How do you know when to increase or decrease your steroids?

Although my vasculitis was not diagnosed as cerebral, from comparison with other people on this site it appears to be. It's onset was sudden with violent headaches and 4 days of intense stomach cramps, in Sept last year. In Nov after about 10 weeks of crippling headaches I was diagnosed and put on a two weekly reducing regime of steroids starting with 60mgs. It never took away the feeling of intense, vice like pressure in my head but it kept severe headaches at bay and I was definitely functioning better.

At 8mg I seemed to get worse so my neurologist advised I returned to 12 and a half, but said I was the only one who could really tell when it was bad enough to have to go up again. I hung in on 8mg and for a few days it seemed to get better. I even felt so 'almost normal' (for the first time in 6 months) that I cleaned my gutters and did some gardening but, as I have just learnt from this site, it was a hot day and I have now plunged again into feeling like I have a meat grinder in my head.

So I have given in and gone back to 12 and a half, which I am advised to stay on now for a month. What I'd like to know is if the steroids are supposed to make us feel normal in the head, or do most people have 'meat grinder' symptoms all the time and we just have to live with that? I guess I'm trying to work out if the 'meat grinder' is part of the disease, regardless of what we are taking, or evidence the steroids aren't working, thus requiring a higher dose. And if so what is considered high and how long can we stay on it?

Also when people speak of flares do they mean a sudden onset of symptoms as I have just described, and when that happens do they increase their dose, or hang in there on the dose they are on and wait for symptoms to abate? My specialist is very hard to get to see for these day to day questions, and I am not in the UK where you seem to have much more information about the disease, so forgive me if these questions are niave.

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  • You have to learn this yourself, based on your own pattern of symptoms. For example in my case when my steroids drop too low my symptom pattern will change, not right away, but often after 3/4 days, or sometimes even after a fortnight. My always severe bladder incontinence goes stratospherically bad, I have enormous trouble controlling my arms and legs (which are already bad to start with, but clearly worsen when my steroids drop too low), I get extremely confused, and I am very slow physically. My headaches may also increase.

    However I wouldn't raise my steroids because of problems I've had due to hotter temperatures, as in the last week here in Scotland. This is a problem caused by the outside environment, and my brain dealing with it, not a sign that the inflammation in my blood vessels has been increased and needs increased steroids to deal with it. So while raising steroids again might make me feel better in the short-term, it would be a mistake because the symptoms would improve as the temperature drops. I've fought hard to lower my steroids, despite a very aggressive case of cerebral vasculitis, and only raise them when I am sure that I absolutely need to.

    My symptoms never go away. Even when my disease is well controlled I have lots of permanent and irreversible brain damage which causes a huge range of day to day symptoms. I am severely disabled, from a young age. The difference between that and a flare is that the symptoms worsen dramatically, and this is sustained over more than 3/4 days. I can have little blips that last a few days, for example due to the heat, or just one of those things. But I don't adjust my steroids if that happens. I wait to see if things settle down. If the blip continues on for a week or more I have to take action though. It's all a question of learning to judge it, and it's not easy. So you have to learn to judge in your case what is the difference between your new normal and things going badly out of control. And that isn't necessarily easy to do, and is unique to you.

    And for the record we don't have more information about this form of vasculitis here in the UK. Patients with cerebral vasculitis are largely left on our own to deal with it, and consultants can rarely give us advice on what to expect, because it's such an extremely rare diagnosis (typically 1 case per million patients per year) and each case is very different. What you've read here has been learned by each of us with the diagnosis from our own difficult experiences, not from information that we have been given by others. This new website really helps to share information, but that information has come from patients themselves and their own experiences, not from outside people.

    I fell ill in 1994, diagnosed in 1997, and when I had my huge relapse in 2004 I went in to see my new GP and said "I think the vasculitis in my brain has gone out of control again". He responded that I would be the one to know. Which was nice, that he trusted me to judge it, but to be honest I had little experience then of judging flares and relapses. Between 2000 and 2004 my cerebral vasculitis was very well behaved. But this time I knew things were very very wrong. I was falling over constantly, to the right side, very weak down my right side, having huge trouble controlling my arms and legs, and my waterworks had worsened dramatically. Even I then knew this was a serious problem.

