My Journey To Understanding Hypermobile Ehlers-Danlos Syndrome (hEDS)

Have you ever been told that you’re “double-jointed”? For some, it’s just a cool party trick. But for others, it can be a sign of a condition called Hypermobile Ehlers-Danlos Syndrome (hEDS). hEDS is a connective tissue disorder that affects the collagen, which is the protein that gives your skin, joints, and tissues their strength and structure.

While hEDS isn’t the most well-known condition, it’s actually thought to be the most common form of Ehlers-Danlos syndrome and recently, at the age of 36yrs I found out that I have it after a lifetime of unexplained symptoms. So, what does it mean to live with hEDS? Let’s take a look…

The Signs And Stretches Of hEDS

The hallmark feature of hEDS is joint hypermobility. This means that your joints naturally have a greater range of motion than usual. You might be able to bend your fingers way back, or your elbows hyperextend. But loose joints can also lead to instability, pain and frequent dislocations. My hEDS was discovered after my finger continuously started to dislocate in my mid-thirties, which is a symptom that I hadn’t experienced until now. I have been super flexible since my childhood, before ever training or stretching, decades before training to become a professional natural bodybuilder and fitness journalist, writing for and featuring in worldwide fitness magazines in my twenties.

hEDS can also affect other parts of your body. People with hEDS often have soft, velvety skin that bruises easily. Fatigue is another common symptom, along with digestive issues, dizziness, and problems with internal organs. My medical records are littered with episodes of me fainting without warning and having being dizzy spells.

I’ve suffered from IBS my entire life, which has gotten worse with age and have often collapsed from sheer exhaustion which was always excused as an acceptable and expected part of life from being a parent, dealing with stress and responsibility or leading a plant-based diet and therefore needing more iron as opposed to finding and treating the root cause of my symptoms.

Seeking Answers, And A Diagnosis From Your Doctor

Doctors diagnose hEDS based on your medical history, a physical exam, and sometimes your family history. After years of unexplained symptoms, it was in fact a stranger on the internet who commented on my tik tok, after noticing that my arms are hypermobile, and asked me if I had hEDS to which I had to google it as I had no idea what it was. A few weeks later my doctor diagnosed me and everything within my life made so much sense.

Your doctor should ask you about your symptoms, when they started, and how they affect your daily life. They’ll also want to know about any past injuries or surgeries that you’ve had, and for me, whilst my symptoms were inconvenient and uncomfortable growing up, they’ve now got to the point of pain, dislocations and premature labour with both of my children.

The doctor performs a physical examination to check your joints for hypermobility, which means they must be able to move beyond the normal range of motion. They’ll also look for other signs of hEDS, such as very stretchy skin or easy bruising. I had a black finger at my appointment because of my knuckles dislocating. It’s worth nothing that if you have a close relative with hEDS, it’s more likely that you’ll have it too.

The diagnosis of Hypermobile Ehlers-Danlos Syndrome (hEDS) is based on two main criteria:

Criterion 1: Generalised Joint Hypermobility: This assesses your joint flexibility. There are age and sex-specific scoring systems using the Beighton score, which involves various tests of joint mobility. You’ll need to meet a certain score based on your age and gender.

Criterion 2: Two or More Features from Category A or Positive Family History. Category A includes features related to skin, tissue, and certain physical characteristics. You need at least two of these, though there are many more listed. I shall mark those that I have with a tick for reference:

• Unusually soft or velvety skin – ☑️ My skin has always been very smooth and silky
• Mild skin hyperextensibility (increased stretchiness) ☑️ The skin on my torso is incredibly stretchy even before having my two children
• Unexplained stretch marks (striae) ☑️ I have had stretch marks to my stomach, hips and thighs from a young age
• Easy bruising with minimal trauma ☑️ The slightest bumps or knocks make me change colour
• Recurrent or multiple hernias
• Specific types of scarring ☑️ I have raised scarring all over me from small and large traumas
• Organ prolapse
• Certain facial features and dental crowding ☑️ I had two upper teeth removed due to over crowding
• Arm span to height ratio exceeding a specific value
• Mitral valve prolapse (diagnosed by echocardiogram)
• Aortic root dilatation (diagnosed by echocardiogram)

Following my diagnosis, my doctor listed to my heart with a stethoscope and then booked me in for a blood test, ECG and echocardiogram to determine if my condition is vascular, as this can in some cases limit life expectancy. The ECG involved returning to my doctors surgery to have a follow up appointment in which a series of sticky pads were placed around my heart and chest and then wired to a machine. I laid on the doctors bed for a few minutes as it monitored my heart, before my doctor translated the results for me for peace of mind before leaving my appointment. I was told that my heart is in good health and am presently awaiting my echocardiogram at the hospital. My blood results have also returned as overall good health, with my vitamin D being a little low and my kidney function lowering slightly since my previous test, however this may not be related to my EDS.

It’s important to note that your doctor will use their judgement and consider all your symptoms to make a formal diagnosis. This information is not a substitute for professional medical evaluation but can be a useful guide to understand if you may need a consultation.

Self-Testing For hEDS With The Beighton Score

The Beighton score is a nine-point system which is used to assess generalised joint hypermobility. The joints assessed are the knuckles of both left and right hand little fingers, the base of both thumbs, both elbows, both knees and the spine.

• One point is scored for each joint that meets the following criteria:
  • Little fingers: Can be hyperextended (bent backwards) beyond 90 degrees with the forearm resting flat on a surface.
  • Thumbs: Can be passively bent back to touch the forearm.
  • Elbows: Can be hyperextended beyond a straight position.
  • Knees: Can be hyperextended beyond a straight position.
  • Spine: While standing with palms flat on the floor in front of you, you can touch your palms completely flat without bending your knees.

A higher score indicates greater joint hypermobility. There’s no universally agreed upon cut-off for a positive Beighton score. However, a common reference is that 4 or more out of 9 points suggests generalised joint hypermobility. It’s important to remember the Beighton score is just one piece of the puzzle. Doctors consider your age, sex and other factors when interpreting the score in relation to receiving a hEDS diagnosis.

Living With Loose: Challenges And Care

hEDS can be a frustrating and unpredictable condition. There’s no cure, but there are ways to manage the symptoms and improve your quality of life. Physical therapy can help to strengthen muscles and improve joint stability. Pain management is also important, and may involve medication, heat/ice therapy and relaxation techniques.

For many with hEDS, pacing and modifying activities are key. Learning to listen to your body and avoid overexertion can help to prevent injuries and flare ups.

Finding Your Zebra Tribe: The hEDS Community

hEDS is a rare condition that can sometimes feel isolating. But there’s a vibrant online community of people with hEDS who share experiences, offer support, and swap tips. You can also connect with specialist doctors and organisations for the latest information and resources online.

Living life with hEDS can certainly be a challenge, but with the right support and management strategies, you can still live a full and active life.

