Julie K. Silver, MD is the Medical Director of the Spaulding-Framingham Outpatient Center. She teaches at Harvard Medical School in the Department of Physical Medicine and Rehabilitation. Dr. Silver's new book "Post Polio Syndrome--A Practical Guide for All Polio Survivors and their Families" is scheduled for release in early-2001 by Yale University Press

"Polio is spectacular, the way it strikes, the way it kills, the way it leaves its trademark." This is the opening line of Robert Hall's memoir, Through the Storm—A Polio Story. Polio is, or rather was, a spectacular and wildly uncontrolled disease that occurred in epidemics worldwide in the first half of the 20th century. The World Health Organization expects to eradicate polio worldwide by the end of 2000, and the last reported case of polio in the United States (from the wild virus and not vaccine related) was in 1979. However, there was a time not so long ago that polio was one of the most feared diseases.

Ironically it was through a series of technological advances, including improving sanitation and cleaning up water supplies, that the poliovirus was allowed to flourish. Counterintuitively, the poliovirus primarily attacked middle and upper class families who had improved living conditions but no immunity (in the past they would have acquired immunity through tainted water supplies). Thus, the number of polio cases reported reached epidemic proportions year after year until the mid-1950's when a vaccine was discovered. Polio will long be remembered as one of the most important and devastating health crises of the 20th century.

Ali Buckley is a 46-year-old nurse who contracted polio when she was 2 years old. Following an initial 2-week hospitalization in which Ali had severe right leg paralysis, she used a wheelchair for more than a year. There were few supports in place for children with disabilities, so Ali reports, "I lost an entire grade and was tutored at home." Ali also recalls that there was such hysteria at the time that she contracted polio that her father's dental practice had to be closed for several months because no one would go to him for fear that he might spread the poliovirus to them. Ali contracted polio in the summer of 1955 and for several summers thereafter, she would endure orthopedic surgeries on her legs to help her regain her ability to walk. For more than 30 years following these surgeries, Ali walked without any mobility devices or braces, but Ali recalls, "I hated summer because it always meant surgeries."

The polio epidemics were an important social and political issue as well as a public health issue. In part because of Franklin Delano Roosevelt, the 32nd president of the United States who was in office from 1933-1945 and who himself was a polio survivor. FDR was instrumental in promoting the National Foundation for Infantile Paralysis (NFIP) that supported nearly all of the research and treatment of polio. The NFIP later became known as the March of Dimes when all across the country people were asked to leave their porch lights on so that mothers could go door to door and collect money to fight this dreaded disease.

Then in the mid 1950s, both the Salk and the Sabin vaccines were found to be effective and the incidence of polio dramatically declined. The March of Dimes went on to fight birth defects and polio survivors went on with their lives…

• Post-Polio Syndrome
  Polio was nearly erased from the public's memory by the late 1970s when polio survivors first began to complain in large numbers that something was happening to them that was causing further disability. The most alarming symptom was new weakness. Polio survivors, who as a group were remarkably successful both personally and professionally, began en masse voicing complaints about new symptoms that resembled what had occurred during their initial illness (but were generally not as severe).

  In 1979, an international journal called the Rehabilitation Gazette published a letter from a polio survivor in which he described symptoms of new weakness, pain and fatigue. The magazine was deluged with responses from polio survivors who reported similar symptoms. This coming together of polio survivors who were experiencing symptoms of Post-Polio Syndrome (PPS) began to widely occur. Post-Polio support groups formed in record numbers with more than 100 groups established in the United States alone. These groups became the first real way that information about PPS was described and disseminated.
  [ Rehabilitation Gazette Online ]

  Doctors and other healthcare providers had learned about polio as a historical footnote. There was a lot of catching up to do now that the majority of polio survivors (more than 60% of the more than 1 million polio survivors in the United States and 20 million worldwide) would likely develop symptoms of PPS. { 1 }

• Symptoms
  The primary feature of PPS is new weakness. This new weakness may affect muscles that were known to have been involved during the initial polio or muscles that were previously thought to be uninvolved. It is important to keep in mind that during the initial polio infection, regardless of the extent of paralysis, more than 95% of the anterior horn cells in the spinal cord were affected. Thus, often muscles that were thought to be "normal" actually were affected and may now be susceptible to new weakness. Even people who were originally thought to have had the "non-paralytic" type of polio may develop new weakness and other symptoms of PPS.{ 2 } Sometimes this new weakness is associated with new muscular atrophy and one or more of the limbs will appear smaller due to loss of muscular bulk.

  David Rubin is a prominent businessman and real estate investor in Boston. He was diagnosed with polio when he was 9 years old and was treated at home. David recalls, "I remember after the onset of the paralysis having my spine tapped on the kitchen table by doctors." When asked about how PPS has affected him, he replies, "The weakening of my legs has been really daunting. I'm used to being very active and independent. It is a continuing difficult adjustment for me to accept the limitations that PPS has imposed."

