I stopped posting here for a few reasons, but this might be worth logging in its own thread. I will be having a phaeochromocytoma removed soon. These adrenal tumours are usually diagnosed through excessive catecholamines in 24 urine collection, or through excessive metabolites of those catecholamines in blood plasma. My results for these tests were consistently only slightly elevated, and the lesion is only 13mm, so the phaeochromocytoma went undiagnosed until I experienced some mild hypertensive episodes which prompted an MIBG scan. It could well be the reason I had a heart attack last year, which was the precursor to developing ME/CFS. Anyway, in common with everyone else here, I have been secretly hoping that some clearly biophysical issue will suddenly be identified and cured, restoring good health. So, of course, I’m full of dangerous phaeochromocytomal optimism, which will probably be dashed, but I will report back either way after the operation.
The causes for optimism are looking a tiny bit stronger. I had the adrenalectomy last Monday and got myself discharged on Wednesday. I just had a call from the endocrine team to say that the cortisol level in bloods taken after the operation was 36 nmol/l, and I needed to start on daily 40mg hydrocortisone ASAP to make up for a serious adrenal insufficiency. They called back two hours later to check that the prescription had been collected, and to advise (rather vaguely) that I increased the dose if I felt at all peaky. Nothing good would come of asking them why they’d waited six days to tell me, the NHS moves in mysterious ways. But I’m intrigued now to note that my previous cortisol levels were rather low (200 ish), and that fatigue and weakness are indicative of Addison’s disease (which is to say, a primary adrenal insufficiency). So it might just be that subclinical hypocortisolism (in concert, somehow, with catecholamine excess) was playing a part in either triggering my particular symptoms, or in triggering the mysterious neuro-immuno-whatever mode we call ME. Anyway, I will continue to guzzle the corticosteroids and will report back if I suddenly start walking unaided and charging up and down stairs.
I'm afraid I don't know, she lives in France and we only talk every month or two. She will have gone to the very best clinic and had the best possible treatment. I'll ask her for more details the next time I speak to her.
Just spoke to her, as far as I can make out she had genetic testing and it took 3 months for the results to arrive. Nothing to worry about and not familial she says. She had a tumour on her adrenal gland. We've just heard that my brother has a tumour on his kidney, results of tests awaited. Seeing as a major symptom when my ME began 9 years ago was pains in the kidney area (which managed to be dull, stabbing and debilitating at the same time), I do wonder. They found nothing at the time, of course.
Quick update. Firstly, apologies, but it turns out that there is little value in this ongoing n=1 anecdote for anyone else, because adrenal insufficiency following unilateral adrenalectomy for phaeochromocytoma seems to be incredibly rare. After lots of trawling I found only one relevant case study, which noted that only five such cases had ever been recorded. Secondly, and annoyingly, the impact of hydrocortisone therapy seems to be to exacerbate ME symptoms, especially weakness, while adding some exciting new ones including head pain. I initially thought this was because the 40mg dose was so high (NICE guidelines are 20-30mg) but several days after taking it down to 20mg (with clinical permission) I’m not really seeing any improvement and have been near-bedbound all week. Thirdly, it turns out that there were no sensible endocrinological grounds for suspecting subclinical adrenal insufficiency prior to the adrenalectomy. The endocrinologists are expressing confidence that the contralateral adrenal gland will eventually kick in, so I can come off the hydrocortisone. It’s not clear to me what that confidence is based on, because the literature only describes adrenal insufficiency after unilateral adrenalectomy for cortisol-emitting lesions (ie, Cushings), in which cases it makes absolute sense for the other gland to have suspended cortisol production. Anyway, in those circumstances, the timescale for adrenal recovery is around six to twelve months. But these are not my circumstances. It might just as easily turn out to be a lifelong condition, as with most primary adrenal insufficiencies. Or, despite the Royal Free’s assumption that it’s not even worth testing cortisol again for a couple of months, it could clear up faster. Whichever, I’m pretty sure that the current level of symptom exacerbation is going to tip the balance against holding down the part-time home-working role that I’ve managed for the last year (and have been bunking off for five weeks now), so any duration more than a couple of months has perturbing consequences. Overall, what I was hoping would be a good news story, an n=1 that would cheer proponents of small-adrenal or hydrocortisone-panacea ME theories, looks more like a cautionary tale against pursuing additional diagnoses and surgical interventions (not that a phaeochromocytoma is a particularly helpful kidney accessory to be lugging around).
