Crazy day coming to an end. (Long)

Today went by in a whirlwind!  We both had a difficult time waking up this morning, but we actually got to our first appointment on time. We waited for about ten minutes to be called for the CT scan of Emily's sinuses; the actual scan was even faster. She did very well. She was a little scared once the machine picked up speed. It sounds like the whirring of a washing machine. That can be scary when you are not expecting it, especially when it is over your head.

From the CT lab, we wandered upstairs. We had a longer stay in the pulmonary waiting area, but they called her at exactly nine. The tech that performed the test was such a sweet lady! In fact, her daughter has chronic conditions, and we share a doctor. She was telling me how her daughter decided that more kids needed things to do in the hospital, so she decided to gather supplies and bring them to kids in a wagon. She was seven years old at the time. That really touched my heart!

Anyway, the tech was wonderful. She made Emily feel more at ease, and she explained everything very well. She took gauze and soaked it in a solution made to produce sweat, then she wrapped the arms, put electrodes on them for five minutes per side and wrapped them in ace bandages. Em grabbed the tablet and watched "My Little Pony" for the majority of the time. When we finished there it was already after 10:30. We walked down to the fourth floor to let them know that we may be late. I'm so glad that we did! Emily hadn't eaten yet, and we still had to get to the ENT for eleven. I stole a few minutes and grabbed her some chicken from Wendy's. The wait was interminable!
We actually made it to the ENT on time. I don't know how considering that we had to wait for valet to bring the van. (Using valet is so much easier than walking for blocks with a hurting, tired child and a bunch of stuff that you need.) I had thought that we were in the home stretch. We would have been, but the ENT was running about forty-five minutes late.

I really thought that I knew how this appointment would go. I was not expecting to see so much inflammation still on her CT. Her right side is nice and clear. The left side remains all swollen and shady. The doctor can't say for sure that it's mucus. What they do know is that it is inflamed. They decided not to worry about treating it right now because she has not displayed any symptoms of a sinus infection. Of course, they didn't have the results yet from the Cystic Fibrosis test. I am assuming that will be negative.


We did not get back to the hospital until one P.M.   I could not believe that we were so late! I was very thankful that I let them know that we may be held up. Shortly after arriving we met up with an advocate who works for a company that works closely with our insurance company. She asked me a ton of questions, and she told me that she will try to get us whatever help she can. Her job is to help get medicine and equipment approved, help to find resources, programs, clinical trials, and things of that nature. She flew in just to meet with me for about an hour. I was very impressed. We are going to work on getting a hospital bed approved!  What a blessing this service is.  And she was wonderful!

After she left things started to settle down a bit. The rushing around was mostly done; we just had to wait for the IViG to get started so that we could move to a room. I didn't expect to see a doctor since we did not fill out any of the paperwork, but as we were packing up to head to our room for the evening our main doctor came over. We totally love her!!! She is just so sweet, but she also has more years of experience than the other two rheumatologists combined. She did the strength tests and was very pleased with Emily's muscle activity. We talked a little about medicines- mostly which ones Emily is on and which dosages. She had just started looking at Emily's hands when she asked if we thought that the Remicade was doing enough. I replied that I didn't think that it had been quite enough for a while. Emily was actually doing so well today that I was surprised that she even asked. She told me that, as much improvement as she has seen in Emily, she doesn't think that it is quite enough. Because Emily now receives her IViG to boost her immune system back up, she feels more comfortable about aggressively attacking the arthritis. She wants to see her running more, being more active, and acting like a kid. If insurance approves it, tomorrow she will be starting Orencia again. She had three doses with our old rheumatologist, but when we changed doctor's, they chose the right route, which at the time was high steroids, Methotrexate as second line and Remicade as third line for Dermatomyositis. It wasn't thought to have any effect on DM before, so we changed. Emily is a little nervous, but I'm actually a little excited. We are pushing the envelope, but lately she's had more days where something hurts than not, and that isn't how it is supposed to work.

As one last little shocker, once we moved to our room and got settled the nurse announced that she was going to briefly stop the IViG and give her a round of Rocephin, a good all-around antibiotic. At first, I was very surprised, wondering what I missed. It was a very long day. I realized that Dr. E likely saw the CT scan results from this morning, saw the inflammation, and decided to tackle it. This is one of the reasons why we love her! She isn't afraid to treat the WHOLE child. While some doctor's only take on little pieces, she takes over everything that she can. And this is why we would rather drive two and a half hours from home instead of drive an hour away.

We shall see what tomorrow holds. It will be today by the time this posts... Hopefully we will also know the results of the sweat test no later than Wednesday. Praying that tomorrow is an easier day!

Appointment and long infusion day

We are at our hospital for our long infusion day, staying over until tomorrow.  Today Emily has received 1 hour of steroids, 4-5 hours for Remicade, 4 hours for Rituxan, followed by a 12 hour round of IViG.   In the past she's had the 4 hour IViG, but it works out better when we just stay over.  Last night we stayed at a hotel because we had an early appointment.  It was easier to stay the night before rather than get two kids up at 5 A.M. to be here for 9.  And we are NOT morning people!  We have agreed to just do the longer infusions now so that we stay overnight here.  I made our next appointment for noon.  That gives us time to drop Zach off at school and run over here.  I like that better.

The doctor saw Zach first this time.  We had to draw his labs here since he's on Methotrexate.  MTX use has a slight risk for developing liver problems.  Zach takes the pills orally instead of sub-Q injections, which increases his risk just a little bit more.  I used Emla (lidocaine numbing cream) on one arm first, with a warning that they may not be able to find a vein.  I am so glad that I thought to utter a disclaimer.  Try #1 produced nothing.  Try #2 produced a blown vein.  Try #3 was handed over to a more experienced poker, who got him on the first try.  Phew!  The doctor was very impressed with how his skin looks, as well as his jaw.   We decided not to change anything with him.  We talked about his hands, how it had looked like he had nodules.  I realized today while looking at them that what we were looking at is a bone, but when his hands swell it looks like a nodule.  Good to know.  So his hands have been swollen a LOT.  Maybe that will change now.  He's only had about 2 complete months on Enbrel.

Emily is always a little more complicated.  Her ankles have been hurting her, but he didn't feel any active arthritis in the really bad one.  Hmmm.  Her back has been bugging her for a long time, too.  When I asked her about her pain level (stupid pain scale 1-10) she said, "that thing doesn't work".  LOL!  My big concern with her right now has been her urine.  I don't think I posted about this before, though I know I mentioned it on my boards. 

About 2 weeks ago she told me that her urine was white.  We kind of "hmmmm"d about it.  I meant to ask the pediatrician about it but at the time I was more worried about her sinus infection.  Well, on Wednesday after school she told me that her urine had blood in it.  That got my attention.  I really didn't know what to do about it.  I couldn't take the day off to go to the doctor's.  I ended up calling the hospital because in the end I thought that it may have something to do with her Mixed Connective Tissue Disease.  She's at a much, much higher risk for kidney problems than healthy people. Kidney failure is a common symptom of MCTD.   We ended up having a urine sample run that day, with the results going to our rheumies.  They had figured it may be a urinary tract infection.  Apparently it isn't an infection or a kidney problem.  They think it's a virus.  He said that if it were a rheumatic kidney problem there would be red cells, increased white blood cells, and something else that I can't think of at the moment, all showing in her urine.  They didn't show, though there was blood in the sample. So the good news is that it doesn't appear to be a kidney problem!  I do believe that this is just another sign of disease progression, though.  It seems like every year the disease gets a little worse, and she gets a little more sick.  She's still doing better than some of our JA kids.  There are several kids that have been forced to go on full-time hospital homebound.  Our doc noted that she looks very pale today, but she seems to be doing ok.  We just filled out her hospital homebound paperwork just in case the home schooling doesn't work out.  I'm trying my best to be prepared.  We also talked briefly about her Raynaud's acting up.  It's not nearly as often as it was before Procardia but the activity has increased.  We're keeping an eye on it.

 She had a meltdown out of the blue.  I'm guessing it's the steroids.  She gets 1000 mgs of SoluMedrol before her other meds.   She was looking at the movie list, trying to pick out movie number 3, when she got upset because she couldn't find Little Mermaid on the list.  I asked to see the list, and promptly found her movie.  She asked for the list back, but still couldn't find it on the list.  Next thing I know she's wailing like it's the end of the world.  And yelling at her brother.  Poor boy has had a really rough day and she's yelling at him.  I know it's the steroids.  It's just surprising because she doesn't normally get upset. She's feeling much better now that she's watching her movie.  

So tonight should be interesting.  Here overnight with two kids.  Usually there's either one sleep chair or one little fold out couch in the room.  Either we'll have to steal a chair from another room or Zach & I will have to share a sleeping area.  That should be fun.  :/  But I'd rather do the longer IViG.  Less chance of reaction that way.  Here's to hoping that the we get some sleep tonight!!!