We've been in the hospital since Tuesday evening and were transferred to the ICU on Saturday. Iris has RSV with pneumonia. The pneumonia spread from her right lung to her left lung, which was why we had to go to the ICU.
We've managed to get Sibyl care covered so that I can be with Iris at the hospital during the day and Evan sleeps at the hospital at night. We are pretty sure that we've also developed RSV. I have had a low grade fever the last two nights, and I can only assume Evan has the same considering the sleepless nights spent in the room with Iris. I've been very worried that Sibyl is also going to get sick since she's so young, but she hasn't shown any signs of illness so far, aside from some snotty nose in the morning.
As far as medications go, we started her on amoxicillin for the pneumonia but it wasn't working so we switched her to an antibiotic called unasyn, which seems to have had the intended effect. We also started giving her atrovent and pulmozyme to help break up the mucus in her lungs.
When I got to the hospital this morning Evan said she had requested mac and cheese for breakfast. She also requested mac and cheese for dinner. We got her two orders of mac and cheese for dinner and she ate almost both of them.
Mac & cheese!
She spent several hours on regular "wall oxygen" rather than on the CPAP machine today and was starting to jump on the bed. She also tried climbing off the bed one or two times. She is definitely feeling better.
CPAP mask = no fun, lots of TV
During the afternoon trial on "wall oxygen" she was at 4 LPM. They usually are willing to send us home when she gets down to 1 LPM of oxygen. (For reference, she usually sleeps with 1/4 LPM of oxygen and doesn't need any oxygen at all while awake).
Managing a hospital stay as parents of two children has proven to be quite a feat but we've had an outpouring of support from all over the place, all of which we really, really appreciate.
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.
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 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.
Thanksgiving this year was very quiet. We had planned to have a bigger gathering, but it ended up being just my dad joining us, because Iris had been terribly sick for more than a week prior to the big day. She had started vomiting before I left for two nights to a conference on the 20th, and basically kept it up until the Tuesday before Thanksgiving. We stopped giving her the Losartan, in case she was having a reaction to the medication, but it seems like she might have just picked up a stomach bug.
Granddad's pretty crazy about Iris
I went to yoga in the morning, which was a fantastic way to start the day. Jill and her family came for a short visit, and Iris got to practice hugging and kissing a lot. We had a big meal to celebrate, and Dad gave us a Thanksgiving Day quiz.
Dara & Iris play with toys
I'm not sure when we'll start her on her medication again. I'd like her to gain some weight back now that she's feeling better. She went a few days without eating anything at all and basically only having formula. It's likely that we'll start her on the Losartan in a week or so.
Otherwise, we are doing okay. And by okay, I mean that there are a
lot of highs and lows, related to all sorts of health and insurance
woes, along with the regular challenges of parenting, taking care of
ourselves, and maintaining a relationship. Things definitely look
brighter when Iris is feeling good, though, so we are relieved to have
her keeping her food in her belly again. So -- there is a lot to be
thankful for this year, and I'm glad to have a day devoted to the
intention of gratitude.
Iris is nineteen months old today. Now that she has mastered saying, "No," she has started to excel at miniature temper tantrums. Another funny thing she does is she tries to hold all the important things at once. So, if she has decided that she absolutely must have the bouncy ball, her two blankets, and her teddy bear all at once, she tries to pick them up and walk around with them. This usually proves impossible.
Here she is smiling for Daddy.
The things I did today to deal with the tantrums seemed to work alright. Usually the tantrums start when she and I want different things, so I explain to her that we are doing my thing first (eat) and then we will do her thing (read). Or, I tell her that we are almost done reading and that after reading we are going to go upstairs and change her diaper. She is usually very good at cleaning up after herself (when we ask her to) and I am trying to reinforce that by having her put her toys away with some regularity.
Her favorite things: baths, running, singing "The Wheels on the Bus," the moon, doggies, bears, people of all ages, and getting tickled.
As far as medical/health items...she is doing fine on her Losartan. They plan to increase the dosage slowly over several weeks. She does occasionally seem a little dizzy. I suppose this will be a life-long medication, though we haven't actually confirmed that.
