Last kidney procedure!

I am so excited to report that the stent is now out of the ureter!  As a quick recap for those who have missed it, Emily had kidney stones embedded in her kidney as a result of the steroids over the past three years.  Because they were large, they realized that they were not coming out on their own, so they opted for surgery.  She had one surgery to place the stent to allow the ureter to open enough to be able to see the stones, one procedure where they lasered the stones apart but left the stent to allow drainage, and one last one today to remove the stent.

I am usually the mom that loves everyone and has a hard time finding fault with the people and situations that pop up in the hospital.  I wasn't even really angry when we had to change rooms two weeks ago at midnight- after I had already fallen asleep.  Today I was livid.

We have two hospital choices when it comes to the urologist.  One is a big hospital with a children's hospital attached, and one is a hospital that is only for children.  One would think that the primary children's hospital would be... child friendly, right?  No.  This would be the same hospital that never checked vitals when we were there for her staph infection that was inside of her port.  We usually won't go there, but in this case I had to make the decision in the middle of Kohl's with my calendar being my phone.  I just thought this would have the stent out two days earlier, and it couldn't be that bad, right?  

I knew we were in trouble when we got there and Emily pointed out that we all forgot to numb her port area with lidocaine (Emla) cream.  We discussed it with a nurse who was more worried about getting Emily to pee.  One would think we would work on the Emla first since it needs time to sit, but nope.  She gave her a gown 12 sizes too big and demanded a urine sample- without the little cleaning cloths.  Em was like, "but this won't be a clean catch!"  They put her chair at the opposite end of the hall, and kept knocking to see if she had peed yet.  We finally gave up and went back to the room.  This time there were 3 nurses in the room, none of which had Emla.  I finally texted Daddy to run out to the van.  Oh yeah, did I mention that only 2 people were allowed back there with her?  At our other hospital there was much less room but we could all go back together.  Here they said that we could bring them back in "a little while".  She also gave me the wrong room number so I almost didn't find them again.  She said 9.  They were in 10 with the curtain closed.  Whatever.  One of the three nurses never mumbled a word until it came time to attempt to access the port.  It took two of them, one asking the other to grab supplies, but neither seemed to know what they were doing.  In the end they hurt Em a bit, and then used a seriously oversized bandage on it.

I could be cool with all of that.  Mostly.  My final problems were that they finally allowed Dad & Zach back after two and a half hours back there, only to have them hang out for only five minutes.  The worst part was that they didn't give her anything to help her calm down before leaving the room.  In fact, they didn't even give us the, "last call for hugs" warning.  It was just bars up, say goodbye.  They said we could have a moment in the hall, but they kept the bars up.  She cried all the way down the hall.  You would think they would have handled that a little better, especially knowing that she is medically complicated.   I was so upset about that.  We all decided at that point that we were never going back there.

There were a few bright spots.  The anesthesiologist was very sweet and he took his time, asking excellent questions.  He even pulled a tooth while he was in there!  :)

The other bright spot was our nurse practitioner.  She has RA, and she used to see our first rheumatologist, now known to us simply as, "He Who Shall Not Be Named".  She made a Freudian slip when discussing him that had me cracking up!  He tried to screw her over, too.  It was great to have someone who understood- if only for a few minutes.  Overall though, we will never go back there.  I don't care how new or beautiful an establishment is, or how good the view.  If the quality of care is lacking, it just ain't worth it.

Endocrinology & stuff

This has been the quietest week, mostly because we scheduled it for recuperation. Emily can feel the stent in her ureter and it hurts, but I don't think it is horrible. I keep telling myself that it will be out in another week, & hopefully she will feel much better. She has been refusing pain meds, so that is a plus.  Prayers would be wonderful, specifically for the ureter to be opened enough to grab all of the stones out of her kidney this time, no reaction to anesthesia, & of course to guide the surgeon's hands. 

Biofeedback is seriously one of the best things that we have ever done for her. I think that every child with chronic pain needs to try it.  Not only is she learning techniques to help her to deflate the pain but she is also learning how to take control over it. I really wish we had done this when she was five and limping everywhere, when the anxieties were so high that she seemed to be obsessive-compulsive. This would have been so life-changing then. I will have to tell our pediatricians that. And like all of that isn't cool enough, she is giving us some common-sense nutritional advice that I would not have thought of. Instead of keeping our fruits in the produce keeper, I am now washing them & placing them in a bowl on a low shelf so that everyone can see them. As Ginny says, people are more likely to eat what is already prepared. So true!

