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EDITORIAL
Year : 2022  |  Volume : 27  |  Issue : 4  |  Page : 372-375
 

Changing paradigms in intersex management: Legal, ethical, and medical implications


Department of Pediatric Urology, Sri Ramachandra Institute of Higher Education and Research, Past Chairman of Society of Pediatric Urology (IAPS), Chennai, Tamil Nadu, India

Date of Submission05-Mar-2022
Date of Acceptance13-Mar-2022
Date of Web Publication26-Jul-2022

Correspondence Address:
Ramesh Babu
Department of Pediatric Urology, Sri Ramachandra Institute of Higher Education and Research, Porur, Chennai - 600 116, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_41_22

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How to cite this article:
Babu R. Changing paradigms in intersex management: Legal, ethical, and medical implications. J Indian Assoc Pediatr Surg 2022;27:372-5

How to cite this URL:
Babu R. Changing paradigms in intersex management: Legal, ethical, and medical implications. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Dec 9];27:372-5. Available from: https://www.jiaps.com/text.asp?2022/27/4/372/352290







   Background Top


The term “intersex” covers a variety of conditions also known as disorders/differences of sex development (DSD). The older terminology, with phrasing such as “hermaphrodite” and “pseudohermaphrodite,” was felt to be pejorative, confusing, and stigmatizing. The new classification proposed is sensitive to individuals and families, and also more reflective of molecular understanding in sex development research.[1],[2] There is still considerable controversy in the field regarding proper and respectful nomenclature. The consensus statement of the year 2018[3] renamed all the conditions known erstwhile as intersex, pseudohermaphroditism, or disorders of sex differentiation proposed in 2006[2] into a more inclusive terminology, DSD. While the term “intersex” has been replaced by DSD in medical literature, patient support groups still prefer this term.

The terms “gender,” “sex,” and “sexual” have discordant interpretations. “Gender” is a social concept, which is the way the society mirrors the “individual identity.” It does not take into account the “individual identity” (“inside identity”) and the future “gender role” (“behavioral identity”), which are invisible at birth and the modalities of which are mostly unknown.[4] Gender identity refers to a fundamental sense of belonging and self-identification of one's gender as male, female, or an alternative gender.[5] The term gender role describes the behaviors, attitudes, and personality traits that a society designates, in a given culture, as masculine or feminine. Gender role should be considered distinct from core gender identity.[6]

The term “sex,” on the other hand, refers to anatomical characteristics (phenotypic sex, genotypic sex – karyotype, gonadal sex, and internal anatomy) and sex of rearing – sex assigned by parents or caregivers. Sexual orientation, a totally different entity from gender, is defined by a person's responsiveness to another person, the salient dimension being the sex of the person to whom one is sexually attracted (heterosexual, bisexual, etc.).[2] Thus, when we discuss further, we would be talking of sex assignment and not gender assignment, as gender is a self-identity.

Several studies[7],[8] have disputed the “optimal gender policy” for infants with DSD. While the notion that “nurture overrules nature” in gender identity prevailed in the past, studies have shown that DSD children do not always conform to the sex of rearing as they grow into adults.[9] Gender dysphoria refers to a conflict between a person's physical or assigned sex and the gender with which they identify. The more recent term for gender dysphoria is gender identity disorder (GID). People with GID[10] have a strong and persistent cross-gender identification manifested in adolescents and adults by symptoms such as a stated desire to be the other sex, desire to live or be treated as the other sex, or the conviction that he or she has the typical feelings and reactions of the other sex.


   Legal Implications Top


The term transgender refers to a person whose sense of personal identity and gender does not correspond with their birth sex. They are totally different from DSD in the sense that they are born with unambiguous sexual anatomy. They seek sex reassignment surgery in adulthood due to gender dysphoria, and it is performed by adult surgeons with their personal consent. On the other hand, intersex surgery is often performed in children with ambiguous genitalia, essentially for medical indications by pediatric surgeons after obtaining parental consent.

However, the LGBTQIA+ activist groups consider intersex children's rights along with transgender rights. The 2019 case in Madurai bench of Madras High Court started with rights of a transgender marriage. When the intersex activists pleaded to be heard on intersex surgeries, the judge[11] asked the director of medical education to formulate guidelines on intersex surgeries. The judge quoted from Khalil Gibran: “Your children are not your children. They are the sons and daughters of Life's longing for itself. They come through you but not from you, And though they are with you yet they belong not to you. You may give them your love but not your thoughts, For they have their own thoughts. You may house their bodies but not their souls, For their souls dwell in the house of tomorrow, which you cannot visit, not even in your dreams. You may strive to be like them, but seek not to make them like you.”

