CONTENTS

From Barbara

Decades Later – Post-Polio Syndrome

Special Poem

What Internists Need to Know….

The Amazing Cucumber

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FROM BARBARA

For those of you that were not at our last meeting, we had a most interesting speaker, Glenn Morin, a pharmacist with Walgreen’s. Glenn went over various medications that we took – he also took blood pressures of those that wanted it.

A discussion was also had as to how our support group should proceed. Remember we sent out a survey locally to approximately 125 members (living in Volusia, Flagler, Orange, Seminole, Brevard and Lake Counties), as to how many meetings we should have each year, whether we should continue meeting at the Red Lobster on Sundays, and whether the newsletter should be continued. We received back more than half – either by mail or e-mail.

The results were basically as follows: -3 meetings a year

February – June – November

Continue at the Red Lobster on Sundays

and that the newsletter should continue, also.

What I found most interesting is that 3 of the 8 responses that wanted us to continue with 5 meetings a year – never came to even 1 meeting in all the years we’ve been meeting.

I’m diverting this year and not having a June meeting but having one in September – the reason for this is that our speaker, Dr. Zilioli will be in Australia for several months visiting family, but will be back for a September meeting.

Now, I did go on another cruise in March (in fact, during Daytona Beach’s Bike Week) – we went on the Norwegian Cruise Line. The inside handicapped cabin we had was the largest of any handicapped cabin I ever had on any ship line. You may remember that my friend, Selma, my roomie, rents a scooter for the cruise and it is delivered to the room for her. Well, when we got to our cabin we were absolutely shocked to see that not only could we easily get both scooters in but that we could probably get another two scooters in. The cabin could easily accommodate four people. We were going to Cozumel, Guatemala, Belize and then back to the USA via Key West. Didn’t go to Guatemala as one of the passengers had medical problems (cardiac arrest) and 3½ hours out of Cozumel we had to turn around so he could get the medical attention needed. We had an “extra” day at sea as we would not be able to meet the time restraints at the Guatemalan port. We did get off in Cozumel and did the duty free shops, as far as Belize, that is done by tender and I no longer can do a tender, so we stay on board and amuse ourselves playing cards, talking with other passengers, etc. Key West was also one that we could get off the ship, but we’ve been there several times before and it was a very short stop-over (approximately 4 hours), so once again we stayed on the ship. Neither Selma nor I have any difficulty with the fact that we stay on board.

This cruise had a little over 400 Spring Breakers from many different colleges. They were delightful, always wanting to know if we needed anything. It seems that whenever we wanted to go into our cabin they were there to help us open the door and hold it while we scooted in. They told us we reminded them of their grandmothers. On the last night of the cruise one of the events as a “Quest”, which was actually like a scavenger hunt, the team that was seating in the area Selma and I were in, didn’t ask us to be part of the team – they told us we were part of their team. It was a fun filled evening.

The staff was always helpful – when we would get to the buffet area, they would immediately come over to hold our dishes and go with us to get whatever we wanted. They would find us a table, would come back to see if we wanted more coffee or, in my case, tea. Our cabin steward always seemed to be available when needed. In the casino there was plenty of room to scoot around. I am not a slot player but do enjoy a $5 Black Jack game – the pit bosses made sure I could get into either the first or last spot so I could play. The players at the table had no problem with moving to accommodate me. I even came out a winner - $50.

Now, for those of you that may not be using a scooter or want to take one you own on a cruise, be aware that they can be rented. The company leaves the scooter in the cabin and picks it up in the cabin at the end of the cruise. If you need help getting onto the ship, you can request wheelchair assistance (like you do at the airport) and they will help you get onboard.

Just to let you know, we’ve booked another cruise with Norwegian next February and requested the same handicapped cabin. But, before that, we’re booked on Holland America for Thanksgiving week, this year – an 11 day cruise of the Southern Caribbean.

That’s it for now – hope to see our local members at the September meeting.

Wishing everyone a truly great rest of Spring and a fantastically fantastic Summer.

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Note: The following article was written for a continuing education program for nurses and published in Nurse Week (Heartland Edition), September/October 2007. I found it to be one of the best descriptions for educating nurses and doctors (as well as family members) of what we, who have post-polio syndrome, are enduring. Permission to reprint is courtesy of Nursing Spectrum CE, a division of Gannett Healthcare Group. Carol Norris Vincent

Decades Later:

Post-Polio Syndrome

By Maureen Habel, RN, MA

The goal of this program is to educate nurses about post-polio syndrome, its symptoms, and associated health problems and to describe ways to help PPS patients manage symptoms and prevent complications. After studying the information presented here, you will be able to —

• Describe how post-polio syndrome is thought to develop.

• List six health-related problems that patients with PPS experience.

• Identify ways in which nurses can help patients with PPS manage symptoms and prevent complications.

These are some of the symptoms experienced by people who had polio as children or young adults decades ago. Between 12 million and 20 million people worldwide have a polio-associated disability.¹ In the United States, an estimated 440,000 polio survivors are now experiencing or at risk of post-polio syndrome.²

The Salk and Sabin polio vaccines, which became widely available in the United States in the 1950s and 1960s,³ have eradicated polio in developed countries. As a result, today many healthcare providers know little about polio infection and its consequences.

When people with PPS use the health system for treatment of common medical conditions, they are at risk for serious complications and often depend on nurses to advocate for their safety, independence, and well-being.⁴

The term poliomyelitis is a combination of the Greek words polios, or gray, referring to the gray matter of the CNS, and myelos, referring to the myelin sheath around some nerve fibers.⁴ A virus that damages or destroys the body’s motor units in the brain and spinal cord causes acute poliomyelitis.⁴ A motor unit is a motor neuron and all the muscle fibers that it innervates.⁵ Motor neuron death deprives muscle fibers of their nerve supply, resulting in flaccid paralysis and muscle atrophy. Although flaccid paralysis results in loss of muscle tone and reflexes, muscle sensation is not affected.

