Thursday, February 13, 2014

Little update

I can't believe how long it has been since I blogged!  Since last I wrote, we have seen the ENT to discuss a plan for the next time she gets sick.  The plan has been that we call ENT when she gets sick to get her in for a culture.  I really wanted them to culture so we could figure out what antibiotic would actually work. While they are in there, they want to suck out the extra crap that seems to never leave her sinuses in her forehead. I'm so over trying different antibiotics and her still getting sick when we stop.   Unfortunately, that time is now but it hasn't all lined up.  They sent the authorization to the wrong fax number, then when our rheumies got it, they only could make out half.  Hopefully since we are at our rheumies now, that will get situated.  She also recently saw endocrinology and had her growth stim test, which she failed miserably.  We have an MRI scheduled for next week to rule out pituitary problems before we start growth hormone.

As of today, Emily has the go ahead from our rheumies to STOP PREDNISONE!!!!!  That is HUGE!!!  She has been on this steroid consistently since October 12, 2010.  She has come a long way!  She started on 42mgs.  She was on the liquid, which is 15 mgs per 1 teaspoon (5mls).  She was on 7mls twice a day, and currently she is on 0.2mls.  I told the doc's that I would prefer to taper, so it will be a week or so before she is fully off.  I am sooooooo excited!!!  So is she!  At this point, the risk outweighs the benefit.  I showed our rheumie the cortisol levels from the growth stim test, and she was floored at how low they still were, despite hydrocortisone twice a day.  The down side is that we have to increase hydrocortisone temporarily, but it is only temporary.  She is still on a plethora of other immune suppressors, but eliminating one is a good start!  No other news today, but if anything else has changed we will know by tomorrow.  Her MRI is set for next Friday, so hopefully we can coordinate ENT before that.

Zach had an appointment today, too.  We have been fighting to get his Enbrel for over a month now.  Thankfully I tend to hoard medicine a bit, so we had some extra.  I've been spreading them out, giving it to him every six or seven days, instead of twice weekly like it's supposed to be.  Apparently it showed.  Our rheumie and nurse practitioner both noted that he has more back pain and tenderness than he should.  If his back is still that bad in 3 months at his next appointment he will have an MRI to see why.  They also pointed out that he is hypermobile.  That is one of those things that I don't (yet!) know much about.  All I know from my fellow JA mom's is that it can be quite painful, and it can cause enthesitis, which is basically like inflammation of the tendons.  Sounds like a good time, eh?  Poor kiddo.  Obviously, all of this shows that he is NOT ready to go off of Enbrel.  In fact, we are also going from Methotrexate pills to the injection.  I am really hopeful that once we get his Enbrel back on track, he will start to do better.  Through the week it's hard to remember that he has arthritis, too.  These are always painful reminders.

I'm doing pretty good.  I'm finally off of Medrol (like Prednisone).  The only down side to that is the neuropathy coming back, along with the joint pain.  While my rheumy feels that my Sjogren's may turn into something else, his nurse practitioner is hopeful that it will not.  I'm choosing to think she is right.  :)  In truth, aside from my right hand going numb every morning, and my one bum shoulder, the worst is the chronic fatigue.  I am always sooooo tired.  That aside, I am holding up well.  I'm thankful that my symptoms are mild enough that they don't prevent me from doing what I need to do.

And that is that.  I will try to find the time to update after the MRI and other important appointments.  Thanks for following!  :)

Wednesday, November 27, 2013

And then there were three

A few years ago I started to notice some things about myself.  I was turning pretty white when it got cold, I would get random pains bad enough that I almost drove us off the road a few times, and chronic fatigue.  The fatigue was bad, really bad.  I didn't have time to deal with it, so I pretty much just ignored it for a while.  Until my shoulder decided to rebel.  And then my knee stopped working for a few days.  Then my hips started popping every day, and aching often.  And my ankles started to feel like glass shattering when I would stand up.  Eventually I brought it up to my doctor.  He quickly dismissed me.  He did a fast strength test on my hands and decided I was fine.  After all, I was too young for Rheumatoid Arthritis, despite having two children with Juvenile Arthritis.  Since it really took a lot for me to say anything at all, and my symptoms weren't bad, I didn't really argue.  I told him that I was pretty used to being invincible but something was starting to happen to me.  I told him I felt like we had to catch it before it got bad because I don't have time to be sick.  He nodded politely and sent me on my way. 

