Update from LPCH Visit / Next Up: Cardiac Cath

We're back at home after our week long road trip to CA. We spent a very, very special few days with family in Carmel that were rejuvenating and so good for each of us. We also spent one day in Palo Alto at LPCH at Stanford.  We left Carmel at 5:45 am and came back at 5:00 pm.  It was a very long day.

After meeting with the surgeon, an urgent care visit for croup, an echo, a meeting with a cardiologist, and then another conversation with the surgeon that night, we have a bit more information about the state of Levi's heart.  But we also don't have the answer to when Levi will need surgery.

Arriving at LPCH

Levi held still for over an hour while two echo technicians recorded a number of images, both 2-D and 3-D, and took measurements of Levi's heart. Though this testing went super well, the surgeon felt that there was still some ambiguity with exactly what is going on with the pressure gradients in Levi's heart.

The images did show two subaortic fibromuscular ridges that are causing a rise in the pressure gradient of the blood as it exits the heart through the aortic valve and into the aorta.  There's a question as to whether or not there is also a subaortic membrane just below the aortic valve as well, or if it's scar tissue from the previous valve repair. The pressure gradients shown by this echo were still high.  The peak was in the 80s. We learned that something this high could be reason to operate, but that it's important to also take a look at the impact it's having on the heart, to make sure that all the data lines up.  

Levi loved this train set at the hospital

At this point, Levi's heart is still functioning quite well.  His left ventricle is squeezing well, there is little thickening of the heart muscle (hypertrophy), there is no mitral valve leakage, and the leakage from the aortic valve is still minimal.  The surgeon thinks there's a chance that the pressure gradient may not be as high as the echoes shows, since the heart does not appear to be that damaged.  The only way to get a completely accurate reading of the pressure gradient, is to go into the heart and take the readings.

Lots of waiting

So, the surgeon wants Levi to get a cardiac catheterization to measure the pressure.  This is done by inserting a thin tube in through the femoral artery (groin area) and up to the heart.  Levi will be under anesthesia and for a child Levi's age, it would most likely involve an overnight stay in the hospital.  So we'll be scheduling the cath in the near future and will travel back down to LPCH for this procedure. I wish it all could have been done while we were down on this trip, but with Christmas week, it wasn't possible to get on the schedule.

The good news is it's not an emergency.  But we'll want to get Levi in for the cath in the next couple months.  Then we'll have a better idea about when surgery is needed.

Just after finishing the echo.  Levi was a rock star through it all.

We also talked with the surgeon about how the best case scenario would be an open heart surgery (OHS) where only the subaortic obstructions would be removed.  However, there's also a chance that Levi would need a valve replacement as well, which is an even bigger deal.

So, while I had hoped to come away from this day at LPCH with answers, we're left in "wait and wonder" mode again.  Though a cath is more invasive than an echo, it's much better than OHS.  If the cath gives us "good" lower pressure gradients, then it's possible we could wait on surgery for now and get Levi even bigger and older.

Next step, get the cath scheduled and see what's really going on in the heart.

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A few pics from the rest of our visit...

All the boys LOVED hanging out at the beach.  What a treat!

Appointments on Monday

Levi singing in his preschool performance

We are all set for Monday's appointments down at LPCH.  We'll start the drive down to CA early tomorrow morning and should arrive in Carmel by tomorrow night, which is where we'll be spending Christmas with family.  

We'll drive back up to Palo Alto early on Monday morning, Dec. 23.  We meet with Dr. Hanley, Levi's surgeon, at 8:30 am to get his take on what needs to be done and when.  

Then at 11:30 am, Levi is scheduled for an echo, followed by an exam by pediatric cardiologist, Dr. Stafford Grady.  

We're very thankful that Levi is able to be seen at LPCH so soon and by such a great team of doctors.  Ideally we'd be able to meet with the surgeon after Monday's echo instead of before, but he'll at least get to take a look at the previous echo done here in Bend.  Then we have a phone consult set up for January 2, after he reviews the new echo.

The roads up here in OR look pretty clear and everyone is relatively healthy so I think we're good to go!  We're eager to get more information on Levi's heart and hopefully leave LPCH with some sort of a timeline for when surgery is needed.  It's still hard, but knowing what to expect next is certainly helpful.  

A snowy weekend up at Elk Lake

Stanford Appointment Set

I received a call from our local cardiologist's office this morning with good news about the next appointment.  Stanford is working on getting Levi in on Monday, December 23!  This is great timing as we'll already be down there for Christmas.

Levi's cardiologist here in town reached out to Levi's surgeon, Dr. Hanley, this morning and he was able to make this appointment happen.  So on the 23rd, Levi will go in for testing (I'm assuming an echo, EKG, etc.) and then we'll meet with a cardiologist--I'm not sure who just yet--and see what he or she says.  Then Dr. Hanley will get the results and we'll have a phone conference with him, most likely on January 2, to discuss the possible surgery.  The last time Levi went in for surgery, we conferenced by phone with Dr. Hanley twice.  He took his time talking to us and answering all of our questions. We never once felt rushed. We just really think he's great and feel like Levi is in the best of hands.  

