Mepsevii

About Vestronidase alfa-vjbk 

• Vestronidase alfa-vjbk is a prescribed medicine for pediatric and adult patients to treat a rare genetic disorder called mucopolysaccharidosis type VII (MPS VII) or Sly syndrome.
• MPS VII is a rare condition where the body lacks a specific enzyme needed to break down certain complex sugars. This leads to the accumulation of these sugars in various tissues and organs, causing a range of health issues.
• Vestronidase alfa-vjbk (Vestronidase alfa-vjbk) is a sterile, preservative-free, non-pyrogenic liquid for intravenous infusion, supplied in single-dose vials. Each mL contains Vestronidase alfa-vjbk (2mg), L-histidine (3.1mg), polysorbate 20 (0.1mg), sodium chloride (7.88mg), and sodium phosphate monobasic dihydrate
• Vestronidase alfa-vjbk works by providing the missing enzyme, which helps the body break down these sugars properly. By doing so, it can help alleviate some of the symptoms and improve the quality of life for individuals with MPS VII. 
• This treatment is typically administered under the supervision of a healthcare professional and may require regular injections or infusions. It’s important to consult with a healthcare provider for more specific information and guidance regarding the process. 

Strength: 

Injection: IV infusion of 10mg/5mL (2mg/mL) in a single-dose vial. 

Recommended Dosage:

The drug is administered by a health care professional every two week directly into the bloodstream through intravenous (a vein) infusion.

• The recommended dosage for Vestronidase alfa-vjbk is 4mg/kg of body weight. It is advised to administer premedication with a non-sedating antihistamine, with or without an anti-pyretic, approximately 30 to 60 minutes before starting the infusion. 
• The infusion should be conducted over approximately 4 hours, with the first hour involving the infusion of 2.5% of the total volume, followed by an increase in the infusion rate for the remaining volume over the next 3 hours, as tolerated. 
• Detailed information on preparation, administration, and storage can be found in the full prescribing information.
• Administer the first 2.5% of the diluted Vestronidase alfa-vjbk solution over the first hour of the infusion. Administer the remaining 97.5% of the diluted medicine solution over the next three hours.
• Use an infusion set with a 0.2 micron filter to administer the diluted Vestronidase alfa-vjbk solution. Do not flush the line containing the medicine. If necessary, add saline through a separate line to maintain sufficient intravenous flow.
• Do not infuse Vestronidase alfa-vjbk with other products in the infusion tubing. Use the medicine immediately after dilution and complete the infusion within 42 hours. Discard any unused product.

Important:

To ensure your safe and effective use of Vestronidase alfa-vjbk, it’s crucial to provide a comprehensive medical history, which includes any allergies, infections, or existing medical conditions. Make sure to disclose all medications you are currently taking, including over-the-counter drugs and herbal supplements. 

Additionally, inform your healthcare provider about your pregnancy status or any plans for pregnancy. Assess and discuss your ability to travel to a healthcare facility for your infusion sessions. Lastly, express your commitment to complying with the necessary monitoring requirements associated with Vestronidase alfa-vjbk treatment. This collective information is important for following up the treatment plan and ensuring your well-being throughout the process.

Warnings & Precautions

• Vestronidase alfa-vjbk can trigger a severe allergic reaction known as anaphylaxis. Patients must undergo close monitoring for anaphylactic signs and symptoms during and after each infusion. Patients must be under observation for 60 minutes following the administration. In the event of severe systemic reactions, such as anaphylaxis, the infusion should be promptly discontinued, and the patient should receive immediate and appropriate medical treatment.
• Common infusion reactions with Vestronidase alfa-vjbk include fever, chills, nausea, vomiting, and rash. These reactions are usually mild to moderate and can be managed with premedication and supportive care.
• It is recommended to inform your doctor if you are pregnant or breastfeeding. Do not take this medicine if you are pregnant or breastfeeding, unless it is advised by your doctor. Because, the safety and the efficacy is not yet established in such population. 
• Patients with weakened immune systems may be at higher risk for infections while on Vestronidase alfa-vjbk and should be closely monitored.
• Inform your healthcare professional if you have any  neurological conditions, as it may worsen neurological symptoms, including seizures and stroke, in patients with pre-existing neurological conditions.
• The impact of Vestronidase alfa-vjbk on the central nervous system symptoms of MPS VII has not been established. Inform your healthcare provider about all medications you’re taking before starting the treatment. 

Common Vestronidase alfa-vjbk  Side Effects:

• Infusion reactions ( Fever, chills, nausea, vomiting, rash and headache) 
• Injection site reactions 
• Hepatotoxicity 
• Anaphylaxis
• Neurological disorders (seizures and stroke)
• Immune system problems
• Blood clots
• Allergic reactions
• Skin reactions
• Eye problems
• Respiratory problems
• Gastrointestinal problems
• Muscle and bone pain
• Fatigue

Use in Specific Population

• Pregnancy and lactation information for Vestronidase alfa-vjbk indicates that there are no available data on its use in pregnant women. However, in animal studies, Vestronidase alfa-vjbk did not show adverse developmental outcomes at doses up to 1.6 and 10 times the recommended human dose in rats and rabbits, respectively. 
• In lactation, there is no data on the presence of Vestronidase alfa-vjbk in human or animal milk or its effects on breastfeeding infants. The decision to breastfeed while using Vestronidase alfa-vjbk should consider the benefits of breastfeeding and the mother’s clinical need for the medication.
• Vestronidase alfa-vjbk’s safety and effectiveness have been established in pediatric patients under 18 years of age, while its use in patients aged 65 and over has not been studied in elderly patients.

Storage and Handling

• Store Vestronidase alfa-vjbk at temperatures between 2°C to 8°C (36°F to 46°F) and ensure it does not freeze.
• If immediate use is not feasible, the diluted solution can be refrigerated at 2°C to 8°C (36°F to 46°F) for up to 36 hours. Following refrigeration, it can be stored at room temperature, not exceeding 25°C (77°F), for an additional 6 hours. 
• Avoid shaking the vial and protect it from exposure to light. 
• Remember that Vestronidase alfa-vjbk is a sterile product, so do not use it if the vial is damaged or the seal is broken. 
• Always employ aseptic techniques when handling Vestronidase alfa-vjbk and dilute it with sterile saline immediately before use. It’s crucial to use the diluted Vestronidase alfa-vjbk and discard any unused portions. Never reuse the vial or infusion set to maintain safety and effectiveness.