Spinal Muskuler Atrofisi Olan Çocuğun Hemşirelik Bakımı

Abstract

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by defects in the survival motor neuron (SMN1) gene. Care and treatment are determinants of the patient’s prognosis and quality of life. In recent years, various drug, stem cell and genetic therapies as well as standards of care have been developed. When left untreated, the disease progresses with the destruction of motor neurons, resulting in muscle weakness, feeding and respiratory problems. In addition to drug treatment, in order to protect the health of children with SMA and improve their self-care skills, nursing approaches should be taken in cooperation with the family for various problems related to nutrition and weight management, orthopaedic and respiratory system. In this review, SMA disease and nursing approach are discussed. Keywords: Child, spinal muscular atrophy, nursing care.