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Tracking a rare cancer

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Tuesday, July 28, 2009

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Between 2001 and 2005, 97 cases of the rare blood disease polycythemia vera were reported to the state cancer registry in Luzerne, Schuylkill and Carbon counties.

The higher-than-usual incidence of this rare disease has prompted public health officials from the Agency for Toxic Substances and Disease Registry and the Pennsylvania Department of Health to screen for the disease in these counties. These screenings will take place at three locations in early August.

Many people have not heard of PV, as it only affects one in 100,000 people and it is not a typical cancer. The patient’s bone marrow does not produce malignant cells; it produces too many healthy red blood cells. It is often referred to as “thick blood,” leading patients to suffer from blood clots, strokes and heart attacks.

According to Dr. Bruce Tierney from ATSDR, the symptoms of PV are vague and non specific, such as headache, fatigue, dizziness, small strokes and bleeding. The most effective treatment for the disease is a therapeutic phlebotomy to remove the patient’s excess blood cells.

The PADOH began to look into the unusual number of PV cases due to community outcry and the close proximity of the “cluster” of cases to the McAdoo Associates Superfund site. Between 1978 and 1979, McAdoo Associates stored toxic wastes including paint sludge, waste oils, pesticides and other suspected carcinogens. Department of Environmental Protection officials shut down the facility in 1979 and it was placed on the federal Superfund list. Residents are looking for answers as to whether the toxic substances stored there were causing the illnesses, fearing that the chemicals had leached into the water supply.

PV has no known cause. A few studies published years ago reported that PV could possibly be caused by exposure to chemicals or radiation. In 2004 it was determined that a gene mutation called JAK2 was found in most PV patients. This mutation is not inherited, but an acquired mutation, according to Vince Seaman, an epidemiologist with ATSDR. This discovery helped document the cases of PV, but still did not lead to a cause.

In October 2006 PADOH, with the support of Sen. Arlen Specter, requested the assistance of ATSDR to investigate the three-county area. Through this study ATSDR was able to determine that of the 97 patients thought to have PV only 33 cases could be confirmed using evidence of the JAK2 mutation. Without any known cause, ATSDR was unable to determine if a link existed between the area’s environmental factors and the high incidence of PV.

“There is no evidence that anything at that site causes this, mainly because no one knows how people get it,” says Seaman. What is known is that “something external” happens that causes the mutation, but this could be due to post-viral infection, radiation, radon or other environmental causes, added Seaman.

Dr. Peter Baddick, an internist who practices in Schuykill County, has been very critical of the DEP, ATSDR and their findings. He felt that other cancers related to PV should be looked at as well; he recognized that an abnormally high incidence of cancer in general exists in this area. He also has raised concerns that the environmental studies DEP has done have not gone far enough; he suggested water samples from the underground mine pools should have been studied further, before the Superfund site was deemed safe. Dr. Baddick would like to see ATSDR test the patients suffering from PV and look for a common denominator, but he applauds the current effort to do public testing.

The testing is part of $5.5 million in federal funding secured by Sen. Specter to study the cancer cluster in the tri-county area. “I am pleased to see the studies under way to better understand the basic biology and causes of polycythemia vera. The community is entitled to the best answers science can give them,” says Specter. He has long been an advocate on behalf of the local communities to investigate this problem.

Dr. Paul Roda, a medical oncologist from Hazleton, has been involved in the studies for several years. He is urging residents in the tri-county area to participate in the screening. The incidence of PV in this area is five times the expected rate and never before have so many cases of PV been traced to one region.

It’s very important to get as many samples as possible from people who lived near the Superfund site as well as those who live a distance away so they can be compared, says Dr. Roda.

Some evidence has linked the PV to environmental factors, but it has not been conclusive. The information learned from these tests will benefit the community by providing more data to identify a cause, as well as provide early diagnosis of PV in patients. This could lessen the chance of complications from the disease, concluded Roda.

According to ATSDR, a small blood sample will be collected. This sample will be tested for the JAK2 genetic marker. There is no charge for the testing and ATSDR will mail you the results in 4-6 weeks.

The public testing will take place Aug. 3-6 and Aug. 10-13 from 9 a.m. to noon and from 1-5 p.m. at Hazleton General Hospital, St. Jerome’s Catholic Church in Tamaqua and the Schuylkill Mall.

You must have lived in Schuylkill, Carbon or Luzerne counties for at least the past year to be eligible for testing.

You can make an appointment by calling 1-877-525-4860.

How to get tested

Testing for polycythemia vera will take place from 9 a.m. to noon and 1 p.m. to 5 p.m. Aug. 3-6 and 10-13. Call 1-877-525-4860 to make an appointment.

Hazleton General Hospital

O&E Building - 1st floor, 700 E. Broad St., Hazleton

St Jerome’s Catholic Church

School Gymnasium, 250 W. Broad St., Tamaqua

Schuylkill Mall

Community Meeting Room, Route 61 and I-81, Frackville

© Copyright 2009 The Times Leader. All Rights Reserved.

Author: By Eileen Cipriani
Source: Times Leader
1.0 from 5 votes
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