Description, Physiology & Onset
Hyperadrenergic POTS is a subtype of POTS that affects about 10% of patients with dysautonomia symptoms due to orthostatic intolerance. (Grubb et al, 2011) The mechanism differs from other types in so far as it is caused by centrally driven sympathetic activation. In other words, symptoms are caused directly from an ANS malfunction, rather than the ANS malfunction being a response to another cause. This results in patients having increased norepinephrine (adrenaline) in circulation and a rise in systolic blood pressure upon standing.
Two additional characterizations of hyperPOTS go hand-in-hand - hypovolemia and the reduction in the activity of the enzyme renin and aldosterone. When a person with hyperPOTS is upright, there is a loss of plasma blood volume into the surrounding tissue (hypovolemia). In people without hyperPOTS, there is a normal reduction in urinary sodium levels when upright. This mechanism doesn't happen effectively in a person with hyperPOTS and this contributes to the severity of the reduced blood volume upon standing. In addition, the plasma enzyme renin plays a major role in the regulation of blood pressure, thirst, and production of urine. When standing, the activity of renin and aldosterone is greatly reduced in a person with hyperPOTS.
The third major characteristic is the elevated norepinephrine and epinephrine levels in a person with hyperPOTS. Adrenaline is a neurotransmitter, a substance communicating within the nervous system and it is active in the synapse, the junction between nerves. When present, it causes activation of the sympathetic nervous system, causing an increase in HR and/or BP, commonly known as the “fight-or-flight response” which causes excitement, tremors, etc. The increase of norepinephrine can be activated by different mechanisms, the ANS produces too much adrenaline ( centrally mediated hyperadrenergic activation ), the excess adrenaline does not get cleaned out of the synapse once no longer needed ( Norepinephrine Transporter Deficiency ) or autoimmune antibodies against cholinesterase receptors. (Vanderbilt)
The onset of hyperadrenergic POTS is largely the same as other forms of POTS, with the onset of symptoms following precipitating events such as viral infection, pregnancy or trauma, including surgery. Another cause found in hyperadrenergic POTS is MCAS ( mast cell activation syndrome ). In this case, the circulating vasodilator produces reflex sympathetic activation which causes symptoms like flushing and orthostatic intolerance ( the inability to compensate for the upright posture ). (Vanderbilt)
The criteria for diagnosis shares many factors with POTS; including the presence of symptoms for 6 months or longer, a Head-Up Tilt Table Test (HUTT) shows tachycardia of 30 BPM or above 120 BPM in the presence of orthostatic intolerance within the first 10 minutes of upright posture. Patients are diagnosed with the hyperadrenergic form of POTS based on an increase in their SBP ( the higher number ) of at least 10 mmHg upon standing or during the HUTT with concomitant tachycardia or serum norepinephrine levels of above 600 pg/ml when upright. (Grubb, et al, 2011)
The symptoms of hyperadrenergic POTS are often shared with other types of POTS but also can be specific to this type of POTS: anxiety, tremors, orthostatic hypertension, and cold hands and feet being specific to this type. Other symptoms include fatigue, palpitations, dizziness and presyncope, syncope, excessive sweating, nausea/ diarrhea/ bloating, excessive stomach acid, increased urine output upon standing. Similar symptoms can be caused by pheochromocytoma ( a benign tumor on the adrenal gland ), so the presence of this must be ruled out before the diagnosis can be made. (Grubb et al, 2011)
In addition to increased fluid intake and compression garments, it can be helpful to increase salt intake, but caution is needed when hypertension is present. Twenty (20) minutes of mild aerobic exercise ( in fresh air when possible ) 3 times a week has been proven effective. A fine balance of activity and rest periods can prevent hyperadrenergic symptoms and can promote healthy sleep patterns.(Grubb et al, 2011)
There are many medications that have been effective in symptom improvement, however, there are no FDA approved drugs for the treatment of this type of POTS. The treatment is highly individual, which means some meds will help one patient but not the other. This can create a frustrating process of trial-and-error but often will lead to the discovery of the right combination.
Some of the medications that have been found helpful are:
Beta-blockers (especially the combination of Carvelidol and Labetaiol)
One of the more significant findings in the treatment of hyperPOTS is the general observation that centrally acting sympatholytics (Clonidine, Methyldopa & others) and beta-blockers seem to work better to manage symptoms in people with hyperPOTS than patients with neuropathic POTS. (Grubb, et.al)
Hyperadrenergic POTS is often chronic and can be progressive. Some patients are disabled and unable to work while others are able to function with limitations. It has been shown that treatment is challenging since often the symptoms change or increase over time and medications may need to be adjusted or changed.
It is important to note that most research into POTS and subgroups of POTS recognize the inherent problems associated with trying to narrow down the specific subtype. One of the most difficult problems is that the abnormalities seen in different subtypes are not mutually exclusive from other abnormalities. For example, a person with the norepinephrine levels indicative of hyperPOTS may also have QSART levels pointing to neuropathic POTS. Therefore, most physicians specializing in the treatment of POTS disorders focus on the specific abnormal findings instead of focusing on categorizing the subtype. (Vanderbilt) (Grubb)
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Blair P. Grubb, Khalil Kanjwal, Bilal Saeed, Beverly Karabin, Yousuf Kanjwal Clinical presentation and management of patients with hyperadrenergic postural orthostatic tachycardia syndrome. A single center experience, Cardiology Journal, 2011, Vol 18 No. 5 https://journals.viamedica.pl/cardiology_journal/article/view/21202/16806
Autonomic Dysfunction Center
Italo Biaggioni, David Robertson, Cyndya Shibao, Amanda Peltier, and additional faculty members, et al. Hyperadrenergic Subgroup and POTS subtype: Does it really matter?https://ww2.mc.vanderbilt.edu/adc/38938
DINET collects relevant research related to dysautonomia disorders and related conditions & illnesses. This is in no way meant to be a complete list of all research currently underway or the results of research currently made public. But it is a summary of key research studies that we hope are relevant and potentially important to our members' ongoing treatment and prognosis. Please check back as this page is regularly updated.
