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Some General Information on Prognosis
There have been several prognosis questions recently, so what follows is my attempt to speak to those questions without speaking to any individual person's prognosis, which is impossible for me to do. I hope this is helpful!
Disclaimer: This is not medical advice. This is not meant to be, nor should it be construed as, prognostic information specific to your case or in applied to your own case. The best place to find out about your individual prognosis is from your own physician and/or an autonomic expert who is well-versed in your individual case.
That being said, here are some very general things that have been noted in research on dysautonomia. There are only two forms of dysautonomia known to shorten a person's life-expectancy; both of these are (fortunately) very rare. One of these, familial dysautonomia [FD], is congenital (present from birth) and basically (anomalous cases have happened, but that's so rare compared to the human population it's almost not a consideration) only occurs in children where BOTH parents are of Ashkenazi Jewish heritage. The other, multiple system atrophy, generally onsets in the later decades of life and can sometimes be diagnostically confounded with Parkinson's.
Those aside, one thing to realize is that some forms of dysautonomia are primary and some are secondary. A secondary dysautonomia means that the autonomic dysfunction is a "feature" or an "effect" of some other underlying disease, condition, toxicity, etc. I do not have room here to list every form of dysautonomia, but I'll try to hit some highlights to paint a general picture. Here I'm using the classification system put forth by Prof. Christopher Mathias in his texts (top autonomic doc in the UK, one of the top autonomic specialists in the world).
There are a few acute primary dysautonomias, one example being Pure Cholinergic Dysautonomia; chronic primary dysautonomias are represented by Pure Autonomic Failure and MSA, for example. The bulk of dysautonomias are secondary. With secondary dysautonomia, the thing to keep in mind is that the prognosis of the dysautonomia is dependent upon the prognosis of the primary diagnosis. As an example, take Vitamin B12 deficiency: usually, replacement therapy will alleviate the orthostatic hypotension. So sometimes in secondary dysautonomia, if you can find the cause, you can simply treat it and the dysautonomia symptoms will entirely resolve. However, familial dysautonomia (hereditary sensory and autonomic neuropathy type III) is also considered a secondary dysautonomia, and so far there is no cure for this; on the bright side, supportive treatment for FD has improved considerably over the last 5 decades or so, such that people with FD are now reaching adulthood and even having children of their own (because it is a recessive gene, their children do not get the disease, though they will be carriers of the gene).
In terms of POTS, if you've read my "POTS is a syndrome not a disease" speech 90 times before, skip this paragraph. If not, here we go ... POTS stands for postural orthostatic tachycardia SYNDROME and the word "syndrome" has a special meaning in medicine. In short, doctors know that there are a set of signs (objectively observable and measurable phenomenon like blood pressure and temperature) and symptoms (subjective phenomenon that only the person who experiences them can report like nausea, dizziness, and fatigue) that tend to occur together in a specific pattern in patients. However, they call it a "syndrome" because it doesn't meet the criteria for disease in one important way: doctors and researchers have not identified an underlying cause. Now, that leaves a question waiting in the wings. Is there ONE cause that leads to all cases of POTS and they're just not finding it, or are there lots of different causes that can lead to this same syndrome? Some experts in POTS have spoken up in favor of the multiple causes hypothesis, others are still looking for a cause or waiting for more research on POTS. One cause of a relatively small number of POTS cases has been identified in NET (norepinephrine transporter) deficiency, but this speaks to such a small portion of POTS cases that it is only the tip of the iceberg in terms of finding an answer to what causes POTS.
Still, even without knowing the exact cause of POTS, or even whether all POTS patients have the syndrome due to the same underlying cause, some observations about prognosis have been made. One important note is that, for the purpose of the case studies on prognosis in POTS (at least the ones I have seen), researchers and physicians consider a patient to have made a "recovery" if the patient is able to return to functioning in life (participating in work, school, etc.), even though this may include having made some lifestyle adjustments to allow this to occur. Which is to say, there are a number of patients who find that this definition of recovery is not in line with their own definition of that word (or hopes for recovery), which may be more along the lines of being returned fully to the pre-disease state (i.e. "cured"). It is also important to note that because POTS is a relatively "new" condition (having only been recognized and named by doctors recently, even though it surely *existed* prior to that, and indeed seems to have possibly been treated under other names in some cases historically), we just don't have as much long-term data on POTS patients as we do about patients with other diseases. Without that data, our understanding of prognosis will be necessarily weak.
