Inborn errors of metabolism:
Indications for: VIJOICE
Treatment of severe manifestations of PIK3CA-related overgrowth spectrum (PROS) in patients who require systemic therapy.
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.
<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.
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).
Phosphatidylinositol 3-kinase inhibitor.
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.
Diarrhea, stomatitis, hyperglycemia, lab abnormalities; ketoacidosis, hypersensitivity reactions, acute interstitial pneumonitis and interstitial lung disease, dehydration, acute kidney injury.
Generic Drug Availability:
Blister pack—1×28 (50mg tabs); 1×28 (125mg tabs); 2×28 (200mg + 50mg tabs)