Our last hospital visit. Ugh.

I've had a few days to ponder and mull now.  I still feel a little numb, and just exhausted.  My Facebook friends know most of this, but I didn't want to leave anyone out.

I had emailed the nurse practitioner at our rheumies office last Monday, in preparation of our visit that Thursday and Friday.  She spoke to the doctor, and we all agree that she needed a new swallow study done.  Emily had told me that she feels like there is a bubble in her throat when she tries to swallow.  Hmmm.  Since she had started to get stuffed up again (on antibiotic number 2 for the month), I mentioned that, as well as the headaches she has been having.  I assume they are either from the sinus infection from hell or maybe from the growth hormones.

I wasn't going to tell her about the upper GI until right before.  I asked for Xanax for her.  She never uses that stuff except for medical testing.  She just freaks out.  It's bad.  I guess that everyone thought I was overreacting.  Nope!  It was supposed to be outpatient at 11, but knowing how difficult it would be to get us out on time, they made it inpatient at 11:30.  She woke up on Friday morning to the nurse telling her it was time to go downstairs for the test.  She got herself so worked up that she almost hyperventilated.  She cried for a good hour.  She didn't calm down until someone finally got her a 1/2 a Xanax, but it was too late by then.  She was rescheduled for 3:30, though no one told the scheduler downstairs.  We were discharged at 1:30, so we had time to kill.  We couldn't really leave, and since she couldn't eat, we tried to avoid all of the food places.

They told me to give her another 1/2 Xanax around 3.  Despite having one full Xanax in her, when they took her back she still had another hour meltdown.  I can't blame her.  She has no control, no say in any of this.  It sucks.  If I didn't suspect it was bad, I never would have pushed.

After she finally calmed down, she worked with them to help herself.  She asked to be slightly elevated.  I watched the barium start to go down, then back up, several times.  At one point, I thought the camera was showing something else, or glitching.  Nope.  What it basically showed is that she has, as the nurse practitioner emailed it, "Her study showed severe reflux and little motility of her esophagus."  Hmmm.   I suspected as much, but it still hurts to see it.  At the moment, that is really all that I know.  This is in conjunction with her patulous esophagus, which if you missed it, means that the muscles at the base of her esophagus have dilated and spread away from her stomach.  .  Well, this explains why she doesn't eat.

At the same time, she has spent 30 days on Augmentin and 10 days now on Clindamyacin, and she is still sick.  She had an x-ray last week that showed she is still gunked up, so the ENT is working on getting an authorization from rheumie to get this expedited so they can culture her and suck out the junk up there.  I really, really think she needs to be on a prophylactic antibiotic.  Then maybe we could stop IViG, and maybe not have to sleep over the hospital every month.

But, wait!  There's more.  I have noticed for the past couple of months that her ALT (muscle enzyme) has been a tad elevated.  I'm no longer the one to panic at the first sign of trouble, so I've just been keeping an eye on it.  However, Monday her thighs hurt badly- like a 6-7 on the pain scale.  That could mean that her dermatomyositis is also coming back, which would mean adding Prednisone back in.  We really, REALLY don't want to do that.  It makes me so sad.

If all of that wasn't enough, they ran her cortisol levels on Thursday morning before she had any of her hydrocortisone.  A normally functioning adrenal system will have a count of 10 or higher.  She was only at 1.3, so the hydrocortisone stays for now.  And the final kicker was the email from camp that she is on the wait list for both sessions.  At least we had a few good months.  Hopefully this will all pass quickly.  I guess we need to find a set Gastroenterologist now.  We've seen a couple before, but mostly as favors to our rheumies.  We don't really have anyone set.  Hopefully endo will be able to help her more with the cortisol, and ENT will see her fast to help her sinuses.  Please pray that all of this comes together just right.  Thank you.

Three years of JM and MCTD

It was September 2011 when we first realized that Emily was very, very sick.  We had gone to her former rheumatologist who had kind of given her a diagnosis... without any solid evidence to prove it.  After blowing us off and telling us to come back in three weeks, we begged a new rheumy to see her.  It was October 13, 2011 when we got the definitive diagnosis.  We didn't get home until late on October 19th.  Six days of hospital time- upper GI, MRI, lots of labwork, EKG, PFT, daily steroids and anti-inflammatories, and a lot of sadness.

Since then, we have been through a lot.  We had the whole Prednisone double-the-weight-in-two-months thing happen, which was horrible all around.  It caused major depression for her, and ripped our hearts out as we watched her eyes swell shut.  We have found out about her throat muscles not working due to dermatomyositis, and then later we found that the scleroderma features are causing the esophagus muscles to spread, leaving a gap between it and her stomach.  We have found out about early changes labeled as mild interstital lung disease.  I saw that at our pediatrician's while reading the newest note they had sent over.  We've had low cortisol and adrenal insufficiency, which contributed to causing kidney stones.  As yet we have not resolved any of the above, with the exception of the stones.  At this point, her pain continues to increase, likely because her pain amplification has come back.  Her medicines just keep increasing.  Below is the list of meds and vitamins that she has to deal with daily.  I print this every time we see any doctor.  I print triple copies when we have a hospital stay.  You never know what kind of a resident you are going to have!

Despite this, we still have hope.  Hope that one day she will be in remission.  Hope that there will be no more progression.  Hope that her pain levels will come down, as they continue to be an 8-9 (on a pain scale that goes from 0-10).  Hope that she won't need to use one of those electric carts whenever we have to go to a big store.

We had an issue the other night at a big box store.  I usually try to avoid this place, but I promised my oldest daughter that we would upgrade her phone there because the AT&T stores no longer carried the one she wanted.  We went in, all of us dressed in business clothes since we had just come from my honor society induction.  We chose an electric cart to help Emily get through the store, and we set about on our way until a greeter stopped us to tell us that she wasn't allowed on the cart unless she sat on my lap.  I pointed out that they were there for disabled people, and she was a disabled child. She said that children were not allowed to ride them regardless.   I am not quick to anger (except maybe at work), but I was livid pretty quickly.  I asked to speak to the store manager, or a manager on duty.  I offered to go to the car and bring back our handicap permit, but Emily decided to show off her port scar and educate this woman.  She claimed that it was company policy, but she did not call the manager for us.  Instead, she told us that she would "let us go this time".  Nice.  I wrote on their Facebook page, and their corporate Facebook, who directed me to a feedback link.  I know you can't see her illness, which makes this our job to educate.  We have never had an experience like this before, and we hope to never have another.  I ended my post with "my child has likely suffered more in the last three years than this greeter has her entire life".  And it is so true.

All in all, we are managing.  For me personally this year has been pretty amazing, but it really sucks to see my little one suffer.  It just shouldn't have to be like this.

_________________________________________________________________________

Conditions:  Mixed Connective Tissue Disease, Dermatomyositis, Juvenile Arthritis, Raynaud’s Phenomenon, Scleroderma, Vasculitis Rashes,  Esophageal Dismotility

Current meds- last updated October, 2013

*Prednisolone- (15mgs per 5ml) Take 0.5 mls in the morning

Hydocortisone 5MGs- Take one tablet twice a day

Prilosec (Omeprazole) 40mgs One capsule daily.