Aquatic therapy is still a challenge for her, but last time she got through it with the help of Xanax.  We try hard not to use them, but when they are needed I am happy to give her a half of one. That is the only way she did well last week. She told us at biofeedback that she is afraid that the kids in the pool will laugh at her, & the old people will pinch her cheeks. We found a time with no kids & few older people. I know she will love it once she chills out a bit. 

The big news of the day is endocrinology.  You may recall that she was seen last month. The doctor, who was absolutely one of the sweetest people I have met, ordered a bunch of lab work to be done. We had it drawn when she went in for kidney procedure #1. She already received 6 of the 8 ordered tests. So far most of it looks great. However, she also asked for copies of her growth chart from our pediatrician. I don't think I ever realized but Emily has always been in the 2nd percentile for growth. Yes- second, and she really hasn't grown this year.  Granted, I am NOT the queen of height (4' 9.5" myself!) but I think even I was taller back when I was her age. I worry about several things: A) the theory that the body holds in more pain when the cells aren't regenerating as quickly, causing her to feel amplified pain, and B) I worry about her jaw. One of the things that I have always noticed about adults who had JA as kids, and especially those that were on prednisone for long periods is that their jaws never seem to finish growing. It's like the jaw only grows to a certain point even if the rest of the body isn't finished growing. I didn't want her to be the kid with the tiny jaw. Imagine the implications of that- not having enough room for your teeth to fit (already a reality here), the need for more medical procedures to correct it. As it stands she only has a few more good years of growing. Her bone age is delayed, but not by a tremendous amount. It may be possible to catch up now- if we hurry. 

The doctor (her name sounds like Tab-ew-wad-a) said that she feels that Emily would benefit from growth hormone. She asked me to talk it over with Emily. I took that as "give her a choice". So, we discussed it last night. Her shots will be every day but she is okay with that.  She wants to try it.   I think she is getting irritated by people always thinking she is so much younger.  When they know her age they express shock. That's probably not good. I really hope that this is the beginning of a new, happy road. We will be so thrilled if this works.  All in all, things are looking up!  Thank goodness because this has been one heck of a summer that we wouldn't care to repeat.  Would anyone like to share their stories of aquatic therapy, biofeedback or growth hormones?  Please let me know!

Stone Update

She had aquatic therapy twice this week so far.  Monday's was not so great.  She promised me that she would give it a chance, but she wouldn't cooperate at all.  On Wednesday it went well!  I cannot say for sure that she enjoyed it, but she at least cooperated.  She looked like she was having fun.  Part of the reason that she did so well, I think, was Tuesday's Biofeedback session.  What a wonderful tool!  Again she was hooked up to the little EKG probes, and again we could see her tension visibly on screen.  The cool part about that was that when she was upset about something we could see it shoot up to like 18, but once she started deep breathing and listening to her relaxation exercise it dropped down to about a 4.  As Ginny pointed out, "see, Emily?  You have the power to relax yourself.  You don't need anyone else to do it for you".  How cool is that?  We discussed aquatic therapy quite a bit that day.  I was very pleased that she did so well the next day.

Yesterday was the kidney stone procedure.  Of course, it wouldn't be our bumpy road if anything worked as intended!  The doctor called me on Tuesday as we were walking into a hospital for Emily's biofeedback to tell me that we may not proceed with the lithotripsy (shockwave of the stones).  I really had no clue what was planned, but we are pretty used to this type of thing.  Things always work out for the best whether we stress or not, so I chose not to stress.  He called me the next day to say that they had a new plan, but it would likely mean two different procedures.  He had initially said that the lithotripsy would require two procedures also, so it wasn't much different to me in that respect.  He told us that he conferred with specialists all over the world to determine the best course of action for our special girl.

They put a stent inside of her urine tube to try to make it bigger.  He told me that it was likely they would not be able to get the ureter opened up enough to grab the stones today.  Sure enough, he was right.  He said that she would have to have the stent in place for 1-2 weeks.  It should dilate the tube enough to pull them out later.  Right now she hurts but not too badly.  They gave her pain medicine, an antibiotic, a medicine to help with bladder spasm, and another med that I cannot recall right now.  The first few hours were awful!  She felt so terrible, and she was in so much pain.  Right now she seems to be her normal self.  It's probably somewhat the pain meds, but it is reassuring.

