About EDS
Funny, You Don't Look Sick
By Claire Forst
Reprinted from Loose Connections Vol X Number 1
People who have chronic illnesses are in two distinct categories: those who look sick and get all kinds of sympathy (wanted and unwanted), and those who, no matter how they feel, just don't look "sick". How do we classify "looking sick"? Well if someone is looking pale and wan, or seems to be in pain, we ask them how they are feeling and if we can do anything for them. On the other side, is the person who may very well be in constant pain or at least discomfort, but is able to walk and talk and carry on with their daily activities, no matter what the cost is to their physical or emotional health. These people are almost never asked how they are feeling because if they look "well", we assume that they are fine. Most times, they aren't fine, however, and it's about time we acknowledge this.
Our daughter has an unknown type of EDS. She is always in pain, yet as a child, she is always striving to keep up with her peers so as not to be different. To look at her, you would not say she is a child with a medical problem, unless you see her when the pain overwhelms her and she cries on her bed. Even her doctor, a kind and caring person, was not tuned into the degree of discomfort she endures each day until he heard her scream in pain one morning. He was shocked. Not because he doesn't care or didn't believe her, but because she "doesn't look sick". Recently, she has gone through a bad time with her legs, and I sent her in alone to talk to her doctor. We are grateful that she is a very articulate 8-year-old who can make her position known in no uncertain terms. They were in the office for quite a while, and when they came out, his whole attitude was changed. No longer was there the "how can she be in such pain when she gets around and looks OK". What we saw, was a true understanding of the life our child leads and how hard she works to be a "regular" kid.
The same principle applies to everyone, no matter what age. When we look sick, we may be accepted with all of our limitations. If we are bent over, someone may rush to help us. If you are visibly handicapped, people may look away, but most people would offer necessary assistance. Most people with EDS look just like everyone else. They may limp, or have visible skin changes. But many people have scars, and that doesn't make them sick.
We must make the general public and members of the medical community aware that the way we look is only "skin deep". The way we "feel", both emotionally and physically, is the bottom line. So the next time you ask someone, "how are you", please take a moment and really listen to the answer; you can be sure that your concern and caring will be appreciated.
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Learning To Manage Fibromyalgia Syndrome
Darlene A. Clarke, R.N., M.S.N.
President & Founder of the Northeast Ohio Branch of the EDNF. Darlene has Hypermobility Type EDS and Fibromyalgia Syndrome.
Individuals with EDS are often also diagnosed with Fibromyalgia syndrome. Fibromyalgia is a common and often disabling disorder whose cause remains obscure. Fibromyalgia frequently resembles other disorders such as rheumatoid arthritis and lupus. Individuals commonly complain of widespread muscle pain and tenderness, as well as fatigue and exhaustion after minimal exertion. A characteristically high “tender point� is evident. For individuals diagnosed with Fibromyalgia, learning to effectively cope with this disorder is essential to one’s emotional and physical health.
It's morning and as you awake you realize you are exhausted and you haven't even left the bed yet. The thought crosses your mind that for as tired as you are, you may as well have never gone to bed. You barely have enough energy to get out of bed and care for yourself, let alone being able to care for the children, or go to work or school. As you struggle to get out of bed, you realize that your body aches all over and you feel as if you are a 100 years old. You are stiff and can hardly move. What is wrong with you? After seeking one or more medical evaluations, you finally get the answer. Your physician informs you that you have Fibromyalgia syndrome. Such a big word that sounds ominous and must mean you have some dreaded problem. You have never even heard the term before.
So, what is Fibromyalgia syndrome?
Fibromyalgia syndrome is a common form of chronic, generalized muscular pain and fatigue. In some cases, the pain is so intense that it can be incapacitating.
1 Despite that Fibromyalgia does not result in serious, long-term target organ damage; it does have a negative impact on the quality of life similar to that of rheumatoid arthritis.
2 To effectively manage and cope with Fibromyalgia syndrome, it is imperative that one has a clear understanding of Fibromyalgia, its signs and symptoms, triggering factors and how it is treated and managed.
