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We have been absolutely stunned by the love and support we have received because of a local news story by a reporter who was moved by Kiira’s story (KCRA3.com), and has now made it’s way around the world. We want to thank you so much for the financial support, kind words, and most of all your prayers. We went to Stanford yesterday and I will post an update about that visit soon, but since it was a long day of meeting with EB specialists and then a very long drive home, we haven’t been able to respond to all of the comments. However, they have been read and are very appreciated.

We hope that all the attention that Kiira’s news story is receiving is bringing more awareness to EB and EB research, which is more of the focus of  Yahoo’s Story. We are in a race against time to keep Kiira’s skin and internal organs from deteriorating, so that is why we are pushing so hard for a cure, as I’m sure all EB families are. Unfortunately some have already lost the fight before a cure was found and my heart aches for those families. I encourage you to check out our EB Resources page–these organizations have been working hard and long to find a way to make EB more tolerable for those suffering from it’s terrible effects.

Some of you have asked to send checks (made out to “Elk Grove Church of Christ” with a Designation: Baby Kiira), supplies, or gifts directly to Kiira. You can send them to this address:

Elk Grove Church of Christ
Attn: Kiira Kinkle
PO Box 1588
Elk Grove, CA 95759

Thank you again and may God bless you all during this Christmas season!

I just got an update that they are planning to air Kiira’s story on the KCRA3 Thursday morning news. We don’t know an exact time, but it might run in the 5AM and 6AM PST news, as well as possibly during the 7-9 AM PST on KQCA My58. (The story will not change–so if you see it once, you will not miss anything in other time slots). It will also be available on http://kcra.com after it airs.

 

 

 

 

 

 

Kiira turned 2 months old on December 12th. We had a good week, where dressing changes overall went well and we haven’t seen any new blisters. One of the little blisters had a scab on it, and from our understanding of EB, it isn’t very often that the skin scabs. It tends to heal from the outside in. I mentioned that it was “weird” to Jason and he said, “Is it?” I guess not, considering the numerous prayers our sweet Kiira is receiving.

In addition, a ton of my family, including Jason and my older girls were sick, but once again, God had his hands on Kiira and me and we did not get sick. Of course, that meant I was caring for the family, but I was not alone. A sweet family brought us chicken noodle soup and I had other helpers for Kiira’s bandages. We have certainly felt the love!

Another God moment happened last night. We were at Bible Study and Kiira decided to spend the entire time screaming. I was in the other room with her listening to the Christmas stories in the other room when some of the moms came in and talked to me about colic, since Kiira’s screaming is somewhat normal on a nightly basis. I didn’t deal with this with the other girls, but they had with their kids, so I asked for tips. One of them was “white noise”. I put Kiira in her carseat and one of the moms put her phone next to Kiira with her white noise app blaring next to her. Kiira immediately fell asleep. While that alone was great, what was even better, was that when we arrived home, there sat an anonymous gift on our doorstep. We haven’t been opening these mysterious gifts since we wanted to save them for Christmas, but Jason decided to open one of them. Lo and behold, it was a white noise maker machine. Wow, just wow. If God can speak to whoever this mysterious gift giver is and tell them exactly what we need when we need it, He can heal Kiira.

I’ve received a couple messages from people recently who are so confident Kiira will receive this miraculous healing. I can’t explain how encouraging these signs from God are to me. Sure I still have some rough moments, but I have felt more uplifted this week than I have in the last 8 weeks. I have also stopped researching EB, as my mind needs a break. I am just trying to let go and let God, as hard as that may be.

Kiira’s 2-month checkup went well. Although her doctor doesn’t really know much about EB, he thinks she is looking great. She gained nearly 2 lbs in one month (11.1 lbs, 43%ile) and she grew 2″ since birth, so she is now 22.5″. I still see that she is eating well and since I wake her every 3 hours during the day, she is sleeping through at night. We had her audiology appointment today and once again they couldn’t get a good reading, so we’ll return at about 8-months old, but I really have no concerns about her hearing since she smiles at our voices and follows us around. Kiira is also starting to play with toys so it’s really fun seeing her bat at toys on her playmat or bouncer and know that we can now start to entertain her with toys. I also have not been great about giving Kiira baths for fear that I’ll hurt her. However, I finally got brave since she spit up all over herself and really needed one. So Kiira got her first full bath and loved it. We kept the bandages on and did bandage changes immediately following, but it actually went so well that I did it again today. I read how painful baths can be later on, so I’m glad she enjoys it for now and hope we can make it a fun “event” for her for as long as possible.

