PSN Library Files – Page 10 – Polio Survivors Network

Health and Demographic Characteristics of Polio Survivors

Health and Demographic Characteristics of Polio Survivors

Sandra S. French and G. Sam Sloss

Associate Professors of Sociology at Indiana University Southeast

A Lincolnshire Post-Polio Library Publication – April 1999

Since 1985, the Louisville and Ashland, Kentucky chapters of the Polio Survivors Organization have collected questionnaire data from polio survivors. The questionnaires included data on:

Social and demographic data – e.g., age, sex, education, and employment history Polio history – e.g., dates, types, and treatments

Current health problems – e.g., fatigue, depression, and breathing problems

Two graduate students¹ keyed approximately half (N=295) of these questionnaires into an SPSS data file. While most respondents are from the Midwest, the sample is national in scope. Respondents include people from the east coast to the west coast and from states bordering both Canada and Mexico. The 295 respondents live in 202 different zip code areas with no more than three people from any one zip code. Below we report on the data from this sample of polio survivors. Due to changes in the questionnaires over time (e.g., marital status is not asked in all versions) and missing data from some questionnaires, the N for most tables is fewer than 295.

Social and Demographic Characteristics of the Sample

Comparing our sample with the 1990 Post-Polio Sample reported on by Bruno and Frick (1991), we find similar demographic profiles (Table 1). Respondents ranged from 29 to 85 years in age, with a mean age of fifty-three (53) and a median age of fifty (50). The female/male ratio was 1.7 with eighty-seven (87) percent of the sample either currently or previously married. While the reported educational attainment of our sample is lower than the 1990 National Post-Polio Survey, it is still higher than both the disabled and non-disabled groups. Over half had some college with one in four reporting a bachelors’ degree and another fifteen (15) percent with a graduate degree.

Table 1
Characteristic	Current Survey	1990 National Post-Polio Sample²
Characteristic	Current Survey	Post-polio	Disabled	Non-disabled
Age (Mean)	53	54	—	47

Female/male ratio	1.7	1.7	—	1.5
Marital Status
Single	13%	11%	13%	22%
Married	70%	71%	56%	58%
Other (divorced, widowed, separated)	17%	18%	31%	20%
Education
High School or below	44%	29%	71%	52%
More than High School	56%	71%	29%	48%
² Data reported in Bruno and Frick, 1991.

Most people in our sample participated in the paid labor force (Table 2). As we might expect from a population with above average education and physical handicaps, polio survivors were under represented in blue collar occupations and over represented in professional occupations.

Table 2. Work Experience
Work Experience Percent		N
Never	2.7	7
Blue collar	10.8	28
Clerical	25.0	65
Manager	8.5	22
Professional	26.2	68
Other	26.9	70
Total	100.0	260

The Polio Experience

This sample contracted polio between the years of 1903 and 1975. Prior to the one case in 1975, the most recent year was 1963. The most frequent year for contracting polio was 1952 which represented thirteen (13) percent of the sample. Half contacted polio during the nine year period from 1947 to 1955.

The questionnaire asks respondents what “type” of polio they contracted, providing responses of cerebral, bulbar, spinal, respiratory and don’t know. While classifications are not based on the type of virus but on the area of the body affected, half reported not knowing where they were affected. Of those who knew, spinal was reported most, striking over a third of the sample.

One year or less was the modal age for contacting polio (14.6%) with the next most frequent ages being two, three and four in that order. Nearly half were five or younger and three of four were fifteen (15) or

younger. Less than five (5) percent were over thirty (30). On average, respondents filled out the questionnaire forty-five (45) years after first contracting polio.

Table 3. Type of Polio
Type of Polio	Percent*	Number
Cerebral	3.1	9
Bulbar	14.9	44
Spinal	36.3	107
Respiratory	9.8	29
Don’t know	44.4	131
* Over 100% as several had more than one type.

Polio struck people about equally on the right and left sides of their bodies. Legs were affected more than arms (see Table 4).

Table 4. Body Area Affected by Polio
Area Affected	Percent*	Number
Left side	81.6	191
Right side	85.7	203
Legs	92.5	222
Arms	48.9	116
* Over 100% as several had more than one type.

Three fourths of the sample was hospitalized. The length of stay in the hospital ranged from one month to 10 years. Nearly a third spent up to six months with one in five spending between one and two years and the final fifth spending more than two years hospitalized. Three people reported being hospitalized for ten years.

Table 5. Hospital Experience
Length of hospitalization	Percent
Not hospitalized	26.3
1 to 6 months	31.4
1 year to 2 years	21.2

More than two years

21.2

Most survivors report receiving some form of physical therapy (Table 6). Half report being treated with Kenny Hot Packs. Six percent were in tank respirators with another six percent on another type of respirator. Very few received psychiatric attention. While the questionnaire did not ask if any respondents received the polio vaccine before they contracted polio, more that two thirds (68.8%) report taking the vaccine after having polio.

Table 6. Type of Treatment
Type of Treatment	Percent*
Physical therapy	82.2
Kenny hot packs	56.3
Occupational therapy	13.1
Psychiatric help	3.6
Respiratory therapy	7.1
Tank respirator	5.6
Other respirator	9.6
Rocking bed	3.2
* Over 100% as several had more than one type.

Polio survivors were left with varying degrees of physical problems, with weakened muscles the most common (see Table 7). For many the weakened muscles required they use a variety of aids for mobility.

Table 7.
Physical condition	Percent*	N
Weak muscles	67.2	180
Paraplegic	21.6	59
Quadriplegic	5.5	15
Ambulatory	19.2	52
Wheelchair	17.2	47
Braces	42.5	116
Respiratory Aids	4.4	12
* Over 100% as several had more than one type.

The questionnaires included two symptom checklists. The first list asked respondents “Have there been ANY changes in your physical condition?” They were instructed to “Check any or all that apply.”

Respondents were then asked to list the problems from the list for which they sought medical advice (Table 8). The instructions for the second symptom checklist read, “In the past few years, have you experienced any of the following?” (Table 9).

Over ninety (90) percent reported at least one recent health problem. Like the 1985 and 1990 National surveys, this sample reported fatigue to be their most common new health problem. On both lists approximately eighty (80) percent report suffering from this ailment. A close second on each list is weak muscles. Items pertaining to sleep, circulatory problems and numbness were checked by nearly half the respondents. Other items reported by significant percentages of the sample include gastrointestinal problems, breathing and respiratory problems, scoliosis, and urinary problems. While most of the problems are physical, significant numbers checked psychological problems of depression, anxiety and personality changes.

Table 8. Checklist 1: Any changes and if medical help was sought for the problem.
Type of change?	Percent	Med help?
Any changes	91.2	71.3
Shortness of breath	42.8	8.4
Headache	41.1	13.3
Fatigue	78.1	20.0
Dizziness	25.5	7.9
Brittle bones	9.6	4.2
Fractures	10.7	5.8
Anxiety	33.6	7.9
Blacking out	5.2	3.6
Scoliosis	30.4	9.7
Personality change	18.5	4.2
Gastrointestinal	38.0	13.3
Depression	39.1	12.7
Interrupted sleep	47.6	12.7
Need to sleep	41.3	12.1
Numbness	48.0	12.1
Respiratory infection	24.3	7.9

Weak muscles	67.2	32.7
Other	42.9	50.3

Table 9. Checklist 2 – Percent experiencing problem in past few years.
Symptom	Percent
Increased fatigue	83.9
New muscle weakness	81.1
Muscle/joint pain	78.6
Scoliosis	44.4
Depression	51.0
Numbness	55.3
Gastro-intestinal	51.9
Ambulatory	67.9
Respiratory	36.3
Circulatory problems	50.0
Arthritis	55.3
Insomnia	54.4
Night sweats	36.7
Nightmares	18.0
Night breathing/choking	26.4
Hoarseness	26.5
Increased thirst	32.6
Urinary problems	35.9
Memory problems	33.9

Forty (40%) were currently under treatment for a health problem. Seventy-two (72%) percent suspect their recent problems are polio related. When asked where they had heard about post-polio problems, the most common response was from a newspaper article (see Table 10).

Table 10. Media Source Suggesting Polio

Source of Suspicion	Percent	N
Television	26.1	41
Radio	5.9	9
Newspaper	60.5	95
Magazine	26.6	41

Nearly three fourths of the polio survivors experiencing a new problem suggested to their physician that the problem might be polio related (Table 11). Respondents report six of ten physicians were receptive to their suggestion. Nearly half report their physicians connected the symptoms with polio. While we can understand why only one in four physicians had previous experience treating polio patients, it was disappointing to find only one in five reported their physicians sought additional information for them. Less than a third of the physicians recommended a rehabilitation evaluation.

Table 11. Interactions with Physicians
Interactions with Physicians	Percent
Did you suggest to physician polio was cause of problem?	73.3
Was physician receptive?	61.9
Did physician suggest polio was cause?	47.1
Had physician treated polio in past?	20.9
Did physician seek information for you?	21.8
Did you have a rehabilitation evaluation?	27.7

Sex Differences

There were no sex differences by type of polio, age of contracting polio, or educational attainment. Polio weakened and crippled both males and female at similar rates. Treatments were similar except females were more likely (61% to 46%) to have been treated with Kenny hot packs.

As we might expect, females were more likely to work in clerical positions. Similarly, females report seeking medical advice more often (76% – 63%) than men.

There were few reported sex differences in current health problems. Of the problems listed in tables 8 & 9, females report more fatigue, while males report slightly higher rates of memory problems and personality changes. Weaker muscles in women are found in the general elderly population (Litt, 1998) so this is not likely to reflect a differential impact by polio. The most interesting finding is that there are virtually no differences in health problems by sex.

Age Factors

T-tests between those experiencing and not experiencing specific health problems revealed few differences in the age each group contracted polio. Those experiencing anxiety and scoliosis were slightly

younger than those not reporting these problems. On the other hand, those suffering from weak muscles and ambulatory problems were slightly older than those not experiencing these problems. The largest age difference was between those hospitalized for polio (11.7) and those not hospitalized (4.8 yrs.).

As we might expect, those who had polio more recently were more likely to have been hospitalized. Contrary to what we might expect, symptoms did not become greater the longer it had been since one was first infected with polio. Of all the problems listed in tables 8 & 9, only those reporting urinary problems had polio in the more distant past. On the other hand, those reporting problems with headache, fatigue, need to sleep, muscle/joint pain, depression, numbness, and memory problems had polio more recently.

The major issue is which health problems are due to Post Polio Syndrome and which to normal aging. Overall we found few differences in current age between those experiencing symptoms and those not experiencing symptoms. When differences do exist, those reporting the problem are younger than those without the problem. Those reporting problems with headaches, personality change, sleep problems and depression, were slightly younger than those not reporting such problems.

Summary and Conclusions

The survey data collected by the Polio Survivors Organization is consistent with and supports research findings from previous populations of polio survivors. While polio survivors have had to deal with a variety of physical disabilities, they are at least as likely to marry as the general population, have been actively involved in the labor force and have above average educational attainment.

Thirty to forty years after being afflicted with polio, many survivors are experiencing new medical problems that appear to be related to their earlier polio experiences. While many polio survivors have sought medical treatment for some of their problems, many physicians have not had experience treating polio patients.

Acknowledgements

We want to thank the Polio Survivors Organizations of Louisville and Ashland, Kentucky for collecting the data. We would also like to thank Indiana University Southeast for funding our Summer Faculty Fellowships that provided us the time to analyze the data.

¹Special thanks to Lee Bruce and Alice Wilson for work on this project.

For more information please contact Sandra S. French (sfrench@ius.edu) or G. Sam Sloss at (gsloss@ius.edu).

References

Bruno, Richard L. and Nancy M. Frick. “The Psychology of Polio as Prelude to Post-Polio Sequelae: Behavior Modification and Psychotherapy.” Orthopedics. V.14:11. November, 1991. 1185-1193. [Lincolnshire Library Full Text]

Litt, Iris F. 1998. “Health Issues for Women in the 1990’s.” in Dina L. Anselmi and Anne L. Law. Questions of Gender. Boston: McGraw Hill. 690-701.

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.

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/usa/kentuckysurvey.html> Created: 14th April 1999.

Last modification: 31st January 2010.

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Dr Henry writes about Polio Literature

Dr. Henry writes about Polio Literature

As an infectious disease, historical evidence would indicate that polio has been in existence for over five thousand years. However, over these many centuries, the literature on polio has been limited. In this century there has been more in the medical literature about polio as a result of the epidemics that began in the early part of this century. With the development of the Salk and Sabin vaccines, the medical literature regarding polio quickly vanished. Now, with the reality of Post-Polio Syndrome (PPS), the literature, both medical, non-fiction and fiction has increased in the last decade. In my opinion, this literature has resulted for two reasons. One is due to PPS and the other is due to books that have been written by polio survivors and family members of polio survivors. In our support group’s library, we have many of the medical books on PPS, but we do not have many of the non-medical books. Listed elsewhere in this newsletter (see below) are some of the books with a polio or PPS connection. This is by no means a complete list. I have read some of these books, but certainly not all of them.

