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Written on 22nd June 2023 by Susan Brown

Healthcare safety watchdog, HSIB, has highlighted the safety risk to patients with sickle cell disease from ‘gaps’ in their NHS care. HSIB published its findings in two national investigation reports, Management of sickle cell crisis and Invasive procedures for people with sickle cell disease, after World Sickle Cell Awareness Day on 19th June 2023. The reports follow national investigations which explored failings in the care provided to sickle cell patients who were in hospital for invasive procedures or for emergency treatment of sickle cell crisis.  

HSIB’s reports call for a review of the training and competency requirements for healthcare professionals and staff in relation to sickle cell disease, and of all aspects of care for people with the condition. In addition, HSIB calls for research to support the development of evidence-based guidelines on the care of patients with sickle cell disease, flagging the current disproportionate lack of research as a potential health inequality. 

What is sickle cell disease?

Sickle cell disease is a genetic blood disorder that affects a person’s haemoglobin (a protein in their red blood cells that carries oxygen through the body).  Normal red blood cells are disc shaped and flexible and can move easily through the blood vessels. People with sickle cell disease have inflexible, sickle-shaped, red blood cells which block the flow of blood and oxygen to the rest of the body.

Sickle cell disease is inherited from a person’s parents when both parents have sickle cell disease or sickle cell trait. People with sickle cell trait are usually healthy but have inherited an abnormal haemoglobin gene from one parent and a normal haemoglobin gene from the other. Sickle cell disease is most common in people of Black African and Caribbean heritage, and also those with family heritage from Central or South America, the Middle East, Asia, India or the Mediterranean. 

Around 15,000 people in the UK have sickle cell disease.

People with sickle cell disease are more likely to undergo surgery during their lifetime than the general population. Surgery also exposes patients to many of the factors that cause their red blood cells to ‘sickle’, so extra care is needed to identify sickle cell disease patients before surgery and prevent complications during or after surgery. 25% to 30% of patients with sickle cell disease experience post-operative complications.

People with sickle cell disease experience episodes of extreme pain or ‘sickle cell crisis’, which are triggered by factors such as stress, infection, inflammation, dehydration, and low body temperature. Sickle cell crisis is a medical emergency which needs urgent hospital treatment with strong painkillers, such as morphine, to reduce the extreme pain and risk of other complications. Hospital treatment, such as surgery, must be coordinated with the haematology (blood disorder) specialists and the patient. The patient’s body temperature (and other conditions) must be maintained during and after any medical procedures, to avoid triggering a sickle cell crisis.

In 2021, the All-Parliamentary Group for Sickle Cell and Thalassaemia published its report, No one’s listening. The report highlighted the issues that people with sickle cell disease experience with healthcare and made 31 recommendations to healthcare organisations to help address these issues. A key finding was 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’.

HSIB’s report: Management of sickle cell crisis

HSIB’s national investigation into the management of sickle cell crisis was triggered by an investigation into the death in hospital of a 46-year-old man.  The man was taken to hospital by ambulance during the covid-19 pandemic in early 2021 suffering extreme pain from sickle cell crisis. After being admitted via the emergency department and then undergoing covid tests in other parts of the hospital he was eventually transferred to a haematology ward for treatment. He was given morphine continuously via a patient-controlled analgesia (PCA) pump and could also press a button on the pump to administer an additional dose of morphine.  He was monitored overnight but was then found to be unresponsive and died after resuscitation attempts failed. A post-mortem report gave the cause of death as cardiorespiratory failure caused by acute sickle cell crisis and morphine toxicity. The coroner found that the nursing staff had insufficient training to manage and monitor the patient and his PCA pump.

HSIB’s investigation of this man’s treatment found that the ward staff were not familiar with treating patients with sickle cell disease and that the nurses’ training had been delivered ‘verbally, quickly and at short notice.’