The term "Fibromyalgia" can be broken down into fibro (fibrous tissue), my (muscles), and algia (pain and tenderness). In Fibromyalgia there is pain in the muscles and fibrous connective tissues which comprise ligaments and tendons. Therefore, while Fibromyalgia feels like a joint disease, it affects muscles and their attachments to bone and joint deformity does not ensue.
3 Fibromyalgia can run in families, possibly suggesting an inherited predisposition. To date, the cause of Fibromyalgia is not known. It may lie dormant until it is triggered by an injury/trauma, stress, or a sleep disturbance. There are other theories as to what triggers Fibromyalgia. One theory is that the syndrome is caused by an infectious agent such as the influenza virus. Other suspected causes include extreme physical deconditioning of the nervous system brought on by a lack of exercise, and changes in muscle metabolism that can result in decreased blood flow to the muscles with fatigue and decreased strength the end results.
4 More recent research points to two key chemicals of the Central Nervous System that facilitate the regulation of pain messages transmitted to our brain. These two chemicals are substance P and serotonin. Substance P begins the pain–signal process following tissue injury. Serotonin reduces the intensity of pain signals in the transmission of pain and is very important in sleep regulation. Low levels of serotonin and elevated levels of substance P have been found in individuals with Fibromyalgia. These findings support the theories that individuals with Fibromyalgia have unusually high pain intensity messages transmitted to their brain as well as deficiencies in the inhibition of pain.
5 One clear fact remains: more research is needed on Fibromyalgia. Despite not being able to identify the exact cause of Fibromyalgia, individuals present with similar signs and symptoms.
Signs and Symptoms
Probably the most frustrating aspect of having Fibromyalgia syndrome, as in EDS, is that you "look healthy." Compounded by the fact that test results are normal, it is difficult to convince others that something is wrong with you. Every X–ray and blood test your physician ordered has come back within normal limits. You embark down the path of self doubt and frustration - is this pain real, or is it all in my head? Why is it so difficult to put a label to the signs and symptoms of Fibromyalgia?
Fibromyalgia is frequently misunderstood and confusing because the symptoms seen in this syndrome are also found in other conditions such as rheumatoid arthritis and lupus. Women are affected more than men and the symptoms usually occur first when an individual is between 20 and 40 years old.1 However, it must be noted that Fibromyalgia can sometimes affect children and the elderly.4 Recent research studies have established guidelines for diagnosing Fibromyalgia based on the presence of certain signs and symptoms.
Pain
The most common complaint from patients with Fibromyalgia is that they have diffuse pain all over, almost like the flu. Although in rarer cases, the pain is quite severe and disabling. More often, the symptoms often begin insidiously and may wax and wane in severity. In almost all cases, individuals complain of some degree of pain continuously. The pain is most often worse at tender points specifically located on the body. Fibromyalgia type pain generally is worse in the morning and late evening and often is associated with stiffness. Muscle groups that are used repetitively result in an increase in the level of pain you experience.
Fatigue and Sleep Disturbances
Have you ever awaked after sleeping and felt refreshed and rejuvenated? Most likely your answer would be Immense fatigue is frequently the debilitating aspect of Fibromyalgia. The fatigue may be exhibited as an overall tiredness and lack of energy, or as muscular fatigue and a lack of endurance. In either case, it can be difficult to perform activities of daily living such as caring for self or others, performing household chores, going shopping, or to function effectively at work. Keep in mind that it takes a tremendous amount of energy to deal with having chronic pain and this too can contribute to an individual's fatigue.