So it will still be a little while longer before we have more details and a timeline for what Levi's little heart needs, but at least the next couple of steps are in place.

Another Surgery?

Today Levi went in for a routine cardiology appointment.  Though I say routine, nothing is ever routine about them. This appointment has rocked our world again.  It's funny how a few numbers and images can cast a shadow on such a healthy looking three and a half year old boy.

Levi's preschool picture -- 3.5 years old

The echo today revealed what looks like a subaortic obstruction of sorts that is causing his pressure gradient to skyrocket.  Today's peak gradient was 93 and the mean gradient was 46.  In comparison, at Levi's last cardiology exam in February 2013 his peak was 48 and the mean was 28.  That's a huge increase and not a good one.  The cardiologist said that the indication for surgery for some surgeons is a mean of 35. It was hard to tell for sure from the echo, but it doesn't look like Levi's previously repaired aortic valve itself is a big contributor to this jump in pressure.  Instead a subaortic membrane appears to have regrown below the valve and is causing a narrowing in the outflow tract.  This high pressure does not only cause the heart to work much harder to pump, but this pressure coming up from below the valve also has the capacity to cause damage to Levi's aortic valve, which was skillfully salvaged during the last surgery.  That's the bad news.

A glimmer of good news is that compared to his last appointment, he has only a slight increase in left ventricular hypertrophy, or thickening of the heart muscle caused by the extra work to pump blood out of the heart.  It's still in the mild range, so that's a plus.  His heart is handling the pressure well so far.  Also worth noting is that his ascending aorta appears in the normal range, whereas prior to his 2011 surgery, it was extremely dilated and needed repair. 

Unfortunately, the solution for dealing with subaortic stenosis is open heart surgery--again.

Levi shooting hoops with a good friend

 The plan right now is to get into LPCH (Lucile Packard Children's Hospital at Stanford) for a full exam, including a 3D echo, so we can get more precise information and see what they recommend.  We already had plans to travel down to that area of CA for the week of Christmas, so we're hoping somehow they can squeeze us in.  Otherwise, we'll need to fly down at a later point.  We'll also probably seek a second opinion from another major center such as Boston.

So we're in a hold pattern for now.  I'm hoping to have information tomorrow as to when we'll have that Stanford appointment.  And then the bigger question hanging over our heads is just how long we have till Levi needs yet another surgery.  And this is what just breaks our hearts.  

It's so hard to think about having to go through all of that again.  Our sweet boy is so much bigger now and more aware of the world around him.  He was a mere 12 months old the first time around.  Now he's a preschooler, a little person with a big personality, but he still doesn't understand that something is wrong with his heart.  He doesn't know what surgery is, but he knows a lot about what to expect around him in his little safe and predictable world, and surgery is not any part of that.  This sucks.  It's hard, and raw, and crushing for James and me right now. We've felt this before and know the emotions will heighten and lessen and change as we walk this road again.  We're thankful for the two and a half surgery-free years we've just had, but we really aren't ready to do this again.  

I'll keep everyone posted as we find out more information in the days to come.

Happy Heart Day and Levi's Frightening Illness

CHD Awareness Week

Happy Valentine's Day.  Heart Day.  Lots of emotions.  Today marks the end of CHD--Congenital Heart Defects--Awareness Week.  I knew nothing of congenital heart defects before Levi was born.  Nothing.  I had no idea that 1 in 120 babies are born with a heart defect--40,000 babies a year in the US alone and 1 million worldwide, making it the most common type of birth defect. Public awareness is a critical step toward improving access to care, increasing scientific research and educational programs, and passing laws that will allow for crucial early diagnosis and treatment.  

Levi - age 2.5 years

Levi's diagnosis of critical aortic stenosis at two days old was a total surprise.  We had a 20 week ultrasound and were told everything looked great and we were having a boy!  We were so excited and I continued to have a smooth pregnancy.  Then our world was turned upside down when the "innocent" murmur that was heard at birth turned into a life or death heart defect that needed intervention immediately in order for our baby to survive.  Levi was airlifted in the middle of the night to another hospital and underwent an emergency balloon valvuloplasty to open his aortic valve.  At one year old, Levi had open heart surgery to repair this same valve as well as an arotic aneurysm.  

I was thrown into a medical world I knew nothing about and was forced to learn more about the heart and the care and the surgeries that my son will require for the rest of his life.  I didn't know to ask not just about the four heart chambers during the fetal ultrasound, but also about the four heart valves and the flow of blood.  Our son's diagnosis was missed. My son's heart defect could have been diagnosed before his birth.  We could have prepared and chosen the hospital that could best meet the emergency needs of our son.  Thankfully he survived!

