In the National CF Centre in Denmark, in the mid-1970s, it was finally established that P. aeruginosa chronic infection was associated with a poor prognosis, and they instituted a regimen of three monthly intravenous antibiotics that had a major impact on survival. This approach was much debated in the UK and not taken up widely; nevertheless, treatment of P. aeruginosa became a major priority. In 1981, the Danish centre also established patient segregation for those chronically infected with P. aeruginosa. This took many years to become universal in the UK, and we still took groups of children from Great Ormond Street Hospital (it had changed its name in the mid-1990s) on skiing holidays in the late 1990s.

In 1981, the use of nebulised antipseudomonal antibiotics with gentamicin and carbenicillin was established at the Brompton Hospital by Margaret Hodson; early eradication with colomycin nebulisers was published from Leeds in 1985, and colomycin with ciprofloxacin for eradication came from the Danish centre in 1991; it was not until 2003, however, that the USA followed European practice after the nebulised tobramycin (TOBI) study.

The first heart-lung transplant in an adult with CF was carried out in 1985 by Sir Magdi Yacoub in Papworth Hospital, Cambridge; and in a child at the Hospital for Sick Children, Great Ormond Street in 1988. They reported on the first 11 children transplanted in ADC in 1991.18 Fortunately, with the newer therapies, transplants in children are almost a thing of the past in the UK.

In 1979, immunoreactive trypsin in dried blood spots was reported as a reliable screening tool.19 ADC published an early review of the topic in 1982 from Cardiff in which John Dodge and Henry Ryley called for a national screening programme.20

In the UK, newborn screening became established very slowly, with its use in a few regions only (initially in East Anglia in 1980) and it eventually became universal in the UK in 2007. Much of the evidence for the benefit of newborn screening came from the randomised controlled trial of screening in Wisconsin led by Pat Farrell, which started recruitment in 1985. Undoubtedly, the health of children with CF improved as a result of this. The technique for prenatal screening was published in Edinburgh in 1985.

Nutritional care improved in the 1980s. Pancreatic enzymes that were resistant to gastric acid due to an enteric coating were introduced in the early 1980s, allowing children to tolerate a diet with a normal fat intake. For those requiring enteral feeding, use of gastrostomies started in the early 1980s. More notice was taken of fat-soluble vitamin deficiencies. In 1976, a letter in ADC suggested the use of cimetidine (an H2 blocker antacid) improved pancreatic enzyme efficacy and they showed a reduction in faecal fat excretion in four out of five children.21 This shows how a small pilot study reported as a research letter can be important, as protein pump inhibitors are still used in CF care when enzyme doses are high.

In 1981, there was an important breakthrough when Mike Knowles in Chapel Hill, North Carolina, USA established that the basic cellular defect in CF was due to a primary epithelial dysfunction, by measuring transepithelial potential differences in the nasal mucosa of 24 patients, and in the lower respiratory tract in four patients.22

In 1985, ADC published a personal practice paper written by Margaret Mearns,23 which was the first of many update reviews of CF that ADC has published.