Approximately 90% of individuals with Fibromyalgia complain of moderate to severe fatigue coupled with a lack of energy or the type of exhaustion that is found with a lack of sleep. In most cases, individuals wake up feeling tired even after sleeping all night. While their minds may be rested, their bodies feel as if they never went to sleep. Many are aware that their sleep has become lighter with frequent awakenings throughout the night. Scientific studies reveal that individuals with Fibromyalgia have abnormal sleep patterns and are deficient in Stage 4 sleep - the deepest stage.1,3,5 It is important to note that a secondary Fibromyalgia syndrome can occur with any connective tissue disease (EDS included) and may not necessarily be related to sleep disturbances.6
Central Nervous System Symptoms
As in other chronic illnesses, mood and mental changes can occur in individuals with Fibromyalgia. Many individuals feel down, although only about 25% are clinically depressed. In most cases, depression and/or anxiety tends to follow the onset of Fibromyalgia symptoms and may be the result of Fibromyalgia and not the cause of it.3
People with Fibromyalgia may also experience numbness and tingling in their hands, arms, face, feet and legs. These symptoms can also be found in other disorders and usually require numerous tests before the diagnosis of Fibromyalgia is reached by a physician.
Other Problems
Muscular as well as migraine headaches are common in Fibromyalgia. Abdominal bloating, pain, alternating diarrhea and constipation are also commonly seen. They resemble irritable bowel syndrome or spastic colon in nature. Similar bladder spasms and irritability results in urinary urgency and frequency. The skin and circulatory system are sensitive to moisture and temperature changes resulting in temporary changes in skin color.2,3
Clinical Diagnosis
Currently, no definitive laboratory tests exist to make the diagnosis of Fibromyalgia. Rather, the diagnosis is made by a physician after obtaining the individual's medical history and performing a complete physical examination.
A hallmark of Fibromyalgia syndrome is reduced pain thresholds at designated tender points, demonstrated upon palpation.2 According to the American College of Rheumatology, the diagnostic criteria for Fibromyalgia includes: a). widespread diffuse pain that has been present for at least three months and b). pain that is present in 11 of the 18 bilateral tender point sites (see figure 1) when 4 kg of force is applied by digital palpation.1,2,7 Recall your response the first time your physician palpated these 18 tender points. Did you say ouch, flinch, make a facial grimace, and attempt to withdraw away from your doctor or even jump? These are the usual responses elicited by an individual with Fibromyalgia. Frequently, patients are shocked by the level of pain they experience when these areas are palpated. Now that you know how Fibromyalgia is diagnosed, let's look at the common triggering factors of Fibromyalgia.
Common Triggers of Fibromyalgia
Three common factors seem to trigger a flare in Fibromyalgia symptoms, physical factors, emotional factors and environmental factors. Keep in mind that your Fibromyalgia can become aggravated by these factors alone or any combination of these factors.
Physical Factors
Several physical factors can aggravate your Fibromyalgia. Infections are one type of physical factor that can produce the most profound exacerbation of Fibromyalgia symptoms. The second type of physical factors deal with ergonomics. The positions we maintain our bodies in has a direct correlation to the pain associated in Fibromyalgia. For instance, do you have a job that requires you use repetitive movements all day? Are you required to work in one spot with your arms elevated above your work surface? Do you need to remain in one position (i.e., standing, sitting or driving) for prolonged amounts time? If you answered yes to any of the above questions, you will be able to relate to the pain that results from maintaining your muscles in a sustained isometric position for any length of time. Ergonomics also plays an important role at home. It's often difficult for someone with Fibromyalgia to push a vacuum cleaner, wash walls, paint or prepare meals. These tasks require the use of shoulder muscles to maintain arms in extension at some level in front of our body. What generally happens is that an ache begins in the neck, shoulders and upper back. The ache rapidly turns into pain that elevates in intensity as it slowly radiates up and down the back. You feel as if you can't maintain your arms in this position any longer, and you quickly return your arms to your sides as you wait for the pain to slowly ebb away. Most, if not all of you, can relate to this all too familiar type of pain.
Emotional Factors
Stress is something that each of us has to deal with on a daily basis. Unfortunately, emotional stress is a prominent factor aggravating Fibromyalgia. We are faced with stressful situations at home, work, school, driving your car on the highway etc. It isn't difficult to identify situations that aggravate your Fibromyalgia symptoms that's the easy part of the equation. The trick is to avoid or attempt to modify any stressful situation.
