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Information Resources

As an individual with Autonomic Dysfunction, I know all too well that it is very important that I communicate effectively with my medical team.  However, I also know that I am one of those people who have difficulty asking for what I want or need from my doctor. Even though I am a healthcare professional, I find that when faced with the need to ask for something for myself, I avoid it like the plague. Even when I really could use some help, I still worry about being a bother to them.  I still feel that I am taking up the time they could be spending with another patient.  
I can’t help but think there are other people in this same situation of worrying more about the doctor’s time than their own needs. But it is critical that you find a way to comfortably talk to your healthcare professionals.  It’s possible to be polite and respectful and to still be assertive at the same time.  Of course, this is common sense, but it’s harder to do than it sounds when you are faced with a daunting appointment.
Without doubt, communication is crucial to good outcomes in healthcare. When people take an active role in their care, research shows they fare better -- in satisfaction and in how well treatments work. A passive patient is less likely to get well.  So, it is very important to be able to ask questions, voice concerns or be able to express it when you need extra support.
I am an active member of the Ehlers Danlos community in the UK, and I have witnessed friends and fellow community members struggle with similar issues because they are nervous about vocalising their concerns. I know many people who have left their doctor’s office in tears because they felt they weren’t being listened to or the appointment ended before they had gained the confidence to ask questions.
If you are worried about any aspect of your health (an ache or pain or symptoms) and your doctor dismisses it, find someone who will listen and help you with the concerns you have. It might be nothing (I know that my autonomic dysfunction is good at playing up for no good reason), but it might be something, so it’s best to be sure.
If your doctor recommends a treatment and you are unsure if you want to try it or not, ask for a second opinion, or even a third. Because although they may all agree with each other (or not) consensus between them may bring peace of mind.
Good doctors and healthcare professionals don’t feel threatened or offended when their patient asks for a second opinion, ordinarily they welcome the input. Good doctors want to be able to answer their patient’s questions to help them better understand what is or is about to happen to them. Within reason, a doctor should be willing to spend as much time as is necessary with their patient to ensure they are fully comfortable and understand their diagnosis and any potential treatments that might be available for them.
It is important that you feel you can trust your doctor. Without trust the relationship just isn’t going to work. You should be able to leave your appointment feeling satisfied that you were able to voice your concerns. And if your questions weren’t answered, be sure that either the doctor will look into them or that they provided you with the information you need to find the answers. Remember, the doctor won’t be able to answer everything – but the key is that they are willing to admit that and try to give you the guidance you need. Listen to your instincts. If someone or something makes you feel uncomfortable, seek help elsewhere. And speak up if you feel you have been inappropriately treated. If not for yourself, do it to save another patient from having a similarly bad experience.
Regardless of the rarity or severity of your condition, you must be your own advocate. Unless you are lucky and have a spouse, friend or family member who can attend every appointment with you, and be your voice, you must find your own.
If you continue to have the same symptoms or problem even after following what you and your doctor (mutually) agreed was a good treatment plan, ask about other options for treatment. Medicine is constantly evolving and the available options may have changed.
While it’s good to stay current on developments about your symptoms / condition, be careful about where you research information. For example, message boards or badly put together “informational” sites and random Internet searches are not necessarily the most sensible places to look. Using the internet is fine, but please ensure that you check the sources of the articles that you’re using. Internet information should generally be taken with a pinch of salt.  The same is true for advice from well-meaning friends and colleagues.  Always follow-up on any information you read or hear about by finding a credible source to back it up.
Ask questions, be heard, listen to your instincts. When in doubt, check it out. You’re worth it.
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Rachel Cox
A couple of years ago, I was at a function.  I was talking to a doctor acquaintance about life, the weather, and Dysautonomia, as you do. Well, when you are me, you do! I never, ever pass up an opportunity to answer questions about Dysautonomia.  Because the more people who know; the less people will suffer through years of mis-diagnosis and lack of understanding. If knowledge of this spreads through the community (both the medical community and the general community) there will be greater understanding towards people who suffer from it. It’s one of my missions. 
This guy, like many doctors, was a very intelligent person.  He asked probing questions, wanted to know who we’d seen and what the current situation was. He was sympathetic about the road we had travelled. He mused that ‘medicalised’ people like me were sometimes seen by doctors as a concern, especially when they look well.  Looking ‘well’ is a bit of a feature of Dysautonomia, so not something we can change. I was interested in his reference to ‘medicalised’ patients.  The term is not something I had encountered before, or heard from others about.  The attitude he spoke of was familiar to me, but not the expression.  Have you watched a doctor ‘think’ this before?
A patient who talks the talk? Who has been googling? (insert eye roll) Could it be a case of somatisation?
Somatisation is when people believe something is physically wrong with them for which there is no definitive evidence. Psychosomatic illness.  Hypochondriasis. There are a range of psychiatric disorders which follow this pattern.  Sadly, for people with Dysautonomias, many of the symptoms commonly present in somatic disorders are like our own.  Dizziness, chest pain, abdominal problems.  This is why, for many people with Dysautonomia, the road to diagnosis may have included earlier mis-diagnoses of anxiety, depression or somatisation before further investigations uncovered the facts.
Recently, the term ‘medical literacy’ came up in my readings.  It reminded me of that social encounter with the doctor and his word ‘medicalised’.  I thought I would look into it, because people like myself, who have been ill for a long time, do develop a different type of jargon than the average patient.  We use the correct medical terminology for things, because it is the most efficient way to explain what has been going on.  It seems far more practical to fast forward through all the translations between doctor speak and layman’s terms. Especially when consultation times are squeezed into just a few moments, a few moments to describe a month’s worth of symptoms. Also, have you noticed how many letters there are in medical words?  Phew, acronyms were invented for a reason!
We also talk with one another online. Among the patient community, the use of acronyms and medical jargon and abbreviations is commonplace.  So much so, that new members take a while to learn the lingo.  For the newly diagnosed Dysautonomiac, to become medically ‘literate’ means incorporating a knowledge of the autonomic nervous system, the various types of Dysautonomia, the range of treatments or medications used for various types (and how they work), recent research findings, the big players (in terms of doctors and medical institutions) and an understanding of the very wide range of symptoms that can be implicated in our conditions.
In New Zealand, ‘Health Literacy’ is the term we use in place of Medical Literacy (I believe the latter is an American term).  The Ministry of Health’s report into health literacy in this country defines it as:
-- Kickbusch et al., 2005; Kōrero Mārama, 2010
Just like literacy with language, being ‘literate’ in a medical, or health care, sense, means being able to respond to the data provided to you in an analytical way.  Literacy really means being able to make sense of information in context.  For people without science based or medical degrees, it can take some time!  For me, learning what was wrong with me felt like floundering in a morass of confusing information; I was at a loss to know how to even begin to ‘make sense’ of my diagnosis.  My cardiologist could tell me about what was happening to my heart, but this was only part of the bigger picture …and it didn’t include why it was happening to my heart.  My artsy brain wanted the philosophical lowdown! Over time, I have assimilated so much information.  There is more than just the condition to understand, there is also the wider health system, the politics of the consulting room, the process by which your needs are met and the differences between our health system and that of our fellow patients overseas.  I’ve seen countless conversations in various patient groups, where good information is redundant, simply because it doesn’t apply to the way our health system works here.  It might relate to my condition, but not to what is possible for my treatment.
These factors can be so daunting to a newly diagnosed Dysautonomiac, or anyone with a chronic, rare or invisible illness.  Developing this ‘literacy’ is crucial to your health plan. Simply having the jargon without the contextual understanding will compromise your care.  Particularly if doctors see you as a well-looking ‘somatic probability’ just because you can ‘talk the talk’.  Your use of medical terms must be accurately supported by a contextual understanding, analytical thinking… a fully literate comprehension of the subject at hand: your health.  This is a key component to getting the help you need from the professionals who are qualified to help you.
Here are four ways you can improve your health literacy:
Ask questions
Don’t be afraid to ask.  A specialist recently mentioned Occam’s Razor to me, assuming that I understood the reference.  I didn’t.  It’s a med school 101 reference.  I was confused, so I said “Can you explain?  I don’t understand …Occam’s what?”.  Occam was a monk a very long time ago who put forward a problem-solving principle: “among competing hypotheses, the one with the fewest assumptions should be selected. Other, more complicated solutions may ultimately prove correct, but—in the absence of certainty—the fewer assumptions that are made, the better”. (thanks Wikipedia for saying that better than I could remember it).  He was explaining why he believed I had one disorder over another.  It was the more likely choice.  The important thing to remember from this story is that asking is the short cut to the understanding you need to have, particularly in discussions with intellectual giants.  In my experience, most doctors like to be asked questions, particularly for clarification.
Read all about it
Use Google and Google Scholar, sign up for newsletters run by organisations who specialise in your diagnosis.  Use the excellent resource websites that are proliferating on the web, many have sections full of pertinent research documents and medical journal articles. Follow the Facebook pages of your not-for-profit organisations; timely information is often added, particularly after large symposiums where all the experts gather to discuss their ideas. Search for blogs written by people with your condition.  They will have access to information too.  Read it all, even if you don’t understand it.  If you like print copies, print things out, highlight, circle terms you don’t understand and find their definition.  If you read something scary, suspend your judgement about its pertinence to you until you have discussed it with your doctor, other patients in your support group, or until you have identified the relevance of what you are reading.  There is a great deal of pseudo-science out there that can trick unsuspecting readers.  Run it by someone with more knowledge than you before freaking out. 
Find your Tribe
Facebook and the internet abound with groups, forums and news boards that bring people with the same conditions together.  Look for groups with a strong set of user guidelines.   Read them and see if you agree with their code of conduct. There are some awful groups where bickering and mindless trolls like to stir the pot.  Avoid those.  If you find yourself in one, leave.  There are much better, well run groups of like-minded people out there.  It took me a long time to find a group that I consider to be responsibly administered.  When you find your ‘tribe’ you will learn a great deal more from them than from any other source. The experience of other patients is invaluable, particularly with a condition that is not common, or not commonly understood.  Finding your geographically relevant tribe is good too, because then all the information will relate to your experiences.
Delve into your Data
Get hold of your medical records.  In New Zealand, you can request these from your GP and your District Health Board.  Organise them into a file and refer to them. Examine your results and look for patterns.  Knowing (for example) what your iron levels or heart rate has been like over time means you can discuss these things knowledgeably with your doctor.  Knowing ‘thyself’ also helps you to see if there are any trends or significant changes in your condition.
Why does it matter?  If you, like me, suffer from a condition which is poorly understood by many in the health sector, being your own advocate, managing your own chronic condition and taking the leading role in your own health plan will be necessary.  Without strong health literacy, the likelihood that you will be able to take this responsibility on will be lower.  Not sure about that?  This is what the Ministry of Health had to say about people with poor health literacy:
The only person who will ever care about managing your health plan properly, is you.
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Amy Keys
Some people honestly don’t remember a time when they were symptom free and healthy.  For others, a condition may have come on suddenly.  No matter how adaptable and flexible your personality may be, illness is guaranteed to be an adjustment.  Most likely it will include going through the grieving process.  Not only is that ok, but it is also necessary for a healthy mental state. 
Before I became ill, I had tummy problems off and on, and some pain, and issues with sleep, but I had good health overall and was living the busy life of a 29-year-old.  Enter dysautonomia.  When any form of dysautonomia is in full force it affects so many different parts and systems of the body.  Like most people newly diagnosed, my daily focus became taking medications, resting, drinking water, logging heart rate, measuring blood pressure, attending physical therapy and countless specialist appointments and testing on a regular basis.
With my health changing, I tried to remain positive and as symptom-free as possible.  I write a blog and I focused my writing on the importance of choosing to find joy.  While there is nothing at all wrong with working at a positive approach for coping with illness – attention also needs to be paid to the very natural emotions that come with loss.  I began to feel out of control in my life and questioned my worth and value.  Eventually I found a psychiatrist to talk to who helped me understand the grieving process and how important it is for mental health.   I had never considered that I would need to take the time to grieve for what “used to be” or that I would need to pay as much attention to my mental health as I do my physical.  Both are essential to having the balance and rational outlook on life that we all deserve.
There are five stages in the grieving process:
 denial  anger  bargaining  depression    acceptance.  There are no hard rules for how to go through these stages. Everyone goes through the process in their own way.  But there are also no shortcuts.  It is a natural desire to want to get on with life as soon as possible.  To try and jump to “acceptance” and skip the difficult steps in between.  But that only leads to suppressing the feelings of anger and depression that are natural and inevitable.  Jumping past the stages proves only to be counterproductive.
It’s ok to admit that you feel overwhelmed and a little bit scared. After all, every aspect of your life has changed.  It’s ok to be sad at times too.  It’s alright to worry about the future and how things will work out.  It’s normal to be mad or upset at times.  Without allowing yourself to face these feelings it is impossible to be able to move forward and cultivate a healthy mental state.  Another point to note, is that once this process is complete it doesn’t mean that you are done.  Grief does not unfold in fixed phases.  There may be specific days that remind you of something you used to do in a healthier time that you can’t do now.  The grief cycle will start over again multiple times throughout your life.  That is normal.
I am about a year and a half out from my hospital stay and diagnosis.  My very active lifestyle has changed significantly to a more sedentary one and with that has come some weight gain.   Little by little, I have cleaned out my dresser to remove clothes that are now two sizes too small.  I’m proud of myself for being brave enough to work on this project.  But, to be completely honest, I’ve cleaned out every drawer but one: my work clothes drawer.  I miss my job terribly and it’s just too difficult for me to get rid of all of my work clothes right now.  The truth is that if I was miraculously cured tomorrow and re-hired the next day, none of those clothes would even fit me.  It’s not the clothes that I am hanging on to, it is the memory of the “old” me. The pre-dysautonomia “me”.  I will clean out that drawer eventually, but it will be on my time.  Because this is all a part of grieving and all our journeys are different.
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POTS Links

