Back to La Cura

Regular readers who have not grown tired of my recent lack of posting
may recall that a little while ago I posted a link to a website called
Open Source Cure, created by an Italian man, Salvatore Iaconesi, who has
been diagnosed with brain cancer. BBC News has recently done a piece
about the website, which has provoked a huge response in terms of both
medical advice, messages of support and artwork. Apparently the public
reaction to the website and Mr Iaconesi's interactions with various
doctors have influenced the way in which his brain surgery is going to
be carried out, and the Italian government have even picked up on the
site's popularity, and is now looking at opening up patients' medical
records.

It's quite an incredible response to see. Many people, myself included,
turn to blogging as a way of venting the frustrations of being ill and
dealing with hospitals, as a way of updating friends and family on how
we are, and as a way of connecting with other people in similar
situations. When you have a rare illness like thyrotropinoma, it's
seriously unlikely that you'll know anyone who's been through the same
thing in real life, so it's natural to reach out to others online. But the concept of
seeking not only support and tips but also actual medical advice on
treatments and surgical techniques from complete strangers - from the
whole world - is a pretty unique approach. Given the difficulty that
people with rare medical conditions can have in accessing doctors with
sufficient (or indeed any) experience in the treatment of their illness,
perhaps it's something that will become increasingly common.

Hyperthyroid Patients, Unite!

Subtitle: You have nothing to lose but your goitres!

So here's the strange thing: I have a TSHoma - a pituitary tumour which secretes TSH (thyroid stimulating hormone). It's super rare. According to my endocrinologist there are maybe thirteen or so other people in the UK with this condition which, in a population of 63 million, is Not Very Many, although it probably does go largely underdiagnosed; I had my first symptoms when I was around 16 or 17 and didn't get a diagnosis until I was 22.

But anyway, the point is that it can sometimes almost feel like two separate conditions; I have the pituitary adenoma, and the hyperthyroidism it causes. My symptoms, apart from the odd nasty headache, are pretty much exclusively those of hyperthyroidism; my treatment is for the adenoma. Consequently, although I am in all seriousness a paid-up member of the Pituitary Foundation and can chat about transsphenoidal surgery and lanreotide injections with the best of them, I'm missing out on the chance to bitch and moan about sinus tachycardia and getting the shakes with other people whose blood has turned to delicious thyroid hormone stew. More fool me!

People with excess thyroid hormone usually get that way from either a) taking too high a dose of thyroxine medication (exogenous thyrotoxicosis), or b) something else. The former can be dealt with simply by reducing the dose. The latter is more complicated. Possible causes include:

Graves Disease - the most common cause of hyperthyroidism, Graves is an autoimmune disease which can be treated with anithyroid drugs, surgery to remove part or all of the thyroid, or a dose of radiactive iodine. After such treatment there's a substantial and ironic risk of hypothyroidism - i.e. not having enough thyroid hormone.

Tumours of the thyroid gland - thyroid adenoma is a benign tumour of the thyroid gland which may secrete large amounts of thyroid hormone, while thyroid nodules may be benign or malignant and again may or may not release thyroid hormone - in some cases they may actually inhibit its production, causing hypothyroidism.

Thyroiditis - inflammation of the thyroid gland. Although this often initially leads to high levels of thyroid hormone being released, it usually progresses to thyroid disfunction with the gland unable to produce sufficient hormones, and ultimately causes hypothyroidism. Around 7% of women experience temporary thyroiditis after giving birth (postpartum thyroiditis) which may lead to hyper- or hypothyroidism, or both; this usually resolves itself.

TSH hypersecretion - super rare, but usually caused by a pituitary adenoma (hurrah!). However, I believe there is also the possibility of a tumour in the hypothalamus causing excess Thyrotropin Releasing Hormone and hyperstimulating the pituitary gland to overproduce TSH, which in turn would hyperstimulate the thyroid gland to overproduce thyroid hormone. Again, it's very rare.

