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Monday February 1

Today, I received a wonderful email from a nurse that works with JDMS out of Chicago. Here's what they said (printed here with their permission):

Overall she [Julia] sounds very active, but the rash is a lingering problem our research is showing. The F8 test you mentioned is the Factor 8 antigen or VonWillebrand Factor lab test. This measure the level of inflammation in the blood vessels. The higher the F8 the more sure we are that the disease is still active and it appears to correlate well with the appearance of the vasculitic changes in the skin (ie. the rash). When the vessels are really inflamed, like a the start of the JDM, the F8 is elevated very high. If the damaged blood vessels are not feeding the muscle tissue well then you get muscle damage, which leads to the release of the muscle enzymes into the blood (ie high LDH, CPK, SGOT, & Aldolase). After the disease has been improving on therapy these enzymes normalize because the muscle damage is repairing, as the blood vessels normalize. So the muscle weakness improves and the rash disappears much after the weakness is gone. We are still learning about JDM, but feel we are well on our way to better understand the Vasculopathy with some new research data analysis and plans for the future.

Wow, that was a lot for me to digest! I guess the message here is that the rash Julia is suffering with is to be expected, although not everyone gets it. There's just so much to be learned yet about this disease. Also:

Just an additional note, where I mention about the rash resolving slowly compared to muscle weakness, you can also add that the nailfold cappillary changes appear to be a good (research still in progress) tool to also evaluate the changes in the blood vessels because they appear to also still reveal blood vessel changes that are no longer evident in the muscle tissue (ie. muscle weakness appears normal).

Thanks very much for the input!

Tuesday February 2

Happy Groundhog's Day! This is truly one of the weirder "holidays" on the calendar. Still, is does remind me of that funny movie of the same name starring Bill Murray.

Today is "Beach Day" at Julia's school. Everyone gets to dress up in beach garb and accessorues. Julia has a seashell necklace, sunglasses, and a straw mini-totebag; she's wearing an authentic Hawaiian tee-shirt, acquired by her parents' during their honeymoon. I'd love to see the class all dressed up!

Saturday February 6

We went on a little road trip to Connecticut today to visit my parents. Unfortunately, we forgot to pack Julia's weekly Methotrexate dose. Sigh. We get her meds at a drug store chain, CVS, and they have a store in the town we were visiting. I figured we could just call them up, have them look up the prescription, and give us a refill. Nope - they needed the prescription number and the store number where is originates. Well, if I had that, I wouldn't need the refill on the road, would I? I had to find the number to our home CVS, call them and get the store number and scrip number, then call back the local store and give them the info. They were very helpful.

We took a ride to the store to pick up the refill, and D'oh! They didn't have enough on hand to fill the prescription. We were from out of town, so giving us a partial refill and coming back in a few days for the rest wasn't an option. The pharmacist was kind enough to give us one dose, nine pills, no charge. This was a tremendous convenience for us, and we really appreciated how helpful they were.

As it happens, Julia's Prednisone dose goes down to 3mg today. This is the biggest percentage drop we've ever had, a full 25%. Her dose stays at this level until we go see the doctor in a couple of weeks. I imagine we're going to have to go down by half a milligram, or start going to alternating days with different doses (3mg today, 2mg tomorrow, etc.). Probably as a result of the drop in Prednisone, plus with Basketball practice last night, her rash is very bad today, especially the wrists and hands.

Current Drug Dose: Prednisone: 3mg Plaquenil: 200mg/day Folic Acid: 1mg/day Methotrexate: 22.5mg/week

Sunday February 7

Julia's rash looked a little better today, especially the face. Maybe the MTX dose kicking in. She received Sacrament of the Sick today, and she always seems in a good mood after we do that.

Julia also seems to be fighting off a bit of a cold; she really complained about her throat hurting when she woke up this morning, but she fortunately didn't have any trouble swallowing her pills.

Monday February 8

Julia was OK this morning, but her mom wasn't, and stayed home from work. However, right after lunch, Julia developed a stomach ache and a headache and came home from school early. You know she wasn't feeling well because she took some Tylenol without complaining; she usually raises a ruckus to take anything. By the time I saw her at the end of the day, her headache was pretty much gone. She did cough a little a few times after she went to bed, but she seemed to sleep all right.

