I've read from various sources that without treatment Wegener's granulomatosis is rapidly fatal : that most sufferers die within a year of diagnosis , e.g. ...
[quote="wikipedia.org/Wegener's_granulomatosis"] Before steroid treatment became available, mortality within one year was over 90%, with average survival being 5 months. [/quote] en.wikipedia.org/wiki/Wegen...
[quote="vasculitisfoundation.org"] GPA/Wegener’s treatment has come a long way. In 1958, patients with GPA/Wegener’s had an 82% mortality rate at one year, with an average patient survival of five months. [/quote] vasculitisfoundation.org/ed...
So am I right in saying that it would be astronomically unlikely , ( say less than 1 in a million ),
for someone confirmed* to have WG to survive 25 years without treatment ?
If possible can you include a link to an internet source in your reply.
Thanks
[ * rather than them actually having a look-alike condition with a better survival rate ,
Surely WG can affect different parts of the body. My husband has it mainly effecting his sinuses/airways/ears plus other symptoms but not in his kidneys, lungs or liver. Failure of those without treatment would/could be fatal.
[quote="Nadine99"]…kidneys…Failure of those…would/could be fatal. [/quote]
two of my blood relatives died of kidney failure in their 60’s , but they had signs of vascular disease for many decades previously and did not receive any immune suppression. So if WG really is rapidly fatal it’s a non-starter as an explanation of my familial syndrome , ( despite an NHS consultant suggesting it as a possible diagnosis in my case ).
What is your point? You have found some verified information that gives you the answer to the question you've posted but you haven't explained why. Personally, I find this a bit negative and unhelpful as most people once diagnosed have treatments to avoid further problems and preserve their mortally for 25 years.
Well I'm glad it's clear to you Bob, here I have not a clue what Scot is on about especially with all those quotes & too technical stuff. Hey ho, best wishes to you anyway. Far beyond my comprehension!!
There have been lots of people who survived WG/GPA for more than 25 years and I certainly intend to! If I recall correctly, the wife of Paul Pegg (who ran V-UK before John took over the reins) lived for more than 30 years with the condition. Mortality rates have dropped dramatically with the improvements in diagnosis and treatments with cortico-steroids and some of the more recent immunosuppressive drugs making a huge impact. That said, early diagnosis and an efficient biological marker to aid detection would help enormously. Healthy wishes. p.s. Maybe someone shoudl edit the Wiki page as it's misleading at best.
[quote="Martin_Thomas"] There have been lots of people who survived WG/GPA for more than 25 years ... Mortality rates have dropped dramatically with the improvements in diagnosis and treatments with cortico-steroids and some of the more recent immunosuppressive drugs making a huge impact. ... Maybe someone shoudl edit the Wiki page as it's misleading at best. [/quote].
Both the quotes in my first post are mortality-rates without-treatment , ( i.e. without corticosteroids or other immunosuppressive drugs ).
One facet of my syndrome was treated with the corticosteroid prednisone for two months, (after an mere eight year unnecessary delay), it's miraculous stuff : it produced my longest-ever period of total remission from symptoms.
During subsequent severe relapses I have asked, even begged, for that treatment , but have been repeatedly refused :¬(
Your situation is clearly serious and complex and whilst I don't believe it should ever be necessary to pay privately to receive treatment we need, I also don't believe there are any private consultants 'out there' who are more 'able' than the best within the NHS, here or overseas when it comes to vasculitis. If I were you, I'd seek a consultation Dr David Jayne at Addenbrookes, who in my opinion, is probably the world's most authoritative clinician when it comes to understanding and treating vasculitis and lupus. The specialist clinic at Addenbrookes has a raft of consultants with a range of specialisms and a wealth of experience. If your existing GP/consultant won't refer you then you can contact the clinic directly and ask to be seen. cuh.org.uk/addenbrookes/ser... Healthy wishes.
Any other suggestions of Doctors are welcome , but best do that by Personal Message, ( xxxxxxxxxxxxxxxxxxxxxxxxxx ) , as naming specific doctors is this forum could get us in trouble.
I have WG/GPA and was not treated for 15yrs appropriately, until I developed severe posterior scleritis in left eye. I fortunately do not have major organ involvement but I dread to think that something awful could have occurred because of the total ignorance of the medical team treating me. Now I am being treated appropriately and have early signs of kidney problems...but I have to say to you as a member of the Nursing profession that this disease needs to be highlighted, even I as a sufferer did not know about it and just trusted the medics...they got it wrong but as I said fortunately I am now on immune-suppressants/steroids.
