Adherence to American Academy of Pediatrics Recommendations for Cardiac Care Among Female Carriers of Duchenne and Becker Muscular Dystrophy

OBJECTIVE. The goal was to assess women's knowledge and heart health behaviors consistent with the American Academy of Pediatrics recommendations for cardiac care among female carriers of Duchenne/Becker muscular dystrophy. METHODS. Using an advocacy group mailing list and working with 50 Muscular Dystrophy Association clinics, we surveyed women who had given birth to a son with Duchenne/Becker muscular dystrophy, thought that they were definitely or probably (>= 50% likelihood) a Duchenne/Becker muscular dystrophy carrier, or both. Self-report data classified respondents as carriers, noncarriers, or women with unknown status. RESULTS. The respondents included 833 Duchenne/Becker muscular dystrophy carriers, 376 noncarriers, and 192 women with unknown status. Carriers were more likely than noncarriers and women in the unknown-status group to have ever undergone electrocardiography or other heart testing and to have seen a cardiologist in the past year, but they were not more likely to report a recent blood pressure or cholesterol level check. Only 64.4% of the carriers had ever had a heart test; 18.3% had seen a cardiologist in the past year. Only 62.9% of the carriers were aware of their cardiomyopathy risks before participating in the survey; 69.3% had informed their health care provider of their carrier status. Among carriers who had informed their provider, 70.2% had ever had a heart test and 21.4% had seen a cardiologist in the past year. In adjusted logistic regression models, factors that significantly increased the likelihood among carriers of ever having had a heart test and seeing a cardiologist in the previous year included older age (>= 50 years), feeling informed about their cardiomyopathy risks before the survey, and having told their provider about their carrier status. CONCLUSION. More health education efforts are needed for both patients and their providers, to improve adherence to the American Academy of Pediatrics cardiac care guidelines for female Duchenne/Becker muscular dystrophy carriers. Pediatrics 2009; 123: e471-e475