Snowballs in summer

Why snowballs in summer, you ask?  Today it was 98F at 3:30 this afternoon in the northern part of the state.  I discovered this after about two hours of a doctor's appointment for Emily that left me feeling like someone had thrown snowballs at me a few times. 

It started out simply enough.  We drove 138 miles (one way) to the new ENT's office for a new culture of Emily's sinuses.  I really didn't know what to expect.  I assumed that he would look at her, take a history and have us come back for the culture.  First a resident came in and took the info.  I gave him my list of her meds, along with some of the bottles because they ask for them.  Because I'm learned how to be pretty prepared now, I had printed out her CVS med history from November to now so that they could see all of the antibiotics that she has been on since November.  I even highlighted them.  We discussed history, her pneumonia in 2007, the somewhat frequent bouts of bronchitis that she gets, and of course this stupid sinus infection from hell.  The resident walked out to confer with the primary doc, and then they both came in to see us together.  The first question that the main doctor asked me after a brief introduction was, "has she ever been tested for Cystic Fibrosis?"  I think my jaw probably hit the floor.  I have always thought of that as something that starts from birth and can kill you pretty fast.  Of course, times have changed and those affected are now living much longer, but also there are apparently different strains.  Some people are very affected while others have milder forms.  It took me a few minutes to mull this over.  We're not sure what to make of this now.  She does have several of their symptoms- quite a few- but not so much the chronic cough that I have always associated with CF.  Also, they say that most people that are tested do not have CF, so that helps me to feel better.  If you'd like to read more, I have found this handy PubMed article on Cystic Fibrosis.

They asked me if she had ever been scratch tested, or if anyone had brought it up.  When I said no they said that they assumed not because (apparently) people with Dermatomyositis cannot be scratch tested?  Good to know!  I had no idea!!!  That kind of hit home that, no matter how much research I do, I will always miss something.  (Unless I go to medical school!!!  :)

The next thing that got me was Emily's reaction.  They asked her if they could put ONE DROP of Lidocaine into her nose to be able to do the culture.  Nope.  I begged, I pleaded, I tried to rationalize.  The doctor's left the room and came back TWICE to give her time to collect herself.  We really needed that culture.  But she just wouldn't do it.  She was so upset and I was so frustrated! 

What they would have done was taken a little cotton scraping of her sinuses, then checked for bacterial growth.  Upon finding bacteria they would have tested it with different antibiotics to see which one would eliminate it best.  Pretty important, huh? 

Instead of the culture, we are now scheduled for sinus CT on May 21st at 8 A.M., a follow-up ENT appointment at 11 A.M., and then... IF she's not sick... (HA!) we will be at the hospital from 12:30 until sometime the next day.  Somewhere in the middle of all this we are supposed to go back up there for the Cystic Fibrosis (CF) test, the sweat test.  They are supposed to call me tomorrow to schedule the CF test, so I will likely point out to them that if she is sick on the 21st she can't get her meds.  We'll see then if they want to change the appointment.  I didn't even think about that until we got home.  Boy, was I ticked!!!  Not at the doctor's.  They did all that they could and they had the utmost patience.  It was just everything else. 

So now I am pretty certain that she is going to be sick for the next 3 weeks.  I am working on going to part-time at work working weekends only, but we need to get more people in first, so I can't take time off right now.  She's simply going to have to deal with her decision.  Maybe it will help her to make better decisions in the future.  It's just hard because it could have been so easy. 

The funny thing is that, between her and Zachary, I have always worried more about Zach with respiratory problems.  If she's positive we'll definitely have to have him tested. 

We are getting closer and closer to all set for home schooling.  Ya'll have no idea how huge that will be for me, for the kids.  I cannot keep running ragged between work, PT/OT, trips to the hospital and not having any time to make phone calls and set things up that I need to.  I think this will be the answer to a lot of prayers.  I know it will be a lot of work, but we all really need it!

