And I was right again, unfortunately...

I didn't have a chance to write more after the last update because when I went to see the doctor he told me I had to have surgery again for yet another peri rectal abscess! He was in shock and couldn't suss it out, which made me feel even more worried. Two in one week is a bit bizarre. He asked if I had eaten when I was at his office and I said yes, so he said he could do it with local anesthetic. Anyone who has read my ER visit last year will know that there was NO WAY IN HELL that I would let anyone ever cut me without being unconscious again. I was quite happy to wait one more day with some pain than to have that happen again. So, that night my mother came in so she could go to the doctors with me. This time they had the demerol ready afterwards. Last time even though I told them I would need it they tried to fob me off with some percaset - which makes me puke my brains out.

I was out from work the day of surgery and the next day. I saw the doctor the day after and he was still perplexed, though happily this time he said that there was a lot of puss and not just blood. Of course I was told that I have to have a colonoscopy for sure, no getting out of this time. It's probably for the best as I've been so bloated the last few months and my insides are schizophrenic. The dr still doesn't think it's Crohn's, and I've done a bit of research myself. I suppose it could be but I don't think so. Not that I'm hoping for any one of these, but for all the symptoms I have I've narrowed it down to a few possibilities. Hopefully (touch wood) it's just IBS, and we shall find out in a few weeks when I have the colonoscopy.

The main problem was with timing as I was off to London on the 1st of May, just four days after the second surgery! I rang the airlines and asked if I could upgrade one way, and they kept saying nope, because you used miles for the ticket, but to ask at the airport. You'd think they'd be nicer to loyal customers! When I got to the airport I asked the check-in man, and he said he couldn't, but was at least nice enough to block the seat next to me. Only problem was that it was a bulkhead which was good, but not when you want to spread out as you can't lift the armrest. Oh well. When I got to the gate I asked again, and I have to say the woman working there was such a nasty cow! No sympathy let alone any assistance and such a bitch! I hope she has to have this surgery and then sit on her arse for 8 hours and see how she likes it! I normally wouldn't say that but she truly was an horrible person! So I get on the plane and ask one of the flight attendants if they could help me, and of course, she checks w/ her head person and no result. At this point I'm getting beyond desperate. I try to get myself sorted in the seat and have my legs over the arm rest onto the other seat, but of course I'm leaning on my right hip at this point and well, that hip was not happy even w/ the lido patch on it. After we took off I couldn't sit anymore so I made a little spot on the floor with blankets and pillows and curled up. Of course then another flight attendant comes over and tells me I can't do that in case we hit turbulence, blah blah blah. I looked at him and was about to cry. I told him the situation and he was kind, and said he'd see if he could get someone in the back w/ extra seats to switch. I didn't think he could because if I had three seats to lay down on I wouldn't switch either to a bulkhead. He came back to collect the rubbish from everyone and said he was still working on it. Fair enough - I held out as much as possible. Then he ran up to the front, leaving his cart just sitting there, and came back a few mins later saying he'd sorted out something and would be back when he was done. Miracle of miracles, he had told the first class purser my situation and that I didn't need any extra attention, just a place to lay down and the bloke had agreed. Thank G-d! This very nice man took me up front, showed me how to recline the seat into a bed and for the remaining few hours I was able to lie on my stomach. I would not have made it otherwise, that's for sure.

The first few days in London were a little rough but I managed okay. By the end of the week I think the cut was healed up for the most part. The flight back wasn't bad for the bum at least. The rest of me was not happy and I am so so glad I now order the wheelchair assistance because there is no way I would have been able to walk off that plane and through the terminal. My whole body was in pain from the pressure on the flight, plus all the walking I had done during my visit.

So now I'm back and have the colonoscopy scheduled in a few weeks. Yesterday I caved and rang my hand/wrist doctor because my right hand has been getting worse and worse and the last week with the camera threw it into pain overdrive. I believe it was over a year ago that I saw Dr. Melone and he gave me an injection. He said if it didn't work I'd need surgery. It lasted maybe a month or so and then, well, I had the whole ER and one surgery after another to deal with on the opposite end, so ignored it as much as possible. Lately though I've taken to rubbing my wrist in with lidocaine cream which seems to be the only way to get through the day. I am still wearing the splint at night, mostly, and it's not doing much of anything. Therefore, I caved and made an appointment to go see him. Trust me, I really don't want to. I don't even have many days off left for this crap, but I don't know if I can wait with the pain for another 7 months to have it sorted. I will hear what he says about the surgery and if it is a quick recovery or not and then will make a decision.

Also because of all that's been going on I've been quite remiss on my PT and the neck and jaw are acting up quite a lot. Started doing some PT last night and will be going to see the acupuncturist this week as well. Praying for some pain relief.

Like I always say, never a dull moment.

Don't you just hate surprises?

