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PubMed1995

Progression of pulmonary disease after disappearance of Pseudomonas in cystic fibrosis.

Once cystic fibrosis (CF) patients become chronically colonized, eradication of Pseudomonas aeruginosa (PA) is rare. We report five patients, each colonized for at least 6 yr, whose subsequent cultures did not reveal PA or any other gram-negative pathogen for at least 2 yr. Two patients harbored yeast, normal throat flora, and occasional colonies of Aspergillus fumigatus, but no PA, Haemophilus influenzae, or other gram-negative pathogens. In two patients, sputum cultures revealed Staphylococcus aureus. Sputum smear showed no gram-negative organisms in any patient. Antipseudomonal antibody titers in all patients decreased during the noncolonized period. However, titers increased in four other CF patients who had continued PA colonization. Despite disappearance of PA, four of five patients had clinical progression of lung disease with deterioration of FEV1. We conclude that (1) PA, even after it has chronically colonized the airways of CF patients, can occasionally disappear; and (2) lung disease can progress despite the disappearance of PA. Thus, the elimination of a milieu favorable to PA, such as might be anticipated with therapy directed at the basic defect, may not be sufficient to halt the pulmonary disease progression in CF.

Adult↗

PubMed2004

Pseudomonas aeruginosa regulates flagellin expression as part of a global response to airway fluid from cystic fibrosis patients.

Cystic fibrosis (CF) patients are highly susceptible to chronic lung infections by the environmental bacterium Pseudomonas aeruginosa. The overproduction and accumulation of dehydrated viscous respiratory mucus and excessive inflammation represents a defining feature of CF and constitutes the major environment encountered by P. aeruginosa during chronic infections. We applied whole-genome microarray technology to investigate the ability of P. aeruginosa to respond to signals found in muco-purulent airway liquids collected from chronically infected CF patients. Particularly notable was the activation of the Rhl-dependent quorum-sensing (QS) network and repression of fliC, which encodes flagellin. Activation of the Rhl branch of the QS network supports the observation that QS molecules are produced in the chronically infected CF lung. The shut-off of flagellin synthesis in response to CF airway liquids was rapid and independent of QS and the known regulatory networks controlling the hierarchical expression of flagellar genes. As flagellin is highly immunogenic and subject to detection by host pattern recognition receptors, its repression may represent an adaptive response that allows P. aeruginosa to avoid detection by host defense mechanisms and phagocytosis during the chronic phase of CF lung infections.

Cystic Fibrosis↗

PubMed1979

Activated complement in the sputum from patients with cystic fibrosis.

14 cystic fibrosis (CF) patients chronically infected with mucoid P. aeruginosa and presenting multiple precipitins in serum against this bacterium (CF + P) and 13 CF patients without P. aeruginosa infection (CF-P) had their plasma and sputum sol phase examined for albumin, Clq. C3/C3c, C4 and C5 by means of electroimmunoassays. Their sputum sol phase was examined also for factor B by rocketimmunoelectrophoresis. C3c was demonstrated in the sputum sol phase but significantly more frequent (p less than 0.01) among the CF + P patients than among the CF-P patients. Factor B was also demonstrated in the sputum sol phase, but no significant difference in frequency could be demonstrated between the CF + P and the CF-P patients. None of the results indicated that a local pulmonary production of complement factors took place. Complement activation was significantly (p less than 0.01) associated with inflammation expressed as increased (formula: see text). The results show the importance of complement mediated inflammation in the pathogenesis of pulmonary tissue damage in patients with CF and support the concept of chronic P. aeruginosa lung infection as an immune complex disease in CF patients.

Adolescent↗

PubMed1996

The effects of recombinant human DNase on neutrophil elastase activity and interleukin-8 levels in the sputum of patients with cystic fibrosis.

In cystic fibrosis (CF), neutrophil-dominated airway inflammation results in high levels of neutrophil elastase (NE). Some of these proteases are sequestered by the large amounts of deoxyribonucleic acid (DNA) present in purulent sputum. Recombinant human deoxyribonuclease (rhDNase), a new treatment in CF, depolymerizes DNA. Our concerns were that this might release proteases bound to DNA, which could be potentially harmful. The in vitro and in vivo effects of rhDNase on NE and interleukin-8 (IL-8) were evaluated. The acute effects of rhDNase were evaluated in CF patients during the first 6 days of treatment. Medium-term effects were evaluated in stable CF patients observed in rhDNase over 6 months. Sputum samples were collected at regular intervals and NE activity was measured by a fluorimetric assay and IL-8 with a radioimmunoassay. In vitro addition of rhDNase resulted in a twofold increase in protease activity and this was reflected in an acute transient rise on initiation of treatment with rhDNase. Medium-term treatment was associated with a decline in NE activity and IL-8. These in vivo results are encouraging, since the increase in protease activity was transient and the trend over 6 months was a reduction in both inflammatory markers.

