Tuesday, February 27, 2007

2/27 Motility Talk

The buzz around Josie’s medical chart today has been her gut motility (movement) or more specifically the lack there of. Her bowel is just not moving…it’s like it’s asleep. They’ve been feeding her into the J tube but most of the food is simply refluxing up the jejunum and into the stomach only to be pumped right back out. The more stuff shoved in, the more stuff comes out…it’s so interesting how that works.

Ideas include motility agents (drugs like Reglan and Misoprostol), inserting a new J tube further down her jejunum, or do nothing at all and wait it out. The GI Team proposed the interventional procedure to get the feeding further downstream with hopes that if the tube feeds below the surgical attachment point from her October surgery that may provide the kick start she needs to fire things up again. Rather than do that they decided to do a dye study to check for an obstruction and see how things actually are moving. That happened this afternoon and the results we about what they thought: still no leaks at the repair site, slow movement of the GI tract, no obvious sign of obstruction. They’ll be checking again tomorrow for further movement of the dye through her system. Dr. Whipple (Vickers) has been visiting every day and is slightly concerned about this because of the length of time since the surgery, but not overly alarmed. He thought GI’s idea of getting a tube further downstream is a pretty good one, but thought they might give her one more week to heal before trying that.

Josie’s day was better than yesterday with just a little nausea, actually a normal temperature for a while, and a rekindled spirit. She was upbeat and feisty today – a welcome change from yesterday. While at physical therapy she was tearing around the floor again on that bike. Pretty soon she’ll be banned from the halls, acting like the other Adams kids.

After that she crashed in her now-normal four-hour nap, waking up and still feeling not too bad. So here our family sits, awaiting lots of poop and full diapers, which would be wonderful.

Monday, February 26, 2007

2/26 Things Are Getting Interesting Again

An up and down day for the girl. Things started out about the same as they ended last night with some vomiting and retching and feeling bad. That heart rate’s been racing again between 160 and 170 bpm…it’s starting to sound like a broken record. But now it’s becoming more of a concern for everyone (save the very optimistic surgeon) because at 10 days after surgery the guts should be moving nicely by now. There’s some, but not a lot of movement in there. Her feeding rate was turned up yesterday but since those guts aren’t moving well, that did nothing but force food backwards up the jejunum into the stomach and out the G tube (which has been on suction).

There was a whole gaggle of docs in the room discussing Josie’s issues and one suggested (and all agreed) to take an x-ray to see if there’s an obvious problem. Then they thought they could use that x-ray to also see where the contrast stuff is they injected into her system from the leak study of a few days ago. Then they thought they should’ve done a motility study along with said leak test…and on it went. – As Deb told me the account of what happened I was reminded of that old story where a loaded truck is too tall to go under a bridge and gets stuck underneath. All the smart people surround the truck trying to figure out how to get the thing unstuck thinking up good ideas of how to use pulleys or straps or tow trucks or levers or wedges or winches to solve this perplexing problem. Of course the four-year old watching the smarties struggling says “…why don’t you just let the air out of the tires?” – In our story Josie is the four-year old…and I swear I see her rolling her eyes at times like these, but she didn’t chime in to solve today’s problem, she just took her blanket and pulled it over her head and checked out while all the smarties tried to figure out her deal.

They agreed that Josie needs another contrast study to check motility of her tummy and GI tract. Indeed the duodenum repair from the surgery seems sound which is great news for sure, but the gut’s got to get moving. She can’t get off her TPN IV food without it which means more pressure on her already angry liver, and that could ultimately cause even more major problems. Ahhh Josie, Josie, Josie. We’re hopeful this is just her making us all crazy with a minor delay and nothing is terribly wrong.

The good news about today was that she had one of her remaining two JP drains removed... tomorrow they’ll probably take out the last one. Later in the day when she was feeling pretty good she was going gangbusters! Josie hopped on that bike of hers and was tearing around the halls like a bat out of H – E – double hockey sticks. They had her all disconnected from the suction and IV medicine and feeds for about 90 minutes. Shortly after that though she was having more intense and more frequent vomiting …so until she gets this whole GI movement thing figured out we really need to keep that tummy suction on. Weird that she’s been having her stomach pumped pretty much full time for the last few months.

On we go.

2/25 Not So Good Today

Josie was feeling plain bad much of the day. It’s now a trend…nausea and vomiting and fever and some tummy pain. Things were going so well there for a while but have slowly changed to not so well and the improvements have been slower to come.

Walking Around CampusWe had a big snow storm yesterday and Josie knew all about it. Of course she wanted to go outside to experience it all after hearing everyone talk about their travel and snow experiences today. We got her all disconnected and headed out for a fun little walk around campus with the fresh foot of snow – Josie had a fun time. We came back in, got her all hooked up again and continued along with what turned out to be a pretty crummy day. We tried to get her up and walk a little and that’s exactly what it was…just a little walking. She was complaining of pain “…where the guy took the tube out…” in the lower part of her abdomen and really didn’t want to have anything to do with it any more.

So what to do? The fevers and heart rate are causing perplexed docs. She’s been on a hardy cocktail of antibiotics but it’s had little effect on those symptoms. They decided to make a change by reformulating the antibiotic potion…kind of like ‘…well, this isn’t working so let’s try this other stuff and see what happens.’. The thought is that perhaps there is a brewing bug resistant to the previous formula. OK…let’s give it a go.

She slept much of the afternoon and early evening. Most of her awake time tonight she was vomiting every 20 or 30 minutes…not a fun way to pass the time at all. The good news is that the JP drains have basically stopped their output and those tubes and drains should be removed sometime in the next two days. That’ll be nice.

Saturday, February 24, 2007

12/24 Not As Good Today

Different day, different way. Yesterday she was feeling so much better and looking great, but today is a different story. She's been sleeping most of the day today. Reason for that is she's feeling sick and crummy. Her heart rate has been on the rise over the last 24 hours or so hovering around 160 bpm most of the day and has currently crept up to 170 bpm tonight. Her temperature is also on the rise. She was running a fever around 100° much of the day then it dropped to normal, but tonight it's been heading north to a max of 101.4°. She's been nauseous and has had a few vomiting episodes today too. More blood cultures were drawn earlier for the umpteenth day now and so far there's been no growth in any. The surgeons don't seems too concerned about it...but we're frowning our brow. Hopefully she'll be able to turn it around on her own overnight.

The surgeons stopped the suction of the JP drains today after several days of minimal drainage output. This is the first step of removing those buggers. It'll be so great having those surgical drains removed...which truly means her repair is healing well.

Friday, February 23, 2007

2/23 Good Things Comes In Three's, Right?

That's what they say at least, and today that's what happened with Josie.

Good thing number one - Today was her one week anniversary of the big surgery and nothing terrible has happened. She's had some downs but mostly ups this last week. She's left the ICU and this time I think for good, she's been up and walking and feeling better, she's been (almost) nausea free, she's had only a little tummy pain, she's coloring and being sassy again.

Good thing number two - Josie went in today for a leak study. They injected her duodenum with a contrast and watched it under a fluoroscope to see if the connection they hooked up during surgery was holding. After several minutes watching the stuff float around her intestines they determined the repair is sound and leak free...this is very good news indeed.

Good thing number three - Josie had one of her JP drains removed today. This is the first time she's ever had a drain removed...and they don't just remove drains for fun. That drain hadn't had anything substantial come out in some time and it wasn't needed anymore, so the exnayed it. The other two drains which are right above and below the surgical repair will stay in for a few more days just in case but those aren't draining anything substantial either.

