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PKU in adolescents: rationale and psychosocial factors in diet continuation
Author(s) -
Levy HL,
Waisbren SE
Publication year - 1994
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1994.tb13463.x
Subject(s) - medicine , psychosocial , anxiety , discontinuation , metabolic control analysis , phenylketonurias , pediatrics , psychiatry , biochemistry , chemistry , amino acid , phenylalanine , insulin , endocrinology
Follow‐up of early‐treated children with PKU has shown that diet discontinuation in childhood presents risks of cognitive and emotional dysfunction in a substantial number of adolescents and young adults. This dysfunction includes IQ loss, mental processing abnormalities, learning difficulties, anxiety and personality disorders. In addition, neurologic deterioration has been reported in several such individuals. As consequence of this current understanding of PKU, diet continuation, at least through adolescence and in the young adult years, is now recommended. Many centers are extending this to policy of “diet for life”. This represents major challenge to adolescents and their families. Metabolic control using the criteria applied during childhood is virtually impossible to achieve past 12 years of age. Time constraints, social pressures, financial limitations and growing independence from the family combine to interfere with dietary control. Added to these difficulties are the biological changes during teenage years which reduce phenylalanine tolerance. To meet these challenges, we have identified number of psychosocial factors that interfere with adherence to medical recommendations. The factors most highly related to metabolic control were social support for the diet and positive perceptions of treatment. This information has led to the development of support programs for adolescents and young adults with PKU. These programs include an annual New England maternal PKU camp, resource mothers program in which mothers of young children with PKU are matched to women with PKU who are either planning pregnancy or are pregnant and assist with meal planning, cooking, metabolic monitoring, and transportation to medical appointments as well as provide enormous emotional support to these young women, and PKU CORPS which brings together on‐diet adolescents for training and emotional support and then matches them with younger phenylketonuric children on diet. The benefits of these programs are becoming clear in terms of improved metabolic control, successful re‐institution of diet, a more comfortable acceptance of PKU and better self‐image.