PHI’s 120-page book, containing 90 entries in dictionary format, is a compilation of the research and experience of more than 40 experts. A valuable resource for polio survivors and their health professionals.
Edited by Frederick M. Maynard, MD, retired physiatrist, currently living in Marquette, Michigan, and Joan L. Headley, MS, polio survivor and past Executive Director of PHI. Copyright 1999.
The Handbook, first published in 1984, retains the easy-to-use dictionary format in presenting information appropriate for polio survivors and health professionals who treat them.
Available for $12.00 USA or $20.00 International (postpaid).
For Contributor/Sustainer Members: $9.00
The Handbook is temporarily unavailable for purchase. An updated edition will be available soon.
Adaptive equipment enables people with disabilities and the elderly to accomplish life's tasks independently and safely. Health care professionals, such as occupational therapists and physical therapists, can assess the functional abilities of the person with a disability, assist in selecting the appropriate equipment, and provide training with that device, if needed. Adaptive equipment may be acquired commercially and may need to be modified or customized. The term covers a whole gamut of appliances, gadgets, utensils, and implements which can be used in almost every area of daily living. Some are very simple and have been available for years, such as enlarged handles for an easier grip. Other adaptations, a result of new technology, are complex and expensive. ABLEDATA (800-227-0216; www.abledata.com) maintains a listing of assistive technology and rehabilitation equipment.
Assistive technology exists for almost every functional deficit, such as weakness in the upper or lower extremities, the use of only one hand, or difficulty in reaching the feet. Many implements such as reachers, bath benches, raised toilet seats, as well as eating, hygiene, and dressing aids, used to be marketed only to people with disabilities but now are available from mainstream retail stores, pharmacies, and mail order catalogs.
Voice-activated computers, zero-effort steering for automobiles, and automated home environmental controls are available for individuals with significant disabilities. State-of-the-art technologies such as these are expensive but are becoming less costly with the passage of time. The aging of the "baby boomer" generation bodes well for people with disabilities. More research time and money will be applied to developing improved methods of compensating for the functional deficits that will occur as this large population ages.
Each state has a federally-funded assistive technology project that can be found through an independent living center, a state's vocational rehabilitation office, or other disability-related services. While the primary objective of most of these projects is advocacy for policy and system change related to assistive technology, they can be a good resource for locating, evaluating, and/or borrowing equipment.
Alternative therapies, also called complementary, can support natural self-healing and encourage behaviors that promote a sense of overall well-being. Some alternative therapies originated in diverse cultures and in earlier times; others have emerged from new discoveries in science. In one survey, the use of alternative therapies by people with disabilities was higher than in the general population (Krauss et al., 1998). People use alternative therapies because of their actual and perceived effectiveness, the widespread and erroneous belief that they are natural and do no harm, and because their use offers the opportunity for more control over treatment options and procedures. Alternative therapies can reduce stress, pain, and/or fatigue. Some therapies are covered by health insurance when offered by licensed health care providers.
Nutritional therapies include special diets, dietary supplements, and herbal products. While the recommended daily intake of vitamins and minerals continues to be debated and studied, cell maintenance and growth requires at least the minimum recommended dietary allowance, and polio survivors should pay particular attention to the daily intake of nutrients (see Nutrition). Herbs should be evaluated for possible interactions with medications. The herbal industry is not regulated by the Food and Drug Administration. As a safeguard, always purchase standardized products through reputable health food companies or pharmacies.
Exercise that involves gentle stretching and slow movement is often recommended for polio survivors. Yoga, yoga breathing, t'ai chi, and qi gong, with or without modifications, promote flexibility, improve oxygenation, and decrease general fatigue. Newer non-traditional techniques include watsu, a water-based version of shiatsu, and ai chi, an aquatic version of t'ai chi. When considering these therapies, request experienced practitioners with special training in therapeutic and adaptive techniques.
he relaxation response, a healthy state in which metabolism is slowed and muscle tension decreased, can be achieved in many ways: visualization and guided imagery (Rossman, 1993); progressive muscular relaxation; and yoga, meditation, and prayer (Benson, 1996). These self-care techniques provide health benefits including decreases in pulse, blood pressure, respiratory rates, and muscle tension. Research on people with chronic pain who regularly practice the relaxation response shows decreases in the severity of pain, anxiety, depression and anger, and an increase in activity (Caudill et al., 1991). Relaxation techniques can be learned from tapes, classes, or trained individuals.
Bodywork includes myofascial release (Smith, 1997), various types of massage, craniosacral therapy, chiropractic treatments, Trager, Alexander Technique, and trigger point therapy (Goldberg, 1995). These hands-on therapies are offered by specially trained people including some osteopathic physicians, physical therapists, or chiropractors. Improvements in range of motion, pain, and fatigue are often reported.
Energy work includes acupuncture and acupressure, traditional Chinese medicine techniques (Guang et al., 1987); therapeutic touch, developed in nursing (Gerber, 1988; Smith, 1997); and reiki and polarity (Goldberg, 1995). All require specialized training in balancing a person's energy flow. Treatments typically promote a sense of well-being and relaxation. Improvements in pain and fatigue have been frequently reported.
Other adjunct therapies, some of which are more conventional, tried by polio survivors are hydrotherapy (see Aquatic Therapy) to ease pain and muscle spasms; biofeedback for learning to promote relaxation and increase oxygen flow to muscles (Schwartz & Schwartz, 1993); hypnotherapy for relaxation and pain relief (Hammond, 1991); magnet therapy for pain relief (Vallbona et al., 1997); and transcutaneous electrical nerve stimulation (TENS) units applied externally to reduce pain. Homeopathy, in which self-healing is stimulated by minuscule doses of a natural substance prescribed by a homeopath, and aromatherapy, which promotes relaxation and improves sleep by breathing or skin absorption of flower essences, have also been tried.
When choosing alternative therapies:
- Do not assume all pain and fatigue are related to prior polio or that all post-polio pain is the same. Try to determine the location and the factors that instigate or increase pain. Understand the problem as completely as possible before choosing strategies.
- Read up on therapies under consideration and interview practitioners and some of their patients. Choose a qualified practitioner who will individualize therapies.
- Select ones that may be of significant help but have minimum potential for harm. Use alternative therapies, if appropriate, in addition to conventional medical interventions, including medication.
- Disclose and discuss any plan for their use with other members of the health care team.
Modern anesthesia has become extremely safe, but many survivors fear it because of reports of problems during and after anesthesia. Potential problems include a greater sensitivity to the paralyzing drugs (muscle relaxants), possible need for mechanical ventilation after surgery, and pain problems after surgery. All survivors, especially those with a history of respiratory involvement, need to tell their surgeon and anesthesiologist about having had polio (Calmes, 1997).
The kind of anesthesia chosen – general, regional, or monitored anesthesia care – depends on what operation is being done, the individual's medical conditions and desires, and the skills of the anesthesiologist and surgeon. There are theoretical reasons to choose regional anesthesia if possible, because it decreases the stress hormone response to surgery (Barker et al., 1995). Planning the proper anesthesia can only take place after the anesthesiologist learns of the survivor's medical problems and takes into consideration the treatment plans for the period after surgery, such as whether mechanical ventilation might be needed, and how pain will be treated.
Past reports of not being able to reverse muscle relaxants, most likely due to drug overdose, have contributed to the fear of general anesthesia. Polio survivors are very sensitive to muscle relaxants because they have fewer neurons to block (Gyermek, 1990). The muscle paralyzing drug curare is rarely used today. Anesthesiologists now choose from better and more controllable muscle relaxant drugs and routinely measure each person's response to muscle relaxants using a nerve stimulator. A safe approach is for the anesthesiologist to use only half the normal dose at first and see how the individual does. More anesthetic muscle relaxant can be added, if needed, and the possibility of overdose is decreased.
No published study documents anesthesia problems in polio survivors. However, some survivors have thought that their typical post-polio symptoms became worse after regional anesthesia.
If spinal anesthesia is used, the current recommendation, for the general population as well as survivors, is to use the local anesthetic bupivacane instead of lidocaine. There are reports of possible nerve damage with lidocaine. It is considered to be safe for dental anesthesia and epidural anesthesia.
Post-operative mechanical ventilation may be necessary to help with breathing after surgery, especially major surgery. More common in the survivor who currently uses mechanical ventilation or needed it in the past, post-operative mechanical ventilation allows the lungs time to recover from the effects of surgery and anesthesia.
Many survivors seem to be very sensitive to pain, particularly in long-paralyzed limbs, and to need additional pain medicine post-operatively. It is important that post-operative pain management be planned. A combination of approaches often works best.
The surgeon can inject local anesthetic at the surgical site; the anesthesiologist can give pain medication, so it is working when the individual awakens; and the patient can be given continuous infusions of pain medications that can be self-adjusted.
One of the most important decisions related to surgery is where the operation should take place. Questions to ask include:
- Does the hospital have a surgical team with experience in caring for polio survivors or people with neuromuscular disease?
- Is the ICU available if post-operative mechanical ventilation is needed?
- Is the anesthesiologist available ahead of time and does he/she show concern for post-polio problems?
- Does the anesthesiologist measure neuromuscular transmission routinely and will he/she in this case?
- Does the surgeon seem aware of possible post-operative problems from polio?
Major medical centers with academic training programs in anesthesiology and an affiliated rehabilitation unit most often meet these criteria. People with fewer post-polio problems might be considered for surgery in an outpatient surgery unit, if it met the criteria above and if the individual has assistance at home after being discharged (see Hospitalization).
One should expect to see the anesthesiologist before having elective surgery to discuss one's health and concerns about anesthesia. If an individual is unable to communicate, it is important that an advocate who knows the potential problems speak with the anesthesiologist. If the anesthesiologist for either elective or emergency surgery does not seem to show proper concern, the individual has the right to refuse surgery until he or she is comfortable with the relationship. However, emergency operations are more difficult because there is little time to plan (see Hospitalization, Emergency).
For any surgery, polio survivors should be in the best possible health pre-operatively.
Many polio survivors report that their feet have always been cold to the touch, their skin a purplish color. As they age, their limbs become more sensitive to pain as the temperature decreases (Owen, 1985). When polio survivors were cooled in the laboratory from 86º F to 68º F, motor nerves functioned as if they were at 50º F, and survivors lost 75% of their hand muscle strength (Bruno et al., 1985).
