Hypermobility Hope

How to Get the Most Out of Your Doctor Visit

2011-07-26T11:15:00.001-07:00

Whether you are being seen by a doctor who is very knowledgeable about hypermobility syndrome or a doctor who doesn't have a clue, there are certain steps you can take as a patient to make sure that you get the most out of your doctor appointment. It is important to be proactive and take responsibility for your health - and remember, the person who knows the most about your body isn't your doctor, its you! You have to be your own health advocate, and when it comes to a doctor visit that means being prepared. Here are some tips to help you do that!

1. Know the Questions You Want to Ask Beforehand
In the days leading up to your appointment, leave a notepad out so you can jot down questions as you think of them. This is a lot easier and less frustrating than trying to brainstorm all your questions at once, and makes it much less likely that you will forget any questions you may have. The night before your appointment look over your list and rewrite your questions in order of importance. This helps you go into the doctor's office with a clear idea of what your purpose is for going and what you want to get out of your visit. Once you get in the exam room, though, it can be useful to start by asking one of your easier/less complicated questions first. This helps start the appointment on a positive note and helps you build a rapport with your doctor which will make asking more difficult or detailed questions a little easier. Its also important to remember that doctors are almost always running behind schedule, and many may try to rush through your appointment. If this is the case, stick to your list and do not let the doctor leave before he/she has addressed all your concerns - remember, you are paying your doctor to be there, and you deserve to have all your questions addressed. If you want, make a copy of your list of questions and hand it to the doctor when they walk through the door. If the doctor has your list, he/she is more likely to stick to it and not try to leave before you have discussed each point.

2. Have a Clear List of Symptoms
When dealing with a complex, chronic illness like hypermobility syndrome it is important to always keep a health dairy. You don't have to write a lot, but you should try to jot down all the symptoms you have each day (pain, headache, fatigue, muscle spasm, etc.), how severe those symptoms were (either on a scale from 1-10 or describe what that symptoms kept you from doing), when they happened, how long they lasted, and if you felt there was a specific trigger for any of those symptoms. It is extremely helpful to bring your diary to your visit so your doctor can get a complete look at your condition and how it impacts your life. It can be a bit unpractical for your doctor to read through your entire diary in one visit though, so the night before an appointment I like to print off a blank calendar on a piece of paper and then write the name of each symptom on the days I had them. This is great because it not only tells your doctor your symptoms, but also helps them see any patterns that might help them make a diagnosis or formulate a treatment plan.

3. Bring Another Set of Ears
If you can, it is always helpful to bring someone with you to your appointment. A lot of times I will leave an appointment and ask myself 'what just happened? what did the doctor say?' Things can make sense and sound good in the exam room, but once you leave it it can be hard to remember everything the doctor said and figure out how that doctor talk translates into real English. Having another person is great because they can not only help you have a good appointment, but afterward you can ask them 'what did you think the doctor said?' 'what did you hear?' It always helps to get another person's opinion, especially when it comes to complicated medical matters. If you can't find anyone to go with you (and even if you do), it helps to bring a notebook so you can take notes. This will help you remember the most important things your doctor said. If you go alone you can also ask your doctor if its okay for you to record your session so that you can go back and review it as many times as you need.

4. Make Sure You Leave with a Plan
Hypermobility syndrome is a complex disorder that cannot be treated or solved in just one visit or even by just one doctor. Because of this, its best not to look at any one appointment as 'the one' that will solve all your problems, instead look at it as helping you take one more step in the right direction. So even though you probably wont be leaving your doctor with a 'cure' its important for you to at least leave with a plan. The plan your doctor gives you should have two components: first, a plan for what to do in the immediate future, and second what your next step after that should be. It is important to leave with a plan for the immediate future so that you can be proactive and feel in control of your condition. Even if your doctor doesn't know or understand what is wrong they can still give you a plan - maybe to try a new medication, increase the dosage on a medicine you are already taking, visiting a specialist, or just watching your symptoms for a few more weeks until your next appointment. It is also important to leave with a plan for what your next step will be so that you know even if your current plan/treatment isn't working that its not the end of the road. Hopefully you can leave with a plan for what to do if your current plan works as well as a plan for what to do if it doesn't.

I hope these guidelines will help you get the most out of your next doctor visit. And always remember that if you see a doctor who doubts you or disrespects you, you can and SHOULD find a new one. Even if you have a great doctor it can still be helpful to get a second opinion. Hypermobility syndrome is a complicated illness that will take multiple visits to get control of, so be persistent and never take no for an answer - its your health and you deserve the best care possible!

Love and Hope,
Chelsea

Gender and Pain

2011-04-01T14:56:00.000-07:00

In the April 2011 issue of More magazine, Alice Lesch Kelly writes a brilliant article entitled “The Male-Female Pain Gap” which explores why men often receive better pain relief from their doctors than women. In her article, Kelly uncovers three disturbing statistics ...

1) Women are less likely to be treated for pain and or/pain disorders than men. Kelly gives several examples of studies which show that women are treated less often for pain than men. Kelly explains that one reason for this is that women tend to describe their pain with more emotion than men, which has led many doctors to believe, consciously or unconsciously, that women tend to exaggerate their pain. As a result, the myth that women make up pain that doesn't exist or that they experience pain that is only 'in their heads' continues to circulate within the medical community. These biases often mean that women are more likely to have their pain dismissed or ignored by medical professionals than men.

2) Women experience more pain than men. Kelly explains that despite the fact that women are treated less frequently for pain, they are much more likely to suffer from long-term conditions that cause pain such as fibromyalgia, osteoarthritis, and irritable bowl syndrome, and are also more likely to to suffer from more than one painful condition at a time. There is also evidence that female hormones like estrogen can diminish women's ability to tolerate pain, and can also affect the effectiveness of many painkillers. Kelly also reveals that although there is a general belief that women can handle pain better than men because of their ability to handle childbirth, women tend to have lower pain tolerance thresholds than men. The result? Women tend to experience more pain in their daily lives than men do.

3) Women are less likely to be prescribed adequate pain medication. Despite the fact that women experience more pain than men, Kelly reveals that women are significantly less likely to be prescribed high-potency painkillers and that physicians are often more apprehensive about prescribing potentially addictive drugs to women. She also found that African American women were less likely to receive proper pain medication than white women with the same condition.

So what does all of this mean for patients with Hypermobility Syndrome? Well, although HMS affects both men and women, the condition is more commonly seen in women, and many of the gender biases described above help explain why many female HMS patients spend months and even years trying to get a proper diagnosis. Like fibromyalgia, HMS is often seen as a 'women's disease,' and as such its seriousness and ability to affect a patient's quality of life are often downplayed because of gender biases that suggest that women exaggerate their pain symptoms. Fighting these gender biases within the medical community is one way to ensure that HMS patients of both genders will their voices heard and their health-care needs met.

So, what can I do to make sure my pain is properly treated? Kelly gives some great suggestions for anyone seeking medical assistance for pain. Try following these tips at your next doctors visit to help ensure that your pain symptoms are properly addressed.

1) Use clinical language when describing your pain - when describing your pain symptoms to your doctor try to not get emotional, instead, try to use objective language and give your doctor concrete examples of how the pain is affecting your life. When describing the severity of your pain, use the ten-point scale, with 0 representing no pain and 10 representing the most pain imaginable. For example, instead of telling your doctor 'my back really hurts' try explaining your symptoms like this - 'three days this week I experienced back pain that was a six out of ten, I was forced to take a half-day off of work, and was unable to attend to my household duties so I had to call a friend to watch my children and have my husband cook diner.' Describing your pain this way, by giving specific information about its intensity, duration, and impact on your daily function will give your doctor important medical information, and encourage them to look at your pain more objectively. Remember, pain is a subjective experience, you can't measure it or see it, but doctors like to treat conditions where their are objective tests that can show them there is something wrong. By being more clinical and objective when describing your pain, you are translating your personal experience into language a doctor can better understand and is more likely to respond to.

2) Keep a pain journal - one great way to convey your pain symptoms to your doctor is to keep a pain diary. Be as specific about your symptoms as possible - what type of pain did you have, was it burning, stabbing, aching, crushing? When did the pain occur, was it triggered by something specific? How long did it last, did it respond to anything you did? Did your pain affect your appetite or ability to perform daily tasks? Try going to www.caremark.com/Imagebank/Health_Diaries/DailyPainDiary.pdf or www.healthinaging.org/public_education/pain/my_pain_diary.pdf to download a free template that you can use as your daily pain journal. Each time you visit your doctor, bring your pain dairy. This will give your doctor information that will help them spot patterns in your symptoms and make it easier for them to recognize the presence of an underlying condition or stimulus for your pain. A pain diary will also help you understand your pain better, help you to spot triggers and the treatments that work best for you, which will help you handle your pain better.

3) Find the right doctor - some doctors are more experienced and more comfortable treating pain disorders than others, so if you feel your doctor is not the right fit or if they downplay your pain and/or accuse you of making it up, find a new doctor! Try looking for doctors in your area who have either studied pain management or have experience with pain patients, or try visiting a pain management center. You deserve to have your concerns listened to and respected, so if you find yourself in a situation where you think you are being doubted or disrespected, you have the right and the duty to yourself to seek medical help elsewhere. In her article, Kelly found that female doctors are often more likely to treat pain symptoms than male doctors, especially if the patient is female. So try finding a sympathetic female doctor who will listen to your concerns with true interest and respect.

4) Know that pain management and treatment is often a long term process - even if you find a doctor who is willing and eager to treat your pain, its important to know that pain management is not an exact science. Pain medications work differently in different people, and even if you are prescribed one, there is only a 40-60% chance that it will be the right one. Its important that you understand this when you are going into your doctors appointment and that you ask your doctor about the benefits and risks of different treatment options and leave with a plan already in place for the next treatment option you would like to try if your doctor's first attempt at treating your pain does not work. Try to be open to different types of treatment - antidepressants are often prescribed for chronic pain because they help block pain-communication pathways in your brain, so don't let the stigma of being prescribed an antidepressant deter you from giving that treatment option a try. While you are working with your doctor to find the right pain treatment for you, it is great to try out alternative pain relieving techniques, which can help you get by while you are waiting for a more formal treatment plan. Acupuncture, message, certain types of yoga, meditation, hypnosis and guided relaxation, biofeedback, and other relaxation techniques are often wonderful at treating pain without the need for medication and can teach you skills you can apply at home to help you treat your own pain.

I hope these tips will help you navigate the tricky process of pain management. Just remember that you deserve the best treatment possible and if you don't feel like you are getting that currently, you have the right to seek treatment elsewhere. Keep your chin up and know that there is a solution to your pain problems out there, you just have to have the perseverance to find it!

Hypermobility and GI Problems

2010-12-29T14:18:00.000-08:00

While joint pain is the most common symptom associated with Hypermobility Syndrome (HMS), HMS can affect other systems in the body. As a result, many HMS patients suffer from digestive problems - constipation, heart burn, diarrhea, IBS (irritable bowel syndrome), nausea, vomiting, delayed stomach emptying, Hiatal hernias, early satiety, and severe abdominal pain. These problems are all related to the underlying collagen defect responsible for HMS. Collagen is an important building block for all soft-tissue, and your GI track is one of the longest stretches of soft tissue in your body, which is an invitation for trouble. Just like in your joints, this collagen defect makes the connective tissues in your stomach stretchier than they should be, which makes it harder for your stomach to pass food. As Dr. Brad Tinkle explains in his book, Joint Hypermobility Handbook, "the increased laxity of the connective tissue [in the stomach] results in more difficulty for the muscles to contract. This results in the inability to close various valves throughout the gastrointestinal tract and difficulty pushing foods along for stooling" (p 121).

