Does Rare Disease—EDS—Plague Fibromyalgia? #ShareYouAreRare

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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.

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Does Rare Disease—EDS—Plague Fibromyalgia?

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.

·        Dysautonomia

·        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).

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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.

https://www.ncbi.nlm.nih.gov/pubmed/23091361

(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.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1005086/?tool=pubmed

(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.

https://www.ncbi.nlm.nih.gov/pubmed/23444824

(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.

https://www.ncbi.nlm.nih.gov/pubmed/28167178   

(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.

https://www.openaccessjournals.com/articles/skin-ultrastructural-similarities-between-fibromyalgia-and-ehlersdanlos-syndrome-hypermobility-type.pdf

(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.

https://www.ncbi.nlm.nih.gov/pubmed/20046548

In healing,

Celeste Cooper, RN / Author, Freelancer, Advocate

Think adversity?-See opportunity!

http://www.CelesteCooper.com

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Learn more about Celeste’s books here. Subscribe to posts by using the information in the upper right hand corner or use the share buttons to share with others. 

David Simons, MD: Pioneer of Myofascial Medicine

Dr. Simons joined Dr. Janet Travell in her intense efforts to understand and educate other physicians regarding myofascial dysfunction, and the contribution of myofascial trigger points to myofascial pain syndrome. 

"Dr. David Simons started his medical career as a military clinician and research scientist. Since 1965 he [practiced] physical and rehabilitative medicine. Dr. Simons, a world leader in myofascial medicine, [lectured and advocated] for research in this “new field” of medicine. Drs. Travell and Simons co-authored Myofascial Pain and Dysfunction: The Trigger Point Manual, Vol. 1, published in 1983, and Vol. 2 in 1992. The revised edition of Vol. 1 was published in 1999." (Cooper and Miller, 2010) 

What you may not know is that Dr. David Simons, born in 1922, was Project Officer for Animal Studies in V-2 rocket flights until 1949. He graduated from the USAF School of Aerospace Medicine at Randolph Air Force Base. He is well known for his part in the part in the “Manhigh project, a series of balloon flights to record altitudes to study the effects of a near-space environment on human physiology.” Because of this any many other aeronautical achievements, he was inducted into the International Space Hall of Fame in 1987.

It was after retiring from the Air Force that he began his work with Dr. Janet Travell and they published the first medical textbooks on myofascial medicine. Dr. Simons also published approximately 200 publications on myofascial trigger points and chronic pain management. He and Dr. Travell are recognized internationally as trustworthy researchers.

To say that it was an honor to exchange email with Dr. Simons regarding myofascial pain syndrome is an understatement. When Dr. David Simons passed away in 2010, it was a great loss. He did so much to help so many, including myself. It is because of him and Dr. Travell that we can have better pain management today by pain specialists, chiropractors, and bodyworkers who know their work.

A friend of mine, Dr. John Whiteside (Australia), studied directly under Dr. Janet Travell. We became connected when he gave me permission to publish his protocol for treating trigger points in our book. When I expressed my remorse for the loss of Dr. Simons, he had this to say:  

"Great scientists and teachers outlive their mortal lives.”

--Dr. John Whiteside

No truer words have ever been spoken.

Celeste 

Janet Travell, MD: Pioneer of Myofascial Medicine

"The pioneer in the study of chronic myofascial pain as we know it today is Dr. Janet Travell, later joined by Dr. David Simons. Travell discovered as early as 1940 that by applying pressure to a trigger point, she could establish and predict referred pain patterns. After successfully treating President John F. Kennedy for residual effects from bouts with myofascial pain and longstanding back problems, she was the first woman and first non-military doctor to be appointed to the post of White House physician. Dr. Travell is considered to be an expert authority, and her work and dedication continue to be internationally referenced.

Thanks to [Dr. Travell and Dr. Simons] dedication to scientific study and education, we now know that specific triggerpoints cause specific pain patterns and symptoms." (Cooper and Miller, 2010) 

One only has to visit Dr. Travell’s family tribute to understand the huge impact she had as White House physician to President John F. Kennedy. I hope you will take a moment to honor her by visiting the family’s memorial website. It is a testament to her work, her dedication, and to her life.

Dr. Janet Travell (1901 – 1997) was not only the personal  physician to President Kennedy, but she was also an Associate Clinical Professor at the George Washington University and made many other contributions to medicine. A collection of her work consists of manuscripts, reports, correspondence, research data, articles, newspaper clippings, photographs, and a variety of material from Dr. Travell's service in the White House. It is said to include 104 boxes, or 44.5 linear feet of materials. The collection was donated to the Gelman Library University Archives in 1998 by her daughters with the stipulation that it can only be used to further the research and study of myofascial medicine. (Accessed,1-24-2015).  Her work will live on.

She made a huge impact on our knowledge of myofascial pain syndrome, its perpetuators to pain, and the role of the myofascial trigger point not only in pain, but also as a contributing factor to many other autonomic, circulatory, and nerve-related symptoms. One might say she was also the founder of ergonomics because she understood, studied, and educated others about the effects of furniture, clothing, and posture on the body. She studied relentlessly to identify and map out the pain and symptom referral patterns associated with the location of each specific myofascial trigger point. 

