with the neck and shoulder pain. It's not gotten better at all. Last week the physiatrist gave me a taper pack of prednisone. Did absolutely nothing. Nil. Nada. Zippo.
I spent the wkend at my parents trying to not move b/c when I move it hurt more. I tried the shoulder sling but couldn't really tell if it made much of a difference or not.
Monday I rang the physiatrist and made an appt for next Tues, the 16th. It was the first available. Tuesday I was still dying so I rang back and got his nurse - yay a live person. She was very understanding and helpful and said she would talk to him and get back to me. She did in a couple of hours and asked about the pain, if it was the same as back in Dec. I said no it's worse, so she said the dr wants me to have another MRI of my neck. I asked if we should do my shoulder but she said he's pretty sure the pain is originating from the spine, so that's what we'll do. It's scheduled that for this Saturday. In the meantime he gave me Valium to take so I can sleep. I took one last night at 21.00 and ended up waking up at 5 this morning. When I got up to take a shower I was loopy and walking into doors and dizzy and thought I'd fall down in the shower. How can people enjoy this feeling? If it at least got rid of the pain, then I might not mind as much. Anyway I hope the MRI results are quick and that there is something the dr can do on Tuesday to get rid of this major pain because I can't take much more of it, mentally, emotionally or physically. Once we (hopefully) sort the pain out then we have to figure out what to do next. It's one thing having stenosis, but it's another when you have HEDS thrown into the mix. None of the normal options seem available.
Today I have to do the prep for the colonoscopy tmrw. I am pretty nervous since just sitting kills my neck and back and I know I'll spend most of the evening in the loo. Oh well, at least I'll get it over with. I have to say though reading about this HalfLytely stuff does put one off, it sounds so gross. I had to do this once before but it was about 15 yrs ago.
Showing posts with label Ehlers-Danlos. Show all posts
Showing posts with label Ehlers-Danlos. Show all posts
11 June, 2009
03 June, 2009
Nothing changes
Unfortunately I was in agony all weekend. I was back at the chiro yesterday and today. Yesterday he suggested contacting my physiatrist because even though we've loosened up the muscles, the pain has not gotten any better. I was able to speak to Dr. Panagos today and he put be on Prednisone for a few days. You take 6 the first day, then 5, then 4, etc. I drove the pharmacist lady nuts because I kept ringing to see if the script had been called in. Finally it was about 15.30 so I went across the street and picked it up. I took 6 pills around 16.00 and so far nothing. I read online sometimes it can work v. fast, which is what my dr said, but he also said it could take days. I don't have days! I am so worn out from no sleep and all this pain that I just want to scream (I've been doing the almost daily crying meltdowns already).
I asked him about an injection like I had in my hips, and he said something similiar could be done but since it's the spine it would be in at least two joints, as opposed to the one. He said if the Prednisone doesn't work (G-d forbid!!!) then that would be the next step.
Please please please let this work and quickly. I am truly at my wits end!
I asked him about an injection like I had in my hips, and he said something similiar could be done but since it's the spine it would be in at least two joints, as opposed to the one. He said if the Prednisone doesn't work (G-d forbid!!!) then that would be the next step.
Please please please let this work and quickly. I am truly at my wits end!
03 April, 2009
So You Think You Might Have EDS?
After reading this I was amazed how many things add up. Before my diagnosis I was made to think I just had tons of random things wrong with me, but when I look at this, it all fits together like a puzzle. But if you do have many of these, or even some, you must get diagnosed by a genetic doctor. If you need a referral for one in NYC I have a great one.
By Sue Jenkins RN with contributions from Liza Sauls (from www.ednf.org)
It has been the experience of many of our members that a final diagnosis of EDS is the result of 'connecting the dots' of a person's history and a comprehensive review of the constellation of all symptoms; and includes, for many, thorough review of the medical histories of their families as well. Finally getting the diagnosis can be a relief to know that the symptoms are real and have a name; however limitations occur here as well. There is no cure, no 'fix', simply because the collection of experiences and symptoms now has a name and identity. But it does allow the patient and their families to know what they may be facing and allow them to become educated and proactive about the care they seek and require.
Based on the accumulated experiences of our members, the following essay is an overview of many of the symptoms and conditions that can be associated with EDS. It is not meant as a substitute for thorough medical review and care and supervision, but to help to view some aspects of EDS. Not every patient will have every symptom, as you will see, EDS can manifest in many different ways.
The challenge of EDS is not to be able to identify each symptom, but to be able to see a pattern among several.Early diagnosis and intervention are the keys to living the best life possible. With the recent TV programs on Ehlers-Danlos Syndrome, many people are coming to this website with questions about having EDS. Here is a list to help you determine if you need to see a geneticist who can correctly diagnose you.
Caution: If after reading the following article, you think you may have EDS, be sure to seek medical advice.
Please do not rely solely on this article or end your search with a self- diagnosis. EDS is a genetic syndrome and is diagnosed by a specialist, usually a geneticist or rheumatologist with extensive knowledge about EDS and its types:
• Classical
• Hypermobile
• Vascular
• Kyphoscoliosis
• Arthrochalasia
• Dermatosparaxis
Ehlers-Danlos Syndrome is a group of disorders that affect connective tissues, which are tissues that support the skin, bones, blood vessels, eyes and other organs. Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos Syndrome, which vary from mildly loose joints to life-threatening complications related to tissue structure and fragility.
A physical examination is required, including taking an extensive family history and using the Beighton scale, which measures Hypermobility. Depending on which type of EDS the physician believes you have, eithera blood test or muscle biopsy will be taken.
How You Get It:
EDS is a genetic disorder, a mutation of one or several genes that make different types of collagen in your body, producing a defective tissue. A mutation in a gene on one of the first 22 nonsex chromosomes can lead to an autosomal disorder. Genes come in pairs.
Some types of EDS are autosomal dominant and others are autosomal recessive. Differing types of EDS affect different types of collagen.
• If a disease is autosomal dominant, it means you only need to get the abnormal gene from one parent in order for you to inherit the disease. One of the parents may often have the disease. Each child has a 50/50 chance of inheriting this disease.
• Recessive inheritance means both genes in a pair must be defective to cause disease. People with only one defective gene in the pair are considered carriers. However, they can pass the abnormal gene to their children.
Wrong Diagnoses:
Most people diagnosed with EDS have come the same long road where it seemed that nobody knew what was really wrong with you. Diagnoses of
• osteoarthritis
• fibromyalgia
• lupus
• rheumatoid arthritis
• rheumatic fever
• multiple sclerosis
• “growing pains”
• and “it’s all in your head” are just some of them
Often people get several misdiagnoses before finally being correctly diagnosed with EDS.
Symptoms of EDS:
Although all types of Ehlers-Danlos syndrome affect the joints and many also affect the skin, features vary by type and severity. An unusually large range of joint movement, hypermobility, occurs with most forms of Ehlers-Danlos syndrome, particularly the HEDS (hypermobility) and CEDS (Classical) types.
Below is a listing of symptoms that persons with EDS often have. These symptoms are broken down according to body systems. This list is not all-inclusive, but include those most frequently encountered.
Not everyone with EDS has them all and if you have some of them you may still not have Ehlers-Danlos Syndrome.
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!
The range of hypermobility differs greatly among EDSers, even within types. The loose joints throughout life are unstable, prone to subluxation and dislocation, cause chronic pain and early-onset arthritis. Some people are only mildly affected by their EDS; others are completely debilitated.
Orthopedic procedures to stabilize or improve the joint's function sometimes put more than expected strain and stress on adjacent joints, leaving many EDSrs disappointed with the results.
So your back, hips, shoulders, knees, elbows, and other joints go out more often than you do, you might have EDS.
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.
So your skin has so many bruises people think you earn your living as a boxer, you might have EDS.
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.
In some types, arteries including the aortas are very fragile and can rupture causing a medical emergency.
Note: IV (intravenous) access and even sometimes simply drawing blood for testing may require multiple attempts; using a "butterfly" needle and syringe is much more successful than the use of a vaccutainer which draws the blood rapidly by the use of suction. People with this concern must use extreme care and inform their healthcare providers of these possibilities.
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.
So you had a school report card that said you were fidgety, uncoordinated, lazy, under-developed, and a complainer, you might have EDS.
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 (tempomandibular 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.
So a dentist ever gave you so much Novocain that his thumb was numb, and you could still feel everything, you might have EDS!
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.
So your favorite foods are your digestive system’s LEAST favorite foods, you might have EDS.
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.
Even during an eye exam, the exam itself can cause vertigo, nausea and headache feeling much like carsickness in some people.
* So you change your eyeglass prescription more often than your wardrobe, you might have EDS!
