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Experience of Westmoreland County, Pa., youth one example of cardiac patient blood needs

Greater Alleghenies Blood Services Region

February 19, 2013
 

While pregnant with her now three-year-old son Avery, Nicole Marzole and her husband, Karl, realized that Nicole’s pregnancy was high-risk, but never imagined the even more serious risks to their unborn child.

Just as she did during her first pregnancy, Nicole, an insulin-dependent diabetic, traveled from the family’s home in Herminie, Pa., to undergo periodic fetal echo and ultrasound tests at Magee Women’s Hospital to monitor the child’s development in the womb. But when she and Karl were told by the technician that a cardiologist was needed for the fetal echo test because the baby was moving so much, they sensed something was wrong.

The cardiologist later shared the grim news. Their son appeared to have a life-threatening heart defect in which his left ventricle was about one-third its normal size and his aortic arch was severely narrowed. Because of his movement, it was also possible that his right ventricle, instead of his left, had the defect, which would have been less serious.

“We were told we had three options,” Nicole recalled. “Terminate the pregnancy, proceed with childbirth and allow our son to have a natural death, or plan for a series of surgeries to begin soon his birth.

“We wanted Avery to have a normal life and agreed that we wanted to pursue the surgical option.”

Even then, surgery was not a certainty. Amniocentesis was done to determine whether Avery’s heart defect was due to genetics. If a chromosomal abnormality was found, the pregnancy would be terminated.

“We later learned the defect had nothing to do with genetics or my being a diabetic. We were told it’s just something that happened in his development,” stated Nicole. “I continued to undergo ultrasounds and fetal echoes to monitor Avery’s growth because sometimes in these cases babies stop growing at six or seven months.”

Avery was delivered by cesarean section two weeks before his April 13, 2010, due date because Nicole began to exhibit symptoms of preeclampsia. Magee had a team waiting in the delivery room to prepare and transport Avery to Children’s Hospital, where the first of three cardiac surgeries would be performed.

“Avery was blue when he was delivered. He was sedated and a breathing tube and feeding tubes were inserted. We weren’t able to hold him until he was six weeks old because of his feeding tube,” Nicole recalled.

A couple of days later, the official diagnosis arrived: Hypoplastic Left Heart Syndrome (HLHS).

The left ventricle had the defect; Avery’s aortic arch was barely getting blood and there was a hole between the left and right ventricles.  “Avery was hooked up to more than a dozen machines and on ten different medications. He underwent his first surgery, called a Norwood procedure, when he was seven days old,” Nicole explained. “Following the surgery, he was placed on 20 additional medications and spent five and a half weeks in CICU.”

Avery’s time in CICU was longer than usual due to difficulties with his feeding tube, which required an additional surgery. By the beginning of June, the family was preparing to take Avery home, but the surgeons suggested that it was possible for him to have his second cardiac surgery.

“During each surgery, the surgeons used at least two units of blood to prime the heart/lung machine, plus had two additional units on hand. They had to take samples of Avery’s blood so often for various tests that he needed a transfusion of 20-40 ccs about every three days to help rebuild his blood,” Nicole said.

When Avery was finally taken home, he was accompanied by an oxygen tank and monitors. A nurse came daily to check on him and make sure he was gaining weight. Each month, for 18 months, the family returned to Children’s for his check-up. He also underwent urological surgery, and a bit later on, his feeding tube was soon after removed.  He began to make normal bodily movements at nine months. He began walking at 18 months and has taken physical therapy.

 All this was leading up to Avery’s third cardiac surgery, which he underwent on December 28, 2011. This surgery, called a Fontan procedure, which changes the pressure in a child’s heart. As a result of the three cardiac surgeries, the heart is reworked so that the right ventricle pumps blood to the body, instead of the left. Fortunately, the surgery was successful on the first attempt. It will be attempted only twice before a child is placed on the list for a heart transplant.

“Avery is now doing very, very well,” Nicole commented. “We still have pulsox and blood pressure machines to monitor him. We also have an awesome pediatrician who is also a certified cardiologist.”

The family’s journey has inspired Nicole to establish Avery’s CHD Avengers (fighting Congenital Heart Defects), to support families and children facing similar challenges. Currently the group makes superhero capes for pediatric cardiac patients and is preparing to make support bags for siblings of such patients. The idea came to Nicole through the experience of her old son, Julian.

The Marzole family also networks with other families as part of a support group founded by Children’s Hospital. They are also involved in charities that help raise money for gas cards and meals to support families of ill children, children being treated by Children's Hospital, Excela Health System in Westmoreland County, as well as by other medical facilities.  

“We have friends and family members whose children were born with holes in their hearts and other defects and have needed surgeries and blood transfusions. I want to do whatever I can to advocate for increased awareness of these conditions,” Nicole stated.

 

Top photo, Avery at approximately three months.

Bottom photo, Nicole holding Avery after he underwent his Fontan procedure.