The only beautiful thing about this is that aside from my working this weekend, we have no appointments planned until 2 weeks from now.  It really just depends on when we go back for the second procedure now.  Thank you so much for your prayers and love.  We felt it, it helped me to be calm and collected.  Emily was still a bit of a mess, but still much better than most kids would likely be.  Thank you for your support.  It is very appreciated!!!

The next post will be a how-not-to-die-at-the-hospital type of post, I think.  I have spent a lot of time comparing how I was ten years ago to now.  I have learned so much, become so organized which is really weird for me.  I feel like I need to share it.  That is coming!  Stay tuned!

Balancing Act

This past summer has been our biggest trial to date.  It has been a series of errors, really.  It started with my thinking that Remicade wasn't helping as much last summer.  Switching her seemed like the right idea at the time because I always had to base my decisions off of actions before that.  She is a child that doesn't like change, so I expected resistance.  I wasn't really expecting Emily to not respond to the next two medications.  Just like I didn't think that taking a break from physical therapy would be too bad.  Well.....  I guess this goes to show that Mom isn't always right.

As some of you may know, I have always known what is wrong with my children.  I know sometimes before they are fully sick that they are sick and with what.  I pretty much understood everything that was happening to Emily, and I was able to anticipate things.  Sometimes I could even tell you exactly what hurt.  And then this year came along and that all flew out the window.  I know that there are several things at play here- for one thing, puberty is rearing its ugly head slowly.  That alone can change all of the games.  I think that some of it is because of the kidney stones, and some from pain amplification.  I think the pain amplification started because of the therapy break and the med changes.  But I think we are on the right path.

This summer has been insane with appointments, but this week alone has been very trying.  We had our hospital medicine overnight appointment on Monday and Tuesday.  No really new changes, though we did have the worst nurse EVER.  I always pretty much love everybody and tend to overlook a lot, but this lady literally asked my 11 yr old to walk her through how to de-access her port.  At first I thought she was kidding.  I was in the middle of saying, "So, if you don't know how to do this why don't you leave it for someone that does?" when she got it out.  I almost died.  It was horrible.  We made it home safely at least.

Wednesday brought us to another out of town hospital for Endocrinology and Nephrology.  We started out so early, but our GPS totally messed us up.  We got there 30 minutes late, but they still saw her.  I did call to let them know we would be late.  I pre-paid for tolls expecting more than last time because we planned on taking the full toll road, but the stupid GPS never even put us close to a toll road.  At all.  That was stressful.  A 2 hour drive turned into 3.5.  Endo is ordering growth charts from her other doctors.  She did a bone age scan.  Em's hand looks about right for an 8-9 year old.  She wants us to do a plethora of blood work.  We are waiting until she has to be accessed next for it as it causes her so much anxiety.  Hey, maybe when she has her kidney stones blasted out!  We shall see.  She said that there is a growth problem marked by either a missing or a mutated X chromosome.  It mostly affects girls, and it can cause problems like arthritis, so she ordered the chromosome test.  I was worried a little about Zach, too because his feet bother him so much.  Since he has PsA, I dig through those medical papers a lot also.  I asked her about this article.   She was very surprised that it discussed type 2 and not type 1, but either way she didn't think that we had to worry about Zach even with the family prevalence.

Nephrology discussed with us how to avoid kidney stones in the future.  In truth, it may be difficult to avoid because they were likely caused by her Prednisone, but drinking more water and eating less salt will be helpful.  She drinks water, she almost always has a bottle with her, but she doesn't drink enough.  They aren't usually allowed soda, either.  She just doesn't drink enough period.  And she craves salt.  She craves popcorn, potato chips, Ramen noodles.  I hadn't realized it before but the little packets of flavoring that come with those noodles is 33% sodium.  OUCH!  Anyway, about 8 weeks after her second procedure he wants to do a 24 hour urine collection, and then see her back.  He also wants us to collect pieces of stone for him to examine.

Before we saw Nephrology I stopped by to pick up copies of the CT scan and report.  Of course I come home and research it all.   Her stones are HUGE.  One is 5X5 mms and one is 8X10 mms.  WOW!!!  That is crazy.  There is no way they would pass on their own.  I also read on the report that she has "gaseous distention of the colon" and a few other things that looked scary.  It all seems to point back to 2 things- her esophagus is still not meeting at her stomach properly, which causes GI problems, and her stomach muscles are still weak, which are causing bathroom issues.  I think once we get those stones out and strengthen those muscles she will feel a lot better.  Her first procedure is scheduled for next Thursday, the 25th.

