Management of sickle cell crisis

HSIB launched two investigations which aimed to add to the understanding of the care provided to patients with sickle cell disease. This is the second of the two investigation reports. It focuses on the care of people who are experiencing sickle cell crisis. The first report looked at the care provided to people with sickle cell disease when they have invasive procedures, which involve accessing the inside of a patient’s body, either through an incision (cut) or one of the body’s orifices.

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 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.

1.1.1 Haematology is the study of disorders relating to the blood and bone marrow. This covers red and white blood cells, and the platelets and coagulation systems which cause blood to clot.

Red cells and white cells

1.1.2 Red blood cells are the most common blood cells in the body. Their main function is to transport oxygen from the lungs to other parts of the body. Sickle cell disease is a red blood cell disease.

1.1.3 White blood cells help fight infections and other diseases as part of the body’s immune system. White blood cell diseases include various types of blood cancer such as lymphoma and leukaemia.

1.2.2 Sickle cell disease can lead to a wide range of complications. These include:

Sickle cell crisis or vasculo-occlusive crisis – which are episodes of acute pain. These can affect a particular part of a person’s body, often major joints (knee, shoulder, hip) and the torso including the stomach and chest. A sickle cell crisis can bring on reduced oxygen levels and vice versa. People in sickle cell crisis require prompt treatment with pain relief, often opiate medication (morphine or similar medications), which should improve their symptoms.

Sickle cell anaemia – where a person has low levels of haemoglobin, which means their body doesn’t get enough oxygen. This can increase the risk of sickle cell crisis or make an ongoing crisis worse. People experiencing anaemia may appear short of breath when walking short distances or feel more tired than usual.

Acute chest syndrome – the sticky red blood cells can become stuck in a person’s lungs, causing fever, breathlessness, coughing and wheezing.

Sickle cell crisis

1.2.3 The frequency with which people with sickle cell disease will experience a sickle cell crisis varies. Some people have them every few weeks while others can go for years without experiencing them. On average, people with sickle cell disease experience one to three bad episodes a year (NHS, 2022a).

1.2.4 Sickle cell crisis should be treated as a medical emergency. It is necessary for staff dealing with a patient in a sickle cell crisis to respond quickly to reduce the person’s pain and reduce the risk of further complications. The National Institute for Health and Care Excellence (NICE) has commissioned and funded a clinical knowledge summary recommending that all people with the signs and symptoms of an acute painful sickle cell episode should be offered pain relief within 30 minutes of being seen in hospital (National Institute for Health and Care Excellence, 2021).

1.2.5 It is also important to co-ordinate care with the haematology team (the team specialising in blood disorders) and the patients themselves. The NICE commissioned clinical knowledge summary (National Institute for Health and Care Excellence, 2021) states:

‘Throughout an acute painful sickle cell episode, regard the patient (and/or their carer) as an expert in their condition, listen to their views and discuss with them:

• the planned treatment regimen for the episode
• treatment received during previous episodes
• any concerns they may have about the current episode
• any psychological and/or social support they may need.’

1.3.1 Sickle cell disease is a genetic condition. People with sickle cell disease are born with it and will have it throughout their life. Sickle cell disease is inherited from a person’s parents; both parents must have either sickle cell trait or sickle cell disease for their child to inherit the condition. A person has sickle cell trait when they inherit an abnormal haemoglobin gene (called haemoglobin S) from one parent, and a normal haemoglobin gene from the other (see figure 2). People who have sickle cell trait are generally healthy (National Heart, Lung and Blood Institute, 2022).

1.3.2 In the UK, approximately 1 in 79 babies carry the sickle cell trait, and around 300 babies are born with sickle cell disease each year. Approximately 15,000 people in the UK have sickle cell disease (Sickle Cell Society, n.d.).

1.3.3 Sickle cell disease can affect anyone; however, it mostly affects individuals from black African and Caribbean backgrounds. In addition, people of Central and South American, Middle Eastern, Asian, Indian, and Mediterranean descent have a higher risk of being affected.