I guess really I should be grateful for this, as effectively I have two health conditions for the price of one. This blog is usually focussed mainly around the dramatic "there's a tumour in my head!" aspects of my pituitary adenoma, and poor little hyperthyroidism barely gets a look in, except for when I complain about my tachycardia/hair falling out/absurd appetite issues. But no longer! From now on I vow to go on about hyperthyroidism more. So you guys will get two lots of whingeing for the price of one!

A Lesson From My Former Landlady: Part 1

I was originally going to name this post something else, but in deference to my readers who have delicate constitutions, I decided against it. Anyway. While it will be something of a massive rant, it's actually a good way of illuminating a topic that I've been wanting to write about for a while.

***

So for about eight or nine months from last summer, I lived as a tenant in a house occupied by my former landlady and her two children, who split their time between her house and their dad's. My former landlady - let's call her crazy bitch June - gave me notice in January that she'd need me to move out in June because she wanted to redecorate the room I was in. This seemed a suspect excuse, as my room was the only part of the house not in need of decorating, but no matter; I didn't have the chance to discuss the issue with her because she took the rather peculiar decision not to tell me in person, but to wait until I went on a weekend away and then slip a note about this under my door. I started to look for a new place almost immediately, because I knew I would have exams in June and didn't need the stress of moving out at the same time.

In March, I found a lovely new house nearby and gave her my notice. She said that this was fine as it didn't matter to her when the room was redecorated. I tried to organise a time to go through the inventory and get my deposit back, but she was strangely cagey about it. I offered to go through it with her on the day I was moving out, having just cleaned the room with my mum, but she refused. After moving out, again I kept trying to arrange a time to go through it all with her but she continued putting me off until I suddenly received a text message ultimatum that it had to be on that Friday (a day which I had already informed her I wouldn't be able to do) because she had someone moving in the next day.*

I spoke with my manager and was able to leave work early that day in order to go and see her, despite the fact that things were super busy that week and I knew that I would have to work over the weekend to make up the time I lost by leaving early. So off I trotted to my old house.

The instant I got there, things seemed strange. I was let in, but although I had explained to June that it was an awkward time and I would be in a rush, she seemed surprised that I was in a hurry and she stomped upstairs, obviously in a bad mood.

June was always a shouty woman. When her kids were staying, there was shouting every evening - about eating dinner, about singing lessons, about baths, you name it. What I was not prepared for, however, was for her to turn her shoutyness on me practically as soon as we stepped into my former bedroom. She pointed to a laughably small build-up of scale on the bathroom tap and had a go about it - then when I pointed out that it had always been there, she claimed that if there had been scale on the tap, she would have written it down in the inventory. I pointed out that this was not the case, as there were various holes in the walls and floor, the broken curtain fitting etc. which were not in the inventory - seeing as the inventory is a list of the room's furniture, not a detailed description of every aspect of its decor. She yelled that the holes in the floor were "not the problem" and continued shouting, accusing me of allowing a terrible limescale build-up in the shower, "ruining" a bookshelf, leaving the room dusty, etc. etc.

She accused me of not cleaning the room at all before I left; I pointed out that a) not only has she actually seen me going upstairs and downstairs with mops and buckets and cleaning products on the day I moved out but I had also spoken to her about which mop she would rather I use to clean the floor, and b) because she refused to do the inventory on the day, obviously the room now had two week's worth of dust in it. Which only made her shout more. At one point, she yelled "Frankly, Emer, I don't believe you did any cleaning the whole time you were here!" Which is a bit of a bloody cheek coming from a woman whose kitchen was so permanently disgusting that a) it attracted mice** and b) my appalled yet kindly mother did some of June's washing up on the day I moved out just to make it less horrific.

By this time, all the shouting was really starting to stress me out; I was on the verge of tears. I'd been on the lanreotide injections for a few months, and the thing about them that I think I've mentioned before is that they really do make me a lot more emotional and easily upset, for some reason. I had never told June about the whole brain tumour thing or the injections or anything, because frankly I didn't think it was any of her business and it tends to make things awkward.

