Health related quality of life survey in pediatric patients with autonomic dysfunction and gastrointestinal symptoms

Introduction: Patients with autonomic dysfunction and postural orthostatic tachycardia syndrome (POTS) have significant symptom burdens many of which are gastrointestinal. In at least one survey, gastrointestinal symptoms were reported in >80% of surveyed POTS patients. There is little evidence in the current literature about quality of life of POTS patients. Higher Nausea Profile Questionnaire scores correlated with worse quality of life in functional abdominal pain disorder patients. Anecdotally, multidisciplinary teams and their therapies focus on functional outcomes for these patients. The PedsQL survey is a validated health related quality of life (HRQOL) survey for children ages 3-21 and adults. Its use has been validated in surveying HRQOL in pediatric patients with functional abdominal pain, irritable bowel syndrome, chronic constipation, Crohn's disease, ulcerative colitis, and gastroesophageal reflux disease. Objective: The objective of this study was to quantify the HRQOL scores for patients with POTS with co-morbid GI symptoms and compare their scores to POTS patients who did not require a referral to a pediatric gastroenterologist, as well as to published HRQOL scores of historical controls in other chronic GI illnesses. The secondary aim was to determine if there are specific gastrointestinal symptoms which are negatively impacting quality of life scores. Methods: This is a single site survey of patients with POTS. We performed a chart review of ICD-10 diagnosis codes for postural orthostatic tachycardia syndrome (postural orthostatic tachycardia syndrome, dysautonomia, I95.1, I49.8, R00.9 ) , and identified adolescent patients ages 12-23 who saw a pediatric gastroenterologist at St. Christopher's Hospital for Children in the year 2020-2021. Exclusion criteria included IBD, celiac disease, known cardiac or neurologic disorder, familial dysautonomia, and not seeing a gastroenterologist for more than two years. A separate population of POTS patients who were not referred to GI in the past year was also identified. Parents were contacted via phone to enroll their children in the survey. The PedsQL survey was electronically distributed via RedCap and responses were logged in the RedCap database. Patients were sent the PedsQL and the PedsQL GI Symptom Scale surveys. Patients data were analyzed if they completed both surveys. Results: Of the 110 patients contacted, 53 enrolled in the study. Twenty-five complete response were recorded. POTS patients referred to GI had significantly lower PedsQL scores (mean 51.1, SD 18.4) compared to POTS patients who did not need a referral to GI (mean 65.6 SD 6.04) , p< 0.05. Both of these groups have PedsQL scores that are significantly lower than those reported in the literature for patients with IBD, GERD, and lower than healthy controls. The POTS patients referred to GI also had significantly lower Peds QL scores than those reported for patients with IBS and functional abdominal pain (table 2). Pearson correlation analysis of Peds QL GI symptom scale showed a statistically significant positive correlation between nausea and vomiting subgroup scores and Peds QL scores (r = 0.42, p < 0.05). That is, the better the reported quality of life score, the lower the severity of nausea and vomiting. Conclusion: Pediatric patients with POTS with and without co-morbid gastrointestinal symptoms have a lower quality of life score than many chronic gastrointestinal illnesses. Additionally, POTS patients with co-morbid GI symptoms requiring referral to GI have worse quality of life compared to patients who do not require GI referral. Specifically within our patient population, worsening symptom scores for nausea and vomiting were associated with worse quality of life scores. These findings highlight the severity of these disorders and the need to better understand them. Limitations of this study include small sample size, potential for recall bias, and the confounding effect the COVID-19 pandemic has had on multiple aspects of our patients lives.