First day of kindergarten

Riley started kindergarten on Monday!

Cardiologist check-in

It's been a while since we've posted about Riley's health, which is A Good Thing. He just finished his first season of t-ball and is almost ready for kindergarten in the Fall. We're all looking forward to a summer vacation in Cape Cod with the Norton in-laws. Other than occasionally feeling tired and being a bit slower and smaller than his peers, life has been reasonably normal.

We went to San Francisco for an echo and appointment with Dr. Tarnoff this morning. Riley's oxygen sats were 81% which is basically unchanged since the Fontan takedown. Tarnoff says the valve is still "mildly leaky" which is also good news. He reduced his Lasix and aldactone dosages and plans to see us in six months. Also, at our request he switched Riley to a pill form of aldactone, which allows us to breathe a major sigh of relief. The liquid aldactone comes in a special compound and needs refrigeration. It also needs to be refilled every few weeks. Not only does it make traveling a hassle, the Kaiser pharmacy can never seem to get it right. Most of the time the compound hasn't been filled when we arrive and/or the attendant can't find the prescription. Pharmacists these days spend most of their time punching buttons and filling pill jars, so what used to be a regular day's work -- crushing, stirring, measuring and mixing -- now baffles them. So pills are good.

Dr. Tarnoff recapped Riley's condition and outlook: either oxygen saturations will drop or the valve will get worse and "something else" will need to be done. Initially they will use medications, then surgical interventions, then ultimately a heart transplant. Nothing new here. But Tarnoff did say something we haven't heard since the Fontan setback. When talking about time and the uncertainty of interventions, he said "one, to two, to three decades." It's the first time we've heard the word "decades" since Riley was on the Fontan circulation. Every little bit of encouragement counts.

One other little piece of trivia: Dr. Tarnoff mentioned that we don't know where Riley's appendix is. Since he is heterotaxic, several of his organs are flipped. That's something to keep in mind as Riley grows - if he ever needs an appendectomy the doctors might want to poke around a bit before they make the standard right abdominal incision.

A poetic moment

After breakfast the other day, Riley blurted out the following:

How fast does traffic go?
Does it speed away or go slow?

Maybe he'll be a writer just like his mom.

Riley has a check-up with Dr. Tarnoff Monday morning. He'll be having an echo and his appointment will follow.

Cough remains, but much better

The vomiting must have been the last hurrah of the pneumonia. Since then Riley has been feeling much better and has had significantly more energy. He still has a rough cough, but it does not seem to affect his mood, his breathing, or his ability to sleep. We are all very grateful for this speedy recovery. As long as he continues to be energetic and the fever does not return, I'm thinking of sending him back to school either tomorrow or Wednesday. Hopefully tomorrow.

A bout of vomiting

All in all, yesterday was a good day. Riley was slightly more energetic and was even getting into a bit of trouble with his little brother. They were shouting at each other--an annoying game they have--and I decided that kind of game was best played outside.

They were out there for about 10 minutes before I decided to go join them in the yard. When I got out there, Riley was holding his tummy, saying that he wasn't feeling well. Then he started throwing up. Four times. And it wasn't a coughing fit that set it off.

He hasn't eaten much in the last 10 days, so it was disheartening to the see the little remains of his lunch on the lawn. Not sure what to make of this episode. I'm hoping we can just chalk it up to a little too much excitement.

Albuterol prescribed, slightly better

The cardiologist gave the green light yesterday for Riley to get the Albuterol inhaler. After some hesitation, Riley has been doing great with it and it seems to be helping.

Riley actually slept soundly last night--a drastic comparison to the previous night when uninterrupted sleep was infrequent--and actually was a teeny, tiny bit smiley this morning. The smiles didn't last long, but I did see them for a couple of minutes.

The cough is still frequent today, but it seems that it might be a little less intense, with less gagging. I have not taken his temperature today because he hasn't seemed grumpy enough for me to think he's running a fever. Overall, it seems that Riley is getting better.

Unfortunately, he will miss his second tee ball game tomorrow. If he's feeling up to it, we are going to take him over to the park so that he can at least watch his teammates play. I also hope that by next Wednesday, he'll be able to go back to school. It's been a long two weeks at home.

Pneumonia in both lungs

It's been a rough couple of weeks here in San Carlos.

Carter was sick for five days with a fever, conjunctivitis in both eyes, an ear infection, and an upper respiratory infection. He's been on a significant dose of antibiotics since last Wednesday evening.

The next day, Riley started to feel sick and ended up with a fever as well. We didn't think much of it because Carter had just had a nasty cold. Anyway, on day 6, Riley still wasn't improving. I called the advice nurse at Kaiser and she wanted me to take him to the ER, since he was having a little bit of labored breathing. We decided not to do that because he showed improvement after some Tylenol reduced his fever and his breathing became more natural and relaxed.

This morning I took him to the pediatrician and he could hear crackling sounds in both of his lungs. We were sent for a chest x-ray and there was quite a bit of white wispy marks on the x-ray confirming "double pneumonia," although he said that the right lung was worse than the left lung.

He was prescribed azithromycin (a powerful antibiotic), and we will go back tomorrow late morning for a follow-up appointment. He may end up with an albuterol inhaler, but it causes an increased heart rate and we wanted to avoid that until Dr. Tarnoff gave it the green light.

Happy but tired

Just two weeks ago, we passed the one year anniversary of the day I took Riley to the ER, which then led to those two additional open-heart operations and those eight weeks at UCSF.

Since he was discharged at the end of April 2007, Riley has gained back all of the weight he lost and then some, he's grown some permanent teeth and lost one baby tooth, he's joined a t-ball league, and he's been registered for kindergarten. We also moved to a new house which has a better outside area for play and bike riding.

Since we moved to the new house in January, he seems to get tired more easily. We're not sure if there is an underlying reason that he's getting more tired or if it is simply because he's exercising more outside. He really enjoys riding his bike, hitting the ball off the tee, and running the imaginary bases. There have been a handful of days when I had to carry him into the house, and he rested on the couch for a couple of hours before going to bed.

Yes we're feeling a little anxious that his increased level of exhaustion is something more than just more exercise. There has been nothing alarming enough that has made us take him in for a check up. Some subtle cues have been bluer lips, more frequent diarrhea, and a more regular upset stomach. Recall that diarrhea and upset stomach were prominent characteristics of PLE. I'm not suggesting that maybe it's come back. But mostly, I think I'm hypersensitive and the past two years during the months of March and April, he's been in the hospital.

For now, since I don't have anything more than heightened awareness and some symptoms that could very well be coincidental, we are going to wait until after his birthday (April 2) to go in for a check up. He will need a check up around his fifth birthday anyway, so I'm not going to offer him up any sooner than necessary.

Let's just keep our fingers crossed that he continues to do well and that his dad's birthday, his birthday, his brother's birthday, his mom's birthday, Halloween, Thanksgiving, and Christmas are all hospital-free. I can hardly let my mind think that we might actually get a whole year without hospitals or surgeries or interventions.

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.