Dear friends, family, loved-ones and curious passersby...
This is my Wegener's granulomatosis [WG] story. My symptoms started in December 2006, I was finally diagnosed in October 2007 and I went into my first quiet phase in April 2008. Now that the WG is quiet we are working to fix the problems I am left with - namely hearing loss and narrowed airways in my lungs. WG is a auto-immune condition and a form of vasculitis, this means that my immune system started attacking healthy body tissue and my blood vessels are inflamed. Treatment is steroids to reduce the inflammation and immunosuppressant drugs to stop my immune system.
September 2008 I will hopefully be exploring all kinds of physical activities following my dilation next Tuesday. Before I caught a cold last week I'd managed to cycle to the hospital and back two days in a row! I also finally got to try out my mini-trampoline. Fantastic, I managed to bounce for probably 30 minutes all told. It took 45 minutes, but I had to stop and rest several times. Definitely a viable alternative on days when I'm not up to walking. I plan on introducing the yogalates soon too. I miss being active so much and it drives me batty not even being able to walk up the stairs. The other good news is that my redundancy payment has come through this week so Kara and I have ordered a rower. I love rowing and it'll be yet another thing I can do inside. I'm not sure how my nose will cope with the cold weather this year. Hearing aids are still strange in places. My ears get rather warm which I don't like. I also find that all the constant sound input is tiring; I prefer leaving them out when I can. Definitely better than the alternative and I can wear them comfortably for a day if I need to.
Update 28th September I hung out in my sexy hospital gown with the fancy open toe DVT socks for a while and finally went to theatre around 1. I woke up fairly comfortably in recovery and was taken straight back to the ward around 4:30. No reaction to the anaesthetic this time. I was hopeful at this point that I might get to go home, I felt fine. Unfortunately, I didn't get to see a doctor until 9:30 though so it was just easier to stay. I got home around 11 on Wednesday. Happily, they managed to dilate all three narrowed spots! My throat was quite sore from the work on the subglottic stenosis. On Friday I asked for numbers and my doctor said he could never remember the measurement conversions to tell me. I got up to eight Frenches. He was really pleased with how it went and so am I. Vasculitis doctors were also very pleased with how things were going. I asked about coming off the drugs and they said they'd consider it in the spring. He gave good advice for while flying/travelling in November. I had my first go on our rower today. I managed 10 minutes and 1600m, on level 4.5. I am pleased with it, but I'm battling myself as usual. I couldn't help but think I used to do over 2000m in 10 minutes on level 8. Never mind, focus on the good. Rower is awesome and I'm so pleased to have access to it any time I want.
August 2008 Now that I can hear I can cycle again too! I've been out three times, it feels amazing. I'm taking it gently since it is much harder on my lungs than walking. I've also been able to hear my piano again so I've been playing. I also treated myself to a beautiful wind chime. I can hear people talking to me, the rain hitting the window and the phone ringing. I am still a little way off being perfect with them, but they are wonderful. I'm very excited for 15th August - I get my next lung dilation. It'll be via a bronchoscopy with sedation instead of general anaesthetic. I had three spots of narrowing - in the lower right, the upper right and sunglotic stenosis. The last dilation fixed the lower right. The hope for this time is that the lower right has stayed open and they'll just be able to dilate the other two. I feel much better in myself; being able to cook and clean more and being able to cycle has helped to no end. I'm hoping to start studying counselling again - maybe before the end of the month. I feel more like me than I have in a year, but I'm still a way off.
Update 16th August I’ll go back again sometime in September for another go under general anaesthetic. I felt really grotty yesterday, coughing a lot. Couldn’t lay down to sleep so didn’t nod off until around 2am, so didn’t wake up until noon today! Trying not to move too much as brings more coughing. I feel rather foolish for getting my hopes up. I knew that there was a >50% chance of it narrowing again and I suspected it had over the last couple of weeks, but I still hoped. Ah well, will continue doing what I can when I can and hope for the best with the next few dilations.
Update 29th August
July 2008 I get my hearing aids fitted on the 30th of July so August is shaping up to be an incredible month for me. I'm going to get to try cycling again which I'm very excited about. I can do it, but I feel very scared not being able to hear. I've been awarded disability living allowance which helps with my travel and care needs. I have also got a disabled persons railcard which will give me and my carer travelling with me a discount. I'm very pleased with all of this. Everything feels so much safer knowing that we have enough money to take care of us and our home. The gardener is coming twice a month to keep the garden in check. The cleaners are coming monthly since they ploughed through so quickly. I feel more relaxed now than I have in a year. Things are definitely looking up finally; I'm hopeful that it'll continue this way!
