Details (TMI)

Sometimes I forget I have a medical background when I post. I apologize, but I was not in the mood to go into details earlier. And after many calls of concern and love I would like to clarify that heart failure does NOT mean Ethan is at the end of his journey. It is just another (major) bump in the road. Ethan seems to be pretty stable now. His kidney function is not great and he has another infection. He did not tolerate the heavy hitter diuretics this time. So we need to give him a week before he goes to cardiac catheterization. Let his kidneys recover and get the infection over with. In the cath they inject dye that can harm the kidneys if they are already in a fragile state. So we won't rush to cath unless we need to. The cath will give us the direct pressures in his heart and lungs and tell us what is going on in there.

The current theory/worry is his left ventricle. As you know he does not have a left ventricular chamber, however, unlike most HLHS baby his left side is a big huge mass of muscle. They describe it like a tumor sitting there. Ethan's right ventricle is what does all the work. It is functioning well. The only problem is it appears to not be able to fill up with enough blood before it contracts to push the blood out. They think the blob of a left ventricle is getting in the way and not allowing it to relax and fill as much as it should.

I could go on for an hour explaining things. But most of it is still theoretical. One thing is for sure. He requires ABSURD amounts of diuretics to keep his heart and lungs functioning right. That means he has to be vascularly dry (or dehydrated)or his heart can't tolerate the new passive glenn flow. When he gets too much blood volume it appears his heart's blood flow backs up into his lungs. Causing his lungs to be wet! Since his thoracic surgery he has still battled wet lungs inside. If this theory proves to be true, then his right ventricle is under stress causing his BNP levels to rise. (By the way his BNP is 13,000 today). High BNP in your body tells your kidneys the heart is in trouble. The kidneys slow/shut down as a defense mechanism to protect the heart. Thus a vicious cylce of trying to keep Ethan vascularly dry so his heart can function properly. Because when his kidneys sense this hormone, they slow down and retain fluid. (I hope you can all understand this.)

Heart failure can be fixed with medications, procedures, and/or surgery much of the time. Sometimes it cannot, and a heart transplant is needed. We won't know more until after the cardiac cath. Once his infection looks better they are also going to trache him. It is much easier to put the ventilator on his trache every time his lungs get wet or sick than it is to intubate him. When he is intubated he is uncomfortable and has to be sedated and restrained, doesn't move around and only becomes more succeptable to infection. With the trach he is alert, moving, and can be playing when he has to be on the ventilator. So it won't be as hard on his body when he has setbacks. As mentioned weeks ago his lungs are also diseased at this point. Meaning they are consolidated from prolonged irritation. So it is going to take a long time of keeping him balanced to let his lungs heal.

The hardest part today has been the talks with our surgeon and one of our cardiologist. There are some medications we can try to trick his body from thinking it is in heart failure. Thus his kidneys will stop being so reactive and complicating his fluid balance. Those may totally help, or may only buy time. But if the true problem is the left ventricle mass, then they told us to prepare to decide if we are willing to try for a heart transplant. But right now we have to get his lungs healthier so he could even be considered for one (if it comes to that). The next couple of weeks will tell. One thing is for sure. The acute phase of this journey just got a lot longer. We appreciate all of your support and sacrifices on behalf of our family.

Much love-
Greg and Heidi
PS: Sorry if this is too much, but I know loving and curious minds want to know!