Nirogacestat Dosage

Medically reviewed by Drugs.com. Last updated on Jul 8, 2024.

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.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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