Galsulfase Dosage

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Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Mucopolysaccharidosis Type VI

1 mg/kg via IV infusion once a week

Pretreatment with antihistamines with or without antipyretics is recommended 30 to 60 minutes prior to the start of the infusion.

Usual Pediatric Dose for Mucopolysaccharidosis Type VI

5 years or older: 1 mg/kg via IV infusion once a week

Pretreatment with antihistamines with or without antipyretics is recommended 30 to 60 minutes prior to the start of the infusion.

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

Anaphylaxis and severe allergic reactions (some life-threatening) have been reported during and up to 24 hours after galsulfase infusion. If anaphylaxis or other severe allergic reactions occur, galsulfase should be discontinued at once and appropriate treatment should be started. Caution is recommended upon rechallenge in patients with previous anaphylaxis or other severe allergic reactions during galsulfase infusion; appropriately trained personnel and equipment for resuscitation (including epinephrine) should be available during infusion.

Because of the potential for infusion reactions, patients should receive antihistamines with or without antipyretics prior to infusion. Infusion reactions (some severe) have been reported despite routine pretreatment with antihistamines. No factors have been identified that predisposed patients to infusion reactions. Infusion reactions have been reported as early as the first week and as late as the 146th week of treatment. Recurrent infusion reactions have been reported in 70% of patients during multiple infusions, but not always in consecutive weeks. Infusion reaction symptoms generally abated with slowing or temporary interruption of the infusion along with administration of additional antihistamines, antipyretics, and occasionally corticosteroids. If a severe infusion occurs, the infusion should be discontinued immediately and appropriate treatment should be initiated. The risks and benefits of readministering galsulfase following a severe reaction should be considered.

Type III immune complex-mediated reactions, including membranous glomerulonephritis, have been reported with galsulfase and other enzyme replacement therapies. Galsulfase discontinuation should be considered if immune-mediated reactions occur and appropriate treatment should be started. The risks and benefits of readministering galsulfase following an immune-mediated reaction should be considered. Some patients have been successfully rechallenged and have continued to receive galsulfase under close clinical supervision.

Galsulfase should be administered with caution in patients susceptible to fluid volume overload. Such patients include patients weighing 20 kg or less, patients with acute underlying respiratory illness, or patients with compromised cardiac and/or respiratory function since congestive heart failure may develop. Appropriate medical support and monitoring measures should be available during infusion. Depending on individual patient needs, prolonged observation times may be required for some patients.

Sleep apnea is common in mucopolysaccharidosis VI patients. Antihistamine pretreatment may increase the risk of apneic episodes. Evaluation of airway patency should be considered prior to initiation of treatment. Patients using supplemental oxygen or continuous positive airway pressure during sleep should have these readily available during infusion in the event of an infusion reaction or extreme drowsiness/sleep induced by antihistamine use. Due to the possibility of acute respiratory compromise, delay of the galsulfase infusion should be considered in patients who present with an acute febrile or respiratory illness.

Dialysis

Data not available

Other Comments

The total volume of the infusion should be delivered over no less than 4 hours.

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