Update: Cellcept and the CTD clinic - plus oral medicine - both today

[MAT51]

I wrote this as as a post on a Lupus forum earlier but I thought some of you with seronegative  Sjögren's (ANA and lip biopsy positive) might appreciate the update too. To fill you in on other news - I had my first ENT appointment ten days ago with a fabulous ENT surgeon. He ruled out throat cancer (his specialist area) and said I have a deviated septum but this is common. The nose bleeds he blamed on dryness and suggested I get a humidifier and that Sjögren's dryness was also responsible for my sore throat and probably also the awful taste taste. He wasn't sure about the tinnitus - which seems to get louder daily! I have a new white noise aid device but to be honest I don't get much benefit from it. He referred me for a CT scan of sinuses which I had on Friday - but doubted anything would show.

Meanwhile I'm hoping to undertake a practice led PhD in my local art college - if I can get funding. It's about invisibility and autoimmunity. He has offered to be one of my supervisors as he alreadr works with design students on new technologies for ENT and service design.

[MAT51]

I'm having a bit of a lucky run with doctors just now. Although, for the sake of famous last word, I'm touching the wooden arm of my seat in the dental hospital where I'm waiting to see my oral consultant for annual review.

So this morning I saw the vascular doctor who specialises in scleroderma and Vasculitis - rather than my new rheum. I was a bit startled initially but when he said his name, after introducing the two young male medical students, I apparently (hubby observed) went pink and exclaimed "oh I know about you! I've seen you on a YouTube video about nailfold capilliaries!". How embarrassing - I do often make a twat of myself it seems.

So after that it went pretty well I think. I had my three questions and went straight in on the GI/ dysmotility issue that is ruling my life presently. I told him about the GP giving my Bisocodyl and the fact that I'm waking even more often with severe stomach pain and am glued to the toilet most mornings. I explained that his rheumy colleague had called it dysmotility and GP had then described it as neurogenic. Neither I or GPs have a clue if the raise in Mycophenolate (Cellcept/ MMF) to 3g is making it worse though - or know for sure that it's neurogenic even.

He explained that it's very hard, if not impossible for them to know if MMF (Cellcept) is working. If I was taking MMF for kidney disease or Lupus Nephritus then it would be easy to assess. I would not be taking it for RA but the effectiveness of Methotrexate or Sulfasalazine etc are easy enough to assess for RA. But for Sjögren's it's extremely hard for them to know, impossible by bloods alone. So he suggests that he refer me to a gastroenterologist to decide whether my problems are neurogenic or whether I have IBS-c. I will be seen again by the CTD clinic once the gastro has made a decision on this. So hopefully in 3 or 4 months.

I asked my question about possible renal involvement  and he said that my urine sample was entirely free of blood and protein. He also observed that my high BP has now settled down. I asked him if this could be due to the higher dose of MMF and he said the only way of knowing would be for me to come right off it for a few months and see of blood and protein returned in my pee - and his view was that this would be unwise. He thinks that, as I'm on it principally for the neurological symptoms of Sjögren's, it's better that I assume that it's controlling this to an extent at least. Less disequilibrium has to be good, bearing in mind that some small nerve nerve damage has already occurred and can't be reversed.

So I asked about the raised total protein/ Creatinine, CRP and PV and where exactly my inflammation is? I told him that I've had a lot of mobility problems due, I'm assuming, to the severe arthritis in my lower back, neck and hips. I'm also really struggling with facial tingle, jaw/ear ache and have been told this is TMJ by my oral consultant. Plus fatigue has been out of this world!

He in turn explained that high IgG levels are causing my PV to always be high because my blood is more dense due to excessive antibodies. This is specific to Sjögren's. He said that this in turn means my body and brain are suffering from Autoimmune fatigue because my body is constantly dealing with excess inflammation throughout the system. This is the best explanation I've ever been given for rheumatic fatigue - add th issues with my neuro pathways in and I'm thinking it's a wonder if got any functioning brain cells left to be considering undertaking a PhD with?!

