C. Richard Fleming

Current use and clinical outcome of home parenteral and enteral nutrition therapies in the United States

BACKGROUND & AIMS Home nutrition support, especially when delivered parenterally, is very costly. The aim of this study is to examine current usage of home parenteral and enteral nutrition (HPEN) in the United States and the quality of therapy outcome. METHODS Medicare HPEN use from 1989 to 1992 was analyzed to assess use, growth, and costs. National Registry information collected on 9288 patients treated with HPEN from 1985 to 1992 was used to assess disease distribution and therapy outcome. RESULTS In the United States, there were approximately 40,000 parenteral and 152,000 enteral home patients in 1992. The usage of HPEN doubled between 1989 and 1992, and a large proportion was in patients with short survival. The prevalence of HPEN in the United States was 4-10 times higher than in other Western countries. Outcome data showed both therapies were relatively safe. The primary disease strongly influenced survival and rehabilitation, and age, per se, was not a reason to deny HPEN. CONCLUSIONS Predicted quality survival at home for several months, rather than a specific diagnosis, seems to be the soundest justification for HPEN. Its role in terminal conditions and patients without primary gastrointestinal diseases needs further evaluations.

Survival of Home Parenteral Nutrition-Treated Patients: 20 Years of Experience at the Mayo Clinic

OBJECTIVE To present the largest single institutional review of demographics, associated primary diseases, and survival of patients receiving home parenteral nutrition (HPN). MATERIAL AND METHODS We conducted a retrospective review of medical records of all Mayo Clinic patients treated with HPN between 1975 and 1995. The probability of survival was calculated by using Kaplan-Meier analysis. RESULTS In the 225 study patients requiring HPN (median age, 51 years), the main underlying primary diseases were as follows: inflammatory bowel disease (IBD) (N = 50), nonterminal active cancer (N = 39), ischemic bowel (N = 35), radiation enteritis (N = 32), motility disorder (chronic pseudo-obstruction) (N = 26), and adhesive intestinal obstruction (N = 18). Other conditions included intestinal and pancreatic fistula, refractory sprue, dumping syndrome, and protein-losing enteropathy. The overall probability of 5-year survival during HPN was 60%. The probability of survival at 5 years based on the primary disease was 92% for IBD, 60% for ischemic bowel, 54% for radiation enteritis, 48% for motility disorder, and 38% for cancer. The probability of 5-year survival stratified by age at initiation of HPN was as follows: younger than 40 years, 80%; 40 through 60 years, 62%; and older than 60 years, 30%. Most deaths during therapy with HPN were attributable to the primary disease. Among the 20 patients who died of an HPN-related cause, 11 deaths were from catheter sepsis, 4 from liver failure, 2 from venous thrombosis, and 2 from metabolic abnormalities. CONCLUSION Survival of HPN-treated patients is best predicted on the basis of the primary disease and the age at initiation of HPN. Patients with IBD and age younger than 40 years have a better 5-year survival in comparison with other groups. Most deaths during treatment with HPN are a result of the primary disease; HPN-related deaths are uncommon.

Selenium Deficiency and Fatal Cardiomyopathy in a Patient on Home Parenteral Nutrition

An adult patient with chronic idiopathic intestinal pseudo-obstruction maintained on home parenteral nutrition for 6 consecutive years died from cardiomyopathy and ventricular fibrillation. Postmortem examination of the heart revealed widespread myocytolysis and replacement fibrosis similar to that seen in the selenium deficient cardiomyopathy in China (Keshan disease) and animal models. Selenium deficiency in this patient was documented with extremely low concentrations of selenium and decreased activity of the selenoprotein, glutathione peroxidase, in blood, heart, liver, and skeletal muscle. Reports of selenium deficient diets causing myocardial damage in humans and animals and the findings in our patient strongly suggest that his fatal cardiomyopathy was caused by selenium deficiency.

Four Years of North American Registry Home Parenteral Nutrition Outcome Data and Their Implications for Patient Management

The OASIS Registry started annual collection of longitudinal data on patients on home parenteral nutrition (HPN) in 1984. This report describes outcome profiles on 1594 HPN patients in seven disease categories. Analysis showed clinical outcome was principally a reflection of the underlying diagnosis. Patients with Crohns disease, ischemic bowel disease, motility disorders, radiation enteritis, and congenital bowel dysfunction all had a fairly long-term clinical outcome, whereas those with active cancer and acquired immunodeficiency syndrome (AIDS) had a short-term outcome. The long-term group had a 3-year survival rate of 65 to 80%, they averaged 2.6 complications requiring hospitalization per year, and 49% experienced complete rehabilitation. The short-term group had a mean survival of 6 months; they averaged 4.6 complications per year and about 15% experienced complete rehabilitation. The registry data also indicated HPN was used for 19,700 patients in 1987 with therapy growth averaging about 8% per year. This growth was chiefly from new cancer patients. The number of new patients with long-term disorders in whom HPN was initiated appeared rather constant. We conclude that these clinical outcome assessments justify HPN for long-term patients, but the utility and appropriateness of HPN for the cancer and AIDS patients remains uncertain and requires further study. Medical, social, and fiscal aspects of HPN management in long-term and short-term patients appear to involve quite separate considerations.

