I assume how you feel about this disease depends upon your perspective of where you are with it right now. In this newsletter I've read about many courageous people who are battling various and sundry ailments and medical problems, large and small, with positive attitudes that buck them up and help keep them fighting. But while you're in the middle of a prolonged battle, it's hard to keep your spirit and your energy up.

Until a year and a half ago, I didn't know I had VHL. I had a brain tumor removed in 1984, when I was 25, but after a year of being misdiagnosed, the actual surgery went smoothly enough. I have since learned - by my husband reading my old records - that my neurosurgeon suspected I had VHL and was monitoring other tumors and cysts in my brain, kidneys, and spine, but he didn't tell me about it. when I did question a pathology report that I read I was told not to worry - that if something ever did come up - and it wasn't likely - he'd make sure that it was taken care of before it caused me discomfort. I was on my own and still basically young and care-free, so I accepted that. For nine years I remained disease-free, and I marvel at the joy of that now. I seldom thought about illness, aside from dutifully getting a yearly MRI, which I considered a necessary chore.

In fact, I was beginning to lag behind on getting an MRI every year, and it had been a year and a half since receiving my last scan when my world fell apart. The past year and a half has been the year from hell. No sooner have I barely recovered from major or minor surgery, than I have needed surgery for something else. In the interim there have been more MRIs and tests. All this has been a real eye-opener, and I feel sadder and wiser for it. Although I know now that my life will never be normal, and I'll be living with an illness for the rest of my days, knowledge is power. I know that I'm a fighter, and now I know for certain that I'm a survivor. Still, this constant coping with medical issues has worn me down, not to mention depleting our finances to an alarming level. And does one ever get over the underlying subconscious feeling of being frightened and vulnerable?

I had just returned from a vacation in February, 1993, when I experienced those old familiar feelings that I recognized from my first tumor - loss of balance, coordination, general fatigue, handwriting problems, dizziness, and the like. Unfortunately, my husband and I had recently moved to my home state of Arkansas, and no one else here in the medical profession is very knowledgeable or experienced in VHL . . . so by the time I went back to my neurosurgeon at Georgetown University Hospital in Washington, D.C., I was in pretty bad shape, having seizures, and unable to walk. After an intensive operation and lots of physical and occupational therapy, I began relearning to walk and write over the summer.

If I can't exactly dance or race-walk anymore, I have figured out other things I can do for exercise and pleasure. And I was lucky; it didn't affect my job - I'm still an editor, and my company has been very supportive throughout my absences for surgeries and recovery. My job allows me to do a lot of work from home anyway, which is fortunate. As soon as I was strong enough, I went to the Cleveland Clinic in the fall for much-needed partial nephrectomy of my left kidney. Immediately after surgery I was told that I needed a partial nephrectomy of the right kidney, too. When I went back to have this operation after the holidays, my left kidney was discovered to be blocked. Thus began six months of minor operations and shunts and tests, and the possibility of having it removed altogether. We were on a first-name basis with everyone at the Cleveland Clinic and adjoining hotel whether we wanted to be or not! A last-ditch effort this spring by my wise and valiant surgeon saved me from another surgery and removal of the kidney.

It is now months later and I've had four more MRIs because my spinal tumors are acting up. I'm going back and forth to doctors in Texas and Maryland. All these appointments and check-ups, with the exception of the MRIs and tests for my eyes, must be done out of state, since the local doctors aren't confident in treating me and I'm not willing to be a guinea pig for educational purposes when it comes to surgery. We're building a strong medical team out of state, and I have several local doctors who are go-betweens and pinch-hitters when necessary. After a few tries, I'm finally lucky enough to find a family practitioner willing to learn about VHL, and help me keep on track for various tests while monitoring the ever-growing file. But my husband still spends hours on the phone and pores over the latest information he can find. I read it all too, but not in the detail that he does.

So life goes on, as you know. I intellectualize that I have been lucky because the outcome of these surgeries has been favorable. But I resent being told how "lucky" I am, unless this statement comes from another VHL warrior or someone who is dealing with or has dealt with a serious illness.

In Chinese calligraphy the character "ji" means both crisis and opportunity. I view having VHL this way. For all the negatives that this disease provides, it also grounds me to the reality of what is important in my life. It emphasizes the importance of the pure value of living each moment in acute awareness and appreciation for what I do have and can accomplish. And like so many others dealing with an illness, I am grateful for the loving support of family and friends.

As published in the VHL Family Forum, 3:1, March 1995. For permission to reprint, please contact the VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.

mystory