Top picks: 8 resources on sickle cell disease

Summary

Sickle cell disease can affect people of all backgrounds but is more common in African and Caribbean communities. It has become one of the fastest growing genetic conditions in England, with 250 new cases every year. People living with sickle cell disease may experience painful crises, fatigue and an increased risk of serious health complications. Despite this, the condition is often not well understood. 

At Patient Safety Learning we believe that sharing insights and learning is vital to improving outcomes and reducing harm. That's why we created the hub; to provide a space for people to come together and share their experiences, resources and good practice examples.

We have pulled together 8 useful resources about sickle cell disease that have been shared on the hub. They include recommendations on tackling inequalities, recognising and managing sickle cell disease in hospital, and managing pain.  

Content

1 Sickle Cell Society: Standards for the clinical care of adults with sickle cell disease in the UK

These standards for the clinical care of adults with sickle cell disease were produced by the Sickle Cell Society in collaboration with a broad multi-disciplinary group of healthcare providers, patients and support groups.

2 The King's Fund: The implicit bias of sickle cell disease

Although health outcomes have improved, evidence shows that people with sickle cell still feel marginalised. Their frustrations largely stem from a perceived lack of empathy in a healthcare system that does not fully recognise their struggles. When compounded with limited treatment pathways, these poor experiences leave many feeling neglected and unsupported. This blog from CJ Nwasike discusses sickle cell health inequalities.

3 Sickle cell comparative review to inform policy report: Providing evidence-based recommendations to tackle inequalities

This study analysed data across a wide range of indicators and revealed striking inequalities, which were often reflected in the experiences of patients living with sickle cell across the country. The report adds further to the urgent need to address the underlying problems affecting the quality of care for patients with sickle cell. Importantly, this comparative research shows that improvements are also needed for other severe chronic conditions, such as cystic fibrosis and haemophilia, and that lessons can be learned from successes achieved in other specialties. This report includes a set of recommendations that aim to help tackle inequalities associated with sickle cell in the UK.

4 HSSIB: Invasive procedures for patients with sickle cell disease

This investigation from the Health Services Safety Investigations Body sets out to review the care of patients with sickle cell disease who need to have an invasive procedure. Invasive procedures involve accessing the inside of a patient’s body, either through an incision (cut) or one of the body’s orifices. Specifically, the investigation focused on:

• how haematology teams – the specialists who treat people with blood disorders – are involved and informed when a patient with sickle cell disease is treated in another area of healthcare
• how patients with sickle cell disease are prepared for invasive procedures
• how and where clinical information relevant to the patient is shared.

5 HSSIB: Management of sickle cell crisis

In this investigation, HSSIB used a real patient safety incident to explore how sickle cell crises are managed within hospital settings. In particular, the investigation considered:

• the knowledge nursing staff may have about the care of patients in sickle cell crisis
• how patient-controlled analgesia (PCA) – where a patient can use a device to give themself doses of pain relief medication – is considered holistically, such as monitoring the patient and staff workload.

6 Sickle Cell Society: 'The difference between life and death'

Following on from the care failures highlighted in the 2021 report, 'No one's listening', this Sickle Cell Society report takes a deeper look at sickle cell nursing care. The findings show the need for vastly more resources, training and support in this critical area of care. The report highlights that not only is no-one listening, but that lives are still being put at risk.

7 Treating chronic pain in sickle cell disease — The need for a biopsychosocial model

Clinicians are often unsuccessful at addressing chronic pain in sickle cell disease. In this perspective article in the New England Journal of Medicine, Childerhose et al. discuss how a biopsychosocial model can help capture people’s experience of chronic pain by affirming that biologic, neuropsychological, and socioenvironmental elements play a role in pain-related processes. 

8 Sickle cell digital discovery report: Designing better acute painful sickle cell care

The report explores the range of technology that is in place for accident and emergency clinicians, red-cell specialists, and ambulance care, to aid timely support to patients with sickle cell on their emergency hospital arrival. A number of focus groups and interviews were carried out with those that have lived experience of the disease, including patients who have suffered acute, painful sickle cell episodes during NHS A&E admissions. Research found a lack of individual care plans in place and, more broadly, no clear definition of what constitutes an actual care plan. A number of recommendations are set out in the report for the NHS and the wider healthcare system.

Do you have a resource on sickle cell disease to share? We’d love to hear about it - leave a comment below or join the hub to share your own post.