Using the Bayley-4 and Vineland-3 in Angelman syndrome: barriers, solutions, and challenging items

Abstract Objective The Bayley Scales of Infant and Toddler Development- 4th Edition (Bayley-4) and Vineland Adaptive Behavior Scales – 3rd Edition (Vineland-3) are outcome measures often considered as primary endpoints in clinical trials for Angelman syndrome (AS). We explored barriers encountered w...

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Main Authors: Sean N. Halpin, Sarah Nelson Potter, Angela Gwaltney, Anjali Sadhwani, Katherine C. Okoniewski, Anne C. Wheeler
Format: Article
Language:English
Published: BMC 2025-06-01
Series:Orphanet Journal of Rare Diseases
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Online Access:https://doi.org/10.1186/s13023-025-03817-x
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author Sean N. Halpin
Sarah Nelson Potter
Angela Gwaltney
Anjali Sadhwani
Katherine C. Okoniewski
Anne C. Wheeler
author_facet Sean N. Halpin
Sarah Nelson Potter
Angela Gwaltney
Anjali Sadhwani
Katherine C. Okoniewski
Anne C. Wheeler
author_sort Sean N. Halpin
collection DOAJ
description Abstract Objective The Bayley Scales of Infant and Toddler Development- 4th Edition (Bayley-4) and Vineland Adaptive Behavior Scales – 3rd Edition (Vineland-3) are outcome measures often considered as primary endpoints in clinical trials for Angelman syndrome (AS). We explored barriers encountered when administering these instruments to individuals with AS and associated guidance for their use in trials and research studies. Methods We interviewed nine clinicians who have administered the Bayley-4 and/or the Vineland-3 to individuals with AS and analyzed their transcripts using a quasi-deductive analysis approach. Results Barriers to administering the Bayley-4 included participant’s lack of interest, overexcitement, emotional impact on caregiver, the mental workload of administering the Bayley-4, and environmental factors (e.g., administration setting). Barriers to administering the Vineland-3 included determining the most appropriate start point, emotional impact on caregiver, distractions, conflicting answers from two caregivers, and the mental workload of administering the Vineland-3. Participants provided potential solutions to each barrier. Lastly, we identified overarching item-level concerns for both the Bayley-4 (i.e., administration challenges, items not aligned with abilities) and the Vineland-3 (i.e., misalignment of assessment criteria and condition characteristics, limitations in observation and contextual understanding, requires specialized training). Conclusion Clinical trials often rely on the Bayley-4 and Vineland-3 assessments as outcome measures, yet our identified barriers threaten their validity. The associated solutions provide a path forward for improving administration of the Bayley-4 and Vineland-3 in clinical practice, research, and future trials focused on individuals with AS and other intellectual and developmental disabilities.
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spelling doaj-art-dfd4c31c766b44ecac55d0f1ff51f2482025-08-20T03:10:41ZengBMCOrphanet Journal of Rare Diseases1750-11722025-06-0120111310.1186/s13023-025-03817-xUsing the Bayley-4 and Vineland-3 in Angelman syndrome: barriers, solutions, and challenging itemsSean N. Halpin0Sarah Nelson Potter1Angela Gwaltney2Anjali Sadhwani3Katherine C. Okoniewski4Anne C. Wheeler5GenOmics and Translational Research Center, RTI InternationalGenOmics and Translational Research Center, RTI InternationalGenOmics and Translational Research Center, RTI InternationalDepartment of Psychiatry and Behavioral Sciences, Boston Children’s HospitalGenOmics and Translational Research Center, RTI InternationalGenOmics and Translational Research Center, RTI InternationalAbstract Objective The Bayley Scales of Infant and Toddler Development- 4th Edition (Bayley-4) and Vineland Adaptive Behavior Scales – 3rd Edition (Vineland-3) are outcome measures often considered as primary endpoints in clinical trials for Angelman syndrome (AS). We explored barriers encountered when administering these instruments to individuals with AS and associated guidance for their use in trials and research studies. Methods We interviewed nine clinicians who have administered the Bayley-4 and/or the Vineland-3 to individuals with AS and analyzed their transcripts using a quasi-deductive analysis approach. Results Barriers to administering the Bayley-4 included participant’s lack of interest, overexcitement, emotional impact on caregiver, the mental workload of administering the Bayley-4, and environmental factors (e.g., administration setting). Barriers to administering the Vineland-3 included determining the most appropriate start point, emotional impact on caregiver, distractions, conflicting answers from two caregivers, and the mental workload of administering the Vineland-3. Participants provided potential solutions to each barrier. Lastly, we identified overarching item-level concerns for both the Bayley-4 (i.e., administration challenges, items not aligned with abilities) and the Vineland-3 (i.e., misalignment of assessment criteria and condition characteristics, limitations in observation and contextual understanding, requires specialized training). Conclusion Clinical trials often rely on the Bayley-4 and Vineland-3 assessments as outcome measures, yet our identified barriers threaten their validity. The associated solutions provide a path forward for improving administration of the Bayley-4 and Vineland-3 in clinical practice, research, and future trials focused on individuals with AS and other intellectual and developmental disabilities.https://doi.org/10.1186/s13023-025-03817-xAngelman syndromeAssessmentNeurodevelopmental disordersRare diseases
spellingShingle Sean N. Halpin
Sarah Nelson Potter
Angela Gwaltney
Anjali Sadhwani
Katherine C. Okoniewski
Anne C. Wheeler
Using the Bayley-4 and Vineland-3 in Angelman syndrome: barriers, solutions, and challenging items
Orphanet Journal of Rare Diseases
Angelman syndrome
Assessment
Neurodevelopmental disorders
Rare diseases
title Using the Bayley-4 and Vineland-3 in Angelman syndrome: barriers, solutions, and challenging items
title_full Using the Bayley-4 and Vineland-3 in Angelman syndrome: barriers, solutions, and challenging items
title_fullStr Using the Bayley-4 and Vineland-3 in Angelman syndrome: barriers, solutions, and challenging items
title_full_unstemmed Using the Bayley-4 and Vineland-3 in Angelman syndrome: barriers, solutions, and challenging items
title_short Using the Bayley-4 and Vineland-3 in Angelman syndrome: barriers, solutions, and challenging items
title_sort using the bayley 4 and vineland 3 in angelman syndrome barriers solutions and challenging items
topic Angelman syndrome
Assessment
Neurodevelopmental disorders
Rare diseases
url https://doi.org/10.1186/s13023-025-03817-x
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