May | 2014 | Neurology Update

On March 6 1987 the unthinkable happened. The roll-on roll-off passenger ferry The Herald of Free Enterprise left the Belgian port of Zeebrugge with it’s bow doors open, and capsized within moments, killing 193 passengers and crew:

The immediate cause of the sinking was found to be negligence by the assistant boatswain, asleep in his cabin when he should have closed the bow-door.

But the official inquiry placed more blame on his supervisors and a general culture of poor communication in the ferry company P&O European Ferries. The first officer was required to stay on deck to make sure the doors were closed, but apparently left the deck with the bow doors still open in the expectation that the assistant boatswain would arrive shortly. The boatswain, who is believed to have been the last person on the deck before the disaster was later asked why he did not close the doors given there was no one else there to do it. He said it was “not his duty”. Finally, the captain assumed that the doors had been closed when he set sail – he could not see them from the wheelhouse owing to the ship’s design and the fact that the shipping company had previously decided not to retrofit an indicator light in the wheelhouse.

Serious medical errors often occur under similar circumstances, including flawed or violated safety protocols and failure of communication.

For example, there were three instances of wrong side brain surgery at the Rhode Island Hospital in 2007. One time an experienced brain surgeon insisted to a nurse he knew what side of the head to operate on — but got it wrong. Another time, a resident cut into the wrong side of a patient’s head after skipping a pre-op checklist. In the third case, the chief resident started brain surgery in the wrong place, and the nurse didn’t stop him.

These errors happened despite required operating-room precautions adopted by the medical profession a few years ago to prevent such “wrong-site surgery” mistakes, including checklists, “time-outs” to double-check everything is correct, and indelible markers to show the surgeon where to cut.

These mistakes at Rhode Island Hospital suggest that such precautions can still be thwarted by the human element — ego and overconfidence on the part of surgeons, and timidity on the part of nurses too afraid to speak up when they see something about to go wrong.

Ongoing efforts to improve patient safety include: The use of information technology, such as hand-held bedside computers, to eliminate reliance on handwriting for ordering medications and other treatment needs. Avoiding similar-sounding and look-alike names and packages of medication. The standardization of treatment policies and protocols to avoid confusion and reliance on memory, which is known to be fallible and responsible for many errors.

However, the most important patient safety step is adopting a culture of patient safety at the hospital.

All staff must be encouraged to speak up if they identify a problem or mistake.

If the boatswain or first officer had simply flicked the switch to close the doors, or notified the captain they were still open, disaster could have been avoided on March 6, 1987.

George Huntington (1850-1916) was a medical practitioner in Dutchess County New York.

His father and grandfather had both been doctors, and his family had lived on Long Island since 1797.

He gave his classic presentation, “On Chorea”, at the Meiga and Mason Academy of Medicine in Middleport Ohio in 1972, and this was subsequently published in the Medical and Surgical Reporter of Philadelphia on April 13, 1872.

This took place just one year after he graduated from his medical training at Columbia University.

He later explained his interest in the condition that now bears his name:

Over 50 years ago, in riding with my father on his rounds, I saw my first case of the “disorder”, which is the way the natives always referred to the dreaded disease. I recall it as vividly as though it had just occurred but yesterday. It made a most enduring impression on my boyish mind, an impression which was the very first impulse to my choosing chorea as my virgin contribution to the medical lore. Driving with my father through a wooded road leading from East Hampton to Amagansett we suddenly came upon two women both bowing, twisting, grimacing. I stared in wonderment, almost in fear. What could it mean? My father paused to speak with them and we passed on. Then my Gamaliel-like instruction began; my medical instruction had its inception. From this point on my interest in the disease has never wholly ceased.

Huntington’s disease (HD) is now know to be caused by a genetic mutation. It is inherited in an autosomal dominant fashion, so that any child of an affected person typically has a 50% chance of inheriting the disease. The mutation is a triplet repeat, so the genetics of HD lead to anticipation, a phenomenon whereby the disease may begin earlier and more severely in each successive generation.

HD can present at any age, but most often begins around 35-44 years with psychiatric disturbance such as depression or forgetfulness. Neurological manifestations such as unsteady gait and the jerky body movements (chorea) noted by George Huntington come later, followed by a progressive dementia.

Genetic testing is available, but pre-symptomatic testing for family members, who are at increased risk for the disease, is controversial since there is no treatment for HD.