I grew up outside Boston, and have been a patient at Children’s since I was diagnosed at age 2 with CF, in 1985. Once an adult, I transitioned to inpatient treatment at Brigham and Womens Hospital (BWH).
In spring of 2006 I was still “too healthy” for transplant, but I began the process at BWH at my doctor’s urging. BWH was one of the hospitals who transplanted cenocepacia patients like me — along with UPMC, Cleveland Clinic, Duke, and a handful of others. “Better to get started early” was the thinking, “so that when you get really sick, you are ready to be listed.” I began the evaluation as an an outpatient and completed a significant amount of its required tests. I continued to remain fairly stable in my health.
Then, in 2008, an administrative assistant from BWH called me, out of the blue, as I was getting ready for work, and told me they would no longer be able to offer me a spot for transplant because of my cenocepacia. Things spun for a minute — “No, I argued, “You already know I have cenocepacia, we’ve been over this, your program takes cepacia patients.”
There had been a change in policy, the woman said.
I wasn’t sure what to think. I was confused before I could be upset. The woman said I should have received a letter. I hadn’t, although it did arrive in the mail, weeks later — simple and boilerplate — another baffling link in the chain of events. Soon, almost all of the other hospitals began to deny transplant to cenocepacia patients. I grew panicky.
Eventually, I worsened and did my evaluation at UPMC. I was listed on April 24, 2014. Because I would need to reach UPMC in four hours, we set up emergency jet service (which is never a guarantee), and began to wait. When December came, my mother and I moved to Pittsburgh to “wait out the winter.”
Winter passed. May of 2015 found me sick and in the hospital. It made no sense to go home at that point, and surely I would be called soon, was the thinking. We reluctantly signed a new lease on an apartment.
A year passed.
I have now been listed at UPMC for 2 ½ years. It’s taken an incredible emotional and financial toll on my family. I own an apartment in Boston, right around the corner from the hospital, that sits empty while I wait here.
So why did all of this suddenly happen?
A study in the American Journal of Transplantation in 2008 concluded this :
Cooperation between CF treatment and LT centers will hopefully provide new insights into virulence, transmissibility and treatment regimens for this unique and challenging pathogen. More specifically, further studies to identify which specific strains of B. cenocepacia may be more virulent, the mechanisms behind the virulence in such strains and investigations to tease out what host factors might influence progression of the infection in the CF population
should be a priority. Until then, we recommend the careful screening of all CF patients for BCC and excluding from LT those harboring B. cenocepacia, regardless of susceptibility profile.
That conclusion, and recommendation, was made despite the fact that the study was based on a very small group of people with cenocepacia – 7. Of those 7, 3 died of Burkholderia-related complications, 2 died of other transplant complications, and 2 were …alive. The data of these 7 individuals was taken from groupings of people who were transplanted between 1992-2002, a time span that began 16 years prior, when many programs were just beginning to offer transplants, and ended six 6 years prior to the study’s date of publication. The group of non-burkholderia CF patients used as comparison was a study of 59 patients. 9 cultured other forms of BCC, and as mentioned before, 7 harbored cenocepacia.
Being that there are so few studies on cenocepacia and their outcomes, and even fewer at the time the article was written in 2008, the conclusions drawn cast a wide net across the transplant community. I personally was immediately removed from the transplant list at Brigham and Women’s. I was upset, but it would still be a couple of years before the reverberations of this decision were truly felt, when I got much sicker. For some, they were already being felt, and for others they meant the arrow pointing towards death was now certain.
This situation is a serious matter of ethics.
The blanket exclusion of one very small group of people from almost all centers, based soley on the organism that they culture, is ethically wrong. Not only is the data presented about cenocepacia and transplant anecdotal and outdated, but the process of eliminating one very small minority group like this on that outdated evidence is directly in contrast with the typical “case-by-case basis” methodology of evaluating patients at most centers.
Transplant centers certainly must reserve the right to evaluate and accept, or reject, transplant candidates. But it should be on a case by case basis.
Where is the logic in being pointed in the direction of certain death, because the risk of possible death is too great in the other direction? Is that what lies at the core of medicine? Balancing risk, but to a fault?
To those who say “it’s a complicated issue,” I say, “It’s not, its a simple issue, with a complicated story.” At 33, I have end-stage CF that ends with certain, early death. There exists a potentially life-saving surgery available, but because this surgery includes a risk of death, I am being denied opportunity for it by nearly every center in the country. When a man is drowning, does the man on shore say “I can’t save you, we could both drown?” Sometimes he does and he has to live with himself, because that person will certainly drown without help. This is even simpler than that. No surgeon will die if I die, no doctor or caregiver. Is my chance at a year, or two, or five at life, worth less than someone else’s? Who gets to ‘decide’ who uses organs “to the fullest?” I challenge you to ask anyone who has had a transplant if they’d do it all over again even if they knew they would die after only a year, and you will hear a resounding, unanimous, YES.
UPMC Pittsburgh practices real medicine. This transplant team is not in the business of cherry-picking the candidates they deem most likely to survive, in order to improve their statistics. Instead, they accept high-risk, last-resort patients like me, in an attempt to save our lives. I am grateful to my team: my compassionate pulmonologist, Joseph Pilewski, and the brilliant surgeons, Drs. D’Cunha, Shigemura, and Hayanga.