A Video Update from Parker...day 3 post op

Oh what a difference this afternoon...Parker is quite happy with his progress and you can see the joy on his face in this video.  We are so happy for him.

Click here to see and hear from Parker

Loose Screw and Stronger Than Titanium

First off, Parker is doing great, especially considering the major surgery he just endured.  We can’t even begin to tell you how proud we are of his “stronger than titanium strength”, his patience with the “unexpected”, and his resilience.  I am marveling at his growth since this time last year, when he only had one foot repaired.  There have been some frustrations that normally would have had him ranting, but he really has kept his cool and been polite regardless and he cried/complained when we were alone in the room.  This is huge, considering how little control he has over things right now.

Secondly, he is on the floor he adores, with the nurses he loves and who know him well.  That is a huge blessing since they were going to try and put him on an adult floor, because you know our kiddo is a man-child now.  Being on this floor gives him some predictability and the staff has amazing kindness and love.  The nurses are incredibly special to him because they care so much.  Plus they have watched him grow over the years.

Now for what HAD to be done…The left foot went as expected and even a little better than expected.  The surgeon was able to get the entire broken screw out of his foot without damage.  That was huge, because he really thought he would have to leave the back half of the screw in.  He used the bone harvested from Parker’s hip to repair the left subtalar fusion and added a new titanium screw.  Then, he went into the right foot and unfortunately there was a screw loose in addition to the cadaver bone failing.  So the loose screw was pulled out and he reinforced the bone with the harvested hip bone and put in a smaller screw to fuse the foot again.  The left foot/ankle is casted and the right foot is splinted.  Both feet/legs are numb from the epidural, which is awesome.  The left hip is screaming at him. They are working on controlling that pain with oral meds, so he can work on movement in the hip to get ready to start practicing transferring from the bed to his wheelchair without using his feet.  Most of that hard work will start on Thursday when the epidural is removed.

In the meantime, he will get SI joint injections.  Also, the nephrologist just called to share that the results of his 24 hours urine study didn’t show improvement and that she wants a med increased and has requested an ultrasound for another possible kidney stone.  I guess we are in the right place … so that is being added to the protocol for today or tomorrow. 

In addition, his lungs have been a little mean.  He has needed to use a rescue inhaler and do some lung exercises.  He has worked like a champ all morning and is now resting…didn’t get much sleep last night. 

We are pleased with his progress thus far.  He is in good spirits and loved having his brother drive into town last night to see him.  Once again, we thank all of you for your never-ending support.  Our family is most blessed to have such good people in our lives.  We love you all.

Just a Bit of How Things Are…

I know we have been quite … Parker has not been feeling well at all.  As a mom, it is incredibly hard to watch your child struggle day after day.  I keep thinking, tomorrow will be better…we are going on two really rough weeks with loads of inflammation and some adrenal issues.  His adrenal insufficiency can be pretty scary and every time this starts up, I feel my heart squeeze so tight it hurts.  We came so close to losing him with one of his adrenal crisis episodes and I tell myself we are better equipped with both knowledge and meds now, but I still get scared. 

Parker is now taking a stress dose for his adrenal insufficiency because he might also be fighting a virus.  And he is also taking meds for his pain and inflammation.  During these last 2 weeks, he has barely been out of bed.  The balance of helping him move so he doesn’t hurt more from being stiff, and the balance of not moving too much that it makes things worse, is quite delicate.  And I admit, I am not sure if we will ever master that.

It’s been hard for me to write blogs lately…I have wanted to allow Parker to tell his own story.  But when Kristen was here for her extended stay, she and Parker told me that a mom’s perspective is also valuable and they encouraged me to blog more.  The thing is, it makes everything so “real”…and I keep thinking and praying that Parker will get better…in some ways he has but in other ways, well… It is just our reality. 

