Benefits of an education programme on the self-management of aerosol and airway clearance treatments for children with cystic fibrosis. A randomized controlled trial of a new behavioral home-based nutrition education program, "Eat Well with CF," in adults with cystic fibrosis. Controlled evaluation of the STARBRIGHT CD-ROM program for children and adolescents with Cystic Fibrosis. Oral protein energy supplements for children with cystic fibrosis: CALICO multicentre randomised controlled trial. Randomized trial of a decision aid for patients with cystic fibrosis considering lung transplantation. Efficacy of high dose phylloquinone in correcting vitamin K deficiency in cystic fibrosis. Nebulized gentamicin in children and adolescents with cystic fibrosis. Evaluation of positive expiratory pressure as an adjunct to chest physiotherapy in the treatment of cystic fibrosis. Effects of short-term inhaled fluticasone on oxidative burst of sputum cells in cystic fibrosis patients. Failure of conventional strategies to improve nutritional status in malnourished adolescents and adults with cystic fibrosis. The management of cystic fibrosis with carbocysteine lysine salt: single-blind comparative study with ambroxol hydrochloride.
Adherence to recommended aerosol medicines and airway clearance techniques (ACT) for children with cystic fibrosis (CF) requires self-management skills. A multi-centre, randomized, controlled trial was conducted to investigate the effectiveness of a self-management education programme called 'Airways' for six- to 11-year old children with CF and their caregivers. Assessments were conducted immediately before and after the intervention period, and six and 12 months after the post-intervention assessment. The pen and paper education programme was completed by the child and caregiver together at home. Participants in the intervention and control groups had similar baseline characteristics. A per-protocol analysis was conducted and for variables that changed significantly, an additional intention-to-treat analysis was performed that included data from participants in the intervention group who withdrew from the study during the intervention period. The intervention group increased the percentage of prescribed aerosols taken (P < 0.001) and this was maintained at 12-month follow-up (P < 0.001). There was no change in the percentage of prescribed ACT performed, although when the child was unwell, caregivers in the intervention group increased the frequency and/or duration of ACT (P = 0.028) in the per-protocol analysis but not in the intention-to-treat analysis. Children in the intervention group increased their knowledge of ACT (P < 0.001) which was maintained at 12-month follow-up (P < 0.001) and felt more positively about their chest treatment regimens immediately following the intervention (P = 0.017) but not at 12-month follow-up. There were no significant changes in the control group for these variables over time. No significant changes occurred in the caregivers' reports of self-management behaviours and self-efficacy in either group. The positive results suggest that 'Airways' is a valuable educational tool for primary school-aged children with CF and their caregiver. Cystic fibrosis (CF) remains the most common genetically inherited disease in the white population and its prognosis is affected by nutritional status. Adults with the disease are now surviving longer and new strategies are required to ensure that they maintain optimal nutrition. This article reports preliminary data from a randomized controlled trial of a 10-week home-based behavioral nutrition intervention, "Eat Well with CF." Outcome measures of weight change over 6 and 12 months and changes in CF-specific nutrition knowledge score, self-efficacy score, reported dietary fat intake and health-related quality-of-life score were compared between the intervention group (n=34) and a standard care control group (n=34). The hypotheses to be tested were that adults with CF completing "Eat Well with CF" would have an improved nutritional status, improvement in specific nutrition knowledge, and an improvement in self-efficacy regarding their ability to cope with a special diet, compared to those receiving standard care. There were substantial improvements in the intervention group's specific CF nutrition knowledge score, self-efficacy score, and reported fat intake compared to control, but no substantial change in body mass index or health-related quality of life over time. Home-based nutrition education incorporating behavioral strategies can be an effective way to support adults with CF, enabling improvement in self-management skills in relation to diet and pancreatic enzyme replacement therapy. This study revealed gaps in basic nutrition knowledge and skills, inadequate knowledge of diet-disease links and pancreatic enzyme replacement therapy. These need to be identified when subjects progress from pediatric to adult care, and programs such as "Eat Well with CF" are a useful adjunct for registered dietitians trying to manage this diverse but growing population. To evaluate the effectiveness of the STARBRIGHT Fitting Cystic Fibrosis Into Your Life Everyday CD-ROM. Data were analyzed to evaluate the effectiveness of the program as an educational tool for children and adolescents with cystic fibrosis (CF).
                Forty-seven children and adolescents with CF between the ages of 7 and 17 years were enrolled in the study. Participants completed an initial evaluation of CF-related knowledge and coping skills and were then randomly assigned to one of two groups: the treatment group or the wait-list control group. Participants then viewed the CD-ROM, and researchers completed posttest measures.
