It is currently estimated that there are approximately 15,000 people living in the UK with Sickle Cell Disease.

The All-Party Parliamentary Group on Sickle Cell and Thalassaemia recently prepared a Report which has highlighted serious failings in care for patients suffering from Sickle Cell Disease, which has included avoidable deaths.

What is Sickle Cell Disease?

Sickle Cell Disease is the name used for a group of medical conditions that affect the red blood cells. These conditions are inherited and the most serious type is Sickle Cell Anaemia.

The condition causes the body to produce unusual shaped red blood cells, which do not live as long as healthy red blood cells, so can cause problems such as blocking blood vessels.

Patients will often receive treatment to help to manage the symptoms. The main symptoms include: –

  • Severely painful episodes called Sickle Cell Crises, where the sickled red blood cells block the small blood vessels carrying blood to the bones
  • An increased risk of serious infections
  • Anaemia, which is where the body struggles to carry oxygen around the body to the different organs, and causes tiredness and shortness of breath

What is the treatment for Sickle Cell Disease?

Treatment will usually be provided by a specialist Sickle Cell Centre. However, patients will often be encouraged to use self-care measures to help look after their own health. This can include drinking plenty of fluids to keep hydrated, painkillers and daily antibiotics to help reduce the chance of infection.

Some patients will also receive a medicine called Hydroxycarbamide to reduce symptoms associated with Sickle Cell Disease. Hydroxycarbamide helps to manage the episodes of severe pain.

Regular blood transfusions can be used if symptoms worsen or if there are signs of organ damage. A transfusion would also be required for patients that develop severe anaemia.

The only cure for Sickle Cell Disease is a stem cell or bone marrow transplants. However, these are not performed often due to the significant risks of the procedures. Here, stem cells from a healthy donor will be given through a drip into a vein. These cells then produce healthy red blood cells to replace the sickle cells.

Why was the Report commissioned?

The Report was carried out following the sad death of Evan Smith, aged 21, who died in 2019 following a Sickle Cell Crisis.

His case was referred for an Inquest and the Coroner determined that there had been a number of failures in his care, including the failure to offer a blood transfusion at an earlier opportunity. The Inquest found that his death would not have happened but for the failures in the care that he received.

The Coroner highlighted that Evan’s case was sadly not alone and that incidents of Sickle Cell patients receiving sub-standard care are occurring too often.

What did the Report find?

The Report highlighted that there was sub-standard care not only on General Wards following a patient being admitted to Hospital, but also how Sickle Cell Disease was managed in A&E Departments.

One of the most common failures in A&E departments, was a delay to administer pain relief early enough, due to a lack of understanding of Sickle Cell Crises. The Report also found instances of poor communication between wards, including General Wards and the Haematology Departments.

It has been suggested that promoting communication with haematology specialists, may lead to a better standard of care, and help to reduce the amount of deaths as a result of the condition.

Inadequate training and a lack of understanding and education of the condition is thought to be one of the causes of the poor care. As well as evidence of poor care in Hospitals, the Report also found that there was a low awareness of the disease, and insufficient investment into researching treatment options for patients.

The Report found that on discussion with patients living with Sickle Cell Disease, there was a lack of trust in the health system, due to the amount of patients that had suffered from poor experiences in the healthcare setting themselves, or were aware of tragic and avoidable deaths.

The Report has called for major improvements in the care provided to patients with Sickle Cell Disease and has made a number of recommendations including:

  • Increased funding for Sickle Cell Research
  • Better training for healthcare staff

Positive steps

It is worth noting that last month, NHS England announced the first treatment for Sickle Cell Disease for more than 20 years. Furthermore, 10 new Sickle Cell Specialist Centres are being set up across the country, to provide better access to treatment for patients affected.

The National Institute for Health and Care Excellence has approved the trial of an antibody treatment called Crizanlizumab. This medication was trialled over a year and found to almost halve the amount of Sickle Cell Crises that a patient will experience. Crizanlizumab works by preventing the sickled red blood cells from sticking together and creating a blockage in the blood vessels, which leads to a significant reduction in pain.

Crizanlizumab will now be a treatment option for patients over the age of 16 and is an important step in helping patients with Sickle Cell Disease to manage their symptoms and lead to a better quality of life.

However, while specialist services are key to the improvements in care, there also needs to be improved training across all healthcare providers, to ensure that if patients are admitted with Sickle Cell Disease, their condition is understood and managed appropriately.

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