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Thursday, April 1

Today is April Fool's Day, but I'm not in a very joking mood this year. Today is also Holy Thursday, and it was extra special this year at our church. This is traditionally the time when the holy oils (holy orders, baptism, and the sick) are brought in each year. Our priest gave Julia the sacrament of the sick during mass, the first one to be anointed by the new oil. She went up to the altar all by herself. It was very special for her.

It's only been a day, but already Julia's skin where we've been putting on the Dovonex is a lot less rough. It's added a few minutes to our morning and evening routines, but it seems to be helping. The Elocon we've been putting on her face hasn't made any difference so far, but we'll give it the full week and see if it helps.

We were hoping that Julia's labs would be in by now, but I emailed Dr. A.W. and they're not.

Friday, April 2

Dr. A.W. called with Julia's results. We find ourselves pretty much in the same ballpark as last time - some numbers are down, others are up. The complete chart is in last month's page, but here are the numbers:

Aldolase 10.2 (up a little, but in the normal range), CK 293 (up), LDH (up, but still normal), AST 47 (up slightly), ALT 36 (down, but this was elevated last time because of a cold), albumin 4.0, WBC 5.7, hematocrit 35.9, platelets 352, and sed rate 9.0.

Dr. S. 1s out of the office until next Tuesday, so Dr. A.W. consulted wth a senior doctor at Brigham & Women's Hospital. Being fully off Prednisone, they expect the numbers to fluctuate. She emphasized that Julia is doing "extremely well ... exceptionally well" clinically. We have no cause, based on these results, to act hastily. Our options are the intermittent short course of oral Prednisone that we talked about (10-15mg/day for about 2 weeks, followed by a rapid taper), and IV Solumedrol (methylprednisolone). Solumedrol is about a 3-hour infusion of high dose steroids. She also said that it's likely that we will raise Julia's Methotrexate to 25mg/week.

We're basically not in an emeregency situation, and we're going to wait until next week to get Dr. S.'s opinion before changing her treatment.

Monday, April 5

I find myself on a business trip in California today, and I won't be back until the end of the day on Wednesday. So, naturally, plenty of stuff happened. I was helpess to participate, so Julia's mom handled this one.

Dr. S. came back a day earlier than expected. He had a look at Julia's lab results, and agreed that pulse IV steroids were called for. Her rash is bad, and her numbers are up, and we need to do something. We had to find a 4-hour block of time for this procedure. After some wrangling, we scheduled Julia for late tomorrow morning.

Some of the things that were discussed: dose, procedures, side effects, and follow up. The medicine that Julia will need is, indeed, called Solu-Medrol, generically known as Methylprednisolone. The standard dose for pulse infusion is 30mg per kg; at Julia's 33kg, her dose will be about 1000mg. She'll need to have an IV needle in her arm for up to 4 hours. Yes, we can use EMLA to help out. Some side effects that they watch for during and immediately after are elevated blood pressure (rare in kids; they monitor her throughout the procedure), and low heart rate ("extremely" rare; a heart monitor is used during the procedure). If indicated, they will monitor her for up to an hour after the procedure to make sure the vital signs remain normal.

Of course, everyone is nervous about this, but it feels good to move forward, to take steps. Oh yeah, this is Julia's last full day on Elocon, the steroid ointment for her face. It didn't do anything. She gets her last treatment of it tomorrow morning.

Tuesday, April 6

Many thanks to Julia's mom for taking such great notes for me in my absence. ("I'm not worthy!")

Julia was brought to the outpatient clinic at Children's for this treatment. Julia got to use a private room, with a bed, table & chairs, extra chairs for visitors, magazines, a sink, and a TV/VCR (they have a video library there, and they take donations!). Julia watched Indiana Jones and the Last Crusade. Very nice setup.

They measured her height and weight at the start of the procedure. She's at 127.8cm/50.5" in height (about the same) and 32.6kg/71.7lb. If you can imagine, Julia's lost another 2 pounds in the past week.

