Reproductive behaviour of mothers of children with beta-thalassaemia major.

Thalassaemia is the most common monogenic autosomal hereditary disease worldwide. This questionnaire-based cross-sectional study looked at the reproductive behaviour of 156 mothers of children affected with beta-thalassaemia major (Cooley anaemia) in Fars province, southern Islamic Republic of Iran. Regardless of the number of affected children, the parents of children with Cooley anaemia had an average of 3 unaffected children, the same as the average for the provincial population. The findings indicate that reproductive compensation occurs in families with a child with Cooley anaemia in this province. Genetic counselling, either before marriage or after the birth of the first affected child, had a significant effect on lowering the number of children born.