One thing I didn’t think about with dealing with Kiira was how much it would affect the rest of my family and probably even friends. I spent the last 2 months stuck in my own cloud of self-pity and constant worry. I struggled to see beyond what Kiira needed now and in the future, that I didn’t see how others were also impacted. In some ways it has brought us closer as we rally around Kiira, but in other ways it’s really tearing people up inside and we all try to deal with it in our own way. Writing Kiira’s story is my outlet, and so sometimes you’ll read very raw, difficult posts, because that is what I need to share at that time. Jason and I got our genetic tests back and it confirms Kiira’s results, as Jason and I both carry one of her mutations. This means some day our other girls will want to get tested to see if they are carriers and some of my other family may want to do the same so they know if they are at risk of passing it on. Again, this is another way it has an impact beyond just Kiira.

Sometimes I see moms holding their babies so easily as I did with my other 2 and I feel jealous that I can’t do that with Kiira. Sometimes I still wonder why Kiira, why us? I feel like over the last 2 months I’ve been holding back with Kiira–not allowing myself to connect with her for fear I would lose her all too soon, but something changed over the past few days. I see her beautiful smile, hear her laugh and my heart swells with love for her. She tries to “talk” to us and I love our baby conversations. People look at her and tell me she is beautiful and no one can tell by looking that she has anything wrong with her. I try to cherish these compliments, because I fear that someday people will look at her all too differently.

At church one Sunday we sang the Jeremy Camp song, “There Will Be a Day” and it reminded me this life is temporary. We all have aches and pains and illnesses and face death all too often. I don’t want Kiira to suffer and to have EB anymore. I want God to perform a miracle on her. While I wait for that, I will try to remember these words:

“I try to hold on to this world with everything I have
But I feel the weight of what it brings, and the hurt that tries to grab
The many trials that seem to never end, His word declares this truth
That we will enter in this rest with wonders anew

But I hold on to this hope and the promise that He brings
That there will be a place with no more suffering

There will be a day with no more tears
No more pain, and no more fears
There will be a day when the burdens of this place
Will be no more, we’ll see Jesus face to face
But until that day, we’ll hold on to you always”

The prayers we can use for this week:

1. Healing for Kiira
2. Peace and comfort for all of those who are impacted by Kiira’s diagnosis
3. Our appointment at Stanford goes well on Friday and we can get some major questions answered and good resources for Kiira.
4. Our family stays healthy through this cold/flu season
5. The news piece done on Kiira will bring much needed awareness about EB so that EB research can find a cure for those who are suffering everyday due to EB.

Again, we want to express our sincerest gratitude to all of you who have been praying, sending supplies, bringing food, etc. We are truly humbled, but feel so blessed to have the support of so many of you. We cannot get through this time without your love and support, so thank you!

This week I started back to work. Although I only work part-time from home, it’s a great break from everything else on my plate. I’ve spent the last few days trying to get a hold of EDD to see about my disability benefits from my pregnancy since I still haven’t received them, but of course you can’t get through by phone, but since it’s hard for me to visit their office in person, I sent them a good, old-fashioned letter. I got a call from Medi-Cal Waiver office telling me I need to apply for SSI for Kiira and it’s best if I go in person. I asked her when I’m supposed to find time to do that, but the woman reminded me that this is a life-long disability and once it’s done and Kiira has SSI, we’ll never have to go again. I feel like it’s a futile exercise though since it’s based on our income and she won’t qualify for benefits. However, before we can be approved for the Medi-Cal waiver, we have to exhaust every other avenue first. She also reminded me to get our genetic testing done (we should have the results this week) so that we don’t bring another EB child into this world since it’s so awful. Thank you–I know it’s awful and even if I had wanted more than 3 kids, trust me, I wouldn’t have another one. Our CA Children’s Services app is also on hold until we get approval from the Medi-Cal waiver. It’s simply amazing how much paperwork, phone calls, and visits are needed in order to get my child much-needed supplies to care for her life-long disability. If a child is born with a condition, it should just be automatic care through either the insurance or the government–both things we’ve paid into for years and never used ourselves or for our other girls!! Through donations, we have supplies and funding to last us several months, which is so amazing, but Kiira will likely need bandaging for years.

