Children with cystic fibrosis benefit from massage therapy. A randomized, controlled trial of a community-based support program for families of children with chronic illness: pediatric outcomes. The effects of biofeedback assisted breathing retraining on lung functions in patients with cystic fibrosis. Controlled evaluation of the STARBRIGHT CD-ROM program for children and adolescents with Cystic Fibrosis. Benefits of music therapy as an adjunct to chest physiotherapy in infants and toddlers with cystic fibrosis. Psychosocial and educational outcomes associated with home- and clinic-based pretest education and cystic fibrosis carrier testing among a population of at-risk relatives. Benefits of an education programme on the self-management of aerosol and airway clearance treatments for children with cystic fibrosis. Self-administration of drugs for cystic fibrosis. Behavioral intervention to improve calorie intake of children with cystic fibrosis: treatment versus wait list control. Randomized clinical trial of behavioral and nutrition treatment to improve energy intake and growth in toddlers and preschoolers with cystic fibrosis.
To measure the effects of parents giving massage therapy to their children with cystic fibrosis to reduce anxiety in parents and their children and to improve the children's mood and peak air flow readings.
                Twenty children (5-12 years old) with cystic fibrosis and their parents were randomly assigned to a massage therapy or a reading control group. Parents in the treatment group were instructed and asked to conduct a 20-minute child massage every night at bedtime for one month. Parents in the reading control group were instructed to read for 20 minutes a night with their child for one month. On days 1 and 30, parents and children answered questions relating to present anxiety levels and children answered questions relating to mood, and their peak air flow was measured.
                Following the first and last massage session, children and parents reported reduced anxiety. Mood and peak air flow readings also improved for children in the massage therapy group.
                These findings suggest that parents may reduce anxiety levels by massaging their children with cystic fibrosis and their children may benefit from receiving massage by having less anxiety and improved mood, which in turn may facilitate breathing. Children with chronic illnesses have a heightened risk for mental health problems.
                To develop, implement, and evaluate child outcomes of a 15-month, community-based, family-support intervention designed to reduce risk for poor adjustment and mental health problems in children with 1 of 4 chronic illnesses (diabetes mellitus, sickle cell anemia, cystic fibrosis, or moderate to severe asthma) and their mothers.
                Randomized, controlled clinical trial design with multiple measures of mental health based on both child and parent reports taken 1 year apart.
                Community-based intervention linked to subspecialty and general pediatric clinics and practices in Baltimore, Md.
                One hundred thirty-six mothers and children aged 7 to 11 years with diabetes mellitus, sickle cell anemia, cystic fibrosis, or moderate to severe asthma.
                The program, provided by "experienced mothers" and child life specialists, included telephone contacts, face-to-face visits, and special family events.
                Outcomes were measured using the following instruments: the Personal Adjustment and Role Skills Scale III, the Children's Depression Inventory, the Revised Children's Manifest Anxiety Scale, and the Self-Perception Profile for Children.
                The experimental group's mean adjustment score increased over the intervention period while the control group's mean adjustment score decreased. Analysis of variance demonstrated that the intervention had a significant main effect on postintervention adjustment controlling for baseline scores (P =.01). Using a cutoff score indicating maladjustment, the percentage of experimental group children in the maladjustment range fell from 19% at baseline to 10% after the intervention; the percentage of control group children in the maladjustment range rose from 15% at baseline to 21% after the intervention. The effect of the intervention was more pronounced for children who had low physical self-esteem than for those who had moderate to high physical self-esteem at the beginning of the program.
