Fighting the good fight against disease

Dust permeates the Brandoms’ home in Newport Beach. Workers come and go via a well-worn path through the garage and into an interior hallway, where they can access a large shaft two stories high that’s under construction.

Soon that space will house an elevator. But after four months, the project — beset by delays, unexpected complications and mistaken measurements — still has a ways to go. It’s surely a test of nerves and patience for family members.

But if any family knows how to deal with adversity, the Brandoms certainly do.

The elevator is being built for Dusty, the eldest child of Neil Brandom and Cath Jayasuriya. Until the elevator is completed, he gets to his second-floor bedroom by means of a chairlift that he has to be transferred into, and once upstairs he is carried to his bed.

Dusty, who turns 23 on Tuesday, has Duchenne muscular dystrophy, a muscle-wasting genetic disorder that has robbed him of the ability to walk and use his arms, and is sapping the strength of his heart to pump blood and his lungs to breathe.

The Brandoms are my longtime neighbors and friends, and I’ve written previously about their inspiring perseverance through the travails of living with this brutal disease, which afflicts one out of every 3,500 male births. With Duchenne, a defective gene results in the absence of the protein dystrophin, which is needed to keep muscles strong.

Symptoms typically surface by about age 5 and get progressively worse until hearts or respiratory systems fail, or infections like pneumonia set in. Until recently, most boys with Duchenne didn’t survive their teens, but thanks to improvements in drug therapies and medical technology that help to keep hearts beating and lungs breathing many are now living into their 20s, and some even beyond that.

But those are stopgap treatments only. There is no cure.

Despite that grim reality, Dusty remains determined to live a rich and meaningful life, which is a testament not only to his own strong will but also to the fierce love of his parents and his close bond with younger siblings Lucas and Gabriella.

Overcoming his inherent shyness, he recently appeared in a CBS Sports program, “Courage in Sports.” In the segment, Dusty discussed the meaning of courage, and a mountain-climbing expedition in honor of those with Duchenne that was organized by Coalition Duchenne, the charity run by his mother.

Dusty is currently taking a break from his studies at UC Irvine, but a class in astronomy he took there sparked his current passion. Using a telescope linked to a video screen, Dusty views nebulae and other space phenomenon by locking on to coordinates through a computer program. (Although unable to lift his hands, he is able to manipulate the fingers of his right hand to work the computer.)

And on Friday, Dusty will attend an annual benefit for Coalition Duchenne at the Marconi Automotive Museum in Tustin. Proceeds from the event, as with all money raised by the organization, will go toward research that the nonprofit has targeted for funding, among several Duchenne-related initiatives currently underway that are providing a measure of hope for the future.

One potentially important Coalition Duchenne-backed research project is proceeding under the direction of Dr. Eduardo Marban, head of the Cedars-Sinai Heart Institute in Los Angeles. Dr. Marban’s team previously found that injections of cardiac stem cells could rejuvenate heart muscle in adult patients who’d suffered heart attacks. Using the same method to address Duchenne-weakened hearts, the institute found encouraging results in earlier testing with mice, and is now working on starting a clinical trial using human subjects.

Meanwhile, other promising drugs and therapies are in the pipeline, some of which might soon receive Food and Drug Administration approval. Taken together, they could work to improve the quality and length of life for those stricken with Duchenne, and possibly even reverse some of the damage caused by the disease.

“I believe a cure will be possible one day,” said Cath.

Because hearts and lungs eventually give out as Duchenne progresses, Cath has decided to focus her funding efforts on research in those areas. “I feel that I’m an advocate for older boys with Duchenne,” she said. “We’re trying to stay on top, trying to be 10 steps ahead.”

It’s that fighting spirit, that unwillingness to give up, which is ever present in the Brandom household. The same determination drove Cath to return to her native Borneo last summer — with Dusty’s blessing — for Coalition Duchenne’s fourth annual mountain climbing expedition just a few months after a strong earthquake claimed the lives of some hikers, including that of her longtime guide.

“I felt that we had to do it,” she said.

Every day, Dusty has his own mountain to climb. He takes heart medication regularly. With only 20% lung capacity, he uses a variety of devices to help him breathe and clear his lungs. Suffering from scoliosis and severe acid reflux, he has difficulty sleeping and receives sustenance from a liquid diet. Sometimes he feels too ill to even work on his computer.

Yet Dusty’s spirit remains as bright as the stars at which he likes to gaze through his telescope each night.

“His mind is very active. He says, ‘We can see a nebula tonight,’” said Cath. “He’s just full of life.”

PATRICE APODACA is a former Newport-Mesa public school parent and former Los Angeles Times staff writer. She lives in Newport Beach.