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.

Nationary Pituitary Awareness Month Pituitary Awareness Quiz

Even the most ardent readers of my blog will probably have forgotten that October is National Pituitary Awareness Month. Oh yes, ladies and gentlemen - that happy time is here again! Try to contain your excitement, please.

Last year, I looked at some of the other National Months that are held in October; this year, I felt like doing something different. But pituitary glands are tricky things to raise awareness for, especially considering that approximately eighty percent of Britons can't even spell the word "pituitary", let alone point to its location in the body.*

And then it hit me.

Fortunately, I wasn't seriously injured.

Dear readers, it's been over a year since I started this blog. Some of you have been here from the start, some of you have joined me along the way, and some of you typed "piglets in a teacup" into Google and arewondering how the hell you ended up here.** Believe me, I'm wondering too. But I feel quite strongly that all of you should have increased, improved and frankly incredible knowledge of the pituitary gland as a result of reading this blog, and now you will finally have the chance to prove it. And so, I present: The National Pituitary Awareness Month Pituitary Awareness Quiz! It's not sanctioned by any healthcare professionals, and it's not recognised as a professional medical qualification anywhere, ever - but the winner will gain both bragging points, a delightful poem about how wise they are, and my eternal respect.

Here's how it's going to work: For the remainder of the month, I will post one (probably multiple-choice) question about the pituitary gland every day.*** To be in with a chance of winning, simply post your answer in the comments section. Correct answers gain you one point. At the end of the month, I will leave a day or so for anyone who wants to join in to answer all the questions, then I'll tot up the points and announce the winner and write a short praise poem about how awesome they are.

Here's the twist: correct answers gain one point. Answers (however wildly incorrect) which include something that amuses me - a pun, rhyming couplet, accompanying picture or just shameless flattery - will get two points. Because life's not fair, and neither is my pituitary gland.

If I get more than five comments before the end of the month, I will count this quiz as a wild success, so please join in!

National Pituitary Awareness Month Pituitary Awareness Quiz

Day 1 - Question 1

Q.1:  Acromegaly is a rare disease caused by a growth-hormone-secreting pituitary tumour, which can lead to gigantism and excessive growth of the body's tissues. But what language is the word "acromegaly" derived from, and what does it literally mean?

Is it:

a) from the Latin acro "extremity; member of the body" and magnus "large"

b) from the Greek arka "repository; hidden place" and megaras "growth".

c) from the Greek akros "highest; extremity" and megalos "large".

It's an easy-to-Google one to start you off. Fly, my pretties, fly!


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* Warning: spurious fact alert.

** This is actually a genuine search that somehow resulted in my blog
coming up. I have no idea why

***In theory.

Acromegaly Community

I am a little overexcited that the piece I wrote recently about Kate Woodward, the girl with acromegaly who sued the NHS, has been posted on the Acromegaly Community blog. It's an excellent website that I have frequently read in the past so I was delighted to hear from them when they asked to post the article! (Thanks, guys). It's definitely highly recommended if you have acromegaly. If you live in the US, it also has some very useful information on financial support for your prescriptions, information on discrimination in the workplace, and medical studies which are currently recruiting.

Plus, if you're passing by you can click 'like' on my post >.>

Girl with Acromegaly Sues NHS

A story broke some time ago about a young lady with acromegaly who sued the NHS, and won. I've been meaning to write about her for a while but I wanted to take the time to create an interesting and informative post contrasting her case with that of Kane Gorny. Alas, I have not had the time, so you'll just have to deal with a dull and uninformative post instead. Hurrah!

The story is this: Kate Woodward, an aspiring actress, developed acromegaly (i.e. a pituitary tumour secreting growth hormone, which lead to uncontrolled growth of her body) as a child, which went untreated between 2001 and 2005 as medics failed to spot the condition. She attained a final height of 6'5" aged 20, and sued the NHS for £2 million. She was awarded compensation of £1.3m.

Incidentally, the award for "least sensitive headline about this story" goes to Healthcare Today UK, which went with "Freak Growth Woman Sues NHS".*

Nice.

Her case for being awarded this level of compensation was that, as a result of her condition being untreated for so long:

- Her height and "ugliness" meant that she could no longer go into a career as an actress.
- She has psychological difficulties and feels like a "freak" because of her condition.
- She has significant problems with her bones, knees and teeth, and cannot buy normal-sized clothes.
- The condition affected her childhood, schooling and friendships.

And, most significantly:

- She wishes to receive all future treatment in the private sector.

Now, not long after this story was first brought to my attention (by a colleague, thank you!) I noticed that it had also popped up as a topic of discussion on the Pituitary Foundation's forum. For those of you who don't subscribe, one member created a poll entited something like "Should Kate Woodward be ashamed of herself?" The results of the poll were overwhelmingly that she should (although in fairness by "overwhelmingly", I mean "there were four votes in total").

Why the anger? Surely she has a fairly good case, right? Undoubtedly, when a doctor is presented with a girl who measured 5'9" at just eleven years old and keeps growing, you'd hope that it wouldn't take four years to just Google it and think "hmm, maybe we should rule out acromegaly". Although we can't know that faster treatment would have effected a cure, it might have controlled or reduced the symptoms. She will have to live with injections and monitoring and joint problems and feeling self-conscious for her whole life.

BUT.

The problem is that this is not, actually, an unusual case - at least, not within the magical world of pituitary tumours. If every person with acromegaly decided to sue the NHS for delayed diagnosis, it would be one hell of a bonus day for lawyers. If you threw in people with other often-missed pituitary conditions like Cushing's Disease and TSHoma, it would probably result in a sudden UK lawyer shortage as they all retired to small Caribbean islands. If you based the level of compensation awarded on the number of years the condition went undiagnosed, the small Caribbean islands would probably reinvent themselves as world financial hubs.

