The implicit bias of sickle cell disease

Growing up, I vividly remember my little sister’s screams when she was going through a sickle cell crisis. I felt so helpless and wished I could take the pain away. In hindsight, I can only imagine how my parents felt.

Unfortunately, thousands endure the pain of sickle cell disease (SCD) in England. Yet this inherited condition remains one of the most misunderstood and underfunded health issues within the Black community. SCD has become one of the fastest growing genetic conditions in England, with 250 new cases every year.

Although health outcomes have improved, evidence shows that long-term sufferers still feel marginalised. Their frustrations largely stem from a perceived lack of empathy in a health care system that does not fully recognise their struggles. When compounded with limited treatment pathways, these poor experiences leave many feeling neglected and unsupported. A report on the London Ambulance Service from earlier this year and recent challenges in Derbyshire do little to mitigate the concerns of Black people accessing care. Instead, they underscore the real tensions within our health and care system, reinforcing the paradoxical relationship between inclusion and inequity.

Access to treatment

Recently, I had an insightful discussion with Kye Gbangbola, a well-known sickle cell campaigner. He highlighted several systemic challenges, including the limited treatment options available in the NHS. Presently, two medications are NICE approved on the NHS for sickle cell disease compared with five approved medications in the United States. In the UK, this is largely due to market access hurdles, such as lengthy regulatory processes, and cost-effectiveness. The impact of this is mainly felt by patients, who are affected by a lack of options and the complexities of a financially stretched health care system.

Earlier this year, NHS England announced genetic blood testing, heralded as the first in the world, to improve the effects of blood transfusions and reduce the side effects of SCD and thalassemia (a similar blood disorder). Before this, in 2020, specialist haemoglobinopathy co-ordinating centres (HCCs) were commissioned across England to support hospitals in areas with less expertise in treating and managing SCD, ensuring equitable, joined-up care. In 2023, these were further developed with additional specialist services known as Hyper Acute Units (HAUs). These units are tasked with improving care during a vaso-occlusive crisis, a painful and traumatic complication of SCD.

However, HAUs are only in London and Manchester. In a life-threatening situation, your only option is the closest emergency department, where clinical staff may not know how to deal with a complex case of SCD. Access continues to feel like a postcode lottery. Unfortunately, getting ill in the wrong place impacts the quality of care, which negatively affects the patient experience.

Experiences of care

In 2021, the Sickle Cell Society published a report that highlighted the harsh realities of people living with SCD. Racism and inadequate care are relived regularly, particularly in emergency departments where pain is underestimated or dismissed. Unfortunately, health professionals often lack the training or empathy to understand patients’ predicaments, resulting in patients feeling unseen and unheard. Nneka Smith, a sickle cell advocate, expressed frustrations around a lack of knowledge from ‘those who ultimately have the power to decide how my life will progress’.

Another issue is that the specialist SCD workforce is disproportionately low. Research shows that in comparison with cystic fibrosis nurses, there are a lower number of specialist SCD nurses, despite SCD having a higher prevalence. These challenges become apparent when looking at the psychosocial effects associated with SCD. Due to the toll of the illness, most people suffer from anxiety and depression. Despite this, there is a perceived lack of accountability in the health service, as mental health trusts feel SCD should be treated in acute trusts. In an already depleted workforce, patients fall through the cracks and struggle with fragmented care. Living with this condition without appropriate mental health support contradicts the principle of ‘parity of esteem’. As a patient, it wouldn’t be wrong to think that SCD is less valued than other conditions in the system.

Racial discrimination

Racial disparities still exist within health care, and SCD is a clear example of how institutional racism has a negative impact on the provision of care. SCD largely affects people of Black African and Caribbean heritage, and is extremely rare among those of white European descent. The fact that the condition is predominantly found in certain racial and ethnic groups highlights how health conditions cannot be fully understood or addressed without acknowledging the role of race and systemic health inequalities. Ignoring the racial dimension of SCD is no different to overlooking the structural inequalities that underpin how health care is prioritised and delivered.

SCD remains a long-term health condition that is not consistently recognised as a disability under UK law. This has grave consequences for those living with the disease, despite the overarching Equality Act which should be safeguarding their rights. This isn’t helped by the fact that SCD is not classified as an NHS prescription-exempt condition, unlike, for example, diabetes insipidus, a rare form of diabetes with a lower prevalence. Patients are penalised for a condition they cannot control, further deepening the existing medical bias. It begs the question: how can you move forward when the system keeps pulling you back?

As a clinician, I understand the daily pressures of our role and the desire to do our best for our patients. But sometimes the system doesn’t enable that. We need to recognise that bias exists and take steps to dismantle it. The first step should be rewiring our approach to how we understand, treat and support people with SCD. In recent weeks, there have been positive steps when it comes to addressing some of these – for example, increasing the number of blood donations to support SCD, additional funding and innovative clinical trials, although more is needed. I hope the 10 Year Health Plan is a turning point when it comes to tackling health and racial disparities, and that there will be a greater expectation to understand conditions that affect different demographics of the population. As Lord Adebowale recently highlighted, Black people deserve equitable care. The truth is that better care is needed for everyone, regardless of ethnic heritage or where they live.