New meds

It's been a while! Too long! Life has been getting in the way of my thoughts.

Ric is out of remission and facing the ugly face of Evans again. I don't know why I thought "oh, the next time will be easier cause there is no element of surprise. Now, we know what we are dealing with." Well, not quite. Yes, we know that he will have flare ups and that he will be in remission, but the emotional side of this just sucks!

It all started with enlarged lymph nodes, again. Then came the mouthsores. Holy mouthsores! Even the dr had no clue where they were coming from. He did a culture to rule out bacterial infection. All clear! Nothing! Nothing! He wanted to rule everything out before he said "it's just one of the things he will have with Evans". ES is a diagnosis of exclusion and it seems that every time it flares up they will look for every other explanation in the planet before they tell us it's because of Evans.

The mouthsores were terrible. He couldn't eat properly or smile properly. We ended up putting him on prednisone. He also had a magic mouthwash his dentist (with heme/onc approval) prescribed him and pink honey. Yes, we applied pink honey on the mouthsores with a qtip and they started healing. Maybe it was a combination of all things!

He is now on sirolimus. Check out the clinical trial on this link  https://clinicaltrials.gov/ct2/show/NCT00392951

Sirolimus is the med they give patients who have had an organ transplanted into their bodies. Apparently it gives ES patient's immune system another perspective. It tricks the immune system. All I keep thinking is "at least it's not chemo". Our dr was explaining to us that research is now showing how Rituximab is not as safe as they first thought. So they won't do that unless strictly necessary. Lovely! He's had 4 rounds of that! This med works slowly so they are assuming he will be on it for at least a year. Numbers haven't been all so great since he started this medication. Apparently that is to be expected because the med works slowly. His next tests are in April, we'll see.

Mouthsores are still popping up every now and then. It highly annoys him, especially now that he has a girlfriend. But, he has to deal with it. This time it has been harder on him. It pissess him off that he has to deal with Evans. I hope one day he realizes it has also made him stronger.

Just breathe

I haven't posted in a while! Sorry! Summer was crazy!!

Ric spent the Summer in Puerto Rico and  did pretty good! He only had a few minor nosebleeds. His platelets were at 73,000 in July. Doctors think it may have been that and allergies. You know, cause they can never give us a definite answer about anything!

Since he came back, beg of August, we had tests done again. WBC are at 2.4 (a drop from July) with an ANC of 372 (a big drop from July). Platelets at 70,000, RBC were normal. 

Breathe!

He has been noticing more swollen lymph nodes than usual. He lives with one on the left side of his jaw that is always there. Now, ones on the right size are popping up. He was very worried. Like, seriously worried. "Mami, what if I have cancer and no one has caught it?" THAT is the reality of my son's life. He has this constant concern. Even when he doesn't show it, it's there. My heart keeps breaking for him. There is nothing I can do, but to try to keep him in a positive, realistic attitude. Of course I told him there was no cancer! He asked to go to the doctor and we went and they said nothing to worry about yet.

Breathe!

We have to test again to see where his numbers are next week. The days need to go by fast. We are back to CBC's every 4 weeks. We were up to 8 weeks. I  am hoping by next week we go back to that.

In the following weeks he gets to be entertained with driving classes. Ugghh....I am so not ready for that! But, I think it will do him a lot of good to have his mind on something else. Not that I want him completely disconnected, but I don't want him constantly worried.

Just breathe!

To the rare mothers

 


This one goes to the rare mothers!!!!

Rare mothers are in a club they didn't ask to be in . Rare mothers hug a little tighter because they know things can change quick.  Rare mothers hold it together when everything around them is falling apart.  Rare mothers are given an exclusive membership to this club because THEY CAN HANDLE IT!  Even when it seems, like they can't, they somehow can.

Every mother tries to teach their kids the best way they can. Rare mothers, do the same, except they have the extra responsibility of teaching their kids weird stuff like "listen to your body", "take the Afrin with you to stop nosebleeds", "is your urine dark?", "are you out of hand sanitizer?", "is this normal tired or weird tired?".  "How do you feel?" becomes an everyday question with heavy connotations and it's like they can't breath until they hear "I feel fine." Rare mothers have to hold it together when the shit hits the fan, which, unfortunately, is more often than they would like.

Every mother makes this parenting shit up as they go. Rare mothers, do the same, except as they go they have to become experts in rare things there are no experts for!

