Up to 20 million polio survivors around the world face the threat of new disabilities 15 to 40 years after their original illness, which could leave them using wheelchairs or ventilators for the rest of their lives, says a new report from the March of Dimes.
A major problem confronting millions of polio survivors is that too few doctors, in both industrial and developing countries, recognize this slow moving and little understood secondary illness, called post-polio syndrome (PPS). The main symptoms of PPS are new progressive muscle weakness that gradually worsens, together with severe fatigue and pain in muscles and joints. It is often accompanied by decreased muscle endurance during activities.
No cure exists for PPS. Doctors advise patients to curtail physical activities and take rest periods during the day, including during working hours. Many doctors also advise more severely affected patients to use wheelchairs part-time or full-time, even if they can walk without them.
As many as 40 percent of polio survivors may get PPS, according to some estimates. The March of Dimes, a nonprofit agency founded by U.S. President Franklin D. Roosevelt, who was a polio survivor, now is alerting medical systems around the world to PPS with its new report, Post-Polio Syndrome: Identifying Best Practices in Diagnosis and Care. It is also releasing a second report to assist people with PPS: Guidelines for People Who Have Had Polio.
As many as 250,000 U.S. polio survivors may have PPS, the March of Dimes says. Some 40,000 polio survivors have PPS in Germany, 30,000 in Japan, 24,000 in France, 16,000 in Australia, 12,000 in Canada and 12,000 in the United Kingdom, according to local polio support groups and doctors. The World Health Organization estimates that 10 to 20 million polio survivors are alive worldwide, and some estimates suggest that 4 to 8 million of them may get PPS.
"In developing countries, where polio outbreaks still occur or have ended much more recently, medical systems will be facing PPS for decades into the future and have little knowledge or understanding of it," says Christopher P. Howson, Ph.D., director of Global Programs for the March of Dimes. "Even in advanced countries, and this includes the United States, many doctors are not trained to recognize PPS or are reluctant to treat it as a new condition."
Grassroots groups of polio survivors in North America, Western Europe, Japan, Australia and New Zealand have been the main advocates for increased medical and government attention to post-polio syndrome.
"The situation is less clear in developing countries, where the syndrome is even less recognized," says Dr. Howson. "Perhaps PPS is underreported because health care providers are not recognizing it. Or perhaps people who are more at risk of PPS in developing countries tend to die before they reach the age of recognized disease onset. Also, PPS seems to be more common and severe in people who have successfully rehabilitated themselves from the initial polio attack. Perhaps a lower percentage of people in developing countries have had the opportunity for rehabilitation and, thus haven’t put themselves at additional risk of PPS."
More than 40 years ago, the March of Dimes completed its original mission to combat epidemic polio by supporting the development and widespread use of two effective vaccines, one by Dr. Jonas Salk and one by Dr. Albert Sabin. Today, the March of Dimes works to improve the health of mothers and babies, but it has not forgotten its historic legacy.
Two Survivors’ Stories
Lucile Harford, an American, and Zohra Rajah of Mauritius are two polio survivors from different regions of the world whose history with PPS is similar to many with this little-understood condition.
Lucile Harford, now 88 years old and retired in Florida, contracted polio (then called "infantile paralysis") in 1925 in her hometown of Geneva, New York, when she was 12 years old. A large red quarantine sign was placed on her front door, causing her nearly as much fright as the disease, and making the days and nights lonely for her, since no one wanted to go near her. Lucile's arms, legs and back were paralyzed. It was two years before she could return to school.
"I couldn't walk, I couldn’t use my arms," she says. "I couldn't even sit up. It was a very painful disease, too. No physical therapy was available, like there is now. I was treated at home. My doctor didn't have a clue how to help me."
"The emphasis was to get me to walk again," she says. "That perhaps was a poor judgment call. I walked before my hips and knees were strong enough, causing irreparable damage. I have never since been able to climb steps without holding onto the railing or rise easily from a chair."
Mrs. Harford finally regained full use of her arms and back, and could walk with a cane. She graduated from college, had a successful career and a happy marriage.
Feeling the stigma of polio-caused disability, she seldom spoke of her limitations or acknowledged that she had had polio. During the 1950s, when the polio epidemics were at their peak, she suffered new difficulty in walking and fatigue that her doctors assured her had nothing to do with polio. Reading of post-polio syndrome, she tried to interest her doctors in the disorder. "To this day," she says, "it is difficult to find a doctor who will say there is such a thing as PPS."