1.4.1 Care for people with sickle cell disease is delivered by haemoglobinopathy teams, which are part of a specialist service set up in 2019 and commissioned by NHS England. The structure of the specialist service is shown in figure 3. The National Haemoglobinopathy Panel sits above the Haemoglobinopathy Coordinating Centres (HCCs), which in turn provide network support for one or more Specialist Haemoglobinopathy Teams (SHTs).

1.4.2 The HCCs and SHTs work within geographical regions as shown in figure 4a and 4b.

Sickle Cell Haemoglobinopathy Coordinating Centres and Specialist Haemoglobinopathy Teams Orange = HCCs, Teal= SHTs

Pain scoring

1.5.1 Pain scores are used by healthcare staff to help assess the level of pain patients may be experiencing and provide appropriate care. Different pain scoring systems may be used (The British Pain Society and Faculty of Pain Medicine of the Royal College of Anaesthetists, 2019) by different organisations or in different care settings.

1.5.2 In the reference event, the Trust used a numerical pain-based scoring assessment tool. This meant that pain scores were categorised as:

0 = no pain

1 to 3 = mild pain

4 to 6 = moderate pain

7 to 10 = severe pain.

Patient-controlled analgesia

1.5.3 Patient-controlled analgesia (PCA) is a method of delivering a set amount of pain-relieving (analgesic) medication, usually an opioid, from an electronically controlled infusion pump containing a reservoir of the medication. When the person presses a button on the PCA handset this activates the pump to deliver a small dose of analgesic. The analgesic is delivered either directly into the person’s blood stream (intravenously) or under the person’s skin (subcutaneously) through a thin flexible tube connected to the PCA pump. The amount of analgesic given when the patient presses the PCA button is known as a patient or ‘bolus’ demand or dose. There is also an option for continuous infusion of analgesic to be given at the same time if needed, known as a background dose.

1.5.4 Allowing a patient to administer their own pain relief means that they have more control and there is less delay in receiving the pain relief they need. The pump can be programmed with a bolus dose and background dose, and a time interval before another bolus dose is successfully delivered (known as a ‘lockout period’ or ‘lockout time’). The lockout period is a safety feature to prevent the person receiving an overdose of pain relief medication. These levels are set according to the patient’s prescription and different organisations have different guidance documents that describe the prescribing, setting up and monitoring of patients receiving PCA.

1.5.5 PCA is accepted as a safe and effective treatment for sickle cell crisis provided it is set up and functioning correctly and the patient is regularly monitored (Anaesthesia UK, 2021).

1.5.6 There are different types of PCA pumps. This report refers to PCA pumps designed specifically for the treatment of sickle cell crisis. These are referred to throughout the report as PCA pumps.

1.6.1 Morphine is a strong pain-relieving medication, one of a group of medications known as opioids. It is used to treat severe pain by blocking pain signals from the nerves to the brain (NHS, 2022b).

1.6.2 Common known side effects of morphine include constipation, feeling or being sick, feeling sleepy or tired, feeling confused and itchy skin (NHS, 2022c).

1.6.3 The higher the morphine dose, the more likely a person is to have side effects. Serious side effects include respiratory depression (breathing difficulty or short shallow breathing) and loss of consciousness (Harding, 2019; NHS, 2022c).

1.6.4 The prescribed dose of morphine can vary from person to person. The dose can depend on numerous factors including how severe the person’s pain is, the sex and weight of the patient, how they have responded to previous analgesics, and whether they are likely to have side effects. Another consideration is whether or not the patient had taken opioids in the last 30 days; if none have been taken this is known as being ‘opioid naïve’ (Pino and Wakeman, 2022).

1.7.1 The All-Party Parliamentary Group for Sickle Cell and Thalassaemia published ‘No one’s listening’, a report detailing the issues people with sickle cell disease experience in relation to their treatment (Sickle Cell Society, 2021). The report highlights that ‘awareness of sickle cell among healthcare professionals is low, with sickle cell patients regularly having to educate healthcare professionals about the basics of their condition at times of significant pain and distress’. The Sickle Cell Society’s standards for the clinical care of adults with sickle cell disease (Sickle Cell Society, 2018) state that any intervention should be planned for and managed with the haematology team.

