Functional status in postural tachycardia syndrome

Participants

We recruited participants via an electronic link on the Dysautonomia International Facebook page over 10 days. Dysautonomia International is a non-profit organization with a worldwide reach; well-respected autonomic researchers utilize it as a source for research recruitment (Shaw et al., 2019). All participants gave electronic informed consent before inclusion in the study. Participants did not receive compensation for their time or efforts. Participants were required to be between ages 18 and 60 and be diagnosed with POTS by a medical doctor. Exclusion occurred if an individual responded as a family member or friend of a person with POTS or outside the stated age requirements.

Design

The present study adopted a survey design with quantitative and qualitative components. The survey contained sections as described throughout this manuscript to include a quantitative single-item functional status question described below and the impact of symptoms on daily life in the month prior by using “select all that apply” from the provided domains. Participants reported cognitive status on a single question regarding the experience of challenges with “thinking” in the week prior. Falls were recorded across the year before the survey, using a single item yes/no response followed by a question regarding the need for medical intervention in those that answered “yes.” Additionally, self-management questions included two topics: (a) strategies with prioritizing the top three most helpful and (b) tools with multiple-select options for use in the month prior. Finally, assessment of therapy experience included a multiple-choice question regarding a history (unlimited time) of receiving occupational therapy, physical therapy, both, or neither, for treatment of POTS symptoms. The authors did not examine patients’ clinical records, rather explored individuals’ perceptions of their lived experience. These individuals’ lived experience is valuable for researchers and clinicians to identify existing strategies found to be successful in addition to understanding areas of focus for treatment and future research.

Procedure

We conducted the survey using the secure web-based platform, PsychData. This study received exception through the Institutional Review Board at the lead university on the study, protocol #20473. Demographic information collected in the survey included age, gender, education status, ethnicity, and employment. Survey questions on the functional domains consisted of multiple-select checkboxes, Likert scales, and multiple-choice questions. A prefacing statement instructed participants to answer all POTS symptom questions to the fullest extent possible, limiting the focus of co-morbid conditions.

Linear analog scale of function

One question included a linear analog scale that asked participants “on a scale from 1 to 10, where 1 is not able to do anything in daily life and 10 can do everything you want to do, where would you rate your ability to do the things you want/need to do in daily life?” Participants then responded on a scale with language as follows: “1: I cannot do anything I want or need to do” and “10: I can do everything I want or need to do.” This was a standalone rating and participants were not specifically instructed to compare to prior level of function. Researchers have used these types of rating scales as patient self-assessments to determine the self-perceived level of functioning and allow individuals to communicate the overall quality of life with care providers and researchers (Locke et al., 2007).

Identification of occupational domains

In conjunction with the Occupational Therapy Practice Framework, the pilot study led to focus on the representative domains of occupational performance and excluded those that were not reported to be commonly impacted (e.g., dressing) in this population (American Occupational Therapy Association, 2020; Rich et al., 2018). Additionally, functional tasks identified as frequently challenging due to POTS (e.g., getting out of bed in the morning, morning routine) were grouped using relevant and easy-to-understand language to ensure applicability and comprehension. We operationalized morning routine as “tooth brushing, dressing, cooking/eating breakfast, styling hair, and/or applying makeup.” Functional mobility included “getting around during the day, climbing stairs, and/or wheelchair mobility,” with “falls” defined as “coming to rest inadvertently on the ground or floor or other lower level (World Health Organization, 2018).” In response to this previous work and the occupational therapy framework, we identified the following occupational domains with specific categories, which can be found in Figures 2–4: BADLs, IADLs, work and school, rest and sleep, leisure and play, and social participation (American Occupational Therapy Association, 2020; Rich et al., 2018).

Self-management

The options for self-management, including both the strategies and the tools questions, were determined through response to the pilot study (Rich et al., 2018). We designed the pilot study options for strategies utilizing best-evidence recommendations for managing the condition in journal articles, from patient support groups, and through input from patients who reviewed the survey. Tools were determined similarly, incorporating most frequent recommendations from expert providers in the literature and patient feedback. In the present study, the authors included the most commonly chosen strategies and tools from the pilot study.

