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Monday March 1
Julia had back-to-back appointments today, one with the eye doctor and one with the dentist. Both went very well. I report thirdhand here because I wasn't at either appointment.
The eye doctor is great with kids, and was very knowledgable about JDMS and the drugs she has to take. He ran her through a complete battery of tests, including color blindness tests and glaucoma/cataract tests. Both conditions are side effects of "Plaquenil poisoning", as he called it. He explained that such effects are very rare in kids at all, and even more so in patients with as low a dose as Julia is taking. He said her vision was completely normal, and we'll be back in six months.
The dentist is similarly well-equipped to handle kids. Julia had no cavities, and they said she did a great job brushing and flossing. There are just a couple of spots that need a little more attention, and we need to check her more closely in these spots. They also talked about orthidonture at some point in the future, as Julia has some gaps in her teeth that are a bit too large. Aside from that, she god a clean bill of oral health.
Wednesday March 3
We note that exactly one year ago today was when we got our first, preliminary diagnosis of JDMS. It's been quite a year for us, to say the least.
Saturday March 6
Julia went with me this morning was I went to give blood, as I do every 8 weeks. She told me last night that she wanted to come, but she had trouble falling asleep because we were holding a meeting at our house and the din was keeping her up. I gently woke her up about half an hour before I had to leave for the donor center, and she told me she wanted to stay home. As I got myself ready, she did too, and surprised me by being ready to go on time. We had a nice conversation at the center about the blood drawing process. I always tell her that the needles they use on me are like garden hoses compared to the tiny needles she gets for blood samples. Watching me taking those big needles doesn't phase her in the least.
While sitting in the snack area after giving, I was noticing what a bad day she was having with her face rash. Her hands and wrists were moderately bad today.
Julia's Prednisone dose plunges to 1mg today.
|Current Drug Dose:
||Folic Acid: 1mg/day
Sunday March 7
We went for our monthly Sacrament of the Sick, despite having to deal with the tail end of a moderate snowstorm - we got about 6" of snow. Her face looks a lot better today, but her hands, knees, and elbows are especially bad. There's just no telling from day to day.
Julia was feeling a bit tired today, and also had a bout with diarrhea. Nothing serious, but we'll keep an eye on her.
Monday March 8
Julia is cranky this morning, complaining about being tired, not wanting to get ready for school. She also was moody this afternoon, much more so than normal.
Wednesday March 10
Julia's been tired and cranky all week so far, and this morning is no exception. This has us a bit worried - the tiredness can be a symptom of adrenal problems that they were looking for at her last checkup. Maybe this tiredness is being caused by her drop in Prednisone dose? The timing is right.
I called Dr. A.W. to ask about this. I had to page her and have her call me back. I described everything that's happened with Julia since Sunday, and she said that it's almost certainly being caused by her reaction to a virus. There's been a lot of diarrhea and low-fever viruses going around, she said, and Julia's compromised immune system will make her not only more succeptable to becoming infected, but also slower to recover from it. Also, her Prednisone dose (1mg/day) is low enough that she's not enjoying any significant anti-inflammatory effect from it. She said that if Julia isn't doing better by Friday, to call her and they will see he in clinic next week, instead of our scheduled appointment in a few weeks.
She also said that her Prednisone dose is "trivial" for her body weight, and her body is producing far more natural corticosteroids than she's taking. That means that the reduction in Prednisone dose is not possibly the cause of this tiredness.
After all that, she asked about how Julia's rash was doing. I said that it's as bad as ever, still better and worse days, but overall it's just as bad as when she was diagnosed a year ago. She asked because she'd been talking to Dr. S. about it, and at our next visit, they want to discuss alternative therapies for the rash. I agreed with her. When we were first diagnosed and Plaquenil was perscribed, Dr. S. said that they typically see results from it in 6-9 months; later, he revised that to 6-12 months. Julia's been on Plaquenil for over a year now, and if 370-odd doses (she's never forgotten to take her meds so far) didn't do much of anything, then it's time to consider something else. She mentioned IVIG (Intravenous ImmunoGlobulin), and I mentioned that Dr. S had talked to us about pulse steroids treatment at the visit she'd missed. Se said we'd discuss it when we were in the office next time, whether it's next week ot later in the month as previously scheduled.
