8 years old......Juvenile Dermatomyositis 6 years old

I at least want to keep the faint heartbeat of my written account alive.........so lets check in.

The last entry talked about trying to figure out why he was so grumpy.  2015 was real rough on him.  So many factors that make up his health, tricking us here, scaring us there, but always works out.

The most challenging thing of all this and always will be is not knowing.  The unknown.  I guess that is a factor in anyones life.   But for families that are plagued by extra circumstances, like autoimmune diseases, that unknown goes a lot deeper.   Last year, not knowing why he felt so unpleasant.  So hostile.  A few months ago the question was answered.  The question was why his blood counts had been treading down for so long.........words like malignancy were mentioned.  Inflammatory storms coming from his immune system.  Sure enough there were no indications that nothing new was going on.  So they prescribed some iron.   It made a world of difference.  He often complained of tummy pain, and his stamina was limited.  Iron has turned it around.   We did adjust a few medications.  Dropped the Orencia and added Tocimizilab......this one has made a world of difference for his calcium.  They have been way less painful.   So we decided to try........for the first time in over two years.....the tapering of prednisone.    Our doctor said we are seeing the compounding effects of being on all these medications for so long.   That motivated everybody to try!   I can't say it is going smoothly.   Labs did come up after only 4 days of tapering but, deep deep deep breath.....and we are checking labs again tomorrow.   Fingers crossed.....he started physical therapy to help him regain some general physical fitness.  He also started flag football.   And although he did need to repeat his grade, he has been going to school full time......after a full day of school, he plays with his friends after school.   Just simply amazing stuff........we work closely still with psychiatry.  Our doctor not only coaches him and helps him deal, but she coaches me as well!  We have identify syndromes that have developed during his treatment.  It has been very helpful.

We did visit the plastic surgeon last week again.   We are waiting to be scheduled to have more calcium removed.  Although he has not formed any new ones.....the existing do not go away easily.  When they try and go away, it is not complete, and leaves him with holes in his skin.   We figured a nice clean incision and the all out removal of the calcium is better than the slow, painful, agonizing way they rid of themselves naturally.

To sum it up.......not to bad!   We are in a little bit a rocky pattern with the steroid taper but I will take that over adding and changing medications anyday!!

Special occasion!  Having a early breakfast at Denny's with the family.  Big brother was headed in to get braces put on his teeth.  So we all joined him in one last meal before the discomfort of his teeth moving around set in.

This is This, and That is That

Before it all started he was doing good enough to come down off his intravenous steroid dose.  I was stoked because I was feeling good about it.  Any symptom or change I could explain.  I want to explain.  Sometimes I think I make myself explain just to avoid these symptoms that are so minimal....

January..........wow he is grouchy.  He must be coming down with something.  Maybe a tummy bug.   End of January an infection on the top of his toe from a tiny cut.   Must be the antibiotics making him grumpy and causing tummy pain.  A stomach virus for a week didn't help.

February.......wow he sure is grouchy.  Not happy about life.  Only things that indulge him like food and video games make him happy.  Maybe he is spoiled.  Surgery to remove calcium for the first time.  Followed by antibiotics again for suspected infection of incision.   Antibiotics must be bothering his tummy.  He didn't want to eat steak.  Weird gestures when eating but not consistent.  No choking.

March.......so grumpy.  Infections of multiple calcium deposits that cause a whole lot of pain not from surgery, just all by themselves are surfacing.  More antibiotics.  More down time.     In the hospital for a week for pain...more antibiotics.  Labs elevated a little bit but explainable with what could be an infection.  Ran Rituxan because.......calcium is scary, painful, and deforming.  Our doctor felt it was time to make sure we just not sitting there while new ones could form.  But as far as we could tell they were not.

April....just when I thought I couldn't take it anymore.  The emotional distress this was causing me was really starting to wear on me.  Like my heart was being scrubbed on by a giant nail file.  The not knowing, not sure, some good, some bad.   Lots of missed school, lots of down time.  More and more complaining.  And another infection of calcium deposits to confuse thing.  This time we went without antibiotics.  We wanted to see if the pain it causes him is just the pain of the calcium exploding out of his skin.  Horrible.  So bad we have to give him narcotic and iv tordal.  Driving to the hospital every day to get iv pain meds.   We managed.    

To my delight, and anxiety.....my 40th birthday brought an amazing trip to Vegas with some of the most wonderful people in my life.  I reluctantly let go and went.  I was 2 hours away, a quick plane ride.  My husband was here to manage everything with my brother in law.  They did amazing.  My husband even drove him to the hospital for pain medication and then took him to a baseball game.  I had labs drawn before I left so they were ready for when we returned.  Our appointment was the day after I returned from my trip.

My suspicions I don't want to say have been right all along.  But finally......finally the labs are cooperating.  They trended up.  So much so the complaints of my legs hurt, my arms hurt, my everything hurts, my FEET..always complaining.....The tummy is the number one complaint.  So that is what we are going after first. Friday he will be put under once again, and scoped.  If the results of the scope are not good, it will take us in one direction.  If they are fine it will take us in another.  We want to try another medication....but we have too wait for Rituxan to kick in.   If it doesn't.......than this is this and that is that, when, and then......its all so complicated trying to figure it out.  I even considered some of it is psychological.  We even have a doctor for that.   But I still like the aggressiveness and knowledge of our doctor.  I like she realizes we can't afford "wait and see".  Gary immune system can be evil and up to no good.  No one wants to wait and see what it has in store for us next.

Best part is Gary came back the other day.   And by that I mean the happy go lucky child.  The 8 year old we have not seen in some time.....he was in a good mood.  He even went to school for half the day. I picked up, he looked exhausted, and heliotroped out but.....he did it.

I was so glad for Vegas....God knows when your about to crack.  It is hard to get yourself to stop fretting once you start.  Especially when it is driven by instinct of a mother that something is wrong!!  I came back, refreshed, renewed, and ready to keep battling.  I think Gary even enjoyed spending time with his brother and sister, and his Dad.  Not that we don't love love love each other.....but when someone has pain the way he has had for so many many months...it is such a strain.  An indescribable strain on both of us.  It exhausts us.  Doing constant wound care.  A few times we talked about going to the hospital so we could get the help of a nurse and not have to worry so much about every little detail.  But its not easy there either so we managed at home.  He is such a good kid.  We can't wait to come out the other side of this dark tunnel.  Every time we think we see that pin point of light, we seem to take a detour.  

Non highlight:   Poor Gary had an awful time getting his stitches removed.  So awful the nurse accidentally poked herself through her glove.  It is known in the medical industry as a blood pathogen exchange accident.  It meant some labs being sent to make sure no one has anything to exchange.  It never occurred to me to be worried Gary could be on the receiving end of that.  Chances are so very low, I will not give it another thought.  

If anyone needs me, I will most likely be at Childrens.   (But poor me was in VEGAS last week at this time.  Blessed!)

Calcium Removed Part Two

The alarm went off on Thursday morning at 4am.

I admit it was really tough hop back on that horse when we just finished being in-patient last week.

I admit I am getting a little worn down.

I admit things seem harder lately on me (let me rephrase that, "for me"   not on me),  from emotions to pinched nerves in my neck.

I am not the one who sat in the hospital for a week with an infection in my knee.  Only to have lots of drugs pumped into me.

I am not the one who just when I was feeling better, they put me under and cut calcium deposits off.

I can only imagine how exhausted he must feel.

Differences
They removed the first calcium deposits in Feb.  He did well with the anesthesia.   He came back to recovery crying a bit but we were able to get him to stop quickly.  Oxycotin helped with that.

This time however, it was a little different.   It is amazing how people are different so things will go different.  Last time they didn't require him to do the dreaded wipe down.  They hand you large wet cloths with disinfecting juice on them.  It is to help remove any germs our body has on our skin.  He hates them hates them thats them.  They leave you feeling sticky, cold, and nasty.   Because of all the open calcium lesions they told we could just skip it.  The risk outweighed reason.  But not this time. This time they insisted.  So we did it.  He did fine but on the inside, I was angry with them.  Be consistent at least.  Either required them or don't.  I suppose I have hit a burnout.  Because I can explain to you how I understand the rational part of it.  The safety of it all.  Wiping away any chance of post-op infections.   Why wouldn't I want that.  Because I am clearly not rational right now.  It is all taking a toll on me.  I know, I just think "OH POOR YOU!!!!".  Get over it self.  Seriously.  You act like you are the one getting all this treatment, and sitting there with over 60 calcium lesions.  I am not.  I am healthy.

