Introduction to POTS (Postural Orthostatic Tachycardia Syndrome)
POTS (Postural Orthostatic Tachycardia Syndrome) is a common co-morbidity seen in patients that are also enrolled in chronic pain rehabilitation1, specifically with chronic headaches.2 It is also a common secondary diagnosis to other chronic pain diagnoses due to prolonged de-conditioning. In our clinical experience, physical and occupational therapy treatment may require only slight alterations compared to other chronic pain diagnoses. Patients with POTS can tolerate gradual exercise training as a way to manage syncope and orthostatic tolerance. This article highlights the potential for adolescents with POTS to tolerate and participate in more rigorous, short term rehabilitation programs. In addition, suggestions for physical and occupational therapy treatment progressions and appropriate supporting evidence will be provided.
POTS is currently defined as an increase in heart rate by 30 beats per minute or over 120 bpm within 10 minutes of standing however is not due to orthostatic hypotension.3 It has been suggested that diagnosis criteria for adults and children should be different and that criteria specifically for children and adolescents does not exist yet. Using the current criteria, of 30 beats per minute, it seems that there is a trend for over diagnosis of POTS specifically in children and adolescents,4 which may be leading to the increased frequency of clinicians seeing patients with this as a diagnosis. There are many pharmacological interventions that may be recommended for individuals with POTS in addition to physical and occupational therapies.
Potential barriers for these individuals during participation of physical activities include drops in blood pressure, dizziness, fatigue, heart palpitations, nausea, and tremulousness3. In some cases patients experience more extreme symptoms including syncope with minimal physical activity or simple transitions which may make it feel hazardous to participate in therapy sessions or their suggested home program. When working with these patients, it is important to have baseline information (such as resting heart rate and blood pressure), as well as other helpful information, such as anxiety and depression, prior to initiating physical activity. This helps to put symptoms they may experience into context so you can reassure the patient (i.e “your resting heart rate is typically 110 bpm, while you road the stationary bike it only increased to 140 bpm which is normal” or “you told me you were worried about trying jumping activities, I noticed your baseline heart rate was elevated before we even started the activity, let’s try to use controlled breathing before we start the next set”).
Exercises and increased hydration as a part of POTS treatment has been recommended by research6–11 however some additional supportive tips may be helpful for clinicians and teams as they work with this patient population. Past research has been completed over a longer period of time (3 months to 1 year) allowing for a very gradual increase in physical activity.8,10,12,13 However, when participating in a standard intensive rehabilitation program often the time line is much shorter. In addition, improved outcomes (decreased symptoms, age appropriate functioning) will more likely be seen if you can more rapidly facilitate more typical functioning.
Specifically in this program, adolescents are seen intensively for 3 weeks, 5-6 days a week with 3-4 hours of physical and occupational therapy each day. Areas to focus on include: education and building body awareness and alignment, joint conservation skills, energy conservation skills, parent education about how slight increases in heart rate are safe and recommended, lower extremity and core strengthening, slow progression of endurance, higher level balance and higher impact skills.8,13
The recommended progression for use in therapies can be broken up into three phases, each with varying time lines based on the individual’s tolerance and participation. In the first phase focuses on background education, basic skill introduction, and foundational movement skills. The second phase incorporates more advanced education and activities, and the third phase focuses on endurance activities, return to “life”, and independence with skills. More thorough phase and treatment information can be viewed in appendix A.
The phase progression discussed is a general outline of the order in which we progress skills at our clinic taking into account past experiences and clinical changes. Clinically our staff avoids continuous or frequent monitoring of specific objective measures as this can lead to increased focus on symptoms however using heart rate to support treatment can be beneficial at times. More often staff monitors the patient using subjective observation (coloring of face, breath pattern, appropriate alignment and activation of musculature for task, etc) to avoid over attending and then provides encouragement about tolerance. Having discussions about how receptive or open patients are to advancing to the next phase is also very important with this population. Using the suggestions from this article, patients with POTS can tolerate gradual exercise training as a way to manage syncope and orthostatic tolerance. Symptoms can improve without increasing resting blood pressures and causing additional orthostatic symptoms if completed gradually and properly.20
Many participants will agree to lower impact physical activities but struggle to tolerate prolonged endurance, circuit training, and high impact activities which would facilitate quicker lower extremity strengthening, improved cardiovascular endurance, and are functionally relevant. So how do we promote tolerance of these more challenging tasks? During challenging physical activity, it is important to gradually progress skills to help facilitate self-confidence, allow for proper coping, and to ensure safety. By teaching the individual to appropriately work into, and tolerate, increased heart rate and other symptoms, they can better elicit muscle fatigue, therefore appropriately strength training and building stamina. It is helpful to provide examples and education about the importance of lower extremity strength training, specifically to encourage venous return14 to help maintain heart rate and blood pressure,6 while teaching the patient to become more mindful to facilitate progression of skills. Clinicians should use caution, at times a patient becoming more in tune with their body (more mindful) can lead to further focus on symptoms. To this end focusing on appropriate use of coping skills and normalizing symptoms to enhance confidence is important.
