Sickle cell disorder - what you need to know

It’s estimated about 15,000 people in the UK live with sickle cell disorder and approximately 300 babies are born with sickle cell each year. Commonly inherited, it predominantly affects people from African and Caribbean backgrounds.

In November 2021, the Sickle Cell Society published the No one’s listening report following an All-Party Parliamentary Group (APPG) inquiry into avoidable deaths and failures in care of sickle cell patients. The key findings included:

• evidence of sub-standard care for sickle cell patients who were admitted to general wards or seen in the emergency department (ED)
• lack of adherence to national care standards
• low awareness of sickle cell among healthcare professionals, partly because of inadequate training.

The main symptoms of sickle cell disorder (SCD) are anaemia and episodes of severe pain, referred to as a sickle cell crisis. Those with SCD are at increased risk of stroke, acute chest syndrome, blindness, bone damage and priapism as well as damage to organs such as the liver, kidneys, lungs, heart and spleen over time.

Importantly, stroke can occur in children, with the risk being highest between the ages of two and 16 years.

MDU cases

Twenty-eight cases were reported to the MDU over a five-year period where the main issue related to the patient’s diagnosis of SCD. Although this number is relatively small, over half of the files (57%) involved the death of the patient with a subsequent coroner’s inquest.

16% of cases related to the member requesting help with an adverse incident report where concerns had been raised about the patient’s management. These were internal hospital trust investigations designed to identify systemic failures and learning points to prevent further similar incidents in the future. These types of internal investigations are an important tool in identifying areas where knowledge and skills of medical staff can be improved and address some of the concerns raised by patients and healthcare professionals, who feel training is lacking in relation to SCD.

In a further 16% of cases, a GMC investigation had been opened into the doctor’s conduct. Three of these cases had originated following the doctor referring themselves to the GMC after receiving criticism at an inquest, in accordance with the GMC’s guidance. Other investigations were opened after concerns were raised by the patient themselves or, in the case of deceased patients, their family.

8% of cases related to NHS clinical negligence claims and 3% related to where a complaint had been made only, with no other medico-legal consequences.

As patients with SCD can present with non-specific symptoms, or symptoms that could be suggestive of another diagnosis, they commonly present to their GP. Of the MDU cases, 39% related to care provided by a GP. There were equal numbers of cases involving general medicine, anaesthetics and haematology.

Anaesthetists and haematologists tended to become involved in the patient’s care later on during their admission, after initial management had been started. 10% of cases involved paediatricians and a small number of gastroenterologists and psychiatrists.

Case example

A 14-year-old girl was brought into the emergency department complaining of headache, diarrhoea, vomiting and abdominal pain. She had been in contact with a child at school who had viral gastroenteritis and it was therefore assumed that this was the cause of her symptoms.

The patient was clearly unwell with a temperature and was dehydrated. Her mother was concerned that she’d not been able to keep fluids down. She emphasised the fact that her daughter had sickle cell disorder and gave the treating doctor the name of the consultant at the haemoglobinopathy service she was under the care of.

Other than dehydration, the doctor didn’t find anything concerning during their examination, although they didn’t monitor oxygen saturations as there were no respiratory symptoms. Paracetamol was given as pain relief and the child was encouraged to increase their oral intake with a view to starting intravenous fluids if she continued to vomit and her urine output was low. The doctor advised that this was a viral illness that would resolve itself and once she was able to keep fluids down, she could go home.

The patient continued to complain of abdominal pain and headache, which was getting worse, but was starting to keep small amounts of oral fluids down. She was reassured that her headache was a result of her viral illness and dehydration and would improve with increased oral fluid intake.

The doctor went off duty and handed the patient over to the doctor on nights explaining that the patient could go home if she continued to drink. When the doctor returned the next day, they found that the child had complained of a severe headache shortly after they’d finished their shift and had had a cardiac arrest. She had been admitted to ITU but had unfortunately died some hours later. The ITU consultant had contacted the child’s consultant who advised that it was likely she was having a sickle cell crisis and had suffered a stroke. The consultant was concerned that IV fluids and oxygen therapy, in addition to adequate analgesia, hadn’t been started and that he hadn’t be contacted for advice.

The doctor in the emergency department contacted the MDU for support in writing an adverse incident report and a report for the coroner. As they were a specialist trainee, they were encouraged to discuss the case with their educational supervisor and engage with some relevant professional development to improve their knowledge of sickle cell disorder and how a crisis can present, in addition to familiarising themselves with relevant guidelines.

As part of the significant event analysis, the trust acknowledged that the care provided to patients with sickle cell disorder was something that needed further attention and updated its policies in addition to asking the patient’s consultant to do some focused teaching sessions for all clinical staff. The coroner accepted the actions that had been taken both by the doctor and the trust and did not directly criticise the doctor for their actions, avoiding the need for them to refer themselves to the GMC.

Managing patients with SCD and avoiding medico-legal consequences

To help reduce the risk of medico-legal consequences involving patients with SCD, based on the MDU cases that have been analysed, we advise the following.

• Seek advice from a specialist early when a patient with SCD presents unwell with symptoms, even if these appear to be due to a common cause.
• Familiarise yourself with local and national guidelines and policies for the treatment of patients with SCD, such as the NICE guidance on sickle cell disease and the NICE guidance on managing acute painful episodes.
• Actively consider whether you need further training on the diagnosis and complications of SCD and how a crisis can present.
• Make sure any patient consultation or interaction is clearly documented, including the history taken, the examination performed, the differential diagnosis and management plan and details of any advice that has been sought.
• Consider SCD in a patient from relevant backgrounds and arrange the appropriate investigations to confirm or exclude a diagnosis.
• Bear in mind that following general guidelines, for example, for the treatment of infections, including post-operative infection, may not be appropriate alone for patients with SCD. More specific management may be required. Seek advice.
• Ensure good communication between multi-disciplinary teams and good documentation so that all parties caring for the patient have up-to-date information.
• Ensure that the diagnosis of SCD is communicated appropriately when requesting investigations, including blood tests.
• Have a lower threshold to treat infections with IV antibiotics. Check your policy for treating infection in patients with SCD.
• Consider checking oxygen saturations, when you wouldn’t normally do so, in a patient with SCD who is unwell and give oxygen therapy if appropriate.
• Be familiar with the signs and symptoms of an acute crisis and remember children can present with or develop a stroke.
• Check the patient’s immunisation status.
• If there is shared care, check who has taken responsibility for prescribing analgesia to prevent duplication.
• Ensure there is a robust system, such as a significant event audit, for analysing patient safety incidents. This can highlight lessons that can be learned and what changes should be put in place to prevent a similar situation in the future.
• Provide patients with an explanation and apology if something does go wrong, particularly if the outcome is poor or unexpected. Take steps to deal with the consequences and arrange appropriate treatment and follow-up.

Members can contact the MDU at the earliest opportunity with any concerns. 

This page was correct at publication on 28/07/2022. Any guidance is intended as general guidance for members only. If you are a member and need specific advice relating to your own circumstances, please contact one of our advisers.