Monday, February 6, 2012

CHD Week: Jacob's Story

Congenital Heart Defect Awareness Week starts tomorrow! This week I will be sharing stories of CHD warriors to help spread awareness. I thought I would kick off the week (a day early) by sharing Jacob's story. Most of you know his story but for those who don't , here is the story of my little heart warrior:

Jacob Baker

Hypoplastic Left Heart Syndrome
Intact Atrial Septum

November 18, 2009 was a day that I will never forget. My husband and I went for our 20 week ultrasound expecting to hear if we were having another little boy or a baby girl. The joy of learning that we were having another baby boy was short lived. Right after the ultrasound tech got extremely quiet and we knew something was not right. She told us that there was something wrong with the heart. We were devastated and so afraid for our son. The next day we went for a heart echo and his diagnosis was confirmed, our son was going to be born with Hypoplastic Left Heart Syndrome (HLHS). HLHS is a severe congenital heart defect where the left side of the heart is underdeveloped of not there at all. Jacob’s was small and completely closed off. He also had a restricted atrial septum. This is the only outlet blood had to move through his heart and children with that added defect have an even lower chance at survival. We wanted him to have the best chance possible so we decided to travel 9 hours to have him at The Children’s Hospital of Philadelphia.

Jacob was born on March 23, 2010. Within hours of his birth that Septum became intact and they were losing him. He was rushed in for an immediate heart catheterization to open it up. His xray showed his lungs were flooded and the doctors were afraid he would not make it. Jacob did make it, 9 days and 2 bacterial infections later on April 1st 2010 he was stable enough for his first open heart surgery (Stage1: Norwood). This was also the day we were able to hold our baby for the first time. Kissing him good-bye and watching them wheel him to the OR was the hardest thing I have ever done. I told him to be strong and that I loved him. Jacob did very well during his surgery and was off the ventilator 5 days later.

Jacob continued to improve however he had very labored breathing. He couldn’t drink much by bottle because it was too risky and to tiring for him. He had a g-tube and Nissen surgery done on April 20th. After a dose of Morphine I watched Jacob’s numbers on the monitor suddenly drop. The nurse ran in and started patting him and telling him to wake up. They continued to drop and I could do nothing but stand there and watch and pray. The resident ran in and put Oxygen by his face and they hit the alarm; a sound I never want to hear again in my life. The alarm meant they need the crash cart, it’s bad. As a parent every time you hear that alarm you are frozen with fear. About 50 doctors ran in, the room flooded so fast. Thankfully the oxygen brought him back and no further intervention was needed. His body had stopped breathing because the morphine was too much for him. I will never forget the fear I had that moment, thinking I lost him. It is not a feeling I would wish on any parent. It took a few days for the drug haze to wear off but he recovered from his surgeries and we were finally released to come home after 8 weeks in the hospital.

At 4 months old we went back to CHOP for Jacob’s second open heart surgery (Stage 2: bi-directional Glenn). They thought his septum had grown back and went in to cldear some more and a hole was punctured in the back of his heart. This is common when going back to get the septum. Thankfully he has an amazing Surgeon and he was able to (with difficulty) close up the hole and stop the bleeding. Jacob recovered amazingly well and we were home just 6 days later.

You would never have guessed he had just had open heart surgery. Jacob came home on Oxygen because his oxygen would drop whenever we tried to wean him at the hospital. He was on it for a month and then once fully recovered was able to keep his numbers where they should be.

Jacob started drinking by mouth and within a few months he no longer needed to eat by his g-tube. This kid has a HUGE appetite; he made up for lost time lol. When he turned a year old we had some more testing done because of his “unique” breathing. We learned that both of his vocal cords were paralyzed. Scar tissue had built up around the vocal cords tethering them together. His airway is the size of a pinhole. It is amazing he could even breathe without assistance. Only 5% of kids with the airway can breathe without a Tracheotomy tube and he not only has half of an airway but also has half of a heart!

Even though Jacob has continued to be able to keep his oxygen at a good level for his heart his breathing is dangerous for him. He is exerting too much effort just to breathe and as he gets older it will become more difficult. Doctors in the top two airway hospitals both agree that he needs a airway reconstruction surgery. He is scheduled to have it on April 25, 2012. They are going to first try to go through his mouth to cut the tissue and see if that works. If it doesn’t then they will have to go through his throat and use his rib to create a graft which will hold the airway open. We are so scared about this surgery. I guess because he is so happy and doing so well at home. I knew about the heart stuff, I was prepared for it. I knew that he needed that to live. I was not prepared for this. Jacob will also need at least one more open heart surgery (Stage 3: The Fontan) which he will have when he is 3 or 4 years old. It kind of depends on what happens with this airway surgery.

Jacob doesn’t speak much because it takes so much effort to breathe. He makes sounds and says 2 or 3 words. He is learning to use American Sign Language which has opened up a whole new world for us. I am so proud of him and amazed that he has found a way to communicate with us. It was so hard seeing him get so frustrated with not being able to communicate. When we learned about Jacob’s heart condition we were devastated. I never could have imagined that my life would have gone down this path. I hate that Jacob has gone through all that he has but I am blessed that he came into our lives. Jacob has taught me so much about the true meaning of life. He has taught me to slow down and cherish even the smallest moments. I won’t lie I still have days where I want to pull my hair out, I am a mom after all. The point is that even in those moments I realize how lucky I am to have those moments.

This life is scary; a little cold makes me worry that it could take him from me. This life is also beautiful; watching him smile and play like any other child. His smile is brighter than anything I have ever seen. I have formed relationships I never would have known had it not been for Jacob. I refuse to spend my life fearing Jacob’s death, instead I spend it focusing on the beautiful life that we were given. Jacob may need a transplant one day, but the way I look at it is 30 years ago his diagnosis was 100% fatal. Today some centers have a 92% success rate. Who knows what they will come up with in another 30 years. Where there is life, there is always HOPE!


  1. Just wanted to let you know that I linked your blog to mine so more people can read the stories you will be sharing!

  2. Stalking your blog...Reading this story made me BAWL because I am looking at what I might have to face. I'm scared out of my mind! I keep trying to convince myself it will all be ok but that's an uncertainty. I'm so glad Jacon's doing good now. It definitely gives me hope!