As complete as this list seems to be, a connection to polio still occurs in literature or the arts when I least expect it. Last fall, I audited a course on Job at a local seminary. The course dealt with “Job in the Bible and Beyond” and how the story of Job has influenced the arts down through the centuries. On one evening, the professor was showing slides of art that best depicted the suffering of Job. One of the slides was a painting by Piero della Francesca in Arrezzo Italy. The painting revealed a recently resurrected Jesus, half in and half out of the grave. There was still some of the look of death on Jesus’ body. At this point, the professor read a passage from a novel, Crossing to Safety by Wallace Stegner.

“But I noticed that Sally stood a long time on her crutches in front of that painting propped temporarily against a frame of raw two-by-fours. She studied it soberly, with something like recognition or acknowledgement in her eyes, as if those who have been dead understand things that will never be understood by those who have only lived.”

In the novel, Sally is a polio survivor who wears leg braces and uses crutches. She and her husband Larry become life long friends with Sid and Charity when both husbands are young college professors at the University of Wisconsin in the late 1930’s. Both wives get pregnant at the same time. While vacationing in Vermont, Sally is stricken by polio. Years later, the two couples travel to Italy and the scene described above occurs. One interpretation offered by this scene is that many polio victims can identify with Job or the suffering of the crucified Jesus. Many polio survivors have risen to full lives despite the damage of polio. However, many can recall the fear and even horror of a near death experience. We may tend to forget that thousands of children died from acute polio, especially before the development of the iron lung. This disease has shaken thousands of families. Many of us do “understand things that will never be understood by those who have only lived.”

I can identify with many interesting passages in this novel. Larry, Sally’s husband, writes this novel in the first person. Larry comments on the uncertainty of life with these words: “You can plan all you want to.

You can lie in your morning bed and fill whole notebooks with schemes and intentions. But within a single afternoon, within hours or minutes, everything you plan and everything you have fought to make yourself can be undone as a slug is undone when salt is poured on him. And right up to the moment when you find yourself dissolving into foam you can still believe you are doing fine.” This sounds a bit likes the process of PPS. Many of us have “fought” to be contributors and not burdens to our individual worlds, only now to feel undone by the “salt” of PPS.

Again Larry speaks of the friendship that has developed over the years between his wife Sally and Charity. “The cant word these days is ‘bonding.’ I suppose some people see in a relationship like that signs of an unacknowledged lesbianism—the same people who probably speculate about the sex life of somebody like me, a perfectly healthy man with a crippled wife. I don’t care how they speculate, or what their answers are. We live as we can, we do what we must, and not everything goes by either Freudian or Victorian patterns. What I am sure of is that friendship—not love, friendship—is as possible between women as between men, and that in either case it is often stronger for not having to cross sexual picket lines. Sexuality and mistrust often go together, and both are incompatible with amicitia.”

Near the end of the novel, Larry writes of his observations upon living his adult life with his polio disabled wife.

“One of the peculiarities of polio is that its victims, once they have recovered from the virus and settled down to whatever muscular control it has left them, live a sort of charmed life. Crippled as they are, they are rarely ill, they are surprisingly tough and durable, they astonish their sound companions with their capacity to endure. But that is not forever. There comes a time in the life of every such patient when the whole system—muscles, organs, bones, joints– -begins to fall apart all at once, like the wonderful one hoss-shay. Every polio patient is warned to expect that time, every polio family lives with the foretold doom waiting for it at some unknown but expected time in the future. One learns to live with it by turning away from it, by not looking. And yet on occasion one is aware of an intense, furtive watchfulness, and the victim, the doomed one, must surely have just as often the vulnerable sense of being watched.”

In my mind, the above paragraph is describing PPS. The author of this novel, Wallace Stegner, lived from 1909 to 1993. Crossing to Safety was published in 1987, around the time PPS was being identified as an entity. Stegner has written many novels and wrote this one after fifty years of writing. I wonder how he knew so much about the character of polio. Perhaps, he had some personal experience with polio. If any of you know, please let me know. In his career, Stegner won the Pulitzer Prize, the National Book Award, and O. Henry prizes.

Henry Holland, Richmond., Virginia, USA. Henry4FDR@aol.com

April 1999

Originally published in the Central Va PPS Support Group (PPSG)‘s newsletter, The Deja View, in 1999. Polio Books.

EDITORIAL NOTE: As an indication of availability we have checked two major US Online Bookstores for each title and provided a link to the title listing. Where both hardback and paperback editions are available we have linked to the paperback entry (alternative hardback and library binding editions may be available). These links may alter in time so we have also included the entry page for each of the three bookstores form which you will be able to search for author or title. Unless you already have a preferred online bookstore we recommend that you check a reasonable cross section of stores as not only

availability varies but also pricing and delivery cost/time. Some titles may also be published under separate imprints in different countries so if you live in the UK for example, it may be quicker to order the UK version from a UK bookstore although it may not necessarily be cheaper. For an extended list of Online Bookstores see our Book Store List.

^{http://www.amazon.com/}http://www.barnesandnoble.com/

Primary Reference: Crossing to Safety by Wallace Stegner. Penguin Books. New York. 1987, pp. 274- 275, 201, 288, 336.

[Amazon]

Small Steps: The Year I Got Polio by Peg Kehret, (Children) reading level, grades 4 to 6. (Non fiction) 1996.

[Barnes and Noble]

Close to Home: A Story of the Polio Epidemics by Lydia Weaver, Aileen Arrington. (Non-fiction) 1997.

[Amazon]

A Paralyzing Fear by Nina Seavey, Jane Smith, Paul Wagner. (Medicine) 1998. (This is the book that accompanies the film of the same name).

[Barnes and Noble]

Managing Post-Polio: A Guide to Living Well with Post-Polio Syndrome edited by Lauro Halstead and Naomi Naierman. (Medicine) 1998.

[Lincolnshire Library Booklist]

Dirt and Disease, Polio Before FDR by Naomi Rogers. (Medicine) 1994.

[Amazon]

In the Shadow of Polio by Kathryn Black. (Diseases) 1997. (the author’s mother died of polio when the author was a child).

[Barnes and Noble]

Polio edited by Thomas McDaniel and Frederick Robbins. (Diseases) 1999. (nine essays written by polio survivors and caretakers).
Post-Polio Syndrome edited by Lauro Halstead and Gunnar Grimby. (Medicine) 1995. [Barnes and Noble]
A Summer Plague by Tony Gould. (Non-fiction) 1997.

[Amazon] [Barnes and Noble]

Breakfast at the Liberty Diner by Daniel Kirk. (Fiction) 1997. (a children’s book about a boy with a brace from polio and his meeting with President Roosevelt at the Liberty Diner, has illustrations). [Amazon] [Barnes and Noble]
Flexible Bodies, Tracking Immunity in American Culture from the Days of Polio to the Age of AIDS by Emily Martin. (Diseases) 1995.

[Barnes and Noble]

The Golden Handicap, A Spiritual Quest, A Polio Victim Asks “Why” and Turns His Life Around by Garrett Oppenheim and Gwen Oppenheim. (Psychology) 1993.

[Barnes and Noble]

Hidden Dangers in Polio Vaccines by Elanor McBean. (Medicine) 1993.

[Barnes and Noble]

Jonas Salk by Carmen Bredeson. (Juvenile biography) 1993.
Polio, A Special Ride by Diane Arlene Hawksford. (Diseases) 1997.

[Barnes and Noble]

Post-Polio Syndrome by Theodore L. Munsat. (Medicine) 1991.

[Barnes and Noble]

Post-Polio Syndrome by Marinos C. Dalakas, Harry Bartfield, and Leonard T. Kurland. (Medicine) 1995.

[Amazon] [Barnes and Noble]

Oh God, Help Me! I Cannot Help Myself, A True Story of Polio in the Life of a Polio Survivor by John Earl Lindell and Ethel Brooks. (Religion) 1988.

[Barnes and Noble]

Passage Through Crisis: Polio Victims and Their Families by Fred Davis. (Medical Ref) 1991. [Barnes and Noble]
Post Polio by Betty Garee. (Medicine) 1987.

[Barnes and Noble]

Through the Storm, A Polio Story by Robert F. Hall. (Medicine) 1990.

[Barnes and Noble]

Blackbird, Fly Away by Hugh Gallagher. (Non-fiction) 1998.

[Amazon]

A Nearly Normal Life by Charles L. Mee. (Essays) 1999.

[Barnes and Noble]

Patenting the Sun, Polio and the Salk Vaccine by Jane S. Smith. (Science) 1990. [Amazon]
Hero of Lesser Causes by Julie Johnston. (Fiction – juvenile).

[Amazon] [Barnes and Noble]

Jonas Salk by Majorie Curson. (Non-fiction – juvenile).

[Amazon] [Barnes and Noble]

The Molecular Biology of Poliovirus by Friedrich Koch. (Science).

[Barnes and Noble]

Measles and Poliovirus by Edward Kurstak. (Medicine).
Polio’s Legacy: An Oral History by Edward Sass. (History).

[Amazon] [Barnes and Noble]

Contagious and Non-Contagious Infectious Diseases by Karen Bellenir. (Diseases). [Barnes and Noble]
Gold in the Garden by Dorothy Harrison. (Fiction – juvenile).
Jonas Salk by Victoria Sherrow. (Medicine).

[Barnes and Noble]

Healing Warrior: A Story About Sister Elizabeth Kenny by Emily Crofford. (Non-fiction – juvenile – biography).

[Barnes and Noble]

Time and Place by Bryan Woolley. (Fiction).

[Amazon]

If It Weren’t for the Honor, I Would’ve Rather Walked by Jan Little. (Health). There appears to be two versions of this title.

1st version: [Barnes and Noble]

2nd version: [Barnes and Noble]

The Cripple Liberation Front Marching Band Blues by Lorenzo Milam. (Health). [Amazon] [Barnes and Noble]
Handbook on the Late Effects of Poliomyelitis for Physicians by Gini Laurie. (Medicine).

[Barnes and Noble]

Scientist and Strategist, June Rooks by Mary Verheyden-Hilliard. (Health). [Barnes and Noble]
God’s Green Liniment by Lois Joh Rew. (Health).

[Barnes and Noble]

Little Billy by Carolyn Baber.

[Barnes and Noble]

Jonas Salk by Michael Tomlinson. (Health).

[Barnes and Noble]

Abandoned Child by Marie Morgart. (Children/Family).
FDR’s Splendid Deception by Hugh Gallagher. (History).

[Amazon]

1998 Memorial Edition [Amazon]

Rehabilitation Surgery for Deformities Due to Poliomyelitis by Edwin Krol. (Medical Reference). [Barnes and Noble]
The Grey Filly by Virgil Cross. (History).

[Barnes and Noble]

Poliovirus Attenuation: Molecular Mechanisms and Practica by Fred Brown. (Medical Reference). [Barnes and Noble]
Epidemic Poliomyelitis: Report on the New York Epidemic by a Collective Investigation. (Medical Reference).

[Barnes and Noble]

Acute Poliomyelitis (Heine-Medin’s Disease) by Ivan Wickman. (Medical Reference). [Barnes and Noble]
The Volunteers, Means and Ends in a National Organization by Columbia University. 50. And They Shall Walk by Elizabeth Kenny. (History/Biography).
Survey of Cripples in New York City by Committee on Survey. (Family/Children). 52. Intelligence and Personality Factors Associated with Polio by E. L. Phillips. (Family/Children). [Barnes and Noble]
This Is My Story, This Is My Song by Mary Bramer. (Self-Improvement).
Tales from Inside the Iron Lung (and how I Got out of It) by Regina Woods. (Health). 55. Definitions of Time and Recovery in Paralytic Polio Convalescence by Fred Davis. (Medical Reference).
Two Steps at a Time by Marc Stein. (Medical Reference).

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

©Copyright Henry Holland 1999 2000.

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

Created: 5th April 1999

Last modification: 24th January 2010.

Dr Henry writes about Polio Literature Read More »

Non-Paralytic Polio and PPS

NON-PARALYTIC POLIO AND PPS

Marcia Falconer, Ph.D. cell biology and Eddie Bollenbach, M.A. biology

A Lincolnshire Post-Polio Library Publication – January 1999

INTRODUCTION AND HISTORY

Convincing medical professionals of the reality and appropriate treatment of post-polio syndrome was and often continues to be a long, hard, up-hill struggle. Today there is general consensus in the medical community that there are late effects arising from acute paralytic polio infections of 20 to 40 years ago. An increasing number of medical doctors no longer dismiss symptoms of fatigue, pain, and increasing weakness when presented by a person with a documented history of paralytic polio. This is so even though there is no objective test available to diagnose Post-Polio Syndrome (PPS) nor is there agreement on, or a clear understanding of, the etiology of this disease. However, people with PPS symptoms and a history of non-paralytic polio have great difficulty receiving a diagnosis of PPS.

