back to the cath lab

This past week has been we have been in a holding pattern.  The doctors wanted to go back to the cath lab to get information about Lucy's lung and heart pressures before making any decisions about going back to the operating room to fenestrate her Fontan.  We were placed on the cath lab schedule for last Friday and then quickly moved to Monday (today).  Which meant this weekend was full of trying to have fun.  Friday Aunt Kate came to visit and brought tons of neat stuff to play with, like a barbie head to do hair, pie face and plenty of books.  Lucy, however, played with the jelly beans and Tupperware.  Saturday Dr. Patel brought us the Frozen match game.  We played that all day and Lucy won every game.  Sunday we were able to make it outside after 17 long days in bed #2.  Lucy loves playing in water, so we brought a basin of water for her to splash her.  Her amazing nurse Ashley bought her some water toys to help with the fun.  I can't put into words how amazing the staff at Advocate Children's is.  They have truly gone above and beyond to make Lucy's hospital stay the best it can be.

And then we made her put on her hat and sunglasses and the fun was over. 

This morning we were prepared for the cath lab.  She was to be third case.  When I arrived this morning I found out third case was at 12 pm, which meant, for four long hours, I would need to keep Lucy occupied so that she didn't notice she wasn't drinking or eating.  For catherization procedures, they require you to be NPO for six hours.  Luckily, we were moved up to second case and she was taken to the lab around 10 am.  Before we packed her up to walk down, Dr Patel came in to speak with me.  Over the weekend he had reviewed pictures from her previous cath and from surgery.  He noticed that there was some narrowing her left pulmonary artery.  He told me that during the cath, they are going to try to balloon and(or) stent the artery.  If you remember, since all her blood flows passively, it will try to take the path of least resistance.  Since the left pulmonary artery is smaller, most of the blood flow is going to the right pulmonary artery and lung.  When that pressure gets to high, it will flow to the left (or any other path of lesser resistance).  In ballooning the left artery, we hope that this will lower her lung pressures all together which in turn would help with the drainage.  I believe this is our last ditch effort to avoid surgery.  They will probably give her a couple of days to see if the adjustment to the left pulmonary artery improves her drainage.  If it continues to drain at this capacity, our only option left would be to fenestrate.

For our prayer warriors, we ask that you specifically pray for the ballooning of her left pulmonary artery to help with any pressure issues she is having and that this small procedure have a high impact on slowing or stopping the drainage.

As always, we could not do this without your support.  Whether you are near or far, we are on this journey together.

Day 12

During Friday morning rounds, Dr. Hoffman decided that a feeding tube was necessary.  Her nutrition was becoming very concerning, as the little she was eating she was also throwing up.  They also requested a visit from a nephrologist (kidney doctor).  As for cardiac and chest tubes, the plan was to do everything possible over the weekend to get the drainage down, then start discussing surgery.

Feeding tube went in Friday afternoon.  We went with an NJ tube instead of an NG tube.  Since she has been throwing up so much of her food, the NJ tube will sit in her intestine and bypass her stomach.  The hope is she will keep most of her feed down.  Her total caloric needs are roughly 1000 calories per day.  We are providing half those overnight via continuous feeds.  It would be 55 mL per hour over ten hours.  I thought to myself, that is way to much, she can't handle that.  But sometimes, the hospital can be intimidating and make you second guess yourself.

Guess what?  Overnight she threw up three times.  MOMS!  Always trust your gut.  Saturday the feeds were reduced to 30 mL per hour and we were able to lengthen the span to twelve hours.  The downfall of tube feedings: it takes away from her drinking during the day.  Her oral intake was set to 500 mL (16 ounces).  You know after a hangover, how you just want to chug drinks, water, soda, anything.  I have to assume that this is how Lucy feels, all day, every day.  Today (Monday), feeds were increased to 35 mL, which brought her down to 400 mL.  The best way I can describe what it's like......we try to give her liquids on the hour, so every one can know where she is at throughout the day.  The other 59 minutes are spent with Lucy crying, saying 'mommy, I want something to drink'.  I was spent emotionally by Sunday afternoon and was on the brink of tears most of the day (if not crying already).

