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Polio Survivors Abroad Canadian Roulette, Rue Britannia

Polio Survivors Abroad: Canadian Roulette, Rue Britannia Dr. Richard Louis Bruno

Lincolnshire Post-Polio Library copy by arrangement with the Harvest Center Library

Over the past few years much heat has been generated by suggested solutions to the difficult problem of reforming the American health care system. A solution promoted by the Clintons’, but despised by their free-market Republican opponents, is a single-payer health care system very much like the ones in Canada and Great Britain. Such systems, where treatment is paid for by the government, provide medical care to all citizens regardless of their ability to pay.

Or do they?

On recent trips to Canada and England, I had the opportunity to view the workings of two socialized medical systems from the point of view of one disability group in desperate need of rehabilitation services: the survivors of the polio epidemics of 40 years ago. What I discovered made me question whether socialized medicine is really too unsocialized to help people with disabilities.

Northern Exposure:

In Canada medical care is available to everyone without charge. So I didn’t anticipate the problems Canadian polio survivors have in obtaining treatment for their Post-Polio Sequelae (PPS), the new and sometimes disabling fatigue, muscle weakness and pain that occur about 40 years after polio.

One problem confirmed the warning I had heard so often during the our own health care reform debate: Canadians can pick their own general practitioner (GP), but cannot chose their own specialists such as physiatrists and orthopedists. The matter of choice may not be an issue when you have a common problem like a broken leg. But health care can become virtually unavailable when you have a newly discovered condition like PPS that most physicians don’t know how to treat.

“Many GPs have never heard of PPS or do not believe PPS is real,” explains Judith Lytle, post-polio services coordinator for Ontario March of Dimes. “So polio survivors are forced to play ‘doctor roulette,’ going from GP to GP until they find one who is informed about PPS and willing to refer to a specialist.”

“Many specialists are also uninformed about PPS,” warns polio survivor Jeannette Shannon, president of Ontario March of Dimes. “So, referral does not ensure treatment.”

Nearly a dozen polio survivors told me similar stories of diagnosis deferred and treatment ultimately denied: Through trial and error a polio survivor finds a GP who has heard of PPS, but knows nothing about diagnosis. She is referred to an orthopedist, but it takes six months to get an appointment. She sees the orthopedist, who believes the condition is real, but is ignorant of the treatment. She is then referred to a neurologist. It then takes six more months to see the neurologist, who does not believe in the reality of PPS, and who sends her back to the GP with the diagnosis “Nothing physically wrong.” It took polio survivors about a year to complete this circuit of doctors and still receive no treatment.

“Our goal is to stop ‘doctor roulette’ by creating PPS support groups throughout Canada that will educate polio survivors, their GPs and specialists about the cause and treatment of PPS,” said Lytle. Until this education process is completed, too many Canadian polio survivors will remain stuck suck on this medical carousel, feeling frustrated and desperate as they continue to lose function.

Yet with the help of an enlightened GP, polio survivors in some parts of Canada can receive outstanding care for PPS. Quebec’s polio survivors can be treated at the Montreal Neurological Institute (MNI), one of the world’s preeminent PPS research and treatment centers. Unfortunately, even MNI is in jeopardy because the Canadian health care system has another problem: It’s broke. There are too many people in need of health care and not enough tax revenue to pay for it.

“Deep federal budget cuts last year caused the MNI to eliminate the therapist in the PPS clinic,” says Sally Aitken, president of Polio Quebec Association. “The PPS clinic’s director was forced to send letters to his patients asking them to mail in contributions to pay the therapist’s salary,” says Aitken. “Thanks to the members of Polio Quebec, the PPS Clinic is open–for the moment.”

The Canadian national health care system hemorrhaging green, and polio survivors are not the only ones in jeopardy. The government has decided to stop paying for surgery to unblock coronary arteries in heart attack survivors disabled by chest pain because such surgery is no longer “worth the additional cost to society.” The result? Thousands of Canadians will be left in pain, non-functional and unemployable. Said Canadian Medical Association president Jack Armstrong, the health care system is becoming a “hollow shell of its former self.”

Cutting treatment to trim the budget is a terrifying aspect of government-run health care. A federal committee can just decide that rehabilitation for a disabling condition — or even curing that condition — is simply not “worth the additional cost to society.” It’s the usual short-term thinking. Long term, thousands of citizens will remain disabled, unemployed and consigned to a life at home waiting for their disability check — until there is no money left in the disability budget, either.

Pass the Parcel:

Britain, I imagined, would be more hospitable to polio survivors. A national organization for survivors — the British Polio Fellowship — has existed for more than 40 years, and 1996 was publicized by the BPF as “Polio Awareness Year.” But the U.K. is even less hospitable to polio survivors than is Canada.

“PPS is a well kept secret in Britain,” says Leicestershire polio survivor Helena Edwards. “And the BPF has taken an ultra-conservative position.” In 1995, the BPF distributed a questionnaire to all of its members asking about PPS symptoms. “When 77 percent reported new physical deterioration,” Edwards says, “the BPF was frightened off.” The BPF declared that the questionnaire was “too long” and had it’s

name removed from the report, this even after the findings were published in the BPF “Bulletin.”

Polio survivors and even many BPF members disagree with the organization’s head-in-the-sand position. As the only organization for polio survivors, the BPF is ideally positioned to educate Britain about PPS and advocate for treatment. Sadly, it has preferred to continue its traditional role of organizing sporting and social events. It is also sad that the British Government views the BPF as the representative of all polio survivors — even though only 7 percent of Britain’s polio survivors are members — and has adopted its inclination to ignore PPS. “I suspect the Government’s ignoring PPS has more to do with the British disease of ‘old-boy networks’ than intentional cruelty,” says Edwards. “Nevertheless, we are being hurt by their neglect.”

One particularly pernicious example of neglect is the decades-long relationship between the BPF and London’s government-funded St. Thomas Hospital. “Whenever we ask the BPF or the government for treatment we are told to go to St. Thomas, a centre for treating respiratory polio in the 1950s,” says Edwards, who did go all the way to London for an evaluation. “The doctor’s only suggestion was that I should have respiratory testing,” Edwards reports. “Nothing was said about my progressive leg weakness and loss of mobility, let alone the possibility of therapy.” To date, Edwards has had no treatment for any of her advancing symptoms.

That a St. Thomas doctor should identify only respiratory problems may be explained in part by the hospital’s own survey of 208 respiratory polio patients of 40 years ago. Seventy-eight percent reported new “progressive functional deterioration” that was deemed to be caused by “respiratory factors.” And although 22 percent were found to have “late functional deterioration” unrelated to breathing problems, new symptoms were always attributable to a clearly defined cause such as rotator cuff tears, arthritis or carpal tunnel syndrome. Although the survey made no mention of late-onset fatigue and muscle weakness, the St. Thomas doctors concluded that not one of their patients had PPS.

St. Thomas’ findings are in stark contrast to the survey of 1,200 BPF members conducted by polio survivor Peter Field. Sixty-six percent reported late-onset fatigue, 57 percent reported muscle weakness and only 16 percent reported new respiratory problems.

But St. Thomas’ position on PPS came as no surprise to those who know the hospital. After all, it originally treated survivors of respiratory polio, who should be reporting more breathing problems than the general post-polio population. And while patients describe St. Thomas’s chief physician as “a lovely kind man,” they say his focus on respiratory problems has caused him to be “unable to see the wood for the trees.

“But even if St. Thomas’ doctor did see “the wood,” and provided comprehensive treatment for PPS, a single hospital in London could not treat all 50,000 polio survivors in England, Scotland and Wales. So in 1994, polio survivor Fred Griffin wrote to local newspapers in the British Midlands describing the neglect polio survivors were experiencing at the hands of the BPF, the government and St. Thomas. These letters encouraged Helena Edwards and Internet maven Jim McVay to join Griffin in forming the Leicestershire Polio Network to lobby county health authorities for comprehensive PPS treatment by local doctors. “Why shouldn’t we have treatment locally,” asks McVay? “The government’s own tallies estimate that there are 1,600 polio survivors in this county alone.”

The Leicestershire Polio Network conducted its own survey of local polio survivors and found “a complete lack of diagnosis of PPS, much misdiagnosis, and the widespread prescription of inappropriate drugs and use-it-or-lose-it physical therapies,” says McVay. British polio survivors were also caught in their own version of “doctor roulette,” what Edwards calls the “pass-the-parcel game, with the patient

being the parcel.”

Hilary Hallam, secretary of another new polio network in Lincolnshire County, was one of those unfortunate “parcels.” A former police officer, lifeguard and occupational therapy aide working with people with disabilities, Hallam developed severe leg pain and progressive leg weakness, then fatigue, pain and weakness in her arms, that forced her to stop working. It took 14 months for her to circulate from GP to orthopedist to neurologist, in the end receiving a tentative diagnosis of nerve root damage and a referral back to her GP. “The neurologist said that I do not have PPS — as it doesn’t exist — and that there is nothing he can do,” says Hallam. In an attempt to end the parcel passing, Edwards wrote to her county’s director of public health about the failure of local neurologists to identify, diagnose and treat PPS. Health department officials spoke to local neurologists, and replied that the doctors “see only three or four new cases each year of polio patients who develop a form of motor neurone disease. No treatments,” it concluded, “are indicated.” What’s more, the officials were puzzled by the Network’s complaints: “You have acknowledged that the service available in Leicestershire does not warrant particular criticism in comparison with others parts of the country.”

Edwards was incredulous. “We complain that local neurologists do not recognize PPS,” she says. “The health department asks the neurologists if they see cases of PPS and their answer is ‘No.’ So the health department concludes there is no PPS. We’re in a Catch 22.”

As for local services not warranting criticism in comparison to the rest of England, Edwards replies, “That’s like saying that because there is no food anywhere in the country, no one should feel hungry. Leicestershire is no different from the rest of the U.K. Treatment for PPS doesn’t exist anywhere.”

Out of frustration, the Network has decided to find other resources, rather than depend on help from the BPF or local authorities. Edwards and McVay have met in Oxford with Hallam, Peter Field and other polio survivors to try to establish Britain’s first comprehensive PPS treatment center. Edwards has also met with Britain’s Secretary of State for Health and has asked the European Parliament for funding. “It is sad that we have to ask other countries for money to help British citizens,” she says. “But Britain has turned a blind eye to its polio survivors.””Understanding the cause and treatment of PPS has progressed in the U.S. by leaps and bounds over the past fifteen years,” adds McVay. “Time has been allowed to stand still in the U.K., with terrible physical and emotional consequences. For polio survivors on this side of the pond, the year is still 1980.”

America: Dollars or Sense?

It may be heartening for America’s polio survivors to know that their country is the world vanguard of PPS research and treatment. There are support groups and clinics throughout the U.S. and widespread — though hardly universal — knowledge about PPS among polio survivors and physicians. Payment for treatment is routine by private insurance, HMOs and even Medicare.

But America’s health care system has begun to change and will change even more. Medical care is far too expensive, Medicare and Medicaid are going bankrupt, and there are now 40 million Americans without health insurance.

There is the clear and present danger that politicians, in promising to balance the budget and cut taxes, might just ignore the rights and needs of people with disabilities and create a lean and genuinely mean government-run health care system that denies rehabilitation — or even medical care — to all those they judge not “worth the additional cost to society.”

How can health care be provided to all without cleaning out the treasury or neglecting those who most need treatment? I must admit to being an unrepentant liberal. I believe, since our Declaration of Independence guarantees “life, liberty and the pursuit of happiness,” that it is the government’s responsibility to make “life” possible by providing medical care to all citizens. I had thought that a taxfunded, government-run, single-payer system was the best way to provide that care. But my experience with British and Canadian polio survivors has caused me to re-think my position. Now I just don’t know what the solution is.

Whatever the shape of America’s health care future, any changes must be made only after considering the failures of both “socialized” and “free-market” medicine, and the rights, needs and input of people with disabilities. Decisions about disabled Americans’ life and health should not be made from the government down … or from the bottom line up.

Richard L. Bruno is director of the Post-Polio Rehabilitation and Research Service at the Kessler Institute for Rehabilitation, Saddle Brook, NJ. He is chair of the International Post-Polio Task Force.

Resources:

Leicestershire Polio Network

c/o Voluntary Action Leicester, P.O. Box 30, Leicester LE1 7ZX; e-mail warrior@stayfree.co.uk

Lincolnshire Polio Network

69 Woodvale Ave., Lincoln, Lincolnshire LN6 3RD; e-mail linpolio@legend.co.uk

Ontario March of Dimes

Post-Polio Services, 60 Overlea Blvd., Toronto, Ontario M4H 1B6; 416/425-3463, 800/263-3463; e-mail omod@inforamp.net

Polio Quebec Association

P.O. Box 745, Succ. Jean-Talon, Montreal, PQ H1S 2Z5; 514/935-9158 (English), 514/334-5395 (French); e-mail aitken@accent.net

Note: Some of the above postal and email addresses may have altered since this article was published. Follow the links which will take you to our Organzations Directory and thence to the organisations own web site which should have current contact details.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to

The Secretary, Lincolnshire Post-Polio Network

PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom

Telephone: +44 (0)1522 888601 Facsimile: +44 (0)870 1600840 Email: info@lincolnshirepostpolio.org.uk Web Site: www.lincolnshirepostpolio.org.uk

The Lincolnshire Post-Polio Network takes great care in the transcription of all information that appears at this site. However, we do not accept liability for any damage resulting directly or otherwise from any errors introduced in the transcription. Neither do we accept liability for any damage resulting directly or otherwise from the information available at this site. The opinions expressed in the documents available at this site are those of the individual authors and do not necessarily constitute endorsement or approval by the Lincolnshire Post-Polio Network.

Copyright is retained by The Lincolnshire Post-Polio Network and/or original author(s). Permission is granted to print copies of individual articles for personal use provided they are printed in their entirety. Links from other Internet WWW sites are welcome and encouraged. We only ask that you let us know so that we can in future notify you of critical changes. Reproduction and redistribution of any articles via any media, with the exception of the aforementioned, requires permission from The Lincolnshire Post-Polio Network and where applicable, the original author(s).

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/roulette.html> Created: January 1997

Last modification: 29th January 2010.

