1/31 Getting Stronger

Josie had a really fun day. Still some bouts of nausea and vomiting but not too much. Most of the day she was full of sass and fun….happy and energetic and bouncing around. She was singing and even dancing in her bed. She sang a song to her doctors which she learned just yesterday. Every day she seems to be getting better and stronger. Josie had some fun with her physical therapy team this afternoon too…she seems so much stronger now than she was when we arrived in August. It’s so nice to see.

This is all good, and we need to keep ramping up all the way to the big day in a couple weeks when her strength and grit will truly be tested. She’ll need all that strength.

1/30 Five Months

Josie celebrated her five-month anniversary in the hospital yesterday. The milestone was celebrated with a nap and a biopsy…that’s about it. Never ever thought it would be possible that she’d be in the hospital this long. I remember asking a nurse when we were about 10 days into this thing how long some folks had been there. The answer was “…some people have been here for several months…” This was shocking at the time how anyone could be there for so long – and here we are at five months and counting.

The biopsy results are in and here’s the report: Portal fibrosis, ductular proliferation, ductular and canalicular colestasis, mild acute portal inflammation. That’s all a fancy way of saying that Josie’s liver is a bit of a mess. These are all abnormal reactive changes most likely caused by the months of IV feeding Josie was on. This is a known side effect of the TPN IV nutrition. Other possibilities are chronic sepsis (which they think she has had), biliary obstruction (which she might have, but not yet determined), and less likely drug toxicity (which she most definitely has had). She’s just put her liver through the ringer these last five months and it’s crying out ‘uncle already’. The docs are confidant that her liver is not damaged beyond repair and when she regains her health after her Whipple then the liver should repair itself nicely.

We’re still working the project to get Josie home but are continuing to run into trouble finding a way with her drug regimen. They’re trying to find a good a low-risk solution to the problem without making many changes to Josie’s drug course. We’re in a let’s-not-rock-the-boat-too-much-because-we’re-in-a-pretty-good-place-right-now mode. Any major change could throw Josie’s relatively stable condition totally out of whack before the surgery and no one wants that.

1/29 Biopsy

Today was interesting. Josie’s feeds were stopped shortly before midnight to prepare for her liver biopsy today. At about 10:00AM we headed for the interventional radiology where it was going to happen. She was sedated with a drug called propofol which is a fast but short-acting anesthetic. They pumped her up with a syringe of this stuff and she was out. They took the probe of an ultrasound machine and slipped a plastic bag over it and used it to direct an inserted a needle guide into her skin next to her liver. The ultrasound showed the guide nicely…it was pretty neat to watch. Then he took the big needle and inserted it into the guide straight into her liver, and then extracted it just as fast. He then shoved the very small but long core into a specimen jar with some liquid. The core looked like a very thin worm thinner than the thinnest guitar string. Then he did it again to get a second core. He inserted some coagulation-promoting foam into the hole, threw on a Band-Aid and that was it. The whole process from sedation to the end of the procedure took all of about 10 minutes with the biopsy extraction itself taking no more than about five seconds each time. We’ll have the results tomorrow or Wednesday.

After coming out of the sedation she was moved back up to her teensy-weensy closet. The rest of the day she was feeling really great. There was no indication she was hurting or feeling bad about the biopsy in the least. Josie’s good times are certainly increasing while the really bad times are becoming less frequent and shorter in duration. Still some vomiting and a low fever today but those vomiting events are short-lived. All those blood cultures taken over the last week are all still negative.

We had a meeting with the GI Docs and the surgeon about the big surgery coming up. They went over exactly what the procedure was and the anatomy changes they will make during the surgery. The Whipple is one complicated operation which they say will take them between eight and 12 hours to complete. Due to the complexity of the operation there is between a five and 10 percent chance Josie will not survive the surgical procedure and its recovery. The real challenge will be her recovery and how her underlying Dermatomyositis will respond. If there is a flare before or shortly after the Whipple it could cause all kinds of problems additional.

As y’all know Josie’s had three major abdominal surgeries back in September and October and each one failed as a result of her underlying disease. The hope is that after all these weeks of healing that her insides have improved enough to handle all the slicing and reconnecting required with the Whipple. I’ll go through the whole procedure in a future post for you.

Now our thoughts are on the matter of going home or not. The decision is ours to make if we want to implement some changes two which will allow Josie to come home for a while before her Whipple. It’s a risk to make these changes and it’s unknown the actual risk level. We’ll figure it out and do our best to make the best decision we can for our little Josie.

1/28 Not Much Happening

A fairly uneventful weekend. While Trip, Henry and I were out of town at a hockey tournament Josie remained at the hospital with Deb continuing her slow recovery. There wasn’t much going on and she remained stable most of the weekend. She was up and down on her fever but nothing major. Her episodes of nausea and vomiting continue. That means thing are pretty much the same.

Dr. Vehe was in and is impressed with how she looks. Last week’s mild Dermatomyositis flare seems to have calmed nicely.

We’re still working the project to go home this week yet. The Cellcept mixing problem might be resolved, maybe. She’s currently on intravenous Cellcept and that’s the one which has such a short shelf life requiring mixing right before administering with special safety gear. They’re considering switching her over to oral Cellcept which is much more stable and can be mixed at home with no problems. The issue is really how well Josie’s gut can absorb the drug. She’ll be tested to check her blood for Cellcept counts to make sure she’s getting enough. That decision will be made later in the week.

Her liver biopsy is scheduled for tomorrow sometime. After chatting with the GI folks they seem pretty optimistic about her liver’s long-term prognosis even though she’s having trouble now. Her lab values from today show a slight drop of her bilirubin level and a fairly significant drop of the ALT and AST liver enzyme levels, but those levels are still at about the upper limit of the sort-of-comfortably-elevated level of five times the upper limit or normal. That’s not really comfortable but higher than five times the upper limit of normal seems to be the threshold of pretty serious liver problems.

We’ll let you know the results as soon as they become available.

1/26 Uneventful

Not much going on this day. Josie's temperature was up again to over 102° and that's never encouraging but she came back down to earth after more blood cultures (which have all not grown out yet).