Then I got a call from Dr. Tolar’s office (the doctor conducting the bone marrow transplants in Minneapolis) and we have to get approval from Kaiser first, which I assumed, but since our dermatologist isn’t on board with a BMT, I’m afraid we’re going to run into some issues. We just hope she sees it as an opportunity to consult with everyone we can so that when we are ready to move with a treatment for Kiira, we can.

While struggling to get all of this paperwork done, we’re also struggling with Kiira’s skin. My last post showed her improved rash, but the next day it returned and it looks like it will be an on-going issue. Dr. Tolar wrote me an email today complimenting our bandaging and management of infections (since she hasn’t had one since she was in the NICU), but it makes me want to cry, or maybe laugh, because in the last 2 days I see how holding her hand too tight during bandages gives her blood blisters, clipping her nails makes them fall off and tear the skin, the wrong outfit gives her a blister on her chest, and picking her up by her armpits makes multiple blisters there too. Her lip keeps bleeding from her popped blister and her tongue is covered in loose white skin. If these things that we are doing causes her so much injury, what is going to happen when she starts moving and causing her own injuries? Oh never mind–I have seen the pictures. I know what is coming.

Many offer to watch Kiira or help us with her so that I can get a break, but I don’t trust anyone with her, because I don’t even trust myself with her! Sure I can have some nurse come in here and watch Kiira a few days a week, but she’s not familiar with EB or how careful you have to be in handling a child with skin like hers. I know I couldn’t blame someone if they did injure her since I do it on a nearly daily basis, but it would be so hard not to. I feel torn between over-protecting her and wanting to run away and do anything else with my time besides watching my baby deteriorate as time goes on, as I know I have not even come close to seeing the worst on Kiira.

I told Jason the other day that it’s funny how we’ve prayed since she was born that this would go away and when it didn’t, we prayed that it would be the most mild case, and when it wasn’t, we prayed it wouldn’t be the most severe, and I guess it’s not, because some EB kids don’t even make it 7 weeks, but it did end up being RDEB with no collagen VII, which is pretty much one of the worst EB diagnoses possible. My constant prayer is “God, please heal her!”, as I can’t muster up any other words.

People keep ask how I’m doing–I could lie and say that I’m great and everything is under control and I’m happy that God gave Kiira her condition. Or you can keep reading my brutal honesty about I feel for now. I feel like an empty shell, just going through the motions. Time to feed Kiira, time to change her diaper, time to feed the other kids and get them dressed, time to pump, time to work, time to do laundry and dishes and wash the bottles for the millionth time, time to do Kiira’s dressing changes, time for Kiira’s 2-hour evening cry fest and at long last, time for bed–my favorite part of the day. One of the kids is crying, or being funny, but I feel nothing. I don’t feel empathy for them and I don’t feel like laughing with them. I can’t get what I’ve lost or what I have coming in our future off my mind. Sometimes I let myself cry, but usually I just try to feel nothing so I can get through the day. Some days I can keep it together and I start treating Kiira like a “normal” baby and then my actions cause her injury and I get so angry that I have to carry her around as little as possible and when I do, it’s on her pillow. Nursing my other daughters was such a bonding time to me and instead I feel burdened by pumping and feeding Kiira with a bottle because it’s hurting her and if I can’t even cuddle her, I can’t imagine trying to nurse her. People want to help but I don’t want the help, not because I don’t appreciate it, but because I don’t want to have to need it! Such silly things even cross my mind like the fact that she couldn’t wear a pretty dress and headband for Thanksgiving, she probably won’t be able to eat chips when we go out for Mexican food, and she may not even be able to color like her sisters do since her hands will be either be wrapped or too damaged to hold a crayon.

