our first month and first biopsy

One of the first things they tell you when you are being evaluated for heart transplant is the wait can be anywhere from six to eight months.  Within the first few weeks of moving to Lurie Children's we met a couple of families who were through the evaluation stage and onto the waiting stage.  Some were there less than two months and others were there going on six months.  Then in a whirlwind two weeks, they all got matched.  Excitedly, we watched the recovery process unfold.  It was amazing to see that in two or so weeks many were on their way to the transplant floor at Ronald McDonald House (explanation coming).  When we were notified that Lucy had been matched to a heart, I couldn't help but think, new heart = better, faster recovery.

BEFORE     |     AFTER

There were two things I failed to realize.  The first, our friends before us, had waited longer for their match, which gave them some extra time to get in the best possible health for this surgery.  The second, Lucy had not fully recovered from her Fontan and had sub par nutrition for almost a year.
Needless to say, Lucy's recovery would take a little longer.

Post surgery Lucy remained intubated for two days, because her lungs were a little wet and that has always impacted her breathing tremendously.  By day three, she was ready to get that tube out.  During extubation, Lucy dropped her saturations to the 40's.  She remained NPO the remainder of the day in case a re-intubation was necessary.  High flow at 20 liters was providing some extra support.

Her first biopsy was set for 2 weeks post transplant, with compromised lungs we were proceeding cautiously  The team had been discussing BiPAP as the next level support.  Lucy never had to wear a mask like this, so we started practicing wearing it to get comfortable.  Ten minutes here and there in hopes that we would not need it.  We carried on with as normal of schedule as possible, including physical therapy sessions.  Get a load of her first walk with a new heart!

As we approached two weeks and our first biopsy, our battle with fluid continued.  We struggled to find the right oral regimen and her x-ray definitely showed that.  Going into the weekend her baseline cough was back effecting her oxygen saturation.  As her x-ray worsened, the team thought a switched back to IV diuretics was necessary.  The night before the biopsy things got a whole lot worse.  Her cough kept her up the entire night and she was dropping saturations into the low 70's.  Tuesday night they cultured her and the result:  Positive for rhino enterovirus.  Biopsy postponed!

A virus, with an already compromised immune system, what luck.  This virus took every ounce of energy from her.  She wasn't sleeping because the cough kept her up and her work of breathing had her dropping saturations, so much so she was up to 20 liters of support with the help of nitric. By Thursday, I had this feeling she would be intubated by the end of the day.  During rounds we decided to give BiPAP a go and man am I glad we did.  She got that mask on and slept for 7 straight hours.  This virus would run its course in ten to fourteen days.  The team postponed the cath for another two weeks and would monitor rejection by echocardiogram.  They decreased her rejection medications to allow her to fight a little better.  I couldn't help but wonder how this would impact the results of the biopsy.

She wore the BiPAP mask for two straight days with two one hour breaks during the day, then we moved to using it solely at night with oxygen support during the day. The cath was rescheduled for May 7th (almost one month post transplant).  We tried once more to move to oral diuretics, however, two days later she was puffy and uncomfortable.  The team upped their game and we went straight to IV bumex (stronger diuretic than lasix) to ensure we would not postpone the cath.  Our first biopsy went better than expected.  We thought she would be grumpy and out of it most of the day.  It was opposite: happy and playful!

We got full results back the following day:  ZERO REJECTION.  We are so thankful for all the prayers and especially thankful for our donor family.

her beautiful new heart

Today marks four weeks since our lives made a turn for the better.  A lot has happened in that time.
Lucy sure does know how to keep us all on our toes.  The biggest difference between congenital heart defect and transplant, surprisingly is there is some planning that goes into each surgery whereas with transplant you get a match and everything is on fast forward.

April 10th will be a day I never forget.  I was in the family great room of the hospital working like usual.  Mike had just gotten up to the hospital.  As I was finishing a call and beginning to pack up my laptop, Mike called me to ask if I could come back to the room.  He never does that. I walked back to Lucy's room, my stomach was sick with fear.  Dr. Joong (transplant cardiologist) and Kristi (transplant APN) are waiting with Mike and she says, 'can we go into the conference room to talk?'.  The four of us are standing in the conference room, I was to nervous to sit.  Dr. Joong and Kristi look at each other and then Dr. Joong says, 'we found a heart'.  I burst into tears.  She followed it up, saying it's a good match and I think Lucy will do well with this heart.

Our last eleven months have been hard and I know Lucy has not felt good in a really long time.  This blessing of a new heart came just at the right time.

The month prior to our fantastic news, we had been planning how to live at the hospital for eight months or more.  Let's face it, Lucy has always made us wait a little longer.  From our experience eight months was most likely on the short side.  We were deciding if we should sell a car.  We looked at possibly moving closer to public transportation to help with commuting.  We had a schedule of who could be with Lucy (and Connor) so both of us could continue to work.  Life happens when you are busy planning, isn't that how the saying goes?

We were told after a match is accepted, it usually takes about twelve to twenty four hours to get everything in line (meaning matches with other organs), so expect Lucy to go to the operating room around 2 pm the following day (April 11th, my birthday).  Our first order of business, telling Lucy.  The benefit of her age, she didn't really get that a new heart meant surgery (and another recovery).  The downfall, her reaction and excitement was not quite up to the level we had hoped.

