PSN Library Files – Page 8 – Polio Survivors Network

Contribution of Childhood Physical and Emotional Trauma to the Development of the Post-Polio Personality

THE CONTRIBUTION OF CHILDHOOD PHYSICAL AND EMOTIONAL TRAUMA TO THE DEVELOPMENT OF THE POST-POLIO PERSONALITY

Nancy M. Frick, M.Div, Lh.D.

Frick NM. Proceedings of the Ontario March of Dimes Conference on Post-Polio Sequelae. Toronto: Ontario March of Dimes, 1995.

Revised April 2000.

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

Introduction: Jeannette Shannon

The violinist Itzhak Perlman once stated, ” When you sit in a wheelchair, you lose your height and you become an ‘it.’ You are a disability.” He went on to say, “I am tired of being a human interest story, a brave handicapped musician. I am a musician for whom life is not easy. But then, is life easy for anyone?”

Our next partnership is with Dr. Nancy Frick, educator, author, counselor and researcher specializing in the psychology of disability. She was one of the founding members of the International Post-Polio Task Force and wrote the first article in the medical literature on the psychological effects of post-polio sequelae. With Dr. Richard Bruno, she conducted the national surveys of polio survivors that documented the relationship between psychological stress, Type A behavior and post-polio sequelae . Dr. Frick received Bachelor and Doctor of Humane Letters degrees from Baldwin-Wallace College. She also received a Master’s degree in Counseling from Drew University and did clinical training at the American Foundation of Religion and Psychiatry at New York Hospital, Cornell Medical Center. Dr. Frick created and is currently the Executive Director of Harvest Center in Hackensack, New Jersey, which provides consulting and education for corporations and people with disabilities, with special emphasis on post polio sequelae and compliance with the Americans with Disabilities Act. Her topic this morning is: The contribution of childhood physical and emotional trauma to the development of the post-polio personality.

Dr. Nancy Frick

Do you remember the old TV program Truth or Consequences? The master of ceremonies used to ask questions and if contestants answered them correctly, they got lots of great prizes, dishwashers and so on, and presumably went home and lived happily ever after. But, if they answered the questions incorrectly, terrible things would happen to them. As you read on, you will be asked many questions about yourself. If your answers are incorrect, the consequences for you will be Post-Polio Sequelae (PPS).

The term “polio survivor” has been used in our community as if anybody who survived the acute stage of polio is a survivor. But I believe that there has got to be more to surviving than that. Surviving ought to mean more than just being alive, but feeling dead. Surviving should mean having quality of life and living without dread, without that clutching feeling in the pit of your stomach because you are so busy pleasing everybody else that you never get around to living your own life. I believe that being a polio survivor should include the possibility for inner joy and not just the tremendous pressure for success that so many people who had polio demonstrate.

I will link the origin of Type A behavior to events that occurred during the acute stage of polio and after polio survivors recovered from the acute stage. I will then show how Type A behaviors actually can cause post-polio symptoms. But I am not going to stop there. I will present a plan that will help polio survivors stop being Type A, reduce their post-polio symptoms and help them become true polio survivors.

Are You Type A?

Most people in the post-polio community have either heard or read that polio survivors have Type A personalities. If you had polio, do you believe that you are Type A? Take a moment and complete the brief Type A questionnaire that we use in our research and is included in harvest center’s Computerized Assessment Program for PPS. For those who are either retired or on disability, please answer the questions as you would have answered them when you were still working. Consider yourself employed full-time if you are a homemaker.

YES	NO	I usually wake up not feeling well rested.
YES	NO	I enjoy competition.
YES	NO	I consider myself to be hard driving.
YES	NO	I have a temper that is hard to control, a temper that’s fiery.
YES	NO	I set at least one deadline a day for myself.
YES	NO	I set at least one deadline a week for myself.
YES	NO	It is very important for me personally to get ahead in life.
YES	NO	I usually spend less than five days on an average vacation.
YES	NO	I spend more than eight hours a week doing overtime work at home.
YES	NO	I have taken less than one vacation a year during the last five years.

If you answered “yes” to five or more questions, you have the hard-driving, pressured, perfectionistic Type A personality. On average, people who did not have polio answer “yes” to four or fewer of these questions, while polio survivors answer “yes” to six or more.

If polio survivors are so Type A, the question is why and what difference does it make in terms of their being true polio survivors? To answer these questions, polio survivors have to go back to their original experience of polio. But, going back is a problem, because most polio survivors don’t want to remember having had polio. Most say, “I had polio, I got over it and don’t ever want to think about it again, thank you very much.” But, polio is a pivotal part of any polio survivor’s personal history and keeping it buried actually causes psychological damage. Yes, it hurts to think about it. But the only way to get a handle on why polio survivors are so Type A today is to remember and deal with the experiences they had yesterday and have so carefully hidden from themselves.

Polio: The Initial Terror.

There are many painful memories associated with the acute stage of polio. First, if polio survivors were old enough, they knew that they had been stricken by the ‘greatly feared disease.’ Polio survivors were the AIDS people of their generation. Nobody wanted to have anything to do with them. Everybody was terrified of polio survivors, and even their families, for years. Second, if polio survivors were old enough to know that they had had polio, many thought that disability was likely and that death was at least possible, especially if they knew someone who had been disabled or had died from polio.

The Hospital Experience: Being Away from Home.

There is a third issue which is even more disturbing. There was the confusion and terror connected with polio survivors’ being wrenched from their homes, dropped in a hospital and being left there for months or even years, while they received treatments that no one explained to them. Polio survivors went to the hospital, usually when they were very young, and lost their emotional support system.

A Texas man had polio when he was five. When I met him he was 52 years old, a colonel in the United States Air Force, 6 feet 4 inches tall, extremely strong and was beginning to have PPS. He had completely repressed the entirety of his original polio experience until the day he heard me speak. While I was talking, it came back to him in a flood. I mean a literal flood because when he came up to talk to me, tears were streaming down his face. He didn’t want to remember and when you hear what happened to him you will understand why.

When he was five years old, he was taken 250 miles from home, admitted to a hospital and didn’t see his mother for two months. When he he did see her again, she was on one side of a glass window and he was on the other. She couldn’t hold him; she couldn’t comfort him. Those who are parents know that when your children are ill, the one thing you want to do is to be able to hold and comfort them. He said to me, “I don’t like how I feel about remembering being in the hospital and I don’t like remembering how I felt afterwards either. All I will say is that it took me a lot of years to begin to like people, or trust them.”

A six year old girl from Ohio was so psychological traumatized by her hospitalization that she forgot what her sister and home looked like, lost control of her bodily functions and attacked the staff.

A man from New Jersey, who had polio at the age of five, was so overwhelmed by what happened to him when he got to the polio hospitalt hat he forgot his name. For two weeks he remembers constantly asking every medical professional who went by, “Who am I? What’s my name?” He lost the certainty of who he was in the hostile and sterile environment of that polio hospital.

A man in Florida, who had polio when he was four, had parents who were so displeased that he was ill, they admitted him to the local polio hospital and never came back. When he was twenty-two and old enough to discharge himself, he left the hospital on his own. He hasn’t seen his parents to this day.

You may think these stories unusual, but they are not. Polio survivors were traumatized just by being in the hospital, especially if they were young. In his book Loneliness, Clark Moustakas says this about the childhood experience of being in a hospital:

Because of his inability to take care of himself in the all-important functions, the possibility of being abandoned or left alone is the most serious threat to the child’s whole existence. Of the many kinds of temporary abandonment, no experience is more desolating to a child than having to be in a hospital alone. The disrespect for the integrity of his wishes and interests, the absence of genuine human warmth, all enter into the loneliness of hospital life.

The Hospital Experience: Medical Abuse.

Not only was being in the hospital emotionally painful, physically painful things happened to polio survivors in the hospital. They had painful and frightening therapies. Many people were burned by hot packs. Many had the nightly experience of being splinted and there being no one willing to remove the splints when they began cutting into the skin; being braced, having physical therapy in pools, even though they couldn’t swim. Few polio survivors had their questions about these therapies answered. It was polio epidemic time. Who had time to explain things to children? The staff became angry when asked questions, sometimes responding, “You want to get out of here, don’t you?” Think about the content of that question. Imagine how it would sound to a child; it was ominous. It seemed to mean that if patients didn’t do exactly what they were told, they were never going to leave the hospital. Research has shown that failing to explain treatments and provide information concerning illnesses makes children think they are sicker than they really are, and allows their inherent fear of death to surface.

As if physical pain and terror weren’t enough, polio survivors were abused by the medical staff. For years before I got polio, I had frequent nightmares and I would wake up in the middle of the night crying. My mother would comfort me. When I entered the hospital the nightmares continued. There was a night nurse at the hospital who did not like my crying and did not comfort me because I sometimes would awaken the other patients. When she came to my bed she would say in a very threatening voice, “You stop crying or you are going to be sorry.” She frightened me for two reasons: I knew I couldn’t control the nightmares and I didn’t know what she would do to me when I cried again. Then, one night I found out. I awoke crying from a nightmare; she came in and said, “I told you not to do this or you were going to be sorry.” She pulled me in my hospital bed into a walk in linen closet, turned off the light, closed the door, locked it and left me there until morning. Such an experience can leave permanent emotional scars. Dozens of polio survivors have told us that they were locked in closets or placed in straight jackets to teach them to behave in a manner more acceptable to the hospital staff. There was the worst kind of abuse. Many polio survivors were sexually abused. One polio survivor was regularly sexually abused by someone on the hospital staff. Yet, she told no one about it. She said, “I was there, they had to take care of me, so I just felt that I had to smile and be a good patient and just grin and bear it.”

Unfortunately, polio survivors were helpless, at the mercy of hospital staff, with a complete loss of control over their lives. Most polio survivors couldn’t move. Some couldn’t feed or toilet themselves. If those who had polio were to have their most basic survival needs met, they had to depend on the medical staff even if they were emotionally, physically or even sexually abusive. Normal, child-like behaviors, such as crying

and asking questions, were punished. Fears of abandonment by their parents made them certain they were totally dependent on a staff that behaved in ways that made them constantly feel, as one polio survivor described it, “As if I was in mortal danger.” Polio survivors had little choice but to suppress their fears and follow their parents’ well-meaning parting instructions, “Be good. Don’t make any trouble and do everything you are told.”

When a child forms a fear so strong and so basic at so young an age, the child takes that fear with him forever and applies it to every other relationship that is formed for the rest of his life. Many polio survivors learned to deal with the feelings of abandonment, loss of control, fear and pain by appearing happy, because it was too dangerous to examine how they really felt, and complying fully with any external expectations. Polio survivors learned to deny their personal needs, feelings, pain, and, worst of all, deny their own identity. This can be seen from a summary of The Good Chart written by the children on the polio ward at Baltimore City Hospital:

Listen to the doctors.

Obey the nurses.

Do not fight.

Do not be bad.

Be good in school.

Do your homework.

Do not talk at dinner or in school.

Little folks should be seen and not heard.

The Return Home.

