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Gender differences in treatment adherence among youth with cystic fibrosis: Development of a new questionnaire
Abstract
Some prior studies have reported that girls with cystic fibrosis (CF) experience higher morbidity and mortality compared to boys. In this study, the authors compared boys' and girls' perceptions of disease-related strains and resources associated with living with CF, and the relationship of these factors to CF treatment feelings and behaviors.
All 10-21 year olds with CF at the Minnesota Cystic Fibrosis Center were invited by mail to complete a new self-report survey (Living with CF Questionnaire--LCFQ). Of these 177 youth, 58% (49 boys and 54 girls) returned surveys.
Exploratory and confirmatory factor analyses revealed nine factors in the LCFQ. Partial support was found for hypothesized gender differences in these factors. Compared to boys, girls reported significantly more illness-related strains and worries, including emotional strains, greater treatment discouragement, lower self-esteem, and lower adherence to some aspects of the CF treatment regimen (coughing, eating high-fat foods, taking meds/pills).
Living with CF appears to have a greater emotional impact on adolescent girls compared to boys. These gender differences may contribute to the poorer pulmonary function observed among girls with cystic fibrosis during the adolescent years.
... Nonetheless, only a very limited number of studies have reported on cough suppression (CS) in pwCF. In two detailed studies with adolescent pwCF, embarrassment about coughing was used as an indicator of CS [6,7]. Sex differences were found in a way that girls were more embarrassed about coughing than boys, with the consequence that girls suppressed coughing in public to avoid negative attention. ...
... With this cross-sectional study, we present for the first time that CS exists in adult pwCF, is more prevalent in women, and relates to reduced HRQoL. In contrast to previous studies in adolescents, which found that CS negatively correlated with ppFEV 1 and BMI [6,7], these findings could not be confirmed in adult pwCF. Furthermore, no relationship between CS and health-related perspectives of the future was observed. ...
... In the present study, 98% of female subjects suppressed coughing, and 40% did so 'frequently' or 'always' . This finding is consistent with previous studies in adolescent pwCF, indicating that females of either age are more self-conscious about the impact of their coughing on others and show a tendency to suppress coughing to avoid negative public attention [6,7]. From a socialcognitive perspective [17], CS undergoes stabilization as development progresses by reinforcement that may include avoiding negative public attention, thus avoiding cough-related negative emotions. ...
Background
Cough suppression assessed by embarrassment about coughing has been shown in adolescents with cystic fibrosis (CF) and negatively affects health-related quality of life (HRQoL) and clinical indicators of disease severity in adolescent females. However, whether cough suppression exists in adults has been studied as little as its effects on clinical and psychological outcomes beyond adolescence.
Methods
Seventy-one subjects completed