hosptial vacation

I went home the other day to get some more supplies for our hospital stay. We just completed a full week at the hospital with Iris. It was strange to walk into the house and see everything as we had left it last Saturday evening. We were in the middle of some big organizing projects when we decided we needed to take Iris to the emergency room. Those projects are still in midstream a full week later.

We took our time getting ready for the ER visit and even packed some extra supplies under the assumption that we would be staying in the ER over night (but not a whole week). Iris had had a bad cold and cough starting Monday but it was far worse on Saturday, when we couldn't keep her oxygen levels up and she was needing more than 1LPM of oxygen to get close. She also had a fever of 103 degrees (Fahrenheit), which we didn't catch at home.
 
Turns out she had two respiratory viral infections that have completely sidelined us. Rhinovirus (the common cold) and human metapneumovirus (hMPV). The more nasty of them is the hMPV, which can cause increased oxygen needs even in perfectly healthy children. She is taking a long time to recover, which is expected with ACTA2 r179 kids. This may be because she has a harder time getting secretions up and out of her lungs because her airways are floppier than typical kids.

Iris has basically been in good spirits the entire time we've been here, though that has fluctuated with her fever. Tylenol helped immensely. This video is from our second night in the ICU. Iris is giving us her version of, "Tom, Tom, The Piper's Son."

Evan and I have been with Iris the whole week. Evan has worked from her hospital room and I have taken sick leave and sloughed off a few time sensitive projects to a co-worker. I did go to work briefly on Friday, and Alyssia and Ky came to help out.

We requested a big bed for her when we got to the ICU, mostly because she wouldn't let me put her in the crib they had for her. So we had to sign a waiver saying we wouldn't leave her by herself in the bed since she was too young to be trusted in a big bed by herself. The advantage of this is that we have a an adult-size twin bed and can "sleep" right next to her. We have also been able to get a sleep room the past three nights, and we've been taking sleeping shifts.

Father's Day in the ICU

The plan of care has involved breathing treatments and suctioning (every four hours) to get the secretions out of her lungs. She hates these, but endures them when she's on Mommy's or Daddy's lap. As soon as two nurses walk in she starts saying, "No boogies!" She has definitely gotten used to the treatments and just yesterday started reenacting them on Buddy Bear. She would say over and over, "Okay, your turn, Buddy Bear.... [pause]....my turn, Buddy Bear...."

"Your turn, Buddy Bear."

"My turn, Buddy Bear."

Her treatments have gotten easier over time, also, because they're actually succeeding in getting everything out. So we don't have to do the deep suction anymore -- the most painful of the four treatments. Overall, the pats on the chest with the CPT cup are fine, the 5-minute breathing mask is also not awful. The nasal aspirator is stronger than our NoseFrida, and involves more saline.

It was a big step forward when we took out her IV, which had been on her left arm, making her left thumb unavailable for sucking. She couldn't suck her thumb for the first few days and so I would hold her hand as she fell asleep. She would ask for her pacifier, which she would just hold in the other hand. Finally, when we took the IV out, she didn't use her left hand, which was worrying. It took probably a full 24 hours before she found her thumb again. Now she will occasionally say, "We took the IV out!"

The "no-no" kept her from moving her IV arm.

It's been a roller coaster ride this week. Iris went from being on "heated high flow" oxygen in the ICU down to "wall oxygen," then back on "heated high flow." Heated high flow separates the amount of air getting pushed into her lungs (the flow) and the level of oxygen mix. So at her highest she was on 8LPM of flow and 80% oxygen. At home (or what they call "wall oxygen" at the hospital), she is on 100% oxygen and the flow can vary up to 4LPM. She'll never get up to 4LPM at home because we are supposed to bring her in to be seen if we have to go over 1LPM.

When she is on the heated high flow she has to stay in bed (or very close to it) because the tubing is so short. On the longer tube she gets to run around and look out the window, which is exactly what she did the first night out of the ICU. There were heavy storms during the week and that night she got to see her first rainbow. [I don't have a photo of the rainbow, but I'll post a photo of her at the window soon.]

Dancing next to bed (on heated high flow)

Shortly after that we had to go back on heated high flow and pump her with Tylenol to fight the fever. The fever dissipated mid-week, around the time we gave her a dose of Decadron, but she stayed on heated high flow.

Yesterday (Saturday), though, was a big step forward. She got off the heated high flow again and over night made it down to 1LPM on the wall oxygen! That is close to what she would have to be at to let us go home. I'm looking forward to rounds this morning to hear their thoughts.

She has started a twice daily course of Flovent, which we will keep her on for the foreseeable future. This seems to be not at all uncommon for other ACTA2 r179 kids so we feel fine about taking this course of action, especially since she does occasionally have increased work of breathing at home, even when perfectly healthy. This is something we had tried hard to avoid, but now seems like an obvious next step. And it's not necessarily something that will be a lifetime medication (like the aspirin and losartan).

