Trying Not to Let Fear Take Hold...Trusting All Will Be OK

Fear can seem to momentarily paralyze us…Michael and I feel like we have been hit hard in our gut and the wind has been knocked out of us.  The last few weeks have been tough to digest.  Parker has seen progress on the RAPPORT study and for that we are incredibly thankful.  However, he is not doing as well as the other children on the study and we have seen the return of some classic systemic JA symptoms.  But today, it was made official… we cannot get the drug approved for continued use after the study and next Friday is his LAST day of the study. 

I broke the news to Parker and he totally withdrew and told me to leave him alone.  So I honored his request…as hard as it was, because this was not his typical reaction to bad news…  About 40 minutes later, one of his hospital homebound teleclasses started and when the teacher asked him how he was doing (like she does with each student during role call), he could barely squeak out “not too good”.  She said he could just listen today.  He said “ok”, muted his mic, and burst into tears.  All I could do was hug him…

Even before he voiced his thoughts, I knew what he was thinking.  He is scared of regressing even more.  He is scared he will have to go back to multiple biologics.  He is scared it will attack his lungs or heart again and he will be hospitalized.  He is scared he will be back on 2 infusions a day of kineret.  The day that we had his Arthritis Foundation Walk (May 5) was quite the celebration because that was the last night of his kineret infusion.  That night we made about a 10 minute video in case other families wanted to see what our twice a day routine entailed.  

He has been through so much such then….

During our last visit to the ped rheumy, we also had confirmation that he does have hypogammaglobulinemia and it is NOT due to his use of biologics but his messed up immune system and we were told he would probably need IVIG infusions for the rest of his life.  She also shared that she really feels he also has psoriatic arthritis in conjunction with is systemic on-set JA and all the other overlapping stuff.  And although he has been back on IVIG, his numbers are not increasing like she would like them to.   It is incredibly frustrating and concerning.

We are processing a lot right now.  Michael is coming with me on his next overnight infusion of IVIG this week, so we can talk about next steps and try to make quick decisions.  We are trusting all will be ok and trying not to let fear take hold of us.

Living While …..Waiting….Always Waiting

This school year has been one of much optimism, but our optimism has been shaped into “realism”.  We have pushed hard to keep Parker IN his middle school because with every new drug (and there have been many) we held on to hope that “any day now, things will get better”… and in moments of time, some things get better, as if to tease us of the possibilities.  Parker becomes so hopeful, rejuvenated, and motivated with every glimmer of hope for better days.  And let’s be totally honest, when it then doesn’t work, the entire family is deflated once again.  It is very difficult to explain this phenomena that keeps happening over and over. 

When we started this journey over 4 years ago, we just wanted an “answer” because we really felt that an answer would lead us to solutions.  And we felt like we would be better able to not only support Parker’s medical needs but also his educational needs.  Well, for 2.5 years we have known what Parker “has”…We STILL don’t know how to best support his educational needs and I feel like so much rides on the RAPPORT study that he is current on.

This disease is so complex.  It’s difficult to comprehend what could happen, while at the same time pushing to maintain optimism, motivation, and faith that it will get better.  We don’t make “promises” anymore around drugs…we don’t make plans without a back-up plan and we write in pencil on our calendar…we don’t expect huge gains….BUT we do continue to hope the cure will come; we do continue to make plans and hope we can follow through and that others will understand when we can’t; and we do push for tiny gains and celebrate successes because the small bits of progress are huge for Parker!

Parker has a huge powerful energy about him.  We really believe that his strength is unimaginable for some to even begin to comprehend.  And he is a great illusionist… he is engaging and smart and puts forth so much love …and he fools people in thinking he is ok “when he looks so good”.  And we are extremely proud of him because he does rise; he does push on; and yet he has had to learn to accept that his body does not cooperate with what he would like it to do…at least not yet…

I say all this because we want people to learn and to really understand…but I am beginning to believe that it is impossible to totally understand systemic on-set juvenile arthritis…shoot…doctors are still trying to “understand” it.  Here is what we do understand…without proper treatment, systemic JA:

·         causes incredible relentless fatigue

·         produces intense pain

·         causes oligo or polyarticular arthritis:  joint swelling is in every joint for our son with heat and redness (Parker has poly)

·         induces fevers

·         brings on rashes

·         causes pleurisy (swelling of the lung lining)

·         irritates the lining of the stomach and causes gastritis (swelling of the stomach lining)

·         causes pericardial effusion (swelling of the heart lining)

·         enlarges the lymph nodes (we learned there are even lymph nodes near the stomach)

·         causes erosion and joint narrowing

·         results in osteopenia

·         causes anemia

·         causes immune issues

·         stunts growth

·         causes thrombocytosis (increased blood platelets)

·         causes hyogammaglobulinaemia (decrease amount of immunoglobulins in the blood and can cause higher susceptibility to getting infections)

·         causes fibrinogen levels to be off (this can affect coagulation of blood)

How do we know this?  Because we have to know this… Parker has experienced all of this and more…because he also has overlap syndrome of:

