Tuesday, March 16, 2004
This is a very lengthly post but well worth the read. This is a paper that was written by the next generation of Occupational Therapists. Joseph has a very good working knowledge of GBS and how it effects the patients life. I give him credit for actually talking with several patients in preparation for writting this paper. I wish more medical professionals would get more involved in learning not only the physical side of the disease, but how the disease effects the patient emotionaly.
Guillain-Barré Syndrome
March 5, 2004
Joseph Hall
Etiology
Guillain-Barré Syndrome is also known as Acute Idiopathic Polyneuritis (On-Line Medical Dictionary), and Acute Inflammatory Polyneuropathy (Cecil). As its names suggest, is a demyelinating disease of the nerves, "characterized by progressive muscle weakness and areflexia, with spontaneous remission being the rule." (UpToDate) Approximately one person in one hundred thousand is diagnosed with GBS each year. While no single catalyst for the syndrome has been identified, more often than not its onset follows an infection of the gastrointestinal or respiratory tract. Campylobacter jejuni has been incriminated as having the strongest correlation; around one quarter of those diagnosed with GBS undergo a bout with the bacteria twelve weeks or less before diagnosis. Other events suspected of causing Guillain-Barré include vaccinations, epidural anesthesia, thrombolytic agents (clot dissolving drugs), and even the consumption of undercooked poultry. In some cases, there is no apparent provocation whatsoever. (UpToDate)
The syndrome causes an individual's immune system turns on the body, attacking the peripheral nervous system, and in particular, the myelin sheath which covers most neuronal axons. This layer of myelin is integral in allowing nerve impulses to travel almost instantaneously from one point in the body to the spinal cord, and from there to the brain. When the Schwann cells which make up the myelin sheath are damaged and destroyed, many of the body's sensory cues never even reach the spinal cord, resulting in numbness, while other messages become corrupted or garbled, leading to feelings of tingling or burning at the slightest touch (or even no contact at all). Conversely, the loss of motor signals traveling from the brain to the legs, arms, trunk, and face results in muscle weakness and even paralysis. Because the nerves running to and from the arms and legs are the longest, they also have the greatest potential for disruption. For this reason, symptoms often first appear in the hands and feet. (NIH Pamphlet)
These manifestations can develop extremely quickly, sometimes in a matter of hours, other times over the course of three or four weeks. The severity of the attack is also quite varied, and seemingly random in nature. In general, and things always seem to be "in general" with Guillain-Barré, the paradoxical mixture of extreme pain and numbness makes its way inward, up the arms and legs and into the trunk. Usually this total redefinition of sensory perception is accompanied by motor issues which range from muscle weakness to total quadriparesis. The neck and face are not spared, either: paresthesia, or abnormal neurological sensation, is not uncommon, while loss of motor control is prevalent. About one tenth of those diagnosed with GBS will even lose respiratory function, due to a loss of innervation to the diaphragm and intercostal muscles, requiring immediate, emergency placement upon mechanical ventilation. (Cecil)
Diagnosis and Prognosis
A definite diagnosis of Guillain-Barré Syndrome can be difficult to obtain, especially in its early stages. For example, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is very similar in symptoms and results. "CIDP is a chronic condition and is only distinguished from GBS by virtue of its pattern of progression. In GBS the low point is reached within four weeks whereas in CIDP the initial progressive phase lasts longer, usually much longer. Some CIDP patients are initially diagnosed as having GBS. Only when the deterioration continues over an extended period, or when one or more relapse(s) occur after a period of improvement, is the illness reclassified as CIDP." (Evers and Hughes)
Guillain-Barré is fatal for about five percent of those it strikes. For the rest, recuperation is often only understood on a person to person basis. According to the statistics, fifty percent of patients recover fully within six months, and half of those remaining reach their previous level of health after another six months. "Full recovery," however, can mean one thing on a medical chart, and something entirely different in a person's everyday life. At any rate, there are numerous GBS survivors who struggle every day with the symptoms which first beset them years before. Many of these individuals will face these difficulties every day for the rest of their lives. Adding to this the sudden and severe loss of independence, one begins to realize that the psychological and emotional damage may linger on at least as long as the physical signs. (Cecil)
Manifestations and Treatment
Males are struck with Guillain-Barré slightly more often than are females, but the difference is negligible. All ages are susceptible, though young adults (in their twenties) and older adults (over seventy) seem to be the most vulnerable. Increasing age also correlates to a more detrimental experience, as recovery time and level of return are, on average, lower in later adulthood. (Stratton, Howe, and Johnston, p. 38)
Medical treatment of GBS is centered in two areas: plasmapharesis and immunoglobulin therapy. With both, the process should be initiated as soon as the syndrome is diagnosed (Broomfield). Plasmapheresis is a complicated process in which the blood is filtered and the plasma is removed. The medical field is not certain as to why this works: only that it does seem to lessen the effects of the syndrome. High-dose immunoglobulin therapy involves the introduction of donated immune system proteins. "Investigators have found that giving high doses of these immunoglobulins, derived from a pool of thousands of normal donors, to Guillain-Barré patients can lessen the immune attack on the nervous system. Investigators do not know why or how this works." (NIH Pamphlet)
Therapeutic Teams and Goals
After the GBSer, as some who have experienced the malady refer to themselves, has stabilized, therapy can begin in an acute setting. Because GBS affects the nerves, muscle damage is not commonly a problem. However, muscle wasting is virtually unavoidable. At this point, the therapy team generally consists of an occupational therapist and a physical therapist, who assist through range of motion on the upper and lower body, respectively, and perform massages to reduce edema, if necessary. (Stratton, Howe, and Johnston, p. 37)
Most GBS survivors, when sufficiently recuperated, are transferred to an inpatient rehabilitation hospital setting. Here they work with a team of professionals which includes occupational, physical, speech, and respiratory therapists, as well as the nursing staff, a primary care physician (PCP), and a neurologist. (Health for Life)
Treatment goals are, as often as possible, oriented towards returning the GBS survivor to his or her previous way of living. One area which receives particular focus is activities of daily living. In most cases, the goal of returning to one's previous level of functioning is not unrealistic. Therefore, the utilization of adaptive equipment or environmental modifications is generally considered temporary (Trombly, p. 903).
Personal Accounts
A recovering Guillain-Barré survivor faces numerous challenges. Rather than sharing what researchers and medical professionals believe them to be, I would like illustrate these adversities by sharing the true stories of three people who have personally experienced the syndrome. All names have been changed.
