Deseret Morning News, Monday, June 27, 2005

Post-polio syndrome

In some patients, polio has a lingering legacy

By Lois M. Collins
Deseret Morning News

Steve Nixon woke up crying on his sixth birthday because he could not move and he didn't know why. It was July 1953 and he'd just become one of the millions stricken by poliomyelitis.

Steve Nixon is in pain if he stands very long or sits very long. And he hurts if he walks too far. August Miller, Deseret Morning News

In the years to follow, he would endure the teasing of kids who called him "four legs" because he used crutches in early grade school, graduating to lifts in both shoes by fifth grade. He would give up his dream of sports as anything but a spectator. And he would meet a doctor, at 14, when he was doing much better, who would tell him to choose work where he would not have to be on his feet all the time.

That same doctor predicted that at some point in the future the effects of the polio would resurface hard.

The polio vaccine turned 50 this year, a medical milestone that stopped polio cold in the United States, though it's still devastating people on some continents, most notably India and Africa. The battle to wipe the virus from the face of the earth is falling a little behind where many health experts expected to be a half century after that first shot was fired — into the arm of someone desperate to avoid the debilitation of polio.

Although polio is no longer a threat in America, its legacy lingers. And just as that doctor predicted, many of the people who survived it decades ago are experiencing an aftereffect that is both frustrating and debilitating.

It's called post-polio syndrome and it is believed that as many as half of polio survivors will experience it anywhere from 10 to 40 years after they recovered from the polio virus, according to the National Institute of Neurological Disorders and Stroke.

It is felt most often as a further weakening of the very muscles that were first affected by the polio infection. But there's more, an unpredictable mix of symptoms that may include fatigue, difficulty swallowing, slowly progressive muscle weakness and, sporadically, muscular atrophy. Joint pain and the development of skeletal deformities like scoliosis are common. In rare cases, the patient develops something that resembles but is not amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease).

Steve Nixon, who contracted polio when he was 6, is now suffering an aftereffect — post-polio syndrome. August Miller, Deseret Morning News

The severity of PPS seems to depend on how hard the polio hit the individual originally. Those who had mild symptoms are likely to have mild PPS symptoms. Those hit hard by the virus, many of whom were left with severe and lifelong residual weakness, may also be hit hard by the post-polio punch.

That's the reality for Yvonne Failner, who contracted polio when she was 10. One minute she was out playing in the yard, the next she was so exhausted she put herself to bed in the middle of the day.

Her polio stabilized and her life went on, marked, like Nixon's life, with marriage and children and work and play.

Today, to travel more than a few yards around her house, she uses a motorized scooter. Twelve years ago, she started having more trouble walking, was tired and achy. At first doctors thought it was arthritis. It took a while to realize the symptoms are those of post-polio syndrome. The effects were serious enough, her husband Vaughn says, that they had to move to a more accessible house.

The most disconcerting symptom he sees among his patients is weakness in the limbs or muscles that were formerly strong, says Dr. Mark Bromberg, a neurologist at the University of Utah who specializes in nerve and muscle disorders, including post-polio syndrome.

There's some debate about what precisely post-polio syndrome is. There's no question, though, about what it is not. "It is clearly not polio revisited," says Bromberg. "What is it? That's harder to answer."

One strong theory is that nerves that go to the muscle are damaged by the polio virus and a number of them die. That means the surviving ones may have to work harder, doing more. Over time, even without polio, everyone loses nerve cells. Once someone reaches age 65, as many as half the nerve cells that go to the muscles may have been lost just as part of a natural aging progression. But when you add normal aging to the initial loss from polio, the result may be the syndrome that is causing so many people trouble, Bromberg says.

Because of a hip problem, Steve Nixon uses a cane to walk and often holds onto something for support and to take a little weight off his legs. August Miller, Deseret Morning News

Another theory is that metabolically taxed nerve cells may have occasional failures and even a permanent failure of some of the branches.

