Cystic fibrosis is an inherited condition that causes severe damage to the cells that produce mucus, sweat and digestive juices, resulting in severe damage to the lungs and the digestive system. It is most common in white people with Northern European ancestry, but is also found in African Americans, Hispanics and Native Americans. It is rare in Asians and Middle Eastern people. Better screening and improved treatments for the symptoms of cystic fibrosis now allows people with the condition to enjoy a better quality of life than in the past. Most people with CF live into their 20s and 30s, and some are living into their 40s and 50s.

Symptoms and Complications

The characteristic symptoms of cystic fibrosis may appear as early as infancy or as late as young adulthood. They vary in severity, even in the same person. Today, newborns are routinely screened for cystic fibrosis in the nursery, so parents are aware they have the condition before symptoms develop and can begin treatment immediately. However, it’s still important to be aware of the symptoms for those people who were born before the newborn screening was widespread. The symptoms and signs of cystic fibrosis include:

* High salt levels in perspiration: to the point where parents can taste the salt when they kiss their children

* Persistent cough: that produces thick spit and mucus

* Wheezing

* Breathlessness

* Repeated lung infections

* Inflamed nasal passages

* Decrease in ability to exercise

* Foul-smelling, greasy stools

* Poor weight gain

* Intestinal blockage

* Severe constipation

* Rectal prolapse: caused by frequent straining to pass stool

Risk Factors and Causes

Cystic fibrosis is an inherited disease that is caused by a mutation in a specific gene. The mutation changes a protein that regulates the movement of salt in and out of the cell, resulting in thick, sticky mucus in the lungs, digestive system and reproductive system.

Treatments

There is currently no known cure for cystic fibrosis. Treatment aims to reduce symptoms and complications of the disease. Managing cystic fibrosis effectively requires a combination of drugs, chest physical therapy, pulmonary rehabilitation and, in some cases, surgery. In addition, there are a number of alternative therapies that may be helpful for children and adults with cystic fibrosis. Emerging therapies for cystic fibrosis include stem cell therapy, gene therapy and CFTR modulation.