Cystic fibrosis

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Cystic fibrosis (CF) is a hereditary disease whose symptoms usually appear shortly after birth. They include faulty digestion, breathing difficulties, respiratory infections due to mucus accumulation, and excessive loss of salt in sweat. These symptoms arise due to a genetic mutation in a membrane bound chloride ion channel. In the past, cystic fibrosis was almost always fatal in childhood, but treatment is now so improved that patients commonly live to their 20s and beyond. The average person with Cystic fibrosis lives to age 37.[1]

Effects and Symptoms

Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky. The abnormal mucus builds up in the patient's lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated life-threatening lung infections. Over time, these infections can cause serious damage to the lungs.

The mucus can also block tubes, or ducts, in the pancreas. As a result, the digestive enzymes produced by the pancreas cannot reach the small intestine. These enzymes help break down food, allowing this body to absorb it. Without these enzymes, the intestines cannot fully absorb fats and proteins.

As a result:

  • Nutrients leave the body unused, leading to malnourishment.
  • The stools become bulky.
  • The body may lack vitamins A, D, E, and K.
  • The patient may suffer from intestinal gas, a swollen belly, and pain or discomfort.

Due to the body's inability to fully digest food, a key symptom of cystic fibrosis is difficulty growing and/or gaining weight despite a healthy appetite.

The abnormal gene which causes cystic fibrosis also causes the sweat to become extremely salty. As a result, when a person with cystic fibrosis perspires, his or her body loses large amounts of salt. This can upset the balance of minerals in the blood. The imbalance may cause a heat emergency.

CF can also cause infertility (mostly in men).

The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild versions of the disease that doesn't show up until they are adolescents or young adults.

Respiratory failure is the most common cause of death in people with CF.

Diagnosis and Treatment

Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old. 40% of people with CF are over age 18.[2]

Around 1,000 new cases of CF are diagnosed each year, and 70% of those diagnosed are age two or younger.[3]

Treatment of CF includes special diets high in fat and calories, treatment to prevent mucus from clogging the lungs, and possibly drugs to thin mucus.[4] Patients with CF are careful to avoid germs, in order to lower the risk of a lung infection. Because of scarring in the pancreas, CF patients are likely to develop diabetes later in life. For this reason, CF patients avoid eating too much sugar. Second-hand smoke can be harmful to CF patients, and it is best that people with CF avoid smoking environments.

Mucus in the lungs can be treated through drugs inhaled with a nebulizer.[5]

Research continues to look for:

  • Better treatments
  • A cure