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I have no idea what it’s like to live with an invisible illness. I’ve never been questioned for using my disability permit, and no one has doubted the pain I was in. A hidden blessing for a very visible disability!
When I was born 8 weeks premature by emergency caesarean, the doctors and Mum and Dad were stunned. Apart from requiring life-saving treatment, I was floppy, and also had major obvious deformities of my limbs and dislocated hips. I had contractures in my hands, fingers, shoulders, hips, ankles and feet. I have very little muscle in my neck, arms and legs. I spent 10 weeks in NICU and was diagnosed with Arthrogryposis at birth.
I walked at 3 ½ with callipers. Throughout my childhood and adolescence, I underwent serial casting and splinting, and many, many surgeries to correct the deformities so I could walk and use my hands. I developed kyphoscoliosis at nine. Once my hips were fixed, I didn’t have any more dislocations but the bones in my feet used to break constantly (from very minor trauma).
By the time I was 18, I did start to notice a few things extra health issues, although I had no idea why! I didn’t heal well at all, and I used to bleed heavily. I was very prone to infection and was in and out of hospital with asthma. My feet were so, so painful (later diagnosed with Complex Regional Pain Syndrome from surgeries to fuse my ankles). I was working as a nurse and I used crutches on my days off and walked as little as possible. My feet would swell so much and turn purple, and my right ankle had multiple infected ulcers and septic arthritis that took forever to heal.
The contractures affect me in different ways. My hands are always clenched, and they do not open out properly. I’m unable to tie my hair up myself unless I am lying on my back, which allows gravity to pull my arms back. I can’t put my hair behind my ears with my right hand unless I’m using my left hand to push it backwards. Although I am right handed, the fingers on my right hand don’t open more than 2 inches and I’m unable to pick anything up with that hand. My left hand is far more deformed, but I can grab things with that hand and use it to stabilise my right. I carry things on my right side in between my arm and torso in the crook of my elbow and I look like an axe murderer when holding a knife. My whole body is generally quite weak, due to lack of muscle.
I knew without a doubt that I had something else going on by the time I hit 30. I had met others with arthrogryposis by this stage and not one had any other health issues. I was on the hunt to explain my health problems, but the issue was that diagnoses stick, and no one wanted to challenge arthrogryposis. Every specialist and doctor were quite happy to treat each individual complaint, but not admit there may be something else going on. They didn’t think there could be someone living with something so rare in New Zealand.
I became really unwell and was transferred to a major hospital outside my province. One of the specialists I was seeing suggested that there is a very rare type of Ehlers-Danlos that includes arthrogryposis, and it could be causing my gastroparesis. I had always brushed EDS off because of my contractures, but I had no idea how hypermobile I was until I started on some serious self-exploration! I couldn’t believe that it took 37 years to realise that I had hypermobility as well as contractures. I can do some crazy party tricks. I still haven’t quite worked out how the same joint can be both contracted and hypermobile at the same time, depending on what direction I move. I can’t do my hair very well, but I can do the reverse namaskar sign effortlessly, as well as hold my wheelchair handles and bring my elbows almost together out the front of me.
I discovered musculocontractural EDS (mcEDS) and I was stunned. Reading the criteria was like reading my own medical history! Sign after sign, and symptom after symptom, the puzzle pieces were put together. I couldn’t contain my excitement that possibly after 37 years I would have a complete diagnosis that would explain everything. It was incredibly validating – I wasn’t imagining it!! So many things made sense.
To describe mcEDS, I would say that it’s two separate conditions put into one: arthrogryposis and EDS. My symptoms are split between vascular and classical types. I have aortic root and arch dilatation, neurogenic bladder, snail’s pace colonic transit, gastroparesis, dysphagia and achalasia, dysautonomia, neuropathy, endocrine issues, kyphoscoliosis (T 100: L 55) adenomyosis, osteopenia and osteoarthritis in most joints including my neck and spine. I have bipolar 1. Those are the big things, but there are plenty more little things.
I can walk but I use a power chair most of the time. I wear a spinal brace which holds me upright, and finger splints to help with the pain, stiffness and hyperextension. I have electrolytes running into my tube 24/7 and manage to eat about 6-700 calories a day. The fatigue is a killer.
To keep me busy, I am involved in a number of community initiatives. I also speak to student nurses about my experiences with mental and physical illness and am a Support Worker for those who are struggling and in respite care for mental health issues. I love my rescue chickens, rescue greyhound and my garden. These things really keep me going.
Susan David says, “Life’s beauty is inseparable from its fragility”. Life is deliciously fragile, inherently beautiful and insanely chaotic, and I don’t I want to miss a second! I want my experiences to be used to help others. For all the pain and suffering, I want good to come out of it. My body has been so faithful! Doomed from the beginning, and even with my crazy looking joints, I really am in awe of my body.Tags: Coping, Diagnosis Journey, mcEDS, Musculocontractural, Musculocontractural EDS
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