[MAT51]

I asked if I might have an IM steroid jab but he said no. He said the problem with steroids for me is that my stomach is already a problem and I'm at quite a high risk of osteoporosis so they wouldn't want to increase that risk by giving me steroids - which I would then want at closer intervals as they wear off faster and faster.

He did also mention in the same breath that this inflammation induced fatigue will inevitably cause heightened pain response - but I decided to ignore this - I'm not getting landed with Fibro and IBS labels at this stage in my life - although I made a point of saying I think my pain threshold is actually remarkably high!

At this stage I should mention that when I arrived he had laptop open showing my nailfold capilliaries from 4 months ago. So we discussed my Raynauds and response to Sildenafil. This was the most interesting bit of all for me. He had looked at photos of my fingers and toes during attacks and said they weren't really typical classic Raynauds and I don't have Raynauds typical of scleroderma either. So, given my normal nailfold capilliary results, his thinking is that the colour changes are a response to cold due the small fibre neuropathy loss of sensation in my fingers and around my body. He explained that small nerve fibres throughout our system sit right alongside and surround the blood vessels. There's a bridge connecting the small nerve fibres and the tiny blood vessels. So if the nerve fibres are dying off then the vascular system registers this by shutting down in response to cold. So this is a particular kind of Raynauds secondary to small fibre neuropathy of Sjögren's I asked? and he confirmed that this was exactly right.

I told him about my superglue fingers during winter with painful tips - he said he really would need to see this for himself to assess and take a nailfold capilliary test while it was occurring. So I'm to get straight in touch if I get superglue finger again. However I failed to get him to look at pink vertical lines running through my nailbeds - which was stupid but there so was so much else to cover Ina short time.

[MAT51]

The oral consultant appointment just now was much more difficult for me. For some reason she makes me feel a bit like an over anxious pudding?! She is lovely but she reminds me a lot of my late mum. She's wise and knowledgeable but, having been pretty upbeat with the CTD doctor, I found I then got a bit emotional with my consultant - who felt that I probably didn't have arthritis of jaw, parotids are clear, said no idea about mouth and nose pressure or bad taste as my mouth really didn't seem particularly dry to her. She took notes of all the CTD doctor had said and of the ENT. She had the cheek to wonder whether or not the sinus CT might have included my jawbone when I'd expressly asked her on phone on Thursday to contact the hospital and request this be included. I told her adamantly that the CT done of my sinuses did not include my jawbone because I'd asked the radiologist!

So she is getting a CT done of my jaw to see if there's any cartilage damage but suspects the tenderness is due to muscles arthralgia of Sjögren's and a small fibre neuropathy. My parotids are fine. She will see me again with the results of jaw CT and will write to my neurologist to say that she thinks the taste issue is probably neurological rather than simply due to dryness as ENT had thought.
I admit that this really upset me for some reason? Exzcess dryness is hard enough to get my head around but the idea of it being more that is neuro stuff related is harder still for me? It doesn't help that I don't like my neurologist much - she makes me feel like I'm a neurotic and says I over think things and look too well to have much wrong. Grrrr! But oral doctor did confirm my suggestion that the deviated septum identified by ENT might be causing me to mouth breathe more than I'm aware and that could be making my mouth dryer perhaps.

Anyway I'm not due for annual neurology review until April and the CTD chap said that he didn't feel IViG was remotely an option for SFN but this would be up to my neuro to determine. My neuro won't even confirm my SFN by taking skin biopsies let alone entertain or discuss IViG!

But at least I'm going to see a gastroenterologist and I'm going to get a CT done of jaw - so two big things on my tick list achieved along with a greater understanding of rheumatic fatigue.This is all great as fodder for my proposed PhD topic at least. I told the nice CTD chap that I'm hoping to see Dr Price (UK Sjögren's expert) privately one day soon for research for my PhD - hoped this was okay? He looked rather baffled because, frankly why, when my own hospital team seem so on the ball about my Sjögren's, would I pay to see someone in the south of England for their perspective? And I have to admit that he was brilliant for explanations - but my for my PhD on visualising invisibility I really need to see Dr Price, if only once, and at my own expense. I'll get a lot more time to ask questions about my seronegative neuro version of this disease.

