Earlier in the summer I wrote about courage and whether Evan and I have been courageous in coping with Iris's diagnosis. I was struggling to figure out what the appropriate actions were for us to take in the face of the pretty gloomy prognosis. I considered quitting my job and quitting my dissertation to focus on being a mom and on raising money for research on the ACTA2 mutation and pediatric strokes. It's easy to find purpose in life when faced with what seems like a constant life or death situation. Your purpose becomes life. It's that simple and that complicated.
As the year has worn on I've continued to struggle with what my next steps should be. I've come to the conclusion not to make any drastic changes. I was a small signature away from quitting my dissertation altogether and somehow I've made the decision to keep going on it, same with my job. If I have to come up with a logical justification for it, I guess it would be: I don't want Iris's genetic mutation to become the most important thing in our lives. It is always going to play a leading role, but I don't want it to be the only thing that we do and the only thing we are about.
Back when we first got her diagnosis - not even a year ago - we decided that we wanted to focus on making her life (and our lives) meaningful and always to emphasize the idea that a long life does not equate to a meaningful life.
The most meaningful thing I think I can do for myself and for my family is to cultivate and spread opportunities for joy. This state of being has almost nothing to do with what we do or do not accomplish; instead, it has everything to do with our perspective and what we choose to surround ourselves with.
With that in mind, I want our lives to be as ordinary as possible and I want for us to find joy in the ordinary.
This was Iris's first legitimate Halloween. We did dress her up last year (as a dog) and passed candy out to the neighbors. She didn't understand why we were so excited to get her dressed up but when we showed her herself in the mirror she tolerated the costume a bit longer. Really, though, we didn't do much to celebrate.
Iris as a dog in 2014
This year we had our housemates to lean on for Halloween oomph. Troy and the kids carved a pumpkin, which put us light years ahead of where we were in 2014.
Gutting the pumpkin
We also took Iris trick-or-treating this year, even though we're not going to let her have any of the candy winnings. She dressed up as a koala and genuinely enjoyed dressing up. She is at an age where she wants to be EXACTLY like everyone else and so it's easy to get her to dress up if everyone else is doing it too. I'm not sure I love this inclination, though it does help with potty training.
A koala, Snoopy, a sunflower, a zombie marshmallow, and a mermaid.
The day after Halloween we asked Iris what she remembered about going trick-or-treating. She said, "dogs." There was a dog that scared her at one house. I asked her if people gave her anything when she was at the houses and she didn't seem to remember the candy...we left it at that.
We had a routine checkup with the GI specialist on Friday. Iris has weird digestive issues due to the ACTA2 diagnosis, but they've been even weirder since August. I was explaining the details of it all to the doctor during our appointment and Iris got very embarrassed and hid her face in my lap. She's shown her ability to be embarrassed since she was a very young toddler, but this is one of the first times I've noticed her being embarrassed when I am talking about her health problems with someone.
I imagine most kids don't want their moms talking about their pooping to strangers, even 2.5 year olds. Generally, though, Evan and I do our best not to talk about her medical issues within her earshot but it's very difficult. We aren't trying to keep things a secret from her, but she's just not old enough for us to try to explain it all to her so it's better to shield her from what we can.
We know from her past visits that she has a small gallstone and that there's not much we can do for that unless we want to get her gallbladder removed or start her on another lifetime medication. We've opted to do nothing since it's small. We also know that she does not have malrotation, which is something that other ACTA2 kids have had.
Iris got glasses. We took her to her regular ophthalmologist appointment and he decided it was time to give her glasses. He has told us in the past that she was very far-sighted (like all kids) and that as you get older you become more near-sighted.
He also said that he wouldn't have decided to give her glasses now but her left eye is doing more work than the right eye since it's less far-sighted, and in fact, he would rather see her more far-sighted in her left eye since we know that both eyes will become more near-sighted in the future (since that's what happens with all kids). He also emphasized that the far-sightedness in the right eye is very slight.
While I did know that many of the other ACTA2 r179 kids have glasses, I hadn't realized until later that almost all are far-sighted, and many need bifocals. So I guess it would be a safe bet to think that Iris might need bifocals in the future.
It's hard to get too upset about her needing glasses, though, considering that we originally thought she had aniridia, a condition far more vision-threatening. And she's so darn cute in these glasses. I took her to pick out her frames and she decided she wanted blue ones since blue is her favorite color. (I'm not sure how long blue will hold on to that title.)
There's nothing that makes you feel like a worse parent than watching your child fall down stairs, fully expecting that you will have been there to catch her.
Iris and I had a miscommunication on our stairs, due to my lack of full attention on her.
