|
|
 |
The legendary McCoys - Response from Joyce Graff
In less than a week following Marilynn Marchione's AP story (link below) about the connection between the McCoy family and VHL, we have been contacted by nearly 100 members of McCoy lineage from all across the United States.
We are very glad that new McCoy family members are reaching out to the VHL Family Alliance and accessing this life-saving information. VHL is a complex medical disorder, often hard to diagnose. The people contacting us have been misdiagnosed with everything from carpal tunnel to Cushing's syndrome. We encourage anyone with symptoms that might imply VHL to contact us at 1-800-767-4845.
What is depicted in this article is the experience of one family. Let me add a bit to the article to give a broader view. VHL is different in each person, and varies considerably from one family to another. VHL occurs in one person in every 32,000, worldwide, in every ethnic group. Twenty percent of the people who have VHL are the first person in their family ever to have VHL. Fewer than 20% of people with VHL experience a pheo.
While the article associated VHL with "rage" as a factor in the Hatfield-McCoy feud, VHL is NOT a rage-causing condition. It is important for everyone to understand that a sudden change in behavior, or any unprovoked outbursts, might in fact be a symptom of an underlying medical issue. Many medical conditions on their own can cause sudden changes in behavior -- blood pressure, diabetes, brain tumor, stress and pheochromocytoma ("pheo") -- are just a few examples. Families who know they may be at risk for a pheo, should pay special attention. But it is also important to understand that rage is not the usual reaction to this type of tumor.
VHL is a complex medical condition that presents a series of challenges, but most people living with VHL are doing so with humor and grace and carrying on their daily lives like everyone else. As one leading VHL researcher said to me, "People with VHL are bank managers, financial analysts, active duty members of the military, lawyers, business people, physicians -- people from all walks of life who are affected by VHL -- brave, accomplished people functioning at the highest levels of society."
VHL does not cause rage, or even hormonal issues. VHL is a disease that may lead to the development of one of six different kinds of tumors, only one of which causes hormonal issues. A "pheo", a tumor of the adrenal gland, can cause surges of adrenaline -- that fight-or-flight response. It gives you extra speed and strength in an emergency. But having it injected into your body at random intervals can give one a feeling like a panic attack or palpitations. Only rarely is it interpreted as rage. Pheos occur in the general population, not just in people with VHL. They may be inherited. A flaw in any one of six different genes may lead to a pheo. VHL is the leading hereditary cause of pheos, but not the only one. 76% of pheos are sporadic, 24% are hereditary. Only two-fifths of the hereditary pheos are related to VHL.
Might a pheo have been a factor in the feud? Possibly. But don't forget that the Hatfields do not have pheos and they participated just as wholeheartedly in the feuding. There's a very strong component of culture and environment operating here in addition to the undiagnosed medical issue.
The article implied that having VHL might be a cause for bad behavior. Having a medical problem is NOT an excuse for bad behavior. Everyone occasionally has bad days, or feelings of rage. Fortunately, few of us act on them. We are all still responsible for our actions.
If you have unfounded feelings of anxiety, rage, or palpitations, or if you have heavy sweating, or uncontrolled high blood pressure, then the best test to determine whether these symptoms might be caused by a pheo is a blood test called "plasma free metanephrines." This test is the most specific for the products of a pheo, and is the most accurate in determining the presence of a pheo. It is not done in every hospital lab, but your hospital can send it for analysis to the laboratory services at the Mayo Clinic.
Untreated, pheos are very dangerous. They can cause heart disease or stroke. Having an accident, a baby, or any surgery or dental procedure can be life-threatening. But once the pheo has been removed, all those dangers go away.
The VHL community is excited to see the wide range of press coverage the story gained, as it brought this rare disease into newspapers and onto televisions across the country. However, we hope that VHL does not gain attention as a "rage disorder." In fact VHL is cited in numerous scientific articles as one of the keys to understanding how cancer works for everyone. Research on the VHL gene, with the help and participation of hundreds of people with VHL, has already led to significant advances in the treatment of kidney cancer for all.
Please take good care of your own health, and the health and happiness of those around you, by testing and taking care of yourself.
Links:
The DNA Testing lab at the Children's Hospital of Philadelphia has worked with this family for decades and has the reference markers to test for the McCoy mutation, or indeed any other alteration of the VHL gene, whether or not related to the McCoys. Click here for information Abstract and Full text of Dr. Atuk's medical article:Atuk NO, Stolle C, Owen JA Jr, Vance, ML, "Pheochromocytoma in von Hippel-Lindau disease: clinical presentation and mutation analysis in a large, multigenerational kindred." Clin Endocrinol Metab. 1998 Jan;83(1):117-20. Erratum in: J Clin Endorcinol Metab 1998 Apr;83(4):1150., Pubmed ID 9435426
Additional resources:Some Blog responses to the article:
|
| Contact Us | Copyright 1993-2008 |