12/30 A Lazy Family Day

The nice thing about Josie’s new hospital room is that our whole family can come and hang out…as chaotic as that sounds…with relative ease. There’s no way we could all be in the same room together before for more than a half hour. It really makes it nice. Now, I’m not certain how much fun everyone is having here, but there’s no real itch for any of them to leave…in fact they seem to want to spend lots of time here. Perhaps it’s all the room, or the fancy lights, or the multiple PCs, or the giant flat panel TV, or the Xbox-360…(do ya think?). Whatever it is, everyone’s responding well to the room.

Josie had another ultrasound on her kidneys this morning. We’ve not seen any results on that yet, but the tech sure seemed unconcerned about anything she saw, and just like all the other tests…this one’ll likely turn out to be just fine again. Other than that, Josie was seen by the GI team today. They ordered up another set of blood cultures because her temperature went up again to over 100.5°. Before that and for most of the morning Josie’s fever was pretty much absent for the first time in a while. Also, her heart rate this morning dipped below 100…we haven’t seen that in months!

Josie was feeling well enough to get out of bed and do some coloring while sitting in her little seat on the floor. It’s so nice to get out of that bed. She also let Deb give her a shower -- a real live shower, so that was pretty cool. Next she got all cleaned up and was looking like a $million. But it wasn’t long before she was in a funk again with a high heart rate and a higher fever.

Her lab work today showed improved values for her liver. ALT, AST and bilirubin were all down a little bit. Dr Vehe, our rheumatologist, is still a little concerned that the daily Cytoxan Josie’s on is contributing in some way to the liver problem. There is very very little research or case reports tying liver problems with Cytoxan though. We’re still struggling with a cause for Josie’s liver and spleen issues. And even though the report from the CT the other day said not abnormal and the nuclear test revealed normal function, the CT showed the spleen and liver much bigger than a couple months ago.

The kids were here for most of the day. Evie and Trip are spending the night here in the room with Josie and me. Should make for a very crazy New Year’s Eve. We’re planning on getting out and having a little fun tomorrow for a night of bowling with the kids. One of Josie’s doctors has offered to sit with her tomorrow so our family can go out and have some enjoyment. And speaking of New Years, we all hope that 2007 is a better year for each and every one. Have a safe holiday and great success with everything in your lives for 2007.

12/29 A lovely Day For A Walk

The results of the Liver-Spleen Radionuclide Scan from yesterday came back this morning and guess what they showed…yeah, you got it right…perfectly normal function for both liver and spleen. I’m feeling a little like Thomas Edison who tried and failed a thousand times before making a functioning light bulb. Every time he failed he would consider it a success by learning another way not to build a light bulb. Now we have another failure to find answers, but now we have learned more about Josie’s well functioning parts.

Renal was in and they want to do a couple tests over the next week or so. They were called in to check why Josie’s blood pressure has been so high. No good explanation so far.

Had a good talk with Surgery today and they seem happy with Josie’s progress…and this is about what they expected…a very long recovery with ups and downs. That certainly fits the description for Josie’s recovery from that big surgery two months ago.

Her liver labs are still pretty bad, with ALT, AST and bilirubin are all still high and holding steady.

Josie looked outside the window this gloomy winter day and decided she wanted to head outside. Why not Mom said. Josie got all bundled up and headed out for a nice cool walk. Not really cold, but cool. Seems winter has forgotten about us in Minnesota. They walked a bit and it was so nice for Josie to feel the cool breeze on her skin. It’s been almost three months since Josie’s been outside and you can tell by the twinkle in her eyes that she enjoyed it. Josie’s excited to get out of the hospital and get skating on our rink. I guess that means I need to get the rink operational…and I’m certain the other kids want that to get underway too. Just waiting on the cold.

12/28 Nuclear Medicine

Another mediocre day for Josie. It started out pretty nice with a great night’s sleep with only one time awake. She’s still a real peach though when she wakes up in the middle of the night. No pulling anything on Josie. When it’s time for vital signs and the nurse approaches the her in not quite the right way, Josie’ll absolutely not allow anyone aside from good ol’ Dadio to do anything. So I’m joyfully roused out of a lovely slumber at the girl’s emphatic insistence that Dad put on the blood pressure cuff, and take the temperature, and take the BP cuff off, and change the poopy diaper, and get more water, and get a tissue, and get a cup, and get the blanket on the right way, and itch the feet, and rub the back, and give a hug and kiss. All just fine by me…accept for the waking up part. By the end of all that I’m wide awake and ready to start the day…but not really.

Josie’s fever came and went today between 99° and 100.5°. Still a concern. Her blood work is looking not the greatest. ALT and AST (liver enzymes) are higher again, and her bilirubin is at 6.3. The seasoned GI liver expert is unimpressed by her liver thinking it’s not that big a deal, and relatively speaking it probably isn’t compared to the crazy-bad liver problems they’ve seen in the past. We just want her liver to start showing signs of improvement, not further trouble. She’s been off TPN now for a couple weeks so we would have expected some improvement in her liver, but it’s only getting slowly worse. Still not critical or even urgent, but a brow frowner and head scratcher for sure.

They wanted to replace her picc (Peripherally inserted central catheter) line today because they’re thinking there’s a bug which has colonized on the line itself. The synthetic material of a picc is a prime place where these bugs like to live…and once they’re on it, there’s no getting ‘em off. The theory sounds good…she’s had lots of infections, and it’s well known piccs are a source of many recurring infections. Her blood cultures do not support this though. She gets a positive culture then nothing then a positive then nothing then her picc is replaced then a new bug and the drum beats on. A new picc would require further sedation and we’re not convinced enough that this isn’t just chasing ghosts. So rather than risk the rather non-risky sedation and re-piccing, we decided to pass this time.

That led to her big nuclear test today. This afternoon they took her to the lab for a Liver-Spleen Radionuclide Scan. That guy was there again. Remember…the guy with the lead tube? That lead tube…he unscrews is and inside is the big syringe with the bright orange radioactive sign on it…takes it out and injects the thing right into Josie’s arm. That, for some strange reason…and I can’t quite put my finger on it…just doesn’t seem right.

There she lay for about 45 minutes or so while the huge machine took lots of pictures of her spleen and liver. They want to track that radioactive stuff through those organs to see how they’re functioning. Again they’re trying to determine if the spleen is malfunctioning.

Here’s your medical lesson for today for you lay folk out there like me who don’t really know what the heck a spleen is (but don’t feel to thick because until recently it was not known what the spleen really does):

The spleen is a ductless organ that is closely associated with the circulatory system, where it functions in the destruction of old red blood cells in holding a reservoir of blood. It is regarded as one of the centers of activity of the reticuloendothelial system (part of the immune system). The spleen produces lymphocytes; it is the largest lymphatic organ in the body. One can live quite well without a spleen, but it’s increasingly recognized that its absence leads to a predisposition to certain infections.

Results of Josie’s test will be reported sometime tomorrow.

The rest of today Josie was feeling just OK, not great but not horrible…much the same.

12/27 Feeling Better

Today was another of the up-and-down kind for Josie. She was up only once in the night with a retching/vomiting episode. Her fever had been less than 100° all night and in the morning it spiked up to 101.3°. This of course meant a whole new concern of sepsis, and that meant more cultures sent to the lab for testing. We'll see what that turns up tomorrow.

She was up and at ‘em working with the physical therapy folks with good success this morning. They got her out of bad and standing a little bit and even taking a few steps. All good news. Josie was sitting up for a couple hours today watching some TV, playing games and coloring. She was chatting away to everyone it seemed…and there were lots and lots of people stopping by. Doctors and visitors and therapists galore. Not a dull moment around here today.

Josie slid back a little tonight with a slight temperature and more stomach pain, but the pain intensity has decreased. She’s not taken much of the as-needed additional pain medication lately and this is nice to see. She’s still getting her scheduled Methadone but not needing nearly as much additional hits as she was a few weeks ago.

The good folks here are looking at a new study on Josie’s liver and spleen to measure function. There’s a though that the spleen may not be operating as well as it might otherwise. The thinking says it may be grabbing red blood cells and destroying them. This is pure theory now. We’ve been seeing continued slow decrease in hemoglobin. Indeed lots of blood is being taken for testing but she should be able to handle and replenish those draws. We’ll see what this now study finds, and, of course, the more logical question…what are we going to do with this information once gathered?…probably nothing, but at least it should give us more information.

In the meantime we’re still hanging out here at the hospital hoping to heal more and more. Looks like things are ever so slowly headed in that direction.

12/26 Another Day...

Josie’s day was pretty mellow with some good progress and some changes too. This morning her feedings were stopped because she was to get a CT scan later in the day. She was up and sitting in her chair coloring for at least an hour this morning…could have gone longer but decided it would be more fun to relax and veg in front of her huge TV set. We weighed her a little later and she stood on her own holding onto the scale bars -- the first time that’s happened since October. We worked on assisted standing with bent knees too trying to increase the strength in her legs. She doesn’t like exercise much at all but she can actually be reasoned with and convinced that it’s good for her.

Later on Josie went in for another CT scan of her abdomen. The last one was December 2nd and they wanted to check for anything new, like abscesses or gas or bowel enlargement. The results were just like before…nothing abnormal (aside form her missing intestine section and all the tubes). This is god news I think…part of me wanted the bad news which would explain her very uncomfortable distention.

