Sickle Cell Disorder is the name given to an inherited genetic blood condition that affects what is known as haemoglobin within the blood.   

Sickle Cell affects people mainly from Africa, the Caribbean, Asia, the Mediterranean, Middle East and Far Eastern countries.  About 13,000 people in the UK are affected with the disorder.

 

The disorder results from an abnormal production of haemoglobin, a substance found in red blood cells, which gives blood its colour and carries oxygen around the body.

 

The abnormal haemoglobin changes its round shape to a ‘sickle shape’ that resembles a half moon shape when a red blood cell gives up oxygen.  Whereas a normal blood cell is rounded and moves through the body easily, a ‘sickle shape’ cell can cause blockage of blood flow.  This leads to a number of complications including:

  • Anaemia
  • Severe episodes of pain, known as a ‘crisis’
  • Infections
  • Organ damage

  

 

  

  

 Some individuals may be carriers of the disorder but are not directly affected by Sickle Cell.  These individuals are described as having Sickle Cell Trait.By itself, Sickle Cell Trait is a healthy state and will not make a person feel ill, although if that person chooses to have a family a child could inherit Sickle Cell disorder depending on the other parent’s haemoglobin type.

The three main disorder types include:

 

  • Sickle Cell anaemia (SS)
  • Haemoglobin (SC) disorder
  • Sickle Beta Thalassaemia (Sßtha)

 

Some individuals may be carriers of the disorder but are not directly affected by Sickle Cell.  These individuals are described as having Sickle Cell Trait.By itself, Sickle Cell Trait is a healthy state and will not make a person feel ill, although if that person chooses to have a family a child could inherit Sickle Cell disorder depending on the other parent’s haemoglobin type. If both parents are Sickle Cell Trait there is a 25% likelihood of their child inheriting Sickle Cell disorder.  The outcome could be one of the following:

 

  • The child inherits a normal haemoglobin type from both parents and is not affected 
  • The child inherits a normal haemoglobin type from one parent and an abnormal sickle haemoglobin type from the other, becoming a carrier or a Trait
  • The child inherits an abnormal haemoglobin type from both parents, causing Sickle Cell disorder

 

The normal haemoglobin type is labelled as Haemoglobin AA, whereas the carrier or Trait types are labelled as:

 

  • Haemoglobin AC
  • Haemoglobin A Beta Thalassaemia
  • Haemoglobin AS

 

 

Sickle Cell Trait is carried by:

  • 1 in 10 Blacks
  • 1 in 20 to 50 Asians
  • 1 in 1000 Caucasians

 

 

People with Sickle Cell Disorder are born with the condition.  It is not contagious and can only be inherited. 

 

Sickle Cell Trait is not infectious and does not change to Sickle Cell Disorder.

 

Whilst there is no known cure for the disorder, a range of treatments and therapies are available to manage the condition.

 

A simple blood test known as ‘electrophoresis’ is available through your GP and can tell you whether a person has Sickle Cell Trait or the Sickle Cell Disorder.  Please contact us or view our list of frequently asked questions for further information.

 

Related links:

 

http://www.sicklecellsociety.org/