Functional status

When given a single-item numerical linear analog self-assessment, 61.3% of individuals reported their current level of function to be at 5/10 or less (M = 4.87, mode = 3) (Figure 1) (n = 922).OPEN IN VIEWER

Impact of symptoms on daily life

An overwhelming majority (98.5%) of participants reported that POTS negatively impacted their daily life throughout the month prior to completing survey (n = 958). When all areas of life that were impacted by POTS symptoms were summed per participant, out of a total number of 18 potential areas of daily life, on average there were 11 areas of daily life that were impacted by POTS symptoms (M = 11.03±4.18, range: 0–18). More than 90% of the sample identified five or more areas of life affected (91.2%), and 33% of participants identified 13 or more areas. The basic ADL (BADL) most frequently cited was bathing or showering (79.2%), followed by getting out of bed in the morning (74.6%) (Figure 2). The most common instrumental ADL (IADL) affected was home management (82.2%), followed by shopping (76.8%) and meal preparation and cleanup (75.1%) (Figure 3). While a majority of participants (69.9%) reported impacts to either work or school, 30.1% identified impairment in both domains (Figure 4).OPEN IN VIEWER

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Figure 3.

Percentage of individuals negatively impacted by postural tachycardia syndrome in their IADLs. These domains do not sum to 100% because “check all that apply” was asked for these questions. POTS: postural tachycardia syndrome; IADL: instrumental activities of daily living.

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Figure 4.

Percentage of individuals negatively impacted by postural tachycardia syndrome in additional areas of life. These domains do not sum to 100% because “check all that apply” was asked for these questions.

Cognitive status

A total of 93.4% of participants reported experiencing “challenges with thinking and/or mental clouding/fogging” in the week leading up to taking the survey (Table 2) (n = 958). A week was chosen as the timeframe to allow more specificity in quantifying incidence and increase accuracy with a more recent timeframe.

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Table 2.

Percentage of individuals reporting challenges with thinking in the past week.

Response	n	%
Yes	895	93.4
No	63	6.6

Falls

In full, 55.3% of participants reported a fall in the past year. A year timeframe was chosen as we anticipated falls to be less frequently occurring; additionally, this is a frequent measure in other populations to serve as a comparison value. Of those who had fallen, a quarter of these individuals required medical attention.

Self-management

Participants also chose from various strategies (e.g., sodium and fluid intake, medications, exercise, compression garments, dietary changes, adaptive equipment, breathing/meditation, and elevating the head of the bed) that they utilized to alleviate POTS symptoms (Table 3).

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Table 3.

Percentage of participants who identified a strategy as one of their top three most helpful strategies in managing symptoms of postural tachycardia syndrome (n = 952). These domains do not sum to 100% because participants were asked to choose three.

Strategy	n	%
Increase salt and fluid	730	76.7
Medications	595	62.5
Exercise	334	35.1
Compression socks/stockings	301	31.6
None of the remaining options were helpful, or have not tried them	258	27.1
Dietary changes	194	20.4
Adaptive equipment	113	11.9
Breathing and/or meditation	107	11.2
Elevating the head of my bed	68	7.1

Separately, patients reported utilizing tools and devices as compensation (e.g., chair to sit, shower chair, and mobility devices). Participants identified multiple items of durable medical equipment and adaptive strategies in the month preceding survey completion, with 10.4% of sample participants indicating no use of adaptive tools, 43.4% reporting use of one to two adaptive tools, and 46.3% utilizing at least three or more (Table 4).

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Table 4.

Percentage of participants reporting use of adaptive tools (either one or multiple tools) in managing symptoms of postural tachycardia syndrome (n = 958). These domains do not sum to 100% because “check all that apply” was asked for these questions.

Adaptive tools	n	%
Chair to sit while getting ready	623	68.8
Compression	609	67.3
Shower chair/tub bench	372	41.4
Cooling devices	174	19.2
Manual wheelchair	133	14.7
None	58	10.5
Cane	91	10.1
Long-handled brush/sponge	86	9.5
Electric cart or scooter	77	8.5
Service dog	57	6.3
Reacher	52	5.7
Rollator	42	4.6
Walker	39	4.3
Power wheelchair	30	3.3
Crutches	18	2.0
Long-handled shoe horn	16	1.8

Therapy

Per report, 26.9% of the study sample received occupational and/or physical therapy to treat their POTS symptoms at some point in time. Physical therapy was more highly utilized (25.9%) than occupational therapy (5.5%). A small number received both therapy services (4.4%) (n = 958).

Functional status

There is limited documentation of patients’ perceived level of impairment and follow-through on using a variety of interventions and compensatory strategies. This relatively large sample reported substantial impairment in functionality. Symptoms limit more than one-third of participants from working or attending school, which further demonstrates a significant loss of functionality. This level of work and school limitation is slightly higher but relatively consistent with other studies (Benrud-Larson et al., 2002).

Impact of symptoms on daily life

Previous studies have not included the specific domains of daily activity most highly impacted by POTS. However, reports of deficits are almost universally present (98.5%). Perhaps most striking in the study findings was the impact frequency of IADLs (home management, shopping, meal preparation, and driving) and often-overlooked areas of socialization and leisure activities. In isolation or comprehensively, these activities likely contribute an underappreciated amount to a decrease in quality of life. Studies less commonly cite these domains in the literature and discussion of interventions to address these functional deficits is almost non-existent in this population. Patients may feel less compelled to share their challenges with these under-recognized areas due to a fear of stigma, the underappreciated necessity of these skills for quality of life, and/or limited time in providers’ offices to open a broader dialogue. Regardless, it is crucial to notice the incidence of these challenges with IADLs, socialization, and leisure.

It is worthwhile to emphasize that despite the relatively low-frequency reports of impact on child-rearing (20.8%), religious/spiritual activities (31.4%), and school (38.5%), these areas were not applicable for all participants. Therefore, influences on these other activities are comparable to BADLs in the segment of the population affected (e.g., parents, individuals with faith, and students). Similarly, the results regarding the impact of symptoms on work may be underrepresented as a portion of the study sample may not be pursuing work endeavors due to age or stage in life. Even so, these data are relevant in directing health care providers to address commonly reported areas of impact while taking roles and routines into consideration.

Cognitive status

The vast majority (93.5%) of the sample reported cognitive impairment. Known areas of impairment in this population include attention, executive function, processing speed, and memory, with frequent reports of brain fog, forgetfulness, challenges with word-finding, and difficulty thinking (Raj et al., 2018). These deficit areas are not unlike what is observed in patients with other central nervous system disorders, occurring in POTS approximately 1.3–2 times as often as outcomes on neuropsychology batteries in multiple sclerosis and Parkinson disease (Benedict et al., 2006; Sumowski et al., 2018; Yang et al., 2016). Note that these reports are based on objective outcomes from standardized assessments, instead of patient-reported perception of impairment, as was used in this study. In a self-reported study of 3108 cancer survivors following treatment, the incidence of cognitive dysfunction was half of that reported in the present study, with similar impairment (Schmidt et al., 2016). Additional research is needed to identify the objective prevalence of cognitive impairment in individuals with POTS; however, from a clinical standpoint, a patient’s self-perception of impairment is often meaningful for understanding patient needs and experience of their condition. These cognitive challenges likely impact socializing with friends and family, work responsibilities, school, driving, and financial management among other tasks. BADL limitations, while less directly impacted by cognitive impairments, are still clinically reported by patients as “fogginess” exacerbated by positional changes or actions such as standing in a hot shower (Ross et al., 2013). Patients report cognitive impairment as one of the most debilitating symptoms of the condition with a known adverse impact on the quality of life (Raj et al., 2018). Although participants self-reported cognitive challenges are extremely valuable, objective standardized performance-based characterization of cognitive domains could help generalize the findings and more importantly address strategies to mitigate them. Further research is necessary to understand better the mechanism of cognitive impairment, and potential treatment modalities, given the breadth of resulting functional deficits for the patients.

