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Standards for Clinical Care of Adults with Sickle Cell Disease in the UK

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Standards for Clinical Care of Adults with Sickle Cell Disease in the UK Standards for Clinical Care of Adults with Sickle Cell Disease in the UK 2nd Edition , 2018 Copyright © 2018 , 2008 Sickle Cell Society . All rights reserved. Although every precaution has been taken to verify the accuracy of the information contained herein, the author s and publisher assume no responsibility for any errors or omissions. No liability is assumed for damages that may result from the use of information contained within. Sickle Cell Society 54 Station Road London NW10 4UA United Kingdom Tel.: +44 (0)20 8961 7795 Fax: +44 (0)20 8961 8346 [email protected] www.sicklecellsociety.org Cover image by michaelj ung /Shutterstock.com Reproduced under license from Shutterstock.com Figure 1: A suggested model of transition, from Musumadi, L., Westerdale, N., & Appleby, H. (2012). An overview of the effects of sickle cell disease in adolescents. Nu rsing Standard, 26(26), 35 -40 [Nursing Standard by Royal Col lege of Nursing (Great Britain)] Reproduced with permission of RCN Publishing Co. in the format ‘Other Published Product’ via Copyright Clearance Center. Figure 6: Percentage of women experiencing an unintended pregnancy within the first year of use with typical use a nd perfect use ( UKMEC 2016. https:/ /www.fsrh.org/ukmec/ ) Reproduced under licence from FSRH. Copyright © Faculty of Sexual and Reproductive Healthcare 2006 to 2016. [This figure is modified from an original version in (Trussell, 2011) .] ISBN 978 -1-5272 -2070 -6 Printed in the United Kingdom First published 2008 Revised 2018 This and the previous edition of the standar ds are also available online at: www.sicklecellsociety.org The costs associated with the development of these standards were supported by an unrestricted educational g rant from Novartis Pharmaceuticals Standards for Clinical Care of Adults with Sickle Cell Disease in the UK , 2nd Edition Contents Tables and figures ................................ ................................ ................................ ................................ .... 1 Editorial/writing group ................................ ................................ ................................ .......................... 3 Acknow ledgements ................................ ................................ ................................ ................................ .. 7 Additional acknowledgements ................................ ................................ ................................ ............. 9 Production ................................ ................................ ................................ ................................ .................. 9 Clinical disclaimer ................................ ................................ ................................ ................................ .... 9 Foreword ................................ ................................ ................................ ................................ .................. 11 Statements of support ................................ ................................ ................................ ........................... 13 Chair’s introduction ................................ ................................ ................................ ............................... 19 Note on Methodology ................................ ................................ ................................ ............................. 21 Standards ................................ ................................ ................................ ................................ ................................ ......... 21 Recommendations ................................ ................................ ................................ ................................ ....................... 21 Overarching standards ................................ ................................ ................................ ......................... 23 General principles ................................ ................................ ................................ ................................ .............................. 23 Transition ................................ ................................ ................................ ................................ ................................ ............... 23 Primary care ................................ ................................ ................................ ................................ ................................ ......... 23 Acute pain ................................ ................................ ................................ ................................ ................................ .............. 24 Acute complications ................................ ................................ ................................ ................................ .......................... 24 Chronic complications ................................ ................................ ................................ ................................ ...................... 24 Prevention of infection ................................ ................................ ................................ ................................ ..................... 24 Annu al review ................................ ................................ ................................ ................................ ................................ ...... 25 Pregnancy ................................ ................................ ................................ ................................ ................................ .............. 25 Hydroxycarbamide (HC) ................................ ................................ ................................ ................................ ................. 25 Transfusion ................................ ................................ ................................ ................................ ................................ ........... 25 Emerging therapi es ................................ ................................ ................................ ................................ ........................... 26 Section A: General principles ................................ ................................ ................................ .............. 27 Chapter 1: Overview ................................ ................................ ................................ .............................. 29 Introduction ................................ ................................ ................................ ................................ ................................ .......... 29 Standards for Clinical Care of Adults with Sickle Cell Disease in the UK , 2nd Edition i Contents Haemoglobin S and other significant variants ................................ ................................ ................................ ..... 29 Sickle cell carriers ................................ ................................ ................................ ................................ .............................. 29 Epidemiology ................................ ................................ ................................ ................................ ................................ ........ 30 Pathophysiology ................................ ................................ ................................ ................................ ................................ .. 30 Clinical presentation ................................ ................................ ................................ ................................ ......................... 30 Clinic al course and survival ................................ ................................ ................................ ................................ ........... 31 Diagnosis ................................ ................................ ................................ ................................ ................................ ................ 31 Screening for sickle cell ................................ ................................ ................................ ................................ ................... 32 National screening programme ................................ ................................ ................................ ............................ 32 Neonatal screening ................................ ................................ ................................ ................................ ..................... 32 Antenatal screening ................................ ................................ ................................ ................................ .................... 33 Emergency and opportunistic screening ................................ ................................ ................................ ......... 33 Predictive factors and variability in phenotype ................................ ................................ ................................ ... 33 Rationale for therapies ................................ ................................ ................................ ................................ .................... 34 Chapter 2: Organisation of care ................................ ................................ ................................ .......... 35 General principles ................................ ................................ ................................ ................................ .............................. 35 Introduction ................................ ...............................
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