To talk or not to talk

One thing we have struggled with is when to discuss different aspects of this nasty disease with Emily.  She's only five years old so it can be a lot to take in that young, but when you have to spend 57 days in the hospital and hear announcements like "code blue room xyz" topics like life and death tend to come up.

At one point Emily decided she would not take any more medicine.  We were still in the hospital and she just decided she was done.  It was at that point we had to sit down and explain the very nature of her arthritis and how the medicine was saving her life.  20 years ago MAS killed and doctors couldn't explain it.  30 years ago SOJIA was called Rheumatic fever and baby Aspirin was the only regimen.  Those children who didn't lose the battle then grew up in wheel chairs and I've watched as some of them have lost their battles as adults.

We found because this was happening to Emily she needed to talk about it.  She needed to understand it was a very serious matter and so it helped put things in perspective for her.  She still doesn't understand it all, but when she introduces herself to another child she will say, "I'm Emily and I have arthritis." Because it is a part of her life now and will be forever.  She is learning to live with it and live around it.

Because talking helps we look for ways to encourage that conversation.  Emily has a drawer filled with medical play items and she enjoys watching Doc McStuffins, a Disney show about a child playing doctor.  We've found it especially helps to talk about other children going through similar experiences.  We met a dog the other day with only three legs and so I made a point to talk to her about how brave the dog must have been to go into the dog hospital and how he had to get shots and IVs too.

Since we may have to re-introduce nightly injections the point of talking about it has come up once again in our lives.  Emily is not happy about the possibility, but she knows it may happen.  We talk about it once and then don't worry about it.  She knows and if it happens we will revisit the discussion and importance of them.  We will also have to explain why the infusions didn't work which will be harder, but we'll figure it out.

Lately Emily has been mimicking Doc McStuffins and even has a door sign to show when she "doc" is in or out.  I think Emily would make an amazing doctor someday, she would be able to relate to her patients and I even told her perhaps she could be the one to cure arthritis in children.

The "doc" is in:

The "doc" is out:

When the child is living the trauma talking is very important.

Doctor

There aren't enough Rheumatologists to treat the 300,000 children in the U.S. There are only about 220, according to the Arthritis Foundation.  Emily's case is pretty sever so we didn't experience the normal 6 month wait to confirm a diagnosis and we've always had priority at the office, but most of these children wait 6 months or more.  I couldn't imagine.  We first heard Still's Disease two weeks into her first hospitalization.  I believe that doctor saved Emily's life because by the time we got her into the children's hospital she was in heart failure and taking a turn for the worse quickly.

Whenever Emily goes to the Rheumatologist it is not a quick, easy visit.  The time with the doctor is quick and usually only takes about 20 minutes, but we wait and wait and wait.  This last time we waited 1.5 hours in the exam room (which is torture for small children) because her normal doctor is on maternity leave.  By the time we saw the doctor for our 10:30 a.m. appointment it was noon, we were all hungry and Emily had drawn all over the entire exam table paper twice.  Our two year old had finally slid his way over the entire room floor (picking up who knows what type of germs) and was considering drawing on the actual exam table with a pen (ok, I may have allow him to draw a line or two, but hey don't shut us in a 10x10 room for that long with a two year old).

We are lucky, some children have to drive hours to see a doctor and some have no choice but to see an adult Rheumatologist and that's difficult because the adult diseases are different from the children's.

We need more pediatric Rheumatologists. 

Life Definitions

Health

This morning on the way to work I heard a commercial about staying healthy and I found myself wondering if anyone really wants to be unhealthy.  I mean health is defined by merriam-webster as "the condition of being sound in body, mind, or spirit; especially: freedom from physical disease or pain" so when I hear the children shows on TV telling kids what to do to be healthy I feel a little pain.  By the definition above our Emily is not healthy.  She is happy and active and alive, but not free from disease and pain. Emily did not choose this nasty disease.  Somehow it found us through genes or virus or a combination of the two or perhaps something entirely unknown (idiopathic means of unknown cause) so we don't talk about being healthy so much as we talk about doing good things for our body and helping us feel good even with arthritis.  We have a new job as parents; we have to teach Emily how to live with this her whole life. 

Motherhood

Someday she will need to understand the medicine she is taking to control her arthritis may keep her from having children of her own, she will need to understand and make peace with that. So being a mother will have a different meaning for her.

Life

Life does have a different meaning know.  Little things don't bother me so much because life means we still have our sweet daughter to hold.  This disease has killed before and the possibility is always present in our minds; so life has a new meaning.

So many new meanings; so many ways to say something and look at life.

Happiness

After Emily was discharged from the hospital she was sad.  She couldn't do things she could before she was sick.  She was so weak walking was difficult and she couldn't jump or run at all.  I was so worried she would hate life.

I remember overhearing someone say they would commit suicide if they got fat and lost their hair due to another similar disease and all I could think about was what if that is how Emily feels.

