Here We Go Again...by Parker

So these last couples of weeks haven’t exactly been the greatest. My arthritis has been at war with my body. I have started getting flares again and that’s pretty weird considering how I was feeling before this flare. Things were really good, I was making it to school almost every day and I wasn’t even using my wheelchair.

But then, all of the sudden, it was like a bomb got set off in my body and that bomb hurt. First it was my lower back, shoulders, and neck that started hurting; so I switched to a rolling backpack. The next thing I knew was my ankles and feet were being attacked. If you look at the picture to the left and look at the red circle, you would probably guess that bump is my ankle. Guess what, it’s not.  That is actually the bone in the arch of my foot trying to push out. This became a problem because I’ve been wearing braces since the surgery.  When I walk, that bone hits the hard plastic and very badly hurts.

In addition to all this, I started having daily fevers again. Now I know that fevers are a symptom of systemic JA but I take a drug called Rilonacept and that drug is supposed to do 2 major things for me. Those 2 things are to keep my pain and swelling at bay and to suppress fevers. So when all this stuff started happening, we were pretty concerned.   

Then, another pretty weird thing happened. There are 2 joints on my body that don’t swell too bad, and those are my elbows and knees. And guess what, my knee got huge this week. Just look at the picture to the right. I mean really, if I didn’t know any better I’d think that was a thigh. The next day I woke up and my knee was still pretty huge.  We called my doctor and she said to go to my orthopedic surgeon to see if he would drain it. So we went to go see my surgeon and he wanted to take some x-rays of the knee before the appointment.  Since  we were there, he wanted to also get some x-rays of my feet and ankles. Well by the time we got up to his office and ready to see him, my knee had gone back down quite a bit. But he still wanted to look at my surgical site x-rays, especially my feet x-rays. And this is where everything went down hill.

If you look at the x-ray to the left, in the red circle you can see what my foot looks like with the cadaver bone fusing with my bone. And it looks pretty good. But if you look at the x-ray to the right, in the red circle you can see where the cadaver bone was located. And well, it’s not there, it’s gone. It was basically rejected by my body and was completely absorbed.

Now my bone has a gap where the surgeon had severed it during the surgery.  And all this time it has felt like the bone is trying to escape my body by pushing out the side of my foot because it is actually pushing out and my arch is completely collapsed again.  As you can imagine, we decided that it’s time to switch to a new drug called Ilaris, as soon as insurance approves it.

So you may be asking, what does this mean? Well, it basically means that the surgery didn’t work in my left foot. And on November 18^th 2015, I go back into surgery but this time to permanently fuse my foot. This was a lot for me to take in and I’m doing the best I can to stay positive. So, I want all of you to remember that you’re never fully dressed without a smile.

NIH Status and Other Updates

Well, I am just going to be straight forward…there is no NIH (National Institute for Health) in Parker’s near future.  Although his doctor made every effort to get him in and even though she talked to the lead doc there, it seems that because of the 17 week long government shutdown, they are now very backlogged.  They are only taking kids that fit criteria for one of their studies.  She did ask the doc to ask other docs if anyone would be interest in a “very unique” kiddo; so he is posting on their JA doc groups. 

Parker was quite upset with this news; we are, however, please that Parker’s health has had some improvements.  His lungs have improved and pulmonology is happy that capacity is increasing.  And he has not had any other internal organ involvement in quite some time.  For this, we are incredibly thankful.  He is still in incredible pain; having skin issues off and on (minor vasculitis, rashes); fevers come and go but are not as high; and he has some joint swelling, but ankles are not as constantly huge as they use to be.  We do feel that the Rilonacept has slowed the progression of this disease.  And we are pleased with this drug in combination with prograf and his hizentra weekly infusions; it seems to be a pretty decent combo for him.  Of course, we wish it was even better, but we are pretty pleased with the progress because it has kept him out of the hospital and he has had less illness.  This is a huge blessing.

After a mini battle with our health insurance, we got Parker’s foot orthotics approved.  His left ankle has turned down towards the ground quite a bit, despite PT and his current orthotics.  So he is upgrading.  At first, the insurance denied the new orthotics saying that only diabetes patients qualify…but we are so thankful that we were advised years ago to ask for a “case manager nurse” with his insurance.  Plus, I am pretty darn persistent.  So after calling her and pleading, she advocated on his behalf and they were approved.  I advise anyone raising a child with chronic illness to ask if your child can have a case manager nurse.  It really helps to have one individual to talk to at the insurance company who knows Parker’s case.