New: Study released by Dr. Julian Stewart and colleagues discusses Postural Hyperventilation and the resulting variant POTS disorder. Pub Journal of the American Heart Association. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6064900/
New: An examination of the experimental and clinical trials conducted in the treatment and diagnosis of MSA. Pub Science Direct, Autonomic Neuroscience Vol 211 https://www.sciencedirect.com/science/article/pii/S1566070217301522
New: The Journal of Parkinson's Disease reviewed the impact of different exercise modes on people suffering from Parkinson's Disease. Pub IOS Press, Feb 2019 https://content.iospress.com/articles/journal-of-parkinsons-disease/jpd181484
Updated Info: Dr. Raj and associate, Dr. Miller's updated article about the pharmacotherapy for POTS. Pub. May 2018 in Science Direct, Autonomic Neuroscience. https://www.sciencedirect.com/science/article/pii/S1566070218300250
NEW: An examination of whether the impairment of the Corticol Autonomic Network (CAN) of the brain is involved int the psysiology of Neurogenic Orthostatic Hypotension (NOH) Pub. Oct 2018 https://www.ncbi.nlm.nih.gov/pubmed/30332348
NEW RESULTS: The need for specific diagnosis and treatment for patients labeled with CFS and Fibromyalgia. Important research results from one of DINET's Medical Advisors, Dr. Svetlana Blitshteyn and her colleague, Pradeep Chopra, Pub date Oct 2018 Read full article: 2018CFS_Fibromyalgia_ChronicPain_PubKarger.pdf
Updated info from the CDC on HPV Vaccine safety for POTS patients https://www.cdc.gov/vaccinesafety/vaccines/hpv/hpv-safety-faqs.html Follow up to: HPV Vaccine and POTS - https://www.ncbi.nlm.nih.gov/pubmed/28689455
Complex Regional Pain Syndrome, Type 1 - Diagnosis and management. https://www.ncbi.nlm.nih.gov/pubmed/29409405?_ga=2.71311891.1204982470.1531704876-989465291.1526426607
Study results: A presentation made by the American Association for Cancer Research at their annual meeting reported findings related to health risks in post-menopausal women using medication for high blood pressure. The report claims an increased risk of pancreatic cancer in post-menopausal women using short-acting calcium channel blocking medications for their blood pressure treatment. Read the information from the AACR.
Study results: NE Journal of Medicine releasing study results for episodic migraine treatment using Erenunab. The publication is reporting a 50% or greater reduction in the average number of migraine days per month. Read more about this study
Study results: Physical maneuvers are viewed as important and promising strategies for reducing recurring episodes of syncope in vasovagal syncope patients. Read the result of the study published Jan. 2018
Study results: University of Alberta & McGill University release their findings for a new therapeutic agent that may hold the potential for the prevention of MS
Article synopsis: https://bit.ly/2GhF5FK
Research Article: https://insight.jci.org/articles/view/98410
Study results: Could gut bacteria be the key to preventing autoimmune disorders? Researchers from Yale, New Haven, CT are reporting that discovery that bacteria in the small intestine can travel to other organs and induce an autoimmune response. They also are reporting that antibiotic treatment or vaccine may be the key to combating this reaction. The study results were originally published in the journal Science. This article from Medical News Today gives a synopsis of the findings and links to the full study. https://www.medicalnewstoday.com/articles/321157.php
Genomics and Health Impact Blog - a discussion about gene testing from the CDC
There have been enormous contributions towards disease prevention in women's health, in particular, the development of consumer testing for the BRCA gene. The benefits cannot be applauded enough. However, health protection & research organizations like the CDC, are increasingly warning consumers about the need for professional counsel when interpreting results. This article, humorously entitled "Think before you spit" tempers the amazing breakthroughs with the cautionary information we need to have to be able to put a perspective on the results testing may give us. https://blogs.cdc.gov/genomics/2017/04/18/direct-to-consumer-2/
1st lab test to detect concussion approved by FDA
For any person who has fainted or fallen and hit their head, the blood test approved recently by the FDA could be a lifesaver. The test detects concussion by looking for specific proteins released into the blood within 12 hours after the head trauma. Known as the Banyan Brain Trauma Indicator it can reduce the need for CT Scans and radiation and will produce results within 3 - 4 hours following injury. Read more about this important breakthrough
Neuroprotective diet having good results for people living with MSA https://www.multiplesystematrophy.org/about-msa/neuroprotective-diet
POTS & Ehlers-Danlos Syndrome
"Postural tachycardia in hypermobile Ehlers-Danlos syndrome: A distinct subtype?" by Miglis MG, Schultz B, and Muppidi S, from the Departments of Neurology and Psychiatry at Stanford University Medical Center.
"It is not clear if patients with postural tachycardia syndrome (POTS) and Ehlers-Danlos syndrome (hEDS) differ from patients with POTS due to other etiologies. We compared the results of autonomic testing and healthcare utilization in POTS patients with and without hEDS."
There is very little literature about the effects of POTS on pregnancy. There is far more studies related to the effects of pregnancy on POTS patients and their symptoms. However, to put anxious minds at ease, there has been no significant change in maternal or fetal related complications and 60% of patients remained stable or improved during pregnancy (Blitshteyn et al., 2012).
