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Pre-conception - advice and management - Management
How do I manage a woman with thalassaemia who wishes to become pregnant?
- Refer all women with thalassaemia to a haematologist for assessment.
- Women who are carriers:
- Seek advice from a haematologist or haemoglobinopathy counsellor (if available) for women who are carriers and have an unusual variant or need further investigation.
- Ensure that the woman's partner has been tested (see Advice when risk of genetic disorder).
- Discuss the standard pre-conception measures (see What to check in all women).
- Women with thalassaemia should receive folic acid 5 mg daily throughout the pregnancy.
- There is also some evidence to suggest that women who are carriers should receive folic acid 5 mg daily throughout the pregnancy.
Basis for recommendation
- Alpha-thalassaemia carriers: the woman may become anaemic particularly if she is a carrier of two defective genes [Letsky, 1999].
- 3-alpha-thalassaemia (HbH disease): the woman will have chronic haemolytic anaemia and may require transfusion [Letsky, 1999].
- Homozygous alpha-thalassaemia (Bart's haemoglobin hydrops syndrome): pregnancy is associated with severe, sometimes life-threatening pre-eclampsia. Vaginal deliveries are often associated with obstetric complications resulting from the large fetus, bulky placenta, and often the small stature of the mother (usually of Far Eastern origin) [Letsky, 1999].
- Beta-thalassaemia minor (symptomless carriers): if iron stores are depleted, the woman may need oral iron supplements during pregnancy. Before giving iron supplements it is important to confirm with the local haematologist that the woman is truly iron-deficient. A low mean cell volume (which is a feature of beta-thalassaemia) does not usually mean the woman is iron deficient [Letsky, 1999]. Primary care practitioners should request a serum ferritin for these women.
- Beta-thalassaemia major (homozygous beta-thalassaemia): pregnancy is rare in these women and is likely to have serious complications [Letsky, 1999]. These women need specialist reproductive endocrinological referral if they wish to become pregnant.
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