Celebration details...

Riley's dad created a website with the details for celebrating Riley's life. Find it here.

Celebration of Riley

We are planning a Celebration of Riley's life on Saturday, Nov. 1 in San Carlos, CA. Details will follow, but just a few things to consider:

1. If you have a picture of your child with Riley or a picture of Riley from a school event, please find it to bring with you, as it will be taped into a memory book. 
2. We are hoping students will be willing to share feelings/stories about Riley, so please help/encourage your children prepare/practice if they are interested in doing so.
3. Children/friends/siblings/parents will have an opportunity to walk a lap in honor of Riley. For each person who participates, Riley's family will donate $5 to Camp Taylor.

Thank you.

UPDATE: You can find information and directions to the event at the Celebration of Riley's Life website.

Eleven and a half

Despite the very special team of doctors and nurses helping him, Riley died yesterday afternoon, Monday, October 20, 2014. I am heartbroken for my family and I am heartbroken for all of the people, especially the children, who know Riley. I wish I could scoop them up and protect this from this horrible truth.

Hopeful with siblings before surgery.

Riley went into surgery scared, but hopeful. He looked forward to running, flying on airplanes without oxygen, and going to the mountains--something he could not do because of his low oxygenation. I like believing that he died still feeling hopeful for those things, and I imagine him running and running and running. I feel grateful that he died while being soothed by his mother, father, and two bonus parents and not in an operating room. He is no longer in pain, no longer suffering, no longer struggling. For this I am also grateful. Afterward, I noticed a vertical beam of light pressed against the wall near the closed blinds at the end of his bed. He is free.

We know you all want to do something and we appreciate your love and support. In lieu of flowers or other gifts, please consider donating in Riley's honor to Camp Taylor, which is a free summer camp for children with heart defects and their families. Also, please donate blood, not just now, but again and again. Riley lived as long as he did because of the generous donations of countless, nameless, and selfless individuals. His corneas will be donated to help others.

At some point, there will be a celebration of Riley’s life, and we will post details here. In the meantime, I wanted to share a poem that Riley wrote last month for a class assignment:

Gracious Green
By: Riley Norton 9-16-14 

Do you know what my favorite color is? Green! 
He is: nature; earth’s greatest gift, 
the leaves that sometimes drift, 
an Exit sign glowing on the wall, 
and a brand new tennis ball 

My color sounds like the wet grass when you step in it 
He tastes like a salad with some vinaigrette 

My color feels like a warm, soft pillow 
Green is the smell of a tree called a willow 

Green looks like a sour apple ripping on a tree
he is someone's favorite color and that someone is me! 
IS YOUR COLOR GREEN TOO??? 

Thank you for loving him, and us, through the years.
Suzanne, Ken, and family

UPDATE: Donate to Camp Taylor in Riley's memory here: http://www.firstgiving.com/fundraiser/rileynorton/donate

Waiting...

I wish I could say that there was a clear path on which Riley was moving, but there is not. He continues to be critically ill on life support. It is difficult for us to even comprehend the size of the mountain that he needs to climb. And so we wait. And wait. And wait.

To read what Riley's mom is thinking about, go here.

Riley still critically ill

Riley continues to be on life support. He is still critically ill. Please continue to hold him in your hearts.

Downward spiral

Riley's condition has spiraled downward and I cannot even comprehend, yet alone begin to explain, how horrible things are. He has lost circulation in his legs, they are performing an extremely risky procedure on them now. That is on top of the clusterfuck of the ECMO circuit. He may lose his legs, he may bleed out. To say that everything, EVERYTHING has gone wrong is an understatement. He is gravely, gravely ill. There is nothing else to say. So this is my last post for the foreseeable future.

That said, thank you for all of your love and concern and support.

Out of surgery

Riley just came up from the operating room--man, it has been a long day. We are waiting for the nurses to get him settled in his room so that we can go see him. Dr. Hanley said that everything went well and after explaining what to expect, I was surprised to learn that he will have four catheters instead of two like last time. There will be two in the left femoral artery, one in the right femoral artery, and one in the jugular.

Basically, ECMO is an external heart-lung bypass machine. All of Riley's blood will leave his body, go to the machine to be oxygenated, and then pumped back into his body. From a scientific standpoint, it's fascinating to actually see blue blood and red blood so vividly. From a parental standpoint, it leans more toward the freaky end of the spectrum. But then again, in all honesty, this whole experience falls on the freaky end of the spectrum.

A doctor from a renal team came to explain that they are attaching the dialysis machine to the EMCO circuit and will slowly remove toxins and fluid. We should see improvements in his swelling in the next 24 to 48 hours.