By edriscoll, in POTS,

Comprehensive information on POTS:
Postural Tachycardia Syndrome, Beyond Orthostatic Intolerance. Dr. Satish Raj
Postural orthostatic tachycardia syndrome: anesthetic implications in the obstetric patient:
Anaesthetic management of a parturient with the postural orthostatic tachycardia syndrome: a case report:
Preoperative considerations in a patient with orthostatic intolerance syndrome:
Autonomic dysfunction - anaesthetic management
Abnormal baroreflex responses in patients with idiopathic orthostatic intolerance:
Baroreflex control of muscle sympathetic nerve activity in postural orthostatic tachycardia syndrome:
The broader view: catecholamine abnormalities:
Endothelial NO Synthase Polymorphisms and Postural Tachycardia Syndrome:
Chiari malformation/spinal cord involvement
Watch a presentation by physicians at The Chiari Institute and NIH as they discuss the possible connection between POTS, Ehlers-Danlos syndrome and Chiari Malformation: (click on the Ehlers-Danlos National Foundation link)
Cerebral syncope in a patient with spinal cord injury:
Chiari Malformation:
Chiari, fibromyalgia, gastrointestinal problems, gulf war syndrome, multiple chemical sensitivities/environmental illness, orthostatic intolerance:
Sinus arrhythmia and pupil size in Chiari I malformation: evidence of autonomic dysfunction:
The Chiari Institute:
Autonomic nervous system disorders in 230 cases of basilar impression and arnold-chiari deformity:
Chiari I malformation as a cause of orthostatic intolerance symptoms: a media myth?
No increased herniation of the cerebellar tonsils in a group of patients with orthostatic intolerance:
Orthostatic intolerance and syncope associated with Chiari type I malformation:
Orthostatic hypotension following spinal cord injury: understanding clinical pathophysiology:
Chronic fatigue syndrome
The importance of orthostatic intolerance in the chronic fatigue syndrome:
MEDLINEplus health information on chronic fatigue syndrome:
Chronic fatigue syndrome: A hypothesis focusing on the autonomic nervous system:
Dental Considerations
Postural Orthostatic Tachycardia Syndrome: Dental Treatment Considerations by John K. Brooks, DDS; Laurie A. P. Francis, RDH: 
Diabetes and autonomic neuropathy
Chronic administration of pharmacologic doses of vitamin E improves the cardiac autonomic nervous system in patients with type 2 diabetes:
Value of scintigraphy using meta-iodo-benzyl-guanidine (MIBG) in the investigation of cardiac autonomic neuropathy in diabetic patients. Comparison with Ewing tests:
Abnormal cardiovascular reflexes in juvenile diabetics as preclinical signs of autonomic neuropathy:
Response of the autonomous nervous system of the heart in diabetes mellitus:
MEDLINEplus health information on diabetes:
Blood volume perturbations in the postural tachycardia syndrome:

