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Wednesday, April 1
Julia was home sick from school today. She was up half the night with diarrhea, cramps, and so much gas that her abdomen was all swollen up. We suspect a virus, or possibly food poisoning. We gave her one anti-diarrhea pill, and modified her diet to be more bland (bread, bananas, etc), and by the end of the today, she was back to normal. Julia does amaze me by how fast she bounces back from stuff like that. Oh, and her cough is much better; she hasn't needed any cough medicine at bed time.
Friday, April 3
I received an email yesterday from a reader suggesting that this page is the work of a medical student doing a thesis. After checking the date to make sure it wasn't April 1, I replied saying that I can't prove I'm not a med student, at least not without a face-to-face meeting, but look around at Julia's home page, at my home page, and so on, and decide if a med student would bother with all that. I did get a good laugh out of the email anyway, so it wasn't a total waste of time.
Julia's rash is looking much better the past couple of days, probably with the return of more seasonable teperatures in the area. In fact, you can hardly see any rash on her hands at all - if you didn't know it was there, you wouldn't notice. Her face still has the rash, but it seems less pronounced. Probably just more day-to-day variability, I'm certain that the next warm day will bring the rash right back. I have gotten a couple of suggestions for creams and lotions to use on Julia's rash - keep them coming, we're going to try each one for a week or so at a time to see if they help. Eventually, I'll publish a report here.
Sunday, April 5
We all attended our church's annual Seder Supper last night, and we were the "host family" this year - we got to sit at the head table, and do some of the readings that are part of the ceremony. Julia thought this was a very big deal, and had practiced her part all week. She did a great job, and we all had a wonderful time. Unfortunately, between staying up late for the Seder, and losing a hour of sleep for the start of Daylight Saving Time, she was pretty cranky today.
We took Julia for the monthly Sacrament of the Sick again today. Our parish has it every month, there were about 10 people there today. Her rash was looking pretty good, but the special oil used as part of the sacrament gets put on the backs of her hands and really accentuates the rash. This is similar to the doctors putting petroleum jelly on her hands to make the capillaries show up better.
Julia's appetite is as strong as ever, but she stepped on the scale today, and she seems to be about the same weight, if not a half-pound lighter. Still, she's looking a bit puffy, and some of her clothes aren't fitting around the waist any more.
Friday, April 10
We are having trouble with another round of diarrhea today. Since this is the second time this has happened, we paged Dr. A.W. She asked about her symptoms - diarrhea, gas, cramps, and extreme bloating of her abdomen. She also asked if there were other symptoms - vomiting, blood in the diarrhea, fever, aches, that might indicate a virus or food poisoning, but Julia has none of that. The doctor said that this could be a lactose intolerance problem. Julia has been getting more milk since she started on the Prednisone, 4-5 glasses a day compared to 2-3 previously.
Dr. A.W. suggested an antacid with simethicone, which is an anti-gas ingredient, and reducing Julia's milk intake to 2-3 glasses a day after she gets over this problem. We will need to compensate for the reduced calcium by giving her more calcium-rich foods, like broccoli and yogurt. The doctor also asked us to call her tomorrow and give her a status update.
Saturday, April 11
Talked to Dr. A.W. this morning. Julia is doing well, back to normal really. She has a normal, albeit small, bowel movement this morning, and the gas, cramps, and bloating are gone. We're limiting her to 3 glasses of milk a day now. Someone suggested to me that we supplement her calcium intake with an antacid tablet daily. The doctor thinks that this is not necessary right now, especially if we keep up with Julia's diet.
Monday, April 13
I finished the book Coping With Prednisone while on a long plane ride. The book is divided into two parts. One part, written by Eugenia Zukerman, chronicles one personal struggle with a rare lung disease and consequent struggle with Prednisone use. The other part, written by Julie Ingelfinger, M.D., is mostly medical facts, written by the sister of the lung disease patient. My overall impression is similarly split.
I found the Zukerman's description well written and descriptive, but not very applicable to the general Prednisone user. She is a professional musician and television personality, so she was rightfully very concerned about her appearance changing because of the Prednisone. She radically changed her diet, almost completely eliminating salt and sugars, and closely monitoring her caloric intake, despite her ravenous appetite resulting from the drug. While this is an admirable ideal, it is somewhere between impractical to impossible to use this approach on a 7 year old. She also described her personal struggle with the emotional and behavioral effects, but these effects vary so widely from person to person that, again, I found it mostly inapplicable to Julia's situation.
