A form of anaemia, characterised by sickle-shaped red blood
cells
Sickle cell anaemia is a serious, long-term, incurable blood
disease resulting from a genetic mutation in a haemoglobin
molecule (HbS), that is transmitted from parent to child. These
abnormal cells have the tendency to clump together, giving them
a distinctive sickle shape, and are also easily destroyed as
they enter the smaller blood vessels in the body - producing the
anaemia.
SymptomsSickle cell anaemia is a variable disease -
while some sufferers are only midly affected, others may
experience considerable discomfort when the sickle cells block
the capillaries, thus impeding the blood supply. Common symptoms
often include, joint pain, fever, fatigue, general weakness and
spleen enlargement. Carriers of this disease often experience no
symptoms at all, and symptoms are seldom seen before 6 months of
age. Growth and puberty may also be delayed.
Incidence
The disease is most common among people of African descent,
and those from the Mediterranean, Turkey, India and the Middle
East. A child has a 1 in 4 chance of developing the disease and
a 1 in 2 chance of being a carrier if both parents carry the
trait.
Potential Complications
Long-term complications are common, and include:
- multiple infections
- congestive heart failure
- eye disorders
- swelling and breakdown of nerves
- renal failure
- death The average life span is currently about 40 years.
Treatment
- Analgesics for pain
- Flu vaccines against flu and pneumonia
- Iron supplements
- Sufficient rest and exercise
- General monitoring for avoidance of dehydration
- Counselling and support for adaption to disease
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