Lightning can strike twice

Friday, April 24, 2009

It takes a celebrity to draw attention to illnesses that often fly under the radar. Such is the case of William “The Fridge” Perry, the Ex-Chicago Bear football player who came to national attention when the team won the Super Bowl with Perry as a defensive star. In 2008, Mr. Perry spent five months in the hospital because of Guillain-Barré syndrome and survived an illness that is a potential killer.

Nobody knows what why Guillain-Barré syndrome occurs. It’s an autoimmune disease in which the body attacks its own tissue; in this disease, the tissue is the myelin that covers nerve fibers and the attack causes failure of those nerves to work. This leads to symptoms that begin with numbness and tingling of the arms or legs and can progress to muscle weakness. The major worry with this condition is that important muscles like those that help us breathe become weak, and respiratory failure can occur.

While doctors don’t know why, Guillain-Barré syndrome often occurs after colds and stomach flu. The disease can progress slowly over a few weeks or can rapidly cause paralysis of the arms, legs, and chest muscles within hours. The symptoms are pretty non-specific when they begin, perhaps a little tingling in the feet and hands. The tingling doesn’t spread, but soon symmetric weakness or paralysis can begin. It most often starts in the legs and spreads upwards to the trunk and the head. There may be problems with vision and swallowing as a variety of muscles fail. The big deal, though, is weakness of the diaphragm and rib muscles that allow breathing to happen.

The disease is rare and presents a diagnostic challenge to doctors. A variety of illnesses cause neuropathy, the failure of nerves to function. From common diseases such as diabetes and alcoholism to those most rare, such as botulism and heavy metal poisoning and many diseases in between, there are plenty of reasons for nerves to stop conducting electricity and fail to work properly. The history and physical examination are what makes the diagnosis along with the doctor’s index of suspicion. Tests to confirm the diagnosis include a spinal tap to check protein levels in the spinal fluid and nerve conduction studies to see whether the nerves are functioning normally.

Treatment is supportive, making certain the patient can breathe. Some of those affected may end up on a ventilator until the nerves begin to function again. Supportive treatment may include plasmapheresis, in which plasma is cleansed of antibodies with the hope of removing those that have attacked the nerve cells and intravenous immunoglobulin which reintroduces normal antibodies to the body.

The Fridge is one of the unlucky persons to have survived the initial attack of Guillain-Barré syndrome and then have a recurrence. More than 80 percent of people recover from the illness with little complication. Only one percent or less can have a relapse and when that happens, the treatment and worry begin all over again.

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