title: Uptake of health monitoring and disease self-management in Australian adults with neurofibromatosis type 1: strategies to improve care.
abstract: Lifelong health monitoring is recommended in neurofibromatosis type 1 (NF1) because of the progressive and unpredictable range of disabling and potentially life-threatening symptoms that arise. In Australia, strategies for NF1 health surveillance are less well developed for adults than they are for children, resulting in inequalities between pediatric and adult care. The aims of this study were to determine the uptake of health monitoring and capacity of adults with NF1 to self-manage their health. Australian adults with NF1 (n = 94, 18-40 years) participated in a semi-structured interview. Almost half reported no regular health monitoring. Thematic analysis of interviews identified four main themes as to why: (i) did not know where to seek care, (ii) unaware of the need for regular monitoring, (iii) futility of health monitoring as nothing can be done for NF1, and (iv) feeling healthy, therefore monitoring unnecessary. Overall, there were low levels of patient activation, indicating that adults with NF1 lacked knowledge and confidence to manage their health and health care. Findings are discussed in the context of service provision for adults with NF1 in New South Wales, Australia.
authors.affiliation: Discipline of Paediatrics and Child Health, The University of Sydney, Sydney, New South Wales, Australia.|The Institute for Neuroscience and Muscle Research, Westmead, New South Wales, Australia.
authors.forename: H A
authors.initials: HA
authors.lastname: Crawford
authors.affiliation: Discipline of Paediatrics and Child Health, The University of Sydney, Sydney, New South Wales, Australia.|Children's Hospital Education Research Institute (CHERI), The Children's Hospital at Westmead, Westmead, New South Wales, Australia.
authors.forename: B
authors.initials: B
authors.lastname: Barton
authors.affiliation: Department of Genetic Medicine, Westmead Hospital, Westmead, New South Wales, Australia.|Discipline of Genetics, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: M J
authors.initials: MJ
authors.lastname: Wilson
authors.affiliation: Department of Clinical Genetics, Royal North Shore Hospital, St Leonards, New South Wales, Australia.|Discipline of Medicine, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Y
authors.initials: Y
authors.lastname: Berman
authors.affiliation: School of Pharmacy and Pharmaceutical Sciences, Ulster University, Coleraine, Northern Ireland, UK.
authors.forename: V J
authors.initials: VJ
authors.lastname: McKelvey-Martin
authors.affiliation: School of Pharmacy and Pharmaceutical Sciences, Ulster University, Coleraine, Northern Ireland, UK.|Northern Ireland Regional Genetics Service, Department of Medical Genetics, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK.
authors.forename: P J
authors.initials: PJ
authors.lastname: Morrison
authors.affiliation: Murdoch Childrens Research Institute, Royal Children's Hospital, Parkville, Victoria, Australia.
authors.forename: K N
authors.initials: KN
authors.lastname: North
mesh_terms: D000293:Adolescent; D000328:Adult; D001315:Australia; D059026:Diagnostic Self Evaluation; D019468:Disease Management; D005260:Female; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000628:therapy; D012648:Self Care; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: adults; health monitoring; neurofibromatosis 1; patient activation; self-management