Sleep disorders in SLOS Elaine Tierney M.D. Kennedy Krieger Institute Johns Hopkins University School of Medicine

– Sleep is a brain process • Not just a response to fatigue Normal sleep What is Sleep? – Sleep is a brain process • Not just a response to fatigue • Multiple systems work together to create sleep/wake

3 systems of sleep regulation Nervous system activation – Sympathetic vs. parasympathetic – Example: conditioned anxiety Sleep debt Drive state (hunger, thirst) Circadian Rhythm – Internal biological clock to regulate systems – 24-26 hour cycle Alertness varies across the day Cycle is reset daily by bright light

Sleep impairment in SLOS 50% snore, 67% mouth breathe, 61% take longer than 30 minutes to fall asleep, 61% week screaming, 44% wake more than twice, 67% difficulty falling back to sleep, 61% wake too early, 50% restless sleep, 50% need parent in room, 44% watch TV or listen to music to fall asleep, 33%, unrefreshed in morning 39%, daytime sleepiness 44%, daytime naps 56% (Sarah Roski 2011)

Hours of sleep recommended National Sleep Foundation recommends: Babies sleep twice as much as adults but it your regular intervals preschool children (11-13 hrs), school age children (10 -11 hrs) and for adolescents (9 ¼ hrs). (sleepfoundation.org)

Medical conditions that can lead to sleep problems gastrointestinal (GI) pain and awakening seizures can awaken a child, sleep deprivation can promote seizures sleep apnea (breathing stops briefly) wakes one repeatedly restless leg syndrome can cause discomfort, itching, the need to move the legs and is worse at night

Psychiatric conditions associated with insomnia: mood disorders affect sleeping ADHD can make it difficult to settle anxiety symptoms affect the ability to fall asleep alone in the bed and stay in the bed

Medicines that can impact sleep: Blood pressure medicines, antidepressants, cortisone treatment, progesterone treatment, medications for asthma such as albuterol, stimulants such as Ritalin and Adderall

Sleep hygiene, slide 1 Sleep environment: bedroom should be dark, quiet and cool. Bedtime routine: predictable, 20 to 30 minutes, relaxing activity such as reading or listening to quiet music, no electronics after 8 PM Sleep/wake cycle to be the same on weekday and weekend

Sleep hygiene, slide 2 Teach a child to fall asleep alone: If a child needs the parent to fall asleep at bedtime, he might need the parent to fall back asleep during normal wakening of the night. Exercise: daytime exercise makes it easier to fall asleep but should not exercise too close to bedtime (effect lasts 2 hours in adults)

maintain a regular schedule open the curtain and turn on lights in AM Sleep hygiene, slide 3 avoid caffeine naps should be avoided except for preschool children. Naps should Not be taken late in the afternoon maintain a regular schedule open the curtain and turn on lights in AM

Sleep hygiene, slide 4 Autism Speaks sleep toolkit: https://www.autismspeaks.org/science/resources-programs/autism-treatment-network/tools-you-can-use/sleep-tool-kit Sleep strategies for teenagers with autism spectrum disorder: http://www.autismspeaks.org/sites/default/files/docs/sciencedocs/atn/sleep_strategies_for_teens_tool_kit.pdf

Medicines that can worsen sleep Serotonin reuptake inhibitors such as fluoxetine, paroxetine, sertraline Asthma medications such as theophylline and albuterol Steroid medication such prednisone Stimulant medication such as methylphenidate, dexmethylphenidate, amphetamine, Adderall

Medications used for sleep, slide 1 Clonidine: Lower doses than used for blood pressure. Medication also used for ADHD. Guanfacine: medication also used for ADHD. Antihistamines such as Benadryl, Hydroxyzine Cyproheptadine, (antihistamine that increases appetite), Claritin , Atarax - all can agitate Nasal steroids such as Nasonex and Flonase (may decrease snoring)

Medications used for sleep, slide 2 Trazodone Melatonin (may affect absorption of cholesterol) It is unknown exactly how melatonin lowers cholesterol levels. Evidence points to a higher rate of conversion from cholestrol to bile acids, which the bile then excretes. Using melatonin as a sleep aide is usually given in doses of less than 3mg, much less than the doses that would cause this minor effect.

Medications used for sleep, slide 3 Chloral hydrate (caused violence in some) is very rarely used Ropinirole hydrochloride –I’ve never used this (typically used for Parkinsons; small dose, 1mg, relaxes) Benzodiazepines such as diazepam,Nitrazapam,Clonazepam Side effect of neuroleptic such as risperidone

Any SLOS individual can participate whether they sleep well or not. Dr. Kothare’s study: Dr. Sanjeev Kothare and team at Children’s Hospital Boston are looking for participants for a sleep study. Any SLOS individual can participate whether they sleep well or not. http://www.smithlemliopitz.org/studies/sleep-study/

Sleep is abnormal in autism spectrum disorder