Sickle cell disease is an inherited disorder of haemoglobin, the protein
in red blood cells that carries oxygen. In this condition, an abnormal
haemoglobin S from one parent is combined with another abnormal
haemoglobin from the other parent. Haemoglobin S inherited from both
parents (genotype HbSS), described as sickle cell anaemia is the most
When oxygen tension is low, haemoglobin S crystallizes and makes the red
blood cells sickle shaped. Sickling reduces red blood cell capacity to
manoeuvre through very small blood vessels causing vascular blockage and
early destruction of red cells. The breakdown of red blood cells and
massive pooling of damaged red blood cells in the liver and spleen cause
anaemia. Acute illnesses include painful crises, pulmonary embolism,
acute chest syndrome and congestive cardiac failure. Therefore, pregnant
women with sickle cell disease require careful management.
Depending on the institutional policy, blood transfusion can be given at
intervals to a pregnant HbSS woman with relatively few or no symptoms to
improve the oxygen carrying capacity of blood by increasing haemoglobin
blood concentration and lowering haemoglobin S levels; or only when
indicated by the development of medical or pregnancy complications.
Giving blood at frequent intervals carries the risks of blood-borne
infections and excessive levels of iron.
This review set out to determine whether giving blood at intervals
before serious complications occur compared with giving blood only when
medically indicated makes a difference to the health of the mother and
her baby. The review authors included two controlled trials that
randomised 98 women with sickle cell anaemia (haemoglobin SS) before 28
weeks of gestation to one of the two blood transfusion policies. The two
trials were of low quality. One trial (72 women) indicated no difference
in severe ill health and death of the mother or newborn. There was no
difference in the risk of delayed blood transfusion reaction. The two
trials suggested giving blood at frequent intervals marginally reduced
the risk of pain crisis, with a large degree of uncertainty about the
size of the effect, compared with giving blood only when medically
indicated. Blood transfusion was delivered at a ratio of four or five to
one for prophylactic versus selective blood transfusion, respectively.
Overall, available evidence on this subject is insufficient to advocate
for a change in clinical practice and policy.
Okusanya, B.O.; Oladapo, O.T. Prophylactic versus selective blood transfusion for sickle cell disease in pregnancy. Cochrane Database of Systematic Reviews (2013) Issue 12, Art. No.: CD010378. [DOI: 10.1002/14651858.CD010378.pub2]
Prophylactic versus selective blood transfusion for sickle cell disease in pregnancy