A few times since Lukas' birth and even beforehand, we were sent questions from others about his diagnosis. Probably the most popular question I have been asked is whether Lukas has DiGeorge Syndrome. I realized that I have never answered this here so I thought I would now.
Lukas does not have DiGeorge. He had a genetic probe after his birth that was finalized just mere days before his operation (although we received the initial FISH results within a few days). Those results showed no q22 deletion (aka DiGeorge Syndrome). This is significant for a number of reasons. DiGeorge and truncus are often comorbid conditions, occurring in about 33.3% of children with a diagnosis of truncus. This is a significant enough finding that we were offered an amnio when we received his official diagnosis. Jonathan and I thought over it carefully and we decided against it. We knew he would be tested at birth anyway and the risk just was not worth it to us. The results of that amnio would not have changed anything in my care or his at the time, so we just thought it would be best to avoid the potential complications surrounding amnio (even though they are small).
The second question we have been asked most frequently is whether Lukas had a partial repair or a full repair. He had a complete repair done on June 20th. The operation which was performed on him is called the Rastelli. I have heard of some surgeons doing partial repairs or "temporary" repairs, but this was not the case for Lukas. Due to his decline before his operation, there was no time for a temporary repair. In fact, a temporary repair was never even discussed with us before or after his birth. The plan all along was to do a full repair once symptoms of heart failure became evident. This is how Dr. Turrentine does things, but every surgeon operates based on a different agenda and timeline. Some like to wait until the child is a particular age regardless of anything else, some surgeons like to perform them immediately after birth. It really just depends on the individual surgeon. The reason Lukas' operation was not performed immediately after birth is because they wanted to allow time for Lukas' heart rate, respirations, and vital signs to stabilize. They also wanted him to gain as much weight as possible before his operation.
These next few things are not so much questions we have received about Lukas or Lukas' care, but rather just general thoughts I believe are worth sharing.
One thing that we did do for Lukas before he was discharged was sign him up for a lifelong study. Basically what this program does is follow him for the remainder of his life unless we or he opts out of the program. His information is collected and stored in a "bank" that other congenital heart surgeons have access to. This study allows congenital heart surgeons to examine the operations performed by other surgeons in an attempt to develop safer, more successful procedures with a prolonged increase in the time between each subsequent operation. Its a great program. We all know that operations vary from surgeon to surgeon and state to state. Different medical professionals do things differently and that is totally fine. This program just allows surgeons from around the world to examine the procedures with the highest rates of success and helps them to educate others about safer, more effective means of operating on these little warriors.
Also, the Contegra graft that Lukas currently has does not have final FDA approval. This might sound scary, but every medical procedure that is FDA approved has to go through these steps. We had to sign several consent forms in order for the Contegra graft to be used and have Lukas' information be part of the study and shared with the FDA about its effectiveness. It has the initial FDA approval, just not final FDA approval. As a result, only select surgeons in the United States are allowed to use them. Dr. Turrentine and his colleague Dr. Brown just happen to be two of those surgeons selected to use them here in the state of Indiana. I watched a video of a truncus repair before Lukas was discharged that used an entirely different type of conduit and valve and even the repair itself was done completely different. If you are curious, always ask your surgeon how they perform the repairs and what type(s) of conduits/valves they use. Our medical team told us the two most commonly used valves are bovine (which is what Lukas has) and donor.
Lastly, we were told after Lukas' operation that his heart is slightly enlarged due to it being overworked in the two weeks before his operation. Dr. Turrentine told Jonathan and I that the walls of his heart were slightly thickened. He was not overly concerned with this finding, but Lukas will continue to be monitored to ensure it is not affecting the performance of his conduit and valve. Additionally, we were told prenatally not to be surprised if Lukas' oxygen saturations were in the high 70-low 80 range. As you know from having read on the blog, up until a few days before Lukas' operation his saturations were always in the high 90s, even though his "target" was only to stay above 75%. His doctors were both happy and concerned with his high numbers for this one reason: higher saturations meant his lungs were receiving more blood, thus working harder than they probably should be. This could have been a contributing factor as to why Lukas went into respiratory distress two days before his operation.
I apologize that I have never directly addressed some of these questions or shared some of my thoughts on the additional topics mentioned. Sometimes that stuff just slips my mind.
Happy Thursday friends!
P.S. - Please pray for one of Lukas' heart friends, sweet little Rebekah. She needs to have another heart surgery, probably sometime before Christmas. You can read about what the operation entails here.
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