Proposal of Standards Of Care
For Harry Benjamin Syndrome
by Charlotte Goiar, Author.
Copyright @ 2006, Charlotte
All Rights Reserved.
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III. Definition and Etiology.
IV. Epidemiological Considerations.
VI. Classification and Terminology.
Requeriments for Professionals.
VIII. Diagnosis and Treatment.
IX. Treatment of HBS in Children.
X. Treatment of
HBS in Adolescents.
XI. Treatment of HBS in Adults.
XII. HBS along with other conditions.
XIII. Artificially Induced
The purpose of the
Standards of Care for Harry Benjamin Syndrome (SOC-HBS) is to articulate a general consensus about the medical management
of Harry Benjamin Syndrome (HBS). Professionals may use this document to understand the parameters within so that they may
offer assistance to those with problems arising from HBS. Persons born with HBS, their families, and social institutions may
use the SOC-HBS as a means to understand the current sometimes misguided tendencies of treatment of patients by professionals.
All readers should be aware of the limitations of research in this area and of the hope that the clinical
uncertainties will be resolved in the future through further scientific investigation. It should be carefully understood that
Harry Benjamin Syndrome is not “a
choice” or a result of nurture; it is an inborn anomaly as are
all intersex conditions. In this paper all references to sex apply to brain sex of the HBS pre-surgical patient.
Harry Benjamin Syndrome is a medical condition caused by a biological variation
in human sexual formation –an intersex condition- where the sex indicated by the phenotype and the genotype is opposite
the morphological sex of the brain.
born with Harry Benjamin Syndrome are born with male and female characteristics, and they seek rehabilitation of their phenotype
and endocrinology to accord with their dominant sexual identity, an identity which is determined by the structure of the brain.
Harry Benjamin Syndrome is about matching one’s physical sex with one’s brain sex, it is not about sexual behavior.
Harry Benjamin Syndrome is about being a particular sex, not about “doing it”. It is also about recognising gender
norms, not challenging them.
Benjamin Syndrome is an ancient and persistent part of human nature, not a modern discovery. Persons born with Harry Benjamin
Syndrome have a legitimate medical condition and it should be given the same consideration and respect and rigorous approach
as any other medical condition.
afflicted with Harry Benjamin Syndrome have the right to obtain adequate medical care for their condition as soon as they
request it. All public health systems should respond to this request to the best of their ability and with all due dignity
and respect to the person making the request.
with Harry Benjamin Syndrome have the right to preserve their intimacy and privacy in respect to their condition and situation.
Any labels naming or suggesting the social or sexual stages during or after corrective treatment must be absolutely avoided
when and if possible. Other labels for identity, apart from Male or Female, are not acceptable. Medical care and treatment
should be soberly conducted and without stigma.
Benjamin Syndrome is a Life-threatening condition; not only because of its nature but also because of the social stigma that
surrounds it and the resulting ignorance that causes societal and family discrimination.
Benjamin Syndrome’s sufferers have the right to function in society with full dignity, respect and legal rights, regardless
of their temporary physical situations during corrective treatments, therefore all international legal systems should adjust
to this as a feasible policy and guarantee it for all patients with HBS.
Benjamin Syndrome should not be confused with gender variant conditions or with sexual orientation issues. It is a physical
anomaly curable with hormonal-surgical intervention.
is unethical to discriminate in providing medical care to HBS patients based on their sexual orientation, physical appearance,
HIV status, race or religion.
Definition and Aetiology.
Harry Benjamin Syndrome is an intersex
condition developed in the early stages of pregnancy affecting the process of sexual differentiation between male and female.
This happens when the brain develops as a certain sex but the rest of the body takes on the physical characteristics of the
opposite sex. The difference between this and most other intersex conditions is that there is to date no apparent pre-birth
evidence and the symtoms only become evident after the baby is born or even as late as adolescence. Harry Benjamin Syndrome
(HBS) is a congenital intersex condition that develops in the womb, involving the differentiation between male and female.
