CYSTIC FIBORISIS

Symptoms

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
• Wheezing
• Breathlessness
• 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

• Infertility

Other medical conditions may be associated:

• Sinusitis
• 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
• Diabetes
• Pancreatitis
• Gallstones

Diagnosis

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:

Preventions

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