Eastern Iowa Review — Changeling

Posted on April 16, 2015 by Lianne

“Afterward, pain shadows my every move. Whenever I stop, the dull ache in my tailbone grows until it consumes each waking thought and pursues me into my dreams.” —Changeling

The inaugural issue of the Eastern Iowa Review is scheduled for an early May release. I’m delighted that a personal essay I wrote on intersex, will be included.

The literary journal’s founding editor, Chila Woychik, was gracious enough to drop by and chat about her new venture.

Lianne: Welcome! The EIR submission guidelines say, “Show us the good and glorious respites between the hurt; show us those good spaces.” I love that sentiment. Tell us a little more about your vision for the magazine. What drives you?

Chila: Coming from a non-faith childhood to an evangelical young adult and middle life experience, then to a more general faith perspective has led me to believe that there is true beauty and balance in this world, and it’s not that hard to find, especially if we approach life with a nonsectarian mindset. Further, having a child of my own has helped me grasp the importance of appropriateness in reading materials, viewing materials, etc., on impressionable minds, and how often “adult” materials are pretty worthless in the overall scheme even to adults. My goal is to try to bring that kind of truth and beauty to the world.

Lianne: If you would, give our readers a bit of a preview—what are some of the special things in this issue?

Chila: Besides your fantastically open and honest essay, we have creative writing ideas from a Pushcart Award winner, stories and nonfiction from MFA’s, MDs, those with little published writing and those with a CV as long as your arm. Of course, I’m grateful for each and every contribution but am extra pleased with our nonfiction and fiction special contributors: one is an English Language Fellow with the US State Department in Russia, and the other has been published numerous times simply because her writing is top notch literary.

Lianne: When I started sending out queries for Confessions of a Teenage Hermaphrodite, my assumption was that I should seek an agent who represented Christians. But even ‘edgy’ Christian Fiction didn’t include novels with an intersex main character.

It was about that time, I think, that I ran across an article you’d written on the issues with Christian Fiction. (Indeed, my novel has a few of the problems so prevalent in the genre.) Has the situation improved much over the past two or three years?

Chila: I just finished judging a group of middle grade / YA samples for a West Coast Christian writing contest, and I was amazed to find about half the entries were quite decent. Yes, there were the usual too-overt religious elements, but overall the good pieces were really quite good. Of course, these were only samples, the first ten pages or so, so who knows how those stories resolved and how they moved toward that resolution. I seldom read Christian fiction anymore; I don’t have time to waste on what has too often been bad writing teamed with an attempt at redemption through in-your-face preachiness. I’m still waiting for the day when serious writers who also believe in God will seek to address universal truths in a way that everyone, of faith or not, will sit up and take notice.

Lianne: A few of the agents that I met face to face—some of whom praised my writing—lost all interest once I explained that I was intersex. The Lord has called me to be more transparent. So I don’t hide what I am any longer. But that makes me more vulnerable.

On a previous occasion, you offered me a contract on a short story. While writing Changeling, I kept reminding myself of that. Yet I worried you might not want to risk publishing what I wrote. My essay seemed well outside what most literary magazines would print. Even given your statements regarding Christian Fiction. Would you share a bit more about your thoughts on determining limits for content?

Chila: I think your story is an important and poignant one. I also think many literary magazines would gladly publish it given you maintain a general, not-overly-religious, stance. For the Eastern Iowa Review, I sought strong nonfiction that was true and still beautiful with redeeming value for the general reader, the homeschooling mom and her brood to the college professor seeking something a little less traditional, a break from the norm. I guess I don’t consider “intersex” a tough topic as opposed to, say, a graphic story with sex scenes or brash profanity or crude jokes. To me, the truth of an intersex birth is a far cry from themes some would consider “adult.” Also to me, I would have gladly (though carefully) explained to my young son about genetic differences; their reality; it’s where we live. We learn about, learn from, and love people, period. There is no distinction, or should be no distinction. It is not unbiblical to be born intersex, so why should people hide it or discriminate against it?

Lianne: You’ve recently become a literary agent. Port Yonder Press publishes books. Eastern Iowa Review prints poetry, fiction, and non-fiction. You’re an editor and a prolific writer. How do you find the time to remain healthy and happy, let alone sane?

