Monday, 30 July 2012

IMFW: A Functional Cure for HIV?

Exciting news that a French research study may have found a way to allow HIV-infected people to avoid developing full-blown AIDS. In the study, newly-infected patients were treated with antiretroviral drugs very soon after acquiring the infection, which is quite unusual, and after three years of treatment they were able to stop their medication and remain well. It's a functional cure, which isn't quite a "cure" in the traditional sense; they still have the virus in their bodies - it's just kept at extremely low levels. And it only works if the infection is caught quickly. But it's a strong indication that antiretrovirals should be given as soon as possible after initial infection.

Thursday, 26 July 2012

Failing Treatment: Patients, It's Your Fault

One of the things you notice, once you've been around doctors and hospitals for a while, is the way that medical terminology sometimes seems to place a slightly unreasonable amount of blame on the part of the patient.

For example, the website for Korlym, a drug used to control high blood sugar in patients with Cushing's syndrome, states that Korlym is used "in adults with endogenous Cushing's syndrome who have type 2 diabetes mellitus or glucose intolerance and have failed surgery or cannot have surgery".

1.) I wasn't aware that surgery was a test.

2.) Surely if it were a test, the person charged with passing it would be the surgeon, not the patient zonked out on the operating table.

It seems strange to me that they went for this phrasing (and not as a one-off either, it appears elsewhere on their website). Would it really be difficult to say "patients whose surgery was unsucessful" or "patients whose surgery did not effect a cure"? It's not an isolated case restricted to one copy editor on one website either; it's fairly common to read things like "the patient failed chemotherapy"/"radiotherapy"/"to tie their shoelaces that morning, leading to chaos on Ward Five."*

Doctors of the world, please note: patients don't fail treatments.** Treatments fail patients.

It's an entirely obnoxious phrase, and when I started researching the question it was heartening to see that I am by no means the only person to object to it. As if patients don't feel crappy enough after discovering that their treatment has not succeeded, it then seems to be implied that this is in some way their fault. Yes, it's a medical convention; no, doctors don't mean to imply that the patient is to blame for the treatment not working. But as this patient points out, it's an entirely unnecessary little phrase which can serve to undermine the doctor-patient relationship. Patients who are not au fait with medical terminology - i.e. almost all of us - are unlikely to be impressed if they hear it.

There are plenty of problems out there in the world which are far more serious and important than this little question of syntax. But is it really so hard to change "[Patient Y] failed [Treatment X]" into "[Treatment X] failed [Patient Y]"?


UPDATE: So, there's a twist to the tale! I wrote to Corcept Therapeutics, Korlym's creators about this, because I'm pushy that way, and their Director of Commercial Operations got back to me like a bolt of extremely speedy lightning, - despite the fact that I do not have Cushing's Syndrome and thus am hardly their target audience. Here's a couple of extracts from his email:

"Our intent was certainly not to place any blame on the patient for the surgery’s failure, but I can certainly see how the words could convey this. [...] In the world of pharmaceutical marketing and communications in the US, we are highly regulated regarding what we say and how we say it, among other things. For many things, we use language directly from our FDA provided Full Prescribing Information (also known as the label or package insert) and Medication Guide. These two FDA provided documents use the exact language  “patients who…… have failed surgery”. In the case of our website copy, we picked up the language directly from these documents.

"I think we have room to improve the language, using language that you suggest, or something similar. We have a compliance team that meets semi-regularly to review our communication material. At a point in the near future, we will review this language change, in hopes to get it approved for a future website update."

So thank you very much, Corcept, and the moral of the story is this: if there's something you don't like, don't keep it to yourself - whinge!
*Well, maybe not the last one.

**Unless of course they're non-compliant with the treatment regime. In which case, have at them.

Wednesday, 25 July 2012

I Need More Surgery

I just had a phone call from the hospital and it's official: I'm going to be having more pituitary surgery. The results of the tests I had at the beginning of the month show that I'm still "thyrotoxic" as they call it (my thyroid hormone levels are too high) - which to be honest I could have told them myself, as I'm still having symptoms aplenty. The sad news though is that they think the pituitary tumour has grown since my previous MRI scan. My last scan was in March. For it to have noticeably grown in just three months is kind of worrying to me (and also makes me slightly more pissed off that they stopped my injections for four months when I was told it would be half that time - the tumour had been stable while I was on the injections...).

So, that sucks. I am reeeeeally not looking forward to more surgery. Last time I didn't know what to expect; this time I know exactly how many blood tests and needles and rubbish things will be involved, plus oh god when they take out the bandages from your nose! I believe it's slightly more risky the second time around, plus I'm going up against the same odds of ending up with diabetes inspidus/hypothyroidism/addisons disease/other pituitary fail.

I am also slightly concerned because I have had a slight sinus infection since last August thanks to the surgery and it just occurred to me that I don't know if they will even be able to operate unless they get rid of that first?

