Mistake at the lab

There was a mistake with Riley's Alpha-1 Antitrypsin test. Somewhere along the way, the results were read incorrectly and the old results were mistakenly reported instead of the new results. And the new results are much, much better!

His new result was 12. A normal result would produce a test results of less than 55. Riley's previous result--from a few days before he was admitted to UCSF in March--was 160, and that was the number that was erroneously reported to us about two weeks ago.

Dr. Tarnoff told me that we are probably out of the woods for PLE, but we will retest in about six months to confirm that things are holding steady.

PLE at bay, not gone

We got the results from Riley's recent stool sample test that we hoped would confirm that the PLE (protein-losing enteropathy) was gone. But it is not. The Alpha-1 Antitrypsin test found elevated levels of protein in Riley's stool. A normal result would produce a test results of less than 55. Riley's result was 160. That is the same number he had from the same test just days before he was taken to the ER, and then admitted to UCSF for his eventual Fontan-reversal.

Dr. Tarnoff said that he was disappointed that we haven't seen any improvements, but we shouldn't read too much into it at this point. He said that it may take many more months to see improvements. On the flip side, we may never see improvements. However, Riley is doing well from a clinical perspective, which is good news and he does not have any physical symptoms of PLE (the puffiness, poor profusion, constant upset stomach, diarrhea). Here are some highlights from my conversation with Dr. Tarnoff:

1. Obviously, we were all hoping to see his numbers start to head back towards a normal range. It may take many months. It may never reach a normal range. He seems to have an "exceedingly mild" form of PLE, and we'll keep an eye on albumen levels and as long as there is no significant difference in albumen, and he continues to thrive from a clinical perspective, then we do nothing. You can have a disease and be borderline normal. Right now, Riley's PLE is so minor that it isn't having any affect on his organ systems.
   
   
2. Since there isn't much known about PLE or why some kids get it, it's not clear what could set it off again. The last two times Riley got a cold (Sept. 06; March 07), we ended up at UCSF. That means it could happen again and the effusions could come back and the puffiness could come back. And with cold season just about to start, we are feeling nervous. If we start to see any puffiness, or if he starts to have the diarrhea and frequent upset stomach, then we need to get in touch with Tarnoff right away.
   
   
3. As for what's next, he will have more tests to check his albumen, globulins, and Alpha-1 Antitrypsin again in six months. If albumen and globulins start to drop, then we could start what Dr. Tarnoff refers to as "voodoo medicine," none of which has much scientific evidence of doing anything.

This is all very discouraging. Yes, it's true that Riley has a good amount of energy and he seems to be feeling better with fewer upset stomachs and less diarrhea, but we were hoping that his Fontan-takedown would cure PLE. That is what we had heard up until after the surgery. Then it was revealed to us that sometimes PLE doesn't go away after the child is reverted to the Glenn circulation. So we are feeling very anxious and worried that this miserable disease might come back and take us back to the hospital. There is just so little known about it, that no one can really tell us what we want to hear: that Riley is going to be hospital-free for a long, long time.

Back from our Make-A-Wish trip

We're back in the Bay Area after our trip to Baltimore, Washington D.C. and Philadelphia. We'll have a complete Wish wrap-up with lots of photos soon, but in the meantime we wanted to whet your appetite with these.

Wish update: Riley in the newspaper!

The Make-A-Wish team delivered Riley's wish this week! A reporter and photographer from the San Mateo Daily Journal were there to document it all - check out the article.

We're overwhelmed by the itinerary - in addition to watching the Orioles game, we'll be getting the royal treatment from MAW and the Orioles organization. Riley will get a special front-row seat for batting practice, a tour of the stadium and an opportunity to meet some players. We'll visit a sports history museum, the Babe Ruth Birthplace Museum and take a harbor tour. The wish volunteers brought t-shirts, a special suitcase for Riley filled with goodies for the trip and a delicious cake shaped like a baseball stadium. Our little guy enjoyed being the center of attention and got sillier and sillier as the evening went by. He's really looking forward to the trip.

We had an echocardiogram and a check-in with the cardiologist earlier in the day. There is no sign of the PLE and his chest is completely clear. When we see Dr. Tarnoff again in a few months we can officially drive the final nail into the PLE coffin, assuming everything still looks good. We've been concerned of late because Riley's mid-morning tummy aches had returned. Continuing to suspect his medication, Dr. Tarnoff suggested we try giving Tums at medicine time.

Riley's valve is still "only mildly leaky." His O2 sats were 82%. We're also off the coumadin and back on aspirin. That means no more finger prick blood tests and the flaky machine that goes with them (the test probably failed one-third of the time, requiring a second stick).

We also got a more detailed explanation about the Glenn circulation, and why we should expect Riley's saturations to eventually decline and require more interventions. A newborn's heart receives approximately 66% of its blood from the upper half of the body and only 33% from the lower half. For an adult, that ratio is almost exactly flipped (babies are pretty much all head). Since Riley's superior vena cava routes blood to the lungs but the inferior vena cava returns blue blood, the overall saturations will decline as Riley grows and the upper/lower circulation balance shifts toward the lower body. Dr. Tarnoff indicated that we'd be looking for saturations in the 60s before we move toward more interventions (recall that we can try some additional fistulas and shunts to boost his oxygen level before proceeding toward transplant).

Wish granted

The Make-A-Wish team has granted Riley his baseball wish. They are sending all four of us to Baltimore to see the Oriels play at Camden Yards. While we are there for our wish, we will also go see the Philadelphia Phillies play at Citizen's Bank Park and the Washington Nationals play at RFK Stadium (and then he'll be the ring-bearer in his Auntie's wedding).

We also went to see the Giants play the Yankees this past Sunday. It was the first part of Riley's all-baseball wish.

Other than that, Riley seems to be doing great. He has so much energy--in small bursts. He rarely stands still. You know the saying about ants in the pants, well, that really would be an accurate description of him lately.

He'll be back to preschool starting July 9, and I think that will be good for all of us. He really misses schools and he needs more interaction and activities that I can manage now. He really is ready. And so am I.

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.