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Adherence and psychopathology in children and adolescents with cystic fibrosis
Abstract
The striking increase in the lifespan of individuals with cystic fibrosis (CF) has largely been attributed to the dramatic improvements in treatment regimens. These treatments are time intensive and may interfere with healthy development and family function. The objective of this study was to investigate the association between psychopathology and treatment adherence in children and adolescents with CF.
Structured psychiatric interviews were performed on 52 patients with CF. Additional information on family function and youth behaviors were also collected. Youth and parent reports of adherence to the CF treatments were obtained and compared with the CF teams' records.
The mean overall adherence to the CF teams' recommendations was 77-81% for the child and parent reports, respectively. Children with anxiety disorders and families who were more cohesive showed significantly higher rates of adherence to the CF treatments. In addition, children in families with a balance of structure and flexibility also report higher levels of adherence to the CF treatments.
Anxiety disorders in children with CF may be associated with increased adherence to the numerous CF treatment regimens. In addition, family patterns that are cohesive and balanced are better able to incorporate the CF treatments into family life.
... The day-to-day activities for youth with CF seem even more difficult when the busy lifestyle of someone in this age group is considered. A person with CF must adhere to a vigorous schedule, which includes chest physiotherapy two to three times per day, multiple oral and nebulized medications, ensuring optimal caloric nutrition is met, exercise, and possible care of a gastrostomy tube for extra nutrition (Cystic Fibrosis Canada, 2013;de Deus et al., 2019;Gjengedal et al., 2003;White et al., 2009). Adherence rates to treatments for younger populations is less than 50%, with higher rates for less time-consuming interventions, such as taking enzymes and oral antibiotics (Dempster et al., 2018;Goodfellow et al., 2015;White et al., 2009). ...
... A person with CF must adhere to a vigorous schedule, which includes chest physiotherapy two to three times per day, multiple oral and nebulized medications, ensuring optimal caloric nutrition is met, exercise, and possible care of a gastrostomy tube for extra nutrition (Cystic Fibrosis Canada, 2013;de Deus et al., 2019;Gjengedal et al., 2003;White et al., 2009). Adherence rates to treatments for younger populations is less than 50%, with higher rates for less time-consuming interventions, such as taking enzymes and oral antibiotics (Dempster et al., 2018;Goodfellow et al., 2015;White et al., 2009). It is imperative that low adherence rates are considered within the context of multiple demands for youth, which may include attending school, homework, extracurricular activities, and having a part-time job. ...
... Effective family functioning was also studied by a team of researchers who developed a cohesion scale to assess how family functioning relates to adherence to care for youth with CF. Their findings demonstrate that high adherence to treatment correlates with families that scored high on the cohesion scale (White et al., 2009). Furthermore, promoting the accessibility of coaching family members on how to support youth is crucial to help impact treatment adherence and family relationships. ...
Background and Purpose: Cystic Fibrosis (CF) is a complex condition that impacts various functions throughout the body. Improvements in health through technological advances and research have increased the global life expectancy for youth with CF; however, access to resources and support remains suboptimal. Therefore, our primary aim was to identify the most common access issues for youth with CF, with secondary aims of raising awareness about these barriers and proposing potential solutions. Methods: An initial literature scan was conducted, where five key themes were identified as the most pertinent access issues for youth, including: treatment complexity, distance to care facilities, financial constraints, family involvement, and mental health factors. To explore these access issues in further detail, we conducted an iterative narrative review across four databases. Results: Twenty-five research articles were identified across the five themes, and included the following designs: review, cross-sectional, survey/questionnaire, descriptive, quasi-experimental, longitudinal, cohort, observational, and qualitative research. Expert opinions and news articles were also included using a systematic grey literature scan. Conclusion: While these articles provide the most up-to-date evidence on access issues for youth with CF, current research remains limited. However, nurses can be key advocates for assisting youth populations with CF to better access health services while minimizing potential barriers to care. Some proposed advocacy activities include supporting policy changes through funding for life-enhancing medication and increasing access to digital health services that improve health outcomes for youth with CF.
... 5 Despite the central role ACTs play in improving pulmonary secretion clearance and helping to maintain lung function, patient adherence is challenging because these therapies are time-consuming and cumbersome for patients to complete daily. Studies have reported low adherence to ACTs in all age groups (ie, children, [6][7][8] adolescents, 8,9 and adults [10][11][12], with adherence declining as patients age and their daily routines become more complex. ...
... 5 Despite the central role ACTs play in improving pulmonary secretion clearance and helping to maintain lung function, patient adherence is challenging because these therapies are time-consuming and cumbersome for patients to complete daily. Studies have reported low adherence to ACTs in all age groups (ie, children, [6][7][8] adolescents, 8,9 and adults [10][11][12], with adherence declining as patients age and their daily routines become more complex. ...
... Adherence rates for the adolescent age group (13-19 y of age) in this study were substantially below the other 2 age groups (2-6 y of age and 7-12 y of age), consistent with past studies of disease management and adherence among children and adolescents with chronic conditions such as cystic fibrosis and type 1 diabetes. 8,9,[15][16][17][18] Although adolescence is commonly a time for increased autonomy and self-determination, there is still a role for caregivers to play in support of therapeutic management for chronically ill adolescents. [19][20][21][22][23][24][25] Our data suggest there may be a need for more frequent and open discussions between providers and caregivers regarding daily therapeutic routines, informed by objective usage data. ...
Background:
Airway clearance techniques (ACTs), including high-frequency chest wall oscillation (vest therapy), are important for maintaining lung function for patients with cystic fibrosis, but daily completion of ACTs is time-consuming and cumbersome. Patient adherence is a persistent challenge, and adherence data are largely patient reported, which may reduce accuracy. To provide definitive adherence data, this study utilized a Bluetooth-enabled vest therapy system to remotely collect objective adherence data from a cohort of pediatric subjects.
Methods:
Vest usage data were collected over a 12-month period and compared to each subject vest prescription. Because vest prescriptions are multifaceted, we developed metrics to examine adherence to the various facets (eg, daily treatment, treatments per day, treatment duration, frequency settings, and frequency and pressure settings combined) as well as an overall adherence metric.
Results:
Among the 73 enrolled subjects, 62 (50% male, age range 2-19 y) completed the study. Among adolescent subjects age 13-19 y, average adherence to daily vest therapy (44.0%) was significantly lower than that among subjects 0-6 y old (77.8%) and subjects 7-12 y old (89.5%). As more prescribed therapy components were combined into a single adherence metric, all age groups declined in mean adherence rates, with overall adherence a decreasing function of age.
Conclusions:
Employing new technology to remotely collect vest usage data allows for a granular examination of vest therapy adherence. While maintaining high levels of treatment adherence becomes increasingly difficult as children age, we also found substantial reductions in adherence rates among all age groups when more complex aspects of therapy prescriptions, such as frequency and pressure settings, were examined. These data illustrate areas providers and care teams can focus on to improve patient adherence to vest prescriptions.
... The smallest sample size was 19 participants while the largest was 95. Six studies included adolescents' caregivers in order to obtain more information on the patients' adherence to CF therapies [19][20][21][22][23][24]. Three studies did not take place in the United States and included participants from the United Kingdom, Denmark and Australia [25,21,26]. ...
... Ten studies were observational, and therefore, analyzed quantitative data. Four studies used a cross sectional approach, primarily by means of administering a questionnaire [19,22,25,27]. One study used retrospective analysis from the previous 12 months to obtain adherence data [26], while another study used a prospective design to collect adherence and associated data for 7 days [28]. ...
... One study used retrospective analysis from the previous 12 months to obtain adherence data [26], while another study used a prospective design to collect adherence and associated data for 7 days [28]. Four studies used mixed methods design and obtained their data in a variety of modalities [19,21,23,24]. ...
... Twenty papers assessed participants' adherence to medication (male, n ϭ 916; female, n ϭ 855; unspecified, n ϭ 69). 3,10,11,20,[22][23][24][25][27][28][29][31][32][33][34][35][36][37][38][39] Eighteen papers were of low quality, and 2 were of moderate quality. The categories of medications are summarized below (Table 3). ...
... Seven studies were of low quality, and 2 were of moderate quality. Six studies used participant questionnaires 3,25,27,29,31,37 ; 3 used parent questionnaires 3,22,31 ; one used participant interviews 38 ; one used 7-d recordings 22 ; and one used pharmacy refill records, daily telephone diaries, and electronic monitoring via the Medication Event Monitoring System (MEMS SmartCap, AARDEX, Richmond, Virginia). 3 Mean adherence ranged from 27.4 Ϯ 22.9% by daily telephone diary 3 to 96.5% by patient report. ...
... All but one study was of low quality. Eight studies used participant questionnaires, 3,4,23,[25][26][27]30,31 4 used parent questionnaires, 3,22,28,30 , 2 used clinician questionnaires, 22,27 one used caregiver questionnaires, 24 and one used daily telephone diaries. 3 Only 2 papers of low quality 30,31 specified the individual aspects of chest physical therapy involved (positive expiratory pressure, coughing, active cycle of breathing technique, chest wall percussion, chest wall vibrations). ...
Background:
The management of cystic fibrosis (CF) includes adherence to a home management program (airway clearance, medication, nutritional advice, and exercise). This has led to an increase in life expectancy, although the benefits depend greatly on a patient's level of adherence to daily treatments at home. To date, no systematic review has established adherence rates to all World Health Organization guidelines in the home setting; hence, this review was undertaken.
Methods:
The review comprised 3 phases. A methodological assessment of databases (Embase, CINAHL, PsychINFO, PEDro, PubMed, Cochrane Central Register of Controlled Trials) identified potentially relevant papers. These papers were screened for inclusion criteria by 2 independent reviewers, data were extracted, and the internal validity was rated using a valid and reliable scale. Results were categorized into 4 themes: medication, nutrition, airway clearance techniques, and exercise.
Results:
The search generated a total of 26 papers, 24 of which were rated as being poor quality. Adherence to a treatment program for CF patients is generally low (from 22% for nutritional guidelines to 130% for oral antibiotics), and it varies greatly depending on the type of treatment and the method of assessment employed (objective tool vs self-reported questionnaires).
Conclusions:
Consensus on how to measure adherence is lacking, and the quality of studies addressing adherence in this population is generally poor. Overall, studies using self-reported measures yielded higher adherence scores than those that used objective measures, suggesting that current efforts to improve methods of adherence are appropriate. The prevalence of non-adherence remains unclear due to these limitations.
... The role of families in passing on information about diet and Creon was essential to their understanding and confidence and impacted on their Creon adherence. The link between family functioning and treatment adherence in CF has been identified previously (22)(23)(24)(25) , with families that find a balance between cohesion and flexibility reporting higher rates of adherence (22) . In the present study, participants in supportive family units were, as children, confident to ask questions and seek the explanations they needed from the CF team; however, all participants urged children to be assertive, seek support and be actively involved in their dietary care. ...
... The role of families in passing on information about diet and Creon was essential to their understanding and confidence and impacted on their Creon adherence. The link between family functioning and treatment adherence in CF has been identified previously (22)(23)(24)(25) , with families that find a balance between cohesion and flexibility reporting higher rates of adherence (22) . In the present study, participants in supportive family units were, as children, confident to ask questions and seek the explanations they needed from the CF team; however, all participants urged children to be assertive, seek support and be actively involved in their dietary care. ...
Background:
Little is known about adults' experience of living with cystic fibrosis (CF) specifically in relation to the gut. However, their unique perspectives may be meaningful to children with CF and inform the understanding and practice of dietitians. The present study aimed to explore adults' lived experience of the CF gut and how they learnt to manage the gut as they were growing up.
Methods:
Semi-structured interviews were conducted with adult inpatients (n = 10). Interviews were audio-recorded, transcribed verbatim and accounts analysed using interpretative phenomenological analysis.
