Sickle cell disease – the final frontier!

October is Black History Month in the UK – a month earmarked for acknowledging and celebrating the immense contribution of Black people to all sectors of British society. During the 1940s, Black nurses came to the UK from Africa and Asia, with a larger number from the Caribbean. They helped to staff the newly established National Health Service, which was experiencing a workforce shortage. To mark the 75th anniversary of the NHS in 2023, the RCN paid special tribute to prominent Black members of the nursing profession, including the group that became known as the Windrush Generation. Some of these global majority nurses are featured in an excellent publication by Jak Beula (2021). Many of these early pioneers were trailblazers who demonstrated excellence in nursing practice. The introduction and development of treatment for sickle cell disease is one notable example. 

Sickle cell disease is an autosomal recessively inherited disease that disproportionately affects people of Black African and Caribbean origin. For nurses who work in the speciality and patients with the disease, there’s frustration that it’s not well understood. Patients and their families are often victims of health inequalities in the NHS, and this has been highlighted in coroner inquests, for example, following the death of Evan Nathan Smith in 2019. Evan was a young man with sickle cell disease whose death in hospital was ruled as having been avoidable and occurred because of neglect, lack of due diligence and system failures. This is just one of a number of examples in the last few years.

Many in the field, including myself for almost 40 years, were asking: “Why is all our effort to educate health care professionals failing to achieve the desired result of preventing avoidable deaths?” A lot has been achieved in the development of services, and specialist doctors and nurses. But efforts to develop a generation of knowledgeable, skilled, competent, compassionate generalist nurses, capable of providing effective care to those with sickle cell disease is what I see as the ‘final frontier’ that we still need to conquer. Many strides have been made in the last 4 decades, but lack of due diligence, poor nursing care, racism and a negative attitude to this patient group remains a challenge facing many patients.

Sickle cell disease affects more than 15,000 people in the UK. It’s very unpredictable with many potentially life-threatening clinical complications that can cause unexpected death. Death, especially of young people, can be very alarming for nurses because many underestimate the severity of the disease and the seriousness of the patient’s symptoms. They thus fail to act quickly enough when there is deterioration which can lead to death. Generalist nurses often make the mistake of assuming that a patient with sickle cell disease admitted to hospital with vaso-occlusive sickle cell crisis pain merely needs opiate medication. They fail to maintain nursing observations and to identify and act promptly on any deviation, yet the condition of these patients can deteriorate very rapidly (National Confidential Enquiry into Patient Outcome and Death 2008).

Some nurses perceive that this group of patients are challenging to nurse and, as a result, adopt a negative attitude towards them. This can result in a failure to act in the best interest of the patient, even when they are seriously unwell. 

Insufficient coverage of sickle cell disease in nursing curricula has resulted in a decline in knowledge about it. Thus, many nurses fail to develop confidence, skills, proficiency and competence in caring for this patient group, leaving nurses and patients vulnerable (All-Party Parliamentary Group 2018, Sickle Cell Society 2021 & 2023).

Much work still needs to be done to reduce fatalities through education, robust system changes and, very importantly, by challenging racism and the negative stereotyping of this patient group.  
Nurses play a pivotal role in care and management of patients with sickle cell disease especially during an acute illness. Skilled nursing is the bedrock for effective management of these patients and crucial for prevention of handicap, morbidity, and mortality.

We need all nurses to be committed to helping conquer the ‘final frontier’ –  to become knowledgeable practitioners with the ability to provide excellent care and eliminate the unnecessary suffering and avoidable deaths of people with sickle cell disease.