It reads like a script from Fox TV’s “House M.D.” Inexplicable fatigue, new muscle weakness and muscle pain. Sometimes muscle atrophy, muscle twitching, sleeping and breathing difficulties, difficulty swallowing, speech disturbances, intolerance to cold, joint pain, decreased attention and concentration, scoliosis. One by one, tests rule out osteoarthritis, fibromyalgia, hypothyroidism, depression, rheumatoid arthritis, polymyalgia rheumatica, ALS. Doctors convene in a small room with a dry-erase board and put their heads together. Later, a chance find on the patient’s medical history reveals a clue: childhood polio. Doctors interrogate the patient about the previous diagnosis of polio, the interval after recovery and the speed of the onset of new symptoms.
Did You Know? The March of Dimes was founded in 1938 to combat epidemic polio in the United States. With the development of the Salk and Sabin vaccines by the early 1960s, the organization realized its mission. Though still participating in some polio-related issues, the March of Dimes changed its focus to improving the health of babies by preventing birth defects and infant mortality. In 1999, the foundation convened an international steering committee to address the needs of polio survivors and post-polio syndrome.
After eliminating all other possible causes for the symptoms, the diagnosis is revealed as post-polio syndrome (PPS). But remove the bright stage lights, exit stage left, brush off the glamour of TV, and a PPS diagnosis does not quite save the day.
For the estimated 10 to 20 million polio survivors worldwide, based on figures from the World Health Organization (WHO), uncertainty abounds as no test exists to diagnose PPS, the management of PPS sparks controversy and even the cause stumps doctors. Determining who is at risk seems to depend greatly on the severity of the original polio infection and the extent of recovery. And once PPS is diagnosed, the process of finding the best management option could worsen symptoms, especially since the knowledge of post-polio has spread slowly among the medical community.
What is known is PPS is not a disease. It is not degenerative. It can be managed. It is not fatal in most cases. Not everyone who had polio as a child will have post-polio. PPS is not “getting polio” a second time.
WHO estimates that an average of 40 percent of polio survivors who thought they were clear of the disease may eventually exhibit the symptoms of post-polio. According to Post-Polio Health International, a non-profit organization headquartered in St. Louis, up to 250,000 people in the United States may currently have PPS. And slowly but surely, PPS will limit a person’s mobility as muscles affected by polio slowly lose function.
What Do You Mean By ‘Post’-Polio?
The first case of new muscle weakness and atrophy many years after polio was reported in 1875, according to Post-Polio Health International, but it wasn’t until the late 1970s, approximately 30 years after the U.S. polio epidemic, that polio survivors started reporting post-polio symptoms in droves. The March of Dimes cites that PPS came to the wide attention of the medical community in the 1980s.
Though research is continual on the late effects of polio, the cause is still unknown. Most sources explain that individuals who experienced severe symptoms with the greatest amount of recovery are most at risk of getting the late effects of polio. Medically speaking, the symptoms seem to be brought on by exhausted muscle nerves.
Holly Wise, PT, Ph.D. Education, Rehabilitation Sciences, at the Medical University of South Carolina at Charleston, explains that the original polio virus in some polio survivors infected select motor nerves, destroying the connections and causing muscles to be “orphaned.” The destroyed motor nerves presented as paralysis. In these cases, surrounding motor nerves that were still functioning sprouted or sent out collaterals to recover the lost motor nerves. In this way, the muscle was reconnected to the lost nerves and able to regain function. The solution was effective, but over time, the overburdened nerves began to wear out, resulting in new muscle loss, or post-polio syndrome.
“It’s normal for all humans as we age. We sometimes have motor nerves die, but then we have collateral sprouting as well,” says Dr. Wise. “But we’ve got thousands to draw on. Individuals that had polio, depending on the extent of the polio, don’t have the same number because the virus killed many motor nerves. So, now if you just have normal aging, it’s much more dramatic with polio survivors than it is for you and me. You and I will lose 1 percent after we turn 30, we lose about 1 percent each year over time, but … a polio survivor who’s operating with 50 percent to begin with and then you have aging, it’s more dramatic.”
Recent research, according to Post-Polio Health International, shows that normal average decline of muscle strength in persons older than 50 is 1 percent per year. The rate for post-polio survivors is estimated at 2 percent per year.