Cystic Fibrosis↗

PubMed1994

Capillary zone electrophoresis of polymerase chain reaction-amplified DNA fragments in polymer networks: the case of GATT microsatellites in cystic fibrosis.

In cystic fibrosis (CF), the most common mutation, delta F508 (a three-base-pair deletion) accounts for ca. 70% of mutations in the worldwide population. The majority of other mutations (more than 350 reported so far to the Genetic Analysis Consortium) have been detected in single cases, thus rendering quite cumbersome a molecular diagnostic approach for the identification of CF chromosomes. As an alternative, linkage analysis based on intragenic polymorphism can be useful for prenatal diagnosis and CF-carrier detection, provided that the heterozygosity of the allelic forms is very high. For this purpose, DNA microsatellites, consisting of two to epta nucleotide repeat clusters, displaying a high degree of polymorphism, are being increasingly used as markers in linkage studies. Two main allelic forms, one hexameric (111 bp) and one heptameric (115 bp), of a tetranucleotide (GATT) repeat polymorphism, at the junction of intron IVS6a and exon 6b, have been amplified by PCR technology. These two alleles can be separated in a 10-20% T polyacrylamide gradient gel and detected by ethidium bromide staining. As an alternate procedure, these two fragments are efficiently separated by capillary zone electrophoresis in a viscous solution of 6%T linear polyacrylamide and detected by their intrinsic absorbance at 254 nm.

Alleles↗

PubMed2004

Electrogastrography reveals post-prandial gastric dysmotility in children with cystic fibrosis.

OBJECTIVES: Cystic fibrosis patients have a wide spectrum of gastrointestinal disorders. The aim of this study was to investigate the function of gastroenteric neuromusculature and its response to a prokinetic. METHODS: 14 CF children aged 8.6 + 1.3 years were studied by electrogastrography and compared to 10 age-matched controls. A second recording was performed in CF patients after administration of cisapride (0.3 mg/kg). Parameters analyzed were percentage of normal gastric rhythm (2.0 to 4.0 cpm), percentage of tachygastria (4.0 to 9.0 cpm), dominant frequency instability coefficient and power ratio. RESULTS: CF and control groups were not different in age, height or weight. A significant post-prandial increase in percentage of tachygastria (26.7 + 4.5 versus 12.4 + 2.6; P < 0.05) was seen in CF patients, which was not corrected by cisapride. The power ratio showed a statistical increase in 3 cpm (3.7 + 0.8 versus 1.6 + 0.3; P < 0.05) and in tachygastria (5.3 + 1.2 versus 1.7 + 0.4; P < 0.03) in CF compared with controls. Cisapride had an effect on tachygastria power ratio (3.0 + 0.5; P < 0.04). Analysis of normal rhythm and the dominant frequency instability coefficient were not statistically different in CF and controls. CONCLUSION: This study provides evidence of gastric dysmotility in CF patients.

Adolescent↗

PubMed2005

[Technical aspects and relevance of energy expenditure and physical activity assessment in clinical research for cystic fibrosis patients].

Cystic fibrosis (CF) is characterized by deteriorating lung function and mal-digestion, which result in growth failure and/or under-nutrition. Several factors, alone or combined, contribute to malnutrition in CF: poor energy intake, elevation of energy loss as a result of malabsorption, increasing resting energy expenditure due to genetic mutation and/or pulmonary exacerbation. Several techniques have been used to assess energy expenditure and physical activity in order to better understand mechanisms of malnutrition in CF and follow therapeutic interventions. Indirect calorimetry (IC) studies have shown that resting energy expenditure (REE) was 10-22% higher than predictive values. This increase could be attributed to chronic inflammation as a result of Pseudomonas aeruginosa (PA) infection. Indeed, intravenous antibiotic therapy decreases REE. Doubly labelled water technique and heart rate monitoring calibrated against IC techniques shows that total energy expenditure (TEE) was not different than in healthy children. Physical activity level assessed by the ratio TEE-REE is also not different between CF of healthy children. Recently, new accelerometry technics, easier to use and less invasive have been successfully used in order to assess physical activity level in CF. Precise and ambulatory assessment of energy expenditure and physical activity permit to check and adapt dietary allowances in CF. These techniques could be simultaneously used and be helpful to assess efficacy of intervention studies.

