Nirogacestat Dosage

Medically reviewed by Drugs.com. Last updated on Jan 30, 2024.

Applies to the following strengths: 50 mg

Usual Adult Dose for:

Fibromatosis

Additional dosage information:

Renal Dose Adjustments
Liver Dose Adjustments
Dose Adjustments
Precautions
Dialysis
Other Comments

Usual Adult Dose for Fibromatosis

150 mg orally twice a day
Duration of therapy: Until disease progression or unacceptable toxicity

Use: For the treatment of progressing desmoid tumors in patients who require systemic therapy

Renal Dose Adjustments

Renal dysfunction: Data not available

Liver Dose Adjustments

Dose Modifications for Elevated Liver Enzymes:

Grade 2 increased ALT or AST (3 to 5 times the upper limit of normal [3 to 5 x ULN] or greater): Withhold therapy until ALT, AST, or both are resolved to less than 3 x ULN or baseline, and then restart therapy at 100 mg orally twice a day.
Grade 3 or 4 increased ALT or AST (greater than 5 x ULN): Permanently discontinue

Dose Adjustments

DOSE MODIFICATIONS FOR ADVERSE REACTIONS:
Diarrhea:

Grade 3 or 4 diarrhea persisting for at least 3 days despite maximal medical therapy: Withhold therapy until resolved to grade 1 or less, or baseline, and then restart therapy at 100 mg orally twice a day.

Hypophosphatemia:

Grade 3 or 4 hypophosphatemia persisting for at least 3 days despite maximal replacement therapy: Withhold therapy until resolved to grade 1 or less, or baseline, and then restart therapy at 100 mg orally twice a day.

Hypokalemia:

Grade 3 or 4 hypokalemia despite maximal replacement therapy: Withhold therapy until resolved to grade 1 or less, or baseline, and then restart therapy at 100 mg orally twice a day.

DOSE MODIFICATIONS FOR OTHER SEVERE, LIFE-THREATENING, OR PERSISTENT INTOLERABLE GRADE 2 ADVERSE EVENTS:

Withhold therapy until resolved to grade 1 or less, or baseline.
Only restart therapy at dose of 100 mg orally twice a day after considering the potential benefit and likelihood of recurrence of the adverse reaction.
Permanently discontinue treatment for recurrence of a severe or life-threatening adverse reaction upon rechallenge at the reduced dose.

Precautions

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 18 years.

Consult WARNINGS section for additional precautions.

Dialysis

Hemodialysis: This drug is highly bound to plasma proteins and not expected to be dialyzable.
Peritoneal dialysis: Data not available

Other Comments

Administration advice:

Administer each 150 mg dose of this drug as 3 tablets of 50 mg.
Swallow each tablet whole; do not break, crush, or chew.
This drug may be taken with or without food.
If a dose is missed or vomited, instruct the patient to take the next dose at its scheduled time.

Storage requirements:

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

Monitoring:

Dermatologic: For new non-melanoma skin cancers (at baseline and routinely during therapy)
Endocrine: For signs and symptoms of ovarian toxicity
Gastrointestinal: For severe diarrhea
General: For drug interactions (regularly during therapy)
Hepatic: Liver function tests (regularly during therapy)
Metabolic: Phosphate and potassium levels (regularly during therapy)

Patient advice:

Read the US FDA-approved patient labeling (Patient Information).
Notify your health care provider if sustained diarrhea occurs that does not respond to supportive care.
This drug may cause ovarian toxicity and impair fertility, potentially even after discontinuation of treatment; inform your health care provider of any symptoms of ovarian toxicity, including hot flashes or menstrual irregularities.
This drug may elevate liver enzymes; your health care provider should monitor liver function regularly.
Contact your health care provider if any new or changing lesions occur on the skin as this drug may cause new non-melanoma skin cancers.
This drug can cause electrolyte abnormalities which may require phosphate and/or potassium supplementation; seek medical attention if any symptoms of muscle pain or weakness occur.
Inform your health care provider of all concomitant medications, including over-the-counter drugs.
Breastfeeding is not recommended during treatment.
Patients of childbearing potential:
This drug may cause potential harm to a fetus.
Notify your health care provider of a known/suspected pregnancy; stop taking this drug if you become pregnant.
Females and males (with female partners of reproductive potential): Use effective contraception during treatment and for 1 week after the last dose.