The plan is to give Julia 1000mg of Solu-Medrol (50cc) over 3 hours - 16.67ml/hour. The EMLA cream made the start of the procedure completely painless. At about noon, Dr. S, Dr. A.W., and (yet another) visiting doctor, Dr. P. from Beirut, came by to see Julia. They told Julia that she might get a metallic taste in her mouth (she did; Julia told me that it tasted like she was sucking on a penny, but a stick of spearmint gume helped), but it would go away soon after the procedure stopped. She would also feel very strong after it was over. They did a quick clinical exam, and they said her neck muscles already seemed stronger. They couldn't really explain why, though - the Solumedrol hasn't had time to take effect.

They said they want blood tests done in a week and in 2 weeks. That second test, though, will have to happen while we're on vacation. They will give us the name of a doctor we can contact while we're away in case we need to, and we can get the name of a clinic where we can get blood drawn. It'll probably be a battle with the insurance company, but, hey, that's life. Finally, they want to hold off on increasing the Methotrexate dose until they see what effect this treatment has. Dr. S.'s "one change at a time" philosophy.

At one point, Julia's hand started to fall asleep. A warm compress on that hand helped. Also, the infusion site on her arm began to hurt her a little bit, but she got through that. After the infusion was done, they stayed for about half an hour, then left. There were no complications or side effects. The nurse in charge was wonderful throughout. They gave Julia 3 tickets to the Big Apple Circus for a special benefit performance on Saturday. She even gave us a copy of her chart for the infusion.

Time Temp. Pulse Resp. BP Notes
11:00 36.5 103 24 126/69 Admittance vital signs
11:15         #22 needle placed in left arm
11:20         Solumedrol 1000mg/D5W 50cc started
11:35   87 22 134/63  
11:50   91 22 135/56  
12:05   94 22 134/64 Drs. S. & A.W. stop by
12:20   89 20 121/62  
12:30   91 20 123/64  
12:45   87 20 118/63 Lunch time
13:00   100 22 124/59  
13:15   93 22 119/56  
13:30   96 22 120/55  
13:45   102 22 126/59  
14:00   108 22 124/69 "Flush"
14:15   103 22 121/64  
14:30   101 20 111/56 Infusion complete
15:00 36.8 103 20 112/52 Release; no problems seen.

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

Friday, April 9

Julia definitely seems less tired the past couple of days, but her rash doesn't seem any better at all. This is very disappointing.

Saturday, April 10

Julia and her mom had a big day in Boston today. They went to the Boston Museum of Fine Arts to see the Mary Cassatt exhibit. They also saw some original works by Monet, Renoir, and Julia's favorite, Van Gogh (did I ever mention that Julia decorated a bathroom in our house in a Van Gogo motif?). They said it was a wonderful exhibit, and they had a good time there.

We also met for an afternoon performance of the Big Apple Circus; we got the tickets when Julia was at Children's on Tuesday. The BAC is involved at Children's with the Clown Care Unit that entertains kids who have to be at the hospital. We've seen them there several times when we in for office visits.

The circus is teriffic! We've been to BAC a few times previously, but they change the acts every year so it's always fresh. This was a special performance for "The Hole In The Wall Gang", which runs a camp for kids age 7 to 15 with cancer, sickle cell anemia, HIV, and other serious blood diseases. There was a wide variety of acts this year, including trained elephants, dogs, horses, and birds (!), some amazing acrobats and aerialists, and a simply dazzling tightwire act. No jugglers. :-( If you've been to BAC in the past, you probably remember the charming clown Grandma. Unfortunately, Grandma has been retired, and replaced by a new, totally different character who is very entertaining and funny. We had a wonderful time.

Sunday, April 11

Julia seems to have some poison ivy. She probably got it on Friday when she was playing in the woods at the sitters' house. It's on her side, just below her rib cage on the right hand side. It's a couple of ugly red spots that look like they want to blister, and Julia says it's very itchy. We put some over-the-counter cortisone cream and calamine lotion on it, but it took a while before the itching would subside.