June 2008 Very good news is that I have been approved for incapacity benefit. This means that we can hire someone to come help me with the cleaning and the gardening. I'm very grateful for that help, it has been very frustrating seeing something I want to clean and not being able to. We are waiting to hear about disability, my application should be even stronger now that we know the hearing loss is permanent.
Update June 12th The best fix for the moment is hearing aids. I'm getting a volume control model with different sound packages pre-programmed in. So if I'm listening to headphones I can have one programme, if I'm listening to TV I can have another, if I'm in a crowded place I can make it directional. They took moulds of my ears and I'll go back to get them in four to six weeks. The good news is that my bone conducting hearing test results are normal. The hearing loss is caused by the scar tissue not nerve damage. So long-term there might be other surgical options, but hearing aids are the least invasive.
Update June 29th I saw the ENT first; he was pleased with the state of my nose. He said we could look at corrective surgery for it and my ears in a year or so. Any surgery has to be done very cautiously because WG loves having a reason to cause more havoc. He said that my eardrums are no longer perforated from the grommets so I'm free to swim etc. The grommet in my right ear is just dangling there, it isn't in anymore. He asked about dizziness and I said only when I lie flat on my back. This is called benign positional vertigo. We went to a treatment room and I sat on the bed and he held my head and brought down to hang off the side of it, facing once side then the other. We did that a few times then he had me sit up. I felt a little strange, but it seems to have done the trick. I couldn't wear my listening device while they did it, but I think they said that I had something foreign in my labyrinth and by moving my head like that they could dislodge it and correct the problem. Saw vasculitis next, they were also pleased. My treatment didn't need any changes and the rash on my back is not a WG rash which is very good news. My last blood tests were all in the normal range! I had a slightly elevated liver function last time but it has calmed now. Hopefully they'll stay normal. I finished up with the chest doctor, they too are pleased. I'd lost a litre of volume in my lungs and now - a month after the first dilation I have gained 550ml! This is excellent news. I'm in the queue to get the next dilation in the first week of August. He has also booked me in for a fit-to-fly test in September to see if I'll be able to go on the plane in November without my own oxygen supply. He said he was fairly confident that my lungs would be near normal in time for my holiday. I'm not counting on it, but it would be very nice. I saw my counsellor last Thursday, we've finished up. I realised that I needed to adjust the way I think about myself and my actions - I need to allow myself to treat me like I would anyone else. I've always had a separate set of rules for me and I need to stop it. I'm happier just knowing what the issue is, I'm hopeful I'll be able to move forward now. Finally bit of good news - only one doctor's appointment in July! I don't see ENT/vasculitis for two months and I don't see chest people for six weeks.
May 2008 The physiotherapist called this morning and I'll be seeing him on the 12th - he'll help with my breathing and the joint pain I've been having. I'm excited to have ways to help myself other than drugs. I was pleased this week because I didn't have a single appointment to attend. I'm making up for that now, I have nine this month. Mentally, I'm feeling better, we upped my antidepressant. I'm also seeing the counsellor; she already had a number of suggestions. Getting a poster for the view [something about humans originating on the plains of Africa so long views help us feel better], volunteer work or something to help me to think outside of myself. I also started my own little programme, a project a day. Things like cooking or cleaning [e.g. brownies, organising our medicine cabinet and tidying up the dining room table]. All of these have made me feel more like me and like I'm worth something.
Update May 9th He did say as far as they're concerned there is no reason I can't go to the US in November, but said I should check with the chest doctor. I see him next week so hopefully I'll be able to book tickets then. I am feeling pretty good mentally now. More like me. I contacted a few charities to see if there was anything I could help with from home. I have a few more to contact still. We'll see how many want a house-bound deaf person's help!
Update May 27th
It also made me very grateful that I didn't dither or change my mind about applying for benefits. Adjusting to life without hearing in a permanent way is going to be a challenge. There is a local and national charity for people with hearing difficulties so we're going to contact them. We're also going to look into sign language classes. Right now I feel very manic, I have been very anxious about this for the last few weeks. So now I know the result so all that anxiety is flowing out. I am in fairly good spirits truth be told. I am very lucky; I have a great team of doctors all working together to provide the best care for me. I can still do most things and I'll just have to adjust. Also want to say yet again how wonderful Kara and her family have been. Her Dad very kindly came to get us at 6:30 this morning for my admission and both her Mum and her Dad came to collect us on a moments notice when it turned out I was being discharged today. Kara waited for me at the hospital all day and kept me calm and ran all our errands. Also, I have seen the counsellor once more and things are going well with that. I also saw my GP, he seemed very pleased with my current state and I'm not due to see him for another five weeks - I have been seeing him monthly until now. Physiotherapist was excellent. He has given me a lot of tips for my breathing and given me exercises to help all my joints. Very pleased with him. And I left the best for last - the chest doctor said there was no reason I couldn't go to the US in November so we've booked the tickets. I get to see my family for Thanksgiving!! This will be my first Thanksgiving in 10 years. I am very excited! I think that's everything, any questions can be sent via e-mail [upper right] or if you know my Mom or Kara call them!
