I am posting this in both the Addison’s forum and the Hugh’s Syndrome forum.
I am just wondering if anyone out there is in the same situation. My husband was diagnosed with Antiphospholipid syndrome 3 years ago after a DVT with no apparent reason. It took 6 months to get the diagnosis due to having to wait for the second blood test to confirm it.
Last year he had surgery and 2 weeks later ended up in Hospital in an Addison’s Crisis. The Rheumatologist and the Endocrinologist can’t agree on why, or how, but they both agree that he has had an adrenal haemorrhage and now has not adrenal function.
The general consensus is that he should have died at the time so we should just be grateful he is alive. I am now wondering if anyone else out there has got Hugh’s AND Addison’s. We have been told it is “very rare” and would like to know HOW rare.
If any of you are on other Addison’s or Hugh’s forums, please post this there as well so I can get an idea if there is anyone, and if so, how many.
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Tiggercat
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I have APS and I suffer from adrenal insufficiency due to the steroids I am on. I have occasions when I have Addison's symptoms when I need additional adrenaline, which my body can't provide. I suppose this is very similar, I hate to think what would happen if I needed a sustained amount of adrenaline and didn't have access to my steroids.
I'd imagine it's quite rare. My family have a strong history of autoimmune disease and maternal grandmother had Addisons, I have APS and Sjogrens, maternal aunt RA etc but none of us seem to have two biggies together. (My Sjogrens is mild).
I have both APS and adrenal insufficiency, but not Addison's. Have wondered myself about the link and how many might have both. Even though I don't have Addison's, having the adrenal insufficiency is connected.
I have other immune problems so feel all this is, indeed, connected somehow.
I had adrenal insufficiency (established by private blood tests of things like cortisol, DHEA levels and daily patterns in these) and that was long before I was diagnosed with SS/APS. Certainly long before I took steroids.
Steroids suppress adrenal output I think, don't they? That's why we have to come off them so slowly, to give our adrenals time to kick back in and start producing the required level of hormones?
So clearly there's a link between steroids and adrenal function. But quite how that ties in with APS, I dunno.
Thanks for the answers so far, it seems many of you have got Adrenal Insufficiency due to Steroids, or other autoimmune problems, but not directly caused the way my husband's was.
My husband was not on steroids, and apart from the APS, of which he had no symptoms other than DVT, and positive blood tests over a 6 month period he was fit and healthy.
This definitely occurred due to a very swift event which they have put down as "Probable CAPS" (not positive as no biopsy, and thankfully no autopsy, was performed) being occlusion of small vessels of various organs and then a heamorrhage. He also appears to have some (not severe) kidney damage as well.
It's thought that it occurred due to cessation of warfarin 5 days before surgery, then the surgery it'self while receiving a Hepron infusion.
He was not "bridged" when they stopped the warfarin, but just left until they gave him the Hepron infusion the night before surgery, at which point is INR was 1.2 from memory, they then stopped the hepron, put him back on warfarin 1 day post surgery, then sent him home with no real follow up. 2 weeks later, after dragging him to so many Dr's it wasn't funny, they finally admitted him to CCU as he was dying and they didn't know why.
My goal is to find out how often this happens. I want to find out because we are trying to claim insurance as it was never considered or mentioned that this could happen. We want to rule out the cause being from an "underlying condition" where this sort of event (CAPS causing Addison's) is common and should have been known about. The insurance we are trying to claim on states it must be a "rare and unexpected event" so I am trying to find out just how rare it is. That way we can use their own wording against them.
Thanks Mary. I have seen the basic report, but have just read the full report.
It all sounds very familiar but with one major difference. It wasn't know at the time that this boy had APS. He was diagnosed after he presented with the Adrenal Insufficiency.
My husband was being actively treated for Primary APS. And that is what we are finding. All those that have had this issue (which is very few) didn't have a diagnosis of APS before hand, but it was found to be a contributing factor (if not the primary cause of the adrenal infarction) after AI was diagnosed.
It is looking more likely his Adrenal Insufficiency could have been prevented if he had his INR monitored before surgery and had been "Bridged" to try and reduce the risk.
