Free time!

I apologize for my slacking off when it comes to posting.  I am finally on summer break from school!  It's been a very tough semester.  I actually failed a class and then got a B in my other class.  I don't approve of either, but I've had quite a bit to adjust to.  I didn't even realize that it was stress until the semester was almost over.  I'm praying this doesn't kill my honor society status!

If we were the type to give up, we would have given up on growth hormone quite a while ago.  However, we stuck with it and she finally got it!  It has been about 2.5 weeks.  The down side is that she has had a major headache from it, so we agreed to skip a few days to see if the headache would go away.  We are hoping that when she re-starts it, her doctor will approve a lower dose, and then gradually move back up.  We have already seen some of the benefits:  improved sleep, for one thing.  It is also supposed to strengthen bones, which is wonderful considering that all of the years of prednisone can cause osteoporosis and brittle bones.  I know that this is the right step; we just have to find the right mix here.

She has another sinus infection.  If you read the last post, you may recall that we had a runaround trying to get her into the ENT for a swab and suction.  She ended up getting better spontaneously on her own before we could get the authorization.  Now she's sick again.  She just had 30 days on Augmentin.  She was sick again after a few days.  Now she is on Clindamyacin again.  I have another call into the ENT.

The other bit of Emily news is that, while we were at our pediatrician's office, she tells me that her throat is "bulging" again.  Apparently it is difficult for her to speak, and to eat.  The eating is hampered, but she also has too much reflux coming up, too.  None of the usual meds seem to be helping, so I emailed our doctor to let them know in advance.  This is a hospital week.  It should be interesting.  They are supposed to test her cortisol levels to see if we can start dropping hydrocortisone, and now they will also run an upper GI to see what is up with her throat.  Also, she has been telling me this week that her muscles are bugging her.  That could be from the growth hormone (we are hoping!), but it may not be. The last two visits I noted that her muscle enzymes were elevated.  Not a huge amount, but definitely increased.  I am hoping this will be just a fluke.

Zach is doing pretty well.  Now that he's had a steady supply of Enbrel he is doing better most days.  He's had a few days with a lot of back pain, a couple with knee pain, but overall he is good.

We are going to try the Paleo diet.  I am definitely going to try the Whole 30 on the advice of two friends.  While Emily is one of the pickiest kids in the world, and she would rather starve than eat broccoli, I am hoping that if I lead by example, they will follow.  I really feel like this could help, but if I push, then I have no chance.  I am not looking for a cure or a miracle; I'm not setting us up for a fall.  However, I see correlations with food myself.  I know that soda makes me more tired, it breaks me out, and I feel yuck.  I recently realized that is the same for most sugar.  I feel worse when I eat certain things.  I had chips this weekend and now I hurt everywhere.  I'm almost positive it's from the chips.  So, perhaps if we cut all of that out, things will be better.  The essential oils have been amazing, too!  There is an oil for everything!  Our favorite is doTERRA's Deep Blue.  Especially when we use a friend's concoction and add more wintergreen and frankincense to it.  Amazing!  Now I see that many of my migraines were from my neck.  It's been a fascinating couple of months!

I am hoping for a calm summer since we didn't get to do anything fun last year.  This year I am hoping that we can go out on Friday's to do fun stuff!  After this week...  I will try to post from the hospital when we know more.  Take care!

Lots to be thankful for

Sometimes it hits me how surreal our life is.  Sometimes I realize how not-normal our "normal" is.  Sometimes I feel worried, scared or nervous about how our kids are doing, but then we walk through this cancer ward and it really puts things into perspective.  We see mothers crying in the hallway because their children are given a terrible prognosis.  Children that look more ill than any old person that I have seen are not even allowed to leave their rooms for fear of germs.  These are the days where I really embrace all that we really have.  Our home life is better than probably 80-90% of families out there.  I love being home with the kids more; I rarely have to raise my voice.  We all feel happy when Ash comes home from school or Kevin comes home from work.  That is how it should be, but I suspect it is rare for many other families out there.  I don't just love my family- I like them, too, and we enjoy each other.

Today has been a really good day at the hospital.  I feel like we've gotten some answers.  While Zach's ankles are still not great, and he has a bad spot in his back, he's doing pretty well overall.  We were talking and thinking back to when one of the doctors here first looked him over at camp.  He was a mess.  He had huge red sores (plaque psoriasis) all over him.  It was horrible.  Enbrel has given him a chance at a normal life in multiple ways.  Thankfully, 2 years down the line it is still helping him.  We got him a prescription for physical therapy because I'm not sure how to help his pain when he is walking a lot or playing hard.  I have some ideas, but I know that his issues are different from mine and what works for me may not for him.  I'm hoping that will help him to gain some endurance, too.  Otherwise he is doing very well!

We established that Emily's Pulminary Function Test came back with no problems, so that is great.  I thought it would be okay, but one can never be sure.  I spoke to the doctor at length about how her pain levels don't seem to jive.  She still hurts a LOT, and it keeps her from wanting to do much of anything.  We agree that some of it is her age with the hormones starting, some of it is muscle memory, and the adrenal insufficiency isn't helping.  We also discussed the growth problems and how growth hormone may be the key to calming her pain down.  To put this into perspective, I am 4', 9.5" tall.  I have been this height since I was 10.  Emily will be 12 on December 6.  She is 4', 1.5" tall.  I don't want her to be doomed to shortness, but I also believe that those cells not regenerating are storing pain in the nerve endings.  If her joints are good, and her muscle strength is good, then it makes sense.  Some of it is that she needs to stretch more.  We have discussed this often; I know that she is trying, but I think we need to switch it up a bit.  Her knees were bright red for months.  Today they actually looked good, but they still hurt.  I've been telling her that I think her IT bands are tight.  They just need to be stretched and she'll feel better.  It's so hard to get her to want to move.  In any case, I will take this to our previous appointments.  I think that the hydrocortisone is helping quite a bit, so hopefully she will start to feel even better from that soon.  It's a slippery slope but I have hope that soon this will all be easier for her.

One good thing is that this doctor really sees why we need endocrinology.  It makes me feel less like a paranoid, over-reactive mom.  I really try hard not to be like that.  I try to approach things with as much distance as I can, and from a clinical approach.  It can be hard, but I put a lot of thought into everything before we make decisions.  Em & I talk about it, and then Kevin & I talk about it.

As far as I know, tomorrow should just be a fast release home, but we shall see.  Sometimes things sneak up at the last second.  I still have to give our doctors here a copy of her 24 hour urine test that nephrology ordered.  It's all over the place.  Almost every section is flagged.  We see nephrology and pain management next week, and we are awaiting an appointment with endocrinology closer to home.  The endo that we saw once is leaving the hospital, and since it was two-three hours away we wanted to find someone close.  I will let y'all know how those appointments go.  I am praying endo will see her very soon!!!


*Friday note:  Dropping to 0.3mls of steroids!!!  She has been consistently on steroids with no break since October 2010.  Again, she started at 42mgs, which was about 7mls twice a day.  UGH!!!!!  She has come a long way, but finally there is an end in sight!  WOOT!!!  This is exciting!

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)

Last kidney procedure!

I am so excited to report that the stent is now out of the ureter!  As a quick recap for those who have missed it, Emily had kidney stones embedded in her kidney as a result of the steroids over the past three years.  Because they were large, they realized that they were not coming out on their own, so they opted for surgery.  She had one surgery to place the stent to allow the ureter to open enough to be able to see the stones, one procedure where they lasered the stones apart but left the stent to allow drainage, and one last one today to remove the stent.

