Trinity River Trails
Trail Head # 2
Corner of University Drive & Riverfront Drive, Fort Worth, TX
Date: October 11th, 2008
Race day registration: 7:00 A.M
Run/Walk Start: 8:30 A.M
Benefiting: MPS Society

The MPS Run for Their Lives 5k is
one of the MPS Society's 20 plus runs
nationwide. This run is an out and back
course along the beautiful Trinity Trails
green belt. Proceeds benefit the MPS
Society funding research for the cure
of MPS. Refreshments and entertainment for the whole family!

Stephen 'Spencer' Holland
(1989-2008)

River Oaks teen beat odds by living active life

By MATT FRAZIER

Star-Telegram staff writer

RIVER OAKS -- Stephen "Spencer" Holland was diagnosed at age 4 with a rare disease that causes defects in bones, cartilage and connective tissue, which lead to short stature, clouding of the eyes and deterioration of the liver, spleen, heart and brain. Doctors estimated that Mucopolysaccharidosis, known as Hurler's syndrome or MPS, would claim the fair-haired boy in a handful of years. They were wrong. Spencer participated in experimental treatments and research studies that allowed him to participate in sports, sing in a choir and win awards for playing the trumpet. And the studies gave researchers information to help improve the lives of other children with the rare genetic malady, including Spencer's two younger sisters, Maddie, 16, and Laynie, 14. Spencer, 18, a senior at Castleberry High School, died unexpectedly in his sleep Jan. 26 while in Los Angeles receiving an experimental new treatment. A graveside service will be held today, and the family plans to conduct a memorial service on March 1. "He was very witty, very charming, very caring and respectful of others," said Debbie Dummann, a member of the National MPS Society. "He was always happy and always smiling."
No symptoms at birth
Spencer was born Dec. 19, 1989, into a seemingly picture-perfect Texas family. His grandfather, Tom Holland, was mayor of River Oaks for 22 years. His mother and father went to Castleberry Church of Christ as teens, became high school sweethearts, attended Texas Christian University together and married soon after graduation. Amy Holland became a special education teacher and Steve Holland became a certified public accountant and River Oaks city councilman. Symptoms of the disease are not apparent at birth, so when Spencer and his younger sisters were born, his family had no reason to suspect that they were sick. Neither parent knew they carried the gene for MPS, suffered by children who are missing a crucial enzyme, until a doctor first diagnosed Spencer's younger sister in 1996. Within a week, the disease that affects 1 in 25,000 people was confirmed in the other two children. "I felt like I was just underwater," Amy Holland told the Star-Telegram in 2002. "You either just sink or swim."
Effective treatment
In second grade, Spencer's favorite subject at Castleberry Elementary School was physical education. His parents encouraged him to play hard because they knew that one day he would not be able to participate. Already, the disease was affecting Spencer, thickening the lining of his throat and making it difficult for him to breathe. He also began to have trouble with mental processing, unable to find an obvious word for what he wanted to say. His spleen and liver were grossly enlarged. Walking became too difficult. And then what seemed like a miracle happened. Spencer was selected as one of 10 children to take part in a clinical enzyme replacement therapy study conducted by the University of California at Los Angeles Medical Center. The intravenous infusions he received gave him the stamina to swim and play basketball. While he grew stronger over the next two years, his sisters grew weaker. It was excruciating for Amy and Steve Holland. It was excruciating for Spencer as well. He was grateful for the enzyme, but told the Star-Telegram in 2000 that his sisters needed it, too. "I want to keep doing all the stuff I get to do now -- like shooting baskets," Spencer said. "I couldn't do some stuff before, like basketball and swimming. I wouldn't be able to do a lot of things by now without the enzyme. I can swim real fast. Probably faster than lightning."
New drug treatments
By 2002, all three children were taking the enzyme. It couldn't reverse damage nor halt damage to the brain. But it appeared that all three were stabilizing. In April 2003, thanks in part to Spencer's participation in the research trials, the FDA approved drug treatments. During this time, Spencer spoke about his illness at MPS Society conferences around the nation. He also helped his parents coordinate an annual MPS run/walk fundraiser. "A lot MPS kids do not live this long and some develop severe retardation, so it was inspirational to parents to be able to see an older child who was not that way," said Spencer's father, Steve Holland. And he participated in an amazing array of activities, a practice that increased as he entered Castleberry High School. While there he won Division I awards for his trumpet solo at a regional UIL competition and for his vocal solo at the UIL state choir competition. He played on the tennis team. He sang in school musicals. He played chess, hunted, drove his sisters around town and sang at Castleberry Church of Christ. Castleberry High School held a memorial for him Tuesday. About 400 people attended. That's what kind of guy he was, Castleberry band director Dave Daniel said. "The one word that describes him is 'greatness,'" said "His time here was very inspirational. It showed that we can achieve whatever we want, not just what others expect you to."
More research
Laynie Holland, Spencer's youngest sister, was in the same experimental research trial as her brother, but is now off the therapy until an autopsy can determine the cause of Spencer's death. "We still have two other children with the disease," Steve Holland said. "Our fight is not over by any stretch." This report includes material from Star-Telegram archives.
MPS or Hurler's syndrome
What it is: A rare genetic condition in which the body does not produce specific enzymes that break down and recycle cellular material. As a result, it causes progressive damage throughout the body.
Symptoms: Coarseness in facial features, a large head and tongue, short stature, heart disease, hearing loss, dental abnormalities, progressive stiffness of joints and developmental delays.
Cure: There is no cure, but the FDA approved a drug therapy to treat its symptoms two years ago. Source: Star-Telegram archives.
National MPS Society
A private, 501 (c)(3) charitable corporation started in 1974 to fund research on MPS and related diseases, provide support for affected individuals and their families and to spread awareness of the devastating disease. Online: www.mpssociety.org
mfrazier@star-telegram.com
MATT FRAZIER, 817-390-7957