language development

I walked in the door yesterday and Iris ran toward me screaming gleefully, "Fuck and damnit!"

Sorry, grandparents.

After seeing my reaction (laughter and surprise), she said it again. And then she said, "Daddy said it." Evan had been working at the dining room table earlier that day and something hadn't been going so well. He didn't know that Iris was in the kitchen at the time...

Oops.

We're trying to completely ignore her now when she says it. Occasionally, Evan will say, "fork?" and she'll shake her head no. I think we just have to hope that she'll stop saying it after we don't respond for a while.

Iris has been parroting everything we say for a while, which is a lot of fun. It obviously has its drawbacks. One of the words she began parroting quite early on was "echo" as in echocardiogram.

We had a checkup with the cardiology team a week ago Friday. In the past she had been doing well with getting the echo. They take us into a dark room and Iris lies down on a bed and can watch cartoons while they put goop on her chest and use their ultrasound wand to get the images they need.

This time she wasn't interested in lying down and being still. I had to lie down with her in a very awkward position to have any chance of success. The first echo guy was mostly successful in getting all the images...except for the one that they really, really needed, which was the image of the dilation of the ascending aorta. So we had to try again with another echo gal. She also had a hard time getting it. Finally, a third lady came in and was able to get it very quickly. Phew.

The news is -- everything looks exactly the same. Her dilation is 21 mm, if I remember correctly. So we will go back in four months for another checkup. We were supposed to see our pulmonary specialist but she had to cancel, so the next specialist visit will be with ophtalmology in March. The pulmonary specialist will catch up with us then.

Although Iris didn't do so well with the echo, she did much better with this month's Synagis shot. We have been reading Elmo goes to the doctor a lot.

So on the day we were supposed to get the shot, I mentioned to her that we were going to go to the doctor for her shot. I haven't normally done this in the past, and I'm not sure it was the greatest idea. She sort of moaned/wailed, "No...." And then I reminded her that it would only hurt for a minute and that Elmo had to get a shot, too. I also told her we could get a sticker.

She basically objected to every part of the pre-shot stuff at the doctor's office. But she didn't cry, she just sort of whined, "No..." She finally started crying when the nurse brought the shots in.

The nurse let me hold Iris in my lap. It was awful, as always, but the thing that really saved the day was that I brought this little computer that my mom bought for Iris a long time ago. We barely ever let Iris see it much less play with it, so she was hooked right away.

I kept telling her what a good job she did and that Elmo had to get a shot, too. I taught her to say, "I got a shot." Except it comes out, "I got shot!"  

So...we're in a very awkward stage of language development. 

cardiology check-up

Iris had a cardiology check up on Friday. I took the full day off work and Evan took half a day. I had been preparing Iris for the check-up with a play doctor's kit that we'd been using with Iris's bears at home. So I knew she would do great with the stethoscope. I wasn't sure about how she would do during the echocardiogram, which she hated last time. I had tried to explain what would happen several times to her. 

When we got there, she was extremely calm and let everyone do what they needed to do. She was so calm during the echo that Evan checked to see whether her oxygen was flowing. She was mesmerized by a cartoon playing in the background for most of the time, but occasionally she would say, "all done," or "up." Evan and I would then try to entertain her with something else, like the itsy bitsy spider. 

We did get some bad news. The echo showed that her aortic root is bulging and the valve is leaking a little bit. This is a new development. They can't determine the size of the bulge based on the echo so we have to do a CT scan in the next couple weeks. Because of this finding, the doctors are pretty confident that Iris has a connective tissue disorder, though we know that she doesn't have Marfan syndrome, which she was already tested for. So that means it's probably just a "not otherwise specified" expression. 

The bulge isn't an emergency situation, and next steps will be determined after the CT scan. They recommended that we start her on losartan, a blood pressure medication. We said we wanted to wait a little while to get some more information about it, and I expect that we'll start her on it in the next few weeks. Dr. Landeck thought that it was likely that there would need to be a surgical intervention at some point, just like Dr. Mitchell thought right after the surgery. 

The valve leakage is minor but should it get more severe and require a valve replacement, then it's likely that she would need a mechanical valve, which would require her to be on blood thinners for life. 

In addition to the CT scan they plan to do further genetic testing. They now are sure that insurance will cover further genetic testing, which they weren't sure of previously.

Evan is being appropriately circumspect, while I am crestfallen. None of this is coming as a surprise, really, and it's far too early to know what the implications are. There was a time when I thought that it might be smooth sailing, but that was a misguided hope. Mostly, I am trying to think about the next step, which is trying to get Iris ready for the CT scan, which will require her to have an IV so they can do an angiogram.

These are some photos of her after the check-up.

steroid burst

We met with cardiology and pulmonology today in a 5-hour visit to the hospital clinic. They did an echo, an EKG, and an x-ray. The conclusions: she has very mild septal flattening while off oxygen so she will stay on the oxygen and continue the sildenafil. We can take her of the Lasix. Her oxygen saturation levels are low so she is now up to 1/2 liter instead of a 1/4 liter. The x-ray showed that she had hyper-inflated lungs so she is starting on steroids to address the inflammation. She will have 3-5 days of a ‘steroid burst’ and then we will continue to give her inhaled steroids for 3-6 months. Evan and I are both unhappy about this, particularly Evan. I am hopeful that we can keep this shorter rather than longer but it is important to protect her lungs as much as possible.

She gained weight again - averaging 25 grams/day for the 18 days between clinic visits. She is now 4.95 kg (10.9 lbs). We think of her as a normal-sized baby but she is really still quite skinny.