Chapter 1

The Birth of an Idea

John Walshe

Abstract

<p>The story began in 1912, but my involvement only began in 1951. In 1912 Dr Kinnier Wilson, working at the National Hospital for Nervous Diseases in London described a new illness affecting youngsters, the principal symptoms being loss of control of movement and contractures of the limbs. This was always associated with scarring of the liver. All these patients died with in a few months of diagnosis.</p> <p>Dr Wilson's life long ambition was to find a cure for this disease which became known as Wilson's disease, technically hepatolenticular degeneration.. In 1948 Professor John Cumings, working at the same hospital, showed that patients dying of Wilson's disease all had large abnormal amounts of copper deposited in the brain and in the liver. He suggested that the new metal binding drug, British Antilewisite (BAL) might be used to arrest the course of the disease.</p> <p>In 1951, working in the Metabolic Unit at University College Hospital in London I discovered that patients treated with the antibiotic penicillin excreted in their urine, a breakdown product of the drug, penicillamine. Later, when studying as a Fulbright Fellow in the Liver Unit at the Boston City Hospital, I saw a patient with Wilson's disease being treated, with little, benefit, with BAL. I suggested that penicillamine had the right structural formula to bind copper and promote its excretion and this would be a more effective treatment than BAL and, could be given my mouth and not by painful injection, it would be easier to give over a lifetime.</p>

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