Demystification

My Ehlers-Danlos Challenge Part 3

Day 21: Wear Red for Vascular Ehlers Danlos Syndrome

So glad I’ve seen the Jesus & Mary Chain three times

Day 22: Proudest Moment

This photo is from two years ago. It was from my first day back to work after my patellofemoral (kneecap) replacement and MPFL (tendons) reconstruction. I was in a locked Bledsoe brace (like the QB before Brady) for two months. I was stuck at home for all that time. It was a painful and difficult physical therapy/recovery.

I was very happy and proud to get back to work at the library. I wasn’t quite able to drive, so my coworker, Donna, picked me up and my husband drove me home. I am very lucky to have a supportive and accommodating work environment!

Day 23: My Wish for Ehlers-Danlos Syndrome

My Wish for Ehlers Danlos syndrome (A day late because I felt like trash yesterday … because of EDS 😂). Here’s a picture of me before I knew I had EDS. Of course I was good at ballet like most hypermobile folks. 🩰

What is my wish for Ehlers Danlos syndrome? Well, first off, I would use any hypothetical medical wish for Huntington’s Disease, which has affected my paternal side of my family so horribly. Or for Parkinson’s Disease and Lewy Body Dementia, which claimed my parent’s life.

I do wish that there was a cure and/or treatments for all types of EDS. I was diagnosed with type III hypermobility. I haven’t had the actual genetic testing to rule out vascular EDS, which is much more serious (thanks, health insurance!). My wish would definitely start with cures/treatments for the more deadly EDS types. Another wish: greater empathy, knowledge, understanding in the medical community.

Day 24: Future Goals

Have a kick-ass life with the man in this photo and our two cats. (Photo by Jessica Cronin Photography)
Hug my nephew on my birthday (June 20) coz he’ll be fully vaccinated! Also hug Lisa Love as soon as my husband is fully vaccinated!
Go to lots of concerts including but not limited to Ash, Nick Cave, Pillow Queens, Nine Inch Nails, Wolf Alice, Harry Styles, The Cure, Piroshka, Garbage, Kero Kero Bonito, The National, Matt Berninger, Hinds, Miley Cyrus (so I can see Stacy and Jamie!) Black Rebel Motorcycle Club, Belly, A Band of Their Own, Vast, Dubstar, Gillian Welch, New Order, Pet Shop Boys, The Joy Formidable, Taylor Swift, Letters to Cleo, Butch Walker, and more!
Visit museums.
Keep working part-time at the library.
Have joint stabilization surgeries.
Continue physical therapy to strengthen my body.
Travel more.
Write a book (I’ve been saying this forever. People tell me I should.)

Day 25: Share Your Visible

Trigger warning: scars/staples

Here are some photos that show the not-so-glamorous side (ha) of Ehlers-Danlos syndrome. In photo 1, I’m dealing with an intractable migraine and insomnia. Photo 2: In the Emergency Department because of a postural orthostatic tachycardia syndrome episode, but looking like ET. Photo 3: Pre-surgery, I think? Dunno which surgery. Photo 4: Taking a break in NYC. I’m at that point that I need a rollator for vacations and walks. My HurryCane doesn’t cut it. I need to upgrade to a rollator that converts to a wheelchair. Photo 5: Post-op photo after my patellofemoral replacement and MPFL reconstruction with cadaver tendons on my left knee. My right foot/ankle is on deck. MRI on Tuesday to determine how extensive the surgery will be. Then I have an upper GI endoscopy and a colonoscopy the following week because of my gastroparesis/gastritis flare/history of cancer. Ah, summer fun!

Day 26: Self-Care

I’m suffering from a horrible, intractable migraine right now, so I’m keeping this one short. I don’t know what I’d do without my cat Candy. My best self-care option is her on my lap while I read, play videogames, watch TV/movies, or listen to music. She makes me feel so much better.

Day 27: Supports/Aids

Here are a bunch of pictures of me with various supports. 1. HurryCane at the Peabody Essex Museum 2. Knee and ankle braces in a Hospital Glam pose 3. Bledsoe brace and Candy O. 4. In NYC with friends and my rollator 5. My cats Ada Monroe (gifted to me by another support Lisa Love 💗) and Candy O. 6. “Dancing” with my HurryCane (decorated in blue roses) and my husband at our wedding. Photo by the lovely Jessica Cronin Photography.