Tuesday, April 13

Julia's poison ivy is still bothering her. We even had to give her Benadryl to fight the itching.

Julia had her first blood test following the Solumedrol infusion today. We probably won't get the results until the end of the week, as usual. She had this test done at our regular doctor's office. Julia's mom took her, and they ran into Dr. L., Julia's regular pediatrician. He commented that her rash looks worse than when he first saw her last year. Confirmation of what we already know, I guess.

We finally got the results from these tests - it took almost 2 weeks because they were sent to the wrong place at first. Grrr. The instructions were very clear, but were just not followed. I'm also going to truncate these charts to show just the most recent results. I may print a complete chart periodically, but I haven't decided yet.

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
3/30/99 Test: 10.2 293 290 47 36
Normal Range: 3.0-16.0 4-150 110-295 10-34 6-59
4/13/99 Test: ? 357 249 54 54
Normal Range: ? 24-146 95-180 10-40 10-40

Also, her WBC is 5.1, Hematocrit 35.6, and Platelets 298. The Normal ranges are different from the Children's Hospital lab's , so interpretation is somewhat difficult. Clearly, however, all the numbers continue to be up, and all are above the normal range. I'm also not sure why the Aldolase results weren't included, I'll have to ask about that.

Thursday, April 15

I emailed Dr. A.W. to see if the results were in yet. Nope. But she did say that the von Willebrand factor results from a couple of weeks are are in. They are normal - her score is 156, with a normal value of 150. I guess there's on thing we don't have to worry about.

Julia's face rash looks just awful today - maybe the worst I've ever seen it. It doesn't look like the name-brand Plaquenil nor the Solumedrol treatment has had any immediate effect on the rash. This is very disappointing. Also, her poison ivy on her side is looking worse, all swollen and it seems to be getting bigger.

Friday, April 16

Today, we head off on vacation. If you're the type who dreads looking at other people's vacation pictures, you may just want to skim my entries for the next week or so, because I'm going into some detail here. Julia has next week off from school (spring vacation), so we're off for 7 days and 8 nights in San Diego! Now, everyone wants to know "Why San Diego? Got family there?". I got asked this question more often than I expected. No, we were attracted to the climate (comfortable year round) and the stuff for families to do in the area - the San Diego Zoo, Sea World, and so on.

This was a travel day. Our ride to Logan Airport in Boston was harrowing - we allowed almost two and a half hours to get there, and only barely made it. Traffic was horrible, the worst I've ever seen that wasn't caused by an accident. Just sheer volume clogging things up. We had a layover in Denver for about 45 minutes. When we got there, it was 28 degrees and snowing! We managed to fly to California on time (about 11PM local time) without further hassle. We collected our rental car, a white Chrysler Sebring LXi convertible. I've always wanted to drive a convertible, and this was a great opportunity. Still, we were all exhausted after travelling and just wanted to get to sleep. Luckily, all the luggage fit in the car and the hotel was just a few minutes' drive away, in the beautiful and historic Old Town section of San Diego.

Saturday, April 17

We tried to sleep late today, but the three hour time difference made that hard, despite being up past midnight last night. Both Julia and I were up at around 6, and mom slept a couple of hours later. We unpacked, and tried to get ourselves going. Our hotel room was an efficiency/suite, with two rooms; a "living room" with a pullout single bed for Julia, and a bedroom with queen-sized bed. There was also a kitchen area with a sink, stovetop, coffee pot, and a microwave oven. This let us easily have breakfasts in our room, which saved us a lot of time and money.

We had a very pleasant lunch at the Cafe Coyote, which was literally right around the corner from the hotel. After lunch, we dropped the top on the convertible (nice!) and made a shopping run to Target and Ralph's for groceries and other stuff. We don't have either store chain on the east coast; Target reminds me of Wal-Mart, and my namesake Ralph's is pretty much like any other grocery store. After that, we shopped around in Old Town and at the Bazaar del Mundo.