We're in a good place right now and this upcoming week will truly determine how well this surgery went for Josie. It's looking really good.

Thursday, February 22, 2007

2/22 Not As Good, But Not Bad

After last night’s disturbing back-pain problem Josie had a restless night. She was tossing and turning and wanted lots of drinks throughout…made for a choppy sleeper for me. In the morning she was a little better but still not her perky self.

Much of the morning she was complaining of back and tummy pain…not really bad but not nothing either. Her G tube had stopped draining the obvious blood mix from last night and was back to its normal greenish yellow.

She’s still on her TPN IV nutrition at almost 100% strength. Her J tube feeds have increased to 15ml per hour. She was at 35ml/hour before surgery and they would like her to be at about 55-60ml/hour for full nutrition. Problem is that her guts have not really fired up yet. Yesterday she had her first poop since surgery (ya gotta love the post-surgery poops!), but nothing since and her bowel sounds are faint at best. That gut need to get crankin’ here pretty soon.

ICU VisitPhysical Therapy stopped in and we all decided together that Josie needed to go for a bike ride. So she walked a little bit to warm up and then hopped on the bike for a cruise down and around the halls. Josie was not pleased at all about this bike ride today…she left her cranky pants on from yesterday, so it was with great reluctance that she pedaled along. We stopped in to visit our friends in the ICU. They were all amazed at how good Josie looked (of course Dadio forgot to comb the Princess’ hair) and that she was up and about so soon after the big surgery. All the doc’s and nurses over there think she’s pretty cool. I think everyone at that hospital knows and loves Josie!

That ride was a lot of work and shortly after finishing she was studying the vein pattern on the backs of her eye lids. Later she wasn’t feeling that well and started her retching thing again for the first time since the surgery. This was not a welcome sight and she did not like it at all. I think the retching was hurting her doubly, not just the vomiting and nausea, but the tightening of her abdomen during each retch contraction must have hurt like a bugger.

Her blood values are holding steady. ALT and AST are still elevated at just over 100, bilirubin at just over 6, no sign of pancreatitis, all drains are draining properly (I’m pretty sure). In fact they said they were going to remove one of the drains tomorrow…I’ll believe that when I see it.

Slow improvements, and lots of activity…just what the doctor ordered.

Wednesday, February 21, 2007

2/21 Out of the ICU

Josie is surpassing all expectations so far…but not without some challenges. Yesterday we had the brown ooze, which the surgery team semi-dismissed as semi-normal (really?!). Indeed that’s what they said. I’ll believe that in a couple days, but for now I’ll just take their word for it with a skeptical eye.

She had her weekly IVIG dose and steroid bolus overnight. Those two doses back-to-back make her really REALLY moody. Last week she was absolutely giddy and joyful over the next 24 hours. This week…well…not. I’m reminded this evening of Linda Blair as a child. Josie is being a super grouch. She definitely slipped on the cranky pants this night.

Around mid-day Josie was released from the Intensive Care Unit to the good ol’ 5B ward. As expected we did NOT get the fancy room. But we’re just happy to be making strides in the right direction. Leaving the ICU is definitely a step in the right direction.

Shortly after settling into the new room Deb and Josie decided on an adventure. Today was about 50° and sunny here in Minneapolis. Josie wanted to go outside…and so she did. Mom and Josie headed outside for a quick little stroll, which was very refreshing for both of them. After coming back inside they stopped into the gift shop and Josie wanted to stand and look at the stuffed animals. She stood up unassisted for about a minute checking things out. This is great to see…awesome progress!

She finally calmed down from her crazy attitude and started acting a little more civilized. Later tonight Josie was sitting up and we were getting ready for a heated game of Candyland when all of a sudden she let out a scream of pain which was startling. She was hurting in her back and needed to lay down right away, then to her side. Shortly after, the output of her Gastro Tube changed color from a greenish yellow (I know…a little too much detail) to a tinged-marroonish-brownish-greenish-yellowish-not-sure-what-color-that-is color. She calmed down after about 15 minutes of crying and then fell asleep 15 minutes after that. The G-tube fluid did test positive for blood so whatever happened to cause the pain also caused some bleeding and that blood found its way to her stomach. The good news is that the other drains did not change their output, but one is still draining frank bright red blood, not a lot but fresh blood just the same. Strange. This isn’t the best news we were looking for.

So as we move along Josie’s continuing her reputation of making things interesting. She seems to love making people wonder.

2/20 There Just Has To Be Something

Josie’s fever was back for a good part of the day but has calmed down tonight. I think we now understand why she’s having the fevers. When Josie sat up tonight a small spot on her incision from the surgery started bleeding and oozing brown stuff. Not a lot, but enough to say “…That sure doesn’t look like it should be coming from there…”. The Doctors agreed and they took some samples and sent them of to the lab for cultures (more cultures!). The surgery team was paged too and they came for a visit later tonight. They said they’d discuss it in the morning and check the lab status in the AM too. The bleeding stopped right away as did the oozing when she laid back down. That’s a little scary. This day brought a little more pain in the tummy, but still nothing like before.

All her drains are still doing well and draining exactly what they’re supposed to. One of the drains in her lower abdomen has pretty much stopped and they think they may pull that one out tomorrow or the next day. There are two others laying above and below the duodenum repair area. Those will likely stay for another week until they stop totally. Then there’s the drain right in the middle of the duodenum which will stay for two months at least. The last drain is the pancreas drain which goes through the skin, through the wall of the duodenum, up into the ampulla of vater (opening where the common bile duct drains into the duodenum) and up into the pancreatic duct to the middle of the pancreas. This is draining the pancreatic enzymes and keeping the duct open during the healing process. That one will stay in for a couple months too. All this is really very interesting, and very logical.

They started her feeding yesterday and increased it just a little bit today. She’s still not moving stuff along too well yet, but that should come in the next few days.

Generally speaking she’s doing really well, save the little oozing setback tonight. She’s scheduled to leave the ICU tomorrow and head back to the floor. It’s very unlikely she’ll be set up with that killer room again, but maybe. I’m just happy we’re outa there in less than a week. Josie’s is surprising everyone…she’ll be back in no time.

Monday, February 19, 2007

2/19 Steady as She Goes

Josie was fighting that fever much of the day today, but later it calmed down. They, of course, drew more blood for cultures after another 102°+ fever. They also changed her treatment to Voriconazole, a stronger anti-fungal, just in case that crazy Rhodotorula fungus Josie had back in October. Generally speaking though, the day went well. She was talking a bit but basically laid low much of the time getting some much needed sleep later in the day.

Fellin' well enough to color!She’s communicating better today with less gurgling brought on by the ventilator. The physical therapy gang were in and they tried to get Josie to do some stuff but Josie wanted nothing to do with them today at ALL…feisty…that’s good (at least for now).

Josie’s blood work is showing improvement. Her ALT and AST liver enzyme levels are half of what they were before surgery, but still elevated and about double the upper limit of normal. Her bilirubin level is still high at 6.8 mg/dL (normal is between 0.2 and 1.3 mg/dL). This is good news for the liver. Everything should calm down over time if this surgery holds and she can get herself off that TPN IV food. Dr Saltzman (surgeon) was in early today and thinks she’s doing just awesome. Well enough in fact that she may be moved to the other floor in another day or two. This is a surprise to me, but she’s doing so well that she doesn’t need to be in the ICU much longer.