I am usually the mom that loves everyone and has a hard time finding fault with the people and situations that pop up in the hospital.  I wasn't even really angry when we had to change rooms two weeks ago at midnight- after I had already fallen asleep.  Today I was livid.

We have two hospital choices when it comes to the urologist.  One is a big hospital with a children's hospital attached, and one is a hospital that is only for children.  One would think that the primary children's hospital would be... child friendly, right?  No.  This would be the same hospital that never checked vitals when we were there for her staph infection that was inside of her port.  We usually won't go there, but in this case I had to make the decision in the middle of Kohl's with my calendar being my phone.  I just thought this would have the stent out two days earlier, and it couldn't be that bad, right?  

I knew we were in trouble when we got there and Emily pointed out that we all forgot to numb her port area with lidocaine (Emla) cream.  We discussed it with a nurse who was more worried about getting Emily to pee.  One would think we would work on the Emla first since it needs time to sit, but nope.  She gave her a gown 12 sizes too big and demanded a urine sample- without the little cleaning cloths.  Em was like, "but this won't be a clean catch!"  They put her chair at the opposite end of the hall, and kept knocking to see if she had peed yet.  We finally gave up and went back to the room.  This time there were 3 nurses in the room, none of which had Emla.  I finally texted Daddy to run out to the van.  Oh yeah, did I mention that only 2 people were allowed back there with her?  At our other hospital there was much less room but we could all go back together.  Here they said that we could bring them back in "a little while".  She also gave me the wrong room number so I almost didn't find them again.  She said 9.  They were in 10 with the curtain closed.  Whatever.  One of the three nurses never mumbled a word until it came time to attempt to access the port.  It took two of them, one asking the other to grab supplies, but neither seemed to know what they were doing.  In the end they hurt Em a bit, and then used a seriously oversized bandage on it.

I could be cool with all of that.  Mostly.  My final problems were that they finally allowed Dad & Zach back after two and a half hours back there, only to have them hang out for only five minutes.  The worst part was that they didn't give her anything to help her calm down before leaving the room.  In fact, they didn't even give us the, "last call for hugs" warning.  It was just bars up, say goodbye.  They said we could have a moment in the hall, but they kept the bars up.  She cried all the way down the hall.  You would think they would have handled that a little better, especially knowing that she is medically complicated.   I was so upset about that.  We all decided at that point that we were never going back there.

There were a few bright spots.  The anesthesiologist was very sweet and he took his time, asking excellent questions.  He even pulled a tooth while he was in there!  :)

The other bright spot was our nurse practitioner.  She has RA, and she used to see our first rheumatologist, now known to us simply as, "He Who Shall Not Be Named".  She made a Freudian slip when discussing him that had me cracking up!  He tried to screw her over, too.  It was great to have someone who understood- if only for a few minutes.  Overall though, we will never go back there.  I don't care how new or beautiful an establishment is, or how good the view.  If the quality of care is lacking, it just ain't worth it.

Stone Update

She had aquatic therapy twice this week so far.  Monday's was not so great.  She promised me that she would give it a chance, but she wouldn't cooperate at all.  On Wednesday it went well!  I cannot say for sure that she enjoyed it, but she at least cooperated.  She looked like she was having fun.  Part of the reason that she did so well, I think, was Tuesday's Biofeedback session.  What a wonderful tool!  Again she was hooked up to the little EKG probes, and again we could see her tension visibly on screen.  The cool part about that was that when she was upset about something we could see it shoot up to like 18, but once she started deep breathing and listening to her relaxation exercise it dropped down to about a 4.  As Ginny pointed out, "see, Emily?  You have the power to relax yourself.  You don't need anyone else to do it for you".  How cool is that?  We discussed aquatic therapy quite a bit that day.  I was very pleased that she did so well the next day.

Yesterday was the kidney stone procedure.  Of course, it wouldn't be our bumpy road if anything worked as intended!  The doctor called me on Tuesday as we were walking into a hospital for Emily's biofeedback to tell me that we may not proceed with the lithotripsy (shockwave of the stones).  I really had no clue what was planned, but we are pretty used to this type of thing.  Things always work out for the best whether we stress or not, so I chose not to stress.  He called me the next day to say that they had a new plan, but it would likely mean two different procedures.  He had initially said that the lithotripsy would require two procedures also, so it wasn't much different to me in that respect.  He told us that he conferred with specialists all over the world to determine the best course of action for our special girl.

They put a stent inside of her urine tube to try to make it bigger.  He told me that it was likely they would not be able to get the ureter opened up enough to grab the stones today.  Sure enough, he was right.  He said that she would have to have the stent in place for 1-2 weeks.  It should dilate the tube enough to pull them out later.  Right now she hurts but not too badly.  They gave her pain medicine, an antibiotic, a medicine to help with bladder spasm, and another med that I cannot recall right now.  The first few hours were awful!  She felt so terrible, and she was in so much pain.  Right now she seems to be her normal self.  It's probably somewhat the pain meds, but it is reassuring.

The only beautiful thing about this is that aside from my working this weekend, we have no appointments planned until 2 weeks from now.  It really just depends on when we go back for the second procedure now.  Thank you so much for your prayers and love.  We felt it, it helped me to be calm and collected.  Emily was still a bit of a mess, but still much better than most kids would likely be.  Thank you for your support.  It is very appreciated!!!

The next post will be a how-not-to-die-at-the-hospital type of post, I think.  I have spent a lot of time comparing how I was ten years ago to now.  I have learned so much, become so organized which is really weird for me.  I feel like I need to share it.  That is coming!  Stay tuned!

Balancing Act

This past summer has been our biggest trial to date.  It has been a series of errors, really.  It started with my thinking that Remicade wasn't helping as much last summer.  Switching her seemed like the right idea at the time because I always had to base my decisions off of actions before that.  She is a child that doesn't like change, so I expected resistance.  I wasn't really expecting Emily to not respond to the next two medications.  Just like I didn't think that taking a break from physical therapy would be too bad.  Well.....  I guess this goes to show that Mom isn't always right.

As some of you may know, I have always known what is wrong with my children.  I know sometimes before they are fully sick that they are sick and with what.  I pretty much understood everything that was happening to Emily, and I was able to anticipate things.  Sometimes I could even tell you exactly what hurt.  And then this year came along and that all flew out the window.  I know that there are several things at play here- for one thing, puberty is rearing its ugly head slowly.  That alone can change all of the games.  I think that some of it is because of the kidney stones, and some from pain amplification.  I think the pain amplification started because of the therapy break and the med changes.  But I think we are on the right path.

This summer has been insane with appointments, but this week alone has been very trying.  We had our hospital medicine overnight appointment on Monday and Tuesday.  No really new changes, though we did have the worst nurse EVER.  I always pretty much love everybody and tend to overlook a lot, but this lady literally asked my 11 yr old to walk her through how to de-access her port.  At first I thought she was kidding.  I was in the middle of saying, "So, if you don't know how to do this why don't you leave it for someone that does?" when she got it out.  I almost died.  It was horrible.  We made it home safely at least.