Surgery was in and thought the source of her distention is simply inflammation of her guts being manhandled during her previous three surgeries. This will slowly decrease over time, so the thought goes. There’s still not a definite date for Josie’s looming surgery. That should be figured by week’s end.

GI was in and decided to cut her feeding to 30mL per hour down from 55mL per hour. They’re thinking her gut is only moving so fast and pumping more stuff in there is kind of like pumping more play-dough through the toy and it squirts out any place it can…and in Josie’s case it’s the JP drain, duodenal drain and back up her stomach through the G tube and through vomit – simple hydraulics, right?. We’ll see if that has any effect on her output over the coming days.

Josie’s bilirubin level has jumped again to 6.3 – way higher than the 5.1 from yesterday. She had a urine analysis done yesterday too to check for electrolytes among other things. The bilirubin level in her urine colored it a lovely shade of orange…a deep tangerine hue. Her liver enzyme levels were up again too…definite brow frowner. Time should take care of her liver problems or so say the liver experts.

There’s no other plan than to get her on her feet more to gain strength. Activity will help on all fronts we’re told.

12/25 Isn’t Christmas Great?

It was a wonderful Christmas morning here at the hospital. Deb and the kids arrived with armfuls of gifts from Santa. We opened our gifts beneath our little tree here and had a wonderful time. Josie didn’t feel the best but she was strong enough to sit upright for about an hour before needing to get back into bed. Grandma and Grandpa came to visit during present time and enjoyed the kids enjoying Christmas. It just doesn’t matter where it is, Christmas is still pretty cool.

The remainder of the day was not as fun for Josie. She had more vomiting spells and sore-tummy spells. Her fever continued at about 100°. Her liver enzyme levels are hovering about the same as yesterday, hemoglobin is steady, white cell count is a little higher but within normal, bilirubin is a little lower but still steady on high. Her general look is shocking with her distension. Her belly is so big it looks like she’s gonna pop. With feeds refluxing everywhere, gut blowing up like a balloon, and pooping almost hourly… I get the feeling that she’s just being overfed. I’ve got nothing to back that one up though and likely get funny looks from the docs, but in my dime-store opinion I think that theory has as good a chance as any other at being correct. Her potassium has been very low for some unknown reason so they’ve been feeding IV potassium supplements. Josie’s urine is very concentrated too – a dark orange. We sent off a sample for analysis, as well as a stool sample was sent to check on electrolytes and other nutrition information.

GI was in and we talked a little about reducing feeds but nothing was changed yet. Also we discussed further imaging possibilities like another CT scan or ultrasound to check that abdomen again. X-rays showed no free air so she’s not gassy (wish I could say that for myself though). Still lots of theories why so big and uncomfortable in the gut but no definitive answers. Time, time and more time…more waiting and watching. There was no word from surgery on a hard date for Josie’s surgery.

Christmas was great, and not as bad as I thought it might be spending it here. Josie had a good day and the rest of us had a great day.

12/24 Merry Christmas

I would have never imagined back on August 30th that Josie would be spending Christmas 2006 in a hospital room. The good news is that Josie’s stay has become a little more comfortable and her stay a little more bearable with such incredible surroundings in her new room. The better news is that Santa Clause knows exactly where she is, along with all the other awesome children out there…and you all know who you are.

Josie’s night last night was filled with hourly vomiting and restless sleep. That fever of hers is still smoldering at 100° to 101°. We had visits from Surgery, Rheumatology, Infectious Disease, Gastroenterology, and Renal today…no Pain Team in sight. This is the first time Josie has seen the Renal Team in a long while. She’s been experiencing a fair amount of hypertension (high blood pressure) lately and Renal are the guys to call on that issue. Josie’s hypertension has been a problem since shortly after she arrived and she’s been on and off blood pressure medication to help since. The drugs seem to be doing their job, but no one’s really certain about the cause of her blood pressure. Renal had no good answers either.

Her vomiting spells became less frequent later and she generally felt a little better. Her heart rate has come down a little too, but that fever still persists. The GI team is concerned that she’s leaking out too much of her feeds from the wrong places. I must admit, there’s a lot of stuff coming from her G tube, JP drain and duodenal drain. All three are showing feeds coming out. The question is how much is coming out, and also, of the stuff moving through, how much is being absorbed? GI thinks Josie is not getting quite enough adequate nutrition because of the leaking food (and perhaps poor absorption) so they’ve decided to add some IV lipids to her diet for the time being.

As bad as it is to be in a hospital for this long, Josie’s in a better state than just a few weeks ago, and certainly from two months ago. And even though she’s suffering through a tough situation and grueling constant pain, she’s getting better. For that we’re grateful. Grateful for all the doctors and nurses dedicated to Josie’s care, grateful for Josie and her amazing spirit and unending endurance, and of course we’re grateful for everyone out there wishing us well. The amount of support we continue to receive from everyone is just amazing. We read all the blog posts and email. We’re humbled by all the generosity shown to us at Josie’s benefit party, the meals, the rides, the phone calls and visits, the thoughts and prayers…everything. We feel the spirit from all of you lifting Josie toward better health.

So I sit here this Christmas Evening watching my young daughter sleeping away, thinking of the excited dream of all the kids big and small this night. A smile is on my face knowing Christmas is here and so is Josie. What a gift that is to us indeed.

12/23 Move Day

Josie finally moved to the floor, just in time for Christmas! It’s been determined that she’s well enough to leave the Intensive Care Unit after 61 days. But is she different today than yesterday? Not really. Her blood work results are mixed: liver enzymes up slightly, bilirubin down slightly, white blood cell count way down to below normal, pancreatic enzymes are normal with no change.

Josie had another up and down day. This morning she was really looking and feeling great but that soured after we moved and this evening she’s been back to her flat barfy self. “…No fun…no faun at…” all is what she says to me after each vomiting spell. And it certainly looks like no fun at all.

Not much new information on the surgical or GI front. I do think her pain is getting a little lighter these days. I think time has slowly, slowly eased the pain and it’s getting a little better so she doesn’t need so much stuff to keep her uncomfortable (as opposed to in big pain) any longer. This change in pain, even though slight seems to have had nothing to do with the Pain Team’s intervention whatsoever. Just Josie’s body taking care of business.

Further on this evening Josie has been vomiting quite a bit…probably 10 times during the last 1/3rd of today. Her heart rate has jumped up to 180 with a fever of 101°. They slowed her fluid replacement today because her weight had been increasing a little bit over the last few days. They think that she’s become a little dehydrated and are now pumping her with a big slug of saline to try and reverse it. No biggie.

We were fortunate to get an added Christmas gift on top of Josie moving from the ICU. We were admitted into one of the super-duper fancy-pants new rooms here. This room isn’t just fancy, it’s FANCY. It’s about three times bigger than the normal rooms, there’s a business area with a computer with web access and a printer, a fridge, microwave, entertainment center like you’ve never imagined a hospital would have. A 55” LCD TV plus two additional 23” LCD TVs to see what’s on a different channel and what’s going on outside. There’s a video camera mounted on the roof that you can maneuver with a remote to look around. The lighting in here is amazing with a big sky-light screen in the celing providing mood-color changes which you can program or completely control. There’s an XBOX-360 game console in here too. It’s absolutely unbelievable what they’ve done with this room. Learn more about these special rooms at http://www.adoptaroom.org/. Staying in this room will definitely make the stay here far more tolerable than otherwise. If we have to be here, this is the way to do it.

12/22 On The Schedule

Josie’s doing better today. Her fever is down and her heart rate is down too. She feels better and generally looks and acts better as well. All good. Her physical therapy today went great…she was standing on her own for about five whole minutes while doing some craft thing…five minutes! Considering last week she was barely able to stand at all…this is a super improvement. She was chatty and generally happy…at least during the part of the day when she was awake.

Up early…about 4:00 AM again…fell asleep at about 1:00PM and slept until 9:15PM. Now she’s ready for a night full of fun with the overnight nurse, who is actually excited about playing with her all night since Josie’ll likely be awake most of that time.

Tomorrow should be the day we move to the floor. After the septic speed bump in the road this week she should be good to go tomorrow (or Sunday at the latest barring any new crisis). And when she moves her flipped sleep schedule will be perfect for good ol’ Dad to change. Up most of the night and sleeping during the day…taking lessons emulating Dad – warms a man’s heart. It’ll take a few days to get her back to a nice schedule of sleep by 2:00AM and up at 9:00AM like the fun days of September and October.

Her liver levels are still not great. ALT, AST and bilirubin are all still high essentially unchanged since yesterday. Her liver is still getting lots of attention. We hope to see at least some improvement soon with her re-feeding the JP drainage stopped and the TPN stopped now for a week. Something’s gotta give in the liver soon.

Josie’s other blood levels are pretty good. Hemoglobin is down slightly. This is totally expected, following a well-established pattern of losing a little blood each day. She’ll likely require another transfusion but not for a while…I predict next Friday -- we’ll see how good I am.

