From left: Richard, London and Shelly Ethington of Mesa. Photo by Daniel Friedman.
Sunlight shines through the window of the second room on the left in the Cardiovascular Intensive Care Unit at Phoenix Children’s Hospital. But the patient whose name is on the door is not lying in a hospital bed. On this day, you would never know that then 3-year-old London Ethington, of Mesa, called this room home during her recovery from open-heart surgery.
The machines and intravenous tubing that once seemed attached to every inch of her little body are powered down and stored away. Today, London is wearing pink—the same color of her glowing cheeks and the ice cream cone adorning the front of her shirt. She is beaming—and twirling.
“I can spin around with no wires and no plugs!” she cries. “I can dance!”
Just four weeks earlier, Richard Ethington walked down the long hall toward the operating room with his daughter in his arms. He gently placed London on the operating table, then hugged her. “Daddy will see you in a little bit,” he whispered in her ear as her eyelids began to flutter above the anesthesia mask.
February is American Heart Month and marks nearly two years since the last of London’s three open-heart surgeries. The American Heart Association promotes American Heart Month with the goal of living healthier lives free of heart disease, which, according to the Centers for Disease Control, is the cause of one in every four deaths in Arizona and the leading cause of death for both men and women in the U.S.
Congenital heart disease is the most common birth defect in the U.S., affecting nearly one out of every 100 live births. London’s condition is one of the most complex heart problems, affecting only five out of every 100,000 live births.
Before she was born, London’s parents were thrilled to learn from an ultrasound that they were having a girl. They wanted to place a recording of the baby’s heartbeat in a pink stuffed rabbit as an announcement to the grandparents.
“Little did I know how special that heartbeat would be,” says Shelly Ethington, London’s mother. “We knew something was wrong with London’s heart” after the ultrasound. She and Richard were sent to several specialists before going to St. Joseph’s Hospital and Medical Center for an echocardiogram.
When Shelly was 20 weeks pregnant, London was diagnosed with double inlet left ventricle (DILV), a heart defect that left her with one functioning ventricle. The rest of us have two: one to pump blood to the lungs and one to pump blood through the body. Without early intervention, London would not survive her first month of life.
London had her first open-heart surgery—a Blalock-Taussig (or B-T) shunt—when she was five days old. After two subsequent surgical procedures—a Glenn shunt and a Fontan procedure—her left ventricle could pump blood to both the lungs and body.
“London’s three heart surgeries were like teaching her heart to ride a bike,” says Kristi West, RN of PCH. “Her first surgery was like putting training wheels on her heart; the second was like taking one of those training wheels away; and the Fontan surgery was like her heart was finally riding a bike.”
“Surgery is the scary part, but recovery is the hard part because it’s not so much keeping them stable as it is managing their pain,” says Shelly, who adds that London was given high doses of morphine and was on a fentanyl drip, which left her highly sedated. “She whined and moaned with every breath the first night.”
To comfort their daughter, Richard and Shelly gently tickled her face, held her hand and climbed into bed with her at night. They played soft, soothing music for her from their iPhones, including that of world-famous pianist and single ventricle heart defect survivor Paul Cardall, of Salt Lake City, who will celebrate his 41st birthday in April after undergoing a heart transplant in 2009.
Little improvements came with a smile here, laughter there, and a good stretch of restful sleep. Still, Shelly waited eagerly to see London “get that spark back in her eye.”
West was pivotal to London’s recovery.
“The first surgery is the easiest because newborns can’t talk or scream or throw their [snacks] at you; they have no personality yet,” says West. “The final surgery and recovery is harder because you are dealing with this little person who has opinions and wants [and who] can express pain.”
The trick was to make London believe it was all her idea.
“You have to make them feel like they’re getting choices, and you have to get creative,” says West, who prides herself on the “sneaky” tactics she employs to get young children to take their meds, a bath and even walks.
London spent four weeks recovering in the hospital. To pass the time, she played hide-and-seek in the hospital wing with her parents, toting her oxygen tank with her everywhere she went. She was visited by therapy dogs and cried when they left. She painted and made crafts almost every day and her dad painted her toenails with a bright red polish called “Cherries in the Snow.”
Although London has successfully recovered from each procedure, the surgeries are not a cure.
“It’s like London has half a heart, and that is fraught with its own problems,” says Stephen Pophal, MD, director of the Children’s Heart Center at PCH. With one ventricle doing the work of two, London will require yearly echocardiograms and lifelong cardiologist checkups. Survivors with single ventricle often return to the hospital between age 20 and 30, at which time they will require a transplant.
“She will need to be followed by a cardiologist for life, but otherwise she should be able to do most things that other children her age can do,” says Courtney Howell, PNP. “She probably will never be a marathon runner or an elite athlete, but she will be able to maintain fitness at her own pace and play like her classmates.”
“Her heart will never function like a normal heart, but it will work for her body,” says Shelly.
The day she heard she was going home, Shelly remembers that “London walked around the halls of the intensive care unit 30 steps ahead of me, like she owned the place.” With no “strings” attached, London twirled, walked and chased a good-humored respiratory therapist all around the hospital wing.
With her heart finally “riding a bike,” the first thing London wanted to do when she got home was ride her tricycle—and she did.
Resources and events
Congenital Heart Information Network
tchin.org
A national nonprofit organization created by the mother of a child with complex heart defects to provide reliable information, support services and resources to families of children with congenital defects and acquired heart disease, adults with congenital heart defects, and the professionals who work with them.
Mended Little Hearts of Phoenix
mendedlittleheartsofphx.org
The local chapter of a national non-profit organization providing support and resources to parents and caregivers of children with congenital heart defects (CHDs). Events this month:
The Mended Little Hearts Virtual Roar ‘N Run 13.1 and Fun Run takes place throughout the country during Congenital Heart Disease Awareness Week, Feb. 7-14. You have the whole week to complete the distance and can do it any time, any place and any way. Register at mendedlittlehearts.org.
The 2014 Congenital Heart Disease Awareness Fair will be held from 10am to 1pm Saturday, Feb. 15 at the Rosenburg Children’s Building at Phoenix Children’s Hospital. RSVP at mendedlittleheartsofphx.org/events.
Throughout February, members are conducting a care bag drive for CHD patients at Phoenix Children’s Hospital. Hotels donate shampoo; individuals donate personal hygiene items. Learn more at mendedlittleheartsofphx.org/february-awareness.
Phoenix Heart Walk
phoenixheartwalk.kintera.org
This event encompasses all forms of heart disease, including heart attack and stroke. Opening ceremonies begin at 8:30am Saturday, March 29 at Wesley Bolin Memorial Plaza. The walk begins at 9am. Organizers hope to raise $1.2 million for patient care, advocacy, education and awareness.
Banner Health Centers
“Walk with a Doc” heart health campaign
bannerhealth.com
Three Valley Banner Health Centers will host “Walk with a Doc” events on the four Saturdays leading up to the Heart Walk on March 29. The walks will start slowly to help people build up stamina. The physician leading each walk will begin with a short talk about heart health. Walks gradually will get longer; the week prior to the 5K, participants will be walking almost three miles.
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