Exploring the impact of paediatric localized scleroderma on health‐related quality of life: focus groups with youth and caregivers

Abstract

Background Paediatric localized scleroderma (LS) can negatively impact healthrelated quality of life (HRQoL) by causing skin fibrosis, abnormal limb development, disfigurement, and side-effects from immunosuppressive treatment. Studies to date have rarely included qualitative data gathered directly from paediatric patients with LS. Objectives To assess the impact of LS on HRQoL among affected youth and their caregivers using qualitative description. Methods Youth with all subtypes of LS and their caregivers were purposively sampled to participate in age-appropriate focus groups (younger children, early adolescents, adolescents). Each group started with a drawing exercise followed by in-depth discussion of topics including skin symptoms (e.g. itch, pain, tightness), functional impairment, physical appearance, family and peer relationships, and treatment burden. Focus groups were transcribed verbatim and co-coded, with adjudication of differentially applied codes. The study findings were triangulated via comparison with adult reports and published literature. Results Eleven youth aged 9-16 years and 16 caregivers participated in three focus groups each. Major identified areas of impact included uncomfortable skin symptoms, physical functioning limitations, extracutaneous manifestations, body image, bullying and teasing, unwanted questioning from others, and treatment side-effects and burden. Conclusions This is the first qualitative study of HRQoL in LS to include all major LS subtypes. We identified domains of HRQoL impacted by LS, some of which replicate earlier findings and some of which were novel. As impact also changed with developmental stage, our findings support the need for ongoing, formal evaluation of HRQoL in children and adolescents with LS. What is already known about this topic? • Paediatric localized scleroderma (LS) negatively impacts health-related quality of life (HRQoL) via skin fibrosis, musculoskeletal and other extracutaneous manifestations from the disease process, and side-effects of systemic immunosuppression. • The full impact of LS and its treatment on HRQoL is incompletely understood, with only one published qualitative study of youth with LS, which was limited to facial involvement. • There are no qualitative studies of HRQoL in other LS subtypes to date.

Parent Perspectives on Addressing Emotional Health for Children and Young Adults With Juvenile Myositis

“…Interestingly, this finding was common across age groups of school-age and teenage children, as well as young adults. Anxiety and uncertainty linger even in remission, highlighting the need for longterm psychosocial support and confirming prior studies in JM regarding the rollercoaster of emotional distress (5,15,22). Our findings also replicate those of prior studies demonstrating high emotional distress in parents caring for children with JM, juvenile-onset SLE, and JIA, as well as negative impacts on siblings and family relations (7,22,24).…”

“…Children with other chronic illnesses experience similar levels of life satisfaction as healthy children, although psychological stress is a negative predictor of such life satisfaction (28). Prior studies of patients with JM, their parents, and other pediatric rheumatic disease populations have also documented such post traumatic growth, although resiliency may wane with disease flares (5,7,15,22). While our findings add to emerging literature identifying resiliency in patients with JM, our study was not designed to identify specific family, parent, and child-level mechanisms contributing to resilient emotional health outcomes.…”

“…Investigators (KA, OA, DMW, EAC, and AMK) developed a semistructured interview guide and reviewed it with Cure JM Foundation board members. The interview guide was developed based on a literature review on emotional health in pediatric rheumatic diseases, including JM, a study (principal investigator AMK) of parent/patient perspectives on emotional health needs for pediatric rheumatology patients, including JM (14), and the adaptation of a focus‐group guide used in a prior study (principal investigator KA) on parent/patient perspectives on quality of life in pediatric localized scleroderma (15).…”

“…They know she can't go out in the sun very much, so instead of asking her, how can we make this work, they just don't ask her anymore. Pools or amusement parks, she's not invited [13][14][15][16][17]. I think probably lowered self-esteem is probably the one that's the most because she's currently still on steroids.…”

The importance of children and young person involvement in scoping the need for a paediatric glucocorticoid-associated patient reported outcome measure

“…Glucocorticoid medications offer potential for rapid clinical improvement, but frequently lead to side effects that adversely impact HRQOL. Many studies looking at the patient experience within paediatric rheumatic diseases, have found the burden of glucocorticoid treatment to be a recurring theme [ 14 – 17 ]. These range from short-term effects that may go unrecognised by clinicians (e.g., weight gain, anxiety, skin changes and poor sleep) to long-lasting adverse events (e.g., delayed growth and puberty, diabetes, loss of bone mass and fractures) [ 15 ].…”