Wednesday brought us to another out of town hospital for Endocrinology and Nephrology.  We started out so early, but our GPS totally messed us up.  We got there 30 minutes late, but they still saw her.  I did call to let them know we would be late.  I pre-paid for tolls expecting more than last time because we planned on taking the full toll road, but the stupid GPS never even put us close to a toll road.  At all.  That was stressful.  A 2 hour drive turned into 3.5.  Endo is ordering growth charts from her other doctors.  She did a bone age scan.  Em's hand looks about right for an 8-9 year old.  She wants us to do a plethora of blood work.  We are waiting until she has to be accessed next for it as it causes her so much anxiety.  Hey, maybe when she has her kidney stones blasted out!  We shall see.  She said that there is a growth problem marked by either a missing or a mutated X chromosome.  It mostly affects girls, and it can cause problems like arthritis, so she ordered the chromosome test.  I was worried a little about Zach, too because his feet bother him so much.  Since he has PsA, I dig through those medical papers a lot also.  I asked her about this article.   She was very surprised that it discussed type 2 and not type 1, but either way she didn't think that we had to worry about Zach even with the family prevalence.

Nephrology discussed with us how to avoid kidney stones in the future.  In truth, it may be difficult to avoid because they were likely caused by her Prednisone, but drinking more water and eating less salt will be helpful.  She drinks water, she almost always has a bottle with her, but she doesn't drink enough.  They aren't usually allowed soda, either.  She just doesn't drink enough period.  And she craves salt.  She craves popcorn, potato chips, Ramen noodles.  I hadn't realized it before but the little packets of flavoring that come with those noodles is 33% sodium.  OUCH!  Anyway, about 8 weeks after her second procedure he wants to do a 24 hour urine collection, and then see her back.  He also wants us to collect pieces of stone for him to examine.

Before we saw Nephrology I stopped by to pick up copies of the CT scan and report.  Of course I come home and research it all.   Her stones are HUGE.  One is 5X5 mms and one is 8X10 mms.  WOW!!!  That is crazy.  There is no way they would pass on their own.  I also read on the report that she has "gaseous distention of the colon" and a few other things that looked scary.  It all seems to point back to 2 things- her esophagus is still not meeting at her stomach properly, which causes GI problems, and her stomach muscles are still weak, which are causing bathroom issues.  I think once we get those stones out and strengthen those muscles she will feel a lot better.  Her first procedure is scheduled for next Thursday, the 25th.

Thursday was her first appointment at a place that offers aquatic therapy.  For a normal child this would be happy news.  For a girl with high anxieties this was like walking the plank.  She was NOT happy, so she kind of took it out on the poor therapist.  She tried every trick to engage her, but Em wasn't having it.  She was so worried about being in the pool that she couldn't see the happy side.  Today was just an evaluation, but we start regular sessions on Monday.

We also started Biofeedback Thursday.  We met Ginny, probably one of the coolest ladies that I have met this year.  She was really chillin', but she also really listened to us.  She asked Emily a lot of questions, and they talked honestly.  It was lovely.  They talked about sleep.  Emily said that she reads to go to sleep every night because she hurts so much once she lays down to fall asleep.  Reading takes her mind off of the pain.  Also, she said the pain wakes her.  (Sigh).  At least now I know.  We have been asking her about that forever.  Ginny will be able to teach her techniques that she can use to fall asleep, to help herself relax, to help manage her pain.  How cool, right?  But it gets better.  She placed electrodes on Em's main tension points around the head, jaw, neck and shoulders, and checked to see how stressed she was.  She said that a normal person without chronic pain will be about a 2 on everything.  Em had almost all of them 6+.  She had one 13.5.  I forget what her jaw was but it was really high.  This is likely the cause of her migraines, headaches and jaw pain. So, we made our next 6 appointments with her.  The week after our last appointment with her we will finally be seeing a good psychologist.  Since the one that we interviewed for her seemed more overwhelmed by her situation than we are, we thought we would try again to make an appointment with the one who only handles kids with chronic pain.  Between these 2 people, if she is still having headaches, Ginny recommended a headache specialist!  I am hoping the Biofeedback will kind of heal that, but one day at a time.  It will give her great coping tools that she can use for the rest of her life.  And she told me that our pediatrician that recommended her called her yesterday to see if we had been to see her yet.  So sweet!  I will text our pedi tomorrow to let her know.  We have some of the best doctors!

All in all, it was a very stressful week but also a very good one.  I feel like we are on the right path, like we have the right tools in place.  In a stressful time a couple of weeks ago I was trying to figure out how I would juggle all of this.  I figured that if I did aquatic therapy after my classes are out on Mondays and Wednesdays and perhaps any Friday that we were in town then we could do biofeedback and psychiatry on Tuesday's, and our out of town appointments on Thursday's.  So far that is working out brilliantly!  I didn't actually expect it to work because it is a lot to ask of other's but the pieces are just falling into place (praise The Lord!!!)  Hopefully this will help a lot.

Anyone have a cheap helicopter for sale?

Looking back on the past few years, I have NO idea how I worked full-time.  This year has been so hectic, and we have added so many new specialists.  The past few months have been extremely difficult and overwhelming.

Zachary is doing better than Emily still, but we have noticed... things.  For instance, now that I have "kicked the kids out of the house" (to play), he complains more about pain from behind his knees.  His rheumies hadn't mentioned anything before about enthesitis-related, but with Psoriatic Arthritis it does make sense.  His ankle pain comes and goes, but lately it is more knees than anything.  Last month when he saw our rheumies, we were talking about a friend of his who has had a lot more disease activity.  I pointed out that Zach's case is relatively mild, but the response that I got back was, "PsA is a lot harder to treat 10-15 years out.  PsA kids tend to be the hardest to treat and control down the road".  Great.  My heart sank.  Fortunately, we live day-by-day!  I am not going to get caught in that trap, and I won't let the kids get caught, either.  I'm just happy that he is outside playing.  Emily has been playing outside too, though she doesn't last as long.  I am sure that she has been depressed, but hopefully the plan we have will address that and correct it quickly.

I have always been the mom that knows what is happening.  It has been very rare that I was wrong- but the past few months I have felt so out of the loop, like I'm part of a guessing game.  I think we are on the verge of getting that situated, but still it has been hard on me.  Being this hard on me, I can't imagine how my little people cope.  We did switch back to Remicade, which definitely seems to be helping.  She has gone from having mostly 7-9 on the pain scale days to 4-5.

I think I am finally figuring out Emily again.  Ok, this is a lot.  So, she does have kidney stones, which we feel are definitely making life more difficult.  However, what makes everything tougher is that she has "Pain Amplification Syndrome".  I have mentioned before that it was brought to our attention that when one isn't growing properly, their cells aren't reproducing the way they should, which makes the body hold pain in.   After a friend mentioned it, I started suggesting it to different doctors.  They all agree.  That said, we see endocrinology on July 17th.  I am truly pinning all of my hopes here.  As KM said, in their case once growth hormone was added, the first month was horrible but after that everything settled down.  Pain came down, growth started; the best part is that the hormone is a natural hormone, and for them it helped them to decrease some and eliminate other medicines.  I would LOVE that!  I am quite sure Emily would, too.  I am also hoping that this will help her headaches.  Neuro-opthamology didn't see any reasons for her headaches, which I now think may be a combination of hormones (pre-pubescent) and the pain amplification.  We are scheduled to see nephrology the same day we see endo.  The very next week Em has her first psychology appointment, and the day after that she has a procedure to blast her kidney stones out by sonic waves.  They will sedate her because a tube needs to be inserted up into her kidney.  They tell me they will repeat this procedure on another day because sometimes they stones are stubborn and either won't break up or will break up but won't leave the kidney.  We really hope that having these little nasties gone will help her pain level go down a bit.