Here are the bright sides:
- Surgery at least carries with it the possibility of cure.
- I am marginally more in shape than I was last time around, so you never know - my crappy muscles might recover a little faster
- More time off work? (Admittedly, it's just time off to lie on the sofa complaining, but still)
- This time I will remember to ask about painkillers that I am not allergic to and am able to take.
- This time I will take photos in hospital and show them to YOU, dear reader.

So, here we go. Brain Surgery, Round 2!

Tuesday, 24 July 2012

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.

Monday, 23 July 2012

IMFW: Lipodystrophy

Today's Interesting Medical Fact of the Week is about lipodystrophy, defined by the ever-noble Wikipedia as "a medical condition characterized by abnormal or degenerative conditions of the body's adipose tissue." Adipose tissue = fat. As you would know if you watched the freaky Dr Who episode which featured little fat aliens called the Adipose.

Dr Who: making obesity cute.
But I digress.

I first heard about lipodystrophy when chatting to a very lovely nurse in the research department of the hospital where I went to have a bunch of tests at the beginning of the month. It's a subject they have been doing some research into, and I have to admit it kind of caught the imagination.

The term "lipodystrophy" actually covers a whole range of conditions in which the body's fat does weird things. It may include a loss of fat from certain areas of the body (lipoatrophy), or even an increase in fat in certain areas (lipohyperthrophy), or even both (lipoWTF-ery):

Congenital generalised lipodystrophy:
A rare genetic disorder in which there is extremely low fat in the subcutaneous tissue.
Other symptoms include having a giant liver and being very hairy. And, unfortunately, heart problems.

Benign Symmetric Lipomatosis:
In which the patient develops extensive fat deposits in the neck, head and shoulder area. Often associated with alcohol abuse.

Barraquer-Simons Syndrome:
This rare condition usually appears after a viral infection in childhood, and is characterised by the gradual loss of fat from the top half of the body; the face, neck, shoulders, arms and chest, often accompanied by an increase of fat in the thighs, and sometimes by other symptoms such as deafness and epilepsy.

HIV-associated lipodystrophy:
This condition occurs in people with HIV, usually those who are being treated with anti-retroviral drugs, although there is some debate about whether it is caused by the drugs or by HIV infection alone. Often there may be fat accumulation in the back, creating a 'buffalo hump', the neck and breasts, and patients often develop abdominal obesity. However, at the same time they may have loss of fat in the face, arms, shoulders, thighs and buttocks.

Those are just a few examples - lipodystrophy is quite poorly understood and quite difficult to treat. Liposuction may be used to treat excess fat deposits, or lipomas (benign tumours of fat tissue) may be removed surgically; equally implants or even fat injections may be used to improve the cosmetic appearance of fat loss from the face and body.

Friday, 20 July 2012

Human Guinea Pig: Part 7 - How To Get Over Your Fear Of Needles

Holy lord! Who would have thought, when I agreed to go into hospital for all this testing, that the experience would prove to be such a rich vein of blog posts?

Anyway! This is the sixth part of the story about my overnight stay in the hospital's research department, having lots of tests to look at the effect my pituitary tumour is having on my body and metabolism. Here are links to: part one, part two, part three, parts four, five and six!

A Whole Lot of Blood Tests

After my GEM ventilated hood calorimetry test was over, and the strange astronaut hood was removed from my person, you might be imagining they'd let me have breakfast. If so, you are imagining wrong. Please stop.

No, it was time for my blood glucose testing! And so I was presented with this:

Yay, breakfast
No, it's not a refreshing beer: it's a pint of Lucozade. At nine in the morning. And I had to drink it all within five minutes.

Basically the idea is that, having eaten/drunk nothing but air for twelve hours, they present you with a massive sugary drink and then do a bunch of blood tests over the next two hours to see how well your body deals with it. I'll be interested to find out the results of this, as I'm pretty sure my body isn't great at coping with sugar; sometimes eating sugary things really noticeably raises my heartrate - and especially when I'm on my lanreotide injections, I have to try to be really careful about eating regularly, and carry around a packet of Starburst (or similar sugary hit) because occassionally I go hypoglycaemic at unexpected moments. Lanreotide messes around with your insulin and glucagon levels, meaning that it can confusingly cause both high and low blood sugar, and it sure is annoying.

I looked it up, and it turns out Lucozade was originally conceived as an Irish health drink and called Glucozade! Who knew! Between that and Guinness, it seems my ancestors had some peculiar ideas about what was "healthy". It also turns out that Lucozade contains 85g of sugar per 500ml, which is almost all of a woman's daily allowance. Holy crap! You can see why they chose it for my morning tipple.

While I was distracted trying to gulp down the hideous stuff, the sneaky nurses set up the trolley ready for my blood tests. Having finally finished all my Lucozade, I turned around to see this:
Oh, dear lord.

Yep. All those tubes were for me. I got to have twenty-three bloods taken in total - lucky me!