Gary went in to surgery at about 9:30am.  I am very unhappy to report that his port did not work for the second time.  I blamed the ER nurse.  And now I bl

Oh, I'm Use To This

I will never get use to this.

Especially when the hits just keep on coming.  No sooner did his stomach bug clear up,  he came down with an infection on his upper arm under a calcium deposit.  Well really its more like a series of calcium deposits all in one place.  And the debate is still out whether or not it truly is infection, or the bodies way of pushing it out, much like a silver.  A tiny silver can produce that annoying pinching pain.  Well, the pain of these calcium deposits are much much much more.  We still are not sure of what is really going on.  We know it hurts very much.  The difference though between these is he will have calcium come out in many ways.....but it does not hurt.  His arm was a small pocket.  So I decided to give him ibuprofen and try warm compresses to get it out.   Four days of agony, and it worked!  He did have to not move around much to stay comfortable.  I was in contact with doctors who did call in an antibiotic.  Which I went and  picked up and had.  But I did not feel comfortable giving it to him unless they were able to see him.   He had been on antibiotics for his little toe in January.  Then again in February for his surgery.  So this would have been the third go around.  They talk so much about the overuse of antibiotics and creating resistant strains.......so I waited on it.  He was able to have a small birthday party with his friends on Friday.  He participated just fine.  He played basketball, played catch, lots of activity.   I did worry he was over doing it but, it was his birthday.   That night he did wake up crying his muscles hurt so we gave him some aleve.  Worked well.  And then......Saturday morning.....he woke up with a giant knee!!!   Que evil suspenseful music!   My heart sank and I thought NOOOOO.   No, please the arm was terrible.

I decide to buck up and give it a go with his knee, like I did the arm.  When a new eight year old is in pain, it is reflected in attitude as well.  He was rightfully grouchy, but it really was starting to wear me out.   He couldn't walk to well on his knee so we encouraged him to take it easy.  We were able to do warm compresses.  But by Tuesday morning I called and said....Im done.  I am coming into the hospital.  We need help.  He is exhausted.  I am exhausted.   The entire family had it.    They told us to come in through the ER.  We were admitted.

Knee after swelling came down

Lucky for us we were put in isolation.  The constant flow of goo coming from both knees made us contagious.   Both knees were sore but the right knee was like it was broken.  I told the doctor I was there because what if his knee cap is joining in on the liquifying of the calcium.  It was red hot.  He would let no one touch it.  It did test positive for staph infection.

We had lots of people visit our hospital room.  We always do.  Its like a sandwich shop at lunch time. Wound Care came.  Child Life came.  Speech Swallow Study came.  A religious hospital chaplin came.  She offered us anything we needed.  I looked at her and said, "I certainly hope we don't need you".  I mean, I know you always need and could use prayer but all's I could envision was last breath sort of thing so I was kind of like, scram.   Of course I didn't say that but I thought it.  I'm sure it was written all over my face.

So before all this happened we were feeling good enough to go down a little on some medications.  We have been watching closely for signs of any changes that might lead to a flare because of the taper.  The calcium deposits doing this has nothing to do with that.   In my opinion anyway.  The ER had not drawn his JDM labs.  They checked his CRP and then all the counts like white red ect,.  I told rheumatology with all the subtle changes,  I really need to see what those are.  He was getting a little heliotrope a few weeks ago, but it subsided.  He had been showing a little bit of swallowing agitation.  He use to love steak but took one bite, and now he won't touch it because he said its hard to swallow. I was having lots of trouble believing it.  You would think more subtle JDM signs would show, like red knuckles or capillary changes.  Why would swallowing be it.  So I even hesitated to report it because he can eat a chewy bagel no trouble?  That is not someone who is having swallowing trouble?  But I thought when you are inpatient, you are advanced some services that you not when its through the clinic.  People will come right to your room instead of waiting weeks for an appointment.  So they came and we didn't get any answers because its so hard to tell.  If its not every time.  We do have swallow study set up for the end of April now but I was trying to tell them that was pointless.

Welcome back Rituxan.
Our rheumatologist walked in and said so guess what I want to talk about.  She had been mentioning it for months now.  She wanted to give Rituxan a try again.  Her sense of passion and confirmation through her gestures were pretty powerful.  With one sentence I said let me think about it, and then the next, it hit me like a lightening bolt.  Do it.  One look at her face, the need to stay in front of this....I was just like do it.  I found myself trying to back peddle out of fight or flight.  The flight part of me asked for his b cell count which Rituxan targets.  The fight instantly came back in and said, "will the b cell counts change whether or not we run Rituxan."   A solid NO.   So I said don't run the labs then.  They did anyway.  So some med changes.  We are dropping Imuran, replacing it with Rituxan.  I asked for a taper on it, while the Rituxan kicks in.  They agreed.  We are adding back in calcium supplements because his calcium levels are low.  Yet, his body shows a lot of calcium, so confusion but I get it is the wrong kind.   We changed a blood pressure med at my request.  There is one that is used to block calcium in the blood.  They said, sure, why not.  We are also going to spend the summer on Sodium Thiosulfate.  She wants to give it another try.

LABS
His JDM labs finally came back.  Like we discussed with the doctors, there is so much going on in his body with the viruses, infections.  It was hard to tease out whether or not the taper was leading him into a flare.  His AST and ALT did pop up a little.  Labs often don't match what is going on but time the subtle signs match the subtle labs.

So much to think about.  I feel like its so much to figure out.  We are still on for surgery next week.  His bottom has been bothering him still so I know it is so much for the little guy but I want that him to get some relief with that.  He has dealt with calcium on his bum far too long, and I don't want to miss the opportunity.

We go back in two weeks for our second round of Rituxan.  It will be an admit.  I will turn forty there in that room.  I guess as long as I am with him, there no is no place I'd rather be??  I would not like to be in say Vegas where we originally planned to go.   No way am I feeling sorry for myself though.  It is just fine.  I am so grateful for the care.  I am so grateful we are still able to keep this quiet and battle back, until it (Gary's immune system) decides its done.

Thank you so much to my baby sister.  She made us dinner Friday night when we returned home.  It was so nice.

Happy 8th Birthday Gary.  Are these boys cute or what?!

The Viral Times

These are germ sharing times.  We can't seem to get away from them.  Bleach and fruits and vegetables was my recommendation.  Oh and hand washing, all that jazz.  Honestly, I think it all helps but unless you walk around in a bubble.

Then trying to figure out viruses with a child that is immune all messed up is like being squeezed.   What if its not a virus, what if its something else.  What if its an infection?  What if a flare?  What if it his port?   In my mind my face is going in every direction while my head stays still.  Drives you crazy.  Then if it truly is a virus, what if I catch it?  Am I feeling sick?  I think I am feeling sick?  Does my stomach hurt? Scream STOP.    Get it under control.

Last night Gary popped up with a I don't want to eat dinner.   Fine with me.  No dinner, nothing else than.  Later in the evening I offered again.  Still a no, and I have a headache.  I get this often from my child.  He does not like going to school so much he will set the stage for the next day.  So headache complaints must be at least 6 or 7 times before I will pay attention to you.  This time however, he said "I'm cold".   Then violent shaking, headache.  I gave him tylenol of for the headache.  We covered him in every quilt our friend Sue has sewn for us.   He really started to feel unwell.  The staging for no school the next day was going a bit far.  So far that this is the real deal.   And just like that 20 minutes later 102.6.  Blankets off, but I'm still cold.    No vomiting,   no action at the toilet.   He was miserable.  It took longer for the tylenol to kick in than I thought.     He finally fell asleep later that night with a  washcloth over his eyes.  I figure I would call the doctors in the morning to report.  Luckily the severe symptoms have subsided.  He woke up the next morning, better but really tired.  Achy tummy.

In February Gary had his first round of plastic surgery.  They removed three calcium deposits.  One from each elbow and the one under his chin.  It went pretty well.  Healing was a little tricky, we knew it would be.  When Gary came out of surgery they told us his skin is thin from all the years of steroid use.  They double nylon stitched him closed to ensure the incision stayed together.  We did go on some antibiotics because we suspected a little bit of infection but other than that they look beautiful!!!    It is definitely tough on him but so worth it in both our opinions.  I know my son feels the same way.  Monday we have our post op appointment.  We will find out if the surgeon is willing to do more or if that is it.   Right after that surgery,  while Gary was still under, they ran him thru the MRI.  NO evidence of muscle inflammation.  So that was terrific news!  His labs have been fine as well.  Nail capillaries are pretty nice looking so all signs are pointing to lets pull back slowly off of some of these medicines.   I can't really say that with extreme confidence because it has been a long six years of this dance.  I don't mean to be negative but the been there done that comes to my heart and mind.   And the not best part of this whole thing is even if we can wean him off medications, then you have sit there and worry he will flare again and you have to start all over again?  I mean, I have known this all along but I.....wait...what?

