11/24/2009 - Update

Now that it’s November (Thanksgiving time) I figure is long overdue (emphasis on L – O – N – G ) that I get you all updated on what’s happening with Josie and all her business. The Reader’s Digest version is – pretty good.

Now that we’ve got that out there I can take another six months off from posting. Thanks for reading. See ya.

……………..

Ok, I can’t leave it just at that…so here goes.

Since May Josie has completed kindergarten, had summer break, gone to a family camp for a week, been to the doctor many times, had gallons of medicine poured and injected into her system, had a surgery, started first grade, been to Florida for a week, started first grade, had tons of fun with friends, and has been generally living life and doing what most kids her age are doing. No professional surfing or mountain climbing…but pretty active indeed.

On the Dermatomyositis front she’s been doing fairly well. It sounds like a broken record but the disease is still active but subdued. Her raging calcinosis has improved which is probably the biggest and best news of the last bunch of months.

The rate of change is so slow it’s fairly challenging to see the improvement or deterioration of her condition. Thinking back, though, at how severe those calcinosis areas were on her knees, elbows, butt, and chin, it’s clear there have been reductions in the severity of the calcinosis. There have not been any of those pancake-batter eruptions lately (although those were kind of fun to pop and ooze – just like that zit you can’t keep your fingers away from, you pop it, gross yourself out, then want to do it again – why are we like that?) and that’s great news. There are just a few spots now where the calcinosis contents are clearly liquid within the skin, most of the calcinosis areas are hard or at least firm like a rubber eraser. Some are little white pebbles or rocks just under the skin, while other areas are more like sheets of hardness. Her forearms continue to be pretty bad and are lumpy and bumpy and a little disturbing to see and feel. There used to be two really big calcinosis spots on her butt that were causing a fair bit of discomfort when Josie would sit on a hard chair, but great things have happened with that keester of hers. One of the spots has completely dissolved…I mean gone…no sign of it at all. The other big spot has dramatically reduced so sitting is no longer a problem. Now of the rest of it all can see such great improvement.

Josie’s been fielding a lot of questions from kids asking what’s wrong with her. She cheerfully explains “… I have a disease but don’t worry, you can’t catch it.” The kids are great because they look and then ask, in contrast to some of the rude adults who just stare. It’s natural, and I find myself doing the same thing with others at times…can’t help it…it’s human nature. But I sure do notice the glaring sometimes.

It was just that unneeded attention which kicked us over the cliff for some surgery for Josie. She had two very noticeable calcinosis growths under her chin which seemed to be the biggest draw of gawks, so we chose to have those removed. Josie was in the hospital last week for a little cosmetic surgery to hack off those ugly bumps. Surgery went great and the stitches just came out a few days ago. We can tell already that it was a fine idea which looks to be very successful.

Josie continues to be a heavy consumer of drugs. She’s currently taking prednisolone (steroid), Cellcept, Metaclopromide, Diflucan, Tacrolimous, IV imunoglobuline (IVIG), Methatraxate, Bactrim, Plaquinil and probably a bunch others I’m forgetting about. Recent visits to our trusty Rheumatologist, Dr. Vehe, have gone pretty well. He seems to be generally pleased with Josie’s overall condition and progress, so he’s essentially not changing course too much. Perhaps a little weaning of steroids and adding a little time between immunoglobulin infusions might be a good idea, but generally it’s a let’s-not-rock-the-boat-too-much-here position on the drugs.

Josie still has her feeding tube in place and is still getting much of her calories overnight with the food pump. Sounds tough, but it’s no biggie, really. Just dump a few cans of the Ensure-like liquid food into a bag, pop the tube into the pump, reset the pump, hook it onto the feeding tube attachment thinger in her stomach, hang the bag and turn it on – no big whoop at all. The best part of a feeding tube, though, continues to be the administering of all the oral medication. That stuff tastes terrible, but all we do is shoot it directly into Josie’s tummy via the feeding tube so she doesn’t have to taste it at all. If she had to actually take all that stuff by mouth twice every day, I don’t think we’d be doing as well as we are. Taking medicine is no problem at all.

First grade is going great. Josie’s reading is coming along and we’re starting to get into some math concepts too, which is exciting (sort of). But mostly first grade is nothing but fun with great teachers and great friends and fun experiences. The tough academics can wait for now.

Recently we had a production crew out to do a story on Josie for inclusion on the website of our home health service provider, PHS. They’ve been doing great things for us by supplying us with many of Josie’s drugs, and the administering of the various infusions over the years. Here’s a link to the story complete with a little video too. http://www.pediatrichomeservice.com/our-patients-josie.php

Generally speaking, Josie is really thriving at home and at school and at life. She has tons of friends, is feisty and friendly and happy and fun and just about perfect (but don’t ask her sister, you might get a different answer). It’d be great if we could snap our fingers and make her better and totally healthy, but we’ll take the next best thing. Life marches on and so do every one of us with challenges and all. Josie is a glass-is-totally-full-all-the-time kind of gal, and that makes all of us who surround her much better because of it. She’s awesome.

5/21/2009 - Update

I know, I know...why has it taken so stinkin' long to post a post about our little darlin'. Whatever I would say to answer would be pretty lame and I'm fully on board with the fact that this kind of delay is completely unacceptable.

ANYWAY...

It's been so long I'm not certain exactly where to begin except to say -- inn a nutshell, I guess the last eight months have been more of and much the same. So that's it. No need to read further.

But if you must...

Josie is thriving from a growth perspective. She gaining weight and height at a nice clip and has now found herself within measurable range on the growth curve well into the normal range. That's great news. Her strength has continued to ever so slowly improve as well. Now she's taking stairs with little difficulty, running with the other kids (though a little slower than the rest), and generally playing around just like every other kid, just a little slower. She does need to be careful being active as she's still relatively fragile.

In just two short weeks from now school will be done and first grade will be on the horizon. Kindergarten has gone very well for Josie. She's made some great friends and has fit in perfectly. She's progressing both academically and socially right in line with all the other kids. After being so sick for so long and really having an abnormal early childhood, Josie has rebounded so nicely and is developing into a really fun little kid...so cool to see.

Probably the most immediate news is that we're back in the hospital for a short three-day stay. This time, though, it was a planned visit to administer a new drug cocktail which is hard-core enough to warrant constant monitoring during its administration. Josie's disease is still very active, but not acutely crazy like before. The big problem now is her raging calcinosis. Calcinosos is the abnormal deposit of calcium salts in body tissues. Josie has extensive calcinosis all over her skin and within her ankles, knees and elbows. The calcinosis was getting bad last summer and fall but over the last three months she's gotten a lot worse. Her arms and legs appear to have tumors all over them; her knees are so calcified they appear very abnormal. When people see them for the first time most are clearly shocked at Josie's appearance. Luckily the calcinosis has stayed away from her face and back for the most part, but she has developed a couple pretty big nodules under her chin which are starting to draw stares and attention. Josie handles the friendly child questions about her appearance well...and it's interesting observing the reaction of some who see her and try desperately to keep their eyes away. The kids just stare, but the adults just can’t help themselves…whatever.

The question of the moment is what causes the calcinosis and what can fix it? Both have the standard medical explanation; 'Idunno'. And it's a fine answer because they absolutely do not know. We've been trying so many drug combinations to try to get the calcinosis to stop but it's been getting worse. Earlier this month we headed back down to the Mayo Clinic in Rochester, Minnesota (nice to be close) and had a very pleasant visit with a big-shot rheumatologist (Dr. Ann Reed) who we saw three years ago when Josie was getting underway with this. Dr. Reed was impressed with Josie's calcinosis and provided us with some different ideas on new courses of treatment. Dr. Vehe and Dr. Reed have since spoken and discussed Josie’s case at some length and along with some other smart folks in the biz cooked up an interesting treatment regimen that will hopefully show some results sometime soon (soon can have so many meanings, can't it?).

We learned that this calcinosis is a known complication of Dermatomyositis and in the bad cases of the disease (like Josie's) calcinosis follows the really bad part of the disease's courses...kind of like fires after a nuclear blast (not sure if that's an accurate comparison, but there it is). As calcinosis progresses the pockets of calcium can be hard like rocks or firm nubs like erasers or liquefied pockets like pus-filled abscesses, or firm and large calcium sheets. Josie has some of each. Her knees seem to be the liquefied kind and are now starting to break through to the surface. One particularly remarkable one seemed to be getting worse and worse over the period of two months and started to really thin the outside skin as it grew. It looked like a humongous whitehead. One evening during a bath Josie called Mom in because it had finally popped, and what a spectacular and disgusting event that turned out to be.
The liquid calcium oozed out like pancake batter and there was lots of itDeb estimated a quarter cup of the goop came out (See picture). All I can say is WOW, was that something to see! Since then a few more have burst, but none with that kind of drainage. There are others that have broken through which reveal a little rock and now look like small white scabs. Most of the areas are not painful unless they get whacked pretty hard and then they hurt. If Josie spikes a little ...fever then most of the areas flare up, turn red and are painful...especially in her ankles and knees, and at those times it does become difficult for her to walk until her fever comes down.

