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

Conversations on Facebook

Michel’le O’Brien
Originally, OII was created because of perceived problems in ISNA, such as it being focused on North America, yet primarily Anglophone, with limited acknowledgement of Hispanic speakers, and no attempt to include Francophones, of whom there are large numbers in Canada as well as Eastern USA. The main opposition was to ISNA’s support of DSD, something I am sure you would support ISNA for (apart from being in a country outside their coverage). It was only when OII became militant in its opposition to the disorder in DSD, six years ago, and ISNA shut up shop, five years ago, that people who had been members of ISNA began to join OII – as it was the only international non-condition-specific organisation in the world. Far from dinosaurs, trail-blazers at that time! As for your suggestion that therapies be denied, that is crap – we support treatment with informed consent, and that the individual has the right to choose what form that treatment should take.

Thanks for your contribution Michelle, and my reply is:

The most fascinating thing of ISNA is the name of it’s newsletter, prior to 2002, “Hermaphrodites with Attitude.” Then Chase and Dreger got involved, two academics who may or may not have DSD’s, or Dreger doesn’t for sure, anyway they steered the ISNA away from Hermaphrodite states to Intersex states, then abandoned ‘intersex’ altogether and initiated it seems against the will of the membership “Disorders of Sex Development” aka DSD’s.

Also in 2002 Dreger wrote an article about why all manner of non ‘intersex’ conditions ought to be included as ‘intersex’ using a theory not of how different diseases come about, but how those with them are treated, inferring everybody with what we now call DSD’s are dissatisfied with our medical care, and from my experience, research, case reports I’ve read, and personal accounts by XXY’s or men with Klinefelter’s syndrome, we’re not.

The problem for us has ALWAYS been getting diagnosed early enough and getting adequate hormone therapy. These are the areas we’re discriminated against as even people with severe mental retardation are not castrated, but we are effectively by the refusal to treat! Quite the opposite to what the ISNA XXY and alike members were reporting.

And an extension was added:

I see I need to qualify that last statement. Men or adult XXY’s always got sufficient hormone therapy, well sufficient to XY average at least. But teenage boys who were found to be XXY and variants were often refused hormone therapy as they had not developed the syndrome, whereas the men were full blown and suffering.

Of course for the XXYY’s being 16 and impotent was no laughing matter, and XXXY’s the same. Their learning and behaviour difficulties were, and still are, being used to deny them hormone therapy. And the adults from ISNA were promoting ideas that they had never experienced as they were not diagnosed as teenagers. They appear to be under some weird impression that their experiences are uniform, which is so far from truth you’d need to be completely naive to believe them.

In reality Michelle, I did not complete male puberty until I was 38 years old for fucks sake! This almost seems typical for XXY males! I didn’t complain when I wasn’t developing as my unaffected peers, and I was delayed anyway, and so long as I could have an erection there didn’t seem any need to make any other changes. I had surgeries willingly to improve my standing in male society, which I do not, of course, regret.

And do not let yourself be led astray, what I had done was and is quite normal for young men with Klinefelter’s syndrome. What your people refer to as ‘normalising’ surgery in the negative, we regard as ‘normalising’ surgery in the positive. We are not the same, and your people who who choose not to be treated for their Klinefelter’s syndrome are making themselves sick. And of course as adults they can choose that course of action. But the changes caused by testosterone therapy are by no means permanent for XXY males, as soon as they stop therapy they regress to a prepubertal state. Information given you by your XXY male members is wrong.

And take out the word ‘intersex’ and we’re blood brothers and sisters Michelle. Yes we all have atypical sex development, I agree. Happy now? 🙂 It’s the real issues that affect each group that is important. We don’t share the same issues, not like the XXY or Klinefelter men who regard themselves as intersex declare. They’re making up stories to fit in, claiming all manner of similarities that aren’t there. By treating us our doctors are doing us a major favour, it’s getting the treatment at the right level that’s the hard part.

But your XXY guys, all diagnosed as adults, are presenting fairy tale events. Claiming they liked their tits, and they were just complying to society, when in fact getting mastectomy if the disease is not severe enough from a doctors viewpoint is almost impossible. Or they like the rounded body shape associated with hypogonadism, but take testosterone that guarantees they won’t have that body shape! Doing a Nicky K D Chaleunphone, arguing in effect both sides of the argument at the same time!