Day 28: Act of Kindness

I didn’t know what to put for this prompt so I talked with my partner. He said, “You help LGBTQ kids at the library.” My response was that it was my job and that it didn’t count. He thinks it does. 🤷🏻‍♀️

Here’s what I do: I work part-time at a library. Since 2018, I’ve been facilitating an LGBTQ+ youth group. At my previous university library, I was trained as part of the university’s “Rainbow Network” to support LGBTQ+ college students, and I’m bi with a transgender family member, so it was a natural fit when I started working at a public library to work with LGBTQ+ youth.

Pre-pandemic, we met in person and would watch movies, do arts and crafts, have guest speakers, etc. Since the pandemic, we’ve been meeting via Zoom. They’re such a great group of kids and I love seeing them thrive. I’m very protective of them.

Day 29: Research Hopes for Our Future

Hospital glam with HurryCane just out of view

I wish that there was something life-changing like Fasenra for EDS. Since I’ve been on Fasenra injections for eosinophilia my allergies and asthma have been so much better. When I see @wheelchair_rapunzel improving with her “twerk juice,” I hope for something like that! Imagine if the collagen could be replenished? If our unstable joints could become stable? If we didn’t have to go through painful surgeries to replace worn out joints and tendons at young ages? I believe in you, science! You can do it!

Day 30: What Is EDS Echo?

“EDS ECHO® a program for healthcare professionals across all disciplines who want to improve their ability to care for people with EDS, HSD and associated symptoms and conditions.” (Taken from their website)

Day 31: Community

Though I haven’t met many of my fellow EDSers in person, I do feel like I have a great little community here online! We’re able to support each other with tips, tricks, and simply commiserating! Who ever knew that @ryan_the_zebra_dulcimist was my twin? Or that @librarychristi and I have more in common than library stuff? @heytrollop has given me countless helpful recommendations! And of course I’m eternally thankful for @karolynprg for her #HospitalGlam inspiration! I know I’m forgetting others that I have connections with, but I am a bit POTSie at the moment. I’m so thankful for my online EDS community and my online friends in general! You all support me and/or let me live vicariously through you.

My Ehlers-Danlos Awareness Month Challenge Part 3

Day 11: Nature/Environment

I try to go for a daily walk with my husband (health and weather permitting) and my favorite part is spotting the little wild bunnies in our neighborhood. There’s also an orange outdoor cat that I’ve nicknamed Irn Bru.

Being outdoors is a challenge for me. I’m allergic to so many things like grass, trees, lilacs, THE SUN. Yes-I have a sun sensitivity. I break out in hives. I have to wear sunscreen and/or wear protective clothing.

But I do still love the ocean. I was lucky to attend my friends’ commitment ceremony on Martha’s Vineyard. That’s where this photo is from. It was a gorgeous day.

In addition to my allergies/asthma, I’m very sensitive to temperatures because of postural orthostatic tachycardia syndrome. My blood pressure runs very low and my heart rate runs high. Combined with EDS and one of my medications that decreases the amount I sweat, it’s very difficult to regulate my body temperature. I overheat very easily. I do best with temperatures in the 60s.

Honestly, I enjoy nature the most when David Attenborough narrates.

Day 12: Nurses/Doctors

I’m lucky to have several medical providers who treat me with great respect and care. I’ve also had truly awful experiences with some doctors and nurses. The majority of my medical care providers are excellent and that’s why I travel from NH to Massachusetts for my care; the staff members at Beth Israel Lahey Health are professional and kind.

Off the top of my head, I currently see specialists in the following fields: allergy & immunology, cardiology, gastroenterology, podiatry, orthopedics, physical therapy, otolaryngology, psychiatry, neurology, neurosurgery, dermatology, pain management, gynecology, cancer, and urology.

I feel like my physical therapist is one of my closest friends. I’ve been seeing her every week for over a decade. When I got married, I almost felt like I should invite her! Having her as an ally is wonderful. She has taken a keen interest in Ehlers-Danlos syndrome (EDS) and is always keeping me in the loop on new specialists who are aware of EDS. Her comprehensive documentation of my health and treatment makes it so much easier for other specialists to get a handle on my case.

Day 13: Medical Appointments

I’m pretty tired today because I just got home from my last medical appointment of the week. I had five appointments this week. I’m exhausted.

Being a patient is like a full-time job for me. I like to take notes and make sure that I get any questions answered. Virtual appointments have definitely made my life easier! I hope that we don’t lose them.

Above are some “Hospital Glam” shots of me (do yourself a favor and check out Karolyn Gehrig!) at various medical appointments. They help me reclaim the space.

Day 14: Explaining EDS

I posted this Stickman illustration to my Instagram stories

Day 15: Isolation

As mentioned in an earlier post, I missed three months of junior high when I was a kid because of pneumonia. I’m used to isolation.

After most surgeries, I’m stuck at home for a month or so. But I’m also stuck at home on a regular basis because my body just refuses to cooperate. Whether it’s a migraine, gastritis flare, or postural orthostatic tachycardia syndrome (POTS) incident, I spend I lot of time alone.

Fortunately, I’ve got my unofficial emotional support cat, Candy, and my husband. Plus all you lovely people on the internets!

Day 16: Coping

I slept most of yesterday because I worked four hours in person at the library the day before and it took a lot out of me. I ended up with a horrible migraine. But thankfully, I have my cats (like little Ada in this photo) and my husband who help me cope. Other things that help me cope are therapy, doodling, connecting with friends and family, reading, watching TV/movies, keeping a journal, and not feeling guilty when I need to rest.

Day 17: Childhood

I was sick a lot. I had my first ear infections at age 2. I had pneumonia twice around age 8, which is when doctors found that I was missing part of my pericardium. My allergist spotted it (or lack of it) on an X-Ray. I spent a good deal of time seeing cardiology specialists because at the time, I was one of only two documented cases of people born without complete pericardia (wall around the heart).

When I wasn’t suffering from respiratory infections, my childhood was pretty normal and excellent. I took ballet and was in Campfire Boys & Girls. My local public library was my favorite place to visit. I spent many hours in books. I was in the “gifted” program in elementary school.

My joints crackled and popped and subluxed, but no one knew why. I’m kinda glad I didn’t know what I had because my life might’ve been more protected. I relished being latchkey kids with my older brother, who was my idol.

Things didn’t really start to fall apart until age 12. First surgery. First migraine. Insomnia. POTS episodes had started earlier. I think high school would have been easier if I had known because I could have worn appropriate braces and used mobility aides.

Day 18: Words to Parents

My EDS is not your fault, Mom. I love you so much! You and dad did everything you could for me. I have all your cardiology notes. I know how thorough you were! It must have been scary not knowing what was wrong with my heart when I was little. You don’t have to worry anymore because I have the best partner and doctors taking care of me.

Day 19: EDS In One Photo

My wedding day. Photo by Jessica Cronin Photography

I think this lovely photo, by Jessica Cronin Photography, from my wedding day works to sum up EDS in one photo. My face is chubby from a month of prednisone (intractable migraine) and I’ve got my trusty rollator all decked out with blue roses in honor of Twin Peaks. And I “don’t look sick,” but my maid of honor was responsible for knowing where my bag of abortive/rescue meds was at all times.

I was lucky to make it through the wedding without any major problems. It was a gorgeous day at the deCordova Sculpture Park and Museum and I felt like a princess! And that is pretty much what EDS is: You don’t necessarily look sick, but man, so many things are just falling apart under the surface. (I almost picked a gnarly post-op photo of staples and stitches in my knee, but who wants to see that?)

Day 20: Behind the Scenes

Here is a photo from the last time I was hospitalized. I had Type A flu in January/February 2020 and got very sick. My immune system sucks. I developed ketoacidosis.

When I was released from the hospital, they told me to wear a mask to protect others from getting the flu and to protect myself from getting more sick.

