backpack walks

We officially started a new family ritual this past weekend: Saturday morning hikes. It has been an unofficial ritual before. We are anticipating a lot of fun mornings with Evan singing at the top of his lungs to entertain Iris. She loves "backpack walks" and has been putting her stuffed animals in the backpack to tell us that they're ready to go out for a hike.

 

Saturday was also the day that Iris had a very weird ailment for most of the afternoon. Her belly started hurting her intensely and cramping up. She wouldn't eat but would drink hemp milk. She just wanted to sit in my lap and wouldn't let anyone else touch her. She kept saying, "belly hurts." Evan and I decided this was due to the fresh mozzarella that was on the pizza she had eaten the afternoon before on our way to meet with the gastroenterologists. She had had a similar episode around the time I first tried giving her cow's milk (which happened to be the weekend of my dissertation proposal defense, thus resulting in the Daddy-Daughter Staycation). 


She did recover by Sunday, thankfully. I was pretty exhausted the whole weekend so Evan took Iris out again for a backpack walk on Sunday morning and I got some work done on my dissertation.

As for our most recent visits with the specialists, Iris had an ophthalmology appointment and a gastroenterology appointment this past week. Ophthalmology went fine, as it always does. Her eyes are totally normal aside from the fixed dilated pupils, which was once thought to be aniridia and is now called congenital mydriasis. I think that the ophthalmology fellow who first diagnosed her was biased by her name "Iris" and so was bound to think "aniridia" first. Anyway, whatever. We're very thankful that her eyes are doing well. After the next visit in six months the doctor said we can move to yearly visits.

The gastroenterology appointment went fine, aside from the exceedingly long wait we had due to a backup of patients. I don't fault the doctors for this. I do fault the American healthcare system and hospital management for trying to cram in too many patients. Evan and I were glad to get some additional information on how Iris's gut health looks from people who study these organs.

Basically, she doesn't move things along fast enough by herself so we need to start giving her MiraLax daily to help avoid constipation. They did tell us that we can't rule out malrotation because ultrasounds are not the best method of observing the intestines, and the ultrasound that we did get did not go down far enough to see the duodenum. So...that means that at some point we will probably do an x-ray with her swallowing some kind of barium or other radioactive liquid. We're not sure when we'll schedule this. I also need to figure out when we are going to do her next MRI. The neurologist wanted to see her back in 3-6 months, and we've reached the three month mark. And we haven't seen our pulmonary specialist since before Iris's ACTA2.r179c diagnosis, which was in December. We were supposed to see her in January and then again in March. I guess I know what my homework is for tomorrow...

I hate to end on that note, though. So here's a picture of Iris making a joke. She put two sippy cups in the collar of her shirt and thought it was hilarious. She has a great sense of humor and is great at creating her own fun.

overly optimistic

Iris had an ophthalmology appointment today. I'd been eager for this appointment to get a better idea of the state of Iris's irises. And if she needed glasses I wanted to get them on her as soon as possible. 

Upon meeting Iris for the first time, most people do not notice that she has any particular eye issue. It's only when Evan and I point out that her pupils are huge does anyone seem to take note. By all accounts, she tracks very well and has no problems with peripheral vision or grabbing on to what she has set her sights on. Knowing this, I was not particularly anxious about the appointment. Even though she is at greater risk for glaucoma and cataracts, it seems too early for those to intrude. 

After waiting for slightly too long a time, the ophthalmologist breezed in to the examination room. A fellow was with him, so he began the encounter by quizzing her on WAGR syndrome while simultaneously typing notes on the computer: "What do you know about WAGR syndrome?" She thought for a moment, "Let's see... Wilm's Tumor, aniridia, G is for...genitourniary, and the R is...renal?" I nodded as she finished the G and the R. "That's right," he said and continued talking -- though I have no idea what he was talking about because following him made my head spin. I did stop him fairly quickly and mentioned, "Yes, that's WAGR syndrome, but Iris doesn't have it. Genetics tested her and she doesn't have the gene deletion." He did some tests on her eyes to see how well she tracks, shined some lights in to check out the rest of the eyes and also tested the eye pressure to see where she stood in regards to glaucoma. 

When he was ready to begin telling us [Evan was on speakerphone] about his assessment and conclusion he started with, "I don't want to be overly optimistic..." and he went on to say that Iris looks great and has no need for glasses at this point. She is slightly far-sighted but he doesn't think she'll need glasses for quite a long time. He also said that she might start having trouble with cataracts years earlier than most adults, but maybe not till her 40s at the earliest. "She is probably one of the top two cases of aniridia I have seen, her eyes look great." 

Evan asked about how much care we need to take with her eyes and mentioned the sunglasses we have for her. The ophthalmologist said, "Don't get an ulcer over trying to keep the sun out of her eyes when you're in the car. She'll close her eyes if it gets too bright." He did say a floppy hat is a good idea. We were very happy to hear this good news and though we certainly may not have the same good news in the future, it seems like an auspicious beginning.

Speaking of that floppy hat and those sunglasses, we took a trip to the mountains together over the weekend:

 Iris is down to 1/16L of oxygen, which is almost nothing. I am hopeful that we'll have her off oxygen by the end of the year. We are going to Los Alamos for Christmas, though, and since it's at 7,300 ft, she might need oxygen there. 

We also went to visit Jill & Dara today. These girls are really starting to have fun with each other and we're looking forward to seeing them grow up together.

Dara's older sister, Liliana, had her 3rd birthday party this past weekend and we went to the place they do music class. I think that I will start doing a music class with Iris in January and am very much looking forward to it. 

And finally, we did some family photos a few weeks ago. The photographer is a nurse in the Cardiac ICU at Children's and took care of Iris the first couple days after her surgery. (Here's her website. Denver people -- her prices are really great since she's not a professional). She got some really great shots even though Iris was really sleepy and needed a nap. Here are a couple of good ones.

photo credit: Visible Life Photography

photo credit: Visible Life Photography