Articles
9/8/2005
Alternative therapies……Acupuncture.
Tony M; an out-of-county member has kindly sent us information on the use of acupuncture for relief of P.P.S. symptoms. We have a (poor) photocopy of a list of research summaries, mostly from China, on the effectiveness or otherwise of acupuncture. The basis of acupuncture, crudely, is the Chinese belief that energy flows through the body along particular pathways which can become obstructed. By inserting very fine needles into specific points on the body, these energy flows can be improved and better health restored. Whatever the truth is about how acupuncture works, there is no doubt that for some people it is very effective, and some western doctors have even used it for anaesthesia in selected patients.
Auriculotherapy ("auriculo” = ear), is a specific type of acupuncture involving the placement of tiny needles in the ear lobes, for the treatment of “neurological” (nerve related) problems, including post-polio problems. Whilst it sounds distinctly odd, I am told the basis of this is that key energy flow routes converge in the ear area. An American called Dr. H`Doubler has published research papers on this in the American Journal of Acupuncture, and can be contacted by e-mail : “hdoubler@smartnet.net"; or Fax : 001-417-882-3210. There is also a 1982 paper on the “Effect of acupuncture therapy in polio paralysis” in the British Journal of Acupuncture Vol.5 (1) p. 5-9. The author is A. Agrawal
Alternative therapies…….at your local chemists.
There has been a lot of support in the international and British polio press for two fairly simple D.I.Y. treatments which I will therefore briefly mention here. Firstly, Boots the chemists and other chemist and health food stores, sell a “co-enzyme” (digestive chemical) called Q10 which apparently helps many P.P.S. fatigue sufferers. The British Polio Fellowship warns that whilst some members are delighted by the results of using Q10, it can affect the balance of chemicals in cells, and should not be taken with other vitamins or medicines without consulting the chemist or your G.P.
Chemists also sell a nasal strip of plastic originally designed for rugby players, that holds the nose more open at night improving oxygen flow. The benefit comes from better sleep, less disrupted by wakefulness caused by low oxygen levels. Manufactured by 3M, these should be easily found or ordered by local chemists. As far as I know no warnings are necessary about these, no doubt the packaging will clarify this.
6/8/2005
Uncovering the Hidden History of Polio to Understand and Treat “Post-Polio Syndrome” and Chronic Fatigue.
by Dr. Richard L. Bruno
Every polio survivor deserves to know the truth about polio and ‘Post-Polio Syndrome’ – its diagnosis, cause and treatment – to be found in The Polio Paradox .
Sir Arthur C. Clarke
Polio Survivor, Class of ‘62
Author, 2001: A Space Odyssey
The Polio Paradox helps polio survivors reconnect with the survival skills, courage and internal resources that allowed us to survive polio and that will help to build a new life – and maybe even a better one – with PPS.
Mia Farrow
Polio survivor, Class of ‘54
Actor, Author and Activist
There have been books about the polio epidemics, the polio vaccine, and “Post-Polio Syndrome.”
The Polio Paradoxis unique. It is about the bodies, minds and spirits of the world’s 20 million polio survivors.
The Polio Paradoxdescribes polio survivors physical and emotional battle against the poliovirus attack, as well as the medical professionals who hurt polio survivors when they were supposed to be helping.
The Polio Paradoxchronicles polio survivors’ Herculean struggle to become “normal” and the fear they are now experiencing as Post-Polio Sequelae – the unexpected “sequel” to polio – threatens to strip them of their hard won abilities and their lives of remarkable accomplishment.
The Polio Paradoxis also a medical mystery and a memoir. Dr. Richard Bruno, chairperson of the International Post-Polio Task Force and director of The Post-Polio Institute and The International Centre for Post-Polio Education and Research, has summarized the world’s medical literature on polio and PPS. In clear terms he explains the awesome power of the poliovirus, the damage it did inside polio survivors’ bodies – both obvious and hidden – and the physical and psychological toll taken on those who contracted “The Dread Disease.” He explains how damage done by the poliovirus so many years ago set the stage for new symptoms and explains what triggers PPS today by describing his 20 years of patient care experience and research on polio survivors and PPS.
Dr. Bruno also describes in detail The Post-Polio Institute program that not only helps polio survivors stop the progression of fatigue, muscle weakness and pain, but also allows them to decrease their symptoms and increase both their ability to function and their quality of life. And he addresses the greatest obstacle and most difficult challenge in dealing with PPS: the psychological scars left by the polio experience.
The Polio Paradoxthen goes further. It describes the nearly 70 year history of chronic fatigue outbreaks that occurred at the same time as the polio epidemics. Dr. Bruno describes the remarkable similarities between the symptoms and research findings on PPS, Chronic Fatigue Syndrome, Myalgic Encephalomyelitis and fibromyalgia. And he suggests that as many as half of those who now diagnosed with chronic fatigue – the Baby Boomers born before the discovery of the polio vaccine – may have had a mild case of polio in childhood and have PPS today, not CFS.
The Polio Paradoxis for polio survivors, those with CFS, ME and FM and anyone aging with a long term disability, who want manage their new symptoms, improve their quality of life and not just survive but thrive in mid-life. Warner Books, 2002
The similarities between CFS and PPS are tantalizing. Dr. Bruno’s work is immensely important not only to the many persons who had polio but to those with CFS as well.
Dr. David Bell
Chronic Fatigue Syndrome Pioneer
The Polio Paradox provides a wiring diagram and operator’s manual for the chronically fatigued body. Dr. Bruno’s treatment programme has amply proved its worth.
Dr. Elizabeth Dowsett
Founder, CFS Diagnostic and Management Service, Essex, England
Caliper technology
Is dragging at the chain
And needs a fresh infusion
From a scientific brain
We can take a broken body
And make a million dollar man
But if a caliper is needed
It tends to spoil the plan
For if our hero is a cowboy
He can’t get in the saddle
And if he is a sailor
He’s up-creek without a paddle
And if she were a dancer
She’d have problems with her rumba
While if she were a model
She’d have troubles without number
A million dollar man of course
Must have lofty aspirations
But lesser mortals such as we
Live within our limitations
Our caliper relationship
Is one of love and hate
But we value our mobility
And accept it as our fate.
Leicestershire and Northamptonshire Trading Standards Service has issued warnings that deserve a wider audience, as they particularly apply to a vulnerable group; elderly and disabled people. They concern inviting salesmen into your home when they contact you (by phone, mail or doorstep call) or when you respond to an advert.
Did you know that the 7 day cooling off period that is a Legal right, enabling buyers to change their mind and back out of a purchase agreement after they have signed it (often to get rid of a persistent salesman) does not apply if the salesperson has been INVITED to your home?
The reason so few advertisements for mobility aids like chairs, baths, and scooters carry PRICES is because the firm wants you to accept them into your home. They may then both overstay their welcome, and take advantage of you having lost your right to change your mind after the deal is done.
However, “If you agree to buy goods or services costing more than £35 and you did not invite the seller to call, you have seven days to change your mind. In these circumstances the law requires a seller to give you written details of your right to cancel. If you would like more information or have not received written cancellation rights, contact your local Trading Standards Service.” (County Council’s run Trading Standings Services.)
Respectable, good value firms are now emerging that advertise prices, and have sales rooms you can visit. If they then offer to bring items to you, at least you know what price is involved in advance. Remember that Trade Associations are precisely that, profit-focussed groups of traders, they are not Consumer or disability organisations. In some areas, Trade Associations have been able to limit competition and thus keep prices high.
The small ads columns of local newspapers carry many examples of poor purchases “only used once” “never used” “5 months old” at bargain prices. Some will be consequent to bereavement or worsened health, but many are simply bad buys. If you have family or friends to support you with travel and transporting goods, some great bargains are to be had in this way, particularly in scooters, riser/recliner chairs, bath and stair lifts, and wheelchairs. Most areas now have Independent Living Centres where volunteers and/or independent Occupational Therapists can help and advise without hard sell,
the Red Cross is sometimes involved. Once you truly know what you need, buying second hand can be a good way to save money.
The exception to these words of warning, would be regarding home visiting by not-for-profit aids providers, as there are several trustworthy organisations that help with disability needs for particular groups, e.g the British Legion, R.U.K.B.A. and others.
Generally though, the message is Buyer Beware! Keep those sales people out!
What is the Expert Patients Programme?
The Expert Patients Programme (EPP) is a new government initiative to help people living with long term conditions maintain their health and improve their quality of life through lay led self management courses.
Self management programmes have been running in the USA since the 1970s and came to this country in 1994. Over this time a substantial body of clinical research and experiential feedback has built up testifying to its efficacy.
The primary aim is not to impart medical information but to facilitate the development of self management skills, such as problem solving and goal setting.
The course has been designed to help people with long-term illness regain as much control over their physical and emotional well-being as possible. It complements existing health care programmes and treatments, empowering participants to be more informed and better able to develop partnerships with their medical practitioners.
What is special about the Expert Patients Programme?
• It is a generic, time-limited course, suitable for people who have any long-term condition.
• The Volunteer Tutors are themselves living with long-term conditions.
• Volunteer Tutors are trained and supported by the EPP.
• Tutors use a detailed, scripted manual, underpinned by ongoing research.
• There is a strong emphasis on participants setting practical and achievable goals that are evaluated each week.
