Sometimes mistaken for, or MASKS, more serious conditions, such as Heart Disease. (As in my case!!)
The extent to which polio survivors will suffer from PPS depends on how seriously they
were affected by the first polio attack. Patients who had only minimal symptoms from the
original attack and subsequently develop PPS will most likely experience only mild PPS
symptoms. People originally hit hard by the polio virus may develop a more severe case of
PPS with a greater loss of muscle function, difficulty in swallowing, and more periods of
fatigue.
More than 300,000 polio survivors in the United States may be at risk for PPS. Doctors
are unable to establish a firm incidence rate, but they estimate that the condition
affects 25 percent of these survivors.
Restoration of nerve function may occur in some fibers a second time, but eventually
nerve terminals are destroyed and permanent weakness occurs. This hypothesis is consistent
with PPS's slow, stepwise, unpredictable course. Through years of studies, scientists at
the National Institute of Neurological Disorders and Stroke have shown that PPS is a very
slowly progressing condition marked by long periods of stability.
Objective assessment of muscle strength in PPS patients may not be easy. A change in
muscle strength is determined in specific muscle groups, or limbs, using various muscle
scales, such as the Medical Research Council (MRC) scale or scales that quantify muscle
force. Doctors use magnetic resonance imaging (MRI), neuroimaging, and
electrophysiological studies, muscle biopsies, or spinal fluid analysis as tools to
investigate the course of decline in muscle strength.
Once PPS is diagnosed, some patients worry that they have polio again, or even ALS. In
general, PPS is not life-threatening. The only exception is in patients who experience
severe respiratory impairment. Studies have proven that, compared to control populations,
PPS patients lack any elevation of antibodies against the polio virus, and since PPS
affects only certain muscle groups, it is not considered a recurrence of the original
polio. Further, there is no evidence that the polio virus can cause a persistent infection
in humans. Other studies have demonstrated that ALS, which progressively weakens muscles,
does not occur more frequently in PPS patients, and PPS is not a form of ALS.
In an effort to reduce fatigue in PPS patients, scientists conducted a study using low
doses of the drug pyridostigmine and found that it did reduce fatigue in some patients.
Research using this drug continues. In a controlled study conducted by NIH scientists, the
drug amantadine was not helpful in reducing fatigue. Scientists are also conducting more
research on the use of selegiline, a drug that in an anecdotal report was thought to
improve strength in PPS patients.
The future in PPS treatment may center around nerve growth factors. NINDS scientists
have plans to begin a multicenter, controlled trial to test whether insulin-like growth
factor (IGF-1) can enhance the ability of motor neurons to sprout new branches, maintain
existing branches, and rejuvenate synapses (the space between nerve cells where signals
pass from one cell to another). A preliminary study is in progress. Since PPS results from
the degeneration of nerve sprouts, IGF-1 may target the heart of the problem, and may help
to regenerate new sprouts. Other growth factors now under investigation have similar
properties.
There is much misinformation on this subject and many doctors do not know what effect
exercise has on individuals with PPS. A commonsense approach, in which patients use
individual tolerance as their limit, is currently recommended. Tolerance is the level at
which one starts having discomfort or fatigue. Researchers at the NIH are addressing the
exercise issue, not only to determine if exercise is helpful, harmful, or ineffective, but
also to define the most effective type -- isotonic, isometric, isokinetic, or repetitive.
Other investigators are searching for the polio virus, or mutated versions of it,
fragments of which have been amplified from the spinal fluid. The significance of this
finding is not known however, and more research is being done.
The organizations listed below can provide printed information and assistance to PPS
patients:
Swallowing difficulties for
Post Polio Patients
Post-Polio Patients Have Swallowing Abnormalities that Increase the Risk of Choking
Many post-polio patients have swallowing abnormalities that increase the risk
of choking but are unaware of their condition, according to a study directed by a
scientist at the National Institute of Neurological Disorders and Stroke (NINDS)
and published in the April 25 issue of the New England Journal of Medicine.* .
Of the 500,000 Americans who survived acute polio, more than 125,000 now
have post-polio syndrome, in which progressive muscle weakness develops 25
to 30 years after the initial disease.
Previous reports have demonstrated that post-polio syndrome weakens the muscles
of the arms and legs -- causing patients difficulty when walking, lifting, or reaching. The
recent study shows that this progressive weakness also affects the bulbar muscles of the
tongue, mouth and throat and triggers swallowing problems -- called
dysphagia -- in most patients.
