/ surgery \

It’s often an inevitable part of EDS unfortunately. We are instructed to try physical therapy first, maybe steroid injections- less invasive measures if possible. When all else fails, we often need surgery.

My left wrist has been bothering me for quite some time. On and off for years, but more specifically and consistently since October of last year. I had a very minor fall last autumn, but it doesn’t take much when your joints are loosy goosy. I developed two cysts that are deep and pressing on a very painful nerve. In addition I have swelling in the surrounding tendons and the combination leaves me unable to bear any weight on that wrist.

I tried PT and steroids, but neither of those would remove the actual problem. One orthopedic surgeon recommended a splint and more PT (too conservative at this point). A second recommended she do a full wrist arthroscopy and explore the surrounding tissue to see what’s wrong (a bit too invasive and unnecessary). A third recommended he excise the cysts and send them off for biopsy (they’re technically tumors and although malignancy is doubtful, it’s important to rule it out) and see if their removal relieves the pressure and crowding of those nerves (juuuust right).

I haven’t set a date yet. I have to see when it makes sense regarding my work and kids schedule. Probably in about six weeks or so.

I’ve only had two prior surgeries to this one. I’m thankful this procedure is nothing more pressing at the moment. I’m also thankful for second (and third!) opinions! The second surgeon was really pushy, and super impersonal. I felt like I had to do what she wanted, but it felt so wrong in my gut.

Surgery is deeply personal. Someone is literally inside your body. Having that person be qualified to be present and skilled both clinically and emotionally is priceless.

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#ehlersdanlossyndrome #EDS #zebrastrong #POTS #MCAS #mastcellactivationsyndrome #craniocervicalinstability #CCI #dysautonomia #hashimotos #chronicillness #spoonies #alopecia #alopeciauniversalis #surgery #chronicfatiguesyndrome #CSF #ME #butyoudontlooksick #invisibleillness

/ dog days of summer \

Every spring I tend to get anxious as the impending and inevitable heat of summer approaches. Heat intolerance is alive and well in this body of mine. It is a symptom of POTS that has been getting worse with time. For me, the heat triggers tachycardia, pain and tingling in my arms and legs, lightheadedness, headaches, and just an overall feeling of yuck. I used to feel like I had to tough it out. Now, I unapologetically hop from one air conditioned environment to another, waiting out the dog days of summer until that sweet crisp autumn air greets me. Who’s with me?

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#ehlersdanlossyndrome #EDS #zebrastrong #POTS #MCAS #mastcellactivationsyndrome #craniocervicalinstability #CCI #dysautonomia #hashimotos #chronicillness #spoonies #alopecia #alopeciauniversalis #chronicfatiguesyndrome #CSF #ME #butyoudontlooksick #invisibleillness

/ what is POTS? \

POTS stands for Postural Orthostatic Tachycardia Syndrome. It’s a condition that affects blood flow/circulation upon standing. Orthostatic means relating to an upright posture. Tachycardia means rapid heart rate (generally greater than 100). Symptoms and severity can range from person to person and can include lightheadedness, rapid heart rate, and fainting (syncope). I know some who are or have been bed bound due to this condition. I know some who are barely symptomatic. I am symptomatic. My heart rate fluctuates from 50bpm sleeping, up to 180s at times just walking around (not exerting myself).

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I was diagnosed with POTS after an active stand test (heart rate and blood pressure are monitored from lying down, standing, standing for 2, 5, and 10 minutes), in addition to a tilt table test, and something called an Autonomic Breathing test. I have a more rare form a POTS called Hyperadrenergic POTS. (Upon finding this out I laughingly said to the doctor “You mean my body can’t even do POTS right 😂?) This form of POTS involves the sympathetic nervous system. Basically, simply standing up puts me in “fight or flight” mode. My heart rate increases, but instead of my blood pressure dropping (as it does in more traditional POTS patients), it actually increases until it can stabilize itself. I’ll get dizzy, often shaky, and clammy.

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Another symptom I get is blood pooling. Basically my veins are not pushing the blood back to my heart the way they should. After many detailed tests, my doctors found I lose almost 50% of my blood volume in my heart just by standing up, a number they both kept saying was “very significant”. My feet and hands turn purple and mottled, and my veins swell up. At work I use knee high compression socks, but in the summer those are difficult to maintain. They started me on a medication that can be used to regulate heart rate and increase venous return. However it was making me even more dizzy than I already am, nauseas, gave me slurred speech, and caused terrible feet and abdominal muscle cramps/spasms. I stopped taking it and will see what our other options are at my next follow up.

