Plexiform Neurofibromas

What are these tumors?  

Why do these tumors do so much damage?  

Is there anything one can do about it once they are diagnosed with these?

(these pictures are NOT my own.  I found them thank to Google images)

Plexiform Neurofibromas are ill-defined (meaning they are not round and typically grow deep within tissue and bone), slow growing masses that have been described as feeling like a "bag of worms".  These tumors are typically benign and are common with NF, but not everyone NF will present with them.

Studies say that about 30% of people diagnosed with Neurofibromatosis, have these types of tumors, affecting them in many different ways.  The growth rate is unpredictable, leaving some not diagnosed until adulthood, while with others these tumors are unmistakably present, causing deformities of the bone and skin in beginning in childhood.

Since NF can affect every organ in the body, "plexis" can appear anywhere, and cause significant damage.  The only treatment is to debulk the tumor as total removal is not usually possible, due to the tumor being  wide spread, attaching to nerves and organs.

Recently, my 13 yr old son was diagnosed with "many" small plexiform tumors, affecting his spine, and under his left arm.  I find it extremely frustrating when the doctors tell me, "there's nothing we can do".  What do you mean there is NOTHING you can do?  Aren't the doctors supposed to have the answers?  The cure?  The treatment?

This is the world of NF.  This disorder leaves most of us praying for it to stay "mild", or for the progression to suddenly stop.  I know for myself, I have a hard time knowing that while my NF may be "not so bad" now, it can take a cruel and steady turn, at any time.

This is why it is so important to focus not on my disorder, but on everything around me that IS good.  I am so much more than NF.  I am stronger and more powerful than anything that comes my way....and my goal is to teach my children this too.

NF throws a fork in the road of life.  You can choose to go down the path of anger, regret, and fear.  Or you can take the path of light and strength.  Both are sometimes very hard roads, but there is only one that will take you to a place of  HOPE.

I am in this fight, just like you.  You are not alone.

Thrive On!

You Have Cooties!

"Ewww....Get away!  You have Cooties!"

I remember playing this game as a kid.  Running around the playground, chasing boys and tagging them, giving them MY cooties, so that they could chase the girls to try and give them back.  It was a fun game and no one ever REALLY caught the dreaded cooties.

The other day, I was waiting to pick up my kids from school.  I waited in the parking lot and saw my 8 yr old daughter walking slowly to the van.  She was noticeably upset and took an extra long time to make it to me.  Tears ran down her face as she looked at me.

"Are THESE cooties Mama?"  She asked as she pointed to her cafe' au laits marks on her neck and arms.  "The kids in my class say I have cooties because I have these. They won't play with me at recess and laugh at me." she continued to cry.

My heart stops.

"You do NOT have cooties sweetheart.  God gave you these marks, so that you stand out, so that the special friends you have yet to make, know who you are."  I explained to her, as best I could, that while NF may make her different, it just makes her job of finding the people who are really special, a little harder...but worth it!

Rachel went on to tell me that there are TWO girls in her class who are her 'bestest friends'.  "See!" I told her.  "Would you rather know 20 people who make fun of you....or 2 people as your special friends?"  She went on to say that 2 was better than 20.

I hugged her, wiped her tears, and promised that she and I would always be special and it was our job to make sure we let other people see our hearts and help people understand that NF wasn't cooties, but instead a special gift that allows us to have genuine friendships.

Follow-Up With Neurosurgeon

It's so nice to be able to walk into a doctors office and not have to explain Neurofibromatosis.  The health care we have been getting here in Colorado has been  REALLY good.  

I remember not to long ago, walking into my kids' doctor in a new city in Washington.  The Doctor sat down and I unloaded a list of issues that my family was dealing with.  "We have NEUROFIBROMATOSIS!"  I said expecting him to know what it was I was talking about.

The doctor just stared back at me.  "You mean Fibromylagia?"  he responded.  "No....I mean Neuro-Fibroma-tosis."  I told him.  "Ohhh ok, the Elephant Man Disease, right?"  I was shocked, hurt and unbelievably confused!