We went to church at Immaculate Conception, also right around the corner from the hotel in Old Town. It's a historic church, one of the first in California. After mass, we went to supper at Casa de Pico, which is famous for it's huge margaritas. They are huge (we each had one), and they weren't bad.

Julia was amazingly perky most of the day, despite the lack of sleep. She fell asleep in church, and was a bit grumpy in the evening during dinner, but under the circumstances, she did great. Her rash doesn't look good, though. We're loaded up on SPF45 sunscreen, plus she wears a hat and sunglasses whenever we're out in the sun. Also, that poison ivy is starting to make her break out in little red bumps all over her arms.

Sunday, April 18

We took a ride north of San Diego to visit the Palomar Observatory. It was a beautiful day in San Diego, but inland in was hot, had to be in the 90's. The road leading to the Observatory is a wonderful driver's road, steep and winding, with stunning views of the valley below. At the top of the mountain (elevation 5550', according to the sign), we parked and checked out the museum (just some informational displays and videos about the observatory and telescope), then walked over to the telescope building. I've seen pictures of the telescope ever since I was a kid - I've always been an astronomy buff - but the puctures don't give you a sense of size and scale that seeing it in person does. They have a life-size model of the 200" telescope mirror in the museum - it's over 16 feet across. The telescope itself is a marvel of engineering. It rotates and tilts and can view any part of the sky. This telescope was built 50 years ago, and for decades was the biggest in the world.

One marvel about the ride out of the Palomar area was the smell. We drove through cirtus groves - acres and acres of orange, lemon, and grapefruit trees. Their smell is incredible! We stopped and bought some oranges on the side of the road. People sell them the way people in New England sell tomatoes in the fall. For some reason, we also noticed a lot of roadside selling of beef jerky. Couldn't figure that one out!

After an exhilirating drive down the mountain, we stopped for lunch, then headed to Wine Country in Temeculah. We visited the Callaway Vineyard. We got a tour of the winery, and also got some wine tasting. It was a lot of fun, but it was so hot!

After driving back to the hotel, Julia wanted to go swimming, so I took her to the hotel pool. It was small, but she loves to just splash around anyway. After swimming, she took a shower and her rash looked so bad, I couldn't believe it. Also, the poison ivy reaction is spreading to her chest and stomach. We thought maybe the rash was caused by the sun, but we've been fastidious about sunscreen, hat, and sunglasses, and we encouraged her to stand and walk in the shade whenever possible. We also speculated about a reaction to her sunscreen, but she was getting the bumps in places where she had no sunscreen, and some places with sunscreen were fine (namely, her face). She's also complaining about it being itchy, and it's woken her up at night. We're giving her benadryl at night, and that helps, but not she says it's itching her during the day.

Monday, April 19

This morning, we found that Julia had a patch of redness on the back of her neck. It looks like sunburn, but I'm certain I put a thick layer of SPF45 on that very spot yesterday. We put more sunscreen on the spot, and today we left her hair down to cover her neck.

Before heading out the door, we watched the start of the Boston Marathon - at 9:00 in the morning! That was very weird, we're used to seeing the start at noon. Today is the Patriot's Day holiday in Massachusetts, but *no one* here has ever heard of it.

We went to Sea World today. This is the easiest ride from Old Town, it takes only about 10 minutes to get to the park from the hotel. It was a lot of fun! We started with the live bird show. They had some beautiful birds to show, plus some trained birds of prey that were released from a blimp a few hundred feet above the stadium. They were incredible to watch swoop down from on high. They said that some birds can reach speeds of 180 MPH during an attach dive. Most prey doesn't even know what hit them.

We also ride the Wild Arctic motion ride, and say the Penguin exhibit afterwards. Julia got a hair wrap done, which is several strands of hair are wrapped by colored string and beads are attached to the end. Looks very nice; she's had this done before. I should learn how to do it.

