When the U.S. Supreme Court intervened recently in the case of Johnny Paul Penry, a condemned killer said to have the intellect of a 7-year-old, it looked for all the world like the issue of whether it is legal to execute the mentally retarded might finally get a fair hearing.
Fifty percent of the estimated 250,000 people in the United States with Down syndrome suffer from heart defects, the most common being an atrioventricular canal defect, which means that their hearts and lungs must work harder to compensate for a hole in the septum in the “four corners spot” where the atria and ventricles meet.
If detected early, this defect can often be corrected with surgery. However, the diagnosis may be overlooked if early symptoms of heart trouble (lack of energy, poor weight gain, etc.) are mistakenly attributed to Down syndrome itself. Also, the improvement in diagnostic tools is still fairly recent (within the past 25 years), so older people with Down syndrome have frequently missed out on early corrective surgery.
The only hope for those with this defect in its late stage is a heart transplant. Yet that hope has been extended only once in the history of the operation to a person with Down syndrome — and that occurred only after she and a group of supporters gained extensive media attention and spoke directly to then President George Bush.
People with Down syndrome are effectively barred from the possibility of a lifesaving transplant — unofficially in this country, officially in places like Great Britain. The reasons vary depending on which surgeon you ask. The response of those condemned to die is uniformly miserable.
Doctors who eliminate patients with Down syndrome from eligibility for transplant often suggest that such patients wouldn’t be able to keep up with the strict postoperative regimens. This is not a trivial excuse; noncompliance with postoperative care is the third leading cause of transplant failure. Patients must be certain to take their medications on time, report symptoms, make follow-up appointments for checkups and so on.
But to use this as the primary reason for denying people with Down syndrome from consideration for a transplant ignores two facts: First, most people with Down syndrome have parents or caretakers who can help. Second, nonhandicapped infants and children are often given heart transplants, and certainly they’re not expected to be responsible for their own aftercare, just as they are not expected to know how to change their own diapers.
Some doctors offer a less politically correct, but probably more honest, answer: People with Down syndrome are not considered as valuable to society.
One of those less than valuable society members is my 21-year-old brother, Paul. Fortunately, he is one of the lucky 50 percent of people with Down syndrome who were not born with a heart defect. That means, more than likely, he will never have to sit in front of a cardiologist and be told that he’s too stupid to live.
Of course, he’d be smart enough to understand that the doctors were telling him he was not worth saving. And since he works in the dining hall at a hospital, he just might deliver breakfast to the former cocaine addict who was scheduled to receive the heart that could have saved Paul’s life.
One baby in every 660 in the United States is born with the extra chromosome that defines Down syndrome. Half of them have heart defects, and one-third of those will suffer complications (including death) if early surgery is not performed successfully. This makes children with Down syndrome the largest group of children with heart trouble.
When it comes to the point at which a transplant is necessary, one overriding issue drives all of the controversy over allocation: There simply aren’t enough donor organs to go around. As a result, doctors must make daily decisions about who will get a transplant and who will not, who will be placed on a waiting list and who will not. Every “cut” from the list represents an almost certain death sentence, and for every person who is given a transplant, there’s another candidate who is dying while waiting for the next appropriate match. Rationing is fierce, and more than half of those on waiting lists will die before getting a transplant. But why are people with Down syndrome categorically left off the list?
Says Dr. Rosemary Radley-Smith, medical director of Britain’s Harefield Hospital, “My main aim is to make people live and to become independent human beings that will live a fulfilled life. I have grave doubts as to whether … a Down’s sufferer [would] ever be able to lead a totally independent life.
“If asked to make a decision between a normal person and a Down’s patient, [we take] the person who is the most whole.”
Others, such as Dr. Len Leshin, a Texas pediatrician specializing in Down syndrome, believe that colleagues like Radley-Smith are making unjust life-and-death decisions based on personal prejudices. Where does the line get drawn? How many of us would feel comfortable if transplant centers started requiring combined SAT scores of more than 1200 or denying transplants to people with other disabilities, like poor eyesight, dyslexia and club feet?
“Once you get into measuring quality of life, you are one step away from deciding between a doctor’s and a janitor’s lifestyle, or between a famous athlete and an obscure mother of two children,” says Leshin.
Leshin isn’t merely speculating. There have been several cases in which a celebrity has gotten preferential treatment, the most notorious being Hall of Famer Mickey Mantle, who received a liver transplant in 1995. Mantle was an acknowledged alcoholic and needed a new liver for alcoholic cirrhosis. He also had liver cancer, and died two months after his transplant. At the time, people raised quite a stink: Did Mantle get to jump the queue because of his celebrity status? And did he deserve to get a transplant, since his own behaviors and choices had brought on his condition?
There is also David Crosby, Rock and Roll Hall of Famer, who received a liver transplant in 1994 after his own liver was ruined from drug use. Four months after his diagnosis, transplant centers found not one but two organs for 53-year-old Crosby. Just before his first scheduled transplant, doctors discovered a tumor on the donor liver. Two days later, they found another suitable match.
Crosby himself stated, “I should have died a dozen times from overdoses and motorcycle wrecks.” But this recklessness about life didn’t stop doctors from determining that his was worth quickly saving. The average waiting time nationally for a new liver or heart is more than seven months.
The transplant community is divided on the issue of organs for alcoholics. The United Network for Organ Sharing, a nonprofit organization that maintains national transplant waiting lists in cooperation with the Health Resources and Services Administration of the U.S. Department of Health and Human Services, recommends that a patient have six months of sobriety before undergoing a transplant.