Before

After

We Gave It A Try......

Sodium Thiosulfate we started on July, second week?  Three times a week.  It was a rough start.  It caused him to have a stomach ache, almost instantly.  It caused vomiting and headaches.  But we soon got it all worked out with medicines to combat those side effects.  Zofran, tylenol, benadryl.  As a review we were in the hospital in August, then again in September for infections of calcium deposits.   Methotrexate was pulled, Imuran lowered.  In October he started to become intolerant of the sodium it seemed.  So we cut down to two infusions a week.   We also stopped seeing improvements or movement of the calcium.  After three maybe four weeks of being off methotrexate, a rash on his face appeared.  I thought it had started almost as soon as we stopped methotrexate but it may have started at the same time.  The rash was not typical JDM.   Although it did cover his eyelids.  It was like a chemical burn or something.  Benadryl helped.  We even stopped in at the dermatologist to ask if this is a JDM rash or a drug reaction from the sodium thiosulfate, what are we looking at here?  JDM labs have been steady.  Strength is pretty good.  Stamina could be better but its winter, its color there.  Nail capillaries are fair to pretty good.  So as far as dermatology was concerned,  they just didn't know.   The very next day we had a rheumatology appointment.   This was a big day.  The rash made a GRAND appearance for everyone.   So much so that he looked like a little red swollen tomato with a tiny nose!  Everyone that came in to say hello would get to the second syllable in his name and their jaw dropped open.  The doctors came in.  They had already been tipped off by nurses.  I love our rheumatologists.  They had their game faces on.   We quickly pulled the obvious, sodium thiosulfate.  No more, we stopped improving anyway so.  Then we discussed what to add back in.  Step two was to up steroids to combat the inflammation.  Only a tiny bit though.  Because a few months ago they came to me to say, we want him off steroids.  Then we decided to add back in the methotrexate.    When we pulled that without weaning, I knew it was a JDM rule violation.  YOU DONT REMOVE, you step away slowly, and see what the immune system will do.  At the time however, we were fighting constant infections.  Our fear is one of the times, it was doing to do more damage then we could control.  Infections are dangerous.  They kill people.  So based off that we felt it was necessary to violate that rule.   We also added more Imuran.  Imuran gets my vote.  I don't know why.

That evening after our drive home, I want to say that I just accepted the way things are.  The way the ended up.  But no.  I began to obsess over it a bit.  Nothing unhealthy mind you but, it was like running around an art sculpture.  Looking at it over and over and over again.  Trying to figure out what it is.  Trying to figure what is going on.  My instinct was telling me that it was not JDM.  That this was not his dermatomyositis flaring.   It was what I did in the beginning.  Maybe he has allergies.  Allergies to what, well himself we have established.  But even the doctor asked have you switched laundry detergents?  Anything new?  I want to be insulted by that but I was right with them.  Let this be something else.  Not his dermatomyositits.  There was just no definitive answer.  I walked out of that appointment feeling like we had too.  We have to treat this like a flare because it would be bad not too.  Even if its not.  That night at three am my eyes flew open.  He is at his worst when he just home from school.  Why.   This started back in September, when school did.  What is it at school that is making him do this.  Sunblock.  I bet he is having an allergic reaction to the sunblock.   For a whole week we kept it off his face.  I even held the methotrexate hoping by taking away the sunblock things would magically return to normal.

UPDATE
It does appear to be a  reaction to the sodium thiosulfate. :(

The Bad, The Good, The We Shall See

Last time I updated my sweet baby boys blog.....can't remember.  I think I was getting tired of nutritional advice or something.  Still on the fence, but getting there maybe.

Recently
Recently we just got out of the hospital.  The catch up for anyone who reads this but not sure if anyone does and that is A ok, is we started a new medicine.  This new medicine is called sodium thiosulfate.  It is for the calcium deposits.  This is where the good and the bad come into play.

The Good
The good is Gary's dermatomyositis seems to be.......shhhhh.....quiet.  Dare I say it.  Nail fold capillaries look good.  Muscle strength is good.  Rash and skin are just fine.  Labs,  good.  They are holding to near normal.

So with Gary's dermatomyositis seeming to be under control we have proceeded.  We have proceeded on to trying to get rid of the calcium deposits dermatomyositis left behind.

In April we landed in the hospital for a week with a infected calcium deposit.  At that time we had the opportunity to do a X-ray survey.  That means they took pictures of his entire body to survey just how much calcium he has and where.  We talked about the next step.  Because now we are dealing with a new problem.  The problem is his dermatomyositis is clinically off.  (In my opinion).  Now his body is left dealing with these deposits it has left behind.  What is it going to do?  It is going to get frequent infections. Like a sliver in your finger or on the bottom of foot.   It is going to put puss and infection around that sliver to push and purge it out of your body.   You can quickly realize why that becomes sort of a problem and no fun at all for a seven year old.  These big chunks of calcium, all over his body, that are not suppose to be there.  Infect them out.

It has however been explained to me that since we started this new medicine which is in theory suppose to dissolve the calcium, it has created the opportunity for germs to be introduced.   Because calcium dissolving wants to come out.....so it does.  Through a hole in the skin that it makes.  An open wound that drains calcium.  Not cute or pretty but nothing from my son is gross or yucky.  Text book yes. Yes, not in my world!  Never will I ever say, "eewww gross".   EVER.  I will never make my child feel like that.  Ashamed or embarrassed over something he has absolutely no control over.  Over something that chose him.    I am getting off track.  So the opportunity for germs like staph to enter are numerous at this points.  Because the good, is sodium thiosulfate doing what it is suppose to?   The bad, immune suppression and frequent infections make for a bad recipe.

We were back in the hospital of May (Memorial Day Weekend) for a fever that would not go away.  It made us nervous but in the end, after almost 2 weeks of fever, it finally stopped.  That was a difficult weekend.

It took months and months but after the April elbow infection, we all agreed to give sodium thiosulfate a try.  Amazing work by his doctors.  I am so grateful for their dedication and determination they put into my son.  I am not always happy but 95 percent of the time,  I will sing their praises!!!

Sodium Thiosulfate
They had never given it to a child at Seattle Childrens Hospital before.  We were the first.  Yes, it was a little scary but with Gary's JDM ok, I felt confident.  I want these deposits gone!!!!!  They are disfiguring.  They draw attention from children and adults alike.  Some cause discomfort.  Some don't.  But it is in my opinion that they can't just stay there.  If it were my body, I would want me to do something to help.  If there was a remote possibility of them magically going away.  Then give it to me!!  The sodium thiosulfate has been given to a few other patients.  They all report it helped.  The first infusion was rough.  It made him throw up.  The second again two.  It took us a few weeks to get him so he felt alright.   Zofran first, tylenol and benedryl by mouth next.  Then the hour infusion of sodium thiosulfate.  Three days a week has been rough.  It kind of kicked our summer out the window by what is one summer?  We are talking about a lifetime here.  A lifetime being free of the disease and its effects.  So its worth a shot.

9 weeks In
About 5 weeks in actually, calcium started coming out from his arms, legs, and hips.  Not a lot but never before has it done this.  We were constantly walking around with bandaids, bandages, pads, gauze.  Because without warning, calcium would some flowing out.  As it exits,  it also creates the entrance.  And that is what landed us in the hospital this week.

Last Friday he started to complain about his elbow hurting.  I thought, "ut oh".  I nursed it through the weekend.  I gave him ibuprofen and did hot compresses.   This is not the first time this has happened. In August he had an infection is his leg on or around a calcium deposit.  It left an actual hole in his leg.  We have seen these before but it still freaks me out.  I mean, as a mother, my mind goes to these places, like, "oh gawd, is his leg going to rot off".  Well, Im not going to stand by and watch that happen!!!!!  Geez.  Exhausting I tell you.  Anyway, a quick ER visit and antibiotics fixed that up.  No swimming or baths for the rest of the summer but it healed.  Back to the elbow.....the elbow was tricky.  Ouch, ouch, ouch but not CRAZY ouch.  So Monday during our infusion I had them come take a look.  I was upset because he had just gotten off antibiotics from his leg.  How could an infection return so soon to any part of his body.  Well, it could and it did.   On Monday they gave him some intravenous pain killer and antibiotic.  They said if it gets worse before Wednesday infusion, call us.  I was happy with that. Until Monday at 1:30 am he spiked a fever of 102.   Another UT OH.  I gave him naproxen and benedryl and decided to let him sleep the rest of the night.  Call the doctors in the morning. I normally would have gone right to the ER.  But with the antibiotic on board I thought it will be fine till morning.  I called right at 8am.  I didn't sleep a wink the whole night.  I cleaned the bathroom,  scrubbed my shower out, and fretted.  He slept all the way to 11am.  At 8:30 they said if the fever returns, come to the emergency room.  I checked at 10am.  He stayed asleep.  98.6.  I was feeling pretty good.  But at 11am, he woke up and began to shiver.   So I started packing.