According to current research 2/3 of women experience improvement of symptoms in the second and third trimester and 1/3 of women develop worsening symptoms. There does not appear to be a difference between first-time pregnancies versus multiple pregnancies and there is no evidence of adverse events in pregnant women with POTS. POTS does not pose an increased risk for pregnancy or birth. (Kanjwal et al.,2009)
Physiological changes in pregnancy
In the first trimester, there is a 50% increase in blood volume to supply the vascular system of the uterus, an increase in cardiac output and peripheral vasodilation. There is a decreased sensitivity to vasoconstrictors such as angiotensin and norepinephrine and an increased production of vasodilators like nitric oxide and prostacyclin. (Goodman et al., 1982; Gant et al., 1980) This can increase the acute symptoms of POTS in the first trimester, such as tachycardia, lightheadedness, fatigue and even syncope as well as other symptoms of POTS. Still, 60% of patients remained stable or reported improved symptoms during pregnancy (Blitshteyn et. al., 2012)
Treatment of POTS in pregnancy
The treatment of POTS in pregnancy is highly individualized and based on symptom relief. It is recommended that general guidelines for POTS treatment be used during pregnancy. (Sheldon et al., 2015)
Usual first-line treatments are:
Exercise - 25 to 30 minutes of mild exercises per week, avoiding upright posture (swimming or recumbent bike are recommended and better tolerated) and exercises performed lying on the left side to minimize compression of the vena cava.
Oral hydration of 2 l of water daily as well as increased salt intake of 3 - 5 gm sodium per day except if hypertension is present or pregnancy is high risk for hypertension.
Compression garments can be helpful and are covered by most insurance plans with a prescription. There are also compression stockings specifically designed for use during pregnancy.
Medications during pregnancy
Whenever possible patients have weaned off medications during pregnancy. For patients with debilitating POTS symptoms, particularly patients with recurring syncope, medications can be safely prescribed. (Ruzieh, Grubb, December 2018)
Some of the more commonly prescribed are ( not limited to 😞
Midodrine - trialed in pregnant patients with POTS with no adverse maternal or fetal outcome (Kanjwal et al., 2009: Glatter et al., 2005)
Beta Blockers - such as Propanolol were found to be effective on lessening symptoms without adverse reactions (Raj et al., 2009)
Fludrocortisone - used by Kanjwal et al., 2009 in a pregnant patient with POTS with no significant adverse effects.
In patients who are not fully helped with the above solutions, Duloxetine and Venlafaxine can be added with particular benefit to patients who suffer from symptoms of fatigue and anxiety.
Pyridostigmine may improve tachycardia in POTS patients (Raj et al., 2005; Kanjwal et al., 2011). However, Pyridostigmine also increases bowel motility. Therefore, although it does not have adverse reactions specific to pregnancy, it is not tolerated in many patients due to multiple GI side effects (Kanjwal et al., 2011)
IV fluids - infusing 1 L of normal saline over 1-2 hours weekly may be helpful in refractory cases. It can then be increased or decreased on an individual basis as needed. If IV Fluids are used, it is recommended that it be done on an outpatient basis and to minimize the risk of infections and thrombosis, the use of central lines and infusion ports should be avoided. (Ruzieh, Grubb, December 2018)
Bedrest - partial bedrest may be recommended in patients with recurring syncope or falls.
There are no special considerations for vaginal delivery vs C-section. Both can be carried out successfully without complications. The choice for what is used should be made solely based on obstetrics. (Glatter et al., 2005; Powless et al., 2010; Blitshteyn et al., 2012; Lide, Haeri 2015) No evidence was found to favor one method or type of anesthesia used - regional vs general vs none. Also, an epidural injection was found to be safe and didn’t trigger POTS symptoms. The birth method or anesthesia used should not be influenced by a POTS diagnosis. It should be solely based on Obstetrician’s recommendations. (Corbett et al., 2006)
Some women experience worsening of symptoms and others find rapid improvement of symptoms after delivery but the majority of women remain stable. Breastfeeding is safe and encouraged, however, caution should be taken if medications are being used to treat POTS symptoms during pregnancy and potentially transfer to breast milk. (Bernal et al., 2016)
According to current research, there is no long term impact of pregnancy on POTS and POTS does not pose an increased risk for pregnancy or birth. HUTT testing is safe during pregnancy.
It is recommended that patients with debilitating POTS symptoms consult with a high-risk obstetrician, and any obstetrician treating a POTS patient should take the time to learn about POTS and dysautonomia in general as well as the medications used to treat it.
Special note for POTS patients with EDS: Pregnant women living with EDS and POTS are at a higher risk for maternal and fetal complications. Therefore these patients require more monitoring and closer follow up (Jones, Ng 208; Sorokin et al., 1994)
For help in gathering or printing materials for your obstetrician, please contact firstname.lastname@example.org
Ruzieh Mohammed, Grubb Blair P., Overview of the management of Postural Orthostatic Tachycardia Syndrome in pregnant patients, Autonomic Neuroscience, Vol 215, Full Text https://www.sciencedirect.com/science/article/pii/S1566070217303442
Dysautonomia is an umbrella term used to describe a collection of disorders related to the dysfunction of the Autonomic Nervous System (ANS). So to understand dysautonomia, it begins with understanding how the ANS works.
The autonomic nervous system regulates certain processes that occur automatically within the body, without a person's conscious effort to make them work. These processes are things like breathing, heart rate, and blood pressure.
When the autonomic nervous system doesn't work correctly (dysfunctions) it can affect any body part or process. These disorders may be reversible or they may progressively worsen over time.
The ANS is the part of the nervous system responsible for the function of our blood vessels, stomach, intestines, liver, kidneys, bladder, genitals, lungs, pupils, heart and sweat,, salivary and digestive glands. It is easy to see how debilitating disorders of the ANS can be since they affect so many parts of the body.
The autonomic nervous system has two main divisions:
Both of these divisions are affected when the ANS is not working properly.
To understand this better, it is important to understand how this works within the body.
When a signal is received by the ANS from the body and the environment, it responds by stimulating processes within the body, usually through the sympathetic portion of the ANS, or it inhibits a process through the parasympathetic division of the ANS. For example; the heat in a room gets turned up. This is an external message that is received by the ANS within the body. A properly functioning ANS would respond by sending a signal causing the body to sweat. This would be a natural reaction to received stimuli.