As a result of new muscle loss, people with post-polio syndrome may experience difficulty walking, lifting, standing, bending, climbing stairs — or any activity that requires repetition or endurance. Those who experienced respiratory problems with the original bout of polio may experience new respiratory problems, such as sleep apnea, as respiratory muscles weaken. Depending on the extent of the original polio symptoms — if a person used an iron lung as a child — weakened breathing muscles might eventually lead to respiratory failure in adulthood and death if left untreated, says Joan Headley, executive director, Post-Polio Health International. Generally, a person experiencing respiratory-related problems as a result of post-polio requires a bi-level positive pressure device to assist with breathing, according to Post-Polio Health International. By contrast, a CPAP device or treating with oxygen therapy instead of assisted ventilation could lead to respiratory failure and death because supplemental oxygen can blunt the function of the brain’s respiratory control center. In more severe cases, a tracheotomy might ease breathing.
Problems with attention, concentration, cognition or behavior can also occur. Researchers have linked these symptoms to prior polio infection involving the brain and also traumatic psychological experiences associated with acute polio infection that were revived, cites Post-Polio Health International. Despite these theories, the cause for such symptoms is still unknown.
The National Institute for Neurological Disorders and Stroke states that post-polio is not getting polio again, nor is it a form of ALS. Medical evidence shows that the polio virus cannot cause persistent infection in humans.
Through the Eyes of Survivors
What Can Be Done?
Currently medication is not available to treat post-polio. The treatment, unique to the individual, involves managing the symptoms to minimize the stress to muscles. Typically, people with post-polio may use mobility aids and ventilatory equipment, avoid activities that cause pain and/or fatigue that lasts more than 10 minutes, pace daily activities, and incorporate lifestyle changes such as eating well, getting the proper amount of sleep, exercising appropriately and avoiding unhealthy habits like smoking and overeating, says Headley. In addition, joining a support group can also help a person adjust to the dramatic change in lifestyle necessary to manage post-polio.
Not every person with PPS will need a mobility aid, says Dr. Wise. Generally, a PT makes that determination by conducting a baseline muscle test and a gait assessment. The muscle test, which rates muscle strength on a scale of 1 to 5, 4 and 5 being someone with functional strength, pinpoints exactly which muscles were affected by polio, says Dr. Wise. The test can help health care professionals determine the most beneficial equipment for a person and also which muscles need to be spared in a fitness program.
“Some polio muscles that test at 4 or 5 one year, in a year or two may have a change,” says Dr. Wise. “And that’s due to aging with a diagnosis of poliomyelitis (polio), aging with a disability.” Therefore, it is important that a person experiencing the late effects of polio be tested often to chart the progression and make adjustments to best manage the symptoms.
Some controversy exists regarding the role of exercise with post-polio. Dr. Wise says that exercising a muscle affected by polio can worsen the symptoms of PPS. For that reason, it is vital that a person with PPS have a muscle test to determine which muscles are affected. “Typically for you or me who haven’t had polio, if we have a weak muscle and we do exercise, we can strengthen it,” says Dr. Wise. But if muscles affected by polio are exercised, those muscles become fatigued and eventually functional strength in those muscles is lost. On the other hand, says Dr. Wise, “if a polio survivor doesn’t exercise at all, they can also lose muscle strength because not every single muscle in their body was affected by polio.” Therefore, it takes a creative fitness program to address the needs of PPS clients.
In addition to evaluating muscle strength, Dr. Wise says it’s important to pay attention to co-morbidities. People with post-polio are still susceptible to conditions associated with aging. “Many times people have weakness and fatigue, not from their polio but because they have diabetes, thyroid (problems), other things going on,” she says. “If they’re effectively treated, they may not require a mobility aid.” Medications used to treat co-morbidities also may cause weakness and fatigue, according to Post-Polio Health International.
Before looking at a scooter or power chair, Dr. Wise says other mobility devices, such as canes, walkers and crutches, should be considered for effectiveness first. Choosing between a scooter and power chair requires special considerations. “When I choose between a scooter vs. a power chair,” says Dr. Wise, “I have to look at the posture of the individual and if they’re able to get out of it for long periods of time or not. Because if someone’s able to get up and move around — let’s say, on Lofstrand crutches or whatever — a scooter might be fine. But if someone is unable to be ambulatory, many times a power chair has better posture for full-time positioning and greater options.”
Sometimes the mobility aid can contribute to the progression of post-polio symptoms, especially if the equipment causes some type of muscle fatigue. For example, getting in and out of scooters can cause significant back pain, says Dr. Wise. Also, steering a scooter might cause strain on the upper body. Likewise, canes, walkers and crutches support leg movement, but put more demand on upper extremities, cites Post-Polio Health International. Choosing the proper device can alleviate some of this strain, but a muscle test is essential for determining if these devices are appropriate.
Manual wheelchairs can also cause problems for PPS clients. “Some manual chairs cause breakdown of the shoulders, elbows, wrists and hands if somebody has to propel using their arms full time,” says Dr. Wise. Therefore, manual chairs can speed the deterioration of muscle strength.
