The GI appointment

Before I begin my post with our news, may I ask you to pray for our friend Parker and his family?  Parker is having a very, very tough time right now.  He needs as many prayers, positive thoughts, or anything else you can throw his way, as he can get.  His family needs some peace and strength, too.  You can follow his story here.

Okay, so when Emily had her swallow study a couple of weeks ago, I knew it wasn't right.  For a minute there I thought they were playing with the camera.  I realized that more comes up than goes down when she drinks.  I actually cried watching the camera, thankful that she couldn't see me.  She was standing up, swallowing hard, and still nothing would clear until she had some water.  Not what I wanted to see.  

Because our rheumy group is amazing, they set up an "emergency" appointment with a new GI doc.  The nurse practitioner emailed me about that Wednesday, and by Thursday we had an appointment set for the following Monday.  I worried a bit about how the doctor would be, but when I pulled up his profile I saw that he was the head of the department for feeding disorders, and he writes the curriculum for the school that his office is affiliated with.  

The first thing that the doctor asked me after he introduced himself was, "how can I help you?"  For just a minute there, I was worried.  I explained that I just want her to be able to eat.  He said that he had already looked through her file.  He asked me if anyone had mentioned a problem artery to me.  Ummmm, no.  Apparently, she has what they call an "aberrant subclavian artery".  This means that there is an extra artery that snakes around behind the esophagus.  (Below is the exact picture the doctor showed us.)  This by itself can cause all sorts of really awesome problems like chronic respiratory infections and problems swallowing.  The NIH Rare Diseases page  says this about it: 

 Aberrant subclavian artery is a rare vascular anomaly that is present from birth. It usually causes no symptoms and is often discovered as an incidental finding (such as through a barium swallow or echocardiogram). Occasionally the anomaly causes swallowing difficulty (dysphagia lusoria).[1][2] Swallowing symptoms in children may present as feeding difficulty and/or recurrent respiratory tract infection.[2] When aberrant subclavian artery causes no symptoms, treatment is not needed. If the anomaly is causing significant symptoms, treatment may involve surgery.[1][2] Children with symptomatic aberrant subclavian artery should be carefully evaluated for additional vascular and heart anomalies.[2]

So, because that's never quite enough with us, he also points out that just because she has that doesn't mean it is the culprit.  It is likely from the scleroderma side of the Mixed Connective Tissue Disease, but it could also be gastroparesis, which is where the stomach doesn't empty properly.  For more info on this, I went to my trusty friends at NIH again.  

How can we distinguish which problem may be causing this?  More tests!  This was one of the two things that we somewhat expected.  Em & I had talked about how she would probably need another upper endoscopy so they could really figure it all out.  (We assumed it was from the esophogeal separation, and would need to be stretched with a balloon.) Not only does he want that done, (minus the balloon) but he also wants a different type of swallow study done.  Instead of drinking barium, they have you eat something like eggs with radiation.  (UGH!!!)  If we didn't really need her to eat I would be against this, but we have to figure out what needs to be done.  Hopefully she will have these tests with her next hospital overnight.  We go back on June 2nd & 3rd.  On June 4th we have her endocrinology follow-up; it will be the first one since she started the hormones.  

She is still having some wicked headaches, but we think that they may be from the sinus issues.  Tomorrow she will be heading to our ENT's office for a sinus culture and suction under anesthesia.  We are hoping he may have some answers for this.  I feel like she may need a prophylactic antibiotic.  She is sick more than she is well.  Perhaps that won't be possible with the artery issue.  Maybe she is just going to have to deal with being sick constantly forever because of it.  I don't know.  I discovered that if she has gastroparesis, sometimes low doses of erythromycin help.  I hope she does not have gastroparesis, but if she does maybe we can kill two birds with one stone there.  Hopefully by having her appointment now, her IgG levels will be high enough to not knock her back, and there will be enough time between this procedure and her endo appointment to really be able to tell if the headaches are sinus or hormone.  We are also hoping that her levels are staying high enough consistently for them to stop her IViG (platelets via infusion at the hospital).  That would eliminate the overnight hospital visits, and really make our lives much easier.  We shall see, I guess.  We are staying hopeful that we are on the right path, just with our usual bumps in the road.