So what to do? Josie had been on a daily injection of Kineret (rheumatoid arthritis drug which is supposed to stop a particular protein (interleukin-1) from causing more tissue damage and to reduce pain, swelling, tenderness and inflammation in your joints) for a couple months with no clear signs of help so we discontinued it (and Josie likes the idea of not taking a painful shot every bleepin’ day). She's been started up again on a small daily dose of steroids to try and stop the progress of the calcinosis but, again, it's unknown if that's had any effect. One thing for sure is that her appetite has improved since starting the steroids again. She's still getting 750 calories every night through her feeding tube to maintain her weight and calorie intake. Other drugs she's on are:

· Diflucan (Anti-fungal) · Tacrolimus (imunosuppressant) · Pantoprazole (Protonix: proton pump inhibitor to decreases stomach acid production) · Cellcept (Immunosuppressant) · Metaclopromide (Reglan: increases muscle contractions in upper digestive tract) · Methotrexate (a chemotherapy drug used in blocking the metabolism of cells) · Prednisolone (steroids) · IVIG (Intravenous immunoglobulin: concentrated antibodies from healthy people to boost immune response) · Sulfamethoxazole (Bactrim: Antibiotics) · Hydroxychloroquine sulfate (Plaquinil: Antiinflamitory)

We've decided to take a little more aggressive approach to the calcinosis problem by walloping Josie's system with a combination of a drug she's had before and a new one. Rituxan is a chemotherapy drug meant to destroy B cells (lymphocytes that play a large role in the humoral immune response). It seems that those B cells are the buggers causing much of the problems with Josie. She's been on Rituxan before with little measurable effect, but in the experience of some big-shot rheumatologists a few patients have responded well on the second go around with Rituxan after marginal first-round success.

The other drug is Pamidronate which is a treatment for osteoporosis and bone loss. Pamidronate pulls calcium into the bones from the body. The bones have a kind of sponge effect and suck up calcium...sounds like it should work, right? Well...there's a lot of calcium available in the body, which explains where all Josie's skin calcium has come from, but there's no way to know if this Pamidronate trick will pull the calcium out of her skin...which it might. What it also may do is throw the calcinosis a curve and interfere with the calcinosis process. There have been a few known cases where calcinosis patients have been given Pamidronate and have shown their calcinosis start to rapidly melt away.

To administer this cocktail Josie had to be in the hospital. It's a good thing we were there too because within a few minutes of the infusion starting Josie had a bad reaction, changed color, started feeling bad, vomited...classic bad drug reaction. Things were put on hold and then restarted again after a little while. Since then things have gone well. And Josie cannot wait to get out and back home. This time, though, it looks to be a short stay.

So there you have it...eight months wrapped up in a rambling mess of a blog entry. Again...it’s much the same and more of the same, but all-in-all, things are going well.

9/12/08 - Update

It's been six months since our last post (I know, I know). A lot has happened, but things are generally much the same with Josie.

Indeed, much has happened: Preschool is done. Summer has come and gone. Lots of family birthdays. Lots of trips to the pool. Lots of trips to physical therapy. Lots of visits with doctors. Lots of infusions. A wonderful family vacation in July. The loss of a pet dog. The addition of a pet cat and new dog. And perhaps most important...Kindergarten has started.

With all of that Josie is basically the same, medically speaking. Her Dermatomyositis is still smoldering, still active but under relative control. There have been no flare ups in the last six months so her skin is basically pretty good. The biggest problem now for Josie continues to be her extensive calcinosis. Josie's forearms are now firmly encased in a cast of fairly thick calcium sheets. Running your hand across her forearms is a little unsettling...it's like the texture of a skin covered potato...hard and bumpy. The surrounding skin looks thin and shiny. Touching her forearms gives me the feeling that I'm causing damage to her skin, though I'm not.

The calcinosis is not just in the forearms, but just about everywhere...scalp, neck, chest, underarms, back, butt, thighs, shins, calves...though not as extensive as her forearms and knees. Her knees look pretty normal, but feeling them reveals the opposite. It's like she's wearing hard-shelled knee pads under the skin. A few weeks ago she was complaining of knee pain and was having difficulty straightening one knee. You could see calcium knee pad under the skin seemingly cut into the low part of her thigh when she straightened her leg. A couple doses of steroids worked like a charm to stop the pain and now Josie isn't feeling any current acute discomfort from the calcinosis, though it's quite tender to the touch, and hurts like a bugger if it's whacked.

So what to do about it. It remains unclear since Josie continues to be an outlier on the bell curve of this disease. We're starting to kick around the idea of a stem cell transplant down the road, which would be radical treatment for Josie since there has never been a Dermatomyositis case treated with such a procedure (as far as we can tell). It's risky and scary and a long way off...just throwin' the idea around now so if no further progress is being made over the coming year or two then we'll dive into that idea further.

Josie's spirit continues to be 100%. She's full of it. Her strength is gaining still, though at a snail's pace -- gaining strength about as fast as she's gaining height, noticeable over time, but not day-to-day or week-to-week. At school she's one of the gang...just a little slower. Not too much jungle gym work, but in class she's right in step. She can easily maneuver the staircases too.

It's still medication central on a daily basis for Josie. IVIG infusions every other week (which our insurance company must dread at around $10,000 a crack (yes--ten thousand dollars)). It's those infusions which our primary Doc (Doctor Vehe) seems confidant is keeping Josie as healthy as she is. Good health is expensive, indeed.

We've started a new TNF (tumor necrosis factor alpha) inhibitor medication, Humira, which we are hoping might have some positive results. Humira helps prevent inflammation (not like ibuprofen) by binding to tumor necrosis factor alpha (TNFa) which is a cytokine (a category of signaling proteins which are used extensively in cellular communication) involved in systemic inflammation and is a member of a group of cytokines that all stimulate the acute phase reaction. TNF is a crazy little bugger natural in the body that causes apoptotic cell death, cellular proliferation, differentiation, inflammation, tumorigenesis, and viral replication. TNF's primary role is in the regulation of immune cells. So being able to control TNFa is thought to help prevent the immune system from malfunctioning and attacking good parts of the body. The big problem with Humira, as with the other TNF inhibitor's she's tried in the past, is that it beats down the immune system further making her a prime target for bugs and fungus, and that can cause all kinds of trouble (you've probably seen some negative press on Humira lately). So we'll see how that goes.

Josie's appetite is improving too, but really slow - just like her strength. She's eating a tiny bit more at each meal, still it's not a lot. She's been known, though, to eat an entire brat at one time (gotta love brats, but for Jos' it means no bun or onions or ketchup or mustard or relish). And she's been known to enjoy my favorite Hamburger Helper meal as one of her favorites too (much to the displeasure of Mom). She's still taking in a liter of liquid food by feeding tube every night which is where she's still getting the bulk of her calories and nutrition. As she eats more we'll start thinking of weaning the feeding tube, but that looks to be a ways off still.

The rest of our kids treat Josie just like any other, though they're all aware of her limitations and do cut her some slack where appropriate. But that's about as far as the slack goes so she gets her fair share of grief from everyone. And that's just fine.

So what's happened the last six months? Life has rolled along, just like with you I'm sure, with ups and downs, happy times and sad times, successes and failures, fun and challenges. And in a nutshell, things are good and ever so slowly improving -- and Josie is in SUCH a better place than she was two years ago.

3/3/08 Update

Wowsers, is time marching on or what!? Another day, another week, another month pass on by and we’re still plugging along, scratching and clawing our way toward a full recovery for Josie.

So what’s been happening these last three months (sorry about that…that’s for sure my bad)? Well…it’s sort of a mixed bag to report. Generally speaking she’s doing great, but examining the components of the whole…some areas are not so good. Her underlying disease is still very active, and that stinks to high heaven.

We’ve been boogying Josie around to PT and doctor visits and this and that. The grind of her lifestyle is now our norm. Her relatively stable condition makes it pretty easy to handle the schedule, though.

In December, Josie had a follow-up visit with the Endocrinologist who wanted to get a read on her growth progress. Things are growing! At that time she had grown 1.5 inches in the previous few months…and that’s awesome! She’s now on the height charts…only at the 3rd percentile…but she’s on the charts. The trend line is really zooming heading to the meat of the curve of normal – that’s for height. As for weight though, that’s a different story. She has not gained any weight in the last 18 months. It’s a steady flat-line curve…a concern for sure, but no one’s really freaking out about it.

Josie’s appetite has increased just a smidge during the last few months. She’s still getting almost all her calories from her feeding tube overnight every night. We’re trying to throw between 1000 and 1200 calories each night and then squirt in another couple hundred during each day. Whatever she eats on top of that is just bonus calories. She’s always eager to eat, but the quantities she takes in are just so small…like two bites of this, and a sip or two of that. We’ve tried lots of different approaches to getting her to take more food normally including appetite-stimulation drugs, but even that doesn’t seem to work – at all. It’s a struggle to get her eating volume up and it’ll continue to be a struggle with no end in sight as we continue to move forward.

Josie’s spirits are really great. She’s happy, feisty and fun loving most of the time. Her spot-on Mr. Hyde impersonation has largely been absent recently and he rarely makes encore appearances (R-O-L-A-I-D-S). She’s having so much fun at pre-school these days…she just loves going and has a great time every time…she’s smiling when I drop her off and smiling when I pick her up, which is so cool.

Josie’s strength is coming along very slowly. She’s able to maneuver small staircases now without too much trouble. She’s still a little wobbly and is easily knocked over so she steers clear of precarious balance situations…and she doesn’t like it when our big dog comes running into the house. Physical therapy is going well and progressing, again, slowly. She likes running and is just barely leaving the ground on some steps now rather than the funky speed-walk running gait she’s become known for. It’s hard to tell if her strength is being hamstrung by the disease still or if it’s just a slow recovery after being so beaten down and withered from last year.

