By Jackie Kancir
Under any circumstances, the emergency room is a scary place. Just being in the ER means that you (or your loved one) have suffered some kind of emergency, which is stressful (at best). But for individuals with a developmental disability, working with both first responders and doctors once they reach a hospital can sometimes escalate into something much worse.
My daughter, Jadyne, is one such patient. She is an energetic, loving, fun and spunky girl with a rare genetic disorder called SYNGAP1. In fact, Jadyne was the first patient in Nashville to be diagnosed with the SYNGAP1 variation in 2019; only 1,164 patients have been formally diagnosed globally, according to the SynGAP Research Fund. (This number increases regularly.)
For Jadyne, SYNGAP1 can sometimes present in the form of extremely aggressive and/or self-injurious behavior. At times, this creates an unsafe environment for her and those around her. Two years ago, she had an episode while leaving the dentist and needed to be taken to the ER as a result.
For most people, a hospital is the safest place you could be. For Jadyne, and those with similar intellectual disabilities, there is often no clear protocol to follow. Existing rules and procedures do not always make sense in the context of a developmental disability. In Jadyne’s case, she was given an assessment and then put under a psychiatric hold, which made it impossible for me to bring her home until the hospital could find a psychiatrist to perform an evaluation, even though she was by then deescalated and the bright lights, loud sounds, and experience of the ER risked re-escalating behaviors all over with every minute we remained.
She was asked questions about terms she doesn’t have the capacity to consider–things like suicidal and homicidal intent. The doctors saw she had bitten herself and labeled that self-harm, despite my attempts to distinguish self-injury from self-harm. Their protocols were intended to protect an individual suffering from an episode of a disorder that might cause them to want to harm themselves or others, something with an imbalance of a neurotransmitter that might be correctable with cognitive behavior therapy or medications–something other than SynGAP1.
Jadyne never wants to harm herself or others. For individuals with SynGAP1, the issue is farther back in the process, at the very start. The SynGAP protein helps our brains make sense of information they receive, a key mediator of synaptic plasticity. Those with SynGAP1 do not produce enough of that protein. Their neurons thus form many extra dendrites, so they often perceive things incorrectly. Something you or I might find delightful, someone with SynGAP1 might perceive as utterly intolerable, dangerous, or even terrifying. Even a neurotypical person might justifiably respond with aggression if they perceived a life-threatening situation. On the other hand, things that are dangerous, such as traffic on the road or a hot stove, someone with SynGAP1 may not process as dangerous at all. In fact, their ability to register pain might be muted.
Life caring for Jadyne involves a lot of time helping her deescalate and understand a situation she didn’t process well and other times mitigating dangers in the environment she would not recognize as a threat. Most of life with her, though, is simply basking in her contagious laugh and unfettered compassion for everyone around her. I spend a lot of time fascinated by her accomplishments and zest for a joyful life. She’s tenacious, kind, and innocent.
That innocence is one of the few gifts that comes with this rare diagnosis. I’m grateful Jadyne doesn’t understand the darker parts of some human conditions like homicidal ideations. I was appalled at the possibility a psychiatric hold would introduce ideas her innocent mind had never known about previously. Fortunately, she was unable to process that either.
As we waited for that evaluation, pure panic simmered just below my skin. What if this situation is misjudged? What if she’s admitted involuntarily? How can I help her understand she can’t come home with me? I regretted calling for help, as calling for help now risked adding trauma onto an already untenable situation.
Jadyne also suffers from epilepsy, autism, swallowing and sleeping issues, and low muscle tone, among other things. Added to her aggressive episodes, these symptoms mean that she needs constant care and often medical intervention. If I feel uncomfortable contacting first responders or taking her to the nearest emergency room because of the way she may be treated, it puts Jadyne and our entire family at risk.
I realized that if it happened to us, it likely happened to other families in our community. That was something I could not live with – for Jadyne or anyone else.
The day after that ER experience, I started sending out emails that detailed the events and what alternative protocols might entail. I wasn’t sure how it would be received (or even if anyone would care to provide the issue attention). However, amazingly, one entity after another joined in on a work group to develop protocols that would better suit this population. Lauren Pearcy of the Tennessee Council on Developmental Disabilities and Michelle Bagby of the Department of Intellectual and Developmental Disabilities formulated a work group with ARC of TN, the Vanderbilt TRIAD Center, the National Center for START Services, and others. They met for a year and worked tirelessly to develop a single sheet document that was both digestible yet comprehensive, providing a list of dos and don’ts when stabilizing an individual with an intellectual or developmental disability.
I cried the first time I held in my hands a specific, tailored protocol with expert input put in place as a resource to help medical practitioners and first responders understand and respect patients like Jadyne.
The protocol contains advice both for what to do and what not to do when interacting with developmentally disabled patients–things that caregivers and families may instinctively know, but doctors who don’t see these types of patients on a regular basis may not know without further education. For example, something as simple as communicating with patients becomes more complex when they have a communication disorder––nonverbal patients might need something to write with, an American Sign Language (ASL) interpreter, or picture communication. It is so important to interact directly with the patient instead of talking negatively about them in their presence or failing to acknowledge them at all. This new guidance is invaluable.
On the other hand, it’s important that things to avoid are also noted; for example, assuming symptoms are part of an individual’s disability, or that the patient can provide consent, when this may not always be the case. There are also instructions for dealing with well-known sensory triggers such as flickering lights, loud noises and scratchy clothing. All of this helps keep patients with developmental challenges much more comfortable and gives them a sense of dignity. The guidance to check for underlying symptoms which may not be communicated could make the difference between someone being hospitalized unnecessarily for “behavior” versus being properly treated for an infection and able to happily return home.
I am so pleased that Vanderbilt hospital has adopted the protocol. Any step we can take towards making all people feel safe and respected during their most vulnerable times is a victory.
However, there is more work to be done.
The protocol is available online for any hospital or practitioner ready to educate themselves and learn how to deal with patients like Jadyne. It is my hope that hospitals throughout Tennessee, and eventually, the entire nation, will adopt this protocol. I believe we can all work together to ensure that the type of situation we found ourselves in – stuck at a hospital in an environment that was not best for Jadyne or her caregivers – does not happen to other families.