Johns Hopkins visit

[Jocelyn]

Just an update on looking for my diagnosis.  Just came back yesterday from Hopkins and waiting for results.  I did visit the Sjogrens clinic there and did all the routine tests.  I would say I was impressed with the interest they take in autoimmune disorders and well as how thorough they were. 
I was wondering if anyone here who is diagnosed with any kind of autonomic dysfunction got the diagnosis?  Is it through your rheumy or neuro?  What tests are intially done to detect this?  I did have the small fiber skin biopsy done but I just wandered if it is based on that result or a further investigation into the symptoms.  At the time of the test, of course, I did not have any pins and needles feeling or weakness.  I wander if it detects it even then?  I guess these are all questions I could have asked the doctor, but as you know, you go over SO MUCH as a new patient and you are very eager for answers that you don't always get everything in.  One question can lead into many different areas of an answer from a doctor. 
I did participate in a research study for Hopkins by donating extra blood for people with Sjogrens antibodies, even after they had take 36 tubes for my own testing.  I was amazed that they could run that many tests. 
Any help with an answer to the whole autonomic thing would be appreciated.  I did read over the articles once again on the forum about the CNS and all the involvement but sometimes I like to ask the actually people experiencing it. 
Thanks,
Jocelyn

[Epson]

Jocelyn,

Mine was diagnosed by the connect the dots meathod, all symptoms led one conclusion, Autonomic dysfunction.  Having IBS which is believed to and autonomic disorder and my Holter study coupled with the fact that I have SS led the heart specialist to the conclusion that there is no doubt that there is an autonomic disturbance going on.

I hope this makes sense, I'm having brain problems today

[inga]

I had a, + lip + skin biopsy for SFN, + ANA and all the autonomic tests showed autonomic dysfunction.  All this took 6 years.

A neuro did things orignially and then a rheum was involved later.  A neuro still orders my treatments, in conjunction with the rheum....but, in all honesty, the neuro is more primary.

[Jocelyn]

Inga,
And in the 6 years, how much did this progress?  Lung involvement?  What treatment stops or helps with further damage?
Jocelyn

[anita]

Jocelyn,

The neurologist usually diagnose autonomic dysfunction, because it's actually commonly referred to autonomic neuropathy.  It is the brain's lack of communication and regulation of basic involuntary functions that create the problem...and a neurologist handles brain related problems.

Some testing includes:

Holter--to check for inappropriate low or high heart rates
BP monitoring (24hr) for fluctuations (posturally)
tilt table--neurally mediated hypotension (postural)
gastric empty test--for gastroparesis
sweat test: very messy but self explanatory--to check if you sweat properly.

Some is as others have said...connecting the dots.  Urinary retention or have to manually push to urinate is another autonomic dysfunction but doesn't require a test...simply the doc asking the appropriate questions.  IBS or even incontinence problems can (but not always) be associated.  If testing is positive for several of the above, you can usually rest assured that an autonomic neuropathy is affecting other functions.

Hope this is helpful.  Talk to your neurologist.  Some larger academic hospitals have specific autonomic testing dept.  Sinai in Baltimore is one, Mayo is another.  But any can test for the basics...heart rate, BP and stomach empty problems.

Anita

[mom16]

Hi,

I also have been to John's Hopkins and was diagnosed with Sjogren's after getting a small fiber skin biopsy there.  I do not have the common dry mouth or dry eye symptoms, but have the pins and needles and deep burning ache that goes along with small nerve damage.  I was not feeling those symptoms at the exact time of the test, but it still came up positive.  I was diagnosed based on the results of that test, my bloodwork (I believe elevated SSA and ANA)  and  other symptoms such as stiffness in my hands in the morning.  I was lucky that my doctor (Dr. Birnbaum) was able to look outside the box and see that my neurological symptoms, although not as common, did lead to sjogren's.  I also had a lip biopsy which was moderate inflammation and, according to him, wasn't enough to show that I had a problem with dry mouth (which I knew anyway).
I hope this helps.

Karen

[Dolly Dimples]

  I'd like to know that if a patient is diagnosed with an Autonomic Dysfunction,
    what is the treatment, if any..
              I suffer with the cold badly, and I understood that to be Autonomic.
                     but I have  never been actually diagnosed with anything other than acute sensitivity..
                                                                                  Dolly 

[dbaratta]

Hi:  Just another Johns Hopkins patient (Dr. Birnbaum).  I am diagnosed primary SS, sero negative, lip proven biopsy, positive Radiological saliva study, positive ANA, negative Sjogren blood tests.  Had skin biopsies at JH which were somewhat positive--he said that I have small fiber neuropathy.  Had EMG, positive for cervical radiculopathy affecting my arms and legs.  Referred to a Neuro closer to home (NJ) who saw that my gait/walking/balance are all really off.  Had ear test (loud ringing both ears, hearing loss/balance disturbance, etc.) -- the ENG was positive, so on to 2 other doctors.   Have had autonomic neuro problems for several years -- still having them but they are less important at this point than the other nasty things going on.  An ENT wants head/brain MRI's, and so many other tests I can't even think straight tonite - probably not important anyway.  This SS has sure set me back on my heels.  Anyway, I have to travel 3 hours each way to get to JH but am glad I went.   They are extremely thorough and I am also in the research projects. Jeeze, we should form a club.  Keep me posted.  I'll do the same.  Diane

[anita]

Hi all,

I'm another patient of Dr. Birnbaum at Hopkins.  It doesn't surprise me that there are several of us posting here...and many more out there for sure.

To answer Dolly:  Treatment is symptom specific.  There is not a single drug to help "autonomic neuropathy" itself.  However, many meds available for some of the symptoms like heart rate, BP or gastroparesis.  Unfortunately, I don't think there is one for the temperature control.  Dr. Birnbaum does lots of research so maybe one day there will be a magic drug for temp issues...or for progression of sjogren's itself.

Anita

[susanep]

What meds were those of you put on that either seen Dr.Bimbaum or whoever at John Hopkins?
Just curious.

susanep

[anita]

Hi Susanep,

I take a vast array of meds for the autonomic neuropathy:  Florinef, midodrine, and acebutolol (all for the heart or BP), then Nexium, Domperidone, and miralax (for the gastroparesis and GERD).

I also take innohep (heparin injections) for the APS, asthma meds, and several specific vitamins, for nerve function and increased homocystine levels.

Dr. Birnbaum is the neurologist for the autonomic neuropathy, but each organ or related function should be treated by the specific specialist...a cardiologist Dr. Birnbaum recommended at Hopkins treats the heart related issues, GI for those issues, etc.  Dr. Birnbaum only orders the plaquenil for the Sjogren's itself and IV solu-medrol during a bad flare or has used Cellcept.

Anita