In the hospital I kept crying out “Why?” and I felt this whisper from God saying, “Because I love you,” but I still can’t wrap my mind around how God’s love for us can also burden our hearts so heavily or how is love for Kiira can still allow her to live a life with EB. I don’t know that I’ll ever understand that whisper to me. If I do, I will surely create an updated post! Supposedly we never get more than we can handle, but I am definitely feeling that theory put to the test. After bible study on Monday I told Jason that I hear stories of how parents are up with their children all night because they’re itching or in so much pain that they can’t sleep. I said, “God can’t give that to me too, because I cannot function without sleep.” Ha! That night Kiira was up nearly all night crying, which is a first for her (remember my last post started with how well she was sleeping), and I got about 2 hours of sleep. I guess God was telling me that even without sleep I can survive another day. I won’t make that mistake again… So the next time you ask how I’m doing, I’m probably lying if I tell you I’m fine, but I just don’t want to get into it. The best thing you can do is tell me you’re praying for Kiira, because I truly believe God is listening.

Even with all of these terrible, raw, empty feelings, I’m reminded once again of Kiira’s middle name, “Faith”. I know I have to keep having faith. I know God has a plan in all of this. I still don’t understand why He allows children to be burdened with conditions and diseases to make a point to the rest of us, and I don’t know why He chose us. But I have to keep having faith that a cure is coming and until then, God will help us get through this one day at a time…

Life with Kiira has been getting more routine. She is sleeping well at night (if you can sleep through her baby grunts) once she falls asleep around 10pm. Most days the wrapping goes quickly, but there are still days when she is not in the mood. After we gave a few of her fingers new blisters after a rough day of wrapping, we decided that it’s better to not do it that day, or at least at that time, than to wrap and cause more injury. Kiira’s lip keeps getting blistered and then it will heal and blister again from the bottle. Hopefully it will toughen up enough that we won’t continue to see the blisters here. Kiira also endured 2 weeks of a rash mostly on her head, shoulders and trunk, but it has finally cleared up. I’m realizing how sensitive her skin is and how long it takes to heal with EB. Some of the rash bumps blistered, but even they’re gone! We are back to lubricating her skin multiple times a day to keep her skin clear. It’s amazing how dry her skin feels without the ointments on her face, which makes her itchy and then rashy. Just another EB side effect.

We are scheduled to go to the Stanford Clinic on December 19th. We will meet with several specialists, such as a dermatologist, physical and occupational therapists, and a nutritionist. We’re hoping to get a lot of questions answered and get more advice about Kiira’s future and possible treatments.

As I have mentioned, we are possibly seeking a bone marrow transplant for Kiira based on her genetic results (if she even qualifies). I know some have reservations about it because of the high risks it imposes. When I talked to Kiira’s dermatologist about it, she felt it was too risky, but told me that we don’t have to worry about her hands and feet fusing until she is about 2. Kiira wouldn’t need an esophageal dilation until she is about 5 and the earliest skin cancer appearances in EB patients is at about 8 years old. Hm, and I want my baby to go through all of those things, plus numerous infections, extensive scarring, nutritional issues, internal damage, physical developmental delay, daily pain and medication and more? While we continue to pray for miraculous healing for Kiira so that she wouldn’t have to go through a BMT or a life with EB, we know we also need to be proactive. We don’t even know if Kiira is a candidate for BMT, but with 2 mutations that result in no collagen VII production, we want to follow up on any possibility that can give Kiira a better quality of life.

Below is a slideshow of Kiira’s progress from birth, some reinjuries, and healing at 6 weeks.

Although I have many things to be sad and angry about, I also have so many to be thankful for. I am so thankful for our friends and family and people I don’t even know that have helped entertain our girls through babysitting or gifts, sent clothes and supplies for Kiira, and the many donations to Kiira’s fund for continued help with supplies and nursing care which we are getting in the upcoming weeks. Others have helped with setting up this website, our Facebook page, our Amazon wish list page, and the ability to provide tax-deductible donations, making special clothing, and so much more. We seriously couldn’t make it through this time without the support of so many of you. I am truly thankful for everyone who has shown us God’s love through giving of time, prayer and resources.