So, what can you do to treat these GI problems related to hypermobility? One trick is to decrease the amount of fat in your diet - when consuming dairy, choose items made from 2%, skim, or fat-free milk. Another trick is to eat smaller meals more frequently throughout the day - instead of eating a large breakfastt lunch and dinner, eat a small meal when you wake up, a follow-up snack an hour or two later, a light lunch, another snack around 4pm, and a lighter dinner later in the evening. Spacing your meals out like this should make your digestion easier and more regular.

For those suffering from severe, chronic stomach problems, it is extremely important to adopt a fiber and laxative regiment. I highly recommend reading Cure Constipation Now: A Doctor's Fiber Therapy to Cleanse and Heal by Dr. Wes Jones (available on Amazon.com). This book provides a simple plan for introducing fiber and laxatives into your diet, and it works wonders! My mom has suffered from horrible stomach problems her entire life, and after being on Dr. Jones' regiment for only a few months her IBS symptoms are almost entirely gone! It has been a real miracle for her - IBS symptoms have been interfering with her life, dictating what she can eat and what she can do for years. Now, with this fiber regiment, she is no longer a slave to her stomach. I too have tried it and am amazed by the results - I take fiber supplements (Benefiber and/or Metamucil), stool softeners (Ducolax), laxitives (Miralax), and Magnesium supplements every day. It may sound like a lot, but if you stick to the regimine, it will pay you back ten-fold. You should not start taking these supplements abruptly, however, but instead talk to your doctor or consult Dr. Jones' book to learn how to gradually introduce your body to these supplements. Also, if you are having severe stomach problems, it is always important to talk to your doctor to make sure they are not caused by a more serious condition like Colon cancer.

Hope that helps!
- Chelsea

Comments

2010-12-27T18:33:00.000-08:00

Hi everybody! I wanted to let you all know that I have really appreciated your comments, I love hearing your stories and suggestions for things you would like to see on the blog. I have, however, been having trouble accessing each poster's contact information, which means I have not been able to respond to many posts. If you have a question you would like answered, or just need some general advice, I would suggest contacting me directly via email at elsesmith@gmail.com. That way I can respond to you in a quicker, more personalized manner.
I can't wait to hear from you!

Love and Hope,
Chelsea

New Hypermobility App on iTunes

2010-12-15T14:46:00.000-08:00

Doctor Bradly Tinkle, author of two great books on Hypermobility Syndrome - Issues and Management of Joint Hypermobility, and Joint Hypermobility Handbook - has recently released an app on iTunes. The app is called 'JHDiagnosis' and can be found in the Utilities category in the app store - it is most easily found by simply searching for the word 'hypermobility' in iTunes.

This app takes you step by step through the Beighton Criteria, the most authoritative test used to diagnose generalized joint hypermobility or laxity. The app features pictures of each movement tested, an explanation of how the Beighton Criteria is used, and narration by Dr. Brad Tinkle himself. The app also includes movements not included on the official Beighton criteria, but which are extremely useful when trying to diagnose HMS.

I would highly recommend that every HMS patient download this app (compatible with iPhone and iPad and its free!) to learn a little bit more about the condition. Then, bring your iPod to work, school, or just out and about and test your coworkers, friends, and family to see if they may have one of the major symptoms of HMS (NOTE: This app cannot diagnose Hypermobility Syndrome, instead, it screens for generalized joint hypermobility, a symptom which can be caused by a number of connective tissue diseases, HMS being chief among these). Also, encourage your doctors - GPs, Physical Therapists, Orthopedists, OBGYNs, Rheumatologists - to download this app as well. This will not only help them understand you and your medical concerns more, but will also help them spot Hypermobility Syndrome in their other patients.

North Carolina Physical Therapy Clinics Interested in Treating HMS

2010-08-22T18:00:00.000-07:00

Below is a list of physical therapy clinics in North Carolina which are either knowledgeable about, or have experience treating patients with Hypermobility Syndrome. Physical therapy is by far the most effective way to treat HMS, however, it must be done properly. If you do exercises that are too strenuous, you can do more harm than good, but if you don’t do enough, then you won’t be able to effectively reduce pain. That’s why working with a PT who has experience with other HMS patients is great. If, however, you don’t live in North Carolina or are not near any of the clinics on this list, don’t worry – you don’t necessarily have to find a PT who is an expert at treating HMS (and honestly, there are only a few handful of those in the world), finding a therapist who will listen to your concerns and is willing to do some research about your condition can be just as good, if not better. When you start physical therapy, let your therapist know about your condition, and warn them that you shouldn’t be treated like an athlete - you should not be pushed to increase reps and weight at a fast paste, nor should you begin with rigorous exercises. Instead, let your therapist know that you need to be handled gently – start out with gentle exercises, increase reps slowly, and NEVER do anything that hurts. The things you should focus on with your PT are core strength, joint stability, and balance. Manual manipulations and chiropractic treatment should be kept to a minimum. Make sure that when you are doing exercises that you are not putting too much stress on your joints – if so, ask the therapist if there is another way to work the same muscle groups without having to strain your joints. Activities such as Pilates, swimming, and tai chi are also great ways to exercise that are kind on the joints and are things to talk about with your PT, who might incorporate some elements of those activities into your therapy.

Physical therapy is critical to managing the symptoms of Hypermobility Syndrome, but must be approached with caution. I saw a PT for months but ended up with more, rather than less pain – but I was told that was a good thing. The whole ‘no pain no gain’ mentality, however, does not apply to HMS. You need to find a therapist that is kind, attentive, and who will not push you too hard. It can be hard and sometimes take time to find a therapist like that, but it is well worth the wait. Before entering PT, do some shopping around so that you know you are going to get the right person and the right treatment.

Best of Luck! And remember, if you have any questions about physical therapy, or HMS in general, feel free to contact me.

Love and Hope,

Chelsea

MOUNTAIN PHYSICAL THERAPY SERVICES

90 SOUTHSIDE AVE, SUITE 225

ASHEVILLE, NC 28801

www.mountainphysicaltherapy.com

CAROLINA PHYSICAL THERAPY SPECIALISTS

PO BOX 408

Hope Mills, 28348

910-429-0600

TOUCH THERAPY SERVICES

PO Box 16518

Asheville, NC 28816

828-665-0442

touchtherapyservices@charter.net

KERNSERSVILLE REHAB SPECIALISTS

1031 E. Mountain Street Blv

Suite 101

Kernsersville, NC 27284

336-996-4980

www.KRStherapy.com

CENTER FOR ORTHOPEDIC PHYSICAL THERAPY

417 BILTMORE AVE.

5B DOCTORS PARK

ASHEVILLE, NC 28801

828-255-4567

copt@bellsouth.net

BALANCED PHYSICAL THERAPY

304 WEAVER STREET, SUITE 103

CARRBORO, NC 27510

and

HWY 54

DURHAM, NC 27713

www.BalancedPT.com

GASTON REHAB ASSOCIATES, INC.

1361-B E. GARNSON BLVD

GASTONIA, NC 28054

And: 2809 AMITY HILL RDSTATESVILLE, NC 28677

704-864-4424

www.gastonrehabassociates.com

BREAKTHROUGH PHYSICAL THERAPY

530 SANDHURST DR.

FAYETTEVILLE, NC 28304

910-483-9300

www.RedefiningPT.com

MOTIONWORKS MANUAL PHYSICAL THERAPY, INC.

1314-C WAYNE MEMORIAL DR.

GOLDSBORO,NC 27534

919-734-9644

SPORTS AND MORE PHYSICAL THERAPY, INC.

8300 HEALTH PARK SUITE 127

RALEIGH, NC 27615

919-319-6160

and

3700 NW CARY PARKWAY

CARY, NC

27513

919-319-3649

www.sportsandmorept.com

PHYSICAL THERAPY OF NORTH CAROLINA, LLC

4214 N. ROXBORO ST SUITE 100

DURHAM, NC 27704

919-479-9001

ptofnc@verizon.net

heelgrad93@msn.com

ATHLETIC ADVANTAGE, INC. SPORTS PHYSICAL THERAPY

5107 SOUTHPARK DR. SUITE 103

DURHAM, NC 27713

919-484-0033

www.AASports.com

ATLANTIC PT AND REHABILITATION

3650 COALITION DR.

MYRTLE BEACH, SC 29578

843-293-7713

MARTHA LEWIS PT

MILLS RIVER PHYSICAL THERAPY

4687 BOYLSTON HWY

MILLS RIVER, NC 28759

828-890-0040

lacy@millsriverpt.com

CENTER FOR PERFECT BALANCE

245 ROSMAN HWY

BREVARD, NC 28712

828-966-9036

perfectbalance@citcom.net

SKYLAND FAMILY REHAB

333 GASHES CREEK RD. SUITE A

ASHEVILLE, NC 28803

828-299-4636

skylandrehab@bellsouth.net

CAROLINA PHYSICAL THERAPY SPECIALISTS

905-B OLD WINSTON RD.

KERNERSVILLE, NC 27284

336-992-2787

www.carolina.com

CEDAR HILL PHYSICAL THERAPY

5409 WHITE BLOSSOM DR.

GREENSBORO, NC 27410

PAUL WEISS and ANGELA COOK

336-644-9661

info@cedarhillpt.com

www.cedarhillpt.com

ASHEVILLE PHYSICAL THERAPY

76 PEACHTREE RD., SUITE 204

ASHEVILLE, NC 28803

828-277-7547

kimfoxpt@gmail.com

brianLawLer@jno.com

CAPE FEAR FAMILY MEDICAL CARE

405 Owen Drive

Fayetteville, NC 28304

KATHERINE J. GRIESSER

910-263-7438

www.bodymechanics.com

Good News for HMS Research!

2010-01-18T11:15:00.000-08:00

The University of Michigan's Genetics department has recently teamed up with the Ehlers-Danlos National Foundation to create a 'comprehensive Hypermobility database.' To that affect, the University of Michigan is conducting a survey of Hypermobile patients now through March which focuses on 'their full range of symptoms, treatment efficacies, and experiences with the healthcare community.' This information is absolutely vital, as much of the scepticsm of the healthcare community towards HMS stems from a lack of sufficient data about how HMS impacts a patient's daily quality of life. The information collected in this survey will help to serve future generations of HMS patients, so please take the time to help out.

Anyone with EDS type III/HEDS/HMS/BJHS is welcome to take the survey (you do NOT have to be a member of the EDNF), and please encourage your hypermobile friends and familymembers to participate as well. Under the 'diagnosis' column please select 'other' and then enter 'Hypermobility Hope' if this website has helped you find answers to your diagnostic questions, it will be much appreciated!

The survey can be found here and takes about 45 mintues: http://www.ednf.org/index.php?option=com_content&task=view&id=1862&Itemid=88889153

Thumb Joint Pain

2009-12-23T08:47:00.000-08:00

Hypermobility Hope was recently recognized by ThumbJointPain.net in their Blog Award section, and was the featured blog for the week of December 21st, receiving 4.5 out of 5 stars! Please check out their website where you can tons of great information about relieving muscle pain from thumbs to shoulders to hips!

Love and Hope,
Chelsea

http://thumbjointpain.net/thumb_pain.php

Think You Might be Hypermobile? Take these Tests Below!

