CIDP, Sjogren's (?)
 

I have not posted on Neurotalk for some time and had, at least for a time, improved. I still see some friendly and familiar faces here.

I have had what is believed to be CIDP (and Sjogren's) for quite some time (1992, I believe), though things had improved to some degree until 2012, when I had to get a pacemaker. This did not set well with my body and autoimmune problems and I have been unable to reduce my Prednisone back down to where I was when I was nearly off of it. My Cardiologist thinks that the heart issue (AV block, Mobitz Type II) relates to the autoimmune disorder.

Most recently,an Osteoporosis drug did a number on my pain level. This was followed by an injection of Depo Medrol, which my body also did not like. Picky, picky, picky... The result is I am finally going to consult a pain management anesthesiologist this week.

The Rheumatologist I see seems to be very bright, and I am happy to have someone who actually wants to help. He said that they are finding that about 20% of the patients diagnosed with Sjogren's, do not have this, but have small fiber neuropathy, which presents in a similar fashion. I think the gold standard for diagnosis is still the lip biopsy, something I would like to avoid.

It looks like the next step will be IV Gamma Globulins now. Wow! Have they increased in price!!! I could almost buy a new car for the cost of one dose...

Anyway, that's my story and I will try to be better about keeping up with
posting...

I was wondering if Silverlady was still on the boards. Anyone know?

Yorkiemom
Cathie

Her last post seems to have been in 2011.

Doc

Good morning Yorkiemom :)
 

Hi Cathie,
I'm so sorry your pain has increased again.

I had a lip biopsy a few years ago. My rheum would like to do another. I told him I want to think about it. My lip really hurt afterward. Plus, I am not sure the treatment would change.

I do get IVIG. I'm lucky my insurance pays for it. I agree the price is outrageous!

I did have my eyes tested and did have really dry eyes. But I do believe the lip biopsy really qualifies for the dx. My rheumo said that the bloodwork may not show anything for years.

I don't know what I believe. All I know for sure is I have SFN. I had the biopsy.

If you do decide to get the IVIG, I do believe I get some pain relief from it.

Hope you feel better soon!

My goodness, you really have been through a lot Yorkiemom. I too would try to avoid the lip biopsy.

I have Sojgrens Syndrome, diagnosed through blood markers, dry mouth and dry eyes. Do you have dry eyes and mouth?

Could you periodically repeat the bloodwork? If you have SS, would it eventually show up?

Would you mind saying what osteoporosis medication caused you trouble? My rheumatologist wanted to put me on Forteo, even though my bone density was actually pretty good. Long story...but I said "no," for a variety of reasons. Perhaps in the future, but I really really really need to think about it.

Recently, I developed neuropathy in one foot. What was very upsetting to me is that my rheumatologist acted like this was weird and unexpected. The neurologist I go to ordered a nerve conduction test and it was fine. The tester said that I should have a small fiber neuropathy test, but the neuro said that I didn't need it at this time and if the neuropathy worsened and I wanted the test than maybe I should have it. :confused: apparently it involves a couple of tiny incisions on your leg.(this neuro is very kind and perhaps he didn't want me to suffer with any more pain at this time).

The Sojgrens society literature talks about neuropathy being rather common for us and also talks about the lack of information/education among medical professionals about SS. Honestly, this has been my experience.

How long will you have to do IVIG? When do you start? Wishing you well.

Thanks to all who replied
 

He told me each treatment is 30K. Does that sound right? I have good insurance, but remember when I had them before, they were about $700 if given in a freestanding infusion center and $2200 if given in the outpatient area of the hospital. That has been quite a while back. I am showing my age here. :)

I don't know when it will start, he didn't say, but I do know the hospital is working through getting the insurance approval.

The osteoporosis drug I took was Atelvia. The doctor says the next step is Prolia, an injectable drug given twice yearly. I have not read much on that. My friend here had a very similar reaction when she took one of the other bisphosphonates. I am now a little gunshy of these drugs, though I was told Prolia works in a different manner...

Cathie

Oops.
 

I do have dry mouth, dry eyes, etc and the blood markers for Sjogren's: SSB antibodies as well as RNP. I also had a positive ANA some time ago.

The doc's response to that was that a lot of people have these markers... Don't know if that is right or not...

Sometimes I wish I could just have a little country doctor who would just keep things simple...

Onto the pain clinic... The doctor there wanted me to go through a multi-specialty clinic, including psych and a bunch of other disciplines. I said thank you very much, but no thank you. I said I could function a lot better if I could just get pain under better control. She wants me to try baby doses of Lyrica, like 25mg... Okie dokey. I am willing to give that a shot.