The only clinical trial open to Kiira’s diagnosis and age is the bone marrow transplant in Minneapolis. A couple of helpful conversations with the parents of participating EB patients and and a review of the process is making them seriously consider this as an option for Kiira. It scares them to death because Kiira would have to go through a week of chemo and then go through the bone marrow transplant, most likely using one of her sisters as a match, and take on so many risks. She would probably be hospitalized for at least a month, but some have stayed much longer. A bone marrow transplant is not a cure, but would deliver collagen VII to Kiira’s skin as needed. Some problem areas, which they can already identify are her calf and heel; may remain problem areas until gene protein therapy is approved for her use. However, the way they look at it is: Kiira will end up with these risks and more just with EB and if there is a chance to give her a better life, why wouldn’t they take it? Of course, they still need to see how things go over the next few months and if she remains mild, they wouldn’t pursue it, but right now, they want to get the ball rolling since so few patients are taken. Thankfully Jason and Kirsti have jobs that would probably allow us to work from anywhere, so even if they ended up in Minneapolis for months, they could make it work.

To learn more about the procedure you can read it here: http://www.bmt.umn.edu/patients-families

So new prayers are needed to help them with this decision. If doors open for them to do it and she is a candidate for it, then they will take that as a sign that this is how they should proceed, but if not, then they will just trust that God has other plans for her.

This post from “Help Anton” explains in detail why they are considering a BMT for Kiira despite the risks involved:

As I have mentioned, we are possibly seeking a bone marrow transplant for Kiira based on her genetic results (if she even qualifies). I know some have reservations about it because of the high risks it imposes, but I feel like the post from “Help Anton” below explains our reasons so well. When I talked to Kiira’s dermatologist about it, she felt it was too risky, but told me that we don’t have to worry about her hands and feet fusing until she is about 2. Kiira wouldn’t need an esophageal dilation until she is about 5 and the earliest skin cancer appearances in EB patients is at about 8 years old. Hm, and I want my baby to go through all of those things, plus numerous infections, extensive scarring, nutritional issues, internal damage, physical developmental delay, daily pain and medication and more? While we continue to pray for miraculous healing for Kiira so that she wouldn’t have to go through a BMT or a life with EB, we know we also need to be proactive. We don’t even know if Kiira is a candidate for BMT, but with 2 mutations that result in no collagen VII production, we want to follow up on any possibility that can give Kiira a better quality of life.

Help Anton Post
“I have had a few people ask me why we are doing the bone marrow transplant now instead of waiting for the gene therapy that Dr. Tolar is working on. I thought it was a great question so I’m going to answer it here just in case some of you were wondering the same thing or were just wondering why we are moving forward with the Bone Marrow Transplant.

We have known about the Bone Marrow Transplant pretty much since we found about about Anton. We have followed Dr. Tolar’s work closely and we were very hopeful that at some point Anton would get the Bone Marrow Transplant done.

The first two years Anton was home we just didn’t think the risk of getting the Bone Marrow Transplant done outweighed the risk of not doing it. He seemed pretty healthy (although he was hospitalized several times for infections). He didn’t seem like he was in a lot of pain, and overall he had a great quality of life.

As more time has gone on I have have seen countless children and adults lose their battle with EB. Most of the people who have passed away these past few years have Anton’s type of EB and most of the people who have passed away have died before they turned 20.

The harsh reality is Anton most likely won’t live to see his 20th birthday unless we do something to intervene.

During the past 6 months or so we have noticed Anton is in more pain than not. He is still happy and joyful, we can just tell that his EB is affecting his daily life. It seems like his quality of life is not what is could/use to be.

Anton had a normal echo done on his heart in October of 2013 then in July of 2014 his echo showed that the left ventricle of his heart was dilating. At this point his heart is still functioning normally but I talked to the cardiologist at the patient care conference and he said almost always in patients with RDEB once the left ventricle starts dilating it will eventually turn into cardiomyopathy. (Cardiomyopathy is a weakening of the heart muscle or another problem with the heart muscle. It often occurs when the heart cannot pump as well as it should, or with other heart function problems. Most patients with cardiomyopathy have heart failure, “google”)

If Anton loses heart function he will no longer qualify for the Bone Marrow Transplant.

Dr. Tolar spoke at the Patient Care Conference we went to this year and it was so amazing to see how far his research has come. It is FAR less risky than it was 2 years ago.

