On November 6 the Scripps Ranch community of Kensington lost a much beloved friend and neighbor to a rare disease called mucopolysaccharidoses (MPS). Christina Ellen Leighton was only 14 years old, but gregarious and mature beyond her years. The whole community feels that they are better for knowing and loving her.

Christina moved into the Kensington neighborhood in 1997. From the moment she moved in she was accepted and treated like any other kid on the block. Christina participated in Halloween carnivals and block parties. Her disability never seemed to slow her down.

Christina was an 8th grader at Marshall Middle School where she enjoyed doing everything a 14-year-old girl loves to do, despite the complications of MPS. She loved going to Britney Spears concerts, visiting Disneyland, singing with the karaoke machine, swimming in her pool, or playing in the playhouse her dad was building.

Her loving parents and sister filled her days with her every wish. She would leave for her doctors’ visits and operations with a smile and look forward to returning to be with her friends. She could brighten anyone’s day as she sat on the back of her father’s white pickup truck in the garage and yell "Hello!" to neighbors walking by.

Christina was faced with many challenges as she coped with MPS. In the past year she underwent spinal surgery and was in a body cast for several months. She participated in a study with the MPS Society, which required weekly infusions, even though she hated getting stuck with needles.

Four weeks before her death, she underwent open-heart surgery for a valve replacement. She was very courageous and cheerful, despite her frequent operations and everything she had to go through.

 

Mucopolysaccharidoses is a genetically inherited disease. MPS I, the strain of MPS that affected Christina, is caused by a recessive gene, meaning the child must receive one gene for MPS from each parent in order to have the disorder. Thus, MPS I is very rare, occurring in the U.S. in about 1 in 250,000 births.

Mucopolysaccharides are long chains of sugar molecules used in the building blocks of the body’s connective tissues. Normally, there is a continuous process in the body of replacing used materials and breaking them down for disposal. Children with the MPS I disorder are missing an enzyme essential in cutting up two of the mucopolysaccharides.

The incompletely broken down mucopolysaccharides remain stored in cells in the body, causing progressive damage throughout the body, including the heart, bones, joints, respiratory system, and central nervous system. While there is no magic cure for the MPS disorders, there are ways of managing and treating the problems they cause. However, most children with this disorder do not live past their teens.

Christina participated in the first ever drug trial on humans, where a synthetic enzyme was infused to replace the one she was missing. Because of Christina, anesthesiologists and surgeons at Children’s Hospital are no longer strangers to the anomalous airways and anatomy of children afflicted with MPS.

Christina touched the lives of everyone who knew her. "Christina made such a big impression on me because she always seemed so happy and thrilled by the things in her life," says Ron Hawkins, neighbor and friend of the Leighton family. "I think we could all learn a lesson from Christina because, despite the challenges she faced, she was always cheerful and got such joy from living life every day."

As is typical of the Scripps Ranch community, everyone participated in creating a special "Celebration of Life" memorial service for Christina. Doctors, nurses, teachers, and friends shared in celebrating the life of this very courageous 14-year-old who left her imprint on so many people.

Christina enjoyed riding her bike to the local toy store, Learning Express. The owner of Learning Express graciously donated a portion of one week’s profits in December to the National MPS Society. For information on MPS disorders, please visit the National MPS Society website at [www.mpssociety.org].

Barry Hawkins
 

[Ed Note: Barry Hawkins is 17 years old and wrote this article for a high school project. Christina’s parents liked it so much they asked Barry and his father to submit it to the SRCA Newsletter.]