TRIGGER POINT: SCHEDULING SURGERY

Levi will be 10 months old on Monday.  I just returned home from Levi's cardiologist appointment.  James is usually at Levi's appointments too, but today he is on a business trip and returns late tonight.

It's time for surgery.  The echo today showed that because of his severe aortic valve leakage, his left ventricle has dilated to a z-score of 3.  Four weeks ago it had a z-score of 2.  That's a significant increase and the main trigger we've been waiting for.

It's been a hard waiting game, knowing that surgery is imminent, but not knowing when.  Going from appointment to appointment, wondering if this is the appointment.  As much as we really, really hoped Levi would be older and bigger before he needed surgery, in all honesty, there's a little part of me that's relieved to have the waiting somewhat over.  But, I'm so sad for Levi.  That he has to go through this surgery, a slew of appointments and tests, plus he'll have to go through these things for the rest of his life. Because there is no cure for aortic valve disease, this is only the beginning.

After receiving all the opinions from the various centers, we've decided to put Levi in the hands of Dr. Frank Hanley at Lucile Packard's Children's Hospital at Stanford.  We spoke with him for the second time by phone just yesterday morning.  He's known as an outstanding surgeon and one that we're very comfortable with.  He has graciously answered our many, many questions and we feel like we can trust him with our baby boy.

As far as what he'll do during surgery, it's still not 100% determined yet.  He'll repair Levi's ascending aortic aneurysm for sure.  Then after beginning surgery and getting a good look at Levi's aortic valve, he'll determine if a durable repair is possible or if a Ross is needed instead.

So from here, our cardiologist will contact the surgeon's office with Levi's latest results and start the ball rolling for scheduling surgery.  She asked me this morning what would be ideal timing for us.  In a perfect world (which this is not!) it'd be after my mom gets out of school (she's a teacher) in mid-June.  Then she could fly up to Bend to watch the older boys while we fly down to Palo Alto, CA for Levi's surgery.  Recovery time, if all goes well, is 7-10 days, plus travel time.  I'd also love it if Levi could make it to his 1st birthday on June 18, without surgery.  It certainly may need to be sooner than that, and we'll do whatever is best for Levi.

We'll see what the surgeon has to say about timing once he sees today's results.  In the meantime, Levi is scheduled for another echo in three weeks on May 3, assuming that surgery is not scheduled before then.

I feel like I say this a lot, but it's hard to imagine sometimes that Levi even has a heart defect.  He is developmentally right on track, active, alert, adorable and happy as can be.  He's crawling everywhere and pulling up and cruising on everything, exploring anything within his reach.  He's babbling and eating table food.  This is such a fun age!  How can something be so wrong inside his little body?

We pray that these seemingly overwhelming challenges facing Levi will only make him stronger as a person. That his life story will be used to impact the people around him and glorify God.  As we approach the year mark of Levi's life, I know James and I are not who we were a year ago.  Our lives took an unexpected turn.  Though we wouldn't have chosen this path for Levi, it has shown us that God is in control and goes before us each day. Our priorities have been rearranged and are more clear now than they were before.

I've been listening to a new song on the radio lately, entitled "Blessings," by Laura Story.  These words about life's struggles have been an encouragement to me.

What if Your blessings come through raindrops 

What if Your healing comes through tears 

And what if a thousand sleepless nights  

Are what it takes to know You’re near

What if my greatest disappointments 

Or the aching of this life 

Is the revealing of a greater thirst this world can’t satisfy 

And what if trials of this life 

The rain, the storms, the hardest nights 

Are Your mercies in disguise

For now it's one day and one step at a time.  We trust God with the tomorrows.  He knows what's ahead and just what we need.  He is walking this journey with us and already knows the story of Levi's heart.

A FEW MORE PICS

I brought a couple cameras on the trip to CA and realized I hadn't downloaded a few pictures from my point-and-shoot.  We drove right by Mt. Shasta as the sun was setting and it was a spectacular sight.

Yesterday at preschool, Noah received a citizenship award for demonstrating faithfulness.  He was so excited about it, and so was I.  We celebrated by going on a mommy & son dinner date to Red Robin, his favorite place.  He's 4 1/2 but seems to be growing up so quickly.  He's such a sweet and thoughtful little boy and I can hardly believe he'll be in kindergarten this coming Fall.

