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Controversy Update

Say Goodbye to the “I” word

As CAH is the most common cause of genital ambiguity, CARES protested the exclusion of a CAH-specific advocate at this meeting (“CARES Letter to Consensus Meeting Receives International Support” Volume 5, Issue 1 Winter 2006). Within the protest letter, CARES also made recommendations about addressing issues related to: terminology, development of centers of excellence, and surgical management.

The “Consensus statement on management of intersex disorders” was published in Archives of Disease in Childhood in April 2006. Of particular interest to the CAH community are the recommendations made about nomenclature, multidisciplinary care and surgical management.

Nomenclature
The working group agreed with CARES and other patient advocacy groups that the word “intersex” and other arcane classifications are “…perceived as potentially pejorative by patients, and can be confusing to practitioners and parents alike.” Instead, the LWPES/ESPE group proposes the term “disorders of sex development” (DSD), defined by congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical.

Multidisciplinary Care

The consensus statement also stresses the need for a multidisciplinary approach when caring for a child with a DSD. According to the consensus statement, the team should include “paediatric subspecialists in endocrinology, surgery or urology or both, psychology/psychiatry, gynaecology, genetics, neonatology, and, if available, social work, nursing, and medical ethics.” In addition to recognizing that this team has a responsibility to educate other health care staff in the appropriate initial management of affected newborn infants and their families, the consensus statement also recognizes the important role support groups have in the delivery of care to DSD patients and their families.

Surgical Management
In alignment with the consensus statement on Congenital adrenal hyperplasia, this consensus statement asserts that only surgeons with expertise in the care of children and specific training in the surgery of DSD should undertake these surgeries. According members of LWPES/ESPE, parents seem to be less inclined to choose surgery for less severe clitoromegaly. The group is firm that clitoral surgery should only be considered in cases of severe virilization (Prader III and up) and should be carried out in conjunction with repair of the common urogenital sinus when appropriate. According to the consensus, “Emphasis is on functional outcome rather than a strictly cosmetic appearance.” Additionally, the group recognizes that surgical reconstruction in infancy may need to be refined at the time of puberty and that vaginal dilatation should not be undertaken before puberty.

The Need for Better Data
While the Consensus statement on management of intersex disorders is fairly comprehensive, the authors continually highlight the need for better long-term outcome data than is currently available. Within this data is a tremendous need for information on quality of life which, according to the authors, encompasses falling in love, dating, attraction, ability to develop intimate relationships, sexual functioning, and the opportunity to marry and raise children, regardless of biological indicators of sex. To that end, the report urges health care staff to offer adolescent patients the opportunity to talk confidentially without their parents as well as to encourage participation in support groups so that they may discuss their concerns comfortably.

If you would like more information on any of the issues discussed here, please call (toll free) 866-227-3737.

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