Chila: A few things changed earlier this year, primarily that Port Yonder Press will no longer publish material, though a few of our books will remain on our sales lists for the next year or two. Also, as of now, EIR is only an annual thing, if that. Re: editing – most of my work as an editor was for Port Yonder Press, so that will no longer apply. I had also managed the Zero Bone Poetry Prize, but this is our last year for that as well. I’ve trimmed my life out of a compelling desire to write, so for now I’m focusing on my personal writing journey, this new literary agenting gig, and possibly the journal. This is very doable and will allow me to follow my dreams at last. I also have a small farm full of animals, an awesome Jeep, and a few hobbies that I adore, such as hiking, boot & book shopping, and keeping up with friends and relatives: these things bring perspective and keep me grounded.

Lianne: I’m really excited about the release of Eastern Iowa Review. Where can people purchase a copy? How much of the content will be available online? When will you be open to submissions again?

Chila: Our goal is to have a link here by May 1st. If you purchase off our site, you’ll save over an Amazon purchase, so we’re hoping many folks will avail themselves of that opportunity; it will also net more proceeds for us and for the cause of good literature. Online content will be very limited, perhaps one-sixth of the total number of stories. “Changeling” will be one of the stories available online as will a couple more essays and a piece of fiction or two. We want people to read some of the best we’ve included so they’ll be encouraged to want the rest. All the stories are good, of course, but I feel we’ll have a good cross-section of styles and topics free online.

Lianne: Thanks for stopping by. May our dear Lord richly bless you. And thank you, dear lady, for your friendship.

Chila: The honor is all mine, Lianne, and your friendship has become special to me. Best to you as well!

[edited September 26th, 2015] Changeling is here.

Why Christians Should Oppose Bathroom Bills

Posted on March 12, 2015 by Lianne

Florida, Kentucky, and Texas, are considering–or have passed–bills that would limit who can use sex-specific restrooms. The stated intent is to prevent men from dressing as women and entering a restroom to expose themselves, rape someone, or commit some other crime against a woman or child. The real intent appears to be to prevent transgender people from using a bathroom at all. As a perhaps unintended consequence, the bills would also affect those of us born with an intersex condition and people who aren’t quite as masculine or feminine as most everyone else.

image from wipeouttransphobia.com

One of the Texas bills specifies that sex is determined by chromosomes, thus rejecting transgender people who have changed their legal sex. Even in another state.

There are several good reasons for Christians to reject such folly.

1) A woman with the complete form of Androgen Insensitivity Syndrome is born with XY chromosomes, female-typical genitals, and testes in her abdomen rather than ovaries and uterus. If her testes are left in place, she’ll have a normal feminine puberty, yet without menstruation. She might well live her entire life without knowing she’s intersex. The Bible would consider her a barren woman. The Texas bill would consider her male. Such is an egregious persecution of an innocent person.

2) The proponents of these bills state that the intent is to protect children from predators. Yet we already have laws that would apply to a man dressing as a woman, entering a women’s restroom, and committing a crime against someone in the restroom. Exposing oneself or raping someone is already against the law.

3) The bills would force transgender people to use a bathroom that goes against their gender, thus making bathroom occupants uncomfortable and increasing the likelihood of violence against the transgender person. Below is a photo of Buck Angel, an XX female-to-male transsexual. Legally, he’d have to use the womens’ restroom.

4) People born with intersex conditions in which their chromosomal sex doesn’t agree with their genital sex would have to use a bathroom inappropriate to their gender. From the wording, it’s likely that I would be unable to legally use either a mens’ restroom or a womens’ restroom. My karyotype is 46,XY,22qs+/45,X,22qs+, which is certainly not standard male or female.

5) One of the Texas bills actually offers a bounty for anyone finding a transgender person in a bathroom different from one that would ‘match’ their biological birth sex. This would encourage people to harass anyone whose presentation doesn’t fit a binary model of sex. A teen not quite as masculine as his peers might well find a bathroom an unpleasant place to visit. As if they weren’t already bullied enough.