Procardia ER 30MG (Nifedipine)- One pill once NIGHTLY. (For Raynaud’s)

Mycophenolate 250MG (generic CellCept) 3 pills A.M. (750mgs), 250mgs P.M.

CVS Iron  65mg- 1 pill once daily

Zyrtec- 10mgs nightly

Amitriptyline HCL 20MGs- Take one tablet nightly at bedtime.

Symbacort 160/4.5-    Two puffs twice daily  (as needed- through winter and sickness)

Sucralfate 1GM- Take one half  tablet twice a day  

Xanax .25 mg- Take 1 half of a tablet as needed for anxiety

Lidoderm 5% patch- apply as needed 12 hours on, 12 off  ******** NEW*********

Multi-Vitamin & 2500 Citracal 400mgs calcium with Vitamin D 500 IUs– once daily

Vitamin D- 1000 IUS, 2 fiber gummies per day

***IV Infusions:***   

Remicade 325mgs monthly

Rituxan 400mg- Every 3 months

IViG Gamunex 10% (15 grams)

Finally! An October spent inpatient ONLY for meds!

It was October 13, 2010 that Emily was diagnosed with Dermatomyositis, and officially diagnosed with Mixed Connective Tissue Disease.  That week, she was kept inpatient for six days for heavy-duty steroids and other meds, along with a plethora of tests to rule out other not-so-nice diseases.  It was last September 26th that she had the port placed, and it was early October when we realized she had staph in there.  That led to an almost month-long stay.  It broke her heart to have to miss the fall carnival at school and Halloween.  Granted, she was able to trick-or-treat at the hospital, but it just isn't the same.

I am happy to say that this October the bad chain was broken; we had our typical overnight for meds, then we were free to go home!  Thank you, Lord!

I didn't mention Zach again this time to our rheumy.  I believe last month was just a fast psoriasis flare, likely due to the change in weather.  He has a small spot or two, but for the most part it isn't big enough to concern us.  I'm keeping my eye out, and I know he will tell me if anything changes.  We are planning on using the Body Check Journal to see if we can establish patterns for both kids.  If you have ANYTHING that you are trying to figure out (rashes, erratic behavior, allergies, flares, etc) I strongly recommend that you try this journal, lovingly made by one of our favorite arthritis mom's.  She has discovered so much about her daughter by doing this herself, which is how she thought to make the journal!

Em's appointment went better than I thought it would.  Our rheumy was sick, and it was a bit hard to understand him over the noise and through his mask, but we decided together not to make any changes yet.  He said that studies show that Rituxan, Orencia and CellCept in combination are proven to help things like her throat issues, but it isn't something that we can do for more than a few months.  He doesn't want to backtrack before giving the combo a fair shot.  Since it's only been 2 months since she's been on CellCept, and 5 or 6 for Orencia, he wants to be patient for just a bit longer.  I'm good with that.  The biggest fear is her throat, so if this combo will help her throat the most, we will wait.  Monday and Tuesday she received her Orencia (30 mins), Rituxan (4 hours) and IViG (over 12 hours).  Wednesday is the day for her to recover from meds, and for me and Zach to recover from stress, and lack of sleep.  Poor little man has a cold, too.

It is crazy how much has changed with Em in the past month or two.  When people ask me how she's doing, I have no idea how to answer.  Here's why:  yes, the throat problem is pretty big, and scary.  However, she has been running around, trying to hoola-hoop, and she has been giggly.  Really giggly.  She sounds.... happy!  On the flip side of that, for a few weeks there she was taking 2-3 baths a day.  Usually she does that when she's hurting or stiff.  I have no idea what to make of it, but I take the running and giggling as good signs.  It's been so awesome to be home with them more!!!  I expect them both to be more giggly when we change our homeschool program.  We are just worn out from the amount of work they have around other things, like therapy.  We found a new program that I suspect will be the winner for us.  I know two families that use it and absolutely LOVE it.  They have similar situations, so I think this will work well for us.

The steroid weight has started dropping more!  She started steroids at 42 lbs. (and really under weight.)  Two months later she was 75 lbs.  Today she was 60.5!  Her clothes are falling off of her.  I had to go buy her new jeans.  She fit into her FAVORITE pair of old pre-steroid jeans!  So exciting!  I realized tonight that if she is wearing a large shirt you can't see her belly anymore.  This is helping with the activity level, I'm sure.  Oh, I am so glad!  Next month I will ask about the next decrease.  Currently she is on 3mgs a day.

Speech came into our room this morning for a consult, but she was so tired that we couldn't wake her.  Our doc wanted them to see if there was anything they could do to help her swallowing issues.  Being the skittish type, she was afraid of talking to them anyway.  I will have to psyche her up for it next month.  Another thing that was supposed to happen was an ultrasound of her hands.  This is more for the doctors benefit for now, but I suspect that it could have long-term benefits.  He is doing an informal study on ultrasound for Scleroderma hands.  I was unable to get specifics out of him, but hopefully that will also happen next month.  I would like to have the opportunity to help with some of the science involved; being a test patient is great when it's painless!  It may even help her one day.

We are talking much more formally around the house about going gluten and processed sugar-free.  We are going to try!  I expect that it will take quite some time to make the full change, but it may well be worth it.  We shall see!  I certainly want to try.  Since getting into making our own household cleaning products, I have been looking into making moisturizer, lip balm, shampoo, soap and laundry detergent.  I am hoping that this may help.  It can't hurt!  

Last thing to add is that we have a blog guest post up! The Arthritis National Research Foundation has made it their mission to highlight stories like this.  It made my day to see this made it up!  And I believe that is all.  I am so exhausted to I cannot recall anything else.  I am heading to bed.  Have a wonderful day ahead!

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!






          

Tough day

While most days I can manage to get through without seriously contemplating the kids' medical issues, for whatever reason, the drive to and from the hospital is where I really feel the stress of the situation. Even now with Em having a port, it is easier but once we're there and ready for them to access she really panics. Just thinking about the fact that they need to access my child is freaky, isn't it? I guess it's because going makes it all more real. Funny, really, since I'm with her when she's miserable or can't walk too, but that isn't as real as hanging at the hospital. Even now I have days where I mourn for my sweet tiny little bean pole that isn't anymore, thanks to steroids.  It seems awfully unfair sometimes, but we move on.

This trip started with the Pulminary Function Test. Now that it doesn't scare her, she actually enjoys it. I thinks she's a little crazy but hey, at least she does it without trauma. She did very well doing the test, & while the doctor hadn't seen the final report, he said the preliminary report looked great. The tech scared me a bit; he asked me with a concerned tone in his voice if we were seeing her doctor that day.  It was the way he said it that made me think, "uh-oh", but it really does seem to be ok.