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The other thing the doctor mentioned is that sometimes some of these symptoms can be caused by or exacerbated by craniocervical instability. So until we get that assessed further (happening soon!) treatment is on hold. For now, I’ll keep up with my water intake, my salt intake, and let the old Fitbit keep me tuned in to how I’m doing.

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Below, first picture, you see a few periods where it really spikes up. At these points I just stood up and was walking slowly.

Below that, second picture, you see a comparison of my heart rate verses my husbands on a typical day. His is the top, mine is the bottom. The yellow indicates your heart is going too fast.

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#ehlersdanlossyndrome #EDS #zebrastrong #POTS #MCAS #mastcellactivationsyndrome #craniocervicalinstability #CCI #dysautonomia #hashimotos #chronicillness #spoonies #alopecia #boundaries #alopeciauniversalis #chronicfatiguesyndrome #CSF #ME #butyoudontlooksick #invisibleillness

/ I N V I S I B L E \

Ehlers-Danlos, POTS, MCAS, and many other disorders are considered invisible illnesses. They are disease processes that are not always apparent to the naked eye. Those suffering from from these illnesses are often told things like “But you don’t look sick”, or given recommendations of diet/supplements that are inappropriate, or are told they should silver line everything and just be grateful they don’t have X or Y. 

While these are definitely frustrating aspects of an invisible illness, I’ve been grateful in a way for the option of anonymity. It’s why I wear my wig, why I got my eyebrows tattooed on, and why I wear eyeliner to hide the fact I don’t have eyelashes. I personally feel comfortable with my hair loss. But this gives me control over who I choose to talk to about my situation. It’s complicated and exhausting to discuss and educate people, especially strangers, over and over again. If I look healthy, boundaries are automatically established unless I choose to disclose what’s really going on. It’s the tiny bit of control I get in this process. 

The diagnoses of craniocervical instability has me nervous. The diagnosis itself is obviously a significant one. But at this stage, the thing I’m most dreading is losing that anonymity. I hear all the time of those needing cervical collars or other assistive equipment getting asked completely inappropriate questions from strangers in public places. My threshold for providing education and grace waxes and wanes. If a cervical collar is necessary, I’m not sure how I’ll handle losing those boundaries that have been provided by my process having the option of being invisible up to this point. Maybe this resonates with you. How do you cope with this? 

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#ehlersdanlossyndrome #EDS #zebrastrong #POTS #MCAS #mastcellactivationsyndrome #craniocervicalinstability #CCI #dysautonomia #hashimotos #chronicillness #spoonies #alopecia #boundaries #alopeciauniversalis #chronicfatiguesyndrome #CSF #ME #butyoudontlooksick #invisibleillness

what is craniocervical instability?

Craniocervical Instability is structural instability of the craniocervical junction (where your head and neck meet). It can lead to a pathological deformation of the brainstem, upper spinal cord, and cerebellum. It can also occur with something called Chiari Malformation, a herniation of your cerebellum down your spine. It primarily occurs in patients with Ehlers-Danlos Syndrome and other hereditary disorders of connective tissue. For more information about CCI, visit this website http://www.thepainrelieffoundation.com/craniocervical-instability/
I was diagnosed with Chiari Malformation in 2015 through MRI, with EDS in March of this year, and just two weeks ago, with Craniocervical Instability through CT scans. CCI can look different in different people. My personal symptoms include tachycardia and HR Instability, headaches, neck/spinal pain, paresthesias, pressure in the back of my head and behind my ears, vertigo, heat intolerance, periodic difficulty swallowing, tinnitus, balance problems, and two episodes of facial numbness. 
While the diagnosis of CCI explains a lot, this is a much more complicated answer than I had hoped for. Treatment is often complicated and surgical depending on prognosis. The first line of treatment is often slapping on a cervical collar to stabilize everything and see if symptoms improve. So if you see me in one of those, this is why. I go July 25th for more extensive imaging, and then have an evaluation with a highly recommended neurosurgeon on July 29th. I’m hoping for more definitive answers on what this new diagnosis means for me. 
I’m a little scared. I’m a little frustrated with my body. I’m a little relieved and validated. But mostly I’m holding a posture of openness to get all the information I need and trust that I’m exactly where I’m supposed to be. 

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#ehlersdanlossyndrome #EDS #zebrastrong #POTS #MCAS #mastcellactivationsyndrome #craniocervicalinstability #CCI #hashimotos #chronicillness #spoonies #alopecia #alopeciauniversalis #chronicfatiguesyndrome #CSF #ME #butyoudontlooksick #invisibleillness

at least it’s just hair

This is me. This picture was taken on my 35th birthday. I had a full head of long, curly hair my whole life. At 30, my eyebrows started falling out. After my first daughter was born, I lost about 80% on my hair on my head. After my second daughter was born, I lost all my hair in my body, including my eyebrows and eyelashes. I’ve had a mixed relationship with my alopecia. I tried everything to stop the hair loss. The hardest part, though, was some of the things people would say to me. No one was ever deliberately mean, but they were thoughtless and would project onto me how they felt about my alopecia, unsolicited. Or they would compare their hair complaints to my alopecia, as though they were the same thing.

The hardest one for me to hear was “At least it’s just hair”. My body was (and still is!) going through so many other internal changes and symptoms. My hair loss was an outward representation of something terribly wrong in my body. Emotionally I’ve grown to embrace, or at least accept my alopecia. I’d obviously prefer to have hair on my head (and eyelashes!) However, I know many in the alopecia community who were devastated by their hair loss. No one should ever tell someone how they should feel about their illness.

At this point, I’ve become grateful for my hair loss. After so many doctors have blown me off for other symptoms for so long, the alopecia waves the red flag that something is up. I’ll take the silver linings where I can find them. 

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#ehlersdanlossyndrome #EDS #zebrastrong #POTS #MCAS #mastcellactivationsyndrome #craniocervicalinstability #CCI #hashimotos #chronicillness #spoonies #alopecia #alopeciauniversalis #chronicfatiguesyndrome #CSF #ME #butyoudontlooksick #invisibleillness

why the zebra?

Medical students are taught the phrase “When you hear the sound of hooves, think horses, not zebras”. This is in reference to a rare disease or condition- consider the more common diagnosis first and don’t jump the gun thinking it’s something more outside the box. Unfortunately, many doctors never get to thinking about zebras even after the horse is ruled out. Ehlers-Danlos syndrome is considered a rare condition (though I personally think it’s more common than we think, just under diagnosed), hence those of us with EDS being referred to as Zebras.

While I can appreciate this mentality, and understand the reasoning behind this kind of teaching, this mindset has been incredibly damaging to me and many of those in the zebra community. This kind of mentality leads to underdiagnoses and years of unnecessary physical and emotional suffering. After I received my diagnoses in March of this year, it took months for it to even sink in and for me to actually believe the doctor and what she was telling me. I kept trying to really internalize these new diagnoses and what it meant for me, but all I kept thinking was “She probably just gave me a diagnosis because she knew I was looking for one”. After unpacking this further, I realized this was a result of being told for many many years that I was fine. That it was me. That I just needed to meditate more. Have you tried yoga? (yes) Have you tried acupuncture? (yes) Are you taking B supplements? (yes) Therapy? (yes). These questions also evoke a sense of shame- like it’s my fault I feel this way. Like I’m not doing enough for myself. If I just did X or Y better, I would feel better. Doctors were condescending and would patronize me like I was making a big deal out of nothing.

Cardiologist: “You’re having heart palpitations all the time that take your breath away and make you super dizzy? Well, sweetheart, with that cup of coffee you drink in the morning, it’s no WONDER you feel that way!” (For the record, I did stop drinking coffee for a season and still got heart palpitations.)

Endocrinologist: “You’re hair is falling out? I mean, you did just have a baby. That’s totally normal”.

Me: <shows him how I’m 75% completely bald at this point, before I lost all of my body hair>

Endocrinologist: “Oh, well, you just need to try and get some rest. Stress’ll do that to ya.”

Former PCP: “You eat two eggs in the morning at the same time? You should never, EVER, eat two eggs at the same time.” (literal actual advise from a doctor. Because clearly, occasional eggs are the source of all my problems. Right up there with that one cup of coffee in the morning).

Functional Doctor: “I have some great meditation apps that can teach you mindfulness and relieve your anxiety.”

Don’t get me wrong, I’m all for mindfulness. But mindfulness won’t fix my labral tears. Mindfulness won’t fix my cervical instability. Over and over again I was told that I was the reason for my health problems. I wasn’t doing enough, yet I was trying so hard. I started to downplay my symptoms. They’re telling me I’m fine, right? Guess I must be fine. Having a doctor tell me for the first time how unstable I am was simultaneously scary and so relieving.  I hear a lot of zebras feeling this way. I wonder how our journey’s could be different if we didn’t have to experience the emotional trauma of disbelief and belittlement, and could move towards a direction of awareness, support, and education from the beginning.

how did i get here?

