Authors: Reymundo Lozano, Talia Thompson, Jayne Dixon-Weber, Craig A. Erickson, Elizabeth Berry-Kravis, Sara Williams, Elizabeth Smith, Jean A. Frazier, Hilary Rosselot, Cristan Farmer, and David Hessl
Summarized by Jayne Dixon-Weber
Most caregivers report that individuals with fragile X syndrome (FXS) have high levels of anxiety. However, anxiety is challenging to measure because most individuals with FXS cannot state themselves that they are anxious or have anxiety. The NFXF conducted a comprehensive survey of the presence, frequency, and duration of what caregivers observed when they said the individual was anxious; the survey was completed by caregivers of 456 individuals with FXS, ages 2–81 years, and 24 female and 2 male FXS self-advocates, ages 15–66 years. Caregivers reported classic observable indicators of anxiety, such as avoidance, irritability, agitation, repetitive behavior, aggression, and self-injury. Self-advocate accounts largely paralleled caregiver data.
The results determined that caregivers are capable of observing and reporting behaviors that are valid indicators of anxiety that are usually reported in self-report standardized assessments. This was an important first step in addressing parents’ report that anxiety is one of the primary areas of concern in the individual with FXS. The next step is to develop an anxiety measure for FXS that can be used in future studies.
Why This Matters
Most individuals with FXS cannot state themselves that they are anxious and self-report is needed in current standardized assessments. The information analyzed in this study will result in the development of a measure where observable and quantifiable data on anxiety in those with FXS can become an outcome measure to be used in future research/trials.
Based on the results of this survey, members of the steering committee of the CDC-funded FORWARD-MARCH project will develop an anxiety questionnaire that will be administered as part of the study protocol.
FORWARD-MARCH is the next phase of FORWARD, a natural history study that allows specialty clinics across the U.S. to work together and gather important information about people of all ages with FXS.
We thank all the families and self-advocates who participated in this study, and the National Fragile X Foundation.