The happy point of the day was finding out that her Medic Alert bracelet and kit came in!  They have it engraved with "Bard Port Implanted Port, Connective Tissue Disease, Raynaud's".  It's perfect.  I'm so glad that I finally did it.  It's been a long time coming.  And with that, I have to head to bed.  I have to go crazy at work tomorrow, then grab the kids from school for Em's therapy sessions.  Fun, fun!  Have a great day!

Our new plan!

Ok, so again I am completely exhausted.  Forgive me if this doesn't make any sense, but I want to get it out before I forget it all.  We had another crazy day.  Hospital time runs at a totally different speed than real time.  It really seemed like I woke up, blinked, and the day was gone.  I didn't even get time to just chill out on Facebook.  Emily got quite a bit done.  She was up early, got some TV time in, went to the playroom for a while, found a book to read that she enjoyed, played some video games, made some creations and watched a couple of movies.

I started my day by running 2 toys that were given to us to take to the infusion room.  I figured that while I was there I would get a copy of her labs.  I figured that they may be off a little, but I didn't expect them to be completely out of whack.  I was shocked at how many exclamation points there were.  (They indicate levels out of range.)  Her inflammatory markers are elevated, but with an infection that is to be expected.  Her urine was all messed up, but they said that looked more like a contaminated sample.  (Phew!)  Her liver enzymes (used to measure her muscle activity) look fantastic!  Those running amok is one of my biggest fears.  Almost everything else was off, though.  That worried me, of course.  I patiently waited for a few hours to see a doctor.  Praise the Lord, we have been through enough of this to not worry just because something looks like it may be a problem.  Problems usually seem to not be so glaringly obvious with us.

Towards the end of the day one of the doctors came in.  Before I go further, let me say that one thing that I love about our rheumy group is that they don't just treat what looks rheumatological and hope someone else will deal with the "other stuff".  Dr. E asked the Infectious Disease doctor to come talk to us.  Let me tell you- I was soooo excited to see her.  Dr. E had told Dr. V that she had ordered the Rocephin antibiotic because it's very good for sinus infections.  We explained once again how the past few months have gone, listing each antibiotic and the amount of time prescribed.  We established that I need to be more firm with these other doctors when it comes to prescribing antibiotics for an actual infection.  I admit, that's not something that I'm good at.  I feel that, while I do probably know more about rheumatology than they do, it's not my place to tell them how to do their jobs, and while I should have a medical degree, I don't actually.  We talked about that for a while.  She said that she would never prescribe less than a 21 day course of antibiotics for anyone for a sinus infection- never mind an immune suppressed child!    I will now be more firm on that. 

Despite me asking the ENT's to send over her file, despite me signing paperwork on Monday for them to do just that, they haven't done it yet.  Dr. E had her nurse practitioner working on it, too.  That said, they could only rely on what I was told by the ENT's from the first culture, which was that it was weak to Augmentin.  Knowing that Augmentin was the only antibiotic that seemed to do anything (except for a few days before her scraping when it seemed to stop working) they prescribed a 21 day course.  They also want us to keep doing IViG monthly to boost her immune system back up and then maintain it.  Yesterday's labwork showed her Igg at 435.  They usually do IViG for levels that fall below 500.  I feel a huge sense of relief with this. 

As I said in yesterday's post, we will wait two weeks to resume her Methotrexate, and then it will be at a lowered dose to begin with.  She's one of the few kids that loves her Methotrexate because it helps her to not be so itchy.  Dermatomyositis can cause severe itchiness, and even though hers is much better now than it was since her muscles aren't flaring, it's still annoying.  I'm more excited about the Prednisone decrease!!!  Again, a drop from 4.5mgs (1.5mls)  to 3mgs (1ml) is huge!!!  She'll have two weeks on 1.25mls, but that's ok.  It's been so long since she's had a decrease.  I realize that the slower the decrease the less chance of flaring so I wasn't complaining, but perhaps on the new dose she'll be able to lose some more water weight.  She is still so self-conscious.  And that should help her growth to get back to a more normal rate.

