Second opinions

Two things I forgot to mention in my last post:

1. Riley's records have been sent for second opinion to UCLA, Children's Hospital Boston, Children's Hospital of Philadelphia (CHOP) and Stanford. It takes some time for them to respond and they will either respond directly to Dr. Tarnoff or to us. In any case, things are in motion.
2. Dr. Karl is back at UCSF and operating. We spared blog readers the details of this sordid affair, but Dr. Karl had come off of Riley's case and stopped operating due to "political reasons." It seemed like he was leaving UCSF for good. Looks like this has been resolved and Dr. Karl is Riley's surgeon again.

Good news all around

The Cardiologist

Riley had an x-ray, echo and check-up with the cardiologist yesterday. On Wednesday we took him in for numerous blood tests.

1. Riley's blood panel looks very good - his electrolytes are normal and his albumin is normal (actually high-normal).
   
2. His chest is completely clear and his hemodynamics look good.
3. His INR (clotting) is hovering between 2.2 and 2.8. We have been doing the home finger stick test every 3-4 days with mixed results. The machine is finicky. We call in his INR result and the coumadin clinic gives us a dosage schedule for the next few days.

The biggest news from the cardiologist was a surprise: Riley's common valve regurgitation is the lowest it's ever been. Dr. Tarnoff now classifies it as "very mildly leaky." Historically it's been "moderately leaky" and it was "severely leaky" when Riley was at his worst in March. Tarnoff is as stumped as we are - this was completely unexpected. If anything, one would expect valve regurgitation to be better with the Fontan circulation than with the Glenn. One possible explanation is that the reduction in lymphatic vessel flows (due to the elimination of the effusions) is responsible. In any case, it's great news.

It's too early to declare victory over PLE but all signs point to the positive - no effusions and his albumin levels are great. When we see Dr. Tarnoff again in 2 months we'll have a stool sample and since it will have been 90 days we can confirm that the PLE is "cured." A Fontan takedown cures PLE like amputation cures a blister.

There is no change for now to Riley's medication schedule and low-fat diet. Before the next visit in 2 months Riley will have a sonogram to look at the clot in his leg. We can expect to go off the coumadin and back onto baby aspirin at that time. We can also expect to stop the low-fat diet.

Make-A-Wish

The Make-A-Wish team visited the house last night. When we spoke with them on the phone, they asked what Riley liked. We told them he likes baseball and cars. When they arrived, they handed Riley a new baseball and bat and four tickets to the Giants-Yankees game on June 24th! They were obviously off to a great start.

Riley had fun acting goofy, playing baseball and generally enjoying being the center of attention. Suzanne and I filled out paperwork while one of the volunteers interviewed Riley in the family room. The forms prepare for all possibilities - everything from driver license numbers and insurance information (in case they rent us a car) to available travel dates and media waivers. Riley talked at length about how he'd like to see baseball games, meet baseball players (he specifically mentioned Barry Bonds, Omar Vizquel and Jason Schmidt) and visit famous ballparks like Wrigley, Fenway and Yankee Stadium. We're not exactly sure what kind of wish they will put together but you can be certain it will have something to do with baseball. We should hear from the wish coordinator in a few weeks with specifics.

Family Camp

Finally, Riley and I spent this past weekend at the Camp Taylor Family Camp in Sanger, California. This is a gathering for kids with heart disease and their families. Carter had come down with a fever on Thursday so Mom and Carter stayed home. We stayed in a tiny cottage and had a blast - playing baseball, singing camp songs and all sorts of games.

Check out the pictures.

Updates from the cardiologist

Riley had a follow-up appointment with his cardiologist last Wednesday; he will have another appointment in two weeks. Here are the updates:

1. When we go back in two weeks, Riley will have the following tests: x-ray, echocardiogram, and a blood draw to check his electrolytes, globulins, albumen, hematocrit, and INR (to make sure the finger stick machine is accurate).
   
2. We hope the PLE will fade away now that Riley is back with the Glenn circulation. The albumen test should be an early indicator of whether or not it is gone or going away.
   
3. Riley will continue on the appetite stimulant until his regular pediatrician decides it's time to stop. Dr. Tarnoff estimated that would be in about a month. Riley says he's hungry quite frequently. However, when he actually gets the food in front of him, he often only eats a couple of bites.
   
4. Riley's oxygen saturation was in the mid-70s. When it dips into the 60s, it will be time for the fistula surgery. That is the primary determining factor for the timing of the surgery. Apparently the fistula and the surgery for the aortic pulmonary shunt are two different surgeries. The aortic pulmonary shunt will connect his aorta and his pulmonary artery with a gortex shunt. Both of these surgeries will likely make his already leaky valve more leaky. "With every gain, there is a loss," said Tarnoff. If the situation with Dr. Karl at UCSF is not resolved by the time Riley needs surgery, Dr. Tarnoff said that another surgeon could perform the surgery. Also, the fistula is not open heart surgery. The surgeon will go through Riley's ribs on his side. For the aortic pulmonary shunt, the surgeon will go through his back.
   
5. In one or two months, Riley will have an ultrasound on his leg to look at the clot. We would expect to find that either the clot is resolved or the clot will be calcified and no longer at risk for breaking off.
   
6. We have been taking Riley to his pediatrician's office for the finger sticks to check his INR (clotting factors). We tried at home the first day and I did not have a large enough sample to get a reading.
   
7. We have heard that Stanford often rejects kids with complex defects at their transplant center. Stanford is obviously the most convenient center for us since we live only 20 minutes away. If Stanford does not accept him, UCLA will likely be where we end up. Dr. Tarnoff said that they "accept almost everything." The downside of being accepted at UCLA is that you need to be within 30 minutes of your transplant center at all times. Then after the transplant (if an organ is found), you need to live near your transplant center for at least a year. We have an informal meeting with someone from the Stanford transplant center later this week.

