Making a difference
Institute recognized globally for expertise in studying, treating mysterious condition
A busy mother of two and full-time teacher, Kristina Sayer, 37, seems to have loads of energy. It’s hard to believe that the optimistic, outgoing wife and mom was confined to her bed with a bevy of debilitating symptoms just 18 months ago.
The mysterious symptoms of extreme fatigue, headaches, chest pain, a racing heart and dizziness came on Sayer suddenly, with no obvious trigger.
Concerned, she saw her doctor and received an electrocardiogram (ECG) – a standard test to check on her heart health. All of Sayer’s tests came back as normal, yet her condition worsened over the summer of 2018 with additional problems like debilitating migraines, lightheadedness and stomach problems.
“I felt like my legs would go out from under me at any moment and I would go ‘splat,’” she says, adding she was in and out of emergency departments seeking the cause of her symptoms. But there was no diagnosis, merely the suggestion she might be stressed.
By the end of the summer, Sayer couldn’t keep food down. Caring for her two young children was difficult. She knew going back to her classroom was out of the question.
“At my worst, I couldn’t get out of bed. It was so debilitating and frustrating because I couldn’t do anything to fix it,” she said.
Fortunately, while at the emergency department in Three Hills, a triage nurse asked if Sayer had received an ECG while standing. The test is generally performed while the patient is lying, and Sayer hadn’t had one in an upright position. The results of the ECG were immediate and dramatic.
“I was upright for about two minutes and my heart rate was up to 184 beats per minute,” said Sayer. “The doctor made a few phone calls to colleagues and that’s when I first heard the term POTS (postural orthostatic tachycardia syndrome).
Sayer was relieved to finally receive a diagnosis and was referred to Dr. Satish Raj, MD, a Libin cardiologist and researcher who specializes in autonomic nervous system disorders like POTS. It is a relatively common autonomic nervous system disorder impacting up to one per cent of the population worldwide (60,000 to 300,000 Canadians).
Raj helped the young mom get her condition under control. Today, Sayer is back teaching and describes her condition as “manageable.” Sayer’s POTS story is a bit unusual in that she received her diagnosis within months of her first symptoms.
On average it takes more than five years for patients to receive a diagnosis. However, her sudden onset and debilitating conditions are similar to the stories of many POTS patients.
According to Raj, who is one of just a handful of POTS clinician-researchers in Canada, symptoms of the condition vary greatly from patient to patient, but the common thread is a dysfunction of the autonomic system. When POTS patients stand up, their heart rate increases excessively, resulting in low blood pressure that leaves them feeling lightheaded and faint.
The condition can impact nearly every system in the body. Besides light-headedness, a racing heart and stomach problems, patients can present with body pain, brain fog, fainting and gut issues, amongst other debilitating symptoms.
According to Raj, the average age of onset is 14 years, and at least 90 per cent of POTS patients are female. However, the condition is not limited to teenagers. Half of all patients first develop POTS symptoms in adulthood.
Although POTS was first diagnosed at the Mayo Clinic in 1993, the condition is still relatively unknown by the public and even health care practitioners. Patients are often misdiagnosed with anxiety or other conditions before receiving a proper diagnosis. The causes of POTS aren’t known, but researchers at the Libin Cardiovascular Institute are working to change that. Their efforts are paying off.
The team made up of Raj, Dr. Robert Sheldon, MD, and Dr. Carlos Morillo, MD, all cardiologist-researchers at the Libin Institute, is recognized internationally as a centre of excellence in POTS research.
Lauren Stiles, founder and President of Dysautonomia International, a non-profit patient advocacy organization that funds research, educates physicians and raises awareness about POTS and other autonomic disorders, says there are fewer than 100 expert POTS experts worldwide.
“The Libin Institute is a leader in the treatment and research of autonomic nervous system disorders in Canada and around the world. We are very grateful for the research and clinical care going on in Calgary,” says Stiles, whose journey to a POTS diagnosis took years and was fraught with misdiagnoses, including cancer.
Besides seeing patients from all over Western Canada, Raj, Sheldon and Morillo give talks around the globe, sit on advisory committees and write guidelines to help improve awareness of POTS and enhance the quality of life for people who live with this condition.
Recently, Raj and Sheldon were part of the Canadian Cardiovascular Society committee that wrote and presented a position paper on the condition at the Canadian Cardiovascular Congress – the largest gathering of cardiovascular specialists in Canada. Chaired by Raj, the document makes suggestions about how to diagnose and treat POTS based on the latest research.
Raj is also the president of the American Autonomic Society, serves as a medical advisor to many POTS patient groups, including POTS UK and Dysautonomia International.
There is also a large research component to the work of these physicians. For example, several projects are underway in the Raj lab, including a cardiac MRI study in POTS patients and an international, collaborative study looking at different methods of detecting an antibody that has been found in some patients with the condition. (See more at https://cumming.ucalgary.ca/labs/calgary-autonomic-investigation/home).
Sheldon is currently studying two medications in the treatment of POTS. All three studies were supported by research grants from Dysautonomia International.
The team is also training the next generation of Libin researchers.
Kate Bourne, is embarking on a PhD in Cardiovascular and Respiratory Science at the Cumming School of Medicine with her supervisor, Raj. She is highly interested in understanding POTS, including the impact it has on patients.
Using data from The Big POTS Survey, an international study of more than 8,000 POTS patients developed by the University of Calgary, Vanderbilt University and Dysautonomia International, Bourne recently completed a study on how POTS impacts the lives of patients economically – something previously underestimated.
“People don’t recognize the huge impact POTS has on young women in their formative years,” she says. “Women who should be going to school or working are instead debilitated with this condition.”
Although POTS is not well understood, Stiles is optimistic about the future, given the targeted research and patient engagement projects underway at places like the Libin Institute.
“We are making headway,” she said. “POTS is not quickly diagnosed or easy to treat, but we are making progress in reducing diagnostic delays and finding more effective treatments.”
For more information on POTS, watch this video at https://vimeo.com/187404694 or visit cumming.ucalgary.ca/rajlab/autonomic-disorders/pots. Patients and caregivers impacted by POTS can find a support group at the following link: dysauttonomiainternational.org/support.