    Good luck!

  • Hi Bobs, your issues so resemble alot of the stuff i have and do go through. The main question i always used to ask the medics was what is classed as a flare up. Because even in my 'remission' period i had incidents where i would be suddenly drained and no strength, pressure sensation in my head sometimes with accompanying pain other times just the vice grip. i would have endless days of feeling dizzy and disorientated although apart from occasional days i stubbornly insisted on working. Up to this last summer when i was hospitalised again, then i realised this was a flare or this was a major flare. The first major one for four and a half years.It was Obvious to me and all around that however frustrating and unpleasant the previous interim episodes had been this (major) flare could not be disputed in any which way or form. But i would and my GP would agree that whatever happens in between is directly attributable to the CNSV i prefer to call these mini flares or a bit like aftershocks after a major earthquake.Last June when things rapidly accelerated painwise and pressure I like you at this stage was put on 60mg Pred which i know and have been told saved my life. This i hasten to add was initiated by my long standing GP who has overseen my care very methodically. Pre cnsv and after diagnosis thankfully. As i was admitted to hospital the specialist on duty not a neurologist or vasculitis aware medic wanted to withdraw my meds after only 12 hours whilst he awaited tests for meningitis. Thankfully my sons contacted the GP who insisted i stay with the pred. The first 48hours the pain stayed excrutiating despite morphine and strong anti inflammatories for pain relief but i started to get some mobility back in my spine and the pressure in my spine and brain although severe started to reduce. But even though the pain was not getting to an acceptable level despite the pain relief the treating consultant locally insisted after only 7 days to reduce to 40mg next 7 days 20mg and then to go weekly to 10, 5, 2.5 and finally 1mg for 2 weeks. Needless to say and to the horror of my GP and vasculitis medics since to expect to come off that level of pred in 6/7 weeks .....total madness. And i was rushed back in to hospital on my first day on 20mg dosage(3 weeks in) where i remained for another 3 weeks on 60mg. My then neurologist locally again tried to reduce slightly slower but still in big increments. This was more succesful and i was starting to have more 'normal' pain free/ or pressure free days until i got under 15mg. Then when i tried to get past 12.5 my body would not accept this level. I was immediately put back up to 40mg by my GP after talks between us,in48 hours what a difference. I am now under the care of Addenbrookes also,as my GP did not want an issue with local consultants again. My reduction is much much slower under their care and using and listening to reports from my GP and my description of what is happening nothing is overlooked no matter how trivial. My reduction now is 0.5 mg every two months. Once the levels start to get within the range of your bodies own tolerance/ point at which the adrenal gland makes its own corticosteroid (Pred) it is essential that the drop is slow to give the body time to adapt so that you do not go into adrenal crisis and allow a window for the CNSV to step back in. Obviously the higher the dose the more risk of problems because of the compromised immune system and risk of osteoporosis but provided this is backed up with alendronic acid. It is a risk that should be taken given the other odds or lack off when a flare reaches or threatens to reach this stage. I am now getting very good at judging where i need to be with my meds to the point that this next drop down will be deferred for a further two months. My GP will listen to what i say and make a decision objectively. He believes as the condition is unpredictable and shows in many ways that i am the only true judge of how i feel.

    Just for the record in the past i have felt desperately ill yet showed an unremarkable readingin the normal range. Yet my lumbar punture showed chronic inflammation within the CNS. This makes me think that Cerebral/CNS vasculitis because it is contained within the CNS does not always shown or can be judged by some of the standard tests used. Again not medically trained just my theory over the last 6 years.

