renewed hope

March 2012


March 2013

Last Easter our egg hunt was in the hospital and Emily was so sick she couldn't walk or stand up from sitting without help. Today she has an egg hunt with her Kindergarten class and she was hopping around class when I left her. Easter seems an appropriate time for good news, we have a renewed sence of hope.

Emily's white blood cell count dropped to 16, still elevated, but finally coming down instead of moving between high (20) and high high (30+) (labeled HH on the results). To give you some perspective on what that means; normal levels are 10, 16-18 means you're sick with a virus or infection, 20+ for prolonged periods means something is seriously wrong and 30+ is when they start looking for cancer.

We aren't weaning steroids until we get lower, but moving in the right direction and our next infusion is Monday.  The rash has popped up a couple of times this week, but it's been very short lived and Emily seems to be more active than ever since her onset so today we have reason to be optimistic. 

I won't even say remission yet, but I could see us getting off steroids by next year and then maybe once we get to that point infusions will not be so frequent.  We will still take it a day at a time, but we have reason to be hopeful this Easter and we thank our Heavenly Father for this second chance for our daughter. 

So many ways...

This disease has changed Emily's life in so many ways.  This morning my husband made the mistake of using the word skinny and Emily began feeling bad that she was not skinny, like she used to be.  She means she doesn't look the same anymore; the steroids cause puffiness which gives Emily the appearance of being overweight.  In fact when she take her anywhere and she rides in the stroller, like the zoo yesterday, people will often stare at her and I feel like they are probably thinking something like "Why is the child in a stroller, she should be walking" or perhaps the term "childhood obesity" goes through their mind.

Even with the weight gain Emily is only in the 30% for height and weight so she is not overweight at all and she is very active, but until we shake the steroids completely we won't be able to shake the puffiness.  Even once we do get off steroids we won't be able to shake this nasty disease.  It's here to stay and there are so many things it impacts.  Going somewhere like Disney Land, that would be a monumental task because steroids + exhaustion + stiffness and pain from an all day adventure = super tantrum or at the very least extreme whininess. 

We have to be careful what we say; we don't talk about weight (or try not to) instead we talk about getting plenty of exercise and eating healthy.  We don't talk about Emily having children, instead we are sure she understands that adoption is a great way to become a mother too and we encourage her to learn and say maybe you could be a doctor or a scientist someday.  Someday we will have to explain to her that these new medicines could make is hard for her to have children.  I imagine when she marries that will be a scary point for her, when her husband and her decide to try she will wonder...

Dentist visits are another thing we learned yesterday.  Cavities are common with this children because they are immune suppressed and cavities are caused by bacteria.  The medicines can also reduce saliva in the mouth and make her teeth and enamel more susceptible to decay.  Now she has a cavity to fill and I don't want to put her through that, but we have to because cavities are because by bacteria and we need to get rid of the bacteria.

We talk about finding the good out of the bad.  I'm afraid I have yet to find the good out of this nasty disease, but I still have my sweet girl and if this had been five or ten years ago I would not and so I can at least say treatments have made progress.  I wish more people knew what this did to these sweet children.  I read a post in a Facebook group about a SOJIA child who tried to commit suicide.  These children are in pain all the time.  It breaks my heart to think what that mother is going through right now.  We've just got to keep showing Emily all the good in life to help the puffy badness of SOJIA maybe not seem quite so bad.  I hope we can do that as she gets older.

Steroids

So even through Emily's inflammation numbers still show as "high" on her blood work results all is well and she will be completely off steroids this week yay!

I thought I would take the time to show you how much the steroids have impacted Emily's appearance.  For a five year old that's a big thing.  She has asked me if she will ever look the way she did before getting sick and I hope the swelling goes away, but we just don't know.  Steroids are a life saver of an SOJIA child.  They work fast and well so we very well may have to use them again sometime in the future.

April 22, 2012 (Emily was diagnosed in March 2012) so this is right after her discharge from the hospital

May 6, 2012


June 6, 2012


The Mormon Battalion is a free place Emily loves to visit (panning for gold is a favorite) and we always get a picture when we go there so this shows how much she changed in two months.  Weight wise she was discharged at 30lbs (about the 15% percentile) and by June she was 50lbs.  She nearly doubled her weight in two months; I can't imagine.  Even after nine months of pregnancy I have never doubled my weight and to have done it only two months.  Clothes size she went from a 4T to a 10. 

