Remembering BJ

BJ (Brian, Jr.) was born at MUSC on October 29, 2005 and weighed 4 pounds, 12 ounces and was 17.25 inches in length. His parents knew ahead of time that BJ, their first child, would be born with a congenital heart defect known as hypoplastic left heart syndrome (HLHS).

Mother Haley had been diagnosed during her pregnancy with gestational diabetes and as a result underwent weekly non-stress tests to monitor how the baby was handling her blood sugar issues. During one of those tests, the nurses noticed the baby’s heart rate wasn’t within normal ranges. So her OB ordered an ultrasound that led to a referral to a pediatric cardiologist, who confirmed that BJ had HLHS.

“Basically, he had half of a heart, he had two pumping chambers instead of the normal four,” Haley said. “Scared doesn’t even begin to cover how we felt.”

The Centers for Disease Control & Prevention estimates that one out of 3,841 infants are born with this critical condition, and the often the causes of hypoplastic left heart syndrome are unknown.

She recalled when he was born. “I never got to hear him cry. As soon as I gave that last push that brought him into the world, a team of doctors and nurses ran into a room to begin working on him. I will never forget the overwhelming fear that came over me at that point.

“Later that night my husband I were allowed to go see him. I looked down at this tiny little boy who had machines beeping all around him, an IV ran through his bellybutton, and he looked back at me and his little hand held my finger tight.”

Over the course of the next 13 years, BJ would undergo multiple surgeries to correct the heart defect, beginning with the Norwood procedure not long after he was born. A new aorta is created and connected to the right ventricle. A tube is placed either in the aorta or the right ventricle so that blood can be pumped to the right lung and the rest of the body.

At five months, he returned to the hospital for the Glenn procedure, a surgery that helps to reduce the work of the right ventricle by allowing blood returning from the body to flow directly to the lungs. 

When he was three years he had what was expected to be the last corrective surgery, the Fontan procedure. Unfortunately, the procedure did not work. His body rejected the new blood flow, and they had to go back in and reverse the Fontan, because doctors felt he would not survive the next 4-6 hours.

But he did and those hours turned into 24 and then a week and then a month. They spent 178 days in the hospital before going home.

He would have more procedures and many more physician visits and ultimately, at age 13, his courageous battle ended and BJ passed at MUSC Children’s Hospital in September, 2019.

Remembering her son, she said, “If everyone had even an ounce of his faith, they would all go to heaven. He was fun, kind, sweet and smart. He was a light for all of us.”

Haley started a Facebook page to keep friends and family informed about BJ’s progress. BJ’s Little Light Foundation has had nearly 4,000 visitors who have friended the page.

She also spoke with great appreciation for the physicians and nurses who “went above and beyond” to care not only for BJ but also the whole family including brother Raylen, who arrived in 2011.

Her gratitude extended to family, friends and the local community that stood by and offered support to BJ and his family.

She also recognized many MUSC care team members, who always explained things without “sugar coating” anything so that they were fully prepared and understood what BJ was experiencing. She especially called out the Palliative Care Team including Dr. Conrad Williams and Hanna Epstein, who made things easier for everyone. She valued Dr. Scott Bradley, his pediatric cardiac surgeon, and Dr. Geoffrey Forbus, who came in to be with BJ on his last day even though he was off that day, and his wonderful nurse Christie Jacobin, who he affectionately called Christie with the brown hair.

For parents who have a similar journey, she said, “It’s a roller coaster ride. Make yourself as knowledgeable as you can. The doctors and nurses at MUSC Children’s are wonderful.”