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Sleep Disorders in Ehlers- Danlos Syndrome. Alan G. Pocinki , M.D. Ehlers- Danlos National Foundation Learning Conference August 9-11, 2012. Overview. Autonomic nervous system (ANS) regulates all body processes, including sleep

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sleep disorders in ehlers danlos syndrome

Sleep Disorders in Ehlers-Danlos Syndrome

Alan G. Pocinki, M.D.

Ehlers-Danlos National Foundation Learning Conference

August 9-11, 2012

overview
Overview
  • Autonomic nervous system (ANS) regulates all body processes, including sleep
  • ANS dysfunction is very common in Ehlers-Danlos and other hypermobility syndromes, and underlies many of their symptoms
  • The most common type of sleep disorder seen in the hypermobility syndromes appears to have an autonomic basis
basics of the ans
Basics of the ANS
  • Sympathetic nervous system: “fight or flight,” the accelerator
  • Parasympathetic nervous system: “rest and digest,” the brake
autonomic instability
Autonomic Instability
  • Concept of adrenaline reserve
  • Central paradox: the lower the reserves, the more exaggerated your stress response, so your body “overresponds” to minor stresses
  • The overresponse often triggers an overcorrection, then an overresponse…
sympathetic and parasympathetic activity with autonomic maneuvers
Sympathetic and Parasympathetic Activity with Autonomic Maneuvers

A

B

C

D

E

F

Normal

EDS with Dysautonomia

A=Baseline, B=Deep Breathing, C=Rest, D=Valsalva, E=Rest, F=Stand

sympathetic and parasympathetic activity before and after treatment
Sympathetic and Parasympathetic Activity Before and After Treatment

At Diagnosis

After 18 months of treatment

A=Baseline, B=Deep Breathing, C=Rest, D=Valsalva, E=Rest, F=Stand

non restorative s leep in eds
Non-RestorativeSleep in EDS
  • Frequent arousals and awakenings
  • Little or no deep sleep

Normal Sleep

Non-Restorative Sleep

heart rate variability associated with sleep disruptions
Heart Rate Variability Associated with Sleep Disruptions

Heart Rate

100

80

60

Awake

REM

N1

N2

N3

Sleep Stages

heart rate variability another paradox
Heart Rate Variability--Another Paradox
  • The lower sympathetic activity is, the greater heart variability, or
  • The more exhausted you get, the more “depleted” your energy reserves, the more exaggerated heart rate fluctuations will be
  • The more your heart rate fluctuates, the more disrupted your sleep (not to mention daytime activities)
  • The more disrupted your sleep, the more exhausted you get—a nasty vicious cycle
sleep misperception another paradox
Sleep “Misperception”Another Paradox
  • Many EDS patients report that they “sleep fine.”
  • “I’m a great sleeper. I can fall asleep any time, anywhere.”
  • But… Do you feel rested when you get up?
  • “No, I never feel rested.”
  • “I wake up feeling like I haven’t slept.”
  • “I don’t think I know what feeling rested would feel like.”
  • Not just a problem in EDS, e.g. 90% of people with sleep apnea are not aware of it
non restorative s leep
Non-RestorativeSleep
  • Frequent arousals and awakenings
  • Little or no deep sleep

Normal Sleep

Non-Restorative Sleep

sympathetic and parasympathetic activity before and after treatment1
Sympathetic and Parasympathetic Activity Before and After Treatment

At Diagnosis

After 18 months of treatment

A=Baseline, B=Deep Breathing, C=Rest, D=Valsalva, E=Rest, F=Stand

treatment of autonomic dysfunction
Treatment of Autonomic Dysfunction
  • Better sleep
  • Address underlying problems:
    • Pain
    • Fatigue
    • Dehydration
    • Low blood sugar
    • Emotional stresses
restoring autonomic balance
Restoring Autonomic Balance
  • Better sleep—quantity and quality
  • Adequate—really—pain control
  • Don’t “push through” fatigue; take breaks
  • Adequate salt and fluid
  • Avoid hypoglycemia
  • Minimize emotional stresses
slide19

