Tuesday, July 20, 2010

July 20: Day +18


Bundled Bella and her Butterfly gift from Ali...

Hello world.

What's it like out there? I live in a fish bowl. It feels like the Bill Murray flick, "Groundhog Day," where he keeps waking up and it is the same day again. Well, it's like that but with an evil twist. Each new day brings a new level of stress and frustration. As they say at the Wellness Community, "What fresh new hell is this?"

Before getting into the details of the day, it is important to remember that this is the time in transplant when the sh*t hits the fan, sorry for the slang. Whatever bad things the chemo does, the effects rain down HARD during this phase (days +7 to +28). We thought that since Bella was as healthy as an ox going into this that organ failure wasn't a main concern. My main concern was what was going to happen to Bella's skin during this phase, and the risk of infection over the next YEAR.

So, on the plus side, Bella's skin is behaving much, MUCH better than I anticipated. I honestly expected her numbers to come in well since she is so young and has a young, 8 out of 8 matched sibling donor. I just felt intuitively that her counts weren't going to be the cross she would have to bear on this journey. To be honest, I hoped that she would simply do well and not get snagged at all by any major side effects or risks. I hoped and prayed she'd be a banner baby; a poster child for a successful transplant story for EB... a source of hope. It's not naive to hope my kid will slide through unscathed; other kids have, why not mine?

One important point to make is that on the one hand, usually, the younger you are, the more chemotherapy levels you can survive. On the other hand, most kids who go through BMT usually go through AFTER They have had an unsuccessful run with chemotherapy, so their immune system is already compromised when they begin their protocol. As such, they do not require the same dosage of chemo to "clear out the existing tenants," as I previously alluded to in an earlier post. With Bella, she was 100% healthy, so they had to use A LOT of chemo on her to clear out her existing marrow and immune system. I didn't really think of it that way until talking with Dr. Wagner after the fact. So, the likelihood of major organ damage really goes up in Bella's case. Didn't really think of it that way, did I mention that?

Sooo, on the negative side, her skin is a moot point if her kidneys and liver don't turn around.

Let me tell you how the day went...

To begin, I had a little "come to Jesus" talk with the herd at rounds. Dr. Verneris got to rounds a little early and I told him how frustrating yesterday was due to everyone changing Bella's plan of care without me. So, at the end of rounds, he wanted to address it on behalf of me, but was unsure how to articulate it. Since I knew EXACTLY what I wanted to say, I 'invited' him to let me take over. This is what I said:

"At this point we are looking at 4 teams caring for Bella: ICU, BMT, surgery, and Nephrology. Well, there is a fifth team: ME. I am her daddy, I am her advocate, and I am up on what is going on with her care as you can tell. I was on the floor all day yesterday, and during the one hour I left for lunch, you all got together and changed her plan of care without consulting me. This CANNOT happen again. You are not to make any decisions on her plan of care going forward without consulting ME FIRST. I have a cell phone; you know how to reach me. You get each other on speaker phone when you need to; I expect the same courtesy going forward. Thank you."

They nodded their heads appropriately and that was that. I was so nervous that I was nauseous ; not because I was afraid, but for how stinkin' hard it was to keep my wits together and deliver that address in the same clinical tone that the rest of rounds were discussed in. I don't want to be labelled an emotional basket case... reason and intelligence are paramount up there (on the unit), and these grant you their attention, and when they are supported by passion and love, the message thereby has the power to get through. I looked around the group as I was talking, and the BMT fellow that used to like Ang more than me was clearly moved by my spiel, and for the rest of the day, we had a new level of partnership that I am happy to now have. I really feel like we are on the same team now, and that makes me very happy.

So, here are the numbers...

Good:

WBC: 8.5 (up from 7.5)
ANC: 5.6 (up from 5.2)

Remember we got rid of that fertilizer drug G-CSF on Sunday? It only took one day for Bella's counts to resume climbing! YAY!

