School of Nursing


Doctor of Nursing Practice (DNP)


Debra Maddox


Education Intervention, Autoimmune Pulmonary Alveolar Proteinosis, Granulocyte Macrophage Antibody, Granulocyte Macrophage Colony Stimulating Factor, National Organization of Rare Disease, Pulmonary Alveolar Proteinosis, Whole Lung Lavage




With over 7000 types, rare diseases affect 25-30 million Americans and contribute to over one trillion dollars in healthcare-related costs. Prescribers lack the knowledge to improve awareness, identification, and treatment of rare diseases. Pulmonary alveolar proteinosis is a rare disease in which patients present with symptoms such as dyspnea, fatigue, and respiratory infections. Diagnosis can take four to 92 months. A differential diagnostic algorithm published in the literature can guide clinicians in identifying the type of pulmonary alveolar proteinosis the patient has. In addition, if the clinicians perform a laboratory test called the granulocyte-macrophage autoantibody test, this test provides 100% sensitivity and specificity, leading to a diagnosis of autoimmune pulmonary alveolar proteinosis that accounts for 90% of cases. This project aimed to implement an educational intervention for providers at pulmonary and primary care outpatient community clinics to increase confidence levels in identifying patients with pulmonary alveolar proteinosis, utilizing the differential diagnostic algorithm, and identifying the type following a case study. Using a pretest and posttest, the Wilcoxon signed-rank analysis showed statistical significance in all five outcomes that education improves the confidence of prescribers to identify and utilize the differential diagnostic algorithm, workup with lab tests and treatments, and provide resources for patients. In addition, 100% of prescribers used the differential diagnostic algorithm to correctly identify the type of pulmonary alveolar proteinosis following a case study. Limitations of this study included sample size and increased provider demand, which limited their time and availability.

Included in

Nursing Commons