Year : 2014 | Volume
: 19 | Issue : 1 | Page : 3--4
Disorders of Sex Development: The quintessence of perennial controversies
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110 029, India
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110 029
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Bajpai M. Disorders of Sex Development: The quintessence of perennial controversies.J Indian Assoc Pediatr Surg 2014;19:3-4
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Bajpai M. Disorders of Sex Development: The quintessence of perennial controversies. J Indian Assoc Pediatr Surg [serial online] 2014 [cited 2019 Aug 22 ];19:3-4
Available from: http://www.jiaps.com/text.asp?2014/19/1/3/125943
From time immemorial, issues related to 'Sex change' operations, Transgenders and Gender dysphoria have kept the historians, ancient scriptures and the media habitually engaged. As recently as the end of 2013 a PIL (Public Interest Litigation) in the highest court of the land was raised that was intended to know the Government's position on gender assignment to the Transgenders. The controversies involving the sports persons revolve around: Gender verification in sports, as, sporting events are limited to a single sex. The brainteaser being the allegation: whether the male athlete competed as woman? As Pediatric Surgeons the first question which comes to our minds is: Did 'he' have DSD?
In this treatise we would initially touch base (repetition being the basis of memory) before aspiring for a bigger picture (setting out for seeking solution).
Deciphering the myths
Disorders of Sex Development (DSD) have been previously known as Intersex disorders. There are misunderstandings attached with these conditions, mostly because of ignorance. These conditions are caused by genetic and endocrine imbalances in fetal life and children are born with genital appearances which do not conform to clearly male or female genital appearances. The infant may be often rejected by the parents and generally discriminated by the society. Corrective surgery is offered to these children to keep the best interest of the child.
The questions which are often asked are:
Is it a sex-change operation; Who is the best person to take a decision regarding nature of surgery: Parents or patientsIs the decision part of the parents' desire duly fulfilled by doctorsWhat is the best age of such surgeriesDo the doctors undertake appropriate investigations before arriving at a decisionWhat is after-surgery life
Social and Health activists want decisions on treatment be delayed till the children reach the age of consent. Parents often do not agree to this.
Controversy exists around ethics of performing genital surgery between individuals, families, professionals, ethicists, and activists in the treatment and management of DSD conditions.
There is lack of clarity even among the general medical fraternity regarding the protocol of management. Molecular and Genetic studies are at the core of making even the basic diagnosis of the Disorders of Sex Development (DSD). Among the other inputs immediately required are, a brief but vigorous orientation programs among the care givers; immediate reassurance to the family of the child backed with correct scientific information, regional socio-cultural considerations and a legislation to protect the interest of the child.
Infants are born with a DSD condition and are unable to make an informed decision to consent regarding their treatment; thus, leaving the issue to be dealt with by parents in the best interests of the child. Some advocates believe such a decision should be made by the courts as parents should not have to make a potentially irreversible decision. Preferred treatment of children with XY chromosomes and ambiguous genitalia (with parents' consent) is to assign them as male to conserve genital tissue leaving the possibility of choice when they are older.
Who is the right person to take decision regarding choice and time of surgery- Parents or Patients?
Decisions about all options for treatment are presented and discussed at length with parents. Parents always have the final say and are given the option as to delay surgery if they feel unable to make a decision. They may be referred to a social worker or psychologist for emotional support and counseling.
Parents pick up signals from their children's pediatricians about what they should do, sometimes picking up signals pediatrician don't intend. The best thing pediatricians can do is to be explicit with parents (and with patients at appropriate age) about the choices available and the evidence regarding outcomes for each.
If there's no urgent need for surgery - as is the case for many DSD surgeries - clinicians can make vivid the option of waiting by introducing the family to adults who have lived without the intervention being considered and to other parents who have decided to hold off and let the child decide. So, when working with parents considering clitoral reduction surgery for their infant daughter, one pediatric urologist has introduced those parents to another set of parents who chose against infant clitoral reduction surgery - and to the daughter herself (now an adolescent).
This way the parents facing a decision have had the opportunity to meet a child who will make the elective surgery decision for herself, and to learn from her and her parents how to manage the psychosocial challenges of raising a girl with a noticeable clitoris.
Doctors wanting to perform surgery to treat ambiguous genitalia in infants too young to consent on their behalf themselves should have to be guided by the appropriately laid down guidelines.
While, debates about the best way to care for patients with DSD continue, of paramount importance is, to consider an approach which minimizes any potential harm to patients and their families. The goal of DSD treatment is the long-term physical, psychological, and sexual well-being of the patient. This approach is therefore termed empathetic and is provided considerate care. It is felt that parental rights and responsibilities as proxy decision-makers should be respected in cases where informed parents continue to request early genital surgery for their DSD child. This situation further highlights the need for data-driven recommendations.
It is being increasingly recognized that, what is normal for one individual may not be for other; care providers should not seek to force the patient into a social norm (e.g., phallic size or gender-typical behaviors) that may harm the patient.
Minimize the potential for the patient and family to feel ashamed, stigmatized, or overly obsessed with genital appearance. Thus, initial gender assignment (boy or girl) is made by the parents after the parents have been fully informed about the results of tests and what is known about gender identity development in patients with similar conditions. Because the parents will be primary care givers for the child, and because they are legal decision-makers for the child, it is critical that their sense of the situation be taken seriously and that they actively participate in the initial gender assignment.
The psychiatrist or psychologist on the team may evaluate the care givers in terms of their education, cognitive capacity, skills coping etc., as relevant to their ability to understand the DSD and to nurture a child with a DSD. Such factors may in some cases, have bearing on the gender assignment recommended by the team. Not infrequently, for various reasons such as the disease's late onset, some will be reared as males and some will request such assignment.
Corrective surgery raises many questions. (To be continued in Part II).
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