2019 Life with Adult EA/TEF can be unpredictable, dangerous, and life threating

A True account from an Adult living with being born unable to swallow

of her visit to a Hospital in the year of 2019

If you were not worried you might be after reading this account, we hope to show you that things need to improve, because there are a lot of Children that one day will become Adults

This is worrying for anyone growing older with this condition if the understanding for Adult care does not change fast

Life with Adult EA/TEF can be unpredictable, dangerous, and life-threating.

Everything can change in a flash without warning. I had a near-death experience with complications of adult EA/TEF a few weeks ago, in the middle of July.  The day started and ended like most other Saturdays.  My parents went to a wedding, my sister went to work, and I spent the afternoon working on a Capstone class for college.  Once everyone got home, we talked and we went to bed around midnight.  By 4:30 AM, I woke up feeling like I was drowning.  It was like running a marathon just to keep breathing.

I walked to the bathroom and tried to cough out what I thought was a phlegm ball from the back of my throat.   It didn’t work, so I gave up and went back to bed.  My original thought was since I can’t clear this phlegm ball right now; I will try to get some rest and ask to be taken back to my primary doctor or urgent care the next morning for more antibiotics and steroids like I have been doing every few weeks for the last six months. I didn’t even know I was going into respiratory failure.

Fortunately, my sister woke up because I had left every single light in the hallway on. She followed me to my bedroom and peeked into my room. I remember telling my sister to wake my parents up because I was really having trouble breathing and it was getting worse. Mom got me out of bed and took me to the spare room and to jiggle with CPT vest and use nebulizer treatments.  We thought it was a bronchospasm and the albuterol would help me breathe.

Unfortunately, that didn’t work so we drove twenty minutes north to the emergency room. The only thing I remember is laying down on the floor in our spare room at home telling my family I couldn’t breathe at all. I was not moving any air in my one lung.  Part of my TEF journey is that my chest anatomy is not normal.  I was born with only one lung and all the respiratory distress has left it rather twitchy at times.  I don’t remember the ride to the ER and being transferred to a trauma room.  The ER we went to said that I was too sick for their hospital to safely treat, so they called for a helicopter.

The transport crew got there and decided that it would be safer to drive to the bigger hospital about 35 miles away.   I barely remember the ride in the ambulance, but they had me on 100% oxygen and it was still exhausting to breathe.  I think I passed out in the ambulance.  The next thing I remember is waking up in the ICU on a vent. I don’t remember very much of the ICU stay either. I slept a lot in the ICU and they gave me meds that mess with your memory.

They sedated me

When I arrived at the hospital via ambulance. They sedated me and intubated me so I could stabilize. While I was sedated and vented, they put a J-tube up my nose so I could get nutrition while on the vent.  I had several chest X-rays that showed half of my one lung covered in a massive pneumonia.   The radiology person wrote in his report that I had both pneumonias and might have an obstructed esophagus. A culture of my trachea showed both signs of aspiration and streptococcus in my only lung. I had gone into septic shock which was resolved in the ICU.  The antibiotics used to kill the bacteria did not agree with my super-sensitive TEF gut and caused massive stooling and cramping for a few days.

When I was recovered enough to no longer be under the care of the ICU floor, I was transferred to the respiratory floor. I was placed on high flow oxygen. I was still unable to eat because my esophagus was full which was pressing on my one and only lung plus my trachea. The gastroenterology specialists were called in.  They ordered a CAT scan to see the esophagus and found a food bolus clumped in my esophagus. The length of my esophagus was half full of old chewed up food.  I got it cleaned out the next day. The tube feeding up my nose was removed because he believed the esophagus would not cause any more problems and I would be back to normal with my eating and swallowing.  The doctor asked why do you need a dilation and a cleanout of your esophagus you had a dilation with your primary GI doctor last month.  I started eating clears and liquids then I was transferred to inpatient rehab on July 29th.     That’s when the real problems and frustrations began.

