Cystic Fibrosis: An Inherited Disorder

According to the Mayo Clinic, cystic fibrosis (CF) is an “inherited disorder that causes damage to the lungs, digestive system and other organs in the body”. CF affects the mucus-producing cells, which secrete fluids, such as sweat and digestive juices, that are normally thin and slippery. In people with CF, the mucus becomes sticky and thick, and which may plug up ducts and passageways, especially in the lungs and pancreas. Children with CF inherit one copy of a mutated gene that causes CF from each parent. If children only inherit one copy of the gene, they will not develop CF. However, they will be carriers and could pass the gene to their own children.

The signs and symptoms of CF may vary, depending on the severity of the disease. They may worsen or improve as time passes, even in the same person. Some people with CF may not experience symptoms until they are teenagers or adults. Cystic fibrosis is progressive and requires daily care. Thanks to improvements in screening and treatment, people with CF may live into their mid- to late 30s or 40s.

To diagnose cystic fibrosis, your doctor may do a physical exam, review your symptoms, and may conduct several tests. Newborn babies are now routinely screened for CF. An early diagnosis means that treatment can begin immediately. CF tests may be recommended for children and adults who were not screened at birth. Your doctor may suggest genetic and sweat tests for cystic fibrosis if you have recurring bouts of an inflamed pancreas, nasal polyps, chronic sinus or lung infections, or bronchiectasis.

Currently, there is no cure for CF. However, treatment can ease symptoms, reduce complications, and improve a person’s quality of life. Most recently, the Food and Drug Administration approved the “triple therapy” for patients 12 and older which can help more than 90% of CF patients effectively manage their symptoms. Managing cystic fibrosis is complex. People with CF may consider getting treatment at a center with a multispecialty team of doctors and other medical professionals that are trained in CF to evaluate and treat their condition. Treatments for CF include medications, airway clearing techniques, pulmonary rehabilitation, surgery, and other treatments.

NARIC’s information specialists searched our collection and found over 700 articles on various aspects of CF research and development from the NIDILRR community and beyond. If you are a person with CF, a parent of a child with CF, a caregiver, service provider, or other stakeholder, contact NARIC’s information specialists to learn more about cystic fibrosis.

Please note: If you think that you may have CF, please contact your doctor. If you think your child may have CF, please contact their pediatrician.

About mpgarcia

I'm the Bilingual Information/Media Specialist at NARIC.
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