The Care and Management of Children and Young People with Ataxia Telangiectasia Provided by Nurses and Allied Health Professionals: a Scoping Review

Khan, Munira; Cassidy, Elizabeth; Parkin, Tracey; Wallace, Amanda; Carter, Bernie; Paton, Joanne; Donohue, Kathryn; Mitchell, Susan; Quin, Gemma; McNarry, Nicola; Hartley, Helen; Bailey, Hannah; Whitehouse, William; Medd, Rachel; Zahidi, Asma; McMullan, Miriam; Bunn, Lisa

Abstract

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.

Citation

Khan, M., Cassidy, E., Parkin, T., Wallace, A., Carter, B., Paton, J., …Bunn, L. (2023). The Care and Management of Children and Young People with Ataxia Telangiectasia Provided by Nurses and Allied Health Professionals: a Scoping Review. Cerebellum, https://doi.org/10.1007/s12311-023-01555-z

• Journal Article Type: Review
• Acceptance Date: Mar 25, 2023
• Online Publication Date: Apr 29, 2023
• Publication Date: Apr 29, 2023
• Deposit Date: May 11, 2023
• Publicly Available Date: May 11, 2023
• Journal: Cerebellum
• Print ISSN: 1473-4222
• Electronic ISSN: 1473-4230
• Publisher: Springer
• Peer Reviewed: Peer Reviewed
• DOI: https://doi.org/10.1007/s12311-023-01555-z
• Keywords: Ataxia telangiectasia · Cerebellar ataxia · Community health services · Child care · Rehabilitation · Multidisciplinary management
• Public URL: https://nottingham-repository.worktribe.com/output/20563253
• Publisher URL: https://link.springer.com/article/10.1007/s12311-023-01555-z
• Additional Information: Accepted: 25 March 2023; First Online: 29 April 2023; : ; : Not applicable.; : The scoping review lead, Munira Khan, received a PhD studentship from Action for A-T to support the undertaking of this project. The authors declare that they have no competing interests.; Free to read: This content has been made available to all.