Last but not least, we had a roommate this time.  Bless her heart, the girl has Crohn's Disease.  She was given the option between trying Remicade via IV or trying a feeding tube at night with formula that would help to heal her colon.  She really thought that the tube would be a good idea.  That poor girl cried and cried, begging her mom to take it out.  Before we left she decided that she would try Remicade- despite having a huge fear of needles.  We were able to help them feel more confident about their decision, and give them some idea on what to expect.  God puts people where they need to be.  I truly think that we may have helped them a bit tonight.  I hope so!!!  I am hoping that Emily feels much better in the morning, too!

*Written at 12:04 A.M.*

Rheumy visit & overnight stay

Well, I feel as though we’re on the right path.  Today we set out early to bring Zachary to school and head to the hospital for Emily’s infusions and doctor appointment.   On our way up the ENT’s called twice to see how Em was doing from yesterday’s procedure.  I asked them to please send copies of everything up here.  Last I knew, that hadn’t happened yet, but I have them working on it here now, too.

I was really hoping that we would see the main doctor today.  I thought it was time to get the many years of experience in to see her.  I was so happy that Dr. E was here today!  She seems so much more relaxed since the new doc was added to her practice.  We went through the whole story again, discussing cultures and the new jaw pain.  As the ENT said that he didn’t think it was from his side, our rheumy thinks that it is.  She played with the jaw a bit and she doesn’t feel any problems with the joints in her jaw.  But she didn’t like the whole “wait to see if there’s an infection” idea, either.  While we’re here, she figured that she would prescribe ROCEPHIN just to be on the safe side.  If the ENT’s had faxed over the sensitivities from last culture we would have a better idea, but it is what it is.  I have to try to remember that these “extra” doctors don’t care the same way, or realize how huge a deal Emily’s conditions and immune suppression are.  I’m happy to have her on an antibiotic.  While I realize that she can’t stay on one forever, I truly believe that she needs one now.

We discussed things like NSAID’s, and how she hasn’t been on one for quite some time.  She’s feeling yucky enough to want to try her hand at Mobic again.  So Dr. E put in a new prescription for Mobic, and they gave her some Singulair here.  We talked about her Methotrexate.  Because Emily gets so itchy and MTX is the only thing that seems to touch it, she is probably the only kid that wants her MTX.  We decided to wait a week or two and then try it at a lower dose.  If she doesn’t get worse after two weeks then we can try her normal dose again.  We were also given the ok to go down on her Prednisone!!!  WOOT!!!  I have been wanting to ask for this, but I know there’s a schedule…  At the moment she’s been on 1.5mls (4.5 mgs).  We are going to go down to 1.25ml for a week, then down to 1ml!  That is huge!!!  I will definitely take 3mgs to 4.5mgs any day.  I was very, very happy about this. And they're going to look into helping us get a hospital bed for her because she has a hard time getting up and she likes to sleep in an almost sitting position.

Tonight’s IViG to boost her immune system was started late so we’ll probably be here until 2-3 tomorrow, but the longer the better.  They are also using a higher dose than they had originally planned to give her a boost.  I’m happy with that.  So is she.  Emily had actually asked me if she could do IViG.  She is so tired of being sick, and I am so tired of worrying about the “what if’s” of her being sick.

While we were in the infusion room we had a med student come talk to us.  She is doing a paper for school about communication between doctors and parents.  I was pretty excited about this, actually.  There are so many things that they don’t teach in medical school that would be a tremendous benefit to doctors.   I think that, like parents, the doctors get conditioned to thinking of little things like surgery as just that- a little thing.  I was thinking about how when we first met our ENT’s with Gir’s ear infections and later with Emily’s adenoidectomy, he so casually declared that she was going to need surgery, have a nice day.  At the time I was devastated.  Now… sometimes surgeries are welcome.  If it helps to change the problem, if it’s the only way… then ok.  But as a parent just thrown into this world, you’re totally overwhelmed.  So we talked about that, relationships with medical personnel, how Em’s diseases have affected us if different ways, and so on.  It was nice, actually.  I had planned on just chillin’ out, catching up on Facebook and maybe mindlessly clicking on some game things.  Real conversation was much better. 