First they put an IV cannula into my arm - basically it's a giant needle that stays in your vein and they can attach tubes and things to so that they don't have to re-stab your arm every time they take another blood. They took about 16 of the bloods immediately, and then I had the rest over the next two hours. I still can't really bring myself to look at cannulas in my arm due to being a wuss, so one of the nurses very kindly bandaged it up for me.

Safely hidden away!

The nurses and my endocrinologist formed a sort of production line around my arm for the first sixteen, with one nurse preparing the tubes, Dr Olive taking the blood, and the other nurse stacking them up (some had to go straight into a bucket of ice; some had to be left to clot).

Once they've finished taking one lot of bloods via a cannula, the tube is flushed out with saline solution (i.e. they effectively inject saline into the tube). This clears the blood out of the tube, preventing it from clotting and blocking the tube in-between blood tests. Of course, then the next time they come to take blood, this means that they get half a tube of saline back before the blood starts coming through properly, which is of no use to anybody. Because of this, before taking further bloods, they take an extra tube which comes out as a mix of the saline & blood, then continue with the tests - and then sometimes (if you're having a lot of bloods taken, like me) they'll re-inject the blood and saline, to minimise blood loss.

I've never written the word "blood" so much in my life as I just did in that last paragraph, but I promise I'm not just trying to gross you out; there's an interesting fact coming up. Before re-injecting the blood & saline, I was asked if I was ok with it. I was very much ok with it, because sometimes having lots of blood taken gives me a Funny Turn (in this case I was fine, probably thanks to the pint of Lucozade) and thus I like to hang on to the stuff. Turns out they always have to ask before re-injecting the blood, in case you're a Jehovah's Witness.

I have long been aware that Jehovah's Witnesses are against blood transfusions, but I'd always assumed that this was due to believing that it was wrong to have someone else's blood put into your body; I hadn't appreciated that in fact the problem is with any blood that has left the body at all, even your own. According to Wikipedia, for Jehovah's Witnesses "Blood represents life and is sacred to God. It is reserved for only one special use, the atonement for sins. When a Christian abstains from blood, they are in effect expressing faith that only the shed blood of Jesus Christ can truly redeem them and save their life".

My Lucozade & Blood Tests Experience:

Hassle: 4/5
Fun: 1/5 (It would have been a zero, but I learned an interesting fact!)
Weirdness: 2/5
Results: 2/5
Total Score: 9/20

Monday, 16 July 2012

Human Guinea Pig: Part 6 - Resting Energy Expenditure Test

This is the sixth part of the story about my overnight stay in the hospital's research department, having lots of tests to look at the effect my pituitary tumour is having on my body and metabolism. Here are links to: part one, part two, part three, part four and indeed part five.

Resting Energy Expenditure and the GEM Ventilated Hood Indirect Calorimeter

I was woken up at half eight the next morning by the very lovely technician* who did my step test and heart monitor the previous day. She was dragging a large piece of equipment into my room, where it was placed next to my bed; it was time for my resting energy expenditure to be monitored. This has to be done first thing in the morning for maximum accuracy, which is why they kept me in hospital overnight.

 There's a tricky balance to be met with regards to this test; on the one hand, you have to be awake for the whole thing, but equally you're not supposed to get out of bed - or even move, as far as possible. For a sleepy creature like me, this makes it pretty hard to stay awake, but fortunately the whole experience was so surreal that I didn't want to let myself drift off.

Firstly, the equipment is switched on, set up, and left to monitor the room for ten minutes. Essentially, your metabolism is monitored by measuring the amount of carbon dioxide you breathe out. These measurements are then used to calculate your respiratory quotient. The technician explained it to me like this: when you're asleep, you usually breathe out a lower level of carbon dioxide, indicating that your body is metabolising your fat stores to produce energy (RQ = <0.7). When you're out and about, you'll be breathing out a higher level of carbon dioxide, indicating that you're metabolising carbohydrates (RQ= >0.7). I have a pituitary tumour which is messing with my hormones so they want to know what the heck my metabolism is actually doing. Or something.

Anyway - in order to make sure that the readings they take of the air you breathe out is accurate, the composition of the air in the room you're in has to be measured before and after the test. So I lay in bed for ten minutes, listening to it whirring happily next to me.

And then the technician returned with the rest of the equipment.

I am genuinely unspeakably ensorrowed by the fact that I didn't get a picture of me in it, but unfortunately it was early in the morning, my brain was still in first gear, and I wasn't actually allowed to move. So you'll have to use your imagination. Essentially, the rest of the equipment (the "ventilated hood") is a large goldfish bowl with a cape hanging off it, a small hole at the top, and a large pipe or two running out of it. Imagine a giant plastic medical jellyfish and you'll be on the right track.

I did find a picture of the machine all bundled up, but it doesn't look as amusing as it did once it was set up:
This piece of kit is called a GEM (Gas Exchange Measurement) ventilated hood indirect calorimeter. Snappy name, right? The GEM Nutrition website describes it as "an open circuit indirect calorimeter designed for nutritionists needing to measure energy balance and substrate turnover. The compact bedside unit measures gas exchange volumes, respiratory quotient and energy expenditure."