I am very happy to report she has been her happy self since I got home from my last work trip.  She is running, smiling, laughing, jumping and playing like she used to.  She might be a little slower and not as strong as before, but she doesn't seem to even notice it any more. 

As a parent I decided if I could teach my children to love life I would have done a good job.  It's still my hope that in spite of infusions and doctor visits and steroids and arthritis Emily will love life and find joy.

flare

With autoimmune arthritis (I specify that because osteoarthritis is completely different) symptoms can come and go.  When symptoms are at the worst they are said to be flaring.  It's not something we have really ever experienced until this weekend.

Emily had been off steroids a little over two weeks when we started to notice her rash come back and stay.  It has already been around (I say it is mocking me), but this time it was different because it was more prominent and itchy.

At her last infusion I noticed her lab numbers were not looking good; d-dimer and fertin where on the rise and hemoglobin was taking a dip, all classic symptoms.  So I called the Rheumatologist and asked if the rash and these labs meant that MAS was back.  MAS is macrophage activation syndrome (I wrote about it here) and it is scary because there is a 20% mortality rate associated with it if not treated.  About ten years ago MAS didn’t even have a name; it was a fever that killed because there was no treatment so it’s not something we like.

Sure enough we get a call back, it was MAS.  Hello steroids, it hasn’t been long enough.  We started her again on 2mls per day, not too bad, but enough to invite ‘roid rage back and increase her energy.  It wasn’t enough though, four days later the rash was worse and she was crying it was so itchy…ugh.  So we were told to raise the steroids to 3mls twice a day.  Not that bad, not the 10mls we were on, but it feels like we took one step forward (ending steroids) and two back (6mls a day was months ago).

It seems to be working.  Emily is her old self; she had not been acting right at all this past weekend so I knew something was up. 

Flares are common with this nasty disease and they can happen anytime.  The one thing I find assuring is that for the most part the worst part of the disease is the onset.  So hopefully Emily will never be that sick again.

First day of school! First day of School! First day of school!

Emily’s first day of school was this week and boy was it rough, on me.  I did not like how it felt to realize she will be depending on others to learn and grow now as well and from me and my husband.  I’m not a big fan of outside influences in the world we live in today.

Aside from how I felt, Emily really seemed to enjoy it.  She even had her teacher reading from Taking Arthritis to School.  We know because we had to interrupt the reading to pick her up for her infusion. 

First day of kindergarten + Actemera infusion = bed time at 5:30 p.m.

I’m still trying to decide if I was more upset about my sweet girl growing up and moving on or because there are so many germs and ways to get hurt at school.  What if someone comes to school with the Chicken Pox or Strep Throat or a cold?  Too many worries.

Her teacher was very nice though and after we discussed our concerns with her suggested we write a note home to all the parents telling them about Emily’s suppressed immune system. We did.  How much of an impact it had we may never know although if Emily does not come down with any viruses this year we will credit the letter with success and try again next year for sure.

Emily does enjoy school, but we can tell it is hard on her.  She comes home exhausted, usually immediately falls asleep and then doesn’t want to go back.  Since she’s been sick she doesn’t like doing things that require a lot of work so school and swimming lessons are on the top of her list right now. We’ve taken it upon ourselves as her parents to push her to work though; it’s part of learning to live with this nasty disease.  Even though she wakes up stiff and in pain she’s got to move past it to live so we are trying to teach her how to cope (warm baths and extra early bed times) and how to suck it up (sometimes life just hurts). 

It’s a learning experience for me.  I hate negative people and believe anything is possible, but trying to instill this belief into a five year old is not very easy, especially when she learned earlier this year that there are things you just can’t do when your body attacks itself, at least you can’t do them until the medicine kicks in.

Our new rule for this school year is every time Emily says “I can’t.” she has to stop and say “I can” five times. We’ll teach her she can even if she doesn’t want us to.

 

Here’s to the rest of the school year being good and Emily getting strong enough to take on first grade.

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.

Sometimes we almost forget...

Since I've been home there have been a few times that I have almost forgotten about SOJIA.  That is a big step in the right direction.  I don't know we'll ever really forget, but watching Emily smile a lot more and just be a happy child and enjoy life is lovely. 

She is noticing her progress as well.  The other day she asked me to watch her jump.  That was her goal for physical therapy; to "jump again" and she was so pleased with herself when she did it.  Last night she wanted to ride her scooter to the play ground.  Usually going to the play ground means we have to push her there and back (a total of two blocks), but last night she rode/walked her scooter the whole way there.

There are just so many things we took for granted before SOJIA.  It's nice to see Emily happy again and enjoying her childhood.  My one wish for her now is to just love life no matter what happens, enjoy being alive and the thrill each new day brings with it.

smaller battles

Emily's tootsy

One of the scariest aspects of SOJIA is that the medicine controlling it also suppresses Emily's immune system.  I read posts and blogs of different families dealing with this and there is always one thing that plagues the child.  Emily has been battling ingrown toenails.  This will be her second case of both big toes getting infected.  Normally you could just wait it out, but with a weak immune system she goes right on antibiotics and if those don't work they increase the dose.  After two weeks we're now going to go to a pediatric podiatrist to see what they can do.  There are lots of small battles to fight with SOJIA.

