Back to December    

Wednesday, January 1

Happy New Year! 2002 was a very good year for us and for Julia's health, broken bones aside. She came off of all medicine for the first time in over 4 years, and has had 100% strength, normal blood work, and a rash that is now all but undetectable. I don't think it is premature to say that Julia is probably in remission and we hope and pray every day that it will stay that way.

As of 2003, the Diary is switching from a Monthly page format. This is because there is little of medical interest going on in Julia's life (which is a very good thing!). The new format will be quarterly, semi-annually, or annually depending on the amount of content.

Sunday, January 12

Julia developed a mild sore throat today but otherwise was feeling OK. We'll keep an eye on it.

Tuesday, January 14

Julia's sore throat was worse today, bad enough that we decided to keep her home. She said that it was pretty bad in the morning when she got up, then got better during the day, and worsened again late into the afternoon and into the evening. She does not have a fever, which is good, but we're a bit worried that this is hanging on like this.

I should also not that we're having a bit of a cold snap here, with nighttime temperatures in the single-digits Fahrenheit and daytime highs in the 20's. We didn't have our humidifiers going until today, and I'm sure that didn't help Julia's throat.

Wednesday, January 15

Julia was feeling better, but we ended up taking her to the doctor today. They didn't think it was strep but gave her a throat culture just in case.

Thursday, January 16

Julia woke up this morning with her right eye all crusty and red. Not good. She felt better overall and her throat was also better, but the eye is worrisome. We placed a call to the doctor who diagnosed Conjuntivitis (Inflammation of the conjuntiva, the white of the eye)) over the phone. Conjuntivitis is extremely infectious until antibiotics have been administered for at least 24 hours, so she will miss school for the rest of this week. We also have to be careful about hand washing, eye touching, etc. for that period. None of the rest of us wants this.

Saturday, January 18

Well, after 36 hours of using antibiotic eye drops, Julia's eyes are finally looking better. She says this morning that her sore throat is a little worse today, but it's not as bad as it was a couple of days ago.

Monday, January 20

Julia's eyes are almost returned to normal, but she doesn't have school today anyway because of the Martin Luther King holiday. She will be going back tomorrow. Luckily, neither myself nor Julia's mom got this.

Monday, January 27

Julia has another minor cold, just a bit of congestion. Not enough to keep her out of school.

Tuesday, January 28

Today we took a trip into Boston to visit the Endocrinology department. This was my first visit there. It looks amazingly similar to the Rheumatology department one floor higher in the same building.

We got there about 30 minutes early, and they actually took us soon after we arrived! Julia is now 4' 9.5" (146 cm) tall and weighs 103.5 lbs (47 kg). Her blood pressure read high at 135/88 so they retook it and it read a more reasonable 136/66. Go figure.

I met Dr. M. for the first time and he seemed very personable. He asked how Julia has been doing since her last visit. The answer is generally very well, with the usual array of kid diseases (colds) and injuries (cuts, bruises, and of course a broken leg!). He asked about her development, ad everything was normal. He then did a physical exam that just took a couple of minutes.

He said that based on the records, Julia has brown 8cm (over 3") in less than a year. This is growth beyond what they would normally expect from a pre-pubescent girl and he seemed very pleased with this. His conclusion is that all or nearly all this growth beyond what they would normally expect is probably because Julia is off of Prednisone, and not because she is approaching puberty. This is good news.

Afterwards, a resident, Dr. K., came in and basically rehashed the same information. He then asked how tall I was (6'3") and how tall Julia's mom is (5'3") and said that based on a formula they use, a girl with us as parents would normally expect to be 5'7" tall. The prediction for Julia is 5'3", with the missing height caused by 4 years of steroid use. Dr. K. said that most or all of that 4" will never be made up, but given that Julia is growing ahead of expectations, she may exceed their best guess by an inch or two.

They also gave us the good news that they don't need to see Julia again! They said they would be available if we needed a consultation in the future, or if Julia (God forbid) should even need to go back on Prednisone. Dr. K. talked about experimental therapy with growth hormones on kids who have to take steroids while undergoing puberty. We hope we never have to worry about that.

We got out of Children's with plenty of time to spare! Even though we had a 4:00 appointment in Boston, we easily made it home in time for Julia to get to dance practice at 6:00.

Monday, February 3

Julia stayed home from school today. She was a bad cold, as it seems does most of her classmates. Julia is feeling very tired and has the usual sneezing, congestion, etc.

Tuesday, February 4

Still home from school. We heard that many of her classmates were also out sick. Julia was feeling better toward the end of today, and indicated optimism that she could go back to school tomorrow.

