Treatment of our choice

[Sharon]

If you could get any treatment you wanted for your SJS (or its symptoms)
of those currently available
and cost/insurance approval/doctor's scripts weren't an issue,
which treatment would you choose? 

[Dawnmist]

IVIG.

In Australia, IVIG is restricted to use for conditions that are life-threatening if not treated with IVIG. So despite IVIG having been successfully used overseas to stem the progression of auto-immune caused neuropathies, it is completely unavailable to me. Sjogren's has caused severe Small Fibre Neuropathy that extends from toes to groin, fingertips to elbows, and now across the mid-to-upper back, shoulders & neck. Pressure or brushing contact on the affected areas of skin cause pain - severe enough in the feet that I need a wheelchair if I'm walking more than about 50m, and in the last week I've had the unpleasant discovery that about 90% of my tops now trigger enough pain that I cannot wear them for a full day at work - they feel "too rough" and abrasive now causing the skin to burn severely by 3/4 of the way through the work day. :-S Sometime between the end of Sept and mid Dec last year, the damage also started to extend to the longer sensory nerve fibres in the legs, so they went from response being "well preserved" to it being "attenuated to absent" in nerve conduction tests. And despite all that, I still haven't been able to get any treatment aimed at trying to slow/stop the relentless progression of damage.

I've requested a referral to a neurologist in Melbourne who specialises in treating neuropathic pain, has a current research grant to investigate the effect of immune modification on chronic pain, and spent some time in London where she helped them put together their testing procedures for Small fibre neuropathy. She's the most likely person I can find with *actual experience* in the area to be able to help. The only problem is that it may take anywhere up to 8 months to get an appointment with her. The way things work is you send them your referral, the neurologist triage's the urgency and can decide to squeeze you in earlier if they feel it is needed, and then the office sends you the appointment time in a letter in the mail. I've submitted my referral and spoke with the office on the same day (which is why I know the default wait time to see her is 8 months), and am now waiting to find out what appointment time I'll actually be able to get.

I am really hoping that someone will let me do something before the damage hits the point that I cannot work anymore. I've been able to work around most of the pain so far by taking medication to deal with the "constant" base-load, and finding ways to avoid triggering break-through pain (e.g. typing on fingernails not fingertips, not using a computer mouse or holding things for very long, using the wheelchair/crutches, etc), but other than choosing very soft cloth I'm limited in how much I can work around pain triggered in the upper back & shoulders...I really can't go to work without clothes on!

[Joe S.]

Arnie Palmer (tea and lemonade) for dry mouth sipping.
Marijuana for pain, blood pressure, and appetite. (This is the one I am not allowed. So I will use carrot Juice and reflexology.)
Acetyl-L-Carnitine and R-Lipoic Acid to repair damage to DNA

[Nymph]

I am pretty happy with my Plaquenil and armamentarium of supplements. However, I think that my treatment of choice would be a vagal nerve pacemaker for inflammation and maybe a fecal transplant for my microbiome. Then maybe I could live drug-free?!?!?   

[WhatYouSjo]

If money wasn't an issue, I would strongly consider the allogeneic mesenchymal stem cell treatment that has been researched in China.

There are a couple small molecule inhibitors that are currently in clinical trials that I would also be interested in.

[warmwaters]

I'm going to more rules! And regardless of past experience, you'll have no side effects.


IVIG  - had to discontinue, but still feels like the right treatment.

[Sharon]

Dawnmist- that's awful that they won't give you the IVIG you obviously need!
Hope you get that appointment as soon as possible and that it leads to effective treatment!

Joe- I second your suggestion of medical cannabis!

Nymph- I've read they now have a vagal nerve stimulator that doesn't require an implant.
I too would love to try that. Last I checked it cost 30,000$! 

Warmwaters- Did the IVIG do anything positive for your other symptoms other than for neuropathy?

WhatYouSjo- Do you feel that the allogeneic mesenchymal stem cell treatment has been researched enough to actually render it safe?

I personally would like to try:
-One of the biologocal treatments available- either Orencia or Rituximab 
-Medical cannabis, preferably CBD
-Dextromethorphan therapy:
https://www.hindawi.com/journals/jir/2013/125643/

[Jasper]

I am happy with my Rituximab infusions because they have vastly improved my symptoms and I now have a life. I did not have a life prior to Rituximab infusions. I was barely existing.

However, I am open to any new treatments if they prove to be more beneficial to me than Rituxan, especially if they also reverse damage.

Because Rituxan infusions have been so beneficial for me, I would hesitate to stop them to try something else unless I was sure the new treatment was definitely going to help me more than Rituxan has helped me.

Allogeneic mesenchymal stem cell treatment does sound promising. I wonder why it seems stalled. The article I read was from 2012. It seems like it should be advancing unless there is some reason we don't know about. Of course, there have been numerous studies demonstrating the efficacy of Rituximab in Sjogren's and yet, it is still not FDA approved for Sjogren's, although it is listed under the new Sjogren's treatment guidelines.

[Dawnmist]

My second choice would be Rituximab. But that's not approved for Sjogren's in Australia, so again I cannot have it unless I get approved through a Lupus clinic or develop Rheumatoid Arthritis as well. My Rheumy did try sending me to a Lupus clinic last year, but the head of the Lupus clinic appeared to decide that I was making things up and denied it. Her argument was that the QST testing (the ONLY tests available for small fibre neuropathy within Australia) was not "objective", so it was not admissible evidence of nerve damage occurring. Without it, there was "no objective evidence that Sjogren's was doing anything at all to me, only 'vague' complaints of pain, and thus there was nothing to treat" (her words). That was about 3-4 weeks before the pain in my feet triggered by walking reached the point where I couldn't get to work in a state *fit* to work without using a wheelchair.

