Tyler's Hope
FOR THE FIRST seven years of his life Tyler Staab was a typical boy—he played basketball, soccer, tennis and golf, just like other boys his age.
Today Tyler is anything but a typical 10-year-old. He has DYT-1 dystonia, a neurological movement disorder that affects the central nervous system. The disorder causes involuntary muscle contractions, which force parts of the body into abnormal, sometimes painful movements or postures. These uncontrollable spasms can affect any part of the body – legs, arms, trunk, neck, eyelids, face or vocal cords.
Tyler’s story began in February 2005. While playing basketball, Rick Staab, Tyler’s father, noticed that Tyler could not bring his arm in to his side without shaking uncontrollably. This initial symptom began a long and disheartening journey for Tyler, his father and his mother, Michelle Staab. Tyler was in and out of Shands Hospital at the University of Florida for the first part of 2005, and his family traveled from their home in Gainesville to nationally renowned hospitals. He was examined by specialists and received numerous, painful tests and treatments that only lead to dead ends and misdiagnosis.
“This was extremely frustrating to everyone, but none more than Tyler,” Rick Staab said.
Finally, in late spring, during one of Tyler’s stays at Shands, Dr. Michael Okun’s diagnosis of primary generalized earlyonset dystonia was confirmed by a blood test for the DYT-1 dystonia gene.
Unfortunately, the disorder progressed rapidly from that point. The earlier a child shows symptoms of dystonia, the more significant those symptoms become. Tyler, who has difficulty walking and speaking, will most likely be disabled within five years of the disease’s onset.
“Though we were relieved to have a ‘diagnosis,’ we were also very distraught with what we learned about this devastating disorder,” Rick Staab said.
Tyler underwent his third Deep Brain Stimulation (DBS) surgery in January, intended to relax the muscle movement disorder and give him some relief from the painful symptoms. During the surgery, Tyler was awake as doctors implanted electrodes in his brain to deliver continuous, electrical stimulation. The electrodes are powered by a programmable stimulator, similar to a pacemaker, which is implanted into the chest wall. The stimulator is connected to the electrodes by thin wires (leads) that are tunneled under the skin in the neck and scalp and is turned on and off with a magnet.
Adjusting the stimulator and medications is a major time commitment for the neurological team and the patient. The maximum effect of the procedure is achieved once adjustment occurs, which may take weeks or months.
Tyler received significant benefits from his first surgery in June 2006, his father said. Because of the growth of his skull and body, he underwent a second surgery six months later. His most recent surgery was in effort to alleviate the severity of the disease on Tyler’s left side. The results of the last surgery have been minimal, but that could change over time, Rick Staab said.
Since Tyler has the genetic form of dystonia, there is a 50 percent chance that his siblings, Samantha, 7, and Luke, 3, will carry this gene and a 30 percent chance that they will develop symptoms. One third of the estimated 500,000 Americans who struggle with dystonia are children.
Tyler’s condition - along with the statistics - motivates Rick and Michelle Staab to find a cure for the disorder. They formed a non-profit organization, Tyler’s Hope for a Dystonia Cure, Inc., in June 2006 to raise awareness regarding this debilitating disorder and to provide funding for neurology research to prevent and cure dystonia. The Staabs are working with nationwide researchers to advance their cause and have traveled to Wake Forest, Boston, Columbia, N.Y. and Texas to see specialists.
“Awareness and research is higher than it has ever been because of awareness and advances in the past two years,” Rick Staab said. “Most scientists now recognize that dystonia is a great disorder to research because they believe it is curable, which would allow them to have a huge impact.”
“We believe that finding a cure for DYT-1 dystonia is only a function of time and funding,”Dr. Okun said. “Dystonia has a known location and known gene product in the human DNA sequence. It is not degenerative like Alzheimer’s Disease and Parkinson’s Disease and, therefore, with investment of the proper resources, a cure should be achieved.”
Dr. Okun and Dr. Kelly Foote, who performed Tyler’s surgeries, are co-directors of the University of Florida’s Movement Disorders Center (UFMDC), with Dr. Hubert Fernandez. The UFMDC was established at the McKnight Brain Institute in 2002 to bring together UF doctors and researchers with special expertise in Parkinson’s disease and other movement disorders. The center’s mission is to provide the highest level of medical and surgical care to patients and to perform research that will lead to better treatments and, ultimately, cures for Parkinson’s disease and other movement disorders.
Dr. Okun and Dr. Foote are key members of the Tyler’s Hope Foundation’s medical advisory board, which consists of leading clinicians and researchers in the field who recommend the most effective use of funds in pursuit of greater insight into the disorder’s cause and cure.
One of the goals of the foundation is to establish a stand-alone Dystonia Research Institute in Florida, and Rick Staab will not rest until this mission is complete. “If we find a cure, everything else is reversible,” Rick Staab said. “For something that affects Tyler and so many people, we believe a cure can happen.”
“Tyler is doing a terrific job fighting his dystonia. He has a winning attitude,” Dr. Okun said. “Tyler and the Staab family can raise funds to find better treatments for dystonia. We desperately need the support of the Gator Nation to help us put together a state-of-the-art facility for diagnosis and treatment of dystonia.” G
If you would like to contribute, please send donations to Tyler’s Hope for a Dystonia Cure, 13351 Progress Blvd., Alachua, FL 32615, or visit the Web site to view other ways to help, learn about upcoming events and follow Tyler’s progress by reading Rick’s diary, at www.tylershope.org .