So Get Your Zebra Stripes On! Join The Ehlers-Danlos UK Dazzle Walk

The Ehlers-Danlos Support UK‘s Dazzle Walk has arrived for May 2024, and it’s a fantastic way to raise awareness for Ehlers-Danlos Syndrome (EDS) while also getting some exercise and having fun.

The Dazzle Walk is a fundraising event which is open to everyone. Whether you can walk 5 km or 50,000 steps, there’s always a way to participate because it’s all about getting involved, raising money for a great cause, and celebrating the zebra community. The zebra is the unofficial mascot of EDS as throughout medical students training, they are taught to think horse and not zebra – that is to say, what is the most likely cause of symptoms (the horse) rather than the rarer possibility (the zebra).

As EDS is a group of connective tissue disorders that can cause a variety of symptoms, including joint hypermobility, chronic pain, and fatigue. It’s often an invisible illness that people don’t realise or recognise, so the Dazzle Walk is a chance to raise awareness and show solidarity with those affected by EDS.

If you’d like to take part in this fantastic event then head over to the Ehlers-Danlos Support UK website where you’ll find all of the information that you need to register, set up a fundraising page, and download fun resources. You can then don your most dazzling zebra attire from black and white stripes to leggings and tutus, the more fun the better!

You can choose to walk anywhere that you like, for any distance that suits you.Whether it’s a lap around your local park with friends or a solo trek through the countryside, the choice is entirely yours. Why not get out in the beautiful sunshine with your family, friends and work colleagues to share your fundraising page and encourage them to donate or join you upon the walk.

The Dazzle Walk is a fantastic way to support the Ehlers-Danlos Support UK as the money raised goes towards funding vital research, providing support services for people with EDS and raising public awareness. It’s always a great day to take exercise, have fun, and make a real difference to the EDS community. I would like to send love and light to you all, a deep-hearted hug and supportive hand towards whatever challenge and obstacle comes our way in life.

Exercise & Movement For Adults With hEDS

Ehlers-Danlos Support UK is a hub of information for all things hEDS, and after reading through their extensive information I thought that it may be helpful here to summerise some of the key points when it comes to health, fitness and exercise. Whilst you can’t prevent yourself from having hEDS/HSD, you can still help to minimise the risk of developing developing further conditions such as diabetes, high blood pressure, high cholesterol, heart disease, obesity, osteoporosis, arthritis, depression and anxiety by taking safe and frequent exercise.

Challenges that a person faces with hEDS when it comes to exercise may include unstable joints, chronic pain, postural tachycardia syndrome (PoTS), limiting gastric symptoms and fatigue, yet taking exercise is still very important, offering you the opportunity to improve your functional ability rather than sitting down all day and seizing up.

Firstly, it’s important to ensure that you speak to your GP or medical professional to ensure that you are suitable to take exercise, which may also include speaking to a cardiologist, physiotherapist, gastroenterologist or rheumatologist. As everyone is individual, and hEDS/HSD affects everyone in different ways, it’s vital that you have an individual assessment for your ability rather than follow a one-size-fits-all programme, as through a professional assessment you can determine the most appropriate exercise regime for you to follow. A physiotherapist is also able to monitor and adapt the exercises that are most suitable for you accordingly.

When it comes to cardio, it’s best to start with low-intensity exercise for 5-10 minutes at a time, two to three times per week. Walking at your own pace is a wonderful way to keep active, get outside and enjoy the calming effects of nature. Start small and build up gradually if and when you are able to, as doing something is far better than doing nothing.

Focusing on strength exercises, such as lifting weights, enhances muscles, ligaments, tendons and bones, making them stronger to reduces the risk of injuries whilst supporting your joints. Starting off with bodyweight exercises, such as sitting on a chair and standing up, can gradually progress to using resistance bands and even dumbbells as you gain strength. However, it’s important that you listen to your body, start low and build up gradually, stopping as soon as you feel any pain, or if your daily pain should worsen.

The basic level mat-work exercises of pilates can be modified to be suitable for hEDS by building core strength, taking caution to avoid excessively difficult movements and making sure that the correct posture is maintained and targeted muscles are working – the instructor or physiotherapist can advise on what modifications may be best.

Despite being hypermobile, parts of the body can still get stiff if unused, which is a common complaint. This occurs because of poor posture and being static for too long, as many tend to sit, stand or lie in one position for extended periods of time throughout the day. It’s important to change position regularly, perform gentle stretches and mindful movements to address any stiffness that develops. Yoga can also be a useful tool providing you don’t over stretch or hyperextend your joints. You can find more detailed and in depth information about exercise and movement for adults with hEDS here.

I hope that if you are facing these symptoms, or know somebody affected by hEDS, that you may take comfort in knowing that you are not alone and there are answers and support available.

UPDATE: Media Coverage

I’ve had people from far and wide get in touch with me via social media to tell me that news of my diagnosis has spread across the UK media, and now overseas to America and Australia, as they send love and well wishes for my journey ahead which has been incredibly touching.

Just this morning I received a phone call from the hospital to inform me that my echocardiogram will take place this weekend at a nearby hospital – 30 days after receiving my EDS diagnosis from my GP. It should take around 30-40mins to complete and I’ll be off to celebrate my daughters grandmothers birthday with a trip to an antiques fair and afternoon tea immediately after my scan, which is a concept that really hits home the severity of this syndrome.

I must confess, I’ve had more than a few overwhelming thoughts of never seeing my children grow up, flourish and have their own lives and children, or ever meet my grandchildren. The birthday that I’m about to celebrate may be an age that I will never live to see myself. The moments that I spend and share with my loved ones now may be shorter and sweeter than I ever deemed possible at this age.

But on the flip side of that, I am truly thankful for finding out about my EDS now, rather than when it is too late, and as a result of this, having the time and potential to make the most of my life, whatever my scan results may be, for as long as I am blessed to have. They say that it takes a life changing event to truly put everything into perspective, and this has certainly been a moment of such magnitude. My heart aches for all who are touched by EDS, the pain, fear and uncertainty that it brings, but also the gratitude and purpose that it nurtures from deep within. It has been such a humbling sense of grounding that I didn’t expect to experience in my thirties.

Whilst the media may be limited in the wealth of information and advice that they can share about specific medical conditions and syndromes, and headlines and statements out of context may seem shocking at first glance, I’m determined to use my reach as an influencer to make a positive difference and raise awareness for the EDS community.

As a member of the Sintillate Talent influencer agency, I am honoured to have spoken with the company director and Senior Fundraising Manager of The Ehlers-Danlos Support UK charity alike, to be able to connect the two to collaborate on an EDS awareness campaign that I’ll personally be orchestrating along with ten fellow influencers. The more that we can speak about this syndrome, recognise the symptoms and take positive steps forward, the more lives we can change, improve and potentially save along the way.

If just one person achieves a diagnosis from raising awareness, imagine the time and resources that it can save the NHS to not look in the wrong direction when trying to understand the cause, improvement or prevention of chronic pain and symptoms related to EDS. This evidently frees up time and funding for other medical conditions, life saving surgeries and diagnostics.

