Oakes is 4 Months Old Today!

And he is doing better than ever and looking cuter than ever. As soon as I got to the hospital this morning, I got busy getting ready for our photo shoot. Oakes got a bath, his hair washed, toenails trimmed, and about a dozen nibbles to the neck and another two dozen kisses!

Have we mentioned Oakes has 2 teeth? (if you click on the photo it will open much larger in another window so you can really see his pearly whites!)

Mid-shoot a nurse came in and offered to take a few pics, do we look alike?

Right after the photo shoot I dressed him and snuggled him back in bed where he quickly dozed off. But not before he smiled a few more times....

I asked if he was tired and he responded, "Just a little bit!"

And have we mentioned that he has found his thumb?!

Sucky-thumb sleeping with a smile, what a great day we have had....We love my little Oakesie!

A Happy & Hiccuping Oakes!

We are just in LOVE with this happy, smiling and noisy little man!!

A bigger update to come, but we have had an uneventful, stable, and healing week in the CICU. So much so that we are heading back to 7West tomorrow. The prayers are working, please keep them coming! Love to all of our friends and followers!

Mighty Oakes Update 7.3.11

Good evening friends and family.  We hope you are in the middle of a fantastic holiday weekend with your families.  The last email we wrote, we had told you that Oakes was doing really well and that he had recently been removed from the ventilator.  For the following week or so of that email, Oakes continued to exceed everyone's expectations.  You can check out the blog, but Becky got some amazing pictures of the Mighty Man.  For that week or so, he was smiling, engaging, super alert and very aware of everything going on around him.  It was undoubtedly the best condition we had seen him since late May, right before he had the emergency heart surgery on Memorial Day weekend.  Becky and I got to hold him numerous times last week and it was totally uplifting to be able to hold him, stare him in the eyes and have him smile back up at us.  As abnormal as our lives have been, there was a certain sense of normalcy around last week because he was just doing so well….

 

But our rollercoaster continued late this past week.  Although Oakes was off of the ventilator, he still was receiving a little help from a cannula delivering just a couple liters of oxygen to his lungs.  They had weaned him so much and he was tolerating it so well that he was moved to the recovery unit (7 West – literally down the hall from the CICU where he's been since 5/27).  But after just barely 36 hours on 7 West, Becky noticed him working a bit harder to breathe.  It got worse throughout the day and when the doctors came in, they did not like how much he was "retracting" (the heavy pulling he does under his ribs when he's struggling to breathe).  She knew right away, they were going to take him back to the CICU.  It was such an emotional letdown for all of us because he was just doing so well we had this false sense of hope that maybe he was going to figure out a way to get home with us….and then completely out of the blue, he turned for the worse.  He was struggling enough when they moved him back to the CICU this past Friday, that they decided to intubate him right away.  They completed an echocardiogram right away and took some labs.  The doctors were concerned enough by the results that they scheduled another cath procedure for first thing Saturday morning (yesterday).

 

If you recall, the last cath procedure he had did not go well at all.  As the doctors were prepping me and Becky for the cath, it was without a doubt, the most direct and the most ominous language they've used with us yet.  Dr. Balzer, the head of the cath lab and the doc who performs all of the major cath procedures at St. Louis Children's Hospital (SLCH), came to talk to us before they took Oakes to the cath lab.  He said that during the last cath, Oakes' pulmonary veins "spasmed" in a way that he had never seen.  He told us that because of that, this procedure was anything but a sure thing.  Their intent was to go in, review the blood flow in the heart and lungs and probably balloon all four pulmonary veins.  Dr. Balzer also mentioned the possibility of the insertion of stents depending on what they found.

 

What has been causing the breathing issues all along has been a disease called pulmonary venous stenosis, which is the narrowing of the pulmonary veins (four smaller veins that take blood from lungs back to the heart).  It's a brutal disease that gets progressively worse over time and because of the fact that most of these veins exist deep within the tissue of the lungs, there is no operation that exists which can be a quick fix like the operation that Oakes had on 5/28 when they were able to increase the size of his pulmonary arteries (two larger arteries which take blood from the heart out to the lungs).  The cath they did on 6/9, scary as it was, actually succeeded in temporarily relieving the narrowing that had occurred in the pulmonary veins, but all of the doctors were very clear that the stenosis would return and continue to worsen.  Sure enough, that's what was happening to poor Oakesie the last week – the veins were just getting more and more narrow and his heart was working harder and harder to get blood through it. 

