Eye Issues with Neurofibromatosis

One of the most common signs of Neurofibromatosis is Lisch Nodules.  These are harmless/benign "elevations" of the color parts of the eyes (the Iris) and a key diagnosis factor in people with NF.  Basically, we have been told that Lisch Nodules are "birthmarks", that have no affects on vision and require no treatment.

Our NF doctors are awesome, don't get me wrong...And were telling us, that it is "highly unlikely" for a child to develop Optic Nerve tumors after the age of 8....And that MRI scans would no longer be necessary...UNLESS, something showed up in the eye exams....

Braden has had yearly eye checks, even before his NF1 diagnosis in 2007.  We were doing exactly what we needed to do, and Braden, to this day, still sees 20/20.

However, during our routine - yearly eye visit 2 yrs ago, the doctor notice "inflammation" in Braden's left optic nerve....

This alerted our Neuro-Oncologist, sending Braden in for an MRI.

It had been about a year since his last MRI (which came back with results as your typical "NF Brain") Click HERE for more on "NF Brain"

I wasn't really sure what I was expecting...We were dealing with Bailey's chemo and I just assumed that the findings were going to be no-big-deal.....But, when I got a call from the doctor before we got home from our drive from the hospital...I knew SOMETHING was wrong.

"But...You told me we didn't have to worry....You told me......"

I was side-swiped

At 14 yrs old....After 2 "normal" MRI's...Braden was diagnosed with an Optic Glioma.  Treatment would depend on his visual exams....Which, thankfully continue to be normal.

BUT...

It's THESE exams, that come every 3 months, that went on to diagnose a NEW problem.

After dilating....Our AWESOME doctor noticed issues with Braden's right eye.  I knew something was going on, after the doctor took an extra long time looking into Braden's eye....leaving the room, and coming back with TWO other doctors.  Specialists.

LOTS OF PICTURES.  ULTRASOUNDS.  VISUAL FIELD TESTS. 

MORE DILATION.    MORE DOCTORS

One doctor quickly rattled off some really long diagnosis, and left me sitting there.  WAIT.  WHAT?

SLOW DOWN!

Braden's right eye has something called a "Combined Harmatoma of the Retina and the Retinal Pigment Epithelium".....

I know right...?  What is this in ENGLISH!?

Basically a tumor that is too far back to be an optic glioma....and too small and in an area that MRI's can't pick it up...But can greatly affect vision, especially peripheral vision.

Eye issues are very common with NF...And with routine exams, most issues just have to be monitored.  The "wait and see" is just the way it goes with NF...Frustrating?  Absolutely.  But as I look into my sons eyes...I see a boy full of future...Full of Love....Full of Spirit...And full of Vision.

Thrive On!

Another Rare Tumor?

I'm not really sure how to begin this blog post, because I am still processing our long day at the eye clinic.  Lots of long words were thrown at me, and I literally had one of the many doctors we saw write what he had told me down on a piece of paper.

What is it with doctors and their messy, short-hand scribbling?

I couldn't read the writing, so the nurse was awesome and re-wrote it--Thank Goodness!

There is an abbreviated term for what the doctors are thinking Braden is dealing with.  CHRRPE (gosh, even the abbreviation is long.)

Combined Harmatoma of the Retina and the Retinal Pigment Epithelium.  *Whew*

Basically this is a "rare" (yay for us...NOT) type of lesion or tumor of the macula. (The back part of the eye that is responsible for central vision)

We were told that Braden would need eye checks every six weeks, to monitor his vision, because once vision problems happen, it can very quickly take his vision and can very possibly turn into cancer.

It was a lot to take in...But we were thankful and comforted by these very attentive doctors...who told me, that this type of condition is often missed by general eye exams.

For Braden, he has this way of "checking-out", when things get complicated or overwhelming, which - in this case, is a blessing.  All he was worried about, was that it was 2pm, and he hadn't had lunch yet.

We stopped at McDonald's before heading home, and I let him choose ANYTHING from the menu.....A #2- "large-sized".....which he gratefully inhaled.  

Where has the time gone, where Happy Meals were all he wanted?

THRIVE ON!

When Things Go Wrong, As They Sometimes Will.....

Today we drove to the Children's Hospital for the 3rd time this week. (Is it REALLY only Tuesday?)

 Today's visit was for Rachel, and a check-up with the Ophthalmology department.  My frustrated mood from the morning, plummeted even further when I watched as Rachel was unable to read the letters that reflected on the wall 10 feet away.

She tried so hard.  Squinting...and squirming in the over-sized chair.  She started reciting letters.  

E?---F?---S?---L?...But those weren't the letters on the wall.  Another set of letters.  "You're doing great Rachel!" the Dr. turns her swivel chair towards my brave girl  (who seems to think she guessed all the letters on the wall right!  And a sneaky sense of "wow-ment" fills her eyes)

The Dr. walks her swivel chair close to me and asks me if Rachel could be overly tired today.  "No...I don't think so." I responded....In my head I was thinking that I just wanted to tell her "YES!", in hopes of giving a reason for the sudden decline in Rachel's vision.

But THAT reason would be too simple.

The doctor told me that Rachel's eye exam has left them stumped.  In the Winter of 2009, we were told that Rachel would be "legally blind" by the time she was 10.....A year later, we got THIS NEWS .  An amazing and wonderful thing for our family to hear.

The scans since have shown "stability"!  

A simple word...But one that causes joyous excitement in our world.

But today...We didn't hear "stable".  

We heard our doctor tell us that Rachel's vision has worsened since the last visit. 

This visit was like witnessing a car wreck...And I felt that helpless feeling that is all-too-familiar.

I wanted to scream.  I wanted to fight against the reality that Rachel may face blindness one day.  There is no "jaws of life" that can free us from this possibility...And as angry and helpless this makes me...I have to remember who is control of this situation.

Rachel tells me as we skip down the hall, after the appointment ..."Did you know that people can see things with their hearts?"  "How do you know?" I ask her...."'Cuz Mama, when I close my eyes and it's all black....I can still see how much you love me!"

My God.  

How can I possibly be angry?  How can I question?  How can I ever doubt?

THRIVE ON!