The Ehlers-Danlos Syndromes (EDS) - Connecting the Dots!

“At this point in time, I put EDS in the category of being in the top three or four most severe pain problems. A lot of people for example think that cancer pain is the worst pain, but let me assure you that many EDS patients have pain far beyond any cancer patient I've ever seen. And so it's one of the pain problems that is severe, has been very troublesome, many physicians are afraid of the disease and of the kind of pain that EDS patients have.”
– Dr. Forest Tennant

Understanding Connective Tissues:

Connective tissue provides strength and flexibility to structures such as bones, ligaments, muscles, organs, and blood vessels and they typically involve a problem with one or more of the proteins. EDS is the most prevalent connective tissue disorder amongst Chiarians and it involves a mutation in our bodies collagen. There are over 200 identified Heritable Disorders of Connective Tissue (HDCTs),[2] conditions such as Marfan Syndrome, Loeys-Dietz Syndrome, Stickler Syndrome, Osteogenesis Imperfecta, Pseudoxanthoma Elasticum, and Epidermolysis bullosa - all involve one of the many proteins that make up our bodies connective tissues. Ehlers-Danlos Syndrome(s) involve a genetic mutation of one or more of our collagen proteins, at a cellular level. There are currently thirteen known subtypes, each involving a different mutation and varying degrees of symptoms, but all symptoms can cross over from one subtype to the next.

Collagen Makes Up 30-40% of the Proteins in the Human Body[3]...

Collagen is often likened to the glue that holds us together. Just as our skin has collagen and as we age we see the effects with sagging eyelids, cheeks and neck areas; our brains have collagen which helps them to keep their form. Your spine is a mass collection of vertebrae bound together by connective tissue and the protein that binds each disc together is collagen. Imagine, if you will, the difference between something being bonded together with Gorilla Glue versus watered down Elmer’s Glue: the creation using Elmer’s Glue wouldn’t be as strong. The consistency of Elmer’s Glue could easily cause anything being held together by it to lose its form and prolapse/herniate.

Understanding the Chiari/Ehlers-Danlos Connection:

The same theory can be used to explain why many of us with Ehlers-Danlos acquire what ends up diagnosed as a Chiari Malformation. EDS is known for causing organ prolapses and your cerebellum really is just a complex organ.[4] Therefore, it is a matter of concern that so many surgeons find herniated cerebellar tonsils, diagnose it as a Chiari Malformation, assume it to be congenital (since most of the literature simply says that an Acquired Chiari is rare without ever defining what would constitute an Acquired Chiari Malformation) and offer relief from decompression surgery, without checking for any pathological conditions that might be causing this hernia of the cerebellar tonsils. Even if a posterior fossa hypoplasia is confirmed, it is irresponsible not to give adequate attention to all the other possibilities when decompression surgery will forever change the patient's cranial anatomy, opening the door to a host of additional problems. And if unaddressed, those pathologies that caused the prolapse in the first place will still be present after decompression, with a newly enlarged foramen magnum to fall further into.

You Might Have EDS if You or a Family Member Have/Have Had...

