My 10 year old and vasculitis

Hi it's been a while since I posted on here. My 10 year old daughter was diagnosed with cerebral vasculitis earlier this year and we are very lucky because it's only in her brain at the moment they classified it as non progressive (at the moment) so I'm assuming it can change. We are under a rheumatologist who wants to do a skin biopsy to do with her mottling which she's always had. Just curious to know if anyone else on here has had this done and what they may be looking for. She's not on any treatment yet for her vasculitis I'm assuming it's because of her age and the rarity of her having this form of vasculitis.

Thankyou Shelley46

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  • If not controlled cerebral vasculitis only in the brain can be progressive, causing further brain damage and increasingly neurological problems over time. Perhaps the medics feel your daughter's has burned out? But I'm a bit concerned at her getting no treatment yet. Not even steroids? There would be concerns at her age re using a more aggressive chemotherapy treatment like Cyclophosphamide, but there are other milder long-term immunosuppression drugs that can be used to reduce the amount of steroids needed, like Mycophenolate Mofetil, Azathioprine and Methotrexate. Ultimately steroids would be the quickest way of reducing any active vasculitis (inflammation in the blood vessels) in the brain, but it's a bit of a sticking plaster, and has long-term consequences. Hence medics using steroid-sparing drugs like the ones I've named.

    I fell ill with cerebral vasculitis aged 22 in 1994. My vasculitis is confined to my brain, but causes a wide range of neurological problems, that have increased over time. But I believe that if I hadn't received good treatment from 1997 onwards (when I was finally diagnosed - it can be a very hard disease to diagnose) I wouldn't be here now.

    My story is online at

    vasculitis.org.uk/living-wi...

    Cerebral vasculitis, or as it is also known central nervous system vasculitis, is an incredibly rare disease. If not part of a wider systemic disease or following on from something like lupus then its incidence is about 1 case per million people of population. Very very rare.

    If you want to send me a PM here please feel free to do so. Would also be happy to chat by email. But remember I am not a medic. Just a patient with this disease.

    Best wishes.

  • Thankyou Vivdunstan, it's really hard because it's her and not me, she had a massive stroke last year aged 9 and it took them 9 months to diagnose the cause because it didn't start to show up until her 6 month MRI and her neurologists didn't know what to make of it as she had none of the other vasculitis symptoms her colleague at great Ormond Street diagnosed her. Just don't know what to expect in the future, when she gets really stressed she has a flare but our local hospital which is a big one not just a&e but a major hospital don't know how to treat her as you can imagine school is one of the biggest triggers. Any help or advice would be really appreciated. Thanks Shelley46

  • Hello Shelly46

    Sorry to hear about your daughter it is very sad. I am not an expert but I have lived with vasculitis for 10 years and it has progressed to the heart. In my considered opinion your daughter must be on a base dose of steroids to suppress the immune system unless your consultants have a definite reason not to do so. Vasculitis is a sinister diease and needs monitoring and control through drugs all the time and for life. I hope I haven't scared you but take this as a cautionary tale and I hope your daughter improves soon.

    All the best

  • It's a bit worrying that the MRI didn't show up the cause for 6 months. That doesn't fit with something that isn't progressive. I really do hope your neurologists are on the ball on this. It can be very difficult to know if cerebral vasculitis is still active. It commonly doesn't show up in blood tests - unlike vasculitis elsewhere in the body - and other tests are often normal too. Symptoms can be the best guide, but with a 10 year old I'd have thought they could be very difficult to follow.

    Again I am concerned that your daughter is not getting any treatment for this yet. This disease process can be very aggressive, and can kill, very suddenly. It is a bit like having a time bomb in your brain. Even if it does burn out it can come back. This is why vasculitis patients tend to be on a maintenance cocktail of immunosuppression and often steroids for life. All to try to keep it from going out of control again. Which can happen even despite those drugs.

    It must be very difficult for your daughter. I suppose the best thing is to try to make things as normal for her as possible, even fitting around the medical appointments. And maybe explain to her - within certain limits - what is going on, so she can understand. It can be a hard enough diagnosis for an adult to come to terms with, let alone a 10 year old.