CATEGORIES OF ACUTE POLIO AND THE DIAGNOSIS OF POST-POLIO SYNDROME

During the acute polio epidemics earlier this century the following categories were used to classify the extent and seriousness of the disease:

Sub-Clinical polio

The person is unaware of infection and gains active (sometimes lifelong) immunity to infection from that strain. Sub-clinical polio usually occurred in infants and very young children.

Abortive

Poliomyelitis

In “Current Diagnosis and Treatment” a polio manual for physicians (Brainerd et al. 1968) the symptoms of abortive polio are described as: “Abortive poliomyelitis may simulate acute respiratory infection or gastroenteritis, and is usually not dangerous. The symptoms are fever, headache, vomiting, diarrhea, constipation, and sore throat.”

Non

Paralytic Polio

Symptoms, quoting again from Brainerd et al. (1968), are “headache, neck, back, and extremity pain; fever, vomiting, and abdominal pain, lethargy, and irritability are present. Muscle spasm – spontaneous shortening of the muscle or hyperactive stretch reflex with limitation of extension by pain and contraction – is always present in the extensors of the neck and back, usually present in the hamstring muscles, and variably present in other muscles. Resistance to flexion of the neck is noted after a varying range of free flexion. The patient assumes the “tripod” position on sitting up, which he usually does by rolling to avoid flexing the back. Straight leg raising is less than 90

degrees. Spasm may be observed when the patient is at rest or may be elicited by putting each muscle through the maximum range of motion. The muscles may be tender to palpation.”

Paralytic

Polio

The symptoms are: “Paralysis may occur at any time during the febrile period. In addition to the symptoms of non-paralytic poliomyelitis, tremors and muscle weakness appear. Paresthesias (tingling) and urinary retention are noted occasionally. Constipation and abdominal distension (ileus) are common. Paralytic poliomyelitis may be divided into two forms that may coexist: (1). Spinal poliomyelitis, with weakness of the muscles supplied by the spinal nerves and (2). Bulbar poliomyelitis, with weakness of the muscles supplied by the cranial nerves, and variable “encephalitis” symptoms. Bulbar symptoms include diplopia (uncommon), weakness of mastication, facial weakness, dysphagia, dysphonia, nasal voice, regurgitation of fluids through the nose, weakness of the sternocleidomastoid and trapezius muscles, difficulty in chewing, inability to swallow or expel saliva and respiratory tract secretions. The most life threatening aspect of bulbar poliomyelitis is respiratory involvement due to pontile (central) involvement. Paralysis of the neck flexors is manifested by “neck drop” on lifting the shoulders from the bed. “…deep tendon reflexes are diminished or lost, often asymmetrically, in areas of involvement.” (Brainerd et.al., 1968).

Part of the difficulty getting a diagnosis of PPS, arises from the current practice of strictly categorizing the acute polio illness as paralytic, non-paralytic, abortive and sub-clinical. When polio was all too common, it was generally understood that there was a wide range of damage within every patient, even though the patient was assigned to a specific diagnostic category. However as time passed, much practical and unwritten knowledge was lost. One result is that the diagnostic categories of acute polio infection no longer carry the implication that polio damage occurs as a spectrum within every patient. Now a diagnosis of non-paralytic polio raises doubts in the physician’s mind that the person had any neuronal damage and even doubts that the person had polio.

Common sense dictates that it was a rare case of polio that did not blend into the adjoining category to some degree. Many sub-clinical cases probably had gastrointestinal symptoms. An abortive case with fever and headache or neck ache, along with muscle spasms, would argue for at least some involvement of the neuronal system. Autopsies on people who had non-paralytic polio, but who died from other causes, show nerve damage and some degree of neuronal death consistent with paralytic polio (Howe and Bodian, 1942; reviewed by Bruno et al. 1995). From such evidence it appears reasonably certain that there was a degree of neuronal involvement in people with non-paralytic polio (Bruno et al. 1991). The damage may have been sufficient to cause weak muscles but not enough to manifest as paralysis (Sharrard, 1955).

In acute polio, the degree of nerve involvement varies within the entire nervous system. Some areas appear clinically unaffected while, in paralytic polio cases, other regions show flaccid paralysis. In abortive polio the nervous system appears undamaged at the symptomatic level. However the symptoms listed for non-paralytic polio are suggestive of neurological involvement. For nerve damage to be visible as weakness or paralysis a threshold of damage to the neuron population must be involved (Sharrard, 1955). When few neurons are damaged or destroyed, the patient presents with no specific muscle weakness or paralysis but can have undetected neuronal damage (Dalakas (a), 1995). We do not know how this will manifest in later life. There were undoubtedly mistakes in diagnosis and misdiagnosis for many patients. Many people with non-paralytic polio probably were paralytic cases with diffuse weakness that recovered quickly (Dalakas (b), 1995).

THE NON-PARALYTIC POLIO PROBLEM

Reluctance to diagnose PPS in symptomatic people with a history of non-paralytic polio has many reasons. The cause(s) of PPS is not completely understood. Often there is an assumption that neurological damage is the only cause of PPS symptoms. Physicians and clinicians may have unreasonable expectations of the diagnostic accuracy of electromyographic (EMG) studies as well as the accuracy of other tests. Little consideration is given to evidence suggesting that PPS symptoms can arise from damage to areas of the brain during the acute illness, from persistent polio virus RNA, from auto-immune problems, and from neuronal damage which wouldn’t necessarily correlate with the expected electromyograph (EMG) readings.

One misplaced criterion for obtaining a diagnosis of PPS is the necessity for a documented case of paralytic polio. Ironically, the reason for this arises out of early research to study PPS. To be certain that test subjects had early polio, and not some other neurological disorder, researchers required that the subjects in the study group have a documented history of paralytic polio with residual weakness or paralysis in at least one limb. Unfortunately, these criteria have spilled over into the clinical area so that many physicians believe you cannot have PPS unless you have a confirmed diagnosis of paralytic polio, despite the fact that systematic studies have never been done to assess PPS in non-paralytic polio populations.

A “typical” presentation to a physician of a person with a history of non-paralytic polio and current PPS like symptoms goes something like this. “As a child, I was very ill with a high fever and a headache. I was hospitalized for a few days (or quarantined and not hospitalized). My mother says I was never paralyzed and I was discharged from the hospital with a diagnosis of ‘non-paralytic’ polio. I had cramps and pains in my back and legs and I was very weak for some months afterwards but then I recovered completely and forgot all about polio. I wasn’t very good at sports, but then, neither were lots of other people. About ten years ago (35 to 45 years after the acute illness), I began tripping on smooth floors and occasionally falling. Now everyday jobs like vacuuming tire me so that I have to lie down for an hour or two before I can do anything else. When I’m this tired, I can’t “think”, can’t focus or remember words. It’s difficult to put in a full day of work. My legs ache after I walk only a short distance and at night the muscles in them “jump” or twitch. My feet are always cold. I can no longer climb a flight of stairs and the weakness in my legs is frightening. I saw a neurologist who specializes in PPS and he said that he saw no evidence that I ever had polio although he did not give me a thorough examination or order any tests. He says I don’t have PPS and suggested that my problems are caused by arthritis or fibromyalgia.”

In one specific case of an individual earlier diagnosed with non-paralytic polio, and now complaining of new problems, the neurologist ordered two EMGs in the area the patient said was weak. According to the neurologist, both EMGs were “normal”. From this he concluded that the patient did not have polio and could not have PPS. EMG tests can miss late paralytic polio if the examiner does not find the spot where denervation occurred. With non-paralytic polio EMG positive fiber groups could be difficult to find.

SYMPTOMS, EMGS AND A PPS DIAGNOSIS

Accepted symptoms of PPS include: progressive muscle weakness, fatigue and pain (see general review by Trojan and Cashman, 1997). One of the descriptive criteria today for PPS is progressive weakening in muscles unaffected by the original polio illness. Tests in people who had paralytic polio have shown that limbs, previously designated as unaffected, had some degree of damage (Halstead et al. 1995). Clearly, a

person with non-paralytic polio could have had an equal amount of damage during the acute infection but would still be classified as having non-paralytic polio. It therefore is reasonable to assume that new

weakness can occur in any muscle of a person diagnosed with non-paralytic polio.

Electromyographic (EMG) studies will not show whether or not a person has PPS or is likely to get PPS. EMGs of paretic muscles reveal neuronal damage that occurred during the acute infection and subsequently resulted in the formation of large motor units in muscle (Dalakas (a), 1995). In a person with a history of non-paralytic polio, the presence of an abnormal EMG (one that matches the typical EMG pattern produced by polio affected muscles) is considered proof of previous paralytic polio and usually results in a diagnosis of PPS (Bromberg and Waring, 1991). However a normal EMG can not prove or disprove whether a person had sub-clinical, abortive or non-paralytic polio.

A normal EMG is an indication that the muscle or groups of muscles which were tested did not undergo denervation consistent with paralytic polio. However many clinicians maintain “the lack of clear evidence for previous denervation after extensive electrodiagnostic testing is a valid means for excluding the diagnosis of PPS” (Bromberg and Waring, 1991); a view supported by Gawne et al. (1995). This conclusion depends on the certainty that PPS is derived only from motor unit abnormalities and death, and no other metabolic or virological problems within intact neurons play a role – a fact which has not been established. Nonetheless, properly conducted and properly analyzed, EMG tests are valuable for ruling out other neurological conditions and may establish a history of previous paralytic polio in people with PPS like symptoms. They should not be used to ‘prove the negative’ (an impossible task), that the person does not have a post viral syndrome due to poliomyelitis.

FATIGUE AND NON-PARALYTIC POLIO

Severe, debilitating fatigue is another common denominator of PPS. Many people describe two types of fatigue: one relating to muscles and another relating to cognition and alertness (colloquially called ‘brain fatigue’). It is not known if there are two types of fatigue nor if fatigue is generated by more than one mechanism. What is known is that severe fatigue that responds to rest is found in survivors of both non paralytic and paralytic polio and is a hallmark symptom of PPS (Bruno et al., 1991 and 1995).

A commonly supported idea is that muscle fatigue results from demands on muscles innervated by neurons damaged in the acute illness or demands on muscles innervated by relatively fragile neuronal “sprouts” which developed to replace destroyed neurons (reviewed by Dalakas, (a,b) 1995, Trojan and Cashman, 1997). There is a hypothesis that ‘brain fatigue’ results from damage to a particular area of the brain involved in maintaining mental alertness, the reticular activating system (Bruno et al. 1991 and 1995). Extensive, post-mortem examinations by Bodian (1949) and others (see Bruno et al. 1995) indicate that some degree of damage to the brain occurred in all polio infections regardless of severity. The 30 to 40 year interval between infection and onset of fatigue could be explained by a combination of damage plus atrophy that occurs with normal aging. However, a counter argument suggests that ‘brain fatigue’ is not a separate entity but a part of the generalized fatigue resulting from overuse of muscles during everyday living. There also is the possibility that ‘brain fatigue’ is caused by a combination of the above factors and possibly others.

WHAT PERCENTAGE OF NON-PARALYTIC CASES WILL DEVELOP PPS?

Estimates of the percentage of people who had acute, paralytic polio and now have PPS range from 25% to 70% or higher and there are predictions that eventually all people who had paralytic polio will have PPS to some degree or other. Statistically there is a minimum of 10 non-paralytic polio cases for every documented paralytic case. What percent of people who had non-paralytic, or possibly even abortive polio, will develop PPS? PPS support groups report that between 1 and 10% of their members had non paralytic polio as children and now have fatigue, new muscle weakness and pain (Falconer, personal

communication). However many non-paralytic cases (and even cases with mild paralysis) were never seen by a doctor and may not know that they had polio. They would not suspect that new muscle weakness, fatigue and pain were due to PPS and would have no reason to join a PPS support group.

A 1951 study of acute polio infection in twins (Herndon and Jennings) and a subsequent follow-up study by Nee et al. (1995) showed that 71% of the twins diagnosed with paralytic polio had PPS symptoms. Interestingly, 42% of twins who had not been diagnosed with paralytic polio also developed PPS-like symptoms approximately 38 years after their affected twin was diagnosed with polio. Herndon and Jennings indicate that most “unaffected” twins actually had sub-clinical or non-paralytic polio. Based on this study it seems that nearly half of the people who had non-paralytic polio may develop PPS.