Anyway, I asked her (politely!) to stop shouting. She shouted "I'M NOT SHOUTING!" at me, then continued shouting. I was so stressed out by the whole thing that I was physically shaking and feeling sick, so when she told me she was going to dock me 10% of my deposit (apparently cleaning a tap costs £65 these days. I'm not sure she's doing it right) I barely even argued because I was so desperate to get out of there. I was still trying to stay calm but I was kind of furious at myself for being so easily upset, so when she continued ranting as she wrote out the cheque I said: "June, I really don't appreciate your attitude today; I made a real effort to fit in with your plans even though it was extremely inconvenient for me, I took time off work and you've been nothing but rude to me the entire time I've been here." Predictably, she started shouting again, threatened to rip up the cheque, blah blah blah.

When I left my former home (with the cheque, thank Christ), I got about four steps down the road before bursting into tears and I was still shaking by the time I got home, at which point I realised that I was probably a bit hypoglycaemic (occassional side effect of the lanreotide injections), ate a couple of biscuits and felt slightly less awful, despite the fact that I had just effectively paid £65 to run away. Fortunately my lovely boyfriend was visiting that evening and he made me feel a lot better, although the whole story made him kind of furious. I believe pissing through June's letterbox was mentioned, and to this day I slightly regret my decision to take the high ground there...

It seems pretty plain to me that June's plan right from the start - before I'd even vacated my room or she had seen it - was to get that money off me. The way she dodged my emails trying to organise it, and then suddenly demanded to have it on a day she already knew I couldn't do, indicates to me that she was hoping I would say I couldn't come, so that she could just arbitraily dock the money and send the cheque in the post without having to do it to my face. The way she avoided doing the inventory on the day I moved out when my mother was there as a witness, and her incredibly aggressive behaviour as soon as I stepped through the door all make it seem as though she was determined to get that £65 by hook or by crook.

That kind of behaviour isn't acceptable from anyone to anyone. Society might consider it rather worse for my landlady to try to intimidate me, a 23 year old girl than, say, a 46 year old bloke. Is that fair? No. Would she even try it on with a 46 year old bloke? Who knows? I certainly doubt she would have behaved in the same way if my boyfriend had been with me. And what about the fact that I'm sick? If June had known about my pituitary tumour, about the fact that I was on hormone treatment which made me feel emotionally pretty delicate and physically resulted in odd bouts of hypoglycaemia, would she have still done it? If I went up to her now, knocked on her door and said "Oh, by the way, June, just so you know - I have a benign brain tumour," would that change the way she felt about her behaviour towards me?

Because it shouldn't.

You can't always tell if someone is sick. Not every ill person has a wheelchair or a bandage or an obvious badge of their personal infirmity. Heck, even if you now them pretty well - even if you live with them - you might not know about it. Yet the default in society is to treat everyone as if they were well and make 'special allowances' for sick people - once they produce a medical certificate. There are plenty of people in the world who would probably be horrified to realise that the shop assistant they were a bit of a dick to yesterday has a brain tumour, or the slightly unhelpful telephone operator they're shouting at has just come back to work after going through chemo. We do these kind of things all the time - and I'm by no means claiming that I'm innocent of this, by the way - and yet, if we knew of the person's illness, or bereavement, or disability, we would never dream of treating them in that way. And the only way to get around this is by trying to treat everyone with as much respect as you can muster, all the time. And even when someone is behaving appallingly, you need to give them the benefit of the doubt, because you just don't know. And that is a really difficult thing to do.

Because here's the killer question: If I found out that June had just been told she had Huntington's, or MS, or cancer, would that change how I felt about her behaviour - even though it wouldn't excuse it?

Of course it would.

And that's why I'm glad we didn't go piss on her doorstep.

Well... mostly glad.

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* So much for "redecorating".

**Which June then killed, which seemed rather unfair. If she didn't leave food for them around all over the place, those poor mice would probably still be alive, frolicking happily in the compost heap.

IMFW: The King's Evil

Recently, the UK celebrated the Queen's Diamond Jubilee, marking sixty years of her right regal reign. At some point over the weekend, I made a joke about scrofula - that most historical of diseases - and no-one got it. Now, people often don't get my jokes, and I'm sorry to report that approximately 70% of the time this is because they are Not That Funny. However, the other 30% of the time they are absolutely hilarious, and this was one of those times. Consequently I was forced to the conclusion that either 1.) my friends are, to a man, ardent royalists who refuse to laugh at an old lady purely because she has an unusually heavy and unfashionably metallic hat, or 2.) they didn't know what scrofula was.

