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Monthly Archives: June 2015
It’s hard to believe the day Kiira is admitted has arrived. I feel like we’ve waited for this day for so long, and here we are. While it’s easy to get overwhelmed through this process, I am finding a peace that I can’t explain. We take one day at a time and deal with each thing as it comes–otherwise it would be far too overwhelming. This is the daily account of our experience during the days of chemo:
Day -7: Today was very low-key. They did vitals, we met nurses and doctors, got settled in and they even let us escape with Kiira for one last dinner together before hooking her up to an IV. The IV is necessary to get her a lot of fluid before starting chemo at 10am tomorrow since chemo can damage the bladder. Starting tomorrow, they’ll be watching the fluid intake and output every 2 hours, meaning diaper changes every 2 hours even through the night. She might also need a blood transfusion tomorrow, because her they’ve drawn so much blood over the last week and her hemoglobin is low. It will be necessary during the process anyway, so we expect multiple transfusions.
Kiira already flashes her infectious smile to all the nurses. She had fun playing on her bed, especially peek-a-boo, since we surrounded her bed with pillows for protection, and is now fast asleep. She really rolls with the punches and has no idea what’s happening, which is good in so many ways.
It was nice to ease into today, but I’m definitely ready to get the process started so we can start seeing results.
Day -6: Today Kiira started chemo. She got two different chemo meds, plus anti-nausea medication and a ton of fluid. With the constant checks of vitals and diaper changes every two hours, she hardly slept and was so tired and grumpy tonight. Jason stayed with her tonight and she fell asleep sitting up while waiting for the last vital check at 7:30pm. Other than that, she seems to be doing well.
Today was also the Turtle Derby Races, an annual fundraiser for the hospital. The girls visited Kiira and I got to take them down to do crafts, watch the turtles race, and win lots of prizes. It was a really good break from the hospital walls–I know, I know–I’ve only been there one day, but it was still a nice break smile emoticon
Just to clarify something, since I was asked–In order to have a bone marrow transplant, you must undergo chemo (5 days for Kiira) and radiation (1 day of total body irradiation) to suppress your own bone marrow. It is low doses for EB, compared to someone with leukemia, but still enough to make you sick, lose hair, etc. Once that is done, they give her the new bone marrow and hopefully it will make its home in Kiira. In the meantime, she will have no immune system, so we have to keep her isolated for about a month, but in a bubble for about a year. Kiira has RDEB, not cancer or anything like that, but still an extremely painful, debilitating condition. This treatment is experimental, but they’re seeing formation of collagen VII in past patients, resulting in less wounding and faster healing. We hope it will give Kiira a great quality of life compared to what she would endure without any treatment.
I felt God’s peace today, so thank you for your prayers.
On June 25th, Noah Coughlin, someone who runs across the country to raise awareness about rare diseases, ran for Kiira and EB. I always love getting EB awareness out there, and love people who dedicate their lives to helping others. Thank you Noah! Noah on the run
Day -5: Today was day 2 of chemo and a good day. We gave Kiira a full bath, which is required daily during chemo. I feel like a first time mom giving a newborn a bath–it’s so awkward trying to hold her and wash her and keep her skin from peeling off. But she loves bath time. It took us 2 hours to do bath and bandages, so we’ll be doing that for the next 5 days, but I’m really hoping most bandaging will be over in about 35 days!
Kiira was exhausted from yesterday and getting woken up every 2 hours for diaper changes, which created several accidents and skin injury. Thankfully, that’s over so she slept for 3-1/2 hours this afternoon. She is back to sleep now and hopefully will get more sleep tonight. She was in a good mood today, but we were told she might not feel too well tomorrow from the ATG chemo drug they’ll give her.
Care Partners brought us a basket of goodies today. They provide volunteer services so they can sit with Kiira if we need a break, among other things. The amount of donations and volunteers at the hospital is inspiring. It really makes life a little easier while we are here.
Kira’s lab numbers were good–we are seeing the white blood cell counts drop, which is what we want at this point.
One day closer!
Day -4: Kiira has been doing great, but we worried about ATG, which she received today, because we’ve been warned by doctors and parents that this one makes you sick. We had a nice morning and did the bath/bandage routine. Sometimes it amazes me how Kiira can lay there for so long while we sing silly songs, make funny noises, and shake toys at her. But this is the life she knows.
