Wednesday, October 27, 2010

Day 3Photobucket

I've always wondered if you will ever or have ever considered BMT?  I am sure it has always been in the back of your mind but wondering if you have a specific "condition" on what would make you decide that "now" is the time (i.e. # of hospital visits, # of infections, etc). Also, has your insurance company been beneficial to you or is it a constant nightmare trying to get things covered?

Well, I wish I had a better answer... but the BMT (Bone Marrow Transplant) is a BIG FAT Question Mark for me right now.  I'm not sure that in our situation, that we will ever "know the right time" to make that decision.  But yes... I know exactly what you mean when you ask that.  There is just no easy answer.  Here are my feelings on it... The BMT is a huge, huge risk (especially for Tripp, who is not 100% healthy) and some children don't make it through the actual transplant.  But aside from that... if it were a chance for a CURE- then I think 100%, we would take the chance.  But it's NOT a chance for a cure... as of right now, it's just a chance to live a better life with EB (if you make it through).  But I want to remind you- that all of this BMT in EB stuff is BRAND, spankin' new-- we don't know what's going to happen for these kids down the road.  Maybe it will take years for the "new cells" to do what they are expected to do, and eventually end up being "a cure"... who knows?  BUT... does Tripp have years to wait and see what happens with these other kids who are going through the transplant...?? Maybe... maybe not.  But then again, how much damage do you allow this horrible disease to do? 

SO... the question is:  Do you sit back and wait?  Or do you do something about it?  The part that I'm torn on is... What if we do take him to do the transplant and he doesn't make it through?  Could I selfishly have had 2, 5, 10 more years with him?  Do you put him in a situation where you are 99% sure that he will be on life support, dialysis, organ failure... just to "see" if he will make it through and have a chance at "better skin"  but also a chance of so many other complications that come along with it?  After following the stories of all the kids that have gone through with the transplant... I try and picture "us" in that situation.  And it's a really hard decision to "knowingly" put your child through something worse- for a chance at something better.  I don't know.. I'm rambling, sorry:) 

But when you ask "what would make you decide that 'now' is the time?" I totally get what you're saying.  Yes, I think if we were in a situation where we "knew" he wasn't getting better and we knew that we didn't have much time left with him anyway... I think we may opt to do the transplant.  BUT he also has to meet certain requirements to be able to go through with it... he needs to be somewhat healthy to even have a chance, I would think.  

Oh, and insurance has been excellent!!  So far, (if I jinx myself, I will have a nervous breakdown) they have covered all of Tripp's supplies, from wound care to feeding supplies to tracheostomy supplies and machines.  Before a few months ago, I had not even appiled for Medicaid... so we were paying co-pays for hospital stays, doctor's appointments (which were sometimes as often as 3x week) and presctiptions (which were outrageous)... but I finally got the chance to apply for the Medicaid as Tripp's secondary insurance, and that has helped out tremedously.  They pick up most of the remaining co-pays.  We are blessed.  

However, if there are any volunteers who would like to be my secretary with no pay, feel free to send me a resume'.  Just playing... but really- I feel like I am on the phone with someone all. day. long.  And it's been 18 months since we've been getting supplies, right? (well, about 15 since his feeding tube supplies)... but you would think they could get an order right, huh?  Nope... something is wrong, different, or missing each month.  That'll make-a-momma-crazy.  Then doctor's offices... sheesh- if I don't say Tripp's name and birthdate at least 47 times a week, then it's not week.  Thank God his doctor's that I have to deal with on a daily/weekly basis give me their personal numbers and e-mails.  Because those poor doctor's office secretaries just don't know the stress an EB momma carries around:)

      I would like to know more about BMT.  How it works and what it's supposed to do and if I can get tested somewhere to see if I am a suitable donor. I would also like to know, if you know that, if they are looking into the cord blood treatments?

      A Bone Marrow Transplant or Cord Blood Transplant both replace your unhealthy blood-forming cells with healthy ones. The cells used in these transplants can come from three sources:
          -Bone marrow
          -Peripheral (circulating) blood (also called peripheral blood stem cell or PBSC transplants)
          -Blood collected from an umbilical cord after a baby is born 

      Right now at the University of Minnesota, the Bone Marrow Transplants (BMT) and the Cord Blood Transplants are being done.  Some children have have both marrow and cord blood infused together. Researchers have been studying the use of stem cells to treat children with severe EB- Recessive Dystrophic and Junctional forms.  So far, the results have showed that stem cells from a healthy donor find their way (home) to the skin and secrete the protein that is missing in EB.  For the child, this can mean a substantial reduction in blistering and enhanced healing of the skin.  However, the BMT journey is brutal to these children and their organs and some don't even make it through the transplant.  

      The first thing that would have to be done if we decided on doing the transplant would be a complete workup.  This consists of complete laboratory and diagnostic tests in order to make sure that your child is physically ready for the transplant. The tests will help doctor's to evaluate the child's lung, liver, kidney, and heart function. The workup evaluation takes approximately one week.  We would have to go up to Minnesota and Tripp would spend every day for a week having these tests done... whoo hoo.