Research shows that people who have attended the course:
• Have greater confidence in dealing with their illness
• Experience less pain and fatigue, depression and anxiety
• Are more likely to continue with exercise and relaxation techniques
• Make fewer visits to their GP
• Have better communication with health professionals
What does the Course involve?
The course takes place over six weekly sessions of two and half hours each.
The areas covered include:
• Goal setting/action planning
• Problem solving
• Relaxation/exercise
• Symptom management techniques
• Fatigue management
• Dealing with emotions
• Communication
• Medication
• Community resources
What support will the EPP Trainers offer?
• Train and support course tutors
• Carry out accreditation, quality assurance and supervision of tutors
• Run courses
• Help introduce new approaches
• Ensure joint working across PCT boundaries
• Help in marketing of courses
• Help in development of structures to mainstream EPP
• Professional Development & Awareness
Basic facts about PPS for polio survivors’ doctors, family & friends.
Dr. Richard L. Bruno
Chairperson, International Post-Polio Task Force
Director, The Post-Polio Institute and The International Centre
for Post-Polio Education and Research
Englewood (NJ) Hospital and Medical Center, USA
WHAT ARE POST-POLIO SEQUELAE? Post-Polio Sequelae (PPS, Post-Polio Syndrome, The Late Effects of Poliomyelitis) are the unexpected and often disabling symptoms – overwhelming fatigue, muscle weakness, muscle and joint pain, sleep disorders, heightened sensitivity to anaesthesia, cold and pain, as well as difficulty swallowing and breathing – that occur about 35 years after the poliovirus attack in 75% of paralytic and 40% of ‘’non-paralytic'’ polio survivors. There are about 2 million North American polio survivors and 20 million polio survivors worldwide. The existence of PPS has been verified by articles in many medical journals, including The Journal of the American Medical Association, the American Journal of Physical Medicine and Rehabilitation, and The New England Journal of Medicine.
WHAT CAUSES PPS? PPS are caused by decades of ‘’overuse abuse.'’ The poliovirus damaged 95% of brain stem and spinal cord motor neurons, killing at least 50%. Virtually every muscle in the body was affected by polio, as were brain activating neurons that keep the brain awake and focus attention. Although damaged, the remaining neurons compensated by sending out ‘’sprouts,'’ like extra telephone lines, to activate muscles that were orphaned when their neurons were killed. These over-sprouted, poliovirus-damaged neurons are now failing and dying from overuse, causing muscle weakness and fatigue. Overuse of weakened muscles causes muscle and joint pain, as well as difficulty with breathing and swallowing.
HOW ARE PPS DIAGNOSED? There is no diagnostic test for PPS, including the electromyogram (EMG). PPS are diagnosed by excluding all other possible causes for new symptoms, including abnormal breathing and muscle twitching that commonly disturb polio survivors’ sleep, a slow thyroid and anaemia. Other neurological or muscle
ARE PPS LIFE THREATENING? No. But because of damaged brain activating neurons polio survivors are extremely sensitive to, and need lower doses of, gas and intravenous anaesthetics and sedative medication. Polio survivors can have difficulty waking from anaesthesia and can have breathing and swallowing problems, even when given a local dental anaesthetic.
IS PPS A PROGRESSIVE DISEASE? PPS is neither progressive nor a disease. PPS is caused by the body tiring of doing too much work with too few poliovirus-damaged, over-sprouted neurons. However, polio survivors with untreated muscle weakness were found to lose about 7% of their remaining, overworked motor neurons each year.
IS THERE TREATMENT FOR PPS? Yes. Polio survivors need to ‘’conserve to preserve,'’ conserve energy and stop overusing and abusing their bodies to preserve their abilities. Polio survivors must walk less, use needed assistive devices – braces, canes, crutches, wheelchairs – plan rest periods throughout the day and stop activities before symptoms start. Also, since many polio survivors are hypoglycaemic, fatigue and muscle weakness decrease when they eat protein at breakfast and small, more frequent, low-fat / higher-protein meals during the day.
ISN’T EXERCISE THE ONLY WAY TO STRENGTHEN WEAK MUSCLES? No. Muscle strengthening exercise adds to overuse. Pumping iron and ‘’feeling the burn'’ means that polio-damaged neurons are burning out. Polio survivors typically can’t do strenuous exercise to condition their hearts. Stretching can be helpful. But whatever the therapy, it must not trigger or increase PPS symptoms.
IS TREATMENT FOR PPS EFFECTIVE? Yes. The worst case is that PPS symptoms plateau when polio survivors stop overuse abuse. Most polio survivors have significant decreases in fatigue, weakness and pain once they start taking care of themselves and any sleep disorders are treated. However, because of emotionally painful past experiences related to having a disability, many polio survivors have great difficulty caring for themselves, slowing down and especially with “looking disabled” by asking for help and using assistive devices.
WHAT CAN DOCTORS, FAMILY AND FRIENDS DO TO HELP? Polio survivors have spent their lives trying to act and look ‘’normal.'’ Using a brace they discarded in childhood and reducing overly-full daily schedules is frightening and difficult. So, friends and family need to be supportive of life-style changes, accept survivors’ physical limitations and any new assistive devices. Most importantly, friends and family need to be willing to take on taxing physical tasks that polio survivors may be able to do but should not do. Doctors, friends and family need to know about the cause and treatment of PPS and listen when polio survivors need to talk about how they feel about PPS and lifestyle changes. But friends and family shouldn’t take control of polio survivors’ lives. Neither gentle reminders nor well-meant nagging will force polio survivors to eat breakfast, use a cane or rest between activities. Polio survivors be responsible for caring for their own bodies and ask for help when they know they need it.
Whether you had polio or not, please COPY and MAIL this letter to your doctors. With your help every doctor will learn about the cause and treatment of PPS and give polio survivors the care we so desperately need. Thank you!
Mia Farrow, polio survivor …………………Thaddeus Farrow, polio survivor
Co-Chairpersons
The POST-POLIO LETTER Campaign
For more information about the cause and treatment of PPS go to www.postpolioinfo.com
Sleep Apnoea Revisited
Ian Neering PhD MSc
Long-time Network member Ian Neering was Associate Professor of Physiology and Pharmacology at the University of NSW and now, as is appropriate with his degree of decrepitude (Ed. his description, not mine!), runs a drug consultancy from his home office.
Ian’s article below provides further insights into the subject of sleep apnoea, and reinforces the message of Dr Colebatch’s article – it is imperative for polio survivors to have their particular causes of sleep apnoea correctly diagnosed and appropriately treated.
Your articles in the last PPN Network News have prompted me to put down my experiences with sleep apnoea as they might be of interest to some of your readers. Let me stress that these are my personal experiences and people should not necessarily extrapolate from them to form any sort of generalisation.
By way of background, I should say that I contracted polio in 1950 when I was around 4 years of age. I was completely paralysed and spent a good deal of time in an iron lung. Over the years, with the sort of therapies most of your readers will know only too well, I made a pretty good recovery and was able to lead quite an active life without any prostheses of any kind. I have quite a marked kyphoscoliosis with significant deformity and associated low functional lung volumes. At around the age of 40, I started to notice the signs of PPS and my general condition started to deteriorate. I took to using a walking stick and was forced to modify my life style significantly. When I had respiratory infections, they lasted longer and were increasingly troublesome. I now need crutches to get around.
In 1993, a dose of flu saw me admitted to hospital with life-threatening respiratory failure which basically means that my lungs could not function sufficiently well to provide me with an adequate supply of oxygen. I was on a ventilator for a few days but was fortunate enough to make a good recovery. In the aftermath of my period in intensive care, sleep studies were performed and sleep apnoea was diagnosed. The respiratory specialist theorised that cumulative fatigue of my respiratory muscles, partially as a consequence of the sleep apnoea, was responsible for the respiratory failure and a CPAP device was prescribed.
Let me tell you, I struggled with that machine! Let alone the fact that no mask seemed to fit around my nose properly and jets of air burbled and squeaked from the poor seal around my face unless I lay “just so” in bed and didn’t move a muscle. Not conducive to a good night as you can imagine. I felt no improvement in my overall well being after weeks of persevering and follow up visits to the doctors.
I’m afraid I have a general antipathy to the medical profession not uncommon amongst old polios and a general mistrust of “blanket treatments”. I, like many polios, have become rebellious towards doctors’ prescriptions and prefer to find my own bumbling way to the solutions that best suit me. At my wit’s end, I decided to take matters into my own hands and here I have an advantage over most in that I am a physiologist by training.
I need to digress for a moment here. Your excellent articles on sleep apnoea didn’t say very much about the sleep study used to definitively diagnose the condition. When you go in for an overnight study, you are fitted up with a number of sensors. Cables are attached to your scalp for an EEG which records your brain waves and allows the investigators to assess your level of sleep. A finger probe records oxygen saturation of the blood which is the critical measure telling us we are not receiving sufficient oxygen as a result of the sleep apnoea. A more sensitive measure of ventilation is that of carbon dioxide but not all sleep studies include this. Wires are also attached to our chest so that the activity of the respiratory muscles can be recorded. This is known as an EMG. It’s important to understand that EMG records muscle activity only. A reduced EMG activity recording could be caused by a reduced drive from the motor nerves controlling the muscle or from a weakened muscle, unresponsive to nerve stimulation. There may be other sensors attached to measure airflow, leg movements and so
on. A good sleep study will look at all of these parameters; an inadequate one will not, and may lead to a misdiagnosis.
