In theory, young people with CFS and orthostatic intolerance (plus or minus some form of hypermobility) should not be at greatly increased risk of severe coronavirus disease compared to others their age, provided they don’t have untreated respiratory conditions like asthma.
The data from China and from the state of Washington both suggest that not only do children and adolescents have milder disease and fewer hospitalizations, but they also seem to get the coronavirus less frequently. Only 1% of the known cases in China were children, even though children represent 20% of the Chinese population. That is a curious phenomenon, and might ultimately prove to be due to the fact that the young people were asymptomatic and didn't go in with illnesses that would warrant testing. In any event, it speaks to the milder nature of the illness in younger people.
The epidemiologic data suggest a marked increase in disease severity for each decade above 50-60, especially for those with lung disease like chronic obstructive pulmonary disease (COPD), high blood pressure, and diabetes. So, my pediatric and young adult patients should be relatively protected from severe disease because of their younger age. They also know more than most about social isolation and avoiding illness than most of the general public, so they have much to teach others about how to manage. That said, we don’t have all the facts to make good decisions yet. All I can tell you is that I have not had anyone from my clinic develop coronavirus thus far, acknowledging that we are early in the pandemic.
I completely agree with being cautious. The more we can do now to prevent person-to-person spread, the less likely we will be to replicate the Italian experience where the hospitals are overwhelmed. Using all of the CDC recommendations makes good sense (hand washing, elbow bumps, sanitizer, social isolation, avoiding crowds). On the issue of using face masks, I differ from some of my medical colleagues. Many of their comments are an attempt to deal with limited supply of face masks. But, we are all advised in the hospital to don a face mask when dealing with patients who have respiratory symptoms. So, as a protective measure I would wear a mask in public even though the official recommendation is that it won’t stop the spread of droplets of virus. I suspect it will stop at least some droplets, but the neglected point about facemasks is that they help remind you to not touch your face and inoculate the nasal passages with virus from your hands. They may also create a bit of social isolation, as people will keep their distance from anyone wearing a mask! Some people have been sewing their own facemasks, in an effort to help address the shortages. This is an amazingly positive effort.
My Pediatric Pulmonary colleagues do not think asthma will be a big risk factor, based on their reading of the evidence. Similarly, we don’t think EDS and mast cell activation will necessarily confer an increased risk.
Most classes have been cancelled by now. For those who are working, I would try to work remotely, especially in the next couple of weeks. This might be acting in an overly cautious manner, but I think none of us have to apologize for being overly cautious at this juncture.
The key at this stage is to be very rigid and strict about complying with the recommendations for social distancing and isolation. Remind your friends and family (skeptical or not) that this is no joke, and that lives depend on us pulling together like we all did in response to 9/11/2001. We can do this!
Peter Rowe, MD
Director, Children's Center Chronic Fatigue Clinic
John Hopkins University School of Medicine
Professor of Pediatrics
Adolescent Medicine, Chronic Fatigue Syndrome, Orthostatic Intolerance, Pediatrics, Postural Orthostatic Tachycardia Syndrome (POTS), Syncope
Chronic fatigue syndrome; Fibromyalgia; Elhers-Danlos Syndrome; Orthostatic intolerance syndromes; Gulf War illnesses; Pelvic congestion syndrome
World Health Organization: Rolling Updates on COVID-19
World Health Organization: COVID-19 Online Courses
Centers for Disease Control & Prevention: Guidelines for people at High Risk
Centers for Medicare and Medicaid Services: Current Emergencies
Centers for Medicare and Medicaid Services: Coronavirus (COVID-19) Partner Toolkit
Cambridge Rare Disease: Current UK NHS & Government Information & Guidance
City of Little Rock: Experiencing symptoms related to COVID-19?
City of Little Rock: ¿Tiene síntomas relacionados con COVID-19? (Spanish)
Food & Drug Administration:COVID-19 What’s New and Fast Facts
National Institutes of Health: COVID-19 Updates
World Health Organization: Coronavirus Information
Centers for Disease Control & Prevention: Coronavirus Information Center
U.S. Government: State Health Department Directory
Coronavirus (COVID-19) Education & Information
Día de la Mujer Latina: Dispelling the Myths and Rumors of the New Coronavirus Disease (Webinar)
Día de la Mujer Latina: Disipando los Mitos y Rumores del Nuevo Coronavirus (Webinario)
GRIN2B Foundation: Flip Book Social Story
SCN2A Australia: Podcast on COVID and Epilepsy
Coronavirus Support Network: FAQ (English)
Coronavirus Support Network: FAQ (Spanish)
Coronavirus Support Network: FAQ (Portuguese)
National Health Council: COVID-9 Preparedness for people with chronic disease (Webinar)
Association of Community Cancer Centers: COVID-19 Resource Center
American Society of Clinical Oncology: Coronavirus Resources
American Academy of Neurology: COVID-19 Neurology Resource
American Thoracic Society: Public Health Information Sheet
American Thoracic Society: COVID-19 FAQ
Laughing at My Nightmare: COVID-19 Resource Relief
Arkansas Community Foundation: Arkansas COVID Relief Fund
Ehlers-Danios Society: Virtual Chat Rooms
Twitter QA: #spooniechat
Our Odyssey: Online Meetups
The Mighty: Come Hang with the Mighty Events
Robert Wood Johnson Foundation Culture of Health Leaders: Coronavirus Support Network
Global Healthy Living Foundation: COVID-19 Support Program for Chronic Disease Patients and Their Families
Cancer Support Community: What Cancer Patients, Survivors, and Caregivers Need to Know About the Coronavirus
Centers for Disease Control & Prevention: Managing Stress and Anxiety During COVID-19
Happy: Online Mental Health Support
Talking to Kids
Kids Health, Nemours: How to Talk to Your Kids About COVID-9
KinderCare: Talking to Children About Coronavirus
At Home Teaching Resources
District Administration: Free K-12 Resources
We Are Teachers: 130+ Amazing Online Learning Resources
Diagnosis, Treatments & Vaccines
Milken Institute, FasterCures: COVID-19 Treatment and Vaccine
U.S. Food & Drug Administration: Clinical Trial Guidance
Mindray: COVID-19 Clinical Diagnostics Webinar Episode 1
Mindray: COVID-19 Clinical Diagnostics Webinar Episode 2
