My latest check-up with endocrinology

So I made it to the hospital on Thursday for my appointment, and it went about as well as I could have hoped for. First stop: getting weighed (58.4kg). Second stop: blood pressure, resting and standing (both totally normal - a good sign as when I'm taking propranolol it's often very slightly low). Third stop: a comfortable chair in the waiting room for an hour.

Just as I was starting to think that I might actually have been forgotten, my endocrinologist swooped in. It was a pretty quick appointment really. My blood tests look good. I had a good response to the synacthen test and can stop taking hydrodortisone (if you don't understand all this medical terminology, in short: that is good!). All my thyroid hormones are solidly in the normal range - including the alpha subunit. The alpha subunit (a.k.a. chorionic gonadotropin alpha) is a protein. People who have TSHomas/thyrotropinomas often have an unusually high level of it in their blood. The normal range of the subunit in blood tests is below 1. When my blood was first tested for the alpha subunit, I think my level was about 13. My most recent test showed it at 0.95 - not only normal, by far the lowest it's ever been for me. Even after my first surgery it was around 5 or so.

So thus far, everything looks good and they don't want to refer me for radiotherapy. I don't particularly want to have radiotherapy, so that is excellent news. My MRI shows scar tissue, but nothing that looks like residual tumour, and my pituitary gland appears to be functioning normally despite two surgeries. Of course, if a few cells or a teeny tiny bit of tumour has been left behind, there's basically no way to tell unless it grows back, so the policy is just to watch and wait.

Next appointment is in six months. I had another round of blood tests and they kept some of my blood on ice for the various studies they do on TSH-secreting pituitary adenomas. I'll have another set of blood tests before the next appointment and see how they change. In the meantime, basically I just keep on keeping on!

Pituitary Awareness Quiz: Day 10

Another day missed! Apologies. I do have an exciting update but I'm not sure I'm up to typing it all this evening. Consequently, I present today's super simple question:

Pituitary Awareness Quiz

Day 10, Question 10

Q.10: What hormone does my pituitary tumour overproduce?

a) Thyroid stimulating hormone

b) Thyrotropin releasing hormone

c) Thyroid hormone

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.

The Sound of Striped Hooves

Around a year or so ago, an interesting thing happened to me. I was in the car with my mother, we were driving down the motorway, and there was a car pulling a horsebox in front of us. We were going rather faster than the horsebox, and as we passed it, I looked inside and saw… a zebra. It was only a glimpse and it confused the hell out of me for a moment, but I am still sure it was a zebra, not a horse, inside that horsebox.* At first I was slightly worried I might be going mad, but my mother pointed out that there are plenty of zoos and animal parks around the place and presumably they must transport their zebras somehow.

Folk of a medical persuasion will probably see where I am going with this, but I promise it is an entirely true story, the proof of which is that it took months and months for it to occur to me that I could use it on this blog. What can I say? I am slow.

Healthy people are probably wondering why I am blathering about hoofed african mammals instead of my usual cheery chat about tumours, but fret not. All shall be revealed.

A zebra is, as Wikipedia (and, I believe, Scrubs) so nobly tells us, the medical term for a surprising diagnosis, which rather begs the question of what we should call those stripey ponies running around in nature documentaries. It comes from the aphorism that when you hear hoofbeats behind you, you don't expect to see a zebra. This is a wise observation which is totally inapplicable to anyone who lives in the African plains, but as it was coined by an American doctor in the 1940s, this oversight may perhaps be forgiven. The point is that when a doctor is presented with a set of symptoms which may be caused by a common illness or an uncommon one, the logical assumption should be that the patient most likely has the more common illness - even though there may be a temptation to go with the more dramatic diagnosis.

I have noticed this idea of being a medical zebra popping up in a few blogs by other people with rare pituitary tumours, particularly in those with Cushing's disease - possibly this is related to the fact that one of the symptoms of Cushing's is the development of dramatic stretchmarks of a stripey and thus zebra-like nature. A few further examples - here, here and here.

A group of Cushing's sufferers at a recent conference.

I guess I count as a medical zebra myself; my symptoms of hyperthyroidism such as tachycardia, hair loss and frenzied blogging** would normally be considered as indicating Graves disease or something - and even when the more common thyroid malfunctions were ruled out, it was considered more likely that I had a condition called Resistance to Thyroid Hormone than thyrotropinoma. But no, my body had picked the most unusual way it could think of to break down, and I was neither a horse, nor a pony, deer, mule, donkey, nor any one of a number of hoofed creatures which are more common in the UK than zebras.

Neighbours of Lord Rothschild may not only have expected to see zebras when they heard the sound of hoofbeats, but also had to leap out of their way.

Medical zebras are a tricky subject, on the whole, and there's obviously a balance to be struck. While it can be frustrating for those of us with unusual conditions to think that we waited a long time for a diagnosis, it would be far worse if doctors went around ignoring common diagnoses in favour of the weird ones. Equally though, doctors should be aware that just because something's weird and unusual doesn't mean it's not sitting in front of you - as anyone who's come face-to-face with Boris Johnson could tell you.

