“Getting stuck with LAM”: patients perspectives on living with Lymphangioleiomyomatosis

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease that affects almost exclusively women and is most often diagnosed before menopause. The main symptom of LAM is shortness of breath. LAM patients’ perceptions of how the disease impacts their lives is largely unknown, but such information could be useful to generate patient reported outcome measures for use in drug trials (or other research studies) and to formulate interventions aimed at easing the burdens LAM imposes on patients. OBJECTIVE: To capture patients’ perceptions of how LAM affects their lives. METHODS: We used reflexive team analysis to analyze transcripts from semi-structured focus groups conducted with LAM patients at LAMposium 2013. We sought to determine what patients perceive as the primary symptoms of LAM and how the disease affects them in their daily lives. RESULTS: The 37 participants described seven primary symptoms of LAM and five common psychological experiences from living with the disease. Shortness of breath and low energy (or fatigue) dominated the symptomatic picture; cough, sensations in the chest, difficulty sleeping, gastrointestinal issues, and mild cognitive difficulties were less common. The common psychological experiences participants reported included frustration, worry, loss of identity, embarrassment, and in some participants, a healthy defiance against the disease. CONCLUSIONS: Patients perceive the physical symptoms from LAM to be intrusive and limiting. Women living with LAM are frustrated by their physical limitations, and they worry about what the future will be like if the disease progresses. Therapeutic interventions should take aim at improving these perceptions.