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Saturday May 1
Well, this is the big day! Julia's been looking forward to her First Communion for months.
Things didn't start out so well at first. Julia was up in the middle of the night with a stomach ache. She mentioned this yesterday, but we gave her a Pepto-Bismal caplet and she felt fine immediately. She did eventually get to sleep, and she slept later than usual in the morning, as if to compensate. Dusring the morning, however, she had a bout of diarrhea and that left her feeling a bit weak and tired for a while. Also, during her First Communion mass, she got more stomach pains, and afterwards, when she would have been The Life of The Party, she was so tired she took a nap for almost an hour. Hopefully, this is not a reaction to prednisone or something like that, and also that this will not develop into anything more serious.
Aside from that, the First Communion mass was wonderful and joyous. Julia class was 58 boys and girls, which we are told is about average. This left seating at a premium (each child had only half a pew of reserved seats), and the church was standing room only by the time Mass started. I'm sure Julia was happy, but it would have been nicer if she wasn't so distracted by her stomach ache. The actual taking of eucharist was bread only, so each family could decide on whether they wanted to have both species.
We had friends and relatives over to our house afterwards, and just about everyone had a wonderful time. The weather was absolutely picture perfect - cloudless and quite warm and comfortable - a better day could not have been engineered if such a thing were possible.
Julia's Prednisone dose drops to 20mg/day today, and her Methotrexate dose increases to 25mg/week for the first time.
|Current Drug Dose:
||Folic Acid: 1mg/day
Sunday May 2
A rare "down" day for us. We just went to church (where Julia could celebrate eucharist again), and afterwards there was a reception for our parish priest, who happens to be celebrating his 30th anniversary of priesthood. It was a lot of fun, and the food was good, too. After that, we just hung around the house and watched a little TV and played a board game.
It was nice to be able to relax for a change, but Julia was still complaining throughout the day that he stomach hurt, and she hardly ate anything all day. Then at about 10:30PM, Julia woke up with another stomach ache. We gave her another Pepto-Bismal caplet, but just a couple of minutes later, she had a long vomiting spell. I've been vomiting-sick before, and I know how miserable that can make make you feel, so I really felt sorry for her. It took her a while to fall asleep, but once she did, she slept through the rest of the night.
Monday May 3
Julia was not feeling well, so we kept her home from school today. She *hates* to miss school, but even she agreed that she needed at least a day to rest and recover. She felt better in the afternoon, but still not 100%. No fever. We'll be taking this day to day.
Tuesday May 4
Julia felt pretty good this morning, despite a small bout of diarrhea before school. I should have taken the hint. Her stomach started bothering her more and more as the day progressed, and late in the morning, we got a call from the school nurse asking someone to come get her. As soon as I got her home from school, she immediately laid down and took a 90-minute nap. She obviously didn't feel too well. Julia was disappointed but understanding when we talked about skipping swimming tonight. She was feeling a little better in the evening, thankfully
Today is the day that I was supposed to call Dr. A.W. about Julia's Prednisone anyway, so I piggybacked that call with questions about how to handle this bug Julia seems to have caught. I spoke to her briefly in the afternoon, and she just took the information about how Julia was doing and said she wanted to consult with Dr. S. before making any recommendations. When she called back later, she was her usual patient self, and talked with us for more than half an hour. She even chatted with Julia for about 10 minutes.
She's concerned that Julia is not feeling well, and also that the virus can cause the JDMS to flare up. We're going to stay at 20mg/day of oral Prednisone for a few extra days. Although Prednisone is an immune system suppressant (moreso at higher doses than what Julia is taking), it's also an anti-inflammatory that will help her fight the virus. We're also going to increase her folic acid from 1mg to 2mg/day, just for a few days, to counteract the effect of the Methotrexate. MTX is much more of a pure immune system suppressant, and it can allow the virus hang on longer than it otherwise would; the extra folic acid will suppress the action of the MTX as long it's taken.
