Spinal Muscular Atrophy
About the disease
Spinal Muscular Atrophy (SMA) is a inherited neurological disease. The disease is in the spinal cord and causes the deterioration of the muscles that stimulate and controls by the spinal nerve cells which are not in place or not functioning as normal. It can debut already at birth, in early childhood or in during teenage years, and it can have very different degrees of severity. The different types of SMA and how they progress without treatment is as follows.
This is the most severe form of SMA. Children born with the disease are normally diagnosed before they are 6 months old. The symptoms are prounounced lethargy and weakness that effects the breathing functions and the chest’s development and form. Nutrition issues can develop, when swallowing and chewing muscles are at risk of being weakened. In these cases the disease will debug before 3 months of age, these children live for a very short period. If the disease debuts later than this then the lifespan can be considerably longer if there is helper methods applied with regards to nutrition and breathing. Since the muscle power and strength is so weakened, the person with this disease is lying down.
SMA type II debuts symptoms and signs in children between 7-18 months of age. Children with this disease usually learn to sit themselves and maybe crawl, but can never walk and have to use an electric wheelchair. There is impaired strength in nearly all of the muscles, mostly in the body, shoulder and hip muscles, less so in the hands and feet. Expected life-expectancy with this group is hard to pinpoint, as it is dependent on lung complications that the child may have and the treatment of them. The muscle strength changes little during lifetime, but there is a risk that the person will lose abilities after infections and longer illness.
This is the mildest form and debuts from 18 months of age all the way up to young adult. Children with these disease achieve more in their motor development. The majority learn to walk, but development of the disease leads to most needing a wheelchair during the first school years.