So now you know.

This strange contraption was connected up to the machine, and then placed over my head, at which point I understood why the nurses had double-and-triple checked with me whether or not I had claustrophobia the previous day. Then, safely ensconced in my plastic bubble, the cape was draped around me, and after checking that I wasn't freaking out and providing me with a call button, I was left.

I can't actually remember whether it was for twenty minutes or half an hour, but it didn't feel like very long. I could hear my breathing inside the big clear plastic helmet, which made me feel like a very lazy astronaut. Every now and again someone popped their head around the door to make sure I was ok, but it actually felt quite relaxing. The lights were still off in the room and it was all very chilled. I tried to keep my resting energy expenditure normal.

At some point later, I was freed from my goldfish bowl prison, and the GEM calorimeter sat burbling away in the corner for another ten minutes, measuring the room's air again.

The machine comes complete with a program which shows you the results immediately, along with little graphs showing the levels of carbon dioxide you were breathing out vs. the oxygen you were breathing in, etc. This would have been pretty awesome if I had had any clue what the results meant, but sadly they have to play with them further before they can draw any actual conclusions.

My Experience of the GEM Ventilated Hood Indirect Calorimeter:

Hassle: 1/5 (all I had to do was wake up and keep breathing, which I've managed to do every day of my life so far)
Fun: 3/5
Weirdness: 5/5
Results: 3/5
Total score: 12/20
*I don't know what her official job title is, so I'm going with "technician" but I could well be extremely wrong; I just know she was neither a doctor nor a nurse.

IMFW: Where are you on the Global Fat Scale?

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

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

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

Saturday, 14 July 2012

Human Guinea Pig: Part 5 - Dinner at the Hospital

This is the fifth part of the story about my overnight stay in the hospital's research department, having lots of tests to look at the effect my pituitary tumour is having on my body and metabolism. Here are links to: part one, part two, part three and part four (I do go on).

Dinner at the Hospital

After a hurried trot back from the MRI department, I was dropped off at my room, ten minutes late for my dinner. The timing of this dinner was apparently important because it had been prepared just for me, and was apparently perfectly calibrated to contain exactly the right amount of calories for someone of my height and weight. I was allowed to eat the prepared meal only, nothing else, because they planned to monitor my metabolism and blood sugar levels in the morning and needed these to be unaffected by what I had eaten the night before.

I had barely had time to change out of my ridiculous scrubs and re-attach my Actiheart monitor when the night nurse's head appeared around the doorframe. "We're just heating your meal," she said, in a firm, no-nonsense voice. "You will be expected to eat all of it, you understand?"

And, with a stern look at me, she left. A few minutes later, she reappeared, tray in hand.

 Every dish had a label to show who it was for and when I should eat it, even to the extent that the milk for my evening (decaff) coffee had been measured out in advance, and I was not permitted any more. The main meal was, happily, spaghetti bolognese, one of my favourites.

But when I saw the dessert, my heart sank.

Close friends and family are aware that when it comes to desserts, I am usually more than enthusiastic in my appreciation of the culinary arts (unless it's cheesecake. Eurgh). However, I have a few basic rules. I don't eat jelly with sponge cake. I don't eat sponge cake with fruit.* And I don't eat fruit with jelly.

Here is a picture of the dessert:

In case you can't tell, it's strawberry jelly with tinned mandarin slices. NOOOOOO!
In fairness, it was partly my fault: the hospital did ask me whether I had any dietary requirements or dislikes, and I said no, because it would be strange and embarassing to have to explain my dessert law triad to a doctor. At least it wasn't cheesecake.

And so, I settled down to sup with good grace and managed to eat the entire thing. I did my utmost to separate the jelly from the mandarin segments wherever possible; where it was not possible, I adopted the approach to eating which has been popularised by the Burmese python, and gulped it down in one bite.

I don't know if the night nurse noticed my face fall when I saw the dessert, or whether she just thought I looked like an untrustworthy character, but she kept sticking her head around the door at unexpected moments while I was eating.

"Take your time," she said, smiling. "But you do have to eat it all."
"Ok, thanks," I replied.
My special tray!
Two minutes later, she popped up again. "You can take your time," she said. "Take as long as you want. But make sure you eat it all."

 And so on. It was very disconcerting. The constant visits created a weird kind of feedback loop where I found myself choking down the mouthfuls as quickly as possible in the hopes that she would finally stop telling me I could eat as slowly as I liked.

Eventually, I was done, and I had the evening to myself. I harassed my parents and boyfriend with text messages about jelly and fruit, read about the exploits of Mr Sherlock Holmes on my Kobo e-reader (which I LOVE), and flicked through TV channels. Eleven o'clock was lights-out and the terrifying night nurse came back again to confiscate my water and order me to pee in a jug overnight,** which was probably the moment I most felt like I was in prison.

