Sad News about Tanya Angus, Acromegaly Sufferer

I've been out of the world for a little while thanks to my surgery, so I was saddened to find out only today that Tanya Angus, an acromegaly sufferer, passed away on the 14th January. She was aged just 34, and died from heart failure and transient ischaemic attack (a "mini-stroke").

Tanya Angus had one of the most severe cases of acromegaly (a.k.a. gigantism) ever seen; it was not until her pituitary adenoma (tumour) grew to the size of a grapefruit that she was finally diagnosed. By this time, the tumour wrapped around her pituitary gland proved extremely resistant to surgical and radiotherapy treatment. Her condition caused her to grow from 5'8" at the age of 21 to 7ft tall by the time of her death, and to go from 130 to 400lbs. Just last August it seemed that injections to inhibit her growth hormone production had been successful, but by October sadly her levels of growth hormone were rising once again.

She was a well-known figure in the acromegaly community for her efforts to raise awareness of the condition, and to encourage education about and early detection of gigantism among the medical profession. Anyone reading her website, or the comments sections of the various news websites which reported her death, can't fail to notice how many of her fellow acromegaly sufferers viewed her as an inspiration.

IMFW: Skinny Minnie or Stressy Bessie?

My favourite medical fact of the past week has been that a study has indicated that men in stressful conditions are more likely to find larger women attractive. Presumably this should prove useful to husband-hunters everywhere - the more slender ladies can go on the prowl at yoga centres and juice bars while their heavier sisters can target office blocks and traffic jams... In less jolly news about things that are definitely true, a man with Parkinson's was arrested at the Olympic men's cycling road race. His illness meant that he did not "visibly appear to be enjoying the event", and this coupled with his proximity to a group of protestors apparently was enough for him to be arrested for breach of the peace.

IMFW: Where are you on the Global Fat Scale?

A bonus Interesting Medical fact of the Week this time - I've skipped a few, because with writing up my hospital trip and all, I've been updating pretty regularly without them - which is more of an Interesting Medical Do It Yourself. The BBC website has created a calculator which allows you to input your height, weight, age and gender, and then calculates your Body Mass Index (BMI). This is then compared to average BMIs for people of your age and gender in countries all across the world.

My attention was first drawn to this when my boyfriend announced that he had the BMI of someone from the Democratic Republic of Congo, which sounded pretty concerning. So of course I had to look it up. As it turns out, my own BMI is most like a woman aged 15-29 in Bangladesh. Hurrah!

Obviously BMI is a problematic calculation given that it does not take into account a person's frame size or muscle mass - and as we now know that I am in fact 36% fat it's pretty clear that in my case it is not even a vaguely accurate measurement! However, it is still a diverting way to spend two minutes, and I highly recommend it.

The Ugliest Woman In The World

Today's post was inspired by a suggestion from one of my lovely commenters - I have forgotten who (sorry!), but thank you anyway for the idea.

Mary Ann Bevan was known as the Ugliest Woman in the World. She suffered from acromegaly, a condition in which her pituitary produced too much growth hormone, which led to deformity of her face and abnormal growth, as well as headaches and joint problems. After the death of her husband, she was forced to support herself and her four children by working in the freak show at Coney Island in the 1920s.

Mary Ann Bevan

In 2006, Hallmark produced and sold a greetings card in the UK which featured a picture of Bevan and the legend: "When the screen went back, he was to always regret the words . . . ‘I’ll go for number three, Cilla.’”

Dr Wouter de Herder, a Dutch endocrinologist who was on holiday in England, recognised the photograph and complained to Hallmark. According to theis article, he said: "“I immediately recognised the photo as I had just written an article about Mary Ann Bevan... She was in several [freak] shows in England and then later in the United States, but she led a miserable, painful life. I simply don’t think its right in 2006 to use her image to create a sick birthday card. I feel that this card is insulting to all patients who suffer from the same condition.”

Ironically, his words in fact echoed those of the famous neurosurgeon Harvey Cushing eighty years earlier, who attempted to treat Mary Ann Bevan towards the end of her life. He wrote to Time magazine in 1927, complaining of the way it had made fun of Bevan: “This unfortunate woman who sits in the sideshow of Ringling Brothers [...] has a story which is far from mirth-provoking,” wrote Dr Cushing.“She, previously a vigorous and good looking young woman, has become the victim of a disease known as acromegaly . . . Being a physician, I do not like to feel that Time can be frivolous over the tragedies of disease.” Bevan died just six years later.

I've written before about the difficulties facing women with acromegaly, but I think this story really hammers the message home. In eighty years, it seems that although our ability to treat this debilitating disease has improved greatly, our attitudes to its sufferers have hardly changed.

