Baby JJ Scheduled to Have Second Hypoplastic Left Heart Syndrome Surgery Today


  • newswise-fullscreen Baby JJ Scheduled to Have Second Hypoplastic Left Heart Syndrome Surgery Today

    Credit: UAB News

    Crystal Burford says those early days in the hospital when she couldn't hold JJ because of all of the tubes and lines connected to him did not carry over to home. "He loves to be held," she says.

  • newswise-fullscreen Baby JJ Scheduled to Have Second Hypoplastic Left Heart Syndrome Surgery Today

    Credit: UAB News

    Adjusting to life with a little brother has been about the same as it would be for any other family, Crystal Burford says. "Isaac and Lilly love to play with him," she said.

  • newswise-fullscreen Baby JJ Scheduled to Have Second Hypoplastic Left Heart Syndrome Surgery Today

    Credit: UAB News

    Lilly said, "I like when I get to hold him."

  • newswise-fullscreen Baby JJ Scheduled to Have Second Hypoplastic Left Heart Syndrome Surgery Today

    Credit: UAB News

    Surgeons will be performing a surgery known as the Glenn, the second of three surgeries JJ will have to have to restore function to his heart. The Glenn involves disconnecting the superior vena cava from the heart and sewing it into the right pulmonary artery. Surgeons also will have to repair his aorta by adding a new smaller patch in a separate procedure that will be performed at the same time as the Glenn.

  • newswise-fullscreen Baby JJ Scheduled to Have Second Hypoplastic Left Heart Syndrome Surgery Today

    Credit: UAB News

    While James and Crystal hoped to get a little more time at home with JJ before the second surgery, they understand that timeframes can — and often will — change. "You can’t plan for everything with this kind of situation,” Crystal Burford said.

• Read this story and see photos at www.uab.edu/news

Newswise — BIRMINGHAM, Ala. – Crystal Burford says, if she didn’t know any better, little Jeremiah James appears to be just like any other healthy 3-month-old baby. He’s smiling, cooing, reaching for anything someone puts near him. He’s happy.

“He’s a very happy, seemingly healthy baby,” Burford said. “If he didn’t have the scars and feeding tube, you wouldn’t know he had an issue. He’s doing really well since we’ve been home. And we thought we’d have a little more time with him at home, but he’s got to go back to the hospital to have his next surgery a little sooner than we expected.”

Indeed, JJ’s second heart surgery, known as the Glenn, will take place Tuesday, July 21, at Children’s of Alabama. The procedure will be the second of three heart surgeries doctors with Children’s of Alabama and the University of Alabama at Birmingham are performing on Baby JJ to help restore function to his heart, which was underdeveloped due to a birth defect known as hypoplastic left heart syndrome, a rare condition UAB physicians discovered when Crystal was four months pregnant. The Burfords are allowing UAB to follow them on their journey.

In babies with hypoplastic left heart syndrome, the left side of the heart cannot pump oxygen-rich blood to the body properly. The oxygen-rich blood bypasses the poorly functioning left side of the heart during the first few days of life. The right side of the heart then pumps blood to both the lungs and the rest of the body. In babies with hypoplastic left heart syndrome, one of these openings closes in the first couple of days of life and prevents blood flow from reaching the body. This results in critical illness and even death in some instances.

JJ cleared a significant hurdle by responding well since his first surgical procedure, known as the Norwood, on April 14 — six days after his birth. The Norwood helps restore function to the underdeveloped heart. The Glenn is usually performed when an infant is 4 to 6 months old, but a routine catheterization procedure this past week identified a narrowing of JJ’s aorta. Surgeon Robert Dabal, M.D., chief of the Section of Neonatal Cardiac Surgery in the Division of Cardiothoracic Surgery, says the discovery — along with how well JJ has responded from his first surgery — accelerated the decision to move up the timeframe for the Glenn.

“JJ has grown very well and looks great,” Dabal said. “It will be slightly more complicated to repair the aortic obstruction in addition to completing the Glenn, but we anticipate that he will do very well. The bidirectional Glenn, though, is much less difficult than the Norwood procedure, both in terms of the surgical complexity and in terms of the physiologic complexity for the patient. The only new issue is that there will be scarring from the first operation that will have to be managed.”

