The History and Reality of Living with Sickle Cell Anaemia, with ChiyènneAdrien

The History & Reality of Living With Sickle Cell Anaemia, with Chiyènne Adrien

Written by Amarion Scarlett-Reid | TBC Young Champion

Sickle cell anaemia is a genetic disorder that affects around 17,000 people in the UK and 8 million people worldwide. The inherited disorder affects the haemoglobin protein of red blood cells meaning a lack of oxygen can cause these cells to transform from their functional disc shape to a – sickle shape. Although sickle cell specifically affects red blood cells, because of the vast network of blood vessels around the body sickled cells can block blood flow anywhere in the body from ‘head to toe’; this can cause major episodes of pain called ‘Crisis’. I spoke with Chiyènne Adrien who expanded on her experience living closely with someone affected by sickle cell, as well as the reality of the condition within racialised healthcare.

Misconceptions and misunderstanding

Often sickle cell has been shrouded in misunderstanding as it mostly affects Black and Brown people, this places the disorder at the intersection of racial bias and healthcare inequality. Since sickle cell’s discovery in Western medicine, racial bias has meant research and education has been inadequate in relaying an understanding of the disease to people who do not suffer with it. “Racism is already prevalent within many institutions within the UK, including the education system and healthcare system so it is clear that as Sickle Cell is an illness that primarily affects people of black and Asian heritage, education will not be pushed out for it, nor will the support from charities and other large organisations to help reach a wider audience.”

Beyond this sickle cell as a genetic disorder means its invisibility can often breed ignorance as “people tend to look at disabilities and forget that there is no specific ‘look’, especially considering many do not present themselves outwardly. Someone close to me has experienced weird looks and had people question why they were parked in a disabled parking spot (whilst presenting a blue badge in their car) by random people”.

Within the Black community “there are not enough people who are aware of the importance of regularly educating themselves and blood donations”. Only 2% of blood donors are of Black heritage and this significant shortage can prove detrimental to sufferers of sickle cell who require blood types that match them. 

“Within the education system, it is also not educated enough. Whilst I was at school I was taken out of a lesson and told I had Sickle Cell, to which I responded with “No, I don’t have Sickle Cell, but I do have the trait”, they proceeded to tell me I had Sickle Cell and explained all of the resources they had in case I needed them. It is great that they had things in place for the individuals in my school who did have it, but their lack of knowledge was highlighted within this scenario because Sickle Cell and Sickle Cell Trait should not be used synonymously.”

Life with sickle cell

Sickle cell affects the daily functions of the body, but how large its impact “completely depends on the day.” Overzealous exercise or work can induce a crisis and so growing up Chiyènne began “to realise how different things impacted him [her father], some as simple as a walk or on the extreme end a concert, and then that’s when reality sets in again, and I realise how much it’s affecting him doing something fun, or a day-to-day task. That’s why I have to think and plan to see what we may or may not be able to do together.”

“Most days it isn’t so bad, and sometimes it feels as though he isn’t sick anymore. But when it is bad, it’s really difficult. It’s not nice seeing how restrictive it is for him, and how it affects him in different ways.”

Each day passed living with this condition is a protest to all that it regularly denies, for many it restricts the simple tasks we take for granted such as rushing to catch a train or bus – for sufferers this can be dangerous and with sickle cell’s ability to affect blood vessels anywhere – life-threatening. 

“It has given me a lot of anxiety over the years of him not being there for significant events in the future, and other things too, so I try not to think of that too much. But luckily, he has been able to show up to more or less everything, and it shows his strength despite suffering from multiple complications and symptoms of sickle cell.”

Although living closely with someone who suffers with sickle cell requires immense preparation and to “observant of what’s going on”, this shouldn’t be unique to these carers or family members. Broader education means we have greater understanding, can help each other, and ensure good communication – something that often feels amiss for those suffering.

“Having children of my own and educating them about food and trying to feed them the right things- that was a start as well, them recognising the foods that they’re eating, and giving them a better relationship with food and more understanding. It’s not just eating food to be full, it’s eating food to promote life- so that is a big inspiration watching them grow.”

Care and the NHS

The history of sickle cell treatment has been one of dismissal and underfunding because it affects minorities, its routinely overlooked in the UK. However new medication has given new hope especially if it can reduce the volume of medication people have to take in a day. New medication still, doesn’t reduce all anxieties as old methods to treat sickle cell will remain the most popular method due to a lack of funding. In the outset of “the blood scandal in the UK a few decades back” a lot of people “do not feel comfortable” with blood transfusions which is the most common method of treating symptoms of the disease.

Care at home requires a lot of help from carers, family and machinery, at the hospital the level of care can be “good or bad, depending on who is there when you go to the hospital. If there are pain nurses it tends to be okay, and if the sickle cell nurse is there too. If not, there can be more issues regarding the treatment.”

“A lot of the nurses and doctors tend to look at people with Sickle Cell as drug addicts when they just want pain relief. People with Sickle Cell can be in there for so long, for example: my dad has spent weeks in there and missed a lot of time at education because of this. As the care varies, depending on this and how long you are in for, it is extremely difficult. The worst experiences my dad has had have been being administered too much drugs or not being administered enough drugs.”

The state of inequality

At the heart of health inequality sickle cell anaemia’s large effect on minorities skews its perception and creates a “lack of health professionals who are educated on Sickle Cell and Trait” which means “if there are no specific nurses present at the hospital, the treatment can be inadequate for people with Sickle Cell.” Beyond this sickle cell presents large financial barriers as individuals require so many prescriptive medications which grow increasingly costly over time “it is upsetting that for a lifelong illness, you must pay to be pain-free and survive.”

In order to overcome stereotypes and misunderstanding of sickle cell anaemia, greater funding is required into research and education on the disease, anything less is to perpetuate racist, invalidating and harmful notions of the disease. We all deserve to be understood and catered for.

(At the time of writing this article the drug Voxelotor which the NHS announced would be rolled out to around 4000 suffering with sickle cell, has been withdrawn and discontinued by Pfizer for reasons that can be found on their website).