Sexuality
During adolescence, puberty may be delayed by 1-3 years but sexual maturation occurs with normal development of secondary sexual characteristics. Postpubertal females with AS are fertile and pregnancy has been reported [1].
The main issues associated with sexual development in normal individuals have an important component as well in the lives of those with AS. The general approach to consideration of sexual issues in the AS is to recognize the sexual development will occur in a relatively normal physiologic manner for both young men and young women. Accordingly, issues of potential sexual abuse, normal masturbation behaviors, approach to contraception, and access to gynecological care are some of the issues of importance.
Sexual education is problematic but important to the child with AS. Educational themes might address emphasis on body parts and on private and public components, differences between boys and girls, issues of how babies are made, understandings regarding acceptable social behaviors, and continued reinforcements and instruction about relationship boundaries. Individuals with developmental handicaps are at increased risk for sexual assault and abuse, and parents should be alert to this and focus on prevention. Boundary issues are particularly difficult in some individuals with AS because of their outgoing personality and fondness of hugging or otherwise wanting to be close to others.
Since seizures are known to be affected by a menstrual periods, some individuals may require adjustment of seizure medications.
Providing medical contraception may be important in some situations. For females, the contraceptive methods are the ones employed in the normal population and may involve oral contraceptives or progesterone only injections (Depo-Provera shot given once every three months) and other methods. Currently there is no report of a male with Angelman reported to have conceived a child although it appears that that is theoretically possible.
Gynecological care is advisable by age 21 years to include a breast and pelvic examination. If a pelvic exam is not possible, ultrasound of pelvic organs may be indicated. Menstrual hygiene is a concern and education should stress identifying body parts, hygienic habits such as washing hands, and of course this must be done in the most basic way due to the cognitive impairments of those with AS.
1. Lossie AM and Driscoll DJ. Transmission of Angelman syndrome by an affected mother. Genetics in Medicine, 1999. 1(6): p. 262-266.