Weekly NAGLAZYME treatment is life-long and compliance is critically important. However, venipuncture can be a painful procedure and can cause much anxiety in patients, especially children. Watch our video to learn how to make the treatment more comfortable.
Following are the steps for setting up the equipment for infusing NAGLAZYME. If you are using plain saline as a primary line, connect the NAGLAZYME tubing at the port closest to the patient.
Use an infusion pump for NAGLAZYME administration.
Infuse the total volume of NAGLAZYME solution over no less than 4 hours as follows:
For 250-mL volume:
6 mL/hour for the first hour. If the infusion is well tolerated, the infusion rate can be increased to 80 mL/hour for approximately 3 hours.
For 120-mL volume:
3 mL/hour for the first hour. If the infusion is well tolerated, the infusion rate can be increased to 39 mL/hour for approximately 3 hours.
Note: Patients and families often ask if the infusion can be administered in a shorter amount of time. For maximum safety and efficacy, do not administer NAGLAZYME at a faster rate than recommended.
Monitor vital signs:
When monitoring vital signs, look for signs of infusion‐associated reactions (IARs), such as:
Other mild reactions (including shivering, rashes, or swelling of the lips) may progress rapidly if not treated.
The first IARs occurred as late as 146 weeks in the clinical studies. Therefore it is important that:
If symptoms subside, consider restarting the infusion at a slower rate (eg, half the rate at which the IAR occurred).
The physician should evaluate the risks and benefits of readministering NAGLAZYME following a severe hypersensitivity or anaphylactic reaction.
Note: Caution should be exercised if epinephrine use is being considered in patients with MPS VI, due to increased prevalence of coronary artery disease.
Please see Important Safety Information below and full Prescribing Information for more information.
Other mild reactions may include shivering, rashes, or swelling of the lips.
IMPORTANT SAFETY INFORMATION
Life-threatening anaphylactic reactions and severe allergic reactions have been observed in some patients during NAGLAZYME infusions and up to 24 hours after infusion. If these reactions occur, immediate discontinuation of NAGLAZYME is recommended and appropriate medical treatment should be initiated, which may include resuscitation, epinephrine, administering additional antihistamines, antipyretics or corticosteroids. In patients who have experienced anaphylaxis or other severe allergic reactions during infusion with NAGLAZYME, caution should be exercised upon rechallenge; appropriately trained personnel and equipment for emergency resuscitation (including epinephrine) should be available during infusions.
As with other enzyme replacement therapies, immune-mediated reactions, including membranous glomerulonephritis have been observed. In clinical trials, nearly all patients developed antibodies as a result of treatment with NAGLAZYME; however, the analysis revealed no consistent predictive relationship between total antibody titer, neutralizing or IgE antibodies, and infusion-associated reactions, urinary glycosaminoglycan (GAG) levels, or endurance measures.
Caution should be exercised when administering NAGLAZYME to patients susceptible to fluid volume overload because congestive heart failure may result. Consider a decreased total infusion volume and infusion rate when administering NAGLAZYME to these patients.
Consideration to delay NAGLAZYME infusion should be given when treating patients who present with an acute febrile or respiratory illness. Sleep apnea is common in MPS VI patients and antihistamine pretreatment may increase the risk of apneic episodes. Evaluation of airway patency should be considered prior to the initiation of treatment. Patients using supplemental oxygen or continuous positive airway pressure (CPAP) during sleep should have these treatments readily available during infusion in the event of an infusion reaction, or extreme drowsiness/sleep induced by antihistamine use.
Pretreatment with antihistamines with or without antipyretics is recommended prior to the start of infusion to reduce the risk of infusion reactions. If infusion reactions occur, decreasing the infusion rate, temporarily stopping the infusion, or administering additional antihistamines and/or antipyretics is recommended.
During infusion, serious adverse reactions included laryngeal edema, apnea, pyrexia, urticaria, respiratory distress, angioedema, and anaphylactoid reaction; severe adverse reactions included urticaria, chest pain, rash, abdominal pain, dyspnea, apnea, laryngeal edema, and conjunctivitis. The most common adverse events (≥10%) observed in clinical trials in patients treated with NAGLAZYME were rash, pain, urticaria, pyrexia, pruritus, chills, headache, nausea, vomiting, abdominal pain and dyspnea. The most common adverse reactions requiring interventions are infusion-related reactions.
Spinal/cervical cord compression is a known and serious complication that is expected to occur during the natural course of MPS VI. Signs and symptoms of spinal/cervical cord compression include back pain, paralysis of limbs below the level of compression, and urinary or fecal incontinence. Patients should be evaluated for spinal/cervical cord compression prior to initiation of NAGLAZYME to establish a baseline and risk profile. Patients treated with NAGLAZYME should be regularly monitored for the development or progression of spinal/cervical cord compression and be given appropriate clinical care.
Please see full Prescribing Information.
NAGLAZYME® (galsulfase) is indicated for patients with mucopolysaccharidosis VI (MPS VI; Maroteaux-Lamy syndrome). NAGLAZYME has been shown to improve walking and stair-climbing capacity.