Friday, March 25, 2016

A new diagnosis

Micah had a cardiac catheterization scheduled today. With his desat and low heart rate episode on Monday and no clear answers for what the problems could be, the doctors felt that a cardiac cath would give us the answers. Shortly after the surgery, I asked if we might need to get a cath in the short term, but the doctors said that they wouldn't want to - only in more of an emergency situation. Knowing that information helped put the situation into perspective.

This is Micah's 6th cath, so Laura and I are pretty familiar with what to expect. Our doctor said that the usual 2-3 percent risk is more like 5-6%, being that he just had his surgery. The plan was to take some images and see if there were narrowings to balloon or locations to place stints along the right or left pulmonary arteries.

I've had a bad feeling about this cath all week. Definitely not in much of a hopeful mood leading up to the cath. A nurse or two commented on my mood, things like "is he always this quiet?" Outside of obvious extremes, in our heads the likely worse case would be that they find nothing and we're kind of forced to wait and hope things get better. The more ideal case would be that Dr. Kanter could make some fixes in the right or left pulmonary artery and that would put some pressure off his heart.

Laura and I waited and got our first update about an hour in. The nurse said that Micah was doing fine. They were taking images, but not doing any corrections. That was a little odd, but the nurse isn't able to fill us in with any real information, so we waited for a little bit longer. We got word that everything was done and Dr. Kanter wanted to brief us with Dr. Donofrio (Micah's cardiologist) and Heather, Micah's social worker. That was obviously a bad sign.

Dr. Donofrio broke the news that Micah's left pulmonary artery isn't passing any blood from the newly replaced conduit to his left lung. Effectively, his left pulmonary artery is disconnected (discontinuous) from his heart. The diagnosis for his condition is pulmonary hypertension. Incurable. The situation that he's in is that he has to rely on his heart pushing all the blood through his relatively narrow right pulmonary artery and only to his right lung. Doctor Donofrio expressed that working with only one lung is bad enough, but the current right lung and heart situation isn't optimal. She said this outcome was her feared worst situation.

We really felt annoyed that Laura and I didn't know about this risk earlier. The left pulmonary artery was really narrow for some time. They've had more success on the right side with the ballooning during other caths. They confirmed that there was blood flow right after the latest operation, but for some reason when the most recent ultrasounds didn't show information about his left pulmonary artery - they didn't bring up this concern with us then.  It's really difficult as parents. We're trying to do as much as we can to help Micah. Half the time, just keeping up is enough of a struggle. But again, we realize that people keep us at a distance, until they can't anymore. The whole situation felt like a bad dream.

As far as the immediate term goes, Micah needs to be weaned off the boys breathing tube so that he's breathing - at least mostly - on his own. As far as the past week goes, he's been able to produce 100% oxygen saturation, which shows that once the blood gets to his lungs.. (lung rather..) it does the job. They're going to start him on some new drugs that help ease the pulmonary hypertension. They want to plan with the intentions of getting him sent home soon, but they'll be on high alert for something like what happened on Monday. It seems like something like that event would be the main concern for someone with pulmonary hypertension - blood pressure and heart rate drop, the right heart can't keep up, pressure is too high, oxygen saturation plummets... all in a very short amount of time. I'm just glad that Micah was here and able to be sent quickly off to the ICU when that happened. Obviously...(or at least hopefully) Micah would only be discharged after the doctors are confident that something like that wouldn't happen again.

It's hard to tell how the longer term will play out, but it was definitely the saddest news doctors have had to deliver to us since telling us that Micah had a heart condition. A surgery to somehow reconnect from the conduit to the left pulmonary artery, or even further on directly to the lungs, is - at least on first discussion - something that has tons of risks and low guarantee for success. The obvious question Laura and I were wondering was what kind of life expectancy Micah will have with pulmonary hypertension. We started asking about it and which lead to comments about how lots of kids are here at children's with a really great doctor (someone new to us), with a range of ages, and that we could probably talk with a 9 year old who is around pretty frequently. I asked if it's likely for kids to make it into their teens and got the vague response that sounded like it's not likely.

At this point, we really just want to get Micah home and back to his usual playing with Dylan, smiling, eating M&Ms and tossing toys down the stairs.