Polycythemia Vera (PV)


    “We have grandkids and great-grandkids who live in the area. I don’t want any of them to get sick because nothing has been done,”

    For Merle Wertman of Tamaqua, Pennsylvania, there never were any answers–only more questions. In 2003, he was diagnosed with polycythemia vera (PV), a rare progressive incurable blood cancer that affects the bone marrow. But Merle’s diagnosis is not rare in northeastern Pennsylvania. In fact, the tri-county area of Carbon, Luzerne and Schuylkill counties is the first region in the country to be recorded as a PV “cluster”. A diagnosis of PV puts a patient at a higher risk of stroke, heart attack, thrombosis, and in some cases, can progress to life-limiting myelofibrosis or leukemia. Studies have also suggested that exposure to toxic substances and high levels of radiation can increase a person’s likelihood of being diagnosed with an MPN1. The average general practitioner may only see a few cases of PV in his or her lifetime, making it a difficult disease to diagnose and treat.

    The PV cancer cluster in Northeastern Pennsylvania is home to three Superfund sites, abandoned strip mines, and a coal-fired power plant. Merle and his wife Linda have lived in the afflicted region of Tamaqua since 1969. In a recent interview, Merle shared that there are thirteen other cases of rare cancers on his small block, and seven residents who have since passed from cancer (not all MPNs) on the street. While he can’t prove it, he has a strong suspicion that his drinking water, which comes from the Still Creek Reservoir, has made himself and many others on his block sick.

    The higher rate of PV diagnoses in Northeastern, PA relative to the general population first caught the attention of internist Dr. Peter Baddick nearly fifteen years ago. At that time several residents from Ben Titus Road in Rush Township had been diagnosed with PV. In one case, a husband and wife from the same household were both diagnosed with it. Ben Titus Road runs parallel to the Still Creek Reservoir, and the reservoir is near a superfund site in McAdoo, where toxic waste and coal ash were buried in mine shafts after a mining company was discovered illegally burning and dumping toxic waste during the 1970s. High levels of cancer-causing Benzene were found at the site, along with dead wildlife.

    The number of patients in Northeastern PA with rare blood cancer was alarming enough for then-Senator Arlan Specter to spearhead a campaign more than a decade ago to secure federal funding for the study. Soon after, the Center for Disease Control (CDC) and the Agency for Toxic Substances and Disease Registry (ATSDR) conducted a federal study that encompassed Luzerne, Carbon and Schuylkill counties2. However, the only conclusion from the study was that the tri-county region had a four times higher rate of polycythemia vera than the national average. During the study, investigators told citizens it would be impossible to pin a cause on the PV.

    Ann Brazeau, CEO of MPN Advocacy and Education International, spent a great deal of time engaging the CDC and local citizens in the tri-county area gathering information on the PV cluster while at the MPN Research Foundation. Ms. Brazeau and Dr. Ronald Hoffman of Mount Sinai traveled to the area for a town hall meeting that ended in frustration.

    “I was thrilled when Senator Specter crossed the aisle to the Dems and was able to convince the Obama administration to appropriate $5M per year to investigate this obvious cluster,” Ms. Brazeau says. “Diagnostic programs were implemented in the area that confirmed the PV cluster existed, but to my knowledge, nothing further was done. We know that certain chemicals were dumped into empty mine shafts and those were designated Superfund sites. Some of the chemicals were also used in Agent Orange.”

    For Merle Wertman, the questions are many and the answers are few. He wants to know who is responsible for this environmental disaster. Another concern from the community is whether a cluster of PV persists.

    “We have grandkids and great-grandkids who live in the area. I don’t want any of them to get sick because nothing has been done,” he says. For the affected residents of the tri-county area, there is no cure for PV or closure from the investigation, only frustration and mounting anger.

    Merle will continue with his monthly phlebotomies to prevent his red blood cells from becoming too thick. He will live with the physical limitations of PV: extreme fatigue and migraines. He will miss out on some walks to the park with his grand and great-grandchildren because he’s too tired. And he’ll continue to wonder why nothing has changed in this town, where secrets are buried like toxic waste.

    Contributed by Jennifer Acker

    These represent first person accounts of real people living with Essential Thrombocythemia, Polycythemia Vera and Myelofibrosis. It does not represent the views or opinions of anyone associated with the MPN Research Foundation. Please consult your doctor before taking any action to manage your health.

    Meet LARRY


    Meet BETTY
    SEE ALL Polycythemia Vera (PV)