  Another prominent symptom (occurring in more than 90%) is fatigue. This fatigue may be muscular fatigue related to overuse or deconditioning of the muscles, or it may be "central" fatigue meaning that areas of the brain that control alertness, memory and concentration are affected.{ 3 }

  Fatigue was the primary reason why 10 years ago, Ali gave up her job as an emergency room nurse. "I couldn't think at 10 o'clock at night." She switched to a part-time nursing position in occupational medicine where she primarily did case management. However, her symptoms of fatigue plagued her and she began to have difficulty with new weakness and pain as well. After 8 years of trying to struggle along, and the realization that "after multiple job accommodations, I couldn't perform my job", Ali made the heart wrenching decision to apply for disability.

  Although many polio survivors complain of pain, the pain associated with PPS is typically muscle pain (myalgias) that is often related to activity. There are many other reasons that pain may be present including musculoskeletal conditions (e.g., rotator cuff injury, lateral epicondylitis arthritis, etc.) and nerve injuries (e.g., carpal tunnel syndrome, ulnar neuropathy, lumbar radiculopathy, etc.). These problems may be attributable or at least exacerbated by a history of paralysis and muscular imbalances and overuse, but do not fit the criteria for Post-Polio muscle pain.

  New respiratory or swallowing problems may occur due to the muscles that control these functions becoming weaker. Cold intolerance, particularly complaints of the limbs being cold is a common symptom.

• Diagnostic criteria
  Post-Polio Syndrome, like all diseases that are classified as syndromes, has no specific diagnostic test. Therefore, it is a diagnosis of exclusion. This means that all other medical conditions that may present with symptoms similar to those found in PPS should be considered and excluded if possible. Furthermore, in order to establish the diagnosis of PPS, the individual must fit the following criteria:
  1. A history of polio (either paralytic or non-paralytic)

  2. A period of recovery from the initial illness

  3. A long stable period (generally more than 10-20 years)

  4. New symptoms that are consistent with PPS and not attributable to any other medical condition

  Treatment
  Treatment for PPS must be individualized and takes into account the patient's primary symptoms. Treatment may include medications, supervised therapy, injections and in some cases even surgery. It is impossible to present an exhaustive treatment regimen because all treatment is guided by the specific symptoms that predominate. Rehabilitation protocols need to take into account that individuals with PPS often present with new weakness. This new weakness inevitably leads to an increase in energy expenditure to perform their usual activities. This promotes both fatigue and pain. Thus, PT/OT should focus on simultaneously preventing overuse and disuse of the muscles. Although this might seem incongruous, in fact, this dichotomy of overuse/disuse is frequently seen.

  Consider the polio survivor who uses a manual wheelchair for mobility. This requires an enormous output of energy in order to propel the wheelchair. The arms are exclusively doing the work. But, the workload is not equally shared. Some muscles will be overused and some will be underused. Moreover, the amount of work or "exercise" that the arms must do on any given day may vary greatly depending on the individual's mobility requirements.

  Dorothy Aiello, a physical therapist who specializes in treating polio survivors at Spaulding Rehabilitation Hospital often recommends switching from a manual wheelchair to a motorized one. In conjunction with this switch, Aiello recommends a formal exercise program that can be done at home which works all of the muscle groups in a more even fashion.

  Without proper instruction and sound advice from skilled rehabilitation professionals, some polio survivors become so fearful of overusing their muscles that they literally barely move about. This promotes disuse of the muscles as well as loss of range of motion of the joints and many other medical problems. The important concept here is to balance activity and rest.

  Supervised therapy should address the issues of overuse and disuse of the muscles in order to prevent further loss of strength. Maria Cole, an OT at Spaulding who works on the same team as Aiello, says, "One of the biggest problems is [teaching polio survivors] how to balance energy conservation and work simplification in their daily tasks." Maria reports that a careful analysis of an individual's home and work environment will often lead to identifiable problems that can often be easily fixed. For instance, "ergonomics in the work environment" pose many problems, says Cole. "How they are seated, where their computer is situated and how their desk is set up can be arranged to improve posture and efficiency."

  Both Aiello and Cole agree that improving the efficiency and safety of a polio survivor's mobility is of utmost importance. This may mean recommending lightweight and durable braces, a motorized wheelchair, a handicapped parking placard, a tub or shower seat, etc.

  A skilled speech and language pathologist can often help someone manage their swallowing problems by simply teaching them a variety of swallowing techniques. Respiratory problems may improve with BiPAP (bilevel positive airway pressure). Avoiding cool climates and wearing warm layered clothing generally treats cold intolerance.

  It is important to recognize that the same treatment plan won't work for everyone. Therefore, it is the job of the rehabilitation professional to meet the challenges of each polio survivor who comes to him or her seeking treatment.

• References:
  
  1. Gawne AC, Halstead LS. Post-polio syndrome: pathophysiology and clinical management. Critical Rev in Phys and Rehabil Med 1995;7(2):147-188. {abstract not available}

  2. Halstead LS, Silver JK. Nonparalytic polio and postpolio syndrome. {Abstract} Arch Phys Med Rehabil 2000 Jan-Feb;79(1):13-8.

  3. Bruno RL, Cohen JM, Galski T, Frick NM. The neuroanatomy of post-polio fatigue. Arch Phys Med Rehabil 1994;75:498-504.