Sickle Cell Disease (SCD) is the most common genetic disorder worldwide, affecting millions of people, including approximately 17,000 individuals in England. As of 2023, SCD was the most common and fastest-growing genetic disorder in the UK. Despite its prevalence, SCD remains significantly underfunded, especially when compared to other genetic disorders. For example, during 2021 to 2022, expenditure on National Institute for Health and Care Research (NIHR) programmes was £2,440,800 for Cystic Fibrosis, compared with only £943,602 for SCD.
Overall, SCD is underserved, and poorly understood within the healthcare system, including amongst pharmacists, pharmacy students, and foundation pharmacists in the UK. This article aims to highlight the urgent need for increased awareness, funding, and improved healthcare access for SCD amongst these healthcare professionals.
Epidemiology and Prevention
In the UK, SCD is most commonly seen in people of African or African-Caribbean backgrounds. However, the disease can affect anyone who inherits the abnormal haemoglobin genes that cause Sickle Cell Trait (SCT) or SCD. Approximately 1 in 79 babies born in the UK carry the Sickle Cell Trait, and almost 300 babies are diagnosed with SCD each year through the NHS Newborn Screening Programme.
Early detection through newborn screening is crucial for preventing complications and improving outcomes, though parents can individually opt out of screening for SCD. Due to various factors including a lack of routine screening amongst immigrant and ethnic minority group populations, variability in disease presentation across patients, limited awareness, and barriers to accessing healthcare services, many individuals remain undiagnosed, particularly among ethnic minority groups.
Current Treatment Options
Currently, licensed treatments for SCD primarily focus on pain management and addressing complications as they arise. The UK recently approved the world’s first gene therapy for SCD. However, access to this groundbreaking treatment remains limited due to its high cost. The inequitable distribution of novel therapies highlights systemic barriers within the healthcare system that need to be addressed.
Improving access to SCD care requires a better distribution of specialist units across the UK. There is a limited number of dedicated SCD units in many regions, which can lead to delays in diagnosis and fragmented care. Greater collaboration between healthcare providers, including pharmacists, is needed to ensure that patients receive treatment in a timely and appropriate manner.
Barriers to Care
The ‘No One’s Listening’ report, produced in 2021 by the All Party Parliamentary Group (APPG) on Sickle Cell and Thalassaemia and the Sickle Cell Society, highlighted various barriers to care. These included low awareness of sickle cell among healthcare professionals, inadequate training, and insufficient investment in sickle cell care. Additionally, there were frequent reports of negative attitudes towards sickle cell patients, often underpinned by racism. Care failings have led to unnecessary patient suffering, ‘near misses’, and patient deaths, including at least one which prompted a Coroner’s Regulation 28 Prevention of Future Deaths report.
Case study
Evan Nathan Smith was a 21-year-old student. He experienced various treatment delays and oversights which led to his death:
- He was admitted under the care of Gastroenterology, who had no experience in treating patients with SCD
- Staff failed to recognise that he was experiencing a sickle cell crisis
- The Haematology team refused to take over his care
- He was put in a bed in a corridor with no call button
- This meant that he was so desperate that he dialled 999 for an ambulance asking for oxygen
- The call was refused as he was already in hospital
- Doctors failed to escalate findings that confirmed low oxygen saturation levels
- Opportunities were missed to provide Evan with a blood transfusion that the coroner’s inquest found would have saved his life.
Education and Awareness
A lack of education about SCD within the wider healthcare community, including pharmacy, greatly contributes to disparities in care. Though the importance of SCD education for pharmacists and allied healthcare professionals is acknowledged, it remains unclear whether this topic is adequately addressed within the MPharm curriculum. Without explicit guidance from the General Pharmaceutical Council (GPhC))/Pharmaceutical Society of Northern Ireland (PSNI) and more comprehensive education surrounding SCD being embedded within the pharmacy curriculum, pharmacists and future pharmacists may remain ill-prepared to manage patients with this complex condition.
Improving Care and Equality
To address the disparities in SCD care, a multi-faceted approach is needed. Pharmacists have a crucial role to play in advocating for increased funding for SCD research and the development of more affordable, accessible, and inclusive treatment plans. Raising awareness about SCD within the pharmacy community and promoting health equity is essential for improving care outcomes for all patients, regardless of their background.
Learn more
- PDA BAME Network launches factsheet to mark Sickle Cell Awareness Month
- Observing Sickle Cell Awareness Month 2024
- UKOSS – Sickle cell disease
- NHS rolls out ‘life-changing’ treatment for thousands with sickle cell disease
- Mum of girl with rare blood disease donating due to urgent shortage
- Government expenditure on sickle cell disease and cystic fibrosis
- NICE guidance – Sickle cell disease
- NHS – Sickle cell disease
- Newborn blood spot screening
- The world’s first gene therapy for sickle cell disease has been approved in the UK
- No One’s Listening – A Report
- Management of sickle cell crisis
- Invasive procedures in patients with sickle cell disease
- PDA BAME Network
Not yet a PDA member?
If you have not yet joined the PDA, we encourage you to join today and ask your colleagues to do the same.
Membership is FREE to pharmacy students, trainee pharmacists and for the first three months of being newly qualified.
Read about our key member benefits here.