Ehlers-Danlos Syndrome (EDS) is
a group of hereditary connective
tissue disorders, characterized by
joint hypermobility, skin extensibility
and tissue fragility. Chronic,
early onset, debilitating musculoskeletal
pain is a symptom of EDS.
Individuals with EDS have a defect
in their connective tissue, the tissue
that provides support to many body
parts such as the skin, muscles and
ligaments. The fragile skin and unstable
joints found in EDS are the
result of faulty collagen. Collagen
is a protein which acts as a “glue”
in the body, adding strength and
elasticity to connective tissue. EDS
is considered to be a severe and
painful chronic musculoskeletal
disorder that can significantly reduce
a person’s quality of life, particularly
in the areas of sleep and
rest, recreation and hobbies, home
management, alertness, emotional
behavior, social interaction, and
movement.
Patients with EDS are often undiagnosed
or misdiagnosed for years.
They may be told they are merely
seeking attention or pain medications.
Clinicians may attribute their
difficulties to depression and refer
them to psychiatrists. When they
seek an answer regarding their
child’s pain or other symptoms,
parents may hear they are simply
“overprotective” of their undiagnosed
child or may be suspected of
child abuse because of frequent
bruising or dislocation.
Because management of EDSrelated
pain has not been studied in
depth, no treatment protocols exist.
One recent, but small, study of people
with EDS indicates that pain
associated with the condition may
be neuropathic in origin, as opposed
to the more commonly
thought musculoskeletal. Even after
correct diagnosis, unsuccessful trials
of multiple medications or modalities
may take their toll on patients
and those who support them
physically, emotionally, and financially.
The majority of adult patients with
EDS report to suffer from generalized
pain complaints, with frequencies
up to 100% for joint pain and
up to 87% for muscle pain. Chronic
pain is most frequently localized in
shoulders, knees, hips, neck and
back, and arms and legs, reflecting
the musculoskeletal pain pattern. In
addition, pain intensity is perceived
as moderate to severe and is continuously
present.
6—iPain Living Magazine