Date of Award

December 2020

Degree Type

Thesis

Degree Name

Master of Science

Department

Occupational Therapy

First Advisor

Brooke A Slavens

Committee Members

Brooke A Slavens, Kris A Barnekow, Joyce M Engel

Keywords

ehlers-danlos syndrome, Hypermobile ehlers-danlos syndrome, kinematics, occupational therapy, pain, pediatrics

Abstract

Ehlers-Danlos syndrome (EDS) is a group of heritable connective tissue disorders, consisting of thirteen different subtypes. Among the thirteen, Hypermobile Ehlers Danlos Syndrome (hEDS) is the most common. Individuals with this condition present with frequent joint instability that results in ongoing subluxations and dislocations. Secondary diagnoses of this condition include chronic pain, Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS), Major Depressive Disorder (MDD), and Generalized Anxiety Disorder (GAD). The diagnostic process of hEDS is convoluted by the lack of a genetic identifier (Malfait et al., 2017). Individuals with hEDS are often not diagnosed until later in adulthood, leaving their adolescent life full of confusion and difficulty in managing the symptoms. As a result, children with hEDS endure a childhood of difficulty in participating in school, activities, and engaging with peersThe primary aims of this study were to characterize upper extremity kinematics during activities of daily living, to characterize pain location and severity, to understand common treatments for pain management, and to understand how pain severity can impact the child’s self-perception of disability due to pain in children with hEDS. This study included 11 children with hEDS. Kinematic data collection consisted of using a retroreflective marker set and 3D motion capture system. To obtain data on pain, a non-standardized Pain Severity and Location Questionnaire and Numerical Rating Scale (NRS) (Breivik et al., 2008) were administered interview style. Obtaining data on treatments, a non-standardized Treatment History Questionnaire was administered to the child’s legal guardian. The Functional Disability Inventory (FDI) was administered to identify the child’s level of perceived disability due to his or her pain (Kashikar-Zuck et al., 2011) Obtaining kinematic data of a normative pediatric population was restricted due to Coronavirus Disease (COVID-19). Existing literature was utilized to identify clinical differences, however, due to the variability between research this study cannot conclude whether children with hEDS have a greater range of motion than a normal pediatric group. However, this study identified that the four ADL tasks can be completed with wrist flexion and extension within values of 12° and 56° respectively and wrist ulnar and redial deviation within values of 20° and 11° respectively. At the shoulder, the tasks can be completed within 55° of shoulder flexion and 46° of extension. Within the four tasks, internal rotation required maximal of 42° and external rotation required 94. At the thoracohumeral joint, maximal shoulder flexion was 142 and abduction was 57 during the combing task. Internal rotation was greatest during the reaching across task, reaching 81. Data on pain location and severity displayed results that all children in this study reported pain in more than one bodily location. Further, all of the children in this study reported pain in their back. When describing their worst location of pain, all of the children in this study reported pain levels greater than or equal to 4/10, designating moderate to severe pain throughout the entire group. The maximal amount of treatments trialed was 10 while the least amount was 0. Parents reported a mode answer of ‘satisfied’ when questioned their level of satisfaction with treatments trialed for pain on a Likert satisfaction scale. The scores from the Functional Disability Inventory ranged from minimal to severe perceived disability. However, higher FDI scores did not correlate with higher NRS scores. movement patterns has the potential to assist in identifying phenotypic characteristics of this group, which in turn could inform treatment practices and guide future research in identifying a genotype. Additionally, obtaining information regarding chronic pain, fatigue, and psychological experiences in pediatric hEDS can inform researchers on pediatric specific symptoms to more accurately diagnose this population.

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