edriscoll

<p>http://www.ncbi.nlm.nih.gov/pubmed/11990670?dopt=Abstract</p>

<p>http://www.thechiariinstitute.com/</p>

Managing & Enjoying Life After reading an article, use your browser's back button to easily return to the Table of Contents on this page. Surviving the Guilt that Comes with Chronic Illness by Amy Keys The Ins and Outs of Owning and Training a Service Dog by Ellen Driscoll Dehydration by Margaret Rose Lombardi Enjoying Life at National Parks by Hallie MacDonald Beat the Heat by Amy Keys Meet the Member: Erin's Story by Chelsea Goldstein Medical Q & A - Member Questions Answered by DINET's Medical Advisors Updated - Research and News about Dysautonomia and Related Illnesses Updated - Open Recruitment Studies

We welcome your letters to DINET's Medical Advisors. Please be aware that the information provided is not meant to be a diagnosis or medical advice. It is provided to give you background information to discuss with your medical team and general information to keep you well informed about dysautonomia disorders. If you have a question for our advisors, please send to webmaster@dinet.org Related Questions from Two Members: Question #1 from Member Bxxx: Hello, Please can you explain how someone can feel extremely lightheaded and faint when they are sitting with a completely normal heart rate and blood pressure? I understand you can still have low stroke volume and hence cardiac output, which is not measurable without invasive tests. I have heard other patients say they also feel presyncopal with normal observations, but medical professionals often do not seem to understand this. Many thanks Bxxxx Question #2 from Christina Hello, I’m a Dinet volunteer, username clb75. I have a question for the medical advisory board. Many people with Pots experience dizziness when upright even though their BP and heart rate are normal. What causes this and what are the best ways to treat this type of dizziness? - Christina Answer from Dr. Satish Raj I think that there are a couple of issues here – one of nomenclature and one of physiology. The nomenclature problem is with the word “dizzy”. It is not a medical term and could refer to two different medical problems: vertigo or light-headedness. Vertigo is where either the room spins around you or you spin within the room. This is usually not a blood pressure (or heart rate) issues but can be due to problems with the inner ear or brainstem. Light-headedness is the feeling that one is going to faint, although to a lesser degree than when actually fainting. In some languages, the term for light-headedness directly translates to “head spinning”, making it difficult at times to sort these out. The second issue, assuming that it is light-headedness that is being discussed, is physiology. Low blood pressure or a low or high heart rate DO NOT, in and of themselves, cause light-headedness. They do this presumably by altering blood flow in the brain, and perhaps the delivery of nutrients (such as oxygen or glucose) to the brain. This disruption could occur on a more local scale (within the brain) as well. Many things alter blood flow in the brain…many more than I can name. Something as simple as breathing can do so. Hyperventilation, for example, can do this. The challenge is that light-headedness is a common symptom that can be caused by many/most medical disorders. That is why, in many cases, more specific presentations (e.g. association with tachycardia or with fainting) are needed to help to narrow down the list of possibilities.