6) Most states have laws that allow a legal change in sex. The Texas bill would ignore the legal sex status of a person, regardless of the place of their birth. But only when they enter a restroom. So, they’d be legally one sex outside the restroom and the other when inside. Does that really make sense to anyone?

7) The law isn’t enforceable without draconian intrusions on privacy. I’m female. I look like a woman. Is someone who doesn’t know that I’m intersex going to stop me at the bathroom door and ask for a karyotype? I think not. Only those whose appearance doesn’t fit someone’s concept of masculine or feminine is going to be harassed. So, enforcement will be selective, discriminating against those whose appearance isn’t masculine or feminine enough.

8) Come on. Really. Most bathrooms have stalls. And most female-to-male trans people aren’t going to be using the urinals. Okay? You’re not gonna see their private parts.

9) Is this really the Gospel? Would the love of Christ harass people whose only goal is to pee in peace? Even if you’re positive that transgender people are breaking God’s law (which they’re not–see Matthew 19:12), the bills would discriminate against people who aren’t transgender.

It’s clear to me that this isn’t about preventing rapes. It’s about a bigoted rejection of people who are already subject to a high suicide rate, a high murder rate, and bullying. Sorry, but what happened to compassion? As Christians, we should share the Gospel and repent of our hate.

‘If anyone says, “I love God,” and hates his brother, he is a liar; for he who does not love his brother whom he has seen cannot love God whom he has not seen.’ 1 John 4:20 (ESV)

See Hating Transgender Kids

A Westminster Divine on Intersex

Posted on June 19, 2014 by Lianne

The Westminster Assembly gathered in 1643 to restructure the Church of England.

Among those who met in the Jerusalem Chamber at Westminster was John Wallis, a mathematician and theologian who acted as secretary.

In addition to writing books on mathematics, logic, and grammar, he published several letters in defense of the doctrine of the trinity.

In one of those, An Explication and Vindication of the Athanasian Creed, while talking about the virgin birth, he goes off on a tangent for a moment and discusses intersex.

“I was about to say, (and it is not much amiss if I do) it is not much more than what (pretty often) happens amongst men, when God gives both Sexes to the same person, (such there are, and have been; and I think there is one yet living, who was first as a Woman married to a Man, and is since as a Man married to a Woman;) and what hinders then, but that God, if he please, may mingle the Effects of both these Sexes in the same Body?”

Many thanks to Miranda Threlfall-Holmes for posting the quote. Here.
She points out that “He doesn’t mention them to condemn them, but merely to underline a rhetorical point. There is not a hint of a suggestion that this is a problem – unusual, yes, but within the normal range of unusual events. Intersex, he says, happens ‘pretty often’, and is God-given.”

#intersex #gender #Christian

The Church and Differences of Sex Development

Posted on June 7, 2014 by Lianne

An open letter to the Southern Baptist Convention.

The Southern Baptist Convention(SBC) is being encouraged to consider A Resolution on Transgender when it meets in Baltimore on June 10th and 11th. Rather than present Scripture passages which address gender identity or differences of sex development, the authors have taken the approach the disciples took with the man born blind.

“His disciples asked him, ‘Rabbi, who sinned, this man or his parents, that he was born blind?'” (John 9:2)

The introduction to the resolution condemns anyone with a gender identity at variance with their biological sex. The proposed resolution also condemns parents who support their children by seeking the only effective treatment currently offered.

According to Denny Burk, one of the resolution’s authors, The Baptist Faith & Message 2000 says that “the gift of gender is…part of the goodness of God’s creation” Indeed, gender identity is an integral part of who we are. Yet the resolution treats gender identity as something learned. It assumes that a person’s gender identity can be brought into alignment with genital sex through the Christian process of repentance.

The resolution is aimed at those with a “transgender identity,” but it has implications for those of us born with a difference of sex development. Since Mr. Burk presented only general Scripture passages to support the resolution, I’d like to call his attention to some verses that actually deal with differences of sex development directly.

Barren Women

Genesis 11:30, Genesis 25:21, Genesis 29:31, Deuteronomy 7:14, Judges 13:3-2, Isaiah 54:1-5, Psalm 113:9, Proverbs 30:16, Galatians 4:27, Hebrews 11:11

There are usually biological reasons for infertility. Many of those involve intersex.

