I hope that this site will be helpful for anyone out there that is struggling with intersexuality, celebrating their intersexuality, or is interested in learning about intersexual conditions in a positive, non-judgemental way. Please post questions and either the Education Administrator or another member of the intersex community will be happy to discuss topics with you.
Welcome! Please take some time to learn more about:
Androgen Insensitivity Syndrome (AIS), complete (CAIS), partial (PAIS), mild(MAIS)
What is Androgen Insensitivity Syndrome?
CLICK HERE for an in-depth overview of AIS from the AISSG
CLICK HERE for the complete Wikipedia page on AIS
Having AIS means that the body is unable to process (insensitive to) male hormones (called androgens).
AIS is a genetic condition that is caused by a recessive gene which is passed from a mother to a child. AIS or the body's inability to process androgens or male hormones (like testosterone,) affects every person differently that has this recessive gene.
Therefore it operates on a spectrum: some people with AIS are relatively responsive to androgens and have a MILD form; others whom are less responsive to the hormones have a PARTIAL form, while others are COMPLETELY unable to use the androgens.
AIS is not completely uncommon. Estimates put children born with a form of AIS between 1 in every 20,000 babies with XY chromosomes. There may be even greater numbers since these numbers only reflect medically reported and recognized cases.
These numbers probably do not take into account men that are very minimally affected with a mild form or MAIS, who might not even know that they have it. They may develop as completely 'normal' male or may have only slight issues with virilization.
A mother that is a carrier of the recessive gene for AIS is generally not affected by the gene, but may pass the gene to her children that are genetically XY. She may also have children with XX chromosomes and some might also be carriers of the recessive gene.
In each of her pregnancies there is a 1 in 4 chance she could have a:
'Normal' XY boy, or
AIS XY baby, or
'Normal' XX girl, or
Carrier XX girl
During the fetal stage of development, the body forms basic gonads that either distend and become testicles or remain within the body and become ovaries. A fetus with the recessive gene that causes AIS cannot respond to their own chromosomes properly resulting in the range of this intersexual condition, based on the individual's level of sensitivity to androgens.
Sometimes with partial AIS or PAIS it can lead to ambiguous genitalia or complications with some genital formations. Some of these individuals are diagnosed earlier in life, while others may not be and may also develop pubic and axillary hair or might possibly have an enlarged clitoris. Children with PAIS or with CAIS may also be born with bi-lateral hernias as babies.
When a child is born with complete AIS or CAIS, the baby will probably outwardly appear as a completely 'normal' female. She will typically be raised as a woman and may have internal 'gonads' that produce testosterone, which the brain alters to estrogen for her. Since male hormones are responsible for sexual virilization or facial, pubic and underarm hair, a person with CAIS may have little, if any. Some of these women do not even find out about having AIS until in their late teens or after puberty when the diagnosis is established because of a lack of menses or a period.
A person with CAIS (although XY) is destined to look female as they cannot take male hormones to change the body's appearance or thus have a 'sexual reassignment' to look male (since the body can't process them). Studies have shown that most women with CAIS (although they technically have XY chromosomes) would not typically choose to be male and are not typically confused about their gender role. Gender identity and genetics are not always one in the same; one's chromosomes do not define "WHO" they are.
Many ("XY") women with forms of AIS have a "blind" ended vagina that can range from very shallow to 3/4 the size of a 'normal' XX woman; and vaginal sexual intercourse is possible for many others with dilation or surgical lengthening, should that be their choice.
Many intersexual XY women with AIS have their internal 'gonads' removed due to an increased cancer risk. Women with CAIS do not have ovaries, fallopian tubes or a uterus and therefore cannot bear children. Many women with AIS take Hormone Replacement Therapy or HRT to replace the estrogen that is lost from removing the gonads.
Having AIS does NOT necessarily imply being inclined to identify one's self specifically with either gender, as this may also occur on a spectrum of feeling more linked to one gender rather than another, or neither, depending on the individual. Having AIS does not imply being homosexual, or having any specific sexual preference. It may not necessarily lead to having gender dysmorphia or the feeling of being "trapped" in the wrong gender. Individuals with intersex conditions might not necessarily consider sexually reassigning themselves (as transsexuals do) or desire to change appearance to become androgynous, or to "gender bend" like transvestites do.