I exagerate. But the mean length of time from onset of symptoms to diagnosis of acromegaly is, according to this MedScape article, around 12 years. Even if that estimate is too high, the point is that pituitary tumour conditions can be hard to diagnose, and they are often missed because they're rare enough that doctors don't see them often, and often don't expect to see them either. Four years to diagnose a patient with acromegaly is, sadly, not uncommon. I first showed symptoms of my TSH-secreting pituitary tumour aged 17, and I didn't get a diagnosis until I was 22. (In case you're bad at maths, that's five years). Even after my superfast heartrate made it clear that Something Was Wrong, it took a year to work out what that something was.

So if there's a slight lack of sympathy from other acromegalomaniacs,** it could be because they're thinking "Damn, £2m! Wish I'd thought of that."

 But I doubt it.  And I'm not just saying that because they're bigger than me.***

Now I want to note right off the bat that where there is evidence of medical negligence or it takes a long time to get a diagnosis, you should complain. Vociferously. You should complain in the hope that procedures can be improved. Otherwise there's nothing to stop future patients from suffering through the same tortuous process to find out what's wrong with them. But there's a difference between seeing a problem, taking a complaint as far as necessary to fix that problem for future patients, and just suing to get as much as you can out of them. Through the normal complaints process, the hospital in question offered Miss Woodward compensation of £700k, no court appearance required. If she lives to the age of 90, that would be £10,000 medical expenses covered every year. Additionally, according to the Daily Mail, which I personally take with a pinch of salt and under the supervision of a doctor, the hospital trust had already spent £288,000 on Miss Woodward for "treatment, dental care, holidays and special footwear".

Even the £500k difference between what she was offered and what the court awarded is a big chunk of money. £500k that could have gone towards treating other patients. I need somatostatin analogue injections (probably much like those Miss Woodward takes), and if you've been reading this blog lately, you'll know that it is proving slightly tricky to persuade someone to fund them. £500k could pay for 666-and-a-bit doses of those injections. Which doesn't sound like that much, until you realise that each dose lasts for a month, so £500k worth of injections would last me for fifty-five years. And six months. Assuming I needed them continuously. And of course by that point I would be 79-and-a-half, so chances are I could be dead by then anyway.

 And here's the thing. If the NHS messes up and, as a result, you end up incurring further costs in your daily life (eg. you're a professional tap dancer going into hospital for an appendectomy, and you wake up with a leg missing) then yep, you should get compensation and if they won't agree to pay it, sue them with my blessing and encouragement. If the clinical negligence of your child at birth leads to brain damage which will affect that child for their entire life, then sue for the money needed to support them. And if/when the NHS messes up so badly that it becomes a danger to other patients, and you follow through that complaints process and nothing changes, sue them until they sit up and pay attention and mend their ways.

 But I am somewhat uncomfortable with suing the NHS for the loss of a career that did not yet exist, and for the costs of having exactly the same treatment as the NHS provides, but done privately. (I'm also intrigued by the concept, since she presumably did not have private health insurance before diagnosis and consequently insurers would be unlikely to cover her pre-existing condition?).  I've had an MRI scan done by a private provider and done by the NHS and the experience was almost exactly the same - except the private hospital was a lot harder to get to on public transport.

I guess in some way I think that when the NHS messes up, part of the compensation is the NHS. The very fact that you live in a country where you don't have to worry about the cost of healthcare means that when the NHS makes a mistake, it - not you - ends up bearing any increased costs of your care as a result of its mistake. Perhaps in some countries when a patient gets a hospital-acquired infection, their bill from the hospital is increased - but in the UK, if a hospital gives you an infection, it has to foot the bill to try and fix it.

But it also means that when you sue an NHS hospital in the UK, you're not taking lobster off the table of a fat cat investor (well... not yet... comments about Mr Lansley on a postcard, please). You are taking money away from that hospital. Any sensible hospital trust will have a big old stash of contingency fund to cover the cost of lawsuits, but I still confess I'm uncomfortable with the idea of suing the NHS for anything less than an ironclad reason, because that money could be put to better use. It could be used to help treat someone just like you.

I don't think Kate Woodward is a bad person, and I don't think she should be ashamed of herself. I simply don't have sufficient details to form an opinion on this case, and she may well be entirely justified. But she sparks off an interesting debate. That's my opinion. What's yours?

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*I noticed Jon Danzig, a journalist with acromegaly, took them to task for this in the comments section of the article, good on him!

** I don't know what the collective noun for "a group of people with acromegaly" is, but it should really be this.

***Common misconception: people with acromegaly only become very tall if the condition develops during childhood/adolescence. If it develops in adulthood, it will lead to bone and soft tissue growth but usually won't increase height much, if at all.

Acromegaly Drug Trial - Oral Octreolin

I noticed on the Pituitary Foundation's website that a drug trial is recruiting for acromegaly patients. It's the phase 3 testing of oral octreolin, taking place at centres across the UK, including Oxford, Manchester and London.

This trial is a pretty exciting prospect! Currently, the somatostatin analogues which are used to treat acromegaly are only available in an injectable form, both as daily injections and in a long-lasting preparation that's injected once a month. This means regular visits to the nurse, plus all the hassle of ordering and storing the prescription, as the drug has to be kept refrigerated. Being able to simply take two pills every day would be so much more convenient (not to mention less painful... those needles are huge), especially for patients who travel or move around a lot.

The biopharma company carrying out the trial is Chiasma, a company specialising in turning injectable drugs into oral formulations. Octreolin is their lead product, but their website indicates they're also working on a drug to treat complications of chronic kidney disease.

It appears Chiasma are conducting clinical trials on the use of octreolin to treat neuroendocrine tumours. In addition to this and acromegaly, octreotide (the injectable somatostatin analogue that octreolin emulates) is used to treat carcinoid syndrome, TSHomas/thyrotropinomas and an extremely rare tumour called a VIPoma. VIP actually stands for vasoactive intestinal peptide, a hormone which the tumour produces in excess, but these tumours are so rare - estimated annual incidence of one per ten million people - that the other meaning of VIP is strangely apposite. A press release from Chiasma also suggested that octreolin was being investigated as a treatment for portal hypertension. So if octreolin is found to be as safe and effective as octreotide, it could benefit people suffering from a whole range of conditions.

