Clarity's 5 Month Update

Whoa, I'm how old?!

Little Miss Clarity is exactly 5 months old today and she had her big visit to get an echocardiogram and visit the pulmonary clinic at Children's Hospital. The pulmonary clinic is kind of the CDH clinic where they address all of the questions we and the pediatrician come up with that are rather unique to her condition so we've been counting down the days. Our last visit was almost exactly 2 months ago, when they said we could take her off her oxygen.

The great news is that her echo looked great with almost no indication of pulmonary hypertension, big news to confirm that she's doing great off of oxygen. To officially get off oxygen at night (although she doesn't wear it very often anyway, although she's hooked up to a pulse oximeter all night to I can administer it as needed) we're going to have an overnight room air test soon. The bring a special pulse ox that records the data all night.

They also got an xray and did a couple of blood labs just to get some more current results for her, partially to track progress and partially to have a healthy "before" snapshot in case she gets sick this winter and we need to compare her lung situation to what is normal for her. Knock on wood that we make it through winter without getting sick. She got approved through insurance for the insanely expensive RSV shots from November through March and will get her flu shots. The girls and I live in fairly quiet self-imposed exile at the house so, fingers crossed. The longer we go in her life before she gets a respiratory virus, it will be that much less likely to require hospitalization. We're praying for 2-3 winters ideally.

The big question we were bringing to her appointment was feeding. 

• She miserably failed a gastric emptying test about a month ago and spits up A LOT. 
• Her stomach is basically always full to capacity as we strive to get enough calories in her, even with her breastmilk fortified to 26 calories (from 20), which is really as dense as they like to get or it puts too much strain on her intestines. She has been gaining weight but only at 3-4 oz. a week and I couldn't foresee any way to get more food in her as she's been getting bigger. 
• The gastric emptying test also showed that she's having severe reflux quite a ways up her esophagus, suggesting that her Nissen isn't really doing anything. This could be a good thing with her slow gastic emptying because she could have been in a lot more discomfort if her stomach isn't emptying into her intestines and a tight Nissen was preventing the excess food from coming up. That would have been a lot of pressure in that little tummy. 
• Some of you also know that she apparently retched enough last week that she got a tear in her esophagus, known as a Mallory Weiss tear, resulting in a trip to urgent care because she was spitting up dried blood and had a whole bunch coming out of her g-tube as well. It hasn't happened again and they put her on Prevacid to hopefully protect her esophagus by making her stomach contents less acidic.

Before we look into a more invasive option, like replacing her g-tube with a gj-tube to deliver some food directly to her small intestines, we've decided to try continuous feeds. She had been getting 3oz. over the course of about 50 minutes (rate of 110ml/hr) every 3 hours. She's now getting 30ml/hr for 20 hours a day with 4 hours off. If that goes well for a couple days, we're going to up it to 35ml/hr; about 23.5oz/day. I need to talk to the feeding therapist to figure out an oral feeding practice schedule with no defined "mealtimes". It's a step farther away from eating like a "normal" baby but if we can keep her growing and not constantly spitting up it's a pretty great trade off.

Oh, the joys of CDH. It's a birth defect that just keeps on giving in so many varied ways. We're awfully lucky though. She's clearly smart as a whip, always looking alertly around her and soaking it all in. Her therapists are also thrilled that she's pretty much meeting her developmental milestones, especially if you consider her adjusted age (born 1 month prematurely). We are so, so thankful. Everything else along the way is small stuff.

Day 58 NICU - Going home!

On Friday, July 26, Clarity finally came home at the age of 8 weeks, 2 days. She's still on 1/8 liter of O2 and wears a pulse oximeter. She gets 68ml of 22 calorie fortified breastmilk through her g-tube every 3 hours along with a dose of sildenafil every 8 hours. She'll be done with her ativan  and  methadone weans in a few days.

It's not like bringing a regular newborn home.  Between the constant alarms from her pulse ox and g-tube feedings that last one hour out of every three, we got almost no sleep on her first night home.  The second one went better after we tweaked the oxygen up a bit and she's keeping in her narrow allowed range (95-99%) more consistently. She seems to like 5/32 liter (which is not on the scale but 1/8 sats a little low and 3/16 ends up satting 100% a bit too much). Mom's going to start helping me out on Monday and I'll get to skip one feeding during the afternoon and get one consolidated sleep period (longer than 1 1/2 hours) each day.

Headed out of the NICU (Clarity is in my wagon, her feet visible covered by a blue blanket.

 The girls strapped in for their first ride together.

Clarity's first meal at home

Our first family meal

Cousin Cullan's birthday party at the park, the day after we got home.
Clarity's stroller is visible in the upper right.

Her first follow up visit to her regular pediatrician is on Monday morning. It will be interesting to see what they feel comfortable handling and what we'll need to follow up with the specialists at Children's through the years.

Day 50 NICU Yum!

