Cystic fibrosis is a genetic disorder characterized by an abnormality in the glands that produce sweat and mucus. Cystic fibrosis affects various organ systems in children and young adults, including the respiratory system, digestive system and reproductive system.
Cystic fibrosis signs and symptoms vary, depending on the severity of the disease. Patients of cystic fibrosis have a higher than normal level of salt in their sweat. Parents often can taste the salt when they kiss their children.
- Salty skin
- Respiratory symptoms
- Persistent cough that produces thick sputum
- Exercise intolerance
- Repeated lung infections
- Inflamed nasal passages or a stuffy nose
- GI symptoms
- Poor weight gain and growth
- Intestinal blockage, particularly in newborns (meconium ileus)
- Severe constipation
Other medical conditions may be associated:
- Nasal polyps
- Pneumothorax. Collapse of the lung often due to intense coughing.
- Hemoptysis. Coughing up blood.
- Cor pulmonale. Enlargement of the right side of the heart due to increased pressure in the lungs.
- Excess gas in the intestines
- Rectal prolapse
- Liver disease
Most cases of cystic fibrosis can now identified with newborn screening. Diagnostic procedures for cystic fibrosis include a sweat test to measure the amount of sodium chloride (salt) present. Higher than normal amounts of sodium and chloride suggests cystic fibrosis. Other diagnostic procedures include:
There is no cure for cystic fibrosis, but treatment can ease symptoms and reduce complications.
Management of problems that cause lung obstruction, which may involve:
- Physical therapy
- Exercise to loosen mucus, stimulate coughing, and improve overall physical condition
- Medications to reduce mucus and help breathing
- Antibiotics to treat infections
- Anti-inflammatory medications
Management of digestive problems, which may involve:
- Appropriate diet
- Pancreatic enzymes to aid digestion
- Vitamin supplements
- Treatments for intestinal obstruction