We also discussed proper hydration while Julia is sick. We need to get as much water into her system as possible, as dehydration is a real possibility when there's diarrhea and vomiting going on. She also suggested more of a BRAT diet (Bananas, Rice, Applesause, Toast), and to avoid milk which can be difficult to digest. Yogurt is okay, though.
Finally, we talked about the next step. We will need to call next week to discuss the Prednisone tapering schedule. We obviously don't want Julia on it any longer than necessary, and neither do the doctors. Dr. A.W. said that, assuming that Julia makes a complete recovery from this virus, Julia should be off of Prednisone again within "a few weeks". She explained that kids with asthma and other diseases routinely go on Prednisone for a month or so, and don't suffer the severe side effects that longer-term therapy causes.
|Current Drug Dose:
||Folic Acid: 2mg/day
Wednesday May 5
Discretion being the better part of valor, we decided to keep Julia home from school today, and she'll miss dance class today, too. We're going to keep her home until she's been well for a day; then she can try school again. She likes to complain about school, but she's complaining a lot louder about missing it. I think she secretly like school a lot more than she lets on.
Julia had several more bouts with diarrhea today. We were much more dilligent today about her diet and fluids, and she seemed to actually feel better in the evening.
Thursday May 6
Well, a pretty normal day today for Julia. No more cramps and diarrhea, and just a minor and brief stomach ache in the afternoon. She even felt well enough to go swimming in the evening. Her swimming stroke looks a lot better already than when I saw her last week.
I also have to note that Julia's rash, especially her face, looks exceptionally good today. It hasn't looked this good in a long time. It's terribly frustrating that the only thing that helps her rash is high-dose oral Prednisone.
Friday May 7
After another good night's sleep, Julia went to school today. All appears to be back to normal. We will contact the doctors next week to get a tapering schedule for the Prednisone.
Monday, May 10
So much for tapering. Sigh.
We (actually, Julia's mom because I was off this week on a business trip) had a lengthy phone conversation with Dr. A.W today. She and Dr. S. reviewed Julia's history back to February - her medication, her blood work - and they don't want to taper her off the Prednisone now, only to have to have her start up on it again in a few weeks because she's having symptoms again. They really want to see the disease under control before we take her off of Prednisone because it works the best for her. Basically, they want to see her numbers back in the normal range before they start tapering. Dr. S. does not feel that, at her current weight, 20mg of Prednisone will not give her any side effects, so they want to keep her here until she gets tested at her next visit.
Dr. A.W. asked what we thought about this, she really wanted our input. This approach made sense. The doctors are very aware of the effect on Julia psycologically, they know how much Julia hates taking the pills. She asked if she hated taking them because they tasted bad, or because they reminded her of her disease; probably, mostly the latter. She also offered that we could get labs done before our next appointment and if they were good we could start the taper before then. Our sense was that we should wait until that next appointment, and that the lab results probably would not be normal yet, so an extra test would not tell us much. She agreed, and said that Julia's recent stomach flu would also have had an adverse effect on her labs, so she wouldn't expect them to be normal yet either.
Dr A.W. then talked to Julia for almost 20 minutes, explaining to her what we wanted to do and why. Julia seemed to appreciate that, and she also did not want labs taken before our next appointment. However, she told Dr. A.W. she hated taking the pills for both of the reasons. The doctor suggested we try to mash them up in applesauce, jelly, or whatever Julia wants. She really doesn't care how we get them into her, as long as it's the least traumatic.
She said that we may need to think about getting Julia her Methotrexate via injection at some point. There comes a point where the dosage is such that it does not get absorbed well enough orally, and you need to give it via injection. She said Julia is almost at that level, we'll need to see where her labs are. If we need to do it, we can use EMLA, it would still be once per week, and we could go to our local doctor, or we could learn to do it ourselves. I imagine we'd learn how to do this ourselves. We used to give Julia injectable MTX orally before we switched to pill form. She also said that we really don't need to worry about Methotrexate's serious side effects until the dose get into the high 30mg's range.
