Abstract
BackgroundLong-term mechanical ventilation (LTV) with non-invasive ventilation (NIV) prolongs survival in patients with Neuromuscular Diseases (NMDs). Transition from paediatric to adult healthcare system is an undervalued and challenging issue for children with chronic conditions on mechanical ventilation.Methodsthis retrospective study aims to compare issues of young adults in age to transition to adult care (≥ 15 years old) affected by NMDs on NIV in two different Paediatric Respiratory Units in two different countries: Bambino Gesù Children’s Hospital, Research Institute, (Rome, Italy) (BGCH) and the Paediatric Respiratory Unit of the Royal Brompton Hospital (London, UK) (RBHT).ResultsThe median (min-max) age at starting ventilation was significantly different in the two groups (16 years old vs 12, p = 0.0006). We found significant difference in terms of median age at the time of observation (18 (15–22) vs 17 (15–19) years, p = 0.0294) and of type of referral (all the patients from the BGCH group were referred to paediatric services (n = 15, 100%), median age 18 (15–22); only 6 patients, in the RBHT group, with a median age 15.50 (15–17) years, were entirely referred to paediatric service). We found different sleep-disordered breathing assessments 6 full Polysomnographies, 7 Cardio-Respiratory Polygraphies and 2 oximetry with capnography (SpO2-tcCO2) studies in the BCGH group, while all patients of RBHT group were assessed with an SpO2-tcCO2 study. All patients from both groups underwent multidisciplinary assessment.ConclusionsIn conclusion, patients with NMDs on NIV in age to transition to adult require complex multidisciplinary management: significant efforts are needed to achieve the proper transition to adult care.
Highlights
Neuromuscular disorders (NMD) comprise a heterogeneous set of diseases which are mostly clinically evident during childhood
This study aims to provide a retrospective comparison between two different Paediatric Respiratory Units in tertiary care hospitals, in two different countries, that are dealing with young adult patients affected by NMD on non-invasive ventilation (NIV), focusing on their main issues
We provided a retrospective analysis of patients affected by NMD, evaluated in the last three years (2014–2016), on NIV and in the age of transition to adult care in two institutions: the Respiratory Unit in the Royal Brompton Hospital (London, UK) (RBHT) where a transition programme starting from the age of 15 years old is in place, and the Respiratory Unit Bambino Gesù Children Hospital, Research Institute (Rome, Italy) (BGCH), where there isn’t any transition plan
Summary
Neuromuscular disorders (NMD) comprise a heterogeneous set of diseases which are mostly clinically evident during childhood. Since the late 1990s the option of long term mechanical ventilation (LTV) support and the improvements in standards. Both non-invasive ventilation (NIV) and invasive mechanical ventilation (IMV) are therapeutic options that can prolong survival in patients with severe muscular weakness. Provision of long term non-invasive ventilation support and the population of children who survive reaching adulthood are exponentially increasing [6, 7]. The increase of life expectancy is having new and unexpected consequences. Long-term mechanical ventilation (LTV) with non-invasive ventilation (NIV) prolongs survival in patients with Neuromuscular Diseases (NMDs). Transition from paediatric to adult healthcare system is an undervalued and challenging issue for children with chronic conditions on mechanical ventilation
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
