Study says LGS questionnaire design may not capture family needs
Sleep, socialization among measures focus groups say need better assessment
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Researchers use questionnaires to assess behavior, communication, and other aspects of life with LGS. (Photo from iStock)
Questionnaires used in research to assess behavior, communication, and quality of life in people with Lennox-Gastaut syndrome (LGS) may not always capture the concerns that matter most to patients and their families, a study found.
In focus groups involving caregivers, clinicians, and advocates, participants identified several areas they felt existing assessments overlooked, including sleep, gastrointestinal health, caregiver well-being, socialization, and safety. They also said completing the forms could be a frustrating and burdensome experience.
“These insights underscore the need for relevant, inclusive tools that are aligned with the lived experiences of individuals with LGS and their families,” the researchers wrote.
The study, “Views of caregivers, clinicians, and epilepsy advocacy group representatives on survey instruments used to assess behavior, communication, and quality of life in individuals with Lennox-Gastaut syndrome,” was published in Epilepsy Research.
LGS is a severe form of epilepsy that usually begins in early childhood. LGS symptoms include several types of seizures, along with a range of cognitive and developmental issues.
Measuring quality of life
More than 90% of people with LGS develop treatment-resistant epilepsy, meaning seizures that are hard to control and continue despite treatment. Although reducing seizures is often a primary goal in LGS clinical studies, families have emphasized that changes in behavior, communication, and quality of life also matter.
Researchers in the U.S. sought feedback from caregivers, clinicians, and epilepsy advocates on the relevance, feasibility, and burden of existing questionnaires used to assess behavior, communication, and quality of life in people with LGS. The work was designed to help select caregiver-reported measures for a larger multicenter study comparing epilepsy surgery with additional anti-seizure medications for LGS.
They held four online focus groups with 22 caregivers, five epilepsy advocacy group representatives, and six clinicians. The LGS Foundation facilitated the recruitment of caregivers, while advocates and clinicians were invited from the stakeholder advisory board.
Among the 20 caregivers, 90% were women and 85% were white. Of the six clinicians, two specialized in pediatric epilepsy, two in pediatric neurosurgery, one in pediatric emergency medicine, and one in developmental and behavioral psychology.
Caregivers described repeatedly answering questions about things their child cannot do as frustrating, exhausting, and sometimes discouraging. Long questionnaires posed another challenge, particularly for caregivers balancing work and having substantial care responsibilities.
Some participants said they would prefer to receive questionnaires before medical appointments so they could complete them at home rather than while simultaneously caring for their child in a clinic.
Participants also said key areas remained unaddressed in existing questionnaires. Sleep and gastrointestinal problems were among the most frequently mentioned. Feeding difficulties, safety in a home setting, social interaction, access to support, and caregiver stress and burnout were also identified as important gaps.
Clinicians reported that survey tools could help track needs over time and identify which professionals, such as social workers or counselors, should be involved in a patient’s care and support.
“Identifying these content gaps prioritized by caregivers, clinicians, and advocates is a key contribution of this work and can inform the development of more comprehensive outcome measurement in LGS,” the researchers wrote.
Reviewing questionnaires
The researchers also sought feedback on participants’ views of questionnaires currently in use.
For behavior, they reviewed three tools: the Aberrant Behavior Checklist (ABC), the Adaptive Behavior Assessment System-3 (ABAS-3), and the Vineland Adaptive Behavior Scales-3.
The ABC received the most positive feedback, with participants noting that it was relatively concise, easy to use, and relevant to behaviors commonly encountered in LGS.
“I liked the ABC because it [covered] ‘what are the things that you are struggling with right now? What are the behaviors that you’re seeing right now that you’re dealing with?’ So, I feel like it’s just more relevant [to my child] in that sense,” one caregiver said.
The ABAS-3 and Vineland-3 drew more concerns. Participants said the questionnaires could be lengthy and some questions did not match the developmental capabilities of people with LGS.
Two communication tools were evaluated: the Communication Matrix (CM) and the Communication and Symbolic Behavior Scales (CSBS). The CM was generally preferred over the CSBS. Participants said the CM better captured nontraditional forms of communication, such as gestures, facial expressions, and body movements. That flexibility was particularly important for people who do not use spoken language.
One caregiver whose son is nonverbal said they could answer more questions accurately using the CM. In contrast, participants said the CSBS did not properly capture nonverbal or alternative forms of communication.
Participants also considered two quality-of-life measures: the Quality of Life Inventory-Disability (QI-Disability) and the CDKL5 Deficiency Disorder Severity Assessment (CDD-SA).
There was no clear preference between the two. Some caregivers liked QI-Disability for its simplicity and for reflecting day-to-day concerns, while others valued the CDD-SA for its depth and attention to functional abilities.
Future work is needed to refine and validate measures that are relevant, inclusive, and feasible for use in the LGS population, the researchers concluded.
