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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

 

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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 The Lincolnshire Post-Polio Network 1997 – 2010.

 

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/dental.html> Created: January 1997

 

Last modification: 29th January 2010.

Ultimate Burnout: Post-Polio Sequelae Basics

Dr. Richard Louis Bruno

New Mobility, 1996; 7: 50-59

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

It’s 8:00 p.m. and only one light is burning at corporate headquarters. Mr. Gray, MBA, the 55-year-old CEO, is awakened by the phone. He lifts his head from the desk to answer and hears his wife asking when he’s coming home. Feeling as if he weighs a ton, his muscles burning, Mr. Gray wheels himself to the car and, with barely enough strength to pull his chair in behind him, drives home. He greets his kids, rolls into the bedroom, and throws himself on the bed. It’s the third night this week he has gone to bed without dinner and slept in his clothes.

The alarm rings at 5 a.m. A clean suit and a cup of coffee later, Mr. Gray is on the road again. Driving to work, he thinks he hears the same faint “sizzling” sound he hears every morning–a sound like electricity arcing between two frayed wires. But on this and every other morning, he ignores the sizzling, ignores his increasing fatigue and muscle weakness and pain. He has a corporation to run. There’s no time to “give in” to his body. But he has to wonder: “What is that sizzling sound?”

Well, Mr. Gray, that metaphoric sizzling is the sound of your nerves burning out. Nearly 80 percent of America’s 1.63 million polio survivors hear the same sound as they, too, experience Post-Polio Sequelae (PPS). PPS are the unexpected and often disabling symptoms–new fatigue, muscle weakness, joint pain, cold intolerance–that occur about 40 years after the original bout with polio. Unfortunately, most survivors of the polio epidemics that terrorized America during the middle of this century are just like Mr. Gray: hard working, time-conscious, perfectionistic super-achievers who, as a way of life, push themselves beyond their physical limits. Like him, they ignore the sizzling sound, refuse to give up control, refuse to “give in” to new fatigue and weakness and pain even though their bodies are begging them to stop.

Post-Polio Past

Why won’t polio survivors listen to their bodies and slow down? When you understand what it was like to

have had polio, you can appreciate why they feel that “giving in” to PPS is the same as giving up their lives. “During the epidemics,” says researcher Nancy Frick, a polio survivor herself, “polio was America’s summer terror. The adorable March of Dimes poster children–clad in metal braces, leaning on their crutches–were everywhere saying, ‘Give money to find the polio vaccine. Don’t let your child become crippled like me.'”

When the polio vaccine arrived in 1954, the poster kids were needed no more. Their braces, crutches and wheelchairs were evidence of a horror that Americans wanted to forget. “So polio was eagerly forgotten by everyone,” Frick says, “including those disabled by it. To be accepted back into society, polio survivors had to look ‘normal’ again. And since buildings were totally inaccessible, even paraplegic polio survivors had to be able to walk if they wanted to go to school or get a job.”

Polio survivors were ripped away from their families for months or years and admitted to rehabilitation hospitals where they underwent multiple surgeries and draconian physical therapy. Many were verbally abused, slapped or even beaten with rubber truncheons by therapists or family members to “motivate” them to walk. “Is it any wonder that polio survivors discarded their braces and crutches when they came home from the hospital,” asks Frick. “They were eager to do anything to look ‘normal’ and stop the abuse.”

Unfortunately, polio survivors went beyond mere normalcy. Two surveys show that, regardless of the severity of their paralysis, more polio survivors marry and go to college than nondisabled Americans. They work more hours of overtime and take fewer sick days. They became the leaders of their communities and the chief executives of the world’s largest corporations. That’s why their nerves have started to sizzle.

Post-Polio Present

PPS are not caused by the return of the poliovirus that’s been hiding in the body for decades. Nor are they the result of some new disease. The simplest explanation is that polio-damaged neurons are mad as hell and are not going to take it anymore.

When polio struck, large numbers of neurons in the brain stem were damaged by the poliovirus. For those who had paralysis, the poliovirus damaged 90 percent of the neurons in the spinal cord that run the muscles; nearly half of those neurons died. The surviving but damaged neurons are less able to manufacture acetylcholine, the chemical that neurons release to make muscles contract. Yet the damaged neurons sent out sprouts–like extra telephone wires–to turn on muscle fibers orphaned when their motor neurons were killed by the poliovirus. This sprouting allowed many people who were totally paralyzed during the initial polio attack to actually walk out of the hospital some months later.

