In the United Kingdom, Orkambi is available in both tablets and oral granules. The oral granules are usually prescribed to children for improved compliance.
Prescription Orkambi contains a combination of both lumacaftor and ivacaftor. Orkambi is prescribed to adults and children 2 years and older in the treatment of cystic fibrosis. Orkambi is prescribed only to patients that have a specfic gene mutation called the F508del gene mutation.
Cystic fibrosis is an inherited disease caused by the mutation of this gene and one active ingredient in Orkambi, lumacaftor, increases the CFTR protein in the cell and the other ingredient ivacaftor increases the defective CFRT protein activity. The action of these 2 ingredients causes the mucus and digestive juices to become less thick.
Improvement in cystic fibrosis can prevent some of the build up of the thick secretions of the lungs which cause inflammation and infections. Another goal of Orkambi treatment is to prevent the blockage of tubes from the pancreas which slows down food digestion.
The recommended Orkambi dose depends upon the weight and age of the patient. Orkambi is taken every 12 hours along with fatty food as directed by your physician.
If you miss a dose of Orkambi, and it is less than 6 hours from its next scheduled dose, you can take the dose as soon as possible with fatty food. If it is more than 6 hours since your last dose, then skip that dose and continue with your next dose at that scheduled time. Never take 2 doses of Orkambi at the same time. If you have missed one week or more of Orkambi, speak with your doctor as you may need to adjust the dose to restart the therapy.
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