Later, things were okay in the infusion room after she was hooked up. She screamed bloody murder first, but she calmed down soon after the needle went in. I guess she will always have major anxiety. I really had hoped that it would get better, but it just doesn't.  She screams like they're trying to kill her- flailing her arms and all- until the needle is in; then she relaxes.  She got her Orencia first, soon followed by her Rituxan. That is a huge relief since Rituxan is what helps her the most. I finally got to speak with someone about scheduling the swallow study. Naturally when they called me back, Em was screaming and I missed the call, but I managed to get them while still in the infusion room. They wanted to schedule the study for the 17th, when we already have appointments up that way on the 21st & the 27th.  If they weren't 2.5 hours away that would be fine. She hung up & called me back to tell me that they could do the swallow study while she is inpatient, but it needed a different referral than the outpatient one. That led to another round of calls because the check-in desk at the hospital doesn't document the way that the other staff does, so I actually had to call the doctor's office even though she sees them there. The funny thing is that not long after the person I left a message for found us in the infusion room. She said she'd find out & let me know what was up. That's the last time I spoke with her. That's cool- as long as it gets to the doctors I don't care. Long story short, they scheduled it for Thursday. That would have worked much better if pharmacy had sent her pre-meds up a few hours earlier. Instead of starting her IViG around 6, it was started at ten.

The plan was to finish the IViG as inpatient, then move to the infusion room for Remicade, while hopefully squeezing in the upper GI somewhere. Eventually they got her scheduled for her testing at two. They also ordered an x-ray for her arm because for once, Ms. Careful fell out of bed onto her arm! I really thought that she broke it. She never gets this upset!  And yes, I was pretty ticked off about that.  She fell while leaning out of her bed to pick something up off of the floor.  I had just told her that if she needed anything to let me know, and the nurse had just walked out of the room.  She'll make sure to ask next time, I'm sure. 

So they tell me that the plan was upper GI & x-rays at two, then Remicade. I pointed out that this would allow her 2 hours for a five hour infusion. (4 for Remicade, one for Solumedrol.). That is how we dropped Remicade & decided to go back to Enbrel. Yeah. Personally, I think it's a better move. I don't think Remicade has been as good for her as Enbrel was so I'm good with that. And we had talked about dropping it after if the Orencia worked for her, but we still have another month or two before it would be considered fully in effect.  That's why the Enbrel is back- probably.  He wants us to wait to see if she will really need it.  Again, I'm good with that.  Enbrel seemed to do more for her before anyway, but also this will allow us to go back to simple 5 hour infusions on the months that she doesn't need Rituxan.  That is awesome because staying overnight isn't really too much fun for anyone.  Benlysta was also thrown at me as a replacement for Rituxan.  I have read up quite a bit, and while I still have a lot more research to do, I think that we will stay with Rituxan.  Rituxan is an awesome medicine for her.  She feels it wearing off about a week before it is due, which is why they discussed switching.  From what I've read, people that do extremely well on Rituxan don't seem to do nearly as well on Benlysta.  It's like a downgrade.  Why go backwards? 

And then there was the upper GI.  She totally freaked out for it. She wanted x-rays of the arm so she was perfect for that but she was scared to death for the rest. The doctor wanted to reschedule but I begged them to do the test.  She just got herself so wound up.  I knew that if we put it off this would only happen again, but probably worse.  I know how she is, and she would be thinking about it every day for the next month until it happened, and then she would have had more time to make it worse in her head.  It needed to happen today for that, because she's been having a hard time eating lately, and because I feel that she's starting a Dermatomyositis flare.  I've seen the red dots reappear, I know she's had some muscle aches lately.  Knowing that she had the "air-filled esophagus" on the chest CT at least helped me to prepare for this.

So, I don't have any official results yet, but what I can tell you is that there is something going on. For those that haven't done this before, all that she had to do is swallow strawberry flavored barium while under a big camera.  The camera is like a video camera; they can watch the barium flow through her esophagus and into her stomach.  The problem is that it really didn't.  Yes, some flowed down, but quite a bit didn't.  And some of what did go down passed out of her stomach, but much of that stayed, too.  They called in an additional doctor to ask what to do.  In the end, we waited about ten minutes and they took one last picture of the barium that was still in her esophagus and stomach.  Is it from JDM, MCTD, possibly Scleroderma or from something different?  Not a clue.  I hope to find that out soon, along with what to do about it.  I'm just so glad that we did the test today.  I just knew that something was wrong.  I really hope that we don't have to go back up on her steroids.  That would really kill us.

We were told that we could go home right after, but we waited around to make sure that the x-rays didn't show a break.  I asked them to give her some pain meds, so they gave me a prescription.  Since they wrote for something that my CVS doesn't usually have, I filled it there.  They told me 30 minutes.  No worries.  After 45 minutes of waiting, I asked them if there was a problem.  They didn't have the correct insurance info right there.  So, instead of calling the cell phone number that I wrote on the scrip, they figured they would just wait until I wandered over there, then they told me it would be another ten minutes.  I was so mad!  I didn't say anything but I was fuming.  We ended up getting our meds as we were leaving, with me pushing her chair and carrying a suitcase, with 2 pillows and a bunch of our bags hanging from the chair.  (Laptop, sleeping bag, blankets, etc...)  I was not happy.  But we're home.  I am so glad that we're home.  Hopefully we will find out more soon.  I will keep everyone posted.  Prayers would be wonderful, please.  Especially for her throat issues and for no flare to hit.  Thank you to anyone that prays for us.  I am off to dreamland now.  I will leave you with the words to a Natalie Grant song that Emily loves off of Natalie's "Relentless" album.  The part that I have in bold I just can't even sing.  It tears me up every time.

"Our Hope Endures"

You would think only so much can go wrong
Calamity only strikes once
And you assume this one has suffered her share
Life will be kinder from here
Oh, but sometimes the sun stays hidden for years
Sometimes the sky rains night after night
When will it clear?

But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged

How do we comprehend peace within pain?
Or joy at a good man's wake?
Walk a mile with the woman whose body is torn
With illness but she marches on
Oh, 'cause sometimes the sun stays hidden for years
Sometimes the sky rains night after night
When will it clear?

But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged

Emmanuel, God is with us
El Shaddai, all sufficient
We never walk alone
And this is our hope

But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged

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.

Favorite New Site

     Today I was lucky enough to stumble across Rheumatology News, a website devoted to research articles of the different rheuamtic diseases.  I was pleased to find that they have a section on Lupus and Connective Tissue Diseases.  While hunting there, I found this article, whose author believes that MCTD is a subset of scleroderma.  While I would love to say that the author is wrong, I believe that she is correct.  I have told several people in the past that I see more Scleroderma symptoms in my daughter than Lupus symptoms.  She has significant hand involvement; her hands are very affected by Raynaud's, arthritis, Scleroderma features (including the sclerodactyl look.)  Her wrists have been affected by Scleroderma in the past, also.  She has had some issues that I don't know how to classify, or how to attribute.  While I don't put a whole lot of stock into research anymore because much of it turns out to be wrong,  I also know that this is far from a fully- proven piece, and there is a lot of research needed to explore this theory deeper, it is something interesting to think about.  What I'm really hoping is that this research will get more funding for some of the diseases in this spectrum.

      This is what I remember reading when we heard the words "Mixed Connective Tissue Disease" for the first time.  "Some studies have found that patients who originally were diagnosed with MCTD often over time develop predominantly the features of one disease (such as Scleroderma or Lupus)", writes Coburn Hobar, and Arnold Postlethwaite.  (See footnote). 

The Lupus Foundation seems a little confused on the matter, but this is still a good comparison between Lupus, RA, Scleroderma, Ssc,Vasculitis,  the Myositis diseases and Sjogrens. 