    Sorry for the essay hope this helps

    Jo

  • Cerebral vasculitis routinely does not show up any abnormal levels in blood tests. I've read thus in medical papers about the subject, and found it through my own experience. Blood tests are no guide at all to this form of vasculitis. Sometimes they can be helpful, but they must not be relied upon. Symptoms are the only reliable guide.

  • On a lighter note Bobs despite my brain damage making my brain not always behave, in my 4 and half years remission time i went back into my post of professional sports coach andwas swimming recreationally at least a mile a day and representing in my specialist sport for the county and high level competitive locally. Throughout this time i never believed i could not get back in and even now on extended leave for the last year i am doggedly regaining stamina and fitness on the days that i can. Some days cutting all the grass at home other days getting totally wiped out just getting the mower out the garage. When you feel good have a go at stuff ( gutter cleaning included) when you are unable to do it just kick back and rest. But don't forget if you manage to clean the gutters and feel psychologically top form because of it. Then Go For It Girl! .... You're in charge of your Vasculitis it is NOT in charge of you...... Good Luck!!!!!

  • Hi Jo and Vivdunstan, thank you both so much for the amazing amount of helpful information, and thank you Jo for finishing on a slightly brighter note, even though it sounds like they nearly killed you through lack of information on reducing your meds after your flare. I was wondering if I was ever going to be able to do anything physical again. At the moment it seems any activity makes me feel worse, which I am beginning to call 'hot brain' as it feels like inflammation - vice-like pressure, eyes ache and are bleary, etc.

    After reading your accounts I don't know if I've done the right thing by pushing my Pred up. My neurologist had said if the steroids hadn't worked I would have died so when over the last week I felt my brain feeling inflamed again I panicked and increased it (2 days ago 8 to 12&half) and now I wonder, after what Viv said, if it was the right thing to do. And it sounds like I can't go back down again for another month at least, and yet so far the increase seems to have done very little good. How long does it usually take to get relief when you increase, and if I get no improvement should I go up even more? This is all pretty terrifying considering how lethal this drug is.

    Some other questions: what does CNS stand for?

    If the adrenal gland makes its own corticosteroid (Pred) do you think this could mean our bodies can eventually restore us to normal health?

    Are all Cerebral V sensitive to the outdoors and heat?

  • Hi Bobs, Don't worry about the steroid raising too much. It's unlikely to do you harm. You are on a low dose. The problem for me is I've been on steroids for over 14 years, and a higher dose than you permanently since 2004. That has caused me huge weight gain and has big risk consequences in terms of osteoporosis, diabetes, and other things. But you have only been on it for a short time, and don't face the same risks. And don't consider steroids as lethal, please. They are not. They look after you. Yes there are long-term health risks, but it depends how long you are taking them for. This disease will kill you without treatment. Steroids are really your friend :)

    I find that when I raise steroids it usually takes several days before I feel better, typically 3/4 days, although it can be longer. It isn't an immediate thing. If you get no improvement I think you should ask your GP for advice. You should not raise higher without seeking more advice. And as I've said before I'm very concerned that you only seem to be being treated with steroids and haven't mentioned any immunosuppression drugs. Steroids alone may not be enough for you. They usually are not enough for cerebral vasculitis where the brain is being damaged.

    CNS = Central Nervous System. Basically the brain and other related bits like the spinal cord. Cerebral means pretty much the same thing, which is why our disease can be referred to as cerebral vasculitis or CNS vasculitis.

    If someone has been on steroids for a very long time they are less likely to be able to manage without them. And no the body will not heal with this disease. It is incurable. The best you can hope is to go into remission, when it really behaves itself and stops the inflammation. But that is not a permanent thing, and the vasculitis can always return.

  • Hi Vivdunstan (and Jo),

    Your comments about steroids are comforting. I will try to befriend them. My biggest fear is the brain damage. That's why I increased the steroids. I thought if the inflammation had increased I was more likely to get damage, so I had to get rid of it as fast as possible with more steroids. Then I got scared I'd increased unnecessarily and have to go through months of getting down to 8 again.

