13 Days til Surgery

Claire figured out how to hold her pacifier AND
get her fingers in her mouth this week. 

Claire's surgical team met yesterday morning to go over all of her scans and medical record. They all feel that her surgery and recovery will be more complicated than usual because she also has pulmonary hypertension. Because of this they have decided to move her surgery to 10/13, she is able to be the only cardiac surgery on that day. We are still being monitored on an outpatient basis for her weight gain and will go in on 10/7 for her two hour pre-op appointment. She will have additional scans done, we will meet with the surgeon and anesthesiologist, and they will draw her blood so that they can get blood for surgery (she will be on full heart/lung bypass so she will definitely be getting blood during surgery).

Our check-in time is 5:30am on Monday the 13th and surgery starts at 8am.  Just like last time, I will create a "surgery updates" blog post and will post updates throughout the morning.  They have a surgery waiting room for us to sit in and they will call us periodically with updates that I will share.

At our appointment yesterday, she was still not showing any weight gain so we have switched to fortifying my breast milk with a "pre-digested" prescription formula.  She is still on continuous NJ feeds (into her intestine) via a pump. We will try the new formula for a few days and go in again on Thursday to see if we have made progress. One of the benefits of a later surgery date is more time to gain weight!  Her surgeon would like her to be a little over 2 pounds heavier than she is now which probably isn't realistic in two weeks time but we're going to try to get as close as we can.

13 days ... trying to get in as much cuddle time as we can as it will be a while before we will be able to hold her again post-surgery.  I am starting to feel like I'll be holding my breath until I hear the words "we are finished and everything went great" ... until then, I'm picturing her in Jesus' arms and filling my heart with as many peace-filled scriptures I can find ;).  I will update again on Tuesday after our pre-op appointment when I will have more information about exactly what they are going to do.

Thank you for praying!

Home!

Claire still has not been able to gain significant weight despite now having a pump that feeds her calorie-dense breast milk directly into her intestine continuously throughout the day and night. But thanks to her amazing GI doctor, we are finally HOME! He advocated on our behalf and will be seeing us in his clinic all throughout the week to monitor Claire and to adjust her "calorie cocktail" as needed. I could have hugged him ;).

Another condition of us going home was promising that we could keep Claire "cocooned" as even a small cold would force them to push back her surgery by 3-4 weeks and she just doesn't have that time to wait. Hand sanitizer and hibernation are our new best friends.

I met her cardio-thoracic surgeon (Dr. Nuri) yesterday for quite some time. He explained the procedure and risks in great detail and the further reasoning why we can't wait any longer to move forward with it. He and his team are meeting tomorrow morning to go through all of Claire's images and to discuss the plan for surgery, she has the bigger hole (VSD) but also a few less-significant things that they also need to repair while they are in there.

Prayer requests:

-that we all remain healthy so that Claire can stay at home until surgery

-that Claire will start to gain weight as her size plays a big part in the outcome of the surgery 

-that her Creator and the One who knows her from the inside-out would impart great wisdom to the surgical team as they review her images and create their plan when they meet tomorrow morning

And remember friends, He holds YOU in His gentle and mighty hands too <3.

Claire telling Daddy all about the hospital

And finally, I'd like to thank all of you.  Our blog reached over 10,000 views this week. I am completely overwhelmed by the amount of people who are following Claire's journey.  Thank you for your continued prayer and support, it is such a comfort to know that there are so many praying for Claire and our family. If you have a moment to comment we would love to hear from you. Have a blessed and restful Sunday; there is always, always, always something to be thankful for.

Update from the Hospital

Today is our fourth day in the hospital and it sounds like we will be here at least two more days, we are just waiting for something to work feeding-wise so that Claire will start to gain weight.

Claire is working so hard just to breathe that she is constantly sweating, she even wakes up in a puddle of sweat, so we are really struggling to get weight on her.  We are fortifying her breast milk as much as her body can handle and feeding her all through the day and night and she is still not gaining even close to what they would like to see her gain each day. Her liver is swollen now as well which is a sign that her heart failure is progressing despite all of her medications. They have decided that she can't wait any longer and have decided to schedule her open heart surgery.  Her cardiologist feels like a significant amount of blood is passing through the hole and is concerned at how quickly the pressure on the right side of her heart is increasing. It will most likely take place on Monday, October 6th, someone is coming to talk to us tomorrow and get us all set up with what we need in order to prepare for surgery.

Our focus over the next ten days (EEEEK!) will be to get as much weight on her as we can, to try to clear up her lungs, and to keep her SUPER healthy so that she can have a successful surgery and recovery.  Every little tiny thing at this point could make a big difference in terms of the length of her recovery.