She got the OK to drink water and eat ice chips and popsicles. This was good because Josie was just itchin’ for something to drink yesterday acting like she was going to prune up like a mummy. Just a little water now and again is a true quencher for her and makes her happy (for about a minute). Later tonight she was feeling well enough that she wanted to sit up and color. Don’t know how she can do it with the insult to her innards just a couple days ago, but up she is smiling and coloring.

Sunday, February 18, 2007

2/18 Extubated!

A great start to the day with some small challenges later. Josie’s morning started about as well as the last day ended. She was presenting just about perfectly. All her drains were draining the right stuff and the right volumes. Her heart rate was hovering at around 105. She was sedated pretty well during the morning but then started to stir.

The ICU docs did their morning rounds and said they would extubate (remove the ventilator and breathing tube) tomorrow sometime, but it was pretty clear Josie had other plans. She's So Cool!She was awake and fairly alert answering questions and trying to talk. Of course she couldn’t talk with the breathing tube going down her throat, between her vocal cords, and into her lungs. Josie kept talking without sound and asking for stuff in her groggy post-op way. They turned down the ventilator to assist her breathing with just four assisted breaths per minute on top of her normal breathing pattern. She did well with just four assisted breaths so they decided to pull the breathing tube this afternoon instead of waiting until tomorrow.

As soon as she was extubated her respiration rate climbed to around 40 breaths per minute from 25 while on the vent’. 40 is kind of fast but not too bad. She had lots of gooey gurgly gunk in her throat which is totally normal for people coming off ventilators. The trick for patients is that they need to cough up that goop so there isn’t that much stuff interfering with breathing. Problem with Josie is that she can’t really cough that well with the extent of abdominal insult to her…and THAT makes it HURT to cough. I can’t even imagine. So the other option to clear the crud is to send a suction tube into her nose and down the back of her throat down into the area of glop to suck it out. That seems super un-cozy…but she’s a trooper.

After calming down from the uncomfortable extubation process she slept for a long time. Soon after, her temperature started rising as did her heart rate. As of right now (9:45PM) Josie is sitting up(!)with a 102.6° F fever and a heart rate of 147 beats per minute. As we know by now, that high temperature immediately requires some blood cultures be sent to the lab just in case there is some kind of infection brewing. The other problem is that her blood pressure has risen pretty high to about 150/105 which is too high. They’re going to try to break that fever and that should help out with the hypertension (high blood pressure). So it’s not all roses...there just has to be some kind of kink thrown in for fun, right?

She was even cool enough to give me just a hint of a smile for the camera this evening.

Saturday, February 17, 2007

2/17 Calm As Can Be

After such crazy hubbub over the last few days, today was as mellow as they come. Everything is going perfect so far. The drains are draining the right things and the right volumes, her temperature is where it’s expected just north of normal, her heart rate is nice and…well...great for a change at around 130 Lots O' Stuff(and sometimes even lower), her blood pressure looks good too. Everything is going really well. The only thing of note today aside from a perfect start to recovery was that they removed the old PICC (Peripherally Inserted Central Catheter) line out of her arm. now that she has the brand spankin new Hickman line, there's no need for the PICC. PICCs are notorious for colonizing infection inducing bugs. If they can get it out, then get it out...and they got it out.

Josie remains totally sedated and intubated (on a ventilator with a breathing tube). She’ll stay that way until tomorrow afternoon, and maybe Monday sometime. If things continue to go well they may shoot for tomorrow to wake her up…and if history is any indicator that process should go pretty quick.

She’ll be hurt’n for cert’n when she wake up and we’ll start this pain management game all over again, but this time we hope it shouldn’t last nearly as long. We’re feeling real good about the state of things this go around. I almost feel as good as the day after she was born, because I really believe she has a whole new lease on life…one which many thought was highly unlikely.

Friday, February 16, 2007

2/16 Surgery Day Updates

Update Seven – 7:30PM:

Things are going swimmingly. All the drains are draining, her heart rate is down in the 160s, her blood pressure is much lower around 80/45...almost too low. She's so difficult to keep sedated they've resorted to using a consciousness monitoring machine. This thing is really cool...it had a sensor sticker stuck to Josie's forehead attached to a lead and the machine monitors Josie's electrical activity in Josie's brain and translates it into a number between 100 (wide awake) and zero (absence of brain electrical activity). Josie's been hovering around 80 and as it creeps it indicates that she's slowly waking. Then it's time to give her another huge hit of Fentanyl to knock her out a little more.

The afternoon and evening have been going well with nothing new to report. It's been a long day for Josie, and she's really beat the odds on this one. All those unlucky breaks she had last fall are now turning in her favor. We'll keep you posted as the days march on. And for this day...It could not have possibly gone better for Josie today.

Update Six– 3:25PM:

And we're totally happy about this! :C)Dr. Saltzman came out to talk to us at about 1:15 to tell us they were just finishing up. He gave us his take on the surgery and was very pleased with the outcome. He thinks it’ll work and is confident about her recovery.

Josie’s now upstairs in her ICU room. After five hours she’s completely done with this surgery and we hope for good this time! She came back with a 210 heart rate and a blood pressure soaring at 170/120…she was definitely uncomfortable and her vitals were telling us that. They gave 400 micrograms of Fentanyl, enough to drop an elephant, and she calmed down a little bit. Her blood pressure is now about normal and a heart rate of about 180. She’s still on the ventilator and sedated…and she’ll stay that way for a while.

There are three JP drains now, one drain coming from her pancreas, one coming from her duodenum, her GJ tube, and an NG tube (nasogastro out her nose). There are monitor leads everywhere, ventilator tubes from her mouth, syringes, bags, the Hickman catheter from her chest, the PICC from her arm, a peripheral line in her hand…and we couldn’t be happier. ..great news! Seeing this picture is a little startling, and it’s really interesting that we’re extremely upbeat about it all. This is all good. Her nausea should be better, fevers should be better, tummy pain should be better, everything should be better.

Today’s surgical procedure has provided Josie not only with an additional chance at success, but about 70% less chance for a leak in the wrong place. She’s well drained and in great shape. She ended up losing about 25% of her blood volume over the length of the surgery and has been topped off with an additional unit of blood. If the Whipple had occurred there would certainly had been far greater blood loss and with far greater risk.

So now we wait and help her heal.

Update Five– 12:05PM:

Dr. Vickers just came out and we had a nice chat. He seemed pleased with the surgery and the decisions made. They made the no-Whipple decision because it provided no added benefit but created a significantly greater risk of trouble. With this new plan there is only one major connection point (the reconnection of the duodenum) and a few minor cuts (gall bladder cut on the bile duct, appendix cut on the intestine, two drain cuts in the duodenum). Also, they decided to NOT close off the pylorus, instead they’ll insert a big drain which will remove anything going through from the stomach. They did the least traumatic surgery to archive the best results…and they still have the Whipple up their sleeve’s for later if needed.

Update Four– 11:45AM:

We just had another visit from the anesthesiologist who said everything is going very well. She’s needed one unit of blood so far and will likely not need any more blood products for the rest of the day. They’re still working hard on her and they should be closing her up by around 12:30. The closing process can take 60 to 90 minutes. Things are going great --way better than expected so far.

Update Three – 11:15AM:

Update Ahhhhhhh…how things change.

No Whipple!

The docs got in there and started dissecting her guts. They said the adhesions were major and everything was totally stuck together. It took them a couple hours to split everything apart so they could take a peek. During the dissection her appendix was all stuck in there too and was in a position where they decided that the best thing was just to take it out…no more appendix.