TESTS TO DETERMINE PRIOR POLIO INFECTION

Right now there are no tests that prove a person has PPS. However tests which indicate that a person definitely had polio are useful in obtaining a PPS diagnosis. There are tests to measure IgG antibody levels to polio virus strains 1, 2 and 3, as well as antibodies to other viruses including non-polio enteroviruses (Leon-Monzon and Dalakas, 1995). Everyone who was immunized will have some level of antibodies. However people who had an acute polio infection should have higher antibody levels to the infecting strain of the polio virus. Significantly higher levels document previous polio although antibody levels decrease with time, and low levels of antibody to polio virus do not rule out a previous polio infection.

By testing antibodies, a correlation was found between increased levels of a particular kind of antibody, designated as IgM, and people who had acute polio and now have PPS symptoms (Illa et al. 1995, Leon Monzon and Dalakas, 1955). IgM antibodies are produced as a “first response” to an infection and may indicate new antibody response to a particular condition, raising the possibility of testing IgM levels as a PPS indicator (Sharief et al. 1991, Dalakas (a), 1995). The presence of lymphocytes in muscle biopsy along with elevated levels of antibody to polio virus also is consistent with this theory (Dalakas et al, 1984).

A more sensitive test, using the polymerase chain reaction (PCR) technique on cerebrospinal fluid (CSF) obtained from a spinal tap, showed the presence of RNA, derived from polio virus RNA in people with PPS but not in CSF from people without a history of polio (Leparc-Goffart et al. 1996). While this test demonstrates a history of polio infection, the necessity for a spinal tap precludes use as a general test for PPS. Moreover, using this test to diagnose PPS must be approached with caution. Muir et al. (1995) found evidence of enteroviruses closely resembling Coxsackie B4 in CSF samples from people with PPS-like symptoms but found no correlation of polio virus RNA and post-polio symptoms (Muir et al. 1996).

POLIO VIRUS, VIRUS RNA FRAGMENTS AND PPS

Polio virus is a “lytic” enterovirus that kills the cells it infects, and researchers long believed no virus existed anywhere in the body for more than a short time after the acute infection. However mutations to the polio virus occur readily during infections (reviewed by Blondel et al. 1998). One mutated form of polio virus can persist indefinitely in tissue culture of human neuronal origin (Colbere-Garapin et al. 1989). These infected neuroblastoma cells continuously produce polio virus into the surrounding medium and are not killed by the infecting virus. Another polio virus, with only two mutations in the virus capsid protein, persistently infects cell cultures derived from human fetal brain cells (Pavio et al. 1996). This evidence suggests that the positive PCR samples, in CSF of people with PPS symptoms and described above, represent either a persistent infection in the nervous system or the continuing presence of pieces of RNA derived from polio virus. In either case it is possible that some PPS symptoms are due to the body

mounting an immune reaction to the presence of the polio RNA. This theory is supported by autopsy findings of chronic inflammation with the presence of lymphocytes in the spinal cord of a PPS patient (Miller, 1995).

There is a view that PPS patients could be divided into two groups (Hollman, 1986). “The first group has muscle deterioration from the early disease. The second group has problems in new muscle groups or those thought to be recovered from the disease. Problems in the second group are thought to have a cause other than simple aging, possibly autoimmune (in origin)”. It is clear that an RNA explanation for the late problems of non-paralytic polio patients could fit into Holman’s second group.

NON-PARALYTIC POLIO – CAUSED BY POLIO VIRUS OR BY A DIFFERENT ENTEROVIRAL INFECTION?

During polio epidemics it was known that “non-paralytic polio symptoms” were not always due to polio virus. Non-polio enteroviral infections, among others, produced the same symptoms. However, if a patient with nonparalytic symptoms had a sibling or other close contact with a paralytic polio case, then the infectious agent usually was polio virus.

Many non-paralytic cases were due to infection with a less neurovirulent variant of polio virus. Sabin and Steigman (1949) studied an epidemic of “summer grippe” in an attempt to isolate a variant of the polio with lower neurovirulence to make a vaccine. Of their “summer grippe” patients, 50% had a non-paralytic polio caused by a less virulent strain of polio, 20% developed paralytic polio, and the infectious agent of the remaining 30% of patients was not established.

To monitor the strains of polio virus causing a Washington, D.C. epidemic, specimens were obtained from 63 paralytic cases, 87 non-paralytic cases with positive spinal taps and 5 non-paralytic cases with negative spinal taps (Shelokov et al. 1955). Of the paralytic cases, 64% had polio virus strain 1 (pv1), 33% had pv2 and 3% had pv3. Of the non-paralytic cases, 25% had pv1, 10% had pv2 and 4% had pv3 while 61% were untypable and presumably had an infection other than polio virus. Interestingly, of the 5 cases with normal spinal taps, one had pv1, one had pv2 and the other 3 were untypable.

The percentage of non-paralytic cases caused by polio virus or another virus varied enormously. During a polio epidemic in Hawaii in 1957, specimens were obtained from 38 people with paralytic polio and from 39 cases of non-paralytic polio. “Of the 38 cases of paralytic disease, type 1 polio virus was implicated in 33, Coxsackie B2 virus was isolated…in 2 and no diagnosis was established in 3. Of the 39 cases of

nonparalytic poliomyelitis, only 4 were related to type 1 polio virus. There were 16 cases of echovirus 9, 7 cases of Coxsackie A9, and 4 to 5 other enteroviruses.” (Johnson 1995).

Depending upon the epidemic, 70%, 39% or 10% of non-paralytic cases were caused by infection with polio virus. It is not prudent, therefore, for an examining physician automatically to conclude that a patient with a history of non-paralytic polio actually did not have polio.

PPS-LIKE SYMPTOMS AND ENTEROVIRAL INFECTIONS

Polio virus is one of more than 70 enteroviruses. A number of enteroviruses (Coxsackie A9 [Gromeier et al. 1997], enterovirus 70 [Gromeier et al. 1997], enterovirus 71 [Melnick, 1984]), as well as a flavivirus, Japanese encephalitis virus [Solomon et al. 1998], among others, can cause polio-like paralysis. These non-polio enteroviral infections may cause PPS-like symptoms as well. Coxsackie virus RNA has been isolated from people with PPS symptoms (Muir et al. 1995), although it is not known if this is a common event. If PPS arises from neuronal damage, then intuitively, enteroviruses causing symptoms that are

clinically identical to polio could cause similar neurological damage during the acute illness which in turn would give rise to PPS-like symptoms in later life. If PPS arises from an immune response to continuous presence of viral RNA, then finding enteroviral RNA (other than polio) in people with PPS symptoms, as was shown by several researchers, also indicates PPS-like symptoms could occur from non-polio virus infections.

NON-PARALYTIC POLIO AND PPS

With respect to a diagnosis of PPS for people with a history of non-paralytic polio and PPS symptoms, there are at least four separate possibilities:

The person had non-paralytic (or abortive) polio. At the time of the acute illness there was no obvious damage to the nervous system although unobserved damage was likely. There are no established figures for the minimum amount of neuronal damage which can result in PPS symptoms. Exclusion of a diagnosis of PPS on the basis of a history of non-paralytic polio is not merited.
The person actually had paralytic polio but was misdiagnosed. Symptoms of paralysis and/or weakness were missed or the symptoms manifested only for a short time. The patient recovered (apparently) fully within a matter of weeks. This type of polio often was labeled as “non-paralytic”. PPS will occur with the same frequency as in paralytic polio cases. Diagnosis of PPS on the same basis as for a patient with a history of paralytic polio is merited.
The person did not have polio but had another disease with clinical symptoms similar or identical to polio and currently presents with PPS symptoms. Some evidence supports the hypothesis that non polio enteroviruses can have late, post-viral effects. If these people have post-viral symptoms identical to PPS, require the same management and have the same prognosis, should they be included under the PPS heading?
The person presents with PPS-like symptoms but the cause is not post-viral in origin. More medical examinations are required for the patient and a diagnosis of PPS is not merited.

It is vitally important that people with PPS-like symptoms see a physician since other neurological diseases can have similar symptoms. However, when these diseases have been ruled out then the doctor should consider PPS, especially if the person has an oral history of having had polio. Moreover a documented history of paralytic polio should not be the exclusive criterion obtaining a diagnosis of PPS.

CONCLUSIONS

A history, either oral or documented, of non-paralytic polio is not sufficient reason, by itself, to exclude a diagnosis of PPS. PPS symptoms may have both physical and molecular origins including neuronal damage, persistent viral RNA and immune response. In the case of non-paralytic polio, some amount of damage to neurons almost certainly took place and this may be sufficient to cause PPS symptoms of new muscle weakness, fatigue and pain. If the presence of persistent viral RNA and/or the immune response or other molecular events can precipitate PPS, then even the absence of neuronal damage does not preclude developing PPS. A history of any kind of polio indicates the disease was severe enough to cause damage. However with only 2 years to the 21st Century we still have no objective test for Post Polio Syndrome. Considering the number of people affected and the morbidity and cost to our population, the cause of PPS should be a priority for clinical research in the next few years.

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Web Site: www.lincolnshirepostpolio.org.uk

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.

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

Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/falconer/nonparalytic.html> Created: 15th January 1999

Last modification: 24th January 2010.

Non-Paralytic Polio and PPS Read More »

Bollenbach Polio Biology 2

Polio Biology II

Post-Polio Syndrome’s Elusive Etiology

Eddie Bollenbach

A Lincolnshire Post-Polio Library Publication

3rd January 1999

In science, self-satisfaction is death. Personal self-satisfaction is the death of the scientist. Collective self-satisfaction is the death of the research. It is restlessness, anxiety, dissatisfaction, agony of mind that nourish science.

Jacques Monod, 1910-1977, New Scientist, 1976.

Monod is someone to listen to. He and his colleagues gave us the first model explaining how a gene can turn on when it is needed and off when it is not. And so, although I don’t need a lot of pushing in the direction of questioning ideas, I do think it is of value to be skeptical, especially when someone gives us an unequivocal answer as to the cause of a complex process like Post-Polio Syndrome.

So, the bells went off the other night when I visited the Web Pages of the National Institutes of Health in the States. I scrolled around until I got to Post-Polio Syndrome. And I found a rather authoritative pronouncement that it was caused by the recovery from polio. That the Syndrome can be completely understood in the loss of regenerated fibers, which compensated for the original damage of disease. And that this regression is the result of overcompensation and years of overwork of the nerves that “carried the day” thirty plus years ago. But why? What actually happens? And, although it sounds very plausible, where is the evidence for this?

Why do some people with a lot of polio damage not experience Post-Polio Syndrome? Do their overworked neurons remain stable for some unknown reason? Why is there new brain pathology (if there is) with Post-Polio Syndrome? And, if there is, do brain neurons just happen to lose connections and die at the same time as motor neurons by coincidence, even though they attach to one another and not to muscle that overwork them? I need a deeper understanding of this. Answering that motor neurons “wear out” begs the question: why, what is the mechanism?

Sometimes when an answer is given, as above, the effect is to stifle further research. If there is to be any deeper understanding, we have to comprehend the mechanics of how neurons “wear out”. I’d like to offer some food for thought without endorsing what follows, but rather present it to try to convey how complicated interactions between host (us) and parasite (polio) can be biologically. And, as a consequence of this complexity, how difficult it can be to find an ultimate cause.

Polio virus is very small. It is classified with this in mind: Picornaviridae. The particle itself is composed of two parts: a protein case, called a capsid, and the genetic material inside: Ribonucleic Acid (RNA). The

capsid is made of repeating units called capsomers, and each capsomer (a triangular piece like a part of a soccer ball) is made of protein subunits called protomers. Each protomer is the same. So you put together a polio virus by assembling protomers into capsomers, and capsomers into a twenty sided (icosahedron).

The genetic stuff (RNA) is inside. The RNA contains very few genes and is unusually small. Unlike larger DNA viruses, polio has no way of fixing mutations when they happen. This would require a larger genome. And mutations do happen during an infection. What I am saying is that when the genetic material duplicates inside an infected cell there is not always perfect fidelity between the parent molecule and its daughter molecule. Pairing mistakes are made. Considering how small polio virus is there can be a million or more duplications within a single cell. We must conclude that there are plenty of mutant genomes (RNA’s) produced during the life cycle of polio virus within a motor neuron.

Now, the presence of mutant RNAs would likely interfere with the proper reproduction of normal genomes. First of all, viral genes shut down the cell’s normal functions, selfishly requisitioning these materials for its own reproduction. We can assume that there would be competition between the normal genomes and mutant RNA inside an infected cell. Many of the mutant RNAs would be unable to produce proteins which could assemble into a complete viral particle. Some of these RNA’s would remain inside the cell, which would present opportunities for them to survive by evading the immune system. The normal viruses outside would be subject to attack by the immune system. So mutant genomes of polio virus could very well be selected for and be capable of surviving within a cell while normal viruses succumbed.

