Monday, June 14, 2010

bmt updates

I'd like to give an update on the EB kids (that I know of) who have had (or are about to have) bone marrow transplants at the University of Minnesota. This transplant seems to be our closest hope for a cure, although it's in the VERY trial stages right now and still extremely risky with inconsistent results.

I'm not trying to be negative. I think what Dr. Wagner and his team are doing is AMAZING, and I'm so thankful that there is someone out there fighting for these kids. We need someone who is dedicated to finding a cure, and Dr. Wagner, from what I read, is beyond amazing. And the families who are stepping out on faith and going through this nightmare in hope of a dream are so brave. I have nothing but respect and admiration for them, and I know we owe it to them and their kids when there is a cure for EB. Jonah has hope because of these kids. Please pray for them and for Dr. Wagner and all the doctors and nurses who are working to cure EB.

Keric - Keric is 17+ months post-transplant and doing well. His blood is 100% donor engrafted and his skin is 16% donor engrafted. His skin is getting stronger and heals faster. They are doing lighter dressings and sometimes eliminating them all together. Keric had an exact sibling match for transplant and is having positive results. You can check out his page here. (I do want to make a note that his mother said, in March, that DebRA of America has not contributed to Dr. Wagner's research. I emailed the folks at DebRA to ask about his, and they said that they give funding to DebRA International who has a panel or advisory board (with doctors and specialists from all over the world, including some Americans) that evaluates all the clinical trials and determines where to give the money. DebRA International HAS contributed to Dr. Wagner's clinical trial, so DebRA of America has contributed in that way... if that makes any sense at all. Nevermind.)

Hannah - Hannah is 14 months post-transplant and is also doing well. She also had an exact sibling match. Hannah's skin is getting stronger, she heals faster, she has no more anemia, and her parents report that she has an energy she has never had before. You can visit her page here.

Sam - Sweet, sweet Sam. Sam has been through so much. She is the oldest of the EB patients that have gone through transplant, and she has had a very rough road. She also had an exact sibling donor. Sam is 290 days post-transplant and is back in the hospital... again. Sam has battled infection after infection and is now battling Graft Versus Host Disease. Her mom says her skin is worse than ever and that she thinks GVHD is worse than EB. That's saying something. She just spent the entire week at home on two IV antibiotics and only got worse, so they are now back in the hospital. Unfortunately, Sam has not seen any consistent betterment of her skin. She (and her awesome mom!) could really use your prayers. You can visit her page here.

Rafi - Rafi is 115 days post-transplant and is doing well, overall. She did NOT have a sibling donor. Rafi seems like a super-amazing kid with an amazing spirit. The stories her dad tells about her little sassy self always keep me smiling, and she is so strong (like all of these EB kids!). Her dad shared some very exciting news. Her most recent biopsy results are possibly showing some evidence of "anchoring fibril." Like all EB kids, she is missing the anchoring fibrils that hold her layers of skin together. This could mean that she is producing Collagen VII (the protein she's missing) and would be AWESOME news! They are seeing general skin improvement and her former "problem areas" are looking so much better now. When she does blister, she's healing much faster. You can visit her page here.

McKenzie - McKenzie underwent transplant twice, but neither one of them took. She has been through hell TWICE, and has now gone home with no results. She is so happy to be back home with her family and her best friend (dog, Shiloh). She will be eligible for transplant again if she decides she wants to. It's a long, hard road, and I can't imagine enduring anything like that. She is SO strong at the ripe old age of NINE. Please be in prayer for McKenzie. Kids are so resilient, but she has been through so much. You can visit her page here.

EJ - EJ is 46 days post-transplant and still in the thick of it. His counts look good, but he's also been battling some GVHD. I have only found out about EJ recently and haven't had time to catch up on his whole story. I know they are still at the Ronald McDonald House, and EJ is missing McKenzie. They had become good buds. You can visit EJ's page here.