Ten years ago, I started down what was the beginning of a long and exhausting journey. I didn’t know it at the time, but my life was soon to be changed in multiple ways. What started as generalized fatigue eventually moved on to constant stomach aches, headaches, joint pain and hair loss. I was diagnosed with Hashimotos disease, hypothyroidism, and alopecia. I was put on medication, but my symptoms progressed.  I started experiencing heart palpitations, vertigo, unexplained rashes, constant muscle pain, insomnia…. One day I collapsed due to debilitating hip pain. I went to the doctor and was diagnosed with adult onset hip dysplasia and a Femoral Acetabular impingement. I needed surgery. Seriously? Where was all this coming from? My body felt like it was falling apart piece by piece, and none of it made any sense. I ate right. I took care of myself. I used to be so physically active. I used to bike 15 miles a day. Now, walking up a flight of stairs is challenging. How is it now that I felt so terrible all the time? 

I started seeing one doctor after another. My endocrinologist recommended I see a neurologist, who recommended I see a cardiologist, who recommended I see a rheumatologist, who recommended I see a neuromuscular neurologist and on and on it went. I tried healing through functional medicine. Natural homeopathic remedies. Acupuncture. Essential oils. Supplements. Anti-inflammatory diets. Test after test was run. I became friends with my phlebotomist. I was worked up for multiple sclerosis, lupus, Lymes disease, rheumatoid arthritis, even blood cancer, which sounds awful. All negative. I was called “medically fascinating” more times than I could count. 

As time went on, I felt defeated. I was Discouraged. Hopeless at times. And felt so alone in the process. I couldn’t even go to a support group because no one knew what was wrong with me. Was this the best I was ever going to feel? Is it all downhill from here? 

This past fall, I started experiencing excruciating shoulder pain. After trying the chiropractor, more acupuncture, and a massage therapist with no relief, I finally went to see my doctor. She sent me to physical therapy, saying I might have to have another surgery depending on what was wrong. The physical therapist diagnosed me with a labral tear and generalized hyper mobility of my joints. A lightbulb went on. A dear friend of mine had suggested I might have this genetic disorder she also had. Hyper mobility was a tell tail sign, and I had many of the other symptoms. This disorder is not well understood by most doctors, and my heart couldn’t take another doctor not knowing what was wrong with me or blowing me off. She suggested I see a specialist in Virginia. 

On March 7th, I was seen by a doctor who not only knew exactly what was wrong with me, but could explain how all the symptoms were connected, including the hip surgery I needed and constant labral tears. I was diagnosed with Ehlers Danlos Syndrome type 3, Mast Cell Activation Disorder, and hyper-adrenergic POTS. After some follow ups and some imaging, I was also diagnosed with something called Craniocervical Instability. This is a scarier diagnosis, but I see a neurosurgeon who specializes in CCI fot patients with EDS on July 29th to find out exactly what this means for me. 

This past decade has felt sacrificing, contemplative, and deepening. For the first time in a decade, I have some answers. The suffering I have endured has ironically been a constant reminder of all that I have to be grateful for. It has given me an opportunity to spend time in some thin places, and hold both the darkness and the light. Through suffering there has been opportunity for resurrection not only in my journey, but also for those who have journeyed beside me. I appreciate it through the lens of C.S. Lewis, “I’m not sure God wants us to be happy. I think he wants us to love, and be loved. But we are like children, thinking our toys will make us happy and the whole world is our nursery. Something must drive us out of that nursery and into the lives of others, and that something is suffering.”

what’s in a spoon

I chose this name for two reasons. The first being for those of us who are #spoonies, I figured you’d gravitate to it and know this is a safe place for you here. You are seen and there is room for you at the table. The second is for those who don’t know “The Spoon Theory” and are seeking enlightenment on how they can better connect with and support their loved ones who experience chronic illnesses. This might be a helpful tool for you too.

The Spoon Theory is a personal story told by Christine Miserandino. Christine suffered from Lupus. The direct link to her story is here, https://butyoudontlooksick.com/articles/written-by-christine/the-spoon-theory/ , but the idea is the metaphor of spoons as finite units of energy. Those who are healthy possess a more-or-less infinite amount of energy to complete the tasks of a normal day. These are for things one can take for granted, like getting dressed in the morning or cooking a meal. However, those with long-term illness don’t have this luxury. They wake up in the morning with a fixed amount of spoons. For some it might be 12, for others it’s 6. And they must choose, do I get groceries? Or do I take a shower? If I have lunch with that friend, will I be paying for it for a day? You are in constant negotiation with yourself, borrowing a spoon from tomorrow to complete a task of today. It is exhausting and difficult to explain to those who aren’t in the trenches as some are.

This place is a space to both learn and grow together. I want to share my experience. I want to hear yours. I want to offer compassion and grace, both to those around me and also, the hardest part, to myself. This is about me, and it’s also about you. Let’s sit at the proverbial table and really know we’re not alone, and recognize what a gift that is.

Don’t worry, I saved a spoon for you.