Another question I’ve thought about recently is why doesn’t polio virus completely decimate all motor neurons? Why do some survive? Bodian’s work in the 1940’s showed that most all of the neurons appeared abnormal during the acute polio infection. Yet some survive, why? Does the infection with virus allow some neurons to modify themselves resulting in an effective defense mechanism against infection? Does the nerve cell surface change, or is there some other biochemical change which may persist after infection, which, ultimately shortens its life cycle and may result in PPS down the line?

Or do cells have within them now bits of polio virus RNA, which have survived all these years because of their evolutionary fitness in competition with all viral progeny? If so these RNAs surely make proteins within the cell and these proteins, no doubt, migrate to the cell’s surface. There, they can be recognized by the immune system and attacked. Has this been going on very slowly? Naked RNA (without the virus capsid) can infect adjacent cells but this is very inefficient compared to intact polio virus. Has this been going on for 30 plus years? Are some or all of these proposed mechanisms in place and do they explain why post-polio motor neurons “wear out”.

There is precedent justifying these questions. Coxsackievirus is a Picornavirus too. It is very similar to polio. One single gene mutation of coxsackievirus B3 can convert it into a virus, which damages the heart. [1]

The progression of HIV virus to AIDS is not clearly understood. The evolving virus, within cells, results in mutants at astonishingly high rates. This virus is highly adaptable and cannot as of yet be exterminated within a patient.[1] And highly mutated polio virus RNA was found in a study of a small number of patients with PPS. One half of patients with PPS (5) had recoverable highly mutated polio RNA. While none who had paralytic polio but no signs of PPS had recoverable mutated RNA. Was this due to damaged neurons that “wore out” releasing the RNA that the control group had? Or was the presence of this RNA causal to Post-Polio Syndrome?[2]

My advice mirrors Monod’s at the start of this essay: don’t embrace any of these ideas, be skeptical, question, and wait for evidence to arrive. Just think about it.

References

Quasispecies Structure and Persistence of RNA Viruses Esteban Domingo,* Eric Baranowski,* Carmen M. Ruiz–Jarabo,* Ana M. Martín-Hernández,† Juan C. Sáiz,‡ and Cristina Escarmís*Centro de Biología Molecular “Severo Ochoa,” Universidad Autónoma de Madrid, Cantoblanco, Madrid, Spain; †John Innes Center, Norwich, United Kingdom; ‡Universitat de Barcelona, Barcelona, Spain , in Emerging Infectious Diseased, December 1998. The entire article can be found at: www.cdc.gov/ncidod/eid/vol4no4/domingo.htm
J Clin Microbiol 1996 Aug;34(8):2023-6 Evidence of presence of poliovirus genomic sequences in cerebrospinal fluid from patients with postpolio syndrome. Leparc-Goffart I, Julien J, Fuchs F, Janatova I, Aymard M, Kopecka H Laboratoire de Virologie, Centre National de Reference des Enterovirus, Lyon, France. leparc@cismsun.univ-lyon1.fr [PubMed Abstract]

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: 3rd January 1999

Last modification: 21st January 2010.

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Dr Henry writes about Polio Spouses

Dr. Henry writes about Polio Spouses

Eleanor Roosevelt is probably the most famous polio spouse of this century. She was born into an aristocratic family on November 7, 1884. However, emotional losses and low self-esteem plagued her developmental years. She was the oldest child and only daughter of Elliott Roosevelt, President Theodore Roosevelt’s brother, and his beautiful and vivacious wife, Anna Livingston Ludlow Hall. At age two and a half, she was crossing the Atlantic with her parents when their ship was involved in a collision. The terror of being lowered from on high to a lifeboat left her with a fear of heights and the water. As a young child, her mother called her “Granny” because she was so serious and mature. When she was six, her mother said, “You have no looks, so see to it that you have good manners.” As a result, Eleanor tried to be dutiful, compliant, obedient, responsible, and useful, but she was also independent, goal directed, and willful.

Death left her an orphan by age eight. She had two younger brothers. One of them, Elliott, died at age four from scarlet fever. When she was eight, her mother died from diphtheria. Her father had a history of alcoholism and chronic depression. He was not awarded custody of Eleanor after her mother’s death. Eleanor went to live with her maternal grandmother in Manhattan. Two years later, her father died from the consequences of alcoholism. She stayed with her grandmother for five years. During this time, she grew to be a tall, but rather awkward and self-conscious teenager. She was a loner and an outsider. At the age of fifteen, her grandmother sent her to the Allenswood boarding school in England. At this school, Eleanor came under the influence of the headmistress, Mademoiselle Marie Souvestre. This school was most progressive for the Victorian nineties. The daughters of England’s more liberal leaders attended this school. Eleanor excelled at Allenswood for three years. She became more self confident, versatile, and a well loved young woman. At age eighteen, she returned to New York, involved herself in social work causes, made her debut in society at her grandmother’s insistence, and soon met Franklin.

In August 1921, she became a polio spouse. During her husband’s acute illness, she was his bedside nurse and performed many necessary duties such as attending to his urinary catherizations. Over the next several years, despite her mother in law’s opposition, she kept the hope alive in Franklin’s mind that he could return to politics. Franklin did become the four times elected President of the United States. During this time, Eleanor became his conscience, promoting progressive social reform. She became his legs and traveled throughout the world as his “ambassador of hope.” She was a person ahead of her times. She was a feminist. She was compassionate and idealistic. She was a friend to the disenfranchised. She was an assertive and hopeful voice for the hungry, the unemployed, and African Americans. She became a writer, a teacher, lecturer, and voluntary ambassador.

When asked about her husband’s polio, she would say his polio was a “blessing in disguise.” Some had argued that FDR had always been a powerful and compassionate man; she believed that Franklin’s struggle with polio “gave him a strength and courage he had not had before. He had to think out the fundamentals of living and learn the greatest of all lessons — infinite patience and never ending persistence.”

Eleanor appeared on the Edward R. Murrow TV Show, “This I Believe.” In response to a question about her life, she stated, “You have to accept whatever comes, and the only important thing is that you meet it with courage and with the best you have to give.”

In my opinion, Eleanor Roosevelt’s perceptions as a polio spouse are astute and wise. As polio survivors, most of us learned long ago about the value of patience, persistence, and trying to think through the fundamentals of living. For many of us, polio brought a precocious understanding of the perils of life. If we did not learn and exercise these lessons years ago, we certainly need to exercise them now with the realities of Post-Polio Syndrome (PPS).

There are a number of polio spouses who are quite active in our post-polio support group. From my observation, these spouses fully represent individuals who have learned “to accept whatever comes” and have met adversity with “courage and the best they have to give.” No marriage is all bliss and joy. The realities of polio and now PPS can stress the best of marriages. Based on last year’s survey of our group, 70% of our members are married. 51% felt that the development of PPS had had some impact on their families. The changes that might result in a marriage relationship as a result of PPS can be adverse, or can be part of the “for better or worse, in sickness and in health” definition of a committed marriage.

I think that polio spouses are often forgotten and overlooked heroes. For many of us, they are “gofers.” They run errands, pick up things, and help with many details of daily living. They also get tired and moody, and have bodily aches and pains. Usually, they say “yes” more often than “no” to us, are our thoughtful personal advocates, and our loving protectors. Marriage relationships are traditionally complementary, as each spouse is expected to manage certain tasks toward the maintenance of a home and the raising of children. The invasion of PPS may alter the dynamics of that complement. As a consequence, the polio spouse may feel somewhat abandoned, needed more than loved, and less valued. This is more likely to occur when the PPSer is having greater difficulty accepting and adjusting to a life with PPS. When the PPSer is more anxious, fearful, depressed and uncommunicative, the polio spouse may be on the receiving end of irritability, complaining, and subtle rejection. Both marriage partners may well understand this process, but feel helpless in changing it. Meaningful communication is the key to working through the changes that have occurred and in establishing a new and lasting complementary relationship. If this level of communication is not possible, professional help may be an excellent next step.

As a first lady, Eleanor Roosevelt made a positive impact on the lives of many people. As a polio spouse, she must have felt the anger, fear, and uncertainty brought to their lives by her husband’s polio. I feel that many polio spouses have felt and may still feel the anger, fear, and uncertainty that PPS has brought. There may be no brilliant dynamic psychotherapeutic models to resolve the realities of the impact of PPS on a marriage. Eleanor’s advice is the best that I have heard.

“You have to accept whatever comes, and the only important thing is that you meet it with courage and with the best you have to give.”

Henry Holland, Richmond., Virginia, USA. Henry4FDR@aol.com

January 1999

Originally published in the Central Va PPS Support Group (PPSG)‘s newsletter, The Deja View, in 1999.

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: 17th January 1999

Last modification: 24th January 2010.

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Poliomyelitis Fact Sheet

Page 1 of 3

POLIOMYELITIS FACT SHEET

An Overview of Polio and Its Late Effects

There are four generally recognized forms of poliomyelitis. The Asymptomatic Carrier, the Abortive, the Non-Paralytic, and the Paralytic. The first two have no known lasting effects and will not be dealt with in this reference.

The non-paralytic form of poliomyelitis is usually indicated by nausea, headache, sore throat, back and neck pain, and stiffness. In addition, there are changes in reflexes and elevated spinal fluid cell count. It is estimated that 65 percent of recognizable cases during the polio epidemics were non-paralytic. It is possible that there may be some unrecognized muscle involvement,

Paralytic poliomyelitis shows the same symptoms as non-paralytic plus weakness in one or more muscle groups. It is commonly classified in the following manner: SPINAL: This type is most frequent and is associated with involvement of trunk or extremities; more often the lower extremities. Weakness does not fit any pattern. BULBAR: Symptoms can include difficulty in swallowing, loss of voice quality, and sometimes tongue and facial paralysis. BULBOSPINAL: This type of involvement is usually severe and is associated with respiratory impairment. Ten to twenty-five percent of paralytic cases seen during the polio epidemics were of bulbar or bulbospinal type.

The Polio virus affects the body; by attacking the central nervous system; specifically the anterior horn cells. These motor neurons are located in the front part of the spinal cord and are essential for any muscle activity.

The motor neurons rooted in the anterior horn travel outward through the peripheral nerves to innervate muscle fiber. Depending on the location of the muscles they supply. These motor neurons can be a few inches or several feet long. Sensory nerves travel from their specialized receptors to the spinal cord and enter the posterior horn. Sensory nerves are rarely affected by the polio virus.

Many individuals lost considerable function during the acute stage of polio but regained a large part of that function. It appears, however, that a percentage of these individuals are experiencing renewed weakness, sometimes accompanied by fatigue or pain. Definitive studies are incomplete, but the best medical evidence at this time would indicate that a combination of factors associated with overuse are responsible.

If we take a closer look at the recovery process we can see why this is so. The polio virus attacks randomly. Sometimes motor neuron damage is not severe and the cells can recover much of their function. Other neurons may sustain more complete and irreversible damage. Even if this is the case, however, function can often be restored by “Sprouting”. Motor neuron cells have the ability to send out new axons that can innervate neighboring muscle fibers whose own neurons have been destroyed. Motor neurons normally innervate between 200 and 500 individual muscle fibers. If a percentage of motor neurons are destroyed, and sprouting takes place, the remaining motor neurons may be innervating as much as four times the normal amount of muscle fiber. It is assumed that the additional load that this places on motor neuron metabolism will, over the years, cause a failure of

Poliomyelitis Fact Sheet

Page 2 of 3

impulse strength. This seems to begin about 20 to 30 years after polio onset, and is probably a contributing factor for the renewal of weakness.

Another possible cause for this renewed weakness, especially in older individuals, is a normal part of aging. It is estimated that a person will begin to lose about one percent of their motor neurons per year after the age of 60. This is not very significant if an individual starts with 100 percent of their motor neurons, and all are intact and undamaged. Unfortunately, this is not the case with many individuals. Their supply was depleted by the polio virus, and this gradual loss will have a much greater impact

Some individuals may have gained a degree of recovery by building up the strength of their remaining musculature by exercise and intense use similar to athletic training; a term called hypertrophy. These individuals, however, used this strength in their day-to-day activities and thus the muscles have been performing continually at a level that is no longer tolerated.

In addition, many affected muscles that were believed to be “good” or “normal” with manual muscle testing, have been found to be less than this when EMG and other studies were done. In normal activities these muscles have also been working harder than was once believed.

Muscle weakness due to polio is often asymmetrical. This can put added stress on the entire neuromuscular system, as well as adding strain to joints and supporting ligaments. The result may be an increase in arthritis-like pain.

The question now arises, what can be done to alleviate this condition or prevent the further degradation of muscle strength?

