Six year old Shelby loves one-on-one time. She likes to interact with people and be read to. She smiles from the moment she wakes up until she goes to bed at night. If she hears someone come into the house that doesn’t come to see her, she will fuss until they do. She also loves to feel the wind and being outside where she enjoys her bird feeders and just watching things move around her since she is not mobile.
Shelby’s diagnosis is Phontocerebellar Hypoplasia (PCH), a group of related conditions that affect the development of the brain. PCH is characterized by impaired brain development, delayed development, problems with movement, and intellectual disability. In addition to the brain abnormalities, PCH1 causes problems with muscle movement resulting from a loss of specialized nerve cells in the spinal cord. Individuals with PCH1 also have very weak muscle tone, joint deformities, vision impairment, and breathing and feeding problems. Most live only into childhood but life expectancy ranges to the mid twenties.
Shelby’s family is making their home more accessible with a ramp, bath chair, and other modifications to ensure that her daily quality of life is maintained and that she is in a safe environment that meets her needs. Her father can no longer lift more than 25 pounds and Shelby has surpassed that weight. One piece of the modification that her family needs assistance with is a backup generator for her. This would not be a whole home generator but one that will allow for her needs to be maintained in the case of lost power.
Generator & installation - $9800