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.
So you have days when you need a nap to rest up from the effort of getting out of bed in the morning, you might have EDS.
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.
But there is hope and help. You can join EDNF and learn how to help yourself, your doctor and your future. You are not alone.
So you are searching for knowledge, learning about EDS and educating others, you might have EDS!
16 March, 2009
Physical Therapy Management of the Hypermobile Patient
Terry Olson, PT, MHS, FAAOMPT, received his physical therapy training at the University of Iowa and he has an interest in the manual treatment of the muscular skeletal problems. He subsequently received his Masters Degree in orthopedic physical therapy and he developed a post graduate program in exercise and manipulative therapy. Mr. Olson is a fellow of the American Academy of the Orthopedic Manual Therapists and we would like you to welcome Mr. Terry Olson.
I am going to present this from the standpoint of looking at hypermobility. We are going to look at a definition of hypermobility, its relationship to EDS, then talk about the role of exercise and joint protection, and a short case study that I thought I would present.
Okay, what is hypermobility? There are quite a few definitions, but the one that seemed to be the simplest was that it is a connective tissue disease, where connective tissue proteins, such as collagen, which give the bodies its intrinsic toughness, are differently formed; the results are mainly felt in the moving parts, that being the muscles, joints, tendons, ligaments, which now are more fragile than is the case for most people. The result is joint laxity with hypermobility and with it comes vulnerability to the effects of injury. The young girl presented here is the one on which I did the case presentation.
Quick review of the categories.
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Classical: Looking at skin hyperextensibility, tissue fragility and joint hypermobility.
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Hypermobility: again, joint hypermobility is the dominant characteristic, may have joint subluxation and dislocation, limb and joint pain.
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Kyphoscoliosis: generalized joint laxity and severe muscle hypotonia, scoliosis, tissue and organ fragility.
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Arthrochalasia: congenital hip dislocation, severe generalized joint hypermobility, recurrent subluxations, tissue fragility, and muscle hypotonia.
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Vascular: organ fragility with the possibility of arterial or organ rupture, tendon or muscle rupture, joint hypermobility primarily in the digits.
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Dermatosparaxis: severe skin rigidity, skin soft and doughy and may have large hernias.
Of all of those categories, the dominant feature from a physical therapy perspective is hypermobility and joint laxity, so those are the primary considerations for a therapist when treating patients with EDS; and that means that treatment needs to focus on joint protection and dynamic stabilization of those joint structures.
Muscle stiffness is a term that is used to describe the spring-like quality of muscle. When a muscle has high stiffness, increased force is required to cause lengthening of that muscle or to perturb it. Muscle stiffness has been described in the biomechanical and neurophysiological literature as one of the most crucial variables in joint stabilization; and in the knee a link has been established between receptors in the ligaments of the joint and muscle stiffness. The slide includes a few of the references for that.
In the residency process (I went through post-graduate residency training in manipulative and manual therapy) you are primarily moving joints that are stiff. Switching to talking about joints that are not stiff, which is the case here, takes a bit of a shift. One of the things taught in the residency process was that you cannot distinguish a problem as being a joint problem or a muscle problem. The structures are so closely interrelated that the feedback between the two is critical to the integrity of that joint. If you have problems within the joint; because the joint provides feedback to the muscles, you are going to have muscular weakness. If you have muscle weakness, again through association with the joint structure, you are going to have impact on the stability of the joint. Thus, any treatment needs to be directed at all of those structures.
It is possible that the sensory properties of structures within joints can be modified by the contraction of the local, stabilizing muscles, and this is usually the proximal muscle groups. If you are looking at the shoulder, you are talking about the scapular cervico-thoracic muscles and the large shoulder muscles themselves. If you are talking about ankles and knees, you are looking at the muscles in the trunk and the pelvic girdle.
Besides providing mechanical stability to the joints, these muscles can contribute to the sensory feedback mechanisms associated in the joint structures themselves. A study done by Blazier, Carpenter, and Houston showed that tightening of the joint structures with active muscle contraction increases the acuity of that shoulder joint. That means if the muscles are functioning properly, then they are going to provide feedback to the joint which, in turn, makes a more stable situation for that joint and the joint has the ability to have feedback as to where it should be positioned.
Dynamic stabilization, or the use of exercise to promote joint stabilization, occurs when tonic (postural and slow twitch muscle units), are activated. These tonic motor units are activated during tonic continuous low load activation of muscle, maximizing muscle stiffness. What we are talking about here is asking the muscle to contract gently, not maximally, and to be able to sustain that contraction for a period of time. This can be influenced by the speed of the activity or exercise, as well as the type of muscle contraction you are trying to acquire. Muscle contractions that are performed in the shortened range of the muscle length are going to be critical in establishing the sensitivity and optimal functional capacity of the sensory feedback system in the muscle.
All that says is that when you are exercising - for folks that are afraid to exercise, for folks that have an inherent increase in mobility - exercise needs to be done in a very small controlled range of motion. Dr. Unger and I have had discussions about patients that we might see commonly as well as the patients I have seen from Dr. Mark LaVallee. One of the things that I have seen somewhat consistently is that the patients who are stretching don't need to stretch. They will tell me about being stiff and they will take their leg and wrap it around their head and it is just difficult to relate to that as being stiff. Now you can have a feeling of stiffness, if the muscles are fatigued or overworked, which to me then relates back to weakness.
Co-contraction and co-activation of muscle groups provides the biomechanical forces for joint stabilizing and protection, especially if they are performed in mid-range or neutral joint positions. Every joint, in its ability to move through range, is going to have positions that are inherently more stable because you have joint surfaces that have better contact with each other. You also have a point in that range where the muscle has mechanical advantage. If I am bending my elbow, the biceps will do that. When the bicep is in its lengthened range (elbow fully straight), I am going to be much weaker than when I get to a point (elbow fully bent) when things are going to shorten or come closer together. Mechanically this is much stronger. And, so, when you exercise EDS patients, you need to make sure you attend to placing them in those mechanically advantaged positions to work and exercise.
This is not just with EDS, but with many of the patients I see that have weakness or hypermobility. They do not exercise their full range of motion, they do not have the control to do so, and building the ability to exercise to the greater range is the goal. So, you start where the patient can perform in a pain-free and stable manner. Closed chain exercise is superior for muscle protection of the joint, although open chain exercise is also beneficial and necessary, especially again if it is performed in the protected range of motion.
When we are talking about open chain exercise/closed chain exercise we mean the following. Open Chain Exercise: if I want to exercise my quadriceps, that is the muscle that straightens my knee out, that is open chain, just moving my leg through range of motion. Closed Chain Exercise: if I stand here and do a squat, I am still working that quadriceps muscle, but by virtue of contact with the floor, we now have other muscles that act to provide increased stability. So a lot of times, starting these patients with exercises that are more closed chain, enhances that ability to stabilize muscles around the joint.
Case presentation
We take a body chart that the patient marks when they come in - and again, typically, when I get a body chart on a patient from Dr. LaVallee, that whole body chart may be colored in - it looks like they have a suit on.
As a therapist, where do you start with that - you cannot treat everything. In this case here, the young girl, the primary complaint was the mid lower back, and the other complaints were part of her overall problem; but in this case not the primary reason for presenting to therapy. Primarily, it was because of back and hip trouble. She had a diagnosis of lumbar back pain, left hip pain, and EDS multiple areas of pain complaint and again those were most notable in back and hip. She described pain complaints of an 8 out of 0-10, (0 being no pain, 10 being like a wild animal tearing your leg off).
She described her pain at her worst as being an 8 on that scale with standing for more than one hour being a provocative activity, as well as her activities of daily living. One of the things that were important at that time was that she had a baby, and that holding her baby was enough to provoke a lot of these pain complaints. She had objective signs of multiple joint hypermobility with back pain reproduction.
In her case, initial emphasis was on symptom alleviation using modalities (heat and cold, TENS units, electrical stimuli), gentle joint mobilizationand biomechanical correction, as well as performing assisted exercise in protected asymptomatic range of motion. We also provided biomechanic counseling on joint protection, as well as back care education regarding lifting, sitting, and performance of her activities of daily living.
Over the course of treatment, as her symptoms decreased, we progressed into more dynamic exercise, dynamic stabilization, and that become the crux of her home program.
One of the things that we can use in the setting that I work in, at least in the acute phases, is equipment designed to start exercising folks at less than full limb loading. If you have someone that has an injury or problems with - as in this case - knee and hip, and those problems occur when they are weight bearing, then the idea is that exercise has value because if we exercise, we have the ability to increase blood flow and circulation, increase oxygen, all of the things that are essential for healing, as well as building blocks for laying down stronger tissue. The problem is bringing that load to an acceptable level for that person.