Little Ms. Claire Bear has started working pretty hard learning how to breastfeed over the last two days. The occupational therapist was impressed that she managed to get a few good sucks and swallows on her first attempt yesterday. We repeated the feat today with more confidence.  She's doing a fantastic job. She's at full feeds now (20ml/hr) through the g-tube and just today they started condensing them so they are no longer continuous. She has been receiving 60ml over 2 1/2 hours today then having 1/2 hour off. Tomorrow it will go to 60ml over 2 hours with an hour of rest and so on until she's getting her whole 2 oz. feed every three hours over the course of 30-45 minutes. Soon, we'll be starting that time by putting her to breast and then putting however much more she needs through the tube afterwards. (We'll determine how much she ate at the breast by weighing her before and after.)

She had her first thorough sponge bath since birth a couple days ago. She seemed to enjoy it and even fell asleep as I was massaging her scalp to get some of the cradle cap off. The shampoo plus letting it partially dry before I brushed it has left her with a pretty crazy fluffy hairstyle.

 Since we stopped continuous feeds today, this was the first time we've removed the feeding extension from her button. Here she is with it all closed up. It and her oxygen cannula are the last things she needs. Through her g-tube she still receives methadone and atavan (continuing to wean on those), sildenafil (for her pulmonary hypertension), and vitamin D. The last IV came out yesterday and everything else is just for monitoring.

When she's being played with and talked to, she rewards nurses and family members with a huge grin. I still haven't managed to catch a photo of it. Needless to say though, it wins her lots of friends.

Day 44/45 NICU Post-op

Pre-op family photo

Immediately post-op

I was telling the nurse the other day that a tiny silver lining of Clarity's circumstances is that she is getting way more photos taken of her and much better documentation of her early life than many second born children.

We have been thrilled with how Clarity has done after her surgery. She came back up to her room for recovery and she was already back on her nasal cannula at that point. She's had a few doses of morphine but they're weaning her back off of that pretty quickly.

While she was under, they did a bronchoscopy to check on her lungs and suctioned out quite a bit of mucusy secretions but there was no sign of infection. It looks like the aspirant just mildly irritated her lungs and now the output they produced to deal with it has been cleared. Her lungs showed no signs of "floppiness", which suggests that the tissue is healthy and functioning properly. All of this was good news. Poor girl seems to have a bit of an irritated throat after all that though. She's been drooling a lot again as though she's avoiding swallowing and there has been a tiny amount of bloody tinge a couple of times.

They started tiny "trophic" feeds into her tube (1 ml/hr continuous) on the day after surgery and started increasing them by 1 ml/hr every 6 hours on Saturday at 12p. Assuming she continues tolerating them we'll be back up at full feeds by midweek and then they can start working on making them non-continuous.

I got to learn how to change the g-tube dressing on the day after surgery, easy but precise and careful work. The more carefully the site is protected from irritation, the less likely she is to have complications like granuloma. We'll have a more thorough class soon on how to care for it as one of our steps toward getting home. I'm still hoping we won't have to have it for very long after we get home but we'll see. Oral feeding trials will start as soon as she's feeling better from the surgery.

I got to hold her for a nice long time on Saturday and she spent a long time in the quiet alert state, taking it all in. I'm so looking forward to bringing her home. I'm not sure if feeding or O2 requirements will be our more time consuming factor for getting home. I think that once she's on full stomach feeds through the g-tube she can be released. I haven't been able to get a good handle on how close she is regarding O2. Apparently she can go home on 25-50cc but can't find a good way to translate between that and her current 40% at 1l flow. I think it will be easier to estimate when they put her back on the wall (100%) and decrease flow, since I believe we will then be working at the same scale that home O2 works. My fear of having her home with O2 and feeding needs like that is gradually getting overcome by a desire to just get out of the NICU to let her start leading a more normal life. As she's becoming more awake and starting to hit places for developmental milestones, I'm more and more struck by how hospital life, no matter how carefully done, can not compare to the healthy stimulation of home life. Let's hope that the g-tube was the answer and she'll rapidly continue to improve now.

Day 41 NICU - Photobomb and Getting Ready for Another Surgery

Look Mom, no IVs! And I now weigh 6 1/2 lbs.!

No IVs = ability to wear sleeves; not that her arms stay in anyway

Lower doses of methadone and atavan mean we get to see her awake a lot more.

                                           

"Auntie" Sarah (and husband Chris) visited from Wisconsin
over the 4th of July weekend. We're so lucky these dear friends
were able to come and stay.

So awake for Daddy

Grandpa got to hold Clarity for the first time on June 28.
Willow wanted to share his lap too.

After my last post at the beginning of last week, Clarity was continuing to have fast breathing and heart rate with increased need for respiratory support. Her blood work indicated she was low on hematocrit so she got a blood transfusion on Tuesday, July 2, followed by a dose of Lasix because her fluid output had decreased a little. This made her very happy; her heart rate and breathing decreased and her saturations have been holding more steady again.

Several days of chest x-rays, however, suggested that a little cloudy something in her right lung was likely aspirated reflux. She hasn't been spitting up but she's been refluxing high enough to be "silently" aspirating some of her feeds. The slight decrease in lung function on an already compromised respiratory system is causing her to need the additional O2 and it would only continue to get worse if we continued full feeds into her stomach and didn't fix it. The short term solution was to place her nasogastric (NG, nose to stomach) feeding tube farther down so it's delivering milk directly to the beginning of her small intestines.

