On Being Too Short to Get On the Ride Jul 8, 2013

By Rick Rader, MD

Sarah has cystic fibrosis, which requires a lung transplant to save her life. But the top of 10-year-old Sarah Murnaghan's head is apparently below the bold line that entitles her to get on the "ride to live."

To those of us who grew up in Brooklyn, a day at Coney Island was a day in Mecca. Besides the hot dogs at Nathan's, riding the Cyclone, playing Skee Ball and grabbing the rings on the carousel, there were the bumper cars. Legal chaos, assault, collisions, confusion, revenge, avoidance and the notion of "watch my back." All the skills, lessons and experiences you needed in preparation for junior high school. It was my favorite ride. And the idea of paying an additional 10 cents for a re-ride (and avoiding having to go and wait at the end of the line) was sheer marketing genius. A ride on the bumper cars was all I needed to justify the three subway trains I had to take to get to Surf Avenue.

There was, of course, one obstacle to overcome; an added bonus to the skills actually acquired on the ride itself. It was getting on the ride in the first place. Right next to the ticket booth was a battered wooden vertical sign that read, "You have to be this tall to get on the ride." The colorful sign depicted a boy dressed like an Indy race driver (cloth helmet and goggles) with a bold black line that demarcated your eligibility to get behind the wheel. While the 1950's and 1960's predated the need to protect the public from the trauma of hot coffee spills and other obvious legal liabilities (like being frightened watching a horror movie) someone must have thought it was important for the pilot of the bumper car to be able to see over the steering wheel and not be strangled by the seat belt. Negotiating the "height chart" was equivalent to getting through customs for entry into a country that promised you everything.

It didn't take us long to realize that two loose Chesterfield cigarettes (liberated from my mother's purse) would encourage the ticket collector to reinterpret where he would "see" the top of your head. We always got the coveted nod and the welcome wave.

If only it were so easy to have the ticket collector who oversees the "ride to live" reconsider the eligibility to receive a needed lung transplant.

The top of Sarah Murnaghan's head is apparently below the bold line that entitles her to get on the ride. In Sarah's situation it's not her verticality that disqualifies her, but her age. Sarah is 10 years old and, at the time I'm writing this editorial, she has only a few weeks to live.

Sarah has cystic fibrosis, which requires a lung transplant to save her life. Cystic fibrosis is caused by a mutation in the gene for protein fibrosis transmembrane conductance regulator (CFTR). This protein regulates the dynamics of sweat, digestive fluids and mucus. It is the mucus which critically affects the lungs by derailing the transport of chloride and sodium leading to thick, viscous and breath-robbing secretions. Most patients with CF have a median predicted age of survival into their mid-30's. Sarah is not alone; there are approximately 30,000 children and adults in the United States with cystic fibrosis. There is no cure.

A pediatric lung would be, of course, ideal. But they are as rare as hen's teeth. An adult lung would work, but it seems you have to be at least 12 years old to be "eligible" to join the "waiting list." Sarah can't wait.

In an article about the case (Politico by Brett Norman and Jason Millman), we learn that "lung transplants are far more common in adults than children, and part of the reason for the different policy for the under 12 group is because there are gaps in medical knowledge about the procedure, which is rare for children. Since 2008, there have been 8,775 lung transplants performed in the country. Only about one percent – or 88 – have been for children under 12. Adults receive the overwhelming majority, more than 8,500, with adolescents accounting for the remainder."

In a plea to have the ruling (for children under the age of 12 to be put on the list for adult lungs) reconsidered to save Sarah's life, Health and Human Services Secretary Kathleen Sebelius refused to order a "set aside" ruling claiming that she, as one individual, shouldn't be making life or death decisions. A plea to a federal judge resulted in Judge Michael Baylson's issuing a temporary restraining order allowing Sarah to be put on the list for an adult lung. Being placed on the list is no guarantee that an appropriate lung will be available and indeed Sarah could die, as do thousands of others "waiting" for organs.

Twelve is an arbitrary age and needs to be revisited. I think the severity of the disease, rather than age, should be a key consideration for organ eligibility. Sebelius has proposed that the national policy on donated lung allocation be reviewed and that is a step in the right direction.

Mildred Solomon, President of the Hastings Center (a respected medical ethics think tank) commented, "I think it's unfortunate that the courts intercede in cases that you would hope would be settled by doctors and families. Transplantation policy is developed after years of deliberation by knowledgeable experts and that there's a lot at stake here that has to do with fidelity and trust in our system."

"Trust" is something that exceptional parents have always wanted from the system and it hasn't always been forthcoming. If the system sets arbitrary heights to get on the ride, then parents will always be looking for ways to get the ticket collector to reconsider exactly where the top of their kid's head is....and will quickly brush their hair upwards to reach that line.

(Editor's Update: On June 12, 2013, Sarah underwent a successful double lung transplant from an adult donor.)

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