Patients Who Took Growth Hormone Live in Terror of Deadly Disease

For 15 years, Timothy Roman was part of a medical experiment to combat dwarfism.

Roman, of Encino, was among 6,500 dwarfs in the United States whose parents fought to have them injected with a growth hormone derived from the pituitary glands of cadavers.

The treatment was hailed as a major breakthrough in 1958. Today, it is considered a disaster.

In 1985, 22 years after the project began, scientists discovered that some batches of the growth hormone had been contaminated by a rare virus-like agent that causes Creutzfeldt-Jakob disease, a malady that attacks the central nervous system and has been described as an accelerated form of Alzheimer’s disease.

Victims of both disorders become disoriented, confused and eventually lapse into a coma. But CJD patients die much quicker than Alzheimer’s victims, generally within a year of the symptoms’ appearance.

Like most of the treated children, Roman does not know if he has the disease. But, in a bizarre twist, Roman’s attorney says CJD or the growth hormone associated with it may have driven his client to kill his mother. Actress Susan Cabot was found beaten to death in her home Dec. 10, 1986, and her son was charged with the murder.

Scientists say there is no medical evidence to suggest that the disease causes violent behavior. That issue may be left for jurors to sort out.

What is certain, at this point, is the anxiety gripping both those who participated in the program and their families. Hormone recipients can only wait to see if they have been contaminated.

“You learn to live with any odds,” said Diane Coussan, whose son, Regan, took the hormone for a year before distribution was suspended in 1985. “It’s the uncertainty that will drive you crazy.”

Four Deaths Since 1985

Four former hormone recipients have died of CJD since 1985. A fifth died of other causes, but evidence of CJD was later discovered during an autopsy, scientists said.

There is no cure for the disease and no way to diagnose it until symptoms appear. But that can be up to 30 years from the time it is contracted, so hormone recipients and their parents must simply wait.

Roman might have reached an adult height of only 4 feet if not for the growth hormone that was injected into his legs three times a week for 15 years. It added more than a foot to his stature, but his attorney, Chester Leo Smith, maintains it bathed his brain in deadly viruses.

Smith is trying to have charges against Roman dismissed by claiming Roman was the victim of a failed medical experiment. But scientists have called Smith’s claims “preposterous.”

Human growth hormone was distributed to 25,000 children worldwide, said Albert F. Parlow, director of a laboratory at the Harbor-UCLA Medical Center, where the hormone purification process was perfected in 1977.

“This certainly is the only instance where a claim of this kind has been made,” Parlow said of Smith’s contention.

In 1963, after years of testing by some of the nation’s most respected scientists, the National Institutes of Health began an experimental program that distributed the scarce supply of hormone to the most needy children. Later distributed commercially, the hormone cost at least $10,000 for a year’s treatment, but it was free to patients in the NIH program.

Parents said they were told the treatment was safe because growth hormone is a natural substance similar to insulin. Even Roman’s mother used the hormone occasionally because it was once purported to slow the aging process and remove excess weight without exercise, court records say.

But what she and other parents didn’t know--what doctors would not learn for years--was that some of the hormone was infected with the deadly CJD agent. When the link was finally discovered in 1985, it was a devastating blow to parents who had fought to get their children into the NIH program.

“Most parents had to stand on their heads to get treatment,” said Coussan, whose son waited almost a year for the hormone. All the while, she said, he continued to endure the kind of emotional pain that comes from being different--the taunting from class bullies, the pushing and name-calling.

“This was a way for him to be like everyone else,” said Coussan, who chairs a parent-support group from her home in Opelousas, La. “We saw this as a chance to make something that could be a handicap not have to be a handicap.”

In laboratories nationwide, pituitary glands were removed from cadavers that had been screened for infectious diseases. The growth hormone was extracted, purified, then distributed to children through the nation’s pediatric endocrinologists.

Roman was 6 years old when he started taking the hormone, which came in powder form, then had to be mixed with water before being injected.

The technology stemmed from research that began in the 1930s, when scientists discovered that rats stopped growing when their pituitary glands were removed. After two decades of animal experiments, scientists demonstrated that hormone-deficient children began growing when treated with purified hormone extracted from human pituitary glands.