Then they took out the gall bladder and got a better look at what they were dealing with. Her liver was looking large, green and dark…definitely sick looking. They took a larger slice of her liver and sent it off for further analysis. Her spleen was not interfering with anything so it going to stay. Good news. But there’s more good news…

When they got down to the real action what they found was a bit of a surprise. Her tissues are looking great! Her duodenum is still missing (I guess intestines really don’t regenerate themselves) but what’s left was in good shape. On the top part of the duodenum there is about one centimeter of tissue remaining south of the Ampulla of Vater, which is the opening of the bile duct into the duodenum. All three surgeons discussed it and agree that there is enough good tissue to work with. So now there’s a new plan.

The south part of the open duodenum is in good shape too so they are going to trim off the end of that and swing it up and attach it to the north part of the duodenum which will close the GI tract. Also, because she’s been healing so long and the duodenum eroded slowly over time rather than being cut, the crazy blood-flow problem is not as much a problem with Josie and they can get this thing done without removing the head of the pancreas and common bile duct a Whipple requires. They’ll leave alone the gastrojejunostomy (stomach emptying directly into the small intestine) and that’ll be the way her tummy will empty for the short term. They’re going to sew completely closed the pylorus (where the stomach normally empties into the duodenum). They will also insert a catheter directly into her pancreatic duct to drain all the digestive enzymes secreted by the pancreas so that digestive juice doesn’t interfere with the healing process. Josie’s pylorus will open back up after two or three month’s time. When the pylorus finally does open, the stomach should start emptying normally, and that will cause the gastrojejunostomy to close naturally.

This is great news -- but still makes us a little nervous because we’ve been down this road three times already with Josie’s duodenal tissue failing every time. The surgeon said this time is different because her Dermatomyosisit is much better under control and that should change the outcome. The tissue should heal well. This also gives us another chance if things head south again. That Whipple was a last-chance thing, and this solution holds back that final card for another dark day which we hope never comes.

They said it will take a couple hours to attach everything together and she’ll be on a ventilator when she goes back to the Intensive Care Unit.

Update Two - 9:50AM:

The Anesthesiologist came out to visit with us with a smile on his face. He said Josie was wide open and the surgeons were working hard. We asked about the level of adhesions (scar tissue from previous surgeries cementing everything together) in Josie's tummy. He said he didn't know but assured us the surgeons are excellent and they're really good at disecting that kind of stuff...that they'd take care of everything. No Problems so far.

Before the surgery Dr. Saltzman came in and was fired up about our Anesthesiologist. He said "...he's the best. Everything is aligning perfectly for today. This is going to be great...".

Update One - 8:45AM:

Just got a call from the operating room with an update. They said the Hickman catheter went in just fine and they just opened her up. Everything is going smoothly.

So far so good.

She's In There

taken this morning
Josie was wheeled into the staging area this morning just past 6:00am. We were visited buy the Anesthesiologist to go over all the sedation happening during the operation and later over the next one to three days.

taken this morning - 2/16/07Josie was giddy heading into the operating room. We got in there and she was full of spunk. She had the nurses laughing. The Anestheiologist gave her a few things and she got the itchy nose she was waiting for. The I asked if she was feeling fuzzy “…not YET!...”. Not 10 seconds later she looked up with the groggy Garfield eyes and softly slurred out “…I feel fuzzzzzzzy…”. Her eyes rolled back, then closed. Lights out at 7:50AM.

Good Luck Josie.

Thursday, February 15, 2007

2/15 Nothing Left To Do But To Do

Josie had a fine day. She carried over a lot of her spunk from yesterday and it made for some fun. Her physical therapy session went great and she was tearing around the halls on the little red bike again practically running down some of the other patients. When she gets healthy and strong she’s going to be a scary one…look out!

We had lots of medical visitors this afternoon. Dr. Whipple (Vickers) came in and sat down with us. We talked again at length about the surgery and I’ve got to tell you, I’m impressed with this man. I’m feeling real fortunate that Josie is in this hospital with this team of surgeons. They are some quality people.

We discussed the islet cell transplant proposed and are now in agreement that she shouldn’t go through with it because it could require an extended time on the table and her liver is not in the best shape as it is, which could lead to further liver complications. Also, she’ll be left with 2/3 of a healthy pancreas which should be able to generate all the insulin she’ll need for the remainder of her life. I’m also banking on the idea that Josie will likely not have other high risk factors for diabetes. So unless there is a change of heart with the surgeons during the procedure then the islet transplant is off. It would have been super cool, but the risk/reward curve was not leaning in Josie’s favor on this one.

She had quite the battery of blood tests this morning to provide information for the big day tomorrow. Her hemoglobin was low at 8.8 gm/dL which is pretty low, and rather than starting out the surgery on low they decided to give her a transfusion of blood this evening to top off the tank before they cut tomorrow. Nothing else interesting came out of the blood work today which we didn’t already have a handle on.

There’s nothing to do now but to do it. She’ll be heading down to the staging area at about 6:00AM and the surgery will begin at 7:30AM. So when you’re on your mid-morning coffee break, give a thought or two about our little angel on the table completely at the mercy of the three surgeons’ skills. We should get visits from the Doc’s periodically throughout the day on her progress and I’ll do my best to quickly post a post when I can.

I feel good about this whole thing and both Deb and I have a peaceful sense of calm before this most important of days. I certainly didn’t expect to feel this way, and it’s kind of nice. I just know it’s a done deal and she’ll be fine, she’s just got to go through it and get it in the rear view mirror. Stay tuned.

2/14 Happy Valentine’s Day!

Sweet JosieI hope everyone had a great Valentine’s Day with the one/s you love. We’re lucky enough to have our four little goblins to celebrate Valentine’s Day with. And now I’m here with the most precious of all my Valentines this year.

Josie’s day was a dandy. She was feeling great most of the day, despite spiking a 102.9° fever this morning and it never dropping below 100°. If that happened to me I’d be in bed seeking sympathy and complaining that I’m about to die…but not Josie. Tonight she’s pretty much bouncing off the walls and chatting my ear off. I’ve been trying to start this post and can’t get two words typed before Josie demands that I look at her so she can tell me about the salt pattern on the Saltine Cracker in her hand, or about the amount of hair on the head of the Doctor this afternoon, or the conversation she had with the nurse when I was in the bathroom, or the games she played with Grandma and Grandpa, and on and on and on. And that’s just fine with me because I’m not sure when I’ll have the privilege of being interrupted by Josie’s spunky wit again -- because Friday is fast approaching.

Preparing For Friday

As y’all know Friday is Josie’s big day. One of Josie’s surgeons, Dr. Saltzman, sat us down to go over the surgery one more time. Here’s what’s going to happen:

Josie will undergo a Whipple Procedure or more fancily called a Pancreatoduodenectomy. Friday morning at about 6:00AM Josie will be wheeled down to the pre-op area to get ready for the operation. We’ll go over the paperwork and then gown up ourselves. Deb and I will head into the operating room with Josie and be with her as she drifts off to sleep. We’ll immediately leave and the docs will get underway with their busy day.

The first thing they’ll do is insert a new and more permanent central line called a Hickman Catheter. A Hickman is an intravenous catheter used for the administration of medications. Hickman lines are much more durable than the PICCs Josie’s had for the last nine months and they aren’t as susceptible to infection as a PICC which are the main reasons they’re putting one in. The insertion of the Hickman involves two incisions, one at the jugular vein and one on the chest wall. At the jugular, a catheter is inserted into the vein and advanced into the superior vena cava (big vein near the heart). It’s then tunneled under the skin to the second incision. The first cut is then sewn closed. They’ll use ultrasound and X-rays during the positioning of the Hickman.

