Welcome to Elhers Danlos Syndrome; 10 Key Management Strategies
I was diagnosed hypermobile at 8years old, but it would be another 34 years before I was formally diagnosed with Ehlers Danlos Syndrome hypermobility type. That was a long and winding road folks. I am filled with anger, sadness, regret, frustration, disbelief, and relief. Even though I still struggle daily, I have learned a few helpful things along the way. There is no single right path. There is no special formula that will cure you, or make you consistently better. We all have to trial and error a variety of management strategies - that may work some days, and not others. The number one management strategy is mental management, and there is no getting around it. Every day, no matter what we have to cope with what is thrown at us. Whether we are having a high pain day, a bad doctor’s appointment, dysautonomia symptoms, or normal people problems.
10 Strategies for Managing EDS
1. Collaborative Primary Care Physician (PCP) This is crucial. Your PCP is the gate keeper to all other care. To labs, imaging, specialists and treatment. A lack of treatment and care centers means patient led care and a collaborative relationship with a PCP is the cornerstone of finding some sense of stability in symptom management. A family Doctor or General Practitioner may feel ill-equipped to manage the complexity of your case. The reality is the specialists also feel this way and we are left without adequate care. The Elhers Danlos Society has seen this problem and created a forum to help health care professionals called “Project ECHO” you can read more at https://www.ehlers-danlos.com/echo/#1552933180941-1fa06360-7b58 If your current provider is not listening to you or providing care, please seek medical help with another healthcare professional.
2. Build your Team I was a very independent person. I could do it all. Looking back I have no idea how. I paddled rivers, climbed escarpments, raised boys, taught young children, worked with people with disabilities, and even ran a small farm at one point. I liked to cook from scratch, build things, grow from seed, travel… When Mast Cell disorder made it challenging to eat, drink, and left me with intractable migraines, I struggled to care for myself. This was the first time I really needed to depend on someone else as an adult. I didn’t like it. It took a few months to arrive at an immunology clinic, but only 5 minutes once there to be diagnosed with Mast Cell Disorder. I was put on appropriate medications which gave me my life back. For a few years I was doing OK. Then Influenza triggered POTS, which angered the Mast Cells and issues with the undiagnosed EDS went wildly askew. In the years since I have put together a pretty great team.
These are the top 3 therapeutic interventionists who make it possible for me to manage some degree of independence on a daily basis:
Physiotherapist - I have one main PT who manages most of my joint and instability issues. She is not specialized or even all that skilled in hypermobility, but she pays close attention and has phenomenal grasp of biomechanics. I have had a PT since I was 8. I have seen more than 25 physiotherapists, and out of all of them, only 3 have been helpful with my hypermobility. Several have significantly injured me and the others didn’t do much at all. Please don’t give up if you have tried a couple and they were not helpful. Most of their training is to treat people with the opposite problems we have and they really do not know how to manage us. There are diamonds out there, they are the difference between daily dislocations and stability with balanced muscle tone. I also see a few at the hospital for the therapy pool program - WOW that is amazing, if you have an opportunity to try the “Spinal rehab” program at your therapy pool. The spinal program really focuses on core stability and spinal stability. I found it very helpful for my dislocating hips too. If you have other problem joints, for example shoulders, you can go for shoulder rehabilitation.
Orthotist - if you do not have one, go get one right now. I will be posting an article on orthotists later this months, but in brief; these are the folks who make custom braces and orthotics. Some of these are covered by government programs and/or insurance. For example AFO’s (ankle foot orthotics).
Occupational Therapist - An OT can really make your life so much easier. They can provide alternative strategies and equipment for energy conservation, poor grasp, painful hands, finger splints (silver ring splints), as well as cognitive stratiges for episodes of brain fog. In some cases these can be provided by community services in your home.
3. Brace, Tape, Mobility Aids I will do separate articles on each of these, but in general it is important to know they exist and they are the glue that can hold you together. There are still doctors and interventionists that believe bracing and splints will cause muscle atrophy. Most EDS specialists will recommend the use of braces and splints since the ligaments and tendons are not functioning appropriately and the muscles cant always manage all the workload. Yes PT and exercise are the first line of treatment, but if this is not enough, if there is still strains, sprains and tears happening regularly - Or pain and instability happen regularly, it is time to brace, For every joint there is an option to tape or brace. A PT can help you with taping. An Orthotist can help you select and fit the most appropriate brace, and an OT can help you select the best mobility aid to suit your needs and lifestyle. Most of use wait way too long to use any of these aids and once we get them (and get over the stigma of using them) cant believe we waited so long. My AFO’s came years too late, I had no idea I needed them. Once I started wearing them I was amazed at the reduction of pain in my ankles and feet. I fell less, and felt so much more stable. I should have been in them years earlier. When I got my wheelchair it was the most freeing experience. I was quick, I had energy, and stamina and such a huge reduction in pain. My best advice *******DO NOT WAIT FOR SOMEONE TO TELL YOU THAT YOU NEED A BRACE OR A MOBILITY AID. You are the only one living in your body. I know it can be hard to know when it is time after having your pain and concerns dismissed for so many years. If you are really struggling as to whether you “need” anything or not, try some Kineso Tape (there are a lot of videos on Youtube on how to tape every possible body part) and you can rent wheelchairs, forearm crutches, canes… from medical supply stores to see if they are helpful for you. If they make a difference, talk to your doctor or healthcare team member about the aids that work best for you.
4. Exercise That word can bring tears to many members of this community. Many of us were very active, some of us came from athletic backgrounds. It is hard for many of us to believe we are “deconditioned” when we are lectured about POTS. So lets look at this from another angle or two… our muscles are doing double duty compensating for our ligaments. Often they are unbalanced, tight, and spasming from subluxations and dislocations. Additionally with dysautonomia the blood vessels are dilating inappropriately and we often try to compensate by tensing our muscles. All the distress from EDS, medical appointments, lack of care, fatigue, disability… leads to issues with mental health and… tense muscles. What are we left with? Muscles that are over worked, tight, spasming and fatigued and here I am suggesting you do more. That is correct. But not all at once. There are books and protocols and groups, but really if you just start with a few exercises from your PT (the best way to figure out where the muscle imbalances are - what isn’t firing when it should), or even just a couple squats, leg lifts, and bicep curls a day this will increase your circulation, improve mood, ultimately help the muscles relax, and build a little more capacity. Of course in a perfect world we would all have massage therapists on stand by, acupuncture, and personal trainers too, but I know that certainly is not my reality. PT in the pool is amazing. Wheelchair sports are fun, engaging, social and provide exercise. Resistance bands and seated works outs are gentle - there are many POTS friendly works outs on Youtube now. It is key to start slow and work your way up. Please understand it may take many weeks to feel progress and that going really slow is imperative to avoiding injury and preventing further fatigue. It is OK to work out just 2 minutes while laying down. The important part is to start. Not where or how we start, just that we start.
5. Managing POTS This one gets me every time. So sneaky! Sometimes I think I actually have Dysautonomia under control. Only to realize, no, no I do not. I push and push and crash, I have learned so many times, this way does not work, and yet I still devote myself to this method. Here’s what I know works for me; medication, salt, electrolyte drinks, compression calf sleeves (or socks), squats (so many), laying down a few times in the day - not to sleep I just need to remove the orthostatic intolerance part of the equation and it seems to give me a little reboot. Doing my learning, reading and writing while laying down. Stress Management. I have very little thermal regulation, so ice packs in the summer, and heating pads in the cooler seasons. Interestingly when I did my spinal program in the pool, it significantly improved my POTS symptoms. If you have a chance to try out how you respond in a warm therapy pool for 20 minutes just walking and paddling around - I highly recommend it.
