Still’s Disease (aka SJIA)

No denying that when your kid gets sick (like really sick), it’s absolutely one of the most devastated and helpless feelings a parent could ever experience.  It’s one thing when your little one has a common everyday cold, a mild fever, even the stomach flu that will eventually subside and another thing entirely when they are hit with one pain/symptom after the next and NOBODY can tell you why or how this came to be.  I typically keep most of my family stuff off the blog here, but because things have escalated so quickly I felt the need to share our newest family story… or nightmare rather.

Exactly four weeks ago when we got home from volleyball practice, Makena realized she was covered in a rash we assumed was hives.  Nearly head to toe and everything in between.  Up until this point she had NEVER had any type of allergic reaction to anything so we thought it was a fluke, gave her some benadryl, and called it a night.  Unfortunately when she woke up the following morning, not only was the rash still present she now had a low grade temperature of about 101 AND she was complaining about her ankle hurting.  There wasn’t any significant swelling at this point, but she was definitely having a hard time walking on it.  We kept her home from school to rest and shake this “virus” off.

That following Wednesday she woke up feeling a bit better.  Rash was not as pronounced, fever was gone, and ankle did feel a bit better.  She went to school that day and even decided that she was well enough to go back to practice that night.  Reluctantly, I took her and within 10 minutes she was in full tears because of the pain in her ankle.  The rash was also beginning to start again.  After about 10 minutes, Makena could not bear ANY weight on her left ankle whatsoever.  Obviously concerned, we left practice and rushed immediately to urgent care.  She was glanced over, given a prescription of prednisone to treat the ‘hives’, and were told to follow up with her doctor in a couple of days.

Well, things continued to progress rapidly over the next 48 hours.  Fevers were running anywhere from 101 to 103, she still had some ankle pain with swelling, rash was alive and thriving, but now Makena was also having extreme knee pain.  It was so bad that again, she could not walk at all.  Not even a hobble.  Scared, confused, and helpless we booked an immediate appointment with her pediatrician who ordered a hefty list of blood work and some imaging.

She may look steady here for her blood draw, but I assure you… she is deathly afraid of needles.  She spent 20 minutes crying before the draw, during the actual draw, and then 10 minutes post draw.

Heartbreaking.

Then it was off to radiology to get scans of her joints…

As it turned out, she had various effusions (fluid) in her joints.  You can’t really tell from this picture, but the swelling was mainly above and below her knee cap.  The right knee was at least double in size as the left, with significant rash markings just above her knee and well into her thigh.

We were sent home and advised to continue the prednisone as directed, take tylenol for the fever, and await the blood test results.  That night it went from bad to worse exponentially.  Makena’s fever began to spike with very little relief from Tylenol or ibuprofen, she could no longer walk, and she began having difficulty raising her arms due to shoulder pain.  As if we weren’t already scared enough at this point, her blood work started to trickle in and we went from concerned to full panic mode.

Blood cell count was abnormal, blood was found in her urine, and her inflammatory markers (also used for autoimmune diagnoses) were beyond elevated.

6 X over normal and 14.5 X over normal…

ERYTHROCYTE SEDIMENTATION RATE (ESR)

CRP (C-REACTIVE PROTEIN)

That following morning we put an urgent call into her doctors office to discuss the new pain/symptoms, high fevers, and test results.  Within 20-30 minutes, her doctor called us directly and told us to sit tight just for a little bit while she spoke with a couple of other departments.  We were warned that bringing Makena in to the emergency room to be admitted was a high possibility. We made it very very clear that this would be absolutely too devastating and traumatic for Makena (and us) so whatever measures could be done to prevent that ER visit, we would take any alternative options.

20 minutes went by and we received another call from her pediatrician.  She told us that after speaking with emergency medicine, they believed this was something called “serum sickness“.  We were told to bring her back into clinic immediately to be evaluated again and run even more blood work.  Mind you, Makena’s absolute worst fear is needles and considering she just had 6-7 vials of blood drawn the day before you can understand how scared she was.  The entire car ride was spent in full tears, hyperventilating.  By the time we got to the doctors office and lab, we were all ready to pass out.