The Very Scary Case of Kane Gorny

Before going into hospital last Tuesday, I stopped off at one of my favourite cafes for a hot chocolate, as a pre-hospital treat. I wasn't allowed caffeine in the three days before going in, so I had been suffering from a woeful lack of hot drinks. I got my hot chocolate (which was delicious) and sat down by the pile of daily papers they keep at the café. Alas, all the more upmarket titles had been taken, so I had to settle for that peculiar bastion of journalistic achievement: the Daily Mail.

You can imagine my feeling of ill-omen when, immediately before going into hospital overnight for further investigations into my pituitary tumour, I saw that the Mail's headline story was about Kane Gorny, a 22-year-old with a pituitary tumour who died of dehydration in hospital after nurses denied him his medication.

You can read the Mail's story online here. According to the Mail, it seems that after his entirely routine hip replacement surgery, nurses did not give Mr Gorny medication necessary for fluid retention; he became so agitated from dehydration that he called 999 in an attempt to get some water, but was sedated and left unattended overnight, despite his mother raising concerns. Even after his death, the attitude of some members of the nursing staff appears to have been appalling.

I thought I would tackle a couple of questions raised by the reporting on this terrible story, before moving on to look at it as a whole:

What was wrong with him?

 Kane Gorny suffered from a pituitary tumour; that much is certain. The exact details of Mr Gorny's condition are still somewhat sketchy and vary depending on which paper you read, but I'm going to guess that he possibly had acromegaly or Cushing's Disease, from the statement of his endocrinologist that he had a "rare tumour", and the fact that he had joint problems.

Most papers have reported that he had a "malignant" brain tumour or "brain cancer". The tumour may well have been cancerous, but it should perhaps be noted that this extremely rare for pituitary tumours; they are sometimes misreported as malignant due to journalists misunderstanding the condition and the fact that a tumour labelled "benign" may still be very harmful. In any case, I'll be looking at the definition of malignancy/cancer with regard to pituitary tumours in a future post, hopefully within the next couple of weeks, because it's an interesting question.

Why did he need a hip replacement aged just 22?

After pituitary surgery, sometimes the healthy pituitary gland is damaged, leaving it unable to produce certain hormones, including ACTH (adrenocorticotrophic hormone), which stimulates the adrenal glands to produce the steroid hormone cortisol. Without cortisol in the body, you can die quite quickly - consequently, it is standard to give patients steroid pills after surgery until doctors are certain their pituitary gland is able to produce ACTH. If the pituitary gland has been damaged, patients will need to take these pills for life.

Most reports have stated that Mr Gorny's steroid treatment left him requiring a hip replacement; high levels of steroids in the body can lead to avascular necrosis (although not in "a couple of weeks" as one paper initially reported). Additionally, if he did indeed have Cushing's or acromegaly, both of these conditions can adversely affect joints.

How did he die of dehydration so quickly?

A healthy human can live for a couple of days without water, depending on exertion and environmental conditions. Kane Gorny could not. After his pituitary surgery, Mr Gorny was left with diabetes insipidus. This is a very different condition from what we refer to as "diabetes" (diabetes mellitus) and is caused by a deficiency in anti-diuretic hormone (ADH, or vasopressin). Anti-diuretic hormone is secreted by the pituitary gland and helps to control the body's fluid balance. In diabetes insipidus, the lack of this hormone means that the body cannot conserve much of the water which it takes in, and consequently the sufferer becomes extremely thirsty and needs to urinate frequently. Unfortunately, developing this condition is a fairly common side-effect of pituitary surgery and pituitary radiotherapy.

In order to treat his diabetes insipidus, Kane Gorny would have needed to take a drug called Desmopressin (DDAVP), which is a synthetic substitute for vasopressin. As long as he was taking this drug, his body would be able to retain a normal amount of the water he drank, and he would not become dehydrated. When the drug was witheld, his body could not remain hydrated, and he died.

How could the hospital get this so wrong?

Unfortunately this is the question that can't be answered. Kane Gorny's death appears to have been preceeded by a number of absolutely catastrophic blunders at the hospital. His endocrinologists were not informed that he was in the hospital for surgery; his surgeon was entirely unaware of his condition; nurses did not read his notes; no-one listened to his mother's concerns; the list goes on.

Diabetes insipidus is a common problem among pituitary patients, but far more rare in the general population. Endocrine and neurosurgery nurses would likely be familiar with the condition, the importance of the medication Mr Gorny was taking, and the crucial need to monitor his fluid balance. The nurses actually looking after him knew almost nothing about it.

It's a sad fact that there are a hell of a lot of medical conditions in the world. Patients with diabetes insipidus or the inability to produce steroid hormones are encouraged to wear MedicAlert jewellery to alert paramedics and medical staff to their conditions in case of emergency, yet it seems even when medical staff have access to full notes on a patient they can go unheeded. 

Sometimes it gets frustrating when you're in hospital or go to the doctors and are asked for the thousandth time to explain what's wrong with you. In the future I'll try to be more grateful that someone is checking...

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Kane Gorny's brother is fundraising for CLIC Sargent. You can donate here.

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.

Double-Edged Sword

I've written a fair bit about acromegaly in the past - caused by a pituitary tumour secreting high levels of growth hormone, it can lead to gigantism if it occurs in childhood. But you may not have heard of pituitary dwarfism, which is effectively the opposite of acromegaly - although there's usually no tumour involved - in which the pituitary gland fails to secrete enough growth hormone. If a child with pituitary dwarfism is left untreated, this can lead to permanently stunted growth.

Recently the world's tallest man, Sultan Kosen, has been in the news; his growth, caused by a pituitary adenoma, has finally been brought under control after surgery and medical treatment. Even so, he stands over 8 feet tall. In contrast, Special Olympics athlete Mackenzie MacDonald who has also been in the news was successfully treated for her childhood pituitary dwarfism, attaining a height of 5'4".

