Easter Seals Colorado - Post Polio - Poliomyelitis revisited

An estimated 75,000 polio survivors are expected to suffer new or reoccurring muscle weakness, joint pain, and severe fatigue resulting from chronic overuse of polio-weakened muscles. A history of polio is not always the best clue, because many patients suffering other neuropathic disorders were misdiagnosed at the time of the polio epidemics. A patient who has a clear history of a febrile episode associated with paralysis, was in an iron lung, or recalls experiencing hot packs is likely to have contracted polio.

Vaccines have reduced the number of cases of paralytic polio in the United States from 28,000 in 1955 to today's average of 10 cases per year, but the polio monster is again rearing its ugly head. Studies estimate that 25% of the 300,000 survivors of the U.S. polio epidemics of the 1940's and 1950's will experience one or more of the following: Muscle weakness, joint pain, fatigue, muscle pain, or sleep and breathing problems - the symptoms of late effects of polio, also known as the "post-polio syndrome." Although the U.S. Public Health Service recently reevaluated its long standing polio immunization policy to prevent the rare case of polio in an adult who has contact with an infant or child immunized by the live-virus oral vaccine, experts are just beginning to deal with the subtleties and complexities of the post-polio syndrome (see " A new effort to prevent vaccine-associated polio").

The development of post-polio syndrome appears to be time related, occurring between 25 and 40 years after recovery from the initial bout of infection. Many polio survivors, particularly those stricken in the mid-1950's have not yet begun to experience the painful and often disabling symptoms reported by some survivors: Although in the older patient some of these symptoms can be attributed to the natural aging process, many patients reporting symptoms are too young for such a cause. Many experts now believe that post-polio syndrome may be the result of chronic overuse of polio weakened muscles or joints.

Most polio patients underwent rehabilitation at the time of their polio. Because these patients had unimpaired intellects and normal muscle awareness and sensation, they could use their neuromuscular system, despite the polio damage, to its limit. And many polio survivors did just that, establishing families and careers (see"Marny Eulberg: Polio patient, MD, clinical activist," page 133). Ironically, this very desire to push oneself to live normally has taken its toll, and the resulting muscle weakness, muscle and joint pain, and severe fatigue have been described by many post-polio patients as similar to the symptoms of the initial polio.

Post-polio syndrome is a physical disorder with psychological implications. The possibility of having to return to crutches, braces, or wheelchairs is frightening, and conjures up memories of metal and leather, iron lungs, and Kenny hot packs. By understanding the emotional makeup of the polio survivor and the progressive nature of the syndrome , you can help significantly in relieving the concerns of the patient and the family.

Post-polio syndrome is a diagnosis of exclusion. The differential includes other degenerative disorders, such as arthritis, tendonitis, Guillain-Barre syndrome, and amyotrophic lateral sclerosis(ALS). Once other medical, orthopedic, and neuralgic conditions have been excluded, look for proof of an episode of paralytic polio, followed by stabilization and a long period of relatively constant, albeit impaired, function before the gradual onset of new weakness. The weakness may develop in any muscle, whether affected previously or not, and is associated with overuse, although disuse can cause weakness as well (see Table 1). But before you take the history at face value, bear in mind that some people diagnosed as polio patients may not have had polio at all. In the late 1940's and early 1950's, when few families had health insurance, many children who had other disorders, such as viral meningitis, encephalitis, or Guillain-Barre syndrome, were diagnosed as polio patients in order to be eligible for financial assistance to pay for the hospitalization and respiratory and rehabilitation equipment provided by the March of Dimes.

Ask whether the patient recalls a febrile episode associated with paralysis. If the history is uncertain, electromyography can confirm previous polio. Determining whether the patient was in an iron lung may prove useful in determining the possibility of respiratory insufficiency as the syndrome progress. Other questions to ask include:

Was the patient hospitalized for a long period? Knowledge of hospitalization does not confirm a history of polio, but it may be helpful. During the polio epidemics it was not uncommon for patients to be hospitalized of three months or longer. Of course, many polio patients at the time were not hospitalized, and patients with diseases other than polio may have been hospitalized for long periods as well. Recollection of other aspects of polio treatment supports the implication that extended hospitalization meant severe polio - and more likely post-polio syndrome later in life.

