Introduction and Summary
(Ali) von Klan suffers from Swyer syndrone, shown age 25.
Swyer syndrome is a very rare medical
condition affecting about 1 in 40,000 women (estimates vary
considerably). A suffer is often initially misdiagnosed as
having the better known Androgen Insensivity Syndrome
AIS) before this is corrected. A core difference from AIS is that
the suffer has all the female sexual organs and
internal structures required to become pregnant
and have children -
Maya Posch, her website
documenting the challenges she faced as an intersex woman with
Swyer is sadly off-line. (Germany)
woman with Swyer is genetically XY (i.e. male) but has
normal female external genitalia at birth. She will almost
certainly to be brought up as a girl
and have no doubts as to her female sex and gender. At puberty
she's likely to develop a tall and rather masculine physique, but only a low
chance (c.30%) of spontaneous breast and menstruation without
A majority of suffers are now identified as such by
their teens, but also many woman with Swyer have married not knowing they have
the condition and often confidently expecting that they can get pregnant and
have children. An eventual lack of success is
likely to result in seeking medical fertilty assistance, and testing that finally
and often traumatically reveals the condition.
If a woman with
Swyer is still desperate to become pregnant and bear a child, this is
sometimes possible through the implantation of donated eggs fertilised
A Medical Description
Swyer syndrome - also called 46XY complete gonadal dysgenesis (lack of development of the gonads)
- is a condition in which people with one X chromosome and one Y chromosome (i.e. male) have a female appearance.
People with Swyer syndrome are usually raised as females, have a female
gender identity, and have typical and fully functional external female genitalia.
Internally, the vagina and fallopian tubes are usually normally formed,
but the uterus is often small and underdeveloped. The gonads (ovaries
or testes) are not functional and therefore they are infertile.
A 10-year old girl suffering from
Swyer syndrone. Click for full image. Warning:
potentially offensive picture.
Most individuals with Swyer syndrome do not experience any
symptoms of this until their early teens when they fail to enter a
female puberty and don't start having menstrual periods (menarche). In a young teenage girl, her ovaries
greatly increase their production of 'female' sex hormones such as
estrogen at puberty (age 12-13), triggering dramatic physical changes
such as breast development.
It is possible but unusual for this to happen in a girl with Swyer
syndrome without hormone replacement therapy. She lacks ovaries,
and the tiny amounts of estrogen produced by other organs is usually sufficient estrogen to trigger puberty.
The lack of estrogen also
means that the development of male traits resulting from having XY genes
is not hampered, most obviously a growth in height to 6 feet (182 cm) or
more is commonplace.
Three teenage girls diagnosed as having Swyer Syndrone. Note
the widely varying degree of breast development - roughly Tanner II, III
and IV/V. No more than one in three is likely to have reasonable
breast development naturally. Estrogen hormone treatment is often essential to trigger breast development.
Swyer syndrome is most often diagnosed age 14-15 when
a young woman seeks medical assistance due to her limited development of
secondary female characteristics and lack of any menstrual bleeding
(medically called primary amenorrhea).
Tests can then discover that she lacks ovaries
and, therefore does not have sex hormones (estrogen or progesterone)
that are required to undergo puberty. For example, Kiana from
"I was a
late bloomer. Not only was I the smallest in the class, I
didn't develop any breasts and I didn't have my period. Still,
I was confident that my puberty would get under way. I
didn't question the fact that I wasn't already falling in love or dating
boys like the other girls my age. But ... the older I got,
the harder it became to stay optimistic. I compared myself
to my older sister, who started puberty when she was twelve, and even my
seven-year-younger sister seemed to catch up with me. In the fall of 2019 (age 16),
after a series of tests, I was diagnosed with XY gonadal dysgenesis,
also known as Swyer syndrome. In January 2020, I began taking hormones [and]
my body is gradually starting to look a bit more feminine ... I've
recently started taking additional estrogen to artificially provoke
In itself, menstruation has no real function for me, but it feels nice
to know that my uterus is cleaned monthly."
Kiana, age 19. 10 cm taller and far happier
with her bady than three years earlier.
For various reasons (ironically
including preventing unwanted pregnancy!) doctors often
prescribe suffers with oestrogen in the form of the contraceptive pill.
Even this small amount is usually sufficient to apparently solve
problems such as rare and irregular menstruation.
hormone replacement therapy is started, these girls will develop
enlarged breasts, underarm and pubic hair, regular menstrual cycles and
other aspects of normal development during puberty.
A major differentiator between AIS
and Swyers is generous vs limited breast development.
Breast augmentation is often sought, as shown here.
It is then treated with hormonal
replacement therapy including replacing estrogen and progesterone that
is usually begun from puberty onward. In addition to helping with normal
development of secondary sexual characteristics, hormone replacement
therapy can also help prevent bone loss and thinning (osteoporosis)
later during life. The undeveloped 'streak' gonads are usually removed surgically because
they place affected individuals at an increased risk of developing a
The vast majority of women suffering
Swyer syndrome have a typically female appearing vulva, the only difference being the suggestion in some studies of a larger than
average clitoris. The vagina is also normal, unlike woman suffering from AIS
it doesn't lack vaginal depth and
penetrative sexual intercourse is very unlikely to reveal any problem
for either participant.
Women with Swyer can climax and
achieve orgasm during sexual activity. A 2006 psychosexual study
three women with Swyer syndrome speculated that this might be less frequent
than for cis-woman, but provided no evidence for this and only
recommended further research..
A review of the admittedly few
on-line sources suggests that women with Swyer
syndrome do seem to experience increased sexual problems and impairment
of sexual function compared to cis-women. Surgical procedures such
as a widening of the vaginal entrance seem common in this small and
Because women with Swyer syndrome lack ovaries,
they are infertile. However, through hormone management, the
uterus can achieve almost normal dimensions and is able to sustain
pregnancy. They can then become pregnant and carry a baby to term
through egg donation and subsequent assisted reproductive technology. The anatomic properties of the uterus
and surrounding structures may not permit normal dilatation and labour, so a C-section is
method of delivery..
Only a small proportion of
women Swyer become pregnant as it iss
an expensive procedure
extensive medical supervision. Also, the baby will not be genetically related to the mother, unless
donated by a close relative such as a sister..
Hayley Haynes shown pregnant and
with her twin babies -
Avery and Darcey..
A rare example may
be Hayley Haynes. Although genetically XY
male, she desperately wanted to bear children. On
24 December 2014 she gave birth to twins.
Press reports said that extensive hormonal development had stimulated the development of her
embryonic uterus, and thenn in vitroofertilization technology (IVF)
was used to make her pregnant - donated eggs were fertilized with her
husband's sperm and successfully implanted into her uterus.
Although the reports often said that she suffered from
AIS, this is very implausible as there is no record of a suffer having a
uterus. She almost certainly actually has Swyer syndrome.
Ali von Klan
Ali has made a very positive biographic video describing how age 16 she
discovered that she was intesex and how she has since coped with that.
Ten years later she is clearly an activist for LGBTI community, but in a
positive rather than confrontional way.
interesting documentary following the battle of "mother of two" Jeanne Nollman
against a "mystery disease" which proves to be Swyer Syndrome can be found here:
But it does have some problems in that it never explains that her
children are adopted and hides the fact that after initially being diagnosed with AIS
age 19, Jeanne
knew by age 25 that she suffered from Swyer