Pain Characteristics and Symptom Management in Children with Hypermobile Ehlers–Danlos Syndrome and Hypermobility Spectrum Disorder

Abstract

Aims

This study aims to investigate pediatric hypermobile Ehlers–Danlos syndrome (hEDS) and hypermobility spectrum disorder (HSD) pain features and management strategies.

Methods

This is a mixed-methods, cross-sectional study design using patient-reported outcomes in 21 children diagnosed with hEDS/HSD. Children who reported bothersome pain were interviewed for pain features. The Child Activity Limitation Interview-21, the Brief Pain Inventory pain interference items, and the Functional Disability Inventory were used to investigate pain interference. To evaluate psychological symptoms regarding pain, the pediatric version of the Survey of Pain Attitude and the child version of the Pain Catastrophizing Scale were used.

Results

Nineteen children had bothersome pain and of them, eight children reported constant pain. The most frequently reported regions of pain were at the ankle (mild pain) and the back (moderate-to-severe pain). Children reported mild-to-moderate pain interference and believed medications were beneficial for their pain management. Nineteen children sought treatment and of those 16 children used to exercise and acetaminophen and 13 visited physicians as a means of treatment. Parents were overall satisfied with their child’s treatment (13 out of 19).

Conclusions

Sufficient awareness of pain-related symptoms and understanding of the treatment strategies in early childhood is needed to prevent deleterious consequences in adulthood.

Keywords:

• Pain attitudes
• pain interference
• pain management
• pediatrics
• treatment

Acknowledgements

We thank Alyssa Schnorenberg, Amy Ott, Anahita Qashqai, Caroline Kielczewski, Chris Cho, Jordyn Prell, Lisa Koneazny, Samantha Schwartz, and Stephanie Gerber for their assistance with this study.

Disclosure Statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

The authors acknowledge the UWM College of Health Sciences for funding this research through a Stimulus Program to Accelerate Research Clusters (SPARC) grant, the Children’s Specialty Group at Children’s Wisconsin, and the Administration for Community Living, U.S. Department of Health and Human Services, National Institute on Disability, Independent Living, and Rehabilitation Research [90ARHF0006].

Notes on contributors

Hyo-Jung Jeong

Hyo-Jung Jeong, PT, PhD, MS, is a NIDILRR ARRT postdoctoral fellow in the Orthopaedic and Rehabilitation Engineering Center (OREC) at Marquette University. Her current research is on understanding the pain and biomechanical phenotypes in children with hypermobility spectrum disorder and hypermobile Ehlers-Danlos syndrome. She is particularly interested in understanding the pain and movement patterns over lifespan.

Joyce M. Engel

Joyce M. Engel, PhD, OT, FAOTA, is a Professor Emeritus in the Department of Rehabilitation Science and Technology at the University of Wisconsin-Milwaukee (UWM). Her research aims to decrease pain and suffering in the pediatric pain populations.

Olivia Wilwert

Olivia Wilwert, OT, MS, is an occupational therapist at Froedtert Hospital. Her research investigates upper extremity kinematics and pain outcomes during activities of daily living in children with hypermobile Ehlers-Danlos syndrome.

Michael Muriello

Michael Muriello, MD, FACMG, currently works as a clinical geneticist at the Medical College of Wisconsin. He has a broad background in clinical genetics and the care of patients with rare disorders through his training at Johns Hopkins University, which included a wide variety of experiences at the Johns Hopkins Hospital, the NIH, and Children’s Nationwide hospital. He was trained to care for and diagnosing patients with hereditary connective tissue disorders including Marfan syndrome, Loeys-Dietz syndrome, and others.

Donald Basel

Donald Basel, MBBCh, FACMG, has worked within the broad field of connective tissue disorders and is intimately familiar with the subtle clinical nuances of hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorder. He has a keen interest in connective tissue biology and in understanding the underlying mechanisms of disease in patients with Hereditary Connective Tissue Disorders.

Brooke A. Slavens

Brooke A. Slavens, PhD, is an Associate Professor in the Department of Rehabilitation Sciences & Technology and Biomedical Engineering at the University of Wisconsin-Milwaukee. She is the Director of the Mobility Lab and has expertise with pediatric rehabilitation research. Dr. Slavens’ research interests include pediatric mobility, musculoskeletal modeling, rehabilitation engineering, and orthopaedic biomechanics.