Oakland Kaiser Conference
Post Polio Syndrome
Aging with a Disability
Notes by Gladys Swensrud
The conference on
Dr. Sandel,
Medical Director of the
Next, Phyllis Hartke, a polio survivor and President of the SFBAPS group welcomed everyone to the conference, and she recognized those who had helped to make this event happen. She particularly thanked Dr. Sandel, Kaiser Permanente and the planning team for making this conference come together. In her opening remarks, Phyllis noted that most health care providers know little about Post Polio Syndrome, but she hoped that those in attendance on this day would know much more by the end of the conference day. Among the facts most important to her, she reminded everyone that a “team approach” to patient care is the best way to handle old Polio. Polio patients have certain requirements that they need for professionals who will be servicing them to know, and hopefully physicians will learn today the most needed requests of their Polio patients. She also urged doctors in attendance to return to work and share the information they would be learning with their peers.
Each guest speaker that spoke to the conference had a partner/discussant as a means to facilitate discussions and share information. Each group also gave enough time for a question and answer session following their presentation.
Speaker - Dr. Fernando Torres-Gil holds a dual role as a doctor and a Polio survivor. Dr. Torres-Gil explained that although he has a disability, he also wants to learn to grow old gracefully with it. He would like to bring us (himself included) to the next stages of aging with a disability; he hopes to inform the larger society in general about long term care, health care reform, and how health care will approach an aging society with many disabilities, as well as how the needs of the masses can be met within the health profession. His extensive credentials began as a grad student in his passions of gerontology/geriatrics. Much of his career revolved around the health policies of those groups. He explained that he is now fifty-five, and he began to feel that he was aging prematurely. He realized that his level of fatigue and discomfort could be related to his disability and not just a regular part of the aging process. He recalled his Polio history of being quarantined and sent from his home to Shiners' hospital for treatment. Like most polio survivors of our era, he put that part of his life behind him and went on to be a super achiever. Now at 55, he realizes that his specific aging problems are related to Post Polio, and he is dedicating his life to being a “poster adult” to aging with a disability. Dr. Torres-Gil made the point that as our generation is aging (we are all getting older), we are reaching a crisis as life expectancy is growing. We see older people now growing into their 60s, 70s and 80s as a normal situation. The world as a whole is an aging society. That comes equipped with it’s own set of problems involving Social Security and Medicare.
As far as disability is concerned, that world is a much more difficult one to bring together for health systems. Each group of handicapped people, Post Polio patients, people with MS or ALS etc, has its own group of advocates. Their needs are diverse, but at the same time, their needs are similar. One of the challenges of the medical profession is that the aging world and the disability world don’t communicate with one another. Together they will be a stronger voice to be listened to. Dr. Torres-Gil feels that the bridge between those two systems needs to bring them together.
Long Term Care (LTC) will be one bridging factor. LTC is about giving options and choices to having that patient, whether elderly, disabled or both, live at home and have assistance available for them to live at home and within their communities. It will need to become part of the lexicon of the medical profession.
Dr. Torres-Gil has learned that on the policy side, more changes are afoot, but funding is a problem to help support this cause. Prices in health care are escalating, and getting funding for PPS, which is a pre-existing condition, is not likely. The big debates are on prescription drugs and the things connected to programs like Social Security and Medicare. Old polio is almost a problem of the past generation, so finding monetary sources to help our cause is difficult.
From his personal experience, he is astounded by his own healthcare plan's ignorance. His personal physicians mean well, but they aren't incorporating needed information into their protocols. He has learned that he has to be his own advocate. He gathers information and brings it together in a presentable format to help himself. PPS is a multi-disciplinary and inter-disciplinary integrated health and service problem. These terms mean that they also bring in the rehab and non-health issues like housing, transportation, income supports and all those supportive issues that will help us to age gracefully.
As an aside, Dr. Torres-Gil announced that he is also interested in alternative medicines like water programs, yoga, etc. He believes there is a role involving those types of programs to bring together a truly multifaceted program. Physicians are now at the cutting edge of the aging with PPS and other disability issues, and how they advocate for these people will have important benefits well beyond the realm of Post Polio Syndrome alone.
