Parents of XXY’s

Do I talk about parents of XXY’s, no not normally.  They have their own ideas, they are interested primarily in their own child, and what they seem to forget is the same, they are only temporary caregivers.  The XXY himself will take over from them pretty damn quickly,  in just 18 years.

What can we say about most XXY’s?  Nothing, most XXY’s are never diagnosed.  What can we say about XXY’s who have been diagnosed,  lots and lots and lots.  Are they representative of all XXY’s, who knows?

Of the XXY’s who have been diagnosed, parents, we can say your son probably will have educational difficulty to some degree.  Do you want to read it? Do I give a shit?   What about XXYY’s and XXXY’s and XXXXY’s etc….?   Yes they are going to be much more severely affected.   Is your XXY son going to be like them, probably not, most unlikely in fact.

Are all XXY’s male, no they’re not, but I’m not interested in XXY females so if you want to read about them, go somewhere else.   I don’t even know where you can go, that’s how interested I am.  Do a PubMed search or something, do your own studies.

Klinefelters’ syndrome…. no I just can’t talk about XXY without mentioning Klinefelters’ syndrome.  Nobody has ever been born with Klinefelters’ syndrome.  Klinefelters’ syndrome develops sometime after the onset of puberty in every single XXY I’ve read about, and met in person EXCEPT 1.  The medical profession won’t say an XXY with normally sized and functioning testes  (balls)  is just XXY, they will say he has Klinefelters’ syndrome, even though it is a physical impossibility.   Klinefelters’ syndrome is a form of male hypogonadism  that manifests (love that word ‘manifests’) in adolescence, and it is the symptoms of the disease Seminiferous Tubule Dysgenesis.  So parents, your XXY son is probably going to develop Klinefelters’ syndrome eventually, it’s just not guaranteed, and it is a disease.

The one instance I know about, where the XXY man had normally sized and functioning testes,  I read in a reference in a report by Dr Shirley Ratcliffe, and he was detected because of investigations for something else. He lived in Israel and he’s probably dead by now.  But as it happened once, who’s to say it won’t happen again?

Oh that disease thing seems to be a big problem for some parents, some XXY adults too. They just can’t abide the idea that some people are indeed born with diseases.  For some reason they have to believe they or their son is  not sick.  Not all sicknesses are medical in nature, some are genetic, XXY is a genetic disease.    Some diseases are psychiatric in nature, AD/HD is a psychiatric disease and is also endemic in the XXY population.

Real MAN Crap

For some reason with some XXY guys they have this weird Real MAN Crap to promote, such as they were supposedly told they can only be a real man if they took testosterone therapy? I believe that like I believe in the Tooth Fairy!

I’m a real skeptic. I need EVIDENCE of claims made, and I’m quite happy to provide evidence of claims I make. My Endocrinologist was most helpful in providing information, just not the right level of testosterone, I was treated to XY average, not enough.

I had lots of questions, more than that I did homework too. I volunteered I’d given myself a sperm test and found azoospermia. I didn’t know what I found was called ‘azoospermia’ but my description of what I did was acceptable to John as a reliable method, and he wrote of what I’d discovered in medical lingo.

22 August 1977 azoospermia

I have never met another XXY man diagnosed with Klinefelter’s syndrome who’s done his own homework. It seems like I’m the only person who was interested enough in the words “Klinefelter’s syndrome” and the genetic signature “XXY” to find out what it all meant before I saw my Endocrinologist? I knew it had something to do with sex, when you get your balls crushed by, well by that stage, at least 4 doctors, you KNOW the balls are the problem.

I am alone. I am unique. I don’t need to be bolstered up by anybody else, as my dad said “Graeme has always been a loner.” Wasn’t that so nice of him to notice. I thought he didn’t give a shit about me. It’s amazing how wrong I can be!

1977 - psych report loner

My partner says I should visit her place more, now why do I want to do that? I know I have what she wants and she’ll be by, sooner or later, for her ‘man fix!’ We had a great laugh this afternoon, after preliminaries, about all the nonsense on the internet, and in books, about me and our other friend, Tiny! Of course you know why he’s called ‘Tiny’ as he’s the tallest guy I’ve ever seen in my life, much taller than any XXY guy I’ve ever met. Tiny is XYY! All I have to do is make myself known and all these other types just gravitate towards me. I have learned, being sociable aint all it’s cracked up to be, I like being a loner.