Educating patients about appropriate rest breaks can help to minimize symptoms and more appropriately utilize energy conservation strategies. As well as giving specific feedback about individualized signs and symptoms that should or should not be utilized as warning signs (i.e typical increase in heart rate vs significant increase in heart rate causing difficulty to breathe) or information regarding resting posture and how it can exacerbate or limit symptoms (i.e closing eyes or placing head in lap before returning to sitting position typically increases dizziness compared to sitting with eyes open and focused on a distant visual will help dizziness resolve). Also providing examples that pertain to each individual about completion of daily activities, attending school, or participating in sporting practices using poor energy or joint conservation strategies, they will most likely be working inefficiently, leading to exacerbated symptoms.
To further improve the quality of treatment sessions, make sure to include education about everything including the purpose of activities, what they are do, why they are important, and how each will translate to improved functioning. Stay focused on patient goals so that they stay motivated and are better able to cope and manage possible anxiety. Provide parent education often, have them observe the skills you are working on, rational, and importance of them continuing these skills at home. In addition, providing education to patients, their families, and other team members (physicians, nurses, teachers, and coaches) can facilitate more consistent physical activity and conditioning.
It is important to discuss that patients with a diagnosis of POTS can tolerate and benefit from participation in an intensive rehabilitation program, however there are many components that may facilitate or inhibit gains. An appropriate exercise progression, verbal education about key aspects of physical activity, and allowing the adolescent to help make progression decisions is important. In addition, looking at perception of physical gains and abilities, along with actual physical function is important as both serve different purposes and are separate constructs in the rehabilitation process.15 With the incorporation of education with these patients, including concepts to help slowly increase activity level, tolerate increased symptoms while maintaining activity level, and better ability to pace, participants have demonstrated improved physical abilities after their intensive rehabilitation program participation. Using the suggestions from this article and other supporting literature, patients with POTS can tolerate various exercise training regimens as a way to manage syncope and orthostatic tolerance. Symptoms can improve without increasing resting blood pressures and causing additional orthostatic symptoms if completed gradually and properly.20
- Kizilbash S., Ahrens S. et al. Adolescent Fatigue, POTS, and Recovery: A Guide for Clinicians- ClinicalKey. Curr Probl Pediatr Adolesc Health Care. 2014;44(5):108-133. https://www.clinicalkey.com/#!/content/playContent/1-s2.0-S153854421400011X?returnurl=null&referrer=null. Accessed December 28, 2017.
- Mohr LD. A Case Report and Review of Postural Orthostatic Syndrome in an Adolescent- ClinicalKey. J Pediatr Heal Care. 2017;31(6). https://www.clinicalkey.com/#!/content/playContent/1-s2.0-S0891524516304382?returnurl=null&referrer=null. Accessed December 28, 2017.
- Dysautonomia International: Postural Orthostatic Tachycardia Syndrome. http://www.dysautonomiainternational.org/page.php?ID=30. Published 2012. Accessed March 22, 2017.
- Singer W, Sletten DM, Opfer-Gehrking TL, et al. Postural tachycardia in children and adolescents: what is abnormal? J Pediatr. 2012;160(2):222-226. doi:10.1016/j.jpeds.2011.08.054.
- Stewart JM. Autonomic Nervous System Dysfunction in Adolescents with Postural Orthostatic Tachycardia Syndrome and Chronic Fatigue Syndrome Is Characterized by Attenuated Vagal Baroreflex and Potentiated Sympathetic Vasomotion. Pediatr Res. 2000;48(2):218-226. doi:10.1203/00006450-200008000-00016.