Another new specialist that we have added is pain management.  That doctor wants her to try aquatic therapy three times a week, as well as biofeedback.  She says that these will both help the amplified pain syndrome.  I'm just not sure how to fit these in.   We have found the aquatic therapy close by, but I still haven't spoken to a provider for biofeedback.  Right now it looks as though it may be an hour south for BF, close to urology.  Comparatively speaking that is not bad.  Rheumatology, who handles pulminology and GI are all two & a half hours north.  Pain management, endocrinology and nephrology are all 2.5 hours east.  With all of these appointments, school work for the kids, and my own schooling come fall, how the heck am I going to swing aquatic therapy three times a week?  I'm not sure, but I know that we will.  If it will really help her, we will do it.

The pain management doctor prescribed pain patches for her.  Just lidocaine, but it seemed to help her knees a bit.  I love that she chose something that didn't require another pill.  I am not familiar with biofeedback, but from what I understand, it involves teaching the child how to cope and deal with their situation using relaxation techniques and monitoring bodily signals like blood pressure and pulse oxygen.  It sounds really cool!  Our advocate has suggested waiting to see what psychology said.  Our psychologist was thrilled that we found a doctor who believes in hypnosis and biofeedback.  I think psychology is a little overwhelmed by our situation.  We had a good laugh over that tonight.  She was pretty amazed at what we are dealing with.  Like I told her, one day at a time and we stay close.  None of us could do it alone.  We need to take this all on as a family.  It would just be easier if we had a helicopter.  Or two.  Or a fleet.

Last thing- Camp starts Saturday for Emily!  She is SOOOOO excited!!!  In truth, for the first time I am glad for her to go to get her out of here, to get a break.  She is so sweet and wonderful, but it seems like every day we are at some appointment.  That will be one week where we are guaranteed not to have to go to an appointment for her.  The down side is that we had stopped Mobic (an NSAID) a couple of weeks ago because it was starting to hurt her stomach.  Since we were stopping Mobic we decided we would drop Carafate (similar to Xantac, it coats the stomach).  Today we ended up back at the pediatrician's office, thinking that her antibiotic wasn't working for her sinusitis.  It turns out that her sinusitis is gone, but her reflux is acting up again.  The Carafate is such a large pill.  I cut it in half for her but it is still so big.  She really hates taking it, and we were so excited to drop basically three pills a day.  Now it's back on the Carafate.  I'm not sure how she handles it, but she is pretty amazing.  I am trying to get us on a wheat-free, gluten-free (or very reduced) diet.  It is very difficult when you have children who are as picky and stubborn as Emily.  She literally won't eat if we don't give her one of her staple foods, but we are learning how to find healthier options.  For instance, I could buy all kinds of fresh fruit but it will sit in my fridge and rot.  I discovered an organic fruit pouch (no GMO!) that my kids have sucked down.  It's actual fruit- not juice.  If that is the only way I can get them to eat fruit, I will do it.  Baby steps.

I will keep y'all updated as we determine new things.  It has just been tough.  Thank you for reading and supporting us.  As always, prayers, blessings, whatever you have to offer, we will always gladly accept and  appreciate fully.

Our latest craziness (written May 18)

For anyone that follows my Facebook page, you already know that we have had a crazy week.  It was actually more crazy than I even let on.

Our lives had basically been put on hold.  We knew that Emily would end up at the hospital inpatient for a while, but we were not sure when.  We had hoped to see a doctor, and that they would keep her, last Wednesday when we went for her meds.  They didn't have a chance that day because they were very overbooked.  The doctor running the clinic is only there once a week.  Emily was devastated.  It's a bad sign when your child actually wants to stay at the hospital.  Especially when said child is the type that never complains.  I spent the days following refusing to unpack, and not wanting to plan anything.

My oldest debated whether or not she had an ear infection.  One day she would want to see a doctor, and the next she felt fine, only to feel badly again the next.  My days were pretty booked up.  I had finally gotten around to scheduling a well-check for myself.  It had been 5 years!!!  I didn't think it had been that long.  While I was there they found a lump, which of course ended up with a mammogram for the next morning.  I had school stuff to turn in, other paperwork to do, and now my own health concerns.  I actually wasn't very concerned until I got there.  They scanned, then they re-scanned.  Then they performed an ultrasound.  Thankfully, I have cysts but not cancerous.  After I left that clinic that day, I ran to school, did a bunch of errands, and finally got home to find that Emily felt worse. We had already scheduled a pedi appointment, so we went there.  We stumped the ARNP.  She didn't know what to do, but she sent us for chest x-rays in case it was due to pneumonia going undetected.  After doing the x-rays we went home. She had already been asking to sleep in our bed over several days before.  One night I just pulled out the sleeper sofa.  Hate that thing.

After we put the kids to bed Tuesday night, Emily decided it was time to go to the ER.  Despite being mostly packed, it still took a while to get us out.  To show you how much of a crazy house this can be, my son's ankle had started to hurt earlier in the day.  He is over-exaggeratedly limping around the house.  He tells Emily as we are packing up to take her to the ER that he is in the worst pain ever that she couldn't even imagine.  Okay.  We got to the ER around 2 AM.  We wanted to go where her rheumy is because we felt sure that all of her problems were rheumatalogical.  This time it was her joints, her muscles, and her chest/ breathing that was bothering her.  They ordered an EKG, chest x-ray, and ultrasound of her liver, kidneys and bladder, and of course they did a urine test.  Apparently she has kidney stones.  They found traces of blood in her urine.  We were unable to see nephrology over the next few days, but we were also told that the best nephrologist is at a different hospital.  .  

Because it is what she wanted, I asked the ER if she could be admitted.  They ran it by rheumatology.  We have been going there since October of 2010, and we have never asked for anything out of the norm, which is likely why they agreed to it.  Her labs look perfect.  That's stupid.  True, but stupid!  I have spoken to a LOT of parents of patients, and patients as well that have said that many times when they are in a flare the labs look great.  Usually the doctors don't understand that, but it is true.  Because her labs looked so good her doctor thinks that her pain is caused by Pain Amplification Syndrome.  Perhaps there is some truth to it, but I really, really think this is a flare- her first since we started going there.  He sent in a physical therapist to do a CMAS (Childhood Myositis Assessment).  I asked the PT about how her score was.  She said "about 20 out of 50".  Her neck muscles seem strong to me, but she can only keep her head & arms up for about ten seconds, and she was afraid to lay down because it hurt too much.  I know the signs with her.  The red spots had started to come back on her hands, two different types.  She had the vasculitis "hot spots", and the smaller red dots that she gets with Dermatomyositis.  Her elbows are pink again at the tips.  Her knees are pink.  This is a flare.  Most importantly, not only did she have trouble holding a toothbrush and walking, she told me how badly her fatigue was affecting her.  The fatigue was getting worse daily.  I guess that we all disagree at some point, and I totally love and respect all of our rheumies, so we agreed to disagree.  :)  I know that he sees a lot of teens that have pain amplification.  He seems to think that is where she is headed because of her age.  I know her well, and I have (obviously) seen her go through everything.  She has always taken it well.  I have some theories.

She started to get the headaches when we started CellCept.  She adjusted after about 2 months, and the headaches went away.  When we started Orencia, the headaches came back, but only for a while.  When we switched to Actemra, not only did they come back but every infusion saw a pain increase with the headaches.  While one would likely assume when reading Actemra's side effects that headaches would be isolated to around the date of infusion, what if that isn't what they mean?  That could solve that.  We discussed going back to Remicade.  Emily is thrilled.  This is my bad: judging by the way she was acting towards the end of Remicade, I felt it wasn't working as well.  I saw her in the bath constantly, something she usually did only when she was hurting.  She is resistant to change on almost everything.  I thought she didn't want to change because she didn't want to try something new.  Apparently it really worked.    He has warned me that it may not work as well the second time around, but we had nothing to lose by trying.

