What is Cystic Fibrosis?

Cystic fibrosis is a life-threatening, inherited disorder, which affects the secretory glands producing mucus and sweat. Fluids secreted by the glands are usually thin. In this disease, the secretions become thick and sticky and block all passageways and ducts, particularly of the lungs and pancreas. The disease results in the accumulation of mucus, which blocks the lungs and air passages. The build up of mucus in the respiratory system allows the growth of micro organisms leading to frequent infections. The build up of secretory fluid in the digestive system can prevent the digestive enzymes from acting on food. Another characteristic of this disease is the presence of excess salt in sweat. This results in an imbalance of minerals in the blood, leading to dehydration, low blood pressure, fatigue, stroke, and weakness.

Cystic fibrosis is caused by the presence of two abnormal genes inherited from one's parents. Children with this disease often inherit one abnormal gene from each parent. This happens when both parents are carriers of the gene. Both men and women are equally affected by the disease, and have equal chances of getting it.

The symptoms of this disease vary depending on the individual. The frequency and severity of symptoms also changes over time.

The most common symptoms of cystic fibrosis include:

• Extra salt in sweat
• Cough with phlegm that continues for a long period
• Frequent infections in the lungs
• Difficulty in breathing
• Wheezing
• Presence of nasal polyps
• Repeated bouts of sinusitis and bronchitis
• Constipation
• Loss of weight
• Persistent diarrhea
• Low bone density

As the disease progresses, inflammation of pancreas, liver disease, diabetes, and gallstones becomes common. One should get medical attention if there are repeated infections of the lungs and sinuses, if the stools are fatty and foul smelling, and if the child has difficulty in breathing.

There is no complete cure for this disease. Most of the available treatments help to control and prevent the symptoms of the disease, including lung infections, accumulation of mucus, and digestive issues.

The most common treatment options for cystic fibrosis include:

• Drugs to prevent mucus build up
• Antibiotics to treat infections
• Anti-inflammatory medications to prevent inflammation of lungs
• Physiotherapy to reduce mucus accumulation

Digestive issues are treated with another set of options that include:

• Vitamin supplements
• Oral pancreatic enzymes
• Using feeding tubes to improve the intake of calories