Monday, June 21, 2010

Last Night Home...

... and when I say home, I mean the Ronald McDonald House. Tomorrow at 11:00 am, we admit Bella to the hospital, and she will begin her treatment immediately. I met today with our social worker, Janet, who is AWESOME, as well as a representative from a volunteer group called Care Partners. They assign a volunteer to the family for the entire journey who is kind of at our disposal. Laundry needs doing? They're on it. Groceries? No problem. Hang out with the girls while I play soccer? Easy. Support, baby support. It's all about having enough support to counteract the immense increase in stress on the family, both individually, and collectively.

Ali's craft work while I met with Janet

Last, but not least, I met with Dr. Wagner today. He's the head researcher on this trial and quite the guru in curing incurable diseases. He is great. No ego whatsoever. We spent an hour going over all the risks and benefits of the trial for one last time to make sure we are clear about what we are getting ourselves and Bella into, and realistically what can we expect to get out the other side.

How to keep a 4 year old preoccupied...

His tone was more measured than in January regarding promise for the protocol being a possible cure. Today, her referred to it repeatedly as "A first step." That really wasn't that cool to hear the night before we're admitting our daughter, but, 6 months have passed and a lot has happened since then. The hard truth is that out of the 10 transplants that precede us (number 11 gets transplanted in three days, and we are number 12), 2 died, and 1 experienced graft failure, but miraculously grew his/her own white blood cells back. That isn't supposed to happen, so that patient is really lucky to be alive. That leaves 7 other patients, and their results range quite a bit in terms of reduced acuity of the disease. So, when you are on the front lines of a new treatment, anything's possible. We accept this and are frankly grateful that Bella is only 12 months old and participating in this rather than 12 years old. We do know that for the majority of the 7, there has been improvement in their skin. How much ranges the entire gamut.

The master artist at work in her shop...

Let me try to break down succinctly if I can what is going to happen. Here's the deal: Bella is going to receive 8 days of intense chemotherapy to effectively clear out her own bone marrow, and completely eradicate her body of any immune system whatsoever. Then, she'll have a day off, and following that day off, the docs will pull bone marrow out of Ali's hip bones, and through a simple blood transfusion, give it to Bella. Now, the idea is that Ali's marrow will start producing stem cells that will turn into new blood cells, and a new healthy immune system. But more than this, the stem cells go to the skin, where the area of injury lies, and these stem cells start to generate new skin cells as well as blood cells. This is where this trial is the first of its kind. Normally, BMT is done for cancers of the blood, where the injury is in the blood and marrow. Not with Epidermolysis Bullosa. The stem cells somehow "know" that the skin is where they are needed so they home in on the wounds and generate healing previously never seen in the patients.

So, this means that all the areas of injury on Bella's body currently will potentially get an infusion of healthy new skin so that these areas no longer blister and wound as easily in the future. Here's where it gets interesting: What about the areas that have no wounds? Well, they aren't included in the equation. Somehow, the trauma sites send out a signal calling for back-up, so to speak, but the uninjured areas have no reason to send that signal (yet) so the stem cells don't go. However, once a site injures for the first time (anytime after transplant), the stem cells will then get in gear and head to that site to increase healing.

We brainstormed about several different subtleties about this disease, and during the conversation, what struck me was that Dr. Wagner is so far down in the trench... literally into the micoscope, with this particular protocol, that he hasn't yet delved into other more global questions about EB that surface when you theorize about what is next AFTER this. At one point, I suggested what I see as a future evolution in treatment, and he looked at me point blank and said, "I never thought of that!" That was fun. Brainstorming with Dr. John Wagner. Sweet.

How to make sure your kid doesn't take a header off the bed while you pee...

So, tomorrow begins the year-long walk down a very dark tunnel to emerge out the other side with a brighter future for Bella. I say year-long, because realistically that is how long it will take her immune system to be back on line firing on its own cylinders. Until then, we walk a dangerous road, hoping to avoid graft failure (Bella's blood attacks Ali's blood), Graft verus Host Disease (Ali's blood attacks Bella's), and infections as best we can.

This is where that time-honored struggle of control kicks in again. How to do everything possible, fight every battle, take every necessary step, and yet KNOW that it is totally OUT of our control while we weigh decisions that APPEAR to demonstrate that we ARE in control... what a conundrum. I like the phrase, "God is in the results business." We are in the process business. If we just do the best we know how, then turn it over to God, it's really the only way to manage any sanity throughout this. This is when I am grateful for my Faith. See, my faith is of my own volition. It is not anyone else's faith. Others may believe similar things I do, but at the end of the day, I CHOOSE to have faith. No one gives it to me, and no one can force it upon me. Consequently, no one can take it away from me either. It is literally my own creation, and since it is such, I create a faith that empowers me, strengthens me, inspires me, motivates me to get out of bed and face my terrifying fears head on. Without this asset, I wouldn't have the ability to get out of bed in the morning. The fear I am experiencing going into tomorrow is far beyond anything I have experienced in my life.

So, what do I have faith in? What do I believe?