The next step was to find a steady supply of growth hormone. In the early 1960s, the NIH collaborated with the College of American Pathologists and encouraged physicians to collect and store pituitary glands, which were then sent to scientists who extracted and purified the hormone.

For years, the only problem seemed to be the limited hormone supply. Treatment sometimes was suspended once a patient reached a certain height: 5 feet tall for a girl and 5 feet, 1 inch for a boy.

But the patients kept coming, up to 2,000 a year.

In 1985, three former hormone recipients--who were 21, 26 and 34 years old at the time--died from Creutzfeldt-Jakob disease, a neurological disorder that normally struck one in a million people in the overall population, generally men and women in their 50s.

Because CJD is so rare in people under 40--one in 100 million--three deaths in a single year among young adults led scientists to conclude that the victims contracted the disease from contaminated natural growth hormone.

The deadly CJD agent apparently made its way into the growth hormone because the pituitary gland of at least one donor was infected. Scientists said one or more donors had suffered from CJD, but because of the long incubation period--10 to 30 years--it had apparently gone undiagnosed.

The government suspended distribution of the growth hormone immediately, as did two companies that distributed it commercially.

Many parents learned about the development from news reports.

“I was devastated,” Coussan said. “I was angry. I felt like the doctors should have known, that I should have been warned. I felt I put my child’s life at risk because I was not given complete information.”

In 1985, Susan Cabot learned during a conversation with her son’s doctor that her son could develop CJD. As a result, according to court documents, she suffered a mental breakdown.

Since 1985, two more deaths among hormone recipients in this country have been linked to CJD, the most recent in 1987. Two more cases have been reported among growth-hormone recipients overseas. One victim in New Zealand received growth hormone that had been processed in the United States. The other received a British growth hormone.

“It is not what I would call an epidemic,” said Judith Fradkin, director of the endocrinology and metabolism branch of the NIH. “When we had three cases in one year, we didn’t know whether it was the tip of the iceberg. But there has been a slow number of additional cases. All of us are feeling reassured by that.”

But Roman is afraid he may be infected with the disease, and his attorney has argued that the CJD agent, which causes dementia, could have played a role in Cabot’s murder by so severely clouding the brain of her son.

But doctors say that, if Roman was displaying symptoms of the disease when his mother was killed in 1986, he would probably be critically ill or dead today.

“The time course of CJD is rapidly downhill,” Fradkin said. “Two years is as long as someone has lived with symptoms of CJD, and most of these people have died within six months to one year” of when symptoms appear.

Scientists believe that children who received the hormone after 1977, when the purification procedures were perfected, have a lower risk of contracting CJD. But they say there are no guarantees.

“The CJD agent is very different from other viruses,” Fradkin said. “It is not inactivated by conventional methods.”

To help parents cope with the uncertainty and lobby for the rights of hormone recipients, Coussan and her husband, Carl, formed a group called the Parents Council for Growth Normality in 1985.

The council mails a quarterly newsletter to 500-600 parents nationwide and acts as an information clearinghouse. It also lobbied Congress for a five-year, $2.3-million study to identify all growth hormone recipients and determine what percentage is likely to contract the deadly CJD agent.

The study, which should be complete in 1991, involves contacting all hormone recipients and reviewing their medical history.

Fradkin said at least two psycho-social studies of hormone recipients have been done to determine how they performed in school and on the job. Both studies showed that children tended to be overprotected by their parents and a large number lived at home as adults. But, Fradkin said, there was no indication that any displayed violent behavior.

During the 22 years of the NIH project, about 6,500 children were treated with hormones from the pituitaries of an estimated 800,000 cadavers.

Regan Coussan, who took the human growth hormone for one year before distribution was halted in 1985, is almost 14 years old. He now takes a synthetic form of growth hormone and his mother expects that he will reach a height of 5 feet, 6 inches.

Regan recently completed a social studies project about the politics of growth hormone and said he still thinks about its horrors.

“The possibility of dying at 20 or 21 is frightening,” he said.