Moved to step-down unit

Riley was just moved to 7 North, the ICU step-down unit. Nurse-patient ratio is more like 4-to-1 than 1-to-1. Fortunately we are in a nice two-bed room with a beautiful view of the skyline. We've been in 7 North three other times and were always in the noisy four-bed room so this is a happy development. The nurse practitioner is currently removing one of Riley's chest tubes. He'll keep the Tenkoff drain, the left pigtail drain and the other two chest tubes until they run dry.

Grampy was with Riley overnight and he did well. At one point he became agitated because he wanted juice but they were keeping his fluid intake low. He turned grey and that escalated his lactate levels, but those came back down when he settled down. He has some congestion in his chest and you can hear it when he breathes. But the x-ray looks fine and he just needs to clear it out. The nurse showed us how to tap on his back and chest area to help him free it up - he just needs a good cough.

Riley's been sleeping most of the morning since we arrived. He had a little morphine and was also given something before they pulled his chest tube. When he starts coming around he'll be able to eat and drink as much as he can handle which should improve his spirits. Doctors will be keeping an eye out for chylothorax now that's he's had some fats. This is the leakage of lymphatic fluid due to a tear in the thoracic duct, usually from surgery (or as the surgeons say, a byproduct of "dissection"). Riley had this after the Glenn, requiring him to be on a fat-free diet for six weeks until the duct healed. In 2004, Riley was still nursing, and that made things very difficult. Hopefully it will be easier this time around now that Riley has much more variety in his diet.

Suzanne and I got to sleep at home last night for the first time in several days, and I even got out this morning for a refreshing bike ride. Riley's birthday is on Sunday and we're going to have a small celebration.

Sunnier outlook

Today was a good day. Riley was extubated and is drinking juice, an arterial line came out and several medications were stopped (including dopamine). He is clearly doing better. His blood and common atrial pressures are normal. His BUN and creatinine levels have dropped. Tenkoff and chest tube drainage has slowed considerably. They were going to remove his chest tubes this afternoon but decided to wait until tomorrow since there was a bit of drainage from one (no point removing two today and sedating him again tomorrow to remove the other). What a difference a day makes.

Riley's doctors also gave us some much better news. Riley's cardiologist and the intensivist/cardiologist both feel he is making great improvements and surgery isn't going to be necessary. But we couldn't feel relief until we'd spoken to Riley's surgeon. He was in the OR all day and finally came by at 5:30pm. He said Riley is doing great and he doesn't think any surgery will be necessary. When asked if fenestration would or would not be an option with Riley's anatomy he basically said "it doesn't matter, he won't need it."

So what caused the Fontan to fail in those first harried 24 hours? The vote is 2-to-1 for arrhythmia. The surgeon and intensivist are pretty convinced. Dr. Karl, the surgeon, said "there is no doubt." The arrhythmia is common with asplenic patients and it was enough to push him over in the critical post-operative period. But what if it happens again? It shouldn't be a big problem in the real world. Arrhythmia can be managed with medication and won't have such dramatic effects when Riley is healthier. I liken it to pneumonia in a 20-year-old healthy person versus an 80-year old lung cancer patient. Riley will have follow-up EKGs shortly after we leave the hospital to monitor the condition, and we won't be allowed to leave until he's stable. We'll see the cardiologist regularly but at some point, he will only want to see Riley once a year (!).

What about that one vote against arrhythmia? That was from Riley's cardiologist who believes we won't ever really know what caused the meltdown on Friday. He saw some evidence on Friday that it wasn't arrhythmia but I didn't catch the technical description he gave to the other doctors. In any case, he thinks we're on the path to recovery and should be home from the hospital in another 2-3 weeks (fingers crossed). Riley's drainage will continue for at least another week.

It's hard to relax and feel like we're out of the woods, we certainly aren't. All of the regular surgical risks still apply - infection (especially for asplenic patients), drainage problems, chylothorax, clotting, irregular rhythms, etc. And the outlook might change again tomorrow. But for now we're feeling better and continuing to hope for a smooth recovery. Grampy is on night duty and Mom and Dad are sleeping at home for the first time in many days.

Dysfunctional family meeting

Riley is extabated and going okay. They've taken him off the dopamine and lipids and are no longer doing the albumin replacement. He is getting some FFP. His BUN and creatinine numbers have come down slightly and his blood gases look good. His blood pressure is much higher today and more stable.

