NUTRITION CARE PROCESS: PHENYLKETONURIA
left untreated. Additional long term complications include multiple organ failure in terms
of hepatic dysfunction, cardiomyopathy and musculoskeletal disorders such as arthritis
(Nelms & Sucher, 2015).
Question #7: The concerned child will have a diet with protein needs 25 to 30% higher as
compared to the normal Dietary Reference Intake (DRI), since a majority of the proteins
provided will comprise of supplements and synthetic sources. Further, as the child grows,
her diet will alter resulting in reductions in intake of amino acid and proteins, since the
rate or velocity of her growth will decrease. Further, musculoskeletal complications in
terms of hypotonia and arthritis, will reduce physical activity and hence, may need to
administer a diet with reduced calorie needs as compared to the DRIs (Nelms & Sucher,
2015). However, considering long term risks of impaired fetal growth and muscle
wastage due to catabolism, there may be need to incorporate adequate protein keeping in
mind intolerances associated with phenylalanine. Considering the same, the patient as she
grows, may be treated with Sapropterin dihydrochloride – a synthetic tetrahydrobiopterin
which assists in facilitation and stabilization of PAH into its active forms, by acting upon
PAH (Balaji et al., 2016). Further, to facilitate her growth and development as a child and
adult, she will be required to increase her phenylalanine, protein and calorie intake to
200-700 mg/day, 30-40 gm/day and 1300-2400 kcal/day (Nelms & Sucher, 2015).
Question #8: From the given results, it can be observed that patient has excessively high
levels of phenylalanine and low levels of tyrosine in the blood as compared to normal
levels, which can be diagnosed as hyperphenylalaninemia and hypotyrosinemia.
Hyperphenylalaniemia is etiologically caused due to high accumulation of phenylalanine
in the blood as a result of its decreased metabolism due to PKU (Burton et al., 2018). The
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Application of Nutrition Care Process: Phenylketonuria

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