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

World Autoimmune Arthritis Day!

Greetings!

I would like to invite you all to come join me for a live blog chat/ interview from 7 P.M. to 8 P.M. EST for World Autoimmune Arthritis Day.  Because it covers several countries and time zones, the event starts on May 19 and runs until May 21st.  There will be many, many features.  Here is a little intro video for it.  Here is the official YouTube event video.  

This link brings you to the home page for World Autoimmune Arthritis Day!  The page puts it this way:

 "Have you registered yet for World Autoimmune Arthritis Day 2014 (WAAD14)?  WAAD14 is 100% ONLINE, which means you can join the convention from anywhere there is an internet connection.  It is designed for ALL people to attend, regardless of location and physical lim itations- all you need is a computer.

The convention site is built just like you are at a physical convention site, with booths to visit, Exhibition Halls, live Presenters (via "Live Chats"), and specialty features, such as this years' major event: A Day in the Life of an Autoimmune Arthritis Patient where patients can invite supporters to 'walk in my shoes for a day' by participating in challenges that mimic those faced by patients on a daily basis. " 

I was a part of this event two years ago.  This non-profit was brand new back then.  They have come a long way!  The founder, Tiffany, realized quickly after her initial diagnosis that people did not understand autoimmune arthritis at all, and that everyone seems to equate it to osteoarthritis.  While the two couldn't be more different, it takes educating people to make them understand it.  This reason is also why I blog.  It started out as a way to vent, until I realized that I was helping people.  I can assure you, that is NOT what I expected, but it's pretty cool!  :)  Anyway, please join me!  If you can't join me, there are many other people who are also doing live chats and interviews.  You have the opportunity to have questions answered.  It's volunteer run, and really has become quite the event.  Please join us if you can!  The registration info is below.  I hope that you can join us!

$5.00 USD Pre-RegistrationEntrance Fee to Live Event

May 19^th 6am ET/USA-May 21^st 5am ET/USA

& Access to Booths & Resources through May 31^st

$7.00 USD Entrance Fee During Live Event

May 19th 6am ET/USA-May 21st 5am ET/USA

& Access to Booths & Resources through May 31^st

FREE to Skip the Live Event

Access to Booths & Resources from May 21^st at 5am ET/USA through May 31^st

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.

Pain management and coping techniques

Pain Management and Coping Skills

Pain management has been somewhat of a hot topic lately.  Since I am on a little vacation, now seemed like a great time to talk about it.  When one has chronic pain, one needs to figure out what works for them to help deal with it.  We have found several things through the years.

Baths and water activities

Emily loves her baths.  Warm baths have a way of taking some of the tenderness out of sore joints and muscles. Sometimes we use epsom salts, which are known to help quite a bit.  Sometimes we use a scent that she really likes, like lavender.  Sometimes we use arnica oil, which is also really good for drawing out pain.  On really bad days she will use the epson salts in combination with an oil.

Water activities, like aquatherapy or water aerobics, are also quite helpful!  The joints are supported better by the water, causing less pain but a harder workout.  Many people that won't do a traditional workout will at least go walk around in the pool.  It is especially useful for people that have severe osteoporosis in their knees.

Paraffin baths

There's nothing like hot wax to help take the inflammation out of the hands.  Paraffin baths come in a variety of sizes, some with adjustable heat settings.  We have also found that you can add things into the wax, such as soothing oils.  We added some lanolin and essential oils to ours.  The longer you can hold the heat in, the better it will work.  It can be used for hands and wrists or feet and ankles.

Essential oils

Yes, we were very skeptical at first.  We really did not put much faith into them to begin with.  I know children that need morphine.  While they really needed it and it wasn't really by choice, it was an option that I did not want presented!  Lidocaine patches have worked for others in the past, but they did not work for my children.  We were running out of options, so we thought we would take a chance.  I still cannot believe how well the doTERRA Deep Blue works!  We use many oils now for many things, but our favorites are definitely the Deep Blue, and their headache blend, Past Tense.  Emily has a lot of headaches lately, partly because of her cortisol levels, partly because of the new hormones, and partly because of the sinus pressure the poor kid has in her head.  The two oil blends have helped her more than anything.  Also, the Deep Blue took her knee pain down to almost nothing.  (Please feel free to PM me if you are interested!  I am NOT a salesperson, but I can sell them if you are interested.)

Biofeedback and psychology

I had never heard of biofeedback before, but it's actually pretty cool.  When I was a kid, I was involved in every possible theatre class and production around.  My wonderful music and drama teacher in high school taught us relaxation techniques.  I tried to teach them to Emily, but, well, my last drama class was 1991.  I searched for apps, but at the time I couldn't find them.  I kind of let it go as a lost cause, until we started seeing our pain management doctor.  She is NOT the type who wants to push pills.  Quite the contrary; she did not want to add pills.  She decided to send us to biofeedback and psychology both.  Child psychologists are not the easiest to find, but child psychologists who deal with chronic pain patients seemed to be even more difficult.  We finally found one, but we had a 2 month wait to be seen.  We squeezed biofeedback in around psychology.  This was the coolest thing!