Julia really seems to enjoy talking to Dr. A.W., we agree - Julia is older than her 8 years and i think she appreciates hearing the logical side of things and being involved in her own treatment.
(thanks, Mom, for the great notes on the phone call!)
Tuesday, May 16
Dr. A.W. said (via email) that, since Julia seems to be fully over the stomach flu, it's OK to go back to 1mg/day of Folic Acid, so we're making that change today. Also, Julia tried her Prednisone mixed with a teaspoonful of jelly, but she said that tasted worse than just swallowing the pills. She decided it was easier to just take one larger 20mg pill than two smaller 10mg pills, so that's what we're giving her for the time being.
|Current Drug Dose:
||Folic Acid: 1mg/day
Monday, May 17
Julia seems to be developing some brownish patches on her skin. The patches have river-like darker brown lines in them. I might have thought this was just suntan, but she's using her SPF45 sunscreen every morning.
Also, the warts that we've been fighting on the bottoms of Julia's feet are back with a vengeance. In addition to the two large warts and a couple of smaller ones, there are now several babies that have started to show. We're being more aggressive with the wart cream, but I emailed the doctor about these two problems.
Naturally, she couldn't tell much without seeing her. Se said we may be able to have a dermatologist have a look when we're in for an appointment next week.
Wednesday, May 19
Julia skinned her right knee playing outside today. It's very sensitive to touch, and the slightest contact with it has Julia howling in pain.
Saturday, May 22
We were on our way out the door to go to church, when Julia slipped and fell down a half a flight of carpeted stairs. No damage done, fortunately. She did aggravate the skinned knee from the other day. It does seem slow to heal, and the fall didn't help.
I do have to say that Julia's rash is looking much better these days. It's moderated from that angry red to a much softer reddish color. Still, we are getting comments from people asking about her "sunburn," people who no doubt are judging us bad parents for letting her get a sunburn in the first place. It does grow tiresome to have to explain about auto-immune disorders and medication (although practically everyone knows at least something about Prednisone), but, like this Diary, I consider it a mission of education.
Tuesday, May 25
Another trip to Children's Hospital for a checkup today. Vital stats: Weight - 32.3kg./71.2lb., and height - 50.5"/128cm, both unchanged from a month ago. This is good considering she's on 20mg of Prednisone a day and her appetite is noticibly increased. Blood Pressure 134/66. The first number is a bit elevated, but we asked about that and they aren't concerned about that in younger patients; if the second number were elevated, that might be a side effect of Prednisone, but hers is normal. Temperature - 98.0°F/36.7°C. Normal.
Pretty much normal clinical exam, first with Dr. A.W. then with Dr. S. and a visitor (Dr. B. from Russia again, she saw Julia back in March). He noted that Julia's neck muscles are still weak, and she was having more than a bit of trouble picking her head up to do the neck strength test. Most of that was just her not knowing what to do for them exactly, but some of it is clearly weakness. Doing sit-ups was tough for her; she can barely do one now, where a few months ago she could snap off a half dozen in short order. On the other hand, they said her legs are stronger than the last visit.
They had a look at the brown patches on her forehead and arms, and they thought they might be a post-inflammatory skin reaction. They had Dr G., the dermatologist, have a look. He looked at these patches quite closely and agreed that they look a bit unusual with the brown lines running through them, but concluded that they are sun related. We will be more dilligent about the sunscreen, especially as summer approaches. Dr. G. also asked about the Dovonex. We'd used it for about 3-4 weeks before deciding that it didn't help. He suggested trying it on one knee for 2-3 months so that we can compare it with the other knee to see if it's helping.
Next, we talked about the warts on Julia's feet. They agreed that both Methotrexate and Prednisone can cause these to take longer to go away, and can allow then to proliferate more quickly. We talked about removing them with liquid nitrogen treatment (freezing), or cutting them away. They also gave us some samples of a prescription ointment to use. However, it's only 15% Salicilate, while the over-the-counter stuff we've been using is 17%. They did suggest we not cover them with ordinary bandages, as they absorb the medicine instead of letting it sit on the warts. We can get some special bandages that don't absorb.
