Understanding What Cystic Fibrosis Is

To better understand how cystic fibrosis affects the body, we need to look at the cause of the disease. Cystic fibrosis is brought about by a faulty cystic fibrosis transmembrane conductance regulator, or simply CFTR gene. This particular gene, in normal conditions, regulates the passage of chloride ions and water in and out of the body’s cells. In patients with CF, the gene is not functioning effectively. Consequently, thick, sticky mucus is produced in the lungs and pancreas.

Each person normally inherits two CFTR genes, one from each parent. When a child inherits an abnormal CFTR gene from each parent, the child will have cystic fibrosis. On the other hand, if a child receives an abnormal CFTR gene from one parent and a normal CFTR from the other parent, the child will not have cystic fibrosis, instead, the child becomes a CF carrier. Although carriers usually have no symptoms of the disease, they can, however, pass the abnormal gene to their children.

Lung disorder caused by cystic fibrosis

The lungs are among the organs chiefly affected by cystic fibrosis. Consistency of mucus in a healthy lung is watery, thus keeping the lungs and air passages moist. However, in people with CF, the texture of the mucus becomes thick and sticky. Breathing becomes more difficult as airways are blocked by mucus build-up. More so, it becomes a favorable breeding area for bacteria which results to seriously recurring lung infections. These infections can lead to lung scarring. Severely damaged lung tissues often lead to respiratory failure, the most common cause of death in CF patients.

Pancreatic disorder and cystic fibrosis

Thick mucus secretions are also the problem in the pancreatic ducts of CF patients. The pancreatic ducts are blocked with thick, sticky mucus and the digestive enzymes made by the pancreas do not reach the area of the small intestines. These enzymes are vital for breaking down food nutrients so it can be easily absorbed in the intestines. In the absence of these enzymes, the intestines are not able to absorb fats and proteins needed by the body. This then leads to nutrient deficiencies in CF patients, which also makes them weaker and more prone to infections.

Getting information on what is cystic fibrosis can prepare parents on how they can provide adequate support for their child. A good doctor, medication and physical treatment will have a positive impact on a patient’s life.

By: Thesa Sambas

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