Conclusie voor de praktijk - Cystic fibrosis

Cystic fibrosis

3.8 Conclusie voor de praktijk

Cystic fibrosis is een erfelijke autosomaal recessieve stofwisselingsstoornis. Via DNA-diagnostiek is voorlichting hierover in families mogelijk geworden. Het niet goed functioneren van chloridekanalen in de celmembraan is de oorzaak van de klinische verschijnselen. De diagnose komt bij de meeste patiënten via hielprik-screening aan het licht. De behandeling is symptomatisch en gericht op het na-streven van een optimale voedingstoestand in combinatie met het bestrijden en voorkómen van luchtweginfecties en longbeschadiging. Multidisciplinaire aanpak en begeleiding in een CF-centrum heeft de levensverwachting, hoewel nog altijd beperkt, doen toenemen en ook de kwaliteit van leven verbeterd. De diëtist is lid van het CF-team en houdt zich specifiek bezig met alle aspecten van de dieetsamen-stelling, de energie-inneming, de pancreasenzymsubstitutie en het voedingspatroon in relatie tot het leeftijdsadequaat eetgedrag van het kind, opdat de voedingstoe-stand optimaal blijft.

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R. van Berkel ()

diëtist, Groningen, The Netherlands

In document Informatorium voor Voeding en Diëtetiek (pagina 68-71)