Accuracy of home enteral feed preparation for children with inherited metabolic disorders

Evans, Sharon; Preston, Frederick W.; Daly, Anne; Neville, Charlotte; MacDonald, Anita

doi:10.1111/j.1365-277x.2010.01132.x

Cited by 11 publications

(15 citation statements)

References 12 publications

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“…Enteral feed composition is usually complex, requiring an individualized protein and energy profile commonly consisting of separate (or combined) modules of sources of protein, fat, carbohydrate, vitamins and minerals. It is essential that all caregivers administering enteral feeding receive professional instruction in safe feed production and that their feed preparation technique is reviewed on an annual basis [136,172-174]. …”

Section: Methodology and Objectivesmentioning

confidence: 99%

Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia

Baumgartner

Hörster

Dionisi‐Vici

et al. 2014

Orphanet J Rare Dis

539

788

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Methylmalonic and propionic acidemia (MMA/PA) are inborn errors of metabolism characterized by accumulation of propionic acid and/or methylmalonic acid due to deficiency of methylmalonyl-CoA mutase (MUT) or propionyl-CoA carboxylase (PCC). MMA has an estimated incidence of ~ 1: 50,000 and PA of ~ 1:100’000 -150,000. Patients present either shortly after birth with acute deterioration, metabolic acidosis and hyperammonemia or later at any age with a more heterogeneous clinical picture, leading to early death or to severe neurological handicap in many survivors. Mental outcome tends to be worse in PA and late complications include chronic kidney disease almost exclusively in MMA and cardiomyopathy mainly in PA. Except for vitamin B12 responsive forms of MMA the outcome remains poor despite the existence of apparently effective therapy with a low protein diet and carnitine. This may be related to under recognition and delayed diagnosis due to nonspecific clinical presentation and insufficient awareness of health care professionals because of disease rarity.These guidelines aim to provide a trans-European consensus to guide practitioners, set standards of care and to help to raise awareness. To achieve these goals, the guidelines were developed using the SIGN methodology by having professionals on MMA/PA across twelve European countries and the U.S. gather all the existing evidence, score it according to the SIGN evidence level system and make a series of conclusive statements supported by an associated level of evidence. Although the degree of evidence rarely exceeds level C (evidence from non-analytical studies like case reports and series), the guideline should provide a firm and critical basis to guide practice on both acute and chronic presentations, and to address diagnosis, management, monitoring, outcomes, and psychosocial and ethical issues. Furthermore, these guidelines highlight gaps in knowledge that must be filled by future research. We consider that these guidelines will help to harmonize practice, set common standards and spread good practices, with a positive impact on the outcomes of MMA/PA patients.

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Section: Methodology and Objectivesmentioning

confidence: 99%

Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia

Baumgartner

Hörster

Dionisi‐Vici

et al. 2014

Orphanet J Rare Dis

539

788

View full text Add to dashboard Cite

show abstract

“…Previous studies have demonstrated that knowledge and skills in the home environment post‐hospital discharge are less than ideal and may impact on patient safety (Evans et al. , 2006, 2007, 2010, 2011).…”

Section: Introductionmentioning

confidence: 99%

Home enteral tube feeding in children with inherited metabolic disorders: a review of long‐term carer knowledge and technique

Evans

Preston

Daly

et al. 2012

J Human Nutrition Diet

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The HETF techniques of caregivers of children with IMD declined over time. Caregivers need to understand that HETF, particularly in IMD, is a serious procedure associated with life-threatening risks. Poor HETF practices may cause feed contamination, incorrect feed concentration, feed intolerance, aspiration, peritonitis and even metabolic decompensation. HETF skills should be reassessed annually, with compulsory retraining if basic 'core' HETF competencies are not demonstrated.

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“…It has been demonstrated that in specialist modular feeds for IMD, increasing number of feed ingredients is associated with greater error and inaccurate feed preparation is common [1], [2], [3], [4]. Inaccurate feed production may be associated with feed intolerance, diarrhoea, hypernatraemia, and uneven intake of prescribed nutrients such as protein or fat and even metabolic decompensation [5], [6], [7], [8].…”

Section: Introductionmentioning

confidence: 99%

Accuracy of formula preparation equipment for liquid measurement

Evans

Ashmore

Daly

et al. 2014

Molecular Genetics and Metabolism Reports

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BackgroundModular specialist feeds may consist of a number of individual liquid ingredients. Accurate feed preparation is dependent on competent liquid measurement. We investigate the accuracy of two measuring jugs (one retail mix-and-measure; and one produced to laboratory standards); and the influence of volume and technique on accuracy.Materials and methods20 health professionals aged 18–60 y (mean: 46 y) measured 3 different volumes of water with each of two measuring jugs. For each volume with each jug, 2 measurements in randomised order were made: 1) eye-level with the jug, and 2) standing upright (total of 12 measurements). Measured quantities were weighed and the difference between measured and target volumes calculated.ResultsThe laboratory jug was more accurate (mean difference 9.3 ml, range − 30.5 to 57.5 ml, std error mean 1.59) than the retail jug (mean difference − 17.7 ml, range − 92.0 to 48.5 ml, std error mean 1.59). Accuracy improved with increased volume (450 ml: mean difference − 9.4 ml, range − 75.5 to 49.5 ml, std error mean 1.95; and 810 ml: mean difference − 0.7 ml; range − 92.0 to 43.0 ml, std error mean 1.95).ConclusionsAccurate measurement of liquid ingredients is difficult to achieve even for trained professionals. The cumulative effect of many different liquid measurement errors (inappropriate jug type, inaccurate volume measured and poor technique) may lead to clinically important errors in the preparation of modular specialist feeds.

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Accuracy of home enteral feed preparation for children with inherited metabolic disorders

Cited by 11 publications

References 12 publications

Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia

Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia

Home enteral tube feeding in children with inherited metabolic disorders: a review of long‐term carer knowledge and technique

Accuracy of formula preparation equipment for liquid measurement

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