We went to the Shamu show. This was quite entertaining. It is truly amazing what they can teach these huge creatures to do on command. After the show, we had a special lunch - "Dine with Shamu". They have a series of tables at tankside where the whales swim. The trainers come around and answer your questions, and later the whales swim right up next to the tables. We literally had a pair of killer whales less than 10 feet from where we were sitting! Even the food was pretty good. We made reservations in advance, and we got probably the best table in the place for seeing the whales. This is highly recommended if you ever visit Sea World.

After lunch, we saw the Dolphin show, fed the rays (they eat raw squid right out of your hand - yuck!), and just walked around and checked out the exhibits. Sea World here is very much like Sea World in Orlando, Florida, which we saw 3 years ago. One big difference - this park was remarkably empty. This is apparently not a school vacation week in California, and it was very quiet, especially compared to Sea World Orlando and our experiences at Disney World. All in all, this was a good fun day.

Tuesday, April 20

Julia's red-bump rash contimues to spread and make her itchy. We're giving her Benadryl during the day now when she complains about it itching. It definitely helps, but she doesn't like to take it. It makes her feel drowsy, which she doesn't like when there's fun stuff to see and do!

We started out today at Children's Hospital of San Diego. Julia needs another blood test, and the insurance people said we could get it done anywhere we wanted, so we got a contact name from our regular doctors and went here. This place is huge, but once we found out where we were supposed to be, it went great. I already have the numbers back from them, so here they are:

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
3/30/99 Test: 10.2 293 290 47 36
Normal Range: 3.0-16.0 4-150 110-295 10-34 6-59
4/13/99 Test: ? 357 249 54 54
Normal Range: ? 24-146 95-180 10-40 10-40
4/20/99 Test: ? 443 263 61 53
Normal Range: ? 40-275 100-250 21-36 10-35

Also, her WBC is 3.7, Hematocrit 36.8, and Platelets 292. Again, the Normal ranges are different from the Children's Hospital lab's. Taking the normals into account, the numbers continue to be up, and all are above the normal range. We'll have to see what they say at Boston Children's next week.

After the blood work, we went to Legoland California. This is a brand new theme park built around those famous, ubiquitous building blocks, Legos. This is only the third Lego theme park in the world, and the only one in the U.S. The others are in London, and in Denmark (the home country of the Lego company). There are way cool sculptures all over the park that are built entirely of Legos. We were told that none of the sculptures were made from cut, sanded, or painted Lego bricks - they used only the same product you can buy in the store (or in the gift shop on the way out of the park :).

The rest of the park was only okay - some of the themed areas were unremarkable, and the rides we tried were pretty lame. The thing is the sculptures. According to their web site, there are more than 5000 sculptures using over 30 Million bricks. On the boat ride (we went twice), they had Lego recreations of the Sydney Opera House, the Taj Mahal, the Eiffel Tower, a New England seashore, and the New York skyline. They also recreated a busy harbor, complete with vehicles, moving boats and trains, and a ship that sinks (and rises again) before your eyes. They have recreated several city settings, including New York (including an amazing reproduction of Grand Central Station), New Orleans (with a Mardi Gras parade), Washington D.C., and San Francisco. They also had a display of how scupltures are put together. They said that it took more that 67 man-years of effort to build all the displays in the park. The scuptures capture incredible detail, and they include animations of people and vehicles. We walked around and around the sculptures, just amazed by it all.

This was not a very tiring day, thankfully. It was sunny, but quite cool and breezy. Julia was rather tired throughout the day, and was walking frustratingly slowly most of the time (she could pick up the pace if she wanted to, though). She was up a couple of times last night because of the itching, so it's not unexpected that she's tired. Also, her JDMS rash as well as her poison ivy rash were both looking particularly bad today. We even had to deal with several well-meaning, but ignorant people who were asking about Julia's "sunburn". One little girl standing in line near Julia was staring and loudly asking her mother what was on Julia's face. Julia was unconfortable with this; I'm sure it reminded her of the time on the school bus last year.

