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.