GERD (gastroesophageal reflux disease) is extremely common in CDH kids, especially ones like Clarity who have their stomach in their chest at birth. The point where the top of her stomach meets the esophagus is too wide and allows stomach contents to escape. The fix is a Nissen fundoplication (described here). The surgery allows food to go down but nothing can come back up. Due to this side effect, babies always have a g-tube placed in conjunction with the Nissen so they can be "burped" by venting the tube. She will also start receiving her milk this way in the beginning as we continue the work of teaching her how to feed orally. 

Ideally, we will manage to get her to start taking all of her calories by mouth, the g-tube will be used only for venting, and it won't have to stay in very long. Fingers crossed. At this point though, the Nissen is necessary for her to continue getting better so if we have to continue feeding her through the tube for an extended period of time, it's a small price to pay. Many children, for a variety of reasons, have g-tubes for several years. If she's just got a g-tube and is on minimal oxygen (or none!) we'll be able to take her home - which is an event that I'm really kind of getting antsy for. Being patient is becoming more difficult as we've been stuck for the last couple of weeks with tiny steps backwards and no forward progress.

Surgery is theoretically scheduled for late this week. It's going to be harder this time to see her afterwards with another line, heavily sedated, and on a ventilator again for a few days now that we've seen her looking so "healthy" (everything is relative, right?) Oh well, she's a tough girl and she's already made it through one major surgery in her short life. Our job is just to support her and advocate for her best interest as she takes whatever journey she's meant to take. It's pretty amazing to think about her path already - she'll be 6 weeks old tomorrow (July 10)!

Poor Willow got her very first stomach bug on Sunday afternoon and spent all of Monday at home with Mama, sleeping and feeling lousy. She was truly pathetic but that didn't stop me from enjoying all of the cuddles from my usually super-energized girl.

Day 34 NICU

 There's not a lot to report but just wanted to say hi!  We really appreciate all the family and friends who are keeping up with our beloved girl's progress.

Clarity has not had any problems with her stomach since they pulled the tubes.  She's got a  nasogastric tube  that runs directly to her stomach now (instead of her intestines)  and she's  getting 6 ml/hour of milk currently,  increasing by 1 ml/hr  every 12  hours until we get to 16 or 17.  We got to try a little bottle feeding over the weekend but it seemed like it was really tiring her out and causing stress so we've pulled back.  She's just over 6lbs.  now, so we know she's getting what she needs.  So funny that she still  weighs les than our little Wil did when she was born (6lb 10oz).

 Yesterday  she started having some tachypnea (fast breathing)  and  minor tachycardia (fast heartrate)  so they're keeping a close eye on her today.  Her lungs are nice and clear (chest x-ray)  so they're just doing everything to let her rest.  We're skipping the methadone step down that was due today and that's also why we went back to continuous feeds.  Her O2  had been at .4l  from the 100%  at the wall and they put her back up to 1l  flow at 40%  on the wall to give her a little extra support as she figures this out.  If it isn't one thing it's another.

 We had our first visit from  physical therapy (PT) yesterday.  It was near to see how they work with these little ones to release the muscle tension that results from her extra work of breathing and lying in her bed so much.  We're going to also start having visits from  occupational  therapy (OT)  as we start bottle feeding (known as "nippling"  around here).

 All that said,  she's spending more  time awake and watching us.  She's also started this adorable smiling thing. I  don't care what people say,  little ones do smile when they're happy (unless she only gets gas when she's looking at one of us).  Five weeks old tomorrow!

Day 29 - The Horrible, Terrible, No Good, Very Bad Day (that turned out okay)

The day after they removed Clarity's orogastric tube, they reinserted it because she started throwing up. We've been having some frustration with continued output ever since, indicating that somehow her stomach isn't emptying properly/rapidly enough. However, the nasal feeding tube that goes to the beginning of her small intestine has been working well, so we knew Clarity's intestines are processing well. Thinking that the nasal tube through the stomach was kinked and causing problems, we took a trip down to radiology to have a new nasal tube placed on Tuesday. Output continued. On Wednesday, we again returned to radiology (a trip down three floors on the elevator that requires four people to take a 5+lb. baby and all her accouterments) to get an upper GI study done. This confirmed that she seems to have no physical reason (blockages, malrotation, etc.) for not allowing digestive juices to empty from the stomach. Frustration ensued because the other option is that she's not having good motility, a common problem with CDH. Dysmotility is much more difficult to treat.

All of this leads us to today. I was already worried due to the research I did on motility issues and really wanted to make rounds to see what was said. There was an accident on the highway route which made parking lots out of some of Denver's main arteries. I ended up getting to the hospital late and missing rounds - first frustration/stressor.

When I got to the hospital I found out that her OG output has increased substantially and clearly has a lot of milk in it, meaning that her feeds are now backing up into her stomach from the small intestine. This is a huge step backwards and was now calling into question how efficiently her whole GI tract is working, not just her stomach.

Around 10a she started being extremely irritable, crying inconsolably before finally falling into exhausted sleep and then waking up 15 minutes later, sobbing again. Finally, around 11:30a, I picked her up and cradled her to my chest at the magic moment and she immediately fell asleep. The nurse moved a chair over to me and I carefully sat down and got all the tubes arranged. Clarity, blessedly, finally took a 1 1/2 hour nap.

