broken

Day 24 broke me.  I don't know if it was getting to spend the morning with Connor doing normal mom things or the breathing tube being in longer than expected or this vicious cycle that Lucy's body is going through.  Whatever it was, the tears started on my drive back to the hospital and continued throughout the day.  When Lucy's nurses decided it was time to re-tape the breathing tube, I knew that was my cue to leave.  I did the only thing I thought would help, I went running.  It's hard to remember in times like these to make sure you are taking care of yourself as well.  Even with Lucy sedated, I find it hard to leave her bedside.  This hospital trip though, I am making a conscience effort to take breaks.

This cycle that Lucy is on, starts with the breathing tube.  We can't extubate until we remove the extra fluid hanging out around her lungs and belly,  when we amp up medicine to help rid the fluid, her blood pressure drops, then her profusion to her kidneys is low so they don't recover as quickly, so we give her more fluid, which then leaks out to her body so the tube can't come out.  It seems like we are fighting an uphill battle.

The dialysis was to help give her kidneys a break and slowly take off some of the fluid.  However, even the smallest amounts were causing drops in blood pressure.  Friday, we stopped removing fluid through the CRRT and just focused on giving her kidneys a rest.  Early Saturday morning the CRRT machine alarmed with a clot.  The nephrologist was consulted and she recommended to turn off dialysis and run TPA (clot dissolving medication) through the line.  Once that was done, they would check access to ensure the line was clear.  Since the machine was off they gave Lucy's kidneys a test run.  Today, they would watch output and evaluate in the morning.  If her kidney labs look good and she consistently pees throughout the night, they may add lasix tomorrow to see if we can pee out some of the extra liquid.  If they see a decline in the output or her labs look worse we'll go back on dialysis.

Maybe part of my breakdown is that it feels like a week has gone by and there doesn't seem to be a lot of progress.  I was told today "her body looks worse on the outside, that it does the inside".  As much as her body looks broken from the outside, I have to remember each day we are taking baby steps to get the inside healed.

If you are praying with us, pray specifically for
-Strength, strength and more strength for Mike and I.
-For Lucy's team to figure out how to remove this fluid safely so we can get that breathing tube out.
-For Connor to continue to be an easy toddler as he bunks full time with my parents.

next stop dialysis

With matters of Lucy's heart and physiology, I am almost an expert.  But when you throw in thrombolysis and dialysis and it takes me some time to process.  Even with a day or so, I still have a bunch of questions.

Tuesday afternoon Dr. Patel did the angiogram and confirmed that the EKOS had broken up the clot.  In nineteen hours the clot was completely dissolved.  Lucy was removed from the machine and we were prepared to move forward with extubating.  This would entail allowing the paralytic medication to run it's course through her body and her breathing to resume.  The ventilator setting would be decreased as Lucy started to initiate breaths.  Eventually the vent would be just support as Lucy would breath on her own.

As we waited, Lucy urine output began to decrease and her plural effusion began to increase.
Fontan patients do better extubated because the positive pressure from the ventilation interferes with the blood flow through the Fontan.  With excess liquid around her lungs,  they were unsure that she could handle extubation.  A chest tube was place on left side to help remove excess fluid.
However, her kidneys were having trouble recovering.  The team brought the nephrologist back in to consult on her kidneys.  What we have learned is there are three things that impacted her kidney:

1.  Dye used in the angiogram, which was necessary

2.  Antibiotics to get ahead of a fever and ensure she stay healthy

3.  Low blood pressure.  

If you can remember, way back when Lucy was an itty bitty, prior to our first discharge we were having trouble with digesting bolus feeds.  This was due to her vital organs needing the best oxygenated blood and the stomach was that last to get good blood.  This is some of the same now, except her low blood pressure is the problem.  It is not allowing good profusion to the kidneys to get the adequate blood flow they need to recover and function correctly.  Our first line of defense is to flush out the dye used in the angiogram and second would be to adjust medication to increase blood pressure.  After rounds, the word dialysis was floated by as a last resort to help the kidneys.  At first it sounded like we were days away from this.  Dr. Nater did everything we could to rid Lucy's body of fluid, even placing a third chest tube, but around 6 pm she came into the room to tell us they were going to start dialysis.  CRRT (continuous renal replacement therapy) is used to treat critically ill patients who develop acute kidney injury.  The blood is removed from the body, waste is filtered, then  replacement fluid is added and returned to the body.  To start we were only filtering but as Lucy is able to handle we will slowly start to remove fluid to help with the effusions.

