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A Demanded Rest: EDS

Ehler’s Danlos Syndrome

As an infant, I had velvety skin. Since I was a young girl, I’ve had various aches and pains. Digestive struggles began when I was about ten. By the time I was thirteen, my knees would swell and hurt. At seventeen, my knees would buckle under me and I had low back problems. When I was 20, I started doing CrossFit and became an avid climber. My symptoms decreased and I found myself in great physical health. No aches and pains. No joint swelling. AH-mazing. Now as a mother, my body is in the worst shape it’s ever been.

I’d never stopped to ask why my body was so frail all my life until I was forced to. This past year has been my most physically painful year. I wake up with strange bruises, swollen and stiff knees. My back is thrown out easily. My elbows begin to ache if I hold my phone, a camera, or even a book for too long. Hips and legs throb after a long day of chasing toddlers and cleaning. But, why? Why all the pain?

Ehler’s Danlos Syndrome is a rare genetic connective tissue disorder. There are various forms of it and I have the most benign form; hypermobile Ehler’s Danlos Syndrome (hEDS), formerly type III. Common symptoms of this condition include stretchy skin, flexible joints, muscular pain, and joint pain. There are various types of connective tissue and I will be the first to admit that I have yet to learn about them. However, there is one that I’ve learned a little bit about called collagen. It’s what holds the connective tissue together – specifically in the joints. Loose collagen creates imbalance and can lead to injury, pains, and strains. This explains why my body doesn’t fare well when my muscles are weak and I’m out of shape.

However, collagen & connective tissue can also be found in other places than the exoskeletal system. And it’s complex. If you want to know why doctors spend so much money and time in school, just look into connective tissue. Connective tissue has various forms and is spread throughout the body. When the very proteins that hold your body together aren’t doing that, the body starts to fall apart. This falling apart results in other illnesses that often coexist with EDS. Here are some examples:

Postural Orthostatic Tachycardia Syndrome (POTS)
Hashimoto Thyroiditis
Mast Cell Activation Syndrome (MCAS)
Dysautonomia
Gastric Dysmotility & Gastroparesis
Cranial Instability
Chronic Fatigue
Sleep & Mental Disorders
Small Fiber Neuropathy

I have most of what’s on that list. Day to day life for me is filled with exhaustion, pain, emotional fatigue, and various symptoms. Trying to rest while managing my body, household, and two toddlers is an impossible feat. But, I take a step back, breathe, and move slowly. I remind myself that this is where I’m at right now and it’s okay. I rely on the truth that Jesus loves me and cares deeply for me. I focus on being present rather than fussing about being sick. Steffany Gretzinger’s album Blackout is on replay in my home. My kids and I watch a lot of Daniel Tiger, Stinky & Dirty, and read books.

I have Ehler’s Danlos Syndrome. I have Christ within me. There is nothing that can separate me from the love of Christ. The peace I have far surpasses understanding and it truly does guard my mind and heart from the foley of self reliance, entitlement, disappointment, and false expectations.

I am okay where I am. It is okay that my body demands rest. As I navigate this demanded physical rest, I will be trying a few things: collagen supplements, simple toning and light strength training, and mindfulness.

Resources:

Gastroparesis

Here’s some raw information. I’ve struggled with constipation since I was about ten years old. (Thanks, Ehler’s Danlos) The vegan diet is the only time I didn’t struggle with it, but still didn’t go to the bathroom often enough. This year, my conditions worsened to the point of needing attention and I finally received diagnoses. All of which are connected. One that is still being tested is Gastroparesis. I’ve been pushing for the testing since June, but I saw a THIRD gastroenterologist once I started vomiting undigested food.

What is Gastroparesis?

For one, it means “stomach paralysis. “It is a disease wherein the stomach cannot empty its contents efficiently. This is due, primarily, to nerve damage to the vagus nerve. Or lack of messages via the vagus nerve like in POTS patients. Symptoms include nausea, vomiting undigested food, heartburn, abdominal pain, poor appetite, weight loss, early satiety, bloating, poor blood sugar control. Some of the risks of this condition are dehydration, malnutrition, bezoars, and bacterial growth due to fermentation of the food sitting in the stomach.

How do you test for gastroparesis?

The primary way is a gastric emptying study. I will share more on that after I have my own next week. In short, you eat some food with radioactive dye and then the food is watched as it travels through the digestive system via a machine for four hours. Another way my doctor informed me of is an electrical test of the stomach. Sort of like an ECG, but for the stomach. Evidently, one can experience tachygastro or bradygastro. Slow muscular stimulation or fast muscular stimulation. Both of which are ineffective.

