We Spent WORLD Arthritis Day at the Hospital

We had our visit today with the doc and we are still here at Shands Hospital since it’s IVIG infusion day/night.  The good news is it’s just an overnight stay.  Parker’s doc is amazing and we are so fortunate to have her helping our kiddo.  She talked with the principal investigator physician for the RAPPORT study and bought us some time.  She got an additional 4 weeks’ worth of med.  I thought I was losing my mind a bit because I could have sworn we had until the end of December with drug.  I really thought that was what we signed up for…come to find out…YES, that is what we signed up for but because they are running out of money to run the study, we were going to be cut after the trial run was complete.  It really seems unfair on so many levels…but we are thankful for the additional time.  His doc is so very upset by this…she felt that he should get it because so many biologics have failed him but she can only do what she can do and we are frustrated right alongside of her.

In the meantime, an appeal was filed.  We are also going to appeal as parents too.  And his doc is going to call the drug company to see what can be done.  She is also contacting one of her connections at the National Institute of Health (NIH) to see if Parker could be seen up there.  We are feeling a bit desperate.  We do not want to see this disease attack his organs again.

We also talked about trying to decrease his steroids to see what happens.  We have a sense that the steroids might be holding some of the symptoms back.  So we will test that while he is still on the rilonacept.

Also, since Parker is going to need IVIG on an ongoing basis, his doc is going to try and set up home infusion…she said it kind-of works like an insulin pump.  He would get it once a week and it would run for 1.5 hours and infuses in the stomach.  There are a few advantages to this:  we would get to be home; and he would get more continuous levels of IVIG.  For those of you who are not aware, IVIG is an infusion of around 2,000 donors’ blood plasma.  This helps his immune system that is definitely not working correctly.  So we will see about that too.

If Parker cannot continue on the rilonacept and cannot get into NIH, then the only choice left is to go “backwards” and put him on what he has been on before and probably multiple biologics.   As for the symptoms that have returned…we will continue to monitor his temp, Raynaud’s, systemic rashes, and his vasculitis.  She said he has “hot spots” with his vasculitis.  And if by some miracle, he gets to continue on rilonacept long term he may still need to alternate it with another biologic because this darn disease seems to be outsmarting the drugs yet AGAIN!  Yes, it is crazy and we so want to see Parker comforted and feeling better.

Parker was super scared today and totally freaked when they told him he was going to have to share a room tonight with another patient (sweet kiddo by the way).  And even through all his fear, Parker was also super sweet…he asked the doc to come back so he could give her a hug.  He gave her a long hug and he cried.  He loves her so much and he knows that she is really trying hard to help him.  So please continue your prayers and encouragement.  We appreciate the support more than we could ever ever express.

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

Well, we just might be over the hump...today 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.

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…

Parker Shares What JA is Like

March 16, 2011

So I am writing today to try let people understand what arthritis is like. Now just let me tell you it is not fun; it is painful.

For example, look at this picture...

Pain-zilla!





To see the vessels and the pain in my back angle the screen.



Also for optimal view, angle the screen.

Now for those doctors that say, it was all in my head; look at this. Bet you don’t think it’s in my head now.

I also have to go through a lot of tests along with the pain.

For example, look at this.

Not fun.

And for all my friends who say I’m lucky because I don’t have to do all the homework and school work. Well their wrong. I have to do all the work they do but while I’m in pain.

 

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.