Tuesday, February 8, 2011

February 8, 2011: Back to CA...


Well, we're back in CA.  It was so cold when we were getting on the plane that they had to de-ice the wings... and it was sunny out.  Never had that happen before.  Beautiful day... on the inside!

We got back to CA this afternoon, and honestly, it felt weird, again.

It felt like we left behind Bella a second time somehow.  When we visited the Ronald McDonald House, all our strollers and car seats were still in the atrium.  Just seeing Bella's car seat just sitting there empty, then leaving it, again.  Now, that was Friday, but it really snuck up and hit me again today.  Yeah, it's nice to be back in pleasant temperatures, but that emptiness... just felt it again.

Ali starts her first day of her new pre-school tomorrow, and we again adjust to our 'new normal.'  The grief is actually hardest in this house.  That probably makes sense.  This was Bella's stomping ground.  I remember the day we came home from the NICU with her.  I remember the day we came home from the PICU without her.  While I know that parents all over the world have felt this same feeling, my hope is that  as few of you out there ever have to experience it.  This is why we are so consumed with funding the docs in MN.  All I can say is, as I read your questions, I got so excited to share the answers.  I'm not going to get into all of them tonight.  The idea is that we create a FAQ page here so that as many people have as much information related to the study as possible.  Okay, maybe I'll spill a little.

In short, the protocol is changing for the youngest patients.  EB patients displayed a very different reaction to chemotherapy than any other disease population in the transplant milieu.  There is something else at work; Dr. Tolar speculates that perhaps the Collagen VII gene is expressed somewhere else in the body that we haven't yet studied besides the skin.  See, now that immunologists and transplanters are looking at EB, they are looking through their lens.  The only lens EB has been studied through till now is essentially the dermatology lens.

I need to say that a different way.

What you see is based on the lens (or context in this case) you see through.  A dermatologist won't think to ask the same questions as an immunologist and vice versa.  So, having the team at the U of M thinking about solving EB, well, they ask a completely fresh set of questions about the pathology of EB.  THIS is why the U of M is breaking the new ground (as usual being the pioneers that they are) in EB.

Also, YES, the treatment breakthroughs for EB will generalize out to other diseases.  This is how all adult cancer treatment arose... from the treatment of childhood leukemia.  The immediate areas of impact include autoimmune diseases of the skin, burns, and aging of the skin.

There are also two completely new concepts for treatment that involve using the patient's own stem cells, NOT a donors.  This would dramatically change the danger / success ratio for transplant.  They still want to identify the key stem cell in donor derived transplant and pursue essentially three paths to the finish line simultaneously.  However, without the funds to do it, it is all just ideas on paper.

Alright.  Man, it just makes me feel better just talking about the work up there.  It gives me so much hope.  There is more to share, but not right now.  I remember finding out about the prognosis for a life of EB, and I don't want any couple to feel the feeling I felt that day.  There is hope.  For the countless parents and children till now who had none, I honor you by making sure this possibility we stand in front of doesn't fall to the wayside.

Thank all of you for still sticking with us in this journey to a cure.  There is no turning back now, baby!  NO turning back.

God night.

P.S.  Anyone you know that hasn't read the blog, and you think would want to be part of a cure to a childhood disease?  Invite them to come follow the blog as well!  Thanks!


  1. Glad you are safely home and sad it had some empty feelings.

    AWESOME and fascinating. So wonderful you are sharing all your info. So exciting to hear there is hope.


  2. Thanks for your post, Tim, all of the research ideas sound marvelous! Wish you and the U of M doctors success!

    Sorry that you feel lonely without Bella in the house. She is with you, always, just not to be seen.


  3. i CANNOT wait to hear about Ali's day...
    Seriously. CANNOT wait.
    Can you post in the afternoon when she comes home??

    And, did the tooth fairy come through?

  4. Glad you had a safe trip home...and yes it makes total sense. Have I told you both lately how amazing you are? Well, you are. I can't imagine going back to RMH and seeing Bella's things there. She is so cute in that picture. Its so great that you have so many pictures. You are right, as few people as possible should ever have to feel that way. I love hearing about the research here at the U.

    I know I have told you this in a message, but I wanted to share it here so others could read. We have an inter-professional course working with other academic health students (I am a pharmacy student), and I was asking a couple medical school students if they have heard of EB to tell them Bella's story. They had not only heard of it, but they had been lectured about it in every class for an entire week on it! How awesome! Go U of M!

