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.

Harvest is Done (followed by a bunch of pictures of the girls because they're awesome)

Today was the first light snow and tonight is the first forecast hard freeze so I rescued the last of the garden. It seems like a fitting time to post the final pantry pictures.

 We got a nice little onion harvest that I think will get us close to through spring. I let them cure on a grate in the garage for a couple of weeks before storing them in one of Grandma's delightful old egg baskets, which Mom was kind enough to loan.

 The pumpkins (6) and jumbo pink banana squash (3) - one of each has already been eaten. The squash pictured are each almost exactly Clarity's size right now, just over 10lbs. and about 21" long.

 I have mentioned before how much I adore our pantry, right? Here's a picture of the food wall, all ready for winter (or a zombie apocalypse). Admittedly, some of the stuff is carryover from last year (if it's a good peach year, you can lots of peaches) but it was significantly added to this season (as you can see from the previous post).

Mom was kind enough to give me some Concord grape juice from her vines and some rhubarb. Raspberries were on a really good sale at the grocery store. 5 pints of raspberry jam, 5 pints of grape jelly, and 3 pints of Rhubarb Vanilla with Earl Gray jam. (The last, once again from Food in Jars, and extremely good.)

We got 4 watermelons out of the garden and they are very good.

 Matching hats

 Willow "ironing" after she watched Mama doing it. She also loved having me iron her doll blanket and getting it back all snuggly warm.

 Tomato crushing and eating with some of the last harvest.

 Eating hazelnut butter and jelly at Children's after Clarity's gastric emptying test. (She failed; stuff for a different post once we decide what needs to be done.)

 Looking at a river in Silverthorne on the way back down from a weekend in Beaver Creek.

Garden on August 14

The sumac/grass forest at the right makes it hard to differentiate the end of the garden. Unfortunately, that's my dream project this week.

Wednesday's harvest - cherry and Roma tomatoes, cucumbers, green peppers and two anchos.

I used the anchos (plus one picked previously) for a first attempt at chile rellenos using this recipe.

They were good although anchos have a bit of kick. I think I'll search harder for anaheims next year and not just trust a tag that says they're good for rellenos. I've still got about 6 anchos on the plant so I'll get at least one more practice session this season.

First eggplant, although the plants are large, healthy and covered in blossoms

I've never gotten broccoli to produce more than a sprig before. I'm not sure when to harvest.

An online article suggested that ripe jalapenos will have corking, which sounds like stretchmark-like blemishes. We'll see. I've never grown them before either.

  About 4 or 5 softball sized watermelons are hiding among the vines

Two cantaloupe like melons, from an Adaptive Seed Company package entitled "Melons", specially chosen for cool climates

We have about 4 pumpkins.

 

There are 2 or 3 banana pink jumbo squash.

 

I had to include a picture of Monday's labor - 4 more quarts of pickles and 3 pints of marinara

Willow keeping baby's seat warm.

Clarity "eating" her breakfast

Odds and Ends

Yep, thanks to the fact mom comes down each day, I had time to make 4 quarts of pickles with cucumbers from the garden today. There should be enough more ready in a couple of days to make another few jars. We absolutely loved the pickles from the recipe I used last time so we're having them again. I can't believe that was four years ago. This time I think we'll end up with fewer jars and more people to share them with so maybe I can start making pickles yearly. I really like doing it. It's so easy and assembling the jars always feels kind of artistic. One of the many benefits of now living close to family is being able to can as much as I want and know I can probably fob the results off on people as gifts, especially since I despise feeding the consumerism that runs rampant around the holidays.

I'm feeling very foolish that I didn't think to plant dill. Next year, next year...

So, having a toddler is awesome. Everybody must get tired of hearing that, but it is. I love her earnest concentration and the way her diapered butt wiggles as she purposefully dashes toward her next job. She sets and clears the table. She shells peas and shucks corn. She puts dirty clothes in the laundry basket and helps "fold" clean clothes (also known as piling them in a heap, topped off by an emphatic pat).  Clarity has diapers and pacifiers fetched for her and gets copious big sister kisses, hugs and pats. She intensely watches and attempts to learn how to do every job and feeling like she's making meaningful contributions is the highlight of her day.

Her play is just as serious. For the last couple of days, she will spend a solid half hour at a time silently absorbed in scribbling with crayons on paper. She sits with her legs straight out in front of her with the phone book on her lap and "reads" to herself until I come to check on her because it's gotten so quiet. Today, she finally started being able to string her beads onto their lace with no help but until now she has spent the last week transferring them from cup to bag to box to couch cushion to cup to...

The best part is the way she will just look up at you in the middle of a task with a huge grin and laughing eyes. "Isn't this the greatest thing ever?"

Once more, toddlers are so incredibly cool.

This weekend was Quentin's grandma's 90th birthday and was the first time all the cousins were together. Q's got pictures of the rest of the family to post. I just took pictures of the kiddos. Cousin Cooper is only 7 weeks older than Clarity but she's so tiny compared to him!

And, finally, Ms. C just keeps clicking along. She only gained 2 oz. in the last week instead of the expected 8-16 oz. so we'll have to talk about that with the doctor on Friday. I'm guessing we'll probably put her on to 24 cal. milk (currently, it's supplemented up to 22 cal/oz) and maybe look at increasing the volume a little through the use of a couple of larger continuous night time feeds. She's doing wonderfully on her oxygen though and gets occupational therapy twice a week. We're working really hard on feeding and I feel like we're slowly gaining a little ground.

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.