I was in the Emergency Department more recently for an intractable migraine (I think in December?) and it sucked because I was all alone. Usually my husband is by my side as my advocate. I wish there was a better way to treat intractable migraines than to send me to the ED when I need IV meds/fluids/infusion. Unfortunately, my hospital doesn’t have an infusion center that can handle folx with postural orthostatic tachycardia syndrome and episodes that look like seizures. Sigh.

Anyhow! A big thank you to Karolyn Gehrig and expressing myself through Hospital Glam!

Ehlers-Danlos Awareness Month Part 2

Part two of the Ehlers-Danlos syndrome Society’s social media challenge for the month of May, which is Ehlers-Danlos syndrome (EDS) Awareness Month.

Day 6: Management Strategies

Aside from joint stabilization surgeries (like the one in this X-ray), joint replacements, and tendon replacements, my biggest management tool is physical therapy. I’ve been seeing the same physical therapist for over a decade at Beth Israel Lahey Health and she’s amazing.

Other tools in my arsenal include loads of medical specialists, therapy (the psychological kind), two monthly injectable medications, daily medications and vitamins, massages by the incomparable Sarah J. Hebb, hair by Sonya at Widow’s Peak Hair Studio, my amazing unofficial emotional support cats, daily walks with my brilliant husband, having supportive family and friends, my hobbies (reading, writing, drawing, watching TV/movies, playing videogames), and jelly beans.

Other strategies include things like carrying snacks and water with me wherever I go, as well as any abortive medication I may need for nausea, migraines, asthma, etc. I’ve been using hand sanitizer since the ‘90s when my immunologist made me a germaphobe (and I was diagnosed with an immune deficiency).

I wear masks whenever I clean or I’m going to be exposed to a large amount of allergens (like weeding books at work or folks mowing lawns). Along with my mask collection, I have an extensive array of braces for knees, ankles, wrists, and more. I also have special pillows. The most special pillow is a large Star-Lord Tsum Tsum that the husband and I rescued from the clearance bin at a Disney Store.

Probably the most important management strategy is to accept that my body is unreliable. As much as I may want to do something, if I’ve just had a postural orthostatic tachycardia syndrome (POTS) episode and passed out, I probably have to call it a day.

Day 7: Comfort

Honestly, I feel best when one or both of my cats are resting on me. They apply just the right amount of warmth and pressure!

Also key to my comfort: our adjustable bed and couch (the couch reclines and has adjustable neck and lumbar support). Plus: all of my special pillows and braces.

Another aspect of comfort control is staying at a reasonable temperature. My body has a hard time regulating its temperature because of the lack of collagen. Also, I have postural orthostatic tachycardia syndrome (POTS), so I need to stay cool. Plus, one of my meds is dehydrating and diminishes the amount I sweat. So! If I don’t want to pass out, I need good temperature control. (Conversely, if I get too cold, my extremities go numb.)

Lastly: comfortable clothes and shoes! Stretchy waistbands and tights are key when you have gastritis and gastroparesis. And I always want my pants, dresses, and skirts to have pockets, so I can carry stuff like my inhaler and phone. I’d like to have more accessible clothing for post-ops, like pants that button along the outside leg and fit over bulky braces or casts.

Day 8: Movement and Activity

Out and about with my rollator (AKA a fancy walker) and my husband

Here’s a shot of me out and about in the Before Times with my partner. I’ve got my rollator and we’re inside a Yayoi Kusama exhibition at Boston’s Institute of Contemporary Art. I use the rollator for our daily walks around our neighborhood (when I’m feeling well enough to walk). When I’m going to medical appointments or errands, I use a cane.

My main source of movement and activity comes from weekly physical therapy appointments and doing physical therapy exercises at home. I used to go twice a week to physical therapy but their appointments are limited because of COVID.

When I lived in a city (4 years ago), I walked way more, though still using a cane. I also practice a little bit of yoga. I used to take classes. I miss those! I wish I had easy access to a pool because swimming is great exercise for those with Ehlers-Danlos syndrome. But my favorite activity is walking around museums!