The next three to five days should just be peaceful (fingers crossed) while his body attempts to heal. With less erratic blood pressures and fluid overloading and irregular heart rate, etc., perhaps it will be a little more peaceful for family as well. We now just need to put all of our energry into believing that it will work and his organs will begin to recover.

No blockage found, heading to the OR

The cath lab didn’t find any blockage, which is good news. However, he is in such dire straits with organ failure that he needs a break. He is going to be put on an external heart-lung bypass machine called ECMO (extracorporeal membrane oxygenation*). For those of you familiar with Riley’s history, he went on ECMO after his Fontan failed in 2006. The procedure happens in the operating room and it will be performed by Dr. Hanley, the same surgeon who did his 1 1/2 ventricle repair last week.

Our hope is that taking complete control of his heart, lungs, and kidneys (he can receive dialysis while he’s on ECMO), his body can begin to recover. Fingers crossed. Our guys needs a break.

*For all of my fellow MFA graduates, you may recall reading about ECMO during workshops or hearing about it during my senior reading at USF in 2011.

Multi organ failure, back to cath lab

Riley’s blood pressure sometimes dips into the high 50s and low 60s, although sometimes stretches into the 70s. There is grave concern about his status. He is having kidney and liver failure because there is not enough oxygen getting to his organs. His platelet count is dangerously low.

Dogs at work. Work, dogs, work.

* They are concerned that he is having heparin-induced thrombocytopenia (HIT), a reaction to the blood thinner heparin. If he does have HIT, the blood thinning medication can have the opposite effect and cause deep vein clots. He is about to have an all-over ultrasound to look for clots.

* As a precautionary measure, they are starting him on an antifungal medication, in addition to the antibiotics he's already getting, just incase an infection is causing the platelet problem.

* He is going to have a cardiac catheterization today to look at one of the connections done during his surgery to make sure that it isn’t blocked. It’s called the baffle connection, which is the connection from the ventricle to the aorta. 

* While they have the catheter in his groin, they are going to start dialysis. It is going to happened exceedingly slowly--possibly over a 24-hour period--because if it happens too quickly it will mess with his already low blood pressure.

And, of course, I'm discouraged by the fact that Riley hasn't roused at all during that last 36 hours despite having removed all of the sedatives. The doctors said that's because his organs cannot metabolize the medicine, therefore it can take days before it works it's way out. Still, I would like to see him open his eyes a bit to let me know he's still in there. So scared...

Blood pressure up, medicine reduced

Snuggling with Freddie & Freddie Jr. (left)

The bulk of the fluid from those pesky pockets drained within the first 20 minutes, although they have trickled as the hours march on. His blood pressure has risen into the 90s while the doses of epinephrine and vasopressin have been reduced. That seems like slightly good news to me. But doctors are not optimistic yet. There have been no signs of improvement in his kidney or liver function. And one of his markers--creatinine, which measures kidney function--is actually worse. Still, I have to believe that removing nearly two liters of fluid from crushing his lungs and other organs has to be an improvement, even if we haven't seen it on any test yet.

Poorly perfused feet :(

Reducing the vasopressin will also help return a bit of circulation to his toes, which have been dark blue to near black in color. I will continue to ever-so-gently plead with him to try and jumpstart those kidneys. We met with the renal team earlier today and while there are no plans to put him on dialysis, it is still a possibility if things don't improve in the next day or two. There is also talk of possibly introducing diuretics tomorrow, but there is the possibility that they can negatively impact his blood pressure. Even though he is so swollen, his vessels may be somewhat dry and adding a diuretic can lower blood pressure. Seems like we're in for a long game of trial and error.

(This blog will provide Riley's medical updates. To read about his mother's state of mind, go here.)

Pigtails placed and draining

Just a quick note to say that more than one liter of fluid poured from the stomach drain and 600 mililiteres came from the lung drain in the first 20 minutes. That means that there is a little more wiggle room to keep giving him IV nutrition. They had to halt nutrition because he was so fluid over-loaded. He needs nutrition for his organs to heal as well.

Critically ill

Guard dogs

Overnight, medications kept Riley’s blood pressure in the mid-80s, however, as soon as they turned the vasopressin down, his blood pressure followed. He received another batch of platelets and FFP (fresh frozen plasma), which has boosted it a bit this morning.

He will be getting two pigtail drains this morning. The platelets will help him clot properly from the procedures. One drain will go in his stomach to drain fluid there and one will go in his lung. His pleural space on the left side of his chest is filling up. The hope is that removing some of the fluid will help his organs start to recover.

