Deutivacaftor / Tezacaftor / Vanzacaftor Dosage

Medically reviewed by Drugs.com. Last updated on Feb 24, 2025.

Applies to the following strengths: 125 mg-50 mg-10 mg; 50 mg-20 mg-4 mg

Usual Adult Dose for Cystic Fibrosis

Vanzacaftor 20 mg-tezacaftor 100 mg-deutivacaftor 250 mg orally once a day

Comments:

Confirm the presence of at least 1 indicated mutation with an FDA-cleared cystic fibrosis (CF) mutation test if the patient's genotype is unknown.

Use: For the treatment of cystic fibrosis in patients who have at least one F508del mutation or another responsive mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene

Usual Pediatric Dose for Cystic Fibrosis

6 TO LESS THAN 12 YEARS:
Weight less than 40 kg:
Vanzacaftor 12 mg-tezacaftor 60 mg-deutivacaftor 150 mg orally once a day

Weight at least 40 kg:
Vanzacaftor 20 mg-tezacaftor 100 mg-deutivacaftor 250 mg orally once a day

12 YEARS AND OLDER:
Vanzacaftor 20 mg-tezacaftor 100 mg-deutivacaftor 250 mg orally once a day

Comments:

Confirm the presence of at least 1 indicated mutation with an FDA-cleared CF mutation test if the patient's genotype is unknown.

Use: For the treatment of cystic fibrosis in patients who have at least one F508del mutation or another responsive mutation in the CFTR gene

Renal Dose Adjustments

Mild to moderate renal dysfunction (Estimated GFR 30 to less than 90 mL/min/1.73 m2): No adjustment recommended
Severe renal dysfunction (Estimated GFR less than 30 mL/min/1.73 m2) or End-stage renal disease: Recommended only if benefits outweigh risks

Liver Dose Adjustments

Mild liver dysfunction (Child-Pugh A): No adjustment recommended; liver function tests should be closely monitored.
Moderate liver dysfunction (Child-Pugh B): Use is not recommended unless clearly needed and the benefit outweighs the risk. Liver function tests should be closely monitored.
Severe liver dysfunction (Child-Pugh C): Not recommended

Dose Adjustments

Dosage Modification for Strong or Moderate CYP450 3A Inhibitors:

6 TO LESS THAN 12 YEARS:
Weight Less Than 40 kg:

Moderate CYP450 3A Inhibitors: Vanzacaftor 8 mg-tezacaftor 40 mg-deutivacaftor 100 mg orally every other day
Strong CYP450 3A Inhibitors: Vanzacaftor 8 mg-tezacaftor 40 mg-deutivacaftor 100 mg orally once a week

Weight At Least 40 kg:

Moderate CYP450 3A Inhibitors: Vanzacaftor 10 mg-tezacaftor 50 mg-deutivacaftor 125 mg orally every other day
Strong CYP450 3A Inhibitors: Vanzacaftor 10 mg-tezacaftor 50 mg-deutivacaftor 125 mg orally once a week

12 YEARS AND OLDER:

Moderate CYP450 3A Inhibitors: Vanzacaftor 10 mg-tezacaftor 50 mg-deutivacaftor 125 mg orally every other day
Strong CYP450 3A Inhibitors: Vanzacaftor 10 mg-tezacaftor 50 mg-deutivacaftor 125 mg orally once a week

Precautions

US BOXED WARNING(S):
Drug-Induced Liver Injury and Liver Failure:

This drug can elevate transaminase levels.
Cases of serious and potentially fatal drug-induced liver injury and liver failure were reported with a drug that contains same or similar active ingredients as this drug. Liver injury occurred within the first month of therapy and up to 15 months after the start of therapy.

Recommendations:

Assess liver function tests (ALT, AST, alkaline phosphatase, and bilirubin) before initiating, monthly for the first 6 months, every 3 months for next 12 months, and then at least yearly thereafter. Consider more frequent monitoring in patients with a history of liver disease, elevated liver function tests at baseline.
This drug should be discontinued if significant elevations in liver function tests or signs or symptoms of liver injury occur.
Consider referring to a hepatologist and closely monitor the patient with clinical and laboratory monitoring until abnormalities resolve.
Resume therapy if abnormalities resolve and only if benefit is expected to outweigh risk with close monitoring.
This drug is not recommended in patients with moderate hepatic impairment (Child-Pugh B) and should only be considered when there is a clear medical need and the benefit outweighs the risk. If used, monitor patients closely.
This drug should not be used in patients with severe hepatic impairment (Child-Pugh C).

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 6 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

Confirm presence of at least 1 indicated mutation with an FDA-cleared CF mutation test if the patient's genotype is unknown.
Before initiating this drug, obtain liver function tests (LFTs) (ALT, AST, alkaline phosphatase, and bilirubin).
Take with fat-containing food (such as food prepared with butter or oils; containing eggs, peanut butter, cheeses, nuts, whole milk, or meats) at the same time each day. Do not take with grapefruit or grapefruit juice.
Swallow the tablets whole.
Monitor LFTs monthly for 6 months, every 3 months for the next 12 months, and then at least yearly thereafter.
Consider more frequent monitoring in patients with history of liver disease, elevated LFTs at baseline, or history of elevated LFTs with drugs containing elexacaftor, tezacaftor, and/or ivacaftor.
If a dose is missed, administer the missed dose as soon as possible and continue the original schedule; if it has been missed by more than 6 hours, skip the missed dose, and continue with the original schedule the next day.

Storage requirements:

Store at 20C to 25C (68F to 77F); excursions permitted to 15C to 30C (59F to 86F).

Monitoring:

Hepatic: Liver function tests (before initiation, monthly during therapy for 6 months, every 3 months for the next 12 months, and then at least yearly thereafter or more frequently)

Patient advice:

Read the US FDA-approved patient labeling (Medication Guide).
This drug can cause elevations of transaminase levels. Before initiating this drug, liver function tests should be assessed monthly for 6 months, every 3 months for the next 12 months, and then at least yearly thereafter. More frequent monitoring may be required in patients with a history of liver disease or liver function test elevations at baseline.
Stop treatment if symptoms of liver injury occur (e.g., jaundice, right upper quadrant pain, nausea, vomiting, altered mental status, ascites) and immediately notify your healthcare provider.
Discontinue treatment and notify your healthcare provider if signs and symptoms of a hypersensitivity reaction, including rash, hives, itching, facial swelling, tightness of the chest, and wheezing occur.
Concomitant administration of this drug with other drugs, herbal supplements, or vitamins may reduce this drug's effectiveness or increase the risk of adverse reactions. Avoid food or drink containing grapefruit.
Report all your concurrent prescription and nonprescription medications, herbal supplements, or vitamins you are taking.