Article. Keywords postural orthostatic tachycardia syndrome, interdisciplinary treatment, adolescents

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1 638663CPJXXX / Clinical PediatricsBruce et al research-article2016 Article Improvement in Functioning and Psychological Distress in Adolescents With Postural Orthostatic Tachycardia Syndrome Following Interdisciplinary Treatment Clinical Pediatrics 2016, Vol. 55(14) The Author(s) 2016 Reprints and permissions: sagepub.com/journalspermissions.nav DOI: / cpj.sagepub.com Barbara K. Bruce, PhD 1, Tracy E. Harrison, MD 1, Susan M. Bee, CNS 1, Connie A. Luedtke, RN 1, Co-Burn J. Porter, MD 1, Philip R. Fischer, MD 1, Sarah E. Hayes 1, Daniel A. Allman 1, Chelsea M. Ale, PhD 1, and Karen E. Weiss, PhD 1 Abstract Significant functional impairment and psychological distress have been observed in adolescent patients with postural orthostatic tachycardia syndrome (POTS). Interdisciplinary rehabilitation programs have been shown to be beneficial in the treatment of chronic pain in adults and adolescents. Only preliminary data have examined interdisciplinary rehabilitation efforts in patients with POTS. This study evaluated the impact of an interdisciplinary rehabilitation program on the functional impairment and psychological distress in 33 adolescents diagnosed with POTS. Patients included in the study were adolescents ages 11 to 18 diagnosed with POTS. Measures completed at admission and discharge from the program included the Functional Disability Index, Center for Epidemiological Studies Depression Child scale, and the Pain Catastrophizing Scale for Children. After participation in the 3-week program, adolescents with POTS demonstrated a significant increase in overall functional ability and significant reductions in depression and catastrophizing. Keywords postural orthostatic tachycardia syndrome, interdisciplinary treatment, adolescents Postural orthostatic tachycardia syndrome (POTS) is a clinical syndrome defined by the presence of excessive tachycardia after assuming an upright posture. 1 Associated symptoms can include lightheadedness, dizziness, and tremulousness; presyncope; nausea; fatigue; exercise intolerance; weakness; hyperpnea or dyspnea; palpitations; sweating; anxiety; and sleep disorders. 2-5 Although the prevalence of POTS in adolescents is not known, it has been estimated that up to patients between the ages of 15 and 50 in the United States have POTS. 6,7 Quality of life in these patients can be significantly affected, and the functional disability observed in these patients can be severe. 8,9 Patients may have impairment in activities of daily living such as eating or showering, be unable to attend school or work, and even lowintensity exercise may profoundly exacerbate orthostatic symptoms It has been estimated that a quarter of the adult patients diagnosed with POTS are unable to work and are disabled. 14 Similar impairments in functioning have been observed in adolescents. 15 Kritzberger and colleagues found depression to be strongly associated with functional disability in a sample of adolescents with POTS and chronic pain symptoms. 16 Symptoms of anxiety and depression are common in these patients as well as sleep difficulties 17,18 and brain fog. 19 Somatic hypervigilance can develop as well as the avoidance of activities that can trigger symptoms such as exercise. Cognitive-behavioral interventions have been suggested as more likely to be of benefit in these difficult to treat patients. 20 Treatment of POTS has included pharmacological treatment that has focused on β-blockers, midodrine, 1 Mayo Clinic, Rochester, MN, USA Corresponding Author: Barbara K. Bruce, Department of Psychiatry and Psychology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Bruce.Barbara@Mayo.edu

2 Bruce et al 1301 and fludrocortisone ,21-23 Nonpharmacological interventions have included water and salt supplementation for suspected expansion of plasma volume, physical countermaneuvers, exercise, and the use of elastic support hosiery or compression garments. 4 Physical countermaneuvers such as contracting muscles below the waist can be used to improve orthostatic tolerance. 11,24,25 Burkhardt and colleagues concluded that the heart rate changes observed in adolescents with POTS were not due solely to deconditioning, despite the findings by some researchers that exercise can be effective in improving orthostatic symptoms or in some patients resolving symptoms of POTS. 12,26,27 Although several medications have been found to be helpful in treating the symptoms of POTS, many patients may not tolerate these medications or are left with symptoms that continue to result in impaired functioning and poor quality of life. An interdisciplinary approach has been recommended when activities of daily living remain compromised. 