A big "thank you" to everyone who has visited us, sent us books, toys, and food. I am pretty sure I've gained at least five pounds given all the food and lack of exercise. I did finally get a walk yesterday afternoon after Evan went home briefly for more supplies (and brought me my walking shoes). The visits and toys/books have really helped pass the time. I'm still not sure how we would have survived without the two TV shows -- Bubble Guppies and Curious George. Let's all hope we get out of here in a day or two, max.

neurology, round 2

Our second meeting with the neurologist was less overwhelming that the first, mostly because we knew more or less what to expect to hear. That's not entirely accurate - I didn't know whether to expect to hear that the white matter lesions had gotten worse. I did my best to prepare to hear that yes, they had gotten worse, though.

The news is basically good: nothing has changed. Iris still has white matter lesions, but the damage hasn't progressed. And the vessels all appear about the same size -- they haven't narrowed. Patients with the ACTA2.r179 mutation have dilated internal carotid arteries and narrower middle cerebral arteries. Her middle cerebral arteries do not look paricularly narrow at this point.

One piece of information I had forgotten, though has been mentioned in the past by various specialists, is that the ACTA2.r179 mutation results in the overproduction of smooth muscle cells. So, over time, the space for blood to flow narrows as the vessel walls become thicker. And at that point the neurologist would tell us that a major stroke is imminent and that it's time for surgical intervention.

Barring any TIA's or other neurological events, I'm hoping to push the next MRI to a year out. It seems that the other ACTA2.r179 folks have MRIs about every 12-24 months...not more frequently than that unless there are major indicators requiring it.

So, overall, good. (Amazing how your bar of what qualifies as "good" changes. We adapt.)

"I love Daddy."

I went into Iris's room the other morning when she announced that she was awake with a cry/wail. I got right up next to her and said, "Did you have a good sleep?" She stretched and looked at me with a big smile and said, "I love Daddy." 

The feeling is pretty mutual. Evan is happy to tell people that playing with Iris is the highligh of his day and the other evening Iris was showing Evan her favorite book. Evan said, "Is that your favorite book? Well, do you know what my favorite thing is, Iris? My favorite thing is you!" 

Iris is one of the most affectionate little people I've ever met - she loves to hug the kids and adults in her daycare classroom and at the church nursery. And when we leave for work she does multiple hugs and kisses with both of us. We certainly encourage it, while also trying to get her to understand that sometimes people don't want hugs or kisses. 

Tomorrow morning (check-in at 6:30am!) she has her second brain MRI, the first having been in January of this year. I bought a couple gas masks that smell like buble gum for us to practice with. I expect the putting her to sleep part of it to be fully awful, as it was last time. 

For every procedure that requires anesthesia I wonder if she'll have a stroke while she's under. It is clear that the likelihood of her experiencing a stroke increases as she gets older and there's almost not a day that goes by that the thought doesn't cross my mind and I wish, "please don't let it be today." I think about it more in the summer when it's hot because being dehydrated can increase the risk of stroke, too. 

We will get the results of the MRI on Tuesday of next week when we meet with the neurologist again. 

mother's day road trip

We decided to take a road trip over Mother's Day weekend to Farmington, NM, to meet my mom and brother for a reunion at the school where she used to teach. The trip was exhausting for us, and I'm not sure we would have chosen to make the trip, except that it was Mother's Day weekend.

I had forgotten how huge the US is, having not undertaken a road trip recently. The land is tremendous and all the larger when there is a two-year-old in the car with you. After trying various arrangements, we discovered that it was most successful for me to ride in back with Iris and let Evan do all the driving. I also admit to letting Iris play on her computer for far too long.

Trying to stave off boredom in the car

I'm glad we had the excuse to travel this way through Colorado and New Mexico, though, because I can't imagine when else we would have decided to make a trip in this direction. Having a child with special medical needs has really cramped our traveling style. We brought her oxygen concentrator with us and she had to wear oxygen over the really high passes. Luckily, she didn't need it during the day anywhere we went. Or, perhaps better said, none of us could tolerate the idea of her being leashed with her oxygen cord for just the whiff she would need.

In any case, I can't adequately describe how incredible the land is. We drove through the most incredible mountain scenes over and over again and then found our way to the New Mexico plains. All breathtaking.

We had snow on the drive back north. 

Mesas by Farmington

For all the road trips I've taken, I don't think I've ever felt anxiety about being out in the middle of nowhere. I had a strange sensation of worry about what would happen if we had some medical emergency.

We met Mom and Mike in Farmington, and Iris got a chance to ride on Uncle Mike's skateboard, which was probably the highlight of the trip for her.

Sally remembers 1968

Moms and kids

By the end of the weekend we were exhausted and ready to be home. We also decided to wait to attempt another road trip for a while. The good news is that we think Iris doesn't need oxygen on planes anymore, so we can fly relatively easily. 

toots

Evan and I went on a date last night and on the way home we started reminiscing about our early days. Strangely, that reminiscing took the form of trying to figure out when we started passing gas openly in front of each other. I'll spare you the details, but I bring it up because Iris is increasingly showing she's ready to advance in the progression from diapers to the potty. We all talk about going potty all the time. And so in our conversation, Evan and I talked about our "toots."

