Weaning practices in phenylketonuria vary between health professionals in Europe

A Pinto, S Adams, K Ahring, H Allen, M F Almeida, D Garcia-Arenas, N Arslan, M Assoun, Y Atik Altınok, D Barrio-Carreras, A Belanger Quintana, S M Bernabei, C Bontemps, F Boyle, G Bruni, M Bueno-Delgado, G Caine, R Carvalho, A Chrobot, K Chyż, B Cochrane, C Correia, K Corthouts, A Daly, S De Leo, A Desloovere, A De Meyer, A De Theux, B Didycz, M E Dijsselhof, K Dokoupil, J Drabik, C Dunlop, W Eberle-Pelloth, K Eftring, J Ekengren, I Errekalde, S Evans, A Foucart, L Fokkema, L François, M French, E Forssell, C Gingell, C Gonçalves, H Gökmen Özel, A Grimsley, G Gugelmo, E Gyüre, C Heller, R Hensler, I Jardim, C Joost, M Jörg-Streller, C Jouault, A Jung, M Kanthe, N Koç, I L Kok, T Kozanoğlu, B Kumru, F Lang, K Lang, I Liegeois, A Liguori, R Lilje, O Ļubina, P Manta-Vogli, D Mayr, C Meneses, C Newby, U Meyer, S Mexia, C Nicol, U Och, S M Olivas, C Pedrón-Giner, R Pereira, K Plutowska-Hoffmann, J Purves, A Re Dionigi, K Reinson, M Robert, L Robertson, J C Rocha, C Rohde, S Rosenbaum-Fabian, A Rossi, M Ruiz, J Saligova, A Gutiérrez-Sánchez, A Schlune, K Schulpis, J Serrano-Nieto, A Skarpalezou, R Skeath, A Slabbert, K Straczek, M Giżewska, A Terry, R Thom, A Tooke, J Tuokkola, E van Dam, T A M van den Hurk, E M C van der Ploeg, K Vande Kerckhove, M Van Driessche, A M J van Wegberg, K van Wyk, C Vasconcelos, V Velez García, J Wildgoose, T Winkler, J Żółkowska, J Zuvadelli, A MacDonald, A Pinto, S Adams, K Ahring, H Allen, M F Almeida, D Garcia-Arenas, N Arslan, M Assoun, Y Atik Altınok, D Barrio-Carreras, A Belanger Quintana, S M Bernabei, C Bontemps, F Boyle, G Bruni, M Bueno-Delgado, G Caine, R Carvalho, A Chrobot, K Chyż, B Cochrane, C Correia, K Corthouts, A Daly, S De Leo, A Desloovere, A De Meyer, A De Theux, B Didycz, M E Dijsselhof, K Dokoupil, J Drabik, C Dunlop, W Eberle-Pelloth, K Eftring, J Ekengren, I Errekalde, S Evans, A Foucart, L Fokkema, L François, M French, E Forssell, C Gingell, C Gonçalves, H Gökmen Özel, A Grimsley, G Gugelmo, E Gyüre, C Heller, R Hensler, I Jardim, C Joost, M Jörg-Streller, C Jouault, A Jung, M Kanthe, N Koç, I L Kok, T Kozanoğlu, B Kumru, F Lang, K Lang, I Liegeois, A Liguori, R Lilje, O Ļubina, P Manta-Vogli, D Mayr, C Meneses, C Newby, U Meyer, S Mexia, C Nicol, U Och, S M Olivas, C Pedrón-Giner, R Pereira, K Plutowska-Hoffmann, J Purves, A Re Dionigi, K Reinson, M Robert, L Robertson, J C Rocha, C Rohde, S Rosenbaum-Fabian, A Rossi, M Ruiz, J Saligova, A Gutiérrez-Sánchez, A Schlune, K Schulpis, J Serrano-Nieto, A Skarpalezou, R Skeath, A Slabbert, K Straczek, M Giżewska, A Terry, R Thom, A Tooke, J Tuokkola, E van Dam, T A M van den Hurk, E M C van der Ploeg, K Vande Kerckhove, M Van Driessche, A M J van Wegberg, K van Wyk, C Vasconcelos, V Velez García, J Wildgoose, T Winkler, J Żółkowska, J Zuvadelli, A MacDonald

Abstract

Background: In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe.

Methods: A cross sectional questionnaire (survey monkey®) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis.

Results: Weaning started at 17-26 weeks in 85% (n = 81/95) of centres, >26 weeks in 12% (n = 11/95) and < 17 weeks in 3% (n = 3/95). Infant's showing an interest in solid foods, and their age, were important determinant factors influencing weaning commencement. 51% (n = 48/95) of centres introduced Phe containing foods at 17-26 weeks and 48% (n = 46/95) at >26 weeks. First solids were mainly low Phe vegetables (59%, n = 56/95) and fruit (34%, n = 32/95).A Phe exchange system to allocate dietary Phe was used by 52% (n = 49/95) of centres predominantly from Northern and Southern Europe and 48% (n = 46/95) calculated most Phe containing food sources (all centres in Eastern Europe and the majority from Germany and Austria). Some centres used a combination of both methods.A second stage Phe-free L-amino acid supplement containing a higher protein equivalent was introduced by 41% (n = 39/95) of centres at infant age 26-36 weeks (mainly from Germany, Austria, Northern and Eastern Europe) and 37% (n = 35/95) at infant age > 1y mainly from Southern Europe. 53% (n = 50/95) of centres recommended a second stage Phe-free L-amino acid supplement in a spoonable or semi-solid form.

Conclusions: Weaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development.

Keywords: Infant; Phe-free infant formula; Phenylalanine; Phenylketonuria; Weaning.

Figures

Fig. 1
Fig. 1
The age of introduction of a second stage protein substitute in each age category by centres in different geographical regions.

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Source: PubMed

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