Issue date: June 2012 Sickle cell acute painful episode Management of an acute painful sickle cell episode in hospital NICE clinical guideline 143 Sickle cell acute painful episode. NICE clinical guideline 143 (June 2012) 1 Developed by the Centre for Clinical Practice at NICE Update information August 2016: An out of date research recommendation was deleted. NICE clinical guidelines are recommendations about the treatment and care of people with specific diseases and conditions in the NHS in England and Wales. This guidance represents the view of NICE, which was arrived at after careful consideration of the evidence available. Healthcare professionals are expected to take it fully into account when exercising their clinical judgement. However, the guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, and informed by the summary of product characteristics of any drugs they are considering. Implementation of this guidance is the responsibility of local commissioners and/or providers. Commissioners and providers are reminded that it is their responsibility to implement the guidance, in their local context, in light of their duties to avoid unlawful discrimination and to have regard to promoting equality of opportunity. Nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties. National Institute for Health and Clinical Excellence Level 1A, City Tower Piccadilly Plaza Manchester M1 4BT www.nice.org.uk © National Institute for Health and Clinical Excellence, 2012. All rights reserved. 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Contents Introduction ...................................................................................................... 4 Patient-centred care ......................................................................................... 6 1 Recommendations .................................................................................... 8 1.1 List of all recommendations ................................................................ 8 2 Evidence review and recommendations .................................................. 14 2.1 Pharmacological management ......................................................... 14 2.2 Non-pharmacological management .................................................. 81 2.3 Clinical signs and symptoms of acute complications ........................ 86 2.4 Settings and skills for managing an acute painful sickle cell episode ... ........................................................................................................ 117 2.5 Information and support needs of patients and their carers during an acute painful sickle cell episode ............................................................... 138 3 Notes on the scope of the guideline ...................................................... 157 4 Implementation ...................................................................................... 158 5 Other versions of this guideline ............................................................. 158 6 Related NICE guidance ......................................................................... 158 7 Updating the guideline ........................................................................... 159 8 References ............................................................................................ 160 9 Glossary and abbreviations ................................................................... 166 Appendix A Contributors and declarations of interests ................................ 168 Appendix B List of all research recommendations ....................................... 173 Appendix C Guideline scope ........................................................................ 176 Appendix D How this guideline was developed ........................................... 177 Appendix E Evidence tables ........................................................................ 178 Appendix F Full health economic report ....................................................... 179 Appendices C, D, E and F are in separate files. NHS Evidence has accredited the process used by the Centre for Clinical Practice at NICE to produce guidelines. Accreditation is valid for 3 years from April 2010 and is applicable to guidance produced using the processes described in NICE’s ‘The guidelines manual’ (2009). More information on accreditation can be viewed at www.evidence.nhs.uk Introduction Acute painful sickle cell episodes Sickle cell disease is the name given to a group of lifelong inherited conditions of haemoglobin formation. Most people affected are of African or African- Caribbean origin, although the sickle gene is found in all ethnic groups. Sickle cell disease can have a significant impact on morbidity and mortality. It is estimated that there are between 12,500 and 15,000 people with sickle cell disease in the UK. The prevalence of the disease is increasing because of immigration into the UK and new births. The NHS Sickle Cell and Thalassaemia Screening Programme also means that more cases are being diagnosed. Acute painful sickle cell episodes (also known as painful crises) are caused by blockage of the small blood vessels. The red blood cells in people with sickle cell disease behave differently under a variety of conditions, including dehydration, low oxygen levels and elevated temperature. Changes in any of these conditions may cause the cells to block small blood vessels and cause tissue infarction. Repeated episodes may result in organ damage. Acute painful sickle cell episodes occur unpredictably, often without clear precipitating factors. Their frequency may vary from less than one episode a year to severe pain at least once a week. Pain can fluctuate in both intensity and duration, and may be excruciating. The majority of painful episodes are managed at home, with patients usually seeking hospital care only if the pain is uncontrolled or they have no access to analgesia. Patients who require admission may remain in hospital for several days. The primary goal in the management of an acute painful sickle cell episode is to achieve effective pain control both promptly and safely. The management of acute painful sickle cell episodes for patients presenting at hospital is variable throughout the UK, and this is a frequent source of complaints from patients. Common problems include unacceptable delays in NICE clinical guideline 143 – sickle cell acute painful episode 4 receiving analgesia, insufficient or excessive doses, inappropriate analgesia, and stigmatising the patient as drug seeking. This guideline addresses the management of an acute painful sickle cell episode in patients presenting to hospital until discharge. This includes the use of pharmacological and non-pharmacological interventions, identifying the signs and symptoms of acute complications, skills and settings for managing an acute painful episode, and the information and support needs of patients. This is an overarching guideline covering the principles of how to manage an acute painful sickle cell episode in hospital. Local protocols should be referred to for specific management plans, including drug choice and dosages. This guideline includes the management of acute painful sickle cell episodes in children and young people and in pregnant women. The guideline