Sufferers Find Parkinson’s Is No Respecter of Youth : Health: Traditionally a disease of the elderly, it is now diagnosed in young adults with startling frequency.
When Ray Vignola learned that he had Parkinson’s disease, the news came as more of a shock than it does for most people: he was only 34 when it happened and was in the prime of his career.
Don Rosenblum, an editor at the Department of Veterans Affairs, was 38 when he noticed the tremor in his left hand. At first, his physician assured him that it was nothing. Later came the diagnosis: It was Parkinson’s.
Such cases are stunning to those affected. Traditionally, Parkinson’s--a progressive neurological disorder that leaves its victims shaking and eventually too debilitated to physically function--has been thought of almost exclusively as a disease of the elderly, its onset typically restricted to those 60 and older.
Much to their surprise, however, physicians have begun noticing that the disease increasingly is showing up among people in their 50s, 40s and even 30s.
Dr. Stephen Reich, co-director of the Parkinson’s Center at the Johns Hopkins School of Medicine in Baltimore, says he has seen Parkinson’s so much in younger patients in recent years that he no longer considers it an aberration.
“The first couple (of cases that) I saw personally, I’d ask several of my colleagues for a second opinion,” he says. “I don’t do that any more.”
There is no long-term research that analyzes age-related trends in Parkinson’s, so it is still not clear whether there has been a real rise in the incidence of the disease in young people, or whether clinicians have simply become more adept at recognizing it in this age group. Mayo Clinic researchers have embarked on one study, but they do not expect to complete it until later this year.
Dr. Thomas Chase, a Parkinson’s expert at the National Institute of Neurological Disorders and Stroke, suggests that Parkinson’s may never have been limited to the elderly, except in the minds of physicians, who tended to dismiss its symptoms in younger people.
“I think that early onset is more vivid in the memories of clinicians because it’s so unexpected,” Chase says. “Therefore, they might tend to overemphasize the cases. They’re quite striking.”
Johns Hopkins’ Reich agrees. “It’s clear from the literature that young onset has always been with us,” he asserts. “The question is whether we’re seeing more.”
Dr. Lawrence I. Golbe, a neurology professor at the University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School, who has studied both groups, says there are significant differences between the course of the disease in younger sufferers and in the elderly.
Younger patients seem to experience less dementia, Golbe says, but they suffer more pronounced side effects from pharmaceuticals--including confusion, hallucinations and excessive movement and a “writhing, irregular” motion called chorea.
Younger Parkinson’s patients can also experience dystonia--a prolonged abnormal posture that often is accompanied by a “twisting component of one or more parts of the body,” Golbe says. “Often, the first sign of Parkinson’s in young people is a turning in at the ankle or arm.”
Finally, when Parkinson’s starts at a young age, the disability generally progresses more slowly, Golbe says. “Someone with Parkinson’s starting at age 40 will be better off 10 years later than someone 10 years later who started at 50,” he says.
Expectedly, the reality of the onset of Parkinson’s can be even more traumatic for younger people than for those who are older. Many of the younger Parkinson’s sufferers are in the midst of rearing children. Some are headlong into careers.
“Their first reaction often is that life is over,” says Hopkins’ Reich. “We have to do a good job telling them that the disease . . . is very slowly progressive--and that it’s very individualized. We try to encourage patients to lead as normal a life span as possible.”
The young also face an uncertain future, not knowing how incapacitated they will be two or three decades later, when most Parkinson’s cases are only just beginning.
Joan Samuelson, a Santa Rosa, Calif., lawyer who was 35 when the disease prompted a swelling in her left knee, says that the going can be rough. “You die by inches,” she says. “Bit by bit, the pieces of what have been an integral part of your life just get ripped away.”
Rosenblum, now 41, recalls the depression he felt after attending a local Parkinson’s support-group meeting shortly after his diagnosis:
“They were all very old people who were in very poor physical condition--I was the only one under 60,” he says. “Everyone had a walker. . . . I felt totally isolated.”
One difficulty is that there is no diagnostic test to determine if an individual has Parkinson’s. Physicians usually diagnose it by knowing the characteristic symptoms and by ruling out other conditions for which screening exists.
Patients are often embarrassed, and try to hide signs of their disease from others--usually through medication that eliminates most symptoms during the first few years, experts say.
“I know a Wall Street accountant in his mid-40s who is . . . certain that the day he discloses his Parkinson’s to his firm he will lose his job,” Samuelson says. “He doesn’t have obvious symptoms yet. . . . He stays heavily medicated.”
But this is often difficult for many young Parkinson’s sufferers to accept--particularly when vital issues of work, marriage, family and lifestyle are at stake.
“You love to hike and backpack, and suddenly you can’t do that anymore,” says Samuelson, now 41. “You have to try to reorder your life and make it meaningful without the things you love to do.”
