Monday, August 30, 2010

August 30: Day +59

The three loves of my life...

Oh Bella... you're such a mystery.

Last night, we went to bed with a night time call-in report to hear that her pressures were "superb." Awesome! We went to sleep content. Once again, we thought we had the puzzle at least solved for the moment. Bella was hopped up on all-you-can-eat portions of steroids and vasoconstrictors. Pressures would surely hold now, and we could get back to getting all that extra fluid off...

I walked in this morning and looked at the monitor, and just like Groundhog Day, her pressures were down in the toilet... again. Chris, one of Bella's primary day nurses, just looked at me totally crushed. My heart started pacing. How could this BE? Bella was on double the highest dose of norepi she'd ever been on, and almost 3 times as much steroids. After less than one day, Bella just couldn't hold her blood pressure, despite the megadoses.

I stood in rounds with my shoulders slumped. I felt like the spine had been pulled out of me. What was left? When you have low blood pressure (hypotension), it's either the pump (the heart) or the tubes (vessels). You can give fluid to fill the pump, or squeeze the tubes to act a little smaller and hope that either or the combo will increase the internal pressure within the system. The problem is that Bella seems to have leaky tubes, so no matter how how much fluid you pump in or how hard you squeeze, the fluid leaks out, and the pressure drops.

I thought we'd played all our hands. We gave fluid, we gave steroids, we gave pressers. Nothing is holding. I asked the new attending doc today if there was a drug to fix capillary leak, and he told me that if he knew how to do that, he'd be a very rich man.

I told him someone needs to make "fix-a-flat" for capillaries.

Now THAT is a million dollar idea!

Anyhow, we ordered yet another echocardiogram, and the dynamic outflow obstruction has returned. We're hoping it's from the whopping dose of norepi, and we are replacing the norepi with yet another pressor called phenylephrine, which structurally looks like norepi, but behaves like vasopressin. What that means is that it shouldn't get dialyzed by Prisma, and it won't squeeze the heart... just the vessels. We are in the process of switching over right now. It should take about 24-48 hours. The hope is that it will relieve the heart from pumping so hard, which in turn will lower the outflow obstruction. Who knows.

There are two other theories / plays on the table, but I'm too tired to get in to them any further right now. If they keep getting talked about, I'll bring them in to the conversation. Otherwise, I won't worry you. Just pray the phenylephrine for some reason does the trick for us. We really need a miracle. It would be the best birthday present I could get tomorrow just to walk in in the morning and see strong pressures. That's all I'd need (aside from the chocolate cake I really really hope Ali and mommy are making for me... :P)

We will probably revisit the A line tomorrow again as we couldn't get good readings all day and night today despite moving her cuff from both arms to right leg over and over again.

We also had Bella's day +60 biopsies and blister tests done today.

Dr. Tolar lining up their blister machine.

Need sleep. Mentally wiped from the day.

P.S. Found out Ali is a carrier for EB today as well.

Not a banner day.

Bella's fabulous socks of the day...

Ali pinching Bella's tootsies...


  1. You are all doing it so tough today.Storming the Heavens for that miracle.Did Bella have all the CTs you spoke of in an earlier blog? Sorry you have the extra saddness of Ali been a carrier but hey the miracle cure is going to bewell wnd truly here by then.Rest in His Love

  2. I'm sorry you're having such a horrible day. I pray that the doctors will soon be able to figure out how to help Bella. Don't lose your faith. Happy Birthday...early! :) Love, Jennifer

  3. Oh no... what does it mean that Ali is a carrier too? Will it affect her own kids when she's grown up? Is her being a donor for Bella still going to work? I'm sending you lots of prayers and virtual hugs

  4. We are praying for you all tonight and every night!

  5. I am sorry things are so rough at the moment! I am praying that Bella will soon turn that corner (and never look back!) and for renewed strength for you and Angelique. May you experience that the Lord is carrying you!

  6. Just hold on now. It doesn't matter Ali is a carrier - so is you and your wife. Everybody's a carrier of some mutations, but you at least know it. 20 years ago they discovered for the first time that EB is genetic, so the tests will be much better 20 years from now.
    Sorry for the leaky vessels of Bella. I hope they will start healing too. Hold on and heal, little girl!

  7. Silly question and I know that there were some meds that Bella could not take, but have they tried Dopamine?? It worked for us on Prisma?

  8. I know you were expecting Ali to not be a carrier, but she had 50% chances of being a carrier opposed to only 25% of having to 2 healthy genes. BUT it really doesn't matter. She is healthy! And the possibility of her "partner" being also a carrier of EB is quite remote. As the above reader mention, we all have faulty genes! Don't let this affect you, now. You have to be focus on Bella's recovery.

    As for Bella, I am really puzzled by her low blood pressure (as everyone, I assume...). I wish there was something I could do. Please, just let me know if I can do any research for you. I have all means available.

    Praying for Bella and your sweet family.

    And, Happy birthday Tim!

    Carla, from Portugal

  9. I miss Dad so much, too. Why does it get more difficult every year? Off to pray for today's intentions - Happy, Happy Birthday baby brother!


  10. Miss Bella has presented a whopper of a puzzle, hasn't she? Praying hard that someone will find the clue that will solve the mystery. Sending strength, sending hope, sending love and hugs for you all...and especially for dear little Bella, hang in there beautiful baby girl, you are strong, and you will be well! Happy Birthday Tim, praying today is a better day!

  11. "Why is Bella's blood so acidic?" For some reason this thought just popped into my head. Is her salt too low? Are her kidneys not producing renin at all?