While looking for statistics, I found this awesome slideshow, presented by Janet Pope for the 2006 Scleroderma National Conference.  While a bit incomplete on a few slides, overall it is excellent for anyone that does not have a basic knowledge of Scleroderma, Lupus, RA, MCTD or Poly & Dermatomyositis. 

 My searching wasn't only limited to MCTD today.  I was first led to an article on Rheumatology News  called "New Anti-Inflammatory Drugs Will End Anti-TNF Dominance., (by one of my Dermatomyositis board mom's) and then I found this article on a new study drug that is showing strong promise for psoriasis.  It does not mention psoriatic arthritis, but I imagine if the drug is similar to Enbrel that it will likely work for PsA.


*Coburn Hobar, M.D., Rheumatology Fellow, and Arnold Postlethwaite, M.D., Professor of Medicine and Director, Division of Connective Tissue Diseases, University of Tennessee Health Science Center, Memphis, Tennessee, and Department of Veterans Affairs Medical Center, Memphis, Tennessee (originally published in "Scleroderma Voice," 2003 #1)

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....  

International Autoimmune Arthritis Movement Awareness!

Please feel free to share this info!  These facts came from IAMM's Facebook Page.  You may also visit their main site here.

Can a baby have degenerative, wear and tear arthritis in his/her joints?  Of course they cannot.  The commonly used term “arthritis” refers to Osteoarthritis, a condition where cartilage around the joints becomes thin due to wear and tear, age, or injury.  A baby has not lived long enough to endure this kind of damage.  A child’s form of ‘arthritis’, therefore, is of an autoimmune nature.  Autoimmune Arthritis is an autoimmune disease which causes the body to attack the healthy tissues of the joints, connective tissues and surrounding areas (including organs). 

So before you say, “that person/child/baby is too young to have arthritis”, realize it may be a different strand of arthritis that is not caused by aging.  See Stacey's amazing awareness video here.

Osteoarthritis, a degenerative arthritis often referred to as simply “arthritis”, is caused by age,  wear-and-tear, or injury.  Autoimmune Arthritis (Rheumatoid Arthritis, Psoriatic Arthritis, Lupus, Scleroderma, Ankylosing Spondylitis, Still’s Disease, Juvenile Arthritis) is caused by the immune system mistakenly attacking its own cells, causing inflammation in connective tissue, joints, and organs.  Autoimmune Arthritis is NOT caused by degeneration, age, weight, or malnutrition.

“Arthritis”, defined, means joint pain.  The term “arthritis” refers to Osteoarthritis, a degenerative, localized joint condition caused by wear and tear, age, or injury.  Symptoms of “arthritis” include joint pain and some inflammation, but rarely swelling.   It is localized to the weight bearing joint that has begun wearing down.

Autoimmune Arthritis (Rheumatoid Arthritis, Psoriatic Arthritis, Lupus, Scleroderma, Ankylosing Spondylitis, Still’s Disease, Juvenile Arthritis), is a systemic autoimmune disease.  Symptoms of Autoimmune Arthritis also include joint pain but there is massive inflammation which often causes swelling.  Systemic arthritis, unlike degenerative arthritis, is not localized; it can affect any part of the body at any time, even at rest.  It is often accompanied by low-grade fevers, rashes, flu-like symptoms and debilitating fatigue.

Do you know that over-the-counter pain relievers, such as ibuprofen or heating creams, used often to treat the symptoms of degenerative arthritis (Osteoarthritis, often referred to as “arthritis”) are not the same medications prescribed to treat Autoimmune Arthritis (Rheumatoid Arthritis, Psoriatic Arthritis, Lupus, Scleroderma, Ankylosing Spondylitis, Still’s Disease, Juvenile Arthritis).  Autoimmune Arthritis medications include anti-inflammatory, disease modifying drugs, including low-dose chemotherapy treatments. 

Total average cost to purchase a month supply of ibuprofen and other topical ointments:  $18.

Total average cost to purchase Autoimmune Arthritis medications:  $2,500* (*number may decline based on insurance coverage).

Osteoarthritis, commonly referred to simply as “arthritis”, is caused by degeneration.  This wear and tear arthritis (unless caused by injury) affects weight bearing joints and can take years to cause damage.   Once the cartilage begins wearing away from the joints, it is referred to as “arthritis”.  Pain begins once damage occurs.

Autoimmune Arthritis, an autoimmune disease, is caused by a problem within the body’s immune system.  This type of arthritis affects any area in the body that contains joints or connective tissues (including organs).  Pain often begins in the form of an unexplained injury, for it mimics that of a sprain.  However, outside of the pain, it is often paired with an unexplained fever, flu-like symptoms and massive fatigue.  Because blood work may return normal for several months or years, diagnosis becomes difficult until the damage can be seen on an x-ray or MRI.  The pain begins often years before damage occurs.

Very good appointment!

I apologize for not updating sooner.  My little bout with a sinus infection has become a battle.  I have been feeling kind of like I was hit by a truck.  Since I ache too much to sleep, I shall write  :)

This past month has been a turning point for Emily, so I knew that this appointment would be good.  Before as the steroids were so high and their effects hit her so hard, she had a rough time adjusting.  I can't imagine how she was able to deal with the dosage that she started out with- physically or emotionally.  Back in October she was started on 42mgs.  She had 21mgs in the morning and the other 21 at night.  With this appointment we continued on the taper that we started months ago.  They dropped down to 6mgs this month!  I had expected it would drop to that.  Last month when they had us take her to 12mgs for 2 weeks & then 9mgs for three weeks, they told us they would likely drop her down to 6mgs this month.  They also told us that this is generally around the time that you start to see the steroid weight drop off.  That is huge because it is so uncomfortable.  It is as hard for her to bend over as it is a pregnant lady, and her center of balance is off.  She still has a long way to go but she knew that it was drop off because she can fully open her jaw now!  That was yet another thing that had made her sad, & she has been waiting for the day where she could finally open her mouth again.  Last visit they said that her muscle enzymes were better than they had seen them, and normal for the first time.  This time they were even lower!  That's huge.

They did decide to increase her Remicade for next time for her joints.  Her back has been bothering her enough so that she wanted to get her infusion.  I still expected the usual fear that grips her the day before, that causes a huge crying jag that night and has her silent and terrified the whole way over.  Usually she barely speaks on the way.  This time she was in a really good mood, really ready to go.  She watched "Tangled" on the ride and was even singing along to the songs!  I haven't really heard her sing since September.  I think she knows that right now it's all downhill, so to speak.  I try to hard to keep her educated and in the loop.  She understands that this is what I've been telling her would happen.  She told me the other day that she's really glad that we switched to Shands.  I'm thrilled that she's happy there now, even if she does have a way of yelling at Dr. E every time we see her.  I'm not sure why.  I think that she resents her still from keeping her in the hospital, even though she knows it was for the best.  I'm just happy that I can finally really say that she's doing very well.  She does still have a long way to go, but she's not flaring right now.  I love how she went from never telling anyone that she was hurting to telling me every little ache and pain.  Sometimes it's silly things, but I'll take the silly if she's willing to talk about it.  She's acting more like herself, talking to her brother like he's her best friend again, really trying to tackle school with a new enthusiasm, & she's really excited about Make A Wish.  I am so, so glad that we have that so she has something amazing to look forward to.  I truly believe that has a LOT to do with how happy she's been lately.  At her lowest points it gave her something happy to think about.  What a huge, huge blessing that is.  I know that we have a long, possibly difficult road ahead with her disease.  It could stay like it is, it could turn into full blown Scleroderma or Lupus.  Or it's possible it could go into remission.  We have to keep a vigilant eye out for signs of pulminary hypertension & other systemic signs, but right this minute she's the best she's been in a while.