Thursday was her first appointment at a place that offers aquatic therapy.  For a normal child this would be happy news.  For a girl with high anxieties this was like walking the plank.  She was NOT happy, so she kind of took it out on the poor therapist.  She tried every trick to engage her, but Em wasn't having it.  She was so worried about being in the pool that she couldn't see the happy side.  Today was just an evaluation, but we start regular sessions on Monday.

We also started Biofeedback Thursday.  We met Ginny, probably one of the coolest ladies that I have met this year.  She was really chillin', but she also really listened to us.  She asked Emily a lot of questions, and they talked honestly.  It was lovely.  They talked about sleep.  Emily said that she reads to go to sleep every night because she hurts so much once she lays down to fall asleep.  Reading takes her mind off of the pain.  Also, she said the pain wakes her.  (Sigh).  At least now I know.  We have been asking her about that forever.  Ginny will be able to teach her techniques that she can use to fall asleep, to help herself relax, to help manage her pain.  How cool, right?  But it gets better.  She placed electrodes on Em's main tension points around the head, jaw, neck and shoulders, and checked to see how stressed she was.  She said that a normal person without chronic pain will be about a 2 on everything.  Em had almost all of them 6+.  She had one 13.5.  I forget what her jaw was but it was really high.  This is likely the cause of her migraines, headaches and jaw pain. So, we made our next 6 appointments with her.  The week after our last appointment with her we will finally be seeing a good psychologist.  Since the one that we interviewed for her seemed more overwhelmed by her situation than we are, we thought we would try again to make an appointment with the one who only handles kids with chronic pain.  Between these 2 people, if she is still having headaches, Ginny recommended a headache specialist!  I am hoping the Biofeedback will kind of heal that, but one day at a time.  It will give her great coping tools that she can use for the rest of her life.  And she told me that our pediatrician that recommended her called her yesterday to see if we had been to see her yet.  So sweet!  I will text our pedi tomorrow to let her know.  We have some of the best doctors!

All in all, it was a very stressful week but also a very good one.  I feel like we are on the right path, like we have the right tools in place.  In a stressful time a couple of weeks ago I was trying to figure out how I would juggle all of this.  I figured that if I did aquatic therapy after my classes are out on Mondays and Wednesdays and perhaps any Friday that we were in town then we could do biofeedback and psychiatry on Tuesday's, and our out of town appointments on Thursday's.  So far that is working out brilliantly!  I didn't actually expect it to work because it is a lot to ask of other's but the pieces are just falling into place (praise The Lord!!!)  Hopefully this will help a lot.

Anyone have a cheap helicopter for sale?

Looking back on the past few years, I have NO idea how I worked full-time.  This year has been so hectic, and we have added so many new specialists.  The past few months have been extremely difficult and overwhelming.

Zachary is doing better than Emily still, but we have noticed... things.  For instance, now that I have "kicked the kids out of the house" (to play), he complains more about pain from behind his knees.  His rheumies hadn't mentioned anything before about enthesitis-related, but with Psoriatic Arthritis it does make sense.  His ankle pain comes and goes, but lately it is more knees than anything.  Last month when he saw our rheumies, we were talking about a friend of his who has had a lot more disease activity.  I pointed out that Zach's case is relatively mild, but the response that I got back was, "PsA is a lot harder to treat 10-15 years out.  PsA kids tend to be the hardest to treat and control down the road".  Great.  My heart sank.  Fortunately, we live day-by-day!  I am not going to get caught in that trap, and I won't let the kids get caught, either.  I'm just happy that he is outside playing.  Emily has been playing outside too, though she doesn't last as long.  I am sure that she has been depressed, but hopefully the plan we have will address that and correct it quickly.

I have always been the mom that knows what is happening.  It has been very rare that I was wrong- but the past few months I have felt so out of the loop, like I'm part of a guessing game.  I think we are on the verge of getting that situated, but still it has been hard on me.  Being this hard on me, I can't imagine how my little people cope.  We did switch back to Remicade, which definitely seems to be helping.  She has gone from having mostly 7-9 on the pain scale days to 4-5.

