Cell Society instigated the update of these standards in 2015. The previous editorial board and members of the UK Forum on Haemoglobin Disorders were invited to apply to join the editorial board or to take part as contributing authors or as part of the sounding board. The editorial/writing group met regularly, each oversaw a section of the Standards and were responsible for co-ordinating co-authors, editing their chapters and reviewing the entire document. The document has been divided into three sections, Section A (General Principles) which includes organisation of care and health and well-being, Section B on the management of acute and chronic complications and Section C which includes other management issues and treatments. In many of the areas discussed, particularly Section A, the guidance is largely practical and organisational with no formal trial or other clear evidence. Even in the clinical chapters there is often a lack of clinical trial evidence and we have relied largely on published retrospective analyses, observational data, expert opinion and the views of patients and families. If a recent systematic review was available its findings were incorporated into the document. If this was not available a literature review was performed and its outcomes summarised. We have adopted two grades of practical interventions: Standards Being that which providers must do to ensure safe and adequate care or where omission could lead to poor clinical outcomes. These include key requirements of any service. Where possible we have tried to ensure these are auditable. Recommendations Being those that would be beneficial and that providers should try to follow, but for which there is less evidence or that are less likely to have a direct impact on clinical outcomes. We note however that there is a lack of research studies for many of these recommendations, especially the non-clinical ones. Practice has evolved over time so we have tried to document what most people do and what is a consensus view of good practice. Note on Methodology Standards for Clinical Care of Adults with Sickle Cell Disease in the UK, 2nd Edition 22 The entire document and in particular the standards and recommendations were debated by the editorial panel. A draft document was sent to the Sounding Board in July 2017 and their comments were incorporated into the final draft. Where evidence was lacking a consensus decision was reached by the editorial board. The patient quotes were collated by the Sickle Cell Society from surveys undertaken with the Picker Institute (Europe); the National Institute for Health Research (NIHR); and Collaboration for Leadership in Applied Health Research and Care (CLAHRC) – North West London. In addition, some quotes were also developed by the Sickle Cell Society’s patient lead as part of the peer review process. Over 700 sickle patients and carers across the U.K participated in the survey and the key themes emerging from the surveys were: · The poor quality of emergency vs planned care · Clinical awareness of dealing with SCD across primary and acute care · Significant variation in care · The poor management of SCD including coping with pain. The survey was carried out anonymously and the main objectives of carrying out the survey at this scale were to support the development of this document, to improve the overall quality of sickle cell services across the country by supporting hospitals through the clinical peer reviews and to represent the voices of the patients. The Sickle Cell Society ensured patients were involved in these peer group evaluations engaging with other sickle patients across the country by providing consistent support. Following from this, the development of this document has also involved patients, carers and the Sickle Cell Society. The objective is to ensure that the voices of sickle cell adult patients are directly represented. Standards for Clinical Care of Adults with Sickle Cell Disease in the UK, 2nd Edition 23 Overarching standards General principles OS 1. Adults with sickle cell disease (SCD) should be offered care close to home where possible, but should also have access to highly specialist multidisciplinary care including specialist nursing support. OS 2. All local hospitals should be linked with a named specialist centre with agreed pathways and protocols for advice and referral for acute and chronic complications. OS 3. Specialist haemoglobinopathy teams should participate in a quality review programme of haemoglobinopathy services against nationally agreed standards. OS 4. All consenting patients should be registered on the National Haemoglobinopathy Registry (NHR) and annual review data and adverse events should be reported to the NHR. OS 5. All patients should have access to specialist psychology support. OS 6. Core staffing of Specialist Centres for SCD should include a psychologist with a special interest and experience in SCD. Transition OS 7. Specialist teams should have a policy and dedicated team for transition, which should include a named transition-lead. OS 8. Further and higher education institutions, universities and colleges should develop and monitor policies for supporting students with SCD with respect to their education, their health and their careers. Primary care OS 9. All adults with SCD should be registered with a general practitioner (GP). OS 10. Each SCD patient should be offered routine