March doctor & IV appointment

Before I talk about our day and the general health of my family, I would like to ask for prayers for all of our juvenile arthritis friends.  Some are suffering from severe systemic affects of the disease, some are suffering from psychological affects, and some from related conditions (because dontcha know that having one autoimmune condition can open up a whole new can of worms).   One friend who is only around Emily's age is having his colon removed.  He's only between 10-12. We have friends who suffer from more autoimmune diseases than you have fingers on your hands.  Painful GI related diseases, and of course other systemic diseases.  The worst that we have seen is Dermatomyositis, which is horrible, but I know that some suffer more than what we've seen. 

Emily asked me yesterday what would have happened if she didn't get treated for her DM, if they didn't know what it was or if we hadn't changed doctors.  I had to explain to her that not only can it affect your internal organs but it can also affect swallowing and breathing.  She did have some esophogeal dysphasia (weakened throat muscles).  If she wasn't treated shortly after she was, she likely would have required a breathing tube until the inflammation was under control and the muscle tissue was rebuilt.  This is what DM kids face, and while there aren't a few million of them, there are too many kids suffering.  Please pray for all children with autoimmune diseases.  They are a terrible way to have to live.

We are very blessed that, while Emily has her share of challenges and hard times, she isn't suffering the way that many kids are right now.  Not to say that she has it easy- she does not.  But she's not doubled over in pain 24/7 or losing blood constantly, she can eat and sleep and walk.  That's a pretty good day right there.  We continue to deal with the chronic sinus infections.  She has another few days of antibiotics.  Our hope is that this round of IViG will kick in and boost her immune system up more.  Her Igg counts on her labs from yesterday looked pretty good, but next month when she gets her Rituxan next (along with Remicade & IViG all in one day, likely overnight) they will drop so it's important to continue to boost her Igg.  This is how it works a bit:  Remicade blocks anti-TNF alpha, Rituxan wipes out the 'B' cells.  The combination wipe out the immune system.  IViG builds the immune system back up.  Things like the particular form of sinus infection that she has are caused by the immune system being too low.  If we can boost it enough she may shake this thing yet.  They ran the IViG over 4 hours again so I'm a bit worried, but it's a different dose & a different brand.  Apparently they use one brand for outpatient & one for inpatient.  Outpatient's get the brand that causes fewer side effects.  Here's hoping.  If she's going to react then it should happen between 3-5 today. 

Otherwise she looks good.  She's had some back, ankle, knee, shoulder and toe pain, but they are not visibly swollen or red.  We discussed her Prednisone taper and the scare that we had, and we are now working on going from 1.25mls to 1ml. (3mgs). 

Camp called us today to talk about our applications.  They said that they won't release who is accepted until May 1st but I don't think they called me last year, the only year that Emily didn't get in.  They confirmed that they received all of our paperwork from us, our pedi & our rheumy's, so hopefully they will both be accepted!  As much as we'll miss them we know how good it is for them.  I love them so much my heart melts!  And that's about it for now.  I have to steal a quick nap.  The day after hospital wipes us both out.  Good day to ya  :)

March has been a blur!

I have been in a bit of a funk, and feeling overwhelmed by Facebook and bad news.  I've been rather antisocial lately.  I can't believe it's been more than 3 weeks since I posted.  The time has been flying by.  I am typing from a hotel as we await morning for our monthly hospital visit.  Emily will be having her first 8-hour  IViG treatment since the one that they messed up in November.  I'm a little nervous about doing a faster one, but our doc has assured us that this is a lower dose and the infusion room nurses don't make the same mistakes that they do on a ward like where we were staying then.  Still, I'm a bit nervous, but more than anything we just want it to kick in and start helping her immune system stabilize.

She is still on 1.25mls of Prednisone.  We attempted the taper when we were supposed to, but a few days later her thighs hurt.  Kevin thought that perhaps it was all of the running around at a birthday party that they had been at, but Emily said that it felt like a flare.  She was also sleeping a little more than normal, so we went back to her old dose, to the 1.5mls.  I was worried.   As terrible and disabling as RA & JA are, Dermatomyositis (& Polymositis) are worse.  The thought of that returning is something I can't think about.  I was very, very thankful when a friend (thank you again RHT!) told me about a plan that someone else was using to help taper.  She's been back on 1.25mls for about a week and so far seems to be handling it ok now.