After all that, I'm pleased to report that Julia's mood and tiredness were both vastly improved this evening. It looks as though Dr. A.W. was right - this was a virus that she took a few days to shake off. We'll watch her closely the next couple of days, obviously, but I'm hopeful that this little crisis is behind us now.
Friday March 12
Julia's energy level has been fine for the past two days now. It apparently was what the doctor said, just her getting over a virus or something.
Julia had the day off from school today. I'm not exactly sure why. She took a good part of the day to read. Lately, she's been into the Animorphs, which is a series of books by K. A. Applegate, and companion TV show. We decided to order the whole set of available books for her birthday last month from Amazon.com, and she's been plowing through them like a maniac! What's amazing is that she's just turned 8, and these books are listed as being for ages 9-12.
Saturday March 20
Regular readers will note the utter lack of Prednisone in Julia's drug dose listing, which I post every time any medication or dose changes. Today is the first day in 378 that she has not taken any Prednisone. This is a good thing. We thought about having a mini-celebration, but we fear that it'll be too disappointing if she has to get back on Prednisone, especially if that happens any time in the near future. I mean, her rash is still bad and we're talking about other treatments for that, so we're going to hold off on any celebrating for now.
We attended a wedding mass at our church this morning, then I went off to get Linux installed on an old PC (unsuccessfully, unfortunately, due to a hard disk hardware problem) while the girls went off shopping. While, at the mall, Julia decided she wanted to get her picture taken with the Easter Bunny. Here it is!
|Current Drug Dose:
||Folic Acid: 1mg/day
Saturday March 27
We went to Palm Sunday vigin mass this afternoon, followed by our church's annual Seder Supper. A great time was had by all, as usual. This is our 6th Seder. It's a wonderful event, and a great way for us to start our Holy Week observances.
Tuesday, March 30
Julia had a very eventful doctor's appointment today. Her rash was bad today, as it often seems to be on doctor visit days. For the first time since November, we saw both Dr S. and Dr. A.W. today. We also had a few other visitors, but I'll get to that later.
We've been getting Julia's blood work done before the exams lately. If we wait until after, sometimes the regular blood drawing center is closed, and the after-hours center takes a long time. The one time we went there, we waited for over an hour before we got our turn, which made for rather a late evening for us by the time we finally got home. I noticed that Dr. A.W. ordered the von Willebrand factor test again; I asked, and sure enough they never got that result last time.
Vital stats: Weight - 33.4kg./73.7lb., lost almost another 2 pounds, woo-hoo! Height - 50.5"/128cm, up another half an inch. This continues a great trend. At this rate she'll be weightless and infinitely tall in no time. Blood Pressure - 119/60, Temperature - 98.5°F/37.5°C, both normal.
Dr. A.W. performed her usual exam - general questions to Julia and to us about strength, tiredness, keeping up with the other kids in dance and gym. The only complaints Julia had were that her right eyelid is itchy sometimes, and that she's cranky in the mornings. I think she's hear me say that about her more than once. The doctor then performed the normal physical exam: strength and flexibility tests, listening to heart and lungs, etc. All seemed normal, except she mentioned a slight weakness in Julia's neck muscles. This is the same thing Dr. S. mentioned last time, and she reiterated that the neck muscles are the last to fully recover.
Dr. A.W. graciously made me a photocopy of an article ("A Controlled Trial of High-Dose Intravenous Immune Globulin Infusions as Treatment for Dermatomyositis") from the New England Journal of Medicine. I only have access to the abstracts of NEJM articles online (why they don't give access to full articles from back issues, I have no idea), and this one looked interesting. I tried to get a copy from our town library, but they only have the Journal back for 3 years, and they don't have access to the full texts online, like I expected. Anyway, the article describes a controlled trial of adults with Dermatomyositis. The results on muscle symptoms were dramatic, and I wanted to see the whole article.