A different hearing on Madras High Court in 2022 on a transgender marriage grievance (they sought police protection from their parents!) took up difficulties faced by LGBTQIA+ community[12] and issued directions to the National Medical Council (NMC) and State Medical Councils to incorporate medical and legal updates in their curricula: avoid pathologization and criminalization of nonheterosexual relations, gender nonconformity, gender incongruence, etc., The NMC and State Medical Council were asked to appoint a committee and submit a compliance report on the recommendations. In this regard, the 2020 guidelines already formed by the Tamil Nadu government (following the earlier court directive) to regulate DSD management gains relevance.


   Ethical Implications Top


Human Rights Watch, USA, Congenital Adrenal Hyperplasia Research/Education/Support Foundation, and Advocates for Intersex Youth (InterACT) along with Dr. Dix Poppas, chief of pediatric urology at Cornell-Weill Medical Center in New York City, produced an extensive document after interviewing several intersex adults.[13] They felt that assigning a sex of rearing to a child never required surgery and suggested society of pediatric urology to issue guidance on surgeries in individuals born with DSD. They also felt that intersex surgeries are unethical and should be avoided until the patient can actively participate in decision-making.


   Medical Implications Top


Assigning a sex of rearing is a great responsibility and should not be rushed. Many factors must be taken into account, and thus, it is essential to collect all relevant information while still being expeditious. When addressing the infant, it is important to initially be gender-neutral and avoid “he” or “she” pronouns that could inadvertently bias the family toward a gender that may later be reversed. Rather than referring to the child as “it,” warmer terms like “your baby” may promote greater bonding. Staying neutral until the decision is finalized helps prevent misunderstandings and confusion. A recent meta-analysis[14] showed that GID is low in women with congenital adrenal hyperplasia (CAH), complete androgen insensitivity syndrome (CAIS), and complete gonadal dysgenesis favoring female sex of rearing in them. GID is high in women with 5-alpha reductase deficiency/17-hydroxysteroid dehydrogenase deficiency favoring male sex of rearing in these DSD. GID is variable in partial androgen insensitivity syndrome (PAIS) or mixed gonadal dysgenesis (MGD), and no recommendations on sex of rearing could be made in these conditions. In PAIS/MGD children, multiple factors such as local anatomy, hormonal profile, and genetics have to be considered. These parents have to be given the option of leaving the sex indeterminate until their children are able to develop gender identity as they go through adolescence.


   Way Forward Top


In view of the growing concerns, it is imperative to understand the intricacies and come up uniform national & state guidelines. DSD is ideally managed only in specific centers with multidisciplinary committee. The medical director/dean of each tertiary hospital/medical college should act as a lead in the formation of such local multidisciplinary committee (LMDC). The committee should include at least three specialists: pediatrician/endocrinologist, pediatric surgeon, and psychologist/psychiatrist. Sex assignment should be done only at designated centers by LMDC after thorough evaluation, diagnosis, and discussion with all stakeholders. An Apex multidisciplinary committee should be formed at the national & state capital (the lead pediatric surgical center).

Decisions about nature and timing of any surgery are made with the family (or involving the adolescent child), by the LMDC acknowledging the considerable psychological impact. When a potentially “life-threatening” circumstance arises (urosepsis/cancer risk to the life) on the intersex child, the interest of the child is of paramount importance. Such decisions can be made after discussion with LMDC if urgent: DSD with holdup of menstrual or mucinous fluid or recurrent urine infection due to stasis of urine in common channel – becoming potentially “life threatening” due to urosepsis (in these children, vaginoplasty/such procedures to clear excretory fluids are justified). In children with XY/MGD with streak gonads, the risk of cancer is high (40%–60%) making it potentially “life threatening.”[15] Excision biopsy of streak gonad/gonad in PAIS/ovotestis with high risk of cancer is justified after getting informed consent. LMDC should keep a record of a signed document from all members and parents about the necessity for surgical intervention.

There are several other conditions like CAH, where the guidelines on timing of surgical intervention are still evolving. Clitoral surgery has been known to alter/impair sensation[16],[17] although Canning felt that follow-up of older techniques is unlikely to represent outcomes of current nerve-sparing procedures.[18] In most cases of CAH, an adequate hormonal suppression is known to control clitorimegaly.[19] Several authors recommend delaying of vaginoplasty until puberty when it can be safely performed with informed consent of the grownup.[20],[21] On the other hand, twothirds of caregivers of female infants with CAH reported not regretting their decisionmaking of early surgery during childhood.[22] Majority of females with CAH and parents believe that CAH should be excluded from the intersex designation, and should be considered separately in legislation pertaining to childhood genital surgery.[23] While the cry to delay cosmetic surgeries to a later date gets louder, unknown effects of having atypical genitalia on children and parents are unclear.[24] Only recently a small feasibility study on deferring surgery in CAH, has reported that girls and their parents did not express significant concerns regarding genital ambiguity.[25] Hence, when forming national/ state guidelines, one has to weigh the pros and cons of genital surgery in CAH. Another area of debate is Prader 5 CAH reared as male, in whom a late feminizing genitoplasty may be unwise as due to androgen effects they often do not have gender dysphoria as men.[26] These children may need hysterectomy/gonadectomy, but this should be delayed until puberty when an informed decision can be made.[27]

