Guillain-Barre (ghee-yan bah-ray) syndrome is a disorder in which the body's immune system attacks part of the peripheral nervous system. In Vern's case his first symptoms included a varying degree of weakness and tingling sensation in the legs. It spread to his fingers, his back and a thin line in front across and just below the ribs. His blood pressure dropped very low when he stood.
Guillain-Barre syndrome can affect anybody. It can strike at any age and both sexes are equally prone to the disorder. The syndrome is rare, however, afflicting 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. Vern had Pneuomnia November of 1999.
What causes Guillain-Barre syndrome?
No one yet knows why Guillain-Barre strikes some people and not others. Nor does anyone know exactly what sets the disease in motion.
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. 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 (axons are long, thin extensions of the nerve cells; they carry nerve signals). The myelin sheath surrounding the axon speeds up the transmission of nerve signals and allows the transmission of signals over long distances.
In diseases in which the peripheral nerves' myelin sheaths are injured or degraded, the nerves cannot transmit signals efficiently. That is why the muscles begin to lose their ability to respond to the brain's commands, commands that must be carried through the nerve network. The brain also receives fewer sensory signals from the rest of the body, resulting in an inability to feel textures, heat, pain, and other sensations. Alternately, the brain may receive inappropriate signals that result in tingling, "crawling-skin," or painful sensations. Because the signals to and from the arms and legs must travel the longest distances they are most vulnerable to interruption. Therefore, muscle weakness and tingling sensations usually first appear in the hands and feet and progress upwards.
When Guillain-Barre is preceded by a viral infection, it is possible that the virus has changed the nature of cells in the nervous system so that the immune system treats them as foreign cells. It is also possible that the virus makes the immune system itself less discriminating about what cells it recognizes as its own, allowing some of the immune cells, such as certain kinds of lymphocytes, to attack the myelin. Scientists are investigating these and other possibilities to find why the immune system goes awry in Guillain-Barre syndrome and other autoimmune diseases. The cause and course of Guillain-Barre syndrome is an active area of neurological investigation, incorporating the cooperative efforts of neurological scientists, immunologists, and virologists.
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 (what the patient feels) and signs (what a doctor can observe or measure). 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 its earliest stages. Two days before Vern entered the hospital,Wednesday, his son helped him walk into the North Shore Group Health Clinic where he was immediately put into a wheelchair and seen by Dr. True MD who ordered a scan of his head which was done the next morning, Thursday. In the afternoon he saw his regular doctor, Dr. Hockeiser MD, who performed many tests . Vern went home but early Friday morning Dr. Hockeiser called and asked Vern to come back, during the night the doctor was thinking about the case and wanted to do more tests which he and Dr. True did finding Vern's blood pressure very low when he stood, administered about 1000 cc's of fluid to stabilize him. Consulting over the phone with other doctors, they had Vern report to Virginia Mason. We are sure glad Dr. Hockeiser was on the ball.
Several disorders have symptoms similar to those found in Guillain-Barre, so doctors examine and question patients carefully before making a diagnosis. Collectively, the signs and symptoms form a certain pattern that helps doctors differentiate Guillain-Barre from other disorders. For example, physicians will note whether the symptoms appear on both sides of the body (most common in Guillain-Barre ) and the quickness with which the symptoms appear (in other disorders, muscle weakness may progress over months rather than days or weeks). In Guillain-Barre, reflexes such as knee jerks are usually lost. Because the signals traveling along the nerve are slower, a nerve conduction velocity (NCV) test can give a doctor clues to aid the diagnosis. In Guillain-Barre patients, the cerebrospinal fluid that bathes the spinal cord and brain contains more protein than usual. Therefore a physician may decide to perform a spinal tap, a procedure in which the doctor inserts a needle into the patient's lower back to draw cerebrospinal fluid from the spinal column.
How is Guillain-Barre treated?
There is no known cure for Guillain-Barre syndrome. However, there are therapies that lessen the severity of the illness and accelerate the recovery in most patients. There are also a number of ways to treat the complications of the disease.
Currently, plasmapheresis and high-dose immunoglobulin therapy are used. Both of them are equally effective, but immunoglobulin is easier to administer. 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. Scientists still don't know exactly why plasmapheresis works, but the technique seems to reduce the severity and duration of the Guillain-Barre episode. This may be because the plasma portion of the blood contains elements of the immune system that may be toxic to the myelin.
Vern was treated with a 500 CC intravenous injection of immunoglobulin on each of the first five days in intensive care, (about $11 a CC). Immunoglobulin is derived from a pool of thousands of normal blood donors.
The most critical part of the treatment for this syndrome
consists of keeping the patient's body functioning during recovery of the
nervous system. Vern
was treated in the Virginia Mason Hospital, Seattle WA under intensive
care for one week. This included treatment for high loss of fluids. His
doctor was Thomas B. Curtis, M.D.
For more information:
Guillain-Barre Syndrome Foundation International
P.O. Box 262
Wynnewood, PA 19096
The National Institute of Neurological Disorders and Stroke of The National Institutes of Health. Guillain-Barre Syndrome. NIH Publication No. 98-2902. November 1997. Last revised August 31, 1999. (Online) http://www.ninds.nih.gov/patients/disorder/guillain/guillain.htm
I hope to return to work the 15th for two weeks, just to get my project knowledge pasted of the required persons. I will retire from Boeing on May 31.