Procysbi

Adult Dosing .

Dosage forms:  DR CAP: 25 mg, 75 mg; DR GRANULE: 75 mg per pkt, 300 mg per pkt

Restricted Distribution in US

[1-855-888-4004 or www.procysbi.com for more info]

nephropathic cystinosis

[initial tx]

Dose: 1300 mg/m^2/day PO/GT divided q12h; Start: 200-300 mg/m^2/day PO/GT divided q12h, then may incr. dose over 4-6wk; Max: 1950 mg/m^2/day; Info: give >30min before or >2h after meals; avoid admin. w/ high fat meal; may open cap; do not crush/chew cap contents or granules; adjust dose by 10% to maintain WBC cystine level <1 nmol ½ cys/mg protein

[conversion from IR form]

Dose: 1300 mg/m^2/day PO/GT divided q12h; Max: 1950 mg/m^2/day; Info: give >30min before or >2h after meals; avoid admin. w/ high fat meal; may open cap; do not crush/chew cap contents or granules; adjust dose by 10% to maintain WBC cystine level <1 nmol ½ cys/mg protein

renal dosing

[not defined]

renal impairment: not defined

HD/PD: not defined

hepatic dosing

[not defined]

Peds Dosing .

Dosage forms:  DR CAP: 25 mg, 75 mg; DR GRANULE: 75 mg per pkt, 300 mg per pkt

Restricted Distribution in US

[1-855-888-4004 or www.procysbi.com for more info]

nephropathic cystinosis

[initial tx, 1-5 yo]

Dose: 1300 mg/m^2/day PO/GT divided q12h; Start: 200-300 mg/m^2/day PO/GT divided q12h, then may incr. dose no more frequently than q2wk; Max: 1950 mg/m^2/day; Info: give >30min before or >2h after meals; avoid admin. w/ high fat meal; may open cap; do not crush/chew cap contents or granules; adjust dose by 10% to maintain WBC cystine level <1 nmol ½ cys/mg protein

[initial tx, 6 yo and older]

Dose: 1300 mg/m^2/day PO/GT divided q12h; Start: 200-300 mg/m^2/day PO/GT divided q12h, then may incr. dose over 4-6wk; Max: 1950 mg/m^2/day; Info: give >30min before or >2h after meals; avoid admin. w/ high fat meal; may open cap; do not crush/chew cap contents or granules; adjust dose by 10% to maintain WBC cystine level <1 nmol ½ cys/mg protein

[conversion from IR form, 1-5 yo]

Dose: 1300 mg/m^2/day PO/GT divided q12h; Max: 1950 mg/m^2/day; Info: give >30min before or >2h after meals; avoid admin. w/ high fat meal; may open cap; do not crush/chew cap contents or granules; adjust dose by 10% no more frequently than q2wk to maintain WBC cystine level <1 nmol ½ cys/mg protein

[conversion from IR form, 6 yo and older]

Dose: 1300 mg/m^2/day PO/GT divided q12h; Max: 1950 mg/m^2/day; Info: give >30min before or >2h after meals; avoid admin. w/ high fat meal; may open cap; do not crush/chew cap contents or granules; adjust dose by 10% to maintain WBC cystine level <1 nmol ½ cys/mg protein

renal dosing

[not defined]

renal impairment: not defined

HD/PD: not defined

hepatic dosing

[not defined]