I’ve lived with von Hippel-Lindau (VHL), a rare (or rarely diagnosed) familial cancer, for 15 years. With VHL, abnormal blood vessels grow in little knots of capillaries called angiomas, or hemangioblastomas, which can cause problems. The disease is different for everybody. Mine has resulted in two brain surgeries, spinal surgery, two partial nephrectomies, and an eye laser treatment.

Everyone leads a complicated life, even without the variables of chronic illness. Those living with a multi-symptom disease, however, have times where we envy those with a cancer that is well researched. VHL has no middle, just a beginning and an end. Symptoms come in spurts. Sometimes they crop up after years of inactivity. Sometimes they appear several times within a year. People die when their bodies become overwhelmed by disparate tumors and problems associated with the disease.

Because VHL has so many varied symptoms, it is difficult to condense into a narrowly worded definition, and even harder to diagnose. Over the years, doctors in good faith have given me wrong information. The depth of medical knowledge on VHL is depressingly slim; even today, many doctors still have not heard of it. While I was suffering from my first brain tumor, my doctor insisted nothing was wrong. I told him I thought I had a brain tumor. I’ll never forget retching into a specimen cup while he told me "70 percent of his female patients had psychosomatic illnesses." I checked myself into the hospital days later and told a resident what was happening. He ordered a CT scan, which confirmed my suspicions. I didn’t feel vindicated. Just grateful. Sometimes half the battle is finding a doctor willing to listen.

I’ve survived episodes of illness, surgery, and recovery while remaining stubbornly fixed on the ideal of getting back some semblance of a normal life. Throughout the years, it has become up to me to redefine "normal." I’ve learned what I can about the disease, joined an information and support group, and interviewed dozens of doctors to form the right medical team. In the process, I’ve learned fundamental truths. I can stand up to doctors, technicians, and surgeons. When I take myself seriously, I gain more self-assurance. And, I am incapable of using a bedpan, even if it means waiting ten hours before going to the bathroom.

I’ve lost friends and a first marriage to the cracking-up-sorting-out-coming-to-terms-baggage that accompanies anyone who lives with chronic illness. Gone are the certainty of bearing a healthy child ... the ability to continue the athletic pursuits I love ... a level of independence and privacy ... at times, personal dignity and modesty ... and the privilege of what I call false positives -- such as taking for granted the fact that you will wake each day and your body will work effortlessly.

At times, I mourn the loss of self-assuredness that preceded this illness. It’s hard weathering things that most people normally do not confront until old age -- or, if they are lucky, never at all. But self-pity can be such a bore. After awhile, I force myself to enjoy life, even if I have to pretend a little. Then one day, I begin to re-appreciate all I have and what I can do. I find activities and hobbies that keep me active and engaged. I resolve feelings of meaninglessness and loss and move on. I become balanced and happy again.

Despite knowing this, last summer I lost my sanity for awhile. I had spinal surgery and it left me with unrelenting pain. After months with no end in sight, I went a little crazy. It surprised me. Being the veteran of five previous surgeries, I thought I had gone through just about everything. As time dragged by, I realized with a sinking heart I had not. I was unable to sleep more than an hour at a time before pain forced me out of bed. Unable to tolerate much of anything, I limped around the house all hours of the day and night until I grew so exhausted sleep overcame me.

When my disease had flared up in the Fall of 1997, I became distraught and frightened. The spinal surgery had been hanging over my head for years. The doctors had wanted to hold off as long as possible, for fear the surgery might leave me paralyzed. I was bewildered. With my husband sorting through the myriad tasks of making airline reservations, scheduling doctor visits, and working through the red tape of the insurance company, I went about my daily obligations and arranged my work schedule. In between, I tried to nurture my embattled body. Now I look back and see it was wrong to squeeze concern for myself in between work demands and the realities of life. Today, I know I should have stepped back, breathed deeply, and carved out more quiet time to absorb and confront the illness, and appreciate and nurture my body. Eventually, time, faith, physical therapy, and medicine sorted it out. But I had to re-realize past lessons learned. The luxury of perspective helped me remember.

To have hope you must find hope within -- often at the precise moment in your life that hope appears most fleeting. When I first learned of my chronic illness, the hope that sprang within was born of courage and determination; not just to survive, but to prevail. I have gained so much the scales have tipped in counter-balance. My marriage and relationships with family and friends are more authentic. I’ve walked through fire and am purified and humbled by the experience.

I know my will power and inner strength is ironclad when I must call upon it, and my spirituality is growing stronger. I am constantly learning how to appreciate, develop, and nurture this gift. Because this lesson was not learned easily, it is all the more precious. Finally, I vow that this disease will never define my life. But it does refine it.

Ending the Madness

Chunks of me are taken out
with Pac Man precision
several holes in my brain, my spine
nibbles to both kidneys
a black laser hole in my eye

Sometimes I feel my soul
leaching out of the openings
a precious resource leeching
from my very bones

rendering me tired and helpless
like an old clock winding down

When I am feeling stronger, however,
I think my spirit plugs up these holes
and the armor it grows
in my skin and bones supports me

I will combat this evil that grows
unwelcome and unwarranted tumors
and cysts, it won't defeat me

The trick is to end the madness
while continuing to generate love for the body
leaving bones, tissues, and organs intact.

-- AGL

This essay was prepared for a contest among cancer patients offered by the Thsepong project, a fundraising project for the Cancer Association of South Africa (CANSA), Pretoria, South Africa.

Thsepong means "place of hope" in South Sotho.

As printed in the VHL Family Forum 7:4, December 1999. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.

mystory