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dc.contributor.authorKhan, M
dc.contributor.authorCassidy, E
dc.contributor.authorParkin, T
dc.contributor.authorWallace, A
dc.contributor.authorCarter, B
dc.contributor.authorPaton, J
dc.contributor.authorDonohue, K
dc.contributor.authorMitchell, S
dc.contributor.authorQuin, G
dc.contributor.authorMcNarry, N
dc.contributor.authorHartley, H
dc.contributor.authorBailey, H
dc.contributor.authorWhitehouse, W
dc.contributor.authorMedd, R
dc.contributor.authorZahidi, A
dc.contributor.authorMcMullan, M
dc.contributor.authorBunn, L
dc.descriptionFile replaced (AAM) on 7.6.23 by NK (LDS)

Ataxia telangiectasia (A-T) is a rare, multisystem progressive condition that typically presents in early childhood. In the absence of cure, people with A-T require coordinated multidisciplinary care to manage their complex array of needs and to minimize the disease burden. Although symptom management has proven benefits for this population, including improved quality of life and reduced complications, there is a need for guidance specific to the nursing and allied healthcare teams who provide care within the community. A scoping review, adopting the Joanna Briggs Institute methodology, was undertaken. It aimed to identify and map the available expertise from nursing and allied healthcare and management of children and young people with A-T ≤ 18 years of age. A rigorous search strategy was employed which generated a total of 21,118 sources of evidence, of which 50 were selected for review following screening by experts. A range of interventions were identified that reported a positive impact on A-T-related impairments, together with quality of life, indicating that outcomes can be improved for this population. Most notable interventions specific to A-T include therapeutic exercise, inspiratory muscle training, and early nutritional assessment and intervention. Further research will be required to determine the full potential of the identified interventions, including translatability to the A-T setting for evidence related to other forms of ataxia. Large gaps exist in the nursing and allied health evidence-base, highlighting a need for robust research that includes children and young people with A-T and their families to better inform and optimize management strategies.

dc.publisherSpringer Science and Business Media LLC
dc.subjectAtaxia telangiectasia
dc.subjectCerebellar ataxia
dc.subjectCommunity health services
dc.subjectChild care
dc.subjectMultidisciplinary management
dc.titleThe Care and Management of Children and Young People with Ataxia Telangiectasia Provided by Nurses and Allied Health Professionals: a Scoping Review
plymouth.publication-statusPublished online
plymouth.journalThe Cerebellum
plymouth.organisational-group|Plymouth|Research Groups
plymouth.organisational-group|Plymouth|Faculty of Health
plymouth.organisational-group|Plymouth|Faculty of Health|School of Health Professions
plymouth.organisational-group|Plymouth|Research Groups|Institute of Health and Community
plymouth.organisational-group|Plymouth|REF 2021 Researchers by UoA
plymouth.organisational-group|Plymouth|Users by role
plymouth.organisational-group|Plymouth|Users by role|Academics
plymouth.organisational-group|Plymouth|REF 2021 Researchers by UoA|UoA03 Allied Health Professions, Dentistry, Nursing and Pharmacy
plymouth.organisational-group|Plymouth|Research Groups|FoH - Applied Parkinson's Research
dc.publisher.placeUnited States

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