1.7.2 The ‘No one’s listening’ report made 31 recommendations to organisations across the healthcare system. The Sickle Cell Society is monitoring the actions being taken to address these recommendations.

The reference event

COVID-19 care pathway

3.1.1 Before the COVID-19 pandemic, all patients experiencing a sickle cell crisis were admitted directly from the Trust’s emergency department (ED) to an adjacent acute medical assessment ward. The care pathway was short and familiar to staff. The Trust, which was located in a high prevalence area for sickle cell disease, was accustomed to seeing patients with sickle cell disease and patients in sickle cell crisis were seen in the medical assessment ward with the support of trained staff.

3.1.2 At the beginning of the first wave of the COVID-19 pandemic the Trust were receiving very high numbers of patients with COVID-19 without a clear understanding of how these patients should be treated or how this may impact on wider care. The circumstances and difficulty in making decisions at this point are reflected in previous HSIB reports (Healthcare Safety Investigation Branch, 2020).

3.1.3 Once patients with sickle cell disease had tested negative for COVID-19 the Trust’s approach was to keep them in areas with other patients who have tested negative, known as ‘green’ areas. As such it was not possible to treat COVID-19 positive and negative patients with sickle cell disease on the same ward. The sickle cell disease pathway was amended so that patients experiencing sickle cell crisis could be treated on two wards – ward 1, a general ward for patients of unknown COVID-19 status, and ward 2, a haematology ‘green’ ward. Neither ward was familiar with receiving and treating patients with sickle cell disease; the haematology ward ordinarily treated patients with white cell diseases. The staff from the acute medical assessment ward were not moved with the patients, which meant that the skills and knowledge of treating patients with sickle cell disease was not moved with them.

3.1.4 One member of staff stated that they were “unable to keep up” with the different wards that had been assigned for patients with sickle cell disease as it “kept changing”. They said that this meant they were unable to keep patients with sickle cell disease in the local community updated on what to expect when they were admitted to hospital.

The understanding of patients with sickle cell disease

3.1.5 The investigation identified that there were a number of different pumps available within the Trust for treating different conditions, and that the PCA pump used to treat a patient with sickle cell disease is not the same as that used in palliative (end of life) care or post-surgery by the pain management team.

Emergency department

3.1.6 The Trust policy ‘Management of sickle pain crisis in emergency department’ stated that patients arriving in the ED who were experiencing sickle cell crisis were to be given subcutaneous morphine on arrival. In Tyrone’s case a plan was made that if his pain did not diminish, he would be prescribed morphine to be administered via a PCA pump when he was moved to the acute medical assessment ward. Tyrone’s pain did not diminish, but it was not possible to set up a PCA pump as:

• no member of the ED staff was trained and competent to set up the pump or to undertake the necessary monitoring for its safe use
• there was no trained member of staff available to go to the ED for this task
• Tyrone could not be moved to the acute medical assessment ward as this had been repurposed due to the COVID-19 pandemic.

3.1.7 The investigation was informed that PCA pumps were not set up in the ED because staff did not have the capacity to meet the associated patient monitoring requirements. Patients on a PCA pump had to be monitored every 15 minutes for the first hour, then hourly for the first 6 hours and at least every 4 hours after that.

3.1.8 Before the COVID-19 pandemic, there was no requirement for staff in the ED to be trained in the set-up of PCA pumps as this was done by the staff on the acute medical assessment ward.

Ward 1 (general ward for patients with unknown COVID-19 status)

3.1.9 Ward 1 was a general medical ward where patients were admitted while their COVID-19 status was confirmed. Patients could then be sent to appropriate areas of the hospital in line with the infection prevention and control guidance in place at that time.

3.1.10 The investigation found that no staff on this ward were trained to set up a PCA pump. In interviews it was reported that, because the acute medical assessment ward had been responsible for setting up PCA pumps, other areas were unfamiliar with the treatment of patients in sickle cell crisis and the setting up of the relevant PCA pumps.

3.1.11 Staff had expected Tyrone to arrive on the ward with a PCA pump already set up. However, when he arrived with a prescription for morphine to be administered via a PCA pump, they could not complete this request.