It is extremely difficult to deal with those situations in which any stressor is unrelenting. These types of situations can present themselves in the home with family members or in the workplace with coworkers or supervisors. In these situations, one must look at not only the circumstance, but how you react to the stressor. Ultimately, it comes down to dividing these stressful situations into two categories: those that you have control over and those you have absolutely no control over. In circumstances that you have no control over, it is imperative that you learn to alter your response to those particular situations. Save your energy for those battles that you can emerge victorious.
Environmental Factors
How many times have you been able to predict the approaching weather without having looked at a weather map? Probably too numerous to count. You feel like you are a human barometer with the ability to predict weather changes before they occur. Most likely you have found that when the barometric pressure drops, it is followed by rain, cold, snow, and fog - all of which aggravate your symptoms.
Many individuals with Fibromyalgia report that certain types of artificial lighting, cold drafts or irritating noises often result in a flare of their muscle pain. When was the last time that you were able to enter a pool that wasn't heated? Just a guess, but it was probably many years ago. Most individuals with Fibromyalgia do not tolerate swimming in cold or cool water. We are usually found in the heated pools that others say are too hot to swim in or in hot tubs. The goal is to determine what factors aggravate your Fibromyalgia and either eliminate them if possible or at least modify them. Now that you know what factors can trigger your Fibromyalgia, let's look at how it is treated and managed.
Treatment and Management
Since Fibromyalgia's definitive cause is not known, current treatments are focused on relieving painful symptoms, not necessarily curing them. Additionally, the treatment program prescribed by your physician will be individualized to address your specific needs.
Numerous treatments have been employed over the years for Fibromyalgia. No one method has been entirely successful, but several methods will be prescribed by your physician in an attempt to control your symptoms. A sense of overwhelming relief comes with the validation of your symptoms and a diagnosis. Just knowing the name of your condition will not be enough though. Your physician and healthcare team will educate you and your family about Fibromyalgia. Specifically, you'll be informed about your prognosis, possible causative factors, the aspects of treatment and specific body mechanics techniques. This education will prove invaluable to you. If you are taught about what to expect and how to deal with flare-ups, you will be able to control your Fibromyalgia. The goal of education is to moderate the effects of Fibromyalgia on your life. Only then will Fibromyalgia not become your entire life.
Medications may be prescribed by your physician to control pain and improve deep Stage 4 sleep. If pain is prominent, your doctor may prescribe a low dose of an analgesic or nonsteroidal anti-inflammatory drug, although most nonsteroidal anti-inflammatory drugs are marginally beneficial and carry the risk of side effects that outweigh their benefits. Therefore, you will be most likely instructed to use over the counter medications such as acetaminophen, aspirin or ibuprofen to relieve pain and reduce stiffness. If you are currently on a nonsteroidal anti-inflammatory drug to help manage your EDS, your physician will likely instruct you to take acetaminophen, not ibuprofen or aspirin, to control your pain.
To improve Stage 4 sleep, your physician may prescribe low doses of tricyclic antidepressants (Elavil, Sinequan) or the muscle relaxant cyclobenzaprine (Flexeril). These drugs are structurally similar and they block the uptake of the neurotransmitters that regulate deep Stage 4 sleep.3,8. These medications carry the risk of potential side-effects which include: drowsiness, weight gain, dry mouth and constipation to name a few. Do not be discouraged if you do not respond to the first medication(s) prescribed. Your doctor may need to adjust dosages or change drugs to achieve an improvement.
Individuals with Fibromyalgia should practice good sleep hygiene to promote sleep. Bedtime rituals can relax you in preparation of sleep. These bedtime rituals can include: taking a warm bath or shower, reading a novel, watching a relaxing television program or listening to soothing music which can promote sleep. Attempt to follow a regular sleep schedule and go to bed at the same time each day. Sleep eight hours nightly if possible. Avoid alcohol, caffeine and tobacco before retiring they will disturb your sleep. Also abstain from taking midday naps.