So I cry in private on the really hard days; and I try as hard as I can to be “normal”…going to work, helping others, being as social as I can, and sometimes just being quiet and to myself.  On the “good days”…I just want to do everything we can fit in because I want so badly for Parker to experience JOY.  Of course, there seems to always be a price to pay for joyful interludes. 

Having a child with chronic illness can be quite lonely…because it is chronic, conversations are hard.  Parties are harder… I don’t know if other moms have these moments too…where you stand in the middle of a room full of people in a buzz, usually for a joyful gathering and you feel totally alone.  It is the weirdest thing ever.  I don’t know how to begin conversations anymore.  And I don’t think people know how to converse with me.  And sometimes, I avoid … because it is easier, less exhausting.  It’s like I am holding onto my reserves.  Just this week, someone asked about Parker and when I started explaining his surgery it turned into “oh, I have arthritis too”….and I got to hear all about how this older woman had it so hard.   Then, I felt horrible because I think she has no idea how her comments hurt …but that is just it, so many have no idea.

And let me tell you…all you moms with young ones with severe chronic JA…well, when 18 hits…watch out.  My world is a bit upside down.  18 makes you reassess, plan for the future that you were certainly not prepared for, and oh the paperwork…  I know not all kids are as severe as Parker, but whoa…this 18 years old stuff is something:  Do we do permanent disability?  Then there is Medicaid (or will there be…)?  Will he live on his own; or should we get a home with a separate apartment or mother-in-law suite? How long should I work?  Will he work?  Will I run out of sick leave?  Will we ever take non-arthritis related trips/vacations?  Do we remove his port and put a new one in or not?

And then I remind myself…I can only do what I can do in a day.  So, we do what is needed in the here and now:  Like buying two pairs of shoes, because one foot needs a nine and the other needs an 11 due to the ankle/foot orthotic…Or bringing the puppy up on the bed for some Parker cuddle time and a joyful moment…And making a yummy snack of yogurt, granola, and berries because Parker will eat that.  So I admit, these last 2 weeks have been really hard and we could really use some better days in the near future.

My Monthly “One Moment” with the Specialty Pharmacy

Filling Parker’s SPECIALTY meds is really a very special kind of experience…I know you all want to see through my lens for a moment…don’t you?!

You know, because Parker is extra SPECIAL, his meds can’t be filled at the local walk-in kind of pharmacy…on no…they are mailed on ice in these little white styro-foam coolers…and every month, I think, how many igloos are now in our landfills.  And I think we have more ice-packs than food.

A call to this very “special” pharmacy goes a little like this…

I dial, then hit the many prompted numbers to get a person, then the kind voice on the other end asks how they can help…thinking they can…but I know that they can’t…and I am about to cycle through at least 3 people…but today was an extra kind of special.  I got to talk to 9 people  … most of who were totally helpless…because their system is BROKEN.

So the first person says “one moment” and puts me on hold…and I get to listen to their jazzy little beat (ba-da-ba-da…bit-dit-dit…bit –dit- dit…do-wa).  That little loop is 30 seconds and it loops and loops over and over.

So I put my phone on speaker and go about my business because I know it will be anywhere from 5 minutes to 10 minutes before the first person will begin assisting.   But I get to listen to that jazzy little bit…

I get the second person and I explain again that I need to speak with someone who can fill HyQvia from the IVIG team (because I know that with this script on the list, even though there are 2 others for today, I can’t "pass go" until this one is filled.).  I hear “one moment please”…cue 5-10 minutes of jazzy little bit loop…

“Hi, who am I speaking with?”  I run through name, parent name, DOB, address… “I see you need to fill Ilaris, Otezla, and HyQvia, but I need to get you to the team that can help you.”  I ask what team I am with and am told, Rheumatology.  I tell her “wait, you can fill the Ilaris and Otezla”.  She replies, “No mam, I need to get you to the correct department, I can’t fill the HyQvia. One moment please.”  Cue jazzy little bit….

