Guests: Dani Bell, Westley McKinley
Speaker: Tracee McKinley & Jessica McKinley
Batten Disease is a rare inherited disorder of the nervous system with no current treatment or cure. Children and adults with Batten disease have inherited genetic material from their parents that may take some years to come to bear in their everyday lives. And when it does, families are often in shock when they learn how a normal child or adult (Kufs form) could change quickly, and without seeming cause. Those affected with Batten Disease suffer progressive neurological impairment, seizures, blindness, loss of motor skills, speaks, and finally loss of life.
Batten disease is one of approximately 50 diseases called lysosomal storage disorders (LSD), meaning that genetic mutations disrupt the cells ability to dispose of wastes. With Batten disease, cells are thrown out of balance with the build-up of proteins and lipids (fats). Because of these damaged cells, patients with Batten disease suffer progressive neurological impairment.
Tracee McKinley knows the devastating effects of this rare disorder as her son Westley was diagnosed with Batten Disease in October 2010. Westley was born a healthy baby boy with no signs of the impending disease. He was a normal 18 month old boy who loved to run around and play with his football. Without warning, Westley started regressing on his behavioral growth and then started having seizures. Tracee immediately knew something was seriously wrong. After months of tests and multiple hospitals, doctors at a Cincinnati hospital ran genetic testing to identify any specific gene mutations. After 3 months of waiting for the result, it was confirmed a CLN6 disease, a late infantile Batten disease.
Tracee was directed to the BDSRA, Batten Disease Support and Research Association, who provides resources to help families manage the disease and its impact through medical, educational, and support of other families who are impacted by this rare & fatal condition. The BDSRA is located in Columbus, Ohio and is the leading researcher for finding treatments and ultimately a cure for Batten Disease.
In April 2011, Westley had experimental brain surgery done at the New York-Presbyterian/Weill Cornell Medical Center. The surgery involved putting the gene that Westley is missing into his brain. Only time will tell if this will help him or not. This surgery is to slow down the progression of the disease, it does not cure it. Westley has also been accepted into an ongoing research program that with hope, will find a cure.
Westley is now 9 years old and has been battling the deteriorating effects of the disease for 7 years. He is still able to see and receive input, but is now bedridden, uses a feeding tube, and unable to communicate. Hope is not lost as Tracee will continue to combat the disease by trying new therapies and studies that may not only help Westley, but future generations to come.
Westley travels to Cincinnati every 2 weeks to see his doctor and must be cared for 24 hours a day with the help of two full time nurses. Jessica & Tracee hold fundraisers throughout the year to help pay for these expenses and donates half to the BDSRA research center in Columbus.
A bank account for “Westley’s Wish” has been established at the Atomic Credit Union in Logan if you would like to help this local family and the fight to find a cure. For more information on Westley visit his facebook page at https://www.facebook.com/Westleys-Wish-195044227210046/ .