I brought Evan to the ED due to increased breathing/desats and continued feeding issues. I could just see in his eyes that he was not feeling well and it was time. We spent the majority of the day in the ED where he had an x-rays and an echocardiogram. Due to Evan’s history and the fact he wasn’t easy they wanted to keep him overnight for monitoring. I fully expected to see a GI (gastroenterologist) and they would figure out why he wasn’t eating and we would make some feeding adjustments and go home. We got a room upstairs and spoke to the doctor and she didn’t have the results of the echo and probably wouldn’t until morning. Shortly after she left she came back and said the attending doctor got an initial echo report that was very concerning and Evan would need to be moved to the CICU. The echocardiogram revealed three out of the four pulmonary veins were narrowing causing his heart to be in failure. The most concerning was how rapidly it progressed. For those of you just joining our journey we knew of one vein narrowing which isn’t necessarily concerning if it is only one vein. The narrowing of the veins is called pulmonary vein stenosis and is unrelated to his first surgery.
Pulmonary vein stenosis is a very rare and serious condition with a very ugly prognosis. Our pulmonary veins bring oxygenated blood back from the lungs and into the heart. The heart in turn pumps the blood out to the body. A healthy heart has four pulmonary veins and three out of four of his were showing signs of narrowing. If the blood from the lungs can’t get into the left side of the heart it causes blood to back up in the right side of the heart which causes increased pressures and ultimately right side heart failure because the right side of the heart has to work so hard in order to compensate for the increased blood flow. It is actually somewhat hard to see these veins on an echo so in order to get a better picture of what was going on and confirm pulmonary vein stenosis (PVS) a CT scan was ordered where they inject Barium in his veins and follow it through and if they didn’t get good pictures he would need another heart catherization. Evan had his CT scan on July 8th which required him to be sedated and on a ventilator during the procedure because they need him to hold his breath for 10 seconds during the test. He did not have any issues with anesthesia or the ventilator and was sleepy afterwards but resting peacefully. He also got a PICC line placed. A PICC line is a peripherally inserted central catheter and can be used for a prolonged time to administer medicine and blood draws. Evan is a very hard stick and doesn’t give blood easy so this would prevent him from becoming a pin cushion. It would also allow him to get a blood called milrinone which takes some of the pressure off of his heart.
The CT scan results confirmed PVS. We were initially told there was not a lot that can be done to fix PVS which makes it such an ugly prognosis …..BUT God. Evan
‘s upper right vein is very small and can’t be repaired surgically… But God. The lower right vein looked good and was compensating for his upper right vein. The two veins on the left formed in a way that they come together and then narrow as they entered the heart so all of the blood was trying to get through the narrow space..But God. The good news they were willing to operate and perform what is considered a sutureless surgery which would open the veins and allow them to grow as Evan grows….Look at God. The bad news is the surgical repair is often temporary as the PVS typically returns….BUT God!
I had done some research on PVS and learned about a trial at Boston’s Children’s Hospital and was going to call for a second opinionand had mentioned it to the doctor’s here in Pittsburgh. After the CT scan the cardiologist mentioned the trial and the heart catherization doctor had colleagues at Boston that she was going to reach out to regarding the trial but her understanding was that the trial was closed… But God.
On Friday, July 11th we received news that the trial in Boston is still open but we would have to get everything done there and essentially move to Boston…. but God. I got the email addresses of the contacts at Boston and emailed them right away. It was late on Friday when I received the news so I went to Google to get a phone number and the first person to answer was a woman who used to be the coordinator for the PVS trial. She confirmed the trial was still open and we could maintain Evan’s care and surgery here in Pittsburgh we would just need to go to Boston for the first dose of the drug… But God. I will provide more details on the trial but essentially a drug called Gleevac which is a chemotherapy drug is given after surgery once the veins are open and the prayer is that the drug keeps the veins from renarrowing. The drug has shown some promising signs of keeping them open. Evan was stable and looking great so they saw no need to keep him in the ICU so we got transferred upstairs to the acute care floor and the goal is to get him to eat in order to prepare for surgery.
Evan continued to have poor feedings by mouth so an NG tube was placed and the goal is for him to have 3.5 ounces of formula every 4 hours. He tolerated them fairly well in the beginning and is being monitored closely.
Don’t be afraid for I am with you. Don’t be discouraged for I am your God. I will strengthen you and I will hold you up with my victorious hand. – Isaiah 46:10
Evan’s heart may be sick but his spirit is not broken. He is smiling and talking and he helping us guide us in our decisions. He is a beacon of light and everyone who meets him falls in love with him immediately. It is the God in him that draws people near. I can’t tell you how many times one of the cardiac doctors in the ICU have just come in his room and said “I just needed a break and you make me happy.” I will not allow a number and what the doctor is telling me to deter me because my faith is in God and I know he is healed and made whole. Evan is truly a warrior with a radiant spirit.