  12. I'm praying for that miracle for Bella - praying also that God give each family member what is needed to keep on keeping on! Your readers and prayer support cannot possibly know what it takes to deal with all of Bella's issues right now, but we know the ONE who does! Praying you feel the Holy Spirit surrounding all of you today.

    Praying in Waterford, MI

  13. Praying for strong pressures today. Happy Birthday!

  14. Hi Tim: First of all, Happy, Happy Birthday. I sure hope you have a great day and that beautiful Bella starts to get better numbers and her puzzles get solved.
    Hang in there guys. It will get better. Lots of hugs and prayers for the whole family. Peace and love. Love Leah's Nana

  15. "Fix a flat" for cappilaries - loved it.
    But isn't that Vitamin K? If it helps prevent bruising - and brusing is the leaking of blood from cappilaries - would it maybe help Bella?
    Still praying for Bella and for y'all.
    Lauren, a momma in TX

  16. Happy Birthday, Tim! May your day be filled with improving numbers and news...and maybe a tasty treat or two. Above all, may you be filled with the peace that comes from resting in the power of our Lord.

    A friend in NC

  17. Happy birthday Tim! Hoping your one birthday wish comes true. We are praying for that miracle for Bella. Praying so hard everyday. Sharing the story with as many people as we can so that there are even more prayers being sent to her. Sorry to hear about Ali being a carrier... we know your heart is heavy. We are here for you in any way we can be.
    Love you all,
    The Vanderbooms

  18. I'm so sorry that Bellas BP is acting this way. I pray that the drs are able to figure things out and get her BP back up to normal. Lots of prayers as always.

    Happy Birthday Tim.

    Take Care,
    Amber McLaughlin,CA

  19. Happy Birthday! I'm so sorry things are not going the way that we all want them too. Bella is in the best of hands - Gods. Our thoughts and prayers will continue. At least Ali will know she is a carrier and tests can be done to see if her partner is also one. (Seems like it will be such a long time until then but man does it go by fast!) God Bless you, your family and Bella.

  20. Happy Birthday Tim! I am so sorry about the tough news...I pray today will be a better day and that progressively over time the 'wins' will trump the defeats...and Bella will be rightfully seated in that carseat next to her sister real soon!
    All my love and prayers,

  21. Happy birthday Tim! I am sorry for the rough times lately. Hearing that Ali is a carrier must have been hard to swallow. :( I'm sorry for that. I hope the new med helps Bella's BP quick!

  22. "Yeah, when I was born, I had this rare horrible skin condition, but when I left the hospital, it all went away" and you know what else, my sister is a carrier but never had the disease......."
    All things to be discussed later in life when this rough time is behind you and your family.

    Lord, I ask that you give this family peace and strength. The past 60+ days have been an emotional rollercoaster and they are leaning on you now more that ever. I trust that you will wrap your comforting arms around their hearts. Lord, I ask that your spirit run through Bella's heart and vessels. Please gently touch her with your healing hands. I trust you Lord and I know you are watching over her.

  23. “The Ringgold Experience”

    That which Allows moments of clarity
    That which Nurtures our souls thru empathy
    That which Alleviates our constant self pity
    That which Binds strangers together
    That which Erupts our innate ability to care
    That which Lifts the spirit
    That which Liberates us from ideas of weakness
    That which Attains the true gift of God...

    to Unite and Love one another as we are.

    T.G. VanderGold.

  24. We are praying for lilltle Bella that the medicines will work and that her pressures will get better. We are praying, praying and more praying for a miracle for beautiful Bella. What a beautiful picture of the 3 loves of your life. What a marvelous blessing to have them in your life. Stay strong, keep the fsith and remember we are all here for you. It has been a long journey, we pray that God will give you the comfort, strength and faith to conitnue on and give you rest. Hope that there will be better news today about Baby Bella. Our hearts are saddened and ache for a miracle. Happy Birthday Tim and Many More. We Love all of You and you are in our thoughts and prayers always.

    Myrna & Dwight CA

  25. Hi, guys- long time reader, first time commenter :o)

    I have been praying for Bella for a long while and just wanted to drop a line and some encouragement. I feel badly even comparing my kids (who are for all intents and purposes healthy...) to Bella, but I am constantly claiming Jeremiah 29:11 over them: "For I know the plans I have for you, says the Lord. Plans to PROSPER you and NOT TO HARM you; plans to give you a BRIGHT HOPE and a FUTURE." See, I serve a BIG God who sees the end from the beginning, doesn't make mistakes, and know exactly what His purpose is in all this! Praise God that HE knows! I know you know all this, I just felt like some good old Word of God encouragement was needed!

    Praying in IL,


  26. Lynne, Bob, Jack and BenAugust 31, 2010 at 1:39 PM

    Holding all of you -- and the intention that this new med works for Bella -- in our hearts. Holding the knowledge that underneath the outward roller coaster of mysteries, meds, and setbacks, her body is gently, constantly healing. Holding the vision of you dropping her off for that first day of school, watching her get her diploma, walking her down the aisle. And all the while, she may never, ever know the hearts and lives she has touched.

    Happy, happy birthday and we hope you can feel the love and prayers we send every day. (Tell Ali that Stella says "meow.")

  27. Hi!

    Thank you for writing in Ellys blog. I ALWAYS follow both Bella and Daylon from Sweden. Every EB parents in Sweden are. You are so strong and I will send 1000 of hugs to you from us!

    with love


  28. Praying all the time for sweet Bella!

  29. Praying in Central Arkansas, as I have been from the beginning of this transplant journey! I am fervently praying for Bella and your whole family unit...