Infusion Time

When Emily was originally in the hospital for SOJIA it was difficult for her to deal with.  As you can imagine the emotional toll we all paid was very high, but Emily's more so then any of us.  Every once and a while during those 60 days I would try to cheer her up and encourage her to get up and move with a visit to the gift shop. 

Shopping therapy. 

On one trip Emily picked out this tiny little ladybug, so cute, but so easily lost.  Her original one did get lost so for her infusion today daddy took her back to the gift shop and she picked out two cute little guys to replace the first one.  Her request was for a ladybug and a turtle.

We believe it's the tiny things that make a big difference.  We want her to love life and so when she wants to replace her tiny lost ladybug we do our best to make that happen.  Life is great, even with SOJIA, and we want her to believe that too.

Parenting at a distance

You may or may not know it, but when Emily first got sick I was on the other side of the world for work.  It was difficult for me to hear she was sick and not getting better.  I'm sure it was scary for my husband who was the one at home dealing with it all, but not being there, having absolutely no control and not even being able to ask the doctors all the questions I had was so hard.  Then when things took a turn for the worse my work rushed me home.  That 13 hour flight was the worst because at least when I was in the office I could get a call from my husband if there was a change, but on the flight there were no phones and for 13 hours I honestly worried that I may have held Emily for the last time months ago and didn't even known it.

I'm gone again for work and being away is surreal.  All of my work items where packed away after I rushed home the last time and still have things the way I had left them.  The book I was reading when Emily got sick still had the sticky note with the nurse's email from her first hospital; my digital picture frame has photos all pre-SOJIA.  It's almost like SOJIA was a bad dream and I just woke up back in the office, although it's a new office and this time I am only a six hour flight away, but sometimes I have to remind myself it really did happen.

It's another dimension to all of this, not bad, just different.  So this is why I have not been posting on Tuesday and Thursday; I am parenting at a distance which consists of lost of wishful thinking and updates from my husband. At least this time there is no fever and there is no rash.

Parenting at a distance

You may or may not know it, but when Emily first got sick I was on the other side of the world for work.  It was difficult for me to hear she was sick and not getting better.  I'm sure it was scary for my husband who was the one at home dealing with it all, but not being there, having absolutely no control and not even being able to ask the doctors all the questions I had was so hard.  Then when things took a turn for the worse my work rushed me home.  That 13 hour flight was the worst because at least when I was in the office I could get a call from my husband if there was a change, but on the flight there were no phones and for 13 hours I honestly worried that I may have held Emily for the last time months ago and didn't even known it.

I'm gone again for work and being away is surreal.  All of my work items where packed away after I rushed home the last time and still have things the way I had left them.  The book I was reading when Emily got sick still had the sticky note with the nurse's email from her first hospital; my digital picture frame has photos all pre-SOJIA.  It's almost like SOJIA was a bad dream and I just woke up back in the office, although it's a new office and this time I am only a six hour flight away, but sometimes I have to remind myself it really did happen.

It's another dimension to all of this, not bad, just different.  So this is why I have not been posting on Tuesday and Thursday; I am parenting at a distance which consists of lost of wishful thinking and updates from my husband. At least this time there is no fever and there is no rash. 

The stages of an infusion

 

1. Anxiously waiting

 2. The IV, includes screaming and lots of stress for all involved

3. Exhaustion

The infusion is no fun, Emily hates being back in the hospital even for a few hours, she hates the IV and let's face it those hurt, she is always asking about when the next one is and how long till it happens.  "How many days till my fusion?" is the big question on this sweet girls mind.  It seems to be working so far, other then being tired a lot there is no rash or fever present so we are happy about that.  We also talk about how she has no more shots.  Since the Kineret burned so much we try to tell her how this is so much better the infusion is, but I don't know that helps much.  Nothing about this nasty disease is pleasant.

No exceptions!

After Emily was officially diagnosed with Arthritis I started looking into swim lessons.  The Arthritis Foundations recommends swimming as a great exercise for your joints and so I figured if I could bother Emily and her brother deep into swimming early perhaps it would stick with them.  At the very least they would know how to if they decided that was their exercise of choice.

Once she was out of the hospital I began looking for programs.  Anything private or semi-private was out of our budget so I turned to the YMCA.  It's still $50 per child for only four lessons, but that's better then $180.  Emily's brother is at risk for JIA in general because it's probably genetic and he is our child too; besides that we aren't going to leave him out so that doubles the cost of us.  I've tried teaching them on my own, but I don't have access to a warm indoor pool and we all get cold easily in the outdoor pools here so it becomes cold and unpleasant very quickly.

