Gettysburg Medical News
The Clinical View
by P.E. Hoffsten, MD
7 May 2008

A TERRIBLE IMMUNOLOGICAL MISTAKE

            I recently received a phone call from a patient inquiring about one of their children living out of state.  That “child” now married with children of his own had developed a condition call Guillain-Barré syndrome.  The caller wanted to know about this syndrome. 

            I harkened back to the first time I cared for an individual with this disease.  I was in Japan with the United States Army caring for Vietnam War casualties in 1970.  A soldier was air evacuated from Vietnam to my hospital where he stayed for 2 months being treated for Guillian-Barre Syndrome before he could be further air evacuated to the United States for recovery.

            This story is colored by the times in 1970 in Vietnam.  The patient was a soldier 20 years old who had come to Vietnam just two weeks before.  Like many new arrivals in the Vietnam War, he wasn’t exactly thrilled to be there.  Many individuals did anything they could to be evacuated home.  This particular soldier’s story begins in the early morning hours when he was called to formation.  He stumbled out of his tent with his boots unlaced, his shirt unbuttoned and barely able to hold on to his rifle.  The sergeant walking down the inspection line grabbed the soldier’s rifle, inspected it, and thrust it back to the shoulder.  The rifle promptly dropped to the ground resulting in the tirade from the sergeant on what a sloppy soldier this person was.  The soldier tried to explain that he just couldn’t hold on to anything or button his shirt or tie his shoes.  He said that his hands just didn’t work.  After further insults and verbal abuse, the soldier was told to go to the dispensary where he went through a similar experience with the intake personnel.  But when he was finally examined by medical personnel it was apparent that this soldier’s muscular strength was very abnormally impaired.  Something was very wrong.

            The soldier was evacuated to my hospital where he wound up on a respirator for almost three weeks barely holding onto his life.  He developed severe pneumonias requiring extensive antibiotic therapy.  It took two corpsmen caring for him 24 hours a day to get him through this illness.  His weight dropped from 172 pounds to 130 pounds by the time he was evacuated back to the United States.  Finally, after about three weeks, his condition began to improve and he was able to breath again without the respirator.  By the time he was evacuated to the United States, his tracheostomy site was closed but he was still too weak to walk although he had recovered enough to button his shirt.  He still required a corpsman to feed him although his tube that had been used to provide sustenance during his illness was removed. 

            I received communications from him over the next several years as his recovery continued.  Eventually, he went to engineering school and got a job with a large firm as a civil engineer.  He never felt that he was as strong as he had been prior to his illness but was married  and started a family at the time of my last communication with him in 1979.

            This patient, like the caller who inquired about Guillain-Barré’s syndrome wanted to know what in the world happened to them.  This illness is a “terrible immunological mistake”.

It is an accident of a person’s immune system in which they become allergic to the insulation on the motor nerves that make their musculature work.  The motor nerves have an insulation material called myelin that protects them and insulates these nerves from adjacent structures.  For some reason not understood, the person becomes immune to their own motor nerve insulation and destroys the cells that make it.  The nerves then short out and their muscles just don’t work.  Mild cases can result in difficulty in their hands or arms but severe cases like my patient above can affect the nerves that run the diaphragm and the person can’t breath.  Prior to the invention of effective respirators, this was a fatal disease. 

            It is now known, that the body’s immune mistake does get corrected and the myelin-producing cells grow back, insulate the nerves again and the person goes on through a relatively normal life minus whatever was lost during their disease process.  In the case of my patient above, he had such severe pneumonias that he had  residual respiratory insufficiency.  Sometimes some of the nerves do not regenerate and the person’s musculature is not completely re-established. 

            As mentioned above, this is a relatively rare disease and I can only think of four more cases that I have ever treated since 1970.  Fortunately, none were ever as bad as that first patient.  But two of the cases terminated the young man’s athletic endeavors. 

            The most important step in care is early recognition of this disease.  We now know that there are two treatments that seem to have very beneficial effect for the Guillain-Barré’s  syndrome.  Specifically, a process called plasmapheresis whereby the offending antibodies are removed from the patient does seem to be effective.  In addition, flooding the person’s immune system with an immune product called gamma globulin seems to turn off the destructive process for the patient’s nerves.  In the past, high doses of cortisone were used but this seems to be ineffective. 

            In answer to the caller, I told him about the soldier that I cared for above.  I indicated that this is a disease over which the person has no control whatever.  It isn’t anything they did that makes it happen.  Early effective treatment can be very beneficial and recovery can be complete unless there are major complications during the course of the illness.  It is a life changing disease if for nothing more than the psychological recognition of just how helpless we can be.

This and other articles available at www.macpierre.com.