28 Interdisciplinary treatment has proven effective in increasing functioning and quality of life in patients with chronic illness/symptoms such as fibromyalgia, 29 chronic pain in adults, 30,31 and chronic pain in adolescents Despite recommendations that health care practitioners address not only the physical aspects of POTS but also the psychosocial implications, 5,8 such treatment efforts have not been examined for patients with POTS in either adolescent or adult populations. The present study evaluated the effectiveness of an interdisciplinary rehabilitation program in improving functional capacity and reducing psychological distress in adolescents with POTS. Methods Sample Thirty-three patients, ages 11 to 18, diagnosed with POTS who completed a 3-week pediatric rehabilitation program at a tertiary medical center served as participants in this study. Patients were considered to have POTS if they had symptoms of fatigue and orthostatic intolerance (such as dizziness and/or nausea) and a postural heart rate change of more than 30 beats per minute on 70 head-up tilt testing. The adolescents with POTS who participated in this study were recalcitrant to usual therapeutic measures. The institutional review board approved the study, all parents provided written informed consent, and children provided assent as appropriate. Measures Measures were completed at the time of admission and discharge from the 3-week program and included the Functional Disability Index, Center for Epidemiological Studies Depression Child version Scale (CESD-C), and the Pain Catastrophizing Scale for Children. Level of functioning was assessed with the Functional Disability Inventory. 36 It consists of 15 items that assess difficulties performing typical activities of daily living for children and adolescents such as ability to walk to the bathroom or walk the length of a football field or be at school all day. Scores range from 0 to 60, with higher numbers reflecting greater disability. Initial validation of the measure showed that a healthy control sample obtained a mean score of 3.5 on the Functional Disability Inventory. 37 A recent study derived a classification system for this measure that places scores into 3 categories of no/minimal disability (0-12), moderate disability (13-29), and severe disability (30 and over). 37 Depression was assessed with the CESD-C. 38 This scale is a measure of depressive symptoms in children and adolescents with acceptable reliability and validity. 39,40 It is a 20-item, self-report scale that ranges from 0 to 60, with higher scores suggesting more frequent and severe depressive symptoms. Pain catastrophizing was measured with the Pain Catastrophizing Scale for Children. 41 It is a 13-item selfreport questionnaire designed to measure an exaggerated negative response to pain. We have found that adolescents with POTS often feel similarly helpless in controlling their many symptoms and in their worry about the future. For this sample, pain was not the primary concern so the staff instructed the patients to replace the word pain in this questionnaire with the word symptoms. Items are scored from 0 (not at all) to 4 (extremely). Scores range from 0 to 52, with higher scores suggesting greater catastrophizing. Treatment Program The treatment program consisted of an intensive 3-week outpatient hospital-based multidisciplinary rehabilitation intervention. It was designed to assist pediatric patients with noncancer pain, POTS, and other chronic physical symptoms to improve coping and level of functioning. Resolution of POTS is not the goal of treatment; functional restoration is the primary goal. 42 Interdisciplinary treatment encompassed integrated care by multiple disciplines including physicians, psychologists, physical therapists, nurses, occupational therapists, 43 and recreational therapists. A cognitivebehavioral model served as the approach for treatment. The program included biofeedback, physical therapy, occupational therapy, recreational therapy, relaxation training, stress management, wellness instruction (eg, sleep hygiene, healthy diet), as well as pain and physical

3 1302 Clinical Pediatrics 55(14) Table 1. Pre- and Posttreatment Means, Standard Deviations, t Tests, and Effect Sizes. Prerreatment, Mean (SD) Posttreatment, Mean (SD) t P Value Effect Size Confidence Interval Functional disability a (11.22) (10.04) 6.17 < Depressive symptoms b (13.15) (10.47) 5.11 < Catastrophizing c (9.64) (8.36) 6.50 < a As assessed with the Functional Disability Inventory. 36 b As assessed with the Center for Epidemiological Studies Depression Children scale. 38 c As assessed with the Pain Catastrophizing Scale for Children. 41 symptom management training. In a group setting, patients and their parents focused on goals related to functional restoration and learned to adaptively selfmanage their chronic physical symptoms. The treatment program consisted of pediatric patients participating in 8 hours per day of therapeutic programming for 15 consecutive weekdays. The pediatric program required extensive parental involvement that included approximately 20 hours of participation per week (total of 60 hours of parental involvement in the 3-week program). The rehabilitation program is based on a biopsychosocial model of pain/ illness that allows parents to learn coping strategies that will assist their child in managing symptoms more effectively and assisting them in returning to a normal level of functioning. Analytic Plan Paired-samples t-test analyses were conducted to examine differences between POTS patients on admission and discharge from the program on all variables including functional status, depression, and catastrophizing. Cohen s d was also calculated as an estimate of clinical significance. Results Participants in the study were 33 patients diagnosed with POTS who completed the 3-week pediatric rehabilitation program within a 2-year span. Twenty-five adolescents were female (74%) and 31 were Caucasian (91%). The average age of participants at the time of entry to the program was 16 years (range = 11-18). See Table 1 for a summary of statistical findings. At baseline, participants showed significantly elevated levels of impairment on the Functional