Cystic Fibrosis

Signs and Symptoms
CF interferes with electrolyte and fluid balances in the body. In most CF patients, sweat is up to five times saltier than normal and severe salt depletion can occur in very hot weather.

Much of the disease associated with CF is due to chest infections and lung complications. From early childhood the abnormal composition of the fluid lining the airways makes them prone to infection. The lubricating mucus in the lungs becomes thick and sticky, leading to chronic infections, severe inflammation and eventual lung damage. However, with early diagnosis through neonatal screening, early recognition of infection and aggressive treatment of the infections using oral, inhaled and intravenous antibiotics, chronic infection can be delayed or even prevented in many of those affected.

The excessively viscid secretion lining the pancreatic ducts leads to obstruction and extensive destruction of pancreatic tissue. The resulting deficiency of pancreatic enzymes and bicarbonate causes severe intestinal malabsorption of fat and protein and fat-soluble vitamins with foul-smelling, greasy stools. Nutritional support with oral high energy supplements, pancreatic enzyme replacement therapy and supplements of fat-soluble vitamins A, D, E and K can, in many patients, achieve near normal absorption, nutritional state and growth. So most patients now survive until they are over 35, but many develop diabetes mellitus, and some have osteoporosis.

Other problems and symptoms associated with CF include:

• Meconium ileus (no stools in the first 24 to 48 hours of life) – intestinal obstruction due to viscid bowel contents, affecting some 15% of newborns with CF
• Jaundice due to biliary cirrhosis caused by blocked bile ducts in the liver
• Inadequate weight gain, suboptimal growth, delayed puberty and eventual below average stature due to intestinal malabsorption
• Recurrent pancreatitis;
• Growths (polyps) in the nasal passages
• Enlargement or rounding (clubbing) of the fingertips and toes due to a chronic reduced oxygen environment from lung involvement
• Rectal prolapse (protrusion of the rectum through the anus)
• Male infertility