Living with wegeners

Hi my name is Russell I have been living with wegeners for a year now when I was diagnosed I was told how rare it was but it seems to be not as rare as they told me I usually just like to read people's post and have noticed a couple of members attend fvrh Larbert as I do for wegeners also the ambulance man when I was being transferred to Monklands his wife had wegeners and a nurse in Monklands mum has it too so for something so rare I seem to have come across quite a few people with it I also suffer with subglottic stenosis due to the wegener's and hearing loss other than that I seem to be coping quite well main problem I have is the dvla finishing their enquiries into my fitness to retain my hgv licence has taken 6 months so far stopping me from returning to work other than that I live life normally seem to have been luckier than some people sorry for the long post just thought it was time to write something instead of reading also had my esa stopped as deemed fit to work

15 Replies

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  • Hi Russell,

    I live in Scotland and attend FVRH as well.

    The definition of a rare disease is an incidence of 1 in 2000 patients I think. GPA is rarer than that but not so rare that you don't come across other patients!

    Do you see Rheumatology at FVRH and what treatment are you on?

  • Hi keyes I attend a pulmonologist as most of my problems were with my lungs I go every 3 months now I take 10mg of prednisone also azathioprine and omeprazole have also had the cyclophosmade I also attend Monklands for the subglottic stenosis had my trachea lasered, dilated and injected with steroids I had minimal kidney involvement but that has cleared up with treatment apart from the tracheal problems I feel ok cheers

  • Hi

    I'd be very interested to hear about the treatment you received for subglotic stenosis. I myself have these symptoms and have struggled with my breathing for the last 2 years. I've been told by the specialist that no surgical procedure can be performed until wegeners is controlled. I'm having high dosage prednisone at the moment with 3 weekly infusions of cyclophosphamide but it seems to have had little or no effect on my breathing. I would love to know more about your case. Where you had treatment, who you saw, what was involved etc. I live in Yorkshire and see specialists in Leeds and ENT at Wakefield.

    Has your breathing/airway improved after these procedures.

    Hope to hear from your.

    Best wishes

    Lee Porter

  • Hi lee sorry to hear you are having the same problems as me I started of with cyclophosmade but it was in tablet form and high dosage prednisone I am now on azathioprine and 10mg of prednisone.i kept telling the dr that I felt as if something was stuck in my throat like when you get emotional about something so finally got an ent to have a look Which showed inflammation and scarring he didn't know what to do as he hadn't seen it before so I had to go to Monklands hospital in Lanarkshire to see a specialist who made five cuts in the stenosis with a laser then dilated it with a balloon then injected the area with high dose of steroids when I woke up the feeling was great being able to inhale large amounts of air freely didn't even have one bit of pain only thing is it isn't a cure but can be repeated to elevate the symptoms mines is returning after 3 months but I have read some people need it done a couple of times to get longer relief the consultant I seen was a Mr kang who is very good I go back to see him in a fortnight sorry about the long reply feel free to ask anything else if I can help best regards Russell.

  • Thanks for the reply Russell. Sounds almost identical to my situation.

    My ENT specialist is not keen on performing any surgical procedure as they claim it could make things worse, but personally I don't think things can get much worse. They have talked in the past about a tracheotomy which would be worse case scenario in my eyes. My airway is about the diameter of a pencil, but they hope my ongoing treatment will improve this. I'm really keen on some sort of surgical intervention and I've heard of the balloon dilation technique. Even if it is a short term fix it is something that might help out in the long run.

    I have been referred to a throaty specialist in Salford Manchester but I'm still waiting to hear from them after 3 months.

    I'd be really interested to know more about your case. Treatment, timescales, medication etc. I could use this then as leverage when I have my appointments.

    Obviously I'm happy to chat via this forum, but if it makes it easier to contact me by email then send to leeboyp@hotmail.co.uk

    Best Wishes

    Lee

  • Hi lee your situation does sound the exact same as mine I was told I was breathing through a straw sized hole I could walk around but couldn't walk and talk the timescale was ent camera then an urgent ct scan two weeks later then I was to wait a month for ent appointment then about 10 days later I was having a shower then getting dried and was really out of breath and I couldn't breath and started to panic luckily it wore off took myself to a&e the Dr's never had a clue I had to tell them to Google it.i then got transferred to Monklands hospital the following day as they have throat specialist.i seen the consultant and he booked me in for the op as soon as he could and I was discharged unfortunately I only lasted a week later back into a&e with breathing problems transferred to Monklands again with blue lights and sirens spent two days hooked up to an oxygen mask I got the op 5 days later I think the op takes about 45 mins done through the mouth I woke up and the instant relieve was amazing I felt great I don't have any meds related to do with subglottic stenosis only the steroids and azathioprine for the wegener's I have only had the wegener's for a year and this is the only problem I have I have been doing a little research as we all do and I have seen someone who takes rutuximab and it keeps the subglottic stenosis at bay my ent told me it depends on the individual how long the results of the op last I to have been told I might end up with a tracheostomy I would rather they keep doing the ops it can be done as a day patient I felt I could have went home straight after it but had to stay in as it was the first time a lot of people seem to rave about the specialists at addenbrookes for treatment my wegeners is in remission just now so I think that's why I got the op I also have a hoarse voice and cough a lot I don't mind answering through the forum I check it everyday for new posts and info best regards Russell

  • Thanks Russell.

