Scheduling a cath and surgery

Riley had a pretty good day. He is in much better spirits and returning to his normal self. We made two visits to the playroom today. During the second visit we got to meet with six officers from the SFPD who were terrific with Riley. The playroom was closing down when we arrived and all of the other kids had left so Riley got special attention from SF's finest. Riley got to hold an officer's badge and was showered with gifts including a squishy SF police car, a football and an SFPD sticker. Dad hopes they remember how special Riley is in the unlikely event that a certain red Prius is ever pulled over for traffic violations on the streets of San Francisco.

A few updates on Riley first:

1. Riley is getting IVIG (intravenous immunoglobulin) to elevate his platelet counts. IVIG can sometimes cause an adverse reaction so the docs like to give it with Benadryl and Tylenol. Unfortunately Riley doesn't like Tylenol and threw a fit when he saw the syringe. He went into such a frenzy that he started gagging and caused himself to vomit all over his bed before he even received the medicine.
2. His potassium levels are now back to normal and he's again receiving aldactone (a diuretic that helps protect potassium).
3. His albumin is down slightly, but they're not giving him replacements just yet.

The doctors tell us they now have all the information they need to determine a course of action. We're still waiting on the second stool sample and the precise MRI valve flow measurements, but they aren't going to change anything.

1. The valve is back to "modest to moderate" leakiness where it has been since the Fontan last year. When we came into the hospital on March 3rd Riley's valve was leaking worse than it ever has. We had some conflicting information about this before. This problem appears to have been due to the effusions, Riley's viral condition and the elevated blood volume. As a result, Dr. Tarnoff does not want to touch the valve surgically. He describes it as a "crapshoot" since there is a strong chance the valve leakage will be unchanged or even worsened as a result. Fixing or replacing the valve also requires the heart-lung bypass, something we can avoid with fenestration (see below).
2. Ultrasound has detected a slight narrowing in Riley's inferior vena cava (IVC) where it meets the Fontan conduit. Stenting it might not make a difference to Riley's condition or outlook, but it is easy to do, has minimal risk and will prevent any long term issues that might arise. This can be done in the cath lab.
3. Some of the other cardiologists continue to discuss fenestration in the cath lab. On this issue Dr. Tarnoff is unwavering - he prefers a surgical fenestration. He feels that the cath lab would be riskier because the type of stent used will require that Riley take anticoagulant drugs "for as long as he has the stent." The drug of choice is coumadin. Coumadin is greatly affected by diet (specifically Vitamin K levels) and requires weekly blood tests. If too much is given, the blood could become too thin leading to the risk of bleeding. If not enough is given, clotting of the fenestration stent is possible. With a surgical fenestration, Riley would only need to be on coumadin for 4-6 weeks and then he'd be back on baby aspirin.
4. Surgical fenestration is considered to be "not a difficult" procedure and one that can be performed off of heart-lung bypass. Any surgery requiring the opening of the chest cavity is risky, particularly with kids like Riley that have already had several heart surgeries (they're called "re-dos"). Their chest cavities are a mass of scar tissue and great care is given to making sure to avoid collateral damage, like the tearing of vessels stuck to the underside of the rib cage. During the Fontan we were told it might take up to 3 hours just to open Riley's chest. That said, avoiding bypass significantly reduces the surgical risk and improves the recovery outlook.
5. We can expect to go home about a week after surgery. We should avoid the post-surgical complications of 2006 where Riley's chest tubes drained and drained for weeks on end.
   
6. We also learned that the literature has shown a strong correlation between PLE and the eventual need for a heart transplant. We've always known a transplant may need to be considered at some point in the future when Riley's single ventricle wears out (we've been told "one to two to three decades"). The cardiologist who told us about that study didn't know what time period was investigated and whether or not this correlation changes the outlook (if it the study was performed over a year or a decade). Still, it reminds us that the long-term outlook for Riley's single ventricle is cloudy.

So the course of action now appears to be - a heart catheterization to stent the narrow IVC followed by fenestration surgery with Dr. Karl. The only question remaining is one of timing. We should learn more in the next couple of days. The cardiologists are fairly certain we could have the surgery next week but the cath lab schedule is more uncertain. We're not sure if they'll proceed with the surgery and follow up with a cath later or if they'll wait for the cath to happen first.

Chances are if things won't happen next week we'll get to go home and then return for the procedures. Riley appears to be a few days away from being able to come home based on the effusions. Our preference is to get everything done as soon as possible, but if surgery and the cath can't happen for several weeks we'd obviously prefer not to sit around a hospital room and wait.