So, in this case, this patient is just doing a bilateral squat activity and the slide board allows her/him to exercise anywhere from less than 20% of body weight taking it all the way up to 70% of body weight and then we know how to graduate that into a gym exercise. We try to exercise people as functional as we can, so if people have complaints of “when I stand too long or when I walk too long, when I bend is when I have my complaints,� I am going to try to set my exercise program up so that they are standing, walking, bending, but using the equipment to again decrease gravity, or their body weight, so that they are exercising with less body weight on those joints.
In this case, with walking, we use a treadmill system that has an overhead pneumatic assist and that harness allows us to hoist someone up. We can take that person, take as much as 70% of body weight (essentially the weight of the trunk - you go any higher than that and you are like Peter Pan and you are unstable, and you are ready to fly) then you are still getting input into the joints, but not full load.
I had a discussion with one of the participants who asked me about Pilates - I am going to beat people to the punch - I don't know. Pilates makes sense to me that it can be of value, but I don't know enough about it and I might make an idiot of myself trying to tell you the value of it.
Not for purpose of this talk, but if it gives any rationale to why; the approach that we use is a system called "medical exercise training". That approach is based on specific tissue exercise, and so we work from a standpoint that muscle requires a different muscle stimulus than does tendon, than does cartilage, than does disc. All of those structures have a different metabolic requirement, so those exercises have to be different, and when you are working with folks who have tendonitis, we try to work out an exercise program that allows that person to go through anywhere from 150 to 200 repetitions - because that seems to be the adequate number of repetitions to get an adequate increased blood flow to the area to promote a healing response. When trying to address structures such as ligament, cartilage, disc, those structures require upwards of 1,000 to 1,500 reps. The problem is taking someone who hurts and tell them you are going to put them on an exercise program of 1,500 repetitions and then say I will come back in an hour and see how you are doing. So to do that, you need to take load off, and the purpose of the equipment is to assist that part so that person can perform repetitions without pain. And then as the structure begins to heal, we gradually put load back on.
So this was the initial phase. In addition, we started to work on trunk. With Pilates or with other approaches, their value is core strengthening of the trunk - because your arms and legs attach to your trunk and if your trunk is not stable, then it is difficult to promote stability into those joints. Oftentimes the trunk is the most logical place to start, and is probably the easiest because everything else has to function off of it.
A simple leg raise will require activation of the transverse abdominal muscles if they are activated and used. The fact that her back is arched and she is unable to keep it from arching is indicative of the fact that a good exercise becomes a very poor one because she doesn't exhibit the ability to recruit enough trunk muscles to actually handle the weight. Here, she is able to keep the trunk stable (you don't see that hollow) and so she is performing that exercise actually really well with good trunk control.
Exercise is controlled trauma. When we exercise someone, we are inflicting trauma - the idea is to control it. If you exercise adequately, you are going to break down tissue, then let it rest and in this phase it recovers and lays down stronger tissue - if you adequately stress it. If you overstress it, then a good exercise becomes a bad exercise pretty quickly. All day long I see patients come in who are failures at exercise programs and it is not because exercise is not a benefit to them, they have just not been trained to do it with good form and to work in a range where they have control. You need to understand what is safe.
I had actually treated Erika about 9 months ago and she did well. I asked her to come back to see how she was doing but also to take some photos for this presentation and it was a good chance to take her upstairs and see if she was doing what we had instructed her to do 9 months ago. She demonstrates incredible form. Her elbows are going the wrong way and you would think that would make sense, but when we first went over her exercises and she did things, a lot of the things she was performing, a lot of work was in an unstable range and it was causing a lot of pain. It was just a matter of again making certain that her trunk was active and then worked in the range that she could exhibit control.
The exercise doesn't matter - when I set exercises for people, it doesn't matter, pushing up, pulling down, pushing away, pulling into them, I do a variety because life is made up of that. You need to be able to do all sorts of things but in a stable manner. The exercises need to be changed and modified as long as the patient understands stabilizing well, good trunk improvement, and that the joints are working in a range where they were stable.
I saw Erika for 9 visits over a 5 week period. Initial treatments consisted of gentle treatment to relieve symptoms coupled with assisted exercise utilizing the assisted treadmill walking and total gym. The patient then progressed to a stabilization and progressive strengthening exercise program. Her symptoms decreased, her discharge pain complaints were decreased, and she was able to stand and sit for more than two hours without symptoms, as well as lift her baby without increase in symptoms.
The final slide provides some excellent overall points in your therapy process. All of these things can be used in treatment and pain reduction. What I have seen with pain reduction, if you are heating people or icing them or electric stimuli, you are doing things that are temporary. Long lasting change is only going to come with making a change in the body, to the extent that you can. So, exercise with emphasis on controlled range of motion or working within a range of control. Pool therapy is beneficial and we use a pool at our facility also. Pool therapy takes the body weight off much the way the harness mentioned above does. Massage certainly has value, but again I think you need to be careful about monitoring skin integrity, especially if you are doing friction massage which is one of the therapy modalities often used in treating tendonitis. Use of splints or bracing - those are done supportively. Annual therapy, manipulative therapy, chiropractic therapy - it is not contraindicated, but you need to make sure you are addressing treatment to a joint that needs to be moved, not the joints that already move excessively. As a general rule, you want to be cautious of vigorous stretching secondary to inherent hypermobility. Again, I don't teach people to stretch, but they do need to strengthen because they have all of this range of motion that they don't have control of and that is painful. A lot of patient education, regarding ergonomics, joint protection, body mechanics, is required.
I am going to present this from the standpoint of looking at hypermobility. We are going to look at a definition of hypermobility, its relationship to EDS, then talk about the role of exercise and joint protection, and a short case study that I thought I would present.
Okay, what is hypermobility? There are quite a few definitions, but the one that seemed to be the simplest was that it is a connective tissue disease, where connective tissue proteins, such as collagen, which give the bodies its intrinsic toughness, are differently formed; the results are mainly felt in the moving parts, that being the muscles, joints, tendons, ligaments, which now are more fragile than is the case for most people. The result is joint laxity with hypermobility and with it comes vulnerability to the effects of injury. The young girl presented here is the one on which I did the case presentation.
Quick review of the categories.
*
Classical: Looking at skin hyperextensibility, tissue fragility and joint hypermobility.
*
Hypermobility: again, joint hypermobility is the dominant characteristic, may have joint subluxation and dislocation, limb and joint pain.
*
Kyphoscoliosis: generalized joint laxity and severe muscle hypotonia, scoliosis, tissue and organ fragility.
*
Arthrochalasia: congenital hip dislocation, severe generalized joint hypermobility, recurrent subluxations, tissue fragility, and muscle hypotonia.
*
Vascular: organ fragility with the possibility of arterial or organ rupture, tendon or muscle rupture, joint hypermobility primarily in the digits.
*
Dermatosparaxis: severe skin rigidity, skin soft and doughy and may have large hernias.
Of all of those categories, the dominant feature from a physical therapy perspective is hypermobility and joint laxity, so those are the primary considerations for a therapist when treating patients with EDS; and that means that treatment needs to focus on joint protection and dynamic stabilization of those joint structures.
Muscle stiffness is a term that is used to describe the spring-like quality of muscle. When a muscle has high stiffness, increased force is required to cause lengthening of that muscle or to perturb it. Muscle stiffness has been described in the biomechanical and neurophysiological literature as one of the most crucial variables in joint stabilization; and in the knee a link has been established between receptors in the ligaments of the joint and muscle stiffness. The slide includes a few of the references for that.
In the residency process (I went through post-graduate residency training in manipulative and manual therapy) you are primarily moving joints that are stiff. Switching to talking about joints that are not stiff, which is the case here, takes a bit of a shift. One of the things taught in the residency process was that you cannot distinguish a problem as being a joint problem or a muscle problem. The structures are so closely interrelated that the feedback between the two is critical to the integrity of that joint. If you have problems within the joint; because the joint provides feedback to the muscles, you are going to have muscular weakness. If you have muscle weakness, again through association with the joint structure, you are going to have impact on the stability of the joint. Thus, any treatment needs to be directed at all of those structures.
It is possible that the sensory properties of structures within joints can be modified by the contraction of the local, stabilizing muscles, and this is usually the proximal muscle groups. If you are looking at the shoulder, you are talking about the scapular cervico-thoracic muscles and the large shoulder muscles themselves. If you are talking about ankles and knees, you are looking at the muscles in the trunk and the pelvic girdle.