                Analyses indicated that both disease-related knowledge and coping strategies generated by children and adolescents with CF improved as a result of the intervention and that this effect was replicated in the wait-list group.
                In sum, the results of the current study indicate that the STARBRIGHT CD-ROM program is a promising intervention for increasing CF-related knowledge and the competence of children's and adolescents' coping strategies. These positive results are enhanced by the brief, inexpensive, and portable nature of this educational program. To determine whether oral protein energy supplements, used long term in children with cystic fibrosis who are moderately malnourished, improve nutritional and other outcomes.
                Multicentre randomised controlled trial.
                Seven specialist paediatric cystic fibrosis centres and their associated shared care clinics and seven smaller paediatric cystic fibrosis clinics.
                102 children with cystic fibrosis, aged between 2 and 15 years, who were moderately malnourished.
                Oral protein energy supplements in addition to usual dietary advice compared with dietary advice alone, for 12 months.
                Change in body mass index centile over one year.
                Use of supplements was not associated with a change in body mass index centile (mean difference 2.99 centile points, 95% confidence interval -2.70 to 8.68) or other nutritional and spirometric outcomes in this group of children.
                Long term use of oral protein energy supplements did not result in an improvement in nutritional status or other clinical outcomes in children with cystic fibrosis who were moderately malnourished. Oral protein energy supplements should not be regarded as an essential part of the management of this group of children.
                ISRCTN: 95744468. We developed an evidence-based decision aid for patients with advanced cystic fibrosis considering referral for lung transplantation.
                To prospectively evaluate whether use of the decision aid increased knowledge about the options, improved realistic expectations, and decreased decisional conflict in adult patients.
                We performed a single-blind randomized controlled trial involving 149 adult patients with cystic fibrosis with an FEV(1) <or= 40% predicted from 14 Canadian and Australian centers. All participants received an education and counseling session from their cystic fibrosis team and were then randomized to receive the decision aid or usual care. The decision aid is available online at http://decisionaid.ohri.ca/decaids.html.
                The primary end points measured were participants' knowledge, realistic expectations, and decisional conflict evaluated 3 weeks after randomization. Patients randomized to the decision aid had greater knowledge about their options (P < 0.0001) and had more realistic expectations about the benefits and risks of lung transplantation (P < 0.0001) compared with those randomized to usual care. The total decisional conflict score was significantly lower in the decision aid group 3 weeks postrandomization compared with the usual care group (11.6 vs. 20.4; P = 0.0007). Decisions were durable; 88% of patients in the decision aid group and 75% in the usual care group maintained the same choice 12 months after randomization (P = 0.06).
                Use of a decision aid for patients with cystic fibrosis considering referral for lung transplantation, in addition to usual education and counseling, improves patient knowledge, realistic expectations, decisional conflict, and patient satisfaction. Clinical trial registered with www.clinicaltrials.gov (NCT00345449). Subclinical deficiencies of vitamin K are universally present in unsupplemented cystic fibrosis (CF) patients. The dose required to prevent deficiencies cannot be estimated from the existing literature. The aim of this study is determine if a supplemental dose of 1 mg/day or 5 mg/day vitamin K1 per day would normalize vitamin K status in a population of children with cystic fibrosis.
                Fourteen pancreatic insufficient CF children, between the ages of 8 to 18 years old, were randomized to receive either 1 mg/day or 5 mg/day vitamin K1 per day, for one month. Fasting blood tests were done at baseline and after one month of the intervention. The degree of undercarboxylation of osteocalcin (%Glu-OC), and serum vitamin K1, were evaluated by descriptive statistics and nonparametric Wilcoxon matched-pair test and Mann-Whitney U test.
                Of the 50% of subjects who were below the optimal serum vitamin K1 at baseline, all rose into the normal range with supplementation. Supplementation also significantly reduced the overall %Glu-OC from a median of 46.8 to 29.1% (p<0.0003).