Finally, we talked about medication. We have seen some improvement since a month ago, being on 20mg/day of Prednisone. Her muscles are noticibly better, and her rash is dramatically better. As I said, now it looks like a sunburn, not a rash. Dr. S. said that the goal is to get Julia "perfect" - meaning asymptomatic, no rash, and all blood tests normal - before we start reducing her medication again. I get the feeling he thinks it would have been better if we'd stayed on Prednisone the first time, but he didn't say this. We talked about other medication, like Cyclosporin, but we have had good results with Prednisone and Cyclosporin has similar side effects.
He then talked about absorbtion. One of the effects of JDMS is that the intestines do not absorb medication as well. He believes this is why the Methotrexate is not working as expected. Julia is suffering not apparent direct effects or serious side effects of the MTX, so he thinks absorbtion is the problem. Depending on the lab results this week (which he expects will be improved, but not perfect yet), he's probably going to recommend that we switch to injectable MTX instead of oral.
We talked for a long time about the injection process. The injections will still be weekly, and they will be subcutaneous (under the skin) instead of into a blood vessel (intravenous) or into a muscle (intramuscular). Going to injectable MTX will be effectively an increase in MTX dose because of increased absorbtion, so we're staying at the same dose, 25mg/week.
They are starting a program with a new drug vendor who delivers pre-filled syringes directly to our home. They deliver a 4-week supply at a time, and they are pre-measured with the correct amount of MTX (1 cc at 25mg/cc). This is cool because it saves us the time and hassle of filling the syringes with medicine. One of the staff nurses walked us through the whole procedure, and gave us several sheets of information to take with us. They included how to properly fill syringes if need be, how to select an injection site, and how to administer the actual injection. The needles themselves are tiny, the smallest bore needles that they manufacture ("30 gauge"). The nurse said they're about the same diameter as a mosquite bite! It looks pretty straightforward, and some kids (like diabetics) have to inject themselves as many as 4 times a day, so we should be okay with this.
We'd actually used this injectable MTX last year, before we switched to MTX tablets, except Julia would just take the liquid MTX by mouth. The procedure was largely the same to fill the needle, except we used needles that were removable from the syringe and just squirted the medicine directly into her mouth. Also, that procedure didn't require sterility that injecting does.
Here are the results of today's blood tests:
Also, White Blood Count 5.8, hematocrit 40.1, platelets 374, and sedementation rate 6. All these are normal.
Wednesday, May 26
Julia was complaining about her calves hurting most of the day today. I don't know what she could have done to make them sore, but that's what the pain she described sounds like. We had a "project night" at Julia's school, and while she was there and her attention was diverted, she didn't complain at all. As soon as she was alone with us, or had a minute to think about it, she started walking around like a 90-year-old. We gave her a bath instead of a shower tonight, and that seemed to help, and we gave her a Tylenol at bedtime, and that helped her sleep.
Dr. A.W. called tonight with the latest blood tests (see yesterday's entry). She said that the lab there has streamlined their procedures and instead of test results taking several days, they now usually get them back within 24 hours.
The results are not as good as we'd hoped/expected. The Aldolase result is not available yet. Julia's CPK number is actually up significantly, much to our chargrin. The LDH is essentially the same. However, the AST and ALT are headed in the right direction. The rest of the numbers are essentially normal. So, the results are mixed. Since we're not seeing a significant improvement, we are going to injectable MTX starting this weekend. Dr. A.W. was adamant that Julia is doing very well clinically. The fact that the numbers are not as strong as we'd hoped are likely the result of the stomach flu she had a few weeks ago. Not only does the virus cause the JDMS to flare up, but the diarrhea she had probably reduced the effectiveness of the medicine she was taking, essentially removing it from her body before it could be absorbed.
