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?