About Cystic Fibrosis

A History of Cystic Fibrosis

A truly documented history of cystic fibrosis (CF) did not exist until well into the 1930s. Many cases back then of what could have been cystic fibrosis were misdiagnosed as whooping cough, chronic bronchitis or pneumonia. However, despite this lack of written history for the disease, there has been an inkling of awareness of what CF is since the 1700s, as popularized by the German saying, “A child whose forehead tastes like salt when kissed will soon die.”

The written history began in the 1930s, when the illness was first described to be a disease of its own nature.

The 1930s

The 1930s can be said to be the period of discovery for cystic fibrosis. What is considered to be the earliest paper written on the disease was made by Swiss pediatrician Dr. Fanconi. The doctor called the illness “celiac syndrome,” which he defined as changes in the pancreas as observed in children.

The term cystic fibrosis was coined by Dr. Dorothy Andersen of the Babies’ Hospital New York. She was the first doctor to give the disease its earliest definitive description. She also theorized that the condition is caused by deficiency in Vitamin A.

The 1940s

Changing theories on the nature of cystic fibrosis marked this decade in its history. Drs. Sidney Farber and Harry Shwachman connected the abnormal secretion of mucus to the disease. The idea that Vitamin A is the underlying cause of cystic fibrosis was challenged by a number of researchers, including Dr. Anderson, the person who proposed it in the first place. The use of antibiotics, particularly penicillin, became part of the treatment for this condition.

The 1950s

The 1950s saw the beginnings of the sweat test, the standard test now used for diagnosing CF. The test was developed as a result of discoveries made by Dr. Paul di Sant’Agnese during the heat wave in New York in 1950.

In 1955, Dr. Shwachman laid the foundation for the modern way of treating cystic fibrosis, which was early diagnosis, active early treatment and proper nutrition. Also, Dr. Archie Norman began studies on high fat diets in treating the disease in London that year.

The 1960s

The 1960s is the period when organizations specializing in cystic fibrosis research were formed. These organizations were initiated by parents of children afflicted with the disease, as well as the rare patients who lived to see adulthood.

The 1970s

Preliminary work on proper neonatal screenings for diagnosis was the hallmark of the 1970s. This was also the decade that saw the mushrooming of many more specialized clinics for cystic fibrosis, as well as the advocacy for high-fat diets to treat the condition. The Cystic Fibrosis Foundation in the United States also pioneered the use of the patient registry during this decade.

The 1980s

Further advancements in treating the disease occurred during the 1980s. It was also during this decade that the greatest finding on the disease, none other than the discovery of the cystic fibrosis trans-membrane conductance regulator gene, was made (this was in 1989).

The 1990s

Use of gene replacement therapies began as part of the treatment for cystic fibrosis. Also, the Food and Drug Administration approved the use of the mucolytic Pulmozyne, the first drug designed to target cystic fibrosis.

The 2000s

Further research is being made by various agencies concerned with CF. The median life expectancy has reached 37 years in 2005, compared to a mere five years around 50 years ago.

The history and development of knowledge on cystic fibrosis is marked with leaps. Perhaps one of these days, this history will be marked with a cure?

Disclaimer: This website provides general information about cystic fibrosis and in no means should be taken as a medical or health advice. Please consult your doctor before acting on any of its information.