Exercise is a key element in Fibromyalgia management. Your physician will recommend that you slowly increase your aerobic fitness with structured, low impact activities such as swimming, water aerobics, walking, or riding a stationary bicycle.2,3,7,8 Research has indicated that aerobic exercise improves sleep, enhances endorphin release and has antidepressant effects.2 It is important to check with your physician to ensure that the exercise program you have chosen is not contraindicated in your specific type of EDS. Remember to always stretch your muscles before engaging in any aerobic activity. Increase your exercise program gradually to reduce the chance of injury. As you continue to exercise, you'll notice that you feel better, have more endurance and experience a reduction in Fibromyalgia symptoms. It's worth the possible pain and fatigue felt initially to achieve these end results.
Always practice proper body mechanics and apply ergonomic principles at home, work and at rest. These techniques will prevent injury and reduce flare-ups of Fibromyalgia symptoms. Your doctor may prescribe various physical therapy treatments.
Possible treatments include: massage, heat or ice applications, ultrasound or whirlpool therapy and microvoltage electrical stimulation. These are all passive physical therapy techniques with varying results from individual to individual. These treatments are usually prescribed for flare-ups and are employed for short periods of time.3,8
Taking Control of Fibromyalgia
You can engage in several actions that will help you gain control of your Fibromyalgia. First and foremost, listen to your body. Identify those triggers that aggravate your symptoms and then avoid or at least minimize them. Get your stress level under control by practicing relaxation techniques. Relaxation techniques are primarily effective for chronic pain and have many benefits. Relaxation techniques enable you to reduce anxiety, pain and stress, reduce muscle tension pain and relieve the helplessness and depression associated with chronic pain. Despite having chronic pain and fatigue, learn to develop a positive attitude. Remember that if you have a constant negative attitude, your Fibromyalgia symptoms will exacerbate. Look for the good in every situation out there, but you may need to look closely.
Eat healthy and avoid fat in your diet. Excess weight is detrimental to your body and can aggravate your symptoms. Eating properly also maintains your health and avoids illness. Keep in mind that an illness (i.e., cold, flu, bacterial infection etc.) can be a triggering factor for your symptoms.
Educate yourself, family and healthcare providers about Fibromyalgia syndrome. Remember that knowledge is a powerful weapon in coping with any chronic illness. Knowledge also helps you to take control of your Fibromyalgia and reduce the exacerbation of its symptoms.
Lastly, seek support from others with Fibromyalgia. Check with your physician, healthcare institution, and within your local community for a Fibromyalgia support group. It is extremely beneficial to receive support and understanding from others who have the same type of problems from Fibromyalgia as you do. Connecting with a support group also helps you and your family to cope with having a chronic illness. And who knows, maybe someday you will have the opportunity to offer support to someone else just diagnosed with Fibromyalgia. I guarantee it will be one of the most gratifying and fulfilling experiences of your life.
Conclusion
Receiving a diagnosis of Fibromyalgia syndrome is an important first step in managing this condition. It is not the last step though! It is imperative that you have a clear understanding of Fibromyalgia, its signs and symptoms, triggering factors and treatments. Learning to effectively cope with this disorder is essential to one's emotional and physical health. It is only then that you will be able to manage your Fibromyalgia and prevent it from becoming your whole life. While there isn't a cure for Fibromyalgia, there are treatments that are effective in managing its symptoms. There is life after finding out you have Fibromyalgia. Tomorrow will be better and holds the possibility of a cure!
References
1 Fan, P.T., & Blanton, M.E. (1992). Clinical features and diagnosis of Fibromyalgia. Journal of Musculoskeletal Medicine, 9(4), 24-42.
2 Wilke, W.S. (1996). Fibromyalgia: Recognizing and addressing the multiple interrelated factors. Postgraduate Medicine, 100(1), 153-“170.
