Respiratory morbidity in children with cerebral palsy: an overview.

Respiratory problems have a significant impact on morbidity and mortality in patients with cerebral palsy (CP). In particular, recurrent aspiration, impaired airway clearance, spinal and thoracic deformity, impaired lung function, poor nutritional status, and recurrent respiratory infections negatively affect respiratory status. Bronchopulmonary dysplasia may contribute to pulmonary problems, but asthma is not more common in CP than in the general population. We discuss treatment options for each of these factors. Multiple coexisting and interacting factors that influence the respiratory status of patients with CP should be recognized and effectively addressed to reduce respiratory morbidity and mortality. WHAT THIS PAPER ADDS: Respiratory problems are a significant cause of morbidity in patients with cerebral palsy (CP). Respiratory status in patients with CP is influenced by recurrent aspiration and impaired airway clearance. Spinal and thoracic deformity, impaired lung function, poor nutrition, and respiratory infections also negatively affect respiratory status. These factors should all be addressed to reduce respiratory problems in patients with CP.

MORBILIDAD RESPIRATORIA EN NIÑOS CON PARÁLISIS CEREBRAL: UNA VISIÓN GENERAL: Los problemas respiratorios tienen un impacto significativo sobre la morbilidad y mortalidad en pacientes con parálisis cerebral (PC). En particular, aspiraciones recurrentes, incapacidad para despejar la vía aérea, deformidades de la columna y del tórax, deterioro de la función pulmonar, un pobre estado nutricional e infecciones pulmonares recurrentes, afectan negativamente el estado respiratorio. La displasia broncopulmonar puede contribuir a estos problemas pulmonares, pero el asma no es más común en PC que en la población general. Discutimos las opciones terapéuticas para cada uno de estos factores. La coexistencia e interacción de múltiples factores que influencian el estado respiratorio de las pacientes con PC deben ser reconocidos y tratados efectivamente para reducir la morbilidad respiratoria y la mortalidad.

MORBIDADE RESPIRATÓRIA EM CRIANÇAS COM PARALISIA CEREBRAL: UM PANORAMA: Problemas respiratórios têm impacto significativo na morbidade e mortalidade em pacientes com paralisia cerebral (PC). Em particular, aspiração recorrente, limpeza deficiente das vias aéreas, deformidade espinhal e torácica, função pulmonar deficiente, pobre estado nutricional, e infecções respiratórias recorrentes podem afetar o estado respiratório negativamente. A displasia broncopulmonar pode contribuir para problemas pulmonares, mas a asma não é mais comum em PC do que na população geral. Nós discutimos opções de tratamento para cada um destes fators. Múltiplos fatores coexistentes ou que interagem e que influenciam o estado respiratório de pacientes com PC devem ser reconhecidos e efetivamente abordados para reduzir a morbidade e mortalidade respiratória.

Airway clearance techniques in neuromuscular disorders: A state of the art review.

This is a unique state of the art review written by a group of 21 international recognized experts in the field that gathered during a meeting organized by the European Neuromuscular Centre (ENMC) in Naarden, March 2017. It systematically reports the entire evidence base for airway clearance techniques (ACTs) in both adults and children with neuromuscular disorders (NMD). We not only report randomised controlled trials, which in other systematic reviews conclude that there is a lack of evidence base to give an opinion, but also include case series and retrospective reviews of practice. For this review, we have classified ACTs as either proximal (cough augmentation) or peripheral (secretion mobilization). The review presents descriptions; standard definitions; the supporting evidence for and limitations of proximal and peripheral ACTs that are used in patients with NMD; as well as providing recommendations for objective measurements of efficacy, specifically for proximal ACTs. This state of the art review also highlights how ACTs may be adapted or modified for specific contexts (e.g. in people with bulbar insufficiency; children and infants) and recommends when and how each technique should be applied.

Cough Augmentation in Subjects With Duchenne Muscular Dystrophy: Comparison of Air Stacking via a Resuscitator Bag Versus Mechanical Ventilation.

BACKGROUND: Air stacking improves cough effectiveness in people with Duchenne muscular dystrophy (DMD) and respiratory muscle weakness. However, it is not known whether air stacking is more effective via a resuscitator bag or a home ventilator. METHODS: This prospective randomized study investigated the effect of air stacking via a volume-cycled home ventilator versus via a resuscitator bag in participants with DMD. Maximum insufflation capacity and peak expiratory flow during spontaneous (cough peak flow) and air stacking-assisted cough maneuvers (air stacking-assisted cough peak flow) were measured. RESULTS: Fifty-two adult DMD subjects receiving noninvasive ventilation were included in the study: 27 participants performed air stacking via their home ventilator (home-ventilator group; age = 25.3 ± 5.1 y; forced vital capacity (FVC) = 809 ± 555 mL), and 25 participants used a resuscitator bag (resuscitator-bag group; age = 24.7 ± 5.7 y, FVC = 807 ± 495 mL). Following a single training session, air stacking could be performed successfully by 89% (home ventilator) and 88% (resuscitator bag) of participants. There were comparable maximum insufflation capacities (1,481 mL for the home-ventilator group vs 1,344 mL for the resuscitator-bag group, P = .33) and mean air stacking-assisted cough peak flow values (199 L/min for the home-ventilator group vs 186 L/min for the resuscitator-bag group, P = .33) between techniques. Air stacking-assisted cough peak flow increased significantly compared with baseline in both groups (mean increase: +51% [home ventilator] vs +49% [resuscitator bag], P < .001), with individual air stacking-assisted cough peak flow improvements ranging from -20 to 245%. CONCLUSIONS: Cough augmentation is an important component of the respiratory management of people with a neuromuscular disorder. No difference in cough effectiveness as measured by air stacking-assisted cough peak flow was found in air stacking via a ventilator compared with via a resuscitator bag. Both methods achieved mean air stacking-assisted cough peak flow values of >160 L/min. Provision of an inexpensive resuscitator bag can effectively improve cough capacity, and it is simple to use, which may improve access to respiratory care in people with DMD.