Quality of life in patients with postural tachycardia syndrome:
Ehlers-Danlos Syndrome (Joint Hypermobility Syndrome)
Ehlers Danlos Syndrome. University of Washington, Seattle:
Ehlers-Danlos National Foundation:
Ehlers-Danlos syndrome, classical type:
Ehlers-Danlos syndrome, hypermobility type:
Joint hypermobility syndrome: a complex constellation of symptoms:
Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial
hypotension: a prospective study:
Association Between Joint Hypermobility Syndrome and Panic Disorder:
Is joint hypermobility related to anxiety in a nonclinical population also?
Neurological manifestations of Ehlers-Danlos syndrome:
Your eyes and Ehlers-Danlos Syndrome:
The prevalence and significance of post-exercise (postural) hypotension in ultramarathon runners:
Certain cardiovascular indices predict syncope in the postural tachycardia syndrome:
National Fibromyalgia Research Association:
MEDLINEplus health information on fibromyalgia:
Elusive syndromes: Treating the biologic basis of fibromyalgia and related syndromes:
Influence of the menstrual cycle on sympathetic activity, baroreflex sensitivity, and vascular
transduction in young women:
Hypovolemia in syncope and orthostatic intolerance role of the renin-angiotensin system:
Is this patient hypovolemic?
Idiopathic hypovolemia: a self perpetuating autonomic dysfunction?
Partial lipodystrophy in a boy:
Erythrocyte magnesium in symptomatic patients with primary mitral valve prolapse: relationship to symptoms, mitral leaflet thickness, joint hypermobility and autonomic regulation:
Cardiovascular consequences of magnesium deficiency and loss: pathogenesis, prevalence and manifestations. Magnesium and chloride loss in refractory potassium repletion:
Magnesium and therapeutics:
Magnesium deficiency in the pathogenesis of mitral valve prolapse:
Review and hypothesis: Might patients with the chronic fatigue syndrome have latent tetany
of magnesium deficiency:
The magnesium web site - Links to over 300 articles discussing magnesium and magnesium

Web Site for Cardiovascular and Autonomic Pharmacology:
Drug protects against nervous system failure:

Treatment of orthostatic hypotension with erythropoietin:

Effects of long-term clonidine administration on the hemodynamic and neuroendocrine postural
responses of patients with dysautonomia:
Cardiac vagal response to water ingestion in normal human subjects:
Medicinal uses of licorice through the millennia: the good and plenty of it:
Licorice root. A natural sweetener and an important ingredient in Chinese medicine:
Licking latency with licorice root:
Adverse drug reactions related to drugs used in orthostatic hypotension: a prospective and systematic pharmacovigilance study in France:
Mitochondrial disease and dysautonomia
Mitochondrial cytopathy in adults: what we know so far:

Mitochondrial encephalomyopathies presenting with features of autonomic and visceral dysfunction:
A case of mitochondrial encephalomyopathy with peripheral neuropathy and autonomic symptoms:
Neurologic presentations of mitochondrial disorders
Mitral valve prolapse
The phenomenon of dysautonomia and mitral valve prolapse:
Exercise response in young women with mitral valve prolapse:
Multiple system atrophy/shy-drager syndrome
SDS/MSA support group:
Web site full of links to other sites about MSA/SDS:

Norepinephrine transporter
Genetic or acquired deficits in the norepinephrine transporter: current understanding of clinical
Orthostatic intolerance is not necessarily related to a specific mutation (Ala457Pro) in the human norepinephrine transporter gene:
Phenotypical evidence for a gender difference in cardiac norepinephrine transporter function:
The Nutcracker phenomenon
Does severe nutcracker phenomenon cause pediatric chronic fatigue?
Nutcracker phenomenon demonstrated by three-dimensional computed tomography:
Diagnosis of the nutcracker phenomenon using two-dimensional ultrasonography:
Magnetic resonance angiography in nutcracker phenomenon:
An effective "transluminal balloon angioplasty" for pediatric chronic fatigue syndrome with nutcracker phenomenon:
Nutcracker phenomenon treated with left renal vein transposition: a case report:
The nutcracker syndrome: its role in the pelvic venous disorders:
Orthostatic hypotension in organic dementia: relationship between blood pressure, cortical blood flow and symptoms:
Neurocardiovascular instability, hypotensive episodes, and MRI lesions in neurodegenerative dementia:
Orthostatic hypotension in Alzheimer's disease: result or cause of brain dysfunction?
Orthostatic hypotension as a risk factor for stroke:
Orthostatic hypotension
Orthostatic hypotension:
Cardiovascular Causes of Falls by Brian J. Carey and John F. Potter:
Orthostatic Hypotension by Bradley JG, Davis KA.
Paraneoplastic syndromes
Immunological and endocrinological abnormalities in paraneoplastic disorders with involvement of the autonomic nervous system:
Biochemical diagnosis of pheochromocytoma:
MEDLINEplus health information on pheochromocytoma:
Germ-line mutations in nonsyndromic pheochromocytoma:
Diagnosis and management of porphyria:
Postural orthostatic tachycardia syndrome/orthostatic intolerance (General Information Links)
Management of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia and Vasovagal Syncope, Dr.'s Satish Raj & Robert Sheldon  https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5013178/ 
Medscape: Postural Tachycardia Syndrome:
The Postural Tachycardia Syndrome (POTS): Pathophysiology, Diagnosis & Management
Orthostatic intolerance:
A review of the classification, diagnosis, and management of autonomic dysfunction syndromes associated with orthostatic intolerance:
Clinical disorders of the autonomic nervous system associated with orthostatic intolerance: an overview of classification, clinical evaluation and management:
The neuropathic postural tachycardia syndrome:
Hereditary dysautonomias: Current knowledge and collaborations for the future:
Oct 3, 2002 http://www.familialdysautonomia.org/NIH_OCT3.pdf
Oct. 4 2002 http://www.familialdysautonomia.org/NIH_OCT4.pdf
Effects of head-up tilting on baroreceptor control in subjects with different tolerances to orthostatic stress:
Vascular perturbations in the chronic orthostatic intolerance of the postural orthostatic tachycardia syndrome:
American Autonomic Society: (be sure to visit the autonomic news section for information on the latest research)
Postural tachycardia syndrome and anxiety disorders:
Postpartum Postural Orthostatic Tachycardia Syndrome in a Patient with the Joint Hypermobility Syndrome:
Postural orthostatic tachycardia syndrome: anesthetic implications in the obstetric patient:
Research Studies
DINET studies page:
NDRF clinical research page:
12 More Pages:
STARS US (Syncope Trust and Reflex Anoxic Seizures)
STARS - syncope trust and reflex anoxic seizures:
National Dysautonomia Research Foundation:
Dysautonomia Youth Network of America (DYNA) (devoted to youth with dysautonomia)
NINDS syringomyelia information page:
Postural tachycardia syndrome in syringomyelia: response to fludrocortisone and beta-blockers:
Involvement of the autonomic nervous system in patients with syringomyelia - a study with the sympathetic skin response:
Hyperhidrosis as the presenting symptom in post-traumatic syringomyelgia: (hyperhidrosis is excessive sweating)
Cardiovascular reflexes in syringomyelia:
MEDLINEplus health information on syringomyelia:
The fainting patient: value of the head-upright tilt-table test in adult patients with orthostatic intolerance:
Autonomic diseases: clinical features and laboratory evaluation:
Syncope Care & Treatment:
The Dysautonomia Information Network presents the first full length documentary about Postural Orthostatic Tachycardia Syndrome.
POTS - Mayo Clinic:
POTS syndrome - Mayo Clinic:
The Woman who Kept Falling Down - Mystery Diagnosis 