Mindray: COVID-19 Clinical Diagnostics Webinar Episode 3
Mindray: COVID-19 Experts Dialogues
Coverage & Benefits
Centers for Medicare & Medicaid Services: Coverage & Benefits Related to COVID-19 Medicaid and CHP
Centers for Disease Control & Prevention: Communication Resources
Patient Advocate Foundation: COVID-19 Operational Response Plan
Nielsen Training & Consulting, LLC: COVID-19 Resources for Nonprofit Leaders
Orangetheory: Orangetheory At Home
Gold’s Gym: Stronger Anywhere
Peloton: Free App
Planet Fitness: Home Work-Ins
Crunch: Crunch Live
305 Fitness: Group Classes
Corepower Yoga: Keep Up Your Practice
American Heart Association: Choose Your Own Workout
Two Disabled Dudes: Podcast
Global Genes: RARECast (Podcast)
Rare in Common: Podcast
Instagram Live: John Legend
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Global Genes: RARE Daily
Below are articles full of information designed to help people living with dysautonomia. There are guides written to help your family and friends understand your illness and help you all learn to live with it together. The POTS Guide for kids was created to be used as a handout for your child's teacher, coach, tutor, school nurse, their best friend's mom, or anyone else that may be responsible for your child when you are not there. Many guides include links to pdfs to make it easier to print, email or share. The Patient's Guide is to help patients with tips to prepare for a visit to the ER or a new medical provider. The Information Sheets are designed to help you remember the information you should have with you. The Life with Chronic Illness section includes links to newsletter articles on a variety of subjects about the issues faced for people with chronic illness.
A guide for family and friends
POTS GUIDE - for the family of children & teens living with POTS
I know a kid who has POTS
Hydration and POTS
Preparing for the ER and new medical visits
LIFE WITH CHRONIC ILLNESS
Managing and enjoying life - surviving guilty feelings, service dogs, dehydration, beat the heat, visiting national parks.
Thriving with chronic illness - traveling, adaptive devices, technology, hyperPOTS patient story, finding balance.
Paths and Stories After reading an article, use your browser's back button to easily return to the Table of Contents on this page.
Mental Health and Chronic Illness by Hallie MacDonald Lefty-Loosey, Righty-Tighty: The Autonomic Nervous System, Blood Vessels and POTS by Susanne Rimm Meet the Member: Claire's Story by Chelsea Goldstein The Story Booth Project: Sharing the Stories of Patients and Caregivers by Ellen Driscoll Meet DINET's Board of Directors Updated: Research and News about Dysautonomia and Related Illnesses Updated: Open Recruitment Studies
By Hallie MacDonald
Q: Statistics report an estimate of 25 to 33% of people living with chronic illness also suffering from depression. What is the difference between situational or stress-related depression and chronic or major depression? Is one type more common for people with chronic illness?
A. First, it is essential to understand the differences between different types of depression. Clinical depression (also called Major Depressive Disorder) often refers to one or more serious depressive disorders that can occur with or without the person experiencing a specific stressor. The symptoms are generally more severe or last far longer in comparison to situational depression, and they usually will interfere with the person’s ability to function in their day to day life (work, school, family responsibilities,etc.). Common examples of clinical depression are:
Dysthymia - persistent depressive disorder - a low-grade depression lasting two or more years.
Seasonal Affective Disorder (SAD) - depression related to the reduction in natural light usually begins at the start of the fall/winter seasons.
Postpartum depression - can be debilitating and has various degrees of severity following pregnancy and giving birth.
Bipolar depressive disorder - the person experiences alternating periods of elation and depression.
A person experiencing a major depressive disorder will generally have five or more of the following symptoms and requires a medical diagnosis:
Sleep difficulties, changes to sleep patterns, including waking unusually early without desire or need.
The inability to feel joy or take pleasure in most or all daily activities.
Noticeable agitation or unusual slow movements - extreme lethargy
Feelings of worthlessness, self-loathing or self-hatred
Excessive feelings of guilt
Suicidal thoughts or beliefs that the world/family may be better off without you.
Significant weight loss or gain in a month without intention (5% or more of body weight)
In children - increased irritability
Commonly, major depressive disorders require medication as well as therapy to manage symptoms and learn coping skills. Clinical depression can be severe and requires the intervention of a medical professional. If you believe you or a loved one may be experiencing symptoms of clinical depression, contact a PCP or mental health professional for diagnosis and treatment.
Situational depression refers to symptoms of depression that are related to one or more specific stressors the person is experiencing. Situational depression is common after significant life changes such as divorce, death, job loss, etc.
Situational depression can cause emotional and behavioral symptoms that can look and feel very much like clinical depression. However, it is important to remember that it is reasonable to feel sad or anxious when life circumstances are difficult or overwhelming. The treatment for situational depression is supportive, such as seeking out organizations or support services that may help by giving and receiving support from others who have experienced similar stressors, such as; grief support groups or chronic illness support services.
Most people who are living with chronic illness experience situational depression or stress at different times during their lives. It is normal and expected. There is a period of mourning and feelings of grief as they adjust to the parts of their life that may be lost and while they adapt to a new self-image. This period of mourning is different for everyone. Finding a support group for people with related physical symptoms or similar disabilities is an essential tool because it offers the opportunity to feel validated and supported as well as to give validation and support to others in a similar situation. Just as crucial for people with a chronic illness is to develop coping skills to help with the everyday stress inherent in dealing with pain and illness regularly. Finding new activities or new ways to enjoy old activities can be an effective de-stressor, as well as continuing to connect with family and friends.