After immediate family came to visit Wednesday morning, Mike and had planned to hang out with Lucy and walk her down to the OR late afternoon.  Around 2 pm, Dr. Joong came into the room and said there was a delay with our donor.  The delay meant that Lucy would not go to the operating room until close to 11 pm that night.  My first question was if Lucy could have some water.  The answer was yes, until about 6 pm.  When Lucy heard the news, she burst into tears.  Dr. Joong and I looked at each other and thought that was the reaction we were looking for with the news of her new heart.  Oh, but water is so much more important.

That night was a blur....if it wasn't for text messages, phone logs and facebook updates, I probably wouldn't have a timeline of what happened at all.  By 8 pm, Lucy had fallen asleep and Mike and I moved to the family great room for the remainder of the night.  The love seats although comfortable, were definitely not made for a night of sleeping.  We were in and out of sleep until about midnight when our nurse called to say they were ready to take her to the operating room.  We got calls every two to three hours with updates.  At roughly 8 am, we were told her heart was in and beating on it's own.  It would be another six hours before we would get to see her.  By 4 pm, I could barely keep my eyes open.  We both left Lucy in the capable hands of her nurse and got some much needed sleep.

cath and lowering oxygen

The other night I am sitting at the Ronald McDonald House talking to Mike about heart transplant and getting a heart and everything that it entails.  And we both say, "I can't even believe this is a conversation we are having right now!"  Not in the sense that we can't believe our circumstances, but that starting a family and having kids would ever lead down this road.  Heart transplant.  I have said it a million times.  Holy crap.  We are four weeks in and it's all still very surreal.

We are slowly starting to get into a schedule.  For most of Lucy's hospital stays I have been able to be on leave or use vacation time, but with the time it take to receive a heart, right now that just isn't an option.  This will be the first time we'll be in the hospital for an extended period of time where I will have to juggle work and the hospital (Mike's been doing it from the get go and I give him major props!!).  I am forever grateful to have two wonderful bosses that have worked with me to make a schedule that benefits both of us.  My office is in the loop and I have been working remotely since Lucy arrived.  Recently there had been talk of consolidating two city offices and moving to the Merchandise Mart.   The move would have extended my walk from the train maybe another 5-10 minutes.  I desperately did not want that to happen because when I started going back into the office (completely arbitrary date) I didn't want a longer walk.  Flash forward to our transfer to Lurie Children's hospital.  I am now going into the office once a week and the Merchandise mart is half mile closer than my current office.  Oh how that move would have benefited me now and it just goes to show you, nothing turns out how might expect.

Two weeks ago Lucy went for her catherization.  A couple people asked why she had to go for another one (she had one beginning of March before our transfer).  The first one was just diagnostic.  Lucy was in no shape to handle any intervention, so they just wanted to get her lung pressures.  After being on milrinone for two weeks, she was in much better shape.  Their plan was to balloon the stent in her left pulmonary artery and coil of some collateral vessels.  She went down to the cath lab around 2:30 and was down there until almost 6 pm.  They were not able to balloon the stent, but instead had to do a second stent inside the original.  Five collateral vessels were coiled as well (3 on her left and 2 on her right).  Collateral vessels are abnormal blood vessels that connect the aorta with the pulmonary arteries.  The human body is so smart, it recognizes that Lucy's blood is below normal oxygen saturation.  It creates these vessels to get more blood to her lungs to help increase the oxygen.  However, with her physiology, they were flooding her lungs with blood increasing the lung pressure.  It's another vicious cycle.  Lung pressure is high, so the blood takes the path of least resistance, her fenestration to her heart.  The heart then pumps that blue blood to her body, through the aorta.  The collateral vessels, pull some of the blood and push is back to the lungs.   The coiling of these collateral vessels will allow her fontan physiology to work and bring up her oxygen saturation.

Unfortunately in the last week, we have seen the opposite effect.  Her saturations have been dropping slowly.  She has been resting between 65 and 68 percent with a couple significant drops while sleeping to the 50's.  We have moved back to high flow and last Tuesday nitric was added.  The bad part about all of this, it makes it really hard to leave the room for walks.  Lucy has again stumped us.  We are looking to maximize her pulmonary medication and slightly increase diuretics to see if that will help.  At this time we are not sure if any other procedures have benefits that will out weigh the risks.  We are praying that God will help to lift her oxygen saturations and she can come off nitric and high flow.  We just want her to be able to move about the unit and get physically stronger as she awaits her new heart.  If you pray, pray with us please!


Two extra things!
First we want to thank everyone who has donated to our youcaring page.    We are so grateful and already feel relieved that when Lucy's new heart arrives we won't have to think twice about taking unpaid leave from work.  I say this often, but we are truly blessed to have so many people that love, support and pray with us.   https://www.youcaring.com/lucyshannon-1141532


Second, this is a post from Lucy's new Facebook page: At the Heart of Lucy.  https://m.facebook.com/heartoflucy/
If you missed it, we are starting a project, creating a book of friends.   If you are so inclined, see the below and help us fill her book!

📣 📣 📣 Project Alert 📣 📣 📣

****************EDITED****************
Please print photo and send in mail.  Do not send via email or messenger.  Part of the fun for Lucy will be opening the mail!
****************************************
Lucy loves to look at pictures on our phones.  Since they are not always available, I want to put together photo albums of all her friends.

