HSIB: Management of sickle cell crisis (22 June 2023)

Summary

Sickle cell disease is the name for a group of inherited red blood cell disorders that affect haemoglobin, which is a protein in red blood cells that carries oxygen through a person’s body. It mainly affects people from African or Caribbean backgrounds, though it can affect anyone. It affects approximately 15,000 people in the UK.

In November 2021, the All-Party Parliamentary Group for Sickle Cell and Thalassaemia published a report detailing the issues that people with sickle cell disease experience in relation to their care. The report made 31 recommendations to organisations across the healthcare system to help address these issues.

The Healthcare Safety Investigation Branch (HSIB) launched two investigations (see also: Invasive procedures for people with sickle cell disease) to find out what additional learning or knowledge could be added in this area and to provide further insights into the practical challenges patients with sickle cell disease may face when receiving NHS care.

HSIB used a real patient safety incident, referred to as ‘the reference event’, 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.

Content

The reference event was Tyrone, 46 years old, who experienced a sickle cell crisis in spring 2021 during the COVID-19 pandemic.

He called an ambulance because of the amount of pain he was experiencing. Tyrone was put onto a PCA pump, which was programmed to deliver morphine at a continuous dose (background rate) per hour, and also allowed him to press a button to deliver a dose at a regulated maximum rate.

He was in pain and was restless throughout the night, but he was found unresponsive the following morning. A post-mortem report stated that Tyrone died from cardiorespiratory failure (his heart and breathing stopped) caused by acute sickle cell crisis and morphine toxicity. The record of inquest stated that ‘the nursing staff had insufficient training to manage and monitor [Tyrone] and his PCA pump’.

Findings

• There is no minimum training requirement or nationally agreed content for Haemoglobinopathy Coordinating Centres to deliver to provide staff with knowledge about sickle cell disease or sickle cell crisis.
• Haemoglobinopathy Coordinating Centres are required to deliver training in line with their service specifications; however, they report finding it challenging to engage NHS trusts and NHS staff in this process in areas where the prevalence of sickle cell disease is lower.
• Trusts faced competing priorities during the COVID-19 pandemic and many trusts may have needed to alter the normal pathway of care for patients with sickle cell disease.
• Where reconfiguration of services requires patients in sickle cell crisis to be cared for in alternative wards/departments within the hospital or by new staff, there may not be the necessary equipment, knowledge or staff training required for care to be delivered safely.
• Staff workload, particularly during the COVID-19 pandemic and in emergency departments, impacted on the ability of staff to conduct increased monitoring requirements, such as the monitoring of patients on high-strength opiates delivered using a PCA pump.

Recommendation

HSIB recommends that NHS England reviews the existing training and competence requirements within sickle cell care provision and specifies the minimum training requirements and content for staff. The content can then be delivered by Haemoglobinopathy Coordinating Centres to increase knowledge about sickle cell disease and how to treat patients in sickle cell crisis.