Qadar's Journey With HLHS

The Fontan has been completed! Yay, the last surgery (for a while at least). He underwent his surgery without any complications. He had the Fontan, arrhythmia surgery, and a new pacemaker. The pacemaker that he originally had was replaced with a dual-chamber pacemaker. He is currently, recovering in Children’s Memorial Hospital.

We had to report to the hospital at 6:00am, Friday April 23, 2010.  He was given a sedative just before he was taken into the operating room around 7:20am. That was when the waiting game began. I got my first update around 11:30am; I was informed that they had a difficult time putting I.V.’s in because he was hard finding his veins (which I knew; because he has such small veins). Needless to say that they did get lines in him and that he didn’t feel a thing. I was also told that he had just gone on the heart-lung bypass machine and they were draining his heart so that they can begin to work on it. I was told that I would get another update around 2:30pm/3:00pm.

When I got my update at approximately 3:00pm, I was told that the surgery was a success and that his surgeon, Dr. Backer will be out to talk to me. The physiologist was the first to come out and give me her update. She said that there were no problems during the arrhythmia surgery (also called a Maze). They went in and froze the arteries that were causing the arrhythmias. They also took out his old pacemaker and replaced it with a dual-chamber pacemaker. The old pacemaker that he had only had one lead, the new one has two leads. She also told me that I will be getting his old pacemaker and that his surgeon will give it to me once he comes out to give me his update.

When Dr. Backer, the surgeon, came out to give me his update he gave me the old pacemaker and a piece of the material used in redirecting his blood flow.  Wow, what souvenirs Qadar will have when he gets older!

I was able to go and finally see him about 4:15pm and of course he was sedated and resting comfortably. He had a breathing tube in, of which, they were going to take out tomorrow (Saturday). 

On day two: Saturday, April 24, 2010: He was in and out of sleep, waking up with occasionally bouts of fussiness because he wanted the breathing tube out. After a couple of hours of weaning him off the ventilator he was finally given his wish – the breathing tube came out. As he’s becoming more and more alert he fusses, not because of pain; but, because he wants to drink – at this point anything. He can’t drink right now because they want the swelling to go down in his throat before that give him any liquids. To moisten his palette; I’ve been allowed to give him dab of water on a swab. He wants to overdo it with the dabs, of course, but we can’t give him too much. Hopefully, in another couple of hours he will be allowed to have a cup. If anything else comes up to report on day two I will post; otherwise, I will post tomorrow for day three.

I will upload photos soon.

I decided to share our story because it is an extraordinary one that my son had overcome and I wanted to share our experience with family and friends but mostly with families new to the Congenital Heart Defect Community. To offer hope, be an inspiration, and be a living testament of so many survivors.

On August 28, 2006 my son, Qadar, was born with a congenital (present at birth) heart defect called Hypo-Plastic Left Heart Syndrome (HLHS). HLHS “is a congenital birth defect that affects the left side of the heart. HLHS is a condition in which the left side of the heart, which pumps blood to the body, fails to develop properly. It is the most common fatal congenital heart disease in newborns; without treatment greater than 95% die before one month of age. Most babies die by ten days of age if the condition is not diagnosed”. Qadar’s condition was known before his birth so there was a special medical team assembled, in my room, that was ready to receive him and tend to him upon delivery. I was only able to admire him from a distance, say I love you and kiss him from a distance before he was stabilized and whisked off to Children’s Memorial Hospital in Chicago. I was in recovery for less than 24 hours before I discharged myself to go be with my baby.

Oh my God, he was so adorable laying there; surrounded by a soft plastic apparatus shielding his crib, in place to keep in his own atmosphere, so peaceful and content not knowing what was to become of him in the days ahead. I remember he didn’t like to be touched; every time the nurse would take his vitals or run tests he would always cry but the moment you took your hands off of him – instant silence. I learned that just by observing him. I tried putting booties on his feet. Guess what, he cried and kicked them nearly off; but, the moment I left him alone there was silence.

I remember staring at his chest watching him breathe wondering why?

There is no concrete understanding as to why this defect occurs. Most research I’ve come across says outright, “they don’t know”. Others attribute it to either the environment or genetic factors.

The survival rate remains to be seen. Thanks to Dr. Norwood, Dr. Glenn, and Dr. Fontan for creating procedures that ultimately extended life expectancy greatly because prior to 1983 (the year Dr. Norwood created the procedure) there was an extremely high mortality rate. HLHS patients have to have a total of three surgeries to ultimately get the right side of the heart to do the work of both the left and the right.

On September 1, 2006 he underwent his first procedure – the Norwood. The Norwood, considered the most complex and the riskiest, offers a 75% survival rate. This procedure has to be done within days of birth because the heart cannot pump blood to the body. The procedure entailed disrupting the blood flow to the lungs and creating a new flow to the lungs along with a host of other redirections, separations and incisions. We stayed in the hospital only two weeks. The doctors was quite pleased with his recovery and we were sent home with a daily routine of giving him a daily medication regime, monitor his oxygen levels daily (it couldn’t drop below 70%) and his weight. By following this daily routine we increased his chances of survival.

About a month after his first surgery, October 11, 2006, I called the hospital because the monitor kept reading that his oxygen levels were  52% (it was 70% the day before; but, each day prior it was dropping a percent). At 7 weeks old I took him in to see cardiology.

It was discovered that the stitching came away from the shunt that they used, as part of the Norwood Procedure, to re-direct the blood flow. We were admitted immediately, do not pass go, go directly to Pediatric Intensive Care Unit (PICU) and scheduled to be the first case the next morning, October 12^th. During our stay, his oxygen levels remained at 52%. It was discovered that if I held him exactly in an upright position, literally, his oxygen levels rose to 70%. So for the rest of the evening, overnight, and following morning, until the nurse was ready to take in into surgery, my baby remained in my arms in an upright position.

He ended up on the heart-lung machine longer than anticipated; so, his kidneys took a hit. He lost kidney function for approximately 2-weeks. The doctors couldn’t tell me if his kidneys would come back, because they said that his case has never happened before; so it was a waiting game for all of us. After a couple of days, wait he ended up going on Peritoneal Dialysis until his kidneys back two weeks later.

We ended up staying in the hospital just over a month having been discharged November 18, 2006.

He successfully underwent his second surgery, the Glenn, April 2007  and we came home in about a week.

I called his cardiologist October 31, 2007, because I was concerned that his hands and feet were swollen. He also had swelling under his eyes. Without hesitation he had me come it in, during testing it was discovered that he was in Congestive Heart Failure (CHF) and once again, we were admitted immediately, do not pass go, go directly to Pediatric Intensive Care Unit (PICU). This time they didn’t have to go back in and make any heart repairs. We spent nearly two weeks in PICU being treated intravenously with heart medication. Thereafter we went home, as with all of his discharges, given a 30-day follow-up appointment to be seen by the cardio-thoracic doctors.

When we returned, December 13, 2007, for the follow-up appointment, guess what, we were once again, admitted immediately. Do not pass go, go directly to Pediatric Intensive Care Unit (PICU) because he did not improve. In fact it was determined that he needed, Permanent Pacemaker Placement.  It was known that he would eventually need a pacemaker somewhere around his teenage years; it ended up being much sooner rather than later.

Since that last discharge on 12/25/07, thank God, we have not had any hospitalizations. He has to undergo one more surgery, the Fontan.