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Cystic Fibrosis Treatments
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Antibiotics
Use: Treat and prevent lung infections. Mechanism: Kills or stops the growth of bacteria. Potential side effects: Resistance, allergic reactions, digestive upset.
High-Frequency Chest Wall Oscillation (HFCWO) Devices
Use: Loosen mucus in lungs. Mechanism: Vibrates the chest to increase mucus clearance. Potential side effects: Skin irritation, discomfort, muscle pain.
CFTR Modulators
Use: Improves function of the CFTR protein. Mechanism: Targets the defective CFTR gene to enhance protein function. Potential side effects: Headache, gastrointestinal issues, chest tightness.
Mucolytics
Use: Thins respiratory mucus. Mechanism: Breaks down the DNA of dead cells in the mucus. Potential side effects: Pharyngitis, laryngitis, bronchospasm.
Chest Physiotherapy
Use: Enhance mucus clearance from lungs. Mechanism: Physical percussion and vibration to loosen mucus. Potential side effects: Discomfort, increased heart rate, bruising.
Lumacaftor/Ivacaftor (Orkambi)
Use: Reducing pulmonary exacerbations. Mechanism: Combination of a corrector (Lumacaftor) and potentiator (Ivacaftor) of CFTR protein. Potential side effects: Dyspnea, diarrhea, fatigue.
Tezacaftor/Ivacaftor (Symdeko)
Use: Improving respiratory symptoms. Mechanism: A combination drug that includes a corrector (Tezacaftor) and potentiator (Ivacaftor). Potential side effects: Nasopharyngitis, headache, sinus congestion.
Ivacaftor (Kalydeco)
Use: Improving lung function. Mechanism: Potentiator that enhances the gating function of the CFTR protein. Potential side effects: Headache, upper respiratory tract infection, abdominal pain.
Pancreatic Enzyme Replacement Therapy (PERT)
Use: Aids digestion. Mechanism: Supplies digestive enzymes to compensate for those the pancreas fails to produce. Potential side effects: Constipation, stomach pain, intestinal blockage.
Hypertonic Saline
Use: Mucus hydration and clearance. Mechanism: Osmotic agent that draws water into the airway to thin mucus. Potential side effects: Cough, salty taste, bronchospasm.
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