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Cystic fibrosis; een vergrijzend ziektebeeld?

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Tijdschrift voor kindergeneeskunde

Summary

Cystic fibrosis (cf) is the most frequent occurring hereditary lethal disease in Western countries. Improvement of treatment strategies in cf have resulted in major improvements of life expectancy. Several decades ago cf was treated exclusively by paediatricians, nowadays the median life expectancy is between 30 and 35 years. Many patients are treated by pulmonologists and other medical specialists for adults. The improved survival has led to the emergence of new complications of the disease, like allergic bronchopulmonary aspergillosis (abpa), cf-related liver diseases, cf-related diabetes mellitus, and osteoporosis. Additionally, patients wish to become parents themselves. Each of these aspects ask attention of both paediatricians (information and prevention) and medical specialists for adults (treatment). Intensive collaboration between these specialists in cf centers and structured transition of care is of urgent importance.

Samenvatting

Cystic fibrosis (cf) is de meest voorkomende erfelijke letale aandoening. Vooruitgang in de behandeling van cf heeft tot een sterke verbetering van de levensverwachting geleid. Enkele decennia geleden werd cf nog uitsluitend door de kinderarts behandeld, thans bedraagt de mediale levensverwachting 30 tot 35 jaar en worden veel patiënten behandeld door longartsen en andere specialisten voor volwassenen. Door de verbeterde overleving komen complicaties van de ziekte naar voren die voorheen niet relevant waren. Te denken valt aan langetermijncomplicaties zoals allergische bronchopulmonale aspergillose (abpa), cf-gerelateerde leverziekten, cf-gerelateerde diabetes mellitus en osteoporose. Met de toenemende levensverwachting ontstaat bij patiënten met cf ook de wens naar kinderen. Elk van deze aspecten vraagt aandacht van zowel kinderartsen (voorlichting en preventie) als van specialisten voor volwassenen (behandeling). Een intensieve samenwerking tussen deze specialisten in behandelcentra en gestructureerde overdracht van zorg (transitie) is dan ook van groot belang.

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Authors and Affiliations

  1. www.kindergeneeskunde.bsl.nl

    C. K. van der Ent, I. Drubbel, A. M. M. de Vrankrijker, H. G. M. Arets & H. G. M. Heijerman

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  1. C. K. van der Ent
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  2. I. Drubbel
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  3. A. M. M. de Vrankrijker
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  4. H. G. M. Arets
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  5. H. G. M. Heijerman
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Additional information

Dr. C.K. van der Ent, kinderarts-pulmonoloog, mw. drs. I. Drubbel, mw. drs. A.M.M. de Vrankrijker en dr. H.G.M. Arets, afdeling Kinderlongziekten, umcu, cf-Centrum Utrecht. Dr. H.G.M. Heijerman, afdeling Longziekten, Haga Ziekenhuis, Den Haag.

Correspondentieadres: Dr. C.K. van der Ent, afdeling Kinderlongziekten, umcu, KH 01.419.0, Postbus 85090, 3508 AB Utrecht, tel: 030-2504690, fax: 030-2504747, e-mail k.vanderent@umcutrecht.nl.

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van der Ent, C.K., Drubbel, I., de Vrankrijker, A.M.M. et al. Cystic fibrosis; een vergrijzend ziektebeeld?. KIND 74, 276–282 (2006). https://doi.org/10.1007/BF03061646

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