Perhaps the worst thing Josie’s dealing with now is calcinosis. Calcinosis is a condition where calcium is deposited in her skin. These deposits create nubs and bumps and disks and hard patches which range in size from a BB to the size of a quarter with the biggest being about the size of a playing card on the back of her left leg. This calcinosis has been coming on for some time but over the last three months its progress has accelerated significantly. She now has dozens of bumps and lumps all over her body with concentrations in her arms and upper legs. These areas are painful to the touch, but most are generally pain-free unless touched with pressure. A further problem Josie is facing now is that there are at least 10 larger bumps on her butt area and the back of her legs which are causing some pain while she sits. If the calcinosis-building process doesn’t stop then these irritated areas could actually wear completely through the skin, which will mean extreme pain and possible infection. There’s no way the good Doc’s know of to stop or reverse the calcinosis process. They do know that if they stop the disease then they’ll stop the growth of the calcinosis areas and then over a long period of time they should eventually dissolve. But, if the bad areas get super bad or way too uncomfortable then we’d have to consider surgically removing some of the nastier ones.

Josie’s medicine regimen is till hefty and dynamic. Cellcept, Methotrexate, steroids, IVIG are the biggies. Vitamin C, Vitamin D, Calcium, Diflucan, Ursodiol, Protonix and Bactrim are all taken daily to help. It’s tough to say what the right cocktail is, and this bizarre disease is so hard to treat. It’s not like a bacteria or virus…it’s simply a matter of telling her body to stop attacking itself. Such an easy concept, but it’s proven to be a futile quest for almost all autoimmune diseases.

So we roll along while keeping the faith that it’ll all work out. Josie’s proven to us that she’ll keep on fighting...what else would you expect, right?

12/3 Birthday Fun

Josie is five years old! What a fun day yesterday. We had some friends over with cake and decorations and presents and ice skating and a cozy fire and everything…it was perfect!

Josie had been looking so forward to her birthday for some time now…counting down the days until she would be five. And when the day finally came yesterday she was very pleased with herself. She did make a point to inform us that she was no longer a child…she is “now a big girl”. And “five-year olds are very smart…a lot smarter than before”. So now that we’ve been straightened out that we can move on to college.

The weather has been good and cold – just perfect for finishing up the skating rink, which Josie’s been very eager to try out. She gave it a go and was out there skating with the whole family. Her sister, with whom she argues regularly, was helping her move along with great encouragement, while at the same time her brothers were more interested in racing around the rink insisting they would be careful and not wipe her out. Seemed to work out well for the most part. Now to get her confidence and wobbly legs to work together, then we’ll be in great shape.

Josie had another dose of IVIG on Wednesday. It’s just an ongoing maintenance struggle here…all the medicine is now a total routine. We draw up all the syringes of liquid medicine (ursodiol, protonix, plaquenil, cellcept, vitamin C, vitamin D, calcium, fluconozole, (maybe a couple more…I’m trying to visualize the med-drawing routine in my head here), and fire it all right into Josie’s feeding tube a couple times each day (in fact, many times Josie just does it on her own. On those days I have to do a double take and ask her if I did it, or what happened, as I get very confused sometimes…and then she straightens me out again). After that, in the evening, we hook her back up to her feeding bag and pump for a night for continuous food –– routine.

And that routine is keeping this nasty disease held back, and it’s trying to break through with its relentless pressure and patience… just waiting there for a little opening and, wham, it can come back with a vengeance. But Josie’s doc’s are watching close, ready for anything that may rear up. For now it’s maintain, and wait and watch.

Everything else is going pretty well. Josie’s Mr. Hyde imitation performances are still coming, but not as frequently. Maybe I can use the I’m-ready-to-go-to-University-because-I’m-a big-five-year-old-and-that-makes-me-so-smart psychology on her when she gets into a Hyde-summoning snit. Think that’ll work? Worth a try, I guess.

11/27 Steady Progress

First thing first…I apologize for not posting more frequently than I have. I know many of you check Josie’s site daily looking for more news and updates. I’ll work to get these posts up on a weekly basis moving forward…and thanks for reading and all your continued support for Josie and our family. It means a lot to know you’re out there thinking of her during her recovery.

Three weeks have passed. Thanksgiving has come and gone. The Holiday season is upon us. The weather has REALLY changed (4°F this morning – yikes!). And Josie’s hangin’ in. Not a lot of change in her condition these days to speak of.

Josie’s skin problems have simmered down quite a bit, and that’s really great. Shortly after having her PICC line removed much of the bad things which were clearly brewing seemed to have stopped in their tracks, pretty much. Her skin has calmed (but not totally stopped) and her fevers have become less frequent and less extreme too. Her vomiting still happens but less and less frequent.

Life for this little girl has become much more routine now rather than one of crisis management. I, for one, prefer the former. We’re still struggling with her nutrition battle, though. Every single night she’s hooked up to a feed bag with a liter of overpriced, unflavored Nestle Peptamen Junior…a bargain at only 32 little dollars a day. Josie’s feeding tube, of course, is still in place and will be for a long time to come (the good news is that Deb and I have managed to go three months without yanking that thing out of her…we’re going for a record…we’re about due for another yankage). The good news is that Josie’s appetite is growing. It’s growing real slow-like, but growing. I think time’ll cure Josie’s eating issues and she’ll learn and reprogram that brain of hers that food is indeed tasty, and kind of a necessity….but for now we’re just maneuvering along down her trail of eating challenges and making some steady and unhurried progress as we go.

Thanksgiving was wonderful. We all were in the Brainerd area (Nisswa, MN) visiting with Deb’s family. The classic Thanksgiving dinner was awesome, and all the kids (Josie included) were able to shake hands with the bird before we devoured it. Nothing but fun around here. It was a far better Thanksgiving this year than last, that’s for sure.

And now that the weather has dramatically turned wintery and we’re in skating rink and hockey mode. Our front-yard skating rink is all set up and ready to fill. Hopefully by this time next week Josie will have been able to get out there and skate on her own. I would have been skeptical about that idea sitting in the Intensive Care Unit one year ago…but here we are travelling on that solid Recovery Interstate. Things are pretty good around here.

11/5 Worse...Then Better…Again

Hospital...Again

After the positive bacteria growth from two weeks ago we all took a let’s-see-what-happens approach. Well, the fevers sustained and Josie was ordered back into the hospital on the 27th. She kept spiking 103°+ fevers even though she was pretty much radio-active with all the big-gun antibiotics on board. Better safe than sorry was the call, but it was really more of a we-know-there’s-something-pretty-bad-going-on-in-there situation.

The conclusion was that Josie’s PICC was infected because the blood sample from the PICC was the one which grew the nasty bugs while a clean-stick blood sample did not grow the bacteria. Time to replace the PICC. They had scheduled a surgery for Josie to have the PICC replaced for Monday, but since it was pretty clear the PICC was the bad guy here they just yanked it out while she was in the hospital that Saturday.

We got to thinking about things and thought we should at least consider not having a new PICC put in at all and just deal with sticks and pokes whenever Josie needed a blood draw or IV medication. We asked Josie about the idea and that she’d need fresh pokes with the big nasty needles every week or two. Not too surprisingly she said she could handle the pokes every now and then rather than maintaining and dealing with the PICC (which she did not enjoy much)…so that’s what we all agreed on. The Doc’s were on board with the idea too as they are always of the less-is-more philosophy. Besides, Josie takes pokes better than the toughest he-man I’ve ever met (she continues to impress the nurses and lab techs as she doesn’t even flinch at needles and pokes…she just holds out her arm and watches them wrap the elastic around the arm and plunge what must look like a railroad spike right into her arm – without the slightest hesitation – is really is quite impressive).

Surprisingly, Josie was discharged on Sunday with a fresh peripheral line in her hand and no PICC. We then gave her the remaining IV antibiotic doses over the next few days, and then she received an IVIG dose that Wednesday at home…and with that they removed the IV line and she was PICC and peripheral free for the first time in almost 18 months. One more tube down! Now the only remaining tube is for food, and that’s just fine.

Halloween

Halloween night brought out all the usual suspects, and Josie was out there trick-or-treating with the rest of ‘em. She was dolled up as a cat with a tail and whiskers and ears and the works. She was as excited as the rest of the gang to head out for the annual sugar mega-high. The weather was cool, but not too cool, and no rain…so it was perfect. All had a great time…and it was only after it was all over that we realized the camera was missing (can you believe it?)! So we may have to dress everyone up in their costumes again for a phony Halloween photo shoot. The next day there was a minor tragedy when the dog found Josie’s candy bag and ate it! Every spec…wrappers and all…the only thing left was the torn up bag and clear slobber smears on the floor. Of course this was simply a minor set-back as the other kids grumpily ponied up part of their candy mountains for Josie…but it’s not like she’s gorging on candy and blimping out…it’s more of a nibble here and bite there. Halloween is so much fun.

Better Skin

Things are good on the skin front. All those worrisome spots on her legs have magically improved. Dr. Vehe thinks the infected PICC was throwing her immune system into a fit which was causing this flare up. Since the PICC was removed a week ago those trouble spots have dramatically improved with her skin. This funny disease keeps us wrinkling our noses and shaking our heads with frustration and wonder. What’s next, we always think…and the conclusion is that it doesn’t really matter…we just keep adjusting and rolling with the situation, and hopefully make collectively good decisions along the way.

