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Sickle cell acute painful episode
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Introduction
This pathway covers the management of an acute painful sickle cell episode in patients presenting to hospital until discharge.
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.
Acute painful sickle cell episodes (also known as painful crises) are caused by blockage of the small blood vessels in people with sickle cell disease. These episodes are often unpredictable. Pain may vary in intensity, but can be excruciating. Repeated episodes may result in organ damage.
The management of acute painful sickle cell episodes is variable throughout the UK, and this is a frequent source of complaints from patients. Common problems include unacceptable delays in receiving analgesia, insufficient or excessive doses, inappropriate analgesia, and stigmatising the patient as drug seeking.
Areas covered in this pathway include 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.
Source guidance
The NICE guidance that was used to create the pathway.
Sickle cell acute painful episode. NICE clinical guideline 143 (2012)
Quality standards
Quality statements
Effective interventions library
Successful effective interventions library details
Implementation
Commissioning
These resources include support for commissioners to plan for costs and savings of guidance implementation and meeting quality standards where they apply.
These resources will help to inform discussions with providers about the development of services and may include measurement and action planning tools.
Service improvement and audit
These resources provide help with planning ahead for NICE guidance, understanding where you are now, and conducting improvement initiatives.
Pathway information
Information for the public
NICE produces information for the public that summarises, in plain English, the recommendations that NICE makes to healthcare and other professionals.
NICE has written information for the public explaining its guidance on:
Patient-centred care
Patients and healthcare professionals have rights and responsibilities as set out in the NHS Constitution for England – all NICE guidance is written to reflect these. Treatment and care should take into account individual needs and preferences. People should have the opportunity to make informed decisions about their care and treatment, in partnership with their healthcare professionals. If someone does not have the capacity to make decisions, healthcare professionals should follow the Department of Health's advice on consent, the code of practice that accompanies the Mental Capacity Act and the supplementary code of practice on deprivation of liberty safeguards. In Wales, healthcare professionals should follow advice on consent from the Welsh Government.
If the person is under 16, healthcare professionals should follow the guidelines in Seeking consent: working with children. If a young person is moving between paediatric and adult services their care should be planned and managed according to the best practice guidance described in the Department of Health's Transition: getting it right for young people.
Updates to this pathway
22 February 2013 Minor maintenance updates.
17 July 2012 Minor maintenance updates.
Supporting information
Glossary
The administration of a medication, drug or other compound that is given to raise its concentration to an effective level. Administration can be intravenous, intramuscular, intrathecal or subcutaneous.
A pathological condition in which a part of or the whole body is deprived of adequate oxygen supply.
Pain with a visual analogue scale (VAS) (or equivalent) score typically within the range of 4 to 7 (this description should not be interpreted as a strict definition and will not apply to all patients, as pain is subjective). [The VAS is a pain scoring tool measured on a linear scale from 0 to 10, with 0 indicating no pain.]
Patient-controlled analgesia (PCA) is a method of safely administering strong opioids which is controlled by the patient (or a nurse for nurse-controlled analgesia).
Pain with a visual analogue scale (VAS) (or equivalent) score typically above 7 (this description should not be interpreted as a strict definition and will not apply to all patients, as pain is subjective). [The VAS is a pain scoring tool measured on a linear scale from 0 to 10, with 0 indicating no pain.]
Adult, young person or child presenting at hospital with a suspected acute painful sickle cell episode
Adult, young person or child presenting at hospital with a suspected acute painful sickle cell episode
Treat as an acute medical emergency
Treat as an acute medical emergency
Treat as an acute medical emergency
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.
See the NICE pathway on acutely ill patients in hospital.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodeIndividualised assessment at presentation
Individualised assessment at presentation
Individualised assessment at presentation
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.
Assess pain and use an age-appropriate pain scoring tool for all patients presenting at hospital with an acute painful sickle cell episode.
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
- respiratory rate
- temperature.
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.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodeOffer analgesia within 30 minutes of presentation
Offer analgesia within 30 minutes of presentation
Offer analgesia within 30 minutes of presentation
Offer analgesia within 30 minutes of presentation to all patients presenting at hospital with an acute painful sickle cell episode.
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.
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.
Consider a weak opioid as an alternative to a strong opioid for patients presenting with moderate pain who have not yet had any analgesia.
Offer all patients regular paracetamol and NSAIDs (non-steroidal anti-inflammatory drugs) by a suitable administration route, in addition to an opioid, unless contraindicated. (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.)
Do not offer pethidine for treating pain in an acute painful sickle cell episode.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodeReassessment and ongoing management
Reassessment and ongoing management
Reassessment and ongoing management
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?
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).
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.
Offer all patients who are taking an opioid:
- laxatives on a regular basis
- anti-emetics as needed
- antipruritics as needed.
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.
If the patient does not respond to standard treatment for an acute painful sickle cell episode, reassess them for the possibility of an alternative diagnosis.
Do not use corticosteroids in the management of an uncomplicated acute painful sickle cell episode.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodePossible acute complications
Possible acute complications
Possible acute complications
Be aware of the possibility of acute chest syndrome in patients with an acute painful sickle cell episode if any of the following are present at any time from presentation to discharge:
- abnormal respiratory signs and/or symptoms
- chest pain
- fever
- signs and symptoms of hypoxia:
- oxygen saturation of 95% or below or
- an escalating oxygen requirement.
Be aware of other possible complications seen with an acute painful sickle cell episode, at any time from presentation to discharge, including:
- acute stroke
- aplastic crisis
- infections
- osteomyelitis
- splenic sequestration.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodeNon-pharmacological interventions
Non-pharmacological interventions
Non-pharmacological interventions
Encourage the patient to use their own coping mechanisms (for example, relaxation techniques) for dealing with acute pain.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodeStep down pharmacological treatment
Step down pharmacological treatment
Step down pharmacological treatment
As the acute painful sickle cell episode resolves, follow locally agreed protocols for managing acute painful sickle cell episodes to step down pharmacological treatment, in consultation with the patient.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodeDischarge information
Discharge information
Discharge information
Before discharge, provide the patient (and/or their carer) with information on how to continue to manage the current episode, including:
- how to obtain specialist support
- how to obtain additional medication
- how to manage any potential side effects of the treatment they have received in hospital.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodeSettings and training
Settings and training
Settings and training
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.
Where available, use daycare settings in which staff have specialist knowledge and training for the initial assessment and treatment of patients presenting with an acute painful sickle cell episode.
All healthcare professionals in emergency departments who care for patients with an acute painful sickle cell episode should have access to locally agreed protocols and specialist support from designated centres.
Patients with an acute painful sickle cell episode should be cared for in an age-appropriate setting.
For pregnant women with an acute painful sickle cell episode, seek advice from the obstetrics team and refer when indicated.
NICE has also produced a pathway on antenatal care, which includes guidance on planning a place of birth for women with sickle cell disease.
Source guidance
Failed to load fragment (default behaviour with no loader supplied): staticcontentfragments/source-guidance-nodePaths in this pathway
Pathway created: June 2012 Last updated: February 2013
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