By Ellen Driscoll When an animal joins your life, you receive health benefits, both physical and mental, that are far beyond what most people expect. Pet ownership has proven to have a positive impact on depression, anxiety, blood pressure, and heart rate changes, to name a few. Add all of that to the companionship, loyalty, and love they give us, and is it any wonder that 68% of U.S. households have at least one furry family member? For people living with a chronic illness, a pet specially trained as a service animal brings another priceless asset...independence. When most people think of service animals, a dog is what comes to mind, and with good reason. Dogs are the most often used species trained to be of service for disabled people. Cats, dolphins, miniature horses, monkeys, ducks, parrots, and ferrets have all been trained to perform specific tasks as “service” to the disabled. However, the legal title of “service animal” is given to different species dependent on state requirements. (1) The ADA (the Americans with Disability Act) defines a Service Animal as a dog (2) “individually trained to do work or perform tasks for the benefit of an individual with a disability including a physical, sensory, psychiatric, intellectual or other mental disability.” The tasks can be a variety of things that help a person manage their day to day lives as long as it is directly related to the person’s disability. The ADA protects the rights the disabled person has regarding housing, workplace, and other public places where a person may want and need to be accompanied by their dog. Most states have specific rules regarding inclusion and exclusion of animals, and each state’s Attorney General’s office can provide the specifics for that state. Types Not all service animals do the same things. Besides guide dogs and hearing aid dogs, there are dogs trained for Mobility Assistance and Medical Emergency Response. Service Dogs can be trained to offer both types of assistance. There are emotional support animals that provide life-changing service for their owners also. While this category of animal is not recognized as a service animal by the ADA, most states will grant qualified, well-trained emotional support animals the same access to public spaces that an ADA dog has. There is a great site from the UK about the difference between emotional support animals and Service Dogs. The site, called “Dog Owner” is a wealth of information about the benefits of dog ownership for mental and physical health - https://www.dogowner.co.uk/dogs-mental-health/ A dog trained as a mobility assistance animal may provide help such as retrieving items from the floor, giving medication reminders, pushing elevator buttons, and many other helpful tasks. Medical emergency response animals frequently referred to as “seizure alert dogs” are trained to pick up changes in their owner’s bodies or behavioral cues that can warn that an emergency is about to take place, giving the owner a chance to react safely. This type of training has been critical for POTS and dysautonomia patients. If trained well, the dog can sense pre-syncope and give an alert signal, providing the owner time to move themselves to a safe space. They will also remain at attention by their owner’s side until the person is revived and can give the “okay” signal to the dog. Anyone who has been alone and incapacitated in a public place knows the potential dangers and the difference a service animal makes in that situation. Two of the most common breeds trained for this invaluable profession are Labrador Retrievers and Golden Retrievers. (3) These breeds are smart, loyal, and known for their patience, all qualities needed for this work. However, It is vital for people to understand and accept the difference between owning a service animal and a pet. There is a good reason that the jackets that these dogs wear in public ask people not to pet or interact with them. Unlike owning a pet that you would encourage to be people friendly, a service animal needs to be focused on the disabled person solely. The distractions inherent in public places can make it difficult enough for the animal. If people are petting and playing with the dog, they could easily miss the subtle cues necessary for them to do their job effectively; to provide the service they are trained to do, the dog needs to be attached to the person they are assisting. So regardless of what method you elect to use, there is always some training needed between the disabled person and the animal. Types of Training There are many ways to bring a service dog into your life. Buy an accredited Service Dog Bring your dog to a professional company for training. Train the dog yourself. Service dog training is a very long process and can be very expensive. For a dog to become accredited, the animal must be able to respond to commands and be proficient in specific skills. They also must be able to pass the Assistance Dogs International Public Access Test. Buying an accredited Service Dog Assistance Dogs International is an organization that establishes the standards for the Service Dog industry. ADI provides links to accredited companies that meet these standards. You can search for a company on their website - https://assistancedogsinternational.org/members/programs-search/ Buying a service dog is not as easy as just picking one out. The application process is a long one with the initial inquiry form taking 6 - 8 weeks to be reviewed. If the preliminary form is approved, then the actual application is sent out. Once received, it takes another 6 - 8 weeks for a medical review board to process the application. At that point, the person wanting a dog is either approved or disapproved. If you pass to this point, the process goes forward. The average cost for a Service Dog is $25,000. Many companies will work with approved applicants on fundraising and cost options if there is financial hardship. Most programs require the disabled person to stay at the company’s training facility for three weeks to a month to train the dog and the owner and to prepare them for the public access testing. Most programs include periodic training, evaluation, and recertifications as needed. Training Your Own Service Dog Some companies provide training for disabled people who have a dog that they believe would make an excellent Service Dog. However, the personality of a great pet is not necessarily the same as a great Service Dog. The American Kennel Club offers the following list of qualities that make a dog a good candidate for service: Calm but friendly Alert but not reactive Able to be touched by anyone, including strangers Willingness to please The natural tendency to follow you around Socialized to many different situations and environments Ability to learn quickly and retain information Many companies that offer training will also evaluate your dog to see if he or she is a good fit. They may also help you find a dog that they believe will offer the best chance for success. The upfront cost of a professional program is much less than buying an already accredited dog, averaging $5,000 to $10,000. However, there is not a guarantee that the dog will pass the accreditation test the first time. Also, this upfront fee doesn’t include ongoing training or recertification in most cases. Those fees are usually in addition to the upfront costs. It is important to talk to the company or the ADI to find out about any ongoing costs required to maintain accreditation. Most professional training programs require about six months to complete all courses and prepare for the access test. Many organizations will offer training classes where professional trainers work in small groups providing the guidance needed for the owner to work at home with the dog to develop those skills. They also will offer private training that involves one-on-one instruction between the owner and the dog. There has been a recent trend toward owners taking on training without hiring a professional. There are many resources online and blogs by people who have trained their animals. On the site, “The New Mobility,” Holly Koester (4) describes the process of teaching her black lab basic obedience and additional commands before seeking the specific training her dog would need for accreditation. Koester clicker trained her dog to be proficient with “sit,” “lay down,” “stay,” as well as other basic commands. After she had full confidence in that stage of learning, she moved on to teach commands like “pull” and “push,” giving her dog the headstart toward learning the skills to offer assistance in things like opening a door or closing a drawer. Koester estimates this approach shortened the amount of time her dog Spokes needed for professional assistance training down to three months. No matter what the approach, training a service animal begins with teaching the owner. For people who don’t feel equipped to handle the basic obedience pre-training, there are companies like Top Dog located in AZ, (5) and The Dog Alliance in TX, (6) for example, that offer in-person and online classes for owners. Top Dog’s introductory class pairs owners with volunteer training assistants, and guides the owner through teaching the dog the basics and also how to understand the dog and how they communicate with you. The program goes on to the Intermediate, which begins the specific course work for assistance skills. Eventually, the owner and the dog are ready for the Assistance Dog Exam. Although the program is considerably less costly (the intro course is $200), it may not save you any time. Top Dog estimates a year and a half of training until the exam and much longer if a dog or owner doesn’t catch on to commands the first time around. The Dog Alliance provides accredited dogs as well as owner training classes. The introductory course requires an owner to take a preliminary seminar on basic obedience training and that their dog either pass that class or has instructor approval to move on to the Service Dog training class. The advanced class focuses on assistance skills and preparation for the Assistance Test. There are also training manuals and online training available to owners who have prior experience with basic command training or have owned a Service Dog before. Regardless of the path taken to get there, the goal should be to have a well-trained animal capable of passing the Assistance Test for Service Animals. Without this credential, there is always a safety risk in using an animal for mobility assistance. A Final Word Regardless of the type of training you decide to