Hypoplastic Left Heart Syndrome - HLHS {CHD week}

(Joshua - 4 months pre-Glenn)

While there are many congenital heart defects, Joshua unfortunately has one of the most severe, if not the most severe. He has hypoplastic left heart syndrome, or HLHS. I still remember when the doctor who diagnosed him uttered those words & thinking to myself, "huh? Was that English?" Then a moment later I realized that incredibly long name would become a regular in our vocabulary. I don't often think back to the day when we found out because it was really tough. The short version goes something like this; I got a phone call from genetics the morning after our 20 week ultrasound. The woman wanted us to come back in immediately for more testing because it "appeared that a portion of the baby's heart looked smaller than the other side."
HLHS is as simple as that (if that is simple). Joshua's left heart is severely underdeveloped. Therefore, his heart required surgery at birth in order to support life.  Other options included transplant, pallative care and termination. We chose to have Joshua undergo the Norwood procedure, as we felt that it offered the best shot at life. The Norwood procedure consists of 3 stage surgical intervention. The Norwood at birth, the Glenn at a few months old (Joshua was 4 months) and the Fontan during the toddler years (or there about). The result of the procedure allows the heart to function with a single ventricle; the right ventricle in Joshua's case. It's actually pretty fascinating the way his body functions right now. The blood flows passively to the lungs from his upper body & then goes to the heart where the right ventricle then pumps the oxygenated blood to the body. After he has his Fontan the blood flow from his lower body will also be directed to the lungs & then to the heart, allowing him to function with higher O2 levels than his current state, which is at around 80-85%. If you know Joshua, you may have noticed that he looks a little bit blue. After he has his Fontan his O2 levels should be in the high 90's, and hopefully his lips will be pinker!

We have mixed emotions about sending our little guy back into surgery. While we know that it's for the best, it's awfully hard to imagine going through recovery again, this time with a boy who can very clearly communicate with us. When he was a baby I told myself time & time again that he wouldn't remember all that he had been through. It'll be harder to convince myself of that this time.

When he had his previous 2 surgeries he went into each of them in poor health. He had tough recoveries that left us in the hospital for 6 weeks each time. When he has his Fontan, I assume he will be in good health. He's enjoyed good health for nearly 2 years & I have faith that he will remain strong heading into the Fontan, which should theoretically make for a faster recovery.

Until recent years, there has been no hope for children born with this disease. We are so very fortunate that Joshua was born at a time where success rates are improved and real hope exists. With a few survivors now in their 20's, there isn't enough data to conclusively say how long Joshua's heart will provide him life. His heart will eventually fail him and he will need a heart transplant. We have no idea when that will be. It could be next year, it could be when he's 20, or 30, we simply don't know.

Having a child with HLHS has taught us many lessons. Lessons that I may not have learned otherwise. I try to see the good in the situation and truthfully while I wish I could take away the pain & suffering, I wouldn't change Joshua. He is a remarkable boy, because of what he's been through. He has the ability to overcome obstacles that others couldn't, and he doesn't even notice them. If I possessed a fraction of his determination, I'm pretty sure I could walk on water.  Obviously, that miracle isn't happening any time soon, but it's okay, because he is our miracle.