Post-polio syndrome affects some who overcame polio in childhood
Emanuel Poznanski dealt with polio for most of his childhood. He overcame it, but he now suffers from PPS, a disease that can take over his life.
By LEIGH ZALESKI Daily Record/Sunday News
Emanuel Poznanski stands briefly in his room at the Cross Keys Motor Inn in New Oxford as he transfers himself from his scooter, right, to his power wheelchair. Poznanski has post-polio syndrome, a disease that affects 25 to 40 percent of polio survivors years after initial onset of polio. He lives in Las Vegas, but he graduated from York Suburban High School, and comes to York County every summer to visit friends for two months. (DAILY RECORD/SUNDAY NEWS - CHRIS DUNN)
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Emanuel Poznanski sits in his scooter by the therapy pool... (DAILY RECORD/SUNDAY NEWS - CHRIS DUNN)
Emanuel Poznanski had 21 surgeries in the first 16 years of his life.
He received muscle transplants and joint fusions, each procedure followed by months of recovery.
During one of his last surgeries, a doctor broke his left leg -- which was an inch and a half longer than his right -- in an effort to decrease the difference.
Poznanski, 62, was born in Israel, where he contracted paralytic polio when he was 4 months old. He said his mother rushed him on a plane to the U.S. to save his life. She took him to the Mayo Clinic in Minnesota and then to Children's Hospital Boston.
In his earliest memories, hospitals were his home. There, he lived and received most of his early education.
"I never realized I was handicapped," Poznanski said.
He's thankful for that.
About 25 percent of his muscle had been destroyed by the disease. By the time he was a teenager, he had a slight limp, but said he could walk for miles. The confinement he felt from the disease and hospitals had ended. He was ready to start living.
In 1964, when Poznanski was 14, his family moved to York County, where he attended York Suburban High School. Poznanski, who now lives in Las Vegas, visits friends in York County for two months every summer. He stays at the Cross Keys Motor Inn in New Oxford.
"It was the most formative time in my life," Poznanski said of living here. "It was the time I was the happiest."
He thought he had overcome the debilitating disease and one-time epidemic. Decades later, he would again feel the effects.
Poznanski has post-polio syndrome, a condition that affects 25 percent to 45 percent of polio survivors years after initial onset of the disease, according to Post-Polio Health International. From 1937 to 1950, 226,306 cases of paralytic and nonparalytic polio were reported.
Symptoms of PPS -- a chronic, degenerative disease -- include pain, fatigue, and breathing and swallowing problems, and can worsen over time.
After staying in the pool for about an hour, Emanuel Poznanski inches his way to the aquatic lift that will carry him out of the therapy pool at the Cross Keys Village retirement community. Poznanski said it takes him two to four times to do what he used to because of post-polio syndrome. (DAILY RECORD/SUNDAY NEWS - CHRIS DUNN)
Some PPS patients say they have difficulty getting the right care because their symptoms are similar to aging -- and because modern doctors don't know a lot about the disease that is no longer prevalent thanks to a 1955 vaccine.
"We're dying off," Poznanski said. "Pretty soon, there won't be any of us left."
More than a year ago, Deborah Stambaugh started a post-polio support group that meets monthly in Hanover. She said about 20 people participate to share their post-polio-related problems and resources.
Stambaugh met Poznanski this summer and said he has a wealth of knowledge.
"He's really gifted in all this," she said.
Poznanski has watched his body decline for the last 14 years, as he searches for answers beyond his reach.
In 1998, he lived in Brazil, where he ran English schools. He felt fatigued and started falling every few months. By 2003, he felt too tired to work and moved back to the U.S. with his wife. Though he knew about PPS, he didn't seek a diagnosis.
"I was in a funk," Poznanski said. "I was aloof and probably didn't want to admit it."
In 2006, he stopped walking and needed a scooter to get around. A year later, he started researching the condition, spending three to four hours a day online trying to learn more.
He was diagnosed with sleep apnea and started gaining weight because his mobility was limited. At his peak, his 5-foot-7-inch-tall frame weighed 220 pounds. He has Type 2 diabetes, and high blood pressure and cholesterol. To maintain his weight of 190 pounds, he said, he can eat only 1,200 calories a day because his body doesn't easily burn energy.
Prescription medicines fill two plastic bags in Emanuel Poznanski's room at the Cross Keys Motor Inn in New Oxford, where he stayed for a month while visiting friends in York County. Poznanski said he takes about 15 pills a day and has 10 more he takes when needed. ( DAILY RECORD/SUNDAY NEWS - CHRIS DUNN)
His neck, joints and left side ache, and his breathing is getting worse. He uses his right arm almost exclusively and has learned to swing it into certain positions so he can use it because he has no muscle in his shoulders.
Poznanski rested his left hand on his protruding stomach as he gestured with his right at a diner in New Oxford. He leaned his forearm on the table to reach his sandwich before leaning in to take a bite.
To perform everyday tasks, it takes him two to four times longer than it used to -- 15 minutes to move his body out of bed, two and a half hours to shower and 45 minutes to get dressed.
"It bores me," he said.
He brings literature about PPS to his doctors and travels to clinics and wellness camps throughout the country.
"I have to watch out for what's ahead of the curve," he said.