Rare Disease Day 2012

It's Rare Disease Day 2012 on Wednesday 29th February, and I am almost as excited as I was for National Pituitary Awareness Month in October. The Pituitary Foundation is supporting Rare Disease UK, and you can find out more about the events on offer and how to get involved by clicking here.

Also, check out this list of 21 rare diseases compiled by the Huffington Post to mark last year's Rare Disease Day. Ok, so some of the conditions they list are really symptoms rather than illnesses in and of themselves, but clearly they've gone for the most sensationalist conditions out there, and more power to them. First illness on the list? Acromegaly/gigantism. Whoop whoop!

Acromegaly: A Monstrous Illness?

Last week I wrote about the first of two websites which got me thinking about acromegaly, and I've been thinking away all weekend. The second thought-provoker was this post, on a blog about horror films, which mentions the little-known 1944 film The Monster Maker. Why is this film of interest? Because the plot centres around acromegaly. Kind of. Let's just say that The Monster Maker is to acromegaly as The Core is to science.

Brief disclaimer: I haven't watched this film, just read about it. But here's a rough synopsis; I can't imagine anyone's too worried about spoilers for a film that came out almost seventy years ago.

We start with one mad scientist, Dr. Igor Markoff.* Years previously, he injected his wife with the "acromegaly virus" for the rather melodramatic reason that he wants to disfigure her so that no other men would want her. Consequently, she committed suicide. In the present, he comes across Patricia, the daughter of a famous pianist, who just happens to look exactly like his dead wife. Before she developed acromegaly, presumably.

But Patricia isn't interested in Markoff's subtle advances.** So obviously, the logical course of action is for Markoff to infect her father with the "acromegaly virus" as well, and then use the prospect of a possible cure to blackmail him into getting her to marry Markoff. The ending of the film seems somewhat confused, but by all accounts it appears to involve a man in a gorilla suit. Because... well, why the hell not?

Leaving aside the dodgy science, and indeed the gorilla, this film sounds interesting. Patricia's father, Anthony Lawrence, becomes sick and deformed, taking on the appearance of a monster thanks to some serious effort on behalf of the makeup department; Igor Markoff looks entirely normal, except for his trademark I'm-an-evil-genius goatee. Lawrence's appearance inspires fear in the viewer; Markoff is the real monster. The moral of the story is so crushingly obvious that a child could pick up on it, although these days most children would probably be complaining that the film isn't scary enough - and besides, it's in black and white! What's that about? And why didn't they just CGI the gorilla? Seriously, WTF.

But people who suffer from disfiguring diseases like acromegaly and Cushing's Disease in real life don't have the advantage of B-movie actors and low budget sets to ram this point home to every stranger they run across in their daily life.

Acromegaly sufferers often experience discrimination because of their appearance. In advanced cases, as well as facial deformity and increased height, massive growth of soft tissues may give the impression of being overweight. The resultant discrimination can be particularly bad for women; activist Tanya Angus has spoken about how she's treated differently since developing the condition.

 Similarly, in Cushing's Disease, a pituitary tumour causes sufferers to put on weight, often to the point of obesity. Not for nothing has it been called "the Ugly Disease". But strangers don't know that; most people are likely to think that a person is overweight due to greed. To quote a Ricky Gervais joke "We all eat too much in the West. But it's people who say it's glandular, isn't it? It's not glandular, it's greed". Well, sometimes... it is glandular. It's pituitary glandular, it's adrenal glandular, it's thyroid glandular. Glands can seriously screw you over, as I have learned. It's difficult enough being diagnosed with a serious, hard-to-treat illness which causes physical pain and makes you fat and destroys your self-esteem. But to also have to put up with abuse from complete strangers who refuse to believe you're even ill must be horrific. And because Cushing's usually takes a long time to diagnose, sufferers often go years believing that it's their fault that they're putting on so much weight, even though they're eating healthily and exercising well.

These are problems that need to be addressed. It's bizarre that while it's now (happily) seen as unacceptable to discriminate against people on the grounds of race, gender or sexuality, discriminating against people who are overweight, and making assumptions about their medical conditions, seems to be ok.

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*N.B. that it's very important for mad scientists to have vaguely foreign-sounding names.

**He sends her flowers every single day, filled with creepy notes and messages. Oh, and he watches her continually. No matter what Twilight's told you, boys, stalking is not a romantic way to woo a lady.

I Want Acromegaly

As many of you are no doubt aware, I'm the kind of sad and tragic person who has enough time in her life to trawl the internet for other blogs about pituitary adenomas. Due to a weird and extremely irritating Blogger glitch, I'm unable to follow anyone anymore, but I still seek them out.

And two separate sites I found today, both about acromegaly, got me thinking. They got me thinking so much that I suspect I'll have to split my thoughts out across a couple of posts, or risk literally boring my readers to death.

The first was this: a conversation on a forum begun by a guy who claims to want to have acromegaly.

Quick recap: Acromegaly is a serious illness caused by a rare brain tumour on the pituitary gland which releases growth hormone into the blood. It can cause uncontrolled growth throughout the body, especially the hands, feet, chin, nose, tongue and forehead. It can also cause impotence, congestive heart failure, kidney failure, diabetes, loss of vision, and death. It is a devastating diagnosis for those unlucky enough to have it.

I quote:

"I really want to have this condition. I want it because I have a "babyish" face and my facial bones are not as developed as they should be. I'd rather not get cosmetic surgeries such as chin impants and jaw implants. When my face becomes as masculine as I want it to be I'll just get treatment for it.

Also my hands are small for a mans and I'd like to have bigger hands.

[...] How can I induce acromegaly in my body? Is it even possible?"

This guy may be a genuine idiot, or he may be a troll, but the fact is that there really are people fuckwits out there who inject themselves with growth hormone because they think it will make them better at sports/bodybuilding.* These people are effectively giving themselves mild acromegaly, despite the fact that there is really very little evidence that it could improve sporting performance, and it may actually decrease stamina despite increasing muscle mass.

