Thursday, May 9 2013

The waiting has begun.  Erin is currently in surgery, and they expect it to be there for anywhere from 4 to 6 hours before we see her again.

We arrived here Tuesday evening, with Erin in terrific spirits.  She was as happy and animated as I have ever seen her.  Wednesday began with a blood draw at 7, which she stayed completely stoic through.  We then followed that up with x-rays, EKG, allergy test, echo, cardiology consult, Social Services, Child Life Services, and then an hour with the surgeon Dr. Dearani.  The allergy test, conducted because she once had a reaction to an antibiotic, was challenging as they pricked Erin's arm in 8 spots twice in a row.  She never reacted or flinched, but later told us just how painful it was.  She is absolutely amazing.  She isn't going to let anyone see her react.

Dearani has had additional thoughts about her surgery after consulting with the cardiologist who did the echo.  They now have decided that fixing the tricuspid valve offers no upside and thus will be left alone.  They are going to fix the ASD, and then take some time to see how she responds before they do the bi-directional glenn.

Waiting for Dr Dearani

Erin was somewhat stoic throughout, and fell asleep during the Dearani meeting.  She fell asleep the first time as well.  I told him it must be something about him.

Erin's favorite store is Target (sorry Uncle Alex).  We noticed that they had a Super Target, which really made her happy (it had to be better because it was Super).  We visited and bought supplies and a princess costume for Erin, which she took great delight in wearing last night after dinner.

We had to be at the hospital today at 7.  She woke up in a great mood, and had yet another bath which included the prescribed 10 minute scrub with special soap.

Getting ready to leave for the hospital

We arrived at the hospital and Erin was given a new pink pig, immediately named Piggy.  She played in the play area until 8, when we moved into the OR prep area.  This was when it became very sobering for Erin.  Getting on the gurney caused her to be very apprehensive, although she didn't say a word.   

In the play room

Doctoring Piggy

Heading to the OR

In the prep room, we got her dressed and spent some time hanging out.  She was extremely clingy as you would expect, and very quiet.  She was scared and it was excruciating as a parent not to be able to comfort her or take away the fear.  The nurse took a picture of the three of us and printed it our for her.  Erin clung to that picture, looking at it constantly.  At 9 am, I put her on the gurney and after saying goodbye to Mommy we headed off to the OR.

In the OR, I transferred her to the operating table, and helped to take off her top.  She asked for grape flavored gas (she had a wide choice...I would have taken bubble gum personally), and accepted the mask while I held her hand.  She was out in less than a minute.  It was only then that she let go of the family photo.

She was worried I was going to doctor her

It took almost an hour to get all of the lines in her, which they don't do until she is out  .At 10:14, the communicator Colleen informed us that they had made the incision.  At 11:03, she went on the heart and lung machine.

And so we wait.

Sunday May 5, 2013 - Picture update

In addition to the first blog post describing the upcoming events at Mayo, I wanted to update photo's and video of Erin to share just who she has become.  She is an amazing little girl, full of life.  She is large and in charge at all times, 4 going on 44.

Grandma Betty, who passed away March 11, 2013

Murphy Moo Moo, who also passed away this year

She loves to cook

Erin's beloved Priscilla

Face paint at the club Halloween party - yikes!

Easter Sunday 2013

Sunday, May 5 2013

It has been a little more than three years since I last posted an update in Erin's blog.  This blog was started originally for the purpose of keeping family and friends up to date on Erin's condition when she was in the NICU at birth due to her heart defect and other medical issues.  In the NICU, cell phones are prohibited, but even if they weren't it would have taken all day just to call the number of people who wanted updates.  Thus the blog was born.  The more I wrote in it, I realized that it had another purpose selfishly for me.  It was cathartic to put down in words what we were feeling and dealing with.  I also realized that it would be an incredibly important journal for Erin should she ever want to look into this part of her life, and understand how far she has come.

While I have received friendly prodding over the past three years to update the blog, I haven't really had a desire to do so, and frankly hoped that I was forever done with it.  I just wanted Erin to be a normal, healthy little girl.  A girl who formerly had a heart issue and now was over it.  A girl who's parents didn't look at her every time she runs out of breath and wonder if this is the beginning of a serious incident.  Unfortunately, that was never in the cards as much as I tried to convince myself that it was possible.  And so here we are.

Erin has reached the point in life where at almost 5 years of age, her heart now needs significant medical intervention to continue to function and allow her a chance at a normal life.  On Tuesday, we fly to Mayo Clinic in Rochester Minnesota where Erin will have open heart surgery on Thursday morning.  The operation is difficult and risky, and involves essentially re-plumbing her heart in an altogether different manner.  The main procedure is called a Bi-directional Glenn.  At the same time, they will close the ASD hole in her heart, and look at the tricuspid valve to determine whether it can be repaired, replaced, or left alone.  Finally, they will also look at the heart wall itself, to see if it needs repair because the tissue has become so thin.

We are told that assuming a successful outcome, she will be in the ICU for 3 - 4 days, and then moved to a regular room for several days after that.  Altogether, we expect to be in Rochester for two weeks.  Luckily for us, since we are "sick" of perfect weather here in Scottsdale, the area received almost a foot of snow this week.

We chose Mayo Clinic after very careful thought and consideration.  Our cardiologist continues to be Dr. Jeff Gossett at what is now known as Lurie Children's in Chicago.  Jeff has done an amazing job with Erin, and she feels a strong bond with him that always culminates in a big hug as she says goodbye after one of their many visits.  Were it not for Jeff, Erin would have had the first operation within a few weeks of her birth, and obviously a very different outcome.  As he puts it, everyone has done their job and gotten her to where she is older, bigger, and stronger, and in a far better place for this type of operation.  It is a Rubik's Cube of emotions.  On one hand, we know that she has a grotesquely distorted heart, one that can't continue to support her as she grows.  On the other hand, she is normal in every way, and you would never know there is anything wrong with her by observing her in everyday life, other than her occasional breathlessness that comes with play.  She knows that she needs to take regular breaks that other kids don't, and it bothers her.  But to live with her, you tend to forget about the medical issues, except when they force their way into your consciousness and hammer you into reality. It's hard to describe, really.

Our three choices of hospitals came down to Lurie, Boston Children's affiliated with Harvard, and Mayo.  We have grown very close and fond of all of the people at Lurie, and Sheila and I both serve on boards for the hospital.  They are one of the world's leaders in heart transplants for children, and is exactly where we would be headed if Erin were a candidate for that procedure.  It would be incredibly convenient to our downtown Chicago home.  However, her condition is so rare and involved that we wanted only the very best in the world.  That happens to be Dr. Joseph Dearani at Mayo.  He has performed more of these operations than anyone else, and that is what caused us to choose Mayo Clinic.  He told me last August when we went for a visit that they have only seen 1,000 cases of Ebstein's.  I thought that sounded like a lot, but he assured me that number is insignificant for Mayo.  He then went on to say that out of those 1,000 cases, they have seen less than 25 like Erin's.  That is a sobering fact.

We were impressed with Dr. Dearani from the start.  We arrived at our 4 pm appointment at 5:30, having been held up by all of the tests that Erin underwent.  He made it completely clear to us that if we needed to stay until 10 pm asking questions, he was going to stay right there and continue answering them in a patient, caring manner.  Sheila has handled the burden of interfacing with Mayo and making all of the hospital arrangements, and we have been incredibly impressed with everything we have observed thus far.  We are hoping for a very good experience based on our current interactions.

I will try and update the blog as timely as possible.  As always, we thank all who take the time to read this for their caring and kindness.  It is incredibly helpful to Sheila and myself.