Effusions not getting better

Just a quick post because I'm super tired:

1. The x-rays/echos show that the effusions (fluid under his lungs) are not getting smaller, despite the massive doses of diuretics. In fact, they seem to be slightly larger, according to the cardiologist.
   
   
2. Riley should be having a cardiac MRI on Wednesday if scheduling can sort it out. The MRI will check blood flow and look for obstructions within his Fontan circulation.
   
   
3. If the effusions are not decreasing by Wednesday, they will be putting in chest tubes to drain the fluid. Since he will be sedated for the MRI, it makes sense to also do the chest tubes at that time, if needed to avoid a second deep sedation.
   
   
4. As a result of the congestion and the fluid build-up, Riley's oxygen saturation has dropped. It was 92 when he first got to the ER on Saturday. When we left the hospital tonight it was in the low 80s while an oxygen mask blowing oxygen towards his face.
   
   
5. Digoxin has been added to his list of medications. This was one of the medications that was discontinued following his cardiology appointment in January.
   
   
6. Depending on the findings during the MRI, a heart catheterization may be scheduled soon after.
   
   
7. Riley has been fighting off a temperature for the past couple of days. It spiked at 102 degrees this afternoon and continued early into the evening. Ken called while we were driving home to tell us it had dropped down to 100 degrees. A blood test to check his white blood cell count had been ordered.
   
   
8. Riley was very sad today. He barely talked, but he did eat a couple of strawberries (thanks Jennifer!!), a couple of bites of applesauce and one animal cracker. This is a big improvement over the past two days, when he ate nothing.
   
   
9. Grampy is here!!! This will be a big help as we try to juggle Carter's needs with Riley's needs. And when Grampy walked into Riley's room, a HUGE smile came over his face, his arms rose up into the air and was thrilled--for at least a minute or so.
   
   
10. Finally, thank you to Christa for watching Carter today so that Ken could get some rest, and for helping to organize some meal schedule for us! Thank you to Jennifer for a surprise visit, a lunch run, and a Sudoku book! And thank you to Lisa for picking Grampy up from the airport, the Bob the Builder DVDs, and for a wonderful dinner!

We are so grateful to all of our friends and family and are humbled by your continued love and support. There is no way we would survive these hospitalizations without you.

Albumen lower, valve more leaky

As part of the daily blood labs, Riley's electrolytes are being tested (since he's getting so many diuretics and peeing too much can tilt them off balance), and so is his albumen (the protein in the blood). Albumen has dropped since his blood test last week. Last week it was 3.0, and today it was 2.2 (normal range is 3.3 to 5.0). This is making the doctors wonder again about the PLE. A stool sample could have confirmed PLE. We should have had the results of the stool sample taken to the lab last week, but it turns out that they ran the wrong test. So we need another sample. However, since he isn't eating anything, who knows when we'll get an opportunity to get another sample. And since he isn't eating, his blood protein levels may have dropped anyway. So it is possible that a stool sample will be useless at this point.

The other thing that was discussed with Dr. Moore (another UCSF cardiologist) was that it seems that his "mild-to-moderately leaky" valve is moving towards being a more "moderately leaky" valve all the time. This could be part of the fluid problem. The valve could be replaced during surgery. This is all second-hand from Ken who was there to talk with Dr. Moore. Ken will have to update this information if I'm off or if there is more to add. [Update from Ken: Dr. Moore didn't give me the impression that Riley's valve leakiness has changed, just that in the situation where effusions are suspicious, a leaky valve is one of the candidates. They'd prefer that Riley's valve wasn't leaky at all but nobody has indicated that it's gotten any worse.]

As for the fluid, there isn't much news to report. We are basically going to be in a holding pattern while we wait for the diuretics to start draining out all that fluid. Riley was very sad and angry whenever anyone wanted to go near his IV to flush it. A couple of weeks ago, we started talking about how it is OK to say "no" if you don't like something that someone says to you. Or if someone does something to you that you don't like.* So he would shout several times, "I don't like that. I want you to stop doing that. I need a break." Sadly, we can't necessarily respect his boundaries in the hospital.

We have not talked with Dr. Tarnoff since yesterday, but UCSF has been in contact with him to keep him up to date. Tarnoff will be at the hospital on Thursday. Perhaps we'll be able to talk with him by phone before then to get his input.