There are many other children out there with arthritis that are not doing so well.  Little GS has so many struggles with his vision.  They actually had to put a drain in his eye.  His family has been fighting for his vision for years.  DSW has had more pain from arthritis that they can't control that she uses her wheelchair now way more than she should have to, but they are stumped.   PL has been trying to be pain-free for three years now, & they still don't know how to help him yet.  H&J are still looking for the meds that will help them, too.  These are just the kids in our state.  There are too many kids suffering silently.  These brave little heroes need a cure.

JA Picnic, Make A Wish

After I got home from work, I laid down to watch TV, and woke up 5 hours later!!!  (Oops!)  The great part about it?  It's 4 A.M., almost everyone is asleep, and I now have time to update without everyone talking to me at once  :)

Emily & Parker

These 4 got along so well!  My son has a huge crush on Dakota, and Emily & Parker clicked quickly.

This has been a wonderful week!  Starting with last Saturday, (which I realize is now more than a week ago).  Last week, we made the trip over to Orlando to join up with the Arthritis Foundation's Juvenile Arthritis Family Picnic  :)  Unfortunately, Kevin was still radioactive and in isolation so he couldn't go, but I packed up all three kids.  Gir thought that she would be bored.  I knew better  :)  I knew that there would be some older siblings there.  It is so awesome to be able to get the kids together with other kids that are suffering in ways similar to them.  The beautiful thing about this is that when they get tired, no one bugs them to keep going.  These kids understand each other in ways that most other kids can't.  If they can't run, no one is going to name call, or harass them to get their way.  The kids had a wonderful time connecting.  It was really cool to look around and see the ways that certain people clicked with others.  My son followed around little Dakota, the older woman for him  :)  Little Carolina & Cole clicked as well.   They are both so little that it was absolutely adorable watching them!  I tried to get pics but totally missed the opportunity.   Emily made a special friend, also.  I had wanted Emily to meet Parker for quite some time.  I just knew that they would click.  They seem to have similar problems at the moment, too.  Em is really shy; she wouldn't just go talk to him.  Somehow, they finally got talking!  They chatted away for quite some time.  They really understood each other- so important right now.     While the kids were busy playing and getting to know each other, the adults had the opportunity to chat and compare notes.  I had met most of our mom's before, but it honestly felt like I knew them all so well.  (Thank you, Facebook!)  It is so important to share your triumphs and frustrations with people that understand!!!  Heidi brought books, including one that she had made based off of her blog!  (Pretty cool, huh?)  She also brought a few classics that got passed around.  Some were for kids and others were for us adults.  When this is what you live and breathe but don't get to talk about much to people in your normal daily life, this is very important.  There is so much sympathy from family to family, children and parents alike.  Those that are going through a LOT still think about other that are going through less.  We have an amazing extended family now that I am so blessed to have met.  Some great things really do come out of bad situations! 

Proving how quickly things can happen for these immune suppressed children, Heidi's daughter got sick on the way home.  She ended up going to the ER the next day and staying at the hospital for four days.  You would never have had any idea that she was ill watching her play.  We all know that often you can't tell with our kids.  These little people are so used to feeling badly that until it gets very, very bad they can't often tell that they are sick.  Of course, I think we all panicked over this.  Worrying for her, hoping she would be all right sooner rather than later, but also hoping that our own children wouldn't get sick.  For some it would be just plain disasterous.  

I had such a wonderful time on Saturday that I actually felt relaxed for the whole week.  Maybe having a three day weekend helped, too.  I have been in a fantastic mood all week.  By Wednesday night I was feeling a little edgy, waiting for the Make A Wish volunteers to come.  On the one hand, I am so thrilled for Emily because just by telling her about, her attitude is better.  Not that she's been unpleasant.  Not by any means!  It's just that she seemed to have no will to do anything before.  It was so sad to always see her sitting at scouts alone while everyone was playing.  They weren't excluding her; she was just too unhappy to want to play.  Just thinking about her wishes has made her happier.  Having them come over just made it real to her; she knew that I was truly following through with what I said.

When our Make A Wish volunteers came, they came bearing gifts for all of our kids.  That is so important.  I'm so happy that they try to make the siblings feel special, too.  I try so hard to love them all equally, but of course there are more demands from Emily.  I have to spend more time with her.  No, we can't just go places like other families can.  It depends on how well she feels for the day, if we need the wheelchair, how easy it will be to use the chair, etc.  Most of the time we are home.  It takes a toll on the other kids.  I can't tell you how many times we've had to cancel plans or change them at the last moment because of health concerns.  Anyway, one volunteer sat with me and we filled out paperwork and talked about the future.  The other one sat with Emily and together they decided on her wishes.  (They do it this way so that they make sure that the child understands that it's almost anything they want, and not what other people want them to do.)  For her wishes Emily did ask for her Disney trip, or a hot tub, or to be a princess.  They have them pick 3 in case they can't do one or two, or if the doctor vetoes the plan.  It really didn't take long, and I think that Emily feels really good about this.  It is so exciting to think about!  Our rheumy said that they almost never turn down a Disney request.

I truly hope that the Disney one gets granted.  That is what she wants most of all, and of course that is what all 3 kids will enjoy most.  I had thought that it would be only Disney, but apparently it's a week in Orlando with tickets to any parks that you'd like to go to.  I have heard that they make it absolute magic.  No waiting in lines for most things, a MAW volunteer guides you through the parks, and often everyone goes out of their way for the MAW families.  There's also a Cinderella meet in the castle.  She would love that!

I am also using this as incentive to get her moving.  I told her that while I don't mind taking her chair, I don't want her to have to use it every second we're there.  I am hoping to use this to build her endurance back up a bit, and her muscle tissue, too.  It's amazing what we'll do when there's something to look forward to!  I'm hoping that this will also help her to burn off some of the steroid weight.  This week we decrease her dosage to 9MGs daily!  I can't wait until we're on 6MGs- that's where they say the weight will really start to drop off.  One wonderful thing about the steroids is that she finally eats!!!  This is the kid that wouldn't eat much of anything, who wouldn't try new things, who ate like a bird.  Now this is the girl that carries one lunch menu around in her backpack, has one next to her bed, frequently reads them in an anticipatory fashion, and talks non-stop about food!  Before we tried everything to get her to gain weight, but now we may have to worry about her gaining too much!  It's better than being 42lbs at age 9, right?  She's earned a new nickname:  Sharky!  :)  She's so funny!  We are soooo blessed!  She's had a few joint issues, but nothing major.  Her Remicade is increasing at her next appointment.  Hopefully that will do the trick.  My only worry is that she should be seen the first Tuesday of April but it will now be the 2nd Tuesday because all of the docs will be out of the office that whole week.  She usually starts to hurt the day before, so I hope this doesn't result in a flare.

