This year

This year has been the hardest yet.  I don't say that lightly either.  I know the first year of Lucy life was hard and it was littered with a lot of 'downs', but the difference is, at the time we didn't know anything else.  We didn't have time at home with Lucy, she didn't have the personality she has developed over the last three years and she didn't have a little brother.  This year has dragged us through the mud.  I sometimes wonder if she will ever be that person she was before this surgery.  I see flashes of her old self, but they are so quickly replaced by tears when I tell her she can't have water or she has to wait x amount of minutes before her next toothbrush dip or we need to do a finger prick to test her coagulation levels.  Yes, that's right.....we got our INR machine, so I have the pleasure of testing her myself.

If you didn't hear, or are not on Facebook, we were back in the hospital for another visit.   It was luckily a short stay and we made it home for Christmas, but it was just another reminder that although we are home, Lucy remains sick and fragile.

A week before Christmas, Lucy woke up in the morning and her mouth was all bloody, her lips were cracked and so dry.  We consulted with her cardiologist and decided to drop her diuretics on Tuesday knowing that we had an appointment on Friday to see Dr. Husayni.  It's no secret that she is on a lot of diuretics and because of the aspiration pneumonia that kept us in the hospital during her G tube surgery, she has been very limited in her oral intake.  Couple that with the decrease in her feeds because of her vomiting and she was super dry.  Wednesday and Thursday she wasn't herself, coughing a lot and more tired than normal.  Friday morning we packed up and headed to the hospital for her appointment.  She looked worse for wear.  We started with an echo to look at heart function and check for effusions.  Unfortunately, we could not see much fluid, so an x ray was ordered and it looked bad.  On all accounts we should have been admitted, but given it was a couple days before Christmas, we thought going back up on her dose of bumex (diuretic) would hopefully clear everything up.   As a precaution, the intensivist that was on over the weekend came down to look at Lucy.  It gave them a baseline.  We left and I was to send a picture of Lucy the following morning for them to review.  We didn't even make it to the morning.  Around 5:30 pm Lucy was still not herself, so we put on the pulse ox and her saturations were in the low 60's.  I immediately called the PSHU to let them know.  We were told to wait an hour to see if they would come up to her baseline of 73.  In that hour, we packed a bag.  An hour passed and nothing had changed, her saturation had actually lowered (upper 50's, very scary).  We packed the car and headed to the hospital.

We got to the hospital and were admitted directly to the PSHU.  Lucy opted for a wheelchair instead of a wagon.  When we got up to the ICU, Dr. Sajan looked at Lucy and was almost puzzled as to why we were there.  Physically she looked pretty good, so we put on a pulse ox and it read 55.  We needed to place an IV, but when we tried, Lucy went ballistic and dropped her saturations into the 30's.  We ordered versed and decided that nitric was necessary.  Seriously, versed is nothing to Lucy at this point.  She screamed bloody murder through all of it (as I held her down).  A couple doses of IV diuretics, some antibiotics and nitric and within twelve plus hours she was back to her norm.  We decided after weaning the nitric and switching back to oral meds, we would give her one more night to monitor and then home.  Just in time to host Christmas eve and for Santa.

We believe this episode was caused by aspiration, clouding her lungs and making the weaning of diuretics impossible.  With Lucy's oral intake at a minimum, her aspiration has to be mostly from vomiting.  So here we are, December 29th, headed back to the hospital for another procedure that will require us to stay overnight at the hospital.  I would not have thought we could have squeezed another stay at the hospital this year but.......we are first case for a vocal cord injection.  We are hoping that the injection will in essence, pump up her left vocal cord and allow the right side to compensate to fully close.  What does this do?  When Lucy goes to swallow, this will close her vocal cords completely closing off her airway and allowing all liquid to go down her esophagus to her belly.
I'll write more later when I have had time to process.  In the meantime we very much need prayers that this procedure will work.  This is a temporary fix which could not work at all.  There's a chance it lasts a year, but the likelihood is it lasts closer to 3-6 months, in which we would need to go back and do another injection.