It is hard to know how much Anton needs this when you see mostly happy posts on his facebook page. The harsh reality is Anton’s eye lid gets stuck to his eye ball at night and when he opens his eyes it literally tears the top layer of his eye, he has a large wound on his knee that has NEVER healed in the almost three years he has been home, he is terrified to poop because the one time his stool was hard it tore the lining of his bottom causing extreme pain, he gets blisters in his throat that pop which makes him throw up blood, his fingers are fused together and he has lost almost all the function in his right hand because his thumb is fusing to his hand, bath time is painful instead of fun, he can’t scratch his face at night without tearing all the skin off his nose. This sweet boy suffers so much and if we can help relieve just a little bit of his suffering we want to.

Knowing that if Anton loses heart function he will won’t be able to have the BMT done, seeing how much pain he has been in, and knowing that the Bone Marrow Transplant could at the very least give him a better quality of life we felt like it was the best option for Anton to move forward with the transplant.

We are in close contact with several people who have had the transplant done and we are very hopeful for what the future will hold for Anton post transplant.

Dr. Tolar is an amazing doctor and he truly cares about people with EB and their families and I know that he won’t stop fighting for a cure.

We are thankful that God has allowed us to move forward with the Bone Marrow Transplant. We are praying for God’s will in all of this and praying that God will be glorified through Anton’s life.

For from him and through him and to him are all things. To him be glory forever. Amen. Romans 11:36″

I thought some of you might be interested in knowing what our daily wound care process entails.

1. Cut all of the needed supplies
2. Unwrap one extremity at a time and gently wash with water
3. Slather petroleum dressings with vaseline or Aquaphor
4. Put small pieces between finger/toes to prevent webbing
5. Continue to wrap hands/feet up the wrist/ankle as far as needed
6. Wrap each finger with the dressing (we don’t do this to the toes)
7. Add a piece of Mepital (a thin foam pad) to the heels to prevent damage when Kiira kicks her feet
8. Wrap hands/feet with stretch gauze
9. Cover hands/feet with tubular bandage
10. Cover hands with mittens
11. Slather Kiira’s other body parts in vaseline or Aquaphor

Jason does the feet and I do the hands. One of us keeps Kiira calm or feeds her and holds the hand or foot while the other does the wrapping. The process has been taking us about an hour once a day when she is cooperative. If she is fussy, it can take us nearly 2 hours. Kiira currently has no open wounds, so we’re just waiting for the old wounds to finish healing and preventing new ones from coming.

We follow this Stanford video approach for an EB baby: http://dermatology.stanford.edu/gsdc/eb_clinic/eb-videos.html.

If you prefer to read about it, we use this procedure:
http://dermatology.stanford.edu/gsdc/eb_clinic/eb-resources.html

The past week has been eye-opening for Jason and me. We received a ton of encouragement, prayers, and support. We feel so loved and know that Kiira is loved as well! Thank you all!!

On Friday we received an email from Kiira’s geneticist telling us that based on her presentation they are classifying her as Recessive Dystrophic Epidermolysis Bullosa – Generalized Other (or previously known as non-HS). We were feeling slightly better about this diagnosis, but still had some doubts, because both mutations show patients that were classified as generalized severe (HS). We also found out that both mutations cause no Collagen VII formation, which means that no matter how much we try to protect Kiira, her skin won’t ever produce it and she will always be at risk, externally and internally and usually no Collagen VII is classified as severe. However, we have to keep remembering that Kiira has her own genetic make-up and some of that could override the lack of Collagen VII, making her have a more mild case. Whether she is non-severe or severe, EB will affect her drastically.

Some have told us we should get her a GI tube to keep her from losing weight and on anti-reflux medications right away to prevent severe, irreversible damage to her esophagus. So far Kiira is still eating really well and doesn’t spit up. However, it’s always in my mind to be aware of any little changes in her so that we make sure she isn’t getting internal damage.

We then talked to an EB mom who is traveling to Minneapolis to get her 5-month old a bone marrow transplant. The idea of such a drastic measure had barely crossed our minds, especially so early in her life. We have just kept praying that a miracle would happen and Kiira would be cured of the disease without any medical treatment, which can still happen, but after the conversation, we realized sometimes we do have to take action. Although Kiira might be presenting mildly for now, we learned that can change. Another conversation informed us that usually between 3-6 months we can see if things are getting more or less severe. A recent post showed us that even after a couple of years, things can take a turn for the worst, so this EB thing is all over the place–we can never go into a day thinking we’re safe from it’s hold on us, which is no way for us or for Kiira to live, and is why we’re now looking into our options to help better Kiira’s life.