The processes the ANS are responsible for are:
Heart rate and respiration (breathing)
Metabolism (affects weight)
The balance of water & electrolytes (affecting sodium & calcium levels)
Production of body fluids (affecting saliva, sweat & tears)
One of the more challenging aspects to understand is that although many organs are controlled primarily by the sympathetic or parasympathetic divisions, sometimes the two divisions can have opposite effects on the same organ. For example, the sympathetic division increases blood pressure, and the parasympathetic division decreases it. Overall, the two divisions work together to ensure the body responds appropriately to different situations. (Low, Dec 2018)
The sympathetic division prepares the body for stress and emergency situations - Fight or Flight. The fight or flight mechanism increases the heart rate and blood pressure (the force that the heart contracts) it dilates (widens) the airways making breathing easier. It causes the body to release stored energy and muscular strength is increased. It also causes your palms to sweat, pupils to dilate and your hair to stand on end. It slows down the processes within the body that are less important in an emergency, like digestion and urination. (Low, Dec 2018)
On the other hand, the parasympathetic division controls the processes in the body during ordinary situations. Generally, it conserves and restores. It slows the heart rate and decreases blood pressure. It stimulates digestion causing the body to process food and eliminate waste. It uses energy from processed food to restore and build body tissue.
Two chemical messengers (neurotransmitters) are used to communicate within the autonomic nervous system:
Nerve fibers that secrete acetylcholine are called cholinergic fibers. Fibers that secrete norepinephrine are called adrenergic fibers. Generally, acetylcholine has parasympathetic (inhibiting) effects and norepinephrine has sympathetic (stimulating) effects. However, acetylcholine has some sympathetic effects. For example, it sometimes stimulates sweating or makes the hair stand on end. (Low, Dec 2018)
The role of cholinergic and adrenergic fibers continues to be studied in dysautonomia research and particularly the role they play in the ANS function of POTS patients.
Dysautonomia is the dysfunction of the autonomic nervous system. Because the ANS does not respond correctly to the messages it receives, it results in the misfiring of the processes described above. The causes of dysautonomia are not always known. It is important to understand that although there are patients who develop dysautonomia disorders as their primary illness, there are many more who develop dysautonomia disorders secondary to other illnesses that cause the dysfunction of the ANS, such as Parkinson's Disease, EDS, Spinal Cord trauma or Brain Injury, and many others.
Follow this link to read more about the mechanisms within the body associated with various types of dysautonomia disorders, Explore the Information Resources section of this site to learn more about specific disorders related to the dysfunction of the ANS.
Phillip Low, MD, Professor of Neurology, College of Medicine, Mayo Clinic; Consultant, Department of Neurology, Mayo Clinic updated December 2018 Merck Manual, Consumer Edition, full text
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
Isabelle is a young, creative entrepreneur but that wasn’t always her plan. She excelled at tennis as a preteen and hoped to pursue it as a full-time career. This seemed possible until an episode of heat stroke during a tennis match caused her heart rate to be sustained around 180 beats per minute for several days thereafter. She knew something was wrong having been a relatively healthy young woman prior to the episode. However, the doctors in the emergency room she visited told her that her symptoms were “all in her head,” and they dismissed her heart rate as a byproduct of being an anxious, preteen girl.
Unfortunately, many of us know this story all too well. Was Isabelle anxious? Maybe. Who wouldn’t be after such an intense health episode with no explanation? In my years of writing the Meet the Member column, I’ve talked with so many women who have conveyed that being dismissed as anxious, dramatic, and overly sensitive have been, by far, the most difficult parts of their dysautonomia journeys. I, too, can relate. Isabelle certainly experienced this dismissal throughout her diagnostic process that took about a year and a half. She visited gastroenterologists, cardiologists and made several trips to the emergency department, but received no explanation for her sustained symptoms. Yet, she knew something was wrong; she went from a top tennis player to not being able to walk a mile in gym class.
Though many of Isabelle’s physicians did not take her debilitating symptoms seriously, she ultimately found an insightful doctor who thought outside the box to give her answers. Her now primary care doctor found a connection between Isabelle’s experiences and his time in the Army when he had witnessed several servicemen never return to full health after severe heatstroke. She was ultimately diagnosed with dysautonomia which provided her the validation, and knowledge, to move forward.
Her diagnosis was bittersweet; it gave her answers, but it also made a full-time tennis career feel unattainable. Her mom bought her a camera in an effort to help Isabelle forge a new path, and she immediately fell in love with photography. Just a few years ago, she was selected to participate in a National Geographic Student Expedition in Yellowstone National Park. This was a life-changing experience; she solidified her love for photography, she was able to learn from some of the best professionals in the field, and she made lasting friendships. Now, at age twenty-one, she is a freelance photographer for the Arkansas Democrat Gazette and works for a couple of music publications doing concert photography. She is also currently applying to internships and hoping to go to college in the near future. (*editor's note: Photo at right an example of Isabelle's work)
Isabelle’s strength, ambition, and maturity were evident during this interview. She has clearly made the best of her life with dysautonomia and credits it with helping her discover her passion for photography. However, her difficult diagnostic experiences—particularly being dismissed as an anxious, preteen girl—have certainly contributed to her identity as a young woman. She discussed some of the issues with the ingrained gender discrimination in some of the healthcare systems. Many women with dysautonomia, like Isabelle, wonder if their initial symptoms would have been dismissed so readily if they were men. She also talked about the insight of her primary care physician who was able to draw a connection between the symptoms of a young, female athlete and full-grown, military servicemen.
The dichotomy between supportive and unsupportive physicians is an important issue in our dysautonomia community. It makes us realize that we need to recognize, more often, the doctors, like Isabelle’s primary care doctor, who have been tremendous allies to all of us. Without their insight, research, and help with advocacy many of us would remain undiagnosed in a healthcare system where some professionals are trained that there is a straightforward answer for our complex, and sometimes elusive, collections of symptoms. We commend and thank these medical professionals, and we will continue to do our part in sharing accurate information about dysautonomia. Hopefully, these continued partnerships between the advocacy and medical communities will minimize diagnoses of female hysteria so that ambitious women like Isabelle can move forward to make their mark in the world.
