Today has marked a new era in Lilliana’s journey.
We knew the test results were in regarding her possible PLE diagnosis
so we made an appointment with her cardiologist to discuss what it all means
for her. Let me start by saying Lilliana did AMAZING at the four hour
appointment, She didn’t even cry during the blood draw!!!! (That has NEVER
HAPPENED). We learned that YES, she has
PLE (Protein Losing Enteropathy), YES, she is in heart failure, and there isn’t
much we can do about it. The oxygen getting
to her brain is fantastic, but to her abdominal organs not so hot.
The development of protein-losing
enteropathy is associated with a poor clinical course in patients after a
Fontan operation. Despite all measures, mortality is 50% within the first 5
years after the diagnosis is made. Multiple treatment modalities exist for
protein-losing enteropathy, and this reflects the absence of definitive
management principles based on its cause. Protein-losing enteropathy remains
one of the greatest challenges in the management of post-Fontan
single-ventricle patients.
Lilliana’s anatomy is so beyond complex, even a “normal
hyperplastic left heart” patient would have a difficult time dealing with PLE.
She is not a candidate for any surgical option and thus we are attempting
medications and diet.
What does all this mean…
Our cardiologist (who we have seen since before Lilliana was
even born) has now passed her on to the “pre-transplant” team.
Instead of
seeing them twice a year we will now be seen once a month.
She will be getting sick often, her immune system will be
lower and she will be prone to infections (which explains the two massive ear
infections within two months).
She will continue to get “puffy” and we will have to monitor
her with diuretics.
She will be losing protein through her intestinal tract and
will need to be on a high protein, low fat diet. Ever bite of food she eats
needs to as nutritionally dense as possible.
She will need blood draws often to monitor her albumin
levels.
She may be on even more medications
Long term PLE will cause gastro issues and liver damage
We were given the “magic” phone number to call 24-7 (aka
high risk transplant patients)
We were told that the next step would be transplant! But
please remember that we have been told by the best heart surgeon in the world
that Lilliana would be the most complex transplant he could imagine. More than likely
she will NEVER even be a candidate.
I have mixed emotions. Part of me is at peace thinking just
enjoy her, soak of every little moment, let her experience everything you possibly
can and the other side of me is scared, angry, sad, worried….
I know if anyone can fight this like a pro it’s her! I’m
still holding out hope that with diet we can greatly minimize the symptoms. She
is so strong and she has made it through horrible prognoses before.
I know deep within my soul that Lilliana chose this life.
She wanted to be a teacher, a healer, and little magnet to draw people closer
to God. She is such a bright light and I can’t possibly imagine this world
without it. I feel determined, I want the world to know her story, I want her
to experience EVERYTHING, I will be her warrior, I will keep her as healthy as
possible, and I will fight for her and never give up!
Please pray for amazing healing.
