QUESTION: Is a diagnosis of JHS associated with an increased risk for other musculoskeletal disorders?
Dr. Grahame: Most clinicians are aware of the susceptibility and vulnerability to trauma and joint instability that accompanies hypermobility, but few seem to be cognizant of the devastating effects on function and quality of life posed by the frequent occurrence of chronic pain and deconditioning that can occur if the condition is not handled correctly.
QUESTION: What are some of the consequences of delayed diagnosis, and what are some of the benefits of early diagnosis?
Dr. Grahame: Delayed diagnosis leaves patients not knowing what is causing their pain and distress. Being told that hypermobility cannot be responsible for their degree of pain only serves to cause further dismay. Even more serious than the upset to their emotional equilibrium is the progressive loss of mobility that they suffer, resulting ultimately in their becoming potentially chairbound or even bedbound. Because movement is painful, they intuitively adopt a strategy of pain avoidance through movement avoidance. The result is a predictable decline in independence and a descent into disability. The result is that people who are generally young and formerly physically active in various pursuits can be struck down in their prime, in spite of having locomotor systems that are largely intact.
QUESTION: What are some of the most important management considerations?
Dr. Grahame: Hypermobile subjects should be encouraged to achieve maximal fitness at all ages as a means of avoiding many of the musculoskeletal lesions to which they are predisposed by virtue of their fragile tissues. Having strong muscles helps to improve proprioception, stabilize core muscle groups and unstable joints, and improve stamina. Physical therapists have effectively adapted the principles of physiotherapy to the needs of patients with lax and fragile tissues.
Chronic pain in JHS generally does not respond well to analgesic medications, but pain management using cognitive behavioral methods has enabled many patients to return to a reasonable quality of life. Finally, surgery should be undertaken only after careful consideration of the risks of delayed healing, after the anticipated impact on outcome of a connective tissue disorder have been considered, and when conservative alternative nonsurgical treatments are not available.
This column, "Ask the Expert," regularly appears in Rheumatology News, an Elsevier publication. Dr. Grahame is also an honorary consultant in pediatric rheumatology at the Great Ormond Street Hospital for Children and emeritus professor of rheumatology at University College London. He disclosed having no conflicts of interest.