Anorexia nervosa: nocturnal supplementary nasogastric feeding in the community

Nocturnal supplementary nasogastric feeding in a domiciliary setting coupled with dietary counselling in 4 patients with a diagnosis of anorexia nervosa is detailed. The four patients were given nocturnal supplementary nasogastric feeds, for periods of 16 days to 12 weeks. In three of the four patients substantial weight gains of 4.3 to 11.5 kg were achieved and all four patients reported substantial increases in daily voluntary oral food intake.
We conclude that this approach may enable the patient to be managed at home and provides a basis for further psychotherapy treatment.     

Oral nutritional supplements promote significant weight gain in cystic fibrosis patients

Background: Malnutrition and poor growth are major problems for many patients with cystic fibrosis (CF), and nutritional supplements can enhance the success of dietary strategies. The purpose of this study was to assess Scandishake ^{(Scandipharm Ltd, U.K.}), an energy dense (2.0 kcal/ml) oral supplement. Methods: The study design included a 4-week monitoring period, followed by 8 weeks of supplementation with Scandishake. The subjects had anthropometry assessed at weeks 0, 4 and 12, and their dietary intake assessed at weeks 0 and 12.
Results: Data from 26 patients (16 male, 10 female; age range 9–34 years) showed a significant mean weight increase of 1.9 kg (range 1.6–4 kg) and a mean increase of 0.5 cm (range 1.1–2.7 cm) in mid-arm muscle circumference. There was also an increase in energy intakes, rising from a mean of 11.15 MJ (2665 kcals) to a mean of 13.42 MJ (3208 kcals) ( P < 0.01), and the mean of individual percentages of estimated average requirement (EAR) for energy increased from 120% (73–182%) to 143% (67–221%).
Conclusion: The energy dense formulation of Scandishake makes it a useful adjunct in the treatment of malnutrition and the promotion of normal growth in patients with CF.     

A review of infant feeding practices at a regional cystic fibrosis unit

We have reviewed the outcome and feeding of the 28 cystic fibrosis (CF) infants diagnosed at St James's University Hospital, Leeds, during 15 years between 1974–1988. During this period most infants were fed on either standard formula milk feeds or a lower fat, higher protein mix (CF milk). It was the practice to prescribe pancreatic enzymes from the diagnosis of CF, wean the infant on to solids between 2 and 3 months of age and provide extra calorie supplements if infants were not thriving adequately. The mean weight at birth (s.d.) was 3.33 (0.45) kg and at 12 months was 9.56 (1.21) kg. It is concluded that CF infants provided with adequate energy intake and appropriate pancreatic enzymes should thrive satisfactorily.     

Five-year prospective analysis of dietary intake and clinical status in malnourished cystic fibrosis patients

Background Poor growth and malnutrition still pose a problem in cystic fibrosis (CF). The aim of the present study was to assess nutrition, as well as clinical status, of malnourished CF patients during a nutritional care programme. Material and methods The study comprised 38 CF patients, aged 1–18 years old. The prospective annual assessment of dietary intake and clinical status was carried out during 1994–98. Results The energy intake increased, in comparison with recommended daily allowances, from 83.6 ± 4.8% in 1994 to 107.9 ± 4.9% in 1998. A similar tendency was observed for the percentage of energy derived from fat (30.3 ± 0.8% versus 35.1 ± 0.8%) and protein (11.4 ± 0.4% versus 13.8 ± 0.4%). In subsequent years of the study, an improvement in the fat profile of the diet (with a higher consumption of polyunsaturated fatty acids) was observed. The observed increase of vitamin A and E consumption was related chiefly to changes in the doses of supplementation. During these 5 years, an improvement in nutritional status (Z‐score: height ?1.34 ± 0.13 versus ?1.08 ± 0.14 and weight ?1.40 ± 0.09 versus ?1.12 ± 0.08) and lung function (forced expiratory volume in 1 s: 75.5 ± 2.0% versus 77.8 ± 2.2%) was observed. Conclusion The nutritional care programme resulted in stable quantitative and qualitative changes in dietary intake. Although the diet does not reach the recommended level of high‐energy intake, the positive impact of increasing nutrient intake on the nutritional and clinical status of malnourished CF patients was documented.     

Vitamin A deficiency in cystic fibrosis resulting in xerophthalmia

We report a case of severe vitamin A deficiency in a patient with cystic fibrosis due to a combination of poor compliance, malabsorption and probable disordered metabolism of vitamin A in the liver. He presented with severe xerophthalmia, clinical resolution of which necessitated intramuscular vitamin A administration.     