  • Also stress is a common trigger for vasculitis flares. Stress probably caused my cerebral vasculitis disease to start in 1994, and a significant number of vasculitis patients on this board have reported having major stress situations before the disease developed. Not a 100% proven link, but highly suggestive. It's why many of us now take active steps to avoid stress as much as possible. Which can be difficult.

  • Thankyou Vivdunstan for your answers we should be back to see her neurologists in a couple of weeks so hopefully will have more answers and plan of action for next steps.

  • Thankyou Rowmarsh she is kept a very close eye by her neurologists who sees her every 6/8 weeks and she has scans every 12 weeks they need to do a skin biopsy to diagnose another condition so think their waiting until they've done this but not sure. Hopefully we'll get sorted soon.

    Thanks Shelley46

  • nhs.uk/conditions/Hughes-sy...

    Hi Shelley46,

    This may be a bit " out there " but I believe that Anti phospholipid syndrome can cause a particular type of skin mottling ( the medical term escapes me at the moment, brain fog! ). I don't know if the above link will help and whether your daughter has ever had it ruled out?

    Best wishes

    Keyes

  • Hi Keyes. Really worried now. But probably just the lack of sleep. I'll keep you posted. x

  • I think maybe you meant Livedo Riticularis Keyes? I posted a photo of my leg with this rash that I developed 6 months ago and still have. I was diagnosed with Primary Central Nervous System 10 years ago but with this relapse and no hard evidence of active vasculitis in my brain, I was tested for APS or Hughes which can cause cerebral vasculitis. I think it would also be a good idea to rule out. It s possible as you said Shelley that they are holding off treatment until the skin biopsy but I think they would do that if they were still looking for proof of Cerebral Vasculitis. Did your daughter's MRI, or angiogram show lesions typical of vasculitis? Did she have a lumbar puncture? cerebral vasculitis can be treated successfully but it would really help to have experienced doctors involved in her care. I know there is a doctor and a clinic specializing in CV in children at Sick Kids Hospital in Toronto. You could check their website for further info. My thoughts are with you at this very difficult time for you and your daughter and all of your family.

  • Thankyou Kat 13, I'm not sure of the name of the condition their looking for, but yes she's had a lumber puncture and blood tests which show she is ANCA positive and yes the angioplasty CT scan showed the vasculitis in her brain but there was nothing in her other major organs not sure as to wether they are hoping it burns itself out or not hence them doing another MRI sooner than normal. Yes she has experienced doctors and consultants looking after her.

  • Thanks Kat13, that was the rash I was referring to!

    I think that knowledge of APS is still in its infancy as its a relatively " new " disease and can mimic quite a few other diseases.

  • Hi Shelley, This must be a very worrying time for you and those in this group don't want to increase your anxiety. But many of these diseases are progressive if untreated. We are all rather concerned at at the apparent lack of action and urgency. You have not said where she is being treated. It is essential that she should be seen by someone - or preferably a team - who take a holistic view of her case, not just treating it as a neurological problem.

    You did mention Great Ormond Street. Dr Paul Brogan at GOSH is a leading world expert on paediatric vasculitis and other allied rare conditions. If he has NOT been consulted and involved in your daughter's case, then he should be. These diseases are very rare in children and generally very variable, so you do need someone with knowledge of and experience in diagnosing and treating them.

    Very best wishes - John

  • Thankyou John she's under the LGI in leeds her neurological consultant is Karen Physden and she's working with her colleague at GOSH but I don't know that person's name. Yes everything is really stressful at the moment, she keeps getting mild headaches and the mottling on her skin, when she's really stressed her speach is slurred, she drowls and her eyes constantly blink these symptoms are happening more and more now she's back at school our local hospital which is HRI in Hull have no idea what to do when I take her to a&e they have to ring leeds to get help which the last time took 4 hrs just to talk to someone and for them to say just observe her for a couple of hours it's so frustrating because I know how serious her condition is. No-one seems to want to give us answers or a time line on treatment or what treatment they are willing to give. Her neurologists Karen is brilliant but we are 90 miles away from her. Any advice or help positive or negative feedback is appreciated.