Infectious Disease talked more with Surgery and the talk has leaned toward getting Josie on the surgical schedule for two to three weeks from now for her big Whipple. Dr. Saltzman will talk it over extensively with Dr. Whipple (Vickers), who will be performing the surgery with Saltzman. They’re moving up the date because of Josie’s pattern of infection yo-yoing. The thinking now is that one of these days she’s going to be hit with a bug which the antibiotics will not cover and then we’ll be in a bad place again. If, though, she recovers from this week’s setback nicely and kicks into high gear to better health then they’ll have no problem cancelling the surgery for a later time. They ideally would like to see Josie much stronger and healthier before she goes in. She’s forcing their hand now, unfortunately. So we’re looking at the moment-of-truth coming much sooner than we had expected.

In the mean time we’ll just keep pressing on, trying to get Josie stronger and stronger, to get her out of bed more, walking and playing, using a commode rather than her diaper…the crawl, walk, run routine. Takes time, but we’ve seen big strides already.

Josie’s Dermatomyositis is showing signs of a slight increase in activity. Today she’s getting IVIG (IV immunoglobulin), a pulse of steroids, and daily cytoxan has been started up again too. Josie’s daily steroid dose is low at a meager 3 mg. When needed she’ll get a weekly or bi-weekly hit of 300 mg to 400 mg on top of her daily dose. This seems to be a good level for her. Methotrexate is a chemotherapy drug used as a popular treatment of Dermatomyositis. Josie has taken methotrexate in the past but been off it for some time now because of the adverse affects to her liver. Since Josie is battling a bout of liver disease methotrexate is off the table. The challenge is to keep her immune system suppressed from its full-strength because when it’s at full-throttle her Dermatomyositis flares like ma…but she would heal faster with a full blown immune system. It’s riding that fine balance to keep the disease (immune system) beaten down far enough to prevent further damage, but strong enough to allow for some healing. It’s a very difficult balancing act indeed, one which anyone would be hard pressed to do. Josie is in fact healing quite well. Her incisions, tube entry holes, scabs and all are healing perfectly fine. Her anastomoses (new attachments of her surgically joined intestines and stomach) are healing very well to be sure. So all things considered, and as bad as it seems, Josie is doing remarkably well. These recurring infections need to stop. And after her Whipple when that bum part of her gut will be gone she should rapidly heal up…then we’ll be sailing smooth. We’re counting on it.

12/21 Ups and Downs

A decent night. Josie’s fever had come down to between 100° and 101° and she got some needed rest. Her heart rate was down to the mid 160 most of the night. She woke up for good at 4:00AM and kept the nurse’s ear bent the rest of the morning. She was chatty and obviously felt better. Her physical therapy went swimmingly today. She was sitting and talking and participating…just like we want. The only problems were her vomiting and fever and pain and bilirubin and drainage and sepsis and a couple other things…I think that’s about it. Other than that, she’s in good shape.

More good news is that Josie’s bilirubin and her ALT and AST enzyme levels dropped a little since yesterday…but just a little. At least they didn’t go up. Her hemoglobin remained steady even though her JP drainage continued to show signs of bleeding…just not crazy red…just pink to dark pink every now and then throughout the day.

After the morning activities Josie slowly reverted back to feeling pretty cruddy. Her fever went back up to 102°, heart rate went back up too. So she hasn’t kicked the sepsis this go ‘round quite yet.

We had lots of chit chat about Josie’s case and her immediate and longer term plan. We’re still planning on moving to the other floor Friday or Saturday. We met with the good folks from the nursing staff over there and talked about primary care to be provided by several specific nurses. We discussed her pain treatment and overall treatment too. This was all good information for everyone and we feel comfortable that everyone is aware of Josie’s case.

We also talked with the Infectious Disease folks and they’re a little more concerned with Josie’s condition than normal. This new bout of sepsis (bacterial blood infection) has them very nervous. See, we’re hangin’ out in the hospital trying to get Josie better (duh). Trying to heal her insides. Trying our best to prepare her for the big surgery which is coming. Josie’s been septic several times now and we’ve been fortunate enough to have found success with her antibiotics beating down the infection. The Infectious Disease folks see this as pushing our luck. Josie has bugs floating around inside her and she’s become very susceptible to infection. Their fear is that next time she gets really sick…or the time after that… the antibiotics may not work as well or maybe not at all and then we’ve got real trouble. So that leads to the logical question. Which is riskier…taking care of her required surgery now, or risk further infections which could have serious consequences? Gooooooood question, and one very difficult to answer.

It’s still unknown how healthy Josie’s innards really are. We’re encouraged that her pancreas has been calm. Clearly this is helping Josie’s cause since we need that pancreas’ cooperation for the big Whipple surgery. As for the rest of her gut…we’re seeing movement, poop, feeding moving through, more poop (by the way, I’m the fortunate one to get to change diapers when I’m there. No one else is allowed by order of the little Dora Sheriff herself). Still uncertain of the level of absorption of her gut though. So the big question remains…When?

We’ll have further discussions with the surgical team as well as with GI to concoct a good plan and schedule for Josie’s big date with the OR.

In the meantime we’ll keep heading down the road toward transferring to the other floor. The nurses there are excited to see Josie again (at least I think they are), and the nurses in the ICU (59 days and counting) are happy and sad that their beloved Josie might be moving on away from their outstanding care.

12/20 Things Are Not Better

Josie, Josie, Josie. She’s keeping everyone guessing.

Last night was another bad night. She was retching and vomiting several times and spiked a 104° fever and a heart rate of over 200 bpm. A reminder of a couple weeks ago when this same scenario occurred. They sent more cultures and fired up more antibiotics so she’s now on four different antibiotics for full coverage. They’re thinking that she’s had a smoldering infection for a long time which is held in check by the antibiotics and each time they stop one or more drugs the bugger fires up again, at least that’s the theory. We’ll wait on the cultures…but for now she should be getting better with the big antibiotic guns blazin’ inside.

Today her bilirubin was up, again, to 5.9 mg/dL from 5.2 mg/dL yesterday…and her ALT and AST enzyme levels are up again too. All this is yelling out that her liver is angry and not doing its job very well. There are more theories and more changes.

Remember the gross-out thing…re-feeding the JP drainage which is basically bile and food draining out her side? Well, the GI team, consisting of the world-famous GI guy and liver expert Dr. Harvey Sharp thinks it’s pretty clear what’s going on. Sharp thinks that within Josie’s JP drain area (where her duodenum is missing), there lies essentially a cesspool of bacteria soup and because we’re feeding it back to her she’s getting sick. So his theory says that we’re poisoning her with her own secretions! OK. That sound logical, plausible, and a good enough answer for everyone to easily agree to stop re-feeding that sickening stuff back too her. Done deal. There’s a slight problem with this theory though. Her bilirubin levels have been steadily rising for the last six weeks or so basically on a linear curve. There’s been no appreciable spike lately…it’s just gotten to a point where her yellow color has made it it blatantly obvious that there’s trouble.

So we were supposed to move to the other floor yesterday or today. Who knows when that’ll happen now…but we’re all planning on this being a small bump in the road and she’ll be headed over there sometime soon. These last two days have been bad, and she feels just terrible. Think about the last time you were knocked down by the flu…vomiting, high fever, chills, aches…you know…the flu. Well multiply this by five or eight or ten, and that’s what it feels like to be septic (or so we’ve been told by those in the know). She hurts, and feels so bad all the time these days (BTW still no visit from the pain team).

It wasn’t all gloomy glumness today because we had an early visit from Santa (and an outstanding Santa too, if I might add). He and the Mrs., along with a few elves and several carts of toys made the rounds in the ICU this afternoon. Josie wasn’t feeling so hot, but I think she liked the visit, even though she wasn’t smiling. It was a nice break for everyone from all the thoughts and discussions on Josie’s condition.

Of course that leaves us thinking…why the heck can’t we get a handle on this whole thing? A logical question we ask ourselves all the time since something crazy happens just about every day. This is a better soap than All My Kids in a sick sort of way. The fact is that she has rare complications from a rare disease and her body is doing things differently than most people. Because so many things are going on at any given time, normal cause-and-effect relationships become cloudy at best most of the time with Josie. There have been so few cases like Josie there’s not a wealth of knowledge from which to draw insight. Her circumstances are bizarre and because of that no one on the medical staff gets a free pass on Josie. She’s a very challenging case and everything needs to be evaluated with a very critical eye. So together we work, think, and try to make the best decisions we can with the information we’re given. It’s not simple and we’re all putting our heads together to come up with good plans for recovery. Often, though, we have to back up into crisis mode, like today.

12/19 We Move to the Floor Tomorrow – NOT

Go figure…right when things seems to be going Josie’s way…they seem to find their way back to badness again…or not-so-goodness I should say. Today wasn’t very good for Josie and her progress.

Her bilirubin continues to creep up and is now at 5.2 mg/dL and she’s getting as yellow as a summer squash. Her ALT and AST (liver enzymes) are still elevated too. Tthese are telling us something continues to be wrong with Josie’s liver. They did a couple liver ultrasounds over the last month as her levels continued to rise and the results were totally normal…no problem. They’ve checked for hemolysis where her body breaks down red blood cells by testing this and looking at that. The conclusion was -- that we’re going to have a conference with the GI team tomorrow to assess the liver issue and conclude on the hemolysis tests.

Josie was hurting again today and continued to experience lots of nausea. She did her barf routine at least a dozen times today. We all know how awful it is to go through a day with the dry heaves and feel just crummy. This has been going on now for weeks. They’re giving her anti nausea medicine regularly with limited success. We anxiously and helplessly sit there and watch, pat her back, discuss with her how we know it’s not much fun.

