International clinical trial networks for NMD



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International clinical trial networks for NMD

Options for clinical trials Industry-sponsored Trial design: industry Run by Outsourced to CRO (clinical research organization) Company Recruitment sites academic Infrastructure de novo trial-specific Academic (investigator-sponsored) Trial design: academics Run by academics Recruitment sites academic Infrastructure either de novo or established infrastructure/network

Pros/cons of industry vs. academic Industry-sponsored Pros Money often not limiting Industry understands regulatory goals eye on ball Robust conduct in regulatory context (i s dotted; t s crossed) Cons Pharma may lack domain expertise in disease CROs typically lack domain expertise in disease De novo infrastructure may have kinks/problems

Pros/cons of industry vs. academic Academic (investigator-sponsored) Pros Extensive domain expertise (key opinion leaders; KOLs) Existing expertise/infrastructure quicker start up, less kinks/problems Cons May not understand regulatory focus, importance of i s t s Can be resource starved Contracting/budgeting a single site can be challenging; multiple academic-academic agreements very challenging

DMD trial infrastructure status Individual site expertise built Clinical evaluator expertise Medical director expertise Coordinator expertise Run: site-specific trials, de novo networks (FOR DMD), and industry participation Exemplars: Newcastle; Rochester NY; Wash U, St Louis Clinical trial networks Create stable infrastructure of many sites CEs trained, reliability studies, across all sites Contracting (master agreements) IRB (federated, centralized, or at least organized) (the iterative nightmare avoided) Data management (obtaining test data, transmitting, analyses) Stable census

Pros of an established trial network Pros (hypothetical) Trial can be initiated much faster Most of infrastructure at multiple levels already there CE, coordinator, equipment, data management, contracting, IRBs, stats Patient population already well characterized, easily recruited Plug and play Trial can be run better Domain expertise Less kinks in context of trial Input on trial design may prevent late stage, expensive trial failures Resource allocation (fiscal, and ethical)

Cons of an established trial network Some cons of academia remain Resource starved Requirement to Feed the Beast. i s t s, eye on the regulatory ball (Contracts, IRBs may have been navigated) Pharma loss of control Their drug program is at stake! Pharma, FDA/EMA recognizes a good CRO do they similarly recognize a good academic clinical trial network? It is not the way it is done.

Changes in drug development landscape Patent cliff, day of blockbusters gone Clinical trials with 10,000+ patients; two phase 3 trials to registration changing Transition to orphan drugs Unmet need for rare disorders Common disorders becoming more stratified into subgroups (e.g. breast cancer) in effect, common disease enters orphan drug space Orphan drugs meant no pharma would pick them up changed, most pharma have new orphan drug divisions

Assuming an established clinical trial network is a good idea, how does one set it up? How does it work in practice? Muscle Study Group FOR-DMD Neuro-NEXT CINRG Cooperative International Neuromuscular Research Group

Brief history 1990 s new experimental therapeutics emerging, but no clinical trials, no infrastructure Need a blank slate, need resources, move to DC Blank slate Children s National Medical Center not a University, not much pre-existing research, able to do whatever you can pay for. Resources - Potential for working with government to remediate disparities ; foundations DMD research funded by US government at 10% of any other disease

Resources: First decade (2000-2010) Lobbying Efforts for DMD Research May 15 th 2000 3 year-old James Wood Diagnosed with Duchenne Parents Dana and Joel Wood lobbyists: investigate therapeutics, treatment and funding for Duchenne No federal dollars appropriated from Congress for Duchenne Joel and Dana Wood launch effort around legislation entitled the Muscular Dystrophy Care Act of 2001 11

Lobbying Efforts for DMD Research Then Representative Roger Wicker (R-MS) teams up with Senator Paul Wellstone (D-MN) to help the cause Creates a bipartisan, bicameral team in Congress to pass legislation On February 14 th 2001, Congressman Wicker introduces MD Care Act Legislation passes overwhelmingly and is signed into law by President Bush in December 2001 12

Lobbying Efforts for DMD Research Legislation included 4 major points: NIH to support Centers of Excellence focused on muscular dystrophy containing basic research, extensive collaboration, shared resources and a clinical study CDC to establish programs focused on Duchenne muscular dystrophy including improving diagnosis, data collection and care considerations NIH and related government agencies would convene the research and clinical community to develop a research plan NIH and related government agencies would establish a steering committee to oversee progress (MDCC) 13

