Raising Awareness of Sickle Cell Disease: A Call to Action During Sickle Cell Awareness Month
September was Sickle Cell Awareness Month, which provided us with an important opportunity to raise awareness through learning, sharing, and discussing the impact of sickle cell disease (SCD) on the people affected.
SCD is the most common genetic blood disorder in the UK, with 1 in every 2000 births affected in England. SCD affects approximately 15,000 people in the UK, yet it remains under-recognised in many clinical settings.
It is essential that we, as healthcare professionals, irrespective of sector, are aware of its challenges and the crucial role we can play in its management to ensure that individuals with SCD receive timely and appropriate care. The ‘No-ones Listening Report’ (2021) highlighted low awareness of sickle cell disease among healthcare professionals and inadequate training identified in medical and nursing training. To understand the health inequalities experienced by people with SCD, we encourage you to look at this e-Learning for Health course on Sickle Cell Disease.
Sickle cell disease is an inherited condition that affects the haemoglobin within red blood cells, leading to abnormal “sickle-shaped” cells. These cells are less flexible and can obstruct and damage small and large blood vessels, causing pain, organ damage, and severe complications like infections and strokes.
While sickle cell disease primarily affects people of African or Afro-Caribbean descent, it also occurs in individuals from the Mediterranean, Middle East, and parts of India and South and Central America. Due to population migration, sickle cell is important in clinical practice in the UK.
Early detection through a newborn screening programme is now standard in the UK. However, awareness and education in the wider healthcare community are essential for early intervention and preventing complications. Delays in diagnosis or treatment can lead to severe consequences, including life-threatening infections, organ damage, and psychological impacts due to frequent hospital admissions and missed education or work.
Non-specialist healthcare professionals, particularly those in emergency departments, general practice, or paediatric departments, can play a pivotal role in recognising and managing sickle cell disease’s common signs and symptoms. These include:
- Vaso-occlusive crisis – acute painful crisis is the most common complication and requires urgent attention and effective pain management.
- Acute Chest Syndrome (ACS) is a form of acute lung injury which can be severe and life-threatening. ACS is a leading cause of death in Sickle Cell Disease and can be mistaken for pneumonia. Therefore, rapid recognition and treatment are critical.
- Anaemia- Sickle cells are destroyed faster than normal cells, leading to chronic anaemia.
- Infection- splenic dysfunction in sickle cell disease increased susceptibility to infection.
This means that people with sickle cell disease are susceptible to a range of bacterial and other infections and at risk of sepsis. Therefore, timely recognition and management are essential.
Though the specialised management of sickle cell disease often involves haematologists, specialist nurses and pharmacists, non-specialist healthcare professionals are frequently the first point of contact for patients during emergencies or routine care. Examples of effective management considerations include:
- Paracetamol, NSAIDs, and opioids are examples of pharmacotherapy typically required for acute pain relief as they are effective pain management options for vaso-occlusive crises.
- It is vital, therefore, that delays in pain relief administration are avoided, as this can exacerbate the pain and lead to further complications. The ‘No one’s listening’ report and other research highlight that patients with SCD often face stigma when seeking pain relief, as their chronic pain can be misunderstood.
- Infection Prevention—People with SCD are more susceptible to infections, so prophylactic antibiotics and vaccinations, including pneumococcal, meningococcal, and annual flu vaccines, are crucial. Early intervention with antibiotics is essential if an infection is suspected.
- Other treatment management options include -hydroxycarbamide, hydration, oxygen therapy and blood transfusions.
Sickle cell disease disproportionately affects individuals from Black and ethnic minority communities, which can contribute to inequalities in healthcare access and outcomes. In the UK, research shows that patients with SCD often experience delays in treatment, discrimination and prejudiced treatment from healthcare professionals and are sometimes perceived as ‘drug seeking’ when seeking pain relief due to acute crisis. As healthcare professionals, we are responsible for providing compassionate, evidence-based care without bias or prejudice. Understanding the lived experiences of SCD patients, tackling our knowledge gaps and addressing implicit biases can help improve the quality of care delivered and patient outcomes.
Primary care teams also play a crucial role in the long-term management of SCD. Regular reviews, medicine optimisation, mental health support, and monitoring for complications such as renal disease, retinopathy, or leg ulcers should be part of routine care for individuals with SCD. Fostering strong relationships with other multidisciplinary team members, Such as haematologists, other specialists, GPs, and primary care staff, can help coordinate care and improve outcomes for patients with SCD.
The NHS Race and Health Observatory’s ‘Digital Discovery report’ highlights areas for improvement and provides a series of recommendations, ranging from interventions to service design, to improve patient access to and experience of SCD care.
Sickle Cell Awareness Month is an opportunity for us all to learn and raise awareness by understanding the complexities of SCD, recognising the early signs, and providing timely, equitable, and patient-centred care. In doing so, we can make a significant difference in the lives of those affected by sickle cell disease.
Let’s use this month as a springboard to commit to our ongoing education, reduce healthcare inequalities, and ensure that patients with sickle cell disease receive the highest standard of care across all healthcare settings.
The Netflix series Supacell highlights sickle cell to a global audience and is worth a watch!
References:
1. NHS Race and Health Observatory. Sickle Cell Digital Discovery Report: Designing Better Acute Painful Sickle Cell Care, 2022
2. National Institute for Health and Care Excellence Clinical Knowledge Summaries (NICE CKS), Revised 2021
3. All-Party Parliamentary Group on Sickle Cell and Thalassaemia. No One’s Listening: The Inequality in Sickle Cell Care, 2021