I'm not pushing the potty all that hard on Iris, mostly because it feels like so much extra work to get her into undies and out of diapers. But, Iris has recently started pushing us by telling us, "I need to go potty." She wasn't really doing the same for poo-poos until last night when she got hit by a big, huge stomach bug that kept us up most the night. She insisted, "I need to go poo-poo!" Over, and over again. Somewhere around 3am I thought for sure that she would be only using the potty in the morning. That didn't exactly happen, but we've made progress, for sure.

We started informal potty training a long time ago with pee-pee stickers and pee-pee prizes. Now we've moved on to poo-poo stickers and poo-poo prizes. (The prizes are wrapped packages of chalk and crayons.) We read books about using the potty and Iris likes to inform us that, "diapers are not forever," which is the title of one of her books.

We had two doctor's appointments this week. The first was her 24 month check up. She was very scared and kept saying, "No shots! No shots!" Technically, she was scheduled for her second dose of Hep A, but the pediatrician said that since she's already had her first dose of it, we could wait just a little while to help ease her anxiety. That was a relief. Nothing special to report, otherwise. Iris is developing well. Not sleeping well, though.

The next day we saw our pulmonary specialist. Thankfully, the pulmonary meetings tend to be some of the more boring appointments because Iris is basically doing okay with her lung development, though she is still on oxygen when she naps and when she sleeps. She is showing improvement, slowly. A minor concern that we'll continue to keep an eye on is that Iris some times breathes fast. She doesn't pant, but it's just a little faster than I'm comfortable with and I worry that she'll develop asthma.

Her pulmonary hyptertension has resloved (about a year ago), so we found out that some of the pulmonologists wanted to enroll her in a big study. It's a long term study looking atchildren who have had pulmonary hypterension that has resolved. They don't use any genetic indicators, which is a bummer. They are hoping to enroll up to 1,000 children across 8 children's hospitals in the US.

Our specialist said that if we fly this summer it's possible that we won't have to rent a concentrator for Iris. This is really good news for us. Lugging the concentrator on a plane with a toddler doesn't sound like the most exciting thing to do on a vacation.

Iris borrowed our specialist's necklace during the appointment.

birthday

Iris finally understood that everyone at the party was there for her when we all gathered around the cake to sing "Happy Birthday." Her face lit up at the line, "Happy birthday, dear Iris..." And she successfully blew the candle out all by herself.

I was very excited for her birthday this year so we decided to invite a bunch of people over to hang out in our backyard. Evan's parents came to town, as did my dad. (My mom couldn't stay for the party because of some appointments she couldn't change back up in North Dakota. But we'll see her again in a couple of weeks.)

Iris and Granddad looking at apple blossoms

Iris with Nonna and Papa.

 In case we were going to forget that Iris is now officially a toddler, she reminds us by constantly saying, "No." Most of the time I then give her two options and she's happy to choose between one of the options. Maybe not my favorite feature of toddlerhood. Something much more exciting is Iris singing the "Itsy Bitsy Spider."

twenty-three months

Iris has outgrown the onesie that I have been using for her month-by-month photos. I blame my tardiness in posting on the loss of that onesie. (It's not actually lost. It's just in our "too small" pile, now). I'm now preparing to start counting Iris's age in years rather than months. That'll take some getting used to. 

Wearing her Easter outfit from Nonna & Papa

Just making a silly face

My mom has been visiting for the month and she has taught Iris the story of the Three Little Pigs. So Iris loves to say now, "Once upon a time, three little pigs..." I've done a bad job of getting photos of the two of them together but they sure do have fun and Iris wears my mom out. She has tons of energy.

I've recently come around to the idea that maybe Evan and I haven't been giving Iris's diagnosis its full weight. We were talking to someone recently and explaining the whole situation with Iris. We are able now to glide over the details pretty easily: there is definitely a second heart surgery in our future, possibly another medication to add if a valve needs to be replaced; she may have brain surgery to help increase blood flow and possibly surgery on her intestines if they find that she does indeed have malrotation. I manage six specialists and have multiple appointments with each of them every year, Iris is on three daily medications (not including the oxygen when she sleeps). And her longevity is not at all a sure bet. Kind of the opposite, actually.

To share all of this information at once gave me some perspective: it's a lot to deal with. Perhaps we haven't been fully acknowledging exactly how big of a deal Iris's health problems are. We have acknowledged it in a sense: we can talk about it freely without being brought to tears anymore. I spend a lot of time thinking about how we'll shepherd Iris though this part of her life without making it her entire life. And we have a growing list of books to read to help on all related aspects.

But I'm not at all sure that we've allowed our lives to catch up with the reality of how stressful the diagnosis is. Neither of us has really taken much off of our plates to make space for the added work of caring for a daughter with special medical needs and an extremely rare genetic diagnosis. Evan is working hard, as always, and I'm technically working full-time and working on my dissertation. 

I have been able to come up with some very creative work arrangements thanks to my extremely accommodating supervisor. But it also seems like I should either figure out how to reincorporate my dissertation into my life or just decide to let it go. And, of course, I want to spend time with Iris while I can.

We are very excited about Spring. Here's some fun we've been having recently: