Hi all, I'm Claire Saul, married to Duncan, 3 children aged 22 (boy),19 (boy), 16 (girl)
I have Ehlers Danlos Syndrome with POTS (postural orthostatic tachycardia syndrome) chronic pain & nerve root damage, migraine, gut motility problems, Raynauds
The EDS is a genetic condition of the Connective tissue – my body produces faulty collagen – so it can affect any part of the body that contains connective tissue. Normal connective tissue is like elastic, EDS connective tissue is more like chewing gum. I have regular dislocations, particularly my hips and shoulders – I started dislocating aged about 8.
POTS is condition that affects the circulation on changes in movement, temperature. When I stand up my blood pressure drops, meaning my heart has to beat quicker and harder to get blood round the body – but the circulation to the brain is reduced, causing me to pass out! This can happen when I stand up, get out of the shower or when the weather is hot/sunny. My hands and feet turn purple as blood pools in them.
Before I was diagnosed I loved my job as a palliative care nurse, I loved socialising & hosting dinner parties – dancing and going to yoga classes! I am not able to work – I was a palliative care nurse, but due to the chronic back and leg pain was medically retired due to disability.
EDS is genetic so I had signs from an early age – “knocked knees”, flat feet, dislocations, poor coordination, pain, headaches (prob early migraines) all before age 10, then during my teens dislocations increased, def migraines, fatigue, circulation probs (now realise POTS), Raynauds and “growing pains” IBS. Aged 21 a disc ruptured in my back and went into cauda equina (foot drop, severe pain, bowels and bladder not functioning) – had first back surgery. Chronic back problems – started here with nerve root damage
My shoulder pain was increasing and preventing me from doing some normal activities, so my GP referred me to the Hypermobility clinic at UCLH (Prof Graham’s clinic). I was lucky that then a secondary referral from a GP was accepted – it has changed now and the patient has to be referred from another hospital consultant (tertiary).
The actual diagnosis was easy as I fulfilled so many criteria and my mum had gone with me so was able to give family history. Although she isn’t formerly diagnosed, we can see that both she and my grandma when she was alive have symptoms. I was given a diagnosis very quickly, but no support or follow up – was told that my management needed to be local.
But the time it took to join the dots of various illnesses was not easy. I was finally diagnosed aged 42
My sons haven’t been formerly diagnosed but both have symptoms, but my 15 year old daughter has been diagnosed by a geneticist and it was relatively easy – I think because I knew who to ask to be referred to and having a good knowledge of the criteria myself.
I searched the internet – once diagnosed, and with increasing symptoms and pain, I set out to find out as much as I could. I flare on my back and neck, leg, feet, hands & wrists, shoulder and hip joints.
My chronic nerve pain in my back and leg increases in intensity and my spinal cord stimulator no longer masks it; the pain in my soft tissue and joints increases and this usually coincides with an increase in dysautonomic symptoms – nausea, fainting, dizzy spells, sweating, hot then cold – and fatigue. Offten unable to lift my head without dizziness.
I have muscle spasm – particularly for dislocations – which can be as painful as the nerve pain.
Blood vessels burst.
The nerve pain – it is variable from sensations of freezing water running, to thousands of razor blades down my leg and in the sole of my foot, comes in bursts
The joint and soft tissue pain is less dramatic – but is constant aching and draining. My joints can burn or feel icy and my shoulder feels like the bone is grating against metal, whilst the soft tissue (ligaments etc) throb
Migraines – visual disturbance…white blobs!, strange smells like burning rubber, pressure inside my head and stabbing pains over eyes
Stomach & gut pain – feeling of food being stuck in gullet/stomach with sharp pains – better controlled now I watch my diet
Usually 3 – 4 days when I am worse than usual….fatigue is awful and often can’t sit up, find it much harder to cope with pain, poor concentration, more difficulty controlling body temp – hot one minute & shivering the next. I need to dress in a swimsuit with a fur coat over the top! Difficulty sleeping and I just don’t want to communicate with anyone! Brain fog!
