The last week and half Orion just wasn't himself... he was grumpy, slept for more than 3 hours on several occasions (we knew it was too good to be true), threw up often, and stopped eating his beloved finger foods. Normal child development diagnosis would point to reflux and his body no longer responding to his reflux medications. We switched from Zantac to a Previcid; a drug that stops stomach acid production all together. The vomiting once a day continued.
Thurday: So after consulting with our cardiologist on thursday we decided to come-in to get an echo. Our Dr scheduled one the next day during his clinic hours. Tawny had an appointment the next day, Friday, at Stanford so we said lets make it a Stanford day! Little did we know it would be a Stanford couple-of-weeks. We'll go to Tawny's appointment in the morning and then go get Orion' echo and go over the results with the Doc then come home that night... that was the plan.
Friday: (ready for the technical side of things?!) We came in for an echo and the results showed that the wall (Atrial Septal | AS) between the right and left atrium (RA and LA respectively, consult heart picture below) was slightly curved toward the left atrium. This was not too alarming because we already knew that the RA's pressure was elevated due to leakage from the tricuspid valve (valve between RA and right ventricle(RV)) <- been programming too much with the double parentheses. So Dr. Axelrod (our cardiologist) examined O, heard some noises and ordered a chest xray to check for plural effusion. The xray came back positive for fluid around his right lung which combined with the curved AS , no fever, vomiting (the lung becomes slightly enlarged which can push up on other organs, and poor guy, through personal experience i know how painful that can be), long sleep periods/drowsiness, pointed towards early heart failure, or as our cardiologist eloquently put-it to keep us from freaking out, "his heart's not doing so well, so we are going to drain that fluid form his right lung first and the do the cath very soon." So we were admitted to the general floor and O was put on IV lasix to drain the fluid.
Monday: They monitored him daily and the IV lasix worked like a charm. By Monday, O was back to his cheerful self, and his chest xray showed up clear! Yeah... so they decided to schedule him for a cath the next day. We were worried, they said he will get the cath tomorrow and depending on how he does after can go home the same day. We hate getting sent home right after a major procedure too early. Signing the consent brought back so many memories and feelings from over a year ago. The doctor had to disclose all the possible risks of the procedure and the one that always hits us and gives us an uneasy feeling is "death." We were however glad to hear that Dr. Perry was going to do our cath as he is world renown for his surgical skills in the cathlab (and also for his people skills... but in the opposite direction, but hey, no-joke, we like the guy a lot, (I actually can communicate with him well by grunting and nodding)). That night we stopped feeding him at 9pm and gave him only clear fluids until 12am after which he had absolutely nothing to eat or drink.
Tuesday: Tuesday morning came and we took O for a long Buggy ride and took him to the preschool to play with toys, trains, blocks, oil pastels, crayons, and do one of his favorite things to do, watch other kids play. It was turning out to be a great day. O went to the cath lab on schedule at 12pm and played with bubbles smiling and laughing (his usual self) in the surgery waiting area. two hours later our Dr came to us saying that they saw some more fluid around his lung so they need a consent to put in a chest tube. (for those of you who don't know, its a tube going INTO his body from his abdomen about 1/8-1/4 inch thick. It is sutured on to his skin and goes all the way to where it needs to be, in O's case his lungs, and attaches to a vacuum that slowly drains fluid, uncomfortable and painful). That's about when my day went downhill (it was already starting out a bad day for Tawny, she had terrible stomach pains). We got the call that O was done with his cath and Dr Perry and Dr Axelrod would meet us to discuss the results.
CATH RESULTS: The cath showed (besides the fluid leftover in the lungs) that the pressure in the RA was 18 when a normal heart would be 6 (12 is about what it was on his last cath). This excessive pressure was built up over time and was probably due to several factors. The ASD (atrial septal defect) that O was born used to relieve some of the pressure in his RA, but after time had probably become smaller and smaller as O's heart grew. One of the big problems is the two valves preceding the RA have leaking back-flow. The tricuspid valve (valve between the RA and RV is leaking into the RA, and the area where the pulmonary valve supposed to be is an open patch. The good thing about the excessive pressure in the RA is that it forces flow through the RV (it really has no where else to go), which makes the RV want to grow... and it has, to the point where it's usable, something that was not expected. So it's a fine line between good and bad, and the pressure has crossed over to the bad.
THE SURGERY: During the cath, Dr. Reddy was called over to look at the pictures and results of the cath. After discussing the info with the rest of the team they decided O would need surgery in the coming days... and since O.R. space is booked solid for a while and there is an opening the next day, they said, "Orion is going into surgery tomorrow, if we can get the O.R." So the plan today (Wednesday) is for Dr. Reddy put O on the heart/lung bypass machine, open up his heart and repair the ASD, the tricuspid valve, and the pulmonary valve, so that there is no longer backflow going into the RA. Then they will test out O's new anatomy, but checking the flow and pressures. If the pressures remain stable and seem to be in a tolerable range, they will be done and Orion will now have an anatomy very close to that of a healthy heart or a "2-ventricle repair." If the pressure in the RA and don't seem to subside and stay dangerously high, they will go ahead and proceed to do the "Glenn Procedure." This is where the SVC (superior vena cava), the main vessel that returns all the un-oxygenated blood from the upper half of the body to the heart, will be rerouted directly to the lungs. Doing this will reduce the load on the RA by half, because the right-side of the heart will only be pumping the blood from the lower part of the body into the lungs. This is called the 1 1/2 ventricle repair, it's basically the SVC bypassing the rightside of the heart. This procedure also makes the LV (left ventricle) work harder, and since O doesn't have a totally normal LV, the 2 ventricle repair would be ideal. Another thing about the Glenn is it is irreversible. With the 2V repair, if O's body changes in the future there is the Glenn is still an option.
We are hoping that the RA will respond to Dr Reddy's repairs and the pressure will go down without having to do the Glenn. Please keep O in your thoughts today, as he is scheduled tentatively for the O.R. at 12 pm today. This is the day we have been waiting for for a long time.