They decided to wait until Monday for the liver biopsy. Still no strong thought one way or the other as to what's going on with her liver and spleen or its cause. We'll learn more on Tuesday or Wednesday after the results are in.

Later today Josie started feeling better and was enjoying herself with Momma. Trip, Henry and I stopped in tonight to say hello and Josie was very happy to see her brother Trip. She gave him a big giant hug and was holding his hand. It was very cute.

Trip, Henry and I are off to Albert Lea, MN for a Squirt hockey tournament. Deb'll be holding down the fort with Josie. I'll check in from there if I can.

1/25 Going Home? Maybe Not

We spoke with the GI team today and they’re pretty concerned with that liver of hers. They’ve decided the best course is to take a liver biopsy and that should happen either tomorrow or Monday. They’d like to know more about what’s happening in there and the gold-standard test is a biopsy. From that they can tell lots of good information, like the level of scarring, or the level of fatty infiltration, or if there’s a smoldering infection in there…lots of good stuff. They’ll take a big ol’ needle and stick it right into the liver and bore out a core of tissue. Upon removal they’ll plug the hole with a foamy cork to prevent bleeding from the big hole punched in her liver.

We talked about the upcoming surgery and the importance of keeping her spleen. Josie’s spleen is acting as a pressure valve for the liver which is partly causing the spleen’s large size. If the spleen is taken out then the liver will have only itself to deal with the pressures and that will likely cause dilation in its vessels and that’s not what a stressed liver needs. Also, the spleen plays a pretty big role preventing infection and if it’s removed that’ll make Josie more susceptible to infection. This is not a very nice thing considering her autoimmune disease. They really need to do whatever they can to keep that bugger in there.

We had our big Go-Home planning meeting today. We talked about Josie’s needs, all the drugs she’s on and how best to manage it all if we end up going home. Looks like the earliest we could go would be next Wednesday the 31st. Coincidentally this is the last day of Josie’s confinement in that fine spa-like sardine can we’ve been enjoying these last two weeks. When home there will be lots of mixing of drugs and lots of administering of drugs, weighing and measuring fluid inputs and outputs, vital sign monitoring, strict time sensitive drug administration, suction of her various tubes, on and on and on. Daunting for sure but we can hack it (I think). Then we went over Josie’s chemo drug, the IV Cellcept. Turns out this is a big problem. Being that Cellcept is chemotherapy and that Josie needs the IV version we need a safety device called a 'chemo hood' in order to mix it properly (or within safely guidelines at least). A chemo hood isn’t something they readily hand out to people, and you can't find one at Target either. It’s kind of like a salad-bar shield with a vacuum cleaner hose sucking the air out. Cellcept has a shelf life of about four hours so it needs to be mixed at the time of administration. This may very well be a going-home show stopper. We’ll see what we can figure out.

Dr. Vehe (pronounced Vay and not Vay He) was in and Josie was all full of it, pulling his tie and pushing him around. She was really full of energy when he was there. It’s so nice to see her enjoying herself some of the time again. This was the call of the day…until later when she spiked a 102.4° fever. Nice! Back on antibiotics and more blood cultures.

Never a dull moment with this girl.

1/24 Just Over Three Weeks Away

Interesting day today for Josie. She was all full of energy and crabbiness. I think her steroid high is wearing off and that’s why she’s being quite surly. She definitely had the cranky pants on today for Mom. But sprinkled between the cantankerous episodes were some fun-loving good times too. She even found the time and demeanor to sit still and have her face painted. It was pretty cute when she was looking at herself in the mirror admiring her beauty.

The results are in from the Doppler ultrasound of the liver. Part of me thought the report was going to show some heavy snow squalls moving in from the left anatomical lobe, but then I remembered this wasn’t Doppler radar…just something similar. The results show enlarged spleen and liver with normal blood flow, as far as I can tell from the cryptic description. There doesn’t appear to be any blockage either. The texture of the liver is coarse and that’s not the preferred state. We’ll see what the GI guys say tomorrow. They had no insight today when we saw them since the report was not available until tonight.

Surgery was in and we’re on for Josie’s Whipple. It’s set for Friday, February 16th in the morning. That’s just over three weeks away. That will certainly be an interesting day and weekend. Talking with surgery about her condition and her sustained distention…it may be necessary to remove Josie’s spleen during surgery. The reason is that with Josie’s tiny size and the location of the pancreas and gall bladder and other business on which they need to surgerize there is limited space. There simply may not be enough room in there to work. It’s kind of like working on your car and needing to take off the radiator to get to the important broken part. The problem is the with Josie’s enlarged spleen it may need to be removed in order to gain better access, and the bad news about that is once it’s out it can’t easily go back in. So looks like Josie may very well be a few organs shy of a person when all’s said and done. Scary thoughts, but still very doable.

1/23 Spleens and Livers

We had a visit from GI today and the talk was about livers and spleens. It’s pretty conclusive now that the distention of Josie’s belly is being caused by both an enlarged liver and enlarged spleen. Those two organs are taking up so much of her abdominal space they’re just pushing the rest of her intestines away and the only way to go is out.

The GI Doc ordered up another ultrasound today with Doppler to track the blood flow throughout the spleen and liver. Hopefully this will provide some good information on what’s wrong. Many times these types of symptoms indicate some sort of blockage or obstruction preventing blood flow or bile flow. There are other consideration with liver disease, but it’s been brought on by her condition throughout her stay since September.

Josie had a pretty good day today after a pretty rough night. She was up at least six times vomiting overnight. Her fever was up to 101° and hear rate above 160. By mid day those both came down with a normal temperature and 130 heart rate allowing her to feel much better the rest of the day. She was up doing lots of painting and coloring. We were walking back and forth across her teensy little room. She’d grab my finger and we’d walk together around the bed, then she’d lean against the bed for a little bit for a break and then she’d head back around the bed to her chair with no help at all. This was hard work and she didn’t much care for it, but she’s so agreeable to this type of challenge. At the end of the walk she’d be whimpering and crying, then sit for a minute or two and be ready for the next rep. Nothing like being a slave driver Dad/Coach getting the most out of his little athlete.