Day 9: Inclusion

Elephant and Piggie at the Boston Book Festival eons ago

This one is tricky. There’s personal inclusion, like what’s represented in this picture of Sarah and me with Cheryl’s child at the Boston Book Festival a million years ago. Both Sarah and Cheryl are examples of excellent friends who always make me feel included, even though they know that when they invite me to do stuff, there’s a good chance I won’t feel well enough to partake.

When I travel with Sarah or plan to do something with her, she always makes sure everything is accessible in advance. She speaks up for disabled access on social media even when it doesn’t involve me! I could ramble on and on about how amazing she is. I’m lucky to have so many friends and family like Sarah, Cheryl, Lisa, Kenji, Milee, Shannon, Bethany, Saint Mary, and more who keep me included in their lives. A special shout-out to Erin who lets me live vicariously through her Disneyland adventures! 💗💗💗

Another type of inclusion that I think of is in pop culture. Seeing folks from the Oscar nominated documentary Crip Camp on the Red Carpet was so exciting! I want to see more disabled people in film, TV, games, literature, etc. Characters who just happen to be disabled but aren’t defined by their disability. I’d love to see a semi-ambulatory character who uses a wheelchair but occasionally walks. Because we do exist!

Then there’s accessibility as inclusion. I hate stairs. Currently, I can go up them, but going down them is VERY painful, even with my cane. During various times of my life I’ve not been able to walk at all. Why do stairs still exist? Why can’t we have ramps everywhere? I bet folks with baby carriages would like that! Architectural and city planning with better accessibility is my dream.

Day 10: Mental Health

I’ve suffered from insomnia since I was a teenager. I’ve suffered from anxiety since my 20s and reactive depression since my cancer diagnosis in 2014 and EDS diagnosis in 2015. Reactive depression means that the depression is triggered by external forces, like a diagnosis of an incurable degenerative disease. I also have PTSD from my first marriage.

I “see” (talk on the phone because of the pandemic) a therapist biweekly and a psychiatrist every couple of months. I’m on an antidepressant, have an as-needed anti-anxiety medication, and taking a sleeping pill.

Right now, my depression is pretty bad because it’s been triggered by a new anti-seizure medication. I’m pretty down unless I’m physically at work at the library. I’m having a major gastritis/gastroparesis flare that is contributing to the depression. I hate eating. I feel sick to my stomach constantly. My cats make me feel better. Seeing my mom and mother-in-law yesterday helped.

In addition to my new med triggering depression, it’s also triggered my postural orthostatic tachycardia syndrome, so I’m pretty light headed and showering is even more difficult than usual. Last week, I passed out after I showered. Fortunately, my husband is the best and now I wait to shower until he can monitor me.

I’m really looking forward to getting my hair cut by Sonya at Widow’s Peak Studio this week. It may seem like a silly thing, but when I look good, it helps my mood. Also, it will be easier to take care of shorter hair/showering.

Once again, I’m lucky to have supportive friends and family. Things that help me deal with depression/anxiety include doodling, crossword puzzles, reading, listening to music, rewatching Twin Peaks, texting with my nephew, watching old episodes of RuPaul’s Drag Race, and watching movies with my husband.

Ehlers-Danlos Awareness Month

May is Ehlers-Danlos syndrome (EDS) Awareness month. The EDS Society has issued a social media challenge to raise awareness. Today is day five and I’ve been making my posts on Instagram. Today’s got rather lengthy, so I figured I may as well start sharing them on the blog as well!

Day 1: This Is Me

This Is Me. I’m all bendy. I’m more bendy than this; it was hard to take the photo.

Days 2 & 3: Diagnosis and Symptoms

I was born was EDS. Check out how I’m sitting in this photo. It was great for my knees! I was diagnosed as being hypermobile when I was in my 20s but I wasn’t diagnosed with EDS until age 40. I had been diagnosed with and cleared of cancer before I received the EDS diagnosis. Heck, I didn’t know what EDS even was until I was probably 39.

Some of my EDS symptoms include long arms (my arm span is 5’7” but I’m 5’3”), long fingers, migraines, joint instability and subluxation, gastritis, gastroparesis, eczema, osteoarthritis, chronic pain, scoliosis, chondromalacia, impingements, muscle spasms, pericardial malformation, and more.