On the way to the hospital, he was alright.  I stopped to get him a gatorage and a treat.  I told him to drink because they could potentially put him on NPO.  So he drank and ate a little chocolate.  We were five minutes away from the ER and he vomited.  All over my car.

In the ER they were a tad frantic about things but I remained calm.  Three patients came into ER at the same time.  Gary being one of them.  They assess who needs to be seen first.  Gary was covered in vomit, pale and red at the same time, and shaking uncontrollably.  They pointed us and told the other patients to have a seat first.  We were immediately taken back.  They could not get a temperature read on him (meaning his temperature was normal).  But you could clearly see something wasn't right.

All of his deposits were inflamed and huge.  They pushed a little morphin because he was complaining his legs and arms hurt.  They also dumped saline into him fast.  He quickly started feeling better.  Antibiotics were ran right there in the ER.  I already knew we were going to be admitted and stay for a few days.    In the next few days our hospital room was like a sandwich shop at lunch time.  So. Many. People.  Key people however was rheumatology.  We all agreed we needed to try and put a stop to these infections.  So, with JDM being hush hush in his body right now, SHHHHHH, I don't want his freakin immune system to hear this, we are stopping some immune suppression.  (My car once broke down on my because it saw me eyeing a new model of it at a car dealer ship.  Im superstisious so don't say anything).  After five years of methotrexate.  We are no longer on it.  At first they wanted to stop immuran.  I didn't agree.  I say improvement with Immuran. I have not seen anything out of methotrexate.  I would rather give that the cut then one that has kind of proved itself.  He has had other infections in the past and immuran had nothing to do with it.  But methotrexate has.  It has been there since May of 2009.  They convinced me to go down on immuran.  I said ok.  Plug your nose, jump in the cold water with me?  We were sent home on antibiotics after Gary completed an MRI awake.  Loopy but awake.  It confirmed cellulitis in his elbow.  We believe it is what made him sick.

There is no case like the other.  We can compare.  I like that.  But all children get handed a level of severity of this disease.  You cannot take a child who has a mild case of juvenile dermatomyositis, who quickly goes into remission, to another child who rolls in and out of remission, to another child who never see's it.  It matters not what doctor you have.  If you believe that then...........

Disease Giver

I am the great disease giver.

I give you dermatomyositis.  You are suddenly having trouble getting up the stairs.  A strange red hot rash is on your face.   You have an unexplained sore under your arm.  You don't recall hurting yourself.     You feel overall tired.  You thought it was hard eating a sandwich the other day.  You had to work to swallow it?    Now, eat your way out of it.  Avoid certain foods.  Intake of others that will make you feel better.  Happy healing!!!

Another Trip Up

A few weeks ago, on a Thursday, Gary woke up and did not want to go to school.

This is not out of the ordinary.  He does not enjoy school.  Everyday the words, "I don't want to go to school."  He will often follow it with every excuse why not.  Including difficult ones for his mother to decipher like, "I'm so tired."  I have to make decisions on whether to push him to go, or ease back and let him rest.

This particular morning he planted himself on the couch during breakfast.  I tried to lite his fire to get him moving but he just layer there starring.   I finally gave in and said, "Ok, lets take your temperature."  As the thermometer beeped, I looked at him and said, "Guess what, you do get to stay home.  You are funning a low grade temperate."    He didn't celebrate.  Normally he would declare success but not this time.

I called the doctors right away.  On Tuesday they had seen Gary in clinic.  He complained a bit of ear pain.  At the time they didn't see anything but along with a running nose, it could have been the start of an ear infection.  They called in a prescription for antibiotics.  It was delayed because it was called in to the wrong place.  So 24 hours later, he was finally able to start it.  He started running higher temps, and was getting severe chills.  (Children or even adults that have ports in, this is a sign of infection, so I have been told).  After being on the antibiotics for less than a day he began vomiting.
I called the doctor on call and said, "how could a cold turn into vomiting?"   I was getting really nervous at this point.   He said it very well could be a virus and there wasn't much they could do for him.   I hung up a little miffed but.....hey I get it.  I have a child with a disease who can get sick like regular kids too.  Its not fair but, shrug, what can you do?  OH I know what I can do.........drive him to the ER.   Sorry, I am not going to just sit around watching my kid vomit with high fevers,  day 5 now of no eating.  So the next morning we took him to the ER.  I couldn't just wait and see if this was a virus.  Honestly, I just wanted them to check him out,  make sure all was ok, and that he in fact was just sick.  We did expect a quick in and out.   A blood test showed counts that we not concerning but expected for someone sick.  (Expect for his white count, usually that elevates when someone is that sick and his was not).  They did a swab of his nose.  It did indeed come back with a positive result for the rhinovirus.  The common cold.   Viruses are tricky.  I immediately asked how could a cold virus make you vomit?  The doctor said they could do whatever they want.   They affect us all differently.   We were ready to go back home and tuck our little guy back in bed.  But they kept us instead.

I suppose that is what I wanted however.  They wanted to run a wide spectrum antibiotic to cover him just in case.  Keep him hydrated.  Make sure his blood didn't grow anything, because it has before.

So Memorial Day weekend we spent, camping in the hospital.

We were over due for a infusion of IVIG and due in Tuesday morning at 8am to get it.  That would mean we would go home, then wake back up only to come back to the hospital.  They agreed he was ok to get his infusion.

We arrived home at 10pm on Monday night.  Thank you to my sister for helping with the older kids. I felt bad they missed  the weekend but I made it up to him.

The doctor on the phone was most likely right about the virus.  Even after coming home, he still ran fevers and was just knocked over.

That was the longest seven days of my life!!!!  Ok that was an expression but I am soooo glad he is finally doing better.  All the kids pants are falling off him.  But he is recovering.  Going back to school this week was really hard for us both.

We did make up our three day weekend.  We took a mini trip out of town to see one of our favorite families in Oregon.   It was just what we needed.

So far so good with his disease.  Usually when viruses invade the immune system, it can kick off a flare.  Gary's body in the past two months fought off a infection in his elbow and now a virus.  As far as I can tell, all is quiet on the JDM home front.  I hate that he has been going through so much but I love we aren't dealing with that.

MPeyeluvu2

Healing

We are healing.  Thank goodness.

Gary returned to school last week on Monday.  He made it all the way to Wednesday.  Wednesday he barely made it in the door.

Things I Don't Like
Every day he would come home from school and run straight to the shower.  Why.  Because he kept his coat on all day because he wanted no one to see his elbows.  My son does not like to be hot.  Never has, never will.  But apparently in the name of not having to answer questions and being ashamed, he kept his coat on as the temperature soared.  I mean, it was warm for our part of the country.  But he has always struggle with being warm from the meds and the weight of the steroids.  So it kind of did break my heart a bit.

When Wednesday rolled around, his exhaustion set in.  The swelling of his elbow was up.  Instead of swimming in the pool and visiting with his Uncle who was in town, he went to sleep.  I decided that the rest of the week would be sent at home.  Resting.   Recovering.  Too much.  Too soon.

Sometimes I wish God would leave a note on the counter so I could read it in the morning.

"Keep Him Home Today".

Fortunately I felt the message.  It was exactly what I was suppose to do.  The antibiotic he has been on was completely necessary, but completely kicking his butt.  The next day was spent on the couch.  Not eating, just laying.  Just recovering.  I suppose I should have done that since Monday.  I feel pressure thought to make sure he is learning.  He had already missed all of last week.  I have already been talked to about him being held back.  (Yes, he is protected by a 504 or the IEP, but still they bring it up how they don't think he is ready to go onto to the second grade because he misses too much school).  At one point I have to let that go and know that it will all be ok.  It will all work out.

Taking the rest of the week off is just what he needed.  By Saturday his elbow was down in swelling dramatically.  By Sunday, he woke me up in the middle of the night to help him itch it!   I celebrate that because that means healing.  The other elbow that has calcium coming out is calming down a bit as well.

His surgery site from April 1 is looking very good!  I am happy about that.

This week we are due back in for a infusion of IVIG and Orencia.  I am not looking forward to his labs per say, but I am.  I am looking forward to seeing they have returned to normal since the infection. 