Bad:

CSA: 201 (still too low for comfort)
Creatinine: 2.02 (up from 1.53... target is .58: means we're still in "acute renal insufficiency")
BUN: 70 (.100 is okay, but jumped up from 53... indicates more toxins in Bella's blood)
Bilirubin: 2.4 (up from 1.6... now we are in V.O.D. territory... now need a liver ultrasound)
I/O: +760 mL (total net volume in/out at the end of the day... means dialysis failed yesterday)

P.S. Bella has been anuric (hasn't peed) in over two days. Not because her bladder is backed up, but because her kidneys have stopped producing any.

The first plan of the day was to begin continuous dialysis first thing in the morning. Bella needed blood before dialysis today, so we had to wait for that. Then, the machine needed to prime itself, so we had to wait for that. Then, once we got all the planets to align, there were literally 7 medical staff in the room to oversee the 'initiation' of the new machine. Within about 10 minutes, things looked good on Bella's part. It is quite a balancing act to begin continuous dialysis in such a sick kid. It is a fine tuned concert between nursing who is managing blood pressure medicine to keep Bella's blood pressure safe, and nephrology, who manage the removal, filtering, and replacing of basically ALL of Bella's blood. Bella's blood pressure was holding steady and people were excited...

...then the software in the machine froze, and the machine simply stopped dead in its tracks.

No kidding.

7 medical staff in a tiny ICU room, and NOT ONE of them can override or reboot the machine. It has to be scrapped and a new one brought in.

Do over.

This was 4 hours into the process, mind you. It took 4 hours of coordinating and prepping to launch this new machine, and in 12 minutes, we were back to square one.

"Take TWO!"


Number 2 in action...

So... out goes number 5, and in comes (literally) number 2. We set up in record time and the nephrology team leaves. The dialysis nurse, Grace, (whom I LOVE) works hard to get the new machine ready to go within 1 hour. Now, we just have to page Nephrology because they have to be in the room when it launches.

Ten minutes goes by.

Twenty minutes goes by. Another page.

Thirty minutes goes by. No answer.

Right about at this point, I tell the nurse who is training under our main nurse that Bella's mouth needs to be suctioned every hour. The main nurse is out of the room, so the nurse in training gets on it her self. I am seated on the window ledge, and my view of Bella is obstructed by the giant new dialysis machine. I hear her beginning to suction, but have someone else in my face asking me questions while I am trying to type an email. After a few seconds, I notice how loud the suctioning is, so I lean my head around the machine to see why, and what do I see?

I see the "forbidden sucker" that tore up Daylon's mouth 2 weeks ago going in and out of Bella's mouth like a dagger.

A scream leaves my mouth, riding on the sudden exhale that launched out my lungs. Within a second, I am airborne and yelling to STOP STOP STOP!!! WHAT ARE YOU DOING??????

The poor nurse in training is shaking in her crocs, holding "the scalpel" above Bella's face.

For a little background on this, there is a type of suction device called a "neosucker" that produces 'too much' suction power to be used in an EB mouth or nose. It is the suction device that sucked the skin right out of Daylon's mouth 2 weeks ago, and has now been outlawed on the two kids. It had been sitting in a wrapper in the bottom of the bag that held Bella's "Red Robinson," a foley catheter that is long and soft and is safe for oral and nasal suctioning on Bella. The nurse-in-training, or NIT(WIT), took the red robinson off, pulled the other sucker out of the bottom of the bag, pulled it out of its wrapper, stuck it on the hose and started jabbing away through Bella's mouth guard into her mouth.

Now, you are probably wondering, if it was outlawed, why was it so close to the action? Good question. The answer is that it MAY be used if the red robinson has pulled a clot OUT of the mouth or nose that is still stuck. You can then take the neosucker and touch it to the end of the red robinson to help pull out a thick clot. This may sound a little confusing, but when in the room and explained clearly, it makes sense to the nurses.

At this point in the day, I literally (no lie) start banging my head into the walls and window.

I am D-O-N-E.