The polio virus spreads person to person by contact with secretions from the nose and mouth of an infected person or by contact with infected feces.⁶ A fever and GI distress are initial symptoms. If the virus enters the vascular system and invades the CNS, the person experiences a rapid onset of muscle pain, neck stiffness, headache, and increasing fever, followed by varying degrees of paralysis.^4,6

Paralytic poliomyelitis is classified as spinal, bulbar, or spinobulbar. Spinal polio affects motor neurons located up and down the spinal cord. Bulbar polio affects the brain stem, including cranial nerves that control vital functions, such as breathing and swallowing.⁶Death from bulbar polio is usually due to respiratory failure. People with spinobulbar polio have both spinal cord and cranial nerve involvement. Damage to motor neurons may affect many segmental layers of the spinal cord. If the cervical segments above C4 or C5 are affected, the diaphragm is weakened to the point that the patient will need a form of mechanical ventilation to live.⁴

In the polio epidemics of the mid-20th century, iron lungs supported respiration for those with bulbar polio and patients with weakened respiratory muscles. Enclosing nearly the entire body, an iron lung is a large negative pressure tank that first places pressure against the chest wall to expel air, then allows air to reenter the lungs.⁴

Therapy consisted of sprinting and bracing to support weak or atrophied muscles, hot packs, muscle stretching, and exercise.³ Surgery to improve function included muscle transfers, tendon lengthening, and osteotomies, procedures to shorten, lengthen, or change a bone’s alignment. Rehabilitation focused on patients’ physical effort and determination to minimize, if not overcome, disability.⁴ Most polio patients regained function.

In the late 1970s and early 1980s, people who survived the polio epidemics of the 1940s and 1950s began to report symptoms such as progressive muscle weakness, overwhelming fatigue, muscle and joint pain, cold intolerance, sleep disorders, heightened sensitivity to anesthesia, and swallowing and breathing problems.⁷ Because polio was thought to be a stable condition, the symptoms were dismissed or attributed to other causes. In 1984, this pattern of symptoms among polio survivors was increasingly evident, and the constellation of symptoms was first referred to as post-polio syndrome.8 PPS is a diagnoses of exclusion; see [below] … for the criteria to establish a diagnosis.

Some people with PPS experience only minor symptoms while others suffer serious disability. PPS symptoms usually develop gradually, but they may be triggered by trauma, infection, surgery, an illness that results in a period of inactivity, or a physically or emotionally stressful event.³ The severity of symptoms is related to how much disability the person had at the time of the acute polio infection. Those who had minor disability tend to experience mild PPS symptoms. Polio survivors who had the most residual disability and who achieved a greater functional recovery are those who may develop more disabling PPS symptoms.²

What’s the cause?

Degenerating post-polio motor units are thought to be responsible for PPS symptoms.^5,9 In an effort to compensate for the neurons that were destroyed during the acute polio infection, the axons of some unaffected neurons grew new nerve terminals through a process called “sprouting.” New nerve terminals began to innervate muscle fibers that had been cut off, or “orphaned,” from previously healthy nerves.² This adaptation is thought to be responsible for much of the recovery that polio survivors experienced. It’s thought that these motor neurons, some of which have often grown to eight times normal size to respond to metabolic demands, can

no longer carry such a heavy load.⁵ The result is the progressive muscle weakness characteristic of PPS.2 Motor unit degeneration can also cause changes at the neuromuscular junction, producing the pervasive fatigue associated with PPS.⁵ Although motor neuron loss begins to occur naturally and gradually in older people, this process is accelerated in people with PPS.

PPS can affect polio survivors decades after recovery. In fact, the more time that has passed from the year of the polio infection, the more likely a polio survivor is to experience PPS.¹⁰

Because people with PPS often experience multisystem effects as a result of having had polio, an interdisciplinary team approach is optimal.⁵ Team members may include a primary care physician, physiatrist, neurologist, physical therapist, occupational therapist, speech pathologist, psychiatrist or psychologist, pulmonolgist, orthopedist, rheumatologist, dietitian, nurse, orthotist, and respiratory therapist.⁵ Most people with PPS report pain, weakness and fatigue, memory problems, breathing and swallowing problems, sleep disturbances, cold intolerance, urinary problems, and emotional stress.

In about 25% of people with PPS, the associated pain is severe enough to interfere with ADLs [activities of daily living].¹⁰ Pain often occurs in the neck, back, and extremities. Joint pain may be caused by chronic overuse of muscles that appeared to be undamaged by the initial polio infection. For example, the right leg of a person with weakened muscles in the left leg may develop pain in later life because of overuse and overcompensation.⁹ Hot or cold packs, biofeedback, and massage can help minimize pain.⁸ Nonsteroidal anti-inflammatory drugs can also help relieve muscle and joint pain.⁹

Fatigue is the most common PPS symptom; up to 91% of people with PPS report new or increased fatigue.¹⁰ People experiencing post-polio fatigue describe physical exhaustion characterized by muscle weakness and decreased endurance and a central fatigue associated with cognitive problems.⁹ For instance, people with PPS may report memory problems, such as difficulty with word finding, especially when they are fatigued or stressed. Deconditioning also exacerbates fatigue. Many polio survivors are significantly deconditioned and may take much longer than expected to recover from illness, injury, or trauma.¹¹

Strategies to reduce PPS fatigue and weakness include exercising carefully under supervision, avoiding muscle overuse, losing weight, bracing weakened muscles, and using assistive mobility devices, such as a wheelchair or motorized scooter.⁵ A tool such as the Borg Rating of Perceived Exertion scale can help patients assess their perception of fatigue and limit activities that cause excessive fatigue.⁵

Energy conservation is an important part of managing PPS fatigue. People with PPS need to pace physical activities, schedule frequent rest periods, and eliminate unnecessary energy-consuming tasks.⁹ Pain, weakness, and fatigue can occur from overusing muscles and joints, but they can also be the result of muscle and joint disuse.² This has caused some confusion about whether to encourage or discourage exercise for polio survivors, including those who have PPS symptoms.² Polio experts emphasize that physical exercise is safe and effective when it is prescribed and monitored by health professionals.² Muscle stretching and joint range-of-motion exercises are important to counteract muscle weakness and reduce muscle tightness that can interfere with function.¹¹