Parker is now seeing a local pain management doctor (this is doctor number 13…jeeze louise).  He is totally on the same page as us and wants to work on getting Parker off the narcotics and use other routes to help with pain.  He did two rounds of tests with him and it looks like he is a candidate for nerve ablation.  We are seriously considering this for his back.  For the first time, in about 5 years, Parker had zero pain in the area the tests were done and it held for about 4.5 hours.  He told this doctor through tears, "thank you for the best Christmas present ever".  Of course, the doc and I got all teary with him.  We are also going to discuss his ankles further because his rheumy is wondering if botox injections in his ankles would help.  Through PT, he has had much improvement but his range of motion is still quite limited due to the destruction that has already occurred in his ankles and she feels botox might help.

 

As for a quick “Logan update”, his JA (spondy) has progressed some but is still somewhat manageable.  We have not had to add biologics.  He is on Celebrex and after discussion with the rheumy, he has decided to try adding plaquinel.  First, we need to get him in to the eye doc to get a baseline on him and also to look at his eyes because he gets some really bad reddening in them (especially just prior to, and sometimes after, migraines).  We want to get the ophthalmologist’s blessing before starting this new med.  Right now, this kid walks around with salanapas patches on his joints…it is just not cutting it.  The doc feels that he too has some mild vascular stuff going on…and we joked that it seems to just be one of those weird family issues.

So in closing, we are so very proud of our two boys.  Logan continues to push through dual enrollment and loves his college classes.  And Parker is juggling massive amounts of medical appointments…Yesterday, he went to PT even though he felt miserable (a little concerned he might be sick)… On the way home from PT, I think Parker summed it up best when he said “I am who I am, and I am really strong!” 

 

LOVE Pushes HOPE Higher and Higher



Waiting for Doc



For the last two and a half years we have made our 2.5 hour drive to the hospital every two weeks for treatment for Parker.  We are moving into 2013 with a much needed break from this schedule along with much hope for continued improvement.  Most of Parker’s treatment is at home now and we don’t have to go back to the hospital as frequent…we now go every 3 months.   In reflecting on this last year, a tremendous amount has happened.  With every hurtle we have continued with strength to take the leap of faith needed to get us to the place we need to be for our family.  Of course, Parker has been incredibly strong through all of this, but Logan has been just as strong, if not stronger.  As adults, Michael and I have tried to maintain day-to-day needs…but so has Logan. 



Logan



Logan's eyes get like this with the really bad migraines.



Logan has his own health issues with his relentless migraines this year.  This is a condition he has had since he was 5 years old and we have a very strong history of migraines in our family, but as Logan has entered his mid-teen years they have been more difficult to manage.   These migraines have not only impacted his health, but also his social life, education and stamina.  He continues to overcome and it is remarkable that he pushes forward while also supporting his brother and trying to create a balance in his life.  We are proud of the young man that he has become and his future has much promise and I am looking forward to the days to come.



Christmas 2012



We also want to celebrate Parker’s progress…this year pushed our hope higher and higher.  This year Parker has been on multiple biologic drugs to combat against juvenile arthritis.  I know that these drug names mean little to the general public (except maybe “Enbrel”) but bear with me as I process this…ready…he has been on actemra, then actemra with Enbrel and soon after he also began IVIG infusions (IVIG is a blood plasma to treat hypogammaglubulinemia and is not a biologic).  Then he took actemra with Enbrel and a round of remicaide, then he needed to stop all these (including IVIG) in order to proceed with the “study drug’s wash out process”.  During the wash-out of these previous biologics we had to up his steroids and begin the biologic kineret (twice a day) because he could take kineret up to 4 days prior to starting the RAPPORT study looking at the effects of the biologic rilanocept on systemic on-set juvenile arthritis.

Soon after starting the study, Parker’s health started to decline…yes, we are pretty darn certain he got the placebo and not the rilonacept.  Parker landed in the hospital in tremendous pain, swelling and pericardial effusion.  But looking back on all this, Parker has no regrets.  He knows he is playing a small role in impacting the future for children with JA.  Our family went into this last year knowing it was going to be a tough one.  We decided to put school to the side and be ok with hospital homebound while we worked on supporting Parker’s health.  We now feel like we are on the flip side…  What we have learned is that with each step forward…we are moving forward at a slow and steady pace…and although the journey may feel endless at times, we have felt tremendous forward propelling LOVE.



