HEALTH TALK: Who is Mr. Guillian Barre?

The question is “Who was Mr. Guillain Barre?” because he is dead. Your guess is as good as mine. All I know is that his name was given to a syndrome which is an uncommon inflammatory disorder in which your body’s nerves are attacked by its own immune system, causing severe weakness and numbness. These symptoms usually start in your extremities and quickly worsen. Eventually your entire body can become paralyzed, even the muscles used for breathing.

What exactly causes Guillain Barre syndrome is unknown, but it sometimes is triggered by a respiratory infection or the stomach flu. This potentially fatal disorder is relatively rare; only one or two people per 100,000 worldwide get it. All age groups are affected, but it occurs most often in young adults and the elderly.

In its most severe form, Mr. Guillain Barre (most likely Monsieur Guillain Barre) is a medical emergency requiring hospitalization. One in three sufferers will temporarily need assisted ventilation – the help of a machine to breathe.

There is no cure for the syndrome, but several treatments can ease symptoms and reduce the illness’ duration. Most people recover completely from even the most severe of Guillain Barre syndrome.

SIGNS AND SYMPTOMS

Guillain Barre usually begins with weakness, tingling or loss of sensation starting in your feet and legs and spreading to your upper body and arms. The symptoms begin for some folks in the arms or even the face. As the syndrome progresses, muscle weakness can become paralysis. Other signs and symptoms include:

•    Difficulty with eye movement, speaking, facial movement, chewing or swallowing.
•    Severe pain in the lower back.
•    Difficulty with intestinal functions or bladder control.
•    Difficulty breathing.
•    Very slow heart rate or low blood pressure.

Most people experience their worst weakness within three weeks after symptoms begin. Sometimes, signs and symptoms may progress very quickly with complete paralysis of legs, arms and breathing muscles over the course of a few hours.

CAUSES

The exact cause is still unknown. In about 60 percent of the cases, an infection affecting either the lungs or the digestive tract precedes the disorder. Why such an infection can lead to Guillain Barre in some people and not in others, is unknown. Many cases seem to occur without any triggers.

In Guillain Barre, your immune system – which usually only attacks foreign material and invading organisms – starts attacking the nerves that carry signals between your body and your brain. What happens is that the nerves’ protective covering (myelin sheath) is damaged, interfering with the signaling process, causing weakness, numbness or paralysis.

WHAT ARE THE RISK FACTORS?

The most common factor that triggers Guillain Barre syndrome seems to be infection with Campylobacter, a bacteria commonly found in undercooked food, especially poultry. It may also be triggered by surgery and, very rarely, influenza immunizations. Other triggers include:

•    Hodgkin’s disease
•    Mononucleosis (kissing disease)
•    HIV,  the AIDS virus

COMPLICATIONS

A potentially fatal complication of Guillain Barre syndrome is that weakness or paralysis can involve muscles controlling your breathing. About 30 percent of sufferers will temporarily require help from a machine to breathe. About 5 percent will die, despite intensive care.

80 percent of people with Guillain Barre syndrome recover completely or have only minor, residual weakness or abnormal sensations, such as numbness or tingling. Five to ten percent get more serious, permanent problems with sensation and co-ordination, including some cases of severe disability. And about 10 percent of those affected is at risk of experiencing a relapse.

TREATMENT

Although there’s no cure for Guillain Barre syndrome, two treatments have been shown to speed the recovery from and reduce the severity of the disorder:

•    Plasmapheresis. This treatment – also known as plasma exchange – is a type of “blood cleansing” in which damaging antibodies are removed from your blood. Plasmapheresis consists of removing the liquid portion of your blood (plasma) and separating it from the actual blood cells. The blood cells are then put back into your body, which manufactures more plasma to make up for what was removed. Why this treatment works is not clear, but scientists believe that plasmapheresis rids plasma of certain antibodies that contribute to the immune system attack on the peripheral nerves.

•    Intravenous immunoglobulin. Immunoglobulin contains healthy antibodies from blood donors. High doses of immunoglobulin can block the damaging antibodies that may contribute to Guillain Barre syndrome.

Each of these treatments is equally effective. Mixing them or giving one after the other is no more effective than using either method alone.

Some people can take months or even years to recover, but most cases of Guillain Barre syndrome follow this general timeline:

•    Following the first symptoms, the condition tends to progressively worsen for about two weeks.
•    Symptoms reach a plateau and remain steady for two to four weeks.
•    Recovery begins.

The treatments mentioned above shorten the time period before recovery begins by as much as 50 percent.

Often before recovery begins, caregivers may need to manually move your arms and legs to help keep your muscles flexible and strong. After recovery has begun, you’ll probably need physical therapy to help regain strength and proper movement so you’ll be able to function on your own. You many need training with devices such as a wheelchair or braces, to give you mobility and self-care skills.

See you next week.