The port is in!

Yesterday was a completely exhausting, emotional roller coaster of a day- but very successful!  Since Kevin had to take our son to school in the morning, he stayed home while Emily & I got up at 2 A.M. to get ready to leave for the hospital.  We had to be there at 6, and our drive is about 2.5 hours.  We made decent time, and there weren't too many people there yet.  She was so brave, sitting there reading a little, chatting, and watching TV.  When they called us and took us back, she didn't try to run away like I thought she might.  She charged ahead. 

The first thing that I noticed was there were two other kids waiting for surgery.  They were both around her age, maybe just a little younger.  One was having a tonsilectomy and adenoidectomy.  The other was there for tubes and adenoids.  Child Life came in to talk to the kids, prepare them, make them comfortable.  We got the adult nurse that started with "I see you have a history".  While he was very nice, I couldn't help but wish that we hadn't gone through so much that we didn't need Child Life.  As they were about to take her back to anesthesia, the surgeons were called back to an emergency.  So we waited.  They found a TV for her, and she busied herself with Cartoon Network.  By 8:40 A.M., they finally took her back.  I hugged and kissed her, told her I love her, and she gave me a quick "see you soon!"  She told me later that as she got back into prep she started to cry.   ;(    We spoke to many, many people about this.  We were told by most of them that the surgery was pretty much just like getting a blood draw, but in the chest.  No worse than that, right?  I went with that because I didn't want to scare her, and it's easier to think about when the picture is all neat and tidy, right?

The wait seemed to take forever, but finally they called downstairs to say that she was done and she did well.  As I went upstairs, I ran into the surgeon so he took me back to her and explained the situation as we walked.  He said that they tried her upper left- where they normally go- but it just wasn't working.  They ended up in the lower right/ middle area of her chest.  (He made it so she could wear a swimsuit without it showing.)  However, even there the vein wasn't good.  He said that she has really bad veins but, "God willing, this should last her 2 years or so".  Because of the veins being so bad, he had to run the catheter tube up into the main vein in her neck.  So she has 3 areas that were cut into.  She said that the worst of the pain is coming from the neck.  

The only thing that she complained to the surgeon about was the IV in her hand.  She yelled at him that she wanted that OUT.  He told her a nurse would be along after a chest X-ray was done. As soon as the surgeon walked away & I turned to her, she started sobbing.  "This was a bad idea, I want it out!  It hurts too badly, Mommy.  Get it out!"   (About the port)  I started crying with her until a very alert nurse came over to help us out.  I also didn't realize that the surgeon's resident had walked in.  They hooked her up with pain meds very quickly.  Not only did she take the meds with no complaint but she also drank right away- a good sign. As soon as they started to kick in she calmed down.  She started chatting with the nurses a little later about why she needed this and how great it was going to be.  As soon as she got her chest x-ray she was cleared to head upstairs to her infusion. 

For the first time ever she wasn't freaking out when the nurses came over to start her IV.   She told them that this was the easiest ever and she was really happy about getting the port.  Totally worth it. 

We had other things to talk to the doctor about.  We didn't have an appointment but I asked the nurses what we should do.  Em has had blood in her stool somewhat frequently lately.  I've called a few times about it.  I know it's hard to tell what's happening based off of that.  I suspected her Mobic.  This happened to her when she was on Naproxen, too.  She wasn't on Prilosec then, though.  One of the nurse practitioners came in to talk to us about it.  A week or two ago, Em had a bad stomach ache.  I knew that it hurt, but I didn't know how badly.  Up in recovery she had said something about "If I die at a young age"... I don't remember the rest because I stopped her to ask "Do you think you're going to die at a young age?"  She replied, "No, but I did the other week when my stomach hurt so badly."  I had no idea it was that bad, so as far as she's come with informing us about her pain, she still has a way to go.  So, after telling them about that, they agree that it may well be the NSAID doing some damage.  Just in case, they gave us a smear test to take home.  We have to take a sample from any 3 random bowel movements, then take all 3 samples back for our next appointment.  They say that if there's really blood in there, it may not be visible but it will show in the test.  All I could think of was NCIS and Abbie's character.  (I do love forensics...)