Ward 2 (haematology ward, COVID-19 ‘green’)

3.1.12 Before the COVID-19 pandemic, ward 2 (a haematology ward) had primarily been used for treating white cell diseases. It was made up of side rooms, some with ante-rooms. The layout meant that staff had to enter the individual rooms to conduct observations for patients who needed close monitoring, such as those being given morphine via PCA pumps or those whose condition may deteriorate over time. Although it was a specialist haematology ward, the focus on patients with white cell diseases meant that staff on ward 2 did not have experience of caring for patients with sickle cell disease or those in sickle cell crisis.

3.1.13 The challenges of the COVID-19 pandemic on staff availability and sickness levels had an impact on resource levels and hence staff workload, with one member of staff stating that shifts at the time were routinely one to two members of staff down. At the time of the reference event, the ward was two staff members short (a nurse and a healthcare assistant) which meant the staff present had more demands on their time.

3.1.14 Members of the nursing team told the investigation that they tried to manage their workload across the team to meet the care needs of their patients. The ward had been reportedly busy overnight with two new admissions and two “very ill” patients with white cell diseases. One member of staff noted that patients with leukaemia or lymphoma can “go downhill very fast” and therefore can need a great deal of attention that cannot be predicted, which can take focus from other tasks.

3.1.15 The staff on ward 2 were trained in delivering chemotherapy, so could be called away to other wards to support this when the chemotherapy suite was closed. This meant they could be absent from the ward for periods of approximately 1 hour at a time. A call of this nature meant that the staffing level was further reduced during the night, such as when Tyrone was on the ward.

3.1.16 The nurse in charge of Tyrone’s care on ward 2 understood that he needed a PCA pump as a matter of urgency to control his pain. The nurse told the investigation that they set up the PCA pump and that they had received training on how to do this. The training was delivered verbally, quickly and at short notice, as the ward was already receiving patients with sickle cell disease due to the rapid nature in which services were being reorganised during the COVID-19 pandemic.

3.1.17 PCA checks were recorded on the relevant monitoring form. The total number of demands for 5 mg of morphine since the was PCA started was 27, of which 11 were successful (see figure 5).

3.1.18 Other staff informed the investigation of variability in the delivery of training, including:

having no training at all in the use of PCA pumps

being given the PCA pump instruction manual to read

being trained solely on the mechanics of the pump, with no training on the monitoring requirements, so they “just did the observations like we do for the patients with white cell conditions”.

3.1.19 The workload associated with monitoring patients on PCA pumps was also raised in staff interviews. Staff reported that carrying out the required regular PCA pump monitoring while also attending to other patients could be challenging.

3.1.20 A senior member of staff at the Trust informed the investigation that during the pandemic, training for staff on the set-up and monitoring of PCA pumps was not formally recorded. Therefore, the investigation was not able to establish what the content of the training was, who had received it, or the level of competency to which staff were trained.

3.1.21 The investigation was told that since the reference event the Trust now delivers and records training on PCA pumps and sickle cell care, including competency checks.

3.1.22 Setting up the PCA pump is only one element of the training required to be able to care for a patient in sickle cell crisis. Regular monitoring of the patient is required while they are on a PCA pump due to the risks of respiratory depression (insufficient breathing) or overdose.

3.1.23 Approximately half of the staff on the haematology ward interviewed by the investigation had knowledge and/or experience of patients with sickle cell disease. However, there had been no formally recorded training provided to the nursing staff on sickle cell disease, including how the pain of sickle cell crisis can affect the behaviour of patients.

3.1.24 Reflective of this, a number of staff told the investigation that patients in sickle cell crisis can be “very difficult to work with”, and that they can be “difficult to treat and horrendously behaved”. One nurse with experience in treating patients in sickle cell crisis stated that although patients can be aggressive and angry during the crisis, they regularly come and apologise after the event. The nurse said that as someone trained in this, they know how this behaviour manifests and can help to mitigate the impact a patient’s behaviour may have on the perceptions of others responsible for delivering care. A senior consultant informed the investigation that patients in sickle cell crisis “are not themselves” because of the amount of pain they are in.