Well I do. Last weekend, the 18th, I woke up early am w/ lots of pain in my bum. The same kind of lovely pain I had when I had the peri-rectal abscess last year. I was none too pleased but hoped it was just sore from sitting the day before. The pain and pressure proceeded to get worse over the weekend. My biggest fear was that it would get so bad that I'd end up in the ER again. After that nightmare last year of being butchered it was the v. last thing I wanted. Also on the Friday I was having pelvic pain and thought I might have an UTI. So Monday am I rang my internist and made an appt for that day. I also rang the CRS and found out he was not in, but was able to get an appointment at 9am on Tuesday. They said if it was an abscess he would do surgery directly.
I knew that Dr. Aronoff would knock me out, unlike the hospital, but I still am a big baby when it comes to getting diced and sliced. Thank G-d for my mum who came in on Monday night so she could come to the appointment with me.
And yes, the dr examined me and next thing I knew I was being prepped for surgery. Never a dull moment around here! After surgery though he told me it was mostly blood that came out, no puss, and he was v. perplexed. I don't know about you but when the dr is more confused than me it makes me pretty uncomfortable. We are still waiting for the lab results, hopefully today. I went on Thursday for a post-op and he said it looked good but was still baffled. I had realised by Monday that both my dr's were going to make me have another colonoscopy after this second incident in a year, but thankfully not until this heals up. I was holding up relatively well this weekend and was able to get errands for my upcoming trip done. I leave on Friday for London, so again timing royally sucked. Not looking forward to sitting on a 7+ hour flight w/ a sore bum.
When I woke up this morning and sat up, I had a very sharp, stabbing pain in my bum. I felt it and it feels very sore and hard again, so I'm being very paranoid. Thankfully the CRS is a v. nice doctor and he said last week to ring him if anything didn't feel right, and since I am off to London, I am going to be overly precautious and see him today, just to make sure. If something else has gone wrong (hopefully not) I'd rather sort it out today than later in the week.
So that's the fun that's been my life lately.

I've been avoiding writing

because I didn't want to just go on a whinge rampage. For the last two months my bottom right jaw has been killing me - aching, throbbing pain that gets so bad it keeps me up some nights.
I went to see my dentist and he took an x-ray but couldn't find anything. Surprise surprise. We tried a week of antibiotics to make sure it wasn't an infection, but no result there. It's amazing how you can WANT something specific to be wrong just so there is a way to fix it (well, hopefully). But when the pain can't be located, as such, it's like looking for a needle in an hay stack!

Next stop was to see the endodontist. I was referred by my parents neighbour - they had gone to dental school together. I find it helps to have some sort of background or introduction to new doctors so they don't look at your chart and listen to you and think you're just an hypochondriac. You'd be surprised how many daft doctors there are out there. Or perhaps you wouldn't be...

I went to the endodontist on Tuesday morning. He looked, banging, made me bite hard things, blew cold air, etc. And again, he couldn't figure it out. It wasn't a failed root canal, it wasn't another tooth needing a root canal. He thinks it's nerve-related, but not "that" nerve type of thing where he would know what to do to fix "that" nerve. He was very nice and not too patronising. He said he would talk to some colleagues to see if they came up with anything. It's Friday and I've still not heard from him, so I'm guessing no one was able to come up with an idea.

Yesterday I went for acupuncture though. I told my doctor what was going on with my jaw, as well as with my hip (it's been wonky and limpish for the last couple of weeks) and knees (they're swollen and not happy). I filled him in on the PT I had been doing and told him I thought my hips were unhappy b/c of the core exercises. He agreed that could be it, even though the PT lady (who was v. nice and good) didn't think it should be a result of that. But he's had two hip replacements and does all sorts of keep-fit things, and he said ever since his hip replacements he's not been able to do core work either as it irritates it. So, yes an answer, but still a new catch - 22 b/c how am I meant to strengthen my core if I can't do the exercises?

Regarding my jaw, he felt it and said - "it could be the muscle there". There's a muscle there, under my tongue, and no one else thought to mention that? This is why it's good to have all different types of people around you - everyone thinks differently. So he shows me his Trigger Point poster - I love this thing because it's like someone who didn't know me drew it and put big X's on all of the parts that hurt me, what a coincidence! :-) And sure enough, on the green part that is the disxxxxx whatever name I can't say muscle, there is a big X EXACTLY WHERE IT HURTS!!! Now mind, is this 100% the problem? Who knows. But at least it's some sort of start and we can try to fix it and see what happens. This time we did more needling on my legs and hips than my neck, but a little on my shoulder and in my tmj. He needled me under my jaw into that muscle and sure enough, there was a trigger point - and a slippery one at that, took a bit to get the needle in (not pleasant) but (touch wood) afterwards I did feel a bit better. He things the trigger point is from clenching my jaw, related to my tmj, which is related to stress, which I've been under because of the world and the pain I've been and not being able to sleep and there goes the never ending cycle. So, as with all acupuncture/dry needling, we will wait a bit and see what happens. I am trying to be hopeful but not overly so because, well, I know my body.
I did feel all over a lot better when I left his office last night though. A combination between him and the treatment me thinks!
So next appointment is 30 April, the day before I leave for England. Fingers crossed!!!