But since these polio-damaged and over-sprouted motor neurons have been doing double and triple duty for 40 years, even polio survivors with minimal loss of function have been heading for a fall. “Sprouted motor neurons can no longer make muscles contract as forcefully as they once did, or for as long as they once could, because their damaged internal chemical factory is failing after years of overworking,” says Kessler Institute for Rehabilitation physiatrist Jerald Zimmerman. “Muscles become weaker and hurt as they try to do more work than they are able to do. And because weakened muscles can no longer support the joints, polio survivors’ shoulders and elbows ache and their knees start to bend backward.” Standing,

walking, lifting, pushing a wheelchair–sometimes swallowing and breathing, especially in those who had respiratory polio–become more difficult and more painful.

While new muscle weakness and pain are reported by more than 75 percent of polio survivors, the most common PPS symptom is fatigue. Fully 91 percent of polio survivors report two kinds: “body fatigue” and

“brain fatigue .” Body fatigue is the feeling that you weigh a ton and that the longer you use your muscles, the more they lose strength. “Because of new muscle weakness, many polio survivors expend as much energy just getting up and dressed and out to work in the morning as does a marathon runner,” says Lauro Halstead, Post-Polio Program director at Washington’s National Rehabilitation Hospital. “Why wouldn’t they feel fatigued?”

Brain fatigue is the feeling that you can’t focus your attention, can’t concentrate on what you’re reading and can’t stay awake as the day goes on. Researchers at Kessler Institute have recently found that polio survivors with brain fatigue do in fact have significantly impaired attention and are not able to release the brain-stimulating hormone ACTH in response to stress. MRI scans of fatigued polio survivors have found damage in the brain areas that control wakefulness and attention, the same areas that were damaged by the original poliovirus infection.

Post-Polio Future

With the coming of PPS, the future might sound bleak for polio survivors. Both the March of Dimes Birth Defects Foundation and the U.S. Congress have refused to set aside funds for PPS research. “In spite of this, much has been learned about the cause and treatment of PPS,” says Paul Peach, medical director of Georgia’s Roosevelt Warm Springs Institute for Rehabilitation. “The future is much more optimistic than had been expected.” Two recent studies have identified drugs that may decrease PPS fatigue. Neil Cashman of the Montreal Neurological Institute found that Mestinon, a drug that prevents the breakdown of acetylcholine in the muscle, may reduce body fatigue in some polio survivors. A just-completed study from Kessler Institute found that Parlodel, an anti-Parkinson’s disease drug, helped to increase attention and wakefulness and reduce post-polio brain fatigue.

But Peach warns that using drugs to treat PPS may be dangerous. “Weakness and fatigue are the body’s way of telling polio survivors to rest,” he says, “like a fuse that blows when their nerves are overworked.” Peach worries that using these drugs will be like putting a penny in the fuse box , allowing polio survivors to do even more harm to polio-damaged neurons.

But, neither Mestinon nor Parlodel is effective if polio survivors don’t follow The Golden Rule for the treatment of PPS: “If what you do causes pain, fatigue or weakness, don’t do it!”

The Golden Rule does not mean that polio survivors should sit home and be come couch potatoes. The Golden Rule does mean that polio survivors should stop exhausting themselves, especially with regard to exercise. Many survivors are prescribed–or actually ask for–the kind of physical therapy they had right after polio: exercising to the point of exhaustion. But several studies show that pumping iron will not increase the strength of muscles that are becoming weaker, and can actually cause an irreversible loss of strength. No free weights, exercise bikes, tread mills, NordicTracs or Thighmasters for polio survivors. “Feeling the burn” means nerves are burning out.

Stretching, however, helps decrease pain and increase range of motion. And gentle, non-fatiguing exercise can be useful for some survivors to help maintain muscle strength and tone, especially after getting a new brace.

Many studies show that changing survivors’ physically and emotionally stressful lifestyle is the best way to reduce weakness, fatigue and pain. On[e study] by James Agree at the University of Wisconsin shows that polio survivors who pace activity–that is work and then rest for an equal amount of time–can do 240 percent more work than if they push straight through the task. Another study shows that survivors use three times less energy in walking if they use a short brace on a weakened leg. And the follow-up study of

Kessler’s six-week program of behavior modification and physical and occupational therapy shows that polio survivors who comply with treatment –pace activities, conserve energy, take two 15-minute rest breaks a day, and use assistive devices (a brace, cane, crutches, wheelchair or scooter)–have up to 22 percent less pain, weakness and fatigue 18 months after therapy ends. But survivors who quit therapy have 21 percent more fatigue and 76 percent more weakness 18 months after leaving the program.