Daylon - Daylon will be the first Junctional EB child to undergo transplant. The other patients so far have had RDEB (Recessive Dystrophic). He has Junctional Herlitz, which is the most severe type of Junctional. Children with JEB-H usually do not survive to their second birthday. Daylon had his Hickman line placed today and is all checked in at the hospital. He starts chemo tomorrow, and things will officially be underway. If all goes as planned he will have his transplant on June 24th. Daylon has an exact sibling match and will turn one in July. You can follow his story here.

Bella - Sweet Bella has just celebrated her first birthday on May 27th and began all of her preliminary tests today. She has an exact sibling match. Her family is residing in Minnesota now and will be there for months to come. This is a quote from her dad from Sunday night, "Tomorrow begins Day 1 in the clinic. Labs, consultations, tests, etc. from 9-3. We'll share much more tomorrow night. We go in your hands, in God's hands, and in each other's hands. I've never felt more proud to be the husband to my wife and the father to my children than tonight. Why? We are ready. We are as ready as we can be for this, whatever it may bring. We are ready, and we are not alone. What else could we ask for? Life is Good. God is Good. Amen, and Amen." AMEN. You can follow Bella's journey here.

Thank you for indulging me. I know it was long (or maybe it was just long on my end). I know this may just seem like a bunch of facts and figures and a lot of blogs and pages to visit, but believe me when I say that it is worth it. I feel so helpless when it comes to DOING much of anything for anybody. I was telling my mother-in-law that I've been completely self-absorbed for the last 16 months. I'm not faulting myself for it. Circumstances called for self-focus. But I do want and wish to do more... for EB kids, for orphans, for the homeless. I want to stop talking and DO. Right now, it's all an abstract general thought, but I hope to start DOING very soon.

So for tonight, I'm putting faces to this horrible disease. I'm sharing these beautiful kids' bravery and courage with you. I'm asking you to pray for, love, and support them. Please, please, go and KNOW these kids. Don't be complacent. It's real. Jonah's not the only one. And I'll tell you, the more I read and know, the more I realize that he's one of the lucky ones. It could be so much worse.

(And speaking of awareness, Zack's mom has let the EB Community know that the public service announcement that Zack is in (in which Jonah's photo also makes an appearance) will air on the Discovery Health channels from 6 pm - 9 pm during the month of June, I believe. Let me know if you see it.)

Even when this post is buried months back, you can still follow these kids (and other EB Sweeties) on the right-hand side of my blog. Thanks, as always, for all of your prayers, concern, and desire to DO. We are not alone.


Katherine @ Grass Stains said...

I love that you love all the kids. I love that you are shining a light on them. I love that you have helped me open my heart to them. I love you!

Emily said...

Thank you for the update on all the EB kids. I had to smile at your line about Rafi's spunky self (as told through the eyes of her dad) because I feel exactly the same way about her. She's a great little girl! I look forward to following the rest of the links on your page and getting to know more EB kiddos. Again, thank you. And don't forget, giving back often means the most in small gestures.. donate a bag of old clothes, give a homeless person something to eat, small acts of kindness go a long, long way.

Melissa said...

i've loved following many of these kiddos. thank you for the updates, and thank you for introducing us to more of the bravest kids in the world. my kids love seeing jonah and were cracking up today at the videos! thanks...and stubborness will benefit him one day!!(or at least that is what i always say about my oldest girlie!)

Beth.. One Blessed Nana said...

Patrice- thanks for sharing all of these precious little ones with us so we can be praying for them.

Love the new jonah haircut! you did a great job and he is more handsome than ever.

Love to you sweet one,

Laura A said...

Hi Patrice-

Thanks so much for sharing these stories. I've been following Sam, Rafi and McKenzie for awhile now, and while I'm really excited for Rafi, my heart is breaking for McKenzie and Sam. Sam has been through an UNBELIEVABLE journey and I know she and her family must feel so discouraged. I can't even imagine how McKenzie and her family must feel. We've added them to our prayer list!

Praying as always for sweet Jonah, too. Maybe one day BMT will be an option for him, but am praying that God will make all that abundantly clear to you and Matt.

Love from TX!

Susanne said...

