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…

Open minds and open hearts bring openings for possibilities!

I want it all to go away today…no more JA!  My heart breaks for my son.  It seems that no matter how hard we try to insert normalcy or fun moments into our lives the price Parker pays scares me. 

We want him to walk.  We want him to be active.  We want him to play.  As he should...  But there is such a fine line between the joy it brings and the misery that follows. 

Don’t get me wrong please…we are happy about so much (as you can read in the previous post), but this yo-yo life is so difficult to witness.  Only Parker LIVES it…we need to continue to raise awareness.  These children need a voice that is loud and proud!

We know that people don’t want to hear about depressing things.  We know that people don’t desire to experience heart ache.  We know that life is going on all around us and there is much beauty in life.  We actually feel that our hardships have taught us to appreciate life’s beauty all the more because when simplicity and good health is no longer…you cherish the moments of joy.  They are truly blessed moments…

But I want ALL to know, to listen, to understand the depth of what our family (and many other families) are dealing with in the privacy of their own homes.  This last week was extremely hard for Parker…again…As many of you know, he is now in hospital homebound.  This week was FCAT week.  He could barely get through the three mornings of testing, not because the content was too hard, but the mere fact that he had to “do it”.  He not only completed the testing in intense pain, but also through his incredible fatigue.  He fell asleep during testing, but also on the ride home, and again once we got home.  (Yes, we know that he can be tested at home…but they thought he could handle it at the test site and we wanted to try.  Parker wanted to try.) 

Parker barely did anything Friday or Saturday following FCAT.  But when he asked if a friend could spend the night Saturday we jumped on it and he did.  He had so much fun with his friend playing video games and chatting.  And hopefully we didn’t scare him too much with the infusions he witnessed. 

Parker then rested all Sunday morning to prepare for our Sunday afternoon fundraising event.  He insisted on going.  He took extra morphine, slept all the way there and he was a complete hit!  I really don’t know how he revved up the energy to be engaging and gracious with everyone who came out to support HIM; but he did it with a smile.  And he has been crashing ever since.  HARD.

When he was getting ready for bed he said to me that it didn’t matter how much money he raised today because he taught some strangers about juvenile arthritis and one of them was an old dude and his old dude friend.  I just love this kid.  He just wants people to KNOW that kids get this and it is an AUTOIMMUNE disease…it is not osteoarthritis!  He is so very strong. 

This disease is devious and deceiving…Parker can look fine on the outside, his spirits can even be high and then in the split of an instance things can change…and we never know when it’s going to hit.  This year we have learned a lot about this disease…one thing that we have learned that I didn’t want to learn is that few children with systemic juvenile ever go into un-medicated remission.  We have also seen children pass away from this form of JA.  That thought totally takes my breath away.  It makes me pause and truly appreciate every single God given moment we have on this planet together.  But the gifts we have are endless…we have seen when people open their minds (to accept this disease for what it really is), with open hearts (to show the love), then opportunities open up to endless possibilities…

I end with “William’s Wish”...as we prepare for our own local Arthritis Foundation’s walk.  William’s family is forever in my prayers and heart.  Rest in loving peace sweet dear William.  Please click on the link below…

William's Wish broadcast

Much love to each of you and may your heart and mind always be open!

We Will ALWAYS Push Forward! That’s What Parker’s Purple Playas Do!

Our journey with this horrible no good very bad autoimmune systemic diseases is teaching us many valuable lessons about strength, love, resilience, faith, hope, relationships, and perseverance…no matter what!  This disease is smack in our face every single day and I hate it with a passion and yet at the same time I love what it allows us to “clearly see” and “completely and fully appreciate”.

Never ever did I imagine that our journey would take us down a path of what feels like NO return to simplicity.  But yet, simple moments are what I am finding that I value the most!

I have told many who are following our journey and who lift us up in prayer or carry us along with their encouragement that I would share what our newest days are like.  Well, we are seeing some “good moments” with Parker’s health and he has been able to enjoy some fun activities again.  For this we are truly grateful to his doctor for continuing to think a bit outside the box.  We all know, Parker needs some “forward thinking” around his health issues.  However, our insurance has changed a bit with our state's new plan (I am a state employee).  We now have to go through a new company when ordering our medicine and that has resulted in some challenges and additional expenses.  Parker’s doctor actually wants him to get twice as much of the newest med but they will only approve half of the amount…Completely frustrating.

Parker has discontinued Actemra and Enbrel.  When he was in the hospital he got a “boost” of Remicade and was also started on Kineret.  He is still on all of his other oral meds with an increase of his steroids due to his recent hospital stay and huge flare of mean ol’ systemic JA.  And he is continuing his monthly infusions (right now every 3 weeks) of IVIG to boost his immune system.  Many families in the world of JA and RA refer to this newest med we are trying, Kineret, as the “evil med” because it is traditionally given as a daily shot and it burns so bad the kids, and adults, beg for something else.  (Yes, every single day these children are being tortured.)  Parker is not getting it as a shot (for that I am thankful)…but he is getting it and not once a day but twice a day.  And I am infusing it into his port.  His port is now accessed all the time.  And every 7 days, a home health nurse comes to change out the port access.

Just to give you a true sense of what a daily infusion entails, we did our best to take pictures of the steps.  Cleanliness is key because the port is a direct access to his heart and we certainly do not want to infect it.  The whole procedure takes me about 20-25 minutes.

1.  Wash really really good…using paper towel to dry and open frig and doors with towel.

2.  Get Kineret shot out of frig and wrap hand around it to warm it up as I prep everything else.

3.  Clean bathroom area with Clorox wipes and clean drawer knobs and plate to hold all syringes/shot.

4.  Pull out 2 saline syringes, 1 heprin syringe, 1 empty sterile syringe, 1 cannula (sterile syringe cap), 2 alcohol wipes, 2 non-latex gloves

5. Place Kineret down (remember it’s been warming in my hand), put on gloves and open all items making sure not to touch tips and only loosening caps

 

6.  Transfer Kineret from syringe with needle to the empty sterile luer lock syringe and carefully cover with sterile cannula, place needle syringe in sharps container

7. Then I can take all of the items to Parker and get ready to administer using the nifty acronym that I was taught:  S.A.S.H. (however I had to add an extra saying to it because otherwise “SASH” doesn’t work at all!)  First “unlock” SASH, then “lock it back up”…

8.  So what this means is: 

Unlock clamp, clean with alcohol

Saline (taking great care to not touch tip AND remove all air bubbles at each and every step)

 

 

Administer med Kineret (over 3 minutes)

Saline (first portion is still very slow because Kineret remains in the tube)

Heprin

Lock clamp

9.  In between each and every step the port access needs cleaned with alcohol and the air bubbles removed from the syringe by holding it up, pulling back on the syringe to remove the vacuum, and then pushing it up to the top of the syringe.

When done…let out a big sigh of relief!

Yes, it is all very stressful! Parker’s fevers are gone now and his movement and pain is a bit better on some days and he is getting a few bursts of energy here and there.  Yes, he is still in considerable pain; he still is having vasculitis, the rashes are trying hard to break through and are faintly there; his joints are still swelling but not as bad as when in the hospital; and he is still have quite a bit of fatigue…but he is also doing more because, like I said, he has small bursts of energy.  This autoimmune stuff is no fun at all because he gets so excited about being able to have some fun and be a bit more physical and then after he does, the price is costly…he spends much time in bed.  But he did have fun and he is glad he did!  He is having some stomach issues again, but we are very thankful that his heart, lungs, and other organs are doing ok right now.

I took his port out after his infusion Friday morning so that he could swim for bit…30 minutes.  My first de-accessing of his port ever!  Also on Fri, he was invited back to his middle school to join their neon dance.  He had a blast.  He saw his buddies, wheelchair danced so smoothly that he drew a big crowd around him cheering him on, and he even dance with a girl (a friend….and very happy that she asked him to dance).  He went on and on about how much fun the dance was and he now wants to go back and visit the school during lunch time so see his friend again!  He really has missed the face-to-face contact.