That Good Chart became the prescription for proper and successful behavior when polio survivors returned home. Polio survivors learned very young and very well how to stay out of mortal danger. But, returning home was no picnic, either. Many people remember being the one child in the neighborhood with whom no one was allowed to play because they might give everyone else polio. There are many documented cases of people actually crossing the street to avoid walking in front of a home where someone was known to have had polio. But, even if polio survivors were allowed to play with the neighborhood children or their former friends, polio survivors often couldn’t keep up with them because of polio’s residual effects. Or, polio survivors were ostracized or teased because they looked disabled or were in any way different. Many polio survivors became isolated, felt unwanted, unworthy and for some reason guilty. Many felt that they had done something terrible or they would never have gotten polio.

Another source of emotional pain polio survivors experienced is being stared at. For a child, being stared at is a torment. Many polio survivors learned to be afraid of children not only because they stared but also because of their endless questions. How do you deal with questions about your disability when you don’t want to talk about it, when you don’t want to think about it, when you don’t even want to admit that you had polio? Because many polio survivors thought so little of themselves, at some level they felt the stares or impolite nosiness of strangers could reduce their value to nothing more than that of a momentary curiosity. Some polio survivors actually believed that curiosity could hurt them and, as a result, it did. Fear of children and resentment of anyone who stared, made polio survivors feel even more alienated, unacceptable and different.

Getting Normal

But, there was hope! The idea was to appear as normal as possible. Get rid of those canes and crutches;

get rid of those braces; get rid of that wheelchair and respirator! Get Normal! In the hospital, assistive devices helped polio survivors regain function. But in the community, braces, crutches and wheelchairs were obvious proof that polio survivors hadn’t worked hard enough to have fully recovered. Necessary assistive devices were discarded as quickly as possible in order to look normal, regardless of the discomfort, fatigue, awkwardness, pain and actual danger in which polio survivors placed themselves. Why did polio survivors do that? Because polio survivors had to Get Normal.

Physical therapy was begun again when polio survivors returned home from the hospital, or begun for the first time for those who weren’t hospitalized. Research had shown that there was no benefit to physical therapy for polio survivors after two years. Yet, many of them had therapy for decades after the acute polio. Why? To Get Normal, of course. One individual who had polio at age five was a triplegic wheelchair user from the time she had polio. When she went home from the hospital, her parents wanted her to walk, even though it was extremely dangerous for her to do so. Physical therapy was continued until she left home for college. Although she could only walk a few steps with crutches, even though the danger of falling frequently was very real, her parents thought walking was better than using a wheelchair. And why was that? They wanted her to Get Normal.

Another client was beaten by his father as motivation to walk without braces. This particularly sensitive individual told his son, “I’d rather see you dead than a cripple.” Now why would a parent who allegedly loves his son say that to him? To make him Get Normal.

Abuse by parents was usually less blatant. Few polio survivors left the hospital and returned to a house that was accessible Why? They had been in the hospital for months, sometimes even years. It wasn’t a surprise to their parents that they had a disability. Yet, many polio survivors lived in houses that made it impossible for them to take care of themselves. Basic activities of daily living, such as bathing, dressing, going to the toilet, were impossible. Many were dependent on a parent who might consistently withhold help, or help only when it was convenient. Roberta Trieschmann tells the story of a girl who had polio who returned home to a very rejecting mother. She allowed her daughter to use a bed pan only three times a day: when she awoke, at noon and at 8:00 P.M. Beyond that this child was on her own – and she was in trouble if she had an accident.

Some parents accepted their child who had had polio. But, parents often showed their children that they were unacceptable if they appeared disabled. Abuse at home may explain the findings of a study of 100 children discharged from the Baltimore City Hospital polio ward. A number of patients actually wanted to go back to the hospital. This finding sounds unbelievable. But, could it be that the children who wanted to go back to the hospital were living in homes that were inaccessible where they were being abused by their parents?

Other parents abused their child subtly. For example, there were over-protective parents who would not let their child out of their sight and say, “Oh no, honey, you shouldn’t do that, you might hurt yourself.” This sounds good to the neighbors; it sounds good to the family; but it wasn’t good for the child.

If a child recovered physical abilities, which most polio survivors did, some parents tried to forget the fact that they ever had had polio by expecting them to equal or even to exceed their previous physical performance. We know of one family who took up hiking for the first time when their son came home from the hospital using crutches and braces. He climbed over rocks and hills because he wanted to be like the rest of his family. Other polio survivors say that they were expected to outperform their peers academically. Some polio survivors performed academically, not for themselves, but to please everyone else. Any grade that was not an “A” meant that they were not perfect. And if they weren’t perfect, they were worthless. It’s amazing what polio survivors’ conditioning caused them to do to Get Normal.

Still other parents, who were shamed by their child’s disability but unable to deny it because of how visible it was, isolated their families from society. The mother of one man, who had a slight limp after his polio, restricted him to the house and would not let him leave the yard until he was eighteen years old. Many polio survivors experienced isolation which impaired their ability to develop social relationships. Also, and this is critical, if polio survivors parents’ felt shame due to their disability, they learned to be ashamed of it, too. These polio survivors learned to expect that everyone they met would reject them. Their parents had rejected them; why wouldn’t everyone else?

What did polio survivors learn from these experiences of dependence and rejection in the hospital and at home? They learned that they had better Get Normal and stay that way. They learned that they had better maximize whatever physical abilities remained, be smarter and work harder than anyone else. Then maybe, just maybe, they would be allowed to participate in society and be marginally accepted, but only if they very cleverly hid their polio. So, polio survivors became Type A overachievers and exquisitely sensitive to criticism and failure. They met the needs of everyone in their lives except themselves. Polio survivors became extremely successful at everything they did on all levels: academically, professionally and personally. Furthermore, polio survivors discarded nearly all of the original assistive devices that they were given and learned to walk without them. They disappeared into society if they could. Those polio survivors who could hide it didn’t want anyone to know that they had ever had polio. One individual said that polio survivors have gone beyond being Type A to being ‘Type E,’ doing everything for everyone every minute of every day. Why do you suppose polio survivors are Type ‘E?’ To Get Normal, of course.

Polio Survivors versus The Vampires.

I have a name for folks who are Type A, and especially those who are Type ‘E,’ and spend more time worrying about what others think about them than they spend taking care of themselves: I call them Vampire Bait .

The world is full of people who want you to do anything they ask, who are eager to suck the life blood right out of your body. You remember that boy who went hiking with his family on crutches and braces? What was he? Vampire Bait. Instead of meekly complying so he would Get Normal, he needed a new inner voice. I am going to give you a new mantra to replace the voice in your head telling you to Get Normal. This is what that boy – what all Type ‘E’ polio survivors – should say when people ask them to do things that are unreasonable, painful and actually dangerous: Get Real! When your boss asks you to do his work and you do it; when your boss asks you to work late again, or every weekend, and you do it, what are you? Vampire Bait. What should you say to the Vampires? Get Real! Practice your new mantra, your new inner voice. Go home, look in the mirror, picture your Vampire boss, your mother, your neighbor and say, “Get Real!”

How many of you have had Thanksgiving dinner in your home for the last 87 years, inviting approximately 372 people to the meal? There are fourteen gourmet courses, all of which you must prepare yourself. Let’s just say for the sake of argument that you decide it’s time to start taking care of yourself, start treating your PPS, and you’re not going to do Thanksgiving next year. So, you are sitting around the table with the 372 people you’ve invited, and you say, “Listen, is there somebody here who would like to make Thanksgiving dinner next year?” What do you hear? Stony silence. The roasted turkey is making more noise than your guests. The problem with which you have to deal at that split second is the voice in your head that says, “Oh, that’s okay, I don’t mind cooking because I hate to ask for help, I don’t want to become dependent, I don’t want to upset anybody.” If you listen to that voice and make Thanksgiving dinner again and again and again, what are you? Vampire Bait!

Why do you keep doing Thanksgiving dinner again and again and again? To Get Normal, of course. Also, doing for everyone is a form of control. When polio survivors were in the hospital, being treated by medical staff, they had no control over what was done to them. So later in their lives, having control became vitally important. When you hear the stony silence when no one offers to cook Thanksgiving dinner, another voice in your head says, “Thank God! Nobody can do Thanksgiving as well as I can. I am needed! I am earning my keep. I really am worth something. I have a right to draw breath.”

It’s time that polio survivors all Get Real. Remember, if you keep doing absolutely everything that you have been doing all these years, if you keep being ‘normal,’ why should anybody believe that you need help? Polio survivors must learn to say, “Listen, I have PPS and it’s not good for me to make Thanksgiving dinner alone anymore. I am valuable just because I’m me, not just because of what I do. Now, are there any volunteers?”

Polio survivors know that physical over-exertion and emotional stress are the first and second leading causes of PPS. If you continue to listen to the voices of the Vampires in your head and keep trying to Get Normal, your constant working, doing, taking care of others and rushing around will give you PPS and kill your spirit slowly – and in a socially acceptable way.

Getting Real: You Can’t Fool All of the Polio Survivors All of the Time.

I wonder sometimes if polio survivors’ real fear is not of dying, but of being alive and looking disabled. Polio survivors have been trying to fool people, including themselves, into believing that they are not disabled. Let me use myself as an example: I had polio at six and have always used a wheelchair since then. I have never been able to walk. At the age of twenty-eight, I saw myself by accident in a full-length mirror and was shocked to see myself in a wheelchair. When I enter the room , everybody else knows I’m in a wheelchair. How did I fool myself for twenty-two years? I Got Normal, of course.

Hugh Gallagher’s book FDR’s Splendid Deception describes the ultimate example of trying to fooling yourself and the entire world. President Franklin Delano Roosevelt was a polio paraplegic. He couldn’t stand a tall without braces to his hips. Not only he, but the entire country, went along with the deception that he wasn’t disabled at all. If you want to know what it means for a polio survivors to fool himself, you should read this book; but, while you are reading it, remember you may also be reading about yourself.

In our first national survey, we asked the question “Did you consider yourself disabled when you first had polio?” Most respondents said “No.” However, in the same survey, polio survivors were asked another question: “When you got PPS, did you feel you had a second disability?” Most people said “Yes.” Apparently, polio survivors knew they were disabled by polio, they just weren’t admitting it. The question is, are polio survivors still unwilling to give up their pretense and activities; are they willing to do whatever it takes to keep up appearances in spite of PPS? Would they rather be in pain and continue to slowly lose function rather than Get Real with themselves?

It is difficult to Get Real after all that polio survivors have experienced during their lifetimes. It is not impossible, but it’s very difficult. The voices of the Vampires have become the voices you hear everyday in your head, telling you what is good and what is bad about you. How many polio survivors are spending today planning for the future, living in the future? If your mind is busy fretting about the future you are missing today, you are missing this moment. And your life is literally slipping through your fingers while you plan later.

Getting Normal = PPS but Getting Real = SURVIVAL.

The original polio equation of Getting Normal = SURVIVAL is now actually causing PPS. Polio survivors must realize that today, Getting Normal = Progressive Disability from PPS. Your body is telling you to slow down, but your conditioning and fear keep telling you to run or the Vampires will get you.

