Sickle cell disease: managing acute painful episodes in hospital

Reasons for the decision

This review considered new evidence on pharmacological interventions for managing an acute painful sickle cell episode (vaso-occlusive crises) in children, young people and adults in a hospital setting. There was insufficient evidence on licensed medicines for this indication to support an update of the recommendations.

For further details and a summary of all evidence identified in surveillance, see appendix A.

Exceptional surveillance review summary

A pain crisis is the most common problem of sickle cell disease and can require several treatments at once, usually in an emergency. The priority is to control the pain, this can be done using medicines (such as opioids, non-steroidal anti-inflammatories and paracetamol).

Reason for the exceptional review

NICE was contacted by NHS England and asked to check if the evidence-base of analgesia for managing an acute painful sickle cell episode had developed sufficiently to support an update to the NICE guideline on sickle cell disease.

Methods

The exceptional surveillance process consisted of:

For further details about the process and the possible update decisions that are available, see ensuring that published guidelines are current and accurate in developing NICE guidelines: the manual.

Search and selection strategy

We searched for new evidence that relates to primary analgesia (including timing) for acute painful sickle cell episodes in hospital, which is covered by 3 areas of the guideline: individualised assessment at presentation, primary analgesia and reassessment and ongoing management.

We found 11 studies in a search for randomised controlled trials (RCTs) and systematic reviews published between 16 February 2016 and 3 February 2023. This included 6 RCTs and 5 systematic reviews. The systematic reviews were all limited by a small number of included studies. There was also heterogeneity across the available evidence base with respect to populations, interventions and settings. Additionally, most studies were small and none of the RCTs were from UK settings.

See appendix A for details of all evidence considered and references.

Ongoing research

We checked for relevant ongoing research; of the ongoing studies identified, 2 were assessed as relevant to the recommendations. Therefore, we will monitor these ongoing studies and will evaluate the impact of the results on current recommendations as soon as the studies have published the findings. These studies are:

See ensuring that published guidelines are current and accurate in developing NICE guidelines: the manual for more details on our consultation processes.

Other relevant NICE guidance

The following NICE technology appraisal guidance is available:

The following guidance topics are in development:

Equalities

People with sickle cell disease face health inequalities because the condition is not well understood, results in disability, and is more common in people of African or African-Caribbean family origin, who tend to also have poorer health outcomes than other ethnicities.

As part of the intelligence gathering for this review we identified the recent All Party Parliamentary Group (APPG) enquiry into avoidable deaths and failures of care for sickle cell patients in secondary care, No one's listening (2021).

The APPG report highlights a distinct lack of investment in sickle cell research and medicines. The current review has sought to identify current and ongoing research within the scope of the surveillance check, as outlined above.

An equalities and health inequalities assessment was completed during this surveillance review. See appendix B for details.

Overall decision

After considering all evidence and other intelligence and the impact on current recommendations, we decided that no update is necessary at this time. However, we will continue monitoring the ongoing studies.

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