recommendations apply to all patients presenting with an acute painful sickle episode unless there are differences in management for these groups, in which case these are clearly outlined. Drug recommendations The guideline does not make recommendations on drug dosage; prescribers should refer to the ‘British national formulary (BNF)’ and ‘BNF for children’ for this information. The guideline also assumes that prescribers will use a drug’s summary of product characteristics to inform decisions made with individual patients. Who this guideline is for This document is for healthcare professionals and other staff who care for people with an acute painful sickle cell episode in hospital. People with sickle cell disease and their family members and carers may also find it useful. NICE clinical guideline 143 – sickle cell acute painful episode 5 Patient-centred care This guideline offers best practice advice on the care of adults, young people and children presenting at hospital with an acute painful sickle cell episode. Treatment and care should take into account patients’ needs and preferences. People with an acute painful sickle cell episode should have the opportunity to make informed decisions about their care and treatment, in partnership with their healthcare professionals. If patients do not have the capacity to make decisions, healthcare professionals should follow the Department of Health’s advice on consent and the code of practice that accompanies the Mental Capacity Act. In Wales, healthcare professionals should follow advice on consent from the Welsh Government. If the patient is under 16, healthcare professionals should follow the guidelines in ‘Seeking consent: working with children’. Good communication between healthcare professionals and patients is essential. It should be supported by evidence-based written information tailored to the patient’s needs. Treatment and care, and the information patients are given about it, should be culturally appropriate. It should also be accessible to people with additional needs such as physical, sensory or learning disabilities, and to people who do not speak or read English. If the patient agrees, families and carers should have the opportunity to be involved in decisions about treatment and care. Families and carers should also be given the information and support they need. Care of young people in transition between paediatric and adult services should be planned and managed according to the best practice guidance described in ‘Transition: getting it right for young people’. Adult and paediatric healthcare teams should work jointly to provide assessment and services to young people with an acute painful sickle cell episode. Diagnosis and management should be reviewed throughout the NICE clinical guideline 143 – sickle cell acute painful episode 6 transition process, and there should be clarity about who is the lead clinician to ensure continuity of care. NICE clinical guideline 143 – sickle cell acute painful episode 7 1 Recommendations 1.1 List of all recommendations Individualised assessment at presentation 1.1.1 Treat an acute painful sickle cell episode as an acute medical emergency. Follow locally agreed protocols for managing acute painful sickle cell episodes and/or acute medical emergencies that are consistent with this guideline. 1.1.2 Throughout an acute painful sickle cell episode, regard the patient (and/or their carer) as an expert in their condition, listen to their views and discuss with them:  the planned treatment regimen for the episode  treatment received during previous episodes  any concerns they may have about the current episode  any psychological and/or social support they may need. 1.1.3 Assess pain and use an age-appropriate pain scoring tool for all patients presenting at hospital with an acute painful sickle cell episode. 1.1.4 Offer analgesia within 30 minutes of presentation to all patients presenting at hospital with an acute painful sickle cell episode (see also recommendations 1.1.7 to 1.1.11). 1.1.5 Clinically assess all patients presenting at hospital with an acute painful sickle cell episode, including monitoring of:  blood pressure  oxygen saturation on air (if oxygen saturation is 95% or below, offer oxygen therapy)  pulse rate NICE clinical guideline 143 – sickle cell acute painful episode 8  respiratory rate  temperature. 1.1.6 Assess all patients with sickle cell disease who present with acute pain to determine whether their pain is being caused by an acute painful sickle cell episode or whether an alternative diagnosis is possible, particularly if pain is reported as atypical by the patient. Primary analgesia 1.1.7 When offering analgesia for an acute painful sickle cell episode:  ask about and take into account any analgesia taken by the patient for the current episode before presentation  ensure that the drug, dose and administration route are suitable for the severity of the pain and the age of the patient  refer to the patient’s individual care plan if available. 1.1.8 Offer a bolus dose of a strong opioid by a suitable administration route, in accordance with locally agreed protocols for managing acute painful sickle cell episodes, to:  all patients presenting with severe pain  all patients presenting with moderate pain who have already had some analgesia before presentation. 1.1.9 Consider a weak opioid as an alternative to a strong opioid for patients presenting with moderate pain who have not yet had any analgesia. 1.1.10 Offer all patients regular paracetamol and NSAIDs (non-steroidal anti-inflammatory drugs) by a suitable administration route, in addition to an opioid, unless contraindicated1. 1 The use of NSAIDs should be avoided during pregnancy, unless the potential benefits outweigh the risks. NSAIDs should be avoided for treating an acute painful sickle cell episode in women in the third trimester. See the ‘British National Formulary’ for details of contraindications. NICE clinical guideline 143 – sickle cell acute painful episode 9 1.1.11 Do not offer pethidine for treating pain in an acute painful sickle cell episode. Reassessment and ongoing management 1.1.12 Assess the effectiveness of pain relief:  every 30 minutes until satisfactory pain relief has been achieved, and at least every 4 hours thereafter  using an age-appropriate pain scoring tool  by asking questions, such as:  How well did that last painkiller work?  Do you feel that you need more pain relief? 1.1.13 If the patient has severe pain on reassessment, offer a second bolus dose of a strong opioid (or a first bolus dose if they have not yet received a strong opioid). 1.1.14 Consider patient-controlled analgesia if repeated bolus doses of a strong opioid are needed within 2 hours. Ensure that patient- controlled analgesia is used in accordance with locally agreed protocols for managing acute painful sickle cell episodes and/or acute medical emergencies. 1.1.15 Offer all patients who are taking an opioid:  laxatives on a regular basis  anti-emetics as needed  antipruritics as needed. 1.1.16 Monitor patients taking strong opioids for adverse events, and perform a clinical assessment (including sedation score):  every 1 hour for the first 6 hours  at least every 4 hours thereafter. NICE clinical guideline 143 – sickle cell acute painful episode 10