WHAT IS THE SICKE CELL DISORDER?

Sickle Cell is an ‘Anaemia’, there are many types of anaemias the more commonly known are General Anaemia, Thalassaemia, Leukaemia, Hypoglycaemia and the most common and fastest growing worldwide is Sickle Cell Anaemia.

 

Sickle Cell Anaemia is recognised as one of ‘if not’ the most serious of anaemias known. It is a life-threatening inherited blood disorder where the red blood cells, which carry oxygen around the body, develop abnormally causing the extreme deprivation of Iron Fluorine, which can cause over 20 other diseases and conditions to manifest.

 

Under the heading of Sickle Cell Anaemia there are many types, such as Haemoglobin SS Disease, Haemoglobin SC Disease, Haemoglobin SB+ Beta Thalassaemia, Beta-Zero Thalassaemia, Haemoglobin D-Punjab plus others.

 

These Sickle Cell disorders affects mainly people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin, however it is manifest in people of all origin.

What causes sickle cell anaemia?

Sickle cell anaemia is caused by a mutation (an abnormal change) in the gene that instructs the body to produce haemoglobin, which is the red protein responsible for transporting oxygen in the blood cells.

 

The sickle cell gene is inherited. To have sickle cell anaemia you would have inherited the defective gene from both parents. If you only inherit the gene from one parent, you have what is known as the Sickle Cell Trait. It is likely that your blood will contain some sickled cells, but you will be able to produce normal haemoglobin and will not usually experience extreme symptoms.

 

However, you would be a carrier of sickle cell anaemia therefore can pass the abnormal gene through to your children. In England, the official statistics remain recorded that about 250,000 people are thought to have the Sickle Cell Trait, with those of African-Caribbean origin primarily affected.

If a couple, both with Sickle Cell Trait are pregnant, there is a one in four chance in each pregnancy, that the child will be born with a form of Sickle Cell Anaemia. In the UK, all forms of the Sickle Cell disorders are most commonly seen in persons of African & Caribbean descent

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WHAT IS THE SICKE CELL DISORDER? Continued.

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Sickle-shaped cells contain defective haemoglobin, Hb for short, ‘which is the Iron-rich protein that enables red blood cells to carry oxygen from your lungs to the rest of the body’. The abnormal cells are devoid of oxygen and are also unable to move around as easily as normal shaped cells and block blood vessels, resulting with tissue and organ damage and episodes of severe pain. Such episodes are known as a ‘Sickle Cell Crisis’ or a vaso-occlusive crisis. They can last from a few minutes to several months, although on average most last from around five to fourteen days.

 

The lifespan of a normal red blood cell is 120 days, however, the abnormal Sickled blood cells have a shorter lifespan as they are not replaced as quickly as normal blood cells and on average sickled cells live for just 20 days.

Complications

This serious condition amongst other ailments manifests symptoms such (but not limited to):

 

Iron overload, jaundice, swollen joints, migraines, breathing difficulties, strokes, permanent damage causing removal of spleen, reduced bone density, ulcers, nocturnal enuresis (bed wetting), blindness, Epistaxis (frequent nose bleeds), priapism, (painful swollen and lumpy penis causing impotency) also pneumonia, organ failure, weakened immune system, gallstones and slow or sudden death, and many more.

 

It is quite common for patients to live with extreme depression, anxiety and have suicidal thoughts.

 

A Sickle Cell Crisis can be triggered completely unexpectedly; however, it is also triggered by cold damp temperatures, stress, dehydration, infection & bruising.

Patients are advised to avoid situations that may cause these symptoms to arise.

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