Click pages 2 and 3 for sidebars: "Fighting the Odds" and "Last of the Iron Lung"
Gary Presley always had a kinetic spirit. He could go to a concert, throw back a couple beers, and dash to work bright-eyed the next day. Around 1985, life seemed to catch up with him.
Presley, a polio survivor, was in his 40s and worked as an office manager at a Missouri insurance firm. He found himself dozing at his desk and constantly felt as if he just finished a marathon in his wheelchair. His family doctor said it might be heart disease and prescribed furosemide, a diuretic, for his edema.
The crisis came a couple years later when he went into respiratory arrest.
Presley, now 65, remembers waking up in an intensive care unit, intubated, with his pulmonologist peering over him. "We're going to do a tracheostomy," the pulmonologist said.
Alarmed, Presley motioned for a pen and paper. "Please consult Dr. Oscar Schwartz in St. Louis," he scrawled. He had come across articles by Oscar A. Schwartz, MD, FCCP, while scouring literature to understand his fatigue and breathing problems. Dr. Schwartz recommended bilevel positive airway pressure to reduce Presley's fatigue, and he began to feel better than he had in 10 years, Presley said.
Disordered breathing is among the most misdiagnosed and misunderstood symptoms polio survivors face later in life. Too often their breathlessness and inefficient coughs are misdiagnosed as asthma or chronic obstructive pulmonary disease. For many aging polio survivors, it largely has been up to them to initiate their care and educate their doctors on the medical literature.
"You have a thousand patients with high blood pressure, and you learn something about high blood pressure," said Brenda Butka, MD, director of pulmonary services at the Vanderbilt Stallworth Rehabilitation Hospital, Nashville, Tenn. "You might have one patient with post-polio, and he might look quite different than the next patient with post-polio."
But there is reason to be prepared for when these patients visit your office. There are 1.6 million polio survivors in North America and 10 million to 20 million worldwide. And the Centers for Disease Control reported polio infections rose 55 percent - 2,000 cases worldwide - from the previous year.
The summer plague
The poliovirus enters the gastrointestinal tract through the mouth, typically after ingesting contaminated drinking water or food. The virus binds to a receptor in a host cell. Most cases are apparently asymptomatic.
Paralytic poliomyelitis occurs in less than 1 percent of patients. In these cases, the virus enters the central nervous system and replicates in the motor neurons within the spinal cord, brain stem, and motor cortex.
Presley remembers the terror his mother felt every summer. Before the polio vaccine was developed in 1955, the summer plague left 13,000 to 20,000 of U.S. children each year in wheelchairs, braces, and iron lungs.
The cruel irony is Presley's mother took him and his brother to get vaccinated in 1956. They received all three vaccinations over three years.
Not long after the final round, Presley's mother would realize her worst fear.
"You don't act like you feel very good," said Presley's father one evening. They were working in the barn on their Missouri farm.
"My head is killing me," said 17-year-old Presley.
"Go on into the house and lay down for awhile," said his father. Presley walked out of the barn, across the field, and into his house. He never walked again.
His father woke him the next morning to milk the cows. Presley couldn't move.
One-third to one-half of polio survivors experience new or increased weakening and pain in the muscles later in life. This typically occurs 10 to 40 years after recovering from original polio. This weakening can affect the diaphragm and breathing muscles, causing such disorders as obstructive sleep apnea, central sleep apnea, pneumonia, pulmonary restriction, shallow breathing, pneumonia, and diffuse muscle twitching during sleep.
The breathing problems are part of a broader constellation of symptoms called post-polio syndrome. The causes of post-polio syndrome are unknown.
One theory suggests the neurons powering the muscles are overworked. Polio survivors, on average, lost half the neurons in their brain stem and spinal cord, according to Richard Bruno, PhD, HD, director of the Post-Polio Institute, Englewood, N.J. The motor neurons perform many times the amount of work they were designed to do, like an electrical outlet powering several toasters. Eventually the outlet will blow a fuse.
Another hypothesis states that the original polio damaged parts of survivors' thalamus and hypothalamus, leading to a metabolic disorder later in life. And still another theory holds that the effects of aging are accelerated in polio survivors because they started off with fewer neurons.
Whether the deterioration is caused by one or a combination of all these theories, many survivors report suffering overwhelming fatigue. Poor sleep quality is a main culprit, said Dr. Bruno, a psychophysiologist who has worked with polio survivors for 25 years. Half of his patients do not realize they are not sleeping at night.
One patient reported feeling tired every day, despite sleeping for 12 hours.
A sleep study revealed he woke up 1,200 times a night - almost every 30 seconds - because of sleep apnea, shallow breathing, and twitching.
He warns that split-night sleep studies are not appropriate for polio survivors. They may miss sleep problems such as hypopneas and muscle twitching that occur after the REM cycle. Dr. Bruno also advises that electrodes should be put on the legs and the arms. Twitching will not show up in the legs if they are paralyzed. But it may be apparent in other functioning extremities.
Changing your perspective
Misdiagnoses can be deadly for polio survivors. Giving them supplemental oxygen rather than mechanical ventilation, for instance, can exacerbate hypoventilation by shutting off the brain signals that detect carbon doxide, putting survivors at greater risk for respiratory arrest. This phenomenon represents compromise of the post-polio patients' hypoxic respiratory drive to breathe.
If polio survivors are incorrectly diagnosed with asthma or COPD, the typical oral corticosteroid therapy can inhibit functioningof motor neurons. The anti-inflammatory agents could make survivors even weaker, Dr. Bruno said.