Polio Survivors Abroad Canadian Roulette, Rue Britannia Read More »

Red Flags for Polio Survivors

Dr. Richard Bruno

(Mis)Diagnosis: Many polio survivors have been incorrectly told they cannot have PPS because they didn’t have paralytic polio. The National Institute of Health has just studied pairs of identical twins, one who had paralytic polio and one who had non-paralytic polio. Seventy-one percent of those who had paralytic polio have PPS. The surprise is that 42 percent of those who had non-paralytic polio also have PPS.

Anesthesia: Polio survivors are anesthetized very easily because the part of the brain that keeps you awake was damaged by the poliovirus. They also stay anesthetized longer and can have significant complications from anesthesia. Even nerve blocks with local anesthetics can cause problems. All polio survivors should have lung function tests before having a general anesthetic. “Polio survivors with a lung capacity below 70 percent may need a respirator or respiratory therapy after surgery,” says Augusta Alba, of New York’s Goldwater Memorial Hospital.

Your complete polio history and any new problems with breathing coughing or swallowing should be brought to the attention of your surgeon or dentist–and especially your anesthesiologist–long before you go under the knife. Also, polio survivors should not have same-day surgery.

Pain: Two studies have shown that polio survivors are twice as sensitive to pain as everyone else and need more pain medication for a longer period after surgery or an injury. Survivors, however, have had to develop an extremely high pain tolerance to deal with their increased pain sensitivity and are not likely to become addicted to medications.

Sleep: Most polio survivors have trouble sleeping due to pain, anxiety or sleep disorders such as sleep apnea or generalized random myoclonus. GRM occurs when muscles in different parts of the body twitch and jump throughout the night.

Amazingly, most polio survivors aren’t aware that they stop breathing or twitch. If you awaken with a headache, with your heart pounding, short of breath or don’t feel rested in the morning, call a local sleep disorders center for a sleep study.

DO’S AND DON’TS

[The following information has been made available with the permission of the Easter Seal Society of Washington. The opinions expressed are those of the individual writer(s) and do not necessarily constitute an endorsement or approval by the Easter Seal Society or Polio Outreach Advisory Council. Although it does not imply endorsement, it is provided as a service for those seeking such information. Always consult your doctor before trying anything recommended in this information or any other publication.

For additional information concerning post-polio please contact your local Easter Seal Society, post-polio support groups, or other such services in your area.!

A GUIDE FOR POST-POLIOS DO’S AND DON’TS GENERAL THERAPIES AND THINGS

TO AVOID

What follows is a general, practical guide for post-polios to use, and summarizes the current thinking about post-polio. It will be most valuable if it stimulates you to seek further and more specific information.

+ Take time to rest. Nap (if possible) during the day, work fewer hours, and take longer vacations

+ If you are experiencing increasing muscle weakness, exercise only under the supervision of a knowledgeable physician.

+ Make sure your food intake is nutritious,

+ Be alert to (but not obsessed with) changes in your body, and heed your body’s signals.

+ Take note of any new symptoms plus clear or gradual changes.

+ Get enough exercise to prevent disuse atrophy, but not enough to produce overuse damage.

+ Learn how to pace yourself.

+ Prevent the secondary complications of weakness, particularly falls, this might entail the use of crutches or a cane, a wheelchair for extended travel, or braces or other adaptive equipment.

+ Avoid weight gain. Too much weight only aggravates stress on joints and muscles.

nsider possible adaptations to your lifestyle; even minor adjustments (changes in hobbies or modes of transportation) can help.

+ Do not assume that every physician fully understands post-polio problems. Educate yourself and never hesitate to ask questions,

+ Minimize alcohol use, particularly at bedtime. Alcohol inhibits swallowing, interferes with nutrition, and causes falls and accidents.

+ Maintain a positive attitude toward your health. Accept change, adapt, and never equate your self-worth with physical disabilities.

Red Flags for Polio Survivors Read More »

Preventing Complications in Polio Survivors Undergoing Surgery

THE KNIFE IS NOT SO ROUGH IF . . .

(PREVENTING COMPLICATIONS IN POLIO SURVIVORS UNDERGOING SURGERY)

Richard L. Bruno, Ph.D.

Lincolnshire Post-Polio Library copy by arrangement with the Harvest Center Library

Director, Post-Polio Rehabilitation and Research Service

Kessler Institute for Rehabilitation – Saddle Brook

Associate Professor,

Clinical Physical Medicine and Rehabilitation

New Jersey Medical School/UMDNJ

Chairperson, International Post-Polio Task Force

When orthopedist Henry Kessler wrote The Knife is Not Enough, describing what would become the specialty of rehabilitation, polio was America’s main event. Today, only a handful of specialists know how to treat polio survivors and Post-Polio Sequelae (PPS). Even more disturbing, few physicians have heard about, and even fewer know, what PPS are or under stand polio survivors’ special needs in a variety of medical settings.

Unfortunately, surgeons are often the most unaware of issues that don’t relate to their specialty. If you have to ‘go under the knife,’ the innocent ignorance of your surgeon (or his thoughtless disregard for your non-surgical problems) can lead to unnecessary emotional discomfort, excessive physical pain and occasionally serious complications.

However, it is important that polio survivors not add PPS to their list of worries surrounding surgery. Not one of our hundreds of patients has died or been seriously damaged even if none of the issues discussed in the following article was addressed. And, only a few of our hundreds of post-polio patients have permanently lost function after surgery.

So, read the article and give a copy to each member of the surgical team and the nurses on the hospital floor where you will be staying. Discuss the article in detail with your surgeon and ask that a copy be placed in your medical chart before you go under the knife. Hopefully, all members of your surgical team – the surgeon, anesthesiologist, recovery room and floor nurses – will listen to you and become educated about the special problems surrounding surgery for polio survivors – and your individual medical issues in particular – long before the order, “Scalpel.” is given in the O.R.

PREVENTING COMPLICATIONS IN POLIO SURVIVORS UNDERGOING SURGERY

Richard L. Bruno, Ph.D.

PPS Monograph Series. Volume 6(2). Hackensack:Harvest Press,1996.

Director, Post-Polio Rehabilitation and Research Service

Kessler Institute for Rehabilitation – Saddle Brook

Associate Professor,

Clinical Physical Medicine and Rehabilitation

New Jersey Medical School/UMDNJ

Chairperson, International Post-Polio Task Force

Unfortunately, only a handful of specialists treat Post-Polio Sequelae (PPS) – the unexpected and often disabling fatigue, muscle weakness, joint pain, cold intolerance, and swallowing, sleep and breathing problems – occurring in America’s 1.63 million polio survivors 40 years after their acute polio.[1,2] However, all medical professionals need to be familiar with the neurological damage done by the original poliovirus infection that today causes unnecessary discomfort, excessive physical pain and occasionally serious complications after surgery. This is a brief overview to inform patients and professionals about the cause and prevention of complications in polio survivors undergoing surgery.

PRE-OPERATIVE PREPARATION

The pre-operative period is the most important, since it is when polio survivors must establish communication with the surgical team. After the second opinion and a polio survivor’s decision to have surgery, the patient needs to ask the surgeon to read this article and the references cited. Then, surgical candidates must meet with the surgeon and anesthesiologist to discuss in detail patients’ complete polio and general medical histories and the problems that will likely arise before and during surgery, in the recovery room and on the nursing floor. It is also recommended that the polio survivor meet with the Supervisor of Nursing on the floor where they will be transferred after surgery to discuss likely problems during the post-op and recovery period.

Lungs. We recommend that all polio survivors have pulmonary function studies as part of their pre operative testing.[3] This is vital for those who had bulbar polio acutely, whether or not they used a respirator or an iron lung. But, polio survivors who have (or had) neck, arm or chest muscle weakness or have swallowing problems should also have their lung function tested so there will be no unpleasant surprises coming off the respirator at the end of the operation. Polio survivors with a lung capacity below

70% may need a respirator or respiratory therapy after surgery.[1]

Of course, polio survivors who use a respirator during the day or at night must discuss their respirator use and maintenance in detail with their surgeon, anesthesiologist, the nursing staff, and with their own pulmonologist, before admission to the hospital.

Physical Assistance. X-rays are a normal part of pre-op testing. Because of workers compensation concerns, many hospital staff are not eager to move or lift patients. Unfortunately, X-ray and examining tables are built at heights that are convenient for the professional, not the patient. Many polio survivors cannot step on a stool to get onto a high table, or even pull themselves over onto a table from a stretcher. Thus, polio survivors must ask for help in transferring.

Since most polio survivors have no experience asking for help under any circumstances, they need to find a phrase with which they are comfortable that will communicate whatever their needs are. Long explanations about having had polio or PPS or the specifics of which muscles are weak or paralyzed are not necessary. For example, a simple “My legs (arms) are paralyzed and I can’t get onto that table; I will need help” should suffice. This phrase may have to be repeated before the polio survivor will be assisted.

If the professional replies, “Oh, I bet you can move by yourself if you try!” or “Don’t expect me to lift you,” an appropriate response is “I cannot get onto the table. Please ask someone else to help me or let me speak to your supervisor.” A pleasant but steadfast refusal to do difficult or dangerous transfers is the polio survivor’s best defense against injury before or after surgery.

General Anesthetics. Polio survivors are exquisitely sensitive to anesthetic. It has been known for 50 years that the poliovirus damaged the area of the brain stem – called the reticular activating system (RAS) – responsible for keeping the brain awake.[4,5] Because the RAS was damaged in those who had paralytic and non-paralytic polio, a little anesthetic goes a long way and lasts a long time.

For example, the pre-operative medication used to ‘calm’ surgical patients – often a combination of Valium® and Demerol® – may by itself put polio survivors to sleep for 8 hours. (Such excessive and prolonged sedation does occur when I.V. Valium® is used alone in patients undergoing invasive but non surgical procedures, like endoscopy.) Add to a pre-operative ‘calming cocktail’ an intravenous anesthetic (like sodium pentothol ) or a gaseous anesthetic, and polio survivors have been known to sleep for days. In addition, polio survivors with respiratory problems may have trouble clearing the gaseous anesthetics. A number of our patients have awakened from anesthetic on a respirator in I.C.U. to the frightened faces of their family, surgeon and anesthesiologist several days after surgery.

Here is the first of rule of thumb – we call “Rules of 2” – for polio survivors’s having surgery: ANESTHETIC RULE OF 2 :

Polio survivors need the typical dose of anesthetic divided by 2.

This first “Rule of 2” is certainly not intended to dictate the dose of anesthetic, but merely to remind anesthesiologists that polio survivors need much less anesthetic than do other patients. This does not mean that a given polio survivor might require less than 1/2 the typical anesthetic dose, or that another won’t need more anesthetic. As always, the dose of anesthetic must be individually adjusted (for body weight, lipid space, etc) and be adequate to keep patients under during surgery but not cause them to sleep for a week.

Even applying the “Anesthetic Rule of 2” polio survivors may be very sedated, if not asleep, for many

hours after the surgery. This is one of the reasons why same-day surgery – even for complicated dental procedures – is not advisable for polio survivors. Sleeping or excessively sedated polio survivors cannot be expected to return home and take care of themselves after same-day surgery, since surgical complications may go unnoticed and sedation-impaired coordination makes falling likely. In spite of HMO pressure, NO POLIO SURVIVOR SHOULD HAVE SAME-DAY SURGERY FOR ANY REASON except for the most simple procedures that require only a local anesthetic.

Nerve Blocks. However, there are also problems with local anesthetics that numb only one area of the body. Spinal anesthetics, like epidural or saddle blocks used for childbirth and lower body procedures, often allow surgery without the patient being asleep and are therefore more desirable for polio survivors. However, the injection of a local anesthetic near the spine results in both pain-conducting nerves and motor neurons being anesthetized. Polio survivors are very sensitive to anything that further impairs their poliovirus-damaged motor neurons and a spinal anesthetic may cause polio survivors to be paralyzed for many hours. If a spinal anesthetic is used, polio survivors cannot be expected to get up and walk after surgery.

Curare-like drugs that are intended to paralyze muscles (e.g., succinylcholine) are typically used during major surgery to relax muscles that are going to be cut and make it easier for the ventilator to fill the lungs while patients are on the table. Again, any drug that interferes with muscle functioning will prevent polio survivors from walking or even moving for hours longer than it would for patients who didn’t have polio.

Regardless of whether a local, spinal or general anesthetic is used, the following applies: POST-ANESTHETIC RULE OF 2 :

Polio survivors require 2 times as long to recover from the effects of any anesthetics.

Blood and Guts. There are yet additional concerns. Polio survivors with muscle atrophy, especially in the thigh muscles, will have a smaller blood volume than would be expected for their height or weight. Therefore, bleeding during surgery may be more of a problem. Polio survivors may want to bank their own blood slowly over the course of weeks, even for procedures where excessive blood loss is not typically expected. However, since polio survivors may be significantly more fatigued and prone to faint after giving blood, relative’s blood may need to be banked instead.

Also, polio survivors can be sensitive to atropine-like drugs used to dry secretions during surgery.[6] Atropine-like drugs also slow the gut, and polio survivors may be excessively constipated after surgery or, rarely, actually have their intestines stop moving (paralytic ileus) for a period of time. These problems can be treated symptomatically as they would in someone who did not have polio.

Positioning. One overlooked problem is the positioning of the post-polio patient on the operating. Muscle atrophy, scoliosis and spinal fusions may make certain positions problematic, especially those involving extension of the spine. Since the polio survivor is usually unconscious during positioning, there will be no report of pain that would normally warn of potential damage. A number of polio survivors have experienced severe back pain for months post-op, and even permanent traction injuries of nerves, after being placed for hours in damaging positions. It would be advisable for the patient to be awake during positioning on the table to prevent such post-op complications.

POST-OPERATIVE CARE

Cold. If the dose of anesthetic is carefully regulated, a polio survivors’s first post-op experience will be

waking in the recovery room. Often, polio survivors awaken from anesthetic shivering violently. Research has shown that polio survivors are extremely sensitive to cold because they have difficulty regulating their body temperature. Polio survivors’ automatic (autonomic) nervous systems were damage by the poliovirus from the brain (hypothalamus) through the brain stem (reticular formation and vagal nuclei) to the spinal

cord (intermediolateral columns). [4-8] Polio survivors cannot control the size of their blood vessels, since the nerves that make the smooth muscle around veins and capillaries contract were paralyzed by the poliovirus. Therefore, polio survivors’ blood vessels open under anesthetic and dump the heat of their warm blood into the cold recovery room. Recovery room nurses need to know about this problem and help polio survivors stay warm. Additional blankets will help, and the surgeon can even write an order for a heated water blanket to be used in recovery.