My doctors are pretty sure I have the condition, but need to do the testing first. Regardless of what is wrong. My day to day life is hard. I want to eat, but know I will experience pain, nausea, possible vomiting, belching and bloating. My appetite is poor and due to possible Mast Cell Activation Syndrome, I can’t eat fresh produce. So, I’m in this catch 22. Eat a burger (because I have to eat SOMETHING) and feel awful for 3 days or eat nothing and feel awful, probably faint, and end up dehydrated. Honestly, I’m to the point where I often choose the latter because of how painful eating is for me and the fear of vomiting. Because it isn’t just vomiting. It’s deep stomach projectile retching that is excruciating, causes a tremor, and leaves me weak and with a migraine.

I miss being able to enjoy food. Especially fruits. Resting with this problem is hard. I find myself in tears most days and frustrated. I lean in though. I can look at it as frequent fasting. Which I hear has great benefits. I take comfort in the fact that I’m close to an answer and that there are lots of medical options out there. I’m sure a feeding tube would beat the physical misery I’m experiencing now. I’ll write more about my gastric study next week.

Inhale. Exhale. My lungs, bladder, kidneys, and liver work wonderfully.

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A Needed Rest: PCS

I’ve carried two humans to term and I’m so thankful for that. After a close call with death during my second delivery, I can no longer have children. My last pregnancy was hard on my body and tipped the first domino of sickness. One of these dominos is Pelvic Congestion Syndrome.

I’d been having pelvic pain for a while. I thought it had something to do with the fact that I had to heal as if I birthed one baby two ways. Or perhaps I needed a chiropractic adjustment. I do have a history of Endometriosis. It was a pain I pushed past until one day my period felt like my first c-section all over again. Sharp, shooting pain traveled across my pelvis and down my leg as I curled up into a ball and cried. Each day of the month was a poco a poco crescendo of an out of tune pitch. My anxiety matched it as I couldn’t stop the crescendo of pain. No pain medicine, home remedies, or self talk helped.

After a CT scan and venogram, my doctor found that my left ovarian vein was incompetent and engorged. This was causing a back up of blood to build up and across my pelvis. My venogram showed severely engorged veins all through my pelvis and down my right leg. It was one of the worst cases my doctor had seen.

An incompetent vein means that the valve within the vein has failed. The valves in a vein are there to stop deoxygenated blood from flowing back down as it is pushed up towards the heart. When that valve stops working, that blood just collects, the vein becomes enlarged, and the back flow of blood effects the surrounding veins causing pain and engorgement.

I’ve had two surgeries to deal with this problem and will likely have to do another one. Insurance wouldn’t cover the embolization the first time. My vein had miraculously shrunk down to normal size the second time and the doctor couldn’t insert the coil to embolize the vein. It was extremely frustrating and as I sit here trying to recover, I have to challenge myself to rest. My normal pelvic pain has returned and trusting this process is hard in this moment. PCS doesn’t just fix itself and my doctor has never seen an engorged vein reverse in size.

Along with the pain, having such severe pelvic congestion actually makes POTS worse! The only form of rest I can muster up for this particular issue is trusting God. Maybe a platinum coil wouldn’t stay put, dislodging and causing an entirely different set of problems. Maybe PCS does reverse itself? Perhaps my pelvis was congested, in part, because of emotional congestion over an assault I endured. I don’t really know. What I do know is that my pelvis hurts and I’m praying for strength to rest. A much needed rest.

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A Forced Rest: POTS

Early 2019 was the first time I experienced what I thought was a stroke or heart attack. My chest ached and a sudden pain shot up from my chest, through my neck and into my jaw. Everything went white and I woke up on the floor struggling to breathe, numb and tingling everywhere, unable to move, and had slurred speech. Paramedics arrived and stabilized me with oxygen and CO2. They tried to sit me up and my stats dropped and I nearly fainted again. A trip to the ER wasn’t of great help as they insisted I was having classic panic attacks. They sent me home with a Ziopatch heart monitor and no answers. The following Monday I met with cardiology. I had a near fainting episode in front of the cardiologist. My ECG read abnormal with lateral and interior infarct and prolonged QT. I was promptly hospitalized and went through various testing.

Lateral & Anterior Infarct

Another way of saying cardiac ischemia. My heart wasn’t getting the blood supply and oxygen it needed resulting in chest pain and the electrical abnormalities.

