Sickle cell disease (SCD) can affect anyone - it is a serious disease and a lifelong health condition.
Approximately 15,000 people in the UK have sickle cell disorder, according to the Sickle Cell Society. In America, it is the most common inherited blood disorder.
It is especially common in people of African and Caribbean backgrounds, with the most serious type called sickle cell anaemia.
People with the SCD suffer painful episodes which can last up to a week, plus anaemia which causes tiredness and shortness of the breath.
They also live with an increased risk of serious infections due to a non-fuctioning spleen.
Legendary jazz musician Miles Davis lived with the SCD.
Actress Victoria Ekanoye of Coronation Street and X Factor has talked openly about her struggles with sickle cell.
What is sickle cell disease?
Sickle cell disease (SCD) is an inherited blood disorder where the red blood cells develop abnormally, forming a rigid crescent shape that is unable to supply adequate stores of oxygen around the body.
Sickle-shaped cells contain defective haemoglobin (Hb) – an iron-rich protein that enables red blood cells to carry oxygen. Further, the abnormal cells have a hard time moving around in the bloodstream.
One "very serious" health complication due to SCD – as ascertained by the experts at the NHS – is neurological damage. This can lead to cerebrovascular accidents, known as a stroke, where the blood supply to part of the brain is cut off.
Once a stroke has occurred, further life-threatening episodes are likely.
What causes SCD?
A gene mutation is the cause of sickle cell disease.
In order for a diagnosis to be made, you would need to have inherited the condition from both your mother and father.
Otherwise, if you only inherit the faulty gene from one parent, you are known as a sickle cell carrier.
Sickle cell carriers will have some abnormal cells in their bloodstream, but there will be enough healthy haemoglobin to eradicate symptoms of the condition.
Be aware that if you are a sickle cell carrier, you may pass the gene on to your children.
Speaking to your doctor for genetic screening is a possibility before deciding on having a child.
"If you are your partner are both affected by SCD there are services that you can be referred to in order to reduce the risk of conceiving a child with a sickle cell disorder," the health experts at the NHS reassured.
Why are people with SCD in danger of infections?
People with SCD are also more vulnerable to infections, as they don't have a functioning spleen.
The spleen is a fist-sized organ situated in the upper left side of the abdomen – next to the stomach, behind the left ribs.
When a person with SCD gets an infection, the consequences can potentially be "fatal".
Such an event usually requires hospital admission for intensive antibiotic treatment.
Other health complications of SCD might include:
- Pulmonary hypertension
- Acute and chronic pain
- Leg ulcers
- Acute chest syndrome
- Eye complications.
The symptoms and process of sickle cell anaemia is said to majorly affect a person's quality of life.
