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!

So Close to Death and Life Wins

We have always carried in our hearts the knowledge that systemic JA is very complex and can be dangerous.  Sometimes this weight is too much to bare. So, as parents, Michael and I focus hard on the things in our lives that we can celebrate.  Our two most joyful celebrations we cherish are our two boys, Logan and Parker.  We have been so very blessed with all they bring to our lives.  As most parents, we wouldn’t trade them for anything in the whole wide world.  And because we adore them with every ounce of our beings, we also can feel such devastation when medical issues arise.

During the final week of school, we were painfully reminded how very fragile Parker’s health can be.  We know this to be true with our “minds”, but oh how we pray with our hearts for protection from this fact. 

For several months, Parker has had heart issues and is seeing a new pediatric cardiologist.  The cardiologist, and several of his other docs, feel that his heart issues are stemming from his autonomic system dysfuctioning.  This was firmly confirmed during his last hospital stay by the team of docs that surrounded the care of our dear child.  What makes this so incredible is that another young lady, whom we are very close to, is also experiencing autonomic dysfunction and it has been much more severe and she has been hospitalized for nearly 4 months (at two prestigious hospitals).  Her mom and I are close and much of the initial onset, lab results, and treatments were all the same for Parker.  Seeing her struggle is heart wrenching and makes us wonder “what’s to come…”.

Although this has complicated things for Parker, this is NOT what landed Parker in the hospital this month.  Very suddenly, Parker got incredibly sick with nausea and an uncontrollable gastro-intestinal virus.  Being nearly 16, I am sure he wants some privacy around this; so I will spare many of the details, but he did pass out and somehow made it back to his room after coming to.  I took his temp and he was running 104.9 degrees F; and he was completely delirious.  After calling his pediatric rheumatologist and leaving a message, I started packing because I already knew I was going to have to take him to the hospital.

Parker is on several immune suppressant drugs and one of them is a biologic, rilonacept (arcylist), that is specifically used for systemic JA because it is to keep fevers down.  In fact, it is also used for something called “periodic fever syndrome”.  One of its main purposes of this shot is fever suppression. We were advised, when he started the study on this drug, that if he ever ran more than 101 fever that we were to take him to the ER. 

After packing, I called the doc’s office back and by that time, Parker could hardly be awakened and was even more delirious.  I somehow got him into the wheelchair and into the car.  As we drove to the hospital, he was completely out of it and when we arrived he was starting to turn blue.  I was completely running through motions…pulling up to the ER entrance, pulling him into his wheel chair with the valet guy’s help, handing over my keys and rushing inside. 

A young man in the waiting room looked at him and said, “Mam, is he ok?” as Parker was sweating horribly, blue, and slumped over.  All I could squeak out was “yes, thank-you”…knowing full well he was not.  As they were handing me the form to fill out, a nurse rounded the corner and just took him from me and said follow me, pushing his wheelchair into triage.  She put the pulse ox on him and his oxygen was low.  And again she pushed him in his chair and said follow me.  As she entered the ER room, I was only steps behind her…and in front of me about 8 people swarmed the room leaving me to stand in the backdrop staring at my blue child, praying, and not knowing what to do.  They were hooking him up to all kinds of things, starting his port with IV bags AND digging for second access point to start another IV line, in his collapsed veins.  After 4 pokes and a second nurse trying, they finally got the second line started in his hand.  Parker never woke.

The lead doc asked me all kinds of questions and thank God I have all his diagnoses, surgeries, docs, treatments, and allergies all typed out on a list in my purse because I honestly was in shock.  All I could think to do was to give him the rheumatologist’s cell number …because I knew she would be able to explain his complexity and what labs to run.  There is something about seeing your child laying lifelessly on a gurney and his clothes being cut off him and him never waking up through all this. In that moment, I was so incredibly thankful that Parker’s sweet, caring, brilliant doctor had actually given me her cell number because even during this I felt like I was intruding on her personal life but I knew this was realllllly bad; and she held many key pieces of information that the ER staff needed.  She had saved his life before.

I was in the moment I have feared most with this disease…he was literally near death…I think closer than he has ever been.  As I type this I can’t stop my tears…I have literally dreaded sharing this in writing because typing this out makes it all too real and I am reliving it.  So I apologize to the many who don’t use Facebook and you are just now finding out that he was admitted into the pediatric ICU on June 3.

As they pushed multiple bags of multiple medications through him, and they finally seemed to begin stabilizing him and calming themselves, I called Michael.  Once again, here we were in crisis and he was out of town.  Of course, he drove straight to the hospital.  I then called Logan, as I knew he came home to an empty house.  I let him know that Parker was in good hands and to pray. 

See, Parker’s body couldn’t handle getting this virus on top of his systemic JA and his immune deficiency. Remember, he was already in autonomic dysfunction (dysautonomia has several forms) and on top of all this, his body was going into adrenal crisis.  Just that, on its own, is dangerous.  I didn’t realize until later in the PICU that this was also going on.  