[quote=“SABG”] Now I am being treated appropriately and have early signs of kidney problems ...just trusted the medics...they got it wrong [/quote]
Having seen my familial syndrome play-out in my other blood-relatives I was able to predict my kidney involvement : Two years after mentioning to a NHS GP that , in the absence of any immune-suppression, I was due for kidney involvement, I presented with self-diagnosed proteinuria, only to be criticized by the NHS GP for taking up their time with this correctly predicted symptom because according to them the level of protein in my urine wasn't sufficiently high to warrant their attention.
IMO, particularly given the very high cost of dialysis, better to treat kidney involvement sooner rather than later.
[ However my affected blood-relatives weren’t offered dialysis when their kidneys failed (x_x) ].
I really hope you will be treated by a 'Vasculitis' team as soon as you can, because I had been left in an indefinite hole not knowing yet aware of consequences of WG, just told I had secondary WG !!! what was that ??? Now I am just thankful that this awful disease did not attack me in the way that it can do with so many other sufferers in early stages.
Please stop being so negative about the Medical Profession and what you presume will be their treatment for you...if you go to the right Docs then you will be properly assessed blood/urine etc analysed.
Maybe your relatives did not have WG...it is not a familial disease as far as I know. Basically I truly feel you need a definitive diagnosis so that you can be treated and you must stop second-guessing your diagnosis, if such as WG has never been put to you.
Take care, I wish you well and hope you get an answer.
[quote=“SABG”] you must stop second-guessing your diagnosis, if such as WG has never been put to you [/quote]
WG was put to me as a possible diagnosis by an NHS consultant, that’s the reason I started this thread. But that diagnosis it’s incompatible with my 25 year history of systemic vasculitis, whereas my photosensitivity and scleroderma matches MCTD, which is a differential diagnosis for WG …
[quote=“SABG”] … just told I had secondary WG !!! what was that ??? …[/quote]
As can be seen from the list of differential diagnoses above it is possible to have vasculitis secondary to rheumatic illness (en.wikipedia.org/wiki/Syste... ). Not all vasculitis is WG.
[quote=“SABG”] Please stop being so negative about the Medical Profession [/quote]
Criticism, justified or otherwise, won’t do NHS doctors any harm : they have a captive market.
Some people would prefer to be in blissful ignorance rather than entertain the thought they could be mistreated by the “wonderful” NHS , ( either through wilful-negligence in the name of economy, or excusable/inexcusable ignorance ). As I’ve seen my syndrome play-out repeatedly over multiple generations of my family , undiagnosed/misdiagnosed by the NHS, then it’s not possible for me to be in “blissful ignorance”.
[quote=“SABG”] ...if you go to the right Docs then you will be properly assessed ... [/quote]
I was sent to an NHS rheumatologist to determine whether my syndrome included dermatomyositis , however he omitted to ask me to close my eyes during the physical examination : the #1 diagnostic sign for dermatomyositis is on the upper eyelids … google.com/search?site=imgh... , [ I don't have enough room here to list all that NHS employee's deficiencies ].
[quote=“SABG”] ...if you go to the right Docs then you will be properly assessed … [/quote]
Please tell me where I can find one in Britain who will take my money,
I see what you are saying and you say you were given a possible diagnosis of WG/GPA but it does seem from what you have also said, that you do have a different set of symptoms from the general run of the mill for WG/GPA.
I personally was not critising the Medical profession in Great Britain for their delay in properly treating my own definitive diagnosis of WG/GPA but in the ignorance of Consultants not 'au fait' with the advance in knowledge about current treatment for such rare vasculitis diseases.
This is why Vasculitis UK has been instrumental in spreading the information and knowledge in cohorts with specialised Vasculitis experts to the Medical fraternity...there is now very wide exposure amongst the Medics to refer patients with Vasculitis to appropriate Teams able to deal with it.
I do not understand why 'on earth' you would have the temerity to ask anyone of us on this site to find you someone who would take your money. If you are so 'rich' why cannot you do it yourself... just look up Vasculitis Teams in the Private Sector.
I doubt very much that whether any of us on this site would want that 'drink' you promised if we found you someone from Britain to take your money.
Do you actually reside in the UK???
Regardless, please take care and I hope you get some answers.