Discussant - Dr. Della Penna, a geriatrician, is Director of the Kaiser Permanente Aging Network. He is working with Kaiser Permanente to bring about change for those who are aging with a disability. Dr. Della Penna told the group that he feels that Kaiser's challenge is to work with an aging population. The KP network has set up four disciplines as structures and accountability - quality measurements to know how well Kaiser is performing. They will be asking question of the patient to monitor their success, such as how does the patient feel a specific program meets their needs; Kaiser will be working to find out if there is some way to know if good programs can be replicated to help many people in need. As a method to teach those in attendance about this issue, Dr. Della Penna asked Dr. Torres-Gil how he would suggest advocating health issues for oneself. Dr. Torres-Gil responded that gathering together with several groups works better to benefit the needs of many people. As he had suggested earlier, combining patients with similar problems needing similar solutions works to bring strength to their issues. He felt to advocate within a single group (i.e. just PPS, just ALS or just MS, etc.) does not attract political influence and support. We, as patients, need to be sophisticated as advocates so as not to polarize those whose help we are requesting.
Speaker - Dr. Susan Perlman is Director of the
Dr. Perlman began her presentation by addressing the fact that our zeal to rehab patients with polio could have probably created the consequences with which PPS patients now live. She added her own set of statistics stating that polio survivors run a 40% risk of developing new problems. She noted that other groups also develop similar syndrome problems...like post stroke syndrome as an example. In each group, approximately half will have future problems and half will not. Most people feel that time and overuse will be risk factors for developing Post Polio problems later in life. Her experience gave less credence to time. She feels a deeper issue is the severity of the residual deficit (while years go it wasn't thought to be an issue) coupled with overuse. Gender is not thought to be an issue. She then ran a history of statistics that are thought to take their toll on this process, such as wear and tear on the body, longevity genes, genes that turn off as we age, genes that turn on which steal resources from other places, bad genes that show up later in life, oxidative stress, increased free radicals, decrease in energy production, hormones in aging related changes, approaches to living longer, the immune system with aging and the older adult and a low grade inflammation in the spinal fluid, brain and blood that cause increased problems just as in MS.
She reviewed the history of polio and the statistics of the vast numbers of those affected. She reminded all that just because you have PPS it doesn't exclude one from other health problems as well.
Dr. Perlman interjected here that there may be two or more types of PPS: some people experiencing fatigue and pain may be in one group, and those with progressive atrophy may be in another. Many studies could be done with funding, if available. Within her suggestions addressing polio survivors, she noted: do not use drugs that weaken nerve muscle communication, don't work muscles to the point of exhaustion, and use assistive devices, like bracing. Dr. Perlman said that improving part of the problems would help others, like breathing help using night ventilation will assist with fatigue. Neurologists should meet with patients annually and do normal strength testing, but not necessarily invasive testing such as EMGs. Use resources for funding where available. In question and answers, she was asked about drugs and some study designs available in the coming years. She discussed the factors of weight and new designs in bracing as examples.
Discussant - Dr. Bakshi is also a neurologist. She has been able to observe many patients
with polio in her native
As far as old polio patients with no PPS symptoms yet, she suggested to Dr. Bakshi and the group that a careful evaluation of health be taken. Then the patient needs to be reminded that there could be ten (or more) years of clinical sub-problems that the patient is not aware of, and some destruction may already have been done. The patient should be warned of the signs to watch for like undo fatigue or weakness that could be the first symptoms that they were experiencing problems. Statin type drugs were also discussed with a note to be cautious in their use.
On the funding issue, Dr. Torres-Gill remarked that more money is now being poured into the NIH than they can spend, but the problem is that there is no cure for PPS, so no money goes into research for it. Contacting members of congress could be one avenue to approaching this issue.
Speaker - Hilary Hallam, from the
Hilary’s objective in her lecture was to address doctors and make suggestions to them how polio facts can be resurfaced so that doctors are aware of the old condition. She warned the doctors in attendance that you can't tell if a person had polio necessarily by looking at them. She talked about the fact that doctors need to realize that polio was systemic, so consider all problems and not just tunnel vision about the part that the patient manifests as a clear problem. In people that are not sure of the why, perhaps the professional needs to ask a few questions about family history. Hilary told survivors to be sure to tell the health professional about a past history of polio, then address the issues that are giving you trouble in a systematic fashion. Both sides need to carefully use correct questions and answers to find total information. She talked about the way we, as PPS patients, have learned to compensate for our limitations. She also addressed manual muscle testing and how inadequate it is for our purposes. Repetitive muscle testing techniques are the most effective for our condition.
