Hematology

Management of an acute painful sickle cell episode inhospital: summary of NICE guidance

BMJ 27 June 2012
د. حسين محمد جمعه
اختصاصي الامراض الباطنة
البورد العربي
كلية طب الموصل
2012

This is one of a series of BMJ summaries of new guidelines based on the best available evidence; they highlight important recommendations for clinical practice, especially where uncertainty or controversy exists.

Sickle cell disease comprises a group of lifelong, inherited conditions of haemoglobin formation. Although the sickle gene is found in all ethnic groups, most people affected with sickle cell disease are of African or African-Caribbean origin. In the United Kingdom 12 500 to 15 000 people have sickle cell disease,1 and its prevalence is increasing because of immigration
into the UK, new births, and diagnostic screening programmes.

Acute painful sickle cell episodes (vaso-occlusive episodes) occur as a result of changes in the red blood cells that may cause tissue ischaemia and pain. These painful episodes may be triggered by factors or conditions such as dehydration, fever,
or hypoxia, but can occur unpredictably, with variable intensity and frequency, and at times the pain can be excruciating.


Although most episodes can be successfully managed at home, patients with uncontrolled pain may need to seek hospital care.
However, the management of such episodes in hospital is thought to vary throughout the UK, and common problems include unacceptable delays in receiving analgesia, insufficient or excessive doses, inappropriate analgesia, and stigmatising the patient as drug seeking.

This article summarises the most recent recommendations from the National Institute for Health and Clinical Excellence (NICE) on the management of an acute painful sickle cell episode in hospital.

Recommendations

NICE recommendations are based on systematic reviews of best available evidence and explicit consideration of cost effectiveness. When minimal evidence is available,
recommendations are based on the Guideline Development Group’s experience and opinion of what constitutes good practice.

Evidence levels for the recommendations are given in italic in square brackets.

Individualised assessment of all patients with
sickle cell disease presenting with acute pain
• Treat the episode as an acute medical emergency. Follow
locally agreed protocols for managing an acute painful
sickle cell episode and/or an acute medical emergency that are consistent with this guideline.
[Based on the experience and opinion of the Guideline Development Group (GDG)]

• Throughout the episode, regard patients (and/or their carers)

as experts 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.
[Based on evidence from qualitative studies, questionnaire data,and the experience and opinion of the GDG]


• Assess pain and use an age appropriate pain scoring tool.
• Offer analgesia within 30 minutes of presentation.
• Clinically assess all patients, including monitoring of blood pressure; pulse rate; respiratory rate, oxygen saturation on air (if oxygen saturation is ≤95%, offer oxygen therapy); 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.

[The above four points are based on moderate to very low

quality evidence from prognostic studies and the experience
and opinion of the GDG]
Primary analgesia
• When offering analgesia, 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. [Based on the experience and opinion of the GDG]

• Offer a bolus dose of a strong opioid by a suitable administration route, in accordance with locally agreed protocols for acute painful sickle cell episodes, to all patients presenting with severe pain and to 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 by a suitable administration route, in addition to an opioid, unless contraindicated.
[The above three points are based on moderate quality evidence from randomised controlled trials and the experience and opinion of the GDG]
• Do not offer pethidine to treat pain in an acute painful sickle cell episode. [Based on the experience and opinion of the GDG]

Reassessment and continued management of pain

• Assess the effectiveness of pain relief:
-Every 30 minutes until satisfactory pain relief has been achieved, and at least every four hours thereafter.
-Using an age appropriate pain scoring tool, and
-By asking questions such as “How well did that last
painkiller work?” and “Do you feel that you need more
pain relief?”


• If the patient has severe pain on reassessment, offer asecond 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 two hours.
Ensure that patient controlled analgesia is used in
accordance with locally agreed protocols for acute painful sickle cell episodes.

• Offer all patients who are taking an opioid laxatives on a regular basis; antiemetics as needed; antipruritics as needed.
• Monitor patients taking strong opioids for adverse events,and carry out a clinical assessment (including sedation score) every hour for the first six hours and at least everyfour hours thereafter.
• If the patient does not respond to standard treatment for anacute painful sickle cell episode, reassess him or her for apossible alternative diagnosis.

• As the acute painful sickle cell episode resolves, follow locally agreed protocols for acute painful sickle cell episodes to step down drug treatment, in consultation with the patient.
[All the above points are based on moderate to low quality
evidence from randomised controlled trials and the experience
and opinion of the GDG]

Possible acute complications

• Be aware of the possibility of acute chest syndrome (the presence of a new pulmonary infiltrate in combination with clinical signs and symptoms) 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 ≤95%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.
[The above points are based on moderate to very low quality evidence from prognostic studies and the experience and opinion
of the GDG]

Management of underlying disease

• Do not use corticosteroids in the management of an uncomplicated, acute painful sickle cell episode as there may be a risk of long term toxicity and there is little evidence of benefit outweighing possible harm.

[Based on moderate to low quality evidence from randomised controlled trials and the experience and opinion of the GDG]

Non-drug interventions

• Encourage patients to use their own coping mechanisms
(for example, relaxation techniques) for dealing with acute
pain.
[Based on low quality evidence from one randomised controlled trial and the experience and opinion of the GDG]
Settings and training
• All healthcare professionals who care for patients with an acute painful sickle cell episode should receive regular
training on pain monitoring and relief; identification of
potential acute complications; and attitudes towards and
preconceptions about patients presenting with such episodes.


• 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 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.
[All the above points are based on very low quality evidence from observational studies and the experience and opinion of the GDG]

Discharge information

• Before discharge, provide patients (and/or their carers) with information on how to continue to manage the current episode, including how to obtain specialist support; how to obtain additional medication; and how to manage any potential side effects of the treatment they have received in hospital.

[Based on evidence from qualitative studies,

questionnaire data, and the experience and opinion of the GDG]

Overcoming barriers

The main aim of this short clinical guideline is to ensure that
all patients who present to hospital with an acute painful sickle
cell episode are offered appropriate, safe, and prompt pain
management. Although healthcare professionals may be cautious
in their use of strong opioids, we recommend that this is the
primary analgesia for patients presenting with severe pain and
those with moderate pain who have already received analgesia.
Patients presenting to hospital with an acute painful episode
should initially have their pain monitored every 30 minutes until
the pain is under control. Although this may be considered time
consuming, this extent of observation is imperative to identify
any complications that can arise.