Monday, September 27, 2010

September 27: Day +87

Mommy showing Bella one of her get well cards...


Bella turned sweet 16 today. Months, that is. Well, today was a good day! We spent the day getting Bella's sedation back on track after the past 48 hours. Bella was off her dialysis circuit for roughly 24 hours, which is the longest she's been off it. They minimized her fluid intake during that time, and pulled back on her sedation. Probem was, they pulled back too far. They were concerned that she would get too snoozy off circuit and this might cause her BP to tank again. Good move, except she ended up with little to no sedation on board by the time she went on the circuit so she was pissed off and feeling the full effects of the withdrawals all through the night and into the morning.

The shelf above our dining room table (there's a lock of Bella's hair up there, too)...

By about 9 am, she CRASHED. She dropped into a deeeeeeep sleep with her heart rate going as low as 75 bpm at one point. However, her blood pressure was generous the entire snooze! We let her sleep all morning so she could catch up on some rest, poor thing. She was shakin' lika a leaf on a tree.


Better to deal with the tremors than lose her blood pressure again, so unfortunately, she just had to grind that run out. We reset her sedation to the levels it was at on Friday. Friday was really a "sweet spot" for her where we didn't have to give any bumps of anything during the day; we had found a happy medium where BP was working fine in and out of sleep, but when she was awake, she wasn't shaking.

We also dropped her pressers to next to nothing! The phenylephrine is down to .1 (minimum dose - we're keeping it "in the line" in case we need it) and the vasopressin is down to .0005. Starting dose on that is usually .0003, so we are just about off and pressures still are generous!

The beautiful sky on my walk to the hospital this morning...

Folks, I can't tell you how it feels like we're in a dream right now. We have been looking at that gal-darn (that's a "Lori-ism" - one of Bella's primary nurses is famous on the unit for great word inventions) monitor with abysmal pressures for months. It is so nice to see these rockin' pressures now, whether Bella's awake or asleep, and especially as we have gotten rid of almost all her BP medication!

We are going to continue to pull the bare minimum of fluid off Bella each day until we get her back to her dry weight. Sam, my buddy the ICU doc, was on tonight and urged me to be patient with how fast we pull off fluid. There is no harm in going slow, but if we pull it too fast, it could dry her out intravascularly (again). So, if it takes 10 days instead of 7 or 8 to get her back down to her dry weight, big deal.

So, where does this leave us?

Well, we're pretty excited about this corner that we have ALL prayed/hoped for for so long.

The next BIG question is...

Avastin or no Avastin?

This is the potential wonder-drug to treat capillary leak syndrome. Having just turned the corner on blood pressure, are we in the clear? What if this drug really can get Bella to pee, THIS WEEK? The drug can cause clotting and bleeding. Bella already has a honkin' clot in her liver.

The wall grows!

Also, the indications from the drug website say don't use this drug within 28 days of having surgery. We're still only 12 days out from Bella's tracheostomy. Hmmm.... is that just a CYA (Cover Your Arse) clause, or a serious warning? It is predominantly used in cancer treatment and patients in all studies who had adverse reactions were over 65, and were given between 8 and 16 doses. Hard to extrapolate from that what one dose could do to a 16 month old. Hard to imagine that one dose could do what it did to the patient in the study i.e. cure him/her of all his/her complications from BMT. Are we supposed to play a part in its future use in pediatric BMT? Please pray today/tonight that God continue to reveal the best path for us. I'm gonna check in with my mind, heart, and gut as well.

There is much to pray about tonight.

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

God night.

Fabulous Socks of the Day! (Ali's favorite pair)


  1. Bella! Your skin looks beautiful!
    I'm so happy for your your wall of notes. Maybe they can go in your memory book, too! Or maybe someone can make you another know, for when you come home.
    Another one of your friends joined Team Bella....we are 13 people strong!!!!

  2. Congratulations to Bella and her family for a third great day! These may feel like baby steps right now, but that's okay - they are steps. Keep on Trucking, little lady!

