Note from the National Guideline Clearinghouse (NGC): This guideline was developed by the Centre for Clinical Practice at the National Institute for Health and Clinical Excellence (NICE). See the "Availability of Companion Documents" field for the full version of this guidance.
Individualised Assessment at Presentation
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.
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.
Offer analgesia within 30 minutes of presentation to all patients presenting at hospital with an acute painful sickle cell episode (see also recommendations below).
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
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.
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 non-steroidal anti-inflammatory drugs (NSAIDs) 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.
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.
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.
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
- Signs and symptoms of hypoxia:
- Oxygen saturation of 95% or below
- 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
- Splenic sequestration
Management of Underlying Pathology
Do not use corticosteroids in the management of an uncomplicated acute painful sickle cell episode.
Encourage the patient to use their own coping mechanisms (for example, relaxation techniques) for dealing with acute pain.
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.
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