Saturday, March 4, 2017

My Experience On Total Parenteral Nutrition (TPN) With Mast Cell Disease

(To preface: While we all have commonalities, every patient is different. I am simply stating my experience. It is always best to weigh the risks versus benefits with your doctor prior to beginning any treatment.) 

Hi, my name is Cheyanne and my mast cells do not know how to behave. If only admitting there is a problem led to a recovery in this case... These misbehaved cells are How I Received A Mast Cell Activation Syndrome Diagnosis to add to the ever growing list of Ehlers Danlos Syndrome comorbidities.


To add to my woes, my mast cells are in abundance throughout my gastrointestinal tract. They tend to get finicky. When they do, my body is awesome at inducing anaphylaxis and/or severe Mast Cell Disease reactions upon the ingestion of food, water, tube feedings, and oral/tube medications. This inevitably poses a huge problem from a nutritional standpoint. Whether it is Mast Cell Disease reactions or other complications from Ehlers Danlos Syndrome, like Gastroparesis, I intermittently need Total Parenteral Nutrition (TPN).
TPN is a form of intravenous (IV) nutrition by passing the entire gastrointestinal tract. All necessary nutritional components are fed directly into the blood stream through a central line. There are many resources to learn the basics of TPN. [x] [x]
Total Parenteral Nutrition (TPN) was initially a godsend. My Mast Cell Disease became severe in 2014 and major flares resulted in the use of TPN ranging from weeks to months. Food, tube feedings, and oral/tube medications were discontinued for the majority of the duration on TPN. Medications were switched to IV too because they induced anaphylaxis when severely flaring. The complete gut rest calmed the mast cells in my gastrointestinal tract enough to resume toleration of my usual safe foods, feeds, and medication regimen. The first time, I was on TPN for a total of three months. The second was about six weeks. The third was two weeks. This worked on each occasion that the flare was induced by a clear trigger. For example, a medication trial gone wrong or an immense diet mistake. After TPN and IV medications inpatient and home, I would get the line pulled and go about my merry way.

My relationship with TPN was not as smooth sailing in the downward spiral of mid 2015. It is where my experience with it takes a turn for the negative. Since then, I have resorted to TPN quite a few times. The complications I experienced undoubtedly stemmed from Mast Cell Disease. The manifestations were different each occasion.

In my past unsuccessful TPN trials, the reactions did not occur immediately. They gradually built up, taking a couple of weeks to reach the point of having to stop the TPN due to the severity of the reactions. 

Reaction + Nutritional Complications 

  • Swelling + Classic Allergy / Mast Cell
Early on in rejecting TPN, the reactions were classic allergic symptoms lasting the length of the infusion: flushing, itching, high blood pressure, tachycardia, nausea, diarrhea, and swelling. The swelling was located in my eyes, forehead, throat, hands, and feet. Symptoms peaked around Benadryl doses and decreased some with medications, but they did not dissipate completely until hours following the infusion. Symptoms were mild at first, increasing in severity the longer I was on TPN. I lasted a little over a month before it got out of hand with the swelling impacting my breathing and requiring Epinephrine.
  • Neurological
TPN also worsened neurological symptoms. They continued to progress, as my mast cells were provoked by the TPN. Tremors that were once isolated to full blown reactions became provoked by movement alone and muscle jerks, tics, and weakness accompanied them. With further triggers (i.e. perfumes, lotions, etc.), the neurological involvement caused stroke-like episodes. In addition, it frequently caused visual disturbances like flashing, colored lights, blurred vision, and floaters.

The newer symptoms caused from the TPN did not entirely disappear, as they were a result of near constant exposure to a mast cell trigger. The symptoms wax and wane with flares. Regardless, they are definitely more pronounced on TPN and most of the neurological symptoms were not evident prior to TPN serving as a trigger.
  • Kidney/Urinary Tract
The last occasion I was on TPN (February - March 2016) caused the mast cells to wreak havoc on my kidneys and urinary tract, taking two weeks to lead to one of my worst reactions to date. The beginnings were exacerbation of bladder cystitis. Later, I experienced severe back pain, urinating blood and what looked like black pepper flakes, swelling, neurological symptoms, cardiac involvement, amongst other unpleasantries.

I do not recall much from that night. I blacked out. The sole reason I was not sent to the ICU was because my doctor did not have rights on that particular floor. He had stated they would make matters worse with little understanding of Mast Cell Disease.
  • Rapid, Unexplained Weight Loss
One would assume weight loss would not be a concern when nutritional needs are met. Unfortunately, TPN triggers my mast cells, which then interfere with metabolism. My weight plummets, despite the pharmacist adding more calories into my TPN than she would give a grown man.
  • Potassium + Wacky Labs
A common manifestation of a body being in a reactive state is critically low potassium. The problem is very evident when on TPN. The pharmacist puts max doses into the formula, yet boluses are still needed.

Other labs go low too. The entire time I am on TPN my protein levels drop, along with triglycerides and vitamins.
  • Iron
TPN has all nutrients necessary for life, with the exception of iron. Eventually, iron will have to be received from another source. Most often, IV iron infusions are how this is done when on TPN and not using the digestive tract. IV iron lends allergy risks for the normal population.

With infusions, oral/tube iron supplements, and food, the no iron in TPN presents added complications for a Mast Cell Disease patient. When I have supplemented iron, the pharmacist reconstitutes Venofer, the IV iron, to an oral solution to put through my J tube. 

Despite these complications, there were (and are) still flares where I temporarily lose toleration of my J tube feedings. When they hit the threshold of becoming too dangerous, TPN is necessary. The interim between the slow building TPN reactions is usually enough to allow my body to decrease its severe response to the regular J tube feedings. 

Causes of Sensitivity/Allergy

It is difficult for those with Mast Cell Disease to tolerate the excipients, fillers, preservatives, and trace metals that are in the elements of the TPN. The culprit(s) of reactions can differ patient to patient, if there is an intolerance at all!

  • Fillers + Preservatives
It is not certain which portion of the TPN was causing the bulk of my reactions or if it was a combination. The pharmacist on my case did call the companies that produce the TPN ingredients to ensure they were preservative free. It is not always offered.
  • Aluminum
The aluminum in TPN is high. A theory is that the high aluminum content was contributing to my reactions.
  • Dextrose
Although strictly speculation, another theory is that it could be the dextrose content, as things tend to get bad once that is increased. Plus, I get the same symptoms from D5 and D10 infusions.
  • Lipids
Lipids are the fat in TPN. They can contain egg or soy ingredients. For those with true IgE allergies, they are to be avoided.

I do not have an IgE allergy to either ingredient. Since triggers for Mast Cell Disease do not have to stem from an IgE allergy though, I have trialled TPN without the lipids and had little to no reduction of symptoms. I have also done lipids only, but encountered significant swelling and had to stop. 

Again, these are just my experiences. Mast Cell Disease does not guarantee Total Parenteral Nutrition (TPN) will not be tolerated. I did well with it for years. Triggers vary. Specific triggers can cause different symptoms in a single patient. The disease changes constantly! Total Parenteral Nutrition (TPN) can be a wonderful, lifesaving option. And its use is unavoidable depending on the situation.