She and Dr. David Simons and published the first medical textbooks on myofascial medicine.

Few in medicine have made such an impact and she and Dr. David Simons has made. Both were children of physicians.

Is there a therapy for you? The role of treatments in chronic pain by Celeste Cooper

Body-work is important to the health of muscles that are dysfunctional, such as seen in myofascial pain syndrome. Myofascial pain syndrome  (MPS) is thought by many experts to be a main peripheral pain generator in most chronic pain conditions.  Chronic myofascial pain from sustained, untreated, or undertreated myofascial trigger points (knotted up pieces of muscle fiber that can be easily felt unless the muscle involved is too tight, too deep, or behind bone) is thought to be kept in perpetuity by the metabolic and autonomic effects of both FM and ME/CFS, meaning it makes treatment more difficult to sustain than it does in other patient populations.

Until myofascial trigger points are treated and muscle fiber is returned to its normal resting length, a sustained hold of the muscles involved, whether it be through Yoga or prescribed by a physical therapist, will not only discondition the muscle, it can create more pain and further development of MTrPs.  This is because the muscle has already reached its maximal capacity of stretch when trigger points are involved. More pain and dysfunction should not be the goal of therapies, but some do not realize they are doing more harm than good because they do not understand the pathophysiology behind trigger points. These same recommended therapies are helpful on down the road AFTER the muscle is returned to its normal state. We must educate those who treat us. We talk more about this in the next edition of Broken Body Wounded Spirit: Balancing the See-Saw of Chronic Pain, Spring Devotions, and our BIG book (here)  is devoted to understanding the role of chronic myofascial pain from trigger points in both FM and ME/CFS.  

Optimally, we need someone standing beside us using trigger point pressure and stroking the MTrPs as we move the muscle through its range of motion in order to coax the muscle back to its normal resting length. This is the theory behind Active Release Therapy, and though MTrPs are not addressed specifically in Feldenkrais Movement Therapy,  Alexander Technique, and Craniosacral Therapy, these therapies do help with restrictions found in skeletal muscle and connective tissue.  Spray and Stretch, Myofascial Release, and Myofascial Trigger Point Therapies are massage-like therapies .  

Addressing perpetuating factors with body work is done in Ashton Patterning, and Trager Work and an important part of prevention. Therapies for addressing the mechanical and emotional aspects of body-work are Hellerwork, T'ai Chi, Yoga, and Rosen Method.  The success or failure of each therapy is dependent upon patient dedication, education, and a therapist who is skilled in communicating with our body.  

All these therapies, including self treatment, are discussed at length in Integrative Therapies for Fibromyalgia, Chronic Fatigue Syndrome, and Myofascial Pain and throughout the Broken Body, Wounded Spirit series. Read more about the books here. 

My New Year’s resolution is to educate others on the myofascial and its role in chronic pain. Many of these therapies are not covered by insurance. However,we have evidence based research to show that body-work treatments are more effective than medications and other invasive treatments in treatment of soft tissue dysfunction as the result of a musculoskeletal problem. We need  to move integrative therapies into mainstream. If we can convince Medicare, Medicaid and private insurance companies that therapies such as these are more cost effective, we have a chance.

For now, some of the therapies can be costly. Most of us with disabling chronic pain have limited financial resources, myself included. For this reason, I believe self care through the use of tennis balls, a Theracane, mechanical massagers, rolling pins, swim noodles, Yoga balls and practicing gentle movement and stretch therapies are good alternatives. 

You can find helpful links on my website here. 

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All answers and blogs are based on the author's opinions and writing and are not meant to replace medical advice.

Celeste Cooper is a retired RN, educator, fibromyalgia patient, and lead author of the Broken Body Wounded Spirit: Balancing the See Saw of Chronic Pain devotional series (coauthor, Jeff Miller PhD), and Integrative Therapies for Fibromyalgia, Chronic Fatigue Syndrome and Myofascial Pain: The Mind-Body Connection (coauthor, Jeff Miller PhD) She is a fibromyalgia expert for Dr. Oz, et al., at Sharecare.com, here, and she advocates for all chronic pain patients as a participant in the Pain Action Alliance to Implement a National Strategy, here. You can read more educational information and about her books on her website, http://TheseThree.com

Pelvic Pain, Bladder Disorders, Prostate Problems, Fibromyalgia, Chronic Fatigue Syndrome, and Other Female and Male Related troubles: Is it more than co-incidence?

The muscles in the pelvic girdle are what keep our organs from falling to the floor. These muscles make up the perineum, the urogenital triangle, and the anal triangle. They support the rectum, the vagina/penis, and the urethra, but they may not be the only muscles involved in your pain and dysfunction.

Causes

Pelvic pain can be from many causes such as, vulvodynia, irritable bladder or interstitial cystitis, infection, vaginal atrophy, prostate problems/pain, testicular and or pain in the penis, pain in the urethra (where your urine comes out), rectal pain, ovarian cysts, ectopic pregnancy, neuralgia, endometriosis, inflammatory bowel diseases, irritable bowel syndrome, diverticulitis, and myofascial trigger points (MTrPs), but for this blog we are looking specifically at the bladder and the perineum (area of the urethra, penis, vagina, and rectum).