Jeff
Jeff was 57 when he was diagnosed with GBS, shortly before the millennium. He says, "The doctors told/knew nothing. I thought I was going to die. My neuro[logist] waited over a week watching my nerves being destroyed before ordering IVIg [intravenous immunogammaglobulins]. She did not know to order it as soon as GBS is diagnosed, as I now know...I lay in bed in excruciating pain for 8 months. I could not do anything. One needs to rest, rest, and rest some more... My spouse had to do everything for me at the start; now she still has to do more than half the housework."
Even though four years have passed since Jeff's immune system inflicted its damage upon his nerves, he still only considers himself to be at about two thirds of his previous capacity: "I can't work. This last year I can finally do light to moderate work around my home in the mornings, the rest of the day is recovery time from the fatigue."
In terms of problem solving, he suggests thinking everything out in advance in order to make a minimum of moves. Being forced to reduce his goals to his newly lowered (but very slowly increasing) capacities has made him a believer in energy management. He says that many people, including some therapists, do not understand that 'no pain, no gain' is not always true: "Nerves heal at their own rates, and to stress them slows their recovery. So with GBS, any exertion that one cannot recover completely from in 12 to 18 hours is too much and must be reduced. This becomes a problem when even breathing, sitting, etc is over exertion. Then you just gotta roll with the punches."
Summer
On the morning of November 21, 2002, Summer awoke to find that her hands and feet were numb, "I was already ill from pneumonia... I could not feel my hands touching myself or anything else. My husband did not hesitate and took me into the hospital: that was at 7 am. I went through countless tests, everything you can think of.......while they were running each test, my body was slowly shutting down....by 5 pm a neurologist came in and conducted his own tests...luckily he had experienced GBS with another patient before & knew the signs and what to look for.....I was admitted right away, later that night I lost my legs, the next morning [found me with] total paralysis & being rushed to the vent. [The] doctors were preparing my husband for the worst; they did not believe I would make it off the vent alive because it had hit me so hard and fast. I was 33 years old."
As far as a cause is concerned, there is no clear answer. "Eight weeks prior to my onset I had started working at a daycare center... and the first year you work there or a school or doctors office or medical facility you catch everything. Well, the second week into my new job I had strep throat, the fourth week, a sinus infection, the sixth week, the flu, the eighth week was pneumonia and GBS. My doctor seems to think it was related to the sinus infection."
Summer believes that reaching her current level has been a huge victory. "GBS beat me up that is for sure but I did not allow it to beat me down. At times it was not easy, I thought maybe I would never be able to walk or enjoy the things I like to do again, but after getting past the critical state, I realized I was given another chance at life......and that was enough for me, I kept my mind and spirit positive even through the rough times!"
After more than a month in acute care, she was admitted to a rehab hospital on January 1, 2003. "When I came home January 21 I was still paralyzed; I could move my limbs a little, but I was still totally dependent on my husband.... Once I was home, I feel I healed so much faster. By February 15 I was walking short distances with my walker and with braces on my knees (I have hyper-extensive knees). A week later, I took the braces off....Forever! To look at me today, you would never know I was so sick and fighting for my life 15 months ago. I can walk mostly without assistance unless I have overdone activities for the day, then I tend to walk awkward and need some assistance. I can do most of the things I want to within reason. I have painful residuals, my upper feet are still very numb as well as my finger tips. I experience sharp shooting/stabbing pains and numbness throughout. I stumble from time to time (usually due to fatigue): I fatigue easily. I refuse to think I will have this for the rest of my life, but if I did continue the way I am currently, how could I complain when I have come from so far?!? Where I am currently with this is "SO" much better than where I was in the beginning. I am alive, can walk and do most of the things I love to do again. I have learned how to live with the residuals I do have and pace myself accordingly."
Speaking of her low-points, Summer says, "Not being able to move was the worst...I felt so useless. I could not take care of myself or my husband or children... Jim was/is so wonderful throughout all of this, he did things most husbands would run to a nurse to do...I could not have gotten through this without his constant love, support and strength, [and that of] my children too... Not only is the outside of your body paralyzed, but the inside as well. I had pneumonia and could not feel my diaphragm so that I could make myself cough, nor could I feel my stomach when it was upset or ready to get sick; I could not feel myself breathe. Another great thing [I now enjoy] is to be able to toss and turn and position myself comfortably in bed. For the most part I had great nurses but there were those who would come in and say 'I only come in once every hour (sometimes longer) to turn you.' That was bad! Try it... try to position yourself in bed and see how long you can hold that position. Pretend like you cannot move one muscle.....not one. That was truly one of the worst times."
Those times of hardship have made Summer relish the 'small things' in life: "One of the greatest things is to be able to feel myself when I have to go to the bathroom, and be able to take care of all that myself: to shower myself and brush my own teeth and hair... As I got better, I made a point to do the things I used to do (folding clothes, setting the dinner table, typing on the computer, brushing my hair/teeth, etc) as part of my therapy, oh it was frustrating at times but I did not give up.... I just cannot get back to work again, the fatigue & pain is just too much. If I overdo things, I get physically ill and tired and this will put me out for 2-3 days, so I stick to a schedule now and try to avoid that."
Karen
Karen's encounter with GBS began on June 22, 2000, just 6 days before her 48th birthday. Less than a week before it struck, she did not feel well, having an achy, upset stomach. Then it passed. This is her only clue as to what could have triggered what was to follow. "My paralysis started in a non-GBS way (i.e. my trunk), with difficulty breathing, then spreading from right to left, not ascending.... Just the fact that my paralysis began oddly could make me atypical GBS... [then] I suffered a blood clot during one of my last Plasmapheresis treatments. I had 15, to slow/stop the GBS progression. And, yes, I was on Coumadin, Heparin shots, aspirin, wearing anti-thrombosis stockings (T.E.D.S.), and a vinyl ‘stocking’ to my groin that would massage my legs to simulate leg movement. My blood clot was in my spine, between [vertebra] C3-4. The clot happened so quietly, it was not suspected for about a week... When it was finally diagnosed, through an MRI, it was already dissolving, so to speak. And my recovery from GBS paralysis went from snails pace to close to non-existent. The left side of my body was about 50% out of paralysis when the clot hit. My right side was just starting to ‘wake up.’"
Nearly four years into her experience with Guillain-Barré Syndrome, Karen does not consider herself as being 'back to normal' in any way. She grades her sensory perception at about 40% of its previous capacity, ("This is hard to explain as I ‘feel’ things differently now than I did before or you do."), her motor skills at around 30%, and her endurance at approximately 30% as well. Her expectations are for a positive change, but she says it has been a very slow process thus far.
