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Shirin
MyGirlsBlood Ambassador - Iran

Shirin has written an article that was published in a Hemophilia Journal.

Supporting Women Affected by Congenital Bleeding Disorders in Iran

Iran has a considerable number of women with inherited bleeding disorders. They are predominantly threatened by serious gynaecological or obstetric problems at the most vulnerable stage in their lives. Affected women still do not receive the care they deserve. On the other hand, our society's perception of a woman who carries or suffers from a hereditary disorder, profoundly affects her psychosocial life, and imposes even further stress.

In order to address the needs of women who suffer from abnormal bleeding, and the profound impact this can have upon various aspects of their lives, the first Iranian Comprehensive Haemophilia Care Centre has adopted a number of strategies in a multi-disciplinary setting. A significant number of cases of women with hereditary bleeding disorders, including von Willebrand's disease, platelet dysfunction, rare autosomal coagulation deficiencies, and carriers of the haemophilia gene have been identified and characterised. The prevalence of bleeding disorders in Iranian women is shown in table 1.

Consanguineous marriage

Not surprisingly, most of these rare diseases, particularly those which are inherited in a classical Mendelian fashion, stem from first-cousin marriage, where both parents have inherited identical gene copies. While first-cousin marriage is usually viewed with opprobrium in most Western countries, it is favoured in Eastern communities, including Iran.

There are many socio-economic reasons behind consanguineous marriage that makes this attitude a social norm, including;

• In patriarchal communities, women have to leave their family to enter their husband's family. A woman is more comfortable when her mother-in-law is her aunt, and her parents are able to stay closer to her.

• There is easy acceptance of a genetic disorder or carrier status among close relatives where awareness of disease has been present in the family since early childhood.
• In societies with a significant discrepancy between low- and high-income families, consanguineous marriage removes all social and religious differences.
• Conservative societies that practice segregation between sexes and where establishing premarital relationships between youths is restricted, young people can get to know each other more easily if they are related.
• Economic strains are less onerous within an extended family, rather than the nuclear family, where the newlywed couple have to seek separate accommodation.
• Rising mistrust and insecurity in relationships, and the general paranoia engendered in a modern, competitive environment, lead young people to return to their traditions and fosters marriage with a relative.

It must be pointed out that education in higher institutions of learning has led to a significant shift in gender roles in Iran. This shift is emerging most particularly in urban regions, where women have the opportunity to work and to be financially independent. However, disparities in rural areas are great, and several traditional barriers affect the empowerment of women in this setting.

Challenges of care for female bleeders

Iranian women with bleeding disorders are deeply affected by the hereditary nature of their disease. They often have difficulty getting married; they keep their disorder or carrier status secret, and they are profoundly distressed emotionally. Most significantly, prevailing moral values in our culture prevent them from talking about their heavy menstrual bleeding, and even in a clinical setting, they are reluctant to discuss such intimate matters, unless they feel confident in the confidentiality of their patient-physician relationship.

Lack of awareness among gynaecologists and obstetricians regarding congenital bleeding disorders and inadequate diagnostic facilities, or the dearth of sufficient therapeutic materials, adds to the great importance of supporting this vulnerable group.

Strategies to promote health-care for female bleeders

Accurate diagnosis and classification of congenital bleeding disorders has been at the forefront of management for women with bleeding disorders. Table 1 reflects the wide spectrum of these diseases and the invaluable results which have been collected and recorded on the ICHCC database, which is accessible at anytime. The coagulation laboratory at the ICHCC has been in close collaboration with our genetics laboratory, in order to confirm the accuracy of results. This is especially important in the identification and classification of the different types of vWD. Moreover, determination of the homozygous state for a female is only possible in the genetics laboratory, which plays a crucial role in genetic counseling.

In order to educate the medical community and to raise awareness among gynaecologists, haematologists and nurses, various educational seminars have been organised. There can be no doubt that the participation of a well-known specialist from abroad, or from the World Federation of Haemophilia, adds to the success of such seminars.

Outreach programmes for teenage girls at school have been implemented. These programmes have been carried out during the National Health Week held at girls' middle schools (between the ages of 12 to 15), by distributing a simple questionnaire and informative booklets. The extra-curricular education of teenage girls at school, about normal and abnormal menstrual bleeding, undertaken by ICHCC physicians and social workers, has been extremely popular and successful.

Bleeding disorders may cause serious complications, including death during childbirth and major surgery, or following injuries. In order to defray the costs which may be involved in dealing with these emergencies, a special charitable fund has been created, which covers the unforeseen expenses of hospitalization and treatment in such cases.

Conclusion

Women with bleeding disorders are integral members of the general community, and they cannot be treated separately. The health of women is essential for maintaining the stability of families, and promoting health-care for women, promotes the health of the entire community.

In many societies, there are additional barriers rooted in socio-cultural factors, which add to the challenges faced by women with bleeding disorders. If we are truly trying to define the best quality care for women with bleeding disorders, we have to know how people in different communities think, and the way people interact and view life. Without thoughtful policies and consideration of our moral obligations towards other human beings, important opportunities may be denied to many individuals. Providing high-quality health services for female bleeders requires perseverance, and the sustained support of evidence-based programmes.