Later in the day she was complaining of tummy pain so we ordered up a fresh dose of Oxycodone (her latest pain medication), but while waiting she dosed off and is still asleep, likely out for the count.

We’ll learn more from the Doppler test tomorrow. Gotta get that liver under control

1/22 Low Key

Not a lot happened today…really low-key. Josie had a good time with the physical therapy gang strapping on some roller skates for some fun and games. She had some laughs at times during the day. But more times than not Josie was feeling pretty bad.

Nothing interesting showed up on her lab-work today. All the services came in for a look see at Josie but nothing different came up. It’s kind of funny…not much to talk about for a day when almost every day over the last five months has brought one surprise or another. This is just the kind of day we need to keep the idea of going home strong.

Josie's pain is still an issue. She's still on a hefty prescribed dose of Methadone and is allowed additional doses of Oxycodone every four hourse if she needs it for breakthrough pain. We've tried to maintain a certain comfort level with her pain and provide Josie with additional pain medicine when appropriate, but at the same time we'd really like to see her become less dependant on these narcotics.

1/21 Snowball Fight!

Liver tests came back today with mixed results. ALT and AST enzyme levels have increased quite a bit to 264 and 223 respectively. Her total bilirubin level is a little lower at 6.3 mg/dL down from a high of 7.4 a week ago. Josie’s distended tummy is really causing her continued discomfort. Her liver is so big…it, along with her large spleen, is causing her intestines to be pushed aside causing her size-large tummy.

Her flaring Dermatomyositis symptoms have calmed a bit and she looks much better, but still some concern there. We’ll wait a little longer to see how things shake out before any further treatment changes are made.

Her fever stayed down today but that didn't keep those uncomfy feelings from creeping back to Josie many times. More vomiting too.

Josie was pretty active today. One of her nurses brought in a bin of snow from the outside. They were in the room and had themselves a snow fight. Of course they did their best not wo make too big a mess, but they still had a lot of fun with the snow. Other than that it was a pretty low-key day.

We’ll probably have lots of home discussions tomorrow to determine if and when Josie will head home this week or next or maybe not at all.

1/20 Dermatomyositis Is Back

I’m not sure how to categorize today…good, bad, or indifferent. Knee jerk would say bad. Josie’s dermatomyositis seems to have flared to a degree higher than we’ve seen in months. Her skin under the arms appears worse…quite a bit worse. Her fingers and hands are looking mottled (a term used to describe an appearance kind of splotchy differing in color). Her toes have areas of redder appearance on the tips. Her nail-fold capillaries are showing signs of inflammation like in yesterday’s picture.

This is not completely unexpected though. Josie’s Cytoxan was stopped and replaced with Cellcept. We knew that Cellcept was on a trial basis with Josie this go around. Dr. Vehe said that by Sunday we would know what the story was. Now I think it’s pretty clear that Cellcept is not the answer. Depending on what happens the next 24 hours Josie may get a dose of pulse steroids and a pulse bolus of Cytoxan. Daily Cytoxan may start so after that if things don’t stabilize very soon. Here dermatomyositis is getting a little bit out of control and we do not want another raging flare.

Josie was generally feeling pretty crappy today. She was throwing up often with diarrhea and a slight fever (nothing like yesterday though). Those little spots on her skin in the pics form yesterday were determined to be petechiae. Petechiae are small red or purple spots on the body caused by a minor hemorrhage (broken capillary blood vessels). There’s no harm with them and they’ll go away in time. The thought is that a virus of some kind has triggered the fever spike and aloes caused the petechiae.

The Infectious Disease folks were in and they wanted a C Reactive Protein (CRP) test done. CRP is an inflammation marker and if it’s high the Infectious Disease people think it would most likely be caused by bacteria. Although dermatomyositis flares also show elevated CRP levels. The lever tested out at 15 today which is lower than yesterday’s 19. This is good and may lead to having her antibiotics stopped if nothing grows out of the cultures over the next two days.

Liver is still a touchy issue. They’ve decided to perform a liver a biopsy during the big surgery in February…long time from now. A liver biopsy will give the GI folks a clean piece of liver with which to experiment. Moving forward from here, liver tests are ordered for Sundays and Wednesdays. Tomorrow’s liver tests should be interesting.

1/19 A Little Action

We were getting so used to the same old thing Josie decided to spice things up a little bit today. A fever of 102.9° and a high heart rate of 180bpm prompted another battery of blood cultures. It also ended the short run of days with no antibiotics. They decided to put her on a three-day regimen of Vancomycin while we wait for cultures to grow. If nothing grows after 72 hours then they’ll stop the drug. There doesn’t seem to be anything which has changed but her we are again in a puzzling situation…but not too bad.

Today she had her weekly dose of steroids and her weekly dose of IVIG. The steroids are really interesting because shortly after she gets the dose she’s bouncing off the walls. So she was feeling great this afternoon and evening. She was up walking around again…and the walking is coming along quickly. Her confidence is growing with each successful series of steps. It’s sure nice to see.

We’re seeing a couple things not so good though. First there are some little purple spots which look like ink splatters on Josie’s hands. They’re kind of hard to see and I tried to take a picture of them for your enjoyment. Not sure what those are all about but we should learn something tomorrow. Also her nail fold capillaries started to flair just a little today too. Nothing startling, but more Dermatomyositis activity. We’ll have to keep a close eye on that. We certainly do not want a big flare before the big surgery.

1/18 More of The Same

More home talk. Everyone is making certain that we’re really prepared about taking Josie home. Just because we go home doesn’t mean she’s well. Josie is still a really sick little girl and requires lots of care. We’ll be performing all that care the nurses do now but in a very different environment at home. I think we know what we’re in for. And the docs have made it clear that if things are just too tricky at home, they won’t lock the hospital doors. One of the docs said today, “…Josie belongs in a hospital. She doesn’t need to be in a hospital, but she belongs in a hospital…”

I would say most folks would not opt for going home with a child in Josie’s condition. But we think it’s time so we’re going to give it a go.

Josie walked today quite a bit. We were doing laps in out tiny room. We would walk around the bed with her holding my hand and the bed side, then we would walk back all on her own with no assistance. We did that a half dozen times or so. She’s slowly getting stronger.

