Exciting news! Dysautonomia International successfully advocated for a unique ICD-10 code for POTS in the US for the first time! US clinicians can begin using the new POTS code, G90.A, as of October 1, 2022 – just in time for our 10th Annual Dysautonomia Awareness Month campaign.

The International Classification of Diseases (ICD) is an agreed upon terminology for medical conditions created by the World Health Organization, a branch of the United Nations. Each country can choose to adopt the ICD, and make some edits to the terminology to be used in their country. ICD-10 is the current version and the 10th revision of the ICD since its inception in 1909.

POTS historically did not have its own unique ICD-10 code. It was listed as a synonym under I49.8 “Other specified cardiac arrhythmias” along with numerous other medical conditions like Brugada syndrome, re-entrant atrioventricular tachycardia, ectopic rhythm disorder and other arrhythmias. POTS, however, is not considered to be an arrhythmia. The tachycardia (fast heart beat) seen in POTS is a normal sinus rhythm. POTS being lumped together with so many other medical conditions made epidemiology, health care utilization, and other electronic medical records research impossible.

This is why Dysautonomia International set out to get POTS its own diagnostic code in the ICD a few years ago. Dysautonomia International Medical Advisory Board member Dr. Jeffrey Boris led the charge, along with Dysautonomia International President Lauren Stiles.

First, they obtained POTS a new ICD-11 code from the World Health Organization, which you can read here. ICD-11 is not implemented yet, but it has been adopted by all World Health Organization member countries and implementation should occur over the next few years. In the US, ICD-11 implementation may begin in the 2025-2027 time frame, if we’re lucky.

While we wait for ICD-11 to be implemented, we pursued an ICD-10 code change in the US, the country with the most number of POTS research centers and POTS aware clinicians. Dr. Boris and Lauren presented before a US Center for Disease Control committee last year, and the committee just issued their decision to approve a unique ICD-10 code for POTS at G90.A. This places POTS in the ICD-10 section with other autonomic nervous system disorders, where it should have been all along. This new ICD-10 code goes into effect in the US as of October 1, 2022.

The implementation of a unique ICD-10 code for POTS in the US will facilitate better research studying the epidemiology of POTS, the evolution of the illness over time, use of health care resources, disability determinations and much more. But… that only works if US clinicians start using the code, so please let your doctors and other medical professionals know about this code change! Let the billing office staff know too, since they do most of the coding. You can give them this Provider Letter.

We are having conversations with POTS experts in other countries to determine if it is feasible to pursue ICD-10 changes in other countries at this time, as each country has its own lengthy legal process to change the coding system. We are also reviewing the ICD-10 and proposed ICD-11 with our Medical Advisory Board to determine if there are other autonomic disorders that are missing unique ICD codes. If any are missing that would meet the criteria for a unique code, we will pursue unique ICD codes for those diagnoses as well. Thankfully, most autonomic disorders already have unique ICD codes.

Having a unique ICD-10 code for POTS in the US is a huge victory for the dysautonomia patient community! This will help us get recognized as a large patient population that is currently being greatly underserved by the healthcare system.

We have many other advocacy projects in progress. You can help Dysautonomia International do even more advocacy that benefits our patient community by making a contribution to support this important work at CureDys.org. Even if you can’t contribute financially, you can still be part of our advocacy efforts by signing up for our email list.

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Hot of the presses! We are excited to announce that two journal articles summarizing the findings of the first National Institutes of Health (NIH) Postural Orthostatic Tachycardia Syndrome (POTS) Research Workshop, written by 30 of the top POTS experts including Dysautonomia International representatives, are now available. Part 1 summarizes the current state of scientific knowledge and POTS clinical care. Part 2 summarizes the top priorities for POTS clinical care and research, and calls for the NIH to fund an additional $50 million in POTS research over the next five years. (links below)

Persuading the NIH to host this workshop was the result of several years of Congressional advocacy by dedicated Dysautonomia International patient, caregiver and physician volunteers. As a result of our advocacy efforts, Congress encouraged NIH to host a POTS research workshop and directed the NIH to report back to Congress on:
(1) the current state of POTS research;
(2) priority areas of focus for future POTS research through 2025;
(3) ongoing and upcoming efforts by NIH to advance the scientific understanding of POTS; and
(4) an estimate of the level of funding that would be needed to achieve the above stated objectives.

