Another year goes by

Today marks the two year anniversary of our introduction to the world of Evans Syndrome.

In February, I found out that New Jersey was the first state to grant awareness to Evans Syndrome. Awareness is key to us, as I have stated in previous blogs.

One Facebook status later, I had a connection.....and a great one, thanks to my friend Kristin. I emailed Senator Darren Soto and a few days later his office contacted me wanting to help out. I dealt with one of the senator's legislative assistants, Angel. I had a lengthy phone conversation with Angel about ES. He then explained that they would draft a resolution and present it on the next meeting and if it got approved we would have to go to Tallahassee when they presented the bill on the floor. He said it could be a matter of weeks or one year. 

In March Angel calls and says "the resolution was approved! Can you come to Tallahassee on April 1st?" Of course!!! 

April 1st, the kids and I (and my awesome friend Deana) woke up at 3:45am to drive to Tallahassee (4hr drive) to have the resolution presented on the floor and to Ric! We were all super excited. William and Jonathan were very excited to go to the state's capital and see the inner workings of our government. This is stuff they read in history books. They never thought they would actually get to see it. Ric, on the other hand was more subdued. He was happy we were doing this, but he doesn't like to be reminded of Evans and today was going to be all about Evans.

We get to Tallahassee and go to our senator's office. His staff was super nice and very welcoming. They walked us to the gallery of the senate where we could sit to hear the whole thing go down. Click to see what happened! 

http://youtu.be/9t5FASD14dk

From now on, April 1 is Evans Syndrome Awareness day in the state of Florida. All it took was one friend who had a friend who had a friend and a senator kind enough to understand this was important to us. In the words of my son William, I really liked this day!

This day was huge in our household. It is important that our children see that when you want something done you need to do something about it. It may not be easy, but it is worth it. This is how you change your world. Not THE world, but our world. The Evans world. We did not ask to be a part of this world. However, we are. I believe we are here for a reason. My kids have a different perspective on life because of this. Ric has grown ages in two years. So have William and Jona. 

Two years have gone by since Ric was diagnosed. Two years ago  my world crumbled around me. We have worked hard at building it back up. The support of our friends and family has been instrumental to the rebuilding of our world. It hasn't been easy to deal with Evans, but it hasn't been impossible. Ric continues in good health and we are thankful every single day for it.

Awareness IS Important!

 February is Evans Syndrome Awareness month. Why is awareness so important? Simple, if people don't know about it they can't do anything about it. 

Did you know that Autoimmune diseases are the #3 cause of death in the US? #1 is heart disease; #2 is cancer; #3 autoimmune.  Do you know that people with compromised immune systems are at a higher risk to have cancer? Well, now you know.

Awareness means, hopefully in the near future, ALL doctors will know about Evans Syndrome and when we walk into an ER nurses and doctors know how to deal with these patients without us having to give them a lengthy explanation (or give them time to google this...true story!)

Awareness gives us the chance for someone out there to have the

 curiosity to look into this and research it. To find a course of treatment that is correct so we don't have to treat it in trial and error mode. Do you know how many times we hear "we will start here and if that doesn't work we will do this and if that doesn't work we will do some other thing." Do you know the emotional toll that takes on someone?  The wait and see game? Do you know the toll it takes on their bodies with the crazy side effects of these medicines?

Awareness allow us to tell you all this. Often, this disease is described by doctors as a roller coaster because one day you'll be fine and the next day hell can break loose. If we have the right medication, the right tools to see how we can prevent the next downfall Evans' patients will be able to live longer and enjoy life a bit more.

Two people have died from this disease in the last month. One has been sent home to hospice in the last week. This shit kills people! Although Evans is manageable, there are still these reminders out there that this can kill you. Period. Simple as that. You get an infection and your body can stop producing white blood cells or platelets and then BAM! Dead. Do you know that if your platelets fall below 15,000 your brain can spontaneously start bleeding? What do you think happens then? You fucking die! Because no brain surgeon goes near brain surgery if patient's platelets are below 50,000!!! Much less 15,000!

The state of New Jersey has passed a resolution to have an Evans Syndrome Awareness Day on Sept 21. This is HUGE! I encourage the Evans Community to reach out to their senators and make this happen in all 50 states in 2015!

Dealing with Evans sucks. Sucks so bad. It is up to us to make it suck a little less!

Brutal reminders

The Evans community has had two reminders this week of how this disease can kill you.