It's enough to make you wonder how rare your condition would have to be before you were considered a medical okapi...

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*Unless someone had painted a horse to look like a zebra. Which would be a pretty weird thing to do.

**Hint: one of these is not actually a symptom.

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.

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!

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

Ding Ding: Round Two

I had been holding off posting for a few days, not out of laziness,* but because I was waiting to find out the outcome of my hospital appointment last Thursday. And then it took me absolutely ages to type this up because, let's face it, it's dull and complex. For the last couple of months I have been largely in the dark as to the results of my blood tests and recent MRi scan, so I really had no idea what to expect.

What I did not expect was to find the television in the endocrine waiting room playing Cash In The Attic. This television has, to my knowledge, never previously displayed anything other than The Jeremy Kyle Show. I always assumed that the hospital trust had performed some kind of patient survey and determined that diabetics and people with pituitary tumours prefer their televisual experiences to involve shouting, paternity testing, and the phrase "Madam, you disgust me".

I digress.

First up, as per usual, I was weighed (lost: one kilogram since February, almost certainly due to forswearing chocolate over Lent rather than the TSH-oma) and had my blood pressure taken (standing blood pressure: 118/76 - score! It would appear that switching my heart medication back to propranolol has made a difference). Then I had a quick review with one of the endocrinologists about how my symptoms have changed since being on lanreotide injections (not much).

Then the doctor ran away and eventually returned with two other endocrinologists in tow, who all stood at the other end of the room and surveyed me like something interesting they'd found in a jar at the Hunterian Museum.**

They have been somewhat confused by the fact that my levels of various thyroid hormones have remained roughly the same since being on the lanreotide injections, despite the fact that you would expect them to drop.

Additionally, the MRI scan of my pituitary was a little tricky to interpret. Essentially, the pituitary gland is like a tiny upside-down lollipop just below your brain. The stick of the lollipop is called the infundibular stalk*** and it connects the pituitary gland to the hypothalamus. My original MRI pituitary scan showed a great big gobstopper lollipop with a dark patch of necrosis in the middle, where the tissue inside the tumour had died off a bit. To picture this, first imagine a lollipop with a liquid centre, and then imagine something a lot more disgusting. But my latest scan shows a rather more normal-sized lollipop which hasn't grown since my surgery (hurrah!). However, the stick of the lollipop is slightly bending to one side.

Given that there's limited space inside your head for these things to grow, a wonky pituitary stalk can indicate that the bulk of a tumour is pushing the rest of the pituitary gland to one side. So the area of pituitary gland on the left-hand side is now under suspicion. I have to say, looking at the scans was kind of awesome, because it's not like when people show you the ultrasound of their baby and you have to pretend you totally see what they're talking about. I actually could see what they were talking about.

But the problem with MRIs is that they can show you what's there, but not what it's doing. So although it looks like there's probably a bit of tumour left on one side of my pituitary gland and that may well be what's still causing my symptoms, it's still possible that it's not that bit at all. And it would be rubbish to have surgery, take that bit out and then discover it was actually healthy pituitary tissue.

So my doctors want me to have two combined PET/CT scans. PET (Positron Emission Tomography) scans are super colourful and pretty, but that's not the only reason they want me to have one (I hope).

PET scan of the brain

 Basically, PET scans can show those areas of the pituitary that are most metabolically active. Inactive areas are dark. So the idea is that I will have one of these scans while not on any kind of medication, which should show the whole pituitary lit up like a christmas tree, and then have another scan after three months on a higher dose of lanreotide, which should suppress the hormone production in the TSHoma. Any areas that go from being bright blue on the first scan to being dark in the second scan are probably tumour. Then, if the tumour is in an easily-accessible area of my head, I might have surgery again. If it's somewhere tricky to reach, I'll probably just continue to have regular injections of lanreotide indefinitely.

So I'm not having any more lanreotide injections for six weeks, so that it's definitely completely out of my system. And in the meantime they want to have me come in overnight one for some research tests on my metabolism and things. Essentially the hospital are collecting patients with pituitary tumours that produce TSH and studying us, so I am now officially a guinea pig.

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*How could you ever think such a thing?

**I'm joking, they were very nice.

***Or just the pituitary stalk, but you don't get to use a word like "infundibular" every day.

Characteristics of Thyrotropinoma

I found an article on a website which lists the characteristics of thyrotropinoma (a.k.a TSH-oma, a.k.a the kind of pituitary tumour I have, which secretes thyroid-stimulating hormone) as follows:

"Thyrotropin secretion by thyrotropinomas is characterized by increased pulse frequency, delayed diurnal rhythm, enhanced basal secretion, spikiness, and disorderliness."

I deeply resent this. In fact, I am a very orderly person.

Famous People with Cushing's Disease?

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

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

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

But where are all the famous Cushing's sufferers?

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

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

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

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

Come on cushies, come out from the woodwork!

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

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