Audit of nasogastric feeding practice at two acute hospitals: is early enteral feeding associated with reduced mortality and hospital stay?

Hospital malnutrition is common and thought to be a cause of morbidity and mortality. Nasogastric (NG) feeding is the most commonly used invasive technique of nutritional support used at the acute Bolton hospitals. A prospective observational study was initiated to audit the use of NG feeding in patients in whom oral energy intake was virtually nil at the time of commencement of tube feeding.
Patients who were starved for 0–5 days prior to commencement of NG feeding had a lower mortality than patients starved >5 days (a) during their feeding episode and (b) during their hospital stay subsequent to cessation of oral intake. The difference in mortality was not related to age or sex. However, in patients of <65 years mortality was only non-significantly higher in patients starved >5 days compared with those starved 0–5 days. In patients of >64 years the difference in mortality between those starved 0–5 vs. >5 days remained significant: (a) during the feeding episode and (b) during the hospital stay. The fact that starvation has a disproportionate effect on mortality in old patients may indicate that older patients are more susceptible to starvation. In surviving patients there was a positive correlation between the length of starvation and: (a) the duration of the NG feeding episode and (b) hospital stay subsequent to cessation of oral intake. Disease severity was not measured therefore its effect on outcome and speed of rehabilitation cannot be excluded.
The study indicates a possible relationship between the duration of starvation and mortality, the duration of NG feeding and the length of hospital stay. Definitive testing of this association would require a prospective trial which controls for age and disease severity.     

Riboflavin deficiency in cystic fibrosis: three case reports

Three cases of clinical riboflavin deficiency are reported in children aged 2–10 years attending a regional Cystic Fibrosis clinic. Riboflavin deficiency presented as angular stomatitis in all three patients. Patients were confirmed to be riboflavin deficient by assaying the activity of erythrocyte glutathione reductase. Patients were not on routine supplements of water-soluble vitamins before presentation and were treated with riboflavin supplements as part of a water-soluble vitamin complex. At presentation, one patient had poor nutritional status, but two patients were adequately nourished, receiving overnight Gastrostomy feeds. Data on these two patients indicate an adequate dietary intake of riboflavin, suggesting a mechanism for increased requirements, inadequate absorption or utilization. Additional deficiencies of thiamin, pyridoxine and iron were also observed. This paper reports the occurrence of a vitamin deficiency not previously reported in the cystic fibrosis population.     

PREMATURE INFANTS FED TRANSPYLORICALLY GAIN WEIGHT SLOWLY

Nasojejunal feeding in hospital caused slow weight gain compared to nasogastric feeding in two similar groups of low-birth-weight babies. Because of faster catch up of growth in a nasojejunally fed group, both groups were of similar size at three months of age.     

Taste preference for oral calorie supplements in children with cystic fibrosis, healthy children and healthy adults

Background: Calorie supplements are commonly prescribed to improve the nutritional status of children with cystic fibrosis. Although the palatability of these products has been extensively researched in adults, little work has been carried out in either healthy children or children with cystic fibrosis. The aim of this study was to identify which type of supplement was preferred by children with cystic fibrosis, healthy children and healthy adults. Methods: 23 children with cystic fibrosis, 65 healthy children and 40 healthy adults were recruited to the study. All were asked to taste four of the main types of prescribable supplements available and indicate which they preferred. Results: The preference score for the fresh milk supplement was significantly higher than the scores for the other three supplements. There was no apparent difference between the taste preferences of the three groups studied. Conclusions: The results of this study indicate that children with cystic fibrosis have similar taste preferences for calorie supplements to healthy children and adults. The fresh milk supplement was preferred by the majority of the subjects, which suggests that when starting a patient on calorie supplements a fresh milk product should be offered first. The preference testing method used here is quick and easy to do and may be useful for assessing patient preferences at the bedside.     

A prospective comparison of the use of nasogastric and percutaneous endoscopic gastrostomy tubes for long-term enteral feeding in older people