Ingelfinger's medical information was just excellent. She was very frank in saying that there is precious little hard medical information about the side effects of Prednisone and other steriods, despite a veritable warehouse of information about their direct effects as an anti-inflammatory, anti-immune drug. This is refreshing and realistic, as most medical professionals would be hesitant to make a statement like this. The myriad positive effects of Prednisone are listed, and a partial list of diseases (Myositis is not listed, unfortunately) that are helped by is also here. The appendix of the book is loaded with tables of background information, technical/chemical descriptions, and other references. I learned a lot, and I can recommend this book most highly based just on this part of the book.
Saturday, April 18
We put Julia on the scale today, and it looks like she may have gained some more weight. This is not good. Unfortunately, Julia has been exposed to a lot of candy this week, being after Easter. We have another doctor's appointment in a few days. This is mostly our fault; it is very hard to keep saying No to Julia, especially when we went her whole life up until now encouraging her to eat. Now, we must discourage.
Sunday, April 19
Julia went to a pool party today, a birthday party for one of her schoolmates. She was in the pool almost the whole time, nearly 4 hours. She looked like a giant prune! I think she can keep up okay.
Tuesday, April 21
This morning, Julia complained of a minor headache, located at her right temple. She said this has been happening every morning since Sunday, but hadn't said anything to anyone. She said is goes away by itself within a few hours. Hmmm...
We had another visit at Children's Hospital today. This one was somewhat more stressful than previous trips, but mostly because we had to wait a long time to see the doctors. We were at the hospital for over two and a half hours, just to see the doctors for a a total of about 20 minutes and to get blood drawn.
First, they took Julia's height, weight, and blood pressure. She is up to 62.5 pounds, a gain of a pound and a half in about 4 weeks. Again, this is not good, but considering that Easter candy was involved, this could have been much worse.
Next, we briefly spoke to the head nurse, B. She was just checking to see if everything was going well. In the past couple of weeks, we had noticed that Julia is very sweaty, and she has had some trouble sleeping because she's "too sweaty". B. said that night sweatiness is a Prednisine side effect, and not to worry.
After a wait, Dr. A.W. came in, apologizing for running late, there was an emergency elsewhere. She said that the blood tests that we had been waiting for since March 6 (Scleroderma, von Willebrand factor) are "complete", but the results are not in the computer yet. She was waiting to get paged back with these results, but we never did get them today. She performed a physical exam on Julia, doing all the muscle flexibility and strength tests as usual. She noticed that julia had a couple of scrapes on he feet; one she got when she tripped getting on the school bus a couple of weeks ago, and the other she got in the pool during swimming lessons around the same time. They are healing slowly, yet another side effect of the Prednisone. Dr. A.W. said that Julia is doing extremely well, and "her strength is excellent".
Soon, Dr. A.W. came back with Dr. S., and a medical student, the same one who had been in with us last time. Dr. S. performed another physical exam, and also noted that Julia is strong, "back to normal". He did note one alarming symptom: Julia's blood pressure was 121/74; last time, her diastolic (lower number) was 55. Prednisone can elevate blood pressure, and while this is not dangerous at this point, this is a bad side effect, and we want to take action.
The plan is to begin tapering Julia off the Prednisone. This is a good thing! We had prepared ourselves for months to years of high-dose Prednisone, but Dr. S. said that 2 years of therapy would result in Julia not growing, gaining a significant amount of weight, and risk losing bone mass that could leave her vulnerable to injury.
By that time, we will be going back to the hospital for another round of testing. One small benefit: the new prednisone prescriptions are for 5mg and 1mg tablets, both of which at tiny, and Julia will have no trouble swallowing them.
- Starting tomorrow, we will be giving Julia all 40mg of Prednisone once a day, in the morning, instead of twice a day. This is supposed to help minimize the side effects. This will continue for a week.
- After that, we will start reducing the Prednisone dose by 4mg per day to 36mg, and administer that for 10 days.
- Reduce the dose to 32mg per day for 10 days.
- Reduce the dose to 28mg per day for 10 days.
If all is going well at the next exam, we will continue tapering the Prednisone dose. If the results are not going well, we will begin Julia on Methotrexate. We talked for a while about the effects and side effects of Methotrexate. Dr. S. called it a "steroid sparing agent", designed to do a similar job as Prednisone, but with less severe side effects. Methotrexate, in extremely high doses (500 times what he is planning to give Julia) is used for chemotherapy, but is taken in low doses by over 500,000 people. The most common side effect is oral sores, but that can be prevented with folic acid supplements, which he usually prescribes at the same time. Other side effects include an allergic reaction in the lungs (which has never been seen in children), liver inflammation (very rare in children, and we will monitor blood liver enzyme levels), and minimal immunosupressant qualities. There is no increase in cancer risk, nor any long-term effect on fertility.