Wednesday, April 21

Today, we went to the world-famous San Diego Zoo. It was a very cool day, no warmer than about 60 degrees, and overcast. It was very damp, and it was misty and even rained a few times. We were expecting better weather, but because of Julia's rash, we almost welcomed this weather. The sun and the heat always do a number on her skin. Julia was very tired today, even more than yesterday. She was walking very slowly again, and actually needed to be carries at times. Her skin was all broken out in the poison ivy rash, but the JDMS rash looked a little less severe.

What a huge zoo! There was so much to see. Some of the highlights included the tiger walk (including dozens of non-tiger species), the aviaries, the pandas (even though they were sleeping), elephants & rhinos, and the albino koala bear (along with the other koalas). Many sections of the zoo were closed for construction, which made getting around something of a pain. Still, there were many animals none of us has ever heard of before, much less seen. They had some very realistic habitats set up for the animals, and they are obviously very aware of the animals' needs.

Thursday, April 22

This day was just as cold and even more damp than yesterday. Today, we did an Old Town Trolley Tour of San Diego. It's a 2-hour loop tour, and you can stop and get off and on whenever you like. I was a little skeptical of this - I thought they were just going to shuttle us around the city. It turned out to be a lot more interesting than that. The trolley (it's a bus, really) drivers were very well informed about the city and all the interesting little stories about the places and things in it. Of course, it's impossible to verify what they were saying, but it was fun to listen and see everything.

We stopped at Seaport Village, a collection of little shops and restaurants right on the ocean. There were some interesting shops there, and it was a pleasant place to spend a little time. Unfortunately, the weather was raw and it rained enough to make things unconfortable. We had lunch at a very nice restaurant called The Fish Market, which was just a 5 minute walk from Seaport Village (once we found it). They had a nice view of the ocean, and excellent food.

Our next stop was some shopping at Horton Plaza. This is an open-air mall, but the architecture is unlike anything else I've ever seen. There seem to be elements of several different styles, but they work together very well. The shops wrap around and twist through a giant structure. It's very hard to describe, but it's a lot of fun to walk around it. They have most of the same stores you'll find in just about any mall in America, but the layout makes it different and interesting.

We didn't make any more stops after getting on the Trolley, but we got to see the rest of the city. Most notable is Coronado Island, with some beautiful real estate and some stunning hotels.

We stopped by the grocery store and just ate supper in our room today. Julia seemed to be feeling a little better, less tired, and her poison ivy rash is starting to actually look better. Her face didn't even look as red today. We didn't do a ton of waking around, so she got a chance to take it easy for a change. We needed a "down" day after seeing and doing so much up to now.

Friday, April 23

This is our last full day in San Diego, and it was a full day indeed. We got an early start and headed up to Anaheim to visit -- Disneyland, the self-proclaimed Happiest Place on Earth. We were blessed with a cool, cloudy morning, thus keeping Juila's rashes under better control. Her rashes continued to improve - the poison ivy rash has subsided further, and the JDMS rash looks marginally better, too. She was still walking around very slowly and seemed somewhat tired.

It was fairly crowded, and more and more so as the day wore on. We've been to Disney World in Florida (twice). To compare, Disneyland is very similar to DW's Magic Kingdom. I'm told that DL is building Epcot Center West.

All we did all day was basically wait in line for a ride, then ride the ride. We went on the Star Tours motion ride, a new ride called Rocket Rods (good ride, but an hour wait :( ), then had lunch in Toon Town (think Roger Rabbit). After lunch, the weather cleared up, the sun came out, and it got a lot warmer. We went on Mr. Toad's Wild Ride, Big Thunder Mountain, the Haunted Mansion, Splash Mountain, and the Indiana Jones Adventure ride. The Indy Jones ride, the last one we rode, was the best.

