Update: Connective Tissue Disease Clinic appointment last week

[MAT51]

This is a 2 part (long!) update about my rheum appointment at CTD clinic. Considering that seronegative Sjögren's isn't on the map much in UK it - went very much as I expected. In some ways it was very good. I saw my rheumy for second time rather than the vascular doctor and was again impressed by her sharp intellect and approachable manner. I took my husband with me and it was his first encounter with her. Afterwards he declared that he could hear her brain whirring and clicking through all the information in front of her, trying to piece together the jigsaw. Unfortunately she admitted that this just isn't possible because research into my form of seronegative Sjögren's is quite thin in the ground. She didn't say this but it was very clear that she hasn't got time to research my neuro problems herself.

I could have pointed her to the latest thinking of big gun doctors from John Hopkins and Mayo - but I felt this wouldn't help my situation here in Scotland much. Anyway she's perfectly capable of researching seronegative Sjögren's herself if felt inclined. The main issue is that, having previously been misdiagnosed with RA, I've tried and failed to tolerate almost all of the disease modifying drugs or nerve pain ones.

My husband said that he could see her checking my many raised bloods and looked up research on autonomic dysfunction in Sjögren's - but just commented "goodness that's a very lengthy piece of research?!" She looked a bit overwhelmed and kept rubbing her face and hair rather frantically throughout my 30 minute appointment, actually inviting me to tell her what I have learnt and what I want her to do. I do like and respect this rheumatologist a lot but all I want is for her to read and learn up for me. I guess that's too much to expect of a very overworked NHS consultant these days though.

She had just read my 3 emails and studied photos of the spots on my feet - which she showed us in the screen, confirming as she did that this was some kind of small vessel Vasculitis. But, like the dermatolgist yesterday, she agreed that, since I had been my third course of antibiotics for supposed UTI, at the time the spots arrived, this could well be the cause for me. Regardless - she doesn't believe the +++ blood in my pee is UTI related. She took very seriously my pelvic/ groin pain and flank pain and thought hard, lots of checks to see what tests I've had etc - quizzed me about drug reactions and asked me about steroids and about Pregabalin/ Lyrica and other nerve pain meds.

[MAT51]

Part 2: She was shocked that no one has yet done a tilt test or skin biopsies for Small Fibre Neuropathy (SFN). She immediately referred me to cardiology for a tilt table test and told me she suspects many of my problems relate to autonomic dysfunction. She confirmed that Sjögren's is probably still the main culprit but thinks my suspicion that I have an underlying small vessel Vasculitis as well is quite likely. She isn't the doctor who orders skin biopsies so prefers to let neurology decide on whether this is necessary. She confirmed that my ANA is significantly positive, rechecked my very positive lip biopsy results and asked if I have trouble swallowing. Of course!

We spoke of Mycophenolate (Cellcept/ MMF) and all the others I've tried and my concern that my systemic inflammation is out of control. She agreed but explained that she feels worried that, with my history of severe chemical - Cellcept is only worth retrying if we feel the benefits outweigh the risks. I said I share her concerns but am not sure what side effects she is attributing to my 15 months on it? She pointed to my visibly twitching and vibrating arms and said these resting tremors are her main concern as they only started when I was on max dose of MMF. This is a known side effect apparently and the fact that they have continued since I've been off it doesn't reassure her. The effects can be permanent. However my new neurologist said yesterday that these tremors aren't drug related but are almost certainly due to SS fatigue.

So we agreed that it's best to hang fire and see what my compliments, immunoglobulins and ANA are doing now since I've been off it. She understands that the inflammation is rampant but we don't want to make my neuro symptoms worse and presently there are no Pharma treatments for autonomic neuropathy. She also understands that I now reject drugs like Lyrica because of severe palpitations and because they cause me even worse fatigue and Sicca - but feels my pain is going unchecked which is of concern to her. She suggested I have an EMG of my face/ head to check for Trigeminal Neuralgia. She can't arrange a follow up until end of January but will discuss my case with the multidisciplimary Rheumatology team over the coming few weeks and will write to me and let me know what is decided - particularly after the tilt table test. I asked that my unpleasant former neuro not be consulted please and she said of course not - this is for Rheumatology alone to decide as I have a confirmed rheumatic disease. My husband got the strong impression that she is very displeased with nasty neuro re lack of tilt table or SFN testing.

I told her my compliments are usually high rather than low and ANA was only 1:160 last year so maybe other non autoimmune things at work? but she said actually the Mycophenolate probably had brought these and IgG down. I also conveyed that I think my PV of 1.97-2.00 isn't due to Sjögren's viscocity alone and she agreed. She also confirmed that my significant degenerative disc disease is only complicating things further.

I have been flaring so wasn't as focussed or demanding of my rheumy as I'd hoped to be. I just told her I couldn't keep working it all out myself and need her to take charge. After all it's what she's paid to do and it's not my fault I have a rare and under researched kind of Sjögren's/ CTD. At the end of the day I'm significantly ANA positive as she told me, I have high levels of inflammation and I feel crap much of the time. I try my best to deal with sensory ataxia, have accessed Rheumatology physio and OT for myself and they help a lot - but they admit that they aren't giving me the mobility or able to resolve my systemic fatigue without systemic treatment to help control my inflammation levels.

As an add on to this post - a week ago I lost my balance and fell and hit the toilet bowl directly on my left side rib cage so now nursing a fractured rib and on tons of heavy duty pain meds. What a difference it makes to doctors and family when I show them this massive dark bruise and swelling/ hematoma size of tennis ball etched onto my side and back! Suddenly I'm eliciting concern from A&E/ ER doctors and sympathy from family and friends. And yet the approximately three to six week recovery period makes the hellish stabbing pain when I move or breathe far more bearable to me than my small fibre neuropathy is!

[irish]

Well, sounds like you had a good visit with a few things addressed. That is all we can hope for when we have autoimmune and Sjogrens. Sjogrens is much more complicated and afflicts us so much more than people think.

Also, you have proven the one thing we all know. If others can see an owie they suddenly listen to us, pay attention and extend their sympathies. I don't recommend falling like you did to get attention though. It takes a lot out of us due to the pain and stress of the event. Will be interested to hear what the tilt table shows. I had one many years prior to diagnosis without any gleaning and significant information. Wonder what it would show now.....but I wouldn't do it. I am what I am.

Have a good weekend and good luck with your further testing. Irish

[MAT51]

Yes thanks I think she's good - but I also know how the NHS works in these financially very straightened times. She has no time to research the neurological manifestations of Sjögren's in the depth that I have as person experiencing them - yet it's she who needs to in order to help me and other patients. The tilt table and head EMG are red herrings for me I'm fairly sure. It's all just fire fighting and unwitting delaying tactics I feel

Hells bells I wouldn't recommend breaking a rib to prove any point in the world Irish - it's agony! I keep making involuntary noises similar to a cross between a noisy tennis player and a woman in the final stages of labour!

[aussie mum]

How sad is it that Specialists don't have the time,  or inclination, to conduct further research that may help their patients. Surely they should make time to increase their knowledge. Things change all the time!

My daughter No 1 (DD1) is my Rheumy's current dilemma. She's presenting with high ESR & CRP  but nothing else auto immune specific.  She's suffering severe fatigue, muscle & joint pain,  brain fog.

(I have sero-negative Ankylosing Spondylitis, DD2 has sero-positive Sjogrens)

In an attempt to find something that helps,  she's been trialling the different anti inflammatory medications and has just started on Plaquenil.

The Rheumy is taking DD1's medical records to an upcoming conference, hopefully the brains of many Rheumatologists might finally get some answers.
 
Hope you find answers too