A woman with the complete form of Androgen Insensitivity Syndrome (CAIS), for instance, might live her entire life without knowing of her condition. Even a specialist might not tell her she’s XY and has testes in her abdomen rather than ovaries and uterus. Her body can’t react to androgens, but it does convert enough to estrogen to give her a normal puberty (without menstruation). She’s the poster child for the barren woman of Scripture. Nowhere is she condemned.

‘”Sing, barren woman, you who never bore a child; burst into song, shout for joy, you who were never in labor; because more are the children of the desolate woman than of her who has a husband,” says the LORD.’ (Isaiah 54:1-5)

Eunuchs

Deuteronomy 23:1, Leviticus 21:16-23, 2 Kings 20:16-18, Isaiah 39:5-7, Isaiah 56:3-5, Jeremiah 38:7, Matthew 19:10-12, Acts 8:26-39

Matthew 19:12 lists three types of eunuchs—intersex, involuntary, and voluntary.

“For there are eunuchs who were born that way, and there are eunuchs who have been made eunuchs by others—and there are those who choose to live like eunuchs for the sake of the kingdom of heaven. The one who can accept this should accept it.” (Matthew 19:12)

Anyone born with ambiguous genitals, anyone with masculine genitals who failed to virilize at puberty, anyone whose testes were removed—all these would have been considered eunuchs.

The passages in Deuteronomy and Leviticus indicate that eunuchs, due to their physical differences, were not allowed in the temple. They were not treated as males. Yet they are nowhere condemned.

‘“To the eunuchs who keep my Sabbaths, who choose what pleases me and hold fast to my covenant—to them I will give within my temple and its walls a memorial and a name better than sons and daughters; I will give them an everlasting name that will endure forever.” (Isaiah 56:4,5)

Man’s Way

There have always been a certain number of babies born between the sexes (i.e. inter-sex). The Bible not only acknowledges their existence, it celebrates by giving them promises of a special place if their faith is in Him.

Men? Not so much.

Since the early 1950s, the treatment of those with a difference of sex development has rested of three pillars—surgery, secrecy, and shame.

Gender identity was assumed to be learned. The nature versus nurture debate had been settled in the minds of many. The theory said that intersex could be treated by early surgical intervention to eliminate genital ambiguity. Parents were instructed to never let their child find out what was done to them. Indeed, parents were sometimes not informed as to exactly what had been done. There was never to be any doubt about the child’s assigned gender. Nurture would make everything okay.

But secrets are difficult to keep, and children soon realize that there’s something so horrible about them that their parents can’t even talk about it. The result was a generation of intersex adults with a deep sense of shame about who they were—especially related to their bodies. For some, this included treatment-induced gender issues.

The John-Joan case, sometimes used to support a nurture theory of gender, was discredited long ago. Unfortunately, the deeply flawed message of nurture over nature has lingered on in much of our culture, including the church.

God’s Way

A Southern Baptist preacher once saved my life. Truman Barrow, an elderly man who bled kindness, was the pastor of a mission work in Rochester, Illinois in the late 1960s.

He could have called me queer. Being intersex, my body hadn’t developed into a man’s. He could have called me an abomination—I was, after all, in love with a boy, while trying to live as one.

At sixteen, I was suicidal. Pastor Barrow could have mocked my feminine face and voice. As others had. Or told me to be a man. Instead, he loved me as I was. That Godly man led me down Romans Road, introduced me to Jesus, and gave me a reason to live.

To him I wasn’t some despised Samaritan. I was just one more sinner in need of the grace of a forgiving God. A wayward lamb to be shepherded like any other.

I was raised for a time as a boy. I wanted to be male, sometimes more than anything else in the world. I thought that if I was good enough, if I tried hard enough, God would make me a real boy. Since I wasn’t, some terrible moral failure must have been to blame. Perhaps the reason was that I often wanted to grow up to be a wife and mother.

As a new Christian, I assumed that God would change me so I was all one sex. As in male. Boy. Instead, the mask behind which I’d hidden crumbled, and I was forced to deal with the world as me instead of someone else. That led to my choosing estrogen over testosterone—pink over blue—and a life that no longer revolved around gender.