Adding to this impression was the fact that my door had a small square window in it which opened onto the corridor and let an annoying amount of light in overnight, and the absurdly thin blanket on the bed. I often get chilly at night and hospitals are not cosy places, so I had brought my warmest hoody to wear in bed over my pyjamas, but I still woke up in the night absolutely freezing and had to ask for another blanket. This is extra annoying considering that hyperthyroidism is supposed to make you too hot, not too cold. In fact, I was given a questionaire about my symptoms, and one of the questions asked me to score my "heat intolerance" on a scale of 0-5, where 0 is no intolerance to heat and 5 is getting easily overheated. I wanted to add a -1 to the scale.

*For those of you who really care about my pudding pickiness, I should note here that this rule only applies where the fruit makes the sponge cake all damp and hideous. I like fruit baked into cakes just fine. Fruit placed on top of a waterproof layer of icing is also acceptable. Trifle is the devil's pie.

**Not just for her own amusement; they did a 12 hour urine collection to monitor various hormone levels overnight.

Wednesday, 11 July 2012

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...

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

Tuesday, 10 July 2012

Human Guinea Pig: Part 4 - Pituitary MRI Scan

This is the fourth part of the story about my overnight stay in the hospital's research department, having lots of tests to look at the effect my pituitary tumour is having on my body and metabolism. You can read the first part here, the second part here and the third part here.

Pituitary MRI Scan

After my Actiheart was fitted, they had to take it right off again as I went straight down to have my pituitary MRI, another scan where no metal is allowed. I was strangely comforted by the fact that the MRI receptionist was fairly rude to Dr Olive when she asked to speak to the technicians - not because I dislike Dr Olive, who is lovely, but just because it's nice to know it's not only patients that bear the brunt of receptionists' bad days. I have a theory that in every hospital the receptionist recruitment protocol calls for exactly 50% completely lovely, can't-do-enough-to-help-you staff, and 50% short-tempered and angry people. That or they slip some kind of Jekyll-and-Hyde potion into their coffees.

"Have you come from Norway today?" the technician asked me.
I think she was quite disappointed that I had not, though I've no idea why.

I was still dressed in the giant scrubs and they keep scanning rooms pretty cool, as the equipment needs low temperatures. Fortunately the technician was both lovely and observant, and she gave me a blanket to keep me warm in the scanner. The usual ritual of ear plugs and glasses removal was observed, and packing was duly stuffed into the strange helmet thing you have to put your head in. Then I was drawn backwards into the machine.

I didn't get a photo, so here's a cheesy stock image of some people smiling through an MRI scanner
MRIs can be quite claustrophobic, even if you're not normally bothered by confined spaces. I guess it is partly down to the fact that you don't have any control over it, partly down to the fact that your head is in a box and you're not allowed to move, and partly down to the weird and incredibly loud noises exploding into existence around you. However, if you do suffer from claustrophobia and you need to have an MRI, I can strongly recommend asking for a blanket. It makes the whole experience seem much more cosy. You can almost believe you're lying in bed on fireworks night. With your head in a box.

Then came the contrast injection, at which point I was given my panic button - usually they give you one at the start of the scan, but I was so happy under my blanket I hadn't even noticed I didn't have it - in case you have a reaction to the contrast dye (unlikely unless you have undiagnosed kidney failure, but still). Then another set of scans - each set took about ten minutes, I would guess - and then freedom! Easy as pre-packaged pie.

The strangest part of the scan actually happened after it was all over. There was another patient with the same condition as me who was having the tests at the same time - this meant we could both have our PET scans on the same day, as the hospital prefers doing two of the same type at once to save money. We'll call him Bob. I hadn't met him at that point, but he was having his pituitary MRI immediately after me, and so I stayed in the waiting room with a very sweet Filipino nurse waiting for his scan to be finished so we could all go back to the research department together.

I was, as I have mentioned before, still wearing scrubs. I was however wearing a pair of exclusive "I'M A PATIENT" bracelets detailing my name, age, hospital no. etc. and my allergies (trimethoprim = brings me out in an attractive full-body rash). The waiting room was pretty full, so I was sitting off to one side, flipping through an elderly magazine,* when a woman came up to me and asked me how much longer the current scan would take.

Well initially, I admit, I gave her a "woman, you crazy!" look, until I suddenly realised that in my current garb I probably looked like a member of staff.
"Sorry, I'm not a doctor, I'm a patient," I said.
"So how long will it take?" she asked.
I got out my "woman, you crazy!" face again. "Er, I don't know…"
"Well, I think it's really bad that we're not being given any information," she told me. "How long do you think it will take?"
"It probably depends on the type of scan?" I suggested.
And with that, she stamped off, muttering angrily to herself.

On the way back to the research department, I got to meet Bob! He is the first ever person I have met who also has a TSHoma, which is not surprising as there's only about 20 of us across the whole of the UK, Ireland and, apparently, Norway (this may explain the MRI technician's cryptic question). Bob was a bit of a legend and had sensibly rejected the scrubs they had suggested he wear, in favour of pyjamas with Animal from the Muppets on; obviously the preferable sartorial choice.