3.1.25 The investigation also noted that in the reference event a large amount of documentation was completed retrospectively and this was brought up in discussions with the staff. Nursing and Midwifery Council guidance states that staff should complete all records at the time or as soon as possible after an event. If records are written some time after the event, this should be noted (Nursing and Midwifery Council, 2018).

3.1.26 Staff told the investigation that they understood the importance of completing records at the time, but that it was not always possible due to the multiple pressures and competing demands on the ward. The documentation used at the Trust was paper based, although an electronic patient record system was due to be adopted in the near future. This meant that staff may not be able to record their notes immediately because the documentation was being used by colleagues.

3.1.27 Staff reported that they often had to stay back at the end of a shift to complete documentation they could not finish during their shift. On reviewing Tyrone’s notes, the investigation found that there were multiple sheets to complete. Some information was duplicated, for example, pain score was requested on both the nursing care plan record chart and the PCA chart. This increased the workload for staff and reduced the time they could spend on direct patient care.

3.1.28 One nurse reported feeling “saturated” with the workload required to look after patients with white cell conditions, even before the addition of patients with red cell conditions who “have completely different care needs”.

National investigation

Landscape

3.1.29 Recommendations from the National Confidential Enquiry into Patient Outcome and Death (NCEPOD) report ‘Sickle: a sickle cell crisis?’ state:

‘As in previous studies, NCEPOD has highlighted examples where patients who were acutely ill were not offered support from sufficiently experienced medical staff. NCEPOD firmly believe that there is a need to monitor patients on opioids more carefully. Nurses and doctors both need to be more familiar with what needs to be done if patients’ vital signs, including the respiratory rate, become abnormal. These simple measures might well have avoided some of these deaths.’
(National Confidential Enquiry into Patient Outcome and Death, 2008)

3.1.30 National Institute for Health and Care Excellence (NICE) clinical guidelines on the treatment of patients in sickle cell crisis in acute settings state that:

‘All healthcare professionals who care for patients with an acute painful sickle cell episode should receive regular training, with topics including:

• pain monitoring and relief
• the ability to identify potential acute complications
• attitudes towards and preconceptions about patients presenting with an acute painful sickle cell episode.’
  (National Institute for Health and Care Excellence, 2012)

3.1.31 The NICE guideline also states that patients who are ‘taking strong opiates’ such as via a PCA pump should be monitored ‘every hour for the first 6 hours’ and ‘at least every 4 hours thereafter’ (National Institute for Health and Care Excellence, 2012).

3.1.32 The All-Party Parliamentary Group for Sickle Cell Disease and Thalassemia ‘No one’s listening’ report (Sickle Cell Society, 2021) identified ‘low levels of awareness of sickle cell among healthcare workers on general wards and in A&E’. First contact with patients experiencing sickle cell crisis will often be in such environments, particularly when there may have been a need to reconfigure or reorganise services in times of NHS crisis.

.1.33 This report also found that patients with sickle cell disease could be seen as ‘drug-addicts’ and ‘aggressive’ by staff not familiar with sickle cell crisis. This was reiterated by consultants spoken to as part of the investigation and can impact on the care and treatment these patients may receive.

Training and oversight

3.1.34 In 2022 NHS England launched the ‘Can you tell it’s sickle cell?’ campaign to raise awareness of the signs of a sickle cell crisis and highlight issues and signposts people to information about sickle cell disease.

3.1.35 The investigation visited and spoke to several Haemoglobinopathy Coordinating Centres (HCCs) and identified variability in the way individual HCCs were attempting to deliver training. One HCC informed the investigation that training delivered by a specialist nurse was not attended by consultants, as it was not consultant led. Because of this they were reviewing the training delivery.

3.1.36 An HCC told the investigation that engagement of trusts for training was a “problem” as they could not mandate that staff attend. The HCC wanted to make the training available online to improve accessibility and uptake but stated that they did not have sufficient local funding or IT resources to achieve this. The training is also delivered by a limited number of staff who also deliver clinical care.

3.1.37 They explained that regional NHS England Specialised Commissioning provides oversight of the network and that NHS England creates the service specification.

