Librarian in Black readers have likely noticed that in the last several months I have been blogging less and less. There is a reason for that. I am sick. I won’t die from my illness, but I am, nevertheless, sick. I have debated for years whether or not to share this with my professional contacts and readers of this blog.
I have Ehlers-Danlos Syndrome (EDS), and since May is Ehlers-Danlos Awareness Month, I thought I’d take this opportunity to do some issue advocacy for something that is part of my everyday life.
Ehlers-Danlos Syndrome is an extremely rare genetic disorder that affects the body’s production of collagen. And you’d be surprised by how much collagen is in your body (overall, the body is 30% collagen). It’s in your skin, your ligaments, tendons, etc.
What finally made me speak up was a recent conversation with a young woman with EDS. She was just diagnosed and she needed to build a support network. Tearful in one conversation, she encouraged me to continue helping others to keep them from wanting to die instead of living with the disease, like she had when first diagnosed.
I am writing to raise awareness and to help explain my own erratic activity and involvement in library-land. I am not writing, however, to ask for sympathy and I most certainly do not want any pity-posts. Take this information and maybe you can help someone else in your life with EDS get a diagnosis faster.
If you want a quick, 5-minute video explanation of what Ehlers-Danlos Syndrome is, try this video from the Discovery Channel:
Due to the EDS, every collagen cell in my body is faulty and is programmed to be too stretchy and ultimately becomes like an old stretched out rubber band. Normal rubber bands are tight and when you stretch them they bounce right back to their previous shape. EDS sufferers have collagen that is already over-stretched, so when you stretch it out again, it does not bounce back to its original shape, but stays lax and loose. Therefore, we’re super-flexible…but pushing our joints to their limits (as with stretching, yoga, Pilates, heavy-lifting) does long-term irreversible damage.
Practically speaking, this means that my joints are all incredibly unstable and frequently dislocate. Some of my joints bend completely backwards (I can do neat party tricks like bending all of my fingers to touch the back of my hand) while other joints like my wrists and knees bend about 5-10 degrees backwards. The muscles around every single joint also tend to tighten way up, to do the job that my tendons and ligaments aren’t doing: keeping my skeleton in one piece. Dislocations and always-tight muscles = whole body chronic pain.
There’s a lot of collagen in skin especially (90% of skin is collagen), so my skin is super-stretchy and velvety which means two things: I have great skin that will keep me young-looking and wrinkle-free well into my life. But my skin (as well as other collagen-based tissues) bruises and tears easily and takes ten times as long to heal as normal skin does. This is bad for surgeries, and even for simple injuries like cat scratches. So, I’ll probably look like I’m twenty when I’m sixty, but I felt like I was sixty when I was twenty. I truly, truly do feel like an old woman. My body doesn’t work the way it should. It has betrayed me.
Below are a couple of photos that show what EDS patients look like, so you can get an idea of what I’m talking about (incidentally, these are not me):
Think of EDS like extreme rheumatoid arthritis in every single joint, with the addition of random dislocations. And “every single joint” means just that: fingers, wrists, elbows, shoulders, ribs, vertebrae (a particular bad set of joints for me), hips, knees, ankles, toes, etc. People with Ehlers-Danlos Syndrome experience acute chronic pain in some or all of their joints and experience neuropathic (nerve) pain in some or all parts of their body, and some even have heart defects that can cause sudden death. Most of us have additional problems with vision, digestion, migraines, pregnancy, and our jaws and teeth. It’s an all-encompassing disorder. I have the “Hypermobility Type” of EDS, and my most serious symptoms are in my entire spine, hands, wrists, ribs, knees, and hips. Thankfully, I do not have the “Vascular Type” of EDS, which can cause sudden and early death.
There is no cure for EDS. There are only limited treatments for the chronic pain and over-flexibility. Due to the rareness of the disorder, there is no money to be made by medical companies doing research into medication or other treatments. Therefore, like other rare diseases, we with EDS suffer with little hope for any changes.
What does having EDS mean for me on a daily basis?
I wake up in intense pain every morning and am in pain all day. Literally, I cannot remember the last time that I didn’t hurt. I am on 5 different pain medications which have numerous side effects including memory loss, loss of verbal recall, and sedation. I have limited mobility and strength. If I move too much, it hurts. If I don’t move around enough, it also hurts like the devil. I usually walk with a cane to avoid hip, knee, and ankle dislocations. I have half a dozen different doctors managing my care, which means a lot of time in medical offices, at the pharmacy, experimenting with trial treatments, and in general a lot of time spent on my illness instead of my life. Last, but not least, my life will never, ever be what I wanted it to be as a little girl. My life will likely not be as long as it would be if I did not have EDS. I will always be in pain. I will always have limited mobility. I will always be unable to do the things I wanted to as a young woman: hiking, traveling abroad, painting, calligraphy, yoga, cooking, etc.
