Saturday, December 31, 2011
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.
Tuesday, December 13, 2011
My thoughts are all over the place these last few days…I have determination, love, family, faith, hope, resilience all at the same time as feeling incredibly frustrated at the fact that no matter how positive I try to be my son is still very sick. We follow directions. We pray. We provide. We chart. We problem solve. We celebrate simplicity. We marvel at the good will of our friends. Yet, we still have a very sick child. No matter what we do, his disease continues to persist. We have very short moments of success only to be faced, yet again, with set-backs.
Each morning, Parker wakes and we assess only to come to the realization that he is not able to go to school. He wants to be at school more than anything. He really loves his service learning class and art because he says he can be himself in those classes and express himself. He misses his friends. It’s breaking my heart over and over. He is so very far behind and yet, when he is awake enough and able to breathe deeply through his continuous pain, he does what work he can and makes fantastic grades on the work he completes. There is just so much work. He has an Individualized Education Plan with an altered schedule and workload for the days he goes to school. But he also takes two virtual classes and that load is not reduced at all. It’s just done at his pace…which is s-l-o-w…We have not been given the same options around home schooling that other families around the country have had. Our one and only option is to take him out of his advanced classes and do all regular ed…and are you ready for this…all classes are at set times and over the phone with a class of many other children who are also on the phone. Not the best of option for a child who needs to often dictate, needs to sleep lots, and learns best visually (not auditory). Plus if we do any form of home bound, he loses his spot at his school, because he is in a charter school. So he would not have his school community nor friends any longer. Can you tell that school weighs very heavily on our minds? Parker is a very social child and our fear is that if he didn’t belong to a school community he will lose his drive to fight this disease.
Then there is the medical …where do I start. Something medical is happening every week and usually more than one event takes place each week, sometimes multiple in a day. Parker has weekly occupational therapy (OT) and physical therapy (PT). And If I reflect on just this week and last week…let’s see…multiple shots and meds, blood work, an infusion treatment (every other Fri), gastro appointment, counseling, and oh…we got his new wheelchair! That is the biggest highlight. He has also been fighting a toe infection (5 weeks now), parvovirus (2 times in a row), and he wears wrist and ankle splints nightly. And as draining as all this is, we would do all of it and more to find a cure.
A cure…our constant hope is a cure…today I feel like a cure is far far away. Today, I feel like we have to accept what feels unacceptable. Parker’s in 10 pain again…has been for 2 weeks. And now he has overlapping syndrome…whatever that means because I can’t find any info of use on this. He has joints swelling and they arehot again…in fact his neck is so flaring that the temperature on his neck today was 101 degrees…but his forehead was 97 (he is usually 95 or 96). And his fingers and toes are peeling…yet again…and still don’t know why that happens. The last 2 times his toes and feet peeled he ended up in the hospital…so yes, we are scared. We just want to understand this disease…we want to know the connections between all these crazy symptoms and what to expect…but all we seem to be able to expect is the unexpected…there is no predicting. This disease has not been easy, even his doc says that…and we hear that he is flaring either because he got sick (he gets sick a lot) or because of the weather (whatever) or because of school starting or doing too much (but he doesn’t go much nor does he do much)…Parker is a fighter, but so is this darn systemic JA…it’s one big bully and we don’t like that big bad Arthur! Parker is scared again.
Saturday, December 3, 2011
Balance…when you look up this word you see synonyms and definitions such as: equilibrium, steadiness, stability, poise, assess, weigh, compare, evaluate, consider…
I am someone who tries to maintain my “sense of balance” in both my professional and personal life. I strive for balance because it helps be “feel” stable. When I am “balanced” I am happier, can achieve more, and am better connected with my loved ones. When I am balanced, I can easily “give to others”, which I love to do.
If you know me well, you know that I also try to insert a balance between the attention I give to my children and what I expect from my children. Balancing my affection, chores, time, and family contributions, etc…
And then there is a balance with my husband…Balancing our time together, tasks, and parenting.
I think, for the most part, our family was pretty balanced prior to our son’s on-set of his systemic juvenile arthritis. I am thankful for that because I believe that our ability to find balance has truly blessed our family with the ability to cope, comprehend, problem solve, and continue to love. But it has also allowed us to ride the waves…
My life is no longer balanced. Our family life is no longer balanced…at least not in the sense of what our family use to be. And certainly not who “I” use to be. I have known this for some time, but I really REALIZED this, this week. There is one person in my life that, unknown to her, has helped me realize this reality because she touches my heart professionally, as a friend, and as family. When I process aloud with her I cannot stay balanced. Even in typing this and recalling our interactions, I am drawn to tears. She knows me fully, through all aspects of my life… she knows that I can ride the waves but for some reason, when I am with her, the wave comes crashing down with a huge splash and disperses and I temporarily lose my balance. She can see the storm in my life…and I have trouble focusing on just one aspect at a time…probably because each decision influences how I remount that wave and balance.
I do feel like we are on the right track with Parker’s treatment. For the first time, we have seen powerful progress…but I was reminded this week how quick things can change. And I am keenly aware of how fragile he is. We had a set-back this week and it really hit me hard. I panicked…right in front of my dear friend. I had to walk away to the privacy of my car and I lost it. Completely and fully lost it. I have been trying so hard to grab onto our moment of progress…to stay positive and optimistic…Parker’s pain had decreased, his enthusiasm in his voice had returned, his attendance at school had increased, he was catching up with homework, he was moving better and we were balanced again. My husband and I were getting tasks done, we were connecting with each other, we were catching up, and able to focus better on work. Logan was chitter chattery, sharing about school, dancing around the living room, talking about his girlfriend… We could all spend more time with each other, and he loved seeing his brother feeling better. All of us riding the wave together and fully enjoying our equilibrium, steadiness, stability, poise, and ability to assess, weigh, compare, evaluate, consider… and oh, did we begin to “consider”…
But rather than riding the kind of wave that slowly begins to roll into other waves and eventually becomes that nice calm bobble where the sunlight bounces joyfully…we crashed. I crashed. And suddenly I was drowning in my emotions. Parker’s pain was increasing, his hips and knees were flaring (these joints only do when bad stuff is about to happen) and intense pain increased in his usual joints…ankles, back, neck, wrists, shoulders, hands, and wrists. And the fatigue was back with a vengeance. He was back to missing school. And yet, we still went to OT and PT this week and both therapists could see the change. And as the PT was working with him, he started a marble like pattern all over his body. It was so bright and intense that she actually stopped her therapy and sent us home. He then got a red rash on his cheek and chin and slowly the rash and marbleing went away. He was left with a rough feeling on his skin across his chin line (nothing you can see, but it feels like a man’s rough beard and is still there). This hit me hard because he had his enbrel, but he was not bouncing back like the weeks prior. All I could think was his disease was going systemically through his body again. I was scared. And I think that Logan was scared too…he got a migraine this week, as all this was happening.
Parker had his infusion yesterday and we met with the doctor and nurse practitioner. They threw me a life preserver, as they explained that they really felt like Parker got an infection and most likely it was parvovirus (a.k.a. fifths disease). It was not his disease going haywire again but rather being triggered by the disease. I was so very relieved. I know that might sound crazy but I was...because what it meant is that we were still on the right track with his meds. I did ask if he should even be going to school and they said yes. But they are carefully tracking his blood. If his counts go below 500, then they will immediately let me know, so that we can keep him home. He needs school…it’s his “balance”…
But we also discussed some other things…they felt that the Actemra (IL-6 inhibiter) has done a pretty decent job with some of his systemic symptoms and issues. It has lessened his rashes and fevers. His blood work also looks tons better. It has also helped with his horrible fatigue. However, it has not done a good job with controlling the inflammation and pain; thus this is the reason for the Enbrel (a TNF blocker). So because we have done a good job at stopping the IL-6 pathway, the systemic JA (a very smart disease by the way), started revving up and going down that TNF pathway even harder. By blocking that pathway too, we are finally seeing the swelling and pain decrease. However, both these biologics compromise his immune system. The doc shared yesterday some new news that once again has begun to boggle my mind. She thinks that Parker is starting to have (or has had) overlapping syndrome…I am still trying to comprehend…but he may have more than one JA disease…they pointed out that he has symptoms of other JA diseases in addition to systemic…like the myositis (weaking of the muscle enzymes)and the psoriatic issues (nail pitting/lines and possibly now the roughness in his skin). I don’t know where this particular wave will take us but we are adjusting meds again…
So I go back to my sense of balance…my work is definitely not balanced right now, in fact I am having to decide what do I REALLY want to do, while keeping in mind that my choices will affect the balance in my other parts of my life. And balance between my children definitely doesn’t mean equality but rather that we, my husband and I, are present and connected and helping them when they each need it most. I know some probably think I should stop working. I have gone part-time, but I need to work. For several reasons: one, for our health insurance; two, for the income to pay for medical expenses; and three, for my “sense of self” and to give back to my community. But I do have some choices to make…
So, I reflect today…And I thank my friend, my colleague and my “family” for allowing me to just “be” in her presence… I was drowning a bit but today I have surfaced and I am riding a new wave of HOPE…As I get better and better at “balancing”.