I need to premise this post by saying it is very different than most of my blog entries.
It is, intentionally, much more medical than what I usually write about, however my purpose is different. Of course I hope people who love Belle and whom are interested in her journey will read because, quite frankly, it’s too much to fully explain a hundred times. But moreso, I’m writing this post for other families like us; families who may be facing transplant, tpn, and/or both and who are looking for a very unprofessional, non-research based, probably a little biased “momanalysis” of transplant consideration, specifically at Children’s Hospital of Pittsburgh of UPMC. I’m also hoping to include an education and awareness component in this post that might encourage more people to be organ donors. Finally, I must say this is a deeply personal decision. Since this is MY BLOG, I will be sharing MY OPINION. Currently, Joey and I have consulted each other, some of the top/most experienced physicians in the world, some close friends and family, and Jesus, so no other opinions about our choice are needed at this time….K THANKS.
Because Anabelle has total intestinal failure, and is 100% TPN dependent for all of her nutritional needs, she was recommended by her intestinal care team for a transplant evaluation. It just so happens that her treatment center for intestinal care (CHP), is also one of the original and top transplant centers in the world for pediatric intestinal transplants, so we, of course, chose to go there for our evaluation.
(With that said, there are other good centers. If we didn’t have such a great center down the road, Nebraska would have been my second choice. Pitt was very complimentary of Nebraska and I was so impressed how they worked together and how Pitt praised them rather than downing them or trying to be competitive. Miami and Georgetown are a few of the other top centers).
What to Expect
Testing-Your child will go through a lot of testing this week. We were incredibly grateful that Belle’s team looked at her as an individual and took in to consideration all that they already know about her. She had already had many of the tests they needed, so they were strategic about not making her go through more tests than were absolutely necessary in order to gather additional information they needed. The tests we had were as follows:
*Echocardiogram, EKG, TB test, vein access studies (x-ray and a VERY long ultrasound in our case), many blood tests (Belle had labs drawn 3 days in a row) and social and psychiatric evaluations.
*An upper and lower GI study, liver biopsy, pulmonary function test, and CT Scan are other tests that are common for a child being evaluated for intestinal transplant. In Belle’s case, she has already had the upper and lower GI (In the last 16 months and at that hospital), so they didn’t require another one. Her liver function labs were normal so the surgeon explained there wasn’t reason for concern enough to put her at risk with a liver biopsy.
*Consultations with the following teams are also part of the evaluation: Pharmacy- to discuss the extensive medication regimen after transplant, Transplant Nurse Specialist-this was four hours for us of being educated about the history and recent advancements of small bowel transplant and what to expect, risks, statistics, etc., Finance-they will do an extensive study of your insurance benefits and give you an idea of out of pocket expenses associated with transplant, Intestinal Rehab-they will look at tests and give their input to whether intestinal rehab is possible and what other options are out there, Social Work and Psychiatrists-they ask a lot of questions to get an idea of if your family can/will actually comply with the medication and appointment schedule expected after transplant, and to help the patient and family make necessary preparations emotionally, physically, financially, and mentally for a transplant and all it entails.
My Opinion of The Pittsburgh Team
I admit, I’m probably bias. We met with Dr. Rudolph (director of Intestinal Failure and Rehab) before Anabelle was even born to make a plan. He and his incredible team started her on Omegaven at one month of age and essentially saved her from liver damage. Our team is incredible. They are not perfect. They have made mistakes with her, but she is very complex and they are incredibly vigilant in watching her. Even more important in my opinion is that THEY LISTEN TO US! We have a very good relationship and they will sit down with us, sometimes for hours, and hash out what we think is best for Belle. They give their recommendations, but we make the final call and they always support us. So I have been overall pleased with Belle’s care to this point, however, seeing the collaboration between the ICARE team and Transplant team was incredible. Our eyes were opened to how it all works together, how both teams collaborate every step of the way which is what makes Pittsburgh so successful. My heart was filled with gratitude to have this hospital so close and these incredible teams of doctors.
I was also very impressed with all of the various consultations. The nurse who did our transplant teaching has been there since the days TPN babies never got to go home from the hospital and she was absolutely brilliant. I hung on every word she said; the stories of “her kids,” the history of where they were only 30 years ago when those babies never went home, the first transplants, and where they are now having very high success rates. Further, the pharmacist who taught us about meds was incredible, and so sweet. I wanted to give him a hug after his talk that was very clearly meant to scare us…in a good way. All of that to say, I would most certainly recommend Pittsburgh as a great option for an intestinal transplant center, and if you are a family reading this considering, don’t hesitate to reach out to us and we would be happy to answer any questions, that is, from a “biased momanalysis perspective,” just to be clear.
So What About Belle?
It’s difficult to put in to words what my heart experienced this week with Belle. First and foremost, she surprised people, a LOT! They haven’t seen her on 7b since she was 2 months old. Her nurse practitioner cried as soon as she saw her, and many of the nurses were quick to snatch her up and snuggle her, although she wouldn’t slow down for even a second, so she mostly walked them around the unit instead of vice versa. They held her pump and she was on her merry way. She “talked” to everyone and smiled and waved and was her happy self. She tolerated the tests “well” for the most part considering how demanding it was at some points. When Belle woke up from a nap at one point wanting a bottle, her long time doctor happened to be in the room talking to me. He saw me struggling with the darn IV pole which is much less mobile than our home equipment and took her bottle, washed it in the sink (very thoroughly I might add), and filled it up for her. We definitely felt the love from everyone on the unit.
Belle represents a new class of kids in the world of intestinal failure. The path use to be so clear for them, TPN until the liver died then transplant. Even 4-8 years ago liver damage was more prevalent (even though it has now been reversed or at least decreased in many of those kids). Belle’s “class” is already different. Her liver is, seemingly, fine and there is no real reason to think that might not continue indefinitely. So without the need for a liver, there is no urgency for a bowel transplant since people can no live longer term on TPN. Throughout the week, there was a lot of scratching their heads going on, not really sure what to do with her, other than to keep on keeping on as she is doing amazing on TPN and has a great quality of life.
One huge blessing we discovered from the testing was that her has full access in her veins since only 6 veins in the body can tolerate TPN. We had no idea what her veins looked like, so we were very thankful for this news.
Other than liver damage, and loss of access, other reasons to transplant would be recurrent sepsis and severe lack of growth, neither of which are major concerns at this point.
It was agreed upon that Belle doesn’t have any of the markers to risk an immediate (or likely soon) transplant, but also that her intestines don’t function and with treatments available at this time, they aren’t going to start functioning.
So all of that to say, we left the hospital not really knowing what they were going to do. We got the news Friday evening that after meeting as a team, it was decided Belle would be listed as a Status 2 for Intestinal Transplant. Status 2 means she does need a transplant, though we won’t get a call right now. Intestines are “wait time” organs though so she is accruing wait time on the list for down the road. She listed in UNOS (United Network for Organ Sharing), and she can quickly and easily be converted to a status 1 should things start to deteriorate. Since I keep getting the question, “how long,” I will say, as things look now, our prayer would be years, and hopefully many years. Our greater prayer would be in that time, a new treatment would be developed to kick her own organs in to gear. Considering the advancements in even just the last 10-20 years, I truly wouldn’t count it out.
So we keep on keeping on, with a plan for transplant in place if things go south and faith in our hearts that God has this all worked. He hasn’t failed us yet.
Oh and one more thing, April is National Donate Life Month! if you aren’t an organ donor, please consider becoming one. You can sign up to be an organ donor by visiting the website, DONATE LIFE. I will be the first to admit, organ donation never meant much to me before Belle. Shame on me. Hopefully we can shed some light on the value of organ donation. After all, what gift could be more valuable than life itself?
For Hope and Health <3