Besides providing mechanical stability to the joints, these muscles can contribute to the sensory feedback mechanisms associated in the joint structures themselves. A study done by Blazier, Carpenter, and Houston showed that tightening of the joint structures with active muscle contraction increases the acuity of that shoulder joint. That means if the muscles are functioning properly, then they are going to provide feedback to the joint which, in turn, makes a more stable situation for that joint and the joint has the ability to have feedback as to where it should be positioned.
Dynamic stabilization, or the use of exercise to promote joint stabilization, occurs when tonic (postural and slow twitch muscle units), are activated. These tonic motor units are activated during tonic continuous low load activation of muscle, maximizing muscle stiffness. What we are talking about here is asking the muscle to contract gently, not maximally, and to be able to sustain that contraction for a period of time. This can be influenced by the speed of the activity or exercise, as well as the type of muscle contraction you are trying to acquire. Muscle contractions that are performed in the shortened range of the muscle length are going to be critical in establishing the sensitivity and optimal functional capacity of the sensory feedback system in the muscle.
All that says is that when you are exercising - for folks that are afraid to exercise, for folks that have an inherent increase in mobility - exercise needs to be done in a very small controlled range of motion. Dr. Unger and I have had discussions about patients that we might see commonly as well as the patients I have seen from Dr. Mark LaVallee. One of the things that I have seen somewhat consistently is that the patients who are stretching don't need to stretch. They will tell me about being stiff and they will take their leg and wrap it around their head and it is just difficult to relate to that as being stiff. Now you can have a feeling of stiffness, if the muscles are fatigued or overworked, which to me then relates back to weakness.
Co-contraction and co-activation of muscle groups provides the biomechanical forces for joint stabilizing and protection, especially if they are performed in mid-range or neutral joint positions. Every joint, in its ability to move through range, is going to have positions that are inherently more stable because you have joint surfaces that have better contact with each other. You also have a point in that range where the muscle has mechanical advantage. If I am bending my elbow, the biceps will do that. When the bicep is in its lengthened range (elbow fully straight), I am going to be much weaker than when I get to a point (elbow fully bent) when things are going to shorten or come closer together. Mechanically this is much stronger. And, so, when you exercise EDS patients, you need to make sure you attend to placing them in those mechanically advantaged positions to work and exercise.
This is not just with EDS, but with many of the patients I see that have weakness or hypermobility. They do not exercise their full range of motion, they do not have the control to do so, and building the ability to exercise to the greater range is the goal. So, you start where the patient can perform in a pain-free and stable manner. Closed chain exercise is superior for muscle protection of the joint, although open chain exercise is also beneficial and necessary, especially again if it is performed in the protected range of motion.
When we are talking about open chain exercise/closed chain exercise we mean the following. Open Chain Exercise: if I want to exercise my quadriceps, that is the muscle that straightens my knee out, that is open chain, just moving my leg through range of motion. Closed Chain Exercise: if I stand here and do a squat, I am still working that quadriceps muscle, but by virtue of contact with the floor, we now have other muscles that act to provide increased stability. So a lot of times, starting these patients with exercises that are more closed chain, enhances that ability to stabilize muscles around the joint.
Case presentation
We take a body chart that the patient marks when they come in - and again, typically, when I get a body chart on a patient from Dr. LaVallee, that whole body chart may be colored in - it looks like they have a suit on.
As a therapist, where do you start with that - you cannot treat everything. In this case here, the young girl, the primary complaint was the mid lower back, and the other complaints were part of her overall problem; but in this case not the primary reason for presenting to therapy. Primarily, it was because of back and hip trouble. She had a diagnosis of lumbar back pain, left hip pain, and EDS multiple areas of pain complaint and again those were most notable in back and hip. She described pain complaints of an 8 out of 0-10, (0 being no pain, 10 being like a wild animal tearing your leg off).
She described her pain at her worst as being an 8 on that scale with standing for more than one hour being a provocative activity, as well as her activities of daily living. One of the things that were important at that time was that she had a baby, and that holding her baby was enough to provoke a lot of these pain complaints. She had objective signs of multiple joint hypermobility with back pain reproduction.
In her case, initial emphasis was on symptom alleviation using modalities (heat and cold, TENS units, electrical stimuli), gentle joint mobilizationand biomechanical correction, as well as performing assisted exercise in protected asymptomatic range of motion. We also provided biomechanic counseling on joint protection, as well as back care education regarding lifting, sitting, and performance of her activities of daily living.
Over the course of treatment, as her symptoms decreased, we progressed into more dynamic exercise, dynamic stabilization, and that become the crux of her home program.
One of the things that we can use in the setting that I work in, at least in the acute phases, is equipment designed to start exercising folks at less than full limb loading. If you have someone that has an injury or problems with - as in this case - knee and hip, and those problems occur when they are weight bearing, then the idea is that exercise has value because if we exercise, we have the ability to increase blood flow and circulation, increase oxygen, all of the things that are essential for healing, as well as building blocks for laying down stronger tissue. The problem is bringing that load to an acceptable level for that person.
So, in this case, this patient is just doing a bilateral squat activity and the slide board allows her/him to exercise anywhere from less than 20% of body weight taking it all the way up to 70% of body weight and then we know how to graduate that into a gym exercise. We try to exercise people as functional as we can, so if people have complaints of “when I stand too long or when I walk too long, when I bend is when I have my complaints,� I am going to try to set my exercise program up so that they are standing, walking, bending, but using the equipment to again decrease gravity, or their body weight, so that they are exercising with less body weight on those joints.
In this case, with walking, we use a treadmill system that has an overhead pneumatic assist and that harness allows us to hoist someone up. We can take that person, take as much as 70% of body weight (essentially the weight of the trunk - you go any higher than that and you are like Peter Pan and you are unstable, and you are ready to fly) then you are still getting input into the joints, but not full load.
I had a discussion with one of the participants who asked me about Pilates - I am going to beat people to the punch - I don't know. Pilates makes sense to me that it can be of value, but I don't know enough about it and I might make an idiot of myself trying to tell you the value of it.
Not for purpose of this talk, but if it gives any rationale to why; the approach that we use is a system called "medical exercise training". That approach is based on specific tissue exercise, and so we work from a standpoint that muscle requires a different muscle stimulus than does tendon, than does cartilage, than does disc. All of those structures have a different metabolic requirement, so those exercises have to be different, and when you are working with folks who have tendonitis, we try to work out an exercise program that allows that person to go through anywhere from 150 to 200 repetitions - because that seems to be the adequate number of repetitions to get an adequate increased blood flow to the area to promote a healing response. When trying to address structures such as ligament, cartilage, disc, those structures require upwards of 1,000 to 1,500 reps. The problem is taking someone who hurts and tell them you are going to put them on an exercise program of 1,500 repetitions and then say I will come back in an hour and see how you are doing. So to do that, you need to take load off, and the purpose of the equipment is to assist that part so that person can perform repetitions without pain. And then as the structure begins to heal, we gradually put load back on.
So this was the initial phase. In addition, we started to work on trunk. With Pilates or with other approaches, their value is core strengthening of the trunk - because your arms and legs attach to your trunk and if your trunk is not stable, then it is difficult to promote stability into those joints. Oftentimes the trunk is the most logical place to start, and is probably the easiest because everything else has to function off of it.
A simple leg raise will require activation of the transverse abdominal muscles if they are activated and used. The fact that her back is arched and she is unable to keep it from arching is indicative of the fact that a good exercise becomes a very poor one because she doesn't exhibit the ability to recruit enough trunk muscles to actually handle the weight. Here, she is able to keep the trunk stable (you don't see that hollow) and so she is performing that exercise actually really well with good trunk control.
Exercise is controlled trauma. When we exercise someone, we are inflicting trauma - the idea is to control it. If you exercise adequately, you are going to break down tissue, then let it rest and in this phase it recovers and lays down stronger tissue - if you adequately stress it. If you overstress it, then a good exercise becomes a bad exercise pretty quickly. All day long I see patients come in who are failures at exercise programs and it is not because exercise is not a benefit to them, they have just not been trained to do it with good form and to work in a range where they have control. You need to understand what is safe.
I had actually treated Erika about 9 months ago and she did well. I asked her to come back to see how she was doing but also to take some photos for this presentation and it was a good chance to take her upstairs and see if she was doing what we had instructed her to do 9 months ago. She demonstrates incredible form. Her elbows are going the wrong way and you would think that would make sense, but when we first went over her exercises and she did things, a lot of the things she was performing, a lot of work was in an unstable range and it was causing a lot of pain. It was just a matter of again making certain that her trunk was active and then worked in the range that she could exhibit control.
The exercise doesn't matter - when I set exercises for people, it doesn't matter, pushing up, pulling down, pushing away, pulling into them, I do a variety because life is made up of that. You need to be able to do all sorts of things but in a stable manner. The exercises need to be changed and modified as long as the patient understands stabilizing well, good trunk improvement, and that the joints are working in a range where they were stable.
