Reaching Down the Rabbit Hole by Allan H. Ropper

Author:Allan H. Ropper [Ropper, Allan H.]
Language: eng
Format: epub
ISBN: 9781494558222
Amazon: 149455822X
Publisher: Tantor Audio
Published: 2014-12-29T16:00:00+00:00

8

Endgame

Facing down Lou Gehrig’s disease

Louise Nagle had been kicking around doctors’ offices and emergency rooms for three months, complaining of breathing trouble while climbing stairs. She was referred to pulmonary and cardiac experts, who could not sort out the cause. Understandably frustrated, she came into the Emergency Department one night at about 8:00 after almost choking to death on a dinner of cubed chicken, peas, and rice.

Hannah was the senior resident on duty. When we entered Mrs. Nagle’s cubicle, we saw the trouble almost immediately: a flutter that can make your heart sink, a slight palpitation between the thumb and first finger that signifies degeneration of the motor nerves. In other words, ALS, or Lou Gehrig’s disease.

As Hannah and I stood over her bed, we could feel Mrs. Nagle’s distress. Her nostrils flaring like a racehorse’s, she was using every muscle in her shoulders and thorax just to breathe. Beads of sweat welled up on her forehead and cheeks. She looked vaguely like someone having an asthma attack, but as she grabbed the side rail of the stretcher with her left hand in an attempt to sit up, I kept my eye on her right hand. It lay motionless on the sheet. If only it had been motionless. I don’t know why I looked, but I couldn’t help noticing the butterfly of doom flitting between her thumb and finger.

Fasciculations—the fluttering of small parts of a muscle, or what used to be called live flesh—are almost always benign. Virtually everyone has had the experience: small contractions around the eyes or the mouth, in the calf or the forearm. It happens when people are tired or they’ve had too much coffee or alcohol. Tending as they do to come in clusters over several days, these muscle flutters can reflect a patient’s self-involvement or their fear of illness. When that patient is a medical student or a physician, he (it’s usually a he) often becomes immobilized by the experience, and will either demand an immediate medical examination or simply hide in fear, hoping to cancel out a degenerative spinal condition by ignoring it. Although my evidence is entirely anecdotal, I have become convinced of two things: (1) physicians complain disproportionately of this symptom and are hard to persuade that it is benign; and (2) only the nicest people get ALS (thus immunizing a substantial number of physicians).

Benign fasciculations can lead to a cramp, but are unassociated with muscle weakness. They go away. The bad twitches, such as the one I saw in Mrs. Nagle’s hand, result from the dying of nerve cells in the spinal cord. They occur when a group of muscle fibers loses the connection to its controlling nerve cell—the dying cell—and an adjacent surviving cell then takes over control of those fibers. This new, larger, motor unit cannot easily be sustained by the single nerve cell, and becomes very unstable, producing the spontaneous contractions in the remolded motor unit.

Mrs. Nagle’s case was advanced enough, as evidenced by the muscle flutters

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