 

As the doctors were assessing Oakes' condition Friday into the afternoon and evening, they began to relay to us that the cath was needed to buy Oakes more time until they can find a proper donor set of lungs.  The slight hope that we had held on to around the small percentage chance that there could be a surgical fix before resorting to a transplant was gone – a lung transplant is definitely on the horizon for Mighty Oakes.  Although the doctors were clear this was a long shot, it was still a crushing dose of reality for us both.

 

As Dr. Balzer was going through his final words of wisdom prior to taking Oakes to the cath lab, Becky and I were half listening to him and half staring at Oakes who had been sedated and sleeping since we had arrived at 9am.  All of a sudden, with Oakes' head facing both of us, he widely opened his eyes and looked right at us!  We immediately thrust our heads down toward him to talk to him.  I told him his papa was there and that we loved him.  He looked right at me and gave me a big ol' smile!  He then looked at Becky and did the same thing.  We, of course, started crying.  It was like Oakes, the one who should be scared, was the one smiling and calming his mama and papa.  It was something we'll never forget.  Of course, the doctors didn't like how awake he was, so they gave him a triple shot of sedation medication and within a couple minutes he was back asleep, but what a moment!!  It was crazy because in the past when he wakes up, he struggles to open his eyes and kind of opens one before the other, but here, he just opened them both right at the same time.

 

So the cath went surprisingly quick, maybe three hours all told.  Dr. Balzer did indeed insert three stents – one to his lower right pulmonary vein (PV), one to his upper left PV and one to his lower left PV.  The venous tissue will ultimately grow in and through these stents which, again, means that the next step has to be a lung transplant.  These stents cannot be removed without significant complications.  What the stents will do is provide more longer term support, hopefully, for Oakes.  They're more permanent and will keep the stenosis at bay, but only where they were inserted; the stenosis will continue elsewhere along his pulmonary veins.

 

So what happens if we haven't found suitable lungs and the stenosis further down his PVs are causing breathing issues?  Then we'd be introduced to the Quadrox machine, the pediatric version.  The Quadrox machine is essentially artificial lung support.  You will find this hard to believe, but the Quadrox is a small "box" that sits at the foot of the patient's bed and oxygenates the patient's blood.  It literally has tubes that run from the heart to the machine and back to the heart, so that the machine is essentially acting as the body's lungs.  Is that insane?!??!  The pediatric version, we believe, was just publicly released in late April of this year.  There have been, we think, three children here at SLCH who have been put on it, including our friends daughter, Elizabeth, just a couple rooms away.  The doctors have told us Oakes would be the ideal type of patient for Quadrox, if he, in fact, does need it.  This is a bit too intense for me to process just yet, but at least I've introduced it in case it becomes reality.  If you're so inclined, here's a link to a picture of the Quadrox - http://www.maquet.com/productPage.aspx?m1=112599774495&languageID=1&titleCountryID=103&productConfigID=126054034616&productGroupID=121118652068 .

 

Thus far, Oakes has done well since yesterday's cath – he's not retracting, he's responding to his medications and he's been calming down very nicely when he gets agitated.  They are very aggressively weaning him off of the ventilator since they believe the stents will better support him this time around.  He's still fairly medicated, but is slowly becoming more alert.  He's sucking on his ventilator tube as I type.  And believe it or not, their goal, as of now, is to extubate him by tomorrow afternoon or evening.  They've already reduced his support down to levels that it took more than a week to reduce after the last cath so it appears the stents are doing what they're supposed to do and that Oakes is kicking butt, as usual. 

 

I know I am biased, but I cannot be more proud of my son.  He just had his fifth surgical procedure in just over three months and continues to rise to each challenge in front of him.  He was not blessed with perfect organs but it's so clear to us that he was blessed with incredible strength and determination and the amazing ability to inspire thousands of people he's never yet met.

 

Becky's oldest brother, Phillip, got married a week ago Saturday (6/25) in Oklahoma City.  Our daughter, Isla, was scheduled to be the flower girl.  With everything going on with Oakes, we just didn't know if we could make the wedding or not.  Also, I had a big company meeting in Dallas for four days last week.  I've not been able to travel much, for obvious reasons, but this was a trip I really had to try to make.  So Oakes being Oakes rallies so well the week leading up to the wedding, that Dr. Gazit told Becky he thought she should go to the wedding, so her and Isla flew in to OKC Saturday morning, went to the wedding, had an absolute blast, then flew back Sunday afternoon.  Phillip and his new bride, Kat, had a great wedding and the pictures of Isla and her cousins romping around the dance floor were hilarious.  Oakes maintained his stability throughout last week allowing me to complete my trip to Dallas.  It was literally upon landing that Becky was told he was moving back to the CICU.  It was such an awesome gift to his parents – it was as if he was saying "I'll be fine guys, go do your thing."  His little body held out just long enough for both of us to be back in town.

