Von Hippel Lindau Disease

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.