Intestinal Pseudo-Obstruction as the Presenting Manifestation of Small-Cell Carcinoma of the Lung: A Paraneoplastic Neuropathy of the Gastrointestinal Tract

A 58-year-old woman who had presented with intestinal pseudo-obstruction died 9 months later from rapidly progressive neurologic symptoms and autonomic insufficiency. Her gastric emptying had been markedly delayed and transit of markers had been slowed throughout the small bowel. A 5-hour manometric recording of the antrum and duodenum had shown absence of the normal interdigestive motor complex, which was replaced by irregular contractile activity of reduced amplitude. A small-cell carcinoma of the lung was found at autopsy. Pathologic study of the gut showed widespread degeneration of the myenteric plexus, which was infiltrated by plasma cells and lymphocytes and contained significantly reduced numbers of neurons. The extra-intestinal nervous system had neuronal loss and lymphocytic infiltrates in dorsal root ganglia. Thus, a gastrointestinal neuropathy causing intestinal pseudo-obstruction may be the presenting manifestation of a paraneoplastic syndrome associated with small-cell carcinoma.

Zinc-Induced Copper Deficiency

Copper deficiency was found in an adult patient who had received excessive daily oral zinc for 10 mo. The deficiency was characterized by hypochromic-microcytic anemia, leukopenia, and neutropenia. Although initially thought to be caused by iron deficiency, the anemia did not respond to oral or intravenous iron. Cessation of zinc tablets and ingestion of an oral copper preparation daily for 2 mo failed to correct the anemia or leukopenia. It was not until shortly after intravenous administration of a cupric chloride solution during a 5-day period, at a total dose of 10 mg, that serum copper and ceruloplasmin levels increased and the anemia, leukopenia, and neutropenia resolved. These data suggest that the elimination of excess zinc is slow and that, until such elimination occurs, the intestinal absorption of copper is blocked.

Pigmented Corneal Rings in Non-Wilsonian Liver Disease

Kayser-Fleischer rings are pigmented corneal rings at the limbus of the cornea in Descemets membrane that have been deemed pathognomonic of Wilsons disease. However, we have observed four exceptions in patients with non-Wilsonian liver disease. Three patients had primary biliary cirrhosis and one patient had chronic aggressive hepatitis with cirrhosis. Pigmented corneal rings were seen only by slit-lamp examination. Hepatic, serum, and urinary copper and serum ceruloplasmin levels were significantly elevated in the patients with primary biliary cirrhosis. Radiocopper (64Cu or 67Cu) studies in patients with primary biliary cirrhosis showed plasma disappearance curves which allowed a clear distinction from Wilsons disease in that all three patients with primary biliary cirrhosis showed a secondary rise in radiocopper that presumably represented copper incorporation into ceruloplasmin. In one patient, in whom 64Cu in ceruloplasmin was studied specifically, incorporation was found to be normal.

Estimated versus measured basal energy requirements in patients with Crohn's disease

Increased energy expenditure associated with active inflammation has been thought to be one cause of weight loss in patients with Crohns disease. Our aim was to test this hypothesis by determining if resting energy expenditure (REE) measured by indirect calorimetry was greater than the predicted energy expenditure (PEE) calculated from the Harris-Benedict formula (variables--sex, age, height, and weight) in each patient. Fifty-four patients with radiographic evidence of Crohns disease were studied. There was a highly significant relationship (p less than 0.001) between REE and PEE, which can be expressed as follows: REE = 39.40 + 0.99 (PEE). The mean REE was 1427 +/- 228 kcal/day, whereas the mean PEE was 1404 +/- 197 kcal/day. Patients with the lowest weights when expressed as percentages of ideal body weights had the greatest resting energy expenditure per kilogram of body weight (r = -0.73, p less than 0.001). The mean REE per kilogram per day was 25 +/- 4 kcal, and only 4 of 54 patients (7%) had REE greater than or equal to 30 kcal/kg X day. Thus, REE measured by indirect calorimetry in Crohns disease patients was not significantly higher than PEE that can be estimated from the Harris-Benedict equation. These findings show that most Crohns disease patients without fever or sepsis do not have increased REE.

Home Parenteral Nutrition—A 3-Year Analysis of Clinical and Laboratory Monitoring

We report a 3-year analysis (1986 to 1989) of the management of 63 home parenteral nutrition patients, 40 with short-bowel syndrome and 23 with chronic intestinal obstruction with or without intestinal resection. Intravenous fluid requirements varied from 0.9 to 6 L/day, and the content of glucose varied between 46 and 531 g/day, protein varied from .0 to 85 g/day, fat from .0 to 100 g/day, sodium from 37 to 695 mEq/day, potassium from 30 to 220 mEq/day, chloride from 60 to 760 mEq/day, and acetate from 0 to 200 mEq/day. Body weight was normalized and well maintained in the majority of patients, but using the strict definition of deficiency as the presence of one abnormal value during 3 years, more than half had abnormal plasma chloride, glucose, alkaline phosphatase, serum glutamic oxaloacetic transaminase, total protein, albumin, selenium, and iron concentrations, and more than a third had low calcium, magnesium, vitamin D, and vitamin C levels. Normochromic anemia was seen in 73% and high blood creatinine associated with low urine volumes in 42%. Most (78%) returned to relatively normal lifestyles, but employability was occasionally impaired by loss of third-party insurance coverage resulting from a therapy that may cost

l-Carnitine therapy in home parenteral nutrition patients with abnormal liver tests and low plasma carnitine concentrations

100,000 per year. Overall mortality was low (5% per year), but 73% needed readmission to hospital, mainly for suspected catheter sepsis. The results indicate that home parenteral nutrition has allowed many patients to survive gut failure and return to work but problems with chronic fluid, electrolyte and micronutrient deficiencies, catheter sepsis, and insurance coverage often restrict optimal rehabilitation.