This time, about 15 minutes later, I get a grumpy lady who is asking questions at a quick time speed of that jazzy bit.  She fills the med and is acting like she is done…I am like “wait…there are supplies too”.  I hear “what do you want”…I kindly explain that all the supplies that I got last month were wrong and luckily I had extra supplies on hand and was still able to administer the infusion.  But I must get the right supplies this round because I don’t have spares.  I ask her to fill the same as December’s supplies.  Again she acts like she is done and wants off with me as quick as possible…”oh, I am not done…I need to fill 2 more meds”  and I explain I need to be transferred back to the rheumatology team.  “one moment please”….cue jazzy little bit for another 5 minutes….

I get the next person and they can’t help out at all…”This is a specialty med mam, I need to transfer you to the team who can help. One moment please”…by now I am laughing because I just have to…(ba-da-ba-da…bit-dit-dit…bit –dit- dit…do…….silence……disconnected).  Here I go…onto repeat mode all over again.

I dial and literally get to go through this all again.  I explain what I am calling to refill and that the HyQvia has been completed and to PLEASE transfer me to the rheumatology team….”One moment please.”  Ok, let’s be real, we all know by now that it will most certainly not be “one moment” and I now officially hate jazz.

And… even though I had explained that I already filled the HyQvia, they transferred me there again.  Guess what, I start laughing because this is too funny not to laugh at (45 minutes later from my initial call).  I get the guy to laugh and he transfers me to who can help, with a sweet “one moment please”.  Cue jazzy irritating bit…

Oh the poor woman who picked up the line…we both didn’t know that I had had enough “moments”…She was with the main refill line, not the rheumatology team and when she said “one moment please”…I said “No, wait.  I really don’t have any more moments to spend trying to fill these scripts.  I need to speak with a supervisor.”  I know I took her off guard.  Because she said “I can’t fill these meds.  They are specialty meds.  I need to get you to the team that can help."  I tell her that I have been trying to fill these scripts for an hour now.  She says..."an hour?"  I explained and then tell her that my son is on a lot of “specialty meds” because he is one special kid and I work full time and I am a mom who is trying to help my child.  And I demanded that she get me to a person who can actually help me complete this order.  She apologizes in a very flustered manner.  I actually feel a little sorry for her, but I stand firm and say I need to speak to a supervisor.  Poor thing…she said “one moment please”….jazzy bit plays and I am now an hour into this.

When she finally comes back on 10 minutes later she says, “I have someone who can assist you”…I say I so hope so and we both wait…silence…I say “hello?”  and the poor soul I let have it says, “Oh my gosh, we were disconnected.”  And she sounds like she is going to cry.  I find myself counseling her, saying it is not her fault and that their system is broken and I don’t know how any of them can work there.  She says she WILL get someone to help me and she told me to hang up, and she would have someone call me back so I wouldn’t have to be on hold.  Halleluiah…No more moments with that dreadful jazzy bit…

John call me back, fills the final meds.  Then explains to me that Parker’s in a very special group of patients with a lot of meds and supplies and there is a note on his file that says “don’t fill, send calls to the IVIG team”…I explain that I already know this and no matter how much I try to help them on the other end of the line to do this correctly, by first going to the IVIG team, and then to the rheumatology team, I always speak to at least 4 people… but today he was my 9^th.  We got through the order and then he said I could set up his records on-line and do his orders over the internet.  SIGN-ME-UP…But…I will still have to call for the IVIG and new scripts.  Well, at least this is improvement…right?

I tell him that I am thankful for this partial solution and then he gives me his direct number for the future and says that if this starts happening again when I call in, that I can call him direct or even have the team text him; and he will call me back and help me fill scripts efficiently, so that I can work and take care of my son.  I tell him I look forward to him helping me in the future … and we both knew that this was probably true because their system doesn’t work in our favor and we will likely talk again.  He admitted that patients like this fall through the cracks and that it is wrong and he hopes to correct it.

I know this is a long rant…but I want other caregivers out there to know that there is this solution. Also I want those with healthy children to be grateful that all you need to do when your kid has a need for a pharmacy is drive down the street.  That is truly a gift.  1 hour and 45 minutes later, I am grateful for these meds that keep our child ALIVE!

A New Diagnosis…A New Biologic

So much has happened and yet it feels a little like time stands still.   Since Thanksgiving, Parker has been struggling.  Even with his multiple nerve ablations, he still has much lower back pain. In addition, he is incredibly fatigued.  He did repeat MRIs and unfortunately, since March, this darn JA has progressed even more.  He officially received an additional diagnosis in November of Anklyosing Spondylitis (AS).  The doctor is very concerned that while we have been treating his systemic JA, the AS has progressed.  So she started talking about a change in treatment.  Of course, any time there are med changes it is scary.

In the meantime, 2 labs were not too good.  Prograf (anti-organ rejection drug) has been working really well for the past 3 years, but now it is affecting his kidney.  So, we had to stop it.  He is now on otezla and they have seen some kids with psoriatic features do well on this.  So we are giving it a try.  In addition, his immunoglobulins were low, even with the IVIG he gets monthly (infusion of blood plasma).  So they increased the amount.  We are hoping this helps boost his immune system.

Then in December, Parker had blood and mucus in his stools.  And we had an appendicitis scare, that was actually swollen stomach lymph nodes.  This has happened before.  For some reason, the systemic JA tends to attack his lymph nodes.  In addition, the lymph nodes in his neck have been large.  We did an ultra sound and it was confirmed.  There was also concern about his thyroid, but that looked ok on the ultra sound. Endocrine is now running thyroid labs and glucose labs (because those were elevated last round).

So, in early January, Parker saw a GI specialist who immediately did a scope.  The scope came back good with no explanation for the blood.  The only issue was his esophigils were elevated, which may mean that his body is responding to the corn he has been eating.  Allergist cleared him as no longer being allergic to corn, but now those labs have been added to his list of tests.  It might be an intolerance response.  Although inflammatory bowel diseases did not come up on the scope, we are going to keep an eye on this because he is at risk with all his issues.

Finally, we thankfully got approval for the new biologic, cosentyx, which is going to target this AS issues.  We are hoping that it will somehow help his systemic JA as we don’t know if it will or not.  It is an IL-17 drug…for those of you who are wondering.  It looks like he may have to stop Ilaris which has been keeping his systemic internal organ issues with his heart and lungs, fairly quiet.

So all of this has been going on while I had an MS scare.  After 3 docs said I could have MS I went through a series of tests and I don’t.  Thank God!  They believe that I have been experiencing something called Hoshimotos encephalopathy.  In addition, I was on B12 injections from when my Graves disease was active, and now I am in Hoshimotos mode.  My body had too much B12 and that is not good.  Since stopping it, some of my symptoms are much better.  My thyroid has not been nice and neither has my own RA.  So if I have not gotten back to you…all of this is why.  I so apologize.  I am working on removing as much stress as possible for myself and inserting as much JOY as possible for Parker and our family.  Love you all and thank each of you for never giving up on us.

 

Parker wants me to share…

Parker asked me why I haven’t blogged lately.  I told him that now that he is older, it really is his blog and his story and I didn’t want to say things that he may not want shared.  He asked me to please blog and tell everyone what is going on, because he has such a hard time staying awake lately and because it is OUR story…our family’s story.  So here I am, writing, and realizing I have also been avoiding voicing out loud that this life with autoimmune issues has been so very draining on all of us.

I have been going through all kinds of tests due to my own autoimmune issues.  Logan has been flaring for about a month and like me, I think he is trying to avoid more medications.  And Parker seems to conquer one thing, just for another issue (or two or three more) to arise.  Michael and I are realizing we barely have time for much else…we want to work on our house, which has been neglected since this journey has begun.  We want to spend time with friends, but barely have down time.  And the thought of adding holiday decorating to our to-do list is just one more thing to do…and yet, we want to because the result is joy and a reminder that life is precious.

