A New Accessory

We had another busy week with at least one appointment every day and we even have a home visit tomorrow (Saturday).

This is how Claire was feeling about spending
another day at the doctor today ;)

Our biggest concern at this point is most definitely Claire's heart. She has lost 7oz since last week and is now down to 8lb 4oz (0.46th percentile) which is officially considered "off the chart." Her heart is working extra hard and she is also working hard to breathe which means that she is burning extra calories but it's also making her too tired to eat well. Because of all of this, she had an NG tube put in today so that we can supplement her feedings with fortified breast milk. She HATED it and has been gagging on it a lot today but hopefully she gets more used to it. They showed me how to put it back in if she pulls it out, I'm really hoping that doesn't happen anytime soon ;). The tube should also help with her aspiration and her stooling since we will not have to add thickener to the breast milk that we put through the tube. Now with all of her feeding supplies/syringes, medicines, and our supplies for her bowel and breathing management, our living room is starting to look like a medical supply store -- and I'm on the hunt for a new purse/diaper/on-the-go bag to fit all of the things that we need for our appointments!

She had her first fever (101.7) yesterday and gave us quite the scare but she didn't have any other new symptoms and her vitals were all fine so we aren't sure what that was all about.

Since we were already up at the health center today for another appointment, Claire's surgeon brought us in for an x-ray and an impromptu visit because she is still having lots of tummy issues - the thickened breast milk (for aspiration) is causing her to become pretty backed up so they've increased her irrigations (and dilations as these will hopefully help stimulate her to go as well) again, hopefully this helps get things moving! Constipation (especially since she just had a fever) is very scary for kids with Hirschsprung's because they can get enterocolitis very easily.  She has increased us back up to weekly visits with her until we can get Claire doing a little better with her stooling.

From here, the plan is just to focus on getting Claire as big as possible and getting as close to 6 months as we can so that her body will be as prepared as it can be for heart surgery. There is no telling how long her heart can stay like this so we might make it and we might not, we are watching her very closely. Her cardiologist called today to move up her next "heart check-up" from 1 month to 2 weeks since she is having such a hard time breathing (this is still terrifying to me), eating and maintaining her weight, this could be an indicator that we might need to do something sooner than we had hoped.

Her pediatrician will see her once per week to check her oxygen levels and I can take her to see him directly anytime I feel like she might be having any worsening symptoms - we did this yesterday with her fever.  I walked in and told them her name and they knew who we were and got us right back to see him, we didn't even have to wait. I love that we have this option of reassurance but it's also scary to know that she has needed and could possibly need that kind of fast/emergent care in the very near future.

We also have to be very careful to keep her healthy as even a cold at this point could cause her to become very ill and require hospitalization. Our doctor has applied to our insurance for extra vaccinations and we have been instructed to keep her "cocooned" until she is fully recovered from her heart surgery. We are quickly (and reluctantly) becoming "germ freaks" and "homebodies" but it will all be worth it to keep her healthy and so that she can go into her surgery as healthy and as strong as possible.

We are back to feeding her every 3 hours day/night until she starts to gain weight so I've got my alarm set to make sure that she gets all of her calories in!  Night <3.

Have a happy weekend friends!!
Thanks for praying for me.
<3 Claire

2 Months and More Answers

As of yesterday, Claire is now TWO months old!  She is 8lb 11oz (2nd percentile) and 20.5" long (also 2nd percentile). She is sleeping through the night [most nights] and smiling more and more when she's awake. She loves tummy time, being held, her siblings, and making silly faces. Here are her two month pictures:

Yesterday morning we worked on insurance/billing things and tried (for 6 hours!) to get Claire to drink the fortified breast milk, finally after 6 hours of her not eating, crying non-stop and me still in my pajamas, I gave her regular breast milk - getting the calories IN her is the most important after all.  Her GI doc called with the results of her Upper GI series, as soon as I heard his voice I knew he didn't have good news. Claire has both tracheomalacia and an artery that is compressing her esophagus (aberrant right subclavian artery). He has sent her imaging to a cardio-thoracic surgeon for a consult, he will review the images and will let us know whether or not it is surgical. He may also request a CT so that we can get better images. If he decides that it isn't surgical, we will just be keeping a close eye on it.

tracheomalacia: 

occurs when the cartilage in the windpipe (trachea) has not developed properly. 

Instead of being rigid, the walls of the trachea are floppy. 

Because the windpipe is the main airway, breathing difficulties 

begin soon after birth.

aberrant right subclavian artery: 

a rare anatomical variant of the origin of the right or left subclavian artery.

