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

2. – 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. 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
– hour cycle
Alertness varies across the day
Cycle is reset daily by bright light

4. 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)

5. 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 ( hrs)
and for adolescents (9 ¼ hrs).
(sleepfoundation.org)

6. 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

7. 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

8. 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

9. 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

10. 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)

11. 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

12. Sleep hygiene, slide 4
Autism Speaks sleep toolkit:
Sleep strategies for teenagers with autism spectrum disorder:

13. 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

14. 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)

15. 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.

16. 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

17. 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.

18. Sleep is abnormal in autism spectrum disorder