Raffle Fundraiser

Contact me at karawley03@yahoo.com 

Or 816-885-9525 for tix

Our address is 

1418 NE 93rd St

KCMO 64155

*if you mail me money for tix, I need name, phone number and email and I will fill out your raffle tix and send you an email with a photo of your numbers. Need not have your copy of tix to win-I just need to have your contact info. 

Emotional week

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. 

Eleven and counting.

Eleven years ago today, I was preparing to marry the love of my life. 

Thank you, Brian for being the one beside me the last 11 years. 

For marrying a girl with a kid who you fell in love with also and leapt right in to be a husband and step-father without a moment's hesitation. 

For giving me Parker, so much like both of us, it's scary. Well, except for the height- I have no idea who he gets that from. 

For giving me Ethan, and through all our struggles with him, being my support. 

We don't always agree on everything-and if we did, how boring would we be?

I can count on you remaining calm in the middle of one of my episodes of craziness, and not holding it against me later. 

For being my rock last year and doing the things I couldn't that needed to be done.

For reassuring the kids when they were little that there were no monsters, and continuing to protect them from real-life monsters now that they are older. 

For shaking your head, but not saying anything with each new project I start. But never finish. 

For making me laugh. 

And understanding when I cry. 

Thank you for being my husband and saying "I do" eleven years ago. 

And for sticking by me through all those vows we shared, and put to the test. 

Because its been a lot different journey than what I thought it would be like on that hot, humid June day in 2002, but one I wouldn't change a bit. 

Happy Anniversary honey. Here's to many, many more. 

The verdict is in.

I heard from Dr Kaine on Tuesday about his presentation of the bald kid to the Team. The team consisted of 11 Cardiologists and two surgeons.   And it was much the news I expected. 

They were extremely discouraged to find Ethan the star of the cardiac valve failure show again in less than two years. 

Tell me about it. 

None of them felt any surgical intervention should be done on Ethan.  Rather, the general consensus was to continue medical management until there was no other choice but surgical intervention. 

Dr Kaine's argument with them was that we don't have the time or the luxury to wait, and  talked them into putting him on the schedule for a PV replacement anytime after August 1st.  Being that we have a follow up with him on July 31st. And if we wait until then and there is no improvement, we will have lost some good months for scheduling and would be looking into fall.  If there is great improvement, we can always cancel, and Dr Kaine said he would love for that to happen and he would happily eat his words, but he's not optimistic.  

Here's the kicker-the surgeons would not buy off on the Hybrid Melody approach which would keep E off cardiopulmonary bypass. They don't feel they have enough, well actually any experience with this approach and don't want to risk it. Dr K said that they felt that even if he still wanted to use the Melody Valve, they would do a traditional open-chest approach anyway, so why not just do the same surgery they did two years ago. It was also felt that they don't have enough success with the Melody Valve, or stent valves, here yet either to use it on Ethan, so just stick with another cadaver valve. 

Ahem. 

I thanked Dr K for his support and care of Ethan since he was born, and told him it was no reflection on the decision or the care at Mercy, but I felt it was time to pursue all our options and that Ethan's records had already been sent to Boston and Philly. 

His next statement kind of surprised me. 

"Rest easy, Kari. There are no hard feelings with you seeking second and third opinions. The more eyes we have on Ethan with the experience those places have in regard to valve replacements, the better". 

Whew. I almost cried. I don't know why that was so hard to do. 

Because we've admired this man for 9 years and put the life of our child in his hands.

Because we respect the decisions he's made in the best interest of our little bald guy for those nine years and didn't want to offend him. 

Because I honestly can't imagine traveling somewhere else where they don't know our guy and feel like we are starting all over. 

Because Mercy is our second home. 

And I wanted the quick and dirty solution, the easy answer, the road most traveled. 

But I ultimately knew they couldn't or wouldn't be able to offer me the approach I wanted, and saying those words made it all so much more real. 

That's part of why it was so hard. 

But a dear, sweet lady I spoke with last week said it best. 

I wanted his approval, his blessing. 

And I probably wasn't going to get it. 

And if I did, the answer I was looking for was in that approval. 

And deep down, in my heart of hearts, I already knew what the answer was going to be. 

"And I am happy to help get him there if needed."  

At the end of the day, approval or not, we are going to get all our options for Ethan and do the best we can for him. 

Even if it is the road less traveled. 