Now, your article referred quite correctly, to two main types of sleep apnoea: obstructive sleep apnoea and central sleep apnoea. In the former, the problem is mechanical in that respiratory muscles may be working well but actual airflow into the lungs is restricted for any of a variety of reasons. In the latter, there is reduced respiratory drive from the brain to the muscles of respiration resulting in a reduced or absent inspiratory effort. Weakness of respiratory muscles will exacerbate both forms of apnoea. Dr Joyner, in her classification, quite reasonably, has delineated a category just for polios which contains aspects of both forms of apnoea though, strictly speaking, does not add a third category to the two groups already defined.
A sleep study, by looking at the temporal relationship between the activity in the respiratory muscles, air flow and oxygen saturation of the blood, can determine what form of sleep apnoea you suffer from. The problem however is that getting to sleep with all those wires attached to you, in what for many of us is an unpleasant environment with unfortunate associations is not all that easy! The consequence is that we may not actually reach the deepest form of sleep during which REM or dream sleep occurs. The investigating physician therefore will not have a complete picture of what your breathing does during sleep.
Enough digression. I was lucky enough to be able to borrow (with appropriate bribing and inducements) some bits of equipment and set up to monitor my sleeping at home. I got hold of an oximeter to measure blood oxygen saturation. Diaphragm EMG was measured using a little battery operated portable device used by sports people. I measured airflow by taping a thermistor (which measures temperature of the air) under my nose. Measuring EEG was beyond the range of my ability to equip. It wasn’t really necessary. The hardest part was logging all the data. After all, I needed to be able to record oxygen saturation, EMG and airflow over a period of at least 6 hours. After fiddling with chart recorders and ending up almost buried in paper upon waking, I ended up recording all the data continuously on my computer. My partner was not particularly impressed that I had turned our bedroom into a laboratory but I soothed her with a vision of a well-rested husband who would not be snappy and grumpy if he had a decent night’s sleep. I can’t adequately describe my satisfaction to you when I finally saw the definitive result. Obstructive apnoea is characterised by an increased inspiratory effort coincident with the decrease in airflow. In other words, when the airway is obstructed, we try a whole lot harder to breath in, at least initially, and you can see this in an increased response in the tracing of the EMG from the muscles that help us inspire. In my tracing, respiratory effort just faded away coincident with airflow, breathing stopped because the muscles were no longer being asked to contract, a sign of a centrally mediated sleep apnoea. My problem I now knew was that during sleep, the drive to my inspiratory muscles, the messages from my brain telling my muscles to breath (respiratory drive), was reduced. In fact it would cease all together, causing blood oxygen content to fall until all those alarm mechanisms that the body has to detect such a dangerous malfunction caused me to wake up.
It is known that respiratory drive decreases during sleep even in normal individuals. Many polios have weakened respiratory muscles. It seems not unreasonable to suppose that for some polios the combined reduction in respiratory drive from the brain and the weaker muscles could result in the sleep apnoea. EMG recordings would be unable to dissect out this scenario. Remember, the EMG cannot distinguish between a reduction in nerve impulses from the brain to the muscle, or a weakly contracting muscle.
How did this new knowledge make a difference? Well, it meant that the CPAP machine was quite inappropriate for my needs. CPAP is great for the obstructive types of apnoeas where the positive air pressure from the machine “splints” the airways in an open position and prevents their collapse during the respiratory cycle. It’s a bit like breathing in a gale. CPAP won’t help you if you simply stop breathing because its flow of air is continuous. There’s no cycling. Armed with my new data, I was able to show my respiratory physician that a different form of therapy was required. He promptly prescribed a BIPAP machine which is a bit like a ventilator in that it cycles through an inspiratory and an expiratory phase. There is an obstructive
component to my sleep apnoea and the BIPAP machine will help against this also. That machine is now more than 10 years old and has become an essential chattel of my life.
Since using sleep support, I have had fewer respiratory infections and those I have had, have had a more benign outcome.
Why have I felt the need to put all of this down on paper? My main motivation is a fear that some of you may go through the same misdiagnosis that I did. My message is: for those of us who had respiratory paralysis during the acute phase of polio infection (and possibly for others), CPAP may NOT be the treatment of choice either because of muscle weakness, reduction of central drive or a combination of both.
I still have trouble with masks. Having spent much time and effort making plaster casts of my face and attempting to mould masks, cast masks, and experiment with various materials, I’ve found that the “disposable” resuscitation masks sold by the St John’s Ambulance people work as well as any. Use of these with a fabric “gasket” and my partner’s design of head harness has enabled me to sleep relatively comfortably even allowing me to toss and turn as the dictates of comfort demand.
One other fascinating aspect of these machines that I’ve not seen mentioned anywhere else is the tendency, for me at least, to cause psychological dependence. This manifests as an inability to sleep at all without the machine. On occasion when I’ve been camping and my car battery fails, in a power outage, or when I have had to travel in an emergency without my machine, I simply can not sleep without it. On one occasion I had no sleep for two consecutive nights. On the third night, I was so exhausted I did manage to sleep fitfully. I wonder if others have noticed this effect?
First printed in Network News
Issue 64, June 2004
the newsletter of Post-Polio Network (NSW) Inc
Frequently Asked Questions
What is Post-Polio Syndrome (PPS)
Post-Polio Syndrome is a group of new neuro-muscular symptoms occurring in patients many years after their Polio infection.
It is estimated that between 1/3 and 2/3 of Polio survivors will eventually experience PPS.
In order of frequency problems may include :
• Abnormal fatigue - both general fatigue and muscle fatigue.
• New muscle weakness
• Pain in muscles and joints
• New breathing difficulties
• Muscle cramping and twitching
• Swallowing difficulties and voice changes
• Muscle atrophy (loss of bulk) or hypertrophy (enlargement)
• Sleep disturbances
• Cold intolerance
History of the term Post-Polio Syndrome
Dictionary definitions (Concise Oxford).
“Post (prefix) after in time or order.”
“Poliomyelitis (noun) – an infectious viral disease that affects the Central Nervous System and which can cause temporary or permanent paralysis”.
“Syndrome (noun) 1. a group of concurrent symptoms of a disease.
2. a characteristic combination of opinions, emotions, behaviour etc.”
The fact there are two meanings of the word “Syndrome”, one purely medical, one more broadly based, has muddied already complex issues in the U.K. Some doctors have fought for control of the language of Polio discussions, and been very resistant to public use of the word “Syndrome”. Others have been happy to use it, but in the limited context of a specific group of symptoms including new neuro-muscular weakness, and excluding wear-and-tear type problems. However many now confidently diagnose PPS after excluding other possible causes for a patient’s new neuro-muscular symptoms, using internationally accepted medical criteria.
This is because alternative labels have proved elusive. The tortuous jargon of health and social care assessments transfers poorly to medical literature, e.g., a 2003 Neurology review article entitled “Late Post-Polio functional deterioration.” Previous attempts including “Ageing with Polio” were misleading since published research had demonstrated normal ageing did NOT explain the new weakness experienced by some survivors. The phrase “The Late Effects of Polio”(LEP) is useful but may be taken to imply inevitability, when in fact some survivors may never experience PPS symptoms. LEP has also been used by some doctors as a distinct diagnosis for groups of abnormal wear-and-tear type conditions including nerve entrapments (e.g. carpal tunnel syndrome), and inflammation of nerves, muscles, or joints (e.g bursitis, arthritis) where new weakness does not feature. This failure to find understandable, concise, alternative language, has however lead to increased use of the term Post-Polio Syndrome.
Hopefully British Polio survivors will consequently be empowered to protect their own heath and functioning through effective information sharing, after being held back for over a decade by misunderstandings, semantics, medical politics, and false reassurances.
Living and Breathing with Weak Respiratory Muscles
Hal John Hester Colebatch AM MD FRACP
Network member Dr John Colebatch was born in 1928 at Northam, Western Australia. He entered first year medicine at the University of WA, completing the course and graduating in medicine from Adelaide University in 1951.
In April 1953 while working at the Infectious Diseases Branch of the Royal Perth Hospital, he contracted poliomyelitis, becoming totally paralysed and requiring treatment in an iron lung. After a year recovering in hospital he rejoined the medical staff of the Royal Perth Hospital with responsibility for infectious diseases and rehabilitation.
In 1958 he commenced research on lung disorders in the Department of Medicine, University of Sydney. He continued his research at the University of California, San Francisco and then at the University of Oxford. In 1964, he was appointed to the academic staff of the University of New South Wales as Senior Lecturer in Medicine and joined the Department of Respiratory Medicine, becoming Associate Professor in 1970 and Chairman of the Department in 1985. In 1970-71 he was a Senior Fulbright-Hayes Research Scholar and a Visiting Associate Professor of Physiology at the Harvard University School of Public Health. From 1968 to 1992, he was a Senior Specialist in Respiration to the Royal Australian Navy. In 1989, he received the inaugural award of the Fisons medal of the Thoracic Society of Australia and New Zealand. In 1992, in recognition of his research on the lungs, he was made a Member of the Order of Australia.
Because of increasing muscular weakness – the post-polio syndrome – Dr Colebatch retired at the end of 1992 and was appointed a Visiting Professor of Medicine, continuing part time work until 1996. Muscular weakness led to the use of a ventilator at night and an electric wheel chair to maintain mobility.
We are grateful to John for writing this article especially for Network News. He will be pleased to respond to any questions on this subject – just send them to the Editor.