Specific prayer requests
~vocal cord injection works and lasts a year
~there are no complications and we are discharged on Saturday
~After the procedure we can start to allow her to drink liquids

Red Tie

In 2014 the Children's Heart Foundation held their first Red Tie Ball, even though Lucy was in the hospital at the time, we were able to attend.  I fell in the love with the event for a few reason:

1. Obviously.....raising money for CHD research.  Without this funding, advancements that have helped children like Lucy live past their first birthday and some into adulthood would not be possible.
2. A night out with my husband.  Anyone with children will tell you they can put strain on a marriage.  When you add a chronic illness and A LOT of time spent apart due to splitting time at the hospital that strain can multiply.  So "nights out" however infrequent are so important
3. Honoring those in the medical field that have had an impact on our lives.  I think sometimes in the wake of critical illness, the doctors, nurse practitioners and nurses take a back to seat to ups and downs of this journey.  The Red Tie Ball is a perfect setting to celebrate them.

The past two years, Lucy's doctor's have been recognized.  Her surgeon, Dr. Ilbawi and her cardiologist, Dr Husayni.  This year, Sarah, a nurse practitioner in the PSHU was honored with the Heart of Gold Award and I was humbled to be chosen to introduce her.  Her impact is beyond words, but I tried hard to encompass how much she means to our family.

Here is what I said:

Roughly four years ago, my husband and I stepped into Advocate Children’s hospital for the first time.  Unbeknownst to us, this hospital would become our second home.  Our daughter, Lucy was diagnosed Hypoplastic Left Heart Syndrome and she has done nothing by the book.  At one of our first appointments, I remember asking about recovery time, little did I know those numbers would never apply to us.  Lucy made us wait two months in the hospital before she was ready for her Norwood.  A routine catherization turned into an extra surgery and this summer after her Fontan she made us go back to the operating room to fenestrate, TWICE.  We have spent a good amount of time in the hospital, so it’s no surprise that the staff also became our family.

I met Sarah in 2014, during our first inpatient stay with Lucy.  While we didn’t have a lot of interaction, I have one memory of her from that time. We are standing in bed 6, quickly approaching 90 days in the hospital.  Lucy was technically not critical, but she cried a lot which was keeping us in the ICU.   We were discussing a move to the floor and Sarah looked at me and said,

"We would send Lucy down to the floor, but they will call up every time she cries.   If you want Lucy to move down there, you’ll have to move into the hospital."

Sounds like a dare, right?  Over the summer I had the opportunity to get to know Sarah.  What sounded like a dare, was just the truth.  My daughter was going to need someone to be with her 24 -7 and Sarah knew that.

Her brutal honesty coupled with her wit and humor help to soften the blow of the reality that is CHD.  It’s what kept me grounded over our 100 day hospital stay. It’s what put a smile on my face at least once a day.  How do you tell a mom whose been bedside for 60 days, there’s no way you’ll be out of the hospital in the next month and still make her smile.  That is Sarah.

Aside from her humor, her knowledge and ability to explain all things medical to a parent that is just trying to survive is impeccable.   Sarah spent countless hours in Lucy’s room discussing all of her issues, the approach and alternatives, medication and their impact, reviewing x-rays until I was comfortable in my understanding.  I often get complimented on how much I understand, and that is a credit to Sarah.

All these characteristics make Sarah an amazing nurse practitioner, but what truly makes her heart gold is her passion for her kids.  She is a fierce protector and advocate of her patients.  She has protected Lucy through and through, and she has stood in when I couldn’t be in the room……two trips placing NJ tubes, a bedside upper GI, an ENT scope and the removal of five chest tubes at the same time.  Lucy has some serious anxiety when it comes to the hospital.  But every time we go for an appointment or labs, one of the first things she says is ‘where’s my new friend Sarah?” and that is proof that Sarah’s impact far extends the hospital room.

I am honored to present this award to Sarah because from a parent and patient perspective she truly exemplifies a heart of gold.