We want to keep celebrating life’s joys, create simple moments with beautiful memories, and keep a focus on what is going right.  And yet, we know, that all of you want to know what is going on with Parker.  And somehow, writing is all down, brings it to the surface.  I realized yesterday, as I spoke to a friend, that I have been trying to smothering it all down inside of me in my efforts to stay as joyful as possible.

I feel like I have lists upon lists…So here it goes…Parker’s “list” of medical needs:

• Parker’s blood labs have been up and down.  Some are decent and some are yo-yoing. 
• His uric acid is high.  He sees a new doc, a nephrologist, next week.  This is doc 18. It is possible that this is due to one of his meds, which we have temporarily stopped and will re-run labs this week.  Or his kidneys are somehow being affected by this mean disease.
• Despite all the things we have tried to do to help his SI joints, they are still bad.  He is having another MRI, at UF Shands, in the near future.
• He is still thinking about continuing the nerve ablations…this is a tough one because he was awake for the last one and it was horrible, but it did help.
• We think he has a leg length discrepancy, so a scan-o-gram is ordered.  His bones will be measured and he may need a lift in his shoe.  Let’s see how that goes since we still can’t get his shoe orthotic inserts, post-surgery, approved.
• He is having blood pressure and temperature issues after showers and doc wants him to go back to cardiologist, which is two hours away.
• We need to remove his port in the near future because he has grown out of it and it is pulling.
• He needs to go back to his regular ophthalmologist because his eyes keep getting really red.
• He has a swollen area on the side of his neck.  So, we have an ultrasound scheduled, since it is near lymph nodes and thyroid.
• Doc is trying to get a new med and a new biologic approved.
• We took him to orthodontist and he needs invisaline and botox injections in his jaw.  Cha-ching…  Doc had others come over and feel his jaw joints because he said they were tighter than anyone’s he has ever felt.
•  We took him to GI doc (actually Michel did…thank you dear God for this man in my life) due to blood in stools and it is likely due to fissures…whew.  Easy solution.
• He is having some pulmonary issues again, so back to pulmonologist and needs to also schedule another sleep study.
• And finally, doc is trying to get him in to NIH with a doc doing genetic studies on kids with multiple autoimmune issues.

Needless to say, this is a lot for a kid to process.  He is doing the best he can with the extreme fatigue in his body.  And for all those people who unknowingly ask about school…to make conversation…oh my word…it breaks my heart every time.  He has various answers for this based on who is asking, but there is always a major pause…The medical stuff seems to consume us. We know and he knows he must get his GED finished, but oh my word, sometimes it just feels like it is the least of our worries.  But he will get there one day.  When it happens we will have a party.

So friends and family, please be patient with us.  We know we don’t connect much and our interactions have been minimal.  We wish we had more time to visit, chat, and go out.  But please also know, you are all in our hearts each and every day.

Entering Back Into the Real World: A Quick Update on Parker

Since Parker’s surgery, on March 2, on his legs, ankles, and feet, it is officially confirmed that Parker is OUR HERO and absolutely stronger than he ever thought he could be.   Tomorrow will be his first outing out of the house and we will be going to the doc to get his casts on.  Once the casts are on, then he can go back to school.  So, he is going to try go back on Friday to take the state writing test; since the deadline is Friday.

He is progressing but definitely has a very long way to go.  Last night was the first night that he didn't call over the “walkie talkie” for help.  Michael had a meeting with his pain management doctor yesterday morning and conference called me in on the phone and we are adjusting Parker’s meds to hopefully help him out.  The struggle has been the shooting pain, some minor bleeding at the pin-sites, numbness, and muscle cramps.  And even though he had a fever yesterday, we think it was more JA related than surgical…so we are incredibly thankful that there hasn't been infection.