HARD

After this last hospital stay, and in four days our anniversary of 5 years of JDM is here, my emotions have been, well, a bit raw.  I am having a hard time being away from him.  My heart hurts every second he is at school.  I fret quit a bit about how he is and what he is doing.  I even fret when he is next to me.  I think I may be experiencing some sort of syndrome since coming home from the hospital.  I act like, oh been there done that sort of thing, but it catches me.  

One of my fellow Moms wrote earlier this month about the mortality of her child.   I think when our kids have been through what they have, it has to be somewhat natural.  ***Anyone can go at any minute doesn't help nor does it help***   I am aware that stepping out your front door could be your last step.   No one is promised tomorrow.  But sometimes that concept feels like a pie, with a few slices taken out. 

Going Along Fine......And Then

I try to keep up with this but sometimes..you know...life.  But life turns you to the subject at hand.

Saturday, April 19th

I enter the house.  My clothes stick to my body from my run.  I dart for the shower.  I am interrupted mid-dart however.   Where is Gary?  Why don't I hear him?  Where is his energy I usually feel?  It's not here.  I can't feel it.  I turn around and go back into the house to find him.  I find him.  He is sitting on the couch, next to the window.  He is just looking out the window.  Looking at nothing.  Just being still.   Feelings sink.   A baseball game later that day confirms I should start to get concerned.  He falls asleep in the car on the way to the game.  Unusual.  All's I can think is, "here we go".  Can't prove it, till it happens

Sunday, April 20th   Easter

We had a Easter Party this year.  I very involved Egg Hunt for the entire family.  It started at 2pm.  I was highly distracted because I was the host.  I hosted the game/hunt, and everything that came with it.  I had a conversation with my baby sister.  "Something isn't right.  I don't know what but something."   Having these conversations occasionally with her happily ended in nothing.  But not this time.  This time I even said it, "after the party wraps up, I am taking him to the hospital, have him  checked out.  I don't know."  You always seem to second guess yourself.  You always think, naw, not this time.   We gave him IBprofen after a brief screaming fit with his elbow hurting.   We also noticed he began to cough.  And cough.  And cough.  His older brother had come down with a  nasty head cold.  It looked like he had also picked it up.  The last cold virus he contracted caused joint pain.  Was that it?  Is that what I was seeing?  The Ibprofen worked like a charm.  Kept him comfortable for the Easter Celebration.  Clue number two, emotionally he was a wreck more than normal.  Off the charts behavior.  Like messing with Grandma.  Everyone knows not to mess with Grandma. He did.  Big Ut Oh.   At bed time I gave him Ibprofen again just to make sure.

Monday, April 21

I had not even opened my eyes yet and decided he would not be going to school because of all the coughing.  He joined me in bed.  I briefly cuddled with him, but decided to get up and take a shower.  Get my day started.  His brother and sisters were still in bed.   I was dressing when I heard one little moan.  "Ouchie", I heard coming from my bed.   My cell phone was in front of me.  I instantly called to leave a message on the nurse line.  I explained the sequence of events on the nurse line.   I asked for someone to call me back so we could talk about what the next step might be.  No more than twenty minutes had passed.  I started packing our suitcase.  I explained to Gary that we needed to go to the hospital.  His sense of urgency and discomfort was at a level of a broken bone at this point.  It came on very hard, and very fast.   I frantically packed everything I could.   I had the two other kids to check out but no time for that.  As we were heading out the door, the phone rang.  It was our nurse.  I believe we have had this conversation before.  Probably exact, word for word.  She advised I take him into the ER.  I replied, "we are already on our way.   No offense, we are not waiting.  We are at threat level midnight.  I repeat, Code Red people."   She stopped and said, "ok, we are expecting you!"   With a red carpet please.

The ride was horrible.   He screamed and screamed.  I could barely keep the car on the road with his plea's for me to help him.  He screamed his arm was broken.  "IT'S BROKEN!!!!" thru hot tears.  I was able to talk to him and talk him back to where I needed him to be so I could drive.  We were about to pass my husbands work exit.  I thought screw it.  I need help!   I desperately and quickly drove to the front door of his work.  Practically parked in the entrance where people walk in.  I knocked on the door and said, "I need him now."

Back on the freeway.  We did our best to make him comfortable.  Loud crying and completely agony. We finally reached Seattle.  Just past the Seattle Seahawks Quest Stadium, he passed out.  He went from yelling to instant snoring.  His lips were white, but it was so much better to see him get that instant of relief.  Don't worry, we made sure he was breathing!  He woke backup less than ten minutes later.  We finally arrived at the ER.  They wheeled him right back.  We were able to talk him into numbing his port in the car.  Lord knows how we did, but I was glad we did.  That meant as soon as they were done assessing what was going on, they could hook him up to pain medication and get him comfortable.

I already knew what he needed.  I knew we would be admitted.  He needed tordal and antibiotics.  Just like last time.  In September of 2012, this happened on the other side before.   It was a little hard getting through the typical student and ER docs, but my ANGEL of a rheumatologist showed up and helped me out.  I told her my feelings were if they sent us back out of there with a dose of IV Tordal we would just bounce right back in.  I told her this is an infection.   Dad was not on the same page as I.  So there was a brief disagreement between us, in front of the doctor (lower my head, blush).  Communicating your maternal instinct can be difficult.  I just know doesn't always cut it for anyone, even the father.    I accidentally said shut your pie hole.  I would like to say it was unprofessional of me but mothers are not professional, they are way beyond that.  It was just fine later on.  Forty-five minutes to be exact.   A fever.   A fever of 101.5.    We were already being admitted before the fever set in.  Because my rheumatologist rocks.  A total rock start.  She was just hired officially on staff at SCH.  She is amazing.   The words are off the page with how great she is.....I wish she came with experience but what she doesn't have in that, she makes up for in listening.  Good hire SCH.

After we got over our little tiff (me and Dad) they ordered a ultrasound to see if they could see an pocket of infection.  We wanted to fight it because it meant them applying pressure on the elbow.  But I also agreed with there argument.  One was going to be ordered anyway, and getting into one on the floor as an in patient is time consuming.  

We were put in isolation because of the head cold.  He was so sick in the room.  :(   Every 4 to 5 hours the fever would break back through the tylenol.  A wide spectrum antibiotic was ran.  Covering our bases.  We ran labs.  Kept him comfortable.

As soon as the tordal would wear off we knew it.  He would ask for his elbow to be wrapped.  We had an opportunity to meet new rheumatologist on staff.  That was a good opportunity.  I was asked a lot about the behavior of the other calcium deposits.   This is what this was.  A calcium deposit had become infected inside his elbow.

April 21-22

We ran the antibiotics through his port along with the pain medication.  By Wednesday they wanted to get us out of there.  Rounds were at 900 that day.  Before examining him, I stood in the hall with a group of people discussing what was next.  They wanted to send us home on oral antibiotics and oral Ibprofen.  We had switched over to it already on Tuesday.  I told them point blank, I wasn't feeling confident.  I told them however, I knew where they were if it didn't go well at home.  I said my maternal instincts wasn't feeling great, but ok.   Orders were in to go home.  They went in one last time to have a look for themselves as they were basing this off the interns observations.  She was ready to kick us out.   Until the head doctor examined him.   He said he expected him to be using the elbow more, and not still be protecting it so much.  That concerned him.   Yes, it concerned me too.  I mean I wanted to go home, but not with a child that was not healthy.   He ordered a MRI.   Oh the dreaded MRI.   We did not want have to do with general anesthesia.  We had tried in the past to do it without.  At the time, he didn't even make it to the DOOR of the MRI machine.   So we tried a little benadryl.  He went NPO right then and there.  Nothing to eat or drink in case they needed to fully put him under.  That was my order.  I knew our chances weren't great.  Sure enough.  It didn't work.  They wanted him to straighten the arm and hold still for 20 minutes.  We walked away defeated but what could you do.  We were hoping to get it done, see nothing, then go home?  But I already knew that was not what they were going to find anyway.  So we went back to our room to what to hear what was the next step.  Was it important enough to put him fully under and when could we get back in on the schedule for MRI.  Especially after hours.  Our nurse (WHO WERE ALL ROCKSTARS) said it would not be till the next day at this point.  I asked them how are we going to keep him NPO that long : (  ........  She said she would call and find out.   She came back in the room with big eyes.  She said, "are you ready for this? Your heading back down in 45 minutes!".  I thought it was because we are VIP but it turns out, we have really great doctors who don't stop.