Anyways, I called the local YMCA to see about signing up Emily for some lessons.  The ones for her age group were without parents in the pool and since Emily is still pretty weak I knew she'd need some extra help so I figured it was as simple as telling our story and getting permission to be in the class with her.  No.  It's not allowed. No exceptions.  Not only that, but the Tai Chi class the YMCA has that is sponsored by the Arthritis Foundation is not for anyone under 15.  No exceptions.  In fact the YMCA made absolutely no exceptions at all.  I couldn't get a manager to call me back to talk to me and the only class I could get into the water with Emily was the infant class.  At first I figured that would work out because her brother is only two and would be in it with me, but we ended up paying $100 for one lesson for just Emily where she was completely ignored by the instructor.  It was horrible.

First, Emily was sick with a cold for the first two classes.  I can’t risk getting her sicker when she has a suppressed immune system.  So I thought perhaps they would understand our situation and allow us to reschedule for another class.  No, no exceptions.  Then it turned out the class was during a holiday weekend so there went another class, they just canceled it.  Finally we made it to the last class and it was an epic failure.  Emily's brother refused to even get into his swim suit.  I paid $50 for him to watch class and another $50 for the instructor to ignore Emily.

Emily and I got into the water a little early to try to get her warmed up.  Anyone with arthritis or joint problems knows warming up is important.  Not at the YMCA, that is not allowed.  No exceptions. We had to get out of the pool and wait five minutes for class to begin, wet and cold. Finally class begins and the instructor comes around to each child.  I tried to explain to her why Emily was a five year old in an infant class, all I managed to get in was that Emily has arthritis and is not as strong as other kids her age.  The teacher took Emily from me, sung the ABCs to her, returned Emily to me and then ignore Emily the rest of the class.  Leaving me to try and get Emily to do the kicking and bubbling on my own.  She skipped us each and every time she went around the class after that.  Finally Emily was not enjoying herself and I was beside myself with disgust so we got out and left.  I didn't even change out of my swim suit.  It was a horrible experience to realize that even though you ask for help and seek out things that will help your child there are places where there are no exceptions.

My Emily

Last week I took Emily to get her haircut.  As she watched her reflection in the mirror I felt like I could see pain on her face.  She was not happy with the way she looked.  Later that day she asked me if her face would ever look the way it did before she got sick.  The swelling is what I consider severe, but we can't get it to go down until we get off all of the steroids.  With 7ml still each day getting to 0 seems like an eternity away.

We have had to be mindful of her self image now.  We no longer tell her she is a big girl, we say she is growing up.  I like to tell her how wonderful motherhood and marriage is, but now I just talk about marriage.  I realize that these treatments are so new and so life altering they may very well take away her ability to ever have biological children and I have no doubt that may be very painful for her one day.

The doctor seems to feel that it's ok for kids to be on the medicine because they aren't aware of how it changes them physically, but Emily is very aware and an intelligent child.  I wish we had a doctor who had a child dealing with this,  not because I wish it on someone else, but because there are so many daily living aspects to this disease that the doctors just don't get; such as the physical appearance and extreme exhaustion.

See, the thing is; you don't really understand what it's like until you live with the disease.  None of us understand except Emily.  You really can't fully understand what it's like to parent a child with a chronic illness until you have one. 

"It's just arthritis."

My husband and I made this video because we had heard the phrase; "it's just arthritis" too many times. It's a good synopsis of what we have be dealing with in the past four months.

I made it because there are days when my mind tries to trick me; Emily seems perfectly fine and I wonder if the medicine isn't doing more harm then good.  Then I look at the pictures and video and remember back to the days in the hospital when I couldn't count how many doctors we talked in a matter of minutes and the two days when a doctor actually sat outside Emily's room and told me to come and get them if "anything changed". 

I also made it because I wanted to show Emily that she isn't the only child with arthritis and so we have watched video after video on You Tube made by parents watching this nasty disease take hold of their children and change their lives.

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.

Even the Best Laid Plans

Ever since SOJIA nothing has really gone the way I have planned it.  Parents don't plan to have a child suddenly become severely ill and so it kind of catches you off guard.  It's like being hit in the chest.  Knocks the wind out of you and even after you recover from it you still feel like you can't quite catch your breath.  Some days it's harder to breath then others.

We took Emily to a counselor today because there has been an emotional side to this illness.  She has an adjustment disorder that is being manifest through anxiety.  The doctor was very nice and helped us put this into words to better communicate it with her school, but it still gets me down.  I think because I remember that this is for the long run.  Once again I am reminded there is no magic pill, the medicine never ends, there is no cure.

Sometimes I feel like screaming to the world; this isn't fair.  We did everything right; this shouldn't be happening to our baby so make it stop.

That's putting the breathless feeling into words.

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.

Our daily dose of pain

Sometimes the daily injection goes well and sometimes it does not.  Last night it did not.  Emily tried to grab the needle twice.  I have to assume it is the anxiety about the pain that makes her do this because no rational child would try to grab something they know is very sharp and would hurt their hand as much as any other part of their body.

In this video the patient talks about having adult on-set Stills, same thing as SOJIA except in an adult, and he cringes before each Kineret injection.  He says it hurts if he touches the injection site and even three hours after it will still hurt him without toughing it. 