“Your suggestion to ratchet down my level of ‘busy-ness’ [by taking frequent short breaks] to facilitate relaxation is great. It’s helpful and enjoyable. It’s good to have ‘doctor’s orders’ to relax and read a book for a few minutes in the middle of the day!”

treatment of sleep disorders
Treatment of Sleep Disorders
  • Don’t overlook the basics:
    • Good sleep hygiene
    • Comfortable mattress
    • Dark and quiet
    • Elevate head of bed (if lightheaded during the day)
    • Treat sleep apnea, limb movements only if significant
treatment of sleep disorders medication
Treatment of Sleep Disorders: Medication
  • Complex medication “regimen” is often required:
    • Multiple medications with complementary effects, e.g. one medication for pain, one to reduce arousals, one to increase deep sleep
    • Finding the right combination can be a frustrating trial and error process
    • Home sleep monitor may be helpful (www.myzeo.com)
treatment of sleep disorders medication1
Treatment of Sleep Disorders: Medication
  • Block extra adrenaline (beta and alpha blockers, clonidine and guanfacine)
  • Offset extra adrenaline (benzodiazepines, SSRI’s)
  • Reduce pain (analgesics, muscle relaxants, Neurontin™, Lyrica™)
  • Increase deep sleep (trazodone, amitryptiline, doxepin)
  • Use “Sleeping pills” sparingly
beta blockers
Beta Blockers
  • Propranolol
    • Start with 10 mg at bedtime
    • Increase by 10 mg every 4-5 days until fewer awakenings, side effects, or no further benefit
    • Switch to long-acting if needed
    • Take some earlier to offset “second wind”
    • Often need smaller daytime dose as well
other beta blockers
Other Beta Blockers
  • Metoprolol
    • Start with half a 25 mg tablet (metoprolol tartrate)
    • Increase by half a tablet every 4-5 days
    • Add long-acting (metoprolol succinate) if needed
  • Nadolol
    • Safe in asthma (Bystolic™ also safe in asthma, but once daily)
    • Start with 20 mg. increase by 20 every 4-5 days
    • Add smaller AM dose if needed for daytime symptoms
  • Carvedilol
    • Start with 3.125 mg, iIncrease by one tablet every 4-5 days
    • Add smaller AM dose if needed for daytime symptoms
clonidine guanfacine
Clonidine/Guanfacine
  • Clonidine
    • Start with 0.1 mg at bedtime
    • Increase by 0.1 mg no sooner than one week
    • No more than 0.3 mg
    • Usually lasts about 6 hours
  • Guanfacine
    • Very similar to clonidine but lasts longer
    • Recently remarketed as Intuniv™ for ADD
alpha blockers
Alpha Blockers
  • Prazosin best studied, shown to reduce nightmares in PTSD, where “a hypersensitivity to adrenaline triggered many of their nightmares.” In a VA study, 75-80% of PTSD patients stopped having nightmares.
  • Usual dose is 5mg
  • Can worsen orthostatic intolerance
  • Not clear if combination alpha-beta blockers (e.g. carvedilol) are as effective, but probably not.
benzodiazepines
Benzodiazepines
  • All have beneficial properties:
    • Sedative
    • Anti-anxiety
    • Muscle relaxant
    • Anti-movement, anticonvulsant
    • “Anti-adrenaline”
  • But also potential problems:
    • Impair cognition, motor performance
    • Depress mood, respiration
    • Cause or worsen fatigue
    • Tolerance
    • Dependence
    • Withdrawal
some common benzodiazepines
Some Common Benzodiazepines
  • Clonazepam (Klonopin™)
    • Longest-lasting, most likely to have residual effects
    • Also effective for restless leg, PLMS
  • Diazepam (Valium™)
    • Typically lasts about 8 hours
    • Probably best muscle relaxant
  • Temazepam (Restoril™)
    • Typically lasts about 7 hours
    • Capsule limits dosage adjustment
  • Lorazepam (Ativan™)
    • Typically lasts about 6 hours
    • Metabolized differently (less variability, interactions)
analgesics
Analgesics
  • Anti-inflammatories
    • NSAID’s: Naproxen, Meloxicam, Celebrex™
    • Prednisone
  • Tramadol, short- and long-acting
  • Narcotics, short-, long-acting; patches (fentanyl, Butrans™)
  • Cymbalta™, Savella™
  • Gabapentin (Neurontin™), Lyrica™
  • Lidoderm™
  • Flector™, Voltaren Gel™, Pennsaid™
muscle relaxants
Muscle Relaxants
  • Cyclobenzaprine
    • Shown to improve sleep quality in fibromyalgia
    • Has analgesic, sedative, muscle relaxant properties
  • Soma
    • Less sedating, ? more analgesic effect, especially with narcotics
  • Skelaxin
    • Less sedating, some can tolerate daytime doses
  • Tizanidine
    • More sedating, high margin of safety
  • Baclofen
    • Potent, use for severe painful spasm only
other agents
Other Agents
  • Trazodone
    • Probably most effective at increasing deep sleep
    • Low dose, 50-150 mg, most people take 50
  • Amitryptiline
    • Also increases deep sleep, especially with pain
    • Start at 10 mg, most people take 20-40mg
  • Doxepin
    • Enhances sleep more at lower doses
    • 10 mg tablet, liquid, or Silenor™ 3 mg, 6 mg
  • DDAVP (Desmopressin)?
sleeping pills
“Sleeping Pills”
  • Zolpidem, short- and long-acting
    • Doesn’t reduce arousals or improve sleep architecture
    • Onset/maintenance, e.g. until other meds effective
    • Retrograde amnesia
    • Zolpidem usually lasts 5 hours, ER about 7
  • Lunesta
    • Doesn’t reduce arousals or improve sleep architecture
    • Occasionally helps with sleep onset and maintenance, e.g. until other medications become effective
    • Usually lasts about 7 hours
  • Zaleplon
    • Good for sleep onset, especially getting back to sleep
    • Lasts 2-3 hours, no cognitive impairment
  • Melatonin/Rozerem
    • Most helpful for Circadian problems e.g. evening “second wind”
antidepressants
Antidepressants
  • SSRI’s often cause shallower sleep, more dreams
    • Prozac worst, Lexapro best
    • Use lowest effective dose, consider liquid formulations
  • Cymbalta sleep neutral if taken in AM
  • Tricyclics generally improve sleep, but often cause daytime sedation
  • Wellbutrin impairs sleep if taken late in day, so take once-daily (XL) form early in day or consider AM only dosing of twice a day (SR) form
  • Remeron generally improves sleep, can cause weight gain
slide40