I have got to get to bed!  Have a wonderful Monday!  :)

Our latest crazy update

Where does the time go?   I feel as though I haven’t had an extra second for days now.  Holy frustrating week!!!   I thank God that I have a 3 day weekend coming up.  I really need some down time, & some fun thrown in! 

Monday started the week with Kevin going in early for his one big radiation pill.  We knew that he would need his own bathroom, and that he was supposed to eat using disposable stuff.  No contact.  Yeah, that was the start of all of the other things that they should have told us but didn’t.  He got home from the hospital as I was trying to get the kids off to school, telling me that everything we were told was just the tip of the iceberg.  He couldn’t be within 6 feet of anyone.  Like, they asked him if there was another bed within six feet, even with a wall separating.  If so, that person in that other bed could be in danger.  Doesn’t that give ya’ the warm fuzzies???  Better yet, anything that he wears or sleeps on has to be stored away after his seven days of confinement  for a full week before it can even be washed.  Wow.  His plates, forks & cups have to be thrown away in their own bag and stored away for a week before we can put it out for the garbage truck.  And he has to carry a card with him (once he’s allowed near people again) saying that he’ll be radioactive until May 21, 2011.  He will set off Geiger counters.  (He has a half life!)  Yay, huh?  Yeah, we had no idea what we were getting into.  This gave us both this grim, scary feeling about it.  It’s been very surreal as he put it.  It’s freaky.  I know it’s necessary.  I know that this is a precaution; I’m trying not to get too absorbed or freaked out by it, but it’s scary.  One funny that came out of it... one of our wonderful, beautiful children expressed their disappointment last night that Daddy wasn't actually literally glowing.  The kids had expected him to glow like a Christmas tree.  LOL!!!  He slept through the first 3 days for the most part, but I think he's recovering now. 

That Monday night, Make-A-Wish was supposed to be here.  Our volunteer has Lupus.  She’s on Methotrexate & steroids, just like Emily is.  She panicked when I called her to ask her if they’d like to reschedule.  We rescheduled for this upcoming Wednesday.  She didn’t think she could be around the radiation.  That made me worry, so I called our rheumy to make sure that I didn’t have to move Emily into a hotel for the week.  I also realized that Tuesday was our Gainesville day, & I had been expecting Kevin to be able to take Zach to school.  I realized that I would have to leave before I could even drop him at school, and there’s no way that I would be back in time to pick him up.  I asked my parents to get him, but dropping him off here wouldn’t be a good option with no one to take care of him, so I kept Zach with us when we went to Shands.  It was a longer drive than usual.  Construction and the endless “are we almost there, Mom?”’s kept me busy.  That boy had me so busy while we were at the hospital that I didn’t have a spare minute.  Usually the time drags by. I totally expected to catch up on Facebook, but I didn't have any time at all to.

Her appointment was AWESOME!!!  They seemed amazed by how well she's doing.  For the first time her muscle enzymes are down!  When she was admitted in October, her ALD was a 15.1.  The normal range is 3.3 to 9.7.    This week Em’s was a 9 -- within the normal range!    I was supposed to get a copy of her labs, but we all forgot.  We have more of a decrease plan for her steroids!  I am so thrilled that by the end of the month she will only be on 9mgs daily, and after her next appointment it should be down to 6mgs!  Her attitude has been better with the decreased steroids, as well as the prospect of Make-A-Wish. That has helped her to feel better.  They said that she will be on steroids for a total of 2 years, but from next month out it should be very low, maintenance  doses.  They said that at about 6mgs you see the water weight start to drop off.  That will help a LOT!!!  Her elbow has been hurting her, and she’s had that sternum pain lately so we’re increasing her Remicade next time, but I’m comfortable with that.  We discussed sun rules, since sunburn can actually cause a disease flare (which would NOT be good!), we discussed shampoos.  For whatever reason, the steroids make her scalp dry, which is normal, but her hair is very oily.  They say that’s weird- usually the hair dries out and starts to break off.  I thought that was interesting.  To make things even better, they were able to get her IV with one poke, and get a ton of blood from her on the first try!  I’ve been telling her that it’s the steroids that were making it hard;  I think she finally believes me! 

Last time she went, she felt well enough the next day to go to school & therapy.  This time… no such luck.  I had panicked a little bit, trying to figure out how to make all of these things work again.  Thankfully, my work is very understanding.  I was able to stay home with Em yesterday.  Heck, I won’t lie- I needed it, too.  I wouldn’t have called out for myself, but Emily not feeling well offered me a chance to breathe & vegetate.  Also, Kevin hadn’t eaten all day Tuesday while we were at the hospital because he’s afraid to touch anything.  I can’t take care of him the way I’d like, but I can kind of help him out that way.  Jeez, had we known how complicated this was going to be he’d have just stayed at the hospital.  Or I’d have taken the week off.  We truly had no idea.  So that has been this week’s roller coaster.  I hope you’ve enjoyed the ride.  Now please get off before you start to lose it, too!!!  J   We’re trending up.  I can feel it.  We can laugh about it now  :) 

What a crazy week!!!

Well, probably 2 weeks is more accurate.  Has it been that long?  I guess so!

I shall start with getting Emily's chair.  You know that things are rough when you're excited about getting your child a new wheelchair.  However, this opens us up to be able to do several things that we haven't been able to do.  Things like, go for a long walk in the woods.  The new chair can handle that!  Go to a fair or other place that would have us walking around a lot.  Granted, we had a chair before, but for one it was "an old people's chair".  She never complained, she just didn't feel comfortable.  Also, there was no restraint.  She felt like she was going to fall out.  So this was very exciting!  We are hoping to be able to go to the Renaissance Fair when it comes to town.  We will definitely need a good chair for that.  (If we wait until March 20th we can renew our wedding vows- just in time for our 15th anniversary on April 5th!)

Kevin had the appointment made for him to go in for his radiation, only to be told that the doctor that made it didn't know the whole situation, and his thryoid counts weren't low enough.  Our doc canceled the appointment, telling him to get more labs done on Wednesday.  Of course, being without meds while they wait for levels is making him feel like the human slug- sluggish, swollen, forgetful, cranky and sore.  Not too much fun.  He's holding up well, but only because there isn't really much of a choice.  He forgot to do labs on Wednesday, and by Thursday we had a sick boy.  DangerBoy stayed home from school Thursday & Friday both, so Kevin didn't go to the lab until Friday.  (By that point our boy felt a little better and wouldn't be contagious.)  We're hoping that they will call tomorrow to schedule him.  We shall see!

My father went in for the angioplasty to find that it wasn't a stent that he needed.  I was very concerned because so many other things around this time seemed to be falling apart for other people.  It almost seemed taboo.  It turned out that, in his words, there was a kink in the artery.  ?????  I don't get it, but that's what he said.  I've asked him to find out the medical terminology so that I can research it.  The good news is that he's fine!  My mother had taken the info to give me at first, but she messed it all up out of nerves.  She told me there was a 40% blockage but they weren't going to do anything about it, and that he had to stay overnight because they had given him something and had to watch him.  Yeah, they gave him a hole in his groin and a six inch metal wand to go inside of the hole!  It was a very confusing, stressful day.  By the grace of God, he's home and doing great now!