Friday, we started two new IV drugs to help with drainage while also protecting her kidneys (Fenoldopan and Octreotide).  The reason we are using this type of diuretic is because Lucy's kidney's took a small hit after surgery.  They were worried with sodium issues that her kidneys were not functioning normally, hence the Nephrologist.  Saturday morning Lucy had liver ultrasound and thankfully it was confirmed that everything was normal.

Our work over the weekend did little to help with Lucy's drainage.  Lucy's has two chest tubes (one was draining minimally, the other has barely decreased in output).  I was convinced they would first thing we would have a definite yes for surgery.  However, Monday morning, Dr. Ilbawi decided he wanted to see an echo before making a decision.  The cardiologist okay'd the tube that was not draining could be taken out.  During removal, Sara (one of the APN's) noticed the other chest tubes sutures were almost out.   Her concern was, that during the night they could come undone and the chest tube would come out causing major damage.  She spoke with Dr. Ilbawi after he reviewed the echo and for safety reasons, mid afternoon, they pulled the second chest tube and placed a new one on her right side under her armpit.  They made some medication changes and are going to see what the output looks like in the morning, then make a decision.

One bright note of Monday, was we finally found something that Lucy will eat.  We were able to concoct a smoothie using no liquid, that allowed her to drink it through a straw simulating drinking.
I think she was so excited to 'drink' she finished the whole thing.  Only to throw it all up 20 minutes later.  Our goal is to get her to drink 500 calories (or more) worth of smoothies during the day in hopes of stopping the continuous feeds and eventually getting the tube out!

you take the good, you take the bad

I wanted it to be easier this time.  Unfortunately I don't think there will ever be a surgery or hospital stay that's 'easy'.  I thought with her age, Lucy would be more vocal about her needs and wants.  I think I underestimated the effect of the hospital surrounding on a three year old and the ability to process what's happening because mostly she just cries.

I also forgot that along with the good days, there are bad days.  Today is a bad day.
After every surgery, you have a balancing act.  Juggling cardiac, pulmonary, nutrition and recovery can be tricky, especially with Lucy's history of chyle.  We have been working to get the chest tubes out using diuretics, while encouraging her to eat but limiting her oral liquid.  But the effort has had some negative effects on recovery.

We had a lot thrown at us this morning.  So I am just going to do a quick overview as a plan isn't really in place yet.

The chest tube drainage is of concern.  They feel that the pressure from the new physiology is causing more drainage.  The diuretics helped, but when her sodium levels dipped, they were all stopped.
Low sodium levels can put Lucy in danger of seizures.  Her sodium level at it's lowest, was 121 (they would like to see it between 140 and 160).   To bring the back up, a sodium drip (Aunt Erin would be in heaven) was started and the push for eating high sodium foods.  Apparently it is easier to raise sodium levels through food, than IV.  Never did I think ramen noodles would be the recommended diet for Lucy in the hospital.

Another concern of the chest output is chylus effusions.  We changed to a low fat diet yesterday, but per Dr. Ilbawi are on a full fat free diet.  This puts us at a disadvantage with her nutrition as there are not a lot of options that are fat free with higher calories and protein.

Her albumin was also on the low side.  Albumin is protein made by the liver that keeps fluid from leaking out of the blood vessels nourishes tissue and transports hormones, vitamins, drugs and substances like calcium and sodium throughout the body.  They are replacing that via IV in hopes to help the chest tube drainage.t allows the body to absorb certain nutrients like sodium better

A tentative plan is to replace her albumin, increase her milrinone and nitric and provide 50% of her calories via TPN.  Then re-evalute in the morning.

As of now, our back up plans would be to place and NG tube for continuous feeds of non-fat formula overnight.  There is also talk about going back to the OR to fenestrate her fontan.

None are a certainty, but hearing the word surgery....it was the first time in morning rounds with all the doctors that I cried....til tomorrow

The Fontan

Here we are, Fontan surgery.  It feels like this day has been a long time coming and with the postponement, it made anticipation that much greater.