Finally, Riley was able to celebrate his birthday over the weekend. He invited two friends over for pizza and non-fat ice cream cake from Baskin-Robbins. It was not a cake with a matchbox car on top, like we saw at the store many months ago. It was a Bob the Builder cake, and Riley seemed perfectly happy with that. He is also doing a great job walking. He's not fast or agile, but he's getting around. We still mostly carry him up and down stairs, he is tired a lot, and he is sleeping 13+ hours a night, but we are all home for now and that is a wonderful thing.

We need your help

The Sacramento support group that we have been a part of is hosting a blood drive in honor of Riley this weekend. Since we all can't be at this wonderful event, please donate blood this week in your area. Afterwards, please post here to let us know you were able to donate. That way we can tally up how many pints were donated to people in need as a result of these efforts.

If you are in the Sacramento area and would like to be a part of the official Blood Drive and Lunch in honor of Riley, please comment with your email address, so that I can let you know who to contact.

UPDATE: If you have already donated for Riley during this hospitalization (and I know there are a bunch of you), please post here so that we can count your contribution.

Thank you!
Suzanne & Ken

Home!

We're home! Riley's INR was at 1.8 this morning, giving us the greenlight to go home. They pulled his PICC line and then we basically sat around for five hours so they could monitor him.

On Tuesday we'll go see Dr. Tarnoff and Riley will have blood drawn for INR. Tomorrow we'll also be receiving an INR testing kit with finger prick so we can begin the home testing on Wednesday. Riley came home on lisinopril, cyproheptadine, amoxicillin, lasix, aldactone, coumadin and ferrous sulfate (iron supplement).

Finally, we delayed Riley's birthday while we were in the hospital and are celebrating everything a month late. Today is my birthday (was 3/22) and we're celebrating Riley's birthday next Saturday 4/29.

Home Monday at the earliest

Just a quick update to say that the earliest we'll be getting out of the hospital is Monday. It all depends on this wacky coumadin. We got the first result back Friday morning and the numbers were off. That means they need to keep tweaking the dose until they get the levels right. I don't totally understand it, but I do know that it is a huge pain in the arse.

Tube is out (again)

They pulled the new chest tube this morning, the x-ray showed that the pneumothorax was gone and Riley's lung has inflated. No fluid actually drained from the tube, just air.

Riley is going to go home on coumadin to treat the clot in his leg. He'll be on the medication for about 90 days. Managing the medication will require a daily blood test. Now that all tubes are out, they're going to discontinue the heparin and start the coumadin overnight (don't ask me why it needs to be done at night). It will take 48 hours for the coumadin to take effect and then they'll test his PTT levels. If they're good, we can go home. If they're not, they'll tweak the coumadin and wait another 24 hours. We'll get some sort of home blood testing machine so we can do the blood tests with a finger stick instead of having to go to the clinic. As you might imagine, we're worried about how Riley will tolerate a daily finger prick.

So Saturday is the earliest we could go home. Nobody goes home on Sunday (in our experience) so if the first 48 hour PTT level isn't good, we're looking at Monday. We were on track to go home today but unfortunately the dislodged chest tube and the pneumothorax set us back.

Pneumothorax

Good times don't last long. The x-ray from this morning showed that Riley has a pneumothorax in his left chest (air pocket causing a collapsed lung). Apparently air seeped in when the chest tube fell out yesterday. He's being moved back to the PICU now to be sedated so a new chest tube can be placed. We're not sure how long he'll need to be back over there, but obviously our hopes for going home in the next couple of days have been dashed for now.

Chest tubes are out

The tubes are out. Riley did great - Child Life kept him occupied with a Cars book and the dilaudid did its magic. He's very groggy and sleepy right now, when he wakes up he should be a lot more comfortable. Next steps: figuring out the anti-coag/blood thinner strategy and getting him learning to walk again.

Chest tubes might come out tomorrow

Here are a few quick updates:

1. Riley is in very good spirits but his eating is still erratic. He ate well for two days but the last two days his intake has been low. Mom had a good conversation with him today and explained that he won't go home until he starts eating. He had a little bit of pizza for dinner, but Carnation Instant Breakfast is our friend.
2. At the playroom this morning one of Riley's chest tubes fell out. Since the stitches came with it, they just taped over it.
3. They're planning to pull the other two chest tubes tomorrow. Keep your fingers crossed that drainage continues to be low overnight. They're so confident the doctors have ordered the heparin to be discontinued at 5am. Yippee. If the tubes come out tomorrow we're hopefully on track to go home later this week.
   
4. Riley's PTT (clotting time) continues to fluctuate. A few hours ago it was low so they increased the heparin a bit. At other times it's too high so they stop the heparin for a while.
   
5. Besides transitioning Riley to his home medicine regime, the big open question continues to be how to deal with anti-coagulation and the clot in his leg. Obviously he can't stay on IV heparin. On Friday we heard he'd only be on aspirin but yesterday we were told Riley will need a medication that requires us to give him shots twice a day for several months. Uncool. Apparently they are still consulting with a clotting expert in Canada who supposedly can be reached at 1-800-NO-CLOT (I kid you not). Actually, that's not enough digits, perhaps it's 1-800-NO-CLOTT (the last T is for "Terrific!").
6. At some point during the past week we officially passed 2006's hospitalization record. We've now been in the hospital for 44 days.

Finally, Riley has been approved by the Make-A-Wish Foundation! Riley's cardiologist has already sent them the required medical forms and they'll be sending volunteers to meet with Riley in the next few weeks (probably once we are home). It's pretty exciting and we're already encouraging Riley to start imagining what he'll wish for.