When she woke up, we started cares and found that even after several hours, her diaper was still totally dry. No urine output is another worrisome sign that something is amiss. She continued to cry and every time she almost calmed down it would start again. A baby with compromised pulmonary function and hooked up to monitors getting really mad is something that gets kind of scary because you can see her heart rate soar and her oxygen saturation can plummet. This also brings all sorts of alarms, adding to the cacophony of a crying baby, who can't be easily maneuvered for soothing because she's hooked to way too many wires and tubes.

At this point, the nurse had the doctor come in to look her over and I went over to the armchair and quietly had my nervous breakdown. It felt like all of a sudden my relatively stable baby was barreling downhill into some sort of crisis and nobody knew what was happening or how to help her. It took a lot of deep breathing to pull myself together with only a little crying so I could try to actively participate in her care. Being her advocate is all I can really do for her sometimes. Even though they had been replacing fluids intravenously, the doctor ordered some more since no urine output pointed toward dehydration. Since the feed back up had started shortly after replacing the NG, she also ordered an abdominal x-ray and the technician got there ASAP.

The picture came back with a brilliantly obvious answer. She was constipated by the barium solution they had given her for the GI study the day before and her entire lower bowels were glowing with radioactive poo. Taking away her feeds for the study had also led to slight dehydration, which compounded the issue. I was so relieved I became practically giddy. Yes, she was still crying in pain and hard to soothe but we had an answer and it was a "normal" baby problem. The doctor ordered a glycerin suppository and I went back to trying to help Clarity feel better while we waited for nature to take its course. She quickly became the anecdote of the day among the staff. Happy outcomes are always celebrated in the NICU.

As I was putting her back in the cot to change her dirty (yay!) diaper, we managed to pull out her NG - the one that we had had to go to radiology to replace only two days ago. At this point, almost at the end of a long day of misadventures, the nurse and I just had to laugh in resignation. Clarity had drooled so much that she had managed to soak off the tape holding her NG, OG and cannula in place. I held them steady until our nurse could retape them. The timing couldn't have been worse though because it was about 5 minutes before her (oral) methadone dose was due. The dose that we could only deliver through the NG tube that was just pulled. She been successfully weaning down so she only gets them every 12 hours now. Within the next 20 minutes, I was now trying to comfort a baby who was still extremely constipated and starting to suffer withdrawal. Thankfully, within about an hour they got approval to give her a dose through her IV. That was quickly followed by her atavan dose that was due. By 5p, we had a very loopy drugged baby girl who already had one dirty diaper. Oh, the relief.

I had to leave but our day nurse called when she got word back from Dr. Crombleholme (our surgeon and head of the maternal-fetal care center) about what he wanted to do. Clarity had her OG removed and he kept her NG out for the night. She's getting all of her meds and nutrition through her IV and we're going to see whether the tubes have been irritating her and causing the problems. Hopefully, she won't start throwing up stomach juices in a few hours and they'll just drain properly into her intestines without all the tubes interfering. I'm trying not to even dare to hope that we will finally solve the stomach drainage mystery with this experiment. No matter what, I'm just so grateful that she is stable and not in any sort of immediate danger.

The good news, in a day of so much regression, is that her sildinafil is now at full dose so they're planning on turning off her NO tomorrow and moving her oxygen to the wall on low flow. This is all proof that her respiratory system is totally kicking CDH booty.

Here's a picture of Clarity at the end of the day, slightly cross-eyed from a combination of medication and sleeplessness. Here's hoping that the night nurse has a boring shift. :)

Day 26 NICU

Okay, we're going to build up to the most exciting news of the day at the end of the post. It was a pretty exciting day all around.  First, above, Willow came to visit with Grandma and went to the Play Center for a couple of hours.  It is awesome free childcare for siblings of patients.  She loves it there.  Today, she painted a wooden duck and created a picture using those precut foam pieces.  When we got back to Clarity's room she proceeded to pull them all off the sheet and adorn herself instead. She was so proud of the results.

 While  Wil was downstairs playing, Grandma got  to hold Clarity for the first time. Both ladies seem to approve of each other.  

 Clarity wore her first actual outfit today. I  couldn't resist this in the thrift store a few months ago.  She still has her PICC  line and she's tiny so we didn't bother with the arms but she's still cute as a bug. I caught the picture while she was yawning too!

Okay, last,  most exciting  news.  Look at her right cheek in this picture and the one above.  We lost a tube between the two shots!  They pulled her orogastric tube today.  It had been suctioning stomach contents.  Even though she was still having more output than they'd like,  it was clearly just stomach bile and no food was coming up.  They decided that the extra tube might be contributing to the problem so they pulled it.  We really hope she doesn't start spitting up now and they decide to reinsert it. They also started increasing her feeds again so hopefully we'll get to taking milk as her only nourishment soon through her nose tube.  When that happens we can lose her last line and she'll have her other hand free. Her mouth is finally completely empty - at least until she started happily sticking her free hand in there.  :-)  It's just the sweetest,  most kissable little mouth.  She also looks even more like Wil now.  So in love with this little girl.

