Living with Dysautonomia: More Than Just a Diagnosis
By: Shannon Pfau CEO, OTR/L, CTP
My dear readers, if you have been here since the beginning you know it’s been awhile since I sat down and did a blog post. It took something more personal to spark that creative spark again. For those who are curious of why I am writing- October marks Dysautonomia Awareness Month, so naturally I find myself reflecting not only as an Occupational Therapist but also as someone who has a continued lived experience with hEDS (Hypermobile Ehlers-Danlos Syndrome) and POTS (Postural Orthostatic Tachycardia Syndrome). My journey weaves through the professional terrain of OT, but it also navigates the personal challenges of managing chronic illness. With two active toddlers at home, the stakes are high to manage my symptoms effectively and efficiently, making my work at mOTivate Minds even more poignant.
Understanding Dysautonomia
Dysautonomia is an umbrella term used for various medical conditions that cause a malfunction of the Autonomic Nervous System (ANS). The ANS controls many functions in your body, like heart rate, blood pressure, and body temperature. Sounds simple, but it's a fundamental part of our existence. Common symptoms include dizziness, fatigue, fluctuations in heart rate, and gastrointestinal issues. Some lesser-known symptoms involve "brain fog," heat intolerance, and irregular sleep patterns. Dysautonomia is not rare. Over 70 million people worldwide live with various forms of dysautonomia.
How the ANS Works
Involuntary body functions are regulated by different parts of the ANS, some of which act in opposition to the other. There are three distinct parts:6
The sympathetic nervous system directs the "flight-or-fight" response during moments of stress, causing the widening of your pupils and speeding up your heart and breathing.
The parasympathetic nervous system counters the sympathetic nervous system and normalizes body functions. This can result in the narrowing of your pupils and slowing down of heart and breathing rate as you recover from stress.
The enteric nervous system directs the movement, blood flow, and secretion of mucus in the gastrointestinal tract.
Common Symptoms:
Dizziness/Lightheadedness: Some individuals (not all) have fainting (syncope).
Orthostatic Intolerance: Difficulty standing upright due to lowered blood pressure or increased heart rate.. It’s caused by a loss of blood flow to the brain, heart, and lungs. This is not a life-threatening decrease in blood flow, but it’s bothersome and some cases causes fainting. When standing upright is an annoying pain in the back of the neck and shoulders. This is not always a symptom nor a primary symptom. Because of the distribution of the discomfort, this is sometimes referred to as the “coat hanger sign” which occurs as pain in the back of the neck when standing.
Tachycardia: Abnormally high heart rate.
Fatigue: Persistent tiredness unrelated to activity levels.
Digestive Problems: Including bloating, constipation, and irritable bowel syndrome.
Brain Fog: Difficulty in focusing, remembering, and maintaining attention.
Temperature Dysregulation: Intolerance to heat or cold.
Sweating Abnormalities: Either too much sweat or not enough.
Urinary Issues: Difficulty in starting urination, frequent need to urinate.
Pupil Dilation: Resulting in light sensitivity.
Diagnosis
The best way to diagnose specifically looking at orthostatics and with POTS - a Standing Test. Take pulse and blood pressure in supine position after 5 minutes. Have patient stand without leaning for 2-10 minutes and repeat pulse and blood pressure every 2 minutes.
Adults: heart rate ≥ 30 bpm upon standing may indicate POTS
Children: heart rate ≥ 40 bpm upon standing may indicate POTS
Blood pressure should not decrease by more than 20/10 mmHg upon standing.
OFTEN as an OT would recommend that you discuss your symptoms with your provider and that you get refered to an autonomic specialist to have a tilt table test and quantitative sudomotor autonomic reflex testing (QSART).
Common Co-Existing Conditions:
Individuals with dysautonomia often experience co-existing conditions, either as secondary disorders related to their primary diagnosis or as separate but overlapping conditions.There are many underlying diseases and conditions that can lead to dysfunction of the autonomic nervous system.
Ehlers-Danlos Syndromes (EDS): A collection of hereditary connective tissue disorders that often worsen the symptoms of dysautonomia. The laxity and instability in the joints and skin can exacerbate autonomic dysregulation, affecting daily functionality and physical activities.
Postural Orthostatic Tachycardia Syndrome (POTS): Considered a subset of dysautonomia, POTS is characterized by a sudden increase in heart rate and a drop in blood pressure (criteria is different based on age group often 30 point difference in adults) upon standing. This can severely impact a person's ability to engage in basic daily tasks like standing or walking for extended periods.