My dance card is filled up with doctors' appointments once again... :) :) :)

Trying to look on the light side,
Cathie

If you have positive SSB makers, positive ANA & RNP...and symptoms, I don't see the need for a lip biopsy. Your diagnosis should be made on the factors/labs above. Sure, the lip biopsy is the gold standard, but I'd consider it overkill at this point and an unnecessary procedure.

Sure, there may be some with the SSB marker without current/active Sjogren's, but they have a good chance of developing symptoms later on. But since you have symptoms NOW, you should be given the proper diagnosis of Sjogren's without the biopsy.

I have always had negative markers (for everything), but my lip biopsy was very positive and I had all the symptoms. I also have SFN and confirmed damage to the dorsal root ganglia via a new MRI at Johns Hopkins (and suspected autonomic ganglia based upon other findings/diagnosis).

The reason they believe my labs are negative is due to the fact that my immune system (in general) is also deficient. I receive IVIG now, but at a dose for immune deficiency,not SFN. I also got IVIG for 9 years starting back in '97, but it didn't help the neuropathy. It was just as expensive then, as it is now. My infusions run about $10,000 a month. Back in the 90's, it was about $8000 (because it did it at home and it is always less with just a home nurse vs infusion center with doctor and others on staff).

Your infusions will be more expensive because your dose (for SFN ) is higher.

Hey there Vowel Lady :)
 

Sjogren's diagnosis
 

I don't know whether my Rheumatologist is correct in saying 20% of the people diagnosed with Sjogren's actually have inflammatory polyneuropathy or not. He seems to be a very knowledgeable doctor and is also on the faculty at our medical school here.

I have the markers, the dry everything symptoms etc. I just don't have a lip biopsy and think I could do without that. My blood work is repeated constantly, and the markers have been present for years. He is very insistent that my problems are neurological, but says that inflammation is present.

I did see a very kind pain management doctor who had me try 25mg of Lyrica, which did nothing. I am now giving Neurontin a try. THe combination of that along with Flexeril seems to help quite a bit...

Maybe I misunderstood the charge for the IV's. That sounds high compared to the above comment.
Cathie

From what I have found it can be connected.

What is Inflammatory Polyneuropathy? Symptoms? How is it dx'd? One doctor I went to mentioned it. Now I'm very curious. :confused:

Inflammatory polyneuropathy--
 

--more often referred to as CIDP (chronic inflammatory demyelinating polyneuropathy) is an autoimmune condition that is often analogized to a slower, less acute onset of Guillain Barre syndrome.

There is an autoimmune attack on the myelin sheathing of the larger sensory and motor nerves, which results in degraded nerve signalling and a host of symptoms. Typically in CIDP the motor symptoms are greater than the sensory ones, though there are variants in which sensory symptoms are paramount. People with CIDP often notice problems with walking or gross motor control initially, and it is easy to confuse the condition with other neuromuscular diseases.

Given that the mechanism is autoimmune, this is a neuropathy condition that can frequently be treated by immune-modulating therapies such as intravenous immunoglobulin (IVIg) or plasmaphoresis. The trick is getting a doctor who suspects the condition, will do the right testing, and who will go to bat for immune modulating therapy if indicated (such therapies are extremely costly and insurance companies are not usually very cooperative).

See:

http://neuromuscular.wustl.edu/antib...mdem.html#cidp

http://www.gbs-cidp.org/home/cidp/cidp/

http://emedicine.medscape.com/article/1172965-overview

Hi Hopeful! It has been awhile. I have been keeping up to date with posts but did not have anything useful to add.

BUT my new rheumatologist did another set of bloodwork and is convinced that Sjogrens is causing all of my issues. My ANA still positive and SSA still high despite results to the contrary at Johns Hopkins. C-3 low as always.

So, I am dumping all the anti depressants and going back to the classic Sjogrens protocol of plaquenil and gabapentin.

Now, he also wants me to add methotextrate to the mix. I declined due to the side effects but am wondering how helpful it might be for others? I am going to start a new thread for this one. Instead he has me on Alleve twice a day - plus I am still on Tramadol. My poor body needs a detox!!!

Going back to the lip biopsy question from Yorkiemom, I agree wholeheartedly with Hopeful and en bloc. It is uncomfortable, and you have to ask the question of whether it will change your treatment. Some say it is the "gold standard," but SOOO many people diagnosed with Sjogrens have a negative lip biopsy and they continue to be on the same medication protocol. There are many uncontrollable variables that can result in a negative biopsy (inadequate sample, inaccurate tissue sample, etc.).

The IVig did not help me (had 6 infusions over a 4 month period), and yes it is expensive, but now that I am not getting it my symptoms are getting worse. It is hard to tell if it is a coincidence! Many people swear it is a miracle worker so if you can swing it it cannot hurt right? You can also try appealing the insurance company decision so that they will cover it if that is the issue...