To top off the day, she shared with me that Emily's last labs showed that her IgG had dropped to 550.  She said that if they dropped below 500 then they would order an IViG for our next Remicade infusion.  Now, to backtrack to when we first started the Rituxan, at first it seemed like it was magically reversing her series of illnesses.  She had been sick constantly until we tried it, which is ridiculous since it's an immune suppressor.  Then she got my cold.  Between the cold and the surgery, I knew her counts would be low.  I even told the nurse to plan on it.  I got a copy of her labs last night before we left.  IgG: 459.  They haven't called me to tell me that yet, but they had said that if they were low they were going to try her on the 5 hour IViG infusion, which will mean another very early day for us, and likely closing the place out.  I expected a call today, actually.  Maybe the rest doesn't mean too much, and I never got around to looking it up today, but she's off on a LOT of things.  Her chloride, Monocytes & Red Cell Distribution Width are high; the RDW is always off, but I don't know why.   Protein, IgG, IGM and Lymphs are low.     I know the meds are working on her inflammation because the general inflammatory markers are awesome!  Her C- Reactive Protein (CRP) is <0.2!  Her Sed Rate is 9.  When she was kept inpatient last year, her Sed was 74 or 79.  Ouch!!!  And her muscle enzymes all look fantastic!  Her ALT & AST are 31 & 32.  I want to say that high starts at 40.  Her ALK looked good, too.  ALD wasn't back yet.   These meds are leading us in the right direction, I'm sure.

The best part of the day?  Hanging with friends in the infusion room  :)  Little Sadie was there, too! I had no idea they were going to be there, but it was so nice to just sit back and chit chat like the day hadn't been an emotional roller coaster.  The girls are sooo funny.  They are both so shy that they won't really talk to each other.  They ask us to ask their questions for them.  Too cute!!!  However, when it came to "Tangled", they chatted like they had known each other a while.  It was a nice end to a very long day.  We finally left around 4:30, making it the fastest infusion EVER for us.  And that was with getting upstairs about 1/2 hour after her noon appointment.

So much has happened that my mind is still whirling.  I'm probably missing a bunch, but I'm so exhausted still.  I was completely worthless today.  I think I've lost everything at least once today.  She's doing great.  They sent her home with about 2 days worth of Oxycodone.  I was a little shocked at that, but I know she needs it.  I was expecting Percocet or Vicodin, Demerol, something not as heavy-duty.  She's slept a lot, and she's having a bit of a hard time doing normal things, but she knows that it won't be long before she's healed.  The incisions look fantastic!!!  I was expecting much worse.  It almost looks like they used a clear glue over the stitches.  That makes the wounds look nicer.  Tonight, despite the pain she's feeling good about it.  She knows that this will make her life better.  No worries, no stress, no anxiety every month.  Thank you, Lord!!!  She amazes me so much.  Most kids would have freaked, but she's calm, cool and collected.  She's so sweet, so smart and so adult-like.  That makes me a little sad.  I'd love for her to be rambunctious, running around, a little ditzy even.  At least then she'd be a kid.  She's never really had that chance.  I am unbelievably proud of her.  I'm a rock for my family, at keeping things going, but she's my little reminder, telling us what we're forgetting, keeping track.  She's so strong, so amazing!!!  Yes, the situation really sucks, but we are so blessed with these kids.  <3  I couldn't have asked for better children.  All of them are amazing! 

