Cystic fibrosis, or CF, is an inherited genetic condition common in people of Northern European and Ashkenazi Jewish ancestry. CF causes a person to have thicker secretions than average, primarily affecting the lungs, pancreas and gastrointestinal system. Progressive lung disease, frequent respiratory infections and other complications often place a child with CF in the hospital. Daily treatments with inhaled and oral medications along with other therapies are needed to help relieve and manage symptoms.
Frequent Respiratory Infections
Surfactant is fluid lining the lungs that helps oxygen and carbon dioxide move in and out of the lung tissue. This fluid is thick in people with cystic fibrosis, reducing the ability to bring in oxygen. In addition, this thicker surfactant traps bacteria, causing frequent respiratory infections. Most children with CF constantly have certain types of bacteria in their lungs. Antibiotics help control these infections, but it is not uncommon for serious respiratory infections to occur. As a result, hospitalizations to treat pneumonia and other lung infections are a common occurrence in children with CF.
Chronic Diarrhea
Due to problems with the pancreas and thick mucus in the gastrointestinal tract, frequent diarrhea is common in individuals with cystic fibrosis. There are other reasons, such as lactose intolerance, that may cause chronic diarrhea. However, frequent diarrhea in conjunction with other symptoms of CF makes the diagnosis more likely. This is especially true if the stool is greasy or has an unusually strong smell. Globs of fat may also be present in the stool.
Chronic Pancreatitis
Pancreatitis is an infection of the pancreas. Symptoms include abdominal pain, chills, weight loss, nausea, vomiting and fever. Children with cystic fibrosis usually have recurrences of pancreatitis, another cause for hospitalizations. These infections can cause scar tissue to build up in the pancreas, which can interfere with the pancreas' normal function and lead to more complications, such as diabetes, as the child gets older.
Vitamin Deficiency
Ongoing deficiency of fat soluble vitamins, such as vitamins A, E, D and K, can cause complications such as high blood pressure, anemia and a skin rash called acrodermatitis. These vitamins are particularly hard for people with cystic fibrosis to absorb, even when a child has been prescribed supplemental pancreatic enzymes to help digest food. To help prevent vitamin deficiency, children with CF should take supplements high in vitamin A, E, D and K every day.
Intestinal Obstruction Syndrome
As a child with cystic fibrosis ages, thickened stools can block his intestines. Poor absorption of food, thick intestinal mucus and decreased movement of the intestinal tract leads to intestinal obstruction syndrome. This symptom is similar to meconium ileus, which occurs shortly after birth in about 20 percent of infants with CF. Symptoms include abdominal pain, vomiting and bloating. Intestinal obstruction syndrome often requires surgery to remove the block.
References
- The Lancet: Cystic Fibrosis
- "Thompson & Thompson Genetics in Metabolism (7th Edition)"; Robert Nessbaum; 2007
