Posted 11/11/2014 3:00 PM (GMT -7)
I posted this back in 2009 a friend with cf made a timeline of how care and treatment and more importantly, knowledge has progressed through the ages. I think it is handy for new parents of children with cf and newly diagnosed adults. In the 28 years since I was born there have been so many advances and improvements in care and treatments and as you can see from this timeline, median age of survival just keeps going up and up and up. It's still a scary disease as it still effects so many things and so much of your life is taken up by treatments, but without those treatments life wasn't nearly as long or as good. Children born today have a much better chance of living a fuller life.
Middle Ages - In medieval folklore, infants with salty skin, a symptom of cystic fibrosis, are considered "bewitched" because they routinely die an early death. "Woe to that child which kissed on the forehead tastes salty. He is bewitched and will soon die." This bit of folklore, handed down through the ages, is an early description of the disease now known as cystic fibrosis. This is one of the earliest references to cystic fibrosis. Folklore from the Middle Ages, from Germany and Switzerland, made this reference. The earliest documented reference in the literature is from 1606, Alonso y de los Ruyzes de Fonteca, professor of medicine in Spain, wrote that it was known that the fingers taste salty after rubbing the forehead of the bewitched child.' This is a clear reference to the fact that children with cystic fibrosis have very salty sweat, due to the abnormal protein manufactured by the cystic fibrosis gene. This protein regulates the movement of sodium and chloride from the body's cells. Sodium and chloride are two components of salt. The sweat glands of a person with cystic fibrosis secrete too much salt, and the person tastes salty. This is also a reference to the fact that in earlier days, people with cystic fibrosis did not live past infancy.
1936 - Fanconi refers to the previously nameless condition as "cystic fibrosis with bronchiectasis."
1938 - Andersen of Columbia University develops the first comprehensive description of cystic fibrosis symptoms. CF also named Mucovicidosis.
1949 - Lowe establishes that cystic fibrosis is a recessive genetic disorder.
1953 - After observing excessive dehydration of cystic fibrosis patients during a New York City heat wave, di Sant' Agnese of Columbia University formally reports to the American Pediatric Society that CF patients secrete excessive amounts of salt in their sweat. This observation leads to development of the sweat test as a diagnostic standard for cystic fibrosis.
1955 - A group of volunteers, including parents of children with CF and physicians meet to form the CF Foundation, headquartered in Philadelphia; among the group is Milton Graub, M.D., a pediatrician and father of two children with CF, who later becomes the CF Foundation’s third president and who remains active in the CF Foundation today; life expectancy for people with CF is 5 years old.
1961 - The CF Foundation establishes the first CF care centers; CF Foundation hosts first conference of CF care center directors.
1962 - Additional CF centers established, bringing total to 30. Predicted median survival increases to about 10 years.
1965 - CF Foundation begins clinical fellowship program; George Frankel, a CF Foundation founder, establishes Guidance, Action and Projection conferences, a fellowship program to bring basic scientists into the field of CF research.
1966 - CF Foundation establishes Patient Data Registry, which tracks the histories of patients treated at CF care centers.
1968 - CF Foundation hosts first National/International Medical Conference.
1972 - First national seminar on needs of young adults with CF held by CF Foundation.
1978 - Headquarters moves to Washington, D.C. to be close to the federal government and the National Institutes of Health (NIH); first nationwide fund-raising event held, called Bowl for Breath, nearly doubling the fund-raising dollars for CF. The number of Cystic Fibrosis Foundation-accredited care centers now total 125.
Early 1980's - Researchers associate organ damage caused by CF with a malfunction of the epithelial tissue.
1980 - Robert J. Beall, Ph.D. comes to the CF Foundation as medical director from NIH.
1981 - Satellite CF care centers for adults were established for the first time; life expectancy reaches 20 years.
1982 - The CF Foundation creates the Research Development Program (RDP) to encourage leading academic and scientific institutions to establish centers of excellence in CF research; Doris Tulcin, Chairman Emeritus of CF Foundation, launches a capital campaign—the first of its kind by a voluntary health agency—to raise $15 million to support the creation of the RDP; Robert K. Dresing becomes volunteer president of the CF Foundation, later becoming the first president and CEO.
1983 - Scientists show that cells lining the lungs fail to properly move chloride into the airways; the Orphan Drug Act is signed into law and provides companies with financial incentives for developing drugs for rare diseases like CF; Frank Deford, sportswriter and novelist, writes the book, Alex: The Life of a Child about his daughter who died from CF at the age of 8; Deford becomes Chairman of the CF Foundation’s Board of Trustees.
1984 - American Airlines, Outstanding Corporate Partner of the CF Foundation, hosts the first Celebrity Ski event to benefit the CF Foundation.
1985 - Life expectancy reaches 25 years.
1986 - The movie, “Alex: The Life of a Child” appears on ABC putting CF in the national spotlight; CF Foundation tops $20 million in fund-raising.