The return of muscle weakness 20 or more years after the initial paralysis and its relentless progression is well known to all who have experienced the post-polio syndrome. In this account, which is based on my own experience, I will be primarily concerned with the recurrence of respiratory muscle weakness in those patients who during the acute stage of their illness suffered respiratory muscle paralysis. I will assume that the lungs are normal and that chronic respiratory failure is the result of respiratory muscle weakness alone.
Breathing, when it fails, is a whole of life issue; it affects all activities during the day, as well as the quality of sleep. The aim of management is to maintain the best possible activity during waking hours. To understand how this can be achieved it is necessary to consider two important issues – first, how breathing is regulated and second, how muscles produce movement and enlarge the chest.
Background
In April 1953, while working in an Infectious Diseases Hospital, I became completely paralysed as a result of poliomyelitis and was placed in an “Iron Lung”. It was 74 days before I was freed from this coffin-like machine. About three months after my initial paralysis when I stood up for the first time I was unable to breathe at all, owing to the effect of gravity increasing lung volume. Since then I have always worn an abdominal support and/or a tight belt around the lower abdomen before standing up. It was one year before I was able to leave hospital, walking with the aid of crutches. I maintained a reasonably active life, including working overseas, but after 25 years it became clear that muscle
weakness was returning and gradually its severity increased until once again I was unable to walk and required assistance for showering and dressing. The relentless progress to dependence has been a deeply distressing experience.
In 1991, after a “Sleep Study” I was advised to use a machine to produce continuous positive airway pressure (CPAP) during the night. I persevered with this treatment, but was able to sleep using it on one night only. Subsequently I realized that CPAP was not the appropriate treatment for anyone with weak inspiratory muscles and discontinued my use of this machine.
In September 1992, after another “Sleep Study”, I started using a fixed volume ventilator at night. Initially, there was some improvement in sleep quality, but this did not persist. Because it prevented spontaneous breathing, I found this ventilator intolerably uncomfortable to use. As a result of its use with an excessive level of ventilation, breathlessness during the day became severe and interfered with activity. After three weeks of nocturnal ventilation, I became troubled with irregular heart beats. By decreasing .the amount of ventilation during the night, the irregular heart beats were abolished and breathlessness decreased.
In November 1992, I started using a BiPAP Ventilator which was intended to allow me to initiate breathing and be comfortable to use. However, this early model was unduly sensitive and was triggered into inspiration because the heart beat caused a small flow of air (about 10 ml) into the lungs. As a result, my breathing was not normally regulated, but was abnormally increased because it was initiated by the heart beat rather than by my own inspiratory efforts. I was soon troubled again with severe daytime breathlessness and irregular heart beats and, in addition, a feeling of impending death. These problems were overcome by the use of a modified BiPAP which was not triggered by the heart beat.
Recognition of Failing Breathing Muscles
Difficulty with breathing is likely to develop in post-polio subjects who suffered respiratory muscle paralysis during the acute stage of their illness. Initially there is increased breathlessness and fatigue and, later, difficulty sleeping. To assess this problem it is essential to measure:
• breathing capacity, and
• arterial blood gases.
Breathing capacity is usually assessed by measurements of vital capacity (VC) and the maximum volume expelled in 1 second (FEV1). Before there is a serious problem with respiratory failure, both VC and FEV1 decrease to less than 50% of the expected value. But a more significant measurement is inspiratory capacity (IC) or the volume which can be inspired from the resting position. It is this volume which represents how much the subject can breathe. As lung volume increases, inspiratory muscles are shortened and IC decreases, that is, the ability to inspire decreases. That is why it is more difficult to breathe when sitting or standing compared with lying down. A tight belt around the lower abdomen will decrease lung volume when standing and increase IC and therefore the ability to inspire.
Arterial blood gases require collection of arterial blood which is normally done in a hospital in close relation to the laboratory where the measurements are made. An increased arterial CO2 level defines the presence of chronic respiratory failure. By the time this becomes a problem, arterial CO2 will usually have risen from a normal level of 40-45 mm Hg to around 55 mm Hg. If arterial CO2 remains in the normal range, assisted ventilation is unlikely to be required. In the presence of normal lungs, oxygen saturation is well maintained, so that oximetry is unreliable and of little value in assessing the presence of chronic respiratory failure.
The respiratory muscles and chest wall together make a pump which moves air into and out of the lungs and is referred to as “ventilation”. The pump is driven by the nervous system. When the pump cannot provide adequate ventilation there is a feeling of breathlessness and the arterial CO2 increases until the quantity produced is expired in the smaller volume of ventilation. This situation is referred to as “chronic respiratory failure”.
When weakness of breathing muscles has caused chronic respiratory failure nocturnal ventilation is required. This ensures adequate ventilation during the night and allows recovery of fatigued muscles. The aim of this regimen is to maintain activity and independent breathing during the day. To minimise the increase in lung volume with gravity, when sitting or standing, it is essential to provide abdominal support. This will help to maintain the ability to inspire.
Managing Nocturnal Ventilation
When breathing is failing, one of the symptoms is difficulty sleeping. Sleep studies may be undertaken to help define the breathing problem. However, these studies are complex and in my view may lead to errors in management. Notwithstanding evidence of obstructive sleep apnoea, which is commonly observed, treatment with CPAP is not appropriate for anyone with weak respiratory muscles. This is because an increase in airway pressure increases lung volume and shortens respiratory muscles making it more difficult to further increase lung volume and maintain breathing. It is as if the subject were sitting up all night instead of lying down and instead of resting the inspiratory muscles it makes their work more difficult.
Breathing is driven by the acidity of fluids around the brain which in turn reflect metabolic activity. The best type of breathing assistance is one which allows this regulation to continue in its natural state. That is, the subject’s brain should drive the ventilator as part of the chest pump, the ventilator should not determine the amount of ventilation independently of the subject’s metabolism. It follows that the subject initiates inspiration to which the machine responds and supports – a demand-driven machine. A pressure support ventilator fits this criterion and such machines are in common use. My preference is for the BiPAP Ventilator operated in demand mode. I have used this ventilator every night for more than eleven years.
Fixed-volume ventilators are uncomfortable to use at night for anyone who retains some ability to breathe independently. They also do not permit the subject to regulate his breathing. A similar disadvantage applies to a pressure-assist ventilator set to a fixed time, which excludes the subject from initiating inspiration. If there is concern about the occurrence of apnoea during sleep, this possibility can be overcome by setting the ventilator to a slow back-up rate, say 8 per minute, which will maintain sufficient ventilation if needed, but not interfere with the subject’s own breathing.
Improving Daytime Activity
One of the objects of nocturnal ventilation is to improve daytime activity. For this to be achieved the level of ventilation during the night must not exceed what the subject can maintain when breathing independently during the day. This is best achieved by using a ventilator in demand mode with the subject retaining regulation of breathing.
There should be no attempt to decrease to “normal” an increased arterial CO2. The level of CO2 is set by the ability of the chest pump to respond to the demands for breathing during daytime activity. To lower CO2 during the night, in these circumstances by excessive ventilation, will increase daytime breathlessness. It may also be dangerous by causing irregularity of the heart beat. Normally CO2 is a little higher during the night than during the day and this situation should be allowed to continue. If excessive nocturnal ventilation is
suspected it may be worthwhile to measure the electrolytes. The risk is an abnormal loss of potassium resulting in irregularity of the heart beat. This disorder may be fatal. Potassium supplements will correct this problem.
Weakness of post-polio muscles is an ongoing problem which cannot be overcome by exercise. It is sufficient to try to maintain the activities of daily living. To do this it is essential to have adequate periods set aside for rest during the day. (Exercise programs are for an earlier stage of life.) Post-polio paralysis is not a disease of muscle, rather it reflects a loss of those motor nerve cells and fibres on which initial recovery depended. The surviving muscles with their remaining nerve supply are normal. It follows that nutritional supplements or anabolic steroids aimed at improving muscle function are unlikely to have any lasting effect. On the other hand an adequate intake of the vitamin B group may be helpful for their essential role in the nervous system.
Besides exercise, metabolism and with it the demand for breathing is increased by intake of food. Breathlessness during the day, while at rest, is distracting and can be decreased by limiting intake of food to the smallest amount that can be tolerated. This regimen has the additional benefit of discouraging an increase in body weight when any increase is a serious disadvantage for continuing activity.
In situations where exertion cannot be avoided such as toileting it is an advantage to have the use of a ventilator. This can make the difference between dependence and independence.
The slowly progressive nature of the post polio syndrome means that new challenges to activity and independence arise year by year and even each few months. To meet the particularly difficult challenge of respiratory failure and yet maintain some independent activity it is essential to consider carefully all possible adjustments. Simple things such as limiting daytime intake of food and ensuring rest periods can help to reduce discomfort and make the days a little more enjoyable.
First printed in Network News
Issue 64, June 2004
the newsletter of Post-Polio Network (NSW) Inc
19/7/2005
Six New Books about Polio
Mary Westbrook
Here are brief reviews of six recently published books about the polio epidemics, the development of the vaccine, and the aftermath of the epidemics. Most have been published by university publishing presses and this is reflected in the quality of the writing. With the exception of Marc Shell and Patrick Cockburn the authors are Americans and their focus is almost exclusively on the American polio experience. Shell, who is a Canadian by birth, knows there is a wider world out there where people lived and still live with polio. It’s nice to see references in his book to many other countries including Australia eg the Tasmanian polio epidemic and its survivors and Carol Mara’s book Iron Cradles. Anglo-Irishman Cockburn contracted polio as a six year old child in Cork. Wilson, Shell, Cockburn and Irvin are identified as polio survivors. Probably for the polio survivor Wilson’s book is the most tempting to buy as it is entirely devoted to what concerns us most, polio survivors’ life experiences. Encourage your local library to order books that appeal to you. I have given the Amazon.com prices. Amazon also sells second hand books via their website so you may pick up a cheaper copy. My experiences of this facility have worked out satisfactorily to date although some second-hand booksellers on the Amazon lists do not send books to Australia.