ALL THREE OPINIONS ARE IN

It's taken me longer than I would have hoped to get an update up.  Over the last two weeks we've received a lot of information and we're still in the process of sorting it all out.  The long and short of it is that we've finally heard back from Boston, Stanford, and LA.  For so long we'd just been wanting to get opinions from these major centers so that we know what the next step is for Levi.  Now that we have them, we wish they all had the same opinion!  That being said, we are getting closer to figuring out the what and when of Levi's heart surgery.

Here's a summary of the opinions, in the order in which they were received.

Boston:  (we spoke with our cardiologist who spoke with doctors in Boston)
Even though Levi's recent CT showed no dissection of the aorta, they are still very concerned about the significant dilation of his aorta.  They feel that surgery for his dilated aorta should be performed soon, whether or not intervention for his valve is imminent.  Then, in addition to fixing his ascending aorta, they would also try to repair his aortic valve.

Stanford: (we spoke with Dr. Hanley by phone)
He believes that Levi's dilated aorta is not of major concern in and of itself, and just needs to be watched for now.  The consensus of his group is that Levi does not require any intervention at this time.  At some point he will need surgery, but this will be based on either the progression of his leakage, and/or increasing left ventricular dimensions (dilation of the left ventricle).  When the time comes for Levi's surgical intervention, his preference is to perform an aortic valve repair.  If a repair is not possible or successful, then a Ross procedure would be performed.  This decision would be made at the time of the surgery.  He wants Levi followed closely for now.

LA: (we met with Dr. Starnes last week)
After looking at Levi's echos, he believes Levi needs a Ross procedure.  Based on how malformed Levi's valve is, he doesn't believe a repair is a viable option.  Even though Levi's left ventricle is functioning fairly well at this time, because the aortic valve leakage is severe, he believes it's better to do a Ross before the heart function worsens and there is irreversible damage.  He'd fix the dilated ascending aorta at the same time.  He recommends surgery in the next few months, between now and July.

Now that all the opinions are in, we'd like to talk to Levi's cardiologist again.  Right before we went to LA, after hearing from Stanford, she said she'd recommend Stanford.  She also said she'd be comfortable if we chose either of the other centers too.  We also felt really good about Stanford after speaking with the surgeon there.  Then we went to LA, and now we aren't sure and have more questions.

So, tomorrow I plan on speaking with Dr. Tajchman about what we learned/heard in LA.  Then James and I would like to set up another phone consultation this week with Dr. Hanley at Stanford.  After that, we hope to have a more clear picture of what the best course of action and timing is for Levi's surgery.

On April 14, Levi has another echo scheduled at his cardiologist's office.  We'll see how his heart is dealing with the severe leakage and we'll go from there.

We pray for clarity and wisdom in this sea of information that keeps on flowing our way.


[For those of you who may want more specifics from Levi's last appointment:]

• aortic insufficiency is in the severe range
• mean aortic valve gradient is 30mmHG (moderate aortic stenosis)
• left ventricle remains mildly hypertrophied subjectively
• ascending aorta is 2.2 cm (z-score of 6) 
• otherwise asymptomatic
• blood pressure 90mmHG systolic
• weight 8.45 kg, height 69 cm as of 3/15/11 (steadily in the 20th percentile)

CALIFORNIA

Our road trip to southern California went really well.  It took about 17 hours to drive each way and the kids did surprisingly well.  We had a jam-packed schedule and very little rest, but it was a wonderful trip filled with family, friends, the beach, Disneyland, and a business trip for James.  We didn't get to see everyone we had hoped to, so that was the biggest bummer.  We could have used another week down there!  That being said, there's no place like home.  We are finding rest and relaxation as we get back into the swing of things here in Oregon.

boys with great-uncle Craig and great-aunt Wendy

A day on Coronado Island with cousins - so much fun!

Levi's first visit to the beach

Uncle Steve and Aunt Lisa love on Levi

Noah LOVED the water!

I love this picture of all the cousins

 I think the kids called this a "cousin sandwich"

We had such a fun time!  The kids were so good too.

Noah went on Big Thunder and loved it

the end of the day

bath time after a sticky hot day at Disneyland

playing piano with Aunt Annie

the boys with Grandma Valerie

the last evening of the trip...enjoying the warm weather and green grass

CARDIOLOGIST APPOINTMENT - ALMOST NINE MONTHS OLD

Levi will be nine months old on Friday.