After that the action starts. They’ll slice open her belly along the same scar line from the previous three surgeries. Josie’s insides will undoubtedly be a solid mass of guts and organs cemented together with adhesions (fibrous scar tissue binding tissues together which shouldn’t be connected) created by all the man-handling of her guts during the three previous surgeries. They will spend a long time dissecting these adhesions separating her innards so they can navigate inside her tummy.

They’ll then lift out her intestines and set them outside her body so they can get to the action underneath. They’ll then take a good study of what is what with her altered anatomy and then begin the repair. The area they’ll be working is way back in the abdomen near the spine so access could be tricky with her enlarged liver and spleen. If that spleen is in the way and is causing risk to the surgery they’ll have to remove it.

Pre-Whipple, Shaded areas are removedA normal GI tract is essentially a tunnel through the body entering at the mouth and exiting you know where. Along the way the body extracts nutrients from food by the digestion process. Much of the action of the GI tract happens inside and just past the stomach. Food empties the stomach through the pylorus (the stomach sphincter…y’all know what a sphincter is, right?), and then enters the first part of the small intestine called the duodenum. The liver creates bile which travels down the bile duct and is stored in the gall bladder. From the gall bladder the bile travels further down the common bile duct. Digestive enzymes are created in the pancreas and empty into the common bile duct mixing with the bile from the liver. The common bile duct enters the duodenum and that’s where the bile enters the GI tract. Food then mixes with the bile and further digests allowing the intestines the absorb nutrients out of the food along its length. This Pre-Whipple picture shows normal anatomy with a pancreatic tumor (the main reason most get a Whipple).

Josie’s current anatomy is very different than normal. A large part of her duodenum is currently missing and is open on both ends of the missing area. This area is where her JP drain is today and it’s draining the fluid leaking from the open ends of the duodenum. She also has a section of intestine attached to her stomach so food and water and her J tube can exit the stomach to the rest of her intestines.

During the surgery the Docs will remove the remaining parts of her duodenum all the way up to the pylorus (end of stomach when food exits) and all the way down to where the jejunum begins which is just past the ligament of treitz (ligament which is kind of like a hanger supporting the intestine in the abdomen). Next they’ll remove her gall bladder and common bile duct taking care not to cut too much of the duct. Next they’ll remove the head of her pancreas which is about 1/3 of the pancreas. The reason all that needs to come out is that it’s all involved in the same blood supply; they just can’t take out the duodenum without removing all the rest of the stuff.

Post-WhippleAfter removing all that there’s a lot of reconstruction to do. They’ll then take the jejunum (small intestine) and swing it up and attach it to the tail of the pancreas by sliding the cut part of the pancreas tail into the open intestine tube kind of like an arm going into a coat sleeve. They’ll stitch that securely together. Next they’ll attach the pylorus of the stomach into that same jejunum section and attach it. Next they’ll attach that little bile duct to the same section of jejunum. Within the bile duct connection they’ll insert a small stent to keep that connection open and flowing.

After all that they’ll decide what to do with her current gastric bypass (the section of jejunum currently attached to her stomach providing an exit for the stomach). They may remove that and sew up the hole in the stomach or they may leave it alone. If they choose the latter, Josie’s body will eventually decide her stomach doesn’t need that second exit anymore and will seal itself up over time.

If it makes sense, and we’ll decide this tomorrow, this will be the time that she’ll receiver her islet cell transplant which I talked about yesterday. We’re still on the fence for that procedure as it may add further risk to the whole operation and recovery.

After that they’ll place three new drains: one by the pylorus connection, and two by the pancreas connection. Then it’s throwing all her guts back into her abdominal cavity and sewing her back up.

She’ll remain in a sedated state for a few days after surgery on a ventilator until she’s ready to wake up. They’ll remove the breathing tube sometime next week and wake her up -- and then the real recovery begins.

This is the most complex abdominal surgery there is. They’ve slated eight to 12 hours for the operation and all three surgeons will be in on the action (Dr. Bob Acton, Dr. Dan Saltzman, and Dr. Whipple himself, Dr. Selwyn Vickers). With a little luck things will go great and she’ll be outa there quicker, but of course there are serious risks with this. There’s between a five and 10% chance she will not survive the surgery due to complications. Recovery can be really hairy for this because of all the cuts and connections. There’s a lot of little arteries in that part of the body and one can easily get cut and that’ll require lots of blood transfusions, and big bleeding can lead to other complications too. The pancreas connection can leak and that could cause real trouble later as that leaking stuff will start digesting her insides. Of course there’s a huge risk of infection with all this. Any one of these complications could prove fatal.

If things are not going well they may just stop the surgery in the middle and move her to the ICU for a day or two or three with her belly still open to give her a little break, then go back and finish the job. This is unlikely but very possible.

So, here we go. The time is finally here and hopefully we’ve been able to provide Josie with the best chance of success, and Josie has shown how incredibly tough she is. There’s no doubt her spirit will take her all the way to recovery.

-- You’ve all been reading and following Josie’s great adventure for months now and we know you’re going to be thinking of Josie a lot on Friday.

The great folks at Christ Presbyterian Church have invited everyone interested to visit their chapel at the Church any time on Friday to pray for Josie. The chapel will be open all day long. There will also be a semi-organized gathering and prayer time between 12:00 and 12:30 Friday afternoon so if you’re so inclined head on over the CPC on Friday.

I’ll try to post periodically during the day on Friday to provide updates as we get them.

We’re feeling better about this whole thing as time has passed and the big day has approached. We really feel good about the team of surgeons here who will be working on Josie. Her little life will be in their skilled hands on Friday, and we’re confident they’ll be bringing their ‘A’ game.

Wednesday, February 14, 2007

2/13 Gett’n Closer

Josie’s day today was filled with lots of retching and vomiting and a fever that wouldn’t go away. Things started out with Josie asking for the cup and feeling warm. Her temp was hovering at around 101° all day and tonight she upped that to 101.9°. Of course that meant blood cultures again. That’s just fine at this point because we really need to watch for infection this close to Friday’s surgery.

Josie had a great time with the Physical Therapy gang today. She went for a walk out in the hall and then for another bike ride, and then some painting, and all kinds of fun. For the most part Josie was a great participant throughout.

Her liver levels have improved slightly with her bilirubin, ALT and AST down, but not by a ton…just a little. But that’s a little better than before, and better than worse, and we all know better is better. Her liver is still really big and is still causing her belly to be hugely distended. That makes her real uncomfortable, and when it's all over she’ll be so glad when that tummy no longer hurts and she can see her feet again.

We’ve been talking about an interesting possibility which may become part of the surgery Friday and that is an islet cell transplant. Part of Josie’s Whipple surgery will require that the head of her pancreas is removed. The pancreas is an important organ in the in the digestive and endocrine system. It secretes digestive enzymes into the GI tract which break down nutrients, but the pancreas also produces the body’s insulin which helps your body use sugar by letting glucose enter cells. Since much of Josie’s perfectly healthy pancreas will be removed she’ll be left with the tail of the pancreas to produce all the insulin she’ll need. Indeed the tail of the pancreas is where most of the insuling producing islet cells reside and there fore the place where most of the insulin is produced. Still, there are lots of insulin producing cells in the head of the pancrease which will be taken out. This will make Josie a good candidate for developing type 1 diabetes in the future because she may not be able to produce enough insulin for her body's needs.