Eventually I gave up.  He wasn't listening.  By this time I had new stuff- my right hand was going numb every single morning, and my right foot would go numb when I was about due for my vitamin B shot.  Eventually I noticed it would still go numb after my shot.  Then my hands started "cramping".  That started to affect my work and my schooling.  I knew it was time to move.  I finally got myself a new primary care doctor, with a little urging from several JA friends.  I guess at this point you could say that I wanted to be tested but I didn't really believe I had anything serious going on.  I mentioned my kids' illnesses to the new doctor, but I didn't tell her I suspected RA for me.  When I started telling her about my hands she immediately asked if I had been tested for it.  I explained that our old doctor didn't feel it was possible.  She actually listened.  I felt totally comfortable with her.  It was nice!

Even though she felt that I probably had the start of it, I still dragged my feet on seeing a rheumatologist.  My labs weren't pointing clearly to anything in any direction, and I still didn't feel like I was bad.  I finally made myself go two weeks ago.  My rheumatologist was hoping that steroids would do the trick and it would disappear.  He said that he sees about one person a week that has such a mild case that steroids alone kick it out and they never come back.  I hoped I would be that case!  Nope.  My shoulder is about 50% improved, but my wrists tonight are aching enough to keep me up.  My knee is still not happy.  So, they also ran additional labs.  They ran a few that I hadn't seen before.  I didn't really have time to look at the prescription, but we talked about it Monday.  I tested positive for both Sjogren's tests.  And suddenly it all makes sense.  The neuropathy, the lighting bolts I sometimes feel shooting down my arm, my anemia, and even my thyroid are likely all related to it.  And then there is the leaky eye that I thought was a blocked tear duct.  That is most annoying.  I have made my peace with it over the past 24 hours.  At least I have some answers.  We are going to try Plaquenil to see if it helps.  It may take 4-6 months to kick in, but if it helps to keep me from getting really sick it is worth a shot.  The PA said that because I don't have high inflammation counts I will not likely see deformities or even a lot of degeneration of the joints.  I guess if I have to have RA, this would be the way to go.  I'm a little sad, but I am determined to stay on top of it and beat it.  My kids have been such examples for me.  I have to be strong for them.  

I still have like the best family ever.  That really helps to keep me sane.  My kiddo's are all amazing in their own way, and I cannot even believe how much I love and like my husband.  I still consider myself blessed.  Just burdened.  :p  

I am spending the next two days cooking!  I hope that everyone has a wonderful Thanksgiving!  Take care & God bless!

Thursday, November 14, 2013

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!

Wednesday, October 23, 2013

Three years of JM and MCTD

It was September 2011 when we first realized that Emily was very, very sick.  We had gone to her former rheumatologist who had kind of given her a diagnosis... without any solid evidence to prove it.  After blowing us off and telling us to come back in three weeks, we begged a new rheumy to see her.  It was October 13, 2011 when we got the definitive diagnosis.  We didn't get home until late on October 19th.  Six days of hospital time- upper GI, MRI, lots of labwork, EKG, PFT, daily steroids and anti-inflammatories, and a lot of sadness.

Since then, we have been through a lot.  We had the whole Prednisone double-the-weight-in-two-months thing happen, which was horrible all around.  It caused major depression for her, and ripped our hearts out as we watched her eyes swell shut.  We have found out about her throat muscles not working due to dermatomyositis, and then later we found that the scleroderma features are causing the esophagus muscles to spread, leaving a gap between it and her stomach.  We have found out about early changes labeled as mild interstital lung disease.  I saw that at our pediatrician's while reading the newest note they had sent over.  We've had low cortisol and adrenal insufficiency, which contributed to causing kidney stones.  As yet we have not resolved any of the above, with the exception of the stones.  At this point, her pain continues to increase, likely because her pain amplification has come back.  Her medicines just keep increasing.  Below is the list of meds and vitamins that she has to deal with daily.  I print this every time we see any doctor.  I print triple copies when we have a hospital stay.  You never know what kind of a resident you are going to have!