3.1.38 The service specification states that HCCs will deliver training, but there is no national curriculum or attendance mandate. This leaves HCCs to devise their own training curriculum and delivery plan while also trying to encourage attendance. This has led to variability in the content, delivery and attendance of training.

3.1.39 The journal article ‘Acute and chronic pain management in sickle cell disease: outcomes of an English national audit’ highlighted that:

‘The infrequent delivery of teaching reveals the need for regular local and national mandatory educational training of all ED staff providing care to SCD [sickle cell disease] patients during acute presentations.’
(Lugthart et al, 2022).

3.1.40 The ‘No one’s listening’ report made the following recommendations in relation to training:

• ‘All universities to include comprehensive training in sickle cell as part of curriculums for trainee healthcare professionals, covering diagnosis, presentations, management, acute complications (such as pain, acute chest syndrome, stroke) and ongoing care and featuring direct contributions from sickle cell patients.
• The Nursing and Midwifery Council [NMC] and the General Medical Council [GMC] to urgently commission a review of their approach to sickle cell training, in collaboration with the sickle cell community.
• The NMC and GMC to strengthen requirements around the level of sickle cell training required for university curriculums to be approved.
• Health Education England to provide additional funding for sickle cell training programmes for healthcare professionals, including for training in the delivery of blood transfusions for non-specialist doctors.’
  (Sickle Cell Society, 2021)

3.1.41 The Sickle Cell Society informed the investigation that, in its opinion, there had been limited progress or uptake of these recommendations.

4.1.1 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.

4.1.2 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.

4.1.3 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.

4.1.4 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.

4.1.5 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.

Decision to investigate

HSIB’s Chief Investigator authorised a national investigation based on the following criteria.

Outcome impact – what was, or is, the impact of the safety issue on people and services across the healthcare system?

Patients with sickle cell disease who are cared for by staff who are not equipped with knowledge of the condition and the skills to treat it may not receive optimal care.

Systemic risk – how widespread and how common a safety issue is this across the healthcare system?

Knowledge and awareness of sickle cell disease varies nationally depending on the prevalence of the disease within the region. This causes clinicians to take different approaches to the care and treatment of patients with sickle cell disease and means that healthcare staff have varying levels of knowledge about the condition.

Learning potential – what is the potential for an HSIB investigation to lead to positive changes and improvements to patient safety across the healthcare system?

An HSIB investigation could help identify issues across the healthcare system related to effective multidisciplinary communication and planning for this group of patients.

Evidence gathering and methods of analysis

Evidence gathering

Evidence gathered during the investigation included:

• a review of Tyrone’s clinical records, Trust policies, procedures, and observations of practice
• a review of the Record of Inquest into Tyrone’s death an interview with Tyrone’s family
• interviews with staff at the Trust where the reference event took place
• a review of the literature relevant to the safety risk
• interviews, telephone calls and email correspondence with representatives from:

- Sickle Cell Society

- British Society for Haematology

- Royal College of Radiologists

- Department of Health and Social Care

- NHS England (Health Inequalities Steering group, Haemoglobinopathies Clinical Reference Group, Specialised Commissioning)

- multiple trusts from high and low prevalence areas (including Haemoglobinopathy Coordinating Centres and Specialist Haemoglobinopathy Teams)

- National Institute for Health and Care Excellence

- NHS Race and Health Observatory.

Methods of analysis

The AcciMap model was used to analyse the reference event information and support the direction of the national investigation. AcciMap (Svedung and Rasmussen, 2002) is an incident analysis method that identifies factors within a system (in this case the healthcare system as a whole) that influence the occurrence of an incident. The analysis focuses on identifying relationships between the different levels of the system (see figure A), which include government policy and budgeting; regulatory bodies and associations; local area management; physical processes and actor activities (linked with staff, people, organisations and systems); equipment and surroundings.

AcciMap is useful for visually representing contributory factors across the entire organisational system and their interrelationships. It removes the apportioning of blame to individuals and promotes the development of systematic countermeasures as opposed to countermeasures which focus on an individual (Salmon et al, 2011).