Boy's 26 arterial narrowings fixed in one fell swoop
Cardiothoracic surgeon Frank Hanley corrected more than two dozen pulmonary artery narrowings in a 6-year-old patient during one marathon surgery.
The first five years of life for Jordan Ervin were an endless cycle of illnesses and medical appointments, from Illinois all the way to California. He’d been poked, prodded, X-rayed and scanned, and even had his chest cut open, but none of it knocked the perpetual smile off his face.
“Jordan is such a champ,’’ said his mother, Seville Spearman, of DeKalb, Illinois. “He’s always been just a really happy kid.”
Happy then, but even happier now. That’s because years of incorrect diagnoses and treatments are now over.
Jordan’s path to recovery started when, just before his fifth birthday, in 2013, a developmental pediatrician put together all of Jordan’s problems and diagnosed him with Williams syndrome, a rare chromosomal disorder that affects just 1 in 10,000 people worldwide.
The disease brings a host of medical issues, including learning disabilities and severe heart defects. But there’s a silver lining: Individuals diagnosed with the rare disease also tend to be gregarious and extremely social.
Sounds like Jordan
“Jordan’s personality made the process easier to deal with,” Spearman said. “He never fretted about going to doctor appointments. He was always excited about getting to see the nurses, the woman at the front desk and everyone else in the waiting room.”
That good cheer, combined with the work of Frank Hanley, MD, cardiothoracic surgeon at Lucile Packard Children’s Hospital Stanford and Stanford Children’s Health, means that Jordan’s big heart is now a properly working heart.
Hanley, who also is a professor of cardiothoracic surgery at the School of Medicine, led an eight-hour surgery Dec. 10 to reconstruct pulmonary arteries to Jordan’s lungs. He is known for tackling some of the world’s toughest and most complex pediatric heart surgeries. For Jordan, this meant correcting the stenoses, or narrowings, in those arteries.
It was a complicated case. The stenotic arteries caused severe pulmonary hypertension. In less-severe cases, in which there is only one area of stenosis near or at the pulmonary valve, doctors can perform a fairly simple surgical catheter procedure that uses a tiny balloon to expand the artery. But Jordan had multiple narrowings: 12 in his left lung and 14 in the right lung. The balloon technique is much less effective in this scenario, and no other surgical techniques have been developed to treat these stenoses. So Jordan would need a different approach.
That approach was developed by Hanley, who receives referrals from all over the world. He’s the pioneer of a one-stage, fix-all-the-defects surgery called unifocalization.
In the last few years, Hanley has taken many of the unifocalization techniques and used them for extensive pulmonary artery reconstruction on Williams syndrome patients like Jordan and other patients with similar heart defects.
To explain Jordan’s operation, Hanley likened the boy’s pulmonary arteries to a large tree, starting with a trunk that goes to large branches and then smaller branches. Normally, the blood flows freely through those branches, but in Jordan’s case, the narrowings in his arteries made it harder for the heart to pump blood to the lungs. This resulted in pulmonary hypertension, a life-threatening condition. In a case as severe as Jordan’s, Hanley said a balloon catheterization procedure isn’t effective.
So, in December’s intricate operation, Hanley and his team placed Jordan on life support and set to work fixing the 26 stenoses. As a result of the procedure, Jordan’s pulmonary hypertension was cured.
Jordan is going to have perfectly normal life expectancy.
It was another of the more than 540 unifocalization surgeries Hanley has performed at Lucile Packard Children’s Hospital for patients with complex pediatric heart defects, and with great outcomes. Hanley taps his operating-room stamina and experience to use this innovative, one-stage approach to decrease overall hospital time for patients, reduce the number of times a heart must be stopped and repair problems before they worsen or become impossible to repair.
“We’re definitely on the leading edge of this kind of surgery,’’ said Hanley, who holds the Lawrence Crowley, MD, Endowed Professorship in Child Health. “Jordan is going to have perfectly normal life expectancy.”
For Jordan’s parents, it was the best gift they could have hoped for. “We were joyfully stunned,” said Charles Spearman, Jordan’s stepfather, thankful for Hanley’s skill and thrilled that Jordan could return home for Christmas to share that famous smile.
A first-grader at Tyler Elementary in DeKalb, Jordan, 6, is about a year behind his peers developmentally, according to his mother. With heart surgery over, she and her husband can now focus on helping Jordan with other challenges that come with Williams syndrome, including social and gross motor skills.
“Things just seem brighter again,’’ said Seville. “There was just a dark cloud hanging over us. I was trying to figure out if my son would still be here in six months or for his next birthday. Now I know he will be here. Everything is back to normal, but I will never take anything for granted again.”
Stanford Medicine integrates research, medical education and health care at its three institutions - Stanford University School of Medicine, Stanford Health Care (formerly Stanford Hospital & Clinics), and Lucile Packard Children's Hospital Stanford. For more information, please visit the Office of Communication & Public Affairs site at http://mednews.stanford.edu.