Those of us cursed with epic fail bodies that go haywire at the slightest provocation tend to strongly resent it when fit and healthy people take risks with their health and fitness for the purpose of vanity. When I was having the packing removed from my nose after my brain surgery, and it was incredibly painful and there was blood everywhere,** the first thought that went through my head was: "Why would anyone ever have a nose job by choice?" (Plus, as a person who regularly has health professionals sticking needles in me, I can't get my head around someone actually volunteering for human pincushion duty).

Going around saying that you want to have acromegaly to gain a better jawline is like saying you want to get cancer so you can lose weight. Not only is it incredibly disrespectful to those people who seriously suffer with a horrible illness, it's just incredibly stupid. Injecting yourself with growth hormone so you can stand on a stage with a bunch of other people and flex your muscles impressively... words fail me. Not only are you putting your body and your health at risk, but you're cheating. All the other hard work you did to get those muscles is wiped out. You didn't win because you happen to be the muscly guy who worked the hardest and had the best muscly genetics out of all the hardworking muscly guys with muscly genes; you won because YOU'RE A DUMBASS.

Actually got surprisingly angry writing this! My next post on the topic of acromegaly, Cushing's disease, and physical appearance is coming soon...

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*I recommend clicking on that link and scrolling down to the photo of the incredibly muscular man cuddling his pillow with his giant melon-packed arms, it's hilarious.

**In fairness, apparently it's not usually as bad as that, I just have a particularly objectionable nose. If you're squeamish, don't continue reading: The first time the nurse tried to pull the packing out, I asked "How badly will it hurt?" and the nurse replied "A bit." I am fairly good at dealing with pain usually, so I braced myself. She gave the packing a firm nug and it felt like my nose had exploded. I shrieked and she gave me a slightly unsympathetic look and told me I had to "get it over with". On the second attempt, she got the packing out, along with really quite a surprising gush of blood that went all over the floor. It hurt so much I was physically shaking and the nurse was forced to admit that it wasn't usually quite so horrendous.

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.

More Famous People With Lumps In Their Head.

So it turns out that by far my most popular post on this blog so far is the one entitled "Russell Watson, It's Not All About You". I don't know whether my readers just happen to have enduring personal grievances with Russell Watson, or are simply opera purists who object to his pop-operatic croonings. Or maybe it's something else.

So, I'm faced with a choice. In my relentless pursuit of pageviews, should I begin writing reams of personal abuse about Mr Watson, or simply continue to shine a spotlight on the other pituitary ademoaners of the world?

I choose the latter option, and not just because of Britain's vast and unyielding libel laws.*

And so, I present:

A Panoply of Pituitary Problems, or: More Famous People With Lumps In Their Head.

Once again I must apologise for the preponderance of people with acromegaly on this list! Acromegaly is an extremely rare illness, caused by a tumor on the pituitary gland which produces excessive growth hormone. However, acromegaly's symptoms are so visually striking that, especially if the illness occurs in childhood - when it will lead to massive growth in height (gigantism), as well as the growth of soft tissue and bone which occur in adult acromegaly - its sufferers are very visible. So-called "giants" are in demand for certain roles in the film and television industry, as well as certain sports where their height gives them an advantage. Consequently, it's relatively easy to find famous acromegaly sufferers, while celebrities who suffer from other kinds of pituitary adenoma may keep their diagnosis private.

I certainly think that it would be helpful if there were celebrities who were known to have Cushing's, or prolactinoma; it helps sufferers to feel that they're not alone, and emphasizes the fact that, while a pituitary adenoma can be a significant bloody nuisance, it's not the end of the world - and that there are plenty of other people going through the same problems.

Obviously I think it would be awesome if a famous person was known to have a TSH-oma/thyrotropinoma. But if that doesn't happen, well I guess I'll just have to step up to the celebrity line!**

Anyway, back to the point. Presenting:

Carel Struycken
Carel Struycken is an actor and an acromegaly sufferer; he stands seven foot tall. Born in the Netherlands in 1948, he's now 63 years old. You might recognise him from playing Lurch in the Addams Family films (click here for a picture!); he's also appeared in Star Trek: the Next Generation, Men In Black, and even Sargeant Pepper's Lonely Heart's Club Band. According to my trusty Wikipedia, he's interested in photography and the development of virtual reality systems. A pretty cool guy by all standards!

Richard Kiel
Richard Kiel is another well-known actor who suffers from acromegaly; his most famous role was as Jaws in the James Bond films. He's now largely retired, although he voiced a character in the animated film Tangled which came out in 2010, and he's written two books; one a memoir entitled Making It Big In The Movies, the second a historical novel co-authored with Pamela Wallace, about the life of Cassius Marcellus Clay, a 19th-Century abolitionist.

Scott Hamilton
Scott Hamilton, the Olympic gold medallist figure skater, is a craniopharyngioma sufferer. I've not previously mentioned craniopharyngiomas, but like pituitary adenomas they're a rare kind of benign pituitary tumour. They cause similar symptoms to pituitary adenomas; they can interfere with hormone production, although they will cause hormone deficits rather than overproduction of pituitary hormones, and they cause headaches and vision loss. They're most common in children and middle-aged adults; Scott Hamilton was diagnosed with craniopharyngioma aged 51. A remarkable guy, Hamilton won four consecutive World Championships and created Stars On Ice.

Hamilton's last performance on Stars On Ice

UPDATE: For those of you who just can't get enough of hearing about famous people with pituitary tumours, I've also written a post about famous people with Cushing's Disease, a post about famous women with acromegaly, my original post about Russell Watson that sparked this whole thing off, and my first post about famous tumourheads!
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*Russell Watson, I love you.

**Admittedly, I haven't quite worked out how I'll achieve fame. Through my own line of edible greetings cards? By streaking at the London Olympics? For my charitable work with walruses? Ideas on a postcard please.

