Addisonians

We met Prof Trainer on Friday. He was a very nicely spoken Scot, who explained everything patiently and clearly, so even I could understand it (I think... though I was scribbling fast so some of the following may not be 100% accurate). We have learned more things:

1. Even though it's tiny, the adrenal gland takes 11 - 12% of the body's blood supply (rich field for growing tumours, so we need to get it out!)
2. There are two parts to the adrenal gland; the adrenal cortex (produces steroids) and the adrenal medulla (producing adrenalin).
3. The Americans call adrenalin 'epinephrine' which is why epipens were so named. Aha!
4. We don't worry about the adrenalin as sympathetic nerves make adrenalin too; you can get a normal adrenalin response without an adrenal gland (good news for the wasp stings).
5. The steroids (aldosterone and cortisol) are more important. Cortisol is the immune / glucose response and is very important if you're unwell (eg. flu or accidents). Aldosterone regulates salts and blood pressure. Both are essential for life.
6. Having no adrenal gland is known as Addison's disease and people who suffer are called Addisonians.
7. Addisonians have to take tablets to replace hormones 3 times a day. Most first thing when they wake, some at midday and then a few around 5pm - 6pm.
8. The quantity used to be 30mg, but now it's 15 - 20mg (with top ups if you are unwell, injured or going to the dentist). The doses have been reduced to avoid the usual side affects of taking steroids, such as weight gain, risk of diabetes, weakened bones etc.
9. The ambition is to imitate physiology, (ie to replace, not to give extra). 
10. There is an owner's manual to Addison's disease (people living with no adrenal gland). We have downloaded this, although Quent is about as good as any man at reading the manual.
11. The user manual states 'Addisonians can bring up a family on their own, hold down a demanding job, run marathons or even become the President of the United States.' Multi-taskers, it seems.
12. The main side effect of being an Addisonian is fatigue / lack of energy. Hmm. We'll see.
13. Quent will be taught how to inject himself if he has an upset stomach and cannot absorb pills. Prof Trainer did suggest self-injecting or spouse-injecting but we've narrowed that down to self injecting, with spouse-blogging!
14. If the levels of hormones aren't right, you may have an Addisonian crisis,with life-threatening symptoms, totally unrelated to your minicab failing to turn up. 
15. The lack of adrenal gland doesn't preclude future IL2 if it's needed, but it would make it trickier.
16. Even very intelligent, softly spoken Professors can wear very loud shirts.

It all sounds relatively straightforward, except the bit when Prof T said "it means you have to be more organised in life". Hmm. Never our strong point.

Last minute appointment

We are seeing Prof Trainer, internationally-renowned (Scottish) endocrinologist, tomorrow, 12 noon, at The Christie. He sounds lovely.

Adrenalin rush

Things we learned yesterday:

1. The adrenal gland is really important. The 'nerve centre' of the body. Linked to the main aorta, the kidney, liver and diaphram - and welded to the main vein.
2. The body tucks away really important organs / glands behind ribs etc. So it's hard to get to.
3. It's makes cortisol (steroids - essential to life), adrenalin (essential to life, says Quent), mineralocorticoids (great word for Scrabble) and a little bit of testosterone (yes, another of life's essentials!)
4. They won't take out part of it - so Quent will be on replacement therapy for the rest of his life.
5. The operation will be done keyhole by a pretty, young, blond surgeon called Miss Warburton.
6. There's a really lovely parade of shops near Withington Hospital, but the operation will be done at Wythenshawe, which apparently has no nice shops nearbly.
7. If there are complications, the operation will be done as open surgery and the scar will be twice as big as the one made to remove Quent's left kidney (which is pretty huge!)
8. Quent will be in hospital around a week.
9. Many of our questions will be answered by the endocrinologist (another good Scrabble word), rather than the surgeon. So don't ask anything about what tablets Quent will be on, because we on't know yet.

As someone said recently, the idea of Quent without adrenalin is tricky to get your head around...

Appointment with the Surgeon

Apologies for the lack of news. We have been waiting for an appointment to see the surgeon, Mr Romani. He's now decided that it's best for us to see a different Urologist, Miss Warburton. We're not sure why, but presumably they specialise in different bits.

Unfortunately, Miss Warburton is on holiday this week and then it's Easter, so our appointment is on 22nd April. She's indicated, via her secretary, that the operation will be early to mid May. We're hoping we can bring that forward - it's already 4 weeks since Quent's CT scan, when we found out the cancer was back. We haven't seen anyone since that day, when the Prof told us, and we're keen to get things moving. The tumour is close to Quent's remaining kidney and presumably the bigger it gets, the trickier the operation.

Meanwhile, the doctors at the Royal Brompton have decided to postpone the desensitisation programme. They will review the situation in 6 months, but in the meantime it is vital that Quentin carries two epipens on him at all times. So he now has a black leather 'bum bag' and is being pretty disciplined about wearing it.

More news as we get it.