Can polio survivors stop their PPS symptoms and become ‘true’ polio survivors? Psychologist Al Siebert became very interested in what he calls the Survivor Personality when he joined a group of paratroopers that had been trapped by Communist armies in Korea. Nine out of ten of the men in that unit had been killed. As Siebert got to know the survivors of the unit, he began to wonder what had allowed them to survive when most of their buddies had been killed. He found out it wasn’t just luck or fate that had saved them, but that there was something specific about them that tipped the scales in their favor. He discovered that they had a complexity of character traits that looked to him like a union of opposites that shouldn’t really appear in the same person. Then he realized it was because of those opposite character traits that they were able to survive. The opposite traits offered them an infinite group of inner possibilities that made them infinitely flexible. Whatever reaction was required in any given situation, they were able to provide it. Look at the list of traits in the survivor’s ‘split personality:’

COLUMN A	COLUMN B
Flexible	Stubborn
Pessimistic	Optimistic
Serious	Easygoing
Excitable	Calm
Involved	Detached
Thorough	Impulsive
Predictable	Unpredictable
Cautious	Trusting
Mature	Childlike
Sensible	Emotional
Conforming	Individualistic
Self-critical	Self-confident
Hard working Playful

If you compare these two lists, I would guess that the Type A polio survivor has more traits from Column A than Column B. Take a look at Column B because these are the traits that one needs to develop to become a true polio survivor. It is possible; you can do it. You have all of these traits inside of you; they are just underdeveloped. The more pairs of traits you possess, the more flexible you are, the more successful you will be in dealing with the physical and emotional trauma of PPS and the more likely it is that you can learn to give up being Type A and say “No!” to the Vampires.

Remember these words of an 85-year old man who learned he was dying:

“If I had my life to live over again, I’d try to make more mistakes next time. I wouldn’t be so perfect. I’d relax more. I’d be sillier. In fact, I know very few things that I’d take so seriously. I’d take more trips. I’d go to more places I have never been to. I was one of those people who lived sensibly and sanely hour after hour and day after day. Now, I had my moments, and if I had it to do over again, I’d have more of those moments. Moment by moment by moment. If I had it to do all over again, I’d start barefoot earlier in the Spring and stay way later in the Fall. I’d watch more sunrises and I’d play with more children, if I had my life to live over again. But you see I don’t.”

And neither do polio survivors. Don’t wait until you are 85 to start living. Be a true polio survivor starting now. How do you develop a survivor’s split personality? Here is the golden rule for becoming a true polio survivor:

If you are not anxious and guilty, you are not a true polio survivor.

Sound crazy? Think about why it is true. If you start taking from Siebert’s Column B and saying “No!” to the voices in your head and to the Vampires, what do you think is going to happen? First, you are going to start feeling better. You are going to feel less fatigue, less pain, less muscle weakness. Then you are going

to become absolutely terrified. You will be slowing down, both in doing and thinking, so there will be time for fear to surface, fear that the Vampires will get you. You are going to begin to worry: “Will my friends and family still want me? Will I be ostracized again? Will the Vampires get me?”

Taking care of yourself for the first time in your life will make all the old feelings of guilt and worthlessness surface. (Will the Vampires get me?) If you ask others to pull their own weight, those awful feelings of helplessness and lack of control from when you originally had polio will return. (Will the Vampires get me?) Polio survivors worked so hard to be independent. They had all that physical therapy and endured all those operations and now somebody wants them to walk less, to use a cane or a wheelchair? The doctors want them to look disabled again? (The Vampires will surely get me.)

Those decades of fear, of conditioning to Get Normal, make psychotherapy necessary if polio survivors are to have any chance to Get Real. The way to test whether a polio clinic is good is to find out whether it has a “shrink” who knows about what polio survivors have gone through and what PPS really means to them. Polio survivors all need some help, somebody to talk to and work with, to help them tolerate the anxiety, the guilt, the fear, the loss of control, and even fear of dying, that the changes in lifestyle and appearance needed to treat PPS are going to cause.

When you become frightened, know that fear is normal, and remember this quote from Grace Hansen: “So often our focus upon death and the possibility of dying, is an escape from our real fear: that of living our lives,” living our lives with an obvious disability.

When Getting Real becomes tough, remember this prose poem written by Ruth Mihalenko after thinking about herself as a child with polio:

Where is that little girl?

She is sick, her family is concerned.

Where is that little girl?

She’s been taken apart and put together again by the surgeons.

Like a fledgling about to leave the nest for the first time she is testing the waters of a normal life. Can she catch up? Can she achieve? Can she excel?

Certainly, she must.

Lost time must be made up.

A lifetime of memories must be crammed into a shorter period.

Where is that little girl?

She is not one hundred per cent.

There is new weakness; there is new pain.

Fatigue sets in long before she is willing to deliver her body to rest or to sleep.

Where is that little girl?

She has been taken apart and put back together again by the surgeons. Braces put her back on her feet. Just as she has been alone with the pain and weakness and fatigue, she is alone in her acceptance and determination.

Her muscles will never grow strong again, but she will be strong again.

She is strong.

Where is that little girl?

She is here and now.

Who is that little girl?

That little girl is me.

Life is a dynamic process filled with changes. Some are frighteningly unwelcome at first. But if you can learn to love yourself and tell the Vampires to go bite someone else, you will start to feel less anxiety and guilt, and become a true polio survivor. I recently received a letter that said in part:

Beginning March ninth of this year, I have made some dramatic changes. Since I have begun this alteration of behavior, I have been feeling better. My cohorts and friends and family members have been very supportive. They have said, “It’s about time!” Funny thing is, I had always expected something horrible to happen when I started taking care of myself. But, it hasn’t. I feel reborn.

I wish each of you your own personal rebirth

REFERENCES

Bruno RL, Frick NM, Cohen, J. Polioencephalitis, stress and the etiology of Post-Polio Sequelae. Orthopedics, 1991, 14 (11): 1269-1276. [Lincolnshire Library Full Text]

Bruno RL, Frick NM. The psychology of polio as prelude to Post-Polio Sequelae: Behavior modification and psychotherapy. Orthopedics, 1991, 14 (11): 1185-1193. [Lincolnshire Library Full Text]

Bruno RL, Frick NM. The origins of “Type A” behavior and stress-induced symptoms in polio survivors: Family pressure or physiological predisposition? Proceedings of the Society of Behavioral Medicine, 1989, 10: 85.

Bruno RL, Frick NM. Stress and “Type A” behavior as precipitants of Post-Polio Sequelae. In LS Halstead and DO Wiechers (Eds.): Research and Clinical Aspects of the Late Effects of Poliomyelitis. White Plains: March of Dimes Research Foundation, 1987. [Lincolnshire Library Full Text]

Frick NM, Bruno RL. Post-Polio Sequelae: Physiological and psychological overview. Rehabilitation Literature, 1986, 47 (5-6): 106-111. [Lincolnshire Library Full Text]

Frick NM. Post-Polio Sequelae and the psychology of second disability. Orthopedics, 1985, 8 (7): 851- 853.

Gallagher HG. FDR’s Splendid Deception. Arlington: Vandamere Press, 1994.

Moustakas CE. Loneliness. Englewood Cliffs: Prentice Hall, 1961.

Siebert A. The Survivor Personality. Portland: Practical Psychological Press, 1993. Trieschmann RB. Aging with a Disability. New York: Demos, 1987. The Lincolnshire Post-Polio Network

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Document Reference: <URL:http://www.zynet.co.uk/ott/polio/lincolnshire/library/harvest/frick.html> Created: 22nd January 1997

Last modification: 29th January 2010.

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Bollenbach Polio Biology 10

Polio Biology X

In PPS Manual Muscle Testing Problems Arise From Judgement and Biology

Eddie Bollenbach

A Lincolnshire Post-Polio Library Publication

18th March 2000

When acute polio struck it was essential to measure the extent of paralysis quickly. One of the techniques used was manual muscle testing. There isn’t too much to it really. The physician holds a hand against a patient’s limb and coaches the patient to push as hard as possible. Depending on the judgement of the tester the patient’s muscle strength is graded from 1, (Trace Strength), to 5, (Normal Strength). This assessment was very valuable because it provided a clinician with enough resolution in measurement to quickly evaluate the extent of neuromuscular paralysis from acute polio in one session.

From the standpoint of muscle biology these tests made sense too. During acute polio, motor nerves, and all their branches to muscle fibers, die. The muscle fibers are then unable to receive motor stimuli from the brain and spinal cord to induce voluntary movement. A manual muscle test would quickly show that many or all muscle fibers were orphaned and had no ability to do work. Appropriate therapy could then be initiated.

The biology of Post-Polio Syndrome is distinct from that of acute polio. In PPS end fibers of motor nerves begin to lose function. This is a slow process which involves fatigue more often than muscle incompetence. Let me explain. If a normal person is standing it is possible that 30% of the muscle fibers in his leg are contracted to maintain his upright position and posture. When those 30% get tired they automatically rest (switch off) — isn’t the human body an ingenious creation? — and another different 30% of fibers contract to allow the first group to recoup. This can go on for hours. Someone with PPS may have only 40% of his original muscle fibers. When he or she stands they have only 10% in reserve, so in a short while there are no substitutes to take the load off. The result is fatigue of contracting muscles.

In the case described above what would show on manual muscle testing? On contraction against a clinician’s hand strength may show normal (5). But this is instant strength, which is what manual muscle testing measures. Many people with PPS are not down in the (1) trace range, especially if they recovered well from their acute polio. They are in the 5,4,3, and 2 ranges.

Dr. Sharrard in 1953[1] found that in order to identify any weakness, by this kind of testing, more than half the anterior horn cells had to have been destroyed during the original polio infection. Other clinicians have also demonstrated the problems with manual muscle testing in grading PPS involvement.[2,3]

The biology of PPS explains the problem. What we should be looking for is fatigue in previously involved muscles, or less frequently in uninvolved muscles. Manual muscle testing just doesn’t do that. The PPS health professional should be clear about what is being measured by manual muscle testing. It may be, for many, that what is being measured is the original weakness and not the new loss.

References.

Sharrard, W. J. W.: Correlations between the changes in the spinal cord and muscular paralysis in poliomyelitis. Proc. R. Soc. Lond. 40:346, 1953.
Perry, J.; Barnes, G.; and Gronley, J. K.: The postpolio syndrome. An overuse phenomenon. Clin. Orthop., 233: 145-162, 1988. [Lincolnshire Library Full Text]
Grading For Manual Muscle Testing (attributed to Dr. Perry – Rancho Polio Clinic). [Polio Survivors’ Page] <URL:http://www.eskimo.com/~dempt/grading.htm>

The Lincolnshire Post-Polio Network

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

Created: 25th January 2000

Last modification: 21st January 2010.

