My Journey to Finding the Right Feeding Tube With Rare Disease

When I was first put on nasojejunal (NJ) tube feedings* by the gastroenterology (GI) team at my local hospital in 2016, I was too weak to object. I had just been diagnosed as failure to thrive and having gastroparesis. Even though I was 19, my parents had signed the consent because I was so out of it. Throughout 2016, 2017, and the majority of 2018, I spent a significant amount of time on a ventilator for everything from influenza, to sepsis, to pneumonia. Eventually, I was diagnosed with a one-in-a-million neuroautoimmune disease, pediatric-onset stiff person syndrome (pSPS), in 2019.

It took me over seven years to get diagnosed with this rare disease. Before this, I had been told my problems with my twisting, spasming limbs, spine and organs were “all in my head,” anxiety, phobia, psychogenic illness, conversion disorder, or a functional neurological disorder (FND). The problems that had plagued me from 2012, when I was 16, had doctors in denial! In their minds, I was “just another hysterical female.”

Despite my dozens of diagnoses and the constant need for a feeding tube, I am so much more than a medically complex individual, and you and your loved one are, too! I am an avid outdoorsman who enjoys fishing and camping, a third degree black belt working toward my fourth degree, a nine-time ATA Martial Arts Taekwondo World Champion, and skier. I am a college graduate, a Masters of Social Work student, blogger, rare disease researcher, and advocate, as well as founder and president of the second nonprofit to raise awareness and funds for stiff person syndrome research in the United States, Meg’s Miracles.

As you might have already guessed, I only have one speed: breakneck! Others tell me this almost daily as I break “speed limits” in stores and while crossing campus in my bright orange wheelchair, affectionately known as “Nemo.” I never mind the joke, though, because I do go fast! Way faster than most manual and even powerchair users I know.

Of course, my wheelchair is a big part of my life, so I’ll tell you what “Nemo” does for me. For the last five years, I have been unable to move my legs below my knees voluntarily; my feet, ankles and toes are stuck in a fixed dislocated contracture that spasm non-stop, even during sleep or under general anesthesia. Contrary to popular belief, however, I am not “bound” to Nemo. He is my sidekick, and we take on and see the world together. It might be weird to personify a wheelchair, but it puts children and adults alike at ease. It normalizes medical devices most people wouldn’t consider “normal.”

As just about anyone with a rare disease or who cares for someone with a rare disease knows, you may have periods of stability, but that roller coaster ride never ends. I thought I had stability until one day in 2018. On November 7, to be exact, my jaw locked up, my limbs contorted, my eyelids spasmed shut, and it looked like I was having a massive seizure during an infusion that should have been helping me. I wasn’t seizing this time, thankfully, but the doctors were gaining valuable clues on my mysterious condition. Local doctors decided to fly me to a larger hospital. I ended up in the intensive care unit (ICU) again for 32 days and had another three NJ tubes placed, all with incredible difficulty. This time, the GI team approached me and suggested I have a surgical tube placed. They thought a gastrostomy-jejunostomy (G-J) would be a better solution than placing and replacing NJ tubes regularly. I declined both GI doctors thinking they were thinking the same thing only because they worked together.

After being released from the hospital, my mom, cousin and I moved to Baltimore, Maryland, to temporarily be closer to my specialist at Johns Hopkins. Not long after arriving there, my NJ tube clogged…as they sometimes do. We went to the emergency department (ED), and they opted to pull the tube since it was hopelessly blocked. The GI team consulted on me in the ED’s unique “ICU Holding Room” and, once again, it was suggested I get a GJ tube.

The interventional radiology (IR) department didn’t have any space available to place an emergent NJ tube, so the ED placed a massive 22Fr. nasogastric (NG) tube that was meant to pump someone’s stomach. I never really minded tube placements, but that tube was giant and hung out of my nose like a two-foot coiled elephant trunk. The worst part was, I couldn’t even get my tube feeds! That tube was so heavy, it ended up falling out.

About a half hour after the “elephant tube” was placed, I told my mom I wanted to have a G-J placed. To my surprise, she was entirely on board. Since then, I haven’t looked back! I think it was the last step to accepting that my body needed the help of another tool to manage the pediatric-onset stiff person syndrome. I learned that my pylorus at the bottom of my stomach is significantly impacted by the pSPS, which impairs emptying.

*Editor’s Note: There is reference to several different kinds of feeding tubes in this article: NJ (nasojejunal) tubes are placed through the nose and pass through the digestive tract to end in the second part of the small intestine (the jejunum); tube feeding administered through an NJ or J (jejunostomy) tube bypasses the stomach, which is important for many people on tube feeding for various reasons. A J tube can be placed directly into the jejunum surgically (surgical-J) or with the help of an endoscope (PEJ). A G-J (gastrostomy-jejunostomy) is placed in the stomach with the tip of the tube ending in the intestines (jejunum); typically the “G” or gastric part can be used for venting the stomach, and the “J” or jejunal part for tube feeding. An NG (nasogastric) tube is placed through the nose and ends in the stomach.

This article was originally published on the author’s website, Meg’s Miracles