I left the hospital this afternoon while she slept through ATG, an 8-hour process. They gave her Tylenol, Ibuprofen and Benadryl to alleviate effects of ATG. Jason stayed with her while I took my other daughters to see Inside Out. It’s funny how guilty I felt leaving Kiira to spend time with them, even though they need me too. The movie was about a little girl who moves from Minnesota to Ca and the emotions she feels about doing so. Quite fitting for our family! I loved the lesson about sadness being a valid emotion that we shouldn’t shove in the corner. While I don’t sit around and cry everyday anymore, there’s still sadness about the life-change we faced when we had Kiira and ever since. However, in the midst of that sadness, we feel the love and kindness of family, friends and strangers. The prayers are like a warm embrace, that covers our sadness with joy.
Please continue to pray for us, and especially Kiira. She did break out in a rash or hives from ATG. Supposedly they’ll go down in a couple of hours, but for now we are seeing it spread. It’s one of the least concerning reactions to the drug and doesn’t seem to bother her, but we hope things don’t get worse.
Thank you for keeping us in your prayers!
Day -3: Only 1 day of chemo left! I can’t wait! So the rash did go away today but when Kiira got today’s dose of ATG, it came back so they slowed it down and gave more Benadryl. Last night she also had a fever, but the culture came back negative, so it was most likely an ATG reaction, not infection.
Today she was pretty happy and has some PT time again to practice crawling and standing. She loves pulling herself up on her knees at the edge of the crib too. After a long nap, she wouldn’t eat, so I fed her through the gtube but she threw up some of it and wouldn’t eat for the rest of the afternoon/night. When she went to bed, I put a little in her tube and will do a little more later to see how she tolerates it. If she can’t get enough milk, she’ll end up in TPN, nutrition through an IV.
One day at a time and we face things as they come.
Day -2: Chemo is done!! Kiira didn’t get any hives or fevers today, so that’s good. However, she is retaining too much water, resulting in too much weight gain and general puffiness. Just before we were told this, we were talking about her face looking more round and swollen and she’s gained 1/2 a lb in 2 weeks. So, they gave her meds to make her pee more and Jason is on shift tonight to deal with lots of diapers and wet clothes–lucky guy! She is still sleepy a lot, but when she is up she is happy and loved playing with a toy stethoscope that a nurse gave her since she always grabs the real one. We’ve also been feeding her 2oz through the tube every hour since she showed no interest in eating and to make sure her tummy would tolerate it, but thankfully tonight she took a bottle again and we hope that continues.
Her big wounds on her elbows, underarm, and thigh are finally healing so we can wrap a little less, but bath and bandages still takes around 2 hours. There’s a song we always make up to the tune of frere jacques (thanks to Aunt Kiki for that tune during bandage changes), and I believe Kiira not only “dances” to the beat, but today sang “ba ba ba ba, ba ba ba” to the tune. It was crazy! It’s the little things smile emoticon
So tomorrow is total body irradiation. It sounds scary and it kind of is, but she’ll be sedated and we can’t be with her, so I’m leaving it to God to protect her little body. It’s 300 RADS, compared to about 1200 that leukemia patients receive, but still enough to possibly cause issues down the road. It all starts at 8:00am, so please pray with us that it kills what it needs to and nothing more!
I’ve been in “heal Kiira” mode that I haven’t thought much about home, but the last couple of days I’ve thought more about what I’m missing out on, and even had a dream that I took a picture of a lake and said “I should be here instead of at the hospital”. I know it’s a season in our life, but like my 5 year old told me, I too miss California and our friends and family.
Her new blood/stem cells should be on a plane from Europe by now. We don’t know what time her BMT will be tomorrow, but probably in the afternoon. I can’t believe day 0 is nearly here! I feel the hope of a new life for our sweet baby girl.
They call the week before Kiira gets admitted, “Work-up Week”. They do a slew of tests to make sure all of her systems are functioning adequately enough to withstand treatment and to get a baseline so they know if something starts failing during chemo, radiation or after transplant. It’s an exhausting week of meeting with doctors, being inundated with information, signing forms to give our consent to all the awful things chemo and radiation do to a person, but we made it through–more importantly, Kiira made it through with flying colors! Here is a recap of the week:
Day -18: Today I had the pleasure of meeting Help Anton ‘s mom and Christie Zink from I Refuse EB. Both of these beautiful women have been an encouragement to me since Kiira was born and I felt privileged to meet them in person.
Plus, we received an amazing gift from my extended family to decorate our home. These family photos will be cherished for years to come!
Kiira is doing well and we hope she will pass all of her tests in this coming week, as they check all of her major organs. It should be an enlightening week!