Lobbying Efforts for DMD Research After passage of legislation in December 2001, few of the promises of the MD Care Act fulfilled by the end of 2002 In 2003, Dana and Joel Wood expand the staff of the Foundation to Eradicate Duchenne in order to directly target various appropriations bills The Department of Defense Appropriations Bill of 2003 becomes the first federal appropriation for Duchenne Muscular Dystrophy research 14

Congressional Appropriation Funding Levels for DMD $40 million Department of Defense 2003 - $3.4 million 2004 - $5.25 million 2005 - $3.5 million 2006 $2.4 million 2007 - $1.9 million 2008 - $4 million 2009 - $4 million 2010 - $3.75 million 2011 - $4 million 2012 - $3.2 million 2013 - $3.2 million $5 million Foundation to Eradicate Duchenne Areas of focus CINRG trial network Morpholino exon skipping VBP15 dissociative steroid Adjuvants exon skipping Chronotherapy TREAT-NMD TACT 15

CINRG Clinical Research Network 28 SITES

CINRG Structure CINRG Executive Committee (EC) CINRG Therapeutic Subcommittee (CTS) Scientific Advisory Board (SAC) CINRG Outcomes Subcommittee (COS) CINRG Coordinating Center (CC) Data Safety Monitoring Board (DSMB) CINRG Publication Subcommittee (CPS) Patient Advocacy Organizations CINRG Clinical Study Sites

CINRG Coordinating Center Staff Director: Avital Cnaan, PhD Central CE Manager: Tina Duong, MPT Operations Manager and Central Genetic Counselor: Lauren Hache, MS, CGC Project Managers: Zoë Sund; Lisa Hunegs, MSW, MPH; Andrea Smith, MS, CGC; and Paula Muir, MS Database Managers: Adrienne Arrieta, MS (lead); Mohammad Ahmed, MS; and Jia Feng, MS Biostatistician: Heather Gordish-Dressman, PhD and Fengming Hu, MS

CINRG Coordinating Center Support Master agreement and project agreements Support for grant/proposal submission Budgeting & liaison with vendors Survey for patient availability/study feasibility Protocol design and development Regulatory support Central Clinical Evaluator training & certification Central Genetic Counselor Database design and data management Biostatistics analyses Publications: abstracts, posters, manuscript support Coordination of operations, investigators meetings and regularly scheduled calls

CINRG Clinical Census Neuromuscular Disorders Duchenne muscular dystrophy (DMD) Curated Genetic Information Non-curated Becker muscular dystrophy (BMD) Defined LGMD Undefined LGMD Facioscapulohumeral dystrophy (FSHD) Nemaline CINRG Census (Last performed 2010) 2,900 patients 375 (curated data) 2,525 (non-curated) 522 patients 316 patients 604 patients 491 patients 89 patients (subset of CINRG sites) Total Neuromuscular CINRG Census Population: 4,922 patients

CINRG Executive Committee Ten Elected Seats Elections for partial Rotations occurs in Oct- Nov. Medical Director: Paula Clemens, MD Scientific Director: Eric Hoffman, PhD Director, Coordinating Center: Avital Cnaan, PhD 5 Site Investigators: Alberto Dubrovsky, MD Julaine Florence, DPT Nancy Kuntz, MD Craig McDonald, MD Monique Ryan, MD Clinical Evaluator: Kristy Rose, DPT Clinical Coordinator: Priscilla Russo, BSN, CCRC

Scientific Advisory Committee (SAC) Role: Reviews accomplishments of the CINRG group and other groups and provides input on scientific priorities and external vision on activities Members: James Boyett, PhD Kate Bushby, MD John McCall, PhD Krista Vandenborne, PhD Glenn Walter, PhD Joel Wood

Data and Safety Monitoring Board (DSMB) Role: To review both data and safety of all ongoing studies at CINRG Members: Chair: Kathryn Wagner, MD, PhD Pat Furlong (Patient Representative) Neal Jeffries, PhD Michael Sirdofsky, MD

CINRG Site Personnel Site Principal Investigator Clinical Research Coordinator Coordinate study visits Two Physical Therapists Trained and certified as Clinical Evaluators for CINRG Performs CQMS tests

CINRG Website (public/private)

Outcome Measures CINRG Quantitative Measurement System (CQMS) Cardiac Outcomes Patient Report Outcomes Genetic and biomarkers

CINRG Quantitative Measurement System (CQMS) CQMS incorporates a standard sequence of assessments, audiovisual feedback, real time data checks designed to improve effort dependent strength measures and test performance This system has the capability of recording quantitative and manual muscle testing, pulmonary function tests, timed tests, functional tests, and range of motion in a standardized fashion

CQMS Set-Up and Visuals On your mark, get set, go. The race is on!