I did my best not to panic as I picked her up by her left thigh (I did catch her, but not before she hit the stairs chin-first). When I try not to panic I do the same thing: "Oh God, Oh God, Oh God... Okay, Okay, Okay...." and try to assess how much of an emergency it is (do I call 911? do we go to the emergency room?). When I saw her face her mouth was starting to bleed but I didn't see any other bumps on her head. I had the words of our neurologist (or cardiologist?) going through my mind: The threshold of taking her to the emergency room is lower than for other children because her blood vessels are more fragile. Bumps on the head should be taken especially seriously because she is at higher risk for hemorrhage.
As I tried to calm her down and explain to her that we might need to go on a car ride, she settled down surprisingly quickly. She really wanted her daddy who, unfortunately for all of us, had just left for two nights on a business trip. I gave her some Tylenol through the undamaged side of her mouth and was able to see that she didn't seem to have hit her head. The only visible damage was to her lip and her lip was the only place she said hurt. All her teeth were in tact and no damage to her tongue or sides of the mouth. She did seem mad at me, that's for sure, but she wasn't acting differently (aside from being in pain). I tried calling Evan (several times) and finally got through. He was calm about it (and not at all mad at me for letting Iris fall down the stairs), and we decided that she probably didn't need to go to the emergency room.
It was getting close to bedtime so I got her a bottle and we went upstairs to sleep. After she spent some time looking at her lip in the mirror, she went to sleep. The night started off quietly but didn't stay that way. She cried for a long time for her daddy and seemed to be in some kind of gut pain, maybe from the lentils I gave her for dinner (?). But after a while she fell asleep again.
I stayed home from work and she stayed home from school on Monday so I could keep an eye on her. I quickly realized that she wasn't going to let anything near her mouth, except a bottle. After a while I decided that I was going to have to try to reason with her instead of reward her with prizes for letting me put stuff in her mouth.
I explained to her that I wanted to touch the undamaged side of her mouth and she let me do that. I asked if it hurt? "No," she said. Then I touched some puffy snacks to the side of my mouth and told her that I wanted to try it on her. She let me, and again, it didn't hurt. She happily ate a lot of puffy snacks, which have basically zero calories, but at least she ate something. That was the extent of her eating until the late afternoon (despite several other attempts), when I was able to get her to eat some buttered pasta and take some Tylenol, with the help of "Bubble Guppies."
This photo doesn't do it justice.
Thankfully, mouth wounds heal quickly so I'm hoping that today will be better than yesterday. I'm still not ready to send her back to school since she's not really eating or drinking without extra convincing.
Lessons learned: I'm glad that I was the negligent parent in this situation because I think I would have had a very hard time forgiving Evan for such a gaffe. The truth is that I'm not having the easiest time forgiving myself, either, but I've had a good reminder that accidents happen to everyone and kids are very, very resilient.
Iris has been saying, "I'm really brave," and she is, even if it's because we force her to be because of our medical treatments or our mistakes that turn into boo-boos. She's also one of the least risk-averse people I know.
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.
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.
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."
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:
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.
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.
Iris is finally starting to sleep better in her big-girl bed. Around the beginning of the new year I bragged that she was starting to sleep throught the night, "for real." Well, that didn't last too long. Especially since we changed her bed around on her. She loves her bed, but it's hard for her to stay asleep sometimes. We've tried to make her bedtime routine very, very predictable and I've gone back to the idea that she still needs milk around 9:30pm or 10:00pm in order for me to have a semi-restful night. Evan is the one who usually gets her ready for bed, brushes her teeth, washes her face, and reads to her before giving her milk. Tonight as I was leaving her room she said, "Daddy, come!"
When not asleep, Iris prefers to run everywhere she goes. She has also managed to get a couple time outs for jumping around dangerously on the couch. At twenty-two months old, she repeats everything we say (for better or worse) and she is really starting to perfect the tantrum. Her latest obsession is my brush. She threw a little fit this morning when I tried to give her a comb instead of the brush. SHe likes to try to comb her hair by herself, but that just lends to the unruliness that is her hair.
We have been working on our yoga moves -- the latest attempt is tree pose. She can balance on one foot for just a split second. She really loves to do happy baby pose. In order to get into the pose she kneels down on the ground, puts her head to the gorund first (so that she won't bang her head when turning over) and then turns her body around so that she is on her back. Then when she has a hold of her feet, she yells, "Happy Baby!"
Twenty-two months old
Balancing on one foot
The weather here has been a little insane. We woke up to snow this morning (Wednesday) after having had several days in the 60s. The good weather inspired Evan to plan a super busy weekend full of hiking, bike riding, and an attempted swimming trip. Iris did really well on the hike, possibly because I was trying to feed her for the entire 3-mile trip. Evan also started singing a song to her that seemed to keep her entertained. The words went something like, "Hiking, and mommy, and trees, and sandwich, and birds, and mommy, and hiking, and backpack, and Iris." He also had so much energy that he was running back and forth with her when she started to ask to be let out for a little while. He would bounce up and down with his arms out like wings. She loved it.
