Indications for: PYRUKYND
Hemolytic anemia in adults with pyruvate kinase (PK) deficiency.
Swallow whole. Initially 5mg twice daily. Titrate by every 4 weeks to 20mg twice daily, then to max 50mg twice daily according to titration schedule. Assess hemoglobin (Hb) and transfusion requirement before increasing to the next dose level. Discontinue therapy if no benefit is observed by 24 weeks based on lab results and transfusion requirements. Concomitant moderate CYP3A inhibitors: do not titrate beyond 20mg twice daily. Concomitant moderate CYP3A inducers (if no alternatives): titrate beyond 50mg twice daily if needed; max 100mg twice daily. Dose titration schedule or modifications for adverse reactions: see full labeling.
Risk of acute hemolysis with subsequent anemia. Avoid abrupt interruption or abrupt discontinuation. Moderate or severe hepatic impairment: avoid. Pregnancy. Nursing mothers.
Pyruvate kinase activator.
Potentiated by strong CYP3A inhibitors (eg, itraconazole, ketoconazole); avoid. Potentiated by moderate CYP3A inhibitors (eg, fluconazole); monitor Hb, adverse reactions, and avoid titrating (see Adult). Antagonized by strong CYP3A inducers (eg, rifampin); avoid. Antagonized by moderate CYP3A inducers (eg, efavirenz); consider alternatives; if no alternative therapies, titrate dose (see Adult). Antagonizes sensitive CYP3A substrates, including hormonal contraceptives (eg, ethinyl estradiol, midazolam); monitor drugs with narrow therapeutic index. Advise to use an alternative non-hormonal contraceptive or add a barrier method of contraception during treatment. May antagonize sensitive substrates of CYP2B6, CYP2C, UGT1A1 or potentiate P-gp substrates; monitor these drugs with narrow therapeutic index.
Decreased estrone (males), increased urate, back pain, decreased estradiol (males), arthralgia.
Generic Drug Availability:
Tabs—28; Blister wallet—7 (5mg); Blister wallet—14 (20mg×7 + 5mg×7); Blister wallet—14 (50mg×7 + 20mg×7)