Care

Question

What are points of attention for care and follow up of people with cystic fibrosis

Answer

This is a very general questions. The answer is thus a complete text book.

In general one can state that regular follow up is very important. Prevention of specific complications is key. For instance regular screening of at least 3 monthly culture of the airway by upper airway sample or sputum to check for early Pseudomonas infection so that this or another infection can be treated intensively.

European guidelines states at least 3-monthly follow up in a multidisciplinary clinic specialized in CF is necessary. At each time a thorough questioning of the complaints and problems of the patients is done (history), a physical exam is done and routine treatments are reviewed and there is specific attention to nutritional status, lung function, evaluation of lung infection and possibly occurrence of specific complications. Depending on the results the treatments will be adapted. General information on follow up and treatment of people with cystic fibrosis, you can read in the following articles:

- Kerem, E., S. Conway, et al. (2005). "Standards of care for patients with cystic fibrosis: a European consensus." J Cyst Fibros 4(1): 7-26.
- Mayell, S. J., A. Munck, et al. (2009). "A European consensus for the evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis." J Cyst Fibros 8(1): 71-8.
- J.S. Elborn et al. Implementation of European standards of care for cystic fibrosis - provision of care Journal of Cystic Fibrosis 8(2009) 348-355
- Borowitz D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa KA, Spear SL, Michel SH, Parad RB, White TB, Farrell PM, Marshall BC, Accurso FJ; Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009 Dec; 155(6)Suppl:S73-S93.
- Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D; Cystic fibrosis adult care: Consensus conference report. Chest 2004 Jan;125(1 Suppl):1S-39S.

www.ecfs.eu
www.cff.org



Kind regards
K. De Boeck

20.01.2011