Stories

A Cut Above: Surgeons Improve Outcomes

Dawson Van Diest and Dr. Raymond Tse

After Dawson Van Diest severed the triceps in his left arm, doctors told his parents he’d never use those muscles again. Today, Dawson, 11, proudly pumps out pushups – thanks to Dr. Raymond Tse’s pioneering work to repair damaged nerves that activate the muscles of the arm.

Seattle Children’s surgeons apply the art and science of their craft to improve outcomes for kids.

Every day, surgeons at Seattle Children’s use the latest, most advanced surgical techniques to make the burden of illness and injury a little lighter for children and their families. Meet four surgeons who are taking a step beyond to tackle vexing problems.

The architect

“There’s nothing we can do for your son’s arm.”

Those words, spoken by a surgeon, sparked disbelief in Sherri and Darrin Van Diest.

After taking a nasty spill on his four-wheel- all-terrain vehicle, their son, Dawson, then 9, could no longer write with his dominant hand or even raise that arm in class. The accident had severed a nerve in his spine that controlled the triceps muscle on his left arm – a condition known as brachial plexus palsy.

“If it had been a diagnosis given to me, I might have said, ‘OK,’” says Sherri Van Diest. “But when it’s your kid, you say, ‘that’s not good enough.’”

Her refusal to believe nothing could be done for Dawson led her on an Internet search that ended when she met Dr. Raymond Tse, one of a new generation of reconstructive surgeons who repairs damaged nerves that “motor” the muscles of the arm.

During a delicate, 10-hour surgery, Tse tried something that had only been done a few times before in the world – a nerve transfer to the triceps. He gathered healthy nerves taken from under Dawson’s ribs and then attached them between the main radial nerve running down the arm and the tail of the nerve still attached to the muscle.

“The old guard may say ‘there’s nothing we can do,’ but things have changed.”

Dr. Raymond Tse

In three months, the transplanted nerves in Dawson’s arm grew through the core of the old nerve tail and into the triceps, activating the muscle. Now, a year and a half after the surgery, Dawson is back to snow skiing, wake surfing, playing basketball, golfing, swimming – and riding that four-wheeler.

Tse was recruited to Children’s in 2009 to lead the first – and only – brachial plexus program in the Pacific Northwest. He now puts his childhood dream of being an architect to use rebuilding arms instead of houses.

“The old guard may say ‘there’s nothing we can do for brachial plexus palsies,’ but things have changed,” says Tse. “The team at Children’s is on par with the best in the world.”

The visionary

Justus Fuccillo and Dr. Sam Browd

Dr. Sam Browd is developing
a new kind of hydrocephalus
shunt that promises to
dramatically reduce failure
rates, so kids like Justus
Fuccillo, 2, don’t have to
undergo multiple surgeries
to replace the failed devices.

For busy mom Sarah Fuccillo, typical toddler issues such as irritability, tummy upset, headache and sleepiness could be signs that her son’s shunt – a device implanted in the brain to draw off excess spinal fluid – has stopped working.

“I’m constantly sitting on the edge of my seat,” says Fuccillo, whose son Justus developed hydrocephalus after his premature birth caused blood vessels in his brain to rupture. “There’s no guarantee that his shunt will last for any length of time, which is unnerving.”

In today’s high-tech world, it’s almost impossible to believe that life-saving shunts have changed little since the 1950s and have the same failure rates as half a century ago: 30% the first year, 40% the second year and 98% within 10 years.

In fact, shunt replacements are the number one reason for pediatric brain surgery. At Children’s alone, Dr. Sam Browd and his colleagues replaced 90 shunts last year. Justus, only 2, has already had 11 surgeries.

Browd intends to change that.

98% of all shunts fail within 10 years.

He and Dr. Barry Lutz, a micro-fluidics expert at the University of Washington, started from scratch with a wish list of modern technologies that could be used to create the shunt of neurosurgeons’ – and parents’ – dreams.

The new device should hit the market in five years and will feature an electromechanical mechanism to reduce the clogging that causes 90% of today’s shunt failures. Electronic data transmission will allow clinicians to check the status of a shunt remotely, reducing the number of clinic visits for families. And the ability to record a child’s intracranial pressure will help doctors manage kids with chronic headaches more effectively.

Perhaps the best feature is the valve that will control the flow of spinal fluid. Besides being failure-resistant, it will have a battery life of eight years.

These design elements will help take the crisis out of replacing a shunt because families will be able to plan for the procedure before the device fails.

