What do Parents Need to Know? Information that Should be Part of Shared Decision Making for Genitoplasty in Pediatric Patients with DSD (Published in: Journal of Pediatric Endocrinology & Metabolism, 23, 789-806 (2010))
Authors Karkazis, Tamar-Mattis and Kon call for the implementation of a ‘Shared Decision Making’ process in genital surgery. The authors produced a list of things ‘What Parents Need to Know’. As it is a useful tool for parents to think through and to help formulate questions to their medical care team, we reproduce it here (please note this relates to an American healthcare setting - written in American English).
What Parents Need to Know (when considering early surgery):
- Full details of the child’s diagnosis, including chromosomes, internal and external organs, and possible atypical hormone exposure before birth.
- Potential for fertility, together with options for assisted reproduction that exist now or seem likely during the child’s reproductive lifetime;
- Potential for sexual function, including the capacity for sexual pleasure, orgasm, and emotional fulfilment as well as for penetration;
- Gender identity outcomes in children with the same condition;
- Psychological outcomes in children with the same or similar conditions, to the extent data are available.
Rationale for Proposed Procedure(s)
- Information about the purpose of proposed interventions (e.g., to alter appearance, to allow voiding of urine, to allow standing urination, to allow for future menstrual flow, to allow for future sexual intercourse, to support gender identity development);
- Where multiple procedures are proposed, the rationale for each must be disclosed;
- Where outcome data in support of the rationale are lacking, it is important to clarify the basis for assertions made to parents (i.e., provider opinion or experience).
Risks and Benefits of Proposed Procedures
- All material risks of proposed procedures, including minor risks if are common, and remote risks if potential harm is serious;
- Potential benefits;
- The likelihood of various outcomes, where this is known, or the uncertainty of suggested outcomes;
- Psychological risks (e.g., if a surgery or its possible complications will increase the necessity for multiple genital exams in childhood, the risk for psychological harm from such exams);
- Risks and benefits throughout the patient’s lifespan, including known or suspected risk of damaging adult sexual sensation and the risk of psychological harm if the patient later regrets the decision;
- Parents’ false assumptions should be corrected (i.e. that genitoplasty is 'reversible,' that early genitoplasty will ensure adoption of the assigned gender or development of a heterosexual identity, or that surgery will obviate the need to tell the child of his or her condition).
Alternatives to Proposed Treatment
- Alternative procedures, along with their risks and benefits as described above;
- Non-treatment, watchful waiting and delayed treatment, along with their risks and benefits;
- Non-surgical treatment options, including psychological counselling for the child and family.
- Parents need to know that children born with DSD may enjoy healthy relationships and a well-adjusted childhood without surgical treatment.
- Alternative treatments must be disclosed, regardless of the patient’s ability to pay or the availability of insurance coverage for alternatives. Where insurance may cover a procedure in childhood but not in adulthood, this possibility should be discussed.
- Parents should be informed of their right to receive a second opinion and their right to change clinicians if disagreements cannot be resolved.
- Expectations for aftercare, including length of hospital stay, need for pain control, and potential for post-surgical complications in the near and long term.
- Where the need for ongoing catheterization or vaginal dilation is a possibility: associated challenges and potential for psychological harm from these procedures. If the parents will be expected to perform the dilations or catheterization, they should know this in advance.
- Surgeons’ level of experience with the proposed procedures along with the surgeons’ and hospital’s outcomes including types and rates of complication.
- Where there is evidence of better outcomes at centres of excellence, this information must be disclosed.
Referrals to Professional and Peer Support
- Where decision-making is based on speculation about the child’s future quality of life, such access may provide information critical for informed decision making.
Where Vaginoplasty is Contemplated
- Lifelong need for dilation;
- Risk of scarring that may preclude intercourse and diminish fertility;
- Risk of psychological harm from vaginal dilation in childhood;
- Patient groups have reported that the available techniques for vaginoplasty (i.e. self-dilation, skin substitution, and bowel vaginoplasty) have varying advantages and drawbacks across the lifespan. Adults who have made choices for themselves about these procedures have demonstrated that idiosyncratic personal preferences play a big role decision making. Parents need to understand these variables when making decisions about vaginoplasty on behalf of a child.