She said that she felt better almost right away after starting the Remicade.  It is likely more because she had three days of IV steroids, combined with Toradol (a strong NSAID) around the clock the three days, but regardless her pain has gone from a 9 down to a 4 today at home all day.

So, right there we need to find a nephrologist, hopefully the one he wants us to see.  He wants her to see pain management because of her age and how they usually deal with pain badly.  He wants her to see a psychologist, because she always seems so sweet and even, and she has never gotten nasty, mean, angry, or moody.  And I mentioned endocrinologist.

Thanks (again!) to KM for bringing this up.  Her son had many similar problems, from lack of growth due to steroids, "something" showing on the MRI, and possible amplified pain.  She explained that (and I apologize if I don't get this 100% right) when the body is not growing right, the cells do not multiply as quickly as they should.  Those cells help the body to heal, thereby reducing pain.  IE: if one is not growing, not only is their body out of whack but their pain can be greater because it does not have what it needs to heal.  I explained this, and the fact that it allowed her son to get off of a considerable amount of medicines!  If we have to add something natural to get off of something(s) unnatural, it is well worth trying.  So we are looking for 4 new doctors.  Apparently pediatric endocrinologists are hard to find also, or we would have seen one while we were inpatient.  At least I feel like we are back on the right road, and we will hopefully be back up soon.  One thing that was beautiful was having so many people check up on us while we were inpatient via phone call, text or Facebook messages.  Thank you all for your love & support!

One last note- I had gotten the camp email saying that she was placed on a waiting list because she had already been to summer camp several times.  She was devastated at first, but she decided that since they could call at any time, she may have a chance.  The following week I received a call from camp.  Due to the severity of her disease they thought that she needed to go anyway!  I tell you, that was exactly what she needed.  She needed something wonderful to look forward to.  Thank you, Camp Boggy Creek!!!  They really helped her when she needed it most.  I was sobbing on the phone.  LOL!  Thank God!!!

*I apologize in advance for anything that doesn't make sense.  I worked a long day, and it is now past my bedtime, not to mention it is a LOT to process.  Back to work in the morning!  Have a fantastic day!  :)

Hoping for real answers on Monday

It seems like it has been a long time since I have posted.  I don't know where my days go!

Overall, Zach is still doing pretty well.  We have noticed that he simply cannot handle being on his feet for long before his heels and ankles begin to hurt.  It isn't the end of the world, but it isn't fair to him when we go places, like Lego Land with our favorite arthritis families!  It was so wonderful to see so many of our extended family members, as well as meet a few whom I have been typing to for years!  It was tough on Zach, though.  I never think to get him a wheelchair.  We should have.  Next time.

I'm not really certain where to start with Emily.  The easiest part is Actemra.  The first three doses seemed to give so much promise; they all bought her about eight mostly pain-free days.  She has had five doses now, I think, but the last one only worked for about two days.  We have spoken to our rheumy group about that, but for the moment they want to see if it will kick in.  If it does not, I am not sure what our next step will be.  She consistently hurts in her knees, hips, back, ankle, and shoulders.  This is the worst that she has felt in quite some time.  She is moving around less and less, because it is so difficult and her balance is off.

The other problem with her right now is the continuous headache.  We have been on the runaround, which I am putting a halt to.  I had mentioned it to our rheumy group the past month or two, but at the time it wasn't significant.  More recently, she started pressing the issue more, so we have delved deeper.  Our pediatrician had sent her for a CT of her sinuses.  The CT came back that she was still congested, but also it claimed that her molar roots were spreading into her maxillary sinuses.  Next step was the dentist.  We had a panoramic done, which our dentist does not think looks as though the roots are an issue.  He referred us to an oral surgeon.  All of that is lovely and all, but I still did not have a reason, so we decided to see our pediatrician.  She concluded that it is likely her TMJ joints in her jaw- the only joints that did not "seem" to be affected prior to this.    So, in desperation I called our rheumies.  Because the scheduler's had messed up our appointment times this past month, we skipped seeing a doctor when we were there.  (I should have bugged our rheumy at Lego Land, but even then I did not realize how much these headaches were affecting her!)  I left a detailed voice mail on Friday afternoon which likely held a note of desperation.  They called me back fairly early on Monday.  Tomorrow we are going locally for two head MRI's.  Hopefully one will be MRI and one will be MRA.  The MRI's will be of her sinuses and TMJ joints, and the other will be of her brain to (hopefully) rule out vasculitis.  These headaches have consistently been about a 4 for her for at least the past month.  Nothing that we do here helps.  Antibiotics had no effect.  She can have 325mgs of Tylenol,  but that's it since she is on Mobic.  She needs the Mobic more for her joints right now.  Tylenol does help her joints a bit, but not her head.

She also popped out here a bit ago to tell me that her chest is hurting again.  We have established that it hurts when she breathes, so tomorrow we will dig out our nebulizer again.  I will have to add that to the list of things that we discuss on Monday at the hospital.  The wonderful thing about having the MRI locally is that we should have answers to our questions about her head on Monday- at least some!  Hopefully they will be able to listen to her chest then, also.  She is already on Symbicort and Singulair daily.  I would like to add Spirulina, the natural blue/green algae.  I have to make sure that it will not interact with her other meds.

Please keep us in your prayers, if you would.  This has been a tough time.  She doesn't want to do anything or go anywhere.  I really feel that having our puppy is such a blessing to her.  He has been so great lately, and he really helps to take her mind off of things, or at least he gives her something good to focus on.

One last thing:  Zachary will be ten on Sunday!  I am not sure where the time went, but he is so sweet, so smart, so funny and loving.  I am so thrilled that he is my boy.  

November appointments

Well, I'm not really sure where to start tonight.

We had already pretty much decided that Orencia wasn't really working for Emily, however I wasn't expecting our doctor to suggest just dropping it and hoping for the best.  Granted, we would be keeping the Rituxan, which she feels is actually helping a lot, but still...  I mulled that over for a little while, and decided that with her recent daily thumb and toe pain, that is probably not a good idea. She has told me that they sometimes hurt so badly that she wants to rip them off.  I totally love our rheumy's- ALL of them- and I think this was the first time the question seemed completely not aggressive.

The night went by uneventfully, with no visit from the doctors until just as I was about to go down the hall to take Zach to his appointment.  Just as we were starting off, one of the other rheumies came over to see us.  She asked me how I felt about dropping the Orencia, and I told her my fears about the thumbs and toes.  She told me that she was not fond of the idea, enough to have mentioned it to the head of the department.  This is part of the reason that I love our team- they actually communicate.  I like our other option much better- instead of dropping Orencia, we will spread it out to every six weeks.  She also asked when our last Prednisone decrease was.  Ummm... long enough ago that I can't remember when.  Well, permission to drop 1/2 of a milligram... granted!  :)  That had me over the moon!  I was so excited!  No, it isn't a huge drop, but it is huge to us!