Backpack walk
Roxborough State Park
I mentioned previously that I had gotten a glass flying pig from a mystery person at work. Well, this same person (I assume) sent another little gift a week or so ago. I'm not sure what to call it, so we just call it "This Guy." Iris calls him "This Guy" now, too.
This Guy
Hugging This Guy
The handwriting of the mystery gift person
As far as medical things go, we see Cardiology on Friday. Iris will have an echo and we hope to see no increased dilation in her aorta, and continued lack of pulmonary hypertension. She is now on her full dose of losartan (when we can manage to remember to give it to her). It looks like we will be starting Iris in day care in March, which Evan and I are both excited about...though there are many logistics to be worked out. Evan still hasn't actually seen the day care yet, so we'll visit it on Friday before the Cardiology appointment.
Then, next week I'll likely take her to the pediatrician to get her second to last dose of Synagis, which is the semi-vaccine for RSV. It's way worse than all the other vaccines -- way more of it, and it's two big shots that go into each thigh. Iris has been asking about shots a lot lately. We've been reading "Elmo Visits the Doctor" book a lot and I think she's going to handle the next round of shots okay... but they're still awful. But I'd rather subject her to the trauma of getting the shot than to the trauma of being hospitalized for a severe case of RSV. And let's just leave the whole talk of measles for another time.
At twenty-to months, Iris's favorite things are her stuffed animal bears, looking for buses when we go outside or are in the car, singing "Wheels on the Bus," and saying "Call Daddy" to get me to call Evan on the phone.
We have added two new specialists to our roster. We were expecting the neurologist, but the hematologist was an added bonus. It's not that Iris has anything wrong with her blood. Her problem lies entirely in the vessels carrying the blood. Some of the bigger ones are dilated (aorta, internal carotid arteries), but the smaller ones in the brain are too windy and make too many corkscrews. When blood bumps into vessel walls the platelets get activated and start trying to form clots. Enter stroke.
As you may or may not know, Iris had her MRI on a Friday (January 23) and then we didn't meet with our new specialists until the following Tuesday. We did get initial results on Friday evening from our cardiologist, but much of the report didn't mean that much to us.
In addition to the brain MRI and MRA, they did an ultrasound of various other organs potentially affected by the ACTA2 r179 mutation. The good news is that those organs--pancreas, liver, gallbladder, spleen, kidneys, bladder--look normal. She does have a small gallstone. So that means another specialist...that'll put us up to around seven. But the good news is that she won't have to have surgery for malrotation of the intestines. Hers are rotated just fine.
Fake smile pre-MRI.
A little zony after the MRI, but still wants Mama's phone.
But back to the brain. Iris has white matter lesions typical of others with the ACTA2 r179 mutation. This means that parts of her brain that help control motor development have suffered some damage. She seems to have compensated, though, since there are no observable delays on either the left or right side of her body. The neurologist thinks that *maybe* her left ankle is slightly more stiff than the right one.
As far as the blood vessels, her internal carotid arteries are dilated, and the middle and anterior cerebral arteries branch abnormally. One of her basilar arteries doesn't make it up to the Circle of Willis. It does feed the parts of the brain it's supposed to before making it to the Circle of Willis, but then just stops. And, as I mentioned above, some of the vessels are like little corkscrews and they're not supposed to be. The blood wants to clot and we need to try to avoid that.
So, what do we do? Aside from try to find time to learn some new anatomy...we've started her on a daily aspirin routine. Aspirin has an anti-platelet action that helps. We also have to keep her really well hydrated and well electrolyted because blood is more likely to clot if it's got less fluid in it. She can't participate in contact sports, and shouldn't go on roller coasters or do anything that will make her crane or contort her neck.
So I'm thinking piano will be great for her...dance, yoga -- just no headstands or shoulder stands. I'm a huge fan of walking, too. I suppose she'll be allowed to run, but we'll have to check on how her aorta handles it.
In the world outside of medical stuff, Iris is a joyful force to be reckoned with. She knows all her ABC's, can count to 10 (sometimes), and is starting to put longer and longer strings of words together. She has taken to naming people she knows and then saying "Come!" because she wants them to come visit.
New words come out of this girl's mouth every day. She is making jokes, lying, and constantly surprising us with the things she remembers. Lately, she has been saying people's names and then saying, "Come!" I ask her if she wants that person to come over and she says, "Yeah!"
It's such a privilege to get to watch this little human grow up. We have been working on having more family fun and mostly succeeding at it. Iris loves backpack walks, though if she's hungry they don't work so well.
Playing with Mama's keys
Backpack walk
Today I was playing Raffi for her and the song "Thanks a Lot," came on. She said, "Hug..." and then, "Kiss..." in the direction of the music. I asked her if she wanted to hug and kiss the song and she said "Yeah!"