Therefore a girl with Harry Benjamin Syndrome would have a female brain sex but her genitals would appear male. Boys born
with this condition have female genitalia even though their brains are male. So far it is impossible to diagnose this condition
at the moment of birth resulting in the babies to be raised in the wrong gender role.
We now know that the brain is the only part of the body that can define
one’s sex. Therefore, one's true sex is determined by the structure of the brain, not by the genitalia. Gender identity
is hard-wired in the brain and in deeper CNS structures. The main difference between Harry Benjamin Syndrome and some other
intersex conditions is that there is no apparent evidence at the moment of birth making it possible for doctors to diagnose
IV. Epidemiological Considerations.
It is believed that 1 child out
of every 100.000 is born with Harry Benjamin Syndrome.
Harry Benjamin Syndrome was known
in the past under many different names, Transsexualism being the most common. Some have even added transsexualism as a sub-set
of transgender and that of course was never intended to be included under that umbrella term when transgender was coined.
However, Harry Benjamin Syndrome is not Transsexualism (or any variation of Transgenderism). At least not under the
current definition of Transsexualism provided by the ICD-10.
Harry Benjamin Syndrome is still incorrectly classified
as Transsexualism today by the ICD-10. “Transsexualism” is classified as a mental disorder with a psychological
cause. Harry Benjamin Syndrome is a physical condition indicative as a fetal anomaly. There is no relationship between
these conditions. Maintaining such an outdated policy only denies patients with Harry Benjamin Syndrome adequate medical care
and societal understanding. It can also often place them under the inappropriate medical standards of care.
Transsexualism was introduced in the Diagnostic and Statistical Manual of Mental Disorders (DSM-III) and fourteen years later,
in 1994 it was changed to Gender Identity Disorder (DSM-IV). It is supposed to be revised again in 2013 for DSM-V.
hope that the condition will be named Harry Benjamin Syndrome for the reasons we explain in the next section. We strongly
urge to remove Harry Benjamin Syndrome from the Diagnostic and Statistical Manual of Mental Disorders.
We propose these differentiated
levels of diagnosis:
-HBS in Children (below age 12)
-HBS in Adolescents (below age 18)
-HBS in Adults.
along with other intersex conditions.
-HBS along with other neurological conditions.
-HBS not otherwise specified.
VI. Classification and Terminology.
Benjamin Syndrome is a neurologically intersex condition and as such must be classified, as an Intersex condition not as a
mental disorder. Should Harry Benjamin Syndrome be denied as an intersex condition classification then it should be classified
among other rare medical conditions not related to any mental disorder.
Harry Benjamin Syndrome is named
in honor of Dr Harry Benjamin, a pioneer in the research of this condition. He
was the physician who contributed much to the understanding and recognition of this
condition. It was through his efforts the entire medical community came to understand HBS was unrelated to homosexuality.
Harry Benjamin Syndrome, (HBS), is greatly preferable to other terms
because it lacks the misleading connotations and inaccurate meanings they possess.
Basic Terminology associated
with Harry Benjamin Syndrome:
Harry Benjamin Syndrome.
Hormone Replacement Therapy. (HRT)
Sex Affirmation Surgery. (SAS)
Affirmation and Affirmation’s
Intersex, Intersexed, Intersexual.
Reflections about other terminologies commonly used
in the past:
a. Transsexualism – Transsexuals.
The most well-known term for Harry Benjamin's Syndrome is Transsexualism
(coined by sexologist Magnus Hirschfeld in the 20's). Unfortunately there are several problems with it. Simply including "sex"
in the name attracts undesirable attention, and the word strongly implies
a connection with sexual orientation that does not exist. It's too similar to "transvestism", a completely unrelated phenomenon
(men who enjoy wearing women's clothing don't have much in common with men
who were born with female anatomy and are very unhappy about it) that bears enough of a superficial similarity to cause much
confusion. Also, labelling people with Harry Benjamin's Syndrome with the term
"transsexual" is a bad thing. Saying that people are transsexuals is dehumanizing, and makes it easier to think of
them as being "other". People with Harry Benjamin's Syndrome are people, who
happen to have a particular medical problem; it is not their identity, and they are not specimens of the condition. Use as
an adjective is not much better; with "transsexual man" or "transsexual woman", it's too easy for "transsexual"
to be interpreted as "pretend", and for some reason many people seem to be confused about whether the appropriate noun for
any particular individual is "man" or "woman".
