sleep, pain, and fatigue in ehlers-danlos syndrome ehlers-danlos national foundation

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Susan Cordes , MS, CGC. Sleep, Pain, and Fatigue in Ehlers-Danlos Syndrome Ehlers-Danlos National Foundation Learning Conference August 9, 2012. Online Survey. - PowerPoint PPT Presentation



Sleep, Pain, and Fatigue in Ehlers-Danlos Syndrome

Ehlers-Danlos National FoundationLearning Conference

August 9, 2012Susan Cordes, MS, CGC

1Online SurveyThe 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.Introduction:Why are we concerned about sleeping patterns in EDS?Interaction of pain, fatigue, RLS with poor sleep in EDS-Its a given that sleep disturbance is correlated with fatigue. But pain and sleep disturbances are also closely correlated. Experience and studies have shown that those suffering from different pain disorders have significant sleep disturbances which are related to pain. Conversely, sleep deprivation can cause fatigue and increased sensitivity to pain. Therefore, improving sleep quality and quantity may help contribute to breaking vicious cycle and thus enhance patients overall health and quality of life.-Fatigue is a common symptom in various conditions with chronic pain. People with chronic pain often experience fatigue as a result of physical strain or emotional stress from dealing with chronic pain. Fatigue can have an impact on pain sensitivity.-RLS characterized in order to identify sleep variables that may explain poor sleep complaints. Also RLS can occur secondary to chronic pain especially due to musculoskeletal disorders. Furthermore, some evidence suggests that sensory pain-related perception is altered in RLS patients. PLM have been found in a significant proportion of EDS patients in a previous study. RLS is associated with sleep disturbance and excessive daytime sleepiness.-All of these can contribute to quality of life and all of these are common complaints in EDS patients-However, research on pain, fatigue, sleep quality, and RLS in EDS is limited-previous pain and fatigue studies by Nicol Voermans et al in Dutch EDS patient organization of 273 patients. Studies more focused on hypermobile vs classic patients (hEDS more prevalent and severe pain and more fatigue than cEDS) as well as what pain is correlated with (hypermobility, dislocations, previous surgery) and possible fatigue determinants (sleep disturbances, concentration problems, social functioning, self-efficiency concerning fatigue, and pain severity)2SurveyDemographicsQuestionnairesPittsburg Sleep Quality Index (PSQI)Brief Pain Inventory (BPI)Brief Fatigue Inventory (BFI)International Restless Legs Syndrome Study Group Rating Scale (RLS-RS)

-Demographics: age, gender, type of EDS-Questionnaires: already validated (have internal consistency with high reliability and clinical validity) 3ResultsResponsesTotal: 1,252Completed: 1, 054 (84.2%)Analyzed 888DemographicsAgeRange 14-83Average 35.7

-Used only those surveys that were completed. Due to numbers, we ended up only using those with hypermobile, classic or those that said mild classic vs hypermobile. Ended up with 888 total.4Demographics: gender

Really no big surprise here Females: 91.6%Males: 8.4%


6Results: PSQIMeasures:Subjective sleep qualitySleep latencySleep durationHabitual sleep efficiencySleep disturbancesUse of sleep medicationsDaytime dysfunctionRatings were 0-3

-PSQI asks about sleep habits. -Score of 3 for each component represents the extreme negative-It is made up of 7 components: subjective sleep quality index, sleep latency, sleep duration, sleep efficiency (hours slept divided by hours in bed), sleep disturbance, use of sleep medication, daytime dysfunction. Global score is sum of seven component scores (ranges from 0-21 with higher number indicating worse sleep quality). A score of >5 indicates good vs poor sleepers.-Average EDS score: 12.97SleepSignificant decrease in overall sleep quality as compared to controlsMean rated it fairly bad (1.87 v. 0.35)Mild degree of insomnia compared to controlsAvg. time 30-60 minutesLess amount of time spent sleeping than controlsReduced sleep efficiency (1.71 v. 0.10)8.5 hours in bed with 6 hours of actual sleepFrequent nighttime awakenings (more than 3 times per week)Including feeling hot and having painOften take sleep aids (1.52 v. 0.04)Difficulty staying awake and energy (enthusiasm) levels (2.06 v. 0.35)

