A Clogged Feeding Tube and the Start of a Marathon Admission to Massachuesetts General Hospital
My marathon admission to Massachusetts General Hospital began at the end of July of 2014. By the time I was admitted I was 68 pounds and in critical condition. The two months beforehand had been spent trying to convince people that I didn’t have a conversion disorder, and that something was really wrong with me. I had gone from ninety pounds to sixty-eight pounds because my feeding tube got clogged and the local hospital refused to insert a new one.
Dr. Trisch, my new GI doctor, had saved my life by insisting that my mom drive me to Massachusetts General Hospital’s ER to get evaluated.
The Eye of the Storm In the Trauma Room
When I got to the ER, triage immediately sent me to one of their high acuity rooms. A whole team of people descended on me. They began hooking me up to monitors, putting in ultrasound-guided IV lines, drawing blood, and getting EKGs. Other doctors and nurses were running pressure bags of IV fluids and replacing electrolytes. Some nurses were giving anti-nausea meds, blood pressure meds, steroids, and cardiac meds intravenously.
It was a flurry of activity around me, and I was the eye of the storm, lying limply in bed completely drained, but relieved that I was finally being taken care of.
Getting Stabilized Enough To Meet the Complex Care Team
Once I was considered to be in stable enough condition, I was introduced to the Complex Care team who would be following me at Mass General every single time I was admitted there, for the rest of my life. Then it was just my mom and I and a whole bunch of loud alarms going off all around us. The nurses would come in and out of the room every once in a while to switch a potassium replacement bag or adjust my oxygen levels.
Finally, I was moved to my room on the Intermediate care floor. Some hospitals call it a Stepdown Unit. It’s a floor for people who are too sick for the regular medical-surgical floors but don’t quite require the ICU.
Too Busy for a Pity Party
For the first two weeks of my admission, I had so many tests done that I had no time to feel sorry for myself.
Dr. Kuo who was a GI doctor who specialized in motility disorders. He did a test where I had to swallow barium and lie under a camera that took pictures of the inside of my digestive tract.
I took a couple of gulps of the thick, nasty-tasting, chalky white liquid and then lay there.
“The barium’s not going anywhere.” the tech told me. “Your esophagus is too slow. We’re going to need to repeat this test after you get your NJ feeding tube placed so we can actually get a picture of your digestive tract.”
Dr. Kuo explained to me that my esophagus was moving in contractile motions and not pumping the liquid through.
My Entire Digestive Tract is Delayed Now
“Your esophagus is severely delayed, not just your stomach. We’ll have to place that NJ tube as soon as possible to get a better picture of your insides. It’s no wonder you vomit so often. The food and drinks can’t go anywhere.”
The NJ feeding tube was placed in interventional radiology the following day. The insertion was easy as usual because I was knocked out for it, and I was so used to the feel of an NJ feeding tube, that they barely bothered me anymore.
Once the NJ feeding tube was placed I had the barium pushed through there to give Dr. Kuo a picture of what my digestive tract looked like and how it worked. It showed delay throughout my entire digestive tract, from my mouth to my rectum.
A Sitzmark Study and a Paralyzed Colon
Dr. Kuo also ordered a sitzmark study where I swallowed a pill full of coils and then they took x-rays every day for five days to track the movement of the coils through my body. It took the first four days to even get the coils out of my stomach and they were all still in my intestines by the end of the five days. More testing revealed that my intestine and colon peristalsis was close to paralyzed as well.
“You may not tolerate a surgical feeding tube very well because even though we’re bypassing your stomach, which is the most severely affected, your intestines and colon aren’t very functional” Dr. Kuo warned me.
“What’s the plan then?” I asked him.
“Well you have been tolerating the feeding tube formula at a rate of 5 ml an hour, but that’s not enough to live off of. We’ll just have to go super slow at increasing your feed rate. The goal is to get you to 50 ml an hour, but it may not be possible.” he explained.
