title: Characterization of pain, disability, and psychological burden in Marfan syndrome.
abstract: The clinical manifestations of Marfan syndrome frequently cause pain. This study aimed to characterize pain in a cohort of adults with Marfan syndrome and investigate demographic, physical, and psychological factors associated with pain and pain-related disability. Two hundred and forty-five participants (73% female, 89% non-Hispanic white, 90% North American) completed an online questionnaire assessing clinical features of Marfan syndrome, pain severity, pain-related disability, physical and mental health, depressive symptoms, pain catastrophizing, and insomnia. Eighty-nine percent of respondents reported having pain with 28% of individuals reporting pain as a presenting symptom of Marfan syndrome. Almost half of individuals reported that pain has spread from its initial site. Participants in our study reported poor physical and mental health functioning, moderate pain-related disability, and mild levels of depressive symptoms, sleep disturbances, and pain catastrophizing. Those who identified pain as an initial symptom of Marfan syndrome and those who reported that pain had spread from its initial site reported greater psychological burden compared with those without pain as an initial symptom or pain spreading. Physical health is the largest predictor of pain severity and pain-related disability. While pain catastrophizing and worse mental health functioning are significant correlates of pain severity and pain-related disability, respectively. Pain is a significant and persistent problem in Marfan syndrome and is associated with profound disability and psychological burden. Further studies are indicated to better characterize the directionality of pain, pain-related disability, and psychological burden in Marfan syndrome. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Traci J
authors.initials: TJ
authors.lastname: Speed
authors.affiliation: Department of Psychology, Texas A&M University, College Station, Texas.
authors.forename: Vani A
authors.initials: VA
authors.lastname: Mathur
authors.affiliation: Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Matthew
authors.initials: M
authors.lastname: Hand
authors.affiliation: Division Pain Medicine, Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Bryt
authors.initials: B
authors.lastname: Christensen
authors.affiliation: Department of Orthopaedic Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Paul D
authors.initials: PD
authors.lastname: Sponseller
authors.affiliation: Division Pain Medicine, Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Kayode A
authors.initials: KA
authors.lastname: Williams
authors.affiliation: Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Claudia M
authors.initials: CM
authors.lastname: Campbell
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D006233:Persons with Disabilities; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000523:psychology*; D008875:Middle Aged; D009483:Neuropsychological Tests; D010146:Pain / Q000175:diagnosis*; D010147:Pain Measurement; D010641:Phenotype; D011159:Population Surveillance; D012042:Registries; D057566:Self Report; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: Marfan syndrome; catastrophizing; pain; quality of life; questionnaire