Tuesday, June 5, 2012

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 7th 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…


  1. /sigh I've been thinking about you all lots. I've wondered how Parker was doing with the trial. I was hoping that it would be going famously by now. I pray and pray that this is the placebo and the real drug is AMAZING!!!!! I'm trying to keep up with you guys but I am struggling with keeping up with anything right now. Just know that I think about your family often.

    Some of the lab levels that you mentioned- I hadn't realized that some were a "JA thing". Em has the hyogammaglobulinaemia & of course the anemia. I hadn't realized that this was common, though I assumed the anemia may be. Interesting. I shall read the article now. Thank you! Great blog. Very direct, very hard- hitting.

    1. Thanks Danielle, been thinking of you all too. I really appreciate you stopping by the blog and checking in. We need to chat soon. You definitely need to read the article. They even refer to sJA being more like JDM than JIA. Very interesting...

  2. This systemic disease is so hard to handle and can cause so many problems. I think that even if people do know the physical issues associated with it - which they don't - they can't even fathom the emotional toll the disease can take on everyone involved that child's life. You guys are so strong. Hang in there. I can't say for sure that everything will change and be better, but things will get better.

    1. People like you, dealing with this disease, are my heros. Parker says he is doing all this to help them find a cure. It will come one day...hugs