She was told in 1964 that she was one of a handful of people ever diagnosed with the rare disease von Hippel-Lindau. Celebrating with family and friends, in July, her 70th birthday, Neatha D., has beaten the odds and lived twice the expected age predicted in the 1960’s for persons with this hereditary cancer.

In 1964, Neatha was determined to have VHL when a tumor was discovered in her left eye on the optic nerve. She had gone to an optometrist who sent her to an ophthalmologist believing she had Histoplastamosos. The ophthalmologist made the diagnosis of VHL. Neatha underwent laser surgery to restore her sight. She was a "guinea pig" for the ophthalmology students at Indiana University. Neatha confesses each visit to the Medical Center was longer than usual because several students needed to do examinations and learn about the eye manifestations of this little known disease.

Neatha feels very fortunate to have had an early diagnosis and excellent medical care which has improved her quality and length of life. She had surgery in 1975 to remove a hemangioblastoma that had caused severe headaches and loss of balance. She says, "that first brain surgery was extensive, involving a large incision from the back of her right ear to the midline of the back of the skull down to the middle of the back of the neck." She spent several days in recovery and it was months before the incision had healed.

Since 1975, Neatha has had two more brain surgeries, the last one in 1992. Annual MRI’s have helped to diagnose and quickly treat the brain tumors. The last surgery was less invasive and Neatha healed within a few weeks. She says, "Medical technology and treatment has "come a long way, baby" since her diagnosis 36 years ago.

Neatha believes her father may have been the carrier of the mutated gene for VHL. She has no brothers or sisters and no genetic testing has been done. Her father had died in his thirties from a growth in his abdomen, but no autopsy was ever performed. Neatha has two adopted children and she has been widowed for 24 years. She retired in 1992 at the age of 62, and she continues to do volunteer work. She enjoys living independently and her favorite hobby is bridge. Neatha says, "I do the crossword puzzle every day to keep my mind active."

Neatha is one of two unrelated persons in her church congregation with VHL. She is pleased to have found in the VHL Family Alliance a global community of people with VHL.

Neatha is a wonderful example of the results of early diagnosis, testing and treatment. She continues to live a full life enjoying her two children, four grandchildren and one great grandchild. Neatha is a testimony to all those who once believed that the diagnosis of VHL was a prognosis for a short life. And she is not alone. We have many members in their 60’s and 70’s, and half a dozen in their 80’s. With careful monitoring, early diagnosis and appropriate treatment, people with VHL are living longer, healthier lives than ever before.

As printed in the VHL Family Forum 8:4, December 2000. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org.

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