use, always check for the certification of the program and professionals you choose by researching the company through the Assistance Dogs International site - https://assistancedogsinternational.org/ This organization sets the standards for assistance animals, and trainers, as well as providing the official test for dogs to be accredited Service Dogs. It is critical to understand that even though it is costly and time-consuming to go through all the hoops necessary to have an accredited Service Dog, it is dangerous and irresponsible not to. To protect the rites of the disabled population, restaurants and other public places are not allowed to question whether an animal is accredited or not, or why they are needed. Also, there are service dog vests that can be bought online without proof that the buyer is disabled or the dog is accredited. Unfortunately, this has led to some people abusing this law as a way of bringing their pets along to places they would not be permitted. This behavior puts accredited service animals at risk and puts any disabled person in their vicinity in unnecessary danger. A Service Dog that finds a human partner as dedicated as they are to be consistent in their training, generous in their praise and affection, and focused on one another, will have a better quality of life together than they ever would have had alone. For more information on Service Animals, visit these resources: https://assistancedogsinternational.org/ https://www.ada.gov/service_animals_2010.htm https://www.akc.org/expert-advice/training/service-dog-training-101/ http://www.newmobility.com/2006/12/training-your-own-service-dog/ https://www.thedogalliance.org/training-your-own-service-dog https://www.dogowner.co.uk/dogs-mental-health/ For personal stories and to connect with the owners of Service Animals, visit: Hannah's Story: https://www.dinet.org/member-stories/lean-on-me-the-remarkable-story-of-a-young-woman-with-pots-and-the-dog-that-keeps-her-safe-r208/ Service Dogs for POTS - https://www.facebook.com/groups/110152023042832/ - this is a closed Facebook group, but if you send them a message to join or to get more info, they are very responsive. RESOURCES 1. Federal and State laws regarding Service Animals can be found at https://usaservicedogregistration.com/service-dog-state-laws/ 2. In rare cases, the ADA will allow for a miniature horse to be certified as a Service Animal. https://adata.org/faq/i-heard-miniature-horses-are-considered-be-service-animals-ada-true 3. AKC Service Dog Training 101, https://www.akc.org/expert-advice/training/service-dog-training-101/ 4. Training Your Own Service Dog by Roxanne Furlong, http://www.newmobility.com/2006/12/training-your-own-service-dog/ 5. “Top Dog Teamwork” from the article Training Your Own Service Dog by Roxanne Furlong http://www.newmobility.com/2006/12/training-your-own-service-dog/ 6. Service Dog Training, Out and About https://thedogalliance.asapconnected.com/#CourseGroupID=12147

T H R I V I N G... 5 Tips for Traveling with Dysautonomia by Chelsea Goldstein Finding Workarounds by Amy Keys Technology and Chronic Illness by Reanna Mathis Living with Hyperadrenergic POTS: A Personal Story by Susanne Rimm Finding Balance by Trudi Davidoff Meet the Member: Isabelle's Story by Chelsea Goldstein Medical Q & A - Your Questions answered by DINET's Medical Advisors Updated - Open Recruitment Studies Updated - Research and News about Dysautonomia and related Chronic Illnesses Celebrating our Volunteers: Melissa Milton: 2018 Rare Artist Contest brings Awareness to Dysautonomia Lauren Mlack: Meet DINET's Social Media Coordinator

T H R I V I N G... 5 Tips for Traveling with Dysautonomia by Chelsea Goldstein Finding Workarounds by Amy Keys Technology and Chronic Illness by Reanna Mathis Living with Hyperadrenergic POTS: A Personal Story by Susanne Rimm Finding Balance by Trudi Davidoff Meet the Member: Isabelle's Story by Chelsea Goldstein Medical Q & A - Your Questions answered by DINET's Medical Advisors Updated - Open Recruitment Studies Updated - Research and News about Dysautonomia and related Chronic Illnesses Celebrating our Volunteers: Melissa Milton: 2018 Rare Artist Contest brings Awareness to Dysautonomia Lauren Mlack: Meet DINET's Social Media Coordinator

We welcome your letters to DINET's Medical Advisors. Please be aware that the information provided is not meant to be a diagnosis or medical advice. It is provided to give you background information to discuss with your medical team and general information to keep you well informed about dysautonomia disorders. If you have a question for our advisors, please send to webmaster@dinet.org Question from member FIONA: I have been recently diagnosed with Dysautonomia following many years of periodic symptoms. I don’t fit into any specific category at the moment although I also suffer from adrenaline surges which suggest an overactive SNS. My body literally crashed last September and I have been desperately trying to deal with my daily symptoms since then. My TTT showed unstable fluctuations in my BP although I didn’t pass out. I still await blood results for lying/standing norepinephrine levels. I’m emailing your team as my most worrisome and debilitating symptoms are now from Orthostatic headaches. Once upright, I quickly feel