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Cystic Fibrosis Life Expectancy

Cystic Fibrosis Life Expectancy

A life-threatening genetic disorder, cystic fibrosis reduces the lifespan of the affected people to a very large extent. Let us take a look at the positive changes in cystic fibrosis life expectancy, brought about by the increasing awareness about the disorder and the advanced treatment options.
Sonia Nair
Last Updated: Mar 12, 2018
Did you know that during the early years of the twentieth century, the average age of survival for kids with cystic fibrosis was six months. Though a cure for cystic fibrosis is still a dream, years of research did yield some positive results. The most important among them is increased longevity. Now, the average survival age is 35 to 40 years, thanks to the advancements in diagnosis and treatment.
One of the most dreaded genetic disorders, cystic fibrosis is most common in Caucasians. As compared to Caucasians, the disease is less common in Hispanics, African-Americans, Native Americans and Asians. In the United States itself, one in 3000 to 4000 babies are born with cystic fibrosis. According to latest statistics, more than 30,000 people in the country, have this condition. However, the disease is highly common in Ireland, where one in 1350 babies born are detected with cystic fibrosis. The most disturbing fact about cystic fibrosis is not its prevalence in certain countries, but is the low survival rate of patients. This genetic disorder has been associated with a short lifespan and life-threatening symptoms. What is the life expectancy of a person, who is affected with cystic fibrosis? Let us find out.
Cystic Fibrosis Survival Rate
Before the 1950s, it was rare for a baby born with this condition, to survive for more than five to six years of age. Over the years, the life expectancy of cystic fibrosis patients has improved a lot. During the 80s, the average lifespan was around 14 to 20 years. As per 2008 statistics, cystic fibrosis life expectancy was 37.4 years. Currently, it is said to be more than 40 years. So, there is a steady rate of improvement, which would have been impossible without the advancement in diagnosis and treatment. However, there are certain factors that are believed to affect the lifespan of cystic fibrosis patients.
  • Pancreatic Function: Normal pancreatic function is a vital factor, that plays a major role in improving the survival rate. Babies affected with cystic fibrosis may live past 50 years of age, if their pancreatic function is normal.
  • Age: The life expectancy of a person born during the 1980s may not be the same for a baby, who is born during the 1990s or the 2000s. As compared to the former, those belonging to the latter category have better treatment options, right from the childhood, which could be advantageous, as far as their lifespan is concerned.
  • Gender: Earlier, male patients used to outlive their female counterparts, for some unknown reasons. Various studies have also come up with similar results. However, nowadays, no drastic difference is noted in the life expectancy of male and female patients.
  • Other Medical Conditions: It has also been claimed that chronicPseudomonas aeruginosa infection may worsen cystic fibrosis symptoms and affect the survival rate negatively. Though allergies are also said to have negative effects, there was only a marginal difference when the lifespan of allergic patients was compared to those with no allergies.
  • Type of Mutation: The severity of symptoms and the survival rate may also vary with different types of mutation. Cystic fibrosis is caused due to mutation in the gene, named CFTR. Mutations that are not so common are said to cause milder symptoms and are often associated with a higher lifespan.
The above said are some of the factors that are believed to affect cystic fibrosis life expectancy. The age of diagnosis, race, nutritional status, pulmonary function, mode of presentation (prominent symptoms) and proper compliance with treatment, etc., are also said to affect the survival rates. As the symptoms tend to worsen during early adulthood, proper treatment during this stage may increase the life expectancy. Though oxygen therapy is believed to improve the health condition as well as the lifespan, studies regarding the same are still underway. It has also been observed that most of the patients, who have opted for lung transplantation are doing well and are said to have an improved life expectancy.
Cystic fibrosis is an inherited disease, which affects the secretory glands of the body, especially the glands that produce mucus and sweat. Symptoms of this disorder include salty sweat, poor growth, excess mucus production, frequent chest infection, incessant cough, breathing trouble, impotence and poor digestion. Those affected with cystic fibrosis produce a sticky mucus that causes blockage of the ducts and passages of the lungs and other organs, like the pancreas, leading to infections and organ failure. Cystic fibrosis was identified and recognized as a special medical condition during the 1930s. The sweat test developed during the 1960s proved to be very much helpful in diagnosing this disease. With such advances in the methods of diagnosis and treatment, the survival rates of cystic fibrosis patients improved a lot. In 1989, the gene, which is responsible for causing the condition was discovered and this discovery became a milestone, based on which, further advancements in treatment was made. Now, the average cystic fibrosis life expectancy is around 40 years, with many patients living much longer than expected.
Nowadays, newborn screening programs in many countries include tests for cystic fibrosis, so that treatment can be started at the earliest. Even prenatal tests can be conducted to rule out the possibility of this disease. Genetic tests can be conducted on the family members of a person, who has been detected with cystic fibrosis. This is done to find out whether he/she carries the gene responsible for this disease. All these diagnostic methods help in early detection, which enables early treatment and a higher rate of cystic fibrosis life expectancy. At present, there is no cure for this disease, but you can get proper treatment, which will help you manage the symptoms in a better way.
Disclaimer: This article is for informational purposes only and should not be used as a replacement for expert medical advice.