·         vasculitis

·         raynaud’s (cold hands and feet that turn purple, red, and white)

·         myositis (weak muscles)

·         psoriatic features

We also know that sJA can cause issues with pretty much any organ in the entire body and many have issues with an enlarged spleen (our son does not have this…wahoo).  And in two thirds of the deaths that happen in children with JIA, they are with children who have systemic JIA (source:  http://rheumatology.oxfordjournals.org/content/44/11/1350.full.pdf+html)

Parker is living…living and fighting this damn disease that is trying to over-take him, but he is fighting hard to stop it.  Parker’s voice was heard when he said he wanted to do the RAPPORT study.  At first, my husband and I were not going to do it because we had some issues with our insurance, but after Parker’s last hospital stay, he is the one who re-initiated the discussion around the study.  At this point, Parker was on 3 biologics (actemra, Enbrel, with a dose of remicade), along with IVIG infusions.  Not to mention the multitude of other drugs to control this disease (pain meds, anti-inflammatory meds, meds for meds, chemo, steroids, blah blah blah).  And the steroids are possibly his saving grace right now…because the “study drug” is not working (or he is on placebo).  One of our main goals has been to get him off steroids…he has been on them for 2.5 years…way longer than we ever expected. 

So with regards to school, Parker has been in hospital homebound because he barely made it to school first semester.  Oh, and first semester he was only on a part time school status with 2 classes on-line.  It took him all school year to complete the first semester of both of those on-line classes and he only has one test and his exam left.  As of today, he completed all of his hospital homebound exams and he is officially done with second semester of 7^th grade.  All year, he has been playing major catch up with school.  We feel like next school year totally rides on this study and if it works.  Needless to say, we have re-initiated hospital homebound for next year just in case.  Like I said, we are becoming “realists”...yes, me, who fought to keep him in school is realizing…but we must concentrate on helping him feel better because we don’t even want to think of what “could happen”…

So we are living…coping….comforting Parker…and constantly praying…and here we are again, waiting…waiting for 5 weeks to pass since stopping biologics and being ensured he will get the study drug…in two more days we know for certain Parker will get the real trial drug.  We are unsure if he has been getting placebo, or as Parker says “if this is the real drug, it sucks”.   Need I say more…

"Arthur Itis" Leads Us Down a New Path...

Here we are, one month later, and we are taking several twists and turns along our journey with “Arthur Itis”.  Saturday, our path took us to Orlando to our first JA Family Connect event.  We had such a wonderful time with our newfound JA Family and met wonderful people who all shared our acquaintance with Mr. Arthur Itis. 

Logan had so much fun playing with all the itty bitty kids (he is definitely his mom’s kid…loves those toddlers and preschoolers)!  He even got to meet some siblings going through the same experience as him.  Most of them were girls, but he was just fine with that :O).



Parker Gets His Certificate



Parker was such a trooper.  He played for a bit and even won a contest and was thrilled when he won a Colby video cam (much like the Flip Cam).  He was also so touched when he received a certificate for his hard work with the Jingle Bell Run/Walk and for being the number one fund raiser for Tampa and number four in the state.  To see his pride when he received that was a priceless moment.

Parker said that this was one of the best days of his life.  He felt like he could be himself and that it didn’t matter to the other kids that he had to stop playing and sit.  One other very special little girl, Emily, sat and talked and talked to him.  They were totally engaged for quite some time as they shared their experiences around their symptoms, many medical tests, medications, treatments, and feelings.  He hasn’t stopped talking about her since we got home!  Actually, he has been talking about all the kids, but Emily definitely had a huge impact on him.  At bedtime, he included each kid he met in his prayers for healing tonight.



Swallow Test



The timing of the family picnic was perfection for our family because on Friday our journey took a very sharp and scary turn for the worse.  Parker spent the day at Shand’s Hospital doing some tests and getting his infusion treatment and meds.  His doctor spent a considerable amount of time with us, was very compassionate and thoughtful.  But nothing could have prepared me for the news she shared with Parker and I.  I had thought we had already passed this road, but she voiced our biggest fear…Parker has systemic JA, also known as Still’s Disease, http://www.medicinenet.com/stills_disease/article.htm.  And as if that wasn’t enough to hear, but she is concerned he could also have a very rare autoimmune illness called juvenile dermatomyositis (JDM), http://www.curejm.com/.  That was quite a moment…I literally had to catch my breath as I fought back my tears for my son’s sake.  She then went on to explain that she is very concerned about Parker and that we have to stop the progression of the JA.  He is failing all the TNF biologic drugs and so we are to only wait one week and if he is the same or worse she is going to change his infusion treatment.  In the meantime, she is setting up an MRI to look at how his muscles and myelin are behaving.  And this may confirm if he also has JDM.  So now we wait for the insurance to approve.  We are getting very good at “waiting”.  So, when we were at the picnic, I was asked to introduce our family and the kind of JA that Parker has…and for a moment I didn’t know if I could say the words aloud:  “He has systemic JA.”  It now feels all too real.