As a family, we’re all for the donor program, but because of Josie’s disease her tissues can not be donated. That means they’re just going to throw her removed tissues in the trash can. But that pancreas is laced with these wonderful insulin-producing cells called islet cells. The islet cell transplant process has a fancy lab process harvest all these precious cells from her dissected pancreas and later in the surgery they’ll use ultrasound and/or radiography to guide placement of a catheter into the portal vein of the liver. The islets are then infused through the catheter into the liver. Once the cells take residence in the liver they should begin producing insulin. A big problem with islet cell transplants is that in almost all cases a donor organ is used and the recipient’s body tries to reject the cells through the immune system process as the body thinks the transplanted cells are enemy invaders…but this shouldn’t be as much of an issue with Josie because she is her own donor. On top of that she’s already on heavy-duty immunosuppressants so the transplanted cells should begin working without much notice from her system – at least that’s the theory. Of course with everything there is a cost and risk. Possible risks include bleeding, or blood clots which could lead to liver failure, or introducing infection, or restricting/clogging the liver’s blood flow. We’ll be talking with surgery and the endocrine gang again tomorrow morning about this procedure along with the all other parts of the big surgery.

Tuesday, February 13, 2007

2/12 She’s Baaaaack

After one last night at home Josie headed back to the hospital today. This past week went really really fast for everyone. The admission was uneventful…they just hustled Josie right up to her room and it’s like we never left. The good news is that we’re no longer on that broom closet; this room is one of the standard rooms. Seems that we’ve had our time in the sweet suite and it would be a stretch to think we’d get that again. So now we’re back to the hospital routine we know so well.

There were the normal visits from the Doc’s which mounted to not too much. There were to constant ins and outs of the nurses caring for Josie…and we don’t have to do it anymore. All that nursing began to become kind of routine after a few days at home. It wasn’t so bad once we knew what the heck we were doing. There were no fires, and everyone survived. Of course Deb did most of the work as usual and that made for an almost guaranteed perfect week for Josie’s care.

Josie’s having a good time today chatting up the nurses and getting back to doing their jobs for them. There’s been a stream of people coming in welcoming Josie back. Of course everyone know what’s coming on Friday and we’re all eager to get it over with…to get her better…and get her home for good.

Monday, February 12, 2007

2/11 Part Two Coming Up

Today was the last full day Josie will be home before Friday’s big surgery. She started today feeling pretty bad… nauseous… tummy pain, and then felt better, then worse again, then better. A yo yo process we wish would go away, but never seems to.

Josie was up and walking a little today – the first day in a few with more than just a couple steps. She walked herself to the bathroom and she had a nice bath which she enjoyed. It would be great giving a batch without all the tubes and drains and lines all over and that time is coming.

Josie knows she’s going back to the hospital for a long time tomorrow; perhaps as long as a couple more months. She knows she’ll make it through and it’ll soon be over when she’ll be back here for good. We’re eager for that time when Josie can finally close this most difficult chapter of her life for good.

Sunday, February 11, 2007

2/10 Skating! (sort of)

Today was a lot like the last few. Generally Josie was feeling just OK. Some episodes of feeling pretty perky, but just as many times where the cup was in hand at the mouth. It’s about half and half these days. Too much feeling bad for my liking, but we have to deal.

Totally Fun on the IceThe Doc’s decided to change the admission again to Monday morning now. This will give us a chance to get all our stuff done tomorrow and then just come in to the hospital at our leisure on Monday. I can’t believe how quickly all this time has past and that the big day is almost upon us.

This evening we finally got Josie out on the ice with the rest of the gang. We were lucky enough to have a bunch of our good friends over for a skating party on this cold 6°F night. The ice was great and everyone had a really fun time, including Josie who watched us all from the front window and then came out for a short while to enjoy the crisp winter air. So after talking about it for so long Josie finally made it out on the ice. Even though she didn’t skate she made it out and that made my weekend.

Saturday, February 10, 2007

2/9 Up and Down Day

Josie headed to the hospital for an outpatient visit to get her IVIG and steroid bolus today. She also had a visit with Dr. Vehe for a once-over.

Dr. Vehe was happy with some things he saw but was frowning his brow on some others. Josie’s face is still splotchy, which is inconsistent with the rest of her skin presentation. Most other parts of her skin look pretty good (yellow, but good) indicating a better-controlled disease. Because of this Dr. Vehe ordered up a CT scan of her head and sinuses as a precaution to check for another sinus infection. Josie had a doozy of a sinus infection last spring which resulted in surgery to fix. The preliminary report on the CT was -- nothing remarkable is going on in the sinuses -- and we’ll get the official official over the weekend.

During her visit today Josie had labs draw and her liver enzymes are higher again, but not crazy-higher. Her bilirubin is back up again to 6.6mg/dL, ALT is 241 U/L, and AST is 220 U/L (normals are 0.2 to 1.3 for bilirubin, and 0-50 for both ALT and AST).

The GI doc came in for a visit as well and was not unhappy, but not really happy either with Josie’s appearance and overall condition. Both Dr. Vehe and the GI Doc (Dr. Khalid Khan)(great guy) would prefer that Josie come back to the hospital early so they can make whatever tweaks to her program in preparation for Friday’s big event.

Of course we’re game for most anything they suggest as we have great trust and respect for their opinions. So Josie will be heading back to the hospital on Sunday night, then we’ll have several days to settle in and prepare for Friday.

Until then though we’re still at home and busier than mosquitoes in a nudist colony taking care of our little princess. Generally speaking it’s going quite well. We just wish she was feeling a little better. There’s been a fair amount of retching and sitting with the cup to her face, and that means she’s feeling pretty bad. This afternoon and evening the steroids were kicking in making her a little crazy person freaking out about this and yelling about that, but she’s so small and cute and funny it makes the Sybil-like behavior almost endearing (but don’t tell her that…we don’t want to encourage her).

Baby it’s cold out there but Josie’s still itching to get outside for a skate. The times she’s feeling up for it though it’s either 2:00AM or meal time or time for a new IV or something, and then she feels crummy again in short order. Sometime tomorrow we’ll get her out there…and she’s gonna to love it!

Friday, February 09, 2007

2/8 Home is Great!

Nothing particularly interesting happened today, aside from all the business required to maintain our important little family member.

Josie been loving hangin’ out with her animals. Monty (our geriatric English Pointer) crawled up and snuggled right next to her. She loved it. She was petting him and scratching him. Then a few minutes later I hear a “…Dad…Dad…I want Monty to leave…” “Why” I said. “Because he keeps tooting. P U – he STINKS!” As I approached trying to keep from howling I entered the fouled air, and she was most certainly correct. There was something rotten in Denmark. It was sick. Of course Monty is sitting and minding his own business totally unaware of what he’d done. -- I Just had to share.

Most of today was just OK. She’s been hurting and feeling worse than she had much of last week. Can’t put our finger on it and it’s not really out of the ordinary, but she’s just not having more great times as was the trend over the last 10 days or so. Here fever is still ther but low around 100°. Still retching and vomiting. Still a pretty high heart rate at about 150 most of today.

Tomorrow Josie’s off to the University again for her outpatient visit for special drugs -- IVIG (human immunoglobulin), and a big bolus of steroids. She’ll also be visiting her favorite Rheumatologist for a once-over too. Her skin is looking pretty good. That increased appearance from last week never mounted to much, and I sure like that.