- Michael J. Joyner. Exercise Training in Postural Orthostatic Tachycardia. Hypertension. 2011;58:136-137. doi:10.1016/S0140-6736(11)60096-2.
- Lorna Nicholson. PoTS – Postural Tachycardia Syndrome. http://www.potsuk.org/exercise. Published 2015. Accessed March 22, 2017.
- Winker R; Barth A; Bidmon D; Ponocny I; Weber M; Mayr O; Robertson D; Diedrich A; Maier R; Pilger A; Haber P; Rüdiger HW. Endurance exercise training in orthostatic intolerance: a randomized, controlled trial. Hypertension. 2005;45(3):391-398. http://www.medscape.com/medline/abstract/15699447. Accessed March 22, 2017.
- Fu Q, VanGundy TB, Shibata S, Auchus RJ, Williams GH, Levine BD. Exercise Training Versus Propranolol in the Treatment of the Postural Orthostatic Tachycardia Syndrome. Hypertension. 2011;58(2):167-175. doi:10.1161/HYPERTENSIONAHA.111.172262.
- White PD, Goldsmith KA, Johnson AL, et al. Comparison of adaptive pacing therapy, cognitive behaviour therapy, graded exercise therapy, and specialist medical care for chronic fatigue syndrome (PACE): a randomised trial. Lancet (London, England). 2011;377(9768):823-836. doi:10.1016/S0140-6736(11)60096-2.
- Mtinangi BL, Hainsworth R. Increased orthostatic tolerance following moderate exercise training in patients with unexplained syncope. Heart. 1998;80(6):596-600. doi:10.1136/hrt.80.6.596.
- Fu Q, Levine BD. Exercise in the postural orthostatic tachycardia syndrome. Auton Neurosci. 2015;188:86-89. doi:10.1016/j.autneu.2014.11.008.
- Brilla LR, Stephens AB, Knutzen KM, Caine D. Effect of Strength Training on Orthostatic Hypotension in Older Adults. J Cardiopulm Rehabil. 1998;18(4):295-300. doi:10.1097/00008483-199807000-00007.
- Goodman CC, Helgeson K. Exercise Prescription for Medical Conditions : Handbook for Physical Therapists. F.A. Davis Co; 2011.
- Kempert H, Benore E, Heines R. Physical and occupational therapy outcomes: Adolescents’ change in functional abilities using objective measures and self-report. Scand J Pain. 2017;14(14):60-66. doi:10.1016/j.sjpain.2016.10.004.
- Serafim THS, Tognato AC, Nakamura PM, et al. Development of the Color Scale of Perceived Exertion: Preliminary Validation. Percept Mot Skills. 2014;119(3):884-900. doi:10.2466/27.06.PMS.119c28z5.
- Utter AC, Robertson RJ, Nieman DC, Kang J. Children’s OMNI Scale of Perceived Exertion: walking/running evaluation. Med Sci Sports Exerc. 2002;34(1):139-144. http://www.ncbi.nlm.nih.gov/pubmed/11782659. Accessed December 22, 2017.
- Szczygieł E, Blaut J, Zielonka-Pycka K, et al. The Impact of Deep Muscle Training on the Quality of Posture and Breathing. J Mot Behav. August 2017:1-9. doi:10.1080/00222895.2017.1327413.
- Bordoni B, Marelli F, Bordoni G. A review of analgesic and emotive breathing: a multidisciplinary approach. J Multidiscip Healthc. 2016;9:97. doi:10.2147/JMDH.S101208.
- Masuki S, Eisenach JH, Schrage WG, Johnson CP D, NM, Wilkins BW, Sandroni P, Low PA JM. Reduced stroke volume during exercise in postural tachycardia syndrome. J Appl Physiol. 2007;103:1128-1135.
Stretching/flexibility training: Utilized to ensure that the patient has the appropriate muscle length to support and protect the body during functional mobility and strengthening activities.
Basic breath training: Educate the patient how improper breathing can exacerbate symptoms, demonstrate how appropriate use of exhale and inhale can support activities and limit symptoms. Have them use ability to manage breathing as a way to continue to discontinue activities.
Basic education and use of coping skills: Introduce basic coping skills that are helpful both during physical activities and at rest. Help the patient understand how to utilize skills with specific practice. Basic skills include: breathing, distraction, problem solving, imagery, acupressure, and progressive muscle relaxation
Strengthening: Isolated strengthening for specific muscles to support and stabilize, especially with focus on lower limb resistance training and joint stabilization, and to improve comfort with movement and confidence with more challenging activities in the next phase.