3 Arthritis Foundation, Inc. (1995) Fibromyalgia Syndromes, 1-12.
4 Dunkin, M.A. (1993, September–October). Fibromyalgia: Out of the Closet. Arthritis Today, pp. 24-28.
5 Dunkin, M.A. (1997, September–October). Fibromyalgia: Syndrome of the 90s. Arthritis Today, pp. 41-47.
6 Ignatavicius, D.D., Workman, M.L., & Mishler, M.A. (1995). Medical & Surgical Nursing (2nd ed.). Philadelphia: W.B. Saunders Company.
7 Goldberg, D.L. (1990) Fibromyalgia and chronic fatigue syndrome: Are they the same? Journal of Musculoskeletal Medicine, 7(5), 19-28.
8 Sherman, C. (1992) Managing fibromyalgia with exercise. The Physician and Sports Medicine, 20(10), 166-172.
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EDS Physical Therapy Management of the Hypermobile Patient
This presentation by Mr. Terry Olson, PT, MHS, FAAOMPT at the 2005 EDNF Conference in Los Angeles is accompanied by a Power Point presentation. Don't have Power Point? Click Here
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.
Thank you
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Water Training and Ehlers Danlos Syndrome
by Lynda Huey, M.S. & Linda Neumann-Potash, R.N., M.N.
In recent years, water training has become the basic building block of therapeutic and conditioning programs for a wide range of people: athletes, dancers, fitness, fitness enthusiasts, post surgical patients, and those with arthritis and Ehlers-Danlos Syndrome (EDS). In water, you can walk, run, jump and kick without any trauma to your weight-bearing joints. The moment you slide into the water for a workout, you feel significantly better. Pain is reduced; mobility is regained. Where you sensed helplessness, now there is hope. For in water, you can perform movements that seem but a dream on land.
I have Ehlers-Danlos Syndrome type III. I started exercising in water with Linda Huey as an alternative to physical therapy in May of 1990. I have problems with many of my joints, and was looking for a form of exercise and rehabilitation that could help me strengthen the muscles around my joints to increase stability. Further, I wanted to prevent dislocations in currently non-affected joints. Having been a runner and a volleyball player during my high school and college days, I missed being active and feeling fit. Besides finding a way to preserve my joints and decrease pain, another of my motivations was to find a way to get in shape. Running, fitness walking, and bicycling were not options because of arthritis and my frequent patella dislocations. I contacted Lynda. I was the first person she had met with Ehlers-Danlos Syndrome, and she was eager to work with me. The program she set up for me includes a cardiovascular workout and isolated exercises for my upper and lower extremities.
You can build a Water Healing Workout that protects the injured body part form further harm, while the rest of the body continues to train. Standing in chest-deep water, you weigh only 10% of your normal body weight, so every exercise is low impact. If no impact is required, you can put on a flotation device and train while suspended in water. Injury areas are immobilized to prevent aggravation either with tape, slings, braces, or waterproof casts. If you don't normally wear braces, you probably won't need them in the pool. If you do wear braces, check with the manufacturer about using them in water and make sure that you rinse them out well after the workout.
Because of joint instability, I have found it necessary to wear protective devices in the water. Deciding which braces I needed was done by trial and error. I had patella (knee cap) dislocations and ankle instability in my left leg if I did not wear knee and ankle braces into the water. I use the CTI Brace (from Innovation Sports) for my knee and a standard ankle brace with a figure-8 wrap around my ankle. Because of multiple hand surgeries, I must also wear a right hand splint for protection. Whenever I have problems with shoulder dislocations, I also wear a shoulder immobilizer. This sounds like a lot to wear during a workout, but it allows me to safely exercise without injury. If I shrug off one of more of the protective devices, I usually experience pain, instability, and/or dislocations.
You may not have exercised in months or even years, yet you can enter the water and begin immediately. Most EDS patients should begin with the Deep Waterpower Workout, in which there is absolutely no weight bearing. You don't even need to know how to swim, for you will be wearing a flotation device that will hold you in a comfortable upright position for exercising. Choose a Wet Vest ($130), a Wet Belt ($40), an Aqua Jogger ($50), or use a standard water ski flotation belt ($10-$20) -- whatever best fits your body and your pocketbook.
