Management of sickle cell crisis

HSIB legacy content

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

Background

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.

Reference event

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’.

National investigation

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.

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.

Safety recommendations

NHS England

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.

NHS England welcomes the HSIB report and recommendations and is committed to taking effective action in response.

In June 2022 we launched a dedicated programme to improve care for people with sickle cell disease (pwSCD). Sickle Cell e-learning module was developed as part of an end-to-end patient pathway review. We wanted to improve access to education and training for healthcare professionals and the wider community.

We have developed and launched an e-learning module 'Addressing Inequalities in Healthcare – Sickle Cell Disease'. This is an online resource for healthcare workers and the public, designed in collaboration with Health Education England, Academy of Medical Royal Colleges and other government departments. We are monitoring the uptake of the module and will assess its effectiveness in providing ongoing education to healthcare practitioners.

Alongside this we launched a National Campaign – 'Can you tell it’s sickle cell' for World Sickle Cell Day in 2022 and 2023. The campaign provided information on sickle cell disease for healthcare practitioners, patients and carers - and was designed to highlight common clinical presentations of Sickle Cell related emergencies e.g. Sickle Cell crisis and the need for prompt medical attention should these occur.

This work aligns with an effort to increase knowledge about sickle cell diseases and how to treat patients in sickle cell crisis.

NHS England commissions Specialist Haemoglobinopathy Teams (SHTs) to provide specialist clinical services for pwSCD who work with Local Hospital Teams (LHTs) in a networked arrangement to enable equitable access to high quality care. Each SHT is linked to a Haemoglobinopathy Coordinating Centre (HCC) which provide coordinated leadership for the network, supporting SHTs to deliver clinical care.

We have recently assessed the SHT and the HCC teams against the national service specifications, which set out the requirements and standards that providers of specialist haemoglobinopathy services must meet. The findings align with the HSIB recommendation in terms of the need to strengthen the network approach to education and training and specifically, to support the engagement of the LHTs.

Therefore, in response to the HSIB recommendation, we are committed to reviewing the service specifications and ensuring that an updated version is published that appropriately specifies the minimum training requirements and content for staff caring for patients living with sickle cell disease.

To inform this work, discussions will be held with some individual networks to understand current challenges to the delivery of training and opportunities for improvements. The HCC specification review will ensure there is an appropriate description of the HCC responsibility to deliver education and training across the network.

Actions planned to deliver safety recommendation:

NHS England will monitor the uptake of the e-learning SCD module after 12 months, by September 2024.
The NHS England Haemoglobinopathies Clinical Reference Group to review and revise the current SHT and HCC service specifications and incorporate appropriate reference to minimum training requirements and content for staff, by March 2024.
As part of the above process, review the existing training and competency requirements within sickle cell care provision, by March 2024.
To complement the above actions, work with individual networks to understand current challenges to the delivery of education and training and identify opportunities for improvement, by March 2024.

Response received on 31 November 2023.

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