I appreciate the prayers from anyone that took the time to pray for us.  I never thought that we would ever need prayers like this, but obviously they are working.  This could have been way worse.  As tired as we are, I feel like a weight has been lifted off of me with this.  I know to lean on Him when I need strength.  He is my strength, He is my refuge.  Thank you for your prayers.  <3

Port placement is Tuesday

As we get closer and closer to Tuesday, I find myself getting more & more nervous.  I'm sure that this port will be the answer to our prayers, but I'm still dreading the procedure.  We had a month long wait to get in to see the surgeon for the consult.  Then, her infusion was scheduled for the next week along with a doctor appointment, but they didn't have an opening for the surgery for that day, so we had to wait another 5 weeks until her next infusion.  That really figured because in August we were there every week, and in July we were up there twice.  This was the first time in a while that we had a few week break.  Unfortunately, that break allowed her anxiety to build and build.  Emily has slept in our room almost every night for the past month.  And yet, when I asked her if she wanted to cancel the procedure, she said no.  She realizes the benefits outweigh the risk.

It amazes me how strong she is.  The things that other kids couldn't handle she breezes through.  She is so logical, so hard working, so brave.  Her diseases may be hell on her, but they have helped to shape her into an amazing little girl that is wise beyond her years.  It's sad in some ways; knowing that her life is shaped around pain, for one.  She's had to make some hard choices.  I'm so proud of her.

I'm feeling very conflicted right now.  On the one hand, I'm so thrilled that port is being placed.  Especially after her last appointment where she hit and kicked the nurses for a while.  I was mortified.  I couldn't get her to stop, and they were all so sweet and patient.  It makes me angry that a surgical implant like this should be a blessing, a great thing for us.  How wrong!!!  No little kid should have to go through this, and yet she wants to do it.  She has really grown up this year.

We've been trying to think of a Christian song that talks about fear.  She told me that she wished she could find one that could help her, but my mind is over-stressed and drawing a blank.  Anyone have any suggestions?  I may still have time to download some.  Tomorrow I will have to be in bed by 8 or 9 P.M.  We have to be at the hospital at 7 A.M.  We live about 2.5 hours away.  I am sooo not looking forward to this.

On a different note, we're still working on getting my son figured out.  Our pedi wrote us a prescription to take him for a panoramic, but the radiology place by us no longer has the panoramic.  They suggest a CT scan, but I think I would prefer an MRI.  I know that's what the TMJ specialist in Birmingham suggests.  I'm going to call them tomorrow (if I remember) and see if they can either give us a different prescription or refer us to a TMJ specialist locally.  I've run into roadblocks at every turn trying to get him checked out.  Also, our pedi didn't give Kevin a prescription for labwork, but they must have read my note after Kevin left them.  I got a call a few hours later saying that I can pick up the paper copy anytime.  I had noted that, at the session in the conference, the Alabama doctor said that 83% of  JA children have arthritis in their jaw, but they don't know it.  It's painless and silent.  He also said that most children's start in their jaw, but it goes undiagnosed.  I'm still praying that we don't need to worry about that.  I've spoken with several people that have psoriasis and TMJ, so hopefully that's all it is.  Anyway, I also requested that *IF* they ordered labs, to please run the RF panel, the inflammatory panel, and the HLA-B tests.  Those point specifically to spondylarthropathies, which run with psoriatic arthritis.  Hopefully, all will be negative.  I don't need another baby in pain, though at least then maybe we could get his psoriasis under control.  It's very stubborn!

Needless to say, I'm having a rough week.  He's cool with this.  It doesn't really bother him, but I'm ticked off that I don't have any answers yet.  I hate roadblocks.  I know- I have time to be patient.  I'm working on it.  Really.  And I'm holding up.  I'm really used to this stress cycle.  When things are quiet and going well, I'm very edgy and nervous.  I think sometimes that most people don't ever have to deal with anything like this.  They can be blissfully unaware that 300,000  kids are out there suffering silently.  I miss being ignorant.  Despite all of the blessings that we've had grace our lives since Em's diagnosis, I would gladly trade it all for 3 healthy, normal children.  But then again, I bet that our house wouldn't be so happy and loving, we wouldn't count our blessings the way we do now, or try to see the bright side of everything, would we?  While it doesn't define our lives, it's certainly an integral part. Life is what you make it.

A Little Good News :)

Hello!