 

Becky's dad, Bill, and stepmom, Jan, helped out this past week (again) while I was out of town and her mom, Connie, and stepdad, Dale, have been here this weekend.  Between the four of them, they've completed about 10 home and yard improvement projects around our house that we have had neither the time or the talent (me), or both, to do on our own.  People keep dropping off food and groceries and sending cards and prayers and well wishes and emails and texts.  You are all too incredible and we never, ever take our friends and family for granted. 

 

In case you do better with pictures vs. my ten thousand words, I've updated the PowerPoint I did a couple emails ago.  I also added a great pic of Oakes from earlier last week when Beck gave him a faux hawk; it's priceless.

 

We will keep you posted, but you may want to subscribe to Becky's blog so that you  get updates in between my email novels….Happy 4^th everyone!!

 

Love,

 

Greg, Becky, Isla P. & Mighty Oakes

www.babyortyl.blogspot.com

 

 

Latest pictures

Lots of smiles and crazy hair. When Oakes has a good day, we all have a good day. Oakes was just moved from the CICU to 7West, the step-down unit. We literally moved about 30 feet, but it is 30 feet closer to home, and we should be home in about a week!

These were taken on Monday...lots of smiles!

Oakes watching his mobile.

...and swinging at it!

Last weekend my brother Phillip got married in Oklahoma City. After hearing from the Attending CICU doctor that he thought I could do a 24 hours trip, I packed Isla and we were off. We had a wonderful time! Referring to the weekend Isla will tell you, "Just girls, two planes!"

Mr & Mrs Fox!

Hanging out at the airport with Ena and T-Franny!

Around and around they went!

Isla having a hollywood moment!

Isla loves her family!

These pictures were taken several weeks ago when Oakes was still sedated and intubated. The nurse was changing his bedding and I got to hold him for about two minutes. Two minutes that we both LOVED!

The last three images were from the scary night when we were told that Oakes would not make it through the night. We had T-Franny bring Isla up to give Oakes kisses and we also had Oakes baptised. We are so grateful and thankful for every little miracle that has happened in our and Oakes' life to get us to where we are today (refer to the first picture in this post)!

Love and hugs to everyone that continues to support us and pray for Oakes and our family! XOXO

Mighty Oakes Update 6.23.11

All, it’s been a couple weeks since our last update.  As you know the last update was gut-wrenching, but this one will have a bit more of an optimistic tone.  Last we talked, we were told that Oakes required a double-lung transplant in order to survive longer term. 

 

There have been a few key updates since then (apologies if some of this is a bit repetitive):

 

1.       Lung transplant list.  Oakes is officially on the lung transplant list as of last Friday.  It typically takes two to three months for a baby Oakes’ age to find donor lungs.  The process for this, as we have learned, is pretty crazy.  Apparently, there will be several offers to St. Louis Children’s Hospital (SLCH) as donor lungs become available, but they will not be accepted for various reasons after intensely reviewing medical records and other pictures/videos available of the donor.  As an FYI, donor lungs become available when another baby has been declared brain dead and the donor’s parents are willing to donate their baby’s organs – an amazing scenario that is hard to even contemplate in our position.  Once an offer is accepted by SLCH, it gets pretty tense and exciting.  Apparently, whichever cardiothoracic surgeon is not on duty (either Dr. Huddleston or Dr. Eghtesady) will fly to the donor’s home city, with a small team of assistants, and thoroughly examine the donor.  Assuming that goes okay, the SLCH surgeon will perform the harvesting of the lungs and situate them in a Coleman cooler.  They’ll immediately return to St. Louis and the operation will follow closely after their arrival.  We’ll be notified relatively early in that process, but if we get the call, it’ll be very urgent that we get to the hospital.  There is the chance that, at any time, the organs can be rejected due to mishandling or other reasons (makes sense why SLCH handles the organs the entire time; I love this type of focus!).

2.       Pre-transplant possibilities.  Dr. Huddleston has continued to leave open the very, very slight possibility of an attempt at a surgical fix before we proceed to the lung transplant, which is clearly the last resort because of the poor mortality rate post-op.  It sounds to us that he may not know for many more days, or possibly even weeks, if he’ll elect to go down this path.  Most of this decision will be dictated by how Oakes recovers from his cath procedure a couple weeks back, how he handles extubation and what the future echocardiograms show.

