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Sickle Cell disorders

What are the symptoms?

Symptoms are rarely apparent before the age of three months due to the continuing effect of fetal (baby) haemoglobin. The most common symptoms are episodic pain in the bones, joints, abdomen and other parts of the body and these are known as "painful crises." They may be precipitated by cold, dehydration, or infections. Other problems associated with the condition may affect the spleen or cause jaundice, strokes, blood in the urine, leg ulcers, problems with the hips and/or shoulders, eye problems, lung problems, priapism (an abnormal, sometimes persistent and often painful erection), enuresis (incontinence of urine) and delayed puberty.

If strokes occur, they usually do so during childhood and they may be recurrent. If a child has had a stroke, treatment with regular blood transfusions may reduce the chance of a further stroke. However, if any person has regular blood transfusions, they will also need regular treatment to avoid iron overload from the blood transfusions. This is because iron overload can cause considerable harm if it is allowed to develop.

The painful crisis can be extremely variable. First, it may occur frequently, or it may occur only rarely. Second, the severity of the pain can vary a lot from one episode to another and from one person to another.

View Background Background  |  How is it diagnosed? View How is it diagnosed?

Medical text written August 2002 by Dr A Stephens. Last updated August 2005 by Dr A Stephens, Consultant Haematologist, King's College Hospital, London, UK.

 

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