A Weighty Matter

Traditionally there are two things you're not supposed to ask a woman: her age and her weight. This fact has led me to conclude that the staff at my local hospital are ill-mannered oiks, because every time I visit they demand to know my date of birth and promptly stick me on some giant hospital scales (I once had a go in a hospital weighing chair. It's just like a normal chair… except that when you sit in it, it weighs you. Surprise!). I am led to understand that they do this to everyone at the pituitary clinic, presumably because so many pituitary problems can affect your weight. Cushing's disease can lead to significant weight gain; acromegaly can as well, and thyroid hormone disturbances can have disturbing consequences for the waistline.

Before beginning treatment with lanreotide last January, I had presumably been hyperthyroid (ie. had too much thyroid hormone) for at least a year, given my symptoms. Hyperthyroidism is "supposed" to lead to weight loss, by affecting the metabolism; hypothyroidism (having too little thyroid hormone) leads to weight gain. In my case, though, this didn't hold true; I'd been hyperthyroid for a long time without weight loss, and in fact it was when treatment began to reduce my thyroid hormone levels that I started to lose weight, because I found that it reduced my appetite from "extremely peckish" to "normal person". It had never occurred to me previously that my appetite was unusually high* - but as it turns out, the hyperthyroidism caused by my pituitary adenoma was giving me an appetite which more than matched my raised metabolism. When I did begin to lose weight, the lanreotide injections may also have nudged things along, because they effectively turn off your gall bladder for a few days after each injection - the gall bladder stores bile, which aids in the digestion of dietary fats, so the lanreotide leaves the body less able to digest fats for a few days each month. Effectively it's an imbalance of the humors.

But anyway, the short story is that I've lost just about a stone over the last year, pretty slowly but steadily. A stone might not sound much compared to the stories of vast weightloss that WeightWatchers et al may peddle you, but it's over 10% of my bodyweight. That's quite a bit.

The pros of losing weight:
1. Being thinner!
2. More piggyback rides may be demanded from boyfriend
3. Getting to buy a bunch of new clothes

The cons of losing weight:
1. Having to spend a load of money on a bunch of new clothes
2. It worries my mother
3. Newfound paranoia about getting really chubby

Previously, I had never really worried about putting on gallons of weight,** but unfortunately the whole experience has underlined for me the fact that, if I can suddenly lose so much weight without trying, I could just as easily gain it, should my hormones decide they want to screw me over in a whole new way. That is a scary thought. Everyone always tells you that as long as you eat right and exercise you've nothing to worry about, and the cruel common wisdom is that everyone who's fat brought it upon themselves - but the fact is that's not always true; as I mentioned earlier, the symptoms of pituitary tumours often involve weight gain. People with Cushing's disease can eat incredibly strict rations and still put on a lot of weight; over the last year, I've experienced for myself how much even relatively small changes in thyroid hormone levels affect appetite, as well as weight and metabolism. Even right at the beginning of my lanreotide treatment, when my thyroid levels had only dropped slightly, my appetite suddenly crashed back to earth, and throughout the year it has noticeably fluctuated from month to month.

I'm really lucky that my weight change has, so far (and fingers crossed) been in an ok direction, but I can't help but worry that, should I need more treatment in the future, that might change; surgery and radiotherapy both have the potential to knock out my body's ability to produce various pituitary hormones and leave me hypothyroid rather than hyperthyroid

I now have a nightmare where I have to use the extra-large doors they have installed in the hospital for obesity clinic patients. I'm hoping that will only happen if 80's style shoulder-pads come back into vogue.

God forbid.

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*Well ok, it had, but I thought I was just extremely greedy.

**Well ok, I had, but only after watching the BBC's classic (and unspeakably horrific) programme Super Size Ambulance.

The Future's Orange...

...and not just because I've applied the wrong shade of fake tan.*

Yesterday, while working hard at work/deleting all the pointless emails I got over Christmas/eating the leftover Christmas chocolates brought in by a coworker, I received a phone call from the hospital.

This happens about once a month, and I have developed a clear routine on such ocassions, as follows:

1. Almost choke on my cup of tea in surprise
   i. Search for somewhere to put my mug amongst all the paperwork on my desk***
2. Scrabble frantically through my handbag to find my phone, whilst cursing either:
   i. my annoyingly raucous ringtone
OR
   ii. the piles of crap and receipts filling my bag and obstructing my progress.
3. Sprint out of the room into the corridor whilst both:
   i. answering the phone
AND
   ii. apologising to my coworkers for disrupting them
4. My endocrinologist says: "Hiya, is now an ok time to talk?"
and
5. I LIE THROUGH MY TEETH and respond "Yes, now is fine, no problem."

Anyhow, the phone duly answered, my endocrinologist informed me that he was calling because, having consulted with his colleagues about me, they have decided that I should start having injections of somatostatin analogue this month - specifically lanreotide (brand name, Somatuline Autogel). They will be at a lower dose than the injections I had previously (I was on 90mg, now I will be on 60mg), which hopefully means fewer side effects. I wasn't too badly affected last time, just had some stomach cramps and nausea for a couple days after each injection - but lanreotide's side effects can be pretty nasty, and I saw on the Pituitary Foundation's forums that some people on these injections have to go on a fat-free diet for the first week after each dose. I am hoping this will not happen, although perhaps I'm more likely to get side effects this time as my thyroid hormones are only slightly above normal, whereas previously they were decidedly eccentric. Interestingly, my endocrinologist mentioned that there's another somatostatin analogue formulation which tends to give people fewer side effects, so if I react badly to it there's the option of switching.

Of course, it all sounds so simple on the phone. "I will call your doctor," my endocrinologist said merrily, "then you can go for blood tests next week and have the injection."

Easy, I hear you cry!

