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.