April 2008 The bronchoscopy didn't happen. They ask that you don't eat for four hours before the 2:30pm appointment. So I thought I'd have a slightly larger breakfast of eggs and toast to keep me from being too miserable while I waited. I finished eating at about 9:45am; well outside the four hours. I went through the check-in, signed the consent form and had my nose/throat numbed and the sedative injected [twice! The first time it must not have been in a vein]. I went out and when they went in I vomited. They tried again and I gagged - I don't remember any of it, the sedative put me out. So I have to go back again next week - this time I can't eat for seven hours beforehand. I haven't decided if I should just not eat at all or if I ought to get up early and eat then go back to bed. Happily, all this nausea had a weight loss side-effect. All of the weight I gained while unable to move and on high doses of steroids is gone, I'm only three kilograms heavier than before I got sick. Kara and I went to see our GP about counselling and I've signed up with the counsellors at the doctor's office. Kara is going to see someone through work. He also suggested we talk to the vasculitis people about more general groups I could join [i.e. vasculitis overall, chronic pain, life limiting conditions]. The problem is WG is so rare [or under diagnosed depending on who you ask] that there is not an awful lot of support for people with it. The people who were running the course we'd signed up for and couldn't attend also passed my name to a community matron and I'm meeting her on the 24th. She seemed fairly positive on the phone - this would be for more practical support like breathing exercises or making it easier to move around the house by myself. We'll see what happens, I figure even if I'm not really in her field it can't hurt to meet her. We've been chasing the vasculitis clinic this week because I was supposed to have my appointment two weeks after my last injection and I'm worried that I haven't heard from them. I haven't heard from the audiology department either. I'm trying to be patient, but I want to hear so badly.
19th April Update This is what we've been working towards for all these months. The theory is that if we can correct the last few lingering problems I'll be able to move on with my life finally. I've started a new drug - Azathioprine. This is a less-toxic version of the cyclophosphamide so it is going to continue to suppress my immune system. I'm still on 5mg of the steroid. When we were finished there we went to see the chest doctor. He said that the bronchoscopy earlier in the week was successful and they know I have narrowing in three places. One is in the main pipe before it splits off to each lung. The left lung is fine [YAY!] and the right has two more narrowings. The plan is to widen these spots with balloons under general anaesthetic. He can only do one spot at a time and there is more than a 50% chance that they'll narrow again. In which case they just do the same procedure again until it sticks. So my hopes of breathing within a couple of weeks were premature, it'll probably be more like two months minimum. If I understood the maths properly I've lost 40% of my lung capacity to this narrowing which is why I get short of breathe so easily. After the chest clinic I had to have another CT scan of my lungs so he'll know if the narrowing continues into my lung beyond these points. Then we went to the day surgery unit to do my pre-assessment for the grommets procedure. If you are unfamiliar with grommets, I'll explain: they're tiny tubes that are inserted into the ear drum to allow fluid to escape the Eustachian tubes. I'll be able to hear better immediately. There is a chance that my hearing has been damaged and I might have to have hearing aids as well, the bone hearing tests I've had have been in the normal range, but I think it is one of those things that you only know for sure after the fact. I am cautiously optimistic I think. I had more blood tests after that. Kara was wonderful and collected prescriptions, lunch and cancelled the hearing aid assessment appointment on Monday for me while I was waiting all over the place. We were also able to cancel my ENT appointment on the 1st because we saw him at the vasculitis clinic. I expect I'll see him after I have the grommets. I don't have a date for any of the surgeries yet but I'll let people know as soon as I do. I want to be happy that I'm officially in my first quiet phase but my other issues make it hard to feel.
23rd April Update Also saw my GP. He went over my blood tests with me. We've been looking at the CPR level to see how I'm doing; at my peak in January I was 148 - normal is 6 or less. In March I was 25, at the beginning of April I was 2 and my last test had me at 6.
29th April Update Physically I feel better now than I have in months. I am very frustrated because I want to start pushing myself and moving, but I can't breathe well enough to do that. So I'm waiting and trying to push myself in other not so taxing ways. I hate waiting.