And there’s the rub. It’s only the refusal to listen to that sizzling sound that causes PPS to continue or progress. That’s why behavior modification and psychotherapy are required to help survivors accept their second bout with polio, change their super-achieving lifestyles and deal with the fear of looking disabled. “There will be no magic pill to cure PPS,” says Paul Peach. “But by making lifestyle changes and eliminating ‘overuse abuse,’ progressive fatigue, weakness and pain can be substantially reduced or eliminated.”

As Mr. Gray knows well, this whole PPS business stinks. But isn’t it far better to accommodate to PPS now than to be forced to give up your entire life because you are too weak and fatigued to function? I know, you’ll slow down and take care of yourself “when you’re ready.” You’ll use a wheelchair “when

there’s no other choice.” Well, you don’t drive your car until it’s out of gas. Why drive your body until it’s out of neurons? Isn’t it time to listen to the sizzling sound? Isn’t it time to take care of yourself? To paraphrase one famous paraplegic polio survivor who refused to take care of himself, “You have nothing to fear but fear itself.”

Red Flags for Polio Survivors

[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. The lesson: Don’t let a doctor tell you it’s all in your head just because you didn’t have paralytic polio.

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 time after surgery or an injury. However, survivors have had to develop an extremely high pain tolerance to deal with their increased pain sensitivity and are not likely to become addicted to pain medications.

Sleep: The majority of 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.

Resources

Orthopedics, November and December, 1991.

These special issues provide all the information needed to start a PPS clinic. A must read for anyone who treats polio survivors.

Harvest Center, Inc., 151 Prospect Ave., Hackensack, NJ 07601; (201)342-6777. Provides the 1995 Audio Symposium for PPS, four audio taped lectures on the cause and treatment of PPS and the psychology of polio. Also available is the Computerized Assessment Program for PPS. After a brief questionnaire, the computer suggests possible causes, tests and therapies for new symptoms and lists the world’s medical literature on PPS.

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 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/burnout.html>

Created: January 1997

Last modification: 29th January 2010.

FAINTING AND FATIGUE: Causation or Coincidence Richard L. Bruno, Ph.D.

 

Bruno RL. Fainting and Fatigue: Causation or Coincidence? CFIDS Chronicle, 1996; 9(2): 37-39.

 

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

 

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

 

Saddle Brook, New Jersey USA

 

As the former autonomic nervous system fellow at New York’s Columbia-Presbyterian Medical Center, and in my current incarnation studying chronic fatigue in polio survivors, I have read with special interest the reports from Johns Hopkins University describing neurally mediated hypotension (NMH) in adults and adolescents with CFIDS.[1] In June 1995, we presented a paper to the American Congress of Rehabilitation Medicine describing several of our post-polio patients who have had episodes of vasovagal syncope.[2] One patient with a 10-year history of severe, chronic and disabling post-polio fatigue had a history of frequent fainting 35 years before she ever experienced fatigue.

 

The 1995 North American Post-Polio Survey

 

Because of fainting episodes in our patients, we included questions about fainting and late-onset fatigue in the 1995 North American Survey of Polio Survivors conducted last Fall. We have recently analyzed data from the first 1,000 respondents to the survey. As was seen in our two previous National Post-Polio Surveys, chronic fatigue was the most frequent symptom in polio survivors; the daily fatigue rating was reported as “moderate” or higher in 76 percent of the 665 polio survivors, versus 29 percent of the 406 non-disabled controls.

 

An equal number of polio survivors and controls (44%) reported at least one fainting episode during their lifetimes. The causes of fainting – from physical trauma to emotional stress – were identical in polio survivors and controls. However, the occurrence of even one fainting episode was significantly related to having a daily fatigue rating of moderate or greater in both polio survivors (X2=7.46; p=.0063) and in

 

controls (X2=7.74; p=.005). Polio survivors who passed out at least twice in their lives had a 14 percent higher daily fatigue rating (p=0.0014) and controls had a 31 percent higher daily fatigue rating (p=0.019) as compared to those who never had fainted. These finding suggest that there may be a relationship between fainting and fatigue in polio survivors and the general population, as well as in people with CFIDS.

 

Physiology of Fainting and NMH

 

The question raised by our data and those from Johns Hopkins is: why do fainting, NMH and fatigue appear to go together? The answer may lie in the physiological mechanisms that regulate blood pressure and actually trigger NMH.

 

NMH has been said to result from a “miscommunication between the heart and the brain which causes blood pressure to lower when it should rise.”[3] Actually, NMH is a survival mechanism, hard-wired into the brain, that turns on when the autonomic nervous system (ANS) is unable to stop blood pressure (BP) from falling. If BP falls for any reason (e.g., loss of blood from a cut artery) pressure detectors in the carotid arteries of the neck (the baroreceptors)stop firing.[4] (Figure 1) The silence of the baroreceptors is “heard” by BP regulation centers in the solitary tract and dorsal vagal nuclei, which release the brake on heart rate that is normally provided by the cardiodepressor center of the reticular formation. The BP regulation centers also trigger the release of vasopressin and norepinephrine, neurochemicals that constrict blood vessels and make the heart beat harder and faster, and should help to increase BP.

 

However, if these measures cannot stop BP from falling, the amount of blood returning to the heart will decrease. If the heart continues beating hard and fast even though it is nearly empty, the heart muscle will be damaged. Stretch receptors in the heart detect that it is “beating on empty,” and send a signal to the dorsal vagal nucleus to protect the heart by stopping its beating for a few seconds. With the heart stopped, BP falls to zero and causes the faint of NMH.

 

But, even fainting has a protective function (if you don’t knock yourself out falling to the ground). Lying flat, your brain will be at the same level as your heart. Without gravity pulling blood toward your feet, the heart will have an easier time increasing BP, pumping your remaining blood to your brain and (hopefully) waking you up again.

 

A Physiological Link Between Fainting and Fatigue.

 

If we assume that the heart itself is normal, there must be something wrong with the BP regulation centers in the brainstem that causes fainting in our post-polio patients and NMH in people with CFIDS. In the case of polio survivors, damage done by the poliovirus to the brain suggests that fainting should be expected.

 

The poliovirus frequently and severely damaged the brainstem, especially the reticular formation (RF).[5] The RF contains the cardiodepressor center that is responsible for “putting the brake” on heart rate. Of those with acute “bulbar” polio (“bulbar” meaning associated with the brainstem) 73 percent had hypertension and rapid heart rate and five percent died of cardiovascular collapse.[6]

 

Importantly, the RF is not only responsible for heart regulation, but also brain activation, keeping us awake and allowing us to focus attention. Using MRI of the brain, we have found lesions of the RF and its connections to the cortex, as well as clinically impaired attention on neuropsychological tests, in polio survivors with chronic fatigue.[7] Recently, Costa, et al. found that decreased perfusion of the brainstem

 

on SPECT was the only physiological finding that differentiated people with CFIDS from patients with depression, neurological disease and controls.[8] These data support our hypothesis that damage to the brainstem “reticular activating system” may be a cause of chronic fatigue in polio survivors and in patients with post-viral fatigue.[7]

 

Outside of the RF, but in the same area of the brainstem, lie the other cardiovascular control centers. The dorsal vagal nucleus, responsible for activating the stomach and slowing the heart, was damaged in 55 percent of those who had bulbar polio, as were the vestibular nuclei. Nausea, reported by 70 percent of those who have NMH, was seen in 27 percent of those with acute polio and attributed to poliovirus lesions in the vestibular nuclei.[1,5,6]

 

Also outside the RF but damaged by the poliovirus, were the nucleus ambiguus and solitary tract nuclei, the main centers for blood pressure regulation. These nuclei “communicate” with the heart via the vagus nerve, regulating blood pressure by sensing and altering the force and rate of the heart’s contraction.[4]

 

The Chemistry of Fatigue, Fainting and NMH.

 

Recent research has also found chemical abnormalities that may be related to fatigue and fainting in polio survivors and CFIDS and NMH. We found a marked blunting of adrenocorticotropic hormone (ACTH) release in polio survivors that was correlated with daily fatigue severity.[7] The secretion of corticotropin releasing hormone (CRH), which stimulates ACTH release, had already been found to be decreased in patients with CFIDS.[9] CRH is secreted by the paraventricular nucleus (PVN) of the hypothalamus, which is known to be damaged by the poliovirus.[5] Both ACTH and CRH are brain activating hormones, and their decreased secretion could cause symptoms of fatigue.[7]

 

The PVN of the hypothalamus also produces the vasoconstrictor neurochemical vasopressin, the secretion of which is also impaired in people with CFIDS.[10] Thus, the PVN may be damaged in polio survivors and people with CFIDS, causing impaired brain activation and faulty BP regulation in both groups.

 

The Coincidence of Fainting and Fatigue

 

Taken together, these findings suggest that polio survivors may be predisposed to fainting because of poliovirus damage to their brainstem cardiodepressor and blood pressure regulation centers and PVN. However, it is important to note that many viruses besides polioviruses (e.g., the Coxsackie viruses) are also known to frequently and preferentially damage the brainstem, especially the reticular formation. [7,11] People with post-viral fatigue may have similar brainstem and hypothalamic damage as is seen following poliovirus infection, as Costa’s finding of decreased brainstem perfusion in CFIDS suggests. Thus, damage to cardioregulatory centers could be responsible for NMH in people with CFIDS. But what of the coincidence of fainting and fatigue?

