Cystic Fibrosis – What you may want to know…


More than 30,000 young adults and children in the United States suffer from cystic fibrosis (CF).  It affects the lungs and breathing and disrupts the normal function of the epithelial cells, causing those with the disease to become sick.  Therefore, children with cystic fibrosis often find it difficult to participate in strenuous activities even if they are very active. Understanding this disease and its effects can help parents, teachers, and children better cope with it. This guide will explore cystic fibrosis, what causes it, and the symptoms and treatments.

What is Cystic Fibrosis?

Cystic fibrosis is a genetic disorder that affects the digestive system and lungs. It causes children to be more likely to have infections in their lungs repeatedly. This happens because there is a disruption in the normal function of the body’s epithelial cells.  These cells line passageways in the liver, lungs, pancreas, reproductive, and digestive systems. The skin’s sweat glands are also made up of epithelial cells.  Patients with cystic fibrosis continuously have an excessive buildup of mucus in the lungs and passageways.

The Dangers of Cystic Fibrosis

Cystic fibrosis poses a serious threat to patients because of the excessive mucus buildup in the lungs. The mucus is very thick and can create the perfect environment for bacteria to develop and grow, especially pseudomonas aeruginosa, which can cause severe life-threatening infections or nosocomial infections in persons with cystic fibrosis.  A cystic fibrosis sufferer has defective CFTRs (cell membranes), and their immune system is unable to offer proper immune response to fight such infections.  Therefore, these infections can be deadly.

A baby with cystic fibrosis usually gets this infection by the age of 3.  By the age of 10, the infection has become chronic in many patients.  Many cystic fibrosis sufferers do not live to be in their 40s.

Another danger of cystic fibrosis is maldigestion due to a poor-working pancreas. This can lead to symptoms of steatorrhea and malabsorption.  The stools will become greasy and large, and may even have visual droplets of fat.  With this condition, the patient might have a difficult time gaining weight.

Categorized as IACFA