*******************************************************************

 As I saw Sarah surrounded by families and colleagues at this event, it is clear she is respected, admired and loved.  I am truly grateful for her knowledge, her input, her humor and her friendship.

new faces, new places

We are just over one week home and starting to settle in.  I am not going to lie, the first two night home were hard.  We were on feeds every four hours that ran over an hour.  The first night looked something like this:
7pm feed
8pm flush line and take of tube.
11 pm feed
12 am flush line and take of tube
3 am feed
4 am flush line and take off tube
5 am start work

We did this for two nights and I quickly realized it was not a schedule any of us could maintain!  Lucy was awake for the beginning and end of the feeds to attach or remove the tube extension, so she wasn't getting good sleep either.  Do you know what a toddler can be like with no sleep....so you can guess that our days weren't all sunshine and rainbows.  Add to that, she threw up after 3 straight feeds going into Friday.  We decided to adjust the schedule.  

We came home on 6 feeds of 135 mL over an hour for a total of 810 mL (and about 650 calories).  Since she wasn't able to keep it down and we also want to cater to a normal eating schedule, we lowered the volume and went to 3 feeds during the day (100 mL) at breakfast, lunch and afternoon snack.  Then before bed we hook her up and do continuous feeds from 8 pm to 5 am.  This way everyone gets some good sleep and we are still shutting off the feeds in time for her belly to prepare for breakfast.  Our medications have not changed, but we are adjusting the schedule to coincide with feeding so we aren't taking the tube on and off.

The g tube is something to get used too.  The first couple days Lucy would cry anytime we picked it up, mentioned feeds or medicine.  I am not sure if it actually hurt when putting the tube in, or if it was more mental than anything, but I too had some anxiety around it.  Just like everything else, the more you do it, the easier it gets.

After being the hospital for so long, I always want our return home to speed up recovery.  I wish it were that easy:  that her eating would turn around, that the throwing up would stop, that physically she could do everything like before.  But all of it takes time and I for one can't wait for us to be once again tube free doing all things normally.  In order to get to that point we decided to get a second opinion regarding matters not of the heart.  We scheduled our first appointment with Lurie Children's hospital in their Aerodigestive Program.  (Unfortunately for us, Advocate children's hospital does not have one).  The program serves children with complex airway disorders, requiring pulmonary, upper digestive tract, sleep voice and swallowing evaluations.  We are hoping this program will be able to help us answer some questions regarding digestion and her vocal cord paresis and also help keep her lungs healthy.  The appointment went well (aside from the flexible scope we had to do).  We met with GI, ENT, speech and the APN.  We came out of it with our first procedure scheduled:  a gastric emptying test at the beginning of January.  If you know me, you know I am a creature of routine and change is hard for me.  I like to plan.  The arrival of Lucy almost 4 years ago has really challenged me in that area of my life.   Yet again, this will be another 'change' we will tackle.  New faces, new hospital, new location.  The clinic is once a month, so we'll head to the city and I can only assume this will become part of our new routine that is bound to change. 

Here we are in the waiting room at Lurie's

And Lucy with her trusty drinking toothbrush

When I started this blog back in 2013, it was a way to keep family and friends updated on what was happening with the pregnancy, birth and Lucy's heart journey.  While that is still the main focus, it's allowed me a place to air my feelings, good and bad, celebrate accomplishments and to share with Lucy (eventually) those that impacted our lives during this journey.....see next post.

frail

Lucy is fragile.  Lucy has had life saving surgery.  Lucy has been close to death.  Those words are hard to say and it's probably why I have never said them.  But the reality is that she is very fragile and minor surgery like a g tube is making that evident.  I know, I know, no surgery is minor.  But compared to what we have been through for Lucy it should be.  It's possible  that I want her to be strong physically so I convinced myself she is.  Yet again, I have been fooled.