That wasn’t true in 1977 though.

Klinefelter’s syndrome 1976 to 1977 Deadly Serious from Graeme-xxy on Vimeo.

XXY Boys should not be referred to as having Klinefelter’s syndrome

For parents out there, your children are children, this fact seems to be lost on many parents, thinking of a related matter I read a couple of days ago. I read of a mother who described her sons penis as a ‘doodle.’ I think children can get their minds and tongue around ‘penis’ more easily than ‘doodle.’ Maybe the mother does’t refer to her sons penis as a ‘doodle’ to him, maybe she was just nervous about mentioning the correct word where she was? People confident to talk about sex have no problem referring to sex organs of both sexes by their correct name, wherever they are.

Children are learning, and I know you all know that, and their first teachers are their parents, I know you all know that too. So if your son is prenatally diagnosed XXY and you decide to keep him, not kill him, I think it would be an excellent idea to learn up on genetics. Not just go along with whatever the prevailing attitude is. Actually think about it, with an analytical, mature, mind.

Go to my previous post if you want links, you can find out all you need to know there when it comes to what Klinefelter’s syndrome is, and how it got it’s name.

Bearing in mind that Klinefelter’s syndrome is the SYMPTOMS of a DISEASE, not a DISEASE in itself. So when are you parents likely to notice your XXY son is developing the beginnings of Klinefelter’s syndrome?

According to common knowledge Klinefelter’s syndrome is seen in very young boys, even babies, and there is nothing the parent can do about it. There’s a nice defeatist attitude, saves having to think about what you’re doing and and why. You’re just observers of your sons predictable life, as you’ve read about other XXY boys who appear the same. That they have 44 unique autosomes and 3 unique sex chromosomes means absolutely nothing to you. Like when you walk down the street and see other men and women you think to yourselves how all just like YOU they are!

The additional X is mostly inactivated. It’s mostly inactivated in everybody with more than 1 X chromosome, that’s all you XX mothers out there too. You can’t resign from the study of genetics just because you’re a mother. So if we look at XXX females, what SYMPTOMS of DISEASE are they named after? It must have slipped my mind, I am XXY after all and I do have a poor short term memory. Maybe I can blame that extra X? Or Klinefelter’s syndrome – the symptoms of a disease? Or my parents for me having a poor short term memory?

Getting back to XXX females, it turns out they do have noticeable educational difficulty, fancy that! They seem to have growth issues too, being slightly taller than average females, in childhood. At puberty they’re fertile, they don’t have interference in their cells preventing the development of complete puberty, and interference of their ovaries to release eggs. They don’t develop soon after the onset of puberty hypergonadtropic hypogonadism or hypogonadtropic hypogonadism. They don’t have any kind of hypogonadism. I think it’s proven, the additional X can have an effect on education and growth regardless of the sex of the person with the additional X.

So those growth issues you parents see in your XXY sons before puberty have nothing to do with Klinefelter’s syndrome, and everything to do with the fact that there is an additional X, but not in every XXY boy. In fact if you decided you could find all XXY boys by karyotyping all tall boys, you’d miss most XXY boys. Most boys who are taller than average have normal sex chromosomes. But by karyotyping ALL boys you’d find ALL XXY and XYY boys, and ALL XX boys, and ALL XXXY boys, and ALL XXXXY boys, and every other variation there is.

So why is it then that some XXY boys are more affected than others? That might have something to do with their parents? For some reason every time I chat with parents of XXY boys they all seem to assume they’re the ‘bees knees’ of parents. How can that be when they have usually never trained to be parents before they were parents? Like most parents who discover they have a child who’s different, they have to learn how to care for that child. I don’t read how the parents of these XXY boys took themselves off to parenting classes after the diagnosis. They always seem to want to be involved in genetic counselling for themselves, and be involved with support groups to compare notes with other parents, and console themselves that they’ve done nothing wrong, and never could.

Curiously Dr Johannes Nielsen (deceased) in Denmark found that of all the XXY boys who did poorly in school, who had behaviour and educational difficulty, and ‘brushes with the law’, all came from poor parenting homes. Other XXY boys who experienced educational difficulty, and emotional disturbance, were assisted by sensible parents, and their children did not end up ‘before the courts’ as they say. Parents do have a lot to do with the way in which their XXY boys learn and communicate, and their progression to adulthood.

