Disease progression despite so many meds...

Back in April 2008, our old rheumatologist thought that perhaps Emily could have Scleroderma.  I didn't know what it was, but trying to be the informed parent, I promptly went home and looked it up.  Then I wished that I hadn't.  This was the first "too real" situation that we found ourselves in.  Trying to compare diseases with limited knowledge, I thought that she had something called CREST syndrome.  "CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome is a member of the heterogeneous group of sclerodermas, and its name is an acronym for the cardinal clinical features of the syndrome."  Bottom line?  It messes you up.

I learned that there are different types of Scleroderma.  There is one type that is called linear; it mainly affects the skin, causing the skin to darken and harden.  It can harden to the extent that it can restrict movement.  It can cause disfigurement.  Then there's Ssc, or Systemic Sclerosis.  This is what really scared me.  The literal translation for Scleroderma is "stone skin".  In Ssc, it can literally harden your internal organs.  Think about it- think of things like hardening of the arteries.  It can slowly harden your insides.  I spent a good two months absolutely sure that this was her problem.... and then we were told that it wasn't.  Phew!, right?  Ok, so another little gem that I have learned is, just because something is ruled out, doesn't mean it will stay out.  Since her old rheumy failed her so badly, we tried to just be more watchful, and of course I started to really research everything.  Lord knows, as my wonderful hubby said tonight, if we stayed in his practice she likely would have been dead last year.  So, it felt like, if I could just research enough, maybe I could find a clue that other couldn't.  Pretty farfetched, yes.  But, it helped me to at least feel like I had an active role and I was doing stuff, not to mention educating myself and anyone else that would listen.

We noticed that her wrists had linear scleroderma, and she had the sclerodactyl hands, which is basically when the hands look like swollen sausages, and of course the Raynaud's was severe.  When we learned for sure that she tested positively for Mixed Connective Tissue Disease, I learned that MCTD can stay the same, go into remission, or it could morph into either Lupus or Scleroderma.  I think I have always seen more Scleroderma features with her than anything.  I decided that I wasn't going to worry about it until we had to, which we hadn't had to do.

That said, Monday we discussed her test results from the chest CT, Echo, swallow study, and whatever the heck else we did.  If you read my post after we did the swallow study, you may remember that I was able to watch the test being done myself, and I saw problems.  The doctor that ran the testing and the 3 techs that were present didn't know what to do.  They called in another doctor for guidance.  The problem was that the barium dye wasn't going away.  They sat her up, laid her down, turned her side to side, but it wouldn't fully leave her esophagus.  Well, apparently her esophagus has narrowed down near her stomach.  Not much is able to get through, leaving her feeling nauseous and refluxing.  She is barely able to eat.  While I knew that there were problems here, I really thought that it would be the muscles from a Dermatomyositis flare, which attacks the muscles of the throat so they are too weak to work properly.  This is more of a Scleroderma feature.  That threw me for a loop.

While I was still reeling from that, he pulls up pictures of her lungs.  He shows me that they can see changes around the base of the lungs.  They look different from her last few tests.  While it doesn't show that she has Interstitial Lung Disease, her lungs are likely trending that way.  Pulminary Fibrosis is one of the hallmarks of MCTD and Scleroderma.  I don't remember a whole lot of the rest of the day.  I focused a lot on trying not to cry.

So, the new plan is to stop her Methotrexate.  She has not been a fan of that one lately, anyway.  In place of MTX, we are switching her to a stronger medicine called CellCept.  The hope is that, if we hit it hard enough, it will bounce back down.  Like, if you start to treat a flare at the beginning, it will usually respond much more quickly than if you let it go.  This is similar in theory.  I can't even find any info on this one that doesn't pertain to transplant patients.  It has a ton of warnings.  It does not look like a fun med.  Yeah, and because it causes such a high risk of infections, she may have to start a sulfa antibiotic daily with it.  It sounds terrifying.  And yet, what choice do we have?

We have a dear friend who is very similar to Emily.  We heard tonight from another dear friend that friend #1 has ditched most of her meds and is working on a holistic approach.  I have heard that she is doing very well this way.  I don't even know where to start, but I will ask them about it a bit.  It may be a while before we can afford something like that, but I will look into it.

The funny thing is that Emily didn't even have an appointment this time.  We discussed all of this in Zachary's appointment.  The bad side of that is I don't think we really talked much about Zachary.  Maybe we did and I missed it... I don't know.  The only thing that I clearly remember is that I asked if his white patches will go away from where his old psoriasis lesions were.  I had hoped they weren't Vitiligo.  I was assured that they are not; it simply takes a while for the skin to go back to normal since he had so many lesions and for such a long period of time.  And I got a new MTX prescription for him. That's all I remember there.  

 So, here we are again in territory that none of us is comfortable with, that makes it all too real.  I try so hard to be positive, but I have to be real, too.  People need to know that these diseases are out there, and they are scary and life-threatening.  I am thankful that we are homeschooling this year.  At least she won't pick up any nasty bugs at school.  School stuff will be on the Homeschooling blog.  A new post should be up this week!






          

Unexpected tests and such

I swear, sometimes I jinx us.  Just the other day I posted on new research that points a bigger finger towards developing Scleroderma for patients with Mixed Connective Tissue Disease.  The funny thing is that, while she has had the sclerodactyl hands and some wrist involvement with the skin on her wristsfor years now, it hasn't really been brought up at all by our current rheumy group.  Until yesterday.  And it wasn't that Dr. S was saying by any means that this is turning; it was more like, "we need to run these specific tests every year so that we catch any disease progression just in case it turns into Scleroderma."  Now, I know that having MCTD puts her at a much higher risk of pulminary fibrosis than almost anyone else, but this is the first time that Scleroderma has been brought up.  So, last night she had a chest CT with contrast, and an EKG.  She had an echocardiogram a little while ago, and she's supposed to be scheduled for another Pulminary Function Test.  It was just surprising.  She did NOT like the echo.  I truly didn't think it would be a big deal, but she said that the ultrasound probes hurt her. She didn't like the contrast for the CT, either.  She spent quite a long time in tears about that, despite my explaining that it was just a dye.  At least they are both over now, done for a year or so.

Leaving for the hospital, I knew that there was something important that I was forgetting.  I knew we had another concern to bring up but I could not remember what it was until after the doctor left.  Her hearing!  Lately we've noticed that her hearing is not that great.  There are many things that she doesn't hear correctly.  I know that, while it's very rare, there are actually joints in the ear that can cause hearing loss.  I believe that it could be the sinus pressure is starting to build once again.  I can't believe that we forgot about that!  I did have a chance to ask the immunologist about it (because he is the one covering for rheumatology today).  He didn't say too much about it because he couldn't see any problems.  But, he does feel that we should schedule a hearing test.  I assumed that would be the case.

The other thing that we were curious about was her current rash.  She's had one like this before.  It starts out with one or two areas that look like mosquito bites, but more keep coming.  They are just red spots.  When she itches a lot, the middle gets a little speck of blood.  They don't respond to Benadryl.  I hadn't even thought to try cortizone on them.  Dr. S agreed that it is likely an autoimmune rash.  He said that often with rashes that are associated with an autoimmune disease that is being treated, they don't look the same as they would in non-treated disease, so it is more difficult to know what to attribute it to.  MCTD'ers are more likely to break out in different types of rashes than people with JA or RA alone.  She's had more vasculitis-type rashes on her face and hands, too.  She gets spots that look like small pink blotches in those areas.  On the bright side, he was showing a medical student what he looks for when looking for dermatomyositis.  He explained that he can't show her much because he sees no active disease!  That made my day!  :)  At onset, she had the speckled capillairies, the pink elbows and knees, more spots on her hands (they didn't look like Groton's Pauples to me, but I guess they were), and she had the classic heliotrope rash around her eyes.  Right now, she looks darn good! 