Day -16: Kiira started her work-up today, which tells the BMT team about her overall health so they can be fully prepared for the BMT. She had a physical, EKG, echocardiogram, chest X-Ray, and blood drawn for labs, all without tears–even the needle poke! We also met with Integrated Health, nutrition, and a PA and Dr on the work up team. Her weight is back up so she is following a 30% curve, which was a relief, since she dropped to 10% in April after her gtube surgery.
We felt Kiira was handled so much that we gave her a full bath in the bathtub without wrapped hands for the first time ever. Kiira loved it! Doing bandages at the end of a day of testing was not easy though and Kiira was ecstatic to get on the floor to play finally at 5:30.
Everyone commented on how good Kiira looks, so hopefully the healing after BMT will be quick. At least that’s my prayer!
Plus, Kiira got to meet Anton (Help Anton) and his mama today, which was such a treat!
Back at the apartment, my mom and Kiira’s sisters, played, walked and swam. Tomorrow is another busy day of appointments so keep us all in your prayers!
Day -15: Although we were at the hospital for 6 hours today, it wasn’t as busy. We started with a radiation consult, where we learned about the risks of total body irradiation, including possible sterilization. At first I was upset, but I have to remind myself that with RDEB, most of the these things will be an issue anyway. Hopefully the low dose at her age won’t leave her with as many side effects.
The next appointment was with Occupational Therapy for a hand wrapping consult. She was impressed with the technique we use and Kiira’s use of her hands, which made me happy since I was so worried about it with our over-wrapping for the first 6 months of her life. We are wrapping in a way that helps prevent or slow webbing, but still give her the ability to grasp and touch things. It’s nice to be reassured that we are doing something right!
After that, we had time with our social worker, who talked to us about stress relief, among other things.
Then we had our GI consult. Tomorrow Kiira has an endoscopy, and much to my relief, he explained that he will go through her gtube to prevent injury to Kiira’s mouth and upper esophagus. They are looking for strictures and webbing of the esophagus. If she has any, they will do a dilation at the same time.
Since we walked to the hospital, we enjoyed a nice walk home as well. Kiira loves being outside so we don’t hear a peep from her while we walk.
We can definitely use prayers for tomorrow. Kiira will be admitted around 8:30am and put under for her central line placement, 5-7 biopsies, and the endoscopy. Please pray that the doctors work carefully on Kiira, don’t find esophageal strictures, and we have no issues with the central line.
On a side note, we also saw Prayers for Cole’s mom. This little guy was getting his BMT today, so please pray for him too!
Day -14: Kiira’s surgery went well. Her central line is in place and ready to use for the very frequent blood draws they’ll do and to administer medication. Then she had her endoscopy. They found inflammation in her upper esophagus, but no strictures or webbing. She is on anti-reflux meds to reduce inflammation and hopefully stop further damage (plus the BMT will help with that). Then they did 5 skin biopsies and a skin integrity test. It took7 minutes for Kiira to get a blister, which apparently is average for EB at her age. We will get tests too to compare, but a non-EB carrier was tested at 45 minutes and carriers, like us, will likely be somewhere in between. Then we went in the OR to do her bandages while Kiira was still sedated. They handled her very carefully, so besides the necessary inflictions, she looked good. Kiira is now home and resting. Thank you for your prayers!
Kiira also got to see Anton (Help Anton) again today! Having others here, going through what we are, makes us feel like instant family, which is such a blessing.
Day -12: Kiira is back to her normal, happy, active self. Yesterday we met with the BMT coordinator to discuss the BMT process, including the types of chemo and medications she will be on. It’s quite a list, but necessary for the new cells to make their home in Kiira’s bone marrow. We also met with infectious diseases and got Kiira cleared by them–she is healthy enough from an infections stand point to go through this process. He reminded us that there will be bumps in the road, but there is a light at the end of the long tunnel and we look forward to reaching that light!
Last night we went to the Ronald McDonald House for dinner and Bingo with Help Anton and Reid Davis Underwood. That is an amazing facility for kids going through medical issues. They really make life easier for the families, so save your soda can tabs and donate to RMH!
Today Kiira had GFR, a 4 hour test that checks her kidney function, and we learned how to flush Kiira’s central line. It’s a little intimidating because you have to do it in a sterile environment, and we aren’t sterile with her skin wound care, but I’m sure we’ll learn how to do this as easily as we do bandages now. We also met with pharmacy to learn more about Kiira’s upcoming medicines and side effects. It’s a long, uncomfortable list, but many EB sufferers live on pain and anti-itch meds, antibiotics, sedatives, among others, so hopefully this year of craziness will prevent us from a lifelong prescription pad.