discomfort/pressure/pain in the back of my neck and head. This intensifies and I then feel weak in my extremities, slightly nauseous and need to lie down immediately. If I don’t lie down fast enough, my SNS triggers adrenaline surges which spike my BP, raise my HR, flush my face and cause me to feel terror as if my head will explode. The intensity and duration of these surges is unpredictable and I always feel exhausted afterward. I’m especially concerned as my ability to remain upright has slowly decreased over the past few weeks due to this headache issue. It’s now difficult to even walk a few steps without the head pressure feeling. In addition, I have suffered from painful shoulder muscles for many years. My other Dysautonomia symptoms wax and wane but this is quite unbearable. I also suffer from migraines and am perimenopausal. Have you a likely explanation for why these headaches are occurring? Is my brain simply starved of oxygen? I’m guessing that my SNS is then acting in an overcompensatory manner? Have you any advice on coping mechanisms? I just don’t know what my new normal is or how to begin to work through this. Any help/advice greatly appreciated. Answer from Dr. Satish Raj: I am sorry to hear you are unwell. Headaches are commonly seen in patients with various autonomic disorders, and they can be difficult to manage. I do not think that these are due to the brain not getting enough oxygen. That can lead to loss of consciousness and not "pain". I would suggest that it is important to make sure that your BP is adequately supported. If you are dropping your BP or perfusion, that could certainly account for the shoulder pain on standing and maybe head/neck pain. Another consideration is that there are some patients that can develop spontaneous leaks of their cerebrospinal fluid. The headaches are like "spinal headaches" that people can experience sometimes after epidurals that go poorly. These can be hard to diagnose, but one can start by looking at an MRI of the brainstem and occasionally doing a myelogram (contrast into the spinal canal to look for leaks). The treatment often starts with attempts at a "blood patch" (blood injection into the spinal canal to try to clot and "patch" the hole). Unfortunately, they are often less effective in this condition with the spontaneous leaks than in patients where a needle caused the hole. Some patients also get better for several days and then get worse again. However, some do get better. Dr. Satish Raj Dr. Satish R. Raj MD, MSCI, FPCPC Associate Professor of Cardiac Sciences Libin Cardiovascular Institute of Alberta University of Calgary | Vanderbilt University Question from member Jan: My cardiologist said I have dysautonomia. My BP is both very low with passing out or very high eg 220/125. In the last 12 months, my high BP has caused some heart damage, left ventricular thickening, left atrial enlargement, mitral valve regurgitation. A geneticist last year said I meet all the criteria for EDS hypermobility Syndrome with the exception of high as well as low bp. I also have chronic secretory diarrhea which appears to have some autonomic component. This does not match the criteria for EDS. Had a normal nerve conduction test. My problem is how to treat labile bp so that treatment doesn’t worsen passing out. My treatment at present is to take a small amount of short-acting bp reducing medicine if bp over 180 sustained for 1 hour, max 3in one day. However, on the occasion, I took two in one day I was on the floor and bp extremely low for hours. Most information on dysautonomia is about dropping bp. Are you aware of my problem and any advice on management? Answer from Dr. Nicholas Tullo: The term “dysautonomia” includes many different disturbances of the part of the nervous system that controls heart rate and blood pressure. However, many factors affect blood pressure other than the autonomic nervous system. These factors include the total amount of blood and fluid in your circulatory system, the strength of your heart’s contraction, and the health of your venous system. The veins become an important issue because a significant portion of a person’s circulating blood volume can wind up pooled in the veins of the legs and even the lower abdominal cavity. The body’s ability to maintain a normal blood pressure depends heavily on how much blood is getting back to the heart since the heart has to be able to fill properly (“what goes in must come out”). When most of the blood is in the “wrong place” (stuck in the lower parts of the body) the blood pressure can drop dramatically. Chronic diarrhea represents an excessive amount of fluid loss that tips the scales towards being a little dehydrated, which just compounds the problem. Hypertension may be a manifestation of age (stiff blood vessels) and “essential hypertension,” which is probably a genetic abnormality in the hormonal control of blood vessel constriction. Ultimately, maintaining an adequate fluid intake is key to avoiding hypotension. People with blood pressure instability should be taking in at least 2-3 liters of fluid per day (maybe more). Having EDS may affect the elasticity of the veins and exaggerate the pooling that goes on when a person stands up for more than a few minutes. External ways to reduce blood pooling includes thigh-high