Post Polio syndrome

Summary

* Polio is caused by a virus that leads to motor neuron damage and death.

* Pain, fatigue and muscle weakness can occur in polio patients as much as 40 years after their initial infection.

* Post-polio is considered a chronic syndrome associated with progressive muscle weakness and fatigue after motor neuron death.

* The original treatment protocol was to exercise aggressively. It is now known that exercise should be performed at no greater than sub-maximal levels.

* Slow exercise progression is a top priority.

* The degree of difficulty scale is a safe approach when assessing an individual's initial fitness level.

* Beginning work levels should be a set of three to five repetitions with a weight that does not rate higher than a three on the degree of difficulty scale.

* To avoid overuse, instruct patients to monitor fatigue.

* The initial interview with the post-polio patient is vital to discover successful and detrimental daily living activities.

* All work should be recorded to help quantify adaptation.

* Weight gain is to be avoided, if possible, because it makes performing activities of daily living more difficult.

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This month's CEU Corner and quiz feature is "Post-Polio Syndrome: Can it be Managed?"

After many years of neurologic and functional stability, people who suffered from polio earlier in their lives may develop late-onset, neuromuscular symptoms. This is referred to as post-poliomyelitis (i.e., post-polio) syndrome and used interchangeably with "late sequelae" and "late effects" of poliomyelitis (Halstead, 1991). Although first reported over 100 years ago, the development of weakness, fatigue, muscle and joint pain experienced by survivors many years after poliomyelitis' onset has only recently been recognized by health care providers (Agre, et al., 1991). Even as much as 30 to 40 years after the acute illness, patients reported additional problems, including breathing difficulty, decreased endurance, problems swallowing, choking, increased sensitivity to cold and psychological problems (Dean, 1991). Fischer (1985) states fewer than 12 people per year have been reported to have developed poliomyelitis since the mid-1970s, yet the disease's initial onset in the early 1950s has left millions to suffer with more than just deformity and debilitation. Many of these individuals are now faced with the negative impact of activity changes, directly related to their mobility and physical demands in the home and workplace (Farbu, et al., 2003).

History

Acute paralytic poliomyelitis was a major health problem during the early 1950s. According to Sunnerhagen and Grimby (2001), many people contracted the disease during the late summer and early fall, typically presenting an acute biphasical viral illness with fever, headache and gastro-intestinal symptoms followed by the rapid development of paralysis. The paralysis is caused by the neurotrophic polio virus attack on motor neurons, which can result in neuronal damage or death. Agre, et al., (1991) add that the disease has a specific affinity for cells of the anterior horn (i.e., the horn-shaped configuration presented by the anterior column of the spinal cord in transverse section). As these cells die, Wallerian degeneration occurs and the muscle fibers associated with those neurons become "orphaned," resulting in weakness. A correlation was also noted between the proportion of destroyed motor neurons and severity of paralysis (Bodian, 1949).

Traditional Management

After polio's acute phase, regaining lost strength was the goal of physical therapy. Therefore, physical activity was encouraged and mandatory programs, including intensive strengthening exercises, were prescribed (Bruno & Frick, 1991). At this time, the prevailing attitude among polio survivors was that exercise minimized disability and maximized independence (Scheer, 1991). Today, treatment philosophy for managing polio's late effects is quite contrary to the initial approach. These exercise programs were apparently too ambitious and resulted in overuse syndrome.

Dean (1991) alludes to a current growing concern that overloading muscles with new or increased muscle weakness might lead to further decrease in function. In addition, Chan, et al., (2003) questioned whether a strength-training program has a beneficial impact on muscle function or harms surviving motor neurons. Many questions are yet to be answered as to the cause of the potential rebirth of this disease. Nevertheless, there is no support for the notion that exercise can reactivate the polio virus (Jubelt & Cashman, 1987).

Proposed Etiologies of Post-Polio Syndrome

A number of pathophysiologic etiologies have been proposed and reviewed by Jubelt and Cashman (1987) for the presumed late deterioration in neuromuscular function, including:

* chronic polio virus infection

* death of remaining motor neurons due to normal aging, coupled with the previous loss from poliomyelitis

* premature aging of remaining normal motor neurons due to an increased metabolic demand

* loss of individual muscle fibers per reinnerv motor unit due to advancing age in the large reinnervated motor units that developed after polio

* predisposition to motor neuron degeneration due to the glial, vascular and lymphatic changes caused by polio virus

* poliomyelitis-induced vulnerability of motor neurons to secondary insults

* genetic predisposition of motor neurons to both poliomyelitis and premature degeneration

* an immune-mediated syndrome.

