Words By Colm Kelliher
Dr Norris Igbineweka, who is presently a Clinical PhD Research Fellow at the University of Oxford’s Weatherall Institute of Molecular Medicine and Imperial College London’s Haematology Department, spoke to BSMS OHP on Monday 26th October 2020 on the title:
“Sickle Cell Disease: A black lives matter disease or a glimpse into the struggle of humankind?”
This was an inspiring talk, shining a spotlight on a disease that does not receive enough attention.
Sickle cell disease (SCD) is commonly studied in genetics, and many people have heard of it. However, we often fail to grasp the severity of the condition, and how it affects the greater population. Dr Norris Igbineweka delivered a webinar for BSMS OHP society to inform us of this. In SCD, a gene mutation leads to abnormally made haemoglobin, which gives the red blood cells a ‘sickle’ shape. These abnormal blood cells get stuck in blood vessels, leading to the death of tissue and inflammation. This causes great pain and suffering for patients, and it can occur anywhere in the body. While the disease is most common in Africa and other areas more heavily affected by malaria (due to SCD’s protective effects against it), there are many people with the disease in the UK.
People with SCD are living longer due to better treatments like hydroxycarbamide, but they often pass away much earlier than a healthy person. Genetic studies have helped to improve treatment options greatly. Yet, a focus on European populations has likely hindered our ability to treat SCD. Few studies have been conducted in African populations. Studying typical and SCD genomes in this group earlier could have led to better treatments for the disease. We need more genetic markers of disease severity, as well as clinical biomarkers and education about the disease. The SCD population require a stronger, more influential political voice to support them like some other diseases have.
Conditions such as cystic fibrosis affect fewer people than SCD but receive much more funding and attention. This disparity in research and funding can greatly decrease SCD patients’ quality and length of life. Studies have shown that the immense pain that the disease causes is sometimes mis-identified by clinicians. Patients can be labelled as drug-seeking for wanting pain relief or given too little analgesia. This disease is invisible to the naked eye, and a lack of understanding often leads to negative outcomes. Undoubtedly, stigma and discrimination play a role too. This has all been documented by NICE and other health organisations. Greater research into and understanding of SCD today may lead to less disparity tomorrow.
SCD is too often thought of as ‘a disease of black people’. While it has origins in African areas, it can and does affect everyone. Extrapolation of 16th century data suggest that 20% of British people who view themselves as white have the sickle cell trait. The pain and chronic multi-organ damage caused causes great suffering in families. Additionally, the hospitalisation due to this is very costly. SCD alone cost the NHS £19 million between 2010-11. Spending money on finding better SCD therapies and treating the co-morbidities of the condition early is important. It doesn’t just vastly improve someone’s quality of life, as vital as that is, it also helps our greater society. With more money available, more beds in hospitals will be available for people that need them, and more money can be spent researching/providing treatments for other diseases. SCD is a condition that must be understood and treated correctly, to improve the lives of all of us.
Overall, SCD is a serious, painful condition associated with a vast number of co-morbidities. People with the condition can face discrimination in healthcare and day-to-day life. The ‘invisible’ condition takes a great toll on the individual, but also on society. It is not just a ‘disease of black people’. There is still a lot of work that needs to be done to educate people on and treat the condition better. We must strive to overcome the obstacles associated with properly treating SCD to relieve the suffering the disease brings. If you would like to learn more about SCD, or OHP, please find the BSMS OHP society on social media or visit the YouTube channel (https://www.youtube.com/channel/UCo7wAWLUnu22Pnd3qt3xQpA) to look at some of our webinars.
We would like to express our sincere thanks and gratitude to Dr Norris Igbineweka for delivering a thought-provoking webinar to our students and staff. We would also like to convey our appreciation for members of BSMS academic faculty who attended the event: Professor Malcolm WR Reed, Dean of BSMS, who delivered a note of thanks at the event; Dr Tim Chevassut, Reader in Haematology and Consultant Haematologist; Professor Melanie Newport, Professor of Infectious Diseases and Global Health.