Still a low fever, vomiting, tummy aches, not feeling well. But a lot of this day was feeling good.

1/17 More Home Talk

More of the same today. Josie felt OK much of the day and pretty crummy for about a third of the time. Some vomiting, low grade fever…you know the drill by now. More walking with the physical therapy girls…slow progress.

This morning the doc’s ordered up an EKG test and some other things because Josie’s blood potassium level was sky high. This can be a very serious problem. Of course after a recheck of the level everything was normal…but that was the third check. Turns out the Tech who took the blood did a poor job and botched the draw. The bigger problem was that the second draw was no better…both the first and second had high potassium levels and then they decided to order up the tests and treatment for the elevated potassium. It’s interesting because they’ve been supplementing her with potassium because she’s been deficient for a while…but today it’s off the charts. Well…the third time’s a charm I guess. The Tech this time got it right and the levels were just where they should be. They concluded the first two draws must have had some of the IV nutrition in the line which would have caused the reading. Whatever…just a few hundred more bucks or so for unneeded tests and treatment…but who’s counting…and we can’t be too careful now can we?

There was more discussion today about leaving for home. Deb is pressing to get it done for sometime next week. There are some real concerns about heading home and lots of coordination needs to be made with the home health care folks who will be visiting us regularly. The pumps need to be programmed, drugs need to be ordered and delivered, we need the training, we need to figure out our schedules and her environment. The docs are worried about all the bugs coming home from all the sickies at the kids’ school. So there are about a hundred legit reasons not to go home, and probably just as many to go home. She wants to, and we want her to. We’ll see how the coordination effort is going and over the weekend we’ll make the final decision. We may also decide to wait a little longer too if that makes sense. If we decide to pull the trigger on it they’ll need a couple days to finalize everything and then we can leave.

Josie’s big Whipple surgery is now on the schedule for Monday, February 19th. This can change as it has a couple times already but for now it’s scheduled so we’ve marked our calendars.

1/16 A Nice Day, Mostly

Josie made some great progress with physical therapy today. This was the first time she’s walked on her own without holding onto a hand or rail or chair or anything. She went about 10 difficult and uncomfortable feet but she did it. Her strength is slowly returning and that’s nothing but good news.

Her fever continued today but a little lower at around or less than 100°. Still searching for the answer. No therapeutic antibiotics for a few days now so it’s likely not a bug. Could be a fever brought on by a drug or drug cocktail. Same thing could be happening with her liver. Those liver levels are still high with no trend in the right direction. Her distended belly is just uncomfortable to look at, and I can only imagine what it must feel like to live with it.

We’d like to talk about doing away with some of her medication and/or make changes to her regimen. It’s simply staggering how many and the amount of drugs she’s had over the last 18 months and especially since August when we arrived. There were no issues with her liver when we got here or before. Her liver and spleen issues have slowly developed since we’ve been in the hospital. Lots of drugs being filtered by the liver, lots of IV nutrition being filtered by the liver. That liver’s a crazy organ, and a person certainly cannot live without a liver…and Dermatomyositis is not related with liver issues. There is also a slight hunch that the daily Cytoxan she’s been on which is so effective on her Dermatomyositis may be the cause of her liver issues. Therefore, on the thought that drugs may be a suspect, we’re going to shift off the daily Cytoxan and moving to Cellcept.

Cellcept suppresses the immune system and is commonly used as an anti-rejection drug for liver and heart and kidney transplants. Now is the best time we can give Cellcept a go and if it’s not effective with her Dermatomyositis then we’ll shelve it for good, because of several reasons, if it doesn’t work now it’s not going to work at all. We’ll see how it goes.

Also we’re going to see about stopping the preventive dose of Bactrim (a different antibiotic) which she’s been on for months. There’s a risk of infection with this plan, and not all the doc’s are on board with the idea, but it’s worth a try to see if there’ll be some sort of change in her condition.

Still some vomiting, still some tummy pain, but a lot of the day Josie was in a good mood and really fun to play with. Her progress is slow for sure, but encouraging.

1/15 Another Day

Here we are, another day under the belt, and we’re about in the same boat – nothing new to speak about. Feeling good then bad, low fever, stomach pain, snoozing, etc.

Josie did have a great physical therapy session today. She was walking (with assistance) a fair amount, and stood for over 10 minutes holding a rail for balance…not bad at all. A long way she’s come in that regard.

But a lot of the day she was holding the cup to her face much like yesterday and every day she feels bad.

If the rest of this week goes well, we’ll try to get Josie home early next week. We’ll see how that prediction holds up.

1/14 A bad and Uneventful Day.

Not much to say about today besides it was awful for Josie. She was feeling nauseas most of the day holding the cup to her face much of the time. Her fever was hovering around 100° from wake to nighty night. She was uninterested in anything, even watching Snow White or Dora, of course standing and walking were completely out of the question. Other than that really nothing happened today worth talking about. I could go into detail about poop texture and odor, or the vomit color, but I think the interest level wouldn’t justify the effort.

Talked with the GI guys and they're theorizing now that Josie does better and is better after her Friday IVIG and steroid bolus. I’ve not seen that pattern but I'll be looking from now on. She did do well yesterday, just one day after those meds, and that certainly jives with the hypothesis. Interesting theory and one which we'll watch carefully.

1/13 Ups and Downs

Not a lot of change today…much the same, except there was no appreciable fever for the first time in a long time. She felt kind of blue and crummy early on then perked up nicely in the late morning. Then a big 4 hour nap. She woke up for a sassy couple hours this evening, then a little turn south toward the end of the day. Her fever crept up above 100° for the first time all day

So it goes with the life of Josie these days. A series of ups and downs. The ups are growing and the downs are waning…it’s only taken a few months. I’d say she was feeling pretty good for 1/2 of the day which is good…better than before when she felt bad all day every day.

Infectious Disease was in and they liked what they saw on the CT scan…nothing remarkable. They want to take her off antibiotics since there’s no evidence of an abscess. Still CRP is up and all the fevers point to an infection, but there is no further supporting evidence. We’ll see how she responds to ending those drugs.

Surgery wants to see her on less pain medicine, and we agree. I think it’s good to challenge things. Josie is such a pain trooper that I think a little more discomfort as payment to get off those pain medicines is a fair price. Soon that discomfort should ease too and we can move toward a substantial weaning.

This week if all goes well we’ll have Evie and Trip tested for signs of any looming chicken pox outbreak. If they test out clean then we may press to get Josie home pretty soon, but as with every possible great thing we’ve planned for Josie over these last four months, something tends to come up – maybe we’ll get lucky this time.

Her bilirubin was a little lower today at 6.8 mg/dL. Not great but better. That liver is still a big concern for us. During Josie’s big Whipple surgery in February they plan to take a liver biopsy to give it a look under the microscope just to see if that’ll help them understand what the trouble is.

1/12 Feeling Better Tonight

Starting today was much like most, fever, retching, feeling crummy. As the day went on and as the day finished she felt much better. In fact tonight she is fervor free for the short term and feeling as spunky as she’s been since October. This is a good sign I think. These better times are becoming more frequent it seems and that’s exactly what we want to see.

Surgery was in and he said she is looking good and assured us that the fevers and distended belly are expected for folks in Josie’s condition.

The CT scan results are in and they look just fine…no abscesses…no fluids…everything looked just fine – normal! And that’s what we expected to find.

As for home…unlikely any time soon. The problem is still this chicken pox thing. Henry may have infected Evie and Trip near the same time as Josie. The incubation period for chicken pox is between 10 days and 28 days from exposure. So they’re in the same boat as Josie and suspected of having the virus until they either actually get it and get over it or the 28 days is up. They aren’t willing to expose Josie to them during the 28 days so we’re sort of stuck here.

Josie’s bilirubin is up again to 7.3 mg/dL (normal is 0.2 – 1.3 mg/dL) and has been steadily on the rise since November. Those yellow eyes and Mediterranean skin are an interesting combo. ALT and AST liver enzymes aren’t raising any longer, they’re holding at an elevated level. Still no explanation for the liver problems from GI.

Continued mystery.

But for now at least she’s feeling really good, and that’s great to see.

1/11 Questions

More fevers and more questions than answers. Still wondering what’s causing the fevers? Why is she so distended? Why does she have liver disease? Why are her drains flowing and then not flowing and then flowing again? Why are her blood inflammation markers high, yet they don’t jive with other lab values? Why is her spleen so swollen? Why is she vomiting a dozen or more times every day?

Josie had another CT scan today. The thought is that perhaps there is an abscess in there which might be causing the fevers…infection. No results yet. I’m predicting nothing remarkable…just like the others. This is good news…I think. It could also mean they might not be diagnosing some other freaky thing we don’t know about, but that thought’s nothing new - we’ve been thinking that off and on for a long time now.

We’re getting used to our sweet suite. Actually we’re not. We may move tomorrow to a slightly larger isolation room down the hall. Still, Josie’s going to be trapped in the hospital for at least until Feb 1. The only chance she would be allowed to leave is if Evie and Trip don’t show signs of the chicken pox, and of course if she’s feeling better and fit to go home…it’s a real judgment call and it could go either way.

Even though she had fevers this morning she was rarin’ to go. She played with the PT people for a long time and was all full of it. She was even talking to the nurses and everything. She was laughing and giggling, she pulled our Rheumaologist’s tie, she talked with the GI doc for the first time (I think ever)…she was really spunky. Not so tonight. She’s feeling like you would think she’d feel with a 102° fever. Not so good.

Looking forward to the new room…and the CT results.

1/10 We're All Giants

We’ve been moved to Josie’s new digs, and…Wow…it’s really something! We're in a bit of shock, but horror might be a better term.

This room totally bites the big one. I’m fine foregoing the great room for a normal one, but this isolation thing is for the birds. This room is a whopping 10’6"x11’ with just enough room for a bed and a chair and about two people…any more than that and it feels like a frat party. This is just about the size of the bathroom from our last place.

This is a serious stroke of bad luck for Josie. Henry just happened to be visiting when an ID doc took a quick peek at his skin as a favor. Well that let the cat out of the bag and the ID containment people love to freak out at the hint of a new bug. Josie’s exposure was minimal, and she’s been immunized, and she’s shown no symptoms, yet here we are in a storage closet for three weeks because of their cover-your-a__ policy…all the while the ID team saunters in and out of rooms with infected kids tracking bugs around all over the hospital. Sure they gown up and glove up, but they don’t put on pant covers, or shoe covers, or hats or stop breathing…and they don’t wash their faces when they leave. So I’m a little ticked at the hypocrisy of Josie’s isolation because of a seriously low probability of her infecting the hospital with chicken pox – but we can’t be too careful now can we. If only Henry’s visit were five minutes later. Now Henry’s all cleared up and Josie’s showing no signs of the virus. And I’ll be the very first the eat a big crow feast if she does come down with it. Of course because of the isolation we can’t have the other kids come visit any more (like there’d be room any way).

But other than that…we love it here! Everything is within arm’s length. I just hope I don’t have nightmares and wake up thinking I’m in the closet under the stairs on Privet Drive. Uncle Vernon’s got us pinned down. I’m hopin’ Hagrid comes to the rescue. But then I’ll really wake up and be in this real hospital isolation room which is better suited for a broom closet. In fact, I think the broom closet is actually bigger than this. - - -Enough of my rant already…

Josie’s feeling much the same today with the fever and high heart rate and the vomiting. Her bilirubin has crept above 7 now and is showing no signs of slowing. There’s still no clear-cut reason for the liver disease. We’re just hopeful that Josie’s liver cells are regenerating in the right architecture which would make for smooth sailing when she’s better, otherwise she’ll have cirrhosis where the liver replaces good tissue with fibrotic scar tissue…not good…but that worry’s way on down the road.

Josie’s Dermatomyositis is coming along nicely. Her symptoms are really improving and it appears the disease is being nicely held at bay, though it’s still active. Her dose of steroids has been dropped to a miniscule 1mg per day. She’s still getting her weekly or bi-weekly 375ml pulses but the daily has been well weaned and should end sometime soon. The surgeons are happy about that because the steroids are suppressing Josie’s ability to heal well. This is good news.

So now that we’re in this walled postage stamp we’re starting to put some real pressure on them to let Josie go home. We had lobbied for this back in October before the big surgeries but haven’t felt the serious home itch, nor thought it a good idea until now. We were all much more comfy in the other room…it was a fine place for Josie to get better with such a great environment. This room at 115 square feet really does blow — big time — and we’re certain home would be better for Josie than this spec of a room (it does have a lovely view of the Mississippi River gorge though, all griping aside).

1/9 More of the Same Again

103° overnight prompted more blood culture draws. They stopped her Vancomycin (one of her antibiotics) two days ago because its course is done. Josie does have a history of getting sicker after stopping Vanco when she’s been on it in the past, so we’re not too surprised.

What's making her spike these fevers and have the higher heart rate? This is a real mystery as y'all have read so many times in the past. Could be an undetected abscess, could be all the guts being manhandled during her previous surgeries, could be smoldering sepsis, it might be her body reacting to her new anatomy and the foreign bodies in her...idunno. Her higher fever today prompted another culture draw which led to some discussions about the need for so many cultures every time she has a fever. That fever has been there for so long and there have been dozens of cultures drawn with all but a few negative, and those positives could easily have been contaminates. Anyway, we agreed that they would not order cultures unless she demonstrates symptoms not normal to her pattern of fevers and high heart rate. Otherwise they’ll just let her keep her blood. So for Josie…102° and a 160 heart rate is now considered kind of normal. 98.6° and 95 heart rate are not normal.

Surgery stopped in and thought Josie looked pretty good. They’re now talking about mid February for the Whipplization, but we’ll see when she actually gets on the surgery schedule. They too were in agreement that we should back off on all the blood draws.

Physical Therapy was in and they took her down to the PT room for some exercise. She was swinging and throwing stuff, playing and walking. She took quite a few steps today which is a big deal for Josie. She has yet to get walking on her own but she’s doing it without much assistance which is way better than before…but she protests big-time before she does it. And that’s nothing new.

Only one more day on our great room, unless the infectious disease containment people have a miraculous change of heart…what do you thing our chances are with that?

1/9 Not much change

Not much change today…a lot more of the same. Hurting tummy, fever, throwing up, sloth, no desire to work out, trouble walking…pretty much a not-too-fun day. Her hemoglobin was low today at 8.6 gm/dL…a full two grams below normal, and she was transfused just yesterday. They didn’t give her too much volume for blood yesterday and that’s the reason for the low hemoglobin. The docs are still comfortable with anything over 8 for sure and get more concerned as it approaches 7. She had two more blood cultures drawn because of the 102° temp twice.

Not a lot of action from the docs today. Infectious disease came in and pretty much said we're done with this room because of Henry's chicken pox. Even though there was little going on…where did the day go? Time seems to vanish while in the hospital for some reason. It only takes a couple hours to endure six it seems a lot of the time.

We’re gett’n real psyched for our new room. It’s gonna be so fun existing in 1/3 the space, ugh. And we’re lucky enough to have it until February…isn’t that great?!

1/7 Holding Pattern

Nothing too scary is happening. We’re in a bit of a holding pattern. There are some good moments sandwiched between the vomiting and retching and more bad-feeling episodes. This morning Josie was up and at ‘em reading books and chatty, but in the afternoon that was replaced by a hurting tummy and no interest in doing anything fun. Lots of tummy complaints and a need for additional pain medicine. Then later in the evening she was better, but still had a 101.4° fever and 160 heart rate.

Josie’s hemoglobin was down again today to 7.4 gm/dL. They decided to not wait any longer so they gave her a transfusion of fresh packed red blood cells. I missed my predicted infusion forecast by over a week. Maybe I shouldn’t become a doctor after all. New prediction…one more infusion on the 25th.

GI was by and decided that with her recurring infections and because of the continued refluxing and draining feeds, even at a sub-nutritionally-optimal dose, that Josie will need to go back onto TPN (IV nutrition) starting tomorrow. They’re afraid she’s simply not getting enough calories with the feeds which are actually entering and going through her system. Once the TPN starts they’ll then cut her feeds to a point where she doesn’t appear to be refluxing any longer.

GI also wants to do another nuclear study on her liver function this time. They want to see her bile production process and then where that bile is going. It doesn’t appear Josie’s bile is going anywhere because the drainage out the JP surgical drain and the duodenal drain is not bilious…just looks like gastric juices and feeds. This study will help rule in or out a bunch of things and confirm if her bile issues are causing her bilirubin levels to remain high. Makes me wonder if this kind of test should have been administered a while ago or if this is just window-dressing information.

More tomorrow.

1/6 Back to Normal -- Feeling Pretty Bad

Low key was the rule of the day today. After such a great evening and overnight Josie slept in until after 10:00 (along with me) and awoke with a little fever again. The fever persisted and rose to over 101° later in the day. Her heart rate was back up a little to the mid 140s later too. I knew it would likely not last, but it was sure nice to see her feel so good last evening. She’s still OK but is feeling pretty crummy with more abdominal pai

Her vomiting was back several times again today as well. Our GI team pointed out the obvious reason why she’s been vomiting forever. It’s actually quite simple. Josie’s body doesn’t like all the business (drains, sutures, rerouting, etc.) going on within her upper GI tract. Her GI tract is angry and is telling her brain stem such, the brain stem is defending the body by assuming the problem is something she ate. The body’s natural defense reaction is to vomit whatever you’ve eaten right out. They’ve said it before and are sticking with the prediction that she’ll likely have these barfing episodes until her GI tract is put back together.

She wasn’t in the most cooperative moods for activity so bike rides and ventures outdoors were out. Just hangin’ with Mom was the order of today. There was really nothing else remarkable happening…so slowly we just slowly press slowly right along and slowly Josie slowly keeps slowly getting slowly stronger…s-l-o-w-l-y…every day…did I mention she was taking her time?

1/5 A Good Ending

Like so many days in the past two + months, this one again started the same…high fever, retching and vomiting. But this day has a little bit better ending and maybe a sign of things to come?

All morning Josie’s attitude was in the tank. She wasn’t up for much of anything. She was crabby and uncooperative. Physical Therapy came by and they tried getting her down to the PT room on her bike just like yesterday. She was a reluctant participant at best and was being a real pickle.

After that ordeal she was back in the room and had a good nap.

Infectious Disease was in and wanted to discuss what happened last night and that event’s related consequences. See our boy Henry has been a little under the weather lately with a fever and sore throat…he was diagnosed with strep just two days ago. He had been on his antibiotics for more than a day so his contagiousness had past. Well Deb noticed a little rash on the boy’s trunk and while he was visiting the hospital last night he lifted his shirt to reveal the rash which startlingly appeared like what we immediately feared. Coincidentally the Infectious Disease doc happened to be on the floor during Henry’s visit and came in for a look-see. Henry has chicken pox…NICE! All our kids, including Josie have been immunized for chicken pox, but it’s not uncommon for an immunized kid to come down with it.

Of course the ID people never miss a chance to be overly cautious and it looks as though Josie will have to go into isolation next week when she would become contagious herself obviously assuming she’ll most certainly come down with it. So it looks as though we’ll be moving to an isolation room because the air in non-isolation rooms is circulated throughout the hospital just like every other room and hallway. Isolation rooms though have their own isolated air circulation. Assuming there are no changes in their thinking it looks as though our days in our lovely room are numbered. We’ll make the most of it while we still can.

After Josie’s nap her fever finally broke. It was normal for the first time in a long time. Her heart rate was in the 110s. She was feeling good, perky, laughing, agreeable, and just a joy to be around. She clearly felt as good as she has for a couple months now. And as I write I’m checking her out and she’s still at a normal temperature and a heart rate of 94 bpm. Maybe she’ll get some good sound rest tonight.

1/4 More Fun

Another interesting day, which started out much like the last. Josie’s fever this morning was 103.9° and a heart rate of 180 bpm. The old picc’s been gone for a couple days now and the penicillin has been on board since the new cultured bug was identified a few days back too…yet the fever is still with Josie.

Infectious Disease is unconvinced that the new bug not a contaminate. That’s a double negative…but in this case it doesn’t make a positive. It doesn’t mean they are convinced that it is a contaminate either. And that means…we really don’t know for sure what’s going on. We’ve been down the road a couple times of stopping her antibiotics because everyone was fairly certain the bugs were gone only to have Josie regress badly a couple days later. It’s really frustrating not getting clear causes of Josie’s symptoms.

GI was in and they seem to be leaning toward an undiscovered yet perpetual abscess. They think this because her altered anatomy has two big sections of GI tract open to her abdomen. For sure there has been containment by walling off and we’ve seen this with some of the images, but if a developing abscess has not yet walled itself off it’s not easily detected with imaging techniques. Another abdominal ultrasound today revealed nothing remarkable…no walled off abscess and no notable fluid collections. After 10 weeks since the surgery I’m not sure I’m buying into this theory, but it seems as good as any. Bottom line is that Josie needs to stay on the antibiotics for what seems to be a smoldering infection of some kind.

GI also commented again on the liver issues and assure us things should be OK. Her altered anatomy is no longer draining bile so that bile is backing up somewhere, which is the reason for her elevated bilirubin and enzyme levels. Once her plumbing is Whipplized and re routed back to one tract then the liver will drain its produced bile freely, clear out the built-up sludge, repair itself and become healthy once again. Livers can take a serious beating and bounce back like a champ.

Another noteworthy surprise this afternoon was to find her big duodenal drain had slid out of her body by about 1 ½ inches(!). This was shocking. The sutures holding that onto her skin all have pulled out of her skin finally after the 10 weeks of constant tugging. The tube started sliding out. This would have been a real bummer if it came all the way out. I slid the bugger slowly back in, and Josie found this fairly uncomfortable (understatement) and taped it down to her skin…you know…guys with tape can fix just about anything. Surgery went to Josie’s room tonight and re sutured the drain back in place exactly like sewing a button onto a coat, except this was a tube flange sewed into Josie’s skin (sorry, I didn’t have the camera for that – maybe tomorrow).

Fevers persisted throughout the day with a heart rate of 150 to 160. It can’t feel too good but her spirits seems genuinely bright at times. She rode her bike a long way this afternoon and continues to make positive strides despite everything else.

1/3 Down Day

Today Josie actually felt like her symptoms would dictate she should with a fever of 102° all day with retching and vomiting episodes. She was flat, uninterested in any talk, and certainly not a great playmate today. She just laid in bed and slept much of the day feeling bad, bad, bad.

Infectious Disease isn’t convinced they know the whole story with Josie’s bug issues. There have been no further positive blood cultures, but they’re leaning toward a smoldering infection in there somewhere. Her hemoglobin today was 8.7 gm/dL. They’re going to hold off on giving her blood this time. They want to wait and see how her bone marrow will react to a low blood cell count. It should trigger the marrow to kick into overdrive producing cells…so we’ll wait it out for a couple days to test her.

GI was in and we talked a little more about her liver and associated issues. Her lab work showed little improvement with liver levels. ALT and AST are marginally higher, but her bilirubin is up to 6.8 today from 6.2 yesterday and below 6 previously. They’re convinced that Josie’s altered upper GI state now with the drains and stuff are contributing to the liver problems. They seem confidant that after Josie’s Whipple procedure and everything not supposed to be there is out of there and her GI tract is once again in one piece things will settle nicely with the liver.

Surgery has decided to wait until after February 5th to perform the big surgery. No date set, but they thought it would be good to wait it out a couple more weeks.

Josie did reluctantly and non-enthusiastically get on her bike again today. As expected she half-heartedly and slowly pedaled softly and performed poorly. I keep looking at this like the stock market. It’s tough to watch on a daily basis because of the volatile ups and downs, but generally over time it always moves in the right direction, and usually at a very slow pace.

So today was a down day. There’ll be lots more good days ahead.

1/2 A New Picc

Today was an interesting one. After Josie’s blood cultures grew out bacteria on the 28th the docs decided enough was enough and the picc had to go. Today was the day. She had her feeds stopped at 6:00 AM for the noon procedure. She was taken down to the pre-operating room at 1:00PM (not noon) and waited around there for 45 minutes before the anesthesiologist came to visit. We chit chatted a bit and then we were off to the OR.

She was to be under general anesthesia today for the catheter replacement. This seems like overkill and risky since the procedure is really not any huge deal. But the problem is Josie’s suppressed immune system. If she were under regular sedation, and with her normal history of vomiting and retching, there was a descent chance she might ralph during sedation and aspirate a little bit. This would likely mean bad gut bugs would enter her lungs and potentially cause all kinds of trouble, including pneumonia. It was a no-brainer decision to go with general and have a breathing tube inserted for the deal.

I gowned up and walked into the operating room with Josie. I moved her to the table and she was holding my finger chatting away making sure I had the Kleenex box, and her cup, and her lip gloss (?). The anesthesiologist took out his collection of syringes and started administering them. Josie was as cool a customer anyone could want. She was asking about the stuff that makes her nose itch (remembering from last time). She kept telling me she loved me and winking at me while she was as calm as could be. Then her eyes started looking real heavy. She said with the biggest Garfield eyes ever “…I can still see you!...” and then it was lights out. All the docs and nurses got a kick out of it.

An hour or so later they gave a call and I went down to the recovery where she was certainly an unhappy camper…but a minute in good ol’ Dad’s lap took care of everything and we were back in business. They wheeled us back to her room and that was that.

Aside from the picc replacement Josie had a pretty good day, generally speaking. She had some sinking spells, but not too bad. Her heart rate was above 150 most of the day and even after returning form the picc procedure she was almost 102° never falling below 100°. They started her on penicillin today to cover the new bug so let’s hope that fever and infection finally can clear.

Josie’s labs today were still showing signs of liver disease. ALT and AST still high, bilirubin is higher today again at 6.2. Her pancreatic enzymes continue to be normal and have been for a long time now. Her hemoglobin (the iron-containing oxygen-transport protein in red blood cells) was down again, probably caused by blood taken for lab draws. Josie should be getting some more blood here in the next day or so.

Speaking of blood infusions…there have been quite a few people who have been donating blood on Josie’s behalf. For that we whole heartedly thank you…what a great gift that is. Donating blood is easy and it’s something most everyone can do. The blood bank is always in need of blood, so if you’ve got nothing to do some afternoon then head on down to the blood bank and give a pint. Someone out there will thank you. Luckily Josie has a well-supplied blood type.

It was a so-so day.

1/1/2007 Another Blood Infection

It could be another contaminate, but maybe not. Josie’s blood cultures from December 28th grew out today indicating another blood infection. This means more antibiotics and a need to get rid of that picc line as a precaution. They want to take it out tomorrow morning sometime and then replace it. But they want to insert it at a different site rather than the same entry point. They were just going to insert a wire through the old picc and slide the line out over the wire and insert the new line the same way in the same hole. The current entry is looking a little red and they want to give that a rest so now it’ll be more or less a fresh new picc insertion procedure.

Josie took another walk outside today with Deb and it was great until we had a little accident. They bundled up and headed out with the pole and IVs going…no problem. They were enjoying this lovely winter’s day, bright blue sky, light wind, temperature of about 25°. They were just walking along, minding their own business when it happened. One of Josie’s IV lines got pulled somehow and it snapped right in half…right in the middle of the tube! I guess the tubing is not made to be in freezing temperatures for very long. It became hard and inflexible…and just a little tug just broke it right off. This was a little problem because blood started running out of the tube…of course that was a mess…but more of a problem was that blood was now in the picc and would clot it shut if not flushed right away. Not a problem except they were a 10 minute walk from any hope of a flush – this was indeed a problem.

They got back to the room and the nurse tried to flush, but of course it was blocked. A call to the good folks at Vascular Access and they came to the rescue. They threw in a little TPA (clot buster) and let that work for an hour or so, and then we were back in business. Lesson: If going for a walk outside, turn off the IVs and leave the pole in the room …at least until spring (we certainly won’t be here that long, right?).

Other than that, it was much the same today…fever between 100° and 101.9°. Lots of retching and vomiting again. It sounds as though that pattern may not resolve until after her surgery is complete and her guts are hooked up proper again. With the 101.9° fever the docs decided they wanted another blood culture. That means more blood from both ports of the picc and a sample from her vein direct. She is such a Champ when it comes to vein pokes. She just watches them stick the needle directly into her hand and doesn’t even flinch, or make a face or anything. Very impressive. And no one should be that immune to needle pokes. Our Champ indeed.

12/31 Happy New Year

This was sure a year of challenge for Josie and our family. One we wish never would have happened the way it did, but one which seemed to bring much unexpected joy. Seems weird to think joy can come from such a stressful and challenging time with Josie’s whole deal, but Josie’s whole deal…the experiences we’ve had – the life experiences, and the positive people we’ve met and the support we’ve been shown by so many are all unexpected and welcome.

Today Josie was able to get out of bed again and have a spin on a little bike down the halls. Notice in the picture there is no medicine pole in sight! We found a nice little window of opportunity where Josie would have no medication scheduled and we disconnected her from everything so she was able to get out and cruise untethered. She was reluctant at first but came around and seemed to enjoy the whirl around the floor.

Her liver labs look a little better yet again today and hopefully it’ll be a real trend over the coming days and weeks. There’s been a lot of talk about actual healing coming from getting back into a nice non-hospital routine. The new room, and the walk outside and the shower yesterday and the bike ride today are all the kinds of experiences which can have a healing effect better than drugs in the veins.

One of Josie’s doctors came and sat with her so the family could get out this New Year’s Evening. The five of us went out bowling and then to a friend’s house after. What fun that was. Bowling on New Year’s Eve seems to be the thing to do. You ought to give it a go sometime.

When we got back to the room tonight Josie seemed to be stepping back a little bit with a 101° fever, retching and a 180 bpm heart rate. Another speed bump in the road it seems. They’ve given her a bolus of fluids thinking she may just be a little dehydrated. Sounds bad, and it’s certainly not fun, but I’ll take days like this with way more ups than downs over the days of a month or two ago. Still lots of pain, but better.

So another year begins for Josie much like the last one…a promise of pain and challenge and success and failure and healing and joy. We’re banking on the latter two.