"I am no longer married. My husband could not ‘handle’ the responsibility of life with me in a wheelchair. Some spouses just cannot do this. My mother has taken over my care, and this is where my current frustration is. I am trying to find a way to ease the mental/physical pressure of all this on my mom. It is a slow process to find out which category of help I fall under for the state of Iowa, but I know there has to be something. To put me in a nursing home would cost more to the state than to find a program for me to fit into. I am not ready for a nursing home. And if there is one thing that I have learned through all this, there is always a way to do something. Always. Make that my mantra: 'THERE IS ALWAYS A WAY TO DO EVERYTHING.'"
Thoughts and Reflections
From communicating with Jeff, Karen, and Summer, I have come to a better understanding of what GBS is like on a personal level, though I am quite sure that no one can adequately understand it without living through it. In response to a question concerning favorite activities abandoned because of the condition, Karen began listing them for me: Job. Sex. Cuddling. Sitting on the couch. Walking her dog. Her home. Independence. Finally, she simply said, "Just...my whole life." I heard similar responses from Jeff and Summer. Jeff even told me plainly, "I am [now] much less satisfied with life." These and other words broke my heart at times, and made me feel sad, helpless, and a little ashamed of the things that I complain about.
I shared these thoughts with Karen and she gently chastised me: "GBS is the luck of the draw, my friend. When your card comes up, it is your turn to play. Your life is what you are going through and mine is what I am going through. I take my life as what it is. And I now live it to the fullest." Crediting Dr. Phil McGraw, she thinks of her encounter as a 'defining moment:' "It gives you to a new awareness of life. It lets you know just how precious life is. That each day should be lived to the fullest doing what it is you were truly meant to do. Never compromise on your life. Do not 'settle'. People tell me that they could not handle all this like I have. I tell them that 'yes, you will; you are stronger than you think you are...really!' ... I just wish there were more hours or my GBS fatigue wasn't with me."
Summer echoed an almost identical sentiment, "Before GBS I was constantly running, never slowing down to enjoy the beauty of life that is out there and around us everyday... [Now] I just take everything one day at a time and enjoy every day that I have been given again...spending more time together with my husband & children...enjoying the simple things of life, stopping and taking in everything around us that most of us are guilty of passing by because of being caught up in our busy everyday lives. Life is short, I was lucky to have been given a second chance...and I am going to live it to my fullest everyday.
Karen told me, "When you have GBS, your body has betrayed you." Throughout the process of these interviews, it was abundantly clear to me that my body has not done so. I struggled with asking total strangers to describe and analyze their suffering for me. However, my feelings on these interviews are now clear to me. I will not feel guilty for my health, for being pain-free, when others cannot even remember what life was like without constant hurt. Guilt is for times when I do the wrong thing, or when I do not do the good that I ought to do. Being healthy when others are not is no cause for remorse. Standing idly by while others suffer is cause enough for me.
Role of the Occupational Therapist
I believe that occupational therapy, when offered in a competent and professional manner, by someone who has taken the time to understand Guillain-Barré Syndrome, is one way to make a significant difference. "Referral to occupational therapy is common when the course of GBS is moderate to severe... [the occupational therapist should] assess communication, control of the environment as appropriate, comfort, and level of anxiety." (Trombly 903)
At the intensive care stage, in addition to initial, upper body range of motion exercises, the occupational therapist may assist by intervening in other ways:
· Developing communication tools, such as [a] sign or picture board.
· Adapting TV and lights to remote control
· Modifying the telephone for hands-free use
· Modifying positions for lying and sitting to those optimal for function and comfort
· Teaching about GBS and recommending other support services
· Teaching strategies to reduce anxiety." (Trombly 903)
When the individual is moved to a rehabilitation hospital, occupational therapy interventions shift in focus to eliciting controlled and coordinated movement, especially of the arms and hands. This can be achieved through tasks which require trunk balance, sitting or standing exercise (with light weights, ball and balloon games, etc) and supervised or assisted activities of daily living. An example of how these sessions can be graded is seen in the range of difficulty between sitting upright in a chair with a back, to completing upper body dressing with no support, in a sitting position (Wingert). In other words, "occupational therapy professionals should be involved early in the rehabilitation program to promote upper body strengthening, range of motion, and activities to promote functional self-care." (Scott, et al.)
The more intangible responsibilities of the occupational therapist in a post-acute setting are threefold. First, to instruct: concerning mobility, conservation of energy, work simplification, and community access. Next, to train: in transfers, modified self-care techniques, and the use of aids and adaptive equipment. And finally, to collaborate in order to adapt routines, habits, equipment, and strategies for daily living. (Trombly 903)
Barriers, Supports and Resources
Standing in the way of a return to one's previous life is a body that has betrayed you. This factor seems to be the biggest hurdle to recovery. Secondary to this is the general societal attitude that if someone looks okay, s/he is okay. Karen addressed this issue, "Looks are deceiving... [People forget about] the residuals…fatigue and neuropathies- such as burning legs- caused by axonal damage... As far as I am concerned, there are no ‘mild’ cases of GBS. It does a number on your life."
Because of this, she believes that all GBS survivors should consider seeing a psychologist, as well as taking low dose anti-depressants, while they consider all the questions which need answering. Another blessing is to be found in the involvement of family and friends, even if it is something as simple as a get-well card. New friends, in a similar situation, are often found in GBS support groups. Notably, among those I interviewed, is Guillain-Barré Syndrome Foundation International (www.gbsfi.com), which features a discussion forum and chat rooms, for questions and support. Another website that is by, about, and for GBS survivors is found at www.aboutgbs.com.
Adaptive equipment is usually helpful. Karen considers the following to be noteworthy: "a motorized wheelchair, [raised] commode, bath bench, lift chair, sliding board, as well as a ‘ladder’ to help pull me up in bed at night."
A "New" Intervention
The more I learn about Guillain-Barré, the more I realize that the only true experts on the subject are those who have experienced its effects first-hand. My intent for this paper is not to dwell upon that fact, or chide occupational therapists for a lack of expertise. Neither is it my intention to imply that GBS is the only condition which results in dramatically different symptoms and problem areas from one survivor to the next. My purpose, first and foremost, has been to increase education and awareness about Guillain-Barré. Second to that, my hope is that this essay will serve as a gentle reminder, an intervention, perhaps, to therapists and other students that GBS survivors know their own limitations better than anyone. 'No pain, no gain' is not always true, especially for one who has experienced Guillain-Barré Syndrome, and has an intimate understanding of pain.
References
On-Line Medical Dictionary, Retrieved on February 25, 2004, from
http://cancerweb.ncl.ac.uk/omd/
Acute Inflammatory Polyneuropathy (Guillain-Barré Syndrome). (2004.)
Retrieved February 11, 2004, from Cecil Textbook of Medicine, University
of Virginia School of Medicine Internet Database.
DeLisser, H., (2004.) Guillain-Barré syndrome. Retrieved February 11, 2004,
from UpToDate, University of Virginia School of Medicine Internet
Database.
