Why is this medication prescribed?

Gamifant is prescribed for the treatment of patients with primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent, or progressive disease or intolerance to conventional HLH therapy. HLH is a rare and potentially life-threatening condition characterized by severe inflammation and tissue damage.

Gamifant is a monoclonal antibody that targets interferon-gamma, a cytokine that plays a key role in the pathogenesis of HLH. By inhibiting interferon-gamma, Gamifant helps to reduce the excessive immune response and inflammation associated with HLH.

How should this medicine be used?

Gamifant (emapalumab-lzsg) is administered as an intravenous infusion. The dosage and administration instructions for Gamifant should be provided by a healthcare professional, typically based on the patient’s weight and specific clinical circumstances. Here’s a general overview of how Gamifant is used:

• Dosage: The recommended dosage of Gamifant for adults and pediatric patients weighing 12 kg (26.5 lbs) or more is 1.25 mg/kg administered as an intravenous infusion over approximately 1 hour. The infusion should be given twice a week until the patient no longer requires therapy for hemophagocytic lymphohistiocytosis (HLH) or until hematopoietic stem cell transplantation (HSCT) can be performed.
• Preparation: Gamifant is supplied as a lyophilized powder in single-dose vials for reconstitution. Before administration, the healthcare provider will reconstitute the lyophilized powder with the provided diluent according to the manufacturer’s instructions.
• Administration: Once reconstituted, Gamifant is administered as an intravenous infusion. The infusion should be given over approximately 1 hour through a dedicated line. The infusion rate should be adjusted if the patient experiences infusion-related reactions.
• Monitoring: During the infusion, healthcare providers should monitor patients closely for any signs of infusion-related reactions or adverse events. Vital signs and clinical status should be monitored throughout the infusion.
• Storage: Gamifant vials should be stored refrigerated at 2°C to 8°C (36°F to 46°F) until the time of use. Do not freeze. Once reconstituted, the solution should be used immediately or refrigerated at 2°C to 8°C (36°F to 46°F) and used within 24 hours.

It’s important for patients to follow their healthcare provider’s instructions carefully regarding the administration of Gamifant. They should also report any side effects or concerns to their healthcare provider promptly.

Other uses for this medicine

Gamifant (emapalumab-lzsg) is primarily used for the treatment of primary hemophagocytic lymphohistiocytosis (HLH) in patients with refractory, recurrent, or progressive disease, or those intolerant to conventional HLH therapy. However, there might be ongoing research exploring its potential use in other conditions.

What special precautions should I follow?

Special precautions should be followed when using Gamifant, including:

• Patient Monitoring: Patients receiving Gamifant should be closely monitored for signs of infection, infusion-related reactions, or other adverse reactions during and after treatment.
• Infections: Since Gamifant suppresses the immune system by targeting interferon-gamma, patients may be at increased risk of infections. Healthcare providers should assess patients for signs of infection and manage them appropriately.
• Hypersensitivity Reactions: Patients may experience hypersensitivity reactions to Gamifant. If a severe hypersensitivity reaction occurs, the infusion should be interrupted, and appropriate medical treatment should be initiated.
• Interference with Laboratory Tests: Gamifant may interfere with some laboratory tests, including but not limited to interferon-gamma release assays (IGRAs) used for tuberculosis screening. Healthcare providers should be aware of this potential interference when interpreting test results.
• Immunizations: Live vaccines should generally be avoided in patients receiving Gamifant due to the potential for increased susceptibility to infections. Patients should consult with their healthcare provider regarding appropriate vaccination strategies.
• Pregnancy and Breastfeeding: There is limited data on the use of Gamifant in pregnant or breastfeeding women. Healthcare providers should weigh the potential risks and benefits before administering Gamifant to pregnant or breastfeeding women.
• Pediatric Use: The safety and effectiveness of Gamifant in pediatric patients younger than 1 month of age have not been established.

It’s important for patients to discuss any existing medical conditions, medications, or concerns with their healthcare provider before starting treatment with Gamifant. Healthcare providers will provide guidance on appropriate precautions and monitoring during treatment.

What special dietary instructions should I follow?

