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Sickle cell is an inherited disease. When parents both carry the sickle cell trait, it is a cruel game of chance as to whether children inherit the trait but remain asymptomatic, have no trait at all, or have to live with symptomatic sickle cell disease.
This randomness is reflected in my own family. My late mother was one of 12 children, but she was the only one of those 12 with symptomatic sickle cell. Some of my aunties and uncles carry the trait, and I found out I carried the trait when I got pregnant with my daughter, now six, who does have the trait, yet my son does not.
This is a disease that can affect anybody. But it’s also one where early screening can identify those with the trait who are at risk of passing it on to their children. This gives us the chance to diagnose the disease early in life and treat it accordingly.
But, sadly, standards of care are too often desperately short of what those with sickle cell deserve.
Sickle cell affects red blood cells and carries severe symptoms for many who are born with it. These include increased risk of severe infections, anaemia, and episodes of extreme pain known as sickle cell crises which can last up to a week.
The ‘No One’s Listening’ report by the APPG on Sickle Cell and Thalassaemia, of which I am a member, was triggered by the tragic death of Evan Nathan Smith in North Middlesex Hospital. Evan was just 21 and mistakes in his treatment led to his entirely avoidable death.
This report, and the issues it highlighted in secondary care for sickle cell patients that has led to misunderstanding, suffering and in some cases death, reminded me of some of the issues my own family faced as we cared for my late mother.
My mother described it as like someone chiselling at her knee, her bones and her joints
As a child, I had to learn about the litany of drugs such as diamorphine, co-codamol, penicillin, folic acid and the variety of painkillers sickle cell sufferers have to take. I knew how important it was for my mother to have access to oxygen when she had shortness of breath. I knew how important it was to properly outline the symptoms my mother was experiencing to the paramedics whenever she was hospitalised, so that she was given the right medication and not just paracetamol.
I also knew the blank expressions on the faces of doctors and nurses who did not understand the searing pain that sickle cell patients face when they are going through a crisis. My mother described it as like someone chiselling at her knee, her bones and her joints. This is unimaginable pain for most of us. It is abhorrent that some describe sickle cell patients as addicts when they need strong pain medication.
Understanding sickle cell can be a matter of life and death. I know how timely it was that my mother got the transfusions she needed, and because of this she was able to have three healthy children and see her granddaughter before she died in 2016, aged 60.
It breaks my heart that, because of failures in the system from those who should have helped him, failures those who have experienced sickle cell treatment in the UK are sadly all too familiar with, Evan will tragically never get the opportunity to fulfil his life ambitions. To hear that Evan had to call 999 from his hospital bed to get oxygen in 2019 is a damning indictment of the failures we are still seeing today.
As our communities become more diverse, it is vital staff across the health system understand this disease. We need a clear action plan so that no one with sickle cell should have to die from a lack of awareness in the future.
Florence Eshalomi is Labour MP for Vauxhall, and member of the Sickle Cell and Thalassaemia All-Party Parliamentary Group
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