Navigating the Complexities of Ehlers-Danlos Syndrome and the Added Fear of Dystonic Storms (or any new and terrifying symptom)

Written by: Amanda Henderson 1-9-26

Note: I'm not a medical professional- this blog is intended to provide experience and information in hopes of helping others. It is not medical advice.

Living with multiple chronic conditions can feel like walking a tightrope without a safety net. My journey with Ehlers-Danlos Syndrome (EDS), Mast Cell Activation Syndrome (MCAS), and Dysautonomia (also known as the EDS trifecta) has taken me through a maze of symptoms and challenges. Along the way, I have faced complications like lymphedema, venous issues, and neurological problems. Recently, a new and more frightening condition entered my life: dystonic storms. This post shares my experience navigating these complex health issues, offering insights and practical advice for others facing similar battles.

Medical chart showing neurological and vascular condition notes

Understanding Ehlers-Danlos Syndrome and Its Impact

Ehlers-Danlos Syndrome is a group of connective tissue disorders that affect the entire body. For me, EDS has meant living with fragile veins, overwrought muscles due to weakened joints and tissues, GI issues, as well as motor control and mobility problems, which have led to nervous system issues, venous insufficiency, and lymphedema, among a myriad of other conditions. I spend more and more of my time at home due to the ever-worsening and overwhelming symptoms of my condition. Lately, though, I rarely leave the house.

Lymphedema and Venous Issues

Lymphedema is swelling caused by lymph fluid buildup, often in the limbs. In EDS, weakened connective tissue can impair lymphatic drainage, making swelling a persistent problem. Venous issues, such as varicose veins and chronic venous insufficiency, add to the discomfort and risk of complications like blood clots.

Managing these symptoms requires a combination of:

• Compression garments to support veins and reduce swelling

• Regular physical therapy to improve circulation and joint stability

• Pneumatic Pumps to help my legs and central body circulate lymph fluids

  
  

These strategies have helped me maintain some control over symptoms, but the unpredictable nature of EDS means flare-ups can happen without warning.

See Prior blog posts for more detailed information.

The Role of Mast Cell Activation Syndrome in My Health

Mast Cell Activation Syndrome complicates my condition further. MCAS causes mast cells to release excessive chemicals, triggering allergic-like reactions that can affect multiple organs. Symptoms range from hives and swelling to gastrointestinal distress that can be very difficult to manage.

How MCAS Interacts with EDS

The overlap between EDS and MCAS is significant. Both conditions involve connective tissue abnormalities and immune system dysregulation. For example, fragile blood vessels in EDS can worsen the swelling caused by mast cell activation. Additionally, MCAS flare-ups can trigger neurological symptoms, making it harder to distinguish between the two conditions.

Managing MCAS involves:

• Avoiding known triggers such as certain foods, temperature changes, and stress

• Using medications like antihistamines and mast cell stabilizers under medical supervision

  
  

This careful management is essential to reduce the frequency and severity of symptoms.

Dysautonomia and Its Daily Challenges

Dysautonomia refers to a group of disorders affecting the autonomic nervous system, which controls involuntary functions like heart rate, blood pressure, and digestion. For me, Dysautonomia means dealing with fatigue, unpredictable heart rate, and digestive issues that can disrupt daily life.

Coping with Dysautonomia Symptoms

Living with Dysautonomia requires lifestyle adjustments, including:

• Utilizing IV fluids to maintain blood pressure and heartrate

• Incorporating gentle exercise to improve cardiovascular health

  
  

Despite these measures, Dysautonomia symptoms can be unpredictable and exhausting.

Facing the New Threat of Dystonic Storms

Recently, I began having a new symptom which is best described as dystonic storms. They are a rare and severe neurological condition characterized by sudden, intense muscle contractions and spasms. These episodes can last several hours and cause extreme pain and disability. They are very violent, rack my entire body, make speaking impossible during the flareup, and even lowers all of my electrolyte levels and changes many of my blood panel levels. EDS is most likely a component of dystonia and dystonic storms, but how it is involved is still a source of debate. It been noted that EDS brings neurological issues of its own into the picture, or it could be that EDS causes craniocervical instability that creats bloodflow issues- a more mechanical problem that leads to neurological complications. This has certainly added another level of mystery to my list of diagnoses.

I have these storms twice a week, but the nervous system windup leading up to each one is constant. Generally, they begin in my large joints—especially my neck, SI joint, and hips—and radiate down my spine to my hips, knees, ankles, shoulders, elbows, and wrists. It's an unnerving feeling—like electrical misfires—as it creeps throughout my body. The closest I can describe it is the achy, zappy feeling you get with a bad case of the flu. Once whatever it is makes its rounds, it attacks, squeezes, and releases all of my muscles as it rolls me back and forth across the floor, lifting my legs and straightening my arms rhythmically. It is a pain so intense I cry out even as I'm unable to form words. My eyes will roll around, looking at everything and comprehending what's going on as I feel trapped inside and surrender to the pain. When it finally lets up at least an hour, sometimes several hours, later, I am weakened and completely spent. I add meds and electrolytes as I can and try to appease my system with as much water as I can tolerate. I think to myself, I survived one more, how many more to go?

