Cure in the long term; treatment in the short

November 15, 2000

Medicine, therapy
offer hope for those
suffering from PPS

Genetic research may one day reveal why the joint pain, muscle weakness and fatigue once suffered by polio victims return decades after they disappeared. But that day may never come for middle-aged post-polio-syndrome patients.

More immediately promising for Mary Clare Schlesinger and other San Diego PPS patients are advances being made in therapies and medications that can stem the syndrome's debilitating effects.

Clinicians like Dr. Susan Perlman of UCLA, who specializes in diseases of the neuromuscular system, provide hope that the degenerative effects of polio's devastating reappearance can be halted or even reversed.

Perlman is following in the path of the late Dr. Kenneth Moser, who specialized in pulmonary care, and the late Dr. W.C. Wiederholt, a specialist in neurosciences. Both physicians, who were UCSD professors, once treated Schlesinger.

She met Perlman in 1995. "It was refreshing to see a doctor who was not only familiar with PPS but very helpful," Schlesinger said.

She contracted polio at age 3 in the epidemic of 1952, spent time in an iron lung and remained permanently weakened on her left side.

After a spinal fusion at age 13 that required her to remain in a body cast for a year, Schlesinger taught herself to walk. She was in college when the first signs of shortness of breath occurred; having a child several years later proved to be an ordeal.

As many PPS patients have done, Schlesinger moved (from Pennsylva
nia) to San Diego because the warm climate aided her breathing problems. With Moser's therapeutic help, she "breathed easily for 10 years" before PPS gradually overcame her.

Under Perlman's care, Schlesinger takes a variety of pulmonary medications, uses a "ultrasonic nebulizer" (a device to keep her lungs clear), and said she has become expert at manipulating the muscles that control breathing. She also relies on "my service dog for balance when walking."

"I do a lot of stretching exercises, but the most important exercises I do have to do with breathing. I have a regimen I must do twice a day; if I skip, secretions can easily build up in my lungs and become a breeding ground for infection," Schlesinger said.

"Pneumonia would be a death sentence for me."

Perlman said her clinic at UCLA is "set up to deal with (PPS patients') needs of proper diagnosis, rehabilitation treatments and medication management."

"We teach the basics of post-polio to neurology and rehabilitation residents and see about 200 patients a year," she said.

Post-polio syndrome is a diagnosis of exclusion, Perlman said.

PPS is suspected when a patient has survived a case of acute polio, has had at least 15 years of stable recovery and is now experiencing "gradual onset of new/recurrent muscle atrophy, weakness, generalized fatigue, pain in the old polio limb" and other symptoms.

Recent breakthroughs regarding treatment of PPS, Perlman said, center on rehabilitation and immune-system research.

"We now know that energy conservation, not pushing to the point of pain or fatigue, plus gentle conditioning exercises and appropriate equipment when needed, like braces and electric mobility, can stop progression of post-polio and even give improvement in all but 5 percent of sufferers," she said.

"There continues to be evidence suggestive of the role of the immune system in causing post-polio. More research is ongoing in the United States and other countries to nail this down."

Clinical research funded by the Warm Springs Institute, the Georgia center established by America's most famous polio patient, President Franklin D. Roosevelt, has added to the understanding of PPS symptoms and treatment.

And research into the causes of PPS at the molecular level has been done at Ohio State University, the Montreal Neurological Institute and several other facilities in North America.

Perlman worries that PPS will not get the research attention and focus it deserves because its sufferers are dwindling in number as those once affected by polio die.

"Post-polio is a model disease for some key questions in neurology about nerve regeneration, the reaction of the immune system and viral diseases of the nervous system," she said.

"More education is definitely needed. I think the errors of society and the medical field are more of ignorance rather than rejection. There are more survivors at risk for PPS in the United States than there are people with multiple sclerosis," Perlman added.

"Polio still has a lot to teach us."

Those suffering from PPS realize molecular-level research such as that being done by Sam Pfaff at the Salk Institute in La Jolla may not be completed in time to help them.

But for Mary Clare Schlesinger and many others who still struggle daily with a disease that was "beaten" decades ago, research provides hope.

There is a sharp division here, however, Schlesinger said.

"Some of us keep in touch with medical developments, ready to try something new. While few expect a Salk-vaccine-level of breakthrough in their lifetimes, most realize medical advances come quickly these days and want to be ready.

"Others with PPS think research is wasted energy and false hope. They think a better use of their energy is to learn to live with their condition.

"But most of us feel that hope is universal, with different meanings to every individual. We find it impossible to judge hope as false or cruel."