For this reason I will be sharing all details about my echocardiogram appointment here and as much information and advice as I can, from my own experience and unique perspective, in the hope that it may help you or your loved ones to understand and find peace should you be walking the same path. Together we are stronger and can be the change that we wish to see; let’s talk more about our symptoms and health concerns rather than suffering in silence.

My Hospital Appointment

Today I went for my echocardiogram at the hospital which involved repeating my ECG -attaching sticky pads across my chest and ribs to wires- along with an ultrasound to my heart. To have the ultrasound, much like during pregnancy, a cold gel is applied to the skin and a hand piece moved across the left hand side of my chest, ribs and collarbone.

My appointment took around half an hour in total and I was asked to take off my clothes from the waist up and put on a front-opening gown, before laying on my left hand side on a medical bed with my back to the nurse who stretched an arm across me to take measurements as she worked on the computer screen behind me which I couldn’t see.

This scan allowed her to scan around my heart, clicking measurements and taking screenshots of my heart contracting and relaxing. She instructed me to take deep breaths at certain points, to inhale and exhale fast and slow, and at one point to hold my breath for a few seconds.

The test itself is comfortable, perhaps an unusual sensation to have a chest ultrasound when I’ve only ever had it on my stomach before, but it certainly wasn’t painful or uncomfortable. And with my back to the nurse and the lights turned down low it wasn’t embarrassing or awkward at all. To think that this is a common procedure and they see so many patients each day, there is no need to be nervous or embarrassed. If anything I felt so relaxed and cosy that I could have easily fallen asleep.

I asked the nurse if she was able to interpret the results whilst I was present, for peace of mind, but she advised that it takes around two weeks for all measurements to be reviewed and an outcome decided, as it’s not as easy as saying yes or no to “am I ok?”

However, she was able to reassure me that if she had noticed anything severe during the test then I wouldn’t be going home after my appointment and would be kept in hospital for monitoring; it was nice to know that anything concerning would be handled immediately. So to be told that I could return home and wait for my results from my doctor in two weeks was a relief in itself.

Whilst I haven’t exactly been given the all-clear, I also haven’t been flagged as needing urgent assistance, so that in itself is something to feel more comfortable about. In the meantime I’m focusing on my family, work deadlines and daily routine as a parent, trying not to dwell on what is outside of my control. Remaining calm, being accepting of what will be and appreciating the positives and blessings in my life is my aim right now.

After weeks of freakishly miserable weather in June, when I left the hospital after my appointment there was such beautiful a blue sky and bright sunshine, and a white feather fell at my feet despite no birds being around. I took this as a positive sign and instantly felt any tension or concern melt away. Life is such a miracle and each day is exactly what we make it. Regardless of what challenges and obstacles come my way, I will always meet them with an open mind and can-do attitude.

Raising Awareness For EDS

My heart is swollen for how proud I am of all of the influencers who took part in the awareness campaign that Sintillate Talent kindly allowed me to head in partnership with The Ehlers-Danlos Support UK Charity this June. I tasked the influencers to wear black and white, along with an EDS pin from the charity, and to share a caption detailing a key message.

The caption that the influencers used, along with attaching a charity information poster was: Today I am wearing black and white in support of EDS with @ehlersdanlosuk and @sintillatetalent 🖤🤍 

The zebra is the unofficial mascot of EDS as throughout medical students training, they are taught to think horse and not zebra – that is to say, what is the most likely cause of symptoms (the horse) rather than the rarer possibility (the zebra). 🦓 🐎 

The Ehlers-Danlos syndromes (EDS) are a group of connective tissue disorders that affect the whole body. There are 13 different types. Most are rare, with the most common type being hypermobile EDS (hEDS).

Common symptoms include joint hypermobility, and stretchy, fragile skin that breaks or bruises easily. Many also experience dislocations, widespread pain, and chronic fatigue. EDS can be a mostly invisible condition, but is multisystemic affecting many areas of the body at once.

If you want to find out more about EDS, The Ehlers-Danlos Support UK is a great place to start. They are a charity that works across the UK to support, advise and inform those living with EDS to improve quality of life. You can visit their website to find out more: ehlers-danlos.org

You can help to make life better for people with EDS by making a donation today. £15 could help the charity to answer a call from somebody who suspects they may have EDS and doesn’t know where to turn. That call could be life changing – giving someone the knowledge and confidence that they need to push for a diagnosis. Donate today at ehlers-danlos.org/donate #eds #heds #ehlersdanlossyndrome #hypermobility #hypermobile #edsawareness

I wanted this content created to slip seamlessly into the news feeds of everyday people, those with and without health conditions, to indicate how silently symptoms can affect our lives and how not every illness, disease or disability is instantly obvious to see. Sometimes people can be put off of viewing online content that is related to medical and health issues because it can be shocking, or look scary, painful or unsightly; wearing a specific type of fashion and explaining why you’re doing it is a really helpful tool to breech a subject without fear.

To see my social media feed awash with influencers wearing black and white, talking about and raising awareness for a condition that the majority of them had never heard of or ever knew existed, it filled me with such hope and positivity. For me this felt like planting seedlings, taking a gentle step forward to nurture discussion and raise awareness that may one day grow into something mighty in time. I hope that this network and audience may reach and inform people far and wide to encourage them to seek a diagnosis and address unanswered questions. Together we can be the change, stand up and be heard, ultimately changing and even saving lives.

I’ve had so many people reach out to me on social media after releasing my own content, telling me that themselves and children had unknowingly suffer from the same symptoms and are now seeking help from their doctor. One in particular that really touched me was a father telling me about his daughter, how nobody could ever explain or understand her leg pain, and how simple days out as a family left her in agony for days, but now suddenly it makes a little more sense to them and they can now take steps to better support her.

My heart goes out to all who are affected by EDS; please know that you are not alone and there are steps that can be taken to help. Even if you’ve only ever experienced silence, your voice matters and will be heard.

Life After Receiving My Test Results

After weeks upon weeks of nervously waiting, I received my test results via telephone during an appointment that I had made with my doctor, and had had to wait for a slot to become available. Despite having time to come to terms with the fact that I could hear good or bad news, it still left me feeling as though my future balanced on a knife edge. I tried to push it to the back of my mind, keep calm and carry on, but I found myself overwhelmed with emotion one day when I had my period. I’d felt so strong, confident and positive throughout that I would face whatever the outcome head-on, but with an influx of hormones from my cycle, I just couldn’t hold back the tears and overwhelming fear of the very real possibility of not being there for my children consumed me.

I cried myself to sleep, helplessly questioning “why me?” and the following morning I woke up with such a heavy head and heart for all of the “what if’s” that relentlessly flooded my mind. But afterwards, I felt cleansed. I was passing through the stages of grieving a future for my family that I may never have. But a part of me acknowledged that no matter how sad or uncomfortable I felt about this all, as sure as we are all born, eventually we must all pass away. We may never know how, when or why it will happen, or maybe it will be entirely evident to us, but we must all share in this experience which is impossible to escape. We can do so with love or fear, one outcome more welcoming than the other. Coming to terms with life ending is therefore an essential part of living in peace for as long as we are blessed to have left. I wasn’t expecting to mourn the thought of possibly dying so young, but in doing so, it gave me a sense of clarity and control over my day, my decisions and my priorities.