Adolescent↗

PubMed1996

Tyloxapol inhibits NF-kappa B and cytokine release, scavenges HOCI, and reduces viscosity of cystic fibrosis sputum.

Cystic fibrosis (CF) patients develop progressive cytokine-mediated inflammatory lung disease, with abundant production of thick, tenacious, protease- and oxidant-rich purulent airway secretions that are difficult to clear even with physiotherapy. In the search for a potential treatment, we have tested tyloxapol, an alkylaryl polyether alcohol polymer detergent previously used as a mucolytic agent in adult chronic bronchitis. Tyloxapol inhibits activation of the transcription factor nuclear factor-kappa B (NK-kappa B), reduces resting secretion of the cytokine interleukin-8 (IL-8) in cultured human monocytes, and inhibits lipopolysaccharide (LPS)-stimulated release of tumor necrosis factor-alpha (TNF-alpha), IL-1 beta, IL-6, IL-8, granulocyte-macrophage colony-stimulating factor (GM-CSF), and the eiconsanoids thromboxane A2 and leukotriene B4 (LTB4). We have previously shown that tyloxapol is a potent antioxidant for hydroxyl radicals ( OH). Tyloxapol (0.05 to 0.1% wt/vol) effectively scavenges the oxidant hypochlorous acid (HOCl; 1 to 7.5 mM) in vitro, and protects from HOCl-mediated lung injury in rats. Tyloxapol also reduces the viscosity of CF sputum (from 463 +/- 133 to 128 +/- 52 centipoise). We conclude that tyloxapol is potentially useful as a new antiinflammatory therapy for CF lung disease, and could possibly promote clearance of secretions in the CF airway.

Adult↗

PubMed2004

Prevalence of pain in adults with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) may strongly condition the life of affected people. CF may be associated with relevant painful conditions caused by complications of the illness and also by therapy itself, which may represent an additional load of suffering. This study was aimed at evaluating the prevalence of pain symptoms in adult CF patients, if they are noticed and treated, and the influence of pain symptoms on patients' life. METHOD: Using a questionnaire, we examined 239 adults with CF (17% of the whole Italian adult CF population). RESULTS: We found a high prevalence of painful episodes among CF adult patients, as for both intensity and frequency. In a 2 months period 32.6% of patients experienced episodes of pain described as intense to severe, and 29.7% had more than 10 occurrences of pain in the same location. Headache, gastric pain and backache were the most frequently reported kind of pain. 59.8% of subjects perceived pain episodes as the cause of unfavorable effects on their life. Only 42.6% of those with pain asked a CF center physician for help and another 3.5% a general practitioner. CONCLUSION: Painful symptoms can be the cause of a worsening of the quality of life for adults with CF; the relevance of pain in CF adult patients may often be underestimated; the assessment of pain should be routinely performed as a part of care in CF centers.

Adolescent↗

PubMed1983

Exercise and heat stress in cystic fibrosis patients.

Cystic fibrosis (CF) is characterized by high sweat sodium and chloride concentrations. CF patients have long been assumed to be at risk for heat illness, but there has been no quantitative documentation that CF patients actually have a greater loss of sodium and chloride than normals when under environmental stress. We compared thermoregulatory responses of eight CF patients with five normal controls during 90-min exposures to exercise and heat stress. Both groups reached similar peak rectal temperatures and peak heart rates; they had similar sweat volumes and rates. CF patients showed the normal rise in renin and aldosterone and the normal fall in urine sodium excretion; however, CF patients lost significantly more sodium (48.8 +/- 23.4 mEq/liter/m2 versus 20.2 +/- 11.2 mEq/liter/m2) and chloride (46.6 +/- 21.6 mEq/liter/m2 versus 18.5 +/- 11.3 mEq/liter/m2) per unit of surface area than the controls. Serum sodium and chloride concentrations fell in the CF patients but not in the normals. After exercise/heat stress, CF serum chloride was significantly less than normals' (99 +/- 3 mEq/liter versus 104 +/- 1 mEq/liter, P less than 0.01). CF patients have normal temperature, heart rate, hormonal, and renal responses to exercise and heat stress, yet still lose significantly more sodium and chloride than normal. These losses are reflected in part in lower serum concentrations of these ions. There may be important effects of these losses on respiratory tract secretions.