This was my entry into rehab – 12 days after going into respiratory failure.

Inpatient rehab started me on pureed meals.  I was assigned a speech therapist to watch me swallow to make sure I didn’t aspirate again.  Because I have no problems swallowing and don’t aspirate as I swallow, the speech therapist advanced me to level three meals which is solid food.  On a Wednesday evening, I ordered pasta with chicken chunks mixed in red sauce. I ate the whole plate with lots of water.  A few hours later I felt a stickie in my esophagus so I notified the nurse. She dismissed my warning and told me to wait and see. By Thursday morning, I was having trouble breathing because the stickie was pressing on both my trachea and bronchial tubes this caused me to produce massive amounts of phlegm in my throat which I was unable to clear. I needed an oxygen tank to breathe and to do therapy.

My breathing became worse and oxygen needs more and more.

I told the rehab doctor on Thursday morning that something was stuck in my esophagus and I needed a GI consult to clear the stickie. He called in pulmonology to check me over because I was needing more oxygen to breathe.   I had been off oxygen 2 days before this stickie happened. The lung doctor asked for an x-ray and a culture of my phlegm. I got more antibiotics. I told the lung doctor that I needed a GI consult because something was stuck in my esophagus. He didn’t believe me and said: “wait and see”.  By Friday I couldn’t even go to therapy and had doubled my oxygen requirements from Thursday morning.

At that point

At that point, I became very afraid that nothing would be done and I would be left alone with a full esophagus.  I was afraid of choking in my sleep or aspirating again which would likely have killed me as my one lung was still recovering from the pneumonia. I was losing sleep over it. I cried a lot because my esophagus felt like a hand was shoved down my throat and squeezing it to death. I was very overwhelmed with the setbacks and the fact that nobody around me besides my family was listening to my complaint and trusted I knew my body and knew what had to be done to fix the situation.   I was refusing to eat or drink anything. I was offered pancakes for breakfast and more noodles for lunch and dinner. Anytime I was brought a tray of food I would turn the plate of food away and tell the hostess to send the plate back.  Friday night was the final straw. I was hurting really bad throughout the night and I told the nurse that my esophagus was full and that it hurt. At first, she told me it was just anxiety and to go to sleep. I told her I can’t go to sleep with a full esophagus. I told the nurse not to lay the hospital bed flat so I wouldn’t choke or aspirate in my sleep. She offered Tylenol for the pain. I told her Tylenol wouldn’t even began to touch the pain that the full esophagus is causing.

The rehab doctor finally listened to me and called in a consult for a GI doctor was called on Saturday morning. The GI doctor didn’t believe me right away because I had a dilation and my esophagus was cleaned out a week prior to entering rehab. He said you had your esophagus cleaned out last week there’s nothing there. I kept pushing to have the problem resolved. He ordered an esophagogram just to prove me wrong, it seems. I was very stressed by that point because I hadn’t been eating in days.  An esophagogram is done in radiology, with the technicians giving you barium to swallow and the radiologist turning on the fluoroscope to see the barium go from mouth to stomach.  This stuff was thick and hard to swallow. I was asked to lay down and drink and when I did that the barium all came back out of my mouth. I felt like I was suffocating so I asked to stop the test. I was sent back to my room.  The radiologist didn’t say he saw anything and I felt completely defeated and panicked that nothing would be done.

Later that evening, the GI doctor got a report from radiology and came back to the hospital to see me and my family. He admitted that I was right and he would remove the stickie right away. He had to book a room in the ICU because endoscopy was closed on the weekend and the stickie was considered a dire emergency because it was endangering my already weak lung.   Just before I was put under sedation, we talked about putting a GJ tube in because I was not able to eat enough orally safely to maintain a healthy weight.  I had already lost 15 pounds in the past year over esophagus issues, so I agreed it would be a good idea.

