Don't forget about Regan

Yes, she is standing pretty much on her own in the last pic. What a nut!

D+70: Rotavirus!

WBC 3.9
ANC 3198

Can you believe it?! Ryan has another *$&#^@ virus! Fortunately, it's unlikely that this one will threaten Ryan's health. The main symptom is diarrhea, and Ryan is already on some medications to treat that. Unlike some of the other viruses that Ryan has had, he probably picked this one up in the hospital (i.e. it was not dormant in his system when we came in). For Cheryl and I, this is just another confirmation that we have to be absolute freaks about cleaning the room and hounding people that come into the room. Ryan actually tested positive for the virus over a week ago, so that gives you an idea of just how benign it can be. Still ...

Ryan had another echo this morning and his heart is pretty much unchanged. We were hoping to see the fluid level drop a bit, but the current level certainly does not appear harmful. Ryan's pulse and blood pressure are normal (well, normal for Ryan), so it looks like his heart his in reasonable shape now. Every once in a while, Ryan's pulse will drop from the 120-130 range to 80-90. We would normally dismiss it as a blip with his pulse monitor, but his breathing appears to be depressed during these periods as well. Not sure what's going on there -- probably nothing.

Cheryl managed to get Ryan down to 30% oxygen on the BiPAP today. She eventually had to bring it up again, but that's another big step in the right direction. Keep in mind that Ryan was on 100% oxygen not too long ago.

Amazingly, Ryan opened his eyes a ton today. He is so strong. I don't know how he can open his eyes and pay attention to his surroundings after being through this much. Cheryl said that he would move his eyes when she would talk to him, which means that his awareness level is increasing. We are both desperate to see Ryan's glowing personality emerge again.

D+69: BiPAP Black Belt

WBC 3.7
ANC 3367

Love those counts! Ryan's respiratory picture is also looking up. He's still on the BiPAP at 40% oxygen, but the amount of time that he can maintain his oxygen saturation without the machine is steadily increasing. This is a good sign that his overall pulmonary function is improving. If we can get the oxygen setting down to the 20-30 range, we will probably move off of the BiPAP and back to the cannula. Never thought I would be excited about putting the cannula back on.

I read the manual for the BiPAP machine today. Pretty interesting stuff. I figured out how to refine some of the diagnostic settings and I inverted the display to a much cooler white-on-black setting. I'm sure it annoys the respiratory therapists to no end, and that really breaks my heart.

Ryan has had a fever in the early morning for the past two days. It's been pretty low (~38) and it goes away on its own in an hour or two. We're not sure what to make of these fevers. Seems like we move in and out of these periods of fever with no real explanation for why they occur. The most obvious cause would be infection, but we haven't seen anything stand out in his labs/cultures. It's also possible that it's a drug-induced fever. Ryan is on like 25-30 medications right now, so who knows what kind of interactions we will see. My pet theory is that one of the colony stimulating factors that Ryan is on, GMCSF, is the source of the fever. Another explanation is that transient weirdness in Ryan's brain is leading to temperature regulation issues. Cheryl did some poking around on the Internet and found that this is not unheard of in metabolic patients. The main reason why it's important to us is that every time Ryan has a fever, we see a new wave of antibiotics, antifungals, and antivirals. The docs have a lot of experience with this sort of thing and the don't want to let an infection get out of control. On the flip side, these drugs are super nasty. The disastrous consequences of prior drug effects/interactions have been well documented in this blog. That's the toughest part of this whole process. Every decision is a "damned if you do, damned if you don't" deal.

D+68: Moving in the right direction

WBC 3.1
ANC 2728

Another day, another couple of steps forward. While things are still really weird and scary right now, the fact that we are seeing some measure of improvement is comforting. The odds of Ryan having a runaway infection (bacterial, viral, or fungal) decline with each passing day.

Ryan had a chest X-Ray this morning. Dr. Graham said that his right lung has improved and the left (site of the pneumonia) is more or less the same. That being said, both lungs have improved quite a bit since Ryan's CT last week. The BiPAP (pronounced: bye-pap) machine that Ryan is on seems to be working wonders. In a nutshell, the BiPAP helps him breathe by increasing the pressure in Ryan's lungs. Increased pressure causes them to "open up" more, similar to what we do when we yawn and take a deep breath in the morning. Ignoring the detailed mechanics (which I will probably cover in another entry), the BiPAP is improving Ryan's pulmonary function -- 'nuff said. One of the things that sucks about the BiPAP is the stupid Top Gun mask and all the associated tubing. What a pain! As grateful as I am to have the BiPAP around, I can't wait to get rid of it.

