The National Institute for Health and Care Excellence (NICE) has issued draft standards that aim to improve the care and management of people presenting to hospital with sickle cell crises. Sickle cell is one of the most commonly inherited serious genetic diseases in England.

There are between 12,500 - 15,000 people with sickle cell in the UK, with an estimated 240,000 genetic carriers. It is more prevalent among people of African and African-Caribbean descent, but it can affect any ethnic group, and as it is an inherited genetic disease it is impossible to automatically exclude anyone. Sickle cell affects the normal oxygen carrying capacity of red blood cells, which should be round and flexible, allowing them to move around the body easily. But in people with sickle cell, the shape and texture of the blood cells can change, and they become hard and sticky, and, as the name of the condition implies, shaped like sickles, or crescents. The cells die more quickly than usual blood cells so that people do not have enough red blood cells. Symptoms may include severe anaemia and intense pain, and the condition can cause damage to major organs and infections. There is no cure for sickle cell but patients can be supported to manage their pain, and with regular monitoring life threatening complications such as stroke can be avoided.

A sickle cell crisis (also known as an episode) is triggered when the abnormal blood cells block the small blood vessels that supply the body's tissues. This causes the cells in the affected tissue to be damaged, resulting in inflammation which irritates nearby nerve endings. Episodes can last from a few minutes to several weeks with an average duration of around five to seven daysⁱ.

The draft quality standard is based on the NICE clinical guideline on the management of acute painful sickle cell episodes in hospital.

In summary the draft standard states:

• A thorough assessment of people who present at hospital with an acute painful sickle cell episode is needed to ensure people have an accurate diagnosis and are given appropriate pain relief within 30 minutes of presentation.
• People with an acute painful sickle cell episode should have an assessment of pain relief every 30 minutes until satisfactory pain relief has been achieved and then at least every 4 hours.
• Acute chest syndrome (ACS) is a major cause of morbidity and mortality in people with sickle cell disease and is often missed as a possible complication. People with an acute painful sickle cell episode should be assessed for acute chest syndrome if they have 1 or more of the following: abnormal respiratory signs or symptoms, chest pain, fever, or signs and symptoms of hypoxiaⁱⁱ.

Professor Gillian Leng, Deputy Chief Executive and Director of Health and Social Care at NICE said: "There are currently around 15,000 people with sickle cell in the UK, but because it is becoming more prevalent, it is important that there are clear standards in place for healthcare professionals and managers to help improve the care and treatment of those experiencing sickle cell crises. We know that the management of this condition in hospitals is variable throughout the UK, and this is a frequent source of complaints from patients. Common problems include unacceptable delays in receiving pain relief, insufficient or excessive doses, and stigmatising the patient as drug seeking. I would urge all those registered stakeholders to submit their comments on the draft standards through the NICE website."

The draft NICE quality standard for the management of people presenting to hospital with sickle cell crisis is available for consultation on the NICE website from 00:01 hrs on Tuesday 19 November. The consultation will close on Tuesday 17 December 2013.

For more information, please call the NICE press office on 0845 003 7782 or out of hours on 07775 583 813, or email pressoffice@nice.org.uk

Ends

Notes to Editors

References

i. NHS Choices

ii. Hypoxia is a medical condition caused by low oxygen levels in the blood.

About the quality standard

1. The draft NICE quality standard for the management of people presenting to hospital with sickle cell crisis is available on the NICE website from 00:01 hrs on Tuesday 19 November. The consultation will close on Tuesday 17 December 2013.

2. The draft quality standard is based on the NICE clinical guideline on the management of acute painful sickle cell episodes:

• Sickle cell acute painful episode. NICE clinical guideline 143 (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.

4. NICE is today also publishing draft quality standards for consultation on conduct disorders in children and young people, and infection control.

Related NICE guidelines and quality standards

Published

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

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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