Polycythemia vera probe gets underway
Schuylkill, Luzerne, Carbon patients urged to participate
This week, the Pennsylvania Department of Health and the Atlanta-based U.S. Agency for Toxic Substances and Disease Registry will begin interviewing residents of Schuylkill, Luzerne and Carbon counties who have been diagnosed with polycythemia vera, a rare blood malignancy that a recent PADOH study found to occur locally at an unusually high rate. However, because the agencies face restrictions on calling patients, they're asking the patients to call them.
"We're hoping people who'd like to participate will contact us directly," says Vince Seaman, an ATSDR toxicologist and one of the investigators.
The information gathered will be used to better understand the area's PV cases and identify possible environmental exposures that may have triggered the disease, in which the body produces too many red blood cells. Because investigators will interview only those PV patients whose cases have been reported to the Pennsylvania Cancer Registry, they are asking patients who want to take part in the probe to contact their doctor and ensure their case has been reported.
Investigators have sent letters announcing the investigation to the 82 residents of the three-county area whose PV cases have already been reported to the registry. Because of state regulations, investigators can't contact patients verbally -- either in person or by phone -- until they have their consent. An official consent form is enclosed with the letter.
If the investigators get no response from the patients after the first letter, they then mail a second one. If they still get no response at that point, only then may they call the patients to ensure they got the letters.
"It's a long, drawn-out process," says Seaman. To circumvent it, he asks local residents who've been diagnosed with PV or who think they might have the disease to contact him directly. He can be reached by phone at 530-902-8249 or by e-mail at VSeaman@cdc.gov.
According to the investigation protocol, the researchers want to ensure that all diagnosed cases of malignant PV in the study area are reported to the state cancer registry. They will also ask participants to answer a questionnaire in order to find any common environmental exposures that might have triggered the disease. The questionnaire will be administered in person by the investigators at participants' homes and takes about 30 to 45 minutes to complete, Seaman says.
In addition, the investigators will confirm patients' PV diagnosis by testing for the presence of the JAK2 V617F mutation in the bone marrow stem cells. A growing body of research has shown that the JAK2 mutation is present in almost all cases of malignant PV.
While the JAK2 test normally costs about $700, it will be provided for free to the study participants. The lab analysis will be done by the University of Illinois Cancer Center under the direction of blood cancer specialist Dr. Ronald Hoffman. A past president of the American Society of Hematology, Hoffman earlier this year received a $1.9 million grant from the state of Illinois to study stem cells in treating blood disorders.
The blood sample needed for the JAK2 test may be drawn at a clinic or, if the participant is homebound or unable to travel, a community health nurse will visit the home to collect the sample. However, patients are not required to take the test to participate in the investigation, Seaman notes.
The ATSDR investigators will be in Pennsylvania for about a month, and the entire investigation is expected to take three to six months. Officials are hoping for the cooperation of local PV patients.
"With something this rare, every single case is worth a lot," Seaman says. "Everyone who participates makes this investigation stronger."
Public concern over PV rates in the Hometown area has been building for several years now. In the local health study released last December, PADOH found that the incidence of PV was four times higher in Luzerne County and three times higher in Schuylkill County compared to the state, whose rate is already elevated compared to the nation's. The difference was especially dramatic among males, whose PV rates were six times higher than the state's in Luzerne County and five times higher in Schuylkill County.
Dr. Pete Baddick, a Hometown area native and general internist who practices locally, has suggested that the rates may be even higher than reported by the state registry. He has also reported observing what seems to be an unusual number of other blood cancers, including leukemia and multiple myeloma.
PADOH and ATSDR began investigating local health concerns after Baddick and other area environmental health advocates -- including Dr. Dante Picciano of the Army for a Clean Environment and Frank Waksmunski of Carbon County Groundwater Guardians -- publicly raised concerns about a cluster of PV cases along Ben Titus Road in the rural Still Creek community north of Hometown.
As many as seven PV cases have been reported anecdotally along a 1.5 mile stretch of the road. The community lies directly downhill from two big sources of pollution: the McAdoo Associates Superfund site and the Northeastern Power Co.'s waste-coal-fired power plant. (A rough analysis I conducted earlier this year suggests a possible association between PV rates and waste-coal-burning power plants.) Still Creek also lies downwind of Air Products' specialty gas manufacturing facility, another a major local polluter.
Among the causes for public concern is the fact that Still Creek lies along the drinking water reservoir that serves Hometown and the nearby borough of Tamaqua. One test found that lead levels in the untreated water were almost five times the allowable levels, though subsequent testing by the Tamaqua Water Authority reportedly found no contamination. In 2004, the most recent year for which data is available, the NEPCO power plant reported releasing to the air 100 pounds of lead, along with tens of thousands of pounds of other highly toxic substances including hydrochloric acid, hydrogen fluoride, barium, chromium, manganese and mercury.
Burning coal, especially waste coal, also releases small quantities of radiation, which at least one study has associated with PV. In addition, the National Association of Radiation Survivors -- an organization representing U.S citizens exposed to ionizing radiation from the development, production, testing, use or storage of nuclear weapons and waste -- lists PV among the health effects associated with radiation exposure.
Other studies have pointed to possible links between PV and exposure to benzene and solvents -- both contaminants of concern at the McAdoo Associates Superfund site.
Labels: polycythemia vera