We are feeding her exclusively through the tube now for a few reasons; so that she isn't using up any of her precious calories for eating and so that we can protect her lungs. We are still trying to find something that will work for her reflux - she is on the maximum dose that they can give her and is still really struggling with it but because there is a big concern that she is aspirating it when it comes up, that's something else that they'd like to figure out before we go home.  Both her GI doc and her surgeon are in the hospital this weekend so we hope to collaborate with them to get this figured out soon so that we can spend a few precious days at home before her surgery.  They expect us to be in the hospital for a couple of weeks post-surgery.

Here are some pictures for you:

Our Skinny Miss Claire

Big Brother stopped by to say "hi!"

Nurse Amber got some Claire cuddles

This is how Claire eats lunch these days

And some encouragement for all of us ...

Have a great night friends <3.  I will update more when we know more!  Thank you for your continued prayer and support, I have loved reading all of your messages.

Three Months & Hospital Admittance

Claire is THREE months old!!  8 pounds 14 ounces (0.18%) and 22.25 (5.8%) inches long. She loves to be held and to swing in her swing. She is starting to "talk" back to us, she can almost roll from her back to her front, she has found her thumb and her hands, and she is starting to reach for and grab onto objects.

She is sleeping so I thought I'd post a quick update.

We saw her cardiologist yesterday and he admitted her to Marybridge, she hadn't kept more than 4oz down in almost two full days, was losing weight pretty quickly, and was really struggling with breathing and dehydration so he felt like she really needed in-patient care.

The great news is that they figured out the diarrhea and vomiting pretty quickly, she has Digoxin toxicity from one of her heart medications.  She hasn't had any more of that particular medication in 24 hours and they are testing her blood every 12 hours to make sure that the levels come down. She was over twice the maximum level when we were admitted and was still about double the maximum this morning :(. We will restart it again when they are back to a safer level but will carefully monitor her levels to make sure that they remain safe.

We will be staying here for a few days while they work on developing a safe feeding plan, she had lost about an ounce per day over the last week and is in the lowest percentile that she's ever been in (0.18%).

Her studies last week confirmed both aspiration and reflux all the way up into the very top of her esophagus (they are thinking she is also aspirating the reflux) and her chest x-ray showed still increasing edema and fluid. Over the next few days we will be trying various things so that we can figure out the best, and least-invasive, way to protect her lungs from permanent damage because of the fluid in them. We've already tried thickening her feeds, the heart medications, and several different reflux medications over the last month or so and her lungs continue to get worse so now we are looking at other options. Lots of doctors, lots of opinions, and lots of things to try! We are so thankful for such an amazing team, we even had two of her specialists call from home to check on us late last night and they have been communicating with each other all day to make sure that we are doing everything we can to get Claire growing.


The GREAT news is that the goal of this admittance is just to find a way to get her eating safely and gaining weight so the plan is that we will only be here for a few days. That could change depending on what we try and whether or not it works but, at this point, they are not thinking that this admittance will end in any surgeries - YAY!!  The cardiologist is still hopeful that, with her medications, we can wait at least another month for her heart surgery so that she can have time to get a little bigger which will help the surgery go more smoothly as well as the recovery.  He has started her on two diuretics, a medication to treat pulmonary hypertension, and one to help her heart work more efficiently.  She is stable, we just need to find a way to get her going consistently in the right direction.  Lots to be thankful for!!

I will continue to update as I can.

Thank you for your continued prayers. "He's got the whole world in His hands <3."

Testing Week

I read an article this week that resonated with some of the thoughts that I've been having about Down syndrome. "Down syndrome does not define an individual any more than my brown eyes define me. It is a part of who that person is- first and foremost a human being." We are far more alike than we are different.

"A person with Down syndrome is not disabled. Rather they are a person who is able with a different set of abilities. In our pursuit for perfection in this cut-throat society it is so easy to put labels on people rather than just seeing them as people with different circumstances. For those of us living outside of society’s definition of “normal” and not just with Down syndrome- the fact that some people wear their challenges outwardly does not make them disabled... In fact aren’t we all challenged and inadequate in some way whether it be emotionally, socially, intellectually, athletically, etc? Oscar Pistorius, a South African paralympic athlete said, “ My mother said to us one morning, ‘Carl, you put on your shoes, and Oscar, you put on your prosthetic legs.’ And that was the last we heard of it. I didn’t grow up thinking I had a disability,” he said, “I grew up thinking I had different shoes.” 

Another person, another gift, another life! Another opportunity for us to learn more about our God for we are ALL created in His image; with unique features, gifts, and abilities. I hope that one day, each of you will get the chance to spend time with Claire. It's hard to put into words but there truly is something so unique and special about her personality and attitude... we are blessed!

....and I don't see any of that here <3.

And for the updates .... 