3.       Extubation.  Speaking of extubation, we had a HUGE day this past Tuesday as Oakes was extubated (taken off the ventilator).  They had been very slowly weaning him off the machine the last couple weeks.  It was challenging because Oakes had a couple bad days including catching a bacteria from the ventilator tube itself (another reason they try to get them off the machine asap), but ultimately he gave them enough confidence to extubate.  We were definitely holding our breath during extubation.  They slowly peeled the tape off Oakes, which hurt the little man quite a bit and once they had the tape off, they literally yanked the tube right out of his throat and mouth.  He fussed quite a bit and they quickly put an oxygen mask on him, which he of course hated as well.  But his numbers (oxygen levels, blood pressure, carbon dioxide output, heart rate, etc.) were excellent!  That was the key.  Once we got him to calm down, things were better.  Now just because he was off the ventilator didn’t mean that he was off oxygen entirely.  They have to continue to give him help for a while before they try to wean him off oxygen entirely, so they had this ugly mask that covered just his nose and was wrapped several times all over his head.  That only lasted about 36 hours because this morning at 5am he decided he had had enough and threw a big fit.  So they pulled the mask off and replaced it with a cannula breathing tube (the tube you’ve seen that has two little prongs going up each nostril).  They said once he got that in, he relaxed big time and went right to sleep.  Becky and I have both got to hold Oakes the last two days which has been awesome!  Without the mask and ventilator tube, you can see his cute little face again!

4.       Fundraising.  As part of SLCH’s comprehensive patient and family support for organ transplants, they have a staff member who advises families on fundraising efforts.  I honestly never thought about this, but it makes sense.  I guess there have always been worries about use of funds from fundraising, so SLCH works with a couple organizations that specialize in this type of fundraising.  Essentially, they have a contact person from the organization who oversees any fundraising efforts and controls the funds.  They deposit those funds into an account specifically set up for Oakes and they establish a trustee from within the organization who controls payouts and they strictly only payout to support fees directly related to Oakes transplant and transplant recovery.  I have no idea if we’ll get around to doing this (I’m sure we’ll need help if we do this because we don’t have the cycles right now) and I’m not sure what we’d even do if we decided to try something, but we’ll keep you posted.

5.       Stable.  The good news the last few days is that Oakes is stable.  So far, in true Oakes fashion, he is impressing everyone with his recovery.  He has done better than his doctors have expected up until now.  They credit the infamous catheter procedure that occurred on June 9^th, two weeks ago today, when we almost lost him.  Apparently the ballooning of his pulmonary veins worked much better than they anticipated.  Now this doesn’t mean that those veins will stay open – history tells us that these veins almost always narrow again and need either future rounds of cath procedures to balloon them up, some stents to try to help for a time or a full lung transplant.

6.       Appendix.  It seems like months ago, but Becky has recovered nicely from her appendectomy she had in the wee morning hours of June 12^th.   She had a follow up doctor visit yesterday which went well and he confirmed things are well.  She’s a great example of toughness for the Oakes man!

7.       CICU friends.  We have made friends with a young couple in the CICU – Dave and Chrissy – who have a little girl, Elizabeth, in the CICU.  Dave and Chrissy, two months ago, had twin baby girls.  One was perfect and one had a hole in her heart and was missing a lung.  Last night, Elizabeth became the third member of St. Louis Children’s Hospital to get an artificial lung transplant.  The surgery went well and she is recovering and will soon go on the lung transplant herself.  So yes, Oakes has a girlfriend already….

8.       Thank you.  I know I’ll miss some key folks, but we are long overdue to thank these groups:

a.       The extended Fox Family.  Becky’s family – her sisters, brothers, parents, step-parents, aunts-uncles, etc. – have been an amazing help.  I cannot count how many times they have traveled from all over the country to help us for days or weeks at a time.  In fact tomorrow will mark the first day since June 8^th that we haven’t had at least one of them staying at our house while visiting from out of town!

b.      Ellen Moriarty and Cindy Fitzgerald.  These two friends established a Care Calendar TWICE to have folks sign up and bring us meals and snacks and goodies and groceries or whatever else we needed for several weeks.  This was incredibly helpful and so sweet of them and everyone who helped out.

c.       Ortyl Family.  My sister and parents have also helped whenever we have needed them.  We’re fortunate to have my parents in St. Louis to help on a moment’s notice.

d.      The SLCH staff.  They have been like family to us and have provided the best care possible for Oakesie.

e.      Everyone else.  We have read every single email and text that all of you have sent and each one is super special for us and means the world, we mean that.  We have not been good, especially after the last 2-3 updates of replying to all of your email notes so we apologize for that, but trust us, they are being read and we feel all of the love and we can assure you that Oakes is too!

