Running underwater

Everything about this nasty disease just sucks.  Every time we do blood work we hope to see a normal white blood cell count.  It has not been normal in over a year.  That is in fact how this all started, high white blood cell count.  So every other Monday we find ourselves a bit disappointed and just feeling as if we have no idea what to do.  I even asked her Rheumatologist if we can just consider high her new normal and was reminded that high is not good and not a new normal.  So each appointment we add a new medicine or increase an existing one.  This last time we increased the cyclosporine, a potent immunosuppressive agent. 

With all that has happened this past year we watch carefully for any changes.  So far Emily has improved so much health wise and emotionally.  The rash has not been around at all and she hasn't had a fever in a year.  She is happy and off physical therapy completely, although we continue with water therapy to keep her joints moving and help increase strength.

In the past few months we have noticed a decline in school.  She is only in kindergarten and since her birthday is at the end of the year she is one of the older children in the class by virtue of missing the cut-off date to enroll.  I just happened to mention this to the Rheumatologist because I know inflammation can occur in the brain and at her onset she had an episode of night vomiting, a symptom of pressure in the brain, and Vasculitis (inflammation of blood vessels) is a constant plague.  When I mentioned it the doctor replied immediately that yes this nasty disease could cause learning disabilities, but to find out if that is the case we'll have to do more testing and that can cost up to $5,000 because it is not covered by insurance, even our great insurance. 

So, since the brain is a muscle we are going to start getting Emily to exercise her brain more.  If she tells us she wants to learn something, she asked if she could learn Spanish, we say yes and so she is going to her first Spanish class after school tomorrow.  We don't know how well it will work out, but just like teaching her how to exercise her joints without hurting them we've now got to teach her how to work around any learning difficulties she may have.

There is just so much nastiness to this disease that such a short time ago we didn't know even existed.  Have you ever had a dream where you are trying so hard to run fast and you can barely move as if you are trying to run underwater? That is how we are feeling right now; but we shall keep pressing forward and do whatever we can to tell everything that children get arthritis too and they need a cure!

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.

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.

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.

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.

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. 

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.