I think I am finally figuring out Emily again.  Ok, this is a lot.  So, she does have kidney stones, which we feel are definitely making life more difficult.  However, what makes everything tougher is that she has "Pain Amplification Syndrome".  I have mentioned before that it was brought to our attention that when one isn't growing properly, their cells aren't reproducing the way they should, which makes the body hold pain in.   After a friend mentioned it, I started suggesting it to different doctors.  They all agree.  That said, we see endocrinology on July 17th.  I am truly pinning all of my hopes here.  As KM said, in their case once growth hormone was added, the first month was horrible but after that everything settled down.  Pain came down, growth started; the best part is that the hormone is a natural hormone, and for them it helped them to decrease some and eliminate other medicines.  I would LOVE that!  I am quite sure Emily would, too.  I am also hoping that this will help her headaches.  Neuro-opthamology didn't see any reasons for her headaches, which I now think may be a combination of hormones (pre-pubescent) and the pain amplification.  We are scheduled to see nephrology the same day we see endo.  The very next week Em has her first psychology appointment, and the day after that she has a procedure to blast her kidney stones out by sonic waves.  They will sedate her because a tube needs to be inserted up into her kidney.  They tell me they will repeat this procedure on another day because sometimes they stones are stubborn and either won't break up or will break up but won't leave the kidney.  We really hope that having these little nasties gone will help her pain level go down a bit.

Another new specialist that we have added is pain management.  That doctor wants her to try aquatic therapy three times a week, as well as biofeedback.  She says that these will both help the amplified pain syndrome.  I'm just not sure how to fit these in.   We have found the aquatic therapy close by, but I still haven't spoken to a provider for biofeedback.  Right now it looks as though it may be an hour south for BF, close to urology.  Comparatively speaking that is not bad.  Rheumatology, who handles pulminology and GI are all two & a half hours north.  Pain management, endocrinology and nephrology are all 2.5 hours east.  With all of these appointments, school work for the kids, and my own schooling come fall, how the heck am I going to swing aquatic therapy three times a week?  I'm not sure, but I know that we will.  If it will really help her, we will do it.

The pain management doctor prescribed pain patches for her.  Just lidocaine, but it seemed to help her knees a bit.  I love that she chose something that didn't require another pill.  I am not familiar with biofeedback, but from what I understand, it involves teaching the child how to cope and deal with their situation using relaxation techniques and monitoring bodily signals like blood pressure and pulse oxygen.  It sounds really cool!  Our advocate has suggested waiting to see what psychology said.  Our psychologist was thrilled that we found a doctor who believes in hypnosis and biofeedback.  I think psychology is a little overwhelmed by our situation.  We had a good laugh over that tonight.  She was pretty amazed at what we are dealing with.  Like I told her, one day at a time and we stay close.  None of us could do it alone.  We need to take this all on as a family.  It would just be easier if we had a helicopter.  Or two.  Or a fleet.

Last thing- Camp starts Saturday for Emily!  She is SOOOOO excited!!!  In truth, for the first time I am glad for her to go to get her out of here, to get a break.  She is so sweet and wonderful, but it seems like every day we are at some appointment.  That will be one week where we are guaranteed not to have to go to an appointment for her.  The down side is that we had stopped Mobic (an NSAID) a couple of weeks ago because it was starting to hurt her stomach.  Since we were stopping Mobic we decided we would drop Carafate (similar to Xantac, it coats the stomach).  Today we ended up back at the pediatrician's office, thinking that her antibiotic wasn't working for her sinusitis.  It turns out that her sinusitis is gone, but her reflux is acting up again.  The Carafate is such a large pill.  I cut it in half for her but it is still so big.  She really hates taking it, and we were so excited to drop basically three pills a day.  Now it's back on the Carafate.  I'm not sure how she handles it, but she is pretty amazing.  I am trying to get us on a wheat-free, gluten-free (or very reduced) diet.  It is very difficult when you have children who are as picky and stubborn as Emily.  She literally won't eat if we don't give her one of her staple foods, but we are learning how to find healthier options.  For instance, I could buy all kinds of fresh fruit but it will sit in my fridge and rot.  I discovered an organic fruit pouch (no GMO!) that my kids have sucked down.  It's actual fruit- not juice.  If that is the only way I can get them to eat fruit, I will do it.  Baby steps.

I will keep y'all updated as we determine new things.  It has just been tough.  Thank you for reading and supporting us.  As always, prayers, blessings, whatever you have to offer, we will always gladly accept and  appreciate fully.

Our latest craziness (written May 18)

For anyone that follows my Facebook page, you already know that we have had a crazy week.  It was actually more crazy than I even let on.

