There are two parts to this study. First, we want to determine the reliability
of a wearable device for sleep quality in RTT against the gold-standard
polysomnography. Additionally, we want to determine whether sleep quality
measured by the E4 device serves as a valid construct for sleep measurement
and is clinically meaningful when compared to existing clinical instruments in
RTT. These assessments will allow for direct comparison of the sleep
assessments using the gold standard PSG to those acquired using the E4.
Participation in this study takes 7 consecutive days. First, participants will
receive an in-person clinical evaluation with a child neurologist (either Dr.
Cary Fu or Dr. Jeff Neul) using the Motor Behavior Assessment. At this
clinical visit, your child will be fitted with an E4 device. Your child will
wear the E4 biosensor watch home and for the next 6 days at home. The
biosensor will monitor and record physiological signals in real time. It will
also measure Blood Volume Pulse, sympathetic nervous system arousal, local
skin temperature, as well as hand movements.
Families can choose to participate in a second optional component of this
study by participating in an overnight polysomnography (PSG) at the Vanderbilt
University Medical Center’s sleep lab, while the child is wearing the E4
watch. We will conduct a standard PSG protocol with monitoring of respiratory
effort, nasal airflow, blood oxygen saturation, heart rate, electrooculography
(EOG), surface electromyography (EMG), and EEG.
Caregivers will be asked to keep a daily sleep diary and complete the Child
Sleep Habits Questionnaire (CSHQ), the Rett Syndrome Behavior Questionnaire
(RSBQ), and the Child Health Questionnaire (CHQ). Caregivers will also
complete the SF-36 Questionnaire which is a 36-item parent quality of life
survey, which is designed to assess eight health concepts: physical
functioning, bodily pain, role limitations due to physical health problems,
role limitations due to personal or emotional problems, emotional well-being,
social functioning, energy/fatigue, and general health perceptions. A subset
of 22 participants will wear the E4 device during the 7th night while
concurrently undergoing a conventional polysomnogram at Vanderbilt University
Medical Center.