Adults are not forced to take testosterone therapy and my doctors were not prepared to forcibly treat me way back in the late 1970’s either, I had to choose hormone therapy. All the guys who protest about testosterone therapy and take it, saying they were told they couldn’t be “real men” without it, are telling porkies, to fit in!

My Official Position on XXY/Klinefelter’s syndrome being Intersex

Originally posted to OII’s Facebook page & Curtis E Hinkle:
Hello Curtis, having now educated myself as to what OII is all about, or mostly about, I see no justification for OII to include anymore the symptoms of disease Klinefelter’s syndrome as an Intersex condition.

All arguments that KS could be considered intersex fail. Such as most of the additional X that brings about the symptoms of disease KS represents, is inactivated. It has no impact on an XXY’s life from soon after fertilisation. The only genes that do have an effect, located on the pseudo-autosomal region of the X’s, have been mapped by the Human Genome Project and none have been found to have anything at all to do with sex or gender. Indeed, in all males, the only chromosome that can be said to be a “sex” chromosome is the Y with an active SRY gene that effectively says “make a male.”

The genitalia of XXY males is “unambiguously male” at birth and the symptoms of hypogonadism seen in men with Klinefelter’s syndrome are not unique to them. Any man who has atrophied testes develops the same symptoms as men with classic KS. Therefore the only difference in the terms of the disease seminiferous tubule dysgenesis and XY men with atrophied testes is the age of onset of hypogonadism. There is absolutely nothing intersex about all men with supernumerary X chromosomes, regardless of how many additional X’s there are.

It is “folklore genetics” and psychiatric illness that causes some men with supernumerary X’s to assume they’re intersex.

Also, OII’s stated position in almost all matters to do with health care is “no intervention.” That policy might sound just and reasonable for persons born with “differences in sex” but it is not acceptable for many XXY and variant boys who regularly require educational, psychological, psychiatric, and medical interventions throughout their lives. To propose any other course and actually follow it will be detrimental to good health.

I propose that men with Klinefelter’s syndrome be regarded as any other XY transsexual man, pre-op, when it comes to describing them as “intersex.” And that a statement to that effect ought to be made in your lengthy articles about inclusions and exclusions. The only justification these men have to claim intersex is psychiatric illness.

How I was diagnosed with Klinefelter’s syndrome

So Onni Neko was diagnosed at age 35, I wonder how old he is now? I was 17 when diagnosed, I was at my 2nd job after I left school, and I was at my 1st job for 11 months before I left and went to that interview. That’s a weird interview, I had to take all my clothes off, and stand about naked with my arms stretched out, as if I were imitating a tree!

I had may balls squeezed, god that was uncomfortable. I had to see another doctor that same day, he rang me himself after I dropped a letter off at his surgery, written by the first doctor. He squeezed my balls too. You know, when you have your balls squeezed and you’re not expecting it, you remember it, well I do anyway!

The 2nd doctor said “We think you’ve got an extra chromosome, we think you’ve got Klinefelter’s syndrome.”

I’ve been studying XXY/Klinefelter’s syndrome since 1976 my year of diagnosis. That makes me an expert on my Klinefelter’s syndrome, of yours only you’re the expert!

I remember everything as if it were 37 years ago, which is was, as everything yesterday has not been saved to long term memory, and is not available for instant recall. I’ve been taking testosterone for 36 years.

I have a video about how I was diagnosed, it’s had over 18,000 hits in 2 years, it’s got lots of comments on it too.

Found Onni Neko on Facebook

Whilst reading messages on Facebook a fellow by the pseudonym of Onni Neko commented where I might comment, and I quoted what he had on his Facebook page here, italicised:

Onni Neko

Oh I thought it was you, let me quote you:

“I was diagnosed at the age of 35 with XXy, commonly called Klinefelter’s Syndrome. I have spent most of my time since then becoming an expert on the topic, because in the end no one cares about my health like I do.”

Do you have any idea about X inactivation? I’ve never seen you write about it so I’ll assume you know nothing. You know what Barr bodies are right? Well X inactivation is associated with Barr bodies, in fact that’s how they come about.

