Procysbi Granules

<1yr: not established. Initiate immediately after diagnosis. Round dose to nearest available strength of caps or packets. Take on an empty stomach. Swallow caps whole with water or fruit juice (except grapefruit). Sprinkle and mix contents of cap (if unable to swallow whole) or intact granules from packet on 4oz of food (eg, applesauce or berry jelly) or 4oz of fruit juice (except grapefruit); also, may administer via feeding tube after mixing with 4oz of applesauce. Cysteamine-naive: initiate at ⅙ to ¼ of the maintenance dose; (1–<6yrs): increase gradually in 10% increments allowing at least 2 weeks between dose adjustments; (≥6yrs): increase gradually over 4–6 weeks. Maintenance: 1.3g/m²/day, in 2 divided doses given every 12hrs; may further increase to achieve therapeutic target WBC cystine level. Max 1.95g/m²/day (if >50kg: max 1000mg every 12hrs). Goal of therapy: to maintain WBC cystine level <1nmol ½ cystine/mg protein (with mixed leukocyte assay). Switching from immediate-release cysteamine: give previous total daily dose of immediate-release formulation. Dose titration, measuring WBC cystine levels: see full labeling.