Some teams follow this advice, and some don’t. But the fact remains that it is statistically more likely for an alcoholic, drug user, felony prisoner or person who has just attempted suicide to get a major organ transplant than a person with Down syndrome. This remains true even if the Down patient is married, holds a full-time job, attends college and has never touched a dangerous substance.
It is worth noting that liver transplants are more common than heart transplants; in 1999, 4,696 liver transplants were performed, as compared with 2,194 heart transplants. Currently, there are more than 16,000 people on the waiting list for a new liver and more than 4,000 on the waiting list for a new heart. These figures don’t take into account all of the patients who are never placed on a waiting list for a variety of reasons; as mentioned, Down syndrome is one of several possible reasons for exclusion.
(Other reasons for exclusion include medical conditions that might reduce life expectancy or make the transplant risky. For example, most patients who are HIV positive are not considered for an organ transplant. Current drug or alcohol abuse is grounds for exclusion, as is age, though the cutoff for eligibility differs depending on the organ needed and the transplant center considering the case.)
Joel Newman, a spokesman for UNOS, said, “In terms of managing complex aftercare and recognizing symptoms, it’s up to the center to decide if there is someone who can be responsible for this. There’s nothing inherent to the condition [of Down syndrome] to make them poor candidates for transplants.” Newman notes that there are some people with Down syndrome (such as Sandra Jensen, the California woman who received a heart-lung transplant in 1996) who are independent enough to meet these criteria, including those who don’t live with their parents. UNOS believes that each case must be considered individually, and it applauds Jensen for being a pioneer in this respect.
In the British version of the television show “Big Brother,” winner Craig Phillips donated all of his prize money — and his spotlight — to get his friend’s sister, Joanne Harris, to the United States. An 18-year-old girl with Down syndrome, she needs a heart transplant to live. In the U.K., the policy is absolute: No medical center will give a transplant to a patient with Down syndrome.
The U.S. policy is unofficial — there’s no written rule excluding people with disabilities. However, in practice, the statistic speaks for itself: Only one person with Down syndrome has ever been granted a heart transplant in this country. So will Phillips’ efforts make a difference? Even if he gets Harris to the United States, the odds are virtually nil that she will get the operation she needs.
This is a fact that Amy Ostertag knows too well. Her daughter, Madison, was a year-and-a-half old when she died of heart complications. Madison’s only hope would have been a heart transplant, but Ostertag’s daughter was never even placed on a waiting list. “She was a joyful, brave girl who loved life,” says Ostertag. “She was the center of joy for our family.”
What is the value of joy? If we believe that the criteria for organ allocation represent our society’s values, then it’s clear that intelligence is far more important to us than behavior and personality. Be an antisocial menace to society and doctors will try to save your life — but don’t you dare have an extra chromosome.
Maybe that’s easy for me to say. My mother, aunt, grandfather and husband never needed an organ transplant. But Andreas Ramos’ wife, Susan Hattie Steinsapir, did. She received her transplant in January 1996 but died from complications after a few days.
How would Ramos have felt if someone with Down syndrome had received a heart transplant that might have otherwise gone to his wife? “If it was up to me to decide,” he said, “I’d give an organ to someone who contributes to the community rather than to someone who is dependent. The opposite decision would be quite unethical to me.”
But how do you evaluate a person’s contribution to the community? How can the family of someone with Down syndrome accept that their son, daughter, sister or uncle doesn’t have as much to give as Ramos’ wife?
Philip Atkinson, whose 10-year-old daughter, Katie, has Down syndrome, is preparing for her death. She was denied a place on the waiting list for a heart transplant. As far as his daughter’s contribution is concerned, Atkinson says he “wouldn’t have missed the experience [of Katie] for the world. Most people don’t feel this kind of love in their lifetime. I feel everything for her, and it hurts so much it’s unreal.”
Dr. Arthur Caplan, author of “Ethics and Organ Transplants,” sympathizes with Atkinson. “There is no excuse for not transplanting a person with Down syndrome unless the condition itself makes compliance a problem, or if the person has acquired infections which would make immunosuppression fatal. Any other reason not to transplant is discriminatory,” he says. “That said, people with Down syndrome are discriminated against all the time.”
Many parents of children with this condition are outraged by the criteria used to gauge whether their children deserve a spot on the waiting list. (They are judged according to an individual transplant center’s opinions of how independent the patient can become and his or her mental capabilities, ability to self-report symptoms and predicted compliance with medication and therapy. This is true whether the patient is an infant or a young child, even though it is not possible to predict how independent an infant will become, nor his or her intellectual potential.)
Says Galen Witt, who has a 6-year-old daughter with Down syndrome, “The value systems almost never include social, emotional or spiritual dimensions. It is often in these dimensions that people with disabilities like Down syndrome may excel. These are not objectively measurable traits and, therefore, are easy to ignore when [transplant centers] make a decision.”
Atkinson suggests, as has been proposed numerous times before Congress, that we should employ an “opt-out” rather than an “opt-in” card, so that all Americans will be presumed to be potential organ donors unless they object. Such a bill has never been passed, and many agree with Ramos, who calls the idea “unconstitutional.”
Still, an increase in the number of donors is the only permanent — and unbiased — solution to this problem. We can shift the parameters, change the allocation criteria and allow more people on the waiting list, but there still won’t be enough donor organs for everyone who needs them. If enough people make the commitment to becoming possible organ donors, the medical community will have no more excuses for turning its back on anyone, regardless of age, lifestyle or disability. And I’ll never have to imagine the look on my brother’s face when a doctor tells him that his life doesn’t matter enough to save.