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Criteria for diagnosis of postpolio syndrome

Prior episode of paralytic polio confirmed by history, physical examination, and EMG

EMG demonstration of changes consistent with prior exposure to an anterior hom cell disease (of which polio is the primary one)

A period of neuralgic recovery followed by an extended interval of functional stability preceding the onset of new problems

Gradual or abrupt onset of weakness in previously affected or unaffected muscles

Exclusion of medical, orthopedic, and neuralgic conditions that might cause non-disuse weakness

Does the patient remember Kenny hot packs? Sister Kenny, an Australian nurse advocated a combination of heat and physical therapy. During the polio epidemics, hospitalized polio patients were wrapped in hot, wet wool packs, then wrapped in a plastic sheet to keep the heat and moisture in. This was done several times a day, and the smell of wet wool is one that a patient is likely to remember.

Has the patient had surgery to correct problems related to polio? Some fairly typical kinds of surgery, such as tendon transfers, bone blocks, contracture releases, epiphysiodesis or "stapling," have been done over the years to stop the growth of an unaffected area or let to allow both extremities to grow to the same length, or to provide mechanical stability or balance.

Are left and right shoes the same size? In a patient who had polio as a growing child, the affected leg may be one to three inches shorter than the unaffected one. Many polio survivors wear different size shoes.

Does the affected arm or leg get unusually cold in cold weather or in an air conditioned room? Often, the affected extremity of a polio survivor will become noticeably colder, and will remain cold for hours. This occurs not as a result of poor circulation - the patient will usually have a healthy vascular system - but seems to reflect a problem with the sympathetic nervous system response and lack of normal muscle pumping action.
The progression of post-polio syndrome can be likened to gradually changing vision: It comes on so slowly that the patient adapts to it, until an accident or near accident convinces him or her that something is wrong. Frequently, unaccustomed fatigue is the first symptom a post-polio patient notices. The patient generally wakes refreshed but may feel exhausted by the middle of the day. Patients have reported falling asleep while driving home from work.

Signs of neuromuscular involvement: Fatigue is often the first symptom, followed by weakness in affected extremities, and then muscle and joint pain. Electromyography and manual muscle testing can determine the extent of muscle involvement. Post-polio syndrome is often associated with painful joints as well as muscles, and is particularly common in the shoulders of patients using crutches or manual wheelchairs. the patient may also have osteoarthritis, tendonitis, or bursitis in the knee or hip bearing the majority of weight.

Other patients find themselves feeling tired and weak when they do certain activities that once were no problem. Many complain of severe fatigue, sometimes referred to as the "polio wall" which is similar to the "wall" that marathon runners say they hit near the end of a run - the point at which the body refuses to go any farther, no matter how the person may try. This fatigue may be followed 6-18 months later by the onset of increasing weakness, and then a feeling of muscle and joint pain

The muscles of the polio survivor may never get sufficient rest to recover the oxygen and nutrients expended in an activity. This is again similar to what happens in marathon runners who over-stress their muscles: Biopsies taken immediately after a run show that many muscle cells have been damaged by ischemia. for the polio survivor who uses his muscles near maximum capacity for several hours every day while walking or simply standing, the cumulative damage can be crippling

Functional signs of increased muscle weakness are probably the most reliable indicator of the post-polio syndrome, and the easiest to measure. for example, the patient may begin to notice himself tripping (often a sign of foot drop) or may have trouble climbing stairs or getting up from a chair. A patient with upper extremity involvement may drop things or may have trouble writing or drawing for any length of time.

In some doubtful cases, electromyography (EMG) is necessary to confirm a previous diagnosis of polio, although it does not allow you to predict whether a polio survivor is likely to develop post-polio syndrome. A patient with a completely normal EMG reading probably did not have polio: his muscle dysfunction is likely the result of another neuropathy or myopathy, and further diagnostic testing is indicated.

