Cystic fibrosis is a genetic disorder that affects the respiratory and digestive tract. In this condition, the secretion of very thick and sticky mucus accumulates in the organs and causes damage. Some of the organs that are affected by cystic fibrosis include the liver, the lungs, pancreas, and intestine.

This disorder typically affects the cells that secrete the digestive enzymes, mucus, and sweat. Under normal circumstances, the mucus produced by these cells is thin and flows easily. However, in cystic fibrosis, the secreted mucus is very thick and thus prevents the smooth flow of the mucus. This highly viscous mucus subsequently gets deposited in the airways and various ducts, causing a complete blockage. The higher viscosity also traps pathogens and disease-causing germs and causes inflammation and infections.

Causes
Cystic fibrosis is primarily caused as a result of a defect in the CFTR gene. Here CFTR stands for cystic fibrosis transmembrane conductance regulator and encodes a protein that regulates the movement of the salt and water into the cells. Some of the common causes of cystic fibrosis have been listed as follows:

• A mutation or sudden change in the DNA structure disrupts the normal functioning of the CFTR protein. The CFTR transports the chloride ions to the cell surface. When chloride ions are present on the cell surface, they attract water which makes the mucus thin.
• In cystic fibrosis, the thick mucus is attributed to the hindrance of the above-mentioned mechanism and, consequently, a thick mucus deposits in pancreas, lungs, liver, and the intestines.
• This can cause life-threatening issues such as respiratory infections, breathing issues, and malnutrition.
• The genetic defect that triggers cystic fibrosis is a recessive mutation which means that two defective copies of the same gene should be present in an individual for the manifestation of the disease. If only one copy is present in an individual, he will not develop the disease. However, he will pass on this defective gene to his children and they will become carriers of the disease.

Warning signs
Approximately 1000 people in the country are diagnosed each year with cystic fibrosis. The disorder gets worse as the disease progresses and lung functions decline. Some warning signs for cystic fibrosis are:

• Persistent cough which is dry. In some cases, the individual may cough up the mucus.
• Wheezing that does not respond to asthma treatment
• Breathing issues (shortness of breath) are common warning signs due to blockage of the respiratory tract
• Sinus infections which often recur
• Chronic allergies, which can last throughout the year
• Excessively salty taste of sweat
• Growth retardation and weight loss
• Frequent infections of lungs due to pneumonia
• Difficulty in regular bowel movement.

Cystic fibrosis develops due to a genetic defect in the CFTR gene which then produces a defective transporter protein, leading to the production of highly viscous mucus. Individuals with the disorder live a compromised life and typically require daily care. Affected individuals often face respiratory problems and immediate treatment is required.