Canadian respirologists' experience with lymphangioleiomyomatosis.
BACKGROUND
Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease occurring primarily in women. A literature review of LAM in Canada found sporadic mention of LAM in case reports or within lung transplant studies. The LAM Foundation, a patient support and research funding organization, lists 23 Canadian patients in its database. The present study was designed to assess the scope of LAM across Canada and to identify potential patients for further evaluation.


OBJECTIVE
To ascertain Canadian respirologists' experience with patients with LAM (current and historical), lung transplantation (single or bilateral) and deaths due to LAM, and awareness of the LAM Foundation.


METHODS
Four hundred twelve brief surveys were sent anonymously to members of the Canadian Lung Association (inserted in their newsletters) to ascertain the experience of Canadian respirologists with LAM.


RESULTS
One hundred twelve surveys were returned (27%). Fifty-one respondents had "ever" been involved in the care of at least one patient with LAM; eight had cared for three or more patients. At the time of the study, 26 respondents were following a total of 46 patients with LAM; 22 of the 51 respirologists (43%) who had ever cared for a patient with LAM reported the death of a patient. Thirty-three patients had been put on wait lists for transplantation; six died while on the wait list. Nineteen patients underwent lung transplantation--six single-lung recipients and 13 bilateral lung recipients. Of the 51 respirologists who had ever cared for a patient with LAM, only 30 (61%) were aware of the LAM Foundation's services. Of the 112 respondents, only 47 (43%) were aware of the LAM Foundation.


CONCLUSIONS
This study identified a moderate level of awareness of a significant existing patient support and research service (the LAM Foundation). There were many patients with LAM who were unknown to the LAM Foundation and could benefit from its resources. Results suggest that there may be more patients with LAM in Canada than are reported in the existing literature.
Pollock-BarZiv
Stacey M
SM
Centre for Research in Women's Health, Sunnybrook & Women's College Health Sciences Centre, 790 Bay Street, 7th Floor, Toronto, Ontario M5G 1N8, Canada. s.pollock.barziv@utoronto.ca
Cohen
Marsha M
MM
Maclean
Heather
H
Downey
Gregory P
GP
D000328:Adult; D002170:Canada / Q000453:epidemiology; D005260:Female; D006801:Humans; D016040:Lung Transplantation / Q000706:statistics & numerical data*; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D010818:Practice Patterns, Physicians'; D015272:Pulmonary Medicine / Q000592:standards / Q000639:trends; D035583:Rare Diseases; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D015996:Survival Rate; D014850:Waiting Lists