1988 - CF Foundation establishes Cystic Fibrosis Services—a specialty pharmacy—to provide availability, access and assistance with the complex insurance issues faced in obtaining CF medications.
1989 - Francis Collins, M.D., Ph.D., John Riordian, M.D., and Lap-Chee Tsui, Ph.D. discover the CF gene with support from the CF Foundation’s RDP; CF Foundation hosts first GREAT STRIDES walk-a-thon, now a $20 million+ annual fund-raising event. A Team led by Tsui and Riordan of the Hospital for Sick Children in Toronto discover the gene responsible for CF and name its protein product cystic fibrosis transmembrane conductance regulator (CFTR). The gene is mapped to chromosome 7q.
1990 - CF researchers achieve test tube “proof of concept” for gene therapy.
1993 - FDA approves Pulmozyme, the first biotech drug designed for CF, which breaks down thick CF mucus following studies in CF care center network; first gene therapy tested in person with CF—shows gene therapy corrects CF cells in nasal passages; CF Foundation and NIH establish nine gene therapy centers for CF studies; Beall becomes president and CEO of the CF Foundation; The Boomer Esiason Foundation redirects its mission to support the CF Foundation when Esiason's son, Gunnar, is diagnosed with CF.
1996 - The Seattle Breath of Life Gala, which has raised more than $24 million since its inception in 1984, becomes the CF Foundation’s first “million dollar event,” raising $1,002,500 in one night; CF Foundation forms public policy alliance, a grassroots group of volunteers who inform policy decision makers.
1997 - FDA approves TOBI, an inhaled antibiotic, after CF Foundation-supported studies showed safety and efficacy; CF Foundation establishes the Therapeutics Development Program (TDP) with seven centers in the Therapeutics Development Network (TDN); CF Foundation forms Adult Task Force to address specific issues facing adults with CF.
1999 - Bill and Melinda Gates Foundation gives the CF Foundation a $20 million grant for drug discovery; Cam C. Cooper is named Chairman of the CF Foundation’s Board of Trustees.
2000 - CF Foundation-supported researchers complete map of Pseudomonas aeruginosa genome; CF Foundation establishes CF National Bioinformatics Center to help spur discovery of new treatments; CF Foundation awards contract to Aurora Biosciences for up to $46.9 million for CF drug discovery (the largest grant of its kind by a nonprofit organization); CF Foundation establishes Cystic Fibrosis Foundation Therapeutics, Inc. to govern CF Foundation’s drug discovery and evaluation efforts.
2001 - Tom Marsico, CEO of Marsico Capital Management, LLC, commits $25 million over several years in support of the TDP; CF Foundation makes first multi-million award for drug development with Altus® Pharmaceuticals, Inc. to develop TheraClec TM Total, a potentially more efficient pancreatic enzyme.
2002 - Results of CF Foundation-supported clinical trial on azithromycin show that the drug is effective in improving lung function in people with CF; the TDN expands to 18 centers.
2003 - Targeted Genetics, Inc., with CF Foundation support, begins a Phase II CF gene therapy trial that represents the largest and most advanced gene therapy trial for CF to date; CF Foundation tops $150 million in fund-raising; CF Foundation launches Volunteer Leadership Initiative, to bring 2,003 new volunteers to the CF Foundation in 2003.
2004 - Results from Inspire Pharmaceutical’s CF Foundation-supported clinical trial of INS37217 to restore ion transport demonstrate both safety and improvement in lung function in people with CF; pipeline of CF therapeutics in development includes nearly two-dozen potential therapies; CF Foundation recommends nationwide newborn screening for CF at North American CF Conference, which now includes nearly 3,000 attendees; life expectancy
increases to the mid-30s.
2005 - Predicted median age of survival increases to nearly 37 years.
2006 - Cystic Fibrosis Foundation Therapeutics-supported studies in Australia and UNC show that hypertonic saline helps clear CF mucus and becomes a therapeutic option.
2006 - The first compound for CF, discovered through high-throughput screening at Vertex Pharmaceuticals, begins clinical trials.
2006 - Inspire Pharmaceuticals begins Phase 3 clinical trials of its innovative denufosol drug to hydrate and clear CF mucus.
2007 - VX-770, a potentiator candidate developed by Vertex Pharmaceuticals, entered Phase 2 testing. This drug may restore the function of the CFTR protein. Vertex also is working to develop corrector candidates, which aim to improve the function of the CFTR.
Since this timeline was created vertex have created kalydeco to treat 5% of those with cf with good results. It's been a real eye opener of what clever minds and research can come up with. They're now working on another version for those with the most common mutation, df508. I believe it has just been submitted for FDA approval so hopefully we will hear more of this soon. There's lots more research ongoing all the time and it's exciting. I'm not sure CF will ever stand for Cure Found like many hope, there's a lot of genes to convert, not just in the lungs but pancreas, liver, fertility........ But it will keep improving as a better treated disease to a point where one day treatment might be so amazing you might still need to take pills but they keep away infection and make life with cf a lot easier.