Wednesday night we said goodbye to Connor and packed for the hospital.  Lucy played with her brave cape (thank you Heart Heroes)!  We also read Franklin goes to the hospital three times before bed.  Thank you Grandma Sherry!

Thursday morning we arrived at the hospital at 6 am.  We were lucky with the delay of surgery that we did not have to redo pre-op testing.  They did however have to draw blood.  Never a fan of the needle prick (I get it, I hate them too) she cried her eyes out.  But she had the Peanuts gang with her along with a new Peanut comic book to help out until it was time to be rolled away.

At about 7:15 am we gave her a dose of versed.  Always funny to see her loopy and she was about ready to head into surgery, but let's back up a little bit.

As most of you know back in July of 2014 Lucy had her Glenn procedure, the second part of the 3 stage surgical approach.  It is the first part of directing her blue blood (oxygen poor) directly to her lungs.  The Glenn brings all the oxygen poor blood from her upper body directly to the lungs by connecting the superior vena cava to the pulmonary artery.  The Fontan procedure completes the task of allowing all oxygen poor blood to flow (instead of being pumped) into the lungs.
The procedure disconnects the inferior vena cava from the heart and connects it to the pulmonary artery (via a conduit) allowing all the blood from the lower body to flow to the lungs.  At this point all  flow to the lungs is now passive, driven by pressure build up in the veins.  The pressure build up can cause fluid to leak into the chest cavity and therefore kids are put on a restricted liquid diet for a period of time.

Alright, we are all caught up!

She was just chilling as we kissed her and said goodbye.  We headed down to the cafeteria for breakfast as they prepped Lucy in the OR.  At 9 am, the nurse (Kathleen) came out for the first time.  Kathleen said she did amazing.  They had beach boys playing in the OR per Lucy's request.   A big thank you to one of our favorites, Sheila, for making that happen.  Lucy had her central line and arterial lines placed and she was already on by-pass.  We had assumed she would have a lot of scar tissue and it would take some time for the doctors to clean it up.  Apparently not the case.  Around 10 am, Kathleen came back out and surgery was complete and Lucy was being closed up.  It felt like the fastest surgery to date.  Although, I think my memory is a little foggy from normal, non hospital life!

Lucy was in her room by 11 am and we were with her shortly after.  Dr. El Zein said everything went according to plan.  They did not have to fenestrate, which is great.  It means her lung pressures were in range of where they needed to be.  The cardiologist said she was really dry and the maintenance level of fluids had to be increased a significant amount to get her numbers where they needed.  

Lucy was not able to eat or drink for 24 hours after surgery.  We really saw her discomfort all day after surgery.  She cried about every ten minutes for at least eight hours straight.  It was so disheartening to have her continually ask for water and have to say no.  To ease the pain and agitation, we gave morhpine, versed, toradol and fentanyl, but nothing was working!  She would not settle down.  Around 9 pm, the doctor decided to put her on a fentanyl drip, in hopes that a consistent amount would allow her to get some rest.

Lucy's intake is reduce by around 50% to ensure there is not liquid build up around the lungs and other places.  Her total liquid intake for day two was 1800 ML (60 oz), but that included any fluid given to her by IV.  When all that was taken into account, she was allowed twenty ounces of water for a twenty four hour period.  I know what you are thinking, why can't they reduce the amount given by IV and let her drink it instead.  I asked!  The IV fluid has electrolytes, sugars and other things her body needs that plain drinking water doesn't.  Bummer!  We rationed her water by giving her 2 ounces every two hours while trying to save about 4 ounce for overnight.  There were still times she asked for water and was not able to have it.  She did eat two servings of yogurt and some applesauce which I think was a small distraction from the water.  

By day three the lack of sleep had caught up.  She slept most of the day only waking up two or three times.  It was nice because every time she woke up she was able to have two ounces of water.  We saw her appetite decrease to nothing, but I managed to sneak in three bites of yogurt around dinner time.

 She was so tired she couldn't even hold her head up to watch the kindle.

