Knowledge of Alpha-1 Antitrypsin Deficiency Among Internal Medicine House Officers and Respiratory Therapists: Results of a Survey

BACKGROUND: Alpha-1 antitrypsin deficiency is a common genetic condition that predisposes to emphysema and liver disease. Alpha-1 antitrypsin deficiency is under-recognized, so affected individuals often experience long delays in diagnosis and visits to multiple physicians before correct diagnosis. Reasoning that inadequate knowledge about alpha-1 antitrypsin deficiency could contribute to this under-recognition, we designed this study to evaluate internal medicine house officers' and respiratory therapists' (RTs) knowledge of alpha-1 antitrypsin deficiency.

METHODS: We evaluated knowledge of alpha-1 antitrypsin deficiency with a Web-based test containing 30 multiple-choice questions. Invitations to take the test were sent via e-mail to all internal medicine house officers and RTs at The Cleveland Clinic main campus hospital. We assessed test scores by profession, years of training/experience, and self-assessed knowledge of alpha-1 antitrypsin deficiency.

RESULTS: Of 332 invitees, 202 (61%) responded, of whom 165 (50%) provided complete responses (99 RTs, 66 physicians). The mean scores (percent of correct answers) were 54% and 52% for physicians and RTs, respectively (P = .25). The scores did not differ among the physicians when examined by subspecialty (pulmonary/critical care vs other) or post-graduate education level (P = .94). RTs who had graduated from a 4-year respiratory therapy program had a higher mean score than those who had graduated from a 2-year program (56% vs 50%, P = .02). Respondents' whose self-assessment of their knowledge about alpha-1 antitrypsin deficiency was “somewhat knowledgeable” had higher test scores than any other self-assessed knowledge level, regardless of profession.

CONCLUSIONS: These results indicate a generally low level of knowledge about alpha-1 antitrypsin deficiency among physicians and RTs. Causes of under-recognition of alpha-1 antitrypsin deficiency, including the possibility of poor knowledge as a contributor, warrant further study.