMV decided to make a comeback and it’s at an all-time high. We took another level on Friday and I’m praying its down or we might be readmitted to the hospital this week.
Kiira and I keep busy, often taking long walks around Minneapolis. Usually she is covered in her stroller, but I pulled it up to snap a picture of her by the Mississippi River. She loves getting out and asks to go for a walk every day and she is content to sit there for hours, taking in her surroundings. She has certainly learned patience through her journey.
Tomorrow she will be 23 months old, and yet her positive attitude, thankful heart, observing mind, and patience surpasses mine by leaps and bounds.
We are so thankful for your prayers. We know they are keeping Kiira strong!
Day +34: Kiira is doing well: her white blood cell and hemoglobin counts are slowly going up on their own. Her blood pressure has stabilized so we took her off BP meds and the CMV (virus) counts are going down, which is what we want. We will continue to give her IV meds twice a day for another week and if it keeps going down, we can go to once a day for the next 4-6 weeks.
Her skin is looking great, except for her booty because of tummy issues and the back of her head, where it rubs on the side of the pack n play. I’m trying to resolve both issues, since they’re painful for her, but we can’t complain too much because I’ve never seen her hands and feet look so good.
I so thankful for your prayers and am hopeful these are long-term results.
Day +30: Kiira’s clinic appointment today went well. Her WBC counts are up slightly so it kept us out of the hospital, but we have to go in for labs and a blood pressure check on Sunday since her blood pressure and hemoglobin are low. Like our doctor said today, her engraftment did what it was supposed to and now we are just cleaning up the mess that chemo made on her body. Kiira is in good spirits, but still throwing up 1-2 times a day. She is getting super smart about all the things done to her and is so agreeable to just sit while her IV is in and to do meds. She says, “Yay heparin!” and “Yay Dr (insert name)!” and knows when it’s time to flush an IV, which is funny, but she isn’t even 2 and already knows these things. The picture is of our morning set-up, which takes me about 90 minutes to complete on Kiira. This is nothing compared to 4-6 hour bandage changes that would be in our future if it wasn’t for a successful BMT or eventual cure.
So the big question–how is the BMT helping her skin? I think it’s too early to tell, though her skin is so soft, not dry anymore, heals quickly and we have only had a couple blisters on her feet that she specifically injured and on the bald spot on the back of her head. Skin improvements are typically seen over the course of years so though I want to be optimistic and hope the lack of blisters are permanent changes, from all I’ve heard, it’s too early to tell. But whatever it is, I’ll take the current improvements.
I’m so thankful for the prayers and encouraging words. I’m praying that God works a miracle here.
Day +29: We got Kiira’s engraftment numbers today: 100% cd3 and 99% cd33, which means she almost completely has Miia’s immune system! Yay!! Numbers may still fluctuate but this is great for now.
We are still treating CMV and I’m currently doing the IV meds at home. Unfortunately it’s like chemo, so it will keep Kiira’s immune system lower for the 6-8 week treatment (have to be even more careful with exposure) and I have to be very careful with handling, like wear gloves to change diapers and throw meds in a special container.
CMV can also lower the immune system and the virus and/or treatment can cause graft failure. Since Kiira’s WBC count dropped more today, the doctors are really weighing options about whether or not to send Kiira inpatient for a different treatment that’s easier on the immune system, harder on the kidneys, but a stronger drug to combat CMV.
CMV is treatable but I am nervous about it affecting engraftment. The doctor and I discussed the balance of waiting too long to treat it more aggressively to save engraftment versus just waiting and seeing what her body does on her own with the current IV meds. They are looking closely at her counts and checking CMV levels every couple of days and hopefully we’ll make the best educated decision.
Today we had an IVIG infusion, putting us in the clinic for over 4 hours. Tomorrow we have another appointment and possibly a dose of GCSF to stimulate Kiira’s immune system.
I’m confident in the team here and they celebrated Kiira’s engraftment numbers as much as I did, so she is in good hands. Thank you for your prayers!
Day +28: Today I got a call that Kiira’s CMV levels were high enough that she needs treatment. CMV is another common virus that nearly all of us carry, and Kiira and both of her donors tested positive for it before the BMT, so they check her levels weekly to make sure it’s dormant. For a healthy human, it might present like a cold, but for immunosuppressed individuals, it can infect various organs and more commonly, cause pneumonia which often leads to death. They check levels frequently to catch it early and treat it aggressively. I was worried about EBV, the virus she had last year, and it could still come up, but CMV decided to take hold of her body for now.
So, treatment required an IV infusion in the clinic today of a high dose IV antiviral. Since it went well, I’ll be administering the IV doses myself 2 times a day for 2 weeks and then do a maintenance dose for another 6 weeks. At first this sounded annoying, but the alternative, which is still a possibility since her WBC counts are dropping, is going inpatient again for a more aggressive drug that throws Kiira’s electrolytes out of balance. Until those things are under control and CMV levels are decreasing, she would have remain hospitalized. Her WBC counts were marginal today so we are rechecking them tomorrow. I really hope her counts stay high enough to keep us out of the hospital.
Kiira is still in good spirits and doesn’t present signs of being sick, besides the runny/stuffy nose she had. We have an IVIG infusion tomorrow, which is common for us now and might help with CMV too. Engraftment results should be back by Friday, so we are anxiously waiting on those results too.
We did say goodbye to my mom yesterday. Kiira keeps asking for her Mimi, but now it’s just Kiira and me. BMTs are never promised an easy path, but neither is EB. So we keep fighting and taking each day as it comes.
Day +26: Engraftment test day. I know I should have positive thoughts and feel confident Kiira’s numbers will come back with great engraftment results, but I’m nervous. Very nervous. I had a lot of scenarios in my head last year, but losing the engraftment wasn’t one of them.
Bringing Kiira to Mn for her 2nd bone marrow transplant was not an easy decision. Beside the concerns for putting Kiira through chemo and radiation again, it would mean more sacrifices on our part than even the first time required. My husband and girls have been gone for over 3 weeks now and my mom, who sacrificed nearly 2 months of HER time leaves tomorrow. I’ve already missed special days in their lives and yesterday I missed my friend’s wedding, which is a huge deal to me.
I’m not afraid of being alone here–I’ve made some amazing friends here who will be a huge support, as they already have been. I know Kiira and I can keep busy, even in her isolation; between feedings and meds every 2 hours, bandage changes, doctor appointments, work, and lots of fun play time, we keep ourselves busy.
What I am sad about, is all the things I miss at home. I know it was our decision to come here and I don’t regret that. Kiira is doing great and I’m beyond thankful–it makes the process easier. Overall I’m even doing well. But there are days when I wish EB wasn’t an all-consuming factor. Some think I write so positively about this life we were thrown into, but reality is that EB touches every decision we make, from how we hold Kiira, to what we wear, to where we take her, to her medical care, and to what we sacrifice for that care. Sure, I let Kiira play and explore with a lot of freedom and she is flourishing; however, EB, like probably many life-altering/limiting conditions, is cruel, not only to the person suffering from it, but to the families too.
For today, I can only pray that her sister’s donor cells have settled into her body and start healing her from the inside out so that our family can start healing too.