Discouraged and dismayed.

[Dawnmist]

I'm from Sydney Dawnmist, but I've heard of Monash. Isn't that one of your top hospitals??

Sadly, life experience has shown me that the arrogance of some doctors is often far greater than their ability.

I hope your Rheumy gets some answers or can refer you on to another Dr who can help you.

Yes, Monash is one of our major teaching hospitals. Hence why it has a specialist Lupus clinic, and why he had tried to refer me to there in the first place. There are a few other hospitals that might have the ability to do it - the Alfred is another major hospital here that I would expect could handle the same stuff.

Arrogance is definitely right with how she behaved during the appointment.

I am seriously considering asking my Rheumy to organize to get a skin punch biopsy kit from Therapath in the US - the difficulty being the refrigerated shipping back to Therapath so that the sample arrives within 5 days or less from when it was taken. No-one could then reject the test like she did - it's the only truly objective test available. 

[Dawnmist]

My Rheumatologist contacted Dr. Julius Birnbaum by email to ask for advice on treatment options.

He responded that there really was no need to do the skin biopsy, as the symptoms & sensory testing plainly showed that I had SFN, so all I'd get by doing it would be pain at the biopsy sites.

He also recommended that I really should be getting IVIG, as it was the only thing that really showed consistent improvement with SFN.

So my Rheumatologist referred me back to the Neurologist that had confirmed the SFN diagnosis, and he confirmed what I thought I had understood about the state of IVIG use in Australia. There are only a very few neurological conditions that it is approved for, and SFN is not one of them.

So despite it really being one of a very few options for actually treating the SFN, it is completely unavailable.

Rituximab is also completely unavailable - that was blocked by the head of the Lupus clinic at Monash two months ago (which was when I started this thread).

I've deteriorated to the point that I need to use a wheelchair to get to work now. If I try to do it using my crutches, I'm in too much pain when I get there to be fit for work - and it can take 4-6 hours for that pain to start to settle down. A powered wheelchair at that - my hands won't tolerate pushing a manual chair.

So this weekend I'll be celebrating my 40th (a month early but it's impossible to get people together for a party in mid December) in a wheelchair - and it looks like I'll have potentially another 40 years (+/-10) in a wheelchair to look forward to.

I was walking 10,000+ steps a day at the beginning of June this year. It only took 4 months to progress from walking freely to needing a wheelchair. Pain management is scrambling to find something that will help get the pain under some control again, as it has broken through everything I was taking. And the medications that I knew had some chance to actually help treat the neuropathy instead of just masking the pain it causes have all been denied.

[SjoGirl]

Dawn I am traveling a similar path, SjS supposedly under control, but increasing neuropathy on the whole left side of my body. The neuropathy diagnosis is official as of yesterday, but I won't know the type (and maybe potential cause) until I see my neurologist on Friday.

My progression has been fairly sudden as well, coming on over the past six months or so. I began having ocular migraines, myoclonus, and increasing nerve pain all at the same time. I would not be able to get through the day without Gabapentin (and I've developed a much higher tolerance for pain than I used to have).

I too have difficulty holding things, smashed a glass dish several weeks ago because I thought I had it in my hand when pulling it out of the cupboard, but apparently did not. I frequently drop things and my husband is certain it's due to lack of feeling in my hand.

FYI it is possible to have neuropathy apart from Sjogrens. When I told my rheumtologist about my test results he said let's see what type of neuropathy it is before determining a possible cause. I don't always agree with him, but have found he and the neurologist to be best at treating me to the degree that they are able (and that is limited).

I feel your pain, literally and otherwise, wishing you strength.

[Dawnmist]

FYI it is possible to have neuropathy apart from Sjogrens. When I told my rheumtologist about my test results he said let's see what type of neuropathy it is before determining a possible cause. I don't always agree with him, but have found he and the neurologist to be best at treating me to the degree that they are able (and that is limited).

