Ehlers-Danlos Syndrome

Do you feel rested in the morning? Or do you want to roll over and go back to sleep? Maybe you’re thinking, “I’m a great sleeper! I can sleep for 15 hours straight!”

Neither of these are signs of someone who gets restorative sleep and both can be signs of fatigue.

Fatigue is a common symptom of many chronic conditions. Today we focus on fatigue in people living with Ehlers-Danlos Syndrome (EDS), and what anyone whether they have EDS or not can do about it.

The Ehlers-Danlos Society describes the Ehlers-Danlos Syndromes as:

A group of connective tissue disorders that can be inherited and are varied both in how they affect the body and in their genetic causes. They are generally characterized by joint hypermobility (joints that stretch further than normal), skin hyperextensibility (skin that can be stretched further than normal), and tissue fragility.

Ehlers-Danlos Syndrome (EDS) is considered a rare disorder. There are 13 different types of Ehlers-Danlos syndromes. The latest statistics list the total prevalence of all the types as 1 in 2,500 to 1 in 5,000 people. Clinical experience suggests EDS may be more common than that.

In fact, I have hypermobile Ehlers-Danlos Syndrome!  Many of us living with EDS will call ourselves “zebras.”

Why the Zebra?

Medical students have been taught for decades that, “When you hear hoofbeats behind you, don’t expect to see a zebra.” In other words, look for the more common and usual, not the surprising, diagnosis.

But many of us spend years pursuing a diagnosis for disorders that aren’t well known. Or aren’t expected in someone who looks normal, or is too young to have so many problems, or too old. Or even, what we might have is considered too rare for anybody to be diagnosed with it.

So the zebra became our symbol to mean, “Sometimes when you hear hoofbeats, it really is a zebra.” Ehlers-Danlos syndromes are unexpected because they’re rare. Hypermobile spectrum disorders are common, but are unexpected because they remain misdiagnosed or under-diagnosed. 

-The Ehlers-Danlos Society

Okay, let’s get back to sleep! 

There are many reasons why a Zebra might have chronic fatigue:

• Autonomic Nervous System Dysfunction
• Metabolic factors
• Inadequate rest (not taking breaks/feeling guilty about it #permissiontorest)
• Cognitive tasks (which are just as tiring as physical tasks!)
• Emotional stresses (draining)
• Everyday life (which sucks up your energy)

It’s important to keep in mind there is no one thing that will work for everyone. Every person’s body is different. You are the expert in what it’s like to live in your body, and no one else.

EDSers tend to have non-restorative sleep. The most common pattern of sleep disorders in EDS is not enough time in deep sleep (my Oura ring can vouch for this!). The continuity of deep sleep is broken by arousals which cause us to wake up feeling tired. 

Did you know you have to be awake for 2 minutes to remember being awake? An awakening is defined as disruption to continuity of sleep that lasts more than 30 seconds. And less than 30 seconds is called an arousal.

Dr. Alan Pocinki in his presentation on chronic fatigue at the Ehlers-Danlos Society’s 2019 Nashville Global Learning Conference shared that in one sleep study of a woman with EDS, she had no deep sleep or REM, and was not cycling through the stages of sleep. The sleep study showed her continuity of sleep had been broken 150 times in seven hours!

I’m exhausted just typing that.

So what can we do about disordered sleeping?

From the perspective of someone living with a chronic condition with a pain characteristic, there are two areas that can be focused on:

1. Practice good sleep hygiene 
2. Manage pain 

There are many lifestyle interventions that promote sleep hygiene (behaviors that contribute to good quality sleep as well as alertness during the day). Let’s explore five of them today.

1. Control your exposure to light

Light is one of the primary drivers of our circadian rhythm and the sleep/wake cycle.

• Avoid blue light in the evenings and hours leading up to bedtime.
• Avoid or minimize use of electronic screens two to three hours before bedtime.
• If you must use screens install a program like f.lux on your computer, or wear orange-tinted, blue-light blocking glasses at night.
• Use blackout shades or an eye mask to keep your bedroom as dark as possible. 
• Cover or remove anything that emits light in your bedroom like digital alarm clocks. We put black electrical tape on the green digital lights of our window AC unit during the summer months. 

Blue light is not all bad. What matters is getting it at the right time of day. In addition to your sleep hygiene in the evening, you’ll want to make sure you get sunlight during the day. 

• Take a short walk when you wake up
• Eat breakfast outside
• Use a light machine (I do this during the long, dark days of winter!)

2. It’s time to move it, move it!

But you don’t need to even do it that much. As little as 10 minutes of aerobic exercise can dramatically improve your sleep quality. Physical activity also improves sleep duration.

However, the timing of exercise can make or break your sleep. According to the National Sleep Foundation:

Early morning and afternoon exercise may also help reset the sleep wake cycle by raising body temperature slightly, then allowing it to drop and trigger sleepiness a few hours later. It can be especially helpful if you are able to exercise outdoors and let your body absorb natural sunlight during the daylight hours.” 

This explains the sleep-like-a-baby feeling I get in the evenings after an afternoon bike ride!

It’s important to note that strenuous exercising too close to bedtime can actually be detrimental to your sleep quality. This does differ from person to person, so as always, you know your body best – find out what works best for you.

