Thursday, February 10, 2011

February 10, 2011: Back on the treadmill...


Well, day 2 back in the gym.  Feels GREAT.  Remember how full of excitement I was a the beginning of the year to hit the Transformation horse?  Well, I fell hard off the wagon.  I realized the easiest thing to do is simply replicate what has worked best in the past.  Pure and simple!  Why re-invent the wheel?  I know that I need to work out before work, otherwise, it won't happen, and I know that I need to work out in a gym.  That's all there is to it.  It's so funny; now that I am physically back in a gym, the gear has already begun to click.  I like a routine.  Structure is good for me.

Anyhow, Ang worked from home today, so we walked over to pick up Ali from pre-school together.  Ali lit up live a firecracker when we both walked in!  Ali's teacher immediately walked up to us to say how well she is adjusting, and how amazing her vocabulary is!  "She is just like, "Here I am!"" was how Mrs. Frazer described Ali's confidence.  Nothing makes a parent prouder than when a teacher beams, right?  LOVE it.  Ali is so excited also because she is going to the same school daddy went to!  I took one class during my degree at this community college, so Ali thinks we've gone to the same school now.  It is too cute.  It works for me; I LOVE having her imprint COLLEGE in her mind at such an early age!

Thanks for the continued questions about the trial!   Really great stuff!  Let me address some of them for you:

What happens after BMT?  Well, BMT unfortunately never really ends.  LOL.  What I mean by that is that there is follow up care that may be as infrequent as annually if all goes well and no side effects are present.  Really, in this case, there is a study to monitor the changes in skin 100 days after transplant, so that is why there is a lid on this study.  The patients still need to come back for biopsies every year.  The whole initial concept is that the Collagen VII will continue to grow into wounded areas over time, so the more wounds that continue, the more healing with working collagen.

Here's the thing though:  the transplant as it exists now does not "Cure" anyone of EB.  It simply ameliorates the disease into a functional, treatable condition, more like dominant dystrophic EB.  The skin gets better, but there are always a couple of wounds.  This brings up another important point.  The older the patient, the more likely that they have bacterial infections that have left permanent colonies in the tissue, almost like TB.  So, when the body gets stressed or depleted, they colony can flare up or break out.  This is most likely why the real problem wounds for many kids NEVER heal... because of the permanent residents in the tissue preventing the growth and healing to occur.  THIS is another reason why we need the funding to get to the next generation of BMT as well as systemic gene therapy.

Kidney issues are not uncommon for BMT, but they are more common in EB.  Again, prior to U of M studying EB, it was primarily studied by dermatologists, not nephrologists.  There seems to be some sort of inflammation that occurs internally at a much greater level with EB, and again, the docs aren't sold that collagen VII only resides in the skin.

Yes, we were able to see dramatic differences in Bella's skin after transplant.  See Aug 2 and Aug 5 posts for some good before and after pictures.

Last, the doctors change the protocol roughly every 7 kids.  The doctors continue to do everything they can for EJ, McKenzie, and Sam.  The one thing all us parents in the study knew from the get go was that there were no promises made, and we all rolled the dice.  The thing is, it's not like it was a walk in the park for any of these kids before hand.  BMT is so complex, and frankly, still so barbaric.  You just pray your child sneaks through, but it's like 10 kids running through a mine field; you KNOW that a certain number of them aren't gonna make it to the other side.  I remember one EB parent offering their condolences to me after Bella died, and I honestly wasn't sure who should be consoling who.  Their child still had EB, was still suffering from it, and was most likely still going to die from it.  At least Bella was in no pain.

The thing that sometimes works at me is that Bella was truly a happy girl most of the time.  She didn't have any persistent, nasty infections.  Her bath time was still relatively peaceful, as she didn't have a lot of nasty wounds that just wouldn't heal... yet.  We figured, she's as healthy as an ox, and at only 1 years old, if we go through this now, she'll have virtually no memory of it, and we'll treat it before it gets worse, which is the only direction the arrow moves with recessive dystrophic EB.  Sometimes I wonder, what if we didn't go through with it?  Would we still have her?  Wouldn't THAT have been better?

God night.


  1. You did what you thought best, so what you did was the right thing to do. Bella may not be here anymore in person, but she is in our hearts. She changed lives for the better.

    There is no doubt that going through the trial is hard, but the alternative -suffering EB- is no better. Seeing Tripp's state now breaks my heart. And i totally respect Courtney's or any other parent's not to try the BMT, as i respect those who decided to try.