HSIB’s national investigation found that there is no minimum training requirement or nationally agreed content for Haemoglobinopathy Coordinating Centres to deliver to provide healthcare staff with knowledge about sickle cell disease or sickle cell crisis. Haemoglobinopathy Coordinating Centres were set up to provide expert opinion on the care of patients with sickle cell disease, to improve their experience of care and reduce their morbidity (ill health or disease) but have struggled to engage NHS trusts and NHS staff in this process in areas with a low sickle cell disease population.

HSIB also found that competing priorities during the covid-19 pandemic had led many trusts to change how and where patients with sickle cell disease were cared for, resulting in a lack of necessary equipment on the wards or knowledgeable, trained staff to provide them with safe care. Staff workload during the pandemic and in emergency departments also reduced their ability to provide increased monitoring, such as for patients receiving high strength opiates (such as morphine) via PCA pumps.

HSIB recommended that NHS England reviews the existing training and competence requirements for sickle cell care and specifies the minimum training requirements and content for staff. Haemoglobinopathy Coordinating Centres can then use this content to increase knowledge about sickle cell disease and how to treat patients in sickle cell crisis.

HSIB’s report: Invasive procedures for people with sickle cell disease

HSIB launched its investigation into invasive procedures for people with sickle cell disease after investigating the care of a woman who attended hospital as a day case for a gynaecological procedure. She had received multiple exchange blood transfusions for her sickle cell disease at the hospital under ongoing care of the haematology (blood disorder) team. The haematologists knew she was having a surgical procedure but were not told when it would happen. Without their prior knowledge, her surgery took place a week before her next scheduled blood transfusion when her haemoglobin levels were low. The painful surgery triggered a sickle cell crisis needing inpatient hospital treatment for six days.

HSIB noted that the ‘No-one’s listening’ report had identified that patients with sickle cell disease found hospital staff were unwilling to contact specialists to seek advice, resulting in severe prolonged pain and trauma. HSIB were also told by haematology clinicians that they were often told too late, if at all, about their patients undergoing non-haematology procedures. They often had to rely on patients to let them know directly about any planned procedures.  

HSIB’s investigation found that the treatment that people with sickle cell disease receive varies depending on where they live and the number of patients that their healthcare clinicians see. There is currently no national information system for storing and sharing clinical information for patients with sickle cell disease. National guidance on sickle cell disease is limited, but where it exists, it relates to specific circumstances, such as sickle cell crisis or blood transfusion, rather than lifelong management of patients with the disease. 

They flagged a potential health inequality arising from the lack of research for sickle cell disease compared with other conditions which affect similar numbers of people. For example, there were 69 research studies into cystic fibrosis, which affects 10,800 people, but only 23 for sickle cell disease which affects 15,000 people.

HSIB recommended that NHS England develops a system for sharing clinical information about patients with sickle cell disease to help clinicians access this information and reduce the risk of a patient’s sickle cell disease not being treated consistently. The watchdog also recommended that the National Institute for Health and Care Research commissions more research into sickle cell disease (including the use of PCA pumps for pain relief) so that evidence-based clinical guidelines can be written and the care of sickle cell disease improved across the country.

HSIB’s safety observations included a suggestion that NHS trusts include a sickle cell disease notification within their electronic patient record systems, to prompt clinicians to consider their patient’s sickle cell disease and seek advice from their haematology teams about the impact of any planned treatments and procedures.  They also suggested that the healthcare sector explores, researches and fully understands the care they provide to all patients with sickle cell disease, at all stages in their life, from everyday treatments through to sickle cell crisis.

Meanwhile, HSIB acknowledged current initiatives by NHS England’s ‘Can you tell it’s sickle cell?’ awareness and signposting campaign, the Sickle Cell Pathway Review Steering Group’s review of the sickle cell disease care pathway and NHS England’s recent announcement of new specialist clinics to provide sickle cell care to patients.

If you have suffered severe injury as a result of medical negligence or have been contacted by HSIB/HSSIB/CQC or NHS Resolution, you can talk to a solicitor, free and confidentially, for advice about how to respond or make a claim by contacting us.