Familial chylomicronemia syndrome: Bringing to life dietary recommendations throughout the life span.

BACKGROUND: Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive disorder with loss of function mutations of lipoprotein lipase resulting in hypertriglyceridemia and accumulation of chylomicrons in plasma, often leading to acute pancreatitis. The mainstay of treatment is a specialized very-low-fat diet. Even adhering to the diet, some patients may experience high triglycerides and pancreatitis. There currently are no comprehensive dietary guidelines. OBJECTIVE: To report best practices and develop comprehensive dietary guidelines for nutrition therapy in patients with FCS. METHODS: Registered dietitian nutritionists (RDNs) convened to develop this report based on experience treating patients with FCS and a review of current literature on the topic. One author provided a patient perspective of living with FCS. RESULTS: This report provides guidelines and rationales for nutrition therapy associated with FCS across the life span. The top global guidelines are to (1) limit fat to <15 to 20 g per day (<10%-15% of total daily energy intake); (2) meet recommendations for essential fatty acids: α-linolenic acid and linoleic acid; (3) choose complex carbohydrate foods while limiting simple and refined carbohydrate foods; (4) supplement with fat-soluble vitamins, minerals, and medium-chain triglyceride oil, as needed; (5) adjust calories for weight management. Recommended foods include vegetables, whole grains, legumes, lean protein foods, fruits in limited amounts, and fat-free milk products without added sugars. Foods to avoid include alcohol and products high in sugar. CONCLUSIONS: These patient-centered nutrition guidelines provide guidance to help patients adhere to the recommended diet and optimize nutritional needs.

Medical nutrition therapy is the essential cornerstone for effective treatment of "refractory" severe hypertriglyceridemia regardless of pharmaceutical treatment: Evidence from a Lipid Management Program.

BACKGROUND: Patients with refractory severe hypertriglyceridemia are at risk of pancreatitis and cardiovascular disease. The role of individualized nutrition therapy in these patients independent of pharmaceutical treatment has not been documented. OBJECTIVE: To document the effect of nutrition intervention on severe hypertriglyceridemia regardless of medication status or prior nutrition counseling. METHODS: Outcomes of new patients with triglycerides ≥ 500 mg/dL presenting to a Lipid Management Program over a 6-year period were tracked. Patients received comprehensive laboratory assessment, nutrition assessment, and initiation of an individualized diet intervention before seeing the lipidologist. Clinical and behavioral outcomes were recorded. RESULTS: In all, 168 patients (117 men; mean age, 49.03 ± 11.22 years; body mass index, 32.61 ± 5.85 kg/m(2); 110 (65.5%) on lipid-lowering medications) returned for assessment of nutrition intervention. Triglycerides were reduced from median (interquartile range) 961.5 (611.5-1785.3) to 493.0 (337-736.3) mg/dL (P < .0001 for log transformation of triglycerides). There was no difference in median percentage reduction in triglycerides after nutrition intervention between those not on lipid-lowering medication, on a fibric acid derivative, on other lipid-lowering medication, or on a combination of lipid-lowering medications (P = .376) in a median (interquartile range) of 5 (3-7) weeks. Effect was independent of prior nutrition counseling (P = .260). Reported percentage fat in the diet at second visit correlated with log-transformed triglycerides achieved, independent of initial triglycerides level (r = 0.290; P = .001). CONCLUSIONS: Individualized nutrition therapy results in changes in eating behavior and reductions in triglyceride levels in patients with refractory severe hypertriglyceridemia independent of lipid-lowering medication(s) and prior nutrition counseling.

Healing the heart: a randomized pilot study of a spiritual retreat for depression in acute coronary syndrome patients.

BACKGROUND: Depression is associated with increased risk of cardiovascular morbidity and mortality in coronary heart disease. Numerous conventional and complementary therapies may address depression. Few involving spirituality have been tested. OBJECTIVE: The aim of this study was to compare the effects of a nondenominational spiritual retreat, Medicine for the Earth (MFTE), on depression and other measures of well-being six- to 18-months post acute coronary syndrome (ACS). DESIGN/SETTING: A randomized controlled pilot study of MFTE, Lifestyle Change Program (LCP), or usual cardiac care (control) was conducted in Southeastern Michigan. PARTICIPANTS: ACS patients were recruited via local and national advertising (n = 58 enrolled, 41 completed). INTERVENTIONS: The four-day MFTE intervention included guided imagery, meditation, drumming, journal writing, and nature-based activities. The four-day LCP included nutrition education, exercise, and stress management. Both retreat groups received follow-up phone coaching biweekly for three months. MAIN OUTCOME MEASURES: Validated self-report scales of depression, spiritual well-being, perceived stress, and hope were collected at baseline, immediately post-retreat, and at three and six months. RESULTS: Depression was not significantly different among groups (P = .21). However, the MFTE group had the highest depression scores at baseline and had significantly lower scores at all postintervention time points (P ≤ .002). Hope significantly improved among MFTE participants, an effect that persisted at three- and six-month follow-up (P = .014). Although several measures showed improvement in all groups by six months, the MFTE group had immediate improvement post-retreat, which was maintained. CONCLUSIONS: This pilot study shows that a nondenominational spiritual retreat, MFTE, can be used to increase hope while reducing depression in patients with ACS.

The lipid management nutrition outcomes project: perspectives from a national experience in protocol implementation and nutrition outcomes tracking.

The Lipid Management Nutrition Outcomes Project was a multicenter prospective noncontrolled observational study in which a network of 51 registered dietitians (RDs) from practice settings across the United States implemented the 1998 Medical Nutrition Therapy Hyperlipidemia Protocol and collected outcomes. Difficulty recruiting RDs and enrolling patients revealed a gap between practice guidelines and clinical practice. Many RDs did not have laboratory values or follow-up visits required by the protocol. RDs able to follow protocol recommendations had the expected positive results. Within a 6-month period, 377 new patients presenting for lipid management met inclusion/exclusion criteria. Some follow-up data were available on 280 (74.3%) patients. There were follow-up lipid data prior to lipid-lowering medication changes for 219 patients. Reported mean dietary fat intake was reduced to <30% (P<0.0001). The population lost weight and increased exercise frequency (P<0.001, P<0.001). In the 175 patients with initial triglycerides <400 mg/dL (4.52 mmol/L), 44.6% had either a 15% drop in low-density lipoprotein cholesterol or reached low-density lipoprotein cholesterol goal. Lipid response occurred in 34.7+/-16.5 weeks with 3.0+/-1.4 RD visits. The Lipid Management Nutrition Outcomes Project highlights frustrations and values of outcomes monitoring in actual practice and identifies areas for practice advancement.