Vomiting. Another post-op problem related to brain stem damage is vomiting. As in anyone who receives a general anesthetic, polio survivors can develop nausea and vomit. However, polio survivors are more apt to faint (have vasovagal syncope and even brief asystoles) when they attempt to vomit. [6] It is very important that post-operative emetic control be discussed with the anesthesiologist and administered before polio survivors go to the recovery room and that additional medication is written as needed in the post-op orders.

Choking. Yet another concern is difficulty swallowing as the patient is awakening. [9] Polio survivors who are aware of having swallowing problems, and sometimes in those without apparent swallowing difficulty, cannot clear secretions and may choke (or feel like they are choking) when they are lying on their backs, still half asleep, as the anesthetic is clearing. Polio survivors’ secretions need to be monitored in the recovery room and they should be positioned on their side if possible so that secretions can drain.

Pain. The single most troublesome problem after surgery is pain control. A number of studies have shown that many surgical patients are under medicated for pain. Under medication is a serious problem for the post-polio patient since two research studies have shown that polio survivors are twice as sensitive to pain as those who didn’t have polio.[8] Increased pain sensitivity is apparently related to poliovirus damage to endogenous opiate-secreting cells in the brain (paraventricular hypothalamus and periaquiductal gray) and spinal cord (Lamina II of the dorsal cord). [4,8]

RULE OF 2 for PAIN:

Polio survivors need 2 times the dose of pain medication for 2 times as long.

Since polio survivors are known to be extremely stoic, they are not likely to abuse or become dependent upon narcotics.

RECOVERY

In keeping with the “get ’em up, move ’em out” trend in medicine, there will be the tendency to get polio survivors up and walking almost immediately after surgery. This is not advisable for a number of reasons. When polio survivors reach the nursing unit, they may still be twice as sedated from the anesthetic as are other patients. Since polio survivors need a very clear head to be able to control their weakened, polio affected muscles to stand and walk, a fuzzy-headed polio survivor is at serious risk for falling.

Even if a polio survivor’s head is clear, the anesthetic or other drugs may have temporarily weakened or even paralyzed the muscles needed to stand and walk. What’s worse, the surgery may have cut muscles (especially abdominal muscles) that substitute for muscles paralyzed by polio (it is often muscle substitution that actually allows polio survivors to stand and walk, even though the muscles that are

typically needed to walk were permanently paralyzed). Not only will post-polio patients be unable to stand or walk, they may also be unable to even move to position themselves in bed. Polio survivors may also have low blood pressure after surgery that could itself cause lightheadedness, fainting and falls.

RULE OF 2 for RECOVERY:

Polio survivors should stay in bed 2 times longer than other patients.

Under any circumstances, polio survivors should get up slowly, first sitting up in bed, then sitting with feet dangling, then getting into a bedside chair with assistance, then standing with assistance and finally walking with assistance and appropriate assistive devices. With the necessity of additional additional bed rest, anti-embolism stockings to prevent blood clots may be a prudent precaution. Gentle physical therapy in bed may be advisable to maintain range of motion and for stretching, since polio survivors are prone to developing painful muscle spasms if they are not up and moving.

RULE OF 2 for LENGTH OF STAY

Polio survivors need to stay in the hospital 2 times longer than other patients.

While polio survivors may become deconditioned with bed rest some what faster than others patients, because of autonomic nervous system damage, the dangers of getting them up and walking too quickly far outweigh those of moving too slowly. Polio survivors have learned to be very aware of what their bodies can and can’t do. They are the best judges of when they can move, stand and walk safely.

Nursing Care and Nurse Caring. Polio survivors often have difficulty merely being in the hospital. They may have insomnia, anxiety, and even have panic attacks. These symptoms are easy to understand when it is remembered that as young children, polio survivors were ripped away from their families and admitted to rehabilitation hospitals for months or even years. [2,10,11] Post-polio children underwent multiple surgeries and painful physical therapy, procedures administered often without explanation and certainly without their consent.

Many post-polio patients have had multiple experiences of psychological, physical and even sexual abuse at the hands of hospital staff. Questions or complaints about painful and frightening therapies were not infrequently met by staff anger or punishment. Patients report having been locked in dark closets overnight when they asked questions, spoke out or cried. Necessary nursing care could be withheld for no apparent reason . Many post-polio children were slapped and some were actually beaten with rubber truncheons by physical therapists to ‘motivate’ them to stand up and walk.[10]

It is not surprising that polio survivors can be terrified of again becoming powerless patients at the mercy of hospital staff. Nursing staff’s appreciation of the childhood trauma polio survivors experienced at the hands of medical professionals, and taking a moment to actually listen and respond to the real needs of the adult post-polio patient, will go far toward making the patient feel safer and more comfortable during their stay.

Returning Home. There is another “Rule of 2” when surgical patients return home: RULE OF 2 for WORK:

Polio survivors need 2 times the number of days of rest at home before they return to work or household duties.

For all of the reasons described above, the entire recovery process takes longer for polio survivors. It is not uncommon for typically overachieving, hyperactive Type A polio survivors, who were taught as children to “use it or lose it,” to return to work or household duties the day after they return home from the hospital.[10,11] Polio survivors must be encouraged to rest and to return to activities slowly, especially if they are somewhat deconditioned and feel weaker or more fatigued post-op. Polio survivors should ask their surgeon for a note that allows them to stay home from work twice as long as the typical patient.

Post-Op PPS? The 1985 National Survey of Polio Survivors has shown that emotional stress is the second most frequent cause of PPS (after physical overexertion).[11] Certainly, there are few emotional or physical stressor more potent then surgery. So, polio survivors should expect some increase in fatigue and muscle weakness resulting from the combination of the physical and emotional effects of the surgery, anesthesia, other medications, and bed rest. However, only a handful of post-polio patients permanently lose function after surgery. Strength or endurance lost after surgery are typically recovered. To aid recovery, gentle physical therapy may be advisable. Passive stretching, range of motion exercises and slowly increasing endurance are more valuable than muscle strengthening exercise which can actually cause muscle weakness. Especially if a polio-affected part of the body has been operated on (stomach, back, arms or legs), a physiatrist who is thoroughly knowledgeable and experienced about the care of polio survivors and PPS should be consulted before surgery so that a post-op rehabilitation plan can be in place. A short stay in a rehabilitation hospital after surgery (especially after back or leg surgery) may make polio survivors recovery safer, faster and more complete.

Polio survivors need to remember:

RULE OF 2 for FEELING BETTER:

Polio survivors need 2 times longer to feel ‘back to normal’ again.

CONCLUSION

All of the “Rules of 2” are suggestions for polio survivors and the surgical team; they are not a substitute for specific information about the individual patient and communication among all members of the treatment team, including the patient. All polio survivors must be evaluated and managed according to their individual needs. Please take the time to read the following references (especially those in bold type) so that you will be fully knowledgeable about and be able to help meet polio survivors special needs.

REFERENCES

Bruno RL. Ultimate burnout: Post-polio sequelae basics. New Mobility, 1996; 7: 50-59. [Lincolnshire Library Full Text]
Frick NM, Bruno RL. Post-Polio Sequelae: Physiological and psychological overview. Rehabilitation Literature, 1986; 47: 106 – 111. [Lincolnshire Library Full Text] 3. Bach JR, Alba AS. Pulmonary dysfunction and sleep disorder breathing as post-polio sequelae: Evaluation and management. Orthopedics, 1991; 14 : 1329 – 1337. [Lincolnshire Library Full Text]
Bodian D. Histopathological basis of clinical findings in poliomyelitis. Am J Med. 1949; 6: 563- 578.
Bruno RL, Frick NM, Cohen J. Polioencephalitis, stress and the etiology of Post-Polio Sequelae. Orthopedics, 1991; 14: 1269 – 1276. [Lincolnshire Library Full Text]
Bruno RL, Frick NM. Parasympathetic abnormalities as post-polio sequelae. Archives of Physical

Medicine and Rehabilitation, 1995; 76: 594.

Bruno RL, Johnson JC, Berman WS. Vasomotor abnormalities as Post-Polio Sequelae. Orthopedics, 1985; 8: 865 – 869. [PubMed Abstract]
Bruno RL, Johnson JC, Berman WS. Motor and sensory functioning with changing ambient temperature in post-polio subjects. In LS Halstead and DO Wiechers (Eds.): Late Effects of Poliomyelitis. Miami: Symposia Foundation, 1985.
Bucholtz DW, Jones B. Post-Polio dysphagia: Alarm or caution. Orthopedics, 1991; 14: 1303- 1305. [PubMed Abstract]
Bruno RL, Frick NM. The psychology of polio as prelude to Post-Polio Sequelae: Behavior modification and psychotherapy. Orthopedics, 1991; 14 : 1185 – 1193. [Lincolnshire Library Full Text]
Bruno RL, Frick NM. Stress and “Type A” behavior as precipitants of Post-Polio Sequelae. In LS Halstead and DO Wiechers (Eds.): Research and Clinical Aspects of the Late Effects of Poliomyelitis. White Plains: March of Dimes Research Foundation, 1987. [Lincolnshire Library Full Text]

POLIO SURVIVORS’ PRE-OP CHECKLIST

Give articles to surgeon and discuss:

Pre-op lung tests.

Lower blood volume and blood banking?

Authorization for a longer stay in the hospital.

Orders for post-op anti-vomiting medication.

Positioning on the table during surgery.

Orders for staying warm in the recovery room.

Difficulty clearing secretions in the recovery room and on the nursing unit.

Orders for increased dose of pain medication.

Physical therapy for stretching and range of motion in hospital.

Placing articles about polio in the medical chart.

Give articles to anesthesiologist and discuss:

Lung problems?

Lower dose of pre-op calming medication.

Lower dose of anesthetic.

Longer-term paralysis of muscles with spinal anesthetic and curare-like drugs.

Orders for post-op anti-vomiting medication.

Difficulty clearing secretions in the recovery room.

Give articles to nursing supervisor and discuss:

Longer-term sedation with anesthetic.

Difficulty clearing secretions on the nursing unit.

Orders for increased dose of pain medication.

Needing help in moving in bed and in the room.

Not standing or walking until you are fully awake and able.

Anti-embolism stockings.

Meet with PPS physiatrist before surgery and discuss:

Post-op rehabilitation plan.

Physical therapy for stretching and range of motion in hospital.

Possible admission to a rehab hospital before going home.

Physical therapy for walking and increasing endurance at home.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to

The Secretary, Lincolnshire Post-Polio Network

PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom

Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk

Web Site: www.lincolnshirepostpolio.org.uk

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/surgery.html> Created: 24th January 1997

Last modification: 29th January 2010.

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Polio and CFS ME

Polio and CFS/ME

Dr. Richard L. Bruno

Dr. Bruno’s response to people who write about “poliovirus causing CFS”.

…have you heard of a Dr. William Campbell Douglas, editor of a publication called “Second Opinion,” and his contention that polio “may be with us again in a disguised form.”

I have read Dr. Douglas’s writings on this. But the idea that polio “may be with us again in a disguised form”, this is not true. I don’t think this is really what he’s saying.

“We may be mistakenly calling it chronic fatigue syndrome (U.S.) or myalgic encephalomyelitis (England). According to him, polio is more common than ever and may actually be caused by the polio vaccination.

Polio is NOT more common than ever and is NOT caused by the polio vaccination. [Dr. Bruno is commenting on the above, not on Vaccine Associated Polio (VAP) resulting from Oral Polio Vaccine (OPV)] There is no evidence that any of the polioviruses cause CFS.

“Dr. Douglas argues that the Salk and Sabin vaccines did not eliminate polio at all but forced it to change. While the vaccines suppressed the polio virus, it was replaced by genetically similar ones, such as Coxsackie virus often found in elevated levels in chronic fatigue syndrome patients.

Poliovirus being replaced by OTHER similar viruses is very different than polio being “with us again in a disguised form,” polio being more common than ever, being caused by the polio vaccination” or poliovirus “changing.” There is no consistent evidence of ANY virus, even the Coxsackie viruses, being “found in elevated levels in chronic fatigue syndrome patients.” However, “replacing” the poliovirus with a similar virus is a different story and may be what’s happening in CFS.

A British infectious disease specialist, Dr. Betty Dowsett, has been collaborating with us on our new studies of the links between PPS and CFS. She has sent me data showing that CFS cases rose significantly in the 1950s when the polio vaccines came on the scene. Her idea, repeated by Dr. Campbell, is that, like when one flock of birds takes over the territory of another flock when the first flock dies, other enteroviruses (especially the Coxsackie viruses) “took over” the territory of the 3 polioviruses when the polio vaccines reduced their number. There are several Coxsackie viruses that damage the brain activating system and could cause fatigue like that seen in CFS and PPS. There is even a Coxsackie virus (Coxsackie A7) that causes a condition identical to traditional paralytic polio, so similar in fact that it has even been called Russian Polio and even Poliovirus Type IV. There is nothing unique about the polioviruses’ ability to chew up the spinal cord and the brain stem.

We have a new paper on the parallels between PPS and CFS, describing the viral links between Coxsackie and other viruses and brain activating system damage, out in September in the American Journal of Medicine. The article is on the Harvest Center Web Page with our other articles on viral damage to the brain a post-viral fatigue.

Dr. Richard L. Bruno

Chairperson International Post-Polio Task Force and Director The Post-Polio Institute Englewood Hospital and Medical Center

Englewood, New Jersey 07631

Phone: (201) 894-3724 Toll Free: 1-877-POST- POLIO Fax: (201) 894-0324 PPSENG@AOL.COM

Many of Dr. Bruno’s papers are also available in the Lincolnshire Post-Polio Library and can be found listed under the catalogue entry for Bruno, Richard L., Ph.D..

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to

The Secretary, Lincolnshire Post-Polio Network

PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk Web Site: www.lincolnshirepostpolio.org.uk

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Created: 22nd October 1998

Last modification: 22nd January 2010.

