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Instructions for Use 'Template Research Protocol' NL61027.091.17 / National study of rare bleeding disorders in the Netherlands RBIN Corresponding author: Dr. Marten R. Nijziel MD PhD Radboud university medical center, Department of Haematology, route 476 P.O. Box 9101 6500 HB Nijmegen The Netherlands Ph: +31 (0)24 3668286 F: +31 (0)24 3542080 E: [email protected] Concept version 3.0, 16-8-2017 NL61027.091.17 / National study of rare bleeding disorders in the Netherlands RBIN PROTOCOL TITLE ‘Rare Bleeding Disorders in the Netherlands (RBIN)’ Protocol ID HEMSTOL47 Short title Rare bleeding disorders in the Netherlands Version 2.0 Date July 5, 2017 Coordinating investigator/ Dr. Marten R. Nijziel MD PhD project leader Radboud university medical center, Department of Haematology, route 476 P.O. Box 9101 6500 HB Nijmegen The Netherlands P: +31 (0)24 3668286 F: +31 (0)24 3542080 E: [email protected] Principal investigator(s) (in Dutch: Multicenter study, participating sites and principal hoofdonderzoeker/ uitvoerder) investigators: Academical Medical Center Amsterdam, Dr. Marjolein Peters MD PhD University Medical Center Groningen, Prof. Dr. Karina Meijer MD PhD Leiden University Medical Center, Dr. Felix J.M. van der Meer MD PhD Erasmus MC, Dr. Marjon Cnossen MD PhD UMCU, Prof. dr. Roger Schutgens MD PhD Máxima Medisch Centrum Eindhoven/Veldhoven, Dr. Laurens Nieuwenhuizen MD PhD Maastricht UMC+, Dr. Erik Beckers MD PhD NL61027.091.17 / National study of rare bleeding disorders in the Netherlands RBIN Investigators Drs. Joline Saes MD Dr. Saskia Schols MD PhD Dr. Britta Laros-van Gorkom MD PhD Drs. Yolba Smit MD Adinda Diekstra Ton de Haan MSc Dr. Sonja de Munnik MD PhD Dr. Paul Brons MD PhD Sponsor (in Dutch: Research center Haemostasis Radboudumc verrichter/opdrachtgever) Nijmegen Subsidising party Radboudumc Independent expert (s) Adults: Mevr. Dr. E.M. Klappe MD PhD, specialist in internal medicine, department Algemene Interne Geneeskunde, Radboudumc (024 – 3616504) Children: Dr. M. Schreuder MD PhD, paediatrician – nephrologist, department of paediatrics, Radboudumc (024 – 3655700) Laboratory sites Dr. Waander van Heerde PhD Laboratory Hematology Radboud university medical center NL61027.091.17 / National study of rare bleeding disorders in the Netherlands RBIN PROTOCOL SIGNATURE SHEET Name Signature Date Acting Head of Department: Dr. Michel Schaap MD PhD Coordinating Investigator Dr. Marten Nijziel MD PhD NL61027.091.17 / National study of rare bleeding disorders in the Netherlands RBIN TABLE OF CONTENTS TABLE OF CONTENTS ........................................................................................................ 5 LIST OF ABBREVIATIONS AND RELEVANT DEFINITIONS ................................................ 8 SUMMARY ...........................................................................................................................10 1. BACKGROUND.............................................................................................................11 1.1. Epidemiology of the rare bleeding disorders ...........................................................12 1.2. Clinical manifestations ............................................................................................12 1.3. Health-related quality of life ....................................................................................16 1.4. Laboratory ..............................................................................................................16 Screening assays ..........................................................................................................16 Confirmation assays ......................................................................................................17 Global assays ................................................................................................................17 1.5. Genetics .................................................................................................................17 1.6. Management ..........................................................................................................18 1.7. Ageing and coagulation ..........................................................................................18 1.8. Arteriothrombotic events and bleeding disorders ....................................................18 2. RATIONALE ..................................................................................................................20 3. OBJECTIVES ................................................................................................................22 4. STUDY DESIGN ...........................................................................................................23 5. STUDY POPULATION ..................................................................................................24 5.1. Population (base) ...................................................................................................24 Characteristics of the Dutch source population ..............................................................24 Willingness of patients to participate ..............................................................................25 5.2. Inclusion criteria .....................................................................................................25 5.3. Exclusion criteria ....................................................................................................26 NL61027.091.17 / National study of rare bleeding disorders in the Netherlands RBIN 6. METHODS ....................................................................................................................27 6.1. Study procedures ...................................................................................................27 6.2. Data collection ........................................................................................................28 Blood sampling ..............................................................................................................29 Saliva sampling .............................................................................................................29 Electronic patient records ..............................................................................................30 Whole exome sequencing .............................................................................................30 6.3. Withdrawal of individual subjects ............................................................................30 6.4. Replacement of individual subjects after withdrawal ...............................................30 6.5. Follow-up of subjects withdrawn from treatment .....................................................30 6.6. Premature termination of the study .........................................................................30 7. STATISTICAL ANALYSIS .............................................................................................31 7.1. Primary objectives ..................................................................................................31 7.2. Secondary objectives .............................................................................................32 8. ETHICAL CONSIDERATIONS ......................................................................................34 8.1. Regulation statement .............................................................................................34 8.2. Recruitment and consent........................................................................................34 8.3. Objection by minors or incapacitated subjects ........................................................34 8.4. Benefits and risks assessment, group relatedness .................................................34 8.5. Compensation for injury .........................................................................................34 8.6. Incentives ...............................................................................................................35 9. ADMINISTRATIVE ASPECTS, MONITORING AND PUBLICATION .............................36 9.1. Handling and storage of data and documents ........................................................36 9.2. Monitoring and Quality Assurance ..........................................................................36 9.3. Amendments ..........................................................................................................36 NL61027.091.17 / National study of rare bleeding disorders in the Netherlands RBIN 9.4. Annual progress report ...........................................................................................36 9.5. Temporary halt and (prematurely) end of study report ............................................36 9.6. Public disclosure and publication policy ..................................................................37 10. REFERENCES ..........................................................................................................38 Appendix 1: Patient information letter and informed consent form ........................................41 NL61027.091.17 / National study of rare bleeding disorders in the Netherlands RBIN LIST OF ABBREVIATIONS AND RELEVANT DEFINITIONS ALAT Alanine amino transferase ANOVA Analysis of Variance APTT Activated Partial Thromboplastin Time CI Confidence Interval F Factor GCP Good Clinical Practice HIN Haemophilia in the Netherlands ICH International Council for Harmonisation of Technical Requirements for Pharmaceuticals for Human Use ISTH(SSC)-
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