Please keep in mind that recent clinical findings will not be included in earlier publications...
Cheltenham Syncope Clinic:
Vasovagal Syncope in 2016: The Current State of the Faint, Dr. Raj et al
All that shakes is not epilepsy YM Hart Consultant Neurologist, Royal Victoria Infirmary, Newcastle upon Tyne, UK
Syncope (NMS) in Adolescents by Martin A. G. Lewin:
Neurally Mediated Syncope by Munir Zaqqa, MD and Ali Massumi, MD, FACC:
The Elusive Pathophysiology of Neurally Mediated Syncope by Rogelio Mosqueda-Garcia, Raffaello Furlan, Jens Tank and Roxana Fernandez-Violante:
Cardiovascular Causes of Falls by Brian J. Carey and John F. Potter:

Cerebral Vasoconstriction in Vasovagal Syncope: Any Link With Symptoms? by Alfonso Lagi, Simone Cencetti, Vanni Corsoni, Dimitrios Georgiadis and Stefano Bacalli:
Randomised Clinical Trials of Drug Therapy for Vasovagal Syncope by M. Brignole
Pacemaker for Vasovagal Syncope: Good for Few by M. Brignole:
Management of Recurrent Vasovagal Syncope by Means of Isometric Counterpressure Manoeuvres by M. Brignole:
Neurocardiogenic Syncope Due to Recurrent Tonsillar Carcinoma: Successful Treatment by Dual Chamber Cardiac Pacing with Rate Hysteresis by Yue A. M., and Thomas, R. D.:
Usefulness of a Tilt Training Program for the Prevention of Refractory Neurocardiogenic Syncope in Adolescents: A Controlled Study byEnrico Di Girolamo, Cesare Di Iorio, Luigi Leonzio, Panfilo Sabatini and
Antonio Barsotti:
Beta-Blockers no Better than Placebo in the Treatment of Vasovagal Syncope:
The Usefulness of Tilt Testing with an Intravenous Beta-Blocker in Assessing the Efficacy of Long-Term Therapy in Patients with Vasovagal Syncope byGrzegorz Gielerak, Karol Makowski, Ewa Dłużniewska, Alicja Stec and Marian Cholewa:

Please keep in mind that recent clinical findings will not be included in earlier publications.
General Information
Multiple System Atrophy, Dr. Andre Diedrich:
Multiple System Atrophy: Medline Plus.gov: https://medlineplus.gov/ency/article/000757.htm
Multiple System Atrophy by Laurie Swan and Jerome Dupont:
Primary Autonomic Failure: Three Clinical Presentations of One Disease? by Horacio Kaufmann:
Clinical Characteristics of Patients with Multiple System Atrophy in Singapore by RDG Jamora, A Gupta, AKY Tan & LCS Tan:
Hypotension and/or hypertension
The recommendations of a consensus panel for the screening, diagnosis, and treatment of neurogenic orthostatic hypotension and associated supine hypertension, by Gibbons, C.H., Schmidt, P., Biaggioni, I. et al. J Neurol (2017) https://link.springer.com/article/10.1007/s00415-016-8375-x/fulltext.html
Sympathetically Mediated Hypertension in Autonomic Failure by John R. Shannon, Jens Jordan, Andre Diedrich, Bojan Pohar, Bonnie K. Black,
David Robertson and Italo Biaggioni:
Neuropsychological functions in progressive supranuclear palsy, multiple system atrophy and Parkinson's disease:
Raynaud's Phenomenon
Raynaud's Phenomenon after Sympathetic Denervation in Patients with Primary Autonomic Failure: Questionnaire Survey by Rajeev Mallipeddi and Christopher J Mathias:
REM Sleep Behaviour Disorder Differentiates Pure Autonomic Failure from Multiple System Atrophy with Autonomic Failure by Giuseppe Plazzi, Pietro Cortelli, Pasquale Montagna, Alessandro De Monte, Raffaella Corsini, Manuela Contin, Federica Provini, Giulia Pierangeli, Elio Lugaresi:
Multiple System Atrophy Presenting as Central Sleep Apnoea by L.J. Cormican, S. Higgins, A.C. Davidson, R. Howard and A.J. Williams:
Diagnosis and Treatment of Multiple System Atrophy: An Update by Gregor Wenning and Felix Geser:
The Pressor Response to Water Drinking in Humans by Jens Jordan, John R. Shannon, Bonnie K. Black, Yasmine Ali, Mary Farley; Fernando Costa, Andre Diedrich, Rose Marie Robertson, Italo Biaggioni and
David Robertson:
The Hypertension of Autonomic Failure and its Treatment by John Shannon, Jens Jordan, Fernando Costa, Rose Marie Robertson, and Italo Biaggioni:
The natural history of multiple system atrophy: a prospective European cohort study, Dr. Christopher Mathias et al:   http://www.thelancet.com/journals/laneur/article/PIIS1474-4422(12)70327-7/fulltext
Support Groups
The Multiple System Atrophy Coalition
8311 Brier Creek Parkway
Suite 105-434
Raleigh,  NC  27617
Tel: 1-866-737-5999
Judy Biedenharn jbiedenharn@msacoalition.org
Vera James vjames@msacoalition.org
Pam Bower pbower@msacoalition.org
Carol Langer clanger@msacoalition.org
Don Crouse dcrouse@msacoalition.org