Results:
Three super-ordinate themes were identified: taking Creon, the learning process and this much I (now) know. Participants accounts of how CF affects the gut predominantly focused on taking Creon (pancreatin, Mylan). Various strategies were employed for coping with peer responses to taking Creon at school. Several participants reached adulthood before they understood and/or accepted that taking Creon consistently needed to be normal for them. Knowledge and understanding developed over time, with 'CF experience' and was shaped by family, CF care teams and other children with CF. All had unmet information needs when growing up. Having key explanations earlier, to make connections between eating, taking Creon, gaining weight and growth, did or would have helped most participants. Participants urged children to be assertive, ask questions and not only be involved in managing their diet and gut, but also begin to take control of this aspect of their CF.
Conclusions:
Supporting development of knowledge, skills and confidence to manage diet and the gut needs to be integral to care throughout childhood.
... studies have reported adjustment problems, increased depression and anxiety levels, and a high rate of psychopathology in these children and adolescents [3][4][5] whereas some others have indicated a comparable functionality with their healthy peers. 2,6,7 Studies so far have reported anxiety disorders, depression, and oppositional defiant disorder as psychiatric disorders in children and adolescents with CF. 3,8,9 The concept of quality of life (QOL) is a multi-dimensional subjective assessment of one's own health and life. This concept encompasses various dimensions such as physical, psychological, and social wellness, and functionality. ...
... Whereas previous studies have reported lower rates of psychiatric disorders in CF groups, the most commonly encountered disorders have been anxiety disorder and depression, as noted in our study. 2,8,9,30,31 Those studies have usually used either self-reported scales, parent reports or unstructured interviews. We believe that a greater rate of psychiatric diagnoses likely resulted from the structured interview technique used in our study and also the lack of adequate psychosocial support for these children in our country. ...
Şenses-Dinç G, Özçelik U, Çak T, Doğru-Ersöz D, Çöp E, Yalçın E, Çengel-Kültür E, Pekcan S, Kiper N, Ünal F. Psychiatric morbidity and quality of life in children and adolescents with cystic fibrosis. Turk J Pediatr 2018; 60: 32-40. The aim of this study was to investigate psychiatric disorders, depression and anxiety levels, and quality of life in children and adolescents with cystic fibrosis (CF), and to compare them with those of children with non-cystic fibrosis (non-CF) bronchiectasis and healthy controls. A total of 103 children and adolescents aged 7-16 years (35 CF, 28 non-CF bronchiectasis, 40 healthy) were evaluated using The Schedule for Affective Disorders and Schizophrenia for School Aged Children (K-SADS), The Child Depression Inventory (CDI), The State-Trait Anxiety Inventories for Children (STAI-C) and the Pediatric Quality of Life Inventory (PedsQL)-C. The three groups were not statistically different with respect to age, sex, and familial sociodemographic variables. 80% of the children and adolescents in the CF group were diagnosed with a psychiatric disorder, which was significantly more compared to those of the two other groups. The CF group had significantly greater rates of depressive and oppositional defiant disorder and the non-bronchiectasis group had a significantly greater rate of anxiety disorder than the control group. The depression and anxiety symptom levels were significantly greater and the quality of life levels significantly lower in both the CF and non-CF bronchiectasis groups than the healthy controls. In the CF group, the presence of any associated psychiatric disorder led to significantly lower total and psychosocial quality of life scores. In conclusion, CF is associated with poorer QOL in childhood. In order to improve quality of life in CF, the psychiatric conditions of children and adolescents should also be evaluated and their follow-up and treatment should involve a multidisciplinary team approach.
... In the current study 55% children (≥11 years) and 92% parents indicated high-adherence to enzyme supplements using self-report methods. This contrasts with previous studies in children using self-report which indicated that adherence to enzyme supplements lies between 77-98% [3,26,27]. Overall only 28% of participants were classified as high-adherers when all methods (including prescription refill and prescription issue data) were taken into account. This rate of adherence is comparable to diary data (27%) observed in studies in the USA [3]. ...
... Children whose parents had reported high necessity beliefs regarding their child's use of enzyme supplements or chest physiotherapy were significantly more likely to be classified as high-adherers to these treatments. It has been reported previously that poor adherence to nebulised therapy in adults was associated with doubts about nebuliser necessity and similarly, non-adherence to antibiotics and chest physiotherapy has been related to adolescents' doubts about their necessity in cystic fibrosis [10,26]. In the present study, necessity beliefs for all treatments were significantly higher for parents of children 11-18 years than those reported by their children. ...
Adherence to treatment is often reported to be low in children with cystic fibrosis. Adherence in cystic fibrosis is an important research area and more research is needed to better understand family barriers to adherence in order for clinicians to provide appropriate intervention. The aim of this study was to evaluate adherence to enzyme supplements, vitamins and chest physiotherapy in children with cystic fibrosis and to determine if any modifiable risk factors are associated with adherence.
A sample of 100 children (≤18 years) with cystic fibrosis (44 male; median [range] 10.1 [0.2-18.6] years) and their parents were recruited to the study from the Northern Ireland Paediatric Cystic Fibrosis Centre. Adherence to enzyme supplements, vitamins and chest physiotherapy was assessed using a multi-method approach including; Medication Adherence Report Scale, pharmacy prescription refill data and general practitioner prescription issue data. Beliefs about treatments were assessed using refined versions of the Beliefs about Medicines Questionnaire-specific. Parental depressive symptoms were assessed using the Center for Epidemiologic Studies Depression Scale.
Using the multi-method approach 72% of children were classified as low-adherers to enzyme supplements, 59% low-adherers to vitamins and 49% low-adherers to chest physiotherapy. Variations in adherence were observed between measurement methods, treatments and respondents. Parental necessity beliefs and child age were significant independent predictors of child adherence to enzyme supplements and chest physiotherapy, but parental depressive symptoms were not found to be predictive of adherence.
Child age and parental beliefs about treatments should be taken into account by clinicians when addressing adherence at routine clinic appointments. Low adherence is more likely to occur in older children, whereas, better adherence to cystic fibrosis therapies is more likely in children whose parents strongly believe the treatments are necessary. The necessity of treatments should be reinforced regularly to both parents and children.
... In one study, optimistic acceptance and hopefulness were associated with greater treatment adherence in CF [Abbott et al. 2001]. Conversely, other studies conclude that patients who worry about their condition have greater adherence [Abbott et al. 1996] and children with an anxiety disorder are more likely to be adherent to CF treatment [White et al. 2009]. Data are inadequate to identify direct causality between psychological factors and adherence. ...
... A balance between psychological wellbeing and disease progression should be the target for adherence strategies [Abbott et al. 1996]. Older children and adolescents in families experiencing unhappy and conflicted relationships are at greater risk of nonadherence to CF treatment [Delambo et al. 2004;White et al. 2009]. ...
Nonadherence to inhaled therapies is a major problem in the treatment of cystic fibrosis that can influence lung function and health outcomes. Potential contributors to nonadherence have been identified, including demographic and psychosocial factors, time and convenience of inhaled therapy, and treatment beliefs. Additional research is clearly needed to clarify the contributors and to determine which interventions and technological advances will enhance adherence to inhaled therapies in patients with cystic fibrosis. Nurses and allied health professionals are ideally positioned to assist patients and families with adherence to inhaled therapies through monitoring, communication, and education about the available therapies and their proper use. This review briefly summarizes the available evidence about contributors to nonadherence, potential interventions, novel delivery devices for inhaled therapies, and opportunities for additional research.
... Lower and elevated rates of depression have been reported in children, adolescents and young adults who have CF compared with healthy controls [12,13]. Normal [12][13][14] and elevated levels of anxiety [15,16] have been reported in adult and adolescent patients. ...
Background
The International Depression/anxiety Epidemiological Study (TIDES) in the UK aimed: (i) to establish the prevalence of anxiety and depression amongst people with CF compared to a normative sample; (ii) to establish the association between mood, demographic and clinical variables; and (iii) to provide guidance for specialist-referral decision-making.
Methods
Patients (≥ 12 years) completed the Hospital Anxiety and Depression Scale (HADS). CF-HADS scores, expressed as percentiles, were compared with a normative sample. Multiple-regression analysis explored associations between demographic, clinical and mood variables.
Results
Thirty-nine CF centres recruited 2065 patients. Adults with CF were similar in terms of anxiety and depression to the general population. Adolescents with CF were less anxious and depressed. For adult patients, older age, unemployment for health reasons and poor lung function were associated with disordered mood. Gender-specific CF-percentile scores were calculated.
Conclusion
Surveillance, with attention to gender and risk factors is advocated. This work provides unique benchmark scores to aid referral decision-making.
... There is substantial evidence that depression and anxiety are common in adolescents and adults with cystic fibrosis (CF) and their caregivers, 1 with adverse impacts on quality of life (QoL), 2,3 disease self-management, 4 healthcare costs, 5,6 and survival. 7 Studies have begun to document analogous findings for mental health conditions in preadolescents with CF, [8][9][10][11][12][13][14] reinforcing the value of prevention and early intervention. 15 People living with CF often experience multiple biopsychosocial stressors over time, increasing risk for the phenotypic emergence of these conditions. ...
This is the second of two companion papers which examine emotional wellness of children with cystic fibrosis (CF) during the early years of life, defined here as the period between birth to age 12. Both papers promote optimal mental health and well‐being, with an emphasis on early identification and intervention. The first paper explores child and family resilience. Here, we discuss strategies for pediatric CF teams to provide routine, systematic mental health assessment, anticipatory guidance, brief intervention, and triage to evidence‐based treatment when needed, while addressing barriers to accessing care. Many mental health conditions emerge before the age of 12, with the potential for lifelong effects on individuals, their family, and society. Living with a chronic illness such as CF can further increase the risk of mental health concerns and, in a bidirectional manner, their consequences for quality of life, sustaining daily care, and health outcomes. There has been a significant focus in recent years on the mental health and wellness of adolescents and adults with CF, but less attention to specifics of depression and anxiety in younger children, or to other common pediatric comorbidities including trauma, developmental disorders such as Attention‐Deficit/Hyperactivity Disorder (ADHD) or Autism Spectrum Disorder (ASD), and oppositional behavior. Given the availability of psychometrically sound screening instruments and effective interventions, routinely addressing the mental health of children with CF and their families is feasible to integrate within multidisciplinary CF care, allowing for a personalized approach respecting individual needs, values, and goals.
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... Most high adherers reported experiencing fear as a factor that affected their adherence as has been reported previously (White, Miller, Smith, & McMahon, 2009). Conversely lower adherers reported avoidance consistent with previous research (Abbott, Dodd, Gee, & Webb, 2001). ...
Objectives
Adherence to nebulizer treatment in adults with Cystic Fibrosis (CF) is poor, and interventions are needed. This research aimed to identify the factors affecting nebulizer adherence using the Theoretical Domains Framework (TDF) and to compare these for participants with different levels of adherence.
Design
Data‐prompted interviews using the TDF.
Methods
Eighteen semi‐structured interviews were conducted with adults with CF during which objectively measured adherence data were discussed. Framework analysis was used to code the data into TDF domains, and inductive qualitative content analysis was used to code different beliefs and experiences. Aspects of the TDF that differed between participants with different adherence levels were explored.
Results
Factors influencing adherence to treatment included all 14 domains of the TDF, 10 of which appeared to vary by adherence level: Skills; Memory and decision‐making; and Behavioural regulation; Environmental context and resources; Social influences; Beliefs about consequences; Beliefs about capability; Reinforcement; Social role and identify; Intentions; Optimism; and Emotions.
Conclusions
This study is the first to use objectively measured adherence data in a data‐prompted interview using the TDF framework to systematically assess the full range of factors potentially influencing adherence. The results highlighted that interventions need to consider issues of capability, opportunity, and motivation. Interventions that challenge dysfunctional beliefs about adherence and which support the development of routines or habits and problem‐solving may be particularly useful for adults with CF.
Statement of contribution
What is already known?
• Adherence to medication in adults with cystic fibrosis is poor.
