Sleep, Pain, and Fatigue in Ehlers-Danlos Syndrome Ehlers-Danlos National Foundation

1. Susan Cordes, MS, CGC Sleep, Pain, and Fatigue in Ehlers-Danlos Syndrome Ehlers-Danlos National Foundation Learning Conference August 9, 2012

2. Online Survey • The purpose was to characterize poor sleep, pain, fatigue, and RLS and determine if there are correlations to age and/or gender in patients self-identified with EDS. • Used SurveyMonkey posted on the EDNF website with consent of local IRB.

3. Survey • Demographics • Questionnaires • Pittsburg Sleep Quality Index (PSQI) • Brief Pain Inventory (BPI) • Brief Fatigue Inventory (BFI) • International Restless Legs Syndrome Study Group Rating Scale (RLS-RS)

4. Results • Responses • Total: 1,252 • Completed: 1, 054 (84.2%) • Analyzed 888 • Demographics • Age • Range 14-83 • Average 35.7

5. Demographics: gender

7. Results: PSQI • Measures: • Subjective sleep quality • Sleep latency • Sleep duration • Habitual sleep efficiency • Sleep disturbances • Use of sleep medications • Daytime dysfunction • Ratings were 0-3

8. Sleep • Significant decrease in overall sleep quality as compared to controls • Mean rated it fairly bad (1.87 v. 0.35) • Mild degree of insomnia compared to controls • Avg. time 30-60 minutes • Less amount of time spent sleeping than controls • Reduced sleep efficiency (1.71 v. 0.10) • 8.5 hours in bed with 6 hours of actual sleep • Frequent nighttime awakenings (more than 3 times per week) • Including feeling hot and having pain • Often take sleep aids (1.52 v. 0.04) • Difficulty staying awake and energy (enthusiasm) levels (2.06 v. 0.35)

9. Sleep • Significant correlation to BFI • i.e. reduced sleep quality correlated with worse fatigue • Did not correlate with pain severity or pain intensity from the PSQI • Pain may only be a contributing factor to sleep issues in EDS

10. Results: BPI • BPI reports two scores: pain severity and pain interference • Nearly all patients reported pain (98%) • Respondents reported 12 sites on average with persistent/recurring pain • Most common were jaw, neck, back (especially lower), shoulder, wrist, hand, digits, hip, knee, ankle, and feet • Pain ranged from a 3-7 (on a scale from 0-10) • Average pain relief from medications 27%

11. Pain • Significantly more pain severity than compared to controls • Significant pain interference affecting general activity, mood, work, relationships, sleep, and enjoyment of life • However, did not correlate with sleep quality or overall fatigue

12. Results: BFI • 92% reported that they are unusually tired/fatigue in past week • Average, least and worst fatigue levels during the past 24 hours ranged from 5-8 on a 10 point scale where 0 was no fatigue and 10 “as bad as you can imagine” • Fatigue significantly interfered with general activities, mood, walking ability, work, relationships, and enjoyment of life • Global score (6.11 v. 3.04 (sleep disorders) v. 4.04 (cancer)) • Correlated with decreased sleep quality

13. Results: RLS-RS • 21% self-report diagnosis of restless legs syndrome • Symptoms occur on average 4-5x per week • Reported to have a mild impact on sleep • 7% diagnosed with PLM • PSQI: legs twitching or jerking a few times a week while asleep (assessed by bed partner) • Over all respondents, averaged moderate symptoms

15. Conclusions • Pain common, severe, involves multiple sites, and interferes with daily living • Sleep disturbance common but frequent awakenings is more common than insomnia • Sleep disturbance related to generalized fatigue but may not be the only factor • Pain is somewhat related to the sleep disturbance but so are other factors • Restless leg syndrome common but has only modest effect on sleep disturbance

16. Acknowledgements • Brad Tinkle, MD, PhD • Sabrina Neeley, PhD, MPH • All the participants!