Her hemoglobin levels continued their slow and steady downward march to 9.0 mg/dL today and they decided to transfuse her with a unit of packed red blood cells. Not any real biggie, we’ve been there, done that a bunch of times. Tonight we’ll get a new baseline hemoglobin level. So where’s all the blood going (this sounds like a familiar question)? Some say it’s all going out her arm in the form of blood draws for tests, others pontificate that she’s still bleeding and her body is just taking care of the blood, another theory is the hemolysis (see above) breaking down her red cells. Whatever the reason she needed more blood, and they gave it to her.

Then the bleeding stared up again. Yep…blood out the JP drain, not crazy full-blown red vampire blood, but watered-down blood which was red for sure but not frank bleeding. This kind of bleeding has been absent from Josie’s condition for a few weeks now. Usually there might be a hint of pink along a tube, not fresh bleeding. She also vomited up a bloody mix, and her poops today tested positive for blood too.

On top of all that she just felt flat today at best. She didn’t want to do much besides watch Snow White and the Seven Dwarfs four more times (I think that’s over 200 times this month now). At least we’ve got a small reprieve from Dora while she catches her breath with Snow White.

The Docs decided that she needs to stay in the ICU for at least one more day, maybe more. So when’s she moving again? Like many have said “…I’ll believe it when I see it”.

12/18 Another Positive Culture

Can you believe it? Josie had another blood culture test positive for bacteria taken two days ago . There’s some suspicion that what they found is a contaminate rather than the real deal, but they’re treating her for it anyway. The bug that grew was coagulase negative staphylococci, or coag negative staph, or a staph infection. Coagulase negative staphylococci is super common and found on skin and is the most common bacteria infection found during hospital stays. It’s also the most common isolate of falsely positive blood cultures too. Because this bug is found in the normal flora of the skin, Josie is a prime candidate for this infection because of all the foreign bodies entering her body through her skin – good paths for bug to travel. So she’s as likely as anyone to actually contract the bug. Subsequent cultures from yesterday and today have been negative so far, but they gotta treat it anyway. That only means more antibiotics.

They stopped the Vancomycin and the Levaquin (brand name with the antibiotic levofloxacin) antibiotics and started Ampicillin antibiotic. She’s also still on her low dose of Bactrim to help stave off infections (like that’s workin’ out pretty well). Of course, regardless if it’s real or a contaminate, it also means that Josie is labeled as having an infection…and that means Remicade is still off the table.

You may recall that Remicade was a drug used to treat Josie’s Dermatomyositis which she responded to very well. We’ve been patiently waiting to use it again but under no circumstances will they consider using Remicade when she’s infected or is teetering on the brink of maybe showing signs of a potential of some new unknown possible infection (can’t take chances you know). Her daily Citoxin, daily and weekly steroids, and weekly IVIG are keeping her still-smoldering Dermatomyositis pseudo contained.

Josie’s JP surgical drain has been really pumping out the volume lately. It’s up to over ¾ of a liter per day. That’s like the equivalent of a wine-bottle full each day and they’re taking ¾ of it and re-feeding it straight into her intestine (it’s still gross thinking about it). What’s likely happening is Josie’s system is moving slow and the added feeds are simply backing up and refluxing into the south stump of her altered intestine and leaking into the open space when the intestine (duodenum) used to be which is now missing and where the JP drain sits. Not much of a concern now but we’ll watch it.

Even though her pain is still there and her fever is still there too Josie’s continuing to slowly improve. Her white cell count is normal. Her hemoglobin is dropping slowly and sits at 9.4 g/dl now. It’s dropping likely because of all the blood testing draws every day. No biggie. Her pancreatic enzyme levels are totally normal and have been for a while… which is exactly what the doctor ordered, some much-needed R&R for her pancreas (to heal up and prepare for the big Whipple operation looming on the horizon). Her liver though…that’s a little different. Liver enzymes and bilirubin continue to stay elevated. Not sure where that’s going but something to watch closely.

We’re on the schedule to move to the other floor on Wednesday sometime. I guess that means we have to start moving all this stuff out of our current ICU residence. Lots of toys, and videos, and stuffed animals, and crafts which have been slowly piling up over the weeks need to move out. No problems here though…we’re headed for the next step toward home.

12/17 More of the Same

More of the same

I guess that’s good news. Not much change from yesterday. Josie was up then down. She had a low-grade fever (still) and was tachycardic (high heart rate) at the mid 150s bpm most of the day. Her lab work look pretty good too…nothing out of the ordinary for Josie there.

The pain is still there, nagging, lurking all the time. She continues with the retching and vomiting. It’s happening so much and so frequently that she seems used to it…and she doesn’t like it that’s for sure. We’ve got a stack of cups at the ready. And she lets us know by saying “…I have to sit up and vomit…”. Last night she was ralphing into her cup, her torso tightening with each hard retch. In between heaves she looked over at me with tear tracks running down her cheeks and said in the sweetest little voice “…no fun…no fun at all…” and then went back to the cup for a couple more barf spasms. Then it was over and she did the Kleenex ritual again, grab, wipe mouth, grab, wipe eyes, tuck ‘em in the cup and hand it over.

Today she’s not been passing her business like we’ve seen her do over the last week or so. Not a poop at all today, and very limited pee too. Interesting for now, and something to watch for. It doesn’t appear to be a source or discomfort though. I think we would expect to see a little more from both considering she’s at full-volume feeds now.

We’re hearing a lot of talk about leaving the ICU this week…and the surgeons keep referring to home…’when she’s home for a while’ or ‘we’d like her to be more active when she gets home’ or ‘she’ll really improve after she’s in a routine at home’. All good, and we can do it…but, -- like…wow. Home.

Home?

How’s that going to work? We’ve just not thought about it for so long. It’s great that they’re talking about leaving, but we’ll see what happens this week when we transition to the floor. There are still too many unknowns and funny thing happening, but that can change.

We’re optimistic for a great week ahead.

12/16 Medium Start – Bad Middle – Good Finish

Josie’s clock is completely screwed up. She was awake most of the night again and asleep most of the day…again. But that’s not the end of the world, though Deb wishes she’d get more normal. I figure as long as she can get rest when she wants, is awake when she wants, then what’s the big woop? Well…when we move over to the floor and I spend the first night again with her…we’ll see how much I like the new clock after all.

During the day today Josie was feeling pretty bad again. She continues to have a higher temperature topping out at 101.6° and then settling down to about 100°. She continued to have retching and vomiting episodes throughout the day….even though she slept most of it. Her pain continues, though I think it’s easing a little bit. But generally speaking she’s feeling definitely more punk than yesterday when she seemed to feel pretty darn good..

Still, it’s perplexing…the pain, the fever, the retching, the off feeling…all signs of a dandy infection. Blood cultures show nothing lately. White blood cell counts are smack in the middle of normal. What’s the cause of the fever? Surgeons think it might still be irritation form all the fluid leaking outside her missing duodenum, even though it seems those leaks are completely contained, they wouldn’t be surprised if that was the root of the fevers. Yeah…that’s a fine explanation…as good as any we’ve heard lately, at least.

More naps

Then she woke up at about 7:00 PM and was feeling pretty good. Really good in fact. She was smiling and talking and actually interacting with the nurse and Doctor when spoken to. This is not normal. I asked the Doc if there was something wrong with the girl…this was VERY unusual behavior, indeed. Anyway, good news, and it gave a good photo-op too.

The constant and unending pain has brought more thought to its cause, and focusing a little more on Josie’s interpretation that whatever it is really does hurt. Tough call for us adults to determine what’s really going on in that four-year-old mind of hers. At any rate we’re going to try a new approach by treating the mind a little rather than the pain receptors with narcotics (which don’t seem to be cutt’n it like we all want). We’re going with a drug called amitriptyline which is a tricyclic antidepressant drug. Even though it’s technically an antidepressant, its use for Josie is to treat her pain by slightly altering her neurotransmitter uptake of chemicals. Even though the biological effect is immediate with the drug, the symptoms don’t really respond for two to four weeks! So this’ll be a longer treatment and we’ll have to watch how things go. Of course, two to four weeks from now things will be different without question and maybe the pain will be better…so will that mean that this new treatment will be considered a success? Good question, and I’m pretty sure the answer will be as clear as pea soup…a shoulder shrug at best, but it’s worth a try considering we’re at three months of more or less constant pain. So bring it on.

So she ended the day with more vomiting and more retching and a low grade fever and complaints of tummy pain and some spunk and some standing and some smiles and some chit chat and some questions and some news drugs and a schedule of maybe Wednesday for a move to the floor. Not a bad night all things considered.

12/15 Slow Improvements

The TPN is done. Her feeds are almost full volume. She’s pooping like crazy. And here I though we were finally done with diaper changes after four kids. Nope, she’s lett’n ‘em fly left and right…sometimes not a few minutes apart. And guess who she insists changes them? Not a lot of protest from the nurses on that demand.

More of the same. High heart rate, though it came down a little bit today and we’re holding in the 150s. Low-grade fever at about 101°. Still retching and vomiting several times each day too. All the lab work has remained steady.