The body's thermostat, the area of the brain that causes blood vessels to contract, and the hypothalmus, the part that controls the body's inner environment, may have been affected during the original poliovirus infection. Also affected were sympathetic motor nerves in the spinal cord that send the message to the capillaries of the skin to contract when it is cold (Bodian, 1949). Consequently, as the outside temperature drops, the capillaries do not contract and warm blood flows to the surface of the skin resulting in excessive loss of heat and cooling of the limbs. When the limbs cool, veins narrow, trapping venous (blue) blood in the capillaries. This causes the feet to look blue. The motor nerves of cold limbs conduct more slowly; the muscles contract less efficiently. Cold also chills tendons and ligaments (like putting a rubber band in the freezer), making them stiff and movement of weak muscles more difficult.
Blood vessels do exactly the opposite of what they do in the cold during a hot bath. Heat causes the arteries to relax, and oxygenated (red) blood rushes to the skin. It becomes bright red. Survivors are advised to be cautious when getting out of the bathtub because blood can suddenly pool in the legs and can cause a drop in blood pressure resulting in dizziness or even fainting (Bruno, 1996). Chronic pooling of blood in the legs causes foot swelling (see Foot Swelling) and increases with age.
Other recommendations related to cold intolerance include: dress as if it were 20º F colder, dress in layers and wear heat-retaining socks or undergarments made of polypropylene (e.g., GortexT or ThinsulateT) or wool, and put on clothes immediately after showering when the skin is warm. Electromyograms (see Electromyography) or nerve conduction tests must be performed in a room that is at least 75º F to prevent abnormal readings, and limbs should be warmed as needed before testing (Maynard, 1985). Heated blankets may be necessary in the recovery room after surgery (Bruno, 1996).
Communication is a two-way street. Participants in any relationship must have a mutual respect for the values, experiences, and skills that each brings to the encounter. Everyone is responsible for his/her own feelings, and experts recommend that feelings be expressed in the first person. Acknowledge them but do not place responsibility for them on someone else. For example, saying, "When such and such happens, I feel..." is preferable to saying, "You make me feel ..." Feelings are not right or wrong, good or bad. An honest expression of feelings by everyone allows the opportunity to understand each other's point of view and gives each the choice to make changes in his/her behavior. Expecting others to be mind readers about personal feelings or experiences is fraught with the potential for serious misunderstandings.
Communication around disability is difficult for both sides, not knowing what to say, where to look. Silence is often the default position for people with disabilities, because silence can protect privacy (Iezzoni, 1998). But silence reinforces the idea that there is something to hide. Many people with disabilities do educate others by speaking out and living by example. One explanation for silence from people without disabilities is fear. Having a disability represents loss of control. Unlike other minority groups, having a disability represents a category a person may join at any time, and one reaction is to invalidate those who personify it by not communicating with them (Iezzoni, 1998). The best advice for interacting with people with disabilities is to be caring and respectful, to offer choices and options and accept the answers, and to remember people with disabilities "are human."
Good communication between polio survivors and their health care providers is not only desirable, but essential for improvement in the quality of life of the survivor. Most physicians practicing today were not practicing during the time that polio was a fairly common disease, and few diseases are similar to polio. Survivors may need to provide information about polio and its late effects to their health care professionals. It may be useful to interview them until finding one who provides a comfortable atmosphere for talking about health concerns and feelings; to prepare a concise written summary of the medical history/experiences for a physician at the first visit; and to make a list of questions and concerns in advance, so important issues are not neglected during the appointment. Likewise, it may be useful to take notes during the visit or ask for a brief written summary of the diagnosis(es) and recommendations; to invite a trusted friend or family member to the appointment to function as another "set of ears" and/or as an advocate (Marny Eulberg, personal communication, 1998); and to analyze situations that were unsatisfactory in the past, such as feelings of being inadequately informed or powerless, to develop a strategy to prevent them from happening again (Korsch & Harding, 1997; Pincus & Sanford, 1992).
The main cause of respiratory difficulties in polio survivors is the inability to take a deep breath due to weakness of the respiratory muscles and to cough effectively to clear secretions and mucus plugs. The lack of a good cough can lead to further pulmonary complications such as pneumonia (including aspiration pneumonia) and respiratory insufficiency.
If an individual's cough is weak (less than 4.5 L [liters] per sec), the onset of a cold must be dealt with as a serious and potentially life-threatening situation. Manually-assisted coughing techniques can provide deep breaths to augment cough flows. Techniques include postural drainage, percussion, and abdominal thrusts administered by a therapist or a caregiver who places their hands on the individual's abdomen and deliver thrusts timed just before the person coughs. For an adult with less than 1.5 L of vital capacity, the individual should be given the greatest volume of air that he or she can hold in the lungs, either with an Ambu bag or volume ventilator, before the thrust (Bach, 1996). Mechanically-assisted coughing involves using a device such as Philips Respironics' CoughAssist©* which applies a positive pressure to the airway and then rapidly shifts to negative pressure to expulse mucus out of the airway.
The criteria for diagnosing post-polio syndrome include:
- a prior episode of paralytic polio confirmed by medical history, neurologic examination, and, if needed, an electrodiagnostic exam to show changes consistent with prior anterior horn cell disease (exam is not required for limbs with obvious polio paralysis);
- a period of neurologic recovery followed by an extended interval of neurologic and functional stability, usually 15 years or more, preceding the onset of new weakness;
- the gradual or abrupt onset of new weakness and/or abnormal muscle fatiguability (decreased endurance), with or without generalized fatigue, muscle atrophy, and/or pain; and
- exclusion of medical, orthopedic, and neurologic conditions that may be causing the health problems listed above. New weakness (usually accompanied by diminished function) is the cardinal symptom of post-polio syndrome.
Without a clear history of new weakness, the diagnosis cannot be made. In addition, the diagnosis cannot be made without excluding other likely causes of new weakness and other new health problems. For this reason, post-polio syndrome is called a diagnosis by exclusion (see Evaluation).
New SSA Ruling for Polio Survivors with "Post-Polio Sequelae"
Two Social Security programs provide benefits based on disability. Both programs define disability as the inability to engage in any substantial gainful activity by reason of any medically determinable physical or mental impairment(s) which can be expected to last at least a year or to result in death.
Title II Social Security Disability Insurance (SSDI) covers three categories of disabled workers and their dependents who are insured under the Social Security Act after having contributed FICA (Federal Insurance Contributions Act) tax from their earnings to the Social Security trust fund. The categories are:
- a disabled insured worker under 65;
- a disabled widow or widower age 50-60 if the deceased spouse was insured under Social Security;
and a person disabled since childhood (before age 22) who is a dependent of a deceased insured parent or a parent entitled to Title II disability benefits.
Under SSDI, disability benefits start six months after Social Security decides the disability began. After 24 months of SSDI payments, recipients are automatically enrolled, premium free, in Medicare Part A, hospital benefits. The other part of Medicare, Part B, which helps pay doctors' bills and other services, is available upon payment of a monthly premium.
Title XVI Supplemental Security Income (SSI) provides payments for individuals (including children under age 18) who are disabled and have limited income and resources. Under SSI, disability payments may begin as early as the date the individual files an application. In most states, individuals who qualify for SSI disability payments also receive Medicaid health care benefits.
Anyone fitting the above description(s) may apply for Social Security disability benefits any time after the onset of disability by phone, mail or in person at any local Social Security office. The initial claims process requires Social Security number; proof of age; names, addresses and phone numbers of doctors, hospitals, clinics and institutions with dates of treatment; a summary of jobs held and employers in the past 15 years; and a copy of the most recent W-2 form or federal tax return.
The Social Security office collects medical evidence from licensed providers and health care facilities regarding medical diagnoses, pain and other symptoms; physical examination findings; results of x-rays and tests; medications; past medical and surgical treatments; and functional activities which are affected by the disease process, such as grooming, dressing, walking, bathing, toileting and homemaking.
Other relevant information may include: work limitations involving concentration; pace in completing tasks; and limitations in standing, sitting, lifting, climbing, stooping, kneeling and manipulating items.
When the evidence from the survivor's own medical sources is inadequate, the review panel may require additional medical information from the treating professional(s) or an independent examiner, or non-medical evidence from social welfare agencies, employers, teachers and other practitioners. Ultimately, a team, consisting of a physician or psychologist, depending on the nature of the disabling condition, and a disability evaluation specialist from a state's Office of Disability Determination Services (DDS), makes decisions regarding disability.
If an individual is denied disability benefits (which is often the case), the person has 60 days to initiate an appeal. Appeals must be filed in writing and may be submitted by mail or in person to any Social Security office. The appeals process consists of four levels: reconsideration by another physician/administrator panel; hearing before an administrative law judge; review by an appeals council; and finally, an appeal to the US federal district court, a complex and expensive process. The whole process may take a year or more and may necessitate updated medical evaluations and resubmission of medical evidence forms.
Benefits continue as long as an individual remains disabled. Periodic case reviews are done to verify ongoing disability. The frequency of reviews ranges from every six months to once in seven years, depending on the expectation of recovery. Benefits will stop if the individual returns to work at a "substantial" level, defined as average earnings of $860 or more per month (January, 2006). Benefits also will stop if Social Security decides that a person's medical condition has improved to the point that the individual is no longer disabled.
In 1987, the Social Security Administration acknowledged the late effects of poliomyelitis and issued criteria for the evaluation of the ability of survivors to continue employment in its Program Operations Manual System (POMS). (The listing number for Evaluation of the Late Effects of Poliomyelitis was DI 24580.010E.3.)
On July 1st, 2003, the Social Security Administration updated the listing. It is now called DI 24580.010 Evaluation of the Postpolio Sequelae.
Survivors are advised to take an active role in the process by assisting with the collection of medical evidence and relevant information, following the claim from step to step, making copies of important documentation, meeting all deadlines, and appealing, if necessary.
For more information, call Social Security (800-772-1213) or visit www.ssa.gov. The National Organization of Social Security Claimants' Representatives (800-431-2804) can suggest an experienced disability lawyer.
Visit www.disabilitybenefits101.org, a website that helps visitors navigate the world of disability benefits.
Electromyography records the changes in electric potential of muscles by means of surface or needle electrodes. An electromyogram (EMG), the record of electromyography, characterizes the electrical activity of motor units and, in polio survivors, will typically show evidence of chronic neuropathy (a disease of the nerves) which reflects the paralysis of many years ago. During recovery from the acute disease, the terminal ends of the motor nerves sent out "sprouts" (Wohlfart, 1958) to the orphaned muscles resulting in larger than normal motor units, detectable by EMG (see Pathology).
