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Writer's pictureKirstin Maltby

Ehlers-Danlos Tubies Stories

Lily is a tubie with Ehlers-Danlos who has graciously shared her story with a variety of feeding tubes and TPN during Feeding Tube Awareness Week (5-11 February 2023)

 

Prior to committing to the invasive placement of a surgical feeding tube for intestinal dysmotility I trialed nasojejunal (NJ) feeding to ensure my intestine could tolerate it. Unfortunately, because of my anatomy, NJ tubes were incredibly difficult to place and constantly flipped back into my stomach. After six weeks with an NJ tube, I had surgery to place a percutaneous endoscopic gastrojejunostomy (PEG-J). PEG-J tubes are typically quite large and bulky, so a patient may prefer to switch to a lower profile gastrostomy-jejunostomy (GJ) tube known as a Mic-Key button. Typically, a mature stoma (tract opening into the stomach) is required before a GJ tube can be placed. In my personal case, my stoma was mature enough to proceed with a Mic-Key GJ button two months after the original operation.



Lily a young Eurasian girl in her early 20s with brow hair and eyes poses in front of a mirror in a bra and track pants, revealing all the medical devices that keep her alive on a daily basis
From left to right in descending order these are the medical devices that keep me alive and their purpose: 1: Freestyle libre blood sugar monitor (this is a small cannula that is placed subcutaneously into the skin to constantly monitor blood sugar levels) 2: Port-A-Cath (this is a form of a central venous catheter that provides access directly into the superior vena cava) 3: PEG percutaneous endoscopic gastrostomy tube (a tube which is placed directly through the abdominal wall into the stomach to drain gastric contents) 4: PEJ percutaneous endoscopic jejunostomy tube (a tube which is placed directly through the abdominal wall into the jejunum to allow direct access to the jejunum for feeding) 5: SPC suprapubic catheter (a tube which is placed directly through the abdominal wall into the bladder to drain urine)

Unfortunately, because of the nature of GJ tubes, the jejunal portion of the tube can flip back into the stomach. This has occurred to me on multiple occasions and typically requires radiological intervention to fix.


Ultimately, because of complications, I ended up having my G and J tube separated to prevent this from occurring. Although this allowed me to tolerate jejunal feeds better for a while, disease progression lead to the inability for me to tolerate jejunal feeds as my intestinal dysmotility worsened and progressed to intestinal failure.


In order to combat my malnutrition and inability to tolerate feeds, we had to administer nutrition intravenously directly into my bloodstream, thus bypassing my defective gastrointestinal system. This was done by providing me with a special nutritional cocktail known as total parenteral nutrition or TPN.


TPN is an incredibly risky intervention, which is reserved when all other means of administering nutrition enterally fail. This is called intestinal failure. Because of the sugars and lipids (fat) in TPN, it’s a prime breeding ground for bacteria. As a result, serious bacterial infections can move quickly as they are “flushed” directly into the bloodstream, resulting in severe infections such as bacteremia (bacteria in the blood), which can cause serious complications like sepsis.


Unfortunate in my case, TPN was unavoidable, however, having adequate nutrition restored my ability to perform basic cognitive functions such as being able to hold a conversation. Despite all the risks involved with TPN, I am forever grateful for it providing me with a source of adequate, reliable nutrition.


A selfie of Lily - a young lady with dark hair and black glasses showing her two medical central lines surgically implanted in her upper chest.
Central lines for TPN

Typically they don’t like to use ports for TPN so I first had picc lines but due to my tiny vascular they were too difficult to place. I then had a tunneled picc line in my chest but my TPN wanted a reliable long term line that could be repaired so I had a Hickman placed

I am incredibly thankful for my amazing TPN team as they have literally saved my life and provided me with the energy to keep fighting the challenges that come with Ehlers-Danlos.


Lily a young girl with dark hair standing in a hospital bathroom in blue PJs taking a selfie beside and IV pole with a bag of nutritional liquids.
Me with my “feeds” that go through my tubes to keep me nourished and alive
 

Many thanks to Lily for sharing her stories and pictures to help others learn. We are always greatful for stories and if you have your own story you wish to publish contact us here.


For more general info on Feeding Tubes please check out our previous post here.


Feeding Tube Awareness week is run by AusEE Inc. to help raise awareness for feeding tubes in Australia. Go checkout their website here.

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This is not a medical site and all information should not be considered as medical advice. Great care is taken to curate this information but has not been checked by a medical professional. 

Updated July 2024

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