    Thanks Shelley46

  • I think vasculitis patients in general don't find A&E to be helpful, and so simply don't waste their time going there. I've never bothered even when I get recurring TIAs. You really need to get the most support from your consultant. I am concerned that they are so far away. Is there not a good local rheumatologist who might be a better option for looking after your daughter's case? It really is your consultant who needs to organise treatment, and we are very concerned here to read that nothing yet has been arranged. This is not a disease that should be under treated. I am also concerned about how long it took to diagnose given a stroke had happened, and do wonder if your daughter is getting the best care.

  • Thanks Vivdunstan.

    We haven't been told of any specialist nearer to us. She was on warferin after her stroke when they diagnosed her with vasculitis they put her on aspirin that's when things began to get worse ie headache, blotchyness, speach slurring and the drowling. The only thing we were told for definite by the rheumatologist was that the warferin had kept the symptoms at bay. The funny thing is she's always had the blotchyness since she was born and we were told it was because she was brought early.

    Shelley

  • Ask John Mills for advice about other local vasculitis specialists. Rheumatologists are the most common consultants to deal with vasculitis, and your neurologist may not know of those outside their speciality. To be honest I've heard a lot of bad tales of cerebral/CNS vasculitis patients and neurologists, including my own experiences. Generally I now advise people that a more general vasculitis specialist, like a rheumatologist, is a better bet. And they are much more widespread. They typically have experience of dealing with vasculitis in many parts of the body, and in the brain too, including isolated cerebral/CNS vasculitis patients. And this means they often have really good knowledge of the different treatment options, sometimes better than a neurologist does.

    Main thing is though you need to have a frank chat with the neurologist when you next see her, re treatment options and what the next steps are. Good luck!

  • Thankyou Vivdunstan

    I'm ringing her neurologists on Wednesday to have a chat about Becky my daughter and I'm going to see if they'll bring her appointment forward for us so we can start to get things sorted before we go away in November.

  • Hi Shelley, I don't know Karen - but then I only know a few neurologists. Can you not talk to her "man-man"?

    I am not a doctor. I don't usually think it's my place to interfere, but in this case nobody seems to be doing or saying anything.

    As positive action, can you describe all her symptoms, in order of when they started. Everything, regardless of how minor or irrelevant they might seem. Can you describe the rash or skin pigmentation esp when it appeared & where it occurs on the body & how big. Can you let me know your surname or your daughter's first name. Either by private message or email john.mills@vasculitis.org.uk I can then contact Paul B at GOSH to see if he knows about this case & if not he might be able to comment.

    BW - John

  • I think it is so great on this site to have the support of both those who have first hand experience with the disease and also those who have good knowledge of

    and the ability to contact experienced experts in our rare diseases. I am so hopeful about what a difference this can make in someone's life. I hope Shelly you will let us know how your daughter makes out with her doctors and treatment.

  • Hi John thankyou so much for this my daughters name is Rebecca Michelle liddle dob is 09/02\2005. Xx

  • Hi all I rang her consultant last week spoke to her secretary and was told she was on the list for September I laughed saying you do realise it's now nearly October, was told October clinic fully booked earliest appointment was 26/11, explained my concerns she said she let her consultant know sent me an appointment letter saying definitely 26/11. Yesterday I got a phone call saying they'd had 2 cancellation for 6/10 did I want one son now going next week with my daughter. Couldn't believe they'd had 2 cancellation it's so unusual for that to happen. So hopefully we'll have some sort of action plan in place by the end of next week.xx

  • Hi all just to give you an update on my daughters cns, had a letter from gosh stating that they think she has got non progressive cns and it's just in her brain they've looked at the last scan an said that they don't think it's gotten any worse. So just keeping her on aspirin for now it took nearly 5 months to get a reply we go for another scan 14/1. We've spoken to her rehab team in December and was told that the reason why we've not been given medication and a positive diagnosis is because they're unsure as to what's going on and don't really no what her condition is, there's not enough information or research out there on children with rare types of vasculitis especially with no history of any symptoms so no-one seems to want to say exactly what's wrong other than to say they think it's non progressive cns as it's only effecting her brain

    I'd just like to say a big thankyou to John and Vivdunstan and all of you for your support and help.

    We are hoping it won't take as long to get the results of the next scan although I do appreciate how busy gosh are.

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