So it was a strange day filled with ups and downs.  I’m so glad that we’re here tonight.  As crazy as that sounds, my hope is that the ENT will call with culture results tomorrow and we’ll have been able to have a plan in place with Dr. E before we leave.  That would rock!  For now, I’m so exhausted that I don’t even really remember what I wrote and re-reading isn’t helping, so I think I’m going to mindlessly click some stuff and head to bed.  I want to thank those of you that are praying for us through this time.  I appreciate every single prayer.  Thank you!

Em's surgery & a sick boy

Well, for those not following the saga on Facebook, we finally got to a point of panic with Emily's sinus infection.  Her surgery date was February 22, and with every day that passed by it became more and more clear that she would not make it that far ahead.  The Septra that her pediatrician had called in was doing nothing but making her worse.  I took her back to the pedi on Monday, panicking because I just didn't know what to do.  How could she continue to go to school, how could I get to work every day, if she felt so ill? 16 days was a long wait.  So I brought her to the pedi, hoping that she would prescribe Clindamycin since that had worked pretty well the first time that she used it.  She didn't like that idea.  Apparently, Clinda can be rough on the joints, but also it can lead to an increased risk of C. difficile (Clostridium difficile- a bacterium that can cause severe diarrhea that doesn't improve and can be contagious.)  Awesome.  So we left with another prescription for Augmentin, which had stopped working 2 weeks before, and a low maintenance dose of Septra, which also wasn't working.  To say that I had little faith in this plan was akin to saying that a shot of Prozack could fix it.  I just knew better.  I have come to this point where I just KNOW things.  It may make no sense, but I'm usually correct, and it usually isn't good.  My intuition with medical is extremely accurate.  Imagine how panicked I was.  So, my brain working furiously, I'm thinking "well, maybe if I go to the Children's hospital I can get an antibiotic IV for her".  I didn't want to undermine our doctor though, so I called her to see if that would be a better idea.  Not liking her answer, I called our ENT.  I asked them the same question, and I asked about their cancellation list.  I begged them for anything that I could do to keep her going until the 22nd', explaining the situation.  When you have a child on 4 immune suppressors, it tends to panic the doctor's sometimes.

About 2 hours after my conversation with the office, they asked me if I could bring her in on the 9th.  HECK YEAH!  I would find a way to make it work.  So she had her surgery yesterday around 2 P.M.  She was so excited about the prospect of surgery.  You know you have a very sick child when she wants to have surgery.  My brave little girl didn't utter the "I'm kinda scared" until we were on the way there.  And even then, she was still happy about it.  The only crimp that we had was that they didn't have a way to access her port.  They had to give her an IV in her hand, which she really hates.  Fortunately, she was warned and they do it under sedation, but she still cried about it.  The thought of an IV in her hand was more scary than surgery, and she's had surgeries before!

Today she is resting semi-comfortably at home.  She is annoyed at all of the drainage, but that's because her sinuses were much worse than what the CT scans showed.  The doctor was shocked at how badly her sinues were packed, and he said that no antibiotics could have cleared that out without help.  In other words, it's a damn good thing we got her in when we did.  So is a little upset because she can't breathe out of her nose, she said she still feels sick, but at least we're on the right track now.  The ENT said that we should have the culture results back by Monday.  That will tell us which antibiotic will help her now that the crap is all out of there.

And my son is a little sick.  Not like that bad, but sick.  His last antibiotic didn't clear his infection completely.  He has some residual drainage also, and his asthma is acting up a bit.  She prescribed Augmentin for him, and I'm going to withhold his Enbrel and Methotrexate this week.  I hate to do that, but I would really hate for him to go through what his sister is going through more.  Take one step forward, two steps back.  All in all, I think we're on the right course.  At least there is a plan in place and things in motion.

