Mitochondrial disease results from failure of the mitochondria
Mitochondrial disease results from failure of the mitochondria, specialised compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. Cell injury and even cell death follow. If this process is repeated throughout the body, whole systems begin to fail, and the life of the person in whom this is happening is severely compromised. The disease primarily affects children, but adult onset is becoming more and more common.
Diseases of the mitochondria appear to cause the most damage to cells of the brain, heart, liver, skeletal muscles, kidney and the endocrine and respiratory systems.
Depending on which cells are affected, symptoms may include loss of motor control, muscle weakness and pain, gastro-intestinal disorders and swallowing difficulties, poor growth, cardiac disease, liver disease, diabetes, respiratory complications, seizures, visual/hearing problems, lactic acidosis, developmental delays and susceptibility to infection.
Tiana Honey Watson had Mitochondrial Disorder Complex 1. Visually she was a beautiful and perfect little girl and appeared to be glowing with health but the disease had affected her muscles, heart, lactic acid levels and respiratory system. Tiana died on the 9th May 2006 of total respiratory failure. She was 2 years and 7 months old. At present there is no cure or treatment for Mitochondrial Disease.
The Tiana Honey Watson Fund for Children with Rare & Metabolic Disorders has been set up to raise money to spend on research and to help find a cure for this ever-growing deadly disease and also to help families of children with similar metabolic disorders.
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