Walking the Transplant Tightrope for a Second Chance at Life
A pulmonary arterial hypertension (PAH) diagnosis can have a significant impact on someone’s life. Without treatment, a PAH patient’s health can rapidly decline and leave them facing a difficult future.
Some may be eligible for organ transplant and are placed on a waiting list with enough time to actually receive one. My son Cullen was listed in 2013 and received a lifesaving heart and double-lung transplant a year later. He came frighteningly close to waiting too long and wondered if maybe he should have been listed before becoming critically ill.
Symptoms and a diagnosis
North Carolina native Rebekah Boyd was diagnosed with idiopathic PAH at 17. Now 22, she shared via email how she has been coping and where she stands mentally with the health decisions being made about her future.
Rebekah was a high school senior working her first job when she started experiencing chest pains, shortness of breath, and chronic fatigue. Depressed and concerned about her symptoms, she decided to seek medical care. After several tests and a lot of bloodwork, she was finally diagnosed with PAH. She started treatment immediately.
Rebekah says she is doing OK now, but has concerns.
“I’m having trouble with fluid in my stomach [and] shortness of breath, and I can’t tolerate heat,” she wrote. “Showers are hard, and standing up is even harder. But if I take it slow, I can do a lot of things.”
Since her diagnosis, Rebekah has been evaluated twice by transplant doctors who said she was too healthy to be listed. They based their opinion on her good heart rate and oxygen levels during standard 6-minute walk tests. They recommended that she lose weight, which added to her frustration.
“They wanted me to be like 100–125 pounds. I’ve never in my entire life been that small,” she said.
The transplant waiting game
The question of when to be listed for transplant is common. Patients often struggle to balance themselves on a metaphorical tightrope in which they must be ill enough to warrant the surgery, but healthy enough to survive it. Being too healthy or too sick can make a candidate ineligible.
Rebekah says she struggles to understand how sick she needs to be before doctors will list her, and why she must wait that long.
The United Network for Organ Sharing is under contract with the U.S. federal government and serves as the nation’s transplant system. According to the network’s website, waiting for a transplant isn’t like taking a number and waiting your turn. It’s better described as a giant pool of patients.
Transplant team doctors monitor their candidates and maintain their health information in a database. When a donor’s heart or lungs become available, they rank each candidate based on need and compatibility to determine who best qualifies as a possible recipient.
Sadly, according to the American Lung Association, “Not all donor lungs are suitable for transplantation, and right now, only about 28 percent of donor lungs meet the criteria needed to be used for a transplant.”
Rebekah’s doctors specialize in PAH. They know how fast the disease can advance and put a patient at risk of being disqualified for transplant. She has great trust in them and respects the precautions they take. Yet there is some disagreement between doctors on her various care teams, as her PAH doctors believe she is ready to be listed for transplant, but those on the transplant evaluation team do not.
Rebekah admits she wasn’t ready during her first evaluation, but she believes she should have been put on the list after her second evaluation in 2019.
Now down to 135 pounds, Rebekah thinks she looks sick. She is hopeful that during her next evaluation, likely later this year, the transplant team will decide to list her.
She says she is tired of taking treprostinil, a PAH treatment commonly known by the brand name Remodulin, and she wants to do things with her 12-year-old sister, Callie, that she can’t physically do.
Worthy of another shot at a transplant
Rebekah believes that PAH patients should be placed on the transplant list as soon as they are diagnosed, because being at the bottom of the list would be better than not making it at all. She views the line between being “too healthy” and “too sick” as a threat to her odds of receiving a second chance at life.
No matter what the future holds, Rebekah says her family encourages her to “keep PHighting.” Her dad, David, is her rock, she says, helping her to stay calm when she is scared or nervous. Her mom, Amanda, also remains by her side, assisting with medical care and daily routines that are complicated by PAH.
Callie helps her feel young: “She is my joy when I’m feeling down.”
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, and are intended to spark discussion about issues pertaining to pulmonary hypertension.