Children born and diagnosed with cystic fibrosis (CF) in the United States in 2010 are expected to live longer than those born earlier, according to a study being published in Annals of Internal Medicine.
CF is a life-shortening genetic disease that causes progressive damage to the lungs and other organs. In 1950, children born with CF did not live to attend elementary school. In 1966, the Cystic Fibrosis Foundation Patient Registry (CFFPR) was established. By 2010, almost half of the 26,000 living people on the CFFPR were aged 18 or older, due largely to advances in pulmonary and nutritional therapies. An updated assessment of survival is important to patients and families who want to prepare for the future. In addition, this information can help plan for the health care needs of an increasing number of patients with CF living to adulthood. Researchers reviewed the CFFPR to assess trends in survival between 2000 and 2010 and to project survival for children born and diagnosed with CF in 2010. The analysis shows that CF survival improved from 2000 to 2010 at a rate of 1.8 percent per year. The researchers project that children born and diagnosed with CF in 2010 can expect to live to about 40 years if the survival rate does not change and to 56 years if the rate continues to decrease at the same rate observed between 2000 and 2010.
The authors of an accompanying editorial discuss the challenges associated with a longer lifespan for CF patients. They write that "caring for adults with CF takes a village." Maintaining the model of care that enabled CF patients to increase their lifespan to this point will require substantial resources that a single nongovernmental organization may no longer be able to provide on its own.
Study 1: Longevity of Patients With Cystic Fibrosis in 2000 to 2010 and Beyond: Survival Analysis of the Cystic Fibrosis Foundation Patient Registry, T. MacKenzie, A.H. Gifford, K.A. Sabadosa, H.B. Quinton, E.A. Knapp, C.H. Goss, and B.C. Marshall, Annals of Internal Medicine, doi: 10.7326/M13-0636, published 18 August 2014.
Study 2: Cystic Fibrosis: Recent Successes Present New Challenges, J.M.Pilewski, and D.B.Taichman, Annals of Internal Medicine, doi: 10.7326/M14-1534, published 18 August 2014.