Drug Interaction:
No information available
Indication:
Mucoploysaccharidosis
Adverse Reaction:
Cardiovascular- hypertension 11%
GI - abdominal pain 53% gastroenteritis 11%
Respiratory - dyspnea 21% nasal congestion 11% pharyngitis 16%
Special senses- conjunctivitis 21% ear pain 42% increased corneal opacification 11%
Miscellaneous- areflexia 11% chest pain 16% face edema 11% malaise 11%
pain 26% rogors 21% umbilical hernia 11%
Contra-Indications:
Special precautions-
Infusion reaction- because of potential for infusion reactions give patients antihistamines
with or without antipyretics prior to infusion.
Nausea, vomiting, elevated blood pressure,retroternal pain, abdominal pain, malaise, and joint
pain were also reported.ntial reactions were observed as late as week 55 of treatment
Symptoms abated with slowing or temporory interruption of the infusion and administration of
additional prophylactic corticosteroids
Sleep apnea- sleep apnea is common in MPS VI patients and antihistamine pretreatment may
increase risk of apneic eisodes. Evaluation of airway patency should be considered prior to
initiation of treatment. Patients using supplemental oxygen or continous positive airway
pressure (CPAP ) during sleep should have these treatments readily available during infusion.
in theevent of an infusion reaction.
Acute febrile or respiratory illness- consider delaying galsulfate infusion in patients who present
with an acute febrile or respiratory illness.
Pregnancy- use this drug during pregnacy only if needed
Lactation- excercise caution when galasulfase is administered to a breast feeding woman.
Children- safety and efficacy in patients younger than 5 years have not been evaluated
Dosages/ Overdosage Etc:
Indication-
Mucoploysaccharidosis
Dosage-
1mg/kg body weight administred once weekly as an intravenous IV infusion.
Pretreatment- pretreatment with antihistaminea with or without antiyretics is recommended
30 to 60 minutes prior to the infusion
Patient Information:
1. Inform patients that a clinical survillance program has been established in order to better
understand the variablity and progression of the disease in the population as a whole.
and to monitor and evaluate long-term treatment effects of galsulfase
2. The clinical survelliance program will also monitor the effect of galasulfase in pregnant
woman and their offspring, and determine if galasulfase is excreted in breast milk.
3. Encourage patients to participate and advice them that participation may involve long-term
follow up.
Pharmacology/ Pharmacokinetics:
Ref -Drug Facts and comparisons (2010)
Pharmacolgy-
Mucopolysaccharide storage disorders are caused by the deficiency of specific lysosomal
enzymes required for the catabolism of GAG. MPS I is characterised by the absence of marked reduction in N-acetylgalatosamine 4-sulfatase . The sulfatase activity deficiency results in the accumulation of of GAG substrate dermatan sulfate throughout the body.
Galsulfase is intended to provide an exogenous enzyme that will be taken up into lysosomes and increase the catabolism of GAG.
Pharmacokinetics-
The pharmacokinetic parameters of galsulfase were evaluated in 13 patients with MPS VI
who received 1mg/kg of galsulfase as a 4-hour infusion weekly for 24 weeks.
The pharmacokinetics parameters at week 1 and week 24 are-
Galsufase Pharmacokinetic Parameters (Median, Range)
Pharmacokinetic Week 1 Week 24
parameter
Cmax (mcg/mL) 0.8 (0.4 to 1.3) 1.5 (0.2 to 5.5)
AUC (h*mcg/mL) 2.3 (1 to 3.5) 4.3 (0.3 to 14.2)
Volume of 103 (56 to 323) 69(59 to 2799)
distribution(mL/kg)
CL (mL/kg/min) 7.2 (4.7 to 10.5) 3.7( 1.1 to 55.9)
Half-life (min) 9 (6 to 21) 26( 8 to 40)
(Area under the plasma galsulfase concentration-time curve ( AUC)
from start of infusion to 60 minutes post infusion)
Pregnancy and lactation:
Pregnancy-
Use this drug during pregnacy only if needed
Lactation-
Excercise caution when galasulfase is administred to a breast feeding woman.
Children-
Safety and efficacy in patients younger than 5 years have not been evaluated
Manufacturer Details