Details

Autor(en) / Beteiligte

• Killian, J.T., MA
• Lane, J.B., RN, BSN
• Lee, H.-S., PhD
• Pelham, J.H., BS
• Skinner, S.A., MD
• Kaufmann, W.E., MD
• Glaze, D.G., MD
• Neul, J.L., MD, PhD
• Percy, A.K., MD

Titel

Caretaker Quality of Life in Rett Syndrome: Disorder Features and Psychological Predictors

Ist Teil von

• Pediatric neurology, 2016-05, Vol.58, p.67-74

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2016

Quelle

MEDLINE

Beschreibungen/Notizen

• Abstract Objective Rett syndrome (RTT) is a severe neurodevelopmental disorder affecting approximately one in 10,000 female births. The clinical features of RTT are known to impact both patients’ and caretakers’ quality of life (QOL) in RTT. We hypothesized that more severe clinical features would negatively impact caretaker physical QOL but would positively impact caretaker mental QOL. Methods Participants were individuals enrolled in the Rett Natural History Study with a diagnosis of classic RTT. Demographic data, clinical disease features, caretaker QOL, and measures of family function were assessed during clinic visits. The Optum™ SF-36v2® Health Survey was used to assess caretaker physical and mental QOL (higher scores indicate better QOL). Descriptive, univariate, and multivariate analyses were utilized to characterize relationships between child and caretaker characteristics and caretaker QOL. Results Caretaker physical component scores (PCS) were higher than mental component scores (MCS): 52.8 (9.7) versus 44.5 (12.1). No differences were noted between the baseline and 5-year follow-up. In univariate analyses, disease severity was associated with poorer PCS (p=0.006) and improved MCS (p=0.003). Feeding problems were associated with poorer PCS (p=0.007) and poorer MCS (p=0.018). In multivariate analyses, limitations in caretaker personal time and home conflict adversely affected PCS. Feeding problems adversely impacted MCS. Conclusions Caretaker QOL in RTT is similar to that for caretakers in other chronic diseases. Disease characteristics significantly impact QOL, and feeding difficulties may represent an important clinical target for improving both child and caretaker QOL. The stability of QOL scores between baseline and 5-years adds important value.