In addition to the BiPAP, we reposition Ryan constantly to try and encourage the crap in his lungs to move around (increasing the likelihood that it will be expelled). We use to limit Ryan to his back and sides, recognizing the difficulty he might have laying prone with all of the tubes sticking out of his chest. As it turns out, laying him on his stomach is the best thing we can do to help Ryan. So ... turning the clock back about three years, Ryan is getting "tummy time" again. Rest assured that he finds it just as distasteful as he did the first time around. One of the sad side effects of laying him prone is that he accumulates a ton of fluid in his face -- most of it in his eyes. The increased level of steroids leads to a significant amount of fluid retention, so we just have to live with it for now. If you're searching for a mental picture, think Rocky after 12 rounds with Apollo Creed.

Bad News

We haven't kept up with the weblog the past few days because things have taken a rapid turn for the worse. I arrived here Monday evening, and all seemed fairly well. Ryan wasn't doing so well from an oxygen saturation standpoint, but there was nothing completely alarming about that. Late Monday night/early Tuesday morning, Ryan desaturated to the point where he needed to be put on a breathing mask that is two steps above (below?) the cannula. This was enough of a crisis to call Dr. Andreansky in at 3:45 a.m.

An echocardiogram the next morning showed fluid around Ryan's heart -- something we have seen before, but this time, there was much more -- so much that the cardiologist wanted to do an emergency procedure to drain it. For reasons too lengthy to discuss right now, we chose another less drastic option, which is the use of a continuous diuretic to attempt to slowly decrease the fluid in Ryan's body. The really bad news is that sfter the echocardiogram, Ryan had a CT scan which showed that he now has pneumonia. The chances of treating this pneumonia are not very good at this point. The doctors cannot tell whether it's bacterial, viral, or fungal pneumonia because none of the cultures are coming back positive. This means that they have to use multiple drugs for each type of pneumonia, many of which are nephrotoxic. It also indicates that some of the antivirals, antibiotics, and antifungals that Ryan already had been taking weren't working.

We have been told that Ryan is extremely sick at this point and may not make it, so Keith and I are both in the hospital now. For those of you who have been sending good thoughts/vibes, praying, or just wishing Ryan good luck, please crank it up because he needs you now more than ever.

D+61 [postscript]

Couple of other things I forgot to mention.

First, Ryan had another CT today. Dr. A wanted to take another peek under the hood since Ryan's fever is hanging around with no known cause. Based on our experience with the last fever, the docs will keep searching for the source of the fever until (a) they find something or (b) it goes away on its own. There's not much of a downside from our point-of-view. According to Dr. A, the radiation is a little bit more than you would get on a transatlantic flight. The real concern for me is that we actually have to leave our safe little room and venture out into the world of filthy people. In the end, the CT came back unchanged from the last one. Kidneys and liver are a little enlarged, which is expected with the meds that Ryan is on. Ryan's bladder, under constant assault from the evil BK virus, is looking much better. Dr. A said it looked nearly normal, with no visible clots. Ryan's sinuses looked much better as well.

In a more exciting development, our night nurse Allison managed to bang her forehead on a desk while practicing her winking skills. Not much of a winker myself, I didn't realize that such a high degree of total body control was necessary to execute a wink. We may have to look into getting Allison a safety helmet to protect her head until she gets a bit more practice.

D+53: Down, down, down ...

WBC 0.6
ANC 264

You don't need me to tell you that these counts stink. With an ANC of 264, Ryan is categorized as neutropenic again. Even though the doctors warned us that the counts would continue to drop, it's so hard to watch it happen. We watched with great anxiety as Ryan built his little wall of immunity brick by brick for 47 days, only to see it knocked down with a sledgehammer over the last five. At some point we're going to hit the bottom and bounce back, I just hope it happens soon.

Aside from the counts fiasco, Ryan is doing quite well. He still requires the occasional blast of oxygen, but he's able to maintain reasonable saturation (90-93) without it for extended periods of time. His rash is definitely looking better. It still covers roughly the same area, but the intensity of the rash has gone down significantly. He's opening his eyes more. He is also maintaining his weight without the use of diuretics -- a good sign that his kidneys are functioning normally.

D+52: Cleaning house

WBC 1.3
ANC 884

Don't get me started on the counts.

We dropped a bunch of meds today. Gancyclovir is out of here (yahoo!). So is another drug called CellCept, which helps fight GVHD but also can stifle counts. I'm glad to see both of these drugs go (esp. the Gancyclovir), but there's a lingering stink cloud. The bone marrow suppression associated with both drugs can hang around for 1-2 weeks after you stop! The other drug we dropped is called Cydofovir and it is pretty toxic to Ryan's kidneys. Dropping the Cydofovir gives us a bit more wiggle room with the Cyclosporine and the occasional dose of a diuretic like Lasix.

In other news, Ryan's GVHD rash is definitely getting better. In fact, the gut-related symptoms of GVHD are better as well. We still have the occasional green explosion of poop (hey, it's been like three days since I talked about it), but it's much better overall. The GVHD is taking its sweet old time going away, but go away it must.

