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.