EMG findings from polio survivors appropriately diagnosed with post-polio syndrome, and those not experiencing symptoms, are not significantly different (Cashman et al., 1987). Consequently, an EMG is not a test to diagnose post-polio syndrome. The diagnosis is a clinical one (see Evaluation) and a diagnosis of exclusion. Clinicians do use an EMG to confirm a history of prior polio if there is doubt, and to eliminate other diseases that may be causing the symptoms and/or co-existing with post-polio syndrome (Peach, 1997).
Research using EMG suggests that the enlarged motor units are not stable and that the disintegration of the reinnervated motor unit occurs over time (Wiechers & Hubbell, 1981). One study demonstrated an increased motor unit size with time after acute paralytic poliomyelitis (Stålberg & Grimby, 1995). Using motor-unit number estimation (MUNE), a study reported that 87% of previously affected limbs exhibited denervation as did 65% of supposedly unaffected limbs, and that the progression cannot be attributed to normal aging (McComas et al., 1997).
Many survivors need to work because their jobs provide a necessary source of income as well as health insurance and retirement benefits. Many derive a sense of satisfaction or an identity from work. However, some face the dilemma of fulfilling work obligations and demands which can increase the strain on overworked muscles and increase "global" fatigue affecting mental acuity (see Fatigue). The struggle to stay employed and the fear of potential loss of income creates a stress on personal relationships.
Survivors are encouraged to undergo periodic physical assessments to determine what kind of work can be done safely and to heed the advice of the evaluating physicians (see Evaluation) to prevent further harm. Health care professionals have noted anecdotally that survivors who are ill or injured may require three to four times the usual recovery period.
Early retirement can be delayed by implementing lifestyle changes and making workplace accommodations. For example, survivors can ask for assistance with certain tasks; use a ventilator while taking a brief nap during breaks; obtain a closer parking space; or use a city's special service transportation system. Employers may be required to provide accommodations, as covered by the Americans with Disabilities Act (see Americans with Disabilities Act). For example, employers could install special equipment such as a phone headset; invest in ergonomic furniture; reorganize job functions; modify work schedules; or install equipment to accommodate work in the employee's home. The Job Accommodation Network (JAN), a service of the President's Committee on Employment of People with Disabilities, has trained consultants available (800-526-7234 V/TDD; JAN at www.pcepd.gov) who can answer questions about job accommodations from both employees and employers. Businesses may take an annual deduction of up to $15,000 a year for expenses incurred to remove barriers in the workplace under the Architectural/Transportation Tax Deduction: IRS Code Section 190, Barrier Removal. Section 44 allows a tax credit; restrictions apply.
When issues related to work are raised, survivors are encouraged to look to employee policy handbooks, human resource manuals, benefit plan documents, and the Americans with Disabilities Act for guidance and assistance. Career and employment services are available through private, university, and library resources. Each state has federal- and state-funded vocational rehabilitation (VR) services designed to assist people with disabilities to obtain or retain employment. To connect with VR, check the phone listings under "Government, state."
Epidemiology is the study of disease in populations or groups rather than in individuals. Epidemiological studies provide important information about frequency, distribution, and the risks of developing a certain type of problem.
Many studies of polio survivors have been done on small, select populations such as individuals who seek treatment at a post-polio clinic, members of a support group, or subscribers to a newsletter. Due to this self-selection, the results often are questioned. Another criticism is the lack of data from longitudinal studies.
A few studies have been completed in which survivors were randomly selected from the population. One study of polio survivors in Olmsted County, Minnesota, between 1986 and 1993, identified 50 individuals who had had paralytic polio between 1935 and 1960. (They were representative of the total of 300 cases of paralytic polio in the county.) Detailed clinical and nerve function tests were carried out at the beginning and after five years. These studies demonstrated stable nerve and muscle function within this group. Although there were no signs of progressive paralysis, 60% of the survivors did report combinations of symptoms of pain, increasing weakness, and fatigue. In two-thirds of the symptomatic individuals, the causes of their symptoms were unrelated to earlier polio and were able to be treated. Overall, 20% of the individuals had muscle pain, perception of weakness, and fatigue, even though they did not have measurable progressive loss of muscle function (Windebank et al., 1996).
A population-based study from Allegheny County, Pennsylvania, using a survey validated by clinical examination, found a prevalence of 28.5% of post-polio syndrome in all paralytic cases. The risk was higher in individuals who experienced substantial permanent impairment after polio and in females. The strongest indicator was the polio/post-polio interval - not age at acute onset, acute severity, or level of physical activity after recovery. Incidence peaked at 30-34 years post-onset (Ramlow et al., 1992). It should be noted that exceeding that interval does not exempt one from future post-polio problems.
A 1987 National Health Interview Survey (NHIS), conducted in the United States, collected information from 821 polio survivors found in 50,000 households (120,000 people). The resulting estimate was of 1.6 million polio survivors, with 640,000 of them survivors of paralytic polio. The preliminary data from the 1994-95 NHIS (445 respondents) estimates 1 million polio survivors with 430,000 described as survivors of paralytic polio. The extensive survey traces the natural history of poliomyelitis and includes numerous questions about the "period of personal best," as well as the current period to capture the amount of decline, if any.
In a 1990 survey of 3,607 Danish paralytic polio survivors (estimated population of 7,200), the three most frequent complaints were considerable fatigue during exercise (59% in men; 76% in women), considerable weakness in previously affected muscles (52% in men; 67% in women), and joint pain (46% in men; 64% in women), with fatigue being the most common new symptom in both sexes (Lønnberg, 1993).
A 1994 Norwegian study (2,392), believed to be representative of all polio survivors in Norway, showed that 50% were experiencing new health problems related to their polio and reported an increased need for technical aids, personal assistance, and multidisciplinary health services (Wekre et al., 1998).
Muscle stretching and joint range-of-motion exercises are important whenever there is muscle weakness. Preventing tightness, where muscles are weak, is important to maximize function and is particularly important in the chest wall and abdominal musculature if there is a limitation of breathing capacity. Preventing tightness in the hip and knee is important to maximize walking ability when there is significant weakness of the hip and thigh musculature.
General conditioning exercises or aerobic exercises, specifically to maintain or improve cardiovascular endurance, are good for many polio survivors and have been shown to be effective (Owen & Jones, 1985; Kriz et al., 1992). The best endurance exercise is swimming (see Aquatic Therapy), because it minimizes mechanical stress on tendons and joints, but beneficially stresses the cardiovascular system.
Conditioning exercises or any repetitive activity, including walking, which causes pain or a sense of excessive muscle fatigue and increased weakness should be discontinued. The primary focus of any exercise program should be on building endurance, not strength (Agre et al., 1997; Ernstoff et al., 1996).
In general, muscles that are significantly weakened by previous polio respond poorly to vigorous strengthening exercise programs. Very gradual strengthening exercises which are guided in intensity and duration by the individual's level of fatigue and/or pain can lead to modest but significant improvements in strength (Agre et al., 1996). Exercise should be focused on functionally important muscles.
An appropriate exercise program will help to maintain the strength of previously involved muscles, and also avoid overloading those muscles which previously were not recognized as having been affected. An adequate exercise program will help to minimize loss in strength and endurance associated with the aging process. Professional advice may be needed to design a feasible and effective personalized exercise program.
Unaccustomed or disabling fatigue is one of the most common symptoms expressed by polio survivors and occurs for multiple reasons. Some polio survivors describe fatigue as a decrease in stamina, in endurance, and in the ability to perform repetitive actions (rapid muscle fatiguability), either measured in ambulatory distance or in the performance of upper extremity tasks. Others report a more global sense of tiredness, describing sleepiness, decreased attentiveness, and forgetfulness. Many require more than normally expected amounts of sleep, and frequently feel refreshed by a nap. Many polio survivors also describe a major decrease in stamina following illness, surgery, or trauma (Yarnell, 1988).
While electrodiagnostic studies have shown that polio-involved muscles commonly show signs of chronic denervation/reinnervation and defective neuromuscular junction transmission (see Pathology), not all polio survivors experience fatigue. Strength and endurance testing studies indicate that survivors who report new symptoms indeed do have greater residual weakness, reduced work capacity, and recover strength after activity less readily than asymptomatic polio survivors (Agre & Rodriguez, 1990). Gait analysis of survivors indicates that weakened muscles are required to work longer and with greater intensity to compensate for paralyzed muscles elsewhere in the lower extremities (Perry et al., 1987). It is clear that fatigue may result from poor pacing or pushing past the point of "tiredness" (see Pacing).
Chronic musculoskeletal pain (see Pain), frequently a complaint of survivors, can cause fatigue and irritability and can also lead to deconditioning and disordered sleep (Yarnell, 1988). Disturbed sleep contributes to daytime sleepiness and fatiguability. Many medications, such as narcotics and benzodiazepines, when used long term for chronic pain, disturb the restful phase of sleep and can contribute to a feeling of fatigue and irritability. Fatigue also can be attributed to medications (see Medications), such as beta blockers, or excessive use of alcohol or marijuana.
Deconditioning is another contributing factor to general fatigue. It can lead to disuse atrophy and new weakness of muscles, as well as reduced cardiovascular fitness (Stanghelle et al., 1993). Polio survivors may take three to four times longer to convalesce from illness, surgery, or trauma, compared to individuals who have a full complement of anterior horn cells. While staying in condition is important, each survivor must find the balance between overworking their delicate old reinnervated motor units and appropriate conditioning exercise (see Exercise).
Respiratory problems contribute to fatigue. Polio survivors may have obstructive or central sleep apnea (see Sleep Apnea) causing daytime sleepiness and fatigue. Survivors with increased respiratory muscle weakness may develop carbon dioxide (CO2) retention due to shallow breathing, especially at night. CO2 narcosis leads to fatigue and sleepiness during the day resulting in napping during conversations or periods of mild relaxation (see Underventilation).
Dealing with additional disability is emotionally draining for many and can lead to depression (see Depression) with decreased attention and concentration, or distractibility (Backman, 1987). Sadness and/or grieving for lost function (see Loss), as well as depression, can cause fatigue.
Finally, there is the theory that some polio survivors have fatigue as a result of having had polioencephalitis. These individuals may experience a sense of central fatiguability (decreased attention and concentration and memory loss) due to involvement of various brain structures including the reticular activating system (Bruno et al., 1991).
