Living with Ehlers-Danlos syndrome (EDS) is a lifelong process that requires constant monitoring on how to remain active, yet also physically secure and safe. EDS is a condition caused by defective connective tissue, the “glue” that holds the body together. Any sudden move or jolt, and your bones can easily sublux or even fully dislocations. There is presently no cure for EDS, so living life with this condition means accepting and addressing chronic pain.
These are safety tips that I have learned to follow that I hope will help others, like myself, living with EDS.
To prevent your sacrum from shifting out of place while getting into and out of a car, it is best to find a car seat where you do not have to either dip down or lift yourself up when getting into the seat. If you can just slide into the seat, you have the best chance of staying in position.
To get into the car with the least chance of slipping out of position, I sit down on the seat facing to the side of the car, turn towards the front of the car and then I swing my legs onto the floor.
Check and see where your legs are when you sit down. It is best if they are at a ninety-degree angle, not above your waist or below. The best way to judge may be to focus on your knees. If they are higher than your hips, you are probably in trouble.
If you are sitting in a chair and something drops to either side, for many of us with EDS, the most damaging thing we can do is lean over to the side and reach down to pick it up.
That will cause what is called an “up-slip,” where the femur jams up into your hip. It does not hurt at first but tends to show up the next day and is very uncomfortable. To check if you have created this problem, lie on a bed, arch up and then gently put your legs down. Check to see if your knees match in height. If there is an up slip, there will be a difference in the leg lengths. Get it corrected as soon as you can before it creates significant pain.
Using downward pressure while attempting to open a can with a can opener can cause you to potentially sub lux your hand, fingers, elbow and/or shoulder. A simple fix to this is to purchase a product called the Handy Can-Opener. All you do is set it on the top on the can, press a button and let it do the magic of opening the can for you!
Sleeping Safely with POTS
Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia, a condition that causes light-headedness, fainting, unstable blood pressure, abnormal heart rates, and sometimes even death. I learned I had it in my 60’s.
I was instructed to sleep at a 30-degree angle by raising the entire frame of the bed at the head. At home, this is a simple process to do by putting boards under the frame. But what about traveling and staying in hotels?
We found out from a physical therapist that there are bed raisers sold that college students use to raise their dorms beds up to be able to store items underneath. We purchased four plastic bed raisers and found that if we use two on each of the head corners, we can raise hotel beds up for me to simulate some of the height we have at home.
It is not as high as my bed, but certainly better than sleeping flat with POTS.
Greeting Friends and Family
If you are like me, when others see you, they assume you are fine and don’t understand that a simple hug can cause subluxations. Many times, when my husband is with me, he will warn others to not touch me.
The hardest thing is when I am by myself and someone throws me off and suddenly is greeting me with a hug. I almost wish I could wear a sign that says, “Do Not Touch.”
Try to stay vigilant and ward off the damage that comes when someone who means well greets you, only end up hurting you my mistake.
I was taught in physical therapy that when you twist, you must move from the hips. I made the mistake of twisting just from the waist and proceeded to sublux my back out. With EDS, when you throw something out of place, it can take weeks for it to settle down and hold properly again.
When sitting, it is also important to not cross your legs, for this can throw your sacrum out of place.
If you have flat feet, getting good arch support is a must. Also, if you are having problems with your legs and/or feet subluxing, then wearing sneakers with the arch support inside them is the best bet. Also find sandals that have a good arch when you are not able to wear sneakers.
Trachea, hyoid and Neck Stability
I have spent many years dealing with a trachea, hyoid and sternum that shifts out of place. Despite sleeping with a bi-pap breathing machine, I have had many episodes in which my breathing was cut off. My lifeline at night for many years has been my service dog alerting me when the air flow has decreased or cut off.
I am now a proud owner of a new pillow another EDSer discovered that is holding the neck and head in the correct position and not allowing the trachea to collapse. I would encourage you to give it a try. It is called Therapeutica sleeping pillow and mine came from Core Products International.
Be sure to get the correct size. I had to exchange mine down to a child’s size to correctly stabilize the head.
For most of us, as we progress with EDS, holding items in our arms is painful and can cause more issues. While I was still teaching, I finally resorted to buying a luggage with wheels, like you see in the airport.
I don’t know why I hadn’t thought of that sooner. I used to carry 125 students essays and my books up to the second floor of a large school. I would ache for days after doing it. But once I switched to pulling the bag, life had a positive change. Today, I pull my swim items into the pool and can be more self-sufficient this way.
ER SAFETY TIPS
People living with Ehlers-Danlos Syndrome (EDS) are often afraid to go to a hospital emergency room, due to a lack of understanding in the ER staff on how to safely care for them. I had a negative experience, one that almost killed me.