First, an individual should seek medical advice to rule out other possible causes for their symptoms. Just because a person has had polio does not make them immune from other physical ailments. Second, it is highly recommended that a complete physical evaluation be done. This should include examination by an orthopedic specialist, and complete pulmonary function tests. Ideally, these should be done by physicians who are familiar with polio. You should ask for these test results in writing. Even if no immediate problem exists, this will provide a baseline for measuring your future status.

Lifestyle modification is the most important therapy for new weakness. This will reduce the strain placed on the body. Learning to listen to the body’s signals is vital to any plan designed to reduce further degradation of muscle strength. If you’re tired, rest. If you hurt, stop.

An orthopedic specialist may recommend braces, or other assistive devices, to help relieve the stress that is being placed upon weakened muscles and joints. In some cases this is sufficient to halt the progression of weakness. Selective surgery can sometimes redistribute the residual control so that strain is reduced.

Using assistive devices (electric wheelchairs, scooters, etc.) is resisted by some individuals. They feel that they are “giving in” to their new symptoms. What they assume to be a burden can actually be very liberating. Using these aids will allow them to conserve their energy for those activities that they feel are most important.

Because pulmonary function has such an important bearing on an individual’s general health, this area deserves special emphasis. Standard tables for vital capacities list variables for age and height.

Poliomyelitis Fact Sheet

Page 3 of 3

These may not have much relevance for many of us. Actual volume, measured in liters or milliliters, is a better indicator of pulmonary status. It is a good idea to have your vital capacity measured lying down, as well as sitting. For anyone with a volume of less than two liters a follow up should be made at least yearly

Arterial blood gas measurements for CO2 may be warranted. Generally, CO2 levels between 35 and 45 are considered normal. The CO2 level in your blood can become elevated, gradually, without one being aware of the change. If the level becomes high enough, the blood’s ability to carry oxygen will be impaired. This could result in headaches or interrupted sleep patterns and may be an indicator of respiratory insufficiency. Respiratory function can also be affected by scoliosis or reduction in rib cage flexibility that often occurs as a person ages.

Many individuals have asked about the role of exercise for those experiencing the late effects of polio. Unfortunately, the jury is still out on this question. There have been studies that indicate a ten percent increase in muscle strength following closely monitored exercise programs. But the number of participants was too small to come to any definite conclusion. If a person feels they may benefit from additional exercise, then an aerobic type of exercise is probably best. A twenty minute program consisting of two to three minutes of activity followed by a minute of rest could be tried. If this makes you feel better continue but, if pain or fatigue results, reduce the exercise until you find a level that can be tolerated. Stopping completely may be best. A significant percentage of polio survivors get all the exercise they can tolerate in their activities of daily living. And, if it is true that overuse is a contributing cause of renewed muscle weakness then strenuous exercise regimens should be approached with caution.

The United States Department of Health estimates that there are 1.6 million polio survivors. Although a significant percentage of these polio survivors are experiencing some late effects there is no reason to expect that all individuals will be affected. If you would like more information on this and related topics we recommend you read the various publications of the Gazette International Networking Institute (G.I.N.I.). The Rehabilitation Gazette, and its predecessor, the Toomey J. Gazette, has been published since 1958. It comes out twice a year and a subscription is $12.00 for individuals. The International Ventilator Users Network (I.V.UN.) News is $8.00 per year for ventilator users, and is an excellent resource for those with pulmonary impairment. The “Handbook on the Late Effects of Poliomyelitis for Physicians and Survivors” contains useful information on polio and related subjects. Inquiries relating to these publications should be directed to G.I.N.I. at 5100 Oakland Ave., #206, St. Louis MO 63110

The Poliomyelitis fact Sheet is published by the Polio Survivors Association, a non-profit corporation.

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Survey of the Late Effects of Polio in Lothian

Rehabilitation Studies Unit

Charles Bell Pavilion

Astley Ainslie Hospital

133 Grange Loan

Edinburgh

EH9 2HL

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Poliomyelitis – New Problems From An Old Infection

POLIOMYELITIS

New Problems From An Old Infection

John Shneerson MA DM FRCP FCCP Director of the Post-poliomyelitis Unit and the Respiratory Support and Sleep Centre,

Papworth Hospital, Cambridge, UK

NA official Yearbook 1998

ERDALERAS SEVER LRESPE RES

National Association of Fundholding Practices

Official Yearbook 1998

Lincolnshire Post-Polio Library copy by kind permission of Dr. Shneerson and the Editor

of the NAFP Official Yearbook

1 of 8

08/12/98 18:59

wvavi UV

There are currently around 30,000 post-poliomyelitis survivors in the UK, although the last major epidemic was over 40 years ago. Around half of these will develop the post-polio syndrome which is a recently recognised disorder characterised by late onset of weakness in muscles affected during the acute illness. Indirect effects of polio, especially degenerative disorders of the joints and soft tissues also contribute to the increasing disability that these patients are developing. It is essential to accurately analyse the cause of any new symptoms in polio survivors so that specific treatments can be applied, or modifications to their pattern of activities recommended.

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Is poliomyelitis a disease of the past? It is now over forty years since the last major epidemic took place in the UK and there can hardly be a doctor still practising who has much first-hand experience of acute poliomyelitis occurring in this country. The general feeling is that once the patient recovered from the acute illness, his or her condition remains stable, and is irrelevant to any new problems. From the patient’s point of view, however, there is a feeling of isolation and frustration. Isolation because of lack of contact with other people who have had polio or with any organisation that can offer help. Frustration because they feel that the medical profession and other health care workers have little expertise or interest in the problems that result from their polio; new problems as well as old.

The numbers of post-polio patients is considerable. In the USA it is estimated that there are over 600,000 survivors who have had this infection and in the UK the number is thought to be around 30,000. The new interest in polio is not however only due to the potentially large patient population, but mainly to the recent recognition of the post-poliomyelitis syndrome (PPS). There has been debate about the importance, or even the existence, of this condition over the last 20 years, but much of this confusion is related to semantic difficulties. PPS is best regarded as the development of new weakness in muscles which were previously affected by the acute infection. The confusion has arisen because postpolio patients also develop muscle weakness and other new symptoms as indirect late consequences of polio, due to, for instance, degenerative soft tissue and joint conditions. The wider term ‘late effects of polio’ is best used to describe these indirect consequences as well as PPS, which should be reserved for the specific late onset neuromuscular disorder that polio causes.

Polio 1912-1993

YE MAPA

verine All notifications

* Deaths

No. of Pollo Patients

wiwiwibowo

1912

1916

1920

1924

1928

1932

1936

1940

1944

096

1964

1968

1972

1976

1984

1992

Poliomyelitis New Problems From An Old Infection

To understand PPS one has to be aware of what happens during and after the acute infection. The polio virus is an enterovirus which in most subjects only causes a non-specific influenza-like illness with or without diarrhoea. The majority of people do not develop classical paralytic poliomyelitis. When this does occur this is due to damage to lower motor neurons which causes the peripheral nerve fibres or axons supplying the muscle fibres to die back. This leaves the muscles fibres ‘orphaned’ and in the healing process the remaining axons sprout new terminals to innervate these muscle fibres. New motor units, which are the group of muscle fibres supplied by any one axon, may increase to as much as 10-15 times the normal size. The formation of these ‘giant’ motor units contributes to the recovery of muscle strength in the weeks, months and even the first couple of years after the acute illness. For many years they appear to function satisfactorily, but in some subjects the axons eventually begin to die back. The muscle fibres cannot then function and begin to atrophy.

The cause of this late degeneration of the axons and the death of their cell bodies in the spinal cord and brain stem is uncertain. There is no evidence for any reactivation of the polio virus or for any abnormal immunological response that might cause cell death. Ageing itself is unlikely to be responsible since PPS is not age-related. Cell and axon death may be related to an inability to sustain the higher metabolic demands required by the giant motor units, particularly if the muscles have been much used. Active muscles have a higher stimulation frequency of their muscle fibres than little used muscles and this

places a greater metabolic load on the peripheral nerve cells.

Diagnosis of PPS

Recognition of PPS is based on identifying which patients are at risk and on recognising relevant new symptoms. PPS is commoner in patients who were more severely affected by the ac who were hospitalised or ventilator-dependent during their initial infection or in whom all four limbs were paralysed are at high-risk. It is more common in those who contracted polio after the age of 10, possibly because the healing process occurred less satisfactorily with more extensive sprouting of new terminals, and also because the infection is usually more severe in those who contracted polio at a later age. It is unusual for PPS to be detectable within thirty years of the acute illness. The mean onset is around 35 years, but it can occur at any time, even as long as 75 years after the original infection. PPS is detectable in around 50% of patients who have had polio, but in 80% of these (40% of the total) it is only slowly progressive. It is the remaining 20% (10% of the total) in whom it advances rapidly. The bulk of the patients who are now developing PPS were those who developed polio in the major epidemics between 1946 and 1957 (figurel).

Electromyographic (EMG) and peripheral nerve conduction studies may exclude other disorders but have proved to be of little help in diagnosing PPS, despite its being due to peripheral nerve fibre degeneration. This is because even in patients without clinical features of PPS there is often some detectable peripheral nerve fibre degeneration. This suggests that many polio patients may show subclinical features of PPS after a certain interval, but in only some is it clinically apparent.

The cardinal symptom of PPS is the appearance of new weakness in muscles which were previously affected by the acute illness. Patients who had polio often develop trick movements and other strategies to carry out activities despite muscle weakness and only a slight loss of strength may prevent these from continuing to be performed. The patient may simply be aware of a deterioration in the function of a part of the body, such as a hand or arm, or there may be difficulty in walking or an increasing frequency of falls, perhaps due to instability of a lower limb joint due to new muscle weakness, Polio causes a patchy muscle weakness and PPS can be equally patchy in the muscles that it affects (figure 2).

Figure 2: Polio patient showing extensive but patchy muscle atrophy

A second important symptom is the development of muscle fatigue. This is the loss of strength of a muscle with continued or repetitive activity. It initially affects muscles that are weakened, since at any given level of exertion they are exercising at a relatively greater fraction of their capacity than a normal muscle. Patients may find that they become progressively weaker as they walk further and have to stop in order to regain their strength. Muscles which are over-exercised may become painful, either during or more commonly some hours after the exercise. This pain may have a burning character to it and muscle cramps are also common. After overexercising patients may feel generally physically tired for as long as one or two days.

While it is commonest for PPS to affect the limb muscles it can also involve those that are responsible for speech, swallowing and respiration. The patient’s voice may become weaker, dysphagia may become a problem and aspiration of food, drink or saliva into the tracheo-bronchial tree may occur. Patients may present with weight loss through avoiding eating because of the difficulties that it causes and this may exacerbate any muscle weakness. If the dilator muscles of the upper airway are involved, particularly those in the pharynx and those controlling the larynx, obstructive sleep apnoeas may be seen. These are characterised by snoring or stridor-like noises during sleep associated with an irregular respiratory pattern, a feeling of waking up un-refreshed and increasing daytime sleepiness. This sleepiness should be distinguished from the feeling of physical weariness due to overuse and fatigue of the limb muscles.

A separate respiratory problem arises if the chest wall muscles weaken as part of the PPS. In this situation they are unable to maintain an adequate level of alveolar ventilation during sleep and both hyrpercapnia and hypoxia develop. This may cause frequent awakenings from sleep, un-refreshing sleep and daytime sleepiness. This type of respiratory failure is commonest in patients who were initially treated in an iron lung as a form of ventilatory support during their acute illness. Their vital capacity, measured with a spirometer, is usually less than around 1 litre. More sophisticated tests of their inspiratory muscles usually reveals profound weakness and many of these patients also have a weak cough due to expiratory muscle weakness or laryngeal abnormalities.

Other late effects of polio

These features which indicate the appearance of PPS should be distinguished from the indirect effects of polio. The most important of these are soft tissue and joint degenerative conditions. The tendons and ligaments may become inflamed or weakened, osteoarthritis is common and joints become unstable. Peripheral nerve entrapment is also common and this often causes, for instance, shoulder pains if the cervical spine is involved or sciatica from lumbar spine disorders.

Assessment of these symptoms is often complicated by the presence of depression and anxiety. These may be the result of years of social isolation due to the physical effects of the original infection, but very often depression worsens when symptoms of PPS or the other late effects of polio appear. Uncertainty about the future, in particular about the ability to continue with the previous way of life and to remain independent, becomes a major problem. The appearance of new symptoms often brings back thoughts of the original illness as a child and all the implications of this. Some patients deny that they are developing new difficulties, but more commonly the fear of what the future will hold and difficulties in coping with a loss of physical function precipitate a consultation with a general practitioner.