Finally, we ended the day at the cardiologist. Throughout the trouble with Claire's breathing and her growing reluctance to eat, we have called her cardiologist a couple of times and he has felt like her heart wasn't contributing but we're running out of things to try and before we start performing surgical procedures, Claire's pulmonologist and GI doc felt like it was worth it to go in and have her heart checked just to be sure. 

I went in thinking that we were just going to do a quick echo so that we could check it off our list and move on to other things. I was not at all prepared for the news that we received, Claire is in the beginning stages of congestive heart failure. Her heart itself is getting larger, the pressure within her heart is too great, and because of all of this, her lungs are filling with fluid. We have started her on a heart medication and it looks like open heart surgery is inevitable, although we would like to hold it off until she is around 6 months old (December). We will continue to go in to see the cardiologist to monitor. 

The cardiologist also feels that with as fast as her lungs are filling with "junk" and how bad her breathing is, that we are also looking at something else going on and agrees with the GI specialist and pulmonologist that we must work on safe(r) swallowing. We will spend Monday morning being evaluated by a speech therapist to get her opinion on whether or not she feels like it's safe to continue feeding Claire by mouth and/or whether it's possible that she's aspirating the reflux. Depending on her weight gain over the weekend and what her GI doc decides based on the therapists recommendation, we may be getting an NG or NJ (looking like NJ is more likely due to the reflux) tube put in for feeding on Monday.

In the meantime, we are back to thickening her breast milk - which creates more problems as it affects her differently because of the Hirschsprung's disease and is why we had to stop before but, at this point, her lungs are more important! Even this morning, she hasn't eating anything since last night around 8pm and has only eaten one ounce, she is exhausted.

It is looking more and more like we will be going "off the grid" until we can get Claire's breathing, weight, and heart better. With all of the treatments that we're giving her, all of the appointments that we'll be adding now with all of this new information, the hours we are spending on the phone trying to coordinate everything, and just the regular day-to-day things too (that, admittedly, aren't getting taken care of very well), it's time to admit that we need to pull back from some other things and focus on our family. Please know that we love you all and will do our best to keep in touch as much as we can, we miss you all very much and appreciate all of your messages even if we haven't been responding like we'd want to! 

As I'm sure you can imagine, I haven't been sleeping much ... it's beyond scary to have a little one who is struggling with breathing. Specific ways that you can pray are:

- Her heart: we are being told that open heart surgery is likely inevitable now but we serve a great God and we believe that anything is possible!

- Her breathing: that we can figure out a way to keep her lungs clear as the longer this continues, the more likely it is that she will have irreversible damage to them

- That we can find a way to get her eating again

- Her tummy: for a reason we still can't figure out, Claire's tummy is full of gas (if you compare her 1mo pictures to her 2mo, you will see what I'm talking about), we're trying all kinds of things to figure it out

We do know that we are very blessed and that this could all be so much worse. As discouraging as it is to feel like so many things are going wrong [I'd be lying if I said there hasn't been a lot of tears] and as much as I feel like we need to "pull back" from things that we love for this season. I also can't help but be so thankful for all of the things that are going right! Claire is here with us and she is happy <3.

As always, thank you for taking the time to follow our journey and for your continued prayers. We appreciate each one of you.

Strong and Courageous

"Have I not commanded you? Be strong and courageous. Do not be afraid; do not be discouraged, for the Lord your God will be with you wherever you go.” - Joshua 1:9

If I could sum up the last two weeks into two words it would be: strong and courageous [and humility... my courage came with a BIG helping of humility!].  Let's see if I can explain.

First, it takes a lot of courage for me to write about our journey, knowing how many of you are following and how many of Claire's nurses and doctors (who know far more than me about all of this!) are following as well is quite intimidating! We had a rough and very emotionally-charged week last week and every time I sat down to post an update, I felt vulnerable and decided not to. Over the weekend, several of you who had no idea what was going on in my heart and mind told us how much they enjoy our blog and how it has encouraged them [a big THANK YOU to those special people] ... so here we go!

Shall we start with the good news?  Claire had her first appointment with the eye doctor, so far so good!  She is far-sighted but she is too little (and too young) for it to warrant glasses, we will check this again when she is older. No cataracts and her optic nerves look great! She does cross her eyes but they will give her a little longer to outgrow this before it becomes worrisome. Our next visit with him will be in December.