2009-12-21T13:04:00.000-08:00

TOP 5 HMS SYMPTOMS

1) Increased Flexibility – ‘double-jointedness,’ majority of joints extend past 180 degrees
2) Skin – extra soft, silky-smooth skin that is very thin, easily bruises and is slow to heal, scarring that is characteristically smoother in texture and lighter in color than surrounding skin
3) Frequent Injury – accident prone and clumsy, due to decreased proprioception (the body’s sense of its own movement through 3D space)
4) Anesthesia Problems – anesthetics (such as novicaine and lignocaine) take longer to take affect and ware off faster than normal
5) Joint Pain – frequent joint pain which does not respond to typical treatments such as ice, rest and anti-inflammatory medication, can be brought about suddenly without any direct injury or trauma and lasts longer than normal muscle inflammation

- If these symptoms sound familiar, it may be worth investigating Hypermobility Syndrome as a possible diagnosis to help explain your medical problems. Below you will find the Beigton Test and Brighton Criteria, the two medical tests used along with family history and certain exclusionary exams (such as Xrays, MRIs and blood work) in diagnosing HMS. First, use the Beighton Test to see if you have Generalized Joint Hypermobility, the primary symptom of HMS, which can be determined by a score of 4/9 or higher. Then, use the Brighton Criteria to asses whether or not HMS is the likely cause of this characteristic increased joint flexibility. Since HMS is a genetic disorder, it may be beneficial to have other family members perform these tests, or to at least have an accurate and detailed family health history at hand. If the Beigton Test and Brighton Criteria indicate that HMS is likely, it may be beneficial to talk to your doctor about Hypermobility Syndrome and Ehlers-Danlos.

*Arthralgia = joint pain

New Study into the Impact of Medical Prejudice on HMS Patients

2009-12-21T12:42:00.000-08:00

There is an excellent new study out this month in the Disability and Rehabilitation Journal, which investigates the effects disrespectful encounters with health care professionals has on Ehlers-Danlos and Hypermobility Syndrome patients. The study found that these negative experiences are not only psychologically and emotionally damaging, but can adversely influence the long-term health of such patients who are often hesitant to seek medical assistance. Below you can find an abstract of the study as well as a link to a newspaper article which provides another great overview. This study sets an excellent precedent for future investigations into the impact of Hypermobility on patients’ lives, as well as the consequences of the current hostility of the medical community towards the disorder.

DIGNITY NOT FULLY UPHELD WHEN SEEKING HEALTH CARE: EXPERIENCES EXPRESSED BY INDIVIDUALS SUFFERING FROM EHLERS-DANLOS SYNDROME

Berglund B, Anne-Cathrine M, Randers I. Nursing Division, Department of Medical and Surgical Gastroenterology, Karolinska University Hospital, SE-141 86 Stockholm, Sweden.

Aim - The principle of human dignity has assumed importance in ethics and constitutional law throughout the 20th century in the Western world. It calls for respect of each individual as unique, and of treating him or her as a subject, never as a mere object. As such, the principle constitutes an ethical cornerstone in health care. Patients suffering from Ehlers-Danlos syndrome (EDS) challenge medical care and knowledge in health-care professionals' as symptoms sometimes are vague. Individuals with this disorder have reported not being respected when seeking health care.

Purpose - To describe encounters in health-care situations when individuals suffering from EDS experienced that their dignity was not fully upheld. A further aim was to describe the long-term consequences of these experiences.

Method - A study-specific questionnaire was designed, where individuals with EDS described their encounters with health care from a personal perspective.

Results - After qualitative content analysis, the following five categories were identified: 'Being ignored and belittled by health-care professionals,' 'Being assigned psychological and/or psychiatric explanations', 'Being treated and considered merely as an object', 'Being trespassed in one's personal sphere' and 'Being suspected of family violence'. Consequences of these encounters were 'Mistrusting the physician' and 'Risking bad health'.

Conclusions - The memory of not being respected is substantial for individuals with EDS and can last for years. As a result, lack of trust for the health-care system is created and they may experience difficulties in future encounters with health care. Therefore, health-care professionals should base their actions on norms that protect human dignity and confirm each patient as a unique human being with resources and abilities to master their own life.

http://www.emaxhealth.com/1275/39/34644/ehlers-danlos-syndrome-patients-seek-respect-understanding.html

New HMS Article

2009-12-19T10:02:00.000-08:00

Please check out my new article in the Daily Tar Heel, the University of North Carolina at Chapel Hill's on-campus newspaper. It is more of a human interest piece than about awareness, but being able to put a face to the disease is extremely useful in getting people to care. Please read and pass along the hope to others!
http://www.dailytarheel.com/content/student-learns-be-flexible

Hypermobility Myths Dispelled

2009-09-25T09:30:00.000-07:00

There is so much material out there detailing every possible facet of Hypermobility Syndrome, so why, one may ask, have most Doctor's not heard of it and/or continue to ignore it? There are four major reasons why HMS is not taken seriously, but as you will see, all of these reasons can be easily dismissed. There is no excuse to be ignorant of HMS, it is a real threat affecting the lives of hundreds of thousands of individuals, so its time to do something about it – lets start spreading awareness and hope!

1) Myth - Hypermobility is a benign condition, so there is no incentive to diagnose it.
Truth - When it was first identified in medical literature in the late 1960s, Hypermobility Syndrome was named 'Benign Joint Hypermobility,' because it was first noticed in patients who were 'double-jointed' but otherwise appeared to be perfectly healthy. Numerous studies in more recent years have linked HMS to very serious disorders such as fibromyalgia and osteoarthritis in addition to its threat of debilitating joint pain. Despite all the evidence to the contrary, Hypermobility is still considered as more of an oddity, a parlor trick, than a serious medical condition.
When I was struggling with finding a diagnosis, I visited the Emory Hospital Pain Management Center in Atlanta, GA which is one of the most prestigious pain management centers in the south east. I was seen by Dr. Baumann, who was named one of Atlanta's Top Doctors for pain management in the August, 2001 edition of Atlanta Magazine. When I arrived I had already received a positive diagnosis of hypermobility, but when I mentioned this, the doctor immediately dismissed it. She told me that I had 'myofacial pain syndrome' (which is just a medical descriptive term, rather than diagnosis, of generalized muscle pain). She told me that there was nothing that could be done, and that I would 'just have to learn to deal with my condition, like the way a person with diabetes or obesity manages their condition.' This comment illustrates that even medical professionals at the top of their fields can be ignorant of the fact that Hypermobility severely affects the health and lives of many individuals. If Dr. Baumann, an award winning doctor, is misbelieving of the significance of HMS, what can be expected of the average family doctor, pain management specialist or rheumatologist?

2) Myth - Since Hypermobility Syndrome is a genetic disorder and therefore cannot be ‘cured’ either through surgery or pharmaceutical treatments, there is no money to be made in researching or treating the disorder.
Fact – The misdiagnosis of patients with Hypermobility Syndrome costs hospitals, doctors, and insurance companies countless dollars due to unnecessary tests and being sent to superfluous specialists. Most Hypermobile patients are bounced from doctor to doctor, and specialist to specialist for months, and sometimes years on end in search of relief. In the process they often miss work and have very costly yet unnecessary tests done, draining the money and time from an already overloaded medical system. If doctors and specialists were to diagnose HMS proactively, they could save their patients from pain and save themselves money in the process. In just five minutes, with a simple test of a patient’s flexibility and family as well as personal health histories, a definitive diagnosis can be made without costing a dime!

3) Myth – Hypermobility Syndrome is just a Women’s Issue. These healthy young girls complaining of inexplicable pain just need to toughen up and stop whining!
Truth – while HMS affects 5% of the female population, it affects men as well. Patients should be respected and trusted, it takes courage to ask for help and terrifying to be admonished by the very people who are supposed to help you. Also, another problem is that many girls with Hypermobility perceive the disorder as an asset rather than a liability. Hypermobility causes certain ‘feminine’ body characteristics, such as silky-smooth porcelain skin, elongated and dainty limbs and digits, and tiny wrists and ankles. Also, the natural flexibility that Hypermobility causes makes many girls highly desired by gymnastics, cheerleading, and dancing coaches, activities in which they excel do to the competitive edge their inherent flexibility and range gives them.

4) Myth – Hypermobility is so rare, one could not really be expected to have heard of it, let alone be able to recognize it in others.
Truth – HMS is extremely common, Ehlers-Danlos Syndrome Type III Hypermobility Syndrome alone affects 5% of all women! Almost all of us has at least one ‘double-jointed’ friend who has freaked us out or entertained us with their abnormal flexibility and contortions. Also, many models and actors/actresses are Hypermobile too – two of the affects of the collagen imbalance that causes HMS are silky-smooth skin and long, lean, dainty limbs, which gives them a natural advantage on the red carpet. See the pictures below of celebrities who are hypermobile and may not even know it!

Below - Allison Harvard (Runner-up America's Next Top Model Cycle 12), Jessica Stroup (Actress 90210), Demi Lovato (Actress Camp Rock, Singer), Meaghan Jette-Martin (Actress 10 Things I Hate About You TV), Kevin Jonas (Musician The Jonas Brothers), Taylor Mommsen (Actress Gossip Girl), Mary-Louis Parker (Actress Weeds), Jennette McCurdy (Actress iCarly), Ashley Argota (Actress True Jackson), David Caruso (Actor CSI:Miami)

A Call for Awareness

2009-09-25T09:28:00.000-07:00

There is a pathological family that falls under the discipline of Rheumatology, known as Heritable Disorders of the Connective Tissues. The individual pathologies vary widely in both severity and symptoms, and include disorders such as Marfan Syndrome (MFS), Ehlers-Danlos Syndrome (EDS), and Osteogenesis Imperfecta (OI). One symptom that each of these disorders present with is joint hypermobility. It is advantageous for rheumatologist, then, to look for joint hypermobility first in all of their patients; if the patient does not display hypermobility then the doctor can safely rule out an entire class of disorders, and if the patient is in fact found to be hypermobile, the doctor will have a very specific idea of what is wrong and the course of treatment needed. The current methods employed to diagnose disorders such as MFS, EDS, and OI are both costly and time consuming – many tests such as blood work, X-rays and MRIs must be repeated to look at tighter and tighter parameters. Since the cause of this family of disorders is genetic, skin biopsies are often needed to make a definitive diagnosis, a process which is costly for both patient and doctor. There is, however, a much more efficient and expedient way to make diagnoses for these disorders; upon determining that a patient is hypermobile, a thorough family and personal health history, which requires no tests and costs no money, can provide enough data to come to a decisive and final diagnosis, without the need of costly tests or wasting any time. The diagnosis of Connective Tissue Disorders today is one of a process of elimination, one that proves costly and grossly inefficient. Ehlers-Danlos Syndrome Type III alone affects 5% of the female population, causing an immense drain on the healthcare and insurance systems as patients are bounced from doctor to doctor and from test to test for months, or in many cases, years. The manpower and money that could be saved by being proactive rather than reactive in the diagnoses of Connective Tissue Disorders is immeasurable.

The strain caused by these disorders is not only felt in the healthcare or insurance communities, but in the workforce and social services as well. Studies show that Connective Tissue Disorders have a significant impact on the quality of life of patients; many are unable to work full-time or consistently, causing many patients who have been unable to receive a diagnosis or reliable treatment plan to rely on the unemployment system and other social services in order to provide for themselves and their families. Once again, the scale of the amount of tax dollars that could be saved by being proactive and spreading awareness about hypermobility in the medical community is impressive. Not only would a focus on diagnosing hypermobility save countless dollars from being spent, but the test for hypermobility itself costs absolutely nothing to perform. The test for hypermobility is known as the Beighton Scale and/or the Brighton Criteria. These tests require the patient to perform a series of movements with various joints to see if/how many of those joints exhibit abnormal or increased mobility. This test takes less than 5 minutes to perform, does no harm to the patient, and costs nothing to perform for the doctor and insurance companies. In 5 minutes more can be determined in the diagnosis and prognosis of a patient than can be learned in months or years given the current stratagem of the medical community. In the same vein, the quicker that a diagnosis can be reached, the shorter the recovery time for the patient, and the faster they can go back to their lives and rejoin the workforce – saving countless dollars in insurance bills, treatments, and unemployment and/or workers compensation payments. Furthermore, as Connective Tissue Disorders are heritable pathologies, so once one member of a family is given a diagnosis, it makes the diagnosis of other family members exponentially easier, ensuring that the healthcare system will not be burdened with subsequent generations of patients, as preventative actions can be taken.