I hate these reminders. I hate them! Because it makes me face the brutal reality that my son is at risk of dying from this shit. Granted, Ric is doing well so far, but that reminder is there. Always....fucking....there.

It's there everyday when I open my eyes and wake up to a new day. My first thought every damn morning is "will the shit hit the fan today?" Mothers are worriers, period. For mothers of chronically sick children, worry is an understatement.  Every morning after that dreaded thought my second one is "choose happiness. Choose peace. The other shoe will not drop today." 

People say "one day at a time" not even realizing that one day at a time is pretty exhasperating. One day at a time basically tells you tomorrow is not guaranteed. One day at a time 

sucks.

It sucks, but we do it everyday. We power through the unknown because we don't have a choice. We have to remain strong for our children, so they can rely on us. 

This week our Evans community lost two people. Science doesn't think these patients are worth a damn. Research scientists don't think these people are worth a damn. Insurance companies don't think these people are worth a damn. "There's not enough people with the disease to justify research", they tell us. So, my son basically is not worth a damn. This is the reality we live in. 

We lost two people to Evans this week. Two people. The reminder staring us in the face that this could happen to my son at any given moment.

You have to live too!

I had a big race this weekend. Earlier in 2014 I had signed up for Goofy Challenge. This is run a half marathon on Saturday (13.1 miles) and a full marathon Sunday (26.2 miles) for a total of 39.3 miles for the weekend.

On Thursday, Ric came home from school feeling weak and with a stomach ache. At first I thought he had caught the stomach flu both his brothers had days prior. Then he ran a higher fever than his brothers did, which I attributed to Evans...because as we know, nothing is simple in the Evans' world. Of course, I immediately texted our pediatrician who said "sounds like the flu, go to the office in the morning and have some wine now". 

Friday morning....8:30am we are at the office. They test for flu, negative. They test for strep, negative. Our regular doctor was out sick so another doctor saw him. She had never dealt with Ric and asked if we wanted to draw blood and check his numbers. Lately, I have been letting Ric make the call (if he feels bad) to draw blood or see the doctor. He said he felt fine and he didn't want his blood drawn. The dr looked at me and I said "It's his body. We drew blood 10 days ago and everything looked pretty good." Then she said "we are drawing blood, end of story. We need to make sure." Meet my 2nd favorite pediatrician! 

Thank God she decided to draw blood cause his platelets were down to 50,000. They had been 115,000 10 days ago! Just 10 days ago! Both pediatricians and the heme/onc think the drop in numbers is due to whatever virus he is fighting. We go Friday for tests again.

Now the real internal battle begins. Do I run the race or stay home and quit the race? The plan was to be out the whole weekend because these Disney races are crazy early. Josh was home and he said "you continue with Goofy plans. I am home and I will take care of things here." Pediatrician also texted and said "go run your races." I am not going to lie, I felt like shit deciding to run the races. I felt selfish. But at the end of the day I ran my races as planned and everything was just fine at home. Ric didn't run any more fevers and didn't have any other symptoms. He played ps4 the whole weekend with his brothers, so he didn't miss me at all!

Today, after all was said and done I thought about how deciding to run my races was the best decision for me. I had a fantastic weekend. I ran the half with one of my best friends from high school. I ran the full with my friends whom I had trained for this. I had fantastic races and I feel completely ready and refreshed to deal with whatever comes.

Today, I saw the pediatrician cause Jona has an ear infection...again. She said "I see you are wearing your marathon shirt. So happy you decided to run your races. You can not live life thinking hell is going to break loose if you are not there for Ric. You have to live too. He is not going anywhere." 

"You have to live too". Easier said than done. The one thing I am the proudest from this weekend is the fact that I tried my hardest to enjoy myself and not be a debbie downer to the people around me.  That I did not break down in the middle of the Expo when I received the call with numbers.  That I sucked it up.

Last test of 2014

Ric has been out of remission for a few months now. As it is to be expected with this, he has been up and down. Today was the last CBC of 2014 and his counts were pretty good! 115,000 platelets, great for Ric; 4.82 white blood cells, actual normal, as in you and I normal! Neutrophils are a bit low, but not low enough to be concerned.

 Ric was in remission for more than half of the year, which is great in our world.  We have been on a wait and see plan, yes that is the actual medical experts plan, for a few months. Shit hasn't hit the fan yet! I'll take it!