If patients have breathing capacity limitations, keeping chest and abdominal muscles from becoming tight is especially important.¹¹ Exercise can also promote continued mobility in a person with hip and thigh muscle weakness.¹¹

Swimming is an excellent exercise for those with PPS; the buoyancy of the water helps prevent joint and tendon stress while the aerobic effects increase endurance.¹¹ Doing intense resistive exercises or using polio-affected muscles to lift weights can further weaken rather than strengthen muscles.² Exercise should be reduced or discontinued if the person experiences increased weakness, excessive fatigue, or prolonged exercise recovery time. Encourage people with PPS to listen to their bodies and avoid activities that cause pain or exhaustion. Teach patients not to overuse their muscles but to continue physical activities that do not worsen symptoms.¹

Physical therapists can prescribe exercises that strengthen muscles and improve endurance without causing fatigue, and occupational therapists can help patients modify their home environment to conserve energy. The most effective exercise regimen can help maintain muscle strength in previously affected muscles while helping to protect muscles that may not have been recognized as having been affected by polio.¹¹ Physical exercise can also help prevent fatigue and weight gain, which exacerbates the sedentary lifestyle associated with cardiovascular disease and osteoporosis.⁶

Respiratory problems — such as dyspnea, hypoventilation, and hypercapnia — and impaired swallowing are common with PPS. Early signs of respiratory impairment include fatigue, morning headaches, restless sleep, anxiety, breathlessness, and frequent respiratory infections.⁸ People with PPS are at increased risk of respiratory problems, such as atelectasis and pneumonia, because their weakened respiratory muscles prevent them from taking a deep breath or coughing effectively.¹¹

Interventions for respiratory impairment include appropriate positioning, increased fluid intake, incentive spirometry, and other forms of noninvasive positive-pressure ventilation.⁸ People with PPS need to pay attention to avoiding respiratory infections, which can worsen breathing problems. People with PPS should consider the first symptoms of the common cold, even a weak cough, as serious and potentially life-threatening.¹¹ To lessen the chance of respiratory infections, encourage people with PPS to avoid smoking, receive pneumonia vaccine, and have annual flu shots.^9,10 Obesity, spinal curvature, prolonged immobility, anesthesia, and medications that compromise respiratory function can also lead to acute respiratory failure.⁹

Use oxygen with caution in PPS patients. If a person with PPS has long-term respiratory impairment with higher-than-normal carbon dioxide blood levels, the stimulus for breathing is controlled by a low oxygen level. Increasing oxygen levels in a person whose respiratory function depends on hypoxia may cause respiratory failure.⁶ In situations in which oxygen is a primary therapy, such as an acute MI [myocardial infarction], the person’s respiratory status must be carefully evaluated.¹¹

Sleep apnea is common with PPS. Because sleep apnea can eventually produce cardiopulmonary failure, prompt diagnosis and treatment is important.¹⁰ Interventions range from avoiding a supine position to using continuous positive-pressure airway devices to help keep blocked airways open during sleep. Sleep disturbances may be caused by brain dysfunction or weakened upper airway muscles.

Dysphagia is often seen in polio survivors who had bulbar involvement. Swallowing problems can lead to malnutrition and dehydration and increase aspiration risk. Signs of swallowing problems include coughing, choking, and frequent throat clearing.⁸ Speech therapists can teach patients ways to compensate for swallowing problems, such as using pureed foods and thickened liquids, postponing eating when fatigued, and using specific swallowing techniques, such as swallowing twice for each mouthful and turning the head to one side while swallowing.^8,11

On the alert

Nurses must be keenly aware of the special needs of people with PPS and the risks they face when being treated for a variety of health conditions. When hospitalized, people with PPS may experience unusually severe pain, cold intolerance, self-care deficits, and impaired physical mobility and are at risk of developing respiratory insufficiency and impaired swallowing.⁴

PPS patients are at special risk when having surgery. Although anesthesia is very safe, many polio survivors are reluctant to have surgery because of reports of problems associated with anesthesia.¹¹ Potential complications include an increased sensitivity to drugs that paralyze muscles, the possibility of needing mechanical ventilation, and increased postoperative pain compared to people who don’t have PPS.¹¹

Muscle relaxants are particularly dangerous for a person with PPS who already has weakened respiratory musculature; polio survivors are more sensitive to muscle-relaxing agents because they have fewer neurons to block.¹¹ Because the initial polio infection might have affected the reticular-activating system (the part of the brain responsible for alertness), people with PPS are also anesthetized easily and take much longer than usual to awaken.⁸ Teach patients to inform their medical and dental healthcare providers in any situation in which anesthesia may be needed.⁶

Preoperative pulmonary function testing may be needed to assess whether postoperative mechanical ventilation will be needed. The need for mechanical ventilation is increased in those who use some form of ventilation assistance or those who needed it previously.¹¹ Postop ventilation allows the lungs to recover from the effects of surgery; however, it may prompt a psychological reaction from people who have traumatic memories of iron lungs or other types of breathing assistance.

