Management of sickle cell crisis

This investigation was carried out by the Healthcare Safety Investigation Branch (HSIB). Find out more about HSIB legacy.

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.

Normally, red blood cells are disc shaped and flexible, enabling them to move easily through the blood vessels carrying oxygen. People with sickle cell disease have crescent or ‘sickle’ shaped red blood cells, which do not bend or move easily and can therefore block blood flow to the rest of their body. If this happens, it can cause a number of symptoms, including episodes of acute pain known as ‘sickle cell crisis’.

Sickle cell disease mainly affects people from African or Caribbean backgrounds, though it can affect anyone. It affects approximately 15,000 people in the UK.

Episodes of sickle cell crisis often require a hospital visit where the pain can be managed with strong analgesia (pain relieving medication) such as morphine.

On average, patients with sickle cell disease will experience a sickle cell crisis 1 to 3 times a year. However, some people may experience them every few weeks, whereas others can go for years without experiencing them.

This investigation 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.

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.

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.

Tyrone, who was 46 years old, 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. The ambulance staff gave Tyrone morphine to relieve the pain and took him to hospital. He was admitted via the emergency department and then moved around the hospital, while having COVID-19 tests, before eventually being treated on a haematology ward (a ward specialising in the treatment of blood disorders).

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. Attempts to revive him were unsuccessful. 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’.

The national investigation sought to understand the role of the national healthcare networks that care for people with sickle cell disease, which are facilitated by regional Haemoglobinopathy Coordinating Centres.

In particular it explored the delivery and content of training for staff by these networks. It also looked at staff awareness of sickle cell disease in areas of high and low prevalence of the disease, and how that impacted upon care delivery.

• 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.