Guillain-Barré Syndrome. (1997.) Prepared by Office of Communications and
Public Liaison, National Institute of Neurological Disorders and Stroke,
National Institutes of Health. Bethesda, MD: U.S. Dept. of Health and
Human Services, Public Health Service, National Institutes of Health.
Evers, E., Hughes, R., (June, 1998.) Guillain-Barré Syndrome Support Group.
Retrieved February 28, 2004, from www.gbs.org.uk/cidp.html
Stratton, K., Howe, C., Johnston Jr., R. (Eds.) (1994) Adverse events associated
with childhood vaccines [computer file]: evidence bearing on causality.
Washington, DC: National Academy Press.
Broomfield, J., (2003.) Guillain-Barré syndrome. Retrieved on February 28, 2004,
from http://health.discovery.com/diseasesandcond/encyclopedia/666.html.
Health for Life: Guillain Barré Syndrome. (1995.) The Ohio State University
Medical Center: Department of Critical Care Nursing. Retrieved on
February 28, 2004, from http://devweb3.vip.ohio-
state.edu/Materials/PDFDocs/dis-cond/general/guillian.pdf
Trombly, C., Radomski, M., (Eds.) (2002.) Occupational Therapy for Physical
Dysfunction. Baltimore: Lippincott Williams & Wilkins.
Wingert, K., (2003.) The Virtual Health Care Team: Guillain-Barré. Retrieved on
March 1, 2004, from www.vhct.org/case799/team_goals_3.shtml.
Scott, D., Talavera, F., Andary, M., Allen, K., and Lorenzo, C., (Eds.) (2004.)
Guillain-Barré Syndrome. Retrieved on March 1, 2004, from
http://www.emedicine.com/PMR/topic48.htm
Guillain-Barré Syndrome
March 5, 2004
Joseph Hall
Etiology
Guillain-Barré Syndrome is also known as Acute Idiopathic Polyneuritis (On-Line Medical Dictionary), and Acute Inflammatory Polyneuropathy (Cecil). As its names suggest, is a demyelinating disease of the nerves, "characterized by progressive muscle weakness and areflexia, with spontaneous remission being the rule." (UpToDate) Approximately one person in one hundred thousand is diagnosed with GBS each year. While no single catalyst for the syndrome has been identified, more often than not its onset follows an infection of the gastrointestinal or respiratory tract. Campylobacter jejuni has been incriminated as having the strongest correlation; around one quarter of those diagnosed with GBS undergo a bout with the bacteria twelve weeks or less before diagnosis. Other events suspected of causing Guillain-Barré include vaccinations, epidural anesthesia, thrombolytic agents (clot dissolving drugs), and even the consumption of undercooked poultry. In some cases, there is no apparent provocation whatsoever. (UpToDate)
The syndrome causes an individual's immune system turns on the body, attacking the peripheral nervous system, and in particular, the myelin sheath which covers most neuronal axons. This layer of myelin is integral in allowing nerve impulses to travel almost instantaneously from one point in the body to the spinal cord, and from there to the brain. When the Schwann cells which make up the myelin sheath are damaged and destroyed, many of the body's sensory cues never even reach the spinal cord, resulting in numbness, while other messages become corrupted or garbled, leading to feelings of tingling or burning at the slightest touch (or even no contact at all). Conversely, the loss of motor signals traveling from the brain to the legs, arms, trunk, and face results in muscle weakness and even paralysis. Because the nerves running to and from the arms and legs are the longest, they also have the greatest potential for disruption. For this reason, symptoms often first appear in the hands and feet. (NIH Pamphlet)
These manifestations can develop extremely quickly, sometimes in a matter of hours, other times over the course of three or four weeks. The severity of the attack is also quite varied, and seemingly random in nature. In general, and things always seem to be "in general" with Guillain-Barré, the paradoxical mixture of extreme pain and numbness makes its way inward, up the arms and legs and into the trunk. Usually this total redefinition of sensory perception is accompanied by motor issues which range from muscle weakness to total quadriparesis. The neck and face are not spared, either: paresthesia, or abnormal neurological sensation, is not uncommon, while loss of motor control is prevalent. About one tenth of those diagnosed with GBS will even lose respiratory function, due to a loss of innervation to the diaphragm and intercostal muscles, requiring immediate, emergency placement upon mechanical ventilation. (Cecil)
Diagnosis and Prognosis
A definite diagnosis of Guillain-Barré Syndrome can be difficult to obtain, especially in its early stages. For example, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is very similar in symptoms and results. "CIDP is a chronic condition and is only distinguished from GBS by virtue of its pattern of progression. In GBS the low point is reached within four weeks whereas in CIDP the initial progressive phase lasts longer, usually much longer. Some CIDP patients are initially diagnosed as having GBS. Only when the deterioration continues over an extended period, or when one or more relapse(s) occur after a period of improvement, is the illness reclassified as CIDP." (Evers and Hughes)
Guillain-Barré is fatal for about five percent of those it strikes. For the rest, recuperation is often only understood on a person to person basis. According to the statistics, fifty percent of patients recover fully within six months, and half of those remaining reach their previous level of health after another six months. "Full recovery," however, can mean one thing on a medical chart, and something entirely different in a person's everyday life. At any rate, there are numerous GBS survivors who struggle every day with the symptoms which first beset them years before. Many of these individuals will face these difficulties every day for the rest of their lives. Adding to this the sudden and severe loss of independence, one begins to realize that the psychological and emotional damage may linger on at least as long as the physical signs. (Cecil)
Manifestations and Treatment
Males are struck with Guillain-Barré slightly more often than are females, but the difference is negligible. All ages are susceptible, though young adults (in their twenties) and older adults (over seventy) seem to be the most vulnerable. Increasing age also correlates to a more detrimental experience, as recovery time and level of return are, on average, lower in later adulthood. (Stratton, Howe, and Johnston, p. 38)
Medical treatment of GBS is centered in two areas: plasmapharesis and immunoglobulin therapy. With both, the process should be initiated as soon as the syndrome is diagnosed (Broomfield). Plasmapheresis is a complicated process in which the blood is filtered and the plasma is removed. The medical field is not certain as to why this works: only that it does seem to lessen the effects of the syndrome. High-dose immunoglobulin therapy involves the introduction of donated immune system proteins. "Investigators have found that giving high doses of these immunoglobulins, derived from a pool of thousands of normal donors, to Guillain-Barré patients can lessen the immune attack on the nervous system. Investigators do not know why or how this works." (NIH Pamphlet)
Therapeutic Teams and Goals
After the GBSer, as some who have experienced the malady refer to themselves, has stabilized, therapy can begin in an acute setting. Because GBS affects the nerves, muscle damage is not commonly a problem. However, muscle wasting is virtually unavoidable. At this point, the therapy team generally consists of an occupational therapist and a physical therapist, who assist through range of motion on the upper and lower body, respectively, and perform massages to reduce edema, if necessary. (Stratton, Howe, and Johnston, p. 37)
Most GBS survivors, when sufficiently recuperated, are transferred to an inpatient rehabilitation hospital setting. Here they work with a team of professionals which includes occupational, physical, speech, and respiratory therapists, as well as the nursing staff, a primary care physician (PCP), and a neurologist. (Health for Life)
Treatment goals are, as often as possible, oriented towards returning the GBS survivor to his or her previous way of living. One area which receives particular focus is activities of daily living. In most cases, the goal of returning to one's previous level of functioning is not unrealistic. Therefore, the utilization of adaptive equipment or environmental modifications is generally considered temporary (Trombly, p. 903).