Parkinson’s, which afflicts more than 500,000 Americans, gradually destroys an individual’s ability to move. Its symptoms include stiffness and rigidity in the muscles, tremors in the arms and legs and a shuffling gait with the body bent forward.
The condition occurs when an area of the brain called the substantia nigra is damaged or destroyed. Cells in that part of the brain manufacture and secrete dopamine, a chemical needed to control muscle movement. The shortage of dopamine causes the tremor and rigidity.
In the advanced stages of the disease, patients can experience difficulty speaking and often are unable to perform even the simplest of tasks, such as buttoning their clothes or cutting their food. They can become totally dependent on others.
Although some Parkinson’s patients experience dementia, the disease usually does not damage an individual’s mental capacity.
But patients frequently encounter severe depression. “It is more than just a normal reaction to the prognosis,” Golbe says. “Some chemical change in the brain seems to make them more susceptible to depression.”
There are drugs to treat Parkinson’s, but over time they can cause unpleasant side effects. They also may work less effectively. And even then, the symptoms can persist. One, called the “on-off syndrome,” leaves patients suffering from spells where they are frozen in place and simply cannot move.
Young Parkinson’s patients also must often make major career changes, or other financial decisions, since they will almost certainly be physically impaired to some degree within 10 years.
Much depends on the nature of the work. Nothing has changed for Vignola and Rosenblum, who work in offices. “In fact, I’m doing more and I recently got a promotion,” Vignola says.
But jobs that require rapid reflexes or a deft hand are soon endangered. A New York anesthesiologist, diagnosed when he was 48, could practice for only about five years after diagnosis--and only under heavy medication.
He began taking drugs immediately so he could hang on to his career for as long as possible. But he stopped clinical work when the symptoms could no longer be controlled, even with the drugs.
“A lot of times you have to work very quickly,” he says. “With this disease, you simply can’t react.” He spent another three years in an administrative hospital post, but hated it and retired.
Samuelson, a civil litigation attorney, has substantially reduced her workload. “I was having to sit on my left hand because it was trembling,” she says. “I thought it could hurt my client’s position because it would look like I was nervous.”
Samuelson and her husband are trying to have a child. Because she fears the effects of drugs on a developing fetus, she has decided against taking Parkinson’s medication.
And she girds herself for what will almost certainly be an overwhelming physical challenge if the couple is successful. She uses a cane and drags the left side of her body in a kind of lurch when she walks.
“It will be hard, I know it will be hard,” she says. “Going to the grocery store is getting harder. But it would be much harder yet to completely give up that aspect of living. And I don’t think physical capacity is the most important element of parenting.”
Despite moments of despair, Samuelson chooses to focus on role models who are fighters. “I now know lots of people who’ve had kids and raised kids who’ve had Parkinson’s for a long time,” she says. “One of my role models has had it for 40 years.”
Ray Vignola’s wife was pregnant with their second child when he learned that he had Parkinson’s disease. He is now 41 and his daughters are 9 and 6.
“I remember when my older daughter sat there one day and held my hand when it was shaking,” he recalls. “The other one has never known me any other way.
“I feel bad at times when I think I’d like to do more with them than I do, and I wonder whether I’ll be able to take them to their high school dances.”
Arlette Johnson, 43, of Santa Maria, Calif., a former teacher’s aide, cried on the way home from the doctor’s office after she was diagnosed five years ago. “Why me?” she recalls asking herself.
Adjustment to symptoms such as her loss of facial expression was difficult for her children. “My younger son . . . would bring his friends home, and they would tell him I looked mean,” she says. They said they didn’t want to come over anymore. . . . That hurt.”
She and others say that Parkinson’s also can take a toll on marital relationships.
“For our fourth wedding anniversary, Mike (her husband) gave me a photograph of Mt. St. Helen’s exploding and said it was symbolic of our marriage,” Samuelson says. “It was a joke, but it was descriptive of how hard it’s been.
“My symptoms appeared during the first year of our marriage--so at the very time our marriage was defining itself, suddenly we’ve got this enormous additional problem,” she says. “Fortunately, we’ve been able to work through it, but a lot of people can’t.”
Since Rosenblum’s disturbing experience at an elderly support-group meeting, he and other young Parkinson’s patients at Hopkins have formed their own group. And Arlette Johnson has started a young onset group in California that is about to go national.
Both find that interaction with other Parkinson’s patients like themselves enables them to remain optimistic.
“The most striking thing to me about our young group is that all of them, for the most part, are still working and doing well,” Rosenblum says. “One fellow, an attorney just came back from Europe.
“They’re affected, but not impaired. They’re living their lives and doing what they want to do. Some are worse than others--they’re not all running around. But, contrasted with this other group I saw in such poor condition, this is still a positive thing to see.”