It seems we’ve transitioned from the crisis stage which we were in for so long with all that crazy stuff going on, to a more stable healing phase. This, of course, is a good thing. Instead of worrying about survival, we’ve moved on to worrying about recovery and those related challenges.

We tried getting Josie to stand and walk today. She cooperated under serious protest, making certain there was no fun had by her. She was able to stand for a minute or two and took just a couple steps but that was all she could handle and then started protesting big and bad to the point of no return and back into bed she went. That was the extent of her physical therapy today. We need more of that because her muscles are so far gone

We had a nice visit from the Child Psych docs this afternoon. They want to get to know Josie a little better to try to assess her mental state just to better understand her person and how best to treat her pain. This is a good thing I guess…It’s always better to have more smart people working on your team than not…unless the smarties are a little too smart for their own britches…if ya know what I mean. It’ll be interesting to hear their take on Josie’s demeanor and attitude relative to their other challenging customers.

Josie was re-fed her JP drainage today (which is completely sickening). They just take the drainage into a syringe and inject it straight into her intestine. The nurse yesterday mixed it in with her food to be fed later and it made her food all green and disgusting. We re-consulted with the surgeons and they said to directly inject it back into her and not in the food…thank goodness.

Things are still working out pretty well. Nothing else, positive or negative, to report

12/14 Good News…She’s Crabby

Crab City is where this train has stopped. This is good news (I think). If she’s feeling good enough to argue and be all negative, then her mind is coming off other stuff. She’s been deciding nothing is good enough. If she can have only have one sip of something she insists on five…if she can’t have anything to drink then she wants something because “…you said…..you promised…”. Oh yeah…that’s right, she’s four years old. Yup, everything was indeed a crisis today for Josie, at least in her own mind.

Yesterday the surgeons wanted to restrict Josie’s drinking to about a liter each day. She was drinking too much and that may possibly cause too much rinsing of stomach stuff out of her, so they though it would be fine to drink but to take it down a notch. Well the overnight nurse didn’t get that memo…plus for whatever reason Ketamine (pain medicine) seems to make Josie super thirsty…so she was sluggin’ water like a Death-Valley marathoner. She plowed through about a half gallon of water overnight.

Josie had to be NPO (nothing orally) from 8:00AM this morning because of the MRI image test scheduled for the afternoon. We headed on down for that which only took 30 minutes. They were taking a look at her bile ductwork and gall bladder checking for an obstruction or some other abnormality. The preliminary results are in and everything looks to be normal. More good news! So the conclusion they’re drawing is that Josie’s biliruben is elevated because her liver is finally showing signs of all its hard work over the last three months processing the TPN (IV Feeding). Sludge has likely built up in her liver preventing it from processing the biliruben in her system and that is causing the jaundice.

Today is the last day Josie is to be on TPN! This is big news. All her nutrition will now go through her gut via her J tube. She’s at about 85% of full-rate feeding and will be at 100% by Saturday. They’ve also decided to provide her with enzyme supplements in her feeds because none of her pancreatic enzymes are making it down her gut because of her new anatomy…which brings us to the other nutrition news. They’ve also decided to re-feed her the stuff coming from the JP drain. Yes…you read that right…they are going to take that gunk which is draining from the JP surgical drain and throw it right back into the J tube straight into her gut. It’s totally gross, but it makes sense. What’s coming from the JP drain is a little bit of her food and a whole lot of bile, which is supposed to be in her guts anyway, so it’ll find it’s way into the gut…through a round about sort of method.

Of course when we got back into the room from the MRI Josie was demanding (I mean DEMANDING) water. She definitely put on the cranky pants this afternoon. No one was immune, and she made it crystal clear, she wanted water and she wanted it NOW! She sounded a lot like Veruca Salt in Willie Wonka and the Chocolate Factory. “OK…fine…let me get your tubes and stuff all hooked up and then you can have some.” “…I want water NOW!...” “OK, ok, ok…here. You can have this much and we’ll have some more later.” “But Dad…you promised I could have a WOT!!…YOU SAID!!! (big cry).” “Oh Brother…here you go.” And so it went. Is this what the future holds? If so…then that’s just awesome…because then we’d have our punchy little rascal back.

We actually discussed moving back to the other hospital floor with the docs today! If we can remove the Fentanyl totally (Saturday) and get her to full feeds (Saturday or Sunday) and give her a few more good days we should be ready to move by sometime next week!

More news – Talked to the surgeons today and they’re thinking mid to late January for the big Whipple surgery. They said they’d like to see her get home for a while and as back to normal as possible before the surgery too. Yeah…they said the H word. I’m not certain they looked at the calendar though. We’re a month from mid January, and I’m not sure I see us getting home by then…but stranger things have happened during our little adventure.

Not all is perfect though. Seems like we’re making good progress…at this moment she’s sleeping with a 101.5° and 185 bpm heart rate. Her Dermatomyositis is beaten down, but certainly active still. Here liver is being challenged now. And she continues to be in a lot of pain. So there you have it.

12/13 Bili Bili Bili

Can you say Jaundice? Josie’s got it. The sclera (whites) of her eyes are yellow now and her skin is looking yellowish tan. She’s looking like a bathing beauty with the color, but jaundice is what it is. Jaundice is the increase of bilirubin in the body which causes the skin and eyes to yellow. When red blood cells die they are broken down into yellow bilirubin…this is what give poop its brown color (lovely) and why bruising turns yellow because the red blood cells near the bruise area are breaking down creating bilirubin. The long and the short of this is that there may be some trouble brewing or already brewed in Josie’s liver and/or bile duct and/or gall bladder. Tomorrow they’re going to perform an MRI-type test on Josie’s bile ducts and surrounding area to search for blockages or other reasons for the increased bilirubin.

Josie’s pain is still about the same…hurting all the time with some times more than others. She’s dropped her Fentanyl drip again to just 1.5 mics/Kg/hr and she should be off it soon. The Methadone seems to be working just as well as the Fentanyl and she’s getting Ketamine every three hours and Fentanyl bumps every hour if need be for breakthrough pain just like over the last six weeks.

Her feeds have increased again to 40ml per hour…so this is almost full strength and full volume nutrition for Josie. With all the tube feeding her TPN IV nutrition has been dropped further and further. They want to get her to a sustained 45 ml/hr tube feeds for a day or two and then they’ll stop the IV feeding altogether…for the first time in over three months. This will be big news…but it’s not here yet. Fingers crossed.

If there’s anything with Josie, she’s certainly unpredictable. We’ve been encouraged before only to be thrown for a loop by this girl. Things are looking better than they have in a while. Her gut leaks seem totally contained, blood volume seems stable, no active bleeding is obvious, pancreatitis is gone, she looks brighter, her guts are moving food and working, she’s gained lots of weight – all good. But – she’s still got this liver thing, and the foot/heel thing, and the feed-reflux thing, and the constant ongoing pain thing…and of course the completely-altered-anatomy-with-tubes-coming-from-everywhere thing going on too. And we’re still in the Intensive Care Unit with no talk about heading back to the floor …yet…but soon (I predict)…anyone have any wood on which to knock?

So we’ll see about the liver/gall bladder test tomorrow, and increase the feeds again, and keep being patient. That’ll be our day.

12/12 Building Momentum?

Today was an interesting day here at the ICU. Maybe it was the dye study, maybe it was the blood work, maybe it was her interaction…not sure, but it just seems things are a little better. And I must say, even though there has been no appreciable improvement today, it just feels like she’s getting better and headed in the right direction.

Pain is the same…pretty bad. More pain discussion was a highlight today. The thought is that until Josie is totally off Fentanyl they’ll not even consider her to move to the floor. Wait…did I just say “move to the floor”? Even the thought of moving to the other floor of the hospital is big progress.

We talked about the different pain meds again and discussed the importance of getting off the Fentanyl. Why the urgency now and not two, three, four, five, six weeks ago? Well, two, three, four, five, six weeks ago we were still interested in taking the edge off the pain…getting control of the pain, but now we’ve moved beyond that and want to get better (even though we never really got a hold of the pain in the first place…funny how that works). She should be off the Fentanyl in just a couple days at this rate, even though the pain is still pretty severe. She’s allowed hourly Fentanyl hits for breakthrough pain still. They’ve also added Ketamine as a pain killer for breakthrough pain…which is supposed to be given before a Fentanyl bolus. A while back Ketamine was a drug which was started and then stopped right away. At that time they were concerned the Ketamine was the cause for the high fever and high heart rate (…remember 105°, 215 bpm), while hindsight has concluded with a shadow of a doubt that the cause was bacteria in her blood, so they’re giving Ketamine another go.

Late yesterday Josie had an echocardiogram test to see if her heart is doing OK. Considering her ticker been ticking away at about twice the normal rate for months now, they wanted to see if all is well in the heartland. Good news -- everything with her heart appears normal.

Yesterday the GI team decided to try the drug Reglan to get her stomach to move things through a little faster and better to prevent this. Well, after some thought and discussion they’ve decided to change drugs to one called Misoprostol. This drug causes her intestine to constrict a bit forcing motility of the bowel. This, the thought goes, will push stuff through her guts and pull stuff down from and prevent reflux back up into the stomach. See, she’s still draining feeds out of her stomach G tube even though the feeds are being directly injected into her jejunum (small intestine) through the J tube.

