Copper: Quest for a Cure

Indexed in: Scopus

This is the story of how Wilson’s disease, a previously rare and fatal inherited disease, was conquered by a series of individual discoveries, leading to highly effective treatments. It also ...
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John Walshe

Abstract

On returning to University College Hospital in London I needed to find patients with this rare disease and supplies of penicillamine to assess its ability to mobilise copper from their abnormal body stores. Three patients were found by my father, Sir Francis Walshe, a distinguished neurologist, and with the small amount of penicillamine available to me I was able to show that in each case penicillamine promoted the excretion of more copper than did BAL. Two patients were returned to their referring physicians but the third stayed with me for over 50 years and made an excellent recovery eventually having three children of her own.

Supplies of penicillamine were secured when I was able to convince the medical director of the Distillers Company, the principal makers of penicillin at the time, to make penicillamine for me.

By the early 1960s reports from my own work and from other centres appeared showing that patients treated with penicillin showed a remarkable improvement in all symptoms.

However Propfessor Denny Brown and Dr Uzman in Boston still believed that Wilson's disease was not due to copper deposition but due to abnormal protein metabolism with abnormal peptides being excreted in the urine. Working with Professor Milne and Dr Asatoor at the Hammersmith Hospital, it was possible to disprove this theory using newer techniques to analyse urine.

In 1957, after failing to get appointed to two different appointments in Oxford I was accepted to a post in the Department of Experimental Medicine in Cambridge.

Affiliation:

University of Cambridge, UK.