You might think that, on finally meeting someone else with the same incredibly rare pituitary tumour, we would discuss symptoms, or treatment. But no! People with TSH secreting pituitary adenomas are nothing if not polite, and of course we would not want the lovely Filipino nurse feeling left out of the conversation. So, as she most likely had no brain tumours of any sort, we instead discussed the best way to travel to Cardiff by public transport.

And a very educational conversation it was too.

My pituitary MRI scan experience:

Hassle: 2/5
Fun: 2/5 (extra point for meeting Bob!)
Weirdness: 3/5
Results: 2/5
Total Score: 9/20

I fear I've just had too many MRIs to find them exciting any more!

*The magazine was old... it wasn't a magazine for old people.

Sunday, 8 July 2012

Human Guinea Pig: Part 3 - Actiheart Monitor & Step Test

This is the third part of the story about my overnight stay in the hospital's research department, having lots of tests to look at the effect my pituitary tumour is having on my body and metabolism. You can read the first part here, and the second part here.

Actiheart Monitor & Step Test

Once my body composition scan was finished, there was a certain amount of fretting that I would be late for my MRI scan appointment, so I was immediately whisked off to have my step test and Actiheart monitor fitted. This meant I was still wearing giant scrubs and no bra when I was supposed to be doing mildly athletic deeds, which proved somewhat inconvenient.

The Actiheart monitor is a teeeny tiny heart monitor which attaches to your chest by means of two sticky pads coated in glue so vicious it's almost impossible to wash off without taking the top layer of skin off too. I was informed I would be wearing it until midday Friday (I had not had any kind of advanced warning about that!) and that they wanted to carry out a step test to calibrate it. Essentially, while standing in a room full of cycling machines and a giant treadmill, you have to spend eight minutes stepping alternately onto and off of a purple plastic step, in time with a computerised voice which says "Up. Up. Down. Down." and gradually gets faster. The Actiheart records your heartrate as you do so, and then this is compared to the recording of your heart over the next few days, and used to gauge your activity level against your heartrate.

It's so tiny!
This explanation seemed pretty bizarre to me, because they were recording my exercising heartrate while I was off medication and therefore had an unusually fast heartrate, and then using this to calibrate the results of the heart monitor recording my heartrate when I was back on the medication. I have not yet had a satisfactory explanation of why this isn't stupid, but I live in hope.

Anyway. The computerised voice started up, and after only a minute or so began to take on a strangely hypnotic quality as I stepped endlessly up and down. By 5 minutes 30 seconds, the voice saying "Up. Up." started to sound more like an evil "Ha. Ha." and I became convinced the computer was laughing at me. The whole time, I had to hold up the trouser legs of my giant scrubs because they were too long as well as too wide, and I was concerned I would trip and fall on my (apparently worryingly fat) arse. Pro tip for the creators of Actiheart: I have to say that the whole experience would be vastly improved if you set the "Up. Up. Down. Down." man's voice to trance music.

Having just been told that I was 36% lard, I became quite concerned that I must be hideously unfit and would utterly disgrace myself by being unable to complete the step test, but in fact it was totally fine and even when he got quite fast at the end I was still happilly stepping away.

There was no printout of results to take away with me; I have to wait until the five-day recording has been analysed and then I should get some feedback through the post. I can't believe quite how small and awesome it is, compared to the 24-hour heart monitors which I've had to wear previously, which have invariably been vast, bulky and inconvenient.

My experience of the Actiheart Monitor and Step Test:

Hassle: 5/5
Fun: 3/5
Weirdness: 4/5
Results: 2/5
Total score: 14/20

Saturday, 7 July 2012

Human Guinea Pig: Part 2 - DEXA scan

This is the second part of the story about my overnight stay in the hospital's research department, having lots of tests to look at the effect my pituitary tumour is having on my body and metabolism. You can read the first part here.

DEXA (Dual Energy X-ray Absorbtiometry) Scans - Bone Density & Body Composition

The first scan I had in the research department was a DEXA scan. People with hyperthyroidism are at risk of osteoporosis, so they wanted to check whether I had lower than expected bone density, a high risk factor. They also performed a body composition scan using the same machine, which analyses your whole body to determine what percentage of your body is made up of fat.

As the machine uses x-rays, you can't wear anything metal. They gave me a pair of scrubs to change into, as my trousers had metal bits in them. Initially, I was secretly very excited about this chance to see what I would look like as a nurse. However, the scrubs they gave me were absolutely huge - so in fact I found out what I look like as a formerly obese nurse who needs to update her wardrobe post-Weight Watchers. The trousers were so large that I could fit in one leg. Don't believe me? Here's a photo: 

Anyone care to join me in here?
I asked the lady doing the scan if I needed to remove my jewellery as well, and she said not to worry, it was just the trousers that were the problem. Seemed odd, but ok. Then I got into the scanning room and she asked me to take off my jewellery. No problem. Then I had to get lined up for the scan. Because it looks at your spine ,it's important you lie down in a perfectly straight line on the machine, which took a few minutes. Then, just as the scan was about to start, she turned around, looked at me and said "Are you wearing a bra?"