Polio: An American Story by David Oshinsky
The author, a professional historian, gives a detailed, very interesting, accurate ‘warts and all’ description of the American polio epidemics and the protagonists in the race for a polio vaccine. The focus is unrelentingly American. The author writes: Americans were primed to see polio as an indigenous plague… Why did it [polio] thrive in the United States? (No mention that there were worse epidemics elsewhere eg Iceland, Tasmania had higher per capita rates). It was the good old Yankee virtues of know-how and can-do that resulted in the conquest of polio and even though the main players, Salk, Sabin, Koprowski and Basil O’Connor were not genetically American, as they were all the children of migrants, that is all the more reason to view the conquest of polio as truly an American story. (How does one become genetically American I wonder?). The heroes have feet of clay. Oshinsky says that Sabin’s best friends described him as arrogant, egotistical… ‘Smart. But a brainsucker’ who took too much credit for a collective discovery. Salk was accused of similar behaviour. Not everyone applauded Salk’s announcement about the development of the vaccine. His co-workers were angry as no mention was made of their contribution. Some saw Salk as a man who cultivated the press with the same care he cultivated viruses. This damaged Salk’s standing among scientific researchers. Oshinsky says: It is revealing that while Salk was awarded his country’s Two highest honours—the Congressional Gold Medal in 1955 and the Presidential Medal of Freedom in 1977—he was denied admission to the elite National Academy of Sciences for the reason, it was said, that he had made no ‘basic scientific discovery’. As Albert Sabin, a long-time academy member sneered: ‘You could go into the kitchen and do what he did’. The feud between Salk and Sabin would outlive them both. There is still ongoing debate about which man produced the better vaccine. Oshinsky concludes that there is much about polio that remains a mystery. One of the ironies of the great polio crusade is….that the successful vaccines helped close the door to future research.
Published by Oxford University Press, 2005, Amazon price $US19.80
Living with Polio: The Epidemic and its Survivors by Daniel Wilson
The chapters in Wilson’s book cover the various stages of life with polio from the diagnosis (titled I’m afraid it’s polio), through life in hospital, going home, living with polio to An old foe returns: Post-polio syndrome. The story is developed from quotations from biographies written by over 150 polio survivors and these are of considerable interest. The experiences of these survivors resemble those of their Australian counterparts: long periods in hospital with limited visiting hours, staff coldness and at times cruelty. Wilson has used a somewhat biased sample of polios as he acknowledges. Survivors who write biographies are usually coping well with their disabilities. Wilson refers to a study of polio patients discharged from the Seattle Respiratory Center during the epidemics. About 10% died within two years. These patients didn’t necessarily have different physical problems…from those who ‘succeeded’, but rather seemed ‘to lack the initiative, drive, imagination and support systems to achieve what the more successful patients had’. In the conclusion Wilson writes: One didn’t so much overcome polio as learn to live with its legacies and compensate for its losses. Polio survivors have endured and have succeeded in many things…but no one who had paralytic polio truly overcame the disease. Wilson speaks of how polio survivors helped break down social barriers for people with disability and were among leaders in the disability rights movement. Reading this book will bring back many memories and give you new perspectives on some aspects of your polio past.
Published by Chicago University Press, 2005, Amazon price $US 19.14
Splendid Solution: Jonas Salk and the Conquest of Polio by Jeffrey Kluger
This book concentrates on Salk’s role in the conquest of polio. Kluger, a writer for Time magazine, has the journalist’s knack of telling a good story and he gives a lively account of the research and politics behind the development of the Salk vaccine. The book begins in early April 1954 when a rumour spread through the US that its government had stashed tens of thousands of little white coffins in depots around the country. The location of these coffins was never specified, and that was just as well. If you knew where the coffins were kept, you’d know where the government expected the people to start dying…Jonas Salk would be the person responsible for the deaths Journalist Walter Winchell, whose popularity was failing, was responsible for broadcasting and writing this story that the Salk vaccine could be a killer. In the storm of anxiety that followed, 150,000 individual children and some areas of the US that had been going to participate in the vaccine trials were withdrawn from the trials. Despite this setback the Salk vaccine trials commenced on April 26th, 1954 and were successful. The book presents a more generous and heroic portrait of Salk than does Oshinsky’s book.
Published by Putnam, 2005, Amazon price $US17.13
Polio and its Aftermath: The Paralysis of Culture by Marc Shell
This was this book I found most stimulating of the six because Shell goes beyond the medical history and the biographies of polio survivors and talks about some of the broader cultural and political implications of the epidemics and the drive to eliminate polio. Shell writes that: No study yet written attempts in any way to take in the whole world of the polio pandemics or allows us to see the universal in the particular. The effects of race, social class, religion, time have been ignored eg the different experiences of black and white Americans. He notes the placing of black and white children in the same polio wards in a few hospitals was an early landmark in racial integration. The polio epidemics coincided with the development of movies and the cold war and influenced attitudes toward these events. Shell’s own story is woven through the book, often in the form of imagined conversations with his parents. When he was a child they denied he had been left with any after effects of polio, never spoke of his polio, insisted he take part in activities that were dangerous because of his polio weaknesses and punished him by strapping his polio affected leg. The book is a fascinating meditative ramble about numerous ways in which polio left its mark. Shell discusses a relationship between polio and stuttering. He compares the approach of orthopaedic specialists who put polios on the rack of perfection with their surgeries (which seem to have been much commoner in the US than here) and physiatrists (rehabilitation specialists). The former seem to convey to polios the message, ‘This is not the way to be’ while the counterstatement from physiatrists is ‘Learn to be the way you are’. Ultimately Shell says, most people with long-term disabilities learn none too soon that there is a limit to what medical science can do to understand and treat us…so we are often, in the last analysis, probably less dependent on doctors for the way we ‘get along’ than we are on…architects, urban planners, and legislators—those who build the city and interpret its books of justice—and engineers, who make devices like telephones, stair-climbing wheelchairs, hearing aids, talking crosswalks, portable respirators, and voice recognition machines. There are many ideas to ponder in this book. This is not everyone’s book and some readers may agree with the reviewer in The Economist (16/6/05) that, Alas, his [Shell’s] own interesting story and that of other famous ‘polios’ are lost in a mess of literary analysis. The result is as wet and woolly as one of Sister Kenny’s treatments.
Published by Harvard University Press, 2005, Amazon price $US35.00
Home bound: Growing up with a disability in America by Cass Irvin
The author contracted polio, aged 9, in 1954. After six months in hospital Cass returned home to spend most of the rest of her childhood in bed. As a member of a dysfunctional family, it was just easier on my mom if I didn’t get in my wheelchair. Cass’s only times of liberation were periods spent receiving treatment at Warm Springs. As an adult Cass gradually took control of her life, moved out of home with attendant care and in the process came to see disability as a social and political issue. The only difference between Roosevelt and most disabled people was power and money. Our degree of living a ‘normal’ life has more to do with resources than with disability…Roosevelt’s message—that you cannot let them see how disabled you are, not if you want to succeed—is the message today’s ‘successful’ disabled person has internalized. This belief has kept most of us from joining activist groups to change society. The book is full of vivid, oh so true, examples of life with disability eg asking whether you can get into a venue. People usually do not know whether their buildings are accessible or not. And, even when they think they know [you] cannot rely on them. This time the school was ramped and he insisted I would have no trouble getting into the building. He forgot about the four inch curb before I ever got to the ramp. Or when Cass was looking for a job; finally a few encouraging words: ‘You know dear, I know if God wants you to have this job, He will provide you with a driver’. I wanted so badly to reply, ‘He told me to call you’ but I didn’t have the guts. Ironically most of what Cass learned about disability issues and the connections she made enabled her to organise good care for her father when he became disabled in old age.
Published by Temple University Press, 2004. Amazon price $US19.95.
The Broken Boy by Patrick Coburn
Patrick Cockburn contracted polio during the Cork epidemic in 1956. Peter Preston who reviewed the book in the Guardian (12/6/05) wrote: Patrick is six, a cheerful cosseted lad….Suddenly he has a fever. Suddenly his whole life turns on its head. He is alone, utterly alone…He’ll be a cripple forever…He remembers the thin grey, hospital slop of minced meat and potato; the fear of tyrant nurses snapping his head off.., the ward tours by lordly consultants brooking no questions nor arguments. It sounds like Children’s Hospital. I remember that when I was at the Collaroy annexe and finally got callipers, one of my first walking activities was to wrap up my bed-bound friends’ and my mince and slop, walk to the end of the grounds and throw it over the fence where fortuitously there was a public litter bin. Very few polio memoirs express the anger that Cockburn does. I suspect many of us buried our anger deep because to show it was so dangerous. Cockburn is angry with his parents for moving the family back to Ireland when there was an epidemic and at the way the authorities handled the epidemic. The book also recounts the goings on of his Anglo Irish ancestors and his research into the epidemic but we are told almost nothing of his own life as a polio survivor once he returned to school. Cockburn says he carries a lot of emotional scar tissue from polio. But I also thought—probably rightly– there was not much I could do about it. He mentions having a severe limp and worrying about post-polio syndrome. From the cover and book’s acknowledgements I learnt he is a foreign correspondent in the UK and is married. Cockburn’s final words are: Polio inflicted and continues to inflict great suffering among its surviving victims. Very occasionally well-meaning people suggested to me as a child that sufferings built character and endurance. Even at the age of seven or eight I suspected I had acquired those supposed benefits at an excessive price.