    Furthermore, I truly ADMIRE those of you who decided to do the trial, because you open the way for the ones who come after you and your kids pay with their life, or worse, with horrible pain.

    I so wish to see an end to this cruel EB.

  2. No one can answer these questions, Tim. No one was in your shoes then. We all prayed for Bella and wished her a happy childhood. The BMT is still so new, no one could have given you a good advise then, even the doctors. I am so sorry for your loss...

    Jardineralinda, Tripp has JEB-H, it's the worst EB. For him the BMT is even more risky. I agree with Tim that condolences must go to the parents of suffering EB children.

    Tim, thank you again for the insight into BMT! Have a nice day!


  3. Like all parents, we want our best for our children, so I'm sure that 's what you did.
    I'm happy to say that I sold 30 packets of Valentine's biscuits for my fund raising !! The 300 biscuits I baked were a success!
    I'm glad to see that Ali is so happy at her new school.
    Have a nice weekend.

  4. What I wanna ask is if Bella was such a happy child and her skin had no infections, then why did you risk and loose her life? What did you gain from it?

    I don't get how you as parents put her through this all in the name to oose Bella and then wanna start this massive donation drive to fund more research. sounds to me like you are looking for a way to bury your grief.

  5. I'm reading these comments and need to respond to anonymous above me. My first thought is to tell that person "Are you kidding me" I can't even believe that person wrote that. Have they read your whole blog? And for that matter do they know anything about EB? My guess is they don't know anyone with EB.
    I will stop at that because really I think why are they judging if they aren't you.

    Just letting you know I'm still here, reading and thinking about you. I just couldn't stop myself from replying to that.


  6. Ohhh anonymous. I'm glad you aren't identifying yourself....just take a moment to go back and read. Its all there. Tim, don't take time to address those comments.....argh!

    On another note, your comment from Alis teacher made me smile. I'm Not surprised though. Even after 2 hours at Molly's party, my friends were impressed (all teachers, too!).
    Glad she's happy. Happy you're glad.
    Love from to anonymous.

  7. It's good to ask those questions anonymous (judgment, I could do without). Those questions are a reminder that a lot of people truly don't get EB and how horrific of a disease it is. I don't think my circle of friends would have understood either if they hadn't lived through it with us and Sarah. Friends who helped with bandage changes ALWAYS said after, that EB was 1000 times worse then what they had ever imagined it was for Sarah.

    December 2007, Sarah was only 3 months old. We were doing a bandage change (one of 3-4 we did a week). I was holding her leg, she wiggled and my hand slipped and the skin on her foot came off like a sock. Sarah screamed and screamed and all I could do was hold her leg, focus and hurry and fix what I had just done before making it worse. The pit in my stomach was horrible and I had to blink away the tears and get clinical while I fixed her. My friends who were helping were sick, one had to sit because she almost passed out from seeing Sarah in that much pain.

    I can't stop people from judging but I get graphic so there is an understanding about how horrible this is for children. THAT is why the Ringgolds are doing massive fund raising and why they took a chance with the BMT.

    Lonni Mooreland

  8. Oh my anonymous, you really don't have a clue, do you? Tim and Ang did what they thought was best for Bella. Maybe you should go to Courtney's house and watch a bath and dressing change. Maybe then you would realize what EB is all about. Until you see it first hand, you really have no idea how awful this disease truly is.
    My grandaughter died from this disease and I am so grateful to Tim for all that he is doing for our small EB family.
    Get a life anonymous. Leah's Nana

  9. I'm so sorry it was your daughter caught in the minefield (good analogy, btw) but it was not for nothing because the doctors really seem to be learning more and more to help the next child. I admire your courage and think you made the right choice. Thank you for continuing to share information about the EB journey.

    And to anonymous, if you are going to judge, at least leave your name.

  10. Anonymous... That was incredibly insensative. I don't understand how you can bring yourself to judge him like that even while hiding behind an anonymous curtain.
    Tim, that person can only treat people on a level that matches how they treat themselves. They must have quite an internal judgement zone, I pray that they will be able to set self free.

    You did the right thing, or else it wouldn't have happened. We don't always know His plan, or how far our ripples of intention reach, but hold on to your faith and know... If it were not right, it wouldn't have happened. I imagine how difficult it is, I'm so sorry. Peace and love to you..from me...
    Angela O.

