Adult Cystic Fibrosis

Authors: Frank Edenborough, Shammi L Ramlakhan / Editor: Jason M Kendall / Reviewer: Kaja Mohammad Rasheed / Codes: A2 / Published: 15/10/2019

Describing the pathophysiology of cystic fibrosis.

After completing this session you will be able to:

• Describe the pathophysiology of cystic fibrosis
• Appreciate that patients with cystic fibrosis are surviving longer
• Describe the chronic complications of cystic fibrosis and identify those conditions most likely to result in presentation to the ED
• Recognise unique problems with cystic fibrosis that may present to the emergency department
• Identify scenarios where emergency department treatment differs between cystic fibrosis patients and non-cystic fibrosis patients

References:

1. O’Sullivan BP, Freedman SD. Cystic Fibrosis. Lancet 373 (2009): 1891-1904.
2. At a glance. UK CF Registry annual data report for 2018. (Last accessed September 2019)
3. Cystic fibrosis: Diagnosis and Management (NG78); National Institute for Health and care Excellence (NICE) October 2017. (Last accessed September 2019)
4. Yankaskas JR, Marshall BC, Sufian B et al. Cystic Fibrosis Adult Care – Consensus Conference Report. Chest 125 (2004): 1S-39S.
5. Davies JC, Alton EW, Bush A. Cystic Fibrosis. BMJ 355 (2007): 1255-1259.
6. Quinton PM. Cystic Fibrosis: inpaired bicarbonate secretion and mucoviscidosis. Lancet 372 (2008): 415-417.
7. Bell SC, Robinson PJ. Exacerbations in Cystic Fibrosis. Thorax 62 (2007): 723-732.
8. Gershman AJ, Mehta AC, Infeld M, Budev MM. Cystic Fibrosis in Adults: an overview for the internist. CCJM 73 (2006): 1065-1074.
9. Flume, PA. Pneumothorax in Cystic Fibrosis. Chest 123 (2003): 217-221.
10. Wilschanski M, Durie PR. Patterns of GI disease in adulthood associated with mutations in the CFTR gene. Gut 56 (2007): 1153-1163.
11. Davis PB, Drumm M, Konstan MW. Cystic fibrosis. Am J Resp Crit Care Med 154 (1996): 1229-1256.
12. Aris RM, Renner JB, Winders AD et al. Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis. Ann Intern Med 128 (1998): 186-193.
13. Smyth A, Elborn JS. Exacerbations in cystic fibrosis: 3. Management. Thorax 63 (2008): 180-184.
14. Flume PA, Mogayzel PJ, Robinson KA et al. Cystic Fibrosis Pulmonary Guidelines. Treatment of Pulmonary Exacerbations. Am J Respir Crit Care Med 180 (2009): 802808.
15. Rubin BK. Overview of cystic fibrosis and non-CF bronchiectasis. Semin Respir Crit Care Med 24 (2003): 619628.
16. Flume PA, Mogayzel PJ, Robinson KA et al. Cystic Fibrosis Pulmonary Guidelines: Pulmonary Complications: Hemoptysis and Pneumothorax. Am J Respir Crit Care Med 182 (2010): 298306.
17. Leeds Regional Adult and Paediatric Cystic Fibrosis Unit. Joint pain and arthritis in cystic fibrosis. Cystic Fibrosis Medicine. September 2019. (Last accessed September 2019).
18. Etherington C, Jones E, Peckham D et al. A review of 168 totally implantable venous access devices [TIVADs] inserted over an 8 year period [1995-2002] in a Regional Adult CF Centre. J Cyst Fibros 4(Suppl 1 (2005): S14.
19. Giannouli E, Sharma S, Maycher B. Cystic Fibrosis, Thoracic. emedicine. 5 Nov 2015. View link. (Last accessed September 2019).
20. Driscoll BR, Howard LS, Davison AG. BTS guideline for emergency oxygen use in adults. Thorax 63 (2008): S6.
21. Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev, 21 January 2009: CD002769.
22. Halfhide C, Evans HJ, Couriel J. Inhaled bronchodilators for cystic fibrosis. Cochrane Database of Syst Rev, 19 October 2005: CD003428.
23. MacDuff A, Arnold A, Harvey J. Management of spontaneous pneumothorax: British Thoracic Society pleural disease guideline 2010. Thorax 65 (2010): ii18-31.
24. Leeds Regional Adult and Paediatric Cystic Fibrosis Units. Treatment of Distal Intestinal Obstruction Syndrome. cfmedicine. April 2008. View link. (Last accessed September 2019).

Please use the following resources for more information:

• European CF Diagnostic Working Group view link. (Last accessed September 2019).
• Guidelines for Diagnosis of Cystic Fibrosis in Newborns through Older Adults: Cystic Fibrosis Foundation Consensus Report Farrell, Philip M. et al. The Journal of Pediatrics, Volume 153, Issue 2, S4 – S14. (Last accessed September 2019).