Hallmark pulled the card from stores, but not before its communications manager issued this memorable statement: “Once we found that this lady was ill, rather than simply being ugly, then the card was [...]withdrawn immediately, as it would breach anything we would do in terms of taking the mick out of anyone who was poorly.”

Ugly yet healthy people - be warned.

The Wonderful Thing About Hormones, Is - SHUT THE HELL UP I HATE YOU

Now, as you may or may not have noticed, I am of the female persuasion. And one of the irritating things about this is that, when you get annoyed, no matter how legitimate your grievance, there are a few gentlemen dotted about the place who will always respond with "LOL IT MUST BE HER TIME OF THE MONTH HAHA WOMEN EH AND THEIR MYSTERIOUS HORMONAL PROBLEMS CAN'T LIVE WITH EM CAN'T KEEP EM ON A CONTINUOUS DIAZEPAM IV TO CORRECT THEIR CRAZY LADY ISSUES".

The trouble with this reaction is not just that it is stupid, but also that there is no way of responding to it that does not justify the accusation in the minds of the accusers. If you remain silent, it implies that you agree. If you punch them in the face, this is taken as further evidence of "hormonal imbalance", when in fact it is self-evidently the correct course of action.

But anyway, my experiences over the last year or so of undergoing treatment for my pituitary macroadenoma have certainly made me appreciate The Power of Hormones. And that's what we're going to learn about today.

So what are hormones? Obviously, as we all kind of slightly know, they're like chemically things that sort of slosh around your body, and if they get imbalanced then you go mental and start shouting at people and probably have a hot flush.

On closer examination, this sounds suspiciously like the theory of the four humours, so I turned to Wikipedia for a delightfully un-technical definition: "A hormone is a chemical released by a cell or gland in one part of the body that send out messages which affect cells in other parts of the organism… In essence, it is a chemical messenger that transports a signal from one cell to another."

The pituitary gland is a little gland at the base of the brain, and it sits around all day secreting nine different types of hormone which help to regulate growth, metabolism, water balance, lactation, aspects of preganancy and childbirth, body temperature, blood pressure and more. Given the complex feedback mechanisms required to keep all these hormones in balance, it's easy to see that when you develop a pituitary adenoma which secretes hormone(s), it throws everything completely out of whack.

Pituitary adenomas may secrete growth hormone (acromegaly), adrenocorticotropic hormone (Cushing's disease), prolactin (prolactinoma), or as in my case thyroid-stimulating hormone (TSH-oma). But then it gets complicated, because each hormone may interact with and affect the levels of other hormones as well. So, for example, in my case my body was producing too much thyroid-stimulating hormone, which caused my body to produce too much thyroid hormone, which had a knock-on effect on other hormones including prolactin and SHBG (technically not a hormone itself but a glycoprotein which affects the levels of other hormones in the body). It's basically like your body has spent ages laying out a complex pattern of dominoes on the floor, in which one starter domino needs to be flicked for them all to neatly fall over, and then some buffon comes tramping in wearing giant boots and stomps right in the middle of them. Lots of the dominoes fall over, some don't, and it's a bloody nightmare trying to set it all up again.

So, this time last year, my hormones were completely messed up and I was experiencing all kinds of crazy symptoms; rapid heartrate, hair falling out, massive appetite etc etc. And then I started treatment with lanreotide injections - the same injections that I'm having at the moment - and started to see an improvement in my TSH levels, which continued for several months and has only just got worse again (damnit!).

I already knew about The Power of Hormones. When your heart's going at 140bpm it's hard to ignore. But I hadn't really been prepared for the fact that when I began treatment, my changing hormone levels would affect me in more ways than simply improving my symptoms. The same thing has happened again in the past week or so since having my first injection. The first thing to arrive, just like last year, was the Crazy Appetite Swings. On Sunday, I had no appetite. I could happily have eaten nothing all day; just having a coffee in the morning made me feel really full. On Monday, I was back to normal; by Tuesday, I was completely ravenous.* There will probably be weeks of me alternately spending days completely uninterested in food, and then making like the Very Hungry Caterpillar and eating my way through anything placed in front of me, up to and including my own desk.

But the Crazy Hunger Swings are not so crazy as the Crazy Cat Lady Mood Swings. I guess I am fortunate in that my mood swings haven't tended too much towards the angry thus far,** but rather to the excessively emotional. For example, yesterday I was walking home from work, listening to my ipod as per usual, when all of a sudden and to my intense embarassment I burst into tears at a Beyonce song. I'm not going to say which song, out of shame, but the fact that I had this sudden surge of emotion struck me as rather hilarious and I then started giggling, so I was walking home laughing and weeping like a complete maniac and generally very glad I didn't see anyone I knew en route. I'm going to try my best not to adopt hundreds of cats and live in a bin liner, but at the rate I'm going the position of local madwoman may well be within my reach very shortly.