Polio and CFS ME Read More »

A Pound of Prevention is Worth a Ton of Therapy

A Pound of Prevention Is Worth a Ton of Therapy

Richard L Bruno, Ph.D., Director of the Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation, East Orange

Dear Tabby: Forty years ago I caught a virus that killed half of the nerves in my spinal cord and brain stem. The nerves that survived were damaged by the virus but have been doing double the work to make up for the nerves that were killed. Even though my leg has been paralyzed for forty years, I have always been very hard-driving and am constantly busy: I work two jobs, have ten kids, take care of my 90-year-old mother and mother-in-law who live with me, volunteer at a hospital and a homeless shelter, and raise money to “Save the Whales” in my spare time. Other people who caught this virus are and are having severe problems with fatigue, new muscle weakness, and pain. However, I feel line right now-except for daily headaches. Should I change my lifestyle to try to prevent new problems? – “Symptomless in Seattle”

There has never been a longitudinal study that looks at the emergence of post-polio sequelae (PPS) in polio survivors who have no symptoms. However, each time someone surveys polio survivors, the percentage of individuals reporting PPS goes up. When Mary Codd did the first population-based study at the Mayo Clinic in 1983, she found that 22.4% of paralytic polio survivors had PPS. The 1987 National Health Interview Survey estimated that of America’s 1,63 million polio survivors, 66% who had paralytic polio and 24% who had non-paralytic polio had PPS. In England, where they don’t even believe in PPS, 78% of the 1940’s-era graduates of one polio hospital are reporting “new symptoms.” Something must be going on that causes more and more polio survivors to report PPS as the years go by. But what? In 1985, Laura Halstead reported (Halstead and Rossi, 1985) that PPSdeveloped in those who were at least 10 years old when they had had polio and in those who had had a more severe infection, i.e. those who had been hospitalized at polio onset and had had more limbs paralyzed. In 1988, Klingman and colleagues found that it wasn’t just the severity of the polio but how much you recovered in the succeeding years that predicted new symptoms. That is, the more damage the polio virus did and the more your central nervous system recovered the more axonal sprouts you created and the bigger your motor units became), the more you had to lose and the more likely you were to have PPS. Then, in the 1985 and 1990 National Post-Polio Surveys, Nancy Frick and I (Bruno and Frick, 1987: 1991) found that both physical overexertion and emotional stress are reported by polio survivors to trigger PPS. Further, we reported that polio survivors who were “Type A” (hard-driving, time-conscious, and overachieving) were more likely to develop PPS. It appears that both physical and emotional stress on a polio-damaged and overworked central nervous system led to the development of PPS. This conclusion is supported by the 1991 findings of Paul Peach at Warm Springs. He found that in individuals who already have PPS, the symptoms of fatigue, weakness, and pain just get worse if patients don’t follow their therapists’ recommendations to decrease physical and emotional stress. So what do polio survivors like “Symptomless in Seattle” do if they don’t currently have symptoms? Well, it doesn’t take “Dear Tabby” or even a post-polio specialist to figure out that people with a smaller number of polio-damaged and severely overworked neurons are at risk for developing symptoms that result from chronic physical and emotional stress. Will all polio survivors eventually develop PPS? Will ALL polio survivors develop ALL post-polio sequelae -from fatigue through swallowing problems? Certainly not. But just as people who’ve had a heart attack are advised to reduce stress and their fat intake, shouldn’t polio survivors without PPS try to protect their damaged and overworked nerves? We advise all polio survivors to follow the Post-Polio Golden Rule: IF SOMETHING YOU DO CAUSES FATIGUE, WEAKNESS, OR PAIN, YOU SHOULDN’T BE DOING IT. Why shouldn’t polio survivors try to protect their quality of life by decreasing physical and emotional stress through the moderation and pacing of activities, the use of appropriate assistive devices (especially those that were readily discarded so many years ago), and reducing hard-driving, Type A behavior (see Agree and Rodriguez, 1991: Bruno and Frick, 1991: Young, 1991)? Will these self-care activities prevent the onset of PPS? No one knows. But self-care is the foundation of therapy for PPS and both our patients’ reports and the research literature clearly show that the progression of PPS is at least halted and symptoms reduced when patients start to take care of themselves. So it’s hard to imagine that self-care won’t have some preventative effect with regard to PPS And if prevention is possible, it would certainly be a lot less difficult and expensive than the physical, occupational and psychological therapy that are necessary to treat post-polio syndrome. What if self-care merely holds PPS off for a while, or if it reduces the severity of symptoms when they do occur? Or what if self-care has no long-term effect on PPS? Why should polio survivors spend their lives beating up their bodies and their spirits taking care of everyone around them, to the point where they don’t even spend a moment of the day thinking about, let alone caring for, themselves? Why should anyone live like that? As you know, polio survivors say, “Self-care is selfish! I feel too guilty to take care of myself.” Self-care, moderation, and reduced stress have been recommended as the foundation for a peaceful and even happy life for nearly 2,500 years, since the time of the Buddha. Taking care of yourself isn’t selfish, say the Buddhists. They say that since compassion for everyone is the basic principle that leads to peace and happiness, people cannot be peaceful or happy if they spend their days exhibiting compassion for the whole world with the exception of just one person: themselves. Dear “Symptomless”: I don’t know anything about viruses. But I do think it makes sense for you to start taking better care of yourself. Why is it so hard for us to take care of ourselves? Why should you be doing for everyone else and ignoring and abusing yourself? Don’t you at least deserve not to have your head hurt all the time? Why don’t you start by doing one thing for yourself for each thing you do for someone else? Maybe you won’t prevent new physical problems. But the worst that could happen is that other people will still be helped and you’ll feel better. Why should you “Save the Whales” and not save yourself – Tabby Source: Newsletter of the New Jersey Polio Network

This site is now maintained by Chris Salter, of the Lincolnshire Post-Polio Network Original Document Preparation: Tom Dempsey. Last modification: 10th January 1998

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Post-Polio Fatigue Where Is The Lesion

POST-POLIOMYELITIS FATIGUE WHERE IS THE LESION?

THE CONTROVERSY – THREE POINTS OF VIEW

A REPORT OF A SPECIAL NEUROLOGY ROUNDS AT THE MONTREAL NEUROLOGICAL INSTITUTE AND HOSPITAL SEPTEMBER 26, 1997.

The origin of post-polio fatigue is controversial. Thanks to Dr. Cashman’s initiative, there was a respectful discussion about the cause and source of Post-polio fatigue at the Neurological Grand Rounds of the Montreal Neurological Institute on the morning of September 26 attended by physicians, other health-care practitioners, students and a few members of Polio Quebec. The speakers were

Dr. Richard Bruno (Psychophysiologist and Director of the Post-Polio Rehabilitation Research Service, Kessler Institute for Rehabilitation, New Jersey Medical School) spoke about the role of cerebral abnormalities on post-polio fatigue.

Dr. Neil Cashman (Co-Director of the MNIH Post-Polio Clinic with Dr.Trojan)

Dr. Robert Miller (Chair of the Department of Neurology, California Pacific Medical Centre) who flew in for the occasion.

A lightly edited resumé delivered by Dr. Cashman at Polio Quebec’s Annual General Meeting the following day appears below for those who were unable to be with us or who would like to “hear” it again.

“Post-polio fatigue is probably the most common and certainly one of the most disabling symptoms that occur after polio. There is a great deal of controversy in the field as to what is causing the fatigue. The stakes are very high, because if we were to understand what is causing the fatigue then we could design a treatment or therapy that would help counter it.

“Dr. Bruno has spent a great deal of time working on an idea prompted by early pathological studies done by David Bodean who did post-mortems and found that an encephalitis existed in the brains of people who had acute polio. Because about 10% of those who had polio actually died from the illness, their brains and spinal cords could be examined to try to figure out what the polio virus was doing to the nervous system. We all know that the polio virus affects and destroys motor neurons, or motor nerve cells in the spinal cord and in the brain stem. This is what accounts for weakness and muscle atrophy. What the original investigators were finding, and Dr. Bruno has capitalized on this, is evidence of destruction of nerve cells outside of the motor system. One area that is commonly affected in people who have had polio is the reticular formation, i.e., a group of nerve cells that seem to support consciousness, so to speak. Dr. Bruno has taken this and said – what if there is a post-polio syndrome of the reticular formation, and how would that manifest itself? Dr. Bruno’s theory would present as a disorder in arousal, retrieval, attention, and it may account for some of the troubles people have with concentration, with memory and with

fatigue. This is the crux of Dr. Bruno’s argument that, in fact the fatigue that people with PPS have is actually brain fatigue – nothing to do with the nerves and muscles. Rather it is a disorder in concentration and memory. He presented some data that suggests that medicines such as bromocryptine that increase the function of the reticular formation can actually improve fatigue and attention in people with this type of post-polio syndrome.

‘Then I (Dr. Cashman) added that, yes, there is involvement of other parts of the brain and the brainstem, but the obvious and clear area of involvement by polio is the motor neurons, and when motor neurons die, remaining healthy motor neurons sprout extra branches to try to supply the muscle fibers that have lost their branches in the polio – a great recovery mechanism for say 10 or 20 years, but ultimately the motor nerve cell which is designed to support approximately 1,000 muscle fibers may, after polio, support 10,000 muscle fibers. Eventually the motor neuron starts to say this is too much. I can’t keep this stability up forever, and these extra branches begin to degenerate. This is not a motor neuron disease like Lou Gehrig’s disease. It’s at the other end of the motor unit – it’s a terminal axonal disease. The evidence we have of this is from muscle biopsies and a number of electrophysiological tests. We’re certain – I’ll put it that strongly – that there is degeneration of terminal axons. Everyone who has done these sorts of experiments in the world, agrees there is degeneration of terminal axons in PPS. But Dr. Trojan and I have tried to take this a step further. When you think about it, a terminal axon is not normal one day and gone the next. There must be an intervening period of poor function – of disfunction. And this may account for some waxing and waning symptoms of PPS, like fatigue. What Dr Trojan and I have done over the past five years is to collect evidence that there is a problem with the function of the terminal axons. One of the things we found is that one of the defects – one of the ways the nerve terminals are disfunctioning is that they have decreased release of a chemical called acetylcholine. This chemical accounts for the nerve talking to the muscle. If there isn’t enough acetylcholine, the muscle doesn’t get stimulated and doesn’t work properly – doesn’t generate a force. So we have tried to increase the communication between the nerve and the muscle by giving a medicine that prolongs the effect of acetylcholine in the junction between the nerve and the muscle. This is the basis for the use of Mestinon. We believe we have good preliminary results to show that Mestinon does work, and trust this will be demonstrated in the report, to be released in November, of a large multi-centred double blind Mestinon trial. [See preliminary report in NewsBites]

“Dr. Miller, well-versed and very knowledgeable about post-polio syndrome and other neuro-muscular diseases, defines fatigue very tightly as a decrease in muscle strength with exertion and a return of normal function with rest. He argued that the fatigue in PPS acts more like muscle fatigue than central or neuromuscular junction fatigue. He has done studies demonstrating the problem with the role of calcium in the muscle. Calcium is used to stimulate a muscle contraction, and if the muscle depolarizes without the release of calcium, then a good force isn’t generated for the effort the nerve has put into it. He believes that all of these ideas about fatigue, whether it be central from the brain, at the neuro-muscular junction, or whether it is at the level of the muscle fiber itself, fail to explain the severity of fatigue experienced by people with PPS. His major idea for therapy of this type of fatigue is to engage in exercise. Dr. Miller believes that a great deal of PPS fatigue is due to deconditioning, and that non-fatiguing exercise, or even fatiguing exercise, can lead to an increase in strength and a decrease in fatigue. I find this a bit hard to swallow, (said Dr. Cashman) but given Dr. Miller’s love to run marathons and sailboard in San Francisco Bay at every opportunity, I can see where he’s coming from…. If you’re tired you go and exercise….

“So, there are at least three camps when it comes to understanding fatigue. People are paying attention and doing a great deal of work to understand the underlying physiology of fatigue and post-polio syndrome. The debate has now been formulated. We’ll see which theory prevails over the next few years. It could be that we’re all right. It could be that some people have one type of fatigue and others another type. It could

be that we are all wrong.

Video and Audio Cassettes (English only) are available of the Grand Rounds Contact Sally Aitken (514 932 6092) – or write the Association. In UK contact the Lincolnshire Post-Polio Network. Prices not available at time of going to press.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to

The Secretary, Lincolnshire Post-Polio Network

PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk Web Site: www.lincolnshirepostpolio.org.uk

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Created: 24th January 1998

Last modification: 22nd January 2010.

Post-Polio Fatigue Where Is The Lesion Read More »

Does Post-Polio Cometh After a Fall

DO PPS COMETH AFTER A FALL?

Richard L. Bruno, PhD From “New Mobility” magazine, May, 1997

Supplied by Tom Walter with permission of the author

About once a month I get a call from an attorney somewhere in these litigious United States. I am asked to be the expert witness for a polio survivor who’s been rear-ended in their car, hit by a bus, taken a header down some stairs or simply slipped and fell.

Regardless of the type of accident, the lawyer always asks the same question: Can a traumatic event trigger Post-Polio Sequelae, the new and sometimes disabling muscle weakness, fatigue, pain and respiratory problems that occur is as many as 77% of polo survivors?

And regardless of the type of accident, my answer is always the same: Yes and No. PPS is not a disease that is just waiting inside polio survivors for a trigger to set it loose to wreak havoc throughout the body. So trauma can’t trigger a disease that is not there.

But our 1985 National Survey did show that PPS symptoms are caused by physically or emotionally stressing the poliovirus-damaged motor nerves that remained after survivors’ original bout with polio. Many polio survivors have been able to function for 40 years with about half the spinal motor nerves of someone who didn’t have polio.

So breaking a leg in a fall, having major surgery — even a whiplash injury — could sufficiently stress the remaining polio-damaged motor neurons to “blow a fuse.” When those fuses blow, neurons function less well and muscle weakness, fatigue, pain may result.

Many polio survivors are terrified about losing function after trauma. One survivor said, “I am afraid if I fall and break something I will never walk again.” Fear also causes polio survivors to postpone even necessary surgery because, as one survivor put it, “I know I’ll never survive the anesthetic. I will spend the rest of my days in an iron lung.”

Because of the fear that an injury or surgery could cause PPS, we wanted to find out just how many of our patients actually experienced new symptoms after trauma, what those symptoms were, whether they spread throughout the body and whether they were irreversible or treatable.

Surgery, Spills and Other Ills

We reviewed the histories of 244 consecutive polio survivors evaluated by Kessler Institute’s Post-Polio Service who had no other conditions that might cause new fatigue, weakness or pain. Of those patients, 44 (18%) said that their PPS began after a traumatic event.

The typical patient was 59 years old and had polio at age 8 in the early 1940’s. There were as many men as women reporting the post-trauma onset of PPS.

The traumas that preceded new symptoms included medical illnesses and surgeries: pneumonia, viral infection, hysterectomy, mastectomy with chemotherapy, pregnancy; fractures of the ankle, leg or hip; falls, auto accidents, and injury or surgery to the leg (ankle sprains, knee surgery, hip or knee replacement) or the back (herniated discs, laminectomies, spinal fusions).

The most common injury was to the leg (71% of patients) while 26% had back injuries. Regardless of the type of trauma or location of the injury, the most common symptom reported was new muscle weakness (55% of patients) followed by pain (34%) and fatigue (11%).

There was no evidence that new symptoms began in an injured area and then ‘spread’ throughout the body. Seventy-one percent of patients had new symptoms only in the body area that had been injured, while 26% had symptoms in the injured area plus one other nearby location.

For example, 40% of those who injured one leg developed weakness or pain in the other leg. This is a common problem for polio survivors, who compensate for injury to one part of the body by overusing another part whose nerves were also damaged by the poliovirus. Only 5% of patients developed symptoms in more than two body areas. One patient who had a hip replacement reported ‘loss of muscle tone all over,’ while another who had been in a coma after an auto accident reported weakness in all of his muscles. Two patients who had had fractures, two with back injuries and one with an ankle injury reported new fatigue.

However, no patient reported that their trauma “triggered” symptoms unrelated to the injury, such as arm weakness after breaking a leg or difficulty swallowing following a knee replacement.

Can Post-Traumatic PPS be Treated?

All of the clinical experience and research on treating non-traumatic PPS supports one conclusion: If patients decrease physical and emotional stress their symptoms will at very least stop progressing and typically will get noticeably better. Does this hold true for post-traumatic PPS?

There’s good news and bad news. The bad news is that the majority (63%) of patients with post-traumatic symptoms refused treatment altogether or refused to complete therapy for their symptoms; more than twice as many post-traumatic PPS patients actually quit therapy.

What might cause this? Seventy-seven percent of those who had a psychiatric diagnosis refused therapy, versus 53% of those without psychological problems. The most frequent psychiatric problem was a major depressive episode; 89% of those who were depressed refused therapy.

Depression has been identified before as a significant cause of therapy refusal in polio survivors and highlights how important it is for psychological problems to be identified and treated if therapies for PPS are to even begin.

The good news is that 86% of patients regardless of the type of trauma or severity of their injuries had

significant reductions in pain, fatigue and muscle weakness after complying with therapies known to be effective in treating PPS: reducing physical and emotional stress, using appropriate assistive devices, energy conservation, adequate rest and the pacing of activities.

The remaining patients experienced a reduction in some symptoms, especially pain, but continued to report muscle weakness or fatigue. Two patients who did not stop strenuous work or recreational activities reported slowly increasing muscle weakness and pain over several years.

Another patient who had been thrown to the floor of a van in 1995 reported that muscle strength and endurance in her legs increased only slightly after therapy even though her severe back pain has been eliminated. It is noteworthy that this patient had completely recovered from two previous traumas: a fall early in 1995 that fractured her lower right leg and another auto accident seven years before that herniated a disc. This patient’s ability to recover from two previous traumas is also good news.

For each of our patients who reported PPS symptoms after a trauma there was at least one other patient who had had the same trauma but did not develop PPS. So while trauma can be sufficient to cause PPS, PPS does not necessarily “cometh after a fall.”

The Golden Rule

These findings in our patients should put polio survivors’ minds at ease. Neither major surgery nor even a fall that causes a fracture will necessarily push polio survivors down a slippery slope toward total disability. Still, caution must be exercised since damaged motor neurons make polio survivors more susceptible to problems that typically do follow trauma.

A leg that has been in a cast for months can become weak, as can the opposite leg that has had to take up the slack for its damaged partner. And bed rest after surgery can more easily cause deconditioning and fatigue in polio survivors.

However, post-traumatic symptoms in polio survivors should not be treated aggressively as they often are in those who didn’t have polio. All PPS need to be treated carefully and slowly.

Polio survivors and their therapists should not assume that a leg weakened after being in a cast has merely ‘been resting too long’ and will respond to an aggressive program of weight lifting.

Polio survivors who have had surgery should not be rushed out of bed to prevent deconditioning, because the lingering effects of anesthetic and post-operative pain are more likely to cause falls than to prevent fatigue.

Regardless of the cause of PPS the “Golden Rule” for polio survivors always applies:

If an activity causes fatigue, weakness or pain, don’t do it !

Doctors, nurses, and therapists must listen carefully to their patients — and polio survivors must listen carefully to their own bodies — to determine how much exercise or therapy causes fatigue, weakness or pain, and to stop before those symptoms appear, so that therapy for PPS does not become just another type of trauma.

The experience of our patients is that post-traumatic PPS are treatable if polio survivors follow through with therapy. But even more important is that many traumas can be avoided, like the falls and fractures caused by compulsive overdoing, ignoring new muscle weakness and refusing to use a needed brace, cane

or crutch.

For polio survivors physical overexertion, like pride, does goeth before a fall.

Dr. Richard L. Bruno is director of the Post-Polio Rehabilitation and Research Service at the Kessler Institute for Rehabilitation, Saddle Brook, NJ. He is chair of the International Post-Polio Task Force.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to The Secretary, Lincolnshire Post-Polio Network PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom Telephone: +44 (0)1522 888601 Facsimile: +44 (0)870 1600840 Email: info@lincolnshirepostpolio.org.uk Web Site: www.lincolnshirepostpolio.org.uk

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/kessler/accident.html> Created: 30th July 1997

Last modification: 30th January 2010.

Does Post-Polio Cometh After a Fall Read More »

A Letter to a Polio Survivor

The following is a genuine letter which has been depersonalised. Publication at the suggestion and with the permission of Professor Richard L. Bruno PhD

KESSLER INSTITUTE FOR REHABILITATION, INC.

300 Market Street Saddle Brook, New Jersey 07663 U.S.A. (201) 368-6057

Email PPSKIR@AOL.COM

Note. Professor Bruno has relocated and his new email address is PPSENG@AOL.COM. For further details see The Post-Polio Institute Englewood (NJ) Hospital and Medical Center in our Specialist Clinics Directory.

POST-POLIO REHABILITATION AND RESEARCH SERVICE Office of the Director

21 February 1997

Dear Polio Survivor:

1) You were asked if you have evidence that you in fact had polio.

[Fill in here your past history of Polio]

2) You have been told that one had to have significant damage from the polio and muscle

atrophy to have PPS.

Neither the severity of the original polio infection nor muscle atrophy, either due to the original polio or late-onset atrophy – are required for the diagnosis nor are predictors of PPS. The postmortem studies of Professor David Bodian of Johns Hopkins University in the 1940’s showed that anyone who had paralytic polio lost on average 50% of their anterior horn cells but required the death more than 60% to demonstrate any paralysis (Bodian D. Histopathological basis of clinical findings in poliomyelitis. American Journal of Medicine, 1949; 6: 563-578.)

However, Bodian also found that at least 90% of all anterior horn cells were in some way affected during the polio infection (Bodian D. 1947. Poliomyelitis: Neuropathologic observations in relation to motor symptoms. Journal of the American Medical

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Association, 1947, 134:1148-1154).

With regard to the prediction of PPS, our research and that of other investigations (see Klingman, et al. Functional Recovery: A major risk factor for the development of PPS. American Journal of Physical Medicine and Rehabilitation, 1988, 45:645-647) show that it is the degree of original paralysis plus the degree of recovery that predicts PPS. Klingman showed that 79% of the variance of PPS symptoms “could be accounted for by recovery alone.”

Our research has also shown that physical overexertion is also a primary factor in triggering PPS in 96% of subjects in the first American National Survey of Polio Survivors (Bruno RL, Frick NM: Stress and “type A” behavior as precipitants of postpolio sequelae. In: Halstead LS, Wiechers DO (eds): Research and Clinical Aspects of the Late Effects of Poliomyelitis. White Plains, NY, March of Dimes, 1987, pp. 145-155).

3) You have been told you must use your limbs and that, if you did not, then the muscles

would wither away.

This advice is particularly inscrutable. All of the world’s medical research and clinical literature on the treatment of PPS shows that persisting in physical exertion in the face of new symptoms, and especially the prescription of exercise, continue the downward progression of PPS.

The recommendations for energy conservation and assistive device use provided you are just right (see Young GR. Energy conservation, occupational therapy and the treatment of post-polio sequelae. Orthopedics, 1991; 14:1233-1239). A two-year follow-up study of patients treated by the Roosevelt Institute at Warm Springs showed that patients who refuse to implement such suggestion have pain and fatigue increase and lose more than 2% of muscle strength per year (Peach PE, Olejnik S. Effect of treatment and noncompliance on post-polio sequelae. Orthopedics, 1991;13:1199 -1203). Patients who implemented energy conservation and assistive device use actually gained muscle strength over time.

Our just-completed follow-up study of patients treated by the Kessler Post-Polio Service since 1990 shows a 76% increase in muscle weakness over 16 months in patients who refuse to implement energy conservation and assistive device use. Patients who do implement these suggestion report a 21% decrease in muscle weakness.

4) You have been told that PPS is all conjecture and that PPS is not a problem in

Lincolnshire.

I would have readily agreed that PPS was all conjecture in 1983. But after four international symposia and thirteen years of research published in journals including

Neurology, the Archives of Physical Medicine and Rehabilitation, the American Journal of Physical Medicine and Rehabilitation, the Journal of Rehabilitation, the Journal of the American Medical Association, and the Annals of the New York Academy of Science, there is no longer any conjecture about the reality of PPS.

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With regard to the prevalence of PPS in the U.K., 74,280 cases of polio had been reported to the U.K. Communicable Disease Surveillance Centre between 1912 and 1961. As in the United States, the ratio of polio cases to actual notification of the C.D.S.C. was quite low before the large epidemics of the late 1940’s, your government describing notification as “far from complete.”

The cases of polio reported to our Centers for Disease Control was 480,000 during the same period, with notification also being described as unreliable since U.S. physicians were not required to notify the C.D.C. of polio cases until 1952. It is not surprising then that 1987 U.S. Department of Public Health National Health Interview Survey calculated that there were 1.63 million Americans alive today who had had polio. It is likely that the actual number of living survivors of polio in the U.K. is closer to 250,000.

With regard to the prevalence of PPS, the recent survey of the membership of the British Polio Fellowship found the incidence of PPS to be 77%. Therefore, between 57,000 and 192,000 British polio survivors are experiencing the same symptoms that you report.

I cannot encourage you strongly enough to be physically examined and to immediately implement all of the recommendations made by your Doctor and your occupational therapist and any other ideas of which you can conceive to save your remaining polio damaged motor neurons.

I hope I have answered your questions and concerns. I encourage you to obtain and show the articles I have referenced herein to your physicians. Please contact me if you, your physicians or therapists have further questions.

Yours truly,

Professor Richard L. Bruno

Department of Physical Medicine and Rehabilitation New Jersey Medical School

University of Medicine and Dentistry of New Jersey and

Director Post-Polio Rehabilitation and Research Service Kessler Institute for Rehabilitation

and

Chairperson, International Post-Polio Task Force.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

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Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/kessler/letter.html> Created: 31st July 1997

Last modification: 30th January 2010.

http://www.poliosurvivorsnetwork.org.uk/archive/lincolnshire/library/kessler/letter.html 26/12/2015, 22:01

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A Letter to a Polio Survivor Read More »

Bromocriptine in the Treatment of Post-Polio

BROMOCRIPTINE IN THE TREATMENT OF POST-POLIO FATIGUE: A pilot study with implications for the pathophysiology of fatigue.

Richard L. Bruno, Ph.D.,¹ ² Jerald R. Zimmerman, M.D.,¹ ² Susan Creange, M.A.,¹ Todd Lewis, Ph.D.,¹ Terry Molzen, M.A.,¹ and Nancy M. Frick, M.Div, Lh.D.³

American Journal of Physical Medicine and Rehabilitation, 1997 (in press)

Lincolnshire Post-Polio Library copy by arrangement with the Harvest Center Library

1. Post-Polio Rehabilitation and Research Service

Kessler Institute for Rehabilitation East Orange, New Jersey

2. Department of Physical Medicine and Rehabilitation

UMDNJ/New Jersey Medical School Newark, New Jersey

3. Harvest Center

Hackensack, New Jersey

Address all correspondence and reprint requests to: Dr. Richard L. Bruno,

Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation,

300 Market Street,

Saddle Brook, New Jersey 07662.

(201) 368-6057

This research was supported by grants from the George Ohl, Jr. Infantile Paralysis Foundation, Sandoz Pharmaceuticals, The Joel Leff Charitable Trust, and the Deluxe Corporation.

Short Title: Bromocriptine and Post-Polio Fatigue

ABSTRACT

Objective: Determine the effectiveness of bromocriptine in the treatment of severe and disabling postpolio fatigue.

Design: Placebo-controlled drug trial in a pilot series of patients.

Setting: Outpatient rehabilitation hospital.

Patients: Of 83 patients without comorbidities who completed treatment with the Post-Polio Service, 5 of 8 patients who had paralytic polio and continued to report moderate to severe daily fatigue after complying with conservative treatments for post-polio fatigue agreed to be studied.

Intervention: Placebo was given for four weeks followed by increasing doses of bromocriptine mesylate (Parlodel®) administered at noon for 28 days reaching a total dose of 12.5 mg/day.

Main Outcome Measures: Daily logs of subjective fatigue and cognitive difficulties.

Results: Three of the subjects reported symptom improvement on bromocriptine but not on placebo. However, all subjects experienced nausea on bromocriptine, likely eliminating blinding. Drug responders had clinically impaired performance on neuropsychological tests of attention and information processing speed. Logged daily difficulty with attention, cognition, word finding memory, staying awake and fatigue on awakening were significantly negatively correlated with days on bromocriptine, but not with days on placebo, in drug responders.

Conclusions: A double-blind, placebo-controlled multicenter study will be needed to confirm bromocriptine’s effectiveness in treating attentionally-impaired polio survivors whose severe and disabling fatigue does not responded to conservative treatment.

Key Words: Bromocriptine; Dopamine; Polio; Post-Polio Sequelae; Fatigue; Attention

BROMOCRIPTINE IN THE TREATMENT OF POST-POLIO FATIGUE: A pilot study with implications for the pathophysiology of fatigue.