It is critical to recognize that more than one type of depression can be present at any time in a person’s life. Therefore anyone experiencing symptoms of depression must be screened by a medical professional. Suicidal thoughts experienced by you or a loved one requires immediate attention, contact the National Suicide Prevention Association at www.suicidepreventionlifeline.org or call the 24/7 hotline at 1-800-273-8255
Resource: National Association of Mental Illness https://www.nami.org/Blogs/NAMI-Blog/April-2017/Situational-Symptoms-or-Serious-Depression-What-s
Q: People living with dysautonomia disorders are commonly treated with BP medications, many of them with depression as a side-effect. How can medication-induced depression be treated? Is the treatment different than other types of depression? How can you tell if the depression is a side-effect or a symptom of the illness or a condition unrelated to dysautonomia?
A. Determining whether the symptoms are a side effect of the medication or are coming from another source requires an in-depth patient history. A physical exam and lab findings confirm the amount of medication in the bloodstream, as well as any additional intoxicants that may be present.
There is no specific treatment for depressive feelings brought on by medication use, other than switching to another medication. Usually, the side effects of most medications lessen or disappear as the body acclimates to it. If there is no improvement, then an alternative treatment will be needed. If the symptoms persist after four weeks, another source for the depression needs consideration.
Q: Anxiety is also prevalent with dysautonomia patients because so many of the symptoms can cause fear - high HR and palpitations, chest pain, fainting or near-fainting, etc. Anxiety can exacerbate the symptoms. Are there techniques that can help a person control their anxiety, or is medication necessary?
A. Many natural techniques can help reduce anxiety.
Practice deep breathing techniques. Take a deep breath in through the nose (breath in as slowly as possible), then breathe out steady and slow through pursed lips (position your lips as though you are going to whistle). It is important to keep the inhale and exhale even (counting helps) to avoid over-breathing that can cause hyperventilation. It will feel awkward at first, but if you practice, it will be a coping mechanism that is ready for you when you need it most. This technique also has the added benefit of being the most effective way to re-oxygenate your body and prevent hyperventilation.
Add a time-out to your day - listen to music, meditate, practice visualization, yoga if you can, do light exercise (whatever you can even from a sitting position).
Develop preventative habits. Eating well-balanced nutritious meals, avoid excessive sugars, and caffeine as both can add to anxious feelings. Give yourself a consistent sleep pattern by forming rituals that help you relax before going to bed.
Find reasons to laugh. Rent a comedy, stream stand-up shows by comedians, hang out with a friend who likes to laugh
Practice acceptance - you can’t control your illness, but you can control how you respond to it. Accepting what you can and can not do and finding ways to feel fulfilled and useful will add to your overall sense of well-being and reduce the feelings of anxiety.
Get involved. Reach out to your support network when you are feeling anxious about your illness. Explore what you are feeling and see if you can recognize a pattern or see triggers. Volunteer with the network if possible or find other volunteer opportunities. There are many opportunities to volunteer online. Getting out of your head is one of the best ways to deal with feelings of anxiety.
Medication is not usually necessary, and for people living with chronic illness, drugs for anxiety may be counterproductive or may not be possible. Consult with your physician if you feel you need medication.
Resource: Anxiety and Depression Association of America https://adaa.org/tips
Q: Dysautonomia, particularly POTS, is caused by a dysfunction in the autonomic nervous system, specifically the Sympathetic Nervous System (SNS) responsible for the fight or flight response in the body (the release of norepinephrine). What effect, if any, does this have on a person’s anxiety level or in causing depression?
A. Norepinephrine is a neurotransmitter associated with the fight or flight mechanism in the body and feelings of anxiety in the nervous system. Because dysautonomia creates an instability in the SNS, a person’s fight or flight response activates without an external need for that response. This instability is at the core of POTS symptoms. Physicians not familiar with the disorder can frequently assume the symptoms described are a result of the person’s inability to appropriately deal with anxiety or are living with depression.
Resource: Science Direct Journal Neuron Volume 87
"Norepinephrine and Corticotropin-Releasing Hormone: Partners in the Neural Circuits that Underpin Stress and Anxiety" by Yaijie Sun, Sarah Hunt, Panka Sah https://www.sciencedirect.com/science/article/pii/S0896627315006443
Q: Chronic illness requires the person to adapt to a new sense of self and to establish a new “normal” for their life. For example, a person's professional identity may need to change if they can no longer work. A person's role within their family as the "go-to" person may also need to change if they now need help from their family instead. How can family and friends help support the chronically ill person making this transition?
A. Loved ones of a chronically ill person should encourage or help (if wanted) the person find a support group or mental health professional that they can talk to without feelings of guilt or the need to censor their feelings or thoughts. Recognize that there are many parts of your loved one's adjustment that you will not be able to help directly. Be available and open to what they need and avoid telling them what they “should” feel. In a patient survey that DINET offered a few years back, members expressed what helped or hurt them in their encounters with family and friends. The number one thing cited as “help” was listening. The second helpful thing was believing the person about how they feel. Example, on Tuesday, your family member says they are too sick to get out of bed. But then later that week, they say they feel well enough to go out. Avoid questioning or discouraging their decision.
Dysautonomia is a disorder with a wide range of symptoms that can change quickly. Believing what they say and letting the person take the lead is empowering and validating.
The top two things that “hurt” patient encounters with family and friends were dismissive language such as “you don't look sick” or “think positive” and being told what they feel or should feel, such as “don’t think that way” or “you should feel grateful that things aren’t worse.”
Take the lead from the person. If they miss what they used to do for work, help them explore ways to use those skills in another capacity. Even the most physically active careers have skills that can translate to other uses without the physicality of the former activity. For example, a person who used to play sports may find a place for themselves in Special Olympics or through coaching. A business person may be able to volunteer with an organization in need of their business skills but without the demands of full time work.
In some cases, people find it too depressing to be on the fringes of what they used to do. In that case, help your loved one explore new hobbies or disciplines. Perhaps they aren’t up to attending classes, but you may suggest or help them find an online course they would find interesting. There may be new hobbies that you can do with the person. Whatever the action, be sure it is something the person wants to do or is receptive to your help. There is nothing wrong with making a suggestion but give the person the respect of truly hearing what they need from you.
People make the transition in their own time. There is no right or wrong way to go through it. You may have feelings of frustration that your loved one isn’t moving forward quickly enough or won’t let you cheer them up in the ways you think they should happily accept. Try and remember that this is their journey, and they have to find their way to their new “normal.” If you are a caretaker or close family member, and you are having feelings of sadness or anxiety about your loved one’s illness, seek out outlets for yourself. The chronically ill person can’t absorb your feelings about this transition. You can be honest while still being careful not to give responsibility to the sick person to make you feel better.
Resource: Help Guide: "Living well with a disability https://www.helpguide.org/articles/healthy-living/living-well-with-a-disability.htm
* Coming up: Part Two of Mental Health and Chronic Illness.
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By Susanne Rimm
Many symptoms experienced by people living with POTS and other dysautonomias are related to a miscommunication within the Autonomic Nervous System) (ANS) between the sympathetic and the parasympathetic systems. The ANS controls the pressure inside our blood vessels. This complicated process involves many aspects of anatomy ( heart and vasculature ), hormones, chemicals, and nerves.
Efficient circulation is necessary to provide the body systems with adequate oxygen and nutrition to perform their specific tasks. Some organs or parts of the body need a constant rate of circulation ( i.e., the brain ), and some need more at certain times ( i.e., the digestive tract ). Maintaining and directing blood flow within the body is dependent on the harmonious communication between the sympathetic and parasympathetic nervous systems. These systems influence blood flow by widening the blood vessels (vasodilation) or tightening the vessels (vasoconstriction). Vasodilation lowers the pressure within the vessels vs. vasoconstriction which raises the pressure. These systems respond differently to various triggers within the body ( endogenous ) and from outside of the body ( exogenous ).
What are blood vessels?
Blood vessels are precisely that – vessels that transport blood. Anatomically they are tubular structures with an inner lining (endothelium), which is surrounded by connective tissue. They contain smooth muscle cells that respond to small nerve fibers directly connected to the ANS. Arteries carry oxygenated blood from the heart through the body, and veins return the blood through the lungs to the heart.
Widening of blood vessels increases blood flow, lowers blood pressure, and occurs when the smooth muscle cells within the walls relax. This can occur in response to endogenous factors such as hormones, chemicals, or nerve impulses. An increase in carbon dioxide, potassium ions, hydrogen ions, and adenosine can all lead to vasodilation. Another cause is the release of the hormone epinephrine caused by the activation of beta-2 receptors in the muscles of the blood vessels. Other causes for vasodilation are the release of nitrous oxide ( a substance released by certain nerves during inflammation ) as well as the release of histamine. (1,2)
Exogenous factors that can cause vasodilation are heat, certain foods, or beverages (e.g. alcohol ) or light and noises from the environment. Pharmaceuticals can also cause vasodilation and can be used to treat certain conditions like angina or hypertension. Vasodilation can lead to hot, flushed skin by drawing circulation to the periphery and away from the core. Other symptoms include sweating, tachycardia, nausea, dizziness, vision and hearing changes, headaches, and more. (1,2)
Narrowing of blood vessels occurs when the smooth muscles of the vessel walls constrict, resulting in restricted blood flow. This increase in vascular resistance raises systemic blood pressure and causes a localized reduction in blood flow. The general mechanism leading to vasoconstriction is an increased concentration of calcium ions in the smooth muscle cells. This concentration occurs because of an increase in epinephrine or activation of the sympathetic nervous system through the release of norepinephrine. These neurotransmitters interact with adrenergic receptors.
Again, vasoconstriction can be caused by endogenous and exogenous factors. Examples for exogenous causes are exposure to severe cold temperatures, which cause the cutaneous vessels to constrict in an attempt to reroute the circulating blood to the core. This constriction is what causes the skin to become pale in cold weather as less blood reaches the surface. Medications such as antihistamines, amphetamines, or stimulants are vasoconstrictors. They can be used to increase blood pressure or reduce local blood flow.
