Women with sickle cell not given adequate information about reproductive health

A knowledge gap may mean that women and girls living with sickle cell disease are not getting the best reproductive healthcare.

As treatment for sickle cell disease has improved, so has the expected lifespan of patients. This means that more women with the disease are reaching childbearing age. A new report from an expert panel convened by the US Centres for Disease Control and Prevention (the CDC) has raised questions about how medicine has failed to address sickle cell disease's impact on female reproductive health.

'There is an immediate need to address long-standing questions about the reproductive health of girls and women with sickle cell disease,' said the panel, whose report was published in the British Journal of Haematology. 'There are many sickle cell disease-related reproductive risks and uncertainties across girls' and women's reproductive life span, with particularly outstanding concerns about menstruation, contraception, fertility and pregnancy.'

Sickle cell disease is a genetic condition where a change in the beta-globin gene causes a change in the protein that carries oxygen inside red blood cells. As a result, the cells carry oxygen less efficiently and can take on a 'sickle' shape, making them more likely to block blood vessels.

Girls and women with sickle cell disease often experience sickle cell disease-related pain during menstruation and are at risk of blood-clotting complications if using estrogen-containing contraception. Further, while sickle cell disease does not rule out the possibility of having biological children, the process of preconception counselling and/or partner testing can be difficult to navigate.

Some women with sickle cell disease will require a stem cell transplant, which can lead to infertility, and there is some evidence that women with the disease can have accelerated decline in ovarian reserve, or earlier onset of menopause, possibly linked to treatments. These are areas that the panel conclude need more research, and also reasons that women with sickle cell disease might wish to look into fertility preservation treatments such as egg freezing.

The report also acknowledges that current limitations are exacerbated by existing societal conditions. Sickle cell disease disproportionately affects women of African and Caribbean heritage, and although the report focuses primarily on US health systems, where black women are less likely to have comprehensive health insurance, there is evidence that black women in the UK also have worse reproductive healthcare outcomes. A recent HFEA report showed that black women in the UK have lower IVF success rates (see BioNews 1089), and in 2020 the Royal College of Obstetricians and Gynaecologists set up a Race Equality Taskforce to tackle reproductive healthcare inequalities.