FUNCTIONAL HEALTH-STATUS OF ADULTS WITH ACHONDROPLASIA

Little is known regarding the functional health status of individuals with achondroplasia. This cross-sectional survey of adults with achond roplasia was undertaken to assess the functional health status of this population and its determinants. The study sample consisted of member s of the Little People of America (LPA) who completed a mailed questio nnaire consisting of a demographics component, a general and disease-s pecific comorbidities component, and the Short Form 36 (SF-36) health status questionnaire. Univariate analyses and multivariate linear regr ession models were used for data analysis. Four hundred thirty-seven i ndividuals with a mean age of 38 years completed the survey. The age- and gender-adjusted Mental Component Summary (MCS) scores did not sign ificantly differ from those of the general population. In contrast, th e age-and gender-adjusted Physical Component Summary (PCS) scores were significantly lower than the general population starting in the fourt h decade of life. Musculoskeletal diseases were most prevalent and had the greatest impact on the PCS scores. Two-thirds of this cohort had undergone at least one operation. Only musculoskeletal procedures were significantly associated with PCS and MCS scores, The functional heal th status of adults with achondroplasia, as measured by the SF-36, is not drastically reduced in comparison with that of the general U.S. po pulation. (C) 1998 Wiley-Liss.