What we do have in common in addition to daily muscle pulling, stretching, tearing, and pains is included below. Not everyone will have the same ones but this is a general list.
Joints: • Different types of EDS have differing degrees of joint problems. Joint dislocation and incomplete dislocation called subluxation is common and recurrent. • Spontaneous easy reduction or replacement of the finger digits and shoulders occurs. • Hypermobile joints cause pain, and sometimes the "cracking" or "popping" of them feels like it relieves the pressure. • ‘Pes planus’ or being flatfooted is common and feet can flatten even more as one ages. • EDSers can develop osteoarthritis earlier than typical, and they often have difficulty or pain walking. They can appear klutzy. • Some EDSers’ hands collapse from the pressure of a simple handshake. It is difficult to write, and often finger splints help a great deal. • Cervical (neck) instability occurs in some types, and some people may have trouble holding up their head. • Another frequent joint problem is fluid effusions into the knees, ankles and elbows, primarily in Classical or Kyphoscoliosis types. (effusion: The escape of fluid from the blood vessels or lymphatics into the tissues or a cavity) • In the Kyphoscoliosis type, many infants have severe muscle hypotonia (floppy babies), generalized joint laxity and scoliosis at birth, or develop a progressive scoliosis (a curvature of the spine) within their first year of life. • With Vascular EDS, joint hypermobility may be limited to the small joints of the feet and hands or may be very lax all over. As with other types, VEDS patients often are first diagnosed as hypermobile, only later learning that they have VEDS!
Skin: General EDS skin traits include: • Easy bruising, delayed wound healing, differing types of scarring. • Thinner skin than normal, especially in the Vascular type. • Those with VEDS have translucent skin where the blood vessels below are clearly visible. • Soft, velvety skin that is fragile and sometimes highly elastic (stretchy) is found, especially in the Classical type. • Classical type EDS may experience wounds that split open with little bleeding and leave scars that widen over time to create characteristic shallow "cigarette paper" scars. • Surgical incisions may present problems with healing, with stitching EDS skin sometimes described as "like sewing butter." often requiring sutures being closer together and left in for a longer time than usual. • With severe CEDS, even just leaning on the table with your elbows can cause the skin to split or may have molluscoid pseudotumors on elbows and knees. (molluscoid pseudotumors are small, spongy tumors consisting of fat surrounded by a fibrous capsule found over scars and pressure points). • Skin that sags and wrinkles is characteristic of the Dermatosparaxis type of EDS. Extra (redundant) folds of skin may be present as affected children get older. • Skin hyperextensibility to some degree occurs in all types of EDS except Vascular.
Cardiovascular: • People with EDS tend to have low body temperatures, may have trouble controlling their body temperatures when exposed to heat or cold, and many have blood pressure problems. • Some have dilated aortic roots, incompetent heart valves, and autonomic dystonia or POTS (a syndrome where you have wide and serious blood pressure swings with position changes). • Many people with EDS bruise very easily and often severely. It can be difficult for a medical professional to "feel" their pulse. • Mitral valve prolapse is not a sign of EDS, though someone with EDS may have MVP; it is not diagnostic for this syndrome.
Neurological Symptoms: • Poor balance, severe headaches including migraines . • Decreased deep tendon reflexes. • Intracranial vascular abnormalities. • Brain "fog", a sense of not being present; absence of focus or a lack of clarity • Spinal stenosis (narrowing of spinal column) and/or scoliosis. • Chiari malformation (the brain tonsils protrude down through the forum at the base of the brain) occurs in some EDS patients.
Dental: • Half of all EDS patients have a hypermobile tongue, and are able to touch at least the end of their nose with it easily. • A high palate and crowded baby and adult teeth are common, even though many EDS patients have smaller than normal teeth. The high palate and smaller teeth can make fitting dentures very difficult even when explained to the dentist prior to the dentures being made. • Pre-molar and molar teeth often have high cusps and deep fissures with root problems, and enamel hypoplasia can cause decay and possible early extractions. Sometimes teeth actually crumble when losing the enamel. • Patients with Classical type offer suffer with juvenile periodontal disease. All EDSers are cavity prone, and have increased bleeding from anywhere in the oral cavity due to the fragility of tissues. Braces can cause problems as they can damage the gums and tongue while moving teeth quickly. • TMJ (temporomandibular joint) pain and clicking occur in about 30% of the general population, and about the same incidence occurs in EDSers. Often if in a dental chair with your mouth open for an extended period of time, the joint will repeatedly sublux. Taking a pillow so you can prop your hand up to support your jaw during the procedure will prevent it from happening and also reduce your pain level from TMJ. • Studies have proven that lidocaine (a local anesthetic used during dental procedures) often works poorly or not at all with EDS patients. • Some people with EDS complain of always feeling like there is a lump in their throat when swallowing, and often have other swallowing and voice problems.