Published by Jonathon Cape, 2005, £11.19 at Amazon.co.uk
12/2/2004
Post-Polio Syndrome: A Guide for Polio Survivors and their Families by Julie Silver MD, director of the International Center for Polio in Framingham, Massachusetts was recently published by Yale University Press. Dr Silver’s mother, brother and grandfather all contracted polio so the disease has always been part of her life. Her book comes three years after Lauro Halstead’s Managing Post-Polio: A Guide to Living Well with Post-Polio Syndrome, which has been available to Network members for several years at a special rate. Inevitably the books will be compared. Silver’s book of 280 pages is 40 pages longer than Halstead’s and has 26 chapters compared to Halstead’s 13. In Silver’s book you can find specific chapters on virtually any post-polio relevant topic eg swallowing issues, nutrition and weight, respiratory problems, prevailing over pain, sex and intimacy, preventing falls, controlling cold intolerance, EMGs and bracing, shoes and assistive devices. It’s harder to find relevant material in Halstead’s book which lacks the excellent cross-referencing between chapters that Silver employs. If you want a practical, clear instructional guide you may prefer Silver. She gives definite doctor’s orders on dealing with post-polio symptoms but also regularly instructs readers to seek their doctors’ advice. If only there were Dr Silver clones around Australia that polio survivors could easily consult! If you prefer a more in depth discursive style which gives greater emphasis to the physiological causes and psychosocial aspects of post-polio then Halstead is your book.
Silver highlights many important practical issues that rarely receive attention. In the chapter Preserving and Protecting Your Arms Silver argues that your arms are your key to independence. Think about it. If you cannot use your legs at all, you can still remain totally independent—living alone, bathing yourself, feeding yourself, driving a car. But if you cannot use your arms at all, you immediately cease to be independent and must rely on others to help you with the most routine (and intimate) activities of daily living. She outlines three major causes of arm problems. Firstly, there is post-polio muscle pain, an aching, cramping, burning or tired feeling in the muscles rather than the joints. It often occurs at night or after activity. It indicates overuse and arms should be rested as much as possible. Secondly, there are soft tissue injuries such as muscle and ligament strains, tendonitis and bursitis. Often these injuries occur in the arms from repetitive activities such as using a computer, chopping vegetables, knitting…In polio survivors these types of soft-tissue injury occur frequently and often without an obvious reason. This is because many polio survivors have some upper body weakness (which may be subtle) that makes their arms more susceptible to injury. Also, polio survivors who have decreased lower-body strength tend to rely on their arms to assist them with mobility (as in getting up from a chair). She urges medical, not self, diagnosis and treatment to avoid further and permanent weakness. Treatment may include avoidance of activities that exacerbate symptoms, ice, heat, splints, physiotherapy, occupational therapy, injections, medication and very occasionally surgery. Thirdly, there is biomechanical pain that usually presents as joint or neck pain. Silver says that such biomechanical pain is often attributed to arthritis and thought to be untreatable. Though arthritis may be present it may not be the major source of the pain. She has found that polio survivors frequently develop neck pain and headaches at the back of their heads due partly to poor posture and muscular strain. Initial treatment may include improving one’s sitting posture and avoiding neck strain, physical therapy, massage, heat oral medications, and topical creams. If these treatments fail, injections may be useful.
The chapter Keeping Bones Healthy and Strong differentiates generalised and localised osteoporosis in polio survivors. The latter often occurs in limbs that are paralysed or very weak. Such limbs are particularly vulnerable to fractures. Some survivors, like the ablebodied population, may develop generalised osteoporosis, which affects bones throughout the skeleton. Silver has little advice on localised osteoporosis (probably because it is not well understood) but she discusses what can be done about generalised osteoporosis. She emphasises the importance of early diagnosis. In Australia this is often done by bone density scans of the spine and one hip. However a polio survivor needs to explain to the doctor about polio related localised osteoporosis or an inaccurate assessment may result. In my case I insisted that both hips be scanned. The hip of my weaker leg is very osteoporotic but the hip of the other leg and spine have better than average bone density.
Silver presents a practical 10-step plan for energy conservation and pacing. She quotes polio survivor Hugh Gallagher: Growing old with polio is a matter of economics: cost/benefit analysis. How much expenditure of limited energy for how much satisfaction. Minimize the exertion; maximize the pleasure. Silver suggests you start by keeping a log of activities for three days. Also document episodes of pain and fatigue, the time they occur and your activities around this time. Highlight low, moderate and high energy activities in different colours. Consider how high energy activities can be modified to become lower energy tasks. Lots of examples are given. Silver cites Josephine, a polio survivor, who is known to her family and friends as the One-a-Day Girl because she will only schedule one major outing each day. When friend contact her they ask, Is your One-a-Day booked on such-and-such a day?
In the chapter on exercise considerable emphasis is given to flexibility exercises. These are a critical but often neglected part of any exercise program. They can dramatically increase range of motion, and reduce pain and degree of disability. Again diagrammatic examples are given. Silver talks about reserve strength, which is something we count on to sustain us as we age. In polio survivors, it is often markedly diminished and contributes to increasing disability that may present without much warning. An example of reserve strength is given. A certain threshold of strength is needed to do any activity. Imagine that it takes 30 percent of your total arm strength to lift a gallon of milk. This means that 30 percent is the threshold of strength your arm needs to lift the milk: if your strength falls below that mark you are unable to lift the milk. If you had polio and lost 50 percent of your strength, this is still an easy task to accomplish. …you have 20 percent in reserve. But suppose through normal aging, disuse, overuse, and perhaps some other factors, you lose 1 to 2 percent of your arm strength each year…when the amount of strength you have lost starts to get close to 20 percent, you are likely to notice that lifting a gallon of milk is becoming more difficult…and if the next year you drop to 29 percent, you will go from being able to lift the milk to not being able to lift it. Silver calls this the all-of-a-sudden phenomenon because polio patients often report such losses. The original bout with polio reduced our reserve strength and this contributes to the accelerated (compared to our ablebodied counterparts) loss of strength we experience as we age.
Some noteworthy comments from the book include: “A syndrome is a collection of symptoms that characteristically occur together…Because syndromes do not have specific tests that can unquestionably identify them, they are subject to interpretation. Often their validity is challenged within the medical community. PPS is no exception…Generally it is inexperienced health-care providers, unfamiliar with treating polio survivors, who dismiss the syndrome. Those of us who routinely participate in the care of polio survivors have no doubt that PPS is real”. “Polio truly is a chronic illness that lasts a lifetime”. “It is important to recognize that some people may not have a classic history of polio but may indeed have had the disease. Moreover some of these individuals may be experiencing PPS”.
The book is available from Internet bookshops barnesandnoble.com and amazon.com for
$US22 plus handling.
19/11/2003
STOP PRESS 2
We have just received the following excellent news from our Polio Network Europe colleagues. Long term members will recall our role in establishing the PNE email discussion group, and we are most grateful to Chris Salter of Lincolnshire Post Polio Network for his sterling work in running the vital Internet sytem that enables PNE to work. Chris has sent us the following email reports from Johan Bijttebier, representing Belgian Polio survivors, who has been a key figure in obtaining the necessary support from Belgium`s Health authorities.
If you do not have time to read all this soon – please note we hope a PPUK member will attend the November 12th meeting in Brussels for us, travel expenses paid. Please contact Helena on 0116 2209188 a.s.a.p. if you can!
From: Johan Bijttebier
Post Polio Syndrome
During both the 2384th meeting of the Council of the European Union
(Health - 15th of November 2001 - Brussels) and the 2470th Council Meeting
(Employment, Social Policy, Health and Consumer Affairs - 2nd & 3rd of
December 2002 - Brussels) the Council took note of statements made by the
Belgian presidency and the Irish delegation on the need for action at
Union level to provide an adequate response to Post Polio Syndrome
(PPS) sufferers.
The questions were
• Is the Council able to tell us what kind of action already has been
taken in this matter, more specifically on the level of:
1. Information about PPS to health professionals and polio survivors;
2. Treatment for polio survivors to prevent further disability;
3. Disability benefits to polio survivors who can no longer work?
• If the Council hasn’t undertaken any action: what are the reasons for
not doing so?
A follow up meeting was held this September.
