Knowledge, Attitude, Lifestyle Practices, and Quality of Life in Sporadic Lymphangioleiomyomatosis Patients

Shahpar Vafamand, Walden University


Lymphangioleiomyomatosis (LAM) is a rare lung disease recognized by abnormal growth of smooth muscle cells proliferating in lungs parenchyma, developing benign tumors, migrating to the other organs, and ultimately leading to respiratory failure and death. Despite existing literature mainly on clinical aspects of LAM, there is a gap of literature in regards to the knowledge, attitude, and lifestyle practices (KAPs) of LAM patients and their effects on their quality of life. The purpose of this quantitative study was to investigate the KAPs of the sporadic LAM patients as measured by the Bristol Chronic Obstructive Pulmonary Disease Knowledge Questionnaire, Beliefs and Behavior Questionnaire, Determinants of Lifestyle Behavior Questionnaire; these KAPs were then analyzed for their relationship to quality of life reports as measured by the St George's Quality of Life Questionnaire. Transtheoretical model (TTM) was used to describe the relationship among the variables. The data were collected through online survey questionnaires from 143 sporadic LAM patients registered at the LAM Foundation. Pearson's correlations and linear regression were used to analyze the data. The results of the analysis showed that there was a significant positive relationship between attitude, lifestyle practices, and quality of life and a negative relationship between knowledge and quality of life. The outcome achieved by this study and its implication on social change identifies the need to initiate more study-specific KAPs within LAM populations, including individuals with tuberous sclerosis complex LAM. The results could also encourage the LAM community as well as other stakeholders to implement programs, workshops, and interventions that could promote and enhance quality of life.