Wednesday, February 5

Julia did go back to school today, but it being gym day, we admonished her to take things easy. She said it was a light gym class and the teacher didn't push anyone.

Friday, February 7

Julia was scheduled to have a sleepover party party tonight. This was to be an early birthday party (the 17th is her birthday). However, starting yesterday, the bad news started rolling in. All the girls she was planning to invite were sick! We had to cancel the party. Julia was devastated - she had her activities and decorations all planned out, and now it wasn't happening.

Thursday, February 13

We have been warned for years that Julia may need braces to correct a couple of minor problems with her teeth. Well, today was day one of the up to five year journey with braces. She got some spacers put in between a couple of teeth to make room for the braces. Julia was complaining about some soreness and pressure, but a little Tylenol seemed to help her.

Friday, February 14

Julia's school had today off. Julia has been styding Egypt in school, and did a report on Mummification, and has been wanting to see the ancient Egypt exhibit at the Museum of Science in Boston. We took a vacation day and did just that!

This was a bitterly cold day in New England - the temperature was hovering just above 0°F when we left the house. We took the train in to Boston instead of driving. With the Big Dig in full swing, traversing the city and parking are a big challenge.

We arrived at the museum just as it opened at 9:00. We started right in with The Quest for Immortality, the collection of ancient artifacts. We also bought the audio tour, like we did when we saw the Van Gogh exhibit at the MFA a couple of years ago. These audio tours are well worth the extra cost. You gain a lot of background information and perspective that you wouldn't get otherwise. The Q4I audio tour was narrated by the actor Jeremy Irons, with help from various experts in the field of Egyptian archeology. I was frankly expecting the exhibit to be dry and boring, but it actually was fascinating. Highly recommended, if you get a chance to see the exhibit before it leaves Boston at the end of March.

At one point, someone from the museum rolled out a black stone tablet. About 25 people were watching and he asked if anyone knew what it was. No one said anything for a moment, then Julia said "It's the Rosetta Stone". Of course, she was right (it was actually a replica). She also seemed to know more about the Mummification process than the woman describing it for the visitors. She's pretty amazing.

After the exhibit, we went to the Hayden Planetarium at the museum and saw the show Stars of the Pharaohs. This show explained some of the Egyptian mythology and how important the stars were to them. Again, this was much more interesting than I expected.

To finish off our Egyptian trifecta, we took in the Mysteries of Egypt show at the Omni Theatre. If you've never been to an Omni theatre, it's worth it. The screen is a dome 50 feet high and wraps around you. The images are enoumous! It is a very immersive experience - you can really get lost in the panoramic vistas on the screen. Anyway, this feature tells some of the stories and explains some of the mysteries surrounding the Egyptian pyramids.

We spent the rest of the day in the regular museum exhibits. We haven't been here in a few years, and there were lots of new exhibits. We had to get us early this morning, and we were on our feet for many hours today, and we were all pretty tired by the end of the afternoon. We ended up staying for dinner at the museum - they transformed the snack area into a restaurant and served light meals with drinks. We were able to enjoy a nice meal and still catch a convenient train out of the city. This was a long, enjoyable day, and Julia seemed to have more energy than her parents!

Monday, February 17

Today is Julia's 12th birthday!! Happy birthday, kiddo.

Today was also the start of a historic snow storm here in New England. I started snowing late in the morning and it snowed for almost 24 straight hours! We ended up getting almost 2 feet of snow at our house, and in Boston they set a 24-hour record of 27.5"! This is relatively light, fluffy snow, but that also means that it's drifting, eaving both bare spots and huge drifts. The worst thing is that we've been getting a lot of snow this winter, and it's been extremely cold, so nothing has been melting. There's no place to put the snow any more! We have piles in front of our house that are 6 feet high, and the plain snow cover is about 4 feet. Just amazing.

Tuesday, February 18

Julia was supposed to have a appointment with Dr. A.W. today at Children's Hospital, but they called yesterday afternoon and said they were cancelling all appointments for today. We haven't seen them for 4 months! I guess that's a good thing.

Thursday, February 20

Julia is 12 now, and that means she's scheduled for some required immunizations. She needs to have a series of Hepatitis shots (one now, one in a month, and the last in the summer), and also Tetanus and others. We emailed Dr. A.W. beforehand to make sure these were OK to give to Julia. We are aware that immunizations can be a trigger for JDMS, so we wanted to check with them first.

Friday, February 21

Today was the day of Julia's rescheduled birthday party! Because this is school vacation week, we were worried that some of the kids she wanted to invite couldn't come, but only 1 (of 4) could not be here. Julia was psyched for this party, and she's been preparing all week.

