NICE standard urges healthcare professionals to improve care for people experiencing painful sickle cell episodes

The National Institute for Health and Care Excellence (NICE) is publishing new standards to raise the bar in the care and support of people experiencing sickle cell acute painful episodes from the minute they present to hospital until they are discharged.

In people with sickle cell disease, their red blood cells can change in shape and texture becoming like sickles, or crescents instead of being round and flexible. These irregular cells do not move around blood vessels easily and have a reduced capacity for carrying oxygen around the body. A sickle cell episode occurs when the abnormal cells block small blood vessels leading to tissue damage around affected areas which can cause excruciating pain. Sickle cell episodes are often unpredictable in nature and can vary from one a year to people experiencing severe pain every week.

With an estimated 240,000 genetic carriers and up to 15,000 people living with the condition in the UK, sickle cell is one of the most commonly inherited serious genetic diseases in England. It is more common among people of African and African-Caribbean descent, but it can affect any ethnic group, and as a genetic disease which can be inherited, it is impossible to automatically exclude anyone.

The NICE standard aims to improve the care and management of people presenting to hospital with painful sickle cell episodes. Building on the NICE clinical guideline on the management of acute painful sickle cell episodes, the standard sets out six concise statements which need to be prioritised in order to enhance quality of life for people living with this long-term condition and ensure that they have a positive experience of care. In summary:

  • To ensure a painful sickle cell episode does not escalate and cause unnecessary distress, acute pain must be recognised as soon as possible and pain relief given promptly and safely. Therefore the standard states that people who present at hospital with a sickle cell acute painful episode should have a thorough assessment and be given appropriate pain relief within 30 minutes. They should then be assessed regularly until satisfactory pain relief has been achieved with careful monitoring of adverse events in those who are taking strong opioids.
  • Acute chest syndrome, a lung-related complication that can lower the levels of oxygen in the blood, can be life-threatening in people with sickle cell disease, yet it is not always recognised. People with a sickle cell acute painful episode should be assessed for symptoms such as chest pain, fever and abnormal respiratory signs which may lead to this possible complication.
  • To ensure high quality care for all people with a sickle cell acute painful episode, healthcare professionals need to have access to locally agreed protocols on treatment and management as well as support from specialist centres. Healthcare professionals should also provide patients with clear written information to encourage involvement in their continuing care.

Professor Gillian Leng, Deputy Chief Executive and Director of Health and Social Care at NICE said: “As an inherited disease the prevalence of sickle cell disease will continue to increase with new births in the UK. We know that the management of this condition is variable across the country and there is a need to address patient concerns such as unacceptable delays in receiving pain relief. This new standard will drive up the quality of care people with sickle cell receive, so that they can be confident they will be comfortable during their stay in hospital.”

Dr Jo Howard, Consultant Haematologist, Guy's and St Thomas' NHS Foundation Trust and member of the committee which developed the standards said:“For people with sickle cell disease experiencing acute pain, any delay in assessing their condition and providing appropriate pain relief will cause unnecessary distress and make their condition worsen. In this standard, the recommendations from the 2012 acute sickle cell guideline are set out as measurable quality statements which commissioners, service providers and healthcare professionals can all use to ensure they are providing the best possible care for these patients.”

Hellen Adom, sickle cell patient and member of the committee which developed the standards said: “Most sickle cell episodes can be managed at home, however there are times that people will experience uncontrollable pain which means they have to go to hospital. Experiencing that kind of pain is difficult enough, and they shouldn't have to worry about being treated quickly and safely. As an integral part of the committee, I learnt that the patient's voice or input can bring valuable advice and direction to the group ensuring that people with sickle cell disease receive the best care, at a time when they need it the most.”


Notes to Editors

About the quality standard

1. The NICE quality standard for sickle cell acute painful episode is available on the NICE website.

2. The NICE quality standard for sickle cell acute painful episode is based on the following NICE accredited guidelines:

Sickle cell acute painful episode. NICE clinical guideline CG143 (2012)

3. NICE quality standards describe high-priority areas for quality improvement in a defined care or service area. They are derived either from NICE guidance or guidance from other sources that have been accredited by NICE, and apply right across the NHS in England.

Related NICE guidelines and quality standards


4. Patient experience in adult NHS services. NICE quality standard 15 (2012).

Future quality standards

5. Pain management (young people and adults)

About NICE quality standards

NICE quality standards aim to help commissioners, health care professionals, social care and public health practitioners and service providers improve the quality of care that they deliver.

NICE quality standards are prioritised statements designed to drive measurable quality improvements within a particular area of health or care. There is an average of 6-8 statements in each quality standard.

Quality standards are derived from high quality evidence-based guidance, such as NICE guidance or guidance from NICE accredited sources, and are produced collaboratively with health care professionals, social care and public health practitioners, along with their partner organisations, patients, carers and service users.

NICE quality standards are not mandatory but they can be used for a wide range of purposes both locally and nationally. For example, patients and service users can use quality standards to help understand what high-quality care should include. Health care professionals and social care and public health practitioners can use quality standards to help deliver high quality care and treatment.

NICE quality standards are not requirements or targets, but the health and social care system is obliged to have regard to them in planning and delivering services, as part of a general duty to secure continuous improvement in quality.

Quality standard topics are formally referred to NICE by NHS England (an executive non-departmental public body, established in October 2012) for health-related areas, and by the Department of Health and Department for Education for areas such as social care and public health.

About NICE

The National Institute for Health and Care Excellence (NICE) is the independent body responsible for driving improvement and excellence in the health and social care system. We develop guidance, standards and information on high-quality health and social care. We also advise on ways to promote healthy living and prevent ill health.

Formerly the National Institute for Health and Clinical Excellence, our name changed on 1 April 2013 to reflect our new and additional responsibility to develop guidance and set quality standards for social care, as outlined in the Health and Social Care Act (2012).

Our aim is to help practitioners deliver the best possible care and give people the most effective treatments, which are based on the most up-to-date evidence and provide value for money, in order to reduce inequalities and variation.

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This page was last updated: 09 April 2014