After much debate among my medical team, we decided to go forward with the placement of a GJ feeding tube. The thought was that this would relieve some of my headache pain, pressure in my face/sinuses, ear ringing, blurry vision, and esophageal pain. My GI doctor explained that an NJ tube was a short-turn fix, but it was time to move on to more of a long-term solution. It made the most sense to have the surgery done by interventional radiology since it is the least invasive option.
The surgery itself went very well. My anesthesiologist had me wear my cervical collar during the procedure to prevent any neck manipulation from the intubation. She also used a small, flexible fluoroscope to intubate me in order to minimize the risk of aggravating my TMJ. I wanted to avoid general anesthesia, however, due to my severe acid reflux, my team thought it best to proceed using general anesthesia to prevent aspiration.
The only thing that went differently than planned was that they used four t-fasteners rather than three. The post-surgery pain was surprisingly better than I imagined it would be. Since I can not take opioids, I was on a ketamine drip, and my anesthesiologist did a quadratus lumborum nerve block to help numb my abdomen. The nerve block was definitely a lifesaver! The most painful part for me was that a couple of times a minute, my tube would suction in and pop out slightly. Later, I found out that this was my intestines trying to digest the new tube, which sounds a bit disgusting, but apparently is normal.
Later that night, I developed a low-grade fever and severe rash on my abdomen; body aches and hypersensitivity were much more painful than the GJ site. I freaked out! My doctors were stumped as to why I was having this reaction. They drew a lot of labs and everything seemed relatively normal. I wondered if I was getting an infection or having an allergic reaction to the new tubing. In regards to my health, sometimes my mind jumps to the worst-case scenario, but in fairness, it seems like the worst-case scenario often occurs with me.
Gratefully, when I woke up the next morning, the rash had disappeared and my temperature was normal. Body aches were still my worst symptoms, and moving (anything) was very painful. I felt very heavy; it was even difficult to pick up my arms. Also, coughing, laughing, crying, and sneezing were excruciating to my GJ site. About 24 hours after surgery, I started shaking. My temperature dropped a little. I was freezing, lost color, and my body felt like it was slowing down. My parents were a bit frantic and quizzed the doctors on their opinions of what could be happening. I remember my mom getting really frustrated and her asking to review all medications and quantities given post-surgery. As the nurse read through this, my mom quickly realized that my medical team had forgotten to give me my antihistamines, which was likely resulting in a mast cell activation syndrome (MCAS) flare. The antihistamines were given immediately and my body slowly regulated and within about 15 minutes I stopped shaking.
Unfortunately, the drama never ceases. Two days post-op, I started to develop breathing issues. Although my oxygen SATs were mostly normal, each breath I took was extremely shallow, and there was pronounced wheezing. Tests were run; a chest x-ray, numerous blood tests, and I was even checked for a pulmonary embolism. Each test came back normal, yet I continued to struggle to breathe. The staff seemed perplexed. The wheezing increased my vertigo, light-headedness, and migraine leaving me incredibly fatigued. Talking became difficult as I needed to reserve all of my energy for breathing. It felt like I was choking while trying to gasp for air underwater. I was given Albuterol breathing treatments and O2, but nothing helped.
The doctors kept giving me Ativan because they thought the root of my respiratory issues was anxiety, however they eventually realized that this must not be the case. It frustrates me so much to have my symptoms brushed off or dismissed as anxiety!
That night, the hospitalist gave me an Ambien to help me fall asleep. However, my mom was petrified that I would stop breathing in my sleep, so she stayed up all night listening to me wheeze and made sure I didn’t stop breathing. My dad was out of town that evening because he and my grandfather had taken my youngest brother to a national tennis tournament in Greensboro, but when my mom shared what was happening, they withdrew him from the tournament and came to the hospital immediately.
The next day was like deja vu; they gave me more Ativan, put me on oxygen, and ran more blood tests. No one had a solution that helped give me relief. But late that night, my night nurse and parents began brainstorming about how I might be having an allergic reaction. My solution-oriented nurse took the time to think outside the box. She thought I might benefit from something like an epi-Pen. She called the respiratory therapy team, and around midnight that evening, I was given epinephrine through a nebulizer.
At the start of the treatment, I began coughing and gagging on mucus in my throat. The respiratory therapist looked at me nervously, but my dad and I smiled because I hadn’t been able to cough previously. After a few minutes, I could breathe normally! I cried tears of joy. I was beginning to think that these breathing issues were yet another symptom that I would have to deal with chronically, and I wasn’t sure I could survive that very long. In my head, I was trying to think of how I could live day to day with labored breathing. How would I talk to ask for meds or a bedpan? Despite what the pulse-ox was saying, was I really getting enough oxygen to my organs, my brain? Would the hospital even discharge me like this? If so, would I have to sleep with monitors? What would we do if I completely stopped breathing at home and there was no response team to help? How would I improve in physical therapy if all of my energy was focused on my next breath? It was such a relief to know that we had a solution. If my breathing ever became compromised again, epinephrine could help me. I am so grateful that my parents and this smart nurse kept fighting for answers!