Adrenal disorders
Allergies/sensitivities to foods (chronic and/or acute)
Allergies/sensitivities to medications/drugs (chronic and/or acute)
Allergies/sensitivities to topicals/metals (chronic and/or acute)
Amblyopia (lazy eye)
Ankylosing spondylitis
Antimongoloid slant
Aquagenic urticaria
Arachnodactyly (long slender fingers)
Arterial rupture
Arthralgia (joint pain)
Atlantoaxial Instability (AAI)
Atrophic/papyraceous scarring
Autoimmune Disorders
Baker’s cysts
Basilar invagination (BI)
Bifid uvula (split tongue)
Blepharitis (reoccurring)
Blue sclerae
Bowel incontinence
Bowing of the legs
Brachial Outlet Syndrome
Breast ptosis (abnormal)
Carotid-cavernous sinus fistula (CCSF)
Carpel Tunnel
Carpo-tarsal fusions
Celiac disease
Chiari Malformation
Chronic diarrhea
Chronic dizziness
Chronic Muscle fasciculations (contractions)
Chronic muscle spasms/spasticity
Chronic nausea
Chronic vomiting
Cleft palate
Cortisol disorders
Cranial settling
Craniocervical Instability (CCI)
Cryptorchidism (undescended testes)
CSF Leaks (both cranial and spinal)
CSF otorrhea
CSF rhinorrhea
Cushing's syndrome
Cutis laxa (loose and inelastic skin)
Degenerative Disc Disease
Delayed cranial ossification
Deviated septum (misaligned nasal cartilage)
Diabetes insipidus
Diastasis Recti
Dysphagia (high/oropharyngeal/structural)
Dysphagia (low/esophageal/obstructive)
Dysphagia (trouble swallowing)
Easy bruisability
Electrolyte imbalances (especially low potassium)
Elongated ulnar styloid process
Empty Sella Syndrome
Epicanthus (eye folds)
Epilepsy (seizure disorders)
Esophageal spasms
Fibromuscular dysplasia (FMD)
Flat foot
Frequent joint dislocations
Frequent joint subluxations
Frozen shoulder
Ganglion cysts
Gastroesophageal reflux disease (GERD)
Gastrointestinal problems
Golfer's elbow
High arched feet
Hip dysplasia
Hoarse voice (vocal cord dysfunction)
Hormonal problems
Hyperdontia (supernumerary teeth)
Idiopathic Intracranial Hypertension (IIH)
Intracranial Hypertension (IH)
Irritable Bowel Syndrome (IBS)
Kyphosis (exaggerated or inappropriate)
Large fontanels
Leaky gut syndrome
Livedo Reticularis
Lordosis (exaggerated or inappropriate)
Malabsorption problems (especially with vitamin B12)
Marfanoid habitus
Menorrhagia (heavy menstrual bleeding)
Micrognathia (undersized jaw)
Mitochondrial disease/disorders (MITO)
Mitral Valve Prolapse (MVP)
Multidirectional Instability of the shoulder (MDI)
Muscle atrophy
Muscle hypotonia
Myofascial pain
Myopia (nearsightedness)
Neurally Mediated Hypotension (NMH)
Neurogenic bladder
Organ Prolapse
Osteopenia (fragile bones from low bone mineral density)
Palmar wrinkling
Palmoplantar keratoderma
Pelvic floor dysfunction
Periodontal Disease (receding gums)
Peripartum perineum tears
Pes planus (fallen arches)
Placenta Previa
Plantar Fasciitis
Polycystic Ovary Syndrome (PCOS)
Pompholyx (dyshidrotic eczema)
Postural Orthostatic Tachycardia Syndrome (POTS)
Premature hair loss
Proprioception Problems
Pseudotumor Cerebri (now called Intracranial Hypertension)
Ptosis (droopy eyelid)
Radioulnar synostosis
Raynaud’s phenomenon
Rectal prolapse
Restless Leg Syndrome (RLS)
Retinal detachments/tears/rips
Retroflexed odontoid (RO)
Rheumatoid Arthritis (RA)
Sensory Intregration Disorder
Sensory Processing Disorder
Short stature (3rd-5th percentile)
Skin hyperextensibility (stretchy)
Skin striae (stripes)
Skin tears/rips
Sleep apnea (both obstructive and central)
Soft tissue rheumatism
Sphincter problems
Spontaneous sigmoid colon perforation
Strabismus (crossed eyes)
Striae (excessive or inappropriate stretch marks)
Swimmer's shoulder
Syncope (fainting)
Syringobulbia (syrinx/cyst in the brainstem)
Syringomyelia (syrinx/cyst in the spinal cord)
Talipes equinovarus
Talipes equinovarus (club foot)
Tarlov cysts
Temporomandibular Joint Disorders (TMJ)
Tendonitis (of every kind)
Tennis elbow
Tethered cord
Thin/transluscent skin
Thoracic outlet syndrome
Tonsillar ectopia
Triphalangeal thumb
Urinary incontinence
Urticaria (hives)
Urticaria Pigmentosa
Uterine prolapse
Uterine rupture
Valvular problems
Varicose/spider veins
Von Willebrand Disease (VWD)

The Diagnosis Criteria

There are many fears associated with a diagnosis of EDS, for patients and doctors alike. Some of these fears are warranted as there are life-threatening complications associated with it. However, the failure to be properly diagnosed does not stop one from having these complications, it only serves to increase the time it takes for doctors to understand the depth and severity of the complications when they happen (and that loss of time can be costly).