Polio's second act

 

 

Illustration by Bonnie Hofkin

I na Pinkney has made the best of polio. Her baked goods win national acclaim, and foodies wait for seatings at Ina’s, her culinary star turn in Chicago, where she is known as “the breakfast queen.”

Diagnosed with polio at 18 months in 1944, she was treated by the famed Australian nurse Sister Elizabeth Kenny, whose then-controversial therapy involved boiling strips of wool, wrapping them around the affected limbs, and using massage to alleviate muscle spasms. After a month of the painful regimen, Pinkney was walking again, but the disease had caused lasting damage. For years, she exercised to retrain her muscles, but her right leg never caught up with the left.

Pinkney remembers going to a gala in New York as a young woman. Count Basie played, and her idol, movie star Fred Astaire, was there. She walked over to him, and Astaire said, “I see you have some difficulty walking. Let’s just pretend.”

“He took me in dance position, and we swayed, maybe 12 times – 12 sways with Fred Astaire, a very big moment,” she recalls.

Pinkney now experiences “mind-numbing” fatigue, her leg feels weak, and she has tried exercising to strengthen it. She reluctantly agreed to be fitted with an ankle-foot orthosis (a brace) and wore it home from the doctor’s office. Leaving the office, she fell in the street. Pinkney, 67, calls the episode a “gut blow.”

“It told me I had a lot of work to do,” she says. “I had to learn to walk with the brace and, more important, I had to accept it.”

Postpolio syndrome

Pinkney’s polio had not come back, but she had postpolio syndrome, a set of debilitating symptoms that strikes survivors at least 15 years after they’ve had the disease. As many as 55 percent of an estimated 775,000 polio survivors in the United States may be at risk of developing it. 

“We’d see more support for polio eradication if people understood the long-range effects of the disease,” says Ann Lee Hussey, chair of the Rotarian Action Group for Polio Survivors and Associates. Like many Rotarians who are polio survivors, she is a strong advocate for Rotary’s US$200 Million Challenge. “There are many polio survivors who serve as their district PolioPlus chair and are active in fundraising. I traveled to Hong Kong for an event that raised $250,000.”

Many people have not heard of postpolio syndrome. Compared with the 20th-century epidemics that spawned a national movement in the United States led by President Franklin D. Roosevelt, it’s an understated illness. Many patients who have postpolio syndrome – an under-diagnosed, under-researched condition without broad-based advocacy from patients – don’t even know it’s related to the disease.

It has been 26 years since postpolio syndrome was identified, but often doctors don’t know how to diagnose or treat it, Hussey says. Because polio is viewed as a conquered disease in the United States, its aftermath has been relatively unexplored in the research and in medical schools.

Post-Polio Health International, a St. Louis group that works closely with the Rotarian Action Group, addresses the dearth of information through a network on its website, www.post-polio.org. It connects patients with each other and the few health professionals experienced in treating the condition.

A new tide of potential patients rises in the developing world.

Most of the people who lived through the U.S. polio epidemics will die in the next 40 years – a fact that may offset the irony that the final phase of the most studied virus in history now gets modest public notice. But even greater resources may be needed in the future, as a new tide of potential patients rises in the developing world.

“They’re going to have horrible lives. Who will address their pain and mobility issues?” Hussey asks.

For Daniel J. Wilson, having postpolio syndome means managing a growing list of things he can’t do anymore. “I can’t walk all over Paris and take the metro,” says Wilson, 60, a professor of history at Muhlenberg College in Allentown, Pa., and the author of a well-regarded history of polio in the United States, Living with Polio: The Epidemic and Its Survivors .

Wilson contracted the disease at age five, his mother doctoring him with the hot packs that had become common. The weakened muscles of his right torso led to scoliosis, a type of spinal disfigurement. At 10, he had spinal surgery that put him in a body cast for six months. He completed fifth grade at home in Wausau, Wis., and regained his strength. He later earned his doctorate from Johns Hopkins University.

