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Friday, January 1, 1999
Happy New Year! We had several friends and relatives over today. It was pretty easy, since everyone brought food. A good time was had by all. Julia's rash continues to look good. This may be the longest string of "good days" we've had since this all started.
Saturday, January 2
Julia played in her first basketball league game today. I didn't attend, because I was volunteering at Julia's school to set up computers and local networking. Scouting reports indicate that Julia played tenacious defense, and showed some passing skills. I hope to be able to see her at her next game. The games aren't serious - there's a lot of coach intervention to make sure the girls are doing the right thing, and they don't even keep score. It's a learning and fun league, and Julia did good keeping up with the other girls, even the bigger, more experienced ones.
Tuesday, January 5
Julia had a doctor's appointment today, so naturally her rash looks bad, despite the fact that it's looked good for over a week before today. Dr S., who saw Julia alone today because Dr. A.W. was stuck in St. Louis returning from Christmas vacation due to bad weather in the midwest, laughed and said that it's usually the other way around, that the symptoms disappear for the doctor. We asked about it, and he said that anything that stimulates the immune system will cause the rash to look worse, including exposure to sun, heat, or cold. It's been frigid here for about a week, so I don't know if that's it. I guess we were just due for a "bad" rash day.
Vital stats: Weight - 34.9kg./76.9lb., lost 2 pounds, over the holidays yet! Height - 50"/125.8cm, up half an inch, but it seems like more to look at her clothes, even the stuff she was wearing today. Blood Pressure - 120/62, Temperature - 97.5°F/36.4°C, both normal.
We asked about the discoloration of Julia's teeth. He'd never heard of it or seen it before, so he suggested we ask the dentist. She has an appointment coming up soon, so we'll ask then unless what we saw becomes more persistent. Dr. S. also asked when Julia is scheduled for an eye doctor appointment. I think it's February, but we'll have to check to make sure. He reminded us that she needs to have her eyes checked every 6 months.
Dr. S. performed his usual thorough clinical examination and said that Julia's strength continues to be excellent. He said that her neck muscles are strong, but not quite 100% (those are usually the first to be affected and the last to fully recover), and all of her other muscles were 100%. In addition to the usual battery of blood tests, he ordered what he called an "F8 antigen" test. This is a repeat of a test we took some months ago, back then he called it the von Willebran factor test. It was negative last time. He wants this test to make sure there's no problems that aren't detected by the other tests.
Dr. S. mentioned that last week he saw two new cases of JDMS. He commented on it, I think, because it's so unusual to see multiple new cases of such a rare disease at the same time. The family of one of those new cases has already found Julia's Diary and has contacted us via email (Hi!).
Finally, we talked about continuing to reduce the prednisone dose. We're currently at 6mg/day and assuming that the blood work done today gives us no counterindications, we will continue to reduce by 1mg every 2 weeks until we get to 3mg/day, which is well below the level that the body normally makes. Julia's been on Prednisone for 9 months, and Dr. S. said that the high dose for that amount of time will cause the adrenal glands (the source of natural corticosteroids in her body) to atrophy. The atrophy happens because Prednisone is present in much higher levels than the body normally produces, so the glands shut down from disuse. He said that this condition is not permanent, that the adrenals will slowly begin doing their job again, and over the course of the next 6 to 12 months, should be fully functional again. We can get an adrenal function test - it's a simple blood test, but may require that Julia be tested first thing in the morning. He will consult with an edocrinologist at the hospital to arrange it.
In the meantime, we have to monitor Julia and if she gets an infection or a fever, we will need to temporarily increase her Prednisone dose. This is called a "stress dose", probably because of the stress it causes parents who have to administer it. If she gets an infection or a fever of 101°F or higher, we have to give her 2mg of Prednisone in addition to her current dose for the duration of the infection; once it passes, we can immediately return to her normal dose of Prednisone. This will cause her no harm whatsoever, and the additional dose does not need to be tapered off.
Friday, January 8
Dr. A.W. made it back safe and sound, and she gave me a call today with Julia's lab test results. She called because there was a lot of information that had to go back & forth, so she called instead of emailing me. In general, the news is good. All her important numbers are in the normal range (see above), so we can continue to reduce Julia's prednisone dose tomorrow, to 5mg.
Okay, so now you're wondering why I have two "Normal Range:" indicators in the latest chart. The reason is that they are sending their tests to a different lab now, and this lab has a different concept of "normal", particularly for the Aldolase test. The normal range is now 3.0-16.0, and Julia's result of 6.2 is nicely in the lower half of that range.
A few more raw results: White count, 6.2; Hematocrit, 36; platelets, 501; sedimentation rate, 24. Dr. A.W. had a few comments about the results. The normal range for sed rate is 0-20, and Julia has always been in the normal range before on this test. This is not the case with all JDMS kids; some have serious problems with their sed rate. In Julia's case, the bump in sed rate, as well as the bump in platelet count and the drop in hematocrit, are probably caused by the cold that she had just gotten over. These changes are the normal result of the body fighting an infection; she said that if I took the same blood tests, that I would show similar changes because I, too, just got over having a cold.
She said that she was very happy about Julia's LDH result, it's almost as low as it's ever been, and her Prednisone dose is now as low as it's ever been. This is a very positive sign, as Julia's has had relatively high numbers in LDH all along. We talked about the trend in Julia's CK number, from 48 in September, progressing up to 77, 99, and now 143. She did not seem concerned, as long as the number was in the normal range of 4-150. She said that there's a lot of bouncing around of this number. We will keep a close eye on this result next time.