Personal Accounts
A recovering Guillain-Barré survivor faces numerous challenges. Rather than sharing what researchers and medical professionals believe them to be, I would like illustrate these adversities by sharing the true stories of three people who have personally experienced the syndrome. All names have been changed.
Jeff
Jeff was 57 when he was diagnosed with GBS, shortly before the millennium. He says, "The doctors told/knew nothing. I thought I was going to die. My neuro[logist] waited over a week watching my nerves being destroyed before ordering IVIg [intravenous immunogammaglobulins]. She did not know to order it as soon as GBS is diagnosed, as I now know...I lay in bed in excruciating pain for 8 months. I could not do anything. One needs to rest, rest, and rest some more... My spouse had to do everything for me at the start; now she still has to do more than half the housework."
Even though four years have passed since Jeff's immune system inflicted its damage upon his nerves, he still only considers himself to be at about two thirds of his previous capacity: "I can't work. This last year I can finally do light to moderate work around my home in the mornings, the rest of the day is recovery time from the fatigue."
In terms of problem solving, he suggests thinking everything out in advance in order to make a minimum of moves. Being forced to reduce his goals to his newly lowered (but very slowly increasing) capacities has made him a believer in energy management. He says that many people, including some therapists, do not understand that 'no pain, no gain' is not always true: "Nerves heal at their own rates, and to stress them slows their recovery. So with GBS, any exertion that one cannot recover completely from in 12 to 18 hours is too much and must be reduced. This becomes a problem when even breathing, sitting, etc is over exertion. Then you just gotta roll with the punches."
Summer
On the morning of November 21, 2002, Summer awoke to find that her hands and feet were numb, "I was already ill from pneumonia... I could not feel my hands touching myself or anything else. My husband did not hesitate and took me into the hospital: that was at 7 am. I went through countless tests, everything you can think of.......while they were running each test, my body was slowly shutting down....by 5 pm a neurologist came in and conducted his own tests...luckily he had experienced GBS with another patient before & knew the signs and what to look for.....I was admitted right away, later that night I lost my legs, the next morning [found me with] total paralysis & being rushed to the vent. [The] doctors were preparing my husband for the worst; they did not believe I would make it off the vent alive because it had hit me so hard and fast. I was 33 years old."
As far as a cause is concerned, there is no clear answer. "Eight weeks prior to my onset I had started working at a daycare center... and the first year you work there or a school or doctors office or medical facility you catch everything. Well, the second week into my new job I had strep throat, the fourth week, a sinus infection, the sixth week, the flu, the eighth week was pneumonia and GBS. My doctor seems to think it was related to the sinus infection."
Summer believes that reaching her current level has been a huge victory. "GBS beat me up that is for sure but I did not allow it to beat me down. At times it was not easy, I thought maybe I would never be able to walk or enjoy the things I like to do again, but after getting past the critical state, I realized I was given another chance at life......and that was enough for me, I kept my mind and spirit positive even through the rough times!"
After more than a month in acute care, she was admitted to a rehab hospital on January 1, 2003. "When I came home January 21 I was still paralyzed; I could move my limbs a little, but I was still totally dependent on my husband.... Once I was home, I feel I healed so much faster. By February 15 I was walking short distances with my walker and with braces on my knees (I have hyper-extensive knees). A week later, I took the braces off....Forever! To look at me today, you would never know I was so sick and fighting for my life 15 months ago. I can walk mostly without assistance unless I have overdone activities for the day, then I tend to walk awkward and need some assistance. I can do most of the things I want to within reason. I have painful residuals, my upper feet are still very numb as well as my finger tips. I experience sharp shooting/stabbing pains and numbness throughout. I stumble from time to time (usually due to fatigue): I fatigue easily. I refuse to think I will have this for the rest of my life, but if I did continue the way I am currently, how could I complain when I have come from so far?!? Where I am currently with this is "SO" much better than where I was in the beginning. I am alive, can walk and do most of the things I love to do again. I have learned how to live with the residuals I do have and pace myself accordingly."
Speaking of her low-points, Summer says, "Not being able to move was the worst...I felt so useless. I could not take care of myself or my husband or children... Jim was/is so wonderful throughout all of this, he did things most husbands would run to a nurse to do...I could not have gotten through this without his constant love, support and strength, [and that of] my children too... Not only is the outside of your body paralyzed, but the inside as well. I had pneumonia and could not feel my diaphragm so that I could make myself cough, nor could I feel my stomach when it was upset or ready to get sick; I could not feel myself breathe. Another great thing [I now enjoy] is to be able to toss and turn and position myself comfortably in bed. For the most part I had great nurses but there were those who would come in and say 'I only come in once every hour (sometimes longer) to turn you.' That was bad! Try it... try to position yourself in bed and see how long you can hold that position. Pretend like you cannot move one muscle.....not one. That was truly one of the worst times."
Those times of hardship have made Summer relish the 'small things' in life: "One of the greatest things is to be able to feel myself when I have to go to the bathroom, and be able to take care of all that myself: to shower myself and brush my own teeth and hair... As I got better, I made a point to do the things I used to do (folding clothes, setting the dinner table, typing on the computer, brushing my hair/teeth, etc) as part of my therapy, oh it was frustrating at times but I did not give up.... I just cannot get back to work again, the fatigue & pain is just too much. If I overdo things, I get physically ill and tired and this will put me out for 2-3 days, so I stick to a schedule now and try to avoid that."