I am not used to her needing Zofran before Methotrexate yet, and we both forgot about it! So at 1:30 A.M. she was still awake with a sore tummy.  The nurse called the doctor, who added Zofran in.  She was able to sleep after that, at least.  Of course, respiratory came in at 6 to give her Symbicort, along with vitals every 4 hours.  She's tired, and she just wants to go home but she's doing well.  At least now we're in the home stretch.  She's got her Remicade going now; when it is done, we are free to leave.  I had originally thought we would be home by now.  Ah, well.  At least she's got chips and TV.  She's comfy, so it's all good. 

Due to the holiday, I'm not sure when we will have her test results, but I will post more once we have some answers.

Our new plan!

Ok, so again I am completely exhausted.  Forgive me if this doesn't make any sense, but I want to get it out before I forget it all.  We had another crazy day.  Hospital time runs at a totally different speed than real time.  It really seemed like I woke up, blinked, and the day was gone.  I didn't even get time to just chill out on Facebook.  Emily got quite a bit done.  She was up early, got some TV time in, went to the playroom for a while, found a book to read that she enjoyed, played some video games, made some creations and watched a couple of movies.

I started my day by running 2 toys that were given to us to take to the infusion room.  I figured that while I was there I would get a copy of her labs.  I figured that they may be off a little, but I didn't expect them to be completely out of whack.  I was shocked at how many exclamation points there were.  (They indicate levels out of range.)  Her inflammatory markers are elevated, but with an infection that is to be expected.  Her urine was all messed up, but they said that looked more like a contaminated sample.  (Phew!)  Her liver enzymes (used to measure her muscle activity) look fantastic!  Those running amok is one of my biggest fears.  Almost everything else was off, though.  That worried me, of course.  I patiently waited for a few hours to see a doctor.  Praise the Lord, we have been through enough of this to not worry just because something looks like it may be a problem.  Problems usually seem to not be so glaringly obvious with us.

Towards the end of the day one of the doctors came in.  Before I go further, let me say that one thing that I love about our rheumy group is that they don't just treat what looks rheumatological and hope someone else will deal with the "other stuff".  Dr. E asked the Infectious Disease doctor to come talk to us.  Let me tell you- I was soooo excited to see her.  Dr. E had told Dr. V that she had ordered the Rocephin antibiotic because it's very good for sinus infections.  We explained once again how the past few months have gone, listing each antibiotic and the amount of time prescribed.  We established that I need to be more firm with these other doctors when it comes to prescribing antibiotics for an actual infection.  I admit, that's not something that I'm good at.  I feel that, while I do probably know more about rheumatology than they do, it's not my place to tell them how to do their jobs, and while I should have a medical degree, I don't actually.  We talked about that for a while.  She said that she would never prescribe less than a 21 day course of antibiotics for anyone for a sinus infection- never mind an immune suppressed child!    I will now be more firm on that. 

Despite me asking the ENT's to send over her file, despite me signing paperwork on Monday for them to do just that, they haven't done it yet.  Dr. E had her nurse practitioner working on it, too.  That said, they could only rely on what I was told by the ENT's from the first culture, which was that it was weak to Augmentin.  Knowing that Augmentin was the only antibiotic that seemed to do anything (except for a few days before her scraping when it seemed to stop working) they prescribed a 21 day course.  They also want us to keep doing IViG monthly to boost her immune system back up and then maintain it.  Yesterday's labwork showed her Igg at 435.  They usually do IViG for levels that fall below 500.  I feel a huge sense of relief with this. 

As I said in yesterday's post, we will wait two weeks to resume her Methotrexate, and then it will be at a lowered dose to begin with.  She's one of the few kids that loves her Methotrexate because it helps her to not be so itchy.  Dermatomyositis can cause severe itchiness, and even though hers is much better now than it was since her muscles aren't flaring, it's still annoying.  I'm more excited about the Prednisone decrease!!!  Again, a drop from 4.5mgs (1.5mls)  to 3mgs (1ml) is huge!!!  She'll have two weeks on 1.25mls, but that's ok.  It's been so long since she's had a decrease.  I realize that the slower the decrease the less chance of flaring so I wasn't complaining, but perhaps on the new dose she'll be able to lose some more water weight.  She is still so self-conscious.  And that should help her growth to get back to a more normal rate.

Last but not least, we had a roommate this time.  Bless her heart, the girl has Crohn's Disease.  She was given the option between trying Remicade via IV or trying a feeding tube at night with formula that would help to heal her colon.  She really thought that the tube would be a good idea.  That poor girl cried and cried, begging her mom to take it out.  Before we left she decided that she would try Remicade- despite having a huge fear of needles.  We were able to help them feel more confident about their decision, and give them some idea on what to expect.  God puts people where they need to be.  I truly think that we may have helped them a bit tonight.  I hope so!!!  I am hoping that Emily feels much better in the morning, too!

*Written at 12:04 A.M.*

Frustrated

While I try very, very hard to remain positive 99% of the time, I am far from immune to rough weeks.  This has been one of them.  This week I find myself simply discouraged.  For once,  I look around and see more negative than positive.  I hate that.  I am trying very hard to build myself back up, but it's not been easy.

I had spoken to our ENT's office twice last week.  First because Em was more stuffy than she was after the first week on antibiotics.  Granted, she didn't have a fever, but that is pretty normal for her.  She had been draining a LOT still- more than I though was normal.  They told me to just keep using saline.  I was having a hard time expressing myself with this.  I couldn't prove that she was sick, but sure seemed to be doing about what she was when she was sick.  When they called to confirm our appointment I brought it up again.  I didn't think that she could wait until today, but she did!

Friday morning I had a very hard time getting her up.  She was struggling, teary-eyed, and not very with it.  She was coughing.  A lot.  And, just when it was time to leave, my son yells to me, "MOM!  Emily is vomiting mucus".  Woot!  She spent about ten minutes vomiting.  This is seriously just not fair to her, and it's breaking my heart.  All that she wants is to get better.  She agreed to the surgery because it is supposed to help.

Today we went back for stage II of the surgery.  By this point her eyes are all bloodshot, around her eyes is bright red, she has what looks like a popped blood vessel- all from coughing.  She sounds all junky, and she's still very sniffly.  I knew that they wouldn't declare her "fixed".  I wasn't expecting more non-answers.  (Not their fault- they want scientific evidence.)  They did a culture to make sure that there is no lingering infection.  They told me today that, while she is still congested, she's not really junked-up in the sinus cavities.  The inside of her nose is still very inflamed.  He said that usually this type on inflammation is more of a systemic reaction, often from other problems like reflux.  Well, I can rule reflux out since she's on an adult dose of Prilosec as it is. 

I had pretty much figured that I would be calling her rheumatologists shortly after our appointment, but I wasn't really expecting to tell them that this may be arthritis-related.  So, I called them to tell them about the nasal inflammation, beg them for IViG (to help boost her immune system) and also to tell them about her brand new jaw pain.  I was really, really hoping this would be the result of blocked sinuses or another infection.   The ENT checked her ears, analyzed the sinus drainage, felt her jaw, and determined that this isn't his department.  Her lymph nodes are swollen, too.    Fortunately, her rheumie's office called me back tonight.  We are staying at the hospital tomorrow night for her IViG.  She didn't technically have an appointment scheduled to see the doctor, but they are going to look at her given how much is going wrong right now.  For that I am very grateful.  I just feel so helpless right now.  One thing that people don't understand is how great the risk of infection is for these kids.  She is at a much higher risk of dying from a silly little infection that healthy people can shake off without a thought.  Another reason why we strive to gain awareness: this is much more serious than people often realize, and it can be very deadly.