The workshop took place on June 29, 2019 at NIH headquarters in Bethesda, Maryland the morning after Dysautonomia International’s 2019 Conference in Philadelphia, which most of the POTS experts had participated in. After presentations from several speakers, including Dysautonomia International President Lauren Stiles, and Dysautonomia International Medical Advisory Board members Dr. Satish Raj and Dr. Steven Vernino, who co-chaired the NIH meeting, and other experts, the attendees created recommendations for the NIH on what POTS research priorities NIH should support in the next five years.

The NIH staff summarized the impressive list of attendees and NIH’s perspectives on the workshop on the NIH website, and in a report to Congress from NIH Director Dr. Francis Collins. The POTS experts wrote their own summary, in the form of two journal articles, to further elaborate on their findings from the workshop and make recommendations to Congress and the NIH:
Part 1 – Postural orthostatic tachycardia syndrome (POTS): State of the science and clinical care from a 2019 National Institutes of Health Expert Consensus Meeting (this article summarizes the current state of scientific knowledge on POTS and the state of clinical care)
Part 2 – Postural orthostatic tachycardia syndrome (POTS): Priorities for POTS care and research from a 2019 National Institutes of Health Expert Consensus Meeting (this article summarizes gaps in scientific knowledge and clinical care, and identifies the top POTS research priorities for the next five years).

“While everything we know about POTS and everything we need to do for POTS patients cannot fit into two journal articles, these articles reflect an incredible collection of knowledge about POTS, and lay out the blueprint for the hard work that we must do to advance POTS research and clinical care.” noted Lauren Stiles, Dysautonomia International President and co-founder.

Dr. Satish R Raj Professor of Cardiac Sciences at the Libin Cardiovascular Institute and the Cumming School of Medicine at the University of Calgary, and co-chair of the NIH meeting explained, “[t]his is an important milestone for POTS research. We hope that the resulting recommendations that resulted from this workshop will be implemented by the NIH and other research funding agencies around the world.”

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Dysautonomia International is proud to be the voice of our patient community in the halls of government. Our strategic advocacy efforts are designed to affect change that will create a better future for people living with POTS and other forms of dysautonomia. We are able to do this advocacy work because of the support of individuals like you. Please consider making a contribution at DysautonomiaInternational.org/donate today to support our ongoing advocacy, research, and education efforts. We are making progress, but with your support, we can do even more!

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We’re putting the volunteer spotlight on Kim DiBona this month. Kim’s started volunteering for Dysautonomia International after her daughter’s diagnosis. “The two and a half year diagnostic delay in getting diagnosed, the hundreds of tests and appointments, the 25 doctors who couldn’t figure out what was wrong… I couldn’t sit back and let other families go through that.”

Kim has volunteered her time on our Patient & Caregiver Advisory Board since the launch of Dysautonomia International in 2012. “There was only one Facebook support group back then. I hosted a few support group meetings at the beginning, and everyone was happy to be in a room with people that get it.”

She co-hosted her first fundraiser in 2013 with Dysautonomia International founder Lauren Stiles and other volunteers at the Stone Creek Inn, East Quogue, NY. “At first I was afraid to ask for donations and gift basket items, but after the first donation came to my house, it inspired me to keep going. So many family and friends donated.  Since then, it lit the fire for me to continue to hold annual fundraisers where we live in New Jersey.”