So, I left our room extremely happy, expecting an easy appointment for Zach and then on to home.  Not so much.  While he looks great, our doc noticed that his toes were swollen.  And they hurt.  And his back has an arthritis spot.  Which usually means Spondylitis in a person with Psoriatic Arthritis.  So, yeah.  Not thrilled.  I was very glad that his appointment was today and not yesterday.  I like thinking our appointments over on the way home.  It's harder to think straight when they keep talking and giggling.  It just hurts your heart to hear these things.  This is not what you want for your kids.  I had really hoped that Zach's would be easier to deal with because we caught it early.  He responded so well to Enbrel at first.  I really thought that this would easy.  He's HLA-B17 positive- that is a psoriasis marker, and it often means a more mild form of arthritis, if I remember right.  I thought we were clear.  So far, it doesn't bother him too much, so it obviously isn't really bad right now.  He has complained of his back once or twice.  He complains of the feet often, and on Halloween it was the ankles.  No changes in meds yet- we are to just keep an eye out and see what happens.  We will go back in 3 months, but call if anything changes.  

On a sweeter note, we were asked by one of the rheumies if we were going to be at the Jingle Bell Walk.  :)  We don't go to that event, only because it is so cold, but I thought it was cute that they were thinking about it, especially since our walk is so far away from the hospital.  I really do love our doctors.  But I would like to put them all out of business by finding a cure, please.  Then we could all just hang out and talk medicine without actually having any medical crises ourselves.  On the bright side, we don't have to go back for six weeks.  Woot to that!    

Oh, and we got a dog!  We swore that we were NOT getting a puppy, and we were NOT getting a big dog.  You know what they say about the best laid plans, right?  We have a shepherd/ husky/ lab mix.  He is three months old.  We were both thinking that he was six months old, but no, he's three months.  His name is Brody, but I swear I may change it to Bitey.  He is definitely like a baby, and so cute!  Assuming I can get his immunizations tomorrow, we should be good to start him in training classes tomorrow!  I am so hoping.  We need to get him to stop biting, and so far the tips that we have found on YouTube are not working.  He is good with the younger kids; it's just me, Kevin and poor Ash.  He especially loves to bite Ash!  She'll be just sitting on the couch texting, and he comes up and starts biting her.  Poor kid!  PetSmart said that they can get him close to therapy dog in training.  I can't wait to start!  I'll keep ya'll posted!

Thankful

Back in 2007 before my children were diagnosed, I was a wreck.  I was extremely depressed.  Nothing in the world could make me happy.  I was crying on my way to work every day.  Yeah- every day.  Aside from trying to raise my family, I had no idea of what else to do with my life- no clear direction.  I was a mess.

After Emily was unofficially diagnosed with Juvenile Arthritis, I was lost... for a while.  I felt hopeless.  Until I stopped feeling sorry for myself and started thinking of how Emily must feel.  After all, she was the one hurting... not me!  I started researching, trying to understand this madness.  At that time, she could barely walk.  Almost every joint was affected.  Her anxieties were extremely high.  She didn't want anyone to touch her.  She couldn't sit on the floor because she couldn't get up. Her kindergarten teacher carried her around school.  Getting out of bed was a huge challenge.  She spent more time in the bath than anywhere else.  And she didn't know what it was like to spend a day out of pain.

Starting Naproxen gave her the first taste of normalacy.  Not that it was enough, mind you.  It took another 8 months to really feel what it was like to be free.  It took 7 months of Methotrexate and then starting Enbrel to help her.  Around that time, the Raynaud's started.  This and some skin tightening made her rheumy feel that perhaps she had Scleroderma.   Oddly enough, this was her good time.  This was the closest she has ever come to remission.  It lasted about a year.

This was also around the time that I realized that she really isn't mine; she was given to me, entrusted to us as parents.  We all belong to God, like it or not.  This is when I learned how to let go, and to trust.  It was pretty tough.  We faced her mortality in a way that no parent should have to.  It was also around this time that suddenly, all around me, I was finding blogs of parents whose children had brain tumors.  I didn't go out of my way looking for them;  they found me.   This helped me to see how blessed we were.  This was also around the time that the little peeps and I went back to church, the only place I can go to cry.  I felt that this was all a way of leading me back home to Him, and bringing the little ones to Him, too.  He was showing me how He was taking care of us.

Emily's diseases have helped our family in so many ways.  We went from being lost and out of touch with each other to being closer than almost any other families that we know.  No, it didn't happen overnight.  It took a lot of work and a lot of pain.  We count our blessings.  We look for the good in everything, because we know that time isn't guaranteed.  The longer she is sick, the more medicines get added to her already long list.  The longer she is sick, the more systemic effects pop up.  The throat issue really scares me.  I have heard of people that require surgery to reopen their throats.  I hope to God that it doesn't come to that.  I refuse to worry about things now until I know for sure that it needs to happen.  We go day-by-day, and we have for so long that it is just habit now.

We expect the possibility that one of the kids will be sick daily, or too tired or sore to go out.  It isn't because they don't want to go places, because they are usually disappointed when they have to miss out, or we rely on her wheelchair.  They have learned to deal with it, and enjoy the good days.  We embrace the good days, and just get through the lousy ones. I truly never know how any day will be.  She is so complex.  He isn't so much, but we need to be careful not to downplay his problems because hers are so much more severe.  I try hard not to trample on his feelings.  My sweet little man.  <3 p="p">
In the midst of this, we have met some of the most amazing families!  This has led me to having some of the coolest friends that I could ever imagine.  Some I have never actually met in person!  My Facebook friends that also have kids with similar problems have become like family.  It is so wonderful to have these people in my life... even if I rarely get the chance to chat with anyone right now!  (This, too, shall pass.)

I never thought that I would ever go to college, or even know what I wanted to do with my life.  Now I have clear direction, and I am empowered by how much I have learned through my research.   I would never have researched if first Emily, then Zachary, didn't get sick.  I have learned all about Dermatomyositis, which took me months to pronounce, as well as Lupus, Scleroderma, Raynaud's, Psoriasis, Psoriatic Arthritis, Spondylitis, and other little things.  I learned what patulous meant,  (spreading widely from a center patulous 
branches, according to Merriam Webster),  and I've learned about esophageal dysmotility and lung changes that warn of pulminary fibrosis.  As crappy as this might be, it has given me a confidence that I never before had, as well as a drive and motivation that I did not have before.  I made a vow to myself to be able to speak with the doctors on their level.  I am 95% there. I am so much stronger than I ever thought that I could be.  Ever.  And the kids?  They are way stronger than I am.
I never, EVER thought that I would be the type to homeschool my children, and yet here I am.  Granted, it is out of necessity, but still... There is no way that she could continue on in public school.  Just simply no way.  She had such a difficult time last year, but that has led to such a great adventure!  Now we have our butterfly garden, crabs and fish.  We have science everywhere!  Everything leads to learning.  I love it!!!  And we are in this together since I am also in school.  We appreciate each other and love each other like we may not have long, because you just never know.  I could get hit by a bus tomorrow.  What regrets would I have?  None.  Could you say the same thing?  Do you have your priorities straight?  Life is too short to worry about competitions and material things when it comes to family.  Drama is way over-rated.  Who needs it?  I don't have time for drama.  We are ecstatic that October wasn't spent with a long stay in the hospital for a third year in a row.  That rocks!!!  Yeah!  We are happy just to be here, just to be together.  That is what matters, and I am thankful and blessed.  Every. Single. Day.

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!