I saw Erika for 9 visits over a 5 week period. Initial treatments consisted of gentle treatment to relieve symptoms coupled with assisted exercise utilizing the assisted treadmill walking and total gym. The patient then progressed to a stabilization and progressive strengthening exercise program. Her symptoms decreased, her discharge pain complaints were decreased, and she was able to stand and sit for more than two hours without symptoms, as well as lift her baby without increase in symptoms.
The final slide provides some excellent overall points in your therapy process. All of these things can be used in treatment and pain reduction. What I have seen with pain reduction, if you are heating people or icing them or electric stimuli, you are doing things that are temporary. Long lasting change is only going to come with making a change in the body, to the extent that you can. So, exercise with emphasis on controlled range of motion or working within a range of control. Pool therapy is beneficial and we use a pool at our facility also. Pool therapy takes the body weight off much the way the harness mentioned above does. Massage certainly has value, but again I think you need to be careful about monitoring skin integrity, especially if you are doing friction massage which is one of the therapy modalities often used in treating tendonitis. Use of splints or bracing - those are done supportively. Annual therapy, manipulative therapy, chiropractic therapy - it is not contraindicated, but you need to make sure you are addressing treatment to a joint that needs to be moved, not the joints that already move excessively. As a general rule, you want to be cautious of vigorous stretching secondary to inherent hypermobility. Again, I don't teach people to stretch, but they do need to strengthen because they have all of this range of motion that they don't have control of and that is painful. A lot of patient education, regarding ergonomics, joint protection, body mechanics, is required.
06 March, 2009
There are six major types of EDS. The different types of EDS are classified according to the signs and symptoms that are manifested. Each type of EDS is a distinct disorder that "runs true" in a family. This means that an individual with Vascular Type EDS will not have a child with Classical Type EDS. More detailed information can be found in our Medical Professionals Section.
General Frequently Asked Questions
Hypermobility (Formerly EDS Type III)
Joint hypermobility is the dominant clinical manifestation. Generalized joint hypermobility that affects large (elbows, knees)and small (fingers and toes) joints is evident in the Hypermobility Type. Recurring joint subluxations and dislocations are common occurrences. Certain joints, such as the shoulder, patella, and temporomandibular joint dislocate frequently. The skin involvement (hyperextensibility and/or smooth velvety skin) as well as bruising tendencies in the Hypermobility Type are present but variable in severity.
Chronic joint and limb pain is a common complaint amongst individuals with the Hypermobility Type. Skeletal X-rays are normal. Musculoskeletal pain is early onset, chronic and may be debilitating. The anatomical distribution is wide and tender points can sometimes be elicited.
To date, no distinctive biochemical collagen finding has been identified by researchers. The Hypermobility Type of EDS is inherited in an autosomal dominant manner.
General Frequently Asked Questions
Hypermobility (Formerly EDS Type III)
Joint hypermobility is the dominant clinical manifestation. Generalized joint hypermobility that affects large (elbows, knees)and small (fingers and toes) joints is evident in the Hypermobility Type. Recurring joint subluxations and dislocations are common occurrences. Certain joints, such as the shoulder, patella, and temporomandibular joint dislocate frequently. The skin involvement (hyperextensibility and/or smooth velvety skin) as well as bruising tendencies in the Hypermobility Type are present but variable in severity.
Chronic joint and limb pain is a common complaint amongst individuals with the Hypermobility Type. Skeletal X-rays are normal. Musculoskeletal pain is early onset, chronic and may be debilitating. The anatomical distribution is wide and tender points can sometimes be elicited.
To date, no distinctive biochemical collagen finding has been identified by researchers. The Hypermobility Type of EDS is inherited in an autosomal dominant manner.
(from EDS National Foundation site)
What is EDS?
Individuals with EDS have a defect in their connective tissue, the tissue that provides support to many body parts such as the skin, muscles and ligaments. The fragile skin and unstable joints found in EDS are the result of faulty collagen. Collagen is a protein, which acts as a "glue" in the body, adding strength and elasticity to connective tissue.
Ehlers-Danlos syndrome (EDS) is a heterogeneous group of heritable connective tissue disorders, characterized by articular (joint) hypermobility, skin extensibility and tissue fragility. There are six major types of EDS. The different types of EDS are classified according to their manifestations of signs and symptoms. Each type of EDS is a distinct disorder that "runs true" in a family. This means that an individual with Vascular Type EDS will not have a child with Classical Type EDS.
What are the symptoms of EDS?
Clinical manifestations of EDS are most often joint and skin related and may include:
Joints: joint hypermobility; loose/unstable joints which are prone to frequent dislocations and/or subluxations; joint pain; hyperextensible joints (they move beyond the joint's normal range); early onset of osteoarthritis.
Skin: soft velvetâ-like skin; variable skin hyper-extensibility; fragile skin that tears or bruises easily (bruising may be severe); severe scarring; slow and poor wound healing; development of molluscoid pseudo tumors (fleshy lesions associated with scars over pressure areas).
Miscellaneous/Less Common: chronic, early onset, debilitating musculoskeletal pain (usually associated with the Hypermobility Type); arterial/intestinal/uterine fragility or rupture (usually associated with the Vascular Type); Scoliosis at birth and scleral fragility (associated with the Kyphoscoliosis Type); poor muscle tone (associated with the Arthrochalasia Type); mitral valve prolapse; and gum disease.
What are the types of EDS?
There are six major types of EDS. The different types of EDS are classified according to the signs and symptoms that are manifested. Each type of EDS is a distinct disorder that "runs true" in a family. An individual with Vascular Type EDS will not have a child with Classical Type EDS. Learn more about the different types of EDS.
How is EDS diagnosed?
The categorization of the Ehlers-Danlos syndromes began in the late 1960's and was formalized in the Berlin nosology. Over time, it became apparent that the diagnostic criteria established and published in 1988 did not discriminate adequately between the different types of the Ehlers-Danlos syndrome or between the Ehlers-Danlos syndrome and other phenotypically related conditions. Learn more about the EDS nosology and how EDS is diagnosed.
How prevalent is EDS?
At this time, research statistics of EDS show the prevalence as 1 in 5,000. It is known to affect both males and females of all racial and ethnic backgrounds.
How is EDS inherited?
The two known inheritance patterns for EDS include autosomal dominant and autosomal recessive. Specifics regarding genetic inheritance may be found by following the link below. Regardless of the inheritance pattern, we have no choice in which genes we pass on to our children. Read more about hereditary patterns.
What is the prognosis of someone with EDS?
The prognosis of EDS depends on the specific type. Life expectancy can be shortened with the Vascular Type of EDS due to the possibility of organ and vessel rupture. Life expectancy is usually not affected in the other types.
What can I do now?
The defining trait of those affected by EDS is the search for information. The rise in Internet usage in the last few years has delivered a significant benefit to families affected by EDS. EDNF members are sharing information on-line and learning from each other in ways that were impossible a decade ago. Learn more about the benefits of EDNF membership.
What is EDS?
Individuals with EDS have a defect in their connective tissue, the tissue that provides support to many body parts such as the skin, muscles and ligaments. The fragile skin and unstable joints found in EDS are the result of faulty collagen. Collagen is a protein, which acts as a "glue" in the body, adding strength and elasticity to connective tissue.
Ehlers-Danlos syndrome (EDS) is a heterogeneous group of heritable connective tissue disorders, characterized by articular (joint) hypermobility, skin extensibility and tissue fragility. There are six major types of EDS. The different types of EDS are classified according to their manifestations of signs and symptoms. Each type of EDS is a distinct disorder that "runs true" in a family. This means that an individual with Vascular Type EDS will not have a child with Classical Type EDS.
What are the symptoms of EDS?
Clinical manifestations of EDS are most often joint and skin related and may include:
Joints: joint hypermobility; loose/unstable joints which are prone to frequent dislocations and/or subluxations; joint pain; hyperextensible joints (they move beyond the joint's normal range); early onset of osteoarthritis.
Skin: soft velvetâ-like skin; variable skin hyper-extensibility; fragile skin that tears or bruises easily (bruising may be severe); severe scarring; slow and poor wound healing; development of molluscoid pseudo tumors (fleshy lesions associated with scars over pressure areas).
Miscellaneous/Less Common: chronic, early onset, debilitating musculoskeletal pain (usually associated with the Hypermobility Type); arterial/intestinal/uterine fragility or rupture (usually associated with the Vascular Type); Scoliosis at birth and scleral fragility (associated with the Kyphoscoliosis Type); poor muscle tone (associated with the Arthrochalasia Type); mitral valve prolapse; and gum disease.