From a cardiac standpoint she is looking very good.  The doctors are just trying to ween anything that isn't needed while making sure her electrolytes, calcium, potassium stay in balance.  I will say it is quite weird to see her oxygen saturation at 97%, which means her fingers, toes and lips no longer have the purple tint.  No more having strangers ask me if Lucy is wearing lipstick or just ate a Popsicle!

We have heard more than once that Lucy has been a model patient.  I have heard her thanking the nursing staff without prompting and that brings a smile to my face.  We cannot wait for her to get back to her old silly self.

We want to thank all our prayer warriors that have been on this journey with us.  I can't express in words what you mean to us.  Whether it was a prayer for overall recovery or more specific prayer for her thirst to be quenched, each and every one was cherished.  We love you!!! 

Surgery date

I know many have been waiting and asking about Lucy's next surgery and we got the call last Wednesday.  Her catherization was what they expected and it was time to go ahead and schedule.

The big date.....April 20th.

We will head down to the hospital April 17th for all her pre-op tests to ensure she is healthy for surgery.  I will do my best to make sure that happens!  If you can't recall her last surgery we did pre-op the morning of surgery and that was dreadful.  Lesson learned.

It's amazing the impact that setting the date can have.  My eyes tear up as I write.  It's not because I am not confident in her team.  Advocate has an amazing team of surgeons, doctors, nurses and care technicians.  As much as we have been hospital free for almost 2 years, setting the date brings me right back to day one.  The memories flood in (not all of them bad)

-standing by her NICU bed waiting for her to get big enough 

- entering the PSHU for the first time 

- meeting Dr. Nater after surgery discussing Lucy's body 

-The hallway packed with doctors after Lucy coded

-Sandy waiting for me with open arms as I sobbed on her shoulder, giving me updates as she could.

-The daily routine of hospital life

-Talking with Jen about her wedding to give my mind a break

-The news we would stay impatient until her next surgery

-The phone call at 9 pm to tell us we needed to be back at the hospital

-Dr. Van Burgen doing CPR as Lucy's heart rate plummeted

-all the set backs

-Graduating to Floor 2 only if we moved into the hospital

-my evening chats with Maureen 

-Dr. Penk telling me I need to try again after the first failed attempt of NG tube placement

-Sitting with Dr. Van Burgen the night before discharge going over our checklist

-going home for the first time after 96 days in the hospital

-All our friends (known and unknown) that prayed with us each step of the way

April 20th we will get up and head to the hospital.  The Fontan procedure will connect the blood vessel (inferior vena cava) that drains deoxygenated blood from the lower part of the body into the heart, to the pulmonary artery by creating a channel through or just outside the heart to direct blood to the pulmonary artery.  At this stage, all deoxygenated blood flows passively through the lungs.  After this surgery Lucy's saturation's will be closer to normal in the high 90's.  The average stay is seven to ten days.  I know it will be different this time.  We are all stronger.  As strong as we have become, sometimes I wish we didn't have to do it.  That I could wave a wand and it would all go away.  But I can't, so we put our faith and trust in God knowing he will carry us through.

3 is the new 2

Our baby girl is three.  I almost can't believe it.

I had the follow-up meeting with the school district about Lucy's evaluation.  When I walked in, there was a look of disappointment across the group of therapists.......because they had wanted me to bring Lucy.  Never did a look of disappointment feel so good.  All of the therapists wanted a little more time to hang with Lucy.  It speaks volumes to her personality.  The results were very good.  She tested at age appropriate skills, with some emerging skills at 42+ months.  Needless to say, she did not qualify for any services.

We started off the month by celebrating heart month and bringing awareness to Congenital Heart Defects by wearing red.

Her birthday week began with making valentines for school.  She got yogurt with sprinkles for breakfast and got to wear the birthday hat at school.

We finished off her birthday with a visit to the bounce house at the mall, cupcakes and presents.  Connor even got in on the fun.  And in full disclosure, I was more excited about her Molly backpack (bubble guppies character) than she was.  She liked the coin purse that was inside much better.

We ended the week with a small pizza party with family.
Lucy brought new meaning to the song......It's my party and I'll cry if I want to!
Apparently when more than 3 people sing happy birthday it is just too much!

