History of Cystic Fibrosis “Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die. ” This proverb, dating back to European folklore in the 1700’s, was the diagnosis for the disease recognized today as Cystic Fibrosis (CF). In that time period the disease was usually fatal and common among infants. The excessive salt in the sweat, just one symptom of CF, made the disease identifiable. However, since the time of European folklore, various renowned scientists worked actively to discover the cause and possible treatments of Cystic Fibrosis.
Documented history of CF began during the 1930’s and each decade following that date brought new advances in the disease. The 1930’s is the decade said to be the discovery of Cystic Fibrosis. The first mediacl description of CF as an individual disease was in 1936 by a Swiss physician, Dr. Fanconi. He labeled the illness celiac syndrome and said it caused changes in children’s pancreas. In 1938, Dr. Dorothy Andersen of Columbia University in New York, named the disease Cystic Fibrosis and distinguished it from Celiac syndrome because there was progressive lung destruction and death in infancy or early childhood. Dorothy Andersen) At this time Andersen believed the disease was caused by vitamin A deficiency. The 1940’s brought a decade full of altering theories about Cystic Fibrosis. Andersen’s vitamin A deficiency theory was contested by many doctors, including herself. In 1946, researchers studied the patterns of the diseases’ inheritance and concluded it was probably caused by the mutation of a single gene. Finally in the 1940’s . Drs. Sidney Farber and Harry Shwachman linked the strange discharge of mucus to the disease.
The 1950’s was a decade that marked new advancements in diagnosis techniques of the disease. The most prominent technique was the sweat test developed by DR. Paul di Sant’ Agnese, in New York, during the heat wave of 1953. Along with new diagnosis developments, some of the first Cystic Fibrosis clinics were created in Baltimore, New York, Philadelphia, Boston, and San Francisco. The early clinics, however, were often too late in treating the disease and waited until the patient had severe pain.
To combat these bad treatment methods, in 1955, Dr. Shwachman proposed proper treatment for Cystic Fibrosis, which was early diagnosis, active early treatment, and proper nutrition. During the 1950’s, Dr. Archie Norman, began studying the effect of high fat diets as a treatment for Cystic Fibrosis. Finally, in the 1960’s Cystic Fibrosis awareness rose dramatically and there was the formation of clinics and research facilities to help Cystic Fibrosis. The organizations were formed by the rare CF survivors and the parents of CF victims.
The 1970’s was not a ground breaking decade for Cystic Fibrosis research. However, the fruition of high fat diets to treat the disease was seen. The 1980’s is said to have been the greatest decade in the history of Cystic Fibrosis research. As well as further advancements in treating the illness, the revolutionary discovery of the cystic fibrosis trans-membrane conductance regulator gene was made, by the scientist who would later go on to completing the Human Genome Project, In 1989.
A mutation of this gene directly causes Cystic Fibrosis. Not too long after the discovery of the Cystic Fibrosis gene, gene replacement therapies began as a new treatment method during the 1990’s. Finally during the 1990’s the first drug made to treat Cystic Fibrosis, mucolytic Pulmozyne, was approved by the Food and Drug administration. Research on Cystic Fibrosis continues today and will for years to come as there is currently no cure. Cystic Fibrosis is a horrible and painful disease that affects thousands of people worldwide.
Cystic Fibrosis affects 30,000 and it is one of the most common inherited diseases among Caucasians. About 1,000 new cases of CF are diagnosed each year. (National Heart, Lung, and Blood Institute) The median survival age of Cystic Fibrosis is now thirty seven years old, opposed to five years old fifty years ago. (Cystic Fibrosis Foundation) Advancements in the research of Cystic Fibrosis have been great and many, however, hopefully one day the history of Cystic Fibrosis will include a cure.