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

Evans, Sharon; Preston, Frederick W.; Daly, Anne; Ashmore, Catherine; Holden, C; MacDonald, Anita

doi:10.1111/j.1365-277x.2012.01274.x

Cited by 17 publications

(25 citation statements)

References 9 publications

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“…If tube feeding is needed in the long-term, gastrostomy is recommended [172]. 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.…”

Section: Methodology and Objectivesmentioning

confidence: 99%

“…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%

See 1 more Smart Citation

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%

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

“…Where carers on-teach other family members, in order that carers can be partially relieved of the need to always undertake procedure(s), there may never be any formal professional assessment of the new carers' skills. Furthermore, continued adherence to professional directives cannot be assumed, as demonstrated by a series of annual studies which used a questionnaire and observation to assess carers' knowledge and safety in administering home enteral tube feeding for children with inherited metabolic disorders (Evans et al 2010(Evans et al , 2011(Evans et al , 2012. Each of these studies reported high levels of inadequate infection control and incorrect measurement of feed ingredients which could pose risks to the recipients (although no data were provided about actual harm having eventuated).…”

Section: Complex Home Care: Issues Of Safety and Responsibilitymentioning

confidence: 99%

Complex home care: challenges arising from the blurring of boundaries between family and professional care

McDonald

McKinlay

Keeling

et al. 2017

Kōtuitui: New Zealand Journal of Social Sciences Online

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Over the last 50 years, increasingly complex care (such as tracheostomy management, dialysis or enteral feeding) has shifted from hospital to home, with a concomitant rise in patient selfmanagement and care given by family members. Recognition of the importance of the contribution of family care to the health system is also growing. This article reports the findings of a New Zealand study which explored the experiences of family carers who manage technical health procedures at home. It then draws attention to some broader issues raised by shifting complex care from professional management in hospital settings to family care at home, namely the ways in which complex home care blurs the boundaries between professional and family care (creating the ambiguous position of the expert carer) and questions of safety and responsibility in family care. We also discuss the implications for policy around family caregiving in New Zealand. Given the potential physical and mental health impacts of caring, it is time for renewed consideration of what family carers should be expected or allowed to manage and how the health system can support them in their important role. ARTICLE HISTORY

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“…Some knowledge was still lacking despite annual review—this included dangers or incorrect tube placement, correct position for night feeding, and feed ingredient storage. Poor HEN practices can lead to feed contamination, feeding intolerance, aspiration, peritonitis, metabolic decompensation, and poor growth 29 …”

Section: Long‐term Considerations In Henmentioning

confidence: 99%

Optimizing Transition to Home Enteral Nutrition for Pediatric Patients

Sevilla

McElhanon

2016

Nut in Clin Prac

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Enteral nutrition is delivered via the gastrointestinal tract when oral intake is not sufficient to maintain nutrition status. There is evidence that long-term home enteral nutrition (HEN) can be advantageous to pediatric patients by improving quality of life and function. Data from pediatric patients receiving either nasogastric tube or gastrostomy tube feeds indicate good outcomes in terms of maintaining and improving nutrition status as evidenced by anthropometric measurements. As the number of pediatric patients requiring HEN increases, development of an effective program to allow smooth transition to the home is necessary. The formation of a multidisciplinary HEN management team can achieve the important task of taking care of children requiring HEN who are technology dependent. Members of a multidisciplinary HEN team can include care coordinators, nurses, physicians, dietitians, and speech therapists. Planning for patient discharge to home should start well before the actual discharge, with timely identification of home caregivers, the family's learning style, and completion of other requirements for safe administration of HEN. Education should be provided in a continuum, starting at the identification of nutrition support while still in the hospital and continuing after discharge to address concerns and long-term complications of HEN.

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

Cited by 17 publications

References 9 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

Complex home care: challenges arising from the blurring of boundaries between family and professional care

Optimizing Transition to Home Enteral Nutrition for Pediatric Patients

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