Gross motor development in children with epidermolysis bullosa.

BACKGROUND: Epidermolysis bullosa (EB) is a group of rare, congenital skin disorders, characterized by skin fragility and formation of blisters. The gross motor outcomes of children with EB are not known. OBJECTIVES: The primary objective of the study was to measure the proportion of gross motor delay in children with EB. The secondary objectives were to measure the difference in gross motor outcomes between EB sub-types and change in gross motor outcomes over time. METHODS: Children with EB, aged between one month and five and a half years of age, attending the Sydney Children's Hospital, Epidermolysis Bullosa Clinic, were eligible. Carers completed Ages and Stages Questionnaires, Third Edition, on behalf of their children. Questionnaires were scored, and outcomes were compared to age-expected norms. RESULTS: There were 24 participants to complete a questionnaire. Eleven participants completed additional questionnaires over the 24 month study duration. The proportion of children with EB with gross motor delay was greater than age-expected norms (29.17% vs. 2.5%). The delay occurred in children with recessive dystrophic (80%) and epidermolysis bullosa simplex (33.33%) sub-types, but not dominant dystrophic (0%). No children with Junctional EB or Kindler EB joined this study. CONCLUSIONS: This study demonstrates a difference in gross motor outcomes in children with EB. Children with recessive dystrophic and epidermolysis bullosa simplex should be prioritized for monitoring of, and intervention for, gross motor outcomes through multidisciplinary care. Further research investigating long-term outcomes for children with EB and the effectiveness of interventions would be beneficial.

Impacts of paediatric chronic pain on parents: A qualitative study.

BACKGROUND: Paediatric chronic pain adversely impacts the child's functioning, health-related quality of life and development. However, there is a need for a holistic assessment of parental impacts of caring for a child with chronic pain. This qualitative study aimed to investigate the possible psychosocial, functional and work impacts of caring for a child with chronic pain on parents, including any positive effects of the experience. METHODS: Ten parents (eight mothers and two fathers), whose child attended the Sydney Children's Hospital Interdisciplinary Chronic and Complex Pain Clinic, participated in semi-structured interviews exploring the impact of caring for a child with chronic pain. Interviews were audio-recorded, transcribed, coded (with good inter-coder agreement) and analysed using thematic analysis. RESULTS: The parental experience of caring for a child with chronic pain was encapsulated by four overarching themes: (1) 'the constant and all-consuming nature of pain' - parents described the unpredictable, yet constant nature of chronic pain, contributing to wide-reaching impacts in various areas of their life; (2) 'dealing with uncertainty' - their experience was commonly characterized by a sense of uncertainty, stress, hopelessness and fear; (3) 'importance of support and self-care' - strong support networks and prioritizing self-care were crucial in alleviating the negative effects of paediatric chronic pain; and (4) 'a revitalized and optimistic view on life and relationships' - some parents identified unique and positive effects, such as stronger relationships, personal growth and a reformed view on life. CONCLUSIONS: This study provided rich data on the various impacts of caring for a child with chronic pain, highlighting the need for the development of holistic, family-centred interventions addressing both child and parental functioning.

Caregiver Burden Associated With Pediatric Chronic Pain: A Retrospective Study Using the Pediatric Electronic Persistent Pain Outcomes Collaboration Database.

OBJECTIVES: This retrospective, cross-sectional study investigated the nature and extent of burden experienced by caregivers of children and adolescents with chronic pain, and factors associated with increased caregiver burden. METHODS: The Pediatric Electronic Persistent Pain Outcomes Collaboration database provided prospectively collected data from 1929 families attending 9 pediatric chronic pain services across Australia and New Zealand. Data included demographic information, responses to child pain and functioning measures, caregiver work impairment, and psychosocial functioning. RESULTS: Caregivers of children with chronic pain reported work impairment associated with their child's pain (mean: 15% ± SD 25 absenteeism; 38% ± SD 29 productivity lost), significantly worse than published international population norms (large-scale community survey data), most other caregiver samples of adults and children with other chronic conditions, and adult samples with various pain conditions. Caregivers reported considerable burden in multiple psychosocial functioning domains, particularly leisure functioning, pain-related catastrophizing, and adverse parenting behaviors (with greater pain-related avoidance). Caregiver psychosocial burden was significantly associated with child psychosocial functioning (ß = -0.308, P < 0.01), school absenteeism (ß = 0.161, P < 0.01), physical disability (ß = 0.096, P < 0.05), and pain duration (ß = 0.084, P < 0.05), but not pain intensity. Caregiver work productivity loss was significantly associated with school absenteeism (ß = 0.290, P < 0.01), child physical disability (ß = 0.148, P < 0.01), child health care utilization (ß = 0.118, P < 0.05), and worst pain intensity (ß = 0.101, P < 0.05). DISCUSSION: These results highlight the significant and varied impacts experienced by caregivers of children with chronic pain. This work is novel in reporting significant work impairment and confirms psychosocial burden in a larger sample than previous studies.

Somatosensory Testing in Pediatric Patients with Chronic Pain: An Exploration of Clinical Utility.

We aimed to evaluate the utility of clinical somatosensory testing (SST), an office adaptation of laboratory quantitative sensory testing, in a biopsychosocial assessment of a pediatric chronic somatic pain sample (N = 98, 65 females, 7-18 years). Stimulus-response tests were applied at pain regions and intra-subject control sites to cutaneous stimuli (simple and dynamic touch, punctate pressure and cool) and deep pressure stimuli (using a handheld pressure algometer, and, in a subset, manually inflated cuff). Validated psychological, pain-related and functional measures were administered. Cutaneous allodynia, usually regional, was elicited by at least one stimulus in 81% of cases, most frequently by punctate pressure. Central sensitization, using a composite measure of deep pressure pain threshold and temporal summation of pain, was implied in the majority (59.2%) and associated with worse sleep impairment and psychological functioning. In regression analyses, depressive symptoms were the only significant predictor of pain intensity. Functional interference was statistically predicted by deep pressure pain threshold and depressive symptoms. Manually inflated cuff algometry had comparable sensitivity to handheld pressure algometry for deep pressure pain threshold but not temporal summation of pain. SST complemented standard biopsychosocial assessment of pediatric chronic pain; use of SST may facilitate the understanding of disordered neurobiology.