13 MORE tests.  Within 10 seconds of seeing all of the new empty vials that would need to be filled, Makena was on full blown fight or flight mode.  She was hysterical, shaking uncontrollably, sweating, and nothing could calm her down.

I do believe this was her first official panic attack.

It took two lab techs to do the draw.  Joe could hear her crying from the waiting room and I felt entirely & utterly helpless trying to console her.

She was evaluated, given crutches to help her walk a bit better, got a referral to see an allergist, and given another heftier round of prednisone (steroids).  This time we were to do a taper on the meds starting with a very high dose and reducing each day by 5mg until the medication was gone.  At this point we weren’t exactly convinced this was ‘serum sickness’ though because she had never had any type of allergic reaction in her life and also hadn’t been sick or taken any medications.  We rolled with it though and went home to rest from the days traumatic events.

Over the next few days Makena ended up improving, but only slightly.  We had her fevers more under control for most of the day, the swelling in her knee began to reduce, but the rash persisted AND she now had pain and swelling in her right ankle.

A couple of things we did begin to notice at this point was she usually woke up feeling more or less ok, not in any significant pain, no fever, and very little rash marks on her body.  However, usually around 3-5pm Makena would absolutely crash and burn.  Rash would begin to become more and more prevalent as nighttime minutes past, she was fatigued, extremely tired and lethargic, and fever would begin to spike, and Makena wanted no part of moving whatsoever.

Two weeks into this journey and we were at a complete loss for words.  We had barely seen Makena crack a smile during this time, definitely hadn’t heard her laugh or even joke around.  All she wanted to do was lay down, watch YouTube, and be left alone curled up on the couch.

Our Makena was no longer the same Makena she was just a few weeks prior.  We were devastated, helpless, clueless, confused, and beyond scared.  There was very little ‘comforting’ anyone in the house this past week.  Everyone was on alert, on edge, and left to be quiet in our own horrible thoughts & feelings.

After seeing the allergist last Monday he ruled out serum sickness and gave another possible diagnoses of ‘urticaria multiform‘.  We were told to finish out the round of steroids and cross our fingers that whatever was attacking Makena’s little body would be wiped out at that point.  Her final dose of prednisone was last Wednesday so by last Thursday/Friday we should know for sure if the medication did what it was supposed to.

Unfortunately, after the last dose of steroids each and every single pain/symptom came back again.  By the time she woke up on Thursday morning her fever was near 103, rash was everywhere, and now her hip/ankle/and elbows were beginning to cause her extreme pain.  Every single day without fail, rash would begin to appear around 3-4pm (usually starting on her face) and slowly progress all over her body.

Yet another call AND message were put into her doctors office and we received a call early last Thursday afternoon.  She had spoken at length with a doctor at Rady’s Children’s Hospital and we now had an urgent ad immediate referral to juvenile rheumatology to see the specialist.  She was to be seen first thing last Friday morning.

But sigh… he was requesting MORE blood work before her visit the follow day.  A few things repeat tests (inflammatory markers) and then another handful of other tests I had never heard of.

Devastated all around.

Full panic attack, uncontrollable sobbing, inability to breathe properly, fear, confusion, dread, and all out hysteria.

 

13 more blood tests.  Did I mention that already?

All we kept saying is “Oh my God, this is a nightmare.  Please make it stop!”

Test results began to pour in again.  Most of them came back within normal range, but inflammatory markers were increased AND another one was well out of range.

LACTATE DEHYDROGENASE

Note… One thing I hate about being able to see all of these test results so readily available to us online is how confusing they can be.  First you have no idea what each test is for without Googling and you definitely have no idea what each of the tests could be indicators of.  Anytime you do a search for “elevated XYZ levels” or “what does this abnormal XYZ blood test mean”… you always get the worst possible answers.

This particular ‘lactate dehydrogenase’ test confirms that Makena does in fact have cells and tissues that have already been damaged.  Where and what and the extent?  NO clue at this point.

