Self- and Parent-Reported Physical and Psychosocial Outcomes for Children with Arthrogryposis

Abstract Background Children with arthrogryposis have impaired motion and strength; however, they develop compensatory strategies to achieve better function than may be anticipated. Impairment measures, such as range of motion and strength, describe physical function, but do not characterize a person’s psychosocial function, or their ability to perform activities and participate in life roles. The Patient Reported Outcome Measurement Information System (PROMIS®) measures domains of function from the perspective of people with a chronic disease, compared to a reference population. We hypothesized that PROMIS® scores would discriminate between children with arthrogryposis and a pediatric reference population, and that children with arthrogryposis would report impaired upper extremity function and mobility, but normal pain interference with activities and normal peer relationships. Methods This is a retrospective cohort study of children with arthrogryposis aged 5-17 years who responded to four pediatric PROMIS® physical and psychosocial domain questionnaires (Mobility, Upper Extremity (UE) Function, Pain Interference, and Peer Relationships) during clinic visits at Shriners Hospital for Children, Northern California from April 2017-May 2019. Responses were converted to a T-score for comparison to the reference population (mean score = 50, standard deviation = 10). Results PROMIS® questionnaires were administered to 65 children with arthrogryposis with a mean age of 9.5 years. Participants reported moderately impaired Mobility (38±9) and UE Function (32±12), both significantly lower than the reference population; 63% reported moderate or severe mobility impairment, and 77% reported moderate or severe impairment with UE Function. Regarding psychosocial function, participants reported excellent Peer Relationships (average 54±8), with 98% reporting excellent or good Peer Relationships. They also reported normal Pain Interference (average 49±10), with 82% reporting normal or mild Pain Interference. Conclusions PROMIS® effectively discriminates between children with arthrogryposis and the reference population. Children with arthrogryposis report moderately impaired physical function (Mobility and UE Function) but normal psychosocial function (Peer Relationships and Pain Interference.) PROMIS® is a useful tool to evaluate and understand the challenges that children with arthrogryposis face.