Here is where we need your help!!!!
1.  Print a 4x6 picture of you, your family or your kids
2. On the back of the photo list the following
   -name and ages of people
   -city and state you live in
   -what you are doing in the photo or a fun   
      fact about you
3.  Send to
      Ronald McDonald House
      C/o Betsy Shannon
      211 E Grand Ave room 1102
      Chicago, IL 60611
4.  Share and tag friends....the more people that see this, the bigger Lucy’s friends book can be!!!!

Each day we’ll open one or two photos and add them to our book. 

We’ll thank everyone in advance for sending photos!!

!

Sacrifice

Having a child with a chronic illness can mean a lot of sacrifice.
You sacrifice family time
You sacrifice your marriage
You sacrifice your friendships
You sacrifice your job
You sacrifice fun
You sacrifice vacation
You sacrifice your home
You sacrifice comfort

When you look at that long list of sacrifices it's hard not to be angry and ask 'why us?'  From diagnosis at 20 some weeks gestation to now, I have asked that a lot, why us?  But rarely do I find myself truly angry at our circumstances.  Yes, it stinks to live in hospital and sacrifice but I am not in control.  My strength and patience are due to that alone.....I AM NOT IN CONTROL.  Someone far greater than me is in control.  As we venture into a different kind of hospital stay I think I will be truly tested with both.  The majority of our other stays have started with a surgery and moved onto recovery where small steps are made to getting healthy enough to go home.  Our last two weeks have been getting Lucy in the healthiest state and then we wait and wait some more and wait some more.
Hearing that patients in need of a heart transplant typically wait six to eight months.  That would put us right around Christmas.  Oye.

When we were in route to Lurie's, I was scared out of my mind, the quick turn of events didn't leave a lot of time for processing all that was happening.  In that ambulance ride the only thing I could think to do was message a heart mom who had been down this road and ask for words of wisdom.
Among them she said:
 "Remain present both with your circumstances and in prayer.  Like really, never stop praying."

How true!  If you just focus on the day in front of you, the overwhelming feeling to not know anything of the future fades.  To be able to focus on just the present day, you need prayer, A LOT of prayer, because your mind can so easily wonder down dark paths.

The last week has been more talking. In case you were wondering, I missed a few specialties that have been by to speak with us:
Palliative care
Hepatology (liver)
Pulmonology
Nephrology (kidney)
Infectious Disease
Chaplin

So much talking!  We have been making small changes to get Lucy to her healthiest state.  We are down to two liters of oxygen and we've weaned a medication (bosentan - pulmonary hypertension medication).  Lucy's demeanor has gotten better each day since we arrived,  Her smiles are back, her giggles are back, her sassiness is back.  This is mostly due to the milrinone (in short, it's a medication that helps with the pumping of the heart) and in part that she has been left alone.  I honestly think this is the best she has felt since last May.  She has been a riot, just chit chatting the day away!  Aside from living in the hospital, I have enjoyed seeing her personality re-emerge.

While making these changes, the team has been back and forth about doing another catherization, weighing the risks and benefits.   With her in a stable state, the team felt another catherization to coil some collateral vessels would be helpful.  We were looking to schedule next week, but of course, the week is full.  So today it is and in true fashion, it's not a definite if we will end up actually going.  We are 4th case, which means roughly 2 pm.  We will be NPO (no food or drink) starting at 2 am.  If we are lucky, one case gets cancelled and we are able to go sooner.  If we are unlucky, one or more cases will take long and they will cancel us after not eating and drinking for most of the day.
We hope that this will improve her health even more and we can work to strengthen her muscles, but another procedure, even a catherization is scary.  I always talk to Lucy about what is happening, so last night, I told we were going to have another procedure and that she couldn't eat or drink before and.  She immediately burst into tears and asked who was going with her.  I told her I would be there,

Bless her heart!  This journey is much harder now that Lucy has a better understanding of everything.

Specific prayers that this catherization does not set us back, that any intervention helps and that both her and I are alive after not being able to drink or eat all day!  Yes, I try not to eat and drink while she is unable.

transplant evaluation

I seriously sometimes think this is a dream.  That it is not really happening.  But then I realize, we've spent the last week in the hospital and I haven't seen Connor this whole time and I know it's more like a nightmare.

Surprising to me, transplant evaluation is more talking than anything else.  I thought Lucy would go through a bunch of procedures, but since we got here Friday night, it's only been two.  She had a PICC line placed and had a CT scan.  The rest of the time has been meeting with the people that make up the transplant team.  This includes (but is not limited, as we haven't met the entire team):
Transplant surgeons
Transplant cardiologist
Fellows and residents (it's a teaching hospital)
Transplant APN
Cardiac nurses
Social work
Psychology
Hemotology
Transplant case manager
Occupational Therapy
Physical Therapy
Speech Therapy
Child Life
Education
Finance

One of our first questions when we sat down with the transplant cardiologist was:  'is Lucy going to be a candidate?'  In simple terms, from all initial data:  yes.  Although we have not gotten official word.  At this time we have stated to the team we would like to move forward with listing Lucy for transplant.

We are still learning about this process but here is what I know right now:
Once listed with UNOS , there are 4 statuses of wait list:
1A - child is admitted to the hospital and in need of support for help stabilize
1B - child is admitted or waiting at home depending on medical care needed
2 - child is stable but will eventually need a transplant
7 - child is temporarily unable to receive a transplant (too sick, work up incomplete, etc)

Don't ask why the 4th category is seven.  It doesn't matter because Lucy will be listed as 1A.  That means we are not leaving the hospital until she is matched with a heart and has her transplant.
Average wait time is roughly 3-4 months, however numbers have indicated wait time is increasing and 6-8 months is more reasonable.  Which means in Lucy time, we're looking at possibly year or more to wait.  We could get lucky and get a heart quickly but if history has told us anything, it's to be prepared for the long haul.

