Cystic Fibrosis

 

Graphic: cystic fibrosis

  Cystic fibrosis is the most common cause
of chronic lung disease in children and
young adults, and the most common fatal
hereditary disorder affecting Caucasians
in the US.
   

Cystic fibrosis is an inherited, life threatening disorder caused by a defective gene. The disorder causes thick mucous to form that can block airways in the lungs and ducts within the biliary tract. In pancreatic-related cystic fibrosis, the small ducts within the pancreas are blocked, preventing the release of digestive enzymes. The long-term result may be nutritional and digestive problems, and a build-up of enzymes within the pancreas that can begin to attack the pancreas itself. For more information about cystic fibrosis or to schedule an appointment with a Virginia Mason pancreatic disorder specialist, call (206) 223-2319.

Symptoms of Cystic Fibrosis

Patients with known cystic fibrosis are monitored carefully for respiratory and digestive conditions. Pancreatic-related cystic fibrosis complications can include:

  • Chronic diarrhea (from a lack of enzymes in the small intestine to digest fats)
  • Nutritional deficiencies (from a lack of fat-soluble vitamins)
  • Constipation, gas, swollen abdomen (from a lack of enzymes in the small intestine to digest fats)
  • Chronic pancreatitis (from the build-up of digestive enzymes within the pancreas)

Diagnosing Pancreatic-related Cystic Fibrosis

Your gastroenterologist may suspect a pancreatic disorder based on your medical history and a description of your symptoms. Common tests and procedures available to aid in the diagnosis include blood tests for low levels of pancreatic enzymes and imaging studies of the pancreas.

Treating Pancreatic-related Cystic Fibrosis

Treatment will center on treating chronic pancreatitis that commonly requires multiple treatment regimens during a hospital stay. Diarrhea and nutritional deficiencies are treated with medications, dietary changes, replenishing liquids, nutritional supplements, and oral pancreatic enzymes. As with any chronic medical condition, an emphasis is placed on preventive measures and self monitoring to ensue that life-threatening complications do not arise.