The other specialists were in and out doing their own testing as well.
These Seizures are the Real Thing
Neurology did an hour-long EEG that came back mostly normal, but with enough “off” for them to want to look into it further, so then they did a 24 hour EKG and they confirmed that my seizures were real and not pseudoseizures like the local hospital had claimed. They said that I was primarily having complex partial seizures but they believed I had occasional grand mal ones as well. They agreed I had some form of dysautonomia. I was started on an anti-seizure medication called Keppra.
All the Specialists Did Their Own Testing Then Met Together
Urology agreed with Dr. Metta that the neurogenic bladder was due to dysautonomia. They told me that I would probably never be able to pee again.
Nephrology ordered labs and a work up and confirmed that I was in Stage Two kidney failure. On admission I was so dehydrated that it was affecting my kidney function. I was also in Stage Two Kidney Failure. They also said that my kidneys were having issues retaining potassium and certain other electrolytes, which was why I was always dropping my potassium levels.
Cardiology performed extensive cardiac testing and concluded that the A-fib was due to low potassium levels and that my heart itself was healthy except when I went into SVT. They said the rapid heart rate was a result of the dysautonomia and that we’d have to be really careful with beta-blockers because, although they lowered my heart rate, they also lowered my already too low blood pressure.
Adding in New Diagnoses, But Missing the Big Picture
Cardiology performed my tilt table test which demonstrated my heart going up 46 beats per minute when I was transitioned to an upright position, along with plummeting blood pressure. This confirmed a diagnosis of dysautonomia and that part of the dysautonomia diagnosis was a condition called POTS or postural orthostatic tachycardia syndrome. It was yet another symptom of some major underlying dysautonomia diagnosis according to cardiology.
Sporting the Green Pediatric Oxygen Tubing
Pulmonology did pulmonary function tests, chest x-rays, and a perfusion scan, and monitored my oxygen. They concluded that I had decreased lung volumes, a raised left diaphragm, atelectasis and scarring from atelectasis, and diaphragmatic weakness. They recommended that I wear oxygen at night as my levels tended to drop most while I was sleeping.
Fighting Against Uncooperative Vital Signs
Physical therapy came every day and did passive range of motion exercises so that I wouldn’t get contractures. They would usually get me up to sit on the edge of the bed, and be able to go no further because my heart rate would hit 200s and my blood pressure and oxygen levels would plummet.
Patricia and Dr. Thomas came to see me every day, ordering tons of urine and blood tests.
The First Hospital Team Meeting
After the first two weeks in the hospital, we had a meeting in my room with me, my parents, all of the specialists, Dr. Thomas and Patricia, the case manager, and my primary nurse.
“The gastroparesis is wreaking havoc in your body as we suspected,” Dr. Thomas told me and everyone else as well.
We all nodded in agreement.
“You are tolerating tube feed through your NJ feeding tube, just at a much lower rate than desired. Because we can only get a minimal amount via your digestive tract, we need to find another way to stabilize you nutritionally and treat the dehydration that is affecting your kidneys. The IVs that we have been placing in your superficial veins are using up your only viable veins.
“So what do we do” my mom asked.
My First PICC Line
“At this point, we feel that the most appropriate action is to place something called a PICC line in your upper arm for more long-term IV access.”
“What’s a PICC line?” my mom questioned.
“It stands for a peripherally inserted central catheter. It is a very long IV line that is inserted with local anesthesia into a vein in the upper arm and then threaded through the arm to just above the superior vena cava in the heart. We can administer medication, IV fluids, and perform blood draws through a PICC line, and it can stay in the body for months.” Explained Patricia.
PICC Line Concerns
“Will it hurt?” I asked.
“You’ll get enough lidocaine to numb your arm so that all you will feel is the injection of lidocaine and then just pressure as the line is inserted in the vein.”
“What are the risks associated with a PICC line? Why have they never placed one before?” my dad asked.