I relieved Suzanne at 5am and she went to the apartment to sleep. When my Dad and Barbara got here at 9am I headed downstairs to get something to eat. When I came back to the room at 10:15 the cardiologist and intensivist were in Riley's room. After chatting for a few minutes I began to get the impression that they considered this the formal family meeting. Unfortunately, nobody bothered to tell the family and Suzanne was not there. Neither was the surgeon. We told the social worker that his presence was critical. Both doctors had positive outlooks and felt Riley's condition had improved, but we didn't have the substantive dialog I'd been hoping for, primarily because we didn't have a quorum and I hadn't fully prepared.

The primary question still is - what went wrong and why did the Fontan fail? The intensivist thinks it was arrhythmia from the trauma of the procedure and that it was a temporary setback. The cardiologist disagrees and says we don't know what went wrong and we may never know. The surgeon didn't express an opinion because, again, he wasn't there. He's the only one with the first hand experience from the OR. An hour after I left I checked my voicemail and there was a message from the social worker - "it's 10 am and the cardiologist and intensivist are in your room so it would be great if you and Suzanne could come up. Oh and the surgeon is too busy today and tomorrow so I figured you could talk to him separately." Not exactly what we'd requested. I was thinking scheduled meeting, people actually showing up, conference room, that type of thing. And nobody from the UC cardiology team who performed the cath was present (the cardiologist who came was Riley's regular Kaiser cardiologist).

Suzanne just arrived and we're going to spend some time with Riley and then we'll go talk to the social worker. Clearly this doesn't count as our family meeting. Oh, and I missed rounds this morning because they closed the door to Riley's room while they chatted outside lest I overhear something I don't understand about another patient I don't know. They didn't bother to reopen the door when they started talking about Riley so I don't know what was discussed.

Consultations

Riley's Tenkoff output is lower today, and he seems better to us. They were going to extabate him earlier but decided to wait because there's no air leakage around his airtube. This may be an indication of swelling in his throat which could cause problems if his airtube is removed. Given Riley's up-and-down fluid levels over the past few days that's to be expected. Docs gave him some steroids to reduce the swelling (if there is any) and they'll wait to extabate him tomorrow. There's no reason to rush since he doesn't seem to be bothered by it.

The project manager in me kicked in today and I've begun whipping this place into shape. As Suzanne mentioned in our last post we're calling a family meeting with each of the key players. Scheduling is still TBD but it will probably be tomorrow afternoon. I also talked on the phone today with two outside cardiologists associated with "Another Top Regional Medical Institution" that shall remain nameless but happens to have a tree for a mascot. We knew one of them through pediatric cardiac support groups and the other is a friend-of-a-friend. Both were extremely helpful and generous with their time. Here are some of the things we learned:

• The biggest question right now, and the one we need to ask tomorrow is: why did the Fontan fail - what went wrong? It's essential that the team get the answer to this question and come to agreement because some causes (like bypass lung injuries or rhythm problems) are reversable and others (like fundamental anatomical problems) are not.



• A cath will definitively answer the fenestration question. The last cath showed the surgeon that fenestration won't help because the pressures in the heart are too high (in other words, even if they put a hole in, blood wouldn't flow into the heart). But the last cath was done when Riley was in severe distress and just before he was put on the ECMO. Things might have changed. My consultants suspect that disagreements between the staff members might be due to whether or not people think things might have changed.



• Fenestration is a reasonable option, and is later closed in a majority of cases. If the fenestration can't be closed later, that means it was the right decision. Think of fenestration as being between a Glenn and a Fontan.



• Fontan takedown isn't a death sentence. Estimates that 2% of Fontans require takedown, but obviously for very complicated cases like Riley's it is probably higher. It's an extreme setback, and serious, but we should have hope. Some kids do better with time and it can go on for some time. They will put a special shunt in to keep Riley's O2 sats above the Glenn level. However, nobody will go back and try a Fontan again unless they know for sure why it failed before and are convinced it won't fail again.



• Asplenia is not a contraindication to heart transplantation. Fontan patients are definitely at higher risk, but there is nothing about asplenia that will disqualify Riley from a transplant, should we reach that stage (either soon or in the distant future).