Management of PPS

The management of PPS and the other indirect effects of this infection requires an accurate assessment of the cause of the current symptoms and in particular a recognition of what is and what is not due to PPS. It is important to establish whether or not the original illness really was polio or a different neurological or infective condition. The severity and extent of muscle, joint and soft tissue abnormalities should be assessed. If the respiratory muscles are thought to be involved, estimation of the vital capacity is essential. A sleep study is often needed to assess whether or not the patient is developing obstructiv sleep apnoeas or hypoventilating during sleep. The evaluation of the patient’s problems may require a specialist opinion, especially if weakness of the respiratory muscles is suspected.

The principle of treatment for PPS is that excessive use of affected muscles should be avoided. This contrasts with the ‘use it or lose it’ theory of rehabilitation which was in vogue for polio ‘victims’ in the 1940’s and 50’s. At that time it was thought best to retrain the muscles as much as possible and to continue with exercise in order to keep them strong. In retrospect this may have contributed to overuse of the muscles and have increased the current prevalence of PPS. Advice about pacing activities, building in time for rest between these, reducing the intensity of exercise, and stopping before fatigue or pain develop, is important. Patients may benefit from relaxation treatment, passive stretching exercises and hydrotherapy or swimming, in which the weight of the body is supported by the water while the joints can be kept mobile. It is essential to try to enable the patient to continue with his or her activities, but to prioritise these and to carry them out in different ways. For instance, if the lawn has to be cut in the summer, this should be done in two or more stages with adequate rest periods rather than all at once. Activities around the home such as washing up should be done while sitting on a high kitchen stool rather than standing if the leg muscles are affected. The height of shelves may need to be altered particularly in the kitchen and broad-handled kitchen utensils used so that they can be gripped with less muscle strength than the conventional types.

Muscle activity can also be reduced by attention to posture and gait. Exercises to improve the mobility of the spine may help. Simple appliances such as a heel lift in a shoe can equalise the length of the two legs, reduce pelvic obliquity, and improve back ache and gait. Calipers for the limbs together with, for instance, lumbar spine supports or a walking stick may enable patients to stand or walk for longer intervals.

Patients who have had polio are often cardiovascularly unfit, particularly if their legs were involved. Individually assessed exercise programmes may improve this. Occasionally malnutrition leads to generalised muscle atrophy which can be reversed following nutritional advice. More commonly however weight loss is required. Obesity increases the energy requirements, may contribute to obstructive sleep apnoeas, and increases the load on damaged or weakened weight-bearing joints.

Mechanical aids or assistive devices may be used to reduce muscle activity. This may be as obvious as a wheelchair which enables the patient to become mobile without having to walk, or a stairlift which enables the patient to use the upstairs level of the home without having to climb the stairs. Carefully positioned handrails may perform a similar function and a walking stick may also be of help with mobility. If the upper airway or chest wall muscles become weak the external aid that is required may be a nasal continuous positive airway pressure (CPAP) system or a ventilator to support respiration. Nowadays this can usually be provided in a non-invasive form either as a nasal mask with a portable ventilator or occasionally as a negative pressure system such as cuirass. These are simpler than the traditional iron lung which was the only non-invasive system available during most of the polio epidemics. Patients may need to be reassured that the need for ventilatory support does not imply the need for an iron lung again. Some patients who have a weak cough or recurrent aspiration may require cough-assistance techniques or even a tracheostomy to protect the airway and to enable ventilatory support to be given. Advice about the consistency of food, head position and tricks to facilitate the pharyngeal phase of swallowing helps to prevent aspiration, and to maintain an adequate nutritional state.

A variety of drugs have been tried in order to attempt to improve muscle function in PPS. These include pyridostigmine, an anticholinesterase inhibitor, which has been promoted in view of its ability to improve neuromuscular junction transmission. Studies of its use have shown that it is no better than placebo and it may even worsen PPS in the long term. Other drugs such as amantadine and selegeline which increase the availability of dopamine in the central nervous system have also been proposed, but have not been effective. Myotrophin, which influences nerve terminal sprouting has also been evaluated. None of these drugs can be recommended in the present state of knowledge. Oral steroids should be avoided in PPS if possible, because of the myopathy that they can cause.

Management of other late effects of polio

The indirect effects of the polio infection such as degenerative changes in the joints and soft tissues may require treatment with non-steroidal anti-inflammatory drugs and occasionally a surgical approach, particularly if weight-bearing joints become unstable. This is most commonly needed for the knee joints. Joint replacements may be required and occasionally even amputation of a useless limb, usually an arm, but it is unusual for a scoliosis in PPS to require a surgical approach. Many patients who had polio develop osteoporosis and preventative treatment should be considered for this. It is also important to assess the psychosocial implications of these late problems following polio, and for other members of the family or carers to understand the nature of the patient’s problems, what measures need to be undertaken and what the outlook is likely to be. Depression may respond to tricyclic or SSRI antidepressants, but betablockers, benzodiazepines and other central nervous system depressants should be avoided.

Conclusion

With this broad-ranging approach the symptoms of the late effects of polio and in particular PPS often improve. Anxiety can be relieved and the previous way of life restored and maintained. The British Polio Fellowship, Ground Floor, Unit A, Eagle Office Centre, The Runway, South Ruislip, Middlesex, HA4 6SE, tel 0181 842 189, acts as a central organisation for patients throughout the UK who have had polio, and provides information and support which most patients find useful. It has local branches and has recently produced a video about the late effects of polio which is intended not only for patients, but also for those involved in their care. Hopefully this will increase the awareness of the new problems that are arising in patients who have had polio and will lead to better provision of care for them across the country.

John Shneerson MA DM FRCP FCCP is Director of the Post-poliomyelitis Unit and the Respiratory Support and Sleep Centre, Papworth Hospital, Cambridge, CB3 8RE. He trained at Oxford and St Mary’s Hospital, London and later at the Brompton Hospital, London. He has a respiratory problems of patients with neuromuscular and skeletal disorders, including poliomyelitis.

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Poliomyelitis – New Problems From An Old Infection Read More »

Unusual Cases: Postpolio syndrome

Unusual Cases: Postpolio syndrome

Patients who thought they had long ago conquered the weakness and disability of polio are now being visited by an unwelcome reminder of their childhood illness.

Paul Ballinger MRCP, MRCGP, DCH, DRCOG

General Practitioner, Cannock, Staffordshire

UPDATE

THE JOURNAL OF CONTINUING EDUCATION FOR GENERAL PRACTITIONERS 4th NOVEMBER 1998 VOLUME 57 NUMBER 8 774-776

Lincolnshire Post-Polio Library copy by kind permission of Dr. Ballinger and the Editor of

UPDATE

The Case

A 55-year-old man asks for a sick note. He claims to have had increasing difficulty driving his lorry recently, and says the task has now become impossible. Weakness in his right leg, which was affected by polio in childhood, is stopping him from operating the pedals, although this has never been a problem before.

Examination reveals profound weakness and wasting of the calf musculature. Despite considerable scepticism, the GP issues a sick note.

A flurry of letters follow from the patient’s insurers, all asking why he has only just developed this problem when the polio attack occurred 30 years ago. It is only after recourse to the literature that the question can be answered. The patient is suffering from postpolio syndrome.

Of the estimated 30,000 Britons with residual limb weakness from old poliomyelitis, about a quarter go on to develop postpolio syndrome (PPS) many years after the original illness. The weakness and muscle pain they endured as children revisits them after 30-40 years of stability, only this time in a more chronic, insidious and deceptive fashion.

PPS comes as a devastating blow to people who have spent a lifetime trying to conquer the functional deficits left by the original illness.

The syndrome involves a constellation of symptoms — new muscle weakness, usually in a limb with previous polio weakness, muscle wasting, muscle pain at rest and with use, arthralgia, fatigue and psychological problems.

GPs face a daily stream of patients with musculoskeletal symptoms, myalgia, arthralgia and fatigue. Most can safely have their symptoms ascribed to degenerative joint disease, soft tissue injury, depression or nonspecific self-limiting causes.

PPS is often misdiagnosed because its symptoms closely resemble and often coincide with these common complaints. However, as a typical group practice is likely to include three or four polio survivors and one patient with PPS, most GPs are likely to see this condition.

DIAGNOSIS

PPS was fully described as recently as 1985.[1-3] Its identification followed numerous case reports in the 1970s and 1980s.

The diagnosis is based on a number of criteria:[4]

history of documented paralytic poliomyelitis in childhood;

partial recovery of motor function and functional stability for at least 15 years;

residual asymmetric muscle atrophy with weakness (Figure 1);

development of new pain and weakness;

no other illness to account for the symptoms.

Figure 1. Atrophy of the calf musculature in postpolio syndrome

Common symptoms

PPS is typically characterised by the development of increasing weakness in a limb previously affected by polio. Some 90% of patients experience pain in the muscle with use, and 40% have muscle cramps at rest. Fatigue, both generalised and specifically on performing physical tasks and activities, is reported by 65% (Box 1).[5]

These symptoms lead to a range of functional problems. New weakness in a leg may result in difficulty with walking or stair climbing. Other activities of daily living such as bathing, dressing and using the toilet may become difficult, depending on the muscle groups involved. Many patients adapt by developing new techniques that preferentially use the unaffected muscles. The onset of functional deficits often has major lifestyle implications — affecting the patient’s ability to work, for example.

Box 1. Major symptoms.

Increasing weakness in the limb previously affected by polio. Pain in the muscle with use.

Muscle cramps at rest.

Generalised fatigue.

Fatigue on performing physical activities.

Less-common symptoms

As well as the major symptoms, a host of others occur with less frequency (Box 2). Some patients report new weakness in a limb or muscle group they had previously considered normal. Usually, this is because the muscle group had a minimal weakness unrecognised by the patient, or because the original polio affected less than 50% of the anterior horn cells supplying that limb and no weakness resulted at the time.

The onset of PPS unmasks this subclinical polio, and the limb becomes weak.

A small number of patients may develop dysphagia because of new weakness in the bulbar musculature. Detailed barium studies have shown that many more have a subclinical involvement of the swallowing mechanism.[6]

Patients who suffered involvement of the respiratory musculature in the original polio attack, for example those who used an iron lung or developed scoliosis, are candidates for further weakness of the respiratory

musculature. This is likely to manifest itself in three ways:

Chronic hypoventilation especially while asleep, leading to nocturnal hypoxia and hypercapnia, which in turn lead to poor sleep, daytime headaches and drowsiness. Some of these patients are treated with assisted ventilation devices.

More frequent chest infections, because clearance of secretions is reduced.

Reduced exercise tolerance, which exacerbates any mobility problems.[7]

Genu recurvatum — hyperextended knee — may occur for the first time in PPS (Figure 2a). The muscle tone around the knee becomes unbalanced through weakness of one group, allowing the deformity to develop. When the knee is hyperextended the leg muscles have to work harder.

Treating the deformity with a leg brace (Figure 2b) which prevents hyperextension reduces the work required to walk a given distance. It also reduces pain in the joint arising from the deformity.

Figure 2. a) Hyperextension of the knee (back knee).

b) Knee-ankle-foot orthosis.

Box 2. Uncommon symptoms.

New weakness in a muscle group previously considered normal.

Dysphagia due to weakness in the bulbar muscles.

Weakness of the respiratory muscles in patients whose respiratory musculature was affected in the original polio attack.

Hyperextended knee.

Psychological effects

As well as the physical symptoms, many patients with PPS have to cope with psychological and cognitive sequelae. These encompass fatigue after mental tasks, problems with attention, concentration and memory, and sleep abnormalities.

One study has shown changes in the reticular activating system on MRI scanning. The researchers postulate that the polio virus damages these neurones at the time of primary infection, and hence cognitive changes can occur with the onset of PPS.[8]

Box 3. Psychological sequelae.

Fatigue after mental tasks.

Attention problems.

Problems concentrating.

Memory problems.

Sleep abnormaiities.

EXAMINATION

Clinical examination is likely to reveal lower motor neurone type wasting and weakness in the involved muscle groups. Fasciculation may be present and be reported by the patient.

Examination of all four limbs is worthwhile, as there may be weakness in a limb that is regarded as normal by the patient.

The most severely affected limbs, particularly the legs, are likely to be cold, and there may be trophic changes of the type found in peripheral vascular disease.

There are no diagnostic tests and the diagnosis is based on the clinical findings.

WHO IS MOST AT RISK?

A well designed study has shown that 28% of all cases of paralytic polio develop PPS 20-40 years later, with a peak incidence at 30-34 years after the childhood illness.[5]

The same study has shown that the risk of PPS is higher if:

the patient is female;

the original attack was severe, with marked residual disability;

if it occurred in late rather than early childhood.

Recent weight gain is strongly associated with the development of PPS.