First of all, the theme of the party is RED. We decorated our family room with strings of red lights, a red bulb for the lamp, red balloons, all Julia's red Beanie Babies, a red tablecloth, red cups and paper plates and plasticware, red crepe paper streamers to cover the doorway, red food (red peppers, pizza, etc), and everyone had to wear red. You get the idea.

They listened to music, Julia opened her birthday gifts, they played Twister. Of course, they stayed up until the wee hours. The girls had a great time!

Thursday, February 27

Today, Julia got her braces installed on her upper teeth. At the appointment, they kept her waiting and then it took them a long time to get them installed, but she now has them. They seem to be anchored to molars in the back of her mouth, and then cemented to each tooth around the front. They gave her a whole packet of information (food to avoid, daily care instructions, etc.) and a care kit (toothbrush, mouth rinse, floss & threaders, etc.).

By the end of the day, she was in a lot of discomfort. She described it as pressure, and we were told to expect this. We gave her ibuprofen for it. Two 200mg tablets worked wonders for her for several hours at at time.

Friday, February 28

Julia had a lot of trouble sleeping last night because of the discomfort the braces are causing.

Saturday, March 1

We took the initiative and brought Julia in for her blood work today, even though we haven't heard back from Dr. A.W. yet. She was due for this; overdue, in fact, because of the big snow storm.

We also used our very last tube of EMLA today. EMLA, for those who do not know, is a local anesthetic that we apply to the site of the blood draw on Julia's arm. It deadens the site and makes it a little less painful for her. We have a prescription for more, but our pharmacy has been unable to fillit for more than 2 months. We wrote Dr. A.W. an email today and told her that we came in for blood work, and to ask about EMLA.

Test Date	Aldolase	CK	LDH	AST	ALT	vW F8
1/19/02	5.3	102	198	21	12	193
2/16/02	5.0	202	230	24	11	100
3/16/02	5.5	101	200	21	14	126
4/23/02	5.9	143	466	49	15	115
5/14/02	5.3	86	197	25	20	110
6/29/02	4.0	92	214	27	22	111
7/25/02	4.8	107	232	25	25	103
9/7/02	7.9	99	199	22	15	109
10/22/02	7.8	122	207	24	15	143
12/14/02	6.6	129	181	19	16	not yet
3/1/03	4.3	91	164	19	8	91
Normal Range:	3.0-16.0	4-150	110-295	10-34	6-30	160

Sunday, March 2

We got email back from Dr. A.W. today. She got us the preliminary results from yesterday's blood work. The raw results we have so far are: AST 19, ALT 8, CK 91, LDH 164, WBC 6.25, hematocrit 37.0, platelets 387, sedimentation rate 9. These all look great!

She also apologized about the problem getting EMLA. It's actually a problem nationwide. The FDA is now requiring the manufacturer of EMLA to use child-proof packaging, and won't allow it to be distributed for home use until those changes are made. She said that there is a small amount of EMLA around the hospital for use for the patients who are here, but they are specifically prohibited from giving any to families for home use. The alternative is an over-the-counter version (elmax?), but insurance usually doesn't cover it because it. She said that the EMLA issue may be resolved by April, so we're keeping our fingers crossed.

Wednesday, March 5

Dr. A.W. sent us the rest of our blood work: Aldolase, 4.2, and von Willebrands, 91. Both are excellent! This means Julia won't be having blood work until May, which is almost 3 months from now.

Thursday, March 6

Today is the fifth anniversary of our Julia's diagnosis of JDMS. It's a weird kind of day - not one to celebrate, but certainly one to commemorate.

Today, medically and in every practical way, Julia is doing wonderfully. She is symptom-free - her strength is 100% and she has no sign of any JDMS rash. She has been medicine-free for nearly a year. She's an excellent student and has been accepted at a wonderful new school for next school year. For all practical purposes, she is in remission and we pray every day that it is permanent.

It's really hard to say whether it seems like longer or shorter than 5 years. In some ways, the time has flown, like with everything else in our busy lives. In other ways, I look back and I can't believe how much we've done in the past 5 years - there's no way we could have scheduled everything that happened!

Through it all, we not only survived, but thrived. Julia has 5 years of hard experience that will no doubt serve her well in life. She already shows maturity beyond her years in many ways. We are closer as a family than we would have been without JDMS. I would never wish the experience on anyone, but it has not been entirely negative for us.