• Previous research has identified a range of contributing factors in relation to subjective reports of adherence.
• There is a wide discrepancy between self‐reported adherence and objectively measured adherence.
What this study adds
• A data‐prompted interview using objectively measured adherence data enabled the systematic assessment of potential factors that could be targeted in an intervention to increase adherence.
• There were some differences in the factors that were identified by high and low adherers.
• There is not one‐size fits all intervention for adherence to medication in cystic fibrosis.
... Higher rates of depression have been linked to poorer adherence, worse pulmonary functioning, and lower quality-of-life scores (28). In contrast, higher levels of anxiety have been associated with better treatment adherence (91). Patients who are concerned about their health and well-being may both report slightly higher levels of anxiety and be more conscientious about their treatments. ...
... An American study 22 on the association of psychopathology (as assessed by structured psychiatric interview and CBCL [parent version]) and adherence (as assessed by self-reports) included 53 patients (mean age 12.4y) and their primary parent. The psychiatric interviews revealed a rather high rate of psychopathology: 57 % of children met criteria for at least one diagnosis of internalising disorders, namely anxiety, the latter accounting for the majority of reported psychopathology (16/30 cases). ...
This chapter is part of my book "Psychology in Medical Settings: Lung transplantation and Cystic fibrosis". Results were first presented at the 29th ECFC in Copenhagen, Denmark.
The book is not available any more. If you are interested, you may, hoewever, buy author copies. Please let me know: Ullrich@med-wiss.com
Thank you for your interest.
... Most high adherers reported experiencing fear as a factor that affected their adherence as has been reported previously (White, Miller, Smith, & McMahon, 2009). Conversely lower adherers reported avoidance consistent with previous research (Abbott, Dodd, Gee, & Webb, 2001). ...
Objectives: To identify the factors influencing nebuliser adherence in adults with Cystic Fibrosis (CF) using the Theoretical Domains Framework (TDF) in order to inform the development of a behaviour change intervention. Methods: Interviews were conducted with 18 adults with CF. The interview guide was developed using the TDF to explore nebuliser adherence. During the interview participants were presented with charts of their own nebuliser adherence data, and this was used to prompt discussion. Interview transcripts were content-analysed using the TDF as the coding framework. Results: All 14 theoretical domains were identified as being relevant to nebuliser adherence although the specific domains of influence were variable across individuals. Motivation for treatment was often related to a range of beliefs about the consequences of adherence to treatment, the amount of feedback that they perceived from treatment, and their treatment and other life goals. Capability was often related to knowledge about treatment, the ability to remember to take treatment particularly outside of a normal routine, and the extent to which treatment was planned and monitored. Issues of Opportunity included both facilitatory and inhibitory social influences, and the extent to which the environment and resources facilitated treatment. Conclusion: The TDF allowed for the identification of a range of factors influencing adherence to nebuliser treatment that could be targeted in a future intervention. Interventions should be tailored to the specific Motivation, Capability and Opportunity issues that influence treatment adherence for an individual.
... 16 Although many studies evaluated adherence to CF-related medications, only few of those investigated adherence to inhaled ABs. 12,13,17 Nevertheless, evidence on prevalence and predictors of nonadherence to inhaled ABs would be highly valuable to the development of effective adherenceenhancing interventions and to improve disease management of CF patients with chronic PA infection. Therefore, the aim of this systemic review was to identify level of adherence to inhaled ABs for chronic PA infection in CF and to determine the possible predictors and consequences of nonadherence on the basis of the existing publications. ...
Background:
Inhaled antibiotics (ABs) are recommended for use in the therapy of chronic Pseudomonas aeruginosa infection in patients with cystic fibrosis (CF). The aim of this systematic literature review was to identify level of adherence to inhaled ABs and to determine predictors and consequences of nonadherence in CF.
Methods:
A systematic literature search of English-language articles was conducted in April 2015 using Medline and Embase. No publication date limit was applied. The literature screening was conducted by two independent reviewers. All of the included studies were assessed for quality.
Results:
The search yielded 193 publications, of which ten met the inclusion criteria and underwent data extraction. Seven studies focused on inhaled tobramycin, one on inhaled colistimethate, one on inhaled levofloxacin, and one on inhaled aztreonam lysine. Medication adherence to inhaled ABs was analyzed by pharmacy refill history, daily phone diary, parent and child self-reports, vials counting, or electronic monitoring. In randomized controlled trials (n=3), proportion of adherent patients (>75%-80% of required doses taken) ranged from 86% to 97%; in prospective cohort studies (n=3), adherence rates ranged between 36% and 92%, and in retrospective studies (n=4) it ranged between 60% and 70%. The adherence to inhaled ABs in CF was found to be associated with the complexity of treatment, time of drug administration, age of patients, treatment burden (adverse events, taste), and patient satisfaction.
Conclusion:
The high diversity of adherence data was because of the different study designs (randomized controlled trials vs real-world studies) and the lack of a commonly accepted consensus on the definition of adherence in the reviewed articles. Routine adherence monitoring during CF care, discussing the possible reasons of suboptimal adherence with the patient, and changing treatment regimens on the basis of patient burden can individualize CF therapy for patients and may improve the level of adherence.
... Some studies show that high levels of anxiety are associated with inadequate adherence to medication in older adolescents with asthma [15]. Others found that adolescents with cystic fibrosis and anxiety disorders were more adherent to treatment than those without an anxiety disorder [16]. ...
... 16 Although many studies evaluated adherence to CF-related medications, only few of those investigated adherence to inhaled ABs. 12,13,17 Nevertheless, evidence on prevalence and predictors of nonadherence to inhaled ABs would be highly valuable to the development of effective adherenceenhancing interventions and to improve disease management of CF patients with chronic PA infection. Therefore, the aim of this systemic review was to identify level of adherence to inhaled ABs for chronic PA infection in CF and to determine the possible predictors and consequences of nonadherence on the basis of the existing publications. ...
... Illness severity can both affect and be affected by adherence, and the effects of severity appear to vary across treatment regimens and possibly family structures. In a survey study of 53 children and their caregivers, White, Miller, Smith, and McMahon (2009) found that lower (versus higher) levels of disease severity trended toward being associated with lower overall adherence. Similarly, in a study of 96 children and their parents, DeLambo, Ievers-Landis, Drotar, and Quittner (2004) found that greater disease severity was associated with mothers' reports of greater adherence to airway clearance and aerosolized medications, but not with adherence to antibiotic, enzyme, or nutrition treatments. ...
Although parents and children must adhere to five primary treatments for cystic fibrosis (CF), and their roles transition over time, the scope of CF studies often has been limited to one treatment regimen or to children within a specified age range. The purpose of this mixed research synthesis study is to integrate findings from qualitative and quantitative studies addressing the transition of CF management from parent to child, as well as factors related to adherence across treatments and over time. An existing grounded theory was used as a framework to synthesize findings in 17 reports from 16 studies. The results confirm the theory and posit 3 additional factors that may influence parent-to-child transition of care management.
... Cystic Fibrosis (CF) is a multi-system chronic condition and it is the most common life-shorting condition in the world [8]. In Tasmania, Australia CF has the second highest incidence in the world with 1 person in every 1800 people [9]. ...
This paper presents findings from the implementation of an online symptom monitoring diary that was part of a broader project aimed to support self-management of cystic fibrosis and the development of an online community of practice (CoP). The findings challenge conventional perspectives on how value is acquired by patients and their families from electronic tools designed to provide them with support. Additionally, the findings highlight complex relationships between user needs and subsequent tool use that challenge approaches to the measurement of value from ehealth projects.
... The present study found that the level of adherence to airway clearance techniques was relatively high in the current sample as well. These results suggest better adherence to airway clearance techniques than those reported in other studies (Arias Llorente et al., 2008;Passero et al., 1981;Quittner et al., 2000;White, Miller, Smith, & McMahon, 2009), which is encouraging since this is a critically important aspect of treatment for CF. However, the inconsistency of this study's findings regarding airway clearance adherence with prior study findings suggest the potential presence of demand characteristics or idiosyncratic differences in the samples. ...
... Lower and elevated rates of depression have been reported in children, adolescents and young adults who have CF compared with healthy controls [12,13]. Normal [12][13][14] and elevated levels of anxiety [15,16] have been reported in adult and adolescent patients. ...
Background
The International Depression/anxiety Epidemiological Study (TIDES) in the UK aimed: (i) to establish the prevalence of anxiety and depression amongst people with CF compared to a normative sample; (ii) to establish the association between mood, demographic and clinical variables; and (iii) to provide guidance for specialist-referral decision-making.
Methods
Patients (≥ 12 years) completed the Hospital Anxiety and Depression Scale (HADS). CF-HADS scores, expressed as percentiles, were compared with a normative sample. Multiple-regression analysis explored associations between demographic, clinical and mood variables.
Results
Thirty-nine CF centres recruited 2065 patients. Adults with CF were similar in terms of anxiety and depression to the general population. Adolescents with CF were less anxious and depressed. For adult patients, older age, unemployment for health reasons and poor lung function were associated with disordered mood. Gender-specific CF-percentile scores were calculated.
Conclusion
Surveillance, with attention to gender and risk factors is advocated. This work provides unique benchmark scores to aid referral decision-making.
This chapter addresses the problem of medication adherence and substance abuse among adolescents with liver disease. Adherence is defined as the degree to which a person’s behavior is consistent with agreed upon recommendations from a healthcare provider (World Health Organization, Adherence to long-term therapies: evidence for action. World Health Organization, 2003). This chapter first provides an overview of nonadherence in adolescents with liver disease and ways to assess for it. Next, it explores which interventions for promoting adherence show the most promise. Lastly, it addresses the growing problem of substance use as it relates to nonadherence and, more generally, among adolescents with liver disease. Most of the research in this area has focused on liver transplant recipients; however, research on nonadherence in other liver diseases has been incorporated whenever possible.
Transition from pediatric to adult healthcare for adolescents and young adults with chronic lung disease can be challenging. Individuals must develop illness self-management skills, including obtaining appropriate knowledge and the ability to independently engage in self-care behaviors. Various individual factors can facilitate or impede this process. Specifically, developmental and psychosocial variables play an important role. Understanding the role of these factors in the transition to adult care, and developing best practices to address them, is critical to helping individuals with chronic lung disease, and their families, successfully navigate transition in care.
Objective:
The purpose of this study was to develop a scale to measure patient adherence to physical therapist intervention and to report psychometric properties in patients with cystic fibrosis (CF).
Methods:
This observational, multicenter, qualitative, and cross-sectional study was divided into 2 phases: development of scale items (content validity) and assessment of psychometric properties (construct validity, reliability, and convergent/discriminant validity). Recruited were 121 patients with CF (aged ≥16 y). Sociodemographic characteristics, lung function testing, Coping with Stress Self-Efficacy (EAEAE), brief Coping Orientation to Problems Experienced inventory (COPE-28), and the AdT-Physio scale were evaluated.
Results:
The final version of the AdT-Physio scale consists of 15 items distributed across 2 subscales: compliance and beliefs about treatment/therapist. The scale showed high internal consistency (Cronbach α = 0.897; 95% CI = 0.868-0.922) and subscales above 0.800. The 2-factor confirmatory factor analysis model fitted the data reasonably well: χ2 (76) = 111.96, comparative fit index = 0.982, Tucker-Lewis index = 0.978, root mean square error of approximation = 0.063, 95% CI = 0.036 to 0.086, weighted root mean square residual = 0.720. No floor or ceiling effects were identified. There was a positive, significant, and moderate-low magnitude correlation with the total COPE-28 (r = .360) and EAEAE subscale efficacy expectations scores (r = .304). For discriminant validity, there was a positive, significant, and moderate correlation between the total and the age of the patients (r = .354).
Conclusion:
The AdT-Physio scale is psychometrically valid and reliable for use in the clinic for the assessment of adherence to physiotherapy in patients with CF.
