Posology : מינונים

2 DOSAGE AND ADMINISTRATION
2.1 Patient Selection
Start NULIBRY if the patient has a diagnosis or presumptive diagnosis of MoCD Type A.
In patients with a presumptive diagnosis of MoCD Type A, confirm the diagnosis of MoCD Type A immediately after initiation of NULIBRY treatment. In such patients, discontinue NULIBRY if the MoCD Type A diagnosis is not confirmed by genetic testing.
2.2 Important Administration Information
•   NULIBRY is intended for administration by a healthcare provider. If deemed appropriate by a healthcare provider, NULIBRY may be administered at home by the patient’s caregiver. If NULIBRY can be administered by a caregiver/patient, advise them to read the detailed instructions on the preparation, administration, storage, and disposal of NULIBRY for caregivers [see Instructions for Use].
•   NULIBRY is for intravenous infusion only. Administer with non-DEHP tubing with a 0.2 micron filter. Do not mix NULIBRY with other drugs (note NULIBRY is reconstituted with Sterile Water for Injection, USP). Do not administer as an infusion with other drugs.
•   NULIBRY is given through an infusion pump at a rate of 1.5 mL per minute.
•   Dose volumes below 2 mL may require syringe administration through slow intravenous push.
•   Administration of NULIBRY must be completed within 4 hours of reconstitution [see Dosage and Administration (2.5)].
2.3 Recommended Dosage and Administration
Recommended Dosage and Administration in Patients Less Than One Year of Age (by gestational age)
The recommended dosage regimen of NULIBRY in patients less than one year of age (by gestational age) is based on actual body weight as shown in Table 1.



Table 1    Recommended Initial Dosage and Titration Schedule of NULIBRY for Patients Less Than One Year of Age by Gestational Age
Preterm Neonates                   Term Neonates
Titration Schedule     (Gestational Age Less than 37       (Gestational Age 37 Weeks Weeks)                           and Above)

Initial Dosage              0.4 mg/kg once daily              0.55 mg/kg once daily 
Dosage at Month 1           0.7 mg/kg once daily              0.75 mg/kg once daily 
Dosage at Month 3           0.9 mg/kg once daily               0.9 mg/kg once daily 
Recommended Dosage and Administration in Patients One Year of Age or Older For patients one year of age or older, the recommended dosage of NULIBRY is 0.9 mg/kg (based on actual body weight) administered as an intravenous infusion once daily.
Recommendations for a Missed Dose
If a NULIBRY dose is missed, administer the missed dose as soon as possible. Administer the next scheduled dose at least 6 hours after the administration of the missed dose.
2.4 Preparation and Administration Instructions
NULIBRY must be reconstituted prior to use. Use aseptic technique during preparation and follow these instructions:
1. Determine the total dose, number of vials needed, and total reconstituted dose volume based on the patient's weight and prescribed dose.
2. Remove the required number of vials from the freezer to allow them to reach room temperature (by hand warming for 3 to 5 minutes or exposing to ambient air for approximately 30 minutes).
3. Reconstitute each required NULIBRY vial with 5 mL of Sterile Water for Injection, USP.
Gently swirl the vial continuously until the powder is completely dissolved. DO NOT shake. After reconstitution, the final concentration of NULIBRY reconstituted solution is 9.5 mg/5 mL (1.9 mg/mL).
4. Parenteral drug products should be inspected visually for particulate matter and discoloration prior to administration, whenever solution and container permit.
Reconstituted NULIBRY is a clear and colorless to pale yellow solution. Do not use if there are particles present or if the solution is discolored.
5. Administer the total reconstituted dose.
2.5 Storage of Reconstituted Solution
Reconstituted NULIBRY may be stored at room temperature [15°C to 25°C] or refrigerated [2°C to 8°C] for up to 4 hours including infusion time. If reconstituted NULIBRY is refrigerated, allow it to come to room temperature (by hand warming for 3 to 5 minutes or exposing to ambient air for approximately 30 minutes) before administration. Do not heat. Do not re-freeze NULIBRY after reconstitution. Do not shake.
Discard all unused reconstituted NULIBRY solution 4 hours after reconstitution.
Chemical and physical in-use stability has been demonstrated for 4 hours at 2-8 °C or 15-25 °C.
From a microbiological point of view, the product should be used immediately. If not used immediately, in-use storage times and conditions prior to use are the responsibility of the user and would normally not be longer than the above mentioned conditions when reconstitution has taken place in controlled and validated aseptic conditions.