MEETING POST-POLIO STRASBOURG - SEPTEMBER 2nd 2003
Meeting held room N11 at 11am
MEP’s in attendance : Bart Staes (B - Greens)
Kathleen Van Brempt (B - PES)
Philip Whitehead (UK-PES)
Max van den Berg (NL-PES)
Chris Heaton-Harris (UK-EVP)
Catherine Stihler (UK-PES)
Other MEP’s pledging support
Marie-Helena Gillig (FR-PES), Brian Crowley (IRL-UEN), Michael Cashman (UK-PES), Mel Read (UK-PES), Nick Clegg (UK-ELD), David Martin (UK-PES), Neena Gill (UK-PES), Glyn Ford (UK-PES), Dieter L. Kock (D-EVP), Richard Howitt (UK-PES), Sarah Ludford (UK-ELD), J.J. McCartin (IRL-EVP), Gerard Collins (UK-UEN)
Presentations by Post-Polio patient group representatives, Anita Molines - France
Johan Bijttebier – Belgium, Peter Jay - United Kingdom
Aims of the meeting
- To create a vehicle for European post-polio organisations to get
together and address the challenges and problems that are faced by polio
survivors on a daily basis, throughout Europe.
- To improve the quality of life for the European polio survivors who
are experiencing new health problems associated with their old polio.
- To raise awareness of the condition, Post-Polio Syndrome (PPS), among
European healthcare professionals and ensure that PPS is a health
priority.
- To implement an action plan for the Commission and to launch
post-polio as an issue in European health policy.
- To plan a press conference in Brussels explaining this initiative and
raise the level of support for people who have had polio. (12 november!)
Summary of the meeting- English version by Peter Jay (edited for length by PPUK)
It was recognised that volunteers (in Europe) have done much of the work
on behalf of polio survivors. Volunteers who live with post-polio
themselves have raised awareness, promoted education and advocacy among
individuals and health care professionals. In the main, it is volunteers
who are battling for the millions of polio survivors worldwide. A huge
number of self-help support groups have been created in many countries.
These groups offer vital support to polio survivors, their friends and
families. These groups also act as a conduit to health care
professionals. Volunteers who understand the needs of polio survivors
take the practical approach when it comes to dealing with post-polio -
championing self-management and self-help by role-modelling and
motivating others.The work done by volunteers is enhanced and legitimised by credible and
well facilitated information research.
A strong case was made by the group representatives that an integrative
medical approach was essential when treating the Post-Polio Syndrome
(PPS). What’s needed are clinics where every aspect of the condition is
treated using a blend of traditional medicine with complementary healing
methods. By using specific criteria developed by the International March
of Dimes it is possible to diagnose and confirm PPS.
As part of any innovative programme a comprehensive evaluation would
include, detailed clinical history, physical examination, lab tests
including nerve conducting studies, high-resolution MRI, pulmonary
function tests and sleep studies. Following a diagnosis the treatment
options would include physio and/or occupational therapy, pain control, orthotics, nutritional advice, massage therapy, stress management,
relaxation and yoga exercises, acupressure, aromatherapy and chronic
disease self-managemen. The multi-disciplinary team working with
post-polio patients should include a; rehabilitation specialist,
neurologist, registered nurses, psychologist, pulmonary specialist,
nutritionist, stress management experts, physio, occupational,
respiratory and speech therapist.
To improve on the fragmented and post-code lottery treatment available
to post-polio patients at present will require dedication and
commitment. The co-operation and willingness to work together between
the MEP’s and polio representatives demonstrated at this meeting was
encouraging.
For additional information and examples of best practise I acknowledge
and thank the following individuals, organisations and publications;
Lauro S. Halstead, M.D. - Managing Post-Polio, The British Polio
Fellowship, Joan L. Headley Executive Director - Post- Polio Health
International and the Post-Polio Programme at Conemaush Memorial Medical
Centre, USA
What is the Post-Polio Syndrome (PPS)
Thankfully the incidence of polio in Europe is very rare due to high
levels of immunisation. In fact, last June Europe was ofïicially
declared to have eradicated polio by the World Health Organisation.
A few months previous, Europe passed the three year milestone without an
indigenous case. The last child infected in Europe was Melik Minas, aged
two-years. Melik, who lived in south-eastem Turkey had never been
vaccinated, and was paralysed in November 1998.
However, those people who survived the huge epidemics during the last
century are still around. In the 1950’s alone there were 45,000 cases in
the UK and hundreds died. It is estimated that there are many tens of
thousands of people alive today in the UK alone who are experiencing new
symptoms for which they need to seek help.
Worldwide, that figure increases to around 20 million.
The other side of the coin is that there are very few health
professionals practising today who have seen acute poliomyelitis or
Infantile Paralysis as it was formally known.
Therefore, it is vitally important to raise awareness at every
opportunity. We are very grateful to these European Parliamentarians who
have joined together to ensure that these new symptoms are accurately
diagnosed and effectively treated by those responsible for health within
the European Union.
Many polio survivors have leamed about their condition and have accepted
a certain amount of responsibility for their own health. They try to
self-manage their long-term chronic condition. Like many other patients
who live with long-term illnesses, self-management does not mean going
it alone - we need help and support from health professionals and other
support agencies to reduce the impact of further disablement.
For many of us who survived polio we were encouraged to get on with our
lives as soon as possible, as if nothing ever happened. Some were able
to recover completely from the experience, and went on to lead fully
independent lives. Others needed help for the residual effects, such as
crutches or callipers, and some were left completely dependent, in
wheelchairs and occasionally, full time mechanical ventilation.
Today, physiotherapists in orthopaedic, neurology, orthotic, respiratory
and rehabilitation settings work with many of these former polio
patients. During the past few years, they have become aware that polio
survivors - whatever their original level of recovery - are now facing
new and unexpected problems.
They often describe people who had polio as polio survivors. Possibly
because we survived isolation and iron lungs, harsh treatment regimes
and the stigma of quarantine. And that’s if we were lucky. Roughly 12%
of those who developed paralytic poliomyelitis in the UK died from
breathing and swallowing complications.
Until fairly recently, polio has been divided into three distinct
stages.
Stage I: the acute illness
The initial symptoms are similar to many other viral illnesses, often a
fever, headache, sore throat, diarrhoea or vomiting and malaise. In most
individuals these symptoms have gone within two or three days.
Unfortunately, in some people the symptoms are more severe, reflecting a
viral invasion of the central nervous system. These symptoms include
high fever, stiffneck, and severe headache and muscle pains. The
infection continues to spread, producing variable amounts of muscle
paralysis or weakness in the limbs, trunk, even the face and neck.
Stage II: recovery and treatment
This stage can last from weeks to years, depending on the severity of
involvement and age of onset. Persons who contract polio as children or
infants and have extensive paralysis take the longest time to recover.
During this period, individuals usually begin an intensive programme of
rehabilitation in hospital or at home, with the goal of strengthening and retraining weakened muscles and leaming to regain lost function.
Stage III: maximum recovery period
This stage begins when a person reaches a plateau of maximum strength
and stamina. The precise time when this stage starts is hard to
determine, especially if the individual is still growing or is
undergoing reconstructive surgery to enhance strength and function. In
spite of these difficulties, most people have a general idea when their
recovery was complete. Often this recovery was impressive and we walked
unaided for many decades. This third stage of polio may last
indefinitely for many individuals who had paralytic polio.
A fourth stage
However, in 1977 an article was published by the Rehabilitation Gazette
on the problems that people disabled by polio were experiencing, often
many years after contracting the virus. This article received
international attention. Consequently, a fourth stage was recognized.
This fourth stage has been described by various terms.
For today’s purposes I will use the term ‘post-polio syndrome’ or PPS as
it more broadly defines the cluster of symptoms that many polio affected
people are suffering.
PPS begins with the onset of new weakness, which is often accompanied by
other symptoms, such as fatigue, pain in muscles and joints, and
decreased function. This new weakness often affects muscles in arms and
legs, but in some people, less common but more seriously, breathing and
swallowing muscles are affected.
PPS occurs an average of 30 years after the onset of the initial polio
infection and after leading active and independent lives. It is clear
that for many of us, the battle against polio is far from over.
A new nerve weakness
Although this is described as new muscle weakness, it is new nerve
weakness.
When a person contracts polio as an infant, a degree of recovery may
take place. The extent of this recovery is determined by three major
factors: (l) the number of motor neurons that recover and resume their
normal function, (2) the number of motor neurons that develop terminal
axon sprouts to reinnervate muscle fibres left orphaned by the death of
their original motor neurons, and (3) the phenomenon of uninvolved or a
recovered motor neuron to “adopt” those orphaned muscle fibres.
Unfortunately, these nerves have a reduced life, around twenty to thirty
years, then they begin to die back and new symptoms develop.
For many years’ polio survivors have been working with a damaged
neuromuscular system that keeps working harder than ever to meet normal
demands. We need to appreciate the fact that each anterior horn cell
innervates several hundred muscle fibres and the surviving horn cells
have been doing 50% more work than normal. Plus it appears they don’t
like being over-worked. Because of this overuse of the neuromuscular
system, new weakness is now developing.
Survivors may develop new weakness not only in polio muscles but also in
other muscles that did not seem to be involved during the acute phase -
stage one.
Millions of polio survivors worldwide face the same problems caused by
new weakness. After many years of use, muscles and joints seem to be
screaming out “I’ve had enough, I need relief, I need some rest!". What
drives many post-polio patients to the GP, consultant or rehabilitation
specialist and eventually to the orthotist or physiotherapist is pain.
This pain should indicate how he or she can be protected from further
damage and if possible maintain mobility. Healthcare professionals must
be careful not to prejudge us post-polio patients. It is essential that
they take the time to listen and properly evaluate our condition. They
need to know what they are dealing with and become familiar with the
polio patient’s history. We are the type of patient that practitioners
need to evaluate hands-on and to know “first hand” the muscle weakness
and range of motion and how the post-polio patient is substituting or
use body tricks to enable function. The challenge for health care
professionals is accurate diagnoses and effective treatment.
