Inborn errors of metabolism:
Indications for: VIJOICE
Treatment of severe manifestations of PIK3CA-related overgrowth spectrum (PROS) in patients who require systemic therapy.
Adult Dosage:
Take with food. Swallow whole. If difficulty swallowing, may dissolve tabs with water only and administer as an oral susp. ≥18yrs: 250mg once daily until disease progression or unacceptable toxicity. Dose modification for adverse reactions: see full labeling.
Children Dosage:
<2yrs: not established. Take with food. Swallow whole. If difficulty swallowing, may dissolve tabs with water only and administer as an oral susp. 2–<6yrs: 50mg once daily (dose increase: not established). 6–<18yrs: initially 50mg once daily; after 24 weeks, consider increasing to 125mg once daily for response optimization. Continue until disease progression or unacceptable toxicity. Dose modification for adverse reactions: see full labeling.
VIJOICE Warnings/Precautions:
Severe hyperglycemia. Test fasting plasma glucose (FPG), HbA1c, and optimize blood glucose prior to initiation. After initiating, monitor FPG or fasting blood glucose at least once weekly for the first 2 weeks, then at least once every 4 weeks, and as indicated. Monitor HbA1c every 3 months and as indicated. Monitor FPG more frequently during first few weeks of treatment in those with risk factors for hyperglycemia (eg, obesity, elevated FPG, HbA1c >ULN, or age ≥75). Interrupt, reduce dose, or discontinue therapy based on severity of the hyperglycemia. Diabetes. Permanently discontinue if severe hypersensitivity occurs. Prior history of severe cutaneous reactions (eg, SJS, EM, TEN, DRESS): do not reintroduce. Interrupt therapy if signs/symptoms of severe cutaneous reactions occur; permanently discontinue if confirmed. Interrupt and evaluate if new or worsening respiratory symptoms occur or are suspected; permanently discontinue if pneumonitis is confirmed. Risk of severe diarrhea; interrupt, reduce dose, or discontinue therapy based on severity. Embryo-fetal toxicity. Pregnancy: exclude status prior to initiation. Advise females of reproductive potential and males (w. female partners) to use effective contraception during and for 1 week after last dose. Nursing mothers: not recommended (during and for 1 week after last dose).
VIJOICE Classification:
Phosphatidylinositol 3-kinase inhibitor.
VIJOICE Interactions:
May be antagonized by strong CYP3A4 inducers; avoid. May be potentiated by BCRP inhibitors; avoid or use alternatives; if unavoidable, monitor closely for toxicity. May antagonize CYP2C9 substrates; monitor closely. Risk for hyperglycemia with concomitant systemic corticosteroids.
Adverse Reactions:
Diarrhea, stomatitis, hyperglycemia, lab abnormalities; ketoacidosis, hypersensitivity reactions, acute interstitial pneumonitis and interstitial lung disease, dehydration, acute kidney injury.
Generic Drug Availability:
NO
How Supplied:
Blister pack—1×28 (50mg tabs); 1×28 (125mg tabs); 2×28 (200mg + 50mg tabs)