The room is now filled with nurses and staff, myself, and my mom, all sitting, standing, and SQUIRMING in the most awkward moment of silence you can imagine. I just turn and look at Bella. Frozen. Helpless. Mute. I barely hold back the tears as I start apologizing to her and talking to her. I page respiratory therapy because we have to change the mouth guard anyway; it was soaked from the night nurse NOT suctioning every hour like she was supposed to. We fixed her head gear and checked for bleeding, and thankfully, there was none. Here's the thing however; when you pull Bella's skin off, it doesn't bleed. When her skin shears off, it still leaves a basement membrane that is above any capillaries. So, I tell the NIT and the staff that it looks like she didn't do any damage, however, I can't see in her mouth to truly determine this.

I quietly pull her trainer aside when she returns to the room and ask her if she had explained the suctioning to her NIT at the beginning of shift. She confesses that while she 'mentioned it,' she didn't emphasize it since things were so chaotic when they came on. Then, she made the fatal mistake of trying to defend the fact that with "all their other kiddos..." they don't have so many precautions. Bad move. So, I have to start training this trainer on how to be PRESENT in the room with the actual patient in front of you, NOT all the patients IN YOUR PAST in order to get out of "autopilot mode."

I am done with training.

By 1 pm, a full 6 hours after Grace began setting up, machine number 2 was up and running. Remember how we paged nephrology at noon? Her pager was turned off. We only found this out when SHE CALLED US to ask why it was taking us so long to prep.

I swear to you I am not making any of this up. I wouldn't - I couldn't - imagine things could go this bad. Grace told me this was the longest set up she'd ever done. Grace is not new at this. Another award I don't want us winning.

We move at the speed of mud in the PICU.


Bella bundled up since dialysis cools her blood and lowers her body temp...

By 4pm, it is time for our liver ultrasound. It goes relatively uneventfully (amazing). Then, at 5pm, the BMT fellow, Christine, who I told you about earlier, walks into the room by herself clutching some "paperwork."

Here we go.

The only other time Christine has entered the room that late in the day was the last time we had an ultrasound done to talk about V.O.D. Remember, we needed a bilirubin of more than 2 to qualify for the drug therapy? Well, the other component they wanted to wait for was to see if the liver got so swollen that it started pushing blood in reverse through its own vessels. Well, the ultrasound saw that today.

The paperwork? The informed consent to participate in (yet another) clinical trial. So, we go over it, and she leaves it with me. Angelique and I read it, talk about it, sign it. The defibrotide begins tomorrow. The benefit? It may safe Bella's liver. the risk? It is an anti-coagulant. No problem, but the continuous dialysis is as well. Two anti-coagulants running simultaneously present a possibly life threatening situation. Bella could essentially stroke from trying to save her liver and kidneys.

I told you, EB is just one big case of choosing between crummy and awful. If she goes into renal failure, she could die. If her V.O.D. spreads to a second or multi-organ, it's 80-90% fatal. To not treat her for fear of a stroke and watch her die of one of these complications, well, that doesn't sound like much fun. She HAS to be on dialysis, and if we let V.O.D. progress, it'll most likely kill her, so we feel, as do all her BMT docs, including Tolar and Wagner, that she NEEDS to be on defibrotide at the same time. We've run out of options. We've been trying to avoid continuous dialysis and defibrotide, but we lost.

So, tomorrow, she begins her first dose. It is a 21 day treatment with no immediate results to hang our hats on. It's more like, "If she's still alive, it's working."

Before we left at 8pm tonight, our night nurse explained that machine number 2 was beginning to fail, and that it won't make it through the night.

Onto machine number 3 in the morning I guess.

You all know that I am a man of deep faith and passion. You have read me work through these issues and arrive at a positive end. After yet another grisly report, you might be left with, "Why are things going so abysmally bad throughout this journey for us?" Only God has the answers, and I frankly don't need them. Yes, things are unbelievably stressful right now, but we were told this was how it was going to go. The details of what pitfalls we might find ourselves in were unclear, but it was LAID out in front of us that this was no easy task, and that peril lurked (and still does in all sorts of forms not yet discussed) on all sides.