          

Quiet time

It's been a good week for me and Zach, but a rough one for Emily. Monday her shoulder and ankle hurt. By Tuesday, despite several rub downs, BioFreeze and a hot bath, the shoulder was really bad. Her occupational therapist rubbed it out. Em cried, she begged her to stop. We all knew that the benefit would outweigh the pain. Sure enough, she woke Wednesday able to move it with little pain. Her knees have been "tired" and her thighs have hurt. The thighs worry me the most. The thought of her JM coming back sends fear ripping through us all. Not as badly this time. The storms that we had in June threw our monthly med schedule off. While 2 of her meds are monthly, Rituxan is every 3 months. So far, it has been the drug that helps her the most. We would normally have gone for meds this week, so I'm sure that is why she is hurting.

We had placement testing yesterday for school. She got lucky; she wasn't entered into the computer yet, & the person that normally does it was in a meeting 2 hours away. She will be able to take it at home soon. We need to set up an IEP meeting for her before she's 100% clear. Zach is all set, complete with passwords for the online part.

Em has been determined to go to the beach for a while. Where our summers usually have a brief daily storm & usually drought levels, this year we have had more rain than we can believe. We finally got to the beach this evening. With her JM, a sunburn can cause her disease to flare, so we go when the threat is less. I'm sitting here at the beach now, watching them play, watching the sun go down. There is a constant breeze as I listen to the crash of the waves and the kids playing. It is a perfect end to my week. She's feeling good today, and the kids are so happy here.

Emily & Zach have such a special relationship. I love sharing their lives with them. I am in awe of them. I kept catching them hugging each other. So much love!

Oh, & on a last, different note, I am so proud of them. They have been sitting in my church services with me. While some adults may talk all through the service, my kids are clasping their hands & bowing their heads in prayer. I am just so proud of the people they are becoming. I am so ready to start homeschooling! I am so blessed to have this opportunity to be with them more. <3

The Misconceptions of Juvenile Arthritis- as written for my Comp class

The Misconceptions of Juvenile Arthritis

Approximately 300,000 children are affected with  forms of autoimmune arthritis that are clumped under one umbrella term, juvenile arthritis.  These different diseases cause pain, stiffness, rashes, fever, and inflammation of the joints and internal organs.  Some forms also destroy muscle tissue.  They can be deadly without proper treatment.  These diseases do not get very much awareness, despite having more children affected than children stricken with muscular dystrophy, sickle cell anemia, and cystic fibrosis combined. (Dr. Manny Alvarez)   One form, Dermatomyositis, is covered under the arthritis umbrella and is a form of muscular dystrophy.  Many believe it is because of the word “Arthritis” in their label that these diseases do not tend to be taken seriously by the public.  There are many misconceptions associated with these debilitating diseases, as well as a negative stigma surrounding those affected and their families.  Even children feel the stigma.  Careful examination of the facts will show that many of these misconceptions are inaccurate.  My goal is to raise awareness for these horrific diseases while dispelling some of the myths.  

A recent research study  written by A. Tong called “Children’s experiences of living with juvenile idiopathic arthritis” that was based on records of 542 children proves that many of the children feel misunderstood,  stigmatized, out of control of their bodies, and they are in overwhelming pain much of the time.  As the parent of two children with arthritis, I agree with this research.  What this research does not tell you is how misunderstood the diseases themselves are.

Why is there a negative stigma?  One problem is that people do not believe that children get arthritis. When people hear the word arthritis, many tend to think about their grandparents who may have severe osteoarthritis.  A larger problem is that many commercials for over-the-counter products ranging from oral pain relievers like Tylenol Arthritis to topical creams and lotions tout that these products work for arthritis.  The truth is that they really only work for specific types of arthritis.  Most of these commercials, as well as commercials for prescription medicines like Enbrel, show older people walking along, smiling, and doing every day, normal things.  These advertisements do not accurately portray most of these diseases that are autoimmune, nor do they show children in them.  If people don’t see proof in advertising, how would they know that kids get arthritis, too?  To quote Tiffany Westrich, founder of  the International Autoimmune Arthritis Movement from an article on HealthCentral.com , “So what is contributing to this gross misunderstanding?  Look around you.  It's everywhere.  It's on our televisions, it's in our print materials and it's on our computer screens.  The best way to stop it is to change what is publicized in the media.”   People will never understand that there are different forms of arthritis that are autoimmune, or that they can affect children until advertising changes are made.

Osteoarthritis is what the older people generally have.  According to Tiffany Westrich, “The truth is Rheumatoid Arthritis  is a systemic disease that affects joints, connective and soft tissues, muscles and sometime organs, while Osteoarthritis only affects the weight bearing joints.”  While they have not isolated an exact cause, researchers do know that Rheumatoid Arthritis, Lupus, Scleroderma,  Mixed Connective Tissue Disease (an overlap syndrome, similar to Lupus), Psoriatic Arthritis, and Dermatomyositis are all autoimmune in nature.  All of these can affect children, but the medical community clumps children under the diagnosis of “Juvenile Arthritis”.   It is possible for some children to have several different types overlapping, such is the case with my daughter.  Yet, when people hear that you have a child with arthritis, the reaction is generally disbelief.

Despite the belief that people with arthritis have poor diets, while some people with forms of arthritis have food sensitivities that can cause reactions severe enough to mimic arthritis, most people do not benefit from changes in diet.  There have been numerous studies based around diet and rheumatoid arthritis, but there is no proven link.  According to the Arthritis Foundation and Dr. Yukiko Kimura in a Fox News report called “Arthritis in Children”, a healthy diet is necessary to keep the child healthy, but there is no diet that will significantly help or “cure” arthritis.  Staying away from inflammatory foods will help a bit, but it is far from a cure.  The most important diet related item for these children would be additional vitamin D as most people in this category are vitamin D deficient.  Children on steroids for long periods of time benefit greatly from a calcium supplement because steroids can cause osteopenia, brittle bones. Of course, weight should be controlled to help the weight bearing joints to not have to work so hard.    

People tend to not believe that affected children are sick because many of them still act the same.  Eventually children get used to daily pain to a degree, and with the medicines that are available now, many people can function at an almost-normal range.  That does not mean that they are cured, or even that they are feeling well; it simply means that they have learned to adapt.  They try to keep up with their peers, and be more like the other children. 

Some  also believe that the children are exaggerating their pain to get out of doing activities.  This is a common misconception before diagnosis, also.  On the contrary, many of these children struggle to be like their peers, and will push past their limits to do so.   No one can “see” arthritis at work.  Aside from joint or muscle pain and potentially affected organs, other possible symptoms include: chronic fatigue, chronic pain, stiffness, disfigurement, disability, joint deterioration, blindness, hearing problems, and problems with speaking when the joints in the throat are affected. 

             People have a difficult time understanding how the affected children may feel well one day, terrible the next, and fine again on the third day.  Chronic conditions come and go. A child may have a sore knee for days, then the pain is suddenly gone, only to be replaced with a sore hip or elbow.  With the medicines being used today, some children do go into remission.  Many will not achieve remission for several years if ever, but their symptoms can be controlled fairly well.  This does not mean that they live pain-free. 

Regardless of whatever myths or misconceptions that people may believe, a special diet will not cure arthritis, it is very real, very painful, and can be life threatening.  These diseases are nothing to take lightly!

Works Cited

 Alvarez, Dr. Manny. “Arthritis in Children”, Fox News, N.p. Web, June 23, 2012

Dr. Alvarez is a doctor for Fox News that reports on medical conditions.  In the article, he interviewed a leading Pediatric Rheumatologist, Dr. Yukiko Kimura, of Hackensack University Medical Center, to learn more about Juvenile Arthritis and the conditions that the umbrella term covers.  They discuss prevalence, diet, and symptoms of the disease.

