Friday, September 12, 2008

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'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.


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