She has her 18-month check up on Thursday, and will also be getting the first round of the RSV shots, which hurt a lot. I'm not looking forward to that. Friday we meet with the four cardiologists.
Iris is doing a good job of growing. It seems strange to think that I used to be so worried about her growth. I remember the worry well, though her growth charts show that she is doing fine. She is WAY taller than most girls her age (above the 97th percentile) and has made it into the 15th percentile in weight-for-length.
Iris is above the 97th percentile for height
Iris is above the 15th percentile for weight-for-length
Iris gets her CT scan tomorrow morning. She has to have been fasting for 8 hours, so we kept her up a little later than usual tonight and gave her some extra food. Then I'll wake up at 4am and give her some formula, which she can have 6 hours before the scan. The fasting is required because she'll be sedated.
The sedation makes preparations easier in the sense that I don't have to try to explain the whole thing to Iris. The hospital does have a little model CT scanner for stuffed animals, so I imagine that we will make use of that tomorrow.
In addition to the scan of the heart, the pulmonary team wants to get some images of her lungs. She'll also have blood drawn for some genetic testing -- an aortopathy panel. And just to round it all out, they'll check to see that her kidneys are flushing the Losartan out properly. I suppose that she'll be on Losartan for the rest of her life, which is a bummer, though I'm glad such a medication exists, I suppose.
We then wait a week to get the scan results, so next Friday we'll meet with a team of cardiologists who will be able to tell us how big the bulge is in the aorta and also give us some more information on the leaking happening with the aortic valve.
In other news, today Iris officially started saying "no." It's fun to see her experimenting with new words to see how people react.
Evan and I are holding up okay. It seems like our emotional reactions to all this news comes in waves. I am much more vocal and weepy with it all, while Evan only occasionally mentions his feelings. This is true generally. We also have been so busy with the rest of life that we've had very little time to sit together and process it all. We work well together when there are decisions to be made, or if there is hard thinking to be done. Situations like this require a lot of that.
I've been looking at photos and videos of Iris over the last ten months. She's growing so well. She's particularly growing well now that she's off digoxin. Her ten month present to me last night was to take 4 oz from a bottle. However, her Valentine's Day present to me was to get a nasty stomach bug and vomit her insides out this morning. She's doing fine, but definitely under the weather for her first Valentine's Day.
Iris on February 11 -- ten months down
For her first Galentine's Day, Jill and Dara came to visit. The girls had fun together but are not sure how to touch each other without grabbing and pulling.
Iris now has nine months under her belt. She is crawling all over the place and pulling herself up and balancing. We have a play pen in the living room that she uses for standing practice. She is also all about eating Cheerio's.
We started a music class this past weekend. Iris loved all the activity, although she was a little overwhelmed by it, too. The music class lasts ten weeks so it will be fun to see her start to learn the songs and the movements. She is very good at clapping her hands and likes to take other people's hands and make them clap.
We had a cardiology and pulmonology appointment on Friday. She is growing well -- 29" long (93 percentile) and 16 lbs 11 oz (25 percentile). We stopped her final medication -- the digoxin. I am supposed to observe her over the next few weeks to see whether she has another SVT episode after coming off the medication. I'm also able to measure her heart rate using the pulse ox.
Iris is still on oxygen, which is kind of a bummer. I had hoped she'd be off it by the new year. But she has had a cold and I'm just taking it pretty slowly.
I spent most of Friday getting ready for our trip to New Mexico. I know that all babies require a lot of gear but this baby's extra gear is a real pain to procure. I had an intention to be very calm and mellow for the whole day but the time spent frantically trying to get Iris her Digoxin refill after the pharmacy botched the refill order shot me right out of any serenity. I spent a good hour or so grumbling to myself about the whole thing. It's amazing to me how many times things would have gone wrong had Evan or I not spoken up.
The good news, however, is that I got the medicine, we have extra pulse ox probes to measure her oxygen levels, we're taking the oxygen concentrator with us, and she got her Synagis shot in the afternoon (a few days early). So she's ready to go...medically speaking. She may not be ready for the six-hour drive, but we're leaving super early so as to maximize sleeping time.