                Our results suggest that both 1 mg and 5 mg of vitamin K1, given over a one-month period in pancreatic insufficient pediatric cystic fibrosis patients improve vitamin K status. A study of 29 children and adolescents with cystic fibrosis over 2 years showed some evidence of benefit from the twice daily inhalation of 20 mg nebulized gentamicin when compared to the inhalation of a nebulized saline mixture. Clinical symptoms, deterioration in pulmonary function, antibiotic usage, days in hospital and development of Pseudomonas aeruginosa in the sputum were recorded. There was no significant difference in antibiotic usage, days in hospital or clinical symptoms between the two regimes. Those subjects with P. aeruginosa in sputum showed significantly less deterioration in lung function over 2 years while using gentamicin aerosol. There was no difference in progress between the two treatment regimes for those subjects with P. aeruginosa in sputum at the beginning of the study, nor was there any difference in the number developing P. aeruginosa in sputum. It has been suggested that positive expiratory pressure may assist the clearance of bronchial secretions in the treatment of cystic fibrosis. It has been compared with currently used postural drainage techniques. Three treatment regimens were compared in 18 patients with cystic fibrosis. Treatment A consisted of breathing exercises emphasising inspiration, interspersed with the forced expiration technique in gravity assisted positions; treatment B comprised breathing exercises with positive expiratory pressure alternating with the forced expiration technique in the same gravity assisted positions; and treatment C comprised breathing exercises with positive expiratory pressure and the forced expiration technique in the sitting position. During treatment A a significantly greater quantity of sputum was produced than during treatments B and C (p less than 0.025 and p less than 0.001 respectively). Treatment B produced more sputum than treatment C (p less than 0.005). There were no significant differences in arterial oxygen saturation, FEV1 or forced vital capacity. Most adolescent and adult patients are able to carry out their treatment independently using gravity assisted positions, breathing exercises emphasising inspiration, and the forced expiration technique. Sputum clearance was less effective when positive expiratory pressure was included in the treatment regimen. Inhaled corticosteroids have been proposed to decrease pulmonary inflammation in cystic fibrosis (CF). In this study the effects of therapy with inhaled fluticasone on clinical and sputum outcomes (leukocyte count, activity of myeloperoxidase, superoxide anion release) in adult CF patients were investigated in an open label design. Twenty-six stable patients (median+/-SD forced expiratory volume in one second (FEV1) 58.1+/-19.9% pred.) were randomly assigned to the study group (500 microg b.i.d., for three weeks) or the control group (n=14; nonsteroid medication). Sputum samples were obtained during inhalation of hypertonic saline (3%, 20 min), which was found not to alter the investigated sputum parameters. No significant changes in clinical parameters, sputum leukocyte count, activity of myeloperoxidase, and baseline superoxide anion release where observed following therapy. Surprisingly, stimulated superoxide anion release increased significantly after therapy (34.1+/-17.7 versus 25.2+/-17.4 nmol x hr(-1) x 10(6) cells, p<0.03) and exceeded spontaneous variability of this parameter (p=0.02 versus control group). In conclusion, in adult cystic fibrosis patients short-term fluticasone therapy had no evident effect on clinical and sputum parameters. Further investigations are necessary to evaluate whether the observed up-regulation of oxidative capacity of inflammatory cells is of concern or benefit in these patients. The use of oral dietary supplements was compared with dietary counseling in 13 malnourished patients (3 males, mean age 18.1 years) with cystic fibrosis. Energy intake and nutritional status were evaluated over 3 months. There was no significant change in energy intake or percent ideal body weight in either group. The effectiveness of carbocysteine lysine salt monohydrate (SCMC-Lys) and ambroxol hydrochloride (ABX) in the management of respiratory impairment was compared in a single-blind, randomized study of 26 cystic fibrosis patients with similar baseline characteristics. Adults received either SCMC-Lys 900 mg or ABX 33 mg three times a day and children under 14 years of age either SCMC-Lys 270 mg three times a day or ABX 10 mg four times a day. All treatments were given orally for 80 days and at the end of this control period both groups showed significant improvement in chest sound score but improvement in cough score was observed only in those receiving SCMC-Lys. Expectorate viscosity and elasticity decreased significantly in both groups. In SCMC-Lys-treated patients paCO2 decreased and paO2 and Hb O2 saturation increased while only paO2 increased significantly in those treated with ABX. An increase in tidal volume, peak expiratory flow values and forced expiratory volume were evident in those receiving SCMC-Lys while significant increases in forced expiratory flow were recorded in those receiving ABX. SCMC-Lys patient's Shwachmann index improved significantly and conversely to the ABX patients. No adverse events were recorded in either treatment group. The study concluded that SCMC-Lys is at least as effective as ABX in improving respiratory function in patients with cystic fibrosis.
The available evidence from this review is of insufficient quantity and quality to draw any firm conclusions about the effects of self-management education for cystic fibrosis. Further trials are needed to investigate the effects of self-management education on a range of clinical and behavioural outcomes in children, adolescents and adults with cystic fibrosis and their caregivers.