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! :)
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! :)
Fantastic post! I'm definitely going to research this with you!! Thanks for the help and ideas!
ReplyDeleteIVIG runs about $4000 a pop. Most doctors consider it to be used as a "last resort". IVIG is made from blood of blood donors and is considered risky in itself. Whenever you recieve blood products there is a big risk.
ReplyDeleteIn response to the Anonymous post above...
ReplyDeleteActually, IVIG is much more than $4000 per dose. The price varies based on supply and demand (and market manipulation by big pharma) but my doses have cost between $10,000 and $16,000 per monthly dose. However, if you have had full blown POTS and you are spending weeks at a time in the hospital, you are totally bedridden and taking dozens of different medicines trying to deal with your symptoms, $10,000 a month to feel really great is cheap, and for some people, definitely worth it. It's all relative. If you only have mild POTS and you can still work and socialize and do "normal" things, then spending that much money probably doesn't make sense. Just like any drug, the pros an cons have to be considered on a case by case basis.
Same thing with the risk. I think you oversimplify and overstate you case when you say "whenever you receive blood products there is a big risk." IVIG has been used safely on millions of people over the past 30 years. It is tested for hepatitis, HIV and other known dangerous pathogens. It is also heat treated, acid washed, etc. Yes, of course there is a risk, but I wouldn't consider it to be "big." An again, the risks and benefits have to be weighed on a case by case basis. I am not saying IVIG is for everyone. I am just saying, why is this drug not even being mentioned in the POTS literature as a treatment option, even if it's only mentioned as a drug of last resort? Let's at least get this info out there in terms of a treatment for some POTS patients, so they can disucss the risks and benefits with their doctor. For me, the risk is worth it. My symptoms were unbearable and not responding to other drugs, therapies, dietary changes, etc. And since I have had 2 doses of IVIG, I went from bedridden and symptomatic 100% of the time, to able to go out to the food store and the movie theatre and cooking up a storm for hourseguests. My life is so much better because of IVIG, and I just want the rest of the POTS patient population to have access to that drug if they and their doctors deem it appropriate - but most people, including doctors, don't know about it and don't know enough about POTS to realize IVIG may help some cases of POTS.
I would bet good money that in a few years, this will be a more standard treatment for POTS.
Hi,
ReplyDeleteI just found your site by googling Lyme disease and IVIG therapy. I have lyme disease and dysautonomia....took me about 13 yrs to get a diagnosis of Lyme and dysautonomia. Most of us with Lyme seem to have Orthostatic hypotension or POTS! It would be so greatly appreciated if you could put links to the papers which recommend ivig in dysautonomic nueropathy on your site? I am now contemplating going to Columbia or Cleavland clinic...I know what I have was triggered by lyme, what I dont know is whether or not I have a persistent infection or auto-immune disease(The jury is still out on that one).
Dr. Mariam Katz has recently recommended ivig in Lyme Disease but the chances of it being covered at this point is slim. Thank you so much for this information! It could help so many people. Any other info you could give me would be great.
Thank you,
Melissa :)