What are the types of EDS?
There are six major types of EDS. The different types of EDS are classified according to the signs and symptoms that are manifested. Each type of EDS is a distinct disorder that "runs true" in a family. An individual with Vascular Type EDS will not have a child with Classical Type EDS. Learn more about the different types of EDS.
How is EDS diagnosed?
The categorization of the Ehlers-Danlos syndromes began in the late 1960's and was formalized in the Berlin nosology. Over time, it became apparent that the diagnostic criteria established and published in 1988 did not discriminate adequately between the different types of the Ehlers-Danlos syndrome or between the Ehlers-Danlos syndrome and other phenotypically related conditions. Learn more about the EDS nosology and how EDS is diagnosed.
How prevalent is EDS?
At this time, research statistics of EDS show the prevalence as 1 in 5,000. It is known to affect both males and females of all racial and ethnic backgrounds.
How is EDS inherited?
The two known inheritance patterns for EDS include autosomal dominant and autosomal recessive. Specifics regarding genetic inheritance may be found by following the link below. Regardless of the inheritance pattern, we have no choice in which genes we pass on to our children. Read more about hereditary patterns.
What is the prognosis of someone with EDS?
The prognosis of EDS depends on the specific type. Life expectancy can be shortened with the Vascular Type of EDS due to the possibility of organ and vessel rupture. Life expectancy is usually not affected in the other types.
What can I do now?
The defining trait of those affected by EDS is the search for information. The rise in Internet usage in the last few years has delivered a significant benefit to families affected by EDS. EDNF members are sharing information on-line and learning from each other in ways that were impossible a decade ago. Learn more about the benefits of EDNF membership.
01 March, 2009
EDS
Ehlers-Danlos Syndrome
• Ehlers-Danlos (pronounced A-lerz-DAN-los) Syndrome, an inherited disease, is a defect in the connective tissue that supports many body parts, including muscles, tendons, ligaments and skin.
• The fibrous protein collagen is faulty, causing connective tissue to not be elastic or strong. Collagen acts as glue in the body, adding strength and elasticity to connective tissue.
• There are six major types of Ehlers-Danlos and symptoms depend on the type. Symptoms include loose, unstable joints and skin that bruises or tears easily.
• The syndrome may be diagnosed with a skin biopsy, a physical exam and a review of family and medical history.
• There is no cure. Treatment options vary and include joint protection, surgery to correct fractures or dislocated joints, pain management and having a strong support network.
• Sources don’t agree on the prevalence of the uncommon syndrome.
• People with Ehlers-Danlos generally have a normal life span. The syndrome does not affect intelligence but physical activity often is restricted.
SOURCES: The Arthritis Foundation, Ehlers-Danlos National Foundation, Mayo Clinic, Healthline
• Ehlers-Danlos (pronounced A-lerz-DAN-los) Syndrome, an inherited disease, is a defect in the connective tissue that supports many body parts, including muscles, tendons, ligaments and skin.
• The fibrous protein collagen is faulty, causing connective tissue to not be elastic or strong. Collagen acts as glue in the body, adding strength and elasticity to connective tissue.
• There are six major types of Ehlers-Danlos and symptoms depend on the type. Symptoms include loose, unstable joints and skin that bruises or tears easily.
• The syndrome may be diagnosed with a skin biopsy, a physical exam and a review of family and medical history.
• There is no cure. Treatment options vary and include joint protection, surgery to correct fractures or dislocated joints, pain management and having a strong support network.
• Sources don’t agree on the prevalence of the uncommon syndrome.
• People with Ehlers-Danlos generally have a normal life span. The syndrome does not affect intelligence but physical activity often is restricted.
SOURCES: The Arthritis Foundation, Ehlers-Danlos National Foundation, Mayo Clinic, Healthline
20 November, 2008
coincidence?
I was going through some old medical files that I had accumulated from when I was trying to figure out what was wrong with my hip. It was a very interesting list - I wrote down some things that might fit:
?Myofascial pain syndrome (neuromuscular disease; affects fascia; trigger points)
treated with PT, massage therapy, trigger point injection
?RA (inflammation of joint and tissue around joints; auto immune disease
?polyenthosopathy
-pinched nerve in cervical spine? feels like pinching/pressure in neck and shoots down right side of body
-Ehlers Danlos Syndrome -unstable joints; dislocating shoulder
What I find most interesting about this list is that I a)had this pain in my neck and arm longer than I can remember, it just wasn't as constant, and b) I think I am more qualified than most doctors. No doctor had mentioned anything about Myofascial pain until quite recently, and that was only because it was a physio in Holland. I knew I didn't have RA as I've never tested positive for it, but I guess I wanted to cover all bases. I was diagnosed w/ EDS after this, by a genetic doctor. And now I have been diagnosed with herniated, bulging discs and stenosis. I really should be a doctor.
Yes, I'm going to rant. And then rant some more. My major problem with the medical system in this country, well, one of them, is that all doctors are trained in parts. There is no doctor that I have found - if you know of one please tell me - that treats a body as a WHOLE. You have to go to a million different types of doctors that specialise in very specific things, and whilst that is good on one level, it is bad when they forget that that part of the body they focus on is actually CONNECTED to the rest of your body!
Where is this all going you ask? Well I had my appointment on Monday with the neurologist, Dr. Fatimi. Please don't go to her, she is dafter than a plank of wood. Perhaps she is just too young and clueless but she really doesn't have any idea what she's doing. My mother came with me to this appointment as I was to have the EMG test. Once we finally got into her office, she said - I thought you were going to have the EMG already? I couldn't believe it. I explained to her how I had had a whole long conversation with her assistants and we rescheduled the visit and the test to that day, from the 3rd. She said yes, I saw you were scheduled and you didn't come. I had to explain that I received a call from her office whilst I was away, and as I didn't return until midnight on the 2nd, I could not ring her office until the morning of the 3rd, and then tell the office that the appointment had been cancelled and rescheduled, which they said oh yeah, we have you for the 17th, for some reason it was still on the 3rd as well. This has to be one of the most disorganised offices ever. It drives me barmy. She didn't apologise or anything. Finally she arranged for the test to be done then, after she kept us waiting in the test room for ever. She didn't really explain what was going to happen, thankfully I had done my research, and did the electrode part first. They write on you for the measurements and then stick electrodes on you and zap you with electricity. This is not fun. I was trying to stay calm. It was extremely painful. Then it got worse, she did the needles. It was like medieval torture but with electricity. At one point she stuck a needle in my hand, in a v. sensitive post-surgery area of my hand that is extremely sensitive, and I lost it. She didn't warn me when she was going to do it or where, and just randomly kept stabbing and zapping. I hope whomever is reading this never has to have this test, but I won't lie. It was horrific.
After she was done, she said - wow you're so sensitive. Gee, a normal person would be in pain, but factor in the fact that I have fibromyalgia and my brain processes pain differently, then of course I was sensitive! That's what I mean about her - completely daft.
We then reviewed my MRI where she didn't even mention half of what the report said, until I brought it up. She then said she thought PT would work and gave me a prescription for that. I had brought along copies of some articles I had read about myofascial pain and dry needling, and gave them to her and explained that I believe (as my chiro does) that I have myofascial trigger points and that they need to be worked out before any PT can be done, because you can't strengthen a muscle that has a trigger point in it. I swear this went in one ear and out the other, then she seemed bothered that I was talking, wrote down the name a physiatrist, and said that I would need to talk to that person about treatment as she doesn't know anything else. What kind of doctor is that? Definitely not the kind that is concerned about a patient. I understand that with the way insurance is set up it is not to benefit the dr or the patient and they all have their numbers to do so they can make money, but if you don't want to treat patients then you shouldn't be a doctor. Period, case closed, regardless of the money. I have been to too many doctors to put up with this kind of crap. I am so fortunate that my primary, Dr. Rosen, takes the time out to talk to me, answer my calls, look into things when I need her to if it's something I can't do myself. And yes, I'm spoiled, but that is what a doctor should be like.
Ok, well, enough about that, sorry, I am just so frustrated.
After the lovely EMG I was in a lot of pain, stabbing, stinging pain. We had some wine and I thought I would be fine by the morning. But I was wrong. I woke up and could barely move my hand and everything was hyper-reactive in my body. The test inflamed everything and set me off down another spiral of bad pain all over my right side. I couldn't even go to work. Yesterday I was still pretty bad, and then I went to the chiro at lunch. He couldn't believe how tight my neck/shoulder area was and said there was a lot of Myofascial stuff going on. So I'm going to see him again tomorrow.