So, currently I am frustrated with this stupid sinus infection that is really messing with her.  I am frustrated that I don't have a medical degree so that doctor's that don't really know me will listen to me.  And I am frustrated that I can't keep her home on days like Friday when she really needs to be home, away from kids that are germy and sent to school anyway.  It makes me feel so terrible for her!  I also feel that perhaps both of my kids would be doing better in school if I could home school them.  We are working on it.  I know that things will work out.  I just would like that to happen now, please!!!

How are your kids doing?

Every day I have people ask me how my children are doing.  I truly appreciate this.  It can help me loosen up a little bit, just knowing that someone cares enough to ask!  However, here's the catch:  I never really know day to day.   Emily is the hardest to tell.  She'll complain for a week about a cold sore, but she won't complain about the fact that she can't walk.  That was exactly the case in October 2010 when she was officially diagnosed with JDM.  She'll complain about a scrape, but not because her head hurts from having her sinuses scraped.  She tends to complain more when there is much less pain.  The worse she feels, the less she says.  When you ask her how she is feeling, the automatic response is "Good".  Sometimes we can pick up cues.  Other times we truly are left to guess.

Zach is more vocal right now.  He's much more quick to tell you when something is wrong.  BUT, the worst parts of the disease are what you can't see, so how can I really know for sure?

I have also had several people lately ask me if this could be because of environmental factors, diet, etc.  I will try to make this easy to understand, but it's difficult subject matter, and the scientists aren't 100% sure yet.  Say you have an electrical circuit that is working fine.  Everything is working as intended.  Suddenly, something happens- a trigger is pulled, a lever or switch.  That opens the circuit up, right?  Until that circuit is closed, it won't be right, it will cause problems.  The immune system is the same way.  Here's the problem- the "switch" in this case is invisible.  It leaves no trace.  And you may not  even know that the circuit is open for many years.  Some people have symptoms many, many years before the disease hits.  Once it hits however... Some people are lucky enough to be well managed with just one or two treatments.  Others never seem to be able to find anything that helps.  For a small percentage a significant diet change will help and heal them forever.  Most people find no relief through diet change.  And, if it's an environmental trigger, removing the trigger doesn't help.  We had mold in our wall that went undetected until 3 out of 5 of us got very sick.  We had NO clue that we had mold.  It was hiding up in the rafters of the attic above our kitchen and bathroom.  It was removed, and I know it's gone (because I am extremely sensitive to mold).  That doesn't seem to matter.  Other triggers are bacteria like strep.  Strep and pneumonia can set off autoimmune diseases like RA.  Again, once that trigger is pulled there is no going back.

When I tell people about my kids' situations they often ask me "Are you sure you don't have mold in the house?"  Yes, but even if we did it wouldn't change this either way at this point.  Oh, it may speed it up if we still had mold, but it won't magically reverse.

My kids "good" is different from other people's.  If I don't know how to answer you, please don't think it's anything more than just "I don't know".  As lame as it may sound,  we just don't know.  Some days bring severe fatigue.  Some days bring severe pain.  Other days bring stiffness, and still others bring all of the above.  For my kids, most of the time their pain is lessened.  Em does pretty darn well.  I think it's actually more because she has gotten used to the pain.  Also, remember that a simple cold can become a huge ordeal because their immune systems are suppressed.  This means that, while you & I can fight off a cold or have a simple round of antibiotics "cure" us, this often isn't the case with immune suppressed people.  They fight and fight but it doesn't always work without a LOT of intervention.  This is our life.  We make the best out of it.

Another good rheumy appointment for both

Yesterday was a very long but good day.  Taking two kids to the hospital for 8 hours isn't really my idea of a good time.  It's rushed and crazy when you're taking one child, but two presents a whole new challenge.  Thankfully I have some really great kids, but it's still difficult.  Filling out everyone's paperwork, getting drinks and snacks, just getting settled!  I brought the laptop, foolishly thinking that I could get some work done.  In reality my son used it more than I did.  It was a great distraction since he was kind of driving me crazy.

For once we stayed at a hotel.  I didn't want to have to get up at 4 A.M. to drive up.  Not with 2 kids.  The morning went just as planned, except that I didn't sleep.  Emily's new antibiotic isn't doing a whole lot, so she was coughing most of the night, which kept me up. I was afraid that it was waking her up, but in reality she actually slept.  We got up on time, packed up, had time for breakfast and got to the hospital with time to spare.

The doctor saw Zachary first.  Despite us keeping the other staff in the loop, he didn't know that Zach had only just received his Enbrel in the mail.  He had thought that he would have had 2 full months on it by this point, but in reality he only had one full dose.  We could see that the Methotrexate is doing what it's supposed to be doing.  His jaw pain has been minimal, and the psoriasis plaques look much less angry.  Still present, but not terrible.  Again the doctor noted that Zach's hands look like Emily's... without the steroid swelling.  It really is curious.  I pointed out that the psoriasis is trying to take over his joints.  He didn't really say much about that other than agreeing with me.  We agreed to keep on the same track with him as we are, making sure we get him in to the opthamologist and have his labs repeated in two months.  His liver enzymes are up.  His ALP and Sodium are off, too.  In reading up, it looks as though a low ALP often shows a Vitamin D deficiency, and many people with autoimmune issues are vitamin D deficient.

Emily requires much more time, so he left and came back to us.  That gave me some time to call her ENT and see if we have a surgery date yet.  Apparently they hadn't thought about that yet, so they scheduled it with me on the phone.  We are set for February 22nd for the first procedure and March 13th for the second.  Our rheumy was upset that she hasn't consistently been on one antibiotic for 21 days.  I need to make sure if either of my 2 little children has an actual sinus infection that they stay on antibiotics for a full 21 days.   I pointed out the red dots that she keeps getting.  It's really strange.  He said that these spots look like spots that some people get when their white cells crash, but hers look good.  Very odd.  Thanks to the ENT's calling for surgical clearance, they ran the immune system panel.  Honestly, I feel that she should probably be on monthly IViG, but I don't want to bring it up.  I don't want to pump more into her than we have to, and she's already on a ton of medicine.  We discussed that her ankles, knees, hips, wrists and back have been flaring mildly in the joints.  Praise the Lord her muscles are quiet!!!  We also discussed her sinus problems.