Once again, the medical community has nothing to lose and everything to gain by shifting its focus towards the diagnosis of hypermobility in patients. Rheumatologists are not the only healthcare professionals who could benefit from testing for hypermobility; a vast majority of patients suffering from Connective Tissue Disorders are seen by orthopedists, gynecologists, physical therapists, psychologists, and pain specialists. The burden placed on the healthcare system, then, is as wide reaching as it is heavy. A recent study shows that less than 10% of hypermobile patients are correctly diagnosed by their primary care physicians. Incorporating a discussion of hypermobility into the common curriculum for general practitioners, then, would be hugely beneficial; the testing for hypermobility requires neither any testing equipment nor specialized expertise, therefore, general practitioners could easily screen for the trait, preventing patients from having to seek the care of specialists in the first place.

In conclusion, screenings for hypermobility would be easy and costless to implement, and would be immeasurably beneficial. Such screenings would eliminate wasted time, money, and testing by doctors and hospitals, and relieve strains on insurance companies, as well as unemployment, disability pay, and other social services that demand a high amount of manpower as well as tax dollars. Hypermobility may at first appear as an insignificant phenomenon, and one that is easily overlooked; however, it is just as easy to recognize as it is to ignore, and once spotted, its benefits are as far reaching and immediate as they are drastic.

Chelsea Smith

Hypermobility Hope

Dr. Brad Tinkle

2009-09-04T19:38:00.000-07:00

I had the distinct pleasure of having Dr. Brad Tinkle comment on my recent article out in the Fayetteville Observer. Brad Tinkle is arguably the most influential advocate for Hypermobility Syndrome in the US today. His book, Issues and Management of Joint Hypermobility: A Guide for the Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome, is extremely helpful - it details a wide range of symptoms related to the disorder, and personally helped me make sense of my medical history - and is availible at Amazon.com. Please read his post, below.

Brad T Tinkle, MD PhD - Chelsea's story is a sad one indeed and unfortunately repeated everywhere too many times. We need to advise healthcare workers and families that joint hypermobility is not a "gift" to be exploited especially in young athletes, and we had better work towards making a long term difference in their lives by re-teaching those to use their joints within normal limits.
The Ehlers-Danlos National Foundation recognizes the hypermobility syndrome as the same as the hypermobile type of Ehlers-Danlos syndrome and would welcome those seeking support or information.

Contact

2009-08-31T11:46:00.000-07:00

If you have questions about your personal health in either diagnosing or dealing with Hypermobility Syndrome, please feel free to email me at elsesmith@gmail.com. I would be happy to answer any questions I can, give referrals to doctors, and send information packets to those who are interested.
As always, love and hope,

Chelsea Smith

New Article on HMS in the Fayetteville Observer

2009-08-31T07:42:00.000-07:00

Hi everyone! Please check out my new health feature article on Hypermobility Syndrome in the Fayetteville Observer! The journalist did an excellent job of conveying a message of awareness, empowerment and hope. The photographer at the interview realized she was hypermobile during the shoot, so it was a great experience for all involved!
If you are new to the site, welcome! Hypermobility Hope is here to be your one-stop-shop for information on hypermobility - scroll through the articles, videos, and links to get a better sense of weather or not you are hypermobile or how to deal with the disorder if you have already been diagnosed.
Love and Hope,

Chelsea

http://www.fayobserver.com/Articles/2009/08/31/926002

Twitter

2009-08-17T09:10:00.000-07:00

Hypermobility Hope is now on Twitter! Follow my tweets as I write about what it is really like to be living with HMS - the daily obstacles faced, the stress of not knowing what to expect from your body, and the hope of knowing that Hypermobility is what makes us strong enough to tackle any of the hurtles thrown our way.
While there is much academic research about Hypermobility out there, there is a shocking lack of studies on how HMS affects patients' quality of life and interefers with thier daily lives. In the hopes of helping to somewhat fill this whole, I will be reporting as much as I can as honestly as I can about the physical and emotional burdens imposed by HMS.
Please encourage others with HMS or who think they might have the disorder to join the discussion! Together we can bring eachother hope and solutions!
Love and Hope,

Chelsea

www.twitter.com/HMShopenow

Hypermobility in the Lower Body - Pictures

2009-08-10T09:01:00.000-07:00

Below you will find pictures that demonstrate Hypermobility in the lower body, mainly the legs, and in posture. The red lines represent the proper/correct/natural alignment the body should have - in other words, the red line should intersect three joints, for example, the ankle, knee, and hip. On the left hand side you will see an example of correct alignment, as would be found with a non-Hypermobile person. On the right you will see an example of improper alignment, as would be found in a Hypermobile person. You can see that on the right hand side, the red line only intersects two joints, namely the ankle and hip. This occurs because the third joint, mainly the knee, is being extended past its intended range of motion (past 180 degrees).

There are two main explanations for this phenomenon. First, the body of a Hypermobile person is different from that of non-Hypermobile people because their connective tissues have a different genetic makeup, which allows joints to have a greater range of motion, extending further than they are intended to do. In other words, the first explanation is a genetic one. The second reason is a bit more subtle, and it has to do with the way in which the brain perceives the physical body. Humans have a sense called proprioception, which is the perception of one's physical body in space. This sense allows us to navigate through 3D space accurately, knowing instinctively where each point of our body is at any given moment. For example, if you were to ask your friend to close their eyes, and then moved their hand to the left 4 inches, your friend would know exactly where their arm was without opening their eyes (even if they can't tell you 4 inches, that is irrelevant - the brain knows where the arm is). If you were to do this to a Hypermobile person, you would have less accurate results; this is because, for whatever reason, people with Hypermobility have an impaired or diminished sense of proprioception. This causes them to be more clumsy, accident prone, and/or uncoordinated. For example, it is common for a Hypermobile person to run into the corner of a coffee table with their knee, despite the fact that they walk past that exact same coffee table in the exact same position every day. For a non-Hypermobile person with perfect proprioception, the brain has a correct vision of space and location, allowing them to navigate around the coffee table without having to think about it. That is another important point about proprioception, it is not an active sense like vision or taste, but an inactive or instinctual sense - you don't have to think about proprioception, or 'turn it on,' your body just does it. Therefore, the hypermobile person is not aware of thier incorrect posture or the overextension of thier joints - that is just how thier body naturally functions. In other words, the brains of both the Hypermobile and non-Hypermobile person, think they are standing in the same position and in the same alignment; it is the Hypermobile person's impaired sense of proprioception coupled with thier innate flexibility that leads to the marked difference between the two.

Hypermobility in the Upper Body - Photographs

2009-07-25T13:54:00.000-07:00

Below you will find photographs illustrating the most common hypermobile joints in the upper body - the fingers, wrist, elbow, and shoulders. The positions/movements shown are commonly referred to as "double-jointedness." The ability to perform these movements is a sign of joint hypermobility and connective tissue laxity.

HMS Health Alert Feature

2009-06-09T10:20:00.000-07:00

I recently contacted my local ABC station, and they were gracious enough to agree to do a story about HMS and my personal struggle with the disorder. Please watch and encourage others to do the same - the single most important thing you can do to help HMS patients is to help spread awareness!
Thank you all for your support!
http://www.wlos.com/newsroom/health_alert/vid_232.shtml

HMS and the Brain

2009-05-19T12:35:00.000-07:00

Neuro-feedback

2009-05-18T15:30:00.000-07:00

Below is a transcription of a conversation I had with Dr. Ed Hamlin, of the Pisgah Institute, concerning my neuro-biofeedback treatment. I had a procedure called a QEEG performed, where my head was fitted with sensors, and I was asked to do different tasks such as reading, doing math, sitting with my eyes opened and eyes closed, and the computer mapped the electrical impulses in my brain. Neuro-biofeedback is a treatment that teaches your brain to make new pathways and nerve connections that promote positive behavior. The therapist places two sensors on different part of the brain, and the patient is asked to look at a computer screen which has a video game similar to Pac Man on it. When the two spots of the brain under the sensors begin to ‘talk to eachother,’ the Pac Man moves on the computer screen. The therapist can adjust what the sensors are looking for, and thus can control what type of energy (theta, beta waves, etc.) your brain produces. Your brain is hardwired to want to win the Pac Man game, and so it teaches itself to produce the sort of activity which the therapist is looking for. Basically neuro-biofeedback is about enforcing and reinforcing your brain to practice efficient neuro-pathways, and discourage any sort of electrical imbalance in the brain that may lead to negative behavior.

* * * * * * *

There are 2 parts of the QEEG
1= a medical part (neurologist looks at tracings)
2= quantitative part, see what its made up of

Each line is a location in your head (eyes closed/open, etc.) and each sensor represents hundreds of thousands of brain cells, and they all are not firing at same rate, so each line is an average, but we want to see what that average is made up of, so we put filters on to see a specific range.

Medical part – clinical neuropsychologists analyze your brain, this should be part of your primary care record so you have something to compare to if you were to have a head injury, but they did not see anything that looked like a ‘medical disorder of the brain’

Quantitative part – of the 19 lines there is one that is different (darker = busier = brain cells firing more rapidly) at T4 above right jaw. If someone has a pain disorder there is a natural tendency to clench the jaw, this is a common finding when there is a chronic pain condition, and it feeds back into the pain center in the brain, priming it up to be on the lookout for pain stimuli.

Pain works by kindling – if something is triggered in the brain, like pain, the cells will fire more rapidly, and when they do so they form connections with each other. ‘Brain cells that fire together, wire together.’ The more of this kindling that is done, the lesser the stimulus needed to trigger a reaction. Everything else looks the way that it should.

Looking at a frequency distribution graph, 0-25 hertz, of all the brain cells that are firing 1x or 2x or 3x etc., when eyes are closed there should be peak in back of the head. 10 hertz is the idling frequency of the brain, when the brain cells are not doing something, but they are ready to do something. When you open your eyes you want that peak to drop. Despite all this, there is a point in back of your head that didn’t change between eyes closed and opened; that is ‘very rare’ and ‘very interesting.’ This is what is called a Rogue Spot, and is very unusual. The area where your Rogue Spot is, is that area where your internal representation of your body resides (that would be consistent with a stroke victim), and is also the area where your senses come together. I think the pain has really messed that area up, and it being messed up feeds back into the pain, so you get a repetitive loop of pain. This is medically normal, however, and the primary importance of knowing this is in prescribing the neuro-feedback. What we will do is, hook this rogue spot up to its partner on the other side of the brain and make the two cooperate together so the rogue site has to do more of what the normal site is doing. The rogue spot doesn’t want to be bad, it is just stuck, and I think the pain set this up, not visa versa.

On this next graph, the places that are blue are doing too little (i.e. not firing enough/frequently), and those that are red are doing too much (i.e. firing too much/too rapidly). Here we see a low level of 18-28 hertz on the left front side, and this tells me the stuff we are looking at, that rogue spot, is causing problems with concentration, focus, verbal processing, and short term memory (this would significantly interfere with school). This is purely a consequence of the rogue spot which is sucking up energy from other areas of the brain, and that left side stuff is seen as expendable to the brain.

