DECEMBER 21–Our Light

IMG_4124.JPGWord is getting out, so I will speak. After my light-hearted update yesterday, everything  spiraled out of control. After an early morning EEG which showed slowed brain activity, morning rounds showed that she had no responses at all. Her left leg had no blood flow below the knee, contributing to her worsening condition, and they had to remove it. Then a CT scan showed a massive brain bleed, the one thing she had always been terrified of. There was no hope.

The lung allocation system is so broken, friends. If she had received these perfect lungs earlier, all would be different. She should never have ended up on life support with the score she had. All the allocation systems are different, for each organ. Liver is the only one that works—at a certain score, need overrides all regional allocation.

Her doctors were in awe of her—that her tiny body endured so much and kept going.

Yesterday, Sinead experienced a reading of Caitlin that was urgent and stunning and accurate, as all of her readings are. She spoke to the surgeon, on his way into the operating room, and he listened.

The wisest doctors know that this life is mysterious, and that they don’t have all the answers. The care she received at UPMC was loving and extraordinary. We are grateful.

We numbly stumbled through these last weeks, but looking back now, I realize with horror all she had to endure. She was terrified when she was put on ECMO: essentially locked-in, immobile. For the first two days, she could speak, but got so agitated she had to be intubated and sedated and then she was in and out of consciousness.

And before that? The last two and a half years, every day was a wake-up-and-do-it-all-over-again effort to stay healthy enough to survive the transplant surgery: force down so many calories in an effort to maintain her 97 pounds, lengthy breathing treatments, chest PT, exercise.

IMG_3927.JPGAll the while, she tried to “have a life.” She was teaching herself guitar until she could no longer sit up and hold it properly. She worked tirelessly, from afar, to help create and run the Friends of the Prouty Garden; an advocacy group for Boston Children’s Hospital’s world-famous healing garden. The group, despite massive outreach and supportive press, ultimately failed to save the garden. The day the 65-foot Dawn Redwood was cut down was the day she finally crashed and ended up on ECMO.  I know that seeing that tree killed  killed something inside her.

Nick is broken and strong at once. She was so lucky to have such a giving father.

Andrew—-never was anyone so devoted to someone. Caitlin loved him with all her heart.

Jess flew in like the wind last week, like the angel she is, after having her chemo in San Francisco. She had to fly back for her experimental cancer drug yesterday and so was not here for the end. She will join us in Boston tomorrow.

To all of our family and her closest friends: She loved you all so so much!  She was having an argument with me about something once, not long ago, and she said, “You think all this is important but all that really matters is loving people and being kind.”

We are going to go home and figure out what kind of service to have.

She did not want to be buried. She did not want to be cremated. She wanted a mausoleum and we are arranging that. I know she wants a service where everyone can have a good cry and a couple of laughs. We will figure it all out and I guess I will post details here.

Caitlin and I do believe that the soul lives on. I know she is out there, but I will just miss her so much! She is my soul friend. She is my person. I really don’t know how I will live without her. When she was very sick twenty years ago, I remember thinking, “If she dies, I’ll kill myself.” I know I won’t do that, but this gaping hole is never going to close, I know.

We do have weird things happen with “pennies from heaven,” and last night, as they turned off the ECMO machine, I saw there was a penny on it. Birds, always a motif in Caitlin’s life, were doing all kinds of strange things this week. I wanted to read them as signs she would be okay, but I feared they were signs that she was going off into the light.

Sinead sent me this message this morning:


Caitlin Elizabeth O’Hara

July 31, 1983—-December 20, 2016

a beach 2.jpg

Today is apparently the darkest night in 500 years. The solstice and an eclipse. Please look at some form of light today and remember Caitlin and the light she carried within her all her life. Share that light. Please keep Caitlin’s light alive.

She loved Freddie Mercury. And loved his cat vest. And this song, which always breaks my heart.

DECEMBER 16–A Matter of Ethics


Many people rightly wonder why Caitlin couldn’t be transplanted at home, in our world-class medical city, Boston. The answer is disturbing, and points to a flaw in the medical system. It’s a fight she was gearing up to fight, one she plans to continue after transplant. Here is a draft of a letter she was working on in October of this year:

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.

—Caitlin O’Hara