One-and-a-half steps forward, one step back

Riley had been eating the past two days. He wasn't eating much, but a little bit was a big improvement over not eating at all. But today, all bets were off. Breakfast: a couple of bites of tofu left over from dinner the night before. Lunch: nothing. Dinner: five bites of cottage cheese.

His chylothorax has him on a restricted diet which allows him 6.6 grams of fat per day. This is very frustrating because he sometimes asks for food--food that he isn't allowed to eat. He has asked for pizza a couple of times. He's also asked for cheddar cheese. Part of his lunch today was cooked carrots. When I told him there were carrots (he probably thought they were raw), he asked for bleu cheese to dunk them in.

Can't say I blame him for not being interested in much of what the kitchen brings to him. Scoop of white rice. Pile of overcooked carrots. No salt or butter on either. Side of applesauce. The frustrating part is that he isn't even interested in eating stuff he normally likes at home, like fresh fruit. No interest in plums, strawberries, bananas or other good stuff.

He's never going to get stronger if he doesn't start eating more regularly. He is now able to lift up his head a bit. But he is still unable to sit up unassisted or stand. Ken has propped him up to a standing position, but him legs can only handle a second or two. Plus, the longer he is immobile, the more muscle tone he will lose.

Much improved

Riley's spirits have come around. Yesterday morning we took him down to the playroom and he started talking more and we even got a few smiles. That seemed to break his shell and he's been much more talkative and happy. He's been playing with his cars and eating. He wants to eat by himself so we move the table over his bed and let him handle his meals on his own. As I type this Riley and Suzanne came by the parent room in the red wagon. He just asked to go for a ride on the elevator.

I stayed overnight with Riley and he got some good sleep. He'd gotten less than four hours sleep in the previous 24 hours. At 4:30am he woke up and said "Daddy, I want to go to the playroom now and see cars."

He has just the one remaining pigtail drain in his left chest. The drainage had come down considerably but yesterday morning he pumped out over 250cc of fluid so there must have been a clot. That happened just after the doctors talked about pulling it. He's still draining a good volume so it won't be coming out today. Doctors are looking for drainage of less than a tenth of what he's putting out now before they consider yanking it. Sometimes the drainage drops slowly, other times it will just come down quickly, so there's no predicting when it will happen. Fortunately this drain is the one thing standing between us going home. Once it's pulled, we'll probably be out within 24 hours. However, the doctors have cautioned us that Fontan patients can sometimes drain for a long time, some have gone for several months. Riley's cardiologist predicts that we'll go home "sometime this weekend or shortly thereafter" but we're not holding our breath. We also know that "real soon" is UCSF code for "before Thanksgiving."

We're thrilled to see the old Riley again and are enjoying hanging out with him, even if he's often uncomfortable. He is on the meds he'll have at home - aspirin, lisinopril, lasix, digoxin and amoxicillin - and he's also getting tylenol with codeine ("tyco") for discomfort. He's not taking anything intravenously but they're keeping the PICC line open and flushed.

There probably won't be much to report until the drain is pulled but we'll keep you all up to date.

Long, sleepless night

I don't know how the whole night can disappear with so little sleep. Actually, it's all the stuff that nurses need to do overnight that make sleeping hard. Poor Riley didn't fall asleep until after 11pm. Then he was woken up at 2:30am so that labs (blood) could be drawn from the PICC line in his neck. Unfortunately. the PICC line wasn't functioning properly--probably a clot in the line somewhere. But it took 15 minutes of screwing around with it, along with a temp reading and a blood pressure before Riley was able to relax again. I imagine, though, that it's hard for him to ever fully relax because he never knows when another doctor or nurse will come in to poke at him. There was something else at 4am, but I can't remember what. And then at 5:30am, the nurse has some kind of solution to put in the PICC line to disolve any clot. Then at 6:15am, she came in to see if the clot was disolved and to draw labs. Fortunately, it worked, but it was a hard night. Ken arrived soon after, and I'm on my way out to get a bit of sleep.

There was some progress made with eating yesterday and Riley did get out for a ride in the red wagon, but Ken will hopefully update you all a bit later. I need to head to the apartment for a little sleep. Thanks to Marcy and Jeff for letting us monopolize their apartment!