The evaluation includes:

An in-depth medical history and physical exam
Blood tests
Blood, urine, and other cultures
Kidney function evaluation
Chest X-rays
  Bone marrow aspiration and biopsy
  Heart evaluation, including an electrocardiogram (ECG or EKG) and an echocardiogram 
A spinal tap (if needed)
Radiation therapy consultation (if needed)
CT or MRI scans (if needed)
Consultation with other medical specialties as needed

      Then you meet with one of the transplant doctors to discuss the workup evaluation results and decide if BMT is the right treatment for your child, and then decide on the best timing for the transplant.      

      To prepare their body for the transplant, the kids are given chemotherapy, sometimes along with radiation therapy. This is called the conditioning regimen. The standard transplant uses a very strong treatment that destroys their diseased cells, and it also destroys their immune system so it will not attack the cells that have been donated. 

      After this "conditioning regimen," the blood-forming cells are given to the child or "infused" through a central line that goes into a vein in their chest. (One of the most common central lines is called a Hickman catheter).  The actual transplant (infusing the cells through the Hickman) takes only about an hour.  These transplanted cells move into the spaces inside their bones where they create new marrow. They grow and make healthy new red blood cells, white blood cells and platelets.  Sometimes it can take several weeks before the transplanted cells produce new blood cells.  During this time the body is unable to produce white blood cells, which fight infection. This means the child is at great risk of infection. 

      Red blood cells carry oxygen to all parts of your body.
      White blood cells help your body fight infection.
      Platelets help control bleeding.
      The actual day of the transplant is called Day Zero. When the transplanted cells begin to grow and make new blood cells, this is called engraftment (and is usually expected around day 30).   The amount of time spent in the hospital following the transplant depends on each child's circumstances.  

      To learn more about Bone Marrow Transplants in EB, visit this website



  1. Thank you for all the information you are giving us. I found myself questioning what day of the week it was. I find myself sad realizing that the week will end before we know it and I have been looking forward to your posts this week for the knowledge I am gaining. Remember I was a kindergarten teacher so I have to have EVERYTHING broken down in small pieces for me. You are doing a great job of that. I wasn't reading baby Bella's blog at the time of her day zero, but I want to go back and read it from that day. Jason couldn't believe the difference in her pics before and after the transplant. I want to read back through and see if Tim says what the swelling was caused from and if that is normal. You will have to check my new facebook album out from my profile. The very first picture is of Julie. Jason told her to show her muscles and the stiffened her arms. We just can't get enough of his videos and sweet face in his pictures.
    Peace, Prayers, and Love,

  2. Thanks, that was my question of the day. Very informative. I read tons of EB blogs so I know the outcomes and risks. But I never knew the deep deep details. Just one more question if you don't mind, does the brutality of the BMT lie in the way the body organs fight the new cells, mainly? Because when I saw the pictures, it looked more like a blood transfusion (I saw them on Bella blog) and I never fully understood, why all the babies undergoing BMT end up on life support and trach before they start getting better, I never understood why is that phase necessary ... And do you think or know whether they are looking into the breast milk or amnio fluids use to fight this?
    Kissies and gentle hugs to baby Tripp !

  3. Hi Courtney: Well you did it again. You added another informative posting. There are so many problems with BMT with EB children I think. The doctors really don't know or understand what is going on inside these children too. EB doesn't just affect their skin, it affects their organs too.
    Will continue the prayers for my favorite little boy in the whole wide world. Love you so much Tripp.
    Love and Peace Love Leah's Nana

  4. Wonderful Wonderful post Courtney! I'm amazed that you are finding the time to research and type up all this information while still having to do constant care with Tripp! Do you ever sleep?? LOL!!

    Keep it up!! BTW - I know you have "your questions" and "About JEB" tabs at the top of the blog - have you considered doing another tab just for these posts? Maybe like "EB Awareness Week Information"? I'll even go out on a limb and name it for you - to go along with the name of the blog "EB"ing a Mommy - what about " 'EB'ing AWARE"...ok, maybe that's a lame play on "Being Aware", but hey, just a thought!! LOL!

  5. Great post! You nailed it right on the head. :)

  6. Thanks for the info Courtney. I'm so new to all of this stuff, literally have just been reading all of the EB blogs over the last 2 months. My husband and I are in the process of adopting our first baby from Uganda which is how I first found out about EB. God has laid EB on my heart for some reason, I'm not sure yet what I'm supposed to do with it, but in the meantime I can at least make myself more knowledgeable. So, I have a few questions, and I'm sorry if they're out of left field, but maybe you can help?
    This might sound weird, but ever since I’ve been reading about you guys and the families at U of M I’ve wondered about the genetic part of EB. Now with you guys you only have one child, but with some of the families at U of M I’ve noticed that they have other children who don’t have EB. So I just wonder how is only one of their children born with EB and the others aren’t? I guess I just think, and again I don’t know how all this stuff works, but if you carry the gene that would cause a child to be born with EB, why aren’t all of your children born with EB, or am I completely missing something and that’s not how it works? And sorry if I’m not asking these questions the right way, but I’m just trying to understand how this works. And one other part of this is, because I’ve noticed that most of the children at U of M that have EB are the youngest of all of their siblings. Once you have a child with EB, if you were to get pregnant again is there a higher chance that child would have EB as well? Does any of this make sense at all, or am I just pulling things out of the sky? I guess this would be the same as having one child that was born with Downs Syndrome and one child that isn’t? Hope my questions make sense?