I'm always very happy to go home to Los Alamos, especially for Christmas. Evan is also looking forward to the break. Here is a photo of him packing. And he just said, "I'm really excited to go to New Mexico."
We then had a family dinner out at an Indian restaurant.
Being 33 feels pretty good, all things considered.
Iris had an appointment at the Heart Institute and also met with the pulmonary nurse. It was an exciting meeting. They have concluded that her pulmonary hypertension is very mild and that she has likely outgrown her Sildenafil dose. So we can stop giving her Sildenafil. This is the medication that I had been giving her three times a day, once at 4:45 every morning. More sleep for me! We will also try her off Lasix in mid-December, about a month before going back for the next appointment. Otherwise, she's staying on Prevacid and Digoxin for a bit longer.
The other exciting news is that we are to start weaning her oxygen. I have had her back up to a 1/4L for the last few days because she seems to be showing signs of a cold. Evan and I both have some lingering sickness and it seems she got a bit of it.
When we first got the pulse ox for home use I was supposed to keep her oxygen saturation level to above 94%. Today they decided that we can lower that a bit, to above 92%. I don't totally love this as a new threshold but I will start weaning her a bit more once she has recovered from this most recent cold. They also mentioned that some babies have to have oxygen while sleeping for years, even after they are off it while awake.
And, she is on track with her weight. I had been quite worried about this, particularly since I've been sick and not producing as much milk. She weighs a whopping 6.61kg (14lbs 9oz).
After losing our babysitter to a full-time job, we've started a search for day care. I would like to find a place we can take Iris part time so that I can actually make some progress on my dissertation. Evan is starting to come home early twice a week so I can leave and work on it, but that's not going to be enough. And all the potential sitters I find have full-time jobs and want to come in the evenings. So we're considering day care. Also, Iris is starting to get bored during the day and I just don't have it in me to sit and play with her all day long, especially when the house still needs to be cleaned and unpacked.
Our first visit to a local day care was this morning at 10:30am. As we left after the tour, Evan said something like, "The idea of leaving her at day care made my stomach sink." Like a vague, sinking dread. We didn't love this day care. Evan was particularly dismayed at the food offered at the day care...chicken nuggets...fruit cocktail in syrup. If Evan and I could have our way we'd take her to a day care where they served fresh cooked organic food, but I highly doubt we'll find something like that. And in any case, she's not even eating anything yet. Well, I did seem to find a rice cereal that works, but otherwise, nothing. [I'm not wild about
giving her rice cereal and plan to give her avocado as her next food. I
tried oats today and she didn't seem to love those.]
Evan took a mini stay-cation this week --- two and a half days off of [office] work. He has done an incredible amount of work on the house and has involved himself in Iris's bedtime routine. She is mesmerized by the guitar every time he plays it. Tonight she snuggled in to Jeff Buckley, Elliott Smith, and Evan's standby -- JS Bach. He plans to continue the much needed break by not working over the weekend, too. Tomorrow we'll probably go out for breakfast as long as Iris is up for it and then he plans to go back to the Brazilian jiu-jitsu training center. He went tonight for the first time in a very long time.
Iris had a rough week. Monday night we had another freaky (but shorter) maybe-SVT episode. I had forgotten to give her her Prevacid [for reflux] on Sunday so I decided to try her off it for a few days to see how she did. She still needs it, it seems. She is also teething and has been in some pain because of that. I started her back on the Prevacid and have been giving her Tylenol for the teeth so today was a pretty good day.
Back to day care. It is controversial [like most things related to parenting, and, well, mothering, specifically]. A recent Slate piece sums it up nicely. Even though Evan and I did not love this day care we visited, Iris did like it. That is, she liked the toys and the nice employees and it seemed that there was a lot of potential for stimulation and social interaction -- two things she lacks at home. Some days I am the only person she sees and even though I do think that the parent-child bond is absolutely essential to her healthy development, I do not think that it is enough for her to see only me. And it's certainly not healthy for me to be "on duty" 24/7. Here's a Forbes article summing up the link between 'intense' parenting and depression in women.