And random versus skewed inactivation, do you know about that? Have you ever wondered why some XXY’s are much more severely affected than other XXY’s? Oh and because of X inactivation you really ought to be typing xXY, to represent the amount of active genetic material on each of the chromosomes.

And PAR, do you know anything about PAR? Since you’re such an expert, I figure you do, so I won’t need to explain it to you, the pseudo-autosomal region, you have excellent information about that too, which reinforces xXY rather than XXy.

“There is a lot of scary mis-information printed about people with Klinefelter’s Syndrome, or 47,XXY, or XXY.”

Is there? Wow never knew that. What I do know is that as time proceeds information becomes more profound and decisive, not that the other information was inaccurate, it just didn’t apply to all XXY’s. But there are mentally retarded XXY’s, and really thick ones too! Some even think if they take testosterone their life will be shortened, they even think there are published reports that say so, but you and I know they’re just deluded, not reading the reports properly.

My excellent education says “There are a great many sex chromosome aneuploidies that can lead to the development of Klinefelter’s syndrome, sometime after the onset of puberty. Not all XXY’s develop Klinefelter’s syndrome, and the syndrome itself is merely the symptoms of the disease seminiferous tubule dysgenesis.”

“Thanks in part to the medical establishment treating each case like a single rare event, something shameful, to be hidden away.”

Since1976 I’ve never noticed that, write to my endocrinologist and ask him how to teach your doctors better skills. My doctors name is John W Delahunt, you’ll find him at Wellington Public Hospital, Newtown, Wellington, New Zealand. I’m sure he’ll be happy to answer any questions you might have. When I see him sometime there are Transsexual people waiting too, and women, as he treats everybody who needs his skills, and he’s very good at it.

“I hold no shame for being XXY, yet I use a pseudo-name. Hi, I’m a working professional in the world of information technology. I’m one of those people you call when your international network breaks, the heavens are falling, and it’s the end of the world as your company knows it. I like to be employable, and because of the negativity printed about this syndrome I must exercise caution on the internet.”

Is that right? I have no problems getting employed in my chosen field. I’ve been making myself publicly known since 1994. But don’t you find your attitude contradictory when you say you masquerade as a feminine entity one moment then as a masculine entity the next, as a joke on your work colleagues? Surely that kind of trickery doesn’t go down well for someone in your industry? Or maybe I have you confused with someone else?

“My apologies to those whom are intersex, and a public persona, I choose not to release my privacy at this time.”

Some people are scared gutless wonders and others aren’t, right? And some people bullshit about being experts, when they’re just drips under pressure, you know, EX (s)PERT! 🙂

Michael Noble – Australian Misguided Academic

Michael Noble on OII Australia says:

Even though it’s evident that I possess a blend of male and female chromosomes, the medical and legal professions had proclaimed that it doesn’t matter how many X chromosomes a person may have, if they possess even one Y chromosome, they are legally classified as a male. In recent years, however, this pedantic definition has been challenged, thus enabling XXY to be legitimately recognized, in some sections of the academic world, as an intersex condition.”

Ummm Michael,  Michael Noble, the ‘owner’ of a degree in Communications and Creative Writing, you’re that Michael Noble? Unless you’re completely thick you MUST realise that every XY man on the planet has a “blend of male and female chromosomes” or did sex education pass you by along with puberty?

And of course being that you’re so well educated, (except in sex),  you MUST realise that it was not the Y that gave you your male sex determination, but your physical appearance at birth! Even you admit you were not diagnosed until age 23, therefore for 23 years you were just a run of the mill, ordinary old Australian liar, like plenty of your ignorant countrymen.

We do not put an R in CAN’T we don’t even pronounce an R, you ignorant bastards* just can’t listen!  

Anyway, no matter how many X’s are present only 1 is fully active, and the only parts of the additional X’s that are active are in the PAR, the pseudo-autosomal region of the X.  Oh and every female is the same, they have two X’s and 1 is inactivated, except for the genes in the PAR.  Therefore the more PAR genes  interacting I predict the greater the degree of difficulty experienced by the affected persons.

So the definition of male chromosomally is not “pedantic” but simply “accurate.”  It’s probably your refusal to take the level of hormone associated with your sex, and the hot hot sun you experience in your barren wasteland of a country, that causes you to imagine every X is equally active, even in females!