Discussant - Dr. Shannon O'Brien was present to discuss the psychological aspects of having had polio. With time waning in the morning presentations, She actually asked the question of Hilary about how she handled the psychological aspects of being limited again in her life after having been saddled with the problem the first time.
Speakers next, on the research panel were Dr. Tessa Gordon, from the University of Alberta (Canada) Neurosciences Center and Dr. Spencer Wang, Director of Diagnostic Medicine, Kaiser Vallejo - a neuroscientist perspective - Progressive activity related regression of the sprouting capacity of surviving motoneurons.
Dr. Gordon explained sprouting, denervation and reinnervation of muscle fibers. Her discussion paralleled that of Dr. Sam Pfaff, Salk Institute researcher, for those of us who have been fortunate enough to hear his presentations. In her report she suggested that “… age-related loss of functional motor units appears to be associated with impaired sprouting, which, in turn, may be directly related to the progressively higher daily activity that the remaining motor units experience.” One of the most notable sentences in her brief report in the syllabus was the following: “Indications that relatively low levels of daily neuromuscular activity may increase muscle endurance suggest that conservative approaches to exercise may be taken with caution.”
Dr. Wang said that the muscle information given to us by Dr. Gordon helps explain the why of what he learns on EMG's and what is happening to nerves. He told us that tests cannot always determine all the answers we want. He explained the EMG process/muscle probe by needle. A typical EMG tests seven to ten muscle fibers. He can see the condition of the muscle. He can tell if cells have been orphaned and if other cells have come to cover for them. He discussed conventional EMG to Macro EMGs. Macro EMG gives a more global perspective of the muscles. It lends itself to missing less data because it covers more territory. He mentioned that there is no “gold standard” to follow pathologically to measure muscle size. Basically you need to follow trends in determining readings. He told the group that if Post Polio survivors over tax muscles, things likely will get worse and deterioration is the end result. But the reverse to that is if survivors do activities to relax muscles, such as water therapy or specifically testing physical therapy with assistance from a professional, things can get better...perhaps not on muscle strength itself, but daily functioning and pain can be improved.
The afternoon session was jam packed with information, and it closely followed the syllabus that each attendee received. Because there was so much Post Polio territory to cover and discuss, time, throughout the conference, was an issue. Because of that some of the syllabus items were quickly brushed over. Because of the completeness of the syllabus information, I have chosen to include some of it directly into this report. Drugs that might work, or at least held some hope of doing so, fell into that category. I have chosen to include this first because the afternoon session continued with a quick review of this subject. Please bear with me on this lengthy info because it is important information for us to know!
The following drug review is a direct quote from the syllabus:
Selegiline (Deprenyl), a neuroprotective agent primarily used for the treatment of Parkinson’s disease, may warrant further study on the basis of initial results from case reports. Two cases showed that selegiline produced an improvement in PPS symptoms; this improvement ended after the drug was discontinued.
Bromocriptine mesylate (Parlodel), a dopamine receptor agonist used to treat conditions such as Parkinson’s disease, was evaluated in 5 patients with PPS and produced improvements in fatigue symptoms in 3 of the participants.
(Dr. Sandel said to the group that she is interested in Dopamine agents, and she sees some promise here for fatigue. The greater issue here is that fatigue must be defined.)
The anticholinesterase pyridostigmine (Mestinon), which is already approved for treatment of myasthenia gravis, has demonstrated some promise in relieving the symptoms of weakness and fatigue in patients with PPS. In an open trial of 17 patients taking a daily dose of 180 mg pyridostigmine, a significant relationship was found between subjective fatigue response to pyridostigmine and improvement in neuromuscular junction transmission after administration of edrophonium (a short-acting anticholinesterase similar to pyridostigimine.) In another open trial with 27 patients, 59% who received pyridostigmine reported an improvement in fatigue with the medication and requested continuation of treatment. In addition, a double-blind placebo-controlled, crossover trial demonstrated improvements in some objective measures of strength and subjective measures of fatigue when pyridostigmine, 180 mg/day, was given to 27 patients. Adverse events, including loose stool and intestinal cramps, blurred vision, increased urinary frequency, muscle cramps and fasciculations or muscle twitches, were seen in approximately 50% of patients participating in these three trials.
The ongoing North American Postpoliomyelitis Pyridostigmine Study (NAPPS) is a multicenter, randomized, double-blind placebo-controlled phase II trial involving 126 patients with PPS. The study is designed to determine the efficacy of pyridostigmine, 180 mg/day, in relieving PPS symptoms using measures of fatigue, muscle strength, and health-related quality of life. NAPPS was also designed to determine the incidence and severity of any side effects associated with pyridostigmine. The study showed no difference between patients who received pyridostigmine and those who received placebo with regard to health-related quality of life, fatigue, and most measures of isometric strength. However, a trend to increase strength in very week muscles was seen at 6 months with pyridostigmine. The result of no significant difference in outcome measures between patient groups did not correlate with the investigators’ clinical impression that the medication appeared to be beneficial in a proportion of patients with PPS.
Of great interest to me were the presentations on
respiratory problems and PPS discussed by Dr. Edward Oppenheimer, a past chief
of the Division of Pulmonary and Critical Care Medicine at the
He then asked the question, “Who is at risk?” And responded that, “All polio survivors are at risk.” He added that the risk is higher if there was paralytic polio with respiratory or bulbar problems (weakness) during the acute polio illness. New breathing problems were reported by 42% of patients with post polio syndrome; and 88% of those who needed assistance during the acute phase needed assistance again in 3 to 59 years. Breathing problems are especially likely to occur in patients with residual respiratory muscle weakness.
In the discussion of the physiology of the respiratory disorders in PPS, among the many facts that Dr. Oppenheimer presented, I found particularly interesting the fact that “Deep stages of sleep worsens respiratory muscle weakness and results in a cascade of event that increase progression of respiratory failure and daytime disability.” Also of particular interest to my situation was that, “Sleep disordered breathing (e.g.: OSA, CSA, hypopneas, etc.) are due to bulbar impairment and/or central) brain changes.” Then came the question, “Is there hypoventilation vs. SDB (Sleep Disorder Breathing) without respiratory muscle weakness?”
He also went through the litany of symptom related to PPS
respiratory difficulty, and he noted that these symptoms are often
misinterpreted. He also noted that, “…the
very slow progression of motor neuron impairment in PPS may result in early
symptoms being overlooked both by the individual and the physician.” He then added a quote by Laghi and Tobin
(2003), “Respiratory muscle weakness
frequently goes undetected in patients with neuromuscular disease until
ventilatory failure is precipitated by aspiration pneumonia or cor
pulmonale. Diagnosis is delayed because
limb muscle weakness prevents patients from exceeding their limited ventilatory
capacity. A few patients develop severe
respiratory muscle weakness despite little or no peripheral muscle weakness.” Dr. Oppenheimer added to the respiratory symptoms
list that, “Central (brain) changes may contribute: such as central
disregulation of breathing and decreased respiratory drive.”
Tests that may be helpful for baseline evaluation and for follow-up:
And that, “Patients with diaphragmatic weakness are at particular risk of developing hypoventilation during rapid eye movement (REM) sleep.”
Dr. Oppenheimer cautioned that sleep disorders occur frequently in patients with PPS and it is important to determine if these are due to respiratory insufficiency/chronic alveolar hypoventilation (CAH) or sleep apnea and other sleep disordered breathing (SDB) with CAH. Sleep apnea may be central, obstructive, or mixed – it is treated similarly to sleep apnea seen in patients without PPS. He also noted that if breathing problems are not recognized, diagnosed and treated, the PPS patient might be susceptible to serious complications and death (as also noted by Frisca Yan-go in 1999).
He told the audience that a survivor who is having breathing problems has four choices:
The goals of noninvasive ventilation include to relieve symptoms, normalize sleep, improve QOL, improve survival, improve cognitive function, survive acute respiratory infections without crisis, avoid hospitalizations, maintain SaO2 greater or equal to 95% without added oxygen.
Because of Respiratory Muscle Fatigue, it is valuable to start ventilation early in the condition of PPS. Some advantages of starting MV (Mechanical Ventilation) “earlier” if related symptoms are present are 1) Using a night breathing machine is never detrimental, 2) it provides experience with noninvasive ventilatory equipment (such as BiPAP or VPAP) before a crisis occurs, 3) the patient will then have the ability to survive an acute respiratory infection if it does develop. And, 4) this also provides a “hands on” trial of the equipment to help decision making (like a test drive).
As a side note, Dr. Oppenheimer added that all patients receiving breathing assistance should have all three breathing options with their machine. They need a mask, nose device and a mouthpiece for when patients have a cold or times when they have nose-breathing difficulties. He also said that a CPAP should not be used for sleep disorders with hypoventilation.
Discussant – Dr. George Grossman
Dr. Grossman had an opportunity to answer a couple of
questions, but the one most important to me was one in which someone in the
audience asked about asthma and it’s relationship to PPS. Dr. Grossman was emphatic that Asthma and
night breathing problems could, indeed, be both related to PPS.
Speaker – Dr. Selma Calmes
Dr. Selma Calmes did an EXCELLENT presentation on Anesthetic Agents and Other Medication Considerations and Contra-Intraindications. She went into great detail explaining what kind of anesthesia is best, describing the three types which are general anesthesia (being completely asleep), regional anesthesia (only the part of the body being operated is on anesthesia) or Monitored Anesthesia Care (MAC – the surgeon injects a local anesthesia at the site of surgery and the anesthesiologist sedates and monitors the patient). One highlight of her talk was that there is fear of regional anesthesia among post-polio patients. Since it might be spinal (administered in the fluid around the spinal cord) or epidural (given in a potential space before the spinal cord to anesthetize the lower part of the body), there is fear that it will further compromise neurons in an already compromised post-polio spinal cord. Whether the spinal cord is normal or already compromised, she had specific recommendations about the drugs patients could use with the input of their doctor and anesthesiologist if a patient chooses a spinal anesthesia. Dr. Calmes did not, however, go as far as to recommend regional anesthesia for Post Polio patients.
Her surgery suggestions were many, but the ones that applied
to breathing were of particular interest to me.
In her information, she noted a that, “If a post-polio patient needs
assisted ventilation for daily living, obviously this will have to continue
postop, and ventilatory requirements may get worse immediately postop. These patients usually use negative pressure
or non-invasive methods of ventilation, unfamiliar techniques for modern
intensivists, who usually manage postoperative ventilation only in hospitalized
patients. Planning for these patients
needs to involve whoever is managing the patient’s ventilatory needs.” ALSO, “Polio patients who were not in an iron
lung can also have layngeal and swallowing problems and may need preop ENT
evaluation also. Evaluating
preoperatively can eliminate some unplanned problems postop.
The remainder of the afternoon session was devoted to Rehabilitation. Involved in the team discussions were physical therapists (which discussed such things as gait, balance, and bracing), a Speech Therapist who talked about Dysphagia, Cognition and Speech related issues, and an exceptionally good occupational therapist, Grace Young, who herself is a polio survivor, that addressed issues of daily function and living. A brief outline of her information was as follows:
a. Store everyday dishes in stacks of their own kind.
b. Vinyl coated wire racks preserve space and energy by limiting lifting.
c. Use lever handles on interior doors and for bathroom faucets.
d. Eliminate stairs.
e. Remove thresholds or install a gentle sloping beveled ramp.
f. Enlarge doorways with Swing-Clear hinges.
g. Automate your home.
1. X-10 technology works through the existing AC wing in your house, gives automatic or remote control lights, heating/cooling, audio, videos, drapes, door locks, alarm system, pet feeder, and hundreds of other devices.
As you can tell, this was an enlightened presenter since she was writing from the perspective of a handicapped person who lives these limitations herself.
The remaining time was devoted to Rehabilitation Interventions in the Vocational Setting. The two presenters in this area were Ana Verran and Jeff Symons of Project Threshold. Project Threshold helps individuals with physical disabilities solve task performance problems through the use of assistive technology or environmental modifications. Individuals who are having difficulties at work, at school, or at home with independent living skills are candidates for the services of Project Threshold.
In their presentations, they covered the issues of on the job modifications for disabled workers who choose to continue working in spite of their handicaps and what equipment modifications are available to make their jobs easier.
I would rate this conference an A+! It had all the components to make such an event successful. The speakers were highly rated professionals, outstanding in their respective fields. There was information available from which everyone could learn whether the attendee was there as a learning professional, an informed Post Polio Survivor or a newly diagnosed Post Polio survivor. The information was current and well delivered.
Video tapes can be purchased through Kaiser Permanente's
Multimedia Office at 1950
Also, they can be checked out of the Kaiser Wellness Library by visiting their website at www.kpwellness.org .