A few days after the stickie was removed, I went to interventional radiology (IR) and they put me under so they can place a fresh GJ tube. I got through the GJ tube placement it without any issues. The real issues started happening around midnight after my mother left for the night that night. The surgery site started to hurt a lot.  It felt like my whole stomach was on fire so I asked the night nurse for some ibuprofen.  She called the doctor, but he was refusing me any pain medication with the expectation of Tylenol.  I could not move or talk all night. I kept pressing the nurse button for pain relief, but she eventually stopped coming in. She sent her assistant instead. The assistant yelled at me for pressing the call button because I don’t really need her help.  I kept pressing the nurse button anyway, but that made the assistant mad.

I left the TV on all night to distract myself.  The morning nurse called the doctor in the morning and I got some pain relief through my PICC line and ibuprofen.  Both did wonders.  The frustrating thing is that the nurses had the wrong phone number for the IR on-call person, so had to rely on the hospitalist.   When the IR doctor came in to check, she said she would have given ibuprofen all through the night because it seems to work with new tube placements.

The next night I was having problems with feeding tolerance because the formula was new.   The nurse came in and said that it was normal to have some cramping and diarrhea at first and it would take some time to stop. I kept pushing for something to be done about it. She told me she would call the night doctor. I disconnected the feeding pump and threw the tubing on the floor while it was still running. I rolled over and went to sleep while she was calling the doctor which created quite a mess. We started over at a slow rate and increased the rate of overtime for a few days.

When we increased the rate, however, there were problems.  I knew which brand of tube-formula would work because I’d been on it before when was a kid, but that wasn’t offered at the hospital.  This was super frustrating because of the requirements to go home was to have adequate nutrition. Once home, we contacted my regular doctor and solved the problem by switching formulas.


I feel like I have gone back at least fifteen years in progress

I know it will really help keep me healthy, but I hate the GJ tube because I don’t like IR changes and I feel like I have gone back at least fifteen years in progress. I had a GJ tube for the first ten years of my life due to severe reflux and aspiration and now I am back in the same boat at twenty-two years old. This is beyond frustrating because I had been eating orally prior to crashing and nearly dying a few weeks ago. The reality is that one reason I crashed so hard is I’m not getting enough vitamins, calories, or protein from just eating.

Malnutrition makes pneumonia much worse.   Eating is hard, it’s no wonder I’m always underweight.

A little background as to why I hate IR changes. I was never sedated or given any pain medication at all when I was small.  The IR people deflated the balloon and yanked the tube out fast and hard. Then they shoved the new tube back in.  They didn’t sedate me because I only have one lung. They didn’t want to decrease my effort of breathing. One change in IR almost cost me my life when I held my breath too long and I turned blue while I was on the vent. Mom rushed back from the waiting room and turned the vent settings higher. The IR doctor didn’t allow my mother to be with me during the IR changes, but that changed when I started holding my breath so I wouldn’t scream. I asked for Ativan for the change to a low-profile button next week so I should be all set.

One thing that made my month-long stay in the hospital much harder than it needed to be was the fact that I had to fight constantly to be treated for complications of EA/TEF.  The doctors and nurses did not understand the condition very well.  This was the first time I had gone to the adult intensive care unit instead of the pediatric ICU.  It is understandable that the hospitalist and ICU doctors didn’t know quite what to make my reconstructed EA/TEF anatomy.   The bigger problem was that they did not listen to me when I explained what was wrong with me and how to fix it.  I had to fight for the basic level of care for EA/TEF.

Life with TEF as an adult can be very scary and overwhelming.  I almost died from the simple act of eating which is a daily activity that most people take for granted. I had to put my foot down and demand to be listened to when I have a TEF related problem as an adult. The fight is very stressful and avoidable if doctors and nurses listened to the patient. I am getting my story out there so people understand the challenges of hospital care with TEF as an adult.  Maybe it will help the next patient because the medical people will listen more.

Or help other adult TEF people know that they are not alone.

Written by Sarah Boury from the USA aged 22 in 2019 added to this website in August of the same year.