We will try to update more this weekend.  For now, we thank everyone for the out-pour of love and we ask that all of you please either sign-up to walk with us on May 2 (it’s free to register) and/or please consider making a donation in honor of Parker and Logan.  Click here to get to the walk page.  Time has slipped away from us and the walk is just over a month away; so we need to get our team together very soon so we can make our shirt order.  We are forever grateful!

Much love,

The Lentini Family

P.S.  There is one more picture below so if you are skirmish and don't want to see a bruised up foot and a pin poking out, don't scroll down .

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Surgery is Done...Now for a Whole New Kind of Torture Full of LOVE

In Pre-Op

Never ever did I imagine my life would take me on this journey…All my life, I have wanted to work with children with disabilities…but never did I think I would have children with disability…I have dreamed of becoming a mother…but never did I believe I would be the mother of two children with chronic illness.  I have hoped to marry a loving, caring husband, but never could I imagine I would partner with the most amazing individual with incredible amounts of love and he shares it all with me and our two beautiful boys; and still he has love to spare.

Some days my brain feels like its on overload.  Today is one of those days.  Mixed feelings fill my heart, giving me a sense of emotions-on-overdrive…I just went from tough mean-mommy, to having a sense of stabbing sadness of what Parker has to endure if he wants to walk, to my heart breaking knowing that he is telling us (PT, nurse and me) the complete truth that he hurts so very bad, to knowing I must muster up that tough love and MAKE him do what he doesn’t want to do because he knows it will cause more and more pain…  I had to make him put those arms with flaring shoulders and wrists down and wiggle and scoot and drag those million-pound legs/feet and lift himself into his wheelchair.  Then the torture continued as we had to then get those legs off that bed and down onto the wheelchair foot rest as tears rolled down his face and “I hate you all” came out of his mouth…But… the one emotion that is emerging more than all the rest is incredible pride in the strength and bravery that Parker is enduring this…because he DID it, he really DID it with splints on his legs/feet, cadaver bones piecing him back together in alignment, and pins criss-crossing through his bones holding it all together and in place.   

I know there are many adults with RA that have had reconstruction…but we only know a few kiddos who have endured some sort of reconstructive surgery.  WOWZERS!  I am so incredibly amazed with each and every one of those kid-heroes.  What they have accomplished is a miracle.  I just watched my kiddo go from hating us to just about doing the entire transfer back into bed, with such determination, that he did most of it with his own strength and not one complaint.  The nurse was shocked!

Gram & Gramp

This hospital does not have pediatric rheumatologist; and so the medical staff rarely work with kids with juvenile arthritis.  So, to have a kid like Parker and all his complexity…well, let’s just say, that all have learned a ton this past week and they have been absolutely open to learning.  I actually really appreciate their interest and questions, verses just pretending that they “know”.  It is very refreshing and Parker feels really smart as he educates them.

So if you are reading all this…YOU are one of those really special people who care a whole lot.  We so appreciate all the out-pour of love, support and prayers we have received this week.  It truly has powered our entire family and we really needed it.  Many of you have asked how you can help…there are two ways you can help both us and children with JA.  One, our whole family is very involved with the National JA Conference this year because it is being hosted in Florida.  If you know a potential donor, in-kind sponsor, or you would like to volunteer with either prep or at the conference event, please contact us or Susan Cuellar (Florida AF office:  813-968-7000 or 1-800-850-9455 x11) and tell her you know us and how you would like to help. And two, as you may know, the walk will be this May and we would love for you to sign-up (it is free) and walk alongside our family on “Parker’s Purple Playas and Logan’s Dream Team”.  (Click here to sign up on our team.)  And of course, we also welcome donations to our walk team, too because we definitely need more research towards a cure, to send kids to camp, and to scholarship families to go to conference.

Like I said, never did I imagine that my life would take me on this journey…but it has and I am proud to say that I have become a nurse, a fundraiser, a conference planner, a JA educator, a counselor, a motivator, but most importantly…I am a wife and a mother who deeply loves my family and am so happy that there are people like you in our lives who love us too.

Parker's first post-op request was to see his brother, Logan.