EDS Awareness Month: Answering Your Questions! PART TWO

Because connective tissue can be found all throughout the body, there are a lot of secondary or associated conditions that are believed to be connected to EDS--Dysautonomia (POTS, orthostatic hypotension, neurocardiogenic syncope), Chiari Malformation, asthma, heart complications (Mitral Valve Prolapse), Mast Cell Activation Syndrome (MCAS), gastroparesis and other GI motility challenges---and the list continues.

 

Comorbidities

What other conditions do you have?

In addition to EDS, I have Chiari Malformation, craniocervical instability, neurocardiogenic syncope and POTS, bilateral acetabular hip dysplasia (corrected in a procedure called Periacetabular Osteotomy--PAO), gastroparesis and delayed intestinal motility, Hashimoto's, Graves', autoimmune neutropenia, and complex regional pain syndrome (CRPS).

 

POTS

What is POTS? 

Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia (autonomic dysfunction) and a form of orthostatic intolerance. Dysautonomia (autonomic dysfunction) is a term used to describe any disorder/disease/malfunction of the autonomic nervous system. It is associated with Ehlers Danlos Syndrome.

The autonomic nervous system controls functions that we do not consciously think about--blood pressure, heart rate, respiration rate, digestion, etc.

POTS is characterized by an increase in heart rate (tachycardia) of at least 30bpm or more or >120bpm within the first 10 minutes of standing in the absence of orthostatic hypotension.

This is a fantastic video that explains POTS in a simplified way, as disorders of the autonomic nervous system are incredibly complex.

 

Can you explain what its like to live with POTS? How does it affect you?

POTS is exhausting and unpredictable. Living with POTS is like running a marathon that never ends. My body has to work 3x harder when I am upright as compared to a healthy individual. It leaves my body with very little reserve. It can feel like a never-ending flu. I feel dizzy, lightheaded, weak, shaky, nauseous, and painfully exhausted every single day. I have good days and bad days, but symptoms are always present and can hit at unexpected/unpredictable times.

What is the treatment for POTS? How do you treat your POTS?

POTS can be very difficult to treat/manage. There is no cure, so the goal is to alleviate/manage symptoms. Every patient is different. Treatments for POTS include increasing fluid intake, increasing salt consumption, wearing compression stockings, raising the head of the bed, and graduated exercise. If non-pharmacological methods are not enough, there are a number of different medications that people take to decrease heart rate and/or increase blood pressure. 

I went through the standard process of treatment. I started with all of the methods listed above; however, I reached a point where I struggled to get out of bed without fainting and medications were not effective enough. Due to low blood volume, I receive IV fluid 3x/week through a port-a-cath (central line). Though it is not a cure and I still experience symptoms, IV fluid has been the single most effective treatment for me. The use of a central line and IV hydration should be the LAST RESORT as there are risks involved.

Gastroparesis

What is gastroparesis?

Gastroparesis (GP) = delayed stomach emptying. Delayed stomach emptying leads to a backup in the digestive tract and symptoms of nausea, vomiting, bloating, feeling of fullness after just a few bites, abdominal pain, belching, lack of appetite, weight loss and malnutrition.

Literally translated, gastroparesis means "stomach paralysis."

 

Can you eat?

Yes, I can eat. However, I do not tolerate enough food orally to sustain myself from a nutritional and caloric standpoint. As a result, I have a feeding tube. To learn more about feeding tubes click here. 

How is it treated?

Everyone is different. Treatment varies from diet changes to medications to feeding tubes to surgical intervention and gastric pacing. 

Chiari and CCI

What is Chiari Malformation? 

Chiari Malformation is a neurological disorder where part of the brain (the cerebellar tonsils) herniates or descends out of the skull into the spinal canal. This results in compression of the spinal cord and brain stem. As a result, this compression disrupts the normal flow of cerebrospinal fluid (CSF) leading to disabling neurological symptoms (headaches, occipital neuralgia, numbness and tingling in hands and feet, weakness, nausea/vomiting, dizziness, low blood pressure, and many more).

How were you diagnosed with Chiari?

In 2010, I experienced a CSF leak from an epidural I had placed for hip surgery. This resulted in the most debilitating post-dural puncture headaches. Despite returning to the operating room to have a blood patch to resolve the CSF leak, my headaches never went away. Eventually, a brain MRI confirmed an 8.5mm herniation. 

Symptoms of Chiari Malformation are much more than just headaches

Symptoms of Chiari Malformation are much more than just headaches

Is there a treatment for Chiari Malformation? Is there a cure?

There is no cure. Treatment focuses on alleviating symptoms. This may include medication and/or surgery to relieve the compression on the brainstem and spinal cord. I underwent a brain surgery (posterior fossa decompression) in 2014. 

To learn more about my journey with Chiari Malformation you can watch my video:

What is CCI? 