SleepSignificant correlation to BFIi.e. reduced sleep quality correlated with worse fatigueDid not correlate with pain severity or pain intensity from the PSQIPain may only be a contributing factor to sleep issues in EDS

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

-asses the severity of pain and its impact on daily functions, location of pain, pain medications -asks patients to rate their current pain intensity as well as pain in the last 24 hours at its worst, least, and average by using a numeric scale of 0 no pain to 10 pain as bad as you can imagine. This makes up the pain severity score.-patients are also asked to rate the extent to which their pain interferes with seven quality-of-life domains that include: general activity, walking, mood, sleep, work, relations with other persons, and enjoyment of life. This makes up the pain interference score.-mild pain is defined as a worst pain score of 1 - 4, moderate pain is defined as a worst pain score of 5 - 6, and severe pain is defined as a worst pain score of 7 10-Pain Severity ScoreAverage: 5.0Pain Interference ScoreAverage: 6.1Worst PainAverage 6.8 (moderate to severe)10PainSignificantly more pain severity than compared to controlsSignificant pain interference affecting general activity, mood, work, relationships, sleep, and enjoyment of life

However, did not correlate with sleep quality or overall fatigue

Results: BFI92% reported that they are unusually tired/fatigue in past weekAverage, 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 imagineFatigue significantly interfered with general activities, mood, walking ability, work, relationships, and enjoyment of lifeGlobal score (6.11 v. 3.04 (sleep disorders) v. 4.04 (cancer))

Correlated with decreased sleep quality-used to assess the severity of fatigue and the impact of fatigue on daily functioning-fatigue: overwhelming sense of tiredness, lack of energy, feeling of exhaustion-asks patients to rate, on a scale of 0 (none) to 10 (severe), their current level of fatigue, usual fatigue level in the last 24 hours, worst level of fatigue in the last 24 hours, and how fatigue has interfered with general activity, mood, walking, work, relationships, and enjoyment of life-score is average of all 9 questions (ranges from 0-10)-severe fatigue can be defined as a worst fatigue score of 7 or greaterUsually tired?92.3% said yesAverage score: 6.1Worse fatigue average: 7.8 (severe)

12Results: RLS-RS21% self-report diagnosis of restless legs syndromeSymptoms occur on average 4-5x per weekReported to have a mild impact on sleep7% diagnosed with PLMPSQI: legs twitching or jerking a few times a week while asleep (assessed by bed partner)Over all respondents, averaged moderate symptoms

-Restless legs syndrome- disorder in which there is an urge or need to move the legs to stop unpleasant sensations.Often occurs at night while awakeHeritableCan disturb sleep-Periodic limb movement- repetitive cramping or jerking of the legs during sleep.

Diagnosis of RLS20.9%Diagnosis of periodic limb movement6.8%


No symptoms: 28.4%Mild: 11.0Moderate 29.7Severe 25.0Very severe 5.9Total average score: 13.4 (moderate range)14ConclusionsPain common, severe, involves multiple sites, and interferes with daily livingSleep disturbance common but frequent awakenings is more common than insomniaSleep disturbance related to generalized fatigue but may not be the only factorPain is somewhat related to the sleep disturbance but so are other factorsRestless leg syndrome common but has only modest effect on sleep disturbance

-Limitations: ascertainment bias, mostly females, controls-Future studies: include other EDS types, differences between genders and ages, differences between hypermobile vs classic, other sources of sleep disturbance such as sleep disordered breathing etc-Management: proper sleep hygiene, cognitive-behavioral therapy, medication for sleep improvement and pain control

15AcknowledgementsBrad Tinkle, MD, PhDSabrina Neeley, PhD, MPHAll the participants!Chart18.491.6


Sheet1MaleFemaleSeries 3Gender8.491.62Category 22.54.42Category 33.51.83Category 44.52.85To resize chart data range, drag lower right corner of range.


EDS type

Sheet1EDS typehypermobile80.3classic16.5h/c3.1To resize chart data range, drag lower right corner of range.



Sheet1RLSNo symptoms28.4Mild11Moderate29.7Severe25Very severe5.9To resize chart data range, drag lower right corner of range.


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