The most crucial endogenous trigger for vasoconstriction is the normal attempt of the ANS to correct the sudden drop in blood pressure that occurs when standing to avert orthostatic hypotension. A critical trigger for vasoconstriction is the narrowing of blood vessels necessary during sudden hemorrhage. As the body attempts to stop or limit blood loss, circulation is diverted towards vital organs and away from the source of the bleeding. The more common symptoms of vasoconstriction are tremors, cold extremities, paleness or bluish color skin, tachycardia, palpitations, chest pains, and more. (3)
Autonomic Tone is the balance of sympathetic and parasympathetic activity. It is regulated by the hypothalamus ( the primary control of the ANS ). In healthy individuals, the autonomic tone is responsible for restoring homeostasis, the body's ability to maintain a constant internal environment. In dysautonomia, this balance is disturbed, and one or the other branch dominates, causing an overcompensation or withdrawal of the sympathetic or parasympathetic activity. (4)
How it all ties together
Since there are so many different ways that the pressure within the blood vessels can be influenced, it is no surprise that a malfunction can have similar symptoms yet completely different causes. Here are a few examples of why different types of dysautonomia cause inappropriate vasodilation or vasoconstriction. (5,6)
This type of dysautonomia is thought to be caused by the partial denervation of the sympathetic nerves of the lower limbs. Since the smooth muscle cells of the vascular walls respond to the peripheral small fiber nerve endings of the ANS, this denervation may lead to inadequate vasoconstriction. Therefore, the ANS is unable to prevent or correct blood pooling, leading to a drop in BP and a compensatory increase in heart rate (HR) in response to orthostasis. A study by Jacob et al. showed that patients with neuropathic POTS had normal neuronal norepinephrine release in their arms but less norepinephrine in their lower body. Meaning there was less sympathetic activation in the blood vessels of the legs. A secondary form of neuropathic dysautonomia occurs in diabetic or alcoholic neuropathy resulting in ineffective blood pressure control via the vascular innervation. (5,6)
Ehlers-Danlos-Syndrome (EDS), or joint hypermobility syndrome, can cause abnormal connective tissue in the vessel walls, which permits the veins to dilate excessively. This dilation causes blood pooling in the lower limbs and a decrease in blood pressure. Some of these patients also display alpha and beta-adrenergic hyper-responsiveness. (6)
MCAD results in the release of the vasodilator histamine and other mast cell mediators, which may contribute to POTS. The excessive vasodilation caused by this reaction can cause many symptoms consistent with POTS and marked by flushing episodes. (6)
Hyperadrenergic POTS (HyperPOTS)
This type of dysautonomia is characterized by an increased level of the neurotransmitter norepinephrine, a vasoconstrictor. This can occur in several ways:
Sympathetic overcompensation - Norepinephrine gets dumped in response to a drop in BP, possibly due to vasodilation
Parasympathetic withdrawal - the ANS fails to counteract sympathetic activation or the inability to clear excess NE out of the synaptic cleft.
Hyperadrenergic POTS is often accompanied by high BP, high HR, and symptoms of sudden and extreme vasoconstriction brought on by the "fight-or-flight" response. This can lead to a loss of circulation to the brain and result in syncope or seizures in severe cases. (7)
The conscious state depends on adequate cerebral blood flow, which is auto-regulated by the ANS. Normal orthostasis is maintained by a brief increase in the sympathetic tone and maintenance of BP. In neurocardiogenic syncope, there is an inappropriate decrease in sympathetic tone followed by a compensatory increase in parasympathetic tone. This fluctuation leads to a sudden drop in both BP and HR due to vasodilation, often accompanied by a sudden loss of consciousness due to a temporary loss of cerebral circulation. The causes include orthostasis, hot temperature, and emotional triggers.
This article is a brief overview of the intricate and complicated correlation between the ANS and our cardio-vascular system. Hopefully, this aids in a better understanding of why we experience the symptoms typically experienced with POTS and other forms of dysautonomia. (8)
Claire is a thirty-six-year-old mom, with a refreshingly real disposition, shares her experiences of living with POTS. Her dysautonomia is accompanied by gastroparesis, irritable bowel syndrome, hypermobile Ehler-Danlos Syndrome, polycystic ovary syndrome (PCOS), and endometriosis among others. She worked as a Certified Dental Assistant for ten years before she resigned due to disability.
Though Claire recalls she has probably had POTS and EDS for many years, she officially sought a diagnosis at age twenty-eight. At the time, she was working full-time, married, and preparing to start a family. While she has always had some symptoms, she attributed them to her PCOS and endometriosis. However, she knew there must be another explanation when her symptoms flared during her pregnancy in 2010. Most of that year was filled with debilitating pain, migraines, unrelenting morning sickness, and both erratic heart rate and blood pressure. While she felt something was not right, her doctors dismissed it as typical pregnancy symptoms. She had a difficult nineteen-hour labor followed by several months of caring for a newborn, and trying to care for herself at the same time. By this time, she knew her symptoms were not typical of a pregnancy, nor postpartum depression, and actively began to seek answers.
In the next four and a half years, she persevered in her search for answers, and saw nearly fifty doctors. Most of these doctors told her she was “too young” or “too pretty” to be so sick. Some argued her symptoms were due to the stress of being a new mom. Even some friends and professionals questioned her experiences, believing she was not sick enough to seek help. She inevitably felt some sense of defeat after years of dismissal, and it was then that a twist of fate led Claire to a diagnosis – a close friend spoke with a doctor about her case, and the doctor wanted to see Claire. During their visit he spoke the magic words that we all long to hear: “I believe you, you are not crazy, and you are not alone.” Her search continued for several more months during which time she also discovered her home was filled with black mold. She was finally diagnosed with POTS and EDS at age thirty-two.
After such a long and harrowing experience, Claire was elated to have a diagnosis. She felt she could finally start treating her conditions, as well as receive belated empathy and validation from professionals. While her diagnosis was empowering and encouraging, Claire astutely articulates that we must remember that a diagnosis does not always change the way people with dysautonomia are treated. Even with a diagnosis, there is not clear and simple treatment for dysautonomia. Additionally, many professionals still do not understand the condition and, at best, provide little support while, at worst, continue to minimize and mock the experiences of patients. This roller coaster is familiar to many of us – we desperately seek answers for a smattering of life-altering symptoms. Then we finally receive them, and we are consumed by relief. However, then we must endure an entirely different emotional process of realizing the complexities of dysautonomia treatment and the lack of understanding about the condition in our society.
Today, Claire still struggles with her dysautonomia. She is in her mid-thirties and uses a shower chair, walker, cane, accessible parking, and occasionally a wheelchair. She used to love camping, but has had difficulty getting outdoors even with adaptations. One of the most thought-provoking points that Claire makes is that there seems to be a stereotypical success story of individuals who are expected to “overcome” illness by running marathons and raising a lot of money. According to that stereotype, many of us feel 'less than' if we don’t reach these often unrealistic heights. I would argue that this stereotype needs to be challenged. On the contrary, Claire is a refreshing and real inspiration through her courage to share her story even when she does not feel she has fully figured out her path. She reminds us that it is human to experience hope, despair, frustration, and inspiration all at once. She shows us that it is okay to be complex because if we all fit the cliché inspiration success stories we would lose the diverse, empathetic, creative, and intuitive individuals who make up our community. I believe that many of these qualities are cultivated among individuals living with dysautonomia specifically because of the unpredictable struggles of their daily lives. Claire teaches us that being real about where we are each day is, perhaps, the most inspiring story of all.