Oftentimes, supportive bracing or orthotics coupled with a mobility aid are the best choice, says Dr. Wise. Orthotics target pain and fatigue and improve stability — the top three complaints of people with post-polio syndrome, she says.
A power chair, on the other hand, is the least demanding option for the upper extremities, according to Post-Polio Health International.
When deciding on a mobility aid, the solution must fit individual needs. For example, says Dr. Wise, “Right now I have an individual who is unable to lift her arms up over her head, and she wants to be active at home as a homemaker. So we’re going to have [a device] with an elevating seat so she can put some of her dishes up on counters that she couldn’t before. She can’t lift her arms up over her head but she can get to 90 degrees. So, that will help her. I have a gentleman who really should be in a power chair. He doesn’t want one because his house … won’t allow him to use his kitchen and he’s not ready to redesign his kitchen.” Dr. Wise says finding a solution must be a partnership among the individual, the physician or physical therapist, the orthotist and the mobility dealer.
What’s the Future?
When post-polio syndrome was still new, many treating physicians were not aware of its existence. Currently, knowledge of post-polio syndrome among the medical community is better than it was 20 years ago, says Dr. Lauro S. Halstead, director of the Post-Polio Program at National Rehabilitation Hospital, Washington, D.C., mainly due to published articles in journals, public speaking among the medical community and awareness campaigns from such organizations as Post-Polio Health International and the more than 300 support groups across the country.
Still, a general physician might not recognize the symptoms of post-polio or even know about post-polio. Typically, a neurologist or a specialist in rehabilitation medicine makes the diagnosis, says Dr. Halstead. The general public might not be aware of post-polio at all, unless they are involved with the medical community or have personal experience with the condition.
“Not too many lay groups are interested in hearing about [post-polio] because the acute illness doesn’t really threaten people in this country anymore,” says Dr. Halstead. “So, you have to say polio is off the radar screen for most people.”
Though no medication or cure is currently available, the National Institute for Neurological Disorders and Strokes suggests that future treatments may focus on nerve growth factors to generate new nerve sprouts.
Until then, however, the condition can only be managed with a dedicated team working to benefit the individual needs of a client.
For management to be successful, mobility dealers must be involved in that team.
Bottom line, says Dr. Wise, “the more informed the mobility dealers are, then the more effective they are.”
Through the Eyes of Survivors
Sunny Roller was diagnosed with polio at age 4 in 1952. Describing that first bout, she says simply, “It almost killed me.” After the help of extensive rehabilitation and learning to walk all over again, she enrolled in first grade with braces, including a back brace, crutches and a small wheelchair. She endured five orthopedic surgeries before she was in eighth grade and continued physical therapy until she was 18. In adulthood, Sunny relied on crutches and braces and had an obvious disability. Despite these challenges, Sunny persevered, attending mainstream school, earning a teaching degree, teaching high school for 10 years and then returning to school to earn a master’s degree.
“When I was in my early 30s … I contracted the late effects of polio,” says Sunny. “And, of course, right at that time in the early 1980s, it was all just coming to the forefront, the whole socio-political-medical issue of the late effects of polio. They had no name for it even. And so one thing led to another and I ended up at the University of Michigan Post-Polio Clinic.”
Her first symptom was pain. “It got to the point where I couldn’t walk very well because my whole left side was in new pain, and I didn’t know what was going on,” says Sunny. “And weakness and fatigue sort of accompanied that pain, and so I ended up at the University of Michigan Hospital (where she was diagnosed). … I went to five different physicians, and they all told me something different. They thought it was my side; they thought it was arthritis. They just couldn’t help me.”
A couple of years after her diagnosis, Sunny began using a scooter for long-distance mobility, such as getting around her community. She also began using a manual wheelchair at home when she wasn’t wearing braces. Because of polio, she developed sleep apnea.
“It’s just ongoing management of a disability, and you just have to be kind of vigilant about what’s going on with you,” she says. “Probably the most recent thing I did was I got a new set of crutches that are more lightweight to make it easier for me to walk.”
Currently Sunny is involved with research at the University of Michigan. As a research associate and program coordinator, she studies various disabilities, including polio.
“I think what’s made my experience better has been being really fortunate enough to have connected with a post-polio physician who was really committed to the work,” says Sunny. Another thing that helped her was getting involved in a local support group. “It feels like you just have a great big family out there that you can contact when you need to, and people are lovely and are very supportive when you need help especially.”
Art of Breathing
On Sept. 1, 1953, at the age of 13, Larry Kohout was admitted to Sister Kinney Institute in Minneapolis with polio. According to Larry, it was also the same day Dr. Jonas Salk inoculated his family with the first polio vaccine.
After playing football in the hot August sun, Larry developed what he thought to be a migraine headache accompanied by aches in his back and neck. Larry was admitted to Sister Kinney, where he experienced paralysis from mid-chest down and involvement in his arms and shoulders. Respiratory difficulties, particularly swallowing problems, also forced him into an iron lung. Despite the extent of the polio effects, Larry made a significant recovery and left the hospital in May of 1954 — approximately eight months later — with only a limp.
“My wife said … [the physical therapists] build muscle and Type A personality. Use it or lose it. Push, push, push,” says Larry. “The thing is that for many of us, much of that was successful.” He was in contagion for approximately three weeks of his eight-month stay at Sister Kinney. The rest of the time was spent in physical therapy doing stretching exercises three times a day, six days a week.
“One of the things that the polio does is,” says Larry, “as the muscles are damaged, the tendons shrink up, and all of that has to be stretched back out again in order to regain some type of flexibility. And then you go through balance training, and you go through muscle training, trying to rebuild muscles. Some muscles will begin to respond, and other muscles are just so damaged that they don’t respond at all. … It is extensive physical therapy, and that’s what most of the hospitalization stay is all about.”
The weakened left leg presented problems for him during growth spurts in his teens. Larry eventually wore a long-leg brace.
More than 20 years after his recovery, Larry collapsed to the ground one day while walking with his wife, Cathy, to their car. “All of a sudden, I’m not sure yet whether it was my knee or my ankle, but something let loose and I was down in the grass,” says Larry. “I remember looking up, and Cathy wasn’t really aware of it. She’d taken four or five more steps. And then she did a classic Buster Keaton double-take. … And I didn’t know what had happened. But that started to happen more and more.”
Despite several tests, the doctors couldn’t figure out what was happening either, aside from muscle atrophy. A year and a half later, Larry’s knee no longer locked.
Post-Polio Health International helped solve the riddle when an article published by the organization in the ’80s caught the attention of Larry’s wife. Larry then made the connection between his symptoms and polio. Armed with new information, Larry consulted with his physician, who scheduled an appointment with a local physiatrist whose father had polio. The physiatrist immediately diagnosed Larry with post-polio.
But Larry continued to push himself, much as he had done during his recovery from polio. As a result he became more fatigued, sometimes staying in bed for two to three days at a time after a particularly stressful event. Larry moved into semi-retirement in 1996 and finally retirement in 1997. After his retirement, he started using a power chair.
Larry’s research was invaluable to his diagnosis, and later the management of post-polio. “Earlier I was in such denial that [the research] didn’t help a whole lot [with the management],” he says. “Probably, I would say from ’80 to ’90 I ignored most of what I read and just kept on going. By ’90, I was in bilateral long-leg braces. … My scoliosis was significantly worse, and I was needing to use the back brace to have any relief from the pain of the scoliosis, and it was getting pretty clear that if I didn’t start listening to Dr. [Anne] Brutlag (his physiatrist), I was just going to really mess myself up big time. And I started to back off.”
After 1999, dangerous post-polio symptoms came to the forefront. Though Larry had probably experienced respiratory problems for a while, he didn’t seek treatment until he began pausing in mid-sentence to catch his breath. Proposing to his doctors that post-polio was to blame, Larry again went through a number of tests before the doctors agreed. Initially, Larry was diagnosed with sleep apnea and used a bi-PAP. Later, his breathing deteriorated further, and he was unable to breathe involuntarily. The doctor prescribed a volume ventilator, and later Larry underwent a tracheotomy, a successful procedure to regulate his breathing.
After years of struggle, Larry is as independent as he can be. The strategic placement of essential devices within reach from his power chair enables him to be left alone, as does the remodeling of his condo to make it wheelchair friendly. Larry attributes his success to his wife, Cathy, who has been instrumental in Larry’s management of post-polio. “Without Cathy …” he begins, “You know, Joan Headley (of Post-Polio Health International) asked, ‘Do you have a caregiver?’ And I started to say no, and then I thought, Oh my God, Cathy is my caregiver.” Without Cathy, Larry never could have mastered, what he terms, “the fine art of breathing.”
Lincolnshire Post-Polio Network
The site features a library of more than 100 articles on post-polio syndrome, a member’s network with a newsletter and a directory of resources.
Post-Polio Syndrome Central
Accessible Web site promotes e-mail discussion lists and Usenet news groups on PPS.
Post-Polio Health International
In addition to comprehensive information on post-polio syndrome, the Networking section includes a “Can You Help?” section to send questions and receive answers from online readers on everything from equipment to jobs, a section on peer advice and a directory of support groups listed by state.