Predictors of family impact of juvenile localized scleroderma

Abstract

Objective To measure the impact juvenile localized scleroderma (jLS) has on family quality of life and to identify predictors of family impact in this population which may inform the development of tailored resources to enhance family functioning for patients with jLS. Methods A retrospective cohort study of pediatric patients with jLS and their families was conducted. Five questionnaires were administered at each visit: Pediatric Quality of Life Inventory Family Impact Module (PedsQL‐FIM), PedsQL 4.0 Generic Core Scales (PedsQL‐Generic), PedsQL Rheumatology Module (PedsQL‐RM), Child Health Assessment Questionnaire (CHAQ), and Children's Dermatology Life Quality Index (CDLQI). Linear mixed models with random intercepts for each patient were used to find relationships between family impact scores and clinically relevant variables over time. Variables of interest included disease activity status, methotrexate use, jLS distribution, and scores for PedsQL‐Generic and PedsQL‐RM. Results The median baseline PedsQL‐FIM total score was 80.9 (IQR = 76.6‐97.4). Adjusting for age and sex, the most significant predictors of family impact were PedsQL‐Generic scores and four of five PedsQL‐RM dimensions (all P < .001); methotrexate use had borderline significance (P = .06). Family impact increased more significantly over time in older patients. In multivariable modeling, PedsQL‐Generic total score and jLS “other” distribution were significant for predicting an increased PedsQL‐FIM score (P = .003 and P = .03, respectively). Conclusions JLS has a moderate family impact. Family impact is predicted by patients’ general and disease‐specific health‐related quality of life (HRQL) and their jLS subtype. There is a trend toward increased family impact with methotrexate treatment. This study emphasizes the importance of family‐centered care in jLS.

Systematic Review of Health‐Related Quality of Life Impact in Juvenile Localized Scleroderma

“…In Szczech et al, skin lesions present in the upper extremities and higher disease activity scoring (modified Localized Scleroderma Scoring Index) were associated with statistically significant lower HRQoL scoring; however, these data were not specific to the pediatric population 13 . Milovanova et al reported moderate impact of JLS on family life; however, this did not correlate with CDLQI scoring 14 …”

“…Treatment impacted HRQoL negatively in patients with JLS in both quantitative and qualitative studies (Table 4). 12,14,24,19 There were reports of negative experiences and negative comments related to medication use, hospital visits, missing school, blood draws, and drug‐related side effects in patients with truncal and limb JLS 17,24 . Nausea associated with subcutaneous MTX injections and weight gain related to the use of oral prednisone were reported as significantly burdensome in patients with facial JLS and their caregivers 24,25 .…”

“…Seven studies used the Children's Dermatology Life Quality Index (CDLQI), a 10-item scale that assesses the impact of skin diseases on QoL over a one-week period. 7,[13][14][15][16][17][18] These studies, which included a total of 231 patients (Table 1), reported little to no effect in HRQoL scoring in patients with JLS compared with healthy controls and other skin diseases, such as acne and eczema. Kunzler et al reported that median CDLQI scores decreased from 3 to 0 over a three-year follow-up period in their analyzed cohort.…”

“…13 Milovanova et al reported moderate impact of JLS on family life; however, this did not correlate with CDLQI scoring. 14 Two studies involved focus groups, one evaluating the impact of living with facial morphea and the second studying the burden of the skin symptoms, physical limitations, ECMs, body image, bullying/teasing, intrusive questioning, and treatment side effects. Higher levels of stress, decreased self-worth, "feeling different," impact from the visibility of skin changes, and a lack of social support were reported by patients with JLS in both studies.…”

“…25 MTX use showed high unidimensional scores in QoL scoring evaluating family impact. 14 Palmero et al published a cohort of 17 patients with facial lesions who had at least one surgical intervention done, including craniofacial plasty, fat injections, and implants and evaluated the psychosocial status after the interventions. 20 This study concluded that these surgical interventions are well tolerated in children and could be useful to mitigate QoL affection in patients with facial disfigurement.…”