title: Hereditary Hemorrhagic Telangiectasia: A Primer for Critical Care Nurses.
abstract: Hereditary hemorrhagic telangiectasia is a rare, autosomal dominant genetic disease that causes abnormal growth of blood vessels and, subsequently, life-threatening arteriovenous malformations in vital organs. Epistaxis may be one of the initial clues that a patient has more serious, generalized arteriovenous malformations. Recommended treatment involves careful evaluation to determine the severity and risk of spontaneous rupture of the malformations and the management of various signs and symptoms. The disease remains undiagnosed in many patients, and health care providers may miss the diagnosis until catastrophic events happen in multiple family members. Prompt recognition of hereditary hemorrhagic telangiectasia and early intervention can halt the dangerous course of the disease. Critical care nurses can assist with early diagnosis within families with this genetic disease, thus preventing early death and disability.
authors.affiliation: Kathleen M. Sacco is the director, Intensive Care Unit and Cardiopulmonary Services, Simi Valley Hospital, Simi Valley, California.Thomas W. Barkley, Jr, is president of Barkley and Associates, West Hollywood, California. kathysacco@gmail.com.
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: Sacco
authors.affiliation: Kathleen M. Sacco is the director, Intensive Care Unit and Cardiopulmonary Services, Simi Valley Hospital, Simi Valley, California.Thomas W. Barkley, Jr, is president of Barkley and Associates, West Hollywood, California.
authors.forename: Thomas W
authors.initials: TW
authors.lastname: Barkley
mesh_terms: D002543:Cerebral Hemorrhage / Q000209:etiology* / Q000451:nursing* / Q000503:physiopathology; D003131:Combined Modality Therapy; D003422:Critical Care / Q000379:methods*; D064648:Critical Care Nursing / Q000379:methods*; D042241:Early Diagnosis; D004844:Epistaxis / Q000175:diagnosis / Q000209:etiology; D020022:Genetic Predisposition to Disease; D006764:Hospitals, Community; D006801:Humans; D007362:Intensive Care Units; D008297:Male; D024802:Nurse's Role; D009733:Nursing Diagnosis / Q000379:methods; D011379:Prognosis; D018570:Risk Assessment; D015996:Survival Rate; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000235:genetics* / Q000503:physiopathology / Q000628:therapy; D055815:Young Adult