The brain is like a walnut with two hemispheres, and there is an area in the middle of the fold, connecting the two sides together. We are seeing blue right there in the middle, which is the area of your brain which allows you to shift between different energy level activities (relaxing to having a conversation to driving in a car). This means your brain is not shifting the way it should, which again would make something like school extremely difficult.

Now looking at high frequency, overactive stuff, we are seeing shading towards the right side of your brain. The right side of the brain, is the side that lets us know something is wrong, even before the left side of our brain can figure out what that thing is. The right side of your brain is really overactive, so left side stuff is underactive because of this.

Now we have a graph of one head with little heads inside it. This is measuring how the brain is talking to itself, i.e. how well one spot talking to all the other spots, or the phase symmetry. The rogue spot is really out of step with the rest of the brain, and it sends ripples through the whole system, so we are seeing less symmetry between the spots than we would expect

In conclusion, your brain is intensifying the pain, so the pain perceived is more than the pain stimulus. Our goal is not to get your brain where it doesn’t respond to pain, but where it is not responding too much to pain. We need to get the area that is stuck unstuck, and that will take away the pain that the brain is adding on top of the physically triggered pain.

My Story

2009-05-14T18:03:00.000-07:00

This time last year I was backpacking through Europe with friends, but now I wake up every morning and have to fight just to be able to walk; and I have to deal with the fact that I may never again get to be that free-spirited girl who was out conquering the world not so long ago.

Last September, in the span of one week, I went from being a happy, healthy college student to being bedridden with crippling back pain. The sudden onset of my symptoms did not correspond with any sort of trauma – I simply woke up one morning with hip pain. The next day my hip hurt so much I couldn’t walk, and by the end of the week the pain had spread to my back, and I was completely incapacitated, unable to get out of bed. My doctors at school sent me home for two weeks of bed rest, and were confident that because I was young and otherwise healthy, I would bounce back. But over those two weeks I got worse, not better, and my family and I began to realize that we were dealing with something very serious.

I was in a state of constant, unbearable pain for six months, and saw seven different doctors, but not one of them could find anything wrong. Every blood test, MRI, and X-Ray came up clean, and everything else about me appeared healthy and strong. Soon these doctors started telling me my pain was psychosomatic, they suggested there was something wrong with the way my brain processed pain, and some told me I simply had to “surrender” to the pain and “deal with it.” The only thing they could all agree on was that there was nothing they could do to make me any better. I was told that school, a career, family, were all things I should not think about anymore; this was my life. Pain was my life now.

On February 20^th, I experienced the most excruciating pain of my life. Even after 6 months of coping with constant, unbearable pain, I was unable to handle the magnitude of the torture. I spent hours writhing in my bed, trying every stretch, every breathing exercise and mind game I could think of, but nothing helped, the pain was too great. I fought as hard as I could but the pain engulfed my entire being, there was not a millimeter of my body that was not racked with pain, and not a nanosecond of relief. Words cannot describe the magnitude of the pain, my own brain could not process the torture, I was completely overtaken. I finally broke down, unable to fight the torment any longer. I began screaming and crying, begging for it to stop, wishing for anything to end the pain; but of course there was no stop button, no end. My mom finally had to feed me a fistful of pills – Oxycodone, Lyrica, Elavil, Flexaril, Motrin – and wait for me to drift off into a drugged stupor. After 45 minutes in Hell, my mind fogged over, but even then the pain was not fully gone, and I fell into a fitful sleep, being awakened by pain and then pulled back to unconsciousness by the drugs. THIS is what the doctors told me I should “manage,” THIS is what they told me to “deal with,” THIS is what the doctors accused me of making up. There is nowhere lower than that, no pain more consuming, no hopelessness more draining. But I survived. All those doctors told me I was weak, but in that moment I knew that I was strong; stronger than they could ever reach, stronger than they could even imagine.

In a final, last-ditch effort, my primary care physician suggested that I might be hypermobile, and sent me to a specialized physical therapist. The only reason she knew about hypermobility, was that she herself suffers from hypermobility syndrome, and has first-hand knowledge of just how painful it can be. The physical therapist confirmed that I was hypermobile after having me do a series of maneuvers, a test that lasted less than five minutes. She determined that my SI hip joint was lax, and moved in and out of socket as I walked, causing one leg to be shorter then the other, throwing my entire back out of alignment. We began a series of special, stabilizing exercises, and I finally, slowly I began to gain relief. By March 28^th, just one month after my lowest point and darkest hour, I was flying up to Washington, DC to interview for an internship. Granted, I was not skipping, or carrying my bags, or completely pain-free, but I did it. It was not fun, it was not easy, but I succeeded. This normalcy would have been unfathomable a month ago, yet here I was, my life was finally starting to come back.

Despite all this trauma, suffering, and physical as well as emotional torture, I feel lucky. I am so blessed. Less than 10% of people suffering from Hypermobility Syndrome are diagnosed by their primary care physicians. The vast majority never receive a proper diagnosis, and even if they do, it is often too late – they have ruined their lives with alcohol, drugs, and depression. And I don’t blame them, there is no choice; when your options are pain or numbness, and the pain is completely engulfing, you must choose numbness because the pain is too impossible a burden. I feel so grateful and lucky that I finally found a physician who said, ‘yes, I can help you.” I am grateful that I had a supportive family who believed in me and fought with me, even when everyone told us to surrender. And I am so lucky that I had the resources to see so many doctors and receive so many treatments. I was a ghost, I was in Hell. And if it weren’t for my family, I would still be there.

The medical community has been biased against Hypermobility Syndrome (HMS) since its first appearance in medical journals in the late sixties. It was deemed “benign joint hypermobility,” since patients appear to be healthy apart from the increased range of motion in their joints. Despite being linked to serious conditions such as fibomyalgia, osteoarthritis, and pregnancy complications, the stigma of benignity has endured. The answer may lie in the fact that HMS occurs in women 5.5 times more than it does in men, and that when females complain of otherwise inexplicable pain, doctors simply view them as weak and unable to handle pain that would not bother a man. In a New Zealand study of 59 men and 43 women presenting with back pain, not one male was found to be hypermobile, and the causes of their pain were straight-forward orthopedic conditions such as slipped disks or spondylolysis. Of the women, only half were diagnosed with similar conditions, and the majority of the remainder were found to be hypermobile. Studies such as this not only suggest that hypermobility is more serious and common than most doctors believe, but also demonstrate that when patients present with symptoms of sever joint pain, men and women should be considered differently. HMS is a major women’s health issue, yet most doctors know little about it, and it is time that changed.

One of the most frustrating parts of this experience has been the lack of information. As a patient, I had to fight for myself, I had to research and dig to find information that my doctors were not giving me. If I had listened to them, my life would be over. But I fought, and though it has proven worthwhile, it has been a lonely road. There are no support groups, no open dialogues about the disorder, which means I have no guidelines to help me figure out how to live my life. I have learned that as a hypermobile person, I have to do things differently; I have to plan and anticipate each move, and there are some motions I can no longer do, and I have to figure out on my own how to get around that obstacle and do it my way. I have no idea what I can expect from my future – there is no one to look up to or talk to in order to help me figure out how my HMS will affect my work and my family life. Do I tell my boss about HMS, would he even understand, or do I keep it a secret and hope that he does not find my inability to sit still for more then 10 minutes or my short attention span on painful days detract from my job performance? Should I have kids if I have a 50% chance of passing HMS onto them, and even if I adopt, how will I care for my children on those days I am in so much pain I cannot even take care of myself? I have no idea how to begin to answer these questions, and that is frightening, the solitude is crippling.

Another hard thing to swallow is that there is no “getting back to normal” for me. This experience has changed me irrevocably. I have to accept that some things I used to do, and loved to do, are things that will forever be off limits for me. I can’t go for a run, I can’t play soccer or field hockey, and I can’t do cartwheels through the quad just because I feel like it. I can’t give my nieces piggyback rides, I can’t play in the Marching Band, I can’t carry heavy books or sit in the library for 5 hours, and I can’t do yoga. It is hard to know who you are, and not loose parts of yourself, when these things are stripped away. Can I still be me if I can’t wrestle with my nieces, or run around being crazy with my friends? I feel at times like I am a prisoner in my body, like there is a constant struggle between how I want to be and how I can be.

Some people say that everything happens for a reason, and I used to be one of those people. I don’t believe that anymore. I cannot accept that someone did this to me in order to teach me a lesson; that would mean the universe is far crueler than I would ever want to accept. What I do believe is that every situation has the potential to become meaningful if we consciously make the choice to make it matter. And so although nothing could ever undo the horrible pain I endured, and will continue to endure, if I can make this experience meaningful by helping even just one other person to avoid a similar situation, then maybe, just maybe, it will be worth it in the end.

Love and hope,

Chelsea

HMS Research

2009-04-21T14:11:00.000-07:00

Hi Everyone! Here is a list of current-ish research being conducted about HMS, which I copied from the Hypermobility Syndrome Association website. That page, www.hypermobility.org is a great resources, however, it is catered towards the UK rather than the US. Enjoy!

Latest Research

Prof Rodney Grahame & Dr Alan Hakim
Department of Rheumatology, University College Hospitals, London

1. Do you have HMS?

We have been working on a 5-part questionnaire to identify hypermobility. If you answer yes to at least 2 of the 5 questions then there is an 80-90% chance you are hypermobile. We hope it will become a simple aide memoire for doctors and allied health practitioners, encouraging them to think about hypermobility as the cause for musculoskeletal problems.

The questions are:

Can you now (or could you ever) place your hands flat on the floor without bending your knees?
Can you now (or could you ever) bend your thumb back to touch your forearm?
As a child did you amuse your friends by contorting your body into strange shapes or could you do the splits?
As a child or teenager did your shoulder or knee cap dislocate on more than one occassion?
Do you consider yourself to be double-jointed?

Ref: Hakim AJ, Graham R. A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain.
International Journal of Clinical Practice 2003; volume 57: p163-166

2. HMS and local anaesthesia

In our survey of questions for the 5-part questionnaire we also asked about local anaesthetics as there has been work published suggesting that patients with joint hypermobility syndromes may be more resistent to them. We found that people with Benign Joint Hypermobility Syndrome are three times more likely than non-hypermobile individuals to have noticed that either local anaesthetic did not work at all or that they needed to be given more than usual to gain any benefit. We do not know the mechanism behind this but clearly it is an important message to get accross to colleagues in both hospital and general practice as well as dentistry.

3. Vascular problems and HMS

There has been some interesting work done recently looking at the many other non-musculoskeletal symptoms experienced by those with benign joint hypermobility syndrome. In particular people often complain of vascular problems of feeling faint, actual fainting or palpitations. A lot of people also experience gastrointestinal symptoms such as heartburn and irregular bowel habit. In our clinic we found that up to 60% of our patients have these kinds of symptoms on top of the more classic ones of widespread pain, anxiety and fatigue.

Dr Gazit and colleagues from Israel have done some important work that suggests that these kinds of vascular symptoms may be the result of poor functioning of the autonomic nervous system. This is a part of the nervous system that we do not have specific control over. It is responsible, in part, for maintaining our blood pressure and pulse when we move from lying to standing, or increase our level of activity. For some people the autonomic nervous system does not work as well as it should and their blood pressure falls and their pulse rises when they stand up. This makes them feel dizzy and experience 'palpitations', possibly even chest tightness and shortness of breath. Prof Grahame and I, with colleagues from the National Hospital for Neurological Diseases, Queen Square, are looking at this in more detail at present.

