Maternal phenylketonuria: A case study suggesting the use of prenatal psychotherapy to help control phenylalanine levels.

Antshel, Kevin M.; Gurian, Elizabeth A.; Waisbren, Susan E.

doi:10.1037/0002-9432.72.4.577

Cited by 3 publications

(3 citation statements)

References 50 publications

(54 reference statements)

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“…The Resource Mothers are especially helpful in providing emotional support and giving assistance to make the diet more manageable. It is more di⁄cult to intervene in changing attitudes; some women with PKU have negative feelings about the diet and a long history of family stress and disordered eating that may require more intensive psychological intervention to change (Antshel et al 2002).…”

Section: Discussionmentioning

confidence: 99%

The Resource Mothers Study of Maternal Phenylketonuria: Preliminary findings

Rohr¹,

Munier²,

Sullivan³

et al. 2004

J of Inher Metab Disea

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Women with phenylketonuria (PKU) must follow a strict low-phenylalanine diet during pregnancy in order to protect the fetus from the deleterious effects of high maternal blood phenylalanine. The Resource Mothers Study of Maternal PKU was undertaken to determine whether a home visitation programme was effective in helping women with PKU attain blood phenylalanine control earlier during pregnancy. Resource Mothers were trained to provide social support and practical assistance to women with PKU during pregnancy. Eight metabolic clinics in the United States participated in the study. Women with PKU who were planning pregnancy or already pregnant were enrolled in the study and were treated with a low-phenylalanine diet aimed at controlling blood phenylalanine to 120-360 micromol/L. They were randomly assigned to receive the services of a Resource Mother (RM group) or to a control group. Fifty women were enrolled, and accounted for 44 pregnancies which resulted in 28 live births, and 6 spontaneous abortions. Ten women are currently pregnant and another 6 have not become pregnant. Fifty-six percent of enrolled women began the diet prior to becoming pregnant. Fifty-three percent of women in the Resource Mother group were in metabolic control by 10 weeks gestation as compared to 39% in the control group. In addition, women who began diet after pregnancy and had a Resource Mother attained metabolic control earlier (mean gestational age of 22.4 weeks in the RM group vs 29.8 weeks in the control group). There was no difference in birth measurement z -scores of offspring born to women in the RM group compared to controls. All but 4 women rated themselves as feeling worse about the diet at the end of pregnancy than at the beginning, and few women in either group remained on diet after delivery.

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Section: Discussionmentioning

confidence: 99%

The Resource Mothers Study of Maternal Phenylketonuria: Preliminary findings

Rohr¹,

Munier²,

Sullivan³

et al. 2004

J of Inher Metab Disea

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“…Programs to help women with PKU to achieve sufficient metabolic control during pregnancy have been demanded for decades [8,21,22]. Attempts include home visitation programs [13] hospitalization during pregnancy [14], or accompanying psychotherapy [15], all with inconclusive results. The study presented here aimed to identify most important factors to gain and sustain good metabolic control in phenylketonuric women throughout pregnancy, assuming that the current programs of health care systems might be off target or insufficient.…”

Section: Discussionmentioning

confidence: 99%

“…Specialized PKU care for adults, especially with respect to care during pregnancy, is still scarce [4,11,12] and most gynecologists and obstetricians are unfamiliar with this disease and the effects of typical pregnancy related issues on the metabolic control in PKU. Attempts to improve care include home visitation programs [13], hospitalization during pregnancy [14], or accompanying psychotherapy [15], all with inconclusive results.…”

Section: Introductionmentioning

confidence: 99%

Preventing maternal phenylketonuria (PKU) syndrome: important factors to achieve good metabolic control throughout pregnancy

Rohde

Thiele

Baerwald

et al. 2021

Orphanet J Rare Dis

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Background Insufficient metabolic control during pregnancy of mothers with phenylketonuria (PKU) leads to maternal PKU syndrome, a severe embryo-/fetopathy. Since maintaining or reintroducing the strict phenylalanine (Phe) limited diet in adults with PKU is challenging, we evaluated the most important dietary and psychosocial factors to gain and sustain good metabolic control in phenylketonuric women throughout pregnancy by a questionnaire survey with 38 questions concerning therapy feasibility. Among them, the key questions covered 5 essential items of PKU care as follows: General information about maternal PKU, PKU training, diet implementation, individual metabolic care, personal support. In addition, all participating PKU mothers were asked to estimate the quality of their personal metabolic control of the concluded pregnancies. 54 PKU mothers with 81 pregnancies were approached at 12 metabolic centers in Germany and Austria were included. According to metabolic control, pregnancies of PKU women were divided in two groups: group “ideal” (not more than 5% of all blood Phe concentrations during pregnancy > 360 µmol/l; n = 23) and group “suboptimal” (all others; n = 51). Results The demand for support was equally distributed among groups, concerning both amount and content. Personal support by the direct social environment (partner, family and friends) (“suboptimal” 71% vs “ideal” 78%) as well as individual metabolic care by the specialized metabolic center (both groups around 60%) were rated as most important factors. The groups differed significantly with respect to the estimation of the quality of their metabolic situation (p < 0.001). Group “ideal” presented a 100% realistic self-assessment. In contrast, group “suboptimal” overestimated their metabolic control in 53% of the pregnancies. Offspring of group “suboptimal” showed clinical signs of maternal PKU-syndrome in 27%. Conclusion The development of training programs by specialized metabolic centers for females with PKU in child bearing age is crucial, especially since those mothers at risk of giving birth to a child with maternal PKU syndrome are not aware of their suboptimal metabolic control. Such programs should provide specific awareness training for the own metabolic situation and should include partners and families.

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