Pure Autonomic Failure Links

By edriscoll, in PAF,

Please keep in mind that recent clinical findings will not be included in earlier publications.
General Information 
Alternative Names: \Shy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson plus syndrome; MSA-P; MSA-C
Vanderbilt University Autonomic Dysfunction Center:
A Review of the Classification, Diagnosis, and Management of Autonomic Dysfunction Syndromes Associated with Orthostatic Intolerance by Blair P. Grubb and Sergio do Carmo Jorge:
The Natural History of Pure Autonomic Failure: a U.S. Prospective Cohort, Dr. Kaufman, Dr. Low et al: https://www.ncbi.nlm.nih.gov/pubmed/28093795
Identification of a Renin Threshold and Its Relationship to Salt Intake in a Patient With Pure Autonomic Failure by Henriette Hohenbleicher; Fabian Klosterman; Ulrike Schorr; Sepp Seyfert; Pontus B. Persson and Arya M. Sharma:
Orthostatic Tolerance, Cerebral Oxygenation, and Blood Velocity in Humans With Sympathetic Failure by Harms M. P., Colier W. N., Wieling W., Lenders J. W., Secher N. H., van Lieshout J. J.:
The Distribution of Lewy Bodies in Pure Autonomic Failure: Autopsy Findings and Review of the Literature by K. Hague, P. Lento, S. Morgello, S. Laro and H. Kaufmann:
Hypotension and/or Hypertension
Stress induced hypotension in pure autonomic failure by R D Thijs and J G van Dijk:
Orthostatic Hypotension by Bradley JG, Davis KA.
Autoregulation of Cerebral Blood Flow in Orthostatic Hypotension by Novak V., Novak P., Spies J. M., Low P. A.:
Neck and other muscle pains in autonomic failure: their association with orthostatic hypotension by K M Bleasdale-Barr and C J Mathias:
Cognitive functioning in orthostatic hypotension due to pure autonomic failure by Heims HC, Critchley HD, Martin NH, Jager HR, Mathias, CJ, Cipolotti L.:
The Hypertension of Autonomic Failure and it's Treatment, Dr. Bioganni, Dr. Rose Marie Roberson et all:
Sympathetically Mediated Hypertension in Autonomic Failure by John R. Shannon, Jens Jordan, Andre Diedrich, Bojan Pohar, Bonnie K. Black, David Robertson, Italo Biaggioni:
Vasopressin and Blood Pressure in Humans by Jens Jordan and Jens Tank:
Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy by N Mabuchi, M Hirayama, Y Koike, H Watanabe, H Ito, R Kobayashi, K Hamada and G Sobue:
A Sound Night's Rest may do no good in Autonomic Failure! by Christopher J. Mathias:
Hormonal and cardiovascular reflex assessment in a female patient with pure autonomic failure by Lopes HF, Consolim-Colombo FM, Hachul D, Carvalho ME, Pileggi F, Silva HB:
The Pressor Response to Water Drinking in Humans by Jens Jordan, John R. Shannon, Bonnie K. Black, Yasmine Ali, Mary Farley; Fernando Costa, Andre Diedrich, Rose Marie Robertson, Italo Biaggioni and
David Robertson:

Postural Orthostatic Tachycardia Syndrome (POTS), Dysautonomia, and the Autonomic Nervous System
What is postural orthostatic tachycardia syndrome (POTS)? What are its signs and symptoms and what are the mechanisms by which all body systems are affected? How does the autonomic nervous system normally function? What is the etiology, or cause, of dysautonomia? How is it diagnosed? Can it be treated? Join professor Carrie on a journey through the autonomic nervous system and develop a deeper understanding of autonomic function in general, and POTS in particular. This lecture is presented at the college level and is meant to be accessible for patients, family members, students, and medical professionals. 
0:00 Background and purpose
1:28 Definition and basic mechanism of POTS
8:10 POTS symptoms and causes: acute cerebral hypoperfusion, chronic cerebral hypoperfusion, chronic organ and tissue hypoperfusion, autonomic dysregulation, sympathetic hyperactivity (hyperadrenergic POTS)
16:59 Organization of the nervous system: central nervous system, peripheral nervous system, afferent pathways, efferent pathways, autonomic nervous system, sympathetic division, parasympathetic division
24:23 Nervous system signaling pathways: preganglionic neuron, ganglionic neuron, acetylcholine, cholinergic receptors, nicotinic receptors, muscarinic receptors, norepinephrine, adrenergic receptors, alpha receptors, beta receptors
43:50 The dysautonomia family of conditions: POTS, neurally mediated syncope, autonomic failure
46:13 Etiology of POTS: genetic mutations, joint hypermobility, Ehlers-Danlos syndrome, norepinephrine transporter (NET) deficiency, catecholamine synthesis, tyrosine hydroxylase (TH), aromatic L-amino acid decarboxylase (AADC), dopamine beta-hydroxylase (DBH), catechol-O-methyltransferase (COMT), monoamine oxidase A (MAO-A), epigenetics, deconditioning, mononucleosis, autoimmunity, Guillain-Barre syndrome
1:06:39 Diagnosing POTS: medical history, tilt table test, catecholamines test, specialized autonomic testing
1:16:18 POTS prognosis
1:19:26 Managing and treating POTS -- non-pharmacologic: diet, methylcobalamin, methyltetrahydrofolate, exercise, environment, psychosocial considerations
1:37:47 Managing and treating POTS -- medications: mineralocorticoids, beta-blockers, alpha-agonists, reuptake inhibitors, psychostimulants
1:53:46 Conclusion and hope for the future


