Ramblings and Ruminations of a VHL Mom

Thankful!

2011-11-24T19:01:00.004-05:00

...a poll...

Dale is thankful for me!

I am thanful for my family.

Steven is thankful for books, but not law books.

David is thankful for baseball.

Mary is thankful for love.

And the puppies are thankful for Steven!

A Day Late

2011-11-11T08:09:00.003-05:00

...and a not a dollar short!...

Yesterday was my sweet son's birthday.

Though we called him bright and early yesterday morning to sing happy birthday, I am a day late in wishing him a world-wide-web happy birthday.

He's a poor, struggling law student now, so his dad and I "padded" his checking account a bit.

So he is no longer a dollar short!

He also asked for a recent picture of his dogs, and I do my best to make birthday wishes come true!

Guess Who is Home!

2011-10-08T10:59:00.003-04:00

While I won't get into the details of the last time I saw my sweet son and why fall break is good for me,

I will let you know that it's wonderful for Steven to be home for a week.

Last night we watched a scary movie, and this morning the sound of animated Japanese cartoons filled the coffee hour!

...and we've already made a Target run...
...and we're having pizza for dinner...
...and the dogs have already enjoyed two LONG walks...
...and we've already teased his dad with stories of missed trains...
...and I've listened to his opinions on events, both local and global...
...and sometimes just touching your child gives your peace...

Conversations from Miami

2011-09-04T12:58:00.004-04:00

"Hey Mom?"
"Hi Steven, what's going on"
"I have a question..."
"What?"
"Did you guys hit the lotto last night?"
"No...why?"
"Just wondering..."

"Hi Steven."
"Hi Mom. I have a question."
"The answer is 'no.' We didn't hit the lotto."
"How did you know my question?"

I see the theme for the next three years of conversations.

Shop for a Cause!

2011-08-23T14:50:00.004-04:00

I've been neglecting my fundraising duties...and I feel horrible about that!

We didn't plan a pancake breakfast this past May because we thought Steven would be having brain surgery...good reason...but still I felt ~~a bit~~ very bad. I thought we could do a breakfast in December, when he is home, but now I've decided, in light of my recent health issues, to skip that also. Again...good reason...but I feel ~~a bit~~ very bad that we aren't helping fund the cure.

Imagine my pure delight when I realized that we can still work together to find a cure for my sweet son.

This coming Saturday (August 27) you can "Shop for a Cure" at Macy's! All that you need to do is contact me, or the VHLFA, for a "saving/shopping pass" (purchased for a mere $5), and then you shop!

And save 25% all day long!
On regular, sale and clearance items!
Oh the joy!

The best part...100% of your $5 saving/shopping pass will benefit our mission!

Law School

2011-08-21T14:47:00.004-04:00

True Stories
For the Record.

Steven bumped into a fellow law student while leaving his torts class the other day. The other student looked at him and asked, "Did you have intent"?

For the record, my sweet son appreciated the humor in this statement.

It seems that many of my sweet son's fellow law students are driving Porsches and other fancy stuff.

How do they do that?

For the record, my son has a 1999 Mercury.

Ready

2011-08-15T11:03:00.007-04:00

My sweet son is starting his law studies tomorrow at the University of Miami.

I have spent the last part of this rather tumultuous summer preparing for this fact and the changes that it means for our family.

I've watched Bad Boys I and Bad Boys II.
Several times.

I read, and re-read, several John Grisham novels.

And last night, in a bit of supreme planning by the cable networks, I was able to view Legally Blonde.

Steven and Dale, ready for the cave!

Steven has also spent the summer preparing for this big step. He's read several books about outlining and other skills that will be so important that first year. He and Dale made a quick trip to Miami during the latter part of July and found a great two bedroom apt with an even greater roommate! And he completed financial aid forms that will basically keep him working hard for the next 30 years.

Today is "reading day" for first year (L1) law students at UofM.

My sweet son opened his email this morning to find his reading assignments.

He has to read an entire book!
And then some!
Today!

Oh dear...I hope he's ready!

News from Building 10

2011-08-04T21:51:00.003-04:00

The Good News

The cyst is the same as it was last May with Dr. V.

It's just a small blimp at the bottom edge of the brainstem tumor.

The Bad News

We didn't get to see the famous Dr. L. In fact, even when things are moving and shaking and looking surgery-bound in my sweet boys brain, we've never seen Dr. L.

We also didn't get to see the fellow until 12:05. (I understand waiting patiently...but we are also on a shuttle/plane schedule!)

I did quickly ask, as the fellow quickly assessed my sweet son's neuro function, if this has ever been seen before with VHL brain cysts, and it hasn't. I was also able to quickly ask if the fellow had an explanation for the disappearing cyst, and he said, "The cyst might have ruptured."

And then we left!

If I hear "we don't know" even one more time...
I'll explode.

News from the Inn

2011-08-04T08:29:00.006-04:00

We just can't help it!

Every time we've checked into the in for the past six years, yes! six years!, I've used the elevator to carry baggage to the room.

I can't help it!
Whether it be from the typical early flight, the two hours in the air, or the monotony of long hours of waiting in the airport, I'm exhausted by the time we reach the inn.

My sweet son, on the other hand, has raced up the stairs for six years, yes! six years! He has boundless energy, takes the steps two at a time, and likes to goad me for my travel weary ways.

He can't help it!

And he beats me to the second floor every time!
We've played with rules and changed approaches, but without unforeseen circumstances like a stampede of five year olds or a broken tibia, I doubt that I'll ever beat Steven to the second floor.

I just can't help it!

Steven's itinerary from the nurse at NIH allowed him reimbursement for a hotel room this trip. Because my sweet son now receives these itineraries himself, and because of his general lack of planning, we didn't know that we had the opportunity to stay at a hotel this time until well after we were checked into the Inn.

My sweet son is honored that he is being acknowledged for the adult that he is, and he will gladly stay at a hotel next time.

I'll be sad when the "race to the room" doesn't happen any more.
I'm tired of these big changes.

We just can't help it!

I hope my sweet son will treasure the memories of "the race to the room" half as much as I will.

We Leave Tomorrow Morning

2011-08-02T18:28:00.002-04:00

We're NIH bound in the morning, via the Baltimore airport and the lovely NIH shuttle service.

Steven's itinerary for tomorrow includes phlebotomy, a nice dinner of his choice, and a very late MRI.

Thursday morning we'll see the brilliant doctors in neurosurgery and try to reach some conclusions about this missing cyst. I plan on seeing the most scans and seeking answers.

We'll be home late Thursday evening.

Because I Live with a Genius

2011-07-21T17:47:00.006-04:00

I get to hear conversations like this,
which fully support and explain Schiffer's dual brain theory.

"Mary, please dont text while I'm talking to you."

"Don't worry Mom. I have two brains."

Sometimes it's best to just sit back, be quiet, and enjoy the treat that will come out of her mouth eventually.

"Yeah, I learned this in science. One side of your brain is for listening, and one side of your brain is for talking. Texting is basically talking, so I can do both at once and fully hear what you're trying to tell me."

Love this girl!

Brotherly Love?

2011-07-15T19:55:00.003-04:00

They're home now.

We're not,

and I miss my boys.

I'm a self-admitting neurotically-obsessive mess of a mom.

Guess Where We Are

2011-07-12T19:51:00.001-04:00

...and guess what we did!

Words Heard

2011-07-07T20:16:00.004-04:00

...before you leave on a 1100 mile adventure...

"Do they have a rainy season in Pennsylvania?"

"No. They have a snowy season in Pennsylvania."

Pray for this sweet son of ours.
I am afraid that he will traipse the campus in sneakers during that first snow storm!

Summer...Part Two

2011-07-03T09:13:00.009-04:00

Part One

I am concerned that these two little doggies will have a very difficult time adjusting to their new Sunday morning schedule after August 15.

Part Two

I am concerned that, as my children grow and journey to far off adventures, I will quickly become one of those old women who fulfills her nurturing needs by

pushing her doggies around in a stroller

carrying small pets in a cute little handbag

sleeping with a menagerie of four legged friends

making cute little Halloween costumes for cute little doggies

posing for church directories with "furry" children

Seriously.

Summer

2011-06-27T18:07:00.003-04:00

The best part of summer

has been staynig up late and
watching scary movies every night.

Every night!

I do believe that I'm going to miss summer come mid-August.

...sigh...

Words Heard

2011-06-04T17:07:00.004-04:00

...when great minds meet...

"You know, Abraham Lincoln was a great president."

"I know, and Martin Luther King Jr. was a great doctor."

My Daughter Has A Problem

2011-05-23T18:30:00.005-04:00

She never listens to me!

I keep telling her to stop growing up,

but she does it anyway.

This bold and brassy misbehavior is seen every morning,
when she counts down the days until she is
officially

a

high school student!

(The official countdown is at 13 days.)

Sob!

Nothing

2011-05-14T12:55:00.017-04:00

...but a missing cyst and clandestine plans...

