Lumacaftor + ivacaftor

Generic name
Lumacaftor + ivacaftor
Brand name
ATC Code
R07AX30
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Lumacaftor + ivacaftor

Dosages
Side effects in children
Warnings & precautions in children
Contra-indications in children

Interactions
PK
Renal impairment
References

Pharmacokinetics in children

The exposures are similar between adults and the paediatric population based on population (PK) analyses  [SmPC Orkambi film-coated tablets]

dose recommendation of formulary compared to licensed use (on-label versus off-label)

No information is present at this moment.

Available formulations

No information is present at this moment.

General Dose Info

Adjust the dose if

  • Co-administerred with moderate or strong CYP3A4 inhibitors
  • In moderate to severe hepatic failure (Child-Pugh B/C)

see SmPC for recommendations

Dosages

Cystic fibrosis
  • Oral
    • 1 year up to 6 years and 9 up to 14 kg

      • 200 mg/250 mg lumacaftor/ivacaftor per day in 2 doses

      • Directions for administration:

        Swallow the tablets whole (do not chew, break or dissolve them).

    • 1 year up to 6 years and ≥ 14 kg

      • 300 mg/376 mg lumacaftor/ivacaftor per day in 2 doses

    • 6 years up to 12 years

      • 400 mg/500 mg lumacaftor/ivacaftor per day in 2 doses

      • Directions for administration:

        Swallow the tablets whole (do not chew, break or dissolve them).

    • ≥ 12 years

      • 800 mg/500 mg lumacaftor/ivacaftor per day in 2 doses

      • Directions for administration:

        Swallow the tablets whole (do not chew, break or dissolve them).

    • 1 year up to 6 years and 7 up to 9 kg

      • 150 mg/ 188 mg lumacaftor/ivacaftor per day in 2 doses

      • Directions for administration:

        Swallow the tablets whole (do not chew, break or dissolve them).

Renal impaiment in children > 3 months

GFR ≥10 ml/min/1.73m2: Dose adjustment not required.

GFR <10 ml/min/1.73m2: A general recommendation on dose adjustment cannot be provided.

The complete list of all undesirable drug reactions can be found in the national Summary of Product Characteristics (SmPC) – click here

Side effects in children

Side effects in children are the same as the side effects in adults.

The complete list of all contra-indications can be found in the national Summary of Product Characteristics (SmPC) – click here

Contra-indications

No information available on specific contra indications in children.

The complete list of all warnings and precautions can be found in the national Summary of Product Characteristics (SmPC) – click here

Warnings & precautions in children

Cases of non-congenital lens opacities without impact on vision have been reported in paediatric patients treated with lumacaftor/ivacaftor and ivacaftor monotherapy. Although other risk factors were present in some cases (such as corticosteroid use and exposure to radiation), a possible risk attributable to ivacaftor cannot be excluded. Baseline and follow-up ophthalmological examinations are recommended in paediatric patients initiating treatment with lumacaftor/ivacaftor.

Elevations in transaminases were seen more frequently in children and adolescents than in adult patients. In the various pediatric age cohorts, increases in transaminases were observed more frequently in the 2 to 5 year olds than in the 6 to 11 year olds. [SmPC Orkambi film-coated tablets and granules]

Interactions

The complete list of all interactions can be found in the national Summary of Product Characteristics (SmPC) – click here

OTHER RESPIRATORY SYSTEM PRODUCTS

This pages provides a list of drugs from the same ATC class for comparison. This does not necessarily mean that these drugs are interchangeable.

Lung surfactants
R07AA02
R07AA05
Respiratory stimulants
R07AB01
Other respiratory system products
R07AX02
R07AX32
R07AX31

Reference

  1. Vertex Pharmaceuticals (Europe) Limited, SmPC Orkambi (EU/1/15/1059) Rev.32. 27-07-2023, www.geneesmiddeleninformatiebank.nl

Changes

Therapeutic Drug Monitoring


Overdose