• Second opinions probably won't help much. There's not a huge difference in how institutions approach this problem and the logistics are a nightmare - i.e. we'd need to get another surgeon up to UCSF to see Riley. What we're doing - talking to people and getting informal opinions - is a great approach.

Anyway, the family resource room is about to close so I'm going to end this. If anybody would like to help financially, please donate enough money to UCSF to allow them to expand their paltry family resource room hours. Ugh.

More uncertainty, mixed opinions

Still not exactly sure what's going on with Riley's condition. After rounds this morning, Dr. Tyndall (one of the cardiologists) came in to give us a very bleak outlook--Riley's not improving overall, fluid from drains is up, kidney functions not improving. He contradicted what Ian (one of the intensivists) told us yesterday that we could continue along this path for as long as needed, as long as there was no deterioration. Tyndall painted a picture that there are increased risks as we wait in this holding pattern with so many interventions and drugs, and that a decision would need to be made in the next day or two as to what the course of action would be--probably a Fenestration.

We told him that Dr. Karl (the surgeon) said he did not think Fenestration was possible with Riley's anatomy, and that the only option was the takedown. Tyndall said that he had not talked with Karl (argh!). After we talked with Dr. Karl again for clarification, he bascially said the pressure in Riley's heart is such that creating a hole between the atriums would not do anything. Rather, the blood flow would just ignore the hole and continue to the lungs. If the time comes when a surgical decision needs to be made, we would head back to the cath lab to see if conditions have changed that would make Fenestration an option. Dr. Karl doubted it would become an option. Still, Karl thinked that Riley looked good, regardless of what the numbers were showing. After that, Ian came in and told us how he thinks Riley is improving. I could only laugh because of my level of frustration after hearing three contracting opinions in the previous 15 minutes.

We are in the process of working with the social worker to set up a family meeting. This will bring several parties into one room where they will be forced to contradict each other in person and hopefully come up with a consensus on outlook and the path we are going to take. We are also reaching out to other doctors and cardiologists that we have met through Camp Taylor and other friends to see how we go about getting a second opinion. If it comes to a Fontan takedown, we want to be sure that there aren't other options being pursued at other top hosptials.

No cath, for now

It's hard to give an update because we're still very much in the dark. Here's what we know. After a fretful 2 hours of waiting while we were kicked out of the ICU for another procedure, the tests are in. That oh-so-important BUN test that everyone was waiting on came back and there was no change. Bad news right? But the doctors said "oh, we don't care so much about that, everything else still looks good." Apparently because the BUN and creatinine levels did not get worse we're not heading to the cath lab at this point. Aarghh. They're going to reduce his volume intake again (to 25% of Tenkoff output) and wait and see how he handles that. His Tenkoff drainage has also declined over the past three hours. If his blood pressure stays constant amidst the lower volume, that's a positive sign.

It's been a frustrating day for all of us psychologically. One doctor will tell us something is a sign of things going downhill and another will come along five minutes later to say he doesn't care about it. The holy trinity of caretakers - the intensivists, the CT surgeons and the cardiologists - rarely agree and frequently contradict one another. If one thing's clear, it's that nobody really knows what will happen and nobody can make a prediction. If Riley doesn't continue to show signs of improvement we're heading to the OR for either a fenestration or a complete takedown. And there's another point of disagreement. The cardiologists (including Riley's regular cardiologist) and the intensivists keep mentioning fenestration as a possible option should things go south. That's obviously preferable to a takedown. But the surgeon continues to maintain that fenestration is not an option for Riley due to his anatomy. We asked for clarification and they paged Dr. Karl who was on his way somewhere and promised to explain it to us tomorrow. We'll try to get it sorted out.

To summarize where things stand: they are keeping a close eye on Riley's oxygen saturation, blood pressure, fluid output, kidney/liver functions and rhythms. We want to see continued improvement across the board. If any of these metrics take a turn for the worse, it's probable that Riley will return to surgery for either a complete Fontan takedown or a fenestration (although the latter might not be an option as I've explained).

Riley is in and out of consciousness and not being sedated at this time. He has opened his eyes on occasion and is moving his arms and legs. They have removed one of his arterial lines and are about to change out the second to accommodate a larger input. The ICU attending is hoping to remove the respirator tomorrow.