Falls

Not only do symptoms limit participation in daily life, but in this study, over half of the participants (55.3%) reported falling in the past year, and a quarter of those falls required medical attention. Most concerning about these falls are the possibilities of head injuries, which may further worsen symptoms. The CDC reports that one in five falls results in serious injury, and falls contributed to more than US$50 billion in medical costs in 2015 (Centers for Disease Control and Prevention, 2019). Commonly reported symptoms in patients with POTS—lower body weakness, difficulty with walking and balance, and vision problems—are frequently cited as risk factors for a fall (“Important Facts about Falls,” 2017). The environment and circumstances around these falls are unclear, limiting the understanding of the cause. Cognitive impairment in patients with more severe deficits may lead to more falls. We would expect a portion of falls to occur in the shower due to static standing in a warm environment, taking into account the mechanism of orthostatic intolerance and anecdotal evidence. Less than half of participants reported use of a shower chair or tub bench; this could be due to various factors including the stigma of using medical equipment, lack of knowledge regarding equipment, restricted financial means, and/or poor recognition of symptom onset and need to sit. Due to the nature of orthostatic symptoms, falls are likely to occur in positions of static standing (e.g., waiting in lines or shopping), moving from a position of recumbency to standing (e.g., getting out of bed or rising from chair/floor), and/or with increased exertion (e.g., exercise or climbing stairs). The use of durable medical equipment such as a shower chair or mobility device may play a role in decreasing additional falls. Per the current study, the cause of falls and the relationship between age and falls is unclear; longitudinal follow up is indicated to better understand this issue.

Self-management

Participants self-identified increased salt and fluid intake as the most effective method for intervention. As this is a thoroughly documented, non-invasive, and well-tolerated first-line treatment in the POTS management, it is not surprising that it was highly cited by the participants. However, it is unclear if this remedy for symptom-reduction results in sustained and impactful improvement, given that most patients continue to suffer from ongoing impairment in function. The specific reason for patients most frequently citing proper fluid balance as efficacious is not concrete, as they may not have tried other strategies, may not have tolerated other methods or adverse side effects, or may truly have not experienced as positive of an effect from other interventions. Additional research is needed to understand the exact prevalence of best outcomes using different approaches.

In a recent survey-based study, nearly one-third of participants with POTS who reported symptom improvement attributed medications as the primary factor (Shaw et al., 2019). In 52% of participants, non-pharmacologic intervention offers some improvement (Shaw et al., 2019). Interventions, both pharmacologic and non-pharmacologic, are limited, and while many individuals may see clinical improvement, most will not be “cured” (Arnold et al., 2018). This ongoing presentation of symptoms highlights the need for specific, targeted intervention to improve patient functionality during valued and meaningful activities.

Therapy

The results showcase the underutilization of occupational and physical therapies, with approximately one quarter receiving one or both services, despite previously reported levels of functional impairment. The reason behind the limited utilization of therapy within the patient population remains unclear. We postulate that this may be a result of multiple factors including (a) lack of research and evidence-based studies in rehabilitation and POTS, (b) knowledge gap for clinicians and absence of instruction on POTS in educational programs, (c) absence of clinical guidelines for therapists, (d) limited awareness of rehabilitation services, (e) adverse experiences in patients who have received therapy, and/or (f) lack of referrals from medical providers.

The average length of time from onset of symptoms until diagnosis is presently 4.7 years (Shaw et al., 2019). Increased awareness of POTS diagnosis and treatment in the medical community is imperative. If physicians and medical staff recognize symptoms of POTS and refer to knowledgeable providers for additional diagnostic work-up, patients have potential for acquiring new treatment strategies. The need for increased awareness of POTS symptomatology and treatment among rehabilitation therapists is less commonly discussed by researchers but is worth addressing due to the potential benefit for this patient population.