Chronic Fatigue Syndrome (CFS): This condition has a significant overlap with dysautonomia, particularly in the realms of persistent fatigue and exhaustion. CFS can limit the individual's capacity to perform regular daily activities, including occupational tasks.
Fibromyalgia: A chronic pain syndrome that often coexists with dysautonomia, impacting an individual’s physical functionality and capability to maintain an active lifestyle. Persistent pain affects not only physical well-being but can also influence emotional and psychological states.
Irritable Bowel Syndrome (IBS): Many individuals with dysautonomia also experience gastrointestinal disturbances like bloating, diarrhea, and constipation. This can interfere with meal preparation and other activities of daily living that involve dietary intake.
Anxiety, Depression, and PTSD: Given the chronic and pervasive nature of dysautonomia symptoms, mental health conditions such as anxiety and depression are often comorbid. Additionally, many of these individuals have a significant history of medical gaslighting and need help finding dysautonomia specialists.
Small Fiber Neuropathy: This condition affects the small nerve fibers responsible for pain and temperature sensations, and is sometimes seen in dysautonomia patients. The neuropathy can inhibit sensory feedback during daily tasks, complicating physical interactions with objects and people.
Migraines, Chiari Malformation, & CSF Leaks: Some individuals with dysautonomia experience frequent and severe headaches or migraines. Many individuals with dysautonomia should be screened for Chiari malformation and csf leaks. CSF leak occurs when there is a tear in the dura mater, the membrane that contains the fluid surrounding the spinal cord and brain. This can result in symptoms like orthostatic headaches, which worsen when the individual assumes an upright position.This can be disruptive to daily routines, potentially making it challenging to focus on work or other activities that require sustained attention.
Autoimmune Disorders: Conditions such as Lupus or Sjogren's Syndrome can coexist with dysautonomia. The autoimmune response can influence various bodily functions, adding another layer of complexity to managing daily activities and self-care.
Mitochondrial Diseases: Dysautonomia can sometimes manifest as a secondary condition to mitochondrial diseases. The mitochondrial dysfunction can affect cellular energy production, impacting a person's ability to sustain physical activity and other daily occupations.
Mast Cell Activation Syndrome (MCAS): This condition involves the inappropriate activation of mast cells and is sometimes seen in individuals with CSF leaks. Symptoms can vary widely, affecting multiple systems in the body and complicating daily functional activities.
Long Haul COVID: Also known as "Post-Acute Sequelae of SARS-CoV-2 Infection (PASC)," this condition involves a range of persistent symptoms long after the acute phase of a COVID-19 infection has resolved. Some individuals with Long Haul COVID develop dysautonomia-like symptoms, such as fatigue, heart palpitations, and difficulty with concentration. These ongoing symptoms can disrupt autonomic nervous system functions
There are many different types of dysautonomia, including, but not limited to, neurocardiogenic syncope, postural orthostatic tachycardia syndrome, inappropriate sinus tachycardia, orthostatic hypotension, autoimmune autonomic ganglionopathy, pure autonomic failure, and multiple system atrophy. No two dysautonomia patients look the same. Dysautonomia can be mild or debilitating. A more severe case will require significant lifestyle changes as you try different treatments to address symptoms. For some patients, the goal is to have more good days than bad. Symptom management is possible, but it takes time and patience. Because the autonomic nervous system adapts and changes, the course of your condition may change as well. Some patients find a treatment plan that works for a while, but then a new symptom appears and becomes their chief complaint. However, what many do not know is that the disability seen in POTS has been compared to COPD and congestive heart failure. And unfortunately, there is no cure at this time with the main focus remaining on symptom management.
Medications often used:The Dysautonomia Project- Pharmacological Interventions.
Non-Pharmacological Treatments
Why Occupational Therapy for Dysautonomia?
Well lets look at some areas of daily life that might be impacted by living with Dysautonomia… (we call this different areas of occupational performance)
Activities of Daily Living (ADLs):
Personal Hygiene: Difficulty standing for prolonged periods may limit showering and grooming activities.
Dressing: Fatigue and muscle weakness may make dressing a challenging task.
Eating: Gastrointestinal symptoms may limit food choices and affect mealtime.
Mobility: Orthostatic intolerance and fatigue can impair the ability to move around independently.
Instrumental Activities of Daily Living (IADLs):
Cooking: Standing for extended periods may become difficult, requiring adaptations in meal preparation.
Shopping: Orthostatic intolerance can make walking around a store challenging.
Finances: Cognitive symptoms like "brain fog" may affect budgeting and bill payment.
Driving: Dizziness and poor concentration may impair the ability to drive safely.
Communication: Fatigue and cognitive impairments could impact meaningful communication.
Work-Related Activities:
Sustained Attention: Brain fog and fatigue may reduce focus and productivity.
Physical Tasks: Exercise intolerance and muscle weakness could impact the ability to perform job duties.
Social Interaction: Symptoms may limit the capacity for effective interpersonal communication.
Recreational and Social Activities:
Exercise: Physical symptoms may limit the type and duration of exercise, which could have social implications.
Hobbies: Symptoms may interfere with the enjoyment of hobbies that require sustained attention or physical activity.
Social Gatherings: The need for rest or symptom management may limit social participation.
Family Life:
Parenting: Fatigue and physical limitations may restrict active involvement in childcare.
Relationship Maintenance: Symptoms could impose strain on relationships with family members.
Emotional and Mental Health:
Coping: Chronic symptoms may lead to emotional stress or exacerbate mental health conditions like anxiety and depression.
Medical Gaslighting: Experiences of not being believed by medical professionals can lead to trauma, further complicating emotional well-being.
Occupational Therapy Approaches & Interventions
Many individuals living with dysautonomia become quite debilitated, presenting with decreased ADLs & IADLs, decreased ability to stand or maintain an upright position, and decreased strength. Exercise intolerance is common and co-existing mental health conditions.
SAFETY: It is important to have baseline information (such as resting heart rate and blood pressure) and it should be noted that exercise is contra-indicated in cases where POTS is comorbid with ME/CFS and long-COVID.
Evidenced Based Approaches:
Levine Protocol: This protocol focuses on increasing blood volume and improving autonomic function through pharmacological means and lifestyle changes. OTs often collaborate with medical teams to integrate this into a holistic care plan, enhancing medication adherence and lifestyle modification.
CHOP Protocol: The Children’s Hospital of Philadelphia (CHOP) protocol involves graded exercise to build stamina and manage fatigue. Occupational therapists tailor this to individual needs, focusing on gradual physical conditioning without exacerbating symptoms.
Polyvagal Theory (Safe and Sound Protocol): This theory aids in understanding the body's autonomic responses. OTs use this framework to teach coping mechanisms such as grounding techniques and deep breathing, aiming to regulate the nervous system and improve engagement in daily tasks.
Trauma-Informed Approaches: Given the overlap between trauma and chronic conditions like dysautonomia, trauma-informed care is integrated into OT practice. Strategies may include mindfulness, somatic experiencing, and cognitive restructuring to address underlying emotional and psychological factors.
Biofeedback and Neurofeedback: These interventions involve real-time monitoring of physiological functions like heart rate and brain waves. Occupational therapists use this data to teach individuals how to gain voluntary control over these functions. For dysautonomia patients, this can be particularly useful for regulating autonomic responses and reducing symptoms like tachycardia or anxiety.
Lifestyle Redesign (requires certification):Lifestyle Redesign® is a type of occupational therapy that guides you through the process of creating habits and routines that are personally meaningful and health promoting. This approach involves - sleep hygiene, pacing, energy conservation, spoon theory, coping skills training, and so much more!
Specific OT Interventions:
Proprioceptive/body awareness sensory input-
Proper body mechanics and ergonomics- Postural Training: For individuals with orthostatic intolerance, OT can provide strategies for slowly transitioning from a supine to an upright position, reducing symptoms like dizziness or fainting. Strengthening of lower extremities is encouraged
Activity Analysis and Adaptation: OTs assess how dysautonomia impacts daily activities and adapt these tasks to make them more manageable, thereby enabling participation in meaningful occupations.
Energy Conservation: Given the fatigue often associated with dysautonomia, energy conservation techniques like pacing are essential. OTs teach strategies to manage energy throughout the day, improving participation in daily life.
Cognitive Behavioral Strategies: OTs can use CBT techniques to help individuals identify and challenge negative thought patterns that exacerbate symptoms of anxiety or depression often seen in dysautonomia.
Mindfulness-Based Stress Reduction (MBSR): MBSR can be useful for teaching clients how to be present and reduce stress, which can, in turn, affect physical symptoms.
Environmental Modifications: Recommending alterations to the home or work environment can improve physical safety and reduce stress.
Social Skills Training: Socializing can be hard when dealing with chronic conditions. OTs can help improve communication and social interaction skills, reducing isolation.
Pain Management Techniques: Introducing non-pharmacological pain management strategies like heat/cold therapy or guided imagery can help in dealing with chronic pain often seen alongside dysautonomia.