  3. Yipee! Corner time! Great things are coming little lady keep going! You can do it! I just know you can!

    I hope that Yi-pee will include Pee again some time soon too.

    Baby steps, in amazing socks I might add.

  4. Rooftops are dark and a bit dangerous at this time of night. So I have not ventured out there to call out and celebrate your daughter tonight. Suffice it to say that l so appreciate my feeling fully integrated into your days and nights. Bella's socks are really wonderful - they keep us all focussed on the familiar-glam spectrum, and that's a kind of nutty but healthy place to be as the days pass. Wishing that the paths for the next few days flow smoothly, clearly, plainly towards that bright future that you all deserve so richly.

    Fondly (and a little less loudly - it's a bit slippery on the roof tonight!)


  5. Good evening, sweet Bella! Love your fabulous socks! And am continuing to praise God for even more progress! We're praying for the day when you can tell everyone about this horrible disease you had once. May the Lord bless you and keep you... and your mom, dad and that cute Ali!!

    Love from TX!

  6. Yay for continuing tolerance of the sedation / pressor wean!

  7. Happy Birthday, Bella! I hope you continue to get better and stronger every day! With lots of love, Mari in Brooklyn

  8. Great news all around- my heart is so happy for you Bella Doll- love you baby girl!

    Tim, I did a little research on Avastin is what I found...GOD is Mighty & I pray that he shield you with wisdom, strength, and guide you towards the decision to use or not to use Avastin. Here is what I found...hope this helps:

    Common Side Effects of Avastin
    Avastin has been studied thoroughly in clinical trials, in which a group of people taking the drug have side effects documented and compared to another group not taking the medicine. Because Avastin is designed to be used in combination with chemotherapy (for most uses), these studies usually compared chemotherapy plus Avastin to chemotherapy alone. Therefore, reported side effects could be due to either Avastin or chemotherapy.

    In these studies, the most common side effects seen with Avastin plus chemotherapy that occurred more frequently than side effects of chemotherapy alone included:

    •Scaly, peeling skin -- in up to 84 percent of people
    •High blood pressure (hypertension) -- up to 67 percent
    •Abdominal pain (stomach pain) -- up to 61 percent
    •General weakness -- up to 57 percent
    •Infections -- up to 55 percent
    •Vomiting -- up to 52 percent
    •Upper respiratory tract infection (such as the common cold) -- up to 47 percent
    •Fatigue -- up to 45 percent
    •Loss of appetite -- up to 43 percent.

    Care must be taken when interpreting these numbers. With many of these side effects, most of the cases were likely caused by chemotherapy, not Avastin, although the drug may slightly increase the risk of such side effects. Your healthcare provider is the best source for information about the side effects you can expect with your particular combination of medications.

    Other common side effects of Avastin plus chemotherapy (occurring in 2 to 40 percent of people) included:

    •Hair loss
    •Mouth sores
    •Muscle pain
    •Shortness of breath
    •Indigestion or heartburn
    •Changes in taste
    •Weight loss
    •Low blood pressure (hypotension)
    •Voice changes
    •Dry mouth
    •Skin sores.

    eMedTV serves only as an informational resource. This site does not dispense medical advice or advice of any kind. Site users seeking medical advice about their specific situation should consult with their own physician.

  9. Bellagirl(I've decided that's my nickname for you, as my own Bella I call Belly..)
    Fab socks my dear! I really wish they came in MY size..I'm very jealous. Leave to another Bella to be a complete diva even while sleeping! Again, I must remark how beautiful you are.
    I rushed home from work, loaded up your page, disappointed to see there was no update. So I did some momma type things(facebook, email, potty) and came back and Glory be, there was this update! Thank you for almost reading my mind.
    I'm glad to hear people like "Lori" are making a special place in your heart with "lori-isms". Being a nurse myself, sometimes we don't always hear the good from people, or even thank you's or..for that matter please. I never get to hear enough GOOD things about us nurses, so I"m tickled pink when I hear that your nurses treat you well, or can tell by your stories that they treat you well. That's really important.
    So in closing my bellagirl, you keep up the good work! Your momma and daddy are very strong, for if MY Belly would be in your shoes (erm..or socks)..I think I'd completely fall apart.
    much love from wisconsin.