 

Since the cardioregulatory centers are either part of or lie close to the reticular activating system, it would be surprising if fainting and fatigue did not occur together. Damage to the RF alone could both decrease brain activation and impair BP regulation. The question that remains is whether NMH is the “cause” of chronic fatigue or if it is just coincidental in those who have chronic fatigue. At this early stage in the research, the answer to both of these questions appears to be “yes.”

 

The Johns Hopkins team reported that 47 percent of their adult CIFDS patients and 57 percent of their adolescent patients with NMH had “complete or near complete resolution of all symptoms” after receiving blood pressure elevating therapies. Could the resolution of fatigue symptoms in CFIDS patients whose

 

NMH was treated have resulted from elevated BP increasing brainstem perfusion, thereby enhancing the functioning of the reticular activating system, or by elevated BP directly increasing perfusion of the brain? SPECT scans in people with CFIDS whose symptoms disappear when their BP is increased could answer these question.

 

But what of the nearly 50 percent of patients whose CFIDS symptoms did not respond or were only “somewhat better” after NMH treatment? Could NMH in these individuals just be a coincidence of generalized damage to the brainstem, including the BP regulation centers? Could fatigue symptoms in these individuals actually result from damage to the brainstem’s reticular activating system, or the hypothalamus or the immune system? Only blinded, placebo-controlled trials of treatments for NMH, combined with SPECT scans of the brainstem, measurement of CRH, norepinephrine, and vasopressin, and assessment of the individual blood pressure and heart rate regulation pathways to and from the brain stem will provide answers to these important questions.

 

Acknowledgements:

 

Many thanks to Rebecca Moore for her stimulating questions and suggestions.

 

REFERENCES

 

1. Bou-Holaigah, et al.: The relationship between neurally mediated hypotension and the chronic

 

fatigue syndrome. JAMA 1995;274:961-967. [PubMed Abstract]

 

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

 

Rehab 1995;76:594.

 

3. Carpman V: News, knowledge & networking. CFIDS Chron 1995;8(4):22.

 

4. van Lieshout JJ, et al.: The vasovagal response. Clin Sci 1988;81:575-586. [PubMed Abstract]

 

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

 

6. Baker AB: Neurologic signs of bulbar poliomyelitis. Poliomyelitis Philadelphia: Lippincott, 1949.

 

7. Bruno RL, et al.: The pathophysiology of a central cause of post-polio fatigue. Ann NY Acad Sci

 

1995;753:257-275. [Lincolnshire Library Full Text]

 

8. Costa DC, et al.: Brainstem perfusion is impaired in chronic fatigue syndrome. Q J Med

 

1995;88:767-773. [PubMed Abstract]

 

9. Demitrack MA, et al.: Evidence for impaired activation of the HPA axis in patients with chronic

 

fatigue syndrome. J Clin Endocrin Metab 1991;73:1124-1234. [PubMed Abstract]

 

10. Bakheit A, et al.: Abnormal arginine-vasopressin secretion and water metabolism in patients with

 

postviral fatigue syndrome. Acta Neurol Scand 1993;87:234-238. [PubMed Abstract]

 

11. Bruno, et al.: The physiology of post-polio fatigue: A model for post-viral fatigue syndromes and a

 

brain fatigue generator. CFIDS Chron1994;7(4):36-42.

 

Figure 1.

 

Brain stem centers damaged by the poliovirus involved in brain activation and blood pressure and heart rate regulation.

 

Figure not available

 

Figure 2.

 

Brain areas damaged by the poliovirus involved in brain activation and blood pressure and heart rate regulation. PVN: Paraventricular nucleus of the hypothalamus; ACTH: Adrenocorticotropic hormone produced by the pituitary; CRH: Corticotropin releasing hormone, produced by PVN, that

 

releases ACTH; VP: Vasopressin, produced by PVN, and released from the pituitary.

 

Figure not available

 

Figure 3.

 

Possible mechanisms of fatigue symptom generation. Damaged reticular formation neurons may decrease reticular activating system (RAS) stimulation of the brain, in combination with reduced CRH and ACTH secretion. Damaged blood pressure regulation centers, in combination with reduced vasopressin secretion, may produce NMH, decrease blood pressure, and reduce the perfusion of the RAS and brain, resulting in decreased brain activation

 

Figure not available

 

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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/harvest/faint.html>

 

Created: 20th January 1997

 

Last modification: 29th January 2010.