Zach is doing well.  :)  For that we are very fortunate.  I will update more when we know more, or to point you towards Parker's page for his next update.  Please pray for all of their family and ours?  Thank you.  

Picture credit:  Sonoworld.com

Our last hospital visit. Ugh.

I've had a few days to ponder and mull now.  I still feel a little numb, and just exhausted.  My Facebook friends know most of this, but I didn't want to leave anyone out.

I had emailed the nurse practitioner at our rheumies office last Monday, in preparation of our visit that Thursday and Friday.  She spoke to the doctor, and we all agree that she needed a new swallow study done.  Emily had told me that she feels like there is a bubble in her throat when she tries to swallow.  Hmmm.  Since she had started to get stuffed up again (on antibiotic number 2 for the month), I mentioned that, as well as the headaches she has been having.  I assume they are either from the sinus infection from hell or maybe from the growth hormones.

I wasn't going to tell her about the upper GI until right before.  I asked for Xanax for her.  She never uses that stuff except for medical testing.  She just freaks out.  It's bad.  I guess that everyone thought I was overreacting.  Nope!  It was supposed to be outpatient at 11, but knowing how difficult it would be to get us out on time, they made it inpatient at 11:30.  She woke up on Friday morning to the nurse telling her it was time to go downstairs for the test.  She got herself so worked up that she almost hyperventilated.  She cried for a good hour.  She didn't calm down until someone finally got her a 1/2 a Xanax, but it was too late by then.  She was rescheduled for 3:30, though no one told the scheduler downstairs.  We were discharged at 1:30, so we had time to kill.  We couldn't really leave, and since she couldn't eat, we tried to avoid all of the food places.

They told me to give her another 1/2 Xanax around 3.  Despite having one full Xanax in her, when they took her back she still had another hour meltdown.  I can't blame her.  She has no control, no say in any of this.  It sucks.  If I didn't suspect it was bad, I never would have pushed.

After she finally calmed down, she worked with them to help herself.  She asked to be slightly elevated.  I watched the barium start to go down, then back up, several times.  At one point, I thought the camera was showing something else, or glitching.  Nope.  What it basically showed is that she has, as the nurse practitioner emailed it, "Her study showed severe reflux and little motility of her esophagus."  Hmmm.   I suspected as much, but it still hurts to see it.  At the moment, that is really all that I know.  This is in conjunction with her patulous esophagus, which if you missed it, means that the muscles at the base of her esophagus have dilated and spread away from her stomach.  .  Well, this explains why she doesn't eat.

At the same time, she has spent 30 days on Augmentin and 10 days now on Clindamyacin, and she is still sick.  She had an x-ray last week that showed she is still gunked up, so the ENT is working on getting an authorization from rheumie to get this expedited so they can culture her and suck out the junk up there.  I really, really think she needs to be on a prophylactic antibiotic.  Then maybe we could stop IViG, and maybe not have to sleep over the hospital every month.

But, wait!  There's more.  I have noticed for the past couple of months that her ALT (muscle enzyme) has been a tad elevated.  I'm no longer the one to panic at the first sign of trouble, so I've just been keeping an eye on it.  However, Monday her thighs hurt badly- like a 6-7 on the pain scale.  That could mean that her dermatomyositis is also coming back, which would mean adding Prednisone back in.  We really, REALLY don't want to do that.  It makes me so sad.

If all of that wasn't enough, they ran her cortisol levels on Thursday morning before she had any of her hydrocortisone.  A normally functioning adrenal system will have a count of 10 or higher.  She was only at 1.3, so the hydrocortisone stays for now.  And the final kicker was the email from camp that she is on the wait list for both sessions.  At least we had a few good months.  Hopefully this will all pass quickly.  I guess we need to find a set Gastroenterologist now.  We've seen a couple before, but mostly as favors to our rheumies.  We don't really have anyone set.  Hopefully endo will be able to help her more with the cortisol, and ENT will see her fast to help her sinuses.  Please pray that all of this comes together just right.  Thank you.