While we’re all familiar with the common refrain to “get more exercise,” exercise is not the only kind of movement that counts. Non-Exercise Activity Thermogenesis is all the movement we do throughout the day that isn’t sleeping, eating, or sports-like. Walking to the store, taking the stairs, doing house or yard work all increase NEAT.

Start observing how sedentary you are during the day and where you might be able to get more NEAT in.

Are you able to take that phone call while on a walk? How about standing at your desk for a bit?

3. Nutrition and Sleep

In general it’s best to not go to bed too full or hungry. And since no two people (or Zebras) are alike, it’s important to pay attention to your body.

Are you someone who needs a snack before bed because you tend to have low blood sugar? Waking up in the middle of the night can sometimes be an indication of this!

Or are you the type of person who does better with a smaller dinner? Is your body getting enough nutrition to help you sleep at night? Low-carb and low-fat diets could also impact your sleep.

Did you know as many as 20% of Americans use alcohol to help them fall asleep?

While it may help you fall asleep more quickly, it leads to poor quality sleep by disrupting your circadian rhythm and blocking REM sleep, your body’s most restorative sleep.

By now we all know about the effects of caffeine on sleep. If you have a caffeine habit that needs breaking it’s best to wean off rather than quit abruptly. 

4. Manage Stress

In today’s day and age, feeling stressed seems like the default.

With a global pandemic, civil unrest, rampant racism, and record rates of unemployment, it’s hard for it not to be!

When every night brings another day’s news cycle depicting the pain, trauma, and devastation occurring around the world to a close, calming our nervous systems can feel like a Herculean feat.

Add to this our propensity to use our smartphones to get those additional hits of the news right before bed and it’s a double whammy of blue-light exposure and stress activation!

Explore different types of stress management techniques and tools like deep breathing, meditation, yoga, prayer, knitting, and more to help you manage stress. 

5. Create and Maintain a Relaxing Sleep Space

Don’t sleep with your kids or pets.

While sleeping with kids or pets might work for some, if you are waking up tired in the morning, you might consider not sleeping with them to see if that helps.

If possible only use your bedroom for sleep and sex.

The intention is for your bedroom to be a relaxing place. If you’re also folding laundry, working on your computer, and eating in your bedroom it can create unnecessary stressors.

Is there a lot of noise outside your bedroom?

Use earplugs, or a noise machine to block it out.

Avoid using electronics in the bedroom.

Study after study shows digital tech activates our sympathetic nervous system making it harder for our bodies to shift into sleep.

I know, “But my phone is my alarm clock!” In some cases, analog is a better choice than digital – this is one of those cases.

The lights from a digital alarm clock (or your phone) are actually bright enough to disrupt your circadian rhythm!

Control the temperature of the room.

Sleeping in a slightly cooler room tends to work best for most.

Many EDSers also deal with dysautonomia, the dysfunctioning of our autonomic nervous systems (ANS). One of the responsibilities of the ANS is to regulate body temperature.

Many EDSers report non-hormonal hot flashes during sleep. Dr. Pocinki in 2019 at the EDS Global Learning Conference shared that these hot flashes are often autonomic in nature. Hormonal hot flashes tend to be in the upper body, chest, and face. Whereas, waking up hot all over usually signifies they are due autonomic dysfunction.

A person with EDS’s body temperatures often bottoms out in the middle of the night, and so it overcorrects. We warm up too much and wake up in a hot sweat with our hearts racing.

The heart racing could be a nightmare or dream, or it could be adrenaline. The ANS is also what tells the body to release adrenaline. 

The Autonomic Nervous System is what keeps us on an even keel. When it malfunctions, things start to fluctuate. Our sympathetic ( fight or flight) VS parasympathetic (rest and digest) responses go out of whack.

When the sympathetic nervous system is activated it causes us to release adrenaline more often. We might overreact to minor stresses in our lives and waste energy that we could be conserving leading to more fatigue. 

How comfortable is your bed?

This might be obvious, but if your bed is uncomfortable, your sleep is not going to be great.

As a hypermobile person, a mattress’s quality and comfort are even more vital. If you haven’t caught on to the theme yet, you know your body best. 

Some considerations for the Zebra:

• A mattress that is too soft could misalign your spine, cause sore muscles, or subluxation/dislocations in your joints
• Too firm and it could cause bruising and pain 

Some people find a softer bed more comfortable, others like a firm mattress, still some folks are in between. Get your Goldilocks on and find yourself a mattress that feels just right.

To sum up

Getting the right kind of light at the right time, moving more, optimizing our nutrition for sleep, managing our stress, and maintaining a relaxing sleep environment all contribute to better quality and duration of sleep. 

When living with a chronic illness like the Ehlers-Danlos Syndromes it’s easy to run out of spoons before midday. The little things add up! And so do the little things you can do to improve your sleep and manage or reduce fatigue. Do you have to cover all five of the areas on this list? No. 

I know how overwhelming it is to live with chronic illness.

What feels like low-hanging fruit? One baby step that feels doable to you now? 

Start there. 

Have you made improvements to your fatigue by working on your sleep hygiene? What worked for you? 

Tell me about it in the comments below!

Stay tuned for Part 2, where I go over how chronic pain promotes fatigue and what you can do about it. 

 

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