Shabby Blogs

Monday, April 4, 2011

Our Real Jack of Heart

Hi everyone! I am so happy to start this blog and please bear with me because I am learning about blogging and working on this site (thanks to a wonderful friend). I want to use this blog to bring awareness about Congenital Heart Defects (CHD) and Hypoplastic Right Heart Syndrome (HRHS). Until we found out about Jack's CHD we had no idea that they are the number one birth defect! Once we started talking to people it seems everyone knows someone who has been affected by some type of CHD. Jack has been diagnosed with HRHS which is a serious and rare heart defect. Specifically, Jack has been diagnosed with pulmonary atresia with intact ventricular septum. HRHS makes up approximately 6% of all CHDs. One of the biggest problems we found when we started searching the internet for information on HRHS was there is little information, most of it is very dated, or your search will lead you to the more common and more serious Hypoplastic Left Heart Syndrome. We decided a blog would be a good idea so people could see our story and hopefully it could help someone with their HRHS journey. We found that other family’s blogs were the most helpful to us in understanding what we are getting ready to take on once Jack is born. Another reason for our blog, and most importantly, we wanted an easy way to keep our wonderful family and friends up to date because we probably won't feel like making calls while we are so focused on Jack’s surgeries, recovery and getting him HOME!! So here is how it all began. Jackson and I knew once we were married we wanted to have a family soon after. I did everything I could in preparation for a healthy pregnancy. I started prenatal vitamins months in advanced, ate healthy foods, and did things pregnant women should do. From the start I have had a healthy pregnancy, but it just goes to show that God has a plan for each of us no matter how hard we try to plan it for ourselves. Our second ultrasound at 17 weeks started out wonderful. We found out we were having a boy and just about everything appeared to be perfectly normal. They told us that they didn’t want to alarm us but they could not see a few things on Jack's heart and that it was likely the fact that he was still so small and moved around the whole time they were trying to look at him. They wanted to look again in 2 weeks. At 19 weeks we went back and had a second look. Again, he moved around like crazy and they could not see parts of Jack's heart. My OB explained that they still wanted to look again in a few more weeks, but he thought something may be wrong with the right side of Jack’s heart. It appeared everything was there but the right side was small (hypoplastic). He told us about Hypoplastic Right Heart Syndrome and that it was possible Jack had HRHS. He wanted to look again to be sure before he sent us to a specialist. At 23 weeks we returned for a third look at Jack’s heart. This time we had a different ultrasound tech and I think God planned for us to have her that day (and every time since). She was maternal, caring, so sweet and really took her time. She began the ultrasound looking at Jack head to toe (I assume to help calm our nerves) before going to his heart. She looked at his heart and calmly told us that there was definitely something wrong with Jack's heart. Out of the corner of my eyes I saw Jackson's head dropped and tried my best not to start crying- yet. The ultrasound tech took her time showing us that the right side was smaller and the valves were not opening and closing like the left side. She answered tons of questions we asked her. My OB came in and told us he suspected it was HRHS, wanted to send us to a specialist and what we could probably expect would happen. We went the next week to see the specialist, hoping something in his diagnosis might change, but our cardiologist confirmed it was HRHS. After recovering from our initial shock, Jackson and I both are accepting of the fact that this is the plan God had for us, He will take care of us and be there with us through out this journey. Since then we have found lots of positives in the process. We have met many wonderful new people and gotten to see Jack on ultrasound so many times! This past week we had our initial visit at MUSC. First we met with the cardiology team and they did an echo on Jack’s heart for an hour and a half. They again confirmed it was HRHS and told us they will not know all the details of his heart until he is born. They also found that Jack has developed at least one sinusoid, which are common with HRHS, but can cause complications after birth. Sinusoids can cause restriction of the coronary arteries and restriction of the coronary arteries would drop Jack’s survival rate significantly. As long as the coronary arteries are not restricted we have over a 90% chance of survival. If there is coronary artery restriction it seems our only option will be heart transplant. Please pray for Jack to have the best scenario possible to use the heart he given. Jack’s due date is May 24th. My best friend agreed to let a very pregnant woman live with her a week before so I can go into labor naturally, as recommended by the OB team at MUSC. Jack will have his first open heart surgery, the BT Shunt, within 7 to 10 days after delivery. The wait is so Jack can get accustomed to the world outside the womb and they can get a better look at his heart. They told us to plan to be in Charleston for 3 to 4 weeks. At 5 months, we will return to Charleston for Jack’s first heart catheter. They will see how his heart is doing and schedule us for his next surgery the Bi-directional Glenn, probably at 6 months. Jack will have his third and final surgery around 3 years old, the Fontan, and should live a very normal life afterward, only taking a baby aspirin every day. MUSC has 100% surgical survival rates on the Bi-directional Glenn and the Fontan. That amazes me and the fact that the only medication he will need is a baby aspirin!! So that should bring you up to date. If you have any specific questions, I would be happy to answer them just shoot me an email! I am hoping to add a link soon where I will describe, to the best of my ability, HRHS and the specifics of Jack’s heart.