Reviewed by Jerry A. Nick, MD

While humans have certainly died from CF for thousands of years, the first clear references to the disease extend back only a few centuries. European folklore from the Middle Ages warned “woe is the child who tastes salty from a kiss on the brow, for he is cursed, and soon must die”. References have been found in medical texts as early as 1595 that linked salty skin and damage to the pancreas with death in childhood by infants who were “hexed” or “bewitched” [2]. Scholars have proposed that the Polish composer Frederic Chopin (1810-1849), who died of respiratory failure after a lifetime of malabsorption and lung infection, likely had a mild form of CF. In 1938, the American Pathologist Dr. Dorothy Andersen provided the first description of the disorder in the medical literature, calling the disease “cystic fibrosis of the pancreas” based on her autopsy findings of children that died of malnutrition. Other physicians of the era referred to the disease as “mucoviscidosis”, which called attention to the presence of thickened mucous.

The modern history of CF is dominated by the definition of the underlying genetic defect, and the rapid increase in survival following the introduction of improved therapies. During a heat wave in the summer of 1948, Dr. Paul di Sant’Agnese observed infants presenting with dehydration to a New York City emergency room. This lead to his discovery that the sweat of children with CF had abnormally high concentrations of salt, and validated the ancient folklore of the disease. In the 1980s, the protein defect was described, and in 1989 the responsible gene (CFTR) was identified and its genetic code was sequenced. With each decade, new therapies have been introduced, leading to a dramatic increase in survival.

In recent years, public awareness of CF has risen dramatically. In the 1950s and 1960s, a variety of organizations were formed worldwide, in part to educate patients, families, and the public about CF. In particular, the CF Foundation (United States) has played a significant role in developing the current model of CF care, as well as providing financial support for much of the current CF-related research and drug discovery. More recently, public attention has been drawn to individuals with CF who are the children (or close relatives) of celebrities, or who have achieved fame in their own right.


Notable People With CF

  • Frankie Abernathy (1981–2007), television personality (U.S.)
  • Lisa Bentley (1968-), Ironman triathlete (Canada)
  • Ricky Briggs (1981-), actor and comedian (U.S.)
  • Christopher Davies (1978-), cricketer (Australia)
  • Oliver Dillion (1998-), actor (U.K.)
  • Bob Flanagan (1952–1996), writer, performance artist, and comedian (U.S.)
  • Chris Fowler (1975-), race car driver (U.S.)
  • Nolan Gottlieb (1982-), basketball player and coach (U.S.)
  • Grégory Lemarchal (1983-2007),sSinger (France)
  • Alice Martineau (1972–2003), singer-songwriter and model (U.K.)
  • Kimberly Myers (1970-97), race car driver (U.S.)
  • Elizabeth Nash, Ph.D., scientist and CF researcher (U.S.)
  • Laura Rothenberg (1981–2003), author (U.S.)
  • Andrew Simmons (1984-), professional wrestler (U.K.)
  • Bill Williams (1960–1998), author and software developer (U.S.)

Notable People With Family Members Who Have CF

  • Gordon Brown, Prime Minister (U.K.), son Fraser
  • Tammy Cochran, singer (U.S.), brothers Shawn and Alan
  • Frank Deford, author and journalist (U.S.), daughter Alexandra
  • Celine Dion, singer (Canada), niece Karine
  • Boomer Esiason, football player (U.S.), son Gunnar   
  • Brian Hill, basketball coach (U.S.), daughter Kim
  • Rosie O’Donnell, comedian (U.S.), nephew Joey
  • Ken Read, skier (Canada), nephew Andrew

Many high quality websites and weblogs are written by people with CF, or by members of their family, and this work has done much to document the wide variety of perspectives and experiences encountered by individuals of varying ages and backgrounds with the disease worldwide.

Clinical Trials

Impact of Combination Therapy on Cystic Fibrosis (TEACH Study)

The purpose of this study is to determine how two medications, azithromycin and inhaled tobramycin, work when taken together to treat cystic fibrosis (CF) patients with chronic pseudomonas aeruginosa (PA) lung infections.

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