Iris's daycare had it's official "back to school" night - our first ever. She goes to a Jewish preschool that is on my way to work. I haven't mentioned it much, but she's been attending 2 and then 3 days a week since April, though with several short breaks over the summer -- some voluntarily (vacations to California and New Mexico) and some not (recall hospital stay).
Iris is possibly the most social and outgoing toddler I've ever met. She loves kids, especially. Even with all that, she still doesn't like going to school in the mornings. She always says, "No kids. No school," while we drive there. But she very rarely cries when I leaave and instead goes off and plays either by herself or with another little child. I think she has two difficulties that contribute to her anxiety at school. One, not everyone understands her; and two, she does not follow directions well. I very frequently have to count to three before she obeys me or I have to tell her what the consequence of her disobeyance will be ("I'll take away your blue blanket...").
Part of the surprise at the end of summer comes because we've been so busy right up until the end. We have welcomed Evan's best friend and his family to stay with us in our house for a while, which has been great fun for us all. Iris loves the three new kids we have roaming around our house. We don't have a huge house so it is admittedly a tight fit, but Evan is right when he says that it wouldn't be as easy with any other family.
We also had a vacation to California for Evan's parents' 40th wedding anniversary (wow). Iris got her first taste of ocean (literally) and spent hours running through the house screaming with her cousins.
Playing in the California sand
And, lastly, the excitement of getting ready for school has been supplanted by preparing for a new baby sister, coming in January. I keep forgetting that I'm pregnant, though recently it's been harder to forget. Iris, on the other hand, announces it to people frequently: "I have a new baby sister!" and "I'm a big sister!"
I've mentioned in passing that Evan and I have amassed a set of books to read as a way to process where our lives are and where they are going. Maybe I'm the one who has mostly amassed the reading list. Included on this list is Being Mortal: Medicine and What Matters in the End by Atul Gawande. One of the final chapters is titled "Courage."
He begins the chapter by telling of Plato's dialogue Laches, which deals with the subject of courage. What is courage? I can't speak for Gawande's interpretation of the text, but he concludes with observations that particularly resonate with our experience:
"Courage is strength in the face of knowledge of what is to be feared or hoped. Wisdom is prudent strength.
"At least two kinds of courage are required in aging and sickness. The first is the courage to confront the reality of mortality - the courage to seek out the truth of what is to be feared and what is to be hoped....But even more daunting is the second kind of courage--the courage to act on the truth we find. The problem is that the wise course is so frequently unclear. For a long while, I thought that this was simply because of uncertainty. When it is hard to know what will happen it is hard to know what to do. But the challenge, I've come to see, is more fundamental than that. One has to decide whether one's fears or one's hopes are what should matter most." (p. 232).
To take this further, one has to decide how to live given the likelihood of a variety of outcomes. The problem with dealing with a rare diagnosis is that medical professionals don't have much to tell us about what to expect. There aren't enough cases to draw statistical conclusions and so we are all running on case studies and personal experiences of other families. So in other words, how do we figure out how likely it is that our fears will come to pass? We have to go on gut feeling and the stories of the roughly 20 other families with the ACTA2 r179 diagnosis.
I think that we've embodied the first type of courage in facing Iris's diagnosis. We have sought the truth of what is likely to happen and haven't shied from it. The second type of courage... the courage to act is more difficult. What do we do with the knowledge we've gained? This is where I'm kind of stuck.
We went home to New Mexico for the 4th of July. Iris is still on oxygen after this most recent respiratory infection, so we rented a concentrator and flew down to Santa Fe. Had the batteries for the concentrator worked predictably, the flight out of Denver would have been very low stress... or at least medium stress. Unfortunately, we discovered that one of the batteries wasn't working while we were at the airport and that pushed Evan and me into disaster management. We don't handle disasters in the same way, which contributes to the stress. We were finally able to arrange for last minute oxygen service while in Los Alamos and were still able to make the flight without medical emergency. Phew.
The flight itself was easy. Iris loved flying in the airplane and napped on Daddy's lap as I daydreamed and looked out the window. We flew over the Great Sand Dunes and I was struck with regret that Evan and I haven't done more hiking since we've been in Colorado. Not that our lives have really offered that opportunity lately.
Once we got to Granddad's house, Iris immediately wanted to play with Granddad. Her favorite things to do at Granddad's house are to play with water and to play the piano. We somehow haven't managed to get to a swimming pool this summer in Denver, but we did make a quick pre-rain trip to the neighborhood swimming pool in Los Alamos. Iris gets cold very quickly in the water, but she loved having Daddy throw her in the air.
A sprinkler and a bucket
Singing, "The Wheels on the Bus," and "Mary Had a Little Lamb."
A new favorite activity
Iris tolerated the oxygen cord well while we were on our trip. She actually doesn't like to take it off sometimes, which seems weird to me. We are doing twice daily Flovent treatments, which she barely tolerates though she likes them better when she can pass the mask around to everyone to make sure we all get our treatments. "Oh! I forgot! It's your turn!" she hollers.
Flovent treatment, twice a day
We got to hang out with my brother a fair amount, too, which is always valuable.
Uncle Mike & Iris discover a pen with a light on it
Feeding the ducks at Ashley Pond
Incredibly, I didn't get any good photos of Iris and Granddad, but here's one of them as we go into the Santa Fe airport on our way home.
Photo with Granddad
The trip did feel like a vacation, in spite of the massive load of stuff we had to pack with us. Traveling with a toddler is much more complicated than traveling solo, and I think we're getting pretty good at it. I still haven't created a packing checklist, which my dad always suggests as a good way to prepare for any trip. Or maybe I have and I forgot about it... In any case, we're headed out to California next for Evan's parents' 40th wedding anniversary. We're very excited and Iris won't have to be on oxygen at sea level. Hooray.
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.
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 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.
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.
