Journey to a New Life: A Nutrition Support Case Study
By Theresa A. Fessler, MS, RD, CNSD
Today’s Dietitian
Vol. 11 No. 1 P. 54

Hospitalized with a bowel obstruction and later enduring severe complications, Hank’s future appeared bleak. Would his care team’s efforts and his own strength of spirit be enough to restore his health?

Hank’s* story is one of the most memorable of my career. He was admitted to the hospital in May of one year with a bowel obstruction due to an ileal stricture. I provided his nutrition care for 13 months, during which time we encountered significant complications, including short-bowel syndrome, enterocutaneous fistulae, and parenteral nutrition-associated liver disease (PNALD). Some of the healthcare team members thought he would not survive—but he surprised us.

Background: Enterocutaneous Fistula
An enterocutaneous fistula is an abnormal tract between the bowel and the outside of the body from which intestinal fluid drains. Estimates indicate that 75% to 85% of enterocutaneous fistulae occur after surgery and 15% to 25% form spontaneously due to underlying disease or injury.1 In their review published in the British Journal of Surgery in 2006, Lloyd, Gabe, and Windsor stated that over the past 30 years, mortality rates of 5% to 41%—and more recently, 6% to 33%—have been reported, with sepsis as the leading cause. Spontaneous fistula closure rates of 19% to 92% have been reported, and closure can occur within four to six weeks. Spontaneous fistula closure is unlikely to occur after two months, and in some cases surgical intervention is required. Complications of enterocutaneous fistulae include sepsis, malnutrition, dehydration, electrolyte imbalances, pain, infection, skin breakdown around the fistula site, anxiety, and poor body image.1,2

Parenteral nutrition (PN) has traditionally been the most common form of nutrition support for patients with an enterocutaneous fistula. It is more often needed for high-output fistulae or those originating in the pancreas, jejunum, or ileum. Enteral nutrition (EN) is preferred over PN and can be done proximal or distal to some enterocutaneous fistulae, depending on the location. EN and oral diet can be used in patients who have enterocutaneous fistulae with low output, for fistulae located in the distal ileum or colon, and in situations in which a feeding tube can be advanced distal to a proximally located fistula. Fistuloclysis is the insertion of a feeding tube directly into an enterocutaneous fistula. This method is used for EN when a fistula is not expected to close on its own.3

Intestinal fluids contain water, electrolytes, protein, and other nutrients in varying amounts, depending on the location in the gastrointestinal tract. These losses are controlled with medications (see Table 1) and can be replaced in several ways, such as through intravenous fluids, oral intake, EN, and PN. Enterocutaneous fistula output can also be reinfused into another enterocutaneous fistula by fistuloclysis or into a jejunal feeding tube.1-3

Initial Hospitalization
At the age of 81, Hank was admitted with nausea, poor oral intake, and abdominal distention. His weight was only 130 pounds, a loss from his usual weight of 145 pounds (height 5 feet, 4 inches). His medical history included unconfirmed Crohn’s disease, deep vein thrombosis, and hypertension. After being diagnosed with bowel obstruction, he underwent surgery, which included a partial colectomy with removal of 15 centimeters of terminal ileum, ileocolostomy, a diverting loop ileostomy, and drainage of abdominal ascites.  Afterwards, the abdominal wound became infected and dehisced. In June, Hank had another surgery for wound debridement and repair, small-bowel resection with a jejunal anastomosis, and repair of a ventral hernia.

At the time of his discharge, Hank had an open abdominal wound and four enterocutaneous fistulae that were draining approximately 250 milliliters per eight-hour shift. Nurses adhered an ostomy bag to his abdomen to collect the fluid. Hank was nil per os (npo) except for ice chips and sips of water. We had adjusted his PN to a 12-hour nocturnal cycle a few days prior to discharge. PN provided 1,800 calories from 265 grams of dextrose, 100 grams of amino acids, 250 milliliters of 20% lipid, and 1.8-liter volume, with standard vitamins, minerals, and trace elements and additional sodium, chloride, and acetate. Jejunal and ileal fluid contains approximately 100 to 120 milliequivalents of sodium, 10 milliequivalents of potassium, 50 to 70 milliequivalents of bicarbonate, and 50 to 60 milliequivalents of chloride per liter.2 Our knowledge of these amounts, as well as the patient’s blood levels, helped us determine what and how much to add to his PN solution.

