Monday, March 20, 2006

3/20 Hovering and Tired of Being Sick

Things have been ... how should I say it ... frustratingly monotone with Josie's progress lately.

We saw Dr. Vehe on the 15th and generally speaking she was doing a little better. The rash on her chest and trunk has decreased over the last three weeks, which is good. Not sure though what has caused the improvement as she's been on so much stuff...Rituxan over the last month, or the IVIG infusions, or the increase in the steroid dosage over the last couple weeks.

Unfortunately we really don't have the luxury of giving something the time needed to make a determined and confidant conclusion...kinda vague speculation as to what might be working better than the other.

Of course the rest of the kids have come down with the flu (yep diagnosed in the Doc's office as this year's strain of influenza...nice). I'm shocked Josie didn't really get knocked down by it, but I'm pretty sure she's picked up something. She's been hacking and sniffing with flushed cheeks and an occasional low-grade fever. Getting better from that though. So it's more drugs to combat sickness, on top of the disease. Dr. Vehe seems to think the abnormal blood work lately is from Josie fighting off all the nasty bugs floating around our home with our troop of sickies.

scarThis is an image of the scarred area under her arm where the skin broke down so badly in the summer and again in the fall (check the October 13th pictures) . The scar has really grown into a thick pink hood...looks like a flattened piece of silly putty. Not too concerned about this now...but we'll probably have to do something about it in a few years (maybe).

Bottom line is that Josie has a fairly nasty case of Dermatomyositis which is giving everyone fits trying to figure it out and make her better.

We're off the the Mayo Clinic for a consult with another Rheumatologist, Dr. Ann Reed with extensive Dermatomyositis experience. Frankly I don't think it'll provide us with anything new, but they always say, it's always good to get a different/second opinion. It'll certainly be interesting speaking with a different doctor on the subject, and we're firmly confidant in Dr. Vehe's care.

Drugs, Drugs, and More Drugs

Josie is a walking Pharmacy and has been for a long time now. This picture is a snapshot of one round of medicine...and this is only the medication we give her at home twice each day...not the IVIG or Rituxan. The girl is actually taking and swallowing pills with no problems. And the yucky medicine which stings the mouth for an hour after tasting (I've done it) is no biggie for her to take any more. She simply takes it all like a trooper. We give her the pill and then wash it down with the nasty stuff, then the steroid which tastes better, then the other junk which all tastes better than the nasty. A small drink of after and it's like nothing happened. We really would like to get this over with and under control as this whole drug regimen has stunted her growth. She's not gained a pound or grown at all since it started 8+ months ago!

Wednesday, March 08, 2006

3/8 Things Are Heating Up Again

The roller coaster ride continues. Things were looking a little worse the last time we were in. Josie was prescribed a steroid dose increase of 33% because some of the blood levels were rising again. This week Jo came down with a cough and flushed look. She was also complaining of knee pain(?). We we took her into the pediatrician and they did some blood work there as well as a general exam. They said there was obvious swelling of the knee, though when we look at it, it seems not too different than the other. The pediatrician contacted Dr. Vehe's office and let them know what they found on exam, in addition to a significant rise in her sed rate...95 (normal 0-20). Josie's sed rate is usually elevated to some degree... the last two measurements were 28 and 36.

Sed Rate
(Also called ESR, or erythrocyte sedimentation rate)

The sed rate, or erythrocyte (e RITH roe site) sedimentation rate, is another method for measuring swelling and inflammation (IN fluh MAY shun) of the muscles. Doctors use the sed rate to watch the progress of the muscle inflammation. This test is not specific to a particular disease but to the presence of the inflammation.

For this test, the technicians mix red blood cells from the blood sample in a prepared test tube. They measure the length of time it takes these cells to settle to the bottom of the tube. For males, the normal rate falls between 0 and 15 millimeters per hour; females, 0 to 20 millimeters per hour. The sed rate will be higher with more inflammation.

The elevated sed rate possibly shows increased inflammation and could indicate a flare-up of the disease. It could also mean infection of some sort somewhere in her body. Of course we were told to bring her in today for another look-see. At Dr. Vehe's, the swelling in her knee was not obvious and Josie was bouncing off the walls in a super cheerful mood (not normal at the Doctor's office). This was welcome behavior considering the sort of alarming sed rate!!

We are now awaiting further blood work taken at that visit in hopes that yesterday
s reading was lab error...but we'll see.

Still set to start the Cytoxan in a week or two.

Hair, Hair Everywhere

Everyone talks about how drugs affect your appearance. Well, these drugs are causing hyper hair growth all over Josie's body. Yes, that's the back of a little 40 pound three-year-old (see the image of her back from late August). Aside from Jo-Jo's back, her forehead is covered, along with her cheeks, arms, legs...basically she's become the Wolf Girl. Just glad this is all just temporary and someday soon we can look back at this in amazement.

Wednesday, March 01, 2006

3/1 Not Much Progress, in Fact a Little Worse

Another month has vanished, and again I apologize for not being prompt with these updates as many have asked what the story is.

Bottom line, it seems things have slipped over the last month.

We've reduced the oral steroids again, and she's had four infusions of Rituxin chemo as well as a couple infusions of IVIG, plus all the other drugs. She was just in today and one of her muscle enzymes is on the rise again--possibly indicating more activity in the muscles, though we haven't had the official word from the doctor.

Her strength seems to be dropping again too. We were pretty fired up over the last couple months at the strength progress, but over the last few weeks we've noticed the strength dwindling a bit. Until recently, she was willing to head up a few stairs, tried to run, got up with no problem...generally better. But now the stairs are getting harder...on and on and on it goes.

She's been enjoying going out on our skating rink, though she really likes it when I push her around on the chair rather than really skating herself.

So, we'll wait to see now what Dr. Vehe thinks about the blood levels and the response (or lack of) to the Rituxin...the effects can sometimes take weeks to surface. I sure don't want to increase the drug doses again.

We've talked about the next step up of a heavier duty chemo, Cytoxan. Not sure if we're headed down that road, but if we do it'll be a nastier one. Her hair will fall out and she'll feel crummier with Cytoxan, but we'll cross that bridge later.

I promise that we'll get better at posting more frequently as many of you want to know what's up.

Thanks for reading and thinking about Josie, and like I've said from the start...things could be way worse, but of course we want them to be way better.