Sickle Cell Anemia: An Ignored Crisis

A single point mutation in the beta-globin gene has shaped the course of Black medical history. Just one small change in the genetic code has manifested into a global health issue, although you wouldn’t know it from the response of governing health bodies.  

Sickle cell disease (SCD) is caused by a mutation in the hemoglobin gene. Abnormal hemoglobin affects the shape of red blood cells, changing them from biconcave and flexible to crescent-shaped and rigid. These misshapen cells are unable to move through blood vessels and consequently cause blockages. Such blockages prevent vital organs and tissues from receiving the necessary oxygen supply, leading to excruciating pain - termed ‘pain crises’ - and in many cases, death. 

Whilst SCD affects more than eight million people worldwide, the burden of disease is not shared equally. Black communities bear the brunt of this condition. In the United States, nine out of 10 people who are diagnosed with SCD are of Black or of African descent. 

Despite this, SCD receives a fraction of the research funding and policy attention given to rarer diseases that primarily affect ‘majority’ populations. 

So, what happens when those affected are met with indifference instead of urgency? The outcome is a silent crisis. One that persists not only because of a lack of awareness, but also because the systems in place have decided it is not a priority. 

The pain crisis

Sickle cell is infamously characterised by vaso-occlusive episodes, better known as pain crises. This occurs when microcirculation is obstructed by sickled, ‘sticky’ blood cells, resulting in extreme pain that can last from a few hours, to a few days.  

As the pain cannot be seen, many struggle to understand what it actually feels like. Some have compared it to “being stabbed repeatedly while having a migraine throughout your whole body,” Others likened it to “broken bones and glass” flowing through their bodies. And for some, words simply failed - only that the pain is anything beyond description. 

Patients consistently experience substandard care during crisis episodes. Healthcare professionals lack knowledge on how to treat sickle cell - direct evidence of inadequate training and investment. In moments of unbearable pain, patients are often forced to explain their own condition to doctors and nurses who should already understand it. 

With SCD predominantly affecting those of African and Caribbean heritage, it is impossible to ignore the racial undertones of the maltreatment of patients. 

In the UK, sickle cell was once framed as a "migrant disease," a condition brought in by Black communities rather than an inherited genetic disorder. The far-right media in the mid-1900s fueled the belief that sickle cell was an infectious disease, passed on through migrants, and even Black nurses. 

This misconception fueled decades of underinvestment in care and research, reinforcing the idea that SCD was someone else’s problem. A problem that didn’t require the same level of attention or resources as other chronic illnesses.

Dying to be heard 

In 2021, researchers estimated that 34,600 deaths worldwide were attributable to SCD. However, after accounting for severe misdiagnosis, the total deaths came to 367,000. 

Many of these deaths are avoidable. This is particularly seen in the UK, where Black patients are failed despite the extensive resources that should be made available to them. 

Evan Smith, aged 21, passed due to a "failure to appreciate the significance of [his] symptoms” and “ a lack of understanding of sickle cell disease in the medical and nursing staff.” After going into septic shock, he was denied oxygen by nursing staff and placed in an isolated “lodging bed”. From this hospital bed, Smith resorted to calling 999 for an ambulance as there seemed to be no other help available. He was then  administered oxygen and received a blood transfusion. However, Smith was already in the midst of a sickle cell crisis and had experienced multiple cardiac arrests, leading to his death the following day. The delay of the medical staff was fatal. 

Evan’s case is not an anomaly. In fact, it is rare to hear a Black patient with SCD give an account of a positive hospital experience. More often than not, they have to scream to be heard. Sometimes even that is not enough. 

As patients with sickle cell disease continue to advocate for themselves, they are not alone in this fight. A number of organizations are committed to increasing awareness and push for better healthcare standards. 

Founded in 1979, the Sickle Cell Society operates at both a community and nationwide capacity. From helping those in local areas, the charity has been instrumental in bringing about parliamentary change. For example, the ‘No One’s Listening’ inquiry, which followed the untimely death of Evan Smith. 

Their work is critical in bridging the gap between patient needs and healthcare realities. Such initiatives have contributed to the fast-tracking of a new therapy, dubbed ‘exa-cel’, which has been shown to prevent pain crises in 97% of patients. The gene therapy edits a patient’s stem cells to reactivate fetal haemoglobin. The modified stem cells are then re-infused into the patient, now giving them the ability to produce functional red blood cells. 

The treatment is currently priced at £1.5 million per dose (covered by the NHS) and is only available to 50 patients a year. However, this is a step in the right direction. 

Professor Bola Owolabi, director of healthcare inequalities at NHS England, echoed this. 

“This represents a monumental step forward in the treatment of people with sickle cell disorder, a condition that mostly affects people of Black African and Black Caribbean heritage,” she said. “This groundbreaking therapy, available on the NHS, represents a very real prospect of a cure for this devastating disorder.”

‍

‍

Many Black people in the UK carry the sickle cell trait. If both parents are carriers of the trait, there is a 25% chance their child will inherit sickle cell disease. 

Request a blood test from your local GP.

Donate blood today to support SCD patients who need regular transfusions

Featured artwork by Richard Akanji Amole Akanji Studio