 

Thank you all for your thoughts and prayers.  I thought I’d attach a picture from today as well – this was Oakes right before I got to hold him this afternoon.  It is awesome that he is awake, alert, comfortable and not yelling at his papa to take the tube out of his mouth!  We will continue to keep you posted as best we can.

 

Love,

 

Greg, Becky, Isla P. and Mighty Oakes

 

 

Mighty Oakes Update 6.16.11

All – quick update.  Oakes had one of his rougher days of late.  They weaned him all of the way off the vent to the point where the machine is only acting as a backup, but we’re still days away from extubation.  Oakes reacted a little rough to the weaning mostly because he’s more awake and less sedated than he’s been in weeks.  So he would get frustrated and try to cry and his numbers would obviously get worse.  Becky and I visited around 2:30pm and right when we walked in Oakes got really ticked off and started to struggle to breath.  Becky was petting his head and Oakes started to turn blue.  The nurse was a bit flustered which got me really flustered.  She asked the doctor for help as she couldn’t figure out what was going on with the ventilator, so while they manually pumped air into Oakes’ lungs, they realized one of the tubes had gotten knocked loose.  Once it was re-attached, all was well again.  Scary!

 

What is also causing issues is that Oakes has another bacterial infection in his trachea related to the plastic ventilator tube.  This is one of the reasons they want to get him off the oxygen asap – the risk of infection.  This is his second infection in the trachea.  They are giving him some antibiotics to help, but the infection is not helping is breathing, especially when he gets upset, but hopefully this is very short term.

 

We talked to one of the main transplant doctors, Dr. Grady, as well as Dr. Lee, Oakes’ cardiologist, and Pegi, the transplant nurse coordinator.  The entire team met this morning at 7:30am CT and decided to move forward on the process of putting Oakes on the transplant list.  This was expected.  So if all goes well, he’ll be on the list as of tomorrow.

 

We’re still in wait-and-see mode related to other surgical options before or instead of the transplant.  We just have to get Oakes off of the ventilator entirely, see how his veins and heart reacts to that and monitor everything with various tests to help tell the story of what is going on inside the little man’s body.

 

That is the latest from the STL.

 

Love,

G,B,I,O

 

Mighty Oakes Update 6.15.11

All – Oakes had another good day today. They’ve weaned him completely off his Versed and his Morphine has been lowered quite a bit. He’s much more alert and awake. The big news of the day – Becky got to hold Oakes for a couple minutes. She kind of had to talk the nurse into it, but I got some great pictures of Mama and her boy (scroll down)!

Oakes is down to a 6 on his vent, which is great. They’re weaning him off his pain and sedation meds so that he can breathe on his own as they lower his vent.

We met with a nurse coordinator on the transplant team who answered some basic questions for us. The entire team will meet at 7:30am tomorrow and formally agree that Oakes should be put on the transplant list as of this Friday.

We had a discussion with Dr. Huddleston again today and we’re simply in wait-and-see mode with Oakes. Dr. H wants to see if Oakes can get off of the vent and how his body responds to breathing on his own. Meanwhile, they’ll continue to track how his pulmonary veins are doing via echocardiograms and possibly another cath surgery. He said, again, that he has not entirely ruled out another surgery before resorting to the transplant but that it’ll take time to see how his veins are doing and how Oakes does off the vent. He still thought it was a good idea to go on the transplant list because it doesn’t mean we’re obligated to go through with the transplant at any time, but we start to accumulate time on the list. He mentioned that the catheter last week was more successful than they thought and that the results of the profusion scan yesterday were better than they expected. The EKG they did Monday showed better bloodflow from the pulmonary veins, but again, they expect that to worsen over time. It’s just not clear how much time will go by before they worsen. He said he’d be “surprised” if they didn’t worsen….

Becky has recovered nicely from her appendix surgery. She was happily popping pain pills the other night and had the best night’s sleep she’s had in decades (I’m joking….about the decades and the “popping”). But she has slept well the last couple nights and has taken it pretty easy around the house. She’s still looking cute as ever as you can see by the pics below!

Love,

G,B,I,O

Mama & Her Mighty One!

Beck talked the nurse into letting her hold Oakes for a couple minutes today. It was emotional to say the least because I know it was our highlight of the last rollercoaster week. I love how you can see Oakes staring up at his Mama. Both Mama and Oakes still looking cute!