The only clinical trial open to Kiira’s diagnosis and age is the bone marrow transplant in Minneapolis. A couple of helpful conversations and a review of the process is making us seriously consider this as an option for Kiira. It scares me to death because Kiira would have to go through a week of chemo and then go through the bone marrow transplant, most likely using one of her sisters as a match, and take on so many risks. She would probably be hospitalized for at least a month, but some have stayed much longer. A bone marrow transplant is not a cure, but would deliver collagen VII to Kiira’s skin as needed. Some problem areas, which I can already identify as her calf and heel, may remain problem areas until gene protein therapy is approved for her use. However, the way we look at it is Kiira will end up with these risks and more just with EB and if we have a chance to give her a better life, why wouldn’t we take it? Of course, we still need to see how things go over the next few months and if she remains mild, we wouldn’t pursue it, but we want to get the ball rolling since so few patients are taken. Thankfully Jason and I have jobs that would probably allow us to work from anywhere, so even if we ended up in Minneapolis for months, we could make it work.

To learn more about the procedure you can read it here: http://www.bmt.umn.edu/patients-families

So new prayers are needed to help us with this decision. If doors open for us to do it and she is a candidate for it, then we’ll take that as a sign this is how we should proceed, but if not, then we’ll just trust that God has other plans for her.

Kiira had been doing really well over the past week. We weren’t seeing anything new and her wounds were healing very well, but a few days ago she kept rubbing her face, causing a rash. We’re not sure if she is itchy or just a wild newborn who likes to fling her arms when she’s upset, but the rash hasn’t improved. We also discovered a couple new, tiny blisters on top of existing ones on her legs, which is where the scarring comes in. Every time we bandage her hands and feet we run our fingers between hers to make sure there is nothing webbing together. We’ve freaked ourselves out a few times when we see dead skin from one finger that looks like it’s connected to another finger, but we realize it’s not. So this is our reality.

In the meantime we’re close to finding an in-home nurse to help us out so that Jason doesn’t have to leave work early every afternoon. Miia (our oldest daughter) was really sick over the weekend, so Kiira and I lived in our bedroom all weekend while Miia infected the rest of the house and family. We pray that Kiira stays well as we don’t want to compromise her immune system since it’s so busy healing her skin. Being housebound for the past 4 days has driven us all crazy, but hopefully we’ll be able to get out soon!

Prayers for this week:

1. Kiira and the rest of our family stays well
2. Discernment about when God is opening doors for us regarding Kiira’s future
3. Continued healing for Kiira
4. Patience for Jason and I as we try to juggle the 3 kids, work (I’m likely going back to work in 2 weeks), and daily life.
5. That we bring in the right nurse to meet Kiira’s needs

Thank you again for your love and support. We could not get through this time without your prayers, words of encouragement and and help with various tasks like research, ordering supplies, and bringing meals. We truly appreciate the blessings so many of you are pouring over us.

So many of you have asked how you can help us. We love all the support you want to provide, so here are a few ways:

1. Pray: pray for total healing, for strength for Jason and me to care for Kiira in the best way possible, but also have energy and time for our other girls, and for comfort that we can feel God is with us through this journey
2. EB awareness: no one has heard of EB until you know someone with it. Even many doctors and nurses have never come across this rare condition and because of that, it doesn’t get much research funding, wound care supplies aren’t covered, and people are afraid of it. Let people know about EB and how it affects families in so many ways. You can read more about Kiira’s diagnosis here: http://www.debra.org/dystrophic 
3. “Like” Kiira’s Facebook page to follow her progress: https://www.facebook.com/kiirakinkle 
4. Caring for Kiira: while we are fighting to get insurance to cover the costs of supplies or get the MediCal waiver and CCS to help with an in-home nurse and supplies, we have set up 2 ways you can donate to Kiira and it’s tax deductible for you or you can buy supplies directly from Amazon:Option 1:
   Http://novatofirefoundation.org: click on donate. There is a form for mail donation, or email link for info.

   Checks can be sent to:
   Novato fire district
   Attn: Novato fire foundation
   95 Rowland way
   Novato, CA 94945

   Or call 425-878-2690 and donate over the phone

   Make sure you put in item line or form or if you call that it’s for Kiira’s fund and through the foundation. 100% of proceeds will go to caring for Kiira.