So You Think You Might Have EDS?

After reading this I was amazed how many things add up. Before my diagnosis I was made to think I just had tons of random things wrong with me, but when I look at this, it all fits together like a puzzle. But if you do have many of these, or even some, you must get diagnosed by a genetic doctor. If you need a referral for one in NYC I have a great one.

By Sue Jenkins RN with contributions from Liza Sauls (from www.ednf.org)

It has been the experience of many of our members that a final diagnosis of EDS is the result of 'connecting the dots' of a person's history and a comprehensive review of the constellation of all symptoms; and includes, for many, thorough review of the medical histories of their families as well. Finally getting the diagnosis can be a relief to know that the symptoms are real and have a name; however limitations occur here as well. There is no cure, no 'fix', simply because the collection of experiences and symptoms now has a name and identity. But it does allow the patient and their families to know what they may be facing and allow them to become educated and proactive about the care they seek and require.

Based on the accumulated experiences of our members, the following essay is an overview of many of the symptoms and conditions that can be associated with EDS. It is not meant as a substitute for thorough medical review and care and supervision, but to help to view some aspects of EDS. Not every patient will have every symptom, as you will see, EDS can manifest in many different ways.

The challenge of EDS is not to be able to identify each symptom, but to be able to see a pattern among several.Early diagnosis and intervention are the keys to living the best life possible. With the recent TV programs on Ehlers-Danlos Syndrome, many people are coming to this website with questions about having EDS. Here is a list to help you determine if you need to see a geneticist who can correctly diagnose you.

Caution: If after reading the following article, you think you may have EDS, be sure to seek medical advice.

Please do not rely solely on this article or end your search with a self- diagnosis. EDS is a genetic syndrome and is diagnosed by a specialist, usually a geneticist or rheumatologist with extensive knowledge about EDS and its types:

• Classical
• Hypermobile
• Vascular
• Kyphoscoliosis
• Arthrochalasia
• Dermatosparaxis

Ehlers-Danlos Syndrome is a group of disorders that affect connective tissues, which are tissues that support the skin, bones, blood vessels, eyes and other organs. Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos Syndrome, which vary from mildly loose joints to life-threatening complications related to tissue structure and fragility.

A physical examination is required, including taking an extensive family history and using the Beighton scale, which measures Hypermobility. Depending on which type of EDS the physician believes you have, eithera blood test or muscle biopsy will be taken.

How You Get It:
EDS is a genetic disorder, a mutation of one or several genes that make different types of collagen in your body, producing a defective tissue. A mutation in a gene on one of the first 22 nonsex chromosomes can lead to an autosomal disorder. Genes come in pairs.

Some types of EDS are autosomal dominant and others are autosomal recessive. Differing types of EDS affect different types of collagen.

• If a disease is autosomal dominant, it means you only need to get the abnormal gene from one parent in order for you to inherit the disease. One of the parents may often have the disease. Each child has a 50/50 chance of inheriting this disease.

• Recessive inheritance means both genes in a pair must be defective to cause disease. People with only one defective gene in the pair are considered carriers. However, they can pass the abnormal gene to their children.

Wrong Diagnoses:
Most people diagnosed with EDS have come the same long road where it seemed that nobody knew what was really wrong with you. Diagnoses of

• osteoarthritis
• fibromyalgia
• lupus
• rheumatoid arthritis
• rheumatic fever
• multiple sclerosis
• “growing pains”
• and “it’s all in your head” are just some of them

Often people get several misdiagnoses before finally being correctly diagnosed with EDS.

Symptoms of EDS:
Although all types of Ehlers-Danlos syndrome affect the joints and many also affect the skin, features vary by type and severity. An unusually large range of joint movement, hypermobility, occurs with most forms of Ehlers-Danlos syndrome, particularly the HEDS (hypermobility) and CEDS (Classical) types.

Below is a listing of symptoms that persons with EDS often have. These symptoms are broken down according to body systems. This list is not all-inclusive, but include those most frequently encountered.

Not everyone with EDS has them all and if you have some of them you may still not have Ehlers-Danlos Syndrome.

Joints:
• Different types of EDS have differing degrees of joint problems. Joint dislocation and incomplete dislocation called subluxation is common and recurrent.
• Spontaneous easy reduction or replacement of the finger digits and shoulders occurs.
• Hypermobile joints cause pain, and sometimes the "cracking" or "popping" of them feels like it relieves the pressure.
• ‘Pes planus’ or being flatfooted is common and feet can flatten even more as one ages.
• EDSers can develop osteoarthritis earlier than typical, and they often have difficulty or pain walking. They can appear klutzy.
• Some EDSers’ hands collapse from the pressure of a simple handshake. It is difficult to write, and often finger splints help a great deal.
• Cervical (neck) instability occurs in some types, and some people may have trouble holding up their head.
• Another frequent joint problem is fluid effusions into the knees, ankles and elbows, primarily in Classical or Kyphoscoliosis types. (effusion: The escape of fluid from the blood vessels or lymphatics into the tissues or a cavity)
• In the Kyphoscoliosis type, many infants have severe muscle hypotonia (floppy babies), generalized joint laxity and scoliosis at birth, or develop a progressive scoliosis (a curvature of the spine) within their first year of life.
• With Vascular EDS, joint hypermobility may be limited to the small joints of the feet and hands or may be very lax all over. As with other types, VEDS patients often are first diagnosed as hypermobile, only later learning that they have VEDS!