“There’s a high risk for life-threatening bloodstream infections with any type of central line including a PICC line. We put central lines in when all other forms of treatment have failed. Becca has no veins left for us to use, and due to the dysautonomia that is now officially confirmed based on the results of the Tilt Table Test, we know she needs large amounts of fluid for her body to function.” Patricia explained to my parents.
Resistance From My Parents
“Can’t she just drink more,” my dad asked.
“She can’t tolerate enough fluids by mouth and she can barely handle the tube feed alone, she wouldn’t tolerate water boluses into the NJ feeding tube on top of the tube feed that she already gets.” Dr. Thomas explained. “At this point, the possible benefits of a central line outweigh the risk.”
“Will this be permanent?” my mom asked.
“Hopefully not, we are hoping that we can get her to a point where she can either tolerate enough volume in her feeding tube to add in water boluses or can take in enough fluids by mouth.” Dr, Thomas answered. “We will continue to try to treat the dysautonomia as well as aggressively as possible to get her back in shape.”
Dysautonomia Treatment
“How do we treat the dysautonomia?” my mom asked.
“The biggest issue with dysautonomia is making sure she gets enough fluids in. We will be putting the PICC line in today and begin her on high rates of IV fluids with saline and electrolytes.”
“Will that help me feel less dizzy too?” I asked.
“Yes, definitely,” Dr. Thomas told me.
“We’re also going to have her start wearing compression clothing.” Patricia continued. “We’re going to start her on a medication called Midodrine to increase her blood pressure so that we can also simultaneously up her dose of Metoprolol to control the tachycardia (rapid heartbeat).”
“That will also make you less dizzy,” Dr. Thomas told me.
A Possible GJ Tube in My Future
“She will also work with OT and PT to get her up and out of bed. Hopefully, the new treatments improve the dysautonomia symptoms enough to get her moving safely. If we can get Becca to tolerate her NJ feeding tube more then we will discuss placing a GJ tube and using the J port part of the feeding tube as a more permanent way of getting her nutrition.” Patricia said.
Diseases I Don’t Have
“We have found a lot of diseases that Becca doesn’t have.” Dr. Thomas added. “She doesn’t have Crohn’s disease, ulcerative colitis, or celiac disease. She doesn’t have Porphyria or cancer of the GI tract; she doesn’t have multiple sclerosis, lupus, or Lyme disease.”
“Well, that’s good at least,” my mom said.
“Sort of,” agreed Dr. Thomas. “But it also means we still don’t know what’s wrong with her.”
“But now we know it’s nothing serious or life threatening.” My mom said.
“Not necessarily,” Dr. Thomas disagreed, there are a lot of dangerous rare diseases out there, we need to work fast to find out what’s wrong with her, if we can treat it, and if so, how.”
“Oh, oh, ok.” My mom looked flustered, she hadn’t been prepared for that response. She wasn’t aware that within the month she would find out that my life expectancy would go from living another 65 years or so, to living another ten years or so at the most.
Checking for Small Fiber Autonomic Polyneuropathy
“We’ve consulted with the Mayo clinic extensively about her case, and they have suggested that we perform a skin biopsy to check for Small Fiber Autonomic Polyneuropathy,” Patricia told us.
“What’s that?” asked my dad.
“It’s a complex neurological disorder that is a combination of bodywide neuroptahy and a non functioning autonomic nervous system. It can be degenerative, and it can be fatal.”
I saw my dad’s eyes twitching for a few minutes before he regained composure.
“The plan is to place the PICC line today and then arrange for one of Dr. Oaklander’s neurologists to perform the skin biopsy in the next few days. The results take a while to come back, so we will do this as soon as possible. The good news is that Dr. Oaklander is the Goddess of Small Fiber Polyneuropathy. She’s one of the researchers that discovered it. If anyone can help us it’s her. You’ve come to the right place.”
I just nodded. This was all so overwhelming, but we were exploring all different directions. Eventually, we would figure something out. We had to.