Home is awesome. Josie’s hangin’ with her siblings and relaxing in her own house as best she can. Even though it’s a lot of work it’s so nice all being together. The support and help and generosity of everyone – all of you -- is incredible. You’re all such a great help and we truly appreciate it all. We love that you read about Josie and are interested in her story. We’re humbled by all the caring and giving and prayers and thoughts and everything…thanks to you all. And it’s GREAT to have Josie home, even for just a short while before the next big chapter of the story…the climactic peak of this saga is coming in just a week…and for now we’re just enjoying our time together.

Thursday, February 08, 2007

2/7 Name of the Game is Nursing

We’ve got a whole new appreciation for the nursing professionals who have cared for Josie these past five months. The amount of work and the number of things to do to care for this girl are astounding. I feel like I’ve enrolled in a crash course at the Josephine Adams School of Advanced Nursing Techniques.

Let’s see…what happened in the last 24 hours?

  • Change a diaper, measure and record the weight.
  • Empty the JP drain, measure and record.
  • Change the JP drain bulb to a bigger one for overnight so we don’t have to get up every 90 minutes to drain it.
  • Turn off the suction, administer Methadone (for pain), keep suction off for 30 minutes then turn it back on.
  • Help her with a retching episode at 1:00AM, measure and record vomit volume
  • Give Adavan (for nausea and to relax, kind of like Vallium) – for this we need to get needle syringe into the little vile, extract .6ml of the drug and mix it into 5ml saline, then go to Josie’s picc, flush with saline, administer the drug slowly over five minutes, flush with saline again, lock the line with heparin, clamp the line.
    Now it’s 1:30AM – Are you kidding me?
  • Then a little sleep, up again in the night to a retching episode, rub her back, measure and record, back to bed
  • 7:00AM Change a super-wet diaper, and change the bedding from the leaky super-wet diaper, weigh and record, administer Methadone (8mg) for pain. Then drain the JP drain, measure and record. Chang the JP container back to the small one for the day. Take her temperature, record it.
  • Turn off suction pump, administer Protonix (20mg) for stomach acid, clamp the G tube for 20 minutes so the drugs don’t reflux up the suction. Remove clamp and turn on suction.
  • Add more feeds to the feed bag, mix 1/4tsp Viokase (amalase, lipase, protease pancreatic enzymes) into a can of liquid food. Pour food in pump bag, reset the pump for the new volume to run for the next six hours.
  • 8:00AM Wipe the inside of her mouth with Nystatin for thrush. Turn off suction, give Ursodiol(150mg) to get bile moving, give Amlodipine(2.5mg) for high blood pressure, give Enalapril(4mg) for high blood pressure – all in the J tube.
  • 8:00AM give Lasix(20mg) diuretic to get her urine moving, Methylprednisolone(3mg) a steroid by IV. Flush the IV with saline, administer drugs, flush, heparin lock, clamp – don’t forget to sterilize with alcohol wipes between.
  • 8:10AM change a super-wet diaper from the Lasix kicking in, measure and record.
  • 8:30AM turn on suction again, change another diaper, measure and record.
  • 9:00AM Take delivery of the Cellcept from the Home Health folks. Set up the pump for the Cellcept, attach the tubing, make sure it’s the right kind, prime the tubing with the drug, fix tubing into the pump, turn on the pump, flush Josie’s line, hook the tube up to Josie, hang the bag and store the pump in a safe place nearby and let that run for two hours. Drain the JP drain, measure and record.
  • More retching and vomit, change diaper, measure and record both.
  • If the pain gets tough then administer Oxycodone for breakthrough pain.
  • 11:00AM Stop the Cellcept, flush with the special solution because it’s not compatible with anything else (be careful on that). Administer Bactim(60mg) IV(only Monday Wednesday and Friday), a preventive antibiotic dose, flush the IV line, administer the drug, flush, heparin block, clamp. Empty JP drain, measure and record.
  • 12:00 take temperature and record.
  • 1:00pm Drain JP tube, measure and record. Administer Methadone(8mg) through J tube, rinse her mouth with Nystatin again, change another diaper, measure and record. Check the suction machine, measure and record volume sucked out of the G tube during the day, empty and rinse collection can and get the machine going again. Pull out new IV fluid bag from refrigerator to warm up for later.
  • 2:00 Add another can of food to the food bag, mix in pancreatic enzymes, reset and restart the pump.
  • 4:00PM rinse her mouth with Nystatin again. Stop TPN which was running the last 20 hours. Change the hydration bag (IV fluids), attach appropriate tubing, prime tubing, remove old bag, alcohol wipe connectors, attach new bag, attach pump to new bag, throw out old bag.
  • 6:00PM been changing and recording diapers all day. Pull out new bag of TPN IV food from fridge to warm up for later. Take temperature and record.
  • 7:00PM wipe the inside of her mouth with Nystatin for thrush, give Ursodiol(150mg), give Amlodipine(2.5mg) for high blood pressure, give Enalapril(4mg) for high blood pressure, give ascorbig Acid(500mg) vitamin C – all in the J tube. Empty JP drain and record.
  • 9:00PM Take delivery of evening Cellcept dose and administer just like the AM dose IV. Let it run for two hours. Administer Zofran for nausea, a disolvable tablet to place under her tounge if needed.
  • 10:00PM Start TPN - precisely measure and mix the three vitamins into the TPN bag. Get the right tubing, attach tubes to the bag, prime the tubes, set up pump, place tube in the pump, alcohol wipe Josie’s picc line, flush the line, attach the tube, turn on pump, hang the bag, set pump in a safe place for the next 20 hours. Administer Amitriptyline(2mg) for pain management in the J tube.
  • 11:00PM stop the Cellcept, flush with the special solution because it’s not compatible with anything else.
I think I forgot to change some diapers, take some temperatures, measure exactly what she drinks and eats (not a lot of eating). Between all that she might get a bath, and some books read, and maybe watch a movie. Maybe get her up and walking around a little bit. Get her in the bathroom if she has to poop (gotta eyeball that measurement and record). Pretty amazing how many syringes, needles, wrappers, caps, connectors, bags, tubes, fluids, medical trash this project is generating. I think medical suppliers have an outstanding business model because people in need need LOTS of their stuff.

But it’s all worth it having her home. I just hope Deb watches me like a hawk. I can just see myself pulling some kind of boneheaded move by, say, mixing the wrong thing with the wrong thing and causing a fire or something. It’s a great relief to know Deb’s got it all figured out, thank goodness for mothers.

Oh yeah, we have three other kids too.

Good times.

Wednesday, February 07, 2007

2/6 We’re All Home!

Welcome Home Josie
Can you believe it? Amazing indeed. We sure didn’t think it was actually going to happen but here we are all under one roof again after 161 days and nights in the hospital. We were sent on our way from floor 5B a little after noon today. When we arrived home Josie promptly started walking around the house and wanted to see all the rooms...to take a tour. I can only imagine being just over four years old and five months removed from your home...it must be like going away for five years for an adult. On her tour she spied a hat in her room and waned to wear it right away. It was really neat to see her so bright and cheerful and full on energy much of the day.

Of course the reality of what we were doing hit pretty hard when the Home Health nurse was at the house waiting for us with loads and loads of stuff for Josie. There are syringes and tubes and drugs and schedules and connectors and all kinds of this and all kinds of that for the next week.