Did I mention the 44 unique autosomes and 3 unique sex chromosomes? Oh yes I did. They all came from their parents, the genetic providers. Are the genetic providers related to all the other genetic providers of all the other XXY boys on the planet? Are all XXY males the offspring of the same family of incestuous genetic providers? I would find that somewhat hard to believe, (makes great science fiction though.) So we all have different autosomes and sex chromosomes that come from different parents, so the possibility of other genetic conditions existing in an XXY boy, that are undiscovered, is just as good as anybody else having an undiscovered genetic anomaly, or maybe even better, since they all do have an additional X chromosome. Genetics is the only area where parents can be freed from responsibility, since XXY is a random event, that cannot be predicted.

The way in which genes work is that they are said to ‘express’ like the expression of a opinion. That expression has an effect somewhere else. So since all our genes are not exactly the same as any other person on the planet, it is conceivable that genes expressing to cause good memory, do not have the intended expression and the signal is interrupted by failing genes, or non existent genes. Just because we have the same shape and number of autosomes and chromosomes does not mean the same genes on that additional X are expressing in all of us, to the same degree. It could be that the most severely affected XXY boys have more genes that escape inactivation on the additional X than most XXY boys?

One way to further settle the matter is to look at a population of persons who have no sex hormone in childhood, do they have poor short term memory? Actually no, not as a group. Maybe there are individuals with Kallmann syndrome with poor short term memory, but it is not a feature of their syndrome, that they are born with, they are hypogonadal in the womb and at birth.

It seems the only time these people have difficulty is when they fail to enter puberty properly. The initial changes of puberty do start, they just fail to continue as they have hypogonadtropic hypogonadism, where the gonadtropins are not produced to tell their gonads to start working.

So if hypogonadism is the cause of all the difficulty in XXY boys before puberty’s onset, why is hypogonadism not the cause of difficulty in childhood for Kallmann syndrome people?

Well I’m convinced:

“Nobody was ever born with Klinefelter’s syndrome. XXY pre pubertal boys cannot have Klinefelter’s syndrome. Klinefelter’s syndrome is the post onset of puberty symptoms of disease XXY men and, XXY teenage boys can develop.”

XXY boys should not be referred to as having Klinefelter’s syndrome.

Kallmann and Klinefelter’s syndromes are not the same Disorders

Author G J Lentz is proposing in his new book “House of Cards” (Amazon) that Kallmann syndrome and Klinefelter’s syndrome are conjoined, as if they can be experienced by one person at the same time, which is impossible, of course and really who would want to?

Great new news, G J Lents is altering his book cover to say Kallmann and Klinefelter’s are 2 separate conditions. He’s still going to say in his book that he was diagnosed with both of them at different times, which is why I pre purchased a copy, I can’t wait to read it, but I guess I’ll have to. And he’s not very appreciative of our protests to 2 Moon Book Publishers, we did him a favour, he’d be made a laughing stock of if he published in the original format! It seems some people just don’t know what side of their bread is buttered.

“The blurb is being changed to read ARE instead of IS and CONDITIONS instead of CONDITION to reverse the inadvertent confusion. Who knew that two little words like: is and condition would cause so much ire. It should be changed, and it will be so, but doesn’t change the fact that after being told that I did not mean to nor intend claims of “having both” you consistently spread this and argue it based on context.
GJLentz 2 days ago on XXYforLIFE’s “Preview of G J Lentz’s book “House of Cards”

Yes I admit I mustered all the support I could find from Klinefelter’s support organisations and Kallmann support organisations to encourage your book’s publisher to alter the grossly misleading description of your book. For some body who wants to be taken seriously, wants I presume, to have their book purchased and read by as many as possible, I would think you’d have made sure no such confusion could exist. It is obvious whatever genetic condition it was that affected your life is a key element of your story. It just seems illogical to me that you’d not want to make sure your information was accurate. If you really do have one (or both) of these conditions, I am surprised you find the insistence for accuracy almost an unwelcome intrusion.