   We also have the ability to accept credit cards, so you can find me or a family member who can swipe your card and it will go through the foundation.

   Option 2:
   Donate through Elk Grove Church of Christ and all proceeds will be tax deductible and go to Kiira. You can add a donation on PayPal here: https://www.paypal.com/cgi-bin/webscr?cmd=_s-xclick&hosted_button_id=PQUP48MK6Y6RW

   Option 3:
   Purchase supplies for Kiira from Amazon: http://www.amazon.com/registry/giftlist/9814Y67RB1OB

    

5. Sign up to bring us a meal. We have really appreciated the meals people have already brought to us as it allows us time to care for Kiira and our other 2 girls. We have it set up here, but you will have to sign in to see it:https://www.mealtrain.com/view/?id=b4csy9CPa5A%3d 
6. Donate to an EB research fund such as DEBRA.org or http://dermatology.stanford.edu/research/research.html.

Please do not feel obligated to give. Your prayers and words of encouragement are needed the most. Thank you for your love and support of Kiira!

6 years ago yesterday I had to say goodbye to my first child due to a miscarriage. The event rocked my world and I thought I would never recover. I still remember the raw pain I felt during that time, but after 6 months, I finally found the strength to try for another child. Since then, I had 2 beautiful, healthy daughters and we decided to try for our 3rd. We conceived Kiira the week that one of our closest friends died tragically and she was born the week of his birthday. I spent a lot of my pregnancy in fear of how I would handle a 3rd child and when we contemplated “Faith” as her middle name, I wondered if I could uphold the meaning of her name in my own life, especially after she was born with EB.

Over the past month I’ve tried my best to hold on to my faith–to believe God can and would heal Kiira. Then we found out she had dystrophic EB–not exactly the diagnosis we prayed for. Today, we got our genetic test back and found it’s recessive–again, not what we were praying for. However, when we talked to the dermatologist about the results and then met with the geneticist, I felt this odd peace and strength, which I can only attribute to God and the prayers that so many of you have prayed for my family. Both doctors feel Kiira is a mild case based on her presentation, but we still don’t understand the genetic results. Jason and I decided to get genetic testing as well to see which gene each of us carries–more out of curiosity than anything, since it doesn’t change the fact that he and I are healthy and Kiira has EB, and that won’t change.

The first mutation shows that Kiira cannot produce Collagen VII which is essential for binding the lower layers of skin to the upper layers. Unfortunately, this is not a favorable mutation, but we’re reminded that our bodies are made up of so much genetic coding, that we don’t know how our other genes will interact with this mutation and what it will end up looking like for Kiira. We know she can wear clothes and a diaper and she is eating and gaining weight. We haven’t noticed any trouble with breathing or reflux, and she seems healthy and rarely gets new blisters. We know some with RDEB are not this fortunate. I often find myself looking down the road, wanting to know what challenges we’ll face with Kiira, but everyone tells us to take it one day at a time. Stay focused on wound care, try to minimize infection, and maximize nutrition–those are our tasks for each day. We are complemented on our wound care approach (thanks to the Stanford videos and Kaiser nurses), but sometimes I still want to scream about how unfair it is that my child cannot spread her fingers, hold my hand, touch things like a normal child should be able to. On nights like tonight where she is so amped up, that it takes us nearly 2 hours instead of 1 and I’m beyond exhausted since I’m lucky to get 2 hours of sleep at a time, I just want to throw in the towel and give up. But there is her name…”Faith”. A constant reminder that I’m not alone and even though things aren’t going my way, there is someone there to walk along side me.

So when I worried about the struggles I’d face with a 3rd child while I was pregnant, I really had no clue what was in store for us. I sometimes get overwhelmed, but I really try to take one day at a time and it helps me get through the day. I’ve also tried to keep busy and get back to some of the routines I had before Kiira and that helps me keep my sanity. As we learn more about the genetic results over the upcoming weeks, I expect that we’ll have slightly more understanding of what could be in store for us and our sweet baby, but I know that I need to keep holding on to the faith that it’s not just genetics that determines Kiira’s outcome–God plays a role in all of this and He can heal Kiira.