Next came the letter from Make-A-Wish foundation!  They sent a parent packet so that you know what to expect, what we will need to have, and so on.  We still had some questions, but we figured we would wait until they called to ask.

I believe it was the next day that I watched a pedestrian get hit by a car.  I have been trying to find some news on her condition, but so far nothing.  The day that it happened I held it together really well.  That's the great thing about constant medical chaos and perpetual stress- you handle trauma very well.  The next morning I couldn't get the images out of my head.  Thank God I didn't see the entire thing.  I watched her cross the road most of the way, then I turned my attention to the traffic light.  I heard the noise, looked up and saw her flying.  I know I didn't have to stop- technically I didn't see the whole thing, but I knew enough to be able to tell them that the driver couldn't possibly have seen her.  She didn't seem to realize that the turn lane didn't stop.  She just ran right into the car.  It turned even more nightmarish after her husband came over, obviously distraught, and yelled out to the paramedics that she was one month pregnant.  Yes, I know it could have been worse, but it was horrible.  Like a scene out of "Final Destination".

Friday & Saturday this week were unremarkable!  I love those days...  Sunday after work was wonderful!  We went over to Kevin's aunt's house for his grandmother's 93rd birthday.  Poor Grandma now has poor memory added to the poor hearing & vision, but she's so sweet, so full of stories.  It was a very pleasant evening spent with family that we really don't see often enough.  We always have such chaos.  It's work, or medical or kids.  We're just so busy!  It's hard to find the right balance, but at least we make some time. 

Lastly, tonight a Make-A-Wish volunteer called to make our appointment!  I went into my room to talk to her, knowing that I would break down a little talking to her.  I asked her- just to clarify- if our doctor had sent her blessings already.  Just to be clear.  I had assumed so, but this is so, so good that I didn't want to get Em's hopes up only to have them crushed.  She assured me that yes, we are cleared.  I cried.  I know that this will give her something to look forward to, and then later it will give her lifelong memories.  And they will take good care of her, and our other children!  Emily has her heart set on going to Disney World here.  She's always wanted to fly so I've been trying to convince her to fly to Disney Land if she wants to do Disney, but I also told her that it's her choice & I don't care what she wants to do.  If she is dead set on Disney here, there is another fantastic non-profit that I discovered on my Juvenile Myositis Facebook group.  Give Kids the World focuses on completely spoiling the family during their stay.  I've been trying to help her to understand that she can pick ANY wish that she desires.  Some kids have gone to Puerto Rico, some to swim with the dolphins.  Others have met their favorite star, gone to an event, traveled to Oregon in an RV, had a bedroom make-over.  As long as it's something that makes her happy we're good.

I'm not sure where we stand on her health right now.  She said the other night that her arm hurt, but she couldn't tell if it was her elbow or her upper arm.  Today it was definitely the upper arm, in the muscles.  Her legs are good though, with the exception of the rash flaring up again on her knees.  Today it looked almost purple.  Most days it's puffy pink.  I'm taking my notes and debating on what to do.  Tonight was her Methotrexate injection.  I'm hoping that will help and she'll be fine.  Otherwise, I think we'll have to go back up to 6MLs (18MGs) of Prednisone.  I really don't want to do that.  I know that she doesn't either.  I'm hoping to prevent it.  We shall see.

Tomorrow I also meet up with fellow blogger & JA mom!  We've been talking on Facebook and occasionally on the phone for quite some time now.  It will be so good to meet!  Can't wait!

Addressing the inaccuracies

I had the misfortune of reading an "education article on juvenile arthritis".  Unfortunately, reading this was akin to how I would imagine reading a JA article in the National Enquirer would be.  The educational content was significantly lacking at best, downright ignorant & hurtful to the cause at worst.  As a parent of a child that has had arthritis for several years now, I have seen, read, and had conversations that absolutely floored me, but this one still has me angry a week later.  The link to this misinforming JA article is here.  If not for copywrite laws I would copy & paste it.  Apparently, the "doctor" that wrote the article writes these and leaves them on his blog.  The majority of his writing is riddled with errors; some advice is dangerous in other articles.

What makes me angry is that this man has no medical training as a pediatric rheumatologist, or in rheumatology at all.  He is writing about the same preconceived notions without doing the research that anyone with an opinion could write, but because he has doctor in his title, people are more likely to believe him.  This can be very detrimental when the subject is something like JA.  We fight so hard to raise awareness, trying to let people know that AutoImmune Arthritis is not because the joints are wearing out from use or old age.  AA is an AutoImmune disease; the body is attacking itself!  No one knows why this happens.

The first (intro) paragraph is innocent enough, though they don't call Juvenile Arthritis "Juvenile Rheumatoid Arthritis" anymore.  Studies show that most children do not have a positive Rheumatoid Factor; children that do generally have other issues, such as Mixed Connective Tissue Disease.  I'll let that slide because some old school docs still refer to it as JRA.  (I prefer Juvenile AutoImmune Arthritis, though that is not what the experts call it.  It's easier to say that and have people actually understand as opposed to making comments based on ignorance because they think they understand.)  Perhaps it's because he doesn't know that the Arthritis Foundation has 100+ different disease that fall under the umbrella of arthritis, including Raynaud's Phenomenon, Mixed Connective Tissue Disease, Lupus, Dermatomyositis, Polymyositis, Behçet’s disease, Lyme's Disease, Tendonitis, Wegener's granulomatosis, Scleroderma, etcetera.  These diseases are all different, yet they are all forms of arthritis.  Yes, children can get them all.

From paragraph number two, the first thing that struck me is that every article in the past that I've read said that most children are generally diagnosed around 18 months because that is when the child is walking, and often seems to be struggling to walk.  Symptoms can appear at any time.  I've never read that boys symptoms show after girls.  That could be correct, but I could neither prove nor disprove that one.   I personally don't believe that is true.
"JRA is usually temporary, and only in rare cases does it last a lifetime. Most commonly, it disappears as the child matures. This is due to the strengthening of the child’s immune system and energy over time."  The first thing that I have to laugh about here is this:  AutoImmune disorders are a result of an over-active immune system.    This would be why these children are put on drugs like Methotrexate, Enbrel, Remicade & Humira, which are all immune suppressors.  Some children are lucky enough to have it "disappear", otherwise known as remission.  They are not "cured".  Many of the children that had JA when I was growing up are the same people that now suffer in silence, told that they can't be sick because we can't see it.  How many people do you know of that had Lupus just go away?

This doctor admits that we don't know what causes JA, but he believes it's due to an energy imbalance caused by the spleen & liver.  Hmmm, some systemic cases may have those organs involved, but most people that I know do not have any organ involvement.  He goes on to say that the weather on the day of conception plays a part, as does the energy of the parents on that day.  So, if you're having a bad day and it's raining out when you're child is conceived you are much more likely to have a child with arthritis?  Somehow, I think not.   He seems to place a lot of blame on the parents.  I can honestly say that I did everything right during my pregnancy with my JA child.  I didn't drink or do drugs (and still don't, thank you!).  I quit smoking, I ate right, got enough sleep, babied myself to a degree, tried to stay away from any stressful situations.  I did everything right.  So, this is my fault?  If any of my children should have gotten JA then, it would have been my oldest.  My doctors advised me to keep smoking since I had a lot of stress, I didn't do any drugs or drink with her either, but I was pretty stupid about nutrition.  I certainly could have done better.  And she's perfect.  Go figure. 