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Bollenbach Polio Biology 9

Polio Biology IX

Peering at Post-Polio Syndrome under the Microscope

Eddie Bollenbach

A Lincolnshire Post-Polio Library Publication

25th January 2000

The Microscope is an instrument that revolutionized the study of biology. Before it existed everyone believed the human body was only composed of amorphous flesh. After Galileo and Janssen, the simultaneous inventors of the earliest microscopes at the beginning of the 17th Century, we knew that we were composed of tiny structures called cells. The human body contains about 10 trillion cells, small circumscribed entities with an organized internal anatomy and a complicated biochemistry. These parts together are the essence of life.

In human beings there are cells of many different types. For our purposes we will concern ourselves with just two of the types of cells that are affected, both acutely and persistently by polio: motor nerve cells and, secondarily, striated muscle cells.

A fairly accurate mental picture of a motor nerve cell can be conjured up by imagining a tree uprooted and cleaned of soil from its roots. The roots of our cell are called dendrites and they would be located inside the spinal cord. Each rootlet would be connected to the branches of another nerve cell above it. Our motor neuron has a branch coming extremely close to a muscle fiber (a long thin cell that can contract (shorten) and elongate).

Picture one nerve cell with 1000 branches, each branch of the cell innervates a single striated muscle fiber. If you can do this you are imagining a “giant motor unit” which is often alluded to in the polio literature. Giant motor units result after acute polio when muscle fibers lose their connection to a branch because the whole tree (nerve cell) dies. Surviving neurons grow extra branches and connect in the process of recovery from polio damage. This giant motor unit consists of a motor nerve connected by end fibers (branches) to 1000 muscle fibers.

When an EMG is performed the technician can tell if muscle fibers are all part of the same motor unit (if there are giant motor units). When the nerve fires, all the muscle fibers connected to it by its end fibers (branches) also fire synchronously. This will show a characteristic pattern recognizable in the EMG.

Striated muscle fibers are encased in bundles. I’ve tried to draw a representation of a group of striated muscle fibers below, although they are not as uniform in shape as my diagram implies. Some are round, some oval, some angular and so on. Anyway, I thought I could make a couple of interesting points about how polio, and post polio syndrome, affect the muscle fibers using these diagrams.

Figure 1.

On the left we have a cross section of a bundle of normal

muscle fibers. For the sake of understanding polio we’ll

suppose that all of them are innervated by healthy nerve cells

and are part of the same motor unit. In an individual with

Amyotrophic Lateral Sclerosis the entire bundle dies sparing

no fibers because the entire nerve cell dies with all of its

branches (the entire motor unit.)

Figure 2.

To the left we have a diagram of a muscle bundle but notice

that individual fibers (dark centers) are dropping out. It is

significant that the entire bundle, with all fibers, is not dying

but only a few scattered fibers. This is characteristic of post

polio syndrome under the microscope. It suggests the nerve cell

is not dying but rather end fibers are degenerating. It is possible

that the fibers dying here could be picked up and reinnervated

by another nerve, creating an even bigger giant motor unit.

This process is ongoing in patients who have had polio with

giant motor units getting bigger and bigger. When a real big

one reaches the threshold of cellular exhaustion an entire

muscle bundle may die.

At times, under the microscope, muscle biopsies of PPS patients show the loss of entire groups of fibers as happens in ALS. However, in ALS this is the rule while in PPS it is rare. I should add here that the warning against overuse is to spare the muscles and not the neurons. In polio biopsies muscle fibers sometimes appear abnormal with internal nuclei and a ragged shape. These microscopic muscle fiber features are probably due to fewer fibers, because of loss of nerve branches, working harder to maintain function.

Obviously the loss of muscle fibers is the result of stress on the motor nerve (our tree mentioned by analogy earlier). There is the obvious stress of age changes limiting new sprouting and shifting the dynamic compensation mechanism where denervation and reinnervation continue with the advantage tipping toward loss of nerve branches. And we now know that there is a lot of inflammation in the spinal cords of people who had polio. There are infiltrates of lymphocytes (white blood cells which mediate cellular immunity) and also anti-polio antibodies. Poliovirus RNA has even been found in PPS spinal cords. Obviously all of this is stressing the giant motor units we were left with after recovery from polio. These recent microscopic and ultra-microscopically derived facts still leave us with a mystery: is it one cause or many causes which result in our plight. I believe many.

The Lincolnshire Post-Polio Network

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

Created: 25th January 2000

Last modification: 21st January 2010.

Bollenbach Polio Biology 9 Read More »

Muscular Effects in Late Polio

Muscular effects in late polio

STIBRANT SUNNERHAGEN and G. GRIMBY

Department of Rehabilitation Medicine, Göteborg University, Göteborg, Sweden

ABSTRACT

New or increased muscular weakness, fatigue and muscle and joint pain with neuropathic electromyography (EMG) changes in a person with a confirmed history of polio constitute the cardinal symptoms of the post-polio syndrome. Unusual tiredness or fatigue is a common complaint in late polio subjects as is intolerance to cold. Fatigue in polio subjects can have several explanations: emotional fatigue, central nervous system fatigue, ‘general fatigue and/or neuromuscular fatigue. Some studies indicate central fatigue, but it is unclear how often and to which degree there will be a central muscular fatigue. Polio patients are known to be deconditioned (reduced function because of low activity level), and aerobic power is reduced. Defects in the neuromuscular transmission may be present but are not seen in all post-polio subjects with reduction in force and increased fatigability. The fatigue experienced by late polio patients is most likely an augmented peripheral muscle fatigue. Possible explanations may be an imperfection in the sarcoplasmatic reticulum with altered calcium release mechanisms (activation) or in sliding filament function (contractile properties). This may be a secondary effect to the enlarged muscle fibres. However, the prolonged subjective feeling of fatigue reported despite unchanged maximal voluntary contraction (MVC) remains unexplained.

Keywords biopsy, EMG, endurance, fatigue, postpolio, skeletal muscle, spectroscopy.

Received 12 December 2000, accepted 24 January 2001

After the introduction of the immunization programme in the mid-1950s, poliomyelitis was rapidly eradicated in developed nations. Sweden has used the Salk vaccine, which requires inoculation. This vaccine is produced from a mixture of types I, II and III virus strains and is heated to kill the viruses. The Sabine vaccine dominates in the rest of the world and is produced by weakening the living viruses and given perorally, thereby theoret ically rendering immunity in the gut, which is the entry port for the wild virus. Before the start of the vaccin ation programme, many people contracted the disease each year, especially during late summer and early fall. Patients with acute polio typically have an acute biphasical viral illness with fever, headache and gastro intestinal symptoms, followed several days later by the rapid development of paralysis. The paralysis is caused by an invasion of neurotrophic polio virus in motor neurones, which is followed by neuronal damage or death. The progression of paralysis usually stops in less than a week, after which there is a period of stability followed by some degree of gradual recovery of func- tion. A main factor for recovery is the result of sprouting and reinnervation of muscle by the surviving motor neurones.

THE POST-POLIO SYNDROME In survivors of acute paralytic polio, new symptoms occurring decades after the acute illness phase are sometimes referred to as post-polio syndrome (PPS) (Tufvesson 1984). The syndrome encompasses such general complaints as fatigue, cold intolerance, musculo-skeletal manifestations, such as muscle and joint pain, neurological manifestations, such as progressive weakness and atrophy, and sometimes sleep-disordered breathing problems. Criteria for the diagnosis of PPS were outlined first by Halstead & Rossi (1987) and revised by Halstead & Gawne (1996). New or more severe muscular weakness, fatigue and muscle and joint pain with neuropathic electromyog raphy (EMG) changes in a person with a confirmed history of polio constitute the cardinal symptoms of the post-polio syndrome (Halstead & Gawne 1996). Unusual tiredness or fatigue is a common complaint in late polio subjects who report new symptoms many years after the onset of disease (Cosgrove et al. 1987). Cold intolerance is an often reported symptom (Halstead 1998, Gandevia et al. 2000).

Correspondence: Katharina Stibrant Sunnerhagen, Department of Rehabilitation Medicine, Sahlgrenska University Hospital, Guldhedsgatan 19, S-413 45 Göteborg, Sweden.

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Acta Physiol Scand 2001, 171, 335–340

The occurrence of PPS has been found to relate to older age at the onset of disease (Halstead & Rossi 1985), the severity of the original symptoms (Halstead & Rossi 1985, Agre & Rodriguez 1990) and a longer time to reach recovery (Klingman et al. 1988). While a number of aetiologies have been proposed in recent years to explain the pathological changes that cause the symptoms Jubelt & Cashman 1987), the question remains unanswered.

MUSCULO-SKELETAL SYMPTOMS

neurones over a 5-year period, although the methods used for testing muscle strength and EMG differed from the earlier mentioned studies and might explain the different findings.

The motor units are enlarged by reinnervation, and the macro motor units’ potential can be demonstrated to increase by more than seven-fold in relation to normal values for the age in question (Einarsson et al. 1990). Even muscles that are clinically not weak are often affected (Luciano et al. 1996). The consequence of this is that the drop-out of such a large motor unit has a much greater functional impact than that of a motor unit of normal size (Stålberg & Grimby 1995, Grimby. et al. 1996, Grimby et al. 1998). There is evidence of unstable neuromuscular connections in the periphery, as suggested by increased jitter and blocking of nerve transmission, as detected by EMG signals (Wiechers & Hubbell 1981, Einarsson et al. 1990). Electromyography and muscle biopsy evidence of ongoing denervation cannot yet distinguish between stable and unstable polio patients (Cashman et al. 1987, Ravits et al. 1990).

In weak muscles, the fibres adapt with hypertrophy (Grimby & Einarsson 1987). Examination of muscle biopsies from the tibialis anterior in patients with excessive overuse showed that almost all were type I fibres (Borg et al. 1988). Muscle fibre transformations occur in polio patients, which seem to depend on the functional demand on the muscle (Borg et al. 1989). These results were confirmed in another study whose results show that contractile properties of the overused muscle did not change in parallel with fibre type

(Tollbäck et al. 1992).

Musculo-skeletal symptoms are often the reason why persons with late polio re-establish contacts with the health establishment. Symptoms may include fatigue, increase in skeletal distortions or pain in biomechani cally disadvantaged joints. General orthopaedic prob lems are quite common (Agre et al. 1989). About one-third have orthoses that are not functioning properly (Perry & Fleming 1985) or are in need of orthoses to compensate for a weak calf muscle while walking (Perry et al. 1995). The impact on the disability level is mainly on mobility-related activities and different physical demands at work and in the home, as paresis seems to be more common in the lower than in the upper extremities (Cosgrove et al. 1987, Einarsson & Grimby 1990, Grimby & Thorén-Jönsson 1994).

