Consumer Monday June 3, 1991 June 1991 Consumer -- Polio Patients Challenged by New Syndrome Former Polio Patients Challenged by New Syndrome by Evelyn Zamula Though Emily (not her real name) was nearly 10 when she contracted polio, she has almost no memory of her illness, nor does she want to recall it. Her mother told her, "Forget, forget"--and Emily forgot. Glimmerings of an endless summer in the hospital and a machine that helped her to breathe sometimes surface in her dreams. Long after she was well, when she was grown up, her mother mentioned that at one point the doctors didn't know whether she would live or die. Emily made a complete recovery from this sometimes paralyzing disease and was able to block out the memory of those days. But now, 40 years later, unwelcome reminders of her childhood illness are returning. She finds it increasingly difficult to walk, especially when she is overtired--which is often--and to breathe while sleeping. More than half of the estimated 250,000 to 650,000 Americans who had paralytic poliomyelitis 30, 40, even 70 years ago are now reexperiencing some of their old symptoms in what is known as post-polio syndrome. They complain of increasing muscle weakness, joint and muscle pain, fatigue, breathing difficulties, loss of stamina, low back pain, and intolerance to cold. In at least 1 out of 25 survivors, the symptoms are disabling. While some of these complaints may be due to aging and years of abnormal stress on weight-bearing joints in those who needed mechanical assistance to walk, this does not explain the progressive muscle weakness and muscle atrophy (wasting). Although researchers are still not sure what causes post-polio syndrome, most agree with Lauro Halstead, M.D., director of the National Rehabilitation Hospital's Post-polio Program in Washington, D.C.--and a polio survivor himself--who says: "The most widely held theory is that the new muscle weakness is related to overuse of polio-damaged nerve cells in the spinal cord." Polio's Three Strains Thanks to an effective vaccination program, polio has been nearly eradicated in the United States. However, as recently as the 1950s, epidemics terrorized the nation, especially in the summer months, leading to the closing of public pools and other places where people congregated (see accompanying article). Polio is a highly contagious disease caused by a virus that has three distinct strains, called types I, II and III. Immunity to one type doesn't confer immunity to the other two. Type I, the strain that causes the most paralysis, is also the cause of most epidemics. Polio epidemics in temperate climates occurred most frequently in the summer and early fall--the poliovirus flourishes in warm weather. Children were more often affected than adults, which is why the disease was once known as infantile paralysis. Persons at greater risk for serious neurological damage during epidemics due to lowered immunity included those who had recent inoculations or recent operations, especially removal of tonsils and adenoids (because the virus enters through the mouth and multiplies in the throat). Pregnancy also predisposed to paralytic polio infection. The virus is found throughout the world, but primarily in undeveloped countries with insufficient immunization practices. It is excreted in large amounts in the feces of someone who has polio or is recovering from it, and is probably spread through hand-to-hand or hand-to-mouth contact. The poliovirus enters the mouth and multiplies in the throat and intestinal tract. Viruses may cross from the intestinal tract into the bloodstream. They are carried to the spinal cord, where they may kill or transiently injure motor nerve cells that control skeletal muscles, causing paralysis. Sometimes only a small group of muscles is affected, sometimes the paralysis is widespread. The legs are affected more often than the arms, but polio may partially or completely paralyze a single limb, one half of the body, even all four extremities. If the virus gets into the brain stem (bulbar polio), it can paralyze muscles that control breathing, swallowing, and other bodily functions. Most fatalities occur among those with respiratory paralysis. Post-Polio Syndrome Only one or two out of every 100 people infected with the polio virus develops an acute paralytic illness. In many cases, the paralysis is only temporary. Up to six months to two years after the acute attack, the muscles may recover to normal strength. The nerve cells that were undamaged or partly damaged take over the function of the dead cells by sending new sprouts to muscle fibers that have been "orphaned," thereby improving muscle strength. The theory is that after years of this extra load, cells of polio survivors tire and rebel, resulting in new weakness both in muscles formerly affected and in those that were originally unaffected. As the muscles lose nerve stimulation, they begin to atrophy. In some cases, polio survivors must begin to wear braces again, or return to wheelchairs. Those who had bulbar polio often need devices to help them breathe. These include several types of portable ventilators or, in more serious cases, the tank ventilator or "iron lung"--a large, stationary tank that encloses all but the head in a chamber that "breathes" for the patient by negative pressure. The new symptoms are more common among post-polio survivors who had the worst cases initially--those who needed hospital care, or were older than 10, or had paralysis in all four limbs, or needed mechanical assistance to breathe. One fact that doctors are sure about is that post-polio syndrome is not a new infection, nor is it a reactivation of the old infection. Drug Treatment To relieve the pain of post-polio syndrome, doctors may recommend nonsteroidal