Mighty Oakes Update 6.14.11

Oakes had the Broviac catheter put in yesterday and the surgery went very, very well.  The Broviac is a more permanent line they actually insert directly into his artery under his clavicle.  It lasts a very long time and they can draw blood, give blood or give him medicine through it.  While they were inserting the Broviac, they did an echocardiogram and Dr. Huddleston told me that it showed that the balloon angioplasty was more successful than they had thought as it appeared the pulmonary veins were passing more blood through than before.  This doesn’t change the fact that these veins will eventually get narrower again, but at least it tells us that we can do another balloon angioplasty at some point if we need to buy more time on the wait list.

 

Shockingly, Dr. Huddleston told me he hadn’t entirely ruled out a surgical fix to the stenosis before resorting to the transplant.  He very much downplayed this and I could tell he really didn’t even want to tell me because he truly hadn’t made up his mind.  It could take a few days of tests and discussion to figure this out.

 

Today, Oakes had a “profusion scan” which will tell us the percentage of pressure between the lungs.  Typical is 40-60% each lung, so they’re curious if this is the case (not likely) or if the right side is bearing 80% or more of the pressure.  We should get the results tonight or early tomorrow at latest.  This is just more information for them as they determine course of action.

 

Oakes has been weaned all the way down to 14 on his vent, which is tied for the lowest it’s been since his second heart surgery (5/28).  He is comfortable and really doing well.

 

More later.

 

Love,

 

G,B,I,O

 

 

 

Mighty Oakes Update 6.12.11 (Part 2)

Thought it’d be good, as crazy as things have been to fire off quick hit updates to immediate family since we’re getting a lot of texts and calls.  I’ll also copy the blog so that anyone who checks in on the blog can get an update every day or two.

 

Oakes is rocking it the last 18 hours or so.  Overnight, they started to wean him off the vent.  He was at 30 breaths per minute as of last night but is down to 20 breaths per minute tonight.  They aggressively weaned him today and he took each reduction very, very well.  They also stopped his blood pressure medicine today and he, again, reacted very well to this.  When I left about 5pm tonight, all of his numbers were great – perfect blood pressure and almost perfect oxygen saturation.

 

Tomorrow afternoon they’re going to insert a Broviac catheter.  This is more of a long-term PICC line.  They can use it to give him medicine, they can give him blood supplments, they can draw blood for tests and it can last several months or a year before it needs to be changed (unlike a normal PICC line or arterial line).  Also, assuming he has the lung transplant, they would do a Broviac catheter anyway. 

 

We’ve postponed our meeting with the transplant nurse so we can have another day or so to read the lung transplant book they gave us.  That’s the latest!

 

Lots of love,

 

G,B,I,O

 

 

Mighty Oakes Update 6.12.11

Friends and family, good evening.  We hope you are all happy and enjoying your families this weekend!

 

This is far and away the toughest email I’ve written and I’ve been struggling with how to even start it.  I guess first things first – Oakes is fine right now and in pretty stable condition.  But he is sicker than we or his doctors ever knew and continues to amaze us with his strength.

 

Let’s start back when Becky wrote the email below, last Wednesday.  We were in Oakes’ room at the CICU waiting for this catheter procedure.  That procedure didn’t take place Wednesday due to a mix up, but we had a consultation with Oakes’ cardiologist, Dr. Caroline Lee.  She wanted to let us know that on the echocardiogram that they had performed on Oakes the previous day, they saw a couple of things that led the staff to believe that there may be some pulmonary stenosis (for us laymen, stenosis refers to the “narrowing” of the veins) present in Oakes’ pulmonary veins that bring oxygenated blood from each lung back to his heart.  So the echo on Thursday would determine if that was indeed the case and, if so, how pronounced it was and they may intervene.  If they were to intervene, that would likely include ballooning these pulmonary veins (similar to what they had done during his May 4^th to his pulmonary “branch” arteries which deliver blood from the heart to the lungs).  The problem is, these pulmonary veins are much smaller than the “branch” arteries which makes it more difficult to balloon or to insert a stent.

 

Thursday, we arrived around 9am with Oakes’ cath surgery scheduled for around 1pm.  This was not foreign to us, since we had arrived to St. Louis Children’s Hospital several other mornings expecting a surgery that day.  Oakes had several doctors and nurses come through that morning, which was nothing unusual for a surgery day.  Dr. Murphy, who performed the 5/4 cath and who was about to perform the this cath, stopped by to say hello as well.  Oakes finally got carted out of the CICU around 2pm.  We were told it’d be a 3-4 hour procedure, so we grabbed a late lunch and just hung out in Oakes’ CICU room.