*If you would like your story to be considered for a future "Meet the Member" article, please reach out to Chelsea at firstname.lastname@example.org
Our Newsletter has been published quarterly by DINET since 2003. Each edition includes information touching on many aspects of living life with dysautonomia. This publication is a labor of love from volunteers that include writers, our medical advisory panel and design and editing staff, all volunteering their time and talents to make each edition informative and worthwhile. New editions are published in article/web format. Older editions are in pdf format and require a download. If you have difficulty downloading, or need a copy of an edition not in the list, please contact email@example.com
February 2020 Newsletter - Paths & Stories
June 2019 Newsletter - Managing & Enjoying Life
February 2019 Newsletter - Thriving
August 2018 Newsletter - Investigation & knowledge
April 2018 Newsletter - Communication
December 2017 Newsletter - Looking back & looking forward
July 2017 Newsletter - Challenges
March 2017 Newsletter - Empowerment
Featured below are ongoing studies related to dysautonomia and POTS related illnesses. DINET does its best to select the studies that we believe will best appeal to our community. If you know of a study that you would like to share with DINET, please email the information with the link to the study, to firstname.lastname@example.org All information will be reviewed.
Open Recruitment Studies (updated February 2020)
***RECRUITING: DINET has partnered with PCORI (Patient-Centered Outcomes Research Institute), the University of Pittsburgh and the University of Pittsburgh Medical Center in the Story Booth project. Research is crucial to helping us further our understanding of POTS and other forms of dysautonomia and to identify ways to prevent or cure these conditions. That's why DINET is parterning with the Patient-Centered Outcomes Research Institute (PCORI) and the University of Pittsburgh on their MyPaTH Story Booth Project. The project aims to help researchers better understand the dysautonomia patient experience with illness, coping and the healthcare system. Researchers hope their findings will lead to the development of reserach studies that address topics that are important to patients and caregivers. Please visit our Story Booth page for more detailed information or to get started sharing your story. https://www.dinet.org/member-stories/story-booth-project-sharing-the-stories-of-patients-and-caregivers-r209/
RECRUITING: John Hopkins University is examining the use of a temporary celiac ganglion block before celiac ganglion resection in dysautonomia-related bowel dysmotility. Autonomic celiac ganglia resection has been shown to alleviate symptoms of dysautonomia-related dysmotility by interrupting the sympathetic stimulation to the GI system. Currently there has been no test to confirm this diagnosis pre-surgery. This studies objection is to temporarily block the ganglion to see if the symptoms go away, therefore confirming diagnosis and allowing for the more permanent surgical action. This study is recruiting patients with chronic dysmotility who are considering Celiac Ganglion resection. For further criteria and information about this study, please visit:
Contacts: Christos Georgiades, MD PHD email@example.com
Beatriz Kohler, firstname.lastname@example.org
RECRUITING: Clarkson University is conducting a study to learn about what factors affect the function and quality of life for people living with various chronic illnesses. The study should take approximately 45 minutes to complete and asks similar questions to those asked by healthcare providers. Questions will include quality of sleep, fatigue, pain, etc. All answers will be anonymous. Participants must be 18 years or older. Each person participating is requested to ask one healthy person to also participate but this is not required. To learn more about this study or to begin the questionnaire, visit https://www.surveymonkey.com/r/V3P7S9J This is an IRB exempt study.
RECRUITING: A study is being conducted to examine the affect on parents who are raising a child with POTS. This study is being conducted through the University of Mississippi, under the Principle, Abigail Armstrong for her Master's thesis. Abigail is a POTS patient and DINET member.
This is an IRB approved study.
You must be a parent or primary caregiver of a child 8 - 17 years of age who has been diagnosed with POTS
The child's diagnosis must have been made 6 months ago or longer
You must be able to speak and read English proficiently
This study will be conducting completely online and will take 10 - 15 minutes to complete. The purpose of this study is to better understand the impact POTS has on the parents and the child. The study will ask you to complete a series of brief questionnaires about yourself, your experiences as a parent, and your child diagnosed with POTS.
Please email Abigail with any questions about the study at email@example.com
To begin the study, click on the link provided: https://usmep.co1.qualtrics.com/jfe/form/SV_cIpzp1ncQXLJdad
RECRUITING: Vanderbilt University is conducting a study entitled "Pathophysiology and therapy of Orthostatic Intolerance." Studies to identify the role of the brain, the autonomic nervous system and the vasculature in Orthostatic Intolerance. Blood pressure and HR are measured laying down and standing at regular intervals. Blood samples are drawn for hormones affecting blood pressure control. Some studies require tilt table testing and medication administration.
Eligibility: All participants are adults. Contact Information:
Principles: David Robertson, M.D., Bonnie Black, RN
Vanderbilt University, Clinical Research Center
RECRUITING: Clinical Trial: Dr. Satish Raj and the University of Calgary is recruiting POTS patients for a study of the effects of compression on the symptoms of POTS https://clinicaltrials.gov/ct2/show/NCT03484273?term=POTS%2C+Postural+Orthostatic+Tachycardia+Syndrome&cond=dysautonomia&rank=5
OPEN ENROLLMENT: The Mayo Clinic is conducting a study to better understand the causes of autonomic disorders. The study includes the collection of DNA for future studies. This study is being conducted in part by Dr. Philip Low, one of the leading researchers in this field.