Missing Cysts

Last December, during a routine scan here at home, a cyst was seen in my sweet son's brain, attached to a tumor and snuggled against the brain stem.

In late December Steven started an oral prescription medication for hair loss.

In February the cyst and accompanying hiccups were noted by the brilliant doctors at the NIH. These doctors, who study VHL more intensely than anyone else, suggested immediate surgery. My sweet son, because he was in the middle of the last semester of college and didn't want to ruin his plans for an August start at law school, asked if the surgery to remove this tumor could wait until May.

After a long and convoluted conversation about symptoms, dangers, and outcomes, all agreed that Steven would wait until May, return to the NIH, have this nasty tumor removed, and start law school without any hiccups or VHL worries.

On the way home I began talking to my sweet son about the importance of keeping his local neurosurgeon advised of these plans. So Steven made an appointment with this Dr in mid-March, and I accompanied him.

Dr. V saw the cyst, commented on symptoms, and asked why the drs at the NIH would recommend surgery when the only symptom is hiccups. We explained what the good drs at the NIH told us. To the best of our muddled understanding, the drs at the NIH are finding that brain stem tumors almost always need to be removed, that beginning symptoms are a precursor to horrible side-effects of the tumor, and that they feel now that it's better to remove these tumors before the horrible side effects occur, as swallowing problems, numbness in limbs, and constant nausea don't always cease after surgery.

Dr. V listened intently, nodded his head a few times, and told Steven to re-scan in May and come back to see him.

Which bring us to the most recent scan of my sweet son's battled brain.

The cyst has all but disappeared.

No miracles, folks, as a miracle would mean that the cyst had completely disappeared. A miracle to us would be a brain free of disease or a gene that corrected itself. A miracle would be a drug that prevented further surgeries.

This is just a cyst that has followed a path not seen in VHL and diminished to a size that is barely visible.

After looking at the recent scans, that were sent overnight to the NIH, a resident dr working in neurosurgery at the NIH phoned my sweet son. This dr mentioned that the difference in the cyst doesn't appear to simply be a different cut of the MRI machine. This dr asked Steven if he has had radiation to that tumor, in an attempt to explain this finding. Steven, of course, replied "no", but he did mention this medication for hair loss that he's been taking since late December. The resident told my sweet son that his scans would be reviewed the next day by the lead drs in VHL studies at the NIH. The next day, in a flurry of missed calls and email correspondences, Steven was told that VHL brain cysts don't simply disappear or diminish significantly in size. He was also told that these drs won't answer any questions about what could make this cyst diminish so brilliantly until his next appointment in August, because cysts attached to tumors in the brain of a VHL patient simply don't diminish significantly. These doctors also deferred to Dr. V in regards to surgery plans. Dr. V, of course, doesn't see surgery as an option now. He would prefer to watch this tumor closely and be informed of the next MRI from the NIH. This MRI will happen in August.

August is also when my sweet son starts law school.

My son, to say the least, is frustrated. He's frustrated because his plans for this summer, including a meaningful internship that he had to pass up and exciting travels that couldn't happen, were cancelled, based on a brain surgery that was all but written in the books. He's abundantly frustrated that even the most brilliant drs in this field don't have answers for him. He is most frustrated that he's now expected to start law school with what we consider a very unstable tumor.

And we, by association, are frustrated.

Clandestine Plans

I teased a bit back in late December, amid the tears and disbelief that accompany a bad scan, that this prescribed medication that my sweet son was taking to treat hair loss might be the answer to prayers for thousands of VHL patients.

I was simply teasing.

After seeing the latest scan, however, I began thinking about the timeline of the changes in the cyst and the medication, the fact that this medication inhibits the formation of certain male hormones, the common but unproven belief that VHL tumors respond negatively to hormones, and the response from the NIH doctors that VHL brain tumor related cysts don't simply go away on their own.

And I brilliantly decided that the decrease size in the brain stem cyst was directly correlated to the taking of this medication.

And I suggested to my sweet son that we now work on proving causation between the medication and the cyst in the little lab that we like to call "the upper left shelf of the corner kitchen cabinet".

I hope this is the miracle pill.

Words Heard...

2011-05-11T18:26:00.000-04:00

...through the phone lines.

After another aberrant twist and unexpected turn
in the life of this nasty brain stem tumor,
I unleashed a rant of epic proportions
while on the phone.

My poor mother had no choice but to listen.

When I was done grumbling and whining about
the atrocities that face my sweet son,
my mother replied,

"I'm not quite sure what you want me to say."

Sometimes I just need to vent.
And I'm so thankful that my precious mother understands
that sometimes I'm not looking for answers.
Sometimes I just need to vent.

For Aunt Jan

2011-05-07T09:22:00.006-04:00

Take lots of pictures before the big event...

because it's difficult to get a good picture at the ceremony!

The Week in Preview

2011-05-02T19:00:00.006-04:00

Monday: Go to work, pick up my girl at school, stop by the grocery store, cook dinner, wash dishes, do a load of laundry.

Tuesday: Go to work, run home and warm the left-overs, wash dishes, attend a school affair, do a load of laundry.

Wednesday: Go to work, pick up my girl from school, run to the university and see my son for a few minutes, pick up dinner, eat dinner, wash dishes, do a load of laundry.

Thursday: Attend the graduation ceremonies of my oldest son,
celebrate,
savor the memories,
acknowledge his accomplishments,
appreciate the sweetness of this semester,
shed a few tears and use a few tissues.

Friday: Go to work, bask in the beauty of grace, pick up my girl from school, shed a few happy tears, stop by the grocery store, bask again, cook dinner, shed a few more tears, wash dishes, do a load of laundry, bask in the beauty of grace one more time, watch a movie, fall asleep at peace.

Conversations

2011-04-23T15:32:00.015-04:00

"Mom, I'm really busy studying today. Can Dad and David run to Lowes and get the free trees for EarthDay?"

"Free trees? Wow!"

"Yeah, they'll be great for the backyard."

His father was not amused by the size of the "tree".
His brother was.
And his puppy considered this "tree"..."lunch"!

"Steven, is 'The Avengers' coming out in theaters this summer?"

"No, Mary...how do you know about 'The Avengers'?"

"Because you took me to see 'The Hulk' that one summer and you told me all about the Avengers."

"Well, 'The Avengers' doesn't come out until next summer, but this summer we can see 'Thor' on May 6th and 'Captain America' on July 22nd."

And my super-hero-loving, movie-going son graduates from college in two weeks. I think I know how he plans to spend the months before law school starts. Now if only a Batman and Spiderman movie would premier this summer, his life would be complete!

"Dad, tell mom that she doesn't need to come for this MRI."

"Ok."

"Ask her if she'll be there for the appointment."

Always my sweet son.
Always.

Tax Day

2011-04-18T19:27:00.005-04:00

Several weeks ago my sweet son called and asked me if I had completed his taxes. I told him that I had estimated his taxes, and it seemed that, even as a college student who works a few hours a week, he owed the government money. His sweet reply was, "Well, please finalize the forms so that I can pay what I owe."

A little more than a week ago, my sweet son called again and asked if the forms were complete. I reminded him that he still had many days before taxes were due. "But mom," he said with a laugh. "I have to pay my taxes now in case the parents of a poor five year old with diabetes needs that money for medicine."

Maybe we should all pay our taxes with a bit of light humor...

and the integrity of my sweet son.

Words Heard

2011-04-16T08:51:00.003-04:00

...When Opposites Attract...

"I think something is eating the lettuce."

"Beans, Dale...they're beans."

Did You Know

2011-03-06T15:39:00.006-05:00

that my
sweet smiling,
book reading,
peace loving,
disease fighting,
deep thinking,
soft speaking,
cause finding,
soccer playing,
movie watching,
scar wearing,
knowledge bearing,
environment saving,

son

also
plants gardens?

FYI

2011-02-27T13:42:00.011-05:00

Just so you know:

Getting accepted to law school

for the second time in two weeks

is pretty amazing,

and so is celebrating with your family!

Just so you know:

-My sweet son is now expecting an acceptance letter
on every Friday for the next six weeks!

-Steven has an appointment with Dr. V,
here in Tampa, in late March,
and he will begin to make decisions about surgery
to remove that nasty brainstem tumor then.

-We're being brave,
expecting the best,
living in hope,
and ordering graduation announcements
tomorrow!

Flexible

2011-02-18T16:54:00.013-05:00

My sweet son has learned to be flexible,
living with this disease.

He understands that surgery is pending,
and he won't be allowed to travel this summer,
let alone run with the bulls.

He accepts these disappointments
with such grace and maturity.

Usually.

But now my sweet son is insisting
on a graduation gift to Pamplona
and a chance to run with the bulls

after

he graduates

LAW SCHOOL!!!!!

(He has received his first acceptance letter!)

Preliminary Report

2011-02-03T13:06:00.003-05:00

It looks as if
my sweet son
will not be

running with the bulls
this summer.