  10. Remember the fable about the tortoise and the hare? Slow and steady wins the race. Decisions, difficult ones on a daily basis are a fact of life for you and I'm thankful that you have a solid team helping with them. I can't wait for Bella to be painlessly awake so that she can enjoy her beautiful growing wall AND her lovely repertoire of fabulous socks. I agree with Ali - these are MY favorites, too!
    Have a great Tuesday.

  11. One day, Bella will say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital, it went away." And then she will go and find the miracle cure for every other baby born with EB.

  12. I found that I needed a little Bella time tonight. Life seems so wrought with do this, do that, go here, run there, this is due, get that done now, that I needed a moment to slow down. Thanks for giving me that moment, I think I'll take another...

    Rest well little one.


  13. One more thing, I am just courious, if you have the stats about how many BMTs on EB babies have been done and with what success. And also, what is or will be Bella's dry weight when it's reached?
    Good night and hugs to your sweetheart

  14. You are amazing Bella!!! Amazing!!!

  15. I love your socks Bella. I think I'm gonna go to the Jazzy Toes website and get some of those fabulous socks for my daughter Malorie.

    Good job with keeping your BP up today Bella. I'm sad to hear you were in such pain though. You are so strong. You amaze me more and more everyday. You are such an inspiration. Please keep fighting ad hard as you have been so that the docs don't have to give you any other meds.

    Bella, you ROCK!!

    Lots of Love,
    Amber, Craig, Malorie(2 1/2), and Liam(8 mos.)
    The McLaughlin Family
    Moreno Valley,CA

  16. Hi Bella! Happy sweet little 16! I believe that this phase is going away, and if our words can get you all better, Bella you can be sure that i will come here every single day just to give you a word of hope and a big Kiss. Have i already told you that i Have two little girls? Carolina is 6 years old and Carminho is almost 2 years, they are very cute but sometimes they "trash" the house completely and make me mad!lolol. But just thinking about you, about you strength, and faith, makes me realize that our children are the best of the World! And i must thank Good every day for this blessing. So Bella, you get all better so one day you can "trash" you parents and you know what? I think they won´t be mad at all...

    Lots of Love

    Sandra Coelho and
    Family Coelho
    Lisbon, Portugal

  17. Hi Bella,
    happy 16-month birthday!
    Glad to hear you're turning a corner. Hope you'll get better soon.
    Martina, UK

  18. Sweet Bella, so good to read you are on your way up! Just keep going up! Baby steps with amazing socks. And Happy 16-mo birthday!!!

    Tim: I hope you are able to find wisdom in your heart and mind to decide whether to use or not Avastin. When confronted with a decision like this, I am mostly analytic. I usually write on a piece of paper the pros and cons, and the alternatives, and decide what to do base on that. After that, I seek for the approval of my heart, and I usually get it.

    Keeping you guys constantly on my mind and prayers,

    Carla, from Portugal

  19. Happy Birthday Bella!!!!!! Keep on fighting- we're all keeping on praying!

  20. Happy 16 months Miss Bella!! Hang in there honey, still here, still have been on my mind almost constantly the last few days. You are at the corner and we're all here turning it with you. You are an strong, so brave...and I am blessed to be a part of your story! Blessings to you Bella, beautiful baby girl, you have already been healed!

  21. You go girl! Still thinking about you all day and sending all my thoughts and prayers your way little one!

    Love those socks! It's like summer is still here (-:

    Lots of love!!

