Wednesday, July 14, 2010

July 14: Day +12

The note written on my bag of goodies from RMH today... we need one...

Well, Daylon and Bella just keep getting closer and closer...

Looks like Bella has V.O.D. as well. Hepatic Veno-Occlusive Disease, also called vascular occlusive disease, turns out to be a relatively common side effect in pediatric stem cell transplantation. It is characterized by swollen abdomen (check), swollen liver (check), weight gain due to fluid retention (check), and jaundice from increased bilirubin (not yet). It is a category of disease that does not necessarily have a defined criteria, i.e. not all 4 have to be present, and up till recently, there has been no success in treating the disease, and if it spreads to other organs, it is 80-90% fatal.

At rounds this morning, the nurse and attending resident mentioned that Bella's abdomen looked swollen, as did her liver. Her eyes looked extra puffy this morning as well. Given those signs, they ordered an ultrasound for her liver which was done around 2. By 4, the attending fellow walked through the door.

I could tell what kind of visit it was within about two steps in the door. So, I just let her explain the whole deal about VOD and the experimental drug that she could go on... like dejavu after following Daylon's journey. He was diagnosed on day +10, we were diagnosed on day +12.
There is a drug that has worked well in clinical trials as treatment and as prophylaxis on VOD in transplant patients called defibrotide. It is not yet FDA approved despite successful studies on the drug in the U.S. going back as far as 1998. It is approved in Europe where it has shown great results as a prophylaxis. In addition to lowering cases of VOD, when given preemptively it also lowers cases of GVHD and increases the effectiveness of G-CSF, a "fertilizer" drug used to stimulate the growth of the newly transplanted stem cells (which Bella is on). These results are from only one study from 2009, but show promise. I already forwarded the research to Dr.s Wagner and Tolar and suggested they take a good look at adding it to their treatment protocol given what is happening with Daylon and Bella.

Anyway, regardless of her bilirubin count, I will be asking in the morning to go on the drug as soon as possible. This way, we try to stay ahead of the progression of this potentially fatal disease.

We knew to expect the unexpected, but we need to KEEP expecting the unexpected. I thought there were only 3 major dangers, GF, GVHD, and risk of infection. I either never heard them talk about VOD, or they never did. Whatever. Either way, here it is, and now it's time to do what we can to manage it.

My heart was breaking and aching as the fellow gave her spiel. It was just like when Bella's Dr. called to explain she had recessive dystrophic EB. I felt the pangs of pressure on my heart as the words started to sound like they were emerging out a long tunnel, feebly finding my ear.

Throw on top of all this that Bella has to go down for surgery tomorrow morning for her new Hickman and her G/J tube, AND it's a dressing change day, and tomorrow is going to be all action. My heart hurts just thinking about tomorrow.

I am sad. I do not know where the road before us leads, but it is going to be more windy and bumpy than I hoped. These are the moments where I am grateful for faith. Why? Faith is NOT hoping or believing in something when there are signs and evidence to support you. That is called expectation, or deduction. Faith, on the other hand, is when you stand in the face of no evidence, and still believe in and stand for an outcome. Despite this setback, I still declare that one day, Bella will look back on all this and say, "Yeah, I was born with this rare condition, but when they brought me home from the hospital, it went away."

Mommy and Ali watching "My Little Pony"...

The good news? My mom ("Nanny") comes into town tonight for 2 weeks. We timed it to fall on days +14 to +28 since this is when the *$#t tends to hit the fan in transplantation. Good timing, huh?

Other good news? The numbers for the day are in: Heart rate, creatinine, BUN and CSA are all now under control and stable, so I'm not going to give those.

WBC: 1,400
ANC: 1,000

watching Ali wiggle in mommy's lap while watching "My Little Pony"...

So, despite the side effects, the primary effect of transplant... Bella growing Ali's cells, seems to be moving right along! For this, I am grateful! It is possible to feel sad and grateful at the same time.

My morning walk to the hospital...

I want to close by thanking you for praying for Bella. She needs your prayers; I am consoled by you helping with this most important part of the equation. Ang is caring for Ali, I am caring for Bella, and your prayers sprinkle us all with the extra fertilizer we all need to emerge from this a healthy and intact family. Thanks again for all the prayers, thoughts, wishes, intentions, comments... ENERGY you send our way. Each one makes a difference.

Good night and God bless you.

I leave you with... Ali... "enjoying" her frosted flakes...


  1. Hang in there. We're all hanging with you.

  2. Sometimes, I check the blog, and I don't really have anything profound to say...just because I don't feel like "words" are enough.
    (and I usually put in some smarty pants comment to cover it up, like, they make My Little Pony videos? SO sorry about that!)

    I've asked everyone I know to wrap Bella (and all of you in prayer), I hope you can have peace of mind tomorrow...

  3. We don't know each other, but I've been following your blog for a few months, and it's now the first website I check after I put my kids to bed each evening. I just want you to know that I've been sending up specific, earnest prayers for Bella's comfort and pain management. During the miserable days before and shortly after the transplant, I found myself begging God that she might find some relief, and I'm already bracing myself for the day she gains consciousness again. Bless you, bless Ang, bless Ali, and may God please, please bless little Bella.

  4. Prayers for your beautiful family and little Bella are coming your way from a stranger in Calfornia...

  5. I've been following your blog daily for a while now and love your perspective as dad. Very refreshing and full of facts and love. You are an awesome daddy with a beautiful family. Prayers from Massachusetts.

