In Defense of Transplant, Part III: Timing the Listing

Third in a series. Read part one and part two.

As I wrote last week, transplant is the only treatment for end-stage pulmonary hypertension (PH). You don’t have end-stage PH? I am so glad. No transplant for you. But if you do have end-stage PH or your disease is progressing at all, you may want to think about the possibility of a heart-lung, double-lung, or single-lung transplant.

The first step of considering transplant is getting past the stigma that the procedure is a “last resort” with poor outcomes. The next step is getting on the transplant list. More troublesome than the mortality rates after transplant are the mortality rates on the waiting list.

My doctor would not list me when my care team saw me in June 2015. He told me, “It would be purely academic.” Meaning, I needed a transplant, but not as badly as patients whose health status warranted higher priority. With the existing deficit of donors, it wasn’t worth the trouble to add me to the list knowing I would never get a call offering organs.

In a heart-lung transplant, the lungs follow the heart in priority, so I received lungs based on my status on the heart list, which has a different ranking system. For single- and double-lung transplants, lung allocation score (LAS) determines priority.

The LAS is a scoring system derived from clinical and demographic characteristics. The calculation accounts for urgency and probability of post-transplant survival. The sicker the patient, the higher their score on a scale from 0 to 100. You can see exactly how the United Network of Organ Sharing (UNOS) calculates LAS here.

Before implementation of the LAS in May 2005, UNOS allocated lungs for transplant based on time on the waiting list. This system disadvantaged patients with rapidly or unpredictably progressive diseases. However, the LAS system has not served PH patients listed for lung transplantation.

A review of lung transplantation for pulmonary hypertension patients states that since the development of the LAS, deaths on the waiting list decreased for all disease groups except idiopathic pulmonary arterial hypertension (IPAH). Patients with IPAH were less likely to be transplanted than patients with idiopathic pulmonary fibrosis or cystic fibrosis (CF), and more likely to die on the waiting list than patients with chronic obstructive pulmonary disease or CF.

The predictors for mortality among PH patients are not in line with the current system. Critics believe the LAS too heavily weighs the results of pulmonary function tests (PFTs). Doctors use the results as indicators of disease progression in most pulmonary disease groups, but they aren’t accurate in tracking PH progression. The last time I had a PFT before my transplant, my lung function was in the 70 percent range for forced expiratory volume in 1 second (FEV1). That’s crazy to my friends with CF who tend to be referred for lung transplantation around 30 percent FEV1 based on early studies of predictors of mortality in CF patients. 

In 2010, UNOS and the Organ Procurement and Transplantation Network started granting exceptions to patients with PAH based on severely reduced cardiac index and signs of right heart failure.

This is one of the reasons it is so important that doctors refer PH patients early for transplant evaluation. It may not be as early as doctors think. My doctors certainly did not predict I would need a transplant less than a year after they sent me for evaluation.

Decline in patients with PH can be terribly rapid. Dr. Sean Wyman recently passed away on my birthday, April 13, just a day after going on the lung transplant list.

A transplant team evaluated Sean three times before he died: once in 2006, again in 2012, and most recently this year. After his third workup in March, Sean said in a Facebook post that the program director told him that on paper, Sean was too healthy to receive a lung transplant. Seeing him in person, though, the director said he knew Sean was a lot sicker than the test results suggested. Together they agreed Sean needed a lung transplant to survive, and the director finally got him listed.

My first reaction to Sean’s death was anger. Why hadn’t they listed him sooner, I pointlessly wondered. Another friend with PH is dead because he couldn’t receive a transplant in time. It’s an all-too-regular occurrence.

I thought back to the day I agreed to go on the list. I was hesitant because I did not want a transplant. Naturally, I would have preferred to stick to what I know. It’s not easy to learn about a new chronic illness, and I’m still learning. I’m still earning my new transplant doctors’ trust after 16 years of developing relationships with PH physicians. But I hope that I can give others hope that transplant is worth it. I hope that this series can clarify the decision process for other patients, and push them to address the taboo subject with their doctors while there’s still time.

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Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, Bionews Services, and are intended to spark discussion about issues pertaining to pulmonary hypertension.