So…things are relatively great and we’re feeling pretty good for the moment. Certainly we’ve got issues with nutrition which we’re trying to get a handle on, and her skin isn’t totally awesome, and there are still subtle signs of disease activity…but she’s doing way better than just a couple weeks ago. It’s nice to see – and she’s not quite so crabby either (wish I could say that about her big bro’).

10/24 Septic

The results from Josie’s blood culture came back yesterday with gram negative rods…and that means there’s bacteria in her blood. This could have been very bad. The good news is that now she has lots of antibiotics swimming around in her system now, hopefully taking its toll on the bugs. Later today we’ll likely learn which bacteria is in there as it takes a couple days to speciate the critters and then we’ll know if the docs selected the correct antibiotic for the problem. She’s been on Vancomycin, her favorite, since Monday which is a high-powered antibiotic having great coverage which usually takes out lots of nasty microbes. The better news is that she’s been fever-free for the last 24 hours at least and had some good rest last night for the first time in five days or so. I’d say things are looking good with this latest challenge so far.

She’s back at Dr. Vehe’s office this morning for another visit. After his examination and lots of head shaking and frustrated brow frowning, we should have a new plan of attack for her skin problems. Who knows what it’ll be, but it seems pretty clear that aggression might be on the forefront, for the short-term at least.

10/22 Temporarily Back in the Hospital

The last few days have been kind of a drag. Henry (Josie’s big brother) came down with some kind bad bug last week, actually keeping him out of school for the first time in his life. Well, it appears that Josie has contracted whatever he has/had (maybe) has been sick as a dog these last three or four days. Usually the bug rears its ugly head at night and then calms down during the day. Her temperature has spiked at around 103.5° each of the previous three overnights, and the icing on that cake was the accompanying full-coverage vomit. That would be vomit in the hair, on the sheets, all over the body, on the pillow – unannounced and disgusting – yuck! Usually a dose of ibuprofen takes care of the fever, bringing it down nicely.

Yesterday Josie was feeling better…back to he spunky self. This was good, and no signs of trouble. She went to bed, then at about 1:00 AM she woke up vomiting…again…with a fever of 103.2°. Since this has become routine as of late, a little ibuprofen and a little sponge bath (we’d deal with the laundry in the AM) and that was that, we thought.

At about 5:30 AM she was awake again with another barf-bout and a fever at 104.5°…too high for our comfort, especially after so many nights with a high temp. We gave some Tylenol and another hour. If there was no break then we’d take her in. At 7:00 her temp was down a little bit to 103.2° – OK but not good enough – so we made the call to her trusty GI doc down at the University of Minnesota Hospital. He said that we should bring her in for some blood cultures and that there was likely some kind of infection in there deep.

Deb took Josie in to the Emergency Room right away where she was processed (paperwork never seems to end, ya know?). They took some blood from a fresh poke (Josie’s so awesome at taking needles…not even a flinch as she watched the tech drive in a thick needle into her arm…impressive) which’ll be down at the lab growing for the next few days while they look for bugs. The antibiotics will be mixed up and administered here this morning and then she should be discharged by afternoon.

So this is probably no big whoop in the grand scheme of things, but a teensy bit of an anxious moment for us.

As for Josie’s skin…not so good. She’s developing more areas of disease activity so later this week she sees Dr. Vehe, and he’ll no doubt be grumpy about it. That means we’ll likely be changing things up quite a bit to get a little more aggressive against the new skin action.

On the bright side, she’s been climbing up one stair without hanging on to anything…up and down without assistance. This is indeed progress worth noting.

10/17 Different Strategery

We’re still here, plodding along…trying to gain the upper hand – again -- against Josie’s disease. I’d like to report that it’s going great, but it’s not. Then again, it’s not going terrible either…it’s just -- going. Josie’s skin is still presenting signs of disease activity with areas of red splotchiness, and other areas continuing to show signs of breaking down again, but they’re holding fairly steady. Her finger nail fold capillaries have presented with slightly more inflammation over the last couple weeks, too. The last dose of Remicade didn’t appear to have much of an impact…but it’s hard to say whether her disease activity would have been worse without it … a.k.a utter frustration…and a big sigh.

Today Josie is getting another dose of IVIG which has always seemed to be a good thing. The real question is how best to now approach this disease attack. Our arsenal is the same, but it’s a matter of rejiggering the formula to convince her immune system that all is well and that it’s OK to leave her good tissue alone.

Her tube feedings are going pretty well, but we’re finding it difficult to get in all the calories she needs. We’ve become semi-slackers regarding the mandatory 1000 calories of tube feeds and absolutely need to redouble our efforts on the nutrition front. Still, Josie’s appetite continued to be weak, weak, weak. So last week we put her on an appetite stimulant drug, which seems to be working (to a degree). We’ve also tried throwing a meal into the blender on the puree cycle, and then injecting the dinner frappe directly into her stomach via the feeding tube. This seems to be a decent trick to load in normal food, but doesn’t help much on the eating-by-mouth-and-desire training regimen. Work in progress for sure, and we do need to pay more detailed attention this eating strategy.

Josie’s muscle strength is stable…some would argue it’s better, and I guess it could be, but it’s hard to tell week to week. Physical therapy is going well still and she’s working out hard and well during those sessions. She’s also working hard at play while at school, and she tries to run at some point of each day – especially at school, but is still not able to leave the ground like a normal running kid. She’s just not strong enough for that yet so she’s got the wiggly speed-walk running style thing going on. As her siblings are all starting hockey now, Josie is gearing up for lots of winter skating, and it’s hard to believe how quickly time has blown by this fall as we head downhill into winter over the coming weeks.

So we plug away and regroup our collective thoughts (more like to smart doc’s figuring it out) on how best to re-conquer Josie’s disease.

Thanks for your continued support of Josie and our whole family. We greatly appreciate all your heartfelt thoughts as Josie keeps fighting on.

I’ll get a nice picture of Josie on here real soon. It’s been a while, hasn’t it?

10/2 Disease Fighting Back

Unfortunately Josie’s Dermatomyositis is making a bit of a comeback. Over the last three weeks things had been pretty much the same with little change…that is until this week when her upper arms and thighs started showing signs of increased disease activity. There are splotches on her skin which are sure signs that things are worsening. This is real downer news because she was doing so well and improving for so long. Her medication had been slowly drawn back and she was looking darn good. It’s not like she’s got a flesh eating bacteria or anything like that…it’s just – activity. Her face is also splotchy and red and scaly…more signs of activity.

Last week Dr. Vehe was hoping to delay further medication, expecting things were continuing to go well. And it was clear he was disappointed at her appearance during her most recent visit last Wednesday. He ordered up another infusion of Remicade which Josie received last Friday. And it’s been our experience that Josie responds well to Remicade, but this time there appears to be not much obvious improvement…total bummer. Next step is to go with another hyper-expensive dose of IVIG (processed human immunoglobulin) which will happen in the next couple days.

On the physical therapy front…there’s really been not much change for some time now. The progress is so slow you kind of need to take mental snapshots in time to compare, and her mobility and strength might be just a smidge better over the last month, but it’s really tough to tell. Her appetite is still almost zip…just a few nibbles here and there. She gets almost all her calories through the tube feedings nightly. She’s been done with the nightly IV hydration for some time now. The good news about that is that she’s free from bags and IVs for most of every day, and that’s much more convenient.

The great news is that she’s so enjoying going to school a twice each week. There’s no hesitation. When we arrive she just heads on out and starts having fun with everyone. All the teachers there are so friendly and encouraging. When I pick her up in the afternoon, without exception, she’s always laughing and playing with the other kids. She really seems to have so much fun.

So, a new battle with this disease seems to have started, and we continue to soldier on with a frustrated smile. One of these times she’ll kick it. And I’ll say it again…we’re quite lucky to even be having this battle as the war was almost lost last winter…so we’ll take it.

9/10 What's New?

So What’s New With Josie?

Clearly we’ve not been updating the blog here nearly as much as in the past…and for good reason. Things are going well -- well…yeah – pretty well, indeed. Josie continues to improve in both strength and with her underlying disease, but the progress persists at a slow pace -- S – L – O – W.

The great news is that Josie’s Dermatomyositis is as quiet as it’s ever been since she was diagnosed! How cool is that? The continued tinkering with the medication formula seems to have done well so far (kudos to Dr. Vehe), now it’s a matter of carefully peeling away the medicine and get that immune system of hers working properly so it no longer thinks she’s her own enemy. Josie’s immune system is still beaten down with the Remicade medicine on board (she had another dose last week) and the evidence is showing that all this stuff is working pretty well. We skipped a week of IVIG to see what effect it would have and there appears to be no adverse effects so that means sort of an altered and staggered IVIG schedule, hopefully heading toward a complete weaning. Her prednisone (steroid) dose was stopped after being whittled down with no problems. It kind of feels like we’re building a big stack of blocks and we’ve got quite a nicely balanced formation on which we’ve placed some of the last blocks which seem precariously perched, and we’re slowly backing away with hands close hoping it doesn’t collapse, yet it still seems to be teetering and could fall any time. Hopefully, over time, we can walk away with confidence that it’ll stay up and solidify.