More tubes are out

The nurse practitioner removed the two remaining bulb chest tubes and the pacer wires today. Riley still has the pigtail drain in his left chest and the PICC line in his neck, but other than that he's tube- and drip-free. They used three nurses to pull things quickly and all at once, we got away with just one big "OUCH". Unfortunately the pigtail comes with the VCR-sized drainage box so moving him is still difficult. This afternoon we're going to try to pack him into a red wagon and take him down to the child life room. We're hoping that a change of pace will improve his spirits. Further, he should be feeling more comfortable now that those tubes are out.

Emotionally, Riley is the same. We still haven't seen him smile and he doesn't speak much. He did ask for Mom to "read a book" when he was sedated earlier for the chest tubes. He also isn't eating much - he has hardly eaten anything today or yesterday. We have been spending time with the child life specialist since we're fresh out of ideas. Just talking to him as if he is the "old" Riley doesn't seem to be getting us anywhere.

Update on blisters and sweating

We never posted an update on the water blisters and sweating. Late last night the ICU fellow came by and looked at Riley. He didn't have a conclusive answer for the blisters but suspected it might be heat rash, a virus or some allergic reaction to the tape. None of these explanations made perfect sense so he sent a venous gas and a lactate test to make sure it was nothing heart related. Everything looked good so the decision was to just leave it and let it heal. His face isn't as red today but the blisters are still there.

Riley hasn't been having the sweats at all today, and coincidentally he also went back on the morphine. The doctors seemed to like our guess that the sweating might have something to do with morphine withdrawal. So there you have it.

Tenkoff, central line removed; PICC line added

Early this morning we cut Riley off from all food and drink in preparation for his sedation associated with removing his Tenkoff drain and central femoral (groin) line. Hard to believe that Tenkoff was removed, considering it drained nearly two liters in a single day just a week or so ago. It was also considered a factor that his Fontan operation wasn't working. But drainage tapered off and doctors felt it was okay to remove. Riley still has three other drains--two bulb drains and one pigtail. They wanted to close up the femoral line because it has a high infection rate, considering it's located inside his diaper.

Since access is still needed to administer meds and to draw blood, a PICC (Peripherally Inserted Central Catheter) line was added. PICC lines can be in place for several weeks and have a low infection rate. The long plastic tube is deep inside Riley's body, which should make giving meds less painful. We believe he's been avoiding telling us that he hurts because administerings meds though the IV in his wrist stings. While he still won't like getting meds (negative association of nurses in general), he shouldn't have any physical discomfort while IV meds are going in. The plan was to place the line in an arm or leg, but because his veins are so tiny, they could not get it to work. As a result the line ended up sewn into his neck. So he'll have a scar on the other side of his neck to rival the one from ECMO.

Unexplained sweating, blisters

Just before I was about to head to the apartment for a much-deserved night of sleep (I was here overnight last night), I touched Riley's face and it felt very bumpy. We turned up the lights and there are tiny blisters all over his face, neck, chest, and underarms. The doctor on call was paged, he examined Riley, and now we wait. Apparently, he wants the fellow to take a look, but we aren't sure if the fellow will be in sometime in the next five minutes or five hours.

This blistery turn followed a day when mom and dad sensed things weren't right. First, Riley's resting heartrate has jumped between 30 and 40 beats per minute to 110. Second, he has been sweating excessively all day long. I have changed his pillow several times because he keep soaking through, leaving a large sweat mark. Thrid, his cheeks have also been very red today. He has not been red like this since before surgery. All of this together makes us very nervous. But so far no answers, just questions. So we wait and hope the boy will at least get a couple of hours of much needed rest, even if his parents don't.

Still not himself

Riley is still withdrawn but we're seeing some slight improvements. He is now making eye contact with Mom and Dad, but saying very little. We are trying to get the nurses to batch all of their procedures together so he will be left alone as much as possible. An hour ago they gave him his oral medicines (digoxin, amoxicillin and tylenol), IV meds (lasix), took his vitals, emptied his drains and changed his dressings and now they should be able to leave him alone for several hours. We're also going to talk to the child life specialist again today about ways to make him feel more like himself. We didn't bother with the birthday celebrations yesterday since he wasn't at all interested. The hospital brought by cake, a big banner and a present but we've stashed it away until he feels better. His birthday celebration can wait until he's ready.

