ehlers-danlos syndrome: what it feels like
10 minute read
growing up, kristy dickinson thought her frequent health concerns were just a case of bad luck.
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growing up, kristy dickinson considered herself unlucky when it came to injuries. joints would pop out of sockets, she had back trouble, and she needed abdominal surgery. it wasn’t until after the birth of her third child that a health crisis led to a rare disease diagnosis: ehlers-danlos syndrome.
ehlers-danlos syndrome is a group of genetic conditions that affect the connective tissue which provide strength and support to the structures in the body, according to the mayo clinic. depending on the sub-type, symptoms may include very flexible joints, fragile skin, and bruising easily. in more severe cases or sub-types, veins and arteries may be at risk of rupturing due to mutations in connective tissue.
healthing spoke to kristy about what it was like navigating the healthcare system with a complex and rare condition, and how the experience led to the launch of her company, chronically simple, which offers a health app to help patients and caregivers manage their care.
this interview has been edited for length and clarity.
what led you to being diagnosed with ehlers-danlos syndrome?
it took me almost seven years to get the diagnosis. i never really thought of myself as sick growing up, but i had a lot of bad luck. i now know that “bad luck” was actually my condition, it just went undiagnosed. as a child i had scoliosis in my back, my joints would pop out and i had a massive abdominal intestinal surgery the year that i got married. every time i saw a doctor, i would hear phrases like, “we just don’t see this and people your age,” or “we don’t understand why this is happening.” it took a long time to put the right pieces together.
what were the clues?
i have three kids, and with each pregnancy my health deteriorated a little bit more. by the time i had my third child, i was in crisis. i had to step back from my business — in my family we call it “the pause.” i was an active parent, on the pta, and i ran my own search firm. i would get sick, it would throw me for a loop, but then i would get better and just keep going, until my body said no more.
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what did the crisis look like?
i try to avoid being in the hospital as much as i possibly can, but that year i had a lot of recurrent kidney infections that required iv antibiotics. sometimes i would have an infection that wouldn’t respond to antibiotics and the doctors would have to try something different. that summer, i’d had an antibiotic resistant infection, which is scary. i was too sick to soldier on. so, when i think about a crisis, it’s when i’m not able to power through or be an active mom to my kids, or to be an active employee in my business, or an active member of society. that to me is a crisis.
how did you finally get a diagnosis?
i got very lucky. i also have medullary sponge kidney, which is what was behind the recurrent kidney issues. if you think about your kidneys, they are a smooth organ; mine is shaped like a sponge. it’s got divots and that leads to recurrent kidney stones and infections. i was in and out of hospitals so much, finally, i got to the point where i knew something needed to change. i began to focus on the only thing i could control — my diet. i met with a dietitian who incidentally also had a job in genetics at mcmaster hospital, and once i described everything that had been going on, she immediately referred me to a geneticist, and said, “i think you’ve got eds.” that was the turning point for me.
and then what?
i’d be lying if i said it was a party. i took some time to mourn my expectations, because no one grows up thinking, “i’ll have kids and then i’ll get a life changing diagnosis.” i thought i had things figured out — i was happily married, had three healthy children and i was running my own business. the diagnosis turned all of that on its head, but it also opened up such a massive world for me in terms of what i chose to do afterwards. i’m grateful that i have the life that i have and that i’m able to live with my condition the way that i am. i can still contribute to society and try to help people — this is what ultimately fuels me.
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what is it like to live with eds?
i’m a little bit complicated because i’ve got a few diagnoses, but eds is my primary diagnosis. basically, connective tissue is the glue that holds your body together, but with eds, that connective tissue is faulty. it can be difficult to diagnose because it presents differently in every person.
for me, i’ve got the hyper-mobility — my joints have severe instability. i also have some vascular symptoms, which make me bruise easily. i’m at risk for things like organ rupture and aortic dissection. and then there are the comorbidities that come along with this disease, such as mast cell activation syndrome which causes anaphylaxis reactions even though i don’t have any allergies. i also have ankylosing spondylitis (as) — a form of arthritis that causes fusion of my spine and other joints.
all of this is a challenge because you treat one thing, and then that impacts another thing. i was diagnosed with eds seven or eight years ago now and the as diagnosis came later. it’s difficult because the treatments contradict each other. for as, you have to keep your body and joints moving. but for eds, my joints are always in pain. so what do you treat? it’s always a trade off.
tell me why you started chronically simple.
because eds impacts so many different areas of your body, my health care is divided over toronto, halton and hamilton and 10 different specialists. i used to think that when i saw a doctor, my health card was swiped and all of my health information just came up on their computer. i now know that that is incorrect. i was always wondering why i always had to tell my story again and again.
i was also on different medications and treatments which dulled my cognitive ability. i just didn’t feel sharp. i would leave doctors’ offices and forget what they said. or i would forget to ask questions. it was very frustrating. i started to ask people who i knew were on a health journey for advice on how to manage all the appointments and keep track of all the information. the answer was, overwhelmingly, binders.
