Development of ATAQ-LAM: a tool to assess quality of life in Lymphangioleiomyomatosis.

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a progressive lung disease that impairs health-related quality of life (HRQL). OBJECTIVE: To develop and conduct initial testing of ATAQ-LAM (A Tool to Assess Quality of Life in LAM). METHODS: A pilot version of the questionnaire was administered to respondents with LAM. We used a deletion algorithm to retain items and then applied multi-trait scaling to place retained items into appropriate domains, thus generating the ATAQ-LAM. Rasch analysis was used to assess item fit to a unidimensional model of HRQL. We determined internal consistency (IC) and floor and ceiling effects of ATAQ-LAM scores and conducted analyses aimed at supporting the validity of ATAQ-LAM. RESULTS: Sixty-nine LAM patients provided response data. Thirty-two items survived the deletion algorithm. Scaling suggested ATAQ-LAM should have a four-domain structure (Exertional dyspnea, IC = 0.94; Cough, IC = 0.91; Fatigue, IC = 0.91; Emotional Well-Being, IC = 0.89). All items fit the Rasch model. Among 17 respondents with spirometry within three months of questionnaire completion, three of five ATAQ-LAM scores correlated with FEV1% (Exertional Dyspnea: r = -0.72, p = 0.001; Fatigue: r = -0.62, p = 0.007 and total: r = -0.53, p = 0.02). Compared with those in the highest tertile of FEV1%, subjects in the lowest tertile had greater ATAQ-LAM total (121.8 ± 14.3 vs. 79.8 ± 13.1, p = 0.04), Exertional Dyspnea (54.4 ± 6.3 vs. 25.5 ± 5.8, p = 0.005) and Fatigue (2.8 ± 2.4 vs. 14.8 ± 2.3, p = 0.03) scores, indicating greater impairment in HRQL. CONCLUSIONS: ATAQ-LAM is a disease-specific instrument designed to assess HRQL in LAM patients. Additional studies are needed to generate data in support of its validity as an instrument capable of assessing HRQL over time in LAM patients.

"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.

Effectiveness of Circle of Life, an HIV-preventive intervention for American Indian middle school youths: a group randomized trial in a Northern Plains tribe.

OBJECTIVES: We assessed the effectiveness of Circle of Life (COL), an HIV-preventive intervention developed specifically for American Indian and Alaska Native (AI/AN) middle school youths. METHODS: By partnering with a tribal community, we conducted a longitudinal wait-listed group randomized trial with 635 seventh and eighth graders in 13 schools of a Northern Plains tribe. We surveyed participants at baseline, 3 months, and 12 months from 2006 to 2007. RESULTS: COL was found to increase HIV knowledge in the short term, but had no effect on sexual activity compared with those who did not receive it. However, COL was found to be effective for delaying the onset of sexual activity, with the greatest reduction in risk occurring for those receiving COL at early ages. CONCLUSIONS: Community partnership was key to successful project design, implementation, and analysis. The project confirmed the importance of the timing of interventions in early adolescence. COL may be a key resource for reducing sexual risk among AI/AN youths.

Circle of life: rationale, design, and baseline results of an HIV prevention intervention among young American Indian adolescents of the Northern Plains.

In spite of significant disparities in sexual health outcomes for American Indian youth, no studies exist examining the effectiveness of HIV-prevention interventions. Circle of Life is an HIV-prevention intervention specifically developed for American Indian middle-school youth. We describe the rationale, methodology, and baseline results of a longitudinal randomized trial of Circle of Life conducted among American Indian youth aged 11-15 in a reservation community. The innovative design includes two pre-intervention waves to determine patterns of behavior prior to the intervention that might be associated with a differential impact of the intervention on sexual risk. We used one-way analysis of variance and chi-square tests to test for significant differences between randomized group assignment at each baseline wave and generalized estimating equations (GEE) to test significant differences in the rate of change in outcomes by group longitudinally. We present the collaborative and adaptive strategies for consenting, assenting, and data collection methodology in this community. Achieved response rates are comparable to other similar studies. Results from the two baseline waves indicate that few outcomes significantly varied by randomized intervention assignment. Ten percent of youth reported having had sex at Wave 1, rising to 15% at Wave 2. Among those who had had sex, the majority (>70%) reported using a condom at last sex. The project is well positioned to carry out the longitudinal assessments of the intervention to determine the overall impact of the Circle of Life and the differential impact by pre-intervention patterns of behavior across youth.

Culture, context, and sexual risk among Northern Plains American Indian Youth.

American Indian adolescents have two to four times the rate of sexually transmitted diseases (STDs) compared to whites nationally, they shoulder twice the proportion of AIDS compared to their national counterparts, and they have a 25% higher level of teen births. Yet little is known about the contemporary expectations, pressures, and norms that influence American Indian youth or how those might be shaped by today's lived cultural experiences, which frustrates attempts to mitigate the apparent disparity in sexual health. This paper used data from focus groups, in-depth interviews, and surveys with American Indian adolescents and young male and female adults from a Northern Plains tribe to contextualize sexual risk (and avoidance). Placing the findings within an adapted indigenist stress-coping framework, we found that youth faced intense pressures for early sex, often associated with substance use. Condoms were not associated with stigma, yet few seemed to value their importance for disease prevention. Youth encountered few economic or social recriminations for a teen birth. As such, cultural influences are important to American Indian sexual health and could be a key part of prevention strategies.