Once outfitted, take to the water and slowly begin water running. Keep in mind your specific limitations. You may feel terrific floating along the surface of the water, but remember to try any movement, particularly ones that involve your affected joints, with caution. Move slowly through a narrow range of motion. If such movements cause no pain, gradually reach for a wider range of motion. Add speed last and with care. EDS patients should use the resistance of the water only; don't apply undue stress to the joints by adding any of the new resistance equipment devices.
Water Running. Keep your chest and head erect. Shoulders are relaxed and down. Knees lift to 90 degrees while the arms pull forward and back with no lateral movement. Hands are relaxed with the thumbs towards the sky. Pull the elbows back, each in its turn, and touch each hand into an imaginary pocket. Don't lean too far forward, or you'll be a dog paddling. Simply lift the knee, and then push the foot straight down behind you. Don't lean to far back or you will have a tendency to kick forward into a bicycling motion. Move around the pool for five minutes as you warm up. (If the pool is small, or if you need to stay in one place because the pool is crowded, use a Perry band ($50) or StretchCordz ShortBelt ($35) to tether yourself to the side of the pool.)
I noticed results right away. I expected to be sore, but I wasn't. I had less pain in my joints after the first workout. More important was the sense of accomplishment I felt. Wow! I could actually exercise without joint dislocation; something I hadn't been able to do in years. Over the months of water training, I have noticed that my joints are getting stronger and even though I continue to have dislocations, they are not as traumatic and not as frequent. The water offers me a soothing affect after any dislocation, and helps lessen the pain. I am now in better shape. I've lost 20 pounds and I seem to have more energy during the day. After years of focusing on what I can't do, I now focus on what I can do. It’s great to be able to run in the water. I feel like a runner again!
Water Walking. Start by establishing an "opposite positions". Hold your right arm forward and extend your left forward at the same time. Begin walking, keeping both your arms and legs straight. Visually check your elbows and knees. Most people think these joints are straight when they are not. Stay upright; don't lean forward or back. Water walk for three to five minutes, or until you become confident of the movement. Insist on opposition: right arm with left leg are left arm with right leg.
Having learned water running and water walking, you can now build a training program by altering those two skills and challenging your muscular strength and aerobic skills.
I've had several interruptions in my water rehabilitation program because of joint surgery, casts, and skin and healing problems. Since I have problems with healing and am prone to infections, I wait until my incisions are completely healed, and I always check with my physician for clearance before rushing quickly back to the water. Occasionally, Lynda and I have figured out ways to keep me active during what normally would have been an interruption. After a thumb injury that required casting, I asked my doctor to use a new waterproof gortex cast padding so I could continue water training. Since I had no open wounds, he agreed and we continued. Other times, we taped my arm into a plastic bag and elevated it above water level on flotation devices.
Water running and water walking are the two key exercises in a deep-water training program. However, other deep-water exercises specifically for the abdominals, arms and legs can be added over time. If you'd like a copy of the Deep Waterpower Workout booklet, due out in early 1992, or any of the equipment listed above please contact:
Huey's Athletic Network
3014 Arizona Avenue
Santa Monica, CA 90404
(310) 829-5622
Fax: (310) 828-5401
Exercise can be very important for patients with Ehlers-Danlos Syndrome, but finding the right form of safe exercise is the hardest part. Although EDS affects all of us differently, I feel that everyone can benefit from some form of water exercise. Just check with your physician before beginning this or any exercise program
Water training has made a big difference in my life. It has helped me strengthen my joints, increase stability and stamina, lose weight, and feel better both physically and emotionally.
Lynda Huey, M.S., has water trained dozens of Olympic and professional athletes as well as worked with many pre-surgical and post-surgical patients. In 1986, she published The Waterpower Workout (New American Library) and is finishing her next book Water Healing Workout: From Rehabilitation to Gold Medals.