Waldemar Carlo, M.D., pediatric cardiologist at Children’s of Alabama and assistant professor in the UAB School of Medicine Department of Pediatrics, says one-third of HLHS patients nationwide present with a narrowing of the aorta after having the Norwood.

“This is why we do a heart catheterization at about 3 months of age — for the reasons JJ has demonstrated to us,” Carlo said. “We do find important residual lesions like aortic arch narrowing that need to be taken care of prior to or during the Glenn surgery.”

How the Glenn worksThe aortic arch repair will add a little bit of difficulty to JJ’s surgery, just because it is another procedure that has to take place in addition to the Glenn.

With the Glenn, surgeons create a direct connection between the pulmonary artery and the vessel returning oxygen-poor blood from the upper part of the body. It is designed to reduce the work the right ventricle has to do by enabling blood returning from the body to flow directly to the lungs.

“The heart will be very slightly bigger than at the time of the Norwood, but we don’t need to do anything inside JJ’s heart,” Dabal said. “The Glenn involves disconnecting the superior vena cava from the heart and sewing it into the right pulmonary artery. We will repair his aorta by adding a new smaller patch.”

The Fontan Procedure, the third surgery, usually is performed between the ages 2 and 5. In this procedure, surgeons connect to the pulmonary artery and the vessel returning oxygen-poor blood from the lower part of the body, enabling the rest of the blood coming back from the body to go to the lungs. Oxygen-rich and oxygen-poor blood no longer mix in the heart once this procedure is complete, and the child’s skin will no longer look bluish — a common symptom for the child after his or her first few days of life.

Infants who have these surgeries are not cured, and they may have lifelong complications. If the hypoplastic left heart syndrome defect is very complex, or if the heart becomes weak after the surgeries, a heart transplant may be needed.

Life at home with JJJJ spent 57 days in the hospital after his birth and first surgical procedure. Burford says there were many scary moments and some difficult times for her and her husband, James, things not unexpected but obviously tough for any parent to prepare for.

While caregivers worked to nurse JJ back to health, other issues the Burfords knew were possible also leapt to the forefront. Burford says coming to grips with his not being able to eat on his own, for example, was hard.

JJ had a g-tube surgically placed, and when that caused some reflux issues, he had to switch to a j-tube to be fed — a tube that is still used to feed him today.

“We are on a feeding tube 18 hours a day, and that is challenging because it’s very hard to be mobile,” Burford said. “You can take it with you; it’s portable. But it’s not easy to get all of it going and working properly, and you have to make sure he’s in a stable place.”

One of the most disappointing things for Burford was not being able to hold JJ very often early on when he was at his most vulnerable. She is able to laugh now at her initial thought in the hospital, which was that he would not want to be held when they finally got to go home.

“Sometimes when he’s crying, it’s just because he wants to be held,” Burford said. “He loves to be held. You would think being in the NICU like he was might give him some issues with that, but it hasn’t at all. He’s very loving.”

While Burford says the family hoped to get a little more time at home with JJ before the second surgery, she understands that timeframes can — and often will — change.

“The simple fact is that you can’t plan for everything with this kind of situation,” Burford said. “Emotionally, sure, it’s hard. You have to prepare yourself for another surgery, and that’s not the easiest thing in the world to do. But we know he’s in a good place with his health right now. He’s doing great. And if the doctors tell us it has to be done now, that’s what you do.”

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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About Children’s of AlabamaSince 1911, Children’s of Alabama has provided specialized medical care for ill and injured children, offering inpatient and outpatient services throughout central Alabama. Ranked among the best pediatric medical centers in the nation by U.S. News & World Report, Children’s provided care for youngsters from every county in Alabama, 42 other states and 10 foreign countries last year, representing more than 653,000 outpatient visits and nearly 14,000 inpatient admissions. With more than 2 million square feet, Children’s is the third-largest pediatric medical facility in the U.S. More information is available at www.childrensal.org.


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