Eligibility: Males and females (non-pregnant) age 13 years and older diagnosed with MSA, PAF, Autonomic Neuropathies, NOH, POTS, Dysautonomia and healthy participants. Orthostatic range must include a systolic BP deduction of > 30 mm Hg or mean BP reduction of > 20 mm Hg that occurs within 3 mins of head-up tilt. For help understanding the criteria or for more information about the study, please contact Tonette Gehrking CAP at firstname.lastname@example.org or call 507-284-4462
Participating Clinics: Rochester, Minnesota.
RECRUITING: University of CA is studying the effect of the drug Ivabradine, a drug currently used for heart failure, to examine the possibility of its use as a treatment for heart rate issues in POTS patients.
University of CA, San Diego
Investigator: Pam Taub, MD
For more information: https://clinicaltrials.gov/ct2/show/NCT03182725?cond=Postural+Orthostatic+Tachycardia+Syndrome&rank=3
RECRUITING: Parent-Child Dyadic Experiences Living with Postural Orthostatic Tachycardia Syndrome (POTS) during Emerging Adulthood. What is it like living with POTS during emerging adulthood? Virginia Tech wants to know. If you are interested in joining a family science study on POTS, please contact Masumeh@vt.edu. Participants must be 18-29 years old, diagnosed with POTS. This is a parent-child study, so both must be willing to participate. Participants must reside in the U.S. Virginia Polytechnic Institute and State University
Department of Human Development
Principal Investigator: Dr. Carolyn Shivers
Co-Investigator: Masumeh Farchtchi
Specifics about the study and a consent form will be emailed to you if you are interested.
ACTIVE NOT RECRUITING: Clinical Trial: University of Oklahoma and Vanderbilt University are recruiting POTS patients for the Autoimmune Basis for Postural Tachycardia Syndrome study. They are looking at the levels of immune proteins (autoantibodies) in POTS patients and their relation to symptoms over time. https://clinicaltrials.gov/ct2/show/NCT02725060?term=autonomic+dysfunction&cond=Postural+Orthostatic+Tachycardia+Syndrome&rank=5
Recruiting: Vitamin D Deficiency in Dysautonomia In previous work the investigator identified a group of children between the ages of 10-18 years whose diagnostic workup for chronic nausea unexplained by conventional diagnostic tests has unexpectedly revealed underlying cardiovascular instability manifesting as orthostatic intolerance, primarily defined as postural orthostatic tachycardia syndrome (POTS) (88%). While this is an atypical initial presentation for orthostatic intolerance in general, the investigator believes that the cardiovascular problem is serious and represents a cause of nausea in a majority of these individuals, as treatment of the POTS with fludrocortisone reduced the symptoms of nausea. While fludrocortisone treatment abrogates the fall in baroreflex sensitivity (BRS) during tilt in part, it did not completely correct the tachycardia symptoms or the BRS suppression during HUT. Furthermore, it caused an elevation in MAP in a supine position, which may lead to future cardiovascular problems such as early onset hypertension and cardiac hypertrophy. This argues for a different treatment approach. The investigator presents preliminary data in this application revealing that OI subjects tend to have lower 25-hydroxyvitamin D (25(OH)D) compared to non OI subjects.
Open to children 10 - 18
Still Recruiting: ME/CFS Activity Patterns and Autonomic Dysfunction The purpose of this study is to identify daily activity patterns, negative life events and autonomic abnormalities that may be related to non-improvement in myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). For both naturalistic studies and behavioral intervention trials, roughly 50% of patients report worsening or unchanged illness. The proposed four-year study would be the first to look at the relationship between illness non-improvement, patient activities at home and autonomic function. Our long-range goal is to identify physiological signals and activity patterns that predict non-improvement and relapse and develop a self-management program that prescribes improvement-linked behaviors and discourages non-improvement activities.
Contact: Particia Bruckenthal, PhD, RN 631-444-1172 email@example.com
Contact: Jenna Adamowicz, MA 631-371-4417 firstname.lastname@example.org
Recruiting: The investigators propose to conduct a safety, tolerability and early proof of concept efficacy study of phosphatidylserine in patients with FD. The long-term goal is to find an effection nutritional therapy that will improve the quality of life for patients with FD and alter disease prognosis Contact & participation information is available - https://clinicaltrials.gov/ct2/show/NCT02276716?cond=Dysautonomia&rank=5
Completed, Results TBA PET Imaging Study of Neurochemical and Autonomic disorders in Multiple System Atrophy (MSA) from the University of Michigan. This study aims to better understand the patterns and timings of nerve degeneration relatively early on and how it affects symptoms and progression.
Eligibility: 30 - 80 years old
Gender: AllSubject: diagnosis of MSA or probable MSA of the Parkisonion subtype (MSA-P) or Cerebellar subtype (MSA-C), Subject must be willing and able to give informed consent. Normal cognition as assessed by MiniMental State Examination
Timeline: Less than 4 years from the time of documented diagnosis See study online for exclusions and further information. https://clinicaltrials.gov/ct2/show/study/NCT02035761?recrs=ab&cond=Dysautonomia+Orthostatic+Hypotension+Syndrome&rank=4 Contacts: Arijit K Bhaumik, B.A. CCRP, 734-936-8281 email@example.com Edna Rose, Ph.D., RN, MSW, BC, 734-936-7359, firstname.lastname@example.org Estimated Primary Completion Date: July 2018
Recruiting: A Study of Pyridostigmine in Postural Tachycardia Syndrome.
This is a 3-day study being conducted by the Mayo Clinic comparing pyridostigmine vs placebo in the treatment of POTS. Researchers expect pyridostigmine to improve tachycardia and stabilize blood pressure.
Eligibility: Diagnosis of Postural Tachycardia Syndrome using the following criteria:
Orthostatic heart rate increment greater than or equal to 30 beats per minute (BPM) within 5 minutes of head-up tilt.
