Background and Purpose: Ehlers-Danlos syndrome (EDS) is a connective tissue disorder characterized by joint laxity, chronic pain, and joint instability. Often, children with EDS exhibit polyarthralgia and fatigue as their main symptoms. Although physical therapy is a mainstay of treatment for individuals with EDS, evidence is lacking for specific exercises or treatment strategies for management of pain. The purpose of this case report is to describe the effectiveness of closed kinetic chain and endurance exercises on reducing pain in a child with EDS.
Case Description: The child was a 9 year old girl diagnosed with EDS hypermobility type, and a 3 week history of bilateral ankle pain. Her pain was worse when running and participating in sports (basketball and softball). She had long-standing polyarthralgia that occurred “all the time”, accompanied by fatigue during activities that had worsened over the past 3 weeks. The child wore ankle stabilizing orthotics at all times, and pain had caused her to discontinue playing sports. The child and her mother were seeking conservative treatment in an attempt to reduce her chronic pain and improve overall function.
Outcomes: Treatment focused on closed chain kinetic exercises. Initially, verbal feedback was given to obtain neutral range in weight-bearing joints in order to reduce microtrauma, facilitate proprioception, and recruit surrounding musculature for stability. After demonstrating proper form and reduced pain, the child was progressed to closed chain exercises within her full range of motion. The child's fatigue was addressed through the use of a Lower Body Positive Pressure Treadmill to reduce the forces applied from normal walking. The fact that she had a history of polyarthralgia suggested tailoring her treatment plan according to her functional capabilities and limitations, mainly hypermobility with reduced proprioception. After 6 weeks, the child reported decreased pain on the Faces Pain Scale from 7 out of 10 at initial evaluation to 2 out of 10 at discharge. Her Lower Extremity Function Scale, which identifies functional impairments, increased from 42/80 (52.5%) to 53/80 (66.3%). During the fourth week of her episode of care, she began wearing her Ankle Stabilizing Braces only during sports or when walking long distances. The child also reported less pain overall and was able to return to playing basketball, softball, and other extracurricular activities.
Discussion: These results support interventions utilizing progressive closed kinetic chain exercises into full hyperextension range for children with EDS to help to achieve functional outcomes. Since EDS is a chronic condition, longitudinal studies could help increase the reliability of these exercise programs by investigating musculoskeletal influences over time. Further research should contribute to developing evidence-based treatment strategies for this condition.
Duong L, Flores M. The Effects of Closed Kinetic Chain and Endurance Exercises on Reducing Pain in a Child with Ehlers-Danlos Syndrome: A Case Report. 2017.