There is debate in both the medical and intersex communities about when is the right time to (if ever) remove the internal gonads or in the cases of PAIS when there is ambiguous genitalia, when to assign a gender.
According the the US-AIS Support Group AISSG- "Based on conservative estimates of frequency, approximately 8000 women in the US and Canada have a DSD (Disorder of Sex Development) identified as Partial or Complete Androgen Insensitivity Syndrome"
CLICK HERE for the complete Wikipedia page on AIS
Having AIS means that the body is unable to process (insensitive to) male hormones (called androgens).
AIS is a genetic condition that is caused by a recessive gene which is passed from a mother to a child. AIS or the body's inability to process androgens or male hormones (like testosterone,) affects every person differently that has this recessive gene.
Therefore it operates on a spectrum: some people with AIS are relatively responsive to androgens and have a MILD form; others whom are less responsive to the hormones have a PARTIAL form, while others are COMPLETELY unable to use the androgens.
AIS is not completely uncommon. Estimates put children born with a form of AIS between 1 in every 20,000 babies with XY chromosomes. There may be even greater numbers since these numbers only reflect medically reported and recognized cases.
These numbers probably do not take into account men that are very minimally affected with a mild form or MAIS, who might not even know that they have it. They may develop as completely 'normal' male or may have only slight issues with virilization.
A mother that is a carrier of the recessive gene for AIS is generally not affected by the gene, but may pass the gene to her children that are genetically XY. She may also have children with XX chromosomes and some might also be carriers of the recessive gene.
In each of her pregnancies there is a 1 in 4 chance she could have a:
'Normal' XY boy, or
AIS XY baby, or
'Normal' XX girl, or
Carrier XX girl
During the fetal stage of development, the body forms basic gonads that either distend and become testicles or remain within the body and become ovaries. A fetus with the recessive gene that causes AIS cannot respond to their own chromosomes properly resulting in the range of this intersexual condition, based on the individual's level of sensitivity to androgens.
Sometimes with partial AIS or PAIS it can lead to ambiguous genitalia or complications with some genital formations. Some of these individuals are diagnosed earlier in life, while others may not be and may also develop pubic and axillary hair or might possibly have an enlarged clitoris. Children with PAIS or with CAIS may also be born with bi-lateral hernias as babies.
When a child is born with complete AIS or CAIS, the baby will probably outwardly appear as a completely 'normal' female. She will typically be raised as a woman and may have internal 'gonads' that produce testosterone, which the brain alters to estrogen for her. Since male hormones are responsible for sexual virilization or facial, pubic and underarm hair, a person with CAIS may have little, if any. Some of these women do not even find out about having AIS until in their late teens or after puberty when the diagnosis is established because of a lack of menses or a period.
A person with CAIS (although XY) is destined to look female as they cannot take male hormones to change the body's appearance or thus have a 'sexual reassignment' to look male (since the body can't process them). Studies have shown that most women with CAIS (although they technically have XY chromosomes) would not typically choose to be male and are not typically confused about their gender role. Gender identity and genetics are not always one in the same; one's chromosomes do not define "WHO" they are.
Many ("XY") women with forms of AIS have a "blind" ended vagina that can range from very shallow to 3/4 the size of a 'normal' XX woman; and vaginal sexual intercourse is possible for many others with dilation or surgical lengthening, should that be their choice.
Many intersexual XY women with AIS have their internal 'gonads' removed due to an increased cancer risk. Women with CAIS do not have ovaries, fallopian tubes or a uterus and therefore cannot bear children. Many women with AIS take Hormone Replacement Therapy or HRT to replace the estrogen that is lost from removing the gonads.
Having AIS does NOT necessarily imply being inclined to identify one's self specifically with either gender, as this may also occur on a spectrum of feeling more linked to one gender rather than another, or neither, depending on the individual. Having AIS does not imply being homosexual, or having any specific sexual preference. It may not necessarily lead to having gender dysmorphia or the feeling of being "trapped" in the wrong gender. Individuals with intersex conditions might not necessarily consider sexually reassigning themselves (as transsexuals do) or desire to change appearance to become androgynous, or to "gender bend" like transvestites do.
There is debate in both the medical and intersex communities about when is the right time to (if ever) remove the internal gonads or in the cases of PAIS when there is ambiguous genitalia, when to assign a gender.
