CF nurses would like better guidance and training in looking after patients who are dying. Results of a survey carried out by British nurses in south Wales, and reported at the recent North American Cystic Fibrosis Conference in Seattle, showed that the vast majority of nurses have great difficulty balancing the needs of dying patients with other aspects of their job.
Eight out of 10 of the CF nurse specialists at 45 CF centres in the UK who answered the questionnaire experienced such problems. Similar numbers reported the huge demands being placed on them; for example, 83% provided bereavement support and 85% ran an out of hours service without any extra payment.
Yet, two thirds of respondents had received no special training in providing terminal care, despite their involvement as key workers. Nine out of 10 wanted more information to help them with their stressful role.

· A major international trial is underway in Canada and the UK to try and confirm whether high dose ibuprofen can reduce declining lung function in CF patients by the 40-60% demonstrated in earlier research. Lingering doubts about possible adverse GI effects of long term treatment have prevented ibuprofen from being more widely used. But data from the American CF registry show rates of GI ulcers and GI bleeding in CF patients taking ibuprofen only slightly higher than those occurring in non-users.

· Early efforts to block a key enzyme of neutrophil activity, neutrophil elastase, with a genetically engineered form of alpha-1-antitrypsin, appear promising. In a phase 2 study, reported by a team from Belfast and Leeds, patients on active treatment had fewer pulmonary exacerbations, improved lung function and felt less tired than those given a placebo.

· An anti-pseudomonal vaccine is being studied in a multicentre, international trial. The vaccine, against antigens on the bacterium's flagella, is being administered to over 400 patients to see whether chronic infection can be delayed or prevented. Results are expected in about three years.

· Results of a study of a new vaccine against respiratory syncitial infection in children with CF should be available more quickly. Three hundred CF children aged 1-12, seropositive for RSV, were immunised this Autumn to see if their risk of infection could be reduced over the winter.

· The benefits of hypertonic saline prior to physiotherapy are being tested in a number of controlled trials, including a study which will compare the effects on mucociliary clearance and lung function with those achieved with DNase.

· Trials of gentamicin are investigating its potential in improving chloride transport mechanisms. Small scale studies of chloride movement in CF patients have already demonstrated improved secretory mechanisms and further studies are investigating the benefits of topical and iv gentamicin on chloride transport and respiratory exacerbations in patients with a

Adolescent CF patients gain the largest improvements in lung function following long term, intermittent treatment with a novel nebuliser formulation of tobramycin, developed by PathoGenesis and soon to be licensed in the UK. But patients of all ages get substantial benefits from treatment.

Final results of a two year phase III trial of '1-month on, 1-month off' tobramycin solution for inhalation, via Pari LC Plus jet nebuliser, in CF patients with Pseudomonas aeruginosa infection showed significant increases in FEV₁ and reductions in hospitalisation and need for iv and oral courses of antibiotics.
The overall increase in FEV₁ in patients taking nebulised tobramycin was nearly 5%, rising to over 14% in adolescents aged 13-18. In this group there was also a mean weight gain of 4.4kg over baseline. In contrast, patients who received a placebo during the first six months of the study never recovered the resulting loss in FEV₁ even after they were switched to active treatment during the 18 month open label phase of the study.
'We saw benefits at all ages but this was a remarkable response in adolescent patients who were at an age when we generally tend to lose control of their disease,' explained Professor Richard Moss, head of paediatrics at Stanford University School of Medicine, Palo Alto, USA.
'The fact that improving lung function and inflammation can produce such an effect on overall health bodes well for future

Many parents hide the stress of looking after a child with CF, but results of a new survey carried out at Great Ormond Street Hospital for Children, London, show that it can have a significant impact on their physical and mental health.
Less than half of 65 parents of CF children were able to return to full time employment after having their child, putting financial stresses on families and affecting parental self esteem. In contrast, two thirds of a very similar group of 41 parents who did not have a child with CF were able to resume their normal working lives after childbirth.
Parents of CF children also scored worse on a range of general health, mental health and quality of life measures than the control families. However, they scored well on coping scales suggesting that, while recognising that they had problems, they were able to deal with them.
'We are expecting parents to carry out an increasingly complex set of treatments at home which we have learned through a whole career in nursing,' pointed out Susan Madge, 'As clinicians, we should be aware that parents are under pressure. They may say that everything is fine when they come to clinic, but we need to look behind their answers and consider what's really happening to families so that we can intervene before the pressure gets too great,'