Society vs the Wheelchair

Rose Marie became a patient of mine at the age of 2, just one month after I began practicing pediatrics in July of 1962. She had typical childhood illnesses and seemed otherwise normal until March of 1965, when on her preschool physical exam I picked up mild ataxia and a positive Romberg's sign. This showed slow but steady progression, so a year later I referred her to a neurologist. He felt she had a progressive degenerative cerebellar disease of unknown etiology. At that time, her only problems were an unsteady gait and "shaky" handwriting. In 1971 her sister, five years younger, was noted to have an identical disability.

By 1972, Rose Marie had ECG abnormalities consistent with Friedreich's ataxia. By that time she was confined to a wheelchair, had slurred speech, and a progressive scoliosis. In five more years she had developed diabetes mellitus (felt to be unrelated), occasional choking spells, and marked weakness and ataxia of her upper as well as lower limbs, distally more than proximally. She has absent deep tendon reflexes and loss of position and vibratory sensation in the lower limbs.

Despite her problems, Rose Marie is a B student and a very personable young lady. In the spring of 1978, a few months before she was to graduate from high school, I was sent a form from the state vocational rehabilitation director regarding her potential and prognosis. Although I felt I knew Rose Marie as well as any of my patients, my immediate reaction was, "Rehabilitation?