Our lives had basically been put on hold.  We knew that Emily would end up at the hospital inpatient for a while, but we were not sure when.  We had hoped to see a doctor, and that they would keep her, last Wednesday when we went for her meds.  They didn't have a chance that day because they were very overbooked.  The doctor running the clinic is only there once a week.  Emily was devastated.  It's a bad sign when your child actually wants to stay at the hospital.  Especially when said child is the type that never complains.  I spent the days following refusing to unpack, and not wanting to plan anything.

My oldest debated whether or not she had an ear infection.  One day she would want to see a doctor, and the next she felt fine, only to feel badly again the next.  My days were pretty booked up.  I had finally gotten around to scheduling a well-check for myself.  It had been 5 years!!!  I didn't think it had been that long.  While I was there they found a lump, which of course ended up with a mammogram for the next morning.  I had school stuff to turn in, other paperwork to do, and now my own health concerns.  I actually wasn't very concerned until I got there.  They scanned, then they re-scanned.  Then they performed an ultrasound.  Thankfully, I have cysts but not cancerous.  After I left that clinic that day, I ran to school, did a bunch of errands, and finally got home to find that Emily felt worse. We had already scheduled a pedi appointment, so we went there.  We stumped the ARNP.  She didn't know what to do, but she sent us for chest x-rays in case it was due to pneumonia going undetected.  After doing the x-rays we went home. She had already been asking to sleep in our bed over several days before.  One night I just pulled out the sleeper sofa.  Hate that thing.

After we put the kids to bed Tuesday night, Emily decided it was time to go to the ER.  Despite being mostly packed, it still took a while to get us out.  To show you how much of a crazy house this can be, my son's ankle had started to hurt earlier in the day.  He is over-exaggeratedly limping around the house.  He tells Emily as we are packing up to take her to the ER that he is in the worst pain ever that she couldn't even imagine.  Okay.  We got to the ER around 2 AM.  We wanted to go where her rheumy is because we felt sure that all of her problems were rheumatalogical.  This time it was her joints, her muscles, and her chest/ breathing that was bothering her.  They ordered an EKG, chest x-ray, and ultrasound of her liver, kidneys and bladder, and of course they did a urine test.  Apparently she has kidney stones.  They found traces of blood in her urine.  We were unable to see nephrology over the next few days, but we were also told that the best nephrologist is at a different hospital.  .  

Because it is what she wanted, I asked the ER if she could be admitted.  They ran it by rheumatology.  We have been going there since October of 2010, and we have never asked for anything out of the norm, which is likely why they agreed to it.  Her labs look perfect.  That's stupid.  True, but stupid!  I have spoken to a LOT of parents of patients, and patients as well that have said that many times when they are in a flare the labs look great.  Usually the doctors don't understand that, but it is true.  Because her labs looked so good her doctor thinks that her pain is caused by Pain Amplification Syndrome.  Perhaps there is some truth to it, but I really, really think this is a flare- her first since we started going there.  He sent in a physical therapist to do a CMAS (Childhood Myositis Assessment).  I asked the PT about how her score was.  She said "about 20 out of 50".  Her neck muscles seem strong to me, but she can only keep her head & arms up for about ten seconds, and she was afraid to lay down because it hurt too much.  I know the signs with her.  The red spots had started to come back on her hands, two different types.  She had the vasculitis "hot spots", and the smaller red dots that she gets with Dermatomyositis.  Her elbows are pink again at the tips.  Her knees are pink.  This is a flare.  Most importantly, not only did she have trouble holding a toothbrush and walking, she told me how badly her fatigue was affecting her.  The fatigue was getting worse daily.  I guess that we all disagree at some point, and I totally love and respect all of our rheumies, so we agreed to disagree.  :)  I know that he sees a lot of teens that have pain amplification.  He seems to think that is where she is headed because of her age.  I know her well, and I have (obviously) seen her go through everything.  She has always taken it well.  I have some theories.

She started to get the headaches when we started CellCept.  She adjusted after about 2 months, and the headaches went away.  When we started Orencia, the headaches came back, but only for a while.  When we switched to Actemra, not only did they come back but every infusion saw a pain increase with the headaches.  While one would likely assume when reading Actemra's side effects that headaches would be isolated to around the date of infusion, what if that isn't what they mean?  That could solve that.  We discussed going back to Remicade.  Emily is thrilled.  This is my bad: judging by the way she was acting towards the end of Remicade, I felt it wasn't working as well.  I saw her in the bath constantly, something she usually did only when she was hurting.  She is resistant to change on almost everything.  I thought she didn't want to change because she didn't want to try something new.  Apparently it really worked.    He has warned me that it may not work as well the second time around, but we had nothing to lose by trying.