Against All Odds

2011-02-02T00:51:00.003-05:00

Against all odds
my sweet son and I
flew on two different airlines,
into two different states,
at very different times of day,
and we both arrived on time!

Against all odds,
the DC airports seem to be covered
with a small pocket of tepid air
that is surrounded
by a maelstrom of ice, snow and sleet!

Steven has an early MRI in the morning,
followed by a visit the next day
with the brilliant neurosurgeons,
who will help us make plans
to allow Steven
to
dream his dreams,
and
live his life
against
all
odds.

Words Heard

2011-01-06T16:08:00.003-05:00

...from the land of the little people...

"My uncle was in an accident yesterday."

"Is he ok?"

"Yeah, he's ok, but his car is a nervous wreck."

You have to love the little people!
They keep you sane!

Wishes

2010-12-30T20:22:00.007-05:00

...from deep within the trenches...

Our house has been a flurry of activity for the past week, from the frenzy of Christmas morning to the aftermath that a Christmas puppy brings. We've feasted with family, visited with friends, and had more family movie times than I care to even mention!

What a blessing this season, so full of joy and hope, has been to my soul this year.

Just a week ago I was still so fiercely battling the effects of seeing the unavoidable fate that is my sweet son's last brain scan.

Just three weeks ago I was living near tears after watching a recent episode of Grey's Anatomy that featured a VHL warrior.

But this week I'm beyond tears and feelings of doom. The past few days have been nothing but Christmas. And because I know that nestled among all the trimmings, hanging on every tree branch, and wrapped in each and every gift, is the story of the birth of baby Jesus, this past week has been filled with peace, and joy, and hope.

And wishes.

1. I wish that eveyone would watch that episode of Grey's Anatomy.

While television can't portray the disease exactly as it's lived, this episode touched my heart.

And after you've seen the show, please, contact our local ABC news station and convince Ms. Linda H. that we need a human interest story about VHL on a Thursday night after the show!

(I'm floundering on this front!)

2. I wish for a cure for my sweet son.

Just freedom from bad scans and brain surgeries.
Just the ability to live a life without cancer.
Just a cure.
Nothing less.

Dear Santa

2010-12-28T21:29:00.003-05:00

Thank you for the new puppy,

but I think I would have preferred the lump of coal.

Love,
Daisy

Sustenance

2010-12-26T18:39:00.004-05:00

My sweet son likes to say that he comes home for the food.

I always thought he was teasing,

but maybe he's not.

Yearly MRI

2010-12-23T08:11:00.006-05:00

Steven had his brain MRI and follow-up visit with Dr. V. last Tuesday. At his preference, I sat in the waiting room.

Dr. V. reported that the tumors in Steven's brain are stable, with some slight growth in a few tumors in the cerebellum.

I peeked at the MRI disc after we returned home.

I see a much dreaded cyst attached to the brainstem tumor. This is a new, and bad, development.

He's headed back to NIH in early February. We'll know more then.

There Are No Words

2010-11-04T20:17:00.002-04:00

Scary

2010-10-23T19:30:00.010-04:00

...brought to you by the month of October...

My sweet boy wants to be Paul Bunyan for our Halloween jaunt through the neighborhood.

And while the thought of Steven dressing like a mythological lumberjack who befriends a blue ox doesn't pariticularly frighten me, the beard that he had to grow to complete the look is totally!
Freaking!
Me!
Out!

...brought to you by the world of VHL...

The small VHL tumor that was discovered on Steven's retina this past summer is now showing signs of growth and needs to be treated. My sweet son will have laser sugery, though we really are hesitant to refer to this incisionless and stitches-free treatment as surgery, on his eye this coming Monday.

While we're confident that this treatment will stop the tumor growth and prevent the loss of vision that so often happens in VHL warriors, we feel so much for our sweet son it hurts.

Words heard...

2010-09-22T22:00:00.003-04:00

...five weeks into the new school year.

"First grade is kicking my bottom."

Just so you know.

Did you know...

2010-09-06T09:44:00.009-04:00

...that my sweet son is now a college senior,

and his devoted dog is becoming seriously sad over the thought that Steven will soon be moving on and exploring the world without her.

As am I.
As is his dad.
And his brother.
And even his other sister.

...that our sweet son wants a plane ticket to Pamplona as an extra special graduation gift.

...that Steven has an internship this semester at a local office of a member of the U.S. House of Representatives. He is thrilled!

...that our sweet son recently purchased his first car.

...that I will edit photos to smudge out dings and dents!

...that every second
of every minute
of every hour
of every day
Dale and I both realize
the implications
of living with VHL.

...that not a day
nor an hour
nor a minute
nor a second
goes by
that we don't give thanks
for the life that our sweet son
lives.

Words heard

2010-08-14T22:18:00.002-04:00

when my husband enters the kitchen.

"The directions say to turn

the oven on the

800 degrees."

Nice!

Observations

2010-08-05T19:19:00.008-04:00

from the NIH

1. First and foremost, stable is a beautiful word.

2. My son whispered, "You staying out here, right?", but my needs prevailed.

Now I feel bad.

3. The depth and scope of a neurological exam seems to be indirectly proportioned to the years that a neurosurgeon has been practicing.

3a. I'd swear that the neurosurgical fellow was testing reflexes in my sweet son's pinkie toes today.

4. Being in a protocol here at NIH is a privilege. "Children" who smoke 30 cigarettes a day should not be given that privilege.

Enough said.

5. The Children's Inn fills my heart.

6. Shuttle drivers are under appreciated.

7. Metro cards are sensitive beasts. Treat them with love.

8. My son has adapted my mantra, "sleep cheap but eat well", when traveling.

9. We still have sites to see, despite five years of bi-annual visits.

10. Last but not least, stable is a beautiful word.

Chances Are...

2010-08-03T22:33:00.003-04:00

If you reserve a flight

based on availability of the NIH shuttle

and appointment times

in the phlebotomy lab

chances are

you will have to rise earlier than humanly possible

on the morning of your departure.

And chances are that your
sweet smiling,
book reading,
peace loving,
deep thinking,
soft talking
son,
upon hearing the proposed rising hour,
will suddenly become
indignant.

Words Heard

2010-07-28T15:06:00.006-04:00

in the world of VHL

"I'm not sure if I'm a

maniac mother

or just my child's advocate."

This mother understands my insanity.

File this Under ...

2010-07-27T17:47:00.011-04:00

...tough.

This summer has been tough for my sweet son. In ways that I expected but perhaps he has never digested, the disease continues to work it's way through his body as he ages.

While my sweet son handled the news of the epididymal cystadenomas with his usual grace, the realization that his retinas are no longer disease free hit him with a punch.

File this under...

...lessons.

As we absorbed the news over brunch, Steven broached the subject of this blog and my lack of recent writing. I reminded him that I'm often uncomfortable writing about his life as he finds his wings, and that I prefer to err on the side of being cautious.

But that difficult mornign he asked me to write.

A book.

About this journey.

I told him that I wouldn't be able to tether my stories to decency and carefulness if the memories were to be true.

He agreed.

I told Steven that I would have to write about often wishing he had been diagnosed with a different disease.

He agreed.

I told Steven that I wouldn't be able to tell this story without describing how now I'm not afraid of anything because I'm fearful of everything.

He agreed.

I told Steven that I would probably lose some friends when I described that comparing Steven's illness to sinus infections sends me over the edge.

He agreed.

I reminded Steven that this story would have to be honest and true.

He agreed.

And I my sweet son that he has so many lessons to share with the world.

He agreed.

The Facts, and More!

2010-03-29T20:00:00.005-04:00

The Facts

We're planning our third pancake breakfast fundraiser to cure von Hippel Lindau disease.

Saturday, May 22nd, 2010.

Cure Steven
Cure VHL
Cure Kidney Cancer

More!

I always hesitate with fundraising for the cure for this disease. As passionate as I feel about this,
various and sundry thoughts keep me from committing until the last moment.

But it needs to be done...that's a fact.

More Facts

In six months, a year or maybe two, this rocky economic time will be at the back of everyone's mind as a very bad period of their financial lives.

In six months, a year and definitely two, my sweet boy will still have a life-threatening disease that causes cancer and more.

And at that period of time when everyone has recovered their funds and all are looking toward a brighter future, we will still need a cure for von Hippel Lindau disease.

Please join us for pancakes
Please celebrate a cure with us.

More info to come!

Just So You Know...

2010-03-22T14:52:00.014-04:00

...this is personal.

I, along with the majority of our nation, watched yesterday as our elected officials in the House of Representatives debated about and voted for the health care reform bill.

But for us, it wasn't about political positioning, yellow journalistic ramblings, lobbying to maintain a status quo, or public displays of disapproval.

It was personal.

Millions of people in this country have what is termed a pre-existing condition. This conglomerate mess of maladies varies from something relatively minor, such as an old sports injury, to an illness of great impact, such as heart disease or cancer.

Cancer, the mother lode of pre-existing conditions, affects roughly 11 million Americans.