OBJECTIVE: To compare the indications for and the outcome of long-term enteral feeding by nasogastric tube (NGT) with that of percutaneous endoscopic gastrostomy (PEG) tube. DESIGN: A prospective, multicenter cohort study. SETTING: Acute geriatric units and long-term care (LTC) hospitals in Jerusalem, Israel. PARTICIPANTS: 122 chronic patients aged 65 years and older for whom long-term enteral feeding was indicated as determined by the treating physician. Patients with acute medical conditions at the time of tube placement were excluded. MEASUREMENTS: We examined the indications for enteral feeding, nutritional status, outcome and complications in all subjects. Subjects were followed for a minimum period of six months. RESULTS: Although the PEG patients were older and had a higher incidence of dementia, there was an improved survival in those patients with PEG as compared to NGT (hazard ratio (HR)=0.41; 95% confidence interval (CI) 0.22-0.76; P=0.01). Also, the patients with PEG had a lower rate of aspiration (HR=0.48; 95% CI 0.26-0.89) and self-extubation (HR=0.17; 95% CI 0.05-0.58) than those with NGT. Apart from a significant improvement in the serum albumin level at the 4-week follow-up assessment in the patients with PEG compared to those with NGT (adjusted mean 3.35 compared to 3.08; F=4.982), nutritional status was otherwise similar in both groups. CONCLUSION: In long-term enteral feeding, in a selected group of non-acute patients, the use of PEG was associated with improved survival, was better tolerated by the patient and was associated with a lower incidence of aspiration. A randomized controlled study is needed to determine whether PEG is truly superior to NGT.     

Life on a slippery slope: perceptions of health in adults with cystic fibrosis

This paper focuses on how adults with cystic fibrosis (CF) attending a specialist CF centre in the UK perceive their health. In common with many other genetic diseases, CF is traditionally conceptualised as a fatal childhood disease, yet the average survival age for those with CF has been steadily rising over the past half century. Thus it is now predicted that those born in 1990 will live on average for 40 years. To date, however, most sociological work has focused on children or adolescents affected by CF rather than on adults between the ages of 18 and 40, the focus of the study reported here. The paper shows that these adults' varying perceptions of health are related to the effects of CF, its treatment, and the context in which adults are placed. Four concepts of health are identified (health as 'normal', controllable, distressing and a release) along with certain styles, ways of coping and related strategies. Through these analytic distinctions the paper aims to make a contribution to the sociological understanding of lay concepts of health in adults with childhood or genetic disease.     

Energy intakes and losses in cystic fibrosis

Energy intakes in cystic fibrosis (CF) are affected by malabsorption, despite the use of enteric-coated pancreatic enzymes. Relaxation of fat restrictions was introduced to aid consumption of a diet sufficient to compensate for malabsorption and help prevent malnutrition. We examined the energy intakes and losses of 50 CF patients on a normal fat intake (mean age 9.6 [s.d.=6.67] years, M:F ratio 33:17). Mean standard deviation scores for height and weight were –1.03 and –0.80, respectively. Nutrient balances were made by means of 7-day weighed dietary inventories and 3-day faecal collections. Mean metabolizable energy intake was 97.3 [s.d. 23.1)% RDA. Mean digestibility coefficient for energy was 0.89 (s.d. 0.07), reducing mean energy intakes to 91.9 (s.d. 24.0)% RDA. The results indicate that despite relaxation of dietary restrictions and the use of enteric-coated pancreatic enzymes, energy intakes of CF patients are still inadequate. Closer monitoring of energy losses as well as intakes is required, to optimize growth and nutritional status in CF patients.     

Reducing pancreatic enzyme dose does not compromise growth in cystic fibrosis

Background : Reports linking high pancreatic enzyme dosages with bowel stricturing in children with cystic fibrosis (CF) led us to review our policy for pancreatic enzyme supplementation. Methods : Twenty-five prepubertal children with CF, aged 3–10 years, underwent a programme of pancreatin reduction. They were encouraged to decrease their enzyme intake by matching their pancreatin dose more closely to fat intake. Patients were reviewed at regular intervals by a paediatric dietician who promoted nutrition aiming for an energy intake of 120–150% of the estimated average requirement (EAR) and > 120% of the upper reference nutrient intake (URNI) for protein. Growth during the 12 months prior to enzyme restriction was compared with the subsequent year's growth. Results : The initial mean pancreatin dose of 26 446 uLipase kg⁻¹ day⁻¹ (range 7305–53 088) was reduced to 12 583 uLipase kg⁻¹ day⁻¹ (range 4705–32 051). Growth was sustained on the lower enzyme dose (mean height velocity: 5.75±1.1 cm yr⁻¹ vs. 6.12±1.4 cm yr⁻¹, P > 0.05). There was a small but significant improvement in mean weight gain post pancreatin reduction (3.14±1.5 kg yr⁻¹) compared with the preceding year (2.12±1.1 kg yr⁻¹, P < 0.01). Nutritional analysis, including a 3-day food diary and measurement of nutritional indices, showed that energy, protein and micronutrient intakes were maintained. Children were not forced to alter their intake of dietary fat by the reduction in pancreatin dose. Conclusions : A substantial reduction in pancreatin dosage can be achieved without an adverse effect on either growth or nutrition.     

