My journey with physiotherapy

By: Sara Bentley

Like most Zebra’s with EDS my journey to diagnosis and therefore treatments has been somewhat long and convoluted. With highs ranging from marrying my wonderful husband, having two incredible children, managing to run a half marathon 9 months after having a pacemaker fitted, then to the lows of constant pain, losing consciousness to the extent I was required to be on bed rest for the last three months of my second pregnancy and a dramatic final straw that meant my treatment was expedited when I lost consciousness emptying the dishwasher, and landed on an upturned knife, effectively stabbing myself. 

Hindsight is a wonderful thing, looking back at my childhood and comments from my dance instructors about how incredibly ’supple and mobile’ I was, severe dental overcrowding that required 10 teeth being removed, my jaw dislocating (but thinking it was normal movement) and lightheadedness, especially during certain parts of my cycle. There were many clues. Going into adulthood, completely oblivious the evidence was mounting, being diagnosed with PCOS after having two cyst’s removed that grew out of hand, my gallbladder removed after having gallstones aged 20. Unstable joints, that I attributed to clumsiness and increasing lightheadedness, and feeling faint, whenever I had an even slightly diminished fluid balance. Constant nausea, frequent urinary infections, kidney stones, and relentless pelvic pain. This was all before I had even turned 21. 

Fortunately, I then seem to have a period of respite, in which time I got married, and fell pregnant with my eldest child. At the time I was still unaware of my diagnosis. I had significant tearing during the delivery. Subsequently, I have been advised that had they had knowledge of my condition they would have taken me to the theatre straight after delivery. However I didn’t know, so I had a simple repair. Before long I had a prolapse. I was initially dismissed and told to come back when I was no longer of childbearing age. However in becoming a Mum, I had also found the strength to advocate more for myself,  I had someone to care for. I pushed for treatment and received anterior and posture repair. This alleviated some of the pelvic pain, I experienced fewer episodes of urinary retention and felt I was starting to feel more in control. No one including myself had pieced together my history. Having moved around geographically probably didn’t help. It made it easier to be dismissed as someone who worried, or had a low threshold for illness. 

 Two months prior to falling pregnant with my youngest child I started losing consciousness. I had previously had fainting episodes but they were months apart, and normally coincided with having a virus or pain, so was easier to dismiss.

I began losing consciousness several times a day. I was fortunate, this happened in hospital a couple of times, whilst I was attached to a monitor, which helped speed up getting me to the right department.

Whilst admitted to the hospital cardiology did the ward rounds and came to see me. One of the more junior doctors suggested I might have POTS, she was openly mocked in front of me, they told her not to look for ‘zebras’. Unable to find an alternative diagnosis, they referred me to a cardiology clinic with a professor who agreed with the POTS diagnosis. By this time I was three months pregnant and unable to have a tilt test or take any of the first lines of treatments. I was losing consciousness up to 8 times a day.

The pregnancy was fraught, I spent a lot of time under observation and was having to inject myself with a drug called Octeotride 8 times a day to try and make my blood vessels dilate, and increase my blood pressure and reduce the episodes of loss of consciousness. 

I was induced early, at a point where they were happy my sons and my safety were balanced, and I was able to start treatment for POTS. I found some relief for a few months. 

However, before long I was losing consciousness regularly without warning throughout the day. This resulted in a lot of dislocations (mistakenly attributed to the trauma), falls, many injuries before landing on a kitchen knife when passing out. 

Thankfully by this time my local hospital had recognised despite confirming POTS with the tilt table, they couldn’t treat me any further, so referred me to the incredible syncope unit at the Royal Brompton Hospital. This is where once again like the junior doctor luck was on my side, the locus doctor at my local hospital knew of this syncope unit, having trained with one of the consultants. 

After a couple of months of heart monitors and an ILR (plus a phenomenal amount of support from specialist nurses) the team had enough evidence to conclude a pacemaker would help. The ILR & monitor together showed my heart rate was displaying bradycardia, along with low blood pressure. Then I would get a sudden episode of tachycardia with a return to bradycardia or a rate of about 29/30 within seconds. Which is when I would pass out. The pacemaker instantly helped, along with medication to increase my blood pressure. I stopped losing consciousness without warning. Suddenly I had my quality of life back. I even managed to run the Royal parks half marathon to raise money for the Royal Brompton as a thank you for the life and sanity-saving care. It wasn’t an amazing time, but given both my big toes dislocated during the race I was proud to finish. The previous year I would not have felt safe going as a spectator let alone a runner. 