Friday February 12

Julia was looking forward to the last basketball practice of her league season tonight. I drove her over there, only to find out that there was no practice!! She was heartbroken, and cried for about 15 minutes. She was apprehensive about the basketball at first, but after playing for a few weeks, she really seems to like the competitiveness of it. At least we still have the game tomorrow...

Saturday February 13

Yes, Julia has her last basketball game today. I cannot believe the improvement in her game since the beginning of the league. She plays scrappy defense, getting a couple of steals and basically denying whoever she was covering the ball, She even scored a basket! I felt so proud of her. I'm even thinking of getting a hoop installed in our driveway this spring and painting properly-dimensioned lines on the asphalt.

Her rash looks so bad afte rshe plays, though. Her face gets so red. It looks like she's exhausted, but she stays out there for more. She played in 5 of the 8 4-minute shifts today! That red-face stays with her all day. I can only wonder what the other parents watching must be thinking.

Sunday February 14

Happy World Marriage Day! Oh yeah, it's St. Valentine's Day, too. Julia made me a card a couple of weeks ago that I've had up in my office, but she made me a couple of other cards for today. My wife's been on a Rubber Stamping kick lately, and Julia is along with her for the ride. The cards are really nice! I gave Julia a Teddy Bear; I think she'll be my valentine.

We took Julia, along with he grandfather and aunt, out to lunch to celebrate Julia's birthday coming up this week. We went to one of her favorite places (Bugaboo Creek, which actually doesn't seem to have a web site!), and we arranged for a birthday surprise for dessert. The whole waitstaff came over and serenaded Julia and brought her a dessert, and they made her kiss a toy moose head. She was *very* surprised, and she loved it.

Tuesday, February 16

Julia had a doctor's appointment today, but her rash looks about average. It's usually remarkably bad on doctor days. Dr S., was traveling today, so Dr. A.W. saw Julia alone, or rather, with a different resident.

Vital stats: Weight - 34.1kg./75.1lb., lost almost another 2 pounds, woo-hoo! Height - 50.5"/126.8cm, up another half an inch. These are excellent trend. At this rate she'll be weightless and infinitely tall in no time. Blood Pressure - 122/60, Temperature - 97.8°F/36.5°C, both normal.

Dr. A.W. performed the clinical examination and thought that Julia's strength was excellent. She really seemed impressed by Juila's strength. She hadn't seen Julia in 3 months. She had some new places where the rash has appeared - her wrists and inner thighs, most notably. She also asked her usual questions about her activities and medications and made small talk with Julia about her activities. She asked about Julia being tired, nauseous, headaches, stomach aches, etc.

Last time, Dr. S. ordered what he called an "F8 antigen" test. I asked about the results, and Dr. A.W. said that it looked like the test was never actually run. Apparently, sometimes the lab misses things that are on the sheet - I know the test was on the sheet, we remember seeing it there. We'd already had Julia's blood drawn before the exam, so she said she'd see if they could add the test after the fact.

The different resident, Dr. F., came by and gave Julia the once-over. He'd obviously been well-prepped by Dr. A.W.. He checked Julia over, asked her and us a couple of questions, then turned to us about Julia's mediciation.

We talked about continuing to reduce the prednisone dose. We're currently at 3mg/day, which is below the level the body normally makes. Last time, Dr. S. was concerned about Julia's adrenals working properly again after being on a high dose for so long. If they weren't, we'd be seeing some of the symptoms that Dr. A.W. asked us about at first - being tired, nauseous, headaches, stomach aches, etc. Since we're not seeing any of these problems, it's safe to assume that her adrenals are beginning to work properly again, and we can continue the taper of the Prednisone - all the way down to zero!!

Assuming that the blood work done today shown no problems, we'll continue to reduce by 1mg every 2 weeks until we get to zero, shich will be in little more than a month. We still need to monitor Julia for high fevers (101°F or higher) and infections and give her a 2mg booster dose until she's been off of Prednisone for at least a month. He said that we should schedule our next appointment for 2-3 weeks after we discontinue the Prednisone.

We did ask about the Plaquenil - he's been on it for nearly a year, and our best judgement is that the rash is just about as bad as it's ever been. Dr. F. said that the pulse steroid therapy would almost certainly knock down the rash, but recommended our current course of action and be patient. He said that the muscle symptoms and the rash are seperate, independant processes in the body, with the muscles the one that needs to be most aggressively treated and monitored. The rash, we need to be patient about.

