A Day in the Life of a Child…Our Child

Friday, December 30, 2011...Tomorrow is the big day!  First infusion of IVIG.  Intravenous immunoglobulin (IVIG) is a blood product that is extracted from pooled human blood plasma from thousands of carefully screened individuals.  This therapy is being administered in hopes that it will help strengthen Parker's immune system and provide protection.  It may need to be repeated. 

As much as I prepared to “sleep” prior to our trip up to Shands Hospital, such was not the case.  I was in bed by 10:30…listened to my hubby and Logan sneak in quietly…11:45 still awake…then sweet slumber…only to see the glowing numbers on my clock at 1:00, 2:10, 3:17, 3:35, 4:08, 4:15, 4:25, 4:30, 4:35…up and in shower prior to the 4:45 alarm.  5:35 on the road.  7:40 nebulizing Parker in the car prior to heading up to infusion room.  8:00 vitals, port inserted and meds starting at 8:40.  The infusion room had only one other child (who happened to be a little guy with AML that we met during one of our hospital stays).  Smiles and waves exchanged as us moms looked sleepily at each other.  We got our coveted corner today, which is so nice since we will be here all day.  Two more kids arrive. And by 9:15 the first little guy is already leaving…

All is still and quiet in infusion room and I watch the sunlight peek through the window blinds.  So thankful for the quiet (before the storm)... So often we enter to screams of the young children protesting their IV insertion for fear it will not work the first time and the nurses will have to dig and prod for their sweet little vein.  Parker’s sweet little buddy, Sadie, was with us when we were here two weeks ago and it took 4 tries to get her IV started.  Pure torture to hear those pleading cries asking if they could see red in the tubie (meaning blood return, good vein).  This is why we got a port for Parker several months ago…to end the torture every two weeks.  And as scary as a port can feel and as much as it makes us feel like we are in this for the lonnnnngggg haul, it was probably one of the best decisions we have made during this journey.  His veins were done and even when they could get it the darn scar tissue would bend the IV cath and push it out.

9:30 pre-meds infusions completed (acetaminophen, zofran, solumedrol, benedryl)…now the actemra is coming on board.  Two weeks ago, after 9 months of actemra at 500mg -10ml/kilo, Parker was increased to 600 (this is the max he can get). He is also still getting enbrel each week in addition to the actemra.  Even though he was very sick these last two weeks with an upper respiratory infection and needing to be nebulized, these were the first 2 weeks we didn’t see any systemic rashes nor fevers.  This was huge.  Don’t get me wrong…he still has multiple symptoms:  visible vasculitis on hips, thighs, back of knees, ankles and feet; rough skin on face; minor peeling at finger tips; very pale; swelling and intense  pain (10) at neck, lower back, hips (sure sign he is sick), ankles, toes, wrists, fingers, jaw, chest/ribs;  and the pink finger tips all persisted.  All signs that his disease is hiding out…BUT, we are celebrating that he had no fevers or systemic rashes…we must celebrate…that is the longest stretch he has ever gone with those major systemic features gone since onset in 2008.  So we continue to HOPE…

This disease if really hard…it sometimes feels very aggressive and as with many diseases and disabilities there is a “spectrum” of severity.  My husband and I have been processing lots of information these last two weeks.  It is really hard to admit how severe Parker’s JA truly is... but it’s pretty severe.  As hard as we fight it, it keeps fighting right back.  It’s almost like it out-smarts the drugs.  And the drugs…there are sooooo many drugs (I think we are at around 29 or 30)…and some of the drugs are for drugs.  And then there is the worry of what exactly are the long-term effects of these many drugs that the medical field is still trying to understand.  And it’s interesting how some people judge us (and other families of kids with JA) and our decisions around these meds…but if they understood the alternative and if this was happening to their child, I am absolutely sure they would do whatever they could to stop the progression of this disease.  And it’s important that we aggressively treat.  Part of the reason Parker has been so difficult is because he wasn’t aggressively treated in the beginning and the disease has had somewhat of a “snowball” effect on his little body.

So, just to get everyone up to speed who has just begun to follow Parker’s journey.  Parker has not only systemic onset of juvenile arthritis, but he also has overlap syndrome.  When trying to find info on this, there really isn’t much out there.  So two weeks ago, I had a long talk with Parker’s doctor about this.  She said to look up mixed connective tissue disease because it’s very much like that disease, but to have MCTD there is one specific marker in the blood that makes the difference.  Otherwise the symptoms and issues are much alike.  For Parker, he seems to have bits a pieces of variety of autoimmune disease, thus this is another reason why he has been so difficult to treat.  Parker presents with severe allergies/asthma, myositis, vasculitis, and some psoriatic JA-like features in addition to having systemic JA.  And there is also the concern about the frailty of his immune system.

We have been given a few new options and are likely to combine some treatments.  First, we have already increased his actemra (IL- 6 inhibiter) and added enbrel (TNF blocker) to the mix.  Now there is the addition of the IVIG in hopes to build up his immune system…his diseases and the multiple immune suppressing medications have influenced this decision.  We also discussed using anakinera (kineret).  This is an IL-1 inhibiter.  Typically this is a shot that is given daily, however they are trying to set up home health care to come in and teach Michael and I how to infuse this daily through Parker’s port (another huge blessing that he has this port because as a shot, kineret burns worse than any drug out there and has to be given daily).  Finally, the doc talked to me again about Parker taking part in the RAPPORT study.  The drug that they are testing is also an IL-1 inhibitor called rilonocet, but is only a weekly shot verses daily.  There was concern though if he would even qualify now that he has overlap syndrome.  He would qualify with systemic JA.  However, my husband and I are VERY concerned about the risk we might be taking if he did this study.  One, we know that he has had some issues already with his internal organs (lungs, lymphs, stomach, and heart)…there is concern that if he got the placebo then we would be putting these organs at great risk and we have spoken with our insurance company and they would NOT cover any complications due to the study and he may even lose coverage.  That is a risk we feel we cannot take.  The study is in its last 6 months of trials.  So, we will wait and if the drug gets approved in the future then we may try it later.  For now, we will see what the IVIG does and hope it works well with his other meds.

10:40 – Actemra is officially done, now methotrexate is going in.  So nice to have one med given here so we don’t have to give it at home every other week!  Methotrexate makes Parker incredibly nauseous and although he gets zofran at home with it …it is just not as good as getting IV zofran.  And today he will get an extra dose of the zofran prior to leaving due to the IVIG. 

11:05 – It’s time…IVIG…praying…hoping….For all the other infusion room buddies out there…the infusion room symphony has begun…hum of multiple TVs/video games, mumbling voices, shuffling feet, metal swish of curtains opening and closing on their tracks, moms “hushing “, synchronized beeps of IV pumps (those beeps enter in my dreams), and the little ones crying…This is why Parker loves his headphones.

11:30 – I lean in and ask Parker how he is doing and he pushes me away…not wanting to be touch nor spoken too…”Just leave me alone.” 

1:15 – Friends arrive and we get our first brief smile of the day J  and then sleep…after a break in the IVIG to administer more benedryl and toradol.  4:00 - IVIG done, saline push, and more zofran on board for the ride home.  Got lab results prior to leaving …and looks like the doc made a great decision in administering IVIG because both his IgG and IgA levels were significantly low.  Here’s HOPING…again…  So Parker could have a severe headache for the next two or three days.  For the next 48 hours we are to give him acetaminophen and benedryl every 6 hours.  5:00 finally back in the car heading to home sweet home.