Day 22 NICU

Our girl is spending more and more time awake and quietly alert. I really enjoy sitting next to her and having "conversations" when she's like this. She continues to be a champion bubble blower, as you can see from the picture. The volume of her "oral secretions" continue to amaze each new nurse who has her.

She was showing signs of withdrawal overnight so they upped her methadone dose. As the nurse put it, there's no need for her to go through withdrawal symptoms and they can eventually just gradually decrease the methadone in intervals that don't stress her body. Her job right now is to get big and strong and grow a lung. She kept falling asleep today and then waking up 1/2 hour later, crying, especially when she was due for a dose in the near future. Poor baby.

The doctors are currently a bit baffled by the volume coming from her OG tube. That's the tube that drains her stomach and they expect it, by this time after surgery, to be producing just a couple of clear mls every few hours. Unfortunately, it's producing more like 15-25ml and it's rather green, which suggests bile. Ideally, the bile should be draining into her intestines and not collecting in her stomach. What is keeping them from being worried is that they are putting lots of milk through her NG tube, which goes directly to the beginning of her intestines and all of it is clearly getting processed and going through the entire intestinal tract. This means that any sort of bowel malrotation (a CDH fear) is extremely unlikely.

If everything past the stomach appears to be in working order, the question becomes, why isn't the stomach draining? The NG tube can apparently block good flow through the pylorus (valve at the base of the stomach), preventing drainage but we can't pull it back into the stomach until the OG tube can be pulled. We can't pull the OG until the stomach is draining it's contents through the pylorus.  Starting to see the paradox?

I'm going to manage to get there early enough for rounds tomorrow to listen to what's been decided. They were consulting her surgeon, Dr. Crombleholme, today for his opinion. They may wait a couple more days to see if it decreases on its own or they may embark on some sort of diagnostic course. I hope it gets resolved soon. It's clear that she is even more anxious than I am to get it out. She mouths around it and tries to spit it out. I think it will also go a long way toward solving her drooling problem and she'll be more able to start working on using her pacifier (which she already likes).

Three Weeks Old

Clarity and I celebrated her three week birthday by having a major snuggle-fest. Skin to skin snuggles is usually called "kangaroo care" and is extremely important for the health of NICU babies and an important step in establishing breastfeeding. A quick informative article is here. She's down to enough tubes that she's easy to transfer and manipulate into different holding positions. I'll be able to lift her easily by myself after the OG tube comes out of her mouth.

They turned off her versed yesterday afternoon and turning off the fentanyl sometime early this morning. She's now strictly using the oral atavan and methadone, which they will slowly and carefully wean her down from to avoid withdrawal. No more meds going in intravenously, just nutrition, which means she will get her last PICC line out whenever she gets to full NG feeds of milk (through her nose). I can't imagine how thrilled she'll be to have both hands up by her mouth.

Yesterday when she was in her crib, I tried teasing her lower lip with the pacifier and she opened up (one reflex for nursing still intact) and even sucked on it a little (two nursing reflexes intact). She had trouble getting the hang of sucking and keeping it in her mouth but she's also working around that silly OG tube.

At this rate, I'm hoping for no more OG tube and no more PICC line by early next week, maybe sooner. Then we'll really be able to focus on learning how to breastfeed.

Day 20 NICU - Extubation!

This was a monumental day for our newest little girl! She got extubated at about 10 this morning (only 6 days after surgery!) and did a wonderful job starting to breath with only a nasal cannula. It all happened so fast. They told me at rounds that it was going to happen today and then, all at once, a couple of hours later, two doctors and the respiratory therapist trooped into the room and just did it. It was amazing hearing her cry for the first time, almost as intense as if I'd gotten to hear her in the delivery room. It is still so soft and is mostly raspy hissing with only a few little vocal mews. Once her throat is less irritated she'll get a hearty cry like other newborns. I have promised myself that I will just think about the nice strong lungs producing that sound and remember to be grateful when it is no longer a novelty. I am so in love with that little mouth.

She's doing all the work of breathing now; the cannula just provides some extra oxygen and the NO which helps the pulmonary hypertension. They'll continue to monitor her blood oxygen levels and slowly wean the levels until, eventually, she'll just be breathing room air.

Shortly after extubation

 After they did a couple more draws to check her blood gases, the also decided to remove the PAL (peripheral arterial line) from her left arm this afternoon. Notice how she no longer has the armboard in the top picture - and looks a lot more comfortable. We're down to only the cannula, the right arm PICC line, orogastric tube (for clearing her stomach, should come out pretty soon), and nasogastric tube (for feeding her). Everything else is just sensors for monitoring her stats. AWESOME! Holding her is going to be so much easier now and I plan on doing it as much as possible without stressing her out.