“I am stunned, amazed, and grateful at the benefits of taking propanolol. The improvement in my sleep quality alone is fantastic.”

  • “The medicine you gave me is amazing. Two worked great but three worked even better. I forgot to take it one night and slept 12 hours and felt terrible. The next night I took it and slept 6 hours and felt great.”
  • The metoprolol seems to help considerably with my sleep. In fact, between metoprolol, flexeril, and good old advil, I’m able to fall asleep and stay asleep. The metoprolol really seems to be particularly important for quality of sleep.
  • Propranolol is working very well in helping me to sleep.
summary
Summary
  • The most common type of sleep disorder seen in the hypermobility syndromes appears to be characterized by excessive heart rate variability at night
  • Medications to suppress, offset, or block this excess activity are effective in improving sleep, measured both by polysomnography and symptoms
  • Improving sleep and minimizing daytime stresses helps to replenish autonomic reserves, which in turn improves daytime autonomic balance and also helps improve sleep, which in turn improves daytime function, which in turn improves circadian rhythms and sleep, which …..
acknowledgements
Acknowledgements

EDNF (Sandy Chack) and Dr. Brad Tinkle for inviting me

Dr. Peter Rowe for encouraging me when others thought I was nuts

Dr. Clair Francomano and Dr. Fraser Henderson for teaching me about EDS and stimulating my interest in it

All my patients, for having the confidence in me to let me experiment on them!