The range of hypermobility differs greatly among EDSers, even within types. The loose joints throughout life are unstable, prone to subluxation and dislocation, cause chronic pain and early-onset arthritis. Some people are only mildly affected by their EDS; others are completely debilitated.

Orthopedic procedures to stabilize or improve the joint's function sometimes put more than expected strain and stress on adjacent joints, leaving many EDSrs disappointed with the results.

So your back, hips, shoulders, knees, elbows, and other joints go out more often than you do, you might have EDS.

Skin: General EDS skin traits include:
• Easy bruising, delayed wound healing, differing types of scarring.
• Thinner skin than normal, especially in the Vascular type.
• Those with VEDS have translucent skin where the blood vessels below are clearly visible.
• Soft, velvety skin that is fragile and sometimes highly elastic (stretchy) is found, especially in the Classical type.
• Classical type EDS may experience wounds that split open with little bleeding and leave scars that widen over time to create characteristic shallow "cigarette paper" scars.
• Surgical incisions may present problems with healing, with stitching EDS skin sometimes described as "like sewing butter." Often requiring sutures being closer together and left in for a longer time than usual.
• With severe CEDS, even just leaning on the table with your elbows can cause the skin to split or may have molluscoid pseudotumors on elbows and knees. (molluscoid pseudotumors are small, spongy tumors consisting of fat surrounded by a fibrous capsule found over scars and pressure points).
• Skin that sags and wrinkles is characteristic of the Dermatosparaxis type of EDS. Extra (redundant) folds of skin may be present as affected children get older.
• Skin hyperextensibility to some degree occurs in all types of EDS except Vascular.

So your skin has so many bruises people think you earn your living as a boxer, you might have EDS.

Cardiovascular:
• People with EDS tend to have low body temperatures, may have trouble controlling their body temperatures when exposed to heat or cold, and many have blood pressure problems.
• Some have dilated aortic roots, incompetent heart valves, and autonomic dystonia or POTS (a syndrome where you have wide and serious blood pressure swings with position changes).
• Many people with EDS bruise very easily and often severely. It can be difficult for a medical professional to "feel" their pulse.
• Mitral valve prolapse is not a sign of EDS, though someone with EDS may have MVP; it is not diagnostic for this syndrome.

In some types, arteries including the aortas are very fragile and can rupture causing a medical emergency.
Note: IV (intravenous) access and even sometimes simply drawing blood for testing may require multiple attempts; using a "butterfly" needle and syringe is much more successful than the use of a vaccutainer which draws the blood rapidly by the use of suction. People with this concern must use extreme care and inform their healthcare providers of these possibilities.

Neurological Symptoms:
• Poor balance, severe headaches including migraines .
• Decreased deep tendon reflexes.
• Intracranial vascular abnormalities.
• Brain "fog", a sense of not being present; absence of focus or a lack of clarity
• Spinal stenosis (narrowing of spinal column) and/or scoliosis.
• Chiari malformation (the brain tonsils protrude down through the forum at the base of the brain) occurs in some EDS patients.

So you had a school report card that said you were fidgety, uncoordinated, lazy, under-developed, and a complainer, you might have EDS.

Dental:
• Half of all EDS patients have a hypermobile tongue, and are able to touch at least the end of their nose with it easily.
• A high palate and crowded baby and adult teeth are common, even though many EDS patients have smaller than normal teeth. The high palate and smaller teeth can make fitting dentures very difficult even when explained to the dentist prior to the dentures being made.
• Pre-molar and molar teeth often have high cusps and deep fissures with root problems, and enamel hypoplasia can cause decay and possible early extractions. Sometimes teeth actually crumble when losing the enamel.
• Patients with Classical type offer suffer with juvenile periodontal disease. All EDSers are cavity prone, and have increased bleeding from anywhere in the oral cavity due to the fragility of tissues. Braces can cause problems as they can damage the gums and tongue while moving teeth quickly.
• TMJ (tempomandibular joint) pain and clicking occur in about 30% of the general population, and about the same incidence occurs in EDSers. Often if in a dental chair with your mouth open for an extended period of time, the joint will repeatedly sublux. Taking a pillow so you can prop your hand up to support your jaw during the procedure will prevent it from happening and also reduce your pain level from TMJ.
• Studies have proven that lidocaine (a local anesthetic used during dental procedures) often works poorly or not at all with EDS patients.
• Some people with EDS complain of always feeling like there is a lump in their throat when swallowing, and often have other swallowing and voice problems.