Reducing the amount of time I need to stand for anything; cooking, washing dishes, gardening… If your POTS is not well controlled it will make your EDS and other conditions much worse. We are so different on our path to stability with dysautonomia, it is nearly impossible to give you a regime to follow and suggest it will magically cure you. The above strategies have helped me get to a more stable place. I still struggle a great deal, and sometimes am flat out. Most of what I based my recovery on was from the book “The Dysautonomia Project” : https://thedysautonomiaproject.org/
6. Managing Mast Cells If it isn’t POTS its probably mast cells. I once had an immunologist explain it to me that being a trifecta is like a stool with 3 legs; EDS, MCAD and POTS. If one of those conditions is not well managed the whole stool falls over. That moment changed my approach to treatment seeking. I didn’t try to get help for the most bothersome symptom anymore. I looked at the cluster of symptoms that were presenting and asked myself were they EDS? POTS? MCAD? Mast cells have a way of causing a lot of problems. From migraines to GI issues, bone pain to hives, really mast cells are all over the place! But you do get to learn your own expression. You learn your triggers, what you can get away with eating one day and when you definitely cannot eat a “maybe” food. When a migraine is CCI (cervical instability) or mast cell related. The best thing I did regarding my mast cells was an elimination diet. It gave me a very clear picture of what foods are triggers, what foods I am sensitive to but can on occasion eat. And that process helped me to really hone in on how my mast cell symptoms presented. So when I am in a new environment I am more able to discern if I am having issues with POTS or MCAD. This is really helpful because I can treat MCAD issues with my medications very effectively. And if it is POTS I know I can lay down and 80% of the time that fixes the problem. An elmination diet is best done with a Registered Dietitian, but there are articles explaining the process on line. I was in a really bad way when I had to do mine. I was down to rice and peas.
The other thing to consider is latex allergy which is common with mast cell disorder. Not only is there the allergy to latex but also “latex cross reactive foods.” This really blew me away, I had reactions to almost everything on the list! The top offenders are avocado, banana, chestnut, kiwi, peach, tomato, potato and bell pepper (source), but I have seen other foods listed as well: apples, melon, papaya (source). By far the most extensive list cor cross reactive foods was at http://www.mygutsy.com/healing-update-2/ where she lists a whopping 48 latex cross reactive foods! One other option is the low histamine diet. There is no objective data on this, mostly due to lack of studies. However there are many subjective reports that low histamine diets are helpful to those in the MCAS community. I have not tried the diet myself as I am very happy with the outcome of the elimination diet. I would love to hear from those in the community that use diet to help control symptoms, what diets they follow, particularly challenging foods… please feel free to connect with me on any of the platforms.
This is a photo excerpt from please read her full article for complete context regarding cross reactive foods http://www.mygutsy.com/healing-update-2/
7. Sleep If you know how to do this, please share. Although I understand the basics of sleep hygiene; an evening routine, no food a few hours before bed, relaxation protocols within an hour before bed, no screens close to bed time… I don’t actually have difficulty falling asleep. But getting a restorative sleep, that is rather illusive. Dr. Alan Pocincki did a webinar with The Ehlers Danlos society where he discuss fatigue and a unique sleep disorder found in EDS and Dysautonomia patients that is often missed in sleep studies https://youtu.be/m7TK_reO82c Dr. Pocincki suggests that the sleep cycle is disrupted in a way that is not generally calculated or evaluated during typical sleep studies and therefore is missed when EDS patients have their sleep study done - and surprise another test comes back “normal.” Double surprise - it isn’t actually normal. He does discuss some treatment options in his lecture.
8. Nutrition This is another tough one. Between POTS, MCAD, and GI issues/gastroparesis how can we possibly get adequate nutrition? Like every other EDS question the answer is it is individualized. For me the elimination diet helped me to find safe foods… but during periods of gastroparesis not much goes in. At those times I practice mindfulness, stress management and mental management - because that is really all I can do. Here is my other trick. I have limited energy, so I choose one day every 3 weeks and spend a couple hours batch cooking. I make 30 frozen dinners. I package them in serving sized containers and then each evening I can pop the contents of my tummy friendly meal in a pot and heat for 3-5 mins. This saves me so much energy and time. I already know I cannot manage more than 1 cup of food in my stomach at a time, so the portion size is perfect. Pre-planning these meals on a good day helps me plan meals that are more well rounded than when I am exhausted, nauseous, and really don’t want to eat at all. My best advice - eat small amounts of food throughout the day, stick to your safe foods that are more protein, fat, fruit/veg, than they are simple carbohydrate (even though our tummy can usually take that in a little easier they do not offer many nutrients and are a little slower to move through the GI tract). and don’t forget the treats. We have it pretty rough, its OK to indulge in a little chocolate, or chips or whatever your version of a treat it.
