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“Living with [Benign Joint Hypermobility
Syndrome] BJHS is not without consequences. When I was seven months pregnant, I
fell down a flight of stairs because of lax hip joints. (JHS can be especially
pronounced during pregnancy.) After my
son was born, I was referred to an orthopedic doctor who diagnosed me with JHS.
I have had 4th-degree sprains of both ankles, four shoulder repairs for torn
tendons and muscles, and one shoulder has been reconstructed because of JHS. I
experience ileo-tibial band laxity causing unstable hips, piriformis syndrome,
and a lot of pain. For years, I have lived with sagging and abnormally thin
skin that tears easily, like that of someone on long-term steroids. And I
battle chronic Achilles tendonitis. But others diagnosed with JHS could have a
vastly different experience than I have, because none of us is affected exactly
the same.”
Excerpt
of part of my story in an article I wrote on joint
hypermobility and chronic pain for HealthCentral.
There
are many theories regarding joint hypermobilty and the connection to Ehler’s
Danlos Syndrome (EDS), a genetic collagen disorder. Some researchers consider Benign Joint
Hypermobility Syndrome (BJHS) to be a milder form of one of the many types of EDS.
(1) According to NORD,
there are thirteen subdivisions.
NORD established the annual awareness day for
rare diseases on the last day of February. RareDiseaseDay.org/ tells us to #ShareYouAreRare
and more.
Last
year, the following appeared on ProHealth and with ProHealth’s permission, I am
sharing it in its entirety here on The Pained Ink Slayer.
_________________________________________________________________________
According
to the National
Organization for Rare Disorders (NORD), there are many Ehlers Danlos
Syndromes and related disorders caused by different genetic defects in
collagen. We will focus on the most common type of Ehlers-Danlos syndrome in
this article.
hEDS
= EDS hypermobile type, previously known as EDS type III or joint hypermobility
syndrome.
Characterized
by joint hypermobility, skin extensibility and tissue fragility, this type of
EDS can co-occur in fibromyalgia and myofascial pain syndrome even though it is
thought to be a rare condition. Fibromyalgia and EDS also share connections you
might not have considered.
The EDS
Connection
Gastrointestinal
problems are noted in both hEDS and fibromyalgia, and myofascial pain syndrome
has been noted in both hEDS and fibromyalgia.
A
2014 study, A Prospective Evaluation of Undiagnosed Joint Hypermobility
Syndrome in Patients with Gastrointestinal Symptoms (2), found upper and lower
GI symptoms increased with the severity of joint hypermobility type. Upper GI
symptoms were dependent on autonomic and chronic pain factors.
A 1993 study published in
the Annals
of the Rheumatic Diseases (3) suggests there is a strong association
between joint hypermobility and fibromyalgia in schoolchildren and joint
hypermobility may play a role in developing fibromyalgia. Keep in mind, in
1993, few understood the incidence of myofascial pain syndrome in chronic pain
conditions, which could be true in this case. It would be interesting to see a
follow up study on the participant group to see how many of these children would
meet today’s suggested fibromyalgia criteria.
In
2013, a French study, Fibromyalgia: an unrecognized Ehlers-Danlos syndrome
hypermobile type? (4), reports some patients suffering from fibromyalgia
present with clinical signs and alterations in tissues, changes in antibodies
that test for certain markers found in tissue, and alterations to the
microscopic structure of skin that is similar to hEDS. They also suggest some
types of fibromyalgia could represent undiagnosed joint hypermobility.
A
2017 case study (5) highlights that patients with EDS type III [hEDS] may
suffer from pain due to myofascial trigger points around the affected
hypermobile joints, not a surprise to me. I have been writing about this for
years. Myofascial pain syndrome is a chronic pain condition caused by
myofascial trigger points and it appears to perpetuate pain in both
fibromyalgia and hEDS.
Symptoms
Symptoms
may vary, but there are general symptoms. Don’t be surprised by the number
similar to fibromyalgia and/or myofascial pain syndrome.
·
Loose,
unstable joints prone to injury
·
Easy
bruising.
·
High
and narrow palate with teeth crowding
·
Small
fragile blood vessels
·
Velvety-smooth
skin which may be stretchy
·
Abnormal
wound healing and scar formation
·
Low
muscle tone and weakness
·
Muscle
and joint pain
·
Joint
pain associated with exercise
Other criteria
may include:
·
Family
history
·
Headaches
caused by resulting Arnold-Chiari malformation (noted in some FM patients)
·
Low
bone density
·
Not
knowing where body parts are in relation to space, loss of proprioception
·
Difficulty
processing information rapidly
·
Functional
gastrointestinal disorders, such as delayed stomach emptying, inflammation of
the stomach, and irritable bowel syndrome
·
Vascular
skin conditions, such as Raynaud’s and skin moddling due to a sympathetic nerve
response known as livedo reticularis
What about you?