To adequately treat the symptom of fatigue, the various contributing factors need to be identified. Fatigue due to complications of chronic pain, deconditioning, medications, depression, or breathing problems, or fatigue due to dysfunction of the motor units (see Weakness) must be treated appropriately.
Being in the hospital can be a frightening experience for anyone. For polio survivors, planning in advance for hospitalizations and unforeseen emergencies is especially important.
A person who has lived with a significant disability for a long time is frequently the most qualified in managing his/her own needs for functioning and general day-to-day care. Hospital personnel should respect this expertise and be flexible and creative in adapting their medical procedures and skills to accommodate these needs. For example, survivors may present a list of medications to avoid or request use of their home mechanical ventilation equipment.
Maximizing an individual's safety and comfort during a hospital stay is essential. To be prepared, discuss hospital arrangements with a primary care physician and/or a treating specialist prior to admittance and preferably when the polio survivor is well. Issues to consider include:
- Specifying an individual who can act as an advocate when one cannot advocate for oneself.
- Allowing attendants or family members, if they choose, to continue their routine care during hospitalizations. This may also require that they stay in the same room.
- Informing staff of the individual's functional limitations due to prior polio along with instructions as to how one routinely functions, e.g., uses night-time ventilation; can walk only with a brace; cannot lie on right side, etc. Request that this information be inserted in the hospital chart.
- Scheduling a face-to-face discussion with the anesthesiologist several days prior to any surgery (see Anesthesia).
- Remobilizing, in the customary way, as soon as possible to curtail disuse weakness.
Emergency hospitalization for polio survivors with respiratory insufficiency is often complicated by the lack of experience of paramedics and emergency room staff with people with respiratory muscle paralysis. Respiratory equipment, such as cuirasses, pneumobelts, iron lungs, and many of the older ventilators, may be viewed by them as quaint artifacts of a past era. Portable volume ventilators and bi-level pressure devices, along with the newer nasal and face masks designed for home use, may also be unfamiliar. The tendency is to replace such equipment with the more familiar critical care equipment and techniques such as endotracheal intubation and tracheostomies. While these interventions may be necessary at times, they may be avoided by using and making adjustments to the existing equipment, such as the settings, interfaces, time on machine, etc. When an emergency necessitates a tracheotomy to save one's life, reverting to the usual equipment can be addressed, and most often successfully, once the crisis is over.
Polio survivors with respiratory insufficiency also are encouraged to discuss their unique requirements with their primary care physicians, pulmonologists, or specialists to obtain their agreement to act as an intermediary during emergencies. To help avoid inappropriate interventions during acute respiratory difficulties, familiarize local hospital personnel with the equipment the person uses.
To minimize confrontations and complications when emergencies occur outside of a local area, survivors are encouraged to carry an information sheet with brief instructions and contact phone numbers. Medic Alert coordinates a nationwide 24-hour response center which will transmit vital medical facts to assist with accurate emergency treatment. To sign up for the service, call 800-432-5378. The initial fees cover the application and an ID bracelet. There also is a nominal annual fee for the service.
Pre-planning (see Hospitalization) can reduce stress, complications such as nosocomial pneumonia, and the duration of hospital stays.
The independent living movement grew out of the anger and frustration of people with disabilities who were excluded from places of education, work, general retail, worship and recreation due to barriers in architecture, transportation and communications (see Americans with Disabilities Act). Attitudes about people with disabilities ranged from low or no expectation to the promotion of the super hero, perpetuating the idea that people with disabilities, with their various "flaws," must work hard and overcome their limitations to be accepted by society. Professional attitudes of "we know what is best for people with disabilities" also contributed to the discontent that propelled the independent living movement.
Gini Laurie, founder of Gazette International Networking Institute (now Post-Polio Health International), advocated for the rights and opportunities of people with disabilities for 30 years, and for this work she was singled out as a grandmother of the independent living movement. Recognizing that the movement was not isolated from the other social and political trends of the '60s and acknowledging that what people with disabilities really wanted was to be included in all aspects of society, Laurie preferred the phrase interdependent living (Laurie, 1988).
Independent living, as used by researchers and health professionals, typically is defined as "control over one's life based on choice of acceptable options that minimize reliance on others in making and performing everyday activities" (Frieden et al., 1979). Occupational therapists and physical therapists can provide an assessment of one's functional abilities and can provide options to increase independence in performing everyday activities (see Occupational Therapy and Physical Therapy). Independence does not require physically doing all the tasks of life alone, but does require that an individual with a disability have the opportunity to make independent decisions about life's tasks (Heumann, 1977). Inherent in making independent decisions is choice, and choice carries with it risk-taking and responsibility.
No curative medication is available for post-polio syndrome. Currently, medications can be used to treat the symptoms and to improve quality of life. Certain other conditions and diseases (elevated blood cholesterol levels, high blood pressure, heart disease and cancers) require use of medications with side effects that can exacerbate the general symptoms of post-polio syndrome, such as weakness and fatigue. These medications should be used, but with careful monitoring of the polio survivor's functioning.
Medications to reverse muscular atrophy or to improve muscle strength by stimulating motor nerve endings to reconnect with muscle fibers, called nerve growth factors, are all still experimental and are being tested currently for use with other neuromuscular diseases. Only insulin-like growth factor-1 (IGF-1), also known as myotrophin or somatomedin-C, has been scientifically studied in people with post-polio syndrome (Miller et al., 1997). (See following table for a summary of drug trials to date.) It brought no change in strength or fatiguability, but did improve recovery from fatigue after exercise. Human growth hormone has been given to increase a person's natural level of IGF-1, but showed little or no improvement in strength (Gupta et al., 1994).
Medications that protect the nerve-muscle connection (neuro-muscular junction) from new damage are called neuro-protective agents. Selegiline has been tested in a small clinical series of people with post-polio syndrome, bringing some possible improvement in symptoms but no clear stabilization of the disorder (Bamford et al., 1993). Although many people use over-the-counter antioxidant preparations of various types, these have never been formally tested to verify any ability to slow down the changes of post-polio syndrome.
Anabolic steroids, often used by body builders to improve muscle bulk and power, have been tried by polio survivors and other persons with neuromuscular diseases, but The Medical Letter on Drugs and Therapeutics reports that side effects, such as risk of prostate cancer in men and masculinization in women, greatly outweigh the potential benefits. Metabolic stimulants such as L-carnitine and co-enzyme Q, used to improve the ability of muscle to make energy and possibly reduce fatigue and improve strength, have also been tried by polio survivors (Lehmann, 1994), but have been associated with rare allergic reactions and insomnia. A placebo-controlled study of L-carnitine showed no significant difference between placebo and L-carnitine (Tesch, 1998).
Specific anti-fatigue drugs can act either in the brain (on pathways controlled by dopamine and noradrenaline) or by improving communication at the neuromuscular junction. These are, respectively, central and peripheral agents. Three centrally-acting anti-fatigue medications, amantadine, bromocriptine and selegiline, have been studied in post-polio syndrome. Amantadine provided no reduction in fatigue (Stein et al., 1995), but bromocriptine (Bruno et al., 1996) and selegiline studies suggested some benefit and may warrant further study. Pyridostigmine (Mestinon®), a peripherally-acting drug, has been scientifically tested because several clinical open trials (Seizert et al., 1994; Trojan & Cashman, 1995; Trojan et al., 1993) suggested improvement in fatigue. However, in a well-controlled, randomized, double-blinded study of pyridostigmine in people with post-polio syndrome, no improvement of fatigue better than placebo was found (Trojan et al., 1997). This excellent study illustrates the limitations of reporting a benefit from a medication based on small promising clinical open trials and quickly adopting its widespread use before a definitive scientific study is completed.
Medications for pain are used when rehabilitation techniques and rest do not give adequate relief. Overuse pain (see Pain) cannot be eliminated by medication, just covered up. When a true analgesic is required, whether it is as simple as acetaminophen or as strong as a narcotic, it should be taken in moderate amounts and on a schedule, not just when the pain is so severe that a higher dose is necessary. If taken together, mild antihistamines or anti-anxiety medication may make painkillers work better and at a lower dose, but do have their own side effects.
Many medications such as narcotics, sedatives, tranquilizers, sleeping pills, alcohol, antihistamines, antidepressants and anti-anxiety agents, may cause drowsiness as a side effect or may increase fatigue within the general population. Polio survivors who take these medications may experience an increase in polio-related weakness and fatigue. Always check the label or ask a pharmacist or physician about the side effects.
Diuretics and laxatives may deplete the body of essential minerals required by nerves and muscles for normal functioning. Many other drugs (antibiotics, chemotherapy agents, even megadoses of some vitamins such as B6) can contribute to nerve damage. Muscle relaxants and drugs similar to them in chemical structure (quinine, quinidine, procainamide), as well as other medications used for heart or blood pressure problems (beta blockers, calcium channel blockers), may add to polio-related weakness and fatigue. Anecdotal evidence suggests that cholesterol-lowering medications of the "statin" family may also increase polio-related weakness and fatigue. Polio survivors, particularly those with a lesser muscle mass, have anecdotally reported fewer and less dramatic side effects when taking a lower dose (determined through consultation with the physician) of a needed medication.
Polio survivors and their physicians should scrutinize all medications used to treat various medical problems to be assured that related conditions, such as fibromyalgia, elevated cholesterol, high blood pressure, etc., are appropriately treated, but with minimal effect on polio-related symptoms. Polio survivors are cautioned not to change essential medications without appropriate medical consultation or advice.
Occupational therapy (OT) provides purposeful activities or interventions in order to promote function, health, and wellness and to prevent further injury or disability. The goal is to assist survivors in achieving their maximum level of independence. To be seen by an occupational therapist, a referral by a physician is usually required.
Occupational therapists utilize purposeful, goal-directed activities to evaluate, facilitate, restore, or maintain a person's abilities to function in daily life routines of self-maintenance, work, rest, leisure, and play. Occupational therapists help individuals learn energy conservation, work simplification, time management, and pacing techniques; adaptive self-care techniques; and new skills for problem solving and for coping (see Coping) with needed changes. A home or job site visit, now being done by video in some rural areas, and evaluation may be necessary for the occupational therapist to suggest appropriate modifications to the environment, to recommend equipment, or to describe new ways to perform activities in order to maintain or improve endurance, decrease fatigue or pain, and improve the probability for task/activity completion.