One reason pulmonologists may misdiagnose breathing problems in polio survivors, is that they are accustomed to treating lung and airway illnesses. "They don't think in terms of muscle weakness," said John Bach, MD, vice chairman of the physical medicine and rehabilitation department at the University of Medicine and Dentistry of New Jersey, Newark.
Pulmonary function testing is designed to evaluate lung and airways disease, Dr. Bach said, and may be inappropriate for patients with muscle weakness.
This subset of patients needs monitoring of end-tidal CO2 and oximetry, as well as spirometry for vital capacity (sitting and supine), maximum insufflation capacity, and cough peak flow measurements.
Polio survivors should be given portable volume-pressure ventilators to use with nasal interfaces for ventilatory assistance rather than CPAP or bilevel positive airway pressure, Dr. Bach said.
Polio survivors do not benefit from the expiratory positive pressure, he said, and it detracts from the positive inspiratory pressure in assisting the inspiratory muscles. "Air stacking" to get deep volumes of air to cough with also is impossible with CPAP and bilevel positive airway pressure devices rather than with volume-type devices, Dr. Bach said.
For daytime ventilation, Dr. Bach recommends receiving air from a 15 mm angled mouthpiece connected to a portable volume-pressure ventilator rather than from an invasive tracheostomy tube. None of the 300 polio survivors Dr. Bach has treated has required a tracheostomy tube, he said.
Some patients can use in-exsufflation belts. These consist of an air bag or rubber bladder inside a corset worn around the abdomen, just under the diaphragm. As the belt inflates and deflates, it pushes against the belly to move the diaphragm and ventilate the lungs.
Cough assist devices may not be effective in the presence of chest deformities and should not be used after chest surgery until healing has advanced, Dr. Butka said.
Polio survivors, she said, should be tested with spirometry to measure vital capacity in addition to noninvasive blood gas tests including oxygen saturations. Peak cough flows below 160 L/m greatly increase the risk for respiratory failure.
Resting the muscles
When Nancy Carter, a polio survivor, started growing weaker in her upper body during her 30s, she asked her childhood doctor from the Roosevelt Warm Springs Institute for Rehabilitation in Georgia for exercises to strengthen her muscles.
"It's not that way anymore," the doctor told her. "Those exercises are probably not going to help you."
As part of the treatment regimen, polio survivors often are advised not to overexert themselves with too much exercise or an on-the-go lifestyle. Dr. Bruno encourages them to stretch their muscles and take two 15-minute breaks a day. During these breaks, they should relax or nap. They can give their diaphragms a rest by turning on their nighttime ventilator, he said. But persuading polio survivors to slow down rather than to push through their pain can be a difficult task.
"This is the American way: Use it or lose it. No pain, no gain. Feel the burn," Dr. Bruno said. "And that's what polio survivors were taught. Now, if they use it, they're going to lose it. If they feel the burn, they¹re burning their neurons."
One criticism of this theory is that not working the muscles can lead to disuse atrophy. Critics also ask, what exactly does "exercise" mean?
"Some people think, 'I need to run three miles,'" said Joan Headley, president of the Post-Polio Health International. "But 'exercise' can be as simple as stretching your arms and legs while still in bed each morning. Many ventilator users stretch their chest walls by breathing in an ambu bag each day."
Dr. Butka tells her patients to be as active as they can be. "But if what you do is so exhausting you're not recovering by one night's rest, then you did too much," she said.
Telling survivors they need to use breathing assist devices can be devastating news. They may feel they are taking a step back from their lifelong attempts to strengthen their polio-weakened muscles. Fears of paralysis, that a spouse will leave them, or that they will get fired from their job, persist.
"Using aids represents a loss of independence," Dr. Butka said. "But I tell them they can become more independent because they can get more places and do more things. These are difficult, difficult stages. And it's very hard to accept."
Psychology of survivors
Carter, who now is 71, reluctantly agreed to use a ventilator about a decade ago. "I wanted to think I was like everyone else," she said. "I wanted to think I was normal and that I would never have a problem that meant I couldn¹t breathe on my own."
Carter never used an iron lung, although she was hospitalized for a year after developing polio at age 11.
The Post Polio Institute has surveyed 3,500 survivors about their lifestyles and attitudes. The answers to the surveys suggest that survivors tend to be more overachieving, time-conscious, and sensitive to criticism than the general population. On average they stay married longer and have more years of education. They are doctors, lawyers, teachers, and chief executive officers.
"They have overachieved to break even," Dr. Bruno said. But they also report 34 percent more physical abuse and 94 percent more emotional abuse than people who do not suffer disabilities, he said. He has found the more resistant they are to using aids, the worse the abuse and alienation they suffered throughout their lives.
When patients come to his five- to six- week polio clinic at the Post-Polio Institute, they receive weekly psychotherapy in addition to physical therapy, occupational therapy, brace and wheelchair clinics, nutrition counseling from a dietician, and a full-night sleep study.
For Presley, coping with polio has been an emotional journey through resentment, fantasy, anger, depression, and finally acceptance. He thinks of himself as two people, the person who walked out of the barn and the one who was carried out of the iron lung.
He retired from the insurance business and now is working on a memoir, "Seven Wheelhairs: A Life Beyond Polio," (University of Iowa Press) due out later this year. He also teaches writing workshops. The decision to go on a ventilator was not difficult, he said. For him, the ventilator is just another tool to enhance his life - like eyeglasses or his wheelchair.
"At some point I decided I wouldn't be a person with a disability," Presley said. "I would just be what that disability had turned me into."
Lauren Meade is assistant editor of ADVANCE. She can be reached at email@example.com.