Impact:
Ultimately, the authors propose this documentary instrument to assess the evaluation of the therapeutic alliance in a valid and objective manner. The AdT-Physio scale provides us with a greater degree of understanding of the problems behind noncompliance with treatment to advance person-centered decisions in physical therapy and thereby enhance the effectiveness of care.
In developed countries, it is projected that there will be a 70% increase in the number of adults living with Cystic Fibrosis (CF) between 2010 and 2025. This shift in demographics highlights the importance of high-quality transition programmes with developmentally appropriate integrated health care services as the individual moves through adolescence to adulthood.
Adolescents living with CF face additional and unique challenges that may have long-term impacts on their health, quality of life and life-expectancy. CF specific issues around socially challenging symptoms, body image, reproductive health and treatment burden differentiate people with CF from their peers and require clinicians to identify and address these issues during the transition process.
This review provides an overview of the health, developmental and psychosocial challenges faced by individuals with CF, their guardians and health care teams considering the fundamental components and tools that are required to build a transition programme that can be tailored to suit individual CF clinics.
Significant advancements in the treatment of cystic fibrosis (CF) have led to drastically improved medical outcomes for those living with this illness. While these improvements have increased life expectancy and resulted in better quality of life, managing the daily care required to stay healthy is burdensome and complex. A time-intensive daily CF treatment regimen may be difficult to maintain and can interfere with many aspects of one’s life. Many factors impact how well an individual adheres to recommendations of their healthcare team. Understanding the barriers and facilitators of treatment adherence in CF is critical as well as the interventions available to help those with this disease live better lives while managing their health.
Mental and physical health go hand in hand. Mental health challenges including depression and anxiety impact patients with cystic fibrosis (CF) and, ultimately, their health outcomes. This chapter provides an overview of various mental and psychosocial difficulties facing the CF community and recommendations for effective prevention, screening, and treatment of mental health concerns. The majority of research to date has focused on the consequences of anxiety and depression on CF adherence, health outcomes, quality of life, and family functioning. This chapter includes a brief discussion of other mental health considerations such as suicidality, disruptive behavior problems, and eating disorders and their treatments to provide a broader view of mental health in CF. Recommendations provided by the International Committee on Mental Health in Cystic Fibrosis (ICMH) for mental health screening and procedural follow-up as well as psychological and pharmacological treatments for disorders in the CF community are included.
Importance
Children, adolescents, and young adults with life-limiting conditions experience various challenges that may make them more vulnerable to mental health problems, such as anxiety and depression. However, the prevalence and incidence of anxiety and depression among this population appears to be unknown.
Objective
To conduct a systematic review and meta-analysis to estimate the prevalence and/or incidence of anxiety and depression in children, adolescents, and young adults with life-limiting conditions.
Data Sources
Searches of MEDLINE (PubMed), PsycInfo, and Embase were conducted to identify studies published between January 2000 and January 2018.
Study Selection
Studies were eligible for this review if they provided primary data of anxiety or depression prevalence and/or incidence, included participants aged 5 to 25 years with a life-limiting condition, were conducted in an Organisation for Economic Co-operation and Development country, and were available in English.
Data Extraction and Synthesis
Random-effects meta-analyses were generated to provide anxiety and depression prevalence estimates. Meta-regression was conducted to analyze associations between study characteristics and each prevalence estimate.
Main Outcomes and Measures
Prevalence of anxiety and depression.
Results
A total of 14 866 nonduplicate articles were screened, of which 37 were included in the review. Of these, 19 studies reported anxiety prevalence, and 36 studies reported depression prevalence. The mean (range) age of participants was 15.4 (6-25) years. The meta-analysis of anxiety prevalence (n = 4547 participants) generated a pooled prevalence estimate of 19.1% (95% CI, 14.1%-24.6%). Meta-regression analysis found statistically significant differences in anxiety prevalence by assessment tool; diagnostic interviews were associated with higher anxiety prevalence (28.5% [95% CI, 13.2%-46.8%]) than self-reported or parent-reported measures (14.9% [95% CI, 10.9%-19.4%]). The depression meta-analysis (n = 5934 participants) found a pooled prevalence estimate of 14.3% (95% CI, 10.5%-18.6%). Meta-regression analysis revealed statistically significant differences in depression prevalence by the mean age of the sample (β = 0.02 [95% CI, 0.01-0.03]; P = .001).
Conclusions and Relevance
In this systematic review and meta-analysis, the prevalence of anxiety and depression among children, adolescents, and young adults with life-limiting conditions was high, highlighting the need for increased psychological assessment and monitoring. Further research is required to determine the prevalence and incidence of anxiety and depression in a larger sample of children, adolescents, and young adults with a broader range of life-limiting conditions.
In an effort to identify pathways for improvements in clinical monitoring and intervention, the current study investigated the role that parenting style plays in treatment adherence and responsibility for youth with CF. Participants (n = 50) completed questionnaires related to treatment adherence, treatment responsibility, and parenting style at baseline and at a two year follow up visit. Aspects of parenting style (e.g., warmth, autonomy support) were related to youth adherence and responsibility in cross-sectional and prospective analyses. These data suggest that aspects of parenting may be important targets of interventions to promote treatment adherence in youth with CF.
Objectives. A meta-analysis examined family functioning and medical adherence in children and adolescents with chronic health conditions. Family functioning was evaluated at the level of the family unit, as well as parent–child interactions.
Methods. We conducted literature searches using PubMed, PsycINFO, SCOPUS, Web of Science, and Cochrane. After reviewing 764 articles, 62 studies met eligibility criteria. Pearson’s r correlations were the effect size of interest. We conducted both omnibus and domain-specific (e.g., family conflict, cohesion) meta-analyses. Meta-regressions examined whether relevant covariates related to the magnitude of the effect.
Results. The omnibus meta-analysis showed that family functioning was significantly related to medical adherence across a variety of pediatric chronic health conditions. Lower family conflict, greater family cohesion, greater family flexibility, more positive communication, and better family problem-solving were each associated with better adherence. There were no significant differences in the magnitude of the omnibus effect based on child age, measurement features (subjective vs. objective or bioassay adherence; family unit vs. parent–child interactions), or study quality.
Conclusions. Consistent with social–ecological frameworks, findings supported links between family functioning and medical adherence. This study highlights several limitations of the extant research, including absence of a guiding theoretical framework and several methodological weaknesses. We offer clinical and research recommendations for enhancing scientific understanding and promotion of adherence within the family context.
Cystic fibrosis has been associated with higher risks of depression and anxiety in both patients and their caretakers. Anxiety specifically has been found to be the driving force of the psychopathology. Proposed causes of depression and anxiety in CF patients include pulmonary symptoms, especially dyspnea and air hunger, as well as vitamin D deficiency, abnormal body image, social stigma, time-consuming treatment regimens, and facing their own mortality at a young age. The cystic fibrosis care guidelines do not currently address screening for depression and anxiety or recommend disease-specific treatment. Depression and anxiety are very treatable as primary processes; thus, as the longevity of CF patients increases, more focus is being placed on addressing screening and treatment of psychopathology in patients affected by the disease.
Purpose
Tobramycin inhalation powder (TIP), the first dry-powder inhaled antibiotic for pulmonary Pseudomonas aeruginosa infection, is associated with reduced treatment burden, increased patient satisfaction, and higher self-reported adherence for cystic fibrosis (CF) patients. We compared adherence in CF patients newly treated with TIP with those newly treated with the traditional tobramycin inhalation solution (TIS), using US insurance claims data.
Patients and methods
From the Truven MarketScan® database, we identified CF patients chronically infected with P. aeruginosa who had been prescribed TIP between May 1, 2013 to December 31, 2014, or TIS between September 1, 2010 to April 30, 2012 with at least 12 months of continuous medical and pharmacy benefits prior to and following prescription. TIP and TIS adherence levels were assessed.
Results
A total of 145 eligible patients were identified for the TIP cohort and 306 for the TIS cohort. Significant differences in age distribution (25.0 vs 21.9 years for TIP vs TIS, respectively, P=0.017), type of health plan (P=0.014), employment status (72.4% vs 63.4% of TIP vs TIS patients in full-time employment, P=0.008), and some comorbidities were observed between the two cohorts. Although a univariate analysis found no significant differences between TIP and TIS (odds ratio [OR] 1.411, 95% confidence interval [CI] 0.949–2.098), TIP was moderately associated with higher adherence levels compared with TIS in a multivariable analysis, once various demographic and clinical characteristics were adjusted for. These included geographic location (OR: 1.566, CI: 1.016–2.413) and certain comorbidities.
Conclusion
This study of US patient data supports previous findings that TIP is associated with better adherence compared with TIS; however, further studies will be required to fully elucidate differences in adherence between TIP and TIS.
Aim:
Patient adherence is integral to the effectiveness of prescribed treatment, and is associated with beneficial disease outcomes, yet in adolescents with cystic fibrosis, adherence is often sub-optimal. Multiple factors may contribute to treatment adherence, including disease knowledge and self-efficacy. In adolescents with cystic fibrosis: (i) to compare the disease knowledge of adolescents and their parents before transition to adult care; (ii) to determine the relationship between disease knowledge (adolescent, parent) and adherence; and (iii) to evaluate self-efficacy and its association with disease knowledge and adherence.
Methods:
Adolescents with cystic fibrosis and their parents were recruited from a tertiary children's hospital. Disease knowledge and self-efficacy was assessed using the Knowledge of Disease Management-CF and General Self-Efficacy Scales respectively. Using pharmacy records, medication possession ratio was calculated to measure treatment adherence in the preceding year.
Results:
Thirty-nine adolescent (aged 12-17 (median 14) years) and parent pairs were recruited. Adherence to hypertonic saline, but not other medications, was significantly associated with disease knowledge in adolescents (r (2) = 0.40, P = 0.029). Mean (SD) adolescent self-efficacy was 30.8 (4.0), and not associated with disease knowledge or adherence. Mean (SD) disease knowledge was less in adolescents than parents (55 (16)% and 72 (14)% respectively, P < 0.001).
Conclusion:
Disease knowledge is sub-optimal in adolescents with cystic fibrosis, even in the 2 years immediately before transition to adult care. Given that adherence with some treatments has been associated with disease knowledge our results suggest the need for educational interventions in adolescents with cystic fibrosis to optimise self-management and health outcomes.
For children with chronic conditions, well-being is closely related to how well their families function. Most prior research syntheses on family functioning and child well-being have focused on children with a single condition, thereby limiting the potential to aggregate and build on what is known across conditions. To address this challenge, research reports were reviewed and meta-analyses conducted of findings on the relationship between family functioning and child well-being across a range of chronic physical conditions. The sample was derived from a larger systematic review study that included 1,028 reports published between January 1, 2000 and March 31, 2014. The current review includes 53 studies in which a relationship between family functioning and child well-being was analyzed using one of four family measures: Family Adaptability and Cohesion Evaluation Scales, Family Environment Scale, Family Relationship Index, or Family Assessment Device. Most studies were cross-sectional and observational (n = 43, 81%). The most frequently studied conditions were diabetes, cancer, sickle cell disease, and asthma. In 37 studies, findings were sufficiently comparable to conduct meta-analyses. Significant correlations were identified between children's psychological health and seven of nine dimensions of family functioning. Significant correlations also were found between dimensions of family functioning and children's problem behaviors, social competence, quality of life, and, to a lesser extent, adherence and physical health. Of the family dimensions, cohesion and conflict were associated most strongly with child outcomes. Understanding the specific family variables, such as conflict, linked to varied child outcomes is key for intervention development. © 2016 Wiley Periodicals, Inc.
Der folgende Beitrag analysiert Forschung auf dem Gebiet chronischer Erkrankung von Kindern und Jugendlichen mit dem Schwerpunkt Bewältigungsverhalten und Krankheitsmanagement. Im Kontext von Familien mit chronisch kranken Kindern und Jugendlichen werden wir in diesem Kapitel die individuelle Bewältigung durch das Kind bzw. den Jugendlichen, die elterliche Bewältigung durch die Mutter und, seltener, den Vater sowie die familiäre Bewältigung, d. h. die vereinten Bewältigungsbemühungen einer Familie, betrachten.
In cystic fibrosis (CF) the mucociliary clearance mechanism is impaired and airway clearance techniques aim to compensate for this by promoting secretion clearance. A substantial body of research has been unable to provide clear evidence for one technique over another. This is in part due to limitations in the study designs used to assess the effects of the techniques. There is great variability in the airway clearance techniques used at patient, centre and country level. Adherence to airway clearance techniques is consistently low and there is an urgent need to focus research on interventions to overcome key barriers to non-adherence. Airway clearance techniques also need to be coordinated with mucoactive medications to ensure the overall effect is optimised. Recent trials have identified the challenges related to using forced expiratory volume in 1 s (FEV1) as a primary outcome measure for airway clearance trials. New testing methods, for example multiple-breath washout and radio-imaging studies, may improve our understanding of the mechanisms underlying airway clearance as well as offering potential alternate clinical end-points.
Poor nutritional status in cystic fibrosis (CF) leads to poor health outcomes. There are a range of contributors to poor nutrition, and deliberate manipulation of food consumption is one of them. Although cases of diagnosable eating disorders are not identified at high rates, it has been consistently reported that people with CF engage in food avoidance and other practices to reduce weight gain. Attention has been paid to these practices, variously referred to as “subthreshold eating disorders” and “disturbed eating.” They have been documented in CF at high rates, sometimes higher than in the general population, with the suggestion that CF itself can stimulate such practices, as there is high emphasis placed on weight gain and fat consumption in the management of the condition. Recent attention has been paid to better identification and diagnosis of disturbed eating attitudes and behaviors in CF so that the multidisciplinary team can provide appropriate treatment interventions. These measures are discussed.
This qualitative study asked two questions: 1) How do teens with cystic fibrosis (CF) feel about their treatments; and 2) What factors lead teens to adhere, or not adhere, to treatments. To answer these questions we used an innovative approach (Video Intervention/Prevention Assessment or VIA), to learn about the experiences of teens with CF. We loaned video camcorders to teens with CF and asked them to create visual narratives of their lives. Researchers logged and coded videotapes, identifying themes that arose from the material. A primary theme was “Doctors don’t understand.” Participants also highlighted the value of routines and parental support in consistently doing treatments. We identify and discuss themes that emerged and discuss how findings can be used to help providers improve care, develop partnerships with patients and increase adherence.
The management of respiratory diseases with organic causes often can be complicated by the emergence of concurrent functional respiratory symptoms. Thus, clinicians must be alert to the possibility that functional symptoms may develop in this setting. For example, anxiety with associated dyspnea can affect the management of many patients with significant respiratory disease such as asthma, chronic obstructive pulmonary disease, and cystic fibrosis (CF). An understanding of functional aspects of respiratory disorders will aid the clinician in identifying the correct cause of symptoms. In turn, this will help guide the application of appropriate therapy, thus preventing unnecessary tests and treatments that may compound an existing medical problem or create a new one. As an example illustrating how clinicians can approach patients with a respiratory disease that can have both organic and functional components, this chapter focuses on patients with CF. Case studies are presented through which the clinician will gain a better appreciation for the interaction of functional and organic causes of symptoms in this setting.
Background:
Nebulised medicines contribute to the high treatment burden experienced by patients with cystic fibrosis (CF).
Objectives:
This study explored experiences of adult patients with CF when using nebulised medicines, factors impacting on their adherence to nebulised therapy and strategies they used to facilitate adherence.
Setting:
Community setting, in Sydney, Australia.
Methods:
Ten patients with CF were recruited through a CF patient organisation. Semi-structured face-to-face interviews were conducted, addressing the study objectives. Interviews were audio-recorded, transcribed verbatim and content analysed for anticipated and emergent themes.
Main outcome measure:
Experiences with using nebulised medicines; and barriers and facilitators of adherence to nebulised medicines.
Results:
Participants' age ranged from 22 to 45 years, with half being male. Four broad themes (with more specific sub-themes) were identified from the interviews: experiences with using nebulised medicines (cleaning nebuliser, time taken to use nebuliser medicine, flexibility in use of nebuliser); feelings about using nebulised medicines (necessary/important, dislike, part of life); factors impacting non-adherence (time consuming therapy, side effects/effects of medicine, work/social demands, lack of perceived importance); factors and strategies facilitating adherence (perceived medicine importance, habit/routine, support, health benefits, technology/medicine dose form, timetabling).
Conclusions:
Nebulised therapy for cystic fibrosis patients takes a substantial amount of time, with patients trying to alter their routine to incorporate nebulising into their daily lives. However there are still many factors that lead to low adherence, including work/social demands and travelling. Patients balance the necessity for nebulised therapy against the barriers, and engage in intentional non-adherence at times. Future strategies and resources should target and address specific factors identified by patients with CF as being important and impacting their adherence to nebulised medicines.
Background
There is scarce evidence on the epidemiology of attention-deficit/hyperactivity disorder (ADHD) in patients with cystic fibrosis (CF).
Methods
This was a cross-sectional analysis of in- and outpatient billing data for Medicaid beneficiaries with cystic fibrosis ages 3-18 years to obtain ADHD diagnosis prevalence and incidence estimates from 1999 to 2006. We employed stepwise logistic regression to examine the association between ADHD diagnosis and selected patient characteristics.
Results
Annual ADHD prevalence increased 1.55-fold from 5.26% (95% confidence interval [CI]): 5.25-5.27) to 8.16% (8.15-8.17) and annual ADHD incidence rose slightly from 1.70% (1.70-1.71) to 2.01% (2.00-2.01). As in the general population, males were significantly more likely to have a diagnosis of ADHD compared to females (odds ratio [OR]: 1.97 [CI: 1.49-2.60]), as were children with recent diagnoses of anxiety, emotional disorder, depression, adjustment disorder and learning, motor and communication disorders. Patients with ADHD diagnoses were also more likely to be in foster care (OR: 4.36 [CI: 2.26-8.40]). Except for recent DNase use (OR: 0.64 [CI: 0.43-0.93]), CF severity indicators and medications including pancreatic enzymes, inhaled tobramycin, inhaled or oral corticosteroids, inhaled bronchodilators and oral antibiotics had no association with ADHD diagnosis.
Conclusion
ADHD prevalence in CF increased during the study period. Clinical and socio-demographic determinants of ADHD diagnosis were similar to the general population, while treatment and severity of CF appeared to have little influence. Our findings warrant future research evaluating diagnostic protocols and assessment of safety and efficacy of ADHD treatment in children with CF.
Cystic fibrosis is a genetic disorder that damages many of the body's organs and shortens life expectancy. Often people with cystic fibrosis and family members need help to adjust psychologically and emotionally. They also need help to manage the demands of treatment. We looked for trials of psychological treatments in people with cystic fibrosis and their families. The review includes 13 studies with 529 people of mainly behavioural and educational treatments. Some treatments aimed to improve people's ability to cope with the disease or genetic risk. Other treatments aimed to improve dietary intake and boost the effectiveness of chest physiotherapy. Some evidence showed that behavioural treatments can improve emotional outcomes for people with cystic fibrosis and their carers. We also found some evidence that psychoeducational treatments can improve knowledge in the short term. In addition, educational and behavioural treatments can help nutrition and growth in people with cystic fibrosis. However, at the moment there is no clear evidence to show the best psychological treatments to help people with cystic fibrosis and their carers. There should be multicentre trials to improve adherence to treatment.
Assessed the role of illness parameters, demographic parameters, and hypothesized psychosocial/mediational processes in the psychological adjustment of 68 mothers of children and adolescents (7-17 years of age) with cystic fibrosis. Together the illness and demographic parameters accounted for only 13-15% of the variance in maternal adjustment. However, the hypothesized mediational processes accounted for 35-40% increment in the variance in maternal adjustment. More specifically, maternal adjustment was associated with lower levels of perceived daily stress, less use of palliative coping methods, and family functioning characterized by high levels of supportiveness.
One hundred patients aged 5 through 20 years with cystic fibrosis, their parents, and their physicians were interviewed to assess patients' perceptions of compliance with prescribed treatments. Measures included self-administered questionnaires and the Medical Compliance Incomplete Stories Test. It was hypothesized that age-related differences in perceived compliance would be found and that such perceptions would vary as a function of four sociobehavioral factors (perceived severity of illness, optimism, level of independence in medical therapy, and degree of knowledge/understanding of illness). In addition, it was anticipated that the relationships between perceived compliance and the sociobehavioral factors would vary with age. Support for each hypothesis was found. Perceived compliance was related to age, with younger children showing greater perceived compliance to the medication regimen. Better perceived compliance was found to be associated with higher levels of optimism and child knowledge of the disease. Age differences in relationships between perceived compliance and optimism and child knowledge were found. Developmental implications for practice and future research are suggested.
This investigation evaluated the utility of a new assessment tool based on a competency/coping skills model in predicting medical compliance of adolescents with cystic fibrosis. The Medical Compliance Incomplete Stories Test (M-CIST) and an assessment of several coping behaviors identified as assisting chronically ill children in their adaptation were used with 40 adolescent inpatients aged 13-23. These data were compared to objective measures of the patient's medical compliance. The total M-CIST score was shown to be positively correlated with compliance and to significantly discriminate between compliant and non-compliant groups. Although the assessment of coping behaviors also discriminates significantly between the two groups, it provided no additional power when used in common with the M-CIST. The data identified differences in coping behaviors between compliant and non-compliant patients which may prove useful to clinicians treating adolescents with cystic fibrosis.
The study comprised three interrelated aims: (1) to ascertain (a) patient compliance with physiotherapy, exercise, enzyme and vitamin regimens, (b) how compliance was perceived by patients, and (c) the reasons for poor compliance (2) to identify demographic and clinical variables associated with compliance; and (3) to determine how accurately patient compliance can be predicted by carers.
Demographic and medical history data were obtained from medical records and a patient questionnaire. The data obtained included age, sex, employment status, inpatient or outpatient status, frequency of contact with the clinic, age at diagnosis, and the number of years practising physiotherapy. Measures of clinical status, including FEV1 and FVC percentage predicted, Shwachman score, and 24 hour sputum weight were recorded before completion of the questionnaire. The questionnaire, administered by a psychologist, assessed the reported degree of patient compliance, their perception of compliance, and their reasons for poor compliance.
Sixty patients participated in the study and 51/60 and 41/55 patients were considered compliant with enzyme and exercise therapies, respectively. Compliance was lower with physiotherapy (32/60) and vitamin treatment (21/45). Patients reporting immediate benefits following exercise and physiotherapy were more compliant than those reporting no improvement. The perception by patients that compliance was sufficient ("about right") was physiotherapy 67%, exercise 37%, enzymes 78%, and vitamins 9%. Compliance was not influenced by demographic details nor by severity of disease, although patients producing large amounts of sputum and receiving help with physiotherapy were more compliant with physiotherapy. The physiotherapist and physician judged correctly the degree of compliance with physiotherapy in 83% and 75% of cases, respectively, and with exercise in 68% and 67% of cases, respectively.
The reported degree of compliance and reasons for poor compliance were treatment specific. Demographic and disease severity variables were not associated with compliance. Those involved in the care of patients with cystic fibrosis were able to predict patient compliance.
To examine the concept of treatment alliance in the care of adolescents with asthma.
Measures of treatment alliance were obtained from 60 adolescents with severe, chronic asthma admitted to a tertiary care facility, their parents, and their asthma specialists at the referral center. Associations were examined between the alliance measures and multimeasure, multiagent data concerning family functioning and asthma treatment adherence and outcome.
Physicians' reports of treatment alliance were associated with concurrent family functioning and asthma medication adherence, as well as with asthma treatment adherence and outcome in the year after the stay at the asthma center.