Then we started going over everything with the nurse. I think it was at about 40 minutes when I stared to glaze over and drift off into the mist of my mediocre mind, fully aware I’ll need to be retrained by my lovely, more intelligent and far more capable wife, who by the way was spot-on during today's training. The suction machine arrived shortly after that and Josie was hooked back up. We got her J-tube (going to the small intestine) feeding was started right away and the IV feeds started later this evening. It’s really a big undertaking getting Josie home, and the Home Health folks are awesome keeping everything straight for us...the efforts of the nursing staff and coordinators who made this home visit a reality get a huge thanks from us. Walkin' all over the houseTalking with the nurse she said Josie might be the most involved customer she’s ever worked with…and looking at this list of drugs and gear laying all over the house…I believe her.

That Cellcept problem is still a bit of an issue. The shelf life of the drug is four hours so the Home Health delivery boy will drop off the first dose in the early AM and we then need to drive about 30 miles or so round trip to pick up the second dose in the evening. Not too bad a trade-off if you ask me.

Josie was hurting later in the day with some tummy pain and some retching and vomiting. Different venue but the game’s pretty much the same…some good times and some bad. The good times are stronger and she’s getting better. The bads are fewer and not quite as awful, still real uncomfortable, but not too bad.

Even though Josie has to go back for another one to two months early next week, it was just great being home tonight with the whole family. I went out and skated with the other three kids on our rink out front while Josie and her mom hung back inside in front of the warm fire surrounded by all our cats and dogs. Josie was itching to get out on the ice with us but it’s just a little too cold now at 0°F. Maybe when it gets into the teens we’ll get her out there but for this day by the fire was the right call. It’s so nice being home again after so long.

Monday, February 05, 2007

2/5 Going Home Tomorrow?

That’s what they’re still telling us. It seems like a non-event but it looks like it’s going to happen.

First thing this morning it sure looked like it’d be a different story when Josie spiked a 101.7° fever. Of course they drew some blood for cultures right away, like they always do, and I thought that was the end of the going-home ideas. For sure she’d be put on some new IV antibiotic and they’d really question the going home thing. But as a care team we agreed a while ago that because Josie had continued to present high fever spikes with no other hard evidence of infection that we’d hold off on treatments unless the fever was out of Josie’s comfort zone. This is good because her fever came down and they didn’t order up a new medication course…what she’s already on is staggering enough as it is.

Our nurse came in tonight and said “…so you’re heading home tomorrow, eh?” “I guess, but haven’t really heard much on the details.” I said, which is quite right…we all talked about leaving for a long time and in-depth for coordination, and I’m certain tons of work has gone into our week-long home visit, but now that it’s time we’re ironically little short on details. I’m completely confidant it’ll all come together tomorrow.

We’ll probably be taking off around 1:00 or so and then meet up with the Home Health people at the house to take delivery of the pallet load of drugs, pumps and supplies for the next few days. We’ll then go over everything, just like we did before our 30-hour home visit back on September 24th. Should be a fun-filled, sleep-deprived outstanding experience. All this is geared for a final strength push for Josie before the big day on the 16th. Can’t wait

Sunday, February 04, 2007

2/4 Another Super Day

Even though we woke up to 14 degrees below zero outside, and as the Mississippi River iced over completely, we were warm as buns in an oven peering through the window checking out the Minnesota tundra outside.

Josie’s day was a good one with just a couple bouts of nausea and tummy pain. She started out with a mild temperature of 100.7° but after an early morning nap she mellowed out and felt pretty darn great the rest of the day finishing up tonight felling a little crummy again. During the day she was walking without protest, laughing, sitting on grandpa’s lap for an hour or so and chatting with everyone.

She’s been hearing about how cold it is out and wants to go for a walk… outside. No can do Josie. Maybe when it’s 30° but not -10°. Thankfully she was OK with that answer. And another big thank you goes to Josie tonight as she actually allowed me to watch the Super Bowl rather than rerun #249 or Dora. Way to go Josie.

As of now we’re still a go for heading home Tuesday. Josie’s excited to get back home to her own house, petting her dogs and cats, with a fire in the fireplace, watching her brothers and sister skating on the rink -- and just maybe slide around on it herself. Should be a fun time, even if it’s only for a week before the next big stay here in the hospital.

2/3 Steady Improvement

Having FunJosie’s days are becoming more and more routine. There were a few bouts of nausea and vomiting today, but for the most part she did great. She had another great session with the Physical Therapy girls…they had her out doing lots of great things, and the way they do it Josie doesn’t even realize she’s working out.

Josie’s skin is looking just a smidge more splotchy, her eye lids are looking a littler redder, and her fingernail fold capillaries are a little more prominent. All are signs of increased Dermatomyositis activity, but nothing's looking too bad. She did this a couple weeks ago too and then it all calmed down.

Other than that it’s been a real low-key day. She’s just plugging along real nice like.

Saturday, February 03, 2007

2/2 Another Pretty Good Day

Things are still going well. Another good day with physical therapy. Because Josie’s done with isolation she was allowed to head out to the PT room. She hopped on her bike and tore through the hospital wings down to PT where she did awesome.

We’ve been talking about going home now for a couple weeks and we’re still planning on it but probably not until Tuesday. We need all of Monday to complete the coordination efforts with the Home Health folks ... to get all the drugs and stuff delivered to the house plus training again and anything else we need to do. Of course something can go wrong in the next few days to throw the whole thing off but that likelihood of that is becoming less and less. She’s been so stable lately we’re pretty sure things will continue to go well and we’ll be able to make it home for a week before coming back to prepare for the big day on the 16th. The Cellcept drug mixing problem is still a problem but we figured out an OK solution. Home Healthw ill mix and deliver the morning dose and then mix a dose later in the day when we will drive to their office to pick it up. They determined two deliveries each day was too just much for them. We're good.

The results of the MRI are in and they’re pretty much in line with what we’ve heard before. There is hepatomegaly (fancy for enlarged liver), enlarged spleen, no lesions identified within the liver or spleen, pancreas appears normal, common bile duct is normal size, they couldn’t see the confluence of the biliary ducts but determined everything is AOK with that because the ducts are of normal size. This is pretty good news indicating that nothing else seems to be wrong with the liver and spleen besides the TPN problems. We’re keeping fingers crossed that the spleen continues to shrink for the next couple weeks so it’ll have a better chance of staying with Josie during and after her surgery.

Thursday, February 01, 2007

2/1 Out of Isolation

One of our GI docs, the Liver Guy, took a look at Josie’s liver biopsy and seemed reassured that her liver structure changes are totally consistent with the effect of IV TPN feedings. The good news is that it’s almost a certainty that the damage is completely reversible over time once the TPN is discontinued. Josie is currently getting around 300 calories worth of TPN nutrition each day to supplement her feeding tube food. She’ll be on that low amount of TPN (I guess it’s technically PPN for partial parental nutrition rather than total) up until her surgery. After that she’ll go to full TPN until her gut starts moving again and then a slow wean off the TPN to normal food over time. Only then will we likely see improvements in her liver.

She also had an MRI today on her liver. They wanted to check again more closely this time to see if there were any duct blockages within the liver which would help further explain her liver issues. No results on that yet.

Today is February 1 and that means Josie’s no longer required to be in isolation. Problem now is that there are no other private rooms available for her so we’re staying in the broom closet until next week when we’ll either get Josie home for a while or stay and move to a different room.

The good folks on our case are working the project real hard to coordinate everything to help get Josie home for a bit over the next couple weeks.

Josie’s feeling strong and spunky more and more or the time. Overnight she was retching and vomiting from around 4:00AM through about 9:00AM but then after that she was really great. Today we went for a walk and she headed out of the room and down the hall. All the nurses were out cheering her on and she seemed to enjoy the journey out and back. It’s nice to see the progress. Keep gett’n stronger Josie!