This last week is a big week for us.  We attended our last Cardiac Neurodevelopmental Clinic.  We got a great report card.  Keep in mind we stopped all therapies in September, so all progress was made on her own (working with my parents, teachers and us).  Gross motor she tested at 23 months in August, that is over 12 months of progress in 6 months.   Language (both receptive and expressive) shows 48+, the + means that the time allotted for evaluation was not enough to test her full range.  We made it up to 48 months testing questions and had to stop, so who knows how much further she could have gone.  It's exciting to see her grow so much without scheduled therapy sessions.

We will end the week with Lucy's pre Fontan heart catherization.  We will get to the hospital bright and early Friday morning as she is first case.  They will take a better look at her lungs and heart to see when surgery is needed (if she is a candidate).  We will need to stay in recovery for at least six hours and there is a possibility of an overnight.  Wish us luck that there are no surprises and the procedure goes as expected.  We'll update as soon as we get the results.

Goodbye 2016

2016 was a good year for us.
- We welcomed Connor into the family
- We graduated from speech therapy, confidently eating all food orally
- We  excelled at both Neurodevelopmental clinics, testing almost at age level in all areas
- We graduated from Early Intervention, six months early
- We managed an entire year without surgery, hospital stay or any major interruption in our daily life
- Connor started food and taught me that eating can be easy
- We are almost all sleeping through the night regularly

This year looks to be a little more trying.  We head into 2017 with the 3rd of the 3 step surgical procedure to repair Lucy's heart on the horizon.  To say we are not prepared is an understatement.  Our routine cardiology appointment ended with a Holter monitor.  Seeing Lucy hooked up with cords and a little satchel to hold the the battery pack sent me back to our hospital days.  The Holter monitor is a battery-operated portable device that measures and records heart activity continuously for 24 hours.  It has wires with five silver dollar sized electrodes attached to her chest.  It is the first step in preparing for surgery.  The echo give us a snapshot at Lucy's heart.  This device will allow us to see how it is functioning throughout a full day.   

  Our next step will be the catherization.  It will allow the doctors to get a better look at her heart function and lung pressures.  We are scheduling the procedure for the end of February.  It will be an overnight stay in the hospital and our first taste back there.  I know the amazing staff at Advocate will welcome us back with familiar faces (and hugs) and Lucy will be in great hands.
  Amidst the black cloud that is looming surgery, we still have so much to be grateful for and look forward too.  

-a third birthday

-a first birthday

-10 year anniversary

- the big 4-0

We had our school district evaluation.  I meet with the team Thursday to review the results.  It will tell us if she has any delays and would qualify for help through the school district.  I spent the hour in a separate room going over Lucy's medical history.  Who knew a medical resume would be such a handy thing!  I am excited to hear how well she did.  But I have to say, my favorite part was coming back into the evaluation room to hear the therapists tell me Lucy was one of the politest little girls they have ever met.  SO PROUD.




















Connor will be turning one in just under three months.  Oh my.  He is a pro at sitting!  He is also a pro at making very loud noises.   He is anxious to move but doesn't quite want to crawl.  I truly believe if he could skip the crawling and start walking he would.  He loves food.  I mean, he cannot open his mouth big enough and the minute he sees that bottle it is all over.  His first teeth are on the verge of popping through the top gum.  His night time sleeping is getting better and better.  We found a little trick that works wonders.  When he is a little squirmy, holding a pacifier in his hand calms him right down and puts him back to sleep.


Other fun things we did were:

Trotting at the annual Dan Gibbons Turkey Trot.  Connor experiencing the race for the first time!  Helping to feed the hungry across Dupage County

We celebrated Thanksgiving.  GG got to feed Connor.

We had a fabulous Christmas.  We hosted both eve and day and Lucy could not have loved it more.  The attention from the grandparents, aunts, uncles and cousins was her most favorite part.  Staying up later than normal was a close second.  Presents were right up there too.  Connor was not a big fan of the crowds and noise.  I have no doubt he will grow to love it as much as Lucy.

We thank all of you, who have kept us in your prayers throughout this year.  We couldn't be more blessed to have you in our lives.