Fatigue is the most commonly reported and most debilitating Post-Polio Sequelae (PPS) affecting the more than 1.63 million American polio survivors. [1] In national surveys of polio survivors, 91% reported new or increased fatigue, 41% reported fatigue significantly interfering with performing or completing their work and 25% reported fatigue interfering with self-care activities. [2,3] Importantly, polio survivors differentiate between physical tiredness that they associate with new muscle weakness or decreased endurance, and a “brain fatigue” that is characterized by fatigue plus difficulties with attention and cognition. Between 70% and 96% of polio survivors with fatigue reported concomitant problems with concentration, memory, attention, word-finding, maintaining wakefulness and thinking clearly, with 77% percent reporting moderate to severe problems with these functions. [3] On formal neuropsychological testing, only polio survivors reporting severe daily fatigue demonstrated clinically significant deficits of attention, concentration and information processing speed. [4] It has been hypothesized that both subjective fatigue and attention deficits in polio survivors result from poliovirus damaging neurons within the brain’s activating system. [3] Postmortem studies of 50 years ago demonstrated the consistent presence of poliovirus lesions in the midbrain reticular formation, posterior hypothalamus, thalamic nuclei, putamen and globus pallidus. [3,5] Recently, magnetic resonance imaging of the brain has shown small discrete or multiple punctate areas of hyperintense signal in the reticular formation, thalamic and caudate nuclei, putamen, centrum semiovale, deep and periventricular white matter only in polio survivors reporting fatigue. [3,6] Postmortem histopathology also found that neurons secreting neurotransmitters

known to activate the brain, especially dopaminergic neurons in the substantia nigra, were also damaged or destroyed by the poliovirus. [5,7] The reduced secretion of dopamine has been hypothesized to play a role in impairing polio survivors’ ability to activate the cortex, thereby generating difficulty with attention, concentration and maintaining wakefulness that polio survivors described as “brain fatigue.” [7] The hypothesized role of the basal ganglia and dopamine in activating the brain suggests that a dopamimetic agent would be the most promising candidate to pharmacologically treat brain fatigue. [7] However, any dopamimetic agent employed to treat fatigue should not stimulate (as do amphetamines and methylphenidate) nor require the functioning of (as do L-Dopa, monoamine reuptake blockers and monoamine oxydase inhibitors) the remaining poliovirus-damaged neurons in the brain’s activating system. Therefore, bromocriptine mesylate (Parlodel ®; Sandoz), a direct-acting, post-synaptic dopamine 2 (D2) receptor agonist, was chosen for a placebo-controlled study of a pharmacological treatment for post-polio fatigue.

METHODS

Subjects. Only patients who were without comorbidities that could cause fatigue or cognitive problems (e.g., psychiatric diagnoses, thyroid, cerebrovascular or cardiac disease, anemia, respiratory insufficiency, sleep apnea or hypopeneas, lupus or diabetes) and who successfully completed treatment for PPS were eligible for the drug trial. Of eighty-three patients who met these criteria, only ten continued to report at least moderate daily fatigue (the fatigue level required for inclusion in the study) after applying the conservative treatments of choice for post-polio fatigue, i.e., reducing physical and emotional stress, using appropriate assistive devices, energy conservation, adequate rest and the pacing of activities. [8,9,10] Of those ten, two met DSM-III-R criteria for a major depressive episode and were excluded. [11] Of the remaining eight patients, six agreed to the drug trial. One patient withdrew after her husband was severely injured in a fall, leaving five patients to be studied.

Procedure. Fatigue research is methodologically problematic since fatigue is subjective and therefore difficult to quantify. This problem is compounded by polio survivors’ difficulty acknowledging, let alone quantifying, their physical symptoms and the variability of their fatigue, both within and between days. [8] Therefore, all subjects gave written informed consent and completed a modified version of the PostPolio Fatigue Questionnaire (PFQ) for 14 days prior to beginning drug, rating subjective levels of fatigue five times during the day and rating daily and difficulty with motivation, attention, mind wandering, thinking clearly, concentration, word finding, object naming, memory, staying awake during the day, and muscle weakness on a six-point scale from “none” through “severe.” [3,4] All but one subject who was severely claustrophobic had magnetic resonance imaging (MRI) of the brain which were read by a radiologist blinded to the subjects symptoms (see 6). On another occasion, plasma ACTH was drawn at 11:00 AM after an overnight fast. (see 7) On the 15th day, blood was drawn at 10:00 AM for a complete blood count, chemistry, thyroid panel and plasma prolactin. Neuropsychological tests that assessed attention, concentration and information processing speed were then administered by a blinded examiner. The Double Letter Cancellation Test required subjects to cross-out two specified letters on a sheet filled with similar letters in the shortest possible time to assess selective and sustained attention. [12] The Gordon Diagnostic Systems continuous performance test, measuring focused and sustained attention, required subjects to watch numbers presented for 6 minutes on a 1 by 2 cm. LED display. [13] subjects were instructed to press a button only when the number “9” appeared immediately following the number “1” while two flanking LED displays also presented numbers including “9” and “1” (distractibility task) and while the flanking displays were dark (vigilance task). The Trail Making Test assessed visual scanning and visual motor speed by requiring subjects to draw a line to sequentially connect circles containing 25 ascending numbers (Part A) or sequentially but alternately connect circles containing ascending numbers and the letters of the alphabet (Part B) in the shortest possible time. [14] The Paced

Auditory Serial-Addition Test (PASAT) assessed complex attention and information processing speed by requiring subjects to sequentially add a series of 60 digits presented by a tape recording at three presentation speeds: one digit every 1.6, 2.0 and 2.4 seconds. [15] All neuropsychological tests were administered and scored according to accepted procedures.

Placebo/Drug Administration. The 14 days of pre-testing PFQs were reviewed with the subjects to determine that they understood how to rate and log their symptoms. Subjects were told that they would receive either placebo or a drug which it was hoped would reduce their fatigue symptoms. They were given 28 daily log forms and instructed to rate their fatigue upon awakening, at 12:00, 3:00, 6:00 and 9:00 PM. Subjects were instructed to describe any side effects experienced during the day and rate their overall difficulty with the subjective symptoms listed on the PFQ at 9:00 PM. Since bromocriptine almost invariably causes nausea, all subjects received placebo first. Subjects were given 10 numbered bottles, each containing three opaque capsules, and instructed to start with bottle number one and to take one capsule each day during the noon meal. Every three days, they would open the next higher-numbered bottle and take one capsule at noon. Subjects were unaware that they were receiving a cornstarch placebo during the first 28 study days. After 28 days on placebo, subjects were re-administered the neuropsychological tests, given another set of log forms, bottles with identical capsules and told to continue taking the capsules as they had been. Subjects began with 1.25 mg/day of bromocriptine and increased the dose by 1.25 mg every three days, reaching a total daily dose of 12.5 mg. After 28 days on bromocriptine, subjects were readministered the neuropsychologic tests and had blood drawn for bromocriptine plasma level and plasma prolactin, which is suppressed by the administration of dopamine receptor agonists.

Data Analysis. Since the small sample size prevented statistical comparisons, subjects’ individual measurements and group means were compared to established clinical norms to determine whether the physiological variables or neuropsychologic test performance were clinically abnormal. (Table 1) SYSTAT (Version 6.0) was used to calculate product-moment correlations and linear regressions for daily fatigue and symptom difficulty ratings with days on placebo and bromocriptine for each individual subject. [16] Correlations were transformed to z scores, averaged across subjects, and then transformed back to correlations. [17] (Table 2) Because the large number of variables being correlated increased the probability of obtaining significant correlations by chance, the Bonferroni inequality was applied to decrease the probability (p) value needed for significance by dividing the p value by the number of variables being correlated. The regression equations were used to calculate the fatigue and symptom difficulty rating for each drug responder for the last day on placebo (day 28) and on bromocriptine (day 56). The mean calculated fatigue and symptom difficulty ratings are shown in Figure 2.

RESULTS

Demographics. Subjects (3 female; 2 male) were 49 ( ± 2.1) years old having had paralytic polio at age 5 ( ± 2.6) in 1949 ( ± 2.4) and 15 ( ± 3.0) years of education. One subject had been a diplegic wheelchair user since polio. The others had ambulated for 35 years without assistance before the onset of new muscle weakness; 3 were prescribed a straight cane and one a molded ankle/foot orthosis when treated for their PPS. Subjects rated their current daily fatigue as moderate to severe. Four subjects were retired on Social Security disability as a result of fatigue preventing continued employment; the remaining subject worked part-time as a secretary.

Drug Response: Neuropsychological Testing. Three subjects reported a noticeable reduction in fatigue and related cognitive symptoms on bromocriptine as compared to placebo. Drug responders reported that they “felt awake” and “had a clear head” for the first time in several years. Two subjects reported no effect at

all on bromocriptine or placebo. There were clinically significant differences in baseline measures between drug responders and non-responders (Table 1). There were more than twice as many areas of hyperintense signal on MRI of the brain on average in the drug responders. Fasting ACTH was abnormal in all of the drug responders in that it was not elevated above the baseline value expected for non-fasting subjects at 11:00 AM. [7] A clinically significant increase in mean ACTH was seen in the non-responders, one of whom had a normal elevation in fasting ACTH. The baseline mean plasma prolactin level was nearly doubled in the drug responders, suggesting reduced brain dopamine activity. In the drug responders, the lower mean number of correct responses at baseline on the Gordon distractibility and vigilance tests and on the 2.4 numbers/second presentation of the PASAT were clinically abnormal, as was the longer time required to perform the Trail Making Test (Part A). Scores on all neuropsychological measures in all subjects moved toward or reached normality with repeated testing on placebo and then on bromocriptine. Prolactin decreased from 11.2 ( ± 6.2) ng/ml at baseline to 3.2 ( ± 4.0) ng/ml on bromocriptine as would be expected with a dopamine receptor agonist. The plasma level of bromocriptine was below the detectable range in all subjects.

Drug Response: Symptom Logs. In the drug responders, the rating of fatigue upon awakening and difficulty with attention, word finding, mind wandering, concentration, remote memory, thinking clearly decreased and were significantly negatively correlated with days on bromocriptine, but not with days on placebo (Figure 2; Table 1). These ratings decreased by about one grade, from above “mild to moderate” to “mild” (Figure 2). Fatigue decreased from about “moderate” to “mild to moderate,” with fatigue upon awakening decreasing most (55%). Subjective muscle weakness did not change on bromocriptine or on placebo. In the drug non-responders, difficulty with word finding, memory, and object naming were significantly positively correlated with days on bromocriptine. However, fatigue at noon and 9:00 PM and muscle weakness were significantly positively correlated with days on placebo. Nevertheless, the nonresponders did not complain of an increase in any of these symptoms during either phase of the study.

Side Effects. The most common complaint was a low-grade nausea that is the most frequently reported side-effect of bromocriptine. All subjects reported nausea for from 2 to 10 days (a mean of 28% days) on bromocriptine. Two subjects reported nasal fullness, one for 9 days and another for 2 days, for a mean of 20% of the days on bromocriptine. The latter subject also reported a daily dull headache on bromocriptine that may have been related to nasal fullness. However, no subject discontinued bromocriptine because of these side effects. At the highest dose of bromocriptine, one subject did have an episode of severe nausea, followed by six brief episodes of syncope with high-grade atrioventricular block when she attempted to vomit. While this subject had only two previous episodes of mild nausea on bromocriptine, she did have a life-long history of syncope during vomiting. Although cardiodepressor syncope was not a previously known side effect of bromocriptine, the drug was discontinued.

DISCUSSION

This pilot study of severely fatigued polio survivors suggests that bromocriptine may be of use in the treatment of post-polio fatigue that has not responded to conservative therapies. However, the small sample size and methodological limitations make this suggestion merely tentative. Since nausea was universally experienced, all subjects may have realized that they were receiving active drug and were thereby biased toward reporting reductions in symptoms. The percentage of days on which side effects were experienced on bromocriptine was higher in the responders (48%) than non-responders (34%). Although the placebo phase was not compromised, the bromocriptine phase of the study may have been as unblinded as an open-label drug trial. Reductions in morning fatigue and fatigue-related cognitive symptoms as the dose of bromocriptine increased were not paralleled by drug-related improvements in neuropsychologic test scores, most likely since the same form of the tests was administered on placebo

and on bromocriptine. Subjects repeatedly taking the same test would be expected to demonstrate a learning effect and have their test scores improve. This has been seen even in polio survivors with severe fatigue who were repeatedly administered the same neuropsychologic tests over the course of several hours [4] It was also surprising that drug responders noticed no reduction in fatigue during the afternoon, since bromocriptine was administered at noon so that a peak blood level would be reached at 3:00 PM when many polio survivors report hitting an afternoon “wall” of fatigue. Since the pharmacokinetics of bromocriptine could differ from the time course of its pharmacological effect, as is seen with D2 receptor antagonists, moving the dose of drug to before sleep or upon awakening may prolong any beneficial effect of the drug into the afternoon. [18]

A Central or Peripheral Pathophysiology of Fatigue? Several other agents have been studied in an attempt to treat post-polio fatigue. In placebo-controlled studies, high-dose prednisone and amantadine, a drug with anticholinergic and dopamimetic properties, did not decrease fatigue. [19,20] An open-label trial of pyridostigmine was conducted in 25 polio survivors whose fatigue had not responded to conservative treatments. [21] After at least one month on drug, 64% of subjects reported a 56% decrease in “general fatigue,” a finding comparable to the 55% decrease in morning fatigue in 60% of the subjects in this study. The authors concluded that although the subjective complaint of fatigue has multiple causes, it was reduced in their subjects by pyridostigmine enhancing conduction at the neuromuscular junction and thereby increasing muscle strength and decreasing muscle fatiguability. Since bromocriptine reduced morning fatigue and fatigue-related cognitive symptoms, but had no effect on subjective muscle weakness, it is likely acting centrally to stimulate dopaminergic receptors within the brain’s activating system. This conclusion is supported by a placebo-controlled study of healthy subjects who were administered remoxipride, a potent and selective D2 receptor antagonist. [18] The most frequently reported effects of D2 receptor blockade were “moderate fatigue,” “mild somnolence” and “difficulty concentrating.” Statistically significant, dose-related increases in subjective “drowsiness” and impairment on neuropsychological tests of auditory vigilance, continuous attention and critical flicker fusion wer also found following D2 receptor blockade. However, decreased neuromuscular junction transmission impairing muscle contraction and decreased attention resulting from impaired brain activation are not mutually exclusive causes of post-polio fatigue. The contribution of both muscle weakness and impaired attention to the complaint of fatigue was documented by administering the Post-Polio Fatigue Questionnaire as part of the 1990 National Post-Polio Survey. [3] Multiple regression revealed that the only significant contributors to the prediction of daily fatigue severity were “difficulty focusing attention” (r=0.31; p=.005) and “muscle weakness” (r=0.19; p=.023). Actually, there may be an interaction between impaired transmission at the neuromuscular junction, decreased brain activation and symptoms of fatigue. As early as 1939, muscle spindle afferent activity was recognized as important in the maintenance of “alertness.” [22] Neuromuscular transmission blockade, which eliminated muscle activation and thereby afferent muscle spindle discharges, was shown to inhibit the ascending reticular activating system, decrease cortical activation, cause “drowsiness” and even induce a level of anesthesia sufficient for surgery. [23,24,25] Further, the relaxation and reduced recruitment of motor units have been identified as peripheral signs of central fatigue, possibly resulting from a decrease in the activity of the descending reticular activating system. [26,27]

