Orkambi - Lumacaftor with Ivacaftor
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Description
Lumacaftor increases the number of proteins that each the cell surface. Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) protein potentiator that increases chloride transport in the abnormal CFTR protein.
By mouth using granules
Child 2–5 years (body-weight up to 14 kg): 100/125 mg every 12 hours.
Child 2–5 years (body-weight 14 kg and above): 150/188 mg every 12 hours.
By mouth using tablets
Child 6–11 years: 200/250 mg every 12 hours.
Child 12–17 years: 400/250 mg every 12 hours.
Adult: 400/250 mg every 12 hours – specialist use only.
Indications
Lumacaftor increases the number of proteins that each the cell surface. Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) protein potentiator that increases chloride transport in the abnormal CFTR protein.
Contraindications
Use with caution in the case of hepatic impairment.
Preparations Available
Tablets
Sizes Available
Tablets
Orkambi 100 mg/125 mg, 112 tablets
Orkambi 200 mg/125 mg, 112 tablets
Granules
Orkambi 100 mg/125 mg, 56 sachets
Orkambi 150 mg/188 mg, 56 sachets
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