Where Clitoroplasty is Contemplated
- That long-term effects of nerve-sparing techniques on clitoral sensitivity and sexual function are unknown;
- Potential negative impact of reduced clitoral sensitivity on future sexual activity and intimate relationships.
- Possibility of need for multiple surgery;
- Risk for complications that would make repeat surgeries non-elective, and of the associated psychological risks if multiple genital surgeries in childhood should become necessary;
- Where hypospadias repair is contemplated for the purpose of improving fertility: other options for addressing impaired fertility (i.e., alternative insemination);
- Where hypospadias repair is recommended to increase the probability that the child will be able to stand for urination: possibility that the child will be able to stand to urinate without the repair, and the possibility of fanning or spraying of the urine stream after repair which could make standing urination difficult.
The Older Pediatric Patient
- As the child with a DSD matures, it is appropriate to involve him or her in decisions about ongoing treatment. The AAP provides guidance regarding assent for procedures that are elective. There is uncertainty about the validity of parental consent alone for genitoplasty or gonadectomy at any age; it is clearly inappropriate, and possibly a violation of civil rights, to perform these procedures on an older child or teen without assent;
- When the patient reaches the age of 18, he or she will have the right to access the entire medical record. Parents who are considering limiting disclosure to the child need to be aware of this;
- Where state or constitutional law accords mature minors the authority to make their own informed choices, the information necessary to make an informed decision must be fully provided to the minor in understandable language;
- Where a mature minor and a parent disagree over treatment choices, or where disagreement over treatment arises between parents after the SDM process, the provider should obtain ethical and legal advice before treatment is performed.
Androgen Insensitivity Syndrome
- Use of misleading terminology (i.e., referring to testes as “twisted ovaries” or referring to an XY genotype as “female chromosomes”) is contrary to principles of informed consent and is ethically inappropriate;
- If reproductive structures such as testes are unexpectedly discovered during surgery, separate informed consent should be obtained before any resection.
Congenital Adrenal Hyperplasia
- Percentage of 46,XX children with CAH who ultimately develop a male gender identity;
- Where a 'one-stage repair' is contemplated: the distinct procedures involved (i.e., vaginoplasty and clitoroplasty), and the differing rationales for each;
- Where vaginoplasty is recommended for functional reasons: whether the functional issue is immediate (i.e., to allow for urine flow) or anticipated in the future (i.e., to avoid possible UTIs; to allow for eventual menstrual flow; to allow for future heterosexual intercourse).
- Where genital surgery is recommended to prevent possible UTIs due to presence of a urogenital sinus: the absence of evidence that UTIs are more common in girls with a urogential sinus, and the possibility that surgery could increase the incidence of UTIs.
Pitfalls to Avoid
Failure to Clarify Quality of Evidence
- Offering information without clarifying which assertions are based on provider’s opinion, rather than evidence-based published studies;
- Failing to explain existence of medical controversy about treatment options or existence of conflicting data.
- Urging parents to decide quickly when the condition is in fact non-emergent;
- Offering biased information (i.e., urging a reluctant parent to consent to genitoplasty in order to prevent urinary tract infections when in fact the risk of UTI is minor, but the provider is convinced that genitoplasty is necessary for psychological functioning or parental comfort);
- Excessive optimism about functional and cosmetic outcomes of genitoplasty;
- Putting a “spin” on factual data. For example:
- A statement such as “In favourable cases, the maximum number of operations can be two or three,” could leave a parent with the impression that the maximum number of operations will be two or three;
- A statement such as “it is possible to achieve both favourable functional and favourable cosmetic outcomes,” without qualification, may leave parents with the impression that such an outcome is likely or even assured.
Practicing Outside the Field of Competency
- If a surgeon with no specialized training in child psychology, child development or a related field recommends genitoplasty for reasons of ensuring normal sexual development, this could be found to be practicing outside the field of competency. In such a case, the physician would be held to the standard of care of a specialist in the field. Consultation with an appropriate specialist is recommended if surgery is contemplated for psychosocial reasons.