With that said, Ethan's records are in Boston and Philly now. Both places called today for more records, and those were overnighted tonight-thanks to the help of Jennifer from Mercy's Medical Records department!  I should be hearing from them both by the end of this week, or early next week. 

Immunology called me today, after getting a copy of E's latest inpatient notes. And we had a very interesting convo. They are concerned that the repeat valve failure could be related to his CVID (Common Variable Immune Deficiency) and want some labs drawn to check his IgG before anyone starts cutting on him. 

Finally, someone is looking for a reason for the failure.  So, maybe, we don't do this a third time in two more years. 

And as if we didn't have enough going on, we are currently building a house. We just signed on the dotted line Tuesday after meeting with the builder.  The home we live in now was a lease-to-purchase option, and the owners are pushing us to buy. We really need to have Ethan's bedroom on the main level, and this two story ain't cutting it with my bum knee and 15 curved stairs to get him from his bedroom to the main level.  Our new house is a Reverse Story and a Half with his and our bedroom on the main level with the kitchen, living room, dining room and laundry. And only two steps to get him in the house.  And the awesome builder we are using is putting a wider door from the garage to the house for E's wheelchair, and a small ramp in the garage so we don't even have to carry him up or down those two steps-we can wheel him!!  The foundation is poured, but it won't be done until October, so we have lots to keep us busy this summer!  

Nice huh? I will keep you posted with pics as it progresses!

Parker is worn slick from football camp this week, he was in bed by 7pm tonight. We are so thrilled to see how excited he has been about it, and can't wait to watch him play.  He also has a birthday coming up in July, oh dear, he will be 14. Where did the time go??  

Best of luck to my nephew Taylor in his Powerhouse Baseball tourney in St Louis this weekend!  Safe travels!

And my thoughts and prayers with a fellow Ch18 family in Utah whose gorgeous little Ring 18  Deak is having a major, major hip surgery tomorrow-basically a bilateral hip replacement.   His Mom Jenny is my online friend and bald kid supporter.  Please send her your strong vibes tomorrow and in the days following as Deak recovers. 

www.jennyandblair.blogspot.com 

We love you Howes!  Thinking of you!!

Leaving you all with a fave pic of ours-Ethan's school pic from last year. 

He wasn't smiling so his teacher said to him "you better smile you bad, bad boy" and he cracked up. 

We love that silly smile.  Thanks for letting us share him with you all. 

Happy Fathers's Day!

No witty words, or Top Ten like last year, just a heartfelt thanks to all the Dads in my life.  

To my Dad for always being there, even in times of my poor decision making. 

I look to your for your wisdom and advice, and you have never let me down. 

I admire you for your honesty, integrity and character, and hope that I can emulate what I have learned from you over the years 

Thank you for being the best Dad ever. 

To my Brothers-Kyle and Eddie-it's a miracle we all ever got out of our teen years unscathed, let alone married and with children of our own. I love that my kids have cousins to grow up with and consider them friends.  I only wish we were closer, though it just makes the time we do spend together more cherished.  Thank you for marrying a couple of awesome women. You both make me proud to be your sister.  You rock!

To my BIL-Trevor-I still don't know know how you've managed to stay married to my sister for as long as you have, but we're glad you did!!  Kim and the boys are truly the lucky ones.  As we are to have you a part of our crazy family. 

To Brian, my baby-daddy.  I love you more today than I ever thought I could.  We have weathered so many storms together, and I have needed you by my side to get through them. 

Thank you for working so hard for our family.  

You are the bread to my peanut butter, wasabi to my sushi, protein to my shake and pot to my kettle. 

Me love you long time. 

You have been the voice of reason in my moments of insanity, and talked me off the ledge many times. Thus, saving the lives of our children on more than one occasion I'm sure.  For that, the kids love you lots. 

You are our Superman. Our hero. We love you. 

And to all the women in my life who have had to take on the role of Father as well as Mother when necessary-you have done a great job, and this day is as much for you. 

To all my awesome Uncles, cousins and cousin-in-law Dads-I hope you know how special you are to me!  

Cheers to all The Dads out there today, and every day. 

Back at square one

Thinking I was going to get the answers I wanted, no needed, today was really wishful thinking I now see.   

First off-good news: Ethan has responded well to the medical management of drugs and oxygen. His pulmonary artery pressures are back down in the 30's, where they had been in the 50's two weeks ago. 

His sats were 100 today on 2 liters of oxygen. 