Transition training: Specific education is provided on how improper form can make transitions more challenging to tolerate and how improved technique can facilitate improved participate with decreased symptoms. Focus on basic transitions such as floor to stand, sit to stand, supine to sit, and turning/pivoting; which are all commonly transitions that cause an increase in symptoms per patient reports clinically.
Lower impact exercise: Focus on low impact exercises to initiate endurance training, such as aquatics, leg or arm biking, pilates, isometric training, or yoga, for short intervals with focus on proper form and pace. Activity intervals are increased each time it is trialed in sessions.
Body awareness: Specific focus on improving proprioception, coordination, and motor planning can enhance comfort and compliance with activities outside of sessions which in turn helps with consistency. Therapists are careful to provide positive feedback more often than negative feedback in regards to body awareness, technique, and coordination.
Energy and joint conservation strategies: A large emphasis is placed on appropriate utilization of energy, as well as improved joint conservation via alignment to enhance energy conservation. With this population the use of extension patterns and end range are common and only seem to exacerbate symptoms with functional mobility and exercise. Education is individualized to ensure improved compliance with habit management and ability to implement strategies for daily activities.
Goal setting: It is very important that patients with POTS can identify specific activities are they motivated to return to and at what level. Without motivating tasks it can make rehabilitation therapies challenging to endure. Consistently bringing the focus on how challenging activity completion will lead to successful completion of their goals can facilitate participation (i.e working on jumping in pool setting is necessary before they can work on jumping on land for return to dance).
Phase 2 (Continue working on phase 1 activities and add the following)
Initiate advanced or challenging activities: Focus on how to change or modify activities to either make basic activities more challenging or hard activities easier to tolerate. Specific areas of activity they can focus on is changing the pace, amount of repetitions, positioning, amount of impact, etc. It is also suggestions that they utilize the rate of perceived exertion scale (RPE)16, or the OMNI-RPE visual scale17, but instead of monitoring heart rate they focus on respiratory rate and muscle fatigue in order to figure out an appropriate level of activity (working between a 7-8/10 at the most).
Focus on breathing technique: Once basic activities can be tolerated it is helpful to focus on appropriate breathing as often patients with POTS avoid using diaphraghmatic breathing18,19, especially exhalation when experiencing increased symptoms.
Work through symptoms: Specific focus is on appropriate “symptoms” to acknowledge and to what extent. Education focuses on the importance of recognizing muscle fatigue, alignment, and proper pace with patients paying less attention to elevated heard rate, dizziness, or other associated symptoms. The staff does review what type of symptoms they should pay attention too which can include: new symptoms, inability to maintain safety, or dramatic increase in heart rate.
In this phase it is important to continue discussion on pacing, appropriate activity modifications, and how to choose appropriate activities in general to prepare for phase 3. Time is spent discussing pre-planning activities, having pre designated rest breaks, and plans for how to manage increased symptoms.
Phase 3 (Continue working on phase 1 and 2 activities and add the following)
Continue to progress physical activity: Begin to specifically incorporate skills that they will need to complete once therapy is decreased or they are discharged including return to gym, sports, recreational activities, and home program utilization.
Endurance: Focus on endurance (muscular and cardiovascular) to facilitate tolerance to functional mobility, sports, and life activities. Provide progression plans so that the patient can begin to complete endurance activities in the home environment without increased symptoms such as stationary bike plans or a jogging progression.
Continue working on challenging and higher level skills: If applicable, this is when you can and should introduce return to running and jumping activities. Ensure that you guide the patient to only change one component of the activity at a time (more reps, longer intervals, different terrain, etc) to ensure appropriate pacing and management of symptoms. In addition, it makes it easier to identify specific areas that the patient should continue to limit for now (i.e if they can do more reps without an increase in symptoms but cannot tolerate activities combining upper and lower extremities then they can do rep work as part of their home program and the clinician can continue working on more challenging activities in therapy sessions).
Focus on specific goals and home going planning:
- Work on independence with all skills and concepts learned so far
- Can they independently modify activities?
- Are they able to take appropriate rest breaks?
- Can they independently identify muscle fatigue?
- Are they able to push themselves appropriately?