I have Sjogren's going on 22 years now--no lip biopsy, just all the symptoms (dry everything) and blood markers-SSA of 900 4 1/2 years ago, 366 now, even after daily Plaquenil (200mg, now 300mg).

I just convinced my rheumie to give me Low Dose Naltrexone. Too soon to say if it will help on the bloodwork, but I'm impressed so far. I have Peripheral Neuropathy and fibromylgia, among a lot of other ailments (notably, Lyme disease). Recently, I was in the midst of a major Fibro flare (leg pain through the roof). A couple of hours after taking the first dose of LDN, the pain dissolved..thank goodness! and hasn't reappeared. I'm also noticing that it is handling the PN, but not as well as the dextromethorphan did. (I backed off the DM to 15 mg to see if LDN could handle the PN on its own-it cannot.)

As for the DM, I've been experimenting with it for back pain, which I suspect is Ankylosing. It works fabulously! I was up to 150 mg before the LDN, now I've backed off to 60 with good results (going down to 15mg daily renewed the back pain, so I quickly went back up to 60mg).

What was surprising to discover was that the DM could handle the PN all by itself. I had been taking 50 mg Topamax for it with good results, but once the DM started handling the back pain, I stopped the topamax to see if I still needed it and was pleasantly surprised to discover I did not. :) I also found that I no longer needed the daily dose of Alleve to handle the tendonitis pain I developed in my achilles tendon and right elbow, or the twice daily Tylenol to handle the other aches and pains. Just DM twice daily (in the form of mucinex DM)--now once daily.

DM is totally experimental, but I find it beneficial. The LDN is also experimental--my rheumie really didn't know anything about it until I gave him an article explaining its benefits--but he was gung ho and I'm pleased so far, especially since the DM definitely does NOT handle full blown Fibro flare pain.

Good Morning Yorkiemom :)
 

Hello
 

Merry Christmas. I see I have again been remiss in keeping up with posting, something I said I would not do.

The latest thing going on here is that I am scheduled to get a new pacemaker on Christmas Eve. Some Christmas present...

This is a bi-ventricular model with a coating that is supposed to be hypoallergenic made from Parylene. If anyone has heard anything about this, please post. I am dreading it because I am so sensitive to so many materials that normal people don't seem to have problems with.

The main med that helps keep symptoms at bay is daily low dose Prednisone, something I thought would fend off developing osteoporosis by keeping it low.That is not a given, for sure. Last week, I was diagnosed with severe osteoporosis. I am hoping to avoid some of the osteo drugs.

I also had the punch biopsy from Johns Hopkins. Two of the sites biopsied were normal, the third (near the ankle) indicated the presence of a neuropathic process. I might be wrong on the wording, but I am assuming this confirms the diagnosis of small fiber sensory neuropathy.

Last night, we went to dinner and I wore a pair of blue jeans containing Spandex. For a while, I had been able to tolerate it. Not now!!! The pain in the bottoms of my feet traveled to my waist and when we came home, I immediately removed them and the pain began to dissipate slowly. I do not know what to make of this, other than the neurologist who did the punch biopsy said that she had patients who were so sensitive, they could not tolerate their bedsheets touching them. Early on, I shared that problem. This sensitivity seems to wax and wane.

Hope all are doing better.

Merry Christmas or Happy Holidays!

Yorkiemom
Cathie

Glad to hear from you.
 

And yes, if that skin biopsy report indicated there was some sort of neuropathic process going on at one site (which site), that would be consistent with at least some form of small fiber neuropathy. And people with small fiber neuropathy can be extraordinarily sensitive to both actual mechanical touch and "phantom" touch; there is a phenomenon with damaged nerves that are malfunctioning called "delayed summation" in which the nerve fiber fires long after a stimulus is removed, and the sensation seems to build over second to minutes, sometimes intolerably, before eventually dying back down.

Some people become so sensitive with small fiber syndromes that they cannot tolerate clothing, breezes, bathing . . .and/or some have spontaneous burning and other sensations when there are no stimuli at all. Damaged nerves do all sorts of things the brain has difficulty interpreting . . .

Do post here whenever you feel up to it; we do worry about long absences. (I get all sorts of messages when I don't post for a while.)

Jeans are the worst, especially if there is a lot of sitting involved. Although I am old enough to know better I am young enough to be vain and want to wear fashionable "skinny" jeans. The adjective applies to the jeans and certainly not to me, but even in a super soft denim advertised as comfortable I am dying by the time I get a chance to change. I have a couple pair I can tolerate when it really matters to me, otherwise I live in three identical pair of grey Eddie Bauer cotton knit pants. They are soft, don't cling or bag, come in tall, and don't collect dog hair. (We have a German Shepherd who thinks she is a lap dog and a pug.) The pants are hideous and only come in dark grey. If I am going outside for more than a few minutes I wear long underwear and fleece lined pants.

