Invasive procedures in patients with sickle cell disease

This investigation was carried out by the Healthcare Safety Investigation Branch (HSIB). Find out more about HSIB legacy.

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.

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.

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.

• 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.