Some days better than others! Try to accept that I can’t do anything and not feel guilty….colouring seems to help calm down the severe pain in back & leg, whilst mindfulness/meditation can help calm mind and breathing when I feel out of control. I get plenty of family support – usually – although my kids can’t understand why an untidy house causes me to freak! Netflix….binge watching easy viewing, and also talking books. If I can, light stretching exercises
Changes in temperature is a main trigger (getting in out shower), weather changes, overdoing it!, not taking rest breaks, some foods with gut problems (onion, garlic….which I love),
Pregabalin 300mg twice a day
Midodrine 5mg three times a day
Fludrocortisone 200mcg daily
Fluoxetine 20 mg daily
Oxycodone, naproxen, co codamol all when needed
Movicol when needed
About to start iron
My biggest tip would be to keep active for as long as you can and look after yourself during your teens – no party tricks!
Also we are all different and don’t compare yourself to others – it is easy to fall into the trap of symptoms becoming competitive, or worrying why something hasn’t helped you that helped another. Don’t assume that certain comorbidities will happen to you!
Accepting your condition isn’t negative, and can actually be very liberating
My illness has impacted positively due to meeting others and the huge support in both local support groups and online. Starting my blog. Understanding the condition and getting my kids help now which may lessen impact/symptoms for them. My husband giving up work to be my carer – double edged sword, but it has benefited the family having him around, and his health too (he now works part time at the local primary school doing sports and loves it) Life has slowed down and it is good . Soft, silky skin!!
Negatively – losing independence is biggest impact! I no longer drive and need someone with me most of the time. Going from being the nurse to the patient has been tough. Reduced mobility and increasing pain – and I feel terribly guilty for impacting on the family’s life.
Neurogenic bladder means I can’t feel when I need to go – so more inclined to urine infections, and sometimes have to self catheterise
Navigating the benefits system! Constantly feeling the need to prove that I can’t do things, and I’m not a sponger.
Being unable to make advance plans and stick with them.
It has stopped me from driving (mainly down to POTS but also not able to drive with my spinal cord stimulator switched on – but pain too bad with it off to travel), travel and over seas trips (hope this will change), walks with the family & dog, just getting up and doing something without having to think about it!
Exercise classes, dancing at parties, anything requiring me to stand
Working as a nurse
I have always been very sociable, gone out with friends, enjoyed parties etc – so the impact from my side of friendships feels huge, and I still miss how my friendships used to be!
Difficulty for friends understanding that I may have to cancel at the last minute, I get tired easily.
Some friendships have just drifted away, whilst with others I feel that I am a nuisance and have to keep excusing myself. I feel that my friends’ lives have moved on and I have stood still.
I feel unable to join in with outings in the same way now and worry that friends will not want to invite me as it is too much trouble.
But then I have a core of very supportive friends – some who I wouldn’t have thought would be with me still now! I appreciate the importance of “good” friends!
Keep busy – don’t let it stop me from doing things other than when in a flare. My blog has led to joining various groups, chronic illness and others, to becoming a book reviewer and also to becoming the volunteer online Pain Advisor for the UK disability charity Scope. I have been a school governor and am now chair for my school’s Friends association (for school alumni). Try to remember that there is always someone worse off than me.
Definitely made it my business to become very aware of my own condition and spread awareness, but also for other conditions too. I think that I have a greater understanding of my patients in the past.
My hope for the future would be for the family to keep as fit and well as possible – and the same for me! Would be great if condition stabilised…
To continue blogging and being able to support other bloggers and meet new people – this year I was able to go to the Bloggers’ Bash and meet some online friends in person.
To stay positive and keep living life as best I can…..enjoying family friends, a meal out and a glass of wine
and maybe see some medical breakthroughs to help future generations!!!
- Medicines described in this blog are prescribed to this individual by their Doctor. Always consult your Doctor about what is best for your needs before taking any medication -