Introduction:Why are we concerned about sleeping patterns in EDS?Interaction of pain, fatigue, RLS with poor sleep in EDS-It’s 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 patient’s 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)
-Demographics: age, gender, type of EDS-Questionnaires: already validated (have internal consistency with high reliability and clinical validity)
-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.
Really no big surprise here Females: 91.6%Males: 8.4%
-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: 12.9
-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)
-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)
-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)
-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
Cordes eds sleep
Susan Cordes, MS, CGCSleep, Pain, and Fatigue in Ehlers-Danlos Syndrome Ehlers-Danlos National Foundation Learning Conference August 9, 2012
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
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!