[Treatment of respiratory disturbance in children with severe physical disabilities to improve their quality of life].

Children with severe physical disabilities frequently have respiratory problems which affect their quality of life (QOL). They commonly stem from central nervous system dysfunctions and/or severe motor disabilities, and consist of various impairments deriving primarily from central and motor dysfunctions, such as dysmyotonia, deformation, dysphagia, and gastro-esophageal reflux (GER), which often influence each other and result in respiratory insufficiency without adequate interventions. Aging is also an important factor to worsen respiratory involvements and to change their underlying pathophysiology gradually, even if the primary cause of the disability is non-progressive. A key to effective treatment and improvement of their QOL is to understand the pathophysiology. Evaluation is primarily based on regular physical examination. Other examinations include blood sampling to examine inflammation, nutritional state and blood gas analysis, round-the-clock SpO2 and TcPCO2 or EtCO2 monitoring, chest X ray and computed tomography, polysomnography, laryngoscopy, bronchoscopy, evaluation of swallowing function, and evaluation of GER. Postural control, relaxation, respiratory physiotherapy, and treatment of dysphagia are the most important and common therapeutic procedures. Treatment of GER, treatment of upper airway obstruction, oxygen therapy, tracheostomy, or mechanical ventilation may be needed. It is also important to take into account the possibility that the treatment procedures themselves could affect the patient's QOL. Counseling should be performed before and throughout the treatment process about how patient's and family's everyday life will be influenced positively and negatively by the treatment, and a multidisciplinary team should support all aspects of their needs.