    As you suggest I won't go up again without seeing my Dr. I'm on day 2 and still feel pretty dreadful but I will give it another 2 days before doing anything.

    I hope I'm not using up your life with all these questions but it is so amazing to get some answers to things I've been stewing on for months. So here goes:

    How can we protect ourselves from damage?

    Does it happen when we are experiencing the most pain? Sometimes I get painful stabs through my head and think to myself 'shit there goes a few more brain cells'.

    So far I have no evidence of damage, however my MRI shows small lesions in my brain so maybe it just hasn't been detected yet.

    Also my head feels bad all the time, so how can we tell what is steroid side affects and what is the disease?

    After a few hours after taking steroids the back of my tongue, my jaw throat and neck all feel swollen and stiff. Is that normal?

    Can you explain what immunosupressants are and why I need them. Are they related to preventing us from secondary infections? So far my body has remained strong and I have had no other illnesses. I have not even had fatigue, I am just partially incapacitated by constant, never ending, 'hot head'. I look normal and can be perfectly lucid in meetings or when doing something publicly, with strange bursts of energy that come from I know not where, but then after it and the next day I am totally wrecked.

    Does the disease have to run some sort of course before you get a remission, if you are going to have one, or can it be any time, perhaps soon (ie for me at 6 months in)?

    Did you say 1 in a million vasculitis sufferers get CNSV or 1 in a million people in the world?

    Soon I will have learnt a lot from you all and not have to ask so much, and hopefully give something back. But these are early days for me of panic, fear and sometimes abject terror.

    So thank you.

    Bobs

  • I can only reply to a couple of your questions. I am very knocked out, and can't reply more at the moment. I am severely disabled, including cognitively.

    1 in a million is per population. So per million people in a population, approximately, there will be 1 new case of cerebral/CNS vasculitis diagnosed per year. This ratio has been found in the UK, Europe, North America, and Australia.

    Immunosuppressants also reduce the inflammation in your body, and as a result they reduce the dose of steroids people need to take long-term. Immunosuppressants have big health risks too, but they are a standard treatment for vasculitis attacking major organs like the brain. They are like a bigger gun sort of thing, if that makes sense - a more aggressive form of treatment that is needed in more severe cases. It would be rare for steroids alone to be enough.

  • Thank you. Your help has been amazing. I'm so grateful for everything you have been able to answer. Bobs

  • Forgot to address the heat issue. I don't know. It hasn't been researched as far as I know. I've only started asking other patients this myself recently, and finding more and more have the same problem as me. It's rare among vasculitis patients, who can fare better in the warmer weather in some cases. But some cerebral vasculitis people, like me anyway, do not do well in heat, just like people with multiple sclerosis do too.

  • Bob,

    I don't think I can add much to what has already been 'said'. Certainly if I have an 'attack' i do raise my steriods up; then slowly, over a period of some weeks, drop them back down again. Good luck anyway.

    AndrewT.

  • Thanks Andrew

  • Bobs,

    Glad I could help; not that i could realy 'say' much, that hadn't been said already.

    Let me (us) know if there is any more help I (we) can offer you.

    AndrewT

  • If it helps, my Rheumatologist recommended reductions of 1mg per month max.

  • Every bit of info helps, thanks Bobs

  • Hi Bobs,

    I have cerebral vasculitis and was initially treated with 60mg pred and infusions of Cyclophosphamide. After approx 8 months of this I was then treated with Mycophenolate as a steroid reducing agent. I slowly came off them and only now need to go back on them as I get a lot of chest infections but inbetween I dont need them at all, just the immunosupressant. There is definitely life after steroids but I relied on the neurologist for a reduction regime and in my case coming off them has been great and worked for me. While I was on them I took the weekly alendronic acid and adcal for bone density and a recent bone scan was fine thankfully. I am still taking adcal which I now have in tablet form instead of the awful chalky tasting tablets.

    Best wishes

    Sarah

  • Very basically, with me anyway, experience-years of it.

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