    Tamar in Boston

  22. Yay Bella! I love that I get to start my comments with yay Bella these days. Makes me smile big. I'm sorry we didnt get to "chat" yesterday, sweet Bella. I had a pretty hard day with my son Gabe, say a few prayers for him if you get a chance. ;0) I am so happy to hear things are going well and I hope that fluid comes off quicker rather than slower. But even if it doesnt, look how far you come! You are doing amazing sweet Bella! You have been a blessing to many and you are blessed to have such a wonderful family!!! Lots of hugs!

    Mary, Lainey, Nick, and Gabe
    Omaha, NE

  23. Sweet Bella and family,

    I am still praying for and rejoicing in your continued recovery. All the best - hugs and smiles and snuggles and cuddles precious girl.

    Lauren in Spring, TX (a momma in TX)

  24. Hi Bella!

    You look great looking at your Mommy! I'm sorry you had a hard time when you were awake, maybe they can get that under control. You are so strong, and such a fighter -

    Happy 16 months!!

    I hope you have a good day today Ms. Bella -

  25. Good Morning Beautiful Bella: Happy 16 months old. Just wait until you turn 16 years old and get a brand new car (hint Daddy). I just know that all you will have to do is say pretty please Daddy, and you will probably get just about anything you want. Something to look forward to, huh Bella.
    Hang in there and continue the fight. You CAN do this, kick some serious EB butt for us all. Love you baby girl. Love and lots of hugs for you. Love Leah's Nana


  27. Hi Bella,
    It is so nice to see you looking at Mommy. I know Mommy can't wait to hold you and cuddle with you and Miss Ali all together! You keep fighting super hard sweet girl and I will keep praying super hard!

    My girls are making up butterflies for your wall! Go Bella Go!!!!

    Tina in NJ

  28. All of you are continually being lifted up in prayer. I am praying for the specific things that are needed today for Bella and I know our gracious God hears. Know that she is being prayed over!
    Love in Christ, Mary Chinn Valparaiso, IN

  29. Keep up the good work Bella! I'm having a hard day today, not feeling well. Let's both rest and heal today, ok? Deal.

  30. Such an answer to prayers that Bella's blood pressure is doing so well! I will continue to pray for healing and strength.

  31. Lord, I raise up to You today a special child, her family and care givers. Please bless them with peace of mind and heart. Continue to work in Bella curing her of all these troubles. Thank You God for all You have done for this family, and for all You will continue to do! In Jesus' name we pray. Amen

    It was great to see those eyes open!
    Much love,

  32. I'm so happy to see Bella improving!

    I have been watching and praying (to whatever power is working with your family ;) ) that you Bella would be strong and hold on while your body healed. i am sure hearing your daddy & mommy's voices read words of encouragement has helped you in your darkest hours to keep reaching back toward them.

    Now it seems you are on your way back to them and I am so happy and relieved, which I am sure is just a tiny bit of the happiness and hope your family is feeling. I also love the pictures of your sister fluttering around near you, waiting for you to heal and be with her. You are so loved!

    I have two beautiful teen daughters and very different issues in raising them. Reading your posts Tim, keeps things in perspective for me in my life and helps me keep a positive attitude in all things and for that I thank you.

    Please continue posting, it is such an encouragement to read about your struggles and faith. I smile when I think my words might be read to Bella and in some way help her, through your voice,to return to you.

    looking forward to better days,
    Love Susan in AR

  33. I’ve been meaning to post on your blog for months now, I wake up and read Bella’s blog every morning with my own daughter snuggled right next to me. Our family has been keeping Bella in our thoughts, and we have been sending positive thoughts your way!

    Tim & Bella - Your strength and perseverance is inspiring, every day you remind me what life is all about.

    I’m so happy to hear that Bella’s pressures have been great! I can’t wait to read that she will be moving back to the RMH, and then back to SoCal. Stay strong Tim, doctors don’t always know everything right off the bat, and sometimes the ones with the worst bedside manner have the best ideas (trust me I’ve worked with my fair share). Stay strong and know that you have support here in Upstate New York!