As emphasized in the recently revised Expert Panel Report: Guidelines for the Diagnosis and Management of Asthma (NHLBI, 1997), the ability of children with asthma, their parents, and their physician to create a positive partnership, or treatment alliance, appears to have an important role in optimal asthma management.
Chest physiotherapy (CPT) has been an important part of cystic fibrosis (CF) treatment regimens for > 40 years. Techniques with different perceived costs, benefits, and patient satisfaction exist. An instrument measuring patient satisfaction with CPT has not been reported. Our goal was to develop and validate such an instrument.
A cross-sectional survey sent to 349 patients seen at a large, urban, academic CF care center. The two-page survey asked 17 questions related to CPT satisfaction (efficacy, convenience, comfort, overall satisfaction), followed by 4 general CF-care questions (disease severity, importance of therapies, prescribed vs missed therapies). A 5-point Likert-type scale was used for responses. Psychometric analysis included itemetric performance, confirmatory factor analysis, test-retest reliability, and evaluation of subject's responses to the general CF-care questions.
One hundred twenty-nine individuals returned completed surveys (39%; 66 males and 63 females; age range, 2 months to 47 years). FEV(1)values were 21 to 155% predicted (mean, 76%; n = 82). Disease severity was as follows: 60 mild, 47 moderate, and 14 severe. Seventy-nine subjects used postural drainage, percussion, and vibration (PDPV), 21 used a flutter device, and 14 used high-frequency chest wall oscillation (HFCWO). Five subjects used more than one technique. Internal consistency analysis found an overall coefficient alpha of 0.87 (range, 0.74 to 0.89 for four domains). Factor analysis demonstrated domains for efficacy, convenience, comfort, and overall satisfaction. Mean total satisfaction scores differed significantly among therapies (R(2) = 0. 118; F[ 2,111] = 7.56; p = 0.0008): PDPV, 3.8 (SD = 0.6); Flutter, 4. 3 (SD = 0.5); and HFCWO, 4.1 (SD = 0.5). Therapies also differed significantly on all subscores. Perceived importance of CPT and compliance with CPT increased linearly with disease severity. Overall satisfaction was positively correlated with CPT compliance.
The CPT satisfaction survey has good reliability and content validity. Significant differences in patient satisfaction exist among therapies. Sicker patients recognize the importance of, and demonstrate better compliance with prescribed CPT. Increased satisfaction is associated with better compliance with therapy.
Cystic fibrosis (CF) is now as much a disease of adults as of children and adolescents. An important focus of recent research has been the impact of the complex, expensive, and time consuming routine of self-care and medical treatment that is required by adults with CF to maintain health. A growing concern for medical and allied health teams is the issue of patient adherence to prescribed health management plans. A summary of the particular medical and treatment context of CF is followed by a review of adherence measurement issues and the determinants of adherence to treatment regimens in people with CF, primarily adults. Evidence for factors which influence adherence decisions of people with CF is examined. The medical and psychological aspects of this complex problem have not been adequately addressed because of difficulties with definition and measurement. Only a small proportion of the variance in adherence has been accounted for in the literature. New measurement technologies and new theoretical directions offer promise for a better understanding of this complex and important issue and may result in more effective intervention strategies to improve adherence.
Cystic fibrosis is a fatal chronic illness that primarily affects the respiratory and pancreatic systems. Treatment includes daily medications, enzyme and vitamin supplements, a high-calorie diet, and airway clearance sessions (e.g., chest physiotherapy, exercise). Although this regimen is essential to longevity, families have difficulty adhering to the multiple treatment components. Adherence is especially problematic with diet, chest physiotherapy, and exercise. Studies utilizing behavioral techniques to increase adherence to cystic fibrosis treatment components have been conducted with varying results. In this paper, a critical review of these treatment studies and suggestions for future work are presented.
To examine associations between observations of the quality of family relationships and problem-solving skills and reported adherence to medical treatments for older children and adolescents with cystic fibrosis (CF).
Reports of adherence were obtained from 96 youth with CF and their parents recruited from six CF centers in the Midwest and southeastern United States. Videotaped observations of family discussions of high conflict issues were used to assess quality of relationships and problem-solving skills.
Hierarchical regression analyses indicated that observed family relationship quality (RQ) was related to parent and child reports of adherence to airway clearance and aerosolized medications after controlling for demographic variables and illness severity. Observed family problem solving was not a significant predictor after controlling for RQ.
Older children and adolescents who come from families experiencing unhappy and conflicted relationships may be at greater risk for poor adherence to treatments; thus, family relationships are appropriate targets for interventions aimed at improving adherence.
The purpose of this study was to systematically identify barriers to treatment adherence for children with cystic fibrosis (CF) and asthma, as well as to examine the relationship between the number of barriers and adherence.
Participants included 73 children with CF or asthma and their parents. The mean age of the sample was 9.9 years, and 58% were males.
Results indicated that barriers were quite similar by illness and informant (e.g., parent and child) for the same treatments, but unique barriers were identified for disease-specific treatments. Frequently mentioned barriers across diseases included forgetting, oppositional behaviors, and difficulties with time management. Trends were identified between adherence and barriers, suggesting that a greater number of barriers were related to poorer adherence.
Overall, this study provided evidence that patients and their parents experience specific barriers within the context of their own illness and highlights the need for disease-specific measures and interventions.
The present research was designed to investigate the adjustment of patients with cystic fibrosis (CF) and their families as well as the relationship between adjustment and physician perceptions of compliance with CF treatment. Patient and family adjustment was assessed by means of the Personality Inventory for Children as well as measures of marital adjustment, depression, and social isolation completed by mothers of CF patients. Results indicated no characteristic pattern of psychopathology or adjustment problems. However, higher levels of perceived compliance with CF treatment were found to be associated with less satisfactory marital relationships and with less frequent maternal social contacts. These findings are discussed in terms of achieving a balance between compliance and psychological adjustment.
OBJECTIVE: To examine the concept of treatment alliance in the care of adolescents with asthma. METHODS: Measures of treatment
alliance were obtained from 60 adolescents with severe, chronic asthma admitted to a tertiary care facility, their parents,
and their asthma specialists at the referral center. Associations were examined between the alliance measures and multimeasure,
multiagent data concerning family functioning and asthma treatment adherence and outcome. RESULTS: Physicians' reports of
treatment alliance were associated with concurrent family functioning and asthma medication adherence, as well as with asthma
treatment adherence and outcome in the year after the stay at the asthma center. CONCLUSIONS: As emphasized in the recently
revised Expert Panel Report: Guidelines for the Diagnosis and Management of Asthma (NHLBI, 1997), the ability of children
with asthma, their parents, and their physician to create a positive partnership, or treatment alliance, appears to have an
important role in optimal asthma management.
Cystic fibrosis (CF) is an autosomal recessive inherited disorder caused by mutations of both alleles of the CF gene. These mutations result in abnormalities in the production or function of the gene product, a protein known as the cystic fibrosis membrane conductance regulator (CFTR). The CF gene is large (250 000 base pairs) and located on chromosome 7. A deletion of three base pairs that results in the absence of phenylalanine at codon 508 is the most common defect (designated F 508) and accounts for about 70% of the mutant genes in North American populations. Other genotypes observed with some frequency (but <3%) are G542X, G551D, W1282X, W1303K, and R553X. The twelve most common CF gene mutations account for approximately 85% of cases in North America. Mutations other than F508 number more than 200 and account for the remaining cases. Most of these 200-odd mutations are "private," observed only in the affected kindred. Because the CF locus has so many DNA sequence changes associated with disease, precise genetic testing cannot replace clinical criteria for diagnosis, and the sweat test remains an important diagnostic test.
The CF gene is expressed in epithelial cells of the pancreas, sweat and salivary glands, intestine, and reproductive and respiratory tracts, particularly in the submucosal glands.
family systems and stress across the family life cycle / changes in a couple system in early marriage / changes in a family system related to physical illness / studies validating the Circumplex Model / update on inventories for family assessment [Cirumplex Assessment Package and Clinical Rating Scale] / goals of family therapy using the Circumplex Model (PsycINFO Database Record (c) 2012 APA, all rights reserved)
Background:
Most patients with cystic fibrosis (CF) require a higher energy and protein intake than their healthy peer group. There are few data on dietary intakes of adult patients. The aim of this study was to determine nutritional intakes in an adult population with CF. The impact of nutritional intervention and disease on macronutrient intake was examined.
Methods:
Retrospective cross-sectional analysis of 94 unweighed food diaries at annual review (1995-2000). Energy and protein intakes were compared to the estimated average requirement (EAR) for energy and reference nutrient intake (RNI) for protein. The effect of diet alone, oral supplements, enteral tube feeding, and cystic fibrosis related diabetes (CFRD), on macronutrient intake was examined and impact of pancreatic sufficency (PS) and lung transplantation.
Results:
Mean energy and protein intakes approached recommended CF guidelines, but in 72% of assessments these values were not achieved. Mean energy and protein intakes for patients on diet alone and protein intake for those with CFRD failed to meet recommendations. Oral supplementation and enteral tube feeding regimens increased energy and protein intake above recommended levels. No group achieved 40% total energy from fat. Patients receiving enteral tube feeds had the highest mean energy and protein intakes but lowest body mass index (BMI) and lung function.
Conclusion:
Adequate mean energy and protein intakes in adult patients with CF mask subgroups of patients who fail to meet recommendations ie. diet alone, diabetic. Oral supplementation and enteral tube feeding increase energy and protein intake but fail to achieve an adequate BMI level in subjects with a decreased clinical status. Individual nutritional assessment remains essential.
• Sixty-one patients with cystic fibrosis were studied to determine the relationship between degree of compliance with taking antibiotics and severity of the disease. The compliance rate was high in these patients, suggesting that perception of the severity of the disease and the potential consequences of discontinuing medication is a principal factor.
(Am J Dis Child 129:1011-1013, 1975)
Found 60% of children 7–12 years old with cystic fibrosis to have a parent-reported behavior problem and 62% met the criteria
for a DSM-III diagnosis based on a structured clinical interview with the child. Mixed internal and external behavior problem
patterns and diagnoses of anxiety and oppositional disorder were most frequent. Support was provided for the hypothesized
psycho-social/mediational roles of child perception of self-worth and maternal anxiety in child adjustment. Together, the
variables of the transactional stress and coping model accounted for 39 and 43% of the variance in mother-reported internalizing
and externalizing behavior problems and for 68% of the variance in child-reported problems.
Reviewed 87 studies of children's adjustment to physical disorders in a meta-analysis. Results indicate (a) children with such disorders show increased risk for overall adjustment problems, internalizing and externalizing symptoms; (b) risk was greatest in studies making comparisons to norms rather than to study controls; (c) risk varied by informant (teacher, mental health professional, parent), and by degree of matching with controls; (d) the self-concept of children with physical disorders across all studies appears significant for studies with careful matching or comparisons with norms; (e) there are interdisease differences, but the number of studies within individual disorders, with a few exceptions, are quite small. The need for improved methodology is discussed.
The present research was designed to investigate the adjustment of patients with cystic fibrosis (CF) and their families as well as the relationship between adjustment and physician perceptions of compliance with CF treatment. Patient and family adjustment was assessed by means of the Personality Inventory for Children as well as measures of marital adjustment, depression, and social isolation completed by mothers of CF patients. Results indicated no characteristic pattern of psychopathology or adjustment problems. However, higher levels of perceived compliance with CF treatment were found to be associated with less satisfactory marital relationships and with less frequent maternal social contacts. These findings are discussed in terms of achieving a balance between compliance and psychological adjustment.
