PI
In which patients is Nexviazyme contraindicated
Patients with life threatening hypersensitivity to the active substance or any of the excipients when re-challenge was unsuccessful
What is the recommended initiation rate for Nexviazyme infusion?
1 mg/kg/hour
What was the primary endpoint of the COMET study?
Change in forced vital capacity in the upright position from baseline to week 49
What is the pregnancy category of Nexviazyme?
B1
What is the indication for Nexviazyme?
For long-term ERT for the treatment of patients 1 yr or older with Pompe Disease
What is the recommended dose of NEXVIAZYME for patients with Infantile onset Pompe disease?
Up to 40 mg/kg administered every other week
In what percentage of patients receiving NEXVIAZYME in the COMET Study were serious adverse reactions reported?
2%
How many patients receiving NEXVIAZYME in all clinical studies permanently discontinued treatment due to adverse reactions?
Two
Is Nexviazyme contraindicated for patients who have acute underlying respiratory illness or compromised cardiac or respiratory function?
No, however monitoring of vital signs more frequently is recommended
What is the concentration of the reconstituted solution of NEXVIAZYME prior to dilution?
10 mg/ml
Which adverse drug reactions (ADR) were among the most frequently reported in patients treated with NEXVIAZYME in the COMET study?
Headache and fatigue
In what percentage of patients treated with NEXVIAZYME in pooled clinical studies did infusion-associated reactions (IARs) occur?
30%
What should be done if severe hypersensitivity or anaphylaxis occur in a patient receiving NEXVIAZYME?
NEXVIAZYME should be discontinued immediately, and appropriate medical treatment should be initiated.
What rate of increases can be made to the Nexviazyme infusion after initiation? (Time and Amt and Maximum).
2 mg/kg/hour every 30 mins to max of 7 mg/kg/hour
In what percentage of patients treated with alglucosidase alfa in COMET were infusion-associated reactions (IARs) reported?
32.7% or 16 patients
In NEXVIAZYME treated patients, anti-drug antibodies to NEXVIAZYME occurred in __% treatment naive adults and __% switch adults.
95.1 and 49.1
Should desensitisation procedures be considered for a patient who is having a severe hypersensitivity reaction to Nexviazyme?
YES
How many milligrams of Nexviazyme powder are contained in one vial?
100 mg
In the COMET study, what was the estimated difference between NEXVIAZYME and alglucosidase alfa in FVC percentage predicted, that achieved statistical non-inferiority?
2.4%
What was the incidence of treatment-emergent anti-drug antibody (ADA)response to NEXVIAZYME in treatment-naïve patients?
95% or 58 patients