What is Sickle Cell Disease?
Sickle Cell Disease (SCD) is a rare condition that affects haemoglobin, a protein carried by red blood cells that delivers oxygen throughout the body.
It is a lifelong blood disorder that occurs when a person inherits sickle cell genes from both parents.
Healthy red blood cells are disc-shaped and flexible so that they can move easily through the blood vessels and carry oxygen to vital organs.
However, in people with SCD red blood cells lose their flexibility and become rigid, sticky and crescent or sickle shaped.
The sickling process causes the breakdown of red blood cells, which leads to haemolytic anaemia (low haemoglobin and low oxygen delivery in the body due to red blood cell destruction).
The sickled red blood cells can damage blood vessel walls, resulting in vasculopathy (lesions occurring in small blood vessels), and this in combination with the sickled cells, causes blockages in capillaries and small blood vessels, causing pain and impeding the flow of blood and supply of oxygen throughout the body.
Patients with SCD experience progressive, lifelong complications and severe morbidity, including damage to major organs such as the liver, kidneys, lungs, heart and brain, which contributes to early death and can erode the potential of patients and their families.
Chronic lack of understanding among medics
Speaking to healthcare professionals, the study found that almost three quarters (73%) said that patients of lower economic status can be ‘more difficult to treat’.
Almost a third of doctors and nurses (31%) found it challenging to understand their patients’ needs.
Only half of healthcare professionals (50%) feel they have sufficient tools to manage the long-term damage that SCD causes.
For Sebastian at Global Blood Therapeutics, the issue comes down to a lack of education among healthcare professionals, and a need for a holistic change in every element of global healthcare systems.
‘It’s really important for medical professionals to have an openness to understand the community, the sickle cell patients, and then to start spreading that message and educating others to really understand that as well,’ Sebastian tells Metro.co.uk.
‘You need to be open in terms of really understanding the underlying beliefs of the community and the SCD patients, and then working with that community to improve care and treatment, and then, as a result, the standard of living for that community as well.
‘For me, it really is about understanding, compassion, humility, and then working together to address things as they come up.’
Sebastian says that for the prospects for sickle cell patients to really improve in the UK and the rest of the world, there needs to be a holistic solution, with governments giving their support to healthcare systems.
‘The study was global – across 10 countries, four continents – and these findings were universal,’ says Sebastian.
‘So, when I look at the healthcare system, it has to be about the patients, the caregivers, the providers, and the governments that support these healthcare systems as well.
‘It’s about all four stakeholders really aligning on what are the priority projects and initiatives that will move this disease area, and then having clear action plans and follow-through on that as well.’
Shocking new research has revealed the extent to which sickle cell patients are being failed by healthcare – with seriously unwell people being treated like criminals.
According to a new large-scale study of sickle cell treatment, nearly half of patients (46%) stated emergency room healthcare providers did not trust them when they told them about their symptoms, and 48% said they were treated like drug seekers in A&E.
Sickle cell disease (SCD) is a rare syndrome that causes red blood cells to form a sickle shape, resulting in excruciating pain, long-term organ damage, and, in some cases, early death. It is most likely to impact Black people from Africa or the Caribbean.
Global Blood Therapeutics (GBT) has released fresh global study aimed at highlighting the ‘unmet requirements’ of sickle cell disease patients, as researchers claim that international healthcare systems continue to fail people of minority ethnic origins.
‘The research shows that SCD is a challenging disease to live with, not just for patients and caregivers, but also for healthcare workers,’ says the paper. Global Blood Therapeutics’ says head of Europe and GCC, Sebastian Stachowiak.
‘When comparing specialized sickle cell treatments to the care patients could receive in an emergency room, this report truly underlines the gap in quality of care.’
The study, which took place in ten countries including the United Kingdom, the United States, Canada, Germany, France, Brazil, and the Gulf Cooperation Council (UAE, Bahrain, Oman, and KSA), discovered that sickle cell disease patients are still ‘dramatically underserved’ by healthcare systems, and that medical professionals do not believe they have the knowledge of the disease or their patients to properly treat them.
People with sickle cell disease, who are more likely to be of African or Caribbean heritage, die between the ages of 40 and 45. GBT feels that this is far too soon, and that better knowledge and communication may help people live healthier, happier, and longer lives around the world.
Nearly 40% of patients had received poor care from emergency medical specialists, which they suspected was due to their ethnicity or socioeconomic status, according to a study of 1,345 persons, including people with SCD, carers, and healthcare professionals.
More than half of patients (54%) indicated they had received unsatisfactory care from emergency medicine providers in the past due to a lack of knowledge regarding SCD.
The new findings follow a landmark UK probe in 2021, which found “severe care deficiencies” in the management of sickle cell patients as well as indications of racist attitudes.
The research from last year showed evidence of substandard care for sickle cell patients admitted to general wards or A&E, a lack of understanding of sickle cell among healthcare workers, and obvious examples of poor training and investment in sickle cell.
This recent study reveals that this is not a problem exclusive to the United Kingdom, but rather a global issue.
The realities of living with sickle cell
Aisatu Beadford-King was born in Liberia and moved to the UK when she was three years old. At this point she was already displaying symptoms of having sickle cell disease. However, her mother was not aware of her daughter’s condition.
‘Every time they would take me to hospital, the doctors would tell my mum it’s malaria, she is fine,’ says Aisatu.
At the age of five, Aisatu experienced her first crises, and she was eventually diagnosed with sickle cell disease. Her family began testing for the hereditary disorder and learning about the ailment as a result. Sickle cell education is poor in the UK and around the world, according to Aisatu.
‘I have had a few negative experiences while receiving treatment for sickle cell, especially in A&E,’ Aisatu says.
‘I’ve had nurses tell me I’m exaggerating my pain, others not listening to me when I’m explaining what works for me and what doesn’t. It makes me feel less than, I feel like maybe my pain isn’t valid and I’m just wasting time.
‘It truly infuriates me when I have to experience things like this.’
Aisatu says seeing how sickle cell affects her family and friends is very hard to deal with.
‘The mental health side of sickle cell has to be my biggest struggle,’ she adds.
‘People don’t understand what sickle cell is and how different environments can even cause someone to have a crisis. People need to know how to get tested, so they know if they have the sickle cell trait and can prevent passing it onto another generation.’
This thought prompted Aisatu and her mother to create a charity – Africa Sickle Aid. They have been able to send care packages to developing countries to support those who may not have access to treatment and care services.
‘It was my mum’s idea to start the charity – to understand my sickle cell. I am thankful to her, not everyone is so lucky.’
Aisatu is determined to advocate for global health equity in terms of sickle cell, to ensure that those living with sickle cell in developing countries know that someone across the pond still remembers them and relates to their journey.
The State of Racism
This series takes a close look at racism in the United Kingdom.
We want to know how, where, and why racism affects people of color from all aspects of life.
Even if it makes you uncomfortable, it’s critical that we improve the language we use to talk about racism and continue the difficult talks about inequity.