Manual muscle testing of a patient with possible post-polio syndrome is best done by a physical therapist who has worked with polio patients or who has strong skills in manual muscle testing. There are many post-polio clinics across the nation that perform baseline and evaluative testing; a listing of these clinics is available from Gazette International Networking Institute (see "Polio support groups: Still playing useful roles"). Primary-care-oriented clinics may offer a two-hour assessment of muscle function and mobility, with consultation by appropriate specialists as required. More comprehensive clinics offer a three day evaluation including EMG physical and occupational therapy, and psychological evaluation. Fees range from $150 to $1,500. Depending on the particular needs and insurance coverage of your patient, you may wish to refer him to a local post-polio clinic for evaluation.

Reasons for adjusting life-style in postpolio syndrome

New health problems -- Fatigue -- Weakness in any muscle -- Muscle pain -- Joint pain -- Breathing difficulties

Problems with activities of daily living
Walking distances
Climbing stairs
Bathing
Dressing
Transfers to and from wheelchairs
Difficulty with work-related activities requiring endurance and strength

The patient with ALS - for which post-polio was commonly mistaken when it was first discovered - tends to have more widespread and frequent fasciculations. In addition, ALS patients usually have hyperactive reflexes and may have extensor/plantar reflexes (positive Babinski reflexes), which are absent in post-polio patients. some have postulated that post-polio syndrome is characterized by a very slow, stepwise degeneration of approximately 1% per year; the patient usually experiences a certain amount of deterioration, which subsides for years before further change occurs. ALS, by comparison, is more rapidly progressive: The majority of ALS patients die within three years of diagnosis.

Post-polio syndrome is often associated with painful joints as well as muscles, especially the shoulders, knees, and hips. A post-polio patient may develop degenerative osteoarthritis in the knee or hip that has carried his weight for all these years, but he will also have significant muscle weakness and wasting in the same or opposite extremity, which you will not see in the patient with arthritis alone. A crutch, walker, or manual wheelchair user may present with tendonitis or bursitis in the shoulders, elbows or wrists.

Assessing pulmonary function: Morning headaches or sleep disturbances may indicate hypercapnia; arterial blood gasses should be measured. The vital capacity of a patient with respiratory involvement during initial polio should be measured every 1-2 years; a measurement of 1,000 mL or less may indicate the need for a full pulmonary evaluation, including pulmonary function tests, sleep studies, and arterial blood gas measurements.

The respiratory musculature should be tested, particularly if the patient required respiratory assistance during the initial attack of polio. Post-polio patients who required ventilator support at the time of their polio may suffer respiratory insufficiency or restrictive lung disease (see "Pulmonary considerations in post-polio syndrome"). Because respiratory therapy as a profession largely came into being during the 1960's, most respiratory therapists have not worked with many polio patients and may not be aware of some of the specific techniques for strengthening respiratory muscles such as the diaphragm, or of chest wall stretching and mobilization. The nearest post-polio clinic should be able to recommend a respiratory therapist who has such experience.

It is essential to measure the vital capacity of a patient who shows signs of respiratory involvement. Vital capacity should be measured at least every 1-2 years in anyone who was in an iron lung during his initial polio, and more frequently - perhaps yearly - if the vital capacity measures below 1,5000 mL (normal is 3,000 to 4,000 mL). A vital capacity of less than 1,000 mL warrants a full pulmonary evaluation, including pulmonary function tests, sleep studies, and arterial blood gas (ABG) measurements.

Screen for respiratory muscle weakness initially with the patient sitting. If the patient has obvious chest wall weakness and atrophy of the muscles, scoliosis, or abdominal muscle weakness, the vital capacity should be measured in both sitting and supine positions. A marked discrepancy in vital capacity between the supine and seated positions suggests that respiratory assistance may be required during sleep.

Measuring ABG levels may confirm the need for some type of ventilator support during the night . Fatigue or energy level often improves if a ventilator is used for the 6-10 hours during sleep, giving respiratory muscles much needed rest and allowing the patient undisturbed sleep.

A patient with incipient respiratory insufficiency may complain of headaches upon awakening, or of waking every hour or two during the night. These symptoms of sleep apnea may be associated with hypercapnia and the need for supplemental oxygen while sleeping. A study of 165 post-polio patients conducted in 1986 revealed that symptoms of sleep apnea were common: 45% of these patients reported daytime sleepiness, and 92% slept poorly, snoring and waking frequently.1 Although aging seems to play a role in sleep apnea in both normal persons and post-polio patients, it does not explain the occurrence of sleep apnea in younger survivors of polio.

It is important to establish the diagnosis of post-polio syndrome in a patient present with sleep apnea, because some of the methods used for managing sleep apnea can be contraindicated in the post-polio patient. For example, surgery to reconstruct the posterior pharynx is often recommended for patients with sleep apnea; however, such surgery will preclude gloss pharyngeal breathing (frog breathing), which is used by some polio survivors to supplement daytime breathing or to assist with coughing. Similarly, obstructive sleep apnea will be exacerbated by negative pressure ventilation - such as with an iron lung or chest cuirass. For post-polio patients with obstructive sleep apnea, a positive pressure ventilator may be a necessary measure .

Therapeutic options: Monitored exercise and weight control are essential in maintaining function. Nonsteroidal anti-inflammatory drugs may help the patient with debilitating muscle or joint pain. Antidepressants - at one third to one-half the dosage used for treating depression - may help nonspecific pain and sleep disturbances. Narcotics should be avoided, and may be deadly in a patient with respiratory involvement. Orthopedic reconstruction may not be successful in many polio survivors.

Although life-style modification is the primary treatment for symptoms of post-polio syndrome, the post-polio patient should also have some kind of exercise program - yoga or mild stretching if aerobic exercise is not possible - to keep the body limber, maintain range of motion, and provide a sense of well being . Because post-polio syndrome is believed to be a disorder resulting from over use of muscles, the key to optimal function is controlled or monitored exercise.

Depending on the muscle power remaining , the patient should be encourage into low-intensity, low-stress, activities that provide cardiovascular conditioning to strengthen the heart and lungs. Aerobics must not strain the symptomatic muscles; these muscles are already working to capacity. If the source of aerobics strains the symptomatic muscles, the patient does not need the aerobic exercise: The affected muscles will never create enough demand on the heart and lungs to warrant the aerobic exercise. A patient with sound arms can probably do some form of aerobics even if the leg muscles are affected.

The patient should come to know his body and its limitations, and adapt his exercise program accordingly. Rehabilitation experts rarely prescribe a specific exercise program for the post-polio patient. Instead, therapists may work undamaged muscles and advise the patient to do the exercises that are fun. Exercise should stop just short of the point of pain, fatigue, or fasciculation's. Pushing beyond that point will further damage the overworked muscle, and capacity lost to the initial disease cannot be regained.

When muscles are tired and weak but not painful, it is difficult to determine how much exercise is too much or too little. Some rehabilitation experts suggest an initial exercise of five repetitions at 50-60% resistance, increasing the repetitions (if the patient can tolerate that) to ten repetitions, and then increasing the resistance. The rule is that if the patient feels better, he can keep going. If the creased exercise makes him feel worse, the exercise should be reduced by half. If the patient still feels worse, he should stop the exercise; he is already using the affected muscles to their full capacity.

Swimming is ideal because most effects of gravity are countered and the buoyancy facilitates use of weak or painful joints. For someone who can hardly move on dry land and finds himself able to swim easily, there is an enormous psychological benefit. And often if the water is heated, the total therapeutic benefit can be greater than that expected from heat or exercise alone. Encourage patients to search for a pool that is heated or equipped with a lift or ramp, if necessary. In all activities, moderation is the key to prolonged value and success.

Exercise is also important for weight control. Obesity is a problem for many post-polio patients, particularly those who use wheelchairs. Imagine asking a marathon runner in addition to running a marathon to carry 5 lbs of butter in one hand and a 10 lb bag of sugar in the other. Now imagine the effect of an extra 15 lb on the overworked muscles of the post-polio patient: Every step, every pace, every movement is weighted down, creating more fatigue and more pain. Obesity can also make the fitting of adaptive equipment, such as braces, corsets, or chest cuirasses, difficult.

Inflammation that results from the initial injury and subsequent overuse may be managed by nonsteroidal anti-inflammatory drugs if the muscle and joint pain is intense. Prescribe ibuprofen (Advil, Midipren, Motrin, etc.) or other antiarthritic agents at the dosages you would give for arthritis and tendonitis. Be careful, however, that the patient does not come to depend on these agents, or use them as a means to continue to "overdo."

Antidepressants, prescribed at one third to one-half the dosage used for treating depression, frequently will help a patient who has non specific pain. This is analogous to giving amitriptyline HC1 (Amitril, Elavil, Endep, etc.) to manage chronic headache or backache and may be especially beneficial when muscle pain interferes with sleep.

The use of narcotics should be avoided because the patient may develop tolerance and addiction. These drugs are particularly dangerous in a patient who suffers respiratory problems during sleep. The patient who needs constant pain medication probably needs to adapt his life-style: If he cannot function without the drug, it usually means he is too active or needs an ambulatory aid or wheelchair.

Many of the complications related to post-polio syndrome are orthopedic. Unfortunately, the post-polio patient is not a good candidate for certain surgical procedures that would otherwise be considered. For example, total reconstruction of an osteoarthritic hip or knee may not be successful in the post-polio patient because the contra lateral joint, in the polio-affected limb, will not be strong enough to carry his weight during the convalescent period. Likewise, a polio survivor with markedly weakened muscles and ligaments around the knee will not be a good candidate for non-constrained knee reconstruction; however a hinged prosthesis may be an alternative.

Life-style adaptations: Reducing the level of activity and taking frequent rests may be the best approach to controlling pain and fatigue in the post-polio syndrome patient. Enlisting the help of the family and referring the patient to a local post-polio support group may make the change to a slower, less debilitating life-style more acceptable. The best person to work with a polio survivor is a polio survivor; the best teacher of life-style modification is one who has already learned.

The post-polio patient must modify his or her life-style to relieve the daily strain on overused muscles. Primary treatment involves breaking daily activities into smaller segments so that there are frequent rest periods. Alternatively, the patient can relieve the strain on muscles by using adaptive equipment: braces, crutches, canes, wheelchairs, or ventilators.

The patient who modifies his life-style can stabilize his condition, but continued over-use will only lead to further deterioration. People who are able to slow down, monitor their activities more closely, pace themselves as they go through the day and use adaptive devices as necessary are resting overused muscles. some of these patients may begin to regain strength and halt symptom progression if damage is not too severe.

Even though many post-polio patients know better, deep down they may consider going back to a brace, a failure - that somehow they are not working hard enough. Part of the sign of success for a polio survivor has been shedding his equipment; the need now to return to these devices is seen as defeat. Keep in mind that it may take a post-polio patient a year or more to work ;up to using a brace or a crutch, and even longer to use a wheelchair. Counsel the family as to the devastating emotional impact the need for these devices can have on the patient, and ask them to gently encourage the patient in his use of them as a positive life choice.

Although most polio survivors are reluctant to admit to a handicap, they may find that subtle devices that conserve energy are most helpful. If the patient can decide where and when to use the device, he may be more inclined to try it. Many patients are unaware of the advances that have been made in adaptive equipment since the time of their initial polio: The 15 lb braces they threw off as kids now weigh 2.5 or 3 lb

In selecting and fitting adaptive equipment, it is best to consult an expert who has worked with polio patients. It is probably best for the post-polio patient who needs a variety of devices, to have a post-polio team - physical therapist, occupational therapist, orthotist, and respiratory therapist, if appropriate - familiar with the specific equipment necessary, such as long leg braces, rocking beds, and other devices. This is particularly important for ventilator users, who will need a unit that will be easy to use at home - one that can be easily transported and has a minimal number of dials and alarms.

