Sleep, Pain, and Fatigue in Ehlers-Danlos Syndrome

Ehlers-Danlos National FoundationLearning Conference

August 9, 2012

Susan Cordes, MS, CGC

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.

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)

Results• Responses

– Total: 1,252– Completed: 1, 054 (84.2%)– Analyzed 888

• Demographics– Age

• Range 14-83• Average 35.7

Demographics: gender

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

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)

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

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%

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

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

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

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

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