Clinical Application and Caveats. These very preliminary findings suggest that bromocriptine may be helpful in treating polio survivors whose fatigue is more central in origin (i.e., associated with impaired attention and cognition) while pyridostigmine may be helpful in treating patients whose fatigue is more peripheral (i.e., associated with muscle weakness, fatiguability or decreased physical endurance). Responders to pyridostigmine had higher fatigue ratings at baseline, while drug responders in this study had clinically significant impairments of attention and information processing speed, more hyperintensities on MRI, an absent ACTH response to stress and increased plasma prolactin. Taken

together, these data suggest that any medication for post-polio fatigue will benefit only the most fatigued, neuropsychologically impaired or neurophysiologically abnormal patients who have not responded to conservative therapies. This conclusion is underscored by the experience with the drug non-responders. One was the least impaired of the subjects, reporting moderate to severe daily fatigue, having two hyperintensities on MRI, scores on only two of the attention tests that fell just below the normal range, and being the only subject able to work, albeit part-time. The other non-responder reported severe daily fatigue, had one punctate hyperintensity in her rostral reticular formation on MRI, but had no abnormalities on neuropsychological testing. However, her daily logs revealed that she was applying none of the conservative treatments that had been recommended. She neither paced her activities nor rested during the day. She went to sleep after midnight on 87% of the study days, compared to a mean of 26% of study days for the other subjects. Following the study, she modified her schedule and her daily fatigue decreased from severe to moderate to severe without the use of medication. This subject’s inability to implement the lifestyle changes necessary to treat her fatigue may be responsible for the positive correlations between days on placebo, fatigue, cognitive symptoms and muscle weakness in the nonresponders. Polio survivors’ frequent non-compliance with fatigue-reducing lifestyle changes is well described. [8,28] However, medication is not a substitute for self-care and no medication to treat postpolio fatigue should be prescribed until all conservative therapies have been consistently applied by the patient and found inadequate to reduce fatigue sufficiently to allow a satisfactory level of personal, vocational and recreational functioning.

Future Research. The finding that only 10% of compliant patients without comorbidities reported moderate or greater daily fatigue after treatment by the Post-Polio Service is hopeful, indicating that conservative therapies appear to be successful in treating most polio survivors’ fatigue. However, the success of non-pharmacological therapies makes difficult identifying a sufficiently large sample of appropriate subjects at any one facility. Clinician/researchers thoroughly familiar with treating polio survivors and PPS at a number of post-polio clinics will be needed if a sufficiently large sample is to be recruited for a double-blind, placebo-controlled, cross-over study of the efficacy of bromocriptine in the treatment of severe, treatment-resistant post-polio fatigue. Since nausea was reported by all subjects, domperidone – a dopamine receptor antagonist that does not cross the blood-brain barrier and has been shown to counteract the nausea caused by dopamimetics in Parkinson’s patients without decreasing bromocriptine’s efficacy – could be given with bromocriptine to allow a blinded, cross-over design. [29] However, the safety of bromocriptine remains a concern. Given the association of nausea and syncope in one subject, dopamimetics may be contraindicated in polio survivors with a history of syncope or even in those with bulbar polio that damaged brain stem cardioregulatory centers. [30,31,32] Also, the long-term effects of dopamimetic agents in polio survivors have not been studied. Receptor down-regulation and the development of tolerance with the chronic use of a D2 receptor agonist is a possibility. Down-regulation occurs with negative clinical consequences following chronic use of dopamimetics in Parkinson’s disease patients. [33,34,35] Bromocriptine-induced receptor down-regulation could actually increase fatigue by combining with reductions in D2 receptor number and the level and activity of dopamine-synthetic enzymes in the nigra and putamen that accompany aging. [36,37] These reductions, thought to occur “due to impairment of cellular regulatory systems or to decreased presynaptic dopamine input” resulting from attrition of dopaminergic neurons, may be accelerated in polio survivors and have been implicated in the pathogenesis of fatigue as polio survivors reach midlife. [3,7,27] On the other hand, it has also been suggested that dopamine receptor agonists may exert a “protective action” on poliovirus-damaged, metabolically impaired dopaminergic neurons since they reduce the demand for pre-synaptic dopamine production. [3,38] Reduced dopamine production would decrease neurons’ internal metabolic requirements and lower oxidative stress by limiting the amount of hydrogen peroxide formed by the action of MAO-B. [38] Thus, administration of bromocriptine may not only potentially decrease symptoms of brain fatigue but also could possibly slow the metabolic failure of the remaining poliovirus-damaged

dopaminergic neurons. [39] Future studies will need to address these concerns as well as objectively document fatigue’s peripheral and central symptoms and signs if the pathophysiology of fatigue, location of drug action, and drug efficacy and safety are to be determined.

ACKNOWLEDGEMENTS

The authors gratefully acknowledge the participation of the subjects, the continuing support of the George Ohl, Jr. Infantile Paralysis Foundation, Sandoz Pharmaceuticals for providing the bromocriptine, and the efforts and expertise of Doris Sipos, Carol Diveny and Mary Ann Solimine, R.N., M.L.S., with out whom this work would not have been possible. We also thank Jim Schoen, R.P., M.B.A. for preparing and packaging bromocriptine and placebo, Joan Alverzo, R.N. for monitoring side effects in some of the patients, and Dr. Oliver Sacks for his comments.

REFERENCES

1. Parsons PE: National Health Interview Survey. Hyattsville, Maryland, National Center for Health

Statistics, 1989.

2. Bruno RL, Frick NM: Stress and “Type A” behavior as precipitants of Post-Polio Sequelae, in

Halstead LS and Wiechers DO (eds): Research and Clinical Aspects of the Late Effects of

Poliomyelitis. White Plains, March of Dimes Research Foundation, 1987, pp 145-155.

[Lincolnshire Library Full Text]

3. Bruno RL, Frick NM, Cohen, J: Polioencephalitis, stress and the etiology of Post-Polio Sequelae.

Orthopedics 1991;14 (11):1269-1276. [Lincolnshire Library Full Text]

4. Bruno RL, Galski T, DeLuca J: The neuropsychology of post-polio fatigue. Arch Phys Med Rehabil

1993;74:1061-1065. [Lincolnshire Library Full Text]

5. Bodian D: Histopathological basis of clinical findings in poliomyelitis. Am J Med 1949;6:563-578.

6. Bruno RL, Cohen J, Galski T, Frick NM: The neuroanatomy of post-polio fatigue. Arch Phys Med

Rehabil 1994;74:1061-1065. [Lincolnshire Library Full Text]

7. Bruno RL, Sapolsky R, Zimmerman JR, Frick NM: The pathophysiology of a central cause of postpolio fatigue. Ann NY Acad Sci 1995;753:257-275. [Lincolnshire Library Full Text]

8. Bruno RL, Frick NM: The psychology of polio as prelude to Post-Polio Sequelae: Behavior

modification and psychotherapy. Orthopedics 1991; 14:1185-1193. [Lincolnshire Library Full

Text]

9. Agre JC, Rodriquez AA: Neuromuscular function in polio survivors. Orthopedics 1991;14:1343-47.

[PubMed Abstract]

10. Young GR: Energy conservation, occupational therapy and the treatment of post-polio sequelae.

Orthopedics 1991;14:1233-1239. [Lincolnshire Library Full Text]

11. Diagnostic and Statistical Manual III – Revised. New York, American Psychiatric Association,

1995.

12. Lezak MD: Neuropsychological Assessment. New York, Oxford University Press, 1976.

13. Gordon M: Microprocessor-based assessment of attention deficit disorders (ADD). Psychopharm

Bull 1986;22:288-290. [PubMed Abstract]

14. Reitan RM: The Halstead-Reitan Neuropsychological Battery: Theory and Clinical Practice.

Tucson, Neuropsychology Press, 1985.

15. Gronwall DMA: The paced auditory serial-addition task: A measure of recovery from concussion.

Percept Motor Skills 1977;44:367-373. [PubMed Abstract]

16. Wilkinson L, Hill M: SYSTAT (Version 6.0). Chicago, SPSS, 1995.

17. Guilford JP, Fruchter B: Fundamental statistics in psychology and education. New York, McGraw

Hill, 1973.

18. Fagan, D., Scott, D.D., Mitchell, M. and Tiplady, B: Effects of remoxipride on measures of

psychological performance in healthy volunteers. Psychopharmacology 1991;105:225-229.

[PubMed Abstract]

19. Dinsmore S, Dambrosia J, Dalakas MC: A double-blind, placebo-controlled trial of high-dose

prednisone for the treatment of post-poliomyelitis syndrome. Ann NY Acad Sci 1995;753:303-313.

[PubMed Abstract]

20. Stein DP, Dambrosia JM, Dalakas MC: A double-blind, placebo-controlled trial of amantadine for

the treatment of fatigue in patients with the Post-Polio Syndrome. Ann NY Acad Sci 1995;753:296-

302. [PubMed Abstract]

21. Trojan DA, Cashman NR: An open trial of pyridostigmine in post-poliomyelitis syndrome. Can J

Neurol Sci 1995;22: 223-227. [Lincolnshire Library Full Text]

22. Kleitman N: Sleep and wakefulness. Chicago, University of Chicago Press, 1939.

23. Gellhorn E: The influence of curare on hypothalamic excitability and the electroencephalogram.

EEG clin Neurophysiol 1958;10:697-703.

24. Hodes R: Electrocortical synchronization resulting from reduced proprioceptive drive caused by

neuromuscular blocking agents. EEG clin Neurophysiol 1962;14:220-232.

25. Shea LT, Davidson GM, Davis J: Electroencephalographic studies of the curarized patient. Med J

Aust 1954;2:656-659.

26. Edwards RHT: Human muscle function and fatigue, in Ciba Foundation Symposium 82: Human

Muscle Fatigue. Pittman, London, 1981.

27. Bruno RL, Frick NM, Creange S, Zimmerman JR, Lewis T: Polioencephalitis and the Brain

Fatigue Generator model of post-viral fatigue syndromes. J Chronic Fatigue Syndrome 1996 (in

press). [Lincolnshire Library Full Text]

28. Bruno RL: Predicting hyperactive behavior as a cause of non-compliance with rehabilitation: The

Reinforcement Motivation Survey. J Rehabil 1995;61:50-57. [Lincolnshire Library Full Text]

29. Quinn A, Illas A, Lherrmitte F, Agid Y: Bromocriptine and domperidone in the treatment of

Parkinson’s disease. Neurology (Ny) 1981;31:662-667. [PubMed Abstract]

30. Baker AB: Neurologic signs of bulbar poliomyelitis. In Poliomyelitis. Philadelphia, Lippincott,

1949.

31. Bruno RL, Frick NM: Parasympathetic abnormalities as post-polio sequelae. Arch Phys Med

Rehabil 1995;76:594.

32. Bruno RL, Frick NM, Creange SJ: Nocturnal generalized myoclonus as a post-polio sequelae. Arch

Phys Med Rehabil 1995;76:594.

33. Lee T H, Ellinwood E H, Nishita JK: Dopamine receptor sensitivity changes with chronic

stimulants. Ann NY Acad Sci 1988;537:324-329. [PubMed Abstract]

34. Kartzinel R, Calne DB: Studies with bromocriptine: I. “On-off” phenomena. Neurology (Minneap)

1976;26:508-510. [PubMed Abstract]

35. Lees AJ, Haddad S, Shaw KM, et al: Bromocriptine in parkinsonism – a long-term study. Arch

Neurol 1978;35:503-505. [PubMed Abstract]

36. Wong DF, Wagner HN, Dannals RF, et al: Effects of age on dopamine and serotonin receptors

measured by positron emission tomography in the living human brain. Science 1984;226:1393-

1396. [PubMed Abstract]

37. Antonini A, Leenders KL, Reist H, et al: Effect of age on D2 dopamine receptors in normal human

brain measured by positron emission tomography and 11C-Raclopride. Arch Neurol 1993;50:474-

480. [PubMed Abstract]

38. Jenner P: The rationale for the use of dopamine agonists for Parkinson’s disease. Neurol

1995;45(Suppl 3):S6-S12. [PubMed Abstract]

39. Yoshikawa T, Minamiyama Y, Naito Y, Motoharu K: Antioxidant properties of Bromocriptine, a

dopamine agonist. J Neurochem 1994;62:1034-1038. [PubMed Abstract]

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/bromo.html> Created: January 1997

Last modification: 29th January 2010.

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Preventing Complications in Dental Procedures

BE TRUE TO YOUR PPS AND YOUR TEETH WON’T BE FALSE TO YOU: PREVENTING COMPLICATIONS IN POLIO SURVIVORS UNDERGOING DENTAL PROCEDURES Richard L. Bruno, Ph.D.