"These findings should prompt physicians to check their post-polio patients
closely for abnormal swallowing," said Marinos C. Dalakas, M.D., the NINDS
scientist who directed the study. "Unlike new weakness in the arm or legs, which causes
obvious problems with movement, deterioration in swallowing is easily overlooked--
even with standard neurological exams," Dr. Dalakas stressed. "If dysphagia is not
specifically tested, problems may remain hidden until the patient suddenly
becomes aware of choking."
During the study, scientists examined swallowing among 32 patients randomly chosen
from a larger group of post-polio patients. Using specialized imaging techniques,
including videofluoroscopy and ultrasonography, they detected dysphagia in 31 patients --
but only 14 were aware of this problem.
Typical signs of dysphagia included excessive tongue movements, a delay in the
swallowing reflex and constriction of throat muscles, and uncontrolled flow of
food from the mouth into the throat. These problems were often more severe
on one side of the mouth and throat.
Dr. Dalakas and his colleague Barbara Sonies, Ph.D., of the National Institutes
of Health also detected dysphagia using an index that assesses oral motor
function based on 10 tests of muscle strength and motion, such as strength of tongue
and lips, voice quality, and swallowing ability. "As the index score rises, the patient's
risk of choking also goes up," Dr. Dalakas said. "Using this index, physicians can predict
which patients are likely to choke."
Dr. Dalakas suggested that patients with post-polio syndrome visit their physician for
a check of dysphagia and, in some cases, change eating routines to reduce choking risk.
For example, patients with significant dysphagia should chew food on the less
affected side, consume softer foods, use smaller bites when eating, and eat more
slowly. He also recommended that family members or companions of such patients
learn the Heimlich maneuver.
The exact cause of post-polio syndrome is unknown. During the first, acute polio
infection, the virus destroys nerve cells that control limb and bulbar muscles. This
causes symptoms that range from weakness to paralysis, depending on the number
of nerve cells destroyed and how well the remaining, healthy neurons can compensate.
In post-polio syndrome, however, the compensating neurons become overworked
and are no longer able to control muscles as effectively, Dr. Dalakas said. Post-polio
syndrome may also be aggravated by the aging process, in which all individuals
lose some neurons. In most individuals, half of the nerve cells controlling a muscle
must die for weakness to result. Since the number of neurons is already abnormally
low in post-polio patients, small additional losses can easily trigger muscle weakness.
"Studies of post-polio syndrome are rapidly advancing our knowledge of how
motor neurons survive and endure and what causes them to dysfunction," said
Murray Goldstein, D.O., M.P.H., NINDS director. "Thus, this research could
have implications for other diseases that affect motor neurons -- such as amyotrophic
lateral sclerosis, or Lou Gehrig's disease, and the neuropathies. It may also help
scientists to understand how aging affects the nervous system. "
The National Institute of Neurological Disorders and Stroke, one of the 13 National
Institutes of Health in Bethesda, MD, is the primary supporter of brain and nervous
system research in the United States.
* "Dysphagia in Patients with the Post-Polio Syndrome." Barbara C. Sonies, Ph.D.,
and Marions C. Dalakas, M.D. New England Journal of Medicine; April 25, 1991; pp. 82-91.
Last Updated: July 09, 1999.
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
We were sent this May '04 article - It should be of interest:
Company Pulls Plug On Iron Lung - Polio Survivors Fear For Their Lives
POSTED: 7:41 am PDT May 12, 2004
UPDATED: 11:23 am PDT May 12, 2004
SAN DIEGO -- Delores Thompson of San Diego has lived with her iron lung for 54 years,
since she was stricken with polio at 9 years old.
Sherry Hurst of Lubbock, Texas, has also spent close to half a century in her iron lung.
She sleeps in it every night.
The same goes for Mona Randolph in Kansas City, Mo., and Susan Armerecht of
Cleveland, both polio survivors.
Marilyn Rogers of Minneapolis lives in her iron lung 24 hours a day, seven days a week.
It's the only world she has known since she fell to polio when she was 9 years old.
Rogers is now 65.
These are a handful of the estimated 40 polio survivors in the United States who still
use iron lungs to stay alive. Each of these polio survivors received the same letter
from the company that services their iron lungs.