I'm happy to report a little good news.  :)  While she's having some minor issues, Emily continues to do well since starting the Rituxan.  I'm just thrilled!!!  She says that an area of her lower back always hurts, and her wrist to elbow has been flaring a bit, but not enough for her to want to go back up on her Prednisone.  She has chosen to continue on the 4.5MG dosing instead of going back up to 6MG.  While it may not seem like a big difference, she feels it.  She is also continuing to lose some of her steroid-belly.  We don't really notice it on her, but we notice in the way her shirts fit her.  The shirts that we ran out and bought her back in November when she suddenly gained 30 lbs are starting to look more like long baggy shirts.  I'm hoping it will be no time before they look like dresses again.

I always worry about her with school.  She likes school, and she works really hard.  She LOVES to read, to write, to be creative.  She loves her teacher this year, too!  Her school is amazing, and they always put so much time and consideration into where the best classroom placement is for her.  I really couldn't have her in a better place than where she is now.  This is the fifth year in a row now that her teacher has told me that her class "mothers" her.  I was told last night at open house that her classmates rally around her.  I have worried often about her being teased or bullied because of the Prednisone weight.  While it has happened on a small scale, she has yet to come home to me in tears.

Also at the open house, they told me how shocked they all were at how well she is doing  :)  Her school physical therapist had a goal for her to climb 5 stairs.  Thanks to Rituxan, she's pretty much given up the elevator.  (I forgot- she did say her knee hurt last night, but not enough to change med dosing for.)  They are all so impressed over how well she's doing.  I'm so glad.  Last school year looked so bleak for her; I didn't think we'd be able to keep her in school.  What a difference a small change can make, even when it looks so scary at first.

She is still stressing over her port placement.  We have talked about it every night now.  Just talking seems to be helping.  Before she told us what was wrong, when she was trying to tough it out, she was spending a lot of nights in my bedroom.  Tonight we discussed how God provides for our needs, we discussed what "Let go, let God" means, and how to accomplish it, and we talked about the phrase "What would Jesus do?",  thanks to my son.  I am so happy that they love church!  Emily especially can draw strength when she's down or feeling bad.  I'm thrilled that she has come to love our Lord!

We could use continued prayers if you would.   We have her port placement on Sept 27th.  It will be a very long day.  Please pray for a great surgeon, for their hand to be guided and their mind as well.  Thank you!

Good news for Emily, new worries for my son

We have a lot happening right now, as always.  I'll start with Emily's appointment.  Hers was actually great!  I suspected that it would be.  Minus the steroid weight that she still carries stoically around, she's feeling better than she has in a very long time.  Since we've added the Rituxan into her infusion schedule, she's now able to strap herself into her seat belt in the vehicle's by herself, she can finally roll her windows up and down in the car, and she's finally climbing stairs again!!!!!  That is huge to us.  I really hated for the kids to go back to school; I'm the weird mom that actually likes hanging out with her kids, but I knew that it would be so good for Emily.  She pushes herself at school in ways that she won't at home.  I'm so excited to talk to all of them when they get home from school, but especially to find out what physical activity she has done.  

The doctor was impressed at how far she has come on her Rituxan.  We are leaving her Remicade dosage the same, but we are able to decrease her Prednisone again!  We tried in July, but that was before the Rituxan.  The doctor did note that her left hand feels tight, but otherwise she was amazed at her energy.  She's also very happy that we have our port surgery scheduled.  Emily & I can't wait for that.  I'm so glad that Em's on board with this.  I really, truly feel like this could potentially be the best decision we've ever made for her.  Every infusion her stress level is worse.  She's great until the nurses come over with the supplies.  This time, they went & got her new favorite nurse (because she can get her on the first try usually) from the unit next door and had her waiting.  Em hates having the needle in or near her hands.  That's the first place they went.  She just freaked- shut down.  Next thing I knew, she's kicking, waving her arms around, thrashing, and screaming frantically.  The more upset a person gets, the harder the veins are to access.  They had to give her a break.  Even worse, her nurse warned her that if she didn't calm down she would have to leave because she had other patients, but Em just couldn't do it.  I think Em understood why she left, but I think she felt bad about it.  I totally agree with her needing to get back to her other patients.  She can't wait around all day- especially when it's not her unit.  I really appreciate how well the people in this facility get along.  There's a high level of support that I really appreciate seeing.  The infusion room nurses have to go through a lot.  The hours are good, but they have a lot of other screamers and harder kids.  Somehow they always seem to be a happy bunch.  It makes it so much more fun to go, because they're all crazy.  :)  For us, after the pokes are done, we can chill out.  (Often literally; it's freezing in there!!!)