The first signs

The first sign of postpolio syndrome came in the mid-1980s, when Wilson had trouble lifting his right foot off the gas pedal to brake his car. Soon his right leg began giving out while he walked, and he experienced increasing muscle pain. His wife, Carol, started carrying packages for him. Now he sits while he lectures, walks with a cane, and uses a scooter for longer distances. When his beloved wheaten terrier Abbey died at 16 last year, he decided against getting another dog because he couldn’t walk one anymore. He installed a stair lift at home, preparing for when he can’t handle stairs. 

“I live with the certainty that I can’t trust my body anymore,” Wilson says.

Abraham Lieberman, 72, medical director of the Muhammad Ali Parkinson Center at Barrow Neurological Institute in Phoenix, started to have difficulty walking in the late ’90s and sometimes used a walking stick. By 2001, his left leg was failing, and he diagnosed himself with postpolio syndrome. He has no joint or muscle pain but suffers weakness in his legs.

“I’m not happy about it, but I’m not going to die from it. I’d be happier if I were 10 years younger,” says Lieberman, who was hospitalized with polio in 1944 at age six in New York with nearly full-body paralysis. His mother wrote to Roosevelt for help, and the sympathetic president wrote back, saying he would do what he could. Lieberman’s young life became a cycle of braces, injury, and surgery, but left him with strong hands and the ability to walk without assistance. He finished medical school, served in the U.S. Air Force as a doctor in Japan, and later specialized in research on Parkinson’s disease.

“You can manage the pain and fatigue, but there’s no simple test for it,” says Julie Silver, assistant professor at Harvard Medical School and former director of the International Rehabilitation Center for Polio at Spaulding Framingham hospital in Massachusetts. “It’s a diagnosis of exclusion.” After thyroid problems and sleep apnea have been ruled out as causes of fatigue, for instance, postpolio syndrome may be considered in a patient who has slowed down.

There's the shock of realizing that they aren't finished with polio.

At the rehabilitation center and the handful of other facilities across the country specializing in postpolio syndrome, an assessment typically includes an examination by a doctor experienced in the condition, a nerve and muscle study, and sessions with physical and occupational therapists, a brace specialist, and a psychologist.

The psychological fallout of a diagnosis can be dramatic. “It feels like a double whammy,” says Silver, author of Post-Polio Syndrome: A Guide for Polio Survivors and Their Families . First, there’s the shock of realizing that they aren’t finished with polio, and then that no recovery from postpolio syndrome is in view. Care recommendations can include home modifications, a brace, stress management for fatigue, lifestyle changes such as reduced work hours, and devices to help with breathing and mobility, alleviate pain, or prevent falls.

In the 1950s, polio survivors learned to exercise during rehabilitation, often in great pain. The new field of physical therapy strongly linked determination with overcoming physical challenges. Charles Atlas was telling men they could build a muscular body through willpower and isometric exercises, and Norman Vincent Peale in The Power of Positive Thinking was saying attitude was everything. Both ideas were part of American culture then.

“When you went into rehab, the emphasis was on pushing as hard as you could, like the Little Engine That Could. Physical therapists and families pushed polio survivors to achieve the maximum results, and in many cases substantial recovery was possible,” Wilson says. “We had won World War II, and we were moving forward. Men had a lot of concerns about masculinity and proving they could take it. Dealing with painful physical therapy demonstrated you weren’t a sissy.”

More harm than good

Pinkney recalls the pressure she felt from the public campaign against polio. “How could you let anyone down, with all of them on your side? Polio children learned to be such good children,” she says.

Then, years later, came postpolio syndrome. Research showed that the exercise that had been recommended actually did more harm than good. Unlearning the old rules was as much a cultural shift as a medical one, says Wilson, whose book devotes a chapter to the illness. De-emphasizing exercise initially strikes many survivors as backward.

To overcome her postpolio fatigue and weakness, Pinkney went back to the old playbook. “But it hurt me,” she says. “I’d be in better shape now if I hadn’t exercised.” Today she walks with a cane and predicts she will rely on a wheelchair full time within six years.

These realities are familiar to Lauro Halstead, director of the postpolio program at the National Rehabilitation Hospital in Washington, D.C., and a key figure in the story of postpolio syndrome. In 1984, Halstead organized the first medical conference devoted to the condition.