That being said, here's some information on POTS prognosis. A number of patients do make a recovery (I'm not willing to hedge bets on the percentages they throw around, because I don't think the data is sufficient to support it), generally within 5 years of onset. Patients most likely to make a recovery are those with childhood onset POTS. (Anecdotally, I've heard of recoveries in childhood onset POTS cases after spans longer than 5 years; part of this may be that doctors are getting better at identifying POTS in younger children so it's taking relatively longer to "outgrow" from time of official diagnosis. It's hard to say.) Some report that viral onset POTS patients are more likely to make a recovery than other subsets as well. Those with JHS/EDS generally do not have as high of a likelihood of recovery. (Personally, I would class this under a Secondary Dysautonomia, and thus it would follow the rule of the dysautonomia following the prognosis of the primary disease, that being the Joint Hypermobility Syndrome.)
Please feel free to follow up with any questions and I'll try to answer to the best of my ability. If anyone has questions about a dysautonomia I didn't cover (or skimmed over) here, let me know. Thanks!
Disclaimer: This is not medical advice. This is not meant to be, nor should it be construed as, prognostic information specific to your case or in applied to your own case. The best place to find out about your individual prognosis is from your own physician and/or an autonomic expert who is well-versed in your individual case.
That being said, here are some very general things that have been noted in research on dysautonomia. There are only two forms of dysautonomia known to shorten a person's life-expectancy; both of these are (fortunately) very rare. One of these, familial dysautonomia [FD], is congenital (present from birth) and basically (anomalous cases have happened, but that's so rare compared to the human population it's almost not a consideration) only occurs in children where BOTH parents are of Ashkenazi Jewish heritage. The other, multiple system atrophy, generally onsets in the later decades of life and can sometimes be diagnostically confounded with Parkinson's.
Those aside, one thing to realize is that some forms of dysautonomia are primary and some are secondary. A secondary dysautonomia means that the autonomic dysfunction is a "feature" or an "effect" of some other underlying disease, condition, toxicity, etc. I do not have room here to list every form of dysautonomia, but I'll try to hit some highlights to paint a general picture. Here I'm using the classification system put forth by Prof. Christopher Mathias in his texts (top autonomic doc in the UK, one of the top autonomic specialists in the world).
There are a few acute primary dysautonomias, one example being Pure Cholinergic Dysautonomia; chronic primary dysautonomias are represented by Pure Autonomic Failure and MSA, for example. The bulk of dysautonomias are secondary. With secondary dysautonomia, the thing to keep in mind is that the prognosis of the dysautonomia is dependent upon the prognosis of the primary diagnosis. As an example, take Vitamin B12 deficiency: usually, replacement therapy will alleviate the orthostatic hypotension. So sometimes in secondary dysautonomia, if you can find the cause, you can simply treat it and the dysautonomia symptoms will entirely resolve. However, familial dysautonomia (hereditary sensory and autonomic neuropathy type III) is also considered a secondary dysautonomia, and so far there is no cure for this; on the bright side, supportive treatment for FD has improved considerably over the last 5 decades or so, such that people with FD are now reaching adulthood and even having children of their own (because it is a recessive gene, their children do not get the disease, though they will be carriers of the gene).