We have come to understand that our girl is rather sensitive to change. In order to pull the vent today, they also had to cut the fentanyl down another bit (after an additional versed wean about 2 hours previously) because it's a respiratory inhibitor along with serving roles in sedation and pain management. The combination of so many changes made her furious. She cried (raspy, hissing sound due to irritation from the tube) for a little over two hours, reawakening herself every time she started to calm down and doze off. I hated feeling so helpless. I tried everything I could without being able to pick her up. I'm not sure how long it would have lasted if they hadn't finally gotten her a dose of Atavan (sedative without inhibiting respiration). They were rather surprised at how long she stayed mad and by the time it became obvious that she wasn't calming on her own, the process of getting the order in and having the pharmacy send it up took quite a bit of time. She also started getting some methadone today to help with the fentanyl wean. Hopefully between the two, her sedation wean will be eased a bit. I find it kind of funny to have a daughter in "rehab", using methadone to wean her from her narcotic dependency. Might have to tease her about that when she's much much older, say about thirty and a productive member of society. :)

The crying fit was even more nerve wracking to watch because her heart rate already seems to run a little high (they're attributing it to just being a little irritable with all the stuff she has to deal with - sensitive, like I said) and when she gets really mad it starts soaring, occasionally breaking 200. "Normal" for a newborn is 120-160 and her usual awake but calm rate right now seems to be hovering around the 160s to 170s.

Her feeds are steadily being increased by 1ml/hr every 8 hours. She's currently at 6ml/hr and is filling plenty of diapers as testament to the efficient functioning of her intestines. Another yay! I'm really looking forward to getting to feed her through more traditional means. It's funny how her great leaping bounds of progress are making me more impatient for what is to come.

As of tonight, her weight is now 5lb. 13oz. She's gained just over a pound from her birth weight of 4lb. 11oz.

Q and his parents went to visit her this evening and she's apparently alert and looking around. Grandma Jill even got to hold her. Tonight is also the first time that she has had to share a nurse with another baby for a shift, further proof that she is doing amazingly well. All day I have had an overwhelming feeling of gratitude for how well she is doing so far. Our path has been smoother than I dared to dream in the months of pregnancy after we got the diagnosis. May she continue on this road!

Day 17 NICU - 1st chance to hold her

We each held her for about 45 minutes. Her stats were great the entire time. It was better than I thought it would be. I'd forgotten how satisfying the limp weight of a sleeping baby is.

Behind the scenes - what it took to get her from her bed to a lap

She's lost a lot of accouterments. She's down to only one tree of meds (above, to her right). The pump sitting alone to her left is the milk for her NG tube. They removed the PICC line from her leg and her catheter so all she has on her lower half now is the pulse oximeter and her ID tag. The space around her crib is so spacious. For comparison, the picture below was taken on her first day of life.

Day 16 NICU

 The infection scare seems to have been a  false alarm.  All  cultures are still negative.  She's showing the elevated temperature and tachycardia again today but we're starting to believe that those are more symptoms of discomfort for her.  They cut her fentanyl (pain  med) in half yesterday  but have been administering fairly regular boluses since then because she keeps getting restless.  Amusingly,  what really seems to work is not the scary meds  but  Tylenol.  When they give her a dose of that she almost immediately goes into the good ranges.  Hopefully we can start controlling the pain with that (which can be given  every 6 hours)  rather than the versed,  fentanyl and atavan boluses.  Of  course,  what we're really hoping for is that in the near future she'll just start feeling better and have less  post-surgical pain.

One of the reasons they lowered the doses on the meds was to wake her up a little so she would start breathing on her own and not letting the ventilator do all the work.  She's at a slightly larger volume now and the vent its set to 32  breaths/min,  down from 42 yesterday. She's now going through spurts of doing a whole lot of breathing over the vent and then letting it do all the work for awhile.  It seems like she gets worn out and just needs a rest sometimes.  At some point they're going to start weaning the NO (necessary to relieve the pulmonary hypertension)  and getting that dose down,  combined with her regularly doing enough breaths  on her own, will be our ticket to getting off the vent.

 The echo looked good so they stopped the milrinone.  With that change,  she's now just on the  fentanyl  and  versed  plus some glucose, TPN (food  replacement),  and lipids through ivs.

She started feeds today. During surgery they placed a transpyloric nasogastric tube, which means it runs into her nose and down past her stomach  to the beginning of the intestines.  This allows her to start digesting colostrum (and eventually milk)  while bypassing her stomach,  which avoids the gastric reflux that's very common with these kids.  She's currently receiving 1ml/hr on a continuous drip,  just to start coating her intestines and waking them up.  This will gradually be increased to full  feeds (150ml/kg/day) before they start moving  the tube up into her stomach.

Two weeks old

Baby girl had a quiet day,  just recovering from surgery and under heavy sedation.  They took her off the paralytic in the morning but,  for the most part,  the vent has still been breathing for her.  When she's more awake she does start taking breaths so we'll see how that goes when  her post surgical pain is decreased and we can let her be more alert.  She has been put down as low as 30% O2.

All day, her temperature was running at  high normal.  Around dinner time it hit the high (38 Celsius)  where they do tests for infection. I  got nervous and when  Mom offered to come stay with  Wil, I  jumped at the chance to go back to the hospital.  When we got back,  they had given her Tylenol for the fever and her temp and heart rate were much better.  The complete blood count, urine culture, and procalcitonin all came back without giving indication that she has an infection.  With those positive preliminary results,  we're assuming the blood cultures will likely come back negative.  The origin of the slight fever is remaining a mystery.  She tends to run warm so it's possibly just her reaction to the surgery.

 We've got an x-ray and an echocardiogram scheduled for Thursday so we'll get a peek at how everything is adjusting to its new placement.