                Our results demonstrate modest positive effects of a family support intervention in promoting the adjustment of children with selective chronic health conditions. Including child life specialists in a community-based intervention may be especially salient for children with chronic illnesses who have low physical self-esteem. The intervention had a similar outcome for all diagnostic groups, suggesting that it could be effective for children with any chronic illness and implemented in a variety of pediatric settings. This study examines the effects of respiratory muscle feedback and breathing retraining (BRT) on lung function in patients with cystic fibrosis (CF). Twenty-six patients with CF were matched for age and severity of disease. Standard respiratory spirometry was performed on all subjects before and after biofeedback training. Thirteen experimental subjects underwent eight sessions of pneumographic or strain-gauge feedback from the abdominal muscles and electromyogram feedback from accessory respiratory muscles to assist in learning diaphragmatic and pursed-lips breathing maneuvers. Control subjects received biofeedback-assisted (hand warming) relaxation training. Results revealed a significant improvement in FEV1 and mean forced expiratory flow during the middle half of forced vital capacity (FVC) for the biofeedback group, while the control group showed no change. A similar trend was noted for FVC. These data suggest that respiratory muscle feedback and BRT may improve lung function in patients with CF. 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. Routine chest physiotherapy (CPT) is an important component of prophylactic therapy in children with cystic fibrosis (CF) and requires a significant commitment of time and energy. It is important, therefore, to establish CPT as a positive experience. In this study, we evaluated the effect of recorded music as an adjunct to CPT. Specifically, we compared the use of newly composed music, familiar music, and the family's usual routine on children's and parents' enjoyment of CPT and the parents' perception of time taken to complete CPT. Enjoyment and perception of time were evaluated via questionnaires designed specifically for this study. Participants were caregivers of one or more children with CF who were aged between 4(1/2) months and 24 months at the commencement of the clinical trial and required CPT on a daily basis. Participants were randomly allocated into control and treatment groups. Control group participants experienced two conditions consecutively: no audiotape (NT; control) and familiar music tape (FT; placebo control). Treatment group participants were given the treatment music tape (TT), which was composed and compiled by a music therapist. After baseline assessment, evaluation occurred at two 6-weekly intervals. Children's enjoyment increased significantly after use of the TT (+1. 25 units) compared to NT (-0.5 units; P = 0.03), as did parents' enjoyment (+1.0 vs. 0.0 units, P = 0.02). Children's enjoyment did not change significantly after use of the FT (+0.75 units) compared to NT (n.s.). Likewise, parents' enjoyment did not change significantly after use of the FT (+1.0 units, n.s.). There was no change in perception of time after use of the TT (-4.5 vs. +0.2 min, n.s.) or the FT (+3.3 min, n.s.). These results indicate that children's and parents' enjoyment of CPT significantly increased after the use of specifically composed and recorded music as an adjunct. We therefore recommend that recorded music, such as that provided in this study, be given to parents to use as an adjunct to CPT when their young children are diagnosed with CF, in order to assist the establishment of a positive routine.
                Copyright 2000 Wiley-Liss, Inc. We report on the psychosocial and knowledge outcomes of two different approaches to cystic fibrosis (CF) gene pretest education and carrier testing offered to 288 proactively recruited first-, second-, and third-degree relatives of people with CF. One group received pretest education and gene testing in a clinical setting from a certified genetic counselor. The other group received pretest education in their homes from a specially prepared pamphlet and were asked to send in a buccal cell sample for genotyping. No statistically significant differences between groups were noted on measures of CF knowledge, anxiety, and positive or negative affect, either while waiting for their test results or within a few weeks after they had learned their results. At both measurement points, participants who had received home education and testing reported that the testing was more convenient, but that they had received less information than they would have liked, and they were more likely to report being confused by the testing, although their level of CF knowledge was comparable to that of people who had been seen by a genetic counselor. In light of the increasing interest in home-based medical testing of all kinds, this study suggests that CF carrier testing in the home warrants further consideration as one possible approach to facilitating access to testing. 