He smiled and laughed and even shook his head "No" at Dr Kaine when he mentioned surgery. 

Bad news:  Dr Kaine felt Ethan looked good, and his echo looked well enough today that we could continue medical management for months, maybe even a year.  I know this doesn't sound like bad news, but we are right back to sitting, waiting for him to get worse before intervening. 

And we would need to keep him healthy in the meantime.  Which means no school, no traveling, and a basically miserable bald kid.

 Dr Kaine has not yet presented Ethan's situation to the team, nor has he talked to the surgeons-Dr O'Brien or the new guy Dr Pastuszko, about the plan he discussed with me two weeks ago. 

And since Ethan's surgeon Dr Lofland has retired and is gone now, he will be presenting this to two surgeons who don't even know E. 

He also mentioned a possible problem with doing the hybrid surgery we discussed.  During the cath before he could pre-stent, they would inflate a balloon in the vessel to make sure it doesn't compress Ethan's coronary arteries. If it does, then he's not even a candidate for the Melody valve at all. 

If it could handle the stent and thus the valve, we wouldn't necessarily need to wait 10-14 days before putting the valve in, it could be done right then. Again, if the surgeon agreed, who doesn't even know about this potential surgery yet, because Dr Kaine hasn't even talked to him about it. 

Argh!!  All of these hinge on the surgeons, and the other Cardiologists conferring and agreeing that this is the best path down which to proceed with Ethan. 

Which I was under the impression would have already been done by now. 

Dr Kaine initially told me he wanted to get Ethan in the cath lab in 2-3 weeks. Tomorrow is two weeks post discharge.  I was under the impression we would be leaving clinic today with a cath date in the next week. How had Dr Kaine planned to get that done having not even talked to the team??  

Oh, wait, I'm guessing that really wasn't his plan at all. At least, from my standpoint it doesn't seem it was.  

And the real issue....Ethan looked good today because we do have him on 2 liters, and yes his sats were 100, but if we drop him to 1.5, he drops to 88-89 in 10 minutes. And it's not even the sats that concern me, if he's below 2 liters, he begins working harder to breathe, becomes fussy and pants. He becomes uncomfortable, which means his heart is having to work harder. 

He also has only been able to tolerate about half his daily feeds.  He can get almost his whole normal morning feed down, but as we proceed to feed him during the day, he begins to retch, cough, hiccup, fuss and become diaphoretic. 

And the real kicker?  Ethan has lost 1 kg, or 2.2 lbs since discharge.  And it's not all just fluid loss from the Lasix.  It's because he's not getting all the calories he needs, and his heart is burning them faster than we can get them in him. 

Only when we shared with Dr Kaine the issues with the oxygen and feeds, and that now was the time we would be looking at CHoP and Boston for any other available options, was when he decided he would present Ethan at conference either this Friday or next Tuesday. 

 When I asked if he felt replacing the valve would ultimately fix the problem, and how many times has he seen this scenario with Tet kids before, he answered cryptically with;

I wouldn't have believed it if you told me two years ago we would be here again with a failed valve after such a short time. 

We know Ethan recovers well when his right heart isn't stressed BUT

he does have this underlying genetic issue that we aren't certain wont play into the outcome. 

Aha. The real answer. The big fear that had been lurking in the back of my mind was affirmed. 

Dr Kaine may not be able to get the team to buy off on a never-performed (at Mercy)hybrid surgery for a kiddo like Ethan. 

Which is why he hasn't mentioned it to them I'm guessing. 

Maybe he didn't mean it that way, but that's how I took it. 

Having this wonderfully complex kid for the last nine years has afforded me the luxury of spotting the look of pity.  

So I will give them until next Tuesday. But in the meantime, I will be on the phone with Boston and CHoP, cause I'm not waiting for months. 

And now I'm mad at myself for not using these last two weeks to move forward with a second opinion, because I thought we had a great plan here. 

Sitting.  Waiting. Watching.

Not this time.

A balancing act....

Ethan has been at home for a week today, and is doing ok.  

He came home with oxygen and instructions to go anywhere from 1/4 liter to 2 liters to keep his sats at 92. He was on 1/4 for the first few days, but we have since had to bump him up to 2 liters to keep him comfortable. 

He had his post-discharge follow up with Dr Russell on Tuesday, and she welled up with tears asking if we felt she had missed something when she saw him the week before his most recent admission. I assured her she DID NOT miss anything with the bald kid she knows so well, he went crappy so fast and it all happened after our visit with her.  