Maybe it was the success of our NG trial that had me so hopeful.  We placed the NG tube Friday night (thank you to those that offered to come help, it was truly appreciated).  We did continuous feeds at 20 mL/hour overnight.  Saturday we increased to 30 mL/hour.  With minimal throwing up, we decided to try some bolus feeds.  I know, optimistic.  To my surprise, she handled 60 mL/hour, then three more bolus feeds at 90 mL/hour.  Going into this surgery that was our biggest concern, her stomach couldn't handle the feeds.  Surgery on Monday went as expected with no issues.  The hardest part was no eating or drinking the first day.  Tuesday we could eat so we made a grocery list to ensure we had all the food she wanted.

-black olives
-extra sharp cheddar cheese
-canned green beans
-peanut butter and honey for sandwiches
-strawberries
-grapes, the peeled ones (she doesn't know I do this by hand or she does and does not care)
-orange juice
-orange chips (sour cream and cheddar)
-crackers

Sour cream and guacamole were added because those are some favorite from home.
Quite the palette for three year old.  As much as she asks for food, she eats hardly any.
Tuesday, we started feeds slowly at 20 mL/hour for four hours.  No issues!  But slowly as the day went on her breathing changed.  We started her first bolus feed of 60 mL/hour, but mid way through she looked really distended.  We stopped and gave her a glycerin chip to help her poop. The doctor decided it was better to do continuous feeds overnight.

By Wednesday morning, holy crap......she was puffy!  So much so, she dropped her saturations to the low 50's and we had to switch over to high flow oxygen at 10 liters.

The stress of this minor surgery (I use that term loosely) wreaked havoc on her body.  Her x-ray on Tuesday and Wednesday (along with her echo) showed a small effusion, but nothing that should be impacting her this heavily.  Wednesday the team decided to give an IV dose of lasix, and boy oh boy.  her diaper was huge.  Although it helped it didn't solve the problem.  Lucy kept dipping her saturations and she started retracting (for those that don't know retractions are a sucking in of the skin in between or around the bones of the chest when inhaling).  Honestly before this....I have never seen Lucy retract.

We hoped the extra IV diuretics would do the trick, so we gave Lucy a day and skipped x-ray on Thursday and took a trip down to radiology Friday morning.  An x-ray can be more clear when you stand than laying in a bed (hence the trip).  Probably a good thing we did.  The x-ray showed the same small effusion and aspiration pneumonia.  This girl seriously can't catch a break.

How did this happen?  It's possible some of this has been happening slowly over the last couple months.  Although we tried hard to restrict her from thin liquids, I can't say she hasn't gotten her hands on some.  It's also possible, it happened over the course of this week.  With being intubated for surgery, more damage to her vocal cords could have allowed small amounts of liquid to slip into her lungs.  Either way it happened, it doesn't really matter, because it's there and we have to stay until it clears up.  Today, in order to do everything possible, we put Lucy back on high flow with nitric, added IV diuretics back, started an antibiotic and restricted her drinking.  Feels like square one, nitric was a bitch to wean over the summer and I am not looking forward to doing it again.

This was a huge blow and evidence of Lucy's frailty but we still have some good news to report.  Lucy is successfully handling her feeds (on a home regimen at that...this is huge!).  Although we will probably be in the hospital longer than we wanted, we can start to play around with her feeds.  The worst part is the tube site is still pretty sore and she HATES when we have to take the tube on and off.  It should get better as it heals, but for know it sucks!

Her demeanor is touch and go.  Sometimes she is great and just hilarious!  Bossing people around, she literally told one of the cardiac fellows to go get her ice.  She also still has a little PTSD from our summer.  I have to give all her meds and anytime someone is by her bed she says "She is not going to touch me, right?"  Breaks my heart!  We have been able to get her up and out of bed.  Unfortunately we have had to use presents under the Christmas tree in the unit to do so.  There is a Mickey Mouse figure that "brings presents" but the caveat is that Lucy has to walk there from her room to get the present.  Her first present was a backpack doll house courtesy of my Aunt Betsy and her granddaughter (who knows hospital life all too well) and her second present was Sadness (her favorite character from the movie inside out) courtesy of one of our favorite nurses, Kate.  Hopefully we bust out of the hospital soon, because she won't have any other presents to get come Christmas