The transplant list is also not just a list where you get a number, say we're number 100.  And each time a heart is available and matched, we move up in line .  There are many factors that go into finding a heart: blood type, size, distance of donor and sickness of child.  The team has also told us that their goal is to find the best possible match, so a heart may be matched to Lucy and the team can in essence 'pass' on the heart.  Their goal is to make sure Lucy thrives after transplant and that can only be done with a best fit.  Once Lucy has been matched, the team would review all the information from the donor and if a good match, they would verbally accept the heart.  I believe at this point we would get the phone call that a heart is available (did you're heart drop too?).  The transplant surgeon would then retrieve the heart personally.  Once there, they inspect the heart.  At this point they can still decline, so we also will need to be prepared for false alarms.

Many have reached out to us and family about ways to help.  We are still figuring out ways of help that will be most beneficial for our family.  While we work to create a new normal for our whole family, we ask for prayers of a new heart.  This request is not lost on me.  I realize that in order for Lucy to live and thrive another family has to endure a fate far worse.  And in that dark time, they choose to be selfless.  So along with prayers for that new heart, I would like to add a prayer of peace and comfort for that family that will eventually give us our life back.

unknown

If you could know what the future holds would you find out?   If you did, would it change how you lived right now?  I've always said I hate the unknown, and that if I could know it now, I would want to.  It's why with both kids, as soon the gender was available, we wanted to know.  I'm a planner, I've said it before.  Knowing means being able to plan.  Unfortunately, CHD means never planning.

I never mind the journey as long as I know the outcome and for the most part I would not change how I lived.  Take for instance Mike and I, I knew I would marry him, right from the start.  But it took two break ups and a move across country to get us there.  That was okay, because I knew!  With Lucy, there hasn't been a lot of 'knowns'.  But if there were, I can say I would changed how I lived at least in one instance.  I would have delayed her fontan, to enjoy one more summer of swimming at the pool, playing at the park and enjoying outside.

Will travel for milk!

Since early December we have been working to clear up small issues that we felt were impacting Lucy's fontan physiology.  The first was her lungs and aspiration.  Our second injection in January has worked wonders and she has been taking all food and drink by mouth with no impact to her lungs.  Then we focused on her GI issues.  At our last inpatient stay, we were able to complete a gastric emptying study.  The results came back normal and we have been puke free since mid February.

However, Lucy's declining oxygen saturations have been of some concern and with no signs of distress (increased breathing or retractions) we were scratching our heads.  Dr. Husayni felt that she was healthy enough for a catherization, so we planned to schedule one mid March.  Then last Tuesday, I put her pulse ox on for her morning spot check and it read 49.  YES, 49!  We called Dr. Husayni immediately and after some discussion we felt that pushing up the catherization would give us the answers we needed.


Tuesday afternoon we waited for a bed to open up and drove down to Advocate Children's hospital.  Lucy was put on high flow to help bring up her 02 levels.  We were scheduled for the catherization Wednesday afternoon.  A couple hours before, they decided to postpone a day to bring her INR level down.  Since this was unplanned we did not have enough time to hold her warfarin the recommended 5 days.  She was given FFP (fresh frozen plasma).  FFP is a blood product made from liquid portion of whole blood.  It is to help with her low clotting factors due to her warfarin regimen.  They added some extra diuretics to remove excess fluid on her lungs to ensure the data from the cath was as accurate as possible.  Once we knew she could eat, Lucy got right to drinking milk.  She chugged (2 mL at a time) almost 16 ounces in about 2 hours.




She was rescheduled for first case Thursday morning.  The cath took about 3 hours.  When Dr. Patel came to get us, I got this feeling in the pit of my stomach.  We walked back to the PSHU and sat down with the entire team, so everyone could here the results at the same time.  Sure enough, the cath results showed her lung pressures were high.  He went over more information and then showed some scans from the cath.  Her right lung was barely getting blood flow and her heart function had decreased.  We went back to Lucy's room to find her still intubated.  She was still really groggy from the anesthesia.  As she started to wake up, her first instinct was to pull that tube right out (I don't blame her).  Within an hour or two we were able to extubate.

Around 6 pm, Dr Husayni came to talk to me about our course of action.  Full disclosure, I knew we were headed for transplant evaluation, but I honestly thought we would get discharged and the process was going to take a couple months.  We moved to the waiting room for some privacy.  He went over the results again, but the one thing that stuck out was her low oxygen levels over the last couple months have resulted in early heart failure.  He had spoken with the team and Lurie Children's Hospital and they suggested a hospital to hospital transfer was best for Lucy and insurance.
"So do you want to go tomorrow?  And just like that.....everything changed.
We thought for Lucy, spending the weekend at Lurie's to get adjusted was going to best, so we started the process for transfer to happen Friday.  Our last night at Advocate Children's hospital had to be special, so Lucy had a princess dinner!  Thank you Dr. Penk for stepping in, as no real princesses were available last minute!