BTW, our night nurse Allison is slowly restoring our faith in humanity. She really goes the extra mile to help Cheryl and me (note: not 'I') out during the night. Her latest idea is to reposition Ryan's pulse/saturation monitor right in front of the window to the room. That way, she can monitor Ryan while we sleep and deliver any necessary pain/agitation meds as the need arises. It seems like a little thing in theory, but it is absolutely huge in practice. Sleep is a valuable commodity in this place, and it's real tough to get it when you are worried about Ryan all night long. Thanks, Allison -- you rock!

D+51: Cannula strikes back

WBC 1.8
ANC 1332

Ryan's weight hit an all-time high today of 19.3 kg. All along, the docs have told us that Ryan's weight, taken in isolation, is not really a big deal. What can be a big deal, however, is weight-related complications like fluid in the heart and lungs (I know that's like the 30th time I've said that). Well, it looks like the weight is starting to introduce some complications. We started up the oxygen again because Ryan's oxygen saturation was dipping below 90 consistently. The nasal cannula is not back, just this tube called a blow-by that sits near Ryan to increase the oxygen content in the air around his mouth and nose. Of course, the blow-by preceded the cannula before, so we're really freaked that we are headed in that direction again. Perhaps I talked crap about the cannula a bit too soon.

To provide some relief on the fluid retention front, Dr. A. prescribed some Lasix for Ryan today. Lasix is basically just a diuretic which causes Ryan's body to purge excess liquid through lots and lots of urine. Of course, there is always a downside to any treatment we consider, and the Lasix is no exception. Diuretics are rough on the kidneys and we are still in the 0.5 range with Ryan's creatinine. Alas, I fear that we don't have much choice at this point. We really need to purge his system of the extra fluid. We went with a one-time dose of the Lasix today and we will monitor his labs to see how toxic it is.

Counts continue to drop. Cheryl and I are really starting to worry (surprise, surprise) about how far this is going to go. Currently, we do not have a positive impression of Gancyclovir. I hope that we can get off of it soon.

BTW, Ryan digs his chair like a ditch. We are going to put him in it every day to provide some laying in the bed all day.

D+50: Buy one get one free

WBC 2.9
ANC 2378

If you pay close attention to Ryan's counts, you probably have noticed that there have been three consecutive days of decline in his WBC and ANC. Looks like one of the main side effects of one of Ryan's antiviral meds is starting to kick in. Gancyclovir helps prevent against reactivation of a particular virus known as CMV (Cytomegalovirus) . CMV is bad news for a number of reasons, so it's definitely a good idea to try and keep it at bay. Now to the side effect --- bone marrow suppression. Yuk! As longs as we are on the Gancyclovir (and apparently for 1-2 weeks after) we can expect Ryan's counts to get hammered. I swear that every decision made here is of the "which leg do I shoot" variety.

To make matters worse, Ryan received a "bonus" dose of Gancyclovir today. Normally, Ryan gets it every other day, but the nurse made a mistake and gave it to him today. I didn't find out about it until the night shift and I was obviously really freaked out (see above paragraph). Apparently, the extra dose is not toxic or anything and shouldn't have a significant negative impact on Ryan. Be that as it may, I would prefer Ryan to get the correct dosage of medication (go figure). The weird thing is that the nurse that gave Ryan the extra dose is one of the most careful nurses on the floor. She is constantly making lists and double-checking things to make sure it all checks out. Given enough time and opportunity, I guess anyone here can make the same mistake.

Ryan's weight is up to 18.9 kg. This is not because he's eating steaks and ice cream all day long. Rather, it indicates that he is retaining a good portion of the fluid that he's getting on a daily basis. The fluid collects in all sorts of places -- some benign, some not so benign. Examples of the latter variety would include lungs and heart. We are keeping a close eye on Ryan's blood pressure and respiration to make sure that things don't get out of control (again).

The doctor ordered an optometry consult to check out Ryan's potential conjunctival GVHD. To make a long story short: everything checked out just fine. No GVHD, no other issues. I really hate to see them pull Ryan's eyes open, and I know that Ryan hates to have it happen. You just have to chalk it up to necessarily evil. Easy for me to say, of course.

Finally, Ryan received a cool new chair from the physical therapy department here. It's called a Tumble Form and it's basically like a big, soft chair that's molded to support your body in lots of different areas. Ryan has been spending way too much time in the bed, but he can't exactly sit upright in a normal chair in his current condition. The TF should provide enough support to let him sit comfortably while maintaining good posture. We'll give it a whirl tomorrow to see what Ryan thinks of it.

D+49: Sodium, Potassim, and Albumin ... Oh my!