POTS What To Avoid

By edriscoll, in POTS,

Ablation of the sinus node may be detrimental to POTS patients. A Mayo Clinic study reported short-term success in five of seven ablated patients with inappropriate sinus tachycardia and postural orthostatic tachycardia features (Shen, Low, Jahangir, Munger, Friedman, Osborn, Stanton, Packer, Rea & Hammill, 2001). However, long-term outcomes were disappointing in these patients. None of the patients experienced complete eradication of symptoms. A follow-up evaluation showed no vast improvement in symptoms, despite better heart rate control. A later publication states "in our laboratory, sinus node modification, total sinus node ablation, or atrioventricular nodal ablation is not recommended for patients with inappropriate sinus tachycardia who have autonomic evidence of postural orthostatic tachycardia" (Shen, 2002).
Ablations have reportedly been detrimental to some POTS patients who were misdiagnosed as having inappropriate sinus tachycardia. After the apparently successful elimination of their "sinus tachycardia", they were left with profound orthostatic hypotension (Grubb & Karas, 1999).
Alcohol enhances peripheral venous pooling, which will exacerbate hypotension (Grubb & Karas, 1999). Alcohol can also lead to a dehydrated state.
Anesthesia can be especially challenging for patients with autonomic dysfunction. When anesthesia is absolutely necessary, an arterial line should be inserted to monitor beat to beat variations in blood pressure. Heart rate should also be constantly monitored, as anesthesia can disturb cardiovascular function. Extra IV fluids are also needed. Read more
Bending up and down, as done when picking items up off of the floor, may increase symptoms. It is best to bend at the knee and squat down rather then to bend over forward at the waist.
Blowing up balloons is similar to the valsalva maneuver and can exacerbate symptoms in some patients.  
Certain Foods, such as dairy products,may increase symptoms in some patients. White sugar and other refined carbohydrates can exacerbate hypotension by causing increased dilation in the gut (Mathias, 2000). It is important to identify and avoid food triggers. Studies show that gluten sensitivity may play a role in neurological disorders (Hadjivassiliou, Gibson, Davies-Jones, Lobo, Stephenson & Milford-Ward, 1996).

Climbing stairs will make some patients feel worse. 
Dehydration is one of the worst states a POTS patient can be in. It is very important to always stay well hydrated.
Doctors who don't know much about POTS will surely leave patients feeling frustrated. Patients of inexperienced doctors may not receive expert care and may not return to optimum health. Finding an experienced physician is not only desirable, it is a necessity. Physicians who treat dysautonomia can be located on our Physician List.
Eating large meals can worsen hypotension in some individuals with dysautonomia (Mathias, 2000). This is because large meals can cause blood to pool excessively in the abdomen.
Energy Drinks, such as Red Bull, should be avoided in those with POTS. Postural tachycardia syndrome associated with a vasovagal reaction was recorded in a young volleyball player after an excess intake of Red Bull as a refreshing energy drink (Terlizzi, Rocchi, Serra, Solieri & Cortelli, 2008).
Epinephrine is used by doctors and dentists for a variety of reasons. It is commonly used in numbing shots. It is wise to avoid epinephrine as it stimulates the heart.
Exercise will make many people with dysautonomia feel worse. Strenuous, exhausting exercise should be avoided. Some patients will experience an exacerbation of symptoms after exercise that may last for a day or more, especially if they suffer from mitochondrial disease. These patients should check with a physician before starting any exercise program.
Exercise may be helpful in abating blood from pooling in the limbs. Exercise to build and strengthen the leg muscles can be especially beneficial. Exercise can help POTS patients to avoid becoming deconditioned. A deconditioned state further exacerbates symptoms of orthostatic intolerance.
Fatigue enhances peripheral venous pooling (Grubb & Karas, 1999). Those with POTS will do well to live life at their own pace. It is important that patients don't overdo it, as this will make them feel even worse. POTS will require some lifestyle modification.
Giving blood can be harmful to people with POTS. Blood pooling in the legs already diminishes the amount of blood flowing to the heart and brain. Further, some patients are hypovolemic (have low blood volume) and need every drop of blood that they have. Blood should not be donated and should only be drawn when necessary for medical reasons. 
Heat dilates blood vessels and will make POTS symptoms worse. Patients should avoid spending a lot of time outdoors on hot days. POTS patients should not take hot showers/baths or subject their bodies to saunas, hot tubs or greenhouses. Heat enhances peripheral venous pooling (Grubb & Karas, 1999).
Holding the arms up in the air can cause problems for some individuals. Holding the arms up requires the heart to work harder to counteract the effects of gravity. This is especially difficult for the heart if there is already excessive venous pooling in the lower limbs. The heart may not be able to effectively pump blood up into raised arms and tachycardia will result from its effort.
Lifting objects can aggravate the symptoms of POTS. This may be due to the increased work load on the heart, especially if blood is pooling in the legs. Pooling blood in the lower body makes less blood available for the muscles in the upper body. Also, straining, bending over, coughing and sneezing all raise cerebral spinal fluid pressure. It has been theorized that some POTS patients may experience symptoms while lifting due to changes in cerebral spinal fluid pressure. 
Many medications will affect autonomic testing results. Some medications that have been reported to significantly affect autonomic testing results include: chlorpromazine, thioridazine, tricyclic antidepressants, bupropion, mirtazepine, vanlafaxine, clonidine, alpha blockers, beta blockers, calcium channel blockers, opiates and topical capsaicin (Sandroni, 1998). Some physicians believe patients should discontinue measures at alleviating symptoms before autonomic testing. By doing so, symptoms are more likely to present during testing. This may help a physician to discern the true nature of a patient's disorder. 
Numerous over-the-counter products, such as melatonin, can negatively effect the POTS patient. Many products stimulate the heart or lower blood pressure or have diuretic effects, etc. Some products can help one person while hindering another. For example, caffeine can have the positive effect of raising blood pressure in some individuals. However, caffeine also increases the length of time that catecholamines remain active and this can be detrimental to those with a hyperadrenergic state. POTS patients should check with their doctor before taking over-the-counter products.
Over-stimulating environments can make POTS symptoms worse. A number of POTS patients report being overly sensitive to bright lights, loud noises and busy environments.
Singing has been reported to temporarily worsen symptoms in some individuals.
Some pharmacologic agents may cause or worsen orthostatic intolerance. Please check with your physician before taking prescription or over-the-counter medications.Some of themedications that physicians have identified as causing or worsening orthostatic intolerance include:
Angiotensin Converting Enzyme Inhibitors (Grubb & Karas, 1999)
Alpha Receptor Blockers (Grubb & Karas, 1999)
Calcium Channel Blockers (Grubb & Karas, 1999)
Beta Blockers (Grubb & Karas, 1999)
Phenothiazines (Grubb & Karas, 1999)
Tricyclic Antidepressants (Grubb & Karas, 1999)
Bromocriptine (Grubb & Karas, 1999)
Ethanol (Grubb & Karas, 1999)
Opiates (Grubb & Karas, 1999)
Diuretics (Grubb & Karas, 1999)
Hydralazine (Grubb & Karas, 1999)
Ganglionic Blocking Agents (Grubb & Karas, 1999)
Nitrates (Grubb & Karas, 1999)
Sildenafil Citrate (Grubb & Karas, 1999)
MAO Inhibitors (Grubb & Karas, 1999)
Stress will often aggravate the symptoms of POTS. The body is continuously adapting to stress, whether it is physical, mental or chemical. POTS patients sometimes lack the ability to correctly process stress due to malfunctioning or excessive functioning of the autonomic nervous system (ANS). Patients may also already have high levels of norepinephrine, which is a stress hormone. POTS patients need to avoid stress (when possible) and live life at their own pace.      
Specific stresses such as surgery, childbirth and trauma (such as a car accident) have preceded or worsened the development of POTS in some individuals. This is thought to occur due to a number of factors. Surgery, childbirth and trauma can result in excessive blood loss in POTS patients who may already be hypovolemic. It is important for POTS patients to be given extra fluids during these times. Some people theorize that surgery may require a positioning of the neck that can aggravate hind brain compression. While the relationship between hind brain compression and POTS is controversial, it may be wise to avoid these positions (if possible) with POTS patients. Trauma to the neck may also aggravate hind brain compression. Trauma can result in damage to the ANS and also to areas that will secondarily effect the ANS. Likewise, some believe the straining of bearing down during childbirth can aggravate hind brain compression and effect the ANS. POTS occurring as a result of the nutcracker phenomenon is also more prevalent after childbirth. Hormonal shifts have been theorized to contribute to the development or worsening of POTS symptoms after childbirth as well.
Travel by airplane is challenging for dysautonomics and may increase symptoms. Airplane cabins are pressurized to about 6,500 feet, which is high enough to cause some dysautonomia patients to hyperventilate. Hyperventilating makes a patient more likely to get symptoms of sympathetic activation (Robertson, 2002).
Those with POTS do need to know that the air in an airplane is some of the driest in the world. Flying can have dehydrating effects in normal individuals. How much more so flying might dehydrate someone with low blood volume. Everyone should be well hydrated before boarding a plane. Also, normal people sometimes have trouble with blood pooling in their legs during flights. Rarely, this can lead to the development of blood clots. Patients prone to pooling blood may want to wear compression stockings when flying. Patients may also want to request a bulkhead seat, as this will give them more room to elevate their legs. 