So a dentist ever gave you so much Novocain that his thumb was numb, and you could still feel everything, you might have EDS!

GI system:
Gastrointestinal complications of EDS run literally from one end to the other. Frequently EDSers suffer from reflux and GERD, stemming from an incompetent esophageal sphincter that allows stomach acid to backflow up the esophagus and cause burns in it. Diverticula have been seen throughout the G.I. tract.
• Tissue extensibility and laxity can also cause lack of contraction of the stomach, causing food to not move down into the intestines.
• Megacolon and rectal prolapse may also occur, primarily in childhood but megacolon is also seen in adults. (Megacolon is an abnormal dilatation of the colon (a part of the large intestines) that is not caused by mechanical obstruction. The dilatation is often accompanied by a paralysis of the peristaltic movements of the bowel.)
• Irritable bowel syndrome is a common co-diagnosis. Constipation can result from the flaccidity of the large bowel, more water being pulled from the stool the longer it remains in the colon, and from pain medications.

So your favorite foods are your digestive system’s LEAST favorite foods, you might have EDS.

Eyes:
An EDSer may have many different eye problems depending on the type of Ehlers-Danlos they have including blue sclera, microcornea (very small cornea), epicanthic folds, and wide-spaced eyes. Other common problems are:
• Many EDSers are photophobic, some squint causing an "angry" appearance and angiod streaks.
• Loose tendons and ligaments around the eye create hard working muscles that get tired. Strabismus is the medical terms for eye conditions commonly called by these various names: eye turns, crossed eyes, cross-eyed, wall-eyes, wandering eyes, deviating eye.
• Myopia (near-sightedness), astigmatism, and early presbyopia (a vision condition in which the crystalline lens of your eye loses its flexibility, making it difficult to focus on close objects.) occur often in EDS patients.
• Dry eyes are a common and uncomfortable problem.
• Other EDS related problems are detached retinas and ectopia (displaced) lenses.
• Persons with Ehlers-Danlos syndrome should see an Ophthalmologist annually so the internal eye can be checked for retinal and lens problems among other things. This is not an O.D, a Doctor of Optometry, but an MD with a specialty in eye issues.

Even during an eye exam, the exam itself can cause vertigo, nausea and headache feeling much like carsickness in some people.

* So you change your eyeglass prescription more often than your wardrobe, you might have EDS!

Pain: Pain with Ehlers-Danlos syndrome can range from none to chronic debilitating pain. It is subjective, individual, and different for each of us. For many patients, this is the worst symptom of all!Causes of this pain can be repeated trauma of constant instability from recent subluxations and dislocations as well as degenerative joint disease. Sometimes poor posture brought on by lax ligaments and weak abdominal muscles cause increased pressure on the spinal joints. Some with EDS do not have pain; others develop it later in life, and others begin to suffer severe pain as children.
• Many things are useful in treating EDS pain such as heated pools (92-94 degrees), gentle stretching, walking (if your joints allow), and emotional support that recognizes the degree of your pain and is non-judgmental.
• Occupational Therapists who make splints and assess what you may need may help to make daily life easier. Heat and cold packs help a lot. Always use cold for the first 24 hours after an injury to decrease swelling and limit bleeding into the area, and then switch to heat.
• Other possibly helpful things are yoga, relaxation therapy, massage, acupuncture or acupressure, diversion, TENS units and chiropractic maneuvers by a knowledgeable chiropractor.
• Common pain management problems are related to medications either in a too low a dose or prescribing the wrong medication, overemphasizing risks, using a "cookbook" approach, patients refusing helpful medications because they worry about addiction, and doctors afraid of prescribing because of their misunderstanding of the DEA laws.
• Often pain is undertreated in children, the elderly, and minorities. Less than 2% of all chronic pain patients (not just EDS patients) using pain medications correctly for pain become addicted. One can become dependent, but can be easily weaned off narcotics in a short amount of time.
• Medications often used with EDS are: muscle relaxants, NSAIDS, steroids, lidocaine patches, antidepressants, narcotic and nonnarcotic pain medications. Remember that over 4,000 mg. of Tylenol daily causes liver damage. Different combinations of medications work for each individual.
• Pain can be completely debilitating and keep you from needed sleep. Often family and friends don't believe you ... the worst part of all.

So you have days when you need a nap to rest up from the effort of getting out of bed in the morning, you might have EDS.

Emotional Effects of EDS:
Should either physical disability or chronic debilitating pain make your life feel destroyed, feelings of worthlessness and profound depression may set in; often talking to a counselor or medical professional will help. Regrettably, a tragedy occurs when we not only have to contend with no longer being able to do the things that we have loved doing, but also has to battle for family and friends' belief, respect and understanding. It appears that everyone with an invisible disability sadly experiences this.