The thing that touches Kim the most is bringing the patients and their families together. “One specific moment I hold dear to me is connecting with a family from Panama at the annual conference. Knowing you make a difference in someone else’s life just by volunteering is something you keep with you forever. Those memories mean so much because I remember each and every face, each and every hug.  Another special moment was the first annual fundraiser I held in New Jersey, seeing my daughter look at the room full of people supporting her and other dysautonomia patients like her, and the money we raised for research. It gave her hope and showed her that lots of people cared.”

Kim is also very active and helps moderate Dysautonomia International’s New Jersey Support Group on Facebook. She is always so kind and helpful to other members in the community. The message that Kim would like to give to the dysautonomia community is, “[k]eep the faith, have hope.  We have come so far in these past eight years. There is so much more to be done, and we could use more volunteers. Every little bit helps. Educating doctors, small or large fundraisers, the resources are here to help you. Some people have asked how I do what I do. My response is, I am just a mom on a mission and there are lots of us.”

Thanks Kim for everything you do for our community!

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We’re shining our volunteer spotlight on Kevin Gagen this month. Kevin has volunteered with Dysautonomia International since 2016. He was inspired to start volunteering during his daughter Caitlin’s junior year in college. “She was playing soccer at SUNY Plattsburg, her team wanted to bring awareness to POTS by having a fundraiser and game dedicated to POTS awareness. We came up with the phrase Kick it to POTS. Even though Caitlin graduated two years ago, the team still continues to have a Kick it to POTS game every year.” The Gagen family created a logo using their daughter as part of the “K” in Kick it to POTS that is worn at each of the games, and sold to help raise money for Dysautonomia International.

Kevin has enjoyed volunteering for Dysautonomia International over the years. “Everyone at Dysautonomia International has been very helpful and supportive. We have gone from our first Kick it to POTS games, to arranging local proclamations for Dysautonomia Awareness Month in both Madison and Onondaga Counties in New York State. It is a great feeling to share those moments with my daughter and to meet other people that are fighting the daily battle of dysautonomia. We have made some wonderful friendships and we have been able to bring awareness to the community.” 

“The best message I can share with the community is the word ‘support.’ My goal is to make people aware of dysautonomia. Each and every person is different, but we all need the support from family, friends and our community.  We will bring understanding, awareness and get closer to a cure each and every day. It has been my privilege to work with Dysautonomia International.”

Thank you Kevin for everything you do, and for always remaining so positive!

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by Lauren Stiles, Dysautonomia International President & Co-Founder

It is with a heavy heart that I share this sad news. Dr. David C. Kem, a leader in POTS research, passed away from COVID-19 complications on the morning of Sunday, November 22, 2020.

Dr. Kem was a good friend to everyone here at Dysautonomia International. We send our sympathies and prayers to his wonderful wife Janet and their many children and grandchildren.

Dr. Kem was a gifted researcher and clinician, who continued to innovate with his research until the very end of his life. Just last week, he submitted a new medical journal article with Dr. Blair Grubb. Upon hearing the news of Dr. Kem’s passing, Dr. Grubb noted, “David Kem was a gentleman and a scholar.” I could not think of a more perfect description.

Dr. Satish Raj, a frequent collaborator with Dr. Kem on POTS antibody research, added, “Dave Kem was a wonderful scientist and a great collaborator. He was passionate about his work, not only for the sake of science, but because of his strong belief that he could alleviate patient suffering. As accomplished as Dave was as a scientist, I will remember Dave as a true gentleman who was devoted to his family.  He truly sparkled when he spoke about his grandchildren. Dave will be greatly missed.”

Dr. Kem graduated from Columbia University’s College of Physicians & Surgeons in 1963. He completed post-doctoral training in endocrinology, metabolism and hypertension at the University of Michigan and the NIH in the late 1960s. Dr. Kem spent decades publishing important research on the relationship between hormones, immune function and cardiovascular disorders. He eventually led his own distinguished research lab at the University of Oklahoma, where he served as the George Lynn Cross Research Professor and a Regents Professor of Medicine.