Two weeks ago Josie went in for a CT scan of her tummy to make sure everything was as it should be. She had this 8 ounces contrast solution which she was supposed to drink…and of course she doesn’t like to eat or drink anything. So we pull out the trusty 2 ounce syringe and pumped it directly into her tummy through the feeding tube. And I have to say, that feeding tube is sure nice to have…it completely eliminates negotiation for taking medicine or other nasty tasting stuff. Just draw it up and squirt it in – done. Sure, we’d rather NOT have a feeding tube, but now that it’s there, ya gotta love it. Anyway, the CT scan went just fine and there are no leaks in her system (good). There is a question as to which direction all her tummy contents are heading. If you’ll recall, her anatomy is no longer normal. She has two paths out of her stomach: the normal exit (the pylorus) into her altered duodenum and small intestine and the new exit which was created during all that nasty business last fall. The theory was that once her normal track was reestablished that the alteration would kind of fix itself and close off. This is a question which is up for debate and will likely undergo some testing to see for sure. There’s no alarm of anything like that, but it’s a question we’ll have to visit at some time as we move forward.

Hyde is still a problem with almost daily outrageously-ridiculous and unreasonable behavior, but it’s usually short-lived. When he shows his ugly self it usually is met with a couple eye rolls, a little patience and he scampers off…eventually (but sometimes a nap is required).

The biggest news of all is that Josie went back to school last week and is thoroughly enjoying preschool again. We get her all unhooked for her feeding tube and IV in the morning and drop her off. No separation anxiety at all…she’s pumped to be there and I really think she’s having a blast with all the great folks there and meeting new friends. When I pick her up in the afternoon she’s motoring around the gym laughing and carrying on…well animated and having some great kid fun. What a pleasure to see.

Josie’s appetite is improving, but like everything in this recovery, it’s going slow. She’s taking in more food each time she eats, but still just meager amounts and not nearly enough to sustain herself. She’s getting hooked up for nightly feedings and we pump in about 1000 calories overnight so most of each day she’s unhooked. Her weight is good and stable.

So all is (pretty) well with Josie these days. More to come.

8/23 Much the same…again.

Over the last two weeks Josie has had her routine fevers every three or four days with the most recent this past weekend lasting two days at about 102°. Because of that the docs are choosing not to do Remicade to treat Josie’s disease. Remicade, you may recall, really blocks the immune system from doing what it should and that makes someone very susceptible to any kind of infection. Of course having a recurring fever leads the docs to think that there’s a bug in there kind of hovering – attacking and retreating but not going away – and if Josie’s immune system is weakened further with Remicade then that bug (if it’s really there) will be provided the opportunity to take off and that could lead to some real trouble. So we’ll wait.

Josie’s still been getting her weekly steroids and IVIG (at about $5,000 to $8,000 a pop) – (!). But I don’t think anyone is questioning its effectiveness. She continues to improve, slowly for sure, but improving. Her physical therapy is moving forward nicely. She’s able to walk up and down short stairs (about half the height of normal stairs) without using a rail. Her duck walk is improving and her gait is becoming more normal. She still lands with a rigid leg…not quite locked…but not the flex of a normal walking stride either. She can move along pretty quick when she “runs”. It’s really more of a fast walk and she takes the form of a speed walker when she runs. As her strength improves she’ll hopefully start to leave her feet like a normal run, but that’s still a ways off, but it’s coming.

Her appetite is still very low. She’s getting all her calories from the feeding tube…still hooked up 14 or more hours each day. She’s taking in less than 10% of her calories by mouth now…but it’s improving too, and time and persistence are the only things which will help. Speaking of feeding tubes, we had another accident yesterday with Josie’s feeding tube being yanked out of her stomach. This time it was Mom doing the deed getting the tube tangled in the car seat, and POP, out it came. We hustled her inside and quickly inserted a new one (which we had on hand here at home just in case). We fixed it in place and that was it…no big deal. So Mom leads Dad in the yanking-the-feeding-tube-out-of-the-daughter contest 2 to 1. There’ll be a big prize at the end of the contest to be determined. It was probably time for that thing to be changed out anyway…the inside part of the tube sitting in the stomach was all black and gross…and I suppose it would be a little gnarly sitting in a stomach for several months with all that stomach business going on…so that kind of justification makes accidents like these a little easier to take.

Mr. Hyde has been making more and more frequent appearances, unfortunately. Josie’s been sleeping in her own room now for a while (which is great) but almost every night she’s waking up and incredibly ornery. Her grouchiness at times combined with the stubbornness of the most cantankerous mule, and the unreasonableness of the most bad-tempered two-year-old, makes dealing with this girl most demanding. Her tenacious insistence on getting what she wants is impressive, but not a lot of fun to deal with…especially for Deb…and that makes for some disrupted sleep at times.

The Long-Haul is the name of the game around here. Everything is looking better! But everything is taking so long to get better; the improvements are small and take weeks to see. It makes recovering from a pulled muscle or broken bone cut or bruise seem like a flash by comparison.

8/10 Time’s just marching right along.

Josie’s doing great! These fevers are still coming every three or four or five days or so, and of course it gets everyone a little bent out of shape (and for good reason). But she seems to be able to take care of it and fight off whatever it is that’s on the attack. Last week she was perfectly fine throughout her stay at the hospital…that was a case of being cautious with the blood cultures coming back positive plus a fever…but no problem.

Earlier this week she again had a 103.5° fever…definitely high, and worthy of a call. They did a blood draw and testing as well as putting her on a course of more hard-core antibiotics. Her white blood cell count was very high at 17,000 or so, which is certainly a good indication there’s trouble brewing. But the next day the fever broke and her white count was back down to 3,000 or so two days after that. We’re liking the way her little body is battling those bugs…now if it would just learn that her own body is not the enemy, we’d be in great shape.

Josie had a nice visit with Dr. Vehe on Wednesday and he was real pleased with her progress. Her symptoms, though not completely gone, have improved! This means things are better with her Dermatomyositis. He decided that it was time to make some further changes with her treatment. We went ahead with her weekly steroid bolus and the IVIG on Wednesday. We’ll be decreasing her daily steroid now and next week we’ll try Remicade again, along with the IVIG. The following week we’ll try skipping a dose of IVIG and see where she stands at that point. Then after a week off give the IVIG the following week. A fine plan I think.

In the mean time she continues to grow strong. She’s maneuvering stairs much better and is getting a touch more fleet of foot. Getting up and down off the floor or bed is no longer a problem. She’ll pick stuff off the floor regularly now too…all good. She’s eating a little more (just a little though) as we continue to feed her through her feeding tube 17 or 18 hours each day. That whole eating thing will take a long time to correct. She’s still getting 12 hours of IV fluids each day too.

Her Jekyll and Hyde personality is still an issue. She’s such a tenacious bugger that when she decides on anything, that’s the way it’ll be, no matter what. Hours of determined stubbornness seems to be involved in some of the most mundane requests…like if she wants something, to just say please or say it nice…she’ll just refuse…or if she wants Mom to put on her clothes but Dad says it’s his turn to do it today…we’ll get through all that too. Those are problems for sure, and I’m glad the be dealing with that than lots of other things.

Summer’s so great and we hope everyone is having a fabulous time. It’ll be coming to an end her real soon…before your know it we’ll be back to building skating rinks.

Guess Where We Are?

You guessed it.

After the successful PICC replacement on Friday Josie headed home…standard and routine. Then on Saturday there was a frantic attempt by doctors to reach us to let us know about the testing and culture results on Josie’s old PICC and blood samples. The results showed growth of gram negative rods which is an indication of a nasty bacteria. The big alarm of this news on Saturday was amplified because Josie is immune suppressed already and particularly now because she has Remicade in her system to continue to fight her Dermatomyositis. Everyone was trying to get a hold of us because this kind of bacteria can take over a body very fast and can be a killer if not treated right away.

Well, the great news is that Josie was not showing any indication of sepsis (actual blood infection) at that time and so all were quite relieved. We were given marching order to watch Josie closely and to keep close tabs on her temperature. If her fever was to rise over 100.5° then we were to call in immediately.

Well, Josie has had a lengthy history of fevers, all of which have corrected themselves within a day or so, some higher than others…but this time we had to be more careful. On Tuesday morning Josie had a 100.8° temperature. The Doc’s were called and we were ordered to head on down to the hospital. Now, Tuesday’s are Josie’s big steroid bolus and IVIG days from the Home Health folks. These doses were put on hold as we headed down to the hospital.

We arrived here Tuesday at about 11:00AM and they immediately took more blood samples: one from each or the two PICC ports and a third from a fresh arm stick. Josie had zero indication of further worsening fever…and in fact the fever went away after a dose of ibuprofen in the morning. Tuesday night and Wednesday morning Josie still showed no signs of any problems. She was feeling and looking great all day and the gears were turning for a quick departure. Then we were abruptly stopped almost at the door because the cultures from the blood taken on Tuesday grew some gram positive rods…again a bacteria. You’ve got to be kidding!

So Josie had to stay another night here just to be careful and make sure nothing was going on. Throughout all this Josie has shown no signs of getting sicker…all was perfectly well aside from the positive blood cultures.


Bridge Collapse!

Last night, unbelievably, the I35W bridge completely collapsed. This bridge is only about one mile from this hospital. Because of the closeness of this disaster and the initial numbers of casualties they thought were going to be involved, this hospital was put on Orange Alert and that more or less completely changed the operations around here. Expecting lots of people converging on the hospital they decided to lock down the hospital completely so no one was allowed in or out of the hospital for several hours after this collapse. Luckily Deb was in the hospital with Josie at the time, but I was turned away at the door at about 8:00PM.