The good news is that she has started losing weight.  It's coming off slowly, but we've been told that is normal as her activity level increases and her appetite and the steroids go down.  She's now only 48lbs and in a size 6 and definitely more active.



I'll do another post like this is a few months to show how coming off steroids effects her as well.  That's one question I had when this all started; will the steroids changes be permanent?

A little bit scary

Today we realized Emily will have completely weaned off her steroids before her next Rheumatology appointment.  That first brought feelings of excitement and relief.  Then I got scared because every time we have tried to come off steroids something horrible has happened.  Then I remind myself that was at the beginning and things have been going very well lately so I take a deep breath and build up my faith a bit.

The truth about it is though that this nasty disease is just all around scary.  Whenever a new SOJIA parent message me on Emily's facebook page I remember all the emotions that go along with suddenly having a very ill child in the family.

Once you come so close to losing a child you realize there is no guarantee on life.  Each new day is a gift that you cherish, but each new change is a little bit scary too.

Rheumatologist

We had our bi-weekly visit with Emily's Rheumatologist and my suspicions were right.  She's been more tired then usual and I had a feeling as we were weaning down on the steroids the disease was becoming more active and labs confirmed this.

There are two different inflammation markers the doctor looks at; one tells if MAS is coming back and one tells us about SOJIA and how active the disease itself is. 

It's now I have to remind myself there is no cure only control through medication.  The medication she is on right now is Prednisone, Cyclosporine, and Kineret.  Kineret is the long term medicine that is supposed to control everything and eventual be the only medicine she takes.  Prednisone is like a band aid medicine, it's what she got in the hospital because she was so very sick.  Prednisone reduced the inflammation quickly and controls it very well, but the side effects are pretty nasty (think best case two hip replacements eventually) and can be life threatening if taken too long so we have to get her off it as quickly as possible. 

We found out yesterday her D-dimer, white blood cell count and platelet counts were all elevated which means SOJIA is still with her and becoming more active as we wean down on the steroids.  Good news was her feritin levels were low, not normal, but not 24,000.  Normal is around 60, Emily's now is a little above 100.  In the hospital with MAS they were 24,000.  So anyways MAS is under control; SOJIA not so much.

I'm worried it's not working as well as we wanted it to, but happy we can try a different medicine.  This one is given via IV in the hospital twice a month.  They can also do labs at the same time so if the IV goes in well that means just two pokes a month...hallelujah!  There is a huge emotional toll that is being paid and if we can reduce that cost just a bit I will take it.  Let's just hope this new medicine works.

How to manage a SUPER tantrum

'roid rage is a live and well at our home.

Most days Emily is very good at controlling it, but sometimes it is just too much for her.  I understand how they make her feel a lot better thanks to Take Me Home from the Oscars; Arthritis, Television and Fashion By: Christine Schwab.  In her book Christine talks how the steroids affected her and I can see some of those same things in Emily.  Trouble sleeping and anger are the big ones we deal with.

So last night when that angry monster got the better of her I employed some technique that I thought I'd share.

First; I have to recognize where the tantrum is coming from.  Part was her not getting her way, part was the steroids.  I blame the steroids but she could also just be frustrated with everything that has happened to her lately.  I know I am frustrated just watching this disease and all the side effects from the medicine.

Super tantrum stages:
1. Whinny, super whinny with a touch of "I'm about to scream in anger." in her voice.
2. Anger, screaming at the world.  Sometimes with words, sometimes just loud screaming.
3. It all comes off.  In the hospital she would throw the pillows off her bed, last night she threw her clothes.  This is the glorious half-way point.
4. Sad crying, no more screaming just tears and sometimes a; "I want my mommy".  This is where mom or dad swoops in to comfort her and tell her it's going to be OK.

So to handle this I put on my patient hat and wait out the first three stages.  My husband and I will acknowledge her anger and even empathize with her, but we make sure she knows how she is dealing with it is not acceptable.  She does not get what she wants.

At the last stage we swoop in and comfort her and talk about it.  Why did she feel so angry?  Why did she scream?  How can we handle this differently next time?