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. Self-administration of medication encourages individuals to participate in their own health care and provides structure for regular assessment and teaching about their drugs. Patient-friendly drug leaflets can provide consistent information to patients cared for by a number of staff. Self-administration studies found in the nursing literature must be interpreted cautiously because of the lack of true experimental trials. Changes in calorie intake and weight gain were evaluated in five children with cystic fibrosis (CF) who received behavioral intervention and four children with CF who served as wait list controls. The behavioral intervention was a 6-week group treatment that provided nutritional education plus management strategies aimed at mealtime behaviors that parents find most problematic. The control group was identified prospectively and was evaluated on all dependent measures at the same points in time pre- and posttreatment as the intervention group. Difference scores on calorie intake and weight gain from pre- to posttreatment were compared between groups using t tests for independent samples. The behavioral intervention group increased their calorie intake by 1,032 calories per day, while the control group's intake increased only 244 calories per day from pre- to posttreatment [t(6) = 2.826, p = 0.03]. The intervention group also gained significantly more weight (1.7 kg) than the control group (0 kg) over the 6 weeks of treatment [t(7) = 2.588, p = 0.03] and demonstrated catchup growth for weight, as indicated by improved weight Z scores (-1.18 to -0.738). The control group showed a decline in weight Z scores over this same time period (-1.715 to -1.76). One month posttreatment, the intervention was replicated with two of the four children from the control group. Improved calorie intake and weight gain pre- to posttreatment were again found in these children. At 3- and 6-month follow-up study of children receiving intervention, maintenance of calorie intake and weight gain was confirmed. No changes were found on pulmonary functioning, resting energy expenditure, or activity level pre- to posttreatment. This form of early intervention appears to be promising in improving nutritional status and needs to be investigated over a longer period of time to evaluate the effects of treatment gains on the disease process. To conduct a randomized clinical trial comparing a behavioral and nutrition intervention (BEH) with a usual care control condition (CTL) for children (ages 18 months to 4 years) with cystic fibrosis (CF) and pancreatic insufficiency. This trial was designed to (1) evaluate a randomized comparison of BEH with CTL over 8 weeks, (2) provide a replication of the impact of BEH by inviting the CTL group to receive BEH after 8 weeks, and (3) examine the maintenance of BEH at 3- and 12-month follow-up.
                Of 14 eligible children, 10 were randomly assigned and initiated treatment (71% recruitment rate). Four participants were randomly assigned to BEH, and 6 were assigned to CTL (5 of whom chose to crossover to BEH). BEH included nutrition counseling to increase energy intake (via types of foods and addables/spreadables) and child behavioral management training to teach parents differential attention and contingency management skills. CTL was consistent with the 2002 CF Foundation Consensus Conference Guidelines for nutritional care.
                BEH led to greater increases in energy intake pre- to posttreatment than CTL as measured by calories per day (842 kcal/day vs -131 kcal/day change). On receiving BEH, the change in energy intake was replicated with the CTL group (892 kcal/day change). At 3- and 12-month follow-up, energy intake was maintained (672 kcal/day increase from baseline and 750 kcal/day increase from baseline, respectively). Children in this study met or exceeded normal weight and height velocities from pretreatment to the 3-month follow-up (mean weight: 1.4 kg/6 months; mean height: 5.1 cm/6 months) and from posttreatment to the 12-month follow-up (mean weight: 2.5 kg/12 months; mean height: 8.3 cm/12 months).
                Toddlers and preschoolers who have CF and received BEH were able to meet the energy intake recommendations for this disease and maintain these gains up to 12 months after treatment. In addition, these children demonstrated weight and height velocities from pretreatment to 12-month follow-up, consistent with the goal of normal growth. BEH is a promising, evidence-based, early nutritional intervention for children with CF. An upcoming multisite clinical trial will test BEH versus an attention control condition using a larger sample (N = 100), providing additional evidence about the efficacy of this treatment for energy intake and growth in young children with CF.
Currently no clear evidence exists on the best psychological interventions to help people with CF and their carers manage the disease. Trials of interventions to improve adherence to treatment are needed. Multicentre approaches, with consequent funding implications, will increase the sample size of trials and enhance the power and precision of their findings.