or waist-high compression hose or abdominal binders. They can make a big difference. Hypertension needs to be treated with medications because of the potential organ damage that comes with excessively high blood pressures. Unfortunately, there is no one medication that solves the problem, and many patients with such an unstable blood pressure have to monitor it closely and make day-to-day adjustments in their meds (like you are doing). It is very important to avoid hypertension at night during sleep, since that induces an increase in urine production at night, leading to very wide blood pressure changes in the early morning hours. Sleeping with the head of your bed elevated 4-6 inches may help reduce nighttime urine production as well. Sometimes it’s a juggling act to have to take medications like fludrocortisone and midodrine to prevent hypotension, but taking hypertension meds… usually something long-acting in the morning and perhaps short-acting like nifedipine or nitrates to take later on if the blood pressure gets too high. You need to find a doctor nearby who is willing to work with you and help you devise a medication strategy that will help you keep your blood pressure in an acceptable range. Generally, daytime blood pressures in the 140-160 mmHg range may be reasonable in patients who are prone to severe orthostatic drops in order to avoid symptoms such as fainting. Best of luck! Nicholas G. Tullo, MD, FACC, FHRS New Jersey Center for Fainting njfaint.com Question from member Megan: Hello there! I wanted to ask a question about Beta Blockers use and Dysautonomia. We have malfunctions of our Autonomic Nervous System and my understanding is that Beta Blockers may increase the amount of Beta-Adrenergic receptors. It seems as though this would then make it more difficult to ever withdraw the Beta Blockers because withdrawal would cause a massive adrenergic response until the receptors down-regulated. It would make it difficult to know when Beta Blockers were no longer needed. The Nervous System is already not functioning properly and then it is stressed even more when the medication is withdrawn. What are your thoughts on that? Answer from Dr. Svetlana Blitshteyn: Beta-blockers may cause an indirect upregulation of the beta-adrenergic receptors in response to blocking of the beta receptors, but in our experience, beta blockers are excellent for many patients with POTS, chronic headache, anxiety, chest pain and intermittent tachycardia at rest. We have not observed patients having difficulty weaning off or stopping beta blockers when their symptoms no longer necessitate the use of beta blockers. Dysautonomia is not a static disorder; it can change in the type and severity of symptoms over a period of months to years. Many patients may use beta blockers for extended periods of time, then wean off or switch to other medications if the use of beta blockers is no longer needed or preferred. We have not observed major deterioration in symptoms when a decision to stop or wean off beta blockers is made appropriately by the specialist and the patient. Sincerely, Dr. Blitshteyn Svetlana Blitshteyn, MD Director and Founder of Dysautonomia Clinic and Amherst Neurology Clinical Assistant Professor of Neurology University at Buffalo School of Medicine and Biomedical Sciences Phone: 716-531-4598 Fax: 716-478-6917 http://www.dysautonomiaclinic.com Return to 02/2019 Table of Contents

We welcome your letters to DINET's Medical Advisors. Please be aware that the information provided is not meant to be a diagnosis or medical advice. It is provided to give you background information to discuss with your medical team and general information to keep you well informed about dysautonomia disorders. If you have a question for our advisors, please send to webmaster@dinet.org Question from member FIONA: I have been recently diagnosed with Dysautonomia following many years of periodic symptoms. I don’t fit into any specific category at the moment although I also suffer from adrenaline surges which suggest an overactive SNS. My body literally crashed last September and I have been desperately trying to deal with my daily symptoms since then. My TTT showed unstable fluctuations in my BP although I didn’t pass out. I still await blood results for lying/standing norepinephrine levels. I’m emailing your team as my most worrisome and debilitating symptoms are now from Orthostatic headaches. Once upright, I quickly feel discomfort/pressure/pain in the back of my neck and head. This intensifies and I then feel weak in my extremities, slightly nauseous and need to lie down immediately. If I don’t lie down fast enough, my SNS triggers adrenaline surges which spike my BP, raise my HR, flush my face and cause me to feel terror as if my head will explode. The intensity and duration of these surges is unpredictable and I always feel exhausted afterward. I’m especially concerned as my ability to remain upright has slowly decreased over the past few weeks due to this headache issue. It’s now difficult to even walk a few steps without the head pressure feeling. In addition, I have suffered from painful shoulder muscles for many years. My other Dysautonomia symptoms wax and wane but this is quite unbearable. I also suffer from migraines and am perimenopausal. Have