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.