IVIG will never happen without life-threatening organ damage or developing a life-threatening nerve-damage condition like Guilean Barre. I can at least accept that - it takes a *lot* of blood donations to make IVIG and we just don't have the population here to produce enough for more than life-saving purposes.

Rituximab didn't happen due to prejudice. That I have made a formal complaint about, but the hospital refused to review the previous decision.

[Anastasia]

I have to agree with Jasper.  Rituxan has been a wonder drug and has given me my life back.  I am not symptom free by any means and I often wish I could comprehend the illness better, but I am so grateful for a treatment that restored my quality of life.  I take Plaquenil also - 400 mg per day.  I'm not sure if it does anything, but am reluctant to quit it because it takes so long to work up to a therapeutic level.  I get 6 month checks with my ophthalmologist to monitor for problems. 
My second Rituxan infusion is tomorrow.  My insurance covers the whole cost. 
On Rituxan, I went back to work after a semester leave.  I teach high school Special Education and have no difficulty managing the demands of my day.  I have plenty of energy and feel good most of the time.  I do still have episodes of pain and inflammation in my tissue.  But it's manageable. 
I would also be very interested in the stem cell treatment.  I believe this will be the breakthrough treatment for autoimmune disease.  Currently, the most reputable research is occurring at the Feinberg School of Medicine at Northwestern under a Dr. Burt. 
I do wish that others did not have to have the constraints I read about here in terms of insurance and health plans.  I wish you all well!

[Sharon]

Dawnmist- From my experience, when you need an additional diagnosis to receive proper treatment
it's necessary to find a rheumatologist who is on your side and wants you to get that treatment.
Look for someone who is caring, empathic and who has a heart.
I have had no success dealing with the inflated egos you may find in hospitals.

Jasper and Anastasia- Did Rituxan help with your sicca symptoms?
Problem is that the results of studies of Rituximab with SJS are mixed, with some showing promising results and others....not.
That's why it's not always approved for SJS patients.

As far as the stem cell research- yes, it's going real slow and I wonder why!

[Nymph]

I would imagine that safety studies for stem cells may take a few years. Add that to the time to get through the bureaucracy and organize a study... Then it takes results awhile to be published after that.

[cccourt1942]

Sharon,
   Your final statement: "I wonder why."  If that is a leading statement, I got it.  And therein lies the big problem when hoping for stem cell research discovery -> treatment.
c3

[Jasper]

Sharon .....

Rituxan has definitely improved my saliva flow. My saliva flow had improved quite a bit with CoQ10 but it improved even more with Rituxan. I still use CoQ10 in additoin to Rituxan. I never even think of water anymore. I don't get a dry mouth. I can go hours without drinking anything and I never need to carry water with me any more. This is a big change because I used to feel dry all of the time and I would get frantic without water.

I still use Restasis for my eyes and notice if I forget to put it in. However, my opthamologist said that my eyes lok the best they have ever looked in terms of cornea, moisture, glands, etc. so I think Rituxan is helping with my eyes as well.

My skin seems as dry as ever. I have never had vaginal dryness but I take hormones so I am sure that makes a big difference. I have never had constipation or dry bowels, so I have no experience on that topic.

All of the studies (except 1) that I have read show significant improvement with Rituxan. One has to read the entire studies, not just the abstracts, in order to see where the improvements are, when they occur, and how significant they are. The abstracts do not give the full picture. The abstracts focus on end points and if the end points are flawed, the results are flawed. The longest study, 120 weeks, showed significant improvement with Rituxan.

I think the biggest problem with so many of us having difficulty getting appropriate treatment is that doctors, including many/most rheumatologists, consistently dismiss our disease and its symptoms as a nuisance disease (with annoying symptoms) instead of treating us like they treat patients who have other rheumatological diseases. With so many of us disabled, I don't know how they can miss the debilitating effects of the disease, but they do. We do not have deformed joints or some other obvious deformity. The lab tests they use do not correlate well with our disease activity. We don't look sick. So, to most of them, we are not that sick and don't need treatment.  In the meantime, we just deteriorate while they are all twiddling their thumbs.

We need to get to the stage where we are taken seriously and treated aggressively at the onset so that we do not keep deteriorating and losing function. With RA, they are now trying to diagnose it very early, before any damage whatsoever occurs and even before most symptoms start, and treat it aggressively to prevent it from progressing to full blown RA. They are even treating autoimmune alopecia with big gun drugs and they treat psoriasis with big gun drugs. I empathize with people who have those diseases, but my point is, if alopecia is considered serious enough to treat, why isn't Sjogren's Disease considered serious enough to treat,. Alopecia does not disable a person, whereas Sjogren's Disease can and very often does disable a person.

I am very fortunate to have one of the few Rheumatologist who actually does take Sjogren's seriously and who does treat the disease.

[Sharon]

Jasper- So very well put! I could not agree with you more on all the points you made!
When I said the studies on Rituxan were inconclusive regarding SJS, I was relying on what rheumatologists have told me so far
when I inquired about the treatment. One went so far as to say she "didn't believe in it". 
I will try to read the studies in their entirety.

Nymph- True, though you have to wonder if the last study published on the stem cell treatment discussed was in 2012,
with nothing on further trials mentioned.

C3- True, unfortunately.