Surprise care package from Sloans via the infusion room nurses!



Thank you Ky for my special Parker Snowman!



This love has come from very unexpected places through very caring actions.  We know people lead busy lives with their own struggles and we are thankful for each and every person who has paused to show us love through your calls, emails, cards, text messages, and Facebook posts.  We are also incredibly appreciative for the actions people have taken…hugs, help with picking up Logan, meal prep/restaurant gift cards, doing laundry/cleaning, dropping by to say hi, leaving notes on the windshield, donations to the AF walk and High-5-Club, help to maintain work, comments on our blog, hospital visits, surprise packages, joining our walk team, helping fund raise, willingness to drive across the state to help us access an “out of stock” med, listening, encouraging, and validating our feelings.

We also want to thank the medical staff supporting Parker.  We know we are still on this journey…  Thank you for your passion in this field, your work is unbelievably difficult and we need more people like you in this world.  And I am not only referring to the doctors and nurses, but also all of the people behind the scenes who process the relentless amount of paperwork and insurance “overrides” because the insurance companies are not always open and willing to charter in untraveled territory.  Thank God that Parker was the first child in the state of Florida to get approval for the study drug once the study ended.  Multiple biologics failed him…this is the first drug that has had enough of an impact on him that we are witnessing some of the “old Parker” return.  When a devastating chronic illness impacts a family, you begin to try to create “new norms” and you work hard to find happiness in the little moments and pretend a bit that they are as wonderful, if not better than the happiness you experienced prior to the “disease” entering your life.  And in some ways they are…because they are felt with an increased intensity…emotional and physical pain does that to a person… But oh the JOY and renewed hope we feel with our “old Parker’s” return.  

Turning in wheelchair wheels for
skateboard wheels.

Logan and Parker are experiencing their brotherly passions again:  quirky fun, chitter chatter, planning events again, gaming together, and skateboarding…yes, skateboarding!  Logan spent his own money this Christmas to refurbish Parker’s board to make it safer for him.  He got him better wheels with a better center of gravity, bushings, bearing spacer, and stronger grip tape. 

Love is totally rolling this pair forward… and Michael and I love witnessing this…Here’s to continued happiness in the New Year.

Look Doc, I am working my stiff ankles!

Our Most Valuable “Playa”, Parker: Thankfulness Sums It Up!



OUR STRONG KIDDO!
Thanks Ashlynn for
his very own Boggy M&M!



A quick update to get all of you up to speed on the Purple Playas’ most valuable “playa”:  Parker!  We are so incredibly fortunate to have a team of doctors, nurses, and medical office staff that powers on right along-side of us!  Through our appeals and persistence, Parker’s study drug, rilonacept, was approved through April.  The office manager said she thinks he is the first kid in Florida to get this approved.  Maybe now other insurance companies will jump on board and get this for the many others who also need it!  Truly the miracle we were hoping for!  We were so concerned about going backwards but now we can continue to move forward!

Another great piece of news is that this Friday will be his last overnight stay at the hospital for his IVIG infusion.  No he is not discontinuing it but rather he will be getting it at home weekly and a little bit differently (not through his port or IV).  The version he is getting is called Heizentra.  If I am understanding it all correctly, this is why:  by getting it weekly in a more concentrated but slower absorbing manner he will have less side effects and hopefully also better results.  This is because there will be a more constant level in his system.  There will be 3 probes (little needles) placed in his stomach and hooked to a small pump.  It will run for 1.5 hours each week and he can do other things while it is pumping in (tele-class, TV, Xbox…).  It also means less hospital stays, road trips to the hospital, and hopefully no more blasting headaches from the med.  He will also learn how to do it himself.   If we can get his levels up, he will also be able to be out and about without so much worry that he could easily catch something and land in the hospital.  Way huge!  We are very excited about this, especially knowing now that he is likely to be on this for life.  This will give him some normalcy back and empower him.  And we will all get to sleep in our own beds more often J!

We appreciate everyone who has rallied around us and supported us during all of this uncertainty.  We are incredibly thankful for your prayers, thoughts, and positive energy.  We know that Parker is an incredible and valuable “playa” and we are so happy you all are part of the Purple Playas Team!

