Clinical experiences in transitioning PKU patients from second to third stage protein substitutes: A global survey

Yılmaz Nas Ö.

SSIEM Annual Symposium 2023, Yerushalayim, İsrail, 29 Ağustos - 01 Eylül 2023, ss.216, (Özet Bildiri)

  • Yayın Türü: Bildiri / Özet Bildiri
  • Basıldığı Şehir: Yerushalayim
  • Basıldığı Ülke: İsrail
  • Sayfa Sayıları: ss.216
  • Ankara Yıldırım Beyazıt Üniversitesi Adresli: Evet

Özet

Background: We assessed the global clinical practices in transitioning young children with phenylketonuria (PKU) from second to third stage protein substitutes (PS).

Methods: A 31 item questionnaire on protein substitute transitioning was emailed to 298 professionals wirking with PKU.

Results: 97 responses were received from 27 countries. The majority (69%) of centres managed children and adults with PKU. The common ages for introducing a third-stage PS were 3-5 (n=37, 39%), 6-10 (n=32, 33%), and >10 years (n=12, 12%). Transitioning age was influenced by manufacturers recommendations, school convenience, taste preferences, nutritional requirements, formula acceptance, and their availability. Transitioning methods varied: 39% (n=37) preferred a step-by-step approach, 30% (n=29) an immediate changeover, and 29% (n=28) an individualized approach. Mean duration of transition varied, with 89% (n=86) reporting a median of 2.1 (range 0-12) months and 11% (n=11) reported according to individual needs. The most common third- stage PS were ready-to-drink (n=73, 75%), and powder (n=55, 57%). Third-stage PS choice was mainly influenced by ease of use (n=65, 67%), patient preference (n=64, 66%), and nutritional content (n=53, 55%). Common barriers to third-stage PS were taste, smell, or texture (n=65, 67%), aversion to new PS (n=64, 66%), and lack of parental consistency/persistence (n=32, 33%). Common facilitators to improve the transition process were child/parents motivation (n=77, 79%), improved taste, smell, or texture of the third-stage PS (n=63, 65%), and effective parental management strategies (n=55, 57%). Useful resources needed were: educational videos (n=49, 51%), step-by-step charts/books (n=36, 37%), guidance books (n=32, 33%), and child reward systems (n=32, 33%).

Conclusions: This study highlights the requirement for guidance and resources to assist healthcare professionals and parents/caregivers in supporting the transition of PKU patients to third-stage PS.