The term "transsexual"
is a combination of two words: "trans" + "sexual".
The word "trans"
is a Latin prefix which means across, beyond, through, so as to change. In modern English it means on, or to the other
side of, across, beyond. The word
"sexual" is an adjective derived from the Latin sexualis, and it means of, relating to, or associated with sex or the sexes.
When these two words are combined, the reason the term has been applied
to some individuals becomes apparent. Now well, those called "transsexuals", must make many adjustments
to overcome difficult obstacles. Somehow it seems unfair to burden people with labels which no longer seem appropriate when
they have made changes, adjustments, overcome obstacles, had surgery, and
taken their places in society in roles in which they are comfortable. In their
minds, it is no longer a question of being between or crossing between the sexes, as the term "transsexual" implies. And certainly,
at that point, they have not need not desires
to have surgical alteration of their genitals. If others needs to refer to them
for the purpose of identification, then they should use the terms "woman"
There is a great stigma associated to Transsexuality:
This is considered to be a "lifestyle choice" for many people or even a kind of "expansion" of one's personality, and the
term "transsexuality" itself reinforce even more this so deeply rooted stigma about Harry Benjamin's Syndrome. That's why
the findings of biological markers for the syndrome in parallel with the update of the terminology in use, are so liberating.
Transsexuality is a natural phenomenon in the animal kingdom and is there where to speak about transsexuality or transsexuals
is adequate, but it is not adequate to use this same terminology with human beings, with persons. Simply because the persons
aren't amphibious changing sex continually in a "natural" way, and of course is not a choice they made neither. People with
Harry Benjamin's Syndrome do not change sex, they don't become the opposite sex, they already are of a determined sex -determined
by its brain structure, which already is how it should be, and it cannot be "changed".
"Transgender" has become popular recently as an all-inclusive
term for a wide variety of extremely disparate groups, especially amongst the queer community This is not a positive development,
as it is far too broad a category to say anything useful, and implies similarities that do not exist. It's like having a word
that means "plays chess, is asian, or has cancer" - using it to descibe someone tells you nothing about them.
the preference to use SAS (Sex Affirmation Surgery) above other terms:
The term "Sex Affirmation
Surgery" (SAS) is preferable to the alternatives of "Sex Reassigment Surgery" (SRS) or "Gender Reassignment Surgery" for
describing the operation used to help correct the anatomies of people with Harry
Benjamin's Syndrome. There is certainly no reassignment of gender involved, and it only involves one aspect of physical sex,
which is not a binary, either-or characteristic.
The sex of the brain is already as it should be, and HRT corrects other sexual characteristics. For men, HRT can even go a
little way towards correcting the genitalia, though nowhere near the
complete development that occurs in the womb. It is about Affirmation (or correction of a physical defect), not about transition
The following are the minimal credentials for special competence with
1. Doctorate degree of Medicine (MD).
2. A master’s degree or its equivalent in a clinical behavioral science field.
3. Specialized training and competence in the
assessment, management and
treatment of Harry Benjamin Syndrome (not in
education in the treatment of Harry Benjamin Syndrome which
may include attendance at professional meetings, wokshops,
or seminars, or
participating in research related to Harry Benjamin Syndrome.
Psychological follow up is very useful for a
proper diagnosis of this condition and its physical treatment afterwards can correct it completely. The early
treatment of Harry Benjamin Syndrome can eliminate virtually all the
symptoms of the condition although
it can be reasonably assumed that having suffered the long terms effects
of untreated Harry Benjamin Syndrome may result in the need for some proper and effective counseling to eliminate or diminish
the lingering and possibly damaging after effects.