The impairment in patients’ muscles varies from none to severe. Early recovery within a few weeks after the acute phase is probably a result of the revival of affected but not degenerated motor neurons. Recovery within the first year is thought to be accomplished through collateral sprouting, i.e. nerve twigs branching off from surviving motor neurones overlapping the denervated ones. Late compensatory effects are caused by increased size of the remaining innervated muscle fibres (Borg et al. 1988) in weak muscles used in daily activities. As a result of this, even normal muscle strength and presumably normal muscle volume can be present despite an estimated loss exceeding 50% of the number of motor neurones. It seems that there is a relationship between the initial degree of involvement and the effect of various compensatory mechanisms and the subsequent clinical picture. The severity of the original paralysis and the degree of recovery have been reported to predict the risk of new weakening (Klingman et al. 1988). Two follow-up studies by Grimby et al. (1994, 1998) showed evidence of progressive loss of muscle strength in the knee exten sors. A comparative study between Swedish and American postpolio subjects reported similar trends (Agre & Grimby 1995). However, in a population based cohort study in Olmstead county, Minnesota (Windebank et al. 1996), no progress in deterioration was noted in muscle strength owing to loss of motor

FATIGUE Many individuals with late effects of polio complain of fatigue that they describe as ‘increasing physical weak ness’, ‘a sensation of loss of strength during exercise’ and ‘heavy sensations in the muscles’ (Berlly et al. 1991). This common complaint in polio subjects can have several explanations, i.e. emotional fatigue, central nervous system fatigue, “general fatigue and/or neuromuscular fatigue (Basmaijan 1978). There is a chain of command for muscular contraction involving a number of steps from the brain to the spinal cord and the peripheral nerve all the way to the actin-myosin cross bridges within the muscle fibres (Edwards 1983). Briefly, central fatigue may occur as a malfunction of nerve cells or inhibition of voluntary effort.

Muscular fatigue can be defined (Edwards 1981) as a failure to maintain the required or expected force. This fatigue may be either because of central or peripheral performance elements. Electromyography can be used to evaluate fatigue. Alterations of the EMG signal over

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K Stibrant Sunnerhagen and G Grimby · Muscular effects in late polio

time, expressed as Root Mean Square (RMS), will mainly reflect changes in muscle activation, such as

firing rate, and number of active motor units.

Central fatigue is defined as a voluntary or invol- untary failure of neural drive resulting in (a) a reduc tion in the number of the functioning motor units and (b) a reduction in motor unit firing frequency. Reduction of the RMS during maximal voluntary activation may indicate central fatigue. In a study carried out by our group, however, we could not demonstrate a change in the RMS signal at repeated MVC (maximal voluntary contraction) with succes sively increasing ramp levels of isometric contractions (Sunnerhagen et al. 2000). This finding would indicate a lack of central fatigue in this particular set-up, where the required force at each step was in proportion to the individual MVC.

Peripheral fatigue may occur either at the neuro muscular junction and cell membrane (excitation), the calcium release mechanism (activation) or at the sliding filaments (contractile processes). A progressive spectral compression of the EMG signal towards lower frequencies has been suggested to be related to peripheral fatigue during sustained contractions (Lind ström et al. 1970). Thus, muscle fatigue can be studied by means of the mean power frequency (MPF).

Magnetic resonance imaging of the brain has also shown hyperintensive signals in approximately the same areas in survivors who reported fatigue (Bruno et al. 1994). There is no clear-cut evidence that emotional fatigue or depression is more common in polio patients than others (Liechty 1995).

In the literature, it is reported that individuals with late polio use their muscles in many daily activities close to the maximum capacity (Borg et al. 1988, Willén & Grimby 1998, Nollet et al. 1999), indicating a correla tion between muscle overuse and fatigue (Borg et al. 1988, Perry et al. 1988).

Reduced capillarization may be expected, especially in relation to the very large fibre area, but no conclusive results have been reported. Borg & Henriksson (1991) found relatively few capillaries in the tibialis anterior in relation to the large fibre area. A reduction in capillar ization over an 8-year follow-up period has been reported (Grimby et al. 1998), although the values were not significantly different from controls. Also, there were low values of citrate synthetase.

defect in the neuromuscular transmission has been discussed, and Trojan et al. (1993a) reported defects in neuromuscular transmission in patients with post-polio fatigue, which led to trials with acetylcho linesterase inhibitors to reduce the development of fatigue. However, a multi-centre trial showed no significant difference between acetylcholinesterase inhibitor and placebo (Trojan et al. 1999) in measures of quality of life, isometric strength or fatigue (Trojan et al. 1999). In the studies by Stålberg & Grimby (1995) and Grimby et al. (1998), jitter and blocking were reported but not such that could explain the reduction in force to any large extent. Defects in neuromuscular trans mission as a major cause of muscular fatigue could not be suggested on the basis of findings in studies using electrical stimulation of the peroneal nerve (Sunner hagen et al. 2000).

RECOVERY AFTER FATIGUE

GENERALIZED FATIGUE “General fatigue can be caused by cardiorespiratory deconditioning (reduced function because of low activity level). Polio patients are known to be decon ditioned, and aerobic power is shown to be reduced (Owen & Jones 1985, Owen 1991). This has more recently been reconfirmed in both Norway (Stanghelle et al. 1993) and Sweden (Willén et al. 1999). The explanation is probably muscular impairment, which limits the patients’ ability to be active enough to put demands on the cardiorespiratory system. This can theoretically be normalized through exercise aimed at influencing the cardio-respiratory system, for instance with a modified walking programme (Dean & Ross 1988), aerobic exercise (ones et al. 1989, Dean & Ross 1991), endurance training (Ernstoff et al. 1996) or pool exercise with an emphasis on endurance (Willén et al. 2001). For some patients, ventilatory capacity may be a limiting factor leading to fatigue of the respiratory system (Stanghelle et al. 1993, Sinderby et al. 1996).

Fatigue that may be related to changes within the central nervous system has received interest. Post mortem studies have showed the presence of polio

virus lesions in the midbrain reticular formation, posterior hypothalamus, thalamic nuclei, putamen and globus palladus (Bodian 1949, Bruno et al. 1991).

The recovery period after exercise has raised special attention in polio victims both because of the patient history of prolonged fatigue and some experimental results. Agre & Rodriguez (1990) demonstrated a reduced recovery rate of MVC after sustained submaximal isometric contractions until fatigue. They also demonstrated that performance increased when sufficient rest periods were given between the contraction periods (Agre & Rodriguez 1991). An interesting but unexplained finding is that the percep tion of fatigue remains for days in polio victims after a fatiguing exercise but without reduction in MVC (Agre et al. 1998). One possible explanation is low frequency fatigue, which is characterized by a substantial

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Acta Physiol Scand 2001, 171, 335-340

unchanged MVC (Agre et al. 1998) may be because of low frequency fatigue.

REFERENCES

reduction of tension at low frequencies of stimulation (Edwards et al. 1977). A feature of this low frequency fatigue is that it can take more than 1 day for full recovery and this may contribute considerably to the feeling of weakness in muscle groups after intense exercise. From studies of isolated single fibres of mouse skeletal muscle, we know that tetanic calcium is reduced in low frequency fatigue (Westerblad et al. 1993), which is probably caused by a reduction in Ca2+ release from the sarcoplasmatic reticulum.

In our own studies using a protocol with increasing intermittent work (Sunnerhagen et al. 2000, H. Ljungberg, K.S. Sunnerhagen, B. Uikhoff-Baaz et al., unpublished), no differences were found in the meta bolic changes in the tibial anterior muscles as shown by MR phosphorous spectroscopy between a group of polio patients with a moderate reduction in the tibialis anterior volume and strength (median 58% of control values) and healthy controls of similar ages. Differences between the polio and the control groups could be noted during the recovery phase, and the recovery half time for Pi (inorganic phosphate) was significantly longer for the patients than for the healthy control group (Ljungberg et al. unpublished). In general, the polio group showed a larger individual variation in the results from the MR spectroscopy both with respect to metabolite concentrations and pH values after exercise, which may be explained by the heterogeneity of fibre type proportions and muscle enzyme levels in the polio patients.

Agre, J. & Grimby, G. 1995. A comparison of symptoms

between Swedish and American post-polio individuals and assessment of lower limb strength – a four year cohort

study. Scand J Rehab Med 27, 183-192. Agre, J. & Rodriguez, A. 1990. Neuromuscular function: a

comparison of symptomatic and asymptomaticasymptotic polio subjects to control subjects. Arch Phys Med Rehabil 71,

545–551, Agre, J. & Rodriguez, A. 1991. Intermittent isometric activity:

its effect on muscle fatigue in post-polio subjects. Arch Phys

Med Rehabil 72, 971-975. Agre, J.C., Rodriquez, A.A. & Franke, T.M. 1998. Subjective

recovery time after exhausting muscular activity in post polio and control subjects. Am J Phys Med Rehabil 77,

140–144. Agre, J.C., Rodriquez, A.A. & Sperling, K.B. 1989. Symptoms

and clinical impressions of patients seen in a post-polio

clinic. Arch Phys Med Rehabil 70, 367–370. Basmaijan, T. 1978. Muscular tone, fatigue and neural

influences. In: J. Basmaijan (ed.) Muscles Alive: Their Function Revealed by Electromyography, 4th edn, pp. 79–114. Williams &

Wilkins, Baltimore. Berlly, M.H., Strauser, W.W. & Hall, K.M. 1991. Fatigue in

post-polio syndrome, Arch Phys Med Rehabil 72, 115–118, Bodian, D. 1949. Histopathological basis of clinical findings

in poliomyelitis. Am ) Med 6, 563–578. Borg, K., Borg, J., Dhoot, G., Edström, L., Grimby, L. &

Thornell, L. 1989. Motorneuron firing and isiomyosin type of muscle fibres in prior polio. J Neurol Neurosurg Psychiatry

52, 1141–1148 Borg, K. & Henriksson, J. 1991. Prior poliomyelitis-reduced

capillary supply and metabolic enzyme content in hypertrophic slow-twitch (type 1) muscle fibres. J Neurol

Neurosurg Psychiatry 54, 236-240. Bruno, R.L., Cohen, J.M., Galski, T. & Frick, N.M. 1994. The

neuroanatomy of post-polio fatigue. Arch Phys Med Rehabil

75, 498–504. Bruno, R.L., Frick, N.M. & Cohen, J. 1991. Polioencephalitis,

stress, and the etiology of post-polio sequelae. Orthopedics

14, 1269-1276. Cashman, N.R., Maselli, R., Wollmann, R.L., Roos, R., Simon, R.

& Antel, J.P. 1987. Late denervation in patients with

antecedent paralytic poliomyelitis. N Engl J Med 317, 7-12. Cosgrove, J.L., Alexander, M.A., Kitts, E.L., Swan, B.E.,

Klein, M.J. & Bauer, R.E. 1987. Late effects of

poliomyelitis. Arch Phys Med Rehabil 68, 47. Dean, E. & Ross, J. 1988. Modified aerobic walking program:

effect on patients with postpolio syndrome symptoms. Arch

Phys Med Rehabil 69, 1033–1038. Dean, E. & Ross, J. 1991. Effect of modified aerobic training

on movement energetics in polio survivors. Orthopedics 14,

1243–1246. Edwards, R. 1981. Human muscle function and fatigue:

physiological mechanisms. In: R. Porter & J. Whelan (eds).

