HSIB: Invasive procedures for patients with sickle cell disease (22 June 2023)

Summary

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. It mainly affects people from African or Caribbean backgrounds, though it can affect anyone. It affects approximately 15,000 people in the UK.

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.

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.

Content

The reference event was a patient, 45 years old at the time of the incident, who 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.

Due to heavy, painful and frequent menstrual periods she was scheduled for a surgical procedure to help 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.

Findings

• 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.

Recommendations

HSIB recommends that NHS England identifies and implements a system for sharing clinical information about patients with sickle cell disease to improve access to this information for clinicians, and reduce the risk of a patient’s sickle cell disease not being treated consistently.

HSIB recommends that the National Institute for Health and Care Research assesses the priority and feasibility of commissioning research into the management of patients with sickle cell disease, encompassing the requirements of patient-controlled-analgesia (pain relief) pumps. This will contribute towards building an evidence base for the generation of national clinical guidance and will reduce unnecessary variance in treatment for patients with sickle cell disease.

Observations

HSIB makes the following safety observations:

• It may be beneficial if trusts included a sickle cell disease notification within their electronic patient record systems, to ensure that clinicians treating patients with the disease consider it and seek advice from haematology teams about the impact of any planned treatments, such as invasive procedures.
• It may be beneficial if the healthcare sector explores, researches and fully understands the care provision to all patients with sickle cell disease, at all points in their life, encompassing day-to-day treatment through to sickle cell crisis.