Cystic Fibrosis

Meaning

A genetic disease that damages the lungs and other parts of the body.

Background

According to the Cystic Fibrosis Foundation, cystic fibrosis causes the mucus in some organs to become "thick and sticky." For instance, in the pancreas, the mucus can cause malnutrition and insufficient growth, and in the liver, the mucus can block the bile duct and cause lung disease. When the mucus builds up in the lungs, it can clog airways and trap bacteria, which causes various complications such as infections and inflammations.

Symptoms of the disease vary from person to person, and may include wheezing, persistent coughing, frequent lung infections, and poor weight gain or growth, among others.

The Cystic Fibrosis Foundation stated that while the disease is progressive and has no cure, treatments and care exist to improve the lives of people living with cystic fibrosis. It has found that quality care advancements "have added years and quality of life to the lives of people with cystic fibrosis... [and] many live long enough to realize their dreams of attending college, pursuing careers, getting married, and having kids."

According to the Cystic Fibrosis Foundatoin, treatments include inhaled medicines, pancreatic enzyme supplements, and individualized fitness plans. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators can boost the CFTR gene-created proteins in people with cystic fibrosis who have certain mutations of the CFTR gene. In 2019, the FDA approved a triple drug combination therapy named Trikafta, which helps people with certain CFTR gene mutations make their CFTR gene-created proteins work better. The therapy can treat people with the most common cystic fibrosis mutation, one that an estimated 90% of people with cystic fibrosis possess.

Cystic fibrosis is covered under the Americans with Disabilities Act. Its Title III Regulations, sec. 36.104, include respiratory conditions that "substantially limit one or more of the major life activities" of people under its definition of physical impairments that constitute a disability.

Additional resources:

Comments