In order to properly realign the person’s body
to its proper brain sex the treatment will include Hormonal Replacement Therapy (HRT) and Sex Affirmation Surgery (SAS).
Most of the medical community of today is perfectly prepared to successfully treat Harry Benjamin Syndrome but
is still lacking the quality diagnosis caused by the lack of information and research updates about this condition therefore
leaving doctors equating treatment with the myths of the past.
It is advisable to visit an endocrinologist and give him or her up to date
information about Harry Benjamin Syndrome, this way you will be opening the door to receive more objective treatment. In most
cases, it is difficult to give a diagnosis before late infancy or pre-adolescence, although countries like the Netherlands
are very advanced in diagnosing and treating this syndrome. Thanks to the hard work of Cohen-Kettenis, people living in the
Netherlands are able to start the treatment before puberty.
It’s important to remind everyone that Harry Benjamin Syndrome is
a physiological condition and not psychiatric, even though the help of a psychologist can be very useful for the patient,
especially the younger ones in the initial evaluation and proper diagnosis. The treatment of this condition includes HRT and SAS. Visiting a psychiatrist or a therapist for evaluation should be required
but not as a long-term treatment parameter unless other issues not related to HBS exist. Then if the patient is diagnosed
as having HBS then the patient should be referred to an endocrinologist for hormone preparation for SAS followed by referral
to a competent surgeon.
follow up (other than for diagnosis purpose only) and any kind of psychiatrist treatment are strongly contraindicated for
patients with HBS unless there are other issues that need to be resolved.
and HY antigen testing are recommended. Genotype and phenotype match in the vast majority of cases, and HY antigen should
be negative in most females with HBS, and it should be positive in most males with HBS –as happens with most men without
Sex Hormones levels should also be tested. Slight variations from
the average levels are often found. Occasionally greater variations are noticed but they are less frequent.
Additional physical features such as hypogonadism
should be noted as well as any other observed morphologic characteristics that might match the true sex of the person (the
brain sex) prior to beginning HRT. As an example, many girls with Harry Benjamin Syndrome have clearly feminine physical forms
and bone structure prior to starting HRT. These are considered secondary indicators and although often found they should not
be used per se as criteria for a diagnosis of HBS. Their occurrence does add to the evidence that HBS is a physical, rather
than mental condition.
IX. Treatment of HBS in Children.
The task of the child-specialist mental health professional is to provide
careful diagnostic assessments of children with HBS. This means that the individual child's gender identity and gender role
behaviors, family dynamics, past traumatic experiences, and general psychological health are separately assessed. A pediatric
endocrinologist can then start Hormone Replacement Therapy in the child after 6 months of the initial diagnosis of HBS, but
only in those cases with persistence and consistence of the feeling of body incongruity for longer than 6 months. Sex Affirmation
Surgery (SAS) can be considered then to correct the physical problem but the child should wait until he/she is at least 12
or 13 years old (depending on each individual development) to enter the following diagnostic of “HBS in Adolescents”
in order to apply for SAS. –Given the lack of biological testing for HBS we should be very cautious with early surgeries.
For the surgical procedure itself it is better to be done after the body and genitals had reached the adolescence period as
well –sometimes the pre-adolescence period.
X. Treatment of
HBS in Adolescents.
As soon as HBS is diagnosed in teenagers there is growing
clinical experience in administering sex hormone blocking agents prior to age 16 to delay the somatic changes of puberty.
HRT can be started immediately
after diagnosis of HBS. Sex Affirmation Surgery can and should be considered after one year of the initial HBS diagnosis.
To qualify for this treatments the adolescent patient should meet
(1) Throughout childhood they
have demonstrated an intense pattern of body’s sexual identity’s incongruity and aversion to
expected gender role behaviors;
(2) Sexual identity discomfort has significantly increased with the onset
(3) The family consents and participates in the corrective therapy.