We're still trying to take it hour-by-hour and day-by-day.

Key kidney test at 1pm Pacific

On rounds this morning the doctors felt that Riley's condition has generally improved over two days ago but that it's still too early to know if he'll avoid additional surgery and/or a Fontan takedown. His Tenkoff remains high. They've now reduced his albumen replacement to 50% of the Tenkoff volume and given him a dosage of lasix. At 1pm Pacific they'll send an electrolyte test to the lab and look closely at values that indicate proper kidney function, specifically the BUN (blood urea nitrogen) and creatinine. Currently his BUN is high, at 64. With the reduced volumes and diaretics his BUN should go down, indicating that his kidneys are doing their job. If it stays where it is, it's another hint that his lower body isn't responding well to the new Fontan pressures. At that point, he'll likely head into the cath lab where they'll look to see if fenestration is an option. If it's not, we're back to Fontan takedown as the primary course of action. (Recall that Riley's surgeon is skeptical that fenestration will work for Riley's anatomy, and he reiterated that this morning.) The cardiologist assured us that everything isn't "hinging" on this test, but it's clearly important and we're keeping our fingers crossed. Best outcome would be a reduction in Tankoff and lowered BUN. If that happens, they'll probably stay the course and give it more time.

Riley has been in and out of light consciousness as they intentionally reduce his comatose state. He has been nodding his head yes (wanted a book) and shaking his head no (no ouchies) although his eyes haven't fully opened.

No change overnight

Hello to all you early risers and East Coasters. Riley had a stable night with no change. His BP has been pretty constant but there has been no reduction in his Tenkoff drainage. He is still averaging ~100cc of output an hour from that drain in his lower torso, or over 1500ml a day. The plan earlier in the evening had been to start to bring him out of the coma a bit to raise his blood pressure, allowing the doctors to reduce his albumin intake, thereby (hopefully) reducing his Tenkoff. That plan was scrapped at about 11pm when his BPs went down again so the intensivists decided to keep him on the current albumin plan (he gets albumin at 75% of the previous hour's Tenkoff volume). The Tenkoff volumes are an indication that his lower circulation system is having a hard time adjusting to the new Fontan pressures. Fenestration is one way to deal with that until his body is able to handle it. We're not sure if that means fenestration is back on the table until we talk to the cardiologists today. We know that fenestration isn't a solution if his ventricle can't handle the Fontan, but it may be a possible course of action if it looks like his heart is recovering but his lower body circulation system needs relief.

Doctors weren't too concerned about the lower pressures during that period since they come up quickly when he is stimulated and when his liver is pressed on. Right now he is in a good range. His bed linens were changed and he got another bath. Dad has had night duty, Mom is at a friend's apartment nearby sleeping.

Status quo

It's been 6.5 hours since Riley came off the ECMO and nothing much has changed. "So far so good" seems to be the common refrain. His blood gases look good. Blood pressure has ebbed and flowed and just now was quite low (62/48 or so). They are giving him more albumin to increase his volumes and hopefully bump up the BP. The output from his Tenkoff drain has also increased substantially since he came off the ECMO. We were told by the cardiologist on Friday that high Tenkoff drainage is an indication that we'll need to do the Fontan takedown, but we haven't had a chance to speak to her this afternoon to see if that concern still applies given stability elsewhere. His nurse took an opportunity to wash his face, re-dress his chest incision and brush his teeth and overall he looks much better, even if his condition hasn't improved. The ECMO machine was also removed from the room.

ECMO turned off, cannulas removed

Riley was taken off the ECMO machine around 10:25 am, and is currently stable. The cannulas are out. They cannot leave them in his neck for too long of a period without functioning because clotting becomes a concern. We've been told that the next 24 to 48 to 72 hours are critical for him to show that he can adapt to the flow of the Fontan. He remains on the pacemaker which is keeping his heart rate at 124 bpm, and they will continue to monitor his blood pressure, liver function, urine out-take, blood gases, etc., as signs to whether he is "turning a corner." Doctors say that the success rate is about 50/50 at this point. If he starts to trend downwards, they will schedule the Fontan take-down. He will not be put back on the ECMO, except as a means to stabilize him while they schedule surgery.

Thanks again to our blood donors. Riley received one unit during surgery. But since then, he has received at least three transfusions, as well as several batches of platelets.