Congenital heart woes a struggle
Jan. 26, 2004

By RENEE GAMELA
Special to the Observer-Dispatch

By ELIZABETH A. MUNDSCHENK, Observer-Dispatch Rebecca Alford installs software on new computers in a lab at Herkimer County Community College where she is a computer technician. She had a heart condition when she was born and has benefited extensively from cardiovascular research.

Rebecca Alford of Ilion remembers shopping in the early 1970s with her mother in Nichols department store. She also remembers the cashier asking her mother why Rebecca’s lips were purple. Rebecca was not cold — it was a 90-degree day, she said — and she did not eat a grape lollipop either.

Alford was suffering from congenital heart disease. She had a hole in her heart.
“I was born with this funky disease,” Alford said with a mild laugh during a recent phone interview. “I was born without a pulmonary artery.”

Heart Center Online describes congenital heart disease as “a heart abnormality, defect or malformation that is present from birth.” That’s exactly what Alford had, and July 16, 1973, at the ripe age of 5, she was diagnosed with a complex cyanotic congenital heart disease with pulmonary atresia and a ventricular septal defect.

Cyanosis, a bluish or purplish tinge to the skin and mucous membranes, caused the discoloration that made the Nichols cashier wonder if Alford had been sucking on a Charms blow pop.

Pulmonary atresia is a defect where the pulmonic valve (the valve that separates the heart from the pulmonary artery) is completely closed. Without the valve, oxygen-poor blood cannot be pumped from the right ventricle of the heart into the pulmonary artery. Ventricular septal defect is characterized by a hole in the wall between the two ventricles in the heart.

This is a complex medical condition for anyone to understand, especially a 5-year-old child who wonders why she can’t keep up with the rest of her classmates.

“Kids are mean,” Alford said. “My lips are still discolored. I used to get picked on a lot. Growing up, all you want to do is fit in.”

Alford faced a series of surgeries. The first was in 1974 where a Blalock-Taussig shunt increased the amount of blood reaching Alford’s lungs. This decreased the cyanosis and made it easier for Alford to breathe.

The late Dr. Reda Shaher, a cardiologist at the Division of Pediatric Cardiology at Albany Medical Center, told Alford’s mother that although there was no way to correct Rebecca’s condition, research within the next 20 years would find a way to correct it, Alford said.

Shaher’s prediction was correct.

Research at Boston Children’s Hospital had developed an instrument to correct patients diagnosed with the condition.

In 1994, at age 26, Alford prepared for a second surgery. Dr. James Lock, the chairman of the Department of Cardiology at the Children’s Hospital and a professor of pediatrics at Harvard Medical School, was prepared to use a catheterization procedure to close off all ancillary collateral arteries before beginning repair surgery on Alford. Catheterization is a diagnostic test in which a catheter — a thin flexible tube — is inserted into the heart through a blood vessel. For Alford’s catheterization, coils would close off the collateral arteries by being transported via the catheter — a new procedure in 1994.

But things did not go as planned.

Since a collateral artery in Alford’s body had grown too large for the coils to close, Lock had to use something he invented — a CardioSEAL. More commonly referred to as a “clamshell umbrella,” the device was used to close ventricular septal defects through cardiac catheterization. He would use it to close Alford’s exceptionally large collateral artery.

Lock had to obtain approval from the Food and Drug Administration during Alford’s procedure because it had never been approved or used in the manner he was attempting on his 26-year-old patient.

This was only day one of Alford’s surgery.

The second day of her surgery in October 1994 went as planned and Alford said she felt better than ever after this surgery.

It was not until 2001 that the FDA approved the CardioSEAL for repair of septal defects in children — seven years after the device was used on Alford. Research had truly discovered a breakthrough in cardiovascular medicine, and Alford had already benefited from the new treatment.

“I am alive because of research,” said Alford, who underwent a third surgery in 1997.