Sarah after the Royal parks half marathon

However, my joints were still dislocating with an absence of trauma. Just going upstairs would make my ankle sublux. Having been a member of some POTS support groups I heard about hEDS, and couldn’t believe it when I went through the criteria. I ticked every box. I went to my GP braced to have to fight for a referral, but they agreed so sent me to rheumatology and finally I had a diagnosis. Which was a relief as my joints began dislocating so much more frequently. I had no idea how to manage. I tried self-managing where possible. Running had to stop, most exercises were having to stop which was not great for my mental health. 

Obviously, there is no easy fix to hEDS, for me my way of coping is seeking every self-management strategy possible. It’s a way of me regaining control, against a body that fights me for it constantly. 

Also going to A & E when you dislocate or sublux every other day is not an option. You get helpful professionals, but even with a diagnosis, there is still a lack of understanding around the condition. So you are not always believed, or even when you are it’s hard not to feel like a burden. This was prior to their being a global pandemic. It’s hard to not feel like a drain on resources at the moment. Having children at home makes the hospital even less practical. Being able to self-manage is vitally important to me, as it helps me to still parent, which is such a huge part of my identity. Whilst I accept my hEDS and that it will limit me in ways I’m not even aware of yet, I am desperate to stage that off for as long as possible. Physio seems to help me with this.

I was receiving physio at my local service and had found a great physio. He was immensely helpful and knowledgeable,  but he was limited by referral processes, so could only see me for one joint at a time. This was useful, as it showed me that physio could help me. I was finding the more physio I could do, the fewer episodes of subluxation in that joint I would get. I was beyond grateful. This physiotherapist too time to learn more about hEDS, once again luck was on my side, and this physio made up for so many negative encounters, like being called a ‘fainty girl’, or a lazy person, or that I couldn’t possibly have dislocated in the way I said I had.  

I needed to feel like my condition as a whole was being managed rather than falling through the cracks and each specialty just working around it.

At this point, I decided having been recommended by Dr. Mittal and having seen such a wealth of information from Jason Parry to try and see him privately at the London Hypermobility Centre. I knew the benefits of physio on one joint, so wanted to extend that. 

Not only did Jason give me guidance of how to reduce dislocations calmly, and resisting the urge to be quite heavy-handed in putting joints, through fear if I don’t do it there and then it won’t go back, on contrary if I slow down, take pain relief and get into the right position for the joint, it goes back, and there is a lot less irritation to the area, from panicked failed attempts.  Once again I can self manage more, only having to attend  A & E when my skin tears as a result of the dislocations/subluxation and I need stitches. He gave me an idea of what to put in what I call my rescue kit. 

He gave me a full assessment, looking at which joints were most unstable. He worked through a wish list and the order with me of what I want to achieve from physio and if it’s real. I’m sure lots of people can relate, but the difference it makes psychologically when you meet a healthcare professional who not only knows your condition, they specialise in it. You are not on the back foot, having to either convince someone your condition exists or educate them on it, they already know and want to help you manage it. In my next session and subsequent session, Jason had put together a series of exercises that would be the start of strengthening and retraining the surrounding ligaments. Not only did he show me the exercises, but he also checked I could do them, and worked through them with me. If I was unable to do them or struggling with them, he took the time to work out why, whether it was the mechanism or the loss of muscle in the area. If it was something that would be painful he found an alternative.

So between the improvement I am starting to see thanks to the physio and beginning to encounter professionals who help me self manage including my GP and the practice nurse, I am taking control back little by little.

My rescue kit includes pain relief, muscle relaxants, salty snacks for blood pressure, water &  heat source, and dressings. Every little thing I have at my disposal accumulates to mean I can leave the house. I won’t lie some days are tough, on the third patella subluxation of the day, with lightheadedness due to the pain-causing and increased heart rate, the relentlessness of having a cycle of subluxations, meaning physio and normal exercise is reduced, the strengthening is diminished and more episodes occur. It can feel like a vicious cycle you can’t beat. However, I just try every day, and sometimes it works. Sometimes it doesn’t. I’m getting better at accepting that it’s ok to take some days off. That it doesn’t mean that I’m weak, or that hEDS is getting the better of me. It just got the better of me that day, tomorrow is a new day and if I rest up I will have better places to deal with tomorrow.