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.

IMFW: Harvey Cushing

This week's IMFW is really more like an IMPW - an Interesting Medical Person of the Week. Harvey Williams Cushing is often regarded as the "father of modern neurosurgery".

An American descended from a long line of doctors, he was born in Ohio in 1869. After studying medicine, he went on to study surgery at the famous Johns Hopkins Hospital just after it had opened, then spent time in Europe before returning to Johns Hopkins as an associate professor in surgery. As well as writing about medicine, he was a talented medical illustrator and several of his drawings were published in textbooks - and as if that wasn't enough, he received the Pulitzer Prize for Biography for his biography of his mentor, William Osler.

Cushing's first monograph was on the subject of the pituitary, and on this blog, you'll have heard Cushing's name from one of his most famous discoveries, the disease named after him - Cushing's Disease; a tumour of the pituitary gland which secretes ACTH (adrenocorticotrophic hormone) and causes a range of symptoms including weight gain, bruising, sweating, high blood pressure and diabetes. Being a modest chap, he had originally named it "polyglandular syndrome", but his name stuck. However, this was far from his only contribution to medicine:

- He introduced blood pressure measurement to America

- He used x-rays to diagnose brain tumours

- He developed medical instruments which are still in use today, including the Cushing Forcep which is used during cranial surgery, the Cushing clip - a small clip for blood vessels to stop bleeding during surgery, which dramatically decreased mortality rates - and the use of electrocautery which he developed with W.T. Bovie, a physicist

- Along with a colleague, Ernest Codman, Cushing devised the first anaesthetic chart to help surgeons and anaesthetists monitor pulse, temperature and breathing - an innovation which was widely adoped

- Cushing pioneered many new surgical techniques including the use of saline for irrigation during surgery

- He developed a surgical cure for trigeminal neuralgia

- His mortality rate was around 10% - which doesn't sound hugely impressive until you realise that before he came along, mortality rates from neurosurgery were 50 - 90% depending on which source you believe

- He developed the transsphenoidal approach for surgery of the pituitary gland

- He invented the macarena (just checking you're paying attention...)

- He identified and named the phenomena of hypopituitarism and hyperpituitarism


Yes... yes, that is a skull.

 So Mr Harvey Williams Cushing deserves our gratitude and respect. He has thoroughly earned the bewildering array of unpleasant medical things named after him, which include: Cushing's Syndrome, Cushing's Disease, Cushing's symphalangism (a.k.a. proximal symphalangism), Cushing's Reflex resulting in Cushing's Triad (a nervous system response to increase intracranial pressure), Cushing ulcer a.k.a. Rokitansky-Cushing Syndrome, Bailey-Cushing Syndrome (he had to share that one), the Cushing forcep and Cushing clip.

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.

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.

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.

Le Meme

Of course, even if you actually have a brain tumor, the NHS will refuse to pay for a rocket to aim at your head. These budget cuts have gone too far.

Headache? Brain Tumour!

So I thought maybe I should write a post about the pituitary tumour headaches that I get. As I have almost certainly complained previously, for many years I never got headaches and then, gradually, I started having them more frequently. Every now and again, right after taking a couple of ibuprofen,* I would entertain the vague thought that maybe I should go to the doctor and get it checked out... and then laugh at myself for being melodramatic. Oops.

Because the thing is, they never happened that frequently. Maybe once every few weeks. I was under the vague soap opera impression that tumours in your head  gave you headaches continously, but apparently this is not necessarily the case! After my operation, I had headaches more frequently for a month or two (not particularly surprising given that surgeons had been rummaging around in there with sharpened implements), maybe a couple of times a week.**

My pituitary adenoma headaches are actually usually pretty similar. Previously, I would get pain all around the edge of my right eye socket, and right at the back of my eye - and it is somewhat disconcerting to feel where your eyeball must be - although since the surgery, it has improved (yay!) - I only really get it in the upper left-hand corner of my eye, which is where the pain used to be worst. I always feel slightly better if I massage that corner of my eyebrow; this has now turned into a habit which I catch myself doing even when my head is not hurting.

But ladies and gents of the internet - fret not if your headache sounds similar, for you probably do not have a tumour in your head (though that does not mean that your headache could not be seriously unfortunate). The Power of The Internet informs me that there are all sorts of different types of headaches even amongst pituitary tumour patients, so pfft, who knows. Just go hassle your doctor about it.

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* Paracetemol makes me cough. Apparently this is odd.

** I wasn't allowed to take ibuprofen due to being on steroids, and I wasn't supposed to cough lots, which ruled out paracetemol. This put me in something of a quandry for painkillers, but as they didn't tend to last too long I am INCREDIBLY BRAVE, I just did without.

Russell Watson

I was originally going to make the title "Russell Watson: What You Got, Son?" but obviously, that would be stupid. So I decided not to mention it.

Ahem.

Anyway, today's post is (surprisingly) about international singing sensation and all-round famous guy Russell Watson. Ever heard of him? NOR HAD I.

But last year he popped up on an episode of Never Mind the Buzzcocks, which aired on the evening of the 2nd December. I had an important exam the next day, so obviously I was watching. Mr Watson mentioned that he had had a brain tumour. I, as yet undiagnosed,* thought nothing more of it, not least because the notorious buffoon Tim Westwood was hosting the show and I was quite busy despising him.

About a week later, I got the call from the hospital announcing that I had a pituitary adenoma. Russell Watson was far from my mind. But then one day, as I was waiting to collect a takeaway in my local Chinese, I began paging through their ancient and tattered copies of the Daily Mail** and came across a month-old article about the same Mr Watson and his second round of brain surgery for a "benign brain tumour".

So obviously I had to look it up. After a bit of searching, my suspicions were confirmed: Russell Watson's pituitary gland also enjoys cultivating adenomas in its spare time. I can't really say why knowing this made me feel better, but I guess it's just vaguely reassuring to know that there are other people out there. The poor guy had a particularly nasty case of the pituitary tumour as well, he's had two surgeries and radiotherapy, and the first surgery apparently had a knock-on effect to his pituitary function, something which so far I've been lucky enough to avoid.

 However, I would like to take this opportunity to glower at the British press for their woeful lack of precision when reporting on brain tumours. It seems that any distinction beyond that of "benign" and "cancerous" is completely beyond them. For anyone who knows their oligodendroglioma from their meningioma, and particularly for anyone trying to identify famous fellow tumourheads, it makes life rather challenging. Are the details of a diagnosis too much to ask for?

Also, while I don't mean to sound like a stalker who's read every article on the subject, I have to say that I particularly empathised with the last lines from with Russell Watson in this article: "Specialists repeatedly told him that he was only suffering from stress, to which he replied: 'The only thing that's stressing me is this pain in my head.'"

Just like all the years of me visiting various doctors complaining that my hair was falling out. And my heart was doing funny things. And I felt tired all the time.***

UPDATE: You may also be interested in further posts I've made about famous people with pituitary adenomas, which you can find here and here, and there's also this post specifically looking at famous people with Cushing's Disease and another post looking at the strange shortage of famous women with acromegaly when compared to their male counterparts.
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*Though admittedly in the realm of "we're pretty sure it's a pituitary adenoma. Because it's the only idea we have left".

** Oh god. I know. I'm so sorry. I'll never do it again.

***Any doctor treating me in future may be interested to know that if any symptom I may display is ever put down to "stress" again, I will not be held responsible for my actions. Of course, being accused of suffering from "stress" comes with the unfortunate Catch-22 that anyone repeatedly insisting that they're not bloody stressed looks exactly like a stressed-out mentalist. Le sigh.