Otherwise, her appointment went well.  I asked if we'll be ok to go to the family weekend at camp since it's the weekend after her port placement, and we're cleared for that.  The rest of what we spoke about wasn't actually about Emily.

I've debated on talking about this since I really don't know what's going on yet, but I decided that no matter what it turns out to be, these are the things that we have to worry about.  For anyone that wonders about the types of things that go through our heads, here ya go. 

I mentioned briefly at the end of my last post that my son had mentioned his jaw was clicking.  I swear, my heart stopped last week.  He showed me how he can pop his jaw and how it clicks and hurts.  I was dumbfounded.  When we were at the conference, we attended a session on JA & Jaw issues.  We missed the first 20+ minutes because we were speaking to another doctor in the hallway.  What we caught was that jaw issues are usually silent, and up to 81% of kids with JA have jaw involvement; the speakers says that evidence shows that the jaw may actually be the first joint affected.  My son has had psoriasis for about 3 years.  He has nail presence and other signs that lead to potential psoriatic arthritis.  Now, I'm not by any means saying that he definitely has PA.  However, when I asked our rheumy if she felt that it could be, she said that given his psoriasis and our family history, he definitely could be developing arthritis.  SO, I'm trying to get a panoramic x-ray appointment set up for him, but since we are totally broke from so many trips to Shands, I can't do that right now.  At least, not with his regular dentist.  I've emailed our hospital about it.  I figure, if it is PA he'll need to see the rheumy too, and since we're always there anyway I may as well schedule it for when we're there with Emily.  I am praying that it is just coincidence.  Not that I would want him to have TMJ, but TMJ would totally be better than deterioration from arthritis.  Not to mention, at least TMJ only affects the jaw area.  Our rheumy said that most PA is considered in the Spondylitis family- something I haven't read up on yet.  I've read up a lot on PA today, but I think we're in for a wait.  I really hope that my precious little boy doesn't have to go through this crap, too.  If he does, I'm hoping that we are catching it way earlier than most kids are diagnosed.  There are some things to be said about a heightened awareness!

And I will leave you with food for thought.  We are very blessed to have (for Emily only) Medicaid as a backup from Social Security.  There is no way that we could afford our copays without it, as I will show below.  This is a copy of our insurance explanation of benefits.  Emily has a $3500 deductible.  If we hadn't met her deductible, and if we didn't have Medicaid, I cannot imagine what our copay may have been.  We have a 20-30% copay normally.  I know that we had a balance from one bill that Medicaid wasn't covering that was over $5000.  There was another for $1200 and another for $1500.  How could any normal person afford these treatments?  Why do they have to be so expensive?  And, if arthritis is "no big deal", then why would we be paying such a high amount of money per visit???  The visits include seeing the doctor, labwork, and infusion meds.  Of course, it's the meds that are expensive.  When she was on Enbrel, we could do her shots at home and with only a $10 copay with their assist program, but without insurance they would have been $2500 per month from our pharmacy.   Someone told me once that they were charged $36,000 for an IViG.  I can't find it again, but I believe I read that Rituxan goes for $11,000 for the first 2 doses.  The rest are smaller doses, going for more like $5,500 each.  

The All Children's bills are from our therapy appointments at a specialty center.  She has occupational and physical therapy weekly.  (Her therapists are awesome!!!)  The last entry is from our pediatrician.  (LOVE her!)  I learned a few years ago that doctors charge you by your conditions.  If you are a very healthy person seeing your doc for something like a sinus infection, you will get charged MUCH less than my daughter will, even if she's being seen for the same sinus infection.  Good times, eh?