I was diagnosed with Ehlers-Danlos Syndrome in 2005, a year after I became symptomatic. It is common for EDS-sufferers to become symptomatic in their late 20s, as I did, and to have a hard time getting a diagnosis due to a general lack of knowledge about EDS in the medical community. Before being diagnosed, most of us are told that instead we have Fibromyalgia or that the pain is simply in our heads–never an inspirational thing to hear.
My first sign of EDS symptoms was (brace yourself) dislocating every single rib on my right side, in my sleep. After an unproductive ER visit, I saw a dozen different doctors and received several different diagnoses before finally being diagnosed with EDS by a geneticist. It fit…all of it. Every symptom, every physical characteristic, my family history, all of it fit.
As you might imagine, in the following week I researched the heck out of the disorder like any good librarian. I checked everywhere. The most useful resource I found was the Ehlers Danlos National Foundation (EDNF). EDNF provides a vast amount of information as well as support groups, research resources, and contacts for advocacy. If you want more information, I would recommend their site as a place to get caught up. You can also review the Ehlers-Danlos Support Group.
After my diagnosis and research, I followed up by trying every kind of doctor or health care practitioner who might be able to help me: acupuncture, chiropractic care, rheumatologists, massage, spine specialists, neuromuscular therapy, cranial-sacral therapy, sleep specialists, chronic pain specialists, support groups, biofeedback, nutritionists, homeopaths, naturopathic doctors. You name it, I did it (and had to pay for it out of pocket, too). I kept busy for a couple of years with doctors, paths of treatment, and hope for a magical cure.
But I never took a leave of absence. I went to work with dislocated joints. I continued to blog. I continued to travel and present. I continued to write. I did not take it easy. I even walked the 7 ½ mile Bay to Breakers Race a few months after my diagnosis to prove to myself that I was OK. But I wasn’t. And a magical treatment wasn’t to be.
In the last few years my health has continued to deteriorate. The last year has been particularly bad. There has been grief; I no longer have that possible magical treatment to look forward to. This is it. I will not get better, barring some spectacular advances in stem cell therapy, which is the only way to heal faulty DNA that produces messed-up collagen.
Living with EDS means that the moment I get home from work I get horizontal to take pressure off of my spine and I don’t get vertical again until the next morning. The same is true at conferences or other events (where you won’t see me at late night drinking fests or early morning breakfasts).
Living with EDS means that I rely heavily on my loved ones (especially my caring husband) to take care of me because I’m frequently incapacitated and unable to take care of myself, whether it will be for a couple of hours, a couple of days, or a couple of weeks.
Living with EDS means that I still am thankful for every day that I do not have to use a wheelchair, days when I can still move and walk around. I appreciate what abilities I do still have even more because I don’t know how much longer I’ll have them. Pain-free days don’t happen, but low-pain days usually result in a flurry of activity while high-pain days mean lots of resting, pain medication, and trying not to cry.
So, to conclude…please do not be sad. Be educated. That is what this post was all about, after all. So, what can you do to make my confession worth it?
- Remember what EDS is and what its symptoms are (hypermobile joints, fragile or stretchy skin, chronic pain). Maybe someday you can help someone else who doesn’t know what’s wrong with them get an early diagnosis and treatment.
- Be patient with me as I have my bad days, weeks, or months. I am not ignoring you, I just can’t muster the energy or get through the pain to do what I have to do sometimes.
- Donate to the Ehlers-Danlos National Foundation.
- Buy flowers through EDNF’s FlowerPetal.com site and a portion of the sale will go directly to EDNF.
- And finally, if you want to learn more, watch this longer video about a woman with EDS, the effect on her life, and her process to diagnosis.
Thank you all for your support, especially those who have known of my disorder and support me through patience, kindness, and making sure I get enough rest at conferences (thanks Aaron!). And as always a huge thanks to my husband for his daily support. It means the world to me.
Update: Librarian Rick Roche has created a wonderful Ehlers-Danlos Resources list for more information: Ehlers-Danlos Syndrome: A Reference Librarian Looks at Consumer Health Reference Sources. Take a look. (and thanks Rick!)
You can download the PDF of the study results, the study instrument itself, as well as a webcast by Char.
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