In children with CAIS, the risk of germ cell tumor is <1% and the risk is only after the second decade.[15] Therefore, gonadectomy can be postponed allowing spontaneous feminization at puberty while avoiding hormonal replacement.[19] The least controversial of all decisions would be hypospadias surgery in those with bilateral palpable testes and XY karyotype. Appropriate timing of staged hypospadias repair is around 9–18 months,[19] and these children do not need to go through LMDC/DSD pathway.


   Summary Top


There is a growing concern among activists that sex assignment is often performed with binary notion (fitting them into male/female framework), discarding the option of third/neutral sex assignment. Although gender-neutral upbringing and a third sex of rearing are accepted by the Law, in the Indian context the parents prefer to have a sex assigned for the baby, in order to provide a safe and socially secure upbringing. Even in Western countries where there is social acceptance of third gender, the psychosocial ramifications of such approach (not choosing a gender) are unclear[28] and there is no law yet to regulate their medical management.

In an Indian setting, the children born with DSD are completely at the support of their parents, who in turn are dependent on their extended family and society (not social welfare/government support). Denying them the necessary medical attention may lead to child neglect, or casting their child away. While the court has directed school authorities to build toilets for third gender, in the Indian setting where open toilets are still prevalent, these children are likely to be bullied and teased by other children leading to depression or self-harm.

Until views of the society are changed via education or self-realization, imposing laws or rules to ban all DSD surgeries will make the parents turn to quackery to achieve their aims. A rather better way is to ensure that DSD children continue to receive required medical care from qualified pediatric surgeons. It is indeed prudent to regulate these surgeries via guidelines, regulations, consensus, and informed decisions involving LMDC and parents. Each DSD patient is unique, and they warrant multidisciplinary care and long-term psychosexual support.



 
   References Top

1.
Lee PA, Houk CP, Ahmed SF, Hughes IA; International Consensus Conference on Intersex Organized by the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. Consensus statement on management of intersex disorders. International Consensus Conference on Intersex. Pediatrics 2006;118:e488-500.  Back to cited text no. 1
    
2.
Houk CP, Hughes IA, Ahmed SF, Lee PA; Writing Committee for the International Intersex Consensus Conference Participants. Summary of consensus statement on intersex disorders and their management. International Intersex Consensus Conference. Pediatrics 2006;118:753-7.  Back to cited text no. 2
    
3.
Cools M, Nordenström A, Robeva R, Hall J, Westerveld P, Flück C, et al. Caring for individuals with a difference of sex development (DSD): A consensus statement. Nat Rev Endocrinol 2018;14:415-29.  Back to cited text no. 3
    
4.
Mouriquand PD, Gorduza DB, Gay CL, Meyer-Bahlburg HF, Baker L, Baskin LS, et al. Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how? J Pediatr Urol 2016;12:139-49.  Back to cited text no. 4
    
5.
Fisher AD, Ristori J, Fanni E, Castellini G, Forti G, Maggi M. Gender identity, gender assignment and reassignment in individuals with disorders of sex development: A major of dilemma. Endocrinol Invest 2016;39:1207-24.  Back to cited text no. 5
    
6.
Hines M. Gender development and the human brain. Annu Rev Neurosci 2011;34:69-88.  Back to cited text no. 6
    
7.
de Vries AL, Doreleijers TA, Cohen-Kettenis PT. Disorders of sex development and gender identity outcome in adolescence and adulthood: Understanding gender identity development and its clinical implications. Pediatr Endocrinol Rev 2007;4:343-51.  Back to cited text no. 7
    
8.
Daae E, Feragen KB, Waehre A, Nermoen I, Falhammar H. Sexual orientation in individuals with congenital adrenal hyperplasia: A systematic review. Front Behav Neurosci 2020;14:38.  Back to cited text no. 8
    
9.
Yang JH, Baskin LS, DiSandro M. Gender identity in disorders of sex development: review article. Urology 2010;75:153-9.  Back to cited text no. 9
    
10.
Cohen-Kettenis PT, Pfäfflin F. The DSM diagnostic criteria for gender identity disorder in adolescents and adults. Arch Sex Behav 2010;39:499-513.  Back to cited text no. 10
    
