In the Europe, 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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Information about Orkambi (Lumacaftor / Ivacaftor)
Orkambi is a combination therapy containing lumacaftor and ivacaftor, used to treat cystic fibrosis (CF), a genetic condition that impacts the lungs, pancreas, and other organs. Orkambi works by targeting the underlying cause of CF, which is a mutation in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. The combination of these two medications works to enhance the function of the faulty CFTR protein, leading to improved lung function and reduced respiratory symptoms in individuals with specific CF mutations.
Product Highlights:
Orkambi is indicated for the treatment of cystic fibrosis in patients who are homozygous for the F508del mutation in the CFTR gene. This mutation is the most prevalent cause of cystic fibrosis.
The combination of lumacaftor and ivacaftor helps improve the function of the CFTR protein, which is defective in cystic fibrosis, leading to better lung function and a decrease in pulmonary symptoms such as cough and mucus buildup.
Orkambi has been shown to reduce the frequency of pulmonary exacerbations and hospitalizations in patients with CF.
Key Ingredients:
Lumacaftor
Ivacaftor
Key Benefits of Orkambi (Lumacaftor / Ivacaftor)
Orkambi helps to improve lung function, reduce the buildup of mucus, and decrease coughing, making it easier for patients to breathe.
The combination therapy has been shown to reduce the number of pulmonary exacerbations—flare-ups of lung symptoms that often require antibiotics or hospitalization.
By addressing the underlying genetic defect, Orkambi can help improve overall health status and quality of life for individuals with CF, reducing the severity of symptoms and preventing further damage to the lungs.
For many individuals, Orkambi may help slow the progression of cystic fibrosis and delay the need for advanced treatments, such as lung transplants.
Orkambi is FDA-approved for use in patients with two copies of the F508del mutation, the most common mutation associated with cystic fibrosis.
Direction of Use
The recommended dose of Orkambi is typically one tablet (lumacaftor 200 mg / ivacaftor 125 mg) taken twice daily with food. Alternatively, for younger patients or those unable to swallow tablets, an oral granule formulation may be used.
Orkambi should be taken with food to help improve absorption. It can be taken with or without meals, but taking it with food may reduce the risk of gastrointestinal side effects.
If a dose is missed, it should be taken as soon as possible unless it is nearly time for the next dose. If you miss a dose, skip it and take your next dose at the usual time. Do not take two doses at once to make up for the missed dose.
For best results, take Orkambi regularly as prescribed by a healthcare provider. Regular follow-ups and lung function tests may be necessary to monitor the effectiveness of the treatment.
Recommended dose of ORKAMBI in patients aged 2 years and older
Age
ORKAMBI dose
Total daily dose
2 through 5 years and weighing less than 14 kg
One lumacaftor 100 mg/ivacaftor 125 mg sachet every 12 hours
lumacaftor 200 mg/ivacaftor 250 mg
2 through 5 years and weighing 14 kg and greater
One lumacaftor 150 mg/ivacaftor 188 mg sachet every 12 hours
lumacaftor 300 mg/ivacaftor 376 mg
6 through 11 years
Two lumacaftor 100 mg/ivacaftor 125 mg tablets every 12 hours
lumacaftor 400 mg/ivacaftor 500 mg
12 years and older
Two lumacaftor 200 mg/ivacaftor 125 mg tablets every 12 hours
lumacaftor 800 mg/ivacaftor 500 mg
Safety Concerns
Orkambi may cause liver enzyme elevations. Liver function should be monitored regularly, especially in the first few months of treatment. If liver problems are detected, dose adjustments or discontinuation may be necessary.
Ivacaftor has been associated with an increased risk of cataracts in some patients, particularly in those under the age of 18. Regular eye exams are recommended during treatment with Orkambi.
Orkambi may interact with other medications, especially those that are metabolized by the CYP3A enzyme. It is important to inform the healthcare provider of all medications being taken, including over-the-counter drugs and supplements.
Some patients may experience shortness of breath, chest tightness, or wheezing when starting Orkambi. These symptoms may resolve over time, but if they persist or worsen, medical attention should be sought.
Common side effects of Orkambi include diarrhea, nausea, abdominal pain, and flatulence. These side effects are often mild but should be discussed with the doctor if they become bothersome.
Avoid Orkambi (Lumacaftor / Ivacaftor) If
Orkambi should not be used in patients with severe liver impairment (e.g., cirrhosis) due to the potential risk of exacerbating liver damage. Regular liver monitoring is essential, and dose adjustments may be required.
Orkambi should not be used in individuals who are allergic to lumacaftor, ivacaftor, or any other ingredients in the formulation. Signs of an allergic reaction may include rash, swelling, or difficulty breathing.
Orkambi should be used with caution in children under the age of 18 due to the risk of cataracts associated with ivacaftor. Pediatric patients should undergo regular eye exams during treatment.
Orkambi may interact with certain drugs, particularly those that affect liver enzymes (e.g., rifampin, carbamazepine). These drugs should be avoided or used with caution while on Orkambi.
Orkambi has not been well-studied in pregnant women, and its use during pregnancy should be avoided unless the potential benefit justifies the risk to the fetus. Women who are pregnant or planning to become pregnant should consult their healthcare provider to discuss alternative treatment options.
