Inborn errors of metabolism:

Recent Updates:

Monograph added.

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)

Pricing for VIJOICE

28 tablets of 50mg carton (Qty: 1)
Appx. price $33160
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