He believes that better nutrition would benefit.  Though that makes a lot of sense, there are no studies that prove that diet has a hand in any of this.  There are specific foods that help reduce inflammation, and others that can cause it (like the nightshade plants- no peppers or potatoes, if I remember correctly).  In the beginning I did a ton of research on this, hoping that I could maybe make it disappear through better diet.  Aside from that info, the best that I could come up with is that many people with AutoImmune Arthritis also have other AI diseases that affect their digestive system.  Many are gluten sensitive and feel much better with a gluten-free diet.  Some can't digest sugars properly either, and they feel better cutting sweets out entirely. 

The last thing that I will pick apart for now is that this man claims that positive thinking "also plays a big role in the speedy outgrowing of JRA".  Ok, I do believe that positive thinking does a LOT of good, but I do not believe that you can cure yourself that way.  I also believe in the power of prayer, but that doesn't mean that Jesus Christ is going to come and cure my child just because I ask Him to.  He has a plan, it is perfect, I have no idea what it entails, & so I will not always get my way.  I realize that Oriental medicine is much different than traditional US medicine.  I know nothing about Oriental medicine partially because I have not trained in it and I do not just decide without fact-finding that something should or should not be true.  However,  I believe that if you're going to write an article on a condition that affects any group in the USA, you should do some research on some material written & proven true in the USA.

On a side note, Emily's new wheelchair came in!  We are very happy about this.  I am thrilled that it came in before a myositis flare left her incapable of walking, for one.  Obviously, we are hoping that will never happen, but since the odds are not in our favor we felt like getting a chair in quickly would be for the best.  Currently her endurance and stamina are not good.  She tries very hard, but if we have a lot of walking to do she simply can't do it.  Walking from the car to her doctor's office at the hospital wears her out.  We use the chair to get the the parking garage on one side to her doctor upstairs and at the opposite end.  If we happen to go to the mall (which is very rare, thankfully) she can't handle that.  Partially it's because her legs can't do it, but also because the steroids have puffed her out so badly that she can't get comfortable.  Of course, she went from 42 lbs to 62 lbs in a month.  I think 30% went to her face, 50% went to her belly, and the rest got spread out.  Her belly went from not having an extra ounce to looking like she was due with twins any day.  That does not make it easy to move.  Her feet are having a rough time adjusting, too.  It's a lot of extra weight added very quickly.  Still, she's very blessed.  Many JM kids are still bedridden for months and months.  She was only mostly down for about 2 months;  at her worst she could still drag herself across the house to the bathroom.  Many kids can't.  I shall leave with a new pic of Em in her chair  :)  We had just gotten her out of her bath;  I hadn't had a chance to comb her hair or anything yet.  We were rushing to get her into bed, but I really wanted to get it set up in case we're in a hurry the next time we need it.  The pictures were an afterthought.

Since I get asked a lot about Raynaud's

One problem that can go hand in hand with several different diseases that are covered under the spectrum of arthritis is Raynaud's Phenomenon. Raynaud's is an over-reaction to the cold. The blood vessels constrict from simple things like being in air conditioning or holding a cold glass of water. The classic signs are the color draining out of the affected hand (or foot, nose, or ear) which turns the area bright white, then turning purple or blue as blood starts to fill in, then red as the blood comes rushing back in. It is painful, prickly, tingly, and causes numbness.  There is primary Raynaud’s, in which case there are no other conditions, and there is secondary Raynaud’s- caused by some other disease such as Scleroderma, Mixed Connective Tissue Disease, and also found to be related somehow to Dermatomyositis.  Secondary is generally considered to be more severe than primary.   The best link that I have found to explain it is The National Heart, Lung, & Blood Institute.    Also, The Raynaud's Association  is a great place to find support and info.

 It was only one year ago this month that my daughter was suffering a great deal from her Raynaud's Phenomenon. Her doctor at the time decided that she was too young for medicine, and that we could control it by keeping her core warm. We would pack her off daily in the winter with two T-shirts, a warm long sleeved shirt, jeans, mittens, and a very heavy winter coat. One would assume that would be enough for a sunny Florida winter, but not the case for my child. It was when she developed frostbite at school that I truly began to panic. This was a life changing disease for her. No longer could my sweet little princess wear her cute little dresses and skirts that she loved so much. Instead, we were piling as much extra clothing on her as possible, but it was never enough. Her school actually made a drill based off of an idea that my hubby had. He would take a sock, fill it with rice, and microwave it for a few seconds to a minute, depending on the size of the sock. The school had several of these socks, and they placed them strategically around the school. Whenever she had a particularly bad episode, they would run for the socks. I worried daily about frostbite, and the possibility of her losing her fingers because she tried to ignore it. The springtime wasn't as severe, but still she continued to have many episodes even when it was 85F outside. And still her doctor didn't think that she needed medication. Our new doctors gave her a prescription for a Calcium Channel Blocker just before the end of December. Even as a child that doesn't like to take medicines, she was afraid of what a cold winter would bring, and she requested it. I can't say that she hasn't had an episode since, but she has certainly had far less. In fact, I have only seen one. Today she can walk around the house in a nightgown or skirt without fear. We can go outside on a cold day without fearing the worst. Yes, she still gets much colder than other people, and she can’t warm back up that well, but it isn’t like it was.  For that, I am very grateful. Her current doctors call her case of Raynaud's "significant". They could tell from a mile away that she had problems with it. Medication was the best decision that we could have made for her. Perhaps it wouldn't be the best course for everyone, but for us it has made a huge mountain look like a small hill.

With this time of year also comes bronchitis.  I thought that we had her on enough preventatives to keep her under control, but I suppose that wasn’t a very realistic thought.  She went to the doctor on Wednesday, but we plan on taking her back tomorrow.  To complicate matters even more, she is due for her Remicade infusion on Tuesday!  Remicade is an immune suppressor, so if she still has an infection it would be very detrimental.  Since we have to drive 2 hours away for it… I’d like her pedi to clear her.  This is her current medication list, with the bronchitis meds being the first 6.

Emily’s current meds as of January 30, 2011

Zithromyacin-  200MGs once daily

Singulair- 5MGs daily

Flovent-  44mcgs twice daily

Ventolin- Every 4-6 hours

Veramyst-  One squirt each nostril nightly

Benadryl- 1.5 tsps (On request for congestion & cough at night)

Prednisolone- Take 18MG in the morning

Prilosec (Omeprazole) ONE capsule once daily

Meloxicam-  5 MLS  (1 tsp) nightly

Nifedipine ER 30MG- One pill once daily

Xyzal- 5MG once nightly

Methotrexate- .5 Sub-Q once weekly

Leucovorin- 1 pill weekly 24 hours after MTX

Miralax- ½ Cap nightly

Multi-Vitamin
IV Infusions:
Remicade- Once monthly
Solu-Medrol- Once Monthly

17 meds.  Now doesn’t that look like a good time?  Poor kid takes more medicine in than food or drink, I think.  This is what having an autoimmune disease does.  And remember folks- she’s only 9.  I'll post tomorrow after work about what her pedi says, or Tuesday after her appointment if she is cleared.  Keep us in your prayers!