As it turned out there were quite a few victims admitted to this hospital and the staff here handled it quite well. Our disruption was minor compared with many here in Minneapolis last night. We’ve traveled over this bridge at least a thousand times since we’ve lived here and being so close to this disaster is almost surreal. Seeing the bridge down in the water is really quite a sight.

Home We Go

This morning Josie is looking good and feeling good with no fever or and indication that this bacteria is infecting her. So we’re outa here sometime in the early afternoon. Hopefully we’ll be able to get the Home Health folks out to our house in the afternoon so she can still get the dose of IVIG administered which has been put on ice since Tuesday. If it doesn’t get in today then it’ll have to be thrown out, which would be a huge waste of a couple thousand bucks worth of good medicine.

So a wild few days should hopefully calm down to more normal ho-humness.

7/27 More Progress

Things are fairly routing now…even the persistent fever episodes. Lately, Josie’s been hot then normal then hot again then normal and on and on and on it seems to go. She’ll go maybe two or three or four days before the next fever arrives. Thankfully, they’ve all come and gone within a 24 hour period --usually. One time, about a week ago, she spiked one up to 103.6° and we had a bugger of a time getting it to calm down…so much so we had to give the good folks down at the hospital a call and let them know – and of course shortly after that she came back down to normal. Usually the fevers are accompanied by one or two vomits, which are no fun, but tolerable for the little munchkin who’s endured a lifetime of vomits crammed into the last year.

Her fevers being persistent but consistently inconsistent has led the Docs to order up a new PICC (central catheter in her arm). As happens frequently with PICCs, they become contaminated and bugs and slime which accumulates on the PICC material inside the vein. When the body fights off the mild infection caused by the PICC slime, the immune system gives the all-clear and calls back the troops. As soon as the bod’ becomes more vulnerable because the immune system has calmed, then the bugs on the PICC invade again and then it becomes a cycle… a seesaw battle with no ending really possible (at least that’s how this lay man has interpreted the explanation). Of course there is no definitive test to check if that’s what’s really going on or not without removing the PICC…so that’s what they’re going to do. In fact, that’s where Josie is this very moment is receiving a new PICC at the hospital. A fairly routine procedure, but still considered an ‘operating room’ procedure so she had to go into the hospital and be processed just like a major surgery. It should go quick because all they really do is insert a thin wire into the old PICC, slide the old PICC off the wire and then guide a new on onto the wire and then remove the wire…pretty easy. She should be outa there shortly.

Her strength is still gaining! This is good, and still so slow. I think she’s stronger than just before she broke her leg back in May, though that leg is still clearly weaker than the other. When she walks it’s not like a lock-legged duck walk anymore with the strong leg at least. When she lands on that leg there is a knee bend like a normal person and that means lots more strength in that thigh. Her broken leg is still a little locked while walking but showing signs of further strengthening. She’s getting around so well now that she’s been discontinued on the in-home physical therapy as they were simply doing a lot of the things she does all the time at home anyway…and that’s further good news. She’s able to go up and down one stair without help and regularly is getting herself down and up off the floor with greater ease and confidence and strength. Josie’s still going to physical therapy at the office twice each week where they do all kinds of fun workout games.

Josie’s skin is improving from the little flair-up from two weeks ago, yet it’s still clear her Dermatomyositis continues to be active with obvious signs. Her face rash is apparent, as is the hot-spots which are slowly healing, and the reticulated lacey rash on her upper arms, shoulders and back. Her color is ‘pleasantly pale’ as Dr. Vehe like to put it, and that’s better than bad..

Josie’s medicine regimen is still extensive with weekly Solu-medrol steroid boluses, weekly IVIG, daily Orapred steroid, Ursodiol, Protonix, Enalapril, Plaquenil, vitamin C, vitamin D, calcium, Cellcept, bactrim, and Diflucan. She’s had two doses of Remicade over the last month which seems to be helping (unsure). Also she’s on IV fluids 12 hours each day as well as 14 hours of tube feeding. Her appetite is low so she’s still not eating any substantial calories by mouth. Her weight is still about the same at around 37 lbs. which is about 50% more than last year at this time.

Generally she’s doing well. Her attitude seems pretty good most of the time. Her crazy behavior still rears its ugly head far too much – she’s a tough egg to crack, this one. But that’s an issue more on our shoulders rather than her problem…she’s just got us figured out right now.

So it’s slow improvement, but improvement just the same and we can only be grateful for that.

7/17 More Slow Improvements

Another week has passed and I’m finding myself simply shaking my head at the speed of time. Summer is half over already. There’s been camping, and a family vacation, baseball and soccer are winding down while overnight camps are just around the corner. Family life in summer is so busy but so fun.

Josie’s improvement has continued at an agonizingly slow pace. She’s getting stronger but at a snail’s pace…and the great news is that she’s getting around better than she has in over a year! Very good news indeed. As far as her disease goes though…she’s still battling. The lesions on her arm have now scabbed over and are slowly healing. These spots are interesting for sure…they’re not the normal lesion which is typical of Josie. They’re more of a scaly lesion rather than a deep vascular lesion like the norm of old. Another interesting aspect of it is that her fingernail fold capillaries are looking almost as good as normal, and those are a real good indicator of active Dermatomyositis. There have been no other trouble spots developing, thank goodness. Her skin is still showing a definite lacy rash over her back and upper arms, as well as a reddish rash about her face.

Dr. Vehe has been unimpressed with Josie’s skin condition, and though not as shocked as we were, he seems disappointed that she did not respond as well to her Remicade treatment. He ordered up another Remicade dose last week and it seems to have helped out. Josie’s skin is looking better now than it has in a couple weeks.

Her little Jekyll and Hyde personality is a tricky one. She’s so manipulative with her tenacious and unyielding demands on Deb’s time and attention. Many times she won’t allow me to do anything for her…totally depends on her mood…and then she’ll insist on Deb doing everything. What are ya gonna do? All the tough parents and shrinks out there promote some tough love, but one can only take so many 90 minute tantrums before caving. Most kids crack after about five minutes…but not our little miracle girl. It’s probably that personality and determination which kept her alive during those dark weeks late last year.

So we keep moving on hoping for the right formula for Josie’s drug cocktail…that winning recipe will be the one to slap her immune system around and back to its senses. It’s pretty hard to get your body to stop doing what it wants to keep doing…to get it to not fight what it thinks is the body’s enemy. It’s just weird, but it’s a weird with which we have to contend.

7/9 A Week’s Worth of Fun

It’s been a week away, and what a fun week it was! We were at Fair Hills family camp for a whole bunch of summer fun. Josie was in the full swing of things all week playing with all the other 4 to 7 year olds doing plenty of groovy things like water slides and playing in the sand and horseback riding and boat rides and swimming in the pool and eating (a teensy bit) and watching Mom and Dad eat like crazy and watching fireworks and fishing and baking cup cakes and arts and crafts and playing on jumpy things and all kind of other camp summer funness. We all had such a great time and none of us can wait to get back for next year.

Of course one of the things at summer camp which is almost unavoidable and usually desired is lots of sunshine. Josie was the queen of slather this week getting her morning, mid-morning, early afternoon, and afternoon cakage of spf 30. Still the sun is not Josie’s friend. Perhaps it was the sun, or maybe it was just a coincidence but Josie’s skin seems to have flared up pretty good. The spots on her arms are much brighter and look to be starting to break down and through...but that might be a little dramatic talk since they’re not open sores... yet. Her lacy rash is more prominent on her arms too and her elbows have fairly bright red marks on them. Josie’s face is quite a bit redder than it’s been lately. Clearly Josie’s disease is still in an active state, but the question is how to we regain control? We thought that the addition of Remicade was going to help, but it’s not provided the kind of impressive response that it had in the past. Discouraging. Josie meets with Dr. Vehe on Wednesday for another important visit. It’ll be three weeks since her last visit with him and I’m fairly certain he’ll be unhappy with her progress slide.

Perhaps another explanation for her apparent relapse is that she missed her IVIG and heavy-duty steroid bolus last week while at camp. We all agreed this was to be a good time to try skipping a dose of each...to see how she responded. I’d be surprised if orders aren’t given to resume weekly doses of both. We need to get Josie more stable to start weaning her off the IVIG. Sure it’s wonderful stuff, but good gravy, it’s expensive...anywhere from $4,000 to $9,000 per dose. Most folks would be perfectly fine to earn that much rather than injecting it into one’s arm...but that’s where Josie is at this time.

Scare of the Week

While enjoying camp, Josie was taking part in almost all the daily activities. On Thursday, she was taking a ride on a horse as part of a structured activity. Can you guess what happened? Of course it had to happen to Josie... all of a sudden the horse bucked and Josie was thrown off its back. The staff person walking along with Josie lost her grip when the horse bucked and Josie fell right off and landed flat on her back. This was a little alarming, given that a one-foot fall onto a carpeted floor just two months ago resulted in a broken femur. Luckily there was a family practitioner, two emergency room doctors, and a general internist staying at the camp too and they were there on the spot.

Josie was shaken up a little bit and immediately complained of pain on her back and chest near her sternum. We all agreed it would be a good idea to take her into the Detroit Lakes Emergency Room to get a once-over from the ER doc on staff and to get a CT scan if needed. The fear was that with Josie’s fragile state and given her enlarged liver and spleen, that kind of a fall could have damaged either of those organs or broken a bone. When we arrived at the hospital Josie was feeling better already and was no longer complaining of pain. The ER doc checked her out and agreed that she was doing well and just to watch her close over the next day. If there were and kind of internal bleeding Josie would demonstrate obvious symptoms. We left and that was that. No problem...just a bit of a scare.