I felt sick waiting for the doctor to call me for my telephone appointment. I wanted to know my test results, but also wanted to have one final minute of not hearing bad news if that were to be the outcome. My mind split in two, with half of me wanting to know, but the other half desperately not wanting to know. A part of me was pumping with adrenaline ready to face the reality of my results, whilst the other part was a scared child. I felt everything and nothing somehow all at once. And I think that’s what’s so important to understand about this process, no matter how strong, calm and confident somebody is, there will always be a moment, a sensation or feeling that restarts the grieving process of living with an illness, disease or disability. It’s a constant journey of uphills struggles and restful tranquility, before the terrain changes again. You’re not either ok, or not ok, you’re constantly adjusting to maintain your footing.

When the doctor told me my results were clear, and showed no sign of having vascular EDS, it felt like my entire body turned to jelly as a great sense of relief and gratitude passed through me. The comforting words echoed in my mind and loosened all tension, stress and fear in an instant. To have felt so anxious, concerned and upset for so long waiting, only to have it swept away in literally minutes was so incredibly grounding for me. And after the call I reflected upon the people, so many kind souls, who had previously, or are yet to receive the opposite results to my own.

It choked me to think of how many lives could change from a simple telephone call such as this. For any illnesses, accidents, injury and hereditary concerns to make life as we know it suddenly, and totally, unrecognisable. And it’s the gravity of this that woke up a deeper part of me, to become more aware of truly living whilst we are still alive, no matter the time. To have true quality, regardless of quantity. Whilst I’ve always felt gratitude and compassion for life, and have had health scares in the past, this hit me so much harder this time and shook me out of my daily routine of work, bills and taxes.

So, after receiving my all-clear, I went to Turkey to have a breast reduction surgery, downsizing my bust three cup sizes. It was something that I’d been thinking about for quite some time, and the daily discomfort of having such a large bust seemed so unnecessary to me now. To truly appreciate what I want out of life, how I can live most authentically and wholeheartedly as myself, without the need to please others. It was an epiphany that I needed, and am so thankful for.

Private healthcare in Turkey is incredible, in less than an hour upon my arrival I had three tests to ensure that I was safe and healthy for surgery. This was a blood test, ECG and radiology scan of my chest which all came back clear. I then spent a week recovering in the sunshine of Istanbul, feeding stray cats, sight seeing, soaking up the sunshine and growing stronger each day.

Upon my return to the UK I received my Access Card in the post, which I received due to my hEDS diagnosis and allows me to not have to stand still on the spot, and have access to a public toilet. I’ve fought a silent battle everyday of my life, without anybody knowing I live in daily pain and discomfort, and my battle will only ever get harder. From constant stomach cramps to the sensation of flames travelling up my legs and fainting if I stand still for too long. I may be a bodybuilder, but standing on a train home or queueing for shopping cripples me for days on end.

Whilst I have a disabled son, I do not park in disabled or parent and child spaces because he doesn’t look disabled and I don’t want to receive abuse from others who need it more. On public transport I avoid disabled seats or crowed areas, and instead I try to sit in the middle of train carriages to not be in the way of elderly passengers or those with children who usually sit closer to the doors. As a single parent and business owner, I’m Mummy, I’ve always been the financial provider, protector, spider catcher, chef, gardener, chauffeur, house keeper and endless supply of energy, love and cuddles for my two children. I can’t be broken, I can’t have a day off, I can’t not show up. But I’m in pain, everyday, and I can’t deny or hide it away anymore.

Instead of forcing myself to stand on the train out of pride, and then suffering for days on end from the pain, I must now take a seat besides a teenager, twenty-something on an i-pad or fellow commuter listening to music without feeling guilty or underserving of sitting in a normal seat let alone going anywhere near a disabled seat or area. I carry my access card with me, but have yet to find the courage to use it or show it to anyone, because I’m still coming to terms with the fact that I’m deserving of this right. Only weeks ago I was normal, now I have a diagnosis and so much makes sense, yet so much I can’t yet get my head around. This wasn’t a choice, or a gradual journey, it was a curve ball out of nowhere when I’d always been told to be quiet and not complain because nothing is wrong with you. But it is, and always was, I just suffered in silence after always been turned away by medical professionals and my questions left unanswered; until a stranger on Tik Tok unlocked this door to an entirely new world for me by noticing my hyper mobility and deciding to point it out. And here we are.

I know that my access card is there should I be in too much pain and need to sit down or visit a toilet at short notice. With it I may be able to ask for help or understanding from others around me who could deem my actions of sitting or using a toilet to be lazy or selfish because I know that my internal pain and suffering is not visible. A smile and healthy exterior can hide so much; wearing gym wear, running a business and tending to my two children and home doesn’t mean that I’m not in pain, I just have nobody else to do it for me. Everybody gets tired, everybody wants to sit down at the end of a long day, not have to queue in the airport or wait for access to a toilet when they’re desperate to go; as a hard working mother of two I understand the aches and pains of life, it’s not easy for anyone. But I have to be kinder to myself now, without feeling like a victim or weak I have to make a conscious effort not to cause unnecessary damage to myself anymore by trying to do what is beyond me in order to fit in and not offend anyone else or receive public backlash. I have to accept that my symptoms and suffering will only worsen with age, that what is invisible now may one day become incredibly obvious to others. And realise that I am as young and capable as I will ever be, right here and right now, and so I mustn’t waste a second of my life feeling sorry for myself, but instead love and support myself. And have these answers for my children who suffer the same, yet milder, symptoms to that of my own. I have to remove the prejudice and judgement now so that they don’t endure a lifetime of misunderstanding as I have.

My pain and suffering from hEDS will continue to worsen with age, but I can make the most of my life. I know that I can work towards processing, accepting, understanding and finding peace with finally having the answers to my questions that had been left unanswered for a lifetime. Whilst there is no cure for my pain, life goes on. These are the cards that I have been dealt in life and I can but make the best of it. With time I know that I will fully accept and better support my lifestyle with hEDS, adapting my way of life accordingly to minimise the struggles that I face. It’s been an awful lot for me to take in, in such a short space of time, and I’m still waiting for the ripples in the aftershock to fully pass before I can move forward and make progress.

But with time we all have understanding, experience, wisdom and hindsight. September is World Pain Awareness Month and it’s something that I had never heard of before. On Instagram @ehlersdanlosuk writes: “September is World Pain Month, an awareness month dedicated to highlighting the challenges faced by those living with chronic pain and the importance of better support and management. It also provides a platform for individuals living with chronic pain to share their stories and connect with others who face similar challenges.

Chronic pain in EDS and HSD is very common and may be severe. It may be widespread or it may be limited to one are of the body such as a limb. Headaches and gastrointestinal discomfort can occur as well as joint, muscle and nerve pain. Management strategies include physiotherapy, medication, and sensible use of medical equipment such as splints and braces.”

