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Monday, July 1
Warm day today! Up in the low 90's (F), sunny and hot and very humid.
Tuesday, July 2
More heat, even hotter than yesterday.
Wednesday, July 3
ANother scorcher! All you people who live in warmer climates will probably laugh and shake your heads at us, but we don't often get stretches of hot weather like this.
We went for a visit in Rhode Island this evening, and they had a pool. Julia wanted to give swimming a try, and since she's scheduled to get her cast off of Friday anyway, we decided to let her go. She put on the Aquashield, and as instructed, we let as much of the air out of it as possible. It worked great! Her cast and leg stayed dry! She really enjoyed being in the pool for a change.
Thursday, July 4
Happy Independence Day for all you U.S. residents. Another scorcher, though perhaps not as humid as yesterday. We spent the day in laid-back mode. We went to see a movie (Men In Black II), went out for lunch, then hung around the house for the afternoon. The Red Sox won, which is always a good thing! We ended the day with a trip to our favorite local ice cream stand.
Friday, July 5
We all had the highest of hopes that Julia's cast would come off today. She has stayed off it for almost the past 4 weeks without exception. She has had no pain or discomfort of any kind for more than 2 weeks. However, it was not to be.
We all went to the Orthopedic doctor's office this time. After a brief wait, we went to the Cast Room, and the Cast Technician took all of about 60 seconds to remove the old fiberglass cast, just as slick as can be. He used a special reciprocating rotary saw and cut straight down the sides of the legs and then down along the foot, and opened the cast like a nut shell. The leg looks good - no swelling, and just a bit of discoloration, which we were told is to be expected. The broken left leg is visibly thinner than the right leg. That is also to be expected.
She went off for x-rays, and after a few minutes, the doctor came by to explain. The break is healed near the end of the bone, by the growth plate, but there is still a break visible on the film. The doctor said that she has to have a cast on for at least 2 more weeks, then we will re-evaluate again. Julia was very disappointed and upset.
However, this time we made 2 improvements. First, this is a weight-bearing cast. Julia was given a little boot to wear over the foot of the cast, and she can and should put increasing weight and pressure on it. This will help the break to heal and also re-acclimate the leg to support weight. They advised us to use a scale so that Julia can get a feel for how much weight she's putting on it, and to increase the weight gradually. She can also graduate to using only one crutch when she feels comfortable enough, and to no crutches after that.
The other improvement is that instead of a cotton gauze inner liner for the new cast, we opted for a Gore-Tex inner liner. Gore-Tex is a rubberized product that is waterproof. Between that and the fact that the cast is fiberglass (not plaster), this means Julia can shower and even swim with the cast on and not have to worry about damaging it. She doesn't even have to wear the AquaShield! Unfortunately, the insurance doesn't cover the extra cost of the Gore-Tex liner (go figure), so we paid it out of our pocket. It's well worth the US$20 for Julia to be able to swim if she wants to.
Julia went with a plain white cast the first time, but this time she went with bright yellow! She has also vowed not to let anyone sign it, but it's possible that the temptation to decorate it like a bumble bee or put a smiley face on it will get the better of her.
So, between the walking cast, the swim capability, and the cool color, Julia is okay with the new cast. Well, as okay as you could expect.
Saturday, July 13
Today, we started our vacation! We decided on a driving vacation this year, and over a long time, we settled on Niagara Falls, with a side trip along the way to the Baseball Hall of Fame. But I'm getting ahead of myself.
We set out early this morning; we were planning a 6:30 AM departure, but managed to get out the door by 7:00. It took a while to pack all the stuff we needed into our car, but it all fit. The first leg of our trip took us to Cooperstown, NY and the aforementioned Baseball Hall of Fame. Cooperstown is picturesque, a very nice place to visit. We were surprised at the high level of foot traffic in the downtown area, but an almost complete lack of cars driving by. Usually places like that are in a constant state of gridlock!
Julia is still dealing with her broken leg. She is on crutches, and although she is able to put some weight on it, she is being very careful. As soon as we arrived at the HoF, we traded the crutches for a wheelchair, which they conveniently provide upon request.
The exhibits were great. Julia's mom is not a very serious baseball fan, and she was impressed. They have displays for "Today's Stars", which includes records, milestones, and notable achievements from the current season; the "Locker Room", with mock lockers for some of the best players; a miltimedia show on baseball. They also have, obviously, a lot of baseball history covered - the formation of the game, the early stars, and other people who helped the game grow. They also have sections on Baseball cards, Ballparks, the evolution of uniforms, and the minor leagues.
After being at the Hall for a couple of hours, we went out for lunch and a little shopping. We had a nice lunch, then shopped at some of the dozens of stores in the downtown area that sell baseball-related paraphernalia. The staff at the Hall made it easy to trade back and forth between the wheelchair and the crutches.
The ultimate display is the Hall of Fame Gallery, with plaques for all the inductees into the Hall. Each plaque has an image of the player, their name and nichname, and a brief history. The Ted Williams plaque was decorated with flowers, as he had died just a few days ago.
By the middle of the afternoon, we were ready to push on. We planned to make a stop partway, and we stayed overnight in Syracuse. Syracuse was just 90 minutes from Cooperstown. We stayed at our favorite hotel, the Embassy Suites. They have nice 2-room suites to stay in, a pretty atruim (with a fish pond and other running water), a "managers reception" each evening, and breakfast is included. We had all been snacking all afternoon and with the goodies at the reception, we didn't even go out for supper. It worked out bery well.
Sunday, July 14
After our (big) breakfast, we found a local church and went to Mass. They apparently get a lot of people from out of town at this church, as one of the pastors commented about our out-of-state license plates. It was a very nice Mass. It's always fun to sample other churches to see how they do things differently that what we are used to.
We drove west for a couple of hours, and at one point Julia's mom saw some signs for Wineries in the area and said, "I'd love to visit a winery!" Well, I fired up my eMap's Find function and came up with a long list of wineries just a few minutes off the highway. On a whim, we decided to go visit one.
We Ended up going to the Knapp Winery, which is located between Seneca Lake and Cayuga Lake in the Finger Lakes region of upstate New York. They were not actually offering tours of the winery, but they were offering wine tasting and we did end up buying a couple of bottles for our trip.
The winery also has a nice little restaurant, and we decided to eat lunch there. None of us were very hungry, but we had a light lunch anyway. It had a nice view of the vineyards and some gardens. It turned out to be very pleasant.
Back on the highway, we still had a couple of hours before we got the Niagara Falls. We were staying on the Canadian side, which we were told by everyone was the only place to stay. We did get lost trying to find the bridge to cross into Canada, but clearing Canadian Customs was pretty easy. Easier than I expected, actually, with all the "heightened security" going on.
After making it into Canada, we got lost again trying to find our hotel! We ended up driving down the road that runs right along the Falls. We got our first look at the falls, and they are amazing! We didn't really want to stop, we were tired and just wanted to get to our hotel, but the quick preview of the Falls was almost worth the side trip.
We did eventually arrive at our hotel, the Hilton Niagara Falls. We checked in, and retrieved our wheelchair. We called the hotel ahead of time and asked about using a wheelchair from the hotel. They wanted to charge us CAN$25 per day to rent a chair! I called the NF Chanber of Commerce, who gave me a couple of phone numbers of places that could rent us a chair for the week. We ended up renting a junior-size wheelchair for CAN$20 per week, plus a nominal delivery fee. (In case you're interested, the place that rented us the chair is the Queen Street Pharmacy.) It worked out great.
The hotel is a couple of blocks from the Falls, but it's 33 stories tall. Our room is on the 25th floor and has a view of the Falls! Here, take a gander at what we could see out of our room every day:
The pictures don't really do the view justice. It's really spectacular. Yes, there is a bunch of construction going on between the hotel and the Falls, but the view is still great. On top of that, there are fireworks over the Falls every Sunday and Friday night, and we have a great view of them from our room!
We ate dinner in the hotel restaurant, the Watermark. The restaurant is on the 33rd floor of the hotel, and faces the falls. Another amazing view, even nicer than the room view. The food was pretty good, too. After all that driving today, we wanted to just lay low this evening.
The last thing we did today was see the fireworks show. It's over the American Falls, and it was quite the colorful display of pyrotechnics. It was just 7 minutes long, but was a nice closer to our first day here.
Monday, July 15
Our first full day in Niagara Falls, and we have lots of touristy plans! Our hotel package includes breakfast every morning, either in the restaurant of the hotel, or in the Denny's right next door. We decided to give Denny's a try. Well, they seated us in a "non-smoking" section with two different tables with smokers at them within 15 feet of us. The food selection is rather limited, and the place was a general mob scene. Also, we figured that we could easily have breakfast for 3 for the CAN$35 allowance the hotel gave us. Wrong! We decided to give the hotel restaurant a chance tomorrow.
After breakfast, we retrieved our car and headed out to Clifton Hill, the mecca of tourist attractions in Niagara Falls. The area features attractions like arcades, museums, fun houses, and haunted mansions, plus hotels, tons of shopping, Casino Niagara, and restaurants. We had Julia in the wheelchair, partly to keep her off her broken leg and partly to keep her from getting tired so quickly. Getting around on crutches is apparently very tiring, and we didn't want that to ruin our vacation.
First, we went to the Ripley's Believe It Or Not Museum. This is a fun collection of displays relaring to RBION features over the years. There's plenty of interesting stuff, amazing stuff, and gross-out stuff. They also have a great section on Niagara Falls BION's, with descriptions of people who went over the falls, successfully or not. The most amazing to me is the 7YO boy who went over the falls in 1960 wearing only a life jacket and survived!
Julia and I went on a motion ride of the kind they have plenty of in Disney World and other similar places. It was actually a double ride, one right after the other. We love these things. This was also the first time the wheelchair can into use - thry brought Julia in to the theater with a special wheelchair elevator.
After this, we went to a Haunted House place. Julia and her mom got a little spooked - it was full of people hiding in the shadows, and we had to make our way through a lot of dark corridors wiht only a penlight, and most people don't even get that! Another family latched on to us because we had the light. We eventually got through it.
After doing a little shopping, where Julia got some Beanie Babies that are only available in Canada, and we picked up some gifts, we headed down to the Falls area to get a look and take some pictures. Here's a nice shot of the American side of the Falls. In this picture, notice: the smaller, separate waterfall called the "Bridal Veil", a Maid of the Mist boat in the foreground, and the catwalk on the lower right of the Falls:
It was very hot this day, which made us once again very happy to have Julia in a wheelchair. She protected by sunglasses and a baseball cap. Although Julia did apply a liberal layer of sunscreen before this trek, we unfortunately didn't bring any extra with us. Also, she forgot to apply some to her exposed toes. We resorted to covering her toes with paper napkins, and made her hold our shopping bags to protect her thighs. Later we bought her an unbrella to hold for shade. It seemed to work; although her skin did appear to get a little pink, it must have been because of the heat because she did not get a sunburn at all.
Our walk took us all the way down to the Canadian side of the Falls, and the view is magnificent and spectacular. It's really stunning how beautiful the "Horseshoe" falls are - it is almost mesmerizing watching all that water go over the edge.
After taking in all this, we headed back. We had to make our way all the way back along the falls, then up Clifton Hill. Before we got to the car, we made one more stop, at the Guinness World of Records. This attraction is very much like the Ripley's attraction - displays of amazing facts, with a few videos and other stuff thrown in. If you come here, pick one or the other, you don't really need to see both. I found them both about equally interesting.
Finally, to our car, then drive to the hotel to clean up. We went out again, to dinner, at Planet Hollywood. It was a nice meal, but like all PH's, it was overpriced. After dinner, we headed to the hotel pool for a nice swim.
Tuesday, July 16
We did breakfast buffet at the hotel today, and now we're sorry we didn't yesterday! The atmosphere is comfortable, the service is excellent, the view is unbeatable, and the food was good, varied, and plentiful. The breakfast voucher covered us adults, and Julia was only CAN$10+tax. Guess where we're eating breakfast for the rest of the week? In fact, each day we generally ate a large, late breakfast, had only a snack for lunch, and then had an early, full dinner out somewhere. It worked out great.
On Sunday, we made reservation for a spot on a tour
bus for today. We reserved early so we could get a spot on
a bus with wheelchair access. A little after 10, Martin's bus
rolled up and we started our tour of Niagara Falls.
Our first stop was the Skylon Tower. The tower is close to our hotel, and you can see it in the picture above (from our hotel room) to the left of the American falls. This tower is 520 feet tall (to the top of its roof beacon) and provides excellent fiews of the Falls and the whole Niagara Falls area. It was another hot but clear day, and lots of pictures were taken.
We made a brief stopover at a local shop that sells hand-blown glass that is made on-site. They do glassblowing demonstrations, which are interesting, but which we have seen before. They specialize in "cranberry glass" which is a unique red color. With the glass-firing ovens, the hot day, and the lack of air conditioning, it was uncomfortably hot in this store and I think we were all happy to move on.
Our schedule got rearranged by a reservations snafu, and our next stop was the Maid of the Mist boat ride. This boat ride takes you right into the base of the Canadian falls, within yards of the rocks! We got into a long line and donned snazzy blue ponchos. Julia rode the wheelchair right up to the ramp for the boat; then she hopped on hung on the a railing until we got off. It worked well.
This ride is amazing! Getting up close and personal with the base of the falls is quite an experience! The mist comes down on you like rain the whole time you are there. There's a constant roar from the water slamming down on the rocks below. The boat is pitching back and forth in the currents. It's very exciting and awe-inspiring at the same time.
Next, we took the "Journey Behind the Falls", which takes us through long tunnels that literally go behind Niagara Falls. Frankly, the view from back there is completely unspectacular. Imagine looking into shower. Anyway, it also takes us to a platform at the base of the falls, which has yet another amazing view. Once again, ponchos (this time yellow) were issued. Julia managed to get through on her crutches.
Next stop was a touristy souvenir shop. It was actually several stores that were connected to one another inside a common building. It also had some tourist-attracting displays and decorations outside.
A ways up the road, we saw The Whirlpool, a roughly circular river gorge. It is so called because the water flows through it either clockwise of counter-clockwise. There is a cable car system that crosses the gorge, and we could also see open and closed jet boats driving around and through this part of the river. Looks like fun!
Next, the tour took us past the Sir Adam Beck #1 and #2 hydro power stations to the famous Floral Clock. This is a giant clock whose 40-foot face is made up of thousands of flowers. Its hands are in the shape of crutches, and the movement chimes every quarter-hour.
That ended our bus tour! It was supposed to be 5 hours long, but actually lasted almost 7. It was actually a pretty good deal, and the driver did a good job of dealing with Julia and one other passenger who had to deal with wheelchairs.
After cleaning up at the hotel, we headed back to the Skylon Tower for dinner, which is just a 5 minute walk from our hotel. It's usually CAN$2 per person to ride the elevator, but we saved our passes from this morning and rode up for free. They even let us to the front of the (long!) line because of the wheelchair. The Tower has a revolving restaurant that takes an hour to complete a revolution. What a great place to have dinner! The view is just stunning, and the food & the house wine weren't bad, either.
Wednesday, July 17
We started off today at the Imax Theater. which is just a couple minutes' walk from the hotel. They have a giant movie screen, and a 45-minute feature film about Niagara Falls called Niagara: Miracles, Myth, and Magic. I went in expecting sweeping images of panoramic vistas and magnificent close-up shots showcasing the power of the Falls. I left disappointed.
The film showed, perhaps, 10 or so minutes of Falls images. The rest of the film played out several vignettes and dramatizations of people in the history and mythology of Niagara Falls. The stories were lame, and the acting was awful. The scenery and props were a complete waste of the Imax system - they would have looked fine on TV. The scenes of the Falls were good, but there weren't nearly enough of them. Maybe, if you hadn't already gone to see the Falls, this might be somewhat entertaining, but really, this was a waste of time. It's about the only thing we did all week that I regretted.
From there, we went to the Butterfly Conservatory. This is a large screened-in area that is full of plants and flowers with paths winding through it. Here they keep thousands of butterflies that you can see and photograph, and they may even land on you!
They have dozens of species of butterfly and some of them are absolutely brilliant. Here are a few samples (click on the thumbnails for a larger version).
We were interested in visiting the Sir Adam Beck #1 and #2 hydro power stations, but both the concierge at the hotel and our bus driver told us that they had put all tours on hiatus after 9/11. We headed north for a ride and noticed that the power station was offering public tours! We stopped and turned into the parking lot so fast, there must have been smoke coming off the tires.
It turns out that they are reintroducing public tours in the next several weeks and they are offering "beta" tours now! We won't be getting the full treatment, which includes a video and other goodies, but it will still be cool to check out the innards of the power station.
A patient and soft-spoken tour gude named Brian started by showing us a giant map and explained the history of hydro power in Niagara Falls. The hydro project is a truly incredible engineering achievement. It involved diverting rivers and creating canals and tunnels to handle the water flow. They even have an artificial lake that acts as a reserve power source!
Next he took down an elevator us to see the machinery of the plant. It's really hard to grasp the scale of a place like this without seeing it. The turbines that they use are big, and the assemblies are enormously heavy! At the end of the tour, Brian was teasing us about visiting the gift shop on our way our, but he was kidding!! We were seriously interested in a tee-shirt of something, but they don't sell any mementos. We were bummed out about that. Still, it was a fun tour, and consider ourselves lucky to have taken it.
We drove a little bit further north, then headed back to the hotel, where we ate our dinner tonight. After dinner, we went for a swim, then watched TV in our room.
Thursday, July 18
We began this morning with a trip on the Whirlpool Jet Boats we had seen during our bus trip the other day. When we called to make a reservation, they advised us to bring a complete change of clothing. They were not kidding!
They started with an orientation that explained what we could expect, and the safety rules that had to be followed. Everyone, including the captain of the boat and our guide, Jessica, has to wear a life jacket at all times. Our crew also included a videographer. Jessica said that we would all get wet, soaked in fact, but the boats were designed for this and had watertight cells so that it could not sink no matter what. Good thing!
More about the boats. They are big, open, and hold up to 48 people. Ours had about 35 people in it. They advised that kids not sit in the most-punishing first 3 rows, so we naturally sat in the 4th row. Jessica said that the boats are powered by twin 1500HP engines and can hit 55MPH fully loaded.
She also explained about the rapids. They are not rapids caused by submerged rocks, but rather by the sheer volume of water that has to pass through narrow parts of the river. She said that the rapids are rated based on their difficulty using the International Scale of River Difficulty , a I - VI level rating. She said we would be traversing some Level V rapids, but looking at the ratings, I'd say they were more likely high III or low IV level.
We were issued our gear - life jacket, scuba boots, and poncho, and we headed out. We went quite slowly at first until we passed by some residences with a strictly enforced speed limit to prevent wakes. Then the captain cranked up the speed. It felt like highway speed! When we got close to the rapids, the captain did a 360° turn to give us a feel for how the boat could perform. They we hit the rapids.
We quickly all got soaked! The steepest rapids felt like we were driving almost vertically up and down their faces, and when we hit the bottom, the boat would be inundated. It felt like getting barrelfuls of water dumped on your head! Again and again, we got drenched. It was exhilirating! The speed, the up-and-down action, and the water combined for a thrilling, unique ride.
The whole trip lasted almost an hour. We got back and changed into dry clothes, then saw the video they shot. They did an awesome job of putting together the video, complete with music and special effects, in just a few minutes. Yes, we bought a copy (it was just CAN$20) - ask to see it next time you're at our house ;-)
After the ride, we spent the day in Niagara-On-The-Lake, a quaint and quiet community about half an hour drive north of Niagara Falls. It is so called because it is on the shores of Lake Ontario. This was the first time any of us had seen any of the Great Lakes, and it's easy to see why they are called Great! Standing on the shore of the lake, it stretches as far as the eye can see. It's like looking at the ocean!
They have a downtown shopping area where we browsed around for a while, then took the scenic route back to NF. We made a stop at a local drug store on the way to replenish our room supplies and snacks. This was fun in itself, seeing items and brands for sale that we never see at home. It was also a lot cheaper than buying stuff in the tourist areas!
We had dinner at the Whirlpool Golf Course restaurant. It was quiet in there, and very inexpensive, and had a nice view of the golf course.
Friday, July 19
This is our last full day in NF, a mostly lay-low day. We checked out the local mall and took in a movie, then went back downtown to the Clifton Hill area to do some shopping and stuff. We had our last dinner out at Rainforest Cafe. Our waitress was unfortunately a NY Yankees fan, and she and Julia gave each other some good-natured ribbing on the subject.
After dinner we headed back to the hotel for a swim, then took in the Friday night fireworks. A nice way to spend our last evening!
Saturday, July 20
This is our travel day. We headed out soon after breakfast at the hotel. We had to finish packing, drop off the wheelchair at the bell desk, and check out. We actually hit the road at about 10:00. It was a long drive, about 8 hours total, and we only stopped for fuel (twice) and a couple of rest breaks. We got home a little before 6:00PM. Julia slept part of the way home, and did not want to go to bed at a reasonable hour! Oh well, we're on vacation, right?
Monday, July 22
Julia went to the Orthopedic doctor today to get her latest cast removed. They removed it, took an x-ray, and it looks a *lot* better. There is no swelling or bruising of the injury site, and the break is all but invisible on the x-ray.
They put Julia in a "gel cast". This is a temporary cast that Julia has to wear for then next few weeks, but only when she's doing significant walking. She does not have to wear it to shower, swim, or sleep. We don't even have to go back! It looks like this broken leg is just about history.
Tonight, we also had a visitor at our house. A couple of weeks ago, at Julia's annual check-up appointment with her regular pediatrician, we agreed to be interviewed by a medical student. She needed to interview a family who is dealing with a long-term illness, with emphasis on coping and support, and make a presentation on it. We're always willing to help out the next generation of doctors any way we can.
She arrived on time and introduced herself. We sat down at our kitchen table, and she just simply asked us to explain how Julia's illness came to be. We hadn't told this story in any detail for a long time, and it seemed like we were a little slow to get started. I guess we were expecting a series of questions to which we could give specific answers, and not such an open-ended one-way discussion like we ended up with.
Well, once we got started, there was no stopping us! It takes a while to go through all the things that happened to us over the past 4+ years. I'm sure we got some of the timeline and details a little wrong, but I thought the overall story came through pretty well. She had seen this web site, and we told her that if there was a discrepency between what we said and the site, go with the site.
She sat there for almost an hour and a half with a notebook and pen, but didn't take a single note! Se said that between our conversation and the web site, she'd be fine. We wished her good luck on her presentation, which she said was tomorrow, and told her to let us know how it went.
Finally, we got email from Dr. A.W. and she said that the von Willebrands from last time was 111, well within normal.
Tuesday, July 23
Julia had a checkup at Children's Hospital today. It was pretty routine, except we were seen by a new doctor, Dr. H. She is a new intern in Rheumatology. As always, we're happy to help out the doctors who are learning.
We started out by doing the history from day 1 - pretty much the same story we gave last night, except abbeviated. It's actually good that we went throught his last night; everything was pretty fresh in our minds. Dr. H. had a few questions along the way, and at one point she asked about the x-rays from Julia's fractured leg. I said that they were on the web site, so she logged in on the spot and looked them up! It's nice to be able to do that. She said that she'd only seen 2 other JDMS patients, and had gotten a link off the Diary links page to give one of them more information about the disease.
After giving the story, Dr. H. did a physical exam. She did all the usual strength tests, which Julia passed beautifully, even with her weaker broken leg. She also looked at Julia's nailbed capillaries for abnormalities, and said she could see them. She also talked a bit about Julia's bone density, and how it's possible that her fractured lag, and her fractured arm from a couple of years ago, may have been caused or influenced by her long-term Prednisone use.
After a few minutes, Dr. H. came back with Dr. A.W., who asked about how Julia is doing and repeated the physical exam, to the same results. We also talked about our upcoming visit to the Endocrinology department. That visit should consist of 2 parts - evaluating how Julia is doing in the aftermath of taking Prednisone for such a long time, and seeing if she is suitable for any studies they are conducting. She said that they are probably looking for kids who are still taking Prednisone, but we will see.
We didn't do blood work today, but left with a bloodwork form filled out with everything that Rheumatology needs. When we go to Endocrinology later this week, they will amend the bloodwork form with any additional tests they need. They will also dictate when the tests need to be done - they may have specific requirements (i.e., first thing in the morning on an empty stomach).
We got out of the hospital just after a Red Sox game let out about half a mile away, just as some intense rainstorms started, and at the beginning of rush hour. It took us a lot longer than usual to get home tonight.
Thursday, July 25
Julia saw the Orthodontist today. This was an evaluation visit that was set up at her last regular dentist visit. After examining Julia, he said that the crossbite that was such a concern for our dentist over the past couple of years is completely gone. However, she does appear to have a slight overbite and a couple of molars that are rotated in their sockets. They will not be doing anythign right now, but we scheduled Julia to have a complete workup done in a few months, which includes head and mouth x-rays and another oral exam. At that point, we can decide how we want to proceed.
In the afternoon, Julia had an appointment back at Children's at the Endocrinology clinic. This appointment was scheduled a while back so they could evaluate the effects of her taking Prednisone for so long. Before the appointment, they sent us a 5-page questionnaire which included questions about Julia's medical history, her parent's and grandparent's history, and more.
Julia got weighed (95.7 lbs), measured (55.5") and cuffed (115/60), then was seen by Dr. M., who is in a fellowship program. This is similar to the position Dr. A.W. was in when we started seeing her 4 years ago. Dr. M. started by going through the questionnaire and confirming all the answers, and getting a bit more background as needed. Then he did a complete physical exam on Julia.
After Dr. M. was through, Julia was also seen by Dr C., who appears to be supervising Dr. M. She addressed a couple of questions that Dr M.'s physical exam raised, and also looked Julia over.
We then had some questions for Dr. M. He seemed to think that Julia did not lose that much height, or gain that much weight, considering how long and how much Prednisone she took over the past 4 years. He also thought that she would not get to be as tall as she would have without taking Prednisone. She probably would have ended up being 5'6" or so, but projecting out will now probably end up at about 5'3". Seeing as her mom is 5'2", that should work out fine.
We also talked about additional blood work to determine how Julia's endocrine system, particularly her adrenals, are doing. First, he wants Julia to have her Cortisol level checked. Cortisol is the natural steroid produced by the body in the adrenal glands. This test involves getting blood drawn first thing in the morning, before Julia eats anything. We'll get that done next week locally.
He also added one additional blood test (DHEAS (Dehydroepiandrosterone Sulfate), an adrenal hormone) to be done any time, because it tends to be more level throughout the day. They had blood drawn after this appointment was over.
We were also give a prescription for a hydrocortisone to give Julia if she has medical stress, like fever (especially fever of 101° or higher), GI bugs, surgery, or trauma. Yes, if we had known, we should have given Julia some of this after her fracture; it probably would have helped her to heal a bit faster. However, no harm was done by not giving it.
Tuesday, July 30
We brought Julia to our local doctor's office lab to have her cortisol level checked. A simple blood test.
Wednesday, July 31
We got an email from Dr. M. today. He received Julia's morning cortisol results from our lab. The level was 11.71 ug/dL. He said that this is not high enough for us to feel comfortable that she can mount a normal response to medical stress. They'd prefer to see a level of at least 18-20 ug/dL.
So, Julia will need an ACTH stimulation test. This will be done in the CAT/CR at Children's first thing in the morning. They will place an IV, draw labs for a baseline level, give low-dose ACTH, draw labs again, give high-dose ACTH, and draw labs again. If she makes enough cortisol (18-20 ug/dL), we can feel comfortable that her adrenal glands are back to normal. Until we have that test, she should get the hydrocortisone during fever, GI bugs, surgery, trauma, as we discussed in clinic. Julia does not need to fast for this test.
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