    Have a great day Ringgolds!

  5. Music therapy was something Dr. Richard Fratianne already had introduced to the burn unit at MetroHealth Medical Center when he had surgery for a brain tumor in 1999.

    But during his recovery and rehabilitation, he came to know music therapy's power for himself.

    While he was grateful to be alive after surgery removed the benign tumor, he also was distressed by the idea that he might never be able to operate on patients again -- performing delicate surgery on burn patients was his life's calling.

    "So I was pretty shaken," said Fratianne, founder of MetroHealth's Comprehensive Burn Care Center. "The surgery had discombobulated my brain, and you can't have that and be a surgeon in a critical-care unit.

    "I was trying to recover, but things weren't working right. I didn't think I'd have the mental skills again that I needed to return to my profession."

    A MetroHealth music therapist paid him a visit in the hospital's brain rehabilitation unit, knowing that he had played piano, by ear, for most of his life. He couldn't do that anymore, either, it seemed, but he began playing a melody slowly, with one finger, then two. "And gradually, sitting at the piano, I started getting some of that feeling back in my brain. What I recognize now . . . was that music involves every part of the human brain. To play music requires rhythm, melody, timing, timbre, harmonics, physical manipulation and responses."

    What was happening, Fratianne said, was that he "was being forced to integrate all parts of brain function. As it came back, I regained my ability to do other things that the rehab therapists were asking me to do."

    Since then, there have been many studies on neuroplasticity -- loosely, the way that the brain rewires itself to go around damaged parts -- and how it is enhanced through the experience of music. This especially has been seen in the brains of stroke patients, he said.

    Fratianne's mental and physical skills came back, and three months after his brain surgery he was able to operate again (the first time, he did so with a backup surgeon next to him, ready to take over.)

    "For a surgeon, that's an incredibly short amount of time to come back, and I can't help but think that it was music therapy that turned the corner for me. "

    Not related to your post but thought you would find this interesting. He is an awesome and highly respected doctor in our hospital. Glad your trip was so invigorating for you! THE CURE WILL BE FOUND!!

    MetroHealth Information Village © 1999-2011 The MetroHealth System
    Send mail to webmaster@metrohealth.org with questions or comments about this web site.

  6. Welcome back to the Tundra, and thanks for the details. I continue to be awed by your and Ang's ability to go through the grief journey with such grace and to use it for something so much bigger than yourselves and your pain. It is very, very clear that we are on the cusp of a miracle.

  7. Sorry it's been a while, but I'm thrilled to hear of all of the great things that will hopefully happen. From a selfish standpoint, do you see the advances that you are discussing affecting EB Simplex also?
    I can't believe Ali lost a tooth already. Katie is so excited to lose her first one. I have a feeling that, if I tell Katie that Bella has lost a tooth, Katie will grab some pliers to pull hers out.
    Thanks you for all of your help in the EB community.

    Rob Hendrickson (and Kristin, Katie and Lizzy too)

  8. De-lurking ;-) to ask some questions

    1) Donor cells seem to be doing their job slowly, to say the least. Results on the receiver's skin aren't all that spectacular. Are doctors SURE this is a correct line to follow?

    2) i have been following Sam Sheridan's blog for quite a while now, and EJ's as well. EJ's life is HELL right now, and Sam went to terrible times too. ... there are several examples of kids who underwent the BMT and their skin/life hasn't improved all that much. The RISK is so high, for such a little improvement...what do doctors think about it?

    3) I read on DEBRA Spain's web that there is a research going on in Spain where they are trying to create artificial skin. What do doctors in UofM think about the other lines of research being done in other parts of the US or the world?

    that's it for now.

    I miss Bella.
    Love and hugs

  9. Wow - great answers (and I know this is just the tip of the iceberg!) Can't wait to hear more. Glad you're back in CA, and praying that the "new normal" will return quickly.

    Love to all of you!
    Laura (for the Team)

  10. I started to think last night after I went to bed and I should have stopped sooner than I did with my questions...my thoughts were not coming clearly. ;) It is so interesting to read your answers...I cant wait for more. I am so glad that they are making changes in the protocol for the youngest patients. It just makes my heart hurt for the "losses"...Bella, Elle and Sarah.

    I am so glad you are back home safely. Sorry it isnt an easy transition back.

    Sending my love to all and the sweetest of kisses for Ali.

    Denise WI