Such a busy week we've had, and it's only Wednesday!
First of all-Happy 14th to Parker, our very favorite middle child! July 2nd at 8:10am you came into this world and have brought us much happiness-and frustration. He is a 14yr old boy after all; dirty room, smelly laundry, junk and wrappers and water and Gatorade bottles under his bed and filling his nightstand drawer, eating us out of house and home and 'forgetting' his homework on more than one occasion. Always ready to argue his case for a later bedtime, more Playstation or TV time or dinner at Fuji. But he is also one of the sweetest boys I know. He loves his younger brother dearly and is his sister's best friend and worst enemy on any given day. He's always been my sensitive child, and wears his heart on his sleeve. Not a stellar athlete, or morning person, but getting up early every day for football camp and conditioning. We love you P and can't believe you will be a freshman this year!
Parker 9 months old. We call this his "fat guy in a little suit" picture. One of my faves!
Parker's pre-k school picture age 4. Pretty cute!
And the big news, the emotional news that has me 'giddy and terrified' at the same time. (Perfect description Jody).
Monday evening I received a phone call from Dr Christopher Baird from Children's Hospital of Boston. Dr Baird is the cardiothoracic surgeon with their Congenital Valve Program. He and Dr Gerald Marx, a cardiologist specializing in echocardiography and fetal echo reviewed Ethan's records.
The next few paragraphs will be described in medical terms, and then I will summarize the best I can in layman's terms.
Ethan does have a failed pulmonary valve (PV)-no question about it. But in the list of things wrong with his heart, it's the least of their concern.
Ethan has what they think is a dyskinetic septum that has suffered an infarction.
He also has ischemic coronary arteries, peripheral pulmonary stenosis, a deteriorated conduit, moderate tricuspid valve regurgitation, failed pulmonary valve, right and left heart dysfunction, pulmonary hypertension and pulmonary edema.
All of these are contributing to his rhythm problem, where he has bradycardiac episodes into the 30's. This combination of problems also puts Ethan at a very high risk of sudden cardiac death. The fact that he's not a typical, on the go, running, jumping nine year old has probably been what's kept him alive.
You can't even begin to imagine how my hands were shaking as he was talking to me.
And then he said what I've known all along.
"Replacing Ethan's pulmonary valve will not fix the problem. It's much more than that."
The septum is the muscle between the ventricles in the middle of the heart. And it is basically dead. Thus not working properly, or in conjunction with the normal pumping action of his left heart. This is why he has the left heart dysfunction and pulmonary edema this time. And is also why his heart rate tends to drop so low.
And the ischemia of his coronaries simply means the same thing-some of the tissue is dead.
This is why even after replacing his PV in 2011 his feet and hands still remain purple/blue/black from lack of oxygen getting out to them.
The right heart dysfunction and pulmonary valve failure is probably a result of the peripheral pulmonary stenosis. The blood simply can't get out to the lungs and is being pushed backwards though the valve. The tricuspid valve regurg is also contributing to the right heart dysfunction because it is the valve between the right atria and ventricle, where blood is already pooling from the failed valve. His right heart is just getting bogged down with blood that has nowhere to go.
So what do we do?
Dr Baird and Dr Marx's recommendations:
Get to Boston.
Sooner than later.
Urgently but not emergently.
We improve Ethan's quality of life. For good, not temporarily.
First he needs a cardiac MRI and cardiac cath to confirm the findings.
Once those are done and reviewed, they would present us with their recommendations for fixing these problems, if they have one.
But it would likely involve Bi-Ventricular Pacing, septal reformation, tricuspid valve repair, pulmonary stenting, and probable removal of the PV conduit and replacing it with either new conduit or a Melody valve, or both.
Whew.
My head is spinning, and has been since Monday.
The giddiness comes from the validation there was more wrong than just the PV failure and they have a solution.
The terror comes from the validation there was more wrong than just the PV failure and they have a solution.
See why my head is spinning??
There are no guarantees, and we know that. But we have to give Ethan the best shot possible. It's all or nothing. And unfortunately we have been offered nothing but a temporary fix here.
Right now we are working with the Boston Out of Region Coordinator for getting everything scheduled.
They would like to do the MRI and cath on the same day, like a Tuesday. Then on Wednesday we review the findings and decide on a course of action. And if surgery is one of those options, surgery the next day or day after. Yep, all in one week. The surgery date will tentatively be set so if needed, we use it, if not needed, cancelled.
It's looking like we will be headed out there end of July or first of August. Right now it all hinges on the MRI scheduling and being able to correlate an anesthesia spot with them and a cath on the same day.
The logistics of getting there are an issue too. Ethan probably can't fly commercially. And if he could, and he starts to go crappy I will freak out and the Air Marshall will have to taze me to calm me down and let's just say it won't be pretty all the way around. An air ambulance is very expensive. And not necessarily covered by our insurance. Funny thing is, in speaking with our insurance, if Ethan were an inpatient and critical, a facility to facility transfer would be much easier to arrange than an outpatient to possible inpatient status. So basically we need to wait until he's critically ill and this would be easier and quicker for them! Seriously? Being pro-active in keeping him and getting him well isn't convenient for them. Or urgent. But Boston does this all the time and works with insurance companies, so we are hoping for the best there.....but you know our luck.
In the meantime, we are formulating our own action plan.
We are considering driving out-me, Brian, E and a lot of oxygen. Brian staying the week of testing and potential surgery and me staying for the full post-op. Brian will fly home-thanks to the wonderful, generously awesome offer of a dear friend (JBJ) giving us her airline miles-and work as much off-duty as possible to help make up for my lost income during this time. Because, let's face it, I have pretty much used up every hour of time off I have accrued in the last year-I just finally built up a nice little cushion of 30 hours! Not gonna last long when the drive out is 24 hours, or two 12 hour days of driving. Then I will be left with a car, and I can sleep in E's hospital room. When it's time to come home-Brian, or my dear friend JBJ, can fly out and drive back with us!
There will definitely be some significant expense in doing this-besides the travel, food, hotel rooms,(we will stay at their medical house at a huge discount if we can, but no guarantee there will be room available). We will probably also have some bills that will simply not be covered by our insurance.
With that said, we hate to do it, but if he can't go, none of us are going, and we are going to raffle off Ethan's Taylor Swift concert tickets for Saturday August 3rd.
We have 5 tickets, section 108, row 25, seats 14-18, to the right of the stage, handicap accessible, at a value of $515. Whatever money we raise will be used for travel and medical expenses to get the bald kid fixed! I am getting the tix printed and flyers made so we can start selling them next week. The lucky winner will be drawn on Thursday, July 25th. If we are in town, we will have a get together and let the bald kid do it. If we are already gone, one of our friends will do it and make arrangements to get the tix to the winner. Raffle tickets will be 1 for $10 or 3 for $25.
I have a list of folks who will have the raffle tickets to sell, and will post as soon as they are available. Feel free to message me if you would like a flyer to post and some tix to sell for us, and we will get them to you.
We told Ethan what we were doing, and he furiously shook his head "NO" for about 5 minutes.
But we will do whatever we have to do to get him to Boston and get him better.
So next year he can hear his girlfriend live.
And with a fixed heart.
