Mamie Lieberman was a dynamo. She raised five children in Camden, N.J., scraping by while her husband disappeared for long stretches. It was Mamie who put the family on a stable financial footing, though not until her children were grown. She was past 50 when she started selling odd lots of shoes to her neighbors. Within a few years, she had opened a series of seven stores.
Mamie could sweet-talk a customer into buying another pair, survey every corner of her store while never letting the cash register out of her sight, and set aside the best shoes for me and her 14 other grandchildren.
But she wasn’t a shoe mogul for long. By the time she was 70, she almost never left the house. She couldn’t dress herself. She spent her days sitting on the sofa, her face expressionless and nothing moving but her hands, which trembled constantly. She had Parkinson’s.
I have it, too. When the disease was diagnosed seven years ago, images of my immobilized grandmother filled my mind. But she had the illness 40 years ago, before polio was wiped out and tuberculosis was still common. I have an arsenal of medications to control my symptoms.
But one thing hasn’t changed. Unlike polio and TB, Parkinson’s still can’t be prevented or cured. Forty years ago, patients were told that a cure was probably a decade away. I still hear the same hopeful estimate. But unless something happens soon to speed up the search for new treatments – more aggressive federal funding of stem-cell and other scientific research, for instance, and a more streamlined approval process for new drugs – then my ultimate fate will be no different than it would have been in 1960.
Signs of Parkinson’s
My astute internist first sent me to a neurologist because of my handwriting, which had always been small and tight but was getting worse. Micrographia, it turns out, is a common symptom of Parkinson’s. I had almost no tremor, the symptom most people associate with the disease, but as I read about it I realized I had many symptoms that I’d never considered more than unrelated quirks. I had trouble getting up from a chair, getting out of bed and even turning in bed. My voice had grown softer, and I was clumsier, often tripping and falling. Putting change in my wallet was awkward and slow; in stores, I could feel the impatience of shoppers behind me.
Fortunately, for my job as a restaurant critic, the disease hadn’t robbed me of my sense of smell, as it does many people. But I was beginning to look less than respectable when I tried to eat spaghetti or use chopsticks. There were days when I nearly fell asleep at lunch with my face in my mousse. Driving terrified me.
Parkinson’s primarily affects a part of the brain called the substantia nigra, and by the time it is diagnosed it has killed about 80 percent of those cells. They supply the brain with dopamine, the chemical messenger responsible for directing and coordinating movement, including autonomic functions such as sweating and swallowing. In early Parkinson’s, a person doesn’t typically swing her arms when walking, at least on the affected side (eventually both sides become affected). Walking itself becomes erratic. In advanced stages, Parkinson’s causes freezing – as if you’re glued to the ground – and slow and halting steps, or sometimes uncontrolled running as if you’re going downhill with no brakes. Because I am at an early stage, I can walk well for half a block (on a good day), and farther with a cane.
The really knotty part of this disease is its unpredictability: Its day-to-day symptoms are variable, and its progress has no predictable pace. I’ve met people who’ve had it for 34 years and still live independently. Others who have had it for just a few years need medication every couple of hours, and some already feel their minds becoming as undependable as their tremulous hands.
I take one to seven pills four times a day – $12,500 worth a year. As I worsen, I’ll be taking more pills and more kinds, more often. At some point, they will create their own problems. The dopamine pills that unlock my rigid body every day often cause dyskinesias (jerky, writhing and uncontrollable movements). Parkinson’s medications commonly cause sleeplessness or sleepiness, weight gain or loss, hallucinations and compulsive behaviors including, for some men (I know this one is hard to believe), uncontrollable gambling. Twenty percent to 40 percent of us will become demented. Among the typical early symptoms that lead to diagnosis is depression, a chemical effect of the disease that can manifest itself at any stage.
More than 1 million Americans share this dismal future with me, and 40,000 to 60,000 more join us each year. The average age of onset is 57, but 15 percent of people with Parkinson’s (PWPs, we call ourselves) receive the diagnosis before age 50, some as young as 18.
Parkinson’s is an expensive disease, partly because people live with it for so long. Nursing care costs can be astronomical, and the newest brain surgery, in which a kind of Parkinson’s pacemaker is implanted, can cost $100,000. Nationally, it has been estimated to cost $5.6 billion a year, including treatment, disability pay and lost income, a figure expected to climb steeply as baby boomers reach prime Parkinson’s age.
Why have there been so few advancements? With a mere million sufferers, Parkinson’s is not worth the risk for pharmaceutical companies to sink big money into unexplored territory. There’s a surer profit in developing another variation of a successful drug than in creating a new kind of drug, for which the clinical trials are apt to be more expensive, the chance of failure runs higher and the approval process will likely take longer. Thus I can choose Mirapex or Requip, which serve the same purpose. Today’s breakthroughs are quick-acting injectable drugs and delivery by patch rather than by pill – useful, but I’d much rather have a wider range of treatments than a choice of brand names.
Of course, as a patient, I don’t have much say in the matter. Or do I? If there is one person who will deserve credit when this disease is eventually cured, it will most likely be my fellow PWP Michael J. Fox. In seven years he has raised so much money that, with matching grants and joint projects, he has directed $90 million worth of Parkinson’s research. His public presence has brought Parkinson’s great attention and a sympathetic response.
The actor also has been a charismatic representative of “patient power.” Parkinson’s patients have developed Web sites, such as www.pdpipe
line.org, that track clinical trials and drugs in the pipeline. They have participated in conferences, spoken about and to drug companies, and rounded up subjects for clinical trials. During the annual legislative forum of the Parkinson’s Action Network in February, PWPs fanned out for 250 meetings with their states’ congressional staffs in one day. At the evening reception it was announced that the Parkinson’s caucus, which last year numbered about 80 members, had that day alone received more than 80 calls from legislators seeking to join, doubling its size.
The next frontier is political. Private enterprise can’t afford the expensive next steps, so promising drugs wait years to be tested. Nor can the public coordinate progress the way a government body can. Research is shooting off in myriad directions, and someone should ensure that the branches are in communication. What is too costly for private enterprise can turn out to be action the government can’t afford not to take.
Politics is particularly obvious in the issue of embryonic stem cells – the thousands of leftovers from in vitro fertilization – which by now are merely a symbol. It’s not that they are so sacred that they are being cared for and protected; rather, they are defrosted and disposed of. Medical waste has more respect than sick people, for whom stem-cell research could prove promising for many debilitating diseases if it had sufficient government funding. This is not a matter of preserving the life of cells, but of preferring to consign them to the dump rather than the test tube.
Limiting federally funded research to adult stem cells isn’t viable. The search for a cure requires both. Story Landis, director of the National Institute of Neurological Disorders and Stroke, was unequivocal when questioned in a Senate hearing recently about how the federal policy on stem-cell research is affecting medical research.
“We are missing out on possible breakthroughs,” she said. The ability to work on newly derived stem-cell colonies, currently precluded from federal funding, “would be incredibly important.”
On Jan. 11, which would have been the 95th birthday of Mamie’s eldest daughter (my mother), the House voted 273-174 to expand embryonic stem-cell research. It wasn’t enough to override a presidential veto, but it was 18 votes more than identical legislation won last year. The Senate vote on stem-cell research is expected in the next few weeks. Feb. 28 was Mamie Lieberman’s birthday. I know just the right belated present for her.
