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What is cystic fibrosis?
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Cystic fibrosis, or CF, is a life-threatening disease that affects the secretory glands. These glands produce mucus, sweat and digestive juices. The fluids secreted from these glands are normally watery and slippery, but with CF they become thick and sticky. This causes a buildup and blockage of the airways and pancreas ducts, resulting in severe damage to the lungs and digestive tract.
CF is an inherited disease, but the parents of patients often do not have the disease themselves. Two faulty genes need to be present, one from each parent, for the child to develop the disease. CF is most prevalent in white people of Northern European descent and approximately 1 in 29 Americans fitting that criteria carry the CF gene. Newborns are now screened for the disease in all 50 states and are typically diagnosed within the first month of birth.
Signs and symptoms of CF can vary, not only from person to person, but within the same person, depending on the severity of the disease. Early symptoms to look for include:
- No bowel movements in the first 24 to 48 hours of life;
- Poor growth and slow weight gain;
- Salty-tasting skin;
Other symptoms include:
- Breathlessness;
- Coughing or increased mucus in the sinuses or lungs;
- Distended (swollen) belly;
- Fatigue;
- Inflamed nasal passages or a stuffy nose;
- Nausea and loss of appetite;
- Recurrent lung infections;
- Severe constipation;
- Stomach pain;
- Stools that are pale or clay-colored, foul smelling and/or greasy; and
- Wheezing; and
- Clubbed fingers.
CF can cause serious complications in the respiratory, digestive and reproductive systems, including;
- Bronchiectasis;
- Collapsed lung(s);
- Blocked bile duct;
- Diabetes;
- Electrolyte imbalance;
- Infertility (in men);
- Intussusception;
- Nasal polyps;
- Nutritional deficiencies;
- Osteoporosis;
- Pancreatitis;
- Pneumothorax;
- Rectal prolapse;
- Recurrent pneumonia; and
- Respiratory failure.
While there is no cure for CF, treatment options are available to help with symptoms and lessen the risk for complications. For complications concerning the lungs, antibiotics, inhaled medications, breathing treatments, flu and pneumococcal polysaccharide vaccines, oxygen therapy and lung transplants are all treatment options. Additionally, therapies to help thin the built up mucus such as deep breathing activities, exercise, airway clearing devices and manual chest percussions, can be helpful.
For complications associated with bowel and nutritional problems, treatments include a special high protein diet, pancreatic enzymes for fat and protein absorption and vitamin A, D, E and K supplements.
Patients with CF should be sure to avoid smoke, dust, dirt, fumes, mold and mildew. Self-care should also include drinking plenty of fluids, exercising two to three times per week and clearing the airways up to four times each day.
Additional information about cystic fibrosis can be found at these websites:
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/?report=printable
http://www.nhlbi.nih.gov/health/health-topics/topics/cf/#