GI system: Gastrointestinal complications of EDS run literally from one end to the other. Frequently EDSers suffer from reflux and GERD, stemming from an incompetent esophageal sphincter that allows stomach acid to backflow up the esophagus and cause burns in it. Diverticula have been seen throughout the G.I. tract. • Tissue extensibility and laxity can also cause lack of contraction of the stomach, causing food to not move down into the intestines. • Megacolon and rectal prolapse may also occur, primarily in childhood but megacolon is also seen in adults. (Megacolon is an abnormal dilatation of the colon (a part of the large intestines) that is not caused by mechanical obstruction. The dilatation is often accompanied by a paralysis of the peristaltic movements of the bowel.) • Irritable bowel syndrome is a common co-diagnosis. Constipation can result from the flaccidity of the large bowel, more water being pulled from the stool the longer it remains in the colon, and from pain medications.
Eyes: An EDSer may have many different eye problems depending on the type of Ehlers-Danlos they have including blue sclera, microcornea (very small cornea), epicanthic folds, and wide-spaced eyes. Other common problems are: • Many EDSers are photophobic, some squint causing an "angry" appearance and angiod streaks. • Loose tendons and ligaments around the eye create hard working muscles that get tired. Strabismus is the medical terms for eye conditions commonly called by these various names: eye turns, crossed eyes, cross-eyed, wall-eyes, wandering eyes, deviating eye. • Myopia (near-sightedness), astigmatism, and early presbyopia (a vision condition in which the crystalline lens of your eye loses its flexibility, making it difficult to focus on close objects.) occur often in EDS patients. • Dry eyes are a common and uncomfortable problem. • Other EDS related problems are detached retinas and ectopia (displaced) lenses. • Persons with Ehlers-Danlos syndrome should see an Ophthalmologist annually so the internal eye can be checked for retinal and lens problems among other things. This is not an O.D, a Doctor of Optometry, but an MD with a specialty in eye issues.
Pain: Pain with Ehlers-Danlos syndrome can range from none to chronic debilitating pain. It is subjective, individual, and different for each of us. For many patients, this is the worst symptom of all! Causes of this pain can be repeated trauma of constant instability from recent subluxations and dislocations as well as degenerative joint disease. Sometimes poor posture brought on by lax ligaments and weak abdominal muscles cause increased pressure on the spinal joints. Some with EDS do not have pain; others develop it later in life, and others begin to suffer severe pain as children. • Many things are useful in treating EDS pain such as heated pools (92-94 degrees), gentle stretching, walking (if your joints allow), and emotional support that recognizes the degree of your pain and is non-judgmental. • Occupational Therapists who make splints and assess what you may need may help to make daily life easier. Heat and cold packs help a lot. Always use cold for the first 24 hours after an injury to decrease swelling and limit bleeding into the area, and then switch to heat. • Other possibly helpful things are yoga, relaxation therapy, massage, acupuncture or acupressure, diversion, TENS units and chiropractic maneuvers by a knowledgeable chiropractor. • Common pain management problems are related to medications either in a too low a dose or prescribing the wrong medication, overemphasizing risks, using a "cookbook" approach, patients refusing helpful medications because they worry about addiction, and doctors afraid of prescribing because of their misunderstanding of the DEA laws. • Often pain is undertreated in children, the elderly, and minorities. Less than 2% of all chronic pain patients (not just EDS patients) using pain medications correctly for pain become addicted. One can become dependent, but can be easily weaned off narcotics in a short amount of time. • Medications often used with EDS are: muscle relaxants, NSAIDS, steroids, lidocaine patches, antidepressants, narcotic and nonnarcotic pain medications. Remember that over 4,000 mg. of Tylenol daily causes liver damage. Different combinations of medications work for each individual. • Pain can be completely debilitating and keep you from needed sleep. Often family and friends don't believe you ... the worst part of all.
Emotional Effects of EDS: Should either physical disability or chronic debilitating pain make your life feel destroyed, feelings of worthlessness and profound depression may set in; often talking to a counselor or medical professional will help. Regrettably, a tragedy occurs when we not only have to contend with no longer being able to do the things that we have loved doing, but also has to battle for family and friends' belief, respect and understanding. It appears that everyone with an invisible disability sadly experiences this. While someone with EDS is mourning their loss of ability and freedom, others often accuse them of just being lazy, malingering, or becoming an addict to the pain medications that allow them to live their life. Because of this, we should not confuse their endeavors to live life and be positive with assuming they are feeling well or doing better. Knowing you have EDS doesn't suddenly make things worse for you physically, but may allow for better physical management, and ideally allow for the prevention of any real problems, even if none exist currently. So knowing you have it is not necessarily a bad thing. Personal doubt about one's mental and physical abilities can add to the fear that others can't possibly believe or understand what you're going through. Inability to cope with daily tasks or mental confusion can have a demoralizing effect.
Reference Citation Crediting to EDNF
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