11.
Justice Swaminathan G. Arun Kumar and Sreeja v. Inspecter General of police, 2019. p. WP(MD)No. 4125 of 2019 (at Madurai Bench of Madras High Court).   Back to cited text no. 11
    
12.
Justice Anand Venkatesh N. Sushma and Seema v. commissioner of police, 2021. p. W.P.No. 7284 of 2021 (Madras High Court).  Back to cited text no. 12
    
13.
Tamar-Mattis A, Ittelson A, Fraser S, Zieselman K. I want to be Like Nature Made Me: Medically Unnecessary Surgeries on Intersex Children in the US. Human Rights Watch. (2017). Available from: https://www.hrw.org/report/2017/07/25/i-want-be-nature-made-me/medically-unnecessary-surgeries-intersex-children-us. [Last accessed on 2022 Jun 01].  Back to cited text no. 13
    
14.
Babu R, Shah U. Gender identity disorder (GID) in adolescents and adults with differences of sex development (DSD): A systematic review and meta-analysis. Pediatr Urol 2021;17:39-47.  Back to cited text no. 14
    
15.
Abacı A, Çatlı G, Berberoğlu M. Gonadal malignancy risk and prophylactic gonadectomy in disorders of sexual development. J Pediatr Endocrinol Metab 2015;28:1019-27.  Back to cited text no. 15
    
16.
Crouch NS, Liao LM, Woodhouse CR, Conway GS, Creighton SM. Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia. J Urol 2008;179:634-8.  Back to cited text no. 16
    
17.
Creighton S, Chernausek SD, Romao R, Ransley P, Salle JP. Timing and nature of reconstructive surgery for disorders of sex development – Introduction. Pediatr Urol 2012;8:602-10.  Back to cited text no. 17
    
18.
Canning DA. Commentary on genital sensation after feminizing genitoplasty. J Urol 2005;173:982.  Back to cited text no. 18
    
19.
Bangalore Krishna K, Kogan BA, Mazur T, Hoebeke P, Bogaert G, Lee PA. Individualized care for patients with intersex (differences of sex development): Part 4/5.Considering the Ifs, Whens, and Whats regarding sexual-reproductive system surgery. Pediatr Urol 2021;17:338-45.  Back to cited text no. 19
    
20.
Ahmed SF, Achermann JC, Arlt W, Balen AH, Conway G, Edwards ZL, et al. UK guidance on the initial evaluation of an infant or an adolescent with a suspected disorder of sex development. Clin Endocrinol (Oxf) 2011;75:12-26.  Back to cited text no. 20
    
21.
Diamond M, Garland J. Evidence regarding cosmetic and medically unnecessary surgery on infants. Pediatr Urol 2014;10:2-6.  Back to cited text no. 21
    
22.
Fisher RS, Espeleta HC, Baskin LS, Buchanan CL, Chan YM, Cheng EY, et al. Decisional regret about surgical and non-surgical issues after genitoplasty among caregivers of female infants with CAH. J Pediatr Urol 2022;18:27-33.  Back to cited text no. 22
    
23.
Szymanski KM, Rink RC, Whittam B, Hensel DJ, Braga LH, Donahue KL, et al. Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex. Pediatr Urol 2021;17:210.e1-9.  Back to cited text no. 23
    
24.
Lee PA, Fuqua JS, Houk CP, Kogan BA, Mazur T, Caldamone A. Individualized care for patients with intersex (disorders/differences of sex development): Part I. Pediatr Urol 2020;16:230-7.  Back to cited text no. 24
    
25.
Bougnères P, Bouvattier C, Cartigny M, Michala L. Deferring surgical treatment of ambiguous genitalia into adolescence in girls with 21-hydroxylase deficiency: A feasibility study. Int J Pediatr Endocrinol 2017;2017:3.  Back to cited text no. 25
    
26.
Chowdhury TK, Laila K, Hutson JM, Banu T. Male gender identity in children with 46, XX DSD with congenital adrenal hyperplasia after delayed presentation in mid-childhood. Pediatr Surg 2015;50:2060-2.  Back to cited text no. 26
    
27.
Bangalore Krishna K, Kogan BA, Ernst MM, Romao RL, Mohsin F, Serrano-Gonzalez M, et al. Individualized care for patients with intersex (disorders/differences of sex development): Part 3. Pediatr Urol 2020;16:598-605.  Back to cited text no. 27
    
28.
Ernst MM, Kogan BA, Lee PA. Gender identity: A psychosocial primer for providing care to patients with a disorder/difference of sex development and their families [individualized care for patients with intersex (Disorders/differences of sex development): Part 2]. Pediatr Urol 2020;16:606-11.  Back to cited text no. 28
    




 

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   Background
   Legal Implications
   Ethical Implications
   Medical Implications
   Way Forward
   Summary
    References

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