Özet:

Spinal muscular atrophy (SMA) is a neuromuscular inherited disease that often progresses with autosomal recessive progressive degeneration. SMA is the most common genetic cause of premature infant death. While it is seen in approximately one in 6.000-10.000 live births in the world, its incidence is approximately 1/6.000 live births in our country. In SMA, degeneration of alpha motor neurons in the anterior horn cells of the spinal cord affects the voluntary muscles, causing proximal symmetric weakness and atrophy of the extremities and body. Although the quality of life in SMA, which has four clinical types, differs according to the type, children become fully dependent due to muscle atrophy due to advanced degeneration in Type 1 and Type II forms, which are common. Although some drugs are used for the treatment of SMA in the world and our country, there is no cure for the disease yet. For this reason, it is important that children with SMA and their families/parents are supported in the care of their children and that they are educated about their children's care needs. Nurses have an important role in the care of children with SMA and their families. In the literature review, it is seen that the publications on SMA treatment and care are very limited in our country. SMA has emerged as a current problem in our country in recent years. In this review, SMA disease, its treatment and nursing care are discussed.

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