The natural progression of EDS
(hypermobility type) has recently
been re-delineated, at which three
phases of disease evolution can be
recognized based on the onset of
specific symptoms affecting multiple
systems, such as pain.
In terms of musculoskeletal pain,
the first phase (onset in the first
decade) is characterized by acute,
local pain due to soft-tissue and
joint injuries. In the second phase
(onset in the second to third decade),
widespread musculoskeletal
pain is dominant, due to pain sensitization.
And the third phase (onset
in the third to fourth decades)
shows that the chronic pain condition
may worsen disability when
maladaptive cognitions (e.g., pain
catastrophizing, fear of pain) have
been developed.
Musculoskeletal pain is not congenital
in Ehlers-Danlos Syndrome
and is often influenced by external
factors, such as lifestyle, sport activities,
traumas, surgery, and various
co-morbidities. Many patients
report their very first painful sensations
acutely, in relation to joint
traumas, such as dislocations and
sprains, and “growing pain” mostly
localized at the knees. These manifestations
are indistinguishable
from those observed in the general
population. Nevertheless, children
and young adults with EDS tend to
report such symptoms with unexpectedly
high rate and intensity.
EDS FACTS
About 1 in 5,000 people has
EDS. Treatment involves managing
symptoms. Individuals with
EDS often experience both acute
and chronic discomfort/pain related
to a variety of causes.
Pain is common and severe in
EDS. The results of a large Danish
study showed that:
Chronic pain in EDS is highly
prevalent and associated
with regular use of medications
Pain is more prevalent and
more severe in the hypermobility
type than in the classic
type
Pain severity is correlated
with hypermobility, dislocations,
and previous surgery
Pain is correlated with low
nocturnal sleep quality
Pain contributes to functional
impairment in daily life,
independently of the level of
fatigue
Acute discomforts from EDS are
often the results of fractures, dislocations,
subcutaneous bleeding
or bruising after falls.
Research in 23 women with EDS
-HT has shown that the percentage
of painful body surface is
significantly higher in the EDSHT
group compared to a healthy
control group. EDS-HT patients
experienced pain for more than
24 hours in the past 4 weeks on
an average of 31% of their body
surface, compared to 1% in the
control group.
Recent research highlighted that
84% of (mostly female) patients
with EDS are severely fatigued.
The impact of fatigue on daily
life is often equal or more dramatic
than the impact of pain.
Some probable contributors to
fatigue-related disability have
been investigated and include
sleep disturbances, concentration
problems, social functioning, self
-efficacy concerning fatigue, and
pain severity.
Additionally, research describing
pain features in 51 individuals
with different forms of EDS
found that neck pain and headache
accounted for 30–40% of
cases.
(Connued on page 6)
Internaonal Pain Foundaon—7