Craniosacral Therapy and Ehler-Danlos Syndrome
Ehlers-Danlos Syndrome (EDS) is a genetic, connective tissue disorder affecting collagen production. Since collagen is found throughout the body, all systems, structures and biomechanics can be affected. The most noticeable physical manifestation is joint hypermobility as described by the Beighton Criteria (6), a scoring system for joint hyperflexion and hyperextension (see fig 1). There are six major types of EDS recognized by the Ehlers-Danlos Society (3): Classical, Hypermobility, Vascular, Kyphoscoliosis, Arthrochalasia, and Dermatosporaxis. The severity of symptoms varies among the types and among individuals. The most common type, EDS-Hypermobility Type (EDS-HT), is the focus of this paper.
EDS-HT is autosomal dominant, meaning that only one parent with Ehlers-Danlos Syndrome is needed to pass an affected gene to their children which will express itself with varying severity per individual. Unfortunately, there has not yet been a gene coded for Hypermobility Type, but some promising new research has been published in which multiple copies of the alpha-tryptase gene (TPSAB1) have been found in some individuals with a compilation of autonomic nervous system dysfunction (dysautonomia), mast cell activation disorder and hypermobility (11). Symptoms of EDS often worsen with the onset of puberty and tend to progress throughout life. Currently, there is no cure for Ehlers-Danlos Syndrome and conventional treatment is focused on palliative care, physical therapy and pain management.
Diagnostic criteria for EDS-HT is an ongoing, international debate among specialists but includes clinical hypermobility assessments using the Beighton criteria, family history and whether or not there is multi-system involvement (12). Unfortunately, the Beighton Criteria is a flawed scoring technique, as it ignores hypermobility in the shoulders, knees, small finger joints, ankles, sacrum, vertebrae and circumferential stability in the hips. In addition it does not consider mediolateral instability, gender or age. Women and children tend to be more hypermobile than men and decreased flexibility increases with age (6,7). Proper diagnosis is often significantly delayed until adulthood and patients are more likely to have spent a lifetime labeled as a hypochondriac, or misdiagnosed with chronic fatigue syndrome, fibromyalgia, multiple sclerosis, conversion disorder, anxiety, and/or depression.
In this author’s opinion, there is an overwhelming lack of education about EDS for doctors and medical personnel. Personal discussions with colleagues and doctors about their individual training and knowledge of EDS-HT have been disappointing. In this author’s experience, many doctors freely admit that their medical school education did little to prepare them or educate them about connective tissue disorders. This educational vacuum heavily contributes to the reasons EDS is not as well-known as it should be and why doctors are reluctant to diagnose and treat it, placing patients at risk, and prolonging the potential suffering
Symptoms and Complications
The name, EDS-Hypermobility Type, is misleading. The joints, are of course affected, but so is every system in the body due to the lack of collagen. These include the structure and function of the craniosacral system, autonomic and central nervous systems, sensory organs, proprioception, cardiovascular system, respiratory system fascial system, integumentary system, gastrointestinal and reproductive systems, lymph system – EVERY SYSTEM is affected (10). Additionally, there is some overlap with mitochondrial disease and possibly ion channel disorders (channelopathies).
One of the primary and most debilitating symptoms of EDS is widespread, unrelenting and varying pain, with episodes of acute, excruciating pain when there is a direct joint injury, subluxation or dislocation. For many, these occur daily with mild physical impact or sometimes no impact at all. Consequently, the ligamentous laxity in the joints causes compensatory muscle recruitment for additional stabilization, which leads to muscle fatigue and small muscle fiber spasm, causing full body pain and exhaustion. Many people with EDS-HT require assistive devices to stabilize joints. Bruising or mild bleeding tendencies are common and variable due to the fragility of the venous structures; the skin is often soft, mildly stretchy, and delicate.
Of importance is the direct involvement of the central nervous system. Dysautonomia or autonomic nervous system dysfunction, is estimated to occur in varying degrees in 100% of people with EDS. It affects both the sympathetic and parasympathetic branches with a tendency towards sympathetic hyperactivity. Patients may experience heart rate, blood pressure and temperature dysregulation, pre-syncope, syncope (fainting), gastroparesis (slow digestion), Reynaud’s syndrome, anxiety/depression, poor sleep/insomnia, and trouble concentrating.
It is unclear why the autonomic nervous system is affected. Dysautonomia occurs in other illnesses as well, however, due to the high percentage of collagen in the dural system, we may be able to extrapolate a craniosacral model (1) as to why the central nervous system is jeopardized. EDS causes laxity in the dural membrane reducing structural integrity of the falx cerebri, falx cereblli, tentorium, cauda equinae, filum terminale, cranial nerves, cerebrum, cerebellum, spinal cord, brain stem, ventricular and intracranial vascular systems, nerve roots, cerebral spinal fluid circulation, and general dural tensegrity. Several conditions may develop due to a loss of dural integrity: Chiari 1 Malformation, dural ectasia, cerebral spinal fluid leaks, tentorium slumping, intracranial hypertension, nerve impingement, and venous and vascular drainage issues.
Depression and anxiety can be primary results of autonomic nervous system dysregulation, rather than secondary to the burden of the disorder (13). As expressed previously, the autonomic nervous system regulates and controls appropriate response and function of the parasympathetic and sympathetic branches of the ANS. Interference of proper signaling between the sensory and motor neurons of the ANS may contribute to a heightened state of sympathetic activity which may trigger a flight or fight response, often interpreted as a panic attack. The result for the patient is very often a misdiagnosis of an anxiety disorder.
The person with EDS is often erroneously labeled as depressed at a young age and prescribed a host of medications which are ineffective. In prepubescent children, Ehlers-Danlos symptoms may originally manifest as panic attacks, behavioral outbursts (due to sympathetic nervous system hyperactivity), poor proprioception, reduced muscle tone, early repetitive injuries and joint pain. Although hypermobility may be present, it is frequently overlooked. The adult with EDS is often prescribed opioid based medications and are at risk to be flagged as “drug seekers” or addicts as their pain increases despite pain medication, or perhaps, because of it. Alternative and Complementary medicine is cost prohibitive for most people with EDS, despite the benefits.
People with EDS are tired. Tired physically, emotionally, and spiritually. They are depleted in every aspect of their physical and emotional self. They are faced with the knowledge that there is no cure or effective treatment for their condition. Hopes and dreams of having a normal life with normal activities are dashed and they frequently lose the ability to do activities that they once enjoyed. Their calendars are filled with doctor’s appointments and medical tests. The young EDS patient faces the disheartening knowledge that they will likely live in pain for their entire lives and activities that their friends can do will not be possible for them to do. Fatigue and pain will control the activities of the day which will be modified to sustain energy for the next day… and the next. The person with Ehlers-Danlos has a very, very difficult life. For some, they cannot withstand the pain and uncertainty of their situation and end their lives prematurely. For others, they hold their heads high and bravely face their pain and fatigue every day.
Eloise Stager is the founder and current leader for the CT Zebras Ehlers-Danlos Support Group. She is a member of the American Massage Therapy Association, International Alliance of Healthcare Practitioners, is a Pain Ambassador with the US Pain Foundation.
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