I absolutely loved talking about POTS research with Dr. Kem. In his late 70s/early 80s, when many other researchers would have long ago retired, he was as excited about new research as a little kid opening presents on Christmas morning. You could hear the enthusiasm in his voice. He was determined to figure out POTS. Even when his health started to fail, he would have his wife Janet call to give me research updates from the hospital.

His last email to me was in August. He heard about a hurricane hitting New York on the news, so he emailed me to ask if my chickens were OK. Even though he was a brilliant academic and a serious medical school professor, he was still a sweet Oklahoma farm boy at heart. The email was also dotted with various research updates, some related to POTS, some not… aplastic anemia, marrow transplant, muscarinic antibodies, manuscript in review, new antibody assays going, now recruiting… I loved getting those emails. The email was followed by a long phone call, where we discussed post-viral autoimmunity in the context of COVID. I introduced Dr. Kem to some other researchers interested in exploring the role of autoimmunity in post-COVID syndrome, and after a few emails, they were collaborating on a new study.

How Dr. Kem became a POTS researcher in his 70s is a pretty cool “serendipity” story. This story was told to me by Dr. Kem and Dr. David Robertson, founder of the Vanderbilt Autonomic Dysfunction Center.

In the early 2010s, Dr. Kem was studying arrhythmias in Graves’ disease, an autoimmune thyroid disorder that is known to be associated with arrhythmias in some patients. He had identified adrenergic receptor antibodies (a type of G protein-coupled receptor antibody) in a subset of Graves’ disease patients, which he thought may be contributing to their arrhythmias, but when he look at their charts, he noticed that many of the antibody positive Graves’ patients had been diagnosed with POTS too.

Like many doctors, Dr. Kem wasn’t too familiar with POTS. He called his hospital’s cardiology department and asked if they had any POTS patients. Like most cardiology departments, they had more POTS patients than they knew what to do with. He checked the serum of some patients from a local clinic who had POTS, but not Graves’ disease, and sure enough, they all had the same adrenergic receptor antibodies as his Graves’/POTS study subjects.

Dr. Kem was not a POTS researcher at the time, but he thought he might be on to something big that warranted further research. He applied for an NIH grant. NIH rejected his application, but suggested that he may want to partner with an existing POTS research center and re-apply.

He cold-called Dr. David Robertson, founder and director of the Vanderbilt Autonomic Dysfunction Center, and asked him to collaborate on a study. He told Dr. Robertson, “I think I found antibodies that may be causing POTS.” Dr. Robertson was skeptical, but open-minded, so he told Dr. Kem if he could send him some serum samples from healthy people and POTS patients from Vanderbilt, and if Dr. Kem could tell who had POTS and who was healthy based on the presence of the antibodies, he would collaborate with Dr. Kem on a study. Dr. Kem agreed to Dr. Robertson’s plan. So Vanderbilt’s autonomic lab sent Dr. Kem 14 samples – seven from people who had been diagnosed with POTS, and seven from healthy people, with no indication of which tube contained which type of sample. Dr. Kem was able to accurately tell Dr. Robertson who had POTS and who was a healthy control, just based on his analysis of their serum.

After the results came back, Dr. Robertson called me and said, “Lauren, David Kem is brilliant, he has found something important, and you should fund his research.” Dr. Robertson was a legend in the field of autonomic research, and researchers don’t often encourage you to fund someone else’s research, so I knew this was going to be big news. Eventually, Dysautonomia International would fund several of Dr. Kem’s studies on autoimmunity in POTS and vagus nerve stimulation in POTS.

Dr. Kem’s research has had a ripple effect across the entire field. Over the past six years, he collaborated on POTS antibody studies with researchers at Vanderbilt, University of Calgary, Lund University, the Karolinska Institute, and UT Southwestern, amongst others. While we still have a long way to go to fully understand the role of immune function in POTS, Dr. Kem’s pioneering work on this topic has launched an avalanche of new research that will be felt for generations to come.