She was very insistent that we go to a medical professional to have Julia's first MTX injection. We will probably go to our local family medical practice to have them show us how to do this, or rather, to have them supervise us as we do it. The nurse at Children's showed us how it's supposed to go, and we decided that we'd go to have someone make sure we were doing it right. We frankly don't have the time to go have this done every week, and doing it ourselves gives us a sense of control. I'm also hopeful that Julia will want to learn to self-administer the injections. They told us plenty of kids her age are doing self-injections.
Thursday, May 27
Julia's leg muscles were feeling better today, thankfully. This means this is not a disease process, but rather just some soreness of unknown origin. She felt fine by the afternoon, and went to swimming lessons. Julia is now Level 4 certified!! Congratulations, Julia!
In the evening, we set up a visit to our family health practice. They are going to supervise us doing this first Methotrexate injection; after that we'll be on our own. We (Dad & Mom) actually going to inject each other first to get the hang of it, and to show Julia that it's just not a big deal. We're friends with one of the pediatric nurses there, and she was generous enough to offer to come in just to help us out. This will make the whole process more comfortable for Julia, since they already knows each other.
Friday, May 28
Snafu du jour - our pre-filled syringes of injectable methotrexate didn't show up. I tried to track down the staff nurse at Children's, but she had the day off. I paged the rheumatologist on call, and explained myself. She tried to track down the status of the shipment, but she had no success. Instead, she called our pharmacy and called in a prescription for a vial of Methotrexate and a 30 gauge needle. We'll have to track down the syringes next week.
Saturday, May 29
Today was our first day to give Julia an injection of Methotrexate. Our friend, who's a nurse at our health center, met us there and walked us through the process. There were a couple of significant omissions in procedure from what we were taught at Children's. More on that in a minute.
First, my wife gave me an injection as practice. I never before thought through all the steps a nurse has to go through to do an injection. First, you wash your hands and put on latex gloves. Next, the needle is prepared - we used a 26 gauge needle, because that's what the nurse happened to bring us to practice with. If the vial is being used for the first time, it's okay, but if not, you have to alcohol swab the vial. Then the medicine (in our test case, it was a vial of sterile distilled water) is drawn into the syringe.
Deviation number 1 - they said that you should express air into the vial in the same amount that you plan to withdraw. They never told us to do this at Children's. We did do this because it's very difficult to draw the medicine out without doing this because of the vacuum that's created in the vial.
As you draw the medicine into the syringe, your will invariably draw air along with it. These air bubbles have to be ejected back into the vial without withdrawing the needle. The technique is to draw back a little more liquid than you need, snap your fingers on the syringe to make the bubbles rise to the top, then push the syringe back to the correct level.
Next, you have to prepare the injection site, scrubbing it with another alcohol swab. You have to be careful where you put down the syringe. Then you have to insert the needle into the site by pinching the skin and inserting the needle with a quick stabbing motion. Then you smoothly inject the medicine, withdraw the needle, swab the spot, and put a bandage on it. The used needle is supposed to go immediately into a sharps container (we will use an old bleach container at home).
Deviation number 2 - they told us to draw back on the syringe after you insert it to make sure that you don't get a blood vessel. They didn't tell us about this at Children's either. We didn't actually do this, but I will ask about it.
As I said, my wife tested on me first, then I tested on her. We had the 26 gauge needles, which are a bit bigger than the 30 gauge Julia will use. On me, I felt the stick, and a bit of pressure as the water went in. My wife said it hurt her quite a bit, but she didn't let Julia see that it hurt her. She said that injecting the water was rather painful.
We had put EMLA cream on Julia about an hour before we went in. I took off the EMLA, then did the injection on her just like I explained above. She didn't feel a thing!! In fact, she wasn't looking and she couldn't even tell when the injection was happening. It was a complete non-event.
Hopefully, all this effort will be worth it. Julia was extremely tired about 2 hours after the injection; she even fell asleep during church this afternoon. However, after she woke up (she slept about 10 or 15 minutes), she was fine. Our priest even gave her the Sacrament of the Sick annointing after mass.
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