While someone with EDS is mourning their loss of ability and freedom, others often accuse them of just being lazy, malingering, or becoming an addict to the pain medications that allow them to live their life. Because of this, we should not confuse their endeavors to live life and be positive with assuming they are feeling well or doing better.

Knowing you have EDS doesn't suddenly make things worse for you physically, but may allow for better physical management, and ideally allow for the prevention of any real problems, even if none exist currently. So knowing you have it is not necessarily a bad thing.

Personal doubt about one's mental and physical abilities can add to the fear that others can't possibly believe or understand what you're going through. Inability to cope with daily tasks or mental confusion can have a demoralizing effect.

But there is hope and help. You can join EDNF and learn how to help yourself, your doctor and your future. You are not alone.

So you are searching for knowledge, learning about EDS and educating others, you might have EDS!

www.ednf.org

Physical Therapy Management of the Hypermobile Patient

Terry Olson, PT, MHS, FAAOMPT, received his physical therapy training at the University of Iowa and he has an interest in the manual treatment of the muscular skeletal problems. He subsequently received his Masters Degree in orthopedic physical therapy and he developed a post graduate program in exercise and manipulative therapy. Mr. Olson is a fellow of the American Academy of the Orthopedic Manual Therapists and we would like you to welcome Mr. Terry Olson.

I am going to present this from the standpoint of looking at hypermobility. We are going to look at a definition of hypermobility, its relationship to EDS, then talk about the role of exercise and joint protection, and a short case study that I thought I would present.

Okay, what is hypermobility? There are quite a few definitions, but the one that seemed to be the simplest was that it is a connective tissue disease, where connective tissue proteins, such as collagen, which give the bodies its intrinsic toughness, are differently formed; the results are mainly felt in the moving parts, that being the muscles, joints, tendons, ligaments, which now are more fragile than is the case for most people. The result is joint laxity with hypermobility and with it comes vulnerability to the effects of injury. The young girl presented here is the one on which I did the case presentation.

Quick review of the categories.

*
Classical: Looking at skin hyperextensibility, tissue fragility and joint hypermobility.
*
Hypermobility: again, joint hypermobility is the dominant characteristic, may have joint subluxation and dislocation, limb and joint pain.
*
Kyphoscoliosis: generalized joint laxity and severe muscle hypotonia, scoliosis, tissue and organ fragility.
*
Arthrochalasia: congenital hip dislocation, severe generalized joint hypermobility, recurrent subluxations, tissue fragility, and muscle hypotonia.
*
Vascular: organ fragility with the possibility of arterial or organ rupture, tendon or muscle rupture, joint hypermobility primarily in the digits.
*
Dermatosparaxis: severe skin rigidity, skin soft and doughy and may have large hernias.

Of all of those categories, the dominant feature from a physical therapy perspective is hypermobility and joint laxity, so those are the primary considerations for a therapist when treating patients with EDS; and that means that treatment needs to focus on joint protection and dynamic stabilization of those joint structures.

Muscle stiffness is a term that is used to describe the spring-like quality of muscle. When a muscle has high stiffness, increased force is required to cause lengthening of that muscle or to perturb it. Muscle stiffness has been described in the biomechanical and neurophysiological literature as one of the most crucial variables in joint stabilization; and in the knee a link has been established between receptors in the ligaments of the joint and muscle stiffness. The slide includes a few of the references for that.

In the residency process (I went through post-graduate residency training in manipulative and manual therapy) you are primarily moving joints that are stiff. Switching to talking about joints that are not stiff, which is the case here, takes a bit of a shift. One of the things taught in the residency process was that you cannot distinguish a problem as being a joint problem or a muscle problem. The structures are so closely interrelated that the feedback between the two is critical to the integrity of that joint. If you have problems within the joint; because the joint provides feedback to the muscles, you are going to have muscular weakness. If you have muscle weakness, again through association with the joint structure, you are going to have impact on the stability of the joint. Thus, any treatment needs to be directed at all of those structures.

It is possible that the sensory properties of structures within joints can be modified by the contraction of the local, stabilizing muscles, and this is usually the proximal muscle groups. If you are looking at the shoulder, you are talking about the scapular cervico-thoracic muscles and the large shoulder muscles themselves. If you are talking about ankles and knees, you are looking at the muscles in the trunk and the pelvic girdle.

Besides providing mechanical stability to the joints, these muscles can contribute to the sensory feedback mechanisms associated in the joint structures themselves. A study done by Blazier, Carpenter, and Houston showed that tightening of the joint structures with active muscle contraction increases the acuity of that shoulder joint. That means if the muscles are functioning properly, then they are going to provide feedback to the joint which, in turn, makes a more stable situation for that joint and the joint has the ability to have feedback as to where it should be positioned.