you a likely explanation for why these headaches are occurring? Is my brain simply starved of oxygen? I’m guessing that my SNS is then acting in an overcompensatory manner? Have you any advice on coping mechanisms? I just don’t know what my new normal is or how to begin to work through this. Any help/advice greatly appreciated. Answer from Dr. Satish Raj: I am sorry to hear you are unwell. Headaches are commonly seen in patients with various autonomic disorders, and they can be difficult to manage. I do not think that these are due to the brain not getting enough oxygen. That can lead to loss of consciousness and not "pain". I would suggest that it is important to make sure that your BP is adequately supported. If you are dropping your BP or perfusion, that could certainly account for the shoulder pain on standing and maybe head/neck pain. Another consideration is that there are some patients that can develop spontaneous leaks of their cerebrospinal fluid. The headaches are like "spinal headaches" that people can experience sometimes after epidurals that go poorly. These can be hard to diagnose, but one can start by looking at an MRI of the brainstem and occasionally doing a myelogram (contrast into the spinal canal to look for leaks). The treatment often starts with attempts at a "blood patch" (blood injection into the spinal canal to try to clot and "patch" the hole). Unfortunately, they are often less effective in this condition with the spontaneous leaks than in patients where a needle caused the hole. Some patients also get better for several days and then get worse again. However, some do get better. Dr. Satish Raj Dr. Satish R. Raj MD, MSCI, FPCPC Associate Professor of Cardiac Sciences Libin Cardiovascular Institute of Alberta University of Calgary | Vanderbilt University Question from member Jan: My cardiologist said I have dysautonomia. My BP is both very low with passing out or very high eg 220/125. In the last 12 months, my high BP has caused some heart damage, left ventricular thickening, left atrial enlargement, mitral valve regurgitation. A geneticist last year said I meet all the criteria for EDS hypermobility Syndrome with the exception of high as well as low bp. I also have chronic secretory diarrhea which appears to have some autonomic component. This does not match the criteria for EDS. Had a normal nerve conduction test. My problem is how to treat labile bp so that treatment doesn’t worsen passing out. My treatment at present is to take a small amount of short-acting bp reducing medicine if bp over 180 sustained for 1 hour, max 3in one day. However, on the occasion, I took two in one day I was on the floor and bp extremely low for hours. Most information on dysautonomia is about dropping bp. Are you aware of my problem and any advice on management? Answer from Dr. Nicholas Tullo: The term “dysautonomia” includes many different disturbances of the part of the nervous system that controls heart rate and blood pressure. However, many factors affect blood pressure other than the autonomic nervous system. These factors include the total amount of blood and fluid in your circulatory system, the strength of your heart’s contraction, and the health of your venous system. The veins become an important issue because a significant portion of a person’s circulating blood volume can wind up pooled in the veins of the legs and even the lower abdominal cavity. The body’s ability to maintain a normal blood pressure depends heavily on how much blood is getting back to the heart since the heart has to be able to fill properly (“what goes in must come out”). When most of the blood is in the “wrong place” (stuck in the lower parts of the body) the blood pressure can drop dramatically. Chronic diarrhea represents an excessive amount of fluid loss that tips the scales towards being a little dehydrated, which just compounds the problem. Hypertension may be a manifestation of age (stiff blood vessels) and “essential hypertension,” which is probably a genetic abnormality in the hormonal control of blood vessel constriction. Ultimately, maintaining an adequate fluid intake is key to avoiding hypotension. People with blood pressure instability should be taking in at least 2-3 liters of fluid per day (maybe more). Having EDS may affect the elasticity of the veins and exaggerate the pooling that goes on when a person stands up for more than a few minutes. External ways to reduce blood pooling includes thigh-high or waist-high compression hose or abdominal binders. They can make a big difference. Hypertension needs to be treated with medications because of the potential organ damage that comes with excessively high blood pressures. Unfortunately, there is no one medication that solves the problem, and many patients with such an unstable blood pressure have to monitor it closely and make day-to-day adjustments in their meds (like you are doing). It is very important to avoid hypertension at night during sleep, since that induces an increase in urine production at night, leading to very wide blood pressure changes in the early morning hours. Sleeping with the head of your bed elevated 4-6 inches may help reduce nighttime urine production as well. Sometimes it’s a juggling act to have to take medications like fludrocortisone and midodrine to