Since we had decided that I was suffering from an allergic reaction, we scaled back to me only taking essential at-home medications along with phosphorus and potassium chloride. Evidently, I needed the phosphorus and potassium chloride because my medical team thought I was beginning to go into Refeeding Syndrome where one’s electrolytes become severely off-balanced. Yet, shortly after my medications were administered, I began having difficulty breathing. The wheezing and gasping for air went on for about two hours before the rapid response team was called, and more nebulized epinephrine was administered. Once again, I could breathe clearly after the five-minute treatment. Once I was breathing better, the ENT team came into my room to assess me, and they scoped me bedside. The laryngoscopy showed that my airway and vocal cords were working properly. We were not surprised since I was breathing perfectly. Even though I was in a very fragile place medically, all I wanted was to be discharged and leave the hospital.
My mom was emphatic that I only be given one medication at a time so we could isolate what was causing this allergic-like reaction. The resident decided that potassium chloride was the most important. A few hours later, potassium was pushed through my IV again, and the respiratory issues came back with a vengeance. However, this time the resident covering my case did not want to give me the epinephrine. He felt that I just needed to “calm down, lower anxiety, and take a few deep breaths.” My mom was furious, confidently articulating multiple flaws in that thinking. She is at her best when there is conflict. They debated for what seemed like an eternity, and finally, the doctor saw it her way, and the respiratory team was called.
Before I was given the nebulized epinephrine, the ENT scoped me again. This time, it was a lot more uncomfortable since I felt like I was choking and could not breathe. The laryngoscope showed that I was experiencing paradoxical vocal cord movement. Then, I was given epinephrine. It was scary to realize that if my mom hadn’t been there to advocate for me, I would not have been given the treatment and would have suffered longer unnecessarily. It is upsetting to think that there are patients who don’t have someone to advocate for them. This is critical for patients like me to survive.
Ultimately, there was not a consensus concerning my respiratory issues. However, we were connected with a knowledgeable allergist who specializes in Mast Cell Activation Syndrome (MCAS) in EDS patients. She believed I was experiencing paradoxical vocal cord movement and difficulty breathing due to an allergic reaction to the way the IV potassium chloride was administered. This reasoning made the most sense to me because the reactions coincided with the IV potassium chloride dosings. Some of the local doctors did not agree, yet had no alternative explanation for me. I think it’s easy to disagree, but that stance loses credibility in my mind when that critic has no plausible reason to offer in exchange. It’s scary when doctors don’t agree because the patient, especially when the patient is a child, loses confidence in their ability to treat successfully.
My parents thought it would be smart to take an Epi-Pen home with us just in case I had another breathing episode. However, despite my history, the hospitalist on call refused to write the script since she couldn’t understand the science behind why the epinephrine would help. My dad was so disappointed in the doctor’s response. My mom whispered to me not to worry, that we had lots of competent prescribers that would write for it and we would have it in 24 hours. She was right.
Now that my respiratory issues were gone, it was time to go home. However, during the week that I had been in the hospital, we had never received any training on how to use, clean, or operate the new tube. No one had really cleaned, or taken the initial bandage off my GJ site. The doctors passed this job off to the nurse, the nurse passed it to the dietician, and the dietician passed it back to the interventional radiologist team. When I had my NJ tube placed, I went home not really knowing much about this tube coming out of my stomach…I didn’t even know I had to flush it. Unfortunately, this and running the rate too high caused some damage to my intestines. Upset we had no written GJ instructions, my dad said to the hospitalist, “When one purchases a car, they get a user’s manual, yet when one has a hole put in their stomach with a tube they are supposed to use for food and water there is nothing written given to them. What is wrong with this picture?!” It was a herculean effort to find a dietician and nurse that felt comfortable teaching me how to operate and clean the tube. Even then, their instructions were not aligned, which was a little confusing. I honestly got more help from friends I’ve met online with feeding tubes.
Finally, I was discharged and welcomed home by my grandparents and siblings! The GJ tube was certainly the right move for me. After removing the NJ, the ringing in my ears became less frequent, my sinus/head pressure decreased, and my esophageal pain improved drastically. Although pushing things through my tube is still painful, it is definitely better now that it is not running through my esophagus. Although it was a stressful and scary week, I am grateful that the surgery went well and had no complications.