  11. Blessings to you as I feel your pain as the grief is such a rollercoaster of emotions. Thank you again for being so open to share what you are going through. God is with you. Donna/NJ

  12. It is easy for people to judge when they are not put in your shoes. You did what you thought was best for Bella and that's all you can do as a parent.

    We have 2 kids who went through BMT's, granted, they do not have EB, but anyone deciding to go through the BMT process does not do it on a whim. It is hard to even put into words how difficult it is for a parent to watch their child go through that process.

    Take care, we think of you guys often!

    Julie Williams
    Foreston, MN

  13. God is our only Judge and He has given you the strength to continue your journey. Please don't let others bring you down. YOU are doing AMAZING things in Bella's name and she is smiling down at you because of it!
    God's Blessings

  14. Hey - Your last paragraph is an example of how much you and Ang (through you) share of what you went through and what you still go through. I often wonder why you continue the blog (I'm glad you do!) and do wonder how Elle's family is doing.

    Just from reading your blog and your honestly, Iknow how dedicated and devoted you were to Bella and still continue to be. I remember wondering after if you regretted going but you addressed it then. I would have done the same thing given Bella's circumstances. I'm sure of it.

    As parents, we will do anything for our children. Anything.

    The photos of Miss Bella at the end show those of us who came on board after BMT changed her looks who she was before. It has been a shock that she was so happy and when I read about Tripp's issues, I am in pain thinking about him. I take comfort that Bella had a different path BEFORE BMT. I'm so glad you share these things.

    Also, as a reader, I want to give you all my support. I have donated money. Right now most of our money is dedicated to things that are affecting living members of our family, but we will always dedicate some for EB until it's cured.

    Thank you for not retreating away from EB but charging forward to not only include those of us who were captured by Bella's spirit and her parents love but for all children with EB.

    Thank you. Thank you. Thank you.

    On another note....I have thought about your reaction to what you have perceived as negative comments. Everyone is "involved" in your blog in different ways. It is emotional and some people who you think are being negative, I see as being honest and feeling comfortable in putting out there their feelings. EVERYONE who knows about EB is angry. EVERYONE is frustrated. It's a hateful disease and that fact that it happens to children, it's just evil. Not everyone has the spirit you fight to keep so clean and kind. Some are on different journeys but by sticking with a blog that is God centered and caring, maybe it's improving their outlook other places. Ya just don't know.

    Part of the hardest thing in life is dealing with the not good stuff. It's easy to be loving towards the love. It's towards the doubters and the perceived unkindness that is the struggle.

    Tim, it just doesn't matter how people put out their anger towards EB, it matters that they are CARING. Do you know how easy it would be for most of us who are not and have never been affected by EB to just walk away from these blogs we stumbled on? Something in the dedication of the parents and their honesty makes us ALL return.

    In some way, everyone here cares.

    I care. I support you.

    I'm amazed at the Ringold family's dedication.

    Please don't let a frustrated person who has not walked in your shoes hurt you. Please don't give them the satisfaction of opening a wound.

    My one and only child is healthy. I thank God everyday. I also thank God for Bella and her family for showing me blessings.

    THANK YOU Tim and Ang. Thank you for sharing why you made your decision. You have shared the most intimate decisions and it has TAUGHT others how to make decisions.

    My relationship with my own husband is better because of things you shared about keeping your own choices about your relationship.

    Please don't get discouraged. Just see the pain and anger. Whoever wrote that isn't really lashing out at you personally or Bella. They are angry at EB. Me too.

    Follow the love and grace of God. The harder you work, sometimes the more criticism comes up. I'm behind you.

    Keep moving forward. Your healing is healing a community. Focus on the love. Focus on the memory.

    HEY. The rest of you lurkers, come out and write positive and loving comments to Tim and Ang if you are reading. Fill them with our presence and love.

    We don't have to clobber a negative comment, we just have to smother it with love!

  15. Love understanding more by these questions and answers. You have taken on the teacher role directly Tim. You are going back and filling in the holes in our understanding.


    Man Bella is going to be so proud.

  16. Tim,

    Thank you for giving us more insight into EB and the BMT process. It is a really great visualization tool to use as we help raise funds for the research that WILL cure this painful disease. You guys continue to amaze all of us. Keep up the good work!

    Lots of LOVE!