I
was born with lax joints, just like my maternal grandmother and aunt. Well into
adulthood, I sat with my legs splayed as if chicken wings, or crossed the other
direction, yoga style. I have gotten myself into some precarious positions that
result in pain, and like fibromyalgia, I can’t always predict when that will
happen. I continue to struggle with the effects. Whether joint hypermobility is
benign (without genetic markers), or genetic (as seen in hEDS), it is not
without consequences. Because of lax hip joints, I fell down a flight of
stairs; I was seven months pregnant. I have had 4th-degree sprains of both
ankles, several bouts of Achilles tendonitis, inflammation of multiple joints,
four shoulder repairs for torn tendons and muscles due to dislocation, knee
surgery, and I experience ileo-tibial band inflammation caused by unstable
hips, compensatory piriformis syndrome, and a lot of pain. And I am not alone.
There are many like me. Are you?
The future
We
need more research on this connection. Folks can’t seem to agree on diagnostic
criteria or terms for hEDS or fibromyalgia, which is extremely confusing and
frustrating. Most likely this is because of the lack of robust research. Funding
for research on rare diseases is grossly lacking. We need to raise awareness.
(Edited
February, 2019) This year February 28th is Rare Disease Day in the United
States. The U.S. Pain Foundation has provided various ways to
engage within our community. Check out the awareness events taking place
and share the word in your communities.
You
can find more information on Ehlers-Danlos Syndromes at NORD,
and the NIH, Genetic and Rare Diseases Information Center (GARD).
_________________________________________________________________________
The
amount of research of this painful rare disorder over the past two decades is
limited, but what we do have suggests more investigation is needed and many
more people may be affected than realized. Symptoms of EDS can overlap with FM and
could present as a mimicking or co-existing condition. Joint hypermobility and
skin disorders have been more frequently observed in children with FM (6) than
in otherwise healthy children, skin ultrastructural similarities between FM and
EDS hypermobility type have been noted (7), and some types of EDS can perpetuate
the development of myofascial trigger points that cause chronic myofascial pain
(8).
Research
Resources:
(1)
Hermanns-Lê T, Reginster MA, Piérard-Franchimont C, Delvenne P, Piérard GE, and
Manicourt D. (2012). Dermal ultrastructure in low Beighton score members of 17
families with hypermobile-type Ehlers-Danlos syndrome. Journal of Biomedicine and Biotechnology; 2012:878107.
(2)
Fikree A, Grahame R, Aktar R, Farmer AD, Hakim AJ, Morris JK, Knowles CH, Aziz
Q. (2014). A prospective evaluation of undiagnosed joint hypermobility syndrome
in patients with gastrointestinal symptoms. Clinical
Gastroenterology and Hepatology; (10):1680-87. https://www.ncbi.nlm.nih.gov/pubmed/24440216
(3)
Gedalia A, Press J, Klein M, and Buskila, D. (1993). Joint hypermobility and
fibromyalgia in schoolchildren. Annals of
the Rheumatic Diseases; 52(7): 494–496.
(4)
Hermanns-Lê T, Piérard GE, Angenot P. (2013). Fibromyalgia: an unrecognized
Ehlers-Danlos syndrome hypermobile type? [Article in French]
La Revue
Médicale de Liège;
68(1):22-4.
(5)
Tewari S, Madabushi R, Agarwal A, Gautam SK, Khuba S. (2017). Chronic pain in a
patient with Ehlers-Danlos syndrome (hypermobility type): The role of
myofascial trigger point injections. Journal
of Bodywork and Movement Therapies; 21(1):194-196.
(6)
Ting TV, Hashkes PJ, Schikler K, Desai AM, Spalding S, Kashikar-Zuck S. (2012).
The role of benign joint hypermobility in the pain experience in Juvenile
Fibromyalgia: an observational study. Pediatric
Rheumatology Online Journal;10(1):16. https://www.ncbi.nlm.nih.gov/pubmed/22704360
(7)
Hermanns-Lê T and Pierard GE. (2016). Skin ultrastructural similarities between
Fibromyalgia and Ehlers-Danlos syndrome hypermobility type.
International
Journal of Clinical Rheumatology; 11(2), 019-022.
(8)
Fernández-de-Las-Peñas C. (2009). Interaction between Trigger Points and Joint
Hypomobility: A Clinical Perspective. Journal
of Manual and Manipulative Therapy;17(2):74-7.
In healing,
Celeste Cooper, RN / Author, Freelancer, Advocate
Think adversity?-See opportunity!
~ • ~ • ~ • ~ • ~ • ~
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