"It got slowly worse," she says. "Not overnight, but over months, years. It was very frustrating. I went from using one cane to two canes, then a walker, and finally fulltime to a wheelchair."
Mrs. Harford can still walk short distances, but uses a wheelchair most of the time now. She says that even though she cannot regain her muscle strength, she is glad that attention to PPS is growing. "Just hearing that the March of Dimes and medical experts are holding conferences on PPS tells me that people really care," she says. "Even though there may not be a cure for PPS, just knowing that there are those looking for a cure is a boon to all of us with this condition."
Zohra Rajah says she always lived an active life, despite the polio she had as a child in 1945. "In 1985, I started having new problems -- extreme fatigue and pain, especially in the back. I visited three different doctors here in Mauritius and they all said I was just getting old," she says. In California during a family visit, she saw doctors who diagnosed her with PPS. She later founded a PPS support group in Mauritius, which now has 75 members. "I mostly treat myself, using what I learn from books, magazines and the Internet, because doctors here just do not recognize PPS," Mrs. Rajah says.
"The problems of these two women are consistent with what many polio survivors experience when they develop PPS," says Dr. Howson. "The most distressing psychological aspect for people with PPS is the unexpected change in their health status, and this is magnified when they can’t find a doctor who will help them or even acknowledge that they have a new condition."
"Some people with PPS go from independent breathing to needing a ventilator. Others go from walking unaided, to walking with the assistance of a cane, to using a wheelchair. Both of these pieces of equipment may improve the quality of life of the individual," says Kristian Borg, M.D., Ph.D., a neurologist who heads Huddinge University Hospital in Stockholm, Sweden.
What Causes PPS?
"The causes of PPS are not thoroughly understood," says Lewis P. Rowland, M.D., professor of neurology at Columbia University College of Physicians and Surgeons in New York City and chairman of the March of Dimes Steering Committee on Post-Polio Syndrome.
"In the initial acute polio episode, patients can lose up to 60 or 70 percent of their motor nerve cells. The surviving nerve cells find muscle fibers that still work and attach to them, restoring function. After 15 to 40 years," Dr. Rowland says, "the ability to maintain function seems to be lost, but no one is certain why this happens."
The most widely accepted hypothesis is that PPS results from a degeneration of motor nerves that sprouted new connections years earlier to make up for other nerves killed by polio. Because the surviving motor nerves have been supplying many more muscle fibers than nature intended, they may wear out prematurely.
The normal aging process and the overuse or disuse of muscles may be contributing factors in development of PPS.
"We are having difficulty in alerting the world medical community to the problems of PPS," says Dr. Rowland. "The problem will go on much longer in other countries, especially in the developing world, so it is important that people learn about PPS. Most doctors in their 40s or younger in developed countries have never seen a case of polio, or recognized a case of PPS."
The March of Dimes Steering Committee found that medical knowledge and commitment to treating PPS varies widely in industrial countries, while knowledge is much lower in the developing world.
PPS first came to wide attention in the medical community during the 1980s -- three decades after the worst polio epidemics in the United States, which struck more than 50,000 people in 1952 alone. Up to 250,000 U.S. polio survivors may have PPS, the March of Dimes says.
"After the vaccines came out, the medical profession and the public in general all thought polio was over with," says Lauro Halstead, M.D., of the National Rehabilitation Hospital in Washington, D.C. and a member of the March of Dimes Steering Committee. Dr. Halstead was struck with polio as a college student in the 1950s. The disease temporarily paralyzed his right arm and put him in an iron lung and then a wheel chair.
"I made a good recovery, finished college and medical school," Dr. Halstead says. "But I began experiencing new weakness in the early 1980s. Doctors had no idea what I had, with some suggesting multiple sclerosis and others, Lou Gehrig’s disease. I think a million dollars was spent on tests, but doctors just couldn’t figure it out. I had new weakness in my legs and very intense muscle pain that was reminiscent of the pain I had with polio. I began to wonder if it was connected." When he met David Wiechers, M.D., who was studying polio, the pieces of the puzzle came together. Dr. Halstead was diagnosed with PPS.
In 1999, in response to requests from a growing number of physicians and polio survivors, the March of Dimes convened an international steering committee of experts chaired by Dr. Rowland. The committee was charged with addressing an issue of critical importance: the need for improved knowledge among health care providers and polio survivors of the best practices in diagnosis and care for PPS. This steering committee drafted the two reports released today by the March of Dimes.