In terms of POTS, if you've read my "POTS is a syndrome not a disease" speech 90 times before, skip this paragraph. If not, here we go ... POTS stands for postural orthostatic tachycardia SYNDROME and the word "syndrome" has a special meaning in medicine. In short, doctors know that there are a set of signs (objectively observable and measurable phenomenon like blood pressure and temperature) and symptoms (subjective phenomenon that only the person who experiences them can report like nausea, dizziness, and fatigue) that tend to occur together in a specific pattern in patients. However, they call it a "syndrome" because it doesn't meet the criteria for disease in one important way: doctors and researchers have not identified an underlying cause. Now, that leaves a question waiting in the wings. Is there ONE cause that leads to all cases of POTS and they're just not finding it, or are there lots of different causes that can lead to this same syndrome? Some experts in POTS have spoken up in favor of the multiple causes hypothesis, others are still looking for a cause or waiting for more research on POTS. One cause of a relatively small number of POTS cases has been identified in NET (norepinephrine transporter) deficiency, but this speaks to such a small portion of POTS cases that it is only the tip of the iceberg in terms of finding an answer to what causes POTS.
Still, even without knowing the exact cause of POTS, or even whether all POTS patients have the syndrome due to the same underlying cause, some observations about prognosis have been made. One important note is that, for the purpose of the case studies on prognosis in POTS (at least the ones I have seen), researchers and physicians consider a patient to have made a "recovery" if the patient is able to return to functioning in life (participating in work, school, etc.), even though this may include having made some lifestyle adjustments to allow this to occur. Which is to say, there are a number of patients who find that this definition of recovery is not in line with their own definition of that word (or hopes for recovery), which may be more along the lines of being returned fully to the pre-disease state (i.e. "cured"). It is also important to note that because POTS is a relatively "new" condition (having only been recognized and named by doctors recently, even though it surely *existed* prior to that, and indeed seems to have possibly been treated under other names in some cases historically), we just don't have as much long-term data on POTS patients as we do about patients with other diseases. Without that data, our understanding of prognosis will be necessarily weak.
That being said, here's some information on POTS prognosis. A number of patients do make a recovery (I'm not willing to hedge bets on the percentages they throw around, because I don't think the data is sufficient to support it), generally within 5 years of onset. Patients most likely to make a recovery are those with childhood onset POTS. (Anecdotally, I've heard of recoveries in childhood onset POTS cases after spans longer than 5 years; part of this may be that doctors are getting better at identifying POTS in younger children so it's taking relatively longer to "outgrow" from time of official diagnosis. It's hard to say.) Some report that viral onset POTS patients are more likely to make a recovery than other subsets as well. Those with JHS/EDS generally do not have as high of a likelihood of recovery. (Personally, I would class this under a Secondary Dysautonomia, and thus it would follow the rule of the dysautonomia following the prognosis of the primary disease, that being the Joint Hypermobility Syndrome.)
Please feel free to follow up with any questions and I'll try to answer to the best of my ability. If anyone has questions about a dysautonomia I didn't cover (or skimmed over) here, let me know. Thanks!
Katy,
I understand completely your desire to return to normalcy. Have you seen Mayo's video on POTS; I think it addresses POTS in youths in particular, and may be helpful for you to watch and further understand that there is definitely hope that you will return to normalcy again.
http://www.youtube.com/watch?v=iJ9bv7jx-Ls
If you haven't checked them out, you might also like to follow this group of young "potsies" that made a youtube channel to reach out to each other and other young people with POTS:
http://www.youtube.com/user/TeenPotsyProductions
And, I hope it goes without saying, we definitely understand how you feel here!!! So feel free to post any and all questions you have, and vent when you need to. Hang in there!
HUGS, Heiferly.
I understand completely your desire to return to normalcy. Have you seen Mayo's video on POTS; I think it addresses POTS in youths in particular, and may be helpful for you to watch and further understand that there is definitely hope that you will return to normalcy again.
http://www.youtube.com/watch?v=iJ9bv7jx-Ls
If you haven't checked them out, you might also like to follow this group of young "potsies" that made a youtube channel to reach out to each other and other young people with POTS:
http://www.youtube.com/user/TeenPotsyProductions
And, I hope it goes without saying, we definitely understand how you feel here!!! So feel free to post any and all questions you have, and vent when you need to. Hang in there!
HUGS, Heiferly.
Thanks for all that good info. Well Im going to do some research on that. My doctor told me I had POTS and I asked him what is that and why me? Thanks for clarifying that for me better than my doctor did. I just wish I had a normal life like most teenagers.
