There are people on this planet who face challenges so difficult some wonder why bother. Yet these same people shine so bright they shoot shafts of joy into our hearts.
Christa Saul is one of those people. In fact, if happiness were visible, Saul would glow.
Never mind the motorized wheelchair that goes everywhere with Saul. Heck, 38 years ago when doctors diagnosed baby Saul on her first birthday with a genetic disease called spinal muscular atrophy she wasn’t expected to live more than a few years.
But because of pioneers such as Dr. Michael Muhonen, director of CHOC Neuroscience Institute in Orange, we live in an era of medical miracles and wonder.
A relatively new drug called Spinraza coupled with Muhonen’s brand new invention to deliver the medicine now allows patients such as Saul to actually get stronger.
It’s micro movement, understand. But over time, micro can become macro.
“My stamina, my energy, my respiratory is better,” shares Saul, who has little motion beyond the thumb she uses to guide her electric wheelchair. “And I can sit up unsupported.”
The last time Saul could sit up by herself? A quarter-century ago.
But if you want to visit with Saul, better make an appointment. Not only is her hospital room filled with friends — dancing friends, mind you — nothing gets in the way of this woman fueled with determination and love.
Not even a raging wildfire.
Debbie Saul, Christa’s mom, looked at her nine-month old daughter and after raising one child into toddlerhood knew something was wrong. It was subtle, but her baby’s movement was off.
Three months later, doctors confirmed the suspected diagnosis: spinal muscular atrophy, a motor neuron disease that, in effect, chips away at nerve cells in the spinal cord and weakens muscles.
Babies are affected the worst, children a little less so, adults the least. As years tick by, spinal curvature is common.
When Saul was eight years old, doctors had to fuse the girl’s spine. More surgery followed with a metal rod inserted into her back.
For many parents, the struggle can be a never-ending nightmare. But Saul’s mother is made of exceptionally sturdy stuff.
Instead of giving up, Mom ramped up. She made sure her daughter lived life to her fullest. Then Mom adopted several children with disabilities.
Standing outside the hospital room where her daughter is getting her latest procedure, Mom shares she adopted for several reasons. One was because she loved the children. The other was because she discovered she was very good at making a difference in the lives of kids with special needs.
I ask Saul how she describes her situation. Challenged? No, that’s not how she feels. She feels she clears hurdles. Disabled? Saul turns over the word, as if the concept is alien.
Well, she’s not exactly able-bodied. OK, she decides, she’s disabled.
The conundrum is that from where Saul sits, she does so much. Except art. When Saul was in high school, she sketched, painted, made jewelry. She misses creating art.
Still, Saul sees her world as one with infinite possibilities.
She loves getting out and doing just about anything. She goes on ship cruises, shops, takes road trips to Las Vegas and San Diego, checks out comedy clubs (she’s met David Spade, the Wayans brothers, George Lopez), and enjoys upbeat musicians such as Jack Johnson and Pink.
At a September concert in Irvine, she rocked out to singer Jason Mraz’s good vibes — along with thousands of others, including my wife and myself.
“Open up your mind and see like me,” Mraz sang. “Open up your plans and damn you’re free.”
Mraz could have been singing about Saul — or Saul’s doctor, who she says “has a heart of gold.”
A desire to cure
When Muhonen was in high school, his four-year-old brother was hit by a car. The boy lived, but big brother discovered his mission in life. He wanted to cure others.
Over the years, Muhonen served as primary investigator on clinical trials for the Strata Valve, a device that treats hydrocephalus; led the research effort for something called an intrathecal baclofen pump that allows medicine to be delivered directly into the spine; helped advance shunting devices.
I ask what’s new and at his office at St. Joseph Hospital in Orange, Muhonen reaches behind him and opens a small Lucite box with a gray sponge holding something that resembles a tiny Seattle Space Needle. Without asking, I pick up the copper-topped device.
It turns out to be incredibly complex, delicate and includes four titanium rods — threads really — in the needle portion. It also turns out, I, um, grabbed an $8 million medical device.
The wireless invention includes coils as well as a computer chip and is so high tech it can be permanently inserted into someone’s skull. Then, with a simple smartphone-like device, it can be used to measure pressure in the brain.
“Problems intrigue me,” the doctor explains, “and because of what I do, solutions outside of existing technology intrigue me as well.”
Muhonen places a regular shunt on his desk. The thin tube is some four-feet long and looks like one my father had that went from from his head to his stomach. Next, the surgeon shows a far sleeker gizmo that can do the same thing.
“The average shunt,” the doctor offers, “has to be changed 11 times.” The new model can be calibrated without further invasive surgery.
“Rather than chopping off the top of the weed,” says the neurosurgeon, “let’s find a cure.”
His latest success is developing a surgical technique and modifying existing hardware that allows patients like Saul to easily and quickly receive the spinal muscular atrophy medication.
Understand, almost everyone with the disease has undergone a spinal fusion and when the spine is fused with metal rods, treatment is dangerous, painful, and typically takes more than an hour.
“With the old technique of standard injections,” the physician says, “many patients have suffered broken bones.”
Muhonen’s invention offers a permanent port that allows patients to remain in their wheelchair during the procedure — and it takes five minutes to complete the entire Spinraza injection process.
Dancing into the future
On Oct. 9, 2017, the fast-moving Canyon Fire 2 jumped the 91 Freeway, marched south along the 241 Toll Road and headed for the Anaheim Hills — right where Saul lives.
Muhonen installed Saul’s port only six days before, and Saul was still recovering at home when trucks with loudspeakers rolled down her street broadcasting mandatory emergency evacuations.
“It was scary,” Saul admits, “and moving was hard.”
But Saul wasn’t worried for herself. She was concerned about her beloved dogs. There is Bubba, a Pekingese; Jagger, a Pomeranian; Charlie, a Morkie; Walter, a French bulldog.
Don’t think for a moment, however, the dogs are only for Saul. That’s not her style.
With friends, usually Cindy Jurs or Jennifer Justice — or both — Saul totes two of the dogs every Tuesday to the VA hospital in Long Beach where she offers them up to long-term patients for pet therapy.
“I want to give back to people,” Saul allows, “and now there are a dozen guys who are like family.”
When Saul went to the hospital for her most recent surgery, two of the veterans hopped three different buses to offer their support.
Saul also spends countless hours counseling children, parents and adult patients about spinal muscular atrophy. Her efforts make strong bonds and she is asked to attend parties throughout Southern California for kids with “SMA,” as the disease is nicknamed.
“They’re my little Minnie Mes,” she shares, smiling. “I tell them don’t let it ruin your life.
“I have an awesome life and I have fun. I just happen to be in a chair.”
How far does Saul think she can go with the new drug and its cousin, the surgically inserted port?
“You just have to give the medication time,” she states. “Anything is possible.”
Perhaps someday, we’ll see a sketch from Saul that captures hope. In the meantime, she dances in her chair.
Article link: https://www.ocregister.com/2018/12/03/woman-with-severe-back-disability-gets-new-life-with-doctors-high-tech-invention/?fbclid=IwAR3do38NQBN_TAWc7tpkHmk9q8vKgpPWl5qySrI9Z4U49WEuRHVESYQweL0