If you have time that would be great, but don't do it if you have other things to do. You seem to be able to access things better than I can. And there isn't a lot of information in NZ about these things.
No APsnotFAB you are not missing the point at all. That is exactly the point. We didn't now anything about bridging, and were never told about it. It is only with research after the event, that we have found out about it. Had we known, we would have asked the questions at the time, but they still won't admit he should have been bridged.
However, his new rhumatologist (gone to since the event) is now writing to his GP to say that even if he needs dental work (extraction etc) he is to be bridged with LMWH. Any time he is taken off warfarin it is to become standard practice.
They are trying to convince us that because he had only had 1 DVT pre surgery, and it was 3 years ago, it was quite safe, and not necessary to bridge. It would appear in hind site, they were very wrong.
I have Primary APS, secondary Lupus and Addisons. Lupus diagnosed 1990, APS diagnosed 1993, long story short (usual misdiagnosis leading to near death) Addisons was diagnosed back in 2006 following an adrenal haemorrage and general consensus is that APS was "almost certainly "responsible rather than long term steroids (have been off and on prednisilone for last 23yrs) but we'll never really know as 'care' received at the time of crisis was non existent as local hospital tried to send me home with paracetamol!
I am wondering if you know of anyone else that has this issue. It seems to be quite rare, especially when APS has already been diagnosed and been treated. I am assuming you found out about and Addison's when you went into crisis.
Was it a gradual thing, or did you just drop down hill at high speed until you were in full crisis?
The very few I have found so far have had a gradual reduction in Adrenal output, and still have some minimal output. Its finding people that have had the adrenal's taken out over a short period (a couple of days) that I am find hard to locate. Seems most don't survive.
If it happened suddenly, did you have other organs affected as well?
Hope you don't mind all the questions.
Thankfully my husband doesn't have Lupus as well. APS and Addison's is enough.
No I have to admit I don't and when I started seeing Karen Breen @ Guys she said I was one of only 2 patients she knew who had both APS & Addissons. Mine happened fairly suddenly, I started getting horrendous pain (similar to when I had PE) on the Friday....but as we all do I tried to pretend I could manage it and by the Sunday evening had to go to A&E as pain was so great I just wanted to be put out of my misery. Unfortunately the local 'hospital' told me it couldn't possibly be my Lupus or APS as the blood tests were coming back normal (all this from a locum Dr who'd never seen me before despite the fact that I have been living with my these conditions for over 20 yrs). I had every 'oscopy' imaginable - was treated as a malingerer (left lying in my own urine for 12hrs before my friend, a nurse, came to visit and changed my sheets) and the shadow on the scans (my adrenals haemorraging) was dismissed as "slight fluid on lung" so they said all I needed was paracetamol and some physiotherapy - even though I was unable to sit up let alone stand and in so much pain I was mostly delerious (according to my visitors - I don't remember much) so over the course of a week my body started to shut down.....first kidneys then liver...it wasn't until I went buttercup yellow and lost consciousness that they believed me and sent me via ambulance to John radcliffe in Oxford. They figured out what was wrong within 12 hrs and once they'd started treatment and given me 6 bags of blood I was back home within a month. It would appear that, thankfully, my liver & kidneys have recovered but I still have issues with fatigue which are bounced back and forth between APS & Addisons specialists........but I am alive and mostly kicking
Just wondering if you have problems with your INR doing crazy things. Hubby's rhumy told him to try and get to 3 (previously 2.5). His INR was 2.2 at the time, so he went from 8mg to 9mg daily. A week later, his INR is now 1.4 and he hasn't done anything different so we aren't sure why it has dropped.
The good news is that his Dr decided to phone the rhumatologist to get advise (he was just going to leave it till next week) and was told that it was too low, and he needed LWMH by injection till next week, increase warfarin, and get tested on Tuesday.
He was putting the lousy feeling down to Addison's because sometimes the symptoms seem to be similar. Now we are not sure which symptoms are for which disease.
