STANDARDS AND GUIDELINES

2001 Standards for the Clinical care of Children and Adults with Cystic Fibrosis in the UK. The CF Trust Clinical Standards and Accreditation Group. London. Cystic Fibrosis Trust, May 2001.
A CF Trust committee of 17 people representing a wide range of interests chaired by Dr Jim Littlewood. After a number of meetings and a great deal of discussion, the principles of good care were agreed and have stood the test of time being widely used in CF centres both in the UK and Europe - before the European Standards of Care were agreed and published (Kerem et al. 2005 below). The document is under revision in 2009.

2004 Doring G, Hoiby N, Consensus Study Group. Early intervention and prevention of lung disease in cystic fibrosis: a European consensus. J Cystic Fibrosis 2004; 3:67-91.
One of a number of valuable Artimino conference consensus reports organised by Gerd Doring, the then President of the ECFS. Delegates representing most countries in Europe were invited to a meeting in Artimino to consider a particular area of interest and produce a consensus report. This one, on early intervention, was particularly relevant in view of the increasing introduction of neonatal CF screening. (Full text on the ECFS website (www.ecfs.eu).

2002 Sinaasappel M, Stern M, Littlewood J, Wolfe S, Steinkamp G, Heijerman H, Robberecht E, Doring G. Nutrition in patients with cystic fibrosis: a European Consensus. J Cyst Fibros 2002; 1:51-75.
The outcome of a European CF Society Consensus Group at Artimino Italy in March 2001 involving 33 experts on nutrition in patients with cystic fibrosis. The contents give an idea of the current nutritional management of people with CF at the time. All the ECFS consensus reports can be seen in full on the ECFS website (www.ecfs.eu).

2005 Kerem E, Conway S, Elborn S, Heijerman H for the Consensus Committee. Standards of care for patients with cystic fibrosis: a European consensus. J Cystic Fibrosis 2005; 4:7-20.
An important document developed at an ECFS Artimino consensus meeting. The document is now generally accepted throughout Europe and provides a basis for developing optimal CF care and a standard against which care in individual centres can be judged. The full text of the article is on the ECFS website (www.ecfs.eu).
I was invited to write a commentary on this article (Littlewood, 2005 below).

2007 Flume PA., O'Sullivan BP, Robinson KA, Goss CH, Mogayzel PJ Jr, Willey-Courand DB, Bujan J, Finder J, Lester M, Quittell L, Rosenblatt R, Vender RL, Hazle L, Sabadosa K, Marshall B. Cystic Fibrosis Foundation, Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Amer J Respir Crit Care Med 2007; 176: 957-969.
In 2005 the Cystic Fibrosis Foundation established a committee to examine the clinical evidence for each therapy used for CF and to provide guidance for the prescription of these therapies. The committee members developed and refined a series of questions related to drug therapies used in the maintenance of pulmonary function. Questions were addressed in one of three ways, based on available evidence: (1) commissioned systematic review, (2) modified systematic review, or (3) summary of existing Cochrane reviews. The strength of the evidence and the estimate of benefit were recorded and the recommendations graded. Grade A- “good evidence and substantial benefit”. Grade B – “fair evidence, benefit outweighs harm”. Grade C – “no recommendation, benefits and harm balance too close”. Grade D – “fair evidence not effective or harm outweighs benefit”. Grade I – “insufficient evidence”.
The treatments received the following grades -
Nebulised tobramycin - grade A for severe, grade B for mild persistent Pseudomonas infection; evidence for other inhaled antibiotics “insufficient”;(surprisingly even in 2005 the important area of early Pseudomonas treatment was not dealt with); rhDNase grade A recommendation; hypertonic saline grade B; inhaled and oral steroids both grade D; non-steroidal anti-inflammatory drugs grade B; leukotriene modifiers insufficient evidence ; macrolides grade B; prophylactic anti-staphylococcal antibiotics grade D; ß-agonist bronchodilators grade B; anti-cholinergic bronchodilators Grade I; n-acetylcysteine grade I.
Dr Patrick Flume (figure 44) works at the Medical University of South Carolina.
The treatments that have been the subject of a CF Foundation Phase III trial are awarded either A or B recommendation. It is quite surprising that even in 2007 the early eradication treatment of Pseudomonas is not considered in a document on inhaled therapy. Not all would agree with the grading of prophylactic anti-Staphylococcal antibiotics for which there is a perfectly good European trial (Weaver et al, 1995 above) and a wealth of practical experience.

2008 Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, Rock MJ, Campbell 3rd. Guidelines for the diagnosis of cystic fibrosis in newborns through older adults. Cystic Fibrosis Foundation Consensus Report. J Pediatr 2008; 132:S4-S14.
A consensus report to provide guidance for the diagnosis of both infants with positive newborn screening results and older patients presenting with an indistinct clinical picture. The recommendations involve combination of clinical presentation, laboratory testing and genetics to confirm a diagnosis of CF. This supplements the previous report (Rosentein & Cutting, 1998 above).

2009 Antibiotic Treatment for Cystic Fibrosis. Report of the UK Cystic Fibrosis Trust Antibiotic Working group. Third Edition. Cystic Fibrosis Trust. London, 2009.
Smyth A (Chairman), Bilton D, Bush A, Clarke S, Conway S, Dack K, Dodd M, Denton M, Foweraker J, Hart A, Southern K, Walshaw M.
A very detailed review of antibiotic use in treating CF - a consensus view taking note of all the available published evidence and the wide experience of the group. Full text available on the CF Trust website (www.cftrust.org.uk).