I hope that in wholeheartedly sharing my thoughts on this journey, the fears, helplessness, ignorance and obstacles can be a cathartic experience for others facing the same challenges because you are not alone. That in sharing the changing of my opinions and mindset, towards recognising weakness and the inability to no longer be able to do simple things that I once was able to without pain, it isn’t the end. The life and achievements that I have worked so hard to build isn’t over; I can still lift weights, run businesses and love my children wholeheartedly. But I’ll take better care of myself now, know my limits and adjust my lifestyle accordingly – no longer a one size fits all, but a tailored plan as an individual for everything that happens beneath the surface.

When you’ve spent a lifetime of having to be strong and survive, it’s incredibly scary and unsettling for life as you know it to change in an instant from a diagnosis, health scare, illness or accident. But having answers, building a plan and understanding the right path to follow and the best direction to head in is half the battle. The rest is just taking one step at a time, each day as it comes, and listening to your body and needs rather than pretending nothing is wrong. It’s ok not to be ok. We’re in this together.

2 Years After My hEDS Diagnosis

I cant believe that two years have passed already since receiving my hEDS diagnosis and in all honestly its been a bit of a rollercoaster to see how my body and abilities are changing with age.

I’ve returned to my doctor multiple times over the past two years with hand exhaustion which causes me to drop things and lose grip as my hands go numb and I lose sensation, mostly when holding cups and opening jars which causes dislocations. I also have extreme exhaustion in my fingers which feels like somebody has literally stretched them out as far as they will go and pinged them back again, or cracked them wide apart like a wish bone and they feel loose, wobbly, incredibly sore and I’m unable to find relief from tenderness whether I curl them up or lay them out straight.

Carpal Tunnel Syndrome

I was checked over and told that I have carpal tunnel syndrome, but they didn’t see my symptoms as significant pain and marked it as minor, even though it impacts my daily life so severely. To have exhausted, weak and fragile hands impacts every aspect of my day, from trying to get dressed to preparing food and taking care of myself. When my hands are broken and unresponsive I’m absolutely incapable, and when they have sensation and are simply sore, I push through the discomfort to use them whilst I can which only causes them to feel worse.

The doctor gave me stretching exercises to help with my carpal tunnel syndrome, but ultimately the only cure is surgery which they didn’t recommend. Just like the rest of my body, my fingers and hands are weak and unsupported, so using them for sustained activities or pressing on my fingers when wiping or touching something makes them feel like they’re going to bend too far backwards and snap off. I wish I could wesr metal gloves!

Something that I find fascinating is that I still have a grip strength to hold my bodyweight in the gym with a hammer/parallel/neutral grip, where my palms face inwards like holding onto ski poles. But when I have a pronated grip with my palms facing away from me for a pull up, and my fingers taking the stress as opposed to my thumbs, I’m super weak and can’t even lift a fraction of the weight of my neutral grip. Again it feels like my fingers will snap off. Using all of my fingers at once to hold a shopping bag is a million times easier than the tenderness of typing on a keyboard or playing the piano, where fingers perform an action individually as opposed to all at once.

I’m conscious of the fact that I have to still train my muscles in order to maintain endurance to not become deconditioned and even more fragile than I alreadfy am, but equally have never been given professional support or understanding for what exercises I should do. And from being a professional bodybuilder, spending over a decade training to be strong and able-bodied, I now focus on light weights and low-impact exercise to support my stability, going from Crossfit ability to gentle cycling for my ankles, knees and hips, along with seated calf raises and cable assisted curls as opposed to free weights. Oh how the tables have turned!

Energy Collapse Without Warning & Being Left Bedridden

I’ve also noticed over the past two years that the frequency of me collapsing is accelerating now, as my body seems to just shut down and my energy disappears at the flick of a switch, like being sick and having the flu as opposed to a common cold. I’ve always visited my parents at the weekend to catch up, have dinner and for them to spend time with the children. More often than I’d like, I’ve sat down on the sofa with a cup of green tea and instantly fallen asleep upright in a knockout, deep sleep and can’t be woken until home time. I’d never even had an afternoon nap before, let alone nodded off during daylight hours, so this is really out of character for me.

I’ve always struggled with sleep, and have insomnia which is likely down to my ADHD, as I struggle to switch my brain off to fall asleep, and am hyper sensitive to light, sound, temperature and pain so wake frequently throughout the night. My arms, legs and hands also tingle, turn numb, ache and make me toss and turn from side to side to find relief from discomfort. And I usually wake up feeling more tired in the morning than before I went to sleep. Needing the toilet, having to put pillows between my ankles and knees and being tortured by trapped nerves in my shoulders and elbows are a constant struggle, my body just can’t relax or shut off until it’s too late and shuts me down and I’m bedridden and sleep for 11hrs straight.

So to get to the point of collapse, where my body has had enough and just turns me off, leaving me dead to the world, is a newer edition to my long list of chronic symptoms that I returned to my doctor to seek help for. I’ve fainted more times than I can count, lost my eyesight and hearing and needed to run to the toilet in an emergency as my bladder and bowels give me no warning. But in my mind, being able to achieve a proper nights sleep will allow my body to recover from the chronic inflammation of my hEDS, and hopefully not get to the point of involuntary collapse where I am bed bound and unable to walk, think straight or take care of myself or children.

My private stress incontinence TOT surgery that I had one year ago now has made a huge difference to the amount of times that I go to the toilet daily, and relieved some of the chronic pain and cramping from my stomach area and involuntary leakage of urine. But this is still something that is a weakened system in my body, as with my bowels, heart and lungs.

Sleep Disorder & Self Medication

My teenage son also had autism, ADHD and sleep disorder for which he is medicated with melatonin daily and is the only way for him to sleep. Despite avoiding medication myself, I saw this as my final option to try to help my body after exhausting all sleep hygiene routines from cool air and gel pillows, eye masks, blackout curtains, ear plugs, lavender pillows, chamomile tea, sleeping on a grounding sheet and taking a bath before bed to unwind. I’m incredibly strict with my bed time, ensuring that I go to bed and get up at the exact same time everyday to support my circadian rhythm, but still I’m far too alert, energetic and in constant pain to sleep.

My doctor explained that the ADHD clinic can administer melatonin to assist with sleep so suggested I call them, for which they told me I was discharged back to my GP after diagnosis and they don’t prescribe melatonin. My GP then sent me a link to a sleep clinic, who told me they don’t provide melatonin prescriptions for which I informed my GP and was then advised to “buy it online”.

The only melatonin that I could buy online was 3mg for jetlag, which cannot be taken for more than 5 days in a row. My teenage son is prescribed 2mg of melatonin daily to sleep and is monitored for his age, height and bodyweight to ensure the correct dosage. I’d never taken melatonin before, and had no guidance from my doctor as to how much to take, but the 3mg knocked me out and has allowed me to be more proactive with my sleep, to self-medicate I suppose, whenever I feel that I am reaching burnout and need to leap before I am pushed.