Saturday, March 22

It's another milestone day for us. When we started our journey with JDMS, I recall one of our doctors telling us that the "official" definition of Permanent Remission is:

- No clinical symptoms (rash or muscle weakness)
- No abnormal blood results
- No medicine being taken

for one year. I don't know if there even is an "offical" definition for this state, but we clung to it. Julia achieved this state today. This marked one year since Julia took any medicine. She is now asymptomatic and her latest blood work results were the best ever. We are thrilled to be here and Julia stands as a living testament to patience and persistence. Hang in there - most JDMS kids eventually get here.

Sunday, March 30

Julia started in with some occasional coughing and sniffles yesterday, and she had a pretty hard time sleeping last night. Today, it's a full-blown cold. We have her on docongestants, but she's pretty wiped out. All we did today was go to a movie (The Core; it's OK as long as you check your brain at the door) and then stayed at home.

Monday, March 31

Julia slept poorly again last night, so she decided to stay home from school. She got steadily better as the day progressed, and she did manage to get some rest. By the evening, she was feeling pretty close to normal. I'm still amazed every time I see how fast she recovers from these things that always seem to wipe me out for a week.

Friday, April 11

Julia went in for another adjustment to her braces yesterday, and sure enough, she was having significant discomfort with them today.  A coupe of ibuprofen every several hours, and at bedtime, took the worst of the edge off for her.

Monday, April 14

Julia had an eye exam today, and all is well.  Her prescription remains the same, which is good.  I remember at her age needing a stronger prescription to correct myopia every time I had my eyes examined.  It's good to see her not needing it.

Wednesday, April 30

Julia has had herself in a frenzy of worry this week leading up to today.  She is taking exams to see if she can be in the academic honors program at her new school this fall.  I swear, Juliais going to give herself an ulcer before she graduates from high school!

Saturday, May 3

Julia will be graduating from hew school in a few weeks, and for her graduation present, we decided to "surprise" her with a trip to Walt Disney World.  At first, we wanted to really surprise her by telling her only the night before we left, but decided instead to let her savor the anticipation as well as the trip itself.  She did a great job planning our trip - we equipped her with a couple of books (PassPorter and Fodor's) and she picked our itinerary.

We had a flight that left at (gasp) 7:00 AM.  That meant we had to be at the airport by 5:00 to allow time for extra security procedures, so we left our house at a little after 4:00 AM.  THis would not normally be a problem, but we had a meeting the night before - that lasted until 1:00AM!  It was going to be a long day.

Our flight left on time and arrived in Orlando without incident.  We here in New England have been suffering with a brutally cold and snowy winter, followed by a cold and wet spring.  It was infinitely refreshing to arrive in Orlando to some actual heat!  We landed around 10:00 and it was already in the 80's.  Heaven!

We had a limo bring us from the airport to our resort, the Coronado Springs. This is one of the newest resorts on the Disney grounds, and it's got a southwest theme.  It's a beautiful resort!  We checked in, but because it was so early, our room wasn't ready so we left our baggage with the valet desk and headed to the parks.

First stop: Epcot!  We are lucky enough to be here during the annual Flower & Garden Festival which beautifies the park with flowers and topiary galore, and provides many digital photo opportunities:

In the World Showcase, which we all love to spend time in, we saw a fife and drum corps play traditional patriotic songs and do precision marching.

By the end of the day, we found ourselves in "The Living Seas", an aquarium and undersea life display, so we could cool off.  After spending some time there, we decided to have an early dinner and go to bed early.  We ate at The Coral Reef, a restaurant with one wall that is actually one of the giant sea life tanks at The Living Seas!  It makes for a great atmosphere and it's fun to watch the fish and the divers swimming by.  Oh, and the food was great, too!

After dinner, we headed to our hotel to unpack and get to bed early!

Sunday, May 4

Off to Disney-MGM Studios today.  We rode the rides, saw the Indiana Jones show, and ate lunch at the 50's Prime Time Cafe.  We went on the Backlot Tour and a bunch of other attractions.  In the evening, we went to Downtown Disney, and after a bite to eat, Julia's mom went shopping while Julia & I went to Disney Quest, a giant indoor arcade.

Monday, May 5

It was early-opening day at Animal Kingdom, so we got up early at arrived before the gates opened.  We headed off to the Kilimanjaro Safaris first thing, since Julia's research shows that there are more active animals before the heat of the day sets in.  That certainly seemed true today!  We saw rhinos, hippos, alligators, gazelles, giraffes, elephants, flamingos, lions, warthogs, and many oher animals and birds.  At one point, our tour bus was stopped for about 10 minutes, and we had several giraffes within a few yards of our bus!

After the safari, we went on the Pangani Forest Exploration Trail, which has many exotic animals on display.  Then, as the day grew hotter, we went on the Kali River Rapids, where Julia & her mom got soaked, and I only got a somewhat wet.