Hormonal treatment should be conducted in two phases. In the initial phase
females should be provided an antiandrogen (which neutralize testosterone effects only) or an LHRH agonist (which stops the
production of testosterone only), and males should be administered sufficient androgens, progestins, or LHRH agonists (which
stops the production of estradiol, estrone, and progesterone) to stop menstruation. After these changes have occurred and
the adolescent's mental health remains stable, females may be given estrogenic agents and males may be given higher masculinizing
doses of androgens. Medications used in the second phase, estrogenic agents for females and high dose androgens
for males, produce irreversible changes.
XI. Treatment of
HBS in Adults.
diagnosis of HBS is complete, HRT can be immediately started. Psychological follow up should be then stopped. Long-term psychological
care or psychiatrist care based on HBS alone is contraindicated. One year
after the initial diagnosis of HBS, SAS can be done if the patient meets the requirements for surgery noted below.
Hormone Replacement Therapy plays an important role in the anatomical and psychological sex affirmation
process for properly selected adults with HBS. These hormones are medically necessary for rehabilitation in their affirmed sex. When physicians
administer androgens to males and estrogens, progesterone, and/or testosterone-blocking agents to females, patients feel and
appear more like members of their true sex.
After a thorough medical
history, physical examination, and laboratory examination, the physician should again review the likely effects and side effects
of this treatment, including the potential for serious, life-threatening consequences. The patient must have the cognitive capacity to appreciate the risks and benefits of
treatment, have his/her questions answered, and agree to medical monitoring of treatment. In the absence of any other medical,
surgical, or psychiatric conditions, basic medical monitoring should include:
serial physical examinations relevant to treatment effects and side effects, vital sign measurements before and during treatment,
weight measurements, and laboratory assessment. For those receiving estrogens,
the minimum laboratory assessment should consist of a pretreatment free testosterone level, fasting glucose, liver function
tests, and complete blood count with reassessment at 6 and 12 months and annually
thereafter. A pretreatment prolactin level should be obtained and repeated at 1, 2, and 3 years. If hyperprolactemia does
not occur during this time, no further measurements are necessary. For those receiving androgens, the minimum laboratory assessment
should consist of pretreatment liver
function tests and complete blood count with reassessment at 6 months, 12 months, and yearly thereafter. Yearly palpation
of the liver should be considered. Patients should be screened for glucose intolerance and gall bladder disease. Females undergoing estrogen treatment
should be monitored for breast cancer and encourage in engage in routine self-examination.
As they age, they should be monitored for prostatic cancer. Males who have undergone mastectomies who have a a family history
of breast cancer should be monitored for the disease.
treatment, when medically tolerated, should precede any genital surgical interventions. Satisfaction with the hormone's effects
consolidates the person's identity as a member of her/his gender. Dissatisfaction
with hormonal effects may signal ambivalence about proceeding to surgical interventions.
Hormones alone often generate adequate breast development, precluding the need for
Requeriments for Sex Affirmation Surgery and Breast Surgery:
1. Six months on Hormone Replacement Therapy after HBS diagnosis.
One year of successful continuous full time living in her/his affirmed sexual identity.
3. Demonstrable knowledge of
the cost, required lengths of hospitalizations, likely complications, and post surgical rehabilitation requirements of various
4. Awareness of different competent surgeons.
5. A physician consent
form for surgery.
Sex Affirmation Surgery is the most important and effective treatment to
correct the underlying problem of HBS. The surgeon should be a urologist, gynecologist, plastic surgeon or general surgeon,
and Board-Certified as such by a nationally known and reputable association. The surgeon should have specialized competence
in genital reconstructive techniques as indicated by documented supervised training with a more experienced surgeon. Even
experienced surgeons in this field must be willing to have their therapeutic skills reviewed by their peers. Willingness and
cooperation with peer review are essential. This includes attendance at professional meetings where new ideas about techniques
Ideally, the surgeon should be knowledgeable about more than one of the surgical
techniques for genital reconstruction so that the surgeon will be able to choose the ideal technique for the individual patient's
anatomy and medical history. When surgeons are skilled in a single technique, they should so inform their patients and refer
those who do not want or are unsuitable for this procedure to another surgeon.