"These reports represent the collective wisdom from post-polio syndrome research and experienced clinicians," says Joan Headley, executive director of the International Polio Network. "The information contained in them is invaluable. Individuals and professionals who have read confusing and conflicting information about PPS will find these reports clarifying."
Some 15,000 polio survivors live in Sweden, says Gunnar Grimby, M.D., professor emeritus and former head of the Department of Rehabilitation Medicine at Sahlgrenska University Hospital in Göteborg, Sweden.
Polio survivors began to complain of symptoms consistent with PPS in large numbers during the early 1980s, Dr. Grimby says. "Initially, Swedish doctors did not recognize the disease, but beginning in the early 1990s, there was enough knowledge that the national medical system began to fund PPS rehabilitation," he says.
Thirteen of Sweden’s 26 counties have special PPS clinics, with several counties having more than one. Current medical students get only a one-hour lecture in polio and post-polio syndrome at Sahlgrenska University Hospital.
"The clinic treats several hundred people with PPS, and 50 to 80 percent of them have progressive muscle pain and weakness," says Dr. Grimby. "The others have more minor weakness problems or their condition is not progressive. Some 20 percent use a wheelchair. Some were advised to use the wheelchair but about one-half declined; some used it before getting PPS."
Sweden is also receiving an increasing number of immigrants from Africa and the Middle East where polio is still prevalent. Some 25 percent of the polio patients at Sahlgrenska University Hospital are immigrants, including those from Albania, Turkey, and countries of the former Soviet Union.
"PPS will be a problem in Sweden for decades to come," Dr. Grimby says. "Most people with PPS have to be in contact with medical personnel for the rest of their lives. They need regular physical therapy and technical aid."
Some 30,000 polio survivors live in Japan, where they get widely divergent treatment from Japanese doctors, says Kimiko Negayama, president of the "Living Well with Polio Association" in the southern city of Kitakyushu.
Only a small number of Japanese doctors have recognized PPS as a separate medical condition, Ms. Negayama says. "Knowledge of PPS is slowly being disseminated by newspaper articles, by patient self-help groups and by some medical doctors," she says. "The Japanese government has not yet recognized post polio syndrome as a condition that merits special attention."
Polio epidemics ended in Japan by 1963. However, it was not until October 2000 that polio was officially declared eliminated in Japan.
No statistics exist about the total number of PPS patients being treated in Japan. The number of those with PPS probably has not yet peaked in Japan, Ms. Negayama adds.
An estimated 20,000 to 30,000 polio survivors live in Canada. The western province of Manitoba, which has an overall population of about one million, has 3,000 polio survivors, says Ken Pearn, voluntary president of the province’s post-polio network.
Mr. Pearn contracted polio in 1952, when he was nine years old. Initially, he was partly paralyzed on his left side and was hospitalized for 14 days. The disease left him with a residual weakness on that side and curvature of the spine that prevented him from entering the police force, but did not affect him much otherwise. He played baseball, hockey, football and rugby as a teenager and took up rugby again after 20 years. Five years ago, the entire left side of his body became weak.
"I can barely lift my arm over my head now," says Mr. Pearn. "It affects my left shoulder and back. I noticed a gradual weakening. I panicked and bought weights, thinking it was aging. I didn’t realize that I might have been straining myself more. Finally I saw a general practitioner."
The GP sent Mr. Pearn to a neurologist, who considered various neurological diseases, but could not reach a diagnosis. Mr. Pearn finally went to another doctor who had experience with polio. That doctor told Pearn that he didn’t have standard PPS, but said he didn’t know what he did have.
"I know a lot of members of my organization say they cannot get properly assessed without going all the way to Edmonton or Vancouver," Mr. Pearn adds.
Some 40,000 polio survivors live in Australia, says Peshotan Katrak, M.D., deputy director of rehabilitation medicine at Sydney’s Prince Henry Hospital. Dr. Katrak says he has seen about 400 PPS patients over the past six years. The province’s PPS network has some 700 members.
"There were not many doctors seeing polio patients in Sydney, so Prince Henry Hospital started a post-polio clinic," Dr. Katrak says. "Many people have to travel for hundreds of miles to visit this clinic. There are very few post-polio clinics in Australia and they are located primarily in the larger cities. The clinic staff are involved in the care of patients with disabilities related to polio and a variety of other conditions."
