guidebook. - Fanconi Anemia Research Fund

268
Fanconi Anemia: Guidelines for Diagnosis and Management
in any population remain to be determined. In addition,
the incidence of other tumors, including gastrointestinal
and breast cancers in particular, may be excessive.
The evolving data will need to be carefully evaluated to
develop appropriate monitoring (and treatment) strategies
that respect the desire to minimize radiation exposure
and treatment-related toxicity.
Gynecologic and Fertility Issues in the
Adult FA Patient
Discussions of expectations regarding fertility and lifeexpectancy
are obviously quite different with an adult
patient than with a child and his/her parents. Adult FA
women experience early menopause, need high-risk
management of pregnancies, and have an increased risk
of gynecologic malignancies (see Chapter 6). Adult FA
men are generally azoospermic and infertile. Advances
in assisted reproduction techniques have led to new
possibilities for the prevention and treatment of infertility.
Early referral to a fertility clinic may be warranted.
These issues may be particularly challenging to address
with newly diagnosed patients.
Diabetes and Vascular Health
While the data on glucose intolerance are becoming
better described in children with FA (see Chapter 7), the
natural history in FA adults is unknown. In addition, the
effects of oral hypoglycemics developed for the general
population will need to be evaluated in this patient
subgroup.
The interaction of FA with vascular disease of aging
is unknown. Long-term follow up studies of children
surviving transplant, as well as other cancer treatments,
suggest that vascular and cardiac disease incidence will