 

Around 4:30pm, Dr. Lee came in to the room to give us an update.  Unlike every other time we’ve talked to her, she suggested that we go to the consultation room of the CICU.  I didn’t really think anything of it at the time.  There was another family in the consultation room, so we sat down in a large conference room behind the main nurse station that the nurses sometimes use as a lunch room.  Dr. Lee has been Oakes’ cardiologist since October when the Truncus Arteriosus was first diagnosed.  We’ve developed a great rapport with her and we can tell she really loves the little Oakes man (she also loves my wife, but who doesn’t?).  Dr. Lee sits us down at the far end of a 20-person conference table.  She has piece of paper with a diagram of a heart on it and a pen in her hand.  When I made eye contact with her, I could tell she was uncomfortable.  “It’s not good,” she says after a deep exhale.  Becky, already knowing the gravity of the situation, asked if Oakes was still alive.  “He is, but it’s really not good,” Dr. Lee responded.  My heart sank like I’d just been punched in the throat and Becky started to cry.  This was not news we were ever, ever expecting to hear.  It just wasn’t.  Dr. Lee told us that his veins were so small, there was no hope in ballooning them or stenting them for a long term fix and that he was not reacting well to the cath.  She said she’d be back with an update.  Beck and I walked like zombies in disbelief to Oakes’ empty CICU room and just bawled uncontrollably. 

 

They had taken the catheter through an artery in his groin up to his pulmonary veins.  What they realized was that only one of the four pulmonary veins (two on the left and two on the right) were letting ANY blood back to the heart.  This was shocking to all of the doctors – they had NEVER ONCE seen pulmonary vein stenosis (again, narrowing) with Truncus Arteriosus and had not seen anything on any previous echocardiogram or cath or x-ray or blood test to tell them this was occurring.  On the echocardiogram they did on Tuesday, they had seen what they thought was some congestion on one of the left pulmonary veins.  So they took the cath directly to that vein in hopes of relieving the congestion.  When they went to balloon that vein, Oakes went into cardiac arrest.  This immediately signaled to the doctors that what they saw on the echocardiogram was not congestion, but rather was relatively normal blood flow – unfortunately, it was the ONLY blood flow coming from the lungs to the heart.  So when they essentially “blocked” that vein with the balloon, Oakes had no blood flowing to his heart.  Dr. Murphy, of course, immediately pulled the balloon out, but Oakes was reeling…..this was about the time Dr. Lee gave us the first update.

 

About 30 minutes later, Dr. Lee came back for another update.  Somehow, things had gotten worse and Oakes was in really bad shape.  He needed to be put on an ECMO machine (extracorporeal membrane oxygenation) which is essentially a life support machine.  Dr. Lee said this would buy us some time to call family to say goodbye to Oakes because things were that bad and he probably wouldn’t come off the ECMO machine.  We were just stunned and totally crushed.  Somehow Becky got up the energy to call her family and tell them to get to St. Louis as quick as they could.  I called my parents who were visiting my sister in Des Moines to tell them the news.  Within an hour, we had almost all of our parents and siblings driving in from Oklahoma City, Tulsa, Des Moines and Wilmington, NC (Becky’s sister, Christina was already here from DC).  Our families were, of course, shocked by this news since Oakes had been so stable, relatively speaking, since his emergency heart surgery on 5/28.

 

I can’t really describe exactly how we were feeling and I hope none of you ever have to experience anything remotely close to it, but to put it simply, we were just a mess. 

 

Dr. Murphy, the surgeon who performed the cath, then came by and took us into the same large conference room that Dr. Lee had us in earlier.  He explained some of the more technical details of what they found and was very empathetic.  We actually got a bit of good news from Dr. Murphy – Oakes had stabilized on his own and was not put on the ECMO machine.  This was, again, a stunning development since we had heard from Dr. Lee just 45 minutes or so earlier that he may not make it and that ECMO was the last resort to save him.  The rollercoaster continued…

 

Shortly after talking to Dr. Murphy, still spontaneously breaking down whenever we would try to think about what had been communicated to us, a team of doctors and nurses wheeled Oakes into his CICU room.  He was indeed not on the ECMO machine and it just broke our hearts to see the little man, knowing what he had just been through.  But the important part was that he was stable, but we didn’t know how long that would last.