So now were all back in town ready to take on the rest of summer. This week it's doctors visits and some more physical therapy, plus a fresh dose of IVIG and steroids tomorrow. Can't wait.

6/29 Steady Progress

There were a few doctor’s visits this week after Josie’s fever spike earlier in the week. All is well and the fever’s stopped. She’s still on the big-time antibiotics which were started as a precaution. Perhaps they were what broke the fever but it had already come down before those big guns were started. We’re just happy she’s feeling better.

One thing which is not a problem is getting her up and about. She’s demanding to get up and walk…almost to the point of contention. -- The other day we all headed off to Target for…you know…the normal Target gear, and Josie refused to be carried from the car to the store, then refused to get in the shopping-cart seat. She insisted – and I mean INSISTED – on walking.

Ya ever get the feeling that people are watching – no, staring is more appropriate – at you when you or someone near you is shouting at the top of their lungs. I can hear the chatter in their minds as they glance sideways at this family headed down the isle with one of ‘em screaming bloody murder until she gets what she wants. See, that’s a problem we’re running into with Josie. What Josie wants, Josie usually get because she has the unyielding determination of no one I’ve ever met in my life…exponentially more determined. She wears us down until there’s nothing we can do but allow her what she wants. Sounds fairly easy to stay firm, stiffen that back, make her understand that her unacceptable behavior is totally unacceptable! Sounds easy until you’re 90 minutes into a tirade because the spoon you’ve provided is not small enough, or the color of the cup is not the green one or any of the other 10 bejillion ridiculous reasons which cause Josie to lose her mind…but I digress.

Josie’s strength is getting so much better. This week at one of Trip’s soccer games Josie must have walked here and there and everywhere. I would guess that she walked at least a mile that evening…no kidding. She’s getting up off the ground and floor on her own many times a day with no problems…a little slow and slightly shaky, but gaining confidence all the time. She’s even found a good way to get up and down a couple stairs at a time – certainly not walking up them, but getting up and down them without help.

We’ve turned up her feeding rate so she can get all her daily food in much less time than before. This means that she can be unhooked and free more often than before. She likes that idea.

Her skin is still not the greatest. It’s not into a full-blown flare up, but it’s not what we want to see either. There are the same trouble spots under her arms and shoulders. Her fingernail fold capillaries are showing signs of inflammation still too. The Remicade she has on board is working, but not to the degree we remember from late last summer. Oh well. We’ll keep pressing and things should work themselves out, right?

Tomorrow we’re off for a week of fun at the family resort we’ve been visiting for the last couple years…Fairhills Resort on beautiful Pelican Lake near Detroit Lakes in northwest Minnesota. Everyone’s excited for a week off. We just have to be careful and make certain Josie gets that sunscreen on and finds the shade.

6/25 Trouble Might be Brewing

After a few days of the Remicade at work Josie’s skin seems better, but still it has not reduced the redness much in her face, although generally speaking she looks well. Things were going pretty darn good until yesterday when she spiked a little fever at 102°. No big woop…a little Tylenol and she’s good to go. After the doses wore off the fever kept coming back, and with it came the vomits too. This morning she really shot up to 103.9° which is just a bit out of our comfort range so we put in a call to the our favorite GI Doc down at the University. He gave us a ring back and said she should have a battery or tests done and she should also get started on the heavy-duty antibiotics of Vancomycin and Ceftriaxone for at least the next week…can’t be too careful when a fever hits while on Remicade. Normally they’d issue orders for someone like Josie to head straight to the hospital, but Josie’s history is such that they’re comfy with Josie staying put and the tests and drugs taken and administered here at home…and we like that just fine.

The home health folks stopped by with our arsenal of medicine grenades and she took a few vials of blood for all the tests, which we’ll get the numbers on tomorrow. Wonder what’s happening in there.

So the excitement around here continues.

6/23 More Drugs On Board

Josie saw Dr. Vehe on Thursday for her weekly visit and he was generally happy with her appearance. He continues to be impressed with her strength and mobility, which keeps on coming. He thought her skin generally looked better but the hot spots were a bit of a concern. Her finger nail-fold capillaries are flaring up just a little bit and the marks on her shoulder and elbows are a little redder, as is the redness in her face. His general feeling was that “…we’re not doing things quite right…but on the other hand we’re not doing things quite wrong either…” He decided to give Remicade another whirl.

Remicade is a heavy-duty drug that blocks the action of a substance in the body called tumor necrosis factor (TNF). Blocking TNF can reduce the inflammation and immune responses caused by too much TNF in the body. So it’s a big-league immunosuppressant, and that means she’ll be at some serious risk for infection due to her beaten-down immune system. Remicade is also the golden-treatment drug for Crohn’s Disease, which is a chronic and serious inflammatory disease of the gastrointestinal (GI) tract. So being treated with Remicade will not only help Josie’s skin (that’s the plan) but it’ll also give bonus help any involvement of her GI tract too, which of course she has a history of.

So yesterday Josie went in for her Remicade infusion in the Cancer Center at the University Hospital. She was there for a couple hours during the infusion and then that was the end of it. This morning her skin looked better. The skin involvement on the hot spots seemed better…that’s of course before she headed out in the heat of the day. It’s just mid-day and just being outside in the shade has brought up some more splotches. It’s pretty clear this girl is some kind of Vampire when it comes to the sun.

Josie’s mobility seems unstoppable as of late. She wants to walk and stand and get around more than ever, and we have to keep a close eye on her so she doesn’t get wiped out by a dog or other kid scrambling through the house. Her heels are becoming loser and her confidence is getting stronger (which might become a problem). She’s still trending in the right direction though.

6/20 Things Are Going Great

Josie’s confidence is growing. She’s really ramping up on her walking and mobility. The improvements are coming faster than ever now…and it’s all good from the walking standpoint. Her flexibility in her feet is improving too. Another visit with Physical Therapy tomorrow should go very well. They may have some splints to continue aiding her feet as they get more and more flexibility.

All this wanting to get around is causing another minor complication…she doesn’t want to be hooked up. With her feeding tube, Josie is hooked to her food bag and pump for at least 22 or 23 hours each day…but hauling that thing around is a serious style cramper so she wants it off. It’s been off for way longer than a couple hours each day for the past few…and that means Josie’s not been getting enough calories. We’re logically thinking that this might be a good thing…she’s walking more and burning more calories and taking in fewer calories…the perfect dieter…so one might think her appetite might improve, right? Not really with this one. She’s never craving any food. She says she’s hungry but only wants a bite or two. She has no extra craving for sweets or ice cream or anything the other rascals are always itchin’ to get. Her desire for food can’t be like anyone else around. Being deprived of food for the better part of the last year…the most recent 20% of her life…must have altered her normal food desire from a mental perspective, one which I can only speculate upon. But we’ll keep throwing food her way and eventually she’ll start taking it in more and more…or so that’s the thinking.

We see Dr. Vehe tomorrow to check out that skin which is still not really improving much. Wonder what he’ll have to say. I know he’ll be impressed with Josie’s physical progress though.

6/18 She’s Got The All Clear

Josie had a nice visit with her orthopedic Doc this afternoon and she got the green light –all systems go for working out on her broken leg. It’s still a little sore and she’s still limping on it, but that’s more a function of not using it for a few weeks and still dealing with not walking for so many months. Physical therapy can now move forward as fast as Josie will allow rather than holding her back due to that leg. Her heel cords are still really tight with little flexibility, but it’s slowly improving.

She’s been continuing to get stronger. Over the weekend she was moving around a lot, walking and riding her little tricycle around the house. She’s even working on getting herself up and down a stair or two…this wasn’t even a thought a couple months ago. She’s getting back to the walking comfort she had just before she broke her leg. She’s clearly more cautious though…watching where she steps, and super careful when dogs cruise by.

Her skin is still a bit of a problem. We’re seeing a few things here and there that aren’t looking the way we’d like. Her little hot spots from before are not really improving…but they’re not dramatically worse either. They’re just there and not going away. We’ll see what they look like after tomorrow’s IVIG and steroid bolus. Josie visits with Dr. Vehe on Wednesday for another look see. It would be fantastic if we could get to an every-other-week or even a once-a-month visit schedule, but that’ll have to wait as she’s not nearly well enough or the disease inactive enough for that…which kind of stinks.

Josie’s been coming and going with a mild fever over the last couple days…and that’s resulted in a few vomiting spells. It’d been quite a while since she had the barfs and, as usual, there’s no real apparent reason for it now, so we’ll just work through it and all will likely be fine in a day or two or three.

She’s been real punchy with her siblings lately…pretty much running the roost. She takes about zero grief from anybody (including Mom and Dad too much of the time). She’s quick to argue, and a lot of times she argues just to argue, which drives me a little nutty. And she knows how and when to lay on the drama…to the nth degree. Josie was in prime form late in the day on Saturday at Trip’s soccer tournament…the third game of the day. She went off on Deb, and Deb had had it up to her eyeballs with the sibling bickering and the demanding four-year old. Deb reached her breaking point and so they were outa there. It’s all good though.

We’ll keep you posted on progress this week with the results of her doctors’ appointments.

6/14 Going Well, and Another Milestone

Josie is progressing well and not so well at the same time. Tuesday she had a visit with an Endocrinologist Doc to assess her normal growth progress. It seems like a good idea to talk to the experts in the know about how well she’s progressing considering all she’s been through over these last two years.