In the case of cold intolerance, patients may need heated blankets in the postop recovery unit.¹¹ People with PPS are also at increased risk for vomiting and aspiration after anesthesia.⁸ Polio experts advise those with significant symptoms to avoid same-day surgery since they require careful and sometime extended monitoring to ensure that they can safely return home.⁶ Those with less severe symptoms may consider same-day surgery if the surgeon and anesthesiologist are experienced in treating polio survivors and if the person has adequate home assistance.¹¹

As a result of damage to neurons in the brain and spinal cord, people with PPS experience a heightened pain response. Injecting local anesthetic at the surgical site, giving pain medication before the person begins to recover from anesthesia, and providing patient-controlled analgesia using a continuous infusion of analgesia can help reduce pain.¹¹

Sedating medications should be given with caution. Common medications such as antihistamines, benzodiazepines, and some antidepressants can also cause profound sedation in a person with PPS.⁸Some antibiotics and certain antineoplastic medications can increase nerve damage to already compromised cells.⁸ Common prescription medications such as quinine, quinidine, procainamide, beta-blockers, calcium channel blockers, and statins can increase fatigue and weakness.^6,8

Emotional stress

Experiencing additional losses after a lifetime of adaptation can create profound emotional stress. Children with polio were removed from their families and cared for in large health centers. Many survived the helpless feeling of being in an iron lung, unable to move or breathe and dependent on others to meet their basic needs. During the acute infection, patients experienced severe muscle pain, followed by months of painful therapy. Many had several painful surgical procedures to restore function. Coping with a second disability is a formidable task, particularly for people who were taught as youngsters that the key to recovery was to push themselves as hard as they could.

Polio survivors had varying experiences during the acute polio infection. Some recovered quickly while others were hospitalized and isolated, and went through years of rehabilitation.^1,4 Those who were very young when they had polio may not remember much. Others may have vivid memories of pain and paralysis from the infection and treatments.

Not a return of polio

People with PPS may worry that they are having a reactivation of the polio virus and may infect others. But people with PPS symptoms have not been reinfected nor are they contagious.²Post-Polio Health International <www.post-polio.org>, a group devoted to education, research, and advocacy for polio survivors, advises people with PPS symptoms to seek medical advice from a physician specializing in neuromuscular disorders and recommends that all polio survivors have yearly health exams.¹ However, some polio survivors don’t seek medical attention because of a fear of reactivating memories of polio or a perception that health providers don’t understand the late effects of polio.¹They may also be weary of physical barriers, such as inaccessible medical offices. One nursing study found a low level of function combined with a high incidence of comorbidity in polio survivors. One of the top barriers to health was lack of supportive health providers.¹²

No intervention has been found to prevent motor neurons from continuing to deteriorate, but research continues.² Some researchers are studying mechanisms of fatigue and the role played by the brain, spinal cord, peripheral nerves, and neuromuscular junction, where a nerve cell comes in contact with the muscle it activates.²

Nurses can encourage polio survivors to contact support groups, such as Post-Polio Health International and the March of Dimes Birth Defects Foundation <www.marchofdimes.com>. Some may need individual counseling to cope with the losses PPS brings. Nurses can advocate for those with PPS in healthcare settings and educate them about situations that may worsen their condition. Knowing more about PPS can help nurses work in partnership with this unique and increasingly vulnerable population.

Criteria for Post-Polio Syndrome Diagnosis²

• Prior paralytic polio with evidence of motor neuron loss

• A period of partial to complete functional recovery, followed by a long interval of stable neuromuscular function

• The gradual onset of progressive new muscle weakness or decreased muscle endurance

• Symptoms persisting for at least a year

• Exclusion of other neuromuscular, medical, and orthopedic problems as the cause of symptoms

Clinical Vignette, Questions, and Answers for the Continuing Education Program not included

Maureen Habel, RN, MA, is a former director of nursing staff development and clinical nursing director at a major rehabilitation center in Southern California. The author has declared no real or perceived conflicts of interest that relate to this educational activity. Nursing Spectrum Continuing Education guarantees that this educational activity is free from bias.

References

1. The late effects of polio: an overview. Post-Polio Health International website. Available at: www.post-polio.org/edu/pabout.html. Accessed July 31, 2007.

2. Post-polio syndrome fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: www.ninds.nih.gov/

disorders/post_polio/detail_post_polio.htm. Accessed July 31, 2007.

3. Acello B. Handling an unwelcome comeback: postpolio syndrome. Nursing. 2003;33(11):32hn1-32hn5.

4. Hazel M, Strong P. The late effects of poliomyelitis: nursing interventions for a unique patient population. MEDSURG Nurs. 1996(5),

(2):77-85.

5. Post-Polio Health International. Handbook on the Late Effects of Poliomyelitis for Physicians and Survivors. St. Louis, MO: Post-Polio Health International. 1999.

6. Bruno RL. The Polio Paradox. New York, NY: Warner Books; 2002:xvii,10-39,120-173,198-225.

7. Halstead LS. Diagnosing postpolio syndrome: inclusion and exclusion criteria. In: Silver JK, Gawne AC, eds. Postpolio Syndrome. Philadelphia, PA: Hanley and Belfus; 2004:1-20.

8. Kramasz V. Polio survivors take a second hit. RN. 2005;68(11): 33-38.

9. Post-polio syndrome. Mayo Clinic website. Available at: www.mayoclinic.com/health/post-poliosyndrome/DS00494. Accessed July 31, 2007.

10. Bartels MN, Omura A. Aging in polio. Phys Med Rehabil Cl No Am. 2005;16:197-218.

11. March of Dimes International Conference on PPS: Identifying Best Practices in Diagnosis and Care. March of Dimes website. Available at: www.marchofdimes.com/files/PPSreport.pdf. Accessed July 31, 2007.

12. Harrison T, Stuifbergen A. Barriers that further disablement: a study of survivors of polio. J Neuro Nurs. 2001;33(3):160.

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I have a confession to make to you --- I don’t remember where this poem came from. I’m sure it was e-mailed to me. My apologies to whoever was good enough to send it to us. Your FECPPSG Editor – Barbara

SPECIAL POEM FOR
A row of bottles on my shelf

Caused me to analyze myself.
One yellow pill I have to pop

Goes to my heart so it won't stop.
A little white one that I take

Goes to my hands so they won't shake.
The blue ones that I use a lot

Tell me I'm happy when I'm not.
The purple pill goes to my brain

And tells me that I have no pain.
The capsules tell me not to wheeze

Or cough or choke or even sneeze.
The red ones, smallest of them all

Go to my blood so I won't fall.
The orange ones, very big and bright

Prevent my leg cramps in the night.
Such an array of brilliant pills
Helping to cure all kinds of ills.
But what I'd really like to know...........
Is what tells each one where to go!