All checked into the 
hospital this morning
for her procedure

Claire is still having lots of trouble breathing (it's getting increasingly worse) despite being on the heart medication for 2 weeks as well as thickening all of her breast milk for the same amount of time so we are in kind of a "testing week" with both GI and cardiology. This morning, she had a procedure to place a probe in her esophagus. It's attached to a little computer and will test the functioning of her esophagus as well as the pH. I push a button when she's eating, lying down, sitting up, coughing, having trouble breathing, or gulping and that further helps them read the results. She is also having another swallow study on Wednesday to see if aspiration is still a contributor.

We saw her cardiologist on Friday for an EKG/Echo/X-ray. She has only gained 7 grams of weight per day since our last appointment with him and that's with the feeding tube and fortifying the breast milk (between the previous two appointments she was at 22g/day). She had an X-ray on Friday and another this morning, the amount of "junk" in her lungs has increased quite a bit even just over the weekend.  He has increased the volume of her current heart medication as well as adding in THREE new heart medications (for a total of 4, and all are taken 2-3 times/day). He has also increased our visits with him to weekly. We are still hoping to hold off surgery until she's a little bigger/older so we need lots of prayer that she turns a corner VERY soon. If her lungs continue to get worse at this rate, heart surgery will not be far off. 

Our insurance is refusing to cover three of her medications (one is for GI and two heart medications) so we would appreciate prayer for that as well. The doctor says that there are no alternatives and that she absolutely needs them right away so we are praying that they agree to cover them and soon! The cardiologist's office has made an appeal and are communicating with the insurance company and I am on hold now waiting to make my own appeal.

Her surgeon is keeping her irrigations and dilations the same (each 2x/day) so that we can just make sure that we aren't adding in any factors as we're figuring out her heart and breathing but she did talk to us a little bit about placing a G tube. This would replace her NG tube, it would be a surgical procedure and involve a few days in the hospital. I am not convinced that we need to do this yet but she wants to keep it on the table so we will see how some of her testing this week goes.

Here's a little peek into Claire's adventures this week:

Thank you for your prayers and encouragement <3. Have a blessed week friends!

Learning and Growing

It was a big week for us, Claire had her first developmental evaluation and her biggest sister, Anna, started Kindergarten! 

All of Claire's specialists have been pleased with how she is doing this week which is refreshing news after the last couple of weeks. The working theory is that now that the NG tube has been placed, she is actually getting her heart medication (and not aspirating it) which is giving her more energy although it still isn't helping her breathing :(. She weighed in at 8lb 11oz yesterday which is back up to what she was a couple of weeks ago before she started losing. She is still "off the charts" at the 0.78th percentile but we're heading in the right direction again! She has "good" days and "not so good" days with eating, some days we use the tube more for feeding and some days where we hardly use it at all.

The theory is that she will continue to struggle more and more with weight gain and breathing but we are trying to get her as big as we can before we go ahead with open heart surgery (we see her cardiologist again on 9/12 to see how it's holding up). Right now, she is getting 24oz of breast milk per day which is exactly on track for a baby of her size BUT because her heart is working so hard and she is having to work so hard to breathe and eat, she is still struggling to gain weight even with the correct amount of breast milk.  So in order to help her, we are fortifying the breast milk. Her tummy is too small and she's too tired to increase the volume of breast milk so increasing calories is the best option. We will diligently keep track of her intake and continue to meet weekly with a dietician so that we can stay on top of both the volume and calorie content as her needs change.

We are still struggling a lot with reflux and bowel movements/gas but nothing new to report, her surgeon is still seeing us every week to help and we are still doing the dilations and irrigations twice each day.

Look what I learned to do this week!

Her developmental evaluation went well, she is behind but we already expected that.  The therapists gave me some things to work on at home but we will not be starting therapy until after her heart surgery. With how hard her little body is working, it just doesn't make sense to add anything else. After surgery her body will be stronger and more ready.

Her newest little trick this week is that she is lifting her head for very brief periods!!  I cried the first time she did it, all of the little milestones are becoming very precious <3.

"O Lord, you have searched me and known me!

You know when I sit down and when I rise up;

you discern my thoughts from afar.

You search out my path and my lying down

and are acquainted with all my ways.

Even before a word is on my tongue,

behold, O Lord, you know it altogether.

You hem me in, behind and before,

and lay your hand upon me."

-Psalm 139:1-5

Be encouraged, dear friends, nothing you are going through is "too small" [or "too big"] for our Father. He loves you so much that he cares about every little part of your day, no matter how big or small. He goes before you and behind you. Have a great week, we are praying and believing for great things for all of us <3!

At our developmental evaluation

Left: Anna on the day we brought her home from the hospital
Right: Anna (morning of her first day of Kindergarten)
& Claire wearing Anna's outfit

Siblings [we miss you Emelyn!]

Sister love!