Our story began on December 27, 1997, when after having had three inconclusive ultrasounds, we were given the diagnosis that our son would be born with Hypoplastic Left Heart Syndrome. Basically, our baby did not have a left ventricle, a mitral valve, or an aorta, all of which are needed to get oxygen to the organs. The news was devastating. We had already lost one son, Matthew, to Sudden Infant Death Syndrome, just four years earlier. The thought of having to endure that agonizing pain and loss again was nearly unbearable.
Dr. Singh, the pediatric cardiologist who made the diagnosis, gave us two options. The first was to have a heart transplant. If we chose that, he said St. Louis would probably be our best bet. The other option he gave us for treatment was a series of three open-heart surgeries, the first (the Norwood) at three days, the second (the Glenn) at three months and the third (the Fontan) at about three years.
After doing extensive research on the Internet and speaking with several parents whose children had undergone the surgeries, we chose the surgeries. The information available showed that too many babies die waiting for a heart transplant, and the five year survival rate was only about 50% (according to what we had read.) According to Dr. Singh, the survival rates for the surgeries were about 75%, 90% and 80%, respectively. These rates were comparable to what we found on the Internet. One additional option we found that parents were sometimes given was "compassionate care", or doing nothing. With this "choice", our baby would likely only live a few weeks. To us, this was not an option. We felt we owed it to our son to at least give him a chance at life.
We had 2 1/2 months between diagnosis and the due date of our son. This was a very difficult time. We'd read and read and read everything we could get our hands on. Sometimes this was good and gave us hope. At other times, the hard reality was too much to think about, and we'd have to take a break. We had to accept many changes along with the diagnosis. I no longer would be seeing the midwife I had come to know so well for my other pregnancies. I also would not be delivering at the hospital my other three children were born at. Our hospital for delivery would be Abbott Northwestern Hospital in Minneapolis. Thankfully, that was only 20 miles from our home. At the time of delivery, the baby would be taken to Minneapolis Children's Hospital and Clinic, which was connected to Abbott via an underground tunnel. The two months prior to our son's birth were filled with meeting with several different perinatologists at Abbott. Following are excerpts from a diary I have kept since we found out about the HLHS.
February 3 - (34 1/2 weeks pregnant)
Dr. Conroy said that when Ben is born, he will look pretty normal until his patent ductus (PDA) starts to close. In utero, Bens body is getting oxygen via this ductus, but as he starts breathing on his own, this ductus will close (which is normal). In Bens case, however, allowing the ductus to close, would ultimately be fatal. To prevent the PDA from closing until they can get Ben to surgery, the doctors will administer the drug prostaglandin intravenously. Dr. Conroy did tell us that a possible side effect of the prostaglandin is apnea, so Ben may be placed on a respirator just to be safe. She also explained all three surgeries again. Each time we hear or read about them, a little more sinks in.
A bumper sticker on the car in front of me on the way home read, Life is fragile - handle with prayer. Yesterday, the sign in front of the church I pass on the way to work read, The power that guides us, comes from the spirit inside us. I have to believe these are hints for us!
March 9, 1998
Dr. Ney explained that there could be a problem with the cord, and she'd hate to send me home just to come in tomorrow to find out something was wrong. So, I was admitted.
At 2pm they broke my water, and just 1 hour and 51 minutes later, beautiful little Benjamin John Cusick arrived, weighing 6lbs. 14 oz. and measuring 19 ¾. The doctors decision to induce may have saved Ben's life because not only was the cord wrapped around his neck, but it also had a knot in it. One more day, and Ben could have been stillborn.
Immediately after delivery, we were able to hold Ben and take several pictures. This was extremely important to me. I did not want something to happen and regret not having held him or having gotten pictures. After the NICU specialty team (who was there during delivery) placed three iv. lines in Bens belly button, they transported him through the underground tunnel to Children's. John went with Ben, and I stayed at Abbott to recover.
After about an hour, I was taken over to see Ben. He looked absolutely perfect. He had already had an ultrasound done on his heart. The good news was that it was not any WORSE than they had seen when doing the ultrasounds on him in utero. The bad news was that it was not any BETTER than they had seen, either.
N.I.C.U. - The Four Days Before Surgery
March 10 - 1 day old
As I held Ben, he looked so content so healthy. Hes definitely the biggest baby in NICU. The others in his room are preemies, one just over a pound. For now, the doctors and nurses say he is doing extremely well. We cant ask for anything to be going better, all things considered.
I am still at Abbott, with John pushing me back and forth between hospitals to be with Ben. The stress, combined with the mega-hormone mess, has made me a big ball of tears! Its so hard thinking about the future. I hope it can go as well as the delivery did.
An additional problem we are facing is dealing with our other two children, Nicole (7) and Connor(3). They have had to endure the stress of the last few months, too, and now they are being passed around from relative to relative as we stay by Bens side. We are so torn. They need us at home, but Ben needs us here.
March 11 - 2 days old
Todays highlights included getting to change Bens diaper for the first time, getting several good photos of him, getting him on video and watching our nurse, Mary, make several sets of footprints for us!
March 12 - 3 days old (1 day
This afternoon, I held Ben for almost three hours. No alarms went off the entire time. It was extremely comforting for me to be with him and really see that he is doing well. His sats are still in the 85-95% range and he has not had to be placed on the ventilator, as we were warned could happen. Ben was put on lasix, a diuretic, today to help him get rid of some fluids, but we were told thats not unusual. Ben is a fighter! He even screams every time he gets his diaper changed!!
Tonight was family picture night. The one thing I really regret about losing Matthew (Bens brother who died of SIDS) is that we never had a family picture with him. Nicole and Connor came in to see Ben (just for the second time), and then we took many pictures. After the kids left, John and I spent some more time with Ben, just rubbing his hands, arms and head, admiring how beautiful he really is. We were allowed to stay in the parent sleep room, just outside NICU, since Ben would be transported to PICU to be prepped for surgery at 7am. PICU is also where Ben would be after surgery.