Now onto the not-so-good news. Claire has started to have trouble breathing. Her breathing is very fast and she has chest retractions that show that her body is having to work hard in order to get air in. We took her to the ER last Thursday and she had an X-ray that showed "hyperinflation" and that her bowels are still very full of gas/distended but they couldn't find any reason why, we went home with lots of questions. We followed up with her pediatrician on Friday who didn't have many answers for us either. Over the weekend, I was still worried and felt like I needed answers but wasn't sure where to go. We had a playdate with a [very strong and courageous] friend on Tuesday who suggested taking Claire to see a pulmonologist. Even after she left, it took me a couple of hours to get up the courage but I called Claire's pediatrician and advocated for a referral. I am so glad that I did! 

**It's scary [at least for me] to talk to doctors, especially since I know that I still have so much to learn, but what I AM learning is that what I do know well is Claire!  Talk about a crash course in courage [and humility!]!!**

So, back to the breathing ... we went to see the pulmonologist yesterday and I was right to feel like something was wrong, her airway and lungs are inflamed and have "junk" in them. He believes this to be caused by one of two things: the beginnings of congestive heart failure or aspiration (or micro aspiration), both of which are very serious. We will be seeing the cardiologist again tomorrow to have her heart checked on again just to rule that out and are working with the feeding therapist and GI doc to work on safe(r) swallowing and management of the GERD (since reflux can be aspirated as well). In the meantime, we have started a steroid/breathing treatment in order to treat the inflammation. It is important that we figure this all out as soon as possible, not only for the long-term health of her lungs but also because she is working so hard to breathe that we are again struggling with her weight gain and eating - she is down to the 2nd percentile. He also went over all of the differences in the airways of children with Down syndrome for me (wow, there are A LOT!) which I found helpful just to keep in mind as we're working through everything. 

As far as the Hirschsprung's disease ... all is pretty good! Her stool is "a little looser than [the surgeon] would like" and she's much gassier than we want her to be but, overall, she isn't showing any signs of enterocolitis or stricture. She has increased our irrigations to twice/day in order to help with the gas/distention and we will continue to do her dilations 3 times, twice/day. 

We are also trying (and almost done with) Flagyl which is an antibiotic to see if "decontaminating" her intestines might help with the gas/distention. I am still on a pretty bland diet and no dairy/gluten/soy. We have also started "fortifying" her breast milk, something that I've been fighting but we're at the point where we just have to, at least for now until we can get her eating again. We have started discussing the possibility of putting in a feeding tube, this would mean another short (couple of days) hospital stay but would help with protecting her lungs and gaining weight.

We also escalated her reflux therapy to yet a stronger medication (Lansoprazole), she is still really struggling with the reflux, even to the point of having pure white boogers :(. Today, she had an "Upper GI series" which is a barium x-ray "video" to check to see if she has any kind of obstruction or webbing, she had to fast for this so she was pretty upset and then she wasn't able to swallow fast enough for the study so she had to have a feeding tube put in, she did NOT like that and it ended up being quite the traumatic morning. She squeezed my fingers hard with both hands during the entire thing. It did help a little that our X-ray tech is one that we've had a few times, it was nice to see a familiar face. Claire has now had over a dozen different ones and quite a few ultrasounds too. 

Admittedly, I'm emotional and just feeling discouraged after all of these long appointment days and TWICE while holding Claire today, I've smiled at her and she's instantly smiled back at me with her big, bright-eyed, tongue-sticking-out grin. Coincidence or not, that girl is touching my heart [and making me bawl like a baby] <3. Talk about strong and courageous.

Growing!

Another busy week of appointments for Claire and she finally made it to 8lbs!! We are still slowly dropping in percentile (4th now for weight) but she's growing which is great.  Our focus now is on figuring out what's going on with her tummy so that we can get her more comfortable.

Claire's full tummy :(

Her stomach looks like it's full of little hills as you can see her intestines sticking out because they are so full and X-rays show lots of air in both intestines so we are trying to help her relieve some of that discomfort with the irrigations (we use a catheter, syringes, and saline to help empty her colon - it is quite a messy process) once per day. I'm on my second week of no gluten, dairy or soy and and have finished almost a full week of no eggs, nuts, fish, caffeine or gassy foods. We originally started the elimination diet to try to help her manage the reflux but although her reflux is unchanged, she seems to be much less gassy. This week we will start slowly adding foods back in one at a time to see if we can find some answers, saving dairy/soy for last.  She already has decreased motility because of both Hirschsprung's Disease and Down syndrome so if she does end up having an intolerance to something in my diet, that would just be making things even worse so we want to make sure that we get all of the answers so that we can keep her as comfortable as possible and to keep her from developing enterocolitis ("a dangerous problem for children with Hirschsprung's Disease" - and she is at an even higher risk because of Ds).  Although I will say that all of the changes at once (the elimination diet, starting irrigations, introducing the breast milk thickener and we also changed her reflux medicine to a different one this week) are making it hard to tell what's truly helping, we would greatly appreciate prayers that the answers would be made clear to us.