Prior to performing any surgical procedures, the surgeon should have all
medical conditions appropriately monitored and the effects of the hormonal treatment upon the liver and other organ systems
investigated. This can be done alone or in conjunction with medical colleagues. Since pre-existing conditions may complicate
genital reconstructive surgeries, surgeons must also be competent in urological diagnosis. The medical record should contain
written informed consent for the particular surgery to be performed.
Genital, Breast, and Other Surgery for the Female Patient.
Surgical procedures may include orchiectomy, penectomy, vaginoplasty and augmentation mammaplasty. Vaginoplasty requires
both skilled surgery and postoperative treatment. The three techniques are: penile skin inversion, pedicled rectosigmoid transplant,
or free skin graft to line the neovagina. Augmentation mammaplasty may be performed prior to vaginoplasty if the physician
prescribing hormones and the surgeon have documented that breast enlargement after undergoing hormonal treatment for two years
is not sufficient for comfort in the social gender role. Other surgeries that may be performed to assist feminization include:
reduction thyroid chondroplasty, suction-assisted lipoplasty of the waist, rhinoplasty, facial bone reduction, face-lift,
and blephoroplasty. Voice modification surgery: Patients who elect this procedure should
do so after all other surgeries requiring general anesthesia with intubation are completed to protect their vocal cords.
Breast and Genital Surgery for the Male Patient.
Surgical procedures may include mastectomy (chest reconstruction), hysterectomy,
salpingo-oophorectomy, vaginectomy, metoidioplasty, scrotoplasty, urethroplasty, and phalloplasty. Current operative techniques
for phalloplasty are varied. The choice of techniques may be restricted by anatomical or surgical considerations. If the objectives
of phalloplasty are a neophallus of good appearance, standing micturition, sexual sensation, and/or coital ability, the patient
should be clearly informed that there are both several separate stages of surgery and frequent technical difficulties which
require additional operations. Even the metoidioplasty technique, which in theory is a one-stage procedure for construction
of a microphallus, often requires more than one surgery. The plethora of techniques for penis construction indicate that further
technical development is necessary. Patients may undergo hysterectomy and salpingo-oophorectomy prior to phalloplasty. The
mastectomy procedure is usually the first surgery performed, but for some patients it is the only surgery undertaken. When
the amount of breast tissue removed requires skin removal, a scar will result and the patient is informed.
Long-term follow-up with the surgeon is recommended in all patients
to ensure an optimal surgical outcome. Surgeons who are operating on patients who are coming from long distances should include
personal follow-up in their care plan and then ensure affordable, local, long-term aftercare in the patient's geographic region.
Postoperative patients may also incorrectly exclude themselves from follow-up with the physician prescribing hormones, not
recognizing that these physicians are best able to prevent, diagnose and treat possible long term medical conditions that
are unique to the hormonally and surgically treated. Postoperative patients also have general health concerns and should undergo
regular medical screening according to recommended guidelines.
HBS along with other conditions.
to be developed in future drafts.
HBS along with other intersex conditions.
HBS along with other neurological
HBS along with other genetic conditions.
XIII. Artificially Induced HBS.
One situation in which the cause of Harry Benjamin Syndrome is quite clear
is the sexual mutilation of children or infants. This most often occurs with physically
intersexed infants, where the individual is operated on to make them conform more closely to a sex chosen by a doctor with
no possibility of consent or regard for their brain structure/gender indentity.
It can also occur if a male infants's penis is acidentally damaged or removed, and it is easier to surgically transform him
into a female and raise him as such than to reconstruct the penis. It is common
for people in these situations to be very unhappy with what was done to them, and revert to living according to their actual
gender later in life.