Pediatrician to Geneticist.....Final

Here it is…… Surgery day.  My mom was amazing and flew down to help support us and Joe and Scott, Jimmy's brother and partner, are coming to the hospital.  Jimmy and I have to arrive at the hospital around 7:30 am to get Ava checked in and set up for the surgery.  I was totally telling myself I would be composed and able to ask any questions I had.  I  AM A LIAR!!!!  I was crying the minute we got Ava to the hospital.

So, walking into the hospital that morning, January 2, 2018, we had no idea if Ava actually had biliary atresa.  The surgery she was scheduled for was exploratory to find out and get a proper liver biopsy (if you read the other blog posts you'll know why).  With that being said, we have no idea if this surgery will be 2 hours or more than 6 hours.  No idea how long our hospital stay will be.  It could be an overnight or 6 weeks.  Literally, we are walking into the unknown.  With all the testing done on Ava we were told that there is an 90% chance she will receive a Kasai Procedure.

We had to stop feeding Ava at about 3:30-4 am and that was ok until right about the time we got to the hospital.  She was hungry and we couldn't feed her.  This on it's own is heartbreaking but add in the day she is about to have and a hormonal postpartum mommy and water works started.  I was able to calm her down and myself just in time for us to chat with reception and get Ava her sweet bracelets (hospital bracelets).  We head back out to the waiting room and of course, wait.  Neither Jimmy or I slept much the night before dreading what was about to happen.  

Pre-Op

Finally, we are called back to the preparation area.  It is a larger room with tons of curtains to separate each patient and family.  Here is where my tears really start up.  The nurses give us a Johnny to put on Ava and she is sooooo tiny in the huge shirt.  The nurse starts to ask questions and Jimmy has to do majority of the answering.  I'm a mess.  Ava is crying because she's hungry, my milk supply has increased some and her crying is making even my boobs cry.  They let us know that someone will be over shortly to discuss the surgery and what to expect.  Of course, we have researched and have a ton of questions to ask.

One of the surgeons stops by to check in and we know her from the NICU.  I immediately start crying again and apologize to her.  I keep telling her I trust her and the team but I'm still terrified.  She is so sweet and tells me she understands and will do everything possible for Ava.  She leaves to gather her part of the team and tells us the anesthesiologist will be in shortly to discuss their procedures.

I start to calm down again and this is when the realization comes in that this surgery will be the longest Ava and I will be a part from each other since she made her grand entrance into our world.  I start crying again.  Poor Jimmy through all of this is absolutely amazing.  Trying to make me laugh and telling me it's ok.

Next, here is the anesthesiologist. He is absolutely amazing and he had such a great idea.  Instead of giving Ava too many narcotics, we give her an epidural.  I had an amazing experience with my epidural when Ava was born that I was 10000% for it.  The gentleman leaves so Jimmy and I can discuss it and we both agreed to it.  Anesthesiologist comes back we let him know and it's all down to the head surgeon to come in, Dr. Padias (this is the amazing man we spoke about in previous blogs that got us admitted into the NICU).

We wait about an hour and Dr. Padias comes in to let us know we are ready to go.  Ava is crying so badly because she's hungry and I'm crying so badly because I just want everything to be ok.  He tells us that he will text Jimmy and let us know if they are proceeding with the Kasai or not.  He even draws us a picture to let us know what will happen if he does have to proceed.  We thank him again and minutes later they come to wheel Ava girl to the back and we head out to the waiting room to meet Joe, Scott and my mom.

At this point, I'm pretty sure my boobs are literally going to explode and my mom comes with me on a quest for a nursing room so I can pump.  I'm so grateful she came along because my crazy anxiety combined with new mom hormones makes for amazing tales of worst case scenario in my brain.  We get into the room and my shirt is totally soaked and I pumped for almost 45 mins to empty the girls.  This is amazing because it means I definitely built up some serious supply while in the NICU!  We load up the liquid gold and head back down to the waiting room with the boys.

Text from Dr. Padias

We are in the waiting room for about an hour and half chatting, mostly me because that's what I do when I'm about to explode with anxiety, and Jimmy gets the most amazing text.  "Your prayers answered.  I'm coming out to explain Ava's normal anatomy!!  This is all cholestasis unless something weird comes back from liver bx.  Great news!!"  My heart exploded!  This is amazing!  Everyone was 90% sure this was biliary atresia.  90! But our little warrior beat the odds.

Dr. Padias comes out and we are hugging, crying (not me because I'm literally dehydrated from crying and leaking so much earlier) and cursing the HIDA Scan.  He heads back to sew our girl up and about a half hour later we are back in post-op hanging out with our sleeping normal anatomy baby.  I wanted to pick her up but she was asleep and still groggy from surgery.  Then it hit me, how were we going to hold our baby?  How was I going to nurse her.  She had an incision that, on her small body, was half of her abdomen.

Finally, we are wheeled up to the PICU (Pediatric Intensive Care Unit) and Ava is transferred to this enormous bed.  She looked so tiny and sad.  Once the nurses got her all settled in I was able to finally nurse her.  This was terrifying for me.  I totally thought I would somehow rip her incision open or pull out her epidural catheter.  Luckily, none of this happened but the nurses were called every single time I needed to nurse her that day and night.

A little while after Ava was comfortable the surgical team stopped in to check on her and let us know how much of a Rockstar she is.  They told us she did amazing in surgery and it was looking like we would only need to stay overnight.  Not 6 weeks like we had originally thought.  Not 10 days like the NICU.  OVERNIGHT!  This was amazing news!