He will be dearly missed by all who knew him.

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We’re shining our volunteer spotlight on Dr. Blair Grubb this month. Dr. Grubb is an internationally recognized expert on syncope and autonomic nervous system disorders, who volunteers his time and expertise as a member of Dysautonomia International Medical Advisory Board.

Amongst many other titles, Dr. Grubb is a University Professor of Medicine and Pediatrics at the University of Toledo, where he runs a very busy syncope clinic. Patients come from all over the world to see him. Fun fact about Dr. Grubb: he was among the first researchers in the United States to employ head upright tilt table testing for the diagnosis of vasovagal syncope, which is now called neurocardiogenic syncope. He is also well known for being a caring and compassionate physician.

Dr. Grubb has had a fascinating life. He has traveled all over the world and speaks several languages. He has also published numerous essays on his experiences as a physician and a patient.

He volunteers for Dysautonomia International because, he notes, “this organization plays a critical role and its presence has helped advance the field.” He enjoys being apart of an organization that is reliable and brings together physicians all over the world who are interested in helping this community to “give a voice to the unheard.” For his lifetime of good work for our patient community, Dr. Grubb was named Dysautonomia International’s 2015 Physician of the Year.  He says, “[t]he award meant a lot to me.”

Dr. Grubb has a tons of helpful advice for people living with dysautonomia, but his most important advice is “[d]on’t give up on yourself. Keep trying to push for your best life.” He also wants other physicians to know, “this illness really does exist and we need to do our best to help these patients and their families navigate through this diagnosis.”

Dysautonomia International is so grateful to have Dr. Grubb on our Medical Advisory Board and our community is so appreciative of his work.

Thanks, Dr. Grubb!

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This month we would like to shine the spotlight on Kate Bourne, an active Dysautonomia International volunteer.

Kate started volunteering for Dysautonomia International shortly after she became a graduate student at the University of Calgary, working under the supervision of Dr. Satish Raj, a Dysautonomia International Medical Advisory Board member and collaborator on the “Big POTS Study.” Kate put her data analysis skills to work, mining the Big POTS Survey data for new information about POTS, resulting in the production of several research abstracts and manuscripts. Kate received a prestigious Vanier Scholarship from the Canadian government for this work and her other academic accomplishments.

Kate served as a representative of Dysautonomia International during the Heart Rhythm Society conferences in 2018 and 2019, where thousands of electrophysiologists gather to learn about the latest research in their field. Kate also volunteered her research skills to assist with Dysautonomia International’s Annual Conference research studies in 2018 and 2019, and she has lectured on her own research data during our annual conferences.

“I have enjoyed all of my volunteer experiences with Dysautonomia International.  Volunteering with the conference research projects has been particularly meaningful to me. It is an amazing experience to meet so many wonderful people, and work with an international research team, while helping to increase our understanding of POTS.”

Kate says that the dysautonomia community is inspiring! “It is an honor to work with a group of patients and their supporters who are fully engaged and very willing to participate in research, and who will do anything they can to help us learn more about POTS and other autonomic conditions – thank you!”

Thank you, Kate, for everything you do!

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This month Dysautonomia International would like to put the Volunteer Spotlight on Amy Kohut who has been volunteering for Dysautonomia International since our launch in 2012, and recently joined our Board of Directors.

Amy fell ill during her first year of law school and ended up receiving a diagnosis of POTS from Dr. Thomas Ahern of Scripps Health in San Diego in 2008. Amy says, “He saved my life. I had an “old school” Tilt Table Test and let’s just say it wasn’t pretty.” Amy started the California POTSies Facebook group in 2009 to see if anyone else had this syndrome, and sure enough, she found out she was not alone in having POTS.