There's always a lot to be thankful for if you take time to look for it.

For example I am sitting here thinking how nice it is that wrinkles don't hurt...

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My apologies – found this article and, honestly, don’t remember where and/or when I received it. Felt it was a good article though, and, therefore, decided to put it into this newsletter. I believe it Dr. Julie Silver is the author, but am not sure.

WHAT INTERNISTS NEED TO KNOW

ABOUT POST-POLIO SYNDROME

JULIE K. SILVER, MD - Medical Director, SpauldingFramingham Out-patient Center, Framingham, Mass; Assistant professor, Department of Physical Medicine and Rehabilitation, Harvard Medical School, Boston, Mass

DOROTHY D. AIELLO, PT -- Senior physical therapist, Spaulding-Framingham Outpatient Center, Framingham, Mass

ABSTRACT
Decades after recovery from polio, many patients develop new muscle weakness and other symptoms that can lead to increased debility. Treatment is aimed at the most prominent symptoms. Medica-tions may help, as well as physical therapy and a carefully paced exercise program. Screening for osteopenia and osteoporosis is recommended.

KEY POINTS

Postpolio syndrome affects an estimated 60% of "paralytic" polio survivors, plus unknown numbers of patients who had subclinical polio. Postpolio syndrome is a diagnosis of exclusion. Symptoms are related to new muscle weakness and may include muscle atrophy, myalgias, fatigue, and problems with swallowing and breathing. No drugs specifically address postpolio syndrome. Pyridostigmine has had mixed results for treating weakness and fatigue, as have methylphenidate and bromocriptine. Modafinil may be helpful for fatigue. Nonsteroidal anti-inflammatory drugs are used to treat pain. Rehabilitation professionals who have expertise in treating polio survivors can be valuable resources in preserving function and preventing deconditioning.

WHEN A POLIO SURVIVOR presents with nonspecific symptoms such as weakness and fatigue, how do you determine whether they are due to postpolio syndrome or to an unrelated problem? Postpolio syndrome is a neurologic disorder defined by a collection of symptoms occurring decades after a patient has recovered from an initial infection with the poliovirus. New muscle weakness is the hallmark, but breathing or swallowing problems, fatigue, myalgias, and cold intolerance are frequently also present.

In this review, we discuss the criteria for diagnosing postpolio syndrome, guide-lines for ruling out other conditions, and treatment strategies to optimize function in postpolio patients.

1 MILLION POLIO SURVIVORS

There are probably at least 1 million polio survivors in the United States, though not all have residual effects. Worldwide, there are millions more. Polio eradication is ongoing, and it is hoped that new cases will be completely eliminated over the next few years.

ACUTE POLIOMYELITIS MAY BE SUBCLINICAL

Historically and even recently, acute poliomyelitis has been thought of as having distinct presentations:

Abortive polio, which presents as a minor illness of fever, malaise, sore throat, anorexia, myalgias, and head-ache.

Nonparalytic polio, which presents as aseptic meningitis

Paralytic polio, which presents as severe back, neck, and muscle pain, with the rapid or gradual development of paralysis.¹In fact, however, the acute viral illness is probably more of a spectrum, in which there are subclinical cases of paralysis that in the past would have been classified as nonparalytic.² This concept is important because although most patients who are at risk for postpolio syndrome had well-recognized "paralytic" polio, others who were never diagnosed with polio or were thought to have had "nonparalytic" polio may also be at risk for postpolio syndrome.
An estimated 60% of "paralytic" polio survivors are affected by postpolio syndrome.³ The prevalence in those who had sub clinical illness is unknown.

WHAT CAUSES THE LATE SYMPTOMS

Postpolio syndrome occurs in polio survivors who had injury to their central nervous system, generally the anterior horn cells in the spinal cord, during the initial infection. The cause of the late symptoms is not well understood but is believed to involve attrition of motor neurons during aging.⁴ Other theories abound, however, and the etiology is likely multifactorial.

When motor neurons are lost in acute polio, the surviving motor neurons sprout collateral fibers that reinnervate the enervated muscle fibers (FIGURE 1). The resulting motor units are larger than normal, and there are fewer of them than before. Therefore, the burden on each of these remaining motor neurons is higher than under normal conditions. With age, we all gradually lose some motor neurons.⁵Polio survivors may be more affected by this loss of motor neurons because they have fewer to begin with. Another theory is that insufficient levels of acetylcholine are released at the neuro-muscular junction, resulting in diminished muscle contraction.⁵Maselli, et al⁶ noted reduced amplitudes of miniature end plate potentials and structural abnormalities of the neuromuscular junction, such as reduced diameter of nerve terminals, but these changes were not noted in all postpolio syndrome patients. Some have found ongoing immune activation and defective viral particles in the spinal fluid,⁷ although the significance of these is unclear. General fatigue may also have a central cause — an abnormality in the reticular activating system in the brain that occurred during the acute polio episode.⁵, ⁷

HALLMARK IS NEW WEAKNESS

The hallmark symptom of postpolio syndrome is new weakness, which may occur in muscles known to be previously affected or in muscles that were thought to be normal.³ The patient may report difficulty with walking or lifting items, falls, needing more assistance with transfers (eg, moving from the bed or commode to the wheelchair), and being less able to do functional tasks. The weakness charac-teristically worsens with increased activity and is most pronounced at the end of the day. Symptoms may also include dyspnea on exertion due to respiratory muscle weakness, other breathing or swallowing problems, pain (myalgias), cold intolerance, and unaccustomed fatigue. New muscle atrophy may also be present.⁸ Pain can be due to factors related to the history of polio, but which are not classifiable as postpolio syndrome. For example, a patient may present with left leg paralysis due to the initial polio and report increased limping and pain in the right hip (ie, the "good" leg). The new symptoms may be due to osteoarthritis of the hip, which is more likely to occur in a polio survivor without good muscular support around the hip and after years of additional wear and tear. Postpolio muscle pain classically occurs in the muscles rather than in the joints. The pain is often described as aching, cramping, burning, or a "tired" feeling. It frequently occurs at night or after the person has been very active.⁸ Numbness or pares-thesias are not typical symptoms of postpolio syndrome.

