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People with sickle cell disease benefit from less funding, staffing and GP support than patients with other comparable conditions, according to a report commissioned by the NHS Race and Health Observatory.
‘Sickle cell: comparative review to inform policy‘, published on 19 June 2024 to mark World Sickle Cell Day and produced by researchers at Imperial College London, compares research on and access to care for sickle cell disease with two other lifelong hereditary conditions: cystic fibrosis and haemophilia.
Sickle cell disease can affect anyone; however, it is particularly common in people with African or Caribbean family backgrounds. Racial inequalities have been identified as “a key factor in the poor and sub-standard treatment reported by sickle cell patients”, the report said.
It added that regional disparities have been reported in access to specialist treatment.
The comparative review found that there are 0.5 specialist nurses per 100 people with sickle cell disease in England, compared with 2.0 specialist nurses per 100 people with cystic fibrosis, while cystic fibrosis receives 2.5 times more research funding than sickle cell or haemophilia.
Chemotherapy drug hydroxyurea and gene-editing therapy exagamglogene autotemcel (Casgevy; Vertex Pharmaceuticals) are the two treatments currently approved for sickle cell diseases.
However, the report said that it “is expected that about 50 people with sickle cell per year might receive this treatment on the NHS.”
In comparison, cystic fibrosis and haemophilia drug development has led to “multiple treatment options,” it added.
The report also found that people with sickle cell disease have reported experiencing racism while accessing treatment, including being called profanities by healthcare staff, staff assuming patients are “drug seekers” and being assumed to have a lower pain threshold.
Sickle cell diseases include sickle cell anaemia, sickle cell–haemoglobin C and sickle cell-beta-thalassaemia.
These diseases, which affect red blood cells and can cause anaemia and episodes of severe pain, which is termed ‘pain crises’.
These conditions affect around 14,000 patients in the UK, compared with around 12,000 patients with cystic fibrosis — primarily affecting the lungs and digestive system — and less than 10,000 patients with haemophilia, which affects blood clotting.
Carl Reynolds, senior clinical adviser at the NHS Race and Health Observatory, said: “This report shows in stark detail what many people with sickle cell already know — that they face systemic inequities in healthcare, research investment and access to specialist services.
“These are not just oversights; they are consequences of structural racism and neglect. We now have clear, evidence-based recommendations for change. It’s time to act, not just listen.”
The report recommended that more sickle cell training for healthcare professionals, including in primary care, is needed.
More funding for research would improve poor awareness of the disease among healthcare professionals and the general public, and increase the number of clinical trials, registered drugs and peer-reviewed publications about sickle cell, it added.
The report suggested that a dedicated call for research from the National Institute for Health and Care Research (NIHR) — as has been issued for cystic fibrosis — “would likely have a far-reaching impact and contribute to address some of the problems outlined”.
The chief executives of national cystic fibrosis and haemophilia charities have supported the calls for funding and research into sickle cell disorders.
Commenting on the report, Kate Burt, chief executive of the Haemophilia Society, said: “As a charity which also supports people living with rare genetic disorders, we appreciate the challenges around funding and dedicated research, but this is no excuse for substandard care and support. We hope this report results in meaningful change which leads to better treatment and improved quality of life for people with sickle cell disorder and other rare, inherited conditions.’
David Ramsden, chief executive of the Cystic Fibrosis Trust, said: “It’s vital that conditions, like sickle cell disease, receive the resources needed for research, care and treatment to ensure everyone can live a life unlimited by their condition.”
The NIHR has been approached for comment.
Read more: ‘Sickle cell disease – causes, treatments and the patient experience‘
