Magazine article Humane Health Care International

Cystic Fibrosis: Past, Present and Future

Magazine article Humane Health Care International

Cystic Fibrosis: Past, Present and Future

Article excerpt

The first reference to cystic fibrosis (CF), the most common lethal recessive genetic disorder in Caucasians, is not in medical texts but in European folklore of approximately 500 years ago. "Woe to that child which when kissed on the forehead tastes salt. He is bewitched and soon must die." However, it was not until 1938 that the first description of CF appeared in the medical literature. At that time, the life expectancy was only a few years. The first clue to the abnormality that causes CF came from observations made during a heat wave in New York City, when it was noted that many children with CF were becoming dehydrated. This lead to the discovery in 1953 that people with CF lose an excessive amount of salt in their sweat. Thirty years later came the breakthrough that the defect in CF is the defective transport of chloride across epithelial cells. The search for the cause of this abnormality culminated in the triumphant discovery of the CF gene by Canadian scientists in 1989.

We are now in the process of learning how a defect in the CF chloride channel can cause the signs and symptoms of CF, which have been so vividly described in Mark Lariviere's story (in this issue of Humane Health Care International). Knowledge has led to more questions, and ultimately to more therapeutic options.

Although CF is still a life-shortening disease, the outlook for life expectancy and quality of life is improving by leaps and bounds. The last three decades have brought us the benefits of better nutritional therapy, chest physiotherapy, more powerful antibiotics, and effective pancreatic enzymes, and the result has been adults with CF. …

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