A shared decision-making process would assist doctors and parents who are facing the extraordinarily complex, challenging and controversial choices presented when infants are born with genetic or anatomical anomalies in sexual development and are being considered for elective corrective surgery, a new research paper suggests.
The paper does not address instances in which infants are born with conditions that pose an imminent threat to their health -- such as when children are born without a urinary opening. Instead, the paper is intended to propose guidelines for use when surgery is being considered to make a child's appearance more typical of their sex in order to facilitate their gender-identity development.
"Difficult Decisions: Disorders of Sex Development and Surgical Intervention" is published online in the August issue of the Journal of Pediatric Endocrinology and Metabolism. In it the researchers suggest that a six-step decision-making approach would afford health-care providers the opportunity to clarify the reasons for their recommendations, identify and fill gaps in parents' understanding of their child's diagnosis and treatment options, and explore the values underlying both parents' and clinicians' concerns.
"The big issue that we are addressing is that there is no standard approach or best practice for physicians and family members to follow to address decision making for infants who are born with disorders of sex development" or with atypical sexual development, said Alexander Kon, senior author of the study and associate professor of pediatrics and bioethics at the UC Davis School of Medicine.
Study first author Katrina Karzakis, a senior research scholar at the Center for Biomedical Ethics at Stanford University, agreed.
"There are a lot of gaps in evidence-based medicine regarding these types of procedures that aren't going to be filled any time soon," said Karzakis, who is the author of a book on disorders of sex development called "Fixing Sex: Intersex, Medical Authority and Lived Experience." "But, every day, physicians are seeing patients in the clinic and parents are struggling to make decisions about the best way to care for them."
Disorders of sex development, or differentiation, refer to congenital conditions in which the development of chromosomal, gonadal or anatomical sex is atypical. The disorders include a broad range of conditions such as ones in which infants are born with genitalia having both masculine and feminine attributes, and infants whose genitalia is atypical for their sex because it is over-masculinzed for a female or else under-masculinized for a male.
Karzakis said that the overall incidence of disorders of sex development is estimated at 1 in 2,000. But approximately 70 percent of patients experience a family of disorders called congenital adrenal hyperplasia. Most of the conditions involve excessive or deficient production of sex steroids and can alter development of primary or secondary sex characteristics.
Numerous health-care organizations -- including the Institute of Medicine and American College of Physicians -- have suggested that there is a need for a clearly defined process for medical decision making. The authors have applied this recommendation to the process for considering elective genital surgery, or genitoplasty, for children born with atypical sex development. In the past, such decisions have been driven by physicians' and parents' personal values and "gut feelings," often with less-than-optimal outcomes, the study says. Health-care providers often report feeling conflicted about whether they have made the right recommendations to families, and parents report feeling rushed into decision making. The researchers said that shared decision making would require clinical caregivers to reveal their reasoning, values and biases and explore their patients' or their surrogates feelings.
"The pediatric literature suggests that about a quarter of families want completely family-driven decision making and another quarter want completely physician-driven decision making," Kon said. "The other half want shared decision making. We tried to develop a process that would allow families to feel comfortable with expressing their feelings and values in a setting that also involves physicians, nurses, chaplains and others in the process."
"We realized," Karzakis said, "that we could help to improve decision making for patients with disorders of sex development just by working on the decision-making process. Part of what we're saying in this paper is that you don't need to know all the answers, and a decision about gender assignment does not have to be a decision about surgery -- you don't have to conflate those decisions."
The researchers recommend these six steps for shared decision making:
Anne Tamar-Mattis of the Advocates for Informed Choice, Cotati, Calif., also is an author of the study.
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