Every mother worries sick about her children.  Rare mothers worry sick about their sick children.

Every mother comforts their children when they are sick. Every mother wipes tears off their children's faces when they are upset. Rare mothers do the same, except they have to sit in a chemo ward watching poison go down their kids bodies. Rare mothers have to explain how the meds will work to make them feel better.   Rare mothers have to be very careful with the ever moving line between overprotecting and letting it go.  Rare mothers don't know of a simple cold anymore because nothing, NOTHING is simple anymore.

Every mother says to their children "everything will be all right."  Rare mothers say to their children things will be all right when, deep down,  they really don't know if that is true.

Every mother wants to guide their children down the right path. Rare mothers want to do the same! Except, for rare mothers it may be a little tougher to guide them and then step away.

Every mother loves their child. Rare mothers love their children too, I dare say they may love a little harder!!

I do realize all I have said here may be politically incorrect....see if I give a damn!

At the end of the day, they are one and the same. Rare mothers are a balancing act of the mom they once were and the rare mom they now are.

How do you say to your child in the night
Nothing is all black but then nothing is all white?
How do you say it will all be alright
When you know that it mightn't be true?
What do you do?

Careful the things you say, children will listen.
Careful the things you do, children will see and learn.
Children may not obey, but children will listen.
Children will look to you for which way to turn, to learn what to be.

Guide them but step away,
Children will glisten.
Temper with what is true
And children will turn,
If just to be free.
Careful before you say,
"listen to me."

-Sondheim

Send in the Clowns!

It's been two weeks since Ric's last tests where his platelets were 58,000 but the rest of his numbers looked good.

Last Tuesday he woke up with a very sore throat, itchy eyes and extremely tired. He asked me to text the pediatrician, which I did. Her response was "We are not testing him. These are allergies. We don't want to create a hypochondriac.Up his allergy meds."

Send in the clowns.

A hypochondriac??? We have been telling him to listen to his body for 2 years! To be communicative of whatever feels "off". For 2 years that is all he's heard from us and his doctors. To be vigilant. Where is the line between listening to your body and becoming a hypochondriac? Where is the fucking line? Aren't we asking so much of a 16 year old??? He already has to deal with the fact that he has a chronic illness.  How much more can I ask of him? Her response was "Now he needs to learn to interpret what his body is telling him." Seriously!  We upped his allergy meds and hoped for the best.

Send in the clowns.

The week goes by and he doesn't seem to be getting any better. He still feels tired, he is still feeling kinda sick.  I am thinking, well they keep telling me it's allergies, so I guess it has to be allergies.  Sunday night I tell him that if he didn't feel any better by the end of this week we would call the doctor again. Well, Sunday night he ran a slight fever. Monday morning we were at the doctor's office. Again, she brings up the whole hypochondriac situation. A bit more subtle, cause this time she was talking to him and not me. She didn't use the word "hypochondriac" to him. She said, for the next two years I am going to teach you how to read  and interpret what your body is telling you. What are the signs that are concerning and what are the signs you can ignore.

Where are the clowns?

Ear infection! That's where the fever was coming from. Or is it? I don't know! They can't tell me for sure! Ric asked to have blood drawn and she refused. She said there was no need.  This morning he wakes up with a bit of bleeding from his mouth. 5:40am and I am texting the pediatrician again.....we have a bit of bleeding, nothing too severe. I tell her I am sending him to school cause the fever is gone. She agrees with me and off to school he goes. Her actual words were "ok, no stress!!". No stress??? WTF??? Of course, I am telling Ric no stress, all is good, call if anything else arises, have a good day! Me, on the other hand, WTF? No stress? Again, where is the line? Where is the line between overprotective mother and real concern?

Send in the clowns.

A friend told me today "Maybe the line is constantly moving". Then my husband said the same thing. I get it, I know nothing in this Evans world is black and white. I know that! I know it's a fucking sea of gray. The fact that I know and understand this doesn't mean I have to like it. I keep hoping there are a set of parameters we can go by, but the more we know, the more we learn there aren't any.

 Isn't it bliss?
Don't you approve?
One who keeps tearing around,
One who can't move,
Where are the clowns?
Send in the clowns.

Send in the clowns!!! There ought to be clowns!!!

**In the circus world, "Send in the clowns" means there has been drama and it is now time to put it at an end. When someone has had a lot of drama going on in their life it is time to send in the clowns, get rid of the grief, and be done with it.

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.