NICE consults on new draft guideline on sickle cell

The healthcare guidance body NICE has today (Wednesday 8 February) opened a consultation on the draft of its first guideline on the management of an acute painful sickle cell episode in hospital. Sickle cell is one of the most commonly inherited serious genetic disorders in England.

The new draft guideline addresses the treatment and care of acute painful sickle cell episodes for patients presenting to hospital. The guideline also covers complications, and the information and support needs of patients.

Sickle cell affects between 12,500 - 15,000 people 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 disorder, it is impossible to automatically exclude anyone. The condition 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 routine 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.

Draft recommendations include:

  • Treat an acute painful sickle cell episode as an acute medical emergency, and follow locally agreed protocols that are consistent with this guideline.
  • 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

    - treatments received during previous episodes

    - any concerns they may have about the current episode

    - any psychological and/or social support they may need.
  • All healthcare professionals who care for patients with an acute painful sickle cell episode should receive regular training, with topics including:

    - pain monitoring and relief

    - the ability to identify potential acute complications

    - attitudes towards and preconceptions about patients presenting with an acute painful sickle cell episode.

Christine Carson, Programme Director, Centre for Clinical Practice at NICE said: "Sickle cell is more common than people might expect, so it is important that there are clear, evidence-based guidelines in place that can assist healthcare professionals in hospital in the treatment of an acute, painful episode. NICE is aware 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 reliefi, insufficient or excessive doses, and stigmatising the patient as drug seeking. I would urge all those registered as stakeholders in the development of this guideline to have their say on our draft recommendations, and submit their comments via the NICE website. For patients and carers it is recommended that they pass their comments to the registered stakeholder organisation that most closely represents their interests."

Anne Welsh, Chairperson, The Sickle Cell Society said: "Pain is the most common cause of morbidity in sickle cell disease and is associated with an increased mortality rate. Patients have varying episodes of pain as well as experiencing a psychological impact. Treatment can pose a challenge to clinicians and because most hospitals have developed their own models of care, the standard of treatment varies geographically. There have been persistent complaints from patients regarding trust and respect, stigmatisation and poor treatment generally. Patients are increasingly dissatisfied with services provided and this is worse in less prevalent areas of the disease. It is therefore an encouraging way forward for NICE to develop these guidelines; the current consultation exercise involves patients, stakeholder organisations, and clinicians. The guidelines will help ensure an authoritative standard of care for people with sickle cell disease. This will help improve their healthcare, overall quality of life and satisfaction with their treatment."

The draft guideline consultation runs from Wednesday 8 February to Wednesday 7 March.

Publication of the final guideline is expected in June 2012.


Notes to editors

1. The draft guideline is available from Wednesday 8 February at:

2. Further details on clinical guideline development.

3. For more information on the Sickle Cell Society, please see their website.


i The guideline recommends that analgesia be offered within 30 minutes of presentation to all patients presenting at hospital with an acute painful sickle cell episode.

About NICE

1. The National Institute for Health and Care Excellence (NICE) is the independent organisation responsible for providing national guidance and standards on the promotion of good health and the prevention and treatment of ill health.

2. NICE produces guidance in three areas of health:

  • public health - guidance on the promotion of good health and the prevention of ill health for those working in the NHS, local authorities and the wider public and voluntary sector
  • health technologies - guidance on the use of new and existing medicines, treatments, medical technologies (including devices and diagnostics) and procedures within the NHS
  • clinical practice - guidance on the appropriate treatment and care of people with specific diseases and conditions within the NHS.

3. NICE produces standards for patient care:

  • quality standards - these reflect the very best in high quality patient care, to help healthcare practitioners and commissioners of care deliver excellent services
  • Quality and Outcomes Framework - NICE develops the clinical and health improvement indicators in the QOF, the Department of Health scheme which rewards GPs for how well they care for patients.

4. NICE provides advice and support on putting NICE guidance and standards into practice throughits implementation programme, and it collates and accredits high quality health guidance, research and information to help health professionals deliver the best patient care through NHS Evidence.

This page was last updated: 08 February 2012

Accessibility | Cymraeg | Freedom of information | Vision Impaired | Contact Us | Glossary | Data protection | Copyright | Disclaimer | Terms and conditions

Copyright 2014 National Institute for Health and Care Excellence. All rights reserved.

Selected, reliable information for health and social care in one place

Accessibility | Cymraeg | Freedom of information | Vision Impaired | Contact Us | Glossary | Data protection | Copyright | Disclaimer | Terms and conditions

Copyright 2014 National Institute for Health and Care Excellence. All rights reserved.