Biomarin Pharmaceutical Inc.
Manufacturer Details
There are currently no manufacturer details available.
Biomarin Pharmaceutical Inc. Drugs
-
![Vimizim (Elosulfase Alfa) Injection, Solution, Concentrate [Biomarin Pharmaceutical Inc.]](http://dailymed.nlm.nih.gov/dailymed/image.cfm?setid=0caa2565-12b2-0ad0-1f9a-273e81c3d4cc&name=vimizim-1.jpg)
Vimizim
2.1 Recommended Dose
The recommended dose is 2 mg per kg given intravenously over a minimum range of 3.5 to 4.5 hours, based on infusion volume, once every week. Pre-treatment with antihistamines with or without antipyretics is recommended 30 to 60 minutes prior to the start of the infusion [see Warnings and Precautions (5.1)].
Parenteral drug products should be inspected visually for particulate matter and discoloration prior to administration, whenever solution and container permit.
2.2 Preparation Instructions
Important Information: This product should be prepared and administered under the supervision of a healthcare professional with the ability to manage medical emergencies.
Determine the number of vials to be diluted based on the individual patient’s weight and the recommended dose of 2 mg/kg.
Dilute the calculated dose to a final volume of 100 mL or 250 mL using 0.9% Sodium Chloride Injection, USP.
The final volume is based on the patient’s weight as follows:
· For patients who weigh less than 25 kg, the final volume should be 100 mL;
· For patients who weigh 25 kg or more, the final volume should be 250 mL.
The solution should be clear to slightly opalescent and colorless to pale yellow when diluted. Do not use if the solution is discolored or if there is particulate matter in the solution. Note that a diluted solution with slight flocculation (e.g., thin translucent fibers) is acceptable for administration.
Avoid agitation during preparation. Gently rotate the bag to ensure proper distribution. Do not shake the solution.
2.3 Administration Instructions
Administer the diluted solution to patients using a low-protein binding infusion set equipped with a low-protein binding 0.2 micrometer (µm) in-line filter.
Note: The safety and effectiveness of Vimizim have not been established in pediatric patients less than 5 years of age [see Use in Specific Populations (8.4)].
For patients who weigh less than 25 kg: initial infusion rate should be 3 mL per hour for the first 15 minutes and, if tolerated, increased to 6 mL per hour for the next 15 minutes. If this rate is tolerated, then the rate may be increased every 15 minutes in 6 mL per hour increments, not to exceed 36 mL per hour. The total volume of the infusion should be delivered over a minimum of 3.5 hours.
For patients who weigh 25 kg or more: initial infusion rate should be 6 mL per hour for the first 15 minutes and, if tolerated, the infusion rate may be increased to 12 mL per hour for the next 15 minutes. If this rate is tolerated, then the rate may be increased every 15 minutes in 12 mL per hour increments, not to exceed 72 mL per hour. The total volume of the infusion should be delivered over a minimum of 4.5 hours.
The infusion rate may be slowed, temporarily stopped, or discontinued for that visit in the event of hypersensitivity reactions [see Warnings and Precautions (5.1)]. Do not infuse with other products in the infusion tubing. Compatibility with other products has not been evaluated.
2.4 Storage and Stability
Vimizim does not contain preservatives; therefore the product should be used immediately after dilution. If immediate use is not possible, the diluted product may be stored for up to 24 hours at 2°C to 8°C (36°F to 46°F) followed by up to 24 hours at 23°C to 27°C (73°F to 81°F). Administration of Vimizim should be completed within 48 hours from the time of dilution. Vials are for single-use only. Discard any unused product. Do not freeze or shake. Protect from light.
-
![Kuvan (Sapropterin Dihydrochloride) Tablet Kuvan (Sapropterin Dihydrochloride) Powder, For Solution [Biomarin Pharmaceutical Inc.]](http://dailymed.nlm.nih.gov/dailymed/image.cfm?setid=af38711e-8873-4790-a92d-4d583e23fb89&name=kuvan-13.jpg)
Kuvan
2.1 Dosage
Patients 1 month to 6 years: The recommended starting dose of Kuvan is 10 mg/kg taken once daily [see Warnings and Precautions (5.3)].
Patients 7 years and older: The recommended starting dose of Kuvan is 10 to 20 mg/kg taken once daily.
If a 10 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with Kuvan at 10 mg/kg per day for a period of up to 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically for up to a month. If blood Phe does not decrease from baseline at 10 mg/kg per day, the dose may be increased to 20 mg/kg per day. Patients whose blood Phe does not decrease after 1 month of treatment at 20 mg/kg per day are non-responders and treatment with Kuvan should be discontinued in these patients.
If a 20 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with Kuvan at 20 mg/kg per day for a period of 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically during the first month. Treatment should be discontinued in patients who do not respond to Kuvan.
Once responsiveness to Kuvan has been established, the dosage may be adjusted within the range of 5 to 20 mg/kg per day according to response to therapy. Periodic blood Phe monitoring is recommended to assess blood Phe control [see Warnings and Precautions (5.3, 5.6)].
2.2 Administration
Kuvan is available as tablets and as powder for oral solution. Kuvan should be taken orally with a meal to increase absorption, preferably at the same time each day. A missed dose should be taken as soon as possible, but two doses should not be taken on the same day.
2.3 Instructions for Use
Kuvan Tablets
Kuvan tablets may be swallowed either as whole tablets or dissolved in 120 to 240 mL of water or apple juice and taken orally within 15 minutes of dissolution. It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster, tablets may be stirred or crushed. The tablets may not dissolve completely. Patients may see small pieces floating on top of the water or apple juice. This is normal and safe for patients to swallow. If after drinking the medicine patients still see pieces of the tablet in the container, more water or apple juice can be added to make sure all of the medicine is consumed. Kuvan tablets may also be crushed and then mixed in a small amount of soft foods such as apple sauce or pudding.
Kuvan Powder for Oral Solution
Kuvan powder for oral solution should be dissolved in 120 to 240 mL of water or apple juice and taken orally within 30 minutes of dissolution. Kuvan powder for oral solution may also be stirred in a small amount of soft foods such as apple sauce or pudding. Empty the contents of the packet(s) in water, apple juice, or a small amount of soft foods and mix thoroughly. The powder should dissolve completely.
For infants weighing 10 kg or less, Kuvan can be dissolved in as little as 5 mL of water or apple juice and a portion of this solution corresponding to a 10 mg/kg dose may be administered orally via an oral dosing syringe. Table 1 provides dosing information for infants at the recommended starting dose of 10 mg/kg per day. Refer to Table 2 for dosing information at 20 mg/kg per day if dosage adjustment is needed.
Table 1: 10 mg/kg per day Dosing Table for Infants Weighing 10 kg or less
Patient
Weight (kg)
Starting Dose: 10 mg/kg per day*
Dose (mg)
# Kuvan
100 mg Packets Dissolved†
Dilution Volume (mL)‡
Administered
Dose volume (mL)§
1
10
1
10
1
2
20
1
10
2
3
30
1
10
3
4
40
1
10
4
5
50
1
10
5
6
60
1
5
3
7
70
1
5
3.5
8
80
1
5
4
9
90
1
5
4.5
10
100
1
5
5
*Starting dose for infants is 10 mg/kg per day. Dosing information for 20 mg/kg per day is provided in Table 2.
† Powder for oral solution provided in single use packets containing 100 mg Kuvan per packet
‡ Volume of water or apple juice to dissolve Kuvan Powder for Oral Solution.
§Discard remainder of mixture after volume to be administered is drawn.
Table 2: 20 mg/kg per day Dosing Table for Infants Weighing 10 kg or less
Patient
Weight (kg)
20 mg/kg per day
Dose (mg)
# Kuvan 100 mg Packets* Dissolved
Dilution Volume (mL)†
Administered Dose volume
(mL)§
1
20
1
5
1
2
40
1
5
2
3
60
1
5
3
4
80
1
5
4
5
100
1
5
5
6
120
2
5
3
7
140
2
5
3.5
8
160
2
5
4
9
180
2
5
4.5
10
200
2
5
5
† Powder for oral solution provided in single use packets containing 100 mg Kuvan per packet
† Volume of water or apple juice to dissolve Kuvan Powder for Oral Solution.
§Discard remainder of mixture after volume to be administered is drawn.
2.1 Dosage
Patients 1 month to 6 years: The recommended starting dose of Kuvan is 10 mg/kg taken once daily [see Warnings and Precautions (5.3)].
Patients 7 years and older: The recommended starting dose of Kuvan is 10 to 20 mg/kg taken once daily.
If a 10 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with Kuvan at 10 mg/kg per day for a period of up to 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically for up to a month. If blood Phe does not decrease from baseline at 10 mg/kg per day, the dose may be increased to 20 mg/kg per day. Patients whose blood Phe does not decrease after 1 month of treatment at 20 mg/kg per day are non-responders and treatment with Kuvan should be discontinued in these patients.
If a 20 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with Kuvan at 20 mg/kg per day for a period of 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically during the first month. Treatment should be discontinued in patients who do not respond to Kuvan.
Once responsiveness to Kuvan has been established, the dosage may be adjusted within the range of 5 to 20 mg/kg per day according to response to therapy. Periodic blood Phe monitoring is recommended to assess blood Phe control [see Warnings and Precautions (5.3, 5.6)].
2.2 Administration
Kuvan is available as tablets and as powder for oral solution. Kuvan should be taken orally with a meal to increase absorption, preferably at the same time each day. A missed dose should be taken as soon as possible, but two doses should not be taken on the same day.
2.3 Instructions for Use
Kuvan Tablets
Kuvan tablets may be swallowed either as whole tablets or dissolved in 120 to 240 mL of water or apple juice and taken orally within 15 minutes of dissolution. It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster, tablets may be stirred or crushed. The tablets may not dissolve completely. Patients may see small pieces floating on top of the water or apple juice. This is normal and safe for patients to swallow. If after drinking the medicine patients still see pieces of the tablet in the container, more water or apple juice can be added to make sure all of the medicine is consumed. Kuvan tablets may also be crushed and then mixed in a small amount of soft foods such as apple sauce or pudding.
Kuvan Powder for Oral Solution
Kuvan powder for oral solution should be dissolved in 120 to 240 mL of water or apple juice and taken orally within 30 minutes of dissolution. Kuvan powder for oral solution may also be stirred in a small amount of soft foods such as apple sauce or pudding. Empty the contents of the packet(s) in water, apple juice, or a small amount of soft foods and mix thoroughly. The powder should dissolve completely.
For infants weighing 10 kg or less, Kuvan can be dissolved in as little as 5 mL of water or apple juice and a portion of this solution corresponding to a 10 mg/kg dose may be administered orally via an oral dosing syringe. Table 1 provides dosing information for infants at the recommended starting dose of 10 mg/kg per day. Refer to Table 2 for dosing information at 20 mg/kg per day if dosage adjustment is needed.
Table 1: 10 mg/kg per day Dosing Table for Infants Weighing 10 kg or less
Patient
Weight (kg)
Starting Dose: 10 mg/kg per day*
Dose (mg)
# Kuvan
100 mg Packets Dissolved†
Dilution Volume (mL)‡
Administered
Dose volume (mL)§
1
10
1
10
1
2
20
1
10
2
3
30
1
10
3
4
40
1
10
4
5
50
1
10
5
6
60
1
5
3
7
70
1
5
3.5
8
80
1
5
4
9
90
1
5
4.5
10
100
1
5
5
*Starting dose for infants is 10 mg/kg per day. Dosing information for 20 mg/kg per day is provided in Table 2.
† Powder for oral solution provided in single use packets containing 100 mg Kuvan per packet
‡ Volume of water or apple juice to dissolve Kuvan Powder for Oral Solution.
§Discard remainder of mixture after volume to be administered is drawn.
Table 2: 20 mg/kg per day Dosing Table for Infants Weighing 10 kg or less
Patient
Weight (kg)
20 mg/kg per day
Dose (mg)
# Kuvan 100 mg Packets* Dissolved
Dilution Volume (mL)†
Administered Dose volume
(mL)§
1
20
1
5
1
2
40
1
5
2
3
60
1
5
3
4
80
1
5
4
5
100
1
5
5
6
120
2
5
3
7
140
2
5
3.5
8
160
2
5
4
9
180
2
5
4.5
10
200
2
5
5
† Powder for oral solution provided in single use packets containing 100 mg Kuvan per packet
† Volume of water or apple juice to dissolve Kuvan Powder for Oral Solution.
§Discard remainder of mixture after volume to be administered is drawn.
-
![Naglazyme (Galsulfase) Solution [Biomarin Pharmaceutical Inc.]](http://dailymed.nlm.nih.gov/dailymed/image.cfm?setid=59341250-deac-ed71-3823-a4f5d64dbd77&name=naglazyme-1.jpg)
Naglazyme
2.1 Recommended Dose
The recommended dosage regimen of NAGLAZYME is 1 mg per kg of body weight administered once weekly as an intravenous infusion.
Pretreatment with antihistamines with or without antipyretics is recommended 30 to 60 minutes prior to the start of the infusion [see Warnings and Precautions (5.2)].
The total volume of the infusion should be delivered over a period of time of no less than 4 hours. NAGLAZYME should be diluted with 0.9% Sodium Chloride Injection, USP, to a final volume of 250 mL and delivered by controlled intravenous infusion using an infusion pump. The initial infusion rate should be 6 mL per hour for the first hour. If the infusion is well tolerated, the rate of infusion may be increased to 80 mL per hour for the remaining 3 hours. The infusion time can be extended up to 20 hours if infusion reactions occur.
For patients 20 kg and under or those who are susceptible to fluid volume overload, physicians may consider diluting NAGLAZYME in a volume of 100 mL [see Warnings and Precautions (5.1) and Adverse Reactions (6.3)]. The infusion rate (mL per hour) should be decreased so that the total infusion duration remains no less than 4 hours.
Each vial of NAGLAZYME provides 5 mg of galsulfase (expressed as protein content) in 5 mL of solution and is intended for single use only. Do not use the vial more than one time. The concentrated solution for infusion must be diluted with 0.9% Sodium Chloride Injection, USP, using aseptic techniques. Prepare Naglazyme using low-protein-binding containers and administer the diluted NAGLAZYME solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 µm in-line filter. There is no information on the compatibility of diluted NAGLAZYME with glass containers.
2.2 Instructions for Use
Prepare and use NAGLAZYME according to the following steps. Use aseptic techniques.
Determine the number of vials to be used based on the patient's weight and the recommended dose of 1 mg per kg: Patient's weight (kg) x 1 mL/kg of NAGLAZYME = Total number of mL of NAGLAZYME Total number of mL of NAGLAZYME ÷ 5 mL per vial = Total number of vialsRound up to the next whole vial. Remove the required number of vials from the refrigerator to allow them to reach room temperature. Do not allow vials to remain at room temperature longer than 24 hours prior to dilution. Do not heat or microwave vials. Before withdrawing the NAGLAZYME solution from the vial, visually inspect each vial for particulate matter and discoloration. The NAGLAZYME solution should be clear to slightly opalescent and colorless to pale yellow. Some translucency may be present in the solution. Do not use if the solution is discolored or if there is particulate matter in the solution. From a 250 mL infusion bag of 0.9% Sodium Chloride Injection, USP, withdraw and discard a volume equal to the volume of NAGLAZYME solution to be added. If using a 100 mL infusion bag, this step is not necessary. Slowly withdraw the calculated volume of NAGLAZYME from the appropriate number of vials using caution to avoid excessive agitation. Do not use a filter needle, as this may cause agitation. Agitation may denature NAGLAZYME, rendering it biologically inactive. Slowly add the NAGLAZYME solution to the 0.9% Sodium Chloride Injection, USP, using care to avoid agitation of the solutions. Do not use a filter needle. Gently rotate the infusion bag to ensure proper distribution of NAGLAZYME. Do not shake the solution. Administer the diluted NAGLAZYME solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 µm in-line filter.NAGLAZYME does not contain preservatives; therefore, after dilution with saline, the infusion bags should be used immediately. If immediate use is not possible, the diluted solution must be stored refrigerated at 2°C to 8°C (36°F to 46°F) and administered within 48 hours from the time of dilution to completion of administration. Other than during infusion, do not store the diluted NAGLAZYME solution at room temperature. Any unused product or waste material must be discarded and disposed of in accordance with local requirements.
NAGLAZYME must not be infused with other products in the infusion tubing. The compatibility of NAGLAZYME in solution with other products has not been evaluated.
Sign Up for a Free Account