Jan 8, 2012

Why is IVIG not listed as a potential POTS treatment?

Those of us with POTS and some medical research savvy have come across the POTS 101 journal articles written by doctors who are considered POTS experts.  They have written these summary articles for other doctors, who don't specialize in POTS, so they know what to do when a POTS patient walks in their office.

In these POTS diagnosis and treatment summaries, there is usually a list of medicines that have been used to treat POTS with varying success.  There are lots of disclaimers in these articles, noting that some of the medicines work better with some forms of POTS, and other medicines are only used in refractory POTS that can not be treated successfully with the first line medications.  Of all of these POTS summary journal articles, I have never seen any mention of IVIG.  Perhaps that is because the doctors writing this articles are usually cardiologists, and as such, they tend to focus on drugs that impact the heart (beta blockers) and the rest of the cardiovascular system through various mechanisms (midodrine, SSRIs, Flourinef, Clonidine, etc.).  

Like many POTSies, I went the cardiologist route at first, since the my most obvious symptom was the insane endless tachycardia I was experiencing.  The cardiologist, as expected, put me on beta blockers.  That only made me sicker, because beta blockers drop your blood pressure and make you very tired.  They are also not good for asthmatics and can make Epi-Pens not work, so if you carry an Epi-Pen for severe allergies, don't take a beta blocker without discussing the Epi Pen issue with your doctor!  A few cardiologists later and I was still being told to try more beta blockers.  Then I learned enough about POTS to realize that I needed a neurologist who specialized in autonomic neuropathy.  If you think it's hard to find a cardiologist who knows about POTS, just try finding a neurologist who specializing in autonomic neuropathy.  I think there are literally less than 50 neurologists in the whole country who legitimately specialize in that field.  I even went to two neurologists in NYC who claimed to be autonomic experts, but I knew more about the physiology of POTS than they did and they made no recommendations other than exercise and "just get used to being sick."  Seriously?  Thankfully I found some real autonomic neurologists at Cleveland Clinic, and then later at Columbia University in NYC.  

As I have discussed many times on this blog, one of the neurologists at Cleveland Clinic figured out the cause of my POTS.  I have an autoimmune disease that attacks my autonomic nerves (and a bunch of other nerves and organs too).  They recommended that I get high doses of IVIG (intravenous immunoglobulin immediately, to prevent any further nerve and organ damage, and to hopefully reverse that damage that had already occurred and that had caused my disabling POTS symptoms.

The more I began to post about this on this blog and on Facebook, the more people responded and said they were getting IVIG for their POTS symptoms too, because they also had some autoimmune problem causing their autonomic neuropathy.  Just from chatting about it on this blog and Facebook, I have found at least 10 women getting IVIG to treat their autonomic neuropathy/POTS symptoms.  They have Sjogren's, Lyme DIsease, CIDP or Guillane Barre as the root cause of their POTS symptoms.

Then I started poking around on the Sjogren's patient group pages, asking if any of the Sjogren's patients have autonomic neuropathy or if any of them frequently pass out and have tachycardia.  Within a few days, I had 20 women e-mailing me saying they've been passing out or almost passing out nearly daily for years, they have terrible tachycardia and their doctors can't figure out why - and since they have Sjogren's their doctors are usually rheumatologists, not cardiologists or neurologists.  So I told all of them about POTS and encouraged them to see neurologists and EP cardiologists.  So far, three of them got back to me and were diagnosed with POTS!!!  This is after years and years of suffering with POTS symptoms and not knowing it was called POTS and not knowing it was probably caused by Sjogren's.  So now these ladies are headed back to their rheumatologists and demanding the proper drug therapies to address the autonomic neuropathy - high dose steroids and/or IVIG, and if all else fails, Rituxan.  Just like many cardiologists aren't too familiar with POTS, many rheumatologists don't seem to know that Sjogren's can cause autonomic neuropathy, since it is relatively rare within the Sjogren's population.

Then the Sjogren's Syndrome Foundation just had an article in their Moisture Seekers newsletter about a young woman who developed acute polyneuropathy after a viral infection, which then turned into Sjogren's Syndrome.  She was getting high dose IV steroids and IVIG and was getting her life back.  This is the same thing I have, although it took my doctors a year and a half to figure it out.  My neuropathy is not just autonomic, it's sensory and motor too, and possible some facial nerves are involved since I have terrible pains shooting up the side of my face/forehead sometimes.  My doctors are continuing to unravel my diagnosis and figure out everything this Sjogren's mess has caused.

So if I can find 20+ women with POTS and autonomic neuropathy getting IVIG just from some basic online conversation, why isn't IVIG mentioned as one of the drugs that may be helpful to POTS patients?  I'm going to guess it's because it's so expensive and/or the doctors writing those articles aren't that familiar with autoimmune neuropathies - not a popular medical specialty.

So I have decided to do something about this.  After I compile some more anecdotal evidence of people I've met with POTS getting IVIG, and after I've organized my journal research on the use of IVIG for idiopathic dysautonomia (essentially another name for POTS with no known cause), I am going to write a formal and polite letter to each of the doctors who have written the POTS summary articles.  Hopefully the next time they update their POTS summary articles, they will include autoimmune testing in their recommendations for a POTS diagnostic workup, and they will include IVIG as a possible treatment option for some POTS patients.

Also, it seems to me that so many POTS patients either developed POTS after a viral infection and/or they have autoimmune tendencies (Celiac, thyroid problems, vasculitus, skin rashes, hair falling out, etc.).  I would argue that some of these patients should be given a trial dose of IVIG to see if it would help.  I know it is quite expensive, and insurance companies probably won't pay for a trial dose for an unknown autoimmune condition.  However, having been bedridden with horrific dysautonomia for over a year, I would have tried ANYTHING to get better, and I am sure many POTS patients feel the same way.  There are several published case studies and other reports documenting the success of IVIG for idiopathic dysautonomia.   I would like to see more research in this area. 

In the meanwhile... let's spread the word about IVIG as a potential treatment option on the POTS boards and the Sjogren's boards, etc.  Power to the patients! :)