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Cystic Fibrosis Treatments

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Antibiotics

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Use: Treat and prevent lung infections. Mechanism: Kills or stops the growth of bacteria. Potential side effects: Resistance, allergic reactions, digestive upset.

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High-Frequency Chest Wall Oscillation (HFCWO) Devices

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Use: Loosen mucus in lungs. Mechanism: Vibrates the chest to increase mucus clearance. Potential side effects: Skin irritation, discomfort, muscle pain.

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CFTR Modulators

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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.

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Mucolytics

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Use: Thins respiratory mucus. Mechanism: Breaks down the DNA of dead cells in the mucus. Potential side effects: Pharyngitis, laryngitis, bronchospasm.

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Chest Physiotherapy

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Use: Enhance mucus clearance from lungs. Mechanism: Physical percussion and vibration to loosen mucus. Potential side effects: Discomfort, increased heart rate, bruising.

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Lumacaftor/Ivacaftor (Orkambi)

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Use: Reducing pulmonary exacerbations. Mechanism: Combination of a corrector (Lumacaftor) and potentiator (Ivacaftor) of CFTR protein. Potential side effects: Dyspnea, diarrhea, fatigue.

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Tezacaftor/Ivacaftor (Symdeko)

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Use: Improving respiratory symptoms. Mechanism: A combination drug that includes a corrector (Tezacaftor) and potentiator (Ivacaftor). Potential side effects: Nasopharyngitis, headache, sinus congestion.

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Ivacaftor (Kalydeco)

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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.

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Pancreatic Enzyme Replacement Therapy (PERT)

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Use: Aids digestion. Mechanism: Supplies digestive enzymes to compensate for those the pancreas fails to produce. Potential side effects: Constipation, stomach pain, intestinal blockage.

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Hypertonic Saline

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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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