Karen
Karen's encounter with GBS began on June 22, 2000, just 6 days before her 48th birthday. Less than a week before it struck, she did not feel well, having an achy, upset stomach. Then it passed. This is her only clue as to what could have triggered what was to follow. "My paralysis started in a non-GBS way (i.e. my trunk), with difficulty breathing, then spreading from right to left, not ascending.... Just the fact that my paralysis began oddly could make me atypical GBS... [then] I suffered a blood clot during one of my last Plasmapheresis treatments. I had 15, to slow/stop the GBS progression. And, yes, I was on Coumadin, Heparin shots, aspirin, wearing anti-thrombosis stockings (T.E.D.S.), and a vinyl ‘stocking’ to my groin that would massage my legs to simulate leg movement. My blood clot was in my spine, between [vertebra] C3-4. The clot happened so quietly, it was not suspected for about a week... When it was finally diagnosed, through an MRI, it was already dissolving, so to speak. And my recovery from GBS paralysis went from snails pace to close to non-existent. The left side of my body was about 50% out of paralysis when the clot hit. My right side was just starting to ‘wake up.’"
Nearly four years into her experience with Guillain-Barré Syndrome, Karen does not consider herself as being 'back to normal' in any way. She grades her sensory perception at about 40% of its previous capacity, ("This is hard to explain as I ‘feel’ things differently now than I did before or you do."), her motor skills at around 30%, and her endurance at approximately 30% as well. Her expectations are for a positive change, but she says it has been a very slow process thus far.
"I am no longer married. My husband could not ‘handle’ the responsibility of life with me in a wheelchair. Some spouses just cannot do this. My mother has taken over my care, and this is where my current frustration is. I am trying to find a way to ease the mental/physical pressure of all this on my mom. It is a slow process to find out which category of help I fall under for the state of Iowa, but I know there has to be something. To put me in a nursing home would cost more to the state than to find a program for me to fit into. I am not ready for a nursing home. And if there is one thing that I have learned through all this, there is always a way to do something. Always. Make that my mantra: 'THERE IS ALWAYS A WAY TO DO EVERYTHING.'"
Thoughts and Reflections
From communicating with Jeff, Karen, and Summer, I have come to a better understanding of what GBS is like on a personal level, though I am quite sure that no one can adequately understand it without living through it. In response to a question concerning favorite activities abandoned because of the condition, Karen began listing them for me: Job. Sex. Cuddling. Sitting on the couch. Walking her dog. Her home. Independence. Finally, she simply said, "Just...my whole life." I heard similar responses from Jeff and Summer. Jeff even told me plainly, "I am [now] much less satisfied with life." These and other words broke my heart at times, and made me feel sad, helpless, and a little ashamed of the things that I complain about.
I shared these thoughts with Karen and she gently chastised me: "GBS is the luck of the draw, my friend. When your card comes up, it is your turn to play. Your life is what you are going through and mine is what I am going through. I take my life as what it is. And I now live it to the fullest." Crediting Dr. Phil McGraw, she thinks of her encounter as a 'defining moment:' "It gives you to a new awareness of life. It lets you know just how precious life is. That each day should be lived to the fullest doing what it is you were truly meant to do. Never compromise on your life. Do not 'settle'. People tell me that they could not handle all this like I have. I tell them that 'yes, you will; you are stronger than you think you are...really!' ... I just wish there were more hours or my GBS fatigue wasn't with me."
Summer echoed an almost identical sentiment, "Before GBS I was constantly running, never slowing down to enjoy the beauty of life that is out there and around us everyday... [Now] I just take everything one day at a time and enjoy every day that I have been given again...spending more time together with my husband & children...enjoying the simple things of life, stopping and taking in everything around us that most of us are guilty of passing by because of being caught up in our busy everyday lives. Life is short, I was lucky to have been given a second chance...and I am going to live it to my fullest everyday.
Karen told me, "When you have GBS, your body has betrayed you." Throughout the process of these interviews, it was abundantly clear to me that my body has not done so. I struggled with asking total strangers to describe and analyze their suffering for me. However, my feelings on these interviews are now clear to me. I will not feel guilty for my health, for being pain-free, when others cannot even remember what life was like without constant hurt. Guilt is for times when I do the wrong thing, or when I do not do the good that I ought to do. Being healthy when others are not is no cause for remorse. Standing idly by while others suffer is cause enough for me.
Role of the Occupational Therapist
I believe that occupational therapy, when offered in a competent and professional manner, by someone who has taken the time to understand Guillain-Barré Syndrome, is one way to make a significant difference. "Referral to occupational therapy is common when the course of GBS is moderate to severe... [the occupational therapist should] assess communication, control of the environment as appropriate, comfort, and level of anxiety." (Trombly 903)
At the intensive care stage, in addition to initial, upper body range of motion exercises, the occupational therapist may assist by intervening in other ways:
· Developing communication tools, such as [a] sign or picture board.
· Adapting TV and lights to remote control
· Modifying the telephone for hands-free use
· Modifying positions for lying and sitting to those optimal for function and comfort
· Teaching about GBS and recommending other support services
· Teaching strategies to reduce anxiety." (Trombly 903)
When the individual is moved to a rehabilitation hospital, occupational therapy interventions shift in focus to eliciting controlled and coordinated movement, especially of the arms and hands. This can be achieved through tasks which require trunk balance, sitting or standing exercise (with light weights, ball and balloon games, etc) and supervised or assisted activities of daily living. An example of how these sessions can be graded is seen in the range of difficulty between sitting upright in a chair with a back, to completing upper body dressing with no support, in a sitting position (Wingert). In other words, "occupational therapy professionals should be involved early in the rehabilitation program to promote upper body strengthening, range of motion, and activities to promote functional self-care." (Scott, et al.)
The more intangible responsibilities of the occupational therapist in a post-acute setting are threefold. First, to instruct: concerning mobility, conservation of energy, work simplification, and community access. Next, to train: in transfers, modified self-care techniques, and the use of aids and adaptive equipment. And finally, to collaborate in order to adapt routines, habits, equipment, and strategies for daily living. (Trombly 903)
Barriers, Supports and Resources
Standing in the way of a return to one's previous life is a body that has betrayed you. This factor seems to be the biggest hurdle to recovery. Secondary to this is the general societal attitude that if someone looks okay, s/he is okay. Karen addressed this issue, "Looks are deceiving... [People forget about] the residuals…fatigue and neuropathies- such as burning legs- caused by axonal damage... As far as I am concerned, there are no ‘mild’ cases of GBS. It does a number on your life."
Because of this, she believes that all GBS survivors should consider seeing a psychologist, as well as taking low dose anti-depressants, while they consider all the questions which need answering. Another blessing is to be found in the involvement of family and friends, even if it is something as simple as a get-well card. New friends, in a similar situation, are often found in GBS support groups. Notably, among those I interviewed, is Guillain-Barré Syndrome Foundation International (www.gbsfi.com), which features a discussion forum and chat rooms, for questions and support. Another website that is by, about, and for GBS survivors is found at www.aboutgbs.com.