And all people with von Hippel Lindau disease, regardless of the state of their health, fall under the category of "cancer."

So this need for reform was personal to our family.

Health insurance companies are not here to save lives. They are in the business of making profits, huge profits. People with cancer are a liability to insurance profits, a huge liability. And as of yesterday insurance companies routinely denied private health care plans to individuals with a cancer diagnosis.

Routinely.

Our sweet son is quickly approaching an age and stage in life which will prevent him from being covered under our employer-provided family plan. So I started testing the water, so to speak, of buying an individual health insurance plan for Steven. But despite my best efforts of searching for alternatives, he was routinely denied coverage by all health insurance companies that we contacted.

Routinely.

My sweet son was simply one year away from being uninsured.

With a pre-existing condition.

And a nasty brain tumor on his brain stem.

And spots on one kidney.

From Steven's perspective, and the personal situations of 11 million others, the need for health insurance reform was huge.

************************************************************************************
There are many resources right now that can explain the nuts and bolts of this plan. While I still think that the plan would be better with a public option, for my very sweet, personal reason, the bill that was passed yesterday is not the demonic creature that many are claiming.

It will not raise taxes for families that make under $250,000 per year.

It will not allow the IRS access to your personal bank accounts.

It will not allow the government to decide if you may receive treatment or if you should die.

It will not take precedence over a group health care plan that you already have.

All major changes are uncomfortable. Please stretch your comfort zone. Read a neutral analysis of the bill. Research your concerns from impartial sources. Stay informed from a source that doesn't include the letter "F", "O", "X", "B", "E", "C", or "K" in its name.

*************************************************************************************

Did You Know

2010-03-15T17:52:00.010-04:00

(Particularly if it involves kindergarten and magnetic letters.)

Apparently so does his dog!

The Lustrum

2010-03-01T19:49:00.021-05:00

Five years is a long time,

The passing of 1825 days, however, hasn't diminished my memories. Sights, sounds, even odors familiar from Steven's first hospital stay can still cause my stomach to turn, my eyes to tear, my heart to break.

and so much has changed.

Steven was a high school sophomore when he had his first brain surgery. He's now a college junior, of legal age, making plans for graduate studies.

My Mary was just in second grade when Steven was first diagnosed with this disease. She's now a teenaged, clothes loving, mall walking, boy watching, texting fiend.

I've aged.

We've had the opportunity to attend two VHLFA conferences and learn more about VGEF, HIF, genes and cells, processes and proteins. We've also been blessed by meeting many kindred spirits fighting the same fight.

I've had the chance to become more involved in the family alliance, at a local level, and now lunch with several VHL warriors monthly.

We raised funds and raised awareness for the first time ever.

But because some things haven't changed,

This disease carries on in its own insidious way in my sweet boy. His two small benign brain tumors have turned into eight tumors in the cerebellum/brain stem area and four in the spine. His pancreas is now muddled with cysts of varying sizes, and his kidneys now also show signs of the disease.

One small and crafty gene continues a path of destruction.

And my sweet son remains unbowed
with his grace,
and his determination,
and his sweet, sweet spirit.

we need to remember the past

That first night home, after settling Steven in bed and checking both the incision at the back of his head and the small sutured spot on his upper leg, I sat at the computer and researched hemangioblastomas. I read statistics that not only clinically diagnosed my son with von Hippel Lindau disease but also wanted to cut his life in half.

I'll never forget those printed words...how they looked, how they sounded, how they felt.

Those few simple words were a catalyst.

while we reach for the future.

Words of pain can also promote great change, change beyond our wildest imaginations. We now know truths that only the privileged know. We understand that another breath is a miracle, and we know that life is made of moments.

And we know and believe that
with a bit more research
and lots of faith,
a cure will be found.
Our sweet son
must have the privilege
of living this good life
filled with hope.

She Doesn't Understand

2010-02-28T18:33:00.005-05:00

...my insanity...

"I need a new bed."

"But your bed is so comfy."

"I don't like how it looks."

"But your bed is beautiful."

"I got it when I was like 5 Mom. It's for babies."

She just doesn't understand.
She's my baby.

Fluff and Stuff

2010-02-08T12:07:00.003-05:00

Not My World

2010-02-07T22:22:00.007-05:00

In the midst of touring the White House, surviving the blizzard of 2010, obtaining a library card for the Library of Congress, being pelted by a multitude of snowballs, and enjoying time with family, Steven had a clinic visit at the National Institutes of Health as part of the protocol studying the natural history of CNS hemangioblastomas.

After a brief debate in the waiting room, mostly with myself, about the benefits of having a second set of ears for information during a doctors visit that involves the words "neurosurgery" and "brain tumors", I decided to go in the exam room with my sweet son.

I also promised that I wouldn't ask a single question.

The doctor, the endearing and energetic Dr. W., basically relayed the same news that Stevens' local neurosurgeon, Dr. V, told him in December. All tumors are stable since last August, and slow and plodding growth is noted in several cerebellum tumors and the brain stem tumor in the past several years.

And I stayed true to my promise of being quiet during this exam. It really was quite easy, as my son is becoming very adept at handling questions posed to him and asking for the pertinent information that he needs to satisfy his mama and better take care of this disease.

And I'm learning to give him the space he needs.

I realize that this is his body. This is his disease. This is his place to ask the important questions.

And he's living with this disease in an amazing way.

This is Steven's world.

I'm happy to be part of his world,

but

this is not my world.

2010-02-04T19:51:00.003-05:00

We toured the White House this morning.

I left a note in the Blue Room.

Tuesday Morning

2010-02-02T08:30:00.009-05:00

It's Tuesday morning,
and I'm feeling anxious and edgy.

We're flying out to Baltimore this morning,
en route to the House of Healing.

I don't believe that my son's vulnerable body
and precarious health is giving me
fits of anxiety this morning.

I'm fairly confident that all
will be stable in his brain and spine.

I'm not able to pinpoint the source
of my nervous energy.

But...

it may be
because, through a planning snafu,
Steven and I are booked on different flights today.

it may be
because we've packed our days
with very tight schedules
and I don't want Steven
to run late
for the privilege that is NIH.

it may be
that my beautiful girl
is battling tonsillitis,
once again.

it may be
that my middle child
is testing more boundaries
than I even knew existed.

it may be
that my mental to-do list
far exceeds
my feeble memory.

it may be
that the bills that I so purposefully
paid, sealed, stamped, and planned to mail yesterday
took refuge in my overhead baggage
and are traveling to Baltimore today...

(Seriously...I'm taking my signed, sealed and stamped bills with me!)

it may be
that Bethesda is experiencing
an Arctic blast of cold and snow,
at the very same moment
that the greater Tampa area
sold out of gloves.

I'm gloveless people!

Gloveless!

And this may just be the source
of my anxiety this morning!

Out of the Mouths

2010-01-31T23:55:00.004-05:00

"Mom, you know if Steven wasn't sick, you never would have found yourself."

As I navigated the many one way streets that make the grid of our city and tried to land within walking distance of our final destination, because close approximations are my specialty, I had to stop and wonder at the profound thoughts that sometimes escape my daughter's mouth.

If Steven wasn't sick, I would never have found myself.

"What do you mean Mary?"

"Well, before you were always scared to drive in the city."

I turned right at the next light and parked six city blocks from the library, close enough for me.

Close enough, my smart, beautiful girl, close enough.

Somebody

2010-01-07T22:30:00.005-05:00

Somebody is ending a nice long break.

Somebody has to work on Saturday afternoon.

Somebody is predicting snow for Saturday morning,

in Florida .

In Summary

2010-01-01T20:23:00.009-05:00

It has been brought to my attention, through a phone call from my sweet mother and an email from my equally sweet sister, that I have failed to give the official, Dr. V approved, December MRI results!

In summary,

(This has to be simply a summary since I sat in the waiting room this visit)

one tumor in the cerebellum and the nasty brain stem tumor

(and I don't know all of the minute details,)

show minimal growth over the past year.

(and I'm having a very hard time with this new stage)

The tumor in the cerebellum has plenty of room to grow

(of letting go)

before it causes problems that would require surgery.

(because my simple addled brain)

The brain stem tumor, while continuing it's lackadaisical pace of growth,

(truly believes that if I have all of the information)

is closing in on the magic size that will cause blockage of the fourth ventricle

(I can somehow gain control of this horrible disease)

and will require surgery in a year or two...or maybe three.

(and heal my sweet son.)

Love

2009-12-25T20:36:00.011-05:00

"For God so loved the world that he gave his one and only Son, that whoever believes in him shall not perish but have eternal life."

Superhero

2009-12-17T18:39:00.008-05:00

Sweetly handsome college kid by day...

disease bearing tumor fighter by night.

Actually this sweetly handsome college kid fights disease during the day too...all day long, every day. He spent the better part of four hours in the MRI tube this morning, checking the many tumors in his brain the the few in his spine. My guess is that all tumors are stable, judging by the lack of new symptoms, but we'll wait until next Tuesday for the final word from the good Dr. V.

