primary health care services at their GP surgery. OS 11. All adults with SCD should have access to community nursing support. Overarching standards Standards for Clinical Care of Adults with Sickle Cell Disease in the UK, 2nd Edition 24 Acute pain OS 12. Patients presenting as a medical emergency with an acute painful episode should be offered appropriate analgesia within 30 minutes of presentation to the emergency department. OS 13. All hospitals with emergency departments should have protocols to guide management of uncomplicated acute presentations of SCD. Acute complications OS 14. All hospitals with emergency departments should have protocols to guide management of uncomplicated acute presentations of SCD including when to seek specialist advice. Chronic complications OS 15. All patients should be offered regular outpatient review to ensure screening for chronic disease complications and early instigation of treatment according to local protocols and national guidance. OS 16. All patients with evidence of chronic organ dysfunction should have access to review in multidisciplinary or specialist clinics. OS 17. Patients with complex pain needs should be referred to a multidisciplinary chronic pain team with experience of SCD, offering both pharmacological and nonpharmacological interventions. Prevention of infection OS 18. Specialist and local haemoglobinopathy teams and GPs should ensure that adults with SCD are adequately vaccinated against the following infections according to advice in the Green Book: · Invasive pneumococcal disease · Haemophilus influenza type B · Neisseria meningitis ACWY and B · Hepatitis B Overarching standards Standards for Clinical Care of Adults with Sickle Cell Disease in the UK, 2nd Edition 25 OS 19. Patients should be periodically warned about the increased risk of invasive pneumococcal disease (IPD) and other forms of sepsis. They should also be educated about symptoms which might indicate infection and to attend for medical assessment if temperature ≥380C. Annual review OS 20. All adults with SCD should be offered comprehensive review from a specialist centre at least annually. OS 21. A pro forma should be used for the annual review visit to ensure thorough and consistent care and to facilitate data collection. Pregnancy OS 22. Pregnant women with SCD should be managed by a multidisciplinary team of obstetricians, midwives and haematologists with an interest in SCD in a unit that manages high risk pregnancy. OS 23. Units which manage SCD pregnancy should have a clear protocol for patient management. Hydroxycarbamide (HC) OS 24. All hospitals looking after adults with SCD should have a prescribing and monitoring protocol for hydroxycarbamide (HC) (also known as hydroxyurea) to maximise benefits and safety. OS 25. Specialist centres should audit their use of HC to ensure it is discussed with all patients who may benefit from its use. Transfusion OS 26. All hospitals that admit SCD patients should have protocols and training in transfusion for SCD including manual exchange procedures. OS 27. Automated exchange transfusion should be available to all patients with SCD and should be provided by specialist centres. OS 28. Specialist centres should audit their use of blood transfusion in the acute and chronic setting to ensure its use is consistent with national guidance. Overarching standards Standards for Clinical Care of Adults with Sickle Cell Disease in the UK, 2nd Edition 26 Emerging therapies OS 29. National Health Service (NHS) England should ensure that all patients have equitable access to high cost interventions. OS 30. Trials for haematopoietic stem cell transplantation (HSCT) in adults with SCD should be available in the UK. OS 31. All patients with SCD should have access to information regarding current clinical trials, to enable participation if the patient so chooses. Standards for Clinical Care of Adults with Sickle Cell Disease in the UK, 2nd Edition 27 Section A: General principles Standards for Clinical Care of Adults with Sickle Cell Disease in the UK, 2nd Edition 29 Chapter 1: Overview Introduction This chapter is a brief description of sickle cell disease (SCD). It is not a comprehensive review and readers are referred to standard texts for more detailed information. Haemoglobin is the oxygen carrying protein found in the red blood cells. It comprises four ‘globin’ protein chains, each wrapped around an iron-containing ‘haem’ molecule. Newborn babies have a form of haemoglobin called foetal haemoglobin (haemoglobin F). This is largely replaced by adult haemoglobin (haemoglobin A) in the first year of life. Haemoglobin A consists of two alpha (α) globin chains and two beta (β) globin chains. The sickle mutation is a substitution of C for A at codon 6 of the β globin gene (βS). The resulting exchange of valine for glutamic acid leads to the production of a sickle haemoglobin molecule (haemoglobin SS). This has a tendency to form aggregates or to polymerise in the deoxygenated state resulting in red cell breakdown and aggregation of red cells in the blood vessels thereby causing blockage. Haemoglobin S and other significant variants SCD comprises a group of genetic conditions associated with βS, which give rise to significant clinical complications. Individuals who inherit βS from both parents are