Dr. Andrew Schutzbank helps Poznanski manage his condition. Schutzbank, assistant medical director of Iora Health in Cambridge, Mass., said he never came across a PPS patient before Poznanski. Poznanski met Schutzbank in Las Vegas, where Schutzbank sometimes works. They communicate mostly by phone and email.
"Everything I know about PPS, I learned from Manny," Schutzbank said.
He said there's not many good resources for the condition, yet the needs of PPS patients can be great.
Schutzbank helps Poznanski take care of his general health and connects him to specialists who might be able to improve his PPS. It's his job to keep Poznanski in the fight.
He said his patient's prognosis is "some variety of poor" and that he's going to get weaker over time.
"It's a race against the clock," Schutzbank said. "Manny's key is his tenacity. He's unwilling to accept that there's no help."
Poznanski can feel his body slowing down. He said he's a 62-year-old with a 90-year-old body, yet his brain is still sharp.
This summer, he ended his trip to York County more than a month early because traveling had become too much.
"It's coming to an end," Poznanski said. "It's becoming physically difficult to keep going."
He said because people who had polio conquered so much, many feel they can overcome anything. But, he said, PPS is not something to go at alone -- patients need to seek adequate care.
"The wall, this time," Poznanski said. "It's insurmountable." 717-771-2101; @leighzaleski
People who had polio can develop post-polio syndrome decades after they first developed paralysis or weakness. Polio patients might develop more weakness, atrophy or paralysis.
Deborah Stambaugh of Spring Grove runs a post-polio support group that meets once a month. She said the group provides an outlet for former polio patients to talk about problems they might experience today as a result of the disease.
The group will meet 2 p.m. Sept. 23 at Hanover Church of the Brethren, 601 Wilson Ave., Hanover. For details, call 717-259-9594.
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A 41-year-old man developed an acute illness at the age of 9 months during which, following a viral illness with headache, he developed severe weakness and wasting of the limbs of the left side. After several months he began to recover, such that he was able to walk at the age of 2 years and later was able to run, although he was never very good at sports. He had stable function until the age of 18 when he began to notice greater than usual difficulty lifting heavy objects. By the age of 25 he was noticing progressive difficulty walking due to weakness of both legs, and he noticed that the right calf had become larger. The symptoms became more noticeable over the course of the next 10 years and ultimately both upper as well as both lower limbs had become noticeably weaker.
On examination there was wasting of the muscles of upper and lower limbs on the left, and massively hypertrophied gastrocnemius, soleus and tensor fascia late on the right. The calf circumference on the right exceeded that on the left by 10 cm (figure1). The right shoulder girdle, triceps, thenar eminence and small muscles of the hand were wasted and there was winging of both scapulae. The right quadriceps was also wasted. The wasted muscles were also weak but the hypertrophied right ankle plantar flexors had normal power. The tendon reflexes were absent in the lower limbs and present in the upper limbs, although the right triceps was reduced. The remainder of the examination was normal.
The patient's legs, showing massive enlargement of the right calf and wasting on the left
What is that nature of the acute illness in infancy?
What is the nature of the subsequent deterioration?
What investigations should be performed?
What is the differential diagnosis of the cause of the progressive calf hypertrophy?
An acute paralytic illness which follows symptoms of a viral infection with or without signs of meningitis is typical of poliomyelitis. Usually caused by one of the three polio viruses, it may also occur following vaccination and following infections with other enteroviruses.1 Other disorders which would cause a similar syndrome but with upper motor neurone signs would include acute vascular lesions, meningoencephalitis and acute disseminated encephalomyelitis.
A progressive functional deterioration many years after paralytic poliomyelitis is well known, although its pathogenesis is not fully understood.2 It is a diagnosis of exclusion; a careful search for alternative causes, for example, orthopaedic deformities such as osteoarthritis or worsening scoliosis, superimposed neurological disorders such as entrapment neuropathies or coincidental muscle disease or neuropathy, and general medical causes such as respiratory complications and endocrinopathies.3
Investigations revealed normal blood count and erythrocyte sedimentation rate and normal biochemistry apart from a raised creatine kinase at 330 IU/l (normal range 60–120 IU/l), which is commonly seen in cases of ongoing denervation. Electromyography showed evidence of denervation in the right APB and FDI with polyphasic motor units and complex repetitive discharges, no spontaneous activity in the left calf and large polyphasic units in the right calf consistent with chronic partial denervation. Motor and sensory conduction velocities were normal. A lumbar myelogram was normal. Magnetic resonance imaging (MRI) scan of the calves is shown in figure2.
Axial T1 weighted MRI scan (TR 588 ms, TE 15 ms) of the calves, showing gross muscle atrophy and replacement by adipose tissue on the left, and hypertrophy of the muscles on the right, with only minor adipose tissue deposition
The differential diagnosis of the progressive calf hypertrophy is given in the box.
Causes of calf muscle hypertrophy
Chronic partial denervation
hereditary motor and sensory neuropathy
spinal muscular atrophy
following paralytic poliomyelitis
Neuromyotonia and myokymia
continuous muscle fibre activity due to: chronic inflammatory demyelinating polyradiculopathy, Guillain Barre syndrome, myasthenia gravis, thymoma, thyrotoxicosis, thyroiditis