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Orkambi (lumacaftor & ivacaftor): Cystic Fibrosis Drug Side Effects & Dosage

What is Orkambi? What is Orkambi used for?

Orkambi is a combination of two drugs, lumacaftor
and ivacaftor (Kalydeco) and is used for treating cystic fibrosis. Cystic
fibrosis (CF) is an inherited disease that affects the secretory glands,
including the mucus and sweat glands. Cystic fibrosis mostly affects the lungs,
pancreas, liver, intestines, sinuses, and sex organs. Cystic fibrosis is due to a mutation in
the cystic fibrosis gene on chromosome 7. The cystic fibrosis gene encodes a protein known as the cystic
fibrosis transmembrane regulator (CFTR). The abnormal CFTR protein in patients
with cystic fibrosis leads to disruption of chloride channels on the cells which are
important for the secretion of water. Cystic fibrosis is characterized by the production of
abnormal mucus that is excessively thick and sticky because it lacks water. The
abnormal mucus leads to blockages within the airways of the lungs. This leads to
repeated, serious lung infections that can permanently and progressively damage
the lungs.

Lumacaftor corrects abnormalities in the CFTR protein and increases the
amount of functional CFTR protein in one type of mutation. Ivacaftor increases
the action of CFTR protein. The combination of both drugs increases the
quantity, stability, and function of CFTR, reducing the production of abnormal
mucous and increasing lung function. The FDA approved Orkambi in July, 2015.

What brand names are available for Orkambi?

Orkambi

Is Orkambi available as a generic drug?

No

Do I need a prescription for Orkambi?

Yes

What are the side effects of Orkambi?

WARNING

  • Orkambi should be used with caution in patients with
    advanced
    liver disease and only when the benefits are expected to outweigh the
    risks.
  • Orkambi is associated with liver-related adverse events. Liver function
    should be tested before treatment and every 3 months during the first year of
    treatment, and yearly thereafter.
  • Chest discomfort, dyspnea, and abnormal respiration may occur.
  • Non-congenital lens opacities/cataracts have been reported in pediatric
    patients treated with ivacaftor. Baseline and follow-up eye examinations are
    recommended in pediatric patients treated with Orkambi.

What is the dosage for Orkambi?

Adults and pediatric patients age 12 years and older should take two
tablets every 12 hours with fat-containing food since fat increases the
absorption of Orkambi. Examples of fat-containing foods include

  • eggs,
  • avocados,
  • nuts,
  • butter,
  • peanut butter,
  • cheese pizza, and
  • whole-milk dairy products such as
    whole milk, cheese, and yogurt.

If a dose is missed it may be taken within 6 hours of the regular dosing
time. If more than 6 hours has passed since the regular dosing time, the dose
should be skipped and taken at the next regular scheduled time. The dose should
not be doubled.

Which drugs or supplements interact with Orkambi?

: Orkambi may decrease blood levels and reduce the effect of
some drugs that are broken down by the liver. Examples of interacting drugs
include

Blood levels of digoxin and effect of warfarin should be monitored closely
because Orkambi may affect blood levels of digoxin and warfarin.

Is Orkambi safe to take if I’m pregnant or breastfeeding?

Orkambi is probably excreted in human milk. The effect of
Orkambi on nursing infants has not been evaluated.

What else should I know about Orkambi?

What preparations of Orkambi are available?

Tablets: lumacaftor 200 mg and ivacaftor 125 mg

How should I keep Orkambi stored?

Orkambi should be stored at room temperature, between 15 C and 30 C
(59 F and 86 F).

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