I have no idea how often I can take 3mg melatonin for non-jetlag, and so I’ve tried to set a personal limit of once per week if necessary. Something as simple as standing in a supermarket queue can send my legs into meltdown mode and engulf me in burning pain which takes me days to recover from, yet I’m able to walk without an obvious problem as my knees are bent and not hyperextended or continuously weight-bearing.

Choking After Running A Short Distance

I finally made the connection between choking and exercise when travelling home on the train last week, as I arrived at the station to the announcement of my train departing in just one minute, with the next one not due for an hour. So I ran perhaps 20 metres to the end of the platform to get to the carriage on time and was thankfully seated seconds before the doors closed and the train pulled away. Only I then have a coughing fit and was unable to breathe properly or stop choking for the 40mins duration of my journey.

This has happened to me several times in my life, always when running for trains and then sitting down and feeling fine, but then moments later it triggers and I can’t hold in the involuntary coughing and choking as passengers look at me in disgust like I’m sick and spreading germs but I’m not, I’m suffocating and turning bright red with my eyes streaming because I can’t breathe properly or speak. I always thought that I’d swallowed saliva into my lungs, which I believed everyone does by mistake sometimes, but it’s actually something else.

Running for a train and then immediately sitting down is known to trigger choking and breathing difficulties in those with hypermobile Ehlers-Danlos syndrome (hEDS) and dysautonomia which is often mistaken for asthma. The combination of hEDS and sudden postural changes leads to this occurrence in three ways:

• Inducible Laryngeal Obstruction (ILO): The vocal cords or soft tissues of the larynx collapse or spasm when breathing heavily after exertion, restricting the airway.
• Dysautonomia and Blood Pressure: hEDS is frequently accompanied by autonomic dysfunction like PoTS. Moving rapidly from exertion to a seated position can cause sudden blood pressure shifts, which trigger breathlessness and a choking reflex.
• Swallowing or Reflux Issues (Dysphagia): hEDS can cause slow esophageal motility or subluxation of the throat joints, making it difficult to clear saliva or liquids from the throat immediately after heavy breathing.

If I train at the gym and cycle, I can typically manage 30mins of steady cardio as I’m dripping in sweat and hitting a wall for energy, yet power walking/lightly running 20 metres to a train on the platform leaves me gasping for air as I feel like I’m drowning. I now know to not sit down directly after getting on the train, but to keep my body moving for a few minutes and perhaps walk along the carriages to find a further seat in order to allow my blood pressure and lungs to return to normal before sitting down and stopping.

As I still experience a seatbelt sensation pulling tight across my chest, and hear my heartbeat in my ear before losing hearing, I am scheduled for an ECG along with my blood test, which I’ve waited for so long to get an appointment for.

Emotional Overwhelm

I cried at my GP appointment because I felt so overwhelmed and confused by my body shutting down, my hands hurting for doing the most normal and smallest of things throughout my day, and the increase in how quickly my symptoms have accelerated these past 48months. One day I feel like I’m 21yrs old and my body is strong and capable, and the next I may as well be 80yrs old, can hardly lift my head and even standing to pour myself a glass of water makes my ankles feel like they’re going to burst through my skin and hit the floor. There is no rhyme or reason to it, my body switches ability in the blink of an eye and I’m helpless to prevent or understand it, but I’m trying.

Unfortunately my GP isn’t a specialist in hypermobility, doesn’t connect the dots of my symptoms and has shockingly failed to even list my hEDS as a health condition despite it absolutely dominating my life. I have begged to be referred to a hypermobility specialist, but it seems that I must first jump through hoops to have assessment for POTS and MACS and join the endless wait list of the NHS in order to even speak to a member of staff who understands, let alone can help me.

I’ve therefore been doing some research into hypermobility online to try to tackle and improve my symptoms, and have spoken with some charities and organisations who provide mobility aids and support such as The Zebra Club and Disability Horizons who very kindly invited me to share my experience of a late life diagnosis and coping strategies.

Two years down the line I am still entirely lost and hopeless for how to manage my symptoms, or how to be heard, but I recognise that time is now of the essence and my decline may be faster the older I become. Some ladies have suggested that the hormone fluctuations of perimenopause may be playing a part at the age of 38yrs, whilst others have suggested that overlapping conditions of POTS and MACS.

Testing For POTS & MACS

POTS is a form of dysautonomia, which is a malfunction of the Autonomic Nervous System, the system that controls every automated process in the body like heart rate, blood pressure, digestion and temperature regulation. My heart beats out of my chest for no reason and I have panic attacks, my blood pressure is consistently 60bpm and has caused trouble when trying to take blood, and my body is either freezing cold or burning hot and I have to pull my clothes off as others sit comfortably.

Core Symptoms of POTS:

• A heart rate spike of 30+ beats per minute within 10 minutes of standing up
• Dizziness, lightheadedness, or feeling like you might pass out when standing
• Brain fog, trouble concentrating, forgetfulness and word-finding difficulties, frequent migraines
• Cold, numb, or purple/mottled legs and feet when standing, sudden internal temperature swings or excessive sweating
• Profound, bone-deep chronic fatigue that is not relieved by sleep, severe exercise intolerance where even minor exertion feels like a sprint

I have all of these symptoms and have therefore requested to be tested by my GP for which a blood test and ECG are the first port of call.

MCAS, or Mast Cell Activation Syndrome is an immune system disorder where mast cells are hyper-reactive and unstable. The body mistakes normal, everyday things like a temperature change, vibration, emotional stress, friction, or specific foods as dangerous attacks, inappropriately flooding the body with an overabundance of inflammatory chemicals, triggering a cascade of allergic-like symptoms across multiple organ systems at the same time.

Core Symptoms of MCAS

• Sudden flushing, turning bright red and feeling hot, unexplained hives, itchy rashes, or dermographism where scratching the skin leaves raised, red welts
• Severe abdominal cramping, bloating, nausea, vomiting, or sudden bouts of urgent diarrhea. Many people develop extensive food intolerances
• Throat tightness, a chronic dry cough, wheezing, shortness of breath, or a post-nasal drip which can escalate to a full choking sensation or closing of the throat
• Repeated, unprovoked episodes of severe allergic reactions that can include a dramatic drop in blood pressure and passing out, requiring emergency care

I have all of the MCAS symptoms apart from severe allergic reactions, as I pass out without warning, but have never been given emergency care.

Dietary Changes

I’ve had a dairy and gluten intolerance for many years now, which I became aware of after having private testing, but I have also become highly sensitive to everyday foods and meals at my parents, for which I have to cook unprocessed, fresh vegan food from home for every meal. I can no longer have garlic or onions which I’ve always loved, let alone the odd treat of crisps, chips or anything fried because it leaves me in agony from just one bite.

Vegan alternatives such as breaded sweetcorn burgers, pastries, cakes, cookies or ice cream that I used to enjoy on a cheat day now feel like swords in my stomach, and I no longer use stock cubes, sauces or seasonings on my food.