3/3/98 Test: 11.8 659 301 60 36
3/24/98 Test: 5.0 39 227 15 12
4/21/98 Test: 7.0 27 310 20 9
5/27/98 Test: 2.0 60 398 31 20
6/30/98 Test: 3.9 68 279 23 18
8/25/98 Test: 5.2 251 332 41 36
9/10/98 Test: 7.0 48 206 22 25
9/29/98 Test: 3.2 77 335 27 25
11/3/98 Test: 2.3 99 295 30 35
Normal Range: 0.0-7.0 4-150 110-295 10-34 6-59
1/5/99 Test: 6.2 143 250 30 20
2/16/99 Test: 8.9 226 276 45 54
Normal Range: 3.0-16.0 4-150 110-295 10-34 6-59

Wednesday, February 17

Today is Julia's 8th birthday! Everyone wish her a happy birthday.

Friday, February 19

We got a call from Dr. A.W. today. Since the information from her didn't come in an email, we immediately knew the news was, at least, not all good. As you can see from the chart above, many of her numbers are up, some to above the normal range (namely, CK and AST). Also, her Sedimentation Rate is 9, Platelets 390, Hematocrit 37.2 and White Blood Count 6.3. These additional numbers are improved from last time.

The good news is that we will not change her Prednisone tapering schedule because of this. She goes down to 2mg/day tomorrow, and contines to taper to zero. This, at least, is reassuring. The plan is to derive most of the anti-inflammatory action from the Methotrexate and not Prednisone any longer, since the side effects are so severe. If we need to, we'll raise her MTX dose instead of putting her on Prednisone again. She said that we may use Prednisone sporatically to control infections and the like. They do want to have her next blood tests done a little sooner than originally planned, so we moved up her next appointment.

The doctor said that these numbers are up partly because Julia has been so active lately. Basketball has been the most physically active activity she's ever done, and the increased activity alone can cause a rise in CK numbers all by itself, even in healthy people. The clinching factor is her clinical results. Dr. A.W. said that Julia's "doing incredibly well" and is "the strongest she's ever been" since this started. Dr F., who Julia saw on Tuesday, was consulted and said that he's seen a lot of kids with JDMS, and many of them are very sick, and Julia is exhibiting none of the general problems (fatigue, weakness) that they often see.

Today and into tomorrow is Julia's birthday party [thanks to everyone who sent her a greeting!]. It's more than that, though - she and 9 (one cancelled at the last minute, or it would have been 10) of her closest friends are having a sleepover. That should prove to be quite entertaining.

Saturday February 20

Well, the sleepover was a big success. We has games, prizes, contests, a piņata, movies, and crafts. Hey, when a party lasts over 18 hours, you have to do something. The girls all stayed up rather late, and woke up rather early, so I expect some widespread napping to take place later on.

At bedtime, Julia was overheard explaining to one of her friends about JDMS. Julia has a very complete and accurate grip on what's going on, what the meds do, and what the plan is. I was pleased to hear it. We've always kept her "in the loop"; we just don't believe it's right to shield her from things.

Julia's Prednisone dose goes down to 2mg today. She usually complains about having to take her meds, but today she had an audience, so everything went down quite smoothly.

Current Drug Dose: Prednisone: 2mg Plaquenil: 200mg/day Folic Acid: 1mg/day Methotrexate: 22.5mg/week

Thursday, February 25

It was a snowy day today, school was even cancelled, and a rather embarrasing thing happend to me and Julia. We locked ourselves out of the house! I had my keys in my other coat and didn't transfer them over and Julia closed the door about 1 second too early. Of all days, it was snowing hard and Julia's mom had to come home from work to rescue us. Julia & I shovelled while we waited. Actually, I shovelled and Julia played in the snow, which is how it should be. We ended up getting about 10" of snow, all of which melted within a few days.

Julia's face rash has been looking pretty good for the past few days, but her hands and wrists are looking pretty fierce.

Friday, February 26

Julia and her mom had the weekend to ourselves because I was away on retreat with a group of men from our church, which is something I do every year. The girls ran errands, went shopping, and rented some videos.

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Updated March 5, 1999
Copyright © 1999 by Ralph Becker <ralphb@whoever.com> send me Feedback!
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