Amy “met” Lauren Stiles (telephonically) in 2012 while studying for the California Bar Exam. “We spoke on the phone for hours about our respective experiences in obtaining proper diagnoses, law school and life stuff. We both volunteered to give a radio interview about dysautonomia in the early days of Lauren’s non-profit vision, but there were technical issues, and Lauren ended up getting locked out of the interview, so to speak. I had to wing it.”

After Amy’s riveting radio performance, she was appointed as the first Chair of Dysautonomia International’s Patient Advisory Board and represented the organization at the very first medical conference the organization participated in, The 10th International Catecholamine Symposium, in 2012. Amy was invited to lecture before a group of the world’s leading autonomic experts. “I did not know what a catecholamine was at the time, but I did know they could be measured and abnormalities in catecholamines were correlated to different forms of dysautonomia.” Whatever Amy said worked, as several dysautonomia experts in the audience join our Medical Advisory Board after that conference. “We were off and running with a new non-profit and a growing patient and clinical community.”

Dysautonomia International even got Amy interested in football. “I never understood the feeling of joy at the end of any Super Bowl until the Eagles won in February 2018. My husband’s family was rooting for the Eagles because that’s their team. I was rooting for them because then QB Nick Foles’ wife, Tori Foles, had been battling POTS and was helping Dysautonomia International bring international awareness to the condition. I was so excited when they won that game, I had to take an extra beta blocker!”

We asked Amy what she would want to share with the dysautonomia community, and she had a great response that will resonate with a lot of us right now. “We are living in uncertain times right now with a global pandemic that doesn’t seem to discriminate among the sick and the healthy, the rich and the poor, or, the old and the young. As long as the dysautonomia community sticks together, as we have done since the early days of social media, we can support one another in riding out this storm. As always, our patient population puts our faith in the scientists, the researchers, the clinicians and the greater volunteer community.  Despite these difficult times, we will continue to bring awareness to all forms of dysautonomia and all known etiologies, continue funding research so that dysautonomia patients do not experience the unbearable diagnostic and treatment delays of the 1990s and early 2000s, and educate the next generation of healthcare providers in autonomic neurology. While we may be in a holding pattern as the world comes together to prevent, test, treat and care for COVID-19 patients and providers, those with dysautonomia know better than most how to cope with uncertainty. We will get through this together, as we have been doing for the last decade as a united and well-informed patient community.”

Thank you Amy for everything you’ve done and everything you continue to do!

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This month Dysautonomia International’s Volunteer Spotlight is shining on Lorin Bales, leader of our Alabama Support Group. Lorin and her husband live in Huntsville, Alabama or as the locals call it, Rocket City, for their NASA and missile defense industry. Like most people from Huntsville, Lorin considers herself a nerd, programmer, and lover of all things science. She has loved astronomy and telescopes since high school, which fueled her passion to major in physics, and eventually led to a career as a rocket scientist.

Lorin and her husband have two big, loveable, goofy newfoundlands. And just like her choice in dog breeds, Lorin has a big heart and is intensely determined.

When we asked Lorin what motivated her to start volunteering for Dysautonomia International she explained, “Dysautonomia International inspires me because I’ve seen the impact that the organization and its volunteers have had in the medical community. I’ve witnessed the increase in awareness, the new research paths of causes and treatments, and the rallying that comes from our support groups.”

One of Lorin’s favorite memories of volunteering with Dysautonomia International is when a group of nurses showed up to one of our local events and began asking questions about dysautonomia. “It wasn’t that they had never heard of it, but they were curious about effects beyond dysautonomia with pregnancy. I’ve had many similar experiences outside our events, where I don’t have to explain what dysautonomia is anymore, but can teach people more about how it affects our lives.”

Lorin’s message to the dysautonomia community is, “you will never stand alone. We are here when you are tired, when you feel the darkness pressing in, and you don’t feel strong anymore. We will be here when you finally get that perfect medication regimen, when that test result is finally positive, when you know why you’re sick, and when you are able to return to the things you love. We will wait with you. We’re listening. We are here.”

Thank you for everything you do, Lorin!

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