Hope - Strength - Community

What does this Alliance do for families living with VHL? It’s probably easiest if we tell you a story -- the story of Troy and Alison and their children Chloe and Caleb. For Troy and Alison, their challenge each day is to keep their own and their children’s lives happy and normal.

VHL has had a profound impact on Chloe and Caleb. Caleb was diagnosed at the tender age of four with three heochromocytomas (pheos) on his aorta. He recently had two more surgeries to remove pheos and is now dealing with a sixth. Chloe had a pheo removed when she was eight, and a second one this fall.

Pheos not only occur in VHL, they also occur in the general population and in people with any of four other genetic flaws. VHL accounts for only 10% of pheos. But because we know that people with VHL are at risk for pheos, we are less often surprised by them. “Pheochromocytoma is still the most treacherous, deceptive tumor on the planet,” says Dr. William Manger of New York University. “Missing the diagnosis almost invariably results in devastating cardiovascular complications or death.” More than half of pheos are not found until after death. The autopsy of President Eisenhower revealed that he had a pheo, provoking his heart condition.

The Cancer Research Fund / VHL Alliance funds research into pheochromocytoma, and provides support for individuals and families struggling to get a diagnosis and find appropriate management for a pheo. Alison is an active participant in our Online Support Group, giving freely to others the support she has been glad to receive. Few doctors have much experience with pheos. She is often asked to make decisions that she feels totally unprepared to make. It helps so much to hear the experiences of others who have been down the same road.

In addition to the pheos, Chloe has also developed several tumors in both of her eyes. Retinal angiomas are fairly common in people with VHL. Unfortunately, her tumors are in bad locations, making them particularly hard to treat without damaging vision. Chloe, like so many others, is literally waiting on the leading edge of medical research, experimenting with new treatment methods in hopes of preserving her vision. Again, sharing thoughts and experiences with experts and other patients has been invaluable.

Despite their battles with this disease, Chloe and Caleb are very normal, happy children. Caleb loves to go swimming, fishing, and just hang out with his buddies. Chloe is a competitive cheerleader with amazing tumbling skills, and thrives in the usual teenage social atmosphere.

Their parents are strong advocates for early DNA testing, as this probably saved the life of each of their children. Knowing early that their children were at risk for VHL, and knowing that Troy had had two pheos and retinal lesions in his youth, they began preventive screening early and were on the lookout for symptoms.

Most importantly, the family relies on their strong faith in God, knowing that He will get them through this hurdle in their lives, and thriving on the joy that He provides despite these challenges.

You can help too, by helping to fund research. Research on the VHL gene is particularly important because of the critical role the gene plays in controlling tumor growth in many cancers. Please help us find a cure for VHL, and ways to halt tumor growth for everyone.

As printed in the VHL Family Forum 15:4, November 2007. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.