Lincolnshire Post-Polio Library copy by arrangement with the Harvest Center Library

Director

Post-Polio Rehabilitation and Research Service Kessler Institute for Rehabilitation – Saddle Brook

Associate Professor

Clinical Physical Medicine and Rehabilitation New Jersey Medical School/UMDNJ

Chairperson, International Post-Polio Task Force

Unfortunately, only a handful of specialists treat Post-Polio Sequelae (PPS) – the unexpected and often disabling fatigue, muscle weakness, joint pain, cold intolerance, and swallowing, sleep and breathing problems – occurring in America’s 1.63 million polio survivors 40 years after their acute polio. [1,2] However, all medical professionals need to be familiar with the neurological damage done by the original poliovirus infection that today causes unnecessary discomfort, excessive physical pain and occasionally serious complications with surgery. This is a brief overview to inform patients and professionals about the cause and prevention of complications in polio survivors undergoing dental surgery.

PRE-OPERATIVE PREPARATION

The pre-operative period is the most important, since it is when polio survivors must establish communication with their dentist or oral surgeon. Patients need to ask the dentist to read this article and the references cited. Then, patients must meet with the dentist (and anesthesiologist, if one will be involved) to discuss in detail patients’ complete polio and general medical histories and the problems that

may arise before, during and after the procedure.

The Psychology of Polio Survivors. Polio survivors often have difficulty with any medical procedure, even dental surgery. They may have insomnia, anxiety, and even have panic attacks. These symptoms are easy to understand when it is remembered that as young children, polio survivors were ripped away from their families and underwent multiple surgeries and painful physical therapy, procedures administered often without explanation and certainly without their consent. [2,3,4] Questions or complaints about painful and frightening procedures were not infrequently met by staff anger or even physical arose.

It is not surprising that polio survivors can be terrified of again becoming powerless patients at the mercy of medical professionals. The dental staff’s appreciation of the childhood trauma polio survivors experienced, and taking a moment to actually listen and respond to the real needs of the adult post-polio patient, will go far toward making the patient feel safer and more comfortable.

Breathing and Swallowing. We recommend that all polio survivors have pulmonary function studies before surgery, especially if a gaseous anesthetic will be used. [5] This is vital for those who had bulbar polio, which affected the respiratory centers in the brain stem, whether or not patients used a respirator or an iron lung following the acute polio. Even patients who have (or had) neck, arm or chest muscle weakness or have swallowing problems should have their lung function tested, since even these individuals may have difficulty breathing or clearing secretions (swallowing saliva) during or after the procedure. Polio survivors with a lung capacity below 70% may need respiratory therapy or even a respirator after surgery if a gaseous anesthetic was used. [1] Of course, polio survivors who use a respirator during the day or at night must discuss their respirator use in detail with their dentist, anesthesiologist, and their own pulmonologist before any surgery. [5]

It should also be noted that breathing and swallowing can be compromised in those who had bulbar polio or chest wall paralysis, not only by anesthetics, but also merely by reclining in the dental chair. Polio survivors often have difficulty breathing or swallowing saliva when reclining. A comfortable reclined position must be identified before the procedure begins. And the procedure may need to be interrupted frequently to allow the patient to breath fully and to swallow. Also, a number of polio survivors have experienced severe neck or back pain following lengthy procedures, since their muscles spasm easily when placed in unusual or awkward positions, including hyperextension (extreme bending backward) of the neck.

Physical Assistance. Transferring to and from the dental chair are important considerations for polio survivors who have long-standing paralysis, newly weakened muscles or joint instability and pain due to PPS. Some patients may not be able to stand or pull themselves into the dental chair. Thus, polio survivors must ask for help in transferring, especially after the procedure when they are still partially anesthetized.

Polio survivors, who typically never ask anyone for help under any circumstances, need to find a phrase with which they are comfortable that will communicate their needs. Long explanations about having had polio or PPS or the specifics of which muscles are weak or paralyzed are not necessary. For example, a simple “My legs (arms) are paralyzed and I can’t get into/out of that chair. I will need help” should suffice. This phrase may have to be repeated before the polio survivor will be assisted.

If the professional replies, “Oh, I bet you can do it by yourself if you try!” or “Don’t expect me to lift you,” an appropriate response is “I cannot get into the chair. Please ask someone else to help me or let me speak to the doctor.” A pleasant but steadfast refusal to do difficult or dangerous transfers is the polio survivor’s best defense against injury before or after the procedure.

General Anesthetics. Polio survivors are exquisitely sensitive to anesthetic. It has been known for 50 years that the poliovirus damaged the area of the brain stem – called the reticular activating system (RAS) – responsible for keeping the brain awake. [6,7] Because the RAS was damaged in those who had paralytic and non-paralytic polio, a little anesthetic goes a long way and lasts for a long time.

For example, the pre-operative medication used to ‘calm’ patients – often a combination of Valium® and Demerol® – may by itself put polio survivors to sleep for 8 hours. Such excessive and prolonged sedation can also occur when I.V. Valium® is used alone. Add to a pre-operative ‘calming cocktail’ an intravenous anesthetic (like sodium pentothol) or a gaseous anesthetic, and polio survivors have been known to sleep for several days. In addition, polio survivors with respiratory problems may have trouble clearing gaseous anesthetics. A number of our patients have awakened from anesthesia on a respirator in I.C.U. to the frightened faces of their family, surgeon and anesthesiologist several days after surgery.

Here is the first of rule of thumb – we call them ‘Rules of 2’ – for polio survivors’ having surgery: GENERAL ANESTHETIC RULE OF 2:

Polio survivors need the typical dose of general anesthetic divided by 2.

This first ‘Rule of 2’ is certainly not intended to dictate the dose of anesthetic, but merely to remind oral surgeons that polio survivors need much less anesthetic than do other patients. This does not mean that a given polio survivor might require less than 1/2 the typical anesthetic dose, or that another won’t need more anesthetic. As always, the dose of anesthetic must be individually adjusted (for body weight, lipid space, etc.) and be adequate to keep patients under during surgery but not cause them to sleep for a week.

Nerve Blocks. Unfortunately, polio survivors also have problems with local anesthetics. While polio survivors are more sensitive to general anesthesia, they seem to require more local anesthetic. Two research studies have shown that polio survivors are twice as sensitive to pain as those who did not have polio, apparently as a result of poliovirus-damage to endogenous opiate-secreting cells in the brain (paraventricular hypothalamus and periaquiductal gray) and spinal cord (Lamina II of the dorsal cord).

[6,7,8]

LOCAL ANESTHETIC RULE OF 2:

Polio survivors need 2 times the typical dose of local anesthetic.

However, the injection of a local anesthetic can result in both pain-conducting and motor nerves being anesthetized. Polio survivors are very sensitive to anything that further impairs their poliovirus-damaged motor neurons, and a local anesthetic may cause facial, tongue and pharyngial (throat) muscles to be paralyzed for many hours, impairing swallowing and breathing, especially in those who use accessory (shoulder and upper chest) muscles to assist their diaphragm in breathing.

Also, polio survivors sometimes have adverse reactions – e.g., tachycardia, panic attacks – to the epinephrine that is typically included with the local anesthetic to cause vasoconstriction (narrowing of blood vessels) to prevent the spread of the anesthetic. If additional doses of local anesthetic are required, a preparation without epinephrine may be advisable.

Regardless of whether a local or general anesthetic is used, the following applies:

POST-ANESTHETIC RULE OF 2:

Polio survivors need 2 times as long to recover from the effects of any anesthetic.

Even applying the ‘Anesthetic Rules of 2’ polio survivors may be very sedated, if not asleep, or have their breathing and swallowing impaired for many hours after the surgery. This is one of the reasons why inoffice surgery for complicated dental procedures is not advisable for polio survivors. Sleeping, excessively sedated or facially paralyzed polio survivors cannot be expected to return home and take care of themselves after surgery, since sedation-impaired coordination makes falling likely and complications may go unnoticed. In spite of HMO pressure or usual practice, NO POLIO SURVIVOR SHOULD HAVE IN-OFFICE OR SAME-DAY SURGERY except for the most simple procedures that require only a small dose of local anesthetic that does not compromise breathing or swallowing.

Blood and Guts. There are yet additional concerns. Polio survivors with muscle atrophy, especially in the thigh muscles, will have a smaller blood volume than would be expected for their height or weight. Therefore, excessive bleeding during surgery may be more of a problem. Prolonged gum bleeding is also more likely since many polio survivors are taking the maximum dose of non-steroidal anti-inflamatory drugs. The dentist should be informed before the procedure of all medications the patient is taking, including over-the-counter preparations.

Also, polio survivors can be sensitive to atropine-like drugs used to dry secretions during surgery. [9] Atropine-like drugs also slow the gut, and polio survivors may be excessively constipated after surgery or, rarely, actually have their intestines stop moving (paralytic ileus) for a period of time. These problems can be treated symptomatially as they would in someone who did not have polio.

POST-OPERATIVE CARE

Pain. The single most troublesome problem after surgery is pain control. A number of studies have shown that many surgical patients are under medicated for pain. Under medication is a serious problem for postpolio patients since they are twice as sensitive to pain as those who did not have polio. [8]

RULE OF 2 for PAIN:

Polio survivors need 2 times the dose of pain medication for 2 times as long.

Since polio survivors are known to be extremely stoic, they are not likely to abuse or become dependent upon narcotics.

Vomiting. Another post-op problem related to brain stem damage is vomiting. As in anyone who receives a general anesthetic, polio survivors can develop nausea and vomit. However, polio survivors are more apt to faint (have vasovagal syncope and even brief asystoles) when they attempt to vomit. [9] It is very important that post-operative emetic (anti-vomiting) control be discussed and administered before the procedure and that additional medication is provided as needed post-operatively.

Choking. As has been described, polio survivors may not be able to clear secretions, may choke (or feel like they are choking) and even aspirate if they are lying on their backs, still half asleep, as the anesthetic is clearing. Polio survivors’ secretions need to be monitored after the procedure and they should be positioned on their side so that secretions can drain. [10]

RECOVERY

When polio survivors do awaken from a general anesthetic they may still be twice as sedated as are other patients. Since polio survivors need a very clear head to be able to control their weakened, polio-affected muscles to stand and walk, a fuzzy-headed polio survivor is at serious risk for falling. Polio survivors may also have low blood pressure after surgery that could itself cause lightheadedness, fainting and falls.

RULE OF 2 for RECOVERY:

Polio survivors need 2 times longer to recover than do other patients.

Under any circumstances, polio survivors should get up slowly after the procedure, first sitting up, then getting into a chair with assistance, then standing with assistance and finally walking with assistance and appropriate assistive devices. Polio survivors have learned to be very aware of what their bodies can and cannot do. They are the best judges of when they can move, stand and walk safely.

Post-Op PPS? The 1985 National Survey of Polio Survivors has shown that emotional stress is the second most frequent cause of PPS (physical overexertion being the first). [4] Certainly, there are few emotional or physical stressor more potent then surgery. So, polio survivors should expect some increase in fatigue and muscle weakness resulting from the combination of the physical and emotional effects of the surgery, anesthesia and other medications. However, patients should be reassured that only a small handful of postpolio patients permanently lose function after surgery. Strength or endurance lost after surgery are typically recovered . Polio survivors need to remember:

RULE OF 2 for FEELING BETTER:

Polio survivors need 2 times longer to feel ‘back to normal’ again. CONCLUSION

All of the ‘Rules of 2’ are suggestions for polio survivors, the dentist and oral surgeon; they are not intended as substitutes for specific information about the individual patient, communication between doctor and patient, and clinical judgment. All polio survivors must be evaluated and managed according to their individual needs. Please take the time to read the following references (especially those in bold type) so that you will be fully knowledgeable about and be able to help meet polio survivors’ special needs before, during and after dental procedures.

REFERENCES

1. Bruno RL. Ultimate burnout: Post-polio sequelae basics. New Mobility, 1996; 7: 50-59

[Lincolnshire Library Full Text]

2. Frick NM, Bruno RL. Post-Polio Sequelae: Physiological and psychological overview.

Rehabilitation Literature, 1986; 47: 106 – 111. [Lincolnshire Library Full Text]

3. Bruno RL, Frick NM. The psychology of polio as prelude to Post-Polio Sequelae: Behavior

modification and psychotherapy. Orthopedics, 1991; 14: 1185 – 1193. [Lincolnshire Library Full

Text]

4. Bruno RL, Frick NM. Stress and “Type A” behavior as precipitants of Post-Polio Sequelae. In LS

Halstead and DO Wiechers (Eds.): Research and Clinical Aspects of the Late Effects of

Poliomyelitis. White Plains: March of Dimes Research Foundation, 1987. [Lincolnshire Library

Full Text]

5. Bach JR, Alba AS. Pulmonary dysfunction and sleep disorder breathing as post-polio sequelae:

Evaluation and management. Orthopedics, 1991; 14 : 1329-1337. [Lincolnshire Library Full

Text]

6. Bodian D. Histopathological basis of clinical findings in poliomyelitis. Am J Med. 1949; 6: 563-

578.

7. Bruno RL, Frick NM, Cohen J. Polioencephalitis, stress and the etiology of Post-Polio Sequelae.

Orthopedics, 1991; 14: 1269 – 1276. [Lincolnshire Library Full Text]

8. Bruno RL, Johnson JC, Berman WS. Motor and sensory functioning with changing ambient

temperature in post-polio subjects. In LS Halstead and DO Wiechers (Eds.): Late Effects of

Poliomyelitis. Miami: Symposia Foundation, 1985.

9. Bruno RL, Frick NM. Parasympathetic abnormalities as post-polio sequelae. Archives of Physical

Medicine and Rehabilitation, 1995; 76: 594.

10. Bucholtz DW, Jones B. Post-Polio dysphagia: Alarm or caution. Orthopedics, 1991; 14: 1303-

1305. [PubMed Abstract]

Bruno RL. Preventing complications in polio survivors undergoing dental procedures. PPS Monograph Series. Volume 6(1):1-8. Hackensack: Harvest Press, 1996.

The Lincolnshire Post-Polio Network

Registered Charity No. 1064177

An Information Service for Polio Survivors and Medical Professionals

All enquiries, book requests, medical article requests, membership fees, items for newsletters and donations to

The Secretary, Lincolnshire Post-Polio Network

PO Box 954, Lincoln, Lincolnshire, LN5 5ER United Kingdom Telephone: +44 (0)1522 888601

Facsimile: +44 (0)870 1600840

Email: info@lincolnshirepostpolio.org.uk Web Site: www.lincolnshirepostpolio.org.uk

Post-Polio Network and where applicable, the original author(s).

Document preparation: Chris Salter, Original Think-tank, Cornwall, United Kingdom.

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/dental.html> Created: January 1997

Last modification: 29th January 2010.

Preventing Complications in Dental Procedures Read More »