I did make an appointment with a physiatrist, Andre Panagos, at NY Pres who does dry needling, but it's not until the 29th. I heard back from Norman Marcus' office and will see about possibly going there. He doesn't take insurance and the first visit is $465, which I guess is better than $1200. I still can't get over that one!
So, onwards and upwards. My head is spinning from all of this and throbbing as well.
?Myofascial pain syndrome (neuromuscular disease; affects fascia; trigger points)
treated with PT, massage therapy, trigger point injection
?RA (inflammation of joint and tissue around joints; auto immune disease
?polyenthosopathy
-pinched nerve in cervical spine? feels like pinching/pressure in neck and shoots down right side of body
-Ehlers Danlos Syndrome -unstable joints; dislocating shoulder
What I find most interesting about this list is that I a)had this pain in my neck and arm longer than I can remember, it just wasn't as constant, and b) I think I am more qualified than most doctors. No doctor had mentioned anything about Myofascial pain until quite recently, and that was only because it was a physio in Holland. I knew I didn't have RA as I've never tested positive for it, but I guess I wanted to cover all bases. I was diagnosed w/ EDS after this, by a genetic doctor. And now I have been diagnosed with herniated, bulging discs and stenosis. I really should be a doctor.
Yes, I'm going to rant. And then rant some more. My major problem with the medical system in this country, well, one of them, is that all doctors are trained in parts. There is no doctor that I have found - if you know of one please tell me - that treats a body as a WHOLE. You have to go to a million different types of doctors that specialise in very specific things, and whilst that is good on one level, it is bad when they forget that that part of the body they focus on is actually CONNECTED to the rest of your body!
Where is this all going you ask? Well I had my appointment on Monday with the neurologist, Dr. Fatimi. Please don't go to her, she is dafter than a plank of wood. Perhaps she is just too young and clueless but she really doesn't have any idea what she's doing. My mother came with me to this appointment as I was to have the EMG test. Once we finally got into her office, she said - I thought you were going to have the EMG already? I couldn't believe it. I explained to her how I had had a whole long conversation with her assistants and we rescheduled the visit and the test to that day, from the 3rd. She said yes, I saw you were scheduled and you didn't come. I had to explain that I received a call from her office whilst I was away, and as I didn't return until midnight on the 2nd, I could not ring her office until the morning of the 3rd, and then tell the office that the appointment had been cancelled and rescheduled, which they said oh yeah, we have you for the 17th, for some reason it was still on the 3rd as well. This has to be one of the most disorganised offices ever. It drives me barmy. She didn't apologise or anything. Finally she arranged for the test to be done then, after she kept us waiting in the test room for ever. She didn't really explain what was going to happen, thankfully I had done my research, and did the electrode part first. They write on you for the measurements and then stick electrodes on you and zap you with electricity. This is not fun. I was trying to stay calm. It was extremely painful. Then it got worse, she did the needles. It was like medieval torture but with electricity. At one point she stuck a needle in my hand, in a v. sensitive post-surgery area of my hand that is extremely sensitive, and I lost it. She didn't warn me when she was going to do it or where, and just randomly kept stabbing and zapping. I hope whomever is reading this never has to have this test, but I won't lie. It was horrific.
After she was done, she said - wow you're so sensitive. Gee, a normal person would be in pain, but factor in the fact that I have fibromyalgia and my brain processes pain differently, then of course I was sensitive! That's what I mean about her - completely daft.
We then reviewed my MRI where she didn't even mention half of what the report said, until I brought it up. She then said she thought PT would work and gave me a prescription for that. I had brought along copies of some articles I had read about myofascial pain and dry needling, and gave them to her and explained that I believe (as my chiro does) that I have myofascial trigger points and that they need to be worked out before any PT can be done, because you can't strengthen a muscle that has a trigger point in it. I swear this went in one ear and out the other, then she seemed bothered that I was talking, wrote down the name a physiatrist, and said that I would need to talk to that person about treatment as she doesn't know anything else. What kind of doctor is that? Definitely not the kind that is concerned about a patient. I understand that with the way insurance is set up it is not to benefit the dr or the patient and they all have their numbers to do so they can make money, but if you don't want to treat patients then you shouldn't be a doctor. Period, case closed, regardless of the money. I have been to too many doctors to put up with this kind of crap. I am so fortunate that my primary, Dr. Rosen, takes the time out to talk to me, answer my calls, look into things when I need her to if it's something I can't do myself. And yes, I'm spoiled, but that is what a doctor should be like.
Ok, well, enough about that, sorry, I am just so frustrated.
After the lovely EMG I was in a lot of pain, stabbing, stinging pain. We had some wine and I thought I would be fine by the morning. But I was wrong. I woke up and could barely move my hand and everything was hyper-reactive in my body. The test inflamed everything and set me off down another spiral of bad pain all over my right side. I couldn't even go to work. Yesterday I was still pretty bad, and then I went to the chiro at lunch. He couldn't believe how tight my neck/shoulder area was and said there was a lot of Myofascial stuff going on. So I'm going to see him again tomorrow.
I did make an appointment with a physiatrist, Andre Panagos, at NY Pres who does dry needling, but it's not until the 29th. I heard back from Norman Marcus' office and will see about possibly going there. He doesn't take insurance and the first visit is $465, which I guess is better than $1200. I still can't get over that one!
So, onwards and upwards. My head is spinning from all of this and throbbing as well.
23 January, 2008
Genetic Doctor
I pretty much knew in advance what she was going to say, but with her positive spin on things she really knows how to make you feel better.
So do I have a genetic condition? Dr. Davis, whom I can't recommend highly enough, is very straight forward and direct. This is a quality I highly admire. I truly hate when people, especially doctors, jerk you around.
She said that whilst it can be confirmed or disproved that I have EDS, the likely hood is high, and that the only thing I can do is to moderate my life. Basically meaning no high wire stunts or climbing the Andes. I already realised this was not where my desires should be. The horseback riding is a hard pill to swallow even though I know it is v. v. bad for me. But such is life. She recommended that I don't do too much weight-bearing exercises and that aqua therapy would be the best thing to stick to. I had been hoping to get to the pool a couple weeks back but because of the hip irritation it hasn't happened yet. Hopefully soon.
I will try my best to preserve what I can for as long as I can, and that is all I can do.
So do I have a genetic condition? Dr. Davis, whom I can't recommend highly enough, is very straight forward and direct. This is a quality I highly admire. I truly hate when people, especially doctors, jerk you around.
She said that whilst it can be confirmed or disproved that I have EDS, the likely hood is high, and that the only thing I can do is to moderate my life. Basically meaning no high wire stunts or climbing the Andes. I already realised this was not where my desires should be. The horseback riding is a hard pill to swallow even though I know it is v. v. bad for me. But such is life. She recommended that I don't do too much weight-bearing exercises and that aqua therapy would be the best thing to stick to. I had been hoping to get to the pool a couple weeks back but because of the hip irritation it hasn't happened yet. Hopefully soon.
I will try my best to preserve what I can for as long as I can, and that is all I can do.
19 December, 2007
13 Week update
In honour of my thirteenth week of recovery, I decided to be brave and ditch the last crutch. Unlike most people I've had to use one for a much longer time. The idea was to keep down the inflammation in the surgical hip, and it did work. The main issue was it made my left hip very upset and inflamed.
Today is my third "official" day going sans crutch. It's been going pretty well. The main issues I've found are if I walk for a few blocks my hamstring starts to get tight and sore on the upper part, and the tendon under my right knee gets a bit irritated. My PT guys doesn't seem to concerned and I feel that the stronger I get it will work itself out. At least that's what I'm hoping. My psoas has been feeling a bit tight and we stretched it out at PT on Monday, and hopefully will do that again tonight. It's tricky for me as we don't want to overdue the stretching because of the EDS, but at the same time, it really needs to be done. I am going back to the genetic doctor in January so hopefully will get a better idea of what I should and should not be doing.
As for the left hip, it's not been too bad the last few days. Ironic as I'm getting an injection on Friday. I think it's very happy to be without the crutch and I am definitely more balanced so not putting most of my weight on it. It was pretty sore this am, but I also feel horrid and achy - it's my fibro kicking in. Sleep wasn't good for a few days and though I did fairly well the last two nights, the weather is a bit off where my body is concerned. I will have to make a concerted effort to get some good sleep in before I leave for London next week, as I don't want to start the trip off like this - the flight itself will be a challenge.
I am a bit nervous about the injection. I want it to make the pain go away, but at the same time, if it works, that means I will most likely need surgery. I am in no mental state to be able to handle that again, but at the same time I do want to get it over with so I can get on with my life. Ok, I'm not going to think about that now. As Scarlett says, tomorrow is another day...
