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MATERIALS AND METHODS

The Little People of America Inc. (LPA)1 is the largestadvocacy support organization of its kind in theUnited States, consisting of individuals with skeletal dysplasias or short stature syndromes their families, and their friends. The general membership of the LPA were asked to participate in a mailed questionnaire survey after the purpose of the study was described in detail at the annual national meeting. Four thousand questionnaires were mailed out to all members of LPA. Fifteen hundred individuals with short stature or, in the case of children under age 11, their parents completed the survey. There were 816 respondents with achondroplasia including 437 adults, 66 teenagers, and 76 children. This analysis focuses only on the 437 adults with achondroplasia. The questionnaire consisted of a disease-specific and general medical comorbidities questionnaire, a general demographics questionnaire (including information on self-reported diagnosis, past operations, family history, and socioeconomic indicators), and the Short Form 36 (SF-36) [Ware and Sherbourne, 1992]. The questionnaire on general comorbidities included hypertension, myocardial infarction, angina, congestive heart failure, diabetes mellitus, and cancer. Disease-specific problems asked about included chronic allergies/sinus trouble, chronic ear infection, arthritis or rheumatism, sciatica or chronic back problems, difficulty with vision, chronic lung disease, chronic skin rash, hearing difficulty, limitations in use of upper or lower limbs due to weakness or paralysis, chronic neck problems, spine deformity, endocrine dysfunction, and sleeping disorders. The SF-36 is a well-developed and widely used ‘‘instrument’’ for measuring a population’s general health status [Greenfield et al., 1992; Stewart et al., 1989; Tarlov et al., 1989]. It comprises eight subscales (physical functioning, role physical, bodily pain, general health, vitality, social functioning, role emotional, and mental health) and a health transition question. Recently, the Medical Outcomes Trust has developed two summary scores based on these eight subscales called the Physical Component Summary (PCS) score and the Mental Component Summary (MCS) score [Ware et al., 1995].