Yep my INR seems to have a mind of it's own and is upset by the slightest thing e.g antibiotics - with everyone else antibiotics reduce their INR, with me they send it sky high. I believe that the APS is causing the majority of my symptoms (fatigue, brain fog, all over pain) as whenever the Endocrine specialists run the two yearly adrenal tests they always assure me that, according to blood results, I am on the correct dose of replacement steroids. I can definitely tell when my INR is low as all symptoms worsen (found keeping a symptom diary invaluable). But it is really difficult as we (Hughesies with Addissons) are apparently as rare as hens teeth so I remain to be convinced that any medical professionals really know how the two conditions interact.....as per usual we just 'keep calm and carry on'!!! Perhaps we need to start a new forum
Having read some of the reports I have been directed to, and other information I have found, I am wondering if the Insurance company we are trying to claim against might find them as well and say "well, it would have happened eventually, so it's due to the underlying condition and we aren't paying", even though it was an acute onset, not progressive tiring of the adrenals.
It seems that the stress of APS, and the pressure placed on the adrenals trying to keep the body functioning, can cause Adrenal Fatigue all on it's own.
Now I am wondering how many APS sufferers out there actually get their cortisol levels checked a couple of times a year to make sure AI isn't causing a raft of it's own problems.
It's amazing what pains my husband had in the two weeks between his adrenals dying and when he went into crisis, which was put down to everything from the stomach being upset because of the Warfarin and other meds following surgery, an infection, diarrahea due to surgery, an unexplained rash that the Dr looking at it said "rashes aren't my area" and ignored it, constant high temperatures (so lets throw antibiotics at it, "just in case"). He couldn't walk 50mtrs without feeling like he needed to sleep all day, but "that was due to surgery".
In New Zealand we also have different specialists who deal with different things but apparently we have one that will be looking after all areas for him. Problem is, he is looking at the medication and saying, "well the book says you only need X, so get down to that level". Problem is, that level is way too low for him. He gets brain fog, and bad gastric upset if his hydro is too low. I wonder if the INR being only around 2.2 (he's been told to get it to 3) is part of the cause of the brain fog.
Thanks for all your help. We obviously have a long way to go before we have any understanding about these problems and their interactions with each other.
Hi Again! Just catching up with the thread.........I had same problem...after adrenal haemorrage they took my INR down to 2.5 (this was back in 2006 before I started going to Guys) and from then until June 2012 my APS symptoms increased exponentially - total exhaustion, memory loss, dizziness, brain fog and pain everywhere. I did my research and pleaded with rheumie to have INR increased but they refused citing the adrenal haemorrage as reason. It was in Jan 2012 that I finally had enough and asked to be referred to Guys and by June Dr Breen had reassured me that the pros of putting my INR up far outweighed the cons. We are still increasing it gradually I am up to 3.1 now and heading for 3.5 to 4 and am definitely noticing an improvement in my APS symptoms.....so if you can find a sympathetic APS specialist I would push for increase in INR!
Thanks for that, unfortunately they are all in Melbourne Australia, I am in New Zealand. It's a very expensive flight to there, and we have to pay their high medical costs.
Auckland is also almost as expensive to fly to as Melbourne. We are in Wellington, the other end of the Island.
We may just stick with the guys we have at the moment, and see how much my husband's Endo knows about APS.
We have a No Fault Government Accident Compensation system in New Zealand. We all contribute and if you have an accident, the government covers you.
This is who we were claiming against for the Addison's. The really good news is, the Dr's on the panel that decide your fate were so confused with everything that happened with my husband that they said "screw this, just pay them". So we have had the claim accepted, and going forward we are now able to get assistance with things like transport to work, sick leave (with medical certificate) if he is not able to work due to the Addison's, possibly even assistance with housework etc because my load had increased due to his not being able to do as much.
On the down side, he seems to be now suffering more aches and pains which we don't know if they are His Addison's or his APS. His warfarin levels have been increased, but he is finding it a lot harder to stabalise. He is now on 12-13mg/day. and on 25mg hydrocortisone per day. He takes meds 6 times a day.
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