After a few more weeks of treatment, hopefully my hormones should be more in balance, but until that happy day every time my eyes well up with tears at the sight of a cute piglet in a teacup, I shall remember The Power Of Hormones.

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*Admittedly not ravenous enough to eat the horrific curried parsnip soup I had for lunch at work, though.

**Although last year I would occasionally find myself becoming suddenly and irrationally furious if someone, say, dared to walk in front of me on the pavement. Happily, as soon as I recognised I was being silly the feeling would go away.***

***And if that didn't work, I used to just push them into the path of oncoming traffic.

Women With Acromegaly

After yesterday's post, I wanted to write about women with acromegaly,* as pretty much all of the pituitary adenoma sufferers I've identified so far have been men. In terms of acromegaly, I suppose that great height for a man may be seen as an advantage for certain roles as an actor or sportsman, and the physical symptoms of acromegaly - such as enlargement of the hands and feet, jaw, forehead and so on - are perhaps less likely to cause adverse comment in a man than a woman.

But I admit, I considered subtitling this post "Where The Hell Are You?" Women with acromegaly are seriously hard to find; the Wikipedia entry of "notable cases" of acromegaly lists thirteen sufferers, who are all male. It's true that male celebrities with gigantism are, in most cases, famous for roles which they play because of their height; like many actors with disabilities or conditions such as gigantism or dwarfism, casting directors or scouts are looking at their physical attributes first, and their acting talent second. Yet there is a fair list of men with acromegaly who are famous for their roles in TV or film, or for their sporting achievements - and there are even a few male acromegaly sufferers who are famous for something completely unrelated to their height, such as Kevin Aucoin, make-up artist and the historic Pio Pico, last governor of Mexican California.

Yet the only famous women with acromegaly that I've been able to track down so far are famous for their height alone; they are record holders, they might be interviewed for the newspapers, but they don't get the acting roles that great height opens up for their male counterparts. They're not on television playing unusually tall people; it seems as though such parts are for men only. They're not playing sport. There's no female Andre the Giant or Richard Kiel; and yet, when it comes to actors with dwarfism, there are well-known female actors out there, even if they are in smaller numbers than their male counterparts.

Gigantism caused by acromegaly is an extremely rare disease, don't get me wrong - even rarer than acromegaly which develops in adulthood. But it is just as likely to occur in women as in men - so why is it that some male sufferers are able to exploit their illness in a way in which female sufferers are not? The symptoms of acromegaly, especially that which develops in youth - great height, large jaw, big hands and feet - are debilitating, but they also closely correlate with traditional physical markers of masculinity; and they're diametrically opposed to feminine physical ideals. Do women with acromegaly suffer greater discrimination than men? I'd be interested to find out.

Women With Acromegaly

Tanya Angus
Tanya Angus is an activist who raises awareness about acromegaly in the United States. She does a pretty good job of it too; she's been interviewed on the Today Show, featured on ABC News, and even made it across the pond with a story in the Daily Mail. Tanya's acromegaly has proved impossible to control with pituitary surgery or medication, and consequently she's still growing at age 33 and 6'6". After her GP consistently failed to acknowledge that there could be anything wrong, she was diagnosed only by the time that her pituitary tumor had grown to the size of grapefruit. Whilst there was some success in reducing her hormone levels in 2010 using bumper doses of Somatuline, they appear to have been creeping back up again in 2011; and in a country where there's no National Health Service, her medications and hospital treatment are hugely expensive. She speaks about the difficulties that acromegaly brings for female sufferers; she is frequently referred to as "sir" and still receives abuse even in her home town.
See her website at http://www.tanyaangus.com/

Sandy Allen
Sandy Allen was the world's tallest woman before her death at the age of 53 in 2008. Sandy was 7' 7 1/2" - for comparison, currently the world's tallest man is Sultan Kösen at 8'3" - only 8 inches taller. In her later years, Allen was restricted to using a wheelchair to get about; many sufferers of acromegaly gigantism find that as they age, their legs and back are no longer able to support their height and weight. She made appearances on film and television, and aimed to educate children about the importance of accepting differences.

Sandy Allen

Svetlana Singh
Svetlana Singh is an Indian woman with gigantism caused by acromegaly; she was featured on a Channel Four documentary called The World's Tallest Woman And Me, although at 6'8" she is significantly smaller than the record holders. Married to a man who is 6'6", their child Karan was already 3'2" aged just ten months old. Svetlana was hoping to play netball for India at the Commonwealth Games in 2010, before a knee injury which failed to heal forced her to abandon a possible sporting career.

UPDATE: I've also written about famous people with pituitary adenomas here and here, and written specifically about famous people with Cushing's Disease.
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*What is acromegaly? It's a growth condition caused by a pituitary adenoma (tumor) which releases too much growth hormone - this site has a good explanation of the condition.