Miles had his annual cardiology appointment today. I didn't have too many concerns. This year hasn't been terribly eventful, well not too much out of the usual anyway. He still has asthma exacerbation's all the time, and he still typically gets pneumonia one or more times a month, but that was all going on prior to his last visit. And in the past year, he's only been hospitalized once (I think).

The paperwork today asked if there were any new symptoms. I did have two. Over the past couple of months, Miles has been vomiting on a pretty regular basis, maybe 3 or 4 times a week, for no particular reason. He has had reflux issues in the past, but that was always preceded by coughing. Now, it's just out of the blue. Number two, there have been a few times, also in the past couple of months, where Miles has come to me complaining that he needed a treatment. There weren't any signs of respiratory distress, so I'd tell him no he didn't. You see, because Miles has been taking so much medicine for so long, he's actually likes it, so I thought he just "wanted" a treatment. After some insistence on his part, though, I'd take his oxygen levels and find them in the low to mid '80s. Very strange since there wasn't wheezing or retracting, so I was curious about that.

As I suspected, the appointment did go very well. Dr. Puchalski said that Miles's mitral valve sounded great. That is kind of the biggest concern of his problems. Last year, there was some worry that the reason for all of the respiratory problems and clouding in Miles's lungs was because of mitral valve stenosis. The mitral valve can become hardened and move as it should, and it can allow blood to pool in the chamber or leak into the lung. Even though, Dr. Puchalski couldn't hear anything that pointed in that direction, last year, he ordered an echo to check it out because a separate condition just seemed too unlikely. As he put it, lightening did strike twice, and the respiratory issues were not heart related. In fact, he thought that the mitral valve actually looked healthier than it had when Miles was first diagnosed, which is almost impossible, improvement, I mean. Anyway, mitral valve still sounded great.

He did hear some leaking from the aortic valve. The typical heart has a tricuspid aortic valve (with three leaflets). In many Shone's kids, the aortic valve is only bicuspid. This is the case with Miles. I believe it's a relatively common issue, and usually doesn't cause problems, but the valve can become narrow and restrict blood flow, or can become leaky, as is the case with Miles. It still isn't anything to cause too much worry at this point. It just has to monitored (with only a yearly check-up unless something worsens), but I was told that it would have to be monitored for the rest of his life. I'm not sure why, but I just thought it was a little funny that the doctor added that last part.

Dr. Puchalski did address my concerns, but really didn't have any super helpful answers. He seemed a little perplexed by both issues. He didn't think that the vomiting would have any relation to his heart problems, but he did think that it would be a good idea to visit a GI specialist since it is such a frequent problem. I didn't really think that this was a result of Shone's, but since I'm certainly not an expert, I'm going to bring up any weird thing that happens. I think that this could just be the result of genetics. Ben has a real issue with throwing up too, so the males in this family may just have very weak stomachs. Dr. Puchalski did suggest something that I found a little interesting though. He said that there is a disease (not a serious disease) where people just throw up a lot. He told me the name, and it was something super hilarious that sounds made up, but I can't remember it. That's part of the reason that he thought that it may be advantageous to see a GI specialist.

As far as the mysterious low oxygen levels go, Dr. Puchalski didn't have a real explanation for that either. He said that it was very strange for that to happen without obvious signs, but he also said that Miles compensates very well. We know that by the fact that he wasn't diagnosed with Shone's until he was one. It's usually diagnosed in utero. By the time he was diagnosed, his coarc was so bad, that it required surgery within weeks. I saw the portion of his aorta that was removed. There was even a pinhole left. The surgeon seemed a little shocked but also excited to show us what Miles had overcome. Without any major blood canal to his extremities, Miles still did not have any obvious symptoms. Most kids would have had swollen hands and feet and blue fingers and toes. Not Miles though. His lesser passageways must have taken control and compensated for the practically useless aorta. Pretty amazing how the body can do that. He did give some recommendations for additional things to watch for. He said that even if I was not seeing bluing around Miles's lips, to check the inside of his mouth. When oxygen levels are particularly low, the interior part of the lips will almost always blue. He also told me to be aware of the color of his lower nail beds. They will usually get a bluish tint as well.

Anyhow, I was assured that this, too, was strictly a respiratory issue, not a cardiac one. He told me that the only reason something like that would happen from a cardiac stance would be if blue blood was mixing with red blood, and right now, that is not happening. It's nice--well, kind of--to know definitively, that the respiratory problems are a separate issue. I say kind of because it would also be really nice to have an explanation because we still know that it's not all asthma. I suppose we'll just continue to see a million different doctors for a million different issues. Right now, Miles has his regular pediatrician, his cardiologist, his pulmonologist, his allergist, he sees a respiratory therapist pretty regularly (although it's not the same person each time), and now, I guess we get to add a GI specialist. You know, many Shone's patients also have neurology and ADHD problems, so maybe down the road, we'll get to add a few more.