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Editor's Note: Melissa Milton is a DINET volunteer who lends her hand to help wherever it is needed, most often on the forum. Melissa has also written articles for this newsletter, sharing her artistic view of the world with all of us. Melissa came to DINET as part of our Featured Members program and more of her art and her story can be seen on her featured page.
Melissa Milton feels honored to be among the 2018 Rare Artist Contest Awardees. The annual Rare Artist Contest is sponsored by the EveryLife Foundation. The purpose of the contest is to raise awareness for rare diseases, as they are often overlooked when it comes to medical research, medical provider knowledge, etc. Melissa’s rare disease is Dysautonomia.
Each awardee will have a chance to share their story and display their art piece at the Rare Artist Reception, during Rare Disease Week on Capitol Hill in February. One of Melissa’s State Senators, Senator Boozman, will be among those attending.
Melissa’s art statement that will be displayed with her art is intended to raise awareness about Dysautonomia, raise awareness about how water therapy can be beneficial for someone who has it and to perhaps provide some hope for those who face their own life challenges. Melissa’s statement (below) will accompany her art piece, “Flora and Fauna” (shown at left).
“My rare disease, Dysautonomia, is a neurological disorder which causes faulty instructions to be sent to the body for many “automatic” functions. It particularly sends incorrect instructions to the circulatory system.
Like many of us with Dysautonomia, my circulatory system no longer supports me correctly. I am now limited in how long I can stand up (5 minutes for me) and in how long I can sit up (2-3 hours). Other than that, I have to lie down to keep my blood pressure from dropping so low I pass out, or from spiking so high, I’m at risk for stroke. As careful as I am, I still randomly pass out from low blood pressure 3-6 times a week. I am homebound and no longer drive.
I became severely disabled in 2016. I took up painting in 2017 for something to do during the long hours I am forced to lie down during the day. Painting soon took on a life of its own and had become a joyful form of self-expression for me. It has also led to me making new friends (other artists) despite being homebound. It has proven to me that; indeed, one door may close on your Life, but if you search hard for a positive new door then you’ll likely find it.
Like many who have Dysautonomia, I am quite limited in standing on land although I can stand for hours if chest deep in the low gravity environment of water. I spend time each day in the water, happily exercising my muscles and enjoying the freedom of movement I no longer possess on land. My physical therapy time spent in water has inspired most of my artwork. Much of it has a recurring theme of a person, object, or animal weightlessly gliding about.
Some of my art pieces are underwater photographs I have taken in my therapy pool and then turned into paintings later on. This art piece, “Flora & Fauna,” is one of them. My daughter and I sat in the bottom of my therapy pool while I took a photograph of her releasing the silk roses. To me, it symbolizes one of those moments when Life requires you to let go of something you love, and you have no choice but to watch it drift away from you. Like the girl in the painting, we have to learn to let go of the roses. Otherwise, our hands won’t be free to hold on to the next good things coming our way. For those of us with rare diseases, those roses we have to let go of may be the life we had back when our bodies were healthy.”
Melissa encourages other people with Dysautonomia who create art to enter the 2019 Rare Artist contest. It’s a lot of fun, and you’ll be helping raise awareness for Dysautonomia. Entries for it begin in June 2019. You may enter through the “Rare Artist.org” Page on Facebook.
You may view artwork from this year’s and prior year’s Rare Artist Contest by visiting their website: https://www.rareartist.org/
DINET congratulates Melissa and all the other rare disease warriors who participated in this fantastic contest. If you have participated in something to bring awareness to Dysautonomia disorders, please let us know by emailing email@example.com Please include the words "dysautonomia awareness" in the subject line.
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“ I am always so dizzy!”
That was the beginning. My doctor shrugged his shoulders and said: “ We will keep an eye on that.” I knew something was off but really had nothing to report, I just knew I wasn’t right. I kept feeling like I was going to pass out. One day, I was home alone with my 4-year-old daughter, and I had to lie down on the floor knowing I was going to faint, heart racing, shaking. “ If Mommy goes to sleep on the floor dial 911,” I told my daughter and pointed to the numbers on the phone. But I recovered. A week later it happened. I was already wearing a heart monitor because of my complaints about having weird heartbeats. I was at work, a nurse at a cardiac unit, walking down the hall, heart racing and pounding, feeling like my heart was going to explode. When my knees got weak, I leaned against the wall …. when I opened my eyes I was on the floor, panic and noises and activity around me. 168/110, heart rate 160 – that was what they said. Minutes later I was in the ER, admitted and scheduled for a stress test in the morning. The heart monitor showed Tachycardia 160’s for 3 hours prior to the faint.
I had never even heard of POTS or dysautonomia or anything like that, and neither had my doctor. He sent me to a cardiologist for a TTT ( Tilt Table Test). I had never heard of that one either. I failed the test by promptly passing out. “ You have the common faint and most likely will continue to pass out “ was the expert opinion by the cardiologist. “ Take this pill – Metoprolol – and good luck”! I went online and typed “tachycardia and hypertension” and kept on reading, realizing that I had POTS. I saw 5 cardiologists, and a neurologist who performed autonomic studies ( which I was told were unremarkable ) and I tried everything they told me to do – drink more, take salt tablets, wear compression stockings. But the fast HR, high BP, fatigue, palpitations, and the ice cold hands and feet continued. I kept passing out several times a week. And then something changed: one day I woke up from a faint at work, surrounded by nurses and doctors, and was told: “You just had a seizure!!!”. That’s when I had enough. I did not want to have this anymore, I needed to go on with my life. Why couldn’t I just take a pill and get better?
