Objective: To create structured interviews for the UHDRS ’99 functional scales that can be remotely administered to caregivers of Huntington’s disease gene expansion carriers (HDGECs) in later stages of illness.

Background: Huntington’s disease (HD) is a genetic neurodegenerative disorder resulting in relentless loss of cognitive, motor, and functional abilities, and eventually, death. The functional scales of UHDRS’99 are commonly used to assess functional decline in HD. Information regarding functional status in later stages of illness is lacking due to inability of HDGECs to provide information, as well as attend in-person appointments. Remotely administered validated caregiver scales are needed to improve our understanding of functional status in later stage HD.

Method: The HD-SIF is composed of interviews for the UHDRS ‘99 Total Functional Capacity (TFC), Functional Assessment (FA), and Independence Scale (IS) designed to be administered remotely to caregivers. The SIF questions were derived from the Examination Guidance for the UHDRS ’99 and Shoulson’s original TFC scale description, and revised following review by a panel of HD experts.

Results: The interviews for the TFC and FA are structured while that for IS is semi-structured. In the structured interviews for the FC and FA, each UHDRS item has a corresponding question set with branching logical structure that the interviewer progresses through based on caregiver responses and terminates in an assigned score for that item. The number of questions in each set varies across UHDRS items. The semi-structured interview for the IS contains 20 questions, each question is administered only if the interviewer does not have enough information to score it. Based on the information from this interview, the interviewer assigns an IS rating. In practice, across HD-SIF interviews, the number questions administered will vary based on interviewees’ responses.

Conclusion: The scoring system on the UHDRS scales and the SIF questionnaires being similar, will convey comparable information about the late stage HDGEC’s functional status. The HD-SIF will undergo clinimetric evaluation in the Later Stage HD Assessments study which is being planned. Ratings obtained using the UHDRS ’99 and the HD-SIF will be compared. Classical Test Theory and Item Response Theory will be used to evaluate the HD-SIF’s item- and scale-level performance.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-huntingtons-disease-structured-interview-of-function-hd-sif-a-remotely-administered-caregiver-interview-for-later-stage-hd/