  17. FINALLY de-lurking again...

    Tim and Ange and Ali, thanks for your continued battle against EB. All of us that have witnessed EB in our loved ones first hand can understand why it has left such a lasting impact on your lives and we are eternally GREAT FULL for all you have done and continue to do. The contacts that you have been able to make in the name of EB fundraising are awsome. I see great things on the horizon because of Bella and because of your continued dedication. Thanks for continuing to include us in the journey.

    All EB parents have such tough decisions to make...EVERYDAY...and even if they are forced to make different decisions, they make the ones that they feel are in the best interest of their children at that point in time. Every EB child is different. Each is in a different stage of this horrible disease, and each with a different form of the defect. There are so many variables that need to weigh into the decisions that are made EVERYDAY. No one can second guess what decisions you or any EB parent has made or will make...unfortunately not even when that means they have left their poor EB child behind for adoption. Although it is hard to fathom that it can be a good decision, if that parent was able to come to that decision it is indeed possible that it was the best for the child, because that parent would not have been able to give the care needed. Sometime due to $$ but most times due to the need of being totally selfless and dedicated. Sad but true. Those of us that have been an EB parent or Grandparent know that it is not an easy road to follow. We can only pray that all EB children left behind can find a forever family who can walk that road with them. Through the EB blogs I/we have witnessed such selfless individuals who have stepped up to the plate of EB. Thank you to you all of you parents and adoptive parents.

    Last of all, all of those reading this blog (minus one), certainly can see that you are not looking for "a way to bury your grief", but are doing the things you do in spite of your grief and while working through your grief. Hopefully he/she will keep following your blog and grow.

    Hope you have a fun and restful weekend.
    EB Nana in SD

  18. I have had very limited knowledge of EB until Bella and her family. What I did know though, and correct me if I am wrong,is that children with Eb can appear to be doing well 1 day but can be gone in less than 24 hrs because of some internal event eg internal bleed organ failure. as Tim says this condition does not only effect the skin.Tim and Ang we older parents of healthy children 2nd guess our child raring almost daily,DO NOT 2ND GUESS YOURSELVES!!You did the very best you knew how for your little girl.You will have the pain of her loss forever but you are teaching so many so much.The unkind "blogger" has just given us all an opportunity for GRACE,lets put that to good use.Love and blessings always

  19. Your family is beautiful and seems so upbeat, regardless of the situation.

    I know that you don't know me from Adam. My name is Michelle Palmisano, I'm a senior at Cedar Crest College in Allentown, PA. As my senior project that I need to do in order to graduate I have decided to have a benefit/awareness and educational event about EB. I did a project on it as a senior in high school and am currently revisiting it. Would you or your family be interested in participating in the educational part of my project? I would greatly appreciate a response if you have time.
    Thank you

  20. Decided to "unlurk" and comment. Thanks, Tim, for some inciteful writing last night. Your first three paragraphs on BMT are wonderful.

    As an RN I always wanted to know the "but why" of everything my patients were experiencing. I wanted to understand on a cellular and molecular level. Yes, I undoubtedly annoyed my co-workers but when they wondered about something I would many times be able to explain some of what was going on. Being a preceptor was always an honor. Anyway, the collagen issue in locations other than the skin was a light bulb moment for a split second of insite and a truely physical reaction to what I glimpsed. I totally get what you and Ang are working toward. I also totally understand why you guys participated in the BMT program.

    Peace be with you -

  21. Hey Tim!!! Some really, really great conversations and support in these comments for this community huh?

    I didn't know some of this and have *enjoyed* becoming more informed (NOT that EB is enjoyable) but it's a big scary monster that I understand a bit more thanks to this community.

  22. Bell'a disease was not going to get better or go away. Just the opposite. When would you attempt BMT, after she is comproromised? No you would have a much better chance at success with a child who is relatively healthy. If you were to wait until the EB got worse Bella would have not been a good candidate at all for BMT> She had a perfect match and was as a good candidate. Why wouldn't you try? Maybe anonymous doesn't have the common sense to realize this. Maybe that is the same reason he/she made such an ignorant post!!

  23. Thanks Anonymous for your insensitive post, it gave me an excuse to comment today :)

    It's awesome to see how educated and intelligent the consistent readers of this blog are. Tim you should be proud of yourself for educating us...look at all the wonderful statements basically stating back what you have taught the readers about EB in the past two years.
    Love you guys,