My genetics aren’t standard XX or XY. That resulted in gonads that were messed up and failed early. My genitals were masculine in shape, small in size, and not entirely functional. I was tiny and frail as a child and had spatial issues that kept me from learning complex sports or dance moves. A small jaw gave me a feminine face.

When I was an infant, a quick glimpse between my legs would have convinced anyone I was a boy. A quick look at my face and behavior got me clocked as a girl. I’m intersex. Between. Yet a happily married woman. And, for what little it’s worth, I don’t identify as transgender.

Most who do identify as transgender haven’t been diagnosed as having an intersex condition. Most have typical sex markers (e.g. genetics, gonads, genitals) that agree with each other. Who knows why their gender identity doesn’t agree with their biological sex? Does it even matter?

The Bible doesn’t condemn cases in which the sex markers don’t agree or are incomplete. Why make an exception for gender identity? Grace and a bit of humility would seem more appropriate.

Take the time to examine the testimonies of Christians who are transgender or who have an intersex condition. Meanwhile, throw open the doors to people with biological sex differences or a gender that doesn’t meet your expectations and let the Holy Spirit sort out what needs to change in their lives. It’s okay that they’re different.

#Baptist #intersex #trans #gender

Learning to Fly

Posted on May 23, 2014 by Lianne

Back in the mid 1980s, I bought a share in a flying club that owned a Piper J-5 Cub Cruiser. They hangared the aircraft at a grass field in northern Ohio called Sunset Strip. The membership cost $800. Flight time in the J-5 was, if I recall correctly, $4 an hour plus fuel. And avgas was a lot cheaper than gasoline because the highway taxes did’t apply. So I spent every free moment in the air.

The J-5 was produced in the mid 1940s. It was a taildragger like its more famous predecessor, the Piper J-3 Cub, but had a wider front seat and a larger engine. It also soloed from in front instead of in the rear like the J-3.

My flight instructor flew helicopters for the Ohio National Guard. He wasn’t like the guys who teach in Cessna 150s. Trust me on that. I spent my first several hours of ‘flight’ time doing high speed taxi manouvers on the grass. Then on that short strip of gravel. And, yeah, that’s a pond next to it. The gravel’s where he made me land. Until it rained. Then we used the water-soaked grass.

I’ve made several emergency landings over the years, and Dave Guerra’s the reason I was able to put my Aeronca Champ down safely in a small cow pasture.

Dave tried to get me to join the Guard and learn to fly Hueys. Seriously? Well, yeah. Have you ever flown in a helicopter? Way cool. Except I was like one year too old to join.

I’m not sure it would have worked out anyway. I have a spatio-temporal deficit that kept me from learning instrument. I can fly. I can fly by instrument. What I can’t do is keep track of where I am without some visual reference. I get half way around a holding pattern and something in my little brain forgets where I am in the loop and how the loop is oriented.

So I gave up ever being an airplane flight instructor. They’re required to have an instrument rating. Instead, I saved up money for my own plane.

Sunset Strip had an active Experimental Aircraft Association (EAA) chapter. Most of the guys were either active or retired union workers–guys who could make anything if company management actually let them. Some were more interested in building their own airplanes than in flying them. So I bought a lovely homebuilt from a gentleman who was a perfectionist.

The Baby Ace is a single-seat, open-cockpit, taildragger. This one had a Continental engine and a nice wooden prop. Even leather trim and an oak dash. Only two minor problems, really. There was no way, at least not wearing a Laura Ashley dress, to get in or out of the cockpit in a dignified manner. And a single-seat aircraft meant learning to fly the Baby Ace without an instructor on board.

Intersex—A Day In The Life

Posted on April 2, 2014 by Lianne

At support group meetings and seminars, I’ve met hundreds of people who have some physical difference of sex development. Intersex, if you will. I don’t even mind the word hermaphrodite when applied to me. Whatever.

There are hundreds of things that can happen during sex differentiation and development. Some of the variations are grouped into syndromes named after the practitioner who ‘discovered’ them—Swyer, Turner, Klinefelter. Some have names a bit more descriptive—Congenital Adrenal Hyperplasia, Mixed Gonadal Dysgenesis, Androgen Insensitivity.

