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Orfadin

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Orfadin

INDICATIONS

ORFADIN® capsules (nitisinone) are indicated as an adjunct to dietary restriction of tyrosine and phenylalanine in the treatment of patients with hereditary tyrosinemia type 1 (HT-1).

DOSAGE AND ADMINISTRATION

Dosage

The recommended dose of ORFADIN is 1 to 2 mg/kg divided into two daily doses. The initial dose is 1 mg/kg/day divided for morning and evening administration.

Treatment with ORFADIN should be initiated by a physician experienced in the treatment of HT-1. The dose of ORFADIN may be adjusted in each patient. In patients whose erythrocyte PBG-synthase activity and urine 5-ALA and urine succinylacetone are not normalized within one month after the start of ORFADIN treatment, the ORFADIN dose may be increased to 1.5 mg/kg/day. In patients receiving 1.5 mg/kg/day, whose erythrocyte PBG-synthase activity and urine 5-ALA and urine succinylacetone remain elevated and whose plasma succinylacetone is not normalized after three months, the dose may be increased to up a maximum dose of 2 mg/kg/day.

If plasma nitisinone concentration, plasma succinylacetone, urine 5-ALA and erythrocyte PBG-synthase activity are not available, clinical laboratory assessments should include urine succinylacetone, liver function tests, alpha-fetoprotein, and serum tyrosine and phenylalanine level. During initiation of therapy and during acute exacerbations, it may be necessary to follow more closely all available biochemical parameters [see WARNINGS AND PRECAUTIONS].

Administration

ORFADIN should be taken at least one hour before or at least two hours after a meal, since food effect is unknown. For young children, ORFADIN capsules may be opened and the contents suspended in a small amount of water immediately before use.

Physicians should counsel patients and their parents or caregivers of the need to maintain dietary restriction of tyrosine and phenylalanine when taking ORFADIN to treat hereditary tyrosinemia type 1.

HOW SUPPLIED

Dosage Forms And Strengths

ORFADIN capsules (nitisinone) are available as white capsules imprinted with “NTBC” followed by “2 mg”, “5 mg”, or “10 mg”, indicating the actual amount of nitisinone in each capsule.

Storage And Handling

ORFADIN capsules are white and marked in black with “NTBC” and identified as 2 mg, 5 mg or 10 mg strengths of nitisinone. The capsules are packed in a high density (HD) polyethylene container with a tamper-resistant low density (LD) polyethylene snap-on cap. Each bottle contains 60 capsules.

2 mg white capsules imprinted “NTBC 2 mg” in black ink, NDC 66658-102-60

5 mg white capsules imprinted “NTBC 5 mg” in black ink, NDC 66658-105-60

10 mg white capsules imprinted “NTBC 10 mg” in black ink, NDC 66658-110-60

Store refrigerated, 2-8°C (36-46°F).

For Drug or Ordering information please call Dohmen Life Science Services at 1-877-473-3179.

Manufactured by: Apotek Produktion & Laboratorier AB, Sweden. Marketed by: Sobi, Inc 890 Winter Street Waltham, MA 02451. Revised: May 2014

Last reviewed on RxList: 7/15/2014
This monograph has been modified to include the generic and brand name in many instances.

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