INVITATION FOR A EUROPEAN PPS-DAY
12th of November - European Parliament - Brussels
Dear friends,
I’m honoured to invite you to a European Post Polio Syndrome (PPS) day
in Brussels. In the morning, European PPS-patients and Members of the
European Parliament (MEPs) will hold a press conference together. In the
afternoon, we will be able to meet under less formal circumstances to
plan our strategy concerning the foundation of a European network of
PPS-patients, which will start a widespread information campaign on this
issue.
On September 2nd, 6 MEPs met 3 representatives from European Post-Polio
Patients-organisations. The three PPS-patients were Peter Jay (United
Kingdom), Anita Molines (France) and myself.
We received warm support from the MEPs. Not only the ones participating
in the meeting, but also 13 others who already support the foundation of
a European organisation of patients suffering from PPS and the launch of
a campaign to raise public and medical awareness of this syndrome. The
supporting MEPs already constitute 6 different European countries and
almost all major political groups.
As this first meeting was a success, we’ve planned a second one to be
held on the 12th of November. This is not only to meet each other on a
European level, but also to hold a press conference during which we’ll
ask the European Community to look for ways to subsidise any kind of
project that enables - on a European level - PPS scientific research and
an information campaign. We hope not only to inform European citizens of
the symptoms, diagnosis and treatment of PPS, but also health
care-personnel.
You’ll have the opportunity to speak with some members of the European
Parliament and with the international press, if not during the press
conference, than at least afterwards.
By Spring 2004 at the latest, we will submit a proposal to get
subventions for this European campaign on PPS in order to get a decision
from the European Commission by Winter 2004.
It would be an honour to me if your country/organisation could send a
(few) representative(s) to this PPS-day in the European Parliament in
Brussels.
If you’re willing to join this meeting, all you have to do is confirm
your presence to: johan.bijttebier@pandora.be
ATTENTION PPUK MEMBERS : TRAVEL EXPENSES PAID - ANY VOLUNTEERS? CONTACT 0116 2209188
PROGRAMME FOR THE DAY
10.30 AM Press Conference (room to be confirmed)
11.00 AM Opportunity to meet MEPs
12.00 AM Lunch in the EP (Personal costs: maximum 10 euro pp.)
2 PM - 5 PM Meeting European PPS-patients (room PHS 1C47)
Participation at this meeting is free. We’re looking for ways to keep
the costs of transport, accommodation and lunch as low as possible. Any
suggestions on this matter are welcome.
If you know of any press contacts in your own country, don’t hesitate to
inform them about this event!!!
On this url you’ll find a map of the European Parliament:
http://www.europarl.eu.int/abc/visit/brux/orientation.htm - Entrance D
By train: (1) Brussels-North or Brussels South (2) Brussels Luxemburg
By car: (1) Rue Belliard (2) Parking at your left hand side just before entering the tunnel to Liège Johan Bijttebier, Post Polio Belgium
Chris Salter polio-network-europe@lincolnshirepostpolio.org.uk
Lincolnshire Post-Polio Network (UK) Registered Charity No. 1064177
URL:http://www.lincolnshirepostpolio.org.uk/>
6/8/2001
Guidelines
for people who have had polio
BASED ON MARCH OF DIMES
INTERNATIONAL CONFERENCE ON
Post-Polio Syndrome
2001
Suggested Reading:
Halstead, L.S. (ed.). Managing Post-Polio: A Guide
to Living Well with Post-Polio Syndrome.
Washington, DC: NRH Press, 1998.
Jubelt, B., Agre, J.C. Characteristics and management
of postpolio syndrome. Journal of the
American Medical Association, 2000, volume 284,
pages 412-414.
Maynard F.M., Headley, J.L. (eds.). Handbook
on the Late Effects of Poliomyelitis. St. Louis, Mo.:
Gazette International Networking Institute, 1999.
Post Polio Information Package for Health Care
Professionals. Toronto: Ontario March of Dimes,
2000.
Post-Polio Syndrome: Identifying Best Practices in
Diagnosis and Care. White Plains, N.Y.: March of
Dimes, 2001. [This report was the basis for the
guidelines in this brochure and derives from a May
2000 International Conference on PPS, sponsored by
the U.S. March of Dimes in collaboration with the
Roosevelt Warm Springs Institute for Rehabilitation.
The report and this brochure are available on-line at
www.modimes.org/Programs2/International.]
Steering Committee
Lewis P. Rowland, MD, (Chair), Department of
Neurology, Columbia University College of
Physicians and Surgeons, New York, N.Y.
John R. Bach, MD, University of Medicine and
Dentistry of New Jersey, New Jersey Medical
School, Newark, N.J.
Kristian Borg, MD, PhD, Head, Huddinge
University Hospital, Stockholm, Sweden
Neil R. Cashman, MD, Center for Research in
Neuro-Degenerative Diseases, University of Toronto,
Ontario, Canada
Marinos C. Dalakas, MD, Neuromuscular Disease
Section, National Institute of Neurological Disorders
& Stroke, Bethesda, Md.
Elizabeth Dean, PhD, PT, School of Rehabilitation
Sciences, University of British Columbia, Vancouver,
BC, Canada
Lauro S. Halstead, MD, Post-Polio Program,
National Rehabilitation Hospital, Washington, DC
Joan L. Headley, MS, International Polio Network,
Saint Louis, Mo.
T. Jacob John, MD, Department of Clinical
Virology, Christian Medical College, Vellore, Tamil
Nadu, India
Richard B. Johnston, Jr., MD, Department of
Pediatrics, National Jewish Medical and Research
Center, Denver, Colo.
Rhoda Olkin, PhD, Department of Clinical
Psychology, California School of Professional
Psychology, Alameda, Calif.
Susan L. Perlman, MD, Department of Neurology,
University of California at Los Angeles, Los Angeles, Calif.
Daria A. Trojan, MD, MSc, McGill University,
Montreal Neurological Hospital, Quebec, Canada
Barbara G. Vickrey, MD, MPH, Department of
Neurology, University of California at Los Angeles,
Los Angeles, Calif.
William W. Wendling, Cleveland Municipal School
District, Cleveland, Ohio
Acknowledgment: We thank Sandra Donahue,
Science Writer, March of Dimes, for condensing
Post-Polio Syndrome: Identifying Best Practices in
Diagnosis and Care.
Questions?
Call: 888-MODIMES
Visit: www.modimes.org
To order additional copies of this pamphlet:
Call: 1-800-367-6630
Or write:
March of Dimes
P.O. Box 1657
Wilkes-Barre, Pa. 18703
31-1523-01 3/01
© The March of Dimes Birth Defects Foundation 2001
Guidelines for People who have had Polio Post-Polio syndrome (PPS) is a disorder of the neuromuscular system that affects many people who had poliomyelitis many years earlier. However, most people who had polio do not develop PPS. PPS usually develops 15 or more years after an acute polio virus infection that resulted in some degree of temporary or permanent paralysis. The new problems arise after many years of stable muscle function. All told, as many as 250,000 U.S. polio survivors may have PPS.
What are the symptoms of PPS?
The main symptom of PPS is new muscle weakness
that gradually worsens. It is often accompanied by
decreased muscle endurance during activities, muscle
and joint pain, muscle wasting and severe fatigue.
These problems usually come on gradually, but may
start suddenly if you have undergone surgery, or
been hospitalized or confined to bed. Even muscles
that were believed to be unaffected by the previous
bout with polio may be affected. Less frequently,
breathing difficulties and swallowing problems may
occur. If these problems persist for at least one year,
you may have PPS.
However, even if you have one or more of these
symptoms, you may not have PPS. A complete medical
evaluation may show that you have arthritis,
tendonitis or cartilage damage, all of which can occur
in the aging process, but are more common when movement
and weight-bearing have been altered by the
effects of weakened muscles. Various other conditions
also cause progressive muscle weakness or fatigue.
If you think you might have PPS, you should
consult your physician, who may refer you to a
neurologist or other specialist, preferably one having
experience with PPS. In addition to the neurological
examination, doctors often recommend tests, such as
imaging studies (computerized tomography and
magnetic resonance imaging), to rule out other
disorders with similar symptoms; however, there
is no definite test for PPS. Continuing care, if
indicated, is usually supervised by a neurologist or
specialist in rehabilitative medicine.
interspersing bouts of physical activity with
rest periods. Some individuals may benefit from
weight loss. Assistive devices, such as braces and
intermittent use of wheel chairs or motorized
scooters for long distances also can help conserve
energy. These lifestyle changes may also help ease
muscle and joint pain, as can use of moist heat,
ice, massage, and when necessary, medications.
Individuals with breathing problems may
benefit from nighttime breathing assistance to
help improve sleep and energy levels. Instruction
on special swallowing techniques and diet alterations
can make eating easier and safer for those
with swallowing problems.
What research is needed on PPS?
An international steering committee made up
of leading PPS researchers, convened by the
March of Dimes, recommends additional
research on the causes, symptoms, prevalence,
diagnosis and treatment of PPS—with the goals
of improving diagnosis and treatment of the
disorder. For example, studies that provide
more information on the typical course of symptoms
in PPS should help distinguish the disorder
from other conditions that may have similar
symptoms, and thereby improve diagnosis of
PPS. A thorough evaluation of current m | | |
