Who has it?

Throughout the world, approximately 70,000 children and adults suffer from Cystic Fibrosis.  Almost half of these cases (30,000) are within the United States.  Both males and females of all races and ethnicities are at risk, but CF is most prevalent in Caucasians of Northern European descent.   About 1,000 new cases are diagnosed in the US each year.  Historically, about half of the CF population is diagnosed by five months of age, and over 75% are diagnosed before their second birthday.  However, some people with less severe cases have been diagnosed well into adulthood.  Diagnosis has been evolving over the past few years, and now all states screen newborns for cystic fibrosis within a few days of birth.  The screening is not 100% accurate, and produces many false positive.  False positives occur when a healthy baby is tested for a disease and the results indicate that the baby has the disease.  To find out if the results were accurate or not, all newborns who test positive for CF go through a follow-up test that is more accurate.  This secondary screening rules out CF in over half of those who tested positive in the initial newborn screening.  Because of the recent implementation of newborn screening, more cases are being confirmed at an early age, improving treatment outcomes and documentation of CF epidemiology. 

It is believed that CF susceptibility is entirely genetic.  However, the severity of the illness is affected by many environmental factors including air quality, exposure to bacteria, access to medical care and age of diagnosis and treatment.  These factors, along with genetics and others health conditions determine the lifespan of each CF patient.  There is no cure for the disease, but people with CF who maintain successful treatment regimens can live into their fifties with reasonably high quality of life.  This possibility is a thrilling advancement in comparison with CF outcomes in 20 years ago.  CF was considered a childhood disease in recent years because so few patients lived into adulthood.  Today almost half of those with CF are 18 or older.  According to a recent article published by BMC Pulmonary Medicine, this improvement is due to earlier diagnosis and follow-up treatment over the past two decades.  The results point toward newborn screening as a major indicator in disease outcome.







Citations

Cystic Fibrosis. http://www.nhlbi.nih.gov/health/health-topics/topics/cf.  Published December 26, 2013.  Accessed January 16, 2015.

Marson FA, Hortencio TD, Aguiar KC, Ribeiro JD. Demographic, clinical, and laboratory parameters of cystic fibrosis during the last two decades: a comparative analysis. BMC Pulmonary Medicine. 2015; 15(3). doi:10.1186/1471-2466-15-3 

O’Sullivan BP, Freedman SD. Cystic fibrosis. Lancet 2009;373:1891–1904.