That night was a very long sleepless night.  I couldn’t sleep, I could only stare at my child and the monitors and pray.  They had to move and shift him many times in that bed and he never ever woke.  I kept telling myself his body is healing…that is all his body can do right now…it is healing…I could not lose my child.  Not now…

Many specialists surrounded our son’s care that next day:  neurology, cardiology, endocrinology, gastrology, pulmonology, and pediatric critical chronic complex care specialists (who we actually have been trying to transfer him to for the past month because his primary doctor felt he is too complex for her to continue to treat him).    It was very surreal…many of these docs were partners to the docs he actually sees at the hospital and to hear their take on all this just made me take slower and deeper breaths.

The first two days in the hospital were quite a blur for me…for Parker they don’t even exist in his memory.  He can’t remember anything.  But I think that is probably a huge blessing for him.  With each day that followed there were more and more labs and tests. On the 5^th and final day, he had an endoscopy that showed gastritis (which he has had for years now) and the doc shared pictures of where his pills hit the atrium wall of the stomach and there are tons of burn marks and blisters. So his meds have been adjusted some. They also took 5 biopsies that all came back negative.  Best news ever.

Parker was so thrilled to come home.  Due to all this, it has impacted school and he still has one final exam to make up but amazingly he passed to 10^th grade.  This is truly a gift and was so needed after all he endured.  He had follow-up appointments with almost all his docs and mostly things are looking better.  He is our miracle kid.  He still has to still see a specialist for the dysautonomia.  We are setting that up, but in the meantime, the cardiologist has ordered weekly infusions of sodium chloride at home…this is one of the least invasion treatments for this, so we are proceeding with that.

For now, we are putting off his 2 minor surgeries but are still trying to get them done for prior to school starting.  In the meantime, the doc will do an MRI and get it all lined up for the neck nerve ablations and the nerve stimulator trial in his spine.  Parker still wants to have them done.  I just can’t imagine living in so much daily pain.

Parker is now enjoying the start of summer, resting a lot during the day, counting down the days for Camp Boggy Creek and anticipating the National Juvenile Arthritis Conference where he is an Ambassador of Hope.  Last night he went to the AF awards ceremony where he and Logan got an award for being the top family fundraising team.  Michael and I have all of you to thank for that amazing moment of pride.  It is because of all of you that our gratitude overflows.  You make it possible for our boys to go to conference and camp.  You make it possible for studies to be funded to push us closer and closer to a cure…and you instill HOPE in our children, so that they can continue to say YES to all the possibilities life has to offer.  Our family loves life a little more fully, a little more deeply, and lot more happily knowing that Parker pushed through this scary time, reminding us how precious life is.  Please hug and love on your children and family and keep on HOPING.

Living in the Moment…

We really try to live in the moment…yes, sounds cliché but it is true.  We’ve learned that living in the here and now is much easier than looking too far into the future of what’s to come.    And we put much faith in believing that we are doing the best we can with each moment…and that is all we can do.

We are rounding up the end of the school year.  Logan has been done for several weeks.  He graduates from high school with an AA because he did early admission into college his senior year.   He received one scholarship and is waiting on several others.  This fall, he will start his junior year at the University of Florida, as he moves towards his dream of becoming a pediatric rheumatologist.    We are incredibly proud of him and we are packing in as much fun as possible, with our grown-up kiddo,this summer.

Parker has missed much school this year because of his chronic illness, the pain it causes, and because of the major reconstructive surgery he endured.  He’s been going back to school part days as much as possible.  He is only two weeks away from the last day of school; and he is still unsure if he will be able to proceed to the 10^th grade.  This is causing him much stress even though we are stressing to him that he can only do his very best and we will support him moving forward no matter what happens.  As much as we try to explain to him that his health had to be taken care of first, it is very hard for a 15 year old to watch his peers move forward over and over…often leaving him behind (in many ways).  In the big scheme of life, we all know that his health is his “life”…but oh how it hurts our hearts  to know that he knows that his friends go “out on the town”, have dates, go to all kinds of fun events, play sports, drive cars, and do all kinds of fun “teen things”.

So we try to focus on the JOYS…Yes, Parker went through a very intense surgery on his legs, feet and ankles and it was way tough beyond words…but now he is walking and he is aligned and his pain in his feet and ankles have gone from a daily 10 plus, down to a 6.  And he doesn’t need his wheelchair much at all.  He even walked across the finish line at the walk, only 2 months after surgery.  And…Parker and Logan made their walk fundraising goal and raise over $10,000 with nearly $13,000 raised this year.

Some of you know, Parker has struggled with some heart issues.  His blood pressure is running low and his heart rate is running really high (even when at rest).  We have been working with all many of his specialists, along with is primary doc.  In fact, his primary doc has felt that he is so complicated that she would like him to see someone with more expertise coordinating multiple health issues.  So she has sent all his records to a pediatric critical chronic care specialist at our local hospital and we are waiting to get him in.  We have done our very best to try and get him the help he needs without the trauma of a hospital stay.  And the last month has been way intense with lots of tests and specialty visits. 

