Dietary Supplement and Nutrition in Children With Cystic Fibrosis

Abstract

Assessment of nutritional status of patients with cystic fibrosis of the pancreas (CFP) showed that poor growth was associated with low concentrations of albumin, urea nitrogen, and cholesterol in serum and with elevated white blood cell (WBC) counts. Patients with CFP maintained weight approximately 1 standard deviation below the mean until age 8 years, after which there was a progressive decline in growth rate compared to normal. A complete dietary supplement consisting of a beef serum hydrolysate, a glucose polymer, and medium-chain triglycerides was given to 15 patients for ayear. Patients who received the diet showed significant gains in weight, significant increase in clinical score, significant increase in serum albumin level, and a significant drop in the WBC count compared to control patients who did not receive the supplement.Pancreatic insufficiency, leading to serious impairment of diges¬ tive functions, can be demonstrated in approximately 85% of patients with cystic fibrosis of the pancreas (CFP). In the majority of patients, progressive malnutrition becomes a feature of the disease in spite of sup¬ plementary enzyme therapy and con¬ sumption of more than the recom¬ mended allowance for calories and protein.1 Shohl et al, in 1943, reported studies of nitrogen and fat metabolism in in¬ fants and children with CFP.2 A striking feature of the disease was the excess nitrogen found in the bulky feces when regular diets con¬ taining whole protein and fat were consumed. When a fat-free diet con¬ sisting of casein hydrolysate and glu¬ cose was administered, nitrogen re¬ tention was in the range found in normal infants, and the amount of fe¬ cal excretion and the nitrogen con¬ tent were reduced to within normal limits. They concluded that even mod¬ erate amounts of protein were not di¬ gested and absorbed, but that gener¬ ous amounts of casein hydrolysate could be utilized. Administration of pancreatin did not lead to any im¬ provement in fat metabolism. Their objective was to develop a rational therapy for the disease by using a diet that was within the digestive ca¬ pacity of the patient.In 1946 West and others3 compared the effects of absorption of whole pro¬ tein with that of a protein hydroly¬ sate in normal children; in sick, mal¬ nourished children; and in children with CFP. They found increases in the blood amino nitrogen level of 1 to 4 mg/100 ml above fasting levels within lk to V-k hours after oral feed¬ ing of whole protein. Children with CFP showed either no elevation or a drop in blood amino nitrogen level and only a slow rise from fasting lev¬ els within three to five hours. Follow¬ ing ingestion of casein hydrolysate, children with CFP and control chil¬ dren showed similar increases in the blood amino nitrogen level. They also concluded that substitution of hydrolyzed protein for intact protein should have therapeutic value for patients with CFP.Substitution of medium-chain tri¬ glycérides (MCT) for long-chain fats was shown to be beneficial to patients with CFP in reducing the bulk and frequ...

Lung Infections Associated with Cystic Fibrosis

“…As treatment standards for CF patients improved over the years, the average mean survival of CF patients increased dramatically. Most notable among such advances was the refinement of nutritional regimens (4,19,290) and the advent of antibiotic chemotherapy (122,193,197,228). While essentially all patients prior to the 1950s died by the age of 10 years, reports published in the 1950s (192) and 1960s (297) described a considerable proportion of CF patients surviving well beyond this age.…”

Nutritional care in children with cystic fibrosis: are our patients becoming better?

“…Through the 1960s and 1970s, growth failure among CF populations was common, 11 as depicted in Figure 1. Patients were in general underweight at diagnosis, improving with therapy but still remaining on average À 1 s.d.…”

“…17 The eventual outcome of the cohort of CF infants fed the MCT formula without PERT was growth failure. 11,18 As alluded to in the above introduction, the Toronto clinic had achieved near-normal linear growth and weight, particularly in their male patients extending into older childhood. A summary of these data is shown in Table 1 in comparison with data from a CF clinic in Sydney with a low-fat dietary regimen.…”

Summary of a systematic review on oral nutritional supplement use in the community

“…Functional changes (assessed in fifty-nine studies) following ONS varied according to the disease or condition of patients, and were typically observed more commonly in those patients with a BMI < 20 kg/m 2 than in those with BMI > 20 kg/m 2 . Functional benefits included improved muscle strength, walking distance and well-being in patients with COPD (Wilson et al 1986;Efthimiou et al 1988;Donahoe et al 1989;Rogers et al 1992), improved growth performance in children with cystic fibrosis (Allan et al 1973;Berry et al 1975;Yassa et al 1978;Kirschner et al 1981;Parsons et al 1983;Shepherd et al 1983;Skypala et al 1998), reduced falls (Gray-Donald et al 1995) and increased activities of daily living (Volkert et al 1996) in the elderly, and improvements in immune function in patients with human immunodeficiency virus (Hellerstein et al 1994). It is possible that detriments due to ONS may occur in some overweight patients with certain diseases (e.g.…”