I don't often comment but I felt compelled to tonight. I think you are selling your self short. You are doing one of the hardest jobs we are called to as Christians and that is being a witness. You are witnessing by sharing your story openly and honestly and praising God along the way. Thank you for sharing your honesty, humor, and most importantly your love for the Lord! You are a prayer warrior for these children who need a voice and you have invited and challenged so many to join in! What a gift! God's blessings to you.

Rebekah said...

Just recently I registered on the bone marrow registry. I hope one day I can be a match for one of these amazing kids!!!! :)

LAW said...

Thank you for updating and sharing your heart about others with EB! I've been reading through all their stories this morning and praising God and praying for each one of them!

While I was reading Keric's site (I think it was his) I was reminded of an article from another blog I follow. There's a blog called "Medical Hemostat" that I follow because my husband has a medical condition that involves collagen, fibrin and clotting at his dura. Any way, I digress... yesterday 'MH' posted an article about scientists replicating human collagen from (of all things) tobacco plants!

Here's a link to the article:

Here's a link to the blog:

I have NO idea if this type of research would be beneficial for EB patients, but we've been at Duke for a couple years now working with my husbands' doctors to find a fibrin product that will help him heal and while their medical conditions are quite different, it's somewhat similar in that both EB patients and others like my husband have collagen-related or fibrin-related healing issues.

So, we continue to pray!

By the way - Jonah looks great, and I REALLY enjoy seeing pics of YOUR great big smile as you delight in him!

Jennifer said...

Thank you so very much for lifting these kids to our attention. I will be praying for them and their doctors and families. You ARE doing something, Patrice. The auction, the attention and your PRAYERS are all DOING SOMETHING. Know God is at work, too. Jennifer of Southeast, NC

Emma and Company said...

Thanks for sharing. I have been wanting to learn more about this horrible disease and I am so thankful you have provided more courageous kids for me to pray for.

Deanna said...

Thanks for posting this, Patrice! I pray for these kids often, and I love having links to keep up with their stories.

Anita Johnson said...

Thank you for posting about these other kids and the treatments available to EB kids. I pray for Jonah from Wisconsin, and check your blog frequently. You continue to amaze me and so does Jonah. God Bless!

Greg and Melissa said...

Thank you Patrice! You are awesome.

Brittany said...

We live in Minnesota and are Golden Gopher alumni of the university - is there anyway we can help? Are all these families staying at the Ronald McDonald house on campus? If you know how we could get meals of breaks for these families - please let me know. We are far too close not to try and do something!!

The Goodman Family said...


Thank you so much for this post, it has really brought light to my day to see that many of these dear children are doing better, especially Rafi, who I keep up with moreso than the others. I know that I say this often, but we are still praying for you guys & it brings such joy to our faces to see pictures of Jonah crawling.


Lucy and Ethel said...

I'm just now reading this because I wanted to have some time (dinner can wait. Surely.). Thank you for sharing these stories, as I feel like an SMAEB mama by now! I'll be adding it to our blog.

I wanted to tell you to stop thinking you're not DOing enough!!! By spreading the word of these precious children, you're not only sharing their stories with countless others, you're opening the way for many, many more prayers on their behalf.

And that is huge.

How you're managing to find the time to do so much is a puzzle. Well, okay, not really; we do what we feel we need to do, no matter what it takes. And what we need to do here is squelch a couple of insidious genetic diseases.

You're doing a TERRIFIC job :)


Amy said...

I don't know how I came across your blog, but I've been following it ever since. And because of you I'm also now checking daily on Raffi and Trip. And last night I spent two hours reading Bella's blog from beginning to end. I don't know anyone personally with EB, but if I ever do I'll know so much about it and what they're going through because of you. Thanks for teaching us!

Angelique said...

Thanks for shining a light on all the EB BMT kiddos! Just knowing there are folks out there pulling for us gives us that extra strength when we need it most.

jardinera linda said...

the sweet "EB family". I follow most of them! I cannot do much to help, but i love reading about their fight and I believe it is a little help for them (and you) to know people KNOW.

EB families: You are fighters and i ADMIRE you.