WBC : 4.1
ANC : 3198

Our nurse this evening made a big mistake. She printed out the results of Ryan's lab tests for the day (comprehensive metabolic panel, CBC, etc.) for me to review. It was a truly nice gesture, since she knows how much we worry about Ryan's lab results every day. She obviously underestimated the depths of my mental dysfunction. Giving a report full of 30+ lab results to someone as neurotic and anxious as yours truly is roughly equivalent to handing a book of matches and a 40-gallon drum of gasoline to a pyromaniac. Oh, the glorious amount of data points and nearly unlimited potential for micromanaging the medical staff!

• Bilirubin is up .1 today? What are we doing about that?!
• What do you make of glucose at 119? The report indicates that is slightly abnormal.
• Alk Phosphatase hasn't been reported yet. Is something wrong? When can we expect that to come in? (Never mind that this lab has always been normal and I have no idea what it means -- at least until I Googled it. hehe)

Speaking of lab results, our focus has changed away from Ryan's WBC and ANC. If you care to remember, these levels indicate the state of Ryan's immune system. They seem to be climbing nicely, so it's time to find something else to worry about. We need look no further than Ryan's kidney function. BUN and creatinine are the standard tests to see how your kidneys are performing. Since healthy kidney function is absolutely essential to proper treatment, we are keeping a really close eye on it.

Steering things back toward Ryan (and away from his crazy parents) .... Ryan kept his eyes open for 20+ minutes tonight. He wasn't exactly in a conversational mood, but it really makes me feel good to see him like that. I guess it just offers a hint of the Ryan that entered this process. We know that he's still in there, but all these damn drugs and viruses have him in turtle-in-shell mode. Ryan is one tough kid.

Administrative Note

Just a quick heads up on the "catch-up" protocol we have been using. As I'm sure you have all noticed, we can fall behind in our daily entries from time to time. Luckily, the blog admin interface provides us with a handy cheat mechanism to back-date blog entries. We use this to keep the chronology intact. For example, I missed the last four days of entries. Instead of entering four blogs on Thursday, I will back-date my entries to keep the dates straight.

Just wanted to point that out for folks who check the site regularly, only to find that entries magically appear from previous days.

BTW, the date of a post is right above the title. The author (cheryl, keith, etc.) is part of the timestamp at the end of the entry.

D+47 & D+48: Cannula, schmannula

Day 46:

WBC 4.2
ANC 3402

Day 47:

WBC 6.9*
ANC 5796*

It's been a pretty tame weekend. No major emergencies. No great news. No bad news. Ryan's counts continue to grow, which makes dealing with everything else a bit easier. The * next to Sunday's counts indicates that I'm a bit skeptical. I believe that they drew the labs on Sunday right after he got GCSF (Growth Colony Stimulating Factory), which tends to produce big jumps in the white count. Nevertheless, we are still on an upward trend.

As Cheryl mentioned in a previous entry, Ryan's renal function is returning to normal and we are starting the cyclosporine up again. It's a real tightrope walk, because his creatinine has been hanging out in the 0.5 - 0.6 range. If memory serves, we got rid of the cyclosporine when we reached 0.6 last time around. Our current situation is a bit different (at least according to the docs), because Ryan's heart and lungs are in good shape. That means we can load him up with fluids to assist his kidneys without having to worry about pneumonia and fluid around his heart (at least, not yet). In related news, Ryan's nasal cannula is toast. I ripped that thing off his face the first night I got here. His oxygen saturation is consistently in the mid-90's, so he's kicking butt on that front.

We finally reached the 'therapeutic range' of cyclosporine on Sunday evening. The hope is that the GVHD will begin to recede as the cyclosporine does its dirty work. Recall that Ryan has the skin and gut forms of GVHD. They are both annoying (rash and diarrhea respectively), but it's really important to keep the gut form in check. It appears as though we are seeing some early results -- Ryan's fever is down and his diarrhea is becoming less frequent and there's less of it. In a new twist, Ryan may have yet another form of GVHD affecting his conjunctiva (conjunctivitis = pink eye). Poor kid. We're hoping that the cyclosporine will work its magic quickly!

Speaking of Ryan's eyes, he actually opened them for the first time in about two weeks on Sunday night. How bad do you have to feel not to open your eyes for that long? I think that we tend to lose some perspective in dealing with Ryan on a day-to-day basis. If each day is a little worse than the next, you don't really notice. Next thing you know, it's been a fortnight since you looked your kid in the eye. This is not exactly a happy place. Anyway, no need to ruin a good thing with a bunch of whining. Ryan does seem like he's getting better and being able to make eye contact with him is a really good thing. Natives rejoice!

Cheryl and I are switching roles this week. I will remain in Tucson with Ryan while Cheryl returns to Phoenix with Regan. My hope is that Cheryl will be able to relax just a little bit at some point and find some peace. Not very likely, but you never know ...