I feel your pain, literally and otherwise, wishing you strength.

Thank you, and wishing you the same.

In my case, it's one of the classical types of neuropathy that can be caused by Sjogren's, so there has been no doubt about its cause. They did double-check early on that there wasn't another condition causing it (diabetes, coeliac disease, etc), and all the alternatives (except genetic) were eliminated.

Knowing what's causing it is no help though, because an auto-immune related neuropathy means that there isn't really anything I can do to help control it. If I was diabetic, I could at least try to halt its progress by being very strict about glucose control. If I was coeliac, I could again try halting it by being very strict about avoiding gluten. If I was an alcoholic, trying to give up alcohol would help (I don't drink anyway)...but what can I do to stop my immune system from eating my nerves - other than eat right, exercise, etc - all stuff that I was already doing and clearly hasn't helped all that much?

At this point, all I can do is accept that I will be disabled for the next few years at least, and likely for the rest of my life, despite the existence of medications that might have been able to prevent that. I know that there is some research happening now about nerve regrowth, nerve pain, etc - so maybe sometime in the future it will become possible to reverse some of it. If there isn't though, or if I continue to be denied - its "only pain" afterall (yes, that is a quote from one specialist) - I need to just accept it as what will be.

[MAT51]

Heck and I thought my Scottish neuro and rheum were being harsh in their attitude to neurological manifestations of Sjogrens?! Mind you much the same applies here and I do usually have a very high ESR and raised CRP. But this will vary from person to person - so my low ESR might be your high one. For your interest it says on the Arthritis Research UK site that people with Sjogrens often have a very high ESR and CRP, but this is because they have Sjogrens  - which can concentrate the blood and protein levels. This is different apparently to having high levels of inflammation, and therefore is not useful as a measure of disease activity with Sjogren's, as it is for RA and Lupus. So your ESR may be be high for you but regardless, it should and be used to determine whether your Sjogrens is active or not.

I had a second, very pedantic rheumatologist who told me in January of this year that, apart from with RA, seronegativity isn't recognised anywhere in Scotland. When I tried to query this he went and got me the sheets for diagnostic criteria for Lupus and for Sjogrens. I pointed out that I'd been tested by him last year when I was still on steroids, having spent 2 years on Methotrexate, Plaquenil and others. He said this would make no difference to my autoantibodies and then leant towards me and said emphatically "you do NOT have a connective tissue disease. You may or may not have had RA but it is now inactive anyway". He just shrugged about the widespread SFN and disequilibrium and said it was not his problem as not caused by a rheumatic disease in my case.

Six months on, in a new hospital and off steroids, I was found to have a +ANA plus raised IgG and IgA and the usual very high sed rate. On this basis the new rheum advised a lip biopsy which came back very definitively for Sjogrens. Both my neurologist and new rheumatologist say it fits entirely with Sjogrens.

The last rheumatologist, who told me I did not have a CTD at all, and wondered if my consistently high sed rate and SFN might be a form of cancer ("not my area of expertise though") - was supposedly an expert in Sjogrens!

It's good to know I have SS now but otherwise, without organ involvement, I am only going to be offered symptomatic treatments I can't tolerate. And this is despite a very high sed rate. So why is an immune mediated small fibre neuropathy not taken as seriously as other inflammatory responses, when I keep reading that once they are dead, nerves cannot be regenerated?  And like you I notice that the emphasis in all related literature says how the main thing is to find the cause of the neuropathy and treat it at source. So for us the cause is known but is still left untreated? Very perplexing I agree. 

[Lotus1]

I'm so sorry you're are going through all this.

It makes me mad that Drs say you're making up things like pain.

I've been told it's all in my head in the past also which I strongly denied.

I sure hope you get real help, support, empathy quickly!

