The survey says... chyle

We are back from a fast trip to Boston two weeks ago.  Tests seem to point that the fluid leaking out is chyle, and that it is limited to the fistula versus the entire bowel. We tested Peter with a 12 hour fast, which was easily accomplished overnight.  Since he is still on TPN 7 nights a week, it was even easier.  Then we collected his ileostomy and fistula outputs for testing.  After he woke up, we gave Peter 3 ounces of whole milk with olive oil in it, waited an hour and a half, and collected both outputs.  Then we gave him the same drink a second time, waited an hour and a half, and collected both outputs again.  The crazy part of the second collection is that due to the timing of collection, it needed to be done in a public restroom and then placed in a box with ice alongside of the other specimens, all the while trying to remain clean about the process, and quick, as we had already checked in to the waiting area to see the doctor.

The fistula output turned very cloudy after the fatty meals, suggesting chyle, and the fistula electrolytes suggest chyle.  The ileostomy had a lot of undigested sugars, which may be due to the fatty meal or due to his condition of short gut, or could be pancreatic insufficiency, so we are now testing the pancreatic enzymes in his ileostomy outputs.  Some questions arise as to why the chyle in the fistula is not high in protein, and why the ileostomy triglycerides were elevated after a fatty diet, but Peter's tests usually lend to results that need interpretation versus "going by the book."  So, with much discussion and interpretation, the impression is that it is a chyle leak in the intestine that is connected to the fistula, but that the rest of the GI tract seems to not have a chyle leak.  It is good news that the chyle leak seems to be isolated to the fistula.


Further good news of all of this is that it looks like surgery will be the answer to stopping the chyle leak.  No one wants to take Peter off all feeds for 4 to 6 weeks to attempt to have the leak close on its own, as his liver is already taxed on the amount of TPN he receives.  Feeding the gut helps to keep the liver happy, even if the feeds are small in amount.  We will actually try to increase feeds slowly, keeping to low fat, and then in several months hopefully surgically remove the fistula and the chylous intestine that is attached to it.  Details of course need to be hammered out and this plan is only in the beginnings of talk, but Peter did so well with the last surgery that it may be feasible to get rid of the fistula altogether despite the concerns that have come up about doing so in the past.  Our earlier talks for the next surgery were for a take-down of the ileostomy, so I don't know yet if the fistula surgery would take precedence over that or if they would be combined surgeries. 

A chyle leak

The predominant thought is that Peter is leaking chyle out his fistula.  Per the information at http://www.medicine.virginia.edu/clinical/departments/medicine/divisions/digestive-health/nutrition-support-team/nutrition-articles/practicalgastro-may04.pdf, "Chyle is an alkaline, milky, odorless fluid that provides about 200 kcal/liter. It contains greater than 30 g/L of protein, 4–40 g/L of lipid (mostly triglyceride) and cells consisting primarily of lymphocytes (1). Chyle leaks are a rare complication; they can occur for a variety of reasons after injury to the intra-abdominal lymphatics." 

Usually chyle leaks go into the abdominal cavity or thoracic cavity, so we are fortunate that this is not the case.  It can be a complication post surgery, but there is some question if Peter has had this for a long time, and it was masked by the leakage of food out of the fistula.   The usual treatment is conservative, and begins with a low-to-no-fat diet to decrease the amount of lymph (chyle) flow.  Peter switched to a formula called Tolerex for the last ten days, but it does not seem to be helping.  The fistula started going up in volume prior to the switch to Tolerex, but rather than going down or holding steady, it has continued to climb to the point he is now putting out 850 mL of drainage from the fistula per day.  Along with the ileostomy increase that we did see correlate with the change to the new formula, Dr. Kamin suggested that we just stop the commercial formulas altogether for a week to see if that helps.  Peter can eat and drink no fat, low sugar drinks and foods still, but we will eliminate the formula to see if maybe Peter responds better to a real-foods approach.  Although it can be difficult to get enough calories to get off TPN without a commercial formula, the idea for now would be to see how well Peter's intestines respond to this type of diet and then go from there.

The next step if this doesn't help is possibly to eliminate all food/drink for awhile, and/or to begin some more invasive procedures to try to look inside and isolate where the leak is coming from.  We may also be collecting fluids to determine with more certainty that it is a chyle leak versus some other complication.  The fistula collection two weeks ago highly suggests chyle leak, but a collection of the ileostomy may help see if the whole intestine is affected, and a new collection of the fistula may help to see if there has been a change in the content make-up.  A biopsy may also be considered to see if there is a food allergy that is causing these symptoms.  Dr. Kamin will take this week to consult with Drs. Jennings and Fishman again to see what tests and approaches seem best to determine what is going on and how to manage it. 

"Quality of life" is a phrase that gets used from time to time with Peter, and it is difficult to reconcile that he really does walk a line with his health when I see him climbing the equipment at the playground, playing and laughing with his siblings, learning the things that a five-year-old learns.   Really, the term "quality of life" isn't a term that is used in any other situation than one where someone has to weigh medical treatments and conditions with the enjoyment of life itself.  As usual, Peter acts just fine, despite the craziness of the things going on inside him.  He remains our miracle child.  Thank you to all who continue to pray for him!