Vasculitis (Churg-Strauss Syndrome) - my story so far .. 11 weeks on

[JontyW]

I thought that I would share my recent, and still ongoing, experience of vasculitis which has made me realise just how fortunate I have been in getting a speedy diagnosis and first class treatment.

(BTW: I have a degree in Physics so am used to doing tests and monitoring trends etc, so this write up is quite detailed based on my high level of observations and daily spreadsheet recording).

First a bit about my medical background which has an extensive history of other autoimmune diseases as listed below. Of these only Sarcoidosis is not definitely an autoimmune disease but involves inflammation of any part of the body (in my case it was lymph glands). So I have quite an extensive history and in addition have had other allergies over many years, including adult onset asthma, hay fever and also nasal polyps.

- Diabetes Type 1 (age 27) - 43 yrs insulin dependent & no complications

- Asthma (age 35)

- Sarcoidosis (age 40 ... but no longer active)

- Addison's Disease (age 66 ... take 25mg Hydrocortisone for this))

- Vasculitis (age 70 - Apr 2013)

I also have Atrial Fibrillation for which I take Warfarin. So my heartbeat is fast (140 beats/min) and irregular.

So how and when did this latest condition start to develop?

My asthma has been well controlled for many years, with the latest range of inhalers and so I've never had a major problem with breathlessness even after heavy exertion doing garden projects and I regularly go caravanning with coastal path and hill walking. However in the winters of 2008 and 2009 I ended up in hospital for 2 and 3 weeks respectively with severe chest infections rapidly developing into pneumonia. However after the normal inpatient oxygen, steroid and antibiotics treatment, I left hospital feeling back to normal with no breathing restrictions and no need for follow up appointments.

Then next two winters (2010 and 2011) I had no problems, so I was starting to feel optimistic (how wrong was I !). Then this winter, at the end of Nov I noticed that I was starting to become breathless when walking briskly upstairs or other exertion. I also noticed that my left ankle and foot has swollen, so I had to raise it to reduce the swelling. Also noticed puffiness around my eyes on waking. My GP prescribed antibiotics, but still no better, so had two more courses of antibiotics .. still no better. This was very unusual based on my past experience.

By mid-Feb breathlessness getting worse so got myself checked into local hospital respiratory ward (Frimley Park Hospital, Camberley, Surrey .. excellent hospital which was runner up in the Dr Forster "NHS Hospital of the Year 2012" awards recently ... frimleypark.nhs.uk/ ) for five days where the usual course of oxygen, steroids and antibiotics got my SAT level (blood oxygen saturation) back up to 94% which is about my max these days (normal is 95 to 100%).

Then .. five weeks later, coming up to Easter, all sorts of strange and unusual symptoms started to occur:-

- lesions appeared on my hands and fingers

- breathlessness started to get much worse

- thigh muscles started to be really painful on climbing stairs etc

- a rash appeared on my lower left leg

- sore throat and sneezing as though I had a heavy cold

- severe nasal crusting

- numbness on side of right thumb

This was really strange since, although I am 70 years old, I am very active and generally fit, and I had never experienced so many unusual symptoms all at the same time .. I was getting worried

By Easter Monday (1st Apr), with no local surgery open, I decided to visit A&E for advice since things were rapidly getting worse, particularly the spread of skin legions which now appeared on my feet and elbows also. All they could offer was another course of antibiotics and hope that would work .. it didn't of course! Also noticed my blood sugars, normally well controlled by four insulin injections per day, started to go high. This is often a sign of uncontrolled infection and all I can do is inject larger doses to bring the blood sugar down into control.

Over the next 3 weeks, despite yet more antibiotics from my GP, nothing improved, but got worse .....

The key date in this story is Mon 22nd April, since on that day I visited my GP, yet again, when she took some basic blood tests and prescribed different antibiotics. Later that day was also my follow up appointment to see the chest consultant following my hospital stay in mid-Feb, and my GP quite rightly said I should mention these symptoms to him in case they were related .. they were! The discharge notes (I get a copy of all letters sent by consultants to my GP on the conclusions from the appointments) contained the following comment ...