prevent hypotension, but taking hypertension meds… usually something long-acting in the morning and perhaps short-acting like nifedipine or nitrates to take later on if the blood pressure gets too high. You need to find a doctor nearby who is willing to work with you and help you devise a medication strategy that will help you keep your blood pressure in an acceptable range. Generally, daytime blood pressures in the 140-160 mmHg range may be reasonable in patients who are prone to severe orthostatic drops in order to avoid symptoms such as fainting. Best of luck! Nicholas G. Tullo, MD, FACC, FHRS New Jersey Center for Fainting njfaint.com Question from member Megan: Hello there! I wanted to ask a question about Beta Blockers use and Dysautonomia. We have malfunctions of our Autonomic Nervous System and my understanding is that Beta Blockers may increase the amount of Beta-Adrenergic receptors. It seems as though this would then make it more difficult to ever withdraw the Beta Blockers because withdrawal would cause a massive adrenergic response until the receptors down-regulated. It would make it difficult to know when Beta Blockers were no longer needed. The Nervous System is already not functioning properly and then it is stressed even more when the medication is withdrawn. What are your thoughts on that? Answer from Dr. Svetlana Blitshteyn: Beta-blockers may cause an indirect upregulation of the beta-adrenergic receptors in response to blocking of the beta receptors, but in our experience, beta blockers are excellent for many patients with POTS, chronic headache, anxiety, chest pain and intermittent tachycardia at rest. We have not observed patients having difficulty weaning off or stopping beta blockers when their symptoms no longer necessitate the use of beta blockers. Dysautonomia is not a static disorder; it can change in the type and severity of symptoms over a period of months to years. Many patients may use beta blockers for extended periods of time, then wean off or switch to other medications if the use of beta blockers is no longer needed or preferred. We have not observed major deterioration in symptoms when a decision to stop or wean off beta blockers is made appropriately by the specialist and the patient. Sincerely, Dr. Blitshteyn Svetlana Blitshteyn, MD Director and Founder of Dysautonomia Clinic and Amherst Neurology Clinical Assistant Professor of Neurology University at Buffalo School of Medicine and Biomedical Sciences Phone: 716-531-4598 Fax: 716-478-6917 http://www.dysautonomiaclinic.com Return to 02/2019 Table of Contents

Meet the woman behind the Survey Sunday feature on DINET's Facebook page. Lauren also is one of the volunteers posting articles about living with dysautonomia and chronic illness. Meet one of DINET's most valued volunteers. Lauren lives in Cleveland, OH with her wonderful parents and Goldendoodle, Riley. She loves cooking, baking, crafting and making metal jewelry in her spare time. When she was little, she was always having health issues that would come and go randomly and cause lots of fatigue. Her mom kept pushing for an answer after going to countless specialists. Finally, when she was 12, she had a tilt table test which diagnosed her with POTS. After having to leave public school and switch to online schooling due to her health, she was finally feeling well enough almost a decade later to attend a local community college. Lauren recently was able to achieve an Associate’s degree in graphic design. She is very excited that she can use her skills to help a great cause like DINET. Editor's Note: Over the next few months, Lauren will be preparing a new video feature for DINET's Youtube channel. The feature will focus on a great variety of subjects. If you have a topic that you would love to hear more about or would like to see DINET cover, email: webmaster@dinet.org Above: Lauren and her Goldendoodle, Riley Return to 02/2019 Table of Contents

Meet the woman behind the Survey Sunday feature on DINET's Facebook page. Lauren also is one of the volunteers posting articles about living with dysautonomia and chronic illness. Meet one of DINET's most valued volunteers. Lauren lives in Cleveland, OH with her wonderful parents and Goldendoodle, Riley. She loves cooking, baking, crafting and making metal jewelry in her spare time. When she was little, she was always having health issues that would come and go randomly and cause lots of fatigue. Her mom kept pushing for an answer after going to countless specialists. Finally, when she was 12, she had a tilt table test which diagnosed her with POTS. After having to leave public school and switch to online schooling due to her health, she was finally feeling well enough almost a decade later to attend a local community college. Lauren recently was able to achieve an Associate’s degree in graphic design. She is very excited that she can use her skills to help a great cause like DINET. Editor's Note: Over the next few months, Lauren will be preparing a new video feature for DINET's Youtube channel. The feature will focus on a great variety of subjects. If you have a topic that you would love to hear more about or would like to see DINET cover, email: webmaster@dinet.org Above: Lauren and her Goldendoodle, Riley Return to 02/2019 Table of Contents