Cystic fibrosis

Chest | Pulmonology | Cystic fibrosis (Disease)


Description

Cystic fibrosis (CF) is the most common life-threatening genetic disorder among Caucasians. Firstly it affects the respiratory system (lungs), the digestive system (pancreas and sometimes liver) and the reproductive system.

Cystic fibrosis mostly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.

Causes and Risk factors

Mucus is a substance made by the lining of some body tissues. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. However, if you have cystic fibrosis, your mucus becomes thick and sticky.

The mucus builds up in the lungs and blocks the airways—the tubes that carry air in and out of the lungs. The buildup of mucus makes it easy for bacteria to grow. This leads to repeated, serious lung infections. Over time, these infections can severely damage your lungs.

Children with CF have an abnormality in the function of a cell protein called the cystic fibrosis transmembrane regulator (CFTR). CFTR controls the flow of water and certain salts in and out of the bodys cells. As the movement of salt and water in and out of cells is altered, mucus becomes thickened. The thickened mucus can affect many organs and body systems including:

(1) respiratory - sinuses and lungs

(2) digestive - pancreas, liver, gallbladder, intestines

(3) reproductive - more so in the male, where sperm-carrying ducts become clogged

(4) sweat glands

Common respiratory symptoms of cystic fibrosis include a cough that produces mucus and wheezing. Other symptoms include abdominal swelling, diarrhea, heartburn, and weight loss.

Diagnosis and Treatment

There is no cure for cystic fibrosis, but treatment can ease symptoms and reduce complications. Close monitoring and early, aggressive intervention is recommended. Managing cystic fibrosis is complex, so consider obtaining treatment at a center that specializes in cystic fibrosis.

The goals of treatment include: preventing and controlling lung infections, loosening and removing mucus from the lungs, preventing and treating intestinal blockage, providing adequate nutrition. ...