Levi is doing well.  Thank God.  His heart will always be monitored and a source of concern for us.  Most likely he will need future open heart surgeries and a valve replacement at some point.  We don't know when.  It's a waiting game from appointment to appointment.  

CHD Awareness Week helps bring pediatric heart disease to the forefront to inspire those who can make a difference.  Survival rates for children with complex heart defects have improved dramatically over the past few decades but early diagnosis and treatment can make the difference. 

Levi's Frightening Illness

a late night in the ER

Levi was scheduled for a routine cardiology appointment this morning.  Unexpectedly, we had to move it up two days to rule our several possible scary infections that could have been wreaking havoc on his heart.  Beginning eleven days ago, Levi started experiencing some bizarre and frightening symptoms.  All of a sudden, after a totally normal morning, Levi started complaining about pain in his legs and in less than an hour, couldn't walk or bear weight.  Over the next days this trouble walking came and went and was accompanied by a persistent fever, a hive-like looking rash, swelling of his feet, hands, knees, eyelids, lips that came and went, cough, oxygen sat was down to 91% at one point, and some odd swelling on his forehead.  It was super scary and new symptoms seemed to crop up every day.  What was happening to my baby?!

swelling

horrible hives

Levi is FINALLY on the mend and just has a little remaining rash and occasional stiffness or pain in his legs.  We don't know for sure what's been causing this, but we've had one urgent care visit, one ER visit, five pediatrician appointments, consult by the one pediatrician with a rheumatologist, one set of chest x-rays (normal), three days of blood testing, and a urine sample!  He continues to take antihistamines to help with the rash and ibuprofen and acetaminophen for pain as needed.  But like I said, he's finally just about back to normal.

swollen hands

waiting for more tests

Levi's cardiologist also squeezed him in early for an echocardiogram and EKG on Tuesday morning.  The best news is that whatever this is/was, it does NOT seem to be affecting his heart.  We were so scared it was.  In fact, Levi's heart looks as good as can be expected!  His heart function has not changed much at all since his last cardiologist appointment in May 2012.  He still has moderate aortic stenosis, but it has not progressed (mean pressure gradient of 28, peak gradient of 48).  His aortic insufficiency (leakage) remains trivial, he still has mild left ventricular hypertrophy, and his ascending aorta looks normal (previously he had surgery for dilation/aneurysm).  This was a huge relief that things are still holding steady!  The doctor says he can go a year till his next appointment!

swollen forehead above right eye

As far as Levi's blood work goes, two tests indicate he has increased inflammation in his body, but can't specify the cause.  His latest blood work shows that these levels are decreasing though, so that's good.  His other tests have looked normal so far and we're still waiting for one more set of results.  After ruling out things like rheumatic fever, Kawasaki disease, endocarditis, and juvenile arthritis, it seems this may just be some strange allergic reaction to a virus that's just really thrown Levi's body for a loop.  Ultimately, if symptoms such as joint swelling and fever return at a later time, then this may be a bigger issue, but for the time being we can't put an exact finger on it.  Up until about three days ago, before the echo and blood work came back, I was a nervous wreck, so afraid that my sweet little boy might have to endure another freak diagnosis.  He was such a sick little guy.  I am SO incredibly thankful that he is finally is on the mend and appears to be almost back to normal!

just about back to normal!

Other Heart Families

I was so excited and moved when I saw the cover story of our local newspaper this morning.  Lindsey Bingham, a nine-year-old girl from Oregon, whose story I have been following, is finally receiving a heart today at Lucile Packard Children's Hospital at Stanford.  After an 8 month wait in the hospital on a mechanical heart, this little girl is getting a second chance at life with a heart transplant that she so gravely needed.  It's her Heart Day.  Finally.  Unbelievably, she is the second child in her family to receive a heart transplant.  And while this is cause for great celebration, it also means that another family is grieving for the child they just lost.  What an extraordinary gift of life and hope this family has given, even in the midst of their own unfathomable grief.  Please pray for the donor family and for Lindsey as she begins a long road to recovery.  As I read the Bingham blog this morning, it was hard not to get choked up. What an awesome time of medical advances that we live in.  What an extraordinarily difficult road it can be for so many CHD children and families.  

Just after reading about Lindsey's special heart day, the sad news came that a baby boy in Oregon, only a few weeks old and born with a heart defect and recovering from surgery, tragically passed away yesterday.  I can't imagine that kind of loss and it happens all too often.  Nearly one-third of birth defect-related deaths of infants are caused by congenital heart defects.

I am forever grateful for the doctors who worked on Levi's heart and gave him a chance for a better life.

So, Happy Heart Day.  Hug the ones you love and hold them close.

all three munchkins at Christmas dinner

Grandma with her grandsons (and the matching shirts she brought them :))