WRONG

These injections are great because they fix me, but they sure are a hassle. Just as with answering my phone, there is an excessively complex procedure to follow before they can begin:

A Spotter's Guide to Somatuline Autogel Injections:

1. I go to my local GP's to get the initial prescription.
2. My doctor looks at me darkly and writes out the lanreotide prescription, muttering "oh my god, it's so expensive... so expensive..." and weeping gently into his rather ugly tie.
3. I take the prescription to the pharmacy and hand it over to the pharmacist, saying "I imagine you'll have to order this one in."
   i. The pharmacist gives me a patronising smile and says "No, no, I'll see if we have it in stock first".
   ii. The pharmacist looks at the prescription.
   iii. The pharacist's brow wrinkles deeply.
   iv. The pharmacist orders it in, saying "it'll probably be here in a week. I'll give you a call when we have it."
4. A week and a half later, the pharmacy calls.
5. I go to collect the lanreotide from the pharmacy (it comes in a pre-filled syringe, which I guess is handy).
6. I take the injection straight to the GP's, because it has to be kept refrigerated
   i. I explain twice to the receptionist why I'm trying to give it to her
   ii. Eventually, she accepts it and puts it in the practice fridge
   iii. The receptionist then stops me as I'm walking out of the door, crying, "Have you written your name on it?"
   iv. I point to the giant pharmacy sticker on the front of the box, listing my name, age, gender, address, GP's name, GP's address, bank details, sexual preferences and preferred pizza toppings
7. I am finally able to book myself an appointment to actually have the injection
8. Several days later, I return to have the injection, and several blood tests beforehand
   i. It is a GIANT needle, I mean literally, even the nurses comment on how giant it is
   ii. The nurse has forgotten to leave it out of the fridge to warm it up a bit, and consequently it's like having a stinging-nettle frappe injected into your hip
9. I go home and feel ill for two days
10. REPEAT PROCESS  

I realise this is the most whiney post ever about a treatment which hopefully should leave me feeling much better.**** In actual fact, I am genuinely quite looking forward to going on lanreotide again; hopefully it might finally stop my hair falling out, sort out my heartrate and generally leave me feeling both pinky and perky. I just wish the process of getting hold of it didn't involve three trips to the doctors and two to the pharmacy each month.

Interestingly, Wikipedia tells me that in the US, lanreotide is only indicated for the treatment of acromegaly; it seems to imply that it is only used against thyrotropic adenomas/TSHomas in the UK, although I don't know whether that's actually true; it's obviously a logical treatment choice, as somatostatins inhibit the production of thyroid stimulating hormone as well as growth hormone. As well as pituitary adenomas, lanreotide is also used to treat symptoms of neuroendocrine tumours; the kind of tumour that Steve Jobs suffered from.
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*Incidentally, I don't use fake tan. I am pale and interesting.**

**Well. One out of two isn't bad.

*** I have NO IDEA what most of the paperwork on my desk is. I inherited it from my predecessor and it was never explained to me. Consequently, I don't know if it's too important to throw away.

****Fun fact: when first typing this sentence something broke in my brain and it read "...an injection which should leave me stealing much better". Because at the moment, I am a rubbish pickpocket.

IMFW: Moral Dilemma

The first Interesting Medical Fact of the Week for 2012 - the first interesting fact for a whole new year - concerns something (or rather, someone) that I've written about before. Highly relevant to the pituitary adenoma theme of this blog, in fact.

In my last post, I mentioned Charles Byrne, the Irish Giant, as he was known in his lifetime, a man who suffered from acromegaly in the 1780s. He was briefly a popular attraction in London before succumbing to alcoholism and dying very young at the age of 22. Although he is probably the most well known, as his skeleton is part of the collection in the Hunterian Museum, there were several well-known Irish giants, all of whom likely suffered from acromegaly, who exhibited themself across Europe at the time.

Byrne's skeleton was preserved against his will; the story goes that he was so keen to avoid his body falling into the clutches of John Hunter, a noted surgeon of the time, that his will requested his friends bury his body at sea in a lead coffin. Hunter managed to get his hands on the body, however, allegedly through bribery.

It's curious how many websites I've found when googling the name "Charles Byrne" which not only fail to mention acromegaly, but fail to mention that Byrne had an illness at all - as though growing to 7'7" is a kind of character flaw which could happen to anybody. His skeleton is still on display to the public at the Hunterian Museum, and around a year ago there was some publicity around some genetic research that has been taking place, looking at a genetic cause for  pituitary tumours which recur in families, which identified some modern-day acromegaly patients who may be related to Byrne.

More on that research another day.

Today, the news is that there have been calls for Charles Byrne's skeleton to be removed from display, and buried in accordance with his original wishes. It seems fairly unlikely that this will happen, he's a significant feature of the museum; but it's an interesting debate, and on the whole, I have to say that I would broadly be in favour of allowing him to be buried. It's interesting that the moral debate is still going on, more than two hundred years after his death.

Famous People with Cushing's Disease?

Being a blogger with a TSHoma (a.k.a. TSH-oma,* a.k.a. thyrotropinoma, a.k.a. a freak) has its pros and cons. On the plus side, as far as I can tell I have definitely found a niche in the market - no mean feat, given the number of enthusiastic and generally excellent blogs written by people with everything from diabetes to acromegaly. On the down side, there's none of that nice cosy caring and chit-chat over tea and lanreotide injections that you get from support groups with other people. People who understand how rubbish it is that your heart's going too fast/your hands have grown enormously/your nose just fell off. So being me, I have been ambling around the internet, happily barging into other people's support groups and demanding to be paid attention because I have a tumour on my pituitary gland too.

A little while ago I found the lovely Acromegaly Bloggers, and more recently bumbled across a blogging group for people with Cushing's disease, called Cushie Bloggers. People with Cushing's disease can at least console themselves with the fact that referring to themselves as "cushies" makes them sound incredibly cute and fluffy and generally charming.

But anyway, finding the blog and reading the experiences of some of the bloggers got me thinking: in an earlier post, I went on a hunt for famous people with pituitary adenomas. Obviously there were no cases of TSHoma/thyrotropinoma - that would be too much to hope for. But there's Russell Watson with his unspecified adenoma, and numerous celebs with acromegaly - even more than I included in my post, in fact: acromegalovelies are all over the shop, from Andre the Giant to Pio Pico.

But where are all the famous Cushing's sufferers?

