Practical aspects of managing low protein diets.
Many inborn errors of metabolism can be successfully managed by restriction of natural protein intake, with or without special supplements. In some cases, there is a need for clear advice on management during metabolic crisis brought about by illness. Even when medical facilities and ongoing support are on hand, and special products are available and affordable, management of such conditions is challenging to families. Tailoring diet instruction to the needs and capabilities of the family is most likely to achieve success. Many Australian families cope better with simple guidelines aimed at reducing protein intake within the needs for growth and development and this style of management is also more likely to be successful in countries in which support is limited. Simple guidelines can take the form of increasing intake of certain types of foods and limiting others. Assessment of vitamin and mineral intake is however essential. Breastfeeding will provide less protein than standard infant formula, as well as immune protection, and needs to be encouraged and supported. Restricting meat and other high protein foods with increased intake of cereal and vegetable protein will still substantially decrease protein intake. Increasing intake of low protein vegetable sources or special low protein foods will also by default decrease protein intake. Families need specific guidelines for the care of their child when metabolic decompensation is a risk, with the aim of maintaining adequate energy intake, in forms that the child can eat or drink. A specialist pediatric dietitian can provide the expertise in formulating management plans appropriate for the family and facilities, with ongoing local management. The support of other families dealing with similar conditions can also be invaluable.
Thompson
Sue
S
Western Sydney Genetic Program, The Children's Hospital at Westmead, Westmead, Sydney, Australia.
D001315:Australia; D018753:Diet, Protein-Restricted / Q000379:methods*; D004046:Dietetics; D006801:Humans; D007223:Infant; D007225:Infant Food; D007227:Infant Nutritional Physiological Phenomena; D007231:Infant, Newborn; D008661:Metabolism, Inborn Errors / Q000175:diagnosis / Q000178:diet therapy*; D015997:Neonatal Screening