I am coping fairly well but it is very definitely time for me to get a bit more of a break than I've had. I lost my wallet and I had a mini meltdown when I realized it. This was the morning after Iris had been up several times in the night and I was sleep deprived and too tired to exercise. A bad mix. The good news is that my wallet was in the lost & found at Ikea [fully in tact]. And now I'm off to nurse Iris one more time before I fall in to bed and I hope to stay there until 4:45am when I have to give her a dose of Sildenafil.
We spent Friday afternoon at the clinic to meet with the cardiology team and the pulmonary team. Iris has been doing really well and I was feeling upbeat about the visit. As I've mentioned in the past, I realize I have not been particularly explicit about all of her diagnoses. I have tried to right that on this page. We had been under the impression that she was perfectly healthy--save for a case of reflux--so this was all a huge surprise to us.
As far as prognoses go, the doctors had prepared us for the worst. The worst being: shortened life span, neurological and cognitive development problems. After being shocked with the worst possible outcomes we have continued to ask what they foresee for Iris.
Their responses are quite positive: from a cardiac perspective, they will monitor her closely for years to come but hope to have her off the medications by age 1. She still benefits from the oxygen's vasodilation [widening of the blood vessels] effect but they do not expect that she will need extra oxygen for the rest of her life. On the pulmonary side, she is breathing much better and her oxygen saturation levels are up. There is added risk during flu season so we are to be extra careful not to let her get sick and if she does to respond immediately. The pulmonologists think that she will grow out of all of her lung problems so we have to just wait and monitor her ease of breathing and oxygen saturation. Yesterday we tried her off the oxygen for a short time and her saturation levels quickly dropped to the low 80s. When she is on a 1/4L of oxygen the saturation levels are in the mid-90s, right where we want them. Finally, we have to wait until October to see the ophthalmologist again. Previously we had been told that the eye structure looks good and that she probably has pretty good vision. They will test her vision when we visit again. She does not have total aniridia so she is able to handle being exposed to some light. She does show sensitivity to indoor light so we try to keep the lights low when it seems to bother her. Outdoors she always has sunglasses on, unless it is dusk.
She has been tested for genetic disorders associated with aniridia and cardiac problems - all of the tests came back negative. This is a good thing, however, it is confusing because aniridia results from a deletion of the PAX6 gene on chromosome 11. The geneticist said that this deletion probably didn't show up on the test because it is a smaller deletion and suggested [at my request] that we get further testing to determine how large the deletion is.
We won't know about cognitive problems for quite some time. The surgery could have caused some brain damage and lack of oxygen could have also caused problems. But she is hitting all her developmental milestones perfectly so we're hopeful. She is one of the most social babies I've ever seen [not that I have much experience] and Evan is very proud of how happy and interactive his extroverted daughter is. "It's so amazing to see her growing and developing," he remarked the other day. "I'm sure all parents say that." Yes, an awful lot do, but we still count ourselves very fortunate to have such a sweet girl.
Because she is looking so well we don't go back to the clinic until November. Now we just focus on growing -- she is now 12 lbs and 25 inches long -- and playing.
Iris was having trouble breathing yesterday so my mom and I brought her to the emergency room just after 5pm. I called them on the way to let them know we were coming. I don't know if that helps things move faster but it couldn't have hurt. We've never had to wait the two times we've been in the emergency room but it probably is because she is a cardiac baby.
The short of it is that the steroids seem not to have worked and that it appears she still needs her Lasix to get rid of fluid in her lungs. In other words, she may not have had any inflammation in the first place and all she needed was to up the lasix dose since she's been gaining weight. The question then becomes - why does she still have fluid in her lungs? Unknown.
We got discharged from the emergency room around 11pm, maybe a bit earlier. I started out wanting very much to go home right away but at one point they talked me in to staying over night because she apparantly needed more oxygen [see below]. Evan drove down to meet us and helped re-convince me that there was no need to stay over night.
A big baby milestone was that Iris laughed for us while we were there. She has been laughing in her sleep but not while awake. Waking laughs are a new treat.