Despite major strength, endurance, and range-of-motion limitations, many polio survivors over the years have developed remarkable and precise procedures to adapt and compensate. Due to the late effects of polio, some of these trusted routines are no longer possible. Therapists and polio survivors must develop a partnership in which there is respect for each other's expertise and ideas (see Communication), and then develop and implement a treatment plan which will assist in modifying the survivor's environment or lifestyle, so an optimal level of functioning and independence can be attained.
The goals of surgery following acute poliomyelitis were to correct deformities, stabilize joints and improve function. Surgery was recommended only when other treatments, such as muscle re-education, stretching of contractures, splinting and bracing failed (Nursing, 1948). The same is essentially true today, with the exception that surgery for the lower extremity rarely eliminates bracing, although it may increase bracing options (Perry & Keenan, 1995). Procedures done then and now include heel cord and iliotibial band lengthening; tendon and muscle transfers; and fusions of the foot (Perry & Keenan, 1995) and spine, including the placement of rods along the spine.
Aging polio survivors may require surgery to repair rotator cuff tendons, for joint replacement due to arthritis, and in some cases, to repair rehabilitative procedures done earlier in life. Others may require orthopedic surgery as a result of a break or fracture due to a fall. In all situations, special attention, including bracing and appropriate rehabilitation, is needed during the convalescent period to avoid developing contractures. The particular concerns are hip flexion, knee flexion and ankle plantar flexion. Overuse of arm muscles also must be avoided during convalescence from leg and spine surgeries.
Spinal surgery for scoliosis (see Scoliosis) is considerably more difficult for polio than for idiopathic scoliosis. It requires a surgeon with excellent technical skills and a team of health professionals who assist in post-operative care. A thorough cardiac and respiratory examination is essential. Additionally, individuals need to be ready for the physical and emotional stress that accompanies major surgery (Siegel & Transfeldt, 1995).
As with any surgery, polio survivors should consult with the anesthesiologist (see Anesthesia) and coordinate with the hospital (see Hospitalization). Most orthopedic surgeries require time for healing, and some polio survivors will need to plan for alternative ways of functioning. For example, rotator cuff surgery may require learning new ways to transfer or to use a wheelchair.
Orthotics is the use of braces and splints (orthoses) to biomechanically assist in supporting and stabilizing parts of the body affected by paralyzed and/or weak muscles (Bunch, 1985). Orthotics, grouped by a description of the area in which they provide support, are usually divided into three categories: lower extremity, upper extremity, and spinal. For example, in lower extremity orthotics, foot orthoses (FO) support and align the foot. Knee orthoses (KO) protect and support the knee joint. An orthotic device designed to support the whole lower extremity is called a knee-ankle-foot orthosis (KAFO). Upper extremity and spinal orthotics have similar classifications.
Increased pain, tripping, falling, dropping objects, and muscle loss are an alert to problems in the joints and muscles of the extremities (Redford, 1980). Many polio survivors who discarded their braces in earlier years, through therapy and sheer will, are in need of support once again. Bracing of joints and muscle groups can reduce pain; can prevent tripping and falling; may prevent further development of a joint deformity; and may conserve energy by making activities, such as walking, more efficient.
Communication among the polio survivor, the referring physician, and the orthotist is imperative to design the best possible brace. Today's braces are often constructed of lightweight plastics and metals with fixed (locked) and/or free (movable) joints, not of leather and steel. The referring physician prescribes the general type of orthosis, including core components such as fixed or movable joints, or metal or plastic fabrication. The physician also includes the diagnosis and the functional goals of the orthosis. The orthotist fabricates a design based on the prescription, including information from a gait analysis and information about the individual's home, work status, and physical activities.
In general, one must have a physician's prescription to be reimbursed for orthoses by any insurance company, including Medicare and Medicaid. Most states do not have certification laws for practicing orthotists, but many are certified by professional trade organizations after a minimum of two years of study and training.
Having to use a brace should not be viewed as defeat, but as making a lifestyle change that will provide added stability and safer, more efficient, and less painful mobility, thus enhancing continued independence.
Oxygen therapy should never be administered casually in the presence of elevated carbon dioxide (PaCO2) levels in the arterial blood, known as hypercapnia. Oxygen, even with as little as 2 L (liters) per minute, may cause a sudden and rapid rise in PaCO2. This may blunt respiratory drive and produce profound apnea with severe sleepiness, especially when oxygen is administered at night (Gay & Edmonds, 1995). It is recommended that polio survivors with hypercapnia confer with a sleep and/or pulmonary specialist prior to using oxygen alone for sleep. The more appropriate treatment may be some type of night-time assisted ventilation (Hsu & Staats, 1998) (see Underventilation and Ventilators).
Polio survivors, as with the general population, may have other conditions which are typically treated with oxygen. For example, oxygen therapy may be needed to temporarily supplement normal air levels when there is danger of tissue death, such as during an acute heart attack. Oxygen therapy may also be needed when the lungs are impaired, for example, from pneumonia or from emphysema. The decision to use, or not use, oxygen must be based on a thorough evaluation and understanding of the polio survivor's breathing problems (Hsu & Staats, 1998) (see Underventilation).
Pain can be due to any number of factors ranging from very benign to quite serious. Polio survivors who are experiencing pain should undergo a comprehensive medical evaluation to diagnose its cause. Pain is most often due to overuse of muscles, tendons, ligaments and/or joints, and primary interventions are directed at alleviating or eliminating the overuse factors.
Pain syndromes associated with the late effects of polio include muscle (myogenic) pain and cramping. Fasciculations, often described as a crawling sensation, are exacerbated by physical activity, stress and sometimes cold weather. Typically, myogenic pain and fasciculations will decrease or disappear entirely with rest (see Pacing, Conservation of Energy, Lifestyle Changes). Gentle stretching may be useful, but must be performed judiciously in situations when there is a greater functional benefit with tighter tendons (Gawne, 1997). Heat and gentle massage are useful adjunctive treatments as well. Fibromyalgia (see Fibromyalgia) and its associated pain have been noted to be more prevalent among polio survivors (Trojan & Cashman, 1995).
Strain injuries are not uncommon and affect the muscles, tendons, bursa and ligaments, and may occur chronically or acutely. Pain due to strain may be related to posture and/or occur as a result of overuse of the arms, shoulders and lower extremities (Smith & McDermott, 1987). Pain radiating from the shoulders is a result of supraspinatus or biceps tendinitis. Elbow pain is common, as is knee pain. Genu recurvatum (back knee) is a condition in which, because of weakness of the ligaments and muscles around the knee, there is progressive backward deformity of the knee (see Joint Deformities). To control or eliminate strain injuries and symptoms, the joints should be protected by bracing (see Bracing) or by a decrease in crutch use.
Another frequent cause of pain is degenerative joint disease. Degenerative changes, also in the spine, are exacerbated by weakened muscles and worsened by walking on unprotected joints with unusual gait movements and abnormal stresses. They can be lessened by improving support with appropriate bracing, adaptive devices (canes, crutches, corsets), special seating and postural modification.
Other pain problems that can occur are secondary nerve compression syndromes, commonly at the wrist and occasionally at the elbows (Werner & Waring, 1989). Median nerve compression, at the wrist (carpal tunnel syndrome), and ulnar nerve compression, at the elbow and wrist, are more prevalent in those who are crutch or manual wheelchair users than in the general population. Stress on the wrist and elbow can be reduced by using power carts, three-wheeled scooters, power chairs and/or by using hand splints.
A common site of pain in polio survivors, as a result of using a backward-sideward trunk lurch to substitute for weak hip muscles, is the lower back. Abnormal trunk movements transfer body weight to the small facet joints at the back of the vertebra, and they cannot tolerate the strain. The concentration of back motion at one level in the low back due to a spinal fusion or scoliosis is another cause of back pain.
Weak abdominal muscles also predispose one to chronic back strain and back injury. Abdominal binders, corsets or girdles can help substitute for weak abdominal muscles. Individuals who depend on excessive lumbosacral motion for walking may not tolerate certain corsets.
Physical therapy such as heat, massage, joint mobilization and stretching exercises can help control or resolve low back pain. A change in posture and gait pattern, such as using crutches or a rolling walker, may be needed to prevent recurrence or to resolve chronic pain. Due to increasing muscle weakness and muscle imbalance, some people may need to use a three-wheeled scooter or wheelchair to control this type of chronic pain.
Radiculopathy (disease of the nerve roots) may be the cause of pain in some polio survivors, particularly those who have abnormal posture and/or severe scoliosis, or neck or low back hyperextension due to trunk weakness. A body corset or body brace, if not being worn, may be an option in some cases, as is improved seating position. In other cases, traction and therapeutic modalities (ice, heat, massage, ultrasound, transcutaneous electrical nerve stimulation [TENS] and trigger point injections) may be beneficial. Symptomatic treatment with medications such as nonsteroidal anti-inflammatories (see Medications) may also be helpful, but their long-term use should be avoided. Surgery may also be needed in select severe cases.
Physical therapy (PT) can provide a comprehensive evaluation against which future problems might be measured; help with managing pain or decline in physical function; evaluate for new bracing and assistive devices or for use of existing devices; or educate in lifestyle modification.
A comprehensive evaluation that may take a few hours spread out over three to four visits should be performed. It includes an interview to obtain medical, social, work and activity histories; current functional complaints and abilities; idea of home arrangement; and a survey of currently used equipment and appliances. The evaluation should also include assessment of the following: heart and lung function, as they relate to activity performance; flexibility, with specific measurements at each joint in all planes of motion; strength (via manual muscle testing of each muscle) and quality of muscle contraction; sitting and standing posture; ability to move in bed and get up and down from the seated position; walking ability; and the skin and integrity of other soft tissues of the body.
The results of the evaluation should be explained by listing problem areas and the implications of these problems, along with proposed suggestions for treatment options to the survivor and significant others, if appropriate. Survivors should be permitted to choose those interventions in which they are willing to participate. A report detailing the evaluation should be sent to the referring physician.
PT intervention can seldom restore polio survivors to their peak level of functioning. However, with communication and cooperation among survivors, their significant others and the therapist, many goals can be achieved that contribute significantly to quality of life. Achievement of goals may require the use of bracing devices, canes, walkers, crutches, scooters or wheelchairs; implementation of changes in lifestyle; or commitment to a lifelong individualized, low-level exercise program (see Exercise). Survivors have choices in implementing such suggestions; however, their reluctance to consider such changes limits the benefits.