I have seen Ric struggle with the thought of being out of remission. What lies ahead won't be easy, but it won't be impossible. The other day someone in the hospital said to me "You are not alone" and I know that, but for whatever reason that simple statement made my stomach turn. I had never seen myself as the mom of a chronically sick child. I have always seen myself as the mom who has no choice but to be strong for her children. I guess at that moment I was looked at as the mom of a chronically sick child. Maybe it was the look of pity in her eyes. I can't quite put my finger on it, but I refuse to be that mom.

2014 was a relatively easier year than 2013. 2014, dare I say, was a better year, a more peaceful year. We are all one year stronger.

Thankful for the fight!

They're back....

Ric had his follow up appt today. When Dr. Eslin saw him he said "it is short of a miracle that you have been off of meds for 7 months!". He then gave me that look...yeah, that one that said "wait for it" and then the words came out of his mouth "we have seen up to 6 months off of meds. Very, very rarely a year off of meds. It is time to watch how his body will behave." 

For a split second I thought maybe Ric can be the exception to the "rule"? But I knew that shit was coming down. I have basically known for two weeks now! He hadn't had any bruising, bleeding or petechiae. However, his eyes have been kind of full of little blood veins every other day or so, which is something that happened at the beginning of our Evans' journey. 

Hours later I get the dreaded call! Let's be real! Even when they call with good news, seeing that number on my caller id always makes me stop breathing for a moment. White blood cells are good, anc is good and platelets are low. 60,000 platelets she said and my heart sunk. Coombs positive (this is the test they do to ck if the evil antibodies are back)..fuuuuuuuck!!!! No meds for now, Dr Eslin wants to draw blood again in two weeks and see where we stand. If symptoms arise before two weeks, then we go. 

In my head this sounded like "they're baaaaack!" from Poltergeist. Just like that! 

I am thankful that we got 7 months of peace. I reminded Ric about this. He has been a bit in shock and awfully quiet this afternoon. He said "I don't want to be the sick kid". These are the moments where I truly don't understand where I get the strength to say "You are not your disease. Your disease does not define you. HOW you deal with it does! So, chin up! You know this is something that will come and go. You will have some good months and some challenging ones. It is what it is. We will deal with what comes."

 I can't even find the words to explain how this makes me feel. Sometimes I see moms on Facebook losing their minds over a simple cold. Don't get me wrong, thank God they are only dealing with a cold and no one wants to see their child suffering! But I can't help but to think "try that a million times worse and with doctors really not knowing how to treat it." I don't mean to sound like an insensitive bitch, but I feel like one thinking that.

This time I feel like we know what we are dealing with. Now, I know chemo is a real possibility again. Now, I can somewhat mentally prepare myself for that because sitting in that room is NO FREAKING JOKE! Now, Ric knows what to expect. Now, Ric has a reminder that yes this is here, this is real and he has to deal with it again and again and again. Now, we know the walls will tumble, but they won't fall.

Hives are just hives

So, yesterday doctors had to do a full autoimmune  checkup on child #2, William. He's been having hives since Sunday. They come and go. He is also complaining of joint pain, but that is to be expected with hives.

Hives can be just hives, said the dr, but we want to make extra sure. Lymphnodes are a tiny bit swollen around his neck. But, that can also be due to a viral infection. These can also bring about hives.

Nevertheless, a full autoimmune check up sent me to a very dark place I rather not visit. It reminds me so much of when we started dealing with Evans. Ric is so concerned for his brother, it breaks my heart. It breaks my heart to hear him say "they told me not to worry at the beginning too, before we knew what we were dealing with. I am expecting the worst, hoping for the best!!" Those were the words out of my 16 yr old's mouth. Those are my words! Prepare for the worst, holing for the best. Somehow, hearing them come out of him broke my heart into a million pieces. I could see and feel the anxiety coming out of him. It is almost as if we both have autoimmune PTSD. 

This shit has us traumatized! As soon as they told William yesterday it wasn't strep he asked "is it something serious? Like Evans?" Holy shit! This is the reality we live in. There is nothing simple anymore. It doesn't matter how I try to appease them both, they are still worried sick and I can't take it away from them. 

Our pediatrician said not to worry, yet! Hives can be just hives! Nothing else! That's what I keep telling them and that's what I keep telling myself.

A friend told me today "kids feel deeper than we give them credit for". She is so right and that is what breaks me. Because I can't fix it. No matter how hard  I try to reassure them, the anxiety of being seriously sick is a reality in our house for one of them. Therefore, they all think that could be their reality too.