A couple of days ago, we received a most generous invitation to visit with a family who lives near Los Angeles. (In fact, they arranged for us to get free passes to Disneyland :) They contacted us because their 10YO son, Alex, was recently diagnosed with JDMS after a series of incorrect diagnoses. We decided to take them up on it, and we went to their home for dinner after Disneyland. Julia in particular was looking forward to meeting Alex, as we'd never met anyone else who has JDMS. Marcia & Bob and their three sons live on the pacific coast near Long Beach, and their house has one of the most spectacular, stunning views I've ever seen. We witnessed a magnificant sunset. It's also cool to walk out to the backyard when you need a couple of lemons to cook with and just pick 'em off a tree.

The evening went very well; we only left because we had a 2+ hour drive back to San Diego, and we have to get up early tomorrow to pack and catch a flight home. Marcia and Bob were gracious hosts, the food was great and the company was better. They showered Julia with gifts, and she and Alex hit it off very well and played together for hours - computer, video games, and just general mischief around the house ;-). We were very sorry to see the evening end. We had a delightful time. Thanks to Bob, Marcia, and their whole family!

Monday, April 26

Julia was extremely cranky getting up this morning. I think she's suffering jet lag - her body thinks it's only 5 PM at bedtime, and that it's 3 AM when it's time to get up in the morning! She had a really hard time with it today.

We got Julia's First Communion formal photos taken today. She wore a dress that her mother made (just finished it yesterday :) and a circlet. It's simple and elegant and quite beautiful. Julia's rash is still quite pronounced, but she was adamant about not having any makeup to cover it up. I think this is a very healthy attitude. I will scan and post a photo when they come back in about a month.

First Communion - click for full size
Here it is

Tuesday, April 27

Another trip to Children's Hospital for a checkup today. Vital stats: Weight - 32.3kg./71.2lb., lost another 2 pounds! This is getting close to her normal weight for her height. Height - 50.5"/128cm, unchanged. Blood Pressure was measured twice, not sure why - 120/59 and 117/57. Temperature - 97.9°F/36.6°C.

Typical clinical exam, first with Dr. A.W. then with Dr. S. and his entourage (two visiting doctors, Dr. D.A., a resident, and Dr. D.H., from Albany). He noted that Julia's neck muscles are still a bit weak, and she was having a bit of trouble doing sit-ups. Dr. S was quite funny today - mostly he talked directly to Julia, asking her all the questions (plenty about San Diego), getting her opinion on what we should do with treatment options, etc.

Her tiredness on vacation was probably a sign of her disease - fatigue and tiredness are the earliest symptoms. Dr. S. is aware that Julia is receiving her first communion on Saturday, and presented us with options to help her look and feel better. He had explained earlier that they will adjust medication to allow patients to be at their best for vacations or other special events like this one, but we'd talked about it, and we feel pretty much that unnecessarily aggressive treatment for something like that is foolish. The first option that Dr. S. gave us was to give Julia 2 or 3 consecutive days of IV Solumedrol treatment, probably starting tomorrow. Although her rash was unaffected by the first treatment 3 weeks ago, the consecutive treatments would probably help, he thought. We rejected this immediately as too aggressive and intrusive. Julia is actually okay with having a rash for her first communion, and so am I - it's who and where she is right now. Also, since we had the formal photos taken already, the appearance issue is no longer a big deal.

The other option Dr. S. gave us was short-term, high dose oral Prednisone with a rapid taper. This has several advantages; The high dose has been somewhat effective against her rash in the past, it certainly helps her strength and energy levels, and the short duration eliminates most of the bad side effects. In conjunction with this, he also wants to increase her Methotrexate dose from 22.5mg/week to 25mg/week. He said that her MTX dose is currently relatively low, and since we've had no serious side effects from it (some tiredness a few hours after her dose that was fixed by moving the dose time from first thing in the morning to late in the afternoon, and some soreness in her mouth that was solved with folic acid supplements) the modest increase should not be a problem.