Adolescent↗

PubMed1991

Longitudinal serum IgG response to Pseudomonas cepacia surface antigens in cystic fibrosis.

In cystic fibrosis (CF), serum antibody against surface antigens of Pseudomonas aeruginosa is detected only after colonization. Since pulmonary acquisition of P. cepacia usually follows colonization with P. aeruginosa and since P. aeruginosa-colonized patients with CF have demonstrable antibody against outer membrane proteins of P. cepacia, it appears that acquisition of the latter organism occurs in the presence of specific serum antibody. To test this hypothesis, serum obtained from six P. aeruginosa-colonized patients 4 and 2 years prior to and 3 months and 2 years after P. cepacia colonization were assayed for total and specific IgG to P. cepacia outer membrane components. Four patients demonstrated 6-fold or greater increases in specific IgG titers to whole outer membranes following colonization. By immunoblot, all patients had demonstrable serum IgG against the 27- and 36-kDa outer membrane proteins of P. cepacia 4 and 2 years prior to colonization. Immunoblots after P. cepacia acquisition demonstrated an intensification of the 28- and 36-kDa bands and the appearance of antibody to a very low molecular weight compound which was not hydrolyzed by proteinase K and was present in purified LPS. These observations suggest that low serum titers of antibody against two P. cepacia outer membrane proteins are present in patients with CF prior to P. cepacia colonization, and that these antibodies fail to protect for intrinsic or extrinsic reasons.

Adolescent↗

PubMed2006

Flow cytometric analysis of HLA-DR antigen in conjunctival epithelial cells of patients with cystic fibrosis.

PURPOSE: Cystic fibrosis (CF) is an autosomal-recessive genetic disorder. The disease affects all secretory epithelia including the eye and belongs to the group of ocular surface epithelial diseases, termed keratoconjunctivitis sicca that develop in dry eye. In the pathogenesis of dry eye, inflammation plays a crucial role. The aim of this study was to investigate the expression of HLA-DR on conjunctival epithelial cells from patients with CF. MATERIALS AND METHODS: Twenty-five patients with CF and 25 normal subjects underwent ocular examination. Tear film break-up time (TBUT), Schirmer test, lissamine green staining, and conjunctival impression cytology were carried out. Cells were processed for flow cytometry, by using monoclonal antibodies to HLA-DR. RESULTS: The Schirmer test and TBUT scores were significantly lower in CF patients compared with controls. A significant increase of HLA-DR expression on epithelial cells was found in patients with CF compared with normal eyes. The Schirmer and TBUT test were positively correlated with HLA-DR expression for the percentage of cells. CONCLUSION: These results suggest that conjunctival epithelial cells play an important proinflammatory role in ocular changes in CF patients. Our findings confirm the presence of an inflammatory background and the immune nature of this disease. HLA-DR measurement might be a useful method for monitoring of inflammatory processes in the conjunctiva and could be helpful in the use of anti-inflammatory drugs in the treatment of ocular findings in CF patients.

Adolescent↗

PubMed1987

Quantitative deposition of aerosolized gentamicin in cystic fibrosis.

In cystic fibrosis (CF), the clinical effectiveness of aerosolized antibiotics is controversial. Previous investigators have not considered the type of nebulizer, droplet size, and dose to the lung in assessing the results of aerosol therapy. The present study tests the importance of these factors by standardizing an aerosol system for delivery of antibiotics and other agents to patients with CF. Particle size, distribution, and output from a commercially available nebulizer were measured. Thirteen patients with CF inhaled aerosol (MMAD = 1.1 micron) containing gentamicin (160 mg in nebulizer) and 99mTc-labeled human serum albumin. Patients' sputum and serum were analyzed for gentamicin levels by immunoenzymatic assay (Emit; Syva Corp., Palo Alto, CA). Using a gamma camera and suitable filters, central versus peripheral deposition (C/P ratio) and whole lung deposition were measured and related to sputum gentamicin levels. Gentamicin deposit averaged 12.3 mg +/- 5.9 (SD) or 7.69% of the original amount placed in the nebulizer. Peak sputum levels averaged 376.6 micrograms/ml +/- 275, whereas serum levels were undetectable in all patients. When peak sputum levels were normalized for the amount deposited, a close correlation with C/P ratio was obtained (r = 0.88, p less than 0.05). Furthermore, an inverse relationship was found between the C/P ratio and the %FEV1 (r = 0.76, p less than 0.05). Finally, a bell-shaped relationship between deposited dose and minute ventilation was seen in the patients (r = 0.88, p less than 0.05), i.e., an optimal minute ventilation was shown. These relationships may be important when designing future clinical studies.

