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.