So, I am blessed with pediatricians that realize that I know what's going on.  They LISTEN to me.  They may not always agree which is fine- they have the medical degree, so we try it their way.  But they listen.  Since November Emily has been on :


Septra- (Sulfamethoxazole-TMP)  January 27, 2012
Augmentin 600MG - January 19,  2012
Omnicef 250MG-      January 13, 2012  (Again did nothing but make her more sick) 
Augmentin 600-         December 28, 2011
Clindamycin HCL-    December 14, 2011
Omnicef 250MG-      December 11, 2011  (Did nothing- she got sicker)
Augmentin 600MG - November 28, 2011

When the last round of Augmentin stopped working midway through, I called and begged them to please give her one more round of a different antibiotic to hold us until her surgery.  Foolishly, I thought the surgery would be a week or two.  Knowing that Augmentin wouldn't work, nor would Omnicef, I asked for either Clindamycin or Septra.  I prefer Clindamycin for 2 reasons:  because Zach & I are allergic to Septra and because Septra can increase the toxicity of Methotrexate.  In English that means that this antibiotic could make her one arthritis/ myositis med work too well, like at a higher concentration.  So, I told them that I would be willing to discontinue her MTX if they prescribed Septra.  It is a triple antibiotic, and she hasn't used it ever, I think.  Our rheumy's biggest concern was that it wasn't prescribed for long enough.  I asked him if I should give her the MTX at a half dose, but he said not to worry because she has so many other meds going.  Of course, she actually likes her MTX, unlike most kids.  (Dermatomyositis causes severe itching, but the MTX decreased it a lot.)  Moral of the story- he gave us another prescription for 15 more days of Septra.  He believes that her arthritis is acting up because of the sinus infection, and once that finally clears she should be better.  I had figured that, too.  It's nice to be on the same page.   And that was about the gist of it.

Today, we were all exhausted.  It's a very long day, especially because first they do pre-meds, then SoluMedrol takes about an hour to infuse, Remicade takes about 3-4 hours, and Rituxan is set for 4 hours.  She's wiped the next day, but so am I.  Even Zach was wiped.  We did almost nothing today, but I did make her go to therapy.  And she cried a little because stretching is so hard, but overall she did very well today. 

I can't tell you how amazed I am with her, how in awe of her I am.  She will correct you if you call her medicines the wrong name, she will spout off the list of her meds, she will talk to you like she's a doctor, but at the end of the day she's acting like a cute squirrel that just wants to be petted.  She is truly such a joy, so sweet, so wise.  I am so blessed.  <3

ENT and Enbrel!

Ok, so just so everyone can see the crazy, up/down days that we have, I will post a breakdown of the day.  This is why we are so used to dealing with stress.

8 A.M.- The kids don't want to get up (and neither do I!) but we get up & ready to go.

9:30 A.M.- We leave and take Zachary to school.

10:20 A.M.- We arrive at the ENT.  We wait for about an hour, which is fine because we were so early.

11:20 A.M.- We get set up with the nurse.  Being a mom now accustomed to the things that doctors need I have a typewritten list of meds that Emily is currently on, a print out of all prescriptions taken since the start of the infection with the antibiotics highlighted in yellow, along with CT scans and the radiology reports.

11:30 A.M.-  The doc comes in with the scans.  He starts showing us how the sinuses should look and how Emily's are not performing where they should be.  The Ethmoid sinus looks good from a frontal view but not so much as you go farther back.  He throws out two ideas.  One is that we can do the culture that our pedi requested under anesthesia to find out which antibiotics will work best against this.  The second option is that we can do exactly the same thing, plus add on another ten minutes to scrape the junk out of the inner sinuses.  That is the best option to me.  If that stuff has been hanging out for God knows how long, it may not come out on its own, and then we'd have to get the scraping done anyway.

Noon-  We're talking to a nurse, in hopes of setting up the procedure, but with all of Emily's medicines and such, she doesn't want to do it without consulting our rheumy group.  They also want to hold off on prescribing a new antibiotic until they talk to our rheumy's, which leaves me a little nervous.

12:30 P.M.- A nice quiet lunch

1 P.M.- A quick visit to the park!  Not that she runs around, but it's nice to go be closer to nature.

2:30 P.M.- We head to my parents house for a visit.  During said visit my mom again discusses how more of her colon is blocked and she's afraid that they won't be able to do the 2nd colonoscopy.  Also, my father feels tired suddenly so he goes to take his blood pressure.  It turns out to be 80/37.  Ouch.  Mom gets him some potato chips to bring his pressure up and he's good.  I take some time to order a bunch of prescription refills.

4:30 P.M.- We head to the school to get Zachary.  He tells me how his throat has been hurting all day.  Once home I pull out my scope (don't hate) and look down his throat.  Bright red.  Great.

5 P.M.- While I really want to nap, I realize that I still need to call the pharmacy about Zach's Enbrel.  It had appeared in my history, then disappeared a day later.  It had also showed that our pay portion was $2100, so I was a lot panicked.  I had called a month ago to give them our Enbrel Support info, and I had emailed it as well.It turns out that they LOST that info.  I was absolutely thrilled to find out that his Enbrel will be FREE!!!  Best. News. Of. The. Day.  True story.

5:30 P.M.- I decide to see if my school's website could tell me why I haven't seen any more info about my financial aid.  I had electronically signed the paperwork, verified everything and returned it all.  I see NO SIGN of that.  I once had an amount that I was supposed to be allowed and now I see nothing indicating that I was ever approved.  Hmmm.  I make a mental note to call on Tuesday when my son vomits.  He hadn't told me that his stomach hurt, just like he didn't tell Daddy that his throat hurt.  Now I'm really in a panic.  Need to take yet another day off of work, make another doctor appointment.  I have Omnicef at home, but with him starting Enbrel and needing his Methotrexate on Friday, I really can't take chances.  So, I call in to work for tomorrow.

7 P.M.- I realize that I need to scan a bunch of documents relating to their healthcare and send it to the school.  I email both kids teacher's.

See the up & down pattern?  Welcome to a typical day in my life.  And I have it easy compared to the kids.  They still have to go to school, pretend that they are normal kids, do their normal work, then have their meds, their shots, the infusions and the constant doctor appointments and tests.  Thank you Lord for making children resiliant!!!

Arthritis is FINALLY getting some press!

For years now, many of our arthritis parents have felt that we needed a celebrity to speak out, to help us to champion the cause.  As Christine Schwab says in her new book "Take Me Home from the Oscars: Arthritis, Television, Fashion, and Me", arthritis has such a stigma associated with it; it is taboo in Hollywood.  People are so into their stereotyping of the disease that they try not to look at it... what little they see of it is with blinders on.  Because it is associated with aging and the elderly and Hollywood is all about being young and perfect, no one wants to acknowledge it.  This has robbed us of  money that a celebrity could raise for research.  A cure will never be found if no one is researching.

As a parent, dealing with the stigma is very hard.  You have people that don't believe you when you say that your child has arthritis.  You have people that assume that you must be doing something wrong as parents.  People look at a child on steroids... and judge that child and the parents.  It is a heartbreaking cycle.  This is why I choose to speak out.  After a year on steroids, my daughter is still so embarrassed that she doesn't want her pictures taken, she doesn't want to be on stage or in a position where people will be looking at her.  For a ten year old, what she has been going through is very, very tough.  She handles her life with a positive attitude, confidence in herself, and a deeper wisdom that a hard life teaches you.  There is something about these sick kids...

Anyway, back to my point.  Christine Schwab was asked to appear on an episode of "The Doctors" show!  She spoke about Rheumatoid Arthritis and the stigma associated with it.  You can watch her episode here.  We are so excited by what Christine is doing!  Fighting this battle for 20 years herself, she fully understands what we are going through.  She is becoming quite the advocate for our children, and we are so thrilled.  This truly is an exciting time to have RA or any form of arthritis.  There are so many people striving to gain awareness, like Kelly the RA Warrior, &  Rheumatoid Arthritis Guy.  There are new drugs that are being looked at; there have been so many amazing drugs that have been approved that are making some serious differences.  My favorite always has and likely always will be Enbrel.  No, it doesn't work for everyone, but it was a miracle for Emily.  While we continue to wait for the green light from insurance to use it for my son, we pray that it will be a miracle for Zachary as well. 

