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Newswise — BIRMINGHAM, Ala. – Crystal and James Burford are preparing for the birth of their son, Jeremiah James, on April 8, with the knowledge that Baby J.J. will be fighting for his life from the moment he arrives.

J.J. was diagnosed with hypoplastic left-heart syndrome by physicians in UAB’s Division of Maternal-Fetal Medicine this past fall. Children born with HLHS have underdeveloped features on the left side of their heart, and it cannot pump oxygen-rich blood to the body properly.

The Burfords are allowing UAB News to follow their journey to their April 8 due date and beyond, in part to bring awareness to a condition that affects 960 babies born in the United States each year.

“We see approximately 10-20 babies who have hypoplastic left-heart syndrome or related variants every year,” said Waldemar Carlo, M.D., pediatric cardiologist at Children’s of Alabama and assistant professor in the UAB School of Medicine Department of Pediatrics.

Although treatments for HLHS have improved in recent years, there is no cure. Nationwide, only 75 percent of babies born with HLHS survive to age 1, Carlo says.

“Survival depends to a great extent upon the rest of the problems that may or may not be present and additional cardiac issues that may not be fully elucidated until the baby is born,” said Bennett Pearce, M.D., pediatric cardiologist at Children’s of Alabama and professor in the UAB School of Medicine Division of Pediatric Cardiology. “We make a diagnosis before birth, and we refine the diagnosis after birth.”

Treatments include three major surgeries — the first occurring within one week of birth.

A successful first surgery is better ensured by bringing the baby as close to a full 40-week term pregnancy as possible, says UAB Maternal-Fetal Medicine physician Richard Davis, M.D.

“In some ways it is a catch-22: You want these babies to stay in the uterus as long as they can, as long as they are growing and have normal fluid levels; but too much prenatal surveillance can sometimes lead to false results, and an additional problem may be indicated that may prompt thinking that an earlier delivery is necessary when, in fact, there are no additional issues,” Davis said. “Our goal is to get these babies to 39 or 40 weeks so they are big, strong babies for surgery. It’s important to tell the mothers that because they often think, ‘Let’s treat the baby as soon as we can.’ But amazingly, these hearts are very stable in the uterus.”

The fetal team comprises physicians and nurses from the high-risk Maternal-Fetal Medicine group, plus pediatric cardiology, cardiac ICU, cardiac surgery and neonatology.

“Our fetal team enables us to combine the best of OB, cardiology and surgery to give these babies the best chances to have the best outcomes they can have,” Carlo said. “We have a great deal of experience in dealing with this type of cardiac physiology and with the surgeries and post-operative care required in these little babies.”

More on the surgery and the challenges faced by the Burfords and other families dealing with HLHS is explained in the accompanying video.

About UABKnown for its innovative and interdisciplinary approach to education at both the graduate and undergraduate levels, the University of Alabama at Birmingham is an internationally renowned research university and academic medical center and the state of Alabama’s largest employer, with some 23,000 employees and an economic impact exceeding $5 billion annually on the state. The five pillars of UAB’s mission include education, research, patient care, community service and economic development. UAB: Knowledge that will change your world. Learn more at www.uab.edu.

EDITOR’S NOTE: The University of Alabama at Birmingham is a separate, independent institution from the University of Alabama, which is located in Tuscaloosa. Please use University of Alabama at Birmingham on first reference and UAB on all subsequent references.

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