I'm not sure how the x-ray today compared to yesterday's x-ray. If there was much change in the fluid, no one said anything about it. They did do some kind of swab in his nose to check for different kids of the flu and some other infections, which all came back negative. They also lower his fluid intake to 880 milliliters, down from 1000 milliliters.

He mostly slept and watched TV today (we watched Free Willy twice). He is so tired because he isn't eating anything, because he's just not getting enough rest in the hospital, and because his body/heart is working so hard with so much extra fluid in his lungs.

(*sidebar: Trying to empower our shy guy to communicate how he feels came up after a very strange incident in San Francisco when some Japanese tourists were so enamored with his blonde hair that they actually picked him up while he was eating his lunch to go be photographed with him. Without permission--from me or Riley. He was very shy about the whole thing and I asked those people to leave him alone immediately!! Anyway, this is totally unrelated to the hospital, but he has been applying this concept properly.)

A long, horrible day

The good news is that Riley does not have a chest tube--yet. The bad news is that he has endured a horrible amount of pain and frustration in an attempt to get an IV line put in. He needs an IV line put in to give him massive amounts of diuretics because he has at least 150 milliliters of fluid under his lungs. The doctors are hoping that they can reduce the fluid with the directs and limited fluid intake. The is going to take time. The alternative was a chest tube, which can have faster results, but it much more intense because he would need a heavy sedation to have it put in.

The day started with labored breathing. He ended up in the ER at Redwood City. I would not let them do any blood work. I know he is a tough kid to get an line in with his tiny veins, so I let them try once and only once to get and IV started. They told me that they would not transport him without an IV. It was unsuccessful, so we dropped it. He did get two giant shots in his leg--big doses of antibiotics--just in case it is pneumonia. They did not need an IV to transport him, which was nice. The nurse in RWC was very dumb and she told me that if his oxygen saturation went below 92 percent (which is his baseline), that he would get brain damage! I told her that wasn't true and that she shouldn't talk about things like that with parents when she clearly doesn't know what she's talking about. I complained about this to the ER doctor and to Dr. Tarnoff, who agreed that she was dumb.

The ride into UCSF was easy and the team was top-notch. As soon as they arrived at RWC, things got much calmer. Riley is normal to them.

Once he was up in 7-North, they needed to get an IV started. They tried his wrist, his arm, his hand, his other hand, his foot. And it wasn't like they just tried to put it in and when it didn't work, they'd back out. Oh no. They would back up a bit and turn the needle and jam it in another direction. And again. And again. At this point, I became the mother from Hell and I started shouting at them and telling them to stop. Riley was hysterical and gagging from shouting. It was horrible. I'm not sorry for yelling at them, even if they are trying their hardest. They should try even harder. I decided to leave and get Carter home. I'm sure the hospital staff was glad I was gone when they came back to stick him some more. Ken said they tried again in his arm and then ended up stitching an IV into a vein in his groin after the arm attempt failed. Apparently Riley was so tired that he just kept repeating that he was tired and wanted to sleep.

As for the fluid, they don't think it's pneumonia because there wasn't any crackling sounds when they listened to his chest. So the fluid must mostly be under his lungs. Hopefully, the diuretics will start shrinking those pockets. We will also have that pesky, hard-to-schedule cardiac MRI to look for obstructions. If needed, a cath will also be done to hopefully fix any blockage.

This is strange. When they weighed him, he weighed 41 pounds. This is up more than three pounds from about a week ago--if their scale is accurate, which I imagine it is. With all the fluid under his lungs and all the puffiness, could he really have three pounds of fluid in his tissue? Ken and I have been talking about how his eyelids have even seemed puffy to us.

Finally, once I got home I was putting Carter to bed when I heard a crash somewhere in the house. It was very odd, I'm at my wits-end, and I'm exhausted. So I did the only logical thing I could thing of and I called 911. They were here within 10 minutes while I was on the phone with the station. They did an outside and inside check and everything was clear and the house is totally locked. I felt silly, but it was weird to hear that loud crash. They did tell me that there are a bunch of teens out on a deck on a neighbors backyard. That was probably it, but I'm too exhausted. That also explains why this post is probably so out of order and confusing.

*This was posted by Suzanne, not Ken as it indicates on the blog. Ken stayed overnight with Riley at the hospital.

