Tuesday, November 1, 2016

Nov. 1st, 2016

I can't believe it has been an entire year  since I wrote on our blog!

Life changes, and things shift.  Finally.

Lots of bumps to get here, but we are here.  Bumps:   Liver complications.  Nodular Regenative Hyperplasia.   We handled it.  

An amazing visit to the National Institute of Health in Washington D.C.  Full work up with great news.  We were told Gary has received great care thus far from Seattle Childrens Hospital.  Over half of the calcium is gone.    We concluded with his psychiatrist because he has just made vast improvements with his anxiety because is health is doing better.

Currently  Gary still gets his infusions every two weeks.   He is very stable.   Next month we hope to start to taper steroids!!!   It will be the true test.

Last week we had a meeting about Stem cell transplant.   Very interesting.  Not on the menu though.

Gary is attending school everyday and he really loves it.

He had a "over do it" sort of situation with Halloween.  So much so that I got an email from his teacher saying he was worried about him because his legs and feet hurt.   He hobbled around all day.  I explained it is probably similar to the way a person shops till they drop.   Well he trick or treated till he dropped.    It's all for the candy.


Sunday, November 1, 2015

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.

Saturday, April 25, 2015

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!)

Sunday, April 5, 2015

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

Saturday, March 28, 2015

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?!


Thursday, March 5, 2015

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

Saturday, October 25, 2014

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. :(