The other issue is still that stupid sinus infection.  UGH!  I am so beyond over it!  She had her 21 days of Augmentin, though missing a dose or 2 cuz' the pharmacist didn't mix it, and I'm certain that I messed the first bottle up.  She had about 2 days off of it when she started coughing up green again.  She had stopped coughing only a week or so before, so we noticed it right away when the cough came back.  Very frustrating.  She's back on another ten day course, and the ID doc is going to pop by to look at her tomorrow.  She still looks pale and sick, but she says she's feeling better and she's acting ok.

Zach is doing all right.  He turned 9 on Saturday!  He gave himself a skinned knee and elbow for his present.  Not so much fun!  Before that he had a day or two where his knee was hurting.  His hands look a little weirder to me every day and he's still getting headaches on the sides of his head.  I'm praying that it's just a coincidence and not a failure of his Enbrel.  Enbrel has done wonders for his psoriasis.  He's almost 100% clear!  Overall he's doing well.  He's going on a field trip with school tomorrow to LegoLand!!!  I wish that I could go, but.... I'm needed here.  I sure never thought that this is how my life would turn out but it is amazing how my heart melts for my kiddos!  Poor Ash is home today not feeling that great.  I hope that she's not sick.  I wish I could be there for her today, too.  Even though she's almost an adult.  How crazy that it!  Anyway, we have to be up early.  I'm grateful we don't have to be up at 4 A.M. to drive here though!!!  That is NOT easy for us.  I'll try to post tomorrow if I have time and don't get sucked into hospital time.  Take care, ya'll!

Our new plan!

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

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

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

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

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

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

*Written at 12:04 A.M.*

Rheumy visit & overnight stay

Well, I feel as though we’re on the right path.  Today we set out early to bring Zachary to school and head to the hospital for Emily’s infusions and doctor appointment.   On our way up the ENT’s called twice to see how Em was doing from yesterday’s procedure.  I asked them to please send copies of everything up here.  Last I knew, that hadn’t happened yet, but I have them working on it here now, too.

I was really hoping that we would see the main doctor today.  I thought it was time to get the many years of experience in to see her.  I was so happy that Dr. E was here today!  She seems so much more relaxed since the new doc was added to her practice.  We went through the whole story again, discussing cultures and the new jaw pain.  As the ENT said that he didn’t think it was from his side, our rheumy thinks that it is.  She played with the jaw a bit and she doesn’t feel any problems with the joints in her jaw.  But she didn’t like the whole “wait to see if there’s an infection” idea, either.  While we’re here, she figured that she would prescribe ROCEPHIN just to be on the safe side.  If the ENT’s had faxed over the sensitivities from last culture we would have a better idea, but it is what it is.  I have to try to remember that these “extra” doctors don’t care the same way, or realize how huge a deal Emily’s conditions and immune suppression are.  I’m happy to have her on an antibiotic.  While I realize that she can’t stay on one forever, I truly believe that she needs one now.

We discussed things like NSAID’s, and how she hasn’t been on one for quite some time.  She’s feeling yucky enough to want to try her hand at Mobic again.  So Dr. E put in a new prescription for Mobic, and they gave her some Singulair here.  We talked about her Methotrexate.  Because Emily gets so itchy and MTX is the only thing that seems to touch it, she is probably the only kid that wants her MTX.  We decided to wait a week or two and then try it at a lower dose.  If she doesn’t get worse after two weeks then we can try her normal dose again.  We were also given the ok to go down on her Prednisone!!!  WOOT!!!  I have been wanting to ask for this, but I know there’s a schedule…  At the moment she’s been on 1.5mls (4.5 mgs).  We are going to go down to 1.25ml for a week, then down to 1ml!  That is huge!!!  I will definitely take 3mgs to 4.5mgs any day.  I was very, very happy about this. And they're going to look into helping us get a hospital bed for her because she has a hard time getting up and she likes to sleep in an almost sitting position.

Tonight’s IViG to boost her immune system was started late so we’ll probably be here until 2-3 tomorrow, but the longer the better.  They are also using a higher dose than they had originally planned to give her a boost.  I’m happy with that.  So is she.  Emily had actually asked me if she could do IViG.  She is so tired of being sick, and I am so tired of worrying about the “what if’s” of her being sick.