What I’ve never seen, however, is a condition that results in someone having both sets of genitals. Why? Because the same bit of tissue that becomes a penis in a typical male becomes a clitoris in a female. Another bit of flesh becomes labia or scrotum. Want to know what intersex bits really look like? The Quigley Scale is used for describing degrees of Androgen Insensitivity Syndrome. The Prader Scale is used for Congenital Adrenal Hyperplasia. This site has some illustrations of degrees of hypospadias. Those are the variations you find in most cases of intersex.

The condition I have is caused by having some cells with a Y chromosome and some without. That led to confusion during fetal development. Hence the differences—not just reproductive system, mind you, but heart, kidneys, brain, metacarpals, fingernails, eyes—everything.

I went to a new dentist today. One of the technicians commented on how small my jaw was. She was concerned that I might not be able to get the digital x-ray thing into my mouth. I explained that it was a genetic thing. I’m mosaic for Turner Syndrome. The condition resulted in micrognathia—a smaller than average jaw—which gave the lower half of my face female-typical proportions.

Which do you think had a greater impact?—a feminine face or genitals that weren’t quite right? The same medical condition—Disorder of Sex Development, if you must—caused both. And much more.

If you meet someone who has a difference of sex development, don’t be surprised if they’re not a two-headed monster. Or obsessed about sex. Or gender. Most of us aren’t. Really. We’re more like you than we are different.

#intersex

40 Years on HRT

Posted on March 10, 2014 by Lianne

1974—For a brief part of the Cold War I searched the ocean noise for enemy signals. I was very good at pattern recognition and minicomputers.

Armed guards watched over us during the day. The men on my team spent their nights in the barracks. The military stuck me in my own quarters, cut off from the rest of the world.

While stateside, my bike and I lived with suicidal abandon. Weaving through traffic, standing on the seat, riding sidesaddle—the only problem I had on a motorcycle was I lacked the muscles or the mass to recover from the situations my recklessness inspired.

I remember lying on my back after my last accident and wondering why I felt no pain. My bike and I had both gone airborne and tumbled down the road. Nothing was broken—other than my bike, and perhaps my pride, but I knew for a certainty that the next wreck would kill me. My Savior made it clear that I could live for him or die from my own foolishness. Time to change direction.

I’d heard of a psychiatrist in Miami who referred people to Johns Hopkins. Dr. Money and his team knew more about intersex and gender than anyone. Right? When I went to see her, though, she told me I’d need at least a year of counseling before she’d send me to Hopkins. My first step, she said, was to try having sex—as a boy—with a boy.

Rather than explain that my biology wouldn’t allow such, never mind my faith, I walked away. As I always had from physical relationships. Oh, I liked boys, alright, but sex was for people who had plumbing that functioned properly.

A few weeks after talking to the psychiatrist, I kept an appointment with an endocrinologist—maybe he’d know what to do. His main concerns were my weight—he thought I was anorexic—and my lack of hormones.

He suggested testosterone to give me a masculine puberty and anabolic steroids to help me build muscle mass. What did you expect? He was, after all, an endocrinologist.

My sexual development had gotten lost somewhere between Tanner 2 and 3. The nature of gonads in people with an XO (Turner Syndrome) cell line is that they start weak and fade away. A woman with the pure form goes through menopause before birth.

I’d always been small for my age. At nine I wore my sister’s size 6x dresses. I was the smallest in my classes until fifth grade. But I kept right on growing into my early twenties. At 5’6″, I felt like a giant. I was no longer the little kid with a cute pixie face. My fear was that male hormones would ruin the rest of me. So I told him no.

But you need hormones to remain healthy. He said that estrogen would help me gain weight and fix at least the chemical reason for my depression. I wasn’t sure that I wanted to be a grown-up woman, but female hormones would let me keep my feminine characteristics.

For the first two weeks, I threw up every day. A few of the guys I worked with swore that I’d gotten myself pregnant, but otherwise work remained pretty much the same. Well, except that the twenty pounds I gained went to my hips and breasts—a bit of an embarrassment for someone who was supposed to be a boy.

After a year, I went home to see my family. My sister thought breast development was the best thing that had ever happened to me. Dad got all mad and wanted me to try sleeping with a girl before living as one. Mom liked that I seemed happier, and changed my legal status for me.

