Monday, July 18, 2011

A few changes

Before we headed off to the JA Conference, we had to stop at our hospital for Emily's infusion and doctor appointment.  I expected a few questions to be answered, a quick appointment and quick poke since she's done so well lately.  Nope. 

The first poke by her favorite vampire, Jason,  got them absolutely nowhere.  That was enough to bring out the tears.  He called in Ariel who thought she had it, but didn't.  They tried the 2nd arm to no avail.  They decided we should use a little more Emla cream and hit up her hand.  She hates that.  They tried and again failed.  We discussed what would happen if we skipped it, which I really didn't want to do.  That would just mean going through all of this again in 2 weeks.  Finally, a nurse helping in the ped's room got it.  Thank God!  That brought the doctor around asking us if we had considered a port.  Well, in truth, I had.  I had been waiting for them to bring it up.  I have my mind made up, but Emily is harder to convince.  I think it will take her a few more months to grudgingly accept.  I hate having to go through the stress, tears, the trauma of having her poked monthly.  To me, it's an easy answer, but since I want her to feel that she has some control over her health, we will wait a bit.

The other thing that I really wasn't expecting was for her to bring up another med.  This really stressed me out a bit at first.  Thinking about picking a new "poison" is always scary, but there aren't very many options sometimes.  Emily has been very tired and her joints are flaring badly a week or so before the next infusion.  She's also been sick a lot.  Our doc suggested that we try Rituxin.  Rituxin kills the "B" cells.  These cells are cancer causing, so it would decrease her risk of cancer, but they also kill off the immune system even more than her other 3 immune suppressors.  To address that little side effect, they will also give her regular IViG.  I'm a big fan of this med.  It's actually taking the platelets of the blood of 2,000 donors, mixing it together, sterilizing, and voila!  You have an organic med that will help to confuse an over-zealous immune system.  I'm for it.  I realize that some people get debilitating headaches from it, but I also know that there are different brands, and if you can try a brand, you will likely find a way around side effects.  Our docs are big on giving IV steroids (Solu-Medrol) with each infusion.  This helps to eliminate side effects.

Needless to say, I was thrilled to get to the JA Conference and talk about ports and Rituxin.  I was a little sad that the Rituxin reps weren't there; I'm assuming this is because it's not actually FDA approved for young children yet, but we're pretty much out of options.  I did, however, have the opportunity to meet and speak to several parents and pediatric rheumatologists about this, as well as get some input from parents in my Juvenile Arthritis Friends group and my JM Moms groups.  The general consensus?  We're going to try it.  I'm not sure what else to do now, to be honest.  Right now, Emily is definitely doing better than some of the kids, but she can't play.  She isn't in agony all of the time like some of the systemic kids, but she does have a lot of discomfort and she's definitely got pain daily.   Her quality of life right now is far from ideal.  Her endurance is very low.  Yes, I still consider us blessed!!!  While MCTD is far from ideal, I'm happy that she doesn't have a terminal disease. 

Since Emily developed the Dermatomyositis on top of the Mixed Connective Tissue Disease, I've often wondered is this what they refer to as "myositis features" that comes with MCTD, or is this its own, primary disease.  I was able to ask 2 doctors this.  I have always felt that, while she had myositis features before (I think from looking at her labs) I truly felt that the DM manifested as a primary disease.  She had a high fever for almost 2 weeks, the rash developed around this time, her weakness was very severe; she couldn't roll over in bed.  Usually with MCTD the patient would have just features, not the whole disease.  Everyone agreed that with an onset like that it is a primary disease.  I really wanted it to be part of the MCTD, but I feel it's important to be honest with ones self.  I also feel that as long as I know the truth, we can find help.  While it wasn't something I wanted, it's good to know.  3 kids in a million have DM.  The odds are similar for MCTD, though I don't know actual figures.  That is really like winning a bad genetic lottery.

We learned a LOT from the conference!  The best part was feeling like we were at home.  I love my FB family!  I have met these people on Facebook, but we all come together because we understand the situations that others have in a way that outsiders couldn't possibly understand.  It was so cool to meet so many amazing people in person... and feel like I've known them forever!  The conference helped my oldest to better understand things, too.  It was really a wonderful weekend.  It's great to swap stories, talk medicine, and ask whatever burning questions you have.  The sessions are set so that newcomers aren't totally confused, but there's enough content to keep a knowledgeable family's interest.  We truly appreciated this special opportunity.  It will probably be the last trip that we take for quite some time, so we didn't take any of it for granted!   

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