Acromegaly, the Shrek Condition – My story. [Part 2: Initial Treatment]

In my previous post, I described how I was diagnosed, in my early 30s, with a Pituitary Tumour causing a one-in-a-million condition called Acromegaly. It is referred to as the Shrek Disease on the Internet after French wrestler Maurice Tillet (upon whom Shrek was based),  although I call it a Condition as you can’t “catch” Acromegaly. In my case it was identified, by chance, by a student nurse in an ER ward; and then confirmed by simple blood test which showed that my Growth Hormone levels were over 30 times higher than the safe maximum for a healthy adult male. That night, I was given four years to live unless I was treated urgently.

In this post I describe what happened next.

By way of reference, the picture on the right shows what I looked like a few months later. Compare that to the pictures in my previous post, Me in Late 2009 showing full Acromegaly Features
which were taken about 7 and 3 years earlier respectively. (They beautiful girl, next to me, I will come to later). I was about 135kg by then, had difficulties getting clothes and shoes to fit. I couldn’t sleep at night because my neck was so thick, causing sleep apnoea and snoring (I kept suffocating at night).

I focused on the need for surgery. The neurosurgeon at St. Vincent’s who diagnosed me suggested immediate surgery, but noticed I was wearing Queensland colours in honour of State of Origin. As it turned out, a well-respected colleague of his, Dr. A.P. (Paul) Poulgrain, was based out of the Gold Coast in Queensland, which is where I lived at the time. So I agreed to an inter-state referral and was discharged that day. I broke the news to my family that night by phone, who were upset and felt helpless, but still being in Europe at the time, couldn’t do that much about it except offer words of support.

Because Australia’s health system is so convoluted, I had to see my GP in Queensland to get his referral to see Dr Poulgrain. I found Dr Poulgrain to be amazing; we got on extremely well from the start, and he took the time to describe to me what was going on. Dr Poulgrain, as is normal, ordered his own MRI scans. He also referred me to an Endocrinologist he works with, Dr. Spero Negas. I didn’t really understand why, because I was so focused on getting rid of this tumour. Because, getting rid of the tumour would solve the problem, right?

I went to see Dr. Negas, who immediately could see something was wrong. Just looking at my facial features, he suspected Acromegaly. Which, of course, was confirmed by the blood tests he ordered. In an effort to get the ball rolling on some surgery, I just humoured him and did what he wanted. He put on a painful injection which had to go into my arse cheek, 30mg Somatuline Autogel, every 28 days; but over time I started to understand that it was the Acromegaly that needed treatment.

My biggest mistake was thinking that the Pituitary Tumour was the problem that needed resolving. It wasn’t. It was actually the Acromegaly, the substantially increased levels of Growth Hormone in my blood, that was going to kill me. As I found out later, it was those that would have killed me via colonic cancer, liver failure and heart failure. Unfortunately i focused on the tumour being the problem, and not the Acromegaly. So how I prioritised my treatment and research was ineffective. The priority was to get the tumour out. And this seemed to align with the quick google search I did on the topic, which pointed to a document written for the American Brain Tumour Association (link dead; but i found it here), that only really discussed the surgical options in medical speak.

I couldn’t find any other information in Australia about this mysterious disease called Agromegaly. I now know there are other sources of help, but I didn’t find them out for another 18 months (which will be in the next post).

Dr Poulgrain also took some advice from Dr Negas, which was basically agreement the tumour needed to be “debunked” in parallel to further endocronogical treatment. Basically, cut it out. How do you do that? Well there’s actually two methods:

  • The “sunroof” method; where they open the skull like a car sunroof, and go in and try and cut out the tumour from the Pituitary, through the brain. This is quite a dangerous approach because of the increased risk of damage. And you have to be kept awake during the surgery, just so they can make sure they don’t damage something vital.
  • The “nasal” method; where they go through the nose. Done under a general anaesthetic, they use Cocaine to open up my nasal cavities, drill through the back of the “softer bone” nose at the back of the nose; and then go through the nose with a camera and a surgical scalpel.  I must admit, I was sold on the free Coke bit, but also it was all done under a general and was least risky. Why could it be done? Because, like everything else, my face and nose was oversized, so was big enough to get the camera/knife in each nostril, so therefore enabled the operation to take place.

I might be shy and quiet (well, actually, you can’t survive in Australia being shy. So over time I had gradually learnt to come out of my shell and to try and laugh and joke around all of the time, otherwise they wouldn’t have let me become an Australian Citizen in 2009); but even Dr. Poulgrain thought that keeping me awake during surgery would be dangerous. Because he knew I would have constantly taken the piss. One of the tests they do is to show you coloured cards and you’re meant to say what colour the cards are. He knew I would have gone “Red … Yellow … Green … hahaha only joking its Blue really”; so he also thought that the nasal method was better. Its also much safer, but thats less of an amusing story to tell.

