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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All About Feeding Tubes

We have finally reached the point where I need a feeding tube. I have been putting this day off for a very long time (nearly 2 years) despite several GI doctors telling me I would need a feeding tube if I could not manage at least 1700 calories everyday on my own. On a good day I consume about 700 calories and I don't have many good days (and I am still far from my goal intake).

Accepting that I need a feeding tube has been a process. It's a big deal. It's a huge change for me and I wanted to reach a place where I felt at peace with the decision. So I stalled and stalled and kept pretending like I would be able to find ways to consume enough calories to nourish my body. But....it wasn't happening. And I knew that. I am underweight. I have little to no energy. My body has been without steady nutrition for far too long. We have tried everything there is to try. It's time.

I wanted to write a post to explain feeding tubes in more detail and discuss some misconceptions with feeding tubes that exist so as to hopefully help people better understand.

There are different types of feeding tubes...
Types of Feeding Tubes 

• Nasal Feeding Tubes - NG, NJ, ND - tube enters the nose (N) and travels down to the stomach (G), jejunum (J) or duodenum (D). Feeds can be given through these tubes and they can also be used to decompress the stomach (NG). 
• Jejunum = small intestine 

• Abdominal tubes
• G-Tube - most common type of feeding tube. They are placed directly through the skin and into the stomach. Those who require tube feeding for more than 3 months are likely to receive a G-tube. A G-tube may consist of a long tube, sometimes called a PEG tube, or a skin-level button. 

  

Mic-Key button

• GJ-Tube - very similar to G-tubes, they enter the stomach directly through the skin using the same site or stoma as a G-tube. There are two ports, one into the stomach, and one port with an internal tube that extends into the small intestine. The G port allows for venting to release pressure from the stomach. The longer internal tube allows feeding directly into the small intestine (jejunum- "J") for those who cannot tolerate gastric (stomach) feeds (as is usually the case with gastroparesis)--feeds are given more slowly through the small intestine. This is the type of tube I have!

  

GJ Mic-Key button

• J-Tube - tubes that are directly placed into the small intestine. These are seen in patients with delayed stomach emptying as delivering feeds into the jejunum (small intestine) bypasses the stomach completely. They require surgical placement. Feedings must be given slowly. 

There are SO many reasons why people need feeding tubes and I couldn't possibly cover them all.

Reasons for Feeding Tubes
Here are some reasons:

• Upper airway disease or obstruction 
• Gastroparesis and delayed intestinal motility 
• Cystic fibrosis and other lung diseases
• Prematurity 
• Failure to thrive 
• Congenital heart defects and other heart conditions
• Metabolic disorders 
• Endocrine disorders
• Malformations/diseases of the gastrointestinal tract 
• Intestinal obstruction
• Neurological/neuromuscular impairment 
• Cancer and chemo
• Renal problems
• TBI (traumatic brain injuries)
• Dysphagia (difficulty swallowing)
• Coma patients 
• Special needs patients 
• Various autoimmune conditions 
• Post-op patients 
• Cleft palate 
• Severe reflux with aspiration 
• Genetic conditions 
• Dysautonomia/autonomic dysfunction 
• Connective tissue disorders (EDS)
• Craniofacial conditions 
• Nerve damage 

As you can see...feeding tubes are used for far more than just gastrointestinal/stomach problems. 

Many people have no experience with feeding tubes and there are many misconceptions about them...

Common Myths/Misconceptions about Feeding Tubes 
#1 When you have a feeding tube you can't eat
While it is possible that individuals with feeding tubes cannot consume anything by mouth, many people with feeding tubes are encouraged to eat as much by mouth (orally) as possible. So yes, even though I need a feeding tube to ensure that I am receiving adequate nutrition (as I am not able to consume enough on my own), I am allowed to/encouraged to eat food.

A feeding tube takes the pressure away from force feeding myself and dealing with the consequences (vomiting, abdominal pain, bloating, etc.). And yes, I do miss food and I do want to eat food even though I no longer feel hungry and struggle to tolerate it.

#2 Everyone with a nasal feeding tube has an eating disorder 
This is simply not true. Nasal feeding tubes are typically used for patients who require a feeding tube for less than 3 months (they can be used longer but the discussion of placing a surgical/abdominal tube then comes into play). They are also used as a trial step to ensure that the patient tolerates feeds.

#3 Feeding tubes are permanent 
While it is possible that someone may need a feeding tube permanently, some patients improve enough to where they no longer need the tube. Everyone is different. Their reasons for needing a tube are different and therefore, it's a decision made between the patient and healthcare team if/when the patient is ready to stop tube feeding.

#4 Feeding tubes are only given to people who are underweight 
There are so many reasons why people need feeding tubes and not all of them have to do with weight.

#5 Feeding tubes are an easy fix
Feeding tubes are not a simple or easy fix for most people. They are a lot of work. It is not as simple as placing a tube and giving yourself nutrition. Some people do not tolerate formula/feeds well, they can can cause pain, infection, skin breakdown, and a host of other issues. The body doesn’t always like having a foreign object lodged into it. It can be a lifesaving measure, but it is not easy or simple.

#6 Feeding tubes are a cure (all of your symptoms go away)
Feeding tubes are not a cure. They do not magically make all of your symptoms go away. Similar to the answer above, not everyone tolerates formula well and you can still experience nausea, vomiting, pain, bloating, etc.

#7 You don't feel hungry when you have a feeding tube
Some people may not feel hungry due to the nature of their medical condition, while others still do feel hungry. Again, everyone is different. This is not a one-size-fits-all tool.

#8 Feeding tubes restrict what you can do
No, not at all. Kids and adults can lead relatively normal and productive lives with feeding tubes. You can remain active in sports, you can swim, you can work, you can exercise, and travel with a tube! Note: this is in regards to a feeding tube not another medical condition that may prevent any of those activities.

#9 Healthcare providers know everything about feeding tubes
Unfortunately, not all healthcare providers have experience with feeding tubes and some are downright clueless. Parents and patients often know their tube best and figure out the tips and tricks to making life with a feeding tube easier.

#10 Feeding tubes are only used if you're dying or near the end of life 
Please refer to the list above about reasons why someone may need a feeding tube. Feeding tubes are tools that help people consume adequate nutrition when they are not able to themselves (for whatever reason). They can be temporary or more long-term but they are not only used for people who are dying or near the end of life.

If you have questions about feeding tubes I encourage you to ask me! 

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