Eustice, Carol. “Parts 1 and 2 of 2- Inaccurate Portrayals of Arthritis Lead to Misunderstandings” .  About.com, September 15, 2008, n.d. Web.

This article is very relevant to my work.  It details the misconceptions about rheumatoid arthritis disease, and gives specific examples of what people seem to think when dealing with a person with arthritis.  She points out that some people try too hard to help an affected person, which does not help at all but can, in fact, cause depression and a feeling of uselessness.  She also discusses the myth that so many people believe arthritis can be cured with diet.  She informs the reader of  what rheumatoid arthritis is and isn’t, and she explains that there is no cure.  This paper is written by a nurse that suffers from rheumatoid arthritis.       

Tong, A., et al.  “Children’s experiences of living with juvenile idiopathic arthritis: Thematic synthesis of qualitative studies”, Arthritis Care Res.  Hoboken, April 13, 2012. Web.  

This was a peer-reviewed medical article on PubMed, added by the American College of Rheumatology discussing how juvenile arthritis affects children.  The researchers used clinical data from electronic databases and other relevant clinical articles.  This research found that affected children feel stigmatized, unable to keep up with their peers, they feel a lack of control over their bodies, and many are depressed.  This source will help me to portray how the affected children feel.   “They have a sense of being misunderstood and stigmatised, and feel perpetually caught between having hope and control over their bodies, and overwhelming pain and despair.”  This was said on page 1 of 1.

 Walker, Gayle-Cillo and Marylou Killian.  “Myths and misconceptions about rheumatoid arthritis”, N.p., July 2010, Vol. 5 no. 7.  Web.  

            This article on American Nurse Today also helps to dispel some of the myths associated with autoimmune arthritis.  This article delves a bit deeper into the treatments and medications,  how these diseases are diagnosed,  and some of the other side effects that people don’t always know about. In paragraph two, the nurses wrote, “The variable onset of RA, subjectivity of symptoms, elusive manifestations, and occurrence in relatively healthy young females can frustrate both patients and healthcare pro­viders trying to make a definitive diagnosis.”  This also shows that many in the medical community have a difficult time diagnosing these diseases.  This will be important when building my case.

Westrich, Tiffany. “Misunderstanding RA- how Much is the Media to Blame?”.  Health Guide on

This article is important to my research because it compares osteoarthritis with rheumatoid arthritis.  Most people do not realize that there is a difference, but in reality these are very different diseases; osteoarthritis is not an autoimmune disorder, for one.  This article also discusses how the media plays a huge part in misrepresenting rheumatoid arthritis as being similar to osteoarthritis.  On page one, Ms. Westrich says, “So what is contributing to this gross misunderstanding?  Look around you.  It's everywhere.  It's on our televisions, it's in our print materials and it's on our computer screens.  The best way to stop it is to change what is publicized in the media.” She also discusses specific advertising methods that over-the-counter drug companies use to mislead the general public into believing that mild products such as Tylenol can stop the pain of their rheumatoid arthritis, and not only for osteoarthritis.  She points out that while many children are affected, the drug companies don’t show children on their commercials, or even younger adults.  She also talks a little bit about the true first line of treatment for each disease.   Ms. Westrich is the founder of the non-profit organization International Autoimmune Arthritis Movement.  She has coordinated with many different rheumatology doctors and other professionals. 

Test results and such

There hasn't been much time to post lately!  I have been studying away.  My last day of summer session courses is Wednesday.  I still have a lot of work to do, but I finally feel a bit less pressure.  And I've had some time to mull things over.

The test results from the EKG, and the echo were both pretty good, but the chest CT showed "an air-filled esophagus".  It took me a while to get a good answer on that but it boils down to they likelihood of dysphasia in the muscles of her throat.  The nurse practitioner replied, "

An air filled esophagus can be seen with dysmotility of the esophagus, or if the muscles are affected in the esophagus. We can see this with MCTD.  We would only worry if this affected her ability to swallow or if anything she was swallowing was aspirated. This would show up on the barium swallow.  Let us know if she has trouble swallowing or chokes easily of foods or liquids. I will try to set up her barium swallow and PFTs for the 1^st." 

I found a nifty research paper on pediatric MCTD that also mentions just this problem.    The entire article is a whole paragraph, but it does say that out of the 12 patients that they followed for fifteen years, they that:  "Inflammatory manifestations (arthritis, fever, and skin rash) improved following treatment, whereas sclerodermatous features (sclerodactyly, esophageal disease, and vasculopathy) persisted and were often unresponsive to therapy. The organ involvement-free rates at 2 years, 5 years, and 10 years were 91.7%, 78.6%, and 52.4%, respectively." 

When Emily was first diagnosed with Dermatomyositis, they did an upper GI and found that the muscles on one side were not functioning properly.  That made sense at the time; she was having trouble swallowing, she was losing her voice, and her bird-like appetite became worse.  This time... I wasn't expecting this.  It was really rather out of the blue.  And I can't help but wonder, if there is dysphasia, how long has it been present?  Is it a result of the Dermatomyositis, or is it a scleroderma feature?  I had noted that her AST, one of the liver enzymes, was just a touch elevated.  It hadn't been elevated in at least a year.  My new mantra has become, "there is no point in worrying when you don't know for sure what you are worrying about."  In every other way she is currently doing fabulously!  

I'm not sure if it's the Orencia kicking in or perhaps it is the magnetic jewelry as she credits, but her joints have done a 180 in the past week or two.  Previously her ankles, knees and hips were really bothering her.  Right now it seems to be more fatigue than anything, and that isn't even that bad.  I made her new anklets and necklace the week after coming home.  For those that don't know, we were told that magnets help somehow.  To buy a magnet bracelet is only about $10, so we thought that we would give it a shot.  She swears that it works, that it really helps with the pain.  I can't say that it really does, or if it's maybe psychosomatic, but either way it seems to help her so why not?  I can buy a bunch of beads, 5 meters of string, and a string of magnets pretty cheap, and it's so easy to make something that she will like and wear.  Since she didn't want to start the Orencia in the first place, I think that even if she thought it was working, she would still say it's the magnets!   We were told that if the Orencia starts to work then we can drop the Remicade.  Remicade is a 4 hour infusion, plus an hour for Solumedrol (IV steroids) first, IViG can be 4-24 for her, and Orencia is a half hour.  So, if we move to Orencia only, unless it is a Rituxan month, we may be able to make an 11 AM appointment and NOT have to stay the night at the hospital!  On a Rituxan month we would have to stay late; that's another 4 hour med, but perhaps we wouldn't have to stay the night.  

In other news, Zachary is doing pretty well.  Despite Prilosec, he's had a few really bad stomach nights, but I may be able to pass it off as a bug.  I'm still keeping a weary eye on the situation, given his penchant for spending very long periods of time in the restroom, but it doesn't seem to be an emergency.  His psoriasis is almost non-existent.  He has had some random pains, but nothing lasting.  I am going to ask next time he is seen what is up with his blotches.  Where there were once psoriasis scales, there are now white patches.  It may possibly be Vitiligo, which wouldn't surprise me.  I really was sure of that for a while, but they do seem to be darkening a little.  I'm hoping that by the time he is seen next they will be gone.  We shall see!  

My next post in the next few days will be from one of my paper's from school.  For our persuasive paper, I chose to write about what I know best- the myths and misconceptions of Juvenile Arthritis.  If we all help to raise awareness,  perhaps one day we won't need to.