Finally, we went to hematology and had blood drawn. We used the EMLA cream, but Julia had some pain this time. I think all the waiting may have made it less effective. The directions say you should put the cream on 1-2 hours before the blood test, but today it was more like 3 hours because of all the waiting. Also, one additional test was ordered; a CBC (Complete Blood Count). This is a baseline for future possible Methotrexate use.
|Current Drug Dose:
Friday, April 24
I did not speak to Dr. A.W. today, but Julia's mother did. First of all, we did get the result back (finally!) from the Scleroderma and von Willebrand factor tests, and both were normal (negative), as expected. However, it looks like some of Juila's enzyme numbers are up slightly. This does not necessarily indicate a problem, and these results are expected to fluctuate somewhat in spite of the drug therapy. I now have the exact numbers, see below. Still, because of this fluctuation, we are going to have to add a methotrexate dose weekly to compensate for the reduction in Prednisone.
My research indicates that methotrexate is a folic acid (a vitamin) inhibitor, and that a possible side effect of methotrexate therapy is folic acid deficiency, which can lead to sores in the mouth. Julia currently gets a daily over-the-counter chewable multivitamin supplement, and it includes 200mcg of folic acid. Dr. A.W. said that they routinely prescribe a folic acid supplement that is administered daily along with the Prednisone, except that the supplement is not given on the day the methotrexate is given. This is because the folic acid will actually reduce the effect of the methotrexate if they are given at the same time. Anyway, the doctor will check to see if the supplement she is taking is adequate to replace the folic acid deficiency, and if not, she will prescribe the supplement.
One problem with the methotrexate is that it is only available in a liquid form. Julia normally *hates* liquid medicines, because of the taste, but we seem to have no choice here. Fortunately, as I said, there is only one dose a week. Also, Dr. A.W. said that even small children tolerate methotrexate liquid pretty well, so we are hopeful that Julia will also.
Saturday, April 25
The prescription for methotrexate came in today, and we do not need additional folic acid supplements, at least not for now. Julia's dose is 0.6 ml, which at 25mg/ml (the strength listed on the bottle), is 15mg (a little more than .5 mg/kg of body weight), once a week. On the day she gets methotrexate, Saturdays, she will not take her daily vitamin tablet.
The methotrexate is shipped in tiny, 2ml, vials. This is just s 3-week supply for us. They do this so that the medicine is always fresh; apparently, liquid methotrexate has a short shelf-life. We have to use syringes to withdraw the liquid from the vials, then remove the needle and squirt the liquid into her mouth. Julia didn't have any trouble with taking the liquid medicine; she said it tasted "like nothing", meaning, I think, bland, like water. She does, however, have to chase it with a drink of milk or soda.
|Current Drug Dose:
Sunday, April 26
I did receive some email today from someone who is using Methotrexate in tablet form (2.5mg each) - in fact, they were told that it was not available in liquid form! I will have to ask the pharmacist next time I go there, just so I know. Julia didn't mind the liquid, so I expect we'll stick with it in any case.
Tuessday, April 28
I returned home form a business trip today, and not having seen Julia for almost a week was a bit of a shock. Her face has changed remarkably - she is now exhibiting the "cushingoid" syndrome, the rounding and puffiness of her face. The name for this effect comes from Dr. Harvey Cushing, a neurosurgeon who first used corticosteroids to control swelling in brain tumor patients. Maybe I never noticed the effect before because it came on so rapidly, but it is very obvious. I'm very glad she's not a teenager who would have to deal with the effects on her appearance in a very different way.
Wednesday, April 29
Today is Julia's first day with a reduced dose of Prednisone - 36mg instead of 40mg. She now takes quite the impressive array of pills in the morning - a multivitamin, a fluoride supplement, a Plaquenil, and a whole series of Prednisone - a 20mg tablet, 3 5mg tablets, and a 1mg tablet. I've read that reducing prednisone dosage can make you feel tired or worn out. She had both gym class and dance lessons today, and her mom said she was complaining about being tired.
|Current Drug Dose:
Thursday, April 30
Julia was definitely complaining more about being tired today. Despite this, she took her regular swimming lessons today and I got to watch her. She is doing extremely well, and has improved remarkably in the past several weeks -- even her instructor told me that tonight.
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