A Glimpse

2009-12-09T07:58:00.005-05:00

Into My Insanity

"I know what I want to be when I'm done with school."

"A baseball player?"

"No. A nudist...will you support me in my dream?"

"Sure."

"Will you still support my dream
if you have to bail me out of jail when I leave the colony without clothes on?"

My middle child...
he dreams big.

Just

2009-12-07T19:42:00.004-05:00

Because

it's finals week,

and my sweet son is also working his way through college,

and we love him immensely,

and his caloric intake never seems to keep up with his metabolic output,,

and I wish I could make his life easier,

and it's the small gestures that matter,

he had "Sunday night dinner" on a Monday afternoon!

Sometimes

2009-12-06T22:37:00.006-05:00

Sometimes
I see friends' children who are 16 years old,
and I realize what a fearful, gut-wrenching year that was in my sweet son's life.

Sometimes
it just hits me,
and it takes my breath away.

Sometimes
I meet a child who is a sophomore in high school,
and I realize that was the year my sweet son's life changed forever.

And sometimes
it just hits me,
and it takes my breath away.

Sometimes
this is tough.

Thankful

2009-11-26T18:09:00.002-05:00

Enough

Give thanks in all circumstances.
1 Thessalonians 5:18

Give thanks always?
And in every situation?

Even when
You are settling in a new land?
And you are scared?
And the winter winds blow through the cracks?
And famine is unrelenting?
And disease is destroying your dreams?

Give thanks in all circumstances.

Give thanks always?
And in every situation?

Even when
The CT scan is ominous?
And the MRI is worse?
And surgery is unsuccessul?
And the diagnosis shattering?
And there is no cure?

Give thanks in all circumstances.

For thankfulness leads to
Joy.
And joy leads to
Hope.

Hope is all we need.

Hope is
Enough.

Thoughts from November 2006,
still true today.

Did you know

2009-11-25T21:10:00.012-05:00

that my
sweet smiling,
book reading,
peace loving,
disease fighting,
deep thinking,
soft speaking,
cause finding,
soccer playing,
movie watching,
scar wearing,
knowledge bearing,
son

is also environmentally friendly?

Today

2009-11-24T22:00:00.003-05:00

Today...

...I poked and prodded my little girl out of her sleepy dreams.

...I laundered many pieces of clothing.

...I shopped for various goods still needed for our feast.

Today...

...I waited patiently in a waiting room.

...I received a great biopsy report.

...I bought numerous bottles of sunscreen.

Today...

...I lunched with friends, new and old.

...I had the opportunity to talk "VHL" for over two hours!

...I dried many tears on my drive home.

Today
was special.

Freudian Slip

2009-11-13T18:00:00.005-05:00

Today I ordered a

"Quarter Pounder Happy Meal."

Even the sweet boy
at the drive-through window
knows
that I'm decomposing
rapidly
at the reality
that my babies are
growing up.

Celebrate!

2009-11-10T18:38:00.011-05:00

Our sweet son is celebrating

a monumental birthday.

And while Steven

asked for various and sundry presents,

we know that this day alone is the best gift.

Scary Stuff

2009-10-24T15:42:00.004-04:00

Someone has really great makings of a Halloween costume.

Rebel with a Cause!

We have the gauze.
He has the scars.

Wild Things

2009-10-17T08:13:00.007-04:00

I know

Steven and David headed out to opening night.

where the wild things are!

Partaking in first-day festivities is just one of Steven's many endearing qualities.

They're at the theater, revisiting their youth!

Yesterday

2009-10-11T09:45:00.017-04:00

Yesterday

we

picked up our sweet boy from his college digs,

shoe shopped for our two sons,

discussed world events,

ate lunch at the mall,

browsed a book store.

Yesterday

we

ventured downtown,

sweltered and melted in the October sun,

witnessed a film festival,

talked about health care reform,

let our sweet boy borrow the good camera.

Yesterday

was a blessing.

Speaking of School...

2009-08-30T19:45:00.004-04:00

"Have you told anyone that I'm ready to babysit?"

"Not yet, but I will."

"Good, because I need to start saving for a car,

because by the time I'm in high school, Dad will be in his 60s."

"What?"

"That's too old to drive me to school."

For the record, Mary's dad is 50. She is in the 7th grade, and will start high school in 2 years.
Don't do her math...it will make your head spin.

School

2009-08-29T08:52:00.008-04:00

The First ~~Day~~ Week

My beautiful little girl is in 7th grade. When did this happen and why is this allowed? Didn't anyone tell her that she is my little girl?

David seriously asked me if he could drop one of his five physical education classes because he thought it might be too much work. He's a senior this year.

My sweet boy came home for an evening. He said that he needed some supplies, but I'm secretly hoping that he just needed to see his family. We ended up having a wonderful time...watching a sweet memorial for one his his favorites, visiting the old bookstore, and just talking. In the course of the ebbs and flows that make our convoluted conversations, we ended in a discourse about natural talents, exceeding your potential, and setting goals high.

Our final conclusion:
What a wonderful world we would live in the the best and brightest eagerly sought out positions to teach.

Status Quo

2009-08-14T23:39:00.007-04:00

Steven was given another nice reprieve at the clinic Thursday morning. The good doctor declared all tumors stable. While he did mention some possible small growth again in the the brain stem tumor, Dr. G was also reassuring in telling Steven that his brain basically looks the same as it did in February.

Building 10, NIH

While thrilled to be living the status quo, this clinic visit was a bit odd. Steven's MRI was late into the evening, and Building 10 was eerily quiet. The MRI and clinic days aren't on the standard TH/F anymore! The clinic itself had moved several floors. And we didn't see any familiar "vhl" faces in the waiting room this time!

It's funny how the smallest changes can throw us off!

Waiting for my sweet boy...

The protocol nurse was able to explain away most of our restless thoughts, as the clinic had simply changed days and needed to change floors to accommodate this new time. When we laughed about being worried that Steven was being phased out of the study, this wonderful nurse did remind Steven that this study will not be forever, and that it has nearly run it's course.

The expected time frame, for now, is another year of biannual visits followed by several years of yearly visits.

Then the study concerning the natural progression of VHL tumors in the central nervous system will be complete, and my sweet boy will again be at a crossroads in the care of his diseased body.

He may decide that it is time to look for a study that treats VHL with chemical agents.

He may simply get lucky if this present study can again be extended.

He most likely will have to rely on a team of local doctors.

He will, without a doubt, need to hope and pray that this country starts finding the status quo unacceptable.

World Travelers

2009-08-11T18:02:00.004-04:00

We're headed back tomorrow

to the house of healing,

Bulletin board at the Chilren's Inn, NIH

and though we haven't truly

traveled the world this summer,

it starting to feel that way.

A Tough One

2009-08-07T16:16:00.008-04:00

While struggling to write a newsletter article about Steven's diagnosis, I was forced to re-live those early, emotional days of Steven's illness. Those days were tough.

Writing about them was even harder.

But here it is.

Diary of a Diagnosis

Steven began complaining of dizziness in December, 2004. I tended to ignore his complaints, as he seemed to be a very healthy boy, and I assured Steven that he would feel better if he slept more and drank more water. However his father, Dale, decided that further investigation was needed, and he took Steven to the pediatrician in January of 2005. Through various tests done that day, it was discovered that Steven had a lesion in his cerebellum that was likely vascular in nature. MRIs were done and neurosurgeon appointments were scheduled.

With one office visit, Steven’s life was literally thrown into a world of unknowns.

Our first trip to the neurosurgeons office prompted more questions than answers. The doctor alluded several times to two genetic illnesses, neurofibromatosis and tuberous sclerosis, that could be responsible for the two small lesions seen in Steven’s brain. However neither MRI results, nor the findings on Steven’s physical exam, nor our answers to the many questions provided a clear path for diagnosis. The only firm answers that we received to our many questions that day were that Steven was neither to play soccer nor drive until the doctor had a better idea of what was causing these lesions.

The neurosurgeon kept the MRI films to confer with the radiologist at the children’s hospital. The recommendation from the radiologist was that Steven’s spine and kidneys should be scanned. The radiologist was looking for more tumors, indicating a different genetic disease, von Hippel Lindau syndrome, as a possible diagnosis. These further scans came back negative for tumors. At the follow-up visit, the neurosurgeon indicated Steven most likely did not have von Hippel Lindau disease. She also reaffirmed that she was 90% sure that these lesions were benign tumors. However without a biopsy she could not tell us the nature of these lesions and associated edema. We opted for a definitive answer, and a biopsy with possible tumor resection was scheduled for February, 2005.

The surgery proceeded without any complications. The results, however, were surprising. The neurosurgeon met us in the family waiting room with the news that while she saw tortuous and unusual vascular structures in Steven’s brain, there was no tumor to be found. Though I couldn’t believe the news, she felt that the lesions on Steven’s MRI films would be consistent with these odd, serpentine vessels. We met Steven in the PICU and had to share with him that this horrible surgery did not go as planned and tumors were not removed.