The creative challenge of health education for chronic illnesses is the translation of theory-based intervention methods into practical strategies that can be organized into a logical series of learning activities to influence changes in environmental, cognitive, or behavioral factors. A case example describing the development and implementation of a comprehensive health-education intervention for the self-management of cystic fibrosis (CF) is presented. The design of intervention strategies began with an assessment of the educational needs for self-management of CF, followed by specification and validation of particular self-management behaviors. Behavioral and learning objectives then were formulated for each of the self-management behaviors. Constructs from social learning theory considered to be important influences on specified self-management behaviors in CF were identified. Taking into consideration the learning needs of the target population and the practical constraints of the system for providing health care, various intervention methods then were devised based on social learning theory. Lastly, the intervention methods chosen were translated into strategies organized into a series of practical learning activities for CF patients and their families. The process described here should prove useful to others who are planning and developing comprehensive health education programs for self-management of chronic illnesses.
This study examined the relationship between parents' and physicians' reports of treatment-related behaviour and adjustment problems of children with cystic fibrosis (CF), and children's clinical status as assessed by measures of pulmonary functioning and global ratings of clinical status. Parents completed a Cystic Fibrosis Problem Checklist which measures the extent to which children experience behaviour and adjustment difficulties with different aspects of treatment, including chest physiotherapy, medication taking and diet. A high proportion of parents in each age group reported at least some treatment-related behaviour difficulties, with significantly more problems being reported with younger children. There was no significant relationship, however, between the child's age, sex and clinicians' ratings of compliance. A stepwise multiple regression showed that the only variables which significantly predicted clinicians' ratings of the severity of the child's disease were forced expired volume and height. Clinicians' ratings of compliance explained a small amount of extra variance (2.4%). It was found that parental reports of treatment-related behaviour problems were not related to clinical status. The implications of the findings for clinical practice are discussed.
Psychiatric symptomatology was compared in 61 cystic fibrosis patients, ages 8 to 15 years, and 36 cystic fibrosis patients, ages 16 to 40 years. When the prevalence of psychiatric symptomatology was compared in the two groups, a developmental pattern emerged: Symptoms of depression and anxiety were more frequent in the older group, while symptoms associated with eating disorders were more frequent in the younger group. Although duration of illness was longer and severity of illness was greater in the older group, these factors were largely unrelated to psychiatric symptomatology in either group. It is hypothesized that younger patients may express psychological distress through less direct means than older patients.
Thirty cystic fibrosis (CF) and 30 matched control children and their parents were administered several psychiatric inventories including the child (DICA) and parent (DICA-P) versions of the Diagnostic Interview for Children and Adolescents, the Child Behavior Check List, the Hopelessness Scale, and the Piers-Harris Children's Self-Concept Scale. Data analysis revealed few differences in either psychopathological symptoms or psychiatric diagnoses between the CF and control children. The differences which did emerge were either physical in nature (reflecting somatic complaints) or did not depart enough from normal scores to merit the label of high psychopathology. The results are discussed in terms of the growing evidence that CF children do not suffer from greater psychopathology than do normal children.
One hundred and twenty-six 6 to 11-year-old latency age children with Cystic Fibrosis regularly attending the CF clinic of the Hospital for Sick Children were asked to complete the Piers-Harris Self-Concept Scale and the Children's Health Locus of Control. Their parents were requested to complete the Child Behavior Checklist and the Family Assessment Measure. One hundred and eight (86%) participated in the study. Twenty-three percent of the children were found to have sufficient behavior problems to indicate a significant degree of maladjustment. Latency-age CF children show an increase in problems compared to a pre-school group, suggesting that leaving the protection of the family is problematic for a child with a chronic physical disorder. Males show more behavior problems than females. Males' behavior is characterized by somatic complaint profile. In spite of difficulties, CF latency children are able to maintain good social competence and self-concept suggesting compensatory mechanisms. These mechanisms are different for males and females. Females' self-concept and social competence are supportive of each other, whereas for males, this is not the case. Similarly, female behavior is relevant to family functioning. Males and females adjust to difficulty as indicated by differences in behavior profiles.
Eighty-five 12- to 15-year-old adolescents regularly attending the cystic fibrosis (CF) clinic of The Hospital for Sick Children were asked to complete the Children's Health Locus of Control and the Tennessee Self Concept Scale. Their parents were requested to complete the Child Behavior Checklist. Thirty-four males (72%) and 28 females (74%) participated in the study. This study found that adolescents with CF are able to maintain a good self concept, be socially competent, and perceive that they are in control of their health while showing an increase in behavior problems. Females rely heavily on denial and are more behaviorally compliant, whereas boys use less denial but show more behavior problems. Males appear to integrate having a physical disorder into their self concept, whereas females do not. The findings demonstrate a difference in mechanisms of coping with cystic fibrosis between male and female adolescents with CF, which may contribute to the decline in physical status in females and better survival of males.
The effects of cystic fibrosis on 50 Victorian families with one or more affected children were examined in a study carried out between August 1972 and February 1973. The social and emotional problems experienced by family members, particularly the patients and mothers, are reported on.
At all ages, socially embarrassing symptoms and awareness of being different create major problems for the patient. The uncertainty of prognosis and doubts about the prospects of employment are specially relevant for the teenager. It is suggested that the patient be given ample opportunity, from early adolescence onward, to discuss all aspects of his disease, and that more appropriate provision should be made for the total care of adult patients.
Many problems, such as depression, feelings of isolation and the physical demands of constant treatment, which the parents of children with cystic fibrosis experience, are probably similar to those confronting parents of other chronically ill and handicapped children. But special difficulties stem from the genetic basis of cystic fibrosis and from the knowledge of an eventually fatal outcome. The roles of professional members of the medical team are touched on, particularly in relation to genetic counselling and the provision of practical and emotional support to parents. Comments are made on the establishment of lay organisations.
The financial strain associated with obtaining regular medical attention was looked at, and suggestions are made for minimizing the economic problems of certain vulnerable families.
In summary, compliance behavior among adolescents is complex and imcompletely understood. Although the study of compliance is important for understanding the adolescent's stage of psychological development, relationships with authority figures, and the beginning of the youngster's career as a consumer of health care, its ultimate importance lies in the prospect of improving the likelihood that medication will be utilized appropriately. The first step in the process involves systematic monitoring of compliance rather than doing so only when noncompliance is clinically suspected. When compliance is found to be problematic for an adolescent, resorting to "scare" techniques or re-education is rarely effective. A more positive outcome may be achieved by determining the circumstances under which the youngster was successful in complying and attempting to tailor-make the regimen accordingly. Barriers to compliance, such as the presence of side effects, previous negative experience with the medicine, and lack of conviction about the diagnosis of appropriateness of therapy, should always be explored. Other potential intervention strategies for improving compliance have been discussed. In the final analysis, however, as Jonson has noted, all strategies aimed at improving compliance must provide the patient with insight into his own situation and himself, as well as his capability of doing something other than conforming when he judges it best.
Parents and patients in the Rhode Island Cystic Fibrosis Center were interviewed to determine the extent of compliance with various aspects of the therapeutic regimen. Current patterns of compliance were compared with retrospective clinic records of reported compliance up to 8 years prior to the time of study. Actual clinic attendance and reported compliance with medications were high. Patients reported significantly lower compliance with diet and chest physiotherapy. Most patients reported less compliance with chest physiotherapy at the time of study than 3 to 8 years earlier, regardless of the severity of their lung disease. Patients frequently reported self-medication with non-prescribed vitamins, antibiotics, or other drugs. Patients' perceptions of the severity of their disease and the possible adverse consequences of discontinuing therapy probably contribute to both good medication compliance and self-medication.
Patients aged between 12 and 16 years with cystic fibrosis were interviewed, as were their parents, to find out if psychosocial problems existed. All 27 of them appeared to be well adjusted and none had special problems at school; they were not socially isolated, and family relationships seemed to be good. Contributing factors were good communications patterns within the family and perhaps denial of the ultimate outcome of the disease, since few patients wished to know more about cystic fibrosis. Comparison with a group of healthy adolescents and their mothers showed that the patients had a tendency (not significant) towards depression. Among the mothers however, the incidence of depression was slightly greater. It was concluded that psychiatric intervention should only be undertaken cautiously with adolescents, but that special help should be given to the mothers as they appeared to carry the main burden of coping with the illness.
The psychiatric morbidity in group of 36 patients with cystic fibrosis (CF) was compared with that in healthy matched control group. Psychiatric diagnoses, based on the ICD scheme of classification, were much more common in the CF group. Social background factors were related only poorly to psychosocial adaptation. There were moderate correlations between impaired pulmonary function and increased psychopathology; stronger correlations were found with variables related to mental health of the parents and family life. It is suggested that many children with CF require psychological help, a need that may increase as the disease progresses.
Cystic fibrosis (CF) is an autosomal recessive inherited disorder caused by mutations of both alleles of the CF gene. These mutations result in abnormalities in the production or function of the gene product, a protein known as the cystic fibrosis membrane conductance regulator (CFTR). The CF gene is large (250 000 base pairs) and located on chromosome 7. A deletion of three base pairs that results in the absence of phenylalanine at codon 508 is the most common defect (designated F 508) and accounts for about 70% of the mutant genes in North American populations. Other genotypes observed with some frequency (but <3%) are G542X, G551D, W1282X, W1303K, and R553X. The twelve most common CF gene mutations account for approximately 85% of cases in North America. Mutations other than F508 number more than 200 and account for the remaining cases. Most of these 200-odd mutations are "private," observed only in the affected kindred. Because the CF locus has so many DNA sequence changes associated with disease, precise genetic testing cannot replace clinical criteria for diagnosis, and the sweat test remains an important diagnostic test.
The CF gene is expressed in epithelial cells of the pancreas, sweat and salivary glands, intestine, and reproductive and respiratory tracts, particularly in the submucosal glands.
The psychosocial functioning of adolescents and young adults with cystic fibrosis still living in the parental home was investigated. With its proven genetic aetiology cystic fibrosis is an ideal model with which to assess the impact of a chronic and life threatening disorder on family and individual psychological and social functioning.
Twenty nine patients with cystic fibrosis and their families were compared with those of 27 patients with anorexia nervosa and 31 well controls. Assessments were made using self reporting, interview, and observational methods.
Most patients with cystic fibrosis were in robust psychological health and only differed from their healthy peers in that they were much less likely to be in employment. Mothers of patients with cystic fibrosis or anorexia nervosa were more likely than the mothers of the well group to be emotionally distressed, although this was not so for fathers. Young people in both illness groups were more likely to have parents with high levels of expressed emotion. Most families of patients with cystic fibrosis had good problem solving abilities.
In spite of the burden of illness in cystic fibrosis psychological functioning in many respects matches that of well young people.
In this study, 33% of the variance in the 10-year trend of forced expiratory volume in 1 second (FEV1), used as an index of pulmonary health, was explained by differences in family characteristics at the start of the study. Balanced family coping, a family emphasis on personal growth, and compliance with treatment for 91 children with cystic fibrosis were assessed at the start of the study and the FEV1 was observed at every clinic visit over the next 10 years. When both parents' coping emphasized family integration, support for self, and medical consultation, the FEV1 trend was better. Compliance with daily chest physical therapy and with quarterly clinic visits was associated with a better FEV1 trend. Poorer FEV1 trend was associated with active social involvement of family members. Older patients and patients whose parents worked more hours outside the home had lower compliance. These findings support the importance of encouraging families to balance their resources between the child's health needs and family needs.
Patients with chronic disease comply with about 50% of their treatment. The complex and time consuming daily drug regimens needed in the care of adult patients with cystic fibrosis encourage non-compliance with prescribed treatments. Understanding the reasons for, and the extent of, non-compliance is essential for a realistic appraisal of the patient's condition and sensible planning of future treatment programmes.
Patients were invited to complete a questionnaire which asked about their compliance with daily treatment. The data were used to calculate a compliance score, the percentage of prescribed treatment taken, and to examine patient attitudes to each individual prescription. An assessment score derived from consultant, cystic fibrosis research fellow, specialist nurse, and physiotherapist ratings of patient compliance was compared with the compliance score. Both scores were correlated with patient characteristics and disease severity, and the compliance score was also correlated with the patient's knowledge of cystic fibrosis.
