Galsulfase

• Target Condition: Galsulfase is specifically indicated for the treatment of Mucopolysaccharidosis VI (MPS VI), a rare lysosomal storage disorder characterized by a deficiency of the enzyme N-acetylgalactosamine 4-sulfatase.
  
• Mechanism of Action: Galsulfase works by replacing the deficient enzyme, reducing the accumulation of glycosaminoglycans (GAGs) in tissues, which is responsible for the progressive damage seen in MPS VI.
  
• Clinical Benefits: The primary benefit of Galsulfase treatment is an improvement in physical endurance, particularly in walking (as measured by the 12-minute walk test) and stair-climbing capacity, which are significant quality-of-life indicators for MPS VI patients.
  
• Urinary GAG Reduction: While Galsulfase significantly reduces urinary GAG levels, it does not completely normalize them. The reduction, however, correlates with clinical improvements, providing symptomatic relief.

Recommended Dosage:

• Standard Dosage: The recommended dose is 1 mg/kg of body weight, administered once weekly via intravenous infusion.
  
• Preparation and Dilution: Galsulfase must be diluted in 0.9% Sodium Chloride Injection, USP, to achieve a final volume of 250 mL for administration. For patients weighing 20 kg or less, a dilution to 100 mL may be considered.
  
• Infusion Rate: The infusion should start at a rate of 6 mL/h for the first hour. If well tolerated, the rate can be increased to 80 mL/h for the remaining three hours. In cases of infusion reactions, the infusion rate can be further reduced, and the infusion duration extended to up to 20 hours.
  
• Pre-Infusion Preparation: It is recommended to pretreat patients with antihistamines and, if necessary, antipyretics 30 to 60 minutes before starting the infusion to minimize the risk of infusion-related reactions.
  

• Healthcare professionals observed some 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 medicine administration immediately, and give appropriate medical treatment to patients.
• Experts observed membranous glomerulonephritis with Type 3 while treatment with Naglazyme 1 mg, as with other enzyme replacement therapies. If immune-mediated reactions occur, discontinue the administration of Naglazyme, and initiate appropriate medical treatment.
• 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.
• 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.
• 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 (SCC) and resultant myelopathy are serious complications of MPS VI. According to professionals, SCC is expected to occur in the natural history of the disease, including in patients treated with Naglazyme.

• Infusion Reactions: Patients may experience infusion-related reactions, which can range from mild to severe. Severe reactions may include angioneurotic edema, hypotension, bronchospasm, and respiratory distress. In such cases, the infusion should be stopped immediately, and appropriate medical interventions should be initiated.
• Sleep Apnea Considerations: Patients with MPS VI often suffer from sleep apnea. Since antihistamines used before the infusion may exacerbate apnea, these patients should be monitored closely during the infusion, with supplemental oxygen or CPAP available if needed.
• Acute Illness Management: It is advisable to delay Galsulfase infusions in patients presenting with acute febrile or respiratory illnesses to avoid exacerbating these conditions during treatment. 

If you are considering the import of Galsulfase to India, please be aware that it can be imported by patients or government hospitals solely in the name of the patients. The following documentation is essential for the successful import of this medication:

Required Documentation for Import:

• A valid prescription from a qualified medical practitioner.
  
• Diagnostic reports of the patient.
  
• Government-issued identification proof for the patient, as recognized by the Government of India.

Order Confirmation Process: 

• The order for Galsulfase will be confirmed upon the receipt of the following documents:
  
• A valid prescription from a qualified doctor.
  
• An import permit, if applicable.

Availability of Galsulfase in India: 

Galsulfase is classified as a prescription pharmaceutical drug. Consequently, it legally requires a medical prescription to be dispensed. Indian Pharma Network (IPN) specializes in facilitating the import of cancer medicines through named patient supply (NPS), ensuring transparency and authenticity by sourcing from reputable suppliers in the USA, Canada, Europe, and Australia.

Galsulfase  can be made accessible to patients, doctors, and hospitals in various cities across India, including but not limited to Mumbai, Kolkata, Hyderabad, Chennai, Ahmedabad, Delhi, Bangalore, Srinagar, Jammu, Jaipur, Chandigarh, Ludhiana, Noida, Gurgaon, Lucknow, and Pune. To confirm an order, a valid prescription from a doctor and an import permit will be required.

IPN (Indian Pharma Network) is dedicated to facilitating the supply of Galsulfase  (prescription medicines) to locations both within India and worldwide, while adhering to relevant legal requirements.

For inquiries regarding the price of Galsulfase in India and to initiate the ordering process, please reach out to us through the following contact details:

Our commitment lies in assuring quality and timely delivery to meet the healthcare needs of patients and medical professionals across the globe.

Indian Pharma Network is proficient in sourcing Galsulfase  from around the world and ensuring efficient delivery to patients. We provide worldwide access to the best available treatments and expedite prescription dispensing and delivery, with all prescriptions being dispensed and scrutinized by registered pharmacists before dispatching them to the patient’s address, exclusively from New Delhi, India.

What is the mechanism of action of Naglazyme?

Mucopolysaccharide storage disorders are caused by the deficiency of specific lysosomal enzymes required for the catabolism of GAG. Naglazyme is intended to provide an exogenous enzyme that will be taken up into lysosomes and increase the catabolism of GAG. The drug Galsulfase uptake by cells into lysosomes is most likely mediated by the binding of mannose-6-phosphate-terminated oligosaccharide chains of Galsulfase to specific mannose-6-phosphate receptors. 

What is the route of administration for Naglazyme?

Naglazyme infusion is intravenously. The recommended dosage regimen of NAGLAZYME is 1 mg per kg of body weight, administered once weekly.

What does Naglazyme treat?

Naglazyme (Galsulfase) treats patients with Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome).

What is the price of  Naglazyme in India?

To know the Naglazyme price in India, you can call or WhatsApp +91-9310090915 or email info@indianpharmanetwork.in.

What is the dosage and form of Naglazyme available?

Naglazyme is available for Injection: 100 mg lyophilized powder in a single-dose vial. 

How does Galsulfase improve long-term quality of life in MPS VI patients?

Galsulfase enhances physical endurance and reduces GAG levels, leading to better mobility and overall quality of life in MPS VI patients.

 Can Galsulfase be administered at home?

While typically given in a clinical setting, home administration of Galsulfase may be possible with proper training and supervision.

 What can patients expect during the initial Galsulfase treatment?

Patients might experience mild infusion reactions like fever or chills, which usually decrease over time with proper management.

Does Galsulfase interact with other medications?

No formal studies exist, but patients should inform their healthcare provider about all medications to avoid potential interactions.

Are there dietary restrictions during Galsulfase therapy?

No specific dietary restrictions are needed, but maintaining a balanced diet and staying hydrated is recommended.

Is galsulfase FDA approved?

Yes, Galsulfase is approved by the FDA.