Home PN
Hank returned home in June, relying on a home nurse for difficult tasks such as changing the ostomy bags and caring for the wound and fistula sites. The pharmacists and I called him and monitored comprehensive metabolic panel lab tests weekly to track his nutritional and clinical progress. Hank and his wife estimated fistula output at 500 milliliters per day, but they needed the nurse’s assistance with measuring the quantity.

After two weeks, Hank’s wife reported that he weighed 120 pounds, 10 pounds less than his hospital weight. We increased the dextrose content of the PN to 325 grams so that it provided a total of 2,005 calories per day. Over the next two weeks, Hank complained of dry mouth and thirst, his urine output was only 600 milliliters per day, and the ratio of blood urea nitrogen (BUN) to creatinine (Cr) had increased. Recognizing these signs of dehydration, we increased the PN volume to 2.6 liters per day. We also added extra zinc (for a total of 10 milligrams per day), as zinc is lost via gastrointestinal drainage, increasing the risk of deficiency.4

Hank’s urine output soon improved to more than 1 liter per day, and his weight stabilized. After several more weeks, the home nurse reported that Hank had jaundice and developed a fever of 102˚F, and his wife said that his eyes appeared yellowish and he was spending much time sleeping. Lab reports showed elevated alkaline phosphatase (Alk Phos), aspartate aminotransferase (AST), alanine aminotransferase (ALT), and total bilirubin (t bili), which raised concern for liver dysfunction. A liver biopsy result was consistent with hepatic cholestasis.  Since manganese and copper are excreted primarily via the biliary route, both can reach toxic levels in severe cholestasis. We omitted manganese and decreased copper to only 0.3 milligrams from the standard 1 milligram per day.4

Second Hospitalization
In late August, Hank was admitted to the hospital with diagnoses of PNALD and fungemia. His t bili level was 8.1. Doctors ordered the removal of Hank’s central line and discontinued PN, treated the fungemia with fluconazole, and started ursodeoxycholic acid for the cholestasis.

Elevations of serum AST, ALT, Alk Phos, and t bili can occur as early as two weeks into PN use. PNALD is still poorly understood. Etiologies include overfeeding of PN dextrose and/or lipid; continuous, rather than cyclic, PN infusion; sepsis; small-bowel bacterial overgrowth; poor bile flow due to a lack of enteral stimulation; deficiency of choline and possibly carnitine, nutrients not normally supplied in PN; and possibly the significant phytosterol content of soybean oil-based lipid emulsions. In severe, prolonged PN-associated cholestasis, liver failure and death can occur.5

The medical team held PN for six weeks, during which time we employed various other methods to nourish Hank. We added multivitamins and trace elements to his intravenous fluids, which consisted of 5% dextrose with half normal saline.  Because of his symptoms of short-bowel syndrome, short transit time, and high fistula output, we used short-bowel diet guidelines to help maximize his nutrient absorption.6 Hank ate small amounts of a low-sugar diet and drank oral rehydration solution and a pediatric electrolyte drink made more palatable by adding a small amount of a sugar-free flavored drink mix. Enterocutaneous fistula output fluctuated from 1,100 to 2,400 milliliters per day and worsened with increased oral intake.

The healthcare team initiated EN via fistuloclysis at 10 to 20 milliliters per hour with a goal of 45 milliliters per hour via a proximal jejunal fistula to supplement Hank’s oral intake. We chose an elemental formula because of the likelihood of poor digestion due to the lack of pancreatic and biliary secretions with feeding directly into the midjejunum. Fistuloclysis can be effective for EN if there is sufficient bowel distal to the feeding site; however, in this case, it resulted in increased distal fistula output.3 We then tried the nasogastric route to use the bowel proximal to the fistulae. Despite these efforts, as we advanced the EN rate, the patient’s fistula output increased to 3 liters per day. Ileostomy output stopped, as the intestinal fluid drained from the fistulae proximal to it. A fluoroscopic small-bowel follow-through test revealed a 15-minute transit time from the stomach to a midjejunal fistula.

The team discontinued EN, nourishing Hank only by oral diet, oral rehydration solution, and intravenous fluids. He became progressively weaker, and his weight declined to 105 pounds. I remember noticing the weakness in his voice during that time. Some thought he would pass away from either starvation, sepsis, or liver failure (if we resumed PN). Out of options, we resumed PN in mid-October at less than 1,000 total calories and 70 grams of protein per day with minimal intravenous lipid (less than 0.5 grams per kilogram of body weight), and Hank was discharged.

