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!