DIAGNOSING POSTPOLIO SYNDROME

Postpolio syndrome is a diagnosis of exclusion and should fit specific criteria (TABLE 1). Screening for other possible diagnoses is essential.

Table 1 Criteria for diagnosing postpolio syndrome

*History of old polio, preferably with recent electrodiagnostic findings consistent with remote anterior horn cell disease.

* A period of at least partial recovery from the initial illness and then a long stable period (10–20 years or more)

*New symptoms consistent with postpolio syndrome that are not attributable to any other medical condition; these may include weakness, myalgias, fatigue, swallowing problems, breathing problems, cold intolerance, and muscle atrophy.

Evaluating weakness

In postpolio syndrome, weakness pro-gresses gradually over months to years. This muscle fatigue is associated with overuse and worsens with increased activity. Rapid loss of strength over weeks to months should suggest another diagnosis (TABLE 2).

TABLE 2

Not available for online publication. See print version of the Cleveland Clinic Journal of Medicine

Evaluating fatigue

Generalized fatigue has many possible causes (TABLE 3) that should be ruled out with screening tests (TABLE 4).

TABLE 3

Not available for online publication. See print version of the Cleveland Clinic Journal of Medicine

TABLE 4

Not available for online publication. See print version of the Cleveland Clinic Journal of Medicine.

Since sleep disorders are common in polio survivors⁹,¹⁰ referral to a sleep clinic should be considered. In our practice, we often see patients who feel tired but say they have no trouble sleeping. However, many of them test positive for sleep disturbances, including obstructive sleep apnea (characterized by morning fatigue, snoring, and difficulty sleeping supine) and random limb movement disorder (characterized by morning muscle pain and overall fatigue). Depression, thyroid dysfunction, or both may be present as coexisting conditions and also contribute to fatigue.

Evaluating pain

Pain related to postpolio syndrome is due to muscle overuse or biomechanical problems, or both. Treatment of coexisting orthopedic problems is vital to alleviate pain and improve function (TABLE 5).

TABLE 5

Not available for online publication. See print version of the Cleveland Clinic Journal of Medicine.

Evaluating respiratory problems

Difficulty breathing can be life-threatening. Conditions other than postpolio syndrome should be considered (TABLE 6).

TABLE 6 Differential diagnosis for respiratory problems

* Cardiac disease

* Chronic obstructive pulmonary disease

* Asthma

* Anemia

* Deconditioning

Polio survivors with a weakened diaphragm breathe shallowly and exper-ience dyspnea on exertion. New respira-tory muscle weakness causes restrictive lung disease, which is associated with chronic alveolar hypoventilation. In obese patients, excess weight over the thoracic cage and abdominal cavity worsens the condition.
Pulmonary function tests can be used for diagnosis and to determine if supplemental oxygen is necessary. Arterial blood gas measurements and pulse oximetry can also help with the diagnosis. Referral to a pulmonologist is indicated if a patient requires a respirator. All polio survivors who undergo surgery need special pre-cautions beforehand to avoid potential problems with respiratory sequelae and ventilator weaning. These include pulmon-ary function testing and consideration of alternatives to general anesthesia when-ever possible. Even well-appearing polio survivors can have significant restrictive lung disease due to paralysis of respiratory muscles.

Evaluating swallowing difficulties

Swallowing problems can also be life-threatening. In view of the risk of choking, family members should be educated in the Heimlich maneuver. Patients presenting with difficulty swallowing may require specific testing, such as a modified barium swallow, or referral to a specialist for further evaluation and intervention.

TREATMENT RECOMMENDATIONS

Treatment of postpolio syndrome should focus on the most prominent symptoms and can include:

• Medications

• Supplemental oxygen

• Physical, occupational, and speech therapy

• An exercise program to preserve mobility and prevent deconditioning.

Medications
Drug therapy for postpolio syndrome has been generally disappointing. No medica-tions specifically address postpolio syndrome. That said, many medications may play an important role in alleviating symptoms. For weakness and fatigue, pyridostigmine (Mestinon), usually given as an oral dose of 60 mg three times a day, has had somewhat mixed results. One study of postpolio syndrome patients found that it improved upper extremity subjective strength and fatigue.¹¹ Another found no significant difference between patients taking pyridostigmine and placebo, except that very weak muscles (25% or less of baseline) were minimally stronger with pyridostigmine.¹²Methyl-phenidate hydrochloride (Ritalin) and bromocriptine (Parlodel) have been tried for postpolio patients with chronic debilitating fatigue, also with mixed results.⁷,¹³

Modafinil (Provigil) has been used to treat Fatigue¹⁴,¹⁵ and may be useful in polio survivors. The starting dose is usually 200 mg orally in the morning and may be increased to 400 mg each morning or given in divided doses. Side effects can be a problem with these medications. Pyridostigmine’s side effects are generally dose-related and can be recalled by the acronym SLUD (increased salivation, lacrimation, urination, and defecation). Respiratory secretions may also be increased with this medication. Modafinil’s side effects include headache, nausea, and nervousness, and modafinil may increase circulating levels of diazepam, phenytoin, and propranolol. Respiratory problems often improve with continuous positive airway pressure or bi-level positive airway pressure at night. Oxygen can exacerbate chronic alveolar hypo-ventilation and should be used with caution. A physical therapist or occupational therapist skilled in treating respiratory disorders may be helpful in teaching the patient breathing and postural techniques and help the patient conserve energy to decrease respiratory demands. Pain can be treated with traditional no steroidal anti-inflammatory drugs, cyclo-oxy-genase-² inhibitors (particularly in elderly patients or those with a history of gastrointestinal problems), and non-narcotic analgesics. Tramadol (Ultram) may be helpful in some patients but should be avoided in those with a history of seizures. Other medications typically used for chronic pain may also be tried, such as tricyclic anti-depressants and anticon-vulsants. Tricyclic antidepressants have cholinergic side effects; the most serious is the possibility of acute urinary retention in men, especially if underlying prostate problems are present. Injections with local anesthetics or corticosteroids or both may be effective for specific conditions that are often associated with postpolio syndrome, such as myofascial pain, trochanteric bursitis, carpal tunnel syndrome, lateral epicondylitis, or rotator cuff tendonitis.