Riley is being transported to UCSF now

Riley had a difficult night with coughing and vomiting. This morning Suzanne noticed that his breathing was labored with flared nostrils and concave chest. She contacted the advice nurse and they instructed her to bring him to the Kaiser Redwood City ER. Riley's oxygen saturation is normal (for him) at about 92% but he is clearly experiencing perfusion problems. He looks very blue to us and his extremities are cold and white to the touch. They performed a chest x-ray and discovered significant fluid build-up on both sides of his chest. His lungs are barely visible on the film, especially when compared to the x-ray from January. After consultation with the cardiologists they've decided to transport him to UCSF. They are freeing a bed for him now and should send an ambulance for him between 3 and 3:30 pm Pacific time.

Based on the x-ray they suspect either pneumonia or Fontan-related complications (or both). They administered some preventative medications in the event it is pneumonia (we didn't get the name). They're working him up for an IV now in advance of transport. I ran home with Carter to pack a bag and grab some stuff for the hospital. I also need to change - I was riding my bike when Suzanne called me and I've been walking around in my cycling kit for the past several hours.

We'll have more updates after we are admitted to UCSF and there is progress to report.

No fluid in abdomen

Riley had an ultrasound of his abdomen today and the test found his abdomen to be clear of fluid. This is a good sign. As a result, Dr. Tarnoff said it is unlikely that it is Protein-Losing Enteropathy (PLE). That said, we are still going forward with the stool sample to test for protein in his stool. If the stool sample test also comes back negative, we can eliminate PLE from our list of concerns. During the exam today, Riley said that he it hurt quite a bit while the technician was pressing around his liver. We're still not clear on whether or not his liver is enlarged. The other thing that was discovered was that there is fluid under both of his lungs again.

Whenever we end up in Redwood City for Riley's appointments or in the Emergency Room, there is always great concern over his condition because his anatomy is so unusual. This usually leads to unnecessary treatment or just sitting around longer while people rush around trying to figure out how to treat him. The latter happened today. Following the test, I was a little extra concerned because we were told that we could not leave until the doctor came to talk with us--a doctor we have never met before. We ended up waiting nearly an hour while the results were reviewed, discussed, reviewed again. When this doctor came to talk with us, he asked about Riley's breathing and directed us to take him to the ER if he started to have difficulty breathing. This is, of course, because Riley has a "significant" pocket of fluid under his left lung. There was also a smaller pocket under his right lung. If this is true, then that has changed since our visit with Tarnoff last month when we were told that the right side had dried up. We're also not sure what he meant by "significant" since the actual pocket was not measured during the exam. Since these technicians and doctors are unfamiliar with Riley, it's hard to know if things have actually changed. I hope to talk with Dr. Tarnoff tomorrow to find out how the effusions are different from the last check, if at all.

I also picked up the stool sample kit from the lab. Once the specimen is handed to the lab, results will take three or four days.

If PLE is eliminated from our list of concerns, it does not explain why Riley's albumen is low (his measured 3.0 instead of the regular 3.3 to 5.0). Tarnoff said that it could be dietary (ie: he's not getting enough protein in his diet), and he may refer us to a nutritionist. Also, we still don't know why the fluid is accumulating under his lungs. Perhaps the best we can hope for at this point is an obstruction that can be fixed with stents in the cath lab.

Finally, I had a very negative experience today with the nurse filling in for Dr. Tarnoff's regular nurse. I was told that this new nurse would call me last Friday to set up the ultrasound and the lab work in Redwood City. To be proactive, I called and left a message expressing my interest in getting things going as soon as possible. I did not hear back from her on Friday. So I called her again this morning. When she finally called me back, she was extremely rude and made it clear that she had no intention of setting up my appointment and that I needed to call radiology in Redwood City for myself. If she had just called to tell me that in a nice way, I would have said okay. But her attitude, tone and annoyance that I had the audacity to call and leave two message in an effort to find out what is going on was inexcusable. I was fuming. Yes I'm anxious to get the tests set up so that we can try and figure out what is wrong with Riley. And yes I'm going to be frustrated when people don't do what they are supposed to do. I can't wait for the regular nurse to come back!

In plain English, thank you very much

Grammy had a great idea to email cousin David for some help understanding a bit more about pressures and Fenestration and stenting. His descriptions are incredibly helpful, so I wanted to share it with everyone else. David has extensive hospital experience as a respiratory therapist and is studying to be a physicians assistant. There have been many days when we wished he was standing by our sides deciphering all the technical lingo.