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.

Progress with Systemic On-Set Juvenile Arthritis and the Drug Rilonacept

Week 17 on Rilonacept...These past few months I feel a bit like I have been “holding my breath”….waiting…to exhale and release…  I have wanted to update Parker’s status but have not known how to put into words the progress….AND lack of.  This morning, as I sit and try to find the words, I am still feeling at a loss.

Our family has been through a tremendous amount of challenges, while at the same time gaining strength, connectedness, new friendships, faith, clarity on value of life, compassion, hope, and love…We are so thankful for the loving support that has come from very unexpected places and people.  There have been strangers who approach Parker at the hospital and inquire about his illness; the video game store cashier has totally connected with our boys and revealed he has RA; the children from Parker’s school (where he hasn’t been for about a year) connect with him on x-box daily; the nurse at his school still collects tabs for the hospital’s Ronald McDonald House; our co-workers check in on us and help us out at work so we can attend to the medical needs; the nurses and child-life staff that come from others areas on the floor to Parker’s hospital room just to say “hi”; the early childhood professionals in the field who just come up to me and hand me a check to raise funds for these children and their families; the other “JA” moms who send me a quick note of encouragement; and the children and babies who look into my eyes and smile filling my soul up with joy…

We also extend our deepest gratitude to the team of doctors and nurses and researchers trying desperately to find a way to help Parker…of course one would "expect" that this would be a group of people who help…but it is “how” they are supporting us and treating our son that is most touching.  This group of people has become our angels on earth.  Systemic on-set juvenile arthritis has been the scariest experience for us and their persistence and patience is never-ending.  I have wanted to yell out to everyone that Parker has made progress on the study.  Rilonacept is definitely making a difference and has helped Parker make some gains…the incredibly hard part is he has soooooo far to still go.  As a parent and a sibling, we watch and want desperately for things to go back to the “way they were”…it is this part of the disease that is relentless.  We, as a family, are coming to the realization that this disease has attacked someone we love so very much and we cannot “stop it”…it keeps attacking.  I have to believe that it is going to get better…I have to believe that the drug is somehow helping and that if we were not doing this treatment it would probably be worse…I have to believe that God will protect our son and give him quality of life…I have to believe that our love and strength will pull us through… I have to believe that a cure will come….all while we hold our breath and wait….always waiting… 

A parent wants to protect their children…it is our instinct….it is our love.  We hurt when we see our children hurt.  Parker is still struggling.  And when Parker struggles, then Logan struggles…even though he doesn’t always “say it”, we see it; feel it.  Parker has been having very “yo-yo” like days.  Some days are better than others and we live in those moments.   But our reality is he still hurts big time.  He intermittently has bruising issues (this scares him because he knows the connections of what this means).  His shoulder is now popping/clicking and sometimes freezing up (we are doing x-rays this week, possible MRI). He is still having jaw issues and I need to get him to a specialist who can make a mouth guard (at the tune of $700-900).  He needs to have his scoliosis evaluated…  He is having red lines again.  The fevers are low grade but still there.  He has red hands and feet again with peeling (usually means he is fighting something).  And his immunoglobulins are still really low.  We find ourselves dealing with the biggest, most urgent medical need of the moment, but they ALL need attending to:  the splints need stretched out (swelling makes them uncomfortable yet again), his teeth are reacting to meds, he could probably use a visit to the counselor, and the list goes on….and on…and on.

But we are trying hard not to focus on the problems (although we cannot ignore them).  He is less swollen.  He does have less rashes.  He is moving better and on some days doesn’t use his wheelchair when out.  For this we are thankful…and he is making all his hospital homebound teleclasses (no absences).  He desperately wants to go back to his school and we were told this week there are no openings in 8^th at the moment and he is crushed.  But we are hoping that he continues to improve and that an opening comes up.

Parker is voicing the impact this disease is making on his life.  He is trying to look into the future with optimism.  He is becoming a little advocate for JA.  And although we try hard to not allow JA to “define” him…it is now a huge part of his life…HE LIVES WITH IT…But he is still sweet Parker…our son, a brother, with incredible drive, undeniable strength, a passionate Tampa Bay Rays fan, with techy talent, and a contagious smile.  We love you so so much Parker!

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.

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 down...now 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!