Katy 174
Katy 174
Whether POTS can be secondary depends a bit upon who you talk to. I don't think there is medical consensus on this yet among experts in the field; but do realize that the reason for this is mostly SEMANTICS. That is to say, it has to do with whether you continue to say a patient has the diagnosis of "POTS" after you find an underlying cause for their autonomic dysfunction or whether you just *change* their diagnosis from POTS to the cause you have found at that point. One can look at it either way: that the patient still has the cluster of signs/symptoms that make up POTS, thus still meeting the diagnostic criteria for POTS, and thus the new condition should be diagnosed as primary and the POTS secondary to that ... OR ... that by definition POTS is a syndrome which implies NOT KNOWING the underlying cause, and therefore once an underlying cause is found, the diagnosis of POTS is superfluous, redundant, or nonsensical. To whit, whatever the primary diagnosis is may very well imply the signs and symptoms of POTS itself, making the secondary diagnosis of POTS superfluous and unnecessary.
In short, some doctors will diagnose [such and such disease] with secondary POTS, where others will drop the POTS diagnosis entirely when [such and such disease] is found. It depends on the doctor/clinic and their view of how diseases should be named as much as anything about your body/condition. I hope that makes sense?
All that being said, there are diseases/conditions/syndromes that we know can sometimes cause "secondary POTS" (or if you prefer "autonomic dysfunction" ... for those not in the "secondary POTS" camp). I've already mentioned NET deficiency, but that is as I said exceedingly rare. Much more common is joint hypermobility syndrome/Ehlers-Danlos syndrome. Some autoimmune diseases have been known to cause patterns of autonomic dysfunction that fit the POTS criteria (e.g., Sjogren's).
(As a sidenote, some of the tests they generally run in a workup for a person with suspected autonomic dysfunction are to look for different causes of autonomic dysfunction; however some are to rule out conditions which have similar symptoms to autonomic dysfunction but are something else.)
PAF, MSA, and their ilk are primary autonomic failures. The autonomic nervous system fails and THAT FAILURE in and of itself is the underlying disease/dysfunction. No deeper, more rudimentary cause can be found by doctors or researchers (at least not yet). In short, they don't really know *why* the autonomic nervous system fails. If you're wondering what the difference between POTS and PAF, MSA, etc. is, without me getting into a long technical discussion, I can say that POTS is not a total autonomic "failure" per se. There is autonomic dysfunction, but it is only partial.
In all those other types, there is a deeper identified cause. In familial dysautonomia [FD], for example, we know it is a problematic recessive gene. In the dysautonomia that accompanies spinal cord injuries, we know that it is the trauma to the spinal cord that causes the autonomic dysfunction. Autonomic dysfunction following a snake bite is known to be attributed to the snake venom neurotoxicity. There are paraneoplastic syndromes, infections (tetanus, e.g.), inflammatory processes (e.g. Guillian Barre syndrome), metabolic diseases (e.g. diabetes), and surgeries (e.g. heart transplant) that cause autonomic dysfunction. The list of conditions and drugs/toxins that can disrupt the autonomic nervous system either in part or in whole, temporarily or permanently, is lengthy and our knowledge is certainly still incomplete.
Unfortunately, our physicians simply cannot test for every possible cause of dysautonomia.
As my neuromuscular specialist said at my last appointment, "we're down to a LONG list of unlikely suspects—the best we can do is try aiming for the most likely of those." I just got the results of my repeat paraneoplastic panel back from Mayo's lab today: negative across the board. So in another six months when I see her again, we'll aim another dart at the board. I have other specialists taking hints from my various symptoms as they crop up and throwing their darts as accurately as they possibly can. I don't think there's any reason that not having a "why" should hold one back from getting the best treatment, and achieving a good quality of life. I will continue to seek my "why," but I don't feel that it's a pressing issue in my life any longer. We have a gameplan for my treatment, we adjust it as necessary, and most importantly I have peace of mind.
I hope everyone can find peace and work with their physicians to develop a workable treatment plan in the meantime while "the jury is out" on what caused their dysautonomia. I know for some of us that can be a long road.
