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Cross-sectional assessment of pain and physical function in skeletal dysplasia patients

Authors

  • Y Alade,

    1. Alan and Kathryn Greenberg Center for Skeletal Dysplasias, McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University, Baltimore, MD, USA
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  • D Tunkel,

    1. Department of Otolaryngology-Head and Neck Surgery, Division of Pediatric Otolaryngology, Johns Hopkins University, Baltimore, MD, USA
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  • K Schulze,

    1. Alan and Kathryn Greenberg Center for Skeletal Dysplasias, McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University, Baltimore, MD, USA
    2. Centers for Human Nutrition, Department of International Health, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA
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  • J McGready,

    1. Alan and Kathryn Greenberg Center for Skeletal Dysplasias, McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University, Baltimore, MD, USA
    2. Department of Biostatistics, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA
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  • G Jallo,

    1. Department of Neurosurgery, Division of Pediatric Neurosurgery, Johns Hopkins University, Baltimore, MD, USA
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  • M Ain,

    1. Department of Orthopedics, Division of Pediatric Orthopedics, Johns Hopkins University, Baltimore, MD, USA
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  • T Yost,

    1. Department of Orthopedics, Division of Pediatric Orthopedics, Johns Hopkins University, Baltimore, MD, USA
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  • J Hoover-Fong

    Corresponding author
    • Alan and Kathryn Greenberg Center for Skeletal Dysplasias, McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University, Baltimore, MD, USA
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  • Dr Hoover-Fong receives consultative funding from BioMarin for study development related to achondroplasia.

Corresponding author: Julie

Hoover-Fong, MD, PhD, Alan and

Kathryn Greenberg Center for Skeletal

Dysplasias, McKusick-Nathans Institute

of Genetic Medicine, Johns Hopkins

University, 600N. Wolfe Street, Blalock

1008, Baltimore, MD 21287, USA.

Tel.: +1 410 955 3071;

fax: +1 410 502 2375;

e-mail: jhoover2@jhmi.edu

Abstract

Short stature skeletal dysplasia (SD) patients have orthopedic and neurologic complications causing significant pain and physical disability. We conducted a large cross-sectional online survey in 361 people with short stature SD (>10 years) to describe pain prevalence, characteristics, and the relationship between pain and function. Chronic pain prevalence per Brief Pain Inventory (BPI) was 70.3%. Women reported more pain than men (73% vs 63% p = 0.04). Pain Severity Score (average of current, worst, least and average pain) averaged 3.3 ± 2, while the Pain Interference Score (with daily activities) averaged 3.4 ± 2.7 on a 10-point scale. Per Bleck scale, 20.5% had little or no functional capacity. Increasing age and decreased ambulation independently predicted chronic pain. Chronic pain is prevalent in short stature SD patients and associated with poor physical function. Further study is required to clarify the temporal relationship among pain, function and treatments.

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