Myofascial trigger points have been identified as the greatest aggravator of chronic pelvic pain, and pain is not the only symptom. Pelvic floor problems can also cause a decrease in urine flow in men and women, erectile dysfunction, urinary retention (setting the stage for infection), urgency (always feeling like you have to urinate), and constipation.

For more on myofascial trigger points and myofascial pain see “Myofascial Pain” at my website and
 my blog: Points That Need More Than Pondering: Defining Myofascial Trigger Points


Offending trigger points

Myofascial trigger points in adductor magnus (thigh), or internal oblique (abdomen), are capable of causing bladder pain and frequency, and MTrPs in the adductor magnus can cause a host of referred pain to groin and inner thigh, pelvic and pubic bones, rectum and vagina and can cause menstrual cramping (as can MTrPs in the rectus abdominus, abdomen), and trigger points in the internal oblique can also cause bladder difficulties. The muscles of the pelvis, and the multi-layered muscles of the pelvic floor can become tight, unforgiving and short due to MTrPs. Myofascial trigger points in pelvic related muscles can refer pain to the urethra, rectum, coccyx, or the crease of the buttocks.

This is speaking in generalities, but it’s important to understand that the source of your pain can be close by or well away from pelvis itself.  Treating MTrPs, whether active (painful without touching) or latent (only painful with touched) that refer pain to a specific region is just as important as treating those directly relatable. Often times, those who claim to know myofascial trigger points do not understand the complexity, this includes physicians, physical therapists, and body workers.


Chronic myofascial pain in fibromyalgia, chronic fatigue syndrome, and pelvic dysfunction

Myofascial pain syndrome often co-exists in fibromyalgia, and has been identified in some chronic fatigue syndrome (ME/CFS) patients, chronic pelvic and bowel disorders.  Myofascial trigger points are a peripheral nerve to muscle problem that lends to centralized (amplified) pain in fibromyalgia, interstitial cystitis, bladder difficulties, ME/CFS, IBS, and other overlapping conditions.  This hypersensitive state is also present in these disorders. Ignoring the obvious bloodies the diagnostic waters and most importantly delays appropriate treatments and leads to flawed research.


Therapies

It is important to identify perpetuating factors, such as, co-existing hip problems, piriformis syndrome, pudendal neuralgia, low back or sacroiliac joint dysfunction, and other overlapping conditions, bringing them under control when possible. Pay close attention to aggravating factors such as, sitting too long or on hard surfaces and chairs that can’t be adjusted to your body type, over activity, infection, poor posture, wearing pants that are too tight, consuming offending foods, etc.

There are a variety of therapies to help you, including intravaginal and pelvic floor trigger point injections, external and internal massage of the perineum and in women the vagina, biofeedback, bladder retraining, transcutaneous electrical nerve stimulation (TENS), tennis ball therapy (as discussed in our book),
acupuncture, dietary changes, over-the-counter probiotics for the bladder, stretching movements, topical analgesics (such as oragel), oral analgesics, and of course specific myofascial therapy by a trained specialist. Sometimes, all are necessary.

Seldom are doctors well informed about myofascial pain s and trigger points, so I am a firm believer that women should see a urogynecologist, that men should see a urologist and in both cases, the physician should understand the role of the myofascial in chronic pelvic pain.  The same is true for the physical therapist. Why? Those who do not understand the role of trigger points chronic pelvic pain and dysfunction may suggest traditional therapies, such as, Kegel exercise, which can worsen your symptoms, and when co-existing conditions such as piriformis syndrome, spinal disease, IBS, etc. are involved; a host of referral patterns are involved.  This is why identifying ALL your pain patterns (whether you feel a trigger point there or not) is important information for your specially trained healthcare provider.

Always discuss your symptoms with your doctor to make sure other causes are ruled out. If your pain and dysfunction is not found to be from another source, please look for those myofascial trigger points and a specialized therapist, they are treatable.

Resources for you:

IC and Irritable bladder
Blatman Pain Clinic
What Your OB/GYN Should Know About FMS and CMP by Devin J. Starlanyl
Pelvic Floor Myofascial Trigger Points: Manual Therapy for Interstitial Cystitis and the Urgency-Frequency Syndrome by Jerome Weiss
Fibro Care Center
National Association of Myofascial Trigger Point Therapists
ICA – Physical Therapy
ICA – Pelvic Floor Dysfunction
International Myopain Society
IC Network


(Signature line appended, March 2018)

In healing,

Celeste Cooper, RN / Author, Freelancer, Advocate

Think adversity?-See opportunity!

http://www.CelesteCooper.com

~ • ~ • ~ • ~ • ~ • ~

Learn more about Celeste’s books here. Subscribe to posts by using the information in the upper right hand corner or use the share buttons to share with others.

All blogs and comments are based on the author's opinions and are not meant to replace medical advice.