Today Levi had another appointment with his cardiologist.  The bottom line is his tests showed that everything is about the same as his last appointment four weeks ago.  That's relatively good.  Levi's aortic valve is still stenotic, still leaking a lot, his ascending aorta is still significantly dilated (aneurysm), and his left ventricle is mildly dilated, BUT it's not worse than four weeks ago.

James asked Dr. Tajchman if she thought Levi would need surgery before the year is out, and she said yes.  So it's coming, we just still don't know exactly when.  She wants to see Levi every 3-4 weeks for echos.  Once his left ventricle starts to dilate more, then we'll schedule surgery.

Dr. Tajchman is still waiting to hear back from Dr. Hanley at Stanford.  I was really hoping to hear that she had already heard from him.  Once she does, she'll give us her opinion on the best course of treatment/surgery.  Probably a Ross Procedure, but repairing the valve is not totally off the table yet.  The Ross would take care of replacing his diseased aortic valve as well as the dilated ascending aorta.  If a valve repair was attempted, they would also need to replace the dilated aorta, and I'm not sure what type of material would be used for that.  No matter what, Levi will still need several open heart surgeries during his childhood as he continues to grow.

At this point, we continue to move along with our plan of gathering information from hospitals/surgeons.  We still plan on meeting with Dr. Starnes at CHLA at the end of this month.  We eagerly await hearing from Dr. Hanley, and Dr. Tajchman is waiting to hear a few more details from Boston.  So, not much new information today.  As much as we aren't looking forward to this surgery, we do look forward to knowing what the plan for surgery will be and who the surgeon will be.

On a positive note, in talking with Dr. Tajchman about open heart surgery, she said kids usually bounce back really quickly.  Maybe a week to ten days in the hospital, and then fully healed in about six weeks.  Assuming all goes well, he should be able to run, play, and ride bikes as he gets older, just like other kids.  She said he may even be able to play sports if he wants, depending on how his heart is doing at that point in time.

Meanwhile, Levi is still doing really well at home, crawling and pulling up on everything.  You'd never know so much was going on with his little heart.

PRELIMINARY CT RESULTS

We drove three hours to Portland yesterday and had Levi's CT scan at OHSU early this morning.  We really wanted to know the results before we got back on the road to Bend, so that we knew there was no emergency.

Not long after the CT was completed, the radiologist came into the recovery room where Levi was beginning to wake up, and was kind enough to give us the preliminary results.  She said she didn't see an aortic dissection, which is wonderful news!  She took another look at the original CT back in June, which she also read back then, and compared it to today's.  She believes it was probably a thrombus (blood clot) that was there before, but that it's since resolved itself.  The doctor pulled up the images and showed them to us too, which was really helpful.  We're so thankful for her taking the time to talk to us and explain everything.

waiting for his turn 

The radiologist is going to study the scans even more, send the results to Levi's cardiologist in Bend, and call her too.  Unless something changes, we expect to hear more about the CT results at Levi's next cardiology/echo appointment on March 15.

One downside about today's appointment was that the doctor had a hard time starting Levi's IV.  The first one worked, but once he was brought into the CT room and the sedative was put in, the IV didn't work.  So, they tried Levi's other arm.  That didn't work either, so they tried his foot and finally got it to work.  Poor Levi was miserable.  We arrived at 6:50 am and didn't leave till 11:00 am and Levi was exhausted by the end of it all.  He slept almost the whole car ride home.

in his cute hospital gown just before IV and sedation

In addition to the good results we had another bright spot in our whirlwind trip.  When we started driving, we still didn't know where we would be staying.  About a half hour into the drive we got a call saying that we weren't able to get into the Ronald McDonald House (RMH) next to OHSU because it was full, but we did get in to the East RMH a few miles away.  Before Levi was born, I didn't really know much about these houses.  They are amazing.  We were warmly welcomed, shown to a beautiful, peaceful room, and had a hot dinner provided by community members.  We had a room at RMH when Levi was first born too.  With all the craziness and emotions surrounding a child with special medical needs, these houses are a such refuge and provide hope and needed rest.  So many families and children are supported by these very special houses.  We are thankful and definitely want to give back to RMH at some point too.

                               

                          Ronald McDonald House East Portland

Our relaxing RMH room

It's really, really good to be home again.  It helped tremendously to know that Noah and Jacob were so well taken care of by our friends while we were away.  Thank you everyone for all of your support and prayers!!!!

POSSIBLE AORTIC DISSECTION

Update: Sunday, 11:00 am

James is now coming with me to Portland for Levi's scan!!!  Our dear neighbor offered to stay with the boys overnight so James could come too!