As I had not been told to take it off, I was indeed wearing a bra. It hadn't even occurred to me that the underwiring would get in the way of the scan. So I had to get up and take it off, then go through the whole aligning-your-spine-to-the-scanner process again. The DEXA scanner is basically a big table which you lie on, with an arm over the top which very ponderously slides (or rather, judders) along its length, just above you. The x-rays are fairly low dose, less than a long-distance flight. The whole process is quite quick; first the body composition scan was taken as I lay down normally, then they scanned my spine. In order to scan it properly, you have to lie with your legs up on a block so that your spine is flat against the table surface. Finally, they scan your femur at the hip joint, which requires that you rotate your leg slightly in an unnatural fashion. In order to assist with this, they tie your foot to a plastic block. It's all pretty weird.

In the corner of the scanning room was a human-sized giant egg with a little window in it. Apparently it was a BOD POD, a different kind of body composition scanner. I was pretty intigued by this piece of kit and faintly disappointed I didn't get to try it out.

The best thing about the DEXA scanner is the fact that you get an immediate print-out of your results and graphs plotting you against the expected and normal values for your age group. As it happens, I actually have slightly higher than normal bone density, rather than lower, which was good news (the blue is the normal range):

Superhuman bone density! ...or something

I was also complimented on my "beautiful" spine (actual quote - hospital folk give you very weird compliments).

Work it, baby.
However, the most interesting results were from the body composition scan. My body composition came out as 36% fat, which is at the upper end of the normal range for my age group, with the average being 30% (for women; obviously men need less fat). As you can imagine, this got me somewhat worried that I should hit the salads with a vengeance, but when I spoke to my endocrinologist the next day, she said that it was most likely due to the fact that extended period of hyperthyroidism tends to lead to muscle loss and that she didn't think I needed a lifestyle overhaul!

The me on the left needs to eat more.
Update: googling values for normal body fat percentages informs me that the American Council on Exercise considers a body fat value above 32% for women to be obese and I've found alternative readings which say anything over 35% is obese! This does nothing to make me feel better. I also found a formula used to estimate body fat based on BMI, which puts my body fat at 24.96% (full disclosure: my BMI is 20.7) i.e. a low average score, close to fitness level. Just goes to show BMI is silly and you can't rely on formulae to estimate these things!

I also worked out that, assuming I don't gain any muscle, I would need to lose 7.5 pounds in order to get down to being 30% fat. That would put my weight at 8 1/2 stone (or 54 kgs), which would put my BMI at 18.6, which would be just 0.1 away from being classed as underweight. So it seems that gaining muscle is the only solution to the problem. I can't win!

I do at least feel better about the fact that I haven't put any muscle on in my arms despite exercise, although I have put some muscle on in my stomach since the beginning of the year (bellydance, whoop!). Feels like more of an achievement now!

My DEXA scan experience:

Hassle: 3/5
Fun: 1/5
Weirdness: 3/5
Results: 4/5 (would have been 5/5, if it hadn't said I was fat!)
Total Score: 11/20

You can read the next part of the story, about my MRI scan, by clicking here.

Friday, 6 July 2012

Human Guinea Pig: Part 1 - The Research Facility

 I turned up at the hospital at 2pm on Tuesday and met up with the endocrinologist who's been in charge of arranging the series of tests which I was due to undergo  (she needs a top secret blogging name; we'll call her Dr Olive). We went to the research department where the tests would be carried out, and I was delighted to be introduced to my private room - complete with en-suite bathroom and free TV (and sharps bin, and cardiac arrest instructions).

My charming room
I think I've mentioned this before, but just in case - the tests which I had done over the past few days were done partly as general research into my condition and partly as a guide on what kind of treatment I should be given next. Firstly, a nurse was called in to do my initial tests; height (5'7"), weight (57.9 kgs), blood pressure (105 over 77), blood oxygen (97%) and resting heartrate (116 beats per minute - normal rate is 70-100 bpm). She had obviously not been informed about my tachycardia, because when she saw how fast my heart was going she looked at me askance and said "Did you come here running?" I assured her I had not, and that 116 bpm was perfectly normal for me. Later when Dr Olive looked at the results I think she was quite surprised; I had explained to her that I had tachycardia but she was surprised that my resting heartrate could be so high; of course, at that point when she took my pulse it was a perfectly standard 79 bpm.

I also had my breathing rate observed which was deeply disconcerting, as it essentially involves a nurse staring at your boobs for a solid minute while you attempt to "breathe normally". It is strangely difficult to breathe normally when you know someone is counting every inhalation.

