What is a registry?
According to the Webster’s New World College Dictionary, Third Edition, a registry is defined as “an official record or list.” Registries vary in their orientation and the services they provide. In 1925, the first registry was established in London, England, at St. Mark’s Hospital. Since then many registries have developed throughout the world. The registries in the United Kingdom, where there is a uniform health care system with specialists that can provide direct patient care and family data are contained within a defined parish, county or city and are an example of regional registries. National registries are typical of smaller countries like Sweden, Denmark and Holland where data collection is standardized to avoid duplication of efforts in tracing families. In North America, registries tend to develop around a physician or team of physicians who work in a tertiary referral medical center and who have a special interest in FAP (Familial Polyposis) and hereditary colorectal cancer syndromes. Patients may self-refer or be referred because of the surgeon’s technical ability or the unique services that the institution can offer. In this way, families are accumulated and a patient base is built. Patients may come from all over the country and make annual “pilgrimages” to the registry for surveillance. An example of such a registry is the David G. Jagelman Inherited Colorectal Cancer Registries at The Cleveland Clinic, established by David G. Jagelman, M.D. in 1979. Like most hereditary colorectal cancer registries, it follows families with hereditary colorectal cancer syndromes as Familial Polyposis, Hereditary Non-Polyposis Colorectal Cancer (HNPCC), Familial Colorectal Cancer, Juvenile Polyposis, and Peutz-Jeghers syndrome.

What is the role of the registry?
The main role of a registry is to prevent colorectal cancer and:

• to promote the knowledge of the risks and implication of a family history of colorectal cancer
• provide the best care to patients and families
• conduct important research.

The registry will: provide patients with education about the disease, tests, surgical options and lifelong surveillance protocols; encourage participation in study protocols; discuss the appropriateness of genetic testing; provide support to ease fear of tests and surgeries; and provide counseling on insurance issues, loss of employment or death of a loved one. The registry can provide extra help and understanding to patients who are fearful of procedures or have incorrect information and may need extra help in understanding the nature of the disease and have necessity for surveillance. This is especially helpful when they are not having any symptoms or discomfort.

One of the most important functions of the registry is to ensure that patients receive the best care and continue to have surveillance exams. The registry will steer patients to understand the importance of maintaining surveillance protocols for themselves and other family members. However, patients may have problems complying with surveillance protocols for a variety of reasons, including health insurance issues, lack of knowledge, incorrect information or fear of tests and surgery. Other patients without medical insurance may not be eligible for federal programs which can provide free medical care. Some managed care providers may not cover testing or may not allow patients to go out of their health care network.

Where do I find information about registries?
Patients are becoming more consumer-savvy and may look up information on the Internet. They may find information on registries by looking under “colorectal cancer, hereditary colorectal cancer, familial polyposis” or searching Web sites of larger hospitals or cancer centers.

Patient education publications from hospitals, cancer centers, government agencies, the library, the American Cancer Society, and medical journals may be another resource. Recommendation by word-of-mouth is always a great resource.

How do I join a registry?
Patients can self-refer and contact the registry themselves. Some patients are referred by their primary care physician or a specialist such as a gastroenterologist or surgeon. Others are referred by a health care provider such as a nurse, an enterostomal therapist or social worker.

Joining a registry can be as simple as picking up the telephone, writing a letter, sending an e-mail or making an appointment with the registry. After talking to a member of the registry staff (usually the coordinator) about your family medical history, it can be determined if participation in the registry is appropriate for you and your family.

Being in a registry does not affect your care with your own physician. It is important to note that patients in a registry are still under the care of their referring doctors. Registries do not assume the care of its participants. The main role of the registry is to educate.

Who do I contact in the registry?
Ask to speak to the registry coordinator when calling a registry. The registry coordinator is the key person in the team, serving as a link between patients, their families and the registry team. The registry coordinator usually will have an educational background in social work, nursing or in the health care profession. Probably the most important person in the registry, this person will be responsible for the daily activities of the registry and patient contact.

The coordinator is part of the team of physicians, nurses, geneticists, genetic counselors, mental health professionals and data managers who work together to promote patient education, provide the best patient care, and participate in research.