Both his kidneys and his liver function are declining. And he is so swollen. He is about 3.5 liters positive in fluid. That means that 3.5 liters more fluid has gone in than come out. As you can imagine, the body’s ability to stretch to accommodate the additional fluid looks incredibly painful. He is swollen all over, but his left arm is especially swollen around the elbow and after doctors examined it, they discovered that its blue and mottled and looks infected. They’ll be watching it.

The renal team is going to be called in today to start watching Riley, so that if they need to do dialysis in the coming days, they are already familiar with his case.

Also, Riley’s hair is falling out.

(This blog will provide Riley's medical updates. To read about his mother's state of mind, go here.)

No pigtail drain yet

Doctors are holding off on putting in a pigtail drain to get rid of the fluid around Riley's stomach for now because the risks outweigh the benefits. There is concern that it will cause too much bleeding becasue his clotting factors are off. They will continue watching the fluid and if his stomach becomes hard or the pool of fluid becomes too large things may change, but for now, they are holding off.

With the help of another dose of FFP (fresh frozen plasma) and an increase in his epinephrine medicine, we are seeing his blood pressure crawl into the high 70s and low 80s. Unfortunately those are only short-term boosts. But we need to keep doing them. The hope is that his cardiac output will increase soon and organs will wake up. In the meantime, he will continue to get medical assistance.

The nice pink color we saw right after surgery has diminished with his low cardiac output and his extremeties are mottled and blue. Basically all of his energy is working to keep his vital organs as healthy as possible. In the meantime, he remains in deep sedation (a drug-induced coma) to help his body rest. We can't thank you enough for all of your love and support.

(This blog will provide Riley's medical updates. To read about his mother's state of mind, go here.)

No improvements

The milrinone was stopped last night. It is a medicine that helps the heart relax and fill properly. The idea was that removing it would help his blood pressure increase into a more healthy range. It worked for a bit, but his blood pressure fell into the high 60s/ low 70s again this morning.

Also, the damage to his kidneys and liver has not shown any improvements. While it's good that they are not getting worse, the residual impact of a build-up of a variety of toxins does make him in overall worse shape. Doctors are considering putting him on dialysis for a bit to remove some of the toxins from his blood. He was also low on calcium, so he's been given some of that.

Riley is also positive more than two liters of fluid. For most people when you drink two liters of fluid most of that comes out as urine, sweat, etc., but for him, very little is coming out. The result is that he's quite swollen. We're seeing it in his face, eyelids, feet, hands, and belly, although it's really everywhere. An ultrasound of his stomach showed that a bunch of fluid is collecting around his stomach and it's estimated that he has 100-200 mls of fluid in there. A pigtail drain will likely be put in to drain that fluid. There is concern that the build up of fluid there will prevent his organs from getting enough blood and oxygen flow. The mindset is that if there is anything visible that they can improve, they are going to do it since his body is struggling so much.

The blood pressure was the main topic of discussion at rounds. Even though he is so swollen with fluid, they need to give him more to boost his blood pressure. Given his Glenn circulation, they need to make sure that he can properly perfuse his brain and low blood pressure can affect how much blood and oxygen gets in there. This is of critical importance.

He will continue to be fully sedated, paralyzed, and on the ventilator for the foreseeable future.

Next 48 hours critical

Riley with Freddie & Freddie Jr.

To rule out any blockage in his ventricles, an echo was ordered. It showed that Riley’s ventricles are both pumping well, his valves are not leaking, and there isn’t any fluid around the heart making it difficult for it to pump. That’s all good news. The bad news is that his ventricles are still not pumping with enough oomph. As a result, his blood pressure, despite all the different types of medicines, blood products, and volume they are pushing into his body, hasn't moved into a safe range yet. It is important for his blood pressure to rise because he needs to be able to properly perfuse his brain and other vital organs.

During the echo, Riley woke up a bit—the first time I’ve seen him move any of his parts aside from his eyelids—and his whole body twitched in agony. Because of the ventilator, he couldn’t vocalize his pain, but it was so painfully obvious. We asked him a series of yes and no questions to identify where it was hurting the most. Where he was being pressed on by the echo technician hurt (no surprise there), his back hurt, and his stomach hurt. Tears dripped from the corners of his eyes. I told them that he needed a break until he had more pain medicine on board. He writhed around for several minutes before the fentanyl warmed through him and settled his battered body. Once he was settled, one of the doctors helped me feel a little better about the episode--we know Riley is still in there. He was able to answer a series of questions and for that we are so grateful.

He is about to be given a muscle relaxer (basically paralyzing him) so that his body can completely relax and not have to do any unnecessary work. “We’re going to let him relax and see where we end up,” his nurse said. Basically, they keep boosting the doses of medicine designed to boost blood pressure, but they aren’t really having any impact on raising blood pressure. The doctor explained that it becomes a point of diminishing returns.

