Invasive procedures in patients with sickle cell disease

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.

This investigation set 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.

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: Management of sickle cell crisis) to find out what additional learning or knowledge could be added in this area and to provide further insights into the practical challenges that patients with sickle cell disease may face when receiving NHS care.

The investigation used a real patient safety incident to explore the issues identified. The case involved a woman who had an invasive procedure which resulted in her being admitted to hospital for sickle cell crisis. This incident is referred to as ‘the reference event’ and was used to examine national issues.

Reference event

The patient, who was 45 years old at the time of the incident, had a history of sickle cell disease that had led to her being admitted to hospital multiple times. For approximately 3 years she had been receiving regular exchange blood transfusions (transfusions that replaced her blood with donated blood) to help treat the symptoms of her sickle cell disease.

She had heavy, painful and frequent menstrual periods and was scheduled for a surgical procedure to help ease her symptoms. The procedure did not have the desired effect and about 16 months later the patient was scheduled to have a further procedure to again try to ease her symptoms.

The haematology team were made aware of the procedure but were not told when it was occurring, the procedure was conducted 1 week before the patient was due to have her regular exchange transfusion. Her haemoglobin levels were low.

The patient experienced a significant amount of pain during the procedure and was transferred to the haematology ward afterwards for treatment of a sickle cell crisis.

National investigation

The national investigation sought to understand how patients with sickle cell disease are prepared for scheduled invasive procedures and how teams within hospitals communicate and share clinical information about patients with sickle cell disease.

The investigation also looked at national networks and guidelines to understand how clinicians were supported in decision making and providing the best care available to patients with sickle cell disease. This was with the aim of generating additional information and action to that already identified in the All-Party Parliamentary Group report.

Findings

The treatment people with sickle cell disease receive varies depending on where they live and the number of patients that clinicians see.
Guidance on the treatment of sickle cell disease is limited to specific circumstances, such as sickle cell crisis or blood transfusion, with limited wider guidance available on the through-life management of sickle cell disease.
Compared to other conditions that affect similar numbers of people, there has been limited research into the lifelong management of sickle cell disease. This may indicate a health inequality.
There is no national information system for storing and sharing clinical information for patients with sickle cell disease.
Currently the main way in which haematology consultants are made aware that a patient with sickle cell disease is having treatment in another area of healthcare is when they are notified of this by the patient.
It may be possible to set up healthcare IT systems so that they alert haematology consultants when one of their patients accesses another type of healthcare. However, consultants in areas with a high prevalence of sickle cell disease may then receive so many alerts that such a system would be ineffective.

Safety recommendations

NHS England

HSIB recommends that NHS England identifies and implements a system for sharing clinical information about patients with sickle cell disease to improve access to this information for clinicians, and reduce the risk of a patient’s sickle cell disease not being treated consistently.

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 persons with sickle cell disease (pwSCD). We reviewed the end-to-end patient pathway and identified opportunities to improve care quality, including greater consistency in patient records keeping. Inconsistencies in patient record keeping can lead to sub-optimal assessment of patients’ needs, particularly in an emergency setting when a care plan is either not available or inaccessible.

NHS England, as part of a wider Sickle Cell Quality Improvement drive is piloting the upload of digital care plans for pwSCD across existing interoperable digital platforms in London and Manchester, and gaps in the flagging of and sharing clinical information of pwSCD. These pilots will be evaluated and consideration given to wider deployment.

In addition, we have produced and launched a SCD Patient Card, making a patient card available for every person living with sickle cell that wishes to carry one. If presented at A&E, the card will enable a healthcare professional to rapidly access a patient’s sickle cell crisis care plan. The launch of the new card formed part of this year’s ‘Can You Tell It’s Sickle Cell?’ campaign announced on World Sickle Cell Day on 19 June 2023.

Our work to ensure every patient has a digital care plan and a SCD patient card aims to mitigate some potential underlying bias pwSCD may face when waiting for or receiving treatment. It also aims to empower pwSCD and ensure they have agency to advocate and take an active role in their care plan.

Actions planned to deliver safety recommendation:

Monitor the dissemination and use of SCD Patient Cards, by September 2024.
Commission and contract teams to review and develop digital care plans for the management of vaso-occlusive crisis (VOC) for pwSCD in England, by March 2025.
Enable access of VOC care plans for pwSCD across interoperable platforms in regions that have the platforms (London and Manchester). This will be evaluated and be scaled up across regions if effective in improving access to care plans.

Response received on 30 November 2023.

National Institute for Health and Care Research

HSIB recommends that the National Institute for Health and Care Research assesses the priority and feasibility of commissioning research into the management of patients with sickle cell disease, encompassing the requirements of patient-controlled-analgesia (pain relief) pumps. This will contribute towards building an evidence base for the generation of national clinical guidance and will reduce unnecessary variance in treatment for patients with sickle cell disease.

The National Institute for Health and Care Research (NIHR) funds health and social care research that improves people’s health and wellbeing. Working with those who use, plan and deliver health services in the UK, we identify and prioritise important topics to fund through specific calls for research.

Following referral from HSIB, the safety recommendation for ‘Invasive procedures for people with sickle cell disease’ will be considered through the NIHR’s established research commissioning processes with the aim of developing practical and deliverable research calls which will provide the evidence needed by decision makers. After an initial ‘in-house’ assessment of the topic to determine what is already known (the existing evidence base), we will engage with a wide range of individuals with a broad spectrum of knowledge, skills and expertise to develop research question(s) which address outstanding uncertainties.

Our engagement will include patients, members of the public, academics, subject experts, clinical staff, service managers and public health professionals. The research question will then be considered by the NIHR’s relevant programme specific prioritization committees who review and advise on the development of NIHR commissioned calls and make recommendations about future advertisements.