I’m not so rich, but I do have sufficient funds to have my illness officially diagnosed if only I can find a relevant doctor who will take my money. I’d like to put an end to the era where my familial syndrome is ignored by the NHS, and am willing to spend everything I’ve got to achieve that goal, ( I can’t take it with me ).
Is it immoral for me to pay for my own treatment and expose wilfully-negligent and occasionally-incompetent NHS employees in the process ?, isn't that good for everyone ? , (except the wrongdoers).
[quote=“SABG”] why cannot you do it yourself... just look up Vasculitis Teams in the Private Sector.[/quote]
All of the relevant UK "private sector" consultants I have found are also NHS employees : to take my case privately would cause them to expose decades on NHS malpractice in my case and that of my affected blood-relatives , that would make them a whistle-blower which could damage/terminate their medical career in Britain. I believe that that conflict-of-interest is the reason they will not take my money. It’s a long-shot that anyone in the UK can get involved, but I thought I’d ask people in this forum who may have been in the same boat as myself, ( which under the NHS is up the proverbial creek without a paddle ).
Sorry if you find the subject of money upsetting, but money is the reason a blind-eye is being turned to this illness in myself and my blood-relatives by NHS employees. [ doubly unforgivable IMO as not treating this syndrome has been a false economy in most cases , but a policy of wilful-negligence as standard-practice applied-nationally saves more money than it loses, so the NHS aren’t going to change that policy any time soon ].
[quote=“SABG”]… Do you actually reside in the UK???…[/quote]
Outside the UK consultants would bite my arm off when I offered them money rather than blanking me or shooing me away as they do in the UK, ( doctors not on the NHS payroll don’t have to turn a blind-eye to keep their job ). It seems seeing a consultant outside the UK is the only way I, (or anyone else in my position), can get a fair hearing.
The moral of the story: if a blind-eye is being turned to you by the NHS don't waste too much time looking for a private doctor in the UK to help you , they don't exist.
I know of a number of the more 'esteemed' specialists who I'd rank alongside David and see no problem in naming them here. Professor Lorraine Harper (Birmingham) and Dr Richard Watts (Ipswich) are both very highly regarded and have supported Vasculitis UK in a very positive manner. There are of course many others, who I'm sure members here would personally recommend but if I were you, I'd approach Dr Jayne again, especially as there have been significant changes in the the understanding of the vasculitides and it would appear, in your own condition.
Clearly your condition is complex and atypical but from my experience, you're not alone. There are many highly variable symptoms and anomalies within test results which make this suite of diseases very difficult to understand, let alone diagnose and treat.
I don't agree with your belief that paying will get you a definitive diagnosis, something you clearly desire. If it were me, I'd be focussing on the most appropriate treatment and I also don't believe there's a private consultant, at least in the UK, who is more able than any of the highly regarded vasculitis specialists who treat people under the NHS.
I had Wegener's type symptoms* for most of my life, until a catastrophic flare up just before my 32nd birthday that caused me to be diagnosed. I'd never been given immunosuppressants, and I didn't die!
Don't forget that Wikipedia can be edited by *anyone*. I could set up an account today and claim to be an expert on an obscure topic. I don't trust that site at all for anything more important than movie trivia.
*I'll never know if it was Wegener's, but it seems very likely
( their WG page apparently written by a Mayo Clinic Doctor ), which largely agrees with the wikipedia entry : "82%" instead of "over 90%" for mortality , but both agree on "five months" average life expectancy without treatment.
Someone mistakenly diagnosed with WG when they actually have another form of vasculitis may still be receiving the correct treatment.
I have had Wegeners Granulomatosis since 1971. Ergo I have been in remission since 1975 which is the year I was asked to go to the NIH across from Walter Reed Naval Hospital. Dr. Anthony Fauchi was my attending dr. After 18 years, I was told that I didn't need go to NIH any more because I had been in remission for too long. God Speed my friend
I have asked that question. Was diagnosed in about 2005, but never treated. Right after diagnosis my parents got into a severe car accident and I had to leave the state for some time in order to help them. After returning, my rheumatologist had retired due to illness and UCSF could not locate my medical record with his notes and tests. I have suffered with illness for so many years. Today I woke up pondering if it would ever end - I am in hell, and this damned plague has not helped my cause. I have read that people die if left untreated so I had accepted that I don’t have it until I was diagnosed with vasculitis again last year - I question it again. My doctor told me I was an actual zebra and not just a horse.
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