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

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!