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!