They still have no idea how autoimmune arthritis happens.  There seem to be different triggers for everyone.  Some people have severe food allergies or sensitivities.  (For those people, drastically changing diet seems to work well.)  For some people, serious infections like strep seem to do it.  There are environmental triggers.  They believe that it's like flipping a switch; once the trigger is released, the arthritis starts.

Some people have done very well by drastically changing their diet.  Most don't.  Some people benefit from glucosamine. Some people swear by long-term antibiotic use.  Most people find their relief instead from drugs like Enbrel, Humira, Remicade, Rituxan, Actemra, and Symponi.  These drugs are incredibly expensive, but they work.  For most.    But this isn't enough.  We need more answers.  We need a CURE.

Still sick!

Well, despite the 2 heavy-duty antibiotics that she's on, Emily is still coughing, sneezing and stuffy.  On the plus side, her fever is down.  Her temp was "normal" today- 98.6F.  Of course, her normal is usually around 97.1F.  She had her sinuses scanned by CT yesterday, and we have a follow-up appointment with her pediatrician tomorrow.  She actually wants to go to school tomorrow.  I have no reason to keep her home if she wants to go, but I am worried that she hasn't kicked this thing.  I made her use our nebulizer a little while ago.  She said that it did help " a little bit", but not significantly.  I don't really know what to think.    I am hopeful that our pedi will have some answers tomorrow.

A Little Good News :)

Hello!

I'm happy to report a little good news.  :)  While she's having some minor issues, Emily continues to do well since starting the Rituxan.  I'm just thrilled!!!  She says that an area of her lower back always hurts, and her wrist to elbow has been flaring a bit, but not enough for her to want to go back up on her Prednisone.  She has chosen to continue on the 4.5MG dosing instead of going back up to 6MG.  While it may not seem like a big difference, she feels it.  She is also continuing to lose some of her steroid-belly.  We don't really notice it on her, but we notice in the way her shirts fit her.  The shirts that we ran out and bought her back in November when she suddenly gained 30 lbs are starting to look more like long baggy shirts.  I'm hoping it will be no time before they look like dresses again.

I always worry about her with school.  She likes school, and she works really hard.  She LOVES to read, to write, to be creative.  She loves her teacher this year, too!  Her school is amazing, and they always put so much time and consideration into where the best classroom placement is for her.  I really couldn't have her in a better place than where she is now.  This is the fifth year in a row now that her teacher has told me that her class "mothers" her.  I was told last night at open house that her classmates rally around her.  I have worried often about her being teased or bullied because of the Prednisone weight.  While it has happened on a small scale, she has yet to come home to me in tears.

Also at the open house, they told me how shocked they all were at how well she is doing  :)  Her school physical therapist had a goal for her to climb 5 stairs.  Thanks to Rituxan, she's pretty much given up the elevator.  (I forgot- she did say her knee hurt last night, but not enough to change med dosing for.)  They are all so impressed over how well she's doing.  I'm so glad.  Last school year looked so bleak for her; I didn't think we'd be able to keep her in school.  What a difference a small change can make, even when it looks so scary at first.

She is still stressing over her port placement.  We have talked about it every night now.  Just talking seems to be helping.  Before she told us what was wrong, when she was trying to tough it out, she was spending a lot of nights in my bedroom.  Tonight we discussed how God provides for our needs, we discussed what "Let go, let God" means, and how to accomplish it, and we talked about the phrase "What would Jesus do?",  thanks to my son.  I am so happy that they love church!  Emily especially can draw strength when she's down or feeling bad.  I'm thrilled that she has come to love our Lord!

We could use continued prayers if you would.   We have her port placement on Sept 27th.  It will be a very long day.  Please pray for a great surgeon, for their hand to be guided and their mind as well.  Thank you!

The JA Conference! (Long)

I know that I touched on this a bit in my last post, but I wasn't able to give it the love that I wanted to.  I'd like to start by thanking the Arthritis Foundation.  I cannot express to you how much they have changed our lives.  We would not have been able to attend this year if it wasn't for them.  They have done so much for us!  We try to give back as much as we can, but I will forever feel in their debt.


The JA Conference this year was near Washington D.C., in a Marriott in Crystal City, VA.  The hotel and its view were both beautiful!  The conference started on Thursday.  We didn't arrive in D.C. until after the first evening's festivities were mostly wrapped up.  We did have the opportunity to attend the first networking sessions.  Those are very important.  They broke the parents into mom & dad groups, and the kids were with their own ages.  It's so cool to get together in a large room filled with other people that are going through exactly the same things as you.  This years added appeal for me?  My Facebook groups!  I am so blessed to have found these wonderful ladies (& a few gentlemen) on Facebook.  Each and every single one of them is amazing in their own way.  Many have raised a staggeringly high amount of money for the AF.  Most are trying to raise awareness, some are trying to start non-profits, some are donating to their hospital's children's rooms.  Some are dealing with extremely sick children; others are just trying to get by as single parents with chronically ill children. Each one is amazing in their own way.  This year as our group is nearing the one year "together" mark, many of us were able to meet in person!  It was truly a huge blessing meeting these people.  (Have I stressed that enough?  You just couldn't know unless you went!)  I truly felt at home, excited, energized, and with my family.  It was a beautiful weekend!  Not nearly long enough.  I soooo wanted more time with the Amy's & children  :)  I need to get out more.  I don't get out enough, so when I do I am completely overwhelmed.  I don't know where the time went!  I don't think there could possibly have been enough time to chat with all of these people the way I wanted to.  Love you all! 

Friday-  The speakers during the opening session talked about some of the CARRA group's research and clinical studies.  One of the speakers was Norman T. Ilowite, MD, the principal investigator of  the RAPPORT study.  This study is extremely important to the parents and children affected by Still's Disease, a systemic form of Juvenile Autoimmune Arthritis, or SJIA. This session also addressed the importance of participating in and supporting clinical trials.  

We had our choice of where to go for the next round.  We chose "Other Pediatric Rheumatic Diseases", hosted by Dr. Natasha Ruth.  This session addressed different forms of vasculitis, Lupus, MCTD, Dermatomyositis, and Kawasaki's Disease.  We learned a lot in this session, including what some of the bumps on Emily's hands may be from, that you can have a +ANA and not have an autoimmune disease; we learned about central nervous system disorders, and we learned the Emily should have an Echocardiogram yearly.    I also asked a question that has been bugging me for a while during this session.  To me, through all of my research I believed that, while many people with Mixed Connective Tissue Disease may have myositis symptoms, from her initial onset I truly believed that Emily had developed the DM as a separate disease.  I also believe that she did at some points have myositis features, before she developed the full-blown disease.  I asked this doctor if that was, indeed, possible.  She agreed that it sounded likely in our case.  This means that, while having no confirmed systemic features right now, Emily has 2 systemic disease.  (I later confirmed with Dr. Rider, also.)


The next class that I went to was "JA Perspectives Panel".  There were several speakers here, all of which either had JA as children or have been parenting children with JA for a significant amount of time.  This is a great time to ask questions about what the future may potentially hold, or how to handle certain situations.


The next class that we went to was "Medications Used Commonly in Children with Rheumatologic Diseases", presented by Dr. Sandra Hong.  OMGosh!!!  If I was ever able to just move to follow a doctor, I would follow her.  I love our rheumy's, I trust them, I know they have our child's best interest at heart.  However, this little lady is a huge ball of energy!  She looks about 25 (gotta love Asians!), and she spoke like a teenager.  This would create obvious appeal to the kids. She was so cool & down to Earth!  Kevin & I totally loved her.  He didn't go to this class with me, but we had a pow-wow in the hallway the next day with her.  She wanted to make sure that we didn't have any questions, that she discussed what we needed to know about, etc.  She gave us some really great advice on meds and tests to keep in mind.  She was seriously awesome. 


Saturday brought an opening session on advocacy, as well as an awards presentation.  We had somewhat of an inside view into the health care plan, how to make friends with your representatives, how to gain approval for your cause, etc.  This was very important information since we really don't have enough people meeting our politicians advocating for our kids.  This was hosted by Kevin Brennan & Calaneet Balas.  


We chose to go to "To Rest is to Rust" next, hosted by Jennifer Horonjeff, MS.  We missed half of this session as we ran into Dr. Lisa Rider, co- author of  "Myositis and You: A Guide to Juvenile Dermatomyositis for Patients, Families, and Healthcare Providers".  I was sooooooo excited to meet her!!!  I discussed another CARRA group research study with her, "Rituximab in Refractory Adult and Juvenile Myositis (RIM) Study".  This was huge to me because our rheumy had asked us Wednesday to consider trying this.  I was able to speak with several people that have tried this, parents of children that are on it, and Dr's Hong & Rider.  This conversation with Dr. Rider helped me to make up my mind for sure to try this approach.  She was so cool, so brilliant and so sweet!  She told me that I could always call her office with questions and she would be more than happy to help.  How many doctors would make an offer like that?  I really appreciated this time with her.

When we finally got into the session we were headed to, it was mostly over.  However, we caught enough to figure out that we don't really fit into a category at the moment.  There are a lot of things that Emily can't do because Prednisone can cause ostopenia (brittle bones).   The speaker was sooooo cool!  She was another twenty-something that I really hope also spoke to the teens.  She was pretty awesome.  She was very funny & animated and used visual aids to help make her audience understand.  

After lunch we chose to head to "JIA & Jaw Issues" with Dr. Randy Cron.This brought up some scary points, like most children have jaw involvement and don't even know it!  Up to 81% of JA kids have jaw issues.  This can cause the jaw to not grow properly, causing deformity with a "short" jaw.  Most docs don't think to order an MRI, and if they do, most radiologists don't know how to read them for children.  How astonishing is that???  We missed a good chunk of this class speaking to Dr. Hong, but what we caught was rather unsettling in content but very informative!  Excellent speaker and session.


Our last session of that day was on "Community & Financial Resources from A-Z".  Honestly, I knew a lot of this already.  There were a few good links that I wrote down, but I had already researched this area a lot.  I thought maybe I had overlooked something, but I guess not.  For a parent new to having a sick child, this would have been a huge help.


Overall, we learned so much!  It was so good to be able to keep Emily & her brother together.  They were in the same room as some other Florida children so Emily felt a bit more comfortable than last year.  (It was a mistake bringing her to Philly without her brother.)  Also, we didn't know that she was more sick at the time.  It was a few months later that the puzzle pieces came together.  It was good for my son to be with other JA kids.  They had a session with Mr. Byron Janis, pianist, songwriter & Psoriatic Arthritis patient.  I was really excited about this since my son has psoriasis with no arthritis.  We don't know anyone else with psoriasis that is his age, or even with psoriasis as visible as his.  


This was also a fantastic time for my oldest.  At 16, I thought that she may be bored.  Apparently, she did, too.  Not so much  :)  She asked if she can come back next year.  I don't think that she has really understood all that her sister goes through, either.  I believe this experience helped her to "get it" more.  I'm so glad that we were able to go as a family.  Everyone really needed this.


One more note on the AF- recently a good friend was laid off.  We all know the economy is horrid.  Arthritis is so misunderstood and under-appreciated, & while the AF has very few paid employees, it's hard to keep all of them right now.  While we are sure that he will do very well in future endeavors, we know how much this person did for our children.  It was his heart that helped many of us in Florida come together.  Between Facebook and planning days for us to meet, he got us together.  He started the "Mom Squad".  This was hugely important for me.  This made me feel I could be more proactive than reactive, & I was making a difference.  I'm hopeful that this program will continue to grow, but I'm sure it won't be with as much love and nourishment as he provided.  And he waited until after the conference to tell us all so we wouldn't be sad there!  How sweet is that???  We are very grateful to have had such a kind, loving heart working so diligently for our children.  I won't name names, but he knows who he is ;)  I know he will remain a friend on FB and we'll probably meet up again, but I will continue to mourn the loss of his presence with the AF.  I will continue to do all I can, maybe even more now.  Thank you for all of your hard work for us.  Luv ya! 













 

A few changes

Before we headed off to the JA Conference, we had to stop at our hospital for Emily's infusion and doctor appointment.  I expected a few questions to be answered, a quick appointment and quick poke since she's done so well lately.  Nope. 

The first poke by her favorite vampire, Jason,  got them absolutely nowhere.  That was enough to bring out the tears.  He called in Ariel who thought she had it, but didn't.  They tried the 2nd arm to no avail.  They decided we should use a little more Emla cream and hit up her hand.  She hates that.  They tried and again failed.  We discussed what would happen if we skipped it, which I really didn't want to do.  That would just mean going through all of this again in 2 weeks.  Finally, a nurse helping in the ped's room got it.  Thank God!  That brought the doctor around asking us if we had considered a port.  Well, in truth, I had.  I had been waiting for them to bring it up.  I have my mind made up, but Emily is harder to convince.  I think it will take her a few more months to grudgingly accept.  I hate having to go through the stress, tears, the trauma of having her poked monthly.  To me, it's an easy answer, but since I want her to feel that she has some control over her health, we will wait a bit.

The other thing that I really wasn't expecting was for her to bring up another med.  This really stressed me out a bit at first.  Thinking about picking a new "poison" is always scary, but there aren't very many options sometimes.  Emily has been very tired and her joints are flaring badly a week or so before the next infusion.  She's also been sick a lot.  Our doc suggested that we try Rituxin.  Rituxin kills the "B" cells.  These cells are cancer causing, so it would decrease her risk of cancer, but they also kill off the immune system even more than her other 3 immune suppressors.  To address that little side effect, they will also give her regular IViG.  I'm a big fan of this med.  It's actually taking the platelets of the blood of 2,000 donors, mixing it together, sterilizing, and voila!  You have an organic med that will help to confuse an over-zealous immune system.  I'm for it.  I realize that some people get debilitating headaches from it, but I also know that there are different brands, and if you can try a brand, you will likely find a way around side effects.  Our docs are big on giving IV steroids (Solu-Medrol) with each infusion.  This helps to eliminate side effects.

Needless to say, I was thrilled to get to the JA Conference and talk about ports and Rituxin.  I was a little sad that the Rituxin reps weren't there; I'm assuming this is because it's not actually FDA approved for young children yet, but we're pretty much out of options.  I did, however, have the opportunity to meet and speak to several parents and pediatric rheumatologists about this, as well as get some input from parents in my Juvenile Arthritis Friends group and my JM Moms groups.  The general consensus?  We're going to try it.  I'm not sure what else to do now, to be honest.  Right now, Emily is definitely doing better than some of the kids, but she can't play.  She isn't in agony all of the time like some of the systemic kids, but she does have a lot of discomfort and she's definitely got pain daily.   Her quality of life right now is far from ideal.  Her endurance is very low.  Yes, I still consider us blessed!!!  While MCTD is far from ideal, I'm happy that she doesn't have a terminal disease. 

