Cystic Fibrosis (CF)
CF is inherited disease.
Build up of thick, stick mucus damaged lungs, digestive tract and another organ.
Abnormally in function of cell protein (Cystic fibrosis transmembrane regulator; CFTR).
Control the flow of water and salts in body and mucous making cells.
Movement of salt and water in and out of cell altered.
Mucous become thickened.
Respiratory system; sinuses and lungs
Digestive system; pancreas, liver, gall bladder, intestine
Reproductive system; sperm carrying duct become clogged,
Sweat gland.
Clinical manifestations
1. Abnormalities in glands that produce sweat and mucus.
2. Thick muscus accumulate in lungs and intestine.
3. Diarrhea
4. Stool - foul smelling and greasy
5. Frequent episodes of Pneumonia
6. Frequent episodes of wheezing
7. Persistent cough
8. Abdominal pain
9. Gas in intestine
Other medical symptoms include;
1. Nasal polyps
2. Hemoptysis (coughing in blood)
3. Cor pulmonary (right-side heart failure); long term high blood pressure in pulmonary artery.
4. Sinusitis
5. Pancreatitis
6. Rectal prolapse; parts of intestine slips out side anus.
Diagnostic Evaluation
1. History taking and physical examination
2. Sweat (chloride) tests; measure amount of chloride in sweats.
3. Genetic test; mutation in CFTR Genes
4. Chest X ray
5. Pulmonary function test (PPT)
6. Sputum culture
7. Stool evaluation; for fat absorption
Management
1. Chest physiotherapy
2. Postrual drainage
3. Medication
Bronchodilators and anti- inflammatory medications
4. Antibiotics
To treat infection
Management of digestive system
Vitamin supplements
Stimulation of pancreatic enzyme to acid digestion.
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