Lung function in the aging Swedish cystic fibrosis population

Abstract

Swedish Cystic Fibrosis (CF) care follows international guidelines in general. The only difference in our CF care package since 25 years is the physiotherapy regimen. Airway clearance therapy has since the early 1980s, from the day of diagnosis, been based upon daily physical exercise in conjunction with techniques for transporting and evacuating mucus. Postural Drainage+/-percussion and vibration has not been used in any age. The aim of this study was to evaluate our CF care package. Lung function data from the start of the study and with a 3-year interval were collected in the entire Swedish CF population > or =7 years old. Data were analysed for the age groups 7-17 and > or =18 years of age. Change of lung function over the study period was calculated. The impact of chronic Pseudomonas aeruginosa (Pa) colonisation and basal FEV(1)%p was also evaluated in a linear mixed model. Data from 99% of the country-wide CF population were available at inclusion. Mean FEV(1)%p was 90+/-21 vs 73+/-26 in the different age groups and mean VC%p was 94+/-18 vs 91+/-20. Forty percent of the adult group was > or =30 years old. Overall, 41% were chronically Pa colonised. Mean annual FEV(1)%p rate of decline was 0.77 and 0.64 in the different age groups. Lung function among Swedish CF patients is good and annual rate of decline low, even in an old cohort. The large proportion of adult patients emphasises future demands on CF care.

The impact of chronic pseudomonal infection on pulmonary function testing in individuals with cystic fibrosis in Pretoria, South Africa

“…[4,[20][21][22] In a study conducted among Swedish CF patients, overall lung function was well preserved with a low rate of annual decline. However, a more rapid lung function decline was evident in the group colonised by PA. [9] Similar results were obtained in the two studies performed in the Western Cape, South Africa, [10,11] in which the study populations with CF more closely resembled the current SBAH study. Pulmonary function decline may be expected to be more significant in CF subjects living in a developing country, where healthcare resources may not be as available as in the developed world.…”

“…[10,15,16] The median age of the patients colonised by PA (19.9 years) in this study is significantly older than in many European centres, but this age is not the age of first colonisation. [17,18] The high rate of PA colonisation in the study subjects (51.7%) is greater than in some international studies, [9] but is comparable to local SA studies. [10,11] Even at study entry, patients who were colonised by PA had lower spirometric indices compared with patients who were not colonised at that stage (p=0.014, 0.0014 and 0.0061 for FEV 1 , FVC and FEF , respectively).…”

“…[7] The rate of annual pulmonary function decline, as well as the associated or aetiological factors, have been explored in some population groups. [8,9] In South Africa, only two studies of pulmonary function decline in CF have been published. [10,11] The aim of the current study was to compare the association between pulmonary function measurements and the presence of bacterial colonisation by PA and SA in patients with CF attending the clinic at Steve Biko Academic Hospital (SBAH), Pretoria, South Africa.…”

Physical training: vital for survival and quality of life in cystic fibrosis

“…4). The respiratory pump can be utilised even further when exercises are carried out in horizontal, rather than erect, positions, since gravity influences regional ventilation distribution and regional functional residual capacity differently in horizontal positions [15,19]. The fact that gravity has effects on mucus clearance during airway clearance therapy has been confirmed, although a study has shown that while lying on their side, most patients had better clearance effect in the dependent lung [19].…”

“…The results are often impressive, but to what extent patients can preserve the achieved exercise level is not discussed. It should be at least as important to assess to what extent patients can retain the effects of training, especially given that long-term studies have reported that physical exercise reduces the rate of lung function deterioration [13][14][15].…”