Dr. Katrak’s clinic offers a full medical assessment of patients, then refers them to local general practitioners who are experienced with PPS. "I think it is very important for post-polio patients to see someone who understands PPS, who can help them manage their problems," Dr. Katrak says.
Some 100,000 polio survivors live in Germany, says Bettina Beck, M.D., a neurologist at a rehabilitation clinic outside Munich.
Widespread knowledge of PPS came to Germany about 10 years after the United States and Canada. "Around 1989 or 1990, we began to see polio patients with new symptoms but we didn’t know why," says Dr. Beck. "I contacted other doctors in polio societies, and we learned a lot, especially from colleagues in the United States." Dr. Beck attended several PPS clinics in the United States to receive training in the treatment of patients with the condition. Her clinic determined how to treat such patients only some five years later, and then published a paper about PPS in Germany. "Once we did that, we received many calls from PPS patients who said we were the first in our country to recognize the condition. There were many cries for help," Dr. Beck says.
"Neither medical personnel nor health insurance companies have generally accepted PPS," she says. "There are some doubts about it among doctors. Insurance companies refuse to pay for certain modern drugs that help to relieve PPS pain and weakness. They also won’t pay for modern wheelchairs, telling me, ‘That guy can walk, he doesn’t need one.’"
Dr. Beck says many of her patients have gone back to wheelchairs after decades because of PPS. "We encourage them to use wheel chairs," she says. "There is some resistance. They tell us, ‘I fought to get out of the wheelchair and now you tell me to go back in?’"
Polio survivors who hold disability cards -- the only official way to estimate their numbers -- total some 50,000, according to Anita Molines of the French Post-Polio Group. "But we think there are 5,000 to 10,000 more without this card. So the estimate is that there are 55,000 to 60,000 polio survivors in France," she says. No official estimate exists in France of the number of PPS patients.
Polio was not eliminated completely in France until 1982. The first people with PPS symptoms presented in the early 1980s. The main symptoms reported were fatigue, pain and loss of physical abilities.
"French doctors still do not take these symptoms seriously," says Ms. Molines. "Today, most of them don't recognize PPS. Polio was taken off the medical school curriculum a long time ago. However, courses about polio and PPS are scheduled to be taught beginning in September of this year in every university in France."
In Paris, some hospitals or centers have set up clinics for polio survivors, but most are open just one day a month. "We are trying to set up a polio survivors medical network in the Parisian region," says Ms. Molines. "To date, the French government has not recognized PPS as a separate condition."
"No specific treatment is available for PPS patients at the moment," says Ms. Molines. "Generally, doctors are at a loss about how to help us."
The number of polio survivors in the United Kingdom is now put at around 30,000, although it may be greater, says Andrew Kemp of the British Polio Fellowship (BPF). He says many have complained of the poor medical treatment that they get when they develop PPS symptoms.
"I began working for the BPF in 1990 and a relatively small number of members reported new difficulties to us then," Mr. Kemp says. "Gradually, the numbers presenting with PPS symptoms have increased year by year. I know it’s unfair to generalize, but a large number of our members have been disappointed with the care they’ve received."
The UK Parliamentary Under Secretary of State, Yvette Cooper, recently issued an official statement acknowledging the existence of PPS. Some British physicians and other health care professionals readily acknowledge that PPS exists, Mr. Kemp says. However, too many others do not recognize PPS as a real condition. Some health professionals "dislike the term post-polio syndrome," he says. He believes that awareness of the condition among health care professionals is improving, but not fast enough.
"In 1998, when we surveyed our members, 98 percent of respondents thought that they had new physical problems caused by their polio, but 66 percent of general practitioners did not attribute such problems to PPS," Mr. Kemp says.
How to Order Reports
In addition to the free download from the March of Dimes Global Programs website, single printed copies of both reports are available free. Contact the International Polio Network by phone at (314) 534-0475, or by email to [email protected], or in writing to 4207 Lindell Boulevard, #110, St. Louis, MO, 63108-2915.
To order in bulk quantities, contact the March of Dimes Fulfillment Center by calling toll-free in the United States at 1-800-367-6630. There is a fee for shipping and handling. Please order by item number:
#31-1522-01 Post-Polio Syndrome: Identifying Best Practices in Diagnosis and Care (each)
#31-1523-01 Guidelines for People Who Have Had Polio (25 per package)