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Table 14 Advice to help nausea and vomiting in maternal PKU

From: The complete European guidelines on phenylketonuria: diagnosis and treatment

Dietary and lifestyle approaches

Small frequent low protein meals and snacks that are high in carbohydrate (e.g. low protein toast, crackers) and low in fat to avoid an empty stomach, feelings of hunger, and abdominal distension [375]. Cold meals may be better if nausea is associated with food smells. For women who have difficulty in eating solid foods, additional drinks of cold water supplemented with glucose polymer may be tolerated if sipped throughout the day. Women should avoid lying down immediately after meals.

Phe-free L-amino acid supplements

Give Phe-free L-amino acid supplements chilled and encourage up to 5 or 6 times during the day in small doses. The high osmolality of Phe-free L-amino acid supplements may aggravate nauseas [347] and so may be better tolerated if given with extra fluid. If the smell of liquid or powdered Phe-free L-amino acid supplements is not tolerated, Phe-free L-amino acid tablets are worth consideration.

Any doses of Phe-free L-amino acid supplements lost through vomiting should be re-given. In extreme cases of vomiting and Phe-free L-amino acid supplements intolerance, hospital admission and administration of Phe-free L-amino acid supplement via a nasogastric tube could be considered.

Medication

Safe antiemetic therapy and acid reducing medications should be considered with persistent vomiting and symptoms of dyspepsia and indigestion.