According the the US-AIS Support Group AISSG- "Based on conservative estimates of frequency, approximately 8000 women in the US and Canada have a DSD (Disorder of Sex Development) identified as Partial or Complete Androgen Insensitivity Syndrome"
Some former terms for AIS have included:
Testicular Feminization Syndrome (Testicular Feminisation Syndrome) or (TFS), Feminizing Testes Syndrome (Feminising Testes Syndrome), and Male Pseudo-hermaphroditism, - all of which are no longer preferred terms since they are inaccurate and stigmatizing.
Some synonymous terms for AIS have included:
Androgen Resistance Syndrome, Morris's Syndrome (CAIS), Goldberg-Maxwell Syndrome, Reifenstein Syndrome (PAIS), Gilbert-Dreyfus Syndrome (PAIS), Rosewater Syndrome (PAIS), Lubs Syndrome (PAIS)
Karyotype=46,XY
Women with a 46,XX karyotype that have the gene for AIS in their family may not have any symptoms/notable physical effects but can possibly be a carrier for the recessive gene.
Other biological conditions that can lead to intersexuality -
XY conditions other than AIS:
5 alpha-reductase deficiency, 17-beta-hydroxysteroid dehydrogenase deficiency, XY or pure gonadal dysgenesis (Swyer Syndrome), Leydig cell hypoplasia, ovo-testes, Mixed gonadal dysgenesis, embryonic testicular regression syndrome, persistent müllerian duct syndrome, Denys-Drash Syndrome, Smith-Lemli-Opitz Syndrome,
XX conditions other than AIS:
Mayer Rokitansky Kuster Hauser (MRKH) Syndrome, Mullerian dysgenesis, vaginal atresia.
More conditions that CAN lead to intersex conditions include but are not limited to:
Congenital adrenal hyperplasia (there are different forms), Klinefelter's Syndrome-XXY, XYY Syndrome, XXYY Syndrome, Turner Syndrome-X, Triple X Syndrome, XXXX Syndrome, XXXXX Syndrome, and sometimes Mosaicism and Chimerism
Testicular Feminization Syndrome (Testicular Feminisation Syndrome) or (TFS), Feminizing Testes Syndrome (Feminising Testes Syndrome), and Male Pseudo-hermaphroditism, - all of which are no longer preferred terms since they are inaccurate and stigmatizing.
Some synonymous terms for AIS have included:
Androgen Resistance Syndrome, Morris's Syndrome (CAIS), Goldberg-Maxwell Syndrome, Reifenstein Syndrome (PAIS), Gilbert-Dreyfus Syndrome (PAIS), Rosewater Syndrome (PAIS), Lubs Syndrome (PAIS)
Karyotype=46,XY
Women with a 46,XX karyotype that have the gene for AIS in their family may not have any symptoms/notable physical effects but can possibly be a carrier for the recessive gene.
Other biological conditions that can lead to intersexuality -
XY conditions other than AIS:
5 alpha-reductase deficiency, 17-beta-hydroxysteroid dehydrogenase deficiency, XY or pure gonadal dysgenesis (Swyer Syndrome), Leydig cell hypoplasia, ovo-testes, Mixed gonadal dysgenesis, embryonic testicular regression syndrome, persistent müllerian duct syndrome, Denys-Drash Syndrome, Smith-Lemli-Opitz Syndrome,
XX conditions other than AIS:
Mayer Rokitansky Kuster Hauser (MRKH) Syndrome, Mullerian dysgenesis, vaginal atresia.
More conditions that CAN lead to intersex conditions include but are not limited to:
Congenital adrenal hyperplasia (there are different forms), Klinefelter's Syndrome-XXY, XYY Syndrome, XXYY Syndrome, Turner Syndrome-X, Triple X Syndrome, XXXX Syndrome, XXXXX Syndrome, and sometimes Mosaicism and Chimerism
Hermaphrodite VS. Intersex- the preferred and MORE ACCURATE terminology
Intersex in media
LINKS
from AISSG-UK books and written materials list for further reading
from AISSG-US links and articles page
Lessons from the Intersexed- Book by: Suzanne J. Kessler
Intersex and Identity- The Contested Self- Book By: Sharon E. Preves
Anthony Briffa article on life with PAIS
Oprah Winfrey Show episode- Growing up Intersex
The Condition: a novel- Book by: Jennifer Haigh
Middlesex- Book by: Jeffrey Eugenides
upcoming novel featuring AIS
Film:
XXY
Boys Don't Cry also on IMDB
Medical/Psycho/Social research:
THE KINSEY INSTITUTE
OUR ADVOCATE the incomparable Dr. Charmain Quigley directory of her cited publications
AIS Does not mean immediate surgery- new medical article
How Sexually Dimorphic Are We? Review and Synthesis. American Journal of Human Biology 12:151-166 (Blackless, Melanie, Anthony Charuvastra, Amanda Derryck, Anne Fausto-Sterling, Karl Lauzanne, and Ellen Lee. 2000.)
College of American Pathologists
Gene Review
E medicine
from AISSG-UK books and written materials list for further reading
from AISSG-US links and articles page
Lessons from the Intersexed- Book by: Suzanne J. Kessler
Intersex and Identity- The Contested Self- Book By: Sharon E. Preves
Anthony Briffa article on life with PAIS
Oprah Winfrey Show episode- Growing up Intersex
The Condition: a novel- Book by: Jennifer Haigh
Middlesex- Book by: Jeffrey Eugenides
upcoming novel featuring AIS
Film:
XXY
Boys Don't Cry also on IMDB
Medical/Psycho/Social research:
THE KINSEY INSTITUTE
OUR ADVOCATE the incomparable Dr. Charmain Quigley directory of her cited publications
AIS Does not mean immediate surgery- new medical article
How Sexually Dimorphic Are We? Review and Synthesis. American Journal of Human Biology 12:151-166 (Blackless, Melanie, Anthony Charuvastra, Amanda Derryck, Anne Fausto-Sterling, Karl Lauzanne, and Ellen Lee. 2000.)
College of American Pathologists
Gene Review
E medicine
AND NOW...ON TO THE BLOG POSTS:
Wednesday, April 16, 2008
To ALL Information Seekers:
I hope that this site will be helpful for anyone out there that is struggling with intersexuality, celebrating their intersexuality, or is interested in learning about intersexual conditions in a positive, non-judgemental way. Please post questions and either the Education Administrator or another member of the intersex community will be happy to discuss topics with you.
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3 comments:
Thank you for all of your hard work and dedication. You are sure to help many, many struggling folks.
As a person with PAIS, I came aross this website and at first glance, was very pleased with the information contained herein. I do have to make note, however, that the use of the word "normal" offends me because I do not think that there can be a "normal" and a "adnormal" sex of people. I am trying to point out here, that it is offense to intersexed people to be classified and compared to "normal" people.
Dear Anonymous (author of the second comment) I'm very sorry that you are offended by the syntax contained within this blog. The intent of this blog is to present and gather information about AIS. It is not to tell others how to think. Unfortunately no matter how information is presented about AIS (which directly affects the sexual classification and sometimes gender assignment of people) there may always be someone that is not happy with just how it is stated. In fairness, I have considered what you have said. Let's face it, AIS is what most would consider a GENETIC or chromosomal abnormality, BUT with that said, it does not mean that individuals with AIS in any form aren't themselves normal. It is a question of personal sensitivity to terminology. The quotation marks around the word 'normal' were meant to indicate that 'whatever normal is' genetically, AIS is somewhat outside of what most people usually anticipate with sexual and physical development. The term 'typical' was also considered for use here, yet the inverse of that term is a-typical. Unfortunately, no matter how we discuss this, one has to take personal responsibility for their own feelings about how the information affects them. As an author I have attempted to be as straightforward and sensitive to this as possible. The use of the word 'normal' on the site was and is not meant as a judgment but rather as way to simplify the explanation for those that are learning about AIS, which is the purpose of the blog. This is especially true when explaining what is typical for human development and what is not so typical. The same trouble arises in using terms like "genetically male or female, or even usual or unusual." If you look at the AISSG UK website the term 'normal' is used in the same way as it is here. I am open to suggestions on how to improve the site and just what you would propose to say instead of using the term 'normal', so please DO contact me about that and if it makes sense and is a better way to say it, the syntax will be corrected. AND as ALWAYS as an individual with PAIS, you are welcomed and encouraged to give input as well as to become involved with the further development of this site by becoming a contributing author. To do so or to continue this discussion in private with more syntax suggestions, please click the contact link on the site and email can be conducted back and forth individually. Thanks for the input already and I look forward to a good suggestion on how to improve what you don't like.
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