Friday, 12:45 pm

It's amazing how much things can change with one little phone call.

I had gone to a kindergarten informational meeting last night.  As I was leaving at about 7:30 pm, I realized my cell phone was buzzing and just missed the call.  I listened to the message in the car and it was Levi's cardiologist.  She said she had heard back from Boston and would try our home phone.

So I raced home and when I walked in James was still on the phone with her.  When he was finished, he handed the phone over to me and Dr. Tajchman filled me in.

Boston had called with both good and bad news.  The good news is that they think they can repair Levi's aortic valve (repair doesn't mean fix in this case, but it could buy him a few more years before a valve replacement is needed).

The bad news is that after reviewing all of Levi's echos and the one CT scan done his first week of life, they think that Levi might have a chronic aortic dissection.  Aortic dissection is a condition in which there is bleeding into and along the wall of the aorta (the major artery from the heart).  This most often occurs because of a tear or damage to the inner wall of the artery.  This is very dangerous and can lead to a rupture and death.  You may remember that this was mentioned back in the very beginning, when Levi was a few days old and in the NICU at OHSU in Portland.  It's believed that the wire used during the balloon dilation when he was first born may have caused an injury in his ascending aorta.  At that time Levi had a CT angiogram done to see if the wavy flap seen on the echos was a dissection or not.  The results came back that it wasn't a dissection (great news at the time), but that instead it may be an intimal tear that could eventually heal itself or a thrombus.  Still not good, but better than a dissection.  Since so much time has passed there has been less concern about this, until now.

After reviewing everything, Boston has reason to believe that it may indeed be an aortic dissection.  Levi's records were reviewed by a team of doctors in Boston, including highly regarded surgeons, Dr. Pedro del Nido and Dr. Christopher Baird.  Dr. del Nido is "the" man over there and one of the best in the world.  They want Levi to have another CT done soon to hopefully give a more definitive diagnosis.  If it's a dissection, that may mean he needs to have surgery to repair that as well as his valve sooner rather than later.  We are really, really hoping this is not a dissection!

So the next step is the CT.  Unfortunately, we need to drive to OHSU in Portland for this, as his cardiologist thinks this is the best facility for this test.

I just received final confirmation that he is scheduled for check-in at 7:00 am on Wednesday, March 9, with sedation at 8:00 am and the scan at 8:30 am.  Food and milk restrictions ahead of time, which is never fun.  At this point the plan is for Levi and me to drive over on Tuesday afternoon, hopefully stay at the Ronald McDonald House next to the hospital, and then have the CT done first thing the next morning and drive home that afternoon.

Once the results are in, we'll know much more and our cardiologist will forward the images to Boston, Stanford, and LA.  Then we'll see what comes next.  I'll update the blog as we learn more.

We're just so sad that our sweet little guy has to go through all of this.  Thinking about open heart surgery scares us.  We've known that it would come to this, but the reality of it is hitting much harder right now.  My whole body aches for Levi.  I didn't sleep much last night.  We also still need to tell the other boys.  They don't know about Levi's condition.  Because of their young ages, we've decided to tell them once things start moving in the direction of an intervention being needed.  I guess that might be soon.

Thank you for all of your prayers and offers of support and help.  That means so much to us.  We pray God will protect and heal our little Levi.  We thank Him for His faithfulness and pray for His perfect will to be done, whatever it may be.  Levi's life has a purpose and a bigger plan than we know.  We are learning to take each day as it comes and know we are not walking this alone.  Please pray for peace and wisdom as we continue to navigate through all the unknowns.

Now I'm off to a kindergarten school tour.  It's weird to still have the normal day to day things happening when so much more is on my mind.

I'll keep you posted.

Love,
Erika

CRAWLING AND SCHEDULE CHANGE

Levi's officially mobile.  He's crawling everywhere and can get his own toys, which is great!

I received a phone call yesterday from CHLA and the consultation with Dr. Starnes needed to be moved to March 28 instead of the 31st.  I just hope it doesn't get changed again since we're only down there for a short amount of time.  The good news is that CHLA did confirm our insurance coverage and it's officially an in-network provider, if we decide to go there for surgery.  So our coverage is the same down there as it is in Oregon--a huge plus!

We're all healthy again and doing well.  I'm sure the next few weeks will speed by and we'll be in California before we know it!

Levi's on his special quilt

heading out for our anniversary dinner

happy boy!