Since Emily developed the Dermatomyositis on top of the Mixed Connective Tissue Disease, I've often wondered is this what they refer to as "myositis features" that comes with MCTD, or is this its own, primary disease.  I was able to ask 2 doctors this.  I have always felt that, while she had myositis features before (I think from looking at her labs) I truly felt that the DM manifested as a primary disease.  She had a high fever for almost 2 weeks, the rash developed around this time, her weakness was very severe; she couldn't roll over in bed.  Usually with MCTD the patient would have just features, not the whole disease.  Everyone agreed that with an onset like that it is a primary disease.  I really wanted it to be part of the MCTD, but I feel it's important to be honest with ones self.  I also feel that as long as I know the truth, we can find help.  While it wasn't something I wanted, it's good to know.  3 kids in a million have DM.  The odds are similar for MCTD, though I don't know actual figures.  That is really like winning a bad genetic lottery.

We learned a LOT from the conference!  The best part was feeling like we were at home.  I love my FB family!  I have met these people on Facebook, but we all come together because we understand the situations that others have in a way that outsiders couldn't possibly understand.  It was so cool to meet so many amazing people in person... and feel like I've known them forever!  The conference helped my oldest to better understand things, too.  It was really a wonderful weekend.  It's great to swap stories, talk medicine, and ask whatever burning questions you have.  The sessions are set so that newcomers aren't totally confused, but there's enough content to keep a knowledgeable family's interest.  We truly appreciated this special opportunity.  It will probably be the last trip that we take for quite some time, so we didn't take any of it for granted!   

Sharing Em's Raynaud's story

About a year ago I discovered The Raynaud's Association.  I was desperate for personal stories that would help me to figure out how to help Emily.  At this point, the Raynaud's was the worst.  Her arthritis was held in check with Enbrel, and it was before the Dermatomyositis kicked in.  This was the time for her Raynaud's to really make a spectacle of itself.  At this point, she couldn't go to a pool- even a heated pool- without turning purple and being in much more pain that before.  She couldn't wear dresses or skirts.  She had to keep mittens with her, and heating pads, constantly.  This was a truly difficult time, trying to figure out how to prevent her from getting frostbite at school again and other such things.

I had started to really get into blogging at this point.  I wanted to write to them after seeing that they were looking for creative ideas on how to cope with the condition.  I was also hoping that we could help some with my husband's rice sock idea.  And that some people would understand because they had been in our position, and would perhaps reach out and tell us how they coped.  Well, much has changed since then.  Raynaud's has been on the back burner since our new rheumy gave us Procardia.  I had begged our old rheumy for a calcium channel blocker but he didn't think a little frostbite could hurt, I guess.  He said it wasn't necessary.  Well, 2 nurse practitioners and 2 other rheumy's think he was wrong!  They have her Raynaud's written up as "Significant"... and they didn't even see a flare, though I did show them cell phone pics.  They said they didn't need the pics because they could see it in her hands from a mile away.

Anyway, the whole point to this?  My story turned up in the Raynaud's Association's newsletter!  I actually got the link last month, but we were so busy then getting ready for our Make A Wish trip that I forgot about it.  I got a Twitter text tonight with the link for the current edition, & I actually remembered to go back. 

Also, I found out that a chiropractor 2 minutes away sells BioFreeze.  We truly love that stuff, so I went over to go buy some for our elbows & pulled muscles, etc.  Being a Sunday, the doc was in but had no staff.  We got talking quite a bit about nutrition, inflammation triggers, other autoimmunes such as Celiac's disease, and gluten sensitivities.  I haven't had a chance to check these out yet, but he told me to look up PubMed, where posted articles are peer reviewed, and also Deflame, Your resource for reducing inflammation and pain with diet and nutritional supplementation.  Again, I haven't had a chance to check either page out except to grab the links, but I accept the possibility that they could hold great value.  :)  It's certainly worth looking into the Deflame diet.  I could use some inflammation control myself for my Rosacea.  It sure couldn't hurt!

Not at all as I expected

One thing that I have learned in our journey so far is to take one day at a time.  I try not to have so many expectations, because you really just can't predict autoimmune diseases.  Usually, just as I think I have things figured out, I get knocked down a peg or two. 

I was really looking forward to Emily's infusion this month.  I knew that she really felt that she needed it since her wrist & elbow were still bothering her, but I also had a long list of questions.  Usually, kids with Dermatomyositis are seen every 4-6 weeks.  It's a rough disease, very hard to get under control and can suddenly swing in the opposite direction quickly.  We weren't seen last month, so I though for sure she would see a doctor this time.  I knew that they had changed the way they schedule patients, but I hadn't realized that they were scheduling the kids for IV's and not telling them that the docs weren't in.  I made sure that they will be in the next time we are there.  I wanted to talk about her flaring arthritis, her inability to eat more than 2 bites again now that she's on lower prednisone, and I wanted to see about repeating the pulminary function tests since Em totally didn't even try last time.  It was the day she was being released from the hospital and she just didn't want to be bothered.  Now she's been sick more in the past 4 months than she has been ever since being on the immune suppressors.  Maybe it's the Prednisone.  I don't think so since they increase her doses when she's sick.  I really wanted to talk about all of that, and a patch on her head that may be psoriasis.

Em did very, very well with her treatments.  It was lovely to walk in to friends saying hello  :)  Emily went off with her favorite nurse to have her needle... BY HERSELF!!!  I was shocked!  This was the very first time that she didn't cry, whimper, or even need me at all.  I was so proud!  And all the while she was talking his ear off.  Unbelievable.  The rest of the infusion went without anything noteworthy... until we were leaving.

I had asked the nurse's for a copy of her labs.  I knew that the doctor's office would do that, but I didn't know the infusion room nurse's would.  The copies they gave me showed that day as well as results from the month of May.  They didn't show high/low, so I had to take them home to compare them with my copies from the doctor's office.  Those copies do show ranges.  We had gotten her muscle enzymes down.  Last month they shot up quite a bit.  This month they are only 2 points away from being in the high range.  I had been watching how pink her knees had been getting, but she didn't seem to feel a problem so I didn't worry about it.  At least it's summer break, but she shouldn't have to go through this.  I know our docs are doing everything within their power.  I know this is a crazy hard disease to get under control.  I just thought that maybe something would be easy.  Just this once.  So, I'm keeping a sharp eye out and trying to figure out what to do.  I'm sure the answer will seem clear in a day or two.  Frustrating.

Well, we have Cub Scout day camp in the morning and it is way late.  Have a good night!

We're back!

The week was crazy, wonderful, exhausting, exhilarating, beautiful.  We got back home on Saturday, earlier than expected, but we were all so tired.  Though it had its rough spots, for the best part the week really was a wish come true for Emily.

I've been too preoccupied to post.  I'm still trying to sort things out in my head.  So much ran through it this week!  So much happened.  So many memories made.  Give Kids the World Village was a wonderful, beautiful and amazing place that I'm having trouble putting into words.  Before I formulate all of that, let me say tonight that I am very grateful to Boston Market, Perkins restaurants, Breyers ice cream, Hasbro toys, Walt Disney World, Universal Studios, Sea World, and the many, many volunteers that help to make up this amazing community.