I believe Ang's and my love for each other deepens every day we walk through this together.
I believe that Ali's character deepens every day she is Bella's big sister.
I believe that Bella knows exactly why she is here. She just smiles blissfully at us while we labor over her bandage changes as if to say, "ah, yes, this is why I chose YOU... because I KNEW you would do a good job with me."
I believe that I will be pushed past what I thought I was capable of and thus know myself as enhanced, increased, and upgraded from my previous image of my self.
I believe that Bella will inspire many, many more people, and that this was her plan all along.
I believe that God has the answers, and I believe that I ask him pretty good questions.
I believe that one day, Bella will look back on all this and say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital, it went away."

What do you believe in?


  1. Psalm 56(verse:3)has always been a comfort to me in times of need. I will be sending you positive thoughts and prayers in the coming days. Laura V.

  2. What a beautiful inspiring update. Your family is such an inspiration to us all. Just remember you are not alone in your journey to find the cure. Your EB family is out here behind you 100%. Loads of prayers are coming for your daughters.
    What do I believe in? I believe that God blessed us with Leah (even though it was only for 10 short months) so that we would change for the better. We really don't sweat the little things anymore, and I will do everything I can to spread awareness of this awful, awful EB. Leah's smirky little smile will be in my heart forever. Take care guys and I will be checking in everyday. Love Leah's Nana

  3. you are fantastic. all of you.
    thank you, again, for sharing your story with everyone.
    I am passing on your blog again, adding more prayer warriors for Bella and your family.

  4. You are all being lifted in prayer by our family and the men of TPC.
    Russ, Jen, Cody, Casey, & Celeste

  5. I believe in you, Ang, Ali and Bella, and send love and prayers for you all.
    I believe that God is with Bella, you, Ang, and Ali through this process, and will give you the wisdom and strength to do what you need to do.
    One of my favorite scripture references that I have clung to during tough times is from Jeremiah 29: 11-14. "...For I know well the plans I have in mind for you, for your welfare, not for woe! plans to give you a future
    full of hope. When you call to me, when you go to pray to me, I will listen to you. When you look for me, you will find me. Yes, when you seek me with all your heart, yoy will find me wih you, says the Lord,and I will change your lot." Mom

  6. I believe in you guys and that all the love and strength of the world is with you. I believe, and you help me remember, that we all choose our journey and that it's a blessing just being on the ride.

    Sending love, peace and healing energy.

    (BTW: Your paragraph on faith is the answer to the questions we were asking in the meeting about how to talk about it.)

  7. Holding back the tears (mostly because I JUST put on mascara) and praying my heart out for both Bella and Daylon. Tim, I just can't imagine what you and Ang are feeling today, but I know that both of you have the strength and faith to see you through. I know this may be a weird thing to say, but I'm just so PROUD of all of you. You are so brave and strong and I know you're fighting for Bella, but you're also fighting for Jonah and Tripp and all the other EB kiddos that need a cure so desperately. And for that, I'm so very grateful. May God hold you tightly and give you limitless amounts of his peace. Hugs and Hope, Patrice

  8. You are in my prayers and thoughts today. You are amazing parents. I am so proud of you.


  9. I came here from Jonah's blog and just wanted to make myself known. I admire all of the EB families so much and wonder if I would even be strong enough to deal with everything like you all do. My family will be praying for Bella. Your faith is very inspiring.

    Martha in MO

  10. I believe good things are here and more are on their way. Much much love to your family today.

  11. I've never commented on your blog before, but read it consistently via the link on my daughter Sara's. I don't know if she has shared her dad's long battle with Hodgkin's lymphoma (he's doing very well following his chemotherapy, radiation, and stem cell harvest). I can understand to a certain degree what you've been through and what you're facing, and am so amazed that you have been able to walk this walk with such faith and love. Continue on your unwavering path, and know that there are tons of us out here on your team. God bless Bella, and all of you.

  12. I believe that one day, Bella will look back on all this and say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital, it went away."God works miracles and we need to believe it! Vicki

  13. I don't know you...probably will never meet you...but I want you to know that I AM praying. You are not in this alone...not by a long shot.
    Kansas City, MO

  14. You are in my thoughts and prayers.

  15. I'm from Johah's blog, encouraged from Patrice to come visit. And I'm glad I did. Be brave Miss Bella- it sounds like your new world is just about in reach.

  16. I believe in you guys, I believe in God's amazing love for us all, and I believe Bella's journey is a purpose-filled story of love, strength, and faith.
    Many Blessings,

  17. Tim

    Thank you for your very inspiring updates. We anxiously read them every night,and we are praying for all of you to get the strength that you need in order to get through this difficult journey.

    You are always in our thoughts


    The McGoverns

  18. May God show you his Forever light through this journey.
    God bless you all!

  19. Many good wishes for your gorgeous daughter. Your wonderful family deserves all the best. You are incredibly strong and brave. Your writing is so poignant and deep. Thanks for sharing your remarkable journey and for highlighting all the wonderful aspects of the Ronald McDonald Program.

    I hope Bella does incredibly well with this process.
    Keep sharing your journey.

    Keep your faith.

    Praying in CT.