I’VE DONE MANY things in my life, but none more important than an event that happened in 1997 called Gene Aid. The national campaign aimed to engage a large, mainstream audience in the debate about Sickle Cell Anaemia. And raise funds for the treatment of Sickle Cell Anaemia and Aplastic Anaemia, two blood disorders that affect primarily black and Asian people.
When a friend described what he called a ‘Sickle Cell crisis’, it sounded horrendous. He said that his organs stopped working the way they should. It can happen anywhere, anytime – being mild or severe – affecting the arms, legs, joints, back, or chest. It can last for a few hours, a few days, or sometimes longer. My brother has a Sickle Cell trait that he inherited from our mother. It didn’t cause him any health problems. But it could have done if his wife also had the trait because there is a 25% chance with each pregnancy of having a child with sickle cell disease.
I wanted to help, and there it was; I set out to devise a campaign to raise awareness for Sickle Cell sufferers, having never done anything like it before. I enlisted the help of friends and set about organising a star-studded concert at London’s Shepherds Bush Empire, featuring Gabrielle, Keith Washington, and Omar. To my great relief, it was a success and it raised thousands of pounds for the charity, Sickle Cell Society, and gained a lot of media coverage.
The following extract is from a Guardian newspaper double-page feature which Lulu Appleton wrote:
“’Blood Brothers Apart’ – Sickle Cell Anaemia, once thought to be a black persons’ disease, will affect every race within two or three generations. The apparent difference between brothers Wayne and Paul has not had much impact on their lives, apart from the occasional times when they’ve played up the particularity of their situation: in what often appears to be an arbitrary allocation of DNA, Wayne is black like their mother (who is of Afro-Caribbean origin, as is his father), while Paul appears white like his Czechoslovakian father. But there is another, more serious, aspect of their differences.”
The same quirk of fate that decided which genes each brother would inherit also conspired to pass on the abnormal red blood cells that characterise sickle cell anaemia. Although neither brother suffers from the full-blown disease, a simple blood test has revealed that one of them is a carrier. The surprise is that this is Paul. ‘Sickle cell is always thought of as a black persons’ disease, associated with African and Caribbean countries. But it can be in fair-haired, blue-eyed children with nothing to suggest any black ancestry.’ says Bernadette Modell, Professor of Community Genetics at the University College Hospital, London.
Sadly, in the subsequent 24 years that have passed, the only FDA approved therapy for sickle cell disease has been L-glutamine oral powder (Endari, Emmaus Medical) to reduce acute complications among adults and children. Yet, it unused by many hospitals and GPs, because they believe it has minimal benefits. Here is the irony: it took only six months to find a vaccine for Covid19. The Government needs to do more for sickle cell sufferers. To date they have failed to even scratch the surface in terms of research and effective treatments. Does it have to affect more white people before they take any positive action?
The Sickle Cell Society publishes the following information:
“Haemoglobin is the substance in red blood cells responsible for the colour of the cell and for carrying oxygen around the body. People with sickle cell disorder are born with the condition; it is not contagious. It can only be inherited from both parents, each having passed on the gene for sickle cell.”
“The main symptoms of sickle cell disorder are anaemia and episodes of severe pain. The pain occurs when the cells change shape after oxygen has been released. The red blood cells then stick together, causing blockages in the small blood vessels.”
“These painful episodes are referred to as sickle cell crisis. They are treated with strong painkillers such as morphine to control the pain. People with sickle cell are at risk of complications, stroke, acute chest syndrome, blindness, bone damage and priapism (a persistent, painful erection of the penis).”
“Over time people with sickle cell can experience damage to organs such as the liver, kidney, lungs, heart and spleen. Death can also result from complications of the disorder. Treatment of sickle cell primarily focuses on preventing and treating complications.”
“The only possible cure for the disorder is a bone marrow transplant, but this is only possible for a limited number of affected individuals who have a suitable donor. A medicine called Hydroxyurea can significantly reduce the number of painful crises.”
Research on Sickle cell disease has mainly focused on symptom management rather than a cure. Isn’t it time to cure the patient instead of treating the symptoms? Science would have us believe that Sickle cell disease is on the cusp of change, saying that it will significantly increase the treatment options available to individuals to improve their quality of life. But until that happens, more education is needed across the board.