However, Agre (1991) states that other than muscle fiber loss from reinnervated motor units with age (fourth bullet item), most of these proposed etiologies are unlikely causes of progressive neuromuscular function loss. He contends the two primary causes of neuromuscular function loss are disuse and overuse, hence considered functional etiologies. Weight gain and chronic weakness are additional functional etiologies.

Clearly, disuse/inactivity leads to decreased muscle strength and cardiorespiratory fitness (i.e., deconditioning). Even among the normal population, muscle strength diminishes 1 percent every year after the third decade (Borges, 1989). Polio patients are known to be deconditioned and have reduced aerobic power (Stanghelle, et al., 1993). Post-polio patients' aerobic capacity is comparable to patients who recently experienced a myocardial infarction with an average maximal metabolic capacity of 5.6 METS (Agre, 1991). Grimby, et al., (1989) reported a low concentration of the aerobic enzyme, citrate synthase, in muscles of post-polio subjects and this was believed to be compatible with a low activity level in these individuals. Besides a muscle mass reduction after polio, increased muscle fatigue can also result from inactivity with a decreased oxidative enzyme level (Willen, et al., 1999).

Overwork/overuse can be defined as a condition in which physiological demand exceeds capacity. These mechanisms include both chronic metabolic fatigue of the muscle and anatomic disruption of muscle fibers from overwork (Agre, 1991). Similar to inactivity, muscle tissue overwork is detrimental even to the normal population because some level of diminished function can occur, manifesting itself in fatigue and/or muscle soreness. However, for healthy individuals, recovery from these symptoms is relatively predictable and successful. Fatigue as well is a common complaint by polio subjects, described as "increas[ed] physical weakness," "a sensation of loss of strength during exercise" and "heavy sensations in the muscles" (Berlly, et al., 1991). For the polio patient, fatigue and muscular weakness seem to mimic chronic fatigue and fibromyalgia in terms of lengthy recovery time. At this point, it would appear prolonged fatigue and lack of recovery might suggest exercise, as is commonly feared, furthers neuromuscular deterioration and dysfunction.

Successful Exercise

There has been much debate regarding strength training's role in the post-polio patient's fitness program. Studies have suggested the extra load imposed by exercise could induce degeneration of chronically enlarged anterior horn cells (Chang & Huang, 2001; Tam, et al., 2002). However, Chan, et al., (2002) point out many potential problems with these studies, such as lack of controls, indirect methods of assessing motor unit survival, invasive macro-EMG technique and lack of central nervous system evaluation. Their study concluded that a moderate intensity strength-training program did not adversely affect motor neuron survival and markedly improved voluntary muscle activation. Spector, et al., (1996) also reported significant dynamic strength gains of both symptomatic and asymptomatic muscles in post-polio patients without serological or historical evidence of muscular damage. According to Gisli (1991), a combination of neural and muscular adaptation may explain the strength increase. Recruitment of muscle fibers in healthy, unaffected, yet deconditioned tissue is likely to occur as it does in the healthy population.

Exercise Prescription

Fitness professionals are continually challenged to determine the most appropriate exercise programming, especially when dealing with special populations. Program facilitation is even more critical because it attracts tremendous scrutiny, not only to achieve the desired result, but to do so with an extra measure of safety. A program's progression plays the most important role in navigating these individuals through the quagmire of afflictions brought about by illness, injury and normal aging.

Although exercise has been shown to improve muscle strength and fitness in the post-polio patient, it must be individually prescribed and carefully evaluated (Willen & Scherman, 2002). Individuals must be assessed properly and taught to pay attention to their exertion and fatigue, both during exercise and daily activities, in order to avoid overuse problems (Agre, et al., 1991). The trainability varies according to the type and degree of polio changes (Grimby, 2002). Furthermore, all exercise should be monitored by recording the work performed.

Due to the variability in which different muscle groups' motor neurons may have been affected in a particular patient (Agre, 1995), the assessment's interview portion can help identify daily living activities the individual can and cannot perform. This can be accomplished by simply conversing about a typical day, week and month. The follow up physical assessment will help determine where the locomotor economy difficulties exist in order to minimize risk of injury and overuse.

