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Are there any circumstances where blood transfusion is considered necessary as a matter of course?
Yes. If a general anaesthetic is required, let's say for a caesarean section, then blood transfusion will be given, usually beforehand unless the urgency for the operation means this is impractical. In such a case, alternative forms of anaesthesia (such as a spinal) may be considered and adopted.
What preparations will be necessary for labour and delivery?
For SCD sufferers, it is imperative that any conditions which could precipitate a crisis are avoided. if the person falls ill, dehydration can be prevented by ensuring adequate fluid intake, usually by the intravenous route. Screening for possible urinary tract infection is normally done regularly, especially towards the end of pregnancy. It will need to be done at the onset of labour as well.
Labour itself will be managed actively to ensure that there is no maternal distress or exhaustion and that it is not prolonged. Blood will always be available, in case transfusion becomes necessary.
What about those women with sickle cell trait in labour?
While no special measures are normally required for these women during pregnancy, the story is different when it comes to labour and delivery. Virtually all experts agree that these women should be managed actively along the lines of SCD sufferers (see above).
Experience has shown that complications and occasionally unexpected (very rare) fatalities have occurred in women with sickle cell trait. Investigations in such cases tend to show that severe dehydration and/or hypoxia (oxygen deficiency) are the precipitating factors. Labour and delivery should therefore be regarded as extreme conditions which could push sickle cell trait women beyond the barrier that normally makes them insusceptible to "sickling".
What about monitoring of the fetus during labour?
Continuous electronic fetal heart monitoring will take place for sickle cell disease sufferers.
Will there be any special measures in the period immediately after delivery?
Yes, for both SCD and sickle cell trait women, during the post-delivery period (especially the first 24 to 48 hours), the measures taken during labour will be maintained. These should include prevention of dehydration and hypoxia.
Simple measures which ensure adequate fluid intake and rest for the mother are normally sufficient. If an infection was being treated, this should be completed.
Who is at risk?
Sickle cell disease, like the other so-called haemoglobinopathies, is a genetic disease. The defective genes are inherited from parents. The condition cannot be acquired any other way.
If one parent passes on the relevant defective gene, the child will be a carrier with what is known as sickle cell trait. If the defective gene is inherited from both parents, the child ends up with a complete pair of the defective genes and will have the full-blown condition known as sickle cell disease (SCD).
Is there any ethnic propensity for this condition?
Different ethnic groups are known to be more prone to different genetic conditions by virtue of their evolutionary genetic inheritance. Just like cystic fibrosis is prevalent among northern Europeans and beta-thalassaemia among people of Mediterranean extraction, sickle cell disease is prevalent among people of black African origin.
The condition is also, found among people from Saudi Arabia, India and the Mediterranean areas. It is, however, less prevalent among these peoples. Inter-ethnic marriages mean that the possibility of finding any of these conditions in a member of another ethnic group may be improbable but is far from impossible. In fact, the increasing trend of global population mobility, inter-ethnic marriages and partnerships mean the defective gene carrier status cannot be reliably predicted on ethnicity alone.
Last update: November 29, 2012