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Real Voices: Campaigning for better sickle cell healthcare

John James OBE is the Chief Executive of the Sickle Cell Society, the only national charity in the UK that supports and represents people affected by a sickle cell disorder to improve their overall quality of life. In this week’s Real Voices, he describes what is needed in terms of policy change and overall awareness to improve the lives of those with sickle cell.

My background is the National Health Service (NHS), in which I have more than 30 years of professional experience, including various Chief Executive roles. I worked for Sir Bruce Keogh, who was then the medical director of the NHS. It was during this time that I was contacted regarding the Sickle Cell Society, which was losing money due to austerity. The Society was getting to a point where it was questionable as to whether it would survive. The reason I was contacted was because during my career in the NHS, I had always done what I could in terms of highlighting sickle cell, as I was aware that sickle cell disorders are often ignored in many ways. I had long been aware of the great work of the Sickle Cell Society, and I got involved to help it restore and regain the reputation it had, and more importantly continue to provide effective services for those living with this condition.

Sickle cell disorder is this country’s most common genetic blood condition. It carries with it a lot of health implications, such as early mortality, which affects people of all ages. Other complications include strokes, acute chest syndrome, huge fatigue issues, and uncontrollable pain when one has a crisis. Simply put, a crisis is when one’s red sickle blood cells get blocked in the blood vessels. When they do get stuck, this causes acute pain which in most cases requires hospital admission. In London, which has the largest population of people with sickle cell, it is in the top 20 reasons given for admission to hospital. People in a sickle cell crisis are then treated with morphine and other strong painkillers. It is a very challenging condition, which affects all aspects of an individual’s life.

It’s important to say that sickle cell is a condition that has been heavily affected by health inequalities over many decades. Last year, we produced the “No One’s Listening” report which was a Health Select Committee inquiry through an all-party Parliamentary group. That work revealed huge areas of failure in the care of people living with sickle cell, which was linked to avoidable deaths. We found substandard care in A&E and in hospital wards towards people with sickle cell. It isn’t at all uncommon for people going to A&E suffering from a sickle cell crisis to actually be accused of being drug addicts by staff, as if they are lying about their illness in order to get some morphine. That happens across the country. We also found a lack of joined-up provision of sickle cell care, limited community services, limited access to automated blood transfusions and general low awareness of sickle cell amongst healthcare professionals (which resulted in the avoidable death of a young man in his twenties).

This negative attitude stems from the fact that sickle cell disorders primarily affect people whose heritage is from Africa, the Caribbean, and the Middle East. Racial bias is undeniably a factor in the quality of healthcare that people with sickle cell receive. This is also why there has been a lack of investment in sickle cell over the decades. I will illustrate with an example: sickle cell is the biggest genetic blood condition in the UK, with over 15,000 people living with sickle cell anaemia in this country. In contrast, cystic fibrosis affects far fewer people in the UK, and mainly affects people whose heritage is white. Yet, cystic fibrosis care and research receives a lot more funding and awareness than sickle cell. The same goes for haemophilia. Some of that is absolutely to do with racial bias, and a lack of interest in serving the community of people who live with sickle cell disorder.

The Sickle Cell Society was founded in 1979 as a response to growing concerns from a group of patients and healthcare professionals that the treatment for those with sickle cell was inadequate and underfunded. Since that time, people with sickle cell have continued to receive unequal care. When the society was founded, there were no effective new treatments for people with sickle cell. During the 1980s there came a new drug called hydroxycarbimide, which was approved to treat people with sickle cell, and for decades this was the only drug available to them. You may again draw comparisons with cystic fibrosis and haemophilia, which both have huge numbers of different drugs and treatments available for patients. The second new treatment for sickle cell was only just approved in November 2021. That is a huge disparity compared to other conditions. For nearly 30 years there has only been one treatment, which was not even developed for sickle cell patients, but rather is a chemotherapy drug which also has benefits for sickle cell patients.

Sickle cell disorders affect every part of an individual’s quality of life. If you’re a child who is experiencing regular crises and going in and out of hospital, your education will be affected. The caring responsibilities for family members of those with sickle cell will have knock-on effects on their lives as well. Fatigue has a hugely significant effect on people’s lives, and doctors and nurses rarely have the tools to deal with it. On average, patients said they missed more than seven days of school or work a month, and more than half of patients said their mental health was affected by their disorder.

It’s not just important to lobby for increased funding and research, but we also need to raise awareness of the facts of sickle cell. Many people seem to disregard sickle cell because they believe it doesn’t affect them, but our narrative at Sickle Cell Society is that these disorders affect everyone. I gave a presentation to a group of doctors and nurses and mentioned that 1 in every 300,000 white babies born in the UK today has either sickle cell disorder or sickle cell trait. This surprised them, and a few admitted that they believed sickle cell only affected black people. There are still trained healthcare professionals who hold misinformed beliefs about these conditions.

We also need policy makers like NHS England and the Department of Health to think more about sickle cell policy. It is a fact that there are avoidable deaths every year because healthcare staff on the wards are ignorant of sickle cell disorder. In my view, this is outrageous. Therefore, we argue that the training for healthcare professionals must be further expanded to include sickle cell, not just in theory but in practice. Action in policy terms around training and education, and more investment in services and research are what is needed to improve the lives of the thousands of people living with sickle cell in this country.

Learn more about the important work of The Sickle Cell Society.