Adaptive equipment is usually helpful. Karen considers the following to be noteworthy: "a motorized wheelchair, [raised] commode, bath bench, lift chair, sliding board, as well as a ‘ladder’ to help pull me up in bed at night."
A "New" Intervention
The more I learn about Guillain-Barré, the more I realize that the only true experts on the subject are those who have experienced its effects first-hand. My intent for this paper is not to dwell upon that fact, or chide occupational therapists for a lack of expertise. Neither is it my intention to imply that GBS is the only condition which results in dramatically different symptoms and problem areas from one survivor to the next. My purpose, first and foremost, has been to increase education and awareness about Guillain-Barré. Second to that, my hope is that this essay will serve as a gentle reminder, an intervention, perhaps, to therapists and other students that GBS survivors know their own limitations better than anyone. 'No pain, no gain' is not always true, especially for one who has experienced Guillain-Barré Syndrome, and has an intimate understanding of pain.
References
On-Line Medical Dictionary, Retrieved on February 25, 2004, from
http://cancerweb.ncl.ac.uk/omd/
Acute Inflammatory Polyneuropathy (Guillain-Barré Syndrome). (2004.)
Retrieved February 11, 2004, from Cecil Textbook of Medicine, University
of Virginia School of Medicine Internet Database.
DeLisser, H., (2004.) Guillain-Barré syndrome. Retrieved February 11, 2004,
from UpToDate, University of Virginia School of Medicine Internet
Database.
Guillain-Barré Syndrome. (1997.) Prepared by Office of Communications and
Public Liaison, National Institute of Neurological Disorders and Stroke,
National Institutes of Health. Bethesda, MD: U.S. Dept. of Health and
Human Services, Public Health Service, National Institutes of Health.
Evers, E., Hughes, R., (June, 1998.) Guillain-Barré Syndrome Support Group.
Retrieved February 28, 2004, from www.gbs.org.uk/cidp.html
Stratton, K., Howe, C., Johnston Jr., R. (Eds.) (1994) Adverse events associated
with childhood vaccines [computer file]: evidence bearing on causality.
Washington, DC: National Academy Press.
Broomfield, J., (2003.) Guillain-Barré syndrome. Retrieved on February 28, 2004,
from http://health.discovery.com/diseasesandcond/encyclopedia/666.html.
Health for Life: Guillain Barré Syndrome. (1995.) The Ohio State University
Medical Center: Department of Critical Care Nursing. Retrieved on
February 28, 2004, from http://devweb3.vip.ohio-
state.edu/Materials/PDFDocs/dis-cond/general/guillian.pdf
Trombly, C., Radomski, M., (Eds.) (2002.) Occupational Therapy for Physical
Dysfunction. Baltimore: Lippincott Williams & Wilkins.
Wingert, K., (2003.) The Virtual Health Care Team: Guillain-Barré. Retrieved on
March 1, 2004, from www.vhct.org/case799/team_goals_3.shtml.
Scott, D., Talavera, F., Andary, M., Allen, K., and Lorenzo, C., (Eds.) (2004.)
Guillain-Barré Syndrome. Retrieved on March 1, 2004, from
http://www.emedicine.com/PMR/topic48.htm
Sunday, March 14, 2004
GBS Newsletter - The Communicator Spring 2003
Residual Effects Following Guillain-Barre
Gareth J. Parry, MD Professor of Neurology, University Of Minnesota
Guillain-Barr~ syndrome is a disorder whose excellent prognosis is invariably emphasized. Widely accepted figures suggest that 75%-85% of patient make a complete recovery. However, many of my patients have complained to me of minor but annoying persistent symptoms continuing for years after the initial paralytic event. Although I have made no systematic study of the proportion of patients with these residual complaints it is certainly more than the 15%-25% that the figures in the literature would suggest. The great majority of studies of the ultimate outcome in GBS are based on telephone interviews or retrospective chart reviews and seemingly minor complaints may have been missed or disregarded. Thus, patients are often asked if they have returned to their previous work or other previous activities but they may not have been asked whether they have more difficulty performing their former activities. A note of caution was sounded in one small study from Dr. J. McLeod and his colleagues in Australia (J. Neurol Sci 1976; 27:438-443) who objectively evaluated a small group of 18 recovered GBS patients and found that half of them had objective residual neurological abnormalities. Even then the residual abnormalities were considered to be significant in only four patients. A recent important paper from Dr. I.S.J. Merkies and colleagues in Holland (Neurology 1999; 53:16481654) has established that residual effects from both GBS and CIDP are much more common than has been generally reported and that seemingly minor neurologic abnormalities may still result in annoying disabilities. The study used a validated index of fatigue severity to assess residual disability. It included 83 patients who had suffered from GBS an average of five years previously. About 80% of these patients experienced fatigue that was considered severe enough to interfere with their life despite the fact that the majority had normal strength or only minor weakness. They noted also that the fatigue did not seem to improve over time; the fatigue index score was the same in patients in whom many years had elapsed as it was in patients whose acute illness had occurred only 6-12 months previously. This paper provides sound scientific support for the validity of the observations of my patients who regularly complain of fatigue even when they have returned to all or most of their former activities and who are working full time at their former jobs. Although their strength may be normal when they are examined in the doctor's office they are clearly unable to sustain the same level of physical activity that they had performed prior to their GBS.
A second under-appreciated symptom that may persist for many years is pain. Certainly, severe disabling pain is very rare. However, a number of my patients complain of persistent discomfort in their feet. The discomfort may take the form of annoying paresthesias (tingling) or there may be a vague aching discomfort. Occasionally there is more severe burning or stabbing pain. The symptoms have the same characteristics as typical neuropathic pain in that they tend to be worse in the evening or at night and are particularly annoying following days during which the patients have been up on their feet a lot. The discomfort is not particularly responsive to analgesics but usually does respond to antiepileptic drugs such as gabapentin, or antidepressant drugs such as amitriptyline, drugs typically used in the treatment of neuropathic pain. However, these medications have to be taken on a daily basis to be effective and one problem with deciding whether to treat this residual symptom is that the discomfort is usually rather mild. Thus, patients may be daily irritated by their symptom but be reluctant to take a drug every day for a symptom that significantly bothers them only once or twice a month. I have seen no mention in the medical literature of this phenomenon. It is possible that I see a highly selected group of patients in my practice who had initially been more severely affected and that the prevalence of this annoying residual symptom is much higher in my patients than in the usual population of recovered GBS patients. I would be most interested to learn whether the group of patients reported by Merkies and colleagues also suffered from minor persistent discomfort.
