What is Sickle Cell?

Sickle cell is a disorder of the haemoglobin in the red blood cells. It is an inherited condition and is never contagious.

Normal haemoglobin cells are oval shaped that carries oxygen round the body. In someone with sickle cell disease, haemoglobin cells change into a sticky sickle shape, no longer able to pick up oxygen. These sticky sickle cells cause blockages in the small blood vessels which cause painful episodes, called Sickle Crisis.

A crisis can result in hospital admission, needing strong painkillers to control the pain.

Sickle cell can affect anyone but predominantly affects people from African and Caribbean backgrounds. Approximately 15,000 people in the UK have sickle cell disorder.

Approximately 350 babies are born with sickle cell disorder in the UK every year.

Children with sickle cell are at increased risk of strokes (the highest risk between the ages of 2 to 16).

People with sickle cell are at risk of complications which include strokes, acute chest syndrome, blindness, bone damage or priapism (persistent painful erection of the penis), swelling of the hands and feet, jaundice, brain damage, seizures, renal failure or mobility problems.

People may experience damage to vital organs such as the liver, heart, lungs, spleen or kidneys.  All these problems may result from complications of this disorder. In some cases it can be fatal.

Treatment for sickle cell mostly focuses on preventing complications and treating the pain.

Everyone is different. Symptoms vary from one individual to another.  When or if you have any symptoms they could last hours, days, weeks even months.

ONE THING IS THE SAME FOR EVERYONE: DRINK PLENTY OF WATER

Sickle Cell Trait

Sickle cell trait means you have inherited one unusual haemoglobin which is the haemoglobin S gene from one parent and one usual haemoglobin A gene from the other parent.  This is not an illness but means that you “carry” the gene and can pass it on to your children.

With two AS parents you have 25% chance in every pregnancy of having a baby with sickle cell (SS) haemoglobin.

Having trait doesn’t mean you develop sickle cell disorder.

Those that have sickle cell trait are at risk of having a child with sickle cell disorder, which can be life-threatening.  Having trait can cause anaemia.  On a day to day basis, trait does not make you ill, although it may cause health problems during situations where you might not get enough oxygen: during extreme sports such as mountaineering and deep sea diving.  Always inform the relevant bodies and declare you have sickle cell trait on all appropriate paperwork – for example :-

  • general anaesthetics like (eg at the dentist)
  • extreme athletic activity/intensive training
  • physical work without breaks
  • extreme stress, poor living/working conditions, mental illness etc.

Except in rare situations, a sickle cell carrier’s life is not at risk and someone with sickle cell trait can live a normal life.

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