Additional Information
| Name | ORKAMBI® Lumacaftor/Ivacaftor 200mg/125mg tablets |
| Description | The CFTR protein is a chloride channel present at the surface of epithelial cells in multiple organs. The F508del mutation impacts the CFTR protein in multiple ways, primarily by causing a defect in cellular processing and trafficking that reduces the quantity of CFTR at the cell surface. The small amount of F508del-CFTR that reaches the cell surface has low channel-open probability (defective channel gating). Lumacaftor is a CFTR corrector that acts directly on F508del-CFTR to improve its cellular processing and trafficking, thereby increasing the quantity of functional CFTR at the cell surface. Ivacaftor is a CFTR potentiator that facilitates increased chloride transport by potentiating the channel-open probability (or gating) of the CFTR protein at the cell surface. The combined effect of lumacaftor and ivacaftor is increased quantity and function of F508del-CFTR at the cell surface, resulting in increased chloride ion transport. The exact mechanisms by which lumacaftor improves cellular processing and trafficking of F508del-CFTR and ivacaftor potentiates F508del-CFTR are not known. |
| Active Ingredient | ivacaftor lumacaftor |
| Indication | Orkambi tablets are indicated for the treatment of cystic fibrosis (CF) in patients aged 6 years and older who are homozygous for the F508del mutation in the CFTR gene |
| Strengths | Orkambi 100 mg/125 mg film-coated tablets Each film-coated tablet contains 100 mg of lumacaftor and 125 mg of ivacaftor. Orkambi 200 mg/125 mg film-coated tablets Each film-coated tablet contains 200 mg of lumacaftor and 125 mg of ivacaftor. |
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Pharma Solution is the marketing agent and Distributor for the listed products for the Gulf and MENA region. The products are either registered and approved by the respective local health authority or contracted by the MAH to supply to the end users on a named patient basis. Some products such as Orphan drugs, rare diseases medicines are imported based on special import permits and as per need of the Hospitals in compliance with full documentation.