Community Reintegration: For those who have been significantly affected by their symptoms, OTs can assist in facilitating a return to community activities, volunteering, or work, thereby improving mental well-being.
Monitoring: Keeping track of physiological responses during exercises (e.g., heart rate, blood pressure) can help tailor the exercise protocol to individual needs.
Education on Safe Exercise: Individuals may require education on how to exercise safely without triggering their symptoms, as well as when to rest or modify their activities.
Lifestyle Adaptations: Helping clients integrate regular exercise into their daily routines in a sustainable way can contribute to long-term symptom management.
Adaptive Equipment
Compression Garments - The use of abdominal and lower extremity compression garments can be part of a guided protocol to improve blood flow and reduce symptoms. Recommend a physicians and PT input
Self Management APPS: Tachymon, Cardiogram, flaredown, and routine/habit tracking (Routines, Me+ etc)
Diet : (RECOMMEND NUTRITION/DIETICIAN IF POSSIBLE)
High-Sodium Diet: One common recommendation is increasing sodium intake to help retain fluids and increase blood volume, thereby improving orthostatic tolerance. This should be discussed with a healthcare provider.
Hydration: Staying adequately hydrated is essential. Special electrolyte-replenishing fluids may be recommended.
Small, Frequent Meals: Consuming smaller, more frequent meals can help prevent the post-meal blood flow shifts that can exacerbate symptoms.
Avoid Alcohol and Caffeine: These substances can have diuretic effects and contribute to dehydration.
Sleep:
Elevate Head of Bed: Elevating the head of the bed can sometimes reduce nighttime symptoms and improve sleep quality.
Consistent Sleep Schedule: A consistent sleep schedule can help regulate circadian rhythms, potentially impacting cardiovascular function and symptom presentation.
Summary
My personal journey with dysautonomia intricately links with my professional mission as an Occupational Therapist and CEO of mOTivate Minds. The challenges of living with hEDS and POTS have enriched my understanding of the complex tapestry that is chronic illness, blending seamlessly with my academic background and years of experience in the field. As someone who knows firsthand the tribulations of managing chronic conditions alongside the daily demands of raising a family, my approach at mOTivate Minds is uniquely nuanced and deeply compassionate. I understand the high stakes of symptom management in a busy life, and that informs each patient interaction and intervention plan. As I navigate my own health challenges, it continues to serve as a powerful reminder of the vital role Occupational Therapy plays in enhancing daily life for individuals facing similar struggles. Thank you for joining me on this journey to shine a light on Dysautonomia, not just as a medical professional but as someone who lives it every day.
Wanting More Resources?
For Healthcare Professionals
OT Potential 1 hour CEU on long haul/POTS
Emily Rich- another OT Specializing in POTS
For Patients Click Here”
https://thedysautonomiaproject.org/resources/
** Want to know more about how an Occupational Therapist can help? Click Here
REFERENCES
Dochod, J. R., & Grapczynski, C. A. (2019). Increasing functional abilities of people with postural orthostatic tachycardia syndrome through occupational therapy. The Open Journal of Occupational Therapy, 7(1). https://doi.org/10.15453/2168-6408.1522
Dochod, J. R. (2022). Enhancing health-related quality of life through occupational therapy: A case report of a client with postural orthostatic tachycardia syndrome. Occupational Therapy In Health Care, 36(3), 202–219. https://doi.org/10.1080/07380577.2022.2051218
Levine, D., Work, B., McDonald, S., Harty, N., Mabe, C., Powell, A., & Sanford, G. (2021). Occupational therapy interventions for clients with Ehlers-Danlos Syndrome (EDS) in the presence of postural orthostatic tachycardia syndrome (POTS). Occupational Therapy In Health Care, 36(3), 253–270. https://doi.org/10.1080/07380577.2021.1975200
Oetjen, L., Johannsen, A., Bean, J., Sim, L., Harrison, T., Tsai Owens, M., & Harbeck-Weber, C. (2022). The goals and outcomes of adolescent and young adults with POTS attending an intensive interdisciplinary treatment program. Occupational Therapy In Health Care, 1–15. https://doi.org/10.1080/07380577.2022.2116666
Rich, E. M., Vas, A., Boyette, V., & Hollingsworth, C. (2020). Daily life experiences: Challenges, strategies, and implications for therapy in postural tachycardia syndrome (POTS). Occupational Therapy In Health Care, 36(3), 306–323. https://doi.org/10.1080/07380577.2020.1824303