Lots to be thankful for

Sometimes it hits me how surreal our life is.  Sometimes I realize how not-normal our "normal" is.  Sometimes I feel worried, scared or nervous about how our kids are doing, but then we walk through this cancer ward and it really puts things into perspective.  We see mothers crying in the hallway because their children are given a terrible prognosis.  Children that look more ill than any old person that I have seen are not even allowed to leave their rooms for fear of germs.  These are the days where I really embrace all that we really have.  Our home life is better than probably 80-90% of families out there.  I love being home with the kids more; I rarely have to raise my voice.  We all feel happy when Ash comes home from school or Kevin comes home from work.  That is how it should be, but I suspect it is rare for many other families out there.  I don't just love my family- I like them, too, and we enjoy each other.

Today has been a really good day at the hospital.  I feel like we've gotten some answers.  While Zach's ankles are still not great, and he has a bad spot in his back, he's doing pretty well overall.  We were talking and thinking back to when one of the doctors here first looked him over at camp.  He was a mess.  He had huge red sores (plaque psoriasis) all over him.  It was horrible.  Enbrel has given him a chance at a normal life in multiple ways.  Thankfully, 2 years down the line it is still helping him.  We got him a prescription for physical therapy because I'm not sure how to help his pain when he is walking a lot or playing hard.  I have some ideas, but I know that his issues are different from mine and what works for me may not for him.  I'm hoping that will help him to gain some endurance, too.  Otherwise he is doing very well!

We established that Emily's Pulminary Function Test came back with no problems, so that is great.  I thought it would be okay, but one can never be sure.  I spoke to the doctor at length about how her pain levels don't seem to jive.  She still hurts a LOT, and it keeps her from wanting to do much of anything.  We agree that some of it is her age with the hormones starting, some of it is muscle memory, and the adrenal insufficiency isn't helping.  We also discussed the growth problems and how growth hormone may be the key to calming her pain down.  To put this into perspective, I am 4', 9.5" tall.  I have been this height since I was 10.  Emily will be 12 on December 6.  She is 4', 1.5" tall.  I don't want her to be doomed to shortness, but I also believe that those cells not regenerating are storing pain in the nerve endings.  If her joints are good, and her muscle strength is good, then it makes sense.  Some of it is that she needs to stretch more.  We have discussed this often; I know that she is trying, but I think we need to switch it up a bit.  Her knees were bright red for months.  Today they actually looked good, but they still hurt.  I've been telling her that I think her IT bands are tight.  They just need to be stretched and she'll feel better.  It's so hard to get her to want to move.  In any case, I will take this to our previous appointments.  I think that the hydrocortisone is helping quite a bit, so hopefully she will start to feel even better from that soon.  It's a slippery slope but I have hope that soon this will all be easier for her.

One good thing is that this doctor really sees why we need endocrinology.  It makes me feel less like a paranoid, over-reactive mom.  I really try hard not to be like that.  I try to approach things with as much distance as I can, and from a clinical approach.  It can be hard, but I put a lot of thought into everything before we make decisions.  Em & I talk about it, and then Kevin & I talk about it.

As far as I know, tomorrow should just be a fast release home, but we shall see.  Sometimes things sneak up at the last second.  I still have to give our doctors here a copy of her 24 hour urine test that nephrology ordered.  It's all over the place.  Almost every section is flagged.  We see nephrology and pain management next week, and we are awaiting an appointment with endocrinology closer to home.  The endo that we saw once is leaving the hospital, and since it was two-three hours away we wanted to find someone close.  I will let y'all know how those appointments go.  I am praying endo will see her very soon!!!


*Friday note:  Dropping to 0.3mls of steroids!!!  She has been consistently on steroids with no break since October 2010.  Again, she started at 42mgs, which was about 7mls twice a day.  UGH!!!!!  She has come a long way, but finally there is an end in sight!  WOOT!!!  This is exciting!

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.