Josie’s growth was on a normal track until about the beginning of last summer when she pretty much stopped growing. That’s when all this GI stuff started happening and the spiral of Doom spun almost completely out of control. It been only in the last few months that she’s shown signs of growth again now that her nutrition is better and she’s not as ravaged as before.

The Doc met with us and gave Josie a poke or two here and there and gave her a once over top to bottom. He was going to go over her nutrition profile and what she might need in order to get back on normal track on the growth scene. We should be getting his recommendations sometime soon.

During this visit we happened to bump into Dr. Saltzman, Josie’s surgeon who saved her life late last year. He was happy to see Josie and thought she looked great. He gave her tummy a quick look and we gave him the lowdown on her pancreatic tube being removed last week and that we were going to come in later this week for the other tube to come out. He said “why don’t we do it right now?”. Perfect! So he put on some gloves grabbed hold of it and pretty much yanked it right out. In about one second it was over. A single band-aid later, and that was that, end of tubes. Josie, of course, needed to show Mom so I had to pick this tube out of the garbage and carry it away in a zip-lock baggie for show and tell later.

Josie visited with Dr. Vehe yesterday and he was definitely frowning his brow at her skin issues. There’s a bit more involvement this week and that’s not what we want to see. Things have been progressing in the right direction for so long, it’s a bummer to be headed backwards again.

He decided to add Plaquenil to her drug list. This is the drug she’s been on before and when effective it can decrease damage to the tissues of the joints, skin and other organs in the body. This pill tastes terrible but because of Josie’s feeding tube we only need to crush the pill up, mix with a little water and then shoot it into her tummy bypassing her mouth altogether. She's in favor of that plan vs. eating it.

Today Josie had some fun with Jocelyn at Physical Therapy. They played and walked and played and worked and played and stretched and played and played. Josie loves visiting Jocelyn. Josie’s strength is improving really well. She’s able to get herself around nicely with her upper body strength but the walking is still a problem. She still real ginger on her broken leg but that’s coming.

The best news of the day though is that Josie got up off the floor from a sit to a stand without any help whatsoever! Not holding onto a chair or table either…all on her own. I don’t think she’s been able to do that for almost a year or more! She was so impressed with herself that she did it again and again and again…about eight times…it was great to see.

6/10 Trouble Might be Brewing

What a terrific weekend we had…the first real weekend of summer. Today we even hit to pool for the first time, along with everyone else in town, on this fine 89° sunny day. Josie’s been saying Hi to lots of great folks who she hasn’t seen in a very long time. It’s so nice for her to be out and about like a normal kid again. She had a great time at the pool even though she had to keep her PICC arm dry and had to play in the shady part of the pool.

But all is not totally well with Josie. As the heat rises so too does the redness of her rash on her face and arms. Perhaps it’s the sun, or it might be the actual sunscreen we use on her, but whatever it is it’s clear her skin is less than well and that bugger of a disease is with her and firmly active.

Also today Josie’s been battling a fever and the pukes. It started last night. Overnight she was at 102.9° and vomiting. Not a good sign. A little Tylenol and things settled down pretty well…and that’s been the call all day long. After a few hours the fever is back at between 102° and 103°. We’ve been alternating about every five or six hours between Tylenol and Motrin and it seems to be working OK at treating the symptoms. But the symptoms (vomiting and fever) might very well be pointing to something bad or even very bad. Perhaps it’s coincidence (hopeful) but this is a suspicious time to be getting some fevers just a few days after that pancreatic tube was yanked from her tummy. Overnight and tomorrow may reveal more and we’ll give the docs another call tomorrow with Josie’s info.

Aside from the bad skin and big fevers, Josie’s broken leg is healing admirably. She’s been up on her feet much of the days (about 1/3 to ½ the time) and her strength is improving nicely. Her flexibility is still a challenge but we’re working on that too.

6/7 Doctor Visit – One Less Tube

A milestone day for Josie. Tonight she’s one tube lighter as her pancreatic drain finally came out. Up until yesterday she had three tubes still entering her body through her belly: her feeding tube going into her stomach, her T-tube going into her duodenum, and her pancreatic tube entering her duodenum through her tummy and ending inside the main duct of her pancreas (supposedly that is, but there’s not a snowball’s chance in H – E – double hockey sticks that it was still in there).

Back in February, just after the big non-Whipple surgery she had those three tubes plus two other JP drainage tube which were removed within a month. The T-tube and the pancreatic drain both needed to stay intact several months to ensure there was a strong enough fistula (scarred tunnel within which the tubes lie) between the duodenum and the outside so there would be the least chance of infection when it would be pulled out. Her feeding tube must stay in there until she doesn’t need anymore supplemental feedings, which will be about the time she’s 39 or 40 at this rate.

As Josie’s pancreatic tube was pulled, she was a little anxious about it thinking it was going to hurt awot…but the hardest part of the whole thing was taking off the band-aid. The Doc just slowly pulled the drain and it slipped out – all 24cm of it. Josie watched it and said in her funny way…”that tickles”. The bad news is that there is a huge dimple in her tummy where that drain was. It’s likely scar tissue attached to her muscle which will probably not ever change without a cosmetic procedure…but that would be years and years down the road and isn’t even a thought right now. Let’s just say the modeling agencies are not lined up in anticipation of a strong bikini modeling career for Josie with all her scars…although by then the scarred look might be the in thing.

Next will be the T-tube duodenal drain which will come out in a week or some. They didn’t want to do both today just because it’s better to stagger these things.

Dr. Vehe checked out Josie’s skin issues and was pleased with how delightfully pale she looked. He was encouraged by her spunk and activity during our visit (which was borderline obnoxious)…she was all over the place. He’s seeing clear signs of strengthening throughout her whole person…even with the broken leg and all – way better. He was a teensy bit concerned about the splotchy marks on her body but was at the same time somewhat encouraged because he thought the spots hadn’t changed too much in the last week. That said, he’s getting a little impatient that her treatments are still not calming her underlying disease better. It’s still active and smoldering. Dr. Vehe made a change in her IVIG dose and if there are signs of further involvement next week then we’re going to try a different tact again as this one isn’t quite doing the job totally.

Stay tuned.

6/5 Drug Day

Today was Josie’s IVIG-and-Big-Steroid-Bolus Tuesday. Our awesome home health nurse was here much of the administering Josie’s dope and it all went smoothly. Earlier in the day though Josie was a real peach. She was in one of those moods where she was just looking for the slightest thing to give herself the OK for a major tantrum. She was complaining about the most ridiculous things…sorry, I can’t remember any of them…I just remember how petty and absurd they were. This was a couple hours before all the drugs. I thought it’s a bad sign being soooooooo crabby before the big steroid thing, but the drugs came and there wasn’t a hint of ‘Roid Rage all day long. She was a model child all day. A little npaski in the afternoon set the girl up for another good stretch this evening. In fact she was in such a great mood she was actually playing and laughing with ALL her siblings tonight…music to a parent’s ears having all the kidlets laughing together without a computer or Xbox in sight. Nice.

Tomorrow Josie meets with Dr. Vehe again and we’ll get his take on her newer splotches. Now she’s got some new marks on the back of the lower part of her upper arm (tricep area) which look a little strange, kind of like irritation from rubbing on something. Maybe that’s exactly what it is…tomorrow we’ll take a closer look.

The other three kids are in their last week of school and everyone’s excited for summer break, save maybe Deb and me. I kinda like school. It reduces daytime stress quite well.

6/3 Continued Progress

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5/31 Another Fine Day at PT

Josie and her PT Professional Jocelyn had a fantastic time playing and getting stronger today. All were impressed with the fun and spunk Josie demonstrated during her entire session…nothin’ but fun. It looks as though Josie will be getting a couple night-time splints for those pesky heel cords of hers to help stretching them. They’re improving, but not well enough. As with most things Josie (aside from broken bones which mend remarkably fast), those heel cords are taking their own sweet time improving. The good leg foot is OK, but no great. Her broken leg foot is way bad…worse than before the break… which really wasn’t unexpected.

Next steps are to get more weight on those legs for Josie and build more strength and flexibility…those are the keys to better mobility. The great news is that Josie’s very motivated about getting more mobile and better…maybe it’s the weather, but whatever it is she’s having a hard time sitting still and on her keester. She’s on her feet several hours each day.

Nutrition is still an issue. Her appetite for real food by mouth is just a smidge better that it’s been, but still almost non-existent. You’ve all had more food in the last 24 hours than Josie’s had in the last nine months. I just cannot imagine that frame of mind…having virtually no food for the last 1/5 of your life and then getting back into eating…it’s a different paradigm which no one I know can relate.

She’s still vomiting about once a day, but that’s usually because we give her too many drugs too quick and it upsets her little tummy. Otherwise her tummy pain has improved over the last month.

Now that it’s June we can expect those other two drainage tubes to finally be pulled from her tummy which have been there since the February non-Whipple surgery. In fact, today I almost did the doc’s work for them by nearly yanking one of the tubes out as the end was stuck in Josie’s pants when I was changing her up…YANK! It was a really close call and it kind of hurt Josie just a little. With summer here and everything way more active, all those tubes are being transported all over the place and it’s really a challenge trying the keep from accidentally catching the loop on a door handle, or stroller wheel, or bathroom cupboard handle, or stepping on it, or a dog catching it, or a person walking by catching a loop, or any of the other couple dozen close calls we’ve had with those tubes the last few days.