Background: Muscular dystrophies (MDs), characterized by progressive muscle wasting, are associated with 1 in 2,500 deaths in the United States. Although treatments slow the progression, these disorders lead to early death, usually due to cardiac or respiratory failure.
Methods: We analyzed death record data from 18,315 MD-associated deaths that occurred in the United States in 1986 through 2005 to assess trends in the age at death of people with MDs.
Results: From 1986 through 2005, the MD-associated mortality rate did not change among blacks, whites, males, or females. The median age at death among white females with MDs was 12 years higher than among black females. The frequency of reported cardiomyopathy increased among white but not black male decedents with MDs, although cardiomyopathy remained more commonly reported among black males. Among white males, the median age at death increased by 0.2 annually for those with and 1.3 for those without indications of cardiomyopathy. Among black males, the median age at death increased 0.3 years annually among those without reported cardiomyopathy. Among white males, the frequencies of pulmonary failure and pulmonary infection decreased significantly over time.
Conclusions: Changes in age at death and reported clinical comorbidities reflect improvements in the treatment of MDs. White males with MDs have shown a greater increase in age at death over time than black males. Contributing factors to this difference might include differences in types of MDs, rates of genetic and environmental modifiers, natural history, socioeconomic factors, and access to and use of treatment options.