She said that she felt better almost right away after starting the Remicade.  It is likely more because she had three days of IV steroids, combined with Toradol (a strong NSAID) around the clock the three days, but regardless her pain has gone from a 9 down to a 4 today at home all day.

So, right there we need to find a nephrologist, hopefully the one he wants us to see.  He wants her to see pain management because of her age and how they usually deal with pain badly.  He wants her to see a psychologist, because she always seems so sweet and even, and she has never gotten nasty, mean, angry, or moody.  And I mentioned endocrinologist.

Thanks (again!) to KM for bringing this up.  Her son had many similar problems, from lack of growth due to steroids, "something" showing on the MRI, and possible amplified pain.  She explained that (and I apologize if I don't get this 100% right) when the body is not growing right, the cells do not multiply as quickly as they should.  Those cells help the body to heal, thereby reducing pain.  IE: if one is not growing, not only is their body out of whack but their pain can be greater because it does not have what it needs to heal.  I explained this, and the fact that it allowed her son to get off of a considerable amount of medicines!  If we have to add something natural to get off of something(s) unnatural, it is well worth trying.  So we are looking for 4 new doctors.  Apparently pediatric endocrinologists are hard to find also, or we would have seen one while we were inpatient.  At least I feel like we are back on the right road, and we will hopefully be back up soon.  One thing that was beautiful was having so many people check up on us while we were inpatient via phone call, text or Facebook messages.  Thank you all for your love & support!

One last note- I had gotten the camp email saying that she was placed on a waiting list because she had already been to summer camp several times.  She was devastated at first, but she decided that since they could call at any time, she may have a chance.  The following week I received a call from camp.  Due to the severity of her disease they thought that she needed to go anyway!  I tell you, that was exactly what she needed.  She needed something wonderful to look forward to.  Thank you, Camp Boggy Creek!!!  They really helped her when she needed it most.  I was sobbing on the phone.  LOL!  Thank God!!!

*I apologize in advance for anything that doesn't make sense.  I worked a long day, and it is now past my bedtime, not to mention it is a LOT to process.  Back to work in the morning!  Have a fantastic day!  :)

Prayers needed (long)

Right now, every time that I sit down to write, something else comes up that makes me think that I should wait for an answer.  However, since writing helps me to think, I am better off just writing.

Last Friday we went for the requested MRI's to figure out why Emily's head has been hurting so much, for a month or two without relief.  While she seemed so prepared, once we were in the room with the machines, the tech mentioned that he would have to poke her for contrast.  This started an uncontrollable wave of crying that lasted about a half an hour.  It made him very nervous.  She finally calmed down enough to try the jaw MRI.  When the time came for the tech to try the contrast, he couldn't get a vein.  Two tries and she was so upset that he did not push.  It was draining and terrible.  In the end, he showed us clips of her brain.  I thought that was really cool & sweet of him.  He didn't have to.

We went to the hospital on Monday.  I was hoping that we would have an opportunity to really sit down with the doctor and talk for quite a while.  What I didn't count on was spring break.  While there are usually 3 doctors, two Advanced Registered Nurse Practitioners and an RN, this week there was only one doctor and her secretary.  She barely had time to breathe, though she did order a Pulminary Function Test when I mentioned that Emily was having a hard time breathing and her chest hurt. I was planning on getting a plan for exactly how long it would take to wean off of Prednisone, but instead I just got a "we are working on it".  Ok, I get that.  Things happen, we may need to increase, etc.  I may not like it, but I understand.  It's hard to push when she still hurts in so many joints.  I am not convinced that Actemra is right for her, but here is to hoping that it kicks in soon.  I was hoping that we would have MRI results, but no such luck.  She couldn't find them.  We didn't officially get PFT results either, but I did receive a comparison with last time which tells me that everything there is ok for now.  Oh, and we got a prescription for the *occasional use* of Xanax.  This way she hopefully doesn't make another tech nervous.  I didn't have nearly enough time with her, for the most part.

