Vimizim

Dosage and administration of Vimizim

The professionals recommend the Vimizim vial dosage of 2 mg/kg given intravenously over a minimum range of 3.5-4.5 hours, based on infusion volume, once every week. Professionals recommend early treatment with antihistamines with or without antipyretics for 30 to 60 minutes before the start of the infusion.
Inspect visually parenteral drug products for particulate matter and discoloration before administration, whenever solution and container permit.

Instructions for preparation

Calculate the number of vials to be diluted based on the individual weight of the patient, and the recommended dosage is 2 mg/kg. Dilute the dose calculated to a final volume of 100 ml or 250 ml using sodium chloride injection. The final volume is based on the weight of the patient as:

• For patients weighing less than 25 kg

The final volume should be 100 ml.

• For patients weighing 25 kg or more than 25

The final volume of solution should be 250 ml; the diluted solution should be clear to a bit opalescent and colorless to pale yellow. Do not use the discolored solution or if there is particulate matter in the solution. Note that a slight crystallization is acceptable for administration in a diluted solution.

Avoid shaking the solution during preparation. Slowly rotate the bag to ensure proper distribution. Experts advise not to shake the solution.

Instruction for administration

Administer the Vimizim vial to patients using a low-protein binding infusion set with a low protein-binding 0.2-micrometer in-line filter.

For patients weighing less than 25 kg: starting infusion rate should be 3 ml per hour for the first 15 minutes and, if the patient tolerates this, increase the infusion rate to 6 ml per hour for the next 15 minutes. If the patient tolerates this, then increase the rate every 15 minutes to 6 ml per hour, do not exceed the rate of more than 36 ml per hour. Deliver the total volume of the infusion for at least three and a half hours.

For patients weighing 25 kg or more: starting infusion rate should be 6 mL per hour for the first 15 minutes and, if the patient tolerates this, then increase the infusion rate to 12 mL per hour for the next 15 minutes. If the patient tolerates this too, professionals can increase the rate every 15 minutes to 12 ml per hour, not exceeding the infusion rate of 72 mL per hour. Deliver the total volume of the infusion over a minimum of 4.5 hours.

Healthcare professionals can slow the infusion rate, temporarily stopped, or discontinued. Do not infuse the medicine with other products in the infusion tubing.

Overdosage

There is no experience of overdose with Vimizim 5mg/5ml yet. If any overdose happens, consult the doctor and keep monitoring the patient for any worsening symptoms.

Side effects of Vimizim

Frequent
Pyrexia, vomiting, etc., are frequent side effects of Vimizim.

Less frequent
Headache, nausea, abdominal pain, etc., are less frequent side effects observed while treatment with Vimizim.

Rare
Chills, fatigue, etc., are known rare side effects of Vimizim.

Precautions and Warnings

Case of Anaphylaxis and hypersensitivity reactions

There are reports on Anaphylaxis and hypersensitivity reactions in patients treated with elosulfase alfa. In premarketing clinical trials, 18 of 235 patients treated with this medicine experienced signs and symptoms consistent with Anaphylaxis. These 18 patients experienced 26 anaphylactic reactions while injecting the drug with signs and symptoms such as cough, erythema, throat tightness, urticaria, flushing, cyanosis, hypotension, rash, dyspnea, chest discomfort, and gastrointestinal symptoms in conjunction with urticaria.

In clinical trials with Elosulfase alfa, 44 of 235 patients experienced hypersensitivity reactions, including Anaphylaxis.

Case of Acute Respiratory Complications

Patients with respiratory illness at the time of Vimizim infusion can be at higher risk of life-threatening complications from hypersensitivity reactions. Careful consideration should be given to the patient’s clinical status before administration of Vimizim and consider delaying the Vimizim infusion if possible.

Spinal or Cervical Cord Compression

Spinal or cervical cord compression is a serious complication of MPS IVA and can occur as part of the natural history of the disease. Monitor the patients with MPS IVA for signs and symptoms of SCC (like back pain, paralysis of limbs below the level of compression, urinary and fecal incontinence) and give patients appropriate clinical care.

Handling and storage of Vimizim

Vimizim comes as a concentrated solution for infusion (1 mg per mL), requiring dilution. One vial of 5 ml contains 5 mg Vimizim.

• Refrigerate Vimizim at 2°C to 8°C or 36°F to 46°F.
• Do not freeze or shake the solution.
• Protect the solution from light.
• Use the diluted Vimizim immediately.
• If immediate use is not possible, one can store the diluted Vimizim for up to 24 hours at 2°C-8°C or 36°F-46°F followed by up to 24 hours at 23°C-27°C or 73°F-81°F while administered.