Ref. Hakim AJ, Grahame R. Symptoms of autonomic nervous system dysfunction in the benign joint hypermobility syndrome. Rheumatology (Oxford) 2003; Volume 42 supplement: Abstract number 47.

4. Other projects that are underway or about to start are:

A study looking at the association between hypermobility and complications of pregnancy, particularly rapid delivery and tissue damage in labour.
A study exploring the benefits and complications of conservative and surgical management of orthopeadic problems in the shoulder and knee in patients with benign joint hypermobility syndrome.

5. Finally a book on the Hypermobility Syndrome...

...'Hypermobility Syndrome - Recognition and Management for Physiotherapists' by Prof Grahame and Rosemary Keer was published on 3 June 2003. Although it is written for physiotherapists, it would still be very accessible to the 'un-medical'.

(Editors note: 'Hypermobility Syndrome - Recognition and Management for Physiotherapists' by Prof Grahame and Rosemary Keer is available from Amazon for £32.39)

Latest Research in Scotland - Prof W Ferrell

Professor William Ferrell was awarded a grant from the Arthritis Research Campaign to compare the differences in muscle reflexes and the sensations originating from proprioceptors - sensory nerve endings which feed back information about muscles and tendons to the brain in individuals with HMS.

10 Quick HMS Facts

2009-04-19T20:40:00.000-07:00

10 Quick HMS Diagnostic Questions

2009-04-19T16:29:00.000-07:00

10 HMS Facts

2009-04-17T10:16:00.000-07:00

10 Quick HMS Facts

1. Hypermobility Syndrome is a genetic collagen defect most people don’t even know they have, that weakens connective tissue around joints
2. HMS is a common cause of unexplained joint pain, but is diagnosed in less than 10% of sufferers who see their primary care physicians
3. HMS is up to 5.5 times more likely to occur in women than in men, and is also more common in people of Asian, African, and Middle Eastern decent
4. HMS symptoms can manifest at any age, from age 3 to 70, and its onset usually does not coincide with any direct accident or trauma
5. HMS symptoms can last anywhere from 15 days to 45 years
6. HMS can predispose sufferers, especially women, to fibromyalgia and osteoarthritis
7. HMS can cause serious pregnancy complications, including premature birth, rapid labor, and uterine prolapse
8. People with HMS have diminished proprioception, or sense of their bodies in space, which increases the likelihood of joint injury
9. HMS patients do not experience the full anaesthetic affect of lignocaine, a drug used in dental work, minor surgery, and epidurals
10. There is currently no consensus as to how HMS should be diagnosed, and no proven treatment plan

10 Diagnostic Questions

2009-04-17T10:14:00.000-07:00

10 Quick Diagnostic HMS Questions

1. Does one or more of your joints have a greater range of motion than most people’s

2. As a kid, did you ever entertain your friends by contorting your body in weird ways or by doing the splits?

3. Do you consider yourself ‘double-jointed,’ and/or does your family have a history of ‘double-jointedness?’

4. Is their a history of rectal, mitral valve, or uterine prolapse or hernias in your family?

5. Have you ever suffered unexplained joint pain, or joint pain that started out of the blue, aka was not due to any direct injury or trauma?

6. Have you ever failed to receive relief from inflammation related treatments, such as nonsteroidal anti-inflammatory drugs (NSAIDs) or ice?

7. Does stability, such as stabilizing exercises or stabilizing tape or braces, help your joint pain?

8. Do you or your family have a history of thin, fragile, or silky smooth skin, easy bruising, or varicose veins?

9. Have you ever had to ask your dentist or doctor for more lignociane during dental work or minor surgery, because you were not feeling the full anaesthetic effect?

10. Have you or any of the women in your family experienced a flair up of joint pain during or immediately after pregnancy?

- If you answered ‘yes’ to three or more of the above questions, you may want to talk to your doctor about Hypermobility Syndrome

What You Need to Know - Scholarly Articles, Batch #2

2009-04-16T13:28:00.000-07:00

"Hypermobility Syndrome" L. Russek (Physical Therapy Journal) 1999
• “HMS has been given relatively little attention in the literature. Most reports are in the rheumatology literature, with virtually none in the orthopedic or physical therapy literatures.”
• HMS patients, however, usually see orthopedists or physical therapists for acute joint pain, and are treated without the underlying problem, aka their HMS, ever being acknowledged.
• “Because HMS lacks a definitive pharmacological or surgical treatment, physicians may have perceived little benefit in its diagnosis.”
• “Individuals may be improperly identified as having hypochondria, as malingering, or as having nonspecific chronic pain, without further investigation into the source of their complaints … Failure to recognize the underlying HMS may lead to unnecessary or inappropriate diagnostic studies, surgical procedures, and patient management.”
• “HMS may be from 1.1 times to 5.5 times more prevalent among females than among men. Hypermobility Syndrome is also more prevalent among Asians than among Africans, ant it is more prevalent in Africans than among Caucasians.”
• “There are neither agreed on criteria nor agreed on scores for the diagnosis of HMS.”
• “Easy bruising and poor wound healing may be seen in patients with HMS as well as patients with EDS or Marfan syndrome.”
• “People with HMS have complaints that have lasted from 15 days to 45 years, and onset of symptoms may occur at any age from 3 to 70 years.”
• HMS patients present “a wide range of traumatic or nontraumatic pain complaints … They typically lack the positive laboratory findings found in rheumatologic disorders and, in the absence of acute trauma, lack the radiological changes, inflammation, swelling, and decreased mobility typical of orthopedic pathology.”
• “People with HMS are more likely (69%) to have anxiety disorders than are comparison groups with rheumatological conditions (22%) … Anxiety may also be due to the perception of joint instability and frequent pain and injury without understandable antecedent.”
• “The high incidence of psychological disorders in people with HMS is similar to that seen with fibromyalgia. Ninety percent of individuals with HMS and fibromyalgia reported sleep disturbances. Because HMS and fibromyalgia appear to be related, the causes of psychological disorders in people with HMS may be similar to those proposed for fibromyalgia: abnormalities in serotonin metabolism, stage IV sleep patterns, and levels of substance P, or perceptual hypervigilance.”
• HMS patients have also reported increased cases of the following; uterine prolapse, rectal prolapse, varicose veins, increased bruising, increased skin elasticity, and decreased skin thickness.
• HMS appears to be a gender-influences dominant trait that causes an abnormality in type I collagen, which is abundant in connective tissues. “In patients with HMS, the ratio of type III collagen to type III + type I collagen is increased. Normally, this ratio is 18%:21%, whereas in patients with HMS, it is 28%:46%.”
• “Nerve tissue also appears to be affected in patients with HMS … Increased mobility and decreased joint position sense could make the joints of people with HMS more vulnerable to damage.”
• “Education is probably the most important treatment that physical therapists can provide to individuals with HMS … education regarding joint protection in HMS may similarly decrease pain, traumatic injury, and subsequent degenerative disease in vulnerable joints. It seems appropriate to advise individuals with HMS in the selection of jobs, sports, or recreational activities that will not exacerbate their condition.”
• “Although exercise will not increase stiffness of the lax ligaments seen in patients with HMS, strengthening and proprioceptive exercises are recommended for musculature surrounding affected joints … Indiscriminant exercise, however, could be harmful … [One should] advise individuals with HMS to use stretching exercises cautiously, distinguishing between stretching muscles and stretching joints.”

"Examination and Treatment of a Patient with HMS" L. Russek (2000)

“The signs and symptoms of HMS are common among patients seen in orthopedic physical therapy clinics; however, the HMS may be overlooked while treating individual joints or tissues causing pain … Recognition of HMS underlying common orthopedic problems may facilitate appropriate patient education and management.”
“Although the pathophysiology in HMS is not yet understood, the disorder appears to be a systemic collagen abnormality. The ratio of type I to type III collagen is decreased in skin. Abnormaility in collagen ratios is associated with joint hypermobility and laxity of other tissues … HMS also affects cardiac tissue and smooth muscle in the gastrointestinal system and in the female genital system.”
What the patient reported

“Pain usually developed without known cause, persisted for several weeks to several months, and then subsided.”
Easy bruising, frequent skin cuts that healed slowly, problems sleeping, chronic low-level gastrointestinal irritability, chronic fatigue
“After several episodes of seeing physicians without receiving diagnoses or recommended treatments, she discontinued seeking medical attention.”
“Mother had diffuse chronic joint and muscle pain … [and] had been various diagnoses, including osteoarthritis of the knee, sacroiliac dysfunction, and lumbar disk herniation, for her lower-extremity pain.”

“It is important to identify and address the underlying hypermobility rather than treat the individual symptomatic joints. This is because I believed her symptoms were caused by stresses that exceeded the hypermobile tissues’ ability to resist.”
“On the one hand, there is no cure for the disorder. The goal for treatment, therefore, is not to return to ‘normal’ (ie, not hypermobile) joint mobility but restoration of relatively pain-free function. That is, treatment does not eliminate the underlying impairment of excessive mobility.”
“Patients with HMS can function and their quality of life often can be improved with treatment but they will usually have chronic or recurrent problems.”
“The primary emphasis of intervention with this patient was education about the syndrome, about body mechanics and joint protection, and about lifestyle modification.”
“Although research on joint protection has not been done on HMS, the joint instability in HMS is similar to that seen in the active phase of rheumatoid arthritis.”
“[The patient] reported that activity modification protecting one joint sometimes transferred stress to other joints, which subsequently became symptomatic. Therefore, she was unable to avoid stress to all joints all of the time.”
“She reported having had continued difficulty sleeping … [and] was prescribed low doses of notriptyline (a tricyclic antidepressant that acts as a serotonin reuptake inhibitor) … Sleep disturbance, although seldom studied in patients with HMS, may be common: 90% of individuals with both HMS and fibromyalgia reported sleep disturbances … HMS might share some of the physiological abnormalities, such as the decreased cerebrospinal fluid serotonin levels, seen in fibromyalgia.”
“Because she knew the limitations of what the medical community could do for her disorder, she decreased use of health care services that she knew would be of no benefit.”

"BJHS: Evaluation, Diagnosis, and Management" M. Simpson (US Army Health Clinic) 2005

“Although BHJS has been well recognized in the rheumatology and orthopedic literature, it has not been discussed in the family medicine literature.”
“When patients with this syndrome are first seen by a physician, their chief complaint is joint pain, so BJHS can be easily overlooked and not considered in the differential diagnosis.”
“In general, women have greater joint laxity than men, and up to 5% of healthy women have symptomatic joint hypermobility compared with 0.6% of men. People of African, Asian, and Middle Eastern descent also have increased joint laxity.”
“First-degree relatives with the disorder can be identified in as many as 50% of cases.”
Patients with BJHS present with joint pain because “excessive joint laxity leads to wear and tear on joint surfaces and strains or fatigues the soft tissue surrounding these joints … [proprioception] impairment can also lead to excessive joint trauma due to impaired feedback from the joint affected.”
“The onset of symptoms can occur at any age … Physical activity or repetitive use of the affected joint often exacerbates the pain. Consequently, pain usually occurs later in the day and morning stiffness is uncommon.”
“Patients with BJHS often say that they are ‘double-jointed’ … or have a family history of ‘double-jointedness.’ Other presentations include easy bruising, ligament or tendon rupture … [and] thin skin.”
“Diagnoses of BJHS is one of exclusion.”
“BJHS is thought to be a mild variation of EDS and most closely resembles EDS type III … researchers have suggested that BJHS lies on a continuum with EDS and may be its mildest form because of their overlapping features.”
Effective treatment may include lifestyle modification, joint protection and stabilization exercises, proper body mechanics, and improving proprioception
“Hypermobility is a common cause of unexplained joint pain, yet is often misdiagnosed in primary care. According to one source, primary care providers recognized generalized hypermobility in less than 10% of patients with generalized hypermobility who were referred to rheumatologists.”
“The potential complications of BJHS underscore the importance of making an early diagnosis and educating the patient … a prompt diagnosis improves pain control and decreases disruptions in these patients’ physical activities, school, work, and quality of life.”