Working with this population is challenging because the established program can become detrimental if too aggressive. Non-fatiguing work is pertinent to avoid overuse stress and can be incorporated by continually monitoring the individual's perception of difficulty. Using a one to 10 scale, similar to the Borg scale, with the degree of difficulty not exceeding a three is a good beginning. However, allow time for adaptation before attempting to increase the load. Furthermore, keep the sets to one and repetitions between three to five in the beginning. Lastly, the program design should be for the entire body, not only affected or unaffected regions.

References

Agre, J., "The role of exercise in the patient with post-polio syndrome," Ann. N.Y. Acad. Sci., 753 (May 25, 1995): 321-34.

Agre, J.C., Rodriguez, A.A. & Tafel, J.A., "Late Effects of Polio: Critical Review of the Literature on Neuromuscular Function," Arch. Phys. Med. Rehabil., 72 (October 1991): 923-30.

Berlly, M., Strauser, W. & Hall, K., "Fatigue in postpolio syndrome," Arch. Phys. Med. Rehabil., 72 (February 1991): 115-8.

Bodian, D. Pathologic Anatomy. Philadelphia: Lippincott, 1949.

Borges, O., "Isometric and isokinetic knee extension and flexion torque in men and women aged 20-70," Scand. J. Rehabil. Med., 21 (1989): 45-53.

Bruno, R.L. & Frick, N.M., "Post-polio sequelae: behavior modification and psychotherapy," Orthop., 14 (1991): 1185-93.

Chan, K.M., et al., "Randomized controlled trial of strength training in post-polio patients," Muscle Nerve, 27, no. 3 (March 2003): 332-8.

Chang, C. & Huang, S., "Varied clinical patterns, physical activities, muscle enzymes, electromyographic and histologic findings in patients with post-polio syndrome in Taiwan," Spinal Cord, 39 (2001): 526-53.

Dean, E., "Clinical Decision Making in the Management of the Late Sequelae of Poliomyelitis," Physical Therapy, 71, no. 10 (October 1991): 752-9.

Farbu, E., Rekand, T. & Gilhus, N., "Post-polio syndrome and total health status in a prospective hospital study," European Journal of Neurology, 10 (2003): 407-13.

Fischer, D., "Poliomyelitis: late respiratory complications and management," Orthopedics, 8 (1985): 891-4.

Gisli, E., "Muscle conditioning in late poliomyelitis," Arch. Phys. Med. Rehabil., 72 (January 1991): 11-4.

Grimby, G., "Post-polio syndrome-symptomatology and measures," Ugeskr Laeger, 164, no. 21 (May 20, 2002): 2752-6.

Grimby, G., et al., "Muscle adaptive changes in post-polio subjects," Scand. J. Rehabil. Med., 21 (1989): 19-26.

Halstead, L.S. Post-polio Syndrome. Ed. T.L. Munsat. Boston: Butterworth-Heinemann, 1991.

Jubelt, B. & Cashman, N.R., "Neurological manifestations of the post-polio syndrome," Crit. Rev. Neurobiol., 3 (1987): 199-220.

Scheer, J. & Luborsky, M.L., "The cultural context of polio biographies," Orthop., 14 (1991): 1173-81.

Spector, S., et al., "Strength gains without muscle injury after strength training in patients with post-polio muscular atrophy," Muscle Nerve, 19, no. 10 (October 1996): 1282-90.

Stanghelle, J.K., Festvag, L. & Aksnes, A.K., "Pulmonary function and symptom-limited exercise stress testing in subjects with late sequelae of poliomyelitis," Scand, J. Rehabil. Med., 25 (1993): 125-9.

Sunnerhagen, K.S. & Grimby, G., "Muscular effects in late polio," Acta Physiologica Scandinavica, 171, no. 3 (March 2001): 335.

Tam, S., et al., "Effect of exercise on stability of chronically enlarged motor unit," Muscle Nerve, 25, no. 3 (March 2002): 359-69.

Willen, C., Cider, A. & Sunnerhagen, K.S., "Physical performance in individuals with late effects of polio," Scand. J. Rehabil. Med., 31, no. 4 (1999): 244-9.

Willen, C. & Scherman, M., "Group training in a pool causes ripples on the water. Experiences by persons with late effects of polio," J. Rehabil. Med., 34 (2002): 191-7.

