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.

A light at the end of the tunnel

Riley continues to improve slowly. Here are some updates:

1. The stitches were removed from his incision.
   
2. His IV medications are being transitioned to be oral medications. The main thing doctors are watching is to make sure that he does not get effusions as they make the switch. IV medications can work differently than oral meds and may not be as effective.
   
3. Riley is eating and is hungry a lot.
   
4. Dr. Tarnoff said Riley will go home with baby aspirin as a blood thinner, not coumadin.
   

We hope that his drainage will continue to slow and that the chest tubes will be removed in the next few days. After that, we should be close to coming home.

Moved to step-down unit

After a disheartening low point late last week when Riley looked terrible with sunken eyes, poor profusion and low stats, he seems to have made some improvements. We moved to the step-down unit earlier today and Riley is in good spirits.

Here are some updates from the past couple of days:

1. His intracardiac line was removed.
   
2. His pacer wires were removed.
   
3. His arterial line from his underarm was removed.
   
4. He is getting an appetite stimulant to increase his interest in food. He is probably taking in 700 to 800 calories per day.
   
5. His drainage has been steady. It is also clearly chylous. The doctors had loosened up his fat restrictions for a couple of days to get him more interested in food. The drainage almost instantly turned to a thick, creamy consistency. He is now back on a fat-restricted diet.
   
6. His PICC line was not drawing blood (a clot had likely formed on the end of it), so some TPA was dripped into the PICC line to dissolve the clot.
   
7. His stats seems to be in the mid 70s. They do occasionally dip into the 60s or rise into the 80s, but 70s seem the most frequent.
   

Finally, we have decided to have Riley's records sent to some other institutions. We're currently dealing with some political issues in the hospital that have resulted in sudden and unexpected changes to Riley's team of doctors. We'll share more information once we get a better understanding of what's happening. We've also begun to question the decisions that have been made for Riley's care that led to three unnecessary open-heart operations--operations that may ultimately make him unable to have a heart transplant. We have read online about a child who was not eligible for heart transplant because of too much scar tissue and another that had too many antibodies in his systems to allow a match to be found. Now that we are on the other side of the Fontan take-down, and more surgery and the need for a transplant is not that far away, we have been questioning the choices that Riley's doctors have made and would like to consult with other experts in the field.

Leg clot resolving itself

A hematologist came in to talk with us about Riley's blood clot and swollen leg yesterday and it was much less swollen than it had been the previous three days. She said that the clot was likely being reabsorbed into his body. There is still a very small chance that the clot could break free and travel to his heart or brain. Therefore, he will need to continue on heparin therapy for at least a month. She said if Riley goes home before a month is up, he will need to get heparin shots once or twice a day to ensure that his blood continues to move freely and not clot so easily.

I also heard from one of the nurse practitioners that Dr. Karl is leaning towards aspirin therapy instead of coumadin as a long-term blood thinner for Riley. This would be wonderful because coumadin comes with a variety of problems. The biggest is that it is affected by how much Vitamin K you eat. As a result, users need to have weekly or biweekly blood tests so that the dose can be adjusted based on what you have eaten.

A decision regarding Riley's appetite has not been made. We are working very hard to get him to eat. Most of his calories are coming from high-calorie liquids like Enlive juice. It looks like a juice box, but each box has 300 calories and 10 grams of protein. Yesterday, he took in about 800 calories thanks to two of those juices mixed with Gatorade. He is not really interested in solid food at all, although he did eat quite a few bites of a mozzarella sandwich (thanks Helen!). Doctors are loosening up his fat restriction in an effort to get more nutrition in him without invasive measures.

We have also started talking with doctors about transplants. We don't want any surprises when we get to that place, so we are asking lots of questions now. Initial conversations indicate that Riley could be a candidate and that his asplenia does not preclude him from an organ (contrary to information we got when Riley was a baby). However, if two kids about the same size with the same blood type were both waiting for a heart, the organ will always go to the other kid because of the asplenia. The organ goes to the recipient with the best chance of giving the organ the longest life. His risk of infection is much greater because he doesn't have a spleen. As a result, we've long been told that he was more likely to die from an infection before his heart wears out and he needs a transplant. Well, it seems his Fontan reversal changes those odds.

Dr. Tarnoff stopped in this morning and Jack had an opportunity to talk with him. He said that within a year, Riley will likely need some kind of surgery. Presumably it would be done to bump up his oxygenation, but I'm not sure. When I have more details on what kind of surgery, I'll include an update here. Tarnoff seemed to think it made sense for us to start talking about a transplant. He estimated that Riley would need one within the next couple of years.

Finally, Riley continues to get albumen replacements and is also getting an intravenous immunoglobulin (IVIG) infusion.

Blood clot and other depressing updates

Riley's right leg has been extremely swollen for the past couple of days. An ultrasound found a blood clot in his leg in the femoral vein--the same vein that was used during his past two cardiac catheterizations. He is already on a therapeutic dose of heparin (a blood thinner), so some of the doctors are talking about putting him on coumadin, an anticoagulant. Anyway, here are some other updates:

1. Riley has been extremely tired. He has been sleeping most of the past two days and then also a good amount overnight as well. I was wondering if he has been extra tired because his oxygen saturation is so low post surgery--down in the low- to mid-70s, instead of in the mid- to high-90s prior to reversing his Fontan. No one seems concerned. They seem to think he's just recovering from "being run over by a truck twice."
   
