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.

Monday, March 21, 2016

Regressing

Micah is one of the most unpredictable people I know. We were discharged from Children's on Tuesday, March 15. I definitely thought we were leaving too early. Micah still had a cough and he could barely maintain a decent SAT. Dr. Jonas seemed confident Micah would heal better at home and after the weeks of hospital life, we agreed with him. That Friday we took him to his follow up appointment with his pediatrician. Like I mentioned in a Facebook post, she was not okay with how he was presenting (low sats, raspy breathing, grey hands and feet) so she sent him to the ER at Children's. When we got there his sats were 44! :( He was admitted and as soon as they put him on oxygen he perked right up and was his old self. We had such a good weekend and at rounds this morning we discussed possibly going home tomorrow with oxygen.

One hour after rounds, his nurse came in to give him his morning meds. Micah began to cry. It's typical for Micah to cry at the sight of hospital staff so we thought nothing of it. She stepped outside the door, just out of view, but Micah continued to cry and his SATs started to steadily decline. He started flailing his body around like a crazy person, I thought he was definitely going to hurt himself. It's like he was fighting just to take breaths. And even though he was being so wild, his heart rate was dipping into the 70s - his normal heart rate is 130s. The room filled with people and next thing I know we're headed back to the CICU. I can't remember all the details but during Micah's episode, his IV went bad so they had to try to replace it. It's so tough to get an IV on him and the doctors needed to administer meds right away so they decided to give him an intramuscular (IM) of ketamine and rocuronium. Then they gave him an intraosseous (IO) of a different drug- an IO is like an IV straight to his bone. Then they intubated him and put in two central lines - an arterial line and a PICC line.

He's totally sedated and paralyzed and I have no idea what's going on. How can we go from talking about discharge to being sent back to square one? My head hurts, my eyes sting and my heart is breaking. Just last night Micah and I were playing and laughing. And now he's medicated and unresponsive. Thinking about his laugh from yesterday just unravels me. What is happening?

I've heard a few theories since this morning - they think he might have the flu, but they also noticed his right ventricle wasn't squeezing as well so he could have a clot in his conduit to his left pulmonary artery. I just don't know... I pray that it's just a virus and not something seriously wrong with his heart or lungs.

This is a video from last night.

video