What is postulated, that may also cause problems for XXY’s is skewed inactivation. It’s just another thing that separates XXY males from our XX female counterparts.  They have random X inactivation, but XXY males such as your totally male self Michael, probably have non random (or skewed) X inactivation.

The more we learn Michael the less impressive your argument becomes, not that it was ever “impressive” I was just being generous!  You’ll get used New Zealanders, we’re like that, generous!

Combined with the PAR genes, and skewed inactivation in XXY males, the reasons for the development of Klinefelter’s syndrome, also known as, “The symptoms of the disease seminiferous tubule dysgenesis”  has probably now been isolated?  It is not that the additional X is female in character Michael, nor that you got yours from your mother – as you claim, (although you’ve never provided evidence of your claim), but rather much more complicated reasons, reasons that are not subject to Folklore genetics!

Michael goes on to say:

“The existence of intersex people has been concealed for many decades, because they threaten the man-made laws of nature, which dictate there are two sexes: male and female. Yet, nature does not manifest in comfortable, finite boxes. Rather it exists as infinite spectra of variation. But to our modern scientific mind this variation threatens to unleash chaos. And what drives science? The desire to control nature.”

Now then Michael, that is complete drivel. The desire of science is not to control nature, but to understand the reasons why things are the way they are, and in the terms of medical science, to alleviate suffering.  If you feel controlled by the medical profession, that may well be your particular psychiatric disease showing through?  Paranoid states & hysterical syndromes are noted features in many men said to have Klinefelter’s syndrome, or as I would say, XXY men.

That pesky little additional X plays a major role in all our lives, and no matter how much you want to pretend I regard it as meaningless, in fact I regard it as the major factor that causes the under educated like yourself, to believe they are mistreated by the medical profession. However Michael, I’m well adjusted, having sought and received proper health care from the very beginning. I took my doctors advice and I do not believe the medical profession has the desires you claim, without any justification.

And again Michael continues with his paranoid ramblings:

“Eventually, the medical profession could no longer pretend that biological sex was confined to the polar binaries of male and female, but rather some infants presented characteristics of both sexes. Yet, instead of simply accepting that anatomical sex could exist as a spectrum of possibilities, the intersex variations were medicalized: thus requiring surgery and/or hormone reassignment therapies in order to ‘cure’ such ‘aberrations’ and ‘disorders’. In other words, the medical profession believed they could play god and determine which sex and gender a child would be reassigned to, and raised as. And they did this, not for the benefit of the ‘afflicted’ individual, but rather to make the medical community, parents, and society in general, feel better.”

I’m certain, completely and totally certain, the medical profession did not set about to make the lives of infants unbearable, nor was there any conspiracy to ruin your life Michael.  Have you actually read this crap you wrote Michael?  Where there are errors in anatomical sex that YOU would see Michael, if you were ever the parent of a child with ambiguity of genitalia, which I expect would be the only way YOU could see, you may well have a different opinion?

You’re not a parent, you’ll never be a parent, you have no concept of parenting and the desire of parents to have their children treated fairly in society.  I can see that some medical procedures as being unnecessary, such as circumcision, but to lump all procedures into that classification by a person such as yourself is somewhat presumptuous.  I believe the medical profession was acting with the best interests of the child in mind,  and for the most part did an excellent job.   Yes they did screw up from time to time, and most of those children had no anatomical differences in sex, until they were butchered, they WERE anatomically NORMAL.

I must remind you that you started your little rampage with the notion that gender plays no role in the determination of one’s supposed intersex status, but here you want to include gender as part of your highly emotively written diatribe.  I wonder how you would feel if you did see the untreated results in an infant that had no interventions, that was born with ambiguity of genitalia?  The indication from opinions delivered by men such as yourself, who were not diagnosed early enough with Klinefelter’s syndrome, suggests the outcomes of no intervention would be disastrous.  It’s a very difficult situation for all involved, and a wee bit of compassion for the parents and individuals concerned would be appreciated.  Blaming and shaming Michael does your argument no good at all.  People are born with disorders Michael,  look around you.

 

*”Bastards” is a term of endearment in Australian common usage lingo.