Second Opinion from Boston Children's

I received a phone call at around 5:30 this evening from the amazing Dr Marx at Boston Children's.  I just have to begin with saying that he is one of the most kind hearted, humble, caring doctors I have ever had the pleasure of speaking with.  One of a kind. 

About six weeks ago, I contacted Dr Marx via email.  I gave him the run down on Owen's cardiac history and asked him if he would review Owen's case for a second opinion.  The entire team at Boston Children's comes highly recommended as they are the top cardiac center in the nation.  He immediately replied to my email and asked me to send him some medical documents. 

I sent his most recent cath report, echo, ekg, labs and clinical notes from Stanford.  He called me on Sunday, the 5th of the month and let me know that he had a chance to fully review everything and was just going to speak with Dr Del Nido, their head cardiothorasic surgeon, before giving me his opinion. 

I have been anxious to get this call.   As we prepared to list Owen for transplant over the past weeks, I have been so stressed.  The thought of moving forward with transplant has had me a ball of nerves.  I just wanted confirmation that we are making the right decision and have exhausted all other surgical options for his heart. 

I received that confirmation today.

Dr Marx and Dr Del Nido had the chance to review Owen's records last week, and again last night.  They took their time on his case and wanted to make sure there was nothing they were missing.  I will do my best to explain here. 

Here is a picture of Owen's current heart anatomy.  The blue part is his Right Atrium, Tricuspid Valve and Right Ventricle.  The red part is his non existent Left Ventricle.

Owen's Tricuspid Valve has always been severely leaking.  It backs up into his Right Atrium and that is why his heart is so dialated and enlarged.  We have tried on several occasions to repair the leaky valve, but it always reverts back to severe regurgitation.  The last valve repair was done by Dr Hanley at Stanford in Feb 2011 and he is one of the best surgeons.  His tricuspid valve has remained at a moderate regurge, but also has a mild/mod stenosis due to the scar tissue on the valve leaflets.  It is only going to get worse with time. 

I was hoping that Dr Del Nido could repair the valve in some way, but here is how Dr Marx explained it.  Owen's right ventricle (which is the single ventricle that he has) has a moderate dysfunction.  It doesn't squeeze and relax fully.  If we fix his valve and get it working perfectly, which is not probable, then it will still continue to leak over time because his right ventricle function is not working perfectly.  So the valve is leaking because of the moderately depressed function.  So, this rules out a valve repair because you can't fix one issue and not the other.

Currently, the only way to improve the ventricle function on a single ventricle patient is by medicine therapy.  Dr Marx agreed that Owen is on the highest doses possible of every medicine out there.  There is nothing we could change to improve his function. 

Dr Marx continued on to give me a possible surgical option because that is was the team at Boston does.. they try to find a way to give hope.  There is a surgical procedure that can be done where Dr Del Nido can band (almost with a rubberband) off his right ventricle to decrease the dilatation of the ventricle.  Then he can go in and attempt to repair the valve further.  The risk?  They have only done this one time on one patient.  There is no research, data, or success rates to go off of.  The infant that had this procedure done is doing great, but that doesn't mean that Owen will do well with it. 

Another issue was that there is no guarantee that even with this surgery, that Owen would ever be a candidate for the Fontan in the future.

The next issue with this surgical option is that if surgery doesn't work out in his favor, we are out of options.  He could possibly end up on ECMO and that is extremely dangerous for Glenn anatomy patients.  The candidacy for transplant could also be eliminated.  It's a big risk.  Dr Marx said it is something that we should only consider if we are thinking "we will do anything to avoid transplant."

I went on to ask him his honest opinion if we are making the right choice to move on with transplant.  Of course, doctors like to give different options to parents and leave the difficult choice up to us.  He couldn't tell me either way what he would choose because both options come with high risk. 

We talked about Owen's current status on the transplant list.  He was very impressed that we are able to wait in Phoenix for a heart at Stanford.  I felt like he genuinely cares about Owen's well being. My biggest concern being long term outcomes and quality of life.  He was completely honest with me and said "I do not envy your decision, it is difficult." 

Difficult indeed. 

Dr Marx ended the conversation asking that I please keep in touch and send him emails on how Owen is doing.  Both he and Dr Del Nido took an interest in Owen's case and I know they both put a lot of thought into what is best for him. 

I am going to take some time to pray about things.  I plan on talking to Dr Rosenthal, our cardio at Stanford.  Right now, I feel like I got the confirmation I so desperately was searching for.  I feel like we are going down the right path for Owen. 

From the very beginning, in September 2007, when we found out at our ultrasound about Owen's heart, I have followed my heart.  We had a couple surgeries at PCH, moved onto UCLA and CHLA for second and third opinions.  Moved on from UCLA to Stanford.  Now to Boston for their opinion.  I have prayed.  I have followed those answered prayers and we have had quite the journey.  I never would have imagined in that ultrasound room 5 years ago, where we would be today!  I am so grateful for the amazing boy we have, the woman I have become, the support my husband has provided and the sacrifices that Kamryn has made.

I know we are moving in the right direction. We just pray that a heart comes before his tired heart wears out.  I forget how sick he is on a daily basis.  When talking to Dr Marx tonight, he really laid it out there and my heart just broke.  Leaky valves, poor function, lack of oxygen, trouble eating, easily winded... I forget that my fun spirited boy is so sick.

Please continue to pray for Owen in the upcoming months as we wait for a call.