More than half the patients claimed to take more than 80% of their treatments. Compliance with individual treatments varied according to their perceived unpleasantness and degree of infringement on daily activities. The most common reason given for omitting treatment was forgetfulness. Professional carers were poor judges of patient compliance. There was no correlation between compliance and patients' sociodemographic characteristics or their knowledge about cystic fibrosis.
Non-compliance is universal and should be recognised as normal behaviour. There are no reliable criteria for predicting any patient's level of compliance. Treatment protocols should be planned around individual patient's requirements, modifying treatment ideals where necessary according to the exigency and pattern of that patient's lifestyle.
To describe the NIMH Diagnostic Interview Schedule for Children (DISC) Version 2.3 and to provide data on its performance characteristics in the Methods for the Epidemiology of Child and Adolescent Mental Disorders (MECA) Study.
Data were collected on the DISC-2.3 at four sites on 1,285 randomly selected children, aged 9 through 17 years, and their parents. Two hundred forty-seven of these child-parent pairs were reassessed on the DISC-2.3 by a clinician interviewer, 1 to 3 weeks later.
Administration time was approximately 1 hour and the interview was acceptable to more than 90% of subjects. The reliability of questions to parents assessing impairment and age of onset was generally good to acceptable for most diagnoses but was less satisfactory for the child interview. Using information from parent and child, the prevalence for any diagnosis ranged from 50.6 if no impairment criteria were required to 5.4 if a Global Assessment Scale score of 50 or less was necessary. The prevalence of anxiety disorders and enuresis was markedly reduced by requiring attributable impairment.
The DISC-2 is a reliable and economical tool for assessing child psychopathology. Reliability of the DISC-P-2.3 is superior to that of the child DISC for most diagnoses but is least good for anxiety disorders. The 2.3 version of the instrument provides a significant improvement over earlier versions.
This investigation examined factors related to adherence to treatment regimens for children with cystic fibrosis (CF) and their mothers. Subjects were 45 children with CF who ranged in age from 6 to 10 years and their mothers. Findings revealed that children's and parents' reports of level of adherence were related to their knowledge of the specific details associated with medically prescribed treatments. In this sample, 12% to 32% of mothers did not have an accurate understanding of physician recommendations for their children's treatments. When controlling for individual differences in the prescribed treatment regimens, parents' and children's knowledge of what had been prescribed accounted for a significant portion of the variance in the children's reported treatment-related behaviors. Results are discussed in terms of implications for future intervention research aimed at enhancing adherence to treatment as well as for future directions for clinical efforts in this area.
The median estimated life expectancy of children with cystic fibrosis (CF) born in 1990 is 40 years which represents a doubling in the last 20 years, and nearly half of all patients are now adults. Since the identification of the gene, more than 1000 gene mutations have been discovered. This gene encodes for the cystic fibrosis transmembrane conductance regulator (CFTR), a protein that is thought to have a role in ion transport, mucus rheology, inflammation and bacterial adherence. Various therapeutic mechanisms are currently being investigated in an attempt to overcome these abnormalities. Recombinant human DNase is beneficial in many patients and the use of anti-inflammatory agents such as steroids, ibuprofen and macrolides have potential. Despite these advances in treatment it is essential that these patients are diagnosed early. Whilst the case for neonatal screening is not absolutely conclusive the evidence is highly suggestive that it would be beneficial. It may be that, in the future, new treatments such as gene therapy will be more effective in those patients who have not yet developed lung disease. Whilst gene therapy and other new treatments such as bilateral living lobar lung donation give our patients optimism for the future it is important to remember that the increase in survival is a result of good physiotherapy, nutrition, aggressive antibiotic use and an increase in our understanding of the disease. It is important that patients continue to be referred early to tertiary CF centres.
Appropriate, regular exercise is of benefit to patients with cystic fibrosis (CF). As with other segments of the population, it has been difficult to devise exercise programs to which most patients will adhere for long periods of time. In healthy children, factors that are related to positive exercise compliance include social support, perceptions of competency and self-esteem, enjoyment of activity, and availability of a variety of activities. In patients with CF, complications associated with the disease, e.g., time required for other treatment and fatigue, make compliance with recommended exercise activities more difficult. Factors likely to increase compliance in this population include explicit and continued encouragement and support from the family and healthcare team, and the introduction of behavior-changing strategies.
This study was undertaken to determine whether families of children with cystic fibrosis were experiencing difficulties in meeting family needs and in maintaining normal family relationships. It was found that families were not deprived of the essentials of living, but they were not able to maintain their usual pattern of family relationships. Time and energy precluded carrying on activities with each other and with the children; and there was breakdown in their ability to communicate adequately between themselves and the children regarding important family issues.
In order to preserve the family as a functioning unit, someone has to be concerned about the entire family. Of necessity, the family has focused on the sick child, leaving the physician, the nurse, the social worker and/or the social agencies to help the family refocus on its total situation, rather than just a part of it.
Traditionally, the mother takes care of the sick child. It is she who takes the child to the doctor's office and is responsible for carrying out his recommendations. In the care of a CF child, she assumes a heavy burden and frequently is fatigued from this responsibility. Because she is so tired and so occupied, she may misunderstand or distort what she is told by the physician, and may not be able to tell her husband or the children what they need to know in order to participate in family activities and in the care of the CF child.
This situation can easily lead to misunderstanding and tension within the family. To avoid this, both parents could be encouraged, at some point, to come together to the physician's office for discussion. Such discussions could lead to more consideration and appreciation being given to each other. It might lessen the tendency for each to blame the other for the child's illness and could avoid the feeling voiced by one mother, "I would like to blow him out of his chair so that he would help me and understand what I go through."
We also need to realize that the CF child is frequently aware of the demands he makes on the family. If these demands are not discussed freely, then everyone is caught in a "web of silence" revolving around his own feelings of frustration. This creates a burden for everyone, including the CF child, and if not discussed it can impair the psychological functioning of all members.
The CF child needs to be encouraged to participate in his own care program and to assume some responsibilities for himself. He should not reach the age of seven being unable to tie his own shoes or dress himself, as has been observed in some CF children. It would seem feasible, therefore, that the CF child should have an awareness of what is wrong with him, and what his abilities and limitations are.
The other siblings should also be given as much explanation as possible because they, too, are part of the family and attention and care is being diverted from them. This explanation could make for more understanding on the sibling's part. While it would still be difficult for him to accept some of the decisions made (such as why the parents could not get home from the hospital in order for him to use the family car for a senior prom), he would know that it was the situation that was causing the decrease in attention and care rather than rejection of him by the parents.
In order to give these families as much assistance as possible, the community's resources should be utilized. Frequently, the parents are unaware of these or need encouragement to avail themselves of services. The homemaker service or visiting nurse service could free the family from constant care; the local youth program could be helpful to the siblings in the family, and Family Service Agencies could be used for counseling on family problems.
In summary, this study points up the need for the total family to have an understanding and awareness of CF and to share such knowledge with one another; that all problems of the family have to be considered and not just those of the CF child; and that help from other professional people should be utilized along with sources of the community.
Dornase alpha improves sputum clearance and pulmonary function in patients with moderate bronchiectasis and cystic fibrosis (CF). Several studies have demonstrated variable adherence to treatment regimens in patients with CF. We aimed to compare self-reported adherence with computer based dispensing records of dornase alpha.
Direct interview of adherence to dornase alpha was compared with pharmacy-dispensing records during a 12-month period. The reasons for dornase alpha usage, and change in lung function in three groups of patients were compared (group 1 (n=42): positive trial, continuing therapy; group 2 (n=13): positive trial, ceased therapy; group 3 (n=22): negative trial). Ninety patients with CF were surveyed. Patients who had ceased dornase alpha provided reasons for drug cessation. Lung function and anthropometric data were collected prior to dornase alpha treatment and at 3 years. Health care professionals rated adherence to therapy.
Of the 42 patients on dornase alpha, the average utilisation was 197 days (54%). From dispensing records, 24% of patients were classified as good adherence, 46% as moderate adherence and 30% as poor adherence. Eighty-two percent claimed to use dornase alpha therapy more than 20 days/month, whereas dispensing records showed that only 24% of collected sufficient drug supplies. Health professionals' assessment of adherence of therapy was often incorrect and an overestimation was more common than underestimation. The change in FEV(1) 3 years after trial was not significantly different between groups. There was a negative correlation between initial response to dornase alpha and baseline FEV(1) (r=-0.44, P=0.001), however, initial response was not related to the change in FEV(1) over 3 years (r=0.20, P=0.16).
There is variability in adherence to dornase alpha in adults with CF and patient self-reporting is likely to over-estimate drug collection from the pharmacy. Further study of the long-term effects of dornase alpha is required. To date, proven interventions to enhance adherence are limited and require investigation in CF.
The life expectancy of individuals with CF has increased to 33 years. Thus, issues such as quality of life and psychological well-being, previously thought to be of lesser importance than physical well-being, are now recognised as significant factors. This study examined the interrelationships between quality of life, family functioning, individual psychopathology and optimism of adolescents with CF.
Adolescents attending the CF clinic completed a number of questionnaires. Quality of Life was measured using the Cystic Fibrosis Questionnaire, family functioning by the Family Environment Scale (3rd edition), general psychopathology with the Symptom Checklist-90-Revised and optimism for the future by the Hunter Opinions and Personal Expectations Scale. Disease severity was assessed using the Shwachman score and spirometry at the time of questionnaire completion.
The level of psychopathology (12.5% of those 13 years and over) in the group was lower than that reported for young people in Australia (15-20%). The results indicated that young people with a delayed diagnosis and those who are alienated from their families may be in need of additional psychosocial support. The group was hopeful and positive about their future and these attributes were independent of clinical measures of disease severity. In general, these young people scored relatively highly on the quality of life scale. For example the mean standardised score for physical functioning was 70 points, for respiratory symptoms was 63 points and for emotional state was 78 points. Increased levels of psychopathology and lack of hope for the future were however associated with lower ratings on a number of quality of life measures. Family cohesiveness, expressiveness and organization were associated with better psychological functioning in the young people.
Adolescents with CF appear to be a psychologically well functioning and well-adjusted group. These findings support the importance of a more sophisticated model of well-being for adolescents with CF, which explores the young person's views on their quality of life and wider support frameworks rather than relying solely on measures of physical health to gauge well-being.
The purpose of this study was to monitor medication adherence in cystic fibrosis (CF) patients and its correlation with disease severity and patient age.
Children less than 12 years of age (group 1) and adolescents 12 years of age and older (group 2) were recruited from the University of Michigan CF Center. The study duration was 3 months. A total of 22 patients per group were enrolled. Adherence to ADEKs, an oral multivitamin, and dornase alfa, a nebulized mucolytic medication, was monitored. Adherence to ADEKs was monitored by using the Medication Event Monitoring System (MEMS) SmartCaps (APREX, AARDx, Inc., Union City, California). Dornase alfa adherence rate was monitored by counting empty medication vials.
Thirty-three patients completed the study, 15 patients in group 1 and 18 patients in group 2. The overall mean adherence rates for ADEKs and dornase alfa were (+/- SD) 63.6% +/- 24.0% and 66.5% +/- 31.2%, respectively. The median ADEKs and dornase alfa adherence rate for group 1 was 84.6% and 79.1%, respectively (p = .08); and for group 2 was 56.7% vs. 78.4%, respectively (p = .07). There was a trend toward significance, suggesting that the adherence rate for ADEKs was higher than for dornase alfa (p = .08) in group 1. Group 2 showed a trend toward adherence to dornase alfa than to ADEKs (p = .07). There was a trend for ADEKs adherence between groups 1 and 2 (p = .09), but not for dornase alfa (p = .93).
Parental supervision and disease severity are likely to play a major role in adherence to medical management. Partnership with patients and families about the treatment plan might be important for improving adherence rate. The MEMS SmartCaps is an electronic monitoring technology that should be used to measure drug adherence objectively both in further larger clinical trials and in the outpatient setting.