June’s Heart Hero of The Month

Heart Hero Angel BJ

Our CHD Journey … 

by, Heart Mom Haley

In March 2005, after four years and three miscarriages, my husband and I found out we were pregnant. We were absolutely over the moon ecstatic. That June we found out it was a boy. I was diagnosed with gestational diabetes and had to go for weekly stress tests to monitor how the baby was handling my blood sugar issues. I got up and went to McLeod’s for one of these NST’s the last week of August and the nurses noticed the baby’s heart rate wasn’t doing what it should have been doing. They called in an ultrasound tech to do a biophysical profile just to check and see what was going on. After that my OB, Dr. Gary Emerson at McLeod Women’s Health came in and told me he saw that something wasn’t right with my baby’s heart. He sent me and my husband over to the Pediatric Cardiologist, Dr. Charles Trant (McLeod Pediatric Subspecialties), who did a fetal echo and confirmed that our son had a congenital heart defect known as Hypoplastic Left Heart Syndrome. Basically, he has half of a heart, he has two pumping chambers instead of the normal four. Scared doesn’t even begin to cover how we felt. In early September we had our first meeting with the Pediatric Cardiology team at the Medical University of South Carolina and were told what to expect.

The possibility that our son would not live was very real.

On October 29, 2005, I gave birth to a 4 lb 12 oz 17 1/4 inch long baby boy. 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 and 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 handheld my finger tight. I knew then that I’d give my life for this little person. Four days after he was born, before his first open heart surgery I got to hold him for the first and possibly the last time.

The first surgery to correct HLHS is called a Norwood procedure. BJ (Brian, Jr.) came through that surgery amazingly well. When he was 5 months old he had the second corrective surgery for HLHS, a bi-directional Glenn. He came through that surgery just fine. At three years old he went in for what was supposed to be the last corrective surgery for his condition, a Fontan procedure. No one, not even the doctors could have predicted what would happen next. While he was in the PCICU after the Fontan the doctors noticed something was not right and they took BJ to the cath lab to make a small fenestration in his heart to help with the new blood flow. BJ coded on the table and we lost him for over three minutes. We were in the waiting room about 2 am that morning and the hospital Chaplin and the doctors came in to tell us what had happened. They don’t know why but his body rejected the new blood flow and they had to go back in and reverse the Fontan back to a Glenn. BJ had also had a stroke that affected 80% of the left side of his brain. We were advised to call our family in because they thought he would pass within the next 4-6 hours. That turned into 24 hours, then a week, then a month. We spent 178 days in the hospital before we came home.

The way our community pulled together during this time of need still amazes me. We spent a lot of time in and out of the hospital in 2016 because BJ struggled constantly with going between being dehydrated and fluid buildup on his lungs. On July 21, 2016, BJ’s care team called a conference to speak with my husband and I and the cardiologists said there were no surgical options left for BJ. His pulmonary arteries are just too small to support his heart and lungs. He was not eligible for a transplant because he wouldn’t survive the surgery and if he did his kidneys couldn’t handle the medications he would be on after it. They were only functioning at 50%.

We spoke with the MUSC Palliative Care team and decided to put BJ on hospice. Our goal was to make him as happy and comfortable as possible for however long we had left with him. Hands of Hope Hospice was one of the best decisions we could have made for BJ. End of life care is just as important as the beginning of life care. In August 2016 Dr. Geoff Forbus, his cardiologist at MUSC, called and told us about a new procedure he thought could benefit BJ. Dr. Scott Bradley placed a shunt from BJ’s intra jugular vein to his carotid artery. This helped the blood cycle through his lungs twice instead of one. We knew it was a temporary fix and we let BJ decide as to whether he wanted surgery or not. He chose to fight, to try.