After a long day of tests and infusions we journey home.

Life can change in a moment… Our hugs and prayers go out to one very dear little friend with systemic JA who was happily riding her scooter and giggling with all the little girls yesterday; and today she was admitted into the hospital.  Love your children dearly and tell them you love them.

Where to Start and Where are We Going?

As I sit to write this very first blog, I wonder how to start putting our "journey" into text.  And I wonder if putting our experience in print will somehow make this experience even more real...too real.  It's been 2 long years, yet it seems like yesterday I watched Parker play and excel at every sport he tried.  He would literally run circles around his older brother...we use to tell him to slow down...but today,  I am pushing him in a wheelchair just so that he is not left out of fun and can participate in his community. 

Two years ago, Parker got very sick and spiked high fevers that lasted months and months.  He had weird rashes and reynaud's.  We knew he wasn't having an allergic reaction because he has also had severe allergies and asthma since he was 18 months old.  These rashes were different than hives and they would disappear as quickly as they came and would come and go.  He also complained constantly of pain in his neck, back, hips, and legs in those first few months.  His lymph glands were very swollen.  His primary doctor began running tests and referring us to his regular allergist, an immunologist/allergist, and a neurologist.  He had tons of blood labs, CT scans, MRI's, bone density scans, x-rays, brain scans, GI and bowel scans...and at first the only thing that would come up as positive was sogren's syndrome and a few other levels were off.  The primary doc suspected juvenile arthritis but the expert immunologist/allergist said no because Parker's intelligence was so incredibly high (WHAT?!)...and he diagnosed him with fibromyalgia and we started PT and continued seeing the neurologist.

The primary doctor continued to monitor him and express her concern and at one point (called us on Christmas Eve) to tell us that he might present with lymphoma and that it often doesn't show up in blood until years after it progresses.  So she has continued to measure his lymph nodes throughout these last 2 years and every time she takes out that measuring tape my heart jumps a beat.

The neurologist couldn't seem to figure out what was going on...then Parker started seeing spots and headaches were getting worse and the neurologist talked privately to my husband and I to tell us that this was all psychological and that I (mom) was likely making it worse for him.  This was a very difficult time as we realized that he was sharing these thoughts with Parker's primary and she also talked to me about psychological help for Parker and myself.  Yet, as my husband and I talked through what was happening we realized he was not catching something...Parker's teachers and school nurse even were sharing physical symptoms that they were very concerned about...paleness, extreme fatigue, difficulty walking, falling, fevers, rashes...and yet he had "good moments/days".  I think that this confused matters. Then we realized that the med that the neurologist put him on had side effects of head aches and "seeing spots"...duh?!

About a year and a half into tests and multiple specialists, we switched to the partner of the immunologist/allergist because he also had a office near our home and he specialized in immunology/rheumatology.  After 2 visits, Parker got the firm diagnosis of Juvenile Arthritis.  So we came full circle.  I called my husband immediately and then called the primary to tell her she was right all along.  It was quite a moment for us...it was not psychological and we thought...oh, this isn't that bad...it's arthritis...we can deal with that...well, we had no idea where our journey was going to take us!

We are now seeing an amazing doctor at Shands Hospital (about 2.5 hours from home), but totally worth the drive.  We have been with her for about 4 months now.  It's been a very intense four months as we have tried to decrease Parker's steroids (on them 8 months now) and add medications that are suppose to slow the progression of rheumatoid arthritis (we are told it's not curable...but kids can go into remission).  For the past 3.5 months we have been giving Parker 2 shots each Friday night.  A biologic drug (enbrel) and a chemo shot (methotrexate).  No luck!  So a few weeks ago Parker went to the hospital for an infusion of sulumedral (1,000 mg)...to get him over the hump.  It worked for 3 marvelous days.  Parker was his old self for a moment in time.  He then got worse...much worse...pain and swelling has been horrible and steroids were once again increased. Parker has pain in every joint...even the little ones in the ribs and jaw and toes... Then we got the call!  Parker's JA is progressing.  The enbrel is not strong enough.  This week we start remicade infusions.  My husband and I had to make the hard decision to put him on this as there are little options...see this medication can increase chances of lymphoma 20%...but if we don't treat the JA he could have permanent joint damage and lots of pain.  So, we will be going to Shands this week for his first infusion and it could take 3.5-6 hours since they need to monitor him closely and check vitals every 15 min.  So I wonder where this new medication will take us...

I wish that more people knew more about how much Juvenile Arthritis impacts a child.  It's not just for "old people"...It doesn't just affect their joints...it affect their internal organs, their developing little social selves, school, their former sense-of-self, and the pain...oh the pain...how do they do it?!  We are very very proud of Parker.  And we can't even begin to talk about how amazing Logan is with supporting his little brother!  The night before our first visit to Shand's Hospital he sat at Parker's bedside singing Bob Marley..."Don't worry, about a thing, every little thing is gonna be alright..."    We're gonna be alright.