Aerosols↗

PubMed2005

[Measurement of daily physical activity in patients with cystic fibrosis].

BACKGROUND: Cystic fibrosis (CF) patients often have a good level of daily activity contrasting with the impairment of pulmonary functions. The aim of this prospective study was to measure physical daily activity (PDA) in CF-patients and compare it with rest pulmonary functions and exercise tolerance. MATERIAL AND METHODS: Fifteen CF-patients and 9 control subjects have been recruted. PDA was measured during 7 days using an accelerometer: Global-PDA (daily mean counts per day), percentage of inactivity (1 to 2 METS), light activity (2 to 3 METS), and moderate-to-hard activity (greater than 3 METS). Spirometry (FEV1, Vital Capacity) and clinical exercice testing (VO2, workload) were also performed. RESULTS: Global-PDA of CF-patients was lower than control subjects (p < 0.05). Light activity (15.5 +/- 3.3% vs 13.4 +/- 2.4%) and inactivity (81.2 +/- 9% vs 79.9 +/- 3.1%) were similar in both groups. Only moderate-to-hard activity was greater in control subjects than in CF-patients (6.7 +/- 1.6% vs 3.3 +/- 3.4%; p < 0.05). There were correlations between Global-PDA and moderate-to-hard activity (r = 0.94), FEV1 (r = 0.72), Vital Capacity (r = 0.77), workload peak (r = 0.88), and VO2peak (r = 0.70). CONCLUSION: CF-patients have a lower PDA than control subjects because of a lower moderate-to-hard activity (>3 METS). In clinical practice, interviewing a CF-patient on his "run-life" PDA (<3 METS) has poor relationship with his real "moderate-to-hard activity", and this is a confusing factor for clinicians.

Adult↗

PubMed2005

Increased risk of idiopathic chronic pancreatitis in cystic fibrosis carriers.

Cystic fibrosis (CF) is a recessive disease caused by mutations of the CF transmembrane conductance regulator (CFTR) gene. The risk of idiopathic chronic pancreatitis (ICP) is increased in individuals who have CFTR genotypes containing a CF-causing mutation plus a second pathogenic allele. It is unknown whether the risk of ICP is increased in CF carriers who have one CF-causing mutation plus one normal allele. In this study, 52 sporadic cases of ICP were ascertained through the European Registry of Hereditary Pancreatitis and Familial Pancreatic Cancer. Individuals with pathogenic cationic trypsinogen mutations were excluded. DNA was comprehensively tested for CFTR mutations using a robotically enhanced, multiplexed, and highly redundant form of single-strand conformation polymorphism (SSCP) analysis followed by DNA sequencing. Fifteen subjects had a total of 18 pathogenic CFTR alleles. Eight subjects had common CF-causing mutations. This group included seven CF carriers in whom the second CFTR allele was normal (4.3 times the expected frequency, P=0.0002). Three subjects had compound heterozygotes genotypes containing two pathogenic alleles (31 times the expected frequency, P<0.0001). A variant allele of uncertain significance (p.R75Q) was detected in eight of the 52 ICP subjects and at a similar frequency (13/96) in random donors. ICP differs from other established CFTR-related conditions in that ICP risk is increased in CF carriers who have one documented normal CFTR allele. Having two CFTR mutations imparts a higher relative risk, while having only one mutation imparts a higher attributable risk.