Sister love <3

In the process of all of this, I am learning more than I ever thought I needed to know about poop! It is typical for it to get pretty messy during Claire's irrigations and dilations. Yesterday during her irrigation, I was in the middle of switching between syringes and was holding the end of the catheter in my hand. At the perfect [or rather not perfect at all!] moment, Claire let out some gas and it, very literally, rained poop all over both of us. We both needed a wardrobe change and a bath! Anna thought it was the funniest thing she'd ever seen and I couldn't help but laugh right along with her.

Our sleeping beauty,
still in newborn diapers and clothes

They moved up to the largest sized dilator this week and those are going well, although we still hate them. Hopefully this means we won't have to do them for as long as we originally thought!

We've also started thickening her breast milk this week because eating is still very uncomfortable for her as she is having it go down the wrong pipe (aspirating) and come back up (reflux) all at the same time that she's trying to swallow. The GI doctor really wants to avoid a feeding tube so we're trying everything we can think to keep her from developing an oral aversion and we are messaging with him almost daily. It takes her about 45-60 minutes to eat 3-4 ounces. The thickener helps a lot with feedings but we are still nervous about the effects of it on her gas/bowel emptying so we will just have to wait and see how it goes.

Her follow-up for her belly button went great. The surgeon removed all of the leftover scabbing and staining from the medicine that they put on. It's all closed up and we're finally cleared to give her real baths!

She still isn't on any sort of predictable eating/sleeping schedule, her reflux and gas wake her up often when she does fall asleep and her eating is very sporadic too so we're learning to just be flexible [and loving every minute!!]. Claire is such a precious little one and is handling all of this so incredibly well. Her smile is nothing like I've ever seen from a baby as young as her, she has the biggest, open-mouthed grin and it melts our hearts. She is our smiley, cuddle bug and we are all so in love <3.

Weekly Update: Irrigations :(

Eating is still not her favorite
thing; the red/puffy/watery
eyes are from aspirating and
reflux.

It's been a rough week for Ms. Claire. Her intestines aren't emptying like they should which has caused her to become very distended and uncomfortable, it also aggravated her reflux and made her even less motivated to eat (because her belly is so full/hard). Everything is checks and balances and we're learning very quickly to take one day at a time!

We also saw the surgeon this week to remove her umbilical stump. They are supposed to fall off in the first 10-21 days and Claire still had hers, underneath was still an open sore and so her pediatrician referred us to a surgeon (luckily we already have a great one!) and she had that removed and treated yesterday.

Our morning "torture" routine
(we have a night-time one too)

She also had a "surprise" X-ray done of her tummy because of the distention.  The X-ray showed that both her large/small intestine were much too full so she had an irrigation done to help release it and we will now have to do daily irrigations at home. This news was really disappointing for us but we are very thankful that it will help her to start feeling better again.

Her dilations are still going well and her surgeon moved us up yet another size this week; we do those three times, twice per day.

Our beautiful, tiny girl

We had more feeding therapy as well. She is still aspirating (this, the reflux, and her struggle with swallowing coordination are due to her low muscle tone which is a characteristic of Down syndrome). These appointments are a lot of trial and error, trying to figure out what we can do in order to position her to minimize this; how long feedings should be, how much she should eat at a time, how often, etc. in order to help her to gain weight but also to keep her from getting fatigued and aspirating. We are only supposed to breastfeed once per day for now so that she can continue to practice but because it takes so much work for her, she isn't able to get enough without fatiguing and that increases her risk for aspirating. She isn't able to "productively" breastfeed yet so we're still having to give her a bottle after these as well but hopefully with time and practice, that will change.

In addition to increasing the dose of her reflux medicine, we are also eliminating dairy, soy and wheat to see if this helps. We are starting with 14 days and then re-evaluating.  If we don't see a change, we will also eliminate eggs, nuts, and fish. This is common for all breastfeeding moms to try when they have a baby who struggles with reflux. Claire's reflux is most likely due to her low tone (reflux is very common with Down syndrome - and the Hirschprung's/distention makes it worse) so eliminating trigger foods probably won't make her reflux go away but if it could possibly help, we're going to try!

She weighed in at 7lbs 15oz today, that's in the 5th percentile. She's dropping lower and lower in percentile (which they don't like) but she IS gaining weight!!!

Claire says "TGIF!"

That's all for this week [I think!]. As always, thank you for your continued prayers and support and for taking the time to read about our journey. We will update again after our appointments next week.