Down in the pre-anthesia they explained they were going to try this without full general.  And just heavy sedation.   It worked!!!  They got him asleep with heavy drugs.  No breathing tube.  He was returned to me after midnight.  It was a nerve wracking 7:30 to midnight.  I was so completely exhausted I thought they sedated him too much and were just to afraid to come and tell me :(  I didn't cry though.   He was loopy.  He was saying things like, "Tell that doctor Thank YOU.  I mean, tell him THANK YOU."   Then he happened to......toot.   Real loud.  He said, "tell him that too."   I was like, "oh my son, I will not."   He then put down a giant sandwich at 2am in the morning.  I swear it was like visiting my young adult years.  Having a great time with my buddy, eating at 2am.  
The MRI revealed an abcess

I mean we knew that already but the question was how big and where and what to do about it.  After three days of antibiotics, they weren't certain antibiotics would be able to penetrate this.  A surgery consult was called in right away.  They instantly came to the room.  We were very familiar with the surgeon.  He had just removed a calcium deposit/molluscum off of Gary on April 1.  (That surgery went well and this infection was not related to that surgery).    He examined the elbow and said no.  He said he didn't want to touch.  He order strong IV antibiotics and hot compresses.  I disagreed.  There was something different about this.  It wasn't acting like it was going to come out.  Although there was a spot of calcium on the center of his elbow, it looked tricky.  It looked like that was the infection.  But sure enough it was not.  We did what he asked.  Packed it in heat and upped the antibiotics.  Rheumatology was not satisfied with this.  I agreed.  You could clearly see on the MRI that the infection is walled off.  Our doctors were able to get a compromise in.  They compromised having radiology go in with an ultra sound guided needle and drain.  Easy peasy.  No food after 2am, they took him down at 8am the next morning.  They put him to sleep and found 2 abscesses.  

I will spare you the debate and the conversation between the surgeon and I.  After the wheeled him off I was a little mad about the indirect comment of "I talked to a lot of people of this", AS they are wheeling him back.  Do your job, and shut it.  And there it is.  My temper.  I honestly got back to our room and nearly broke down.  I am Mom.  Just a Mom.  No I don't have  a license for it but God downloaded an instruction manual in my head, so TAKE THAT.  I was able to text my sister and calm down.  But I felt emotionally beat up.  

After the procedure they kept us one more night for observation and IV antibiotics.  He will continue to be on antibiotics for 2 weeks to help with continued healing.  

I can hardly believe we have to get back to business as usual tomorrow.  I am not ready yet.  I want everyone to stay home.  But, doesn't work that way.

Special thanks to my family for your support.   Helping with brother and sister.  Thank you for checking in on us.  Thank you to the Saas Family for visiting.  I had the most wonderful time with my niece.  It made that afternoon much more bare able.   I am so lucky.

The good news is although his JDM labs were a little bit out of whack, his JDM doesnt seem to be flaring.  We got IVIG in the hospital with a small dose of steroids to keep on schedule.  

End of the Year

I can't believe the last time I updated this was in September.....I suppose I will just sum up the rest of these past months to end the year.  I always said I would not be one of those who blog drop.  Just forget about it.

Gary is doing good.  We are coming off of a pretty good flare.  Still determining what caused it.  It was enough and long enough to warrant a medicine add.  What was most important that we did not add more steroids if we didn't need too.  Last month Gary was pulling full days in first grade.  Even riding the bus.  Suddenly his attitude changed.  His self changed.  He was angry a lot.  He was not happy.  Eventually, he would head to his room and insist he lay in his bed and relax.   If we did have an errand to run, it ended in full on misery.  It was hard to be around him.   Then some hip pain, swelling of his face, super red rash all over.  He would look chemically burned especially if he became active.

Right now he is getting IVIG every two weeks.  He is getting 350mgs of steroids every two weeks.  He gets Orencia once a month.  His labs have been generally good.  We did have a few in the past three months that jumped but came back down.  It literally was like a his labs were on a bumpy road. Not enough to make changes, but enough to add that bit of information to make the recent change.  We can't call that stable.

Gary's calcium deposits are doing.  They are still there but no new ones.  The ones around his bottom have been slowly shedding out.  They bother him but don't hurt him.  During a recent X-ray, they thought he was hiding something or things in his underpants.  It was showing up on the X-ray.  It ended up being calcium deposits.  It didn't take me long to figure it out.

With this recent flare, school has become a challenge.  Gary seems to be putting a lot of pressure on himself.  He gets overwhelmed.  Academically we want to give him extra help.  By the time he gets home however, he is done.  It will be ok though.  It will work out.  We will find what works for him.

This new medicine we added is called Immuran.  We have to be careful to make sure he eats with it but he gets really nauseated.   So far so good though.  Immuran is another immune suppressor.  It is two yellow pills.  It is suppose to be a medicine that blocks the skin pathway.  So we will see.

Not the Worst

After our clinic appointment on Sep. 3, it was decided that we would go to see our GI doc, check in with him.  Well I expected that to take awhile but they got us right in.  I explained that we were seeing a bit of increase of diarrhea.  Nothing crazy but here and there throughout the summer.  So after collecting a "lab sample" which Gary can give on demand, turning it in to eliminate viruses and bacteria's that could cause, the doctor decided he wanted to do a repeat scope of both ends.  Again, I thought ok, I agree we can't be to careful with Gary.  I definitely didn't was make him go through that if it wasn't necessary.

I expected that to take awhile to get in.  But nope.  They got us on the schedule immediately.  We even had to stick around on Monday the whole day to hit a pre-op appointment.  That meant Gary was to immediately alter his diet for the bowel prep.  We had a light breakfast that morning.  Gary was already talking about lunch.

The nurse said to run out, get lunch, then start.  We had to avoid certain foods like fruits, vegetables, nuts, seeds, red dye.  So that wasn't terribly hard.   I decided since Gary had to go through it I would do.  All except the laxative part.  The next day Gary could only have clear liquid, jello, and Popsicles and 8 glasses of water with 3 doses of Miralax four hours apart.   Since I wasn't going to do the laxative part I completely fasted and did water only.  After 7pm the night before Gary could only have water.  By 8am the morning of, nothing by mouth.   He truly handled it like a champ.  It got a little rough in the morning.  We had several conversations about why he couldn't have anything.  He kept forgetting.   Our appointment was at 12:15.  We arrived at the hospital by 12:00.  At this point, Gary's body was starting to respond to his fast and clean out.  He became extremely lethargic.  He was getting the hot and cold sweats.  He basically could not stay awake.  He passed out in the hospital waiting room.  It was about 45 minutes past our time they said to be there so I went and asked.  He was sleeping on the floor at this point.  I felt his pain but I didn't do the laxative so at most I was spacey with trouble focusing.

I approached the desk to ask where we were at in line.  I asked how much longer it would be.   I informed them that he was passed out on the floor.  They said it would be soon.  I asked for a warm blanket to keep him covered.  At 1: 30 they came out to bring us back to the room prep.  We wheeled him back in the wheel chair.  He was even having trouble holding his head up and keeping his eyes open.   I was thankful he slept but it was hard seeing him in that state.  We got him in his gown.  Finally got his port accessed.   And at 3 o'clock,  he went to back.

I was able to go all the back with him.  They let me help with the gas.  He was so lethargic that he did not fuss once bit.  Very little anxiety.  He didn't have anything to give.  His eyes were glassy.  He could barely respond.  I was concerned but he did answer questions, and even gave a half smile to a nurse.  I was so happy that got on with it.

The results weren't perfect, but they were better from his last scope last year.  I think it has been even more than a year?  His colon did have signs of ulceration's, but I think those were the old ones.  The only other thing was 2 parts of his stomach were irritated.  His esophagus looked fine but further down the tummy right before the small intestine, there was inflammation.  There was no ulceration's just irritation with a little blood.

Yes, I wanted him to come out and say, "everything looks normal".  But with what he saw, the doctor is almost certain he would like to run the medicine we gave him last year, Remicade.  He told us that he would wait for the biopsies to come back but he was pretty sure he leaning towards that.  I told him that a discussion with rheumatology will definitely need to happen as I had talked with them about we were worry that the Remicade worked really great on his IBD or Crohn's, or whatever they want to call it, but not so great on the JDM.  We can't be sure it was the Remicade that suddenly had him limping and having trouble swallowing.  I would hate to go on, well lets try it and see.  That is hard.  I am very curious to see what rheumatology has to say about it.  I was clear with one thing.  I do not want an increase in steroids.  I mean if it was a life saving thing then of course.