Cardiac Outcomes Speckle tracking echocardiography (STE) Myocardial Performance Index (MPI) Fractional Shortening Ejection Fraction

Genetic Polymorphisms and Biomarkers SNP GWAS Biomarkers Proteomics microrna Metabolomics

Central Database

1.Open Clinica Layout All studies in one database Current Study you re working on The info section shows details of the study you re working on. Click here if you want to change study or site. Navigation bar In house database - CRF designed to incorporate NIH common data elements - TREAT-NMD registry minimum data set - 21 CFR part 11 compliant

Launching Studies Financial and Legal Master Agreement Study Specific Agreements Invoices and Payment Ethics and Initiation CINRG CC Project Email Launch Preparation of ethics documents Site initiation, training and Enrollment

CINRG Studies Enrolling Now Overview Clinical Trial 1. Clinical Trial of Coenzyme Q10 and Lisinopril in Muscular Dystrophies Sponsor: Department of Defense N=120 (Ages 8 and up) Non-Interventional 2. DMD Natural History Study Sponsor: DOE, NIH, DOD, PPMD Re-open young DMD (4-7 years) and controls (6-30 years) N = 440 3. FSHD Sponsor: FSH Society N=50 4. BMD Natural History Study Sponsor: NIH N=80 (Ages 4 and up) 5. DMD Tissue Bank Sponsor: NIH N=50 (Ages 4 and up)

CINRG Completed Studies Study Title Study Chair Sponsor Publications CQMS Reliability Studies D. Escolar J. Mayhew N/A Muscle and Nerve (2001) 24:787-93 and Muscle and Nerve (2007) 35:36-42 Open-Label Pilot Study of Oxatomide in Steroid-Naive DMD Open-Label Pilot Study of Coenzyme Q10 in Steroid-Treated DMD Double Blind RTC to Assess the Efficacy and Safety of Glutamine and Creatine in DMD Open-Label Pilot Study of Pentoxifylline in Steroid-Naïve DMD Double Blind RTC of Daily Pentoxifylline as Rescue Treatment in DMD Double Blind RTC of Daily vs. High Dose Weekly Prednisone in DMD G. Buyse D. Escolar AFM MDA European Journal of Pediatric Neurology (2007 ) 11:337 340 D. Escolar MDA Muscle and Nerve (2011) 44:174-178 D. Escolar MDA Annals of Neurology (2005) 58:151 155 D. Escolar MDA Muscle and Nerve (2011) 44:170-173 D. Escolar FED Neurology (2012) 78;904-913 D. Escolar P. Clemens CINRG DMD natural history cohort 1 C. McDonald NIH, DoD MDA Neurology (2011) 77:444-452 Muscle Nerve 2013 CINRG DMD natural history cohort 2 C. McDonald NIH Muscle Nerve 2013 DoD Genetic modifiers of DMD E. Hoffman NIH Neurology 2011

CINRG: What Makes the System Work Collaboration with basic scientists Central infrastructure CINRG Executive Committee and by-laws SAC, DSMB, and subcommittees Central Pharmacy & Laboratories Multi-center structure Standardized Protocols Evaluator Training and Reliability Program Diverse clinical centers

Transitions/challenges Transition off DoD appropriations Successful in competitive grants Change ratio of observational studies vs. clinical trials Improve strength of portfolio of sites Survival of the fittest, meet recruitment goals Increase relationship with pharma CINRG as a CRO

Acknowledgements Joel Wood, Bill Quirk and appropriations team Abby Bronson, Ed Connor and industry alliance team FED, MDA, CureDuchenne NIH, Department of Defense CINRG participants, patients/families, Lauren Hache TREAT-NMD