By mid-afternoon, we saw everything we wanted to see at AK and headed over to the Magic Kingdom.  Julia & I went over to Tom Sawyer's Island, which is a real oasis from the crowds.  You can explore caves and a fort and we also fed the ducks, which became a theme for this vacation.  Every time Julia would see ducks, she's feed them!  Go figure.

Tuesday, May 6

Back to our favorite park, Epcot.  We rode Test Track, which is a great ride, very fast, and took in the Universe of Energy, which is interesting and entertaining no matter how many times I see it.  We checked out some more flower and topiary displays, then headed back to the World Showcase.  I also saw my first Segway HT, being used by a cast member to sell water sprayer/fans. 

We ended our day with a trip to Downtown Disney for dinner and shopping, and then to Pleasure Island and the Adventurer's Club.

Wendesday, May 7

We spent the day at Blizzard Beach, a water park with an interesting theme.  Lore has it that a freak anowstorm blanketed the area one day, and the idea of a snow-themed park was born!  BB has a river that circumnavigates the park, plus water toboggan rides, water slides, and a giant pool with an artifical wave generator.  It was a beautiful, hot day and we had a great time!

Thursday, May 8

We went back to Animal Kingdom to catch some of the attractions we missed the other day.  We watched the Bug show at the Tree of Life, and took the train to Rafiki's Planet Watch.  We ate lunch at the Rainforest Cafe, then took in the bird demonstration.  The birds are well trained and pretty amazing!

In the afternoon, we went back to the Coronado Springs, and took a boat ride.  They rent paddle boats that you can paddle around the central lake of the resort.  The boat is covered, so we didn't have to ride out in the hot sun, and of course Julia fed the ducks.

That night, we headed back to Epcot to watch Illuminations, the big nightly fireworks show.  We had dinner at the Mexico pavillion and grabbed a good spot to watch the fireworks.  This show is amazing!  It's choreographed with motion, music, lasters, and of course incendiary devices.  Great show and a good vantage point.

Friday, May 9

This is our last full day at WDW!  We spent most of the day at Disney-MGM Studios. Julia & I roamed around the park while her mom got her hair done back at the Coronado. We met and had lunch at the Sci-Fi Dine In Theatre, which is like a drive-in movie theate indoors.  They even have a giant movie screen that displays clips form campy old sci-fi movies.  The food is pretty good here and the atmosphere is a lot of fun.

In the afternoon, we went to Who Wants to be a Millionaire -- Play It! based on the TV show (which I've never seen).  It's some sort of trivia game.  The contestants they pulled out of the audiance didn't do too well.

Finally, we headed back to Epcot to check out some of the Innoventions exhibits we'd missed earlier. 

Saturday, May 10

All good things must come to an end, and our time here at Disney is no exception.  We packed our stuff and checked out of the hotel, then headed out to Magic Kingdom for a while. We did Space Mountain, the Buzz Lightyear ride and a spin on ExtraTERRORestrial Alien Encounter.  We took a ride around Tom Sawyer Island on the Liberty Square Riverboat.  We rode Big Thunder Mountain and Splash Mountain.

By late morning, the park was very crowded.  This was the dirst day we could say that - up until today, the parks were all relatively enpty and the lines very short.  But after all, this was a Saturday and in fact the day before Mother's Day.  We left in the early afternoon to take a shower, to relax at our hotel for a while, and wait for our ride to the airport.

Overall, we had a great vacation, and as always we were extremely careful about Julia's sun exposure.  We all used a heavy application of sunscreen every morning, and Julia at least reapplied at least once or twice during each day.  All the dilligence paid off - Julia did not get any sunburn at all despite being out in the Florida sun all day, every day.

Thursday, May 15

Julia had another appointment to get her braces adjusted.  As usual, she had fairly significant discomfort today, and needed ibuprofen to help her get some sleep.

Tuesday, May 20

Julia had a staggeringly routine visit to Children's today.  She had blood drawn, and the results are below.  We are moving to a blood test every 3 months, the next one in August, and a clinic visit ever 6 months. 

Dr. A.W. also asked us about Julia participating in an Endicrinology study into the effects of long-term Prednisone use on bone density.  Sounds like Julia would be a candidate!  We have always been willing to help out with studies, and this is no exception.  We told her to go ahead and submit Julia's medical information for consideration.

Here are the rest of the blood test numbers not in the chart: Sedimentation rate 9, WBC 5.9, Hematocrit 37.4, Platelets 414, BUN 11, Creatinine 0.5.

Tuesday, June 10

This is Julia's graduation day from the only school she's ever attended.  The school put on a beautiful ceremony.  Julia participated as part of the Religion Club, and she also did a reading.

Congratulations Julia!

Wednesday, June 18

Julia got a clean bill of oral health from her dentist today.  They even complimented her on what a good job she's doing brushing and flossing with the braces!  Good job, Julia.

Thursday, June 19

Another trip to the ortodontist, and we're ready for another couple of days with Julia in a bad mood because her mouth hurts. 

Tuesday, July 15

After several phone calls & emails back & forth, we finally arranged to get Julia evaluated for the bone density study, and today was the visit to Children's.  Julia met with Dr. G. and Nurse C. The official title of the study is "Double Blind Controlled Trial of Alendronate for the Treatment of Children and Adolescent Glucocorticoid-associated Osteopenia and Osteoporosis".  Alendronate is better known as Fosamax, a drug that is supposed to help reverse bone loss.  This is a pretty widely used  and studied drug in adults, and although it is prescribed to children, its efficacy and safety have not been studied in pre-adults.  Until now.  This first visit is an evaluation to see if Julia is eligible for the study. 

The nurse and the doctor spent a lot ot time explaining the study and how it would affect Julia.  Part of this process included an 8-page parental consent form, a 2-page assent form for Julia, and a 3-page HIPAA form.  The consent form is very detailed and explains a lot about the study.  Here is a summary:

The study is being conducted at 5 sites across the US with about 90 children being tested, incuding 15-20 at Children's.  As implied by the title, this is a study with two groups - a treatment group and a control group.  Everything is the same for both groups - test, visits, evaluations, etc - except the treatment group gets the Fosamax, and the controls get a placebo.  "Double-blind" means that neither the patients nor the doctors know who is getting the drug and who is getting the placebo.  Assignment to one group or the other is random, so we don't know if Julia will be getting Fosamax or not until the study is over.

The form then goes into exactly what tests will be administered at which visits to the clinic.  There are scheduled to be 11 visits total over the next 2.5 years.  It also says that they will be giving each child daily Calcium plus Vitamin D supplements daily, and the Fosamax or plcebo weekly, for 18 months, then Vitamin D alone for 12 months.

First, they took an history and did a physical exam.  Then they drew blood for blood tests.  This did not involve a tedious trip to Phlebotomy, as they did the blood draw in the clinic.  In addition to routine blood tests, they also did electrolyte levels, calcium and potassium levels, Thyroid function, and liver function tests.  They also took a urine sample.

Next, they took an x-ray of Julia's hand.  This is done to take precise measurements of the growth of her bones.

They also took a DXA (dual x-ray absorptiometry) scan of Julia's spine.  With a DXA scan, low-dose x-rays of two different energies are used to distinguish between bone and soft tissue, giving a very accurate measurement of bone density at these sites.  This was apparently a very fussy test, and hey had to repeat the scan several times with Julia in various positions before they got images that the technicians were satisfied with.

Finaly, they gave Julia an inert tablet to make sure she could swollow the study medication.  She's been swallowing pills since she was 4, and took hundreds of doses of medicine because of JDMS, so this was s piece of cake for her.

After all the tests, they sent us home.  They will call us within about a week and let us know if Julia is eligible for the study. 

Thursday, July 24

We received a call from Dr. G's office with the results of the tests.  It turns out that the bone scan, the DXA test, gave a result of -1.12.  The result means that Julia has a somewhat reduced bone mass, by about 11% lessa than "normal".  This is not severe, and in fact, this study requires that patients have a scan result of -1.5 or lower in order to qualify.  This means that Julia is too healthy for the study!  They did offer to see Julia on a consulting basis if we like, because she does have this reduction.

The also FAXed us the rest of the results of her blood tests (some listed above):

Sunday, August 3

Several months ago, we were invited to join our friend, Jim Kilpatrick, and his wife Lucille, for a few days' vacation in New Orleans.  One thing led to another and we were able to make the trip!  Today, we headed out *very* early for our flight to The Big Easy.

In the past, Julia has had trouble when flying with ear pressure.  It would cause her a lot of discomfort.  Sure enough, on the first leg of our trip on a flight from Providence to Detroit, it happened again.  While at the airport in Detroit, we picked up some ear plugs that are designed to alleviate this problem.  She tried them on the flight from Detroit to New Orleans, and sure enough, she had no problem.  She'll be using these from now on when she flies anywhere.

We arrived at our hotel, the Royal Sonesta New Orleans, a little before lunch time.  The hotel occupies a whole city block, one side of which is Bourbon Street. It's an elegant hotel that's recently been refurbished.   It's also in a great location with easy access to a lot of sights.

Royal Sonesta New Orleans

We put our bags in storage and headed our for lunch and had some local fare at a nearby restaurant.  After a very filling lunch, we still had several hours to spend until Jim & Lucille arrived, so we prowled around a bit.  Our first destination was the Pharmacy Museum.  By coincidence, I'd been reading an article about this same museum in a back issue of Discover Magazine on the plane down here!

I was skeptical at first, but the Museum turned out to be a very cool place.  They documented not only the history of the pharmacy, but captured a lot of local culture in the process.  You could see some gruesome medical instruments that were state-of-the-art in their day, and records of the spread of Yellow Fever.  There is also quite a bit about leeches.  Not for the faint of heart.

Display at the Pharmacy Museum

Next, we took a walk down by the riverfront.  There is much shopping and such in this are, but the Mississippi River is quite impressive.  There are several riverboats that will presumably let you have a ride if you buy a ticket.  We may look into that later in the week.

Late in the afternoon, we met up with Jim & Lucille, who drove to New Orleans from their home in Texas.  Jim has Inclusion Body Myositis (IBM), which is a form of the Myositis.  It manifests itself very differently than JDMS does; it tends to be progressive and does not respond well to treatment.  Jim has limited mobility and gets around in a motorized scooter.  The limits some of his activities, and makes us all very aware of where he can and cannot go in this city.

Jim, Julia's mom, and Julia

On top of all that travel, the hotel did not provide Jim & Lucille with a handicapped-accessible room as they promised.  They were moved from room to room until they were finally put in a satisfactory room. It was a frustrating ordeal!

Finally, we had ourselves a nice dinner at a local restaurant.  Since we all had a long and tiring day, we headed back to the hotel to call it a night early.

Monday, August 4

We headed out early to do some sightseeing and shopping today.  We started out with breakfast at what was to become our favorite place, Café Beignet. It has a quaint open-air dining area that had just the right atmosphere for us, and as their name implies, they served beignets, the local breakfast favorite.  Beignets are sweet, fried dough that are smothered in powdered sugar and are delicious!

We walked down to see the Saint Loius Cathedral, the statue of Joan of Arc, and the French Market.  In case you didn't guess, it gets hot in New Orleans in the summer! 

     
St. Louis Cathedral; Joan of Arc statue; French Market arch

Julia and her mom wanted to take a horse-drawn carriage ride This was something that Jim could not do, so he and his wife went off by themselves for a while.  We picked a likely looking horse, and off we went!  This was a very pleasant ride that took us through the French Quarter and the driver gave us plenty of interesting commentary along the way. 

Russell

A few hours of walking around and everyone was ready to head back for some rest. After we got back, Julia wanted to go for a swim in the hotel pool.  That was fine with us!  We relaxed and chatted poolside (after another frustrating ordeal with finding the key to the wheelchair elevator at the pool area) and had a few drinks.  It can be fun when no one has to drive anywhere!

Tuesday, August 5

We started today with a trip to the Audubon Aquarium of the Americas.  This is a nice aquarium with a lot of interesting displays.  Some of our favorites include the frogs, the rays, the seahorses, and of course the sharks. It was a nice way to spend the morning!

   
Frog, Ray
 
Seahorse, Hammerhead Shark

After the aquarium, we met up with Jim & Lucille.  We decided to go on a riverboat ride.  We bought tickets on the Cajun Queen riverboat. The ride promised a nice ride up the Mississippi, and a stop at a historic site.

The Cajun Queen

Saturday, August 16

We took Julia in for her regular blood work today.  Here are the results:

Sunday, August 17

Dr. A.W. emailed us back the preliminary results already.  They are: CK 104, LDH 176, AST 17, ALT 19, WBC 5.79, HCT 38.1, platelets 399, Sedimentation Rate 9. Aldolase and von Willebrands are still pending.  She says that we'll probably get the rest of the labs back early next week, but things are really looking great!

She also promised to write an EMLA prescription for us. EMLA has been off the shelves for a while, but I heard that it is available again. 

Saturday, August 23

Dr. A.W. emailed us with the rest of the results.  The aldolase was normal at 6.4, but the vonWillebrand's factor was slightly elevated at 192. The vonWillebrand's factor actually isn't a muscle test.  With all her muscle tests normal (especially the LDH and the aldolase), it's hard to know what this means, if anything.

The vonWillebrand's factor, like most tests, has multiple uses, and is influenced by multiple things. It tends to be used as a marker of blood vessel activity, so sometimes it is helpful in JDMS if the muscle enzymes are also elevated. The difficulty is that it can be transiently elevated by illnesses, sometimes minor injuries, a whole host of things--it is in no way an indicator for JDMS.