    1. Grubb, B. P. (2002, October). The heterogeneity of symptoms related to    dysautonomia. Symposium conducted at the meeting of the   National Dysautonomia Research Foundation Northwest Ohio   Support Group. Toledo, Ohio.     2. Grubb, B. P., & Karas, B. (1999). Clinical disorders of the autonomic   nervous system associated with orthostatic intolerance: an overview of   classification, clinical evaluation, and management. Pacing and Clinical   Electrophysiology, 22, 798-810. Full text:   http://www.ndrf.org/PDF%20Files/disorders.PDF     3. Hadjivassiliou, M., Gibson, A., Davies-Jones, G. A., Lobo,  A.J.,   Stephenson, T.J., & Milford-Ward, A. (1996). Does cryptic gluten     sensitivity play a part in neurological illness? Lancet, 10, 369-371.   PMID: 8598704 [PubMed - indexed for MEDLINE]     4. Mathias, C. J. (2000, July). Other autonomic disorders. National Dysautonomia   Research Foundation Patient conference. Minneapolis, Minnesota.     5. Robertson, D. (2002, July). Drug Therapy. National Dysautonomia   Research Foundation Patient conference. Washington, DC.     6. Sandroni, P. (1998, November/December). Testing the autonomic nervous     system. In C. B. Berde, & M. C. Rowbotham (Eds.) International    Association for the Study of Pain: Technical Corner From IASP    Newsletter. Full Text     7. Shen, W. K. (2002). Modification and ablation for inappropriate sinus   tachycardia: current status. Cardiac Electrophysiology Review.   6(4), 349-355. PubMed     8. Shen, W. K., Low, P.A., Jahangir, A., Munger, T. M., Friedman, P. A.,   Osborn, M. J., Stanton, M. S., Packer, D. L., Rea, R. F., Hammill, S. C.   (2001).  Is sinus node modification appropriate for inappropriate sinus   tachycardia with features of postural orthostatic tachycardia syndrome?   Pacing & Clinical Electrophysiology, 24(2), 217-230. PubMed     9. Terlizzi, R., Rocchi, C., Serra, M., Solieri, L., Cortelli, P. (2008). Reversible postural   tachycardia syndrome due to inadvertent overuse of Red Bull.   Clinical Autonomic Research, 18(4), 221-223. PubMed

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