CONCLUSIONS Fatigue is a complex phenomenon or many complex phenomena. Part of the difficulty in identifying fatigue is the lack of a clear definition of the word itself. The relative workload is different for different muscles in everyday activities, and thus limiting weakness in one leg is often described as fatigue. Some studies indicate central fatigue in patients with the post-polio syndrome (Bodian 1949, Bruno et al. 1994), but it is unclear how often and to what degree there will be central muscular fatigue. Defects in neuromuscular transmission might be present in post-polio patients (Trojan et al. 1993b) but will probably not explain the reduction in force that is present and the development of fatigue to any major degree (Stålberg & Grimby 1995, 1998). There is most likely a development of augmented peripheral muscle fatigue in polio patients. Possible explanations may be an imperfection in the sarcoplasmic reticulum with altered calcium release mechanisms (activation) or in the sliding filaments (contractile properties). This might be a secondary effect to the enlarged muscle fibres. The prolonged subjective feeling of fatigue reported despite

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K Stibrant Sunnerhagen and G Grimby · Muscular effects in late polio

Ciba Foundation Symposium, Vol. 82. pp. 1–18. Pitman

Medical, London. Edwards, R. 1983. Biochemical basis of fatigue. In:

Knuttgen (ed.) Biochemistry of Exercise, pp. 3–28. Human

Kinetics, Champaign, IL. Edwards, R.H., Hill, D.K., Jones, D.A. & Merton, P.A. 1977,

Fatigue of long duration in human skeletal muscle after

exercise. J Physiol (Lond) 272, 769–778. Einarsson, G. & Grimby, G. 1990. Disability and handicap in

late poliomyelitis. Scand J Rehabil Med 22, 113–121. Einarsson, G., Grimby, G. & Stålberg, E. 1990.

Electromyographic and morphological functional compensation in late poliomyelitis. Muscle Nerve 13,

165-171. Ernstoff, B., Wetterquist, H., Kvist, H. & Grimby, G. 1996.

Endurance training effect on individuals with post

poliomyelitis. Arch Phys Med Rehabil 77, 843–848. Gandevia, S.C., Allen, G.M. & Middleton, J. 2000. Post-polio

syndrome: assessments, pathophysiology and progression.

Disabil Rehabil 22, 38–42.. Grimby, G. & Einarsson, G. 1987 Muscle morphology with

special reference to muscle strength in post-polio subjects. In: Research and Clinical Aspects of the Late Effects of Poliomyelitis, Vol. 23, pp. 265–274. March of Dimes Birth

Defects Foundation, White Plains, NY. Grimby, G., Hedberg, M. & Henning, G.B. 1994. Changes in

muscle morphology, strength and enzymes in a 4-5-year follow-up of subjects with poliomyelitis sequelae. Scand J

Rehabil Med 26, 121–130. Grimby, G., Kvist, H. & Grangård, U. 1996. Reduction in

thigh muscle cross-sectional area and strength in a 4 year follow-up in late polio. Arch Phys Med Rehabil 77, 1044

Grimby, G., Stålberg, E., Sandberg, A. & Sunnerhagen, K.
An eight year longitudinal study of muscle strength, muscle fiber size and dynamic electromyogram in

individuals with late polio. Muscle Nerve 21, 1428-1437. Grimby, G. & Thorén-Jönsson, A. 1994. Disability in

poliomyelitis sequelae. Phys Ther 74, 415-421. Halstead, L. & Gawne, A.C. 1996, NRH proposal for limb

classification and exercise prescription. Disabil Rehabil 18,

311-316. Halstead, L. & Rossi, C. 1987. Research and Clinical Aspects of the

Late Effects of Poliomyelitis. March of Dimes Birth Defects

Foundation, White Plains, NY. Halstead, L.S. 1998. Post-polio syndrome. Sci Am 278, 42-47. Halstead, L.S. & Rossi, C.D. 1985. New problems in old polio

patients: results of a survey of 539 polio survivors.

Orthopedics 8, 845–850. Jones, D.R., Speier, J., Canine, K., Owen, R. & Stull, G.A.

Cardiorespiratory responses to aerobic training by patients with postpoliomyelitis sequelae. Jama 261,

3255–3258. Jubelt, B. & Cashman, N.R. 1987. Neurological

manifestations of the post-polio syndrome. Crit Rev

Neurobiol 3, 199-220. Klingman, J., Chui, H., Corgiat, M. & Perry, J. 1988.

Functional recovery. A major risk factor for the

development of post-poliomyelitis muscular atrophy. Arch

Neurol 45, 645–647. Liechty, J. 1995. Psychosocial issues and post-polio: a

literature review of the past thirteen years. In: G. Halstead, L.a.G. (ed.) Post-Polio Syndrome, pp. 177-198. Hanley &

Belfus, Inc, Philadelphia. Lindström, L., Magnusson, R. & Petersén, I. 1970. Muscular

fatigue and action potential conduction velocity changes studied with frequency analysis of the EMG signals.

Electromyography 4, 341-353. Luciano, C.A., Sivakumar, K., Spector, S.A. & Dalakas, M.C.

Electrophysiologic and histologic studies in clinically unaffected muscles of patients with prior paralytic

poliomyelitis. Muscle Nerve 19, 1413-1420, Nollet, F., Beelen, A., Prins, M.H. et al. 1999. Disability and

functional assessment in former polio patients with and without post-polio syndrome. Arch Phys Med Rehabil 80,

136–143. Owen, R.R. 1991. Post-polio syndrome and cardiopulmonary

conditioning. West J Med 154, 557–558. Owen, R.R. & Jones, D. 1985. Polio residuals clinic:

conditioning exercise program. Orthopedics 8, 882–883. Perry, J., Barnes, G. & Gronley, J.K. 1988. The post-polio

syndrome. An overuse phenomenon. Clin Orthop 233,

145-62. Perry, J. & Fleming, C. 1985. Polio: long-term problems.

Orthopedics 8, 877–881. Perry, J., Fontaine, J.D. & Mulroy, S. 1995. Findings in post

poliomyelitis syndrome. Weakness of muscles of the calf as a source of late pain and fatigue of muscles of the thigh

after poliomyelitis. J Bone Joint Surg Am 77, 1148–1153. Ravits, J., Hallett, M., Baker, M., Nilsson, J. & Dalakas, M.

Clinical and electromyographic studies of post

poliomyelitis muscular atrophy. Muscle Nerve 13, 667–674. Sinderby, C., Weinberg, J., Sullivan, L., Borg, J., Lindstrom, L.

& Grassino, A. 1996. Diaphragm function in patients with cervical cord injury or prior poliomyelitis infection. Spinal

Cord 34, 204–213. Stanghelle, J.K., Festvag, L. & Aksnes, A.K. 1993. Pulmonary

function and symptom-limited exercise stress testing in subjects with late sequelae of poliomyelitis. Scand J Rehabil

Med 25, 125-129. Stålberg, E. & Grimby, G. 1995. Dynamic electromyography

and biopsy changes in a 4 year follow up: study of patients

with a history of polio. Muscle Nerve 18, 699–707. Sunnerhagen, K.S., Carlsson, U., Sandberg, A., Stalberg, E.,

Hedberg, M. & Grimby, G. 2000. Electrophysiologic evaluation of muscle fatigue development and recovery in

late polio. Arch Phys Med Rehabil 81, 770–776. Tollbäck, A., Knutsson, E. & Borg, J. 1992. Torque-velocity

relation and muscle fibre characteristics of foot dorsiflexors after long-term overuse of residual muscle fibres due to prior polio or L5 root lesion. Scand J Rehab Med 24,

151-156. Trojan, D., Gendron, D. & Cashman, N. 1993a.

Antichloinesterase-responsive neuromuscular junction transmission defects in post-poliomyelitis fatigue. J Nerol Science 114, 170-177.

339

Muscular effects in late polio · K Stibrant Sunnerhagen and G Grimby

Acta Physiol Scand 2001, 171, 335-340

Trojan, D., Gendron, D. & Cashman, N. 1993b. Stimulation

frequency-dependant neuromuscular junction transmisson defects in patients with prior poliomyelitis. J Nerol Science

118, 150–157. Trojan, D.A., Collet, J.P., Shapiro, S. et al. 1999. A multicenter, randomized, double-blinded trial of pyridostigmine in post-polio syndrome (see comments).

Neurology 53, 1225–1233. Tufvesson, A. 1984. Polioskadade, finns dom? SVCR: Bromma. Westerblad, H., Duty, S. & Allen, D.G. 1993. Intracellular

calcium concentration during low-frequency fatigue in isolated single fibers of mouse skeletal muscle. J Appl

Physiol 75, 382–388. Wiechers, D.O. & Hubbell, S.L. 1981. Late changes in the

motor unit after acute poliomyelitis. Muscle Nerve 4, 524–528.

Willén, C., Cider, A. & Sunnerhagen, K.S. 1999, Physical

performance in individuals with late effects of polio. Scand

J Rehabil Med 31, 244–249. Willén, C. & Grimby, G. 1998. Pain, physical activity, and

disability in individuals with late effects of polio. Arch Phys

Med Rehabil 79, 915_919. Willén, C., Sunnerhagen, K.S. & Grimby, G. 2001. Dynamic

water exercise in individuals with late poliomyelitis. Arch

Phys Med Rehabil 82, 66-72. Windebank, A., Litchy, W., Daube, J. & Iverson, R. 1996.

Lack of progression of neurologic deficit in survivors of paralytic polio: a 5-year prospective population-based study. Neurology 46, 80–84.

340

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PSN Leaflet

PSN Leaflet Read More »

Oxygen is NOT for Hypoventilation in Neuromuscular Disease

Oxygen is NOT for Hypoventilation in Neuromuscular Disease E.A. Oppenheimer, MD, FCCP

Editor’s Note: The IVUN office continually hears from people with post-polio or other neuromuscular diseases and conditions being inappropriately prescribed O2 therapy. This anecdotal evidence (confirmed by the Mayo Clinic case series) supports the need for accurate information from the physicians most expert in the pulmonary aspects of neuromuscular disease to be disseminated more widely to alert people to the reasons why they should be wary of O2 therapy.

If progressive respiratory failure occurs in people with neuromuscular disease, an abnormal nocturnal oximetry study is often an early indication that hypoventilation is occurring. There are significant periods of decreased oxygen levels in the blood or hypoxemia during sleep when lying flat, in addition to decreases in vital capacity (VC), maximum inspiratory force (MIF), and maximum expiratory force (MEF). Decreased oxygen saturation (SaO2) combined with increasing carbon dioxide (CO2) retention or hypercapnia are the hallmarks of hypoventilation. This is sometimes called ventilatory pump failure, due to the weakened respiratory muscles.

Patients with neuromuscular diseases who are developing progressive respiratory failure due to respiratory muscle weakness will die unless mechanical ventilation is used. The rate of progression is often hard to predict. Some patients seem suddenly to experience life-threatening hypercapnic respiratory failure. They may not have been aware of gradually increasing symptoms and signs, particularly since they are often not physically active and are often not being regularly monitored with simple pulmonary function tests.

