Jagelman Registries for Hereditary Colorectal Cancer Syndromes Prove Their Value Long-Term

Largest inherited CRC tumor registry in North America tracks families, save lives

Cleveland Clinic’s Jagelman Registries, the largest tumor registries for inherited colorectal cancer (CRC) in North America, were established in 1979 by David Jagelman, MD, a colorectal surgeon and a pioneer in recognizing the importance of such registries for cancer patient care and cancer prevention. The database, which began as the Familial Polyposis Registry, was expanded about 10 years later to include all inherited colorectal cancer syndromes.

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Today, the registries include about 2,000 families, consisting of approximately 15,000 people.

Although only 5 percent of all cases of CRC are hereditary, physicians recognize that it is vital to identify them because the cancer can then be controlled through timely testing and treatment, and other affected family members also can be identified through genetic testing before cancers develop. The registries now follow patients with polyposis and nonpolyposis syndromes, the most prevalent being familial adenomatous polyposis (FAP) and Lynch syndrome, respectively.

Preventing cancer in families

“The primary purpose of the registry is to care for people and families with these diseases, to prevent the development of cancer, and to prevent deaths from inherited colorectal cancer,” says Matthew Kalady, MD, Co-Director of Cleveland Clinic’s Comprehensive Colorectal Cancer Program.

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The registries provide education for patients and families, genetic counseling and testing, and collect data for research. The registries are housed within the Sanford R. Weiss, MD, Center for Colorectal Neoplasia, founded in 2008 at Cleveland Clinic.

Patients come to the registries in different ways. “Patients may be diagnosed with a cancer or cancers at Cleveland Clinic, and we recognize that they have a CRC syndrome. These patients are enlisted in the registry, and we follow them,” Dr. Kalady explains. “Others are diagnosed elsewhere with one of the syndromes and come to Cleveland Clinic because of the specialized, multidisciplinary care available here.”

First, the family tree

Collecting family health histories and drawing a family tree is the first step in the process. This record graphically illustrates which family members were affected by what disease and when. “Identifying which disease family members have or had and when they developed it, gives us the information needed to diagnosis various syndromes, or at least guide our decision making for genetic testing and required interventions,” Dr. Kalady notes.

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Study shows the registries save lives

Cleveland Clinic researchers conducted a study in 2013 examining the registries’ impact on preventing mortality from cancer in patients affected by hereditary colorectal cancer syndromes. They identified families with at least three generations enrolled in the registries whose disease status could be documented by medical records. At the time of the study, they found a total of 12 families who fit these criteria. Nine families included three generations and three included four generations, representing a total of 335 individuals. Of these 335 family members, 100 were affected by FAP, including 35 patients in the third generation. None of the individuals in the third generation had died from colorectal cancer.

In multigenerational families in the registries, FAP is now being diagnosed at puberty and death rates are decreasing significantly, the study concludes. Registry coordinator, Lisa LaGuardia, RN, presented the study at the 2013 American Society of Colon and Rectal Surgeons (ASCRS) annual meeting.

“Members of earlier generations often died of cancer at an early age because the syndromes were not recognized yet and extensive screening was not always done. Now, by identifying the syndromes and screening at-risk family members, we have been able to lower the cancer mortality rate in the third generation to zero. That’s the power of a registry,” Dr. Kalady says.