Supacell: The importance of its success and how it has helped to raise awareness for sickle cell disease

Written by: Kimberly Amon-Lamptey

Millions of viewers around the world recently tuned in to watch Netflix’s new superhero drama ‘Supacell’. Supacell, the most recent project of Andrew Onwubolu MBE, depicts the story of five seemingly random individuals from South London who unexpectedly develop superpowers and must work together to avoid entrapment by an unethical organisation who seek to both control and exploit them. It has done exceptionally well with both its audience and critics, and has scored a whopping 100% on rotten tomatoes, which television and film lovers will know is no easy score to achieve.

Andrew Onwubolu MBE, more commonly known by his stage name ‘Rapman’ is a multi-faceted man known not only for his success in the music industry as a rapper and record producer, but also as a respected screenwriter and film director.

The show explores a number of different themes including knife crime, rehabilitation and abusive relationships but its recent success has helped to particularly raise awareness of sickle cell and how it can affect those living with the disease.

NHS England describes sickle disease as, “a group of inherited health conditions that affect the red blood cells,” with its most severe form being sickle cell anaemia. It is most common amongst individuals of African or Caribbean descent but can affect anyone. Sickle cell disease is caused by a gene that affects the development of haemoglobin in red blood cells which results in them being crescent (or “sickle”) shaped and rigid, as opposed to being disc-shaped and flexible as expected.

The genetic mutation responsible for the impaired haemoglobin in sickle cell is thought to have been an evolutionary response to malaria, as the parasite that causes malaria is halted by sickled red blood cells. Therefore, carriers of the sickle cell trait are deemed to be more resistant to malaria.

These sickled red blood cells have a significantly shorter lifespan than healthy red blood cells and can cause obstruction within blood vessels due to being unable to bend. This leads to a range of uncomfortable symptoms that can affect day to day life including (but not limited to): severe pain, fatigue, visionary issues, frequent infections, swelling of the hands and feet, and delayed growth or puberty in children and teenagers. Severe pain is often attributed to episodes knowns as sickle cell crises, which can last for days or weeks.

Sickle cell anaemia can sometimes lead to further, more severe, complications. Some of these include stroke, pulmonary hypertension, organ damage, complications with pregnancy and blindness.

Currently the only registered cure for sickle cell disease is a stem cell or bone marrow transplant. However, this option is not frequently utilised due to the risks involved (such as the risk of rejection) and for this reason current treatment aims to manage symptoms, control pain and improve quality of life. Treatment options include regular blood transfusions, painkillers, daily antibiotics, regular vaccinations and hydroxycarbamide (an anti-metabolite that interferes with DNA synthesis and repair, which helps to reduce symptoms in sickle cell disease).

Research is continuously being conducted to develop novel therapeutic therapies for sickle cell to improve quality of life. NICE (National Institute for Health and Care Excellence) have very recently approved a new drug - Voxelotor (Oxbryta?). This once-a-day tablet may result in fewer hospital visits and decrease the requirement for blood transfusions. The MHRA (Medicines and Healthcare products Regulatory Agency) have recently authorised the world’s first gene therapy that aims to cure both sickle cell disease and transfusion-dependent β-thalassemia, in those over 12 years of age. According to the MHRA, “Casgevy is designed to work by editing the faulty gene in a patient’s bone marrow stem cells so that the body produces functioning haemoglobin.” Casgevy is still undergoing to clinical trials, but current results appear to be promising.

In order for a baby to have sickle cell anaemia, it must have inherited the sickle cell gene from both parents – which can occur if both parents are carriers of the sickle cell trait. In instances where only one copy of the gene is passed on to the child, the child is said to be a carrier. Carriers do not tend to have symptoms of the disease but should be aware that they can pass the gene on to their children.?

?It is always a positive thing when a prominent form of media highlights an important real-life issue. Rapman has done a stellar job of highlighting a prominent issue in his community – especially for those who may be unaware of the disease and its implications. With sickle cell awareness month on the horizon (September) make an effort to discuss sickle cell with those in your nearest vicinity, as education on this topic is important not just for today, but for future generations.?

References

Sickle cell disease - NHS (www.nhs.uk)

Sickle cell anemia - Symptoms & causes - Mayo Clinic

Hydroxycarbamide - an overview | ScienceDirect Topics

NHS England ? NHS rolls out ‘life-changing’ treatment for thousands with sickle cell disease

MHRA authorises world-first gene therapy that aims to cure sickle-cell disease and transfusion-dependent β-thalassemia - GOV.UK (www.gov.uk)

History and Evolution of Sickle Cell Disease (sparksicklecellchange.com)