"Genetic basis of joint hypermobility syndromes" F. Malfait, A. Hakim, A. De Paepe, R. Grahame – (2005)

“Rheumatologists have long considered that joint hypermobility is inherited … Later it became apparent that connective tissues other than joints, such as skin, bone and eye, participated in the connective tissue fragility seen in JHS.”
“It became evident that JHS is itself an under-recognized form of an heritable disorder of connective tissue (HDCT). Patients with JHS can present mild expression of marfanoid habitus, osteoporosis, blue sclerae, skin hyperextensibility, atrophic scarring or easy bruising. Since these features can also be present in the most common form of EDS, the hypermobility type (EDS III), it seems increasingly likely that JHS is, if not identical, indistinguishable from the hypermobility type of EDS.”
“To date, the genetic basis of joint hypermobility remains largely unknown … Reports of genetic studies in patients and/or families with EDS, hypermobility type, or with JHS are scarce.”
“Classic EDS is characterized by soft and velvety skin, abnormal scar formation and marked joint hypermobility. The joint hypermobility is usually generalized, affecting both large and small joints, and it can range in severity from mild to severe, with major articulate complications such as habitual subluxation and dislocation of the joints.”

"Time to take Hypermobility Seriously" R. Grahame (Brit Soc of Rheum) 2001

“From the outset it was perceived more as a curiosity than as an entity that could have significant (let alone serious) import”
“There is now abundant evidence … that hypermobility can have a serious impact on people’s lives”
“Patients with BJHS have diminished joint proprioceptive acuity and are less responsive than normal to the local anaesthetic effects of lignocaine”
“Failure to receive effective intervention not infrequently results in a complicating chronic pain syndrome, fibromyalgia, and depression”
There appears to be a total lack of consensus on what criteria to use in diagnosis … a distinct lack or familiarity with recently published literature, in particular on the question of the impact on patient’s lives in terms of the effects of chronic pain and the psychosocial sequelae”
“BJHS is not an easy condition to treat. Physiotherapy forms the mainstay of treatment but has to be tailored to the needs of intrinsically vulnerable tissues, otherwise it may aggravate rather than relieve. Analgesics and non-steroidal anti-inflammatory drugs are generally ineffective, and surgery can be counterproductive”

"British Consultant Rheumatologists' Perceptions of the Hypermobility Syndrome: A National Survey" R. Grahame, H. Bird (UCL) 2001

Joint hypermobility is a condition considered controversial by rheumatologists

92% believed in HMS as a “distinct clinical entity” but only 39% accepted it as a “distinct pathological entity.”
Only 42% would comment on whether HMS and EDS III (hypermobility type) are one in the same, and only 14% believed it was true
50% were skeptical that HMS has “a significant impact” on people’s lives

“The only logical explanation for these perceived differences [in opinion] is the failure on the part of many consultants to recognize the presence of hypermobility in their patients.”
“Joint hypermobility is easy to recognize if our looks for it, but equally easy to overlook if one does not.”
“In terms of the clinical consequences of hypermobility – pain, instability, dislocation, etc. – which may occur in a single lax joint, it matters not how many other joints may or may not be affected. It is clearly illogical to deny a patient a (correct) diagnosis of BJHS” simply because they do not show sufficient hypermobility in the rest of their joints
“A striking finding was that 61% [of rheumatologists] required a negative laboratory screen before they would be prepared to summon up significant courage to make a diagnosis of BJHS. This smacks of defensive medicine. It is due to a lack of confidence in one’s clinical findings, a need to make such a diagnosis by exclusion rather than by inclusion, or does it signify a lack of conviction in the very existence of the entity itself?”
“The low perception of the impact of the syndrome on individuals’ lives … is challenged by the patients themselves, and is contrary to published findings.”
“This survey confirms previous suspicions that the BJHS is a condition that is under-recognized and under-estimated by rheumatologists. There was little sign of awareness of the findings of recent published studies.”

"Living with the Hypermobility Syndrome" S. Gurley-Green (HMSA) 2001

A majority of HMS patients feel that their doctors don’t understand the impact HMA has on their lives, nor is the medical community able to provide effective treatment
There are 2 types of HMS patients 1) Those who suffer periods of pain (for a few weeks) but can be symptom free for some time 2) Those for whom every day is a struggle against pain
Literature suggests symptoms decrease with age, but the stiffening with age does not always mean less pain, and many patients feel their condition deteriorates over time, especially after pregnancy
There is currently no effective treatment for the majority of patients, for whom pain is sometimes unbearable
HMS makes performing in the workplace and caring for a family extremely difficult; for some the daily tasks of living, like grocery shopping and laundry, are too much
Parents are often afraid of passing HMS to their children, and many women are worried that they will not be able to meet the demands of motherhood
Many patients are accused of having psychosomatic pain because there is often no clear accident or trauma that coincides with the start of the pain, so doctors have a hard time taking the patient’s complaints seriously
The problem of chronic pain is exacerbated because there is often no link between actions and the onset of pain, so it is difficult to lower pain through behavioral modification
Many patients see multiple doctors, and suffer from the lack of continuity in treatment and conflicting advice

Scholarly Articles - Batch #2

2009-04-16T13:14:00.000-07:00

Below are links to some great articles concerning HMS.

1) “Hypermobility Syndrome” L Russek

2)
“Examination and Treatment of a Patient with Hypermobility Syndrome” L Russek

3) “Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management” M. Simpson

4) “The Genetic Basis of the joint hypermobility syndromes” F. Malfait, A. Hakim, A. De Paepe, R. Grahame

5) “Time to Take Hypermobility Seriously” R. Grahame

6) “British Consultant Rheumatologists’ Perceptions about the Hypermobility Syndrome: A National Survey” R. Grahame, H. Bird

7) “Living with the Hypermobility Syndrome” S. Gurley-Green

8) “Is the Benign Joint Hypermobility Syndrome Benign? H. El-Shahaly, A. El-Sherif

What You Need to Know - Scholarly Articles, Batch #1

2009-04-16T12:50:00.000-07:00

R. Grahame (UCL Hospitals) HMS and Pregnancy
• Symptoms of HMS during pregnancy include,
o Joint and spinal pains increase
o Tendency to premature labour and delivery
o Tendency to rapid labour
o Resistance to effects of local anaesthetics can cause problems during the epidural
o Healing of tear or episiotomy may be impaired and/or prolonged
o Pelvic floor problems (especially uterine prolapse), therefore post-natal exercise is very important
o 50% chance child will have HMS

Larson, Baum, Mudholkar 1981
• “Hypermobility was a predominantly female characteristic”
• Joint laxity declines naturally with age, but “not to a statistically significant degree”
• In men, joint laxity started to decline in mid-twenties, in women it continued through mid-forties

Bridges, Smith, Reid
• Most patients with hypermobility had common musculoskeletal problems as the main reason for their referral (usual to a rheumatologist)
• “Results show that joint hypermobility is common, familial, found in association with common rheumatic disorders, and statistically associated with osteoarthritis”
• “The findings support the hypothesis that joint hypermobility predisposes to musculoskeletal disorders, especially osteoarthritis”

R Graham (UCL Hospitals) Pain and Hypermobility Syndrome 2009
• “At the present time we simply do not know for certain whether or not HMS is a less severe type of Ehlers-Danlos Syndrome III.”
• “Usually unexpected exercise or a change of job or lifestyle provokes the onset of pain. Whenever symptoms commence … the term ‘Hypermobility Syndrome’ is used … hypermobile people without pain are just called hypermobile people.”
• “So how is it that people with hypermobility can be literally fine for decades, only to be laid low from widespread pain, often out of the blue, which may make its unwelcome appearance during childhood, adolescence, or adult life?”
o A hypermobile person has an inbuilt weakness if their “strength-providing” collagen. Injuries occur “whenever there is a mismatch between the physical demands on one hand, and the strength of the parts being asked to perform on the other… If one only knew one’s strength (or lack of it), one should be able, in theory at least, to stay within the safety margin and thereby protect oneself from injury.”
• “Neither susceptibility to injury or overuse … explains all the pain that is felt in HMS.”
o Pain is a subjective experience, and “the severity of the pain we feel is greatly influenced by our state of mind.”
o “Lack of understanding of the condition is widespread, and this, coupled with failure to receive adequate treatment for relief of symptoms, leads to frustration, resentment, anger, and ultimately depression.”
o HMS and fibromyalgia occur together quite often, and “fibromyalgia should be regarded as a signal emanating from a distressed HMS person.”
• There may be two clues to explaining the burden of pain bourn by HMS patients
o First, people with HMS have diminished proprioception, that is to say, “people with HMS are not quite as good as other people in knowing exactly where their fingers or arms, etc. are in space. This could lead to a further increase in the risk of injury.”
o Secondly, “patients with HMS for some reason do not appear to experience the full anaesthetic affect of lignocaine injections when these are given for dental purposes, minor surgery or for epidural anaesthesia.”
o The above “suggest that people with HMS … may also have a fault in the way their pain signals are picked up for onward transmission to the brain, where they reach consciousness.”

Howes, Isdale (New Zealand)
• They studies 102 cases of backache – 59 men, 43 women. Out of the 59 men, none had hypermobility, but rather had disc lesions, spondyiolysis, etc. One half of the 43 women suffered from those same ailments, while the majority of the remainder suffered from HMS
• “It is suggested that this is an important differential diagnosis of backache in women”

Scholarly Articles - Batch #1

2009-04-16T12:42:00.000-07:00

Pregnancy and JHS/EDSHM

RODNEY GRAHAME CBE, MD, FRCP, FACP
Emeritus Professor of Rheumatology, University College Hospital, LONDON.

Unlike the vascular form of the Ehlers-Danlos Syndrome ( EDS ), formerly EDS Type IV, the Joint Hypermobility Syndrome (JHS), which is equivalent to EDS–hypermobility type, formerly EDS III ) is not associated with heart disease or major hazards during pregnancy and labour. However there are a number of considerations that should be borne in mind, which are listed here:

Joint and spinal pains may increase during the course of the pregnancy.
There is a tendency to premature rupture of the membranes and thus of premature labour and delivery.
There is a tendency to rapid labour.
There is an apparent resistance to the effects of local anaesthetics is seen in about two thirds of patients and can cause problems for the unwary during epidural anaesthesia or infiltration for repair of a tear of episiotomy.
Healing of tear or episiotomy may be impaired and/or prolonged and surgical technique may need to be modified accordingly.
She may find lactation and care of the newborn baby more taxing than most.
Pelvic floor problems (uterine prolapse etc) may occur in later life so that the practice of post-natal exercises is particularly important.
Since JHS follows a dominant pattern of inheritance there is a 50% chance that any offspring will carry the gene, although this does not mean that he/she will necessary develop symptoms of tissue laxity subsequently.

Hypermobility: features and differential incidence between the sexes.
Larsson LG, Baum J, Mudholkar GS.

Department of Medicine, Monroe Community Hospital, Rochester, NY 14603.