Dynamic stabilization, or the use of exercise to promote joint stabilization, occurs when tonic (postural and slow twitch muscle units), are activated. These tonic motor units are activated during tonic continuous low load activation of muscle, maximizing muscle stiffness. What we are talking about here is asking the muscle to contract gently, not maximally, and to be able to sustain that contraction for a period of time. This can be influenced by the speed of the activity or exercise, as well as the type of muscle contraction you are trying to acquire. Muscle contractions that are performed in the shortened range of the muscle length are going to be critical in establishing the sensitivity and optimal functional capacity of the sensory feedback system in the muscle.

All that says is that when you are exercising - for folks that are afraid to exercise, for folks that have an inherent increase in mobility - exercise needs to be done in a very small controlled range of motion. Dr. Unger and I have had discussions about patients that we might see commonly as well as the patients I have seen from Dr. Mark LaVallee. One of the things that I have seen somewhat consistently is that the patients who are stretching don't need to stretch. They will tell me about being stiff and they will take their leg and wrap it around their head and it is just difficult to relate to that as being stiff. Now you can have a feeling of stiffness, if the muscles are fatigued or overworked, which to me then relates back to weakness.

Co-contraction and co-activation of muscle groups provides the biomechanical forces for joint stabilizing and protection, especially if they are performed in mid-range or neutral joint positions. Every joint, in its ability to move through range, is going to have positions that are inherently more stable because you have joint surfaces that have better contact with each other. You also have a point in that range where the muscle has mechanical advantage. If I am bending my elbow, the biceps will do that. When the bicep is in its lengthened range (elbow fully straight), I am going to be much weaker than when I get to a point (elbow fully bent) when things are going to shorten or come closer together. Mechanically this is much stronger. And, so, when you exercise EDS patients, you need to make sure you attend to placing them in those mechanically advantaged positions to work and exercise.

This is not just with EDS, but with many of the patients I see that have weakness or hypermobility. They do not exercise their full range of motion, they do not have the control to do so, and building the ability to exercise to the greater range is the goal. So, you start where the patient can perform in a pain-free and stable manner. Closed chain exercise is superior for muscle protection of the joint, although open chain exercise is also beneficial and necessary, especially again if it is performed in the protected range of motion.

When we are talking about open chain exercise/closed chain exercise we mean the following. Open Chain Exercise: if I want to exercise my quadriceps, that is the muscle that straightens my knee out, that is open chain, just moving my leg through range of motion. Closed Chain Exercise: if I stand here and do a squat, I am still working that quadriceps muscle, but by virtue of contact with the floor, we now have other muscles that act to provide increased stability. So a lot of times, starting these patients with exercises that are more closed chain, enhances that ability to stabilize muscles around the joint.

Case presentation

We take a body chart that the patient marks when they come in - and again, typically, when I get a body chart on a patient from Dr. LaVallee, that whole body chart may be colored in - it looks like they have a suit on.

As a therapist, where do you start with that - you cannot treat everything. In this case here, the young girl, the primary complaint was the mid lower back, and the other complaints were part of her overall problem; but in this case not the primary reason for presenting to therapy. Primarily, it was because of back and hip trouble. She had a diagnosis of lumbar back pain, left hip pain, and EDS multiple areas of pain complaint and again those were most notable in back and hip. She described pain complaints of an 8 out of 0-10, (0 being no pain, 10 being like a wild animal tearing your leg off).

She described her pain at her worst as being an 8 on that scale with standing for more than one hour being a provocative activity, as well as her activities of daily living. One of the things that were important at that time was that she had a baby, and that holding her baby was enough to provoke a lot of these pain complaints. She had objective signs of multiple joint hypermobility with back pain reproduction.

In her case, initial emphasis was on symptom alleviation using modalities (heat and cold, TENS units, electrical stimuli), gentle joint mobilizationand biomechanical correction, as well as performing assisted exercise in protected asymptomatic range of motion. We also provided biomechanic counseling on joint protection, as well as back care education regarding lifting, sitting, and performance of her activities of daily living.

Over the course of treatment, as her symptoms decreased, we progressed into more dynamic exercise, dynamic stabilization, and that become the crux of her home program.

One of the things that we can use in the setting that I work in, at least in the acute phases, is equipment designed to start exercising folks at less than full limb loading. If you have someone that has an injury or problems with - as in this case - knee and hip, and those problems occur when they are weight bearing, then the idea is that exercise has value because if we exercise, we have the ability to increase blood flow and circulation, increase oxygen, all of the things that are essential for healing, as well as building blocks for laying down stronger tissue. The problem is bringing that load to an acceptable level for that person.

So, in this case, this patient is just doing a bilateral squat activity and the slide board allows her/him to exercise anywhere from less than 20% of body weight taking it all the way up to 70% of body weight and then we know how to graduate that into a gym exercise. We try to exercise people as functional as we can, so if people have complaints of “when I stand too long or when I walk too long, when I bend is when I have my complaints,� I am going to try to set my exercise program up so that they are standing, walking, bending, but using the equipment to again decrease gravity, or their body weight, so that they are exercising with less body weight on those joints.