Treatment options may include: individualized therapeutic exercise for gentle stretching and strengthening; training to improve breathing, posture and gait; massage; treatment with devices that help to relieve pain, swelling and circulatory problems; assistance with ordering and instruction in the use of devices to assist with activities of daily living (ADL), walking or alternative means of loco-motion; and/or referral to other allied health professionals.
A physician's referral, or prescription, typically is required for insurance coverage of PT services. A referral with a specific diagnosis, such as gait dysfunction or knee ligament strain, is more likely to be covered than a general diagnosis of post-polio syndrome. Either specialists or primary care physicians may refer to PT, and polio survivors may consider asking for a referral.
Pulmonary function testing can be done in a pulmonary function laboratory. Simpler tests can also be done in a physician's office or in the home. Testing most often is used to identify airflow abnormality, reduced lung volume (restrictive abnormality), diffusing capacity changes (as in interstitial disease), and blood gas abnormality (too much carbon dioxide, too little oxygenation). The following tests are commonly performed.
Spirometry includes a number of simple measurements. Vital capacity (VC) measures the total volume of air one can breathe out completely after inhaling a full breath. VC is usually done forced, as fast as possible, and is known as FVC. When this fast forced expiration is performed, the volume breathed out in the first second is known as FEV1. The ratio of FEV1/FVC is an important parameter for assessing airflow and possible airflow obstruction. (Airflow obstruction is often defined as FEV1/FVC of less than 70%.) VC is sometimes done slowly and is called SVC. SVC avoids dynamic airway compression and may yield a larger VC than FVC, but cannot be used to assess airflow. The maximum airflow, or peak expiratory flow (PEF), and other airflow measurements can be made from the same forced expiration. These tests can be done while standing, sitting or lying supine. A significant change in VC when measured in a different position may be due to respiratory muscle (particularly diaphragm) weakness.
Tests used to evaluate neuromuscular diaphragmatic function include VC (either sitting or lying supine), maximum inspiratory force (MIF), maximum expiratory force (MEF), maximum voluntary ventilation (MVV), and nocturnal oximetry. MIF and MEF are measured by breathing in and out with maximal effort, through a closed mouth tube attached to a pressure measuring device. This measurement reflects inspiratory and expiratory muscle power. MVV is measured by breathing in and out deeply and rapidly for a short time. MVV is a more global measurement of maximal breathing capacity and respiratory muscle endurance.
Lung volume testing measures VC and the residual volume (RV) of air still in the lung after breathing all the way out. This allows calculation of the total lung capacity (TLC), which may be reduced due to causes such as neuromuscular disease, kyphoscoliosis and pulmonary fibrosis.
Diffusing capacity (DLco) is a measurement of gas transport (transfer) across the lung's alveolar air sacs and capillaries into the blood. Causes of a reduced DLco include interstitial pulmonary disease and fibrosis.
Blood oxygen, carbon dioxide, bicarbonate and pH are measured from an arterial blood specimen. Arterial blood gas (ABG) measurements can be done at rest or with exercise, breathing room air or oxygen. The oxygen saturation can be measured noninvasively using an oximeter. One type of oximeter has a memory module so that it can record 8-12 hours of oxygen and pulse rate data; this is useful to evaluate data noninvasively overnight (nocturnal oximetry).
Sleep studies are usually performed in a sleep laboratory with an overnight study recording multiple variables simultaneously to assess sleep disorders (such as sleep apnea or underventilation). These studies include EEG (brain wave), ECG (electrocardiogram), airflow measured at nose and mouth, oximetry, muscle measurements of chest and abdomen, and often a video record of sleep movements. As respiratory muscle weakness develops in neuromuscular disease, night-time underventilation and a drop in oxygen saturation may be an early finding. Sometimes nocturnal oximetry alone may provide sufficient information; this can be done at home.
A polio survivor with an abnormal sleep study should consult a pulmonologist experienced in neuromuscular diseases to determine the best treatment (particularly CPAP versus assisted ventilation for respiratory muscle weakness).
A polio survivor scheduled for pulmonary function tests should at least have spirometry with SVC (if possible sitting and supine), FVC, FEV1; MIF and MEF; and oximetry on room air. Nocturnal oximetry should also be considered since it is a simple useful test for abnormalities that often occur first during sleep.
Home or office monitoring of VC, PEF, and oximetry can be done with simple devices. For home monitoring, people often use a peak flow meter or an incentive spirometer (both are relatively inexpensive), to assess whether they are stable or worsening, especially during a respiratory infection. A nurse or respiratory therapist can easily measure the oxygen saturation on home visits with a small portable oximeter.
Defined as the lack of breathing through the nose and mouth for at least ten seconds, sleep apnea can be obstructive or central or mixed. Obstructive sleep apnea (OSA) occurs when tissues in the throat collapse and block airflow in and out of the lungs during sleep, although efforts to breathe continue. Central apnea occurs when the brain fails to send appropriate signals to the body to initiate breathing. There is neither airflow nor chest wall movement. Central sleep apnea is uncommon, and when present, is often associated with severe heart failure or the result of a stroke.
In sleep apnea, breathing ceases, oxygen in the blood decreases, arousal occurs, sleep ends and breathing resumes. The individual then drifts back to sleep and another apnea occurs with this cycle continuing throughout the night, resulting in hundreds of arousals from sleep. (People without neuromuscular disorders experience up to five apneas per hour of sleep.) The person arises in the morning unrefreshed, fatigued and is sleepy during the day.
Associated findings of OSA include loud snoring, obesity, positive family history of apnea and snoring, daytime sleepiness and when very severe, right-sided heart failure (see Cor Pulmonale), and hypoventilation (see Underventilation). OSA at first occurs when individuals sleep on their backs, but eventually apneic episodes are present with any sleep position. A number of factors make snoring and apnea worse, such as nasal obstruction. Smoking causes the lining of the upper airway to swell, alcohol and sedative drugs cause the muscles in the back of the upper airway to relax, and excessive weight decreases the size of the upper airway.
Many polio survivors have abnormal breathing during sleep, including both OSA and hypoventilation. OSA is probably more severe in polio survivors than in people without other medical problems. OSA also was found to be more common in obese survivors with normal lung function, whereas hypoventilation was found to be more common in those survivors with scoliosis, restrictive lung function, and a history of diffuse neurological problems during the acute phase of polio (Hsu & Staats, 1998).
Sleep apnea is diagnosed by polysomnography in an overnight sleep study, generally performed in a sleep laboratory by experienced technicians. The test monitors sleep stages, eye movements, snoring, airflow at the nose and mouth, heartbeat, chest wall breathing motion, and oxygen saturation. Oxygen saturation can be monitored easily in the home by nocturnal oximetry and serves as an adequate screen for severe OSA.
Medical treatment of OSA includes weight loss if obese, relief of nasal obstruction if present, avoidance of alcohol and sedative drugs, and sleep positioning on one's stomach or side instead of back. If apnea is more severe (15 to 20 apneas per hour of sleep or more), nocturnal noninvasive ventilation, such as nasal continuous positive airway pressure (CPAP) or bi-level pressure support, is often prescribed (see Ventilators). The upper airway in OSA is most vulnerable to closure during REM sleep (Ellis et al., 1987), and CPAP gently "splints" the airway open and stabilizes it. Obstructed breathing during sleep is relieved as long as nocturnal ventilation is used. Compliance with nasal masks has proven to be a problem in some people, particularly those with claustrophobia and those with OSA which is not very severe. These individuals do not have severe symptoms (sleepiness and fatigue), and they may not be convinced that nocturnal ventilation helps them (Hsu & Staats, 1998). Upper airway surgery to remove excessive tissue in the tonsils and soft palate areas, known as uvulopalatopharyngoplasty, is more effective to eliminate snoring than to cure apnea, but it is inadvisable in polio survivors who depend upon frog breathing (Alba, 1985).
The goal of a support group is to empower its members with the tools necessary to make adjustments needed to continue a life of dignity and independence. Contrary to the image sometimes portrayed in the popular media, healthy support groups are not "pity-parties" and do not promote the idea that "misery loves company."
During the original illness, many polio survivors were hospitalized for extended periods of time and established an esprit de corps. After successful rehabilitation, the same survivors lived active, integrated lives. Many of today's successful support groups have rekindled this sense of belonging to a unique group. Also contributing their perspective to support groups, however with some hesitation or even resistance, are individuals who never were a part of a group based on having polio or a disability.
- Share a common health concern.
- Govern themselves and their agenda with success dependent on each member's feelings of ownership.
- May use professionals as resource persons but not as leaders.
- Provide non-judgmental emotional support.
- Gather and share accurate and specialized information.
- Have membership which is fluid; newcomers are helped by veterans and become veterans who may outgrow the need for a group.
- Have a cause and actively promote that cause.
- Increase public awareness and knowledge by sharing their unique and relevant information.
- Charge small or no dues for involvement and typically struggle to survive (Laurie & Headley, 1988).
- Support groups provide a forum for people to learn from each other about how to enhance the quality of their lives (Koop, 1992). Historically, support groups related to a common health condition developed to help people resolve problems with bureaucracies. For this reason, groups are encouraged to operate with minimal structures and rules. Spending excessive time on organizational details detracts from the primary goals of providing personal support and advocating for systems' change.
Successful support groups promote "personal empowerment to overcome personal adversity" (Koop, 1992) by encouraging members to become active, assertive managers of their health care, challenging attitudes of helplessness, hopelessness and victimization. Successful groups create a confidential environment for people to share their feelings safely. Healthy groups balance a time for "me," a time for "us" and time for "you" (Koop, 1992).
Communication is vital in a support group. Members should be encouraged to own their ideas and reactions by using "I" statements, such as "I think ... I feel ... I suggest ..." Participants should avoid speaking for the group without consultation, generalizing by stating, "all polio survivors ...," or telling others what to do, such as "you should ..."
Equally important is listening to whoever is speaking by not interrupting or engaging in cross-talk (Ziegler, 1996). Sometimes distressed members digress on tangents and tell detailed stories rather than staying focused on the topic. To minimize these situations, groups should develop ground rules for the meeting time and recognize the limits of the group's role by encouraging persons who experience continuous or intense distress to seek professional assistance.
Effective leadership is also vital. Many groups function successfully with co-leaders or a committed core team. Effective leaders:
- Acknowledge members' ideas and seek suggestions and feedback.
- Consult with the group about major decisions and respect the group's consensus.
- Request assistance from members.
- Refer to the group as "our" not "my."
- Maintain an atmosphere of respect and order.
- Arrange for reputable, skilled speakers to present topics of interest.
- Obtain and offer names of ethical, knowledgeable professionals and service providers.
- Model self-acceptance and responsibility for their own health and well-being.
- Ensure that individuals who want to speak have an opportunity.
- Respond to the expression of emotion, tension, and conflict openly and with sympathy.
- Offer feedback but avoid authoritative directives by using "I suggest ..." statements, and intervene competently when appropriate.
Survivors join a support group for different reasons, voluntarily attend events and leave the group when they choose.
Although swallowing difficulty (dysphagia) is not recognized as a primary symptom of the late effects of polio, many cases have been reported in the literature (Coelho & Ferranti, 1988, 1991; Ivanyi et al., 1994; Sonies & Dalakas, 1991). Incidence of dysphagia in polio survivors has been estimated to be approximately 18% (Coelho & Ferranti, 1991; Cosgrove et al., 1987) and is most prevalent in those individuals who had bulbar polio (Buchholz, 1994). Many of these individuals may not have had swallowing problems during their initial bout of polio.
The dysphagia may include the following problems: unilateral or bilateral pharyngeal wall weakness, reduced ability to retract the base of the tongue and reduced laryngeal elevation resulting in decreased airway protection. These problems result in residues of food remaining in various areas of the pharynx, leaving the individual at risk for aspiration after the swallow (Logemann, 1998). Evidence also suggests that although impaired breathing may complicate swallowing dysfunction and vice versa, it does not appear that one can be predicted from the other. However, individuals with dysphagia and a reduced capacity to generate a productive cough appear to be at greatest risk for breathing difficulties (Coelho & Ferranti, 1991).
With regard to general guidelines for management of dysphagia, any individual suspected of having swallowing problems should be referred for a modified barium swallow (MBS) study, which is critical for defining the physiology of the swallowing dysfunction. The MBS study should, whenever possible, include presentation of a variety of consistencies, e.g., thin and thick liquids, purées, soft solids, solids, etc., and quantities of contrast material consistent with the polio survivor's everyday diet. The MBS study also is key to identifying the most suitable noninvasive compensatory techniques to facilitate swallowing function.
Swallowing function can be significantly improved by modifying positioning during swallowing, such as turning the head to one side or tilting the chin downward, or by modifying eating habits, such as avoiding certain consistencies or "problem foods," alternating liquid and solid swallows, and swallowing twice for each bolus of food. Polio survivors with swallowing problems should avoid eating when extremely fatigued. Even minor swallowing problems appear to be aggravated by fatigue. In most cases, aggressive exercise will fatigue the mechanism more than strengthen it.
Polio survivors with dysphagia should have their swallowing assessed at regular intervals to monitor progressive changes as well as to determine whether compensatory techniques continue to be effective.
Traumatic injuries, such as fractures, joint dislocations and severe sprains, strains and bruises, commonly require significant periods of immobilization and rest in order to heal. Deconditioning from bedrest or immobilization is a common phenomenon in neurologically intact individuals. Disuse atrophy of polio-affected muscles immobilized by casts and splints occurs rapidly, with rates of 5-10% loss of strength per day, or 10-20% per week. Weakening of other muscles often occurs because of reduced exercise/activity necessitated by the pain and immobilization of the injured body part. For example, people who must use crutches to walk while keeping weight off fractured leg bones, rarely walk their usual daily distances, are not able to perform their typical routines, and thus become deconditioned.
After a period of post-injury weakening and deconditioning, a polio survivor may take much longer to recover strength and conditioning to pre-injury levels. It has been estimated from clinical observations that the recuperation period after surgery, severe illness, or injury is at least three to four times longer. Survivors are vulnerable to additional strain injuries during post-injury periods due to altered activity and/or the post-injury rehabilitation efforts. They also are vulnerable to an increase in fatigue due to deconditioning as a result of weeks of reduced activity. Traumatic injury may make obvious previously asymptomatic respiratory muscle weakness and result in new breathing difficulties.
Therefore, it is not surprising that about one-third of polio survivors report the onset of "profound fatigue" and/or "post-polio decline" during a period after illness, surgery or trauma (Agre et al., 1989; Yarnell, 1998). Minimizing the period of immobilization and anticipating a longer and more carefully prescribed period of rehabilitation and/or recuperation after traumatic injury can be very helpful for, and is often crucial for, a post-polio survivor to make a full recovery to the pre-injury level of function.
New breathing problems in aging polio survivors can be insidious and often not recognized by either polio survivors or health care professionals. Individuals who used an iron lung, or barely escaped one, during the acute phase should be aware of potential problems to avoid underventilation and possible respiratory failure. Those survivors who did not need ventilatory assistance during the acute phase, especially those who had high spinal polio and who have upper body weakness and/or diaphragm weakness, may also be at risk.
Underventilation (hypoventilation) begins during sleep and results in an elevation of CO2 levels and a decrease in oxygen levels in the blood (Romaker, 1995). Causes include chest wall deformities such as scoliosis, respiratory muscle weakness, and sleep apnea, either central, obstructive or mixed (see Sleep Apnea).
Other contributing factors include smoking, obesity and diminished vital capacity (VC). A diminished VC is common in everyone who is aging. Polio survivors who have impairment of the diaphragmatic and/or intercostal (rib) muscles, or scoliosis, combined with the normal changes due to aging may lose VC at a rate of 60-90% greater than normal, thus exacerbating the development of underventilation (Bach, 1994).
Signs and symptoms include: daytime sleepiness, morning headaches, not feeling rested in the morning, need to sleep sitting up, sleep disturbances (including dreams of being smothered, nightmares, restless sleep, interrupted sleep), snoring, fatigue or exhaustion from normal activities, poor concentration and impaired intellectual function, shortness of breath on exertion, claustrophobia and/or feeling that the air in the room is somehow bad, anxiety, difficulty in speaking for more than a short time, quiet speech with fewer words per breath, use of accessory muscles to breathe, and a weak cough with increased susceptibility to respiratory infections and pneumonias.
An individual experiencing a combination of any of the above should immediately seek a respiratory evaluation by a pulmonologist (see Pulmonary Function Tests), preferably one experienced in neuromuscular disease, and possibly a sleep study. When VC declines from examination to examination to a range under 1 L (liter), assistance with ventilation must be considered (Bach, 1994).
Management of underventilation can be achieved through the use of noninvasive positive pressure ventilation (see Ventilators). Often nocturnal use is enough to correct the condition. Those who begin using nocturnal ventilation may find themselves gradually adding periods of ventilator use during the daytime. In some cases, invasive tracheostomy positive pressure ventilation may be necessary. Underventilation can be aggravated by the use of oxygen therapy (see Oxygen). Additionally, underventilation is a very serious condition which, if ignored and left untreated, can lead to death (see Cor Pulmonale).
The two types of trivalent polio vaccines currently available are the inactivated poliovirus vaccine (IPV) developed by Jonas E. Salk (1955) and administered by injection, and the oral poliovirus vaccine (OPV) developed by Albert B. Sabin and approved for use in 1962.
The World Health Organization (WHO) recommends the use of the OPV in its mass campaign to eradicate the wild poliovirus from the world by the year 2000. The OPV is easier to administer to large populations of infants and children and is less costly than the IPV injection. The OPV stimulates immunity in the intestines, a site where the virus multiplies, and reduces the risk of spread to other children.
Since the OPV is a "live" vaccine, there is a risk that one dose in 2.4 million will cause polio, or one in 750,000 individuals receiving their first dose will contract poliomyelitis (Centers for Disease Control and Prevention, 1997). Acute poliomyelitis also has been reported in non-immunized persons who came into contact with the poliovirus shed by an infant or child recently vaccinated with OPV, most often from changing diapers. OPV should not be used to immunize individuals with a compromised immune system, their household contacts, or nursing personnel in close contact. OPV should not be given to anyone who is pregnant or over 18 years of age, since they are reported to be more susceptible to vaccine-induced poliomyelitis.
The Centers for Disease Control and Prevention (CDC) recommends that travelers to countries where polio is epidemic or endemic receive the IPV before departure. For an up-to-date listing of the countries in question, call the CDC's automated vaccine information line at 888-232-3228 or the autofax line at 888-232-3299 or log on to www.cdc.gov and choose "Traveler's Health."
In the United States, the current (1999) immunization schedule against poliomyelitis approved by the Advisory Committee on Immunization Practices (ACIP), with the support of the American Academy of Pediatrics and the American Academy of Family Physicians, recommends that the first two doses of poliovirus vaccine be IPV administered at ages 2 and 4 months, followed by two doses of OPV at 12-18 months and 4-6 years. Use of IPV for all doses also is acceptable and is recommended for immunocompromised persons and their household contacts. OPV is no longer recommended for the first two doses of the schedule and is accept-able only for special circumstances, such as children of parents who do not accept the recommended number of injections, late initiation of immunization which would require an unacceptable number of injections, and imminent travel to polio-endemic areas. In June 1999, the ACIP recommended an IPV-only immunization schedule. The CDC must approve the recommendation before it takes effect January 1, 2000.
Ventilators suitable for home use are dependable and simple to operate and to maintain. Most are small enough to be portable and can operate on batteries (DC) as well as house current (AC). They are divided into two main groups: negative and positive pressure. The positive pressure ventilators are further divided into pressure-limited and volume units.
Negative pressure ventilators – The iron lung ventilates by enclosing the person's whole body, except for the head, in a chamber. Negative pressure is created intermittently in the chamber in the space around the body, causing the chest and lungs to expand for inspiration. Iron lungs are very dependable, very efficient, but are quite large, immobile, and limited to AC power. The Porta-Lung™ is a smaller fiberglass version of the iron lung and is somewhat more transportable.
Chest cuirasses, or shells, operate on the same principle as the iron lung. Instead of the whole body being placed in a chamber, only the chest and upper abdomen are covered. The cuirass is then connected by hose to a negative pressure ventilator (such as the NEV-100, Emerson 33-CR, or the old Thompson Maxivent). The cuirass is approximately 60% as efficient as the iron lung, but it cannot overcome a stiff chest wall effectively. Care must be taken to obtain a proper seal between the cuirass and the chest, or there will be a loss of pressure. There are problems of chest shell fit as body weight changes or as spinal curvatures increase. A negative pressure ventilator may also aggravate a tendency to upper airway obstruction, blocking effective ventilation, especially during sleep (Oppenheimer & Baldwin-Myers, 1993).
The rocking bed ventilates by tilting. The bed moves in an arc of about 20 degrees, alternating head down with feet down. With the feet down, the abdominal contents drop, causing inspiration, and move upward with the head down, causing expiration. This unit also requires that the person have no increased airway resistance. Contrary to popular belief, motion sickness is rare since the person is moving in only one plane. Although no longer manufactured, the rocking bed is still used by a few survivors to aid circulation and for daytime naps.
Positive pressure ventilators – Some polio survivors still use the pneumobelt, or exsufflation belt, as a companion to either the iron lung, cuirass, or rocking bed. The system includes an inflatable bladder, placed over the abdomen with a corset holding it in place. The bladder is inflated by positive pressure (30-50cm H20) pumped into it, causing the abdominal contents to displace upward, producing exhalation. When the pressure on the bladder is released, the diaphragm descends by gravity, thus facilitating inhalation. The individual must be sitting upright for inhalation to occur. The pneumobelt is about 50% as efficient as the iron lung and is useful if a polio survivor has compliant lungs. Those with a major degree of chest restriction, or mucus production, probably will not benefit from this system. Pneumobelts are still available on a rental basis, but many polio survivors are switching to other types of assisted ventilation.
Pressure-limited ventilators deliver air until a pre-set amount of pressure has been achieved in the circuit. The primary ventilators in operation from the late '60s until the late '70s, these units, such as the Monaghan, Huxley, and Bantam, are control-cycled with the respiratory rate pre-set. Controlled ventilation generally has worked well on individuals with severe quadriplegia, since these individuals exercise little and usually do not have to change their respiratory rate. These pressure-limited ventilators are no longer being manufactured or serviced; many polio survivors are converting to volume ventilators or to bi-level pressure systems.
In the mid '80s, bi-level pressure support systems became available for noninvasive nasal positive pressure ventilation (NPPV), usually with an interface of a nasal mask or nasal pillows held in place with headgear with adjustable straps. Lightweight (about 10 lbs.), easy to use, and more efficient than other noninvasive techniques to assist ventilation, these systems evolved from CPAP (continuous positive airway pressure) devices for treating sleep apnea and provide expiratory positive airway pressure (EPAP) as well as inspiratory positive airway pressure (IPAP). As of 1999, they are approved as assisted ventilation devices, but are not approved for continuous use over 16 hours per day (life support) or to be connected to a tracheostomy (Make et al., 1998).
Bi-level pressure support systems are approved to assist ventilation for people who can breathe spontaneously at least part of the time. They do not have a built-in battery, but an adapter is usually available for an external battery. Models are available with or with-out timed cycling and backup rate and have limited alarms. Examples include: Respironics Bi-PAP® S/T and Tranquility Bi-Level; Puritan Bennett KnightStar™ 320, 335 from Mallinckrodt; AirSep Remedy™; Sunrise Horizon Bi-Level; and ResMed VPAP II® ST (Resource Directory for Ventilator-Assisted Living). Examples outside the United States include: Breas® PV 101, PV 102, PV 401; Dräger RespiCare; Nippy from Friday Medical; and Taema DP90 (Simonds, 1996).
Portable volume ventilators, used since the late '70s, deliver a pre-set volume of air and adjust the pressure needed to deliver the air, depending on the airway resistance. Volume ventilators can be used to stack breaths to augment effective cough. They have an internal battery that will run the ventilator for about one hour and can be connected to an external rechargeable marine battery to provide portable power for over 12 hours. Equipped with built-in alarms, such as for disconnection or machine failure (low pressure) and for air blockage (high pressure), they are heavier and more expensive in the United States than the bi-level devices, but more powerful. Also, they have more safety features and thus are approved for use up to 24 hours per day for people using tracheostomy positive pressure ventilation (TPPV), as well as for people using NPPV with nasal mask or pillows, or mouthpiece. Examples include: Puritan Bennett LP6 Plus, LP10 from Mallinckrodt; Respironics PLV-100, PLV-102; and Bear 33. Outside the United States, examples include: BiOMS AIROX Home 2; Brompton PAC; Breas® PV 501; Dräger EV 801; Taema Monnal D, Monnal DCC; Saime Eole 2, Eole 3; and Puritan Bennett Companion 2801 from Mallinckrodt (Simonds, 1996).
Ventilators manufactured in the late '90s, such as Pulmonetic Systems' LTV1000™, Impact Instrumentation's UniVent™ Eagle™ 754, and TBird® Legacy from Thermo Respiratory Alternate Care, may prove to be a new generation of positive pressure ventilators for long-term home care: compressorless, turbine-driven, smaller, and lightweight. However, the efficacy of these units has yet to be reported by polio survivors.
When using a positive pressure ventilator noninvasively, a variety of nasal mask interfaces are available in addition to nasal pillows and mouthpiece assemblies. Many people can be ventilated with mouth positive pressure during the day (Bach, 1996). The mouthpiece can be placed on a movable arm to allow it to stay next to the person's mouth for intermittent use – "sip and suck." Persons with no vital capacity can be ventilated during the night with a nasal mask or pillows or a Bennett Seal Mouthpiece Assembly held in the mouth with a headgear assembly or straps. These ventilator users become conditioned to take in sufficient air during their sleep from a cycled ventilator with a fixed rate and a pressure set high enough to compensate for leaks.
The success of NPPV often depends upon an effective cough. There are manual techniques to augment a cough and improve clearance of bronchial secretions, as well as mechanical devices, such as the J.H. Emerson Company's In-Exsufflator to assist cough (see Coughing).
TPPV, invasive positive pressure ventilation via a tracheostomy, may be necessary when noninvasive ventilation is not successful (see Tracheostomy).
New muscle weakness is the hallmark of post-polio syndrome and can significantly impact activities of daily living. Some amount of new muscle weakness is likely to occur in about half of post-polio individuals (Jubelt & Drucker, 1999). Muscle weakness is most likely to occur in muscles previously affected during the acute poliomyelitis followed by a partial or full recovery (Cashman et al., 1987; Dalakas & Illa, 1991). Less frequently, "previously unaffected" muscles may also develop this new progressive weakness. However, these "previously unaffected" muscles had subclinical involvement at the time of the acute poliomyelitis. About 50% of the motor nerve cells supplying a muscle must die before a muscle is noticeably (clinically) weak in usual daily activities.
The new progressive weakness has many of the characteristics of weakness seen during the acute polio, i.e., it is usually asymmetric, can be proximal or distal, and is often patchy (weakness may affect one or two muscles in one extremity and skip the other muscles). New muscle weakness can involve specific muscle groups and can result in respiratory insufficiency (Bach, 1995), bulbar muscle weakness (swallowing difficulties, aspiration, loss of voice) (Sonies & Dalakas, 1995), and sleep apnea. Other symptoms resulting from new neuromuscular dysfunction are increased muscle fatiguability, described as the rapid weakening of a muscle with exercise that then recovers quickly (minutes to hours) depending on the amount of overuse (Sharma et al., 1994), and muscle pain and tenderness.
The new muscle weakness of post-polio syndrome has been estimated to typically progress at 1-2% per year. In these studies, strength was assessed by either manual muscle strength testing (Dalakas et al., 1986) or by mechanical strength measurements (Agre et al., 1995; Stålberg & Grimby, 1995). The course of new weakness is variable. Some survivors experience slow, continuous progression while others report a stepwise course with plateaus between periods of progression. Because of the slow and variable course of the weakness, it may be difficult to demonstrate progression (Jubelt & Drucker, 1999).
The exact cause of new muscle weakness (see Theories) is not known, but it is clear that there is progressive deterioration of the motor unit (Wiechers & Hubbell, 1981) (see Pathology). It frequently has been hypothesized that the increased metabolic demand on the nerve cell to maintain more muscle fibers than normal results in the premature exhaustion or death of the nerve cell, beginning with its distal processes. The overuse of muscles resulting in excessive muscle fatigue may contribute to this premature exhaustion of the motor units (Grimby et al., 1996; Sharma et al., 1994; Sivakumar et al., 1995).
To manage new weakness, it is important to use energy conservation measures (see Conservation of Energy) and to stop overusing weak muscles (Agre et al., 1996). This can be accomplished by pacing activities (see Pacing ) and using orthotics (see Orthotics) to support weak muscles. Also, it now has been demonstrated that nonfatiguing exercise programs can improve the strength of mild to moderately weak muscles (Agre et al., 1996; Jubelt & Drucker, 1999; Spector et al., 1996). The important point is to avoid frequent or continuous muscle overuse, or muscle exhaustion, and to use a nonfatiguing exercise program (see Exercise).
Throughout life, people move in one direction or another on the wellness continuum ranging from maximum performance on one end to serious illness or premature death on the other end. Polio survivors do not need to constantly struggle from one health crisis to the next. While some health problems require professional assistance, others can be controlled by the individual. Once they have seen appropriate health professionals to alleviate and manage the late effects of polio and other unrelated diseases, survivors should consider the following to improve their day-to-day overall sense of wellness and ability to participate in life.
Focus on personal wellness and the prevention of new health problems. Concentrate on being in the best state of health and/or enjoying a sense of harmony with oneself and life. Moving toward wellness is an ongoing process based on a personal decision to maintain a balance among the physical, emotional, mental, social and spiritual aspects of life.
Make conscious and achievable lifestyle and behavior choices that promote good health. The smallest changes, if consistently practiced, for example, eating one more piece of fruit a day or doing deep breathing exercises three times a week, can make a powerful difference over a period of time.
Conduct a self-inventory. What health and lifestyle behaviors are currently gratifying and working well? What unhealthy behaviors and habits would best be eliminated or diminished? What health-promoting practices are missing?
Commit to regular checkups to monitor health with a primary care physician who understands the strengths and limitations of being a polio survivor.
Read reputable, educational information on acknowledged, beneficial health practices, including tactical suggestions on how to achieve individualized wellness goals.
Set a few realistic wellness goals.
Connect with supportive people. Start or join a post-polio or disability group which emphasizes wellness, not "being sick." Knowledgeable health care professionals can present on a variety of health topics, such as advice on stress management, appropriate physical activity, balanced diets, smoking cessation. Other important topics related to wellness are breast self-examination techniques; screenings for cancer, diabetes and cholesterol levels; and adult vaccinations.