    Almost 100% identical.

    I might push for Rituximab and once things settle hopefully go for day surgery.

    I've gone 11 years without major issues but this time nothing seems to be helping with my breathing. Unfortunately because I've been on really high steroids 60mg + extra during infusions, I've put a bit of extra weight on which obviously doesn't help with general fitness and breathing.

    Hopefully I'll keep you updated over the next few months.

    Take care

  • Hi lee

    I understand about the weight gain I lost 3 stone before diagnosis then put 4 back on because of the steroids I forgot to say I have also lost some hearing in my left ear if this stenosis remains my only problem and I can continue to get treatment for it I will be quite lucky compared to some gpa sufferers I am also of similar age 45 used to run 6 mile a day until this misfortune I will put a new post up after my next visit to the ent hope you get help as I know how frustrating it can be have you checked on you tube there are quite a few videos about subglottic stenosis and the ops to alleviate the symptoms all the best Russell

  • thank you guys for sharing; my boyfriend have a pulmonologys check his lungs and throat due to his severe difficult to breath; luckily everything was OK, but the breathing problems seems to come from the inflammation on his ear and tonsil area. when inflamed it seems to close his airways.

  • There are around 8000 "recorded" cases of Wegener's Granulomatosis here in the UK, it is now known as Granulomatosis with Polyangiitis. As Keyes says it is rare but not as rare as the other ANCA associated types of vasculitis.

    My husband John was diagnosed nearly 16 years ago but the WG affected his lungs. He returned to work 3 months after being taken ill and starting the treatment. He worked for another 5 years before taking slightly early retirement. If you want to chat with John on the phone you can call this number 0300 365 0075 as he is manning the helpline at the moment. If you do phone he will also give you his home number should you want to call again. It is important you are seen by a doctor who has experience of vasculitis. We know a guy called James who also has WG diagnosed 8 years ago and he lives in Glenrothes and attends the hospital in Kirkcaldy and is seen by a Dr John McLaren who is a Rheumatologist there.

    There are pages in the VUK website you may find helpful as limited WG ( WG not affecting any vital organs) can affect the nose, sinuses, hearing and throat. I know many people where the WG has affected them this way, the youngest being 17 years old.

    This is the page which tells you about how it can affect the hearing vasculitis.org.uk/living-wi...

    There is also a good page on the website discussing work and sick issues and benefits. This was just recently updated

    Sick from Work vasculitis.org.uk/living-wi...

    In Work Benefits vasculitis.org.uk/living-wi...

    In fact the full list of information about benefits can be found on this page vasculitis.org.uk/living-wi...

    Hope all the above is helpful

    Best wishes

    Susan

  • Hi Susan thanks for the reply I had to take myself to a&e to find out why I was so ill I lost three stone in two months I could hardly walk because of joint pain and was coughing up blood my local gp just gave me pain killers I was admitted straight away with suspected lung cancer my lungs were full of shadows and bleeding luckily after a lung biopsy and five days later I got a diagnosis and began the usual treatment of steroids and cyclophosmade I am glad I found these sites as this is where I have all the info there is about gpa the consultant I attend has been treating another person for ten years so I hope he knows what he is doing I attend every three months now and my lungs and kidneys have repaired with the treatment I am now on maintenance treatment of low steroids and azathioprine and seem to be doing well thanks

  • John first presented coughing up blood too , a lot of blood, but he was admitted as an emergency. The doctor on duty that day had seen WG before and a "hunch" it might be WG. Luckily for John his hunch was right. John is seen every 12 weeks too, but he now takes Methotrexate after 9 years taking MMF, sadly for John the Azathioprine did not control his WG.

    Good to hear you are doing well 😊

  • Hi Russell, I also attend the FVRH Larbert. I was diagnosed in 2010, with Vasculitis Microscopic Polyangiistis (MPA) and attend the renal centre as my kidneys are involved. Just going through a flare at the moment, and waiting to see if they are going to put me on Retuximab as they have said they cannot give me anymore Cyclophosphamide, or iImmunesuppressants . Never met another person with Vasculitis except on this site. Wish you all the best Steph.

  • Hi Steph thanks for the reply I also used to attend the renal dept as I had limited kidney involvement I have been discharged from the renal clinic as I now have normal kidney function I only attend the pulmonologist every three month's as most of my problems were with my lungs I also attend Monklands for my tracheal problems had an op two months ago I hope you get the treatment you need I am on azathioprine now it seems to be working for me all the best with your recovery Russell

  • Hi Stephanie,

    Sorry to hear you are flaring. Here is a link to the SMC guidance on Rituximab which states quite clearly that it should be funded if Cyclophosamide has failed to control MPA. It has been a wonder drug for so many patients with ANCA associated Vasculitis

    scottishmedicines.org.uk/fi...

    My e mail address is lynn@vasculitis.org.uk if I can help in any way.

    Best wishes

    Lynn

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