In short, some doctors will diagnose [such and such disease] with secondary POTS, where others will drop the POTS diagnosis entirely when [such and such disease] is found. It depends on the doctor/clinic and their view of how diseases should be named as much as anything about your body/condition. I hope that makes sense?
All that being said, there are diseases/conditions/syndromes that we know can sometimes cause "secondary POTS" (or if you prefer "autonomic dysfunction" ... for those not in the "secondary POTS" camp). I've already mentioned NET deficiency, but that is as I said exceedingly rare. Much more common is joint hypermobility syndrome/Ehlers-Danlos syndrome. Some autoimmune diseases have been known to cause patterns of autonomic dysfunction that fit the POTS criteria (e.g., Sjogren's).
(As a sidenote, some of the tests they generally run in a workup for a person with suspected autonomic dysfunction are to look for different causes of autonomic dysfunction; however some are to rule out conditions which have similar symptoms to autonomic dysfunction but are something else.)
PAF, MSA, and their ilk are primary autonomic failures. The autonomic nervous system fails and THAT FAILURE in and of itself is the underlying disease/dysfunction. No deeper, more rudimentary cause can be found by doctors or researchers (at least not yet). In short, they don't really know *why* the autonomic nervous system fails. If you're wondering what the difference between POTS and PAF, MSA, etc. is, without me getting into a long technical discussion, I can say that POTS is not a total autonomic "failure" per se. There is autonomic dysfunction, but it is only partial.
In all those other types, there is a deeper identified cause. In familial dysautonomia [FD], for example, we know it is a problematic recessive gene. In the dysautonomia that accompanies spinal cord injuries, we know that it is the trauma to the spinal cord that causes the autonomic dysfunction. Autonomic dysfunction following a snake bite is known to be attributed to the snake venom neurotoxicity. There are paraneoplastic syndromes, infections (tetanus, e.g.), inflammatory processes (e.g. Guillian Barre syndrome), metabolic diseases (e.g. diabetes), and surgeries (e.g. heart transplant) that cause autonomic dysfunction. The list of conditions and drugs/toxins that can disrupt the autonomic nervous system either in part or in whole, temporarily or permanently, is lengthy and our knowledge is certainly still incomplete.
Unfortunately, our physicians simply cannot test for every possible cause of dysautonomia.
As my neuromuscular specialist said at my last appointment, "we're down to a LONG list of unlikely suspects—the best we can do is try aiming for the most likely of those." I just got the results of my repeat paraneoplastic panel back from Mayo's lab today: negative across the board. So in another six months when I see her again, we'll aim another dart at the board. I have other specialists taking hints from my various symptoms as they crop up and throwing their darts as accurately as they possibly can. I don't think there's any reason that not having a "why" should hold one back from getting the best treatment, and achieving a good quality of life. I will continue to seek my "why," but I don't feel that it's a pressing issue in my life any longer. We have a gameplan for my treatment, we adjust it as necessary, and most importantly I have peace of mind.
I hope everyone can find peace and work with their physicians to develop a workable treatment plan in the meantime while "the jury is out" on what caused their dysautonomia. I know for some of us that can be a long road.
That was a fantastic response. Thank you.
So if I understand you correctly, it is important to determine if one has primary or secondary dysautonomia. Can POTS be secondary as well?
If secondary dysautonomia can be the result of a "disease, condition, toxicity, etc." can you name some known examples? (In my case, I had what seems like a million tests from spinal tap to MRI. They've so far found nothing wrong except a positive EBV blood test showing I had it some time ago.)
Also, what characterizes the more primary PAF and MSA from other types of dysautonomia?
So if I understand you correctly, it is important to determine if one has primary or secondary dysautonomia. Can POTS be secondary as well?
If secondary dysautonomia can be the result of a "disease, condition, toxicity, etc." can you name some known examples? (In my case, I had what seems like a million tests from spinal tap to MRI. They've so far found nothing wrong except a positive EBV blood test showing I had it some time ago.)
Also, what characterizes the more primary PAF and MSA from other types of dysautonomia?
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