In listening to him, she confirmed what we already knew-his lungs were beginning to sound crackly again, and he was showing a little increased work of breathing. But he was pretty feisty with her too, and she was happy to see that!  A balancing act she called it. When she said she wasn't sure he was to going to be able to stay out of the hospital before his cath, or even before seeing Dr Kaine next week, the bald kid began emphatically shaking his head NO!  She hugged him and rubbed his head and I was able to snap a pic of the two of them.  We just love her!

He is really beginning to struggle with his feeds, and we may have to revisit going back to jejunal feeds to avoid an aspiration.  He tolerates his first two feeds of the day ok, but by the third one, he's panting and sweaty, and retching, so we've slowed the rate way down and broken each feed up into two separate feeds so as not to wear him out.  He's sleeping 12-14 hours at night and taking naps during the day.  But when he does feel good, he's kicking and doing crunches and bridges until he wears himself out!  To say Ethan doesn't tolerate pulmonary insufficiency very well is putting it mildly I would say.  

If we can't keep him comfortable at home, we may be going back in. At least Dr Kaine is on service this month! 

 I worry about him being well enough for anesthesia for a cath.  But I worry waiting that day won't come, and he will only get sicker. 

I worry about whether Dr O'Brien has agreed to do the Hybrid version of the Melody Valve placement Dr Kaine and I talked about. And I worry if he doesn't, what is our other option. Dr Russell said we can still get him to another facility if someone else has a solution, but Ethan cannot travel by commercial air at this point and he would need to go by fixed wing medical transport. Argh!  The worrying is worrying me. I think I'm holding it all together right now pretty well. Brian and I have had some serious talks in the last week about stuff you never want to have to discuss in regard to your children, but necessary. 

I know I am silently hoping and waiting that seeing Dr Kaine next Tuesday will bring me some more definitive answers and a bit of relief from the worry.  

Expect and prepare for the worst, and it won't happen. 

At least that's what I keep telling myself. 

While an in-patient, Ethan is still followed by the PACC Team, or Pediatric Advanced Comfort Care Team. Linda is our contact person that comes in and chats with Brian and I during his stays. This woman is amazing and I just love her, and what her team provides for families.  She also confided something to me this last stay that just sealed our bond.  

She gave me a book to read that I just finished. 

"Shelter from the Storm; Caring for a child with a Life-Threatening Condition". 

It is an excellent book, and for parents like us, I highly recommend it. I couldn't read it start to finish, had to put it down for a couple of days and then pick it back up when I had time to re-group.  It's not stories about these types of situations, it's written from the perspective of a pediatric oncologist and another pediatric physician with the input from families they treated.  It's about being able to process and accept and arrange a peaceful death for a child. It was very validating to me, and drove home some of the things Brian and I have already been discussing. If you are the parent, care-giver, grandparent, aunt, uncle, cousin, or friend to the parents of a child with a life-threatening condition, I suggest you read it, and I am happy to loan you my copy. Not all parts or chapters will apply to each case, but I guarantee it will help if ever faced with this situation. 

In the meantime, life goes on.  Hailey started volleyball camp this week and conditioning sessions at 6am!!  Parker started freshman football camp, complete with weight and conditioning sessions from 6am-noon Tuesday-Fridays all summer. And on top of that, he is going to basketball camp immediately following football from noon-2!  He was plum tired the first day he came home. He took a shower and went and laid down and was asleep before his head hit the pillow I'm sure.  We had to wake him up and make him eat dinner before climbing back in bed!  

Both kids are going to be busy this summer, which helps take their minds off of Ethan. 

Ethan's PCA, Denise, was at the house today so I could sleep before work, and he's been missing social interaction so much,  that after she left he cried for 20 minutes. It was very sweet to see how attached he's gotten to her. We are also interviewing a CNA tomorrow for help while Brian and I are both working on the weekends, and the Monday evenings I will be working. Otherwise we aren't comfortable leaving him alone with the kids right now. We feel like that's too much pressure and don't want to do that to them. 

We are hoping to watch the Gunther boys play some ball this weekend, as it may be Kellen's pitching debut. But my work schedule change occurs this weekend, and we may not have enough oxygen tanks to keep the little bald guy where he needs to be!  We'll just have to wait and see. 

One day at a time is how we are taking things right now. One day at a time.