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i started keeping paper copies of everything. i also wrote everything down. i’d have a list of questions to take into an appointment with me and then, afterwards, i would sit in the waiting room and write out everything that the doctor had told me so i wouldn’t forget. this started as a file folder, and then it became a binder, and then a very thick binder. i started thinking that there’s got to be a better way.
i went looking for healthcare apps that could help me consolidate my health care. there were thousands of health and wellness apps, but i couldn’t manage 10 different apps — i was having a hard enough time keeping track of everything in my binders. and so chronically simple was born. a centralized hub that helps patients and caregivers keep everything together from an administrative perspective so that we can focus less on managing the full time job of being sick and more on just getting to our version of better.
what makes me most proud about chronically simple is that every data point in that app comes from a patient’s or a caregiver’s pain points of a real lived experience. it doesn’t matter what the diagnosis is or whether or not it’s a rare condition or cancer journey, we’re all trying to navigate the same healthcare system. ultimately, we know that an engaged patient — someone who takes an active role in managing their health care — typically has better outcomes.
that’s a lot. living with a rare disease, being a mother and running your own company.
i am very grateful that i have a supportive family. i built chronically simple from my bed for the first year. i had no technical background but i surrounded myself with people who had expertise in areas that i didn’t. i can do a lot of it from home and at times that work for me — i have more energy in the morning and up until early afternoon. i do a lot of my work, and i’m most active with my family, usually until about four o’clock, and then things are pretty quiet for me. it’s taken years, a lot of therapy and self-awareness to be able to acknowledge when my body needs to rest. i’m not very good at it. i still try to accomplish what i could have 10 or 15 years ago and i am constantly challenged to acknowledge when maybe i’ve done a bit too much.
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can you share some tips on how to manage activities with energy?
i know that there are a lot of eds patients or community members that are significantly more impacted than i am. i’m still mobile. i can’t pick my kids up, but i can go for walks with them. we don’t go skiing, but i can certainly stand at the bottom of the hill and wait for them. so, it’s just been about finding that balance. my body also lets me know when i’ve pushed it too hard — it’s taken a lot of years, but i’m really in tune with my body now and knowing what i can and can’t do.
part of this is understanding that everything comes at a cost. i know that if i want to take my kids to canada’s wonderland, that means i can’t do anything the next day or that i will be working from home that next day because, physically, i won’t be able to go into the office. i have to put everything into my hierarchy of needs: my family comes first — being present for my kids and active in my family is my top priority. but a really close second is the work that i do with chronically simple because i know that we’re making an impact on people’s lives.
if i only have a set amount of time on this earth, i want to do something good with it. if i can raise awareness for my condition and help people feel more empowered to take an active role in their care, then i feel like maybe my path or my journey has meaning.
are there treatments for eds?
there is no cure for eds. i manage my pain on a daily basis, and then i might have acute symptoms or occurrences. for example, i might roll over during the night and my rib will pop out. that can cause costochondritis, which makes breathing challenging. i never know when a joint is going to slip out, and that instability for me impacts daily activities. there are also specific exercises and physiotherapy that help strengthen the area around the joints.
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how do you explain eds to your children?
i’m careful with how much they know because i don’t ever want to put the burden of my illness on them. they know that i have a rare disease, and there are things that i can’t do. a lot of times we read books together in my bed versus theirs if i’m having a bad day or if i’m ill. i balance a lot of guilt because i wish that they didn’t have to worry about me.
i don’t ever want to be a burden on my family but i also believe in being honest and transparent. they know that we have to be careful about germs because i’m on immunosuppressant drugs. my son had pneumonia a few years ago and it was my husband that took him to the hospital instead of me. that killed me as a mom but makes practical sense for our family. that is by far the hardest thing about my condition — i would deal with my physical symptoms 150 times over if it meant that i didn’t worry my family or be a burden on my kids, my parents or my husband.
one thing eds has done is teach my kids a bit about empathy. one day, my oldest son came home and told us that he has a new friend. i didn’t know for the first few months that this friend was in a wheelchair and nonverbal because my son never described him that way. he just saw him as this amazing little boy that he had met.
my kids understand that i’m a little bit different and that’s okay. there’s a lot of people out there that are navigating different conditions or living with disabilities and that doesn’t define them.
click here to find out more about chronically simple.
if you or someone you care about is living with eds, there are resources available to help provide support, education and advocacy. contact ehlers-danlos syndrome canada for more information.
what it feels like is a regular series that explores the experience of being diagnosed, treated and living with a health challenge. have a story to tell? email info@healthing.ca.
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emma jones is a multimedia editor with healthing. you can reach her at emjones@postmedia.com or on twitter @jonesyjourn
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