You can’t imagine everything that began to swirl around our heads.

Again, can this nightmare be OVER???  It’s already gone from bad to really bad to worse .

Friday we woke up, got dressed, and headed to our appointment at Rady’s Children’s Hospital with juvenile rheumatology.  We had no idea what to expect, if he would be able diagnose anything, or if Makena would need to be admitted.  All I know is that it was the most quiet 30 minute car ride we have ever had.  Radio wasn’t on, nobody was on their phones, nobody was talking.  Everyone was in their own world, left to their own thoughts.

Our lives’ were forever changed in this room on February 10, 2017

After a very very lengthy hour+ appointment discussing blood tests, history, and the past three weeks of events Dr. Sheets felt very confident in a firm diagnoses…

Still’s Disease

It’s also known as an aggressive form of Systemic Juvenile Idiopathic Arthritis.  This is not the same type of arthritis most people get as they simply begin to age.  This is considered an autoimmune disorder where the body begins to attack itself.  In this case, Makena’s body is attacking the healthy cells and tissues in her joints AND causing systemic (“throughout the body”) misfiring as it tries to fight.

“SJIA is also associated with systemic signs such as swollen lymph nodes, enlarged liver or spleen, and sometimes inflammation of the lining of the heart (pericarditis) or lungs (pleuritis). Pericarditis may be an early sign in 10 percent of children.”

You can imagine the flood of emotions we were all faced with.  Dr. Sheets was talking the entire time, but I’m not sure many things were actually absorbed into our heads.  We were given a lot of information, loads of technical terms, his thought processes, prognosis, treatments we needed to immediately consider, and a bunch of other scary things that no parent should ever have to be faced with.

We were/are living every parents worst nightmare.

A sick child, a really sick child who now had a chronic condition that would more than likely be debilitating more often than not.

Makena couldn’t control herself in the office.  She was in tears the entire time (I was trying my best to keep my emotions at bay so I wouldn’t scare her of course… but it wasn’t easy… as you can imagine), then she got hysterical and had to leave the room once treatment options were discussed.  I took her down the hall and before I could say anything, she burst out and cried:

“He’s wrong.  He’s wrong.  He doesn’t know what he’s talking about.  I’m going to be better tomorrow and all of this will go away.  He’s wrong.  I don’t have this!”

I couldn’t control myself.  My baby was hurting.  She was scared.  We wept together in the hall way amongst all of the patients, nurses, and doctors walking by.  It was a moment in time I will never forget…  the world stood still and all we needed was to hold one another.

After a few minutes we gathered ourselves together. Makena went into the bathroom to clean up her face.  I went back to the room where Joe, the doctor, and now the intake nurse were talking about the “plan”.

There was so little time to process ANYTHING during any portion of our appointment last Friday.  All we did know leaving Rady’s children hospital was that Makena had a serious condition that could possibly effect her entire body and all of her organs.  Everything in our lives’ would change.

In the hustle and bustle of everything during our appointment, we did manage to catch a few words while processing…

1. treatment needed to be started IMMEDIATELY.  Because her SJIA onset had already been attacking her body for 4 weeks now, Makena runs the risk of many of her organs being impacted (heart, lungs, spleen, kidneys, liver, lymphatic system, eyes, etc.)
2. we needed to decide on which therapy we would initially proceed with.  Three options were given, but unfortunately they ALL were horrible.  Painful daily injections OR hour long IV infusions AT the hospital.
3. more tests and scans that needed to be done in the next 24-48 hours
4. urgent referrals for Makena to be seen in cardiology, oncology, hematology, AND infectious disease

What the actual F&*CK?

Are you serious?  Oncology?  We understand ‘why’ he wants her to be seen by the departments (to ensure nothing else was missed in his diagnoses), BUT that doesn’t make hearing ‘oncology’ or any  of the other ‘ologies’ any easier.

Again, the car ride home was spent in silence.  I sobbed quietly while staring out the window, Joe drove like a robot absorbed in his own thoughts, and Makena rested her head on the car door with tears running down her face thinking about God knows what.