We have been working closely with his pediatric cardiologist and he really feels that this last surgery triggered his autonomic system to go haywire.  And although he sees this more often in teen females, he has seen this happen in a few others after surgery on limbs.  He feels that Parker has autonomic dysfunction or POTS.  His heart rate actually went over 200 right in the doctor’s office during his EKG…  The cardiologist said the heart monitor he wore confirmed his concerns and since then, he has worsened.  He is also having some GI issues, trouble swallowing, difficulty urinating, and sweats/chills.  So, we are trying to control the symptoms, while we try to figure out what hospital to take him to.  There are only a few in the US that deal with this. He said the wait time is usually 2-4 months to even get in and he will be there a minimum of 5 days.  Our choices are Mayo Clinic-Rochester, Cleveland Clinic, Children’s Hospital of Philadelphia, and NYU.  Vandy has one too but they really only treat adults.  So in the next 2 weeks, we are trying a few things and researching to figure out, given all his issues, which hospital would be the best.

In addition, we have been working on approval for 2 surgical procedures that he hopes to have this summer so that it doesn’t interfere with school.    One procedure is neck nerve ablations and the other is insertion of a nerve stimulator in his back for a trial.  The cardiologist feels that he can proceed with these procedures, but I have to admit, I do have my reservations.  But Parker has a ton of HOPE with what these procedures could do to alleviate his pain.  He dreams of getting off of pain meds and learning to drive.

On a joyful note, Parker's papallidemia is healed and so no more pressure on his optic nerves and they are almost completely restored.  This also means that the psuedo tumor is likely healed too!  Thank God!

Also, we are celebrating that Parker was nominated by his school for the Prudential Presidential Community Service Award and he won this year.  It was a complete surprise and he walked to the stage, in front of his cheering peers, to receive this amazing recognition that only the top 10% in the nation receive for their service.    

He is also thrilled that he got into Camp Boggy Creek for a week at summer camp.  This camp is really special to him and he really feels it is his heaven on earth.  This year, Michael’s work has a promotional fundraiser to send kids to camp too…I just love that they see the benefit of this amazing camp and what it does for kids with serious illness so that they can enjoy some serious fun!  We hope all of you have some serious fun planned this summer.

Parker is Getting Surgery in 2 Weeks

We finally made it to the pediatric orthopedic doc.  Yes…we have been putting it off, only because it didn’t seem to be “the most urgent need”.  We went, thinking that the doc was going to recommend “bracing” Parker’s left ankle.  We were definitely shocked to hear that Parker needs extensive surgery.

As Parker got off of the examining table for the doctor, his right foot hit the floor first and the doc immediately commented “Oh that right foot doesn't look too great”…but when that left one met the ground, he quickly shifted all focus to that left foot and ankle saying, “Oh he definitely needs surgery; this left one is way worse.”  Well, we knew it was worse but …gulp…surgery?!  He then went into great detail to explain that Parker needs surgery and for several reasons:  both ankles are collapsing towards the ground and the ligaments and tendons are not doing a good job holding things in place; his foot/toes are turning along with his tibia bones; and he felt that the issues with his feet, ankles, and legs could be contributing to the extreme pain he is having with his knees, hips, and spine. 

The solution, in two weeks, if all his docs clear him, he will be having surgery on both of his feet, ankles, and legs.  We had several docs recommend the doctor that we saw; and we are very glad that he is also the orthopedic surgeon; so it was a one stop shop.  He will be working on tendons, ligaments, and bones, making cuts in his bones to insert cadaver bones and pins with a goal of aligning both feet and ankles.   

Of course, Parker is not a straight up easy patient…not that this surgery is easy, but there are many things for his surgeon to consider.  The last time he had broken bones, it took 6 months to heal…much longer than usual.  He has full-on osteoporosis and of course juvenile arthritis.

His immune system is compromised due to his meds and also because he has hypogammaglobulinemia.  He has pulmonary obstruction and restriction.  And so, the surgeon is talking to his docs and he is going to use a bone stimulator to help with bone healing.  We are praying hard that his body won’t reject nor attack the foreign objects that they are about to insert to correct his feet, ankles, and legs.

He will have at least three days in the hospital; thank goodness this doctor is local.  He will then need to be out of school for at least a week.  The good news is the surgery is right before spring break…the bad news is recovery is during spring break…  But we are really trying to focus on the fact that he will not lose days at school since he is so close to going over 51% days missed at school.  But Michael, Logan, and I feel so sad for this kid.  It seems that every school break, he is struggling with something big, making it difficult to enjoy time off.  This means that Logan and Michael will be vacationing with our friends without us…so Parker and I welcome visitors to cheer him up.  Of course, call first.

Parker will have the first set of casts that cannot get wet, on both feet/legs for 4 weeks and he cannot bare weight on them either.  Then he will get a second round of casts that he will be able to put small amounts of weight on.  He should be able to return back to school, in a wheelchair, after spring break.

When I think back on last weekend at Camp Boggy Creek and how he danced and kept stopping due to pain, I just cannot imagine how this kid does it.  But he DANCED and he was HAPPY.  We love him so very much.

So here we go... on our newest journey...the journey to maintain walking and hopefully towards less pain.  Then, Parker, you can DANCE your little heart out!