All the people I know with Klinefelter’s syndrome prefer accuracy, and the ‘it’s not important’ attitude that came through from some of the people on the Kallmann syndrome support group on Facebook specifically, suggests to me you never had Klinefelter’s at all. We Klinefelter’s sufferers being much maligned since 1959 with a great need to prevent the next generation from being aborted out of existence is probably the reason we are sticklers for accuracy. Some Kallmann people on the other hand, who have no concerns of abortion before they’ve breathed air, as their genetic condition is undetectable prenatally, I understand?

I hope your new book is a great success, and you go on to more and more success.

Graeme Tucker
47XXY

The medical profession does not refer to Klinefelter’s syndrome as Intersex

You might see the medical profession regarding Klinefelter’s syndrome as an intersex condition, but I don’t. I’m referred to in medical literature as “a person…..” A PERSON, first and foremost I’m a person….. a person suffering from….. a disease, It’s called collectively as there are a great many disorders in this area, A disorder of Sex Differentiation. This is something I have, not something I am. I am a man with a sex differentiation disorder.

Now then, not really surprisingly, EVERYBODY on the planet has “differentiation” of their sex. Everybody is on that sex continuum, and yes it is the medical profession that determines where people fit on that continuum, which to me seems kinda logical really as it’s the medical profession that treats those with such severe “differentiation” that they won’t be able to happily exist in society without their assistance. And it’s not just a matter of appearance, there are other serious health issues to consider as well, like cancer, osteoporosis, and auto-immune diseases three debilitating illnesses that if they weren’t treated for prevention or cure, would kill us. This isn’t just a “lovey dovey” description of the shape and function of genitals, this is life and death!

Then there’s the influence of society on the medical profession. Yes it does happen. Everything that happens is not the medical profession dictating to parents, often it’s the parents dictating to the medical profession, the parents who are in regular, normally sex differentiated society. They don’t want their little darlings growing up being ridiculed by all the rest of society. Who would? If a child is born with cleft palate and the medical profession says “we’ll wait until the child is older to decide if s/he really wants to eat and speak properly” I can assure you the parents would not accept that decision. They would go absolutely nuts!

XXY boys have a penis when born, and testes and scrotum, and if their penis is deemed “too small” they’re given low level testosterone therapy, they’re not castrated and turned into girls! The conditions of sex differentiation that exist that bring about sex assignment surgeries are when sex is unknown, where just looking isn’t an absolute indicator.
XXY boys do not fit into that category.

This is where I have sympathy for Transsexual people, I know what genital surgery feels like when adult. If it were in any way possible for my surgeries to be performed when I was a baby, I’m sure I’d have opted for it then, if I could. But of course my differentiation disorder didn’t develop until after the onset of puberty, and there was no need to do repair surgery before adulthood. So I have nothing in common in that regard with persons born with ambiguous genitalia, even though we’re loosely joined with a medical descriptive of the disorders we suffer from.

I find it fascinating that some intersex activists argue against the use of measuring equipment to gauge the size of a clitoris or penis to determine where the infant is on the differentiation scale, yet at the same time see some XXY men use their smallness of penis as their main justification for claiming intersex. It’s seems like a contradiction. How well virilised an infant is, is the standard to determine where the child fits on the sex differentiation continuum. That’s the way it is in the womb, we all start off the same, then were virilised if there’s the right genes and testosterone is present too. Whatever the chromosomes children who are not virilised are female, and those that are partially might be considered ambiguous.

The medical profession is simply trying to determine where a person ought to be on the sex continuum, and they get a lot of criticism because they care.

Klinefelter’s syndrome and XXY are not “Intersex”

Klinefelter’s syndrome and XXY are not Intersex

Response to “intersex is NOT a disease” blog by The *F* Word 

I see you’re referring to those brain cells nuclei images, I’ve seen those before in a series of videos by Veronica Drantz PhD called “Myth & Science of Sexuality” that you can find on YouTube. Dr Drantz supports the notion of male and female and transsexual and homosexual and heterosexual brain.The argument against hers is that the study she references has never been replicated, even though others have tried, many times. It may be that there are insufficient Transsexual brains to study, or it may be that cellular structure alters as the owners of them perform different exercises.  Such as we all know that if we learn to type well, without need to look at the keyboard, the cellular structure of the brain cells that control that act become larger. In me, my brain cells in that area are definitely not well developed.  But our actions determine how well our brains cope with those actions. There may be differences in the various brains because of the way the brain is used, not our actions being dictated by our brain chemistry?