We got to say hi to Dr. Tolar, one of the genius doctors behind EB research that’s led us to this point. I’m so thankful that God gives some people the ability to enjoy science and dedicate their lives to research that helps so many people.
We get the weekend off, have our exit consult on Monday and if all goes well, Kiira gets admitted next Wednesday. It’s been a long week, but we are excited the day leading to recovery is almost here!
God is good!
Day -9: We are finally below double digits! We had a great weekend off and celebrated Father’s Day at Minihaha Falls. Today’s exit meeting went well. Kiira is in good shape except she has slightly slow kidney function. Since some of the meds slow the kidney even more and trigger high blood pressure, they’ll keep a close eye on that. Kiira and the donor also tested positive for CMV, a common cold-like virus, but chemo and radiation don’t always kill it off, which can cause major issues when you have no immune system, so Kiira will get anti-virals for that. The positive side of it, if there is one, is that I can keep giving her my milk. If I was positive and she was negative, I would not be able to. We signed off on all the crazy side effects and we are moving forward to have Kiira admitted at 11am on Wednesday. She will get connected to an IV for lots of fluids and start chemo Thursday.
It’s been a long road and I know it’s not over, but with the amount of blistering Kiira has had the last couple of weeks (bigger and more than ever), I am reaffirmed in this decision. I would appreciate prayers as we go forward: for the donor; that Kiira will fly through this without complications and God will use this to heal her; for us, as parents, to have faith and strength; for my mom’s strength and energy as she watches Kiira’s sisters everyday; and Kiira’s sisters–that they will understand why we aren’t around much and be strong through this too.
Day -8: As I sit here putting Kiira to bed for her last night at home until after BMT, I have this bittersweet feeling. We’ve waited so long for this day to come… Today we spent our last day of freedom at Nickelodeon Universe. Kiira enjoyed watching her sisters on rides and when we got home, I observed Kiira rolling, sitting up, and laughing with her sisters. I wonder if she will be the same, happy baby when she returns. I wonder if the BMT will allow her to play with her sisters as actively as they do. I wonder if the BMT results will be enough to make me happy and I wonder “What is enough?” I don’t know yet, but so far Kiira exhibits amazing strength and resilience and as she watches my reaction with curious, soul-searching eyes, I only hope I can be strong for her.
In the midst of our own journey, this article popped up. As much I hate that other families have to deal with EB too, it is encouraging to see how other families are using their experience to help others, like this father, who the co-founder of EB Research Partnership, a non-profit aimed at finding a cure for Epidermolysis Bullosa. Father to ‘butterfly’ child: ‘I would give anything to take away the pain’
Happy 8 months to Kiira! She is starting to mimic our sounds, on the verge of crawling, wants to put everything in her mouth, and loves to be outside. Her giggles are contagious and despite the nightmare EB presents, Kiira fills us with joy.
We were able to go to the Como Zoo today since the girls were feeling better and it wasn’t raining. We only have a few more days before our days are spent at the hospital so it was a nice family outing.
We appreciate your prayers and comments. It really helps having the support. Thank you!
Our days leading up to hearing about the donor’s phsyical were tough. We were anxious and Kiira got more and larger blisters than ever. Here is our journey:
Day -29: Today we had our first visitors! Jenna, an 18 mo old with RDEB, came over with her mom, aunt, and sister and bags of groceries. We ate so much delicious food today! Thank you! It was so nice to meet another family going through what we do. Plus, between their family and a couple other locals, we have a big list of things to do. Since we haven’t really left the past few days, we better get to it, because in 3 weeks we will have very little freedom for quite some time.
Kiira also tried sweet potatoes and nearly gagged. We need to keep working on this food thing since we haven’t had much luck the past few weeks. My other girls loved them, so I’m not sure if it’s just these and cereal or all food. But, we’ll get there!
Plus, a huge thank you to Usborne Books and those who donated for my kids to have books and activities to do while we are here in Mn. They were delivered to us by the St. Paul firemen.
Day -28: You know it will be a rough day when your baby wakes up with an area soaked with fluid from a blister. We are trying so hard to keep Kiira wound-free for the next 28 days, but sometimes things just happen like this giant blister under her arm. Usually I check her body at least once a day to avoid these monsters, but it must have grown in the night. Then she tried to chew on her new books and cut her chin, under her nose, and her forehead. She can’t even hold a book! Plus, I lost count of all the blisters we popped today. I don’t know why they come in waves, but today I am NOT feeling accepting of EB. Ugh, this BMT can’t come soon enough!