The letter from Respironics said the company "will no longer be able to procure
service parts to support or repair" iron lungs. The letter goes on to recommend
these patients contact their physician to determine what equipment would be
"appropriate replacement."
The new policy went into effect March 1, 2004, and has set off a quiet panic among
these physically fragile men and women.
Many said other respirators don't work as well as the tried and true iron lung.
Rogers said she tried others, but they were unsuccessful.
Thompson said she's too frightened to try anything else.
"I could gradually suffocate to death and I'm not willing to risk that," said Thompson.
"This is a death sentence for these patients," said Dr. Richard Bruno of the
International Post Polio Task Force.
Bruno said Respironics' decision to pull the plug on iron lung patients is
symptomatic of a bigger problem.
"Nobody is concerned about polio survivors," Bruno said.
Fifty years after researchers rolled out the Salk Vaccine vaccine for polio, more
than 1.6 million survivors are still struggling in silence. Many, who were less
seriously impacted during the epidemic of the 1950s and 1960s, are now
suffering more painful and debilitating affects of post-polio syndrome,
(also) called sequelae.
Bruno has concerns for these patients, as well.
To contact Bruno, visit www.postpolioinfo.com.
Polio survivor and advocate Mary Clare Schlesinger said the iron lung is truly
different from other respirators or ventilators. She said it works on a negative
pressure system that literally takes over the patient's breathing.
Other ventilators work on a positive pressure system that makes it more
difficult for polio patients to fill their lungs and keep enough oxygen streaming
through their bodies.
Schlesinger said, "Respironics has made this decision (to discontinue service)
for their convenience without regard for the possibly life-threatening impact of their actions."
(ED NOTE: REPORTEDLY THE *MAIN* REASON IS BASED ON THE LACK OF
AVAILABILITY OF PARTS, SCHEMATICS, AND SERVICE COMPONENTS FOR
THESE OLD MACHINES -- See below)
She said Respironics has a moral, if not legal, obligation to continue to service
iron lungs.
"To fail to do SO is outrageous and negligent," Schlesinger said.
Schlesinger runs a post-polio support group and can be reached at
sandiego_polio.tripod.com or via e-mail at postpolio@cox.net.
Patients Come To Troubleshooter For Help
Current iron-lung patients said they came to the Troubleshooter after their pleas
for a "reprieve" fell on deaf ears at Respironics. (sic)
The Troubleshooter then took their case straight to Respironics' vice president,
Tim Murphy. Murphy said the decision has been made and there is no
turning back.
He also claimed there was no division or sub-contractor within the umbrella of
his $750 million, publicly traded corporation that is capable of making parts or
providing ongoing service for the iron lung.
"It is no longer commercially viable for Respironics to continue servicing iron
lungs," said Murphy.
He suggested a few options to patients:
a.. Transition to an alternative device, perhaps one made by Respironics.
b.. Continue using the iron lung with the understanding that if the device
fails Respironics may not be able to repair it.
c.. Accept donation of the device and pursue other support and repair options.
Since the Troubleshooter began this inquiry, Respironics has sent a
further response:
"In an effort to continue to support these patients as long as possible, Respironics
will put all returned iron lungs through a comprehensive Quality Assurance check,
and providing the device meets standards, it will be held in a rental pool in the
event one is needed in an emergency situation by those opting to remain on
the iron lung.
Returned devices which do not pass the overall QA check will be held as parts
resources. The reality, however, is that this may only provide a limited and
unpredictable source of replacement product and repair parts and the best long
term resolution for patients may be to transition to an alternative therapy."
The Respironics letter to the Troubleshooter goes on to say the company,
"has great compassion for our patients facing this decision and the concerns
and fears of transitioning to an alternative therapy and away from something
that's been a part of their life for decades. We will continue to work with these
patients, their physicians and their caregivers to help them transition to an
alternative therapy."
Delores Thompson said if Respironics genuinely cares about her fears, the
company should make more of an effort to maintain her iron lung.
"We won't be around very long. I don't think it's asking for much," she said.
"But I do think it's about money when push comes to shove."
For more on Respironics plan, or to comment, visit www.respironics.com.
.
BULLETINS FROM WORLD NEWS!
about Lipitor effects.
We've managed to undertake another cause,
or two.. :-)