   
2. Reversing his Fontan and reverting him back to the Glenn circulation may not eliminate the protein-losing enteropathy (PLE). We thought that putting him back to the Glenn was a guarantee to get rid of PLE. However, that's not true. We are hoping that it will do the trick, but there have been another cases where PLE did not go away. We will know in a couple of months. Hopefully if it does not go away, we will be able to treat it medically with heparin injections or occasional albumen infusions.
   
   
3. There is a lot of concern regarding Riley's nutrition. He has been taking less than 300 calories a day for the past couple of weeks (probably the whole month we've been here). We have started keeping a log of all food/drinks to get an actual number on calories. He may need to get IV nutrition. This is not necessarily a good thing. Apparently, IV nutrition can cause liver damage. Another option would be an NG tube--a tube threaded up his nose and down his throat into his stomach. Then high-calorie fluid would be pumped into this stomach. Neither of those options sound very appealing. His fluid allotment was bumped up to 1500 milliliters per day in an effort to get more calories in. However, more fluid in may lead to an increase in chest tube output.
   
   
4. His chest tubes are still draining enough fluid to mess with the albumen and electrolyte levels in his blood. He is now getting 25 percent albumen replacements.
   
   
5. The reason Dr. Karl did not put in the shunt during the Fontan reversal was because the extra blood flow through his heart would strain his already compromised valve. If his oxygen saturation does not land at an acceptable level (70 or higher), Riley may need to have the shunt put in (yes, that would mean more heart surgery). That would likely lead to valve failure and he would need that replaced. And recall that valve replacement means more valve replacement surgeries as he grows.
   
   
6. We have also come to the realization that a heart transplant is not "one to two to three decades" away, as we always thought. Rather, reverting Riley to the Glenn makes it more likely that he will need a transplant in one to two to three years.
   
   
7. Ken had read about another child online who had all the same surgeries Riley had an then was not a candidate for a transplant because of all the scar tissue in his chest. One of the doctors here told me that scar tissue does not preclude him from being eligible. However, every time he gets a blood product, his body builds antibodies. There comes a point where he has so many antibodies in his blood that it makes it very unlikely that a match will be found.
   
   
8. Finally, to top off all of this depressing news, when I was talking with one of the doctors, he used some extremely disheartening language when talking about Riley and Riley's future. He said, "assuming that we are able to get Riley healthy enough to get him out of here." And I stopped him and said, what do you mean "assuming we can?" He basically said (and I guess we know this on some level), that there is no guarantee that Riley will get healthy enough to ever go home.

Happy 4th birthday Riley

Today feels like a complete rewind to last year. It's Riley's fourth birthday (and Saturday and Sunday my cycling team raced in Brisbane for Riley again). Since Riley is still frustrated, grouchy and depressed, we've decided not to mention his birthday. We hope there will be plenty of time for celebrating when we get home.

Riley had a fairly good night, he was extabated at 9pm before Mom and Grampy went home, and the first thing he said was that he was hungry. He also asked Dad to spend the night again so I pulled a double shift. We had to hold him off from eating or drinking for an unbearably miserable four hours, but finally he was able to drink apple juice and eat some fruit.

His numbers have looked good. Only source of concern has been his oxygen level, which dips into the low-70s and even high-60s without oxygen blow-by. It's in the low- to mid-80s with the oxygen. It's too early to be worried about that. His chest tube output is amazingly low compared to his last three surgeries. He has three tubes and they have each put out about 300-400ccs since he got back to the ICU. Counterintuitively, his overall perfusion looks better now with his sats in the 70s than it looked with his Fontan sats in the mid-90s.

Out of surgery

Riley is back in the ICU and we've been able to see him. He was on bypass for an hour and received a lot of blood products (three units of whole blood plus FFP and albumin). Dr. Karl did not put the aortopulmonary shunt in so he has been reverted to a pure bidirectional Glenn. His saturations are mid-80s right now. He will probably stay intubated through the night. More as we know it.

Hug your children

Riley has spent the last several days feeling overwhelming sad and angry. He does not want us to touch him or talk with him. He tells us to stop talking and to go away. This is so hard for us to hear. We want nothing more than to scoop him up and to tell him that we're going home and that we'll never, ever have to come here again. We don't want him to feel pain or to be poked and stuck with needles or drugged with sedatives anymore. We want to love him and to hear his voice and to see him smile. We miss our little boy so much. My whole body aches and I feel so sick. I just want him to come home and to sleep in his own bed, to play with his cars. I want to hear him in the backseat of my car reading the street signs and telling me that he sees a Porsche or an Audi or a Volkswagon. I want him to ask us to read Busy Town and Cars, Trucks, and Things that Go (aka Goldbug). I want him to sit at the kitchen table and eat a big bowl of pasta with olive oil and cheese on top. I want him to keep teaching his baby brother all of the letters of the alphabet. I want to see him play baseball again. All of those things seem like a dream now, like something that I made up. I don't understand why this is happening.

Riley is now in surgery. There was some concern that he is developing an infection because his white-blood-cell count was rising. His blood-sugar level was also high, so he was getting a insulin drip this morning. He was taken down around 8:45 am. We were told that surgery could take six or seven hours.

We need him to get better so that he can come home. I just want to hold him and kiss him.