So, with her feeling well with normal strength, there isn't anything to do differently at this point other than recheck it in 3 weeks or so instead of waiting for our next 3-month blood test.  vW is probably the least reliable indicator of Julia's disease.  Looking back, there have been times when JDMS was very active and vW was normal or close to it; at other times, it have been elevated when the diseasee was quiet.

All things considered, I am not worried about this.  Julia is feeling great looks great, too.  Hopefully, this will not be a bump in the road in Julia's recovery.

Thursday, September 11

Dr. A.W. wants a followup bloodwork because of the littel blip in last month's von Willebrands number.  Because Julia is doing so well physically, they don't want to see her unless there are additional anomalies in the lab results.  We took Julia in today because it was simply the only chance we're going to have; things are very busy for us these days!

We're a bit concerned about how these will come out because Julia had Gym class in school just a few hours before blood was taken.  We know that physical activity can skew the CK number higher, so we'll let the doctors know this when we request the results.

Wednesday, September 17

Dr. A.W. emailed us the results of Julia's labs. CK 205, LDH 190, vWF 106, AST 21, ALT 20, aldolase 6.5, WBC 6.11, hematocrit 35.8, platelets 299, sedimentation rate 8, and C-reactive protein 0.02.

She thanked us for telling her about gym - she said that that could easily account for the slight elevation in the CK. The others all look great, most importantly the LDH and the aldolase, which were the stubborn ones when Julia's disease was active. Since Julia continues to feel well and strong, we can recheck in October.

Monday, October 6

Julia was feeling funky throughout the day and at badtime last night, complaining of a stomach ache.  This morning it was worse, so we decided to keep her home from school.  She was running a mild fever and was having some vomiting and diarrhea all day, so we suspect a virus or possibly food poisioning. 

Tuesday, October 7

Julia was feeling better this morning, but not 100% and still had a low grade fever.  We decided to keep her home from school one more day.  She didn;t have any more epidodes of vomiting or diarrhea, and she got steadily better throughout the day.  By the afternoon, she was pretty much back to normal.

Saturday, October 11

We brought Julia in for blood work today.  Afterward, we decided to go to downtown Boston to look around and have some fun   It's a holiday (Columbus Day) weekend, plus the Red Sox are playing an afternoon game, so the city was very crowded.

We started out at Feneuil Hall and Quincy Market for some lunch.  We decided to take a Trolley tour.  We bought tickets, and got in line for a trolley.  The way it works is the trolleys runs a loop and you can get on and off as often as you like.  They is supposed to be a trolley every 10-15 minutes at each stop.   It starts out at the waterfront by the New England Aquarium, and goes all around the city and hits all the touristy spots.  We got on, and went part way around, and stopped to shop at one of the early stops.

The problem arose when we went to get back on.  Trolley after trolley can and were full, and we couldn't get on, even after waiting for 40 minutes!  Yikes.  We gave up and a driver told us how we could get our money back.  We were so upset that we just waled back to our car and headed for home.

We actually didn't go straight home.  We stopped and had a very nice dinner out and watched what turned out to be a great Red Sox game while we ate. 

Thursday, October 16

Dr. A.W. emailed us the results of last Saturday's blood tests.  CK 170, aldolase 7.9, LDH 213, vWF 106, AST 20, ALT 15, WBC 5.14, HCT 35.4, platelets 340, Sed. Rate 5.

She says that the labs look good. The CK is still just slightly elevated, but she ran the numbers past Dr. S., and he agreed that with everything else looking good and her feeling well, this is almost certainly due to activity. They are are comfortable just seeing us next month when we have a clinic visit scheduled.

Sunday, October 19

Julia had a sore throat today, which was making it painful for her to swallow.  Ibuprofen was working on the pain, and she had no fever.  At bedtime, she was also congested so she took some decongestents.

Monday, October 20

Julia's sore throat was still pretty bad, so we decided to keep her home for school and take her to the doctor.  They didn't find any other problem or any reason to suspect strep throat, but they did advise that she would probably get a full-fledged cold out of this.

Tuesday, November 18

Julia went in to Children's for a regular exam today.  It was incredibly routine.  Here numbers are: Blood Pressure121/63; Pulse 74; 97.5°F; 59.5"/151cm; 110.9 lbs/50.3kg.  The physical exam was like the last few, routine questions, strength tests (that Julia passed easily), etc.  Dr. A.W. said that Julia didn't have to com in for 6 months if her blood tests were ok.  Here's hoping.