Approach to Sleep Challenges in Angelman Syndrome
Sleep challenges are common in individuals with Angelman Syndrome, with 80% of children experiencing sleep concerns. These concerns include decreased sleep duration, fragmented sleep, and decreased REM sleep. Poor sleep can worsen other aspects of Angelman Syndrome, such as seizures, learning and memory, attention, and behavior regulation. To address sleep challenges, it is important to identify the sleep concerns and consider a sleep study if necessary. Behavioral approaches, such as establishing a bedtime routine and creating a sleep-friendly environment, are the first step in managing sleep challenges. Medication management, including the use of benzodiazepines, melatonin, clonidine, SSRIs, and Seroquel, may be considered if behavioral interventions are not effective. Each case requires an individualized approach, and ongoing communication and adjustments are necessary to find the most effective strategies for each individual.
Hello, and thank you to the Foundation for Angelman Syndrome Therapeutics for having us today. In this talk, we will be discussing an approach to sleep challenges in Angelman Syndrome. My name is Dr. Jessica Duis, and I am the director of the Chromosome 15 Clinic at Children’s Hospital Colorado. I am joined by Terri Katz, a psychologist who has helped start a sleep clinic for individuals with neurodevelopmental challenges at Children’s Hospital Colorado.
Sleep Challenges in Angelman Syndrome
Sleep issues are very common in individuals with Angelman Syndrome, with studies showing that 80% of children with Angelman experience sleep concerns. These concerns include a decreased need for sleep, fragmented sleep with frequent nighttime awakenings, and decreased REM sleep. Poor sleep can have a significant impact on individuals with Angelman Syndrome, exacerbating seizure activity, impairing learning and memory, affecting attention and behavior regulation, and causing gastrointestinal issues such as reflux and constipation.
Identifying and Managing Sleep Concerns
To address sleep challenges in Angelman Syndrome, it is important to first identify the presence of sleep concerns. This can be done through discussions with families about the impact of poor sleep on the individual and the family unit. Sleep studies may also be recommended in certain cases, particularly if there are concerns about sleep-related breathing problems or unusual behavior during sleep. Other methods of identifying sleep problems include keeping a sleep diary or using wearable devices like actigraphy.
In addition to identifying sleep concerns, it is important to address any underlying medical issues that may contribute to restless sleep, such as reflux, anxiety, low iron stores, or allergies. Treating these medical problems can help improve sleep quality. Creating a safe and comforting sleep environment, promoting good sleep hygiene, and establishing consistent bedtime routines are also crucial steps in managing sleep challenges.
Behavioral Approaches to Sleep Management
Behavioral management of sleep is often the first step in addressing sleep challenges in Angelman Syndrome. This includes establishing consistent bedtime routines that are calming and relaxing, reducing stimulating activities in the evening, and ensuring a sleep environment that is conducive to sleep. Visual supports, such as visual schedules or objects representing bedtime activities, can also be helpful in signaling the transition from wakefulness to sleep. It is important to consider the timing of bedtime, avoiding the “forbidden zone” when the child is more alert, and gradually adjusting bedtime to a desired time.
In some cases, behavioral interventions may not be sufficient to address sleep challenges, and medication management may be considered. Medications such as benzodiazepines, melatonin, clonidine, SSRIs, and Seroquel may be prescribed to help with falling asleep, staying asleep, and managing anxiety. The choice of medication depends on the individual’s specific needs and should be discussed with a healthcare professional.
To provide practical examples, we discussed two case studies. In the first case, a seven-year-old girl with Angelman Syndrome was having difficulty transitioning from falling asleep with her father to sleeping in her own bed. She also experienced frequent nighttime awakenings. Behavioral interventions focused on gradually transitioning the father out of the bed and establishing a consistent bedtime routine. Medication management included the use of clonazepam or melatonin if needed.
In the second case, a three-year-old boy with Angelman Syndrome was experiencing restlessness and multiple nighttime awakenings. The parents were concerned about his safety as he was starting to climb out of his crib. Behavioral interventions included transitioning to a safe sleep bed, creating a comforting sleep environment, and using visual cues to signal sleep time. Medication management focused on addressing any underlying medical issues and considering medications like clonazepam or melatonin.
- Title: Approach to Sleep Challenges in Angelman Syndrome
- Author(s): Jessica Duis, Terry Katz
- Author(s)’ affiliation: Children’s Hospital Colorado
- Publication date: 2020-12-31
- Collection: 2020 FAST Educational Summit