Surgery! Day 13 NICU

 

 

 

 

 

 

 

 

 

 

 

 

On the morning of surgery

The day finally came today. Everything looked good and this was our window to get the repair done. They did the whole repair in her room. The NICU rooms are designed to have everything needed so they can be used as ORs and a huge surgical team descended on it, draped everything for sterility, performed the surgery, then removed everything. The repair was scheduled for 10a but a previous procedure ran over so they didn't begin until about 10:45a. We got the call that they were all done and just closing her up at 12p. It went perfectly.

The hole was large enough that they did end up having to do a patch. Dr. Crombleholme has had a remarkable amount of success (success = no reherniation) using a flap of the innermost layer of her own abdominal muscle. The way it was described to us is that they literally just fold it up into the hole in the diaphragm and stitch it in place. When he talked to us after the surgery, he confirmed that there was just a tiny bit of liver up, her stomach, and most of her intestines. It all barely fit into her belly so they're keeping her on paralytic muscle relaxers for the next little while to keep her abdominal muscles loose as her tummy gets used to accomodating everything that's supposed to be in there.

We were not prepared for the total change in her shape. It's a wonderful change but we'd never really noticed how concave her abdomen was until we got to see her with a big round tummy.

 

In the picture above, taken just an hour or so after they finished closing her up, you can see her big tummy. The incision is on her left at the level of her belly button. With the way they do the dissolving stitches, it's likely that the scar will be very faint by the time she grows up. She's doing a great job tonight and has come through like a trooper.

The post surgery x-ray doesn't have the radiology report attached yet but our nurse looked at it and it looks to her like there's already quite a bit of left lung that has expanded since it was given room. They're also hearing a lot more breath sounds on the left side. We're anxious to get more reports tomorrow.

The anxiety of today left me totally exhausted when I came down off my nervous high. I hope we sleep well tonight. We got a sleep room at the hospital and Mom is staying with Willow because we knew we wanted to be extra close to Clarity tonight. We're so relieved that's over and now it's on to the hurdles of recovery over the next few weeks before we take her home!

Day 10 & 11 NICU

Terrible 1st picture of the entire family
at one of the rare moments that her eyes were open

Willow visited the hospital on Saturday for the first time since Clarity was born.  It was a short visit but she liked seeing the baby, although she was frustrated that she couldn't kiss or touch her. We also got visits from my mom, my brother's family and Q's cousin's family. It was fun showing off our girl.

On Saturday, Clarity was continuing to wean on the ventilator settings and dopamine and epinephrine amounts. In late morning, they had to do the weekly dressing changing over her lines and she was extremely unhappy about it. It took the rest of the day for her vitals to return more to where we want them.

Overnight, she continued to seem agitated (grimacing, sucking her tube, wiggling her hands and legs) and she ended up getting several extra doses of Versed and fentanyl, along with a little Atavan. By the time rounds happened this morning, the biggest question for the nurse was what we were going to do to continue trying to calm her down. She got another bolus of Versed/fentanyl at 8:15a and then, somehow, she managed to finally get calmed down to the point that she didn't need any additional meds beyond her usual drips. When we came back from lunch, her vitals were good. She was awake, opening her eyes and looking around a little. She moved a little but not with the restlessness she had exhibited earlier and then she closed her eyes and went back to sleep - without any medical assistance. Since we wanted to spend the afternoon at home with Willow, that was a very reassuring way to leave her for the day.

Her blood pressure is hanging out just above where they really want her but the dopamine and epinephrine are for keeping her blood pressure higher so it should be going down as we continue to wean her on those. The dopamine was at 4 this afternoon. I still can't believe that it was at 12 just a few days ago.

Today, she also had a blood transfusion due to a low hematocrit count. Apparently, with as frequently as they have to take little blood samples, it's pretty common problem for these little ones. We assume she's also going to need more transfusions due to the surgery, so it's nice to get through the first one in a low stress situation.

Clarity is scheduled for another echocardiogram on Monday morning and doctors are saying that if it looks like they expect it to, they're planning on scheduling her repair surgery for either Tuesday or Wednesday. Crossing our fingers that she'll just keep doing so well!

Grandma has managed to start catching Willow for ponytails.
She looks like such a big girl!

Day 9 NICU

Baby Foot

It's kind of misleading to use Quentin's thumb for measurement because he dwarfs lots of people. I have tiny hands though and her entire arm, shoulder to fingertips, is the length of my hand, palm heel to middle finger tip.

1 day old

9 days old

I think her face is already filling out.

Clarity continued to improve on Friday. She was doing an amazing job of tolerating all of the bother of cares (nose and mouth suction, diaper change, sensor checks and movement) with only a bit of raised blood pressure and heart rate, both of which quickly recovered to normal rates. 

At the end of the day, she was also weaned down to 33% oxygen. For perspective, her high was about 45% (could have been closer to 50% at the very beginning) and room air is 21%. Not bad for a girl with only one lung's worth of volume.

By early evening, her dopamine had been weaned down to 7 (after a high of 12) and her epinephrine was at .03 (after a high of .09). These were helping her maintain her blood pressure but she's been tolerating the weans very well. The night nurse was going to monitor her closely but potentially wean the dopamine down to 5 by her end of shift this morning.

Her calcium chloride drip was cut in half yesterday. Apparently, a person's body uses up the free calcium in their blood more readily when under stress, so they were supplementing when her blood tests came back deficient. Her levels have now been holding steady so they'll be watching her blood tests over the next 48 hours to see if they are still within the appropriate range with less supplementation.

At the very end of the day, she got another CRP, which was very close to her previous number. Since it's not a direct indicator of infection but of inflammation, the new result that has stayed fairly constant, combined with the clean bacterial cultures, suggests that the elevated number is a result of her condition and current state. They were planning on stopping the prophylactic antibiotics last night.

We know there can easily be setbacks but are awfully proud of our little girl for keeping up the positive trend. We're heading in the right direction. They're still talking surgery for sometime next week. We'll see, based on how she keeps doing, if they end up deciding early or late in the week. The idea of surgery is getting scarier as it gets closer, so I'm willing to let them take their time. :)

A big girl with important work at Grandma and Grandpa's
In true toddler fashion, she was on a mission, clear only in her own mind.

Day 8 NICU

 Last  night's scare seems to have been short lived.  Clarity was showing no more signs of infection today. If something was starting the prophylactic antibiotics seem to have nipped it in the bud.  We're waiting  for her cultures to come back to see if anything grew. All her stats were a bit better.  Her blood pressure has still been hanging out a bit high so they got her dopamine weaned to her baseline and they started weaning down her epinephrine.  I'm guessing that by this time we go in tomorrow that will be weaned to baseline.  I  don't know how those baselines were set and whether they'll keep decreasing the amounts if her bp  tolerates it;  they may be using their properties for something besides controlling bp.

 They also weaned her back down on some O2 today.  She was at about 45%  and now she's down at around 40%.  This is not the first time we've tried this but the change happened midday and by the time we left her blood gases still looked good and saturation was very high. The one frustrating thing about her ventilation is that on the high frequency oscillating vent they don't want her breathing on her own, "fighting"  what it's doing for her.  She keeps putting in a bunch of breaths of her own,  which is counter productive and increases the likelihood of getting air in her bowel as she swallows it.  Feels kind of funny,   hoping that your child will stop breathing.

Fentanyl is the big thing we haven't been weaning.  In fact, she's been getting extra here and there when she gets wiggly and tries to cry.  These fits tend to make her heart rate go way too high and more fentanyl brings her back pretty quickly.  She's clearly having some sort of discomfort/distress at those times but we can't figure out what.  I'm suspecting that until she can get weaned down on the fentanyl to about half of what she's on they won't want to do surgery because their additional pain relief options will be limited.  I'm not in any hurry.  If she can get rock steady before surgery she'll be that much more likely to pull through without trouble.

 She looked so good tonight that it wasn't even stressful to leave her in the care of the nurses while we came home to sleep and spend time with sweet  Willow.  After we left, Q's  parents went by for a visit so it was nice getting a mid-evening report that she was doing well.

 In the sad news of the day, a CDH boy whose story I've been following online  and who
was born about 24  hours before Clarity,  at almost the exact same gestation, passed away today.  He was having some sort of electrical heart problems they hadn't expected that were causing arrhythmias and those challenges plus the CDH  were too much for him. It was a pretty shocking blow for me and sent my anxiety over baby girl through the ceiling.  They're just so fragile. Thankfully the other little one, a girl, who was born a couple days after Clarity is doing well.  My thoughts just keep going back to that little boy's family, hoping they're finding some comfort in their grief to get them through tonight.

 Tomorrow's another day of sitting and watching out beautiful girl,  trying to send her strength and love.  We're over a  week in without any major surprises.  Let's just keep our fingers crossed that it will stay that way.

One week old

Today started off well and we're pretty excited that she's made it one week.  Unfortunately,  her temp has been running just a little bit high, her blood pressure
 is a bit high,  and her heart rate kept going a little too high.  Late this afternoon,  the doctor ordered a CBC (complete  blood  count) to check her white blood cells (came back  normal) and a CRP (C-reactive protein),  which came back elevated.  CRP is a  marker for inflammation  and can indicate infection. It is high enough that they reacted  but not super high so if there is an infection,  they likely caught it early.  They started her on  two wide spectrum antibiotics  and took more samples to start some cultures for specific bacteria.  Preliminary results will come back in about 24 hours. They are also going to rerun the CRP tomorrow at  some time to see which way the numbers are trending.

 We tried to go home tonight.  It was wonderful to see Willow  before bed and get some snuggles.  We really tried to convince ourselves to stay home but just knew we'd feel better at the hospital.  Mom is so wonderful to stay with our big girl.  When we got to the last highway entrance,  traffic just stopped.  The last mile and a half to the hospital took  an  hour,  not good when our stress levels are already high.  We were unimpressed with their traffic  management choices for this road work because we got no warning until it was too late to take a different route.

 Keep the positive thoughts coming.  We knew there would be bumps in the road but we just keep holding on to our picture of the destination -  two healthy girls safe at home.

I  didn't happen to get pictures of Clarity today so here's are pictures of my beautiful big girl,  courtesy of Grandma.

I think she looks like a  Renaissance painting (until  you see the bulldog  jammies).