As for special dietary instructions, there are no specific dietary restrictions associated with Gamifant. However, maintaining a balanced and nutritious diet can support overall health and immune function during treatment.

What should I do if I forget a dose?

If a dose of Gamifant is missed, patients should contact their healthcare provider for guidance. It’s important not to double the dose to make up for a missed one. The healthcare provider will provide instructions on when and how to administer the missed dose.

What side effects can this medication cause?

Gamifant (emapalumab-lzsg) can cause various side effects, ranging from mild to severe. Not all individuals will experience these side effects, and some may experience none at all. Common side effects of Gamifant may include:

• Infusion-related reactions: These may include fever, chills, flushing, rash, headache, nausea, vomiting, or other symptoms. These reactions typically occur during or shortly after infusion and may require slowing down the infusion rate or stopping the infusion altogether.
• Infections: Due to its immunosuppressive effects, Gamifant may increase the risk of infections. Patients should be monitored for signs and symptoms of infections such as fever, cough, sore throat, and difficulty breathing.
• Hypersensitivity reactions: Some patients may experience allergic reactions to Gamifant, which can range from mild to severe. Symptoms may include itching, hives, swelling, difficulty breathing, and anaphylaxis.
• Liver enzyme elevation: Gamifant may cause elevations in liver enzymes, which can indicate liver inflammation or damage. Patients should undergo regular monitoring of liver function tests during treatment.
• Decreased levels of certain blood cells: Gamifant may lead to decreased levels of certain blood cells, including platelets and red blood cells. This can result in symptoms such as easy bruising, bleeding, fatigue, and weakness.
• Other adverse reactions: Other less common side effects of Gamifant may include gastrointestinal disturbances (such as diarrhea or abdominal pain), respiratory symptoms, muscle pain, and dizziness.

It’s important for patients to report any side effects or concerns to their healthcare provider promptly. Healthcare providers will monitor patients closely for adverse reactions and provide appropriate management as needed.

What should I know about storage and disposal of this medication?

Storage and disposal of Gamifant:

• Storage: Gamifant should be stored refrigerated at 2°C to 8°C (36°F to 46°F) until the time of use. Do not freeze. Once reconstituted, the solution should be used immediately or refrigerated at 2°C to 8°C (36°F to 46°F) and used within 24 hours.
• Disposal: Any unused or expired Gamifant should be disposed of properly according to local regulations or guidelines. Patients should consult with their healthcare provider or pharmacist for instructions on the safe disposal of Gamifant.

In case of emergency/overdose

If you suspect an overdose or experience severe adverse reactions after receiving Gamifant, seek emergency medical attention immediately. Overdose may lead to an increased risk of adverse effects such as severe hypersensitivity reactions, liver toxicity, or other serious complications. Emergency medical treatment should be initiated promptly to address any overdose symptoms.

What other information should I know?

• Follow-up: Patients receiving Gamifant should undergo regular follow-up appointments with their healthcare provider for monitoring of treatment response and assessment of any adverse effects.
• Patient Education: Patients should be educated about the proper administration technique for Gamifant, including the importance of adhering to the prescribed dosage and infusion schedule. They should also be informed about potential side effects and instructed to report any unusual symptoms to their healthcare provider.
• Interactions: Patients should inform their healthcare provider about all medications, supplements, and herbal products they are taking before starting treatment with Gamifant, as certain medications may interact with Gamifant and affect its efficacy or safety.
• Pregnancy and breastfeeding: Pregnant or breastfeeding women should discuss the potential risks and benefits of Gamifant with their healthcare provider before starting treatment. There is limited data on the use of Gamifant in pregnant or breastfeeding women, and the decision to use Gamifant during pregnancy or breastfeeding should be made based on individual circumstances and in consultation with a healthcare provider.
• Storage and handling: Gamifant vials should be handled and stored properly according to the manufacturer’s instructions to maintain their stability and efficacy. Patients should avoid exposing Gamifant to extreme temperatures, light, or physical damage during storage and handling.

As always, patients should consult with their healthcare provider or pharmacist if they have any questions or concerns about the use of Gamifant or any other medication.