Lotus1

[irish]

Pardon me if I mentioned this before but I am wondering if you ever got a different or new neuro consult after you got the report and info from Dr. Birnbaum. It might help. I do know what it is like to be told it is all in your head and it is not pleasant. My diagnosis was held up by a clinic witih some doctors that literally blackballed me.

I am in the USA and don't know how your system works there, but I hope that you don't give up. . Have you documented your daily activities and how you managed to do them. Also, the next time you go to the doctor can you find a friend who knows you well and has a big mouth.lol  You need someone who will stand up to a doctor and tell him how you are suffering and that your condition is slowly progressing downward. These darn docs who think we do this running to doctors for fun and attention. It is terrible!!!!

In the USA we have patient advocates that are employed by the hospitals, some clinics and the county health system. We are able to go and speak freely to them about the health care we are receiving and how and where things are going wrong in our qwest for better health and diagnosis. I wish there was someone like that in your country.

Whatever you do please continue finding different doctors to see. I have had some doc visits years ago when I struggled so for diagnosis and the first word I would say to my new doctor was "help. I can remember sitting in the doctors office being examined and trying not to talk too much or say the wrong thing cause I didn't want to upset the doctor. We should not have to do that when we go to a doctor.l Good luck and know that I am thinking of you and praying for your well being. Good luck. Irish

[Dawnmist]

My normal Rheumatologist did send me back to the Neuro to try to get IVIG, but it simply is barred for most people. Small Fibre Neuropathy is not on the permitted condition list, so there is no chance that it could be approved for that. Sjogren's is on there as "extreme circumstances only" - which from what I have been able to determine means when it's attacking major organs. "Simple" neuropathies that won't kill you - just make you wish you were dead sometimes - have no chance at all.

My normal Rheumatologist is 100% behind me. My Pain Specialist has actually referred me to a second Pain Specialist and together they're trying to find something that will work. My GP is 100% behind me too. My (occasional) Neurologist has confirmed (again) the diagnosis of Small Fibre Neuropathy. It's just the head of the Lupus clinic that has blackballed me - and since Rituximab is only "approved" for use with Rheumatoid Arthritis & Lupus (and various cancers), the only way I'd get access to it with Sjogren's is through that Lupus clinic.

I am not going to give up - but it's not going to be a fun problem to solve.

Thank you for your good wishes, I appreciate them.

Cheers,
Janeene

[irish]

Sometimes a doctor can write in to the IVIG committee and make a request for infusions based on medical symptoms. I have forgotten if you are in the USA. This probably doesn't apply to other countries. Hope there is something they can come up with. If you have Sjogrens I would think they could apply for it under extenuating circumstances. Small fiber neuropathy is a problem with Sjogrens patients. Good luck. Irish

[MAT51]

If you are in Australia as I think you mentioned previously,  then it might be worth joining HealthUnlocked for the Australian Sjogrens Syndrome Association HU community and asking there if anyone has successfully appealed or managed to access these big gun treatments for their SS?

I know of two people in the U.K who have primary SS and are on Rituximab but don't yet have organ involvement. I know of one person from Scotland who was able to get IViG for SFN despite not having a formal diagnosis of Sjogrens. I think they were sent to London to have biopsy of their dorsal ganglion nerves though. It seems so unfair when I think of two people I sat with on a neuro ward last year  having IViG for CIDP despite the fact that only very small areas of their bodies were affected and all they suffered from otherwise was chronic fatigue which the IViG really helped them with they explained.

[cccourt1942]

Is IVIG administered for numerous conditions at various levels of advancement? Or is this a result of "a" test, then Rxed?

[Dawnmist]

IVIG is administered for various conditions based on the "quality" of the existing evidence at the time of last study (2012) showing that the condition was actually helped by the use of IVIG and the lack of availability of alternative treatments that could be used instead (or the alternatives were contradicted) for that condition.

https://www.blood.gov.au/pubs/ivig/executive-summary.html

Sjogren's falls into the "extreme circumstances only" category, and Small Fibre Neuropathy isn't listed at all.