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Guillain

Guillain-Barre Syndrome

Guillain-Barre syndrome is a disorder in which the body’s immune system attacks part of the peripheral nervous system. I interviewed a patient, Yvonne, who was diagnosed with Guillain-Barre when she was only 11. She struggled with the disease, went into remission, and the relapsed even worse. She is now in remission and has several checkups a year.

The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs. In many instances the weakness and abnormal sensations spread to the arms and upper body. For Yvonne, it started in her pinky toe. She thought that her toe was just "asleep". But when the tingling, sleepy sensation started to travel up her legs after a few days, she started to get concerned so she went to a doctor for a check-up, only for the doctor to say it was nothing to be concerned about. (1)

These symptoms can increase in intensity until the muscles cannot be used at all and the patient is almost totally paralyzed. In these cases the disorder is life threatening-potentially interfering with breathing and, at times, with blood pressure or heart rate-and is considered a medical emergency. The patient is often put on a respirator to assist with breathing and is watched closely for problems such as an abnormal heart beat, infections, blood clots, and high or low blood pressure. Most patients, however, recover from even the most severe cases of Guillain-Barre syndrome, although some continue to have some degree of weakness. (4;pg104-107)

Guillain-Barre can affect anybody. It can strike at any age and both sexes are equally prone to the disorder. The syndrome is rare, however, hitting only about one person in 100,000. Usually Guillain-Barre occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection ( 3; pg 16-19) Occasionally, surgery or vaccinations will trigger the syndrome. The disorder can develop over the course of hours or days, or it may take up to 3 or 4 weeks. Most people reach the stage of greatest weakness within the first two weeks after the symptoms appear, and by the third week of the illness 90 percent of all patients are at their weakest (5; pg 17-21).

What causes Guillain-Barre syndrome?

No one quite knows why Guillain-Barre strikes some people and not others. Nor does anyone know exactly what sets the disease off. What scientists do know is that the body’s immune system begins to attack the body itself, causing what is known as an autoimmune disease (4; pg. 29-33). Usually the cells of the immune system attack only foreign material and invading organisms. In Guillain-Barre syndrome, however, the immune system starts to destroy the myelin sheath that surrounds the axons of many peripheral nerves, or even the axons themselves (2; pg90-94).

In diseases were the peripheral nerves’ myelin sheaths are injured, the nerves cannot transmit signals efficiently. That is why the muscles begin to lose their ability to respond to the brain’s commands that must be carried through the nerve network ( 4;66). Yvonne does not know what caused the onset of the disease in her case, but after only 2 weeks, she lost all ability in her legs and in her arms (1).

How is Guillain-Barre syndrome diagnosed?

Guillain-Barre is called a syndrome rather than a disease because it is not clear that a specific disease causing agent is involved. A syndrome is a medical condition characterized by a collection of symptoms and signs. The signs and symptoms of the syndrome can be quite varied, so doctors may, on rare occasions, find it difficult to diagnose Guillain-Barre in it’s earliest stages ( 6; 99-101).

Yvonne had a hard time figuring out what was wrong with her. After excruciating tests which included tests for Aids, Multiple Sclerosis, Lupus, Lime’s disease, Polio and other diseases, she finally had a test done called an EMG or electromagnetic gram. This tested the velocity of her nerves. When the doctors finally figured out that it was Guillain-Barre, they started a treatment called plasmaphresis(1). Plasmapheresis is a method by which whole blood is removed from the body and processed so that the red and white blood cells are separated from the plasma, or liquid portion of the blood. The blood cells are then returned to the patient without the plasma, which the body quickly replaces(3). Yvonne went through plasmapheresis every other day for a week straight. She could not go every day because her body would go into shock from the blood loss from day to day. After a week straight of plasmapheresis, she finally started to regain feeling in her arms and was sent home for her first remission(1). Another type of treatment that is effective is called high-dose immunoglobulin therapy. In this treatment, doctors give intravenous injections of the proteins that, in small quantities, the immune system uses naturally to attack invading organisms. Investigators have found that giving high doses of these immunoglobulins, derived from a pool of thousands of normal donors, to Guillain-Barre patients can lessen the immune attack on the nervous system(4; 201-205).

What are the long-term effects for those with Guillain-Barre?

Guillain-Barre can be a devastating disorder because of it’s sudden and unexpected onset. In addition, recovery is not necessarily quick. As noted above, patients usually reach the point of greatest weakness or paralysis days or weeks after the first symptoms occur. Symptoms then stabilize at this level for a period of days, weeks or, sometimes, months. The recovery period may be as little as a few weeks or as long as a few years. About 30 percent of those with Guillain-Barre still have residual weakness after 3 years. About 3 percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack(4; 74-78).

For Yvonne, she stated that her nervous system is really out of whack. For example, when her funny bone gets hit, there is extreme pain. When she gets something as minor as a cold or the flu, while in most people, it would be gone is a few days, it take her body a few weeks to fight the infection. When she gets pinched or cut, it also takes her a few seconds to realize that it is painful, and then the pain becomes real intense. She stated that just the other day she was painting her fingernails and she could feel the stroke

of the brush on the finger underneath her nail, and on the fingernail next to the one she was painting.

Yvonne now goes through physical therapy and has checkups once every two or three weeks. She has been in recovery for about 2 years(1).

Patient Care for Guillain-Barre

Patient care involves coordinated efforts of a neurologist, general physician, GP, physiotherapist, occupational therapist, social worker, nurse and psychologist or psychiatrist. Emotional support from family and friends, and information about this rare disorder may help the patient learn to deal with this frustrating, disabling and potentially catastrophic illness. A particularly frustrating consequence of this disorder is long-term recurrences of fatigue and/or exhaustion as well as abnormal sensations or muscle aches. These problems can occur following the exertion of normal walking or working and can be alleviated or prevented by reduction of activity and rest(7).

Pertinent facts about Guillain-Barré syndrome include the following (the figures are approximate):

Frequency is about 1 to 2 cases in 100,000 population each year (0.001 - 0.002%); the disorder is rare.

About 50% of cases follow a viral illness.

Diagnosis can be difficult in the syndrome's early stages.

The disorder is not contagious.

Half the patients initially develop abnormal sensations; 25% present initially with muscle weakness (often difficulty walking); 25% present initially with both abnormal sensations and weakness.

Rehabilitation is the major form of treatment.

Recovery may occur over 6 months to 2 years or longer. It can occur sooner.

In the early stages of the illness, prognosis or long-term outcome is not predictable.

Up to 90% of patients eventually have complete or almost complete recovery.

Five to 15% of patients will have significant long-term disability or handicap.

Perhaps 35 to 45% of patients have long lasting, but mild, abnormalities.

The frequency of death is about 1 - 5% usually due to respiratory or cardiovascular complications. (7)

Bibliography:

1) Balleto, Yvonne- Personal friend. Interview and questions done by Julie Salzmann.

2) Brachnow, Mitchell Understanding Guillain-Barre Syndrome New York Publishing House, New York 1993.

3) Daniels, Aimee Guillain-Barre:French Polio Christoff Publications, Orlando 1995.

4) Harris, Scott Guillain-Barre Monmouth Publishers, Indianapolis, 1994.

5) Jokenstein, John Karl The Truth About Guillain-BarreCancer Research Institute, 1995.

6) Guillain-Barre Fact Sheet. www.ninds.nih.gov

7) www.cancer.com

Source: Essay UK - http://www.essay.uk.com/coursework/guillain.php



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