The registry coordinator will always be an advocate for the patient regarding problems with health insurance, social or family stress, a need for a second opinion, or other problems that affect the patient’s medical care. Patients can rely on the coordinator as a resource, knowing that the registry will always have their welfare and their family’s welfare in mind. Through intimate contact with patients and families, the registry coordinator will build a trusting relationship, allowing for honest feelings and fears to be discussed and dispelled.

What information will the registry need?
At your initial meeting, whether in person or over the telephone, the coordinator will start taking a family history. Information on your immediate, as well as extended family (grandparents, cousins, great aunts and uncles, etc.) regarding medical problems, especially colorectal cancer, colorectal polyps and other cancers will be collected. The cause of death and the ages of onset of polyps or cancer is particularly important. Birth dates and death dates are also important.

Constructing your family pedigree is an ongoing process. It requires time and patience to gather all the initial information. Some families have already started their family tree, or have a family bible with information recorded, and may have death certificates on deceased family members available. It is usually a long process, taking multiple phone calls with other family members, to gather accurate information. Since families are forever changing, updating a family pedigree is an ongoing process.

It is necessary to collect accurate information since it will be used to determine the risk factors and surveillance recommendation for the entire family. It is important to keep in contact with the registry to update changes in your family, because it will affect the risk factors and surveillance recommendations.

What will I have to do?
The registry will send out a packet of information, including the following forms, to be completed and returned in a timely manner.

• Family history
• Consent for participation
• Medical release

Filling out the family history form is essential when developing the family pedigree. It will categorize names, birth dates, ages, causes of death, and who had polyps, colorectal cancer or other cancers.

Another essential task is signing the consent for participation form and returning it to the registry. Informed consent must be obtained to cover confidentiality and privacy issues such as the ability to contact other family members and the process of obtaining outside medical records. Prior to enrollment in the registry, all patients and family members are asked to sign a consent for participation form. The consent form will include information on the benefits of participating, risks, treatment options, registry staff names and phone numbers and costs. Specific items can include a request for a blood sample for banking and future research, confidentiality of records, discussion of screening and identification, and possible diagnosis and treatment. Participation in the registry is voluntary.

Patients and certain family members may be asked to sign a medical release form. This form is necessary to collect medical data on patients and relatives. Diagnostic medical information, such as pathology, procedure, and surgical reports, is necessary for documentation of polyps, colorectal cancers and other cancers. Patients can sign their own medical release forms. For deceased relatives, the next of kin or a person who has power of attorney can sign the medical release form. Living relatives must sign their own release.

What is done with this information?
The signed medical release forms are sent to the hospitals where treatments were provided. Medical records may also be obtained from physician offices. A copy of the requested medical records is sent to the registry. The coordinator will review the information and document the necessary data in the family pedigree. These records are stored in a family chart and kept in confidential files.

Documentation is essential to confirm family history. Information on causes of death, occurrences of polyps, colorectal cancer and other cancer must be collected to make an accurate evaluation of the risk of colorectal cancer and to make screening recommendations for diagnosed and at-risk family members.

Is genetic testing available?
Patients who are interested in genetic testing can be referred to a clinical genetics program or a Risk Assessment Program, where the appropriate method of testing is determined. Genetic testing can be performed within the institution. Patients will be sent to the blood laboratory where several tubes of blood will be drawn. The blood specimens will be sent to an outside laboratory for testing. The outside laboratory will notify the genetics department and genetic counselor of the results.

Results of the test are presented to the patient at a meeting with the geneticist and genetic counselor. The results and risks, including preventative and screening recommendations, are explained to the patient. A report will be mailed to the patient, and the copy will be kept in a confidential file in the registry.

What about confidentiality?
All contacts with the registry, data collected and genetic information (including test results) are maintained and kept in secured confidential files within the registry. The information collected for the registry is not part of the patient’s permanent record.

What patient educational literature is available?
You also will receive several patient booklets, newsletters and education materials.

The David G. Jagelman Inherited Colorectal Cancer Registries
Colorectal Surgery / A30
The Cleveland Clinic Foundation
9500 Euclid Avenue
Cleveland, OH 44195
800/998-4785
E-mail: laguarl@ccf.org