Our plan for the next few days is to slowly remove as much fluid as we can from her body.  Once we get to a point where we can successfully extubate, we will bring her off sedation and take out the breathing tube.  My hope is that can be done by this weekend. The dialysis has thrown a curve ball at our dosing of pain and sedation, so we continue to manage and increase as she shows signs of waking up or pain.  I believe we will also be on dialysis for another two days.  While we are seeing the creatinine normalize, her kidneys still need time.  Following that we will start to look at chest tube drainage and then finally move on to revisiting fenestration.  Luckily we are showing no signs of impact to her heart.

I have asked if we should be concerned about her recovery and their response was that they feel she will make a full recovery, but it will be very slow.  I have a feeling we will be here through the end of June and into July.

CHD sucks

I hate CHD.  I hate that her future is a big question mark.  I hate that transplant is a possibility.  I hate that I think about what would happen if we lost Lucy to this disease.  I hate the unpredictability.
When you can go from this:

To this in a matter of six hours:

But with the bad come the good.  And we have a had A LOT of good: two years free of hospital, no developmental delays and a healthy brother (which Lucy does not like all the time).  And we do not take any of it for granted, even the tantrums!

Yesterday Lucy went to the cath lab for what originally was diagnostic.  Prior to walking her down, Dr. Patel told me that they planned to stent her left pulmonary artery hoping to lower her lung pressures.  Around noon, the nurse call to say the stent was placed and everything went according to plan.  Usually she would be up in her room about an hour later.  When 2 pm came and went, I started to worry.  Shortly after, Lucy's nurse came in and said they found a blood clot.  To break up the clot, Lucy was placed on EKOS (catheter assisted thrombolysis).

Here's how it works:

a catheter is guided through blood vessels to the location of the blood clot.  The catheter will deliver special clot dissolving medicine called thrombolytics to help dissolve the clot,  The new device called EKOS, will also deliver simultaneously ultrasound energy to mechanically dislodge and loosen up the clot to enable lower and safer doses of the thrombolytic drugs to be used (credit: Brigid Shelton).

The ultrasound helps to disperse the drug allowing us to use a lower dose.  This machine also speeds up the time it takes for the clot to dissolve.

The catheter was placed in her neck.

In order to keep it in place, it was secured along her entire torso.  They also have her on sedation, pain and a paralytic medicine to keep her comfortable and still. 

The EKOS machine can run anywhere from 24 to 48 hours.  After the cath lab, her left leg appeared blotchy and they were worried of a possible clot.  Also because of the medication there is a possibility of bleeds.  They did ultrasounds of her leg and abdomen to ensure everything looked okay. 

 Overnight Lucy had some issues with blood sugars, blood pressures and heart arrhythmia's.  Her medication was adjustd to help with arrhythmia and blood pressure and some insulin was given to regulate her blood sugar.

Today Dr. Patel will do an angiogram bedside to check if the clot has been dissolved.  If it has, they will remove the EKOS machine.  Then re-evaluate the plan for today.  Once the machine is removed, the next step would be to ween her off ventilation and then bring her off her paralytic and sedation medications.

While some of these pictures may seem as if Lucy is in critical condition, the doctors have assured me she is stable.  Due to the turn of events, we are back at square one.  We will have to start diuresing while watching sodium, potassium, calcium and creatinine levels.  I am unsure how this will impact surgery, but we will keep updating as we have information.

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!!!