Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome, according to many sources are interchangable terms. I won’t linger on that in this post, as that’s not its purpose. Whatever you want to call it, not many people are aware of what it is and what it can entail. Whilst many people are hypermobile, EDS and JHS are a whole different kettle of fish and can bring joint pain and a whole plethora of other problems. Joints may dislocate, the sufferer may have stretchy skin and/or bruise easily and may have problems with their bowel or other organs.
If there’s one thing I’ve learnt from my blog, it’s that although I may be an introvert, I can still get my voice heard. So far I have helped people by sharing my problems with anxiety and breastfeeding after Autumn fell seriously ill , as well as my postpartum body confidence issues. I have also written a post about my husband’s trichotillomania. As such, I thought I’d also use it to try and spread awareness of EDS/JHS this EDS Awareness Month!
I have slight scoliosis and have a winged scapula which I often suffer with pain from. It was when I was receiving physiotherapy for the latter in late 2014 when it was noticed that I had hypermobile joints. Completely by chance when I was doing one of the exercises, my physiotherapist noticed that my arm wasn’t normal when I put it out in front of me. He tested some of my other joints and I got a Beighton Score of either 6 or 7. I can’t remember which, but 4 or above is hypermobile in adults. He talked to me about JHS and suddenly it all made some kind of sense. The clicky toes my parents had had X-rayed as a toddler, my painful joints particularly in damp weather, my stiff and painful knee that sometimes caused agony when walking up steps. If I get stuck in traffic and have to use the clutch a lot, it feels stiffer and really hurts for the next couple of days. Occasionally, I’ve struggled to fall asleep and kept waking up because it has been so painfull. JHS potentially even explained the scoliosis, winged scapula and my Irritable Bowel Syndrome. It could explain why my wrists are weak and why them and my ankles have given out from time to time over the years. My jaw often clicks or pops if I try to take a big bite out of something, or I can do it on demand. I also occasionally have a problem with my thigh where I feel something ‘pop’ and it causes a lot of pain. I have to wince and grit my teeth while I move it back into the right place. Although I haven’t officially been diagnosed, as the doctor I visited about it ‘doesn’t believe’ in JHS, everything adds up.
However, I don’t have it anywhere near as badly as many people. Everyone’s story is different and this post won’t sum it up completely, but hopefully it will give you a better idea.
Mind the Mummy’s Story
Mind The Mummy is a fellow blogger who you can find over at Mind the Mummy. Here is her story:
Living with hyper mobility is more than clicking joints and hips that suddenly pop out of place when you’re walking. Depending on the severity it has the potential to really reduce your quality of life. In my case I have been lucky that I have been able to lead a normal life despite some limitations. My issues have mostly revolved around postural problems because my joints don’t ever lock and like a bendy doll I hunch, bend my knees backwards and sit and stand in all sorts of awkward positions which then aggravate the condition further.
At 14, when my breasts started to grow I hunched to hide them and my posture worsened to the point where I had to wear a back brace to keep my back straight. But the difficulty resided primarily in the fact that even if I wanted to I could never sit or stand straight because that’s like asking a puppet to suddenly find a central point of stability. It just doesn’t happen. I developed scoliosis, and pronounced lordosis and kyphosis. I never really grew much, I am five ft tall, and hated all sports except ballet, which I was pretty good at but ironically it wasn’t the best for me. I took refuge in food and that further impacted my postural issues. My joints, especially my knees, hurt most of the time when going up and down stairs and if I’m out dancing or if I run after my son in the park for more than 10 minutes I know I will be in real knee pain the next day. My feet twist suddenly when walking which often presents the risk of serious sprains. In gyms, in the rare event that I go to one because I am too scared of doing something which will make my joints hurt and because gym trainers don’t know enough about this condition, I have always been the one who needs help to stretch because a self stretch just doesn’t go far enough.
As much as I love yoga and pilates these are actually no-nos for me because they make you even more flexible. I end up staying away from gyms and classes altogether even though that is not exactly a recipe for healthy living. After having my son I was left with diastasic recti, a condition where your tummy muscles separate to make room for baby and then never stitch back together leaving you with a gap in the middle of your stomach and very little back support. A slouchy connective tissue and a lifetime of postural problems plus my petite height (5 foot) were all to blame. I also have pretty bad and painful TMJ, early onset osteopenia (a precursor to osteoporosis) and a host of gastrointestinal problems that have had doctors scratching their heads but never produced an absolute diagnosis. I was always given IBS as a cause for my extreme and constant bloating, constipation and general slow digestive system but a rheumatology specialist that has been following me for over 20 years has hinted that hyper mobility may have been playing a part in it all these years. It’s a struggle but I know I am not suffering as much as other people whose lives have pretty much but been interrupted by this problem. As age pushes on I live one day at a time and am trying to find the right kind of exercise to do, to ensure I can hold on to my mobility and physical independence for as long as I can.