Next came my ECG (electrocardiogram, a.k.a. EKG), where they put a bunch of incredibly sticky pads on your wrists, ankles, and across your chest around the heart. Then they attach a bunch of wires to the pads, which feed into a machine, which then prints out a trace of your heartbeat. It doesn't take very long but it is a bit of a hassle - the worst part is peeling the sticky pads off afterwards, although as the very nice nurse - let's call her Carla - pointed out afterwards, it's definitely a lot worse for blokes with hairy chests!

After that, I had to give my consent to the various tests that they wanted to do, and answer a few questions about my medication, my pituitary adenoma and any other medical conditions. It wasn't particularly exciting, although when Carla asked me if I ever suffered from any kind of recurrent pain, I said no. It was only after she'd asked me two further questions about pain that I finally remembered the fact that I have hypermobility syndrome and consequently have fairly regular bouts of joint pain, and have done since I was a child. I apologised for apparently being a massive idiot and she laughed and said people often forget these things when it becomes normal for them.

Some people with hypermobility/Ehlers Dahnlos Syndrome are incredibly flexible - later, Dr Olive came to ask me another set of questions, and when I mentioned the hypermobility again I think she was faintly disappointed at my lack of hilariously bendy joints. My little fingers do bend backwards alarmingly, but the joints that are most affected are in my legs and feet so they're not very impressive - and at the moment I can't even get vaguely close to touching my toes thanks to damaging a muscle in my left leg dancing.

Once I had been thoroughly questioned, I was left alone to contemplate my crimes - or at least, someone else's crimes, thanks to Arthur Conan Doyle, Sherlock Holmes and my Kobo e-reader, a birthday present from my parents and an excellent device for anyone planning to spend time in hospital.

Not quite an ocean view...

You can read the next post about my time in the hospital, and my DEXA body density scan, by clicking here.

Thursday, 5 July 2012

Medical Test Top Trumps

In relation to last night's post: Panic over! Turns out the darn thing is completely waterproof after all and I should have just left it on. Another example of my excellent sievebrain: good at draining pasta, not so good at retaining pertinent information about medical devices.


I am back from l'hôpital and I did indeed have an interesting time of it. While I did not appreciate waking up to an array of 23 blood tests yesterday morning, I can't deny that they hospital have been pretty darn thorough in their endeavours to understand what my pituitary tumour is doing to my metabolism! They also thoughtfully froze some spare blood - in case they later think of other blood tests they wish they'd done at the time. The tests were partly for research purposes into TSHoma and other rare thyroid conditions, and partly to decide how they want to move forward with my own treatment.

Here is a list of the tests and scans they did:

MRI Scan
CT Scan
PET Scan
DEXA Scans:
     - body composition
     - bone density (spine and hip/femur)
ECG heart trace
Step test
Actiheart 5-day heart rate and activity monitor
12 hour urine collection
Glucose tolerance test
GEM resting metabolic rate testing
TFT bloods
Blood pressure, blood oxygen, etc.
Symptoms/Quality of life questionnaire

Don't know what half of those acronyms stand for? Never fear, neither did I. All will become clear shortly. I had so many tests that there's no way on earth I could describe them all in one post, unless it was the longest and most terrifying blog post in the world, so I figure I'll go through what happened chronologically. In order to introduce an element of competition to the proceedings, I have decided to rate each medical test against a set of four criteria: Hassle (how much hassle the test was to undergo), Fun (how fun the test was. I may have to introduce minus scores), Weirdness (self explanatory) and Results (does the test produce a pretty picture/interesting information once completed).

Each type of test will be scored on a range of 0-5, and by the time I have finished it should be possible to play Emer's Medical Test Top Trumps. Just what you've always wanted!

p.s. - forgot to mention, I took my camera with me as promised so I actually finally have a few photos to show you!

Wednesday, 4 July 2012

Almost forgot I was wearing a very tiny, very expensive heartrate monitor as I was about to have a shower. Fortunately I remembered before getting in!

More updates on the very exciting past two days will be arriving shortly...

Tuesday, 3 July 2012

Once More Unto The Breach

Good morning my fine friends! I have now heard back from the endocrinologist about where I'm supposed to be going and at what time. 2 o'clock is zero hour! Also I don't think I mentioned in my last post that on Saturday she also informed me that I was banned from vigorous exercise and caffeinated drinks until I leave the hospital on Wednesday. Obviously I was distraught on both counts, but the lack of tea is starting to take its toll. One of my colleagues very kindly let me drink her herbal red berry tea all day at work yesterday when I realised I'd forgotten to bring in my own decaf alternative to the Altar to Caffeine which is provided by our tea fund, but it's just not quite the same.

Anyway, I won't be blogging in hospital but just you wait til I get out... Hopefully I'll have something halfway interesting to write about.

Sunday, 1 July 2012

Terminal Stupidity

So the endocrinologist managed to send all the information on the metabolic testing I'm due to have on Tuesday-Wednesday to the wrong address. Realising her error she called me and agreed to drop off the information today. When I arrived home an envelope was waiting, but sadly while it does contain some info about the tests I will be undergoing, it does not contain any details of where this testing will take place or indeed at what time on Tuesday I should arrive at this undisclosed location...