For
UK
audiences
The professional's guide to product selection
The professional's guide to product selection
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.
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.
Use with caution in the case of hepatic impairment.
Tablets
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
Reduction of sputum viscosity.
• Child 2–4 years: 62.5–125 mg, 4 times daily
• Child 5–11 years: 250 mg, 3 times daily
• Over 12 years: initially 2.25 g daily in divided doses, then 1.5 g daily in divided doses as condition improves.
Mucolytic to facilitate expectoration.
• Child 2–4 years: 62.5–125 mg, 4 times daily
• Child 5–11 years: 250 mg, 3 times daily
• Over 12 years: initially 2.25 g daily in divided doses, then 1.5 g daily in divided doses as condition improves.
Mucolytic to facilitate expectoration.
• Child 2–4 years: 62.5–125 mg, 4 times daily
• Child 5–11 years: 250 mg, 3 times daily
• Over 12 years: initially 2.25 g daily in divided doses, then 1.5 g daily in divided doses as condition improves.
Mucolytic to facilitate expectoration.
• Child 2–4 years: 62.5–125 mg, 4 times daily
• Child 5–11 years: 250 mg, 3 times daily
• Over 12 years: initially 2.25 g daily in divided doses, then 1.5 g daily in divided doses as condition improves.
Mucolytic to facilitate expectoration.
• Child 2–4 years: 62.5–125 mg, 4 times daily
• Child 5–11 years: 250 mg, 3 times daily
• Over 12 years: initially 2.25 g daily in divided doses, then 1.5 g daily in divided doses as condition improves.
Mucolytic
Osmohale is used for an indirect osmotic bronchial challenge test which can be used to identify bronchial hyperresponsiveness, a clinical feature of some respiratory conditions such as asthma.
Contraindications: Use with caution in renal impairment and to avoid in pregnancy. The Osmohale test should not be used in patients below 6 years of age due to their inability to provide reproducible spirometric measurements.
There is limited information on the use of Osmohale in patients 6-18 years of age therefore Osmohale is not recommended in this population.
Dose: See manufacturers’ instructions for more information. The test should be repeated until the patient has a positive response or 635 mg has been administered.