Like any chronic disease, post-polio affects the entire family. Many post-polio patients married and started families long after their initial polio. This makes their condition particularly distressing, since everyone, including the patient, has become accustomed to a particular level of functional ability. when the patient often at the presumed peak of adulthood, begins to lose strength and becomes increasingly fatigued, his symptoms often are not readily perceived by office colleagues, friends, or even family members.

A loving and supportive family can be the most important resource the post-polio patient has. Encourage family members to show they love and care about the patient , who may still harbor unresolved feelings of abandonment stemming from the initial polio episode of childhood, endured at a time when families were not permitted to stay in the hospital and help with rehabilitation. Because many people have long been led to believe that love and acceptance are determined by productivity, there is an underlying assumption that being productive at any cost is something to strive for. Assuring the patient that this is not the case will enable him to modify his life-style and conserve energy without fear of losing those he depends on. It is important, however, that the family recognize that it is itself a limited resource. If caring for a bedridden or ventilator dependent post-polio patient becomes exhausting, the family should be encouraged to seek the services of a part-time attendant or aide.

Changes in life-style should not make things harder than they need be, and common sense should prevail. Understanding, acceptance, and patience, combined with the offer of resources and support systems, will provide the best foundation for care for patients with post-polio syndrome.

Prepared by Janice L. Jencarelli

Endnote references available on request.

A New Effort To Prevent Vaccine-Associated Polio
In an effort to reduce the estimated eight cases of paralytic polio that result each year from immunization of infants and children with the live virus oral polio vaccine (OPV), the Advisory Committee on Immunization Practices of the U.S. Public Health Service commissioned the Institute of Medicine to reevaluate the current polio immunization policy in the United States.
Although the Committee continues to recommend OPV for use in infants and children, it advocates that the enhanced-potency inactivated polio vaccine (IPV), manufactured by Connaught as Poliovirus Vaccine Inactivated, be the vaccine of choice for immuno-compromised patients, their household contacts, and all previously non-immunized adults at increased risk for associated paralytic polio.
The supplementary statement from the Immunization Practices Advisory Committee (ACIP) says that persons at increased risk for developing paralytic polio from trivalent OPV include those with congenital immune deficiency diseases (such as agammaglobulinemia), acquired immune deficiency diseases (such as AIDS), or altered immune status resulting from other diseases or immuno-suppressive therapy (such as therapy for leukemia). The ACIP recommends that these persons and their close contacts receive enhanced-potency IPV rather than OPV.
The risk of exposure to wild polio virus would be increased if IPV alone, or even a combination of OPV and IPV, were to replace OPV as the primary vaccine. Although both the World Health Organization and the Pan-American Health Organization have vowed to eradicate the transmission of wild virus polio by 1990 through campaigns to increase awareness of the need for immunization, it remains to be seen whether education will be nearly as successful at eliminating the wild-virus polio as OPV has been.

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Fischer DA: Sleep-disordered breathing as a late effect of poliomyelitis, in Halstead LS. Wiechers DO (eds): Research and Clinical Aspects of the Late Effects of Poliomyelitis. White Plains, New York, March of Dimes Birth Defects Foundation, 1987, pp 115-120

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Reproduced with permission from PATIENT CARE, June 15, 1988.
Copyright © 1982, Patient Care, Oradell, NJ. All Rights reserved.

Marny K. Eulberg, MD; Lauro S. Halstead, MD; Jacquelin Perry, MD
Postpolio Syndrome:
How you can help
Are you missing the diagnosis of postpolio syndrome? Many of those who struggled to survive are now re-experiencing weakness, pain, and fatigue long after the acute phase.