Functional independence of Taiwanese children with Prader–Willi syndrome

Abstract

Prader-Willi syndrome (PWS) is a genetic disorder with obesity, developmental delay, short stature, and behavioral abnormalities. The study aimed to assess the functional independence in children with PWS. The Functional Independence Measure for Children (WeeFIM) was used to evaluate 81 children with PWS (44 boys and 37 girls) with a median age of 11 years 1 month (range 2 years 8 months to 20 years 2 months) were recruited between January 2013 and December 2016. The mean total WeeFIM score was 103.8 (maximum 126). Sixty-five patients (80%) had deletion type PWS, 16 (20.0%) had nondeletion type. The scores were 103.6 ± 18.5 for deletion and 104.8 ± 18.3 for nondeletion type (p = .405), 104.8 ± 19.3 in boys and 102.6 ± 17.3 in girls (p = .293). The mean self-care, mobility, and cognition scores were 47 (maximum 56), 33 (maximum 35), and 24 (maximum 35), respectively. All total scores and 18 subscores in the three functional domains were positively correlated with age (p < .05). Most children required assistance in problem-solving, comprehension, and expression. The WeeFIM identified the strengths and limitations of children with PWS and confirmed that support and supervision were needed in cognitive and self-care tasks.

Functional Independence of Taiwanese Children with Osteogenesis Imperfecta

“…Among those tools, PEDI (Pediatric Evaluation of Disability Inventory) and WeeFIM are most frequently used and both had been validated in Chinese children with disabilities [ 14 , 46 ]. The PEDI instrument, composed of 197 questions, is more time-consuming and costly in the setting of an outpatient department in Taiwan, while the WeeFIM instrument is more convenient and showed reliability in our previous studies regarding functional independence in Taiwanese children with rare diseases [ 18 , 19 ].The WeeFIM is a reliable instrument to evaluate the functional status of children with disabilities. It was first validated in American children [ 47 ], and then used by Wong et al [ 14 ] and others [ 13 ] in similar studies.…”

“…The WeeFIM questionnaire was designed for primary caregivers to record their child’s abilities directly, and in 2002, Wong et al translated the questionnaire into Chinese. It has been used to estimate functional independence in children from 6 months to 7 years of age, and up to 21 years of age in those with developmental disabilities [ 17 , 18 , 19 , 20 ].…”

Association Between Pediatric Inpatient Rehabilitation Services and Children’s Functional Outcomes: King Fahad Medical City Experience

“…28 In general, through an interdisciplinary inpatient rehabilitation approach, children made significant changes from admission to discharge, which is supported by the previous studies that reported an overall change in outcomes after rehabilitation. 2,5,14,17,18,[29][30][31] Functional gains, as measured by the Wee-FIM instrument, vary depending on diagnoses, time from injury/diagnosis to rehabilitation admission, age, and LOS. The results of this study showed significant differences in gains across age and impairment groups, children older than 7 years made larger gains than younger children.…”