However; to argue that intersex is not a disease cannot be taken seriously.  All the people I know who claim intersex as an identity do so because they have some kind of medical issue that affects their sexual development, either primary or secondary.  I see it as ‘burying their heads in the sand‘ to say their medical issues that lead them to their identity are not diseases, they clearly are diseases.

How is Klinefelter’s syndrome, (and syndrome means “a collection of symptoms indicative of disease“), not the disease that leads them to their identity if they choose to say Klinefelter’s syndrome is an intersex condition?   I argue strenuously that Klinefelter’s syndrome is by no means intersex as it only manifests after the onset of puberty in males!  How can it possibly be that these men, who have always regarded themselves as male prior to their Klinefelter’s diagnosis, suddenly become ‘intersex’ after their diagnosis, when they never made any comments prior to their diagnosis that they didn’t really feel male?  It is always the case that this ‘change of sex’ declaration comes about post diagnosis. In my view the only way an XXY man treated for Klinefelter’s syndrome can claim he’s intersex is to assert that he’s a natural Transsexual, as he chooses his intersex identity as a result of a disease he’s treated for. Therefore intersex is indeed a disease in him.

In the blog “intersex is NOT a disease” the author uses in part the karyotype  most often associated with Klinefelter’s syndrome as a description of an intersex condition.  I regard that as somewhat an erroneous application since everybody on the planet with more than 1 X chromosome only has 1 X active.  It literally does not matter how many X’s are present only 1 is actually working.  All biological women have at least 2  X chromosomes, which are inactivated randomly, meaning the chromosome is probably from the mother and father active in a 50/50 split.  It appears men with Klinefelter’s syndrome, who often have an XXY karyotype, have non random inactivation of their X chromosomes, which may well be the cause of their wide ranging difficulties?

There is also the region on the X chromosome called the “pseudo-autosomal region” (PAR) that is active, for all people with more than 1 X chromosome, all biological women, and XXY (and variant) men. The active genes in the PAR have to date nothing to do with the development of sex in XXY males, but the influence of the non random activated genes probably do account for the difficulties XXY males experience, and the more X’s present the greater the range and degree of difficulty.  The additional X is by no means irrelevant  or “meaningless” as at least one commentator has stated as argument against my assertion that XXY is not an intersex condition either.  The additional X has dramatic, chronic medical and psychological affects on us.  It can hardly be described as “natural.”

This is just a disease I know about, as I’m treated for it, and have been for 36 years.  It is absolutely true that in high school I learned that males have XY sex chromosomes and females have XX sex chromosomes, and of course that means something to me as I have always seen myself as male, just like every other XXY man saw himself as male.  But most people do not go on to higher education and do not learn about X chromosomes, or any chromosomes and autosomes. What they learned in high school was good enough for their chosen career, which in all likelihood had nothing to do with biology or science in any way.  Then they get diagnosed with Klinefelter’s syndrome and discover their karyoptype to be XXY and they’re suddenly thrown into a state of total confusion as it had never entered their head that men can also have XX, and XXY and XXYY and XXXY and all sorts of other variations and still be male.

When we’re all born our sex is determined by our physical appearance. XXY babies with male genitals are said to be boys.  As these babies grow they develop into male children and from there teenagers to men.  After the onset of puberty these  boys seem to always develop Klinefelter’s syndrome, even though there are examples of XXY boys who never develop the syndrome.  The most common characteristic of Klinefelter’s syndrome is infertility caused by Seminiferous Tubule Dysgenesis, or malformation of the seminiferous tubules, where sperm develop and grow.  Owning to that malformation, or disease of the testes, the testes themselves fail to grow to normal size. This is caused by the malformed seminiferous tubules retarding the growth of the leydig cells, the cells that produce testosterone.  Seminiferous Tubule Dysgenesis is a slow developing disease that CAN have an effect prenatally, and CAN have an effect in childhood, but has accelerated and devastating effects in puberty. With insufficient testosterone production  after the onset of puberty, gynaecomastia can develop, along with other  secondary sex characteristics normally associated with females, especially hair growth and fat development.  Any man with low testosterone can develop similar symptoms, but the reason XXY’s develop them so early is because of the degeneration of the testes at a very young age.

