Jerusalem Post online

It's All Relative: A lifesaver

Schelly Talalay Dardashti

July 1, 2004

No longer mere lists of ancient ancestors, genealogy now saves lives IF only," muses researcher Stanley Diamond of Montreal, visiting Israel for the 24th International Conference on Jewish Genealogy, one of some 600 people attending the week-long event beginning Sunday at Jerusalem's Renaissance Hotel.

"If only I had asked my grandmother the questions I have today," he says, "I could have had the answers in a few minutes."

In Stan's case, however, the answers could mean life or death.

On Wednesday, he will present "Combining Genealogical and Family Trait Genetic Research," providing insights on reconstructing family trees for genetic or family health reasons. For all conference registration information, www.ortra.org/jgen2004.

WHAT began as a quest to inform relatives about a rare genetic problem, beta thalassemia, documented in only a handful of Ashkenazi families, led to the Internet-accessible 2.5-million unique records of the non-profit Jewish Records Indexing-Poland, www.jri-poland.org. Experts on this database will offer programs on Tuesday.

Stan's main goal is to find other carriers of this specific mutation carried by his family, from Ostrow Mazowiecka (Ostrova) in the old Lomza Gubernia, Poland, and that of a Jerusalem family originally from Bobruisk, Belarus. He wanted to find the earliest carriers of this mutation in both families and to alert unsuspecting Ashkenazi carriers.

HIS outreach has turned up six other Diaspora Ashkenazi families with the trait, although not with his family's unique mutation. Some have already launched programs to alert extended families about potential danger to future generations. The families were located through JewishGen, www.jewishgen.org, secular/Jewish press coverage, newsletters and journals of worldwide Jewish genealogical societies.

On-site research in Poland and the cooperation of the Polish State Archives and key Civil Records Offices enabled Stan to document 50 more families who may be at risk.

The 19th century Lomza Gubernia and Bobruisk families who may be carriers of this rare mutation are listed at http://www.diamondgen.com/, click on "Search Surnames List." The earliest known carrier has been identified as Herzk Tsvi Widelec, born 1784 in Ostrow Mazowiecka.

"With descendants of so many families to be traced and contacted," says Stan, "my task, already immense, has now become virtually unmanageable. I need help from my fellow genealogists, their friends, relatives and physicians - anyone who might have knowledge of a carrier."

THE beta thalassemia genetic trait is not usually found among Ashkenazim unless doctors are looking for it. In Stan's family, most carriers learned about it late in life, after having grandchildren.

There is a one in four chance that the offspring of two carriers will inherit thalassemia disease (thalassemia major, or Cooley's anemia), which until recently was fatal by early adulthood. It remains a deadly disease.

Although well-documented in Greek, Italian, Asian and Sephardi families, it is almost unknown in Ashkenazim. Here in Israel, where there is mass pre-natal screening for the mutation, fewer than 10 Ashkenazi carrier families have been identified.

TEN years ago, Dr. Ariella Oppenheim of Hebrew University, Jerusalem and Dr. Charles Scriver of Montreal independently discovered that Stan's family and a Jerusalem family of Belarus origin carried the newly identified mutation.

Dr. Oppenheim says that Stan's work "serves as a paradigm for the link between genealogy research and the study of the evolution and spread of genetic diseases."

McGill University-Montreal Children's Hospital Medical Research Institute and Hebrew University-Hadassah Hospital, Department of Hematology, Jerusalem conducted joint research. A paper on the subject appeared in the scientific journal Human Mutation (9:86-87; January 1997), "Probable Identity by Descent and Discovery of Familial Relationships by Means of a Rare Beta-Thalassemia Haplotype."

PATIENTS require frequent blood transfusions to keep up the blood s oxygenating ability. Red cells are destroyed, causing large amounts of iron to be deposited in the body's organs, leading to their breakdown. There is no cure and until recent years most victims die in early adulthood. In its less serious form, patients suffer only mild anemia. And, according to the Children s Hospital Boston website (www.childrenshospital.org) the disease is often incorrectly diagnosed, with patients prescribed iron supplements in the mistaken belief that the anemia is the iron-deficient type. That's what happened to Stan when he was young.

With growing intermarriage between Jews and non-Jews, as well as increased Ashkenazi-Sefardi marriages, there is an increasing risk of the devastating disease occurring in an unaware Ashkenazi population.

Of Stan's children, one daughter is a carrier. Before she marries, the prospective bridegroom will undergo a blood test for thalassemia, and if he is of Sephardi origin, the risk is significantly higher.

To find out if you are a carrier, ask your doctor about your MCV level, normally recorded in a standard blood test. If it is less than 78, you should have an evaluation of your hemoglobin A2.
IN May 1994, Jerusalem Post health columnist Judy Siegel-Itzkovich wrote the first article about the project, while Stan was attending the Fourth International Conference on Jewish Genealogy, also held in Jerusalem that year, and where he launched the project requiring the help of genealogists, the press and the international medical and scientific community. Meeting the conference keynote speaker Prof. Jerzy Skrowronek, director-general of the Polish National Archives, was a turning point for both Stan and Jewish genealogists tracing their roots in Poland.
SAVING LIVES

ANOTHER way in which JRI-Poland's records and the organization's connections in Poland cooperate to help save lives is through enabling matches for bone marrow transplants.

The US-based Gift of Life Bone Marrow Foundation (www.giftoflife.org) began as a grass-roots organization focused on finding a donor for one person. Today, it is an international bone marrow registry, recruiting donors primarily from the Ashkenazi Jewish population.

It commends JRI-Poland's efforts to catalogue the genealogy of Jewish families of Polish descent and its experience confirms the importance of having genealogical information available to patients around the world, not simply as an academic exercise, but to answer medical questions and potentially save lives.

Gift of Life's mission is to assist patients suffering from diseases best treated by a matching stem cell donor. The best source is from related donors who share common tissue-typing characteristics. The faster and easier it is to locate relatives, the better the chances of treating patients.

Recently, the Foundation and JRI-Poland worked together to reconstruct, in just a few days, a family tree for a young leukemia patient. They are now actively working to find the best possible donor.

On Stan's most recent trip to Poland, he met with the head of a Warsaw Bone marrow registry to explain how genealogists are playing a role in the search for matches.

JUST a few months ago, Stan was the focus of a Canadian TV show, "Past Lives," which featured him in an episode, "Finding a cure through genealogy." Available on DVD to those making contributions to JRI-Poland, it is a moving presentation illustrating the impetus for his family research, JRI-Poland and more. For information, email Documentary@jri.poland.org.

AN active family historian since 1991, Stan is the founder and president of the Jewish Genealogist Society of Montreal, a lecturer, author of (and the subject of) many articles. He received the 2002 Lifetime Achievement Award of the International Association of Jewish Genealogical Societies. He is married to Ruth Mirjam Peerlkamp and has three daughters and four grandchildren.

For more information, contact Stan, SMSDiamond@aol.com.

Better yet, register for the conference, even for just a day or so if you cannot attend the full event, and learn about this project firsthand.