Our sweet boy was now living in a world of inconceivable.

At this point the radiologist who had originally looked into but discounted von Hippel Lindau disease as a possible diagnosis felt that a cerebral angiogram was an appropriate next step in solving the mystery of these lesions. This too was done at the children’s hospital, and while Steven was being tended to after the brief procedure, the radiologist discussed the findings with me. He described two areas of “blush stain” on the angiogram that indicated that the lesions were small tumors called hemangioblastomas.

That evening I immediately began researching hemangioblastomas. I learned that the unusual tortuous vessels that the neurosurgeon viewed in Steven’s brain were associated with hemangioblastomas. More upsetting, however, was the mention in an article that the presence of multiple hemangioblastomas was indicative of a clinical diagnosis of von Hippel Lindau disease. That evening I read of the devastation of VHL for the first time.

That night the struggles that Steven would face in this new life seemed insurmountable.

The next three months were spent filling our time with visits to the pediatrician, conferring with an ophthalmologist, submitting blood and urine samples, and scheduling a second opinion with a different neurosurgeon while we waited for the most important visit to the geneticist. The genetic counselor and the geneticist reiterated the information that I had read weeks earlier. The discussion revolved around tumors and genetics, surgeries and statistics. And while the doctors never mentioned life-expectancy, I knew from my son’s questions that day that he too had picked up a bit of information from the internet. Blood was drawn and sent away, and we left with still a bit of hope that the two hemangioblastomas could simply be a bit of bad luck, a chance occurrence.

The final answer was delivered on May 11, 2005 when the results from genetic testing showed a deletion of three bases from the VHL gene.

Our sweet, brave son’s life changed forever to incurable.

Sometime during those early days of diagnosis, I realized that Steven was in for a long and arduous battle for his life. I also knew that how he chose to fight this battle was nearly as important as the outcome itself. I did not want Steven to live an unknown, inconceivable, insurmountable life with an incurable disease. I wanted Steven to face the impossible with hope. I wanted Steven to live a beautiful life in the face of a monstrous disease.

That he has.

Brought to you by the letter M

2009-07-29T11:24:00.007-04:00

Missing

Mary!

She's out exploring the world again, with friends this time, and having fun...

Blessed in the Storm

2009-07-23T18:28:00.008-04:00

Mary and I are home from a long and convoluted vacation with my parents.

Oh my did we ever create some memories...fun times...silly stories...new experiences...and enough site seeing to last a life time!

And just as quickly as we settled the dust and took a breath from our crazy summer, we are once again faced with a new twist and turn that a journey in life presents.

Dale will be seen at the cath lab at Tampa General Hospital tomorrow morning at 8 for an invasive test that will look at his ventricular septal defect. I'm not into "heart talk" yet...but basically the test involve a catheter that will run from his femoral artery to his heart and will have an "inside look" at the hole in Dale's heart. It will also determine the amount of blood that is moving in the wrong direction and evaluate the two valves that seem to be faltering because of this incorrect blood flow.

And, as an extra special 50th belated-birthday present, the sweet doctor will also check the status of Dale's arteries!

The first cardiologist that Dale saw this summer indicated that since this hole didn't close on it's own during childhood, and since there are some signs of high pressure in Dale's right ventricle and pulmonary artery, surgery will be needed.

Results from tomorrows testing are expected to support that doctors opinion.

Long story short,
we still feel blessed...

and we'll praise Him
in this storm.

Relfections from our Universe

2009-07-01T20:41:00.011-04:00

Notes from a Conference

* When checking into the hotel, the front desk clerk asked if we were visiting for
"The von Hipe..the von hipp...the family reunion?"

We certainly were!

* The health and nutrition presentation was amazing this year!

* Kale, a super-power in the world of antioxidants, reportedly makes a delicious dish when combined with pine nuts, dried cranberries, and olive oil.

*Eat your apples! Though most accept the old adage, "an apple a day keeps the doctor away", the nutritional specialist recommended up to six apples a day.

Of course that makes sense to the VHL population..."six apples a day keeps the geneticist, ophthalmologist, neurosurgeon, urologist, endocrinologist, and oncologist away!"

* Dr. G rocks the world of ophthalmology!

* We now have a much better "plan" when Steven's eyes show the ravages of the disease.
Knowledge gives peace.

* "I never forget my VHL patients."
And, of course, I got teary at the sentiment..

* A molecular biologist I was never meant to be.

* The anti-angiogenesis chemotherapy drugs, Avastin and Sutent, act on very different parts of the cell.

* I can nimbly throw around terms like human vascular endothelial growth factor and tyrosine-kinase inhibitor and have some idea what minute structures these big words represent.

(Cool party trick!)

* I need to learn my HIF before next year.

* In the past 13 years, Joyce and the VHLFA have raised nearly one and a half millions dollars for VHL research.

While this is wonderful news...I want more!

Could I interest you in a little 3-day or relay for VHL?

* It is awe-inspiring to sit in a room with people describing what year they lost half of their last kidney to cancer or how a tumor caused paralysis and realize that the "brave" factor in that room can only be described exponentially.

* Pommegranite juice is being used medicinally...with no propositions needed! The super-juice has actually been shown to slow the progress of protrate cancer, and the studies are now moving on to the effects of pommegranite juice in kidney cancer!

* I realized that my mind is to small for this enormous world in which we now live. I've tried for four years to understand the truths which face my sweet son by picking apart genes and cells, processes and proteins. But this world of VHL is too big for me.

A doctor referred to VHL as being our universe.

That it is.
That it is.

Reflections from 35,000 Feet

2009-06-29T12:07:00.010-04:00

1. Though I never saw the Grand Canyon from the sky, I do believe that we flew over the Mojave Desert...but it could have been the moon by the looks of it!

2. I bonded with a fellow passenger on the flight over...sadly I don't mean in an emotional or psychological way.

3. My little girl doesn't understand "time zone theory", and we received calls at the oddest times.

4. Hollywood Blvd. is just plain odd. Venice Beach is just plain odd too, but in a fun sort of way!

5. The "Hollywood" sign is visible even through layers of throat-scratching and eye-rubbing smog!

6. My level of patience is indirectly related to the number of time zones I travel through and exponentially lowered by the number of days that I'm not in my zone!

7. Three nights stay in a nice southern California hotel requires a small mortgage!

8. Pacific Ocean breezes are more aromatic and refreshing than the Atlantic Ocean winds...sorry!

9. Fine dining can be had at a taco stand.

10. I have finally had that $5 cup of coffee...though strangley enough it tasted much like the 99 cent cup I frequently buy at McDonalds.

11. I believe that I saw a small enclave of real housewives while circling Orange County.

12. Though my brain may have been a bit radiated at 35,000 feet, I do believe that it feels good to be home...cranky kids, laundry, bills and all!

Memories

2009-06-27T01:02:00.007-04:00

Venice Beach, California
June 26, 2009

Thanks Carole!

xj5fykie67...don't ask!

We're Here

2009-06-25T21:19:00.007-04:00

And we think we've discovered a few things on this trip today that our president should know:

1. Our sweet boy is very, very brave and should be awarded a presidential medal of sort for bravery. We already knew he was made of tough stuff, enduring two brain surgeries with grace and strength,

but now we know he's braver than brave, as he wore his "Michelle's shirt" for five hours today at the George Bush Airport in Houston!

2. We found the energy crisis. It is creeping along Interstate 5 in southern California.

3. The health plan that congress is now molding needs to include Botox.
And I mean now!

(And tummy tucks!)

Wishes from the Trenches

2009-06-24T18:20:00.008-04:00

Today,

I will not...

*worry about earthquakes.

*wonder how a big plane lands.

*read stories about de-railed metro systems.

I may or may not...

*finish the laundry.

*prepare three days of meals.

*run to the post office.

I most definitely will...

*watch a special video.

*read a bit more about chromosomes, proteins, VEGF, and elonginB and C, and be prepared to learn more.

*wish

...for a safe trip to California.

...for good times with great people.

...a cure for my sweet boy.
Nothing less.
Cured.

Happy Father's Day

2009-06-21T08:38:00.004-04:00

"Our fathers toil with hands and heart
To make our lives complete.
They quietly brave the winter cold,
Endure the summer heat."

The Summer of My Insanity

2009-06-09T17:58:00.010-04:00

Short, sweet, and to the point.

1. Dale has an appointment with a cardiologist on June 19th to check the status of his newly "re-diagnosed" ventricular septal defect.

I'll be sure to attend that appointment with him, and I might ask the good doctor to check Dale's ears...since he says "what?" every time I talk to him!

2. David had a little accident in my car last week.
(I'd share pictures of my missing front end, but my computer had a little accident too.)

Does it frighten anyone else that he hopes to pay for his soon-to-be-exorbitant-car insurance by working at a little bunjee jumping kiosk at the mall?