How are men treated for Klinefelter’s syndrome and claiming to be intersex not burying their heads in the sand?  It is the standard mantra of intersex support organisations to maintain that ‘intersex’ is a natural variation of human.  In my opinion natural variations of human can all breed, without assistance from the medical profession.  Of the men who are infertile who do not have Klinefelter’s syndrome, would they say they are a “natural variation” of human?  I don’t think so, I don’t think they’d say the disease that makes them infertile is supposed to be. I think men with Klinefelter’s syndrome pretend to be ‘intersex’ to cover their disappointment of being infertile, as in their eyes they can’t be ‘real men’ and be infertile.

Intersex is indicative of DISEASE.  Every person with an intersex identity has a pre-existing medical condition that can only be described as a DISEASE that brings about their identity.

ManHOOD – (generic for genital development)

A very recent picture of my chromosomes and autosomes

Even though I was diagnosed with Klinefelter’s syndrome in 1976, and had all the descriptive letters from my doctors, I’d never once seen my actual karyotype. Earlier this year (2012) I had my karyotype done again as the lab that originally did it had misplaced my photograph, and they kindly agreed to do it again. I suppose deep down I was hoping it would come out different, but it didn’t, as expected in all cells examined, I’m 47,XXY.

 

Intersex is a generic term because it covers such a wide range of medical conditions, yes people they are medical conditions, or diseases, or anomalies, or abnormalities, or aneuploidies in the chromosomal sense.  Get used to it, it’s a fact of life, people HAVE diseases, not people ARE diseases.  So if you say to me you are intersex, they I interpret that as saying you ARE a disease.   The conditions covered in the medical books are all described with another phrase, “Disorders of Sex Differentiation”  which to me is a damn sight more palatable than ‘intersex’, as ‘intersex’ implies there’s something mixed up about my sex, or something representing how much I don’t know what my sex organs look like, or that they don’t fit within standard and desirable normal sex organs.   You can read below about my sex organs and how they were never considered mixed up or intermediate, or intersex.

I was diagnosed with Klinefelter’s syndrome in 1976. My visible symptoms at the time were very thin build, no musculature, no facial and sparse body hair, female pattern pubic hair, normal penile and scrotal development, small firm testes. No breast tissue was present, my span was greater than my height, I was not over 6 feet tall, merely 5’8″ and I had broader hips than chest. My complexion was very pale, anemic almost. By mid 1977 I had testicular failure and had no testosterone production from my testes. I was 18 years old. It is estimated I developed Klinefelter’s syndrome itself when I was 14 years old. I had azoospermia (no sperm in my ejaculate).

About my manhood, it is fantastic. I kid you not. XXY guys are supposed to have a small penis, some of the XXY guys claim to have a small penis. When I was first diagnosed all my doctors wanted to give me an erection as my notes say “….he has good penile and scrotal development….” and I agree. At the time I never thought I had anything special, it worked, did the job it’s supposed to do, no big deal really.

After I started taking testosterone though, at a rate to age me, I noticed substantive increase in penile length and breadth. It was amazing what the right level of testosterone can do, even in someone who has “good development.”

Of the XXY men I know who claim intersex as their identity, often they claim they were either forced to take testosterone therapy, or that the therapy didn’t work for them. For many years it didn’t work for me either, I didn’t shave over my whole face, I had no body hair, no under arm hair, almost no pubic hair and what there was of it was very thin or sparse and fine, not in any way course. In my mid 30′s I had the appearance of a 15 year old in regard to hair growth and muscle tone.

Then I decided, with my wife, to complain to my endocrinologist about my lack of masculine development. I didn’t see myself as intersex, I saw myself as immature. I was treated as immature. When involved with outside work and all the men I’m with are hairy muscular beasts, compared to me, I needed to look the part, or be ridiculed forever! I’m sure you do realise the stress of not being taken seriously, and the stress of not being able to perform physically in the working environment.

The upshot of it all was 500mg Testosterone injections weekly, however that meant 2X250mg injections, which were painful, way too painful for me, so I cut back to 250mgs testosterone weekly, and 5 years later I was fully mature, and being quite successful in my job.