We decided to go with the second option. The regimen is this: 20mg Prednisone twice per day (20mg today, then 40mg/day) through Friday, then 20mg/day through next Tuesday, then we call in and discuss how she has reacted. Tapering expected to only take another week or so. Dr. S. feels that this will moderate her rash , and give her a much needed boost in energy. He assured us (again) that Julia is doing extremely well clinically, despite the rash being a constant reminder of her disease. He also said that until JDMS goes into permanent remission, it is perfectly normal and expected to see ups and downs. That's why we monitor and adjust her treatment to compensate. They want to see us again in 3 or 4 weeks.

Our town swimming pool has been closed for renovations, and Julia hasn't has swimming lessons for almost a year. The pool reopened a couple of weeks ago, and she started a new round of lessons today. I stayed during the lesson and watched from the balcony. I thought she was doing pretty well, better even than some of the other kids. She was definitely having trouble getting out of the pool, though - she didn't seem to have the arm strength to pull herself all the way out. She compensated by propping herself on her elbows, then swinging her legs out to the side and basically crawling out of the water. Aside from that, I thought she was doing very well. However, after the lesson, she started crying, then sobbing pretty much all the way home. She felt that she couldn't do anything right, beating herself up because the instructors were correcting her stroke form. We tried to tell her that the instructors were just doing their jobs, teaching the kids the right form to use, and I told her that she did actually do very well, but she wouldn't have any of it. It was sad to see her like that.

Current Drug Dose: Prednisone: 20mg/day Plaquenil: 200mg/day Folic Acid: 1mg/day Methotrexate: 25mg/week

Okay, just got the blood test results emailed to me by Dr. A.W. Here they are:

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
3/30/99 Test: 10.2 293 290 47 36
Normal Range: 3.0-16.0 4-150 110-295 10-34 6-59
4/13/99 Test: ? 357 249 54 54
Normal Range: ? 24-146 95-180 10-40 10-40
4/20/99 Test: ? 443 263 61 53
Normal Range: ? 40-275 100-250 21-36 10-35
4/27/99 Test: 11.4 413 348 68 69
Normal Range: 3.0-16.0 4-150 110-295 10-34 6-59

In addition, the other results are; WBC 3.95, Hematocrit 37.7, Platelets 333, and Sedimentation Rate 10. Basically, not very different from last week. These just confirm that we're making the right move with the prednisone and the increase in methotrexate.

Wednesday, April 28

Julia woke up today with a lot more energy and perkiness than she's had since we got home. Good to see. I think it's a combination of finally getting adjusted to Eastern Time, and taking Prednisone last night. Unfortunately, she was also very sensitive - I just reminded her to take her meds like I do every morning, and she snapped at me. I guess it's to be expected.

Current Drug Dose: Prednisone: 40mg/day Plaquenil: 200mg/day Folic Acid: 1mg/day Methotrexate: 25mg/week

Thursday, April 29

Julia had swimming again tonight. Before the lesson, she asked me to speak to the lead instructor. I explained basically about JDMS, and it's effects on her strength and endurance. I also said that explains her rash, and that it's not contagious. I also said that we did not expect them to hold Julia to an inferior standard, and that she's have to do everything anyone else would have to do to complete the level she's at. He seemed very understanding about it, and promised to explain it to the other instructors. I didn't want to explain this, but Julia insisted.

In the actual lesson, she did *much* better! She felt like she had a lot more energy, and she was able to get out of the pool with much less effort. She said she had a lot more endurance, too. We always marvel about how well the Prednisone works for her, at least at first before the side effects set in.

Overall today, Julia Julia had a lot more energy, but was also a lot more easily upset. She was moody, but not too bad. Her appetite seems quite normal, so far. Her rash even seems a little less angry today. She knows that the 40mg/day dose is just for three days, and I think that's helping her cope.

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Updated June 7, 1999
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