His kidneys and liver which had begun to show signs of improving have just begun to take a turn for the worse. It's not critical yet, things are just going in the opposite direction of what we want to see. Also, his lungs are starting to sound wet. That is always a concern because one possible side effect of being on a ventilator is pneumonia. Ugh. They’ll be watching and listening closely. 

No improvements, stiff ventricle

Riley with Burton Belt (temporary name)

Despite getting another dose of Vasopressin, Riley’s blood pressure is still hovering in the upper 60s and low 70s. He is getting some FFP (fresh frozen plasma) now and like albumen, blood products that are protein-rich are supposed to draw fluid from his tissue into his vascular space and then into his kidneys in addition to raising blood pressure. It seems that the doses of this and that are short-lived. And unfortunately, Riley’s kidneys and liver suffered some damage during surgery and aren’t working too great just yet. He is have low urine output and is still quite puffy.

Also, doctors speculate that part of the reason his blood pressure is so low is because his ventricle is stiff from surgery, and as a result, it just isn’t working that well right now. It’s sort of like when you twist your ankle and it swells up and isn’t flexible. Same idea with the ventricle, it’s stiff from being so manhandled. As a result, he is expected to be on the ventilator until mid-week. His recovery is just going to take longer than expected because he did have a long and extremely complex operation.

He is answering questions by nodding slightly. Just now the nurse asked, “Is this position comfortable?” To which Riley shook his head ever so gently back and forth. She is now trying to help find something that makes him more comfortable.

Struggling with blood pressure

Gell well soon Riley!

For the last 24 hours Riley has been struggling with low blood pressure. They gave saline and packed red blood cells and that worked temporarily. They gave albumen which was supposed to boost his blood pressure with more lasting results, but that was also temporary. And because saline and albumen are both fluid, he has gotten swollen and all the extra fluid on board was affecting the pressure in his Glenn circuit and in another part of his circulation (I cannot remember the name), which is bad. Just before heading home last night, he was given a dose of Vasopressin. It worked to keep his blood pressure near normal levels for several hours, but now it’s wearing off and his blood pressure is back down into the high 60s/ low 70s. His blood pressure and how to treat it will be discussed during rounds that will begin in the unit at 9 am.

He started opening his eyes last night a bit and became a little communicative overnight responding to conversation with some nodding when his pressure was up. But because he still has some medicine on board that makes him largely paralyzed, he cannot move much. The nodding is a big step. I suspect he is also quite terrified to try and move anything anyway, even if he could for fear of pain. I remember that from previous surgeries that we could tell when he was really uncomfortable because he would become exceedingly still. 

When I spoke to the cardiologist last night she said, "Even though he's making progress, he is still a long way from being stable." That is becoming more and more clear unfortunately.

Blood pressure drops, gets albumen

Riley has struggled with his blood pressure all day and it continues to be lower than what doctors would like to see. Earlier today he received several boluses of saline to boost volume, which in turn boosts pressure. But with all the extra fluid in his blood and because his drainage tubes continue to have output, he’s also received packed red blood cells so that his blood isn’t too thin. But his pressure is still low, so he just received some albumen, which will hopefully stabilize his blood pressure as it "sticks in the veins" a bit better than saline. Another dose is being cued up right now.

Team Riley's Captain still sedated

A lot of what we see triggers unpleasant memories from previous hospitalizations. His blood pressure was exceedingly low about 24 hours after his Fontan. Soon after, he crashed and ended up on the extra heart-lung bypass machine called ECMO.

Since surgery yesterday, he has been kept paralyzed and sedated so that the does not move and disrupt any of the lines or IVs. However, in an attempt to help him be a little more comfortable, some of the medicine to keep him paralyzed is being weaned so that he can twitch and move just a bit like we do when we are asleep. The first muscles to wake up are usually the lips or the tongue. We haven't seen any movement just yet.

He received a dose of aspirin to try to prevent blood clots. In other news, we haven’t seen the arrhythmia in a few hours. Riley’s temperature is near normal now at 37.5 degrees, although nurses would like it to be closer to 36 degrees. 

Fever, arrhythmia, and other regular post-op stuff

Lots of medicine

First of all, Riley looks pink and we can see freckles on his nose that we’ve never noticed before because of his coloring. That is cool. His toes are pink, his fingernail beds are pink. His torso, arms and legs are a creamy color and not a reddish color. His lips—that are mainly occluded with tape from the ventilator—are pink. It’s crazy awesome.

