Patients with sickle cell disease may experience potentially dangerous gaps in care across the NHS as understanding of the condition remains limited, a health care safety watchdog has warned.
The genetic lifelong blood disorder, mostly affecting people of Black African and Caribbean background, is characterised by episodes of extreme pain known as sickle cell crisis, and affects around 15,000 people in the UK
The Healthcare Safety Investigation Branch (HSIB) launched two investigations on the care provided to patients with sickle cell disease, with the reports published this week, following World Sickle Cell Awareness Day on 19 June .
In 2021, an All-Parliamentary Group published a report detailing the issues patients with sickle cell disease experience and it highlighted 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 HSIB's two reports had similar conclusions, finding that knowledge and awareness of sickle cell disease varies across the NHS, with patients at risk of poor outcomes due to this gap. It has requested the NHS to improve and standardise training and treatment guidelines for staff, and increase research on the disease.
Key findings from the HSIB Management of Sickle Cell Crisis report
- There is no minimum training requirement or nationally agreed content to provide NHS staff with knowledge about sickle cell disease or sickle cell crisis.
- In the reference event, a 46-year old man with sickle cell disease, Tyrone, was taken to hospital after experiencing a sickle cell crisis. The nursing staff on his ward had insufficient training to manage and monitor Tyrone and he sadly died.
- HSIB has made a safety recommendation to NHS England to review minimum training requirements and content for NHS staff.
- Treatment of people with sickle cell disease varies depending on where they live and the number of patients that clinicians see.
- In the reference event, a 45-year old woman was in hospital for six days (rather than as a surgical day case) because of a lack of communication between her haematology team and her surgical team.
- There has been limited research into lifelong management of sickle cell disease, which could indicate a health inequality.
- The HSIB made two safety recommendations, to NHS England and the National Institute for Health and Care Research, to address these issues.
Common Strands in the Reference Cases
Commenting on the reports' findings, Dave Fassam, national investigator at HSIB, said: "Through our work in this area we have concluded that the knowledge and awareness of sickle cell disease varies across the NHS. This is usually down to the prevalence of the disease in their area and how experienced they are at dealing with this condition. The knock-on effect of this is that at any one-time different approaches can be taken for one patient."
Mr Fassam said that both investigations ran in tandem with each other, and there were "common strands" in both reference cases, that were also observed when they spoke to other organisations.
The HSIB acknowledged in the reports that there are now a number of national initiatives to improve expert care for this group of patients. "However, it is important that the findings and recommendations are fed into this work because we have provided powerful examples of what can happen when there is a gap in care for patients – they can suffer pain, distress and in the worst situation, it can be a contributing factor to serious harm or death," Mr Fassam added.
An overall message from the HSIB reports is that more research and information is needed to improve training and equip all clinicians with a good level of understanding of the treatment and care needed for patients with sickle cell disease, he reiterated.
"Optimising care and ensuring it is consistent wherever patients live in England will reduce the risk of extreme pain and complications for patients and provides a safer environment for all."