anti-inflammatory medications, such as aspirin and ibuprofen (Advil, Motrin IB, Nuprin), and heat applications on the affected area. Narcotics are avoided because they depress the central nervous system and may adversely affect breathing. A study in progress at the National Institute of Neurological Disorders and Stroke under the direction of Marinos Dalakas, M.D., chief, neuromuscular diseases unit, is evaluating the effects of prednisone (a synthetic compound that has the same actions as a substance called cortisol produced by the adrenal gland) on patients with post-polio syndrome in a double-blind study, in which neither the investigator nor the patient knows who is getting the drug and who is getting the placebo. The current study is based on data from an ongoing study conducted since 1983 that led Dr. Dalakas to hypothesize that the immune system may be involved in the syndrome. "We have found a number of irregularities in the immune system of post-polio patients, which is why we're using prednisone in this study," says Dr. Dalakas. "We don't know if these changes are responsible for the manifestation of muscle weakness. However, the use of prednisone as an agent that can modulate [affect] the immune system, as well as act as an anti-inflammatory agent, may prove to be effective in relieving some symptoms of post-polio patients." The immune system fights infections by attacking bacteria and viruses that invade the body, but it can also attack parts of the body itself, causing what are known as autoimmune diseases. Prednisone can suppress a component of the immune system and is often helpful in relieving muscle weakness and pain in muscular inflammatory diseases, such as polymyositis (inflamed muscles). Post-Polio Management In treating the post-polio person, the doctor must first rule out or treat other conditions that may be causing loss of muscle function, especially as they affect breathing capacity. This includes a thorough medical history and physical examination, as well as testing by electromyography to determine which nerves and muscles are affected. Muscle strength should also be evaluated annually by a physical therapist. The doctor and physical therapist must also review any medical devices or adaptive equipment currently being used by the post-polio patient. The patient may need a higher level of assistance, whether for walking and daily activities, or for breathing. To reduce fatigue and stress on muscles and joints, it may be necessary for the patient to lose weight. However, maintaining an adequate and nutritious diet is also important. Non-fatiguing exercises should be substituted for any exercise that increases weakness and muscle fatigue. Rehabilitation experts recommend gentle muscle stretching exercises and swimming, which is the best general conditioning exercise for former polio patients. Changes in lifestyle may be useful in preventing overuse of muscles. That may mean adjustments in leisure-time activities, as well as on the job. It's essential for the post-polio patient to avoid becoming overtired. An upbeat mental attitude also helps. Joyce Oakes, of Rockville, Md., who was paralyzed from the waist down at age 6, tells her story: "I didn't have a chance to feel sorry for myself when I had polio, because at that young age, I couldn't analyze what had happened to me. I did have to learn to crawl and walk again, even though I was told that I never could. And I learned to dance and to ice skate. I think I was a better competitor because of it. "I also came from a family that couldn't afford to send me to college, so I put myself through college on scholarship. Having to learn to walk again and do other things, I became an overachiever in some ways, like many other polio people. And when I encountered unhappiness, things that didn't go my way in later life, I think I weathered them better, with a better perspective and a sense of humor. I tried to accept adversity and find a way to make it work for me, rather than wallowing in it. In many ways I don't consider it [polio] a hardship in my life. "What I don't like is that in the past year I find that I am losing my strength. I can't ice skate any more, I can't go hiking. But you don't give in to things like that, you don't dwell on it. As you experience this weakness, you learn to pace yourself, conserve your stamina. You play the cards you are dealt, and get on with your life as best as you can." n Evelyn Zamula is a freelance writer in Potomac, Md. Vaccine Conquers Killer When vaccines against polio were developed in the mid-1950s, they spelled the end of a disease that became more disabling, deadly and feared as time went by. Though polio was not unknown in ancient times, the disease wasn't mentioned much in the medical literature throughout the ages and did not occur in large epidemics until modern times. In fact, it was only in the late 18th century that the disease was first identified as polio. Today, polio is once more an insignificant disease in the United States and advanced Western societies. Most American doctors have never seen an active case of polio. But in the first half of this century, polio was called the last of the great childhood plagues. How polio emerged from centuries of obscurity to becoming a killer just a few decades ago has to do with sanitation--and the lack thereof--and the fact that the poliovirus is excreted in the feces up to six weeks after infection. In less hygienic times--when the contents of chamber pots were blithely tossed out of windows, open sewers fouled the street and outhouses stood in the backyard--there was plenty of opportunity to contract polio. Polioviruses infected each new generation of babies, who were protected in part by antibodies passed on to them by their mothers. These infections