Pancreatic enzyme supplements and vitamins in cystic fibrosis

The management of pancreatic insufficiency and the maintenance of satisfactory fat-soluble vitamin status are major issues in the nutritional management of cystic fibrosis (CF). This article discusses the pancreatic enzyme replacement preparations currently used in the United Kingdom. It also provides an overview of the importance of fat-soluble vitamins in CF, the supplements currently used, and assesses where future research might be directed. We conclude that dietitians play an important role in helping CF patients to achieve optimal growth and nutritional status, and should therefore be involved in management from diagnosis. Monitoring of pancreatic enzyme replacement therapy (PERT) and vitamin therapy is an integral part of the dietetic assessment, and should be carried out on a regular basis to ensure optimal nutritional management for each individual. Future research needs to concentrate on the efficacy and safety of high lipase enzyme preparations. Research is required to assess the absorption, metabolism and losses of fat-soluble vitamins amongst today's CF population, and to determine requirements according to age group.     

A study of a single high potency multivitamin preparation in the management of cystic fibrosis

Background: ADEKs® is a new multivitamin preparation formulated specifically for cystic fibrosis (CF) patients, containing all four fat-soluble vitamins. There is no data currently available on its use in CF. Method: We carried out a retrospective study to evaluate ADEKs® in 54 CF patients attending the specialist CF Unit at Nottingham City Hospital. Plasma vitamin A and E levels were collected from annual review reports when taking ADEKs® and a year previously when using other vitamin preparations. Dietary assessment data was available for 24 of these patients (11 children, 13 adults). Results: ADEKs® was taken for a median of 9.5 months by 19 children (mean age 11.3 years; range 7–15 years) and for 4 months by 35 adults (mean age 24 years; range 17–36 years). Median plasma vitamin A levels were significantly higher ( P <0.05) for all age groups (27% increase for 7–10 year olds, 48% increase for 11–15 year olds, 100% increase for 16+ year olds) following the change from other vitamin supplements to ADEKs®. Median plasma vitamin E levels were also significantly higher (59% increase for 7–10 year olds; 16% increase for 11–15 year olds; 26% increase for 16+ year olds). Fifteen (28%) and 9 (17%) patients went from below to within the normal plasma reference range for vitamins A and E, respectively, when changed onto ADEKs®. Four (7%) and 11 (20%) patients had values above the normal plasma range for vitamins A and E, respectively, whilst on ADEKs®. Conclusion: This study suggests that ADEKs® tablets are useful for CF patients aged over 10 years as plasma vitamin A and E levels were improved. The use of a single vitamin preparation such as ADEKs® is likely to improve compliance. We have reservations over its use amongst the 7–10 year olds because of high plasma vitamin A (3/9) and E (7/9) levels, although further research may ultimately endorse its future use within this group.     

Appetite stimulants in cystic fibrosis: a systematic review

BACKGROUND: The use of appetite stimulants in cystic fibrosis (CF) is controversial because of doubts concerning efficacy and side-effects. The aim of the review was to establish whether appetite stimulants should be recommended in CF. MATERIALS AND METHODS: Medline, AMED, British Nursing Index, Cinahl, Embase, The Cochrane Library, National Research Register and Google were searched. Unpublished studies, case reports and conference abstracts were sought from experts, including authors of identified published articles, and suppliers of appetite stimulants. RESULTS: Fifty-four articles were located. Fifteen studies were suitable for inclusion in the review: 10 studied, megesterol acetate (MA); 1, dronabinol; 2, mirtazapine; and 2, cyproheptadine. MA showed a beneficial effect on lung function, weight, appetite, fat free and fat mass. Adverse effects for MA included adrenal suppression, abnormalities of glycaemic control, mood changes and testicular failure. There was no consistency in the dose, frequency and duration of therapy for MA. CONCLUSIONS: The review shows MA may have a role in the management of anorexia associated with CF. It is not possible to be conclusive for the other agents given the low numbers in the studies. Larger randomized, controlled trials of MA are necessary to confirm its safety and validate efficacy in CF.     

Physiological and psychosocial contributors to malnutrition in children with cystic fibrosis: review

Malnutrition was once thought to be an inevitable consequence of cystic fibrosis (CF). It is now considered preventable but still contributes considerable morbidity in children. Malnutrition is linked to poorer pulmonary function, reduced survival and quality of life. As the anticipated lifespan of children with CF continues to lengthen, the prevention of malnutrition attains greater importance. This review explores the complex organic and psychosocial factors implicated in the aetiology of malnutrition associated with CF.