We never went back to that doctor.

Yesterday, Braden and I spent half the day talking to NF specialists about the new findings in his latest MRI scans.   Just a year ago, Braden  was "stable" and showed no signs of tumors on his optic nerves.  Now, he is presenting with a pencil eraser sized growth on his left optic nerve.

Plexiform tumors are scattered along his spine, causing balance and bladder issues.  Dr. Handler looked at me and said...."In the 'NF' world...this doesn't look too bad."  The 'NF' world??  I hate the NF world!  But at the same time, I was grateful for the news.

We were sent off to Neuro-Oncology to set an appointment and meet with Dr. Rush, who also sees Bailey, for the tumor on her auditory nerve.  We were told she would review Braden's latest MRI and would need to see him back in a few weeks.

I was feeling overwhelmed until I saw a family holding on tight, to their young son.  His bald cute little head...his bright, wide eyes.....and His hand wrapped up, with an IV tube leading up to a bag of medicine.  Chemo.  

NF causes so many unknowns, I vowed to keep living in the moment.  To make the most out of each and everyday that I have.  This is the only thing I have real control over.

What If....?

Do you have regrets? Silly question - virtually impossible to go through life without regrets of one type or another, even if its just that lousy Mexican restaurant you thought you'd try last week. But some regrets stay with us longer than others.

What "what ifs" do you look back on?  Every New Year, I look back on years past and wonder about the decisions I have made.  Did I do all I could do medically for my children? For myself? Was I a good enough wife?  Was I a good enough friend?    

What if I could pull a 'Superman', like when he went back in time to save Lois Lane's life?  

What would I change?  Whose life would I go back and try to touch?  What words would I take back and never say?

The life we live is the legacy we leave behind.  Everything we do, counts for something and means something to somebody.  

I don't want to live my life always looking back, wishing I had done something different, or said something that didn't hurt someone.  Reality is that there is no "take-backs", no "do-overs", so we have to get it right the first time.

I'm reminded of an old quote: "Every choice we make probably seemed right at the time". Our choices are based on a lot of things that we have only so much control of - our life experience, our physical and emotional maturity, our financial means, and sometimes even our geographic location. When our conditions change, our choices often change.

Today, I know I'm making the best choices for my family I can, based on my current circumstances. Like you - heck, like everyone - I want my circumstances to change. When they do, my choices will change or stay the same accordingly.

There's really only one reason to look back at your old choices, and its NOT to foster regret. Instead it is to foster education. To learn from prior mistakes, to have a better understanding and appreciation of where you are by remembering where you have been. 

Learn from your past - laugh at it, shake your head its dismay, celebrate it - whatever. But don't spend too long there. If you do, before you know it you'll be looking at today with the same regrets, as it fades into the past as well.

Happy Monday! Thrive on!

Acting isn't Enough

There is no doubt that uncertainty breeds fear. Anyone dealing with a Neurofibromatosis diagnoses knows this to be true. But sometimes the uncertainty of the unknown isn't as scary as knowing something and not being able to do anything about it.

The news that my 13 yr old son now has many issues related to NF, when just a year ago things were stable, has thrown me into a place that I never thought I would be. The “rules” of NF don't apply to my family, since we have broken any predictable rule that NF had.

Acoustic Neuromas which typically affect people with NF2, now plague my NF1 daughter, Plexiforms that appeared virtually out of no where cover my 13 yr old sons spine, Optic Gliomas that were not there a year before, now being prominent....Our family alone, proves that you cannot predict anything in life...especially Neurofibromatosis.

Why? I often ask the air around me. Why this cruel relentless disorder? What is the point of all of this? And WHY won't God just wave His hands and take it away?

The only thing I have power over, in regards to my kids health, and my own health, is how I choose to handle what is being placed before us. If I run and hide and act scared....how do I expect those around me to act? At the same time, if I act all brave, pretending things don't bother me how is that giving a true picture of what is happening?

It's a battle that I am sure most of us face. The only answer I have for ME...Is to stop ACTING and start living up everything I say I am.