Don't get me wrong. (pardon my upcoming French...) It TOTALLY SUCKS that this is where Bella is. We knew this was going to probably be a bad period, but again, for different reasons.

Here's also what I know. I have personally treated patients in FAAAAAR worse shape, who were far closer to death than Bella, and met them after they made a complete recovery. This too shall pass. I have no evidence that this is the case at this point, and so I am relieved that I can fall asleep tonight lying in the care of my faith.

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."

I believe this. I believe this because I choose to believe this. Belief and faith are a choice. More than that, they are a gift. They are a gift you can give yourself, because only you get to choose what you believe in and what you have faith in. If you have no faith, you chose that. If you do not believe in anything, you chose that. If you believe in God, you chose that. If you believe in no God, you chose that. No one chose it for you. Your heart alone is the final vote in accepting what comes from without, and from within. My heart POUNDS for Bella. My heart POUNDS at the thought of dancing with her on her Wedding day. All that I am, all that I say, all that I do, all that I write POUNDS in dedication to Bella, and in dogged alignment with the vision I will once more recreate:

One day, Bella will look back and say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital it went away."

Life is good.

See you tomorrow.


Home being goofy with Ali... who took this shot...


Ali scolding nanny telling her she was talking to me... SASSY!

Ali called out the following commands:


"Cheese!"


"Sausage!"


"Pepperoni!"


"Wine bottle princesses!"

Awesome. I love you, Ali.

33 comments:

  1. First, thanks for those last pictures of Ali.
    I know I say it all the time, but she is fantastic :) What a special little soul!

    And, I copied your FB status onto mine...more prayers coming.
    Stay strong, friend. We are all here for you...

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  2. Every fiber of my being is locked in a prayer for Bella...this prayer will last all night, through tomorrow, and all the tomorrows, for as long as you and Bella need it. And though our hearts ache for what your sweet baby has had to endure, underneath the ache is hope, and a peace that only faith can bring. Dear sweet Bella, beautiful baby girl...will be well. Blessings Tim, for all of you.

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  3. Jack and Molly's NanaJuly 20, 2010 at 11:21 PM

    Keep the faith.

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  4. I heard about your family through my cousin, Sara. I've been praying for all of you...for peace, joy and HEALING. We are holding your family close to our hearts ~ God bless you!

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  5. I will continue to plead with God for little Bella! May He also give you the strength and inner peace to carry on. God bless!!

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  6. Praying for your precious fighting princess!
    (friend of Sara)

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  7. Our God is a loving God...HE is in control...and HE is the King of miracles...I BELIEVE and I have FAITH that he is watching over Bella.

    Our Father in Heaven, we come before you and give thanks to you for your unconditional love, mercy and grace. We lift Bella up to you in prayer and we ask with humble hearts that you please protect/heal Bella's kidney and liver. I pray that you please protect her with the blood of Jesus...please dear Lord, let tomorrows events go seamless and peacefully. Please let the nurses/doctors work with clear and sharp minds. I pray and ask that the machines, medicines and all lines, run smoothly and in unison. Please put your hands upon this experimental drug and let it lead Bella to a full healthy recovery. I thank you so much for giving Bella such an amazing Daddy, Mommy and sister. Thank you for watching over her and for blessing her WBC/ANC increase counts...this is great news. Faith is something that we cannot see, but it is something that we believe and are active in daily. Please see our hearts and the hearts of Bella's family...we all come together in prayer before you and ask that you perfom a miracle with a full recovery. Thank you for your love. We love you Bells....in Jesus name...AMEN

    xoxo
    nicole

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  8. Tim,

    I love the butterfly gift from Ali. Your girls have definite style! I posted a little bit about Bella this morning on my own blog to call attention to your amazing little girl. So.... more prayers and thoughts coming your way from Rochester, NY and Gilroy, CA.
    With lots of love and hope and faith,
    LaurenBev

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  9. Oh Tim...again I have no words for you. But I know that words aren't really what you want from us. So please know that I am praying on behalf of you. I am praying when you don't have the strength to pray. What a wonderful thing, this "community of believers"...
    You inspire me. I know you don't feel inspiring or really even WANT to be the one to inspire...but despite all the humaness around you, God is shining through you. I can see Him brightly.
    Kathryn
    KC, MO

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  10. I'm praying for Bella's complete recovery from the BMT and all the horrible side effects. Praying her liver and kidneys resume their functionality. I'm thanking God Bella has you to advocate on her behalf.