Normally we have the MASS pike running into Rt 93. A nice big road that can carry lot of volume and its 4 lanes. Not a lot of back up (IDEALLY).

Just like we have our superior Vena Cave(SVC) and our inferior vena cava (IVC) running into our right atrium of the heart. NOt a lot of pressure, big wide roads.

Mass pike goes down to 93 then smaller down to 128 to Rockport with all its little roads.....to stockholm ave. just like from the heart to the lungs, normally.

Usually from the Right side of the heart, it can handle the volume of blood, not a lot of back up, and it pumps it forward using pressure into the smaller, but strong pulmonary arteries and into the lungs with small vessles where there more pressure, but since the heart is pumping it forward, it can't go back....

Well with Riley, the mass pike is like his(SVC/IVC) and bypasses the heart nad hooks up to 128 directly (pulmonary arteries), going from 4 lanes down to 2. This traffic jam/bottle neck leads to back up of blood, Without going to the heart to pump it forward directly into the lungs, backs up into the liver and sounds as though it leads to some unkown process in the liver which causes protein to leach out into the intestines. (osmotic pressure blahh blahh blahh)....

What they need to do, and sounds like they are going to is image the area (MRI)and see if there is some sort of anatomical "road block". and/or go to the cath lab and measure the pressures and see how high they are.

They can then either stent open the anatomical "bottle neck" at the mass pike/128 junction or they may have to build a bypass road (Fenestration) running off to the right heart. This allows all the backed up "traffic/pressure" to be releived and to go through the heart and pumped out, some into the lungs, but some into the body (due to his heart defect) without being oxygenated.

Long story short, its a waiting game for them to diagnose exactly what is going on. They may need to just stent some sort of narrowing...which they can do in the cath lab, or they may need to build the "bypass road" (Fenestration) with an operation.

Albumen low, PLE possible

Riley had some blood work done yesterday. We had hoped to eliminate some of our fears, but for now, we have more questions. Dr. Tarnoff called this morning to let me know that Riley's electrolytes are within the normal range. However, his albumen levels are slightly lower than normal. Albumen is the protein in the blood. Low albumen levels can be a sign of a serious condition called Protein-Losing Enteropathy or PLE.

Dr. Tarnoff said it is unusual to have slightly-lower levels of albumen in the blood. It is not clear if lower levels mean that he is just started to develop the disorder or if it is something else. So more testing is needed. First, Riley will have a stool sample. If they find protein in his stool, he is losing protein from his blood, and that would confirm PLE. He will also have an abdominal ultrasound to look for pockets of fluid in his abdomen and also at his liver and the hepatic veins that were moved during Riley's Fontan. A cardiac MRI is still in the plan so that they can look for obstructions in the Fontan circulation.

If this is PLE, Dr. Tarnoff said that it is a very rare form of it and there is no established treatment. We have tried diet (with the two rounds of non-fat diets). We have tried lots of diuretics, which have not worked. Other options include a round of steroids. Intravenous globulins are also an option, which might make his chemistry better for a short amount of time. Heparin injections might also be a possibility. "Everything is a two-edged sword," Tarnoff said.

Mostly, it seems that we're heading back to surgery (in case you lost count, that would be his 4th heart surgery). If the stool sample and abdominal ultrasound confirm PLE, it seemed that Fenestration would be the viable option. Recall that Fenestration was a hot topic when Riley's heart was in severe distress following his Fontan. Fenestration would create a hole between his inferior vena cava and his atrium. If he got better as a result of the lower pressures, they could then close the Fenestration in the cath lab several months or years later. The downside, of course, is more heart surgery. In addition, Riley would end up with lower oxygen saturation levels. As a result, he would likely be bluer in color and have less energy.

Clearly, this is not the road we want to head down. It is frustrating that his albumen levels were not tested in September when Riley was in the hospital for a week and the pockets of fluid under his lungs were discovered. We have also been asking about PLE and his puffy hands and feet. Why haven't we looked for pockets of fluid elsewhere before now? Why haven't we looked at his liver to see if it's enlarged before now?

Oxygen saturation unchanged

Just wanted to update my previous post regarding the blueness we witnessed with Riley's coloring Monday night. Per Dr. Tarnoff's request, I took Riley to Kaiser to check his oxygen saturation today. The numbers haven't changed at all from our January visit--both clocked at 92 percent. So the big question remains as to what caused the blueness.