early in life were usually mild and non-paralytic, sometimes appearing with cold-like symptoms, sometimes with no symptoms at all. They were often indistinguishable from a host of other childhood diseases, and were rarely diagnosed as polio. Ironically, cases of paralytic polio began to rise when improved public sanitation and other health measures, such as purification of the water supply and milk pasteurization, were put into effect in economically advanced countries. With better hygiene, there was less chance for babies and young children to contract the mild form of the disease and acquire immunity. When the disease struck older children or adults, it was more likely to take the paralytic form. In northern Europe and the United States, small epidemics of paralytic polio began to appear in the late 19th and early 20th centuries. However, polio's full impact wasn't felt in the United States until the summer of 1916, when 27,000 people were paralyzed, with 6,000 deaths. The Northeast was particularly hard hit--in New York City and the surrounding suburbs, more than 9,000 cases were reported, with 2,448 deaths. Many attributed these cases to the large immigrant population. The 1916 epidemic caused widespread panic. Thousands fled the city to nearby mountain resorts. Movie theaters were closed, meetings were cancelled, public gatherings were shunned. Doctors postponed tonsillectomies (then a common operation for children) until cooler weather when the epidemic abated. Children were warned not to drink from water fountains; amusement parks and bathing beaches were off limits. In some towns, visitors from the New York City area were turned away by armed citizens who feared the spread of contagion--shades of the 14th century's Black Plague. Compared to the flu epidemic that killed 500,000 Americans just two years later, the 1916 polio figures seem modest, but polio was especially frightening because it could cripple. A life in a wheelchair, or in an "iron lung," was a fate to be feared. An epidemic appeared each summer thereafter in at least one part of the country, with the most serious occurring in the 1940s and 1950s. The 1952 polio epidemic was the worst in our nation's history. Nearly 58,000 cases were reported that year; 3,145 Americans died and 21,269 were left with mild to disabling paralysis. More children died of polio in 1952 than of any other infectious disease. Vaccines did for polio what they had done earlier for other childhood diseases, such as whooping cough and diphtheria. The 1954 field trial sponsored by the National Foundation for Infantile Paralysis, in which 1.8 million children participated, proved that the polio killed virus vaccine developed by Jonas Salk, M.D., was highly effective in preventing polio. Americans were jubilant, and Dr. Salk became a national hero. Following the licensing of the Salk vaccine in 1955, an intense public health campaign was mounted to inoculate all American children. Newsreels of the time show long lines of school children patiently waiting to get their shots. Similar scenes were repeated in 1961 when the attenuated live virus vaccine developed by Albert Sabin, M.D., was licensed, only this time the children were given a sugar cube soaked in the liquid vaccine. Polio was virtually eliminated in the United States in a few short years. Both vaccines contain all three virus strains and effectively prevent polio. The federal Centers for Disease Control in Atlanta recommends vaccination with the attenuated live virus vaccine for primary immunization of children in this country, because its liquid form is easy to administer and well accepted by children. It is supplied to doctors in single dosage forms that can be administered in a number of ways, including directly into the mouth with a dropper (the most common way for infants); mixed with distilled or chlorine-free tap water, simple syrup, or milk; or placed in foods, most commonly sugar cubes. It also blocks implantation of the virus in the intestines, where it multiplies. The killed virus vaccine requires a series of injections, which promotes more anxiety among youngsters. However, many countries use the injectable vaccine with great success. On average, fewer than 10 cases of polio a year were reported from 1975 to 1990. Some of these cases have been associated with the administration of the attenuated live virus vaccine, according to CDC. Other cases have been brought into the country by immigrants or visitors from abroad. The last U.S. polio epidemic occurred in 1979, when 10 Amish children, whose parents had refused to have them vaccinated on religious grounds, came down with the disease. Worldwide, the World Health Organization estimates that 250,000 cases of paralytic polio occur each year. Many health professionals believe polio can be completely eradicated through vaccination, as was smallpox in 1977. WHO has set the year 2000 as a goal for worldwide eradication of polio. However, Donald A. Henderson, chairman of the Pan American Organization's Technical Advisory Group on Immunization, believes this objective will be achieved only when ongoing research produces a vaccine that is stable in high heat (polio vaccines deteriorate in hot climates unless they are refrigerated) and that can be given in a single dose rather than three to ensure patient compliance. --E.Z. Recommended Polio Immunization for Infants Primary Series - First dose: when baby is 6 to 12 weeks old (often given with first DTP inoculation at 2 months) - Second dose: not less than 6 and preferably 8 weeks after first dose (commonly given when baby is 4 months old) - (In areas where polio is common, an optional dose also may be given at 6 months of age.) - Third dose: 8 to 12 months after second dose. Booster Upon entering elementary school.