Physical and occupational therapy

From a quality-of-life perspective, perhaps the most important thing a physician can do is to help patients preserve mobility and avoid falls and resultant injuries. Physical and occupational therapists can be extremely helpful in treating patients with musculoskeletal pain, weakness, decreased endurance, impaired balance, and difficulty walking. They can recommend appropriate adaptive equip-ment, such as shower grab bars, a raised toilet seat, sturdy and lightweight braces, assistive devices such as canes and crutches, and footwear modifications such as heel lifts and lateral wedges. Therapists can also advise patients on how to pace themselves, which is especially important for polio survivors. Home safety, work simplification, falls prevention, and proper exercise are also strategies that can enhance function.

Exercise
One of the most common questions polio survivors ask is, "How should I exercise?" This has been much debated. General guidelines for patients:

• Maintain an active exercise program to avoid deconditioning and cardiovascular sequelae

• Avoid overly aggressive exercise (fatiguing)

• Resist the impulse to exercise through pain.

Muscle fibers of polio survivors have very limited endurance because of the loss of aerobic enzyme activity and greater reliance on anaerobic metabolic capacity.¹⁶ Cross-training programs, such as alternating cycling with swimming and walking, are a good way to involve different muscle groups, but such programs should be consistent in terms of repetitions, resistance, and time. For most people, using daily activities as a primary way to exercise is too erratic and may lead to overuse, fatigue, and further weakness.

Is a wheelchair needed?

For patients who are having difficulty with walking or who may be at risk for falls, a motorized wheelchair or scooter can be useful, either full-time or part-time. Such vehicles can improve functional mobility, decrease risk of falls, and help conserve energy. Manual wheelchairs have the advantage over motorized wheelchairs of being lighter and easily folded for transport. However, manual wheelchairs tend to promote overuse syndromes in the arms and are generally recommended only when another person will push the patient.

Ancillary health care

Referral to other appropriate health care providers can markedly improve the quality of life for polio survivors. For example, speech and language pathologists can be extremely helpful in teaching patients compensatory mechanisms for swallowing. Referral to a mental health counselor, with pharmacologic intervention if needed, should be considered for patients who are depressed or have other psychological sequelae.

AVOIDING COMPLICATIONS

Osteoporosis. Patients with significant paralysis often have associated loss of bone density. Recent studies indicate that male polio survivors are at risk for osteopenia and osteoporosis, and may be at higher risk for fracture.⁸,¹⁶,¹⁷ We recommend that all polio survivors be screened for bone density loss and be appropriately treated.

Falls. Polio survivors are also at greater risk of tripping and falling due to poor balance and weak arms or legs, and are less likely to be able to protect themselves as they fall.^18–21 Since the complications of a fall can be serious, interventions for fall prevention are crucial. Both physical and occupational therapists typically address fall prevention. Upper extremity injuries. Because polio survivors tend to overuse their arms, they are also at risk for upper extremity injuries, including carpal tunnel syndrome and ulnar neuropathy.⁸,^22–24

REFERENCES
1. Cohen JI. Poliovirus. In: Fauci AS, Braunwald E, Isselbacher KJ, et al, editors. Harrison’s Principles of Internal Medicine. 14th ed. New York: McGraw-Hill, 1998:1120–1121.

2. Halstead LS, Silver JK. Nonparalytic polio and postpolio syndrome. Am J Phys Med Rehabil 2000; 79:13–18.

3. Gawne AC, Halstead LS. Post-polio syndrome: path physiology and clinical management. Crit Rev Phys Rehabil Med 1995; 7:147–188.

4. Dalakas MC. Pathogenetic mechanisms of post-polio syndrome: morphological, electrophysiological, virological, and immunological correlations. Ann NY Acad Sci 1995; 753:167–185.

5. Halstead LS. Managing Post-Polio: A Guide to Living Well with Post-polio Syndrome. Washington, DC: NRH Press, 1998.

6. Maselli RA, Wollmann R, Roos R. Function and ultra-structure of the neuromuscular junction in postpolio syndrome. Ann NY Acad Sci 1995; 753:129–137.

7. Dalakas MC. Pathogenetic mechanisms of post-polio syndrome: morphological, electrophyiological, virological, and immunological correlations. Ann NY Acad Sci 1995; 753:167–185.

8. Silver JK. Post-polio Syndrome: a Guide for Polio Survivors and their Families. New Haven: Yale University Press, 2001.

9. Bruno RL. Abnormal movements in sleep as post-polio sequelae. Am J Phys Med Rehabil 1998; 339–343.

10. Dean AC, Graham BA, Dalakas M, Sato S. Sleep apnea in patients with postpolio syndrome. Ann Neurol 1998; 43:661–664.

11. Seivert BP, Speier JL, Canine JK. Pyridostigmine effect on strength, endurance and fatigue in post-polio patients [abstract]. Arch Phys Med Rehabil 1994; 75:1049.

12. Trojan DA, Collet JP, Shapiro S, et al. A multi-center, randomized, double-blind trial of pyridostigmine in postpolio syndrome. Neurology 1999; 53:1225–1233.

13. Bruno RL, Zimmerman JR, Creange SJ, Lewis T, Molzen T, Frick NM. Bromocriptine in the Treatment of post-polio fatigue: a pilot study with implications for the path physiology of fatigue. Am J Phys Med Rehabil 1996; 75:340–347.

14. Kingshott RN, Vennelle M, Coleman EL, Engleman HM, Mackay TW, Douglas NJ. Randomized, double-blind, placebo-controlled crossover trial of modafinil in the treatment of residual excessive daytime sleepiness in the sleep apnea/hypopnea syndrome. Am J Respir Crit Care Med 2001; 163:918–923.