She said that Dr. S. had talked to a resident endocrinologist about Julia's need for an adrenal test. He thought that unless there was a reason to suspect that there is a problem, and there isn't in Julia, that it isn't necessary to perform this test now. He suggested that we perform this test about 6 months after Julia is off of Prednisone. That means we won't have to come in first thing in the morning next time.
Finally, she reminded me about the stress dose of Prednisone for Julia any time she's very sick, such as fever, vomiting, etc.
Saturday, January 9
Prednisone goes down to 5mg today. Julia's rash is back to looking bad most days. Our reprieve was apparently temporary.
Julia came with me today while I went to donate blood, which I try to do every 8 weeks. It's very routine for me (it should be after 111 pints), and Julia was comparing how my donating blood compares to when she has to have blood drawn for lab tests. The needle they use on me is much bigger, and it stays in for 7 to 10 minutes; they use tiny needles on kids, and it only stays in long enough to fill the vials, about a minute or less. She was also telling all the nurses about her disease.
She had a basketball game today in the afternoon. I was supposed to fill in for the coach today (groan), but his camping trip got postponed because of bad weather. Darn. Anyway, Julia played hard, and she was winded at the end, but she kept up very well.
|Current Drug Dose:
||Folic Acid: 1mg/day
Sunday, January 10
Julia took us to see A Bug's Life, which she had already seen. Great movie! Very entertaining, the voices were well done, and the animation is simply brilliant.
Friday, January 15
A nasty winter storm hit us starting yesterday, capping off a week of bad weather and worse commuting. It started as sleet and freezing rain, then snowing a few inches. This morning was very nasty, as the temperature was about 15°F and it was raining. This is what we call an ice storm. I was driving to work, which is about 30 miles north of our house. I had the defroster in our RAV4 on full heat, full blast the whole way, and I had to keep spraying windshield washer fluid almost constantly to keep the windshield clear. It was raining and turning to ice the instant it hit anything, especially my windshield. I used over half a gallon of fluid in less than an hour! Needless to say, school was cancelled for Julia, giving her a 4-day weekend (because Monday is the day we celebrate Martin Luther King's birthday - it's a national holiday in the U.S.)
The blast of cold weather is making Julia's rash look very bad this week. I think we're back to that normal "bad rash" that we were hoping to be rid of. :-(
Monday, January 18
We had more than a bit of a hassle with one of Julia's medicines this week. Our usual drugstore stopped carrying the Plaquenil (hydroxychloroquine) that we're used to, and replaced it with pills that are much larger in size. They're at least twice as big, and instead of being a smooth oval shape, they're rough cricles with relatively sharp edges. They're bigger than the 20mg Prednisones we had so much trouble with! Julia tried to take one and just about gagged on it. I didn't want to imagine that scene every morning.
We called the pharmacist and he just about blew us off on this. That got us all very angry. Why couldn't they do a little leg work and try to get us the same stuff we'd been getting from them for months? I was just about ready to have all our prescriptions moved over to another pharmacy chain, and a strongly worded letter to thir corporate offices soon to follow. We went back and forth a few times before we finally talked to someone who was sympathetic. They called around and got us some of the old-style pills. They only give us a 30 day supply (HMO rules), so we may be going through this on a more-or-less regular basis. Sigh.
Wednesday, January 20
Julia scored a Furby today. I have to admit, it is cute. I wonder if I can talk to it (or better yet, control it) with my Palm III?
Saturday, January 23
Three days of Furby bliss came to an end today. All of a sudden, without warning, Julia's Furby "locked up" and started buzzing loudly. I quickly tried to press his reset button, to no avail. I finally grabbed my Leatherman, unscrewed the battery comprtment, and pulled the batteries out. I put them back in and hit reset, but now his voice sounded funny (funny-weird, not funny-haha) and his gears seemed a little louder. He was very sick. We sadly decided to return him and see if we could get a replacement.
Julia also had a basketball game today. She's looking better and better as the weeks progress in basketball league. She's working hard out there, and keeps up very well. She looks so flushed and red at the end, though, and she's also quite tired. Nothing a shower doesn't help, though. However, that rash has been looking particularly bad this week, and it's spreading into her palms, wrists, and ankles.
Yes, it's time for another drop in Prednisone dose, down to 4mg now.
|Current Drug Dose:
||Folic Acid: 1mg/day
Sunday, January 24
This is a slightly better day for the rash; she got her Methotrexate dose yesterday, and that sometimes seems to help the next day.
Monday, January 25
Julia's mom dilligently called every toy store in the area and tried to find Julia a replacement Furby. No luck - until she called the store she got the original. They told her that they didn't have any more when she bought it, but she alled and they had another, exactly like Julia's! This was nothing short of a miracle. Julia was extremely pleased.
Saturday, January 30
Julia had another game today. She's really working hard out there, playing tough D, and working hard to get open when her team had te ball. She got a steal and even scored a basket! There were only 8 girls at the game this week, and Julia worked 6 of the 8 4-minute shifts. She was pooped afterwards, but she kept up very well, right to the end.
Not coincidentally I'm sure, her rash looked terrible today, her hands and wrists especially.
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