In the process of being admitted, after passing out over and over due to low blood pressure, things went terribly wrong. While being transferred from the ambulance to the hospital stretcher, my hip was dislocated. This dislocation was unintentional, but avoidable, as it was a direct result of the rough way the transfer was managed.
On top of this, they gave me no IV fluids for several hours, which should have been the first thing done to help elevate my blood pressure. Complicating matters even further, no food was brought to my room that I could safely eat and metabolize for the two days I was there.
And then, when a nurse thought I had stopped breathing, she compressed my chest to stimulate my heart, even though I was wearing two medical bracelets warning I shouldn’t be given chest compressions. Two years later, I am still paying for these mistakes.
As a result of my traumatizing and life-threatening experience, I sent a letter to the hospital in the hopes of never having another EDS patient experience what I did. I was shocked to receive a call that resulted in the hospital taking me up on my offer to meet with their doctors and nurses to help them understand how to properly handle us.
To prepare for this meeting, I asked other EDS patients to submit suggestions to enhance my program. I hope that this list will be helpful to both patients and hospital staff.
How to Treat Ehlers-Danlos Patients
- Consider having a generic EDS protocol for staff to get a quick understanding of this condition with new patients.
- Put notes or a bulletin board or patient chart to share information and keep the patient safe from shift to shift.
- Be cautious, for EDS is an “invisible condition” so remember to do no harm. Understand that touching and moving us can create more problems, so listen to the patient. Tread lightly using chest compressions, because our ribs sublux, dislocate and break easily. Allow EDS patients to position themselves safely before any procedure.
- Subluxations are a real thing. Don’t just take a quick x-ray and tell us, “It’s nothing, you’re fine.” When a joint feels wrong, there’s an injury worth finding some relief for.
- Because we bruise easily, don’t rush to judgment with EDS children before reporting abuse.
- If someone arrives with an ID warning bracelet, please read and respect what is says!
- If a patient has low blood pressure, elevate their bed to a 30% angle. Hook up IV fluids quickly and approve the patient’s BP medication in time for their next dose. Consider using a PICC line if the IV does not hold.
- Many EDS patients are drug reactive, so respect if a DNA drug test has been done or listen to what medications have not worked in the past. Pain relief is difficult to achieve with EDS so please believe the person.
- Some of us use compounded medications that need to be accepted in place of what you have in stock in the pharmacy. Some also use supplements, so please respect the use of them. Many are using the Cusack Protocol supplement routine.
- If a patient is using cannabis for pain control, consider allowing its use in the hospital in an oil, tincture, topical or pill form.
- Many of us are food reactive, so send your dietician to the room to meet the patient and create a safe meal plan.
- If a patient sleeps with CPAP or BIPAP mask, be sure that it gets brought in and worn during sleep.
- Have on staff a physical therapist that can use manual energy techniques for re-alignment or allow an EDS manual therapist on the floor.
- If there is a need to draw blood, use a butterfly or small pediatric needle.
- If there is a need for intubation, be careful with the movement of the neck and use small equipment. If an EDS patient presents with a neck fusion, do intubation using the fiber optic glide scope.
- If stitches are needed, try to use natural products over synthetic.
- Many of us have wound healing issues, so please be careful with the choice of tape and its removal. Some of us have skin that is fragile and easily tears.
- All types of EDS are at increased risk of scary vascular events. Any sudden or severe chest or abdominal pain needs a scan to rule out an aneurysm or another serious condition.
If surgery is needed, be sure to have your anesthesiologist do a pre-op interview before any procedures. Be careful about joint positioning and manipulation when performing anesthesia. Yes, that “jaw thrust maneuver” may make intubation easier or more comfortable, but it’s not worth the months of rehab from a dislocated jaw.
Please reassure your orthopedic residents that we’d prefer to avoid surgery, too. Having them share their fears out loud that they don’t want to operate on EDS patients because “that will just make things worse” may be true, but it’s not helpful. Nobody wants to feel like an untouchable leper. Instead, please focus on what you can do to help. It might be as simple as helping to reposition the joint to a more neutral spot, and then bracing or splinting it there to give things a rest before starting physical therapy
Ehlers-Danlos can be a very painful, isolating and heartbreaking condition to live with. We would love to come to a hospital for emergency help and not be afraid to be sent home in worse shape. Let’s all work to educate the medical field and improve the future for us all coping with this condition. May this list be a start for you!
May life be kind to you,
Ellen Lenox Smith
Author of: It Hurts Like Hell!: I Live With Pain– And Have a Good Life, Anyway, and My Life as a Service Dog!
The information in this column should not be considered as professional medical advice, diagnosis or treatment. It is for informational purposes only and represents the author’s opinions alone. It does not inherently express or reflect the views, opinions and/or positions of National Pain Report.