Living with Sickle Cell & Caring for Black Skin
In the summer of 2024, Supacell landed on our TV screens, firmly putting the blood disease sickle cell in the conversation. The cast of all-Black heroes are transformed by the disease which gives them superpowers. Whilst a great watch, in real life the disease can be more tricky to deal with. Globally eight million people have sickle cell; in the UK 17,000 people experience the disease and life expectancy can be reduced by up to 30 years than the general population. Although sickle cell disease equally affects men and women with no correlation to age, there’s a clear ethnic predisposition, primarily affecting those of African descent.
“Before Supacell, ask anyone about sickle cell and one out of five people know about it. It’s crazy how unknown it is, especially for a disease that affects so many people.”
What is sickle cell?
Giving the condition its name, sickle cell disease is a genetic blood disorder where red blood cells take on an abnormal, crescent-like ‘sickle’ shape. This mutation affects the blood’s ability to carry oxygen efficiently and can also block blood vessels. Whilst treatment can help manage the symptoms of sickle cell disease, it’s a lifelong condition that you are born with.
Red blood cells with the crescent ‘sickle’ shape
Dr Nellie Adjaye MBE, Medical Adviser to the Sickle Cell Society explains that “sickle cell is caused by an inherited genetic mutation. To understand it, we need to know about haemoglobin, which makes our blood red.” She continues, “Haemoglobin is made of ‘haem,’ the iron component, and four chains of amino acids: two alpha and two non-alpha (or beta). In sickle cell, the non-alpha chain is altered. If one parent has the mutated beta chain, you carry part of the sickle cell gene. If both parents pass on the gene, there’s a one-in-four chance their child will have sickle cell disease.”
Dr Adjaye adds, “This is why it’s important to get screened before having children, as knowing your status helps you make informed decisions when choosing a partner. You can ask your GP for a blood test to check if you carry the gene.”
The effects of sickle cell on the body
The impact of sickle cell disease can begin in early childhood, with symptoms including painful episodes called ‘sickle cell crises’, an increased risk of infections, and chronic anaemia due to the rapid breakdown of the red blood cells, leading to fatigue and jaundice.
Affecting any part of the body, the painful crises are a hallmark of the condition, occurring when sickle cells block the blood flow in small vessels. Dr Adjaye clarifies, “As a result, the part of the body that is not well-oxygenated can become very painful. This is called a ‘crisis’ and affects the bones and joints, sometimes causing chest infections, back pain, headaches, and occasionally strokes. Those who have had strokes are sometimes treated with exchange transfusions, which is why we encourage Black people to give blood, as there are certain elements in the blood that are unique to the Black community.” Charities such as the ACLT work to improve the chances of finding blood and donor matches for individuals in the African and Caribbean community, for this very reason.
How does sickle cell affect the skin?
As with many health conditions, sickle cell can have a negative impact on the skin. Consultant Dermatologist Dr Derrick Phillips, tells us that “the skin manifestations of sickle cell disease are common and can be a source of disability and isolation for those affected. The changes that precipitate sickle cell crises can reduce blood flow to the skin resulting in the development of painful ulcers. These ulcers are most common on the legs, as they are prone to trauma. They can take many months to heal and leave dark marks and scars in their wake.”
Those with sickle cell also need to take extra care to avoid infections. “Adults with sickle cell disease are susceptible to infections from unusual organisms as a result of damage to the spleen. This can manifest as a rash that fails to improve with conventional treatments or infected ulcers.” Dr Phillips adds. The anaemia that comes with sickle cell can also lead to itchy, dry skin, due to poor circulation and low oxygen levels in the blood.
Beyond the disease itself, there can also be side effects from the prescribed treatments. “Medications like Hydroxyurea which are commonly used to treat sickle cell disease, have been associated with nail changes, eczema and loss of pigmentation.”
How should you care for your skin?
Respecting and reinforcing your skin barrier is crucial - it’s responsible for locking in hydration and protecting you from external irritants and aggressors like pollution and UV rays. “People with sickle cell disease should ensure that their skin is well hydrated and moisturised at all times. This will reduce the risk of damage to the skin, ulceration and infection,” advises Dr Phillips. “I would recommend using oil or cream-based cleansers such as Uriage Xemose or CeraVe Foaming Oil Cleanser, and moisturisers rich in emollients such as shea butter, cocoa butter and ceramides like CeraVe Moisturising Cream or even the Advanced Repair Ointment, to repair the skin barrier and keep the skin moisturised.” To address scarring and dark marks left behind once an ulcer heals, Dr Phillips recommends KeloCote Silicone Scar Gel and Eucerin Anti-pigment Body Cream.
Itch relief products, such as E45 Itch Relief Gel, and soothing mists like La Roche-Posay Cicplast Spray can help relieve itchy skin associated with anaemia. The spray is particularly helpful for hard-to-reach areas and for when the skin is too sensitive to touch.
Soothing Skincare Essentials
What should you avoid?
As sickle cell increases your risk of infection, so it’s best to avoid invasive treatments that create wounds in the skin such as microneedling and chemical peels. “Energy-based devices such as lasers should be approached with caution to avoid injuring the skin and precipitating a crisis. It is best to consult with a dermatologist or your haematologist before undergoing professional treatments.” recommends Dr Phillips. For the same reason, he also suggests avoiding harsh exfoliants and physical scrubs.
In addition to skincare treatments, it’s important to be mindful of heat. “Avoid hot showers and baths, which will damage the skin barrier by stripping away essential oils from the outer layer of the skin.” Whilst warmth will be comforting, particularly during painful crises, keeping water to a tolerable, lukewarm temperature rather than hot will help mitigate this.