Adult↗

PubMed2006

Impaired gastric myolectrical activity in patients with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is frequently associated with gastrointestinal complaints that can be due to gastrointestinal dysmotility. Electrogastrography (EGG) is an attractive, non-invasive procedure to assess gastric electric activity. The aims of our study were to investigate EGG abnormalities in pancreatic sufficient and pancreatic insufficient CF patients, and to examine whether EGG correlates with gastric emptying as assessed by scintigraphy. METHODS: EGG was performed in 23 CF patients (12 pancreatic sufficient patients, 11 pancreatic insuffficient) by using cutaneous recording pre- and postprandialy. Pre- and postpostprandial EGG indexes were compared to 19 healthy control patients. Gastric emptying was assessed simultaneously by gastric scintigraphy in 11 of the 23 CF patients. Six patients underwent a repeated scintigraphy recording following a month of treatment with cisapride. RESULTS: Abnormal patterns of EGG were found in 78.3% of CF patients compared to 31.3% of controls during fasting (p<or=0.003) and in 56.5% of CF patients compared to 15.7% in healthy controls postprandially (p<or=0.018). In the CF patients, dysmotility consisted mostly of bradygastria during both fasting (16 of 18 with abnormal EGG) and postprandially (12 of 13 [92.3%]). Gastric emptying results on scintigraphy were in agreement with EGG results in 9 of 11 (two normal and seven pathological). Five of the six patients treated with cisapride (83.3%) showed significant improvement in EGG indexes. CONCLUSIONS: Our results suggest that EGG abnormalities are frequently found in CF patients. The similar rate of EGG and gastric scintigraphy abnormalities suggests that EGG may be a useful clinical tool in CF patients.

Adolescent↗

PubMed2005

Inhibition of p38 mitogen activated protein kinase controls airway inflammation in cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) airways are characterised by chronic inflammation, increased interleukin (IL)-8 secretion, and neutrophil activation which are considered the principal factors of morbidity and mortality in CF patients. Optimising management of this chronic inflammatory response is therefore a key issue of basic and clinical CF research. Several reports have addressed ways to manage CF airways inflammation, and an attractive therapeutic strategy may be the inhibition of the p38-mitogen activated protein kinase (p38-MAP-k) pathway. METHODS: A new ex vivo model was used to study the mucosal inflammatory response to environmental airways stimuli. Nasal biopsy tissues from CF patients and controls were cultured ex vivo for 20 minutes, 4 hours, and 24 hours in the presence of lipopolysaccharide (LPS) from Pseudomonas aeruginosa (PA) with and without the p38-MAP-k inhibitor SB203580. Quantitative mRNA assessment, immunohistochemistry, and Western blots were used to detect the expression and modulation of inflammatory markers. RESULTS: PA-LPS challenge induced a time dependent mucosal inflammation indicated by rapid epithelial activation, IL-8 release, COX-2 upregulation, and neutrophil migration to the upper mucosal layers. Some of these LPS induced changes (IL-8 release and neutrophil migration) were specific to CF tissues. SB203580 significantly controlled all LPS induced mucosal changes in CF tissues. CONCLUSION: These findings provide a rationale and proof of principle for the potential use of p38-MAP-k inhibitors to control inflammation in patients with CF.

Adolescent↗

PubMed2002

Parents and partners: lay carers' perceptions of their role in the treatment and care of adults with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is the most common autosomal recessive genetic disease in Caucasian people, traditionally conceptualized as a condition whereby sufferers died in childhood. However, the current median survival age of 30 and a predicted median survival age of 40 for those born with the disease over the last decade ensure that families members will assist hospital staff with treatment and care well into most patients' adulthood. AIMS: This study explores the perceptions and experiences of lay care-giving amongst parents and partners of adults with CF who were being treated at a specialist CF centre in England. METHODS: Thirty-one relatives of adults with CF were interviewed in their own homes using an interview topic guide. All interviews were audiotape recorded and transcribed verbatim. Analysis of data was assisted by ATLAS-ti, a software package for qualitative research. FINDINGS: Two main themes surrounding lay carers' role in treatment and care were identified. Firstly, the notion of lay carers giving 'expert' care, both in hospital and at home was recognized. Parents' expertise was greater than that of partners until the patient required intensive hospital interventions, when partner expertise increased. Secondly, the degree of lay carers' felt inclusion in the hospital consultation appeared to depend on the nature of their relationship with the patient and the patients' health state. CONCLUSION: Lay carers are routinely performing tasks for adults with CF that were once the remit of trained nurses. Families need higher levels of nursing and social support when certain treatments are used at home. Attention needs to be directed to how lay carers of adult patients can be included in hospital consultations.

Adolescent↗