Hand Impairment and Function in Children and Adolescents With Heritable Disorders of Connective Tissue

Importance: Heritable disorders of connective tissue (HDCTs) affect hand function and participation in daily activities for children and adolescents. Objective: To describe hand impairment and function and determine the extent to which hand impairment and function explain the variation in self-reported functional performance. Design: Cross-sectional observational study. Setting: Specialist tertiary hospital. Participants: Children and adolescents ages 8–18 yr with HDCTs (N = 73). Intervention: None. Outcomes and Measures: Hand function outcomes included grip strength (digital dynamometer), manipulation and dexterity (Functional Dexterity Test, Nine-Hole Peg Test), and fine motor skills (Bruininks–Oseretsky Test of Motor Proficiency). Upper limb hypermobility was assessed using the Upper Limb Hypermobility Assessment Tool. Hand pain and fatigue were recorded for a timed button test and 3- and 9-min handwriting tasks. Functional performance was measured using the Childhood Health Assessment Questionnaire. Results: Scores on all hand function measures were below expected norms. Pain and fatigue were significantly worse after the writing tasks (p < .001) but not the button test (p > .40). Secondary students had significantly lower handwriting scores than primary students (p = .03) but similar grip strength z scores (p = .95). Variation in self-reported functional performance was explained by grip strength (6%) and upper limb hypermobility and dexterity (16%). Conclusions and Relevance: Young people with HDCTs have poor hand function attributable to poor grip strength and hand pain and fatigue. Comprehensive upper limb evaluation and ongoing monitoring throughout the school years are warranted to inform timely intervention. What This Article Adds: Children and adolescents with heritable disorders of connective tissue have difficulty with hand function that affect their participation in daily activities. The results of this study can help clinicians identify, assess, and monitor daily activities, performance skills, and symptoms of children and adolescents with HDCTs to promote their participation in all aspects of daily life.