There are some things we are worried about only because we have such a traumatic history with him post-operatively. He has a fever. It is 38.7 degree C now; it was 39 degrees C when I went home after midnight; the nurse said it should be 35 or 36 degrees. At least it’s moving in the right direction. Fingers crossed. He’s on top of a “cool pad” to help bring it down.

He's pink!

He is having a bit of an abnormal heart rhythm called an arrhythmia. The nurse said it’s been going on since early this morning and they are called PACs (premature atrial contractions). We are leery of them because after his Fontan, Riley had arrhythmias that became life threatening.

His left arm is also somewhat swollen. He has a history with thrombosis or clotting after his fourth or fifth surgery and we’ll be keeping a close eye on it and mentioning it to the doctors when they appear for “rounds” to discuss Riley’s case.

Other stuff: he was on by-pass for 378 minutes and should be completely ventilated until tomorrow at the earliest. They typically keep the kids on the vent for 24-48 hours and Riley should be closer to 48 just because he came into the unit so late at night. 

All in all, given the length and complexity of his surgery, he's doing really well.

The good siblings

Hattie, Riley, Ben & Carter in pre-op waiting room

Riley had quite the entourage this morning before surgery. In addition to mom and dad, he had stepmom, stepdad, Grampy, and his three siblings too! We all got up at 5 am and held off eating in solidarity with Riley. But once surgery was delayed due to a possible transplant coming into the center, the three siblings were able to have breakfast. The two stepsiblings headed off to school once Riley went in, but not before we managed to score a picture of them looking somewhat sensible (a rarity!)...

The sleeping sibling

Riley's brother Carter hung with us all day at the hospital and tolerated boredom and stressed out parents very well. After getting an update that things went well for Riley (and being somewhat confused by all the tears), he promptly fell asleep. He was a total rock star brother. Love that boy! Well done kiddos! We know that this has been hard on all of you too.

"Things went well..."

After what feels like weeks of sitting outside the CVICU, Dr. Hanley appeared in front of us. Riley is still in surgery as doctors are finishing up, but the five heart parts are all done. "Things went well," he said, "Things went really well." Due to all of the scar tissue from previous surgeries, it took a long time for them to get access to his heart cavity. Even so, Hanley said that he went to work around 11:30 this morning, which means he's been working on Riley's heart for more than 10 hours. He will be coming up to the CVICU in about 45 minutes and we'll get to see him about 30 minutes after that. Amen!

No news...

It's almost been 12 hours and I wish we had something to report. Honestly, at this point, no news is not feeling like good news. Quite the contrary. Keep sending positive thoughts our way. We need all the encouragement and positive energy the universe can offer us. Thanks.

"Things are going well"

We just heard that "things are going well," but doctors don't have an estimate for when they'll be done. At this point, he's been in surgery for more than seven hours and since they didn't have an estimate, we're guessing that they are not close to finishing up. It's probably going to be a few more hours. At least I've managed a few naps on the sofa in the waiting area outside the CVICU (cardiovascular intensive care unit) where Riley will end up after his surgery.

A pre-op view

R distracted with SF Giants highlights

Just a picture of Riley sporting his hospital-issue, Mickey Mouse pajamas before going back for surgery this morning. Nothing to report yet, just waiting, and waiting, and then wandering the hospital before waiting some more. Fingers crossed that no news is good news at this point.

Full speed ahead

The transplant that delayed us this morning fell apart and the team moved quickly to get Riley ready for his surgery. Once he said goodbye to his siblings, bonus parents, and Grampy from the registration waiting area, he went into the pre-op suite where he shedded his civilian clothing for Mickey Mouse scrubs, plain blue hospital pants, and grippy yellow socks.

Numbing medication went onto his hands to prepare IV sites. He also received a large dose of Versed to help relieve some of his anxiety and despite it's miserable taste, he managed to keep it down with some distraction. One of the team members overhead that Riley likes the Giants and promptly handed over his iPhone so that he could watch some Giants' highlights. That relaxed him almost instantly...and I'm sure the Versed was already kicking in as well.

From there Ken and I gave him some hugs and the team rolled him out of there to get prepped for surgery. That was around 9:30 am. Dr. Hanley would go into the operating room a few hours later. The whole procedure should last less than nine hours, but we should get periodic updates from the team. We had the opportunity to meet briefly with Dr. Hanley just outside the family waiting room. He went over the complex procedure and talked a bit about chylothorax, which is something Riley has suffered from in the past. The takeaway is that he may get it, but it's unlikley. The biggest risk is that he comes out with a permanent pacemaker.

Surgery delayed

An emergency transplant has delayed Riley's surgery. At this point we are simply waiting to see if it is delayed for a few hours or if it will be rescheduled for another day.