    Ashley Wells

  34. Bella-just wanted to let you know people love you so very much. you are a beautiful girl with a ton of strength and persistence. Keep it up! we are praying for you and are going to color in the butterfly for you tonight so it can hang on your wall with all the others! keep it up girly girl! lovin the socks too. tres chic! Oh and happy 16 month birthday! one day you will come home and be free from all those silly machines. All the love in the world to you sweetheart!

    hello to ali, angelique, and tim! you guys stay up too. you are all one amazing family!Bless you all!

  35. Happy 16 months sweet-heart! Thinking so of you and praying for you every day.

  36. Love the socks!!! I like that there is a butterfly on the top of them. I haven't been able to check in in the last couple days b/c I don't have the internet at home then I got sick so I wasn't at work. Of course I still prayed for you Bella. When I finally got to check in today I was so so so happy to see the updates from the days I missed. I'm so thankful your blood pressure has been good and continues to be good. I went through-out the day yesterday feeling terrible for myself because I got the flu. I haven't had the flu in 10+ years and I just was miserable. When I went to bed last night I thanked God for giving me the flu. I thanked him for the day and even for all the pleasantries that come with having the flu. You have taught me that Bella. With all the stuff you have gone through - you still rally and amaze your parents and the doctors each day. At 1 point in the day I was like "REALLY . .. REALLY Lindsey you are going to be THIS upset over having the stomach flu for 24 hours." You Bella make me thankful for the having the flu. Keep on keeping on little girl!! Can't wait for another update.

  37. Sweet Bella, happy 16 months.
    Big Kiss

    Sandra, Lisbon, Portugal

  38. Happy Sweet Sixteen Months Bella! Every day just keeps getting brighter.

    Love from Texas!

  39. Happy Sweet Sixteen Months!!!! Praying for you from New York City and giving God and his angels your care and the power for them to be the hands and minds of the doctors treating you! Been following your blog for a long time but had never posted a comment. I look forward to reading every night the updated posts your father so promptly writes to inform us of how your day went. You are a very strong little girl and you have wonderful parents that are by you every step of the way.

    I will keep praying for you sweet Bella and one day you will look back and say "I was born with this rare skin condition and when my parents brought me home from the hospital it had gone away"

  40. T,A,A&B,
    We love and miss you.

  41. Hi there Bella, way to go angel, seeing you "sitting up" and awake makes my heart smile, - because my it tells my nurse brain that your BP is great enough to allow you to be upright
    Keep fighting sweetheart, big hugs to you and your super sister Ali, and Mom and Dad of course :)

    Tim thanks so much for making your book downloadable, I was almost late for work last night because I couldn't stop reading ;)

    Take care now
    Jess - South Africa
    p.s your get well card will be in the post soon ;)

  42. heavno y'all :)

    it's great to see bella's eyes! i've been praying and will continue to pray for all of y'all....that decisions will be guided by the holy spirit and that bella will continue to have good days!

    i agree with ali, i love those fabulous socks too!

    remember, let go - let god and just keep moving :)

    god bless y'all!
    w-s, nc

  43. Lord thank you for the outpouring of blessings you have sprinkled upon our hearts and Bella over the last few days. In our darkest hour, we know you never fail. We know that sometimes, we dont not understand your path but trust its always in the right direction. Thank you again.

