What do you really know about Sickle Cell Disease?

Ahead of Sickle Cell Awareness Day on Friday 19th June 2020 (don’t forget to wear red to commemorate the day), I wanted to spread some awareness of some facts and general information about Sickle Cell Disease, for people with or without the disease. Knowing key information about Sickle Cell Disease is the first step to spreading awareness of this complex disease, but will also help to enhance your understanding on how it really affects those that live with the disease. So, read on, absorb, comment and don’t forget to share…

Sickle Cell Disease (SCD) is a genetic, serious, lifelong health condition that affects the red blood cells. Those with SCD produce sickle red blood cells (which are shaped like a crescent moon or kidney bean), while healthy (normal) red blood cells are round in shape. People with SCD do not have enough healthy red blood cells to transport oxygen around the body and their sickle red blood cells are quite hard and sticky, and often gets stuck in the blood vessels.

Healthy red blood cells live for approximately 120 days in the body before it needs to be replaced. However, sickle red blood cells live between 10-20 days in the body, which as a result, leaves a constant lack of supply of red blood cells (anaemia) in our bodies. This is why people with SCD are often anaemic, meaning they are often tired and drained, and need a lot more rest than those without SCD.

As SCD is genetic, those with SCD is caused by inheriting the sickle cell gene from both of your parents. The genes involved in SCD control the production of a protein in red blood cells called haemoglobin. Haemoglobin binds oxygen in the lungs and delivers it to the peripheral tissues, such as the liver and the muscles. Parents tend not to have SCD themselves but are only carriers of the sickle cell gene. If both parents are sickle cell carriers, there’s a: 1 in 4 chance that each child will not inherit any sickle cell genes; 1 in 2 chance that each child will inherit a copy of the sickle cell gene from one parent and be a carrier of sickle cell; and a 1 in 4 chance they will inherit copies of the sickle cell genes from both parents and have SCD.

There are different forms of sickle cell disorders, which all affect the haemoglobin. The most common types of the disorder are called: Sickle Cell Anaemia (HbSS) – this is when two sickle cell genes are inherited from his/her parents [one from each parent] – this is the most severe form of the disease; Haemoglobin Sickle C Disease (HbSC) – this is when someone inherits a sickle cell gene from one parent and from the other parent, s/he inherits an abnormal haemoglobin called “C” – this is a milder form of the disease; and Haemoglobin Beta Thalassemia (HbS Beta-Thalassemia) – when someone inherits one sickle cell gene from one parent and one gene of beta-thalassemia (another form of anaemia) from the other parent. [I will talk more about the different forms of the sickle cell disorders in a different blog posting…keep an eye out for it!]

SCD is one of the most common, serious genetic conditions worldwide, with it being particularly rife in the Middle East, Mediterranean regions, South-East Asia and sub-Saharan Africa, especially in Nigeria. Approximately, 250 million people worldwide carry the gene responsible for SCD and other haemoglobin diseases. About 5-7% of the global population carries the abnormal haemoglobin gene, with the most dominant form being inherited, is SCD – the most severe form of the sickle cell disorders. Worldwide, there are approximately 300,000 babies born every year with the more severe form of the disease – mainly from low and middle income countries. Additionally, approximately 5% of the world’s population are healthier carriers of the SCD gene or thalassemia. In some regions, this figure skyrockets to 25%, that are carriers of the gene worldwide. Interestingly, sickle cell disorders are more prevalent in tropical regions, however with population migration, this has spread these diseases to most countries in the world.

In America, SCD occurs in approximately 1 in every 365 Black or African-American births. Roughly, 1 in 13 Black or African-American babies is born with sickle cell trait (the carrier gene). There are approximately 100,000 Americans living with SCD, with 10% of the population at risk of SCD. Additionally, SCD is a major public health concern in America, as research has shown that over a four-year period, an average of 75,000 hospitalisations are due to SCD alone, costing approximately $475 million.

Similarly, SCD is now also one of the most common, serious genetic conditions in England, which affects1 in 2,000 babies. Approximately, 15,000 people in the UK have a sickle cell disorder, with approximately 350 babies born with SCD in the UK every year. Additionally, 1 in 76 babies that are born in the UK every year, carry sickle cell trait.

People with SCD have less access to comprehensive team care than people with genetic disorders such as Haemophilia (a condition in which the body’s ability to make blood clots is impaired) and Cystic Fibrosis.

Sickle cell disorders causes a lot of complications and illnesses within the body, which those living with sickle cell deal with on a daily basis. These include:

– Sickle Cell Retinopathy (eye complications, which affect those with HbSC in particular) – when sickle red blood cells become trapped within blood vessels and interrupt blood supply to the retina, and causes vision loss;

– Persistent forming of gallstones (due to excessive billrubin production as the body breaks down the red blood cells;

– Spleen organ damage (as well as impaired spleen functions);

– Poor blood supply to joints, leading to chronic pain, swelling and restricted mobility;

– Kidney damage;

– Liver impairment due to iron overload because of frequent blood transfusions;

– Mental Health illness, such as anxiety and depression, due to acute and chronic pain;

– Necrosis, due to the death of the bone due to poor blood supply;

– Osteonecrosis, which usually affects the femoral head of the hip, but can also affect the shoulder, elbow, wrist, knee and/or ankle;

– Multiple organ damage;

– Stroke;

– Brain haemorrhage (when the blood vessels rupture);

– Leg ulcers (due to the poor blood circulation to the skin);

– Jaundice (yellow tint to the eyes caused by high levels of bilirubin. Accompanying symptoms could also include abdominal pain, fever and/or fatigue);

– Delayed growth during childhood and delayed puberty;

– Bone and joint pain;

– Persistent and painful erection of the penis, which can last several hours and could lead to impotence;

– Serious lung condition called Acute Chest Syndrome, which can cause a fever, cough, chest pain, and breathing difficulties;

– Swelling of the spleen, which can cause shortness of breath, rapid heartbeat, abdominal (stomach) pain, swollen stomach and anaemia;

– Eyesight problems such as floaters (dots, lines or flashes in the eye), blurred or patchy vision, reduced night vision and could lead to sudden vision loss;

– High Blood Pressure (in the blood vessels that carry blood from the heart to the lungs);

– Kidney and/or urinary problems, including blood in the urine and bedwetting;

– Learning difficulties such as memory problems and a lack of concentration, as well as behavioural, emotional and academic difficulties;

– Sleep disturbances and/or anaemia;

– Brain injury.

There are many different symptoms and accompanying illnesses/conditions that come with having SCD, and as no two people with SCD are the same nor will they have the same symptoms, the most important thing to take away is to know your condition, watch out for symptoms and seek treatment as quickly as possible. If you don’t have SCD but have friends and/or family who do, the important thing to take away is to understand how SCD works and the symptoms that may arise. Be there for your loved ones living with SCD and be their crutch when they are weak and act fast, in order to reduce the pain, suffering and frustration that your loved one(s) may be feeling.

For now though – keep well and keep safe.

Peace and love! X

Published by Iliana Ike

Passionate creative who likes to explore different art forms for expression, awareness and healing

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