"Bloods showed raised inflammatory markers. Interestingly, bloods also showed a raised eosinophil count of 1.4 - ? cause" .. HR HT Scan needed.. follow up appt in respiratory outpatients made (22nd Apr)"

So .. when I had my appointment with the chest consultant later that day, I immediately mentioned about the lesions and other symptoms I was experiencing. He gave me an examination, and then said .. "This is an allergic type reaction and I'm almost certain I know what this is .. it is an autoimmune disease called Vasculitis".

I of course had never heard of this condition before, but realised just how lucky I was to have this wide range of symptoms showing on the exact day of my appointment with an specialist consultant. So it was only 3 weeks since the condition had really started to get active (early April), and I now had a diagnosis ... wow! Judging from some of the stories on this web site, that would appear to be a very quick time to get a definitive diagnosis of a disease which is rather rare and hard to track down for quite some time. So hopefully not too much damage had already been done to any organs affected, which in my case appeared to be mainly my lungs .. how lucky was I then!

The chest consultant immediately prescribed 25mg Prednisolone plus a full blood test. He also got me an appointment 4 days later to see the Rheumatology consultant, who then prescribed Mycophenolate (CellCept) and strongly suggested it was Churg-Strauss Syndrome. He then got me an appointment one day later with the Dermatology consultant to inspect the lesions, and then a biopsy from my left calf was arranged to confirm vasculitis rather than the possible autoimmune skin disease called bullous .. it was confirmed as vasculitis. So in just one week I had seen THREE specialist consultants, a defined diagnosis, had multiple blood tests, medication prescribed and already the symptoms were starting to subside.

Regarding timescales, it was 10 weeks ago that the hand lesions started to appear, and the diagnosis was 7 weeks ago yesterday, so I'm now entering the 11th week of vasculitis and 8th week of treatment.

Medication Timeline & Symptoms

wk 1 (22 Apr) - diagnosis: SAT 89% (low); Pulse 140!!. Prescribed Prednisolone 25mg.

- bloods showed high inflammatory marker eosinophil count of 5.2

- coughing thick green phlegm with blood

- by end of week: breathing improving & lesions drying up.

wk 2 (29 Apr) - Pred reduced to 25mg + added Mycophenolate 500mg 2/day.

wk 3 (6 May) - Pred up to 30mg + increased Mycophenolate 1000mg 2/day

- bloods showed inflammatory marker eosinophil now normal at 0.1

wk 4 (13 May) - Pred down to 25mg

wk 5 (20 May) - Pred down to 15mg

wk 6 (27 May) - Furosemide 20mg added to treat bad leg/foot retained fluid (oedema)

- Inflammation marker: CRP 101 (up)

wk 7 (3 Jun) - Pred down to 10mg. Inflammation marker: CRP 48 (down)

wk 8 (10 Jun) -

Current status:

- breathing much improved, but not back to what I would 'ideally' like: SAT 94 - 95% (up); Pulse 110 (down from 140). Phlegm now clear and white

- lesions have long since gone and no new ones

- slight rash on lower left leg

- biopsy sites (x2) still healing & dressing changed every 3 days at medical centre

- leg/foot swelling: still some on left leg/foot but totally gone on right leg (why is only one leg affected?)

- leg muscle pains: totally gone

Comments

Like most others on this website I had never heard of this autoimmune disease before, and consider myself very lucky in my experience of it so far, because:-

- diagnosis was very quick

- just by chance my chest consultant's follow up appt coincided with the eruption of symptoms

- my GP admitted that otherwise it would have taken her sometime to get a consultant (dermitology) appointment to further investigate, with possible organ damage due to delayed diagnosis. She had only experienced vasculitis very rarely

- Frimley Park Hospital are quite rightly rated amongst the best hospitals in the UK. The Dr Forster rating is based on inpatient mortality rates and efficiency of operations and financial control, but their OPD and consultants are also really good. The inpatient food is also excellent Well done NHS

- Appointments with three specialist consultants within 6 days is really impressive

- My regular consultant (rheumatology) sees me every Wed at the start or end of his clinic.

He also emails me regularly to update on latest blood test results and I email him on Friday afternoon on my current status.

Any questions or comments?

I have related my experience of vasculitis in some detail here so as to provide any newly diagnosed patients on what possibly to expect through their early journey in this disease. I realise that this disease manifests itself in a very wide range of severity and symptoms, and what I've related here is just my own experience of a not-too-severe case of Churg-Strauss Syndrome.

If you have any questions or comments .. please feel free to add them.

JontyW

[hamble99b Volunteer]

thankyou for posting Jonty, have glanced through it, but will have a good read tomorrow.

regards,

sandra.

[HiveMind]

Hello Jonty,

I too have CSS; diagnosed in 2008. I wasn't diagnosed early and my heart & lungs were affected. Some of your symptoms were similar to mine especially the asthma, joint pains etc. I was also coughing up blood tinged almost solid lung plugs the size of a cigarette butt (vile I know but might be of relevance to someone). I felt as bad as it's possible to feel and still be breathing. (I'd been to my GP constantly throughout the previous year insisting I was unwell).

In reality I was extremely poorly and my heart was failing fast. My saving grace was my (previously) healthy heart and the brilliance of a cardiac consultant who diagnosed CSS when I was eventually admitted to ER. It's thanks to him I got to see my 2nd granddaughter born 4 months later. I will be forever grateful to him.

Obviously this is a brief overview of what my story has been but I hope you feel less "isolated" as a rare Vasculitic (CSS) sufferer!

Stay well.

[JontyW in reply to HiveMind]

Hello BerkshireBird,

Thank you for replying and giving an overview of your CSS experience.

It is interesting to hear about what others have gone through from reading many of the blogs and I'm amazed at the high level of variability in terms of symptoms, different treatment medications, and how it has affected their quality of life. It is still early days for me to comprehend how it will affect my future quality of life, but I'm hopeful that, apart from possible restricted breathlessness, I won't experience too many other problems .. but you just never know do you. We were planning on taking our caravan to Luxembourg, Switzerland and France for three weeks in early July, but have had to cancel due to the uncertainty and the need to stay in the UK just in case.

Yesterday I had my weekly consultant appointment and he is happy with progress so far, so no change in medication and next appointment in three weeks, rather than weekly. I also found out that the CellCept will continue for a total of six months and I'll remain on 10mg steroids in the meanwhile. One issue that I have to struggle with, being an insulin dependent Type 1 diabetic, is sudden increases in blood sugar for no apparent reason. This has happened both yesterday and today after a week when they were running at normal levels. So I have to do extra injections of quick acting insulin to bring the level down. Clearly something is going on inside which is causing this increase, and I awoke on both days not feeling that good with a sore throat and headache both of which clear during the day. But there is still no nasty looking phlegm and my SAT is steady at 94%, so I'm pretty sure that I don't have an infection.

BTW: I see that you live near Thatcham in West Berkshire. That is an area I'm familiar with since our daughter owns a house just off Floral Way in Thatcham, but this is rented out and she now lives in the village of Compton on the edge of the Berkshire Downs. So I guess you attend the Royal Berkshire Hospital in Reading for your ongoing treatment?

JontyW

[Church-mouse]

Hello Jonty,

I have recently been diagnosed with CSS at 51, after two years of seemly unconnected illnesses, bumps and lung problems.

I have been off since April 23rd when I was taken ill at work. I was taken by ambulance to my local hospital where I spent 2 weeks being tested and analysed before a consultant recalled a similar case locally. I will be signed off for the longer term after that. My chemotherapy is with IV with cyclophosphamide continues with the last cycle on 22nd August. I've already just started to cut the steroids down from 40 to 35 mg per day.

It looks like I won’t be returning to work in the foreseeable future. I am a lecturer in Childcare and Education at a local college, specialising in training students to support children with additional learning needs. I am missing the students, staff and children. Suddenly stopping this busy part of my life has been quite a shock! I live in Somerset, and I have spent a bit of time trying to find another person locally with the same condition. I have already made a couple of contacts through this website. I also belong to a Facebook group called 'Churg Strauss Friends' which is very interesting if you are looking at an international perspective. You can request to join. We are putting together a spreadsheet of all the members details to get a database setup - an approach that I think would interest you.

I think your approach is really positive. One of the things that has struck me is the need to take active control of the treatments and it is amazing how much time is spent organising appointments, evidence and managing your drug routines. It almost as if I wouldn't have anytime to go to work anyway!

Thank you for sharing your adventures with us.

[JontyW in reply to Church-mouse]

Hello Church-mouse,

Firstly I like your clever user name (Churg-Strauss --> Church-mouse) .. nice

Thanks for mentioning about the Facebook group 'Churg Strauss Friends' and I have already joined it and introduced myself ... I am the third 'Jon' to join the group so Jon III. And I suspect that you maybe one of the other Jons?

Regarding the spreadsheet, who is managing this and how are you collecting the required information .. i would indeed be interested in this.

[Church-mouse]

My children came up with the name! A member of the group Suzanne Comstock Schulte is collating the spreadsheet, and it is interesting because it has a worldwide spread (with an emphasis on the US as that is where the founder member is based.) A draft is available if you look at the tabs at the top of the group page marked 'Files' and it is called 'CSS Stats Draft pdf'. I'm not sure how to attach a link or document to this! I know she is working to update this list, and I've sent her my basic information. I think it would be useful for someone to look at it at some stage who was able to do some valid statistical work on it - I'm too rusty.

[dskizs]

Thank you for sharing...I just joined and have sent a blog out somewhere to try to settle my nerves down. I am in the process of finding out just what is wrong. My blog is under DSKIZS and would love for you to read it and respond...I am a little scared! Thanks again you give me some hope and have calm my nerves.

[Hidden]

Hi I would like some Advice I saw a CSS ( Private) Specalist who did No Tests, did Not look or want to know my Medical History. I had a Blood a Test at my Gp. Who Refered me to this Specalist.

I was Refered because my Birth Mum in NZ has just been Diagnosed. Her Specalist said for any family member with Asthma.Sinus and Heart Problems to have test. Both me and my Birth Mum have exactly same symptoms...

But I feel I haven't been correctly tested. Just fobbed off

[JontyW]

Hello CassieBee,

Please provide information on the following .....

1) Where do you live? If in the UK then you need to be referred ideally to a NHS consultant (usually a rheumatologist) who specialises in vascultis and has previous knowledge of CSS.

2) What are your symptoms and how long have you had them? Provide as much detail as possible please.

3) Re ... " I had a Blood a Test at my Gp."

------ What were the results of this .. in particular % Eosinophils (that is Eosinophils value as a % of White Cell Count? If % is > 10% then that is a strong indicator of CSS, so really important to get that measurement.

------ What was the value of CRP (C-reactive protein) which is measure of inflammation ... target level is < 10 mg/l?

------ You need to contact your GP to get this data. If these metrics were not tested, then insist on another test to include them.

4) For diagnosis of CSS see these Six Criteria, and if you have at least 4 then highly likely it is CSS ...

mayoclinic.org/diseases-con...

[Susie_b]

Such interesting reading i am awaiting skin biopsy results so many pains feet, left arm, hands both legs and swelling knees to toes . Feel old at 52! Oh heck...... not good. Feel like im having a long flare up of this illness. Clinic letters mention CSS....... recent ct lung shows likely vasculitis to have repeat. My question is do i continue to push myself and get to work or do i rest and give into fatigue and awit results due on 12 th Jan. Any advise on self care greatly welcomed. I have nasal polyps, chest wheeze rash coming and going over last 5 mnths to lower legs and have had rash and weird chest symptoms over 3 yrs plus but didnt get anywhere. Also blood protein and leucocytes urine testing, low calciu

m and low folate and mild easonophils.