US children with cystic fibrosis have better lung function compared to UK counterparts

There are significant differences in the lung function of children and young adults in the United States (US) who have cystic fibrosis compared with those in the United Kingdom (UK). A paper published today in BMJ Thorax shows that young people in the US have better lung function compared to their UK counterparts.

The study used 2010 data from two countries with well-developed yet different healthcare systems, and it shows that when US and UK patients aged six to 25 years of age are compared, lung function is on average 3.31 percent higher in US patients.

In both countries a number of advances in the care and clinical outcomes of people with cystic fibrosis have occurred over the last two decades, but people with cystic fibrosis are managed differently in the US and UK. In the UK, patients have universal access to care via the National Health Service, but in the US that is not the case. 

The paper does not explore the reasons behind the difference in lung function in detail, but it notes that in the UK chronic pulmonary therapies had been used much less frequently to treat young people compared to the US. The most striking differences were noted in the use of hypertonic saline and rhDNase in both children and adults. Chronic macrolide antibiotics were also used less frequently in the UK.

A further possible explanation for the difference in lung function is that in the US cystic fibrosis is treated only at specialist centres while in the UK cystic fibrosis care is managed between local hospitals and specialised units. In some parts of the UK young patients only visit a specialist centre once a year and then visit their local paediatric hospital the rest of the time, where they may not be seen by a cystic fibrosis expert.

The report recommends that further studies are required to determine whether there needs to be earlier and more aggressive treatment of children with cystic fibrosis in the UK. Further research is also needed to determine whether any changes to care since 2010 have had an impact on patient outcomes.

One of the report’s authors, Professor Diana Bilton, Consultant Physician at Royal Brompton Hospital, and Chair of the UK Cystic Fibrosis Registry steering committee, says:

This paper shows the power of Registry data in helping us ensure we are giving the best to our patients. At this stage there is a lot more work to do to identify the cause of the differences. It is important that we all look at our practice as a result of this paper and check whether we are using the medicines already available in the best way. We also need to look at how we deliver care.

Janet Allen, Director of Research and Care at the Cystic Fibrosis Trust said:

Cystic fibrosis is a degenerative condition which attacks the respiratory organs and destroys the lungs. Fifty years ago, when the Cystic Fibrosis Trust was established, 80% of people with cystic fibrosis did not reach their fifth birthday. Today, over half of patients live into their 30s. But it is still unacceptable that so many people die so early.

Comparing clinical outcomes between countries can be informative where clear differences in care models and treatment approaches occur and it is important that we learn from other countries.

We need to carry out further investigations into the reasons for the differences in lung function between young UK and US patients, and determine whether this is just a one off for the 2010 study cohort.

The Trust is also continuing to fund research into innovative and live-changing treatments that will not only ensure that all patients with cystic fibrosis receive the very best care and treatment, but that they will one day have the same life chances as those who don’t have the condition.