  6. Jack and Molly's NanaJuly 14, 2010 at 10:54 PM

    I am sitting here looking at my unmoving fingers on my keyboard. I am speechless (anyone who knows me understands how rare that is). On the bright side, however, is the fact that this new addition to your list of concerns was spotted early AND that there's the possibility of relief. After reading your blog tonight, the first thing that popped into my head was that old adage - if He leads you TO it, He'll lead you THROUGH it. That's my prayer for you all of you. Keep the faith.

  7. I am SO sorry VOD has come Bella's way as well! I am praying for her everyday. God bless and keep you!

  8. My heart has been aching since we talked tonight. I know that consuming, chest crushing feeling during the VOD chat with the docs. I hate that you guys had to experience that. I don't think I've ever felt like we live such a parallel life with anyone until we met you guys. I feel so blessed to have you in our lives and do go through this journey beside you. We've said extra prayers for sweet Bella tonight. We love you guys! Love, Jenn

  9. Take a deep breathe, step back and give him Praise. We thank you Lord for being with Bella, wrapping your embraceful arms around her, Lord we thank you for this day and your will. God is good, all the time, Amen.

  10. "Be not afraid, neither be thou dismayed. For the Lord thy God is with thee, withersoever thou goest." (Joshua 1:9) You are not alone in this. We are all with you and I hope that our prayers & well wishes will being you comfort. Faith sometimes is all we have ( my husband and I feel like this a lot lately, especially when we think of Daylon). You and Ang have been so strong during all of this. Take comfort in each other, your faith and the knowledge that there is a world of us out there praying for Bella every day and cheering her on.

  11. Thinking of you sweet Bella...Jesus loves you! I have faith and believe that God is at her side. Good night sweet Bella...can't wait to check back in tomorrow to see you again.
    Nicole Baca *Christian's mommy from Grandma Joan's*

  12. I wish that there was something to say that would relief your pain a bit... All I can say is that you and your family are beautiful, inspiring and so full of faith! God will not abandon you! This is just a drawback that soon will be overcome.

    Sending my most truthful prayers for sweet Bella, from Portugal.

  13. Saying many prayers for your family.

    Blessings from WA.

  14. With God ALL things are possible.

    I pray for your daughter and your family constantly.

  15. Dear Tim:
    I am so sorry that you guys are going through this right now. You have to know that so many people are praying for your beautiful Bella right now. Please hang in there and I sure hope today goes much, much better. Will be praying this morning during Bella's surgery. God Is Good All The Time. Love you guys. Love Leah's Nana

  16. Please know that we are praying for your sweet Bella's recovery and will especially think of her today during her surgery.

  17. I will be sharing Bella's story with as many more people as I can...God is already carrying you today. Blessings and prayers for Bella...beautiful baby girl, be well.

  18. Love and prayers to all of you - I SO wish Bella the speediest recovery and will be thinking of her today during her surgery ~

  19. Praying for sweet Bella!! Thinking and checking in on you all daily.

    Kara, NY

  20. "Jesus loves me, this I know.
    For the Bible tells me so.
    Little ones to Him belong.
    They are weak, but He is strong."

    Gotta love those last 2 lines, especially during difficult times. Praying for your sweet family.

    A friend in NC

  21. Pryaing for you in The Woodlands, TX.

  22. Good morning! I'm Jonah Williams' aunt, and I just wanted to tell you again how heavy you are on my heart and how often I think of you all. I continue to be inspired by your faith and hope! Praying in Birmingham, Ala.

  23. Thank you for sharing your story with us, I am praying for Bella every night. I believe in miracles. I am living with one, my daughter has E.B. too. Give Bella a very gentle hug from the Norton family.

  24. No no no!!! Bless her sweet little heart. I am praying for you all. Hugs from Louisiana.

  25. I am so heart broken for you guys and this new issue. Just praying even more now!


  26. Wish I had the words to say that would make you feel better. Your family are in my thoughts and prayers ALWAYS! Concentrate on Bella's Healing. and I LOVE your Declaration! :) I believe it will happen!

    \o/ Praise God for Bella's Healing!

  27. Prayers for your beautiful baby girl. You have such a wisdom about this; despite the unknowns and the unexpected. Praying the unexpected is over and the good news keeps coming for Bella. Great news on the WBC; praying for the same good news for her surgery and beyond.

    Your explanation of faith was wonderful as is your love for your family.

  28. Lynne, Bob, Jack and BenJuly 15, 2010 at 9:35 AM

    Wrapping all of you up in prayer. I read the blog. Then I read the comments. And I'm blown away and inspired, every time. In a quiet moment, and even in the midst of what tomorrow will be, I pray you and sweet Bella can feel every particle of the tidal wave of love coming at you from people who've loved you for years. From people you've never met, but who love you too. We're not in the room, but we're all there with you, holding you in our collective arms.

  29. Wow, what a difficult turn....and yet you all remain relentless in your faith! You are all amazing testiments to God. We are showering Bella with prayers for fighting VOD, healing, and new cell growth. All our love!

  30. Lord God, you have called your servants to ventures of which we cannot see the ending, by paths as yet untrodden, through perils unknown. Give us faith to go out with good courage, not knowing where we go, but only that your hand is leading us and your love supporting us, through Jesus Christ our Lord. Amen.

  31. Ramping up the praying here. I asked my FB friends and my friend who is at school to be a pastor to pass on the prayer request for Bella and Daylon (if anyone can get through to God it is an entire campus of pastors to be) :). Thinking of you all tonight.

    Denise (WI)