They have improved since we left the hospital, but any tantrum is not fun in my book.

Hungry Monster

The steroids make Emily constantly hungry.

Emily's meal requests:
pumpkin pie
noodle soup with egg in it
mini pizza's

She will usually requests all three (not always in that order) for each meal.  The steroids cause tummy trouble so I am usually not picky about what she wants, but this super weight gain (15lbs in two weeks) has me worried.  So in an effort to curb her eating and add nutrients we require she has one yogurt for breakfast, 10 carrots for lunch and dinner and before any snack she has to have something healthy usually carrots or raisins.  She whines and complains about this and then I wonder why am I forcing more calories into her, because she eats what I require and then what she requests.

So some of you will say, force her to do what you want.  This is a girl who is capable of a four hour screaming tantrum on two doses of calming medicines.  Seriously I have to pick my battles here and an super tantrum over dinner is not where I want to go.  Really, should you force your kids to do anyhing.  I don't believe that is why we are hear on earth so I have requirements and then teach.  We do a lot of talking about healthy eating and encourage daily excersise. 

That is another reason I want a companion dog.  Someone to encourage her to get up and move.  We'll see, we did find one place that will give us a dog and help with funding it, we just need the doctor to sign off on it.

Moon Face

These photos were taken within 6 months of each other.  The before picture was taken right before my deployment.  I wanted to show how drastically the steroids have affected Emily.  I guess they call it "moon face", but I bet if you didn't know any better you might first think the chubby girl in the stroller was just fat and lazy.  People stare a lot now, I just hate thinking they are judging us and Emily.  So I make a new effort now not to judge; I give everyone the benefit of the doubt because you can't judge a book by it's cover.

Twinkle, Twinkle Little Star

Night is not my favorite time of the day.  Emily does not sleep through the night and honestly never really did.  So I have a hard time believing parents who tell me their six week old sleeps through the night.  Really?  Neither of my kids ever have.  My two year old is getting better and there are some nights he does go all night, but even that took two years. 

Emily hates nights too.  I am not sure if it's the disease, or maybe the medicine, or perhaps just this whole traumatic experience, but she will cry during the night and usually wakes up to get a new ice pack for her injection site and then another time to go to the bathroom.  If I am on the top of my game I try to include both things in one wake up.  Usually I am not.  Usually I am lucky if I can be and loving mother at those times.  Not that I am mean to my child, but I am most definitely not happy to be up that late/early.

The worst part is when she just wakes up crying.  It must be some form of a night terror because she won't tell us what is wrong or respond to us.  So I just lay next to her waiting for her to wake up more or stop and tell me whats wrong.  The thing about the crying that makes it hard to deal with, for me, is that I can't take away what is making her cry.  I can't stop giving her the injections (lately those have been very hard for her to take) and I can't make this nasty disease go away.

Super Tantrums

My goal with this blog is to tell the whole story.  Each child's story with JIA is different and SJIA is even more different.  I tell my husband that Arthritis should not be the name for this, just a symptom.

My daughter has been taking prednisone, a steroid, to control the inflammation, and I hate it.  Yesterday when she woke up I could see the puffiness in her face had increased dramatically and then there was the grumpiness.  I am reminded of the book Alexander and the Terrible, Horrible, No Good Very Bad Day. Only in this version I watch it play out in my daughter's life and I have no idea how to help. 

My husband and I talk to her about how the medicine can make her feel angry, but that doesn't make it ok to kick and scream and throw and all out fit.  In the hospital I came up with the phrase, super tantrum, because that is what they are.  Can you imagine your child screaming for you to go away, for everyone to just leave her alone for four hours?  It is a horrible experience. 

We use a sticker chart and reward system now that we are home.  With nine stickers she can earn a trip to the History Museum, Science Museum, time to color some hard boiled eggs (a favorite activity) or an ice cream from the ice cream truck.  She has options to save up and get other stuff like mail-order lady bugs (yes they have them), but she loves to spend her stickers as soon as she saves them up.  Right now she can earn up to three a day one for no tantrum in 24 hours, one for holding still during her injection (she gets it no matter what because sitting for a painful injection is hard for anyone let alone a five year old) and then if she does some excersises she can earn one more.

This is a nasty disease.