Another interesting test today was a dye study of her upper GI tract. They injected a contrast dye into her J tube. The dye shows up on x-ray so they watch her with a fluoroscope as the dye goes in. They wanted to prove the theory that the south part of her eroded duodenum is leaking and that’s why we’re seeing feeds coming from her JP drain. Watching the screen we could see the dye heading down her bowel as normal, but then it showed that the dye headed back up that south duodenum stump toward where the staple line which was supposed to seal it closed. Of course a minute or two later we could see the dye seeping from the stump out into the open space where the old duodenum used to be, and the dye then headed out the JP drain proving the theory. The best news about this is that the scope showed the dye was completely contained and did not flow beyond a well defined space. This means that even though we’ve got some great big leaks in there, it all seems enclosed and that means no more free flowing bile in the abdominal space and no more angry pancreata. Further proof that the pancreas is happier is that both her amylase and lipase levels are finally in the normal range and the lowest level they’ve been since they started measuring them in September. This is big news I think.

Her liver enzyme levels are still up and so is her bilirubin level. This is indicating trouble with her liver. I think we're going to press a little harder on the docs to address, or at least make us feel better about her liver and its health. More on that tomorrow.

Lastly, she seems brighter and more alert than she’s been in a while. I think this is a function of the pain medication moving from Fentanyl to Methadone. Which brings us back around to the pain. Yes she’s appearing brighter, but she’s still hurting just as much and just as uncomfortable, and retching and ralphing just as much. Low-grade fever still around 101° all day with a heart rate in the 160s most of the day too. So good news and same news…but things are…idunno…looking more positive than negative. Fingers crossed for positive.

12/11 More Of The Same Again

More pain management today. There continues to be a debate about how much pain medication should be administered to keep the girl comfortable. We’re of the camp where we’d like there to be less narcotics and less pain…du…I think everyone will say they want that. Some feel that if she’s showing discomfort then drugs are the way to go to get her feeling better…lots to be said in favor of that too. Comfort is the name of the game with pain management, but at what cost? Again we get to the question of does this really hurt that bad. Then you take a look at her and say…yeah…it hurts. Then there are times when you can tell…nah…it’s not as bad as she’s saying. I think we’re in tune enough to tell. Problem is that sometimes you just cant tell, and she gets the extra hit, and then another question comes up in the back of my head…is that extra hit helping or hurting? – Just don’t know.

This day she did her share of retching and vomiting. It’s kind of funny in a sad sort of way. It goes like this: She starts hurting more than normal…but we can’t give her more medication yet because of the schedule. Then the pain will trigger the nausea and then the retching begins. She’s got the trusty Dixie cup to her face and gives the big gut-splitting, torso-spasming, full-body heave. The pressure inside squishes out the contents of her stomach out her mouth and out her G tube. The pressure pushes more stuff out the JP drain too. On each subsequent heave you hold her upright, helpless to comfort her, and watch the stuff flow out of her body through the tubes. After the heaves she hands me the cup, asks for a Kleenex, wipes her mouth, sticks it into the cup, asks for another Kleenex, wipes her eyes, stuffs it into the cup, asks for a new cup – done. It’s a routine now.

The transition from Fentanyl to Methadone has gone slower than the Pain Team wants and even the ICU docs want. We’re now giving 4mg Methadone every 6 hours and have dropped the Fentanyl to 2 mics/Kg/Hr. The goal is that we’ll get her off the Fentanyl totally sometime soon (two days?).

Her blood work levels continue telling a story, but we’re still a little cloudy of the plot line. ALT and AST are elevated indicating a little Liver Disease, CRP (inflammation marker) is higher again at 37mg/l (normal is 0-8 mg/l), white blood cell count is normal at 14, lipase (pancreatic enzyme for fats) is 325 units/l which is a little bit elevated but it’s down from Friday, hemoglobin is 12.2 g/dl and holding. One can look at this and be encouraged by some of the numbers.

Her heels continue to be hard. We may have a podiatrist come visit Josie for an opinion. Seems we’ve still no good answer for the heel swelling. There’s a thought that she might be suffering from lymphodema (lymphatic obstruction). Here’s your medical lesson for the day because we’re certain you all want to know about the lymphatic system of the body…here goes –

The lymphatic system (often referred to as the body's "second" circulatory system) collects and filters the interstitial fluid (fluid within and surrounding tissue cells) of the body. The lymphatic system is a major component of the immune system. The lymphatic system has three functions: (1) removal of excess fluids from body tissues, (2) absorption of fatty acids and subsequent transport of fat, chyle (lymph and emulsified fats, or free fatty acids), to the circulatory system and, (3) production of immune cells (such as lymphocytes, monocytes, and antibody producing cells called plasma cells). Lymph originates as blood plasma that leaks from the capillaries of the circulatory system, becoming interstitial fluid, and filling the space between individual cells of tissue. Plasma is forced out of the capillaries by pressure gradients, and as it mixes with the interstitial fluid, the volume of fluid accumulates slowly. Most of the fluid is returned to the capillaries by hydrostatic pressure gradients. The proportion of interstitial fluid that is returned to the circulatory system by osmosis is about 90% of the former plasma, with about 10% accumulating as overfill. The excess interstitial fluid is collected by the lymphatic system by diffusion into lymph capillaries, and is processed by lymph nodes prior to being returned to the circulatory system. Once within the lymphatic system the fluid is called lymph, and has almost the same composition as the original interstitial fluid.

How ‘bout that?

It’s just a theory about the lymphodema causing her heel issues though, but it’s fun learning about the lymphatic system isn’t it? We’ll keep working that project while the docs try to get that black box of a tummy better.

She’s still leaking her feeds from her tubes, but the volume has not changed much even though they’ve increased the feed levels. This is good news, so we’re working that angle too. More food in the system means less IV food and that means better all-round health.

12/10 More Of The Same

Josie had another mediocre day today…hurting tummy…retching and vomiting several times…many pain-medication bumps…more feeds exiting where they shouldn’t…and an interesting twist.

Josie experienced what the docs are convinced is Red Man Syndrome. Symptoms include redness in the face and neck, burning and itching, chills, fever and general discomfort. Red Man Syndrome is a hypersensitivity reaction to the rapid infusion of Vancomycin antibiotic and is not uncommon. Red man syndrome was in the past attributed to impurities found in Vancomycin preparations, earning the drug the nickname 'Mississippi mud'. But even after they improved the purity of the drug it kept happening. Studies have shown that an unknown percentage of the population may be prone to releasing a large amount of histamine in response to Vancomycin. It’s really not a big deal at all…they just give it to her slower and that takes care of it. It’s interesting because she’s been on and off Vanco for months now starting back during her hospital stays in July of this year so why is she getting this reaction now? Good question, and it’s been documented that it can happen this way…and no one has a good explanation for it.

Josie did have a spell today where her temperature was 101.9° and a heart rate in the 190s. That’s since dropped to about 150 bpm and 99.5°. Better. So what does that mean? Don’t know, but time will tell as it usually does. It seems we can tell you exactly what has happened – hindsight is predictable that way, donchathink? While at the time everything happened everyone seemed to be inwardly (and many times outwardly) scratching their heads…like now.

When Deb arrived at the hospital this morning Dr. Saltzman (surgeon) was playing little piggies with Josie and she was interacting along just great. See this is what happens. When Deb or I are there, Josie pretty much clams up but as soon as we’re out of her hair she seems to interact with the hospital staff real nice like. It’s kind of funny. Now that everyone knows Josie quite a bit better, it’s now become an endearing quality of hers.

Nothing else new to report…I don’t think.

12/9 Another Up And Down Day-And A Big Party

We got some bad news today. Once again Josie has had a blood culture come back positive. If this was the first time she had been found to have bacteria in her blood we might be freaking out…but since it’s about the 18th time…it’s almost like…’oh, that’s interesting - ho hum, we’ll go with a little Vanco and clear it right up.’. Part of me really feels that way, but the more practical part knows that bacteria in your blood is bad news and can get nasty pretty quick, and I also know those guys are all over it.

Josie was feeling – well – it’s hard to say how she’s feeling without saying the same old thing. She feels pretty bad. At times during the day she was ok, but more times than not she was hurting, with her trusty cup to her face. Clearly we’re not hitting a home run on the pain management front…and it’s not from lack of effort because they’re trying hard to find the balance between keeping her comfortable and having her totally snowed. She’s requiring her Fentanyl bumps every hour…which is telling us something. So they upped the Fentanyl and the Methadone drips. I wish she would just stop hurting.

Her surgeon was in and was encouraged by her progress. He didn’t seem too fazed that her feeds were draining so much out of her JP drain and her G tube…which means the food is refluxing from the small intestine back up into her stomach and out the G tube. He wants her to get more active and said we need to force it on her just to get her going. This afternoon we tried just that but simply standing her up on her bed. She tried it but it didn’t go so well. She had a hard time holding her weight with her legs, but the more troubling thing was that it really hurt her feet.

Ah those feet of hers - her feet have been an issue for weeks now. Ever since the surgery in late October Josie’s feet have been very hard…like unripe plums. The situation hasn’t seemed to resolve at all during her recovery and the condition, whatever it is, is causing pain when standing or putting too much pressure on her heels. The task now with Physical Therapy is to get her on her feet again…but we need to resolve the heel problem. None of the Docs have a good idea what’s going on there. We’re going to try some massage therapy, and some other tricks so her feet won’t be a reason not to get up and walk.