I really don’t want to give Parker these infusions twice a day!  I really don’t want to be scared twice a day that I might do something wrong or hurt him in some way!  I really don’t want to see this horrible autoimmune systemic disease outsmart every drug we try!  But we must, so we do!  And we will ALWAYS keep pushing forward…until there is a cure!  We are hoping to get Parker into the RAPPORT study that is an National Institute of Health (NIH) because that drug is much less intrusive than what we are doing now and is a drug already approved but for other uses.  But the other uses are somewhat related to symptoms of systemic JA.

If you are reading this, you can see that Parker is in the fight for his life.  We continue to be inspired by his strength, wittiness, and outpouring of love.  We want to support him every step of the way, but we cannot do it alone.  So thank you to each and every one of you reading this and thanks to those who are following our journey, praying for him, supporting us, and/thanks to those donating your time and/or money for our upcoming walk.  We only have a little more than a month until Parker’s Purple Playas Pound at JA at Al Lopez Park in Tampa, Florida on the morning of May 5^th.  We will be the sea of 50 plus people in purple!

With HOPE...anything is possible, even a CURE!

Join US for the AF Walk May 5th and/or Make a secured donation:  http://2012awtampa.kintera.org/faf/search/searchTeamPart.asp?ievent=1002436&lis=0&kntae1002436=22CB076514A4413A842EFBD89E814BCB&supId=0&team=4910470&cj=Y

Peace, Power, Purpose…It’s Within Us All

Well, we haven’t blogged lately…partly because things have been so crazy in the Lentini House, but also because we have really been trying hard to look at the bright side and to stay positive through all our struggles.  Somehow, by writing, it feels like admittance of how hard things have been.  But we also realize that for many, reading about such difficult times is not very “uplifting” and is probably draining on our family and friends.  But our reality is we are definitely being challenged lately.

We are so fortunate to have so many people who care about us and we do know that you want to know what is happening…so I will try to explain as briefly as I can but it’s a lot.  Since this is “Parker’s” blog, I will start with him.  As much as we didn’t want to admit it, Parker has slowly been declining.  I think we were hoping that the IVIG would be the little extra something that would bump everything into gear.  It has helped.  We are seeing his immunoglobulins improve.  And with this improvement he is better able to fight off infections.  However, the actemra (the biologic infusion he has been on for about a year) has failed him.  His vasculitis is horrible, pain is through the roof, swelling is over the top, skin coloring is pretty bad, rashes are back, and he is behaving like he is having fevers with sweats and chills but temp is hanging between 96 and 98.4 degrees.  But actemra suppresses fevers ( a scary thing because then you also don’t have warning signs of illnesses).   

Day 3 and He Still Can Find His Smile

Last Friday was Parker’s regular infusion day.  He was doing so bad he was admitted into the hospital.  He is still in the hospital.  Because the actemra has now officially failed, we must change biologics once again.  He has now tried enbrel, humira, remicaide, actemra, and then a combo of both actemra and enbrel together.  All have failed.  People this is why we desperately need more research! 

We Are More Thankful Than Ever That We Decided on a Port for Meds

Today, the doc is moving to another class of biologic drugs called IL-1.  He is being given kineret (also called anakinera).  He just got his first does.  She says that some of the systemic features of his JA will slowly improve with each dose, but the decreased pain may take longer.  He is going to get 2 doses a day infused directly into his port by his loving, caring, and a little bit scared momma.  This means that his port will be accessed 24/7.  One of the reasons we have moved to this drug is that we have exhausted most options.  Another is because his doctor really wants him to be in a study.  He can be on kineret up to 4 days prior to the study.  We are highly considering this study and I am currently talking with insurance about this as being a very important option for us.  The RAPPORT study is also with an IL-1 biologic (Rilonacept) but it only has to be given 1 time a week.  It is in its third trial study; they are seeing great results; and it is a drug that is already FDA approved for intermittent fevers which is a huge feature of systemic JA. 

It's hard to see in this picture but his ankles were about the size of
tennis ball.  There was also redness and swelling above his little toe.

This was one of his ankles the next day.  A little less swelling, but vasculitis is
evident and he kept getting rashes.  There is a bit of a rash here
above his ankle.

Swollen little hands, with red fingertips, grey middle segments
and white hands.  (Picture doesn't show clearly)

Rashes on arm, again it's not clear, but there were pink lines.
Rashes come and go daily again.

Systemic JA is very very complicated and scary.  Children can die from this.  It attacks not only the joints but internal organs.  Parker has already had complications with his lungs, lymph nodes, stomach, and heart.  Michael and I are not willing to let this disease take over without everything we have to fight with being utilized.  We also will continue to advocate for each and every child with JA and related autoimmune diseases.  The world needs to know how desperate all families are for a cure.  No child should ever have to endure intense pain and no parent should have to observe powerlessly and with constant worry.  We pray that the time for a cure is soon!  We need to the cure now! 

A Gaspirilla Krewe visited and
handed out beads to Logan.

In addition to our battles with JA, our other son, Logan, was hospitalized multiple times with severe migraines.  I know many out there reading this will be thinking about his well being and the stress of JA on our family system impacting Logan.  Of course it does!  How could it not?!  But we are pretty sure that these migraines were a combination of triggers.  One, he’s had migraines (that have been under pretty good control) since he was 5.  More recently, the combination of JA stress with his brother, puberty, and the IB program have certainly triggered this 7.5 week long intense migraine.  After a huge scare that this episode could have also been autoimmune related (he is ANA positive which is a marker in the blood) and thus ruling out vasulitis of the brain, we are very relieved that a combination of meds is now controlling his migraines again.  He is also going to be in biofeedback therapy soon which we are sure will give him some great coping tools.  Then hopefully we can also wean some of the medications.

Logan's Spinal Tap

Then there is my health…Without going into too much detail because we are still trying to figure “me” out, it is looking like I have an autoimmune disease.  The rheumatologist that I have been seeing for about 8 months now thinks it is a connective tissue disease.  I wasn’t going to talk about it on this blog, but then I got to thinking about how brave Parker is being and if I am going to be “true” about advocating, I thought it was time to share this news.  Actually, today was going to be the day that I talked to the doc about my own treatment, but that is now put off a bit due to Parker being in the hospital.  But as you can see, there seems to be a genetic link with all this autoimmune stuff.  I feel stronger than ever that this autoimmune stuff needs to be figured out now for our future generations of children.  I recently had an ER visit myself and ended up being admitted.  The intense pain that I felt hurt me emotionally more than physically because all I kept thinking is that my child is in this kind of pain every single day and I cannot seem to stop it for him.

Michael is the rock in our family.  He helps us all “keep it together”.  Thank goodness he is healthy.  His love, support, and understanding means more than words could ever portray.

Today, we have a glimmer of HOPE again as we journey down yet another path.  Parker also saw his karate teacher that he met in the infusion room.  He had class on the floor today and Parker went to part of it.  We learned to bow with peace, power, and purpose.  Today we worked on building peace within us, using the power within us, and utilizing our purpose…to teach the world. Please, if you are reading this, consider supporting us and other families battling autoimmune diseases.  Of course juvenile arthritis is near and dear to our family’s heart.  We invite you to join our team, Parker’s Purple Playas Pounding at JA, as we walk on May 5^th.  It is free to walk with us and you simply need to go to this link to sign up:  Click to link to Parker's Team Page

If you are not local, consider joining your community’s Arthritis Foundation’s walk.  And of course, donations are also needed as they help fund research, education, and camp for our children.  You can also support us in another way; join us virtually on International World Arthritis Day on May 20^th.  For more info go to: World Arthritis Day - Click to learn more   

And finally, you can support us by continuing your encouragement, prayers, positive vibes, and by sharing our story and educating others.  Only when the word spreads will there be enough awareness to “teach the world” and promote research for a cure.  Be there for the future of our children and their children.  

Much hopeful love, the Lentini Family