Disability Index (mean = 25), placing the average POTS initial scores as moderately disabled. By discharge from the program, significant improvement was observed for the POTS patients, with disability discharge scores averaging 12, which fell in the no/minimal disability range. This was a significant increase in overall functional abilities (t = 6.166, p <.001), suggesting a large effect (d = 1.27). At baseline, participants showed significantly elevated levels of depressive symptoms, with a mean CESD-C score of Over half of the participants scored in the severe range at baseline (56%). Notably, on discharge, only 3 (9%) patients continued to report severe levels of depressive symptoms. The sample showed a significant reduction in depressive symptoms (t = 5.109, p <.001), suggesting a large effect (d = 1.0). The construct of catastrophizing, which is the use of a negative mind-set linked to disability, was assessed at baseline and discharge from the program using the Pain Catastrophizing Scale for Children. At baseline, scores were significantly elevated for patients with POTS (mean = 22.70). A significant reduction in catastrophizing scores was observed at the end of the program (t = 6.494, p <.001), suggesting a large effect (d = 1.24). Discussion The present study shows that adolescents with POTS achieve significant reductions in functional disability and psychological distress after participation in a 3-week intensive hospital-based outpatient rehabilitation program. This is the first report of an effective structured intervention for the treatment of POTS that significantly reduces the comorbid functional and psychological difficulties observed in these patients. The multidisciplinary program incorporated the best practices available for the treatment of POTS such as increasing fluid and salt, aerobic exercise, increased standing and upright tolerance, continued use of medications for POTS already prescribed if tolerated, and added the structure of a daily schedule, daily group-based physical therapy program, occupational therapy, and recreational therapy activities such as floor hockey and video dance games that aided in endurance training. Parents learned skills for supporting the active self-management of symptoms of dizziness or light-headedness by the adolescents themselves rather than reacting to symptoms

4 Bruce et al 1303 with an emergency response. Biofeedback, relaxation strategies, and cognitive strategies for managing symptoms were also taught to enable the adolescents to cope with physical symptoms in a productive way. The first and major limitation in the design of this study is the lack of a control group that did not participate in rehabilitative treatment. No randomized treatment control studies are yet available that assess the effectiveness of interdisciplinary treatment in adolescents with POTS, but such studies are needed to address the issues of selection bias. Second, the applicability of these findings to other populations of adolescents with POTS may be limited because of inherent selection bias in the study sample. Additionally, some patients and families who are recommended to participate in rehabilitation do not commit to treatment for a variety of reasons including the length of time required for parents to be away from home with their child to participate in the program at a tertiary medical center often many miles from the family home. Although participants in this study are characterized by long-standing debilitating POTS and/or pain symptoms, the patients and their families willingness to undergo rehabilitative treatment may distinguish them from other patients with POTS and/or chronic pain. POTS is being increasingly recognized and diagnosed in adolescents. However, the treatment options currently available for recalcitrant patients are few and may not produce resolution of symptoms. The current study provides preliminary data that suggest that adolescents with recalcitrant POTS can learn strategies for effectively managing their debilitating symptoms and return to a normal level of activity. Although the immediate posttreatment results are favorable for these patients, observation over an extended time is necessary to establish the durability of these findings. In summary, this is the first group study to examine the benefits of participating in a rehabilitation program for adolescents with POTS. The tremendous burden of disability, psychological distress, and impact on quality of life observed in these patients prior to treatment and improvement in these areas posttreatment provide strong rationale for a structured interdisciplinary approach to treatment. The present study demonstrated that a structured 3-week outpatient rehabilitation program can be highly effective in reducing the functional impairment and psychological distress associated with POTS in adolescents. Author Contributions All authors contributed substantially to the work presented in this paper. BKB, TEH, SMB, CAL, C-BJP and PRF made substantial contributions to the conceptualization and design of this study. SEH and DAA were instrumental in the data acquisition and BKB, CMA and KEW contributed significantly to the analysis and interpretation of the data. All authors edited and approved the manuscript as submitted. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Funding The author(s) received no financial support for the research, authorship, and/or publication of this article. References 1. 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