The basis for both of these seemingly minor residual symptoms (fatigue and pain) is probably axonal degeneration. During the acute illness the predominant underlying pathology in most patients is segmental demyelination, a completely reversible phenomenon. However, some degree of axonal degeneration is almost invariable and in some patients it is severe. As recovery occurs function is restored by a number of mechanisms. Axonal regeneration of motor axons probably plays very little role in restoration of function except in the more severe cases. Rather, surviving axons send out small branches called collateral sprouts that restore the nerve supply to those muscle fibers whose nerves have been damaged. This process of collateral sprouting is very effective at restoring strength to a muscle but the efficiency of the muscle suffers the muscle must work harder to achieve its goals. Thus, fatigue may result even when there appears to be full restoration of strength. On the sensory side, even a small number of damaged sensory axons may be sufficient to generate spontaneous discharges that are registered as pain or discomfort.
It is entirely appropriate that the good outcome of GBS should be emphasized during the acute illness. During this time, the patient is losing control of many motor functions, sometimes including life-preserving functions, and constant reassurance from the attending physicians plays a vital role in the recovery process. However, it is equally important to be aware that residual problems are experienced by "recovered" GBS patients. Acknowledgement that such residual problems exist will go a long way towards helping patients deal with the frustration of their incomplete recovery.
More research is needed to discover an effective treatment for the residual fatigue. In addition, since these persistent symptoms are probably related to the degree of axonal damage that occurs at the time of the initial attack, we also need to continue to strive for earlier and more effective treatment of the acute stage of the disease so that these residual problems are minimized.
Residual Effects Following Guillain-Barre
Gareth J. Parry, MD Professor of Neurology, University Of Minnesota
Guillain-Barr~ syndrome is a disorder whose excellent prognosis is invariably emphasized. Widely accepted figures suggest that 75%-85% of patient make a complete recovery. However, many of my patients have complained to me of minor but annoying persistent symptoms continuing for years after the initial paralytic event. Although I have made no systematic study of the proportion of patients with these residual complaints it is certainly more than the 15%-25% that the figures in the literature would suggest. The great majority of studies of the ultimate outcome in GBS are based on telephone interviews or retrospective chart reviews and seemingly minor complaints may have been missed or disregarded. Thus, patients are often asked if they have returned to their previous work or other previous activities but they may not have been asked whether they have more difficulty performing their former activities. A note of caution was sounded in one small study from Dr. J. McLeod and his colleagues in Australia (J. Neurol Sci 1976; 27:438-443) who objectively evaluated a small group of 18 recovered GBS patients and found that half of them had objective residual neurological abnormalities. Even then the residual abnormalities were considered to be significant in only four patients. A recent important paper from Dr. I.S.J. Merkies and colleagues in Holland (Neurology 1999; 53:16481654) has established that residual effects from both GBS and CIDP are much more common than has been generally reported and that seemingly minor neurologic abnormalities may still result in annoying disabilities. The study used a validated index of fatigue severity to assess residual disability. It included 83 patients who had suffered from GBS an average of five years previously. About 80% of these patients experienced fatigue that was considered severe enough to interfere with their life despite the fact that the majority had normal strength or only minor weakness. They noted also that the fatigue did not seem to improve over time; the fatigue index score was the same in patients in whom many years had elapsed as it was in patients whose acute illness had occurred only 6-12 months previously. This paper provides sound scientific support for the validity of the observations of my patients who regularly complain of fatigue even when they have returned to all or most of their former activities and who are working full time at their former jobs. Although their strength may be normal when they are examined in the doctor's office they are clearly unable to sustain the same level of physical activity that they had performed prior to their GBS.
A second under-appreciated symptom that may persist for many years is pain. Certainly, severe disabling pain is very rare. However, a number of my patients complain of persistent discomfort in their feet. The discomfort may take the form of annoying paresthesias (tingling) or there may be a vague aching discomfort. Occasionally there is more severe burning or stabbing pain. The symptoms have the same characteristics as typical neuropathic pain in that they tend to be worse in the evening or at night and are particularly annoying following days during which the patients have been up on their feet a lot. The discomfort is not particularly responsive to analgesics but usually does respond to antiepileptic drugs such as gabapentin, or antidepressant drugs such as amitriptyline, drugs typically used in the treatment of neuropathic pain. However, these medications have to be taken on a daily basis to be effective and one problem with deciding whether to treat this residual symptom is that the discomfort is usually rather mild. Thus, patients may be daily irritated by their symptom but be reluctant to take a drug every day for a symptom that significantly bothers them only once or twice a month. I have seen no mention in the medical literature of this phenomenon. It is possible that I see a highly selected group of patients in my practice who had initially been more severely affected and that the prevalence of this annoying residual symptom is much higher in my patients than in the usual population of recovered GBS patients. I would be most interested to learn whether the group of patients reported by Merkies and colleagues also suffered from minor persistent discomfort.
The basis for both of these seemingly minor residual symptoms (fatigue and pain) is probably axonal degeneration. During the acute illness the predominant underlying pathology in most patients is segmental demyelination, a completely reversible phenomenon. However, some degree of axonal degeneration is almost invariable and in some patients it is severe. As recovery occurs function is restored by a number of mechanisms. Axonal regeneration of motor axons probably plays very little role in restoration of function except in the more severe cases. Rather, surviving axons send out small branches called collateral sprouts that restore the nerve supply to those muscle fibers whose nerves have been damaged. This process of collateral sprouting is very effective at restoring strength to a muscle but the efficiency of the muscle suffers the muscle must work harder to achieve its goals. Thus, fatigue may result even when there appears to be full restoration of strength. On the sensory side, even a small number of damaged sensory axons may be sufficient to generate spontaneous discharges that are registered as pain or discomfort.
It is entirely appropriate that the good outcome of GBS should be emphasized during the acute illness. During this time, the patient is losing control of many motor functions, sometimes including life-preserving functions, and constant reassurance from the attending physicians plays a vital role in the recovery process. However, it is equally important to be aware that residual problems are experienced by "recovered" GBS patients. Acknowledgement that such residual problems exist will go a long way towards helping patients deal with the frustration of their incomplete recovery.
More research is needed to discover an effective treatment for the residual fatigue. In addition, since these persistent symptoms are probably related to the degree of axonal damage that occurs at the time of the initial attack, we also need to continue to strive for earlier and more effective treatment of the acute stage of the disease so that these residual problems are minimized.
And the beat goes on... Not a lot to report lately, My recovery continues to be painfully slow. One bit of news for my fellow Colorado GBS'ers, May 1st, 2004 from 1:00 pm till 4:00 pm we will be having our statewide meeting at the Denver West Mariott Hotel in Denver,Co. For more information please contact Ann Brandt at anngeob@msn.com or myself at jim_cannon27@yahoo.com. Either one of us can get you directions and further details. We look foward to seeing all of you there!!
