VHL and the Hawaii Family

Long before they became the subject of genetic research in the early 1980s, Rodney B’s Hawaii family would talk among themselves about why so many of them had cancer. In a sense, learning that it was a genetic disease helped to make some sense of it, and to give them ways of fighting back.

At the turn of the century (about 1900), their ancestor, Lazarus R., brought his young family to Hawaii after a hurricane devastated Puerto Rico. Lazarus died en route, but his widow and two little children ended up on Kauai. The little girl, Helen, had the flawed gene. She had three husbands and some 15 children. Her brother Frank, who also had VHL, had eight children who lived to adulthood.

Helen’s descendants, now scattered across the Islands and the Mainland, number some 260 or more members over six generations.

"I’ve always said, ‘Ah, the family curse,’" Says Roxane M., another family member. "But David T., another cousin, says, ‘It’s no curse, it’s a blessing.’ If it were not for VHL, I would never have met David. I would never have started a family newsletter. Now I want a family reunion."

Members of this extended family have shown extraordinary grace, and grown closer, as they fight VHL. They support one another, encourage each other to get tested for the gene, and grieve when the news is bad. Some worry, others try to forget.

"I talked to my brother and he’s just glad he doesn’t have it," says Rodney. "He told me once he’s afraid if he had it, he would be devastated. But you do what you have to do. When I find out others have problems, it bother me more than my own. I don’t want them to suffer."

Kidney cancer has been a particular problem in this family. Rodney has had eye and brain tumors, but kidney cancer has been his single biggest issue. In his twenties a series of large cancerous tumors led to removal of both kidneys. He had a transplant which failed after six years. At age 40, Rodney has dealt with years on dialysis and two hip replacements, complications from long-term use of steroids.

His is a very tough story of life with VHL. "There’s some days you cry. But I think God gave me grace in deal-ing with it," says Rodney, staring with blurred vision at the blaze of bouganvillea on his townhouse lanai.

His wife Vanessa has been a wonderful partner. They travel together with their one-year-old daughter, no small undertaking for someone on dialysis. But they have learned to organize around dialysis and life’s other challenges, medical and otherwise, with good humor and determination.

Roxane watched her father die at age 49, also of kidney cancer. "My Dad told my husband, ‘Don’t have kids,’" says Roxane. "We talked about it, but I decided I was going to have kids whether or not they might have VHL. You can die from other things too." Roxane knew that, like everyone in her family, she was at 50% risk of having VHL, and has gone every 3 years for the last two decades for thorough screening which includes an MRI brain scan, gynecological check, eye exam, and kidney ultrasound.

"What scares me is my children are at risk," Roxane said in an interview last year. "But you have to go on about life. You can’t worry about this thing. One of my aunts doesn’t have it, and she was just diagnosed with breast cancer. If it’s not one thing, it’s something else. You’ve got to remain positive."

Since the discovery of the gene in 1993 there is now a blood test to screen for VHL. A grant from Kapiolani Medical Center is making the test available free to family members. Already 34 people have been tested.

"It’s a cancer-susceptibility disease," explains Dr. Y. Edward (Ted) Hsia, the geneticist who has pushed national researchers to isolate the gene and find better medical treatments for people with VHL. "It’s a tumor-suppressor gene. When the gene is working properly, it prevents cancers from forming. When it doesn’t work right, it allows tumors to form." People with the flawed VHL gene get more tumors more often than people in the general population.

Annual screening could save a wealth of human suffering and medical costs, Hsia says. Yet medical insurers are sometimes loathe to pay for such screening. DNA testing can help to determine who is and is not at risk. After living for 35 years believing she was at risk for VHL, Roxane recently learned that she does not have the gene. "It’s a great relief in some ways, especially knowing now that my children are not at risk for VHL. But it’s also a tremendous adjustment. In a sense I have to redefine who I am. I feel almost guilty for not having the gene, almost as if I had lost my place in the family." Roxane’s feelings are quite normal, a reaction known as "survivor guilt," typical of unaffected children in a family united by a common problem.

In a little Hawaiian country town, David T. was Kauai’s first successful kidney transplant, which has lasted him nearly eighteen years. He receives full military disability, and serves as pastor to a tiny Missionary Baptist Church. David was 22 when doctors first diagnosed the VHL tumors in his kidney, eyes, and brain stem. Now his 17-year-old son has been diagnosed with VHL too.

"With my boy, we knew he had a 50-50 chance of having it. He grew up in this house. He was always aware of VHL.

"When we got the news he had these growths in his kidney and pancreas. Initially we were swept away. Just like me, he had no symptoms, so we had no warning. But he has a really good attitude.

"I told him, ‘You can’t let this thing take away from your quality of life. You can have a good life. We just have to manage the disease.’ And we are learning more every day about better management and better treatment."

Although some of the family members are bitter and angry about the disease that has taken some lives and left others with disabilities, both Rodney and David express optimism.

"Tomorrow isn’t guaranteed to any of us," says David. "You have to be wise and appreciative of today."

Rodney, who spends several hours three times a week on dialysis and keeps hoping for another transplant, has similar sentiments. "When people go through a lot, their mercy grows. When I see someone hurting, my heart goes out to them. When they’re having pain, I understand."

Thanks to Beverly Creamer, Honolulu Advertiser, for her assistance in the preparation of this article

As published in the VHL Family Forum, 4:2, June 1996. 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.

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