Abbie is an old work colleague and friend of mine’s daughter. Here is her story, written by her mum, Lisa:
Abbie was diagnosed with Ehlers Danlos Syndrome Type 3 when she was 11 years old. We had some ideas thats Abbie was struggling from an early age. Abbie was reluctant to walk and constantly complained of pain and fatigue. We noticed she was extremely flat footed and walked with a slight limp. Abbie also had problems with her bowel. She was constantly constipated and had pain in her stomach. At her worst she was on 12 laxatives a day just to produce a bowel movement. Abbie spent days and weeks in hospital whilst doctors tried to figure out what was wrong with her but no-one mentioned EDS.
After lots of reading and researching online we came across EDS. Abbie seemed to tick all the boxes for this genetic illness. Frequent dislocations, pain and fatigue, speech issues, hind foot deformities, arthralgia and chronic constipation to name a few! We were convinced that Abbie had EDS but needed a medical diagnosis. This is when life became very difficult as we tried to convince medical professionals to listen to us. No-one seemed to care blaming it on a poor diet and a lazy child. We felt like giving up until one day we found a doctor who knew about EDS. He agreed with us and sent us to a specialist in a top London hospital. A referral to a genetic consultant in London gave us the diagnosis. EDS 3, hypermobility sydrome! Abbie had an ACE stoma fitted aged 12 and needs daily bowel washout/flushes to open her bowels. She uses a wheelchair occasionally when she needs to and goes to a special school where she can be looked after and where her illness is understood. She receives daily physio and therapy and has learnt to administer her own medications so that she can become independent and manage her illness as she grows up. EDS is a life long progressive illness to which there is no cure. In simple terms the body cannot make collagen properly hence lax joints and poor muscle tone. EDS is a complicated illness which affects every individual in different ways but with help, support and understanding, life doesn’t have to be difficult.
When I was talking about writing this post on Twitter, Wendy kindly reached out to me and wrote the following. The following explains hypermobility far better than I have, including the Beighton Score, which I mentioned briefly earlier:
I am Wendy Rohin, PT, DPT. I am a pediatric physical therapist and you can find me at my website, everythingbabies.org
Hypermobility is common among the children we see for physical therapy. When children’s joints are excessively flexible or move beyond the normal range, we describe the child or the child’s joints as “hypermobile.”
Children and adults who are “hypermobile” may be more prone to:
Back pain, joint pain (growing pains)
Increased injuries like ligament sprains and joint dislocation
Weakness or muscle fatigue
Clumsiness or frequently falls
Popping or clicking in the joints
A tendency to join the circus 😉
Hypermobility is a characteristic or symptom of MANY other physical conditions like:
Hypotonia–low muscle tone causes too much mobility in the joints
Down Syndrome–low muscle tone and loose joints cause weakness
Muscular Dystrophy–the muscle weakness causes too much mobility in the joints
Ehlers-Danlos Syndrome– the connective tissues (the tissues around the joints or other body parts, not including the muscles) are too loose, causing hypermobility and weakness. Connective tissue is everywhere in the body including the skin and vital organs like the stomach and the heart. So some kids with EDS have problems with digestion, blood pressure, and thin skin.
But it is important to note that some babies and children are hypErmobile without any other known conditions or syndromes.
Babies may fall behind in their milestones because of the hypermobility.
And some young children may not be aware of their hypermobility until they start to have problems with muscle fatigue, lack of coordination or frequent injuries. They may just be called “accident prone.”
For a lot of our patient-kiddos in physical therapy, their joints are hypErmobile because their muscle tone is “hypOtonic.”
Hypotonia is a fancy way of describing muscle tone (the tightness of the muscles) that is more loose or floppy. When the muscles are floppy, then the bones are loosely connected at the joints, making them hypermobile. Kids with floppy muscles and loose joints can be weak and uncoordinated, and everything they do to move and play takes a lot more effort, so they get tired easily.
In physical therapy, we work on strengthening the legs, tummy and shoulder muscles (known as the core or stability muscles.) The stronger the muscles are around the hypermobile joints, the fewer problems kids will have like pain, sprains, and dislocations.
We also work on balance, coordination and endurance so kids with hypermobility can participate in all play activities with more confidence, more energy and less pain or injuries. Yay!
So if you have any questions at all about the looseness of your child’s joints or the floppiness of your child’s muscles, I’m your girl! Don’t hesitate to reach out to me. No question is a dumb question, and no concern is too small when it comes to your children’s health and happiness!!
If you are curious about how hypermobile you or your children are, you can do the test on your own using the Beighton Hypermobility Score.
(Disclaimer: This test is not diagnostic. If a child scores a 7 or above, in just means their joints are hypermobile. The score does not mean they have hypermobility syndrome or any associated diagnosis. Your doctor is the only one who can diagnose your child.)
**Important note before taking the test:
Children are naturally more hypermobile than adults. A score 4-6 is still considered NORMAL for children and greater than 6 is HYPERMOBILE. (For an adult, a score of 4 or greater is considered hypermobile.)
Use this link to see a video and take the test: Beighton Hypermobility Score
Please get in touch if you or someone you know suffers from EDS/JHS, I’d love to hear from you! For more information, I can recommend going to Ehlers Danlos Support UK.