Live every day like it's your last.
Many times you see that saying in the wake of a death.  And it's probably true that enjoying everyday is the best way to live.  But that's easier said than done, especially in daily life.  While no one has passed, our life as we knew it pre-Fontan has died and it is a reality, that it will never be the same.  Does that mean it can't get better? No.  However, everything going forward is unknown and that is scary.

perspective

Monday I was having a pity party.  I am sick of the hospital, so is Lucy.  I am tired of Lucy being sick.  Let's be honest, she hasn't been healthy since last May.  Yes, we have good days, but overall Lucy just doesn't feel good which leads to a lot of crying.  This hospital stay, my patience are at an all time low.  I find myself getting annoyed when Lucy is extra 'needy' or is struggling to get comfortable enough to fall asleep.  Then I hate myself for it because she can't help it.  How can a four year cope with everything that's happened in the last year and here I am annoyed?

Then yesterday, from a distance, I watched two parents make a decision to say goodbye to their child.  Siblings and extended family came to comfort and say final goodbyes.  And then they left without their child.  Leaving a void that will never be filled.  My heart ached for them.  My mind easily went to "what if that was us?"

Man, does a day like yesterday change your perspective.  Lucy is alive and although she is not healthy, she is not nearly as sick as she has been.  And I thank God that she is here to annoy me and remind me that when my patience are short, I need to lean on him.

I probably should have foreseen this hospital stay.  About two weeks ago I was checking her saturations and she was resting in the mid sixties.  I immediately called Dr. Husayni's office and after sending a video of her breathing we all agreed that bumping her up to four liters for some extra support was fine.  At her appointment that same week, she showed no signs of distress and she was actually a little silly during our visit.  Then Friday Connor woke up with pink eye and a cough.  Within a day Lucy had all the symptoms too.

Last Tuesday afternoon I noticed Lucy's respiratory rate had increased and that night we were up most of the night coughing.  By Wednesday the cough had gotten even worse and she started retracting.  We spoke with Dr. Husayni again and he did not want to wait until our Thursday appointment, so we made the trip to the ER.  In the ER we drew a blood for a virus panel and electrolytes then tested for the flu.  We headed up to the PSHU that evening on high flow oxygen at 10 liters to watch.  Even on 10 liters during coughing fits she would drop her saturations to the low sixties.  Over the weekend we bounced around on flow, down to three then back up to fifteen and anywhere in between.  They put her on a stronger antibiotic to cover  aspiration and anything that may show up.  However, all tests came back negative.  The likelihood was that it was a virus that did not show up on the panel and we would just have to let it run it's course.

One thing the team was worried about was the injection had worn off and Lucy was again aspirating.
Dr. Sherman stopped up yesterday evening to scope her and look at her vocal cords.  Thankfully what he saw was good news.  She is still getting very good closure.  So we do not have to restrict oral intake!!!!!!  This confirmed that we are probably dealing with a virus.

This morning we moved off high flow to a regular nasal cannula.  We will need 24 hours with no episodes and then we can talk discharge.  While we are inpatient though we decided to go ahead with gastric emptying study to see her stomach motility.   This should give us an idea of what is going on with her stomach and help us to determine if something can be done about the throwing up.



She hasn't been super upbeat while we have been here and that's understandable, but SpongeBob SquarePants made a visit to the unit and we got the biggest smile.






Music therapy and a mustache party have helped a bit, but we really can't wait to get home!

Chaos

If I could describe what life has been like since G tube surgery, it would be chaos.  I am a creature of routine and when I don't have one, it feels like I am spinning.  It's hard to focus on anything.  Each day, hour, minute, it seems there's a list of things to do, but nothing ever gets done.  And it's probably why I have started this post a dozen of times.

Prior to g tube surgery, we had gotten into a nice routine.  Lucy medication and feeding schedule was set, both kids were for the most part sleeping through the night.  I had adjusted my work hours to accommodate Lucy and it allowed me to go to the gym a couple days a week.  Oh how I forgot how therapeutic running was for me.  But after surgery, everything seemed to turn upside down.

During Lucy's recovery over the summer from her Fontan, I had been asked multiple times if this was her last scheduled surgery.  My answer has always been the same:  'Yes, it's the last planned surgery'.
I say that because CHD is unpredictable and for the majority of people with congenital heart defects a transplant will eventually be necessary.  Transplant.  It's hard to describe my feelings when I hear that word.  Prior to the Fontan, it seemed like a distant conversation.  However, during Lucy's December 22nd appointment, I sat in Dr. Husayni's office looking at her awful x-ray and he said it.  We need to start thinking about transplant evaluation.  You think you can prepare yourself.....because transplant is not an 'if' it's a 'when'.  But you're never ready....cue the tears.

Why are we talking about it now?  Most of it stems from diuretics.  We have tried to ween her from the massive amounts of diuretics and have been unsuccessful twice.  We have to start thinking into the future and what it will mean if Lucy cannot handle the Fontan physiology without diuretics.  A transplant evaluation is one of the first steps.  Luckily, her team felt that there were a couple other issues that are impacting the Fontan.  The first and biggest is her aspiration and lung issues.  In simple terms:

-aspiration causes aspiration pneumonia
-pneumonia increases her lung pressures
-high lung pressure creates venous congestion
 *venous blood flow is deoxygenated blood coming back to the lungs.
-Venous congestion means the fluid looks for other places to move

This can mean fluid leaking through the lymphatic system, creating more pressure on the lungs or higher use of the fenestration, which means lower oxygen saturation (and in turn lower profusion to organs).

