J3Z-MC-OJAB is an open-label, Phase 1/2, multicenter study to evaluate the safety and efficacy of single-dose LY3884961 (formerly PR001) in infants diagnosed with Type 2 Gaucher disease (GD2). For each patient, the study will be approximately 5 years in duration. During the first 12 months after dosing, patients will be evaluated for the effects of LY3884961 on safety, tolerability, immunogenicity, biomarkers, and efficacy. Patients will be followed up for an additional 4 years to monitor safety and changes on selected biomarkers and clinical outcomes.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
7
UCSF Benioff Children's Hospital, 5700 Martin Luther King Jr Way
Oakland, California, United States
University of Minnesota Masonic Children's Hospital, 2450 Riverside Avenue
Minneapolis, Minnesota, United States
Children's Hospital of Pittsburgh, 4401 Penn Avenue
Pittsburgh, Pennsylvania, United States
Lysosomal & Rare Disorders Research and Treatment Center
Fairfax, Virginia, United States
Number of Adverse Events (AEs), Serious Adverse Events (SAEs), and Adverse Events leading to discontinuation
Time frame: Year 5
Immunogenicity of AAV9 and GCase in blood
Time frame: Up to Year 2
Immunogenicity of AAV9 and GCase in CSF
Time frame: Up to Year 1
Time to death
Time frame: Baseline until event or study completion, up to Year 5
Time to clinical event
Clinical event defined as tracheostomy/invasive ventilation, and/or percutaneous endoscopic gastrostomy (PEG) tube placement, and/or nasogastric (NG) tube placement
Time frame: Baseline until event or study completion, up to Year 5
Change in cognitive function
Measured using Bayley Scales of Infant and Toddler Development (BSID-III)
Time frame: Months 6,12 and up to Year 2
Change in cognitive function
Measured using Wechsler Preschool and Primary Scale of Intelligence (WPPSI-IV) as appropriate. (Not all patients begin the study at birth. Only patients who are age 36 months at the designated study visits will be assessed using this measure)
Time frame: Study Month 12 and up to Study Year 2
Change in motor skills
Change from baseline in motor function using Gross Motor Function Measure (GMFM-88).
Time frame: Months 6, 12 and up to Year 2
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Administered orally as concomitant medication, followed by dose tapering.
Manchester Centre for Genomic Medicine, 6th Floor, St Mary's Hospital, Oxford Road
Manchester, United Kingdom
Change in motor skills
Change from baseline in motor function using the BSID-III.
Time frame: Months 6, 12 and up to Year 2
Change in Clinical Global Impressions (Severity)
Change from baseline in the clinical severity of illness (CGI-Severity {CGI-S}).
Time frame: Months 6, 12 and up to Year 2
Clinical Global Impressions (Improvement)
Clinical improvement from baseline (CGI-Improvement \[CGI-I\]).
Time frame: Months 6, 12 and up to Year 2
Change in adaptive behavior and functioning
Change from baseline in adaptive functioning using the Vineland Adaptive Behavior Scale (VABS-2) (2nd edition)
Time frame: Months 6 and 12 and up to Year 2
Change in most troubling symptoms
Change from baseline in the Visual Analog Scale for the Most Troubling Symptoms (VAS-MTS)
Time frame: Months 6, 12 and up to Year 2
Change in behavioral symptoms
Change from baseline in the Child Behavior Checklist (CBCL)
Time frame: Months 6, 12 and up to Year 2
Change in GCase (glucocerebrosidase) enzyme activity levels in blood
Time frame: Up to Year 5
Change in GCase enzyme activity levels in CSF (cerebrospinal fluid)
Time frame: Up to Year 3
Change in glycolipid levels in blood
Time frame: Up to Year 5
Change in glycolipid levels in CSF
Time frame: Up to Year 3
Individual Vector Shedding data
Time frame: Up to Year 5