Through the rest of the day Ava was looking in on by the surgical team, attending doctors and lots of nurses.  That night was pretty intense.  There were a lot of little ones there without their families.  There's a million different reasons why families can't always be there and we are so grateful we were able to make it happen.  The little ones were crying out during the night and it was heartbreaking.  The little girl in the room next to us kept yelling for her mommy.  I literally wanted to go in and stay with her.

The next morning came and Jimmy headed off to work and my mom came in to stay with Ava and I.  We were cleared to leave and by 5 pm that evening we were off to our home. We were taking our little warrior baby home to heal.  We were so happy!

Victory!

POST-OP AND HEALING.....

Now we've been home and learning how to be parents without 50 people walking into the room to check on the baby.  We get to be parents and like mess stuff up like regular parents.  Ava's incision is healing awesomely and we even get to give her a bath.  We are still doing weigh ins every couple of days and meeting with a gastroenterologist to make sure Ava's liver is functioning properly.

January 15th comes and it's Ava check up with Dr. Padias.  Dr. Padias let us know before Ava's surgery he would be leaving USF and Florida in January.  We were so sad to hear but were grateful to have him here for Ava's surgery and totally hoped we wouldn't need to have a pediatric surgery again anytime soon.  Ava was his last client before jumping into his car and driving to his new location.

We were chatting with Doc and telling how Ava is putting weight on and eating like a champ.  I'll never forget the day we actually saw 10 lbs on the scale for her. She is developing like she should and more.  He checks out her incision and tells us she is healing perfectly.  Then, come the liver biopsy results.  I literally had stuffed the biopsy on the backburner thinking nothing else could go wrong.  Well, according to 2 pathologists thing could still go pretty wrong.  Ava's biopsy came back testing positive for Niemann Picks Disease Type 2C .  To say my heart dropped would be an understatement.  I had to sit down.  I thought I was going to pass out or throw up.  This couldn't be happening.  We just beat biliary atresia.  We don't want to do battle again.

He tells us the results can only be verified by a geneticist and refers us to someone he trusts.  He explains that he doesn't know enough about the disease to really answer questions but does reinforce that no case is ever the same.  The case we need to focus on is Ava's case.  He also suggests we not let our families know until the geneticist does testing because there is that 90% chance like before that Ava is in the clear.  We do as he suggests which was one of the hardest things we have ever done.  I'm close with my family and we can share anything with each other but Jimmy speaks to his family every day.  He has nieces and nephews that range from 2 to 21 years old.  With Niemann Picks most kids start to develop symptoms pretty early on and need a lot of care when they show up.

We head home heartbroken all over again.  Praying Ava will defy the odds yet again and off to work Jimmy had to go.  He is definitely one of the strongest people I know.  I couldn't hold it together.  I would look at our baby and just think of how much I didn't want her to leave us.  I think I cried for the entire day in between we called the geneticist and had bloodwork scheduled for January 17th.

THE GENETICIST-

We show up for bloodwork at the hospital on the 17th and I was doing ok.  There was some kind of mix up with the appointment and the receptionist but about 25 minutes later we were straightened out.  The entire time I am trying my hardest to slow my thousand beats per minute heartrate and lock up the tears that are trying to make their way out.

We get back to the ladies to have Ava's blood drawn and I hear the woman saying it was too much blood for such a small baby.  Alarms start going off and immediately I tell her not to continue.  I ask her to please double check with Kristin our geneticist's assistant and they agree to not take as much as requested.

Once Ava starts crying during her blood draw I lost it.  I couldn't stop crying.  Kristin tries to calm me down and I explain how we were just learning how to be parents on our own.  Just started to live life without an impending doom hanging overhead.  She explains that the results will be rushed and there is still a chance Ava is in the clear.  She also let's us know there is another family here in Tampa that have 2 children with Niemann Picks and my heart broke for them.  She explains there are clinic studies that they reached out to already for Ava.  None of this helps as we head home and I'm still sobbing.

That same day our amazing pediatrician called to check on us.  She was so incredibly sweet and asked if she could come with us to the geneticist for the results.  We of course say yes and are so grateful she offered.  She also said something to me that 100% helped my broken heart.  She said no matter what happens she believed that we were made Ava's parents for a reason.  That we were chosen to take care of her because we could.  This woman is literally the best.

Dr. Rush and Ava

THE RESULTS-

So after all of this bullshit, we are still going to weigh-ins and check ups with the gastroenterologist.  It is at the gastro's office that Ava and I were chatting with her doctor and cheering on her weight gain that my phone starts to blow up.  Text messages, phone calls and voicemails.  I look at my texts first, like any other human being, and there is a group chat from Jimmy and Dr. Rush.  Ava tested negative for Niemann Pick Disease.  Did you read that???? NEGATIVE!!!!!

Our girl beat the odds again!  I started to cry and yell in the gastro's office.  I almost Gronk spiked my phone! We had just got orders from our gastro to get a whole liver panel done but with this phone call it all changed.

We are truly blessed and absolutely know it.  We enjoy every day with our baby girl, even the hard ones.  Our girl beat the odds not once but twice!

Nowadays, we only see the doctor on routine check ups and talk about normal parents/baby stuff.  Her weight, well, if you see her photos little girl got mommy's legs and rocks that mermaid life.  She is healthy, happy and excelling at most things she tries and is most definitely independent.

I posted this story to help out any other parents going through a hard time and need a little bit of hope.  The happy ending can happen.  I'm sure I might've used some incorrect dates or missed whole scenarios but I needed to share Ava's story to be free of it and offer help to anyone who might need it.