PREFACE

Medical genetics is a relatively new field of study. The chromosomal basis of many recognizable syndromes, for instance, was not known until 1958 when the underlying cause of Down syndrome (trisomy 21) was discovered. In the 1970's and 80's it was possible to catalog what was known about single gene disorders in a single text. However, through innovative research in a number of different fields, knowledge about genes and genetic disorders is expanding at such a rate that catalogs of genetic disorders are now out of date at the time of printing. The integration of basic research and clinical application has spawned a new specialty in medicine, Medical Genetics, which was officially recognized as a medical subspecialty by the American Board of Medical Subspecialty in 1993.

Genetics is essential in many areas of medicine. It is now possible to identify the presence of deleterious genes and chromosomal abnormalities prior to implantation or birth. The underlying cause of many syndromes and genetic disorders have been identified and improved treatment strategies are being developed. Genetic tests can also be used to establish a diagnosis or identify gene carriers prior to the onset of symptoms. Additionally, DNA-based molecular diagnosis is replacing or complementing routine laboratory tests.

The role that genetics plays in the etiology of common diseases has captured the attention of the general public. It is not uncommon to peruse a popular magazine and find articles on prenatal diagnosis, the genetics of breast cancer or the latest in gene therapy. As our knowledge of medical genetics expands and public interest in the subject grows, the challenge for health care providers is to recognize individuals who might benefit from a genetics referral, to share accurate information about the new screening tests that are being developed for common and chronic diseases, and to respond to the unique problems in the care of patients and families with genetic afflictions.

Recognizing the importance of genetics in health care, and the unique role that community health nurses play in the delivery of health care services in South Dakota, the State Department of Health has requested the Birth Defects Genetics Center staff to conduct in-service training for CHNs on a number of occasions. Given the high turnover rate of nurses and the limited time available, a self-study guide was deemed essential. The goal of this effort is not to create more genetic experts but rather to provide health care professionals (social workers, nutritionists, physical therapists, occupational therapists, nurses, nurse practitioners, physicians, physician assistants, and educators) with the necessary background information, skills and tools to recognize the need for a genetics referral, identify area resources and provide supportive care for individuals and families with a genetic disorder.

We would like to thank the South Dakota State Department of Health for the inspiration, the University of South Dakota School of Medicine for the support and the Great Plains Genetics Service Network Education Committee for the resources to make this curriculum a reality.

Virginia P. Johnson, MD Carol Christianson, MS

Professor, Medical Genetics Genetic Counselor

Departments of OB/GYN, Pediatrics, and Laboratory Medicine

University of South Dakota, School of Medicine

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