A few days ago Suzanne experienced a scare when Riley's uncovered incision popped open after he was picking at it. He wasn't in any danger but it certainly isn't pretty to look at. Fortunately the underlying bone is wired shut. They just leave the wound open to treat, inserting wet dressings into the wound every 12 hours and covering with a light bandage. As a precautionary measure they put him on some Vancomycin to conquer staphylococcus (staph infection) although there are no visual signs of infection and the wound looks very clean. If the cultures come back clean they'll discontinue the Vancomycin since it can have kidney-related side effects.

Riley spent a few hours in a special chair yesterday and Saturday, and will spend some more time in it today. Getting him up and moving about is critical to drainage and a good recovery. He's in a very weakened state and has a hard time keeping his head up so the physical therapist will come by today with some more recommendations. It's difficult to get him sitting up with the pigtail drain in place. It's attached to a drainage box about the size of a cheap VCR. They're looking at more mobile replacements.

Yesterday we noticed that the pigtail drainage was taking on a more milky consistency. We'd been watching for this as a sign of chylous fluid since Riley had eaten some foods with fats (milk, french fries and crackers). Sure enough, it looks like Riley does have chylothorax as anticipated. That means we need to put him on a low-fat diet for the next six weeks until his lymph nodes can heal. Given all that we've been through, chylothorax isn't all that bad.

Happy birthday and racing for Riley

Riley is still feeling very sad and is barely interacting with us. His language has regressed and he pretty much just says "no" and "I don't want it." We talked to the child life counselor, and she says this is a normal coping mechanism. Kids will often regress in different ways. Since Riley is so vocal, he knows that communication is one thing he can control and he's withholding it. These will be the hardest days for Riley who doesn't understand where he is or why he's being kept here. I stayed with him overnight and he slept a lot, but woke up crying several times saying "no, no, no." His vocabulary is slightly more extensive today, at one point he said "I want to say bye-bye" and "I want to go home."

On the plus side, he's drinking a lot of juice and eating crackers, french fries and cookies. When asked if he wants anything he just says "no" so we've stopped asking - he'll usually take what we offer. The child life team brought a Happy Birthday banner, cake and a present. He's not into it so we'll try again later, or tomorrow if necessary. His birthday can wait until he's feeling better.

The Ronde van Brisbeen was this weekend, one of the biggest bike races on my team's calendar and I wish I could have raced with them. The guys have been extremely supportive and a lot of them raced in honor of Riley with photos in their pockets, pinned to their jerseys and taped to their top tubes. (Amazingly there are two other guys on my team with kids with heart defects, one has had surgery and the other has surgery planned for June.) Riley must have brought everyone luck because Peninsula Velo spent a lot of time on the podium - first and second places yesterday, first and thirds today and first, second and fifth places in different categories for the overall omnium. Randy Smith sent this picture. Thanks guys for all of the support and congrats!

Grumpy

Riley is out of sorts today. He really hasn't said much besides "no," "Mommy" and "I'm sad." For him, these are the toughest days. He doesn't realize that there's good reason to be happy now. He was unconscious during our hardest moments, but now he's awake, uncomfortable and not understanding why Mommy and Daddy are letting him go through all of this. He's mad at us and with good reason. Hopefully he'll be in better spirits tomorrow for his birthday.

In an hour we'll try sitting him up and performing chest therapies to get him to clear out those lungs. Dr. Karl came by to say that he looks good, but that we should be prepared to be in the hospital for another two weeks. The docs always give the worst case estimate, in 2004 we were told two more weeks and it was another ten days. But in any case, we're going to be here for a quite a bit longer.

Finally, in a surprising development the chairs of cardiothoracic surgery, cardiology and pediatrics dropped in this morning along with the president of UCSF. They apologized profusely for the lack of communication, promised it wouldn't happen again and shared a group hug with us all. Tears were shed and - I believe - lessons learned. To top it all off, they announced that they plan to name the PICU unit after Riley and would be donating $1 million in the hospital's name to his college education.

UPDATE: For those who still haven't figured it out, this last paragraph is a (pretty lame) April Fool's joke. It's a complete fabrication, even the part about the group hug.