“Our immediate goal is to reduce shunt malfunctions by 50%,” explains Browd. “If we can meet that milestone, we’ll have vastly improved the way hydrocephalus is treated.”

The investigator

Dr. Kim Riehle

Dr. Kim Riehle is one of the
few pediatric surgeons in the
nation doing basic science
research. She studies how
the liver responds to injury
and regenerates, with the aim
of creating new therapies to
fight liver failure in infants,
children and teens.

Dr. Kim Riehle was a medical student intent on becoming a cancer specialist until she assisted a surgeon in the operating room on one of her rotations and was immediately hooked.

During her residency at Children’s, the trajectory of Riehle’s career changed again after she read an article about liver regeneration written by Dr. Nelson Fausto, a world renowned liver expert and researcher at the University of Washington.

“I was so excited that Dr. Fausto was located here. I asked him if I could work in his lab, thinking he would surely say ‘no,’ but he didn’t,” she recalls. “Since then, figuring out how the liver responds to injury and renews itself is another passion for me.”

Riehle came back to Seattle after completing a surgical fellowship at Boston Children’s – she was only the third female in that hospital’s history to do so. She works 80% in Fausto’s lab and 20% as a general surgeon at Children’s. It’s an unusual arrangement for a pediatric surgeon and reflects Children’s commitment to understanding the cellular mechanisms of disease through basic science research – an important step before new treatments can be developed.

Basic lab research to understand the cellular mechanisms of disease is an important step in developing new treatments.

Her current studies investigate why scar tissue in the liver affects signaling pathways that make healthy liver cells stop regenerating or turn into cancer cells.

“I want to help children whose liver cells used to be able to function normally before a condition like Tylenol toxicity, hepatitis, severe sepsis or a genetic disorder repeatedly injured them,” she says.

Riehle’s efforts in the lab set the stage for her to test targeted therapies that may eliminate the scar tissue altogether or prevent healthy liver cells from responding to signals to act abnormally in the presence of scar tissue – a promising advance that could restore health to a child’s failing liver.

The game-changer

Shakira and Kevin Locke

Shakira Locke, 5,
(with her dad Kevin Locke)
can now breathe and eat
normally after tumors that
cut off her airway and
esophagus were successfully
treated by a blood-pressure
medication. Before taking
propranolol, tumors covered
Shakira’s neck, face and ear.

Dr. Jonathan Perkins

Dr. Jonathan Perkins was the
first in the region to use
blood-pressure medication to
treat hemangiomas that are
usually surgically removed.
Perkins also studies why
blood-and lymph-vessel
tumors develop in some
kids and not others.

Right after Lorene Locke gave birth to her daughter, Shakira, she noticed a rash on the newborn’s face. Within two weeks, the tiny girl’s breath sounded hoarse and a week later Shakira was gasping for air.

An abnormal lump of blood vessels called a hemangioma – the most common type of tumor among infants – was growing out of control inside her throat and on her neck, face and ear. By the time Shakira was 6 months old, she had tubes in her trachea to breathe and in her stomach so she could eat.

Fewer than 10 specialists in the country investigate why infants like Shakira develop hemangiomas and other mysterious tumors made up of blood or lymph vessels – which are known collectively as “vascular anomalies.” Children’s otolaryngologist Dr. Jonathan Perkins is one of only a handful of researchers publishing on this topic.

Shortly before Shakira’s second birthday in 2008, Perkins ran across a scientific article from France describing how propranolol, a blood pressure medication, had – quite by accident – been found to shrink hemangiomas. He put Shakira on the drug and within four months her tumor was completely gone.

Dr. Jonathan Perkins is one of less than 10 specialists in the U.S. studying why some infants develop hemangiomas.

Although Shakira was the first in the region to be treated with propranolol, she isn’t the last. Today, Perkins estimates that he performs 75% fewer surgeries to remove hemangiomas because the blood pressure medication is so effective.

Perkins’ work to improve treatment options for children with all types of vascular anomalies includes studying how signals at the cellular level trigger the formation of lesions – research that may reveal why some children are born with tiny birthmarks and others with football-sized tumors.

His efforts recently inspired three moms to start the Vascular Anomalies Research Guild.

“He saved our son’s life,” says Tanya Rome, a Billings, Mont., resident who found Perkins through her own research on the Web. “We want to do everything we can to support Dr. Perkins’ work, so better treatments can be developed for all of our kids.”

Connect with other families at Facebook.com/VascularAnomalies.