I got a job with a different company in a different city—doing the same thing. And the people who handed out security clearances didn’t care at all that my papers now said female on them.

Turner Syndrome Boys

Posted on January 31, 2014 by Lianne

About once a month I respond to someone online who claims that only girls can have Turner Syndrome. Some suggest that Noonan Syndrome is the male equivalent. Sadly, they often get such misinformation from a genetic counselor or physician. Although Noonan Syndrome results in some of the same medical issues as Turner Syndrome, it is caused by mutations in the PTPN11, SOS1, RAF1, KRAS, NRAS and BRAF genes rather than the loss of a sex chromosome.
>>Noonan Syndrome

The Genetics of Turner Syndrome

Turner Syndrome is caused by the loss of a sex chromosome during the first few cell divisions of a human conception. Ordinarily, each cell has 46 chromosomes–22 pairs of autosomes and two so-called sex chromosomes. Usually male is 46,XY and female 46,XX. When one of the sex chromosomes is missing in all cells, the karyotype is written as 45,X.

Cells need all 46 chromosomes to function properly. Indeed, the only chromosome that a cell can live without is the Y or the second X. It is the lack of a second sex chromosome that causes the developmental issues in Turner Syndrome. Most 45,X babies are stillborn. Some speculate that all of those who survive have at least some cells with two sex chromosomes. Current technology doesn’t allow us to determine the genetic makeup of every cell in the body. A karyotype is usually based on 25 cells from a blood sample. The rest of the cells in the body may be different.

Genetic Mosaicism

When only some cells are missing the second sex chromosome, the karyotype is written as 46,XY/45,X or 46,XX/45,X or something similar. This is called mosaicism. As I said, some speculate that all Turner Syndrome babies who survive have some mosaicism.

The degree of mosiacism varies over time because the 45,X cells don’t reproduce at the same rate as the other cells. The degree varies from person to person. What matters is where the 45,X cells are during fetal development. A woman with as little as five percent 45,X cells in her blood may be born with streak ovaries and be short statured as an adult. Or she may have no medical issues related to Turner Syndrome.

Y Chromosome Mosaicism

A woman with Turner Syndrome may be missing an X or a Y chromosome. What if she has mosaicism?–and she has a Y chromosome in some cells? Her karyotype would be written as 46,XY/45,X or perhaps 45,X/46,XY. But 46,XY usually results in male. So what determines whether the mosaicism results in a boy or a girl? Again, it’s where the 45,X cells are during fetal development.

46,XY/45,X can result in a baby with male genitals, female genitals, or somewhere in between. It can result in any of the medical issues commonly associated with Turner Syndrome.
>> 45,X/46,XY Including Y Chromosome Rearrangements (Great pamphlet from Rare Chromosomes site)
>> The Phenotype of 45,X/46,XY Mosaicism
>> X/XY Chromosome Mosaicism: Turner Syndrome and Other Clinical Conditions
>> Turner’s syndrome in the male with chromosomal mosaicism
>> Turner’s syndrome in the male (Br Med J)
>> Turner’s syndrome in the male (JAMA)

I knew a boy with a 46,XY/45,X karyotype who was taking growth hormones. I know a woman with typical Turner Syndrome features who has the same 45,X percentages as the boy. On rare occasions, 46,XY/45,X identical twins have been born one male and one female.
>>Monozygotic twins discordant for sex
>>46,XY monozygotic twins with discordant sex phenotype

On A More Personal Note

My karyotype is 46,XY,22qs+/45,X,22qs+. The 22qs+ is an exceedingly rare improperly-satellited autosome. Let’s ignore that for now. Without it, the karyotype would be 46,XY/45,X. Or XY/XO for short. Turner Syndrome mosaicism.

My Turner Syndrome mosaicism gave me mildly malformed kidneys and heart valves. I’m hypothyroid. I have visuo-motor and spatio-temporal deficits. My joints were hyperextensive. (Check out my left leg in the photo above.) I have the pixie face common to Turners, due to my small jaw. I was cross-eyed as a child. My ears are set low. I was the smallest of my peer group until fifth grade. At nine, I wore size 6x clothes. And my puberty came out of a bottle.