Sadly, the list of foods that I can now eat has gotten smaller and smaller, and I never go to restaurants or food stalls for lunch or a snack anymore, as it’s not worth me being in pain and discomfort for days on end, even when I eat from a vegan-friendly, gluten-free menu.

I’ve had to cook homemade soups daily in order to give my bowels relief from digestion pain, and all and any processed foods, or anything out of my regular diet leaves me feeling like I’ve been hit by a bus. My stomach goes from being a trim six pack to looking like I’m eight months pregnant within minutes or eating something, the flatulence is shocking and the cramps leave me wincing and doubled over in pain.

I therefore now take meal prep with me wherever I go, and plan my day around having access to homemade food and snacks that my body can tolerate, which is certainly one way to kill the vibe and offend people when invited to lunch or for coffee with work colleagues or relatives. I used to be able to keep calm and carry on, pretending to be comfortable eating from a vegan, gluten-free menu, having to pop open a top button on my trousers or discreetly go to the toilet to pass wind or empty my bowels after having a nibble of bread or a risotto.

But what was once discreet bloating is now emergency labour, and it’s a risk that I can no longer take or manage in public anymore. Oh what I’d give just to have one day of being able to eat like everybody else!

My Specialist Referral

Two years after my initial diagnosis, my GP has now booked me an appointment with the First Contact Physio this weekend based in the doctors surgery, for which I have no further information or understanding of what they’re able to address, nor the specific services they provide, but believe that this is the first step in seeking a specialists support for hypermobility and the total body symptoms that I face.

I’ve also begun at home lung training to help with my air hunger and shortness of breath, using a 3 ball incentive spirometer and lung trainer that I purchased affordably from Amazon. These devices have shown me the areas of weakness in my lungs that I hope to be able to improve on through at home lung training. I hope to eventually be able to raise the three balls in a slow and controlled inhale for a longer duration, as well as eventually lift the blue ball during exhalation – something that most people are capable of doing but is presently impossible for me no matter how hard I try.

It seems a lot of symptom management must come from social media and seeing posts and recommendations from other patients online who suffer from hEDS, as medical staff have very little to no knowledge or understanding of Ehlers Danlos and fail to spot, let alone correlate lifelong symptoms or know what medical course of action to take.

I’m really hoping that the physio will understand the way that my body works and be the first time that I feel heard or understood, but a part of me is also not expecting miracles and hardwired to be told that I’m just stressed or tired again, and dismissed.

My Appointment With The Physio

I felt more than a little deflated after my appointment with the physio today, which left me crying on my friend moments later when they innocently asked me “how are you?” I just couldn’t contain my overwhelm, despite trying to put on a brave face, keep calm and carry on as normal, as days like these make me feel so incredibly helpless and misunderstood.

I do not know the history of my physio, whether they had many years of experience in their field or just a few but they were very polite, professional and older than me as opposed to being a student. Unfortunately they didn’t understand or have any help or advice for me, and had never heard of Ehlers Danlos Syndrome before.

I made sure to prepare a list of helpful information to take to my appointment with me, accessible on my phone, which I handed to the physio to read during my 20mins appointment when they said they didn’t know what hEDS is.

The details that I gave to the physio are here for reference:

Physiotherapy Assessment Notes – Hypermobile Ehlers-Danlos Syndrome (hEDS)

Reason for Referral: Assessment for generalised joint hypermobility and musculoskeletal features consistent with hypermobile Ehlers-Danlos syndrome (hEDS), together with associated conditions including suspected POTS and Mast Cell Activation Syndrome (MCAS).

Joint Hypermobility
Please assess:

• Beighton Score (0–9)
• Generalised joint hypermobility
• Joint instability
• Range of motion
• Evidence of subluxations or dislocations
• Joint laxity

My symptoms

• Extremely flexible joints since childhood
• Joints frequently move beyond the normal range
• Joints feel unstable
• Joints sometimes “give way”
• Frequent clicking and popping in multiple joints
• Pain after standing or exercise
• Difficulty maintaining joint stability
• Chronic widespread joint pain

Pain

• Chronic pain affecting multiple joints
• Joint inflammation
• Muscle aches
• Pain worsens after activity
• Fatigue following exercise
• Recovery from activity takes longer than expected

Fatigue

• Persistent exhaustion
• Episodes where I am largely bed-bound because of fatigue
• Reduced exercise tolerance
• Activities require much longer recovery than expected

Cardiovascular Symptoms (possible POTS)
Please note these symptoms for my GP/cardiology referral:

• Feeling faint when standing
• Episodes of collapse
• Dizziness when upright
• Light-headedness
• Shortness of breath
• Air hunger
• Exercise intolerance
• Low blood pressure (typically around 115/58)
• Symptoms improve when resting

Breathing

• Frequent sensation of not getting enough air
• Air hunger
• Using an incentive spirometer to help breathing exercises
• Breathlessness that seems disproportionate to exertion

Neurological Symptoms

• Numbness in hands
• Numbness in feet
• Weakness after activity

Gastrointestinal

• IBS symptoms
• Digestive problems
• Previous colonoscopy was unable to be completed because my bowel was described as very twisted

Skin & Soft Tissue
Please assess whether skin findings are suggestive of connective tissue disorder:

• Skin softness
• Skin stretchiness
• Easy bruising
• Delayed healing
• Abnormal scarring

Pelvic Floor
History of stress urinary incontinence requiring surgery.

Other Diagnoses
Current diagnoses include:

• Autism
• ADHD
• IBS
• Rosacea

Possible Mast Cell Activation Syndrome (MCAS)
Symptoms I would like considered alongside hEDS:

• Episodes of flushing
• Allergic-type reactions
• Food sensitivities
• Gastrointestinal symptoms
• Skin reactions

Functional Impact
My symptoms affect:

• Walking
• Standing for prolonged periods
• Exercise
• Household activities
• Sleep
• Daily quality of life

Questions I’d appreciate being documented

• My Beighton score
• Whether I have generalised joint hypermobility
• Whether my symptoms are consistent with hEDS
• Whether physiotherapy findings support referral for formal hEDS assessment
• Any evidence of joint instability
• Whether my symptoms could explain chronic pain and fatigue
• Appropriate strengthening and joint protection advice

The appointment primarily involved the physio looking at this list and not knowing what it was or what to do with it. He asked me to stand up and touch my toes, rise up onto my toes, twist at the hips to the left and right, lean from side to side and hyperextend my legs. I also volunteered the demonstration of my shoulders full range of motion, the flexibility of my hands, wrists, elbows and fingers, pointing out my dislocations and fractures.

Following this, he asked me to press towards, and pull away from his hands with my hands and knees. He squeezed my arms and it specifically hurt my elbow, but he didn’t offer any explanation for this. The physical examination took no more than 5mins in the appointment. He suggested referring me back to my GP as he didn’t know what to do and gave me no advice or guidance for my symptoms or condition.