And so, in the interests of education, this week's extremely overdue Interesting Medical Fact of the Week will focus on scrofula, a.k.a. the King's Evil, a.k.a. tuberculous cervical lymphadenitis - and how's that for a rapper name?

Scrofula is effectively tuberculosis of the neck, resulting in swollen lymph nodes. In children it is often caused by infections other than mycobacterium tuberculosis, but when tuberculosis is the underlying cause, there are also associated symptoms of fever, weight loss, and malaise. It's a very unsightly illness; the swelling of the neck can become so large that the skin around it ruptures, leaving open wounds.

With the huge decline in tuberculosis rates over the past sixty years, scrofula has become a rare disease, except among the immunocompromised, but historically it was much more common. For hundreds of years in England and France it was commonly believed that scrofula could be cured by the touch of a king,* and indeed monarchs would hold huge events in which they touched hundreds of scrofula patients. From 1633, the Anglican Book of Common Prayer even included a special service for the ceremony, and it was traditional for the monarch to give the affected person a coin. Kings and queens varied in how far they were prepared to go with this tradition, however; although Queen Anne (r. 1702 - 1714) was fond of helping the scrofulous and even touched a young Samuel Johnson, who suffered from the illness, her successor King George I (r. 1714 - 1727) abandoned the practise as being "too Catholic" and also, presumably, too gross.
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*Queens were an acceptable alternative for the scrofula sufferer on a budget.

5 Things Healthy People Miss Out On

Over at Cushie Bloggers for the month of April, some bloggers took part in the Cushing's Awareness Challenge during April; the aim was to blog about something to do with Cushing's Disease every day of April. Well, I don't have Cushing's Disease, and it's now May. But the Challenge includes a list of prompts and suggestions of topics for bloggers with Cushing's disease to write about, and it looks like a good list to me for anyone with a pituitary adenoma to co-opt.

So, my first post from the list is in honour of both Cushing's Awareness and TSHoma Cognisance. And in fairness, I strongly suspect that awareness of thyroid-stimulating-hormone-producing pituitary adenomas is even lower than awareness of Cushing's Disease...

I like to think I am a fairly optimistic and sunny person, so instead of going down the depressing route, I have decided to start with a post focusing on the awesome aspects of being sick. Presenting:

5 THINGS HEALTHY PEOPLE MISS OUT ON

Or: Why It Is Great To Be Me and Be Ill 

1. You always have an excuse.

"Excuse me, madam, but you would appear to have inadvertently stabbed this man".
"Oh, I'm so sorry officer, it must be my brain tumour playing up again. Forgive me."
"Not at all, my good woman. Please continue about your business. And get well soon!"

In fact, I never have stabbed a man, but I fondly imagine that were I to do so, this is roughly how the conversation with the policeperson would go afterwards. Having a tumour in your head is rubbish, but it's one hell of an opportunity to get away with doing what you want.

Want a seat on the bus? Mention the tumour.
Late for a deadline? Mention the tumour.
Want to freak out a stranger? Mention the tumour.
In detail.

2. Improved and terrifying knowledge of medical terminology.

You know, if I didn't have a pituitary adenoma, I would never have heard of adrenocorticotrophic hormone, let alone be able to pronounce it. And now, not only do I have an expanded vocabulary for actual words, but I know the abbreviations too. I know that my form for TFT bloods will check my TSH, FT3 and FT4 - plus usually my endocrinologist requests SHBG and alpha subunit. Because that is just how he rolls.

3. A warm and fuzzy glow about UK taxation.

You know what I don't mind? When, at the end of the month, my paycheck is shrunk by several hundred pounds thanks to paying tax and National Insurance contributions. You know why I don't mind it? Because one monthly dose of lanreotide costs more than twice what I pay in tax. And that's not including the costs of my regular visits to the doctor, the nurse, the hospital, my other prescriptions, my MRI scans, my surgery, my blood tests.

