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

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.

A regional evaluation of the health care utilization and outcomes of children and young people with long-term ventilation needs.

BACKGROUND: Globally, the number of children and young people (CYP) with long-term ventilation (LTV) needs is increasing, with high associated health care costs, due to frequent hospital admissions and contact with community health care services. However, demographic, health care utilization and outcome details of the CYP cared for locally is unknown. This study aimed to examine health care utilization and outcomes for this patient population. METHODS: Routinely collected data from 2014 to 2018 were extracted from local LTV team records and from hospital electronic patient records. Descriptive and inferential statistical analysis was performed using SPSS 17. RESULTS: A total of 112 CYP aged 0-17 years old were included in the evaluation. Sixty per cent (n = 67) commenced ventilation in hospital, and 62% (n = 69) had at-least one hospitalization event whilst they were on LTV, with a median length of stay of 3 days. Most hospitalizations were unplanned and respiratory in nature. Ninety-five per cent (n = 106) of CYP accessed at least one clinic appointment whilst on LTV, with a median of 20 outpatient clinic appointments during the study period. The majority of CYP received time-intensive support from LTV nurses and physiotherapists during the period that they received LTV. Minimal seasonal variation existed in relation to hospital admissions. Year on year increasing trend of hospital admissions was noted. The observed mortality rate was 3.6% (n = 4), 72.3% (n = 81) remained active on LTV, 14% (n = 16) were liberated from their ventilation and 9% (n = 10) transitioned to adult care by the end of the study. CONCLUSION: The study highlights the most common modes of health care utilization for CYP with LTV needs. To enable formalization of future resource planning and accurate assessment of health care utilization in evaluations, there is an urgent need to create a systematic approach for relevant LTV data collection.