Emily screamed in anger after the shot last night.  It was painfully loud (I'm holding her during this).  Then I think about the fact I have to travel for work for two months very soon and my husband will be the one.  His wonderful sister is giving a week of her vacation time to come out and help when I first go, and it's only for two months, but I already know what she will be screaming; "I want my mommy!" and that will be so loud it already hurts my heart.

We've talked to the doctor about this and we could eventually try a weekly infusion instead, but that would be time in the hospital once a week.  It sure wouldn't hurt as bad so we may go that road, but we have to wait until I get back from my travel and Emily has come down more on the steroid dose.  Then there is still a chance she may relapse because the IV medicine could take up to a week to start working.  Then there is also the fact that Kineret is supposedly the best there is out there for SOJIA especially.  Would we be risking her health only to improve her quality of life slightly?

I'll leave you with something I read last night because it seems to fit (although I am sure Emily would not think this trial very "light" and to me is feels heavy too).

2 Corinthians 4: 17 
For our light affliction, which is but for a moment, worketh for us a far more exceeding and eternal weight of glory;

Scentsy party

Scentsy Party ID 87232069

One of Emily's wonderful Facebook friends (Emily's Facebook page) is having a virtual Scentsy party for us.  I always thought Scentsy products were fun and sweet smelling so if you need something sweet anyways go check out her page.

We'll use the money to help train Pearl.

Happy shopping. (Emily and I love ourselves some good shopping therapy too.)

$ The cost of a chronic disease $

I can be honest and say we have good insurance that covers most of our medical costs.  The difficulty comes from the fact that unconventional things are what help JIA kids most.  We have Emily taking physical Therapy twice a week which she hates and insurance covers and swim therapy once a week which she loves and insurance doesn't cover.  We wanted to get a YMCA membership because they have an indoor pool, but since we choose to keep our children at home and out of day care we can't afford it and can't apply for the YMCA scholarship because both parents don't work. 

There is another JIA girl who I heard takes dance class six days a week to help keep the stiffness at bay.  Movement does help, but of course insurance only care if it's a medical condition.  So they will pay for a wheelchair if we want to just give up and let JIA win.  Since we don't just want to let JIA win we have sacrificed other things.  We were considering buying a second car when Emily started kindergarten, now we won't.  We'll be inconvenienced a bit and save the money we would pay on insurance, maintenance and a car payment so we can continue water therapy and buy food for Emily's new friend Pearl.

On top of the unconventional, but successful treatments, we have to pay to park each time we go to physical therapy or the doctor and then most of the appointments are at least two hours if not more which usually cuts into a meal so we either rush home and pop something in the microwave while hungry, irritable children growl or stop to get something to eat and reduce the stress load a bit.  That all costs extra money.  It's not something you think about. 

When Emily was in the hospital they fed her, but not us.  Sometimes I would just eat her leftovers because she wasn't eating much.  Most days, except on Saturday and Sunday, the Rhonald McDonald house would feed us, but when Emily was in the ICU I would have to leave her to get food.  I ran back and forth and lost weight not eating as much.  One or two days in the hospital is no big deal, but 57 and the meal bills add up.

We also have the co-pays for her medicines and then we have to pay for the alcohol wipes, syringes, gauze and band aides.  Emily doesn't like the feeling of the alcohol wipes so since it's already very traumatic we bought some gauze to dry her off before we give her the injection, but that's expensive and we may stop doing that and have to have her suck it up and deal with the wetness for a few seconds.  We hate doing that, but what can you do?  We don't go on family vacations, we hardly travel, my husband and I never go on dates any more and unless we're at the doctor appointment during a meal we never eat out.  We don't buy new movies or go to the movies, the last time my husband or myself bought new clothes was over a year ago. 

JIA has not only taken some of my daughter's mobility it has also taken our money.

Tuesdays and Thursdays

I've decided to post here every Tuesday and Thursday and when I need a little extra writing therapy.

Emily had a mommy day yesterday.  I am in the military and in-between deployments so I figured it was a good way to ensure some quality time.  She loves to come to work with me so I brought her to work and then we left early to get her blood work done and go to a baseball game with my office.

Blood work was quick, but not painless.  Quick is good though, I hate sitting in a room full of what could be sick kids with my immune suppressed daughter waiting for some stranger to inflict more pain on her.  Unfortunately because the medicines can have so many adverse side effects and even the disease itself can harm her other organs (we saw it hit her heart and lungs already) every time we go see the Rhuematologist we also have to get labs done.

After labs we headed to the ball park.

I had called ahead of time to be sure they would allow stroller in the park because Emily can walk, but not far.  They do, however the nice gentleman I had explained her condition to failed to tell me that we would have to check it into Guest Services which is not close to our seats at all.  Then of course we had to walk up and down the stairs to get to the seat.  Emily didn't want to stay in the stroller, why would she when we had seats right behind third base, so I encouraged her to walk down the steps.  She did a good job and had a good time.  The thing I noticed was it is a lot of work to go to a game.  She made it up the steps twice, but only with my help and it's not just holding her hand I actually had to reach under her arms and allow her to put most of her weight on me as she stepped up.  She only made it two hours too.  By the 5th inning she had stopped talking and that's how I know how tired she is.  She gets so tired she won't even talk, just sit really still as if she is trying to conserve her energy.

There is a kind-of sort-of mass transit system in San Diego that's a red trolley so being from D.C. and knowing how awesome the metro is I figured we could just ride that home.  Emily enjoyed the "train ride", but that was also a ton of work.  There are only steps up to the car so I had to ask if we could use the handicap elevator to get into the car and the driver didn't believe me that we needed it.  He did end up letting us use it, but I don't think we'll ride it again.  I feel for any disabled person that depends on that for transportation. 

Last visit we had asked the doctor to sign a form allowing us to get a handicap placard for our car and she told us that we were no different then any other family with two children.  It doesn't feel that way and sure I want my daughter to get all better, but she can't walk far or fast so the stroller is our only option right now.  I'm so tired of being asked for help and told no.  On the one hand I understand the doctor doesn't want us limiting our daughter, but let's be real, this is a long process and she's not going to be running around anytime soon.

So far we've been told she's not sick enough to get a wish, not disabled enough for a dog and we don't need a handicap placard because we're just like other families with two kids.  If that's the case then why did the two year old at the ball park beat my five year old as we walked back to our seats?  If that's the case why does Emily get so tired that at times she doesn't have the energy to talk?  Perhaps I should invite the doctor to go to the next ball game with us and stay a day at our house and then tell us that.

Will you...

Awareness has been our biggest hurdle.  This not "just arthritis", it's not osteoarthritis that the elderly tend to get.  It's an autoimmune disease that is wreaking havoc on 300,000 children in the U.S. alone.

Will you share our story on your facebook page?  Will you like Emily's facebook page.  Help us tell others that kids get arthritis too.

https://www.facebook.com/ArthritisIntrospective#!/SystemicOnsetJIA

Side Effects

Every time I give Emily her medicine I think about the side effects.  Short and long term; I wonder if when she wants to have children someday if she will be able to.  A six year-old boy died last year from complications of all these medications so I worry about that too.  The thought that I could be hurting her as much as this nasty disease is always in my mind, but what choice do we have?

Prednisone

Get emergency medical help if you have any of these signs of an allergic reaction to prednisone: hives; difficulty breathing; swelling of your face, lips, tongue, or throat. Call your doctor at once if you have any of these serious side effects:

blurred vision, eye pain, or seeing halos around lights;

swelling, rapid weight gain, feeling short of breath;

severe depression, unusual thoughts or behavior, seizure (convulsions);

bloody or tarry stools, coughing up blood;

pancreatitis (severe pain in your upper stomach spreading to your back, nausea and vomiting, fast heart rate);

low potassium (confusion, uneven heart rate, extreme thirst, increased urination, leg discomfort, muscle weakness or limp feeling); or

dangerously high blood pressure (severe headache, blurred vision, buzzing in your ears, anxiety, confusion, chest pain, shortness of breath, uneven heartbeats, seizure).

Less serious prednisone side effects may include:

sleep problems (insomnia), mood changes;

acne, dry skin, thinning skin, bruising or discoloration;

slow wound healing;

increased sweating;

headache, dizziness, spinning sensation;

nausea, stomach pain, bloating; or

changes in the shape or location of body fat (especially in your arms, legs, face, neck, breasts, and waist).

Cyclosporine

headache

diarrhea

heartburn

gas

increased hair growth on the face, arms, or back

growth of extra tissue on the gums

acne

flushing

uncontrollable shaking of a part of your body

burning or tingling in the hands, arms, feet, or legs

muscle or joint pain

cramps

pain or pressure in the face

ear problems

breast enlargement in men

depression

difficulty falling asleep or staying asleep

Some side effects can be serious. If you experience any of the following symptoms, or those listed in the IMPORTANT WARNING section, call your doctor immediately:

unusual bleeding or bruising

pale skin

yellowing of the skin or eyes

seizures

loss of consciousness

changes in behavior or mood

difficulty controlling body movements

changes in vision

confusion

rash

purple blotches on the skin

swelling of the hands, arms, feet, ankles, or lower legs

Taking cyclosporine or cyclosporine (modified) may increase the risk that you will develop an infection or cancer, especially lymphoma (cancer of a part of the immune system) or skin cancer.

Kineret

Injection Site Reaction

Upper Respiratory Tract Infections

Headache

Nausea

Diarrhea

Sinusitis  

Arthralgia

Flu Like Symptoms

Abdominal Pain

Kineret has been associated with an increased incidence of serious infections.

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.

Exhaustion

Out of sheer exhaustion I let Emily sleep in my bed last night.  I just am not able to function well waking up 3 to 4 times a night, getting up at 0530 for my work out, working all day and then getting back home to clean, cook dinner and spend time with both children.  I am pooped.  If I am honest too I don't clean much in the afternoons and dinner is so low key I'm not sure you can count it for much effort on my part.

It's kind of ironic because now we will have to work on getting our son to sleep in his own bed to make room for his sister so I can get some sleep.  The irony comes from the fact that we worked to get Emily out of our bed when our son was born. 

If we want to have any more children we will need a larger bed.  Which brings me to another point that bothers me.  Before SOJIA my husband and I were ready and wanting another child.  I was looking forward to coming home from deployment and starting an adventure leading to a third child.  Having a child who is so sick and will be for a long time sure cured that desire.  Eventually we will probably have another child, but I thought it might be a possibility this month and completely freaked out.  Probably because of the exhaustion; up three times with Emily, once with Thomas and let's see with a newborn that is about four to five  more times a night.  I would never sleep.

I feel guilty that I got upset about the possibility, especially now that it's not.  Derek handled it much better then I and was even excited about the possibility so maybe in a few months we can consider it.  Once we are better at managing SOJIA we can think about it again.  I'm not getting any younger and I think there is at least one more member of our family waiting to join us.  I'll have to talk myself into it.

Unconventional

I want to get one of these for Emily.  There are actually two types of specially trained dogs that I believe would really help her.  One is a service dog to help her move around and pick things up from the ground, the other is a therapy dog which would be her constant companion, especially during the injections, and would be trained to obey her and go with her to the playground to play.

They cost a lot of money and apparently juvenile arthritis is not debilitating enough to qualify for help in getting one.  I haven't given up yet though.  One organization told me she qualifies, but we'd have to raise the funds for them.  Not ideal with working full-time, one sick child and one going through the terrible-twos adding something else to my schedule doesn't seem like it would work either.  We'll have to talk about it some more.

So far the only other thing that helps Emily is the water therapy which of course we pay for ourselves because insurance doesn't cover unconventional things like that or therapy dogs.  Oh, but they would cover a thousand dollar stroller/wheelchair.  Let's not get her up and moving, let's confine her. 

On a positive note, Emily went back to her dance class yesterday.  I was of course very worried it would be an epic failure.  All that movement, especially the up and down, I was afraid it might discourage instead of encourage.  She really wanted to though and my husband and I have said from the beginning of all this that we would not limit her, she would have to set her own limits.  So we took her, I went in the class for the first half (all in uniform and everything), because she was anxious and I was not going to let the very perky teacher brush her off.  After the first 30 minutes the teacher could see what her limitations were and Emily seemed to be more comfortable so I left to watch from the door (plus they changed from their tap shoes to their ballet slippers-tap shoes are very slippery and I didn't want any broken bones).  Emily seemed to really enjoy it.  She tried everything, even just a little except for the jumping, and even did a forward roll with a lot of help from the teacher.  She also got down and laid on her belly which she refused to do in PT.  So I was happy about that.  Of course afterward she is exhausted so we took it easy the rest of the day, but I was happy to see her enjoy herself.

I hope to make a lot of progress this next month because I will be leaving for two months for work again very soon and don't know how much progress if any will be made while I am gone.  Derek is great with the kids, but Emily get's really upset when I leave and with this nasty disease I don't expect she will be willing to do much more then wait for me to get home.

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.

Thrush

WHAT IS THRUSH? Thrush is the term we use to describe an overgrowth of yeast in a baby's mouth. This yeast, called candida, normally lives within our mouth, intestines, and genital area. It thrives in moist, warm environments. Occasionally this yeast is allowed to overgrow so much it becomes visible as white patches. The Rhuematologist warned us we may see some thrush because of steroids. Emily had a super case when she was an infant. Seems like it's back and here to stay. I hate these steroids.

Don't Quit

I like to read this poem three times when things feel at their worst.

When things go wrong, as they sometimes will,
When the road you're trudging seems all uphill,
When funds are low and the debts are high,
And you want to smile but you have to sigh,
When care is pressing you down a bit,
Rest if you must, but don't you quit.

Life is queer with its twists and turns,
As every one of us sometimes learns,
And many a failure turns about,
When he might have won if he'd stuck it out.
Don't give up, though the pace seems slow -
You may succeed with another blow.

Often the goal is nearer than
It seems to a faint and faltering man;
Often the struggler has given up
When he might have captured the victor's cup,
And he learned too late, when the night slipped down,
How close he was to the golden crown.

Success is failure turned inside out -
The silver tint of the clouds of doubt,
And you never can tell how close you are -
It may be near when it seems afar;
So stick to the fight when you're hardest hit -
It's when things seem worst that you mustn't quit.

Author Unknown

All Alone

There are some days that I feel all alone in this.  Even my husband and I don't always see eye to eye on how we should proceed.  This is so new, scary and frustrating for both of us and neither of us really know what will help.

Sometimes I walk down the medicine isles in the grocery store just looking.  I think I am looking for that magic pill, the box that screams out "Cure Your Arthritis NOW!".  I have yet to find it.

My approach is exhausting.  I want to do everything that could possibly help and I want to have started it yesterday.  My husband likes to pace us.  His way is probably the best.  Three doctor's appointments this week and three therapy appointments is a lot for all of us.  I hate it.  On the other hand Kindergarten is only three and a half months away!

I have to keep reminding myself that there is no magic pill.  There is no cure.  SOJIA is here to stay.  I'll never like it, but I'll need to learn to work with it. 

Our real job is helping Emily learn how to live with it.

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.

Macrophage Activation Syndrome

I failed to mention that one main reason Emily was in the hospital for so long was a diagnosis of Macrophage Activation Syndrome or MAS.  Mainly because it is incredibly difficult to understand let alone explain.  This is from Arthritis Today, Kids Get Arthritis Too Volume 11 Issue 4 By Mary Anne Dunkin.

As I understand it the cells that normally kill damaged cell or viruses get over activated and begin killing normal, healthy cells. 

What Is MAS?

Although not wide­ly recognized, MAS is a relatively common and potentially serious complication of rheumatic diseases in chil­dren, particularly systemic JIA, lupus and vasculitis, says Randy Q. Cron, MD, PhD, who helped diagnose Jacie’s condition and started treatment that may well have saved her life. The condition affects up to half of children with systemic JIA; as many as many as 7 percent to 10 percent of children with systemic JIA will develop a case severe enough to require hospitalization, he says.

The problem occurs when the immune system gets ramped up to fight an infection but then fails to slow back down when infec­tion has been eliminated, says Dr. Cron, director of the UAB Division of Pediatric Rheumatology. Normally, once an infection has been eliminated, white cells called CD8 T cells kill off the cells – typically macro­phages or dendritic cells – that stimulate the immune system to fight invaders. In MAS, a defect in this mechanism sends the immune system into overdrive, producing proteins, called cytokines, including tumor necrosis factor, interferon gamma and interleukin-1.

“When these proteins are in excess, you get what is called a cytokine storm,” says Dr. Cron. “This leads to this pro­longed fever but also leads to cells eating up other cells in the body. Macrophages go around engulfing other cells.”

As a result, blood cell counts fall, lead­ing to a problem known as pancytope­nia. In the worst cases, organs fail, which leads to death if not treated immediately.

New Findings Offer Understanding and Hope

For a doctor not familiar with MAS, it can be mistaken as septicemia, a life-threatening blood infection. But scien­tists have discovered the syndrome shares features with a genetic disorder called familial haemophagocytic lymphohis­tiocytosis (FHLH). Triggered by infec­tion during the first year of life, FHLH is almost always fatal unless the child receives a bone marrow transplant, says Dr. Cron.

To have FHLH, children must receive two mutated copies of one of the critical genes involved in cell lysis (killing), he says. Research in the past few years has revealed that children with MAS often have only one mutant copy of these same genes (e.g., perforin, MUNC 13-4, etc.), suggesting that the syndrome is actu­ally a milder form of FHLH, he says.

While this finding offers understand­ing that could eventually lead to improved therapies for both conditions, another find­ing with more immediate applications is that the biologic agent anakinra (Kiner­et) – which inhibits interleukin-1 (IL-1), a key cytokine produced in both system­ic JIA and MAS – is a rapid and effec­tive component of treatment for it.

“Typically, we use a combination of high-dose corticosteroids, cyclospo­rine and anakinra,” says Dr. Cron. “We are usually able to control it. Anakin­ra, at least for our group, has revolu­tionized the care for these kids.”

What Parents Need to Know – and Do

Although MAS can occur at any time during the course of rheumatic disease, research shows it commonly occurs short­ly after or even before or around the time of diagnosis. Parents should be aware of the condition because it can come on suddenly and become life-threat­ening very quickly, says Dr. Cron.

The main symptom that parents need to watch for is a high fever, he says. “Unlike the typical systemic JIA fever that occurs once (usually in the after­noon) or sometimes twice daily and comes back down, MAS tends to be a more continuous high-grade fever.”

Another sign of danger is the child is not as alert or as arousable, because MAS can affect the central nervous sys­tem, says Dr. Cron. Jaundice (yellow skin) is a sign that comes later, indicat­ing the condition is affecting the liver.

Parents who notice these symptoms should get their child to a doctor as soon as possible. Although there are numer­ous specialized tests for helping to diag­nose MAS, he says one simple and widely available blood test, a serum ferritin lev­el, is a good place to start. The test, which measures the amount of iron-contain­ing protein in the blood, is almost always highly elevated in children with MAS. “Other conditions can also elevate your fer­ritin, but usually not to the degree that it does in patients with MAS,” he says.

If your child is in the emergency room or sees a doctor who is unfamiliar with MAS, he recommends asking for a ferritin test. “There is no downside and it is remark­ably sensitive for picking this up,” he says.

Most of all, he says, be your child’s advocate. Be persistent in pursing the causes of your child’s symptoms.