I can't imagine how horrendous it would be to have to pay for this stuff in a country without a decent national healthcare system.

4. Joie De Vivre

It sounds pretty cheesy to say that being ill gives you a newfound appreciation for life. That's because it is a cheesy thing to say. However, it is also true. The feeling of achievement when, after pituitary surgery, you manage to very slowly walk the ten minutes to the nearest pub is as satisfying as learning to play the tuba. Probably more satisfying, in fact. After all, I've never met a happy tuba player.*

5. Writing this blog

I would never in a hundred years have managed to keep up writing a blog this long (since last August… that's ten months!) if I had not been ill. No matter what topic I picked, it would surely have lapsed, just like every other diary I've ever attempted to write has lapsed, usually within about a week. But writing so often has been really good for me; it's improved my style, it's made me more comfortable with just sitting down and typing - and searching for Interesting Medical Facts of the Week has taught me a hell of a lot. And being ill is what has made me keep this up. I can't ever lapse for long, because there's always going to be something I want to complain about.

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*Disclaimer: I have never met a tuba player.

IMFW: Brain Injuries in Sport

An interesting story that has been popping up now and again for the last few months concerns brain injuries in the US NFL (National [American] Football League), so that's what we're focusing on in this week's Interesting Medical Fact of the Week.

American Football is obviously a high-contact sport, hence the crazy amounts of protective gear the players wear - including helmets. However, evidence has been mounting for some time that all this protective gear may not be enough.

Several high-profile former players have killed themselves in the last few years. Dave Duerson committed suicide in February 2011, after texting his family to request that his brain be donated to the NFL's "brain bank" - namely, the Centre for the Study of Traumatic Encephalopathy, which was set up by former athletes to investigate the long-term effects of the concussions suffered by sportspeople and military personnel.

The Centre focuses on studying chronic traumatic encephalopathy (CTE), which is a progressive, degenerative disease of the brain. As early as the 1920s, there was recognition that this condition particularly affected boxers, but it now appears that it is also associated with repeated brain trauma (i.e. concussions or other head injury). Professional American football players, ice hockey players and those involved in sports such as wrestling, are particularly likely to sustain these kinds of injuries.

Chronic traumatic encephalopathy is a nasty illness. Much like Alzheimer's Disease, it involves the build-up of tau proteins in the brain, and the initial symptoms of memory loss, confusion, depression, and self-control problems eventually lead to full-blown progressive dementia with some symptoms of Parkinson's Disease.

When Duerson's brain was studied, researchers found that the repeated trauma to his head during his professional career appeared to have caused severe damage. One of the difficulties with chronic traumatic encephalopathy is that there can be a significant latency period between the original injuries and the appearance of recognisable symptoms; however, along with Duerson, many other former NFL players - and those involved in other high-contact sports - have been diagnosed with chronic traumatic encephalopathy after their deaths. Chris Henry, another American football player who died after falling from a moving vehicle, was the first player to be posthumously diagnosed with CTE while still active as a professional sportsman; but the earliest signs of the disease have even been found in the brain of an 18-year-old student athlete.

As well as Duerson, former professional footballers Junior Seau and Ray Easterling also recently committed suicide. Along with several other American football players, Easterling was involved in a class action lawsuit against the NFL over its handling of concussion-related injuries, which alleges that the NFL continuously denied any knowledge of a connection between NFL players sustaining repeated head injuries and the later development of dementia or CTE. In addition to this, another lawsuit was recently launched on behalf of over 100 former professional players, claiming that the NFL repeatedly denied the existence of any connection.

Chronic traumatic encephalopathy is a very rare disease, and one of the difficulties in researching it is that it can only be definitively diagnosed post mortem. But the work of the Centre for the Study of Traumatic Encephalopathy is already causing waves in the world of American football, and there have already been calls for changes to the game's regulations, effectively banning head contact from the game. The Centre's research is still ongoing, so whether that will actually happen remains to be seen.

But are those helmets enough?

If you're interested in this topic and would like to find out more, I recommend the Centre's very informative website, which also has a selection of case studies you can read about.