Poliomyelitis Today

As recently as 35 years ago, poliomyelitis was a major cause of paralysis and death in children and young adults in developed countries. In 1985, Rotary International, a non-profit, non-political service organization from the private sector, committed to help immunize the world's children against polio. The target date for a polio-free world was set for 2005. In 1988, the public sector joined Rotary, launching the Global Polio Eradication Initiative in what is described as the largest public health initiative in history. Through the efforts of this global partnership, established between Rotary International and The World Health Organization (WHO), UNICEF and the United States Centers for Disease Control (CDC), the incidence of polio has been slashed by 99 percent. However, the polio virus still remains endemic in 10 countries: Afghanistan, Angola, Egypt, Ethiopia, India, Niger, Nigeria, Pakistan, Somalia and Sudan.

There is great anticipation to eliminate polio throughout the world. In addition to raising the consciousness of government heads, a large sum is needed to finance the project. Fundraising for this monumental effort has been tremendous and everyone is working harder than ever. With the financial goal of" $1 billion, so far $725 million has been pledged. In 2002, Rotarian's surpassed their annual goal of $80 million, totaling $88 million for the year. These figures put Rotary's contribution to the initiative in the arena of $600 million. Carol Bellamy, UNICEF's executive director, congratulated Rotary stating "Rotarians really are the heart and soul of the Global Polio Eradication Initiative." Dr. Stephen Cochi, director of the Global Immunization Division for the CDC, applauded Rotary for its "ceaseless dedication to the eradication effort."

It is inspiring to see a private sector organization take on such a challenge. It is also impressive to witness the change these Rotarian's have brought about through 100 percent volunteer effort. Rotary's motto is "service about self" and Rotarians obviously take it very seriously.

QUESTIONS: Code No. 0061

"Post-Polio Syndrome: Can it be Managed?"

1. The term postpoliomyelitis (i.e., post-polio) syndrome is used interchangeably with-of poliomyelitis.

A. "late sequelae"

B. "post effects"

C. "late effects"

D. A and C.

2. Physical characteristics of postpolio syndrome include--.

A. weakness

B. fatigue

C. muscle and joint pain

D. All of the above.

3. Neurotrophic polio virus attack on motor neurons causes--in polio victims.

A. infection

B. paralysis

C. rash

D. None of the above.

4. Motor neuron--results in weakness.

A. degeneration

B. elevation

C. rash

D. None of the above.

5. The goal of traditional physical therapy was to regain lost strength, therefore programs, including--, were prescribed for polio victims.

A. swimming

B. running

C. intensive strengthening exercises

D. All of the above.

6. Polio victims originally believed exercise minimized--and maximized--. A. independence; disability

B. disability; independence

C. lethargy; ambition

D. pain; mobility

7. The onset of weakness, pain and fatigue 30 to 40 years after the initial polio affliction led many to believe--muscles might lead to further decrease in function.

A. overloading

B. massaging

C. stretching

D. MI of the above.

8. There is no support for the notion that exercise can--the polio virus.

A. reactivate

B. eradicate

C. diminish

D. suspend

9. Agre (1991) contends that--and--are the two primary causes of neuromuscular function loss.

A. stress; lack of sleep

B. lack of sleep; exercise

C. disuse; overuse

D. over-stimulation; hypermobility

10. Polio patients are known to be--and have--aerobic power.

A. healthy; normal

B. deconditioned; reduced

C. deconditioned; normal

D. deconditioned; increased

11. Regarding exercise, polio victims should be taught to pay attention to their--in order to avoid overuse problems.

A. program load

B. repetitions

C. exertion

D. None of the above.

12. Some studies suggest the extra load imposed by exercise could be detrimental to the polio patient. The problems with these studies are due to--.

A. lack of controls

B. indirect methods of assessing motor unit survival

C. lack of central nervous system evaluation

D. All of the above.

13. Fatigue is the most common complaint from polio patients and described as--.

A. "increase[ed] physical weakness"

B. "sensation of loss of strength during exercise"

C. "heavy sensations in the muscles"

D. All of the above.

14. When incorporating the concept of perceived exertion and the one to 10 modified Borg scale, a degree of difficulty of--is a good beginning.

A. one

B. three

C. five

D seven

15. Training the post-polio patient is similar to training the normal population in terms of--.

A. adaptation

B. better weather

C. more equipment

D. All of the above.

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Gregory L. Welch, M.S., is an exercise physiologist and president of SpeciFit, An Agency of Wellness and Competitive Performance Enhancement, located in Seal Beach, California. He is also the wellness director for the Wellness Institute at the Downey Family YMCA. Welch is an accomplished writer and lecturer dedicated to raising the level of academia within the fitness industry. He can be reached at (562) 862-4201.

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