    Bella, my 4 year old Grace is going to town on the butterfly request....get ready and onto a lighter note:

    Dad, your gonna have to sing this in your best voice:
    The itsty bitsty pres-sure climbed up the scale today,
    down came the flui-id to wash the meds away,
    I only hope tomorr-ow is as good as today,
    thank you daddy for singing with me today

    Love from Louisiana,

  44. Good Morning Bella and Family,
    Happy 16 month birthday baby girl. Boy you are a fighter and never cease to amaze us all. We are so happy to hear that you were able to get some needed rest and that your bp's are looking fabulous (not as fabulous as your socks). Little lady you are so loved and we hope you can feel our energy, love, positive spirits and prayers each minute of everyday.
    All of your daycare buddies will be coloring those beautiful butterflies so they can send you love and kisses, they miss you and Ali! We can't wait til you and your family can return home back to sunny California. And boy was it extra sunny and hot yesterday. We had a record breaking day here, some places up to 113 degrees...Maybe God decided to have the sun shine extra bright because of joy that you bring to "TEAM BELLA." Your team is so overjoyed to see you improve and to show the Dr's a differnt way... we all know Who is in control of your recovery (-: Sweet baby girl, we are looking forward to tonight's post. I will be focusing my prayers towards a clearer answer to Avastin. Keep fighting sweet Bella and we will keep praying.
    The Davidson's

  45. Beautiful Bella,
    I have not written in a while due to technical problems (I've now discovered I can post comments from explorer, but not from mozilla which is the browser I normally use...) but I have worked it out and now I can write whenever I want. I often have a lot to say. I never did answer the question on why I come here. For me, it started out as in interest in EB (which I knew about, but not on a personal level) and the BMT. But very quickly, way before the BMT actually started, I cared more about Bella and how she was doing day-to-day.
    Everything seemed perfect going into this transplant, so just like everyone else I'm baffled as to why Bella is still in intensive care today. But I don't necessarily think it's a bad thing. Bella is healing, despite the stress that causes her body.
    I've not even had a flicker of doubt that Bella will get through this (that is the bonus of watching it from afar and seeing Bellas fight for wellness continue, for you all there I can imagine it is far from easy to do this!) My heart melts when I see the pictures of sweet Bella in her hospital bed. She looks so small and helpless. But we all know she isn't, Bella's a fighter! This journey may be longer than anyone ever imagined, but it's not over. We will all witness the miracle of Bella, who will become well due to the wonders of science and God.
    I am here every day praying for Bella, and I 'check-in' on her every morning. She is an amazing little girl, and you are an amazing family. Things will get better!
    Becky (scotland)

  46. Happy 16 months sweet Bella. You are beautiful. Happy to hear you had another good day. I agree with one of the previous posts - some day you will be talking about this disease and teaching/helping others. I hope at Christmas you are at home with your wonderful family, eating x-mas cookies and deocroating your tree. You keep fighting and stay strong. Hugs and Kisses. KD Minnesota

  47. Dearest Bella -

    You rock sweet babe in fab socks! Keep turning those corners - no U turns allowed! I continue to pray for you and your family - infact prayer seems to be continuously swirling in my head. God is great and you are proof!

    Amarillo, Texas

  48. Happy Sixteen months!!! Our butterflies are on their way from the UK.
    Praying for Tim and Ang to take the best decision about this new medicine.

  49. I read your update this glad to hear the good news. I just had to read it again tonight. I'm so glad you didn't need surgery. Way to go in showing those doctors who's the boss!!! I can't wait to see your eyes wide opened and beautiful smile. Keep staying strong, sweet Bella.

    My little girl just loves your name. She said such a pretty name for such a pretty girl. Oh, and she really likes your cool socks!!!!

  50. Bella...just a quick note to let you know I am thinking of you today! xoxox

    Denise WI

  51. Beautiful Bella,

    Still here, still praying. You are one amazing little girl. Keep fighting. You will be well, and we'll be watching!

  52. Continue to pray till Bella is home in CA and is free from BTM problems.

    It seems that Bella has grown longer. How much taller is she now? sg-KS

  53. Go Bella, praying that you continue to get better and will soon be able to go out with your sister and enjoy the lovely fall weather.

    The Cooley family in Nebraska

  54. Storming the gates of heaven for Bella tonight from South Carolina!!!

  55. I'm touched! Thank you for enlightening us with such an amazing story. I hope that you and your family are staying strong. God bless you all.
    The Madera Family