What are the Symptoms of Cystic Fibrosis?

Cystic Fibrosis causes the body to produce thick secretions that particularly affect the lungs and digestive tract.

• Lungs
• Symptoms include a persistent cough, repeated chest infections, prolonged diarrhea and poor weight gain. These symptoms are not unique to Cystic Fibrosis.
• Digestive system
  

Cystic Fibrosis affects the pancreas, which makes it difficult for people with CF to digest food. This can cause malnutrition, which can lead to poor growth and delayed puberty. In older patients, insulin production can become deficient due to increasing pancreatic tissue damage. Some develop CF related diabetes mellitus and their blood sugar levels are no longer controlled. This rarely happens to children with Cystic Fibrosis.

• Common symptoms of diabetes include thirst, hunger, weight loss and excessive need to urinate.
• Other Affected Organs
• People with CF develop thin, brittle bone due to the nutritional and other problems involved with the disease. Bone disease in adults is because of the adverse effects of steroids taken to control lung disease.
• In most men with CF, the tubes that carry sperm are blocked, which causes infertility. Because underweight women are more likely to have irregular menstrual cycles, the nutritional problems associated with CF may affect fertility. Women with CF do produce healthy, fertile eggs so effective contraception is necessary. Cystic Fibrosis does not cause sexual impotency.
• Cystic Fibrosiscan cause the blockage of small ducts in the liver. This only happens to approximately 8% of people who have CF, but it is a serious health risk and may require liver transplantation.
  

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Cystic Fibrosis in Children:

The diagnosis of Cystic Fibrosis is being made earlier and earlier, usually in infancy. However, about 15% of those with CF are diagnosed later in life (even adulthood). Symptoms involve the lungs and digestive organs and vary in severity.

Some kids with CF have symptoms at birth. Some are born with a condition called meconium ileus. Meconium  is the thick, dark, putty-like substance that usually passes from the rectum in the first few days of life. In CF, the meconium can be too thick and sticky to pass and can completely block the intestines. Poor fat absorption makes the stools appear oily and bulky and increases the child’s risk for deficiencies of the fat-soluble vitamins (vitamins A, D, E, and K). Unabsorbed fats also cause excessive intestinal gas, an abnormally swollen belly, and abdominal pain or discomfort.

Babies born with CF don’t gain weight as expected. They fail to flourish in spite of a normal diet and a good appetite. Mucus blocks the passageways of the pancreas and prevents pancreatic digestive juices from entering the intestines. Without these digestive juices, the intestines can’t absorb fats and proteins completely, so nutrients pass out of the body rather than absorbed in bloodstream and help in growth.

Because CF also affects epithelial cells in the skin’s sweat glands, kids with CF may have a salty “frosting” on their skin or taste “salty” when their parents kiss them.

Because CF produces thick mucus within the respiratory tract, kids with CF may suffer from nasal congestion, sinus problems, wheezing, and asthma-like symptoms. As CF symptoms advance, they can develop a chronic cough that produces thick, heavy, discolored mucus. They also may suffer from recurrent lung infections.

As chronic infections decreases lung function, person with CF may eventually begin to feel short of breath, even at rest. Despite aggressive medical therapy, lung disease develops in nearly all patients with CF and is a common cause of disability and decrease life span.