Before we had any chance to even breathe properly though, we needed to head back to the hospital for the additional tests.  A chest XRAY to scan for fluid in her lungs and a TB test.  Again, her rheumatologist just wants to make sure that as of right now, her lungs are healthy and not being drastically effected by the disease at this point.

Chest XRAY, no problem.  TB test though?  Are you kidding me???  Yeah, those damn needles again.  In addition to the emotional roller coaster Makena was already on, the fact that she had to get poked AGAIN (33+ vials of blood drawn in only 2 weeks at this point)… full panic attack from the time she realized where we were going and what she was going to do.  Makena was shaking uncontrollably and couldn’t control her breathing.

Heart. Broken…. again.

Within an hour of being home last Friday afternoon, we already started receiving the calls to schedule the appointments with the other departments.  EVERYTHING is moving at lightening speed and we aren’t sure if we are grateful for that or concerned.  Of course we appreciate how proactive and on top of everything the doctors and staff have been, but at the same time they are also leaving very very little time to think!  One question after another, information being spouted off in every direction.

So.

So.

So…

Much.

Any parent can imagine the barrage of emotions that we are now faced with.  Trying to think about how our lives’ will change and to what capacity, how treatment will go, did we choose the right therapy for her, what if it doesn’t work, how soon will we know if its helping, what if her body rejects it, what if she has an allergic reaction, how long she will be in this initial onset (we were told it could last up to six months), could she possibly go into complete remission, what will remission look like, how could we possibly calm her anxiety surrounding needles (something she will be seeing a lot of from now on), how many days of school will be missed, how traumatized will she be as a result of dealing with this at such a young age, will she be able to continue with volleyball, to what extent will other organs be effected, what will her already high anxiety look like on the way to treatment and during treatment, what can we expect after each infusion treatment, is there anything we could help to reduce the amount of pain she is in on a day to say basis, are there any substantial short OR long term side effects of the medication she will need to be on, what will our new “normal” look like, what does her prognosis look like…

So many questions.  So many feelings.  So many thoughts.

It’s now been three days since receiving her diagnoses.  It was a very very long and hard weekend for us all.  I feel like I had been pretty strong and practical Friday and Saturday, but yesterday?  Yup, I broke down.  Hard.  I cried and cried and cried.  Cried by myself, cried to my husband, cried to my sister, cried to my mother in law.  So many tears.  So much fear.  So much “unknown” to now worry about.

We now move forward though as this family will always do.  Processing, research, proactivity, appointment scheduling, crossing our t’s and dotting our i’s, phone calls with our new pediatric care manager for Rady’s and Kaiser, and then also trying our hardest to deal with this crap hand we have been dealt.

One great thing about all of this… Makena is so much like her father and I.  In just a few short weeks, I have really seen her heart & soul shine through.  We’ve seen so many glimpses of ourselves in how she is dealing with all of this.  Makena continues to amaze and surprise us.  Not once did she ever say “I CAN’T go to school” or “I CAN’T go to practice”  or “I CAN’T walk” or “I CAN’T make it upstairs” or any kind of “I CAN’T do XYZ”.  Not once.  She’s powered through, self regulated her most of her activity, and is processing in her own little way.  Her personality and will help make us confident that she will have the ability to deal with whatever comes her way!!!

Still’s Disease?  Watch out.  The Thomas family is coming after you!  You may be a powerful disease, but we pack a pretty big punch also and have unrelenting will 🙂

So next steps for us this week:

• TB reading today (monday)
• hematology, oncology, and infectious disease appointments (tuesday)
• cardiology & ecocardiogram (thursday)
• possibly starting treatment (friday)

I’m aware I’ve asked for selfish prayers and positive thoughts to be sent our way regarding my medical conditions.  This is NOTHING like I’m asking now though friends & family.  Please please please send every single ray of light to our sweet & amazing little girl!!!!  NO child should ever have to deal with such a serious chronic debilitating issue like this… ever!