The second issue is her gut.  Since surgery in May she has been getting less than optimal nutrition.  We all know good nutrition fuels healing.  We were hopeful the g tube would assist in reducing the vomiting, but it's still a once a day thing.

At the beginning of January, her medical team (cardiologist, surgeons, intensivists, APN's, ENT) gathered to discuss Lucy and where we could go from here.  What I wrote above was a majority of what they discussed (but probably with more sophisticated words!).  What came out of it was a plan to eliminate the issues impacting the Fontan using minimally invasive procedures.  As you know, the first procedure was a second injection using a more permanent material.  As with the first injection, we don't know how long it will last (anywhere from 1 month to a year).  We can continue to do injections, but it is not a long term solution, so we are also looking into vocal cord regeneration.

Our second procedure will be a gastric emptying test (not scheduled yet).  This will allow us to see how her stomach is functioning.  Feeding is where I feel most of the chaos comes from.  Since December 6th (our discharge from surgery) we have been adjusting her feeds constantly.  I was so focused on getting her all of her nutritional needs that at first I didn't realize it might be better to only give her what she can keep down.  Add to that some restriction from drinking (at first) and trying to keep her liquid volume around 33 ounces.  I did everything. 6 feeds over an hour, small feeds every 3 hours, half her food via tube and the rest by mouth.  It was almost all consuming.  And the constant waiting for her to vomit, also polarizing.  While we are still vomiting, we have a routine that seems to suit Lucy's need to drink, while getting a majority of her calories.

She gets three ounces of milk/pediasure (alternating) every hour.  She gets tube fed to give her potassium.  The dose is rather high and can upset the stomach, so we give it slowly over 45 minutes.
If she doesn't drink all her allotted liquids, we make up the differences with overnight feeds at one ounce an hour.  It seems that after two months of trial and error we are starting to hit our stride again.
Just in time for Lucy's 4th birthday!

I think my biggest fear is the knowledge that the injection will wear off.  When that happens, will she silently start aspirating again and will we need to go back up on her medications.  There is a possibility her vocal cord never heals and I'm not sure where that would leave us.  It's been three weeks since the injection and we have been able to ween the diuretics a good amount.

So right now, we focus on the good and pray for the following:
~healing for her vocal cords
~increase in appetite so she can eat more calories
~that her body can continue to handle less diuretics

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

G tube here we come

Here we go, prepping for another surgery, another hospital stay and another chance to showcase Lucy's strength.  Surgery is officially scheduled for next Monday, the 27th.  Deep breath.  These past couple weeks we have been meeting with some new doctors and discussing plans for this surgery. 

First we met with Dr. Smith (GI doctor) to discuss her history, talk about her feeding schedule and what issues we had with her stomach during the last hospital stay.  The evaluation was minimally invasive as the most he did was take a look at her belly and listen.  We discussed GJ tube versus G tube and the complications that come along with surgery.  Then ended the evaluation with a follow up phone call after some discussions with cardiology.

Two days later we had our pre-op appointment with Dr. Chokshi (pediatric surgeon).  He came recommended and at first meeting, I could tell I was going to like him.  Megan (RN for general surgery) was a familiar face from back in 2014 when we spent two weeks in the step down unit.  Their demeanor through our appointment put me at ease as we discussed our surgery options.  The big theme I was getting from both appointments is that nobody wants Lucy to have the GJ tube.  It comes with many complications post-op and can extend surgery time.  But there are also reservations regarding the G tube:  her throwing up, she hasn't been fed to her stomach since May and her stomach's motility.  So, what happens?  We go for an immediate upper GI.  Radiology was able to squeeze us in immediately.  Thanks a bunch, wink, wink.  Remember last post when I said the ENT evaluation was bad.  THAT.  WAS.  NOTHING!

The upper GI consists of drinking about 2 ounces of Barium, then filming how the liquid moves through the digestive tract.  From her swallow study, I knew she wouldn't be able to drink it (because of the taste she immediately threw it up), which meant we would have to place an NG tube to get the barium to her stomach.  She flailed and screamed and cried her eyes out and it was really hard to watch her go through it.  I try hard to never cry while it's happening, to talk her through it, stay calm and reassure her that it will only be for a little bit.  I've tried to adopt a saying 'we can do anything for just a little bit'.  I am not sure if it has helped, but I will say, Lucy always ALWAYS bounces back so quickly.  Most of the time I do too, honestly most of my tears come after the fact, talking about it (or recapping here).

The results of the upper GI were good.  They showed her stomach emptied rather quickly and gave Dr. Chokshi some data to discuss options.  Last Thursday Megan called to tell me everyone wanted to do a NG feeding trial to see what her stomach can handle.

Our first option was a week trial with the NG tube.   Absolutely not.  If she was not able to handle it and threw up most of what we fed her, that would leave us with a week without food heading into surgery.

Next option, admit to the hospital on Friday (11/17), pull NJ, place NG and trial feeds over the weekend.  If feeds go well, go home with NG tube.  If they do not, place the NJ on Monday and go home.  Absolutely not.  Placing the NJ tube was painful.  Her current NJ tube took three hours to place.  When we pull the NJ tube, we are pulling it for the last time.

Last option, admit this Friday (11/24), NG trial over the weekend and go to surgery Monday morning. I asked if we could do this at home.  If she was not able to handle it, I would bring her in immediately for admit so we could get her fluids before surgery.  They obliged.

I am thankful to have a voice for Lucy and that our doctors take to heart what we talk about.