The vast majority of children with a 46,XY/45,X karyotype are born with normal male external genitals. Of those, more than a quarter have gonadal anomalies. Some will require growth hormone. Some hormone replacement therapy for sex development.

>> The Phenotype of 45,X/46,XY Mosaicism

What was between my legs wasn’t typical female. I was raised as a boy. I wasn’t typical male either, though, and took estrogen for my sexual development. This is the nature of intersex conditions. I find it, at times, mildly amusing that some might think I could only have Turner Syndrome mosaicism after surgery to make my genitals more typical of a girl.

Another Thanksgiving

Posted on November 26, 2013 by Lianne

Eight months ago, my husband lost consciousness, fell, and fractured the base of his skull in back. He appeared to be all right when the paramedics came. He even drove home by himself. But his personality had changed in a way I’m not sure I can describe.

He insisted nothing hurt, but his face radiated pain and he wanted to sleep. After four hours of arguing with him about going to the emergency room, I panicked at the bruises that appeared between his eyes and his nose.

Some old and dear friends drove across town and helped me coerce my husband into going to Emory Johns Creek. With his examination incomplete, he pulled out his IV, tore off the EKG leads, and tried to walk out of the hospital. I refused to take him anywhere, and when the nurse opened her hand long enough to show me a phial, I nodded. A burly nurse held my husband down, and a second drugged him.

Later, when they tried to move him from ER to intensive care, he regained consciousness enough to fight his way upright. I walked into his room as two nurses were trying to stop him. It took nine of us to hold him down long enough to sedate him again. Yes, I helped restrain my husband even when he pleaded with me to let him leave.

Under sedation, he failed to maintain his airways, so they put him on a ventilator. By then, they were more certain of his injuries, but Johns Creek didn’t have round-the-clock neurological support. With my permission, they arranged to transfer him to Clifton Road as soon as a bed opened up.

I took the photo above in the Emory-Clifton Road Neuro Intensive Care Unit. The clown hat is a Styrofoam cup covering the intracranial pressure sensor they put through his skull. The bandage around his head protects the EEG sensors that monitored him for seizures. The patch on his left shoulder covers a subclavian IV line. The yellow line is a feeding tube. Another line is a blood pressure sensor they inserted into an artery(?).

I wore my husband’s wedding ring on my necklace. They had removed it from his finger before the swelling got too bad.

A number of the people who visited him in the hospital now say they didn’t think he’d make it. I never doubted. Not because my faith is strong. Yes, I prayed to a merciful father for my husband’s recovery. But a part of me rejected the reality of my husband’s situation. When people visited, I’d tell them about all the technological marvels—isn’t it cool they can drill a hole in someone’s head and insert a pressure sensor?

He spent two weeks in NICU. And yes, death hung over him for much of that time. He spent a week in ICC and another in inpatient rehab. After a month of outpatient therapy, he returned to work.

And I crashed.

Only now are the photos becoming real to me. Forever bless my Redeemer that he let me deal with it all in bits and pieces after the fact.

And let His name be praised for all those who prayed, for all those who offered a word of encouragement, who brought food, who cleaned the house. You know who you are.

Shadows Of A Broken Childhood

Posted on November 7, 2013 by Lianne

Last weekend, my best friend and I went to an early Christmas bazaar. I’m not much on decorations, but they were also selling baked goods and various knitted items.

My grandmother was one of those short and stout Scottish ladies with a pouf of white hair. She was always doing crewel work or knitting for her Methodist church ladies’ group. That’s the sort of thing I found at the sale.

Not much remains from my childhood–my baby ring, my first set of books, a set of miniature china, and a number of the things I used when helping Mom in the kitchen. Nothing remains of the ‘boy’ years. Not that I threw everything out, mind you. There was simply nothing to which I was attached.

Some of the remains of my childhood bring back pleasant memories. Like helping Mom bake for Christmas. Some only have a vague nostalgia associated with them. A scarf I found at the bazaar brought that same warmth when I first spotted it. Perhaps grandma knitted me a similar one. I don’t know. All that’s left, sometimes, are the shadows.

Lianne Simon

a Christian writing about intersex

Category Archives: Personal