Friday, July 29, 2011

More changes possible

I've been trying to write about this for a few days.  Since I kept getting an error on Facebook saying my account was down due to a site error, I waited until I could log into Facebook again.  Like a true addict jonsing for a fix, I think I checked my Facebook every 5 minutes for the majority of the 24 hours it was down.

Anyway, Tuesday was our first infusion day for Emily's new medicine, Rituxan.  Apparently, the first 2 doses are double doses, then in about 4-6 months a smaller booster dose is administered.  I guess someone forgot to tell the scheduling desk this; they scheduled us a 6 hour slot for an 8 hour dosage.  Oops!  I realize that our scheduling desk has been suffering from "growing pains" due to a staff change.  I have learned when staff changes to expect things like this to happen.  I can't say I wasn't surprised, but it takes a lot more than that to upset us.  I was also pleasantly surprised to find that we had been put on the doctor's schedule.  I knew that we were scheduled for next week, but apparently they added us in because I had called to let them know that we have weird things going on. 

I have been trying to figure out what the heck is going on with Emily's little body for months now.  On the high doses of Prednisone she was doing very well, except for getting sick more often than usual.  At first, I just assumed it was because she's on 3 immune suppressors.  (Now 4 since we've added Rituxan).  However, the more we've gone down on her Prednisone, the more changes we notice.  Aside from getting sick more often, specifically her asthma flaring, she started getting fevers the week before her Remicade was due.  I thought this was just because she was sick, but Kevin pointed out last month that she's probably been flaring.  I don't actually remember her complaining about her joints before, but the past 3 months she has.  (Thankfully her muscles have been quiet & under control.)  So, now what we have is a child who is on daily asthma controls, who is at high risk for pulminary hypertension and other systemic disease effects (since both MCTD & Dermatomyositis are systemic diseases), who is getting ill and flaring the week before her meds are due.  This past month her Remicade did NOTHING for her knee, and about 2 weeks in, her hip socket was causing more pain.  The hip only lasted about 2 days, but her knee pain didn't stop until we increased her Prednisone from 4.5mgs to 15mgs.  I don't like that, not at all.  But she was in enough pain to agree with it, and her asthma was acting up so it helped both.  This got me thinking.  How often was she sick because her Remicade was wearing off?  How much has the Prednisone masked what the Remicade has done?  And my answers were ones that I didn't particularly like.

I'm not one to panic anymore.  Yes, at the start of all of this I did a LOT of worrying.  We had a lot of diagnosis ruled in, then back out.  I realize that with autoimmune & rheumatic diseases, you just don't know until there is concrete proof.  Many things are not what they appear.  However, one night out of the blue it occurred to me: What if the Remicade isn't working as well because Remicade isn't meant for systemic patients?  What if this "asthma" isn't asthma, but possibly the start of systemic features?  Despite not wanting to make it real by putting it out there, I forced myself to ask the nurse practitioner if that could be. She had already arranged for a repeat pulminary function test at my request.  She explained that's why she agreed to the test, why they wanted to try the Rituxan, and why they increased the Prednisone.  We also increased her Symbicort strength from 80/4.5 to 160/4.5.  I think I'd feel more comfortable leaving her on the higher dose at this point, but we'll find out more next week.  She said the PFT can usually distinguish between asthma and other pulminary restrictions.  Good to know.  Depending on what the PFT shows, we may repeat her chest xray.  We haven't had one done since October.  By this point, it was already an hour into our time there and we still hadn't gotten into the infusion room.  I still had no idea how long the infusion time was for this med.

As soon as the nurse started looking to find a vein Emily got very upset.  Em knows that if she can't feel it, then the nurse can't either.  I prayed and prayed for one poke.  Nope.  Poke number 1 produced this:
  Needless to say, by this point Emily was really freaking out.  She was sobbing, yelling at us, seriously freaking out.  I can understand how she feels.  No one wants to go through all that she has to.  It really isn't fair at all.  However, by this time we're at a point where they have to hurry & find a vein or it will be too late to do the infusion.  Great.  We had a lovely conversation with a little girl across the way about her port.  Well, I had the conversation.  Emily sat there angry at me for talking about it.  I spent almost the whole time there talking to various people about the port.  I saw the doctor 3 times, the nurse practitioner 4 times, the Child Life specialist came to sit with us and bring us a book to take home about ports.  All of the regular nurses took turns talking about it.  One in particular teamed up with the nurse practitioner.  I ordered the referral to the pediatric surgeon during this conversation.   And later on, after the doctors and infusion room nurses went home and we went to an inpatient room for a few hours, our night nurse & I talked about it.  I think she's now heard enough to be ready.  She's certainly considering it.  She's told me 3-4 times now that she thinks it may be a good idea.  We're both tired of the stress and trauma associated with blown veins.   Before we left, the other nurse practitioner came in.  She was actually downstairs with her son at the orthodontist but she got sucked in to help us get Emily's Methotrexate dose there instead of me giving it that night at home.  (This is the nurse that many other people don't like, but she's always been really cool with me.  We even joked around a bit.)  While I had her there, I asked her the one question that I had forgotten to ask.  Many of the JA kids are systemic, which used to be called Still's Disease.  Still's classic symptoms are at least one fever spike daily and a specific type of rash.  I didn't think that Emily would ever have any of those symptoms with her MCTD, but I wanted to ask to be sure.  I'm happy to say that I don't need to try to remember to monitor her temp constantly.  She shouldn't have any rashes like Still's, either.  The practitioner that came in for the MTX orders also called the house the next day to make sure that we made it home safely.  How sweet is that?!?!?!

Em didn't sleep well that night after we finally got home around 12:30 A.M.  However, she slept in the car and in the hospital.  The next day she slept a lot, and today she's had a lot of rest.  Hopefully this med will help her to have more energy, to feel better.  We won't know for quite some time if it works, but we're certainly hopeful.   Next week we go for her Remicade & PFT.  We were given the choice to go back the week after or skip a week.  We will skip a week and go back for her next Rituxan after that.  Depending on how her immune system looks, she may also be given IViG to boost her system.  Hopefully we'll know more about what's happening on Wednesday. 


  1. My goodness Danielle, poor Emily just can't seem to catch a break!! I was shocked when I saw the picture of her arm. I tend to agree that it looks like it may be time for a port.

    I hope the Rituxan does the job and helps Emily have more energy.

    Big Gentle Hugs,

  2. Oh Danielle, as hard as all of this is on Emily, I mean of COURSE it is, I just can't help but picture you sitting there going through all of this as a Mother. My heart REACHES to you all the way from NC to FLA. This cannot be easy on you. Know that my heart and my prayers are with you my friend.

  3. I know that you both understand. Maybe in different ways, but you understand. Sometimes I think I'm still in denial a bit, but we'll just keep taking things day by day. Love you both!