Naglazyme is a formulation of galsulfase. It is a purified human enzyme produced by recombinant DNA technology in a Chinese hamster ovary cell line. GALSULFASE, a lysosomal enzyme that catalyzes the cleavage of the sulfate ester from terminal N–acetylgalactosamine 4-sulfate residues of glycosaminoglycans, chondroitin 4- sulfate and dermatan sulfate. The inactive ingredients are sodium phosphate monobasic monohydrate, sodium phosphate dibasic heptahydrate, polysorbate 80 and an extractable solution.
Naglazyme is used in treating patients with Mucopolysaccharidosis type IVA, also known as Morquio A syndrome. It is a rare metabolic disorder in which the body cannot produce certain sugar molecules called glycosaminoglycans. There are clinical studies that prove that patients treated with Naglazyme improved walking and stair-climbing capacity.
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.
Instructions for the use of Naglazyme
Prepare and use Naglazyme according to the steps mentioned below:
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.
Most of the medicines have some side effects, which can be ordinary and can be severe. These are categorized as:
Abdominal Pain, Ear Pain, Arthralgia, Pain are common side effects of GALSULFASE.
Conjunctivitis, Dyspnea, Rash, Chills, Chest Pain, etc., are some uncommon side effects.
Pharyngitis, Areflexia, Corneal Opacity, Gastroenteritis, Hypertension, Malaise, Nasal Congestion, Umbilical Hernia, Hearing Impairment are rare side effects.
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.
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.
Naglazyme is a formulation of galsulfase. Naglazyme is used in treating patients with Mucopolysaccharidosis type IVA. It comes in the form of an injection of the strength of 5 mg/5ml.
The active ingredient of Naglazyme is galsulfase and inactive ingredients are sodium phosphate monobasic monohydrate, sodium phosphate dibasic heptahydrate, polysorbate 80 and an extractable solution.
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.
For Naglazyme medicine, Biomarin is the innovator. Ikris Pharma Network is an authorized partner of it, who can help access this medicine in India for the patient through legal procedure. To know more, contact Ikris at toll-free no. 18008891064 or write at email@example.com.
In India, you can buy Naglazyme with the help of Ikris Pharma Network, an authorized partner of “Biomarin” for this medicine.