Naglazyme

Dosage and Administration

Naglazyme is colourless to a pale yellow solution and clear to a bit opalescent single-dose vial. Naglazyme medicine comes in the form of an injection of the strength of 5 mg/5ml.

Dosage recommendation

• The dosage routine recommended by professionals is 1 mg per kg of body weight administered once weekly through intravenous infusion.
• Professionals recommend earlier treatment with antihistamines with or without antipyretics is 30 to 60 minutes before starting the infusion.
• Professionals should deliver the total volume of the infusion over a while of not less than 4 hours. Dilute the Naglazyme with 0.9% Sodium Chloride Injection to a final volume of 250 ml and deliver it by controlled intravenous infusion using an infusion pump. The starting rate of infusion should be 6 mL per hour for the first hour. If the patient tolerates this infusion, increase the infusion rate to 80 m per hour for the remaining 3 hours. If infusion reactions occur, extend the infusion time up to 20 hours.
• For patients weighing 20 kg and under or those who are susceptible to fluid volume overload, Physicians can consider diluting Naglazyme in a volume of 100 ml. Decrease the infusion rate so that the total infusion duration remains not less than 4 hours.
• Each Naglazyme vial provides 5 mg of galsulfase in 5 ml of solution and is advised for single use only. Do not use the vial more than once. Dilute the concentrated solution with 0.9% Sodium Chloride Injection, using aseptic techniques. Prepare the medicine using low-protein-binding containers and administer the diluted Naglazyme solution to patients using a low-protein-binding infusion set with a low-protein-binding 0.2 µm in-line filter.

Instructions for the use of Naglazyme
Prepare and use Naglazyme according to the steps mentioned below:

• For using the vials, calculate the number of vials needed, based on the weight of the patient, and the professionals recommend the dose of 1 mg/kg:
  Patient’s weight (kg) × 1 mL/kg of medicine = Total number of ml of the medicine.
  The total number of ml of Naglazyme ÷ 5 ml per vial = Total number of vials.
  Round up to the next whole vial. To allow vials to get at room temperature, remove the required number of vials from the refrigerator. Do not let vials remain at room temperature longer than 24 hours before dilution. Do not heat vials.
• Before withdrawing the solution from the vial, inspect each vial visually for particulate matter and discolouration. The Naglazyme solution should be clear to a bit opalescent and colourless to pale yellow. Professionals advise not to use it if the solution is discoloured or in the case of particulate matter in the Naglazyme solution.
• From a 250 ml infusion bag, withdraw and discard a volume equal to the volume of the medicine to be added. In the case of a 100 ml infusion bag, this step is not required.
• Slowly withdraw the calculated volume of the solution from the appropriate number of vials using caution to avoid excessive shaking of the solution. Do not use a filter needle because this can cause the whisking of the solution. Whisking may denature the solution, making it biologically inactive.
• Slowly add the solution to the 0.9% Sodium Chloride Injection, using care to avoid shaking the solutions. And do not use a filter needle.
• Slowly rotate the infusion bag to ensure proper distribution of the medicine. Do not shake the solution.
• Administer the diluted Naglazyme solution to the patient using a low-protein-binding infusion set provided along with a low-protein-binding 0.2 µm in-line filter.

This medicine does not contain preservatives; therefore, use the infusion bags immediately after the dilution. If it is not possible to use it immediately, store the diluted solution by refrigerating it at 2C-8C or 36F-46F and administer it within 48 hours from the dilution to completion of administration.

Side effects

Most of the medicines have some side effects, which can be ordinary and can be severe. These are categorized as:

Common
Abdominal Pain, Ear Pain, Arthralgia, Pain are common side effects of GALSULFASE.

Uncommon
Conjunctivitis, Dyspnea, Rash, Chills, Chest Pain, etc., are some uncommon side effects.

Rare
Pharyngitis, Areflexia, Corneal Opacity, Gastroenteritis, Hypertension, Malaise, Nasal Congestion, Umbilical Hernia, Hearing Impairment are rare side effects.

Precautions and Warnings

Anaphylaxis and serious hypersensitivity reactions
Professionals observed such reactions in patients during and up to 24 hours after the medicine infusion. Some of the reactions were life-threatening, with many worst symptoms. If anaphylaxis or other severe hypersensitivity reactions occur, discontinue the administration of medicine immediately, and give appropriate medical treatment to patients.

Type III immune complex-mediated reactions
Experts observed membranous glomerulonephritis with Type 3 while treatment with Naglazyme, as with other enzyme replacement therapies. If immune-mediated reactions occur, discontinue the administration of Naglazyme, and initiate appropriate medical treatment.

Risk of Acute Cardiorespiratory Failure
Exercise caution when administering Naglazyme to patients susceptible to fluid volume overloads, like patients with 20 kg or less weight, patients with acute underlying respiratory illness, or patients with compromised cardiac and respiratory function, as congestive heart failure can occur. Make appropriate medical support and monitoring measures readily available during drug infusion. Some patients may require longer observation times that should be based on the patient’s individual needs.

Acute Respiratory Complications Associated with Administration
It is seen that Sleep apnea is common in MPS VI patients, and earlier treatment with an antihistamine can increase the risk of apneic episodes. Patients who use supplemental oxygen or continuous positive airway pressure during sleep should have these treatments quickly available during infusion in the event of an infusion reaction.

Reactions due to infusion
Due to the potential for infusion reactions, patients should receive antihistamines with or without antipyretics before infusion. Despite routine earlier treatment with antihistamines, infusion reactions, some severe, occurred in 33 of 59 (56%) patients treated with Naglazyme. If severe infusion reactions occur, discontinue the infusion immediately, Naglazyme and start appropriate treatment.

Spinal or Cervical Cord Compression
Spinal or cervical cord compression (SCC) and resultant myelopathy is a serious complication of MPS VI. According to professionals, SCC is expected to occur in the natural history of the disease, including in patients treated with Naglazyme.

Handling and storage

Naglazyme injection is supplied as a sterile colourless to pale yellow, clear to a bit opalescent solution into a clear Type I glass 5 ml vial containing 5 mg of galsulfase per 5 ml solution. The closure contains a siliconized chlorobutyl rubber stopper and an aluminium seal with a plastic flip-off cap.

• Store Naglazyme under refrigeration at 2°C-8°C or 36°F to 46°F.
• Do not freeze or shake the solution.
• Please protect it from light.
• This medicine does not contain any preservatives.