There are several threads on here about clothing for sensitive skin. For me it is my left thigh that reacts to anything touching it. Skirts are better, especially on long car rides if I want to look presentable.

Did you end up doing the IVIGs? I have some kind of autoimmune disorder that is triggered by trauma. Back in 97 I broke my leg and soon after had burning sensations on my skin on upper body and after that weakness. In was diagnosed with bi lateral Bachial Plexitis. Everything returned within a year to normal except for a parylized left diaphragm. Then in 05 I had a major disc herniation which required a fusion. Soon after I developed severe weakness which was diagnosed as CIDP. Was using a walker for 3 mos. They put me on IVIG's every 3 weeks for about 5 months. It did help but it was very gradual. They were $30,000 each back then I was told. At the beginning of one of my treatments the nurse was trying to hang the large glass bottle of IVIG onto the IV tree and she lost her grip. The bottle hit the floor and broke. Very expensive oops. The worst part was when they mix that stuff it has to sit for 1-2hrs before infusion because its frothy. I pretty much spent the whole day there.

Sjogren's or something else
 

I have not posted on here in quite a while. Life pulled me in other directions (raising an infant to a now almost 5 year old). On the Sjogren's, I would advise any one with a positive ANA or lip biopsy to explore an autonomic disorders physician.

I was told I had Autoimmune Autonomic Ganglionopathy, AFTER about a dozen different diagnosis. THAT is the correct diagnosis and I still do fit most of the diagnostic criteria for Sjogren's except for positive SSA or SSB.

I think there are a LOT more of us out there with AAG than any one comprehends because most of us never make it to an autonomic disorders clinic. After 3 years on IVIG I stopped it due to my kidneys trending south and I didn't see enough improvement on IVIG, however there are other treatments, such as plasmapheresis. IVIG tends to be tougher as you age. I am on low dose prednisone right now.

If you have a diagnosis of SFN or PN and have dry eyes, dry mouth, vision issues, severe headaches, especially 'coat hanger' headaches or 'migraines', insomnia, bloating, swallowing issues, constipation, feeling very full after not eating much, heart palpitations, chills, hot flashes, inability to sweat or adapt to either heat or cold temperatures and 'chronic fatigue syndrome'......you may have autonomic issues. The dry eyes and dry mouth tend to put people into the Sjogren's diagnostic work up and when nothing is found by rheumatologists, they don't seem to understand that a neuro referral is needed and the patient is discharged or frustrated enough to leave and not get treatment. I have found very few rheumatologists who are informed on neurological issues.

AAG is a different entity than Sjogren's but the symptoms are very similar in terms of pain, dry eyes and dry mouth. The hypotension of AAG, will eventually leave you with little or limited ability to be upright.

Yorkiemom,

I agree with Cyclelops about AAG. I have both dorsal root ganglionopathy as well as autoimmune autonomic ganglionopathy...causing peripheral neuropathy and severe autonomic symptoms. I too have a pacemaker (since 2004) and recently had a new one placed last year. My NMH (neurally mediated hypotension) is at the point of severely limiting my ability to be upright at times. It requires life-style adjustments as well as medications.

I can no longer use steroids (even low dose) as I have Cushing's syndrome from years of taking steroids either by IV or high & low dose orally. So I currently receive high dose IVIG monthly.

I have had plasmapharesis, but it is last resort type treatment and not effective or appropriate to do this every couple days for long term help...but it can/will help for acute or emergent situations.

The skin sensitivity (allodynia) can be from the reduced small nerve fibers (SFN). I don't think there is much to be done for this (other then finding materials that don't bother you so much). The magnesium lotion MrsD suggests for PN symptoms may help...worth a try.

My days on prednisone are limited too...been on too long and it shows. :/ I'm in the process of weaning off, but I just weaned off pain pills too, so it has been a slog. AAG doesn't really give a person a lot of options with medication...Every pill you put in your mouth affects the neurological system and with AAG, it can cause profound issues. (Makes me wonder if they cause AAG?) It's been a long slow slide...with a way to go yet. I'm still up and around. I can walk a mile or even two on some days. I can do a treadmill for a short time. I can not do housework much...little bits, now and then. I could not live independently in my current setting. :/ Hubby is home all the time and we are still raising my grandson, who is now 5. (Had him since he was 6 months.)

As to what causes this, who knows? Until we really organize, raise money and raise awareness, we won't know much and people, especially women, will continue to suffer and die from this while rheumatology and neurology chase each other's tails.

I'm done being a guinea pig for a while, until they have some concept of what they are doing.