Sorry to hear that, @Shadrach Loom. It must be a real struggle. This is another N=1 anecdote, and I really hesitated about posting it. But here goes, in case it's useful. I also have problems with steroids, even the small topical doses in my asthma inhaler and nasal sprays. The inhaler leaves me struggling to do anything much, and the nasal spray leaves me bedbound and barely able to move. This isn't at all typical for ME, and I puzzled about it for decades. Long story short, I might have hypokalaemic periodic paralysis. I still haven't even been referred for diagnosis yet, so I can't say anything firmer than that. But my adverse responses to glucose, rest after exercise, and steroids, plus the unexplained permanent upper leg weakness, are suggestive. I dismissed the idea in the past because I've only ever had a few episodes severe enough to be described as paralysis; it's mostly varying degrees of weakness, and when weakness is only at a low level, it's hard to distinguish from fatigue. But that pattern can be how HPP manifests. It was the steroid thing that finally made me decide to try and get diagnosed. There's more than one type of periodic paralysis, all of the primary types are rare, and my hesitation is because it's very unlikely to be behind your response. But then again, if no-one had mentioned it to me, I'd still be in the dark! As it is, even without a diagnosis I've been able to gain a lot of function by avoiding the triggers...though that's obviously not something you could do right now. I hope you pick up soon, anyway. I know from experience that it's particularly hideous when you're struggling to keep up with work, but not working isn't really an option.
That’s really interesting @Kitty - would you mind updating if you get diagnosed or find anything else out about how steroids are affecting you? The endocrinologists seem very keen on measuring potassium and sodium - I was hypokalaemic just after the op - so hopefully they’ll be sighted on this. But as you say, I don’t currently have the option to avoid steroids.
Unless there is some urgent medical reason for reducing hydrocortisone by 50% all in one go, I strongly suspect that you would cope better by reducing in much smaller amounts - say 5mg at a time - would be less of a shock to the system. Do you split your dose? You might feel better if you do. https://www.pituitary.org.uk/information/treating-a-pituitary-condition/hydrocortisone/ I hope you have a steroid warning card and/or a medical alert bracelet that you keep with you all the time. You might even need to keep a steroid injection available in case of emergencies. Taking high doses of hydrocortisone could shut down your remaining adrenal gland leaving you reliant on exogenous steroids permanently. Hydrocortisone can interact with a lot of other drugs : https://www.healthline.com/health/drugs/hydrocortisone-oral-tablet Drugs.com is usually a very good source of drug info : https://www.drugs.com/cdi/hydrocortisone-tablets.html
I was only on the 40mg dose for a few days before halving it, and an endocrinologist okayed the decision. I don’t think my system had had time to acclimatise to anything! Yes, I split 10/5/5. Drug interactions are a possible concern and yes, the hydrocortisone might exacerbate the adrenal deficiency but there isn’t really any other option when I am not producing cortisol. I’ve taken prednisone before, for Bell’s Palsy, and it’s horrid. I have been promised a special pack with bracelets and cards and so on, but as I never leave the house except to visit clinical settings, and am always accompanied by my wife, the kit will be fairly pointless. This is all good advice, and thank you, but I’ve had plenty of time now to get genned up on Addison’s, which is far less mysterious than ME, even if it does take some managing. And as the most high profile sufferers with the condition are John F Kennedy and Osama Bin Laden, the greatest clinical risk turns out to be assassination.
Will do. I'm not hopeful, though! They can reduce potassium levels or shift the electrolyte balance, which will be the reason you're being monitored. In HypoPP, glucose intake has the same effect, as does salt intake. Other things that cause unhelpful shifts are alcohol, getting very cold, or resting after exercise; it's an easy-to-recognise pattern, but only if you know about it in the first place. Very small shifts can depolarise muscles, and whilst some patients do experience dangerously low K levels, others rarely test below the normal range. Their symptoms aren't necessarily caused by globally low potassium, it's more that it's in the wrong place.
Update after a synacthen test is that my cortisol production is now satisfactory and hydrocortisone has been entirely withdrawn. ME symptoms aren’t any better, but here’s hoping. So apart from CT and gallium PETs every so often to see if anything has grown back, I’m not going to have to see endocrinologists any more. That’s a mixed blessing. There’s been some very interesting stuff posted recently here lately about the interaction of hormones with mitochondria, and it would be nice to find out more about rogue HPA behaviour. The HPA, I only recently learned, is implicated in benign prostate hyperplasia from excessive dihydrocortisone production, so I have been wondering whether some banal (if age-inappropriate) LUTS problems might fit into an n=1 grand unifying theory of ailments: a hypothalmic hypothesis. But although I’m now on three-monthly PSA surveillance, urologists are practical folk with no interest in aetiology, so there’s little chance of them being interested. TL/DR to summarise the thread: if you have ME with two adrenal glands you’ll probably still have it when reduced to one, and if anyone spouting nonsense about “adrenal fatigue” offers you off-label hydrocortisone, be very wary.
I've just started with a new doctor, who seemed interested about ME. When I told her the above, she asked if I'd like my kidneys given a thorough looking at. Not quite sure what she means by that, but I've an appointment in the next few weeks for whatever examinations she has in mind. Also a general check-up (first one in 3 years), she was quite accepting about me not doing an exercise bike test (whacking my diagnosis on Charite headed paper on the desk seemed to get me taken seriously, although to be fair she was seeming to take me seriously anyway). Meanwhile my brother is having his kidney removed next Tuesday.