Maybe it isn't as amazing with adults because many adults have their minds set that this will not work.  (That is what our specialist told us, anyway.)  They talked.  Emily was hooked up to monitors.  Yeah, really.  They attach sensors to the sweat glands, and then show you how nervous or calm you are on the screen.  The great thing is that we could see that when we mentioned broccoli, her stress level shot through the roof.  (Seriously.  It's bad.)  However, after learning a few techniques, she could also see her stress level go down.  The cool thing about that is she could see how she could calm herself down.  The great thing is that these are techniques that she can use for the rest of her life.

Movement

I know- nobody wants to hear it.  I know I don't.  But it's true.  The days that I don't move, I tend to hurt more.  The days I make myself go to the gym, I have more energy and feel better.  If you can strengthen the muscles around the bad joints, the joints will have better support and hurt less.  Besides, we all know it is when we stop and try to re-start that we hurt the most.  Emily has to play on the Wii Fit at least three times a week.  I try to hit the gym as often as I can.  And I kick Zach outside to play with his friends.  

I have an amazing friend, also from high school, who has lupus.  She has had more back surgeries than  I can bear thinking about.  She is running marathons.  Yeah.  She totally puts me to shame.  I've been thinking lately that if she can do it, I can do it.  Right?  Maybe in time....

Stress Balls

For our hands we use stress balls.  Squeezing them can help to relieve some of the discomfort.  It's also good therapy.  Using your hands as much as possible is very helpful.  It can be hard when your hands are aching, but even wiggling them is good.  

Any activity is good activity! 

Free time!

I apologize for my slacking off when it comes to posting.  I am finally on summer break from school!  It's been a very tough semester.  I actually failed a class and then got a B in my other class.  I don't approve of either, but I've had quite a bit to adjust to.  I didn't even realize that it was stress until the semester was almost over.  I'm praying this doesn't kill my honor society status!

If we were the type to give up, we would have given up on growth hormone quite a while ago.  However, we stuck with it and she finally got it!  It has been about 2.5 weeks.  The down side is that she has had a major headache from it, so we agreed to skip a few days to see if the headache would go away.  We are hoping that when she re-starts it, her doctor will approve a lower dose, and then gradually move back up.  We have already seen some of the benefits:  improved sleep, for one thing.  It is also supposed to strengthen bones, which is wonderful considering that all of the years of prednisone can cause osteoporosis and brittle bones.  I know that this is the right step; we just have to find the right mix here.

She has another sinus infection.  If you read the last post, you may recall that we had a runaround trying to get her into the ENT for a swab and suction.  She ended up getting better spontaneously on her own before we could get the authorization.  Now she's sick again.  She just had 30 days on Augmentin.  She was sick again after a few days.  Now she is on Clindamyacin again.  I have another call into the ENT.

The other bit of Emily news is that, while we were at our pediatrician's office, she tells me that her throat is "bulging" again.  Apparently it is difficult for her to speak, and to eat.  The eating is hampered, but she also has too much reflux coming up, too.  None of the usual meds seem to be helping, so I emailed our doctor to let them know in advance.  This is a hospital week.  It should be interesting.  They are supposed to test her cortisol levels to see if we can start dropping hydrocortisone, and now they will also run an upper GI to see what is up with her throat.  Also, she has been telling me this week that her muscles are bugging her.  That could be from the growth hormone (we are hoping!), but it may not be. The last two visits I noted that her muscle enzymes were elevated.  Not a huge amount, but definitely increased.  I am hoping this will be just a fluke.

Zach is doing pretty well.  Now that he's had a steady supply of Enbrel he is doing better most days.  He's had a few days with a lot of back pain, a couple with knee pain, but overall he is good.

We are going to try the Paleo diet.  I am definitely going to try the Whole 30 on the advice of two friends.  While Emily is one of the pickiest kids in the world, and she would rather starve than eat broccoli, I am hoping that if I lead by example, they will follow.  I really feel like this could help, but if I push, then I have no chance.  I am not looking for a cure or a miracle; I'm not setting us up for a fall.  However, I see correlations with food myself.  I know that soda makes me more tired, it breaks me out, and I feel yuck.  I recently realized that is the same for most sugar.  I feel worse when I eat certain things.  I had chips this weekend and now I hurt everywhere.  I'm almost positive it's from the chips.  So, perhaps if we cut all of that out, things will be better.  The essential oils have been amazing, too!  There is an oil for everything!  Our favorite is doTERRA's Deep Blue.  Especially when we use a friend's concoction and add more wintergreen and frankincense to it.  Amazing!  Now I see that many of my migraines were from my neck.  It's been a fascinating couple of months!

I am hoping for a calm summer since we didn't get to do anything fun last year.  This year I am hoping that we can go out on Friday's to do fun stuff!  After this week...  I will try to post from the hospital when we know more.  Take care!