Once a patient develops PPS, a slow step-wise deterioration is likely, with periods of relative stability interspersed with periods of further increasing weakness. Overall muscle power is lost at a rate of 1% a year.[2]

WHAT CAUSES PPS?

Theories abound, but the jury is still out on the definitive pathogenesis of PPS. The favoured theory

suggests that after an attack of polio the number of lower motor neurones supplying a muscle is depleted by over 50% because of death of the anterior horn cells. The remaining lower motor neurones then attempt to compensate by forming terminal axon branches (sprouts), which innervate neighbouring muscle units that have lost their innervation. However, these new giant neurones with multiple terminal branches are inherently unstable and there is continuous reinnervation and denervation of adjacent muscular units.

A point is reached at which the rate of denervation exceeds the rate of reinnervation, possibly due to metabolic exhaustion of the motor neurone. At this stage, muscle strength is lost, and PPS is believed to

start.[9,10]

MANAGEMENT

Treatment is largely supportive, with the GP playing a central role.

Correct and early diagnosis is probably the most important contribution the GP can make. Delay in diagnosis can lead to anxiety, and compound the patient’s difficulties.

The onset of PPS may mean enforced early retirement, and have a major bearing on social security and insurance entitlements.

Drug treatment has been ineffective. Trials with several agents, including corticosteroids and anticholinesterases, have failed to show any convincing benefit.[11,12]

Interventions that may help include the use of orthotic devices and advice on exercise. Orthotic devices

These are devices that restrict the movement of a joint. Foot drop is a common deformity in PPS (Figure 3a), due to weakness of the dorsiflexor muscle group. If uncorrected, it can lead to a permanent deformity through shortening of the Achilles tendon.

An orthotic device that maintains the foot in dorsiflexion prevents the deformity, and reduces the effort required in walking (Figures 3b and 4).

A knee brace is another example of an orthotic device that may be useful in PPS.

Figure 3. a) Weakness in ankle dorsiflexion (foot

drop). b) Ankle-foot orthosis.

Figure 4. An orthosis for foot drop. Exercise

PPS patients are likely to be unfit because their disabilities preclude vigorous exercise. However, an exercise programme forms an important part of any treatment package.

It has been shown that a graded programme, in which the affected muscle groups are gradually subjected to increasing isotonic exercise over a period of time, can produce increased strength and improved function. Patients should be advised to avoid exercising to the point of muscle pain or exhaustion.

An occupational therapist or physiotherapist can often tailor an exercise programme to suit the individual patient, based on muscle strength in each limb.

REFERRAL

Referral to a rehabilitation specialist is often beneficial. The consultant can then co-ordinate further referrals for physiotherapy, exercise, orthotic devices and other interventions.[13,14]

There are also several support groups, where PPS sufferers can seek additional help (see useful addresses).

The Outcome

The patient’s weakness continued to increase, and later he went on to develop a troublesome foot drop. This was treated with an orthotic device to maintain the foot in dorsiflexion.

The patient also complained of increasingly troublesome rest pain, muscle spasms and cold in the affected limb. A combination of conventional analgesia and a small dose of amitriptyline provided only partial symptomatic relief.

He has been referred to a specialist in rehabilitation, and is slowly coming to terms with this latest phase of a chronic disease.

REFERENCES

Halstead LS, Wichers DO, eds. Late effects of poliomyelitis. Miami: Symposia Foundation, 1995. 2. Dalakas MC, Elder G, Hallet M et al. A long term follow up of patients with post poliomyelitis neuromuscular symptoms. N Engl J Med 1986; 314: 954-63. [PubMed Abstract] 3. Cashman NR, Maselli MD, Wollman RL et al. Late denervation in patients with antecedent paralytic poliomyelitis. N Engl J Med 1987; 317: 7-12. [PubMed Abstract]
Mulder DW, Rosenbaum RA, Layton DO. Late progression of poliomyelitis or forme fruste amyotrophic lateral sclerosis? Mayo Clin Proc 1972; 47: 756-61. [PubMed Abstract] 5. Ramlow J, Alexander M, LaPorte R et al. Epidemiology of the post-polio syndrome. Am J Epidemiol 1992; 136: 769-86. [PubMed Abstract]
Sonies BC, Dalakas MC. Dysphagia in patients with the post-polio syndrome. N Engl J Med 1991; 324: 1162-7. [PubMed Abstract]
Howard RS, Wiles CM, Spencer GT. The late sequelae of poliomyelitis. Q J Med 1988; 251: 219- 32. [PubMed Abstract]
Bruno R, Cohen JM, Galski T et al. The neuroanatomy of post-polio fatigue. Arch Phys Med Rehabil 1994; 75: 498-504. [Lincolnshire Library Full Text]
Cashman NR, Trojan DA. Correlation of electrophysiology with pathology, pathogenesis and anticholinesterase therapy in post-polio syndrome. Ann NY Acad Sci 1995; 753: 138-50. [Lincolnshire Library Full Text]
Dalakas MC. Pathogenetic mechanisms of post polio syndrome. Ann NY Acad Sci 1995; 753: 167- 85. [PubMed Abstract]
Trojan DA, Cashman NR. Anticholinesterases in post-polio syndrome. Ann NY Acad Sci 1995; 753: 285-95. [Lincolnshire Library Full Text]
Stein DP, Dambrosia JM. Amantadine and fatigue in patients with post-polio syndrome. Ann NY Acad Sci 1995; 753: 296-302. [PubMed Abstract]
Agre JC, Rodriquez AA et al. Late effects of polio: critical review of the literature on neuromuscular function. Arch Phys Med Rehabil 1991; 72: 923-31. [PubMed Abstract] 14. Agre JC, Rodriquez AA, Tafel JA et al. The role of exercise in the patient with post-polio syndrome. Ann NY Acad Sci 1995; 753: 321-34. [PubMed Abstract]

USEFUL ADDRESSES

The British Polio Fellowship, Ground Floor, Unit A, Eagle Office Centre, South Ruislip, Middx HA4 6SE, tel: 0181 842 4999; fax: 0181 842 0555.

Provides helpful advice sheets, literature and other support for patients. Most of its work today is concerned with PPS.

Lincolnshire Postpolio Network, PO Box 954, Lincoln LN5 5ER, tel: 01522 888601, web site: http://www.lincolnshirepostpolio.org.uk/.

Informal support network run by PPS patients.

Dr Henry writes about Recovered Memories

Dr. Henry writes about Recovered Memories

In the field of psychology, the topic of recovered memories has been popular for some years. There is considerable controversy regarding these “recovered memories” in legal cases of alleged sexual abuse. Some children may admit to having certain “memories” when questioned by “therapists” who, while pointing to the child’s genital area, may ask very suggestive questions such as “Did Daddy touch you here?” There are even adults who, when involved in this “uncovering” therapy, “recover” long-lost memories of sexual abuse in their childhood.

It is not the purpose of this essay to discuss the pros and cons of recovered memories in a therapeutic or legal setting. I am more interested in the possibility or even probability of recovered memories of early childhood polio. I have noticed that many polio survivors have a renewed interest in memories of their

acute polio experience as a result of their now dealing with Post-Polio Syndrome (PPS). PPS for many is a “déjà vu” experience. Déjà vu means the sensation or illusion that one is seeing what one has seen before. As a result, there is the possibility that some of us are remembering events from the past: feelings such as fear, anxiety, and panic and/or perceptions of an auditory, visual, tactile or even olfactory nature that are reminiscent of our acute polio experience. The older the age of the original polio illness, the clearer the memories may be.

What do we remember from our childhood, especially before age five? Ask yourself this question. I recall starting public school; I recall seeing a steam shovel outside my bedroom window when I was three years old. How and where are these memories stored? The neurobiological basis of learning is located in certain

structures of the brain. The hippocampus, cerebellum, and the cortex areas of the brain are among these structures. There are one hundred billion neurons in the brain involved in forming memory.

Learning begins when the senses take in some perception from the environment and that perception is transformed into a memory link. Long term memories result from the increased time such memories have had to link up with a number of locations in the brain’s cortex. The repeated reliving of a particular memory enhances its permanence and storage. Cramming for an exam will work for the short term, but the crammed information will not likely remain as a stored memory.

Smell and emotion may be examples of our earliest long-term memories. For example, scent information is sent via the olfactory nerve to the hippocampus, the portion of the brain which has a strong role in the control of emotions. Thus, early in development, infants learn to distinguish between pleasant and unpleasant odors and will demonstrate or express an emotional reaction.

Memory and learning are affected by stress. Sometimes stress can increase the production of adrenaline which might actually enhance learning as when one studies for an exam under some pressure. However, if the stress is too great, learning may be inhibited (and performance on an exam may be poor). Most people will learn more efficiently while in a pleasant or positive mood. Thus, most childhood memories are pleasant memories. Most early childhood memories that survive are those associated with the period when

the child learns to speak between the ages of three and five. Before age three, only memories associated with traumatic events or with smell are likely to be remembered.

I would think that most of you would agree that the event of a significant case of acute polio in early childhood would qualify as a “traumatic event.” Involved in this trauma was often the sudden and misperceived separation from parents (trusted love objects) during a sometimes lengthy isolation, being placed in a new and rather sterile environment, and receiving care from total strangers. What did these young children with polio store in their memories? Since smell is one of the earliest memories for storage, perhaps some of these children stored the smell of hot moist wool (Kenny hot packs). Some may have stored the terror of isolation, the fear associated with abandonment along with the shared panic of other crying children. Some may have stored the sensation of claustrophobia, the bellows breathing sounds, or the odor of a sweaty rubber necked collar while in an iron lung.

Many of these bad memories may have been repressed over the years. Repression is a defense mechanism of our minds in which unacceptable ideas, fantasies, emotions, and impulses are unconsciously banished. If the acute polio damaged us, many of us also denied some of the realities of this damage as we grew older. Our unconscious memories have lost their connection with verbal symbols. When some stimulus or skilled treatment helps to reconnect these forgotten memory traits, fragments or even clear memories may be recovered.

I have noticed in conversations, interviews, and in dialogue over the Internet with numerous PPSers, that many who had polio as preschool age children have recalled memories of odors and separation anxiety feelings. Also, some of us have recovered some details we long ago forgot as a result of sharing our polio memories in support group discussions.

Some time ago, at one of our regular meetings, we divided into small groups based on the age we initially contracted polio. We had a “toddler” group, an elementary school age group, and a late adolescence/adult age group. This process was most conducive to the sharing and recalling of many memories. Two members of our group, Shirley Miller and Pat Poole, who were hospitalized at the same time in 1950 in DePaul Hospital in Norfolk, Virginia, actually had some shared memories, which is rather unusual among support groups. Because of the Internet, I have discovered a lady, Jane Shaw, who lives in Florida. At age four, Jane was at the Medical College of Virginia Hospital with polio during the same time as myself. Regarding her polio memories, she wrote me:

“I do remember vaguely being in the iron lung, but I don’t recall having any fear of it. I remember not liking a lot of the food that was served and being made to eat it! To this day, though, if I walk into a hospital, the smell will give me a chill — so I guess I do have some repressed memories!”

She still remembers the ordeal of distasteful food and the emotions associated with a hospital smell as she did at age four. Together, we have remembered some doctors and nurses names. A shared experience is often remembered with more ease.

It seems plausible, as some of these memories are recovered, that some delayed symptoms of posttraumatic stress disorder (PTSD) may occur. PTSD could involve some depression, anxiety, flashback experiences, sleep disturbance, startle responses, disturbing dreams and even panic feelings. I have noted that many have reported such symptoms in dealing with PPS. Perhaps, some of these symptoms are a variant of delayed PTSD or simply related to PPS itself. I think this would be most interesting to examine among toddler or preschool age polios.

If you have the capacity to venture way back into the recesses of your memory, you may remember more than you ever thought possible about your earlier experiences with the polio event in your life. I challenge you to attempt this exercise. Share what you remember with someone or come to one of our meetings and

share with our group. Our group is most interested in listening. If you find the memories too painful to verbalize, write about them and send the text to our newsletter editor. It is my belief that recovering and dealing with these early memories has a beneficial effect on our adjustment to our lives with PPS. A living witness to a life with polio is well worth examining and sharing.

References:

Synopsis of Psychiatry, Seventh Edition by Harold I. Kaplan, MD, Benjamin J. Saddock, MD, and Jack A. Grebb, MD, Williams and Wilkins, Baltimore, 1994, pp. 170 and 242.

The American Psychiatric Association’s Psychiatry Glossary distributed by the American Psychiatric Press, Inc., Washington, D.C. 1984, pp. 26 and 119.

Henry Holland, Richmond., Virginia, USA. Henry4FDR@aol.com

November 1998

Originally published in the Central Va PPS Support Group (PPSG)‘s newsletter, The Deja View, in 1998.

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

Created: 20th November 1998

Last modification: 24th January 2010.

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