March 2008 Physically, I have had pain in my muscles and joints; every day has a different pain. In WG circles we call the 'roving pain' because there isn't any direct cause or anything that helps [in terms of exercise or stretches]. Luckily, a mild over-the-counter painkiller takes care of the worst of it. I've been sleeping better this month than I have in the last two years. The night after my last injection I slept for 17 hours. Unfortunately, things on the work front do not look too positive. Two of my colleagues have left and I fear that there will not be a place for me when I am ready to return. Anytime I think about returning to work I get stressed because I don't know how to work with WG. I'm scared of flares. I worry that I might never get to a quiet time and I'll have to spend the rest of my life on incapacity benefits. I am trying not to think about it because I end up feeling stressed and helpless which doesn't do me any good. At my last appointment with...
Turns out that the course is only for terminal people so we are not going. We have an appointment 4th April instead to see about counselling in some other capacity. We need some help. As always I welcome any e-mails that anyone cares to write - address on the right. I'm not so good on the phone, we have got an amplifier for me to use but I still find it stressful. Kara is happy to talk to anyone though so if you'd like to know more she is happy to share.
February 2008 * This was my third injection, now I can move to having them every three weeks instead of fortnightly. Next one is on the 22nd. The injections take a long time, there's saline solution, an anti-nausea drug and the cyclophosphamide itself - we were at the hospital from 9am to 4pm. This makes me very tired and I spend the weekend after lying around. I'm still coughing a lot, losing my voice randomly, wheezing and being generally nasty in the lung area so I have been referred back to the chest people. Presume I'll get a letter with the appointment sometime this week. My left pinkie and the outside edge of my hand are constantly asleep [pins and needles] this moves to the tip of my left ring finger sometimes. My other limbs fall asleep very quickly so if I'm not careful and change positions often I end up being very tingly. I gather this is a normal feature of WG, one woman I read about has both her legs asleep all the time, so I'm grateful that it is limited to just my pinkie. Saw the ENT today [21st]. My left ear drum is perfect - no more scary bubbles! My right ear still has inflammation behind the drum. He suspects there is inflammation behind the left too, but we could probably put a grommet in anyway. He is going to consult some colleagues and send me a letter. After cleaning my ear [bliss!] he said I could stop the ear drops! I have had to put them in three times a day for the last two months. This is excellent. He gave me a kit to do the douche with - no more sniffing the salt, soda and water mix from my hand! I have to douche one to three times a day at the moment to keep my nose clear. All in all a satisfactory visit, I see him again in three weeks. I've been having a lot of pain in my leg muscles, ankles and feet. Despite this I am currently walking an hour a day - I'm hoping to add a second 20-30 minute walk daily. I love the freedom of being outside and moving, without my hearing though I feel vulnerable. I rode my bike to the grocery store weekend before last, first time I've been on my bicycle since October. It was wonderful, but again without my hearing I felt vulnerable. The pain in my legs started to mirror in my forearms and hands yesterday. I'm hoping it is a one-off, but we shall see. I'll be discussing this with vasculitis doctor when I see him next. Happily, I've been able to reduce the liquid morphine - I haven't had any since the 16th of February. I'm still taking 120mg of morphine MST every day though. I see my GP on Monday and I hope we will look at reducing that as well. The steroid reduction is going well; I'm down to 25mg and still feeling okay. My breathing is still laboured and wheezy, but I haven't had a serious coughing fit for a few days. I'm sleeping fantastically well - last night I went to bed around 9:15 and got up at 7. I woke several times in the night, but I was able to go straight back to sleep. I need at least 10 hours a night at the moment, I get tired very easily.
January 2008 I get another CT scan of my chest, a bronchoscopy, lung function test and more blood tests. The end result is that the methotrexate was beginning to affect my lungs and I had a chest infection. I'm in hospital for the day and at the end they tell me they want to admit me for a few days and do more intravenous antibiotics. Following the disastrous stay in November I beg them to please come up with another plan, I'd do anything. So I end up going to the hospital twice a day [8am and 5pm] for a five days. This is much better for me and the treatment seems to go well. Meanwhile, I'm still on steroids, painkillers and oral antibiotics. I finally feel that the antidepressants are doing their job and consider reducing or stopping the beta-blockers. I've been having a lot of pain in my legs, started on the 18th. At first I thought it was just rebuilding muscle, but it moved into my ankles, feet and thighs. Walking, stretches and/or resting didn't seem to make any difference to the pain. I have kept up with the walking and I can do 20-30 minutes at a fairly quick pace so I'm pleased. I still can't go far enough to get to any shops of note, but I'm getting to see things I haven't seen for months. Saw GP on the 31st, I am off the beta-blockers. I've been signed off work for another four weeks. I have a new antidepressant [a tricyclic one] to help with night pain and sleeping. He also recommended taking paracetamol full-time until my leg pain goes. I only have to do drops in the right ear now - left ear is looking better.
Keep reading... December 2006 to December 2007 |
Thank you for reading! Send questions to amy[at]spacedog[dot]gotadsl[dot]co[dot]uk
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