This Thursday we will also be thankful for all we have overcome and Friday will start our next journey.  Friday morning we will give Lucy's morning meds, then pull her NJ tube.  We'll give her the day to enjoy and then early evening we will place the NG tube and start feeds overnight.  I am starting with a lower volume to test her and then will slowly increase over the course of the weekend.  Wish us luck!

Our specific prayer requests are
~Our home trial works and we do not have to be admitted early for IV fluids.
~The trial determines that we can move forward with the less risky G tube.
~Surgery and recovery is as close to standard as possible.
~An open bed in the PSHU, because of Lucy's anxiety, I'd like her to be in a familiar place.  If no beds are available, it would mean a bed in the PICU (i.e. new faces)

Never have I ever

'Never Have I Ever' is a drinking game I played in college.  The gist is you follow up that phrase with an action and if you have done the action you have to drink.

   Never have I ever gotten lost ..........drink
   Never have I ever been arrested.......drink
   Never have I ever thrown up in public.....drink

You get the idea.  As a mom, I have said a bunch of time, I never thought.  If I took this game from my college days and changed it around a bit, I would be face down on the bathroom floor.

Never did I ever think I would be so familiar with the hospital...........................drink
Never did I ever think I would l to worry about my child's fat intake.................drink
Never did I ever think I would potty train my kids at the same time..................drink
Never did I ever think I would have a medication schedule..............................drink
Never did I ever think I would carry around a medical resume.........................drink
Never did I ever think after all the hard work a G-tube would be needed........drink

Here's the thing about CHD, it impacts so much more than just the heart.  And sometimes that isn't always discussed when you get the diagnosis. I mean, I get why.  Not all kids are the same.  Some have their surgeries and encounter minimal other issues.  But kids like Lucy, that is just not the case.  A perfect example would be our latest hospital stay.  In those 100 days we saw at least seven specialties.

Cardiology
Respiratory
Nephrology
Infectious disease
Gastroenterology
ENT
Psychiatry
Radiology

 Last week we went back to the hospital for a swallow study, ENT evaluation and her bridle change.
The swallow study went really well.  It was so fun to watch Lucy interact with Jen.  She was goofy,  talkative and showed no signs of being scared.  Quite a difference from our hospital meetings.  Lucy got up in the special chair, next to the big camera and sampled the food that Jen "made".  For the study, a dye has to be put on all the food so it can be tracked through chewing and swallowing.  We sampled thin, nectar, puree and solid food.  She passed on everything as long as the volume was small.  We got approved to give nectar liquids in amounts of 2.5 to 5 mL at a time.  We are not on a liquid (amount) restriction since her diuretics are so high, but we are trying not to go overboard.  We aim for four to six ounces by mouth a day.  The ENT evaluation was a little more traumatic and Dr. Sherman had to put the scope up her nose to view her vocal cords.  She sat in my lap and I held her like a straight jacket, while the nurse held her head and Dr. Sherman placed the scope.  She screamed, maybe the loudest I have heard in awhile.  Lucy's vocal cords have healed a bit but  there is still some compensation from the left side to close them all the way.  At this time, no intervention was needed, but he stressed that the NJ tube is impacting all of it.  And then he said those dreaded words:  G tube.
Now, I'm not scared of the actual g-tube.  I have heard amazing things about kids getting them.  I think it's that is another surgery and another hospital stay.  But honestly the hardest part is that we worked SO SO HARD to get her eating, that to be back here talking about g-tubes is just another punch in the gut.  I was hopeful we would have some time to work on her eating before definitively deciding about the surgery, but my gut was telling me get prepared.

Fast forward to yesterday and our cardiology appointment.  We had a blood draw for labs and it only took me two months and five trips to remember the lidocaine for arm.  It didn't help much, so maybe it's not that big of deal that I haven't remembered.  Then an EKG and vitals.  Last we sat down with Dr. Husayni to discuss what has been happening over the last month.   Since our last appointment we have seen her physical strength increase along with her energy level, but her oral intake has decrease tremendously.  We are lucky that we have not seen her weight tank as that would mean an admittance.  And he finally said it, we need a GI evaluation so we can get an idea of what her digestive system is doing.  I asked what the probability of the G-tube was and his response was, she needs it.  My eyes get watery as I start to dread another hospital stay.
Me:  How long is the average stay
Dr. Husayni:  about 2-3 days
Me:  So Lucy at least a week
Dr.  Husayni:  You are veteran, you know how it goes
Me:  When?
Dr. Husayni:  We should do it this year.

And there you have it.  2017 can officially suck it!!!!  We will start with GI evaluation, then look to schedule the surgery.

While this news definitely overshadowed the good that has happened this month.  There is still good!  We have decreased some medications and stopped another one.  We have adjusted her feeding times.  Hooking her up at 4 pm was not working out because Lucy can't quite grasp that the feeding pole has to come with her when she gets up to move.  We started feeding her at nap time so that we could delay the night feed until she went to bed.  The other change we made was moving up her night dose of diuretics to 4 pm, so that there were no more accidents and I don't have to wake her up to change her diaper 3 times overnight.  BUT what I love is that Lucy loves Dr. Husayni.  While we were getting vitals he walked by and she got so excited she made him come in for a hug.  Then she helped him listen to her.  When we were finished, she asked if he would walk us out.  I hope this sweet relationship grows stronger as he helps to guide her through this journey.