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What a party it was. Over 700 people came to show their support to Josie and our family. It was a truly uplifting event. The amount of work our friends put into this party to make it all happen was humbling. When we arrived at the church we were floored at what we saw. The amount of food which was baked by everyone was simply jaw-dropping. There were tables of goodies galore, simple to fancy, and all delicious. I was thinking we’re never going to sell all this stuff…but at the end of the night, there was hardly a crumb left over. And the silent auction was a whirlwind of activity and incredible last-minute donations…over 250 items total…which all sold! Amazing. We do have a small distribution issue now though. Quite a few people who won their auction left before the end of the auction and need to claim their items. So if you think you may have won an item please contact Shelly at doran-home@mn.rr.com. Then the food production to serve so many people without the slightest hiccup -- no lines at the food trough all night. They were cooking and serving fast and furious. The music was perfect – And a most unexpected surprise – A song inspired by and written specifically for Josie. Kyle and Lindsay Sumrow from the band Poema wrote a song just for Josie called Dear Josie, and it was just beautiful. How cool is that? And probably the most fun and biggest hits for the entertainment buck were the snow-cone machine and the bouncy things. I’ve never seen such big indoor bouncers. And it didn’t seem like the kids have either. There were lots of rosy cheeks and sweaty heads flying around the gym. The star of the show might have been Goldie. He was great. All the kids were getting his autograph and pictures taken…it was so fun to watch them see THE Goldie Gopher, right there at their party! But before Goldie came to the big party, he stopped by Josie’s hospital room in the ICU so they could have their own little party. She was into it…she smiled a little and high-fived Goldie…and after he left she told her nurse who came on the night shift that she met and high-fived Goldie Gopher. Thanks Goldie for cheering up our little Josie!

What a success! But the greatest thanks of all has to go to everyone who came out and supported Josie and all she’s been through and will go through. This was a moving and humbling experience for us, one which will stick for a lifetime. Our hearts are so lifted by the outpouring of support from you. Thank you all!

12/8 Pain and Glowing

Josie has another painful day and of course there were a few episodes of retching to top off the pain. Not good. They dropped her Fentanyl pain killer to 1 microgram per kilogram per hour down from 7.5 mics/Kg/Hr just a day ago…too fast if you ask me. She was really hurting and needing the extra bolus bumps every hour. As a result they moved her back up to 2 mics/Kg/Hr and may well kick it up again to 3 shortly.

She was wide awake again starting at about 3:30 this morning and wanting to do books with her nurse. Her internal clock has completely flipped I think. During the morning she was kind of spunky and wanting to do things and talking and being even a little sassy again… between the painful bouts that is. It seems like a constant dull to not-so-dull to pretty bad pain but not none. What I wouldn’t give for NONE!

Her fever was up again and topped out at 101.9° and her heart rate was hovering in the 150s to 160s. These symptoms, even though they’ve been pretty standard for Josie lately, can possibly be attributed to narcotic withdrawal. It’s hard to say…just like everything …because so many abnormal things are going on in this little lady’s body.

Other news…the CT scan from a few days ago was revisited by the surgeon and he seems fairly confidant now that the JP drain HAS NOT perforated into her small intestine as we had all thought it had. They’re now fairly certain that the south end of her duodenum which was stapled shut during her last surgery has opened back up, meaning the duodenum has continued to erode past the staple line. This is certainly bad news…but we’re feeling a little better about that than with a new perf which we thought yesterday. The JP drain is doing its job nicely and as long as it stays clear and draining like that then we should be OK.

Tonight Josie got a hold of one of her birthday gifts …glow bracelets and necklaces. She thinks they’re pretty cool. She wanted me to take her picture with them and was happy enough to even smile for the camera. Fun.

Benadryl, IVIG, Chloral Hydrate, Tylenol, Fentanyl, Methadone, Cytoxan, Solu medrol (steroids) – That’s quite a list, eh? That’s the drug cocktail combination which Josie had just today. Makes a guy wonder what the long–term affects these drugs will have on Josie…just a head shaker.

Josie knows there’s a party tomorrow and wishes she could be there with everyone, but we’ll take some pictures and video for her to see later. And maybe the best news about tomorrow is that Goldie Gopher is coming to the hospital just to see Josie before he heads down to the big party. Should be great fun. See you there.

12/7 Sleepy Day

Today was kind of quiet. Josie was awake much of the night...but not for any new ailment or crisis. She just couldn't sleep so she and her nurse read some books and watched some movies. Of course that meant she ended up sleeping most of the day. Go figure. She was awake a bit and was fairly uncomfortable as usual but when she was sleeping it seemed to be pretty restful.

Last night she had some food refluxing up from her intestine into her stomach which was coming out of her G tube. The surgeon (Dr. Saltzman) decided to stop the feeding for a few hours over night to give her gut a chance to recover from the placing of the new tubes and the anesthesia from yesterday. At 8:00 AM the feeding started once again. Her JP drain is still draining part of the food she getting though her J tube mixed with bile confirming again that her bowel is perforated and draining part of its contents out the drain...which is certainly unwanted but now totally expected.

Her belly seems more distended today than it's been, but measuring around her tummy shows it's the same as before. She's been complaining pretty badly that her tummy is hurting still.

And that brings us back to the pain. It's still there...big and bad...bad enough to bring any he-man to his knees, but not too much to stop Josie from fighting her little heart out. She just takes it and rolls along. Our nurse even commented today that Josie is so strong and so polite about her pain. When she hurts she just tells them it hurts and asks them to make it stop very politely without a lot of the drama they often see in the ICU. Perhaps that's a big reason why Josie is so popular with the ICU staff, aside form being a semi-permanent fixture here.

They started the transition from Fentanyl to Methadone for her pain management. They gave Josie the first dose of Methadone this morning, then a second dose went in six hours later. At the same time her Fentanyl was dropped by almost half. The idea is to transition her completely in 24 hours. This was a little alarming to us because of all the understanding of Josie's dependence on Fentanyl. You don't just cut off a junkie over 24 hours (do you?). The docs seem confidant this is the way to do it. We'll be keeping a watchful eye on her for signs of withdrawal over the next day or so and hopefully she'll transition nicely so we can be in a better pain place. We really want her feeling better so she wants to color again and do projects and stand up and get active...and heal.

Things may be heading to a more challenging place though. Her temperature has climbed just tonight from normal all day to 100.3° and her heart rate which had been in the 130s and 120s all day has now crept up into the 160s. This could be a function of the weaning of the Fentanyl, or it could mean something completely different. The Doctors on the ICU floor will be watching her all night.

Nothing's ever easy.

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Looks like there's going to be a great turnout for the big Joy For Josie party Saturday. If you haven't yet replied to your evite please do so and let us know if you're coming. Everyone is welcome so if you've not received an invitation click here.

We're in need of a few volunteers for a couple different things. If you're interested in helping for 20 or 30 minutes Saturday night click here

12/6 If It's Not One Thing It's Another Part VI

Another interesting day in Josie’s little world…the kind of which we’ve come to expect unfortunately.

Josie had a decent night and the pain was still there. She was still pumping out the fluid from the JP drain (refresher: JP stands for Jackson-Pratt…Jackson and Pratt were a couple of guys who had the bright idea of using a turkey baster to suck out stuff from where it shouldn’t be). The JP fluid was presenting little white particulates…kind of a sediment. This is pretty new over the last two days so we decided to save a sample and ask the docs when they rounded this morning.

Today Josie went into the operating room to have her NG (naso-gastro) and J (jejunum) tubes replaced with a single GJ (gastro-jejunum) tube coming from her belly. It’s a double-port thing which has a tube going into her stomach and a second one heading down through the stomach into her jejunum (small intestine). They also inserted a new picc line into her arm.

So here’s the big news of the day, and it’s not good news. After looking at the drain sample we saved, they had a hunch that the stuff settling in the drain fluid was actually her feeds. This is not very good at all because the JP drain sits outside the intestines, and the food is inside the intestines…see where we’re going here?

She has another bowel perforation! The JP drain has somehow worked its way through the bowel wall and sprung a leak passing gut juice and all outside the gut. Can you stand it?! This could have been the cause of all the badness over the last 10 days or so. The good news is that the JP is draining the stuff. At some point she’ll need that fixed too, but when? Much of Josie’s GI tract is altered, now she has a second perf, things don’t seem better, though some are arguing things are much better. I guess some things are, but generally speaking we don’t seem to be any closer to leaving the hospital than we were a month ago. There’s not even a ray of hope of leaving the ICU any time soon, let alone actually becoming a healthy little girl once again.

Her numbers are OK. Lipase (pancreatic enzyme for fats) is up a little but not much, ALT and AST are elevated, and her bilirubin is elevated too still indicating possible liver disease. This is to be expected (just a little though) with folks on TPN (IV food) for so long. That makes the liver work really hard which it doesn’t like, but when the feedings become full-bore with the feeding tube and without TPN then the liver should calm down and recover nicely.

GI stuff aside, her dermatomyositis seems to be in better shape. Her weight is steadily increasing, her skin has plumped up, and though there is still the rash there are no new breakdown areas and her general skin appearance seems better. This is good. It’s hard to say what happening with her muscles because she’s been sitting and felling like crud for so long…she’s been atrophying away. We’ve been trying to get her out of bed for a while now but the tummy pain has prevented us.