Right now we are pretty tired.  We still have a MRE to go in the next couple weeks.  Tomorrow we have an all day infusion.  This was a bumped infusion from Wednesday because of the scopes.  They originally wanted to do it today (Thursday).  We did not get home until after 8 last night.  He was exhausted.  He slept so much in recovery.  He woke up crying and needing to go to the bathroom.  The first thing I had waiting for him was a chocolate starbucks frappacino thing (no coffee in it of course).  I started to let him drink it when the transport nurse stopped me and said, "he is going to throw that up with the anesthesia in him!".  I apologized but was like so be it.  I told this child he had to make it  to 12:15.  You guys took it all the way to 3 o'clock.  But that was my inner dialogue.  I fully recognize they don't have control over everything.  Besides Gary did at least sleep. I kept giving sips of his drink when they weren't looking.  It is my child, my vomit, I can clean it if I want too. I just knew that he wouldn't barf it up.  He didn't either.   If he did, at least it would taste good.

I am so impressed with my son.  He is starting to handle things a little better.  It makes it a little less challenging dealing with a that 2 to 5 year old when all he did was cry and behave badly in response to what was happening to him.  Now I think he understands a little bit better.  I wish he didn't have to but he does. I can be sad for that but no use in getting angry by it from a mother stand point.  That just doesn't do any good.  You do what do.

CALCIUM
Wow.  On top of that we have been dealing with calcium deposits.  He has a deposit on his elbow and another in his bottom that have made there way to surface of his skin.  The one on his elbow grew to the size of a larger marble.  After a hot shower it burst.  I know it sounds gross.  I am just completely fascinated by it.  The one in his bottom is quite tender.  But the one on his elbow doesn't seem to bother him like that. Once it released a little bit, it seems to fill back up.  So now every time he take a bath or shower, it releases calcium.  Gary helps it along by gently pushing on it.  I will stop right there with the details.  The one thing that is important to me that no one in this family is allowed to say is, "gross".  I do not want him to feel embarrassed or ashamed by this.  I encourage everyone to maybe raise an eyebrow or say "wow"!, but it is just so important to me that no one acts as if it the most disgusting thing they have ever seen.  I don't care if you do.  Don't show it.  I'm sure he knows it yucky.  The tender deposit on his bottom might need some sort of wound care eventually.  The open wounds do concern me but they seem to seal off with calcium when it dries up.





Thank you so much to my baby sister who came with us.  It makes such a difference.  He loves his Aunties to pieces.  It was a big sacrifice for her because she hasn't been feeling good.   Don't worry, you can't catch what she has : )

This morning he sleeps.

FASTING
My husband was suppose to fast with Gary and I.  I understood the other kids not doing it with being in school and everything but.....we agreed we would wait until Gary was done.  I estimated it would be about 3pm. So I told him at 3pm he could eat.   He made it to 11:45.  Good thing he was at work.  I understand you have to be at work, but if your not gonna be with him, then at least starve to death, LOL   :)  It was less than 24 hours for Dad.

Summer to Fall

I think this might be the longest stretch I have gone without posting an entry.  We left off in June.  I suppose I will just dive right in.

RE-CAP
So we did a little taper at the beginning of summer.  It started with his home does.  We went from 10mgs to 8mgs.  That was successful.  Very small move.  The next change we made was the IVIG and Methopred dose.   He was getting 350mgs of methoprednisone once a week.  IVIG and Orencia once a month.  I wanted to try splitting the dose to IVIG up.  My reasoning was his skin became so clear after those monthly infusions but didn't last long.  So our doctors consulted with the experts and they agreed it couldn't hurt.  That is when we went ahead and implemented the second taper.  We did not go down in dose but rather spaced out the steroid to every two weeks.   That was in July and August.  Our September appointment was yesterday.  Gary is doing well but not well enough to keep tapering.  We had a few labs tick up twice.  Not jump up, just slight tick up a few points.  His AST and ALT are a low high.  I really wanted to hear lets keep tapering.   There are smalls signs of inflammation.  Calcium deposits have been making there exits.  Our doctor says she would be more comfortable if they were just gone first, then taper steroids.  But we have to try.  1 year and 2 months of once a week methopred pulses is enough.  But if we get to aggressive with our taper, that could lead us back to a place we don't want to be.   A place we are heading?  His cuticles look great.  His skin is ok.

Two years ago when we tapered all the way down to 1mg of prednisone with no weekly pulses, it ended in disaster.   The onset of this diarrhea.  Nothing that was alarming but just enough that we ended up with one little sick boy.  A boy who wasn't absorbing medications.  And now, it is slowly making its return.  I am thrilled with how well Gary does with what he has.  I am being pulled into the great darkness of worry again. At the beginning of summer I was feeling hopeful and empowered.  I told them to be careful with me.  I am in this game with myself of were tuff, we can take it.  Let Gary's body figure this out on his own.  I want to see him normalize.   Feels like my rug was pulled out from underneath me yesterday.  I am standing backup however.  We will get this figured out.  We have a repeat stool  test and see the GI doctor on Monday.

SCHOOL
Today was the first day of first grade.  I made it all the way out to the parking lot before I boo hoo'd!  Yay me.  Drove to school at lunch to check on him.  Put more sunblock on him.  Everything was going great which made me feel better.  Cookies and fresh  bread are baking.

Steroid Taper: Ready or Not

Summer is here.  Kindergarten has been completed.  It definitely was a rock n roll kind of year but we muddled through somehow.  Gary finished out the year amazingly well.  We did get to the end of the year only to find out there was some error in communication.  He was under doctors orders to have sunblock applied mid day, everyday.  Not sure what happened but that stopped happening.  That is ok though, well, it is unfortunate but since he will be going to school full time in September, I will drive there myself at noon to apply it.  I put it on in the morning but it needs to be applied again.  So.

Gary is doing pretty good.  I am really sick, and concerned about too much steroids.  Last week at our monthly admission of IVIG and Orencia infusion I let it be known.  We are not sure if his disease activity is off but trying to figure out if his body is just steroiding out (red skin, muscle irritation) or if the steroids are holding JDM and it is still getting through.

GETTING BOLD
The first bold attempt we made was holding off on his Rituxan treatment.  We are in and have been in the war of what is working and what is not.  So in May I was schedule for surgery.  I had to call and delay the treatment until I was better.  We had already talked about not giving it.  Well I had said (before I even knew about my surgical future), what about not giving it to him?  The doctor shrugged and said, "oh yeah, why?" and "ok/".  We are all really at this lets cross that bridge when we get to it in his treatment.  I think we all know that we need to make a move.  We need to see what his body does.  It is like playing Texas Hold Em.
I know this can get dangerous and only end in a monstrous flare.  The old me would have been on the NO WAY train but I really want my little guy to have a normal life, or something that resembles it.  He sported the moon face the entire kindergarten year.  I hope we can start first grade with no cheeks, and no flares.

What starts to stop me in this is his calcium deposits.  Some have gone way.  Some have shed out.   Some are large.  Some are angry.  Some are staying the same.  What I haven't noticed is any new ones.  It is often hard to tell because he has so many.  Trying to figure out if something is new, is next to impossible.

So the taper.  The doctor decided to first mess around with his home dose.  We are going from 12mg to 10mg, to 8mg.  Meaning this week he went down to 10mg.  In two weeks we will try to nudge down to 8mg.  So no Rituxan, and tapering steroids.  Told you we are getting bold.  He will still get a weekly pulse of 350mgs at the infusion center.  Sure it is nerve wracking.   Two weeks ago his ast and alt popped up but came back down on there own.  So my theme for this summer is, "On Your Own".  Come on Gary's body, do this on your own.

NOT ABOUT GARY: SHOTS and IV's
I had a salpingo oophorectomy on May 28th.  It is the first time I have ever had surgery.  There was a large mass on my ovary (10cm).   I got to experience the weigh-ins, height measurements, temperature readings, arm huggs, shots, pokes, prodes, medicines, ect,.  Everything my little man goes through.  I am still not over the shot of heparin they gave me in the back of my arm.   They did an excellent job with my iv's. I had a total of three.  But the big one, the man nurse did amazing.  I didn't even feel it.  It was on the top of my hand too.  However, I have had IV's started when having the babies, and those did burn and hurt.  Not trying to take anything away from Gary by saying that.   The month of May for our family was pretty stressful.  Walking around not sure if you will get to grow old was hard to take.  I went from running 50 miles a week to a stand still.   Like hitting a brick wall.  My family did a wonderful job supporting and nurturing me through it.  Recovery has been good but a little hard.  My diagnosis ended up being benign but I did have endometriosis.  I get the pleasure of taking birth control pills to stop it from happening again to my other ovary.  I am post op 4 weeks tomorrow.  I am still uncomfortable at times, working my way off of narcotic pain relievers.  I am having a really hard time sleeping but as soon as I can get back to my regular exercise it will be better.   I can sweat this stuff out of my system and get back to me.  Did I tell you I got a shot in the back of my arm and there is still a big knot.  I don't know how these kids do it.  My favorite was when they took my temperature.  They handed me the thermometer.  I was use to having to watch Gary have it put in his mouth for him for the past four years, I opened my mouth and waited for the nurse to put it in my mouth.  When I realized she was handing it to me, I blushed. LOL.  I also gave Gary's date of birth a few times because I am in such habit of saying his numbers.