So, time to visit another surgeon. This time, an Ear Nose and Throat surgeon who also worked closely with the other two, Dr. Alison Sprague. Again Alison was amazing, explained what was going to happen and reassured me that she worked with Paul all the time. She did her own scans and agreed that a “nasal” approach was the way to go. As I was seeing both surgeons as a private patient (thank feck I had Private Health Insurance), it didn’t take too much time. In fact, things went extremely  quickly, and apparently a lot quicker than it would have done under the public system.

Within a matter of weeks, I was back in hospital. Three surgeons and an anaesthetist all lined up for a Tuesday Morning procedure. I was given the general anaesthetic, and a few hours later, woke up back in bed plugged into various machines. The surgeons each came to see me to say that the surgery had been a success, and everyone was happy with how it went. I was kept in hospital for a week with various things plugged into each of my orificies (I’m sure hospitals do that just to give the nurses a laugh at the pain endured when they remove the tube from inside the man’s “vital area”, but at least the hospital served wine and beer with dinner, so it wasn’t so bad.

I was told to stay away from work and travelling (partiularly by plane) for a couple of months to recover from quite traumatic surgery. I met the very beautiful girl in the photo not long after. I used to run a coffee group on the Gold Coast, in a vain attempt to try and meet people and build a friendship circle on the Coast. The Goldie, as most Australians know, is very tourism-driven and is full of “temporary residents” working in the tourism sector, women who are after a sugar daddy  or backpackers looking for their own visa through marriage, so it is virtuality impossible to find anyone genuine there. After 4 years of trying I gave up – who’d would really want to date or eventually marry someone with brain cancer after all? And who looks fat and not like a model? Well the girl in the photo was very realistic, kind-hearted, down-to-earth, and had a medical background, so she knew the risks and our relationship blossomed. She also wasn’t offended by my Pommie accent, suprisingly.

Checking in with each of the surgeons afterwards, Dr Spague had no issues getting through my nose, and whilst she was there apparently she cleaned up the Rhinitis I didn’t know i had. I had wondered why, for the first time in about 20 years, I stopped having constant hayfever/sneezing symptoms and a running nose; turns out she fixed it whilst she was there. Dr Poulgrain was even happier about how it went – although he did keep me under for a couple of hours longer than planned. Apparently during surgery he saw some “interesting” looking cells on the cell wall of the Carotid Artery next to the Pituatary. He scraped those, biopsied them, and unfortunately those turned out to be tumour cells. Another growth-hormone secreting tumour had started to grow outside of the pituitary, but maybe we just got it in time. “Skippy”, my Aussie Pituatary Tumour as tagged on Farcebook, now had a new friend, “Boomie” (after Boomerang/Boonie – and both Skippy and Boomie had been discovered once I had come to Australia).

Over the next couple of years, it was a case of watch and monitor. My doctors again suggested I left the travelling/Consulting world and do a “normal” 9-5 job if I could, so I did.  I found a job in Brisbane which had no travel commitments. My growth hormone levels in the blood plummeted from around 840 down to 80 (so i was only 2-3 times higher than the safe level, rather than dangerously high). However, the hormones didn’t drop as much as they had wanted, and had stayed at around 80.

Dr Negas had increased the injections to the highest dose available on the PBS (120mg Somatuline Autogel); and then we tried a similar drug (Sandostatin) with similar results. The lastest MRI scans kept showing “residual disease”, i.e. some of the tumour was still there and, we suspected, was causing more growth hormone to be produced. I had lost a little weight, about 10kg, but the growth hormone levels remained at the same level from just after the surgery.

Overall though, life was looking up. We had survived the Brisbane Floods, and were having a normal relationship. In fact, the beautiful girl in the picture had agreed to my marriage proposal (well, eventually. It took her dog to “approve” the ring first by sniffing it, licking it and giving the “go on Mum, you want to marry him, he’s not that bad, and knows how to rub my belly properly” look). I had virtually weaned myself off the antidepressents, and was down to the one last tablet per day. I had lost a little weight, but was sleeping a lot better, wasn’t snoring and things were starting to go right in my life, for once.

In early 2011 one MRI showed some ‘undefined soft tissue” near to the area Dr Poulgrain had biopsied those additional tumour cells from. I suspected that there was more tumour there, and asked the surgical team to consider having another go at the remaining tumour, and looking at what this “soft tissue” was. Having run out of options on the medical front (the only remaining drug to be tried is not available on the PBS in Australia, and I couldn’t afford $10,000 per injection), we decided to go for another look.

I had lined up my client  to know I would be away for a few weeks and that the brain surgery wasn’t much to worry about. I was feeling pretty positive that we would get the remainder of this tumour and this would be the end of the Acromegaly. With the same surgical team, it was back to the same hospital, and we were feeling pretty positive.

Except, things went wrong during surgery. Very wrong. I very nearly died, and am extremely lucky to be alive. What happened? That’s what I’ll cover in the next post.