Medical history: Antonio is a 17-year-old male and, based on monitoring, has no apparent cognitive impairment
Antonio had an umbilical hernia present within first months of life, confirmed at the age of 1.1
Antonio had repeat visits to the pediatrician due to multiple ear infections.1
Antonio’s parents noticed his stomach seemed larger than other boys’, but they did not see it as unusual.1
The unresolved hernia was repaired by a pediatric surgeon.2
A heart murmur was detected during an annual check-up.3 Antonio was referred to a cardiologist, who performed an echocardiogram that identified morphological changes in the mitral valve, but ultimately determined that no intervention, only monitoring, was required.4
Antonio’s behavior changed as he appeared to be ignoring his teachers and parents. His doctor discovered he was experiencing progressive hearing loss, which was improved by hearing aid use.4,5
Antonio experienced progressive joint stiffness that affected his walking capacity; he often walked on his toes.3
The accumulating combination of symptoms led Antonio’s pediatrician to suspect a genetic condition. He referred him to a geneticist, who diagnosed Antonio with Hunter syndrome.
*These are hypothetical patient profiles intended to represent patients with MPS II. Individual signs and symptoms vary widely.
ELAPRASE is administered at a dose of 0.5 mg/kg body weight every week by intravenous infusion. ELAPRASE is supplied in 3 mL vials containing 2 mg/mL ELAPRASE.
Treatment with ELAPRASE was initiated. Antonio weighed 22 kg; therefore, he received an infusion of 11 mg ELAPRASE (1.8 vials of ELAPRASE) per week.
22 kg x (0.5 mg/kg)6 = 11 mg
11 mg ÷ (2 mg/mL) = 5.5 mL
5.5 mL ÷ (3 mL/vial) = 1.8 vials
Antonio's continued joint stiffness was compounded by carpel tunnel syndrome; intubation was challenging during release surgery.2
Antonio started to noticeably fall behind other boys his age in height.5
Antonio’s complaints of sleeping poorly were discovered to be caused by worsening sleep apnea.3
Antonio underwent spinal fusion surgery under general anesthetic for scoliosis and kyphosis. He had to be seen by many specialists before being anesthetized due to upper airway obstruction.5
ELAPRASE is dosed according to weight,6 so Antonio is weighed regularly. He weighs 60 kg; therefore, his weekly ELAPRASE dose is 30 mg (5 vials).
60 kg x (0.5 mg/kg) = 30 mg
30 mg ÷ (2 mg/mL) = 15 mL
15 mL ÷ (3 mL/vial) = 5 vials
*These are hypothetical patient profiles intended to represent patients with MPS II. Individual signs and symptoms vary widely.
ELAPRASE is administered at a dose of 0.5 mg/kg body weight every week by intravenous infusion. ELAPRASE is supplied in 3 mL vials containing 2 mg/mL ELAPRASE.
WARNING: RISK OF ANAPHYLAXIS
Life-threatening anaphylactic reactions have occurred in some patients during and up to 24 hours after ELAPRASE infusions. Anaphylaxis, presenting as respiratory distress, hypoxia, hypotension, urticaria and/or angioedema of throat or tongue have been reported to occur during and after ELAPRASE infusions, regardless of duration of the course of treatment. Closely observe patients during and after ELAPRASE administration and be prepared to manage anaphylaxis.