15. Mitler MM, Harsh J, Hiroshkowitz M, Guilleminault C. Long-term efficacy and safety of modafinil (PROVIGIL) for the treatment of excessive daytime sleepiness associated with narcolepsy. Sleep Med 2000; 1:231–243.

16. Silver JK, Aiello DD. Bone density and fracture risk in male polio survivors [abstract]. Arch Phys Med Rehabil 2001; 82:1329.

17. Silver JK, MacNeil JR, Aiello DD. Effect of Fosamax on bone density in a male polio survivor: a case report [abstract]. Arch Phys Med Rehabil 2001; 82:1329.

18. Silver JK, Aiello DD. Fall prevention strategies in a polio survivor: a case report [abstract]. Arch Phys Med Rehabil 2000; 81:1309.

19. Silver JK, Aiello DD. Polio survivors’ attitudes regarding falls [abstract]. Arch Phys Med Rehabil 2000; 81:1296.

20. Silver JK, Aiello DD. Risk of falls in polio survivors [abstract]. Arch Phys Med Rehabil 2000; 81:1272.

21. Silver JK, Aiello DD. Polio survivors: falls and subsequent injuries. Am J Phys Med Rehabil. In press 2002.

22. Veerendrakumar M, Taly AB, Nagaraja D. Ulnar nerve palsy due to axillary crutch. Neurol India 2001; 49:67–70.

23. Waring WP, Werner RA. Clinical management of carpal tunnel syndrome in patients with long term sequelae of poliomyelitis. J Hand Surg 1989; 14:865–869.

24. Slowman LS, Silver JK. Prevalence of median and ulnar neuropathy in post-polio patients [abstract]. Arch Phys Med Rehabil 2001; 82:1312–1313.

ADDRESS: Julie K. Silver, MD, Spaulding-Framingham Outpatient Center, 570 Worcester Road, Framingham, MA 01702; e-mail jksilver@bics.bwh.harvard.edu.

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The following has been sent to me several times – this time by a good friend, Millie Kimbar. Thanks, Millie.

The Amazing Cucumber

This information was in The New York Times several weeks ago as part of their "Spotlight on the Home" series that highlighted creative and fanciful ways to solve common problems.

1. Cucumbers contain most of the vitamins you need every day, just one cucumber contains Vitamin B1, Vitamin B2, Vitamin B3, Vitamin B5, Vitamin B6, Folic Acid, Vitamin C, Calcium, Iron, Magnesium, Phosphorus, Potassium and Zinc.

2. Feeling tired in the afternoon: put down the caffeinated soda and pick up a cucumber. Cucumbers are a good source of B Vitamins and Carbohydrates that can provide that quick pick-me-up that can last for hours.

3. Tired of your bathroom mirror fogging up after a shower? Try rubbing a cucumber slice along the mirror. It will eliminate the fog and provide a soothing, spa-like fragrance.

4. Are grubs and slugs ruining your planting beds? Place a few slices of cucumber in a small pie tin and your garden will be free of pests all season long. The chemicals in the cucumber react with the aluminum to give off a scent undetectable to humans but drive garden pests crazy and make them flee the area.

5. Looking for a fast and easy way to remove cellulite before going out or to the pool? Try rubbing a slice or two of cucumbers along your problem area for a few minutes. The phytochemicals in the cucumber cause the collagen in your skin to tighten, firming up the outer layer and reducing the visibility of cellulite. Works great on wrinkles too!!!

6. Want to avoid a hangover or terrible headache? Eat a few cucumber slices before going to bed and wake up refreshed and headache free. Cucumbers contain enough sugar, B vitamins and electrolytes to replenish essential nutrients the body lost, keeping everything in equilibrium, avoiding both a hangover and headache!!

7. Looking to fight off that afternoon or evening snacking binge? Cucumbers have been used for centuries and often used by European trappers, traders and explores for quick meals to thwart off starvation.

8. Have an important meeting or job interview and you realize that you don't have enough time to polish your shoes? Rub a freshly cut cucumber over the shoe. Its chemicals will provide a quick and durable shine that not only looks great but also repels water.

9. Out of WD 40 and need to fix a squeaky hinge? Take a cucumber slice and rub it along the problematic hinge and, voila, the squeak is gone!

10. Stressed out and don't have time for massage, facial or visit to the spa? Cut up an entire cucumber and place it in a boiling pot of water. The chemicals and nutrients from the cucumber will react with the boiling water and be released in the steam, creating a soothing and relaxing aroma that has been shown to reduce stress in new mothers and college students during final exams.

11. Just finish a business lunch and realize you don't have gum or mints? Take a slice of cucumber and press it to the roof of your mouth with your tongue for 30 seconds to eliminate bad breath. The phytochemcials will kill the bacteria in your mouth responsible for causing bad breath.

12. Looking for a 'green' way to clean your faucets, sinks or stainless steel? Take a slice of cucumber and rub it on the surface you want to clean. Not only will it remove years of tarnish and bring back the shine, but is won't leave streaks and won't harm you fingers or fingernails while you clean.

13. Using a pen and made a mistake? Take the outside of the cucumber and slowly use it to erase the pen writing. It also works great on crayons and markers that the kids have used to decorate the walls!!

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Have an absolutely GREAT SUMMER =

See you in September!!

Barbara

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FLORIDA EAST COAST POST-POLIO SUPPORT GROUP - Vol. 17 #6

12 Eclipse Trail / Ormond Beach, FL 32174 / 386 676-2435

E-Mail:- bgold@iag.net - Web Site:- home.iag.net/~bgold

MAY / JUNE 2011

WISHING ALL OUR FRIENDS

A FLOWER FILLED and LOVING MOTHER’S DAY

A SUNNY MEMORIAL WEEKEND

A FANTASTICALLY LOVING FATHER’S DAY

A SAFE AND HAPPY FOURTH OF JULY

- and -

Emotional stress

Not a return of polio

References

Support Group I belong to:- ____________________________________