My biggest fear with this surgery is how it will impact Lucy's mental state.  She still has anxiety when I leave and many times at night she asks "you're not gonna leave".  We try really hard to ease her fears and to explain everything that is happening but sometimes that is just not enough.  While she is smart enough to understand she still get scared.  I truly hope I will be able to say, this was the easiest one yet.

Eating, it's always the eating

Where do I start?  HOME, HOME is amazing.  I actually went back and read my blog from our first homecoming (Lucy was six pounds at five months old, that seems crazy) and some of the feelings are the same.  New medications, oxygen and feeding pump were a big change from when we came to the hospital and I definitely was feeling a bit overwhelmed.  But just like last time, a day in and I felt a lot better.  Our wild card: Connor.  Luckily, my parents kept him at their house for our first week home.  It really allowed us to focus on Lucy and get settled.  The first few days Lucy slept a lot and she needed it.  She was able to be awake for about two hours at a time and then would crash.  She would fall asleep wherever, family room floor, sitting up on the office couch or her fabulous, comfortable BIG GIRL bed.

Her extended napping allowed me to re-organize the medication cabinet and unpack.

our schedule looked like this:
7 am - 8 medications
7:15 - weigh in  and breakfast
9 am - napping
12 pm - lunch
1 pm - 3 medications
2 pm - napping
4 pm - 1 medication and start feeds
6 pm - 4 medications
6:30 pm - bed time
7 pm - 8 medications
8 pm - add formula to feeds
11 pm - 1 medication
12 am - add formula
4 am - shut off feeding pump and flush NJ tube

The one thing I did not anticipate (although I probably should have) was the effect of the diuretics.  The first two nights, at least once, Lucy peed through the diaper.  Let me tell you, there is a fine line you walk, allowing her to sleep without interruption and changing her diaper before it leaks through  to her sheets.  When that happens, it's a big production.  Getting her out of bed, changing the sheets, you know the drill.  After three times of missing the window, I came up with a new plan.  Her bed is now an arsenal of protection against a leaky diaper.  First fitted sheet, then mattress protector, next another fitted sheet, and last a thick blanket.  This leaves me three leaks before I have to completely strip the bed.  We are on to week five and have a pretty good night time routine.  She gets her last diuretic at 7 pm.  If she goes right to bed, then we diaper change at 8:30, 11 pm and 3 am.  If she stays up until 8 pm, we get a clean diaper on then a change at 10 pm and 3 am.  3 am is when her feeds usually error out, so I am getting up anyway.  Error out sounds like the wrong thing to say, but what I have found with the feeding pumps is they are not calibrated very well.  She usually gets her total amount of formula (720 mL) in under the twelve hours and when the formula is gone, the pump will display a error message.

While I thought our biggest obstacle at home was going to be physical therapy, it appears that once again feeding issues will take the cake.  But not in the way I thought.  Lucy very much wants to eat and aside from thin liquids and fat our options are unlimited.  (Did you get that joke?).  Our fat free diet has us really restricted, but I have been able to get some variety into her diet.  Purees are our best because they take the least amount of energy to consume.  Our diet has been made up of soups, fruits, veggies, mashed potatoes and noodles.  Her favorite, by far, sour cream, plain, in a bowl eating it with a spoon.  Here is our hurdle, Lucy has thrown up at least once (but most times 2 or more) every single day since we have been home.  Sometimes it happens immediately after she eats.  Other times it's hours after a meal.  However, it doesn't seem to phase her.  She has thrown up and in the next breath asked for more food.  I was hopeful that her want of eating would allow us to decrease her tube feeding quickly, but her throwing up does not really let us get a good picture of her caloric intake.  Until we have a string of days without throw up, we will probably remain on twelve hour feeds.  The good news is we are coming up on the end of our eight week fat free diet.  I asked our cardiologist if we could remain on fat free formula so that we could increase her fat intake by mouth expand her variety of food.  We got the green light, so we will be able to foods with 3 grams of fat per serving.

We started physical therapy through Easter Seals and our therapist is able to come to the house while Lucy is still on two liters of oxygen.  We'll see her once a week, then when Lucy goes back to school, Laura would be able to come take her out of school for therapy and it would not be an extra trip.  She is so ahead of where I thought she would be.  She wasn't able to stand when we first came home but know, she is walking and WITHOUT her walker most of the time.  Then we will just need to strengthen everything.  In no time, she'll be jumping and running just like she was.  Let's just hope when that time comes she is not still on her feeding tube.  It has been slightly hard to explain to her that when she is getting feeds she cannot just get up and go.  She has to make sure her pump comes with her.  I have been trying to get her to use it as support for walking but it's quite awkward to do on carpet.

Lastly, our one month follow-up went very well.  We ended up not changing the bridle on her NJ tube which was why it went so well.  Her echo had no changes from her previous one.  Clinically she looked good and her saturations were in the high 70's.  Her last two INR's (test for warfarin) were both 2.0 (which is in range: 2.0-2.5), so we are able to now test every two weeks.  We decreased one of her diuretics and also stopped another medication, which was nice because I was able to re-arrange her medication schedule so that she no longer is getting medication during nap time.  We go back in another month and we will probably start talking about a swallow study and ENT consult.  She has snagged Connor's sippy cup a few times and even drank her bath water, so I think we are at the point where we need to give her another try at thin liquids.

We continue to be blessed by all of you who keep us in your prayers for continued healing, THANK YOU!
Here she is taking her first few steps!