Belated: Cushing's Disease Awareness Day

So it turns out that the 8th of April was Cushing's Disease Awareness Day, and I completely missed it. Not least because it was Easter Sunday, i.e. the first day I could eat chocolate again having given it up for Lent. It does seem cruelly ironic that the awareness day for a terrible disease, a key symptom of which is central obesity, should fall on a day that the rest of the world dedicates to filling their faces with fatty treats.*

Anyway, dedicated readers of my blog already know what Cushing's Disease is, but for everyone else: Cushing's Disease is caused by a tumour, known as an adenoma, on the pituitary gland. The tumour produces high levels of adrenocorticotropic hormone (ACTH) which can result in obesity, high sweating, hair loss, hypertension, diabetes, headaches, painful joints, and other not-so-fun symptoms. Treatment is chiefly through surgery and radiotherapy, as Cushing's is difficult to treat with medication.

There's a group of bloggers who suffer from Cushing's Disease who've been doing the "Cushing's Awareness Challenge", blogging on a different topic about their illness for every day of April. You can find out more about the challenge and read some of their blog posts by clicking here.

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*Well, I do anyway.

IMFW: Nodding Disease

For today's Interesting Medical Fact of the Week, we're going to get a little bit Agatha Christie. I love Agatha Christie, because a) I love mysteries and b) I'm an old granny at heart. But in terms of mysterious illnesses, this one takes the biscuit.

So-called nodding disease (or nodding syndrome) was first described in the mountains of Tanzania in the 1960s. Since then, it's spread to areas in south Sudan and northern Uganda. It's an extremely serious progressive disease which only affects children, and it's almost always fatal. There is no cure. And no-one is even sure what causes the illness.

The symptoms of nodding disease include complete and permanent stunting of growth, including development in the brain, which leads to mental retardation. The name of the illness comes from the characteristic seizures suffered by affected individuals, which cause their heads to nod rapidly; the only treatment currently available for nodding syndrome is epilepsy drugs which can help to control these seizures. Extra weird is the fact that these seizures seem to happen most commonly when the child begins eating or feels cold. Children become severely malnourished because they are frequently rendered unable to eat. Many children with nodding disease actually die from falls or accidents like drowning or burning, which they're at higher risk of because of their mental impairment.

At the moment, the hypothesis that seems most likely to explain this disease suggests that it may be linked to river blindness (onchocerciasis), an illness caused by a kind of parasitic worm which is transmitted to humans through the bite of the black fly. Cases of nodding disease are concentrated in areas where there are high levels of infection with the river blindness parasite and it seems there may be a link, although no-one's yet worked out what it is. There may also be a link with low serum concentration of the blood.

There is a little bit of good news. The mysterious illness has attracted some international attention, and this week Uganda opened its first treatment centres specifically for children with nodding disease. At the moment, though, the best they can aim for is to control the illness's symptoms.

A New Treatment for Cushing's Disease?

I've just been reading about a drug called Pasireotide. It's been around for a while, but on January 23rd this year, the European Medicines Agency's Committee for Medicinal Products for Human Use recommended that pasireotide be approved for use in the treatment of Cushing's Disease. This is exciting, because Cushing's Disease - in which a benign tumour of the pituitary gland causes the body to release excess steroid hormone - has long been very difficult to treat medically, with surgery and radiotherapy being the main treatments. But they're not always appropriate or successful for every patient.

Pasireotide (brand name Signifor, developed by Novartis) is a somatostatin analogue - like the medication lanreotide, which I take at the moment to prevent my own pituitary tumour from releasing thyroid-stimulating hormone. Like lanreotide and octreotide, the two somatostatin medications currently on the market, pasireotide looks likely to be effective in treating acromegaly and neuroendocrine tumours; unlike them, it appears to be much more effective at blocking tumours from producing adrenocorticotrophic hormone.

At the moment, there are no approved medicines in Europe for the treatment of Cushing's. Other drugs are used in practise, off-label, but with less data about their safety and effectiveness against the disease, so for Signifor to be approved would be a very significant step. The European Commission looks set to make its decision in the next few months.

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!