Wednesday, June 20, 2012

A Week in the Hospital and Parker is Finally Feeling a Bit Better

Well, we just might be over the is definitely better than yesterday.  Yesterday, Parker was in so much pain that he couldn't even stand without intense pain.  And he was having sweats so bad he soked his sheet.  Unfortunately, yesterday was also that final day of toradol; so he ended up getting additional morphine.  My heart was breaking for my sweet kiddo...

Systemic on-set juvenile arthritis is one mean vicious disease.  Parker's joints (feet, ankles, wrists, hands, knees, shoulders, neck, ribs, hips)  were sizzling hot yesterday and the vasculitis in his feet and ankles was pretty horrible.  The pictures below don't capture the thickness of the vessel swelling with justice and the red and purple shades of his skin were pretty intense, but they will give you a bit of a sense of what this horrible disease "looks" like.


His pericardial effusion is looking better and he does not have h-pylori.  All good news.  More labs were drawn today.  He is staying until tomorrow with hopes of being discharged.

We are thankful for the friends and family who have checked in with us and who are thinking of our son and saying prayers.  We have been taking it one day at a time.  Tomorrow will be his third shot of rilonacept so we are hoping for a bit more progress with each new sunrise.

Sunday, June 17, 2012

Kids with JA are My Heroes!

Parker says, "Happy Father's Day to Dad and Grandpa Nick!"

Parker is still at Shands Hospital.  We are happy to say that he is improving.  Pain is still pretty intense but the meds are helping to take the edge off.  He got up and walked a bit today so that is great.  His doc came in this morning and although he is still symptomatic she was pleased that many of his blood levels are improving.  There are improvements with his ferritin, platelets, white and red blood cells, CRP, fibrinogen, and his sed rate is good.  She said that with the study she is seeing improvement in the blood first, so this is fabulous news. 

Swollen hands with red tips, greyness between joints,
paleness on hand

Red heels

Parker's abdomen and kidney ultrasound was good.  She does want to keep him in the hospital though because of the pain, pericardial effusion, stiffness, swelling and she is still waiting on some labs and she ran his immunoglobulins today.  He seems to breathing easier today.

We feel so fortunate to have such an amazingly thorough group of pediatric rhuematologists helping Parker.  In the last four days, he has actually seen all 3 of the docs and Michael and I know that their 3 brilliant minds always have Parker's best interest in their hearts because they not only treat him medically but with loving care.  We know that Parker has been difficult to treat and we appreciate their forwardness and honesty and their ability to explain things so that we can understand the whys and next steps and choices we have to make.  So, although it is never fun being in the hospital, we know that he is in excellent hands.

We are glad that Parker has moved past the "placebo phase" of the RAPPORT study and he says he has no regrets in doing it because he knows that he is helping find a cure.  And although Michael and I are incredibly sad to see our son in pain and that this darn disease has once again gone after his heart, we too feel that this next phase of treatment brings much hope. 

Last night was methotrexate night (all those families out there who have "shot night"...I feel for you all). We all thought Parker would be getting it as an infusion in his port....but because of the study and not wanting to compromise anything, it was ordered sub-Q (shot form).  Parker had a melt mind you, he had his steroids increased again and he has been scared and then he was told he was getting the shot form...oh my gosh....we both were in tears.  He is fine with shots...but this method of giving him the methotrexate really makes him very nauseous and he was afraid he would puke.  Remember his stomach has also been really bad and they have been treating that along with the heart since they think his gastritis is flaring big time.  Methotrexate (for those of you who don't know) is a chemo drug and they not only were giving him a shot but an increased dose.  He finally agreed after much coaxing to do it.  They gave him IV zofran first to help and we did it right before bed so he could sleep through the worst part and I lay with him all night. 

It's crazy how I can hold it all together through so much and then something like this shot sorrows me to the core,especially since the doc is talking about continuing the shot.  For the last several months he has been doing the pill form of this because there is a methotrexate shortage of the shot.  And Parker really prefers the pills.  But we also think that the pills is what is irritating his stomach so bad.  I really hate systemic juvenile arthritis...I hate that we must treat it and so many of the drugs are so harsh that you have to give drugs for the drug side effects.  And yet, to not "treat" could have very grave consequences.  And , somehow these little kiddos endure it all....they truly are my heroes forever and ever.

Parker's brother, Logan, came to see him yesterday with Michael (Dad) and Great Aunt Sandy and Uncle John.  That was such a treat for Parker.  Especially when Dad showed up with a tattoo on his arm, just for Parker.  It says "Kids get arthritis too.  Parker's Purple Playas."  All the nurses and docs loved it too!  Pretty cool, huh!

We thank everyone who has checked in with us and who are continuing to say prayers.  Parker sends love out to all of you!

Friday, June 15, 2012

Parker is in the Hospital...

Just a quick note to update...Parker was admitted into Shands Hospital yesterday.  We visited cardiology yesterday for an EKG and Echo-cardiogram.  He has been having that feeling again and chest has felt pressure filled and he has had difficulty breathing.  Last week his nebulizer treatments seemed to help but not so much this week.  After the echo we went and had lunch and you know the saying "no news is good news", so after hanging in Gainesville for a bit we headed home.  About half way home we got the call to come back.

Parker has mild pericardial effusion again.  The good news is that they feel that it will correct itself and we won't have to drain it.  So, for now they are trying to make him confortable and we might increase his oral steroids because of this.  But we will see.  He is already on a hefty dose.  He is also having bad stomach pain and they are also treating that.

Because he is on the RAPPORT study there are some strick rules around medication and we don't want to jepordize the study but of course if he gets any worse we will not hesitate to do what we need to do to keep him safe.  He is looking better today, which is good.  He definitely has had 2 shots of real drug (rilonocept) because at week 4 kids are guarenteed real drug.  However we are pretty certain he was getting placebo the first 4 weeks.  He is just into week 5.

I will keep you posted as we know more. Thanks all for continuing your prayers and for keeping us in your thoughts.  It means a lot.  We are hoping this is a short stay.

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:
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…