During a storm muscles contract and move on their own- I have no control and it is very painful

Because I've suffered from these for a few months now, all of my major muscle groups are retaining the rigidity that these storms bring on even more than they had prior to them. I wear out quicker every day and have even less energy spending power. The last few times, they've ended in aphasic events that make me unable to speak for hours afterward, as noises issue from my open mouth rather than words—a sure sign of brain injury. I have to be supervised, and my husband has gotten really good at checking blood pressure, oxygen levels, and keeping me as warm and hydrated as possible.

I'm unable to do a lot of my usual routine because they can trigger these events. For reasons unknown to me yet, my saline IVs, leg compression pumps, inordinate temperatures, overactivity, even a regular shower can lead to a storm, depending on where I'm at in the nervous system dysfunction and overwhelm process. I'm constantly spent, exhausted, in more pain than ever before (which is saying a lot), and am having issues accessing my brain skills again, like finding words and remembering things accurately, just as I had with the aphasic event in 2016—then I lost reading comprehension, memory access (had to relearn how to do everything over and over again), and speaking ability—though I also couldn't comprehend what I was going through at the time until my brain healed and made new pathways years later.

The unpredictability of dystonic storms adds a new layer of injury to my health journey. I am doing my best to cope with these traumatizing events by:

• Working with my primary care doctor to develop an emergency plan

• Implementing relaxation and breathing techniques to try reduce stress triggers

• Keeping a symptom diary to identify potential warning signs

  
  

While dystonic storms are terrifying, proactive management and support have helped me regain some sense of control over them. Perhaps I'm also getting used to them since I've had so many in rather quick succession, as well.

Practical Tips for Managing Multiple Complex Conditions

Living with EDS, MCAS, Dysautonomia, and dystonic storms requires a holistic approach. Here are some practical tips that have made a difference:

• Build a trusted medical team: Specialists familiar with these conditions can provide coordinated care.

• Keep detailed health records: Tracking symptoms, medications, and triggers helps guide treatment.

• Prioritize self-care: Rest, nutrition, and stress management are crucial.

• Advocate for yourself: Be clear and persistent when communicating with healthcare providers and if you're incapable of that, ask someone who is knowledgable in your medical conditions to escort you.

• Connect with support groups: Sharing experiences with others facing similar challenges offers emotional support and practical advice.

  
  

The Importance of Mental Health Support

Chronic illness takes a toll on mental health. Anxiety, depression, and isolation are common and often occur out of necessity rather than what a person seeks to become in life. Counseling or therapy can provide coping strategies and emotional relief and are not a weak answer to an overwhelming issue- choosing such guidance requires strength and perseverance. Mindfulness practices and gentle physical activities can also improve well-being and engage the parasympathetic system for an overall health approach.

Looking Ahead with Hope and Preparedness

My journey with these complex conditions is ongoing. Each day brings new challenges, but also opportunities to learn and adapt. By staying informed, connected, and proactive, I continue to navigate this difficult path with resilience and heavy helping of spiritual guidance.

If you or someone you know faces similar health issues, remember that you are not alone. Seek support, stay curious about your health, and don't hesitate to ask for help. I've never been so vulnerable and in need of so much help in all my life. I am incapable of more tasks than I can count and have had more help than I could have thought possible. Instead of becoming completely defeated by this drastic change in lifestyle habits, I have chosen to see it as a learning experience—that needing help is a sign from the universe that we require connection and support along the way. It helps the giver as well as the receiver feel a part of something bigger than ourselves. While the routine I had built up over the years felt comfortable, sometimes stepping outside of those comfort zones can be a better answer than we realized for health and well-being. Just keep in mind that true help comes from the heart, and with that comes the compassion and capability that is capable of changing someone else's life for the better.

I'm sharing this part of my journey to highlight the ups and downs I've faced over the past twenty years in the medical world. It's been a ride full of answers, but even more questions at times, and never a lasting upward trend. The latest issues have been difficult to navigate and to talk about. It's not exactly a well-known condition that has answers and remedies readily available, which holds its own struggles, as well. Nevertheless, I hope that it can help others who have felt the same way, for whatever reason your life circumstances have been unpredictable. The only constant is change itself, and it really can take outside influence to help get you through the ups and downs that inevitably come and go, ebb and flow. Have great courage, my friends, and let's master this roller coaster ride together.