Second Period of Home PN
At his clinic visit two weeks later, Hank walked slowly, smiled broadly, yet was very thin, weighing 110 pounds, with a leg bag full of greenish-brown fluid connected to the fistula drainage appliance. With continued goals of weight gain and healing, we increased home PN to 1,230 calories and further increased it to 1,430 calories one week later.

Hank still had a long way to go. Continued fistula drainage precluded oral intake. PNALD limited our ability to safely increase PN calories. Fortunately, the low-calorie PN maintained his weight and strength, and the abdominal wound healed remarkably well. Soon, he would be ready for surgery to repair his bowel. We all hoped that eventually he would be able to eat normally again.

Third Hospitalization
Hank underwent surgery in December, which included jejunal resections, closure of the ileostomy, stricturoplasty, reduction of a small-bowel hernia, another ventral hernia repair with mesh, and closure of all four enterocutaneous fistulae. We used PN throughout his hospital stay and advanced it to 1,470 calories, 80 grams of protein, and 1.9 liters per day. By early January, Hank was discharged.

Third Period of Home PN and Weaning to Oral Diet
Hank’s postoperative course was very difficult, as a new ileal fistula had formed and was draining about 300 milliliters per day. The surgeon initially allowed clear liquids for comfort, but to allow the fistula to heal, he then called for more limited oral intake. For nearly four months, Hank was allowed only small sips of water and ice chips, and he occasionally asked when he would be able to eat. The liver function tests AST, ALT, and Alk Phos declined to near-normal levels. Fistula drainage decreased to as little as 25 milliliters per day as the weeks progressed. Fistula output was significantly diminished after an injection of Sandostatin (octreotide) LAR-depot, a long-acting somatostatin analog.

In mid-January, Hank weighed 100 pounds in the clinic. We increased the dextrose in the PN for a total of 1,720 PN calories per day, hoping he would gain weight. But during the following weeks, Alk Phos, ALT, and AST again rose above normal, and once more we had to decrease calories. We also added 100 milligrams of L-carnitine per day. Carnitine has not been proven beneficial in adults with PNALD, but it was reasonable to use, as patients dependent on PN are at risk for deficiency.5,7 In April, serum selenium was below normal, copper levels were normal, and whole blood manganese was elevated. We increased selenium to 80 micrograms per day and omitted manganese.

In early May, the surgeon approved the advancement of oral intake with short-bowel diet precautions. An estimated 190 centimeters of small bowel and most of the colon remained, enough for full rehabilitation.6 Hank started with a diet low in simple sugars and advanced gradually to a nonrestricted diet. His progress was slow. He had little appetite, likely because he had not eaten for so long. During weekly phone calls, we discussed his intake and food choices. As oral intake improved, the ileal fistula output increased to about 50 to 100 milliliters per day. This amount was acceptable; according to his surgeon, the remaining fistula was now functioning similarly to a diverting ileostomy. Hank described his bowel movements as normal and regular.

When I asked how he was feeling, Hank typically replied, “Could be better, could be worse.” Besides answering my clinical questions, he would tell me about his favorite foods, some of his activities, and, most encouragingly, his weight gain. He also told a good joke at the end of nearly every conversation.

Hank gained 1 pound per week for a three-week period; after that, his weight increased more quickly. By the time he reached 110 pounds in early July, we decreased the PN macronutrients but kept fluid volume the same because of persistent elevation in his BUN/Cr ratio and inadequate oral fluid intake. After two more weeks, to further wean, we changed Hank’s PN regimen to every other night. His oral fluid intake improved, his weight increased to 114 pounds, and after that week, we finally discontinued PN.

Because of Hank’s age and surgical history, I recommended vitamin B12 monitoring, although patients with terminal ileal resections of less than 20 centimeters have been found not to be at greater risk for B12 deficiency.8 His vitamin B12 level was within normal range three months after we discontinued PN.

Positive Outcome
Today, Hank is active, enjoying life, and taking vacations with his wife and their friends. At 110 pounds, he still struggles to improve his weight, and one very–low-output ileal fistula remains.

Working concurrently in the areas of acute care, outpatient clinic, and home health, RDs have the opportunity to provide nutrition plans and follow patients’ progress for months, even years. I’ll never forget Hank and the long journey our team took with him, all that we learned, his tenacity and positive outlook, and the trust he had in his healthcare team. Most of all, I will never forget his enduring smile.

