We spoke with both Drs. Karl and Tarnoff this morning. The takeaway: surgery is being scheduled, ideally to immediately follow the cath on the same anesthetic. The O/R is more flexible than the cath lab as Dr. Karl can always add Riley to the end of his day, the cath lab is going to see if they can take Riley sooner than Friday.
Some specific notes:
- The narrowing in Riley's veins is not in the IVC but in the hepatic veins that all connect to the Fontan conduit (Dr. Karl reattached Riley's hepatic veins last year). The stent to be used can also be stretched at a later date in the cath lab as Riley grows.
- UCSF stopped doing routine fenestrations with Fontans in 1994 when they switched to extracardiac Fontans.
- Dr. Karl might be able to perform the fenestration without bypass. If not, he can still do it without cross-clamping (stopping the heart). This is important because cross-clamping can lead to arrhythmia like we experienced last year. It will be about a 2-3 hour operation.
- The surgeon will leave a snare so the fenestration can be narrowed or closed at a later date without reopening the chest. Dr. Tarnoff told us we could try closing it in 1-2 years and if it's tolerated, close it permanently.
- Dr. Karl has performed 4 or 5 late Fontan fenestrations at UCSF. One eventually required Fontan takedown. Riley would be the latest after Fontan he's ever seen, usually fenestrations soon after surgery when kids crash (like Riley did in 2006).
- Both doctors feel much more comfortable with surgical fenestrations. Cath fenestrations are still in their infancy, only 3 have been performed at UCSF and one failed, requiring surgery. Surgery gives the doctors a greater degree of precision over the stent size - you can't tell exactly how big the stent is in the lab, you need to use x-rays and some guesswork. It is too soon to know about the long term prospects for cath fenestrations.
- Dr. Karl would like to operate as soon as possible, he believes there is no reason to let Riley sit around in the hospital and run the risk of developing an infection or another virus. Since he is in good health, the time to do it is now.
- After the fenestration, Riley would have his albumin and immunoglobulins tested by the pediatrician. If PLE is gone or remains in a "manageable" state, we'll leave it (meaning Riley wouldn't need protein replacement or would only need it every few months). If PLE is still significant, two options remain: Fontan takedown and heart transplant.
- Riley will need to be on a course of Coumadin (warfarin) for 4-6 weeks. Coumadin therapy requires weekly blood tests to calibrate the correct dosage based on dietary variables. It's possible to do these tests at home with a finger stick kit, but we have heard from other parents that these are less than reliable. In any case, Riley would need to have blood taken on a weekly basis until the Coumadin is replaced by aspirin.
9 comments:
Wow, it seems like they've cleared up a lot. I hope they can move the cath up so he can get his surgery sooner.
Thinking down the line: if the PLE persists, would they try any kind of non-surgical management of the PLE using steroids or heparin, before proceeding with the Fontan takedown? I guess it comes down to whether the Glenn circulation without drugs is preferable to the Fontan circulation with drugs. But it would seem worthwhile to at least attempt steroids or heparin before the takedown.
But we're praying it doesn't come to that. I'm hopeful and confident that the fenestration will do the trick.
Thanks again for the updates (thanks to Google too, for the wireless! :-))
WOW! some action finally. Guess my trip is well timed after all. Was beginning to have doubts about that. See you all tomorrow night. Loveya, Grammy and Wayne
I am glad to hear that they have a plan and that Riley's Grandma will get to be with him. Our thoughts and prayers remain with you for an efficient process and successful outcome. Hang in there!
It must be a relief to finally have a plan. I'm praying that they can do the surgery as soon as possible and that Riley has the smoothest recovery yet. I know its extremely difficult for you to be in the hospital with Riley and try to be at home with Carter too. It must be exhausting. Hopefully now that there is a surgical plan you can see the light at the end of tunnel. We're thinking of you guys.
Just wanted to give some feedback regarding Mike's question about the use of steroids to manage PLE. Mostly, Dr. Tarnoff wants to avoid steroids at all costs because steroids tend to lower the recipient's immune system. And since Riley's already has a weakened immune system, we don't want to go there. Also, in most cases, the patient is never able to be completely weaned off the steroids. I guess when they try to wean off, the symptoms of PLE return.
At last some solid plans for action.
Praying for good health so procedures can move ahead!
Praying for all.
Thanks for clarifying that, Ken. We're all hoping and praying that such questions will be rendered moot by a successful fenestration and subsequent elimination of PLE.
Hi guys,
I know it's not ideal to be heading back to the O.R. but I'm really relieved to hear that the fenestration won't be done in the cath lab. It sounds like things are moving in the direction they need to be going. I know it's difficult but hopefully it means Riley will be back home as soon as possible. Also, ask about whether Riley can go home with his PICC line so that he doesn't have to go through all the excruciating blood draws while they get his Coumadin levels stable.
Again, please know you are in our minds constantly and call if you need ANYTHING. Thank you so much for keeping us informed and remember to take care of yourselves, too!!
love,
Gwynne
I am hoping that everything goes well. Jilly will be at UCSF April 3rd for her cath. Although it would be very nice to see you all, I am hoping that the recovery time at the hospital is minimal and Riley is home celebrating his birthday with his family. Our thoughts continue to be with Riley.
Dina and Russ
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