In the 1970s and ’80s, survivors started reporting symptoms reminiscent of polio. Patients and doctors feared the virus was back. Other doctors suspected the chronic condition fibromyalgia or multiple sclerosis. Some told patients the symptoms were in their heads. To make sense of the reports, Halstead, then a doctor at the Institute for Rehabilitation and Research at Baylor University in Houston, organized a national meeting of experts at the Roosevelt Warm Springs Institute for Rehabilitation in Georgia, the polio center founded by Roosevelt.

The neurological system makes adaptations that can wear out the surviving motor neurons.

A polio survivor in his late 40s, Halstead was having unexplained leg pains himself. He had polio after his freshman year of college and split the next year between an iron lung and a wheelchair until he regained his strength. He lost the use of his right arm and hand but taught himself to write left-handed and finished his schooling. Becoming a spinal cord injury specialist at Baylor, he assumed polio was behind him until the pain returned.

“The leg pains were very like the leg pains I experienced during the acute phase of polio. Fortunately, there were a lot of hotshots at Baylor to look into it. It wasn’t polio, but nobody could figure out what it was,” says Halstead, 74, editor of Managing Post-Polio: A Guide to Living and Aging Well with Post-Polio Syndrome .

Then he read an article by David Wiechers, a researcher at Ohio State University who was working with electromyographic diagnosis, which monitors electrical activity in the muscles to diagnose neuromuscular problems. Wiechers had tested some polio survivors who had the same symptoms and noticed surprising neurological changes. His work raised more questions than it answered, though.

Media were riveted to the notion that polio was back, which generated plenty of publicity for Halstead’s conference. But the triumph of the event was setting a research agenda. Studies would show that polio was not back. Fragments of the virus were found in patients but weren’t reinfecting them. Researchers soon named the new disorder and clarified its characteristics.

Now the pathology is clear. During the acute phase of polio, patients can lose motor neurons, the nerves that carry signals to the muscles. More than 50 percent of them can experience weakness and possibly paralysis. The neurological system makes adaptations that can wear out the surviving motor neurons.

Imagine a right arm attacked by polio and an unaffected left arm, Halstead says. The right arm’s dead nerve cells no longer stimulate the muscle, so the muscle atrophies but still sends out a chemical signal that instructs the healthy left arm to develop more “axon sprouts” – the endings on the motor neurons where chemical changes take place for muscle stimulation. Catastrophically, the number of sprouts increases.

Neurons

“Think of the tremendous metabolism it takes to generate the chemicals needed by each axon sprout. The motor neurons get worn out. That accounts for the new weakness in the muscle,” Halstead says. Exercise is thought to spur the unwanted growth of new sprouts.

Questions about postpolio syndrome still outnumber the answers: Why do some people get it while others don’t? What might cure the condition? Can it be prevented?

There’s not much new in the research. Postpolio syndrome has always been an orphan disease – the crisis of the few since polio became a footnote in U.S. history. Before the illness was even identified, the once polio-centric March of Dimes had changed its focus to birth defects. 

Some work continues. The John P. Murtha Neuroscience and Pain Institute in Johnstown, Pa., is exploring nonfatiguing exercises and stress-reduction behaviors at its polio-survivors clinic. Studies in Canada, France, Norway, and Sweden show that the immune system may have an influence on postpolio syndrome, and interest in a long-term U.S. clinical trial to replicate them is growing. Research may lead to a gamma globulin shot to reduce symptoms.

Halstead says studies have fallen off in the past 5 or 10 years, as U.S. polio survivors die and the medical complications of aging make it harder to research them. But the syndrome could continue for years.

“It is just now becoming an issue in India, and it will be eventually in all areas of the developing world as the average lifespan increases,” says Hussey, of the Rotarian Action Group. While Rotary’s eradication efforts have dramatically slowed the rate of polio infection, the World Health Organization estimates that survivors number between 10 and 20 million worldwide. “Long after the last polio case, postpolio syndrome will persist as a significant personal, social, medical, financial, and political challenge,” Hussey says.