Boy born with half a heart survives and thrives

May 27, 2009

Four-year-old Jason is still going strong after three open-heart surgeries.

Rhonda Ellison was six months pregnant when she and her husband, James Gaines, got the kind of news all parents dread going into an ultrasound: something was wrong with their baby. They needed to go to the Children’s Heart Center at Golisano Children’s Hospital.

The couple consulted with a cardiologist and were devastated to learn that Jason had hypoplastic left heart syndrome, a condition that meant the whole left side of his heart – aorta, aortic valve, left ventricle and mitral valve – was underdeveloped. The left side of the heart does the critical job of pumping oxygenated blood to the rest of the body. Hypoplastic left heart syndrome is the leading cause of death from cardiovascular disease during the first two weeks of life and is responsible for about 2 percent of all congenital heart defects. Without surgical intervention within days of birth, babies with the syndrome die.

Initially fearing the worst, Rhonda and James were relieved when Dr. (George) Alfieris (chief of pediatric cardiac heart surgery) assured them that there was a medical procedure that could increase Jason’s chance for survival.

On Sept. 12, 2004, Rhonda went into labor and she delivered almost without incident.

“I got to hold him, and he cried, just like my other babies. He was actually breathing on his own,” Rhonda said.

Alfieris and his specialized team had three goals going into the first surgery: to open the wall between the top chambers of the heart so that both the oxygenated and blue (or unoxygenated) blood in the heart could mix; to rebuild a very small aorta and allow it to receive blood from the right ventricle; and to place a shunt or tube from the aorta to the lungs so that blood could get to Jason’s lungs.

“His first of three planned surgeries went very well, however, his breast bone was not put back together immediately because we were concerned about swelling. His chest remained open for about five days after surgery,” Alfieris said. “He’s a really strong little boy.”

Caring for Jason was not the same as it was for the couple’s older children (Jason was one of 11; he now has a little sister, too). Rhonda and James were nervous about keeping him safe, but his cardiologist, Cecilia Meagher, M.D., reassured them Jason was doing well.

Six months later it was time for Jason’s next surgery. The second surgery connected the blue blood usually returning to the heart directly to the lungs. Jason pulled through the surgery well but he developed a chylothorax or a building up of lymphatic fluid in the chest cavity, a serious complication if not caught early. Jason’s team, which included Regina Cable, N.P., caught the condition early. A chylothorax can develop in children after open heart surgery due to high vein pressure in the lungs. To treat it, the fluid is drained from the lung cavity and then the child is placed on a low-fat diet.

“Reducing fat consumption is important because fat is absorbed into the lymphatic system and – in this situation – then leaks into the lung cavity making it difficult for the infant to breathe,” Cable said. “Jason was on a low-fat diet for about three months.”

Until Jason’s lungs were mature enough to handle the final procedure, Meagher kept an eye on his heart’s development, making sure he would be ready. Other than some routine childhood illnesses, Jason was doing well.

On Nov. 3, 2008, Jason had his third open-heart surgery. During a seven-hour surgery, Alfieris finished routing Jason’s blue blood past his heart and to his lungs and allowed the right side of his heart to just pump oxygenated blood to the rest of Jason’s body. The surgery took seven hours, but it felt like an eternity to Rhonda and James.

“The surgery went well. It was only a few days before we were able to unhook the ventilator,” Alfieris said.

Unfortunately, Jason was unable to keep his oxygen level high enough, so he was reattached to a ventilator. But his blood oxygen levels remained very low.

“Then he had some changes in his lungs that scared all of us and required an emergency procedure in the pediatric cardiac intensive care unit (PCICU),” Meagher said.

An ultrasound of Jason’s heart revealed a clot in the tube that was taking Jason’s blue blood directly to the lungs. Jason was given a clot-dissolving medicine and that decreased the size of the clot. Daniel Miga, M.D., a pediatric cardiologist, took Jason to the cardiac catheterization lab to determine the cause of his low oxygen levels and discovered that his left lung was not picking up oxygen. Jason’s heart was functioning well, but his left lung had completely collapsed.

“They kept trying things, but they didn’t know a magic fix. That’s when they decided to open up his chest again,” Rhonda said. “His dad called the family to come to the hospital, fearing Jason, who was clinging to life, would not survive. I remember Dr. Meagher hugged me. The fear was paralyzing.”

Jason’s breast bone was reopened to give his lungs more room to expand. When Rhonda came out of the PCICU, Jason’s extended family was there. It was reassuring for Rhonda to have them there, but the reason for their presence was unnerving.

“It was very scary, but I didn’t give up on him. I never do,” Rhonda said. “I knew in my heart that my son would pull through. I knew he wasn’t going anywhere.”

Jason stayed in the hospital for more than seven more weeks and his chest was left open for the majority of that time. A special wound healing machine was placed on his chest to help him heal and allow him to breathe off the ventilator despite his breast bone being separated.

“Dressing changes in this situation can be very frightening especially to a 5 year old but Jason handled it like a trooper,” Cable said. “When Jason’s wound was close to being healed he was discharged home.”

Rhonda still had to change his incision bandage once a day and bring him back to the hospital weekly. Today, his parents say the whole experience hasn’t really fazed him, even if his mom and dad continue to worry about him.

“When I check the area, Jason will often ask, ‘How does it look? Am I OK? Does it look good? Or, are we going back to the hospital?’” Rhonda said. “He isn’t scared by any of it. He’s our miracle child.”

Join Jason and his family for Miracle Weekend

The 13th annual Stroll for Strong Kids and 5K Run will take place 9 a.m. to 1 p.m. Saturday, May 30, on the Wilson Quad at the University of Rochester. This year’s event is sponsored by M&T Bank. For more information or to sign up for the Stroll or the new 5K this year, please visit www.gchas.org.

The Gaines, along with four other special Miracle Kids and their families, will share their remarkable stories at the annual telethon on 10NBC, live from the Strong Memorial Hospital lobby, 10 a.m. to 1 p.m., and 7 to 11 p.m., Sunday, May 31. The telethon is sponsored by Perkins Restaurants. To make a pledge during the event, please dial (585) 241-KIDS or log on to www.gchas.org.

For Media Inquiries:
Heather Hare
(585) 273-2840
Email Heather Hare

 

 

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