ENT and Enbrel!

Ok, so just so everyone can see the crazy, up/down days that we have, I will post a breakdown of the day.  This is why we are so used to dealing with stress.

8 A.M.- The kids don't want to get up (and neither do I!) but we get up & ready to go.

9:30 A.M.- We leave and take Zachary to school.

10:20 A.M.- We arrive at the ENT.  We wait for about an hour, which is fine because we were so early.

11:20 A.M.- We get set up with the nurse.  Being a mom now accustomed to the things that doctors need I have a typewritten list of meds that Emily is currently on, a print out of all prescriptions taken since the start of the infection with the antibiotics highlighted in yellow, along with CT scans and the radiology reports.

11:30 A.M.-  The doc comes in with the scans.  He starts showing us how the sinuses should look and how Emily's are not performing where they should be.  The Ethmoid sinus looks good from a frontal view but not so much as you go farther back.  He throws out two ideas.  One is that we can do the culture that our pedi requested under anesthesia to find out which antibiotics will work best against this.  The second option is that we can do exactly the same thing, plus add on another ten minutes to scrape the junk out of the inner sinuses.  That is the best option to me.  If that stuff has been hanging out for God knows how long, it may not come out on its own, and then we'd have to get the scraping done anyway.

Noon-  We're talking to a nurse, in hopes of setting up the procedure, but with all of Emily's medicines and such, she doesn't want to do it without consulting our rheumy group.  They also want to hold off on prescribing a new antibiotic until they talk to our rheumy's, which leaves me a little nervous.

12:30 P.M.- A nice quiet lunch

1 P.M.- A quick visit to the park!  Not that she runs around, but it's nice to go be closer to nature.

2:30 P.M.- We head to my parents house for a visit.  During said visit my mom again discusses how more of her colon is blocked and she's afraid that they won't be able to do the 2nd colonoscopy.  Also, my father feels tired suddenly so he goes to take his blood pressure.  It turns out to be 80/37.  Ouch.  Mom gets him some potato chips to bring his pressure up and he's good.  I take some time to order a bunch of prescription refills.

4:30 P.M.- We head to the school to get Zachary.  He tells me how his throat has been hurting all day.  Once home I pull out my scope (don't hate) and look down his throat.  Bright red.  Great.

5 P.M.- While I really want to nap, I realize that I still need to call the pharmacy about Zach's Enbrel.  It had appeared in my history, then disappeared a day later.  It had also showed that our pay portion was $2100, so I was a lot panicked.  I had called a month ago to give them our Enbrel Support info, and I had emailed it as well.It turns out that they LOST that info.  I was absolutely thrilled to find out that his Enbrel will be FREE!!!  Best. News. Of. The. Day.  True story.

5:30 P.M.- I decide to see if my school's website could tell me why I haven't seen any more info about my financial aid.  I had electronically signed the paperwork, verified everything and returned it all.  I see NO SIGN of that.  I once had an amount that I was supposed to be allowed and now I see nothing indicating that I was ever approved.  Hmmm.  I make a mental note to call on Tuesday when my son vomits.  He hadn't told me that his stomach hurt, just like he didn't tell Daddy that his throat hurt.  Now I'm really in a panic.  Need to take yet another day off of work, make another doctor appointment.  I have Omnicef at home, but with him starting Enbrel and needing his Methotrexate on Friday, I really can't take chances.  So, I call in to work for tomorrow.

7 P.M.- I realize that I need to scan a bunch of documents relating to their healthcare and send it to the school.  I email both kids teacher's.

See the up & down pattern?  Welcome to a typical day in my life.  And I have it easy compared to the kids.  They still have to go to school, pretend that they are normal kids, do their normal work, then have their meds, their shots, the infusions and the constant doctor appointments and tests.  Thank you Lord for making children resiliant!!!