Westley Groseclose is a typical boy very much like every other child his age. He enjoys Legos, video games and playing outside. Westley has always been physically slower than his classmates but never gives up trying to keep up with them. In September 2010 we discovered the source of his physical limitations. Westley was diagnosed with Duchenne Muscular Dystrophy. Duchenne is a form of muscular dystrophy which causes rapidly-worsening muscle weakness that starts in the legs and pelvis, and later affects the entire body including his heart and lungs. It is caused by a defective dystrophin gene (a protein in the muscles). This protein is like the chocolate chips of a cookie. Those without Duchenne are Chocolate Chip Cookies and boys like Westley are Sugar Cookies. Westley’s body is missing the “chips” that make and keep muscles strong. If you met of him in a crowd of people you wouldn’t know his chips are missing. However; over time this disease will begin to limit his activity. Our family is committed to fighting this disease along with Westley by raising money for research and giving Westley all the love, fun and confidence to help him keep the positive outlook he has on life.
Duchenne Muscular Dystrophy
Duchenne muscular dystrophy (DMD) is a recessive X-linked form of muscular dystrophy, which results in muscle degeneration, difficulty walking, breathing, and death. The incidence is 1 in 3,000. Only males are affected, though females can be carriers. The disorder is caused by a mutation in the dystrophin gene, located in humans on the X chromosome (Xp21). The dystrophin gene codes for the protein dystrophin, an important structural component within muscle tissue. Dystrophin provides structural stability to the dystroglycan complex (DGC), located on the cell membrane.
Symptoms usually appear in male children before age 5 and may be visible in early infancy. Progressive proximal muscle weakness of the legs and pelvis associated with a loss of muscle mass is observed first. Eventually this weakness spreads to the arms, neck, and other areas. Early signs may include pseudohypertrophy (enlargement of calf and deltoid muscles), low endurance, and difficulties in standing unaided or inability to ascend staircases. As the condition progresses, muscle tissue experiences wasting and is eventually replaced by fat and fibrotic tissue (fibrosis). By age 10, braces may be required to aid in walking but most patients are wheelchair dependent by age 12. Later symptoms may include abnormal bone development that lead to skeletal deformities, including curvature of the spine. Due to progressive deterioration of muscle, loss of movement occurs, eventually leading to paralysis. Intellectual impairment may or may not be present but if present, does not progressively worsen as the child ages. The average life expectancy for patients afflicted with DMD varies from late teens to early to mid 20s. There have been reports of a few DMD patients surviving to the age of 40, but this is extremely rare.
Who are we?
Dad – Dallas Groseclose; Mom – Jessica Groseclose; 1st born – Westley Groseclose; last but NOT least – Wyatt Groseclose
Dallas and I met in high school and married in 1998. We live a simple life in Virginia and LOVE our little guys. They drive us crazy, make us laugh and give us the best moments life has to offer. We truly feel blessed!