Thursday, November 30, 2006

11/30 It’s Always Something

Sorry for not posting a post yesterday. I’ve been throwing together an information site about the big party next weekend. I’ll post a link later today. I’ve been getting some concerned email about why no post yesterday, so here’s the dope.

Yesterday and today Josie has been doing well and not so well. Yesterday afternoon she did need another blood transfusion. There’s been no real active visible bleeding, but her hemoglobin was lower again and her heart rate was up as well as temperature, so they decided it would be a good idea for some more blood. In it went and the heart rate stayed high and the temperature stayed higher around 101° all day yesterday.

She’s been drinking like crazy. I can’t tell if it’s a mental thing or if she’s really thirsty, but it seems as though she’s trying to make up for all those weeks without drinking. She drank almost five liters (!) of water yesterday. Talk about a fish -- jeepers. Of course the water just flows right back out the NG tube so it’s no biggie. The docs were questioning if the water was stimulating gastric juice production but it doesn’t seem to be. You should see it. She’ll get a full bottle of water and totally slam the whole thing without it leaving her lips. As soon as it’s slurped dry she’s asking for more in a way that seems she’s dying of thirst.

Last night her fever spiked at 102.8° measured in her arm pit, so she was HOT. Her sleeping heart rate was in the 190s much of the night (that’s not 90’s…that’s one hundred ninety beats per minute…my calculator says that’s over three beats per second!). This is a little concerning but not emergent. One of Josie’s blood cultures came back positive with Group D Enterococcus bacteria in her blood. So now she’s septic again – nice. They fired up the Vancomycin antibiotic for about the millionth time and hopefully that’ll take care of it like before.

Visible Blood VesselsHer pain has become a source of debate between the different docs and nurses. She was on some serious volumes of Fentanyl…enough to drop any 300 pound lineman in their tracks. The pain team doesn’t really like that because of the dependency. It’s clear…Josie is addicted to narcotics. Go figure…I’ve always dreaded the future worry about my children getting into drugs later in life…I never thought in my wildest nightmares that my three-year-old would become the narcotic junkie of the family! No worries though…it’s a physical dependency and one we’ll be able to take care of while here at the hospital, hence the Pain Team’s concern. They started her on Ketamine, another pain killer, and started the weaning process of the Fentanyl. Of course her fever spiked and heart rate went way up, but they can’t say for certain if it had anything to do with the pain medicine. Anyway, they dropped the Fentanyl drip by about 15% and halved her hits. She’s been in a fair amount of discomfort today, again the source is in question, so they bumped up the Fentanyl hits and added Ativan for nausea (it’s also used for anxiety – similar to Valium) and that whole cocktail just knocked her out this afternoon.

Her hemoglobin was down again today to 9.9 gm/dL even after fresh blood yesterday. Another interesting observation without definitive cause is that the veins on her trunk are very pronounced today for some reason, much more than before. Could be extra fluid volume in the blood (which might help explain the lower hemoglobin levels), or it could mean there’s a clot somewhere, or the liver flow is bad…both of which were ruled out with ultrasound. Another mystery, and we’ll watch it and ask more questions.

More to come

Tuesday, November 28, 2006

11/28 Are We Better?

It's a shock to us, but Josie did not have a blood transfusion today. She's held her own and has maintained a low but acceptable hemoglobin level. We'll see just how good her bone marrow really is (Josie has a huge reputation of having the healthiest bone marrow of us all...makes these Vikings marrow pale in comparison). Her red blood cells are going somewhere, whether they're being deposited somewhere internal, or just bleeding out through her drains, or her body may be destroying the cells naturally. We just don't know. They'll be keeping a close watch on that hemoglobin number and if/when it drops it'll be Transfusion City.

So how's Josie doing? This is a very good question, and one which we bantered about with everyone today. For some reason everyone was asking; “…is Josie better today than she was a month ago?” The most popular answer was "...I don't really know..." followed closely by "...Maybe..." and trailing way back was "...I think there's been progress...". That tells me that we're not really worse I guess. Fact is that, as Dr. Vehe put it, Josie's belly is a black box, one which we wish had a zipper. We are all speculating on -- what's causing the pain? What's causing the bleeding? What's happening with the eroding intestine? Why is the healing so slow? We just don't know and no one is pretending to know, thank goodness. They're all working really hard to figure it out and provide Josie the best opportunity to heal.

We finally heard back from pathology (FYI…if you’re looking for some quick answers about anything, avoid the pathology lab – especially on a four-day holiday weekend) and the results are that Josie has NOT been sloughing the insides of her intestines as feared. This is good news for sure. But not all is well inside. The poops are still bloody and funky, and a real brow frowner.

Josie’s Liver enzyme level (ALT) is up a little, her pancreatic lipase enzyme level has dropped again (good news). Her bilirubin (byproduct of red blood cell breakdown) had jumped a little too which is certainly a clue to but not revealing what's happening. More patience is the key...but at the same time I just hope we don't get further down the road and with perfect hindsight say to one another "...I wish we knew that was happening", or the classic " was so obvious, why didn't we see it before?". Time will certainly reveal the truth.

They're not THAT bigThe Big Guys from the Vikings were back in the ICU for another visit with Josie today. Jason Whittle (#65 9th year guard, 6’4” 305lb) and Mike Rosenthal (#75 4th year tackle, 6’7” 315lb) stopped in. We talked a little trash, told them I couldn’t make the game this weekend and they understood. These hulking guys came in with their little rubber gloves on (Mike had to wear a mask…he must have had a lingering cold or something) and were as friendly as two giants could be, and of course Josie tried her best to ignore them…just like last time. No Barney cracks this visit though. It’s great these guys make the time to visit folks in the hospital.

Josie's fourth birthday is just four days off on December 2nd. We've got plans for a nice little birthday bash here in the hospital and we hope she'll be well enough to enjoy that.

Also, don't forget about the big fun-filled get-together/dinner/family night next Saturday evening. Look for lots of details coming in the next day or two (can anyone say 'bake-sale'?) It’s gonna be tons of fun with big giant jumpy things for the kids, silent auction (we’re looking for some great item suggestions), maybe a raffle, entertainment, and be careful about those white shirts and sweaters with all that spaghetti flying around. There might even be a surprise appearance by a certain loveable and very popular rodent. Can’t wait to see y’all there.

Monday, November 27, 2006

11/27 Some Changes

Today started like many of the past 10 days…feeling bad, nauseas, feeling bad, retching, feeling bad, blood in the NG nose tube, feeling bad, bile in the JP drain, feeling bad, not much energy, feeling bad, higher temperature, and she didn’t feel very good either.

The good news was that the inflammation markers (CRP and ALT) were down as was the lipase (pancreatic enzyme for fat) down to 500 or so. Her hemoglobin was down again below 10 gm/dL so the bleeding, or at least loss of red blood cells, continues – and that’s part of the bad news.

The other bad news is that her bleeding seems to have increased this evening. At around 7:00PM she started bleeding a fair amount from the JP surgical drain. The drain was bright red, but what was more concerning is that the entry point of the tube into the skin was oozing blood too indicating heavier internal bleeding. We’ll have to keep a close eye on that and see what tonight and tomorrow brings. Another blood transfusion is a probability overnight. We’ll keep you posted.

11/26 Still The Same, Pretty Much

Watchin' a Little TubeAnother day much like the last with not much to report. Still in the ICU. Still feeling bad. Still retching and vomiting. Still a low-grade fever. Still pancreatitis. Still holding the security Dixie cup to her face. Still hurting in her tummy. Still no answers. Still need to be patient.

Henry and Deb spent most of the day with Josie today. They watched movies between pain and vomiting episodes. Josie was able to have a nap too. Tonight she was feeling well enough to watch some TV, but not well enough to color and certainly not weel enough to stand or get any more active.

Her hemoglobin is stable even though she’s still bleeding from the NG tube and much less so form the JP drain tube. Pancreatic lipase, liver ALT, inflammation CRP are all a little (marginally) lower (better) today. Her Dermatomyositis clinical symptoms are not showing any new activity and seem to be staying pretty calm. Nothing much else to report.

Saturday, November 25, 2006

11/25 Stable And Still Feeling Bad

We've strung together several days now of relatively uneventful ICU existence. Not so bad one would say...including us, but the stability isn't the kind of stable anyone would want for their enemy let alone their own child. Josie's still in a lot of pain. She's on a lot of pain killers. She's been nauseous for days now. She's not wanted to do much of anything besides an occasional movie, or read a book. Mostly it's lying in her bed with her Dixie cup to her face looking sick, feeling sick, and making us sick that we aren't able to make her better.

She had another abdominal CT scan today which was rather unremarkable. The good news is that her gall bladder has decreased in size a bit, and the tennis-ball sized hematoma has completely resolved, but other than that there was nothing. Frustrating for sure because we're looking for a reason for her feeling so bad. I suppose one could argue that one wouldn't really feel like doing cartwheels with three tubes coming from one's belly and another coming from one's nose...and that a good sized section of one's duodenum is missing...and that one's been suffering from pancreatitis for months now. I guess Josie has a couple good reasons to not feel so good. Still...that lingering question...Why is she not feeling or seeming to be getting any better?? Doc's don't have the answer, and we surely don't have the answer.

No word yet on the Charton Leyden test from yesterday (by the way, thanks for all the responses to my question from yesterday...I think I need some web lessons...anyone have a copy of 'Google for Dummies' you could send?).

Josie's temperature has been between 100° and 101.5° all day. Her hemoglobin has dropped to 10.1 again (where's the blood going?). Her lipase pancreatic enzyme level has risen to almost 1000 indicating more pancreatitis, ALT (liver enzyme indicating liver damage) is up, CRP (general inflammation marker) is up again for a second consecutive day. Her drainage from the JP surgical drain has been blood-free but flowing at a good clip. Her nasogastro (NG) tube has been draining intermittent blood. She's been retching regularly today too.

This is just not fun at all for our little Josie.

Friday, November 24, 2006

11/24 Patience

Today marks a month since Josie been back here to the Intensive Care Unit.


Still nothing from pathology on all that stuff coming out of her from the other day. They sent orders for another test to look for Charton Leyden crystals (yeah, no one else has heard of them either…in fact if anyone out there knows what they are, drop me a line.) Her hemoglobin had dropped to 9.1 at 3:00AM so, as promised, they ordered up some fresh red blood cells for another infusion. That brought up the hemoglobin level to 11.8 this afternoon. It didn’t really help out her higher heart rate though. It’s still hovering around 170 bpm and lowers to near 150 bpm from time to time.

Dr. Saltzman, Josie’s surgeon, was in and we discussed the healing and what’s happening and what’s taking so long and what the plan is….you know…all the questions from genuinely exasperated parents. He gently and compassionately reiterated that we must be patient and allow Josie to heal. Josie heals at a remarkably slow rate, and because of this he is reluctant, no, adamant that she is a bad candidate for surgery right now. The Whipple surgery is a tricky and very involved on. There will be many things removed and lots of things reconnected. With her recent history of poor GI healing and complications, it’s likely that a Whipple would be just as challenging to recover from for her. This is bad because the Whipple is pretty much a one shot deal and if it goes bad…well…

Healing. Healing has now taken on a much greater meaning for us. I’m used to getting a sore calf muscle or Achilles tendonitis or a sore throat or a touch of the flu and waiting for whatever the to heal, which takes some time, certainly, but not forever – which is about how long it’s been taking Josie to heal from just about everything. After this experience and after watching all the other families here in the ICU, healing is something to cherish for sure and not to be taken for granted.

Today was much the same as yesterday – feeling crummy, high heart rate, tummy pain.


Thursday, November 23, 2006

11/23 Happy Thanksgiving

Feeling Bad - No appetite For TurkeyWhat a lovely day here in Minneapolis. The weather was certainly not frightful…in fact it had a little spring-like feel rather than a start-of-winter feel. We all hope everyone had to loosen the belt just one notch this afternoon, and that your stress levels were low wherever you were today. Who’s up for some shopping tomorrow at 5:00AM?

Josie’s day wasn’t what we would call great…more like flat, and sleepy, and a bit more painful than anyone would want. She really didn’t want to do much of anything today. She was only awake for a few hours and slept uneasily the remainder of the day. Definitely a low-motivation day…she just laid there feeling bad. Her heart rate has been elevated at about 170 bpm all day. She’s had many painful episodes today accompanied by retching and just a lot of discomfort. Not fun at all.

Most of Josie’s labs were OK today. Her pancreatic lipase enzyme and liver enzyme levels were about the same – elevated, while most everything else seemed pretty good. Her painkiller medicine (Fentanyl) drip dose was increased to 7 mics per kilo per hour and her as-needed boluses were increased to 100 micrograms per hit. She took 14 extra hits since midnight so that’s why they increased the dosage...she just hurts. We’re still in a manage-the-pain-as-best-we-can-while-we-wait-for-her-innards-to-heal mode, which is frankly getting a little taxing. The patience game is a tough one to win and all the families here in the ICU would agree. Everyone wants answers and improvement and less pain and success. And we’re ever so slowly creeping and scratching and clawing our way toward the finish line which is out of sight and seems so far off at the moment.

We had a very nice meal prepared for us by some friends at home this Thanksgiving Day while Josie held down the fort with the ICU team. The meal was great and really hit the spot. We all then trucked on down to the hospital for a visit with Josie afterwards. Josie was getting a little over-stimulated, or just plain old ticked off at her siblings, shooting her heart rate upward of 190 bpm. That didn’t last too long though so it was a nice visit. The gang enjoyed seeing Josie again – they haven’t seen much of her this past month since being in the ICU. Hopefully we’ll get that turned around in the coming weeks.

Of course, that gets us wondering when are we going to get out of here. The take-it-as-it-comes approach has been working well (well...not that well) and will continue to work well (again...not that well) with us…I think that’s the only way we can think about it. Every now and again (and sometimes way more often) we think about the big picture and what’s going to happen later…where is this train headed? Six months, one year, ten years…all will come, and what will it be like? Who will be around and who won’t? What will we be doing and celebrating? What challenges do we have ahead? What successes will we cheer for? How much more pain will this girl need to endure? When’s it all going to get better? Or not? -- And isn’t life great that way? No one knows, and no one’s supposed to know. We all want answers - from the mundane questions to the bigger picture, to the grand scheme. Funny – we’re not allowed the answers, so it’s gotta be a Take-it-as-it-comes approach whether we like it or not.

Right now Josie is as alert as she’s been all day and she’s watching a little Dora (my fav). Her temperature is 101.5° with a heart rate of 178 bpm. Her hemoglobin this afternoon was 10.1 so the docs decided that if it’s any lower tonight that she’ll get another blood transfusion. They’re thinking that a lower blood volume might be a contributing factor of her high heart rate. Her drainage from the NG and JP tubes has been the right color with some - but very little - active visible bleeding. Because she’s felt so crummy she’s not wanted to do much of anything during her awake hours today…she just lays there and holds the cup to her face. That cup is telling everyone that she feels plain terrible.

On we go to another day for some more adventurous excursions into the unknown jungle within which we seem to be lost (don’t tell the doctors that though).

Wednesday, November 22, 2006

11/22 More Waiting And More Pain

We had some good discussion today with the GI team about Josie and her condition. We're all in agreement that her condition is stable (sort of) but the level to which her insides are healing is likely pretty low. The GI team and the surgical team want the steroids reduced. Being immunosuppressive, steroids interfere and hinder good healing of the body. This is making it hard for Josie to generate a nice fistula for drainage. Of course she's on the steroids to suppress her dermatomyositis; hence we're in a bit of a tight spot, so balance will be the name of the game as we tiptoe along the edge of disease control and healing. A dermatomyositis flare is the last thing we need right about now. GI is to discuss with Dr. Vehe (Josie’s Rheumatologist) on the proper decrease of steroids moving forward.

Coloring, but still hurtingGI examined Josie’s NG fluid output, JP drainage fluid output, and her poop. They wanted it all sent to pathology for testing to see exactly what the little fleshy floaties are in the drainage. They hesitantly agreed that the globules could be sloughing tissue from the bowel…but the exact tissue source is unknown. We discussed the big bleed from last week and agreed that it was likely an exposed vessel of her duodenum which has probably eroded further in the last month. All that blood inside the gut and peritoneal space can cause all kinds of different things to happen and what we’re seeing could just be the end of all that stuff finally moving its way through. Unfortunately the pathology lab processing is not as fast as the blood lab so it’ll take a few days to get definitive results. The two questions we need answered are: is that stuff tissue, and from where did it originate.

GI though it prudent to perform another ultrasound to take a peek at her liver and gall bladder to check for blood flow and any abnormalities. The results were similar to before with nothing remarkable other than a little sludge in the gall bladder.

Josie’s labs look about the same as yesterday with things nice and stable. Her heart rate has been elevated much of today averaging about 160bpm. Her temperature has been normal. The big problem has been her pain level which has stayed high most of the day. When she hurts it triggers nausea and retching…total discomfort, and it’s hard to watch since there’s nothing we can do about it. I know how I feel when I’ve got the dry heaves…it hurts and is so unbelievably unpleasant – now add a lot of abdominal pain on top of it, weakness at a level most of us have never experienced…I just can’t imagine how she can cope with it the way she has and continues to.

So we had lots of questions for the surgeons and for the GI team but as with Josie’s deal all along there are far more questions than answers, and today was no different. The thing we all agreed on was that her condition has not improved too much in the last four weeks since the big surgery…not nearly as well as we would like.

Tomorrow is Thanksgiving, and we all have so much to be thankful for. We all hope you have a wonderful day with your friends and families and wish you all the best.

11/21 Can't Tell If It Was A Good Day Or Not

We're not quite sure what to think about today. Everything seemed to go just fine...the blood work looked pretty good -- stable hemoglobin, decreased pancreatic and liver enzyme levels, normal white blood cell count. Her heart rate was in the mid 120s, temperature is normal, she looks better generally speaking. the JP surgical drain has had no blood, just green bile with short tinges of slight bleeding... (which normally would not be a good thing for the average person 3 1/2 weeks post-op for abdominal surgery...but for Josie this is good). All good for why are we uneasy about all this?

Well, since Josie's last surgery she has really felt pretty bad generally speaking. There have been some times when she's felt good...good enough to be up and ready to do stuff. She'll want to color every now and again but it doesn't last too long (sometimes but not too often). Today was a good example. She was up coloring for just a short time but her tummy was hurting most of the day...pretty bad. She needed further hits of pain killers. They decided that because she was requiring basically hourly bumps of the narcotic that they would increase the constant drip and then provide bumps as needed – hopefully fewer. Now she's needed the hits about every two hours for the rest of the day. She's been retching too which usually coincides with the pain. All this pain, even though the numbers look pretty good.

Josie's NG tube from her nose has been bloody much of today – not super bloody, but the liquid that come out has fresh blood within it.. She's been taking water every so often which just gets sucked right back up the NG tube by design and this helps to irrigate the inside of the tummy.

The JP surgical drain today got more focus for a couple reasons. First, it was flowing green most of the day. The shocking part was that during a span of about four hours the drain was producing three or four times the normal amount of bilious fluid. At one point it was just pouring out, kind of like an oil well striking green oil. Within the JP drain flow today and over the last few days have been chunks of dark matter and chunks of lighter-colored matter which look a lot like little pieces of tissue. It's difficult to determine exactly what it is but it's becoming a little bit of a concern for us...we'll be asking more about that tomorrow during our team meetings. There was talk that the increased flow might be the gall bladder emptying since it had been so full last week during the ultrasound and CT scan...and this would be a good thing and might explain the rapid flow...but for now it’s all speculation…no one really knows.

Also, and most disturbing today, was within her poop. Poop for Josie is a good thing indicating the bowel is working. Within today’s output it looked like small balls of fleshy-like material mixed in with the old blood passing through remaining in there from Friday's big bleed. The fleshy globules are about 1 millimeter in diameter and look like they really shouldn’t be there considering she's had nothing to eat recently. There had been talk that it might be the inside villi of the bowel sloughing off...but that's just speculation with no confirmation at all. We'll be asking questions of the GI service tomorrow regarding that.

The GI team wants to get Josie’s steroids decreased to aid in the healing and creation of a strong fistula (a new tube which Josie's body is hopefully making to encapsulate the bile flow from the upper bowel leak to the outside of her body. Of course we need to balance those requests with trying to maintain the weak and uncertain control of Josie's underlying dermatomyositis.

So, here we are, things are looking good, but some mysterious things happening what are giving us an anxious, uneasy, restless feeling about what's really going on inside that belly of Josie's. Time will certainly tell, won’t it?

Monday, November 20, 2006

11/20 Another Pretty Good Day

We're starting to string together some good days in a row now. Josie's feeling better today but her tummy is still hurting pretty bad. Her lab work was stable for the most part. Hemoglobin has maintained in the normal range. Her pancreatic enzymes are mixed with the enzyme for starch (amylase) was normal while the enzyme for fat (lipase) went up quite a bit. Her liver enzymes are elevated too. Everything else is looking pretty good considering how bad things were just on Friday.

All the teams were visiting Josie just like every other day. There's still debate at what caused the big bleed the other day. Our Rheumatologist decided that things were going pretty well so no changes in her treatment were ordered. Josie's JP drain bulb produced a positive culture for Group D Enterococcus which is a bacterial infection. They decided to change the JP drain bulb and retest in case the bug was just festering in the bulb rather than inside her peritoneum space (abdomen).

The only thing remarkable today was that her tummy pain was so severe while all her vital signs and labs were all fairly good. Josie needed frequent hits of Fentanyl often they decided to increase her dose to 6 micrograms per Kilogram body weight per hour which doesn't seem like much, but it's quite a bit.

Josie's Party is Taking Shape

The gang of folks got together this evening to talk about the upcoming get-together for Josie on December 9th. We'll put together a separate page with all the details in the coming days along with contact information if you'd want to help out with something. It's going to be a low-key fun time for everyone...can't wait. Stay tuned for more details

Sunday, November 19, 2006

11/19 Good News But Feeling Pretty Bad

Feeling Bad With the Vomit CupLabs are all looking better or stable or normal. Heart rate is lower. Hemoglobin levels are normal and stable. Pancreatic levels are normal to just a little elevated. Josie's nasogastro (NG) tube and JP surgical drain are both draining gastric juice with just a little remnant blood from the other day. The JP surgical drain is producing bile again at the normal rate with no visible blood. All good news -- and it's funny (not funny funny, but funny) that bile coming from outside the GI tract is good news...but it sure is. Her Dermatomyositis symptoms are better too. The bad news is that she's nauseous and feels generally crummy. She's also in a fair amount of pain and has been all day. Her tummy hurts on the inside so she's not felt like doing anything at all today besides watching some tube. You can always tell when Josie is feeling barfy because she insists on having the vomit cup nearby. When the cup leaves she generally feels better.

Deb was able to get her in a little wheel wagon and cart her around the floor a little bit. They went and visited the other floor to say Hi to all the nurses we met over there in September and October. Everyone is looking forward to seeing Josie when she arrives back tot he floor sometime soon.

Other than that Josie's day was rather uneventful. We just hope she starts feeling a little better and that tummy pain goes away.

Saturday, November 18, 2006

11/18 Better

Things are way better today. After losing so much blood yesterday Josie needed another transfusion to get things topped off. Josie stayed up too late with me last night...past 2:00AM. After I went home for the night the nurse had to order her to sleep. Josie kept claiming " Mom let's me stay Mom lets me stay up...". For the record, Deb does NOT allow our children to go to bed past 1:00AM let alone 2:00AM. Just kidding - It's 9:00PM in the Adams household most nights. Josie slept in this morning and has had a rather uneventful day feeling so much better than yesterday's near disaster.

Josie's JP surgical drain finally opened up and is flowing again. After two tries with the TPA and multiple flushes, it finally cleared giving a good path for the leaking bile to find its way out. This was a real relief. So here we sit to gain health again, slowly. And for the move to the other that's going to happen any time soon. Maybe we'll talk about it when we get a week's worth of boring days.

Josie’s labs look pretty good today, her heart rate is lower at about 125 to 140, her temperature is normal, her attitude and appearance are good, she watching TV and chatty

It's so nice to just relax. As I write Josie is engrossed in a videotape of Dora the Explorer. At the very same time the Michigan/Ohio State game is on TV. I pleaded with Josie to watch the game, and as usual the little woman won the debate - so here we sit watching the same episode of Dora we've seen at least 46 times in the last month. Who needs the most anticipated football game of the season when you've got viewing number 47 of Dora?

Friday, November 17, 2006

11/17 A Bloody Bad Day

Feeling Bad - Bloody TubesWhat a bad day this was. Things were heading the wrong direction last night and at about 4:00AM this morning they really headed south. Josie started the Big Bleed again. She was bleeding a lot...I mean a LOT out of everything and everywhere. Her duodenal drain, JP surgical drain, Nasogastro (NG) tube were all flowing bright red blood. The openings where all the tubes go into her skin were oozing blood. She was vomiting blood and pooping lots of brilliant red raspberry jam. This was not a good sight – just a big bloody mess. Her hemoglobin level went from 13 gm/dL down to 10 gm/dL in a matter of five hours (that’s a pretty big drop). The Doc's made the call to get more blood into her because she was bleeding it out; she sure needed some to go back in. Throughout this time her resting heart rate shot way up and was hovering about 200 bpm (don't think I’ve ever had my heart rate that high in my whole life). We were getting the feeling we were headed to the Dark Place again.

Josie's normally busy room was exceptionally busy this morning with all the docs checking her out and then conferring out in the hall. The surgeons were saying the blood was a result of her Dermatomyositis (and technically everything is related to that) flaring inside. I at first was thinking that a vessel had been exposed form her duodenum (first part of the small intestine just past the stomach) continuing to erode further from last time. There are several good explanations for where the bleeding was coming from...but the bottom line was this: it would be interesting to know where it was coming from, but what are we gonna do if we find out? The short answer is nothing. The general consensus is that we'd likely cause more harm than good if we go in with another surgery. The surgeons have held tight that unless things are grave and very emergent, they're not going in again...and both Deb and I are completely onboard with them.

Lots of things changed with Josie's care today. She's continuing her antibiotics of Vancomycin, Bactrim. They added Zosyn to the mix today which was stopped last week. She was re-started on Octriatide which helps reduce pancreatic enzymes and constricts blood vessels in the gut as well as reduces gastrointestinal motility. Her feedings were stopped through her gut because with all the bleeding they didn't think there was any benefit. They started up the Protonix again too to ensure the proper stomach acid pH. The ICU Docs have correlated Josie's sinking spells over the last few weeks with her IVIG (Immunoglobulin) treatments wearing off...which was an interesting observation and worth noting. Then Dr. Vehe, our awesome Rheumatologist, came for a visit and we all agreed with his recommendation to unleash a full assault on her Dermatomyositis. Today Josie got a heavy dose of 375mg (30mg/kg) Solu-medrol (steroids), a fresh infusion of IVIG, and a heavy two-week dose of Cytoxan (chemotherapy).

Feeling Better DrawingOne of the most concerning problems of the day was Josie’s JP surgical drain, which stopped draining again. We tried getting it to go and flushed it a bunch of times but whatever is in there blocking it is tough and not cooperating. They injected some TPA (Tissue Plasminogen Activator to dissolve clots) and let it sit for a couple good. So it's still blocked late in the day. The surgeons will address it in the AM. This is concern for me because that drain has a history of consistently producing 10ml of bile with some blood mixed every hour for weeks now. The fluid production has likely not stopped so all that fluid is going somewhere if it's not coming out of the drain. We'll tackle this problem tomorrow.

Later in the day her temperature spiked up to 102.5° and she was feeling just awful with a high heart rate but a little lower at 180bpm. She continued to vomit and retch for a few hours on and off. She then slept for quite a while and with less bleeding. Her pain throughout the day was managed by hourly 75 microgram Fentanyl bumps on top of her 5 mic’s/Kg/hr constant Fentanyl drip. Her heart rate slowly dropped throughout the night and she began feeling a little better. Now she's sitting up and coloring and feeling the best she's felt all day (don't tell Deb she's still awake at 11:45PM or I might be in big trouble).

An exhausting, wild and unexpectedly stressful day.

Thursday, November 16, 2006

11/16 Ups, Downs, Dogs, and Parties

Things are progressing nicely...well, not really nicely...more like slowly and painfully, but progress just the same...slow but (pretty) sure. It's kind of what I think turning around a cruise ship with a paddle must be like...lots of work, lots of time, slow to change, can hardly notice a change of course, but eventually it turns and heads in the right direction ever so slowly. Josie's morning was not so good. She felt tired and sick, a fair amount of pain, some vomiting, and generally feeling bad. All this while she looked generally good. She seemed a little less distended in the belly, her skin looked good, her labs looked pretty good too, but she felt crummy. Josie's pain medicine was changed yesterday. They kept the Fentanyl drip going but moved to a different pain medicine called Dilaudid for the extra bumps when the pain gets bad. This was the first step toward transitioning off the Fentanyl. From observing things, Josie's sick feeling seemed to coincide with the Dilaudid switch. We tried moving back to the Fentanyl and she perked right up. A note will be going into her chart that Dilaudid is unwelcome.

A Visit From Josie's Dog, Birkie -- Nice Tattoo on the forehead!We had a special guest visitor this afternoon. Josie's big yellow dog 'Birkie' came to say hello. Imagine the stares we got when the big guy was trotting down the hallway of the ICU panting like he was in the desert on a 120° day -- he indeed was an attention draw. Josie heard him coming (as did the rest of the unit) and when she saw him she perked right up and fixed herself on him, reaching for a scratch behind the ear. Of course Birkie didn't disappoint...he was sniffing all around her and gave her a couple big licks with that big steak of a tongue smothering her face providing a nice Labrador glaze -- Josie sure didn't mind, giggling through the wetness. It was a great visit.

Surgery was by and continue to be encouraged by Josie's slow-but-sure progress. They made very small incremental changes to her feeding plan adding just a little bit to the tube feeding and taking just a little bit away from the IV TPN (total parenteral nutrition) which she's been on for so long now. For the first time in a coon’s age they talked about sending her back to the other floor. If all goes well over the weekend she might be heading back early next week.

Josie was up and playing with the gals from Physical Therapy for a while and having a great time. But it wasn't long after that she started feeling pretty bad again. The rest of the afternoon and evening she was either sleeping restlessly or awake retching and vomiting and feeling crummy. Her pancreatitis levels are about the same as yesterday...not so high... so we're not quite sure what to make of it. The deal has always been ups and downs with hopefully more ups than downs with Josie's fight. Lately it's been about 10 steps forward and nine steps back...lots and lots of downs but trending ever so slowly better.

Party Time - Benefit for Josie

A group of ambitious friends have decided that Josie needs a party so they've decided to throw a benefit dinner/event on Josie's behalf on December 9th. We're not exactly sure about all the specifics yet but it sounds like it'll be a fun time for the whole family to get together for a meal and rally around Josie. Set your calendar for Saturday, December 9th at 5:30PM - 8:00PM or so -- come one...come all. The event will be held at Christ Presbyterian Church on 70th Street and Hwy 100 in Edina. It'll be a good time for sure. We'll send out e-invites and set up a volunteer sign-up for those who might want to help out a little. I'll post further details as we get them. Also, if anyone has any great ideas on what we can do please let me know be sending me an email at Stay tuned.

Wednesday, November 15, 2006

11/15 Better Days

July 17, 2004 1½ years oldWe're starting to string together some better days. Last night Josie did really well. She only needed a couple of extra pain-med hits which is big departure from the last few days. She also slept most of the night just fine. This morning she was up and feeling good. She continued to do well bleeding wise. Only trace amounts of blood from her JP surgical drain and NG tube. This is great. Not any of the doctors consider it any more than a coincidence that her bleeding seemed to stop right after the double-dose of blood yesterday...seems pretty not-coincidental. But who cares? She stopped bleeding -- until this afternoon. She again started complaining of tummy pains more a little later in the day. She vomited once and then the NG tube produced a little fresh blood...and then the JP surgical drain did the same. The good news is that it was short-lived and we're back to no visible blood this evening.

Her labs looked pretty good...white blood cell count is normal, CRP coming down, hemoglobin was expectedly way up. Nothing else was really tested. Pancreas levels were dropping last check and they've gone to twice per week testing so no tests today on that. Final result of the gall bladder ultrasound was negative. They saw the sludge hanging in there, but nothing else was abnormal…so we’re good.

Josie was up and sitting, doing crafts for a couple hours today. She's now able to drink as much water as she wants...and was allowed to have a popsicle whenever she wants one. Funny thing though -- she didn't want one...afraid she would get too chilly. We'll get her straightened out tomorrow.

The surgeons said they want her to completely convert to tube feedings through the gut by the week after Thanksgiving and off the TPN IV feedings. She tolerated the full-strength fat-free feeding and they’ll bump up the rate tomorrow to about 30ml per hour (about 2 tablespoons full).

So another good day has come and gone, and we're looking forward to a lot more.

The above picture was from summer two years ago when Josie was just 1½ years old.

11/14 More Good Than Bad (I Think)

Feeling Pretty GoodToday was one of those days which I can't tell if it was good or bad or what.

This morning Josie's hemoglobin level was stable so no blood was needed then. She had a semi-restless night, sleeping much of it but waking about every hour or so complaining of pain, so the nurse would give her another Fentanyl hit and that would take care of it. When she woke up and most of the morning her JP drain was draining thick blood and blood was coming from the NG tube out of her stomach. She was also pooping maroon colored goo...more blood.

The surgeons were in and these guys like to see poop -- it excites them. ...uhh -- hmmm...that sounds kinda's not like that at all. They're encouraged because it means her guts are working nicely and moving things along which is a great thing for Josie considering all the trauma her little guts have gone through these last few weeks. That fact that it's maroon of course means there is fresher blood in there but it's not bright red and she's not losing THAT much blood. The surgeons are not too worried about all the bleeding...they're more focused on the good progress of the whole package and Josie’s definitely in a better place now than we were three weeks ago. Slow steps...some downs and more ups trending northward. They said she would get very sick on her way to health and they were sure right so far.

The surgeons were also focused on her guts becoming healthier so they ordered her feeding to go to full strength from the half-strength fat-free formula. The GI team reluctantly agreed but remains cautious -- understandable. It’s been so long since she's had anything into the gut, it can become too stimulated which could draw water in and cause some serious diarrhea.

Surgery was also thinking much of Josie's blood loss has been in the form of lab draws. Add it all up and that's a lot of blood they take to testing. It makes sense, but I would think we would see more of a linear drop in her hemoglobin over time...but what we've seen is more of a steady and slightly varying level and then a pretty big drop. I think the bleeding comes and goes and sometimes bleeds too much and she needs some more. That happened today... when they checked the hemoglobin again and it had dropped from 9.0 gm/dl down to 8.0 gm/dl. Because of thins they decided to give Josie a double-dose blood infusion. Shortly after the infusion was complete her bleeding slowed and as of this evening the bleeding has stopped from the NG and JP tubes. This is good news, but probably temporary.
Later in the day Josie had an ultrasound to check on her potential cholecystitis (inflammation of the gall bladder) which was first detected on the CT scan from a few days ago. They just wanted to check it out again with an ultrasound...the results are not back yet...but during the ultrasound the tech pointed out sludge within the gall bladder which was really interesting to see on the screen. We'll talk about it with the Doc's tomorrow I'm sure. The inflammation marker in the blood didn't really support the gall bladder condition...and the antibiotics they put her on when they first suspected it would have helped the concern about this is pretty low.

Josie's attitude today has been pretty good. She's regularly needed quite a few bumps for pain, but all in all it was a reasonably good day. Every day we get further from the surgery the better. Things should be healing up nicely throughout her body...but I'm sure the duodenum is still a real mess. Her Dermatomyositis symptoms are not very strong now indicating a calming of the disease...more good news. But that can change can all this stuff with her.

Monday, November 13, 2006

11/13 Feeling Way Better And A Big Step

Feeling BetterThat Vancomycin works wonders. Josie's turn-around started last night and has continued all day long. She's been pretty happy and chatty and coloring and back to her sassy least between the heavier pain episodes. Overnight she needed the extra hits of the Fentanyl just about every hour. This morning the Doc's upped her constant Fentanyl dose to five micrograms per kilogram per hour which is up from four ug/kg/hr....and five times the dose she was on two weeks ago. This has really helped with the pain for the rest of the day. She's definitely building a hefty Fentanyl tolerance, but as long as it's keeps the pain in check and she's not having bad side-effects then they're going to keep her going on it. She's still needed extra pain bumps but a little less frequent.

Nummy WaterThe surgeons were in and gave the OK to start drinking water! She's allowed to have 250 ml of water each day now. Josie insisted the doctors had said she could eat chips today, and she was eager to chow down. Of course we were correcting her on that idea, but she kept insisting that the doctor said it was OK -- "he did, he did!". Well, we were wrong and Josie was right. The Doctors did say she could start having ICE chips today. It was a little bit of a let-down for her when she figured it out. So we'll have to hold off on the chips & salsa party.

She was loving the little sips of water so much. She took a little nap in the afternoon and when she woke up she was begging "...water, water..." like she'd been in the desert for a week. After a little sip of water she looked up and said "...Nummy!" Of course this is a bit of an illusion for Josie. It's almost funny watching her drink the water because no sooner does it go into her mouth then it was sucked right back out through the NG tube. It was kind of like a human crazy straw...every swallow you would see it all flow back through the tube...kind of a cruel joke...but she loved it just the same. She really liked the idea of drinking again and after 64 consecutive days without water, I bet it tasted great!

Josie's labs are looking pretty good with the expected levels for a child with sepsis (blood infection). The good news is that her pancreatic enzyme levels were much lower with her amylase level (enzyme to breakdown starches) in the normal range and lipase level (enzyme to breakdown fats) still elevated but way down from yesterday. Her CRP (C Reactive Protein - inflamation marker) skyrocketed to 130.7 mg/l due to the infection -- normal is less than 8 mg/l. Her hemoglobin was holding steady until tonight when it dropped to 8.8, so that means another blood transfusion is headed her way probably. They'll check it again in the middle of the night and when it drops further they'll order up a new infusion. Josie's belly is becoming more distended (bloated), probably because of the bleeding inside...all that blood is going somewhere and the drains don't account for all the blood volume loss. Once again...time will tell.

So more ups than downs today, but generally speaking things are better than yesterday when it looked like we were headed back to that dark place again. We'll take it.

Sunday, November 12, 2006

11/12 More Sliding - Now Bacteria

Josie is continuing to slide further today...but there may be a positive turn starting. Josie's JP surgical drain was definitely plugged overnight and morning. This is a major concern because they certainly don't want a belly full of bile...this is a recipe for disaster if bile starts pooling inside the peritoneum space (abdomen). They tried to gently inject some saline to de-plug the thing but that didn't work. They didn't want to press too hard to explode everything in there so the next trick was to inject a substance called TPA (tissue plasminogen activator). This stuff is used to dissolve blood clots and is applied to patients who have had stroke to dissolve blocking blood clot. Anyway, they injected this stuff into the drain a couple times to try to dissolve the gunk blocking the many drain holes along the length of the drainage tube. This worked just a little and the flow has started again but not quite like before so they're keeping a close eye on it. If it’s still a problem then they may try to completely replace the drain which would likely be a surgical procedure. To do this they’d use a guide wire and insert into the drainage tube, the old drain would then be removed while leaving the guide wire in its place. Then a new drainage tube would be slid into the same spot along the length of the guide wire, and finally the guide wire would be removed. This is not a certainty for Josie, but it is possible depending on how things shake out, and only time will tell. Tonight the drainage has been steady and with some volume, but not like before.

In the mean time, Josie was in pretty serious pain most of the day. She was vomiting more blood and has been pooping old blood. Her hemoglobin level dropped again indicating more bleeding within and they had to give her another unit of blood. Her tummy is getting a little more distended, so things are teetering a little bit in the wrong direction once again. Her heart rate was racing most of the day between around 170 to 190bpm. Last night was pretty bad with high heart rate and pain and restless. She was able to get some sleep during the day today but it was lame sleep...waking often, retching, needing pain medicine.

More bad news was that the blood culture from yesterday came back positive, which means she is septic, which means Josie has bacteria in her blood. Yikes! This can be a very serious problem, but thankfully, modern medicine is to the rescue. The hardcore antibiotic, Vancomycin, which has traditionally been referred to as a drug of last resort, is now on board and she's coming back. Josie has been on Vanco’ quite a lot over the last three months, and now she’ll be back on it for a while. As of tonight her heart rate has dropped to below 150 bpm and her fever has dropped to about 100.5°. We were pretty anxious about today’s developments with the plugged drain and high heart rate and bloody vomit and bloody poop and bloody NG drain, and septic blood and further bleeding and more pain and -- let's there anything else? Believe it or not things are feeling a little better now that the heart rate and fever have turned in the right direction...the hemoglobin has remained steady since the unit of blood too. She’ll continue with her tube feedings as those don’t seem to be causing further trouble. The pancreatitis seems to be still there but not as bad as before…but that doesn’t jive with her pain which is consistent with pancreatitis. There are so many unknowns here, it gets frustrating at times…but we’re all doing our best to do what’s best for the girl.

More action tomorrow

11/11 Still Slipping - More Pain

Bloody Nasogastric and JP drains, blood oozing from the base of the duodenal drainThings are about the same or trending worse. Josie spent most of the in pretty bad pain. She needed extra hits of Fentanyl about every 90 minutes to 2 hours all day and night. She's been vomiting blood several times today too. The NasoGastric (NG) tube has been draining bloody stuff all day. The JP surgical drain is the big concern for me right at the moment. That has been consistently draining at about 10ml per hour for days and days now. Well, tonight it has pretty much stopped draining indicating to me at least that the thing is plugged up. This has happened before two other times with Josie and has been nothing but bad news in the past. It was the blocked drain that sent her back to the ICU for the second time after her first duodenal repair. Then three weeks ago we were back in the ICU with a blocked drain and it was only an hour after that was cleared when Josie was back in surgery. If those 10ml of fluid are not draining out the JP each hour, then where's it all going? It could be going down the correct plumbing paths but the likelihood of that is about as good as me winning the hot dog eating contest at the state fair next summer. The docs now know about it and are going to keep a close eye on it overnight.

Another development is that her gall bladder has become inflamed and dilated. It’s surrounded by some kind of fluid right now as discovered by the CT scan yesterday. They’ve started her up on a different antibiotic which are supposed to penetrate the gall bladder better than most in case there is an infection going on in there. If that doesn't work well then they'll consider sticking in a needle to drain the gall bladder using ultrasound to guide the needle. Not exactly sure about that whole thing so we'll learn more, so stay tuned for that.

Her labs are not as good as yesterday. Hemoglobin has been dropping indicating a new or renewed bleeding; pancreatic enzyme levels are about the same again so no relief of the pancreatitis (although sustained elevated levels are not the greatest pancreatitis indicators after a while); white blood cell count is back up again to 19 from 14.5 earlier in the day indicating an infection of some kind (could mean other things too as you doctors out there well know); her resting heart rate was 170 tonight at 11:00PM; and she's been running abut a 102° temperature much of the day even with Tylenol and tons of pain medicine on board.

The pancreatitis is still rolling along even without the feeds so they decided to fire up the feeding again with the fat-free formula through the Gastro Jejunal tube. They'll keep this up for a while per surgery's orders. Remicade is off the table now that it's pretty evident that Josie has some kind of crazy infection somewhere. Daily Cytoxan, weekly IVIG (Immunoglobulin), gobs of steroids are the treatment for her dermatomyositis for now.

It seems as though we're hovering between the road to recovery and disaster...just teetering there wanting it to tip just the right amount in the right direction. It’s still an anxious waiting game, with lots of clues and variables in this game needing attention as we wait.

More later.

Saturday, November 11, 2006

11/10 Sliding Back?

Day 18 of this fourth trip to the ICU
Day 62 without food or drink

Josie's day was the worst of the last few. Her tummy pain has returned, the retching is back, and she now has a fever. Blood has returned from out of the NG tube and JP drain. She feels pretty bad indeed. They’ve decided to keep going with the Fentanyl drip at a rate of 4 micrograms per Kg per hour up from 3 yesterday. The hits she's been getting are at 100 micrograms in one shot. Not much, but it doesn't take much of that stuff to make things better. She's had many more extra hits today to take out the fresh pain.

The good news is that the results of the CT scan were very positive. The tennis-ball sized hematoma is now way smaller...a little bigger than a normal marble now (from 60mm diameter to 15mm) that's great news. Also noted was that her liver, spleen, kidneys, adrenal gland, and most notably her pancreas all have no visible abnormalities, and there are no visible abscesses either...all good news.

Josie's labs are looking OK to pretty good still (besides the pancreatitis). Pancreas enzyme levels are about the same – not good, hemoglobin is normal -- good, white blood cell count is 17.7 probably coinciding with the fever, ALT (Liver enzyme) has dropped a bit but still elevated.

This all sounds like good news, and it is, but no one likes what's happening with the fever, increased pain, dry heaves, bloody drains -- that stuff is telling a a whole different story. They ordered up some blood cultures as soon as she spiked the fever. Additional bumps of Fentanyl have helped the pain. There's no evidence about what's causing the new pain and fever aside from continued we just keep a close eye and wait it out.

Thursday, November 09, 2006

11/9 Much The Same, A Little Better

Day 17 of our fourth trip to the ICU and all is calm...for the most part.

Josie's pancreas is still ticked. Amylase and lipase levels are still high and holding about the same as yesterday. ALT levels (liver enzymes) are pretty high too indicating active dermatomyositis (but this is nothing new). Her hemoglobin is stable and normal so she's still not losing further blood. Platelets are good indicating her marrow is doing its job of making new blood cells and platelets quite well. White blood cell count is now normal. We're still negative on the Stenotrophomonas bacteria culture growing. There's other labs which might be interesting...but the bottom line is that from the lab's standpoint...things look pretty good aside from the pancreatitis.

Josie was started feeding with a fat-free formula into her small intestine through the GJ tube. There are two differing views on this: one says it's these feeds that are setting off the pancreatitis; and the other view says the feeds were started so small and so mild and so far downstream from the pancreas it's unlikely this has triggered the anger within. We agreed with the step-up on the formula feeding as we're in the latter camp thinking the pancreas problems are less related to the re feeding and more related to Josie's severe plumbing problems...we'll see over the next couple days.

The JP drain bulb (from the surgical site) was mostly bile today...just a couple episodes of blood in the drain. Josie’s NG (Naso Gastro) tube was bloody red just once today while the rest of the day was more of the coffee-ground look indicating old blood. This means there is still bleeding within but it's not too bad.

There is a CT scan ordered for tomorrow morning to recheck the hematoma size and perhaps getting a better idea of the exact tissues involved.

Most of today Josie felt pretty comfortable. She had several extra hits of pain killer which worked just fine during those bad spells. Tomorrow we'll try a little harder to get her up and about just a little more. All in all...a little better than yesterday...and kind of dull. Those days 10 to 20 days back which were so crazy make these current days seem very uneventful...and that's just fine.

Wednesday, November 08, 2006

11/8 Not As Good

Feeling Not Great ... Just OKNot as good today...things are sliding a little bit. Josie's pancreas levels spiked up. Her lipase (enzyme that breaks down fat) rose to 883u/l (units per liter) today from 408u/l on Monday (0-160u/l is normal). Her Amylase (the enzyme that breaks down starch) rose to 157u/l today from 120u/l Monday (23-85u/l is normal). This of course is technical mumbo-jumbo telling us all that Josie’s pancreatitis is back and causing a fair amount of discomfort and pain. Josie has required quite a few hits of Fentanyl on top of her fairly heavy constant dose to manage her pain today. We're likely to start the slow transition to a different pain medicine real soon (at least I think that's what they were talking about). The popular thought is that the pedialyte feeding has touched it off…but the surgeons are skeptical about that idea…and frankly we are too. I think the hematoma has started to break up and that’s touching off the pancreas…but I’m just a dime-store GI specialist, Rheumatologist, Surgeon, Infectious Disease expert, ICU Attending, Computer Scientist, Carpenter, Brick Layer, Ditch Digger, Garbage Man, and Politician all wrapped up in a middle aged package – so I don’t really know what the heck I’m talking about half the time anyway. They’ve decided to keep going with the Pedialyte with hopes of moving on to more substantive stuff soon.

They've schedule a CT scan for Friday to track the size of the hematoma. It's looking like from the outside that it must be shrinking. Her belly is not as distended and is nice and soft. The JP drain has been draining lots more bloody, clotty output today. It's undetermined exactly where all the draining blood is coming from but the consensus is that it must be the clot liquefying and draining. It looks like old chunky motor oil when it's mixed with the continual bile flow. Meanwhile her hemoglobin levels have remained steady throughout the last day.

The surgeons were in and gave us the go-ahead to try and get Josie up and around a little bit more just to get her moving. She's been so sedentary these last bunch of weeks they just want to see a little more exercise out of her just to help everything in there heal up a little faster.

The Infectious Disease folks were in again this morning just to check up on the girl. They said that the culture from her JP drain was still not growing the Stenotrophomonas bacteria that grew out of the old sample last week. This is good news and hopefully her treatments are working well against that. Dr Vehe talked a little more about Remicade again today. We decided that if the Stenotrophomonas does not grow then we'll fire up another dose of Remicade for the girl ASAP.

Generally Josie is feeling worse, retching a few times today, many times complaining of tummy pains -- about a 6 out of an ICU 1-10 scale (there’ve been a few 1s over the last couple weeks). On we go.

Tuesday, November 07, 2006

11/7 We're In A Good Pattern

Things are good...well...stable at least. No jumping jacks today and maybe not feeling quite as well as yesterday...but all things considered she's feeling pretty darn good. The Doctors continue to be encouraged because things continue to remain stable. Every day we get is a day further down the road to healing those fragile insides of this little woman. Not a lot happened today...just some coloring, some books, lots of TV, more crabbiness unrelated to feeling bad -- more like a standard issue three-year old stuff.

Where's Waldo?We need to get some images of Josie's abdomen again maybe on Friday to see what's happening with the hematoma. It's still up for debate exactly where the hematoma is, and it really doesn't matter where it is so long as it's not getting bigger. Judging from her hemoglobin levels which have been very stable now for a few days and minimal bloody output from the JP drain, NG suction, and poops, the consensus is that that big ol' thing should be shrinking a little bit and definitely shouldn't be getting bigger. They'll probably do a CT scan again. There has been discussion about doing an MRI but typically MRIs are not done on tummies unless the patient is totally still – like knocked out. A CT scan takes about 20 seconds but an abdominal MRI can take upwards of an hour or more. Just normal breathing moves the belly and that'll throw off the imaging of an MRI. MRIs are long-exposure imaging and the slightest movement greatly reduces the image quality. Also, an additional CT will give a like image to compare from last time. It would be interesting to know where the hematoma is located (within which exact tissue) but that’s information isn’t necessary at this point. If the size is unchanged or smaller then it'll be a basic waiting game being patient with Josie's body as she gets rid of it naturally.

Since she's doing so well they decided to get her back on feedings again. They'll start out like last time with the Pedialyte in the GJ (Gastro Jejunum) tube at a slow rate and if all goes well then they'll step up the volume and then switch it over to formula. This time though they'll go with the totally fat-free version so the pancreas is less likely to get angry…and we’re now all too aware of what angry pancreata can do.

Hemoglobin is higher at 13 gm/dl well within normal range, white blood cell count has come down to normal, the C Reactive Protein (CRP - an inflammation marker) has dropped from 50 mg/dl a couple days ago to 15 mg/dl today (normal is almost zero)…headed in the right direction. Her lymphocytes are low but that's normal because of her Cytoxan treatments. The pancreas levels were not tested last night...but they'll test for those tomorrow. Dr. Vehe has decided that since her dermatomyositis seems to be getting a little more under control, we should be able to reduce Josie’s steroids by about 15% which will also help reduce her chubby face (it's been blowing up lately). The re culture of the JP drain has still not yet grown out the Stenotrophomonas maltophilia from a few days ago...we'll keep a close eye on that too.

There's no chance (for now at least) that we'll be heading over to the other floor soon. Several, maybe many more stable days need to happen before that'll occur. The main reason is the size of that hematoma and all the trouble she had after the last surgery. If her duodenum is continuing to erode in there that might expose a little artery which could become a big-time bleeder pretty they want to watch her pretty close, even though not much is going on.

So that sounds like a lot but it's really been kind of a ho-hum kind of day -- just still feeling pretty good and getting better.

By the way…Where’s Waldo?

Monday, November 06, 2006

11/6 Another Good Day

Feeling better, joking around with the nurses.Things are going great. We're still a little unnerved about the overall status of Josie's condition, but the short-term outlook right now seems good (long-term outlook is still up in the air). Hemoglobin levels continue to be good. Pancreas enzyme levels are a little better too. White blood cell count is better again as well. Her Naso-Gastro tube has drawn no blood at all today...just normal clear goop. She still has the constant Fentanyl drip going to treat pain but has needed far fewer bumps...only three today. This is all good news. Yesterday they switched her Bactrim antibiotic from a preventive low dose to a therapeutic higher dose to take care of the Stenotrophomonas bacteria. They also changed the JP drain collection bulb and re-cultured to see if the bacteria bug will grow out again. The Infectious Disease Team also decided that Josie should get off all the other antibiotics and the only one she's on now is the Bactrim.

Josie's dermatomyositis is still active but it appears that it's just smoldering rather than a full blown flare. We can't really determine what's happening with her duodenum since the imaging (CT scan or normal X-ray) can't help determine that. Her treatment is still daily Cytoxan and she's been tolerating that just fine. She's also getting weekly pulses of solu-medrol (steroids) and weekly infusions of IVIG (IV Immunoglobulin). We're all eager to get the Remicade going again but with any type of infection, starting up Remicade is out of the question for now.

Today Josie was being her 'normal' sassafras self with Mom...arguing about TV shows, or body positions, or which books to read, or how to take temperatures, or apply bandages, ya' know - just about everything you'd expect from a typical three-year old. And of course when I arrived she turned back into the perfect little angel that Daddy's girl has become. We read a pile of books, watched Sponge Bob, took vitals, conversed with nurses, smiled for cameras, and was agreeable to everything just like the well behaved, well adjusted, perfect little person every parent dreams of. And so it goes for mothers. It's not quite fair, is it? I'm sure lots of moms out there can relate. Over the last hour that I've been checking my email and writing this post and processing pictures, Josie has been sitting up on her flat bed reading out loud every book in the stack of 20 completely content. Now she's coloring again with not even a hint of pain. This is great to see, and frankly something I didn't expect to happen for a very long time.

If the last two days are any indicator it really looks as though Josie has stabilized and the immediate GI crisis has moved from DEFCON 1 (all out war) to DEFCON 3 (readying for war). We're looking for DEFCON 5 on all fronts...and we'll let y'all know when we're there. We're hopeful that with this new-found comfort and stability that we might be headed to the other floor sometime soon...but we haven't talked about that with the Doc's at all yet.

PS Don't forget to vote Tuesday!

Sunday, November 05, 2006

11/5 An Unbelievable Change

Feeling BetterTruly remarkable! That's the only was to describe the change in Josie’s whole presentation over the last 24 hours. Just yesterday we were thinking some pretty bad thoughts...needing two units of blood with an obvious significant bleeder somewhere...pancreatic enzyme levels very high...high blood pressure...super-bloody output in both naso-gastro tube and the JP drain...lots and lots of pain...super-high resting heart rate.

As of this evening Josie has turned a full 180°. Resting heart rate at 120 bpm...lower blood pressure...much lower pancreatic enzymes...hemoglobin levels steady or rising (meaning the blood leak had gone way down or has stopped)...white blood cell count much lower...much less blood output in the drains...but best of all, Josie is feeling so much better! She's been coloring, and chatting, and being sassy, and ordering everyone to itch her feet, and telling the nurses how to do their job, and everything. She was actually standing for a few minutes and sat upright (not leaning back on her bed but sitting upright) for more than a half hour. She was playing stickers with one of the nurses, and watching movies...and generally feeling pretty good!

Belly Tubes...Gastro-Jejunal tube on the right (GJ); Duodenal Drain on the left front; JP surgical drain - darker left rear; The open hole from the old jejunal tube on the right frontIs there any bad news? Well...her blood-oxygen levels have been dropping from time to time, and for Josie that means she has to cough up some gunk from her lungs to get that blood-oxygen level back up to normal...not a biggie. The most significant thing is that one of her peripheral IV catheters had become infiltrated (vein burst) and her arm became pretty inflamed so they had to take out both catheters in that arm and start two new lines; one in each foot. Josie didn't like that little project too much and made certain the IV access people know about it. She's currently on so many drugs that she needs four IV access points, and tonight she has her double-port picc and two IVs - one in each foot.

The Doctors are impressed that Josie can have an abdominal hematoma the size of Rhode Island and feel good, let alone down right perky. My theory is that the hematoma has walled itself off becoming encapsulated, and is no longer irritating anything nearby and decreasing the pancreas aggravation, lessening the pancreatitis pain and making the abdominal pain go way down. Of course that's just me making up theories (which I like to do these days) since we're getting no definitive explanation for the turn-around of symptoms and demeanor. This little person continues to get heads shaking, but this time in a good way.

Because this change was so sudden, we're all watching carefully for bad things...things that could cause her to come back to the dark side, and we'll take it for sure. So talk about a good day -- this was a great one.

Saturday, November 04, 2006

11/4 Plusses and Big Minuses

We’re not quite sure where we’re headed in the coming days…but it’s not looking like easy recovery from here.

Josie’s hemoglobin had dropped to 6.9 gm/dl at about midnight last night so she had a blood infusion at about 2:00 AM. The levels rose, of course, to over 10 gm/dl after that. Then at about Noon the hemoglobin had dropped again to 7.1 already, so they threw another unit of blood in her. At 7:00PM the hemoglobin level has dropped again to around 7.1 gm/dl and it looked like for sure another unit of blood was headed Josie’s way…but a new result of 10.0 gm/dl at 10:30 PM indicates a bad reading at 7:00 and we’re OK for now. Still…two units of blood for a little person like this is a pretty bad sign that something is amiss (I’ve been told I’m a master of the obvious before). All this blood is missing and not that much is coming from the drains and not any bowel action (poop) at all. This can only mean all that blood is finding its way somewhere outside of her circulatory system and likely (almost definitely) into her abdomen.

Josie 21 months ago before all thisThe conclusion of the CT and the tennis-ball sized hematoma is inconclusive. They're uncertain where this blood mass is. It could be in her stomach, or within her stomach wall, or in her peritoneal space (open abdomen), or inside her bowel, or in the bowel wall…it’s not clear from the imaging. If it’s in her bowel I can’t imagine the bowel would be able to stretch that much without bursting…but it’s unknown. It’s amazing that with all these techno-whiz-bang toys they can’t really tell what the heck is up.

Josie’s pancreas levels are just a little worse than yesterday and her white blood count is a little higher too at a little over 18,000…but still not terrible. Her blood pressure rose dramatically last night (156/113) and they gave her some hypertension medicine for that. Shortly after it came back down to just elevated…no biggie.

More bad news is that they speciated the bug growing out of the JP drain culture. It’s a thing called Stenotrophomonas maltophilia which is an uncommon (can you say patttern) bacteria difficult to treat because it’s basically immune to most antibiotics. It can be tricky and nasty if an infection happens…but this little critter likes to colonize rather than infect…and it’s most likely the case here with Josie. The best drug for this is Bactrim…but the curious part of Josie’s story is that she’s been on Bactrim for months now. We need more info…but this is another one of those weird sideshows in this crazy medical-circus case. The silver lining if this particular sideshow is that because this Stenotrophomonas bacteria is contagious, Josie needs to be in isolation…and that means private rooms – and because this bug is tough and can take weeks to eradicate, we’ll be in isolation and private-roomed for the long-haul -- How’s that for making lemonade out of lemons?

Josie 20 months ago just over 2 yrs oldJosie’s pain overnight was much worse and she didn’t sleep a lot because of it. Her constant drip-dose of Fentanyl (pain killer) has tripled over the last four days. And when her pain gets really bad her Fentanyl bolus hits are more frequent and double the dose of what they were just a few days ago. She’s being helped a ton by all the pain killers…but we’re getting on to about 10 days post op’ with heavy Fentanyl. At about 15 days they need to move her off the Fentanyl to something different and when they do they’ll have to be very careful to avoid withdrawal symptoms of her addiction to this powerful painkiller which can include anxiety, irritability, confusion, tremors, sweating, abdominal pain, and rapid breathing. They’ll go slow but the nurses say it can get really ugly if it’s not monitored close.

Now for the good news. Josie has been much more animated and lively this evening. She’s been talking, sitting up, and she even colored for the first time in 10 days. This is big news…but frankly I can’t believe what I’m seeing considering how much blood she’s mowing through and how much dope she’s on. It’s almost like this is the false calm before the storm. Hope upon hope that I’m wrong on that – and it’s sure fun to see her feeling just a little better for now.

This sickening trip keeps changing hourly and...yeah it’s sure interesting…but we’re all really ready for many more positive changes rather than two critical negative, three regular negative and only one positive change which seems to be the pattern lately. Onward the fight goes.

I was looking back at some past images of Josie and these are two from almost two years ago before she came down with dermatomyositis. As sad as it is…I don’t really remember how she used to look…and peeking back at these got a couple of wows out of me.

Friday, November 03, 2006

11/3 Another Complication - Feeling Worse

CT ScanOvernight Josie's hemoglobin level dropped from 13.3 to 11.0. The Doc's ordered hemoglobin tests every three hours and that level stayed about the same in the mid 10s throughout the day. The JP drain output was bilious for the most part up until about 8:00 AM this morning. Shortly after the output became heavy coagulated blood...thick and gooey...and a lot of it...which was way different indicating perhaps big changes (and maybe big trouble) within.

At 10:00AM we headed to radiology for a new CT scan to take a peek inside. Josie was a trooper as always during the scan process, and she made sure everything was good with us too. When I put on the protective lead apron Josie looked at me, pointed and said "Daddy, don't forget the neck thing." Of course I had forgotten to put on the lead neck protector, but she made sure I was playing by the rules.

We got back up to the ICU and Josie was feeling just crummy. A high heart rate between 160 and 190 bpm...and it hasn't come down much in the last 24 hours at all. She's been getting more bumps of pain medication to relieve the tummy pain but she was still complaining about it. The good news today is that her white blood cell count and pancreas enzyme levels had dropped quite a bit in the last 24 hours, maybe indicating that the feedings from the other day had touched off the pancreas after all. A culture was started yesterday of the JP drain output and there has been some cultured growth indicating some kind of infection. We'll have to wait to see what’s exactly growing and then how to attack it...this is a big deal, but a relatively minor complication when looking at the big picture.

The results of the CT scan were startling! It revealed that there is a hematoma (collection of blood) the size of a tennis ball in her abdomen! heard it right. There is a sphere shaped collection of blood 6 centimeters in diameter sitting there. This is a giant thing within the belly of a little tiny person. It's difficult to determine exactly where this thing is as the final report from radiology had not yet been submitted…and of course I’m looking at this thing sideways wondering which side it up. The surgeons were in, saw the scan, and they didn't like it (du) but were not considering more surgery to remove it (at least for now). It's going to be a wait and see deal. The hope is that there’ll be more waiting, but I think that's wishful thinking.

Spaghetti Tubing, Bloody JP Drain Bulb and NG Drain CupAs of 10:00PM tonight things are still about the same except that her hemoglobin had dropped another gram to 8.6 grams per deciliter (gm/dl). This means she's still losing more blood than she's making (maybe making it a baseball by now?)...and it'll likely mean a new blood transfusion sometime in the next 24 hours. The exact plan has not yet been established as there are still some unanswered where exactly is the tennis-ball sized hematoma? Within what tissue? How are we going to break it up and get it out? Does that mean more surgery? Or are we just gonna leave it there? No matter how you look at's sure looking like trouble.

Josie is hangin' in difficult as it is. This ordeal is a real test for her. She's been retching, and hurting, and feeling really really bad for a long time now. It makes you wonder just how much more this little champion can take, because you or I would be crying uncle after about 10 minutes of this torture...that, I can guarantee.

Ya'd think being is the hospital would be anxious but quiet, dull, boring, and a total drag. Granted it's a total anxious drag for sure, but it's certainly not boring or quiet or dull. I think we'd all prefer that right about now -- a nice long boring, dull, month-long, uneventful span. I don't think that'll be happening. I've got the uneasy feeling there'll be more active days coming up than boring ones.

Thursday, November 02, 2006

11/2 Staying In ICU -- For Now

Sleeping UncomfortablyI guess the lesson here is to not predict anything with Josie's case. We were set to move to the other floor and then this morning things changed. Josie's heart rate has climbed to a resting rate of about 160 bpm. This is a very high rate...even for a little squirt like Josie. Sleeping at a 160 heart rate is a bit of a concern, not just for the parents but for the doc's as the ICU is where she'll stay for a while longer.

Josie is feeling really bad still. She just wants to lay there and do nothing. She really hurts when you move her around or change her diaper. She doesn't want to sit up at all. She's retching often and ... just hurting. Her pain medication is still heavy and she's certain to become addicted to these narcotics if she's not already. Josie will have some uncomfortable withdrawal symptoms and a lengthy weaning process as she comes off these drugs. But for now they are required because that pancreatitis is very painful and anything else in there trying to heal. It's uncertain if there is further pain coming from the problem duodenum, and we have to assume there is.

There's good news and bad news. Josie's pancreatitis enzyme levels are worse today than they've been since last week's surgery, showing much higher today. On the other hand her hemoglobin has remained steady up to now, which means she's not in a deficit blood cycle (losing more blood than she's replacing). Her white blood cell count has risen significantly in the last day to 29,000 or so. On the other hand she's had no fever to speak of.

Visit From Grandma GammieThere's debate as to why her pancreas is getting angry and releasing enzymes. One theory is that yesterday’s jejunum tube feedings could have naturally stimulated the pancreas, which is the GI service's theory. The Octreotide (Sandostatin is the active drug) was stopped two days ago in order to start up the tube feeding, which may have allowed the pancreas to act up again -- and no one has hung their hat on that theory for some reason. Another theory is that the duodenal leak is sending bile into the peritoneal space, touching the pancreas, and making it angry. All good could be one of them, it could be all of them, or it could be none of them. My vote goes to the duodenum/bile theory. If this is the case, the biggest concern is that the bile is not only leaking as far as the pancreas, but throughout the abdominal cavity as well. This is the scenario that occurred last week…the one that gave Josie chemical or bile peritonitis – when her guts were swimming in bile just before the last emergency surgery.

We stopped the feeds so if it was that we should see some decrease in levels tomorrow. Unfortunately even with all the smart people on the case, Josie's condition is still such an unknown and it truly remains a guessing game -- things only seem to become more clear about one to three weeks after the fact. That hindsight is sure a great thing, isn't it? I just wish we all had foresight like our hindsight...I think we'd all make far better decisions throughout our lives...and it's pretty clear medicine is the same way.

So we're still driving down that unknown windy mountain road in a foggy nighttime snowstorm...and overshooting our headlights...and that's the way it needs to be because this runaway truck's not stopping right now. This go around has an eerie feeling of Déjà Vu all over again from last week. Time will certainly tell, and in the mean time I just hope that everyone involved can make the best decisions possible.

Wednesday, November 01, 2006

11/1 Tough to Watch

Josie is still in about the same state. She’s just hurting and laying there with little ambition to move or talk or do anything. Her heart rate and body squirms tell us she’s in pain, but she won’t say anything. Her dose of pain killers was reduced by 60% and she was just a little more alert and said a few things today, but she was mostly unresponsive.

Her drainage tubes are telling a story, but we’re not exactly certain what that story is. She’s producing lots of biley liquid out of her JP surgical drain with intermittent bloody output. Those bloody episodes are still coinciding with pain. There’s hardly anything coming from the duodenal drain, and her NG from the nose to the stomach is producing a bit and occasionally bloody. She had a couple poops today which mean her guts are moving along better, but one of the stools was maroon in color indicating undigested blood in the GI tract coming from somewhere. Her hemoglobin level is holding steady and that’s good news because whatever she’s bleeding out, her body appears to be replacing.

Josie started feeding into her J tube (jejunum) directly into her small intestine. Yesterday she was on pedialyte and today she started up on formula. This is a big step. She’s only taking on about a teaspoonful each hour, but if she tolerates it well then we’ll increase the volume daily.

Things are stable now, which is good, but I wish it was more of a pleasant stability rather than painful. Because she’s stable we’ll be moving to the other floor of the hospital sometime tomorrow barring any unexpected problems. Hopefully she’ll be able to get stronger and have this pain of hers go away soon.