CCI stands for craniocervical instability. It is a structural instability of the craniocervical junction, or where the skull and spine meet. This is often seen in conjunction with Chiari Malformation in patients with Ehlers-Danlos Syndrome. Treatment includes the use of a cervical collar, physical therapy (by an EDS knowledgable PT), or a craniocervical fusion. 

 

Concerns about family members

Diet tips for people with POTS?

With POTS it is often common and recommended to increase fluid and salt intake. This helps boost blood volume because where salt goes, water follows. As I am not a physician and cannot offer medical advice, any concerns about dietary changes or the use of multivitamins should be discussed with your doctor.

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Flares with Chronic Illness

Different problems flare in different ways.

A flare with EDS typically involves an increase in pain (and instability) in specific joints. For example, a bad dislocation of my shoulder can lead to severe pain (and bruising) for days and the need to immobilize for a period of time to allow the shoulder (and all of the supporting tissue) to calm down. The same applies to all of my joints.

When my neck "flares" I am typically dealing with several cervical rotations that lead to unpleasant neurological symptoms. Fortunately, we are pretty good at figuring out the cause of the problem based on the specific set of symptoms I am experiencing. To manage I receive manual therapy weekly to work through the cervical rotations and muscle tension. I  also wear a cervical collar when the neck pain and instability are unmanageable (I try to avoid the cervical collar as much as possible as my neck muscles tend to “forget” to hold my head up after wearing the collar for extended periods of time).

A flare for POTS usually involves several days or weeks with a lower blood pressure than my normal baseline, tachycardia at rest and with standing, difficulty tolerating being upright for more than a few minutes, more pre-syncopal and syncopal episodes, and usually the need to increase IV fluids and blood pressure medications for a period of time. A POTS flare can be caused by a number of different factors--dehydration, heat intolerance, too much exertion (such as with travel), illness, cervical rotations (a flare in my neck can lead to a flare in POTS---the body is connected).

A flare with MCAS involves allergic reactions (primarily rashes) to unknown allergens/triggers more regularly. Sometimes a MCAS flare leads to an asthma flare (as I have allergic asthma). I wear a mask for precautionary reasons and always in the presence of grass (as that triggers an asthma attack almost instantly).

CRPS flares often bring the most panic because I have no medication options anymore with the exception of Ketamine. I try my best to avoid tiggers and until recently CRPS had been in remission. Unfortunately, my body decided to react to the hardware in my leg and that has led to a flare of CRPS. With a flare, I get severe pain in my legs. They feel like they are on fire and even the breeze blowing against my legs is excruciatingly painful. Hopefully, having just removed the hardware, this particular flare will subside and I’ll return to baseline shortly. In the meantime I will work on desensitization techniques by using different textures to rub on the problematic area(a).

For GI issues (gastroparesis), I am not sure what a flare is at this point as I haven’t tolerated food in a very long time. If not tolerating food is a flare then I have been stuck in this flare for 2 years.

Flares are frustrating but inevitable. Sometimes they occur with no known trigger or reason. Other times they occur as a result of pushing yourself. Often times, multiple problems are flaring at the same time. It is exhausting and just adds to my baseline fatigue. But you can’t stop living your life just to avoid the repercussions. It’s important to find a balance.

It is important to remember that there are many faces to chronic illness and everyone experiences flares and symptoms differently. 

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My "Normal" with EDS

I often hear comments such as "get well soon," "that must be rough to have all of that in the summer," and "hope your recovery goes smoothly" from people when I am out somewhere. One time a lady even approached my mom to tell her she was thankful I survived whatever it was that happened to me.

I realize their intentions are good, but the funny thing is....the braces, the crutches/wheelchair, the mask, the port...that's part of my normal...everyday.


So what is my normal really like?

This is a difficult question (at least lately) because my “normal” or baseline changes weekly.

For me, my “normal” involves multiple joint dislocations everyday (most notably my kneecap, shoulder, ribs, and right hip--but this too can change on any given day), an average pain score of 6/10, daily headaches and neurological symptoms from craniocervical instability, allergic reactions to God only knows what, random POTS episodes with pre-syncope and/or syncope, difficulty tolerating food, weekly IV infusions for blood pressure and heart rate, and unrelenting fatigue/exhaustion.

Everyday I wear a knee brace to hopefully decrease the number of dislocations and subluxations I experience (it still doesn’t stay in place). I wear an ankle brace (or walking boot when I break my foot) to prevent my ankle from rolling out (inversion). I sometimes wear a hip brace to limit extension and hopefully prevent my hip from sliding out the front. With bad shoulder dislocations I often need to spend a few days in a pillow sling. When headaches and neuro symptoms (from neck instability) are really bad I use a cervical collar until my neck can be assessed by my physical therapist.

I use either crutches or a wheelchair everyday due to pain, fatigue, joint instability, and POTS.

My week consists of physical therapy 2-3x and multiple appointments. Unfortunately I am unable to work at this time but hope to contribute in a meaningful way one day.

Life with EDS is frustrating and unpredictable. But with every curveball I just try to keep swinging.

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