Also, when Riley was complaining of his upset stomach tonight, Ken asked him to point to where it hurts. Riley pointed to his side, not in front where one typically feels an upset stomach. When asked if it also hurt on the other side, Riley said no. I'm not sure where Riley's liver is because of his heterotaxy, but Dr. Tarnoff's liver theory (see previous post) seems plausible, based on this little experiment.

Blue incident

Last night after Riley's bath, his lips turned quite blue, as did his feet and hands. This was a bit shocking to us because we had never seen him get so blue before. It looked like he had eaten a blue popsicle or lollipop. The blueness in his lips lasted five to 10 minutes and the blueness in his feet lasted longer. There was also some significant patches of mottled skin on his arms, especially near his elbows.

I called Dr. Tarnoff's office this morning to alert him to this incident, and he asked me to take Riley in to have his oxygen saturation checked to see if there have been any changes since his appointment in January. We'll be doing that after Carter gets up from his morning nap.

I also asked him about the constant upset stomachs that Riley suffers from. We had hoped that the stomach issues would discontinue when he stopped taking his Digoxin last month. But since they have continued, Dr. Tarnoff said that the problem is "probably anatomical rather than chemical." He speculated that the issues could be related to the hepatic veins, which were moved during his Fontan operation in March 2006. The hepatic veins drain part of the liver circulation, and because pressures change after the Fontan, it can raise pressures in the liver. And since the liver and the stomach are both in the belly region, Riley could actually be feeling discomfort there and not in his stomach.

When Riley has his heart cathertization in March or April, they should be able to determine if there is a pressure problem the liver. I'm not sure what they would do to alleviate the problem, however, if it is a liver issue. If it is not a liver issue, Dr. Tarnoff said that he could refer Riley to a gastrointestinal specialist. To me, that sounds like a whole new can of worms.

A second opinion

We had the privilege of talking with Dr. Desai today at the Lucile Packard Children's Hospital annual Valentine's Day party. This is not Riley's regular cardiologist, but he knows a bit about Riley's case because he talked with us extensively while Riley was on ECMO at UCSF following his Fontan surgery last March.

We gave him a brief update on Riley's health over the past year after being discharged from UCSF: The ups and downs with diuretics, the week-long hospitalization in September that revealed fluid under Riley's lungs, the disappointment that the fluid was actually accumulating instead of disappearing when we had a follow-up in January. We also talked about the current plan that includes a cardiac MRI, probably a heart catheterization, and numerous blood tests.

Dr. Desai's reaction affirms some of our fears. His said that there is no reason a Fontan patient should still be draining a year after surgery unless there is a problem. He estimated that diuretics will not fix the problem. Rather, the increase in diuretics is likely just a band-aid covering up a bigger problem. He gave three possible reasons for the continued fluid accumulation: 1) some kind of blockage within the Fontan system, 2) a serious condition called protein-losing enteropathy or PLE, 3) some kind of lymphatic problem. Because Riley is not his patient, he said many times during our conversation that he cannot tell without all the information what is the true cause of the continued fluid accumulation. He also said that Riley looked great, and as Dr. Tarnoff often says, they way a patient looks is sometimes more important than any specific test result.

This doctor said that some questions need to be answered soon. For example, what is Riley's albumen level? Albumen is the protein in the blood. If the albumen level is low, that would be an indicator that Riley is developing PLE. This can be answered with a blood test. Riley is scheduled for a series of blood tests before his next appointment in March.

Dr. Desai also wondered about Riley's Fontan pressures--the pressures in the different chambers of the heart and lungs. This could be determined with a heart catheterization. If the pressures are not balanced properly, then that could be an indication that the Fontan is not working properly.

Fixes for both of those problems could happen by re-doing the Fontan.

The third possibility is that there could still be damage in his lymphatic system. That would mean that the fluid under his lung is lymph fluid. However, Dr. Tarnoff felt it was not lymph fluid, although Ken and I cannot remember his explanation. Still, if it was lymph fluid, Dr. Desai said that there could be some surgical repair for the lymph system, if and only if the source could be identified, which is very difficult, he said.

Still, we will hold out hope that if there is some blockage, that it can be repaired in the cath lab and NOT the OR.