 

Over the next few hours we talked again to Dr. Lee and to Dr. Gazit, the Director of the CICU.  The story that was forming was that the narrowing of the veins were so bad and went so deep into the lungs (from the heart) that it was irreparable.  Leave it to Oakes – not just the truncus, but also narrowing of the branch arteries; not just tracheal rings, but complete tracheal rings that went the length of his trachea; not just narrowing of the pulmonary veins but intense narrowing the entire length of the veins.  They don’t when this narrowing occurred – if he was born with it or if it developed from some infection along the way – and explained that they didn’t diagnose it until now because it was so rare and had never been seen in conjunction with truncus arteriosus.  Any issues Oakes was having were assumed to be related to the significant heart issues he had, so previous echocardiograms and other tests weren’t really focused on the pulmonary veins.

 

The other thing that was clear was the only chance Oakes had at survival was a transplant.  Not a heart transplant, but a double-lung transplant.  The heart is functioning as it should, but the severe stenosis in the pulmonary veins has put the lungs in bad shape.  The lung transplant would include a new set of pulmonary veins.  Nerd that I am, I have attached a PowerPoint with pictures to (hopefully) make things easier to understand

 

So now what?

 

Now we study up on lung transplants and hope Oakes stays stable as can be.  We’ve been told he should qualify for a transplant in spite of his heart issues – since they’ve all been repaired and it is functioning well, it’s not held against him.  We were given a 67-page packet Saturday that tells us everything we’d ever want to know about pediatric lung transplants.  Once we review that, we’ll get a chance to sit down with the transplant nurse so she can answer questions and ask us some herself.  Additionally, they’ll be running some blood tests on Oakes to ensure that all of his other organs are properly functioning and that there is nothing which would disqualify him from a transplant.

 

Here is the reality of what we’re trying to absorb – around 50% of pediatric lung transplant recipients do not make it to five years past surgery.  That number dips to around 30% ten years out.  And the staff has been really, really clear with us – the transplant is NOT a cure, it is a treatment.  A lung transplant and the complications afterward are much more difficult than even a heart transplant, which boggles our minds.

 

Miraculously, we live in St. Louis and Oakes is in St. Louis Children’s Hospital.  Would you believe that St. Louis Children’s Hospital has performed more than 350 lung and heart/lung transplants since 1990 making it the most active pediatric lung transplant program in the world?  And would you believe that Dr. Huddleston, Oakes’ cardiothoracic surgeon, has probably performed more pediatric lung transplants than anyone in the world?  I figured you’d believe that, but it’s still miraculous to us.

 

If all goes well, we can have Oakes on the transplant list by Friday.  The average wait to find donor lungs is about 2-3 months for a baby Oakes’ age (they’re much more in supply than for a teenager with cystic fibrosis, for instance).  It’s critical that Oakes stays stable throughout this entire process.  If he regresses significantly and needs to go on ECMO, he will be disqualified for a transplant.

 

As you can imagine, our brains are not absorbing this painful information completely yet.  We’re doing our best to stay strong for little Isla P., but I’d be lying to you if I said it was easy.  The rush of emotions that come over me and Becky when we’re snuggling with Isla or reading her books at night are too intense to describe considering what is going on with Oakes and what may or may not happen with him.  We’re just trying to take it one day at a time, as hard as that is right now.

 

Wondering why I am sending this out at 5:30am Sunday morning?  As “unluck” would have it, Becky experienced some intense pain this evening after dinner and I won’t bore you with the infamous “details” here, but we’re sitting in Missouri Baptist Hospital and she’s about to have her appendix removed.  No, I’m not kidding.  She will be here until Monday morning some time.  We’re waiting for Ashton Kutcher to jump out from behind a curtain or something.  I’ll try to keep you posted on her surgery too (no news on that should be considered good news).

 

I know we are in your thoughts and prayers, so please keep us there for a long time.  If you’re inclined to be an organ donor, go for it..…little Oakes wouldn’t have any shot at all without it.  I’m the first one who needs to change the checked box on my driver’s license.  I know we’ve heard it all before, but it’s put into unique perspective at times like these – just appreciate every single day with your family.

 

We love you all.

 

Greg, Becky, Isla P. and Mighty Oakes

www.babyortyl.blogspot.com

 

 

Our Weekend.

Visiting our little Oakes in the hospital.

Still just as cute as can be!

Ena and Isla making cookies!

Aunt Sally and Isla are navy stripped twins! (Isla calls Sally "Die-yos", too cute!)

Ena with her little buddy!

Aunt Sally bought Isla a little water pump that fills balloons, so we are combining some of our favorite things, balloons and the pool! Thanks so much Sally!

Rolling the water balloons down the slide...

clapping when they splash...."Yay!"

Girlfriend kisses!!