Living with Ehlers-Danlos Syndrome
See full version of this guest blog written for the Mummy MOT here:
30th June 2020
Hypermobile Ehlers-Danlos syndrome (hEDS) is an inherited connective tissue disorder that causes joint hypermobility. Physiotherapist Helen Forth shares what it is like to live with the condition, and the huge benefits she has gained from Clinical Pilates.
‘I was the type of child who never sat still. Or if I did, it was doing splits, or with my legs behind my head. I was incredibly flexible, and for many years this was a great asset for me as gymnastics was my passion. I joined Sudbury gymnastics club when I was five years old, with my next-door neighbour. We were quickly hooked, and it became our lives, working our way up through the ranks to competitive elite squad gymnasts. I was frequently injured, almost always in pain, but passionate all the same. Because of my injuries, I was introduced to physiotherapists from a young age and was frequently seen training with wrist, ankle or knee supports in the hope of gaining some stability. I had a few fainting incidents as a child, hated having to stand still in assembly, but no other symptoms.
I was a studious child, but I spent my playtimes at school backflipping around the playing field, or having handstand competitions. I stopped competing as a gymnast aged 14, becoming a coach and a qualified judge aged just 16. However, after a while numerous other sports took over – I was also a keen hockey and tennis player. Competing for Suffolk in hockey, and Sudbury hockey and tennis clubs. I was still often injured (although refused to stop playing) and began to suffer with my knees by the age of 15. At the time, I was convinced I wanted to be a PE teacher when I left school. Unfortunately, I had to have knee surgery in 1991 and realised that perhaps this wasn't a great career choice for me. This is when I decided to become a physiotherapist.
As a student I was aware that my body was different. I was used as a model for almost every joint or body part we were studying. I remember my supervisor trying to manipulate my lumbar spine when I was on a clinical placement, and finding it impossible, as I was just so mobile. I fainted a few times when I was standing still observing others at work – notably in surgery when watching a hip replacement, and on the intensive care unit. This was not because I was disturbed by what I saw, but rather, it was because of POTS – postural orthostatic tachycardia syndrome (where the heart has to pump incredibly fast to get enough blood to your brain). I found written exams incredibly difficult at university due to my hypermobility. Our exams were sometimes three hours long, and my right hand would be in absolute agony from gripping so tightly when I was writing.
My lower back began to give me significant trouble when I started work as a physiotherapist. I quickly realised I would not be able to work in an area where heavy rehabilitation was required, and so ruled out neurology (my early career choice). In 1998, at the age of 23, I bought my first flat and injured my lumbar spine quite badly. This was not a work-related injury, but an ‘I can move a double bed on my own’ kind of injury. It looked like I had managed to prolapse a disc, and was in significant pain for several years afterward. I had neurological symptoms in my left leg and went on to develop problems with my thoracic spine, neck, and later also had left arm pain. I remember attending a conference and having to spend the entire time lying on my stomach, as it was the only comfortable position. Hands-on physiotherapy treatment helped a great deal, but I believe that Pilates was the thing that made the biggest difference for me (and continues to do so now). I eventually had an MRI scan in 2001, which confirmed a disc bulge at L5/S1, but also a spondylolisthesis – where one vertebrae slips off another (common in gymnasts).
In 2001, I attended one of the Australian Physiotherapy and Pilates Institute (APPI) first-ever courses on Pilates for Women's Health Physiotherapy and also began attending their practice in Hampstead as a patient. I initially did equipment-based Pilates twice a week, eventually able to reduce this to once a week, and eventually able to attend a reformer class rather than individually. It was a real life-line for me. I gradually began to regain the strength in my core, and my pain lessened.
In 2006 my daughter was born. I had quite a difficult pregnancy with acute sacroiliac joint pain, which meant that I needed crutches for a few weeks to walk. My labour was long and difficult (she was two weeks early – common with hEDS – and back to back), but my pelvic pain resolved quickly after birth. I made sure I got back to exercising from 6/52 after she was born, and again found Pilates incredibly helpful. I also joined a buggy fitness class Pushy Mothers which is how I met several of my mum friends. In 2008 I had my next pregnancy. My sacroiliac joint pain started again before I even knew I was pregnant. I also had severe Pelvic Girdle Pain (symphysis pubis joint) and again had significant difficulty walking. When I got up from sitting, I would have to take tiny shuffling steps before I could get moving freely. I also had a lot of pain in my hands and feet and struggled to get comfortable at night time. I also had a fainting episode in my second pregnancy – this happened whilst I was still working and having to stand still whilst treating a patient. I was about 35 weeks pregnant at the time. My second delivery was completely different from my first – my son came shooting out at 100mph – born in just 35 mins from start to finish. This type of labour – known as precipitate – is common amongst women with hEDS.
My POTS became much worse when the children were little and I was playing on the floor with them. When I got up, I would frequently find the world had gone black and I was having to hang on to a piece of furniture for dear life, while whispering ‘don't faint, don't faint’… I still didn't fully appreciate what this was. I’d also find that I couldn't pick something up from the bottom shelf in a shop, without then finding the world was spinning when I stood up again. I had the same exacerbation in my POTS symptoms when we had a new puppy, getting up from the floor quickly can be a real issue.
As an adult, I have also suffered with headaches, gut issues, anxiety, and later Gastro-oesophageal Reflux Disease (GORD) – all conditions which I now know are commonly associated with EDS. In 2016 I finally decided I was fed up with feeling like I was inventing these problems and decided to seek a formal diagnosis. As a physiotherapist, I was already well aware that I was hypermobile, but it was such a relief to see Prof. Rodney Grahame and to have the hEDS diagnosis confirmed. He then encouraged my GP to refer me for further testing within the Autonomic Team at UCLH, where my POTS was also confirmed. UCLH provided me with excellent support to learn how to manage the POTS (they trusted me to know how to figure things out from a Physiotherapy point of view!), and their advice has been invaluable to me.
Throughout this time, I had continued to attend my weekly Pilates sessions, but in 2018 I decided I should also train to be an APPI accredited Pilates matwork instructor. Since training and teaching numerous classes every week, my body has never felt better. I have even started running 5K regularly with no ill-effects (something I would never have previously been able to do without my body breaking down). Honestly – for my hypermobility, clinical pilates has been the answer. My kids are both hypermobile, and they already do Pilates regularly as a preventative measure.
My hEDS is not severe (some sufferers are wheelchair-bound, and have many other more severe comorbidities), but it is a significant part of who I am, and has affected and continues to affect my life in many different ways. I am in pain in some part of my body every day. My patients frequently comment that I am always moving, that I never stand still for long when I'm treating. I can't have a shower the moment I wake up in the morning and why I need to drink inordinate amounts of water (to keep my blood volume up). I also now understand why I have never felt particularly well when visiting museums or art galleries – again standing still. I have also made sense of the mystery of why my trousers always felt tight when I put them on after a day in uniform on the wards – all the blood would pool in my legs and feet during the day. I'm just sad it took me until the age of 41 to understand.
I’ve written this blog, to raise awareness. These conditions – the hypermobility spectrum disorders, are still widely misunderstood and undiagnosed. I hope it might give the parents of a newly diagnosed child hope, that they will be able to find ways to manage their condition and live an amazing life.’
Helen recommends the following websites and support groups: