Baltimore
— Cloacal exstrophy <http://en.wikipedia.org/wiki/Cloacal_exstrophy>
is the single most severe birth defect affecting children
born today. Once considered a death sentence, this rare
disease threatening one in 400,000 births, now has a 95%
survival rate for those without spina bifida
<http://en.wikipedia.org/wiki/Spina_bifida>
and hydrocephalus <http://en.wikipedia.org/wiki/Hydrocephalus>.
Children with this
disease face a myriad of complications from birth. They
have a perforated anus which prevents waste from being
eliminated and lower abdomen organs such as the bladder
and intestines that are exposed outside of the abdomen.
Additionally 50 to 70
percent of those born with cloacal exstrophy have spina
bifida. This debilitating condition prevents their spines
from closing properly. In severe cases, the spinal cord
protrudes through the back and may be covered by skin or
a thin membrane.
These and the possibility
of abnormal kidney development are all life-threatening
conditions, which must be operated on within the first 12
hours of birth.
Another symptom present,
which becomes more pronounced after infancy, is their
mutilated genitalia. For genetic males this means the
penis is only tiny nubs which are split in two. In
genetic females a similar situation occurs in which the
clitoris is split in two pieces.
Around five years of age,
surgeons traditionally have repaired the clitoris in
females, making it whole and ordinary-looking. Barring
any complications from spina bifida or hydrocephalus,
which is an abnormal build-up of cerebrospinal fluid in
the brain and frequently occurs when spina bifida is
present, females can lead normal, intellectual, sexually
active lives. They can have traditional intercourse and
give birth, the caveat being that they must deliver
through caesarean section.
The early medical
thinking regarding males was to gender-convert them into
females and make their deformed penises into clitorises.
Though both males and females with cloacal exstrophy have
normal hormone levels for their respective sex, this
gender reassignment was deemed psychologically necessary
for males because they would never be fit for intercourse
as adolescents or adults.
This gender-assignment of
males is an intricate process that involves splitting
open the pelvic bones to make a larger, more womanly
pelvis among other surgeries.
Researchers John Gearhart
and William Reiner from The Johns Hopkins University
<http://www.jhu.edu/>
questioned the logic to reassign males as females. They
presented a study of 16 males who had come back to
Hopkins for follow-up evaluations or gender-reassignment
surgeries in The New England Journal of Medicine
<http://content.nejm.org/>
"Many of the kids were
unhappy in the female gender and gender-converted or
wanted to gender-convert themselves back to males." said
Gearhart.
In their study, two of
the males had not undergone the gender reassignment
surgery. Thus, among the fourteen patients
gender-assigned as females, eight have made the switch
back to become men. Gearhart's observations of the
patients in this study, who were gender-converted to
females in their early childhood, were that "they felt
more masculine and wanted to do something about it."
Gearhart attributed much
of the gender-conversion of males to females to a lack in
the surgical techniques to make prosthetic penises for
them when they went through this procedure years ago. He
praises the reconstructive techniques which enable
patients to have phalluses that are suitable for
intercourse. Today, males who choose to remain males can
reproduce, not through intercourse, but through
fertilization of their semen which can be taken from
their testes.
While there is no known
cause for cloacal exstrophy, it is detectable in prenatal
exams. Gearhart commented that while many women do choose
to terminate their pregnancy as a result of the
screenings, "a number of patients are still being seen at
Hopkins," meaning that even with the advances in modern
medicine, this birth defect is still
problematic.
Preliminary findings have
shown that an increase in babies born with cloacal
exstrophy has occurred in women who underwent invitro
pregnancies. Although, he cautions this is not at all
conclusive and the literature has consistently stated
that there is no known cause, genetic or otherwise, for
this defect.