Day -27: Today wasn’t as eventful for Kiira’s skin, but we are trying to be ultra-careful. I think we had only 5 small blisters to poke. We still haven’t made much progress with baby food, but Kiira wants ours! I believe the donor gets her physical in the next day or two so please pray all goes well!
Kiira has decided to get very upset when we don’t let her touch the books, so we added some soft books to her Amazon wishlist.
Thank you for your prayers and comments showing love and support to our family. They’re very comforting!
Day -26: I am so thankful to you who are supporting us with prayer and the gifts for Kiira and our family. She will be so happy to read her own soft books! And to my friends who got the kids tickets to various activities in the area–thank you!!! It helps lighten our load when we feel lifted up in prayer and you bless us so kindly.
Today my mom took the older girls to Mall of America, while Jason and I worked and Kiira played on her blanket. I love watching Kiira observe her sisters and her face lights up when she sees them. It’s really precious. I also notice that Kiira loves being outside–when she is fussy, she instantly calms down when we go out. Although it’s raining a lot here, the temperature is more tolerable for Kiira (heat makes her blister more).
Please pray for Kiira’s arm to heal. It’s looking pretty bad and we keep having to pop blisters on top of the big one, which isn’t good. I don’t want it to get infected.
Thank you again, and may you feel God’s blessings as you have blessed us!
Day -25: Our house has been hit with sickness! Between sore throats and fevers, I’m not sure if Kiira is escaping this one. Despite wanting to eat, she couldn’t take a bottle tonight. I couldn’t see any mouth blisters, so either they’re too far back, she isn’t feeling well, or maybe teething. Thank goodness for the gtube! Kiira, Mimi, and I took a long walk today, and it was beautiful out. Then Jason and I got to have a late dinner out, which was a first in the last couple of months. Overall, a nice day, despite the kids being sick.
We also received a new toy and diapers for Kiira, so thank you to those who sent them!
We should hear next week if the donor passed her physical. I keep praying she did and that she is the best option for Kiira so that we can start on the 15th.
Thank you to those who sent Kiira packages that we received today. She loves her new, safe toys and clothes. The cares, prayers, and love you show us is soothing to our souls in a way we can’t describe. Thank you!
Day -21: Today started off with Kiira’s arm soaking with fluid again, this time from a giant blister on her elbow. We’ve never had issues with her arms, besides her hands, so this was a hard reminder that Kiira lacks Collagen VII so no part of her body is immune from blistering, even though her arms have been wound free until today. She is rolling all around the room, so unless I confine her in the stroller, I know we will see more wounds, but I hate it. I hate EB.
Thankfully, the day ended with a couple more gifts for Kiira and the VERY exciting news that the donor passed her physical and is donating on June 30th. Thank you, donor, for this amazing sacrifice!! We begin work up on June 15th. That day begins at 8am with a series of 6 appointments, taking us until 3pm. We continue with appointments through the week, including a biopsy and central line placement on June 17th (just reading about it makes me want to pass out). Her transplant, or infusion, date is July 1.
I’m so happy this day is coming, but I’d be lying if I didn’t also say I’m terrified. Please pray that Kiira remains healthy so she can receive the new bone marrow, and for our emotional health as the day we’ve waited for over the last 7 months approaches.
Sometimes I try to show the ugly sides of EB because it is horrible, and I want to share, so that you will share with others and EB research will get the funding it needs to find a cure.
Despite the fact that Kiira has RDEB-HS, I know she has been more fortunate than many other babies. I didn’t know if she would live for a week, a month or a year. I didn’t know how I would survive dealing with it, watching her skin peel back with the slightest friction, poking her with needles (which used to make me feel like I’d pass out), doing daily bandage changes, and watching her suffer. But nearly 8 months later, here we are–both surviving, with smiles on our faces no less. She hasn’t had breathing problems or too many eating issues and she rarely has infections, but RDEB gets progressively worse. That’s why we MUST do something for Kiira. I can’t wait for her to need throat dilations, a wheelchair, hand and feet surgeries, to be on pain, anti-itch and antibiotics on a daily basis, or any of the other numerous side-effects of EB. That’s why this post by an EB mom on Facebook confirms my belief that a BMT is the best route for Kiira. I pray for the best outcome, but whatever happens, I will know we tried our best option available at this time.
These kids need a break from the pain. EB needs a cure. EB Research Partnership