Next, Dr. S. came in with a visiting doctor from Russia, Dr. B. (whose whole name I didn't quite get). Dr. B. was there to observe; apparently, she'd never seen a case of JDMS before. Dr. S. then repeated the physical exam, and confirmed about Julia's neck muscled being a bit weak, but everything else being very good.
Talk next turned to Julia's rash. It was very bad today, as I said before. Dr S. mentioned about the Journal article, and said that he had some concerns with it. First off, it was a study of IVIg effect on muscle symptoms in adults with dermatomyositis. Not very applicable to JDMS skin rash. Also, the results have not been repeated in the 5 years since the article was published, and the patients in the study did not have encouraging long-term results. Still, he said that IVIg was effective in treating the rash in his experience.
That said, he went on to talk about the risks. IVIg is a human blood product, and there are inherent risks any time a blood product is used. He said that there was a Hepatitis C outbreak among IVIg users a few years ago, before there was a reliable test for that disease. Of course, all blood products are tested for HIV and Hepatitis, but it's impossible to know what the next infectious agent might be. There are also side effects in up to 30% of kids who get IVIg, most notably headaches, and ironically, skin rash. Still, it is one of the few treatments that have proved effective against JDMS rash.
The other treatments besides IVIg that have proved effective are Prednisone and Plaquenil. We don't want to put Julia back on long-term high-dose steroids unless it's absolutely necessary, but short-term, moderate-dose therapy may be something we can try. He was talking about a dose of 10-15mg/day for about 2 weeks, with a rapid tapering schedule. He said this would not cause the adverse side effects that higher-dose therapy would cause, and this kind of course of Prednisone is prescribed routinely for patients with things like asthma.
We mentioned that Julia makes her First Communion in a few weeks, and he said that we could try to time the Prednisone so that her rash is minimized that that day. This is done with other patients who are planning a vacation, or have a similar event coming up that they want to be at (and look) their best for. We talked about this at some length afterwards. If this Prednisone therapy is something that we wouldn't otherwise be doing, then we'd be giving Julia a powerful drug for essentially cosmetic purposes. This decision is going to require some thought.
Dr. S. also mentioned that some patients think that the rash responds better to name-brand Plaquenil instead of the generic hydroxycholroquine that Julia's been taking. The drugs are supposed to be chemically equivalent, so it shouldn't matter. Dr. S. said that he's skeptical of this claim. However, it can't hurt anything to try it, so we're going to go with Plaquenil for a couple of months and see if we see any difference.
Dr. S. wanted to bring in a dermatologist to see if there's anything we can try topically that would help. Who did he get, but Dr. G, the dermatologist who saw Julia on that first day at Children's over a year ago. He seemed to remember Julia. He looked over her rash and he prescribed a couple of ointments and a cream for them. The first is Elocon, which according to the package insert is a 0.1% ointment of mometasone furoate. This is a steroid ointment for Julia's face, to be put on twice a day for one week only. Extended use of medicines like this can cause thinning of the skin, so we're going with short-term use to see if it has any effect. The other stuff is called Dovonex (0.005% calcipotriene), a synthetic form of Vitamin D and that was prescribed in both cream and ointment form. The cream is a bit lighter and is more appropriate to use at the beginning of the day. We can and should still use sunscreen on top of it. The ointment is heavy and greasier, and is more appropriate to use at the end of the day. We'll be trying Dovonex on Julia's knees and elbows for about 2 weeks to see if it has any effect.
Dr. G. asked if we wouldn't mind if his resident, Dr. L., could come in and have a look. Julia says she doesn't like all the doctors coming in, but I think she really likes the attention. Julia had already changed from a hospital gown into her clothes, and he didn't make her change back. He just looked at her face and elbows.
In closing, Dr. S. said that Julia's rash is the most persistent he's ever seen. The plan, then, for now is to use the Elocon and Dovonex topically and change the Plaquenil to name brand form. In short order, we will have to decide if we want to try the short-term Prednisone therapy. If none of that helps in 2 months, we will probably try IVIg.
|Current Drug Dose:
||Folic Acid: 1mg/day
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