Attitudes, beliefs and behaviours towards exercise amongst individuals with joint hypermobility syndrome/Ehlers Danlos Syndrome – Hypermobility Type

Abstract

Background: Idiopathic slow transit constipation (STC) describes a clinical syndrome characterised by intractable constipation. It is diagnosed by demonstrating delayed colonic transit time. STC is found to be more common in children with generalised joint hypermobility and is a documented clinical manifestation of Ehlers Danlos Syndrome - Hypermobility Type (EDS-HT). Children diagnosed with EDS-HT and STC have a chronic condition that may significantly impair quality of life. Laxatives may not be effective and/or not well tolerated due to coexisting upper gastrointestinal symptoms. For children who are non-responsive or unable to tolerate traditional constipation strategies, more invasive and expensive treatment options in the UK currently include appendicostomy formation through which antegrade continence enemas can be performed, antegrade colonic evacuation, Piristeen enemas system or Botox. In adult STC, subtotal or segmental colonic resection is proposed as the treatment of choice. Recent research has demonstrated improvements in transit times, reduced constipation and gastrointestinal pain and improved QoL for children with idiopathic STC with daily interferential stimulation. Purpose: IFT has been introduced within the care pathway as a novel service development since October 2013 for children diagnosed with STC and EDS-HT. We report a clinical audit to help inform practice development and future research. Methods: Seven children (4 males, 3 females) diagnosed with EDS-HT and STC aged between 6 and 12 years have been offered IFT. The protocol includes; toileting education to child and parents and 30 minutes of daily IFT at a sensory stimulation range of 80-160 Hz for 3 months. Outcome measures (OCM) completed by child and/or parent at base line, 3, 6 and 12 months include; Paediatric Quality of Life Scale (PedsQL), Gastrointestinal Quality of Life Score (GQLS), Visual Analogue Scale (VAS) for abdominal pain, Bowel Diary information includes; stool frequency, soiling, and medication chart. Data analysed using descriptive statistics. Results: 5 children have completed the 3 month course of treatment. One child did not find the treatment acceptable due to anxiety and abdominal sensitivity. Another child ceased the intervention after 3 weeks as could feel no benefit. A full set of base line, 3 month and 6 month follow up OCM data has been collected and analysed from 5 children. Results include mean, median and standard deviation for each OCM. PedsQL: Baseline 45.6 (45, 4); 3 months 67.2 (65, 7.6); 6 months 69.4 (69, 8.8);GQSL Baseline 37.8 (36.1, 2.5); 3 months 47.8 (44.4, 3.1); 6 months 45.5 (44.4, 2.1); VAS abdominal pain: Baseline mean 7 (6, 0.6); 3 months 6 (6, 0.6); 6 months 5.8 (6, 0.4). Stool frequency (per week): Baseline 2.4 (2, 0.9); 3 months 4.4 (4, 6.5); 6 months 4 (4, 0.7). Conclusion(s): Children with EDS-HT and STC have a chronic condition that may significantly impair quality of life. Audit shows promising results in 5/7 children. One child found the stimulation unacceptable. Implications: Interferential may be an acceptable, effective and cost efficient non-invasive intervention for children with EDS-HT and STC. Future audit and research will explore subgroups, mechanism, cost effectiveness and IFT dose optimisation.