Cystic Fibrosis Median Survival Rate Has Quadrupled Since the 60s

Those with cystic fibrosis and their families have reason to celebrate.

Recent data from the United Kingdom reveals a 60% increase in people diagnosed with cystic fibrosis (CF), but they also project that 50% of those newly born with CF are expected to reach a median age of 47.

This information was published by the U.K Cystic Fibrosis Trust and reflects statistics gathered from England, Scotland, and Wales. The group attributes their success in uncovering these findings to "improvement in the documentation process and upgraded treatment and follow-up practices."

There is reason to celebrate for those cystic fibrosis along with their family members since, as per recent data, it was revealed that, although there has been an increase in the number of individuals diagnosed with CF, at the same time, it is also projected that 50 percent of those newly born with CF will live until at least the median age of 47 years. The success at uncovering these various findings is attributed to new improvements in the proper documentation procedure as well as an upgrade in the available treatment options, accompanied by proper follow-up practices. The current life span of individuals with CF is about 37 years of age. The predicted survival rate used to be about ten years, wherein a percentage of patients were expected to survive only into their teen years. From this new data, it is obvious there has been a great advancement in terms of CF treatment.

CF is a life-threatening genetic disorder that is also classified as an autosomal recessive disorder. Such disorders are known to pass on their mutated genes. In the case of CF, it is said that both biological parents are carriers of the mutated CF gene or already suffer from CF themselves when they conceive children who develop this condition. One of the hallmarks of CF is the presence of gelatin-like mucus found in the respiratory tract as well as a few other organs, including the digestive tract, or issues pertaining to the digestive system, leading to damage of the cells in the pancreas. In 1930, the first diagnosis of this disease as a medical condition was recorded, and a decade later it was identified as a hereditary disease.

The various studies and successful attempts being made in the diagnosis and treatment of CF in the past half century are very encouraging for everyone involved. The researchers have identified the role of the CFTR genetic protein, which is to regulate the passage of salt and water across cell membranes. A defect in the CFTR can prevent this procedure from happening, resulting in the build-up of thick, sticky mucus in the body. For most individuals with CF, the mucus blocks as well as damages the ducts of the pancreas, which prevents the organs from manufacturing the pancreatic enzymes that are necessary for food digestion. In the case of newborns, it can lead to a fatal case of intestinal blockage.

The Cystic Fibrosis Foundation has been actively involved in funding this promising research as well as any kind of advancement towards specialized care and more effective treatment options. Most of the CF drugs currently available were made possible thanks to this foundation. The organization also provides free genetic testing for those with CF, as well as helps in providing early-stage funding for numerous ongoing studies for these and newer drugs.