I told him that I perform low impact exercise at the gym through cycling to try to stabilise my ankles, knees and hips, along with assisted cable machines for my upper body using low weights for proprioception. He made no comment about this and gave me no advice on what exercises I should add, modify or remove. I requested not to be returned to my GP, as they didn’t know what to do either, but to be referred onwards to a hypermobility specialist instead.

He advised that he had made a note and put in a request, but it may take four months to hear back from anyone and it can be automatically declined if whoever receives it feels that it’s unnecessary or not applicable to them.

The RCCX Theory

Whilst looking for help and support myself, after I returned home, I came across the RCCX Theory which I had never previously heard of. It is a medical hypothesis proposing that mutations in a complex, repetitive cluster of genes, known as the RCCX module, explain why certain conditions frequently occur together.

Theorised by psychiatrist and internist Dr. Sharon Meglathery, it links joint hypermobility (EDS), chronic fatigue, autoimmune diseases, and neuro-psychiatric conditions (autism / PTSD).

The genes in this cluster (TNXB, CYP21A2, and C4) heavily influence collagen, stress hormones and the immune system, suggesting that having a mutated variation can cause an individual to experience chronic illness, making them highly vulnerable to severe stressors. 

You can test for the individual genetic components and the resulting downstream biological effects:

• CYP21A2 Gene Testing: You can be tested for mutations on this gene, which affects the 21-hydroxylase enzyme and can impact cortisol production. 
• TNXB Gene Testing: Testing for the Tenascin-X protein is available to check for genetic predispositions to hypermobile Ehlers-Danlos syndrome. 
• Complement C4 Testing: You can test for C4 levels or C4 copy number variations. 

Standard clinical genetic sequencing often misses these mutations and so specialised clinical panels are sometimes required. 

I then logged a note to my GP in an online form, rather than booking a face to face appointment, providing the RCCX Theory and a link to The Ehlers Danlos Society Programs For Healthcare Professionals.

As it is now the summer holidays for the next six weeks, I am juggling childcare, working and and taking care of the home, children and pets as a single parent, trying to maintain a fine balance between constantly burning the candle at both ends and avoiding imminent collapse. I hope that answers and support comes soon, rather than later. And as soon as I have a clear route to diagnosis and support, my daughter who has the exact same symptoms as I, and father who shows symptoms of Ehlers Danlos (minus hypermobility in old age) can hopefully fast track this process and go directly to the source for diagnosis.

Meeting With My GP & Further Testing

I had three appointments at my doctors clinic this week, which I received a text request to book in for. The first was a blood pressure check, the next was a face to face GP appointment and the third was a blood test for my sugar levels.

My blood pressure was “normal” but lower than average as usual for me, I’ll have my blood sugar level results within a week, with all others coming back as “normal” and my GP asked me to confirm what it feels like to live with my symptoms.

I described the exhaustion as feeling like I’ve been hit by a bus, as everything feels weak and tender without warning. Whether I have an active day or a fragile day, my body endlessly aches, like having the flu, it’s to my very core. And no matter how much I rest or try to recover, I can’t stop my body from shutting down without warning, over the smallest of things that should never be an issue in everyday life.

We spoke about pain and I told my doctor it’s not possible to measure it on a scale from 1-10 as that would be an injury, or something isolated, but I have chronic pain. Pain is normal to me, pain never ends. It feels as though I have been stretched to my limits like a rubber band and then let go and pinged back, stretched out, loose and floppy.

I’m sore, my joints are hot and buzzing, and I feel exhausted each and every day no matter what I do. Some days I can’t get out of bed, some days i can’t be after after lunchtime, and others I can push through the pain and try to lead a normal life like everybody else, but then I am bed ridden and pay the price. It’s a daily balance of rest and movement. Rest to reduce inflammation and stop from yawning and curling up into a ball feeling broken, and movement to bring my numb, tingling and cold limbs back to life again.

I showed her my 80+ tattoos, and how I don’t take pain relief, how I don’t even flinch, because constant pain is a part of my life and my pain tolerance is so high because of it. But it doesn’t mean it doesn’t hurt me, and just because I smile and put on a brave face to not make others feel awkward or upset, doesn’t mean it cures me or I’m feeling good. I’ve just learned to hide my suffeering and not complain.

I updated her on the progress that I’ve made with buying melatonin online, in 3mg doses which is for jet lag only as it’s highly addictive. And since buying it from an online pharmacy, as my doctor cannot prescribe sleep medication and cannot refer me to any local services, self-medicating has allowed me to thankfully avoid collapse.

I take a melatonin tablet before bed up to twice per week, primarily on days when I know that my body is struggling. It could be after having a poor sleep due to the summer heat, before a day when I know that I have to leave the house and need energy, or after doing something physical with the children like visiting grandparents for a few hours or going for a walk.

Whilst I’m able to hold my posture around people, be engaged, attentive and enthusiastic, I return home, flop down, put my legs in the air to relieve them and melt physically and mentally. It’s exhausting! It’s as if my bones have turned to water and can’t hold me up, my neck feels broken, my legs want to snap off and my hands are so exhausted you’d think I’d just played the guitar for 3hrs straight. When i’ve had a cup of tea in the garden and used two hands to lift a teacup so as not to dislocate my fingers from holding the handle like everybody else.

My GP advised she would now refer me for gene testing which takes place at a nearby hospital. whether this is the same referral as the physio, or something different I do not know, but I suspect that the physio referred me back to my GP rather than onward to a specialist, hence why I received a text message to make an appointment with her.

Today felt like the very first time that I was truly listened to and understood. It didn’t feel as though I was speaking a foreign language or saying something outrageous and unbelievable. I didn’t have to explain what hEDS is, or how to spell it. It was a repeat appointment for an ongoing health condition that affects my entire family and future generations of our bloodline perhaps. And I feel as though all of the information, websites and details I had provided to my GP had maybe been read and understood?

Again, I do not know how long this hospital referral will take, whether it’s part of, or in place of the physio referral, or if two different appointments check for two different things. The issue being that nobody has understood or taken overall responsibility for my health in order to see each process through, as it’s been a case of being passed from pillar to post and back again with seemingly no communication between each professional, just a dead end until today.

Upon reading my NHS notes on the online app, I was very shocked to see that my chronic health conditions were only ever listed as “minor” “past” and closed immediately after each appointment that I attended asking for help. I made sure that my GP understood today that none of my symptoms have improved or removed from my daily life, they have only worsened with time and become more extreme with additional symptoms occurring.

I also learned that my John Hopkins adjusted group clinical score was increased from a 3-4 at the start of this year. I’m not sure of what was the cause of this, and had never heard of this score before so I had to google it. Upon searching for what my score should be with my symptoms, i was surprised to see that I should be around an 8-11 in the moderate to high need group, rather than a 4.

I suppose when they have a specialist assess me, and are able to provide a formal diagnosis, this score can be adjusted accordingly, as my medical records have left my history as a vision of health, when my reality has been begging for answers as I deteriorate and suffer in silence for a lifetime with every question asked left answered. If only my body matched my paperwork!!

I hope that you will come back soon to see my updates and how I’m getting on.

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