A several day stay at an epilepsy monitoring unit finally showed it: when I fainted while hooked up to EEG and heart monitor, the EEG showed a change in brain waves - excessive vasoconstriction causing blood flow to the brain to stop. The monitor showed tachycardia, an unusual finding since they had expected a drop in HR or even for the heart to stop beating. The neurologist was stumped. So was I because this was not what I had read about POTS. So, I found myself an autonomic specialist and one year and another TTT later I sat in his office, unloading my story to an endlessly patient and understanding physician Dr. Blair Grubb. For 2 hours I spoke without hardly taking a breath while he listened. Then he told me what I thought was true all along – that I had POTS. And after drawing blood to check my neurotransmitters, the diagnosis was confirmed: hyperadrenergic POTS. Norepinephrine levels were elevated both lying down as well as being upright. “What now?” was my response. And from then on I saw him frequently, even passing out in his office. He implanted a loop recorder that monitored my heart rhythm for 3 years until the battery ran out. All it ever showed was tachycardia and PVC’s (premature ventricular contractions - extra heartbeats that can cause a fluttering or a skipped beat in your chest). Every faint, every seizure – tachycardia. Harmless in the eye of cardiologists but life-altering to me. I tried many, many medications, different beta blockers, Midodrine, Mestinon, Norvasc, Clonidine, Lexapro and more … all with varying results. All along, despite still being ill, I knew that I was in good hands and that Dr. Grubb would never give up. Every time I saw him I was crushed, hopeless, and frustrated, but he always gave me hope, and if one thing did not work, he tried another.
The worst thing was not even the physical symptoms, it was the fact that my life had changed. I was no longer the multi-tasking, healthy, always-on-the-go and productive nurse/mother/wife/woman that I was used to being. I was reduced to a cripple, unable to work, to take care of my family, to function. I had lost my independence. I could not drive or work or shop or go out or eat out or see my daughter dance or get awards at school. My smiles were faked grimaces, my laughter was a sigh; my days were dark and my nights were long. I did not want to get out of bed, the only place where I could feel better and was not afraid of losing consciousness. This anxiety was my enemy, always being scared of what might happen if I get up, seizure or syncope? My body and spirit were covered in bruises. In the mornings, I would wake up from a restless sleep and think: “ Do I really have to do this all over again? Do I really have to leave this bed?” And I started to believe that it would be so much easier for everyone if I could just die. There was no use for me any longer, I was just a burden to society. But then I thought of my daughter, and I realized – this is depression!!!
I made an appointment with my doctor and told him how I felt. I talked to my best friend. I saw a neuropsychologist and found that these feelings were normal for someone living with chronic illness. But this was not me. I was strong, I was young, I was better than this. I started to eat better and to exercise ever-so-slowly. I began to join my husband on shopping trips in my wheelchair, and I played games with my family. I found joy in preparing meals and baking my own bread. I went to church as often as I safely could. I got my groove back. I was better than that. I did not have to be a victim of this illness. If I could not live WITH POTS, then I would live DESPITE POTS.
Today I am well. I am disabled and no longer work, and I still have POTS. But my life is good – just different from what it was 10 years ago. I realize that for many people, the depression becomes an additional struggle on top of the physical ones, but luckily my depression improved with the changes I made. The medications prescribed for my physical symptoms keep me upright and functioning. The most effective treatment for me is weekly IV fluids. It took me years to convince my doctor that I needed them on a long term basis. I had spent more observation stays in the hospital for IV fluids than I can recall. Over the years I was in the ER or hospital every 6-8 weeks. I would literally crawl in, and the next day I left skipping and hopping. My doctors were against inserting a port and my receiving IV fluids on an ongoing basis. The reason being the risks associated with a port ( blood clots, infection ). I begged my PCP for several years, but he did not feel the benefit would outweigh the risk. Until last year, when some doctors refused to give me IV fluids because “ I could drink.” This led to several unnecessary seizures and me having to crawl again. So finally my PCP had had enough and agreed to the port and weekly home infusions – and my life changed to the better. I used to pass out frequently, the longest I went without a seizure or faint was only 11 weeks. But recently, I had my first syncopal episode after 5 (!!!) months!!! And the best thing is not just the fact that the spells have stopped but that my quality of life has improved. I am no longer fatigued. I can be active. I can exercise. I sleep better. I am no longer afraid to leave the house for fear of passing out.
Today I shed tears of laughter, and the hope that I had almost lost once is my daily companion. We CAN live despite POTS and better days ARE coming. We just can’t give up.
Editor’s note: Depression can be a natural response to chronic illness. For some people, like Susanne, making changes in your outlook and lifestyle can be enough to help you through that depression. But that is not the case for many people who suffer from depression and there is no shame or blame for the patient or the family. If you have depression or are caring for someone who does, please seek help from a professional. Long lasting feelings of despair, hopelessness or thoughts of hurting yourself need to be addressed immediately. Contact the National Center for Suicide Prevention https://suicidepreventionlifeline.org/ Or call 1-800-273-8255
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Technology is bringing chronic pain and chronic illness patients together, allowing us to participate in life in ways that we normally would not be able to. I volunteer with a non-profit as the Co-Editor of a blog, so I get to see first hand how technology is bringing patients together all over the world. Technology is not only making things more convenient but also creating a sense of family and comradery. It is helping to combat the sense of loneliness and isolation we all have experienced. Resources are also something that are being made more available through the wonder of technology.
Applications for creating art
There are so many ways that technology can enrich the lives of people with disabilities. Currently, my new favorite toy is a painting application. Before I got sick, I loved to paint and make art. Today, I have to weigh the cost to my health inherent in the traditional process of painting. My health is usually the one to win that analysis. However, using the painting app there is no cleanup, and I can do it from my bed on my tablet. This is as close to painting as I can get, and if you have a stylus, it can make the art even more rich in detail. There are many different apps out there, some free and some a nominal cost, but for an artist who lost the ability to create, it is priceless.
Zoom, Skype, Facebook Messenger Video and similar programs and apps.
Many of us know the loneliness and isolation that comes with being chronically sick. I often wonder how people with these conditions connected in a consistent and fulfilling way before our techno boom. I would say 90% of my interactions are through the marvels of technology. I have attended chronic pain support group gatherings via zoom. Zoom is a video conference chat app so you can see the faces of the people you are meeting with. There are times when I cannot get out of the house for my mental health therapy. In those instances we utilize zoom. That practice alone saves my sanity regularly. I find comfort in seeing the face of the person I am conversing with. It feels more personal and more like I am making a connection than a phone call does.
Accessibility programs and speech to text
Whatever form your disability manifests, most operating systems have accessibility options. My personal favorite is Talk to Text dictation. This is an essential tool for days when symptoms create physical barriers to typing and writing. For me, there are days when my fingers don't work correctly. Having the option to speak my thoughts and have them dictated, allows me to communicate more often with the people I care about.
For people who are blind or vision impaired, another form of accessibility is found on most websites. Typed descriptions of images are included to allow access to those images via sound. For the deaf and hearing impaired, let’s not forget closed captioning, which is a feature on almost every new TV, computer and movies. Since 1990, when the Americans with Disabilities Act was signed, closed captioning has been required for instructional and educational videos. However, over the years it has become an accepted practice for many other types of media. More recently, programs have become available that can be operated by eye movements. These features are often utilized by ALS patients and people with paralysis. These types of programs allow patients who cannot communicate in any other way to interact. There are even art and music programs being introduced that are designed to operate with eye movements.
By definition, social media is designed to bring people together and to connect us. It is one of my favorite tools in my toolbox. On almost every platform you can find a support group or community for whatever your disability is. It has had an overwhelmingly positive impact on the disability community. I have heard many people say that when they found “their tribe” they no longer felt alone in their battle. It can connect you with people all over the world who share your conditions. Also, social media has done wonderful things for awareness causes. Many illnesses, like dysautonomia and EDS, were virtually unknown by the general population. I have been able to use social media to educate family and friends and to share my day to day struggles. It also fosters resource sharing. When new research or studies are released, the news quickly spreads across social media. Through "following" platforms like Facebook or Twitter belonging to groups like DINET or other dysautonomia organizations, patients have access to feeds of information that they wouldn't have had 15 years ago. This gives patients the ability to better advocate for themselves with their medical team and to explore the latest trends in treatments. Of course, it is vital that care is taken to confirm the identity of the groups you follow and to be sure that you check all the information you see with medical professionals.
Smartphone and tablet apps
These days there is an app for EVERYTHING! Including symptom trackers and health apps. One that I like is backpack health, you can put in all of your information, and they give you a personal URL that can be printed on a medical ID bracelet.
The Amazon Alexa is another device and app that is proving to be helpful for disabled people. Along with the Echo, you can call for help if you fall from any room within listening range of the device. You can activate the tablet or phone with Alexa’s name and give a command to call 911 or a family member for help. I have personally used it to call my husband after falling. There are also smart plugs that have apps to turn on and off electronics, saving you from the need to get up. There are also FitBit and apps like that that allow you to track health information. Being able to monitor heart rate and sleep cycles can be a useful tool in managing symptoms.
There is no way I could list all of the ways smartphone apps make my life easier. I can do most everything I need to do online now. Grocery Shopping, medication renewals and "to do" list applications, have all made my life easier and save me precious spoons that I need so I can be present with the ones I love. We are even able to read books through apps in bed or have them read to us. When I am having a tough day and cannot read my daily meditations, I have them read to me through the app. I am virtually unstoppable as long as I have my phone!
Virtual Walk and Races
I have taken part in a Virtual 5K races for an organization that I volunteer with, and I loved the accomplishment. Runners have been competing in virtual races for a long time, but it recently has been a part of awareness campaigns to bring attention to illnesses where people cannot attend in person. You track your steps or distance in your wheelchair and register them with the event site that is doing the walk. Doing it at your own pace is a way we can reach our goals without compromising our health. I wrote an article for the mighty on the impact of these virtual races. You can find it here: https://themighty.com/2017/11/virtual-races-awareness-walks-dysautonomia/
I polled the community before finishing this article, and I was amazed at how much we rely on technology to make our lives easier and more fulfilling. Mobility devices are also making huge strides in technology and are utilized very often. So often, in fact, it may end up being a separate topic for a future article. Adjusting to a less active life is one of the hardest transitions most people with chronic illness need to make. It was a hard transition for me. Looking at the way I used to do things and finding easier ways, was a turning point for me. We found many solutions in using technology, and it has helped me live my best life. I still have a few things on my wishlist; such as a laundry folding machine (yes they make those!), a vacuum robot and a robot version of Dr. House. I hope after reading this, you also add some tech to your life! If you have some ways that technology has made your life easier or more enjoyable, I would love to hear about it. Please share with the community in the comments.
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Traveling can be an undertaking for anyone, but it is particularly difficult for those of us who are balancing our precarious health and our everyday lives: you could be thinking, “How could I possibly think about travel in addition to everything else?” Travel IS possible with dysautonomia though it can be difficult. However, the rewards of traveling, whether they are exploring a new place or visiting loved ones, can be well worth the struggles.
Here are five tips for traveling with dysautonomia:
1. Build in margin. This was the best travel advice I ever received from a stranger on a plane. She told me the key to reducing stress in life was building margin into everything you do. Do you think you need two hours to get through the airport? Schedule two and a half. That margin allows for any unforeseen mishaps (which will certainly happen) and prevents you from rushing. For me, rushing is almost guaranteed to cause a flare, whether it be moving too quickly and getting my heart rate too high, or the stress of rushing causing me to forget something important.
2. Prepare, Prepare, Prepare. For anyone, forgetting something on a trip is annoying but, for us, it can mean the difference between being relatively healthy and really, really sick. Not to mention, we have that pesky little thing called brain fog that can make remembering things tricky.