Administering oxygen does not provide assistance to the weakening respiratory muscles, but gives both the patient and the doctor the false impression that appropriate treatment is being provided. While in fact hypoventilation is mistaken for an oxygen transfer problem. Indeed, administering oxygen can mask the problem. Also there is a danger of causing respiratory depression by giving oxygen. Oxygen is NOT the treatment for hypoventilation. It will improve the SaO2, but not the hypoventilation and may increase the danger of dying of sudden respiratory failure.

In hypercapnic respiratory failure due to hypoventilation, the SaO2 falls due to the rise of the CO2. The alveoli in the lungs (tiny gas exchange units) should clear most of the CO2 out with each breath. Instead, with hypoventilation, CO2 accumulates and thus there is decreased room in the alveoli for oxygen. When mechanical ventilation using room air is provided, it lowers the CO2 in the alveoli, corrects the SaO2, and rests the respiratory muscles. The ventilator should be adjusted to

achieve a normal SaO2, on room air. If oxygen is being administered, one cannot use noninvasive oximetry to tell whether enough assisted ventilation is being provided; repeated arterial blood gas specimens (ABGs) would be needed.

When there is respiratory failure in neuromuscular patients (ALS, post-polio, SMA, muscular dystrophy, etc.) who have no additional pulmonary disease that impairs oxygen transfer, the ventilator set-up is adjusted to:

be comfortable for the patient;

achieve SaO2 of 95% or higher on room air (this can be measured with a

finger-sensor oximeter);

assist the patient to effectively cough and clear secretions;
provide improved oral communication (if vocal communication is possible).

It has been common for people using noninvasive nasal ventilation (NPPV) with a bi-level positive pressure unit to use inadequate settings; frequently, they are not monitored with clinical evaluation and oximetry. The EPAP is often set too high usually it should not be higher than 3-4 cm H2O; the IPAP is set too low – usually it needs to be 12-16 cm H20 and adjusted to achieve an oxygen saturation of 95% or higher.

Some situations may require administering oxygen, such as pneumonia due to infection or aspiration. If this occurs in patients with respiratory muscle weakness and hypoventilation, then it is important to provide both assisted ventilation and supple-mental oxygen, and use ABGs to monitor them.

Address: E.A. Oppenheimer, MD, FCCP, Pulmonary Medicine (eaopp@ucla.edu).

References

Bach, J.R. (1999). Guide to the evaluation and management of neuromuscular disease. Philadelphia, PA: Hanley & Belfus. Gay, P.C., & Edmonds, L.C. (1995). Severe hypercapnia after low-flow oxygen therapy in patients with neuromuscular disease and diaphragmatic dysfunction. Mayo Clinic Proceedings, 70(4), 327-330. Hsu, A., & Staats, B. (1998). “Postpolio” sequelae and sleep-related disordered breathing. Mayo Clinic Proceedings, 73, 216-224. Krachman, S., & Criner, G.J. (1998). Hypoventilation syndromes. Clinics in Chest Medicine, 19(1), 139-155.

Additional Observations about Oxygen in Neuromuscular Disease

Anita Simonds, MD, FRCP, Royal Brompton Hospital, London, England (a.simonds@rbh.nthames.nhs.uk)

I agree completely with Dr. Oppenheimer that assisted ventilation is the appropriate therapy for alveolar hypoventilation. Apart from a limited number of situations such as pneumonia or lung fibrosis, oxygen therapy is usually inappropriate and may prove hazardous. Clearly, in an acute pneumonia O2 therapy can be entrained into the ventilator system. Fortunately, in the United Kingdom, this message is getting across to healthcare workers and patients. There is still some inequity in providing noninvasive ventilation, but the situation is improving.

Lisa S. Krivickas, MD, Instructor in PM&R, Harvard Medical School, Director of EMG, Spaulding Rehabilitation Hospital (L Krivickas@compuserve.com)

The analogy that I often use in regard to patients with respiratory failure from neuromuscular disease is that their lungs are like a deflated balloon that they are not strong enough to inflate. To inflate the balloon, mechanical assistance to force air into the balloon is needed. Blowing oxygen across the

of the balloon (the equivalent of using supplementary oxygen delivered by nasal cannula) will do nothing to inflate the balloon.

The case series published by the Mayo Clinic (see reference to Gay & Edmonds, 1995) demonstrates the dangers of administering as little as 1 to 2 L/min of nasal cannula oxygen. Patients with a variety of neuromuscular disorders experienced marked CO2 retention; several became obtunded and required intubation or died when they were placed on 0.5 to 2 L of nasal cannula oxygen.

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Dr Henry writes about You Were There

Dr. Henry writes about “You Were There”

Do you remember the popular TV show of the 1960s entitled “You Are There” narrated by Walter Cronkite? The show would typically allow the viewer to be an eyewitness to a significant time in history. Let me take you back to March 1954. Summer Polio epidemics had been spreading fear and terror across America and much of the world for several decades. In 1952, only two years previous, a record 60,000 cases of polio had been reported in the USA. Another summer was approaching, but finally, as reported in the news, there was hope for a successful vaccine. Much of the hope ironically depended on monkeys.

The Rhesus monkey (weight 4 to 8 lb.) was in great demand. A world monkey hunt was underway. In northern India, Moslem trappers went about catching live monkeys. Hindu believers would not participate in this hunt because of religious convictions. Once captured, the monkeys would be carried in bamboo cages on shoulder poles to the nearest railway station and from there to New Delhi. After some health screening, 1000 monkeys would be flown 4000 miles to London. From London, these monkeys would fly the 3000 miles to New York’s Idlewild Airport. From New York, these same monkeys would travel by trucks to Okatie Farms, South Carolina. Monkeys from the Philippines were also brought to Okatie Farms. An average of 5000 monkeys per month passed through Okatie Farms. Even so, the demand for monkeys exceeded the supply. After more health screening these monkeys were dispersed to four laboratories in Toronto, Pittsburgh, Detroit, and Berkeley. What happened to these monkeys? If you love monkeys, skip the next paragraph.

The monkeys were anesthetized and their kidneys were removed. The monkeys were then sacrificed with medication. The kidneys were sliced up, placed in a viable solution and rocked gently in an incubator for about six days. This process promoted monkey kidney cell growth.

One cubic centimeter of a fluid containing live polio virus was introduced into each quart of kidney cell tissue fluid. This solution was gently rocked for four days in which time the polio virus multiplied a thousand fold. Now the polio virus was ready for harvest. The resultant virus laden solution was chilled and carefully placed in 2.5 gallon containers and shipped to pharmaceutical laboratories for vaccine production.

Five American pharmaceutical companies agreed to produce the vaccine on a non profit basis. Those companies were Parke, Davis and Co. in Detroit, Pitman-Moore and Eli Lilly and Co. in Indianapolis, Wyeth Inc. in Philadelphia, and Cutter Laboratory in Berkeley California. When these companies received the 2.5 gallon containers of live virus, the solution was filtered to eliminate the kidney cells and combined into 12.5 gallon lots stored in steel tanks. A diluted formaldehyde solution was added to the tanks to kill the polio virus and the killed virus vaccine was the final product. Before the resultant vaccine was injected into humans, the vaccine was injected into monkeys, guinea pigs, rabbits, and mice to determine safety in mammals. All three strains of killed polio virus were contained in the vaccine. Each vaccine injection was only one cubic centimeter.

A major breakthrough in the quest to find an effective vaccine occurred in 1949 when Harvard virologist Dr. John F. Enders reported in Science journal that the polio virus could be cultured in non nervous tissue (monkey kidney cells). As a result, a great demand for monkey kidney cells occurred. This was the impetus that the determined Dr. Jonas Salk needed.

Salk was born in Manhattan in 1914. He graduated from a public high school at the age of 16 and finished the College of the City of New York at age 19. He graduated from NYU Medical School, did his internship in Manhattan and immediately entered the field of medical research. He initially worked at the University of Michigan Medical Center and came to the University of Pittsburgh in 1947. It was at Pittsburgh that Salk directed his energies in his deliberate, organized style to develop a vaccine to prevent polio.

Also, in 1949, Dr. David Bodian and Dr. Howard Howe at John Hopkins Medical Center in Baltimore had concluded that all known strains of polio virus belonged to three types. Thus, any vaccine developed would have to grant protection for all three types of polio.

By March of 1954, Salk had already vaccinated 5000 first, second, and third graders in Pittsburgh. Eighty to ninety-five per cent of parents had consented to the experimental vaccine despite reservations expressed by health officials. Results indicated that a polio antibody response had occurred in these children.

Statistical predictions in 1954 indicated that among one million children, 700 would contract polio, 483 would recover without paralysis, 175 would have some permanent paralysis, and 42 would die. In 1954, twenty-two per cent of polio victims were over age twenty. Under age twenty, more boys were infected more than girls, but over age twenty, more women were infected than men.

Countless parents and polio caretakers now had some real hope that victory over polio was possible. Eighty-one million of the three billion dimes donated to the March of Dimes had been appropriated to Salk and his colleagues for his promising research. This large financial investment seemed on the brink of a major positive return.

Here was the plan to determine if the vaccine was really effective: Dr. Thomas Francis at the University of Michigan was selected by the National Foundation for Infantile Paralysis to do a large scale double blind study on the Salk vaccine. In a one month period, 500,000 to 1,000,000 first, second, and third graders would receive the Salk vaccine in 200 chosen test areas, beginning in the warmer Southern USA and moving to the North. One half of these children would be given the Salk vaccine and one half would be given a placebo injection. From ten per cent of the children involved, blood samples would be taken to determine what percentage already had a natural immunity for polio. One year later, the double blind would be broken and the verdict on the new vaccine would be known. The National Foundation was taking a major gamble on this study. What would the results be in one year? You were there. You know what happened on April 12, 1955.

Forty-five years have passed since March 1954. The natural live polio virus has been greatly reduced around the world. Thanks be to God for the courage and determination of the human spirit.

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

December 1999.

Reference: Time Magazine, March 29, 1954, “Polio Fighter Salk, Is This the Year?” pages 56 – 66. (25 cents)

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: 28th November 1999

Last modification: 24th January 2010.

Dr Henry writes about You Were There Read More »

Bollenbach Polio Biology 8

Polio Biology VIII

Post-Polio Pathogenesis

Eddie Bollenbach

A Lincolnshire Post-Polio Library Publication

27th November 1999

There are words we use to describe aspects of pathology, which means the study of disease. Often, when we look for a clue to unlock a mystery like Post-Polio Syndrome we neglect the words invented by epidemiologists and get confused about what we are truly seeking. Let me give you an example of what I’m getting at. Everyone with the debilitating and uncomfortable fatigue of Post-Polio Syndrome eventually asks the question: “what is causing this?” When they find out they have PPS they may obsess about the cause. When you talk to people they will tell you PPS is caused by the overwork of already over-burdened muscles. Or some may say it is caused by aging of a damaged neuromuscular system (this really doesn’t tell us much — muscles or nerves? — how is the aging manifesting itself to cause [that word again] damage)? Some are beginning to say that PPS is caused by latent viral particles inside cells of the central nervous system.