Our surgeon has decided to have a sit-sown with Dr. Whipple (Dr. Selwyn Vickers, Chief of Surgery at the U of M Medical Center who only performs Whipples (and lots of them)) to discuss Josie’s case and the new developments. They’ll talk and ponder Josie’s fate and their decision may be to try the Whipple earlier than the hoped March timeframe as she’s not progressing like we all want. We’re likely to be discussing that quite a lot before any decisions are made. So…how bout that??


Just three days until the big Joy For Josie party. If you’ve not responded to your evite please let us know if you’ll be joining us, it’s going to be great.

12/5 Kind of OK

Today was one of feeling bad and not much action.

They decided not to go to the operating room to place the new picc because her blood culture from two days ago grew out indicating an infection. If the picc is inside her arm and there is Group D Enterococcus bacteria in her blood, that stuff will attach itself to the picc line and just fester...continuing to make her sick, kind of like what happened over the last 10 days ending Saturday. The doctors don't really think it's this kind of bug though...they're "90% sure" that it's a Gram-Positive Staphylococcus bacteria (staph infection) which can be caused by a contaminate from her skin rather than a bug in her blood, in which case they'd go ahead and insert the picc. So they have her slated to go in for the procedure tomorrow.

Josie had some perfect reasons to feel really great today. Her temperature was normal all day and her heart rate was in the 130s most of the day and dropped to the 110s this evening, the lowest it’s been in weeks! Yet she feels totally crummy. She had retching episodes at least five separate times...everyone knows how bad the old dry heaves are…and gauging by how she looks, it feels no better when you’re a fresh four year old with nothing in your tummy.

The other notable event of the day was Josie’s JP surgical drain. This thing was pumping out the fluid all day long…a lot of fluid. Her previous record output from that was just over 500ml. Today she was almost 700ml at 10:00PM. A drain at a surgical site still draining almost a full wine bottle full of bilious liquid six weeks after surgery might be a concern…ya think? A head scratcher for sure but nothing to worry about according to the docs.

And that brings us back to the pain medicine. We’ve been told before that too much volume of narcotics can flip the response to it and actually cause more pain and discomfort…no one’s sure if this is happening but it’s something to consider. Everyone is concerned about Josie's discomfort. She should feel bad but not this bad. It makes us feel helpless watching her sit and sit and not interact with people and not talk to us and retch and feel bad and not smile and not laugh and not want to watch TV and not want to sit up and not want to color and not...

12/4 Pain Management

Josie’s in a better place than on Friday, that we can all agree. But that doesn’t mean things are rosy. She's still hurting pretty much all the time.

Today we focused on the pain a little bit. Her pain management has been an issue for weeks and it appears that’ll keep up for a while. Her steady Fentanyl dose was increased today to 7.5 micrograms per kilogram per hour with 75 microgram boluses as needed up to once per hour. Over the previous 24 hours Josie received 19 bolus bumps. This is a problem.

Here’s how it goes…she might feel better (not good, just better) for a while and then the tummy pain will kick in again and she’ll be grabbing for the cup and complain about her tummy hurting often followed by a retching/vomiting episode…so that means the pain isn’t under control. Clearly she needs a hit of painkillers…in it goes and then she might feel good, or she might get knocked out by the dose. Too little and things stay bad, too much and it’s lights out. Baby Bear’s porridge is hard to come by these days.

The Pain Team came in again and we discussed the issues. We’ve got some serious pain challenges with Josie, partly because we don’t really know where the pain is coming from, and partly because she’s not really been stable, and partly because it’s sometimes tricky to guage her real level of pain. It's kind of like...we’ve tried this and because of that this was discontinued and that was started and this was dropped down and that was combined with this other thing and that stuff was increased…then we all agreed because of this, that was a bad idea, and this wasn’t right, and that wasn’t right so we scrapped the plan and started back up with this and that (seriously…that’s what it seems like).

Here’s the new pain plan. We’ve moved up on the Fentanyl drip …enough to snow an elephant…plus big hourly boluses as needed. After tomorrow Josie will begin taking a new painkiller, Methodone (a synthetic opioid used as a painkiller and in the treatment of narcotic addiction (like Josie)). The plan is to transition her onto Methodone over time. Methodone works well as a painkiller and is used often with burn patients because the drug’s really good with pain and is long lasting.

We’re waiting until after tomorrow because earlier in the day she set to go into the operating room (again) to have a new picc line inserted. Two days ago they pulled the old picc (peripherally inserted central catheter) because it became fairly clear that is was the picc which was causing her blood infection from last week. It’s well understood that bacteria likes to grow on the synthetic material at the end of a picc…after a while bacteria coats the end of the picc in a scummy film which continue to infect the patient. Out it came. Problem is that with so much medication and the IV feeds, Josie needs a central line, so a new one’s set for installation tomorrow…under general anesthesia.

They’re also planning on inserting a GJ tube (gastro-jejunum(stomach-intestine)) into her tummy tomorrow too to replace the current J (jejunum(intestine)) and NG (nasogastro(nose-stomach)) tubes. This will be nice because it’ll mean Josie will be able to take out that nose tube, hopefully for good.

Her labs are looking pretty good these days. Her pancreatic levels are both near normal so her pancreatitis as stopped…and good riddance. White cell count is normal, hemoglobin is stable but low at 9.5. Her lymphocytes are low (expected when on the Cytoxan chemo). Liver enzymes are a little elevated indicating a bit of liver disease. We stopped her weekly steroid blast with hopes that it would help healing with less steroid on board.

So on we move with pain on the brain and we’re hopeful she can begin to enjoy life again rather than live between simply hurting and drugged out of her mind.

12/3 She's better! (a little)

I’m sorry for not posting anything this weekend…the blogging server was on the frits and we were running around with very little time.

Anyway…Things are better!

It was a touch-and-go kind of day on Friday and Saturday. Josie’s nasty condition on Friday was really really scary. Josie’s temperature was so high for so long and she was becoming so distended we were convinced that she had another perforation…which would likely mean another date with the operating room. I keep telling her she and her surgeon need to stop meeting like they’ve been.

Josie’s Friday CT scan turned out to look pretty good, what was a nice surprise for everyone. There’s still a chance that there is a walled-off perforation which has caused the infection which was raging inside her Friday.

The Infectious Disease doc’s decided to change her antibiotic regimen and cooked up a new antibiotic cocktail which has seemed to do the trick. She’s now on clindamycin, ampicillin, gentamicin, and bactrim (spell checkers don’t like these big fancy words) which is not a hard-core big-gun cocktail, but it was the right one for Josie this weekend. Her heart rate dropped on Saturday through the 180s to 160s and settling in most of the day today in the mid 140s.

Her fever was till pretty high Friday night into Saturday but has now broken and is back to normal. They had the cooling blanket in here which Josie was laying on. You could tell she was uncomfortably chilly even though she was raging hot inside.

Most of Saturday night and Sunday went well…she was chatting up the nurses, playing a little bit, making certain they were doing all the things they do well. But Josie was insisting, once again, to replace her own heart monitor leads (putting them in the right place in the right color order among other things.

Tonight Josie is feeling a little worse. Her fever is back a little bit at about 100° and her heart rate has climbed again into the low 160s and she feels generally not very good.

There was a little excitement today when a local news crew came in to film some pictures of Josie for a little piece which aired on the channel 9 news at 9:00 tonight. You can watch the piece here or go to myfoxtwincities.com.

12/01 105.2° !!

You read it right. What an up and down night…first way up then way down. We celebrated Josie’s fourth birthday last night in the family lounge with the whole famdamily. Deb’s parents, brother and sister were there along with all their kids…it was a roomful for sure. Josie was able to sit in a wheel chair while we rolled her down to the event. She got tons of wonderful Dora stuff along with other great gifts. We all wore hats and had some great food too.

During the party and much of the day Josie was feeling pretty bad. Her tummy hurt, but she was a real trooper to hang in the way she did. We even got some smiles out of her when the gift opening began.

Josie’s fever was higher all day around 101° to 102° with that high heart rate. We brought her back to her room and she was feeling worse and her temperature was rising and so was her heart rate. At about 10:00PM her heart was racing at 195 bpm with a 102° fever. We talked with the docs and they were to watch her closely.

They did just that as they watched her heart rate climb to a whopping 215 bpm and a staggering 105.2° fever. The heart was over 200 most of the night. They were giving her fluids and fans and wash cloths and ice and a cooling blanket.

It looks as though her bowel has perforated again! We’re not certain but it’s not looking good. She had some bile seeping out of the skin opening of her jejunum tube and she is pretty distended. Infectious disease was in and they put her on an antifungal and her antibiotics are continuing. They are now pumping some contrasting solution into her guts so they can do another CT scan to see if and/or where any perforation is located. We’ll keep you posted on that progress over the coming days.

On a lighter note, Josie’s benefit dinner event is coming next Saturday December 9th at 5:30PM. We’ve put together a little web site explaining the whole thing and are eager to see all of you there. There’s going to be a big ol’ bake sale, so get cooking; tons of spaghetti, big bouncy contraptions for the kids, a silent auction (we need some more great items for that), musical entertainment, and a special visit from Goldie Gopher himself. Check it out at http://www.deebers.com/joyforjosie