NICE recommends acute sickle cell episodes to be treated as medical emergencies in new guideline

In a new guideline published today (Wednesday 27 June), NICE, the healthcare guidance body, has recommended that acute painful sickle cell episodes should be treated as acute medical emergencies, and analgesia offered to all patients within 30 minutes of presentation. Sickle cell is one of the most commonly inherited serious genetic diseases in England.

The guideline focuses on the management, until discharge, of acute painful sickle cell episodes (also known as crises) in patients presenting to hospital. The guideline also covers complications, and the information and support needs of patients.

There are between 12,500 - 15,000 people with sickle cell disease 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 disease, the shape and texture of the blood cells can change, and they become rigid and sticky, and, as the name of the condition implies, shaped like sickles, or crescents. The cells do not survive as long as usual blood cells so that people do not have enough red blood cells and become anaemic. Symptoms may include intense pain and severe anaemia, and the condition can cause damage to major organs and infections. There is no routine cure for sickle cell disease but patients can be supported to manage their pain, and with regular monitoring life threatening complications such as stroke can be avoided.

Key recommendations include:

  • Treat an acute painful sickle cell episode as an acute medical emergency, and follow locally agreed protocols for managing the episodes that are consistent with this guideline.
  • Assess pain and use an age-appropriate pain scoring tool for all patients presenting at hospital with an acute painful sickle cell episode.
  • Offer analgesia within 30 minutes of presentation to all patients presenting at hospital with an acute painful sickle cell episode.
  • 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.

Professor Mark Baker, Director of the Centre for Clinical Practice at NICE said: “The prevalence of sickle cell is increasing in the UK and there are currently between 12,500 and 15,000 people with the disease. It is important, therefore, that there are clear, evidence-based guidelines in place that can aid healthcare professionals in the care and treatment of an acute, painful episode. We are 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 relief, insufficient or excessive doses, and stigmatising the patient as drug seeking. We hope that this new guideline will be welcomed by both patients and healthcare professionals alike, and that it will help improve the care for people experiencing acute painful sickle cell episode.”

Damien Longson, Consultant Liaison Psychiatrist, Manchester Mental Health and Social Care Trust and chair of the guideline development group said: “I was very pleased to be involved in the development of this guideline. Painful sickle cell episodes can be debilitating and very upsetting for those affected. These new guidelines cover the principles of how to manage such crises in hospital as well as the information and support needs for patients.”

Hellen Adom, patient/carer member on the guideline development group said: “I was diagnosed with sickle cell disease at birth, although I didn't realise my condition until I was 16 (I'm now in my 40s), so I know how incredibly painful and distressing an acute sickle cell episode can be. The guideline development group found the firsthand experience of sickle cell disease I was able to bring to the table extremely useful in understanding the condition from a patient's perspective and thereby enabling the development of a guideline that properly reflects the needs of patients, as well as being helpful to healthcare professionals in hospitals involved in the management of those with sickle cell.”

The new guideline is available from Wednesday 27 June at: .


Notes to editors

1. The new guideline is available from Wednesday 27 June at: .

Embargoed copies are available on request; please contact the press office.

2. For further details on clinical guideline development, please see the NICE website.

Related NICE guidelines

3. Opioids in palliative care. NICE clinical guideline 140 (2012).

4. Patient experience in adult NHS services. NICE clinical guideline 138 (2012).

5. Depression in adults with a chronic physical health problem. NICE clinical guideline 91 (2009).

6. Antenatal care. NICE clinical guideline 62 (2008).

7. Intrapartum care. NICE clinical guideline 55 (2007).

8. Acutely ill patients in hospital. NICE clinical guideline 50 (2007).

About NICE

9. 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.

10. 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.

11. 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.

12. NICE provides advice and support on putting NICE guidance and standards into practice through its 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: 27 June 2012