Symptoms of orthostatic intolerance. These include weakness, lightheadedness blurred vision, nausea, palpitations, and difficulty with concentration and thinking.
Both criteria must be fulfilled
See study online for exclusions and further information http://www.mayo.edu/research/clinical-trials/cls-20128843 Mayo Clinic location, Rochester MINN, Principal Investigator: Dr. Philip Low Contact: Tonette Gehrking, CAP, 507-284-4462, email@example.com
(Completed, Results TBA) The Safety and Tolerability of Kinetin, in Patients with Familial Dysautonomia The overall objective of this study is to assess the safety and tolerability of administering kinetin in patients with FD. The specific aim of this proposal is to determine the safety of a once-daily dose of kinetin in patients with FD using a dose ascending titration and to determine the long-term safety and tolerability during 3-years of receiving a maximum tolerated steady state dose of kinetin. The investigators hope to also demonstrate early proof of concept that kinetin enhances the ability of the neuronal tissue to correctly splice IKAP mRNA.
Eligibility: Male or female 16 years of age and older, confirmed a diagnosis of familial dysautonomia by genetic testing, written informed consent to participate in the trial and understanding that they can withdraw consent at any time without affecting their future care. And the ability to comply with the requirements of the study procedures.
See study for more info and exclusions: https://clinicaltrials.gov/ct2/show/NCT02274051?cond=Dysautonomia&rank=3 New York University School of Medicine Estimated Completion date: September 2019
Contact: Horacio Kaufmann MD, 212-263-7225,firstname.lastname@example.org Contact: Jose Martinez, MA, 212-263-7225, email@example.com
Two new studies open for patients with MSA The NYU Dysautonomia Center has 2 new clinical trials to test new drugs that are being developed for the treatment of OH in patients with MSA. Both compounds work by enhancing the body’s levels of norepinephrine. Both studies are also being carried out at the Autonomic Dysfunction Center at Vanderbilt University. NYU Dysautonomia Center and Vanderbilt are longtime collaborative partners in rare autonomic disorders.
(CLOSED RECRUITMENT) Study 1: A Phase 2 Study to Assess the Effect and Safety of TD-9855 in Subjects with Neurogenic Orthostatic Hypotension
This Study is in collaboration with Theravance Biopharma R & D They are testing the acute efficacy and safety of a new compound (TD-9855) in improving blood pressure and reducing orthostatic symptoms in patients that have neurogenic orthostatic hypotension (nOH), which includes those with MSA. This study is also open to patients with Pure Autonomic Failure (PAF) and Parkinson’s Disease (PD) with NOH.
Eligibility: 40 years and older, All Genders
Diagnosed with symptomatic orthostatic hypotension due to Parkinson’s disease, MSA or PAF (ie.neurogenic orthostatic hypotension)
At screening, a subject must meet the diagnostic criteria of neurogenic orthostatic hypotension, as demonstrated by a > 30 mm Hg drop in systolic blood pressure (SBP) within 5 minutes of standing.
Impaired autonomic reflexes, as determined by the absence of BP overshoot during phase IV of the Valsalva maneuver, in subjects where Valsalva is performs as appropriate.
For the optional open-label extension study subjects must have demonstrated a pressor effect and completed dosing in Cohort 1.
Estimated completion date: June 2018 (Completed, results not yet published) See study for further info and exclusions: https://clinicaltrials.gov/ct2/show/NCT02705755 NYU Information sheet: https://dysautonomiacenter.com/2017/04/10/two-new-studies-open-for-patients-with-msa/
STILL RECRUITING: Study 2: Norepinephrine Transporter Blockade, “Autonomic Failure”
This study is supported by The Food and Drug Administration’s Office of Orphan Product development. , We are testing whether we can repurpose an available drug (atomoxetine) as a treatment for neurogenic orthostatic hypotension (nOH). This study is also open to patients with MSA, pure autonomic failure (PAF), and Parkinson disease (PD) with OH.
Eligibility: 40 to 80 years old, any gender, with Neurogenic Orthostatic Hypotension (defined by a reduction of > 30 mmHg drop in SBP within 3 minutes of standing, associated with impaired autonomic reflexes as assessed by autonomic function tests.
Estimated completion date: July 2020
Contact: Bonnie Black, RN, 615-*343-6862, firstname.lastname@example.org Contact: Jose Martinez, 212-263-7225, Jose.Martinez@nyumc.org
See study for further info and exclusions: https://clinicaltrials.gov/ct2/show/NCT02784535
NYU Information sheet: https://dysautonomiacenter.com/2017/04/10/two-new-studies-open-for-patients-with-msa/
Recruiting: Vagal Stimulation in POTS - The Autonomic Inflammatory Reflex (Pilot 3) The purpose of this study is to investigate how the electrical stimulation of a nerve in the skin of your earlobe (transcutaneous vagal nerve stimulation) affects the way your autonomic nervous system controls your heart rhythm.
Eligibility: Participants must be female between the ages of 18-45 diagnosed with POTS.
See Study for further information and exclusions: https://www.rarediseasesnetwork.org/cms/autonomic/6111 This study is a part of the Rare Disease Clinical Research Network and the Autonomic Disorders Consortium
Contact: Vanderbilt University Medical Center Nashville
Misty Hale CCRP, 615-322-2931, misty.hale@Vanderbilt.edu
Recruiting: The Big POTS Survey is still recruiting This study’s lead investigator is Dr. Satish Raj MD MSCI, Adjunct Professor of Medicine at Vanderbilt University’s Autonomic Dysfunction Center. Dr. Rah says that the information collected as part of this survey “will help us learn more about the possible underlying causes and risk factors for developing POTS, treatments, and the economic, educational and social impact of POTS on patients and their families.” Dr. Raj serves on the Medical Advisory Board for DINET and Dysautonomia International. DI is sponsoring this survey.