Cystic fibrosis is a genetic disease that results in abnormal mucus production in the body. DNA differences in cells that line the sweat glands, liver, pancreas, lungs and digestive tract increases the viscosity of the production of mucus. The result of this are the variety of symptoms which are apparent in individuals who suffer from cystic fibrosis. This increase in mucus production also places individuals at an increased risk for a variety of different complications.
Sufferers are at a higher risk for respiratory system complications that include recurrent airway infections, sinusitis, respiratory failure, pneumothorax, emphysema and Staphylococcus infections. The majority of these complications occur because of the deposits of mucus in the pulmonary system which are fertile breeding ground for many bacteria.
Digestive system problems will include pancreas damage, blocked pancreas ducts, malabsorption, pancreatitis, diabetes from the damage to the pancreas, bile duct blockage, liver disease, gallstones and rectal prolapse. Individuals who suffer from cystic fibrosis require a pancreatic enzymes in order to digest the food and absorbed the correct amount of vitamins and nutrients through the intestinal tract.
Without these pancreatic enzymes children and adults are unable to absorb nutrients from their food and suffer from malabsorption. The increased mucus production will also cause blockages in the pancreas, liver and intestines which leads to liver disease, gallstones and rectal prolapse from chronic constipation.
Because these cells which line the reproductive tract in both men and women are affected by the changes from cystic fibrosis, most men are infertile and some women can achieve successful pregnancy through in vitro fertilization. Many women also suffer from menstrual irregularities and abnormal cervical mucus.
Digital clubbing, and enlargement and shiny appearance of the ends of the fingers and toes, is another complication of cystic fibrosis. It happens in nearly all patients who have advanced lung disease and is a function of the decreased oxygen supply to the peripheral areas of the body.
Cor pulmonale is an enlargement of the right side of the heart as a result of a high resistance to blood flow through the lungs caused by the increased mucus production and decreased oxygen transmission. This is a prominent feature in individuals who have advanced lung disease and will lead to heart disease.
Another complication of cystic fibrosis are nasal polyps. These are fleshy growths that occur inside the nose and happen in approximately 15% to 20% of all patients. They often require surgery to reduce them in order to allow nasal breathing.
Individuals who suffer from cystic fibrosis may face several different complications from their disease. However, with increased research and technological advances in treatment protocols individuals are living longer lives with less side effects and complications. It is extremely important for people who suffer from cystic fibrosis to be compliant with their treatment protocols that includes both pancreatic enzymes, pulmonary toilet and antibiotic therapies in order to decrease their risk of complications throughout their lives.
RESOURCES
MayoClinic: Cystic Fibrosis
MedLine Plus: Cystic Fibrosis
Journal of the Royal Society of medicine: Gastrointestinal Complication in Cystic Fibrosis
Respiratory Care: Pulmonary Complication of Cystic Fibrosis
American Journal of Respiratory and Critical Care Medicine: Cystic Fibrosis Pulmonary Guidelines: Complications: Hemoptysis and Pneumothorax