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  11. God bless you and your precious family. I'm not sure how you are even functioning at this point. Well, actually I do know...Love is such a powerful force and through its power we are able to do and endure. Fervent prayers that today is better than yesterday and that all the new procedures bring the desired results. I'm inspired by your strength and faith; I'm amused by your sense of humor that comes through even in the midst of such trying times. Praying that today will be calmer than yesterday.

    Susan
    A friend in NC

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  12. hi Tim. You all are in our thoughts and prayers. You are doing an amazing job. Hopefully you can squeeze in a little guitar for you and Bella. Take care. Russ F.

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  13. I'm so glad that you are speaking for Bella, who can't speak for herself in this situation! As parents, we HAVE to advocate for our children. We can't be sure anyone else will. Yea for yelling when needed!

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  14. You are so amazing! I can't even want to image the stress you guys are under. I hate that things are so so bad right now.

    Still praying, praying and praying more.....

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  15. You are doing the most important job of any in that hospital right now - advocating for Bella. The fact that you are able to do it calmly and respectfully, is amazing. I hope that Bella turns the corner today and the machines work smoothly.

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  16. Tim, Angelique, Ali and Bella,
    First of all I want you to know that we believe and stand beside you in how you are speaking up in the hospital setting. You must be the voice and do not feel a bit wrong in doing so. We understand how hard it is to keep the control over your overwhelming emotions to put on the "face" of reason while speaking with medical staff. Do not hold back at any time when you feel you need to speak. I know Tim, that you are very good at this, but I can imagine that it gets tiring and you get disgusted that you have to and cannot just be present for your child without all of the pressure of being constantly on guard. In the big scheme of things the wonderful personality that you were given will be most helpful in this case, and you will look back and be glad you used it! We are truly sorry that things are so stressful for all of you right now. Our good thoughts and good energy are being sent to you daily along with strong prayers.
    BIG hugs,
    Michael and Robin Setto

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  17. Hello, I heard about your beautiful family and gorgeous Bella from Sara Cooper. Sara and I were teachers together when I was teaching special education at Richman Elementary. My heart is full of sorrow for your family's difficult battle right now! But I am going to be thinking about Bella and praying for her today. I want to watch her get well and get bigger here on this blog. She is a very beautiful girl, as is Ali!!! I also pray that you, Mom and Dad, find peace from the constant stress and feeling of unknown! May you both have a momemt of feeling God's touch and ultimate quiet in your mind at some point today.
    Sincerely,
    Le Anne

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  18. Major prayers are being sent your way from Auburn! You are such an example to me in my walk the Lord because you are so strong. Thank you so much for that!
    War Eagle,
    Katie Werner

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  19. I read your Facebook post before I went to bed last night and drifted off to sleep with an image of Bella being held by Jesus. God IS there and I pray that this "fresh hell" too shall pass. Prayers from Los Angeles...

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  20. Wow. All this bumbling by the medical community. A surprise? NOT. Grrrr. I am an RN and see this kind of stuff all the time, unfortunately. Seriously, when my daughter is hospitalized, I cannot leave her side for one minute. I do not joke here. And this is at Children's in Boston. It goes on everywhere, which is often surprising to parents and life threatening to children.
    Great things happen too, children do survive and go on to live miraculous lives and wonderful things are done. But the day to day in there is something else.
    It's excruciating enough to watch your child suffer but to have to police the crew every minute of 24 hours is oftentimes asking too much. But there is no choice here.

    Carry on Daddy Bear. You roar!!!!