And brilliant as they seem, my new team are not a dedicated Sjögren's experts as she is. I forgot to ask oral med consultant for a copy of my lip biopsy results to take with me so that's a bit pants. I kind of fell apart. But hey ho - guess I'll see her again soon and will hopefully be less of an anxious bundle next time. 3pm is really not a good time of the day for me.

[irish]

Man, that was really a good once over, so to speak. You got a lot of a answers in all those visits. The one thing that really hit me is the fact that you cant get IVIG for the SFN. In the states iVIG is the the treatment of choice. Most of the time people start out with some of the oral meds but people may not even be diagnosed with SFN then as it takes time to get all this stuff figured out.

I hope the neuro doc gets this figured out. By the way, I have suffered from dryness for about 40 years.  I didn't know why until diagnosed in 2003. I has chewed gum for years and past 20 years have sucked on sugar free cough drops. It eases my dryness when I am doing things and unable to sip fluids. Also, it helps with the the rotten breath I have from this dryness.

We can just use all the products on the market and alternate them off and on to change the feelings our mouth gives us. I also suck on ice. The dryness is not fun and I get sick of the metal taste I always have in my mouth. I think it goes with the disease. Thankfully, it is not fatal.
Good luck with all your doctors as time goes by. I

Ii wanted to tell you to pursue pancreatic enyme deficiency also. I was just diagnosed with this and it really gave me weird bowel habits and misery. Also, I was on cellcept for a while and stopped it for many reasons  but am hoping to start back on it. I found that it upset my stomach also and I was on 2000 mgm. It mightn be that just dropping back 500 mgm would cut down on some of the misery. Good luck. Irish

[MAT51]

Thanks so much for replying (and for reading my novel lol!)Irish. I know it's a bummer about IViG but it's a very expensive and scarce resource. The other problem would be that I had skin biopsies taken from my calves two and half years ago and zilch showed. The neuro says that's because mine is non length dependent so it would be hard to know where to take samples from and she is happy just to diagnose it without the histological evidence. I've tried arguing but she just talks over me or says I'm fixated! I know from Anita that, without this confirmation, I'm on a non starter. And regarding the length dependent this is erant nonsense because it started in the soles of my feet but it did also start in my arms a few years earlier - much more subtly (warm waves!). It's now only really mean in my lips and gums and stockings and gloves during flare ups,

The weird thing is that I have never had bad breath or thrush others speak of. And I live on saliva stimulants - apart from ice which triggers awful neuralgia and oesophageal spasms. Maybe at last the gastro will test me for gastroparesis and also test the pancreas thing. I had this tested once before early last year with a stool sample but it was clear. My amylaise too is clear. I'm really fed up with the bowel stuff. And I also know the bad taste (and mine isn't just rust - it's like off milk, garbage and poo mixed up!) is killing my quality of life so that's not such of a benign thing really? It is barely alleviated by sucking sweets or chewing gum (oral consultant says 2 bits gum a day only due to TMJ) I must admit. I was most disappointed that she couldn't see any sign of oral dryness or anything to explain - makes me worried when there's no visible indicators. I feel I must be going nuts - and I worry they will think I'm making it up? 

[warmwaters]

Thanks for the detailed posts. Several things you mentioned gave me some things to think about.

[MAT51]

Thanks for the detailed posts. Several things you mentioned gave me some things to think about.

Warmwaters I am glad my novel length post has given you a few things to think about at least. 

[anita]

Thanks for the update.  I have been out of town and just now catching up with reading threads.

I, of course, agree with Irish about the IVIG and have suggested to you many time about getting a new biopsy done (since they only did one site and didn't do any morphology).  He cannot know if it is non-length dependent because they only took one site and length dependency is based upon results comparing at least two sites.   I would be more inclined to think it IS non-length dep. because you have so much involvement in your mouth and face...which is common with NLD SFN.  But all this has to be confirmed to be properly treated.  Tough spot you're in.  Wish you had more options for testing.

But as for Cellcept, you would know if it's working if your symptoms improve...simple as that.  If they didn't improve, then it likely isn't helping.  You have been on it for several months now, so you would have seen some changes by this point if it was going to help.   Can you say for sure whether 'anything' has improved...and stayed better since starting the cellcept? 

On a positive note, you do seem to have some good doctors on your side...for some of your issues.  Just wish you can get everyone on the same page.

Overall, do you think you are worse or better than you were before starting the Cellcept?  I know that may be hard to answer as some symptoms may have gotten worse, while others better.  But overall, do you feel your quality of life is better now?

[irish]

I was interested about your muscle biopsy coming from your lower legs. I had one quite a few years ago and they did it in my upper thigh under anesthesia. They were really fussy about what doctor did it and how it was done. I guess it is tricky to get a good biopsy. Just some info. Good luck. Irish

[MAT51]

Thanks for the update.  I have been out of town and just now catching up with reading threads.

Thanks Anita for your reply. I did specifically ask the Scleroderma/ vascular doctor about IViG after he spelt out that Cellcept was the last option. He looked very surprised because I think he was trying to say that seronegative Sjögren's doesn't respond well to Rituximab so wouldn't be able option for me. He said the usual thing about how he'd never heard of IViG as treatment for SFN but anyway this would be for my neuro to determine - and he looked at me and said "and I don't think she would consider it for you" - they work closely with my neuro I know. So we are back to same old thing of NICE guidelines and BSR guidelines not having SFN on the IViG criteria. These links might be of interest to you though - perhaps you will be able to interpret them better than I can and spot loopholes?academic.oup.com/rheumatology/article-lookup/doi/10.1093/rheumatology/kex163

academic.oup.com/rheumatology/article/55/suppl_1/i179/1795516/290-The-Burden-of-Systemic-Disease-in-Patients?searchresult=1

I am determined to see the UK expert who helped draw them up because she's supposed to be very nice and approachable - and extremely committed to improving things for Sjögren's sufferers here. But the thinking is that my SFN is same as it was pre Mycophenolate and isn't progressing - and also the facial stuff, BMS and horrible taste is much the same. The aspect that seems to be worsening is the GI stuff but I'm on a new laxative regime now which may be helping. My food issues are worsening but I think this is SFN/ TN/ BMS related rather than gastroparesis because I can swallow most of the time but just can't bear certain textures or tastes anymore. I seem to need food to be either salty or sweet to be able to swallow it?!

My constant tinnitus is defintely progressing to be louder but I ignore it as best I can. I often wonder if it related to the confirmed arthritis in my neck.

So the thinking about Cellcept is that it is being used to prevent the SFN progressing to my large nerve fibres. I'm not needing a stick for my balance now so I think my proprioception issues have improved somewhat - although they worsen again when I'm extra fatigued - and I've had a few collapses from this. Also the confirmed lumbar OA is defintely progressing and this causes me mobility issues too. The Cellcept won't help with this of course.

But this doctor and I decided that, on balance (literally lol!) the Cellcept was probably helping more than I realise. Also my BP has normalised (sometimes too low) after a few years of being extremely high - and there is no longer any microscopic haematuria. But this may be more related to my weight loss over the past few months as I've lost all pleasure in food - especially anything cold or mushy or meaty. I would cheerfully live on slowly nibbling toast and marmite with an urn of hot tea and Koko milk if I could! Quality of life rather poor because of the increasingly awful fatigue and facial/ oral issues. However I'm still awaiting my ENT's report on sinus CT and have just been referred to sleep clinic for assessment on whether I may have sleep apnoea.

Also maybe getting my deviated septum sorted and getting my neck looked at again would help me to determine what is going on more. My finger tips are very sore again and I am assuming now that this is SFN too. So I'm really not sure where the skin biopsies would be taken from - if I could persuade anyone to do them again? My feet are pretty much cold and numb to the touch but only in the outer layers/ skin. Although my rheum was unable to elicit a response from my Achilles tendons back in April.


[/color]

[MAT51]

Thanks for the update.  I have been out of town and just now catching up with reading threads.

I, of course, agree with Irish about the IVIG and have suggested to you many time about getting a new biopsy done (since they only did one site and didn't do any morphology).  He cannot know if it is non-length dependent because they only took one site and length dependency is based upon results comparing at least two sites.   I would be more inclined to think it IS non-length dep. because you have so much involvement in your mouth and face...which is common with NLD SFN.  But all this has to be confirmed to be properly treated.  Tough spot you're in.  Wish you had more options for testing.

But as for Cellcept, you would know if it's working if your symptoms improve...simple as that.  If they didn't improve, then it likely isn't helping.  You have been on it for several months now, so you would have seen some changes by this point if it was going to help.   Can you say for sure whether 'anything' has improved...and stayed better since starting the cellcept? 

On a positive note, you do seem to have some good doctors on your side...for some of your issues.  Just wish you can get everyone on the same page.

Overall, do you think you are worse or better than you were before starting the Cellcept?  I know that may be hard to answer as some symptoms may have gotten worse, while others better.  But overall, do you feel your quality of life is better now?

Sorry made a hash of quote - can't edit to unquote now but hope you don't mind?! X

[MAT51]

Anita - to quote your rheumy, Julius Birnbaum - this is what I think is probably affecting me most. Not progressing as such but back with a vengeance. I'm adding the 2017 BSR links I attached earlier - the work of the UK's equivalent of Julius Birnbaum - Dr Elizabeth Price. Hoping to see and discuss IViG and SFN with her in October. Meanwhile my excellent oral consultant says she is going to write to my neurologist about my ongoing facial issues. These and the GI problems make life pretty grim regardless of Cellcept having improved my balance.

academic.oup.com/rheumatology/article-lookup/doi/10.1093/rheumatology/kex163

academic.oup.com/rheumatology/article/55/suppl_1/i179/1795516/290-The-Burden-of-Systemic-Disease-in-Patients?searchresult=1

www.ncbi.nlm.nih.gov/m/pubmed/16733343/
- re bad taste

"2. Trigeminal neuralgia and glossopharyngeal neuralgia

Sjögren's syndrome can cause numbness or burning of the face, called "trigeminal neuralgia." Pain in the back of the throat, which may worsen while swallowing, is called "glossopharyngeal neuralgia." Patients with trigeminal or glossopharyngeal neuralgia can have agonizing mouth and facial pain. These neuropathies may co-exist with other neuropathies in different parts of the body. For example, up to 20% of patients with a "small-fiber" neuropathy may also have trigeminal neuropathy."

[irish]

The trigeminal neuralgia is often treated with Gabapenten with fairly good relief. Usually start off at a lower dosage and then can increase as needed. Im sure others will offer some info on what med they use for this condition. The trigeminal is more common than anyone wants for some reason. Probably has to do with the neurological involvement with the Sjogrens.

Hope you can get the IVIG issue resolved in your favor!!!! Good luck. Irish

[MAT51]

Thanks Irish. I'm afraid I have refused to take any more of the anti convulsant/ antidepressants now having had dreadful side effects on most of them, including Gabapentin. The only one I haven't tried is Pregabalin but I can't face it (pun intended!). The only thing I would try it for is if it eliminated the awful rancid taste, the raging tinnitus and the burning gums and lips. Can't see it doing these things when Gabapentin made me see double, had me full of uncharacteristic anger and couldn't walk in a straight line

It would be good to have the nerves that run down the back of my head checked for possible lesions because that is what an oral consultant suggested to me a few years ago. But my neuro at the time, pre Sjögren's diagnosis,  felt it was all functional following a traumatic health year, and wouldn't do further investigations. I agree that this problem probably affects many more than is acknowledged - all gets out down to migraine or functional/ anxiety/ Fibromyalgia or idiopathic.

I'm hoping to start a PhD looking at the role of invisibility so I need to find some way around the neuropathic pain in fingertips for typing, the waking all through the night (nasal congestion, insomnia) and hugely debilitating fatigue. I've elicited a referral to sleep clinic out of my GP to rule out sleep apnoea. Got a theory that the tinnitus and possibly other facial stuff, is being caused by confirmed arthritis in my neck. So maybe some appropriate manipulation or massage would help. Take care and thanks