The story of Give Kids the World can be found here.  I was first told this story while in the pool with other families.  We were enjoying watching all of the different children, some in PVC crafted wheelchairs especially for the water.  It was amazing how many different families were there, yet there was a feeling of kinship and understanding here.  The volunteers come from all different walks of life.  Some are high school students doing their community service projects.  Some are college kids earning credit.  Some are retired folks, occasionally coming from different states to spend their vacations helping these families.  And many are employees of the theme parks.  We had figured that the theme parks may feel we were a burden- getting free tickets should be enough, right?  No, they totally understand that they are giving some children the hope to beat their diseases.  Some children will cherish these memories as they fight long, life-long battles.  And others don't have time for any other wishes.  They know the tolls that having sick children can take on a family- mentally, physically, financially.  These theme park employees would sometimes come up to us and ask us how we were doing, how we were enjoying our stay, and all mentioned how they loved the time that they volunteered there.  Many are still active volunteers.  Others can't fit it in right now but look back fondly.  I cannot begin to express to you how amazing a community this place is.  And they think of everything!

On our first day we walked in to find a toy in our villa.  (They left toys daily for the kids.)  The villa was larger than our house.  The kids bathroom had a jacuzzi and a huge handicap shower. We were given coupons to take to the Star Tower to make "dream pillows" for the kids, and also to place Emily's star.  Since some of these little people lose their fights, they decided to give them a star to place up on the ceiling that would stay forever.  This would always keep a part of them in the village.  I believe they said there are 150,000 stars up in the main area, and they expect the new star tower (only opened 3 weeks ago now) to be full in 6-9 years.  A star for every sick child that will be placed, and with the guidance of a star fairy, always found by the child or their family if the child passes away.  What a beautiful concept!  They want your child's week to be "a week of yes's".  



Though she felt fragile this week, camera shy still due to the steroid weight, and flaring a bit, Emily still managed to have a wonderful week of dreams come true.  We laughed, we made new memories, we had fun, we enjoyed the rides but also the nature around us, the animals, and feeding the animals!  My favorite moment?  Watching my girls feeding the dolphins.  My son was busy pouting because he wanted to go ride a roller coaster, but my girls enjoyed the experience for what it was- an amazing experience and momentary connection with a dolphin.   I wanted to purchase the actual Sea World photos, but with the crowds of people trapping us then moving us along, combined with the drama that my son created, we somehow forgot to go look at them.  This is my consolation prize  :) 

I shall give a real update soon with actual details of the trip.  Let me say now that Gir seriously almost killed herself before we even got into our villa.  She tripped on a bag while trying to exit the van.  I saw her feet in the air flying as she went out the door.  She scared the heck out of me!!!  I was terrified, but she was somehow totally ok.  She was bruised up all over, and she bit her lip but otherwise she was fine.  She was very, very lucky.  I was worried that may set the tone for the week, but it was surprisingly calm and peaceful.  More to come soon  :)

Thank you, April. Much needed!

April has probably been one of the best months we have had in quite some time.  April has been filled with good news, and happiness over things to come  :)

April was kicked off by our camping trip with our Cub Scout family.  I had really been looking forward to this weekend as a little vacation.  I was hoping that Emily would be ready to run around with her friends, but she actually wanted to be with me for 98% of our time there.  The first night I think we kept everyone awake :)  She has some, ummm, flatulence issues.  Honestly, my little princess can let 'em loose better than any guy I know.  Our first night in the tent she had gas so badly that she woke up her brother, and across the campsite her farts were heard.  Kevin got a text saying "Gas gas gas :)"  and we all about died laughing!  This was around 4 A.M.  We had a few people come over to the tent to see what all of the commotion was about.  We were hysterical!!!  What a great day that was.  I love being out in the woods, and being there surrounded by family (including extended!) was really cool.  We took a nice hike the next day.  I wish I didn't have to push the wheelchair, that Emily wouldn't need the chair, but I'm so happy that she was able to come because of it.  We saw some beautiful plant life along the way, & even stopped to grab some wild blackberries!  That weekend was just what I needed to feel back in tune with reality.

Though it's not a huge, noticeable difference yet, Emily is definitely losing some of the steroid weight.  She is so excited about that!  She keeps telling me about different things that she can do, like button her pants more easily.  I see a huge change in her attitude this month, likely due to the last steroid decrease.  She has more energy, she wants to do more, she has some goals set.  Because of this she is happier, doing things like walking more and playing Wii Sports again, and finally she is back to loving her brother like a friend!  That has been the hardest part of all of this.  She & her boy were always so close; suddenly she was cranky towards him more often than not, yelling at him and just being mean to him.  I'm so glad they are playing together again.  As Kevin said recently, harmony has been restored.  It's the little things that we've missed, like that.  She actually wants things again!  I never thought that would make me happy!  Yes, truly April has been a turning point.

She's doing well in school, too!  She had the 2nd best grade in her class on her Time test.  That is exciting partially because she missed that chapter & she worked extra hard to catch up!  For a while she didn't care at all about her school work, or anything else.  She's really determined right now, & I'm so thrilled to watch it happen.  We had a fantastic IEP meeting in school for her.  They are increasing her time with the school Physical Therapist, trying to pull her out during PE since she has such big limitations in there.  She can do low impact things like walking, but nothing that could potentially cause injury as the steroids can cause brittle bones.  Her teachers are very proud of how far she has come this year not only with her school work, but also with her attitude.  She used to have "math breakdown", but she seems to have moved past that.  We are very, very blessed that our school is as caring as they are.  Before placing her in this class they really stopped to think & talk about where the best place for her would be.  They were absolutely right to place her in this class.  She isn't just taught; she is cared for.  Obviously, any parent would want their kids cared for in school, but when it's a child that has dealt with so much, that really needed to be nurtured and loved, it is much more appreciated.  I love these teachers in a way that I don't think they could understand.  You just couldn't know how much their love for her has meant to me, or to her.  They have impacted her life in a huge way.  Truly that is God watching out for us, trying to make some things easier.  They have been a gift.

Our Make A Wish volunteers called just the other day to say that we have been approved for our Disney Make A Wish trip!  We are all so excited!  All that I need to be happy is to watch the kids be happy; that makes this a dream come true for me.  I was so happy that as soon as I hung up with our volunteer, I burst into tears.  The kids kept asking me what was wrong :)  I was just so happy.  There have been so many things that Gir has wanted to do, but we haven't been able to accomodate her because of time or money.  There are many things that this trip will address for her.  And it will be wonderful for my dear, sweet little man.  He's such a good boy, so tolerant & sensitive.  He needs something really good.  My mom told me the other night that he was telling her how things were going so well that he was worried about what was going to go wrong next.  That's how things roll around here.  I swear, it's always things that we could never have controlled in a million years.  But hey, life is never boring!

More good news is that Kevin went for his yearly physical to find that everything looks good!  His labs are where they should be, too.  He is getting better slowly but surely.  We go on our trip at the end of  May.  My hope is that by then Kevin & Emily will feel so much more like themselves.  I want them to both feel good & have a great time.  This trip needs to be magic, especially for Em, but for Kevin & the kids, too.  I am so excited that I could burst!  May is also our arthritis walk, too  :)  We look forward to that every year.  It's so nice to be with people that understand you, even if you don't know them.  I'm sure that I'm forgetting to mention a bunch of things, but I've covered the big stuff now.

I found a blog post that really hit me.  It made me stop and think about how Emily must feel.  Here is the "Arthritis Angels: A Walk In Someone Else's Shoes" post. 

Hey, it's spring!  Don't forget to look around at some of the beautiful flowers, including weeds, that have popped up  :)  I truly believe that these are gifts to us, meant to put things into perspective.  Will you stop to smell the roses today?  You really should....