Today is my third "official" day going sans crutch. It's been going pretty well. The main issues I've found are if I walk for a few blocks my hamstring starts to get tight and sore on the upper part, and the tendon under my right knee gets a bit irritated. My PT guys doesn't seem to concerned and I feel that the stronger I get it will work itself out. At least that's what I'm hoping. My psoas has been feeling a bit tight and we stretched it out at PT on Monday, and hopefully will do that again tonight. It's tricky for me as we don't want to overdue the stretching because of the EDS, but at the same time, it really needs to be done. I am going back to the genetic doctor in January so hopefully will get a better idea of what I should and should not be doing.
As for the left hip, it's not been too bad the last few days. Ironic as I'm getting an injection on Friday. I think it's very happy to be without the crutch and I am definitely more balanced so not putting most of my weight on it. It was pretty sore this am, but I also feel horrid and achy - it's my fibro kicking in. Sleep wasn't good for a few days and though I did fairly well the last two nights, the weather is a bit off where my body is concerned. I will have to make a concerted effort to get some good sleep in before I leave for London next week, as I don't want to start the trip off like this - the flight itself will be a challenge.
I am a bit nervous about the injection. I want it to make the pain go away, but at the same time, if it works, that means I will most likely need surgery. I am in no mental state to be able to handle that again, but at the same time I do want to get it over with so I can get on with my life. Ok, I'm not going to think about that now. As Scarlett says, tomorrow is another day...
29 November, 2007
10 (ish) week Post Op
I spent the long weekend watching my nephew and that did my hip in. Well, my left hip. The right one held up for the most part, but I did have to ice is every now and then. The left one however was completely miserable. I'm sure it had to do with running after Jack and picking him up.
I told my PT guy on Monday and he agreed. I still did the round of leg lifts w/o weights, and then the 3 lb on left side and 6 lb on right side with the stim and bolsters. I was so sore on Tuesday and very stiff on Wednesday. I am not happy about this as when I get like that I feel like I start to actually compensate with my right and then that starts to hurt. As I was meant to go to the theatre last night I hadn't booked my 2nd appointment for the week, so will have to work it out at home until next week. I don't have another appointment until Wednesday as I have company in town.
Last night I received a letter from the genetic doctor. I have already made an appointment with her (January was the earliest) to review and discuss what I should be doing to prevent more injuries. The letter basically summed up the biopsy results and what they meant. I will copy the relevant bits below. None of this was a surprise as she had explained that these tests do not really work for the type of disorder she believes I have, but it's good to rule out the ones it does work for.
"The results of your collagen study revealed no abnormalities. This is good news as this test excludes most forms of Ehlers-Danlos syndrome (EDS) type IV. This is a type of EDS that is associated with cardiovascular problems. The test you had also excludes some forms of EDS type VII another type of EDS which does not affect the cardiovascular system.
Unfortunately, the other major genes that affect collagen synthesis cannot be tested by means of this assay. No existing assay can help us better define your situation at this time.
I believe that your clinical situation is fibromyalgia plus hypermobile joints. Hypermobile joint disorder is a type of underlying connective tissue disorder that is in the EDS family.
Unfortunately, I do not have a specific test for this form of EDS at this point in time.
Many investigators are working on the various forms of connective tissue disorders in an effort to better understand them. I have no doubt that within a few years' time better testing will be available. It is also hoped that such testing could be performed on samples of venous blood."
So, nothing new or surprising, but in some ways it feels good to at least have a diagnosis that covers the multitude of issues I have had.
I told my PT guy on Monday and he agreed. I still did the round of leg lifts w/o weights, and then the 3 lb on left side and 6 lb on right side with the stim and bolsters. I was so sore on Tuesday and very stiff on Wednesday. I am not happy about this as when I get like that I feel like I start to actually compensate with my right and then that starts to hurt. As I was meant to go to the theatre last night I hadn't booked my 2nd appointment for the week, so will have to work it out at home until next week. I don't have another appointment until Wednesday as I have company in town.
Last night I received a letter from the genetic doctor. I have already made an appointment with her (January was the earliest) to review and discuss what I should be doing to prevent more injuries. The letter basically summed up the biopsy results and what they meant. I will copy the relevant bits below. None of this was a surprise as she had explained that these tests do not really work for the type of disorder she believes I have, but it's good to rule out the ones it does work for.
"The results of your collagen study revealed no abnormalities. This is good news as this test excludes most forms of Ehlers-Danlos syndrome (EDS) type IV. This is a type of EDS that is associated with cardiovascular problems. The test you had also excludes some forms of EDS type VII another type of EDS which does not affect the cardiovascular system.
Unfortunately, the other major genes that affect collagen synthesis cannot be tested by means of this assay. No existing assay can help us better define your situation at this time.
I believe that your clinical situation is fibromyalgia plus hypermobile joints. Hypermobile joint disorder is a type of underlying connective tissue disorder that is in the EDS family.
Unfortunately, I do not have a specific test for this form of EDS at this point in time.
Many investigators are working on the various forms of connective tissue disorders in an effort to better understand them. I have no doubt that within a few years' time better testing will be available. It is also hoped that such testing could be performed on samples of venous blood."
So, nothing new or surprising, but in some ways it feels good to at least have a diagnosis that covers the multitude of issues I have had.
23 August, 2007
M.I.A.
I've been MIA because this weather is killing me. Regardless of the hip, the rest of me feels like it's been run over by a very large 18-wheeler.
So the update is as follows:
I still have not heard back from Dr. Kelly's office about a Dr to see regarding what the PA had been talking about. I've sent multiple messages, and as I'm sure this does not rate high on their list, it's essential to me. So off I've been doing loads of research. My parents Dr recommended a rheumatologist at NYU, and I was going to see him (even though he doesn't take any insurance and it's $550 for the first visit - what cheek!). However, I came across some other names on some hyper-mobility boards I was reading and researched them. The one that seemed to stick out for me was Jessica Davis. She is a genetics specialist and when I rang the office happily found out she takes insurance! I also spoke to her assistant and told her (briefly) my various issues, and she said that is the kind of patient Dr. Davis sees. I try to not get too excited about these little things, but it was a relief to hear that I was barking up the right tree, or at least heading into the right forest. So my appt is for 6th Sept. I cancelled the appt w/ the $$$ rheumatologist for now. I figure that if this Dr says I should see one I can do that, but if there is no need I saved a nice chunk of change, and time too.
Dr. Davis' office asked me to bring any sort of records I could obtain. I believe I had an EKG and chest films a year or so ago so contacted my GP for those and also had to ring the horrid ex-rheumatologist's office to get the last blood test results she did for me. I can't tell you how much I was dreading that she's answer the phone. Thankfully she didn't and she seems to have a new assistant who seemed very nice and hopefully will send over those results quickly. Fingers crossed.
Now it's just for me to get through this horrid weather. When will it stop???
On a happier note, my friend and I are planning to go to London for New Years. I'm not sure how great an idea that is with my hip and what situation I will be in. I figure I deserve a reward for all that I am going through, so bugger it, why not!
So the update is as follows:
I still have not heard back from Dr. Kelly's office about a Dr to see regarding what the PA had been talking about. I've sent multiple messages, and as I'm sure this does not rate high on their list, it's essential to me. So off I've been doing loads of research. My parents Dr recommended a rheumatologist at NYU, and I was going to see him (even though he doesn't take any insurance and it's $550 for the first visit - what cheek!). However, I came across some other names on some hyper-mobility boards I was reading and researched them. The one that seemed to stick out for me was Jessica Davis. She is a genetics specialist and when I rang the office happily found out she takes insurance! I also spoke to her assistant and told her (briefly) my various issues, and she said that is the kind of patient Dr. Davis sees. I try to not get too excited about these little things, but it was a relief to hear that I was barking up the right tree, or at least heading into the right forest. So my appt is for 6th Sept. I cancelled the appt w/ the $$$ rheumatologist for now. I figure that if this Dr says I should see one I can do that, but if there is no need I saved a nice chunk of change, and time too.
Dr. Davis' office asked me to bring any sort of records I could obtain. I believe I had an EKG and chest films a year or so ago so contacted my GP for those and also had to ring the horrid ex-rheumatologist's office to get the last blood test results she did for me. I can't tell you how much I was dreading that she's answer the phone. Thankfully she didn't and she seems to have a new assistant who seemed very nice and hopefully will send over those results quickly. Fingers crossed.
Now it's just for me to get through this horrid weather. When will it stop???
On a happier note, my friend and I are planning to go to London for New Years. I'm not sure how great an idea that is with my hip and what situation I will be in. I figure I deserve a reward for all that I am going through, so bugger it, why not!
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