Ever since the writings of Copernicus we, in the Western Hemisphere, have used reductionism in our thinking to the extent that we think problems must always have a single cause. So we say this, not that. And we seek until we find a single simple explanation. Nothing but a single understandable cause, it seems, will relieve the nagging uncertainty about what is going on inside our bodies. I don’t think things are this simple, especially inside the nervous system.

Let’s get back to the words that epidemiologists use to clarify aspects of disease. First, there is the etiology of a disease. Etiology means cause. When we are asking what is the cause we are really asking for the etiology. The etiology could be very simple: “People who overuse muscles enervated by giant motor units (one nerve cell with multiple fibers) will over tax those nerves and the nerves will die”. This is the most common idea for an etiology of PPS right now. Another epidemiologist’s word, which is a bit more complex in its meaning, is pathogenesis. The pathogenesis of a disease is the mechanism, or steps which occur in the disease manifesting itself. This can be very complicated.

We have found, recently, that motor nerve cells infected with polio virus do not die the way we thought they did. We thought that if a neuron was infected with polio virus the virus multiplied inside the cell and lysed it. The infected cells were always killed in this lytic model. This is one reason why many medical doctors think that once an individual recovers from acute polio it is clear sailing for life. Now we know differently. Many infected cells did die because the cell itself, not the virus, proceeded through a series of programmed steps toward its own death and disintegration. This programmed cell death occurs after certain cell structures have been damaged and appears to be an evolutionary adaptation that removes cells

which have no chance of normal function. The process is called apoptosis. Many polio infected cells underwent apoptosis and not lysis. The significance of this is that many cells that were infected did not apoptose and consequently trapped viral particles inside. Pathogenic organisms that live inside cells have a neat adaptation. They evade the immune system by being safely tucked away where immune processes cannot remediate. Add to this the blood brain barrier and you have an ideally insulated hideaway. The blood brain barrier consists of extremely tight junctions between capillary cells nourishing the brain and spinal cord. Because of it many drugs can’t even get into the brain and this has been a problem in medical intervention for tumors and other disorders of the central nervous system. But I digress.

There are other pathogens which elude immune attack by living inside cells. The tubercule bacillus is one of them. Now also, with polio, we have detected unquestionable signatures of the virus in a little more than half of those studied who have PPS but we find no virus in people who have had paralytic polio without late effects.[1] What are the role of these polio viruses in PPS? Are they etiological or are they a part of the pathogenesis of this disease? I think right now there is a consensus building that they may be a part of the pathogenesis of Post-Polio Syndrome.

What results in their release? It is likely that cells are suddenly undergoing apoptosis many years after acute polio. Why? Overuse? Immune attack? Age? Each answer leads to a new question. And it seems we’ve arrived where we started, looking for causes. One thing is for sure: we will find many steps in the pathogenesis of PPS along the way toward understanding an etiology.

References.

J Neurol 1999 Jun;246(6):472-6, Postpolio syndrome: poliovirus persistence is involved in the pathogenesis., Julien J, Leparc-Goffart I, Lina B, Fuchs F, Foray S, Janatova I, Aymard M, Kopecka H. [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

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.

Created: 27th November 1999

Last modification: 21st January 2010.

Bollenbach Polio Biology 8 Read More »

Bollenbach Polio Biology 7

Polio Biology VII

Holistic Polio

Eddie Bollenbach

A Lincolnshire Post-Polio Library Publication

17th October 1999

On reflection, it is quite obvious that everything in the body works together. Put another way: nothing can happen inside the body without an effect on all parts of the system. After reading some recent articles on nerve and muscle function I started thinking about Post-Polio Syndrome and how closely a skeletal muscle fiber and attached nerve work together. The relationship is so close that it is quite impossible, at times, to tell what the primary cause of neuromuscular weakness is. This is particularly true when we are focusing on the synapse, the gap between nerve and muscle through which stimulant chemicals like acetyl-choline act. Without a nerve connection a muscle is an orphan and incompetent. Without a muscle a motor-nerve is useless. It is commonly understood that in Post-Polio Syndrome the end fibers of an axon (long fiber which is part of a nerve cell) break down leaving a small but effectively insulated moat between muscle and nerve. Since each nerve end fiber innervates only one muscle fiber the muscle cell is then cut from stimulation. Without stimulation we know that the receptors of stimulation on the surface of the muscle fiber are forever lost.[1]

I think most of us assume that the loss of end fibers in PPS is due to overuse. And we have a tendency to extrapolate to say that if this could only be remedied we would defeat our foe. However, during the increased neuromuscular load of exercise the earliest response of the neuromuscular system is to allow for increased efficiency of nerve stimulation. A later response is the production of muscle protein and the consequent hypertrophic growth of muscle fibers. Also, even in normal neuromuscular systems there is only one end fiber per muscle fiber. During synaptogenesis several fibers may innervate one muscle but eventually all lose touch but one. This evolutionary competition for muscle fibers by nerves is believed to result in efficient and varied innervation of muscle because different kinds of fibers have different effects on the contraction of muscle.[2] In normals there would be a tendency toward a diversity of muscle tension strength along the length of a muscle which would be produced by the natural competition of early nerve type synapses. Polio obviously alters this process by killing neurons. This allows for secondary synaptogenetic events.

What happened to skeletal muscle fiber receptors while they were left without stimulation for months during acute polio? We know now that they break down. People hoping for cures to spinal injury must exercise their muscles with electrical stimulation to keep muscle cell receptors alive. The old maxim “Use em or lose em” applies here. We with polio are in a position of slow loss of both end fibers and muscle receptors. How can we say that polio is a nerve disease when it is also, obviously, a muscle disease? And, by the way, I’m not convinced that the “cause” of PPS is overuse. There could be a host of other reasons

why these end fibers of nerves, and/or muscle receptors, lose function. When analyzed there may be more than one cause. With some of the advances appearing now in measuring and differentiating antibodies, along with similar advances in DNA and RNA cloning techniques, which have been used to identify polio virus fragments in PPS, it may not be long until we have more definitive answers. Until then we should understand that we are a system and that whatever proximately caused our PPS, reversing secondary and tertiary problems will likely prove very difficult, because we are whole.

References.

Akaaboune, M., Culican, S.M., Turney, S.G. et al. 1999. Rapid and reversible effects of activity on acetylcholine receptor density at the neuromuscular junction in vivo. Science 286(5439):503-507.) [PubMed Abstract]
Introduction To Muscle Physiology and Design, University of California at San Diego, 1999. http://muscle.ucsd.edu/musintro/jump.shtml

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 October 1999.

Last modification: 21st January 2010.

Bollenbach Polio Biology 7 Read More »

Dr Henry writes about PPS and The Toddler Mystique

Dr. Henry writes about PPS and The Toddler Mystique

Two years ago, a survey was done on our support group. Among the 61 PPSers that responded, 24 or nearly 40% had acute polio at age five or younger. The next larger number was 18 or 30% in the age group 6 to 10. Other age groups were ages 11 to 15 with 18%, ages 16 to 20 with 8%, and ages 21 and older with 5%. Most of the age 5 and younger group would naturally have few memories of their acute polio experience. Further analysis of this youngest group reveals the following distribution of ages of acute polio.

Birth to age 1 — 10 cases

Age 1 to age 3 — 9 cases

Age 3 to age 5 — 5 cases

This data reveals that almost one-third of the entire group had acute polio under age 3. Retrievable memory of a stressful event under age three would be most unlikely, although not impossible. You may recall that I wrote an article entitled Recovered Memories on this topic last year.

Several years ago at one of our support group’s regular monthly meetings, we divided into three groups based on our age when we had acute polio. One group was age five and under, one was age 6 to 18, and one was over 18. We discussed our memories of the acute polio experience. The two older groups were

most serious in their discussion and recall of memories. The younger group had a joyous, somewhat noisy, and upbeat discussion. Thereafter, this subgroup of PPSers has been identified as “Toddlers.” As a result of having acute polio at such a young age, were the Toddlers less stressed by the acute polio event than older children and adults who have many vivid memories of their acute polio experience? Most of the Toddlers admit that they have very few memories of acute polio. Some do recall the smell of wool hot packs, the diagnostic spinal tap, and the feeling of separation from their parents when in isolation. Some have stories of their early polio rehabilitation, but some of these stories are often stories that they have heard from their parents.

If residual damage resulted from acute polio, the older children would have memories of being whole in body. For example, I had polio at age eleven. I have many wonderful memories of running in races and playing various sports. I remember simple things like bounding up steps two at a time. I treasure these memories. Now, which is the better situation? The toddlers have few if any memories of being physically whole. The older children and adults have many memories of being physically whole. Are the toddlers more “freed up” and less encumbered by stressful polio memories? Are they better defended and thus, simply able to have more fun? Are they more extroverted and engaging? Are the non-toddlers more serious and more introspective? I do not believe these questions have categorical answers, but a number of speculations might arise.

The group of polio children over age five may have experienced more of a conscious experience of loss.

They remember being whole and being normal. Thus, they knew the difference between before and after polio. Their loss and grief was conscious. The less than five age group (toddlers) started their conscious life as after polio and had few if any memories of being any different. Perhaps, they did not experience a feeling of loss or grief and were emotionally spared this stress.

Psychologist Erik Erikson is known for his formulation of the Stages of the Life Cycle. The stages in the first five years are:

Basic trust versus basic mistrust, age birth to 1 year;
Autonomy versus shame and doubt, age 1 to 3;
Initiative versus guilt, ages 3 to 5.

Would a stressful and damaging disease such as polio have a negative impact on a child’s developing personality during these five years? During the first year of life, consistent parenting is most important in developing basic trust. If polio resulted in a prolonged separation from parents, would the polio stricken infant feel less trust, less hope, and less confidence?

From age 1 to 3, would the polio child feel more self conscious and even shame at being different or being treated different by parents and other caretakers?

From age 3 to 5, would the polio child develop less initiative due to failure to compete equally with able bodied peer children, resulting in some feeling of guilt?

Among the Polio Toddlers that I have met, most have demonstrated healthy trust, a determined autonomy, and an outgoing initiative. I am not sure that any generalizations can be applied to polio children. If there is one, it is the resolve to persevere and never give up. Hope, initiative, independence, and courage are other shared characteristics.

It is my opinion that polio children with an absence of able-bodied memories and those with able-bodied memories have evolved from their experiences toward healthy character development. Nevertheless, the joy, optimism, good humor, initiative, exuded confidence, and pure pleasure of being among our group’s Toddlers may possibly only be explained as the Toddler Mystique. My Webster’s defines “Mystique” as “an air or attitude of mystery and reverence developing around something or someone” and “the special

esoteric skill essential in a calling or activity.” That definition does fit our Toddlers. I wonder if other Post-Polio Support Groups have noticed a similar Mystique among their Toddlers?

As I asked previously, which is the better situation; to have memories of pre polio bodily wholeness or not? My conclusion is that there is no definitive answer. To me, each polio child has made every effort to live life to the fullest. The necessary psychological adjustments made as a result of polio probably helped in experiencing life to the fullest.

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

October 1999

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

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

Created: 3rd October 1999

Last modification: 24th January 2010.

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