Prolonged QT interval

This is an incredibly dangerous thing to find on an ECG. The QT interval is the measurement of time on an ECG between the repolarization and depolarization of the ventricles. The longer it takes for the electrical impulses to tell the ventricles to contract and push blood out to the body, the more at risk you become for a fatal arrhythmia to occur. I do have some genetic variations, but not ones typically seen in Long QT Syndrome (LQTS). I’ve not been given the diagnosis of LQTS, just “prolonged QT interval.”

Postural Orthostatic Tachycardia Syndrome (POTS)

They finally did a tilt table test that confirmed Postural Orthostatic Tachycardia Syndrome. My prolonged QT interval doesn’t fit with this or anything else wrong with me. However, I imagine it’s a problem unique to me based on how my mother’s pregnancy went and that I had slow fetal growth. Each day is different. Today just happens to be a day where I am pale, lightheaded and struggling to be on my feet. I have fainted a total of nine times in the last year and have three to five near syncopal episodes weekly. And that is while taking medications to manage my condition.

POTS is complex. It involves the autonomic system overcompensating for lack of cardiac output upon postural change. At rest, gravity pools blood in the lower extremities. When someone stands up who has POTS, it’s as if the blood doesn’t move. Our veins and arteries are innervated by the sympathetic nervous system to create tone that maintains blood flow. Because of the autonomic dysfunction piece of POTS, there is a lack of tone. Thus, vasodilation. And we simply don’t have enough blood supply to fill veins when they are completely dilated. This creates what is called hypovolemia, or low blood volume. I stand up, my blood pressure drops and my heart doesn’t get the blood supply that it needs so my heart rate increases in an attempt to pump more blood. Diagnostic criteria for POTS is a HR change of 30+ BPM upon standing and 120+ BPM after standing for ten minutes. This condition commonly coexists with Ehler’s Danlos Syndrome. Theoretically because of the connective tissue in the venous system.

Fainting is typically a productive thing for the body to do. It’s a forced rest. The brain isn’t getting enough blood supply so you faint in order to restore blood flow to the head. My fainting becomes a problem when my QT interval is sporadically prolonged. This is still a puzzle my physicians are attempting to solve and I currently have a cardiac monitor implanted. So far, we are only seeing short bursts of sinus tachycardia where my heart rate suddenly goes from 75 to 150. That’s a good thing because the alternative is Torsades de Pointes, pauses, Vfib, Afib, and cardiac arrest.

I’ve never fainted alone. I’ve always been near someone who was able to help and there has usually been an EMT present or arrived quickly. Knowing that I have a monitor watching my heart for the next three years and I have doctors who hear me and have worked diligently to figure my case out helps me relax. I lean on Isaiah 40:31 quite literally.

“…but they who wait for the LORD shall renew their strength; they shall mount up with wings like eagles; they shall run and not be weary; they shall walk and not faint.” ESV

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My name is…

Brandi.

Beloved. Wanted. Accepted. Beautiful. Saved by Grace.

Sick. Broken. Messy. Needy.

Truthfully, there are some days I don’t believe that I’m both of those things, just the sick needy girl. Today has been one of them. Nevertheless, I’m here to write about the messy process of resting in the midst of struggle.

What do I mean by rest?

Physically: Lay on the heating pad, couch, or bed as long as I need to.
Mentally: Not fretting over what I’m not achieving or accomplishing.
Spiritually: Recognizing that God has me right in his hands, comforting me and not harming me.

Resting in this season has been a process. I’ve wrestled a lot and have come to a place of recognizing that most of what I’ve been through this year has been outside my control. I’ve learned to take a step back, breathe, and just enjoy the little moments. The extraordinary in the ordinary. Leaning into the rest of Christ when I’ve gone days without eating or I’ve fainted again. Resting, for me, is to be okay. No fear. No shame. Just trusting and being present.

Why focus on being okay when I should try being better?

Better isn’t always the answer.
Being okay with the now leaves room for the surprise of when better comes.
Striving for something “better” isn’t restful. It’s anxiety provoking and tiring.
Being okay in this moment makes me a better person in character.

This isn’t a place where I have all the answers. I don’t have cool tips and tricks for getting better, less sick, or some other cure all. This is a place for process, trial and error, and trying different things to manage my various illnesses. I hope to find relief through some things I try. But, the goal remains the same: resting well.

Forgive the typos and brevity.