"Keep in mind that some of these things are not unique to polio," he adds. Other medical problems can cause nerve damage and the same kind of symptoms. "The whole post-polio syndrome, the things we've told you about, really are not absolutely unique to polio."

Many health experts point out some similarities between post-polio syndrome and chronic fatigue, for instance.

Diagnosis is a matter of making sure that something else is not going on, Bromberg says.

It may be a shoulder tear, a torn rotator cuff, carpal tunnel syndrome, spinal disk disease, nerves being pressed upon by disks. There are many tools that can be employed to determine what's happening — or, in the case of post-polio, what isn't. They include magnetic resonance imaging, neuroimaging, electrophysiological studies, muscle biopsies or spinal fluid analysis. If everything else is excluded and the patient had polio decades ago, the likely diagnosis is post-polio syndrome.

The doctor's job is to listen carefully to the patient, Bromberg says. "If we don't find any superimposed explanation," he reassures his patients that "this is not polio coming back. And it is not the beginning of a degree of further deterioration." Then a physician tackles treating the individual symptoms, which vary patient to patient.

For discomfort, that may mean prescribing a non-steroidal anti-inflammatory drug like ibuprofen, or even something stronger in severe cases. Physical therapy is used, not for strengthening but for stretching. And if someone has gained weight as time has passed, which is common, "it may be worthwhile to help them reduce a little weight."

Research is ongoing for a treatment that would effectively combat the fatigue and other symptoms. So far, most study results have been somewhat discouraging, the proposed treatments ineffective or not very effective.

Nixon always had trouble walking after polio. His leg was weak and sporadically "jittered." He stumbled a lot. As a result, even Little League rejected him.

By high school, though, he had mostly recovered. Kids no longer teased him and he didn't talk about the polio. Despite the virus' epidemic reach, he knew only a few people who had it.

In 1990, he developed a pronounced hip problem that was later diagnosed as post-polio syndrome. For a long time, he says, "I was in some denial."

If he sits very long, it hurts. If he stands very long, it hurts. If he walks far, it hurts. He often uses a cane or grabs onto fixed objects for support.

He was a custodian at a school for 25 years, finally benched by the post-polio. In his 50s, he has taken a medical retirement. These days, he's given up riding his beloved motorcycle and no longer buys season tickets to see the Utah Jazz. It's just too hard to get into the arena and move around.

Winters are hard on him; cold often bites someone with post-polio very hard.

Although deterioration feels rapid to the person who's experiencing it, post-polio syndrome deterioration is actually very mild and very slow, Bromberg says. The National Institute of Neurological Disorders and Stroke says its research has shown that PPS "is a very slowly progressing condition marked by long periods of stability."

Many people who have post-polio are under the impression they can't exercise, that doing so simply burns what life is left in the nerve cells and the muscles. And that's the message from some doctors, while others teach just the opposite. There's little question that the extra effort expended by the nerves and muscles that survived the polio virus in the first place taxed them. Still, the institute recommends a common sense approach. Its own studies have found that exercise is safe and helpful, but should not be overdone. When one starts to experience discomfort or fatigue, it's time to ease up. "No pain, no gain" is not valid with post-polio.

There's no treatment that reverses post-polio syndrome, Bromberg says. But he believes that the pain, fatigue, depression and sagging spirits that may go with those who have PPS syndrome can be reversed.

Mia Farrow is perhaps the best-known of those who publicly talk about post-polio syndrome. The actress is very open about the muscle weakness, fatigue and difficulty sleeping that now bother her decades after she contracted the polio virus.

She's a co-author of the "Post-Polio Letter," a campaign designed to provide basic information to doctors who don't specialize in it and may not know much about post-polio syndrome.

Physicians most likely to be well versed in the condition, Bromberg says, include neurologists who focus on nerve and muscle disorders and physicians who specialize in rehabilitation or are affiliated with a department of physical medicine and rehabilitation.

Finding someone well versed in the syndrome is just one more frustration for patients living with an already-frustrating syndrome, he says.

More information on post-polio is available online, including a summary by the National Institutes of Health at http://www.ninds.nih.gov/.