Oh my!

3. Two bills for Steven and Dale's health issues arrived this week.

Oh my!
Exorbitance!
Again!

4. I am borrowing a nice, new car from my parents right now. I had to drive their old car one day last week...mine missing the front and all...and my dad told me, after I was already seated, belted and in gear, that the transmission was slipping.

I made it home safely by shifting into neutral above 30 m.p.h., coasting, and then shifting back into drive below 30 m.p.h.

Unfortunately that was a bit too much technology for me, and now my brain is slipping too.

5. My little girl is growing up!

Is that allowed?
Someone stop this insanity!

Because I Live with a Genius

2009-06-03T09:25:00.005-04:00

I have to draw the line somewhere

Child 1: "Mom, someone didn't flush again."

Mom: "Please remember to flush the toilet."

Child 2: "But that takes a gallon of water everytime."

Mom: "It doesn't matter...flush!"

Child 2: "But we need to conserve water."

Mom: "Stop taking 20 minute showers, then I might talk about not flushing the toilet. OK?"

In Case You Were Wondering

2009-05-26T15:54:00.010-04:00

In case you were wondering,
even after the flurry of serving close to 200 people at our breakfast,

we're still making pancakes,

and eating them too!

And we're still looking for a cure for our sweet boy.

In case you were wondering
how that cure will be found, the following information, taken from the Alliance website, describes the protocols that are supported by our fundraising efforts!

The Cancer Research Fund of the VHL Family Alliance was able to fund 3 proposals of the 13 very high quality proposals that were submitted this year. The research that will be performed by these groups will address important clinical, translational, and basic research questions that are very relevant to our understanding of VHL and how VHL patients will be treated in the future.

Thera P. Links, M.D., Ph.D., of the University of Groningen in the Netherlands, is pursuing a project of “Visualizing VEGF producing lesions in Von Hippel-Lindau.”

The first thing that happens in the development of a VHL lesion is that the second copy of the VHL gene is inactivated in a cell. Without the VHL protein, there is heightened production of vascular endothelial growth factor (VEGF). The greater the production of VEGF, the higher the growth rate of the tumor.

Until recently, it was not possible to measure the VEGF levels inside the tumor without removing the tumor. Dr. Links and colleagues have developed a radioactive labeled antibody, based on bevacizumab (Avastin) which binds to VEGF, making it possible to visualize and quantify VEGF in tumors of colorectal and melanoma patients using a PET scan.

This project will use PET scans on 30 people with VHL to detect and quantify the level of VEGF production in VHL-associated lesions. The goal is to use this information to formulate a plan of monitoring and treatment with surgery and/or drug therapy to manage the health of these patients.

Rupal Bhatt, M.D., Ph.D., of Beth Israel Deaconess Hospital and the Dana Farber/Harvard Cancer Center in Boston, will study the “Role of the Interferon Gamma Pathway in Resistance to Antiangiogenic Therapy.”

People with VHL are sometimes being offered treatment with one of a class of drugs known as tyrosine kinase inhibitors (TKI’s). Eventually the tumors become resistant to treatment, and begin to grow again. Even though the drug is blocking the VEGF pathwaythe tumor seems to find a “detour” and begin to grow again. Bhatt’s team believe they have identified the “detour” being used. The project will confirm this hypothesis in mice, test it in VHL patients, and try to find a way to block this path so that these and newer TKI’s can be used effectively for longer periods of time.

James Handa, M.D., of the Wilmer Eye Institute of Johns Hopkins Medical Institute in Baltimore, will study the “Use of a novel genetic animal model to study the molecular pathogenesis of retinal hemangioblastomas in VHL disease.”

Retinal hemangioblastomas are the most common manifestation of VHL, occurring in the vast majority of VHL patients, often as the first sign of the disease.

It is essential to identify lesions early and control them. However, there are only limited treatment options available, which are not always successful. Better treatment options are needed. Dr. Handa and his colleagues have recently developed an animal model that reproduces the retinal hemangioblastomas observed in patients with VHL disease. This novel genetic model represents the first animal model for VHL retinal hemangioblastomas. In this research proposal they will use this animal model to identify new methods for the early diagnosis and treatment of VHL-associated retinal hemangioblastomas.

Not Just for Steven...

2009-05-15T18:54:00.008-04:00

Steven's lived a relatively uncomplicated two years...health wise...since his last brain surgery.

He's had a few more brain tumors show on scans. But they are still so small and so asymptomatic.

The brain stem tumor has continued to grow in it's dawdling slow way. The few symptoms that Steven's experienced from this tumor, namely odd yet irregular hiccups, gather nary a glance from neurosurgeons familiar with this disease. Surgery for that tumor has been mentioned, but in a very vague and futuristic manner.

His pancreas continues to function well despite the several cysts that reside there.

And Steven has a wonderful life...continuing his studies, playing soccer, living the college life, and being our sweet son.

Isn't it crazy that I continue to raise money and awareness for this rare disease when Steven's been relatively well?

It's not just for Steven...but for many more

Other young adults, not much older than my sweet boy, have had a horrible battle with von Hippel Lindau disease in recent months.

One young man, just a year or so older than Steven, is now struggling to learn to walk again. His studies have been on hold, his soccer playing days are over, and his life is lived out in rehab because a rather nasty hemangioblastoma took home inside his spinal cord.

It's not just for these young warriors...but for so many more.

Studies with VHL patients are producing chemotherapy drugs that are helping thousands of other cancer patients.

While not used with the VHL population yet, drugs like Sutent, Avastin, and Nexavar are being used to treat glioblastoma multiforme, advanced renal cell carcinoma, and metastatic cancers of the colon, lung, breat, ovary and liver.

These next generation of chemo therapies are being studied and produced because of a little crazy gene on the short arm of the third chromosome.

It's not just for so many others...it's also for Steven.

His abdominal scan from early May is now showing VHL involvement in his left kidney.

It's very small spot...all of 4 mm...and not really newsworthy, but it's there. And it's the beginning of a new phase in our sweet boy's diseased body.

This never ends.

We'll continue to raise funds and awareness forever, or at least until my sweet boy can go to a scan and be fairly confident that nothing will show.

The Words

2009-05-12T22:55:00.006-04:00

To describe our amazing breakfast!

Empowering!

It's so important for Steven to know

Amazing!

that we will help him every step of this journey.

Wonderful!

And when we need it, that help can come from friends

Inspiring!

who care enough eat breakfast with us!

These Hands

2009-05-10T21:05:00.005-04:00

These hands
drove 10,080 miles of carpools,
changed 7865 diapers,
washed 18,250 underpants and twice as many socks.

These hands are busy.

These hands
costumed many a pirate and princess,
crafted play-dough castles,
colored purple dinosaurs.

These hands create.

These hands
flipped pages in books,
guided pencils,
supplied the extra fingers for adding.

These hands teach.

These hands
wiped fevered brows,
washed skinned knees,
dried many tears.

These hands soothe.

These hands
held the soft dimples of a little hand,
straightened the ties of tuxes,
cradled many home made cards,

These hands cherish.

These hands are thankful.
These hands are blessed.

Happy Mother's Day
May, 2009

Pictures

2009-05-09T23:40:00.011-04:00

Words to follow...
a warm bubble bath,
an application of moisturizer,

a dose of extra-strength ibuprofen
a good book,
and a long nights sleep!

Just a Thought

2009-05-08T21:45:00.007-04:00

A fleeting but worthy thought danced through my busy mind this evening as I was
stacking and organizing,
folding and lining,
spiffing and buffing
at the fellowship hall.

What if everyone who watched the video for 4000+ hits also donated a dollar to our firstgiving account on the sidebar?

Wouldn't that be wonderful?

Tickets

2009-05-07T20:59:00.015-04:00

We have tickets.

And while we still have many, many pancake tickets left to raise money....

the tickets I'm most excited about this evening are two airplane tickets to California...

because Steven won the YouTube contest!

We're thrilled beyond thrilled, amazed beyond amazed, and stoked beyond stoked to go to the VHLFA yearly conference in California.

And we're humbled beyond humbled that we only sent out a few emails...and so many people all over the world took the reins by sending emails to friends, posting on Facebook, and putting the notice on their blogs.

Thanks you so very, very much!

And as for the video...

We aren't videographers.
We aren't script writers.
We aren't directors.
We aren't photographers.
We aren't professional soccer players.
We aren't artists.
We aren't painters.

We are just a family forced to believe that nothing is impossible.
Steven is just a sweet boy in search of a cure.

Keep watching!
I promise you'll be inspired!

Seatbelts

2009-05-05T22:52:00.011-04:00

My sweet boy's operative notes make me cry every time I read them.

It's not the mention of general anesthesia, intubation, or suboccipital midline incisions that make me sad.

I can read about prophylactic antibiotics, steroids, and irrigation with relative detachment.

Even the mention of the bur holes and the drill used to open my sweet boys skull and the documentation of sutures and titanium plates used to put him back together don't leave me overly emotional.

