Prayers needed (long)

Right now, every time that I sit down to write, something else comes up that makes me think that I should wait for an answer.  However, since writing helps me to think, I am better off just writing.

Last Friday we went for the requested MRI's to figure out why Emily's head has been hurting so much, for a month or two without relief.  While she seemed so prepared, once we were in the room with the machines, the tech mentioned that he would have to poke her for contrast.  This started an uncontrollable wave of crying that lasted about a half an hour.  It made him very nervous.  She finally calmed down enough to try the jaw MRI.  When the time came for the tech to try the contrast, he couldn't get a vein.  Two tries and she was so upset that he did not push.  It was draining and terrible.  In the end, he showed us clips of her brain.  I thought that was really cool & sweet of him.  He didn't have to.

We went to the hospital on Monday.  I was hoping that we would have an opportunity to really sit down with the doctor and talk for quite a while.  What I didn't count on was spring break.  While there are usually 3 doctors, two Advanced Registered Nurse Practitioners and an RN, this week there was only one doctor and her secretary.  She barely had time to breathe, though she did order a Pulminary Function Test when I mentioned that Emily was having a hard time breathing and her chest hurt. I was planning on getting a plan for exactly how long it would take to wean off of Prednisone, but instead I just got a "we are working on it".  Ok, I get that.  Things happen, we may need to increase, etc.  I may not like it, but I understand.  It's hard to push when she still hurts in so many joints.  I am not convinced that Actemra is right for her, but here is to hoping that it kicks in soon.  I was hoping that we would have MRI results, but no such luck.  She couldn't find them.  We didn't officially get PFT results either, but I did receive a comparison with last time which tells me that everything there is ok for now.  Oh, and we got a prescription for the *occasional use* of Xanax.  This way she hopefully doesn't make another tech nervous.  I didn't have nearly enough time with her, for the most part.

This time on our overnight, the pharmacist came by to go over meds; we never even saw a resident.  I actually like it better that way.  It's nice to cut out the middleman.  For the first time we got a room that was not properly cleaned, but they brought up a dinner tray- something that we usually don't see- and it was what she wanted.  Bonus!

When we got home I started preparing for the appointment with the oral surgeon.  I had debated on whether or not to cancel this, but in the end I decided to keep it because if her headaches were caused by arthritis of the TMJ joints, we would need an oral surgeon.  I had the panoramic from the dentist, the CT results that suggested the molar roots in the maxillary sinus cavity, and the paperwork.  I arranged to also pick up copies of the MRI's from Radiology Associates.  I was not prepared for the report.

They had done 2 MRI's- one of her jaw and sinuses, and one of her brain.  The one of her jaw apparently was not that fantastic.  She moved and they did not get good images.  They did not even mention the sinuses.   Of course they ask me some questions, as well as get a report from the doctor.  We checked yes to headaches, tinnitus, dizziness, feeling off-balance.  We discussed MCTD, Dermatomyositis and the intricacies of rheumatic diseases.  I mentioned that vasculitis had been thrown around here and there, but it never went further than that.  Then I get the report.

  Sometimes it is a beautiful thing to be blissfully ignorant.  I have somewhat looked up vasculitis before, but since it was never (in my eyes) an official thing, I didn't worry about it, so it struck me at the bottom there- "history stated CNS vasculitis".  Whose history?  Presumably from the doctor's since I never said that.  So, what else would I do?  I google.  And I find this:  "If a blood vessel with vasculitis is small, the vessel may break and produce tiny areas of bleeding in the body. These areas will appear as small red or purple dots on the skin. If a larger vessel is inflamed, it may swell and produce a nodule (lump or mass of tissue), which may be felt if the blood vessel is close to the skin surface."  
I had asked in the past what those red spots on her hands were, but I guess I just didn't really put two and two together.  She also pointed out on Friday that she has a "nodule".  I wasn't convinced that it was a nodule, but I guess it may be, or it may be vasculitis.  Yes, this is why the MRI was supposed to have contrast.  So, that floored me, but only later.  What really got me was the Chiari I Malformation.

I had heard of this before, so it wasn't a completely foreign term.  I had even studied it briefly at one point, just because it sounded interesting, and a Facebook friend had one.  I'm pretty sure my heart stopped when I read that, though.  My first conscious thought was "it has to be from the damn steroids!"  Apparently not. They say that it is genetic.  Hmmm.  Of course, I read.  As soon as we got into the oral surgeon's office I googled Chiari, and was stunned to find that she really does have many of the symptoms.  On my phone that was the best I could find very quickly, but she has many of those symptoms.  It even explains her chest pain.  I also read that it is usually diagnosed off of symptoms and an MRI, so my guess is that she will be diagnosed as soon as we get into a neurologist, though I want to speak to an endocrinologist to make sure that it isn't cysts affecting her growth.  (Thanks again, KM!)  In an effort to expedite things, I popped by her pediatrician's office and left a copy of that report.  I am hoping that somebody should call me back no later than Tuesday.  

And the oral surgeon didn't have the greatest news, either.  Pretty much, she does not technically have TMJ, but the muscles that support the joints are weak and sore, which is causing that pain, though it is also possible to have pain there from a Chiari I malformation.  He said that given her history, this is likely an early warning sign of TMJ arthritis.  He pointed out that her bottom jaw is not growing the way that it should; it is a little small, so when she is 16 they will likely yank a few teeth to make room for her molars and give her braces to help extend the jaw.  Hopefully by that point she will have grown, and maybe her jaw will catch up by then.  The roots are NOT in the maxillary sinuses- just close to.  However, those are only her 2nd molars, and there is no room for them.    

So, yeah, pretty much in a nutshell, Friday sucked.  The icing on the cake for me was finding out that they messed up when they did our taxes, and we are getting back $300 less than what we were told.  Yeah, and that refund check will definitely not be here by this week.  We should have had our refund by now, but this is how we roll.  

There is so much to process, and figure out.  Hopefully we can get the ball rolling this week.  If she wasn't in pain daily, not able to concentrate, than I would maybe not worry about it.  They say that most people that have a Chiari I malformation have no idea that it is there, but of course she does.   It really makes me sad that everything happens to her.  I now cling to the knowledge that she may go into remission on the MCTD front later on in adulthood.  She really needs a break.  Please pray for Emily that we get all of this taken care of without the need for surgery.  Thank you.

Good news for Emily, new worries for my son

We have a lot happening right now, as always.  I'll start with Emily's appointment.  Hers was actually great!  I suspected that it would be.  Minus the steroid weight that she still carries stoically around, she's feeling better than she has in a very long time.  Since we've added the Rituxan into her infusion schedule, she's now able to strap herself into her seat belt in the vehicle's by herself, she can finally roll her windows up and down in the car, and she's finally climbing stairs again!!!!!  That is huge to us.  I really hated for the kids to go back to school; I'm the weird mom that actually likes hanging out with her kids, but I knew that it would be so good for Emily.  She pushes herself at school in ways that she won't at home.  I'm so excited to talk to all of them when they get home from school, but especially to find out what physical activity she has done.  

The doctor was impressed at how far she has come on her Rituxan.  We are leaving her Remicade dosage the same, but we are able to decrease her Prednisone again!  We tried in July, but that was before the Rituxan.  The doctor did note that her left hand feels tight, but otherwise she was amazed at her energy.  She's also very happy that we have our port surgery scheduled.  Emily & I can't wait for that.  I'm so glad that Em's on board with this.  I really, truly feel like this could potentially be the best decision we've ever made for her.  Every infusion her stress level is worse.  She's great until the nurses come over with the supplies.  This time, they went & got her new favorite nurse (because she can get her on the first try usually) from the unit next door and had her waiting.  Em hates having the needle in or near her hands.  That's the first place they went.  She just freaked- shut down.  Next thing I knew, she's kicking, waving her arms around, thrashing, and screaming frantically.  The more upset a person gets, the harder the veins are to access.  They had to give her a break.  Even worse, her nurse warned her that if she didn't calm down she would have to leave because she had other patients, but Em just couldn't do it.  I think Em understood why she left, but I think she felt bad about it.  I totally agree with her needing to get back to her other patients.  She can't wait around all day- especially when it's not her unit.  I really appreciate how well the people in this facility get along.  There's a high level of support that I really appreciate seeing.  The infusion room nurses have to go through a lot.  The hours are good, but they have a lot of other screamers and harder kids.  Somehow they always seem to be a happy bunch.  It makes it so much more fun to go, because they're all crazy.  :)  For us, after the pokes are done, we can chill out.  (Often literally; it's freezing in there!!!)

Otherwise, her appointment went well.  I asked if we'll be ok to go to the family weekend at camp since it's the weekend after her port placement, and we're cleared for that.  The rest of what we spoke about wasn't actually about Emily.

I've debated on talking about this since I really don't know what's going on yet, but I decided that no matter what it turns out to be, these are the things that we have to worry about.  For anyone that wonders about the types of things that go through our heads, here ya go. 

I mentioned briefly at the end of my last post that my son had mentioned his jaw was clicking.  I swear, my heart stopped last week.  He showed me how he can pop his jaw and how it clicks and hurts.  I was dumbfounded.  When we were at the conference, we attended a session on JA & Jaw issues.  We missed the first 20+ minutes because we were speaking to another doctor in the hallway.  What we caught was that jaw issues are usually silent, and up to 81% of kids with JA have jaw involvement; the speakers says that evidence shows that the jaw may actually be the first joint affected.  My son has had psoriasis for about 3 years.  He has nail presence and other signs that lead to potential psoriatic arthritis.  Now, I'm not by any means saying that he definitely has PA.  However, when I asked our rheumy if she felt that it could be, she said that given his psoriasis and our family history, he definitely could be developing arthritis.  SO, I'm trying to get a panoramic x-ray appointment set up for him, but since we are totally broke from so many trips to Shands, I can't do that right now.  At least, not with his regular dentist.  I've emailed our hospital about it.  I figure, if it is PA he'll need to see the rheumy too, and since we're always there anyway I may as well schedule it for when we're there with Emily.  I am praying that it is just coincidence.  Not that I would want him to have TMJ, but TMJ would totally be better than deterioration from arthritis.  Not to mention, at least TMJ only affects the jaw area.  Our rheumy said that most PA is considered in the Spondylitis family- something I haven't read up on yet.  I've read up a lot on PA today, but I think we're in for a wait.  I really hope that my precious little boy doesn't have to go through this crap, too.  If he does, I'm hoping that we are catching it way earlier than most kids are diagnosed.  There are some things to be said about a heightened awareness!

And I will leave you with food for thought.  We are very blessed to have (for Emily only) Medicaid as a backup from Social Security.  There is no way that we could afford our copays without it, as I will show below.  This is a copy of our insurance explanation of benefits.  Emily has a $3500 deductible.  If we hadn't met her deductible, and if we didn't have Medicaid, I cannot imagine what our copay may have been.  We have a 20-30% copay normally.  I know that we had a balance from one bill that Medicaid wasn't covering that was over $5000.  There was another for $1200 and another for $1500.  How could any normal person afford these treatments?  Why do they have to be so expensive?  And, if arthritis is "no big deal", then why would we be paying such a high amount of money per visit???  The visits include seeing the doctor, labwork, and infusion meds.  Of course, it's the meds that are expensive.  When she was on Enbrel, we could do her shots at home and with only a $10 copay with their assist program, but without insurance they would have been $2500 per month from our pharmacy.   Someone told me once that they were charged $36,000 for an IViG.  I can't find it again, but I believe I read that Rituxan goes for $11,000 for the first 2 doses.  The rest are smaller doses, going for more like $5,500 each.  

The All Children's bills are from our therapy appointments at a specialty center.  She has occupational and physical therapy weekly.  (Her therapists are awesome!!!)  The last entry is from our pediatrician.  (LOVE her!)  I learned a few years ago that doctors charge you by your conditions.  If you are a very healthy person seeing your doc for something like a sinus infection, you will get charged MUCH less than my daughter will, even if she's being seen for the same sinus infection.  Good times, eh?