In some of the mice studies for the genetic diagnosis Kiran has, it has been noted that two of the areas most affected are heart and brain. None of the other children that have been studied with this diagnosis have reported heart defects, so Kiran is unique in that way. But the brain abnormalities reported in others, along with the diagnosis of intellectual disability, are similar.

We just happened to see both Kiran’s cardiologist and neurologist on Wednesday this week. Gotta love those trips when you leave at 5 am. Kiran did not know what to make of me waking him up so early!

Neurology was our last appointment of the day, and it went exactly as I had hoped. We have only seen neurology a few times in Kiran’s life, because we have no reason to be seen regularly. We were following up with her for his unilateral flushing issue. And she agreed with the hematology/oncology doctor, that we have been chasing a cause for this long enough. We have essentially ruled out any serious cause, and Kiran has seen every specialist regarding this issue to do so. So we just accept that it is idiopathic, which means “of unknown cause”. It is just another thing that makes Kiran a unique, special little boy. Unless other symptoms pop up or it starts bothering him or increases in frequency greatly, we don’t need to follow up. One more specialist off the scheduled docket!

Our first appointment of the day was Kiran’s echo, and we got a special treat! Our favorite echo tech, who is retired but comes in to help on occasion, was there and available, so we got to have him for Kiran’s echo! But our new favorite requested echo tech is leaving next month, so we will have to find a new favorite once again. Still, it was so nice to catch up and have the calm, expert experience first thing in the morning.

Cardiology did not go as I had hoped. I think after years of hearing “Everything is stable, see you next year!”, I was lulled into a comfortable space, even though my brain knew that Kiran would eventually require interventions. It is nothing we weren’t expecting, and in fact, is exactly what we have been monitoring. The pressure gradient in the conduit that was placed between his right ventricle and pulmonary artery has increased, somewhat substantially. However, there is no indication the right ventricle is having to work harder yet, which would be an increase in size – a growth of that muscle, so to speak. Because of that, it is not something we need to urgently address. Dr. R said Kiran will probably need a cath lab intervention, to balloon the stent, in the next 12-18 months. The ballooning helps to increase the size of the space so blood can flow more easily, lowering the pressure. It essentially prolongs the life of the valve, because we don’t want to have to put in a bigger one (which is open heart surgery) until we absolutely have to. The bigger/older he is when we replace it, the fewer we will have to replace over the course of his life.

So, we do need to keep a closer eye on things, so we will see cardiology again in six months, rather than waiting an entire year. At that point, if things are stable, we will go another six months. If the pressure gradient has increased a lot and/or we see right ventricle wall growth, then it will be time for the intervention.

So the positive here is it is exactly what we knew was going to happen, and we have a plan in place to address it. This is nothing surprising and is just part of having these procedures with conduits placed. He’s in great hands with his cardiologist, who I trust completely. I was so thankful for his calm guidance and explanations. He is always good at taking the time.

It’s a long road ahead, but we journey together.