Six hundred sixty individuals from a music school were studied to determine the frequency of incidence and the nature of their hypermobility. They were interviewed and examined for the 5 recognized features of hypermobility (laxity of the thumbs, fingers, elbows, spine, and knees). The incidence of hypermobility, according to the number of joints involved, followed an empiric geometric law. Approximately 27% of the individuals had 1 lax joint, whereas only 3% possessed all 5 features. Specific features were present at different frequencies in the 2 sexes. The ratio of the occurrence of 2 features in women compared with the occurrence in men was 2:1. Ratios for the occurrence of 3, 4, and 5 features were 4:1, 8:1, and 3:1, respectively. Thus, hypermobility was a predominantly female characteristic. Joint laxity declined with age, although not to a statistically significant degree. In men, the decline started when they were in their mid-twenties; however, in women, joint laxity continued through the mid-forties.

PMID: 3435571 [PubMed - indexed for MEDLINE]

Annals of the Rheumatic Diseases 1981;40:541-546; doi:10.1136/ard.40.6.541

A clinical and echocardiographic study of patients with the hypermobility syndrome.

R Grahame, J C Edwards, D Pitcher, A Gabell and W Harvey

Three age- and symptom-matched groups of patients with a hypermobilityscore of 5-9, 3-4, and 0-2 (controls), respectively, were examinedfor clinical and echocardiographic evidence of mitral valveprolapse and other stigmata of a collagen disorder. Mitral valveprolapse, a reduced upper segment/lower segment mitral ratio,reduced skin thickness, spinal anomalies, and a history of fracturewere found to be significantly commoner among the hypermobilepatients than the controls. The data suggest that the so-calledhypermobility syndrome, far from being a benign locomotor disorderof healthy persons, may be a forme fruste of a hereditary disorderof connective tissue.

Annals of the Rheumatic Diseases 1992;51:793-796; doi:10.1136/ard.51.6.793

Joint hypermobility in adults referred to rheumatology clinics.

A J Bridges, E Smith and J Reid

Department of Medicine, University of Missouri School of Medicine, Columbia.

Joint hypermobility is a rarely recognised aetiology for focalor diffuse musculoskeletal symptoms. To assess the occurrenceand importance of joint hypermobility in adult patients referredto a rheumatologist, we prospectively evaluated 130 consecutivenew patients for joint hypermobility. Twenty women (15%) hadjoint hypermobility at three or more locations (greater thanor equal to 5 points on a 9 point scale). Most patients withjoint hypermobility had common musculoskeletal problems as thereason for referral. Two patients referred with a diagnosisof rheumatoid arthritis were correctly reassigned a diagnosisof hypermobility syndrome. Three patients with systemic lupuserythematosus had diffuse joint hypermobility. There was a statisticallysignificant association between diffuse joint hypermobilityand osteoarthritis. Most patients (65%) had first degree familymembers with a history of joint hypermobility. These resultsshow that joint hypermobility is common, familial, found inassociation with common rheumatic disorders, and statisticallyassociated with osteoarthritis. The findings support the hypothesisthat joint hypermobility predisposes to musculoskeletal disorders,especially osteoarthritis.

Pain & the Hypermobility Syndrome

RODNEY GRAHAME CBE, MD, FRCP, FACP
Emeritus Professor of Rheumatology, University College Hospital, LONDON.

When the Hypermobility Syndrome was first put on the medical map in 1967, it was defined as the presence of musculoskeletal symptoms (predominantly pain) occurring in otherwise healthy individuals. Thirty years down the line we now think that there are probably two types of hypermobility.

The first is a milder type occurring in people whose joints are just like everyone else's but which have the capacity to move more than most people's joints. The other, a more marked form, has features that suggest that it may be part of an inherited connective tissue disorder similar to the hypermobile form of the Ehlers-Danlos Syndrome, formerly called EDS III. It probably is EDS. At the present time we simply do not know for certain whether or not HMS is merely a less severe type of EDS III. Pain can occur in other forms. For the rest of this article, in order to avoid confusion, I will refer to it as one condition, the HMS/EDS. There are many people with joint hypermobility in the community who experience no (or very little) pain. Most of them probably do not even know that they are hypermobile at all. Then something happens, and they start getting pains for the first time in their lives. Usually unexpected exercise or a change of job or lifestyle provokes the onset of pain. When ever symptoms commence, and irrespective of the cause of the hypermobility, the term 'Hypermobility Syndrome' is used to describe the condition. Hypermobile people without pain are just called hypermobile people.

A little known fact is that hypermobility occurs in many individuals in a few joints only. It does not necessarily have to affect all one's joints. Even hypermobility in a single joint can cause pain and/or instability in that joint; the diagnosis is still Hypermobility Syndrome (HMS/EDS).

People are born hypermobile. It is in their genes. It is the way they are made. So how is it that people with hypermobility can be literally fine for decades, only to be laid low from widespread pain, often out of the blue, which may make its unwelcome appearance during childhood, adolescence, or adult life? To explain these curious and seemingly inexplicable happenings, we postulate that the affected person, in this case the hypermobile person, had an inbuilt weakness of her (it is usually a 'her' but not always) strength-providing collagen or similar protein. This weakness renders body tissues less robust and hence less able to stand up to the physical strains of everyday life. We can explain a lot of the pain that arises on the basis of a series of (often quite minor) injuries that occur whenever there is a mismatch between the physical demands on one hand, and the strength of the parts being asked to perform them on the other. There is a whole host of painful injuries that can result, ranging from dislocations to fractures, disc prolapse, ligament sprains, muscle strains, pulled tendons (like tennis elbow or plantar fasciitis), etc.- conditions that can happen to anyone, but which occur more easily in those with the HMS/EDS. If one only knew one's strength (or lack of it), one should be able, in theory at least, to stay within the safety margin and thereby protect oneself from injury. Up to a point this is so. Many people with the HMS/EDS are able to modify their lifestyle and do just that. Others find it more difficult.

A joint that is beginning to wear starts to lose its cartilage or gristle, which is essential for the smooth movement between the adjoining bones. This wear heralds the onset of osteoarthritis, a condition painful in its own right. It is a very common form of arthritis in middle and old age, and one to which hypermobile people seem to be prone, in many cases at an early age. It is important to establish as far as possible to what extent the pain is due to trauma/overuse or to early onset osteoarthritis, as the treatment is very different. The emphasis with the former is on prevention, while with the latter it is on treatment by one form or another.

However, neither susceptibility to injury or overuse (repetitive injury), nor osteoarthritis, explains all the pain that is felt in the HMS/EDS. There is more to it than that.

"Emotional influences can amplify pain, but they do not cause it."

Let us try to piece the jigsaw together. Pain is something we feel. Even if it has a physical cause, as it undoubtedly has in the HMS/EDS, it is still a subjective experience. It is often accompanied by an intense sense of exhaustion. the severity of the pain we feel is greatly influenced by our state of mind. If we are upset or agitated it tends to increase. If we are content, relaxed or just happy it tends to diminish. The HMS/EDS people are often in the former category, and for good reason! Lack of understanding of the condition is widespread, and this, coupled with failure to receive adequate treatment for relief of symptoms, leads to frustration, resentment, anger (and lots more emotions which I could list but readers know them all only too well!) and, ultimately depression. These emotional influences can amplify pain, but they do not cause it.

Another piece of the jigsaw is a mysterious condition called fibromyalgia. This condition causes widespread chronic pain in muscles and is identified by the finding of multiple 'tender points' at specific sites in the body. For years the debate has raged as to whether it is a physical disease affecting the muscles, or whether it is an emotional disorder. The prevailing view is that it is probably some form of distress signal that can arise in people with a number of different and unrelated conditions. How this relates to hypermobility is that it has been shown that hypermobility and fibromyalgia occur together in the same person more often than one would expect on the basis of a chance happening. It does not mean that they are part of the same condition. It would, indeed be very unlikely that what is clearly an acquired disorder (fibromyalgia) could be an integral part of what is clearly a genetic one (HMS/EDS). More likely, the fibromyalgia should be regarded as a signal emanating from a distressed HMS/EDS person. But fibromyalgia occurs only uncommonly in the HMS/EDS. We must look further afield.

It seems quite likely that there may be a totally different explanation for the burden of pain borne by people with the HMS/EDS. Here are two clues:

The first clue relates to the sense called proprioception, which means knowing where parts of one's body are in space. If you close your eyes and somebody bends your finger for you or picks your arm up, you know immediately how far your finger is bent or where your arm now is. That is because you have good proprioception. Scientists have shown that people with the HMS/EDS are not quite as good as other people in knowing exactly where their fingers or arms etc are in space. This could lead to a further increase in the risk of injury.

The second clue is the discovery that patients with the HMS/EDS for some reason do not appear to experience the full anaesthetic affect of lignocaine injections when these are given for dental purposes, minor surgery or for epidural anaesthesia (I wonder how many readers are nodding their heads as they read this!).

At the present time we do not know quite what either of these clues means or whether they relate to one another. But they do suggest that people with the HMS/EDS, in addition to their proneness to injury, dislocation and osteoarthritis, may also have a fault in the way their pain signals are picked up for onward transmission to the brain, where they reach conciousness. Research work is in progress to try to sort out this enigma. Much more needs to be done. Watch this space!

Readers will know that many conventional methods of treatment, of the kind offered for rheumatic complaints in general, are not particularly helpful in the HMS/EDS. Physiotherapy still carries the best prospect for pain relief, and it is encouraging that more physiotherapists are training in methods that are helpful in this condition. Another noteworthy development is the network of units throughout the country that are offering pain management programmes, an approach which has been shown to be beneficial in the HMS/EDS where intractable pain is present.

Revised 15 March 2009

THE LOOSE BACK: AN UNRECOGNIZED SYNDROME

R. G. HOWES and I. C. ISDALE

From the Queen Elizabeth Hospital Rotorua, New Zealand

A prospective study of 102 cases of backache with particularregard to the presence of ligamentous laxity is reported. Amongthe 59 men there was no excess of cases showing unusual spinalor peripheral joint mobility; nearly all had features of disclesions, spondylolysis, spondylosis, or spondylitis. In contrast,only half of the 43 women could be diagnosed as suffering fromthese conditions, while the majority of the remainder showedcentral and peripheral hypermobility. The clinical featuresof the "loose back syndrome" are outlined. It is suggested thatthis is an important differential diagnosis of backache in women.

Welcome to Hypermobility Hope!

2009-04-14T11:11:00.000-07:00

Hi everyone and welcome to Hypermobility Hope!
The purpose of this site to to raise awareness about Hypermobility Syndrome, and to give those who suffer from HMS, or know someone who has HMS, or just think they might have HMS, the information they need to make sure they receive proper care. As a HMS patient myself, I know how frustrating it is to find doctors who will listen to you, who understand how difficult and destructive HMS can be, and who genunuinly respect the implications and complications HMS can have on daily life.
I have tried to create the kind of site that I wish I had been able to find - a compassionate and welcoming environment where one can easily find explinations, information, advice and comradery. I have done extensive research into HMS, and I have provided links to as much information as I could find. Along with the links to scholarly journals I have provided cheatsheets that will tell you what is important and what you need to know from that particular article. I also have attached a fact sheet and diagnostic question list that will help you learn what you need to know about HMS in the most painless way possible. I also hope to upload video interviews with various doctors and other HMS patients.
In addition to general HMS information, I will be sharing my own personal journey with HMS in the hopes that others out there with HMS will be able to relate to my pain, anger, and lonliness, and will be able to glean some hope knowing that they are not alone in this fight.
The medical community has ignored Hypermobility Syndrome since its appearence in medical journals in 1967, and most doctors view HMS more as a curiosity than a serious medical condition. It is my mission to make them see that HMS is a real condition with disasterously negative affects on patients, and that it is time to do something about it!
Please feel free to post comments or suggestions, it is up to us to raise awareness, our fate is in our own hands.
Remember, Awareness = Power + Hope!
Always,
Chelsea