9. Pain Management Without pain management there will be no good sleep - without good sleep the pain will be worse. Pain effects our GI system and appetite, our mood, our ability to exercise, perform self-care, hygiene, dysautonomia, and mast cells. EDS effects how pain medications work in our bodies. First line is PCP, which is why it is so important to have that collaborative relationship with your PCP - there are mild analgesics to start with, SSRI’s, SNRI’s, NSAIDS, and more significant pain medications. There are also biologics - medical marijuana. The thing with pain management is, it will never just be about the medication. There will always need to be some mental management piece and complimentary care. Psychology/social work, mindfulness meditation, hypnosis for pain management, acupuncture, massage therapy, hydrotherapy, support groups… Personally, I use a combination of NSAIDs (because my MCAD lets me), antispasmatics, muscle relaxants, PT, hot packs/heating pads, Kinesio Tape, mindfulness/meditation, hypnosis for pain, wheelchair and mobility aids, pillows and bolsters for sleep, light massage (mostly from myself and partner because COVID), hot bath (POTS not always happy about that), and chocolate. Again, this is very personalized, many EDSers cannot use NSAIDs, some cannot use muscle relaxants, some are in such unbearable pain they need a heavy duty pain pain medication. Some patients do extremely well on Medical Marijuana, which in reports has not been shown to spark fury in mast cells. The bottom line is pain management is critical for over all health and quality of life, this depends on your relationship with your PCP.
10.Mental Health We live with a condition that has no cure, no real treatment, and most doctors wont even see us. We have been dismissed for years and decades to only finally be diagnosed, when we are literally falling apart. Although anxiety, depression and other mental health issues are not the cause of our disorder, the trauma we have often experienced as a result of our condition (and lack of care), certainly play a role in our state of mind. The very nature of dysautonomia creates anxiety. The neglect from care providers for years and decades can create a sense of hopelessness. A solid mental health strategy can be the difference between pulling you through to keep seeking treatment support and completely giving up.
Firstly please know you are not alone. Every area has a suicide hotline and a crisis hotline. In Ontario, Canada the crisis line is 310-COPE. Please feel free to use this or any of the hotline numbers anytime you need support. Secondly there are a multitude of support groups through Facebook and other platforms on-line. It does take some time to find a group you feel is a good fit, that is normal. We don’t all have supportive family or friends, so reaching out to the EDS community or communities for the other conditions you have is so important. It is a place to be heard and understood. Next, self-care, yes you deserve it! In Canada and the USA many hospitals offer Stress Reduction Mindfulness courses. Managing your levels of stress and anxiety really does help to reduce the amount of suffering you experience. You can do the at home version on your own, at your own pace by ordering Jon Kabat-Zinn’s “Full Catastrophe Living: Using the Wisdom of Your Body and Mind to Face Stress, Pain, and Illness” - which the program is based on. I believe this is also available on audible or through your public library at no charge. If you have the resources to do so a counsellor of some variety, MSW, Psychologist… can be very helpful in coping with the medical trauma, your personal trauma as well as coping with pain and mental health in general. Your PCP may know of free community programs, or government paid for programs/subsidized programs. Please make sure to ask them about this.
Final Word… That was a lot crammed into 10 items, and it barely scratches the surface. But it is a start, and we all have to start with a single step in one direction. So chose one place to start and move forward from there. It is a process to find stability in a body that is unstable.
Join the conversation at Homesteading with a Disability where we are all experiencing issues with disability and chronic illness.