Cystic fibrosis (CF) is a chronic, inherited disease that affects the secretory glands, which are responsible for the production of mucus and sweat. In healthy people, mucus is a watery and slippery substance that moistens the lining of the organs and prevents dehydration or infections. In CF patients, however, the mucus is thick and sticky, adversely affecting the lungs, pancreas, liver, intestines, sinuses, and reproductive organs. The mucus ends up accumulating in the lungs and airways, making it easier for bacteria to grow and infection to set in.
CF patients experience frequent difficulties breathing and recurrent infections. In addition, patients often experience other respiratory symptoms like persistent coughing, wheezing, exercise intolerance, and inflamed nasal passages or a stuffy nose. Digestive symptoms including foul-smelling and greasy stools, poor weight gain and growth, intestinal blockage, particularly in newborns (meconium ileus), and severe constipation are among many other symptoms.
Current CF Life Expectancy in the US
The symptoms and severity of the disease vary according to the genetic defect that caused it, as well as patients’ age and other existent conditions. Similarly, the symptoms are not constant and differ over time. There is currently no cure for CF, but research leads to new medications that helps patients improve their quality of life and life expectancy. According to the National Institutes of Health (NIH), the current average life expectancy for CF patients in the United States who live past childhood is about 37 years.
The majority of pediatric CF patients are able to manage symptoms and maintain quality of life such as taking part in school and other activities. In fact, many young adults with cystic fibrosis manage to finish college and find jobs. Despite the tendency for life expectancy in CF patients to increase, lung disease continues to be common and gets worse over time, which eventually disables the patient. Death is most often caused by lung complications. Though life expectancy continues to be globally uneven, with the U.S. where CF patients tend to live longer, the NIH believes that current research will lead to improvements in treatment and life expectancy worldwide.
Evolution of Cystic Fibrosis Life Expectancy
A cystic fibrosis diagnosis changes the life of any family, treatment for the disease has greatly changed in recent decades. Children who are born today with CF have a completely different prognosis from those born more than 30 years ago.
While the diagnosis was already possible through a sweat test developed in 1959, there were no methods of treatment and the mucus would rapidly accumulate in the lungs and digestive tracts of CF patients. There were also no effective antibiotics for the treatment of infections and the digestive and pancreatic manifestations of the disease frequently resulted in delayed growth and malnutrition.
“In 1962, the predicted median survival for CF patients was about 10 years, with few surviving into their teenage years,” according to the NIH.
From a fact sheet published online, the NIH maintains: NIH researchers found that the underlying problem for patients with CF was a defect in salt transport through their cell membranes. The CF transmembrane conductance regulator (CFTR) gene, which is defective in people with CF, was discovered in 1989. Carrier detection and prenatal diagnosis of CF were developed for families with a history of the disease. Mouse models of the disease were developed in the early 1990s, but unlike humans, mice without CFTR did not exhibit typical symptoms of CF, including the lung and liver disease that cause the premature mortality seen in humans.
Research achievements in the study of cystic fibrosis resulted in the quadruplication of median life expectancy in the country, but in countries where medicine is not advanced, life expectancy remains much lower. In the majority of the U.S. states, newborn screening for CF is provided even without the presence of symptoms.
Today, antibodies exist to fight infections; other medications slow the progression of lung disease; and new mechanical chest physical therapy devices loosen the mucus and make it easier for patients to clear the lungs. Lung transplant has also become an alternative for the treatment of CF patients with severe lung damage.
Note: Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.