I've found it difficult to find information specifically on the incidence** of Cushing's Disease, probably due in part to the fact that it's a subtype, as it were, of Cushing's Syndrome, accounting for about 70% of endogenous Cushing's syndrome cases. I have found the incidence of such new cases quoted as about 13 cases per million, so we're talking 9 new cases per million per year, compared to an incidence of 3 - 4 new cases of acromegaly per million, per year. So it is seemingly rather more common than acomegaly, although the massive tendency towards underdiagnosis of both illnesses (and TSH-omas as well) makes these rather shaky figures.

Given that it's relatively easy to find well-known figures suffering from acromegaly (even outside the field of sports, where young sufferers' large frames could be considered to give them an attractive advantage in sports like basketball or wrestling) and given that Cushing's is more common, you'd think it would be easier to find a celebrity with Cushing's. (You'd also expect to find people with prolactinomas, but I suspect that (particularly male) celebrities might prefer to generalise prolactinomas as "tumours" rather than publicly imply that they're leaking milk from their manboobs.) Celebrities diagnosed with Cushing's, on the other hand, are (I would have thought) surely be more likely to publicise their condition, to explain that any excess weight gain was not due to greed or laziness but a medical condition.

So where are they all? I've looked around the place and all I can find is a vague hint that Elvis may have had Cushing's syndrome (as distinct from the disease, Cushing's syndrome does not necessarily imply a pituitary tumour, but could be caused by steroid overdose or adrenal tumours etc). If true it would certainly be something of a PR coup for cushies, but one article in the Daily Mail is not much to go on. The Daily Mail seems to have a little bit of a penchant for Cushing's Disease (is there a sufferer amongst the editorial staff?) and I've also found an article suggesting that King Henry VIII may have had Cushing's Disease. Who knows? When it comes to historical figures, it's fun to speculate, but speculation is all it can remain.

The only - literally the only - modern-day sufferer I've found who comes close to the designation of "celebrity" is J. Jordan Bruns, an artist featured in this article - but he's not exactly a household name, lovely though his art is.

Come on cushies, come out from the woodwork!

UPDATE: If you're interested in reading about famous people who suffer from pituitary tumours, see my other posts looking at famous pituitary ademoaners here and here; my post about Russell Watson, the opera singer who had a pituitary tumour; or check out my post looking at famous women with acromegaly.
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*Never doubt the power of punctuation. If you search for the wrong one, Google will offer you Tshoki Tshoma's naked photos. Nice.

** I would prefer to write about prevalance but I've found it harder to find figures for that.

Let's Get Technical, Baby

Apologies for my lack of posting lately! I have been doing exams. They are now over, and my new excuse is that it's Christmas (in case you hadn't noticed). But, as of a phone call from the hospital on Wednesday last week, I have been meaning to give an update on my health. In short: my thyroid stimulating hormone levels are still normal, but I will have to start new treatment shortly... because I have high levels of the TSH alpha subunit.

"The what-now? What in god's name is that?" I hear you cry.

Don't cry.

My endocrinologists have always been cagey whenever I've tried to get them to explain what the alpha subunit actually is,* although in fairness, my shrewd personal judgement leads me to believe that this is probably less because it's a dark and terrible secret and more because they're not paid to teach biochemistry.

Anyway, the combined powers of Wikipedia and Google have provided me with the answer: it turns out that thyroid-stimulating hormone (TSH) is a dimer; a chemical compound of two identical or similar monomers: in this case, the alpha subunit, and the beta subunit. No-one wants to get bogged down in the detail, so at this point I promise not to use the word "glycoprotein" without extreme provocation.**

So: TSH is made of two kinds of stuff. And as it turns out, TSHomas (pituitary tumours which produce TSH) have a bit of a habit of producing more of the alpha subunit than would normally be expected. Normally you would expect the ratio of free alpha subunit to TSH to be less than 1. Before my surgery in April, my ratio of alpha subunit to TSH was 14:1. Currently, it's 7:1.

Obviously, 7:1 is better than 14:1. However, it is equally obviously Not Quite Right.*** Consequently it seems likely that the TSHoma is still active, and even though my total thyroid-stimulating hormone levels are within normal limits, it may still be secreting some TSH as well. Active tumour = growing tumour.

So I'm going to start having injections of some kind of somatostatin analogue again. I haven't really written about this up to now (it's on my list, promise) but for three months before my operation last year I was having monthly injections of Somatuline, the brand name for lanreotide. It's a synthetic form of a hormone naturally released by the hypothalamus,**** which inhibits the production of TSH and growth hormone, and which is used to treat pituitary adenomas which are releasing either one of those hormones. If you're lucky, it can shrink the tumours a bit - at the least, it should check their growth and can also help to make their structure less fibrous, which makes it easier for surgeons to chop them up.

It's also super expensive. When my GP first looked up how much it cost after my endocrinologist had requested a prescription, he laughed in a slightly manic way for about five minutes at the price. Awkward.

I'm going to start the injections again, probably in January, for three months, to assess whether they shrink the tumour at all and whether they bring down the high levels of alpha subunit in my blood. I'm not massively looking forward to starting again - the injections mess with your body a bit, last time I felt nauseous for three days after the first one, and they're a bit of a hassle because you have to order the injections a week in advance from the pharmacy, then drop them off at your GP's because they have to be kept in the fridge. The injections sting, they have to use a massive needle because the stuff is so thick, and you end up with a lump in your hip - basically it's injected under the skin where it sits and slowly decreases in size over the month as it gets absorbed into your system.

On the plus side, having these injections should (in theory) get rid of the last of my symptoms. And that would be very nice. I am really quite bored now of my hair falling out/heart randomly getting overexcited/headaches. As for what happens after three months, who knows. The injections are a possible long term treatment option although they are an expensive hassle and they're quite likely to give you gallstones sooner or later. So that brings you back to surgery/radiotherapy. Oi vey.

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*Apart from an excellent title for some kind of spy film.

**Incidentally, the α subunit is thought to be the effector region responsible for stimulation of adenylate cyclase

***I must stop using these technical medical phrases.

****Somatostatin.Lol.