Day 4: A Day With Ehlers-Danlos Syndrome

Last night I got to sleep around 2 AM after a gastritis flare. I’m seeing my gastroenterologist next week for this latest flare-up. I woke up at 2:30 PM when my husband woke me up. I was exhausted. I got up and made some toast with vegetarian butter and had some iced coffee. I took my three “morning” pills and sat on the couch with my work iPad.

I ended up working from the couch for three hours with this cat on me. She makes my knees feel better and our couch reclines and even has lumbar support. Now it’s almost 7 PM and I’m due to take more meds. Meds again at 9:15 PM and then 11 PM. I’ll try to take a shower, but my husband will have to sit in the bathroom with me because my postural orthostatic tachycardia syndrome is acting up and I passed out yesterday after I showered. Oh, I also have a migraine.

The husband and I will probably watch a movie or some TV later. I’ll read some of the new Kazuo Ishiguro before bed. I feel down because I didn’t go for a walk today. And because I slept so much. And because I have no motivation. The gastritis/gastroparesis flare severely limits what I can eat. I’m scared that the ulcer in my stomach is getting worse. I have to eat a low-fiber, low-density diet. No raw fruits or veggies. 😢 Now I’m just on a rambling rant. At least I got to rest today. On Thursday I have 4 medical appointments. 🥺

Day 5: School/Teachers

Playing the glockenspiel (note my excellent friend, Kim, on sousaphone)

Here I am in my awesome marching band uniform! I couldn’t handle marching with the glockenspiel because my shoulder would dislocate so the marching band teacher created a percussion pit! Whenever we marched in parades, I would play the cymbals instead.

But not all of my teachers were so accommodating. I remember a math teacher being mad because I couldn’t take a test because I couldn’t write. I had a bad dislocation of my right shoulder and my arm was in a sling. I was severely underweight and everything was always falling off me. 😂

I had a lot of “tendonitis” and dislocations in high school, but junior high was the worst. I was out of school for three months (pneumonia) and had what would be my first of three sinus surgeries. My parents hired tutors so that I wouldn’t fall behind at school. Three months of medical isolation with no internet prepared me for 2020.

When it came to school, I always got my work done early because I never knew when I would get sick. I had my first loss of vision migraine at age 12 after gym class. I never knew when my body would fail me. I always got all A’s and B’s. I was in National Honor Society and took Advanced Placement classes. I did a million extracurricular activities. In college, I was always on the Dean’s list. Now, I don’t know how I did it!

The answer: supportive family, friends, and medical providers.

Prednisone

It’s been eons since I’ve written. I had a bad bout of depression and insomnia. Not saying that either is gone, but now I’m on prednisone for another intractable migraine and I just hate how it makes me feel and I wanna vent.

I feel like my legs aren’t part of my body. The past few days they’ve just been moving of their own accord. Maybe like a cross between myoclonic jerks and restless leg syndrome? I’m not sure. I had nine medical appointments last week and I’m depleted.

My heart rate is very low for me (93 BPM resting), but it feels like it’s so fast. I have postural orthostatic tachycardia syndrome, so usually my heart rate is high and my blood pressure is low.

I got a lot of sleep last night and when I woke up, I whispered to my husband, “I feel better. Don’t tell anyone!” He said he could tell that I felt better. But then I took the prednisone and I’m back in the PredniZone.

A graphic that reads “Warning: I’m in the Zone … The PredniZone.”

I want to do things, but my body isn’t cooperating. I managed to wash some dishes and fold some laundry before the myoclonic jerks kicked in again. I want to take a shower, but that’s such a daunting task. Sigh.

The upside: I’ve been fully-vaccinated. I got my first dose of the COVID-19 vaccine in January. My husband is scheduled to get his first dose later this month. I started a new medication that I hope will help with the “spells” I have. (I had another abnormal EEG last week)

For now, I’ll try to calm myself with some music or entice a cat to sit on my lap. The cats really help! I’m so lucky that they are in my life. And that my husband just brought home Eggo waffles. I’m like “Eleven” from Stranger Things and live off of them.