By Ellen Lenox Smith

People living with Ehlers-Danlos Syndrome (EDS) are often afraid to go to the hospital, due to a lack of understanding amongst the staff on how to safely care for them.

Last year, I had a negative experience in the emergency room (ER), 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, I have food sensitivities, and no food was brought to my room that I could safely eat for the two days I was there.

At another point, 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.

Please note the information in this post should not be considered as professional medical advice, diagnosis, or treatment. It is for informational purposes only and represents my opinions alone.

Tips for managing EDS patients in inpatient settings

General

• 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.
• If someone arrives with an ID warning bracelet, please read and respect what it says!

Avoiding subluxations

• Be cautious, for EDS is an “invisible condition.”. 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.
• Have on staff a physical therapist that can use manual energy techniques for re-alignment or allow an EDS manual therapist on the floor.

Food and Drug Sensitivities

• 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.

Invasive procedures

• If there is a need to draw blood, use a butterfly or small pediatric needle.
• 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.

Comorbidities

• 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.
• If a patient sleeps with CPAP or BIPAP mask, be sure that it gets brought in and worn during sleep.
• 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.

Surgery considerations

• 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 surgery is needed, make sure the anesthesiologist does 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.

I hope that some of the suggestions here will help improve the quality of your life, especially as it relates to inpatient or emergency room care. We need to help each other as we wait for the medical world to get on board recognizing this is a real condition that is often misdiagnosed and mismanaged.

If you missed the first two parts to this series, you can click to read part one or part two.

Ellen Lenox Smith has emerged as a leading voice for patients living with pain. Featured in local and national press accounts, Ellen brings a reasoned and compassionate perspective to the need for safe patient access to effective therapies, especially medical cannabis. Currently, Ellen serves as Co-Director of Medical Cannabis Advocacy for U.S. Pain Foundation and is a member of its Board of Directors. She also serves on the Rhode Island Patient Advocacy Coalition as secretary, was appointed by the governor to the Adaptive Telephone Equipment Loan Program, and is part of the Oversight Committee for the Compassion Centers in RI. She is also active with the EDS RI support group. Prior to patient advocacy, Ellen was a longtime middle school social studies teacher. She has been married for 46 years and is the proud mother of four adult children and grandmother to five grandchildren. She also is the author of two books, an organic gardener, and was previously a master swimmer and high school swim teacher.