This time on our overnight, the pharmacist came by to go over meds; we never even saw a resident.  I actually like it better that way.  It's nice to cut out the middleman.  For the first time we got a room that was not properly cleaned, but they brought up a dinner tray- something that we usually don't see- and it was what she wanted.  Bonus!

When we got home I started preparing for the appointment with the oral surgeon.  I had debated on whether or not to cancel this, but in the end I decided to keep it because if her headaches were caused by arthritis of the TMJ joints, we would need an oral surgeon.  I had the panoramic from the dentist, the CT results that suggested the molar roots in the maxillary sinus cavity, and the paperwork.  I arranged to also pick up copies of the MRI's from Radiology Associates.  I was not prepared for the report.

They had done 2 MRI's- one of her jaw and sinuses, and one of her brain.  The one of her jaw apparently was not that fantastic.  She moved and they did not get good images.  They did not even mention the sinuses.   Of course they ask me some questions, as well as get a report from the doctor.  We checked yes to headaches, tinnitus, dizziness, feeling off-balance.  We discussed MCTD, Dermatomyositis and the intricacies of rheumatic diseases.  I mentioned that vasculitis had been thrown around here and there, but it never went further than that.  Then I get the report.

  Sometimes it is a beautiful thing to be blissfully ignorant.  I have somewhat looked up vasculitis before, but since it was never (in my eyes) an official thing, I didn't worry about it, so it struck me at the bottom there- "history stated CNS vasculitis".  Whose history?  Presumably from the doctor's since I never said that.  So, what else would I do?  I google.  And I find this:  "If a blood vessel with vasculitis is small, the vessel may break and produce tiny areas of bleeding in the body. These areas will appear as small red or purple dots on the skin. If a larger vessel is inflamed, it may swell and produce a nodule (lump or mass of tissue), which may be felt if the blood vessel is close to the skin surface."  
I had asked in the past what those red spots on her hands were, but I guess I just didn't really put two and two together.  She also pointed out on Friday that she has a "nodule".  I wasn't convinced that it was a nodule, but I guess it may be, or it may be vasculitis.  Yes, this is why the MRI was supposed to have contrast.  So, that floored me, but only later.  What really got me was the Chiari I Malformation.

I had heard of this before, so it wasn't a completely foreign term.  I had even studied it briefly at one point, just because it sounded interesting, and a Facebook friend had one.  I'm pretty sure my heart stopped when I read that, though.  My first conscious thought was "it has to be from the damn steroids!"  Apparently not. They say that it is genetic.  Hmmm.  Of course, I read.  As soon as we got into the oral surgeon's office I googled Chiari, and was stunned to find that she really does have many of the symptoms.  On my phone that was the best I could find very quickly, but she has many of those symptoms.  It even explains her chest pain.  I also read that it is usually diagnosed off of symptoms and an MRI, so my guess is that she will be diagnosed as soon as we get into a neurologist, though I want to speak to an endocrinologist to make sure that it isn't cysts affecting her growth.  (Thanks again, KM!)  In an effort to expedite things, I popped by her pediatrician's office and left a copy of that report.  I am hoping that somebody should call me back no later than Tuesday.  

And the oral surgeon didn't have the greatest news, either.  Pretty much, she does not technically have TMJ, but the muscles that support the joints are weak and sore, which is causing that pain, though it is also possible to have pain there from a Chiari I malformation.  He said that given her history, this is likely an early warning sign of TMJ arthritis.  He pointed out that her bottom jaw is not growing the way that it should; it is a little small, so when she is 16 they will likely yank a few teeth to make room for her molars and give her braces to help extend the jaw.  Hopefully by that point she will have grown, and maybe her jaw will catch up by then.  The roots are NOT in the maxillary sinuses- just close to.  However, those are only her 2nd molars, and there is no room for them.    

So, yeah, pretty much in a nutshell, Friday sucked.  The icing on the cake for me was finding out that they messed up when they did our taxes, and we are getting back $300 less than what we were told.  Yeah, and that refund check will definitely not be here by this week.  We should have had our refund by now, but this is how we roll.  

There is so much to process, and figure out.  Hopefully we can get the ball rolling this week.  If she wasn't in pain daily, not able to concentrate, than I would maybe not worry about it.  They say that most people that have a Chiari I malformation have no idea that it is there, but of course she does.   It really makes me sad that everything happens to her.  I now cling to the knowledge that she may go into remission on the MCTD front later on in adulthood.  She really needs a break.  Please pray for Emily that we get all of this taken care of without the need for surgery.  Thank you.