Now then, on to the oxygen story. I have become increasingly unimpressed with the medical folks we're dealing with. That's not to say that I am not sympathetic to the very real human aspect of practicing medicine. As a mother, though, it's infuriating when you think you are doing the best thing for your child only to find out that you have not been told the whole story. It's a problem of asymmetrical information combined with inconsistent care from the millions of nurses and doctors who see Iris. I could go on bu I'll get to the point.
When we arrived and were checked in, I confirmed that Iris was on 1/2 L of oxygen as directed by the pulmanology team at our Friday appointment. As far as I knew they left her on 1/2L when we were sent to the room. So imagine my surprise when the fellow came in to relay the message that they were concerned that she needed a full liter of oxygen to keep her sats [saturation levels] up. No one told me they had changed her oxygen flow at any time. The opposite happened at one point. When we took her to do a chest x-ray we put her back on our mobile oxygen tank at 1/2L. If she was on 1L beforehand they should have put her on 1L for the x-ray. They did not. All that time her sats were fine, meaning above 94%.
When I was told of their concern about her needing 1L we got into a long discussion about the chart and when they would have changed her oxygen level and particularly why they would have done it without telling me. Basically, I think it was mischarted and the nurses had a harder time setting her oxygen level on the wall than on our home machine. So they meant to set it to 1/2L but it was accidentally set higher.
Anyway, to avoid beating the dead horse, I later requested that they lower her oxygen when it was clear that 1L was keeping her in the high 90s and even 100, higher than necessary. At 1/2L she dropped to where they wanted her all along, above 94%. (This was after receiving her dose of Lasix, too, so that muddied the waters.)
We all came to an agreement that we could go home and come in for a follow up in a day. She had a previously scheduled appointment with Dr. Sagel today, so we're keeping that and then return to the cardiology clinic Friday a 8:30am.
Today Iris is 3 months old. I typed this all with one hand while she sleeps in my arms.
We met
with cardiology and pulmonology today in a 5-hour visit to the hospital clinic.
They did an echo, an EKG, and an x-ray. The conclusions: she has very mild
septal flattening while off oxygen so she will stay on the oxygen and continue
the sildenafil. We can take her of the Lasix. Her oxygen saturation levels are
low so she is now up to 1/2 liter instead of a 1/4 liter. The x-ray showed that
she had hyper-inflated lungs so she is starting on steroids to address the
inflammation. She will have 3-5 days of a ‘steroid burst’ and then we will
continue to give her inhaled steroids for 3-6 months. Evan and I are both
unhappy about this, particularly Evan. I am hopeful that we can keep this
shorter rather than longer but it is important to protect her lungs as much as
possible. She gained weight again - averaging 25 grams/day for the 18 days between clinic visits. She is now 4.95 kg (10.9 lbs). We think of her as a normal-sized baby but she is really still quite skinny.
The baby monitor arrived yesterday in the mail so Iris slept in her big-girl crib last night. I got almost no sleep. We had been keeping her in her bassinet, which had been in our room with us (actually on our bed). It is much easier to check on her when she is sleeping right next to us so last night was pretty sleepless for me. I hope that I'll learn to sleep better with her in the other room.
The big event of the day for Iris is that she started lifting her head forward as if trying to sit further forward (or up) in her bassinet. I have been worried that she won't keep up with her physical development because of the surgery. We're supposed to be doing tummy time with her but keep an eye on her pain cues.
I have been successful in incorporating walks into our daily routine. Yesterday's walk was quiet and cool. Today's was much hotter. I also was also able to read some of Gulp: Adventures on the Alimentary Canal by Mary Roach. This is the book my book club is reading this month and I am very hopeful I'll be able to attend the June meeting. This is the same book club I was in back in 2010 when I was living in Denver. A couple of my girlfriends from high school are in it as well as some of their friends. I really look forward to getting involved again.
I've been getting many compliments about what a good job I'm doing with Iris. While I (of course) appreciate the positive feedback, I really don't think I'm doing anything that anyone else wouldn't do. I had planned to be home with Iris during this time even before we knew of her health issues. Confronted with this new reality, we are responding the only way we know how... I think. That said, it would be very easy to wallow in self-pity. I have found it tremendously helpful to hear from those who have had similarly difficult experiences. I generally enjoy reminding myself that I am not at all that unique; in the case of Iris's recent health issues, I doubly enjoy knowing that our story is not all that unique.