Despite this, we still have hope.  Hope that one day she will be in remission.  Hope that there will be no more progression.  Hope that her pain levels will come down, as they continue to be an 8-9 (on a pain scale that goes from 0-10).  Hope that she won't need to use one of those electric carts whenever we have to go to a big store.

We had an issue the other night at a big box store.  I usually try to avoid this place, but I promised my oldest daughter that we would upgrade her phone there because the AT&T stores no longer carried the one she wanted.  We went in, all of us dressed in business clothes since we had just come from my honor society induction.  We chose an electric cart to help Emily get through the store, and we set about on our way until a greeter stopped us to tell us that she wasn't allowed on the cart unless she sat on my lap.  I pointed out that they were there for disabled people, and she was a disabled child. She said that children were not allowed to ride them regardless.   I am not quick to anger (except maybe at work), but I was livid pretty quickly.  I asked to speak to the store manager, or a manager on duty.  I offered to go to the car and bring back our handicap permit, but Emily decided to show off her port scar and educate this woman.  She claimed that it was company policy, but she did not call the manager for us.  Instead, she told us that she would "let us go this time".  Nice.  I wrote on their Facebook page, and their corporate Facebook, who directed me to a feedback link.  I know you can't see her illness, which makes this our job to educate.  We have never had an experience like this before, and we hope to never have another.  I ended my post with "my child has likely suffered more in the last three years than this greeter has her entire life".  And it is so true.

All in all, we are managing.  For me personally this year has been pretty amazing, but it really sucks to see my little one suffer.  It just shouldn't have to be like this.


Conditions:  Mixed Connective Tissue Disease, Dermatomyositis, Juvenile Arthritis, Raynaud’s Phenomenon, Scleroderma, Vasculitis Rashes,  Esophageal Dismotility
Current meds- last updated October, 2013
*Prednisolone- (15mgs per 5ml) Take 0.5 mls in the morning
Hydocortisone 5MGs- Take one tablet twice a day
Prilosec (Omeprazole) 40mgs One capsule daily.
Procardia ER 30MG (Nifedipine)- One pill once NIGHTLY. (For Raynaud’s)
Mycophenolate 250MG (generic CellCept) 3 pills A.M. (750mgs), 250mgs P.M.
CVS Iron  65mg- 1 pill once daily
Zyrtec- 10mgs nightly
Amitriptyline HCL 20MGs- Take one tablet nightly at bedtime.
Symbacort 160/4.5-    Two puffs twice daily  (as needed- through winter and sickness)
Sucralfate 1GM- Take one half  tablet twice a day  
Xanax .25 mg- Take 1 half of a tablet as needed for anxiety
Lidoderm 5% patch- apply as needed 12 hours on, 12 off  ******** NEW*********
Multi-Vitamin & 2500 Citracal 400mgs calcium with Vitamin D 500 IUs– once daily
Vitamin D- 1000 IUS, 2 fiber gummies per day
***IV Infusions:***   
Remicade 325mgs monthly
Rituxan 400mg- Every 3 months

IViG Gamunex 10% (15 grams)

Thursday, October 3, 2013

Would YOU like to win a book?

My wonderful friend Kim has written a parents guide to dealing with JA.  There is a contest happening RIGHT NOW that can help you to win a copy!  The contest ends November 1st.  Just go here: 

I promise I will update soon.  I have just been swamped with school work, and honestly we are still in that "I don't have a clue what is happening with this disease" chapter.  I guess it's the hormones trying to work in her that are making things go crazy.  I have no clue.  I will be back when I have some idea of what is happening.  As it stands, she hurts pretty badly every day.  That hasn't changed at all.  Her head, her joints, and sometimes her muscles.  It's been a really tough year.  This too shall pass............

Wednesday, August 28, 2013

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.

Thursday, August 1, 2013

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!