My next challenge is a 1/2 marthon in Chicago on July 21.  Yep, it should be interesting.  As always I am excited to here about the lastest and the greatest in the world of JDM.

I HAVE AN AWESOME DOCTOR
Well, Gary does.  Think I have mentioned my liking of her before but I'm back to praise again.  Dr. A is amazing.  She took on a huge project.  She charted and graphed Gary's care since diagnosis.  I myself had attempted it.  I had put it into a timeline app.  I had it all finished when the app crashed and I lost it all.  But I was moved to tears.  She handed me this 27 page packet.  It has a summary of every visit.  What is symptoms were, what he was on, ect,.  She also graphed it!   She got graph paper and was making a visible timeline.  They are in search of a connection.  TEARS  They often asked me has there ever been a time when you thought Gary was JDMless.   Like what medicines was he on when I felt like that.  My answer has always been, "I don't know."  We have always been on so many things at once.  Like cell cept and tacrolimus.  I think his skin was good back then but he had this under lying muscle weakness that I failed to see until JDM finally took over as the calcium developed and everything was abruptly changed because he was going over a cliff.  He had good skin for a short time but had a lot trouble with stairs, getting up on beds, into cars.  We always just dismissed it away or being on eveything he was on you completely rationalize it.  Now, if I could go back and whisper to myself, I would have said, "get off this train, its goes nowhere".  But just like we are right now, its the same predicament.  Do I feel like he is good enough to taper steroids.  It is hard to tell.  It is hard to say.  I am not sure.  I do know at this point in time we are ready to see what happens.  My biggest fear is getting him a path that we can't get him off, waiting for something new to kick in.  Or going back to what worked before, but didn't fix it.  Cytoxan, only got him so good.   Rituxan,  fine and dandy, but it didn't turn it off.   That is our ultimate goal.  A cure.  A cure is our ultimate goal.  

Thank you Dr. A!  We love you!  That packet is tough to read through.  It is like reading a story from the -Horror/Fiction section.

Ok, so I over reacted.

I would like to temporarily apologize to Orencia.   We ended up doubling the dose.  One last ditch.  I like what I saw.  I like what I see.  Remission worthly someday, maybe.  I don't know though.

We have some calcium deposits that are on the move.  That means they are coming to the surface, red puffy, but not bothering.  They look no good but this aint a beauty contest.

This was month number two of double dosing Orencia.  We were set to run Rituxan last week but for the first time in four years, I had to call and cancel.  We might run it next month.  I feel guilty because it was I just couldn't handle it right now.  I'm not ready to share why but I will.    Everything is for reason however.  I was hesitant to run Rituxan again because it is so difficult to tell what is working and what is not working.    Labs are good.  Strength is fine.  Calcium, no new ones but bigger.  Capillaries not bad just red fingers.

He is an amazing kid.  He is so angry but whatever.  What  a special boy.  He says the greatest things!

Thank you to my family for all your help with everything.  My baby sister is such a great mother to my kids.  They really love her and my brother-in-law.  She is almost too good.  Makes me wonder if other plans have been set in motion.  I know in my heart that if something like that is true, like I can't be here, she will be so perfect.

Orencia

Farewell.

Your not the one.

I tried.  I know we have only been in a relationship since December.  Harshly enough, we are breaking up.  You don't block muscle weakness.

Buh bye.  I will handing in my next resignation very very soon.  Kiss it too the.............phew.    

When family members notice strength isn't what it was.   When weird rashes appear that you try to explain away.  You make me grieve.

Conversation:  "We should sign Gary up for baseball."     "Yeah I don't know.   It's too sunny and he seems, not all the way."

A DREAM

Gary ended up in my bed one night last week.  I woke to him tapping me.  In a whisper, "Mom, do you think I am going to pass away?  What would happen if I did?".   In my light sleep, "don't, I would be so sad."

But if you need to some day.  I don't want you to be in pain because of me.

But know, we will FIGHT.  I will drag you there if I have to baby!  F to the U JDM.  That is F to the U to the returning muscle weakness.   To the labs that don't relieve themselves.  Oh, I got this.

Happy 6th Birthday, Gary

March

It's March.  Time is marching.   Fast.

This month as been pretty good.

Gary had weird rash pop-up on his face these past couple weeks.  It was confusing for me because the rash was exactly where a dermatomyositis rash would be.  Cheeks, across the eyes.  But this rash was a little different.  It was raised, little bit of dry skin over the top of it, and it was on only one side of his face.  Last week he woke up and it looked like it was making its way down the side of his face to his neck.  I called the doctors.  I explained what was going on.  I thought maybe he was having a reaction of some kind to the steroid itself.  We are after all still getting pulsed once a week.  We keep expecting see improvement with the pulses but we weren't all the sudden.  In fact, the methylopred or whatever is in it or the methotrexate or ect., was making it worse.  I was concerned he was allergic to something or JDM was being activated somehow.

I love my doctor.  She takes the time to listen, troubleshoot with me.  We problem solve together.  She checks in with our lead doctor.  Our main rheumatologist was not interested in making any changes.  I had suggested trying a different kind of steroid.  I know they have them but I had yet to do any research on them.     She didn't want.  Instead, they ordered a dose of IV benadryl and tylenol.  The reasoning was because the only time he hasn't reacted or he walks out of the hospital looking like a new man, is with the IVIG.  IVIG has given us trouble in the past.  This new brand however, stopped that and all seems well.  So they talked about the difference between the dose of methylpred he gets with IVIG and the weekly infusions.  What was the difference?  The benadryl and the tylenol.   Worked like a charm.

Once the IV benadryl went in, the rash on his neck instantly started to go away.  It took another 24 hours for the one around his eye to go away but, it seem to solve a problem.  Now, WHY?  I have concluded when we go to our next appointment in clinic next week, I will have them point me in the direction of the allergist people.  I wanted him tested for allergies.

SICK OF IT
I am growing tired of the media and there reports on what is causing autoimmune diseases.  Since Gary has been diagnosed I tend to pay to the news when the title of the article reads, "Autoimmune" and scientists.  You know, things of that nature.  Of course it is going to catch my eye.  So far I have read the following of too much causes autoimmune diseases:   SUGAR, SALT, GLUTEN, PROCESSED FOODS, SUN, GENES.  I would like to add my own.  The WIND, RAIN, GRASS, CLOUDS, SKY, YOUR MOM, YOUR DAD, YOUR CAR.. . . . . . . . . . ..........I am aging.  My tolerance is less.  I do feel a bit arrogant and ungrateful with the information shared.  Because what is comes down to is of course, I want to know.  If we truly new then we would know where to pull the plug on it.  I admit it triggers my "FAULT" emotion.  If indeed it is salt, well then, its my fault.  I fed him too much salt.  I fed him too much sugar.   But if that is the case then why doesn't his siblings have it.  Why doesn't my best friends son have it?  I will continue to read the articles.  But I don't know what to believe.  I have really started laughing it all off because it is frustrating for a parent.  I suppose I compare it when they first introduced cigarettes.  They marketed it was healthy.  People believed it.  I know that is a whole different realm.  I was pointed out the media effect I suppose.  Only God knows.  I can't wait to get up to heaven.  I will ask Him, "so, what is the cause really? don't worry I won't mad You can tell me."  No worries, He won't be mad at me for saying that, He knows my sense of humor.  He gave it me.

SCHOOL
Gary started at at new school.  The schedule of this school actually isn't too bad at all.  I was fearful but out the other two kids, Gary is doing very well.  I met his gym teacher yesterday.  He was very nice.  He made me smile because he admitted he was nervous with Gary.  He asked about his limitations, and his port.  I told him that, yeah, he could take hit for someone, and it theoretically could knock the port out of placement.  I explained however, it has been in since June.  Gary runs, jumps, wrestles, swims, bathes, everything.  I told him not worry.  I think Gary does a great job protecting what he needs to protect on his body.  I am glad that the transfer as gone smoothly.  Excellent school.   He goes full day on Monday, and Tuesday, and every other Wednesday.  I switched his weekly pulses to Thursday, gives him the weekend to recover.  Nice, huh?