In this case, with walking, we use a treadmill system that has an overhead pneumatic assist and that harness allows us to hoist someone up. We can take that person, take as much as 70% of body weight (essentially the weight of the trunk - you go any higher than that and you are like Peter Pan and you are unstable, and you are ready to fly) then you are still getting input into the joints, but not full load.

I had a discussion with one of the participants who asked me about Pilates - I am going to beat people to the punch - I don't know. Pilates makes sense to me that it can be of value, but I don't know enough about it and I might make an idiot of myself trying to tell you the value of it.

Not for purpose of this talk, but if it gives any rationale to why; the approach that we use is a system called "medical exercise training". That approach is based on specific tissue exercise, and so we work from a standpoint that muscle requires a different muscle stimulus than does tendon, than does cartilage, than does disc. All of those structures have a different metabolic requirement, so those exercises have to be different, and when you are working with folks who have tendonitis, we try to work out an exercise program that allows that person to go through anywhere from 150 to 200 repetitions - because that seems to be the adequate number of repetitions to get an adequate increased blood flow to the area to promote a healing response. When trying to address structures such as ligament, cartilage, disc, those structures require upwards of 1,000 to 1,500 reps. The problem is taking someone who hurts and tell them you are going to put them on an exercise program of 1,500 repetitions and then say I will come back in an hour and see how you are doing. So to do that, you need to take load off, and the purpose of the equipment is to assist that part so that person can perform repetitions without pain. And then as the structure begins to heal, we gradually put load back on.

So this was the initial phase. In addition, we started to work on trunk. With Pilates or with other approaches, their value is core strengthening of the trunk - because your arms and legs attach to your trunk and if your trunk is not stable, then it is difficult to promote stability into those joints. Oftentimes the trunk is the most logical place to start, and is probably the easiest because everything else has to function off of it.

A simple leg raise will require activation of the transverse abdominal muscles if they are activated and used. The fact that her back is arched and she is unable to keep it from arching is indicative of the fact that a good exercise becomes a very poor one because she doesn't exhibit the ability to recruit enough trunk muscles to actually handle the weight. Here, she is able to keep the trunk stable (you don't see that hollow) and so she is performing that exercise actually really well with good trunk control.

Exercise is controlled trauma. When we exercise someone, we are inflicting trauma - the idea is to control it. If you exercise adequately, you are going to break down tissue, then let it rest and in this phase it recovers and lays down stronger tissue - if you adequately stress it. If you overstress it, then a good exercise becomes a bad exercise pretty quickly. All day long I see patients come in who are failures at exercise programs and it is not because exercise is not a benefit to them, they have just not been trained to do it with good form and to work in a range where they have control. You need to understand what is safe.

I had actually treated Erika about 9 months ago and she did well. I asked her to come back to see how she was doing but also to take some photos for this presentation and it was a good chance to take her upstairs and see if she was doing what we had instructed her to do 9 months ago. She demonstrates incredible form. Her elbows are going the wrong way and you would think that would make sense, but when we first went over her exercises and she did things, a lot of the things she was performing, a lot of work was in an unstable range and it was causing a lot of pain. It was just a matter of again making certain that her trunk was active and then worked in the range that she could exhibit control.

The exercise doesn't matter - when I set exercises for people, it doesn't matter, pushing up, pulling down, pushing away, pulling into them, I do a variety because life is made up of that. You need to be able to do all sorts of things but in a stable manner. The exercises need to be changed and modified as long as the patient understands stabilizing well, good trunk improvement, and that the joints are working in a range where they were stable.

I saw Erika for 9 visits over a 5 week period. Initial treatments consisted of gentle treatment to relieve symptoms coupled with assisted exercise utilizing the assisted treadmill walking and total gym. The patient then progressed to a stabilization and progressive strengthening exercise program. Her symptoms decreased, her discharge pain complaints were decreased, and she was able to stand and sit for more than two hours without symptoms, as well as lift her baby without increase in symptoms.

The final slide provides some excellent overall points in your therapy process. All of these things can be used in treatment and pain reduction. What I have seen with pain reduction, if you are heating people or icing them or electric stimuli, you are doing things that are temporary. Long lasting change is only going to come with making a change in the body, to the extent that you can. So, exercise with emphasis on controlled range of motion or working within a range of control. Pool therapy is beneficial and we use a pool at our facility also. Pool therapy takes the body weight off much the way the harness mentioned above does. Massage certainly has value, but again I think you need to be careful about monitoring skin integrity, especially if you are doing friction massage which is one of the therapy modalities often used in treating tendonitis. Use of splints or bracing - those are done supportively. Annual therapy, manipulative therapy, chiropractic therapy - it is not contraindicated, but you need to make sure you are addressing treatment to a joint that needs to be moved, not the joints that already move excessively. As a general rule, you want to be cautious of vigorous stretching secondary to inherent hypermobility. Again, I don't teach people to stretch, but they do need to strengthen because they have all of this range of motion that they don't have control of and that is painful. A lot of patient education, regarding ergonomics, joint protection, body mechanics, is required.