It also helps to remind myself that none of this is permanent. Soon Iris will have grown out of her reflux (cross your fingers) and she will not need the various meds and oxygen anymore. That will be a happy day. A year out (for the digoxin), but a happy day.
In my limited free time I have done some perusing of available jobs nearby. After living at Children's Hospital for nearly 3 weeks, I had in my mind that perhaps I'd try to get into hospital administration rather than higher education administration. I miss having a job (outside of the house) quite a bit. I have decided to take a leave of absence from my PhD program. This means that instead of defending my proposal in August I will take off the the fall semester and hope to defend in the spring semester. After I defend my proposal I will be ABD (all but dissertation) and I will then (only) have to complete the dissertation itself and defend it.
Today was a two-vomit day. I keep trying to tie it to a particular medication. Really, I just want her off of all of them.
Tomorrow's goals: walk, nap, call opthamology for appointment (I keep forgetting that one), write Anne a letter.
Trips to the doctor take pretty much all day. I was prepared for this but unfortunately it meant that it was unlikely for me to get a nap. (Luckily, I was able to squeeze one in after Evan got home). Our appointment at the Cardiac Institute was at 12:30pm and I left the house around 11:40am to get there. It's amazing how much longer things take when towing a baby and baby gear.
While we were in the waiting area the cardiac surgeon who operated on Iris passed by and he stopped to say hello. He's done that several times when we've been in the hospital. Today we chatted about Iris's health, as always, and he asked whether the opthamologists had said anything more about her eyes. He also shared that his dad lives at home with him and therefore has learned how trying it can be to deal with oxygen tanks. That's true, but what's more trying is Iris's medication schedule.
Unfortunately there was no weaning from her numerous medications today. There was discussion of whether to take her off of Lasix (a diuretic) but no dice. So she remains on Digoxin (for SVT), Sildenafil (for pulmanory hypertension), Lasix (for pulmanory hypertension), Zantac (for reflux), Prevacid (for reflux), Simethicone (for gas), and (newly) Nystatin (for thrust).
Here's the good news: she gained weight since her Monday clinic visit. She tipped the scales at 4.49 kg (9lbs 14oz). This is essentially the same as at the pediatrician's on Thursday (yay for consistency) but more exciting is that this weights equates to a 21-gram daily gain since her last clinic visit. That falls within the "normal" range what babies are supposed to be gaining: 20-30 grams per day. The nutritionist told me not to change anything except that I can incorporate slightly more nursing as long as I make sure to keep up with the fortified breastmilk using formula. Honestly, it's a pain to have to pump every three hours so although I'm glad that she's gainig weight, I would love not to have to continue with the feeding/pumping regimen.
Lastly, we can now stop putting a bandage on her incision wound. It has healed well and can now stay open to air. So all in all, the visit was a success. We'll return in two weeks, at which time they'll discuss weaning off meds/oxygen depending on the results of tests (which tests? not sure). Next up: vaccinations, on which she is late.
Evan rode his bike to work today (50 minutes door-to-door) and I was able to get a bit of exercise in the morning and a nap early in the evening. As I write this the dishwasher is running and I keep thinking I am hearing Iris cry. She is asleep -- not crying.
Tomorrow's goals: go on a walk with Iris during the day, no vomit, start to get her on a better eating schedule, possibly catch up with some friends, read a book (?).
We are home now. Iris was discharged from the hospital on Monday (June 3) and we have been trying to establish the semblance of a routine in our new apartment. It has basically been really difficult ever since we got home, starting with the first panicked dash to find where our diapers and wipes were (still) packed.
It seems that the reflux medicine is either not working or I have not been able to get the dosing correct for the gas medicine. (I'm sure Iris will appreciate my writing about her gas pains on the internet). Yesterday I was concerned because she vomited and we were told to call the Cardiac Clinic if vomiting recurred. So on our first full day home I was convinced that she was having heart failure and that I screwed up her medications. Today seems to be going a bit better but reflux rears its head in the afternoons (so they say) so it's too early for me to say for certain how today is going.
.JPG)
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