My mother died at the age of 27 from a very rare disease called VHL. VHL, or von Hippel-Lindau, is a cancer syndrome during which blood vessel tumors grow and continue to grow throughout various parts of the body where blood vessels are prevalent; important parts of the body such as the brain (cerebellum), spinal cord, kidney, pancreas, inner ear, adrenal glands and of course, the eyes.
Although VHL is inherited, it treats every individual differently, even within the same family. VHL tumors can be removed but they always grow back. That’s the “beauty” of VHL. Our kidney tumors grow into cancer once their growth increases past a specific size. Brain tumors and spinal cord tumors can be removed but we generally only opt for that if we experience problems that affect our quality of life. Otherwise, we generally prefer not to operate, and we must live with tumors and cancer.
In the 60’s and 70’s, when my mother was first diagnosed with VHL, there wasn’t much knowledge about the disease. And that meant that every time a brain tumor popped up, she had brain surgery. It was the many surgeries themselves that ultimately killed her. I was 6 years old when she died.
In my twenties, I was incorrectly diagnosed with polycystic kidney disease and didn’t think much of it until I had my first and only child in my thirties. It was a few years later when I was finally diagnosed with VHL.
Because my family had no real knowledge of VHL, I dubbed it a killer. And I was scared. I was scared for my daughter. I was scared for me. I was scared for the future. And because I was always such a worry wart, I knew I wouldn’t be able to handle the stress of this disease. Especially when my daughter was genetically diagnosed with VHL.
Eventually, I found an organization called the VHL Alliance. They taught me about my disease and how to take care of myself. They taught when to screen, how to screen and when to operate. They know where in the world I can go to find a facility who knows about VHL and doctors who are experienced in VHL.
The VHL Alliance wasn’t around when my mother was alive. She and my grandmother never got a chance to meet others with VHL. They never understood the disease nor did my mother’s doctors. They were utterly alone.
Because those with VHL, like me, have to deal with tumors and cancer their entire lives, while also dealing with family members with VHL (brothers, sisters, mothers, fathers, uncles, aunts, daughters, sons, grandmothers, grandfathers, cousins), it can be a lot to bear. It is a heavy, never-ending weight and there is no cure.
When a VHL patient gets a brain tumor or a kidney tumor or kidney cancer (which is what I have, along with other tumors), we carefully monitor them. We can’t always run to the operating table. We have to deal. We have to relax and learn to live with the tumors. We have to learn to live with the fear of possible effects of our tumors. Some of us lose our eyes or lose our other organs and some even end up paralyzed.
Because of the reality of my tumors and the effects they could have on me and my daughter, I have learned to relax — to not freak out every time I have a new growth. I have learned to enjoy and appreciate life more. I have learned to spend each day without worrying about every little (or big) tumor. Living life with tumors and cancer has taught me to live life with less stress. It has taught me to see the good that may come, instead of the bad. For none of us will never know what may come and worrying about it is a waste of precious time.
VHL has taught me to live a Very Happy Life.
We’ve partnered with Inspire, a company that builds and manages online support communities for patients and caregivers, on this patient-focused series. For the last five years, patients affected by serious and often rare diseases have shared their unique stories.
Jule Fobert, of Dallas, Tx, is the CEO/owner of WebModuleMedia.com and the mother of one. She is an active volunteer for VHL Alliance.
Photo courtesy of Jule Fobert