But I always have a very difficult time reading, thinking or talking about the seat belts...the seat belts that so very tightly strap him to the table.

Seat belts.

Isn't it odd that I lose my composure over seat belts?

But all of the tools and procedures that are mentioned in the operative report have big, fancy names that sound impressive and curative. The BrainLab navigation system, 4-0 Nurolin sutures, Midas Rex drill, and Kerrison rongeur all sound like instruments of healing.

And the use of seat belts sounds so barbaric.

The reality of Steven's life with von Hippel Lindau disease, however, is purely barbaric.

Because brain surgery, stripped of the fancy names, is brutal. Opening the skull with a drill and maneuvering a blade through brain matter is horrific at the most basic level.

And brain surgery is the only treatment for VHL.

My sweet boy needs a cure.

Bits and Pieces

2009-05-04T19:27:00.012-04:00

Housekeeping

1. A winner for the YouTube contest has not yet been announced...as far as I know!
Keep watching Steven!

2. If you live far away, please eat vicariously Saturday morning and consider giving a donation by using the "firstgiving" button on the side of the blog.

3. If you live locally and haven't yet bought tickets...

4. T-shirts are here!

All the "flippers" and "servers" can now be duly attired on Saturday morning!

5. This week while I am finishing preparations for the breakfast, I will also be planning and celebrating a Mother's Day Tea with my class, cooking dinner three out of five nights, watching David play in the regional high school baseball tournement, laundering ten loads of dirt and grime, working, and beginning preparations for year-end conferences and festivities.

I hope I don't forget to buy groceries!

Have I mentioned that I'm a bit frazzled at times?

6. Steven scanned abdominally today. He drank his gazillion mL of contrast successfully and tolerated the scan like a pro.

I'm not sure when or how we'll get results.

Hope

2009-05-03T19:40:00.004-04:00

If you flip over 600 pancakes,

scramble 100 cups of eggs,

brown 400 sausages,

and brew 200 cups of coffee,

can you make

a miracle?

Von Hippel Lindau Disease

2009-05-02T16:15:00.008-04:00

As we frantically search for balloons, ribbons, pancake batter, and other forms of revelry for the upcoming pancake breakfast, I'm relieved that I don't need to search for the perfect words today. A post from July 2007 still holds true.

Von Hippel Lindau Disease
The fact...the reality

The Facts

VHL is a genetic cancer syndrome. A person with VHL has a mutation in a very specific spot on the short arm of the third chormosome in every single cell of his body. This area of the chromosome is called the "VHL gene." The VHL gene, in it's intact form, is a tumor suppressing gene. When the VHL gene has gone awry, tumors form. The tumors associated with von Hippel Lindau disease occur in the brain and spinal cord, the retinas, the kidneys, the pancreas and the adrenal glands. Sometimes the tumors are benign, sometimes they are malignant.

The whole genetics behind this disease is really much more complicated than this, with mention of proteins, elonginsB and C and VEGF, and there are many resources out there that do much better job explaining tumor formation than I ever could.

Von Hippel Lindau is a life threatening genetic cancer illness without a cure.

Bottom line.

The Reality

The reality of this disease, for us, is that one day our lives changed...forever.

When Steven was just a baby
I never imagined that I would be holding a basin for him in an ICU room because brain surgery tends to make you vomit...a lot.

When Steven was just 1
I never thought that one day, at a young age, he would have a cancer diagnosis, and that the diagnosis would never leave.

When Steven was only 2
I didn't realize that doctors don't have answers for everything.

When Steven was 3
I didn't know how many stitches it took to sew up an incision in the back of a head.

When Steven was 4
I had never received a call from a doctor in the evening.

When Steven was only 5
I had never checked hourly for bleeding from the removal of a line in the femoral artery.

When Steven was 6
I never worried about Steven missing college because of a brainstem tumor.

When Steven was 7
I didn't know what an MRI sounded like.

When Steven was 8
I had never faced my child's mortality.

When Steven was 9
I didn't know that a post-surgical ICU patient had a distinct smell.

When Steven was 10
I had never contemplated the enormity of "forever".

When Steven was 11
I had never thought that I'd be kissing a teary teen as he was being wheeled away for brain surgery.

When Steven was 12
I had never seen how brave and courageous my son could be.

When Steven was 13
I had no idea how to accept help.

When Steven was 14
I had never been to a pediatric hospital.

When Steven was 15
I never dreamed that my child could blinded by tumors in his retinas.

When Steven turned 16
life was easy.

When Steve was 16 years and 2 months
life changed.

I learned about a rare disease. I learned of genetics and tumor growth. I heard that one day, at a young age, my son will be told that he has renal cell carcinoma, bilaterally. I learned all about hemangioblastomas, cysts, edema, and neurological symptoms. I learned of many other tumors that will afflict the body of this sweet boy. I started listening to the thunder of an MRI, too many times to count, and I began studying pictures of my son's brain. I spent long and lonely nights in ICU rooms, holding a basin and wondering how we ended up there, in both a pediatric and a full-service hospital. I watched as sutures were tugged and clipped from incisions at the back of my son's head. I learned that the odds for a long and healthy life are stacked against my oldest child.

But...

I also know now that this diagnosis can be a catalyst for us. It can change us in ways beyond our wildest imaginations. We can learn truths that that only the privileged know. We can accept the miracle of another day, and we know that life is made of moments. We have seen scars that can build unspeakable resolve. We've learned that the demons we face can only conquer us if we allow them. We know that bravory is our strength. We've realized that we can do things that we never dreamed possible. We know that sometimes wishes do come true and that people are tremendously kind. We see that we can only live in hope and that we have to live in faith. We know that life is what you make it. We are stronger than any gene.

We have a mission.

May 9th
2nd Annual "Cure Steven"
VHLFA Pancake Breakfast
First United Methodist Church of Seffner
(Fellowship Hall)
1310 S. Kingsway Rd.
Seffner, FL

8-11 a.m.

May

2009-05-01T21:41:00.002-04:00

May is VHL Awareness Month.

Just a warning.

You're about to become aware.

Very, very aware!

Beautifully Broken

2009-04-09T18:14:00.010-04:00

My sweet boy...

enjoying a night at the ballpark,
laughing at his brother's antics,
living the life of a college sophomore,
delighting in the moment,
and ignoring the monsters that grow within.

He is so beautifully broken

and so wonderfully made.

Just Askin'

2009-04-06T10:01:00.006-04:00

If you've been asked twice, even at the happiest place in the world, if you are your daughter's grandmother,

does that mean that your daughter, and her friends, look particularly youthful for their twelve years,

or that you are aging at an exponential rate and can be called "rather mature" for your forty-eight years?

Please don't answer that question!

(And I did the math...after the sweet grandmotherly attendant at Disney remarked about my three beautiful "grand-daughters" yestersday. I guess that in a less-than-perfect-world this side of the Mason-Dixon line, I could be their grandmother!)

Small Dose of Happiness

2009-03-30T17:13:00.006-04:00

From YouTube

** Christian **

** Charlie **

** Wintley **

** The Puppy Whisperer **

** Steven! **
Keeping watching...the contest continues until April 30th!

And mark Steven's video as your favorite!

I Need Help!

2009-03-27T15:17:00.009-04:00

Help!

I had a horrible dream last night...the sort of dream where I was an hour late to my own fundraiser!

I've talked to my therapist about this,
(and if you know me...then you know I'm my own therapist...and therapy typically happens when I'm driving in my car...when the booming silence gives me the space to think)
and my therapist told me that I need help!

Literally!

*I desperately want to pre-sell tickets to this event. While I believe that we have an amazing resource for purchasing the food, I'm very conscious of the money being spent. I want to plan well and waste nothing.

*I need to start the hunt again for inexpensive t-shirts. ("Cheap" being the key word in that sentence.)

*I also need to start the hunt for a clown/balloon connoisseur because, though the last man did indeed live up to his own inflated reputation, I've lost my contact name/number!

*And I desparately NEED raffle items. It's been frustrating, the lack of general enthusiasm from local businesses, and that's all I'll say!

And I think I will use a slightly creative way of giving back to the many who care. I believe that our sweet gifts will be raffled to all who pre-buy a ticket!
(But if you truly prefer selling those little $1 tickets...let me know and we can work both ways!)

*I may need a little help thinking that through and planning accordingly!

*I need to plan for out-of-town family and friends. I would love for all to be able to be part of the breakfast festivities. Realistically, however, I also need a way for all to be able to donate to the cure if travelling is not an option.

"Pancakes". version 2007...my inspiration!

Help!

We need more hits on YouTube!!!
I'm panicking for my sweet boy!!!

So, please, go to "YouTube", put "VHL Family Alliance" in the searchbox.
After the search completes, look for the video with a picture of Steven standing with a soccer ball.
Click on that video and watch it completely until the end!
Repeat once a day!

And please feel free to share this video with family and friends!

Help!

Thank you for caring so much about my sweet boy!