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  21. Lynne, Bob, Jack and BenJuly 21, 2010 at 10:39 AM

    This from my mom: "Our church responded to my request for prayers as if Bella were one of their own. Several of the congregants came up to me after the service and wanted to talk about it. And one woman, an elderly retired RN, recalled that I had made a similar prayer request last fall, and she had been wondering ever since about the health of this little girl and her family. I hope it may be comforting for this family to know how many people are pulling for them."

    To your faith, we add our own. One day, Bella will look back and say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital it went away." One day, you will walk her to her first day of school, watch her graduate, and dance with her at her wedding. We hold these intentions -- and all of you -- in our hearts. Love you guys.

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  22. I am praying for your sweet Bella.

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  23. Ali did the same thing to me when I was starting to talk to Ang, she is too funny!
    I had a serene moment 13 months ago when I first read about the BMT trial, I think I've told you this before. I KNEW in that moment that Ali was going to save Bella. I didn't know when or at what age, but I knew this was the future. In that moment it was so clear that one day Bella would be free of EB. I hold that feeling with me everyday, because it will be so. The road to get there has turned out harder than anyone of us could have imagined. It is not straight and flat, unfortunately, but you and Ang are fantastic drivers on this road and Bella and Ali will be stronger due to the bumps. Hold your own vision, and it will be so. "The gang" is here for you when you need co-drivers. We love you all so much.
    JT

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  24. I'm sorry that things have been so rough for Bella. I often feel like reading your blog is recaping my own feelings about the day. I've said it before, and I'll say it again: I'm glad we're in this together. We're just two doors down if you need to talk...or rant! Love, Jenn

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  25. Thinking of you and praying for you Bella Doll....xoxo
    Nicole B

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  26. My heart pounds for your family as well. My family will be praying for your little blessings recovery. May god give you strength and peace and especially healing!!!

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  27. Tim I am constantly amazed by you and so proud to call you our friend. I LOVE that you stand up to anyone for what you know in your heart is right. I know Bella appreciates everything her daddy is doing to protect her best interest. We are praying for you now and always! Give bigs hugs to Ali and Ang.
    xoxox
    The Vanderbooms

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  28. Did you notice the "heart" shape above Ali and Angelique in the pictures above? See, love is all around you. Terri

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  29. Lord, thank you for entrusting the care of YOUR Bella to the best dad (and mom) on the planet!
    Lord, please fill his soul with courage, faith and endurance through this difficult time.
    Lord, please wrap your arms of comfort around tiny Bella as she receives her treatment.
    Holy Spirit of Healing, flow through her little body, helping her to a speedy recovery.
    and last, Lord, please guide and lead Bella's team of nursers and doctors in the right direction. And yes, I know goof-ups are inevitable, but please dont let them negatively affect little Bella. I trust in your Lord. I, Bellas dad, and Bella can do all things through Christ who stregnthens us.

    Anonymous in Louisiana (friend of Tripp Roth)

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  30. I have been praying for Bella all day--for a recovery so swift & complete that only God can take the credit.

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  31. Heavenly Father, I come before you weeping for a little child who desperately needs your healing touch, protection, and comfort. I pray that You will keep her strong, help her fight, and see her through these uncertain days and into days of laughter, joy, peace and health. Be with her family, provide them respite from worry, and let them be comforted by not only the prayers of thousands but by the presence of Your light and love in their lives.
    In Jesus name, Amen

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  32. Thinking of your family and praying for your continued faith. I am singing this song for Bella
    "I am sending you light to heal you to hold you I am sending you light to hold you in love." Vicki

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  33. Tim,
    You all are an inspiration to us all, especially miss Bella. God is working and doing amazing things, and will continue. We had some health issues with our daughter (though significantly less severe than yours), but feel and hear your heart. God is carrying you, using you, and ultimately doing the most incredible work, and behind the scenes. He wants you to let Him fill you up, as you've given so much and unconditionally, and let Him continue to lead you.

    You all are so covered in prayer. Sending hugs and peace and strength your way.

    In Him,
    Tiffany Modica

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