Bexdeco

Indications

Ivacaftor is used to treat cystic fibrosis (CF), an inherited disease that causes thick, sticky mucus in the lungs, gut and other organs. It is used only in people who carry certain changes (mutations) in the CFTR gene that respond to it, confirmed by genetic testing. It may be given alone or together with other CFTR modulator medicines such as Lumacaftor, Tezacaftor or Elexacaftor.

How It Works

The CFTR protein is a channel on the surface of cells that moves salt and water in and out. In CF this channel is faulty. Ivacaftor is a CFTR potentiator: it helps the channel stay open longer, so more chloride and water can flow. This thins the mucus, improves breathing and lung function, and helps weight gain and digestion.

Dosage and Administration

Take exactly as prescribed by your CF specialist; the dose depends on age, weight, the combination used, and liver function, and may need to be reduced if you take certain other medicines. Tablets or granules are taken by mouth, usually every 12 hours, with food that contains fat (for example eggs, butter, peanut butter, cheese or whole milk), as this greatly improves absorption. Granules are mixed with a small amount of soft food or liquid. Do not take with grapefruit or Seville orange.

Interactions

Strong CYP3A inhibitors such as Ketoconazole, Itraconazole, Clarithromycin and Ritonavir raise Ivacaftor levels, so the dose must be reduced; moderate inhibitors such as Fluconazole and Erythromycin also need dose changes. Strong inducers such as Rifampicin, Rifabutin, Carbamazepine, Phenytoin, Phenobarbital and St John's wort make it much less effective and should be avoided. Ivacaftor may raise levels of Digoxin, Ciclosporin, Tacrolimus and some benzodiazepines such as Midazolam. Grapefruit raises its levels.

Contraindications

Do not use if you are allergic to Ivacaftor or any ingredient of the product.

Side Effects

Common side effects include headache, sore throat, stuffy or runny nose, upper respiratory infections, stomach pain, diarrhoea, rash, dizziness and raised liver enzymes. Non-cataract clouding of the lens of the eye has been seen in children, so eye checks are advised.

Get urgent medical help if you develop yellowing of the skin or eyes, dark urine, pain in the upper right side of the abdomen, severe rash, swelling of the face or throat, or difficulty breathing.

Pregnancy and Lactation

Information on use in pregnancy is limited. Your specialist will weigh the benefits for your health against possible risks. It is not known whether it passes into breast milk; discuss breastfeeding with your doctor.

Precautions and Warnings

• Liver blood tests are needed before starting and regularly during treatment.
• Eye examinations are recommended for children and adolescents.
• Dose reduction is needed in moderate liver disease; use with care in severe liver or kidney disease.
• It may cause dizziness; take care when driving.
• Keep taking your other CF treatments unless told otherwise.

Overdose

Experience with overdose is limited. If too much has been taken, contact a doctor straight away. Treatment is supportive with monitoring.

Therapeutic Class

CFTR potentiator (cystic fibrosis transmembrane conductance regulator modulator)

Storage

Store below 30°C in a dry place in the original pack, away from light and out of the reach of children.