Start packing early and always use some type of checklist that you refer to multiple times. If you travel frequently, keep a core list of items you need, organized by category (e.g.; medications, toiletries, core clothing, assistive devices, etc.), and you may even consider making supplemental lists for specific types of travel (e.g.; summer trip, winter travel, staying with friends/family, etc.). Also, NEVER cross anything off your list until it is permanently in your bag. This prevents major mishaps such as taking your medications out of your suitcase the night before and forgetting to put them back in your bag because they were already crossed off your list.
Next, *try* to pack minimally. This does not mean leaving your essential medical devices and medications at home—we know that it may be impossible to pack light with these items. This does mean, however, that if you are going to be lugging a lot of medical items around you may thank yourself if you leave that cute, but impractical outfit at home. Try to pack things that are multifunctional (e.g., comfortable shoes that can be dressed up or down) to limit the physical and mental stress of keeping track of (and carrying) so many things!
3. Know that air travel IS possible. I used to love the thrill of flying and the energy of the airport. I still do, as long as I am realistic about how I navigate these situations with dysautonomia.
Start preparing the week before you fly. Prioritize light, daily stretching to prevent muscle cramps. Drink even more water than usual two days before the flight. Make sure you have everything you need; for me, this includes high-quality compression socks, comfortable clothes, my medication (always, always in my carry-on!), a vomit bag, a neck pillow, two empty water bottles (refill stations are in most airports), salty snacks, a packet of electrolyte mix, and a tube of Icy Hot. Your list may be different, but put some thought into it ahead of time. Also, the flight will likely be uncomfortable, so balance that by treating yourself! Save a book you’ve been wanting to read/listen to for the flight. Download your favorite TV show to binge watch. Bring snacks you love.
Next, it never hurts to ask. Call ahead and arrange a wheelchair or trolley escort through the airport, if needed. If meals are being served on the plane, call the airline to be sure your dietary restrictions are on file; they will bring you meals to accommodate your restrictions, and you will get served first! If your suitcase is overweight, or you have extra bags, due to medical devices remind your gate agent that they cannot charge you baggage fees for these items. Request a hand security check if removing your medical devices is a hassle. Make your airline aware of your condition (even if it is invisible), and request to board with those who need additional assistance. Sometimes, upon request, the airline may even give you the bulk seat which has extra legroom, for no additional charge. It doesn’t always work, but it is worth a try! It is always helpful to have documentation of your condition with you, in case anyone questions you.
Lastly, try not to be embarrassed about asking for needed accommodations. Admittedly, I do sometimes get shy about asking for help, but I am always so grateful I chose not to stress my body more than it needed to be.
4. Plan for arrival fatigue. Travel is tough. You will be tired when you arrive at your destination. If you flew to your destination, have a plan in place to get to your accommodations painlessly—call ahead to arrange a pick-up car or shuttle, check to make sure Uber/Lyft regularly frequents that airport, or have a loved one pick you up. Save walking and exploring public transportation for later in your trip when you feel more rested.
Plan for a travel flare, but hope it never comes. Try to limit your activity on your first day of arrival. Also, be easy on yourself if you need to take one, two, even a few days of your trip to rest. I used to get angry with myself when I traveled to an amazing location and spent half the time in bed and, ultimately, pushed myself too hard. I’ve changed that mentality. If I need to rest for three days, so be it, even if it means that I only get one day of truly enjoying my trip. To me, one day of actual enjoyment is far better than five days of pushing myself to the point of distress.
Jetlag can be a whole different beast for those of us with dysautonomia when changing time zones. Do your best to follow the golden rule of jetlag – try not to sleep during the day. That being said, those who created the golden rule probably never felt the crippling fatigue of dysautonomia. If your body is forcing sleep upon you that you can no longer fight, do not stress. Sleep. You may take longer to adjust to the new time zone, but it is worth it to keep yourself as healthy as possible. You will probably wake up at strange hours of the night. Have a good book on the ready to distract yourself because stress will only keep you awake. You will adjust eventually.
5. Choose accommodations wisely. I am all for getting local experiences, but accommodations may not be the way to do that when you have dysautonomia. The benefits of a chain hotel include standardized rooms and knowing what to expect. They may have transportation to and from the airport, you can control the temperature of your room, you will know the quality of the beds and linens, you will have a private bathroom, and you can request an accessible room. Upon asking, you can usually get needed items like mini refrigerators, extra pillows, and microwaves. Staying in a local hotel or Airbnb is not impossible, but you should do your research ahead of time to make sure you will be comfortable.
It can be trickier to navigate staying with family, friends, or renting a house with a group of people. You will probably have less control over access to food, the comfort of your bed, noise, lighting, and temperature. You may also feel torn between engaging with your companions and retreating for much-needed rest. In these situations, do your best to educate your loved ones early and often about dysautonomia. You can even have an advocate help you do this if you are uncomfortable. That being said, many loved ones have the best intentions but still may not fully understand your needs so you may need to make extra preparations. If you are driving, bring your own bedding that you know is comfortable. I have come to love my sleeping headphones; a headband I can pull over my eyes with soft headphones built in to drown out noise. If food is a concern, offer to grocery shop for the hosts as a way of thanking them, which also ensures you have some foods that you can eat. Or, cook dinner one night – sharing a favorite recipe is a great bonding experience, AND you can cook something that works with your body. If you are renting a group house, offer to be the person that manages the booking situation to find a house that meets your needs. In doing so, you can be sure you won’t be stuck on a rickety pull-out couch or sharing a bathroom with eight people.
Most of all, be honest with your loved ones. Most people will appreciate your honesty, and will not view you as difficult or high-maintenance. I have found that when my needs aren’t considered in group travel situations, it is usually the result of a misunderstanding, which can be rectified over time with communication and education.
While each of these tips could be an article in their own (perhaps, they will be in the future!), we hope these brief tips provide you with some ideas—and confidence—in planning your next adventure.
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