— Theresa A. Fessler, MS, RD, CNSD, is a nutrition support specialist at the University of Virginia health system in Charlottesville and a freelance writer.

* Name is fictitious.

The Patient’s Perspective
The night of my entry to the emergency room, I stopped breathing, and the staff started me breathing again. On my way to my room after being admitted to the hospital, I was introduced to Dr. Morgan.* I told him that I was different from other patients, but I heal very quickly. He said, “Good!” During my first consultation with him, the doctor explained what was wrong and the operation necessary to correct the problem. I said, “Good, go for it.”

Every morning, the good doctor and a battery of medical persons would visit me and ask questions. I would ask, “What kinds of experiments are you going to perform now?” The doctor would smile and say, “None.”

My inspiring nutritionist helped keep me alive while I was a patient there for what seemed like a million years. She would come into my room with that sunshine smile and greet me by saying, “Good morning (or afternoon), Mr. Burrows.* You are looking better today.” She would ask how I was feeling while taking a seat at the same time, as though she were a visitor. This would make me feel as though she truly cared about my welfare. She made me feel at ease and relaxed, which helped me more easily refresh my memory. She would also explain why she was recommending each new food or drink.

I also thought very highly of my nurse coordinator, Emily.* Any time I needed a question answered, she would answer it or say, “I don’t quite know the answer, but I will get it for you and call you back.” Emily was instrumental in my decision to have my last operation, which the doctor said would be very dangerous and I would have a 50-50 chance to live. Another physician informed me that it would be too dangerous, and he would not recommend this surgery for his parents.

I did not want to be unable to care for myself and leave that responsibility to my wife. I decided to talk to Emily, who told me that if Dr. Morgan said he would do it, it would most likely be successful. This was all the encouragement I needed. I called the doctor and informed him that God is not through with me, so I’m ready to have the surgery whenever he would be ready. As you can see, a dead person has never written a line.

After the successful surgery, Dr. Morgan spent much time at my bedside tracking my progress. I thank my excellent doctor for seven operations and five procedures. Thanks to him and his magnificent staff for a new life.

* Names are fictitious.

Table 1
Some Medications Used During Hospitalization and at Home1,2,5,6,9
• Lansoprazole: Proton pump inhibitor to reduce acid and volume of gastric secretions

• Loperamide hydrochloride, diphenoxylate hydrochloride and atropine sulfate, codeine, tincture of opium: Gut-slowing, antidiarrheal drugs

• Ursodeoxycholic acid (Ursodiol): A form of bile acid to improve biliary flow and biochemical markers of cholestasis

• Octreotide injection: Somatostatin analog to decrease gastrointestinal secretions and motility

References
1, Lloyd DAJ, Gabe SM, Windsor ACJ. Nutrition and management of enterocutaneous fistula. Brit J Surg. 2006;93(9):1045-1055.

2. Willcutts K, Scarano K, Eddins CW. Ostomies and fistulas: a collaborative approach. Pract Gastroenterol. 2005;29(11):63-79.

3. Teubner A, Morrison K, Ravishankar HR, et al. Fistuloclycis can successfully replace parenteral feeding in the nutritional support of patients with enterocutaneous fistula. Brit J Surg. 2004;91(5):625-631.

4. Fessler T. Trace element monitoring and therapy for adult patients receiving long-term total parenteral nutrition. Pract Gastroenterol. 2005;29(3):44-65.

5. Kumpf VJ. Parenteral nutrition-associated liver disease in adult and pediatric patients. Nutr Clin Pract. 2006;21(3):279-290.

6 Matarese LE, O’Keefe SJ, Kandil HM, ey al. Short bowel syndrome: clinical guidelines for nutrition management. Nutr Clin Pract. 2005;20(5):493-502.

7. Shatsky F, Borum P. Should carnitine be added to parenteral nutrition solutions? Nutr Clin Pract. 2000;15(3):152-154.

8. Duerksen DR, Fallows G, Bernstein CN. Vitamin B12 malabsorption in patients with limited ileal resection. Nutrition. 2006;22(11-12):1210-1213.

9. Kandil HM, O’Keefe SJD. Medications: Antidiarrheals, H2 blockers, proton pump inhibitors, and antisecretory therapy. In: Materese LE, Steiger E, Seidner DL, eds. Intestinal Failure and Rehabilitation. A Clinical Guide. New York: CRC Press; 2005.