While we were in the infusion room we had a med student come talk to us.  She is doing a paper for school about communication between doctors and parents.  I was pretty excited about this, actually.  There are so many things that they don’t teach in medical school that would be a tremendous benefit to doctors.   I think that, like parents, the doctors get conditioned to thinking of little things like surgery as just that- a little thing.  I was thinking about how when we first met our ENT’s with Gir’s ear infections and later with Emily’s adenoidectomy, he so casually declared that she was going to need surgery, have a nice day.  At the time I was devastated.  Now… sometimes surgeries are welcome.  If it helps to change the problem, if it’s the only way… then ok.  But as a parent just thrown into this world, you’re totally overwhelmed.  So we talked about that, relationships with medical personnel, how Em’s diseases have affected us if different ways, and so on.  It was nice, actually.  I had planned on just chillin’ out, catching up on Facebook and maybe mindlessly clicking on some game things.  Real conversation was much better. 

So it was a strange day filled with ups and downs.  I’m so glad that we’re here tonight.  As crazy as that sounds, my hope is that the ENT will call with culture results tomorrow and we’ll have been able to have a plan in place with Dr. E before we leave.  That would rock!  For now, I’m so exhausted that I don’t even really remember what I wrote and re-reading isn’t helping, so I think I’m going to mindlessly click some stuff and head to bed.  I want to thank those of you that are praying for us through this time.  I appreciate every single prayer.  Thank you!

Frustrated

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

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

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

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

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

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

How are your kids doing?

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

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

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

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

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

Little update

It's been a good week so far :)  Zachary seems to be recovering now from his sinus infection.  We withheld his immune suppressors this past week until the end of antibiotics.  We started them again on Friday.  It was another one of those decisions that is risky either way you go.  It's either hold off on his arthritis meds and let the infection clear but risk more disease progression or it's letting the infection linger.  I don't think I could handle him also having a 3-4 month sinus infection.  I don't want him to go through what his sister just went through.  My biggest concern at the moment is his hands.  Kevin and I noticed that his hands look weirder now.  Almost like the tops are all swollen but with a new little nodule-like things on the knuckle under each pointer finger.  Kevin pointed out that Emily's nodules were hard so they may not be, but I remember hers being squishy.  Then again, I've been wrong on a lot of things lately, so who's to say?  I guess we'll find out at his next appointment in April (I think) if they are still there.

Well, so far Emily is actually doing pretty well!  Yesterday marked her first day off of antibiotics and so far she's doing ok.  Now we'll just hold our breath for a couple of days...  Hopefully this sinus scraping did the trick.  She sees the ENT in 2 weeks, just before going for her next round of medicines.  I don't think that her pediatricians realize quite how many immune suppressors she is on, and what they do to her immune system.  She was able to do a little work yesterday with her homebound teacher, and today she was able to spend the whole day at our local science museum for an Arthritis Foundation event. 

We LOVE events like this.  Not just because we are spending the day at a science museum, but also because we get to spend time with people that understand our world.  It is like going home when we meet our arthritis families.  I get so overwhelmed when there are many of us :)  We never have enough time to all talk, but I just love everyone.  We really have the best families.

I finally got to meet the Kelly behind RA Warrior!  It was so good to meet her!  She is so sweet in person. I think she made everyone tear up when she spoke.  Two of our other friends also spoke, and did an amazing job.  :)  My son stole a few hearts when he went up to hand one friend tissues.  My boy is a heart breaker.  :)  I love that little man.  <3  All in all we had an amazing day.  I am so happy that we had the opportunity to go without things going wrong.  We really needed a happy day.  And it was!  A big thank you to the Arthritis Foundation for hosting such wonderful events.  We know that most of the money raised for them goes to research and things to help the kids, like summer camp (which is HUGE) and the JA Conference.   It is imperative that people realize that many of the breakthroughs in medicine have come because of the Arthritis Foundation.  They have helped us a LOT.  So we need to try to help them to better help not only our family, but many other families as well.  That said, it is time to gear up for our fundraising for them!  Our May walk is coming up quickly!  I will post here when I get our page up!