Background

Lymphangioleiomyomatosis (LAM) is a rare, complex lung disease that occurs sporadically or in the context of TSC.  Comprehensive information about LAM and management decisions are essential for improving patient knowledge and adherence to therapy. Aim of our study was to investigate the level and resources of information in patients with LAM.

Methods

In cooperation with the German LAM foundation a questionnaire-based online survey (Lime Survey) and four semistructured focus group interviews were conducted. The questionnaire was descriptive, and interviews were analyzed using structured qualitative.

Results

105 completed questionnaires were analyzed. 53.33% of patients felt well or very well informed about the disease, but 10.48% felt inadequately informed or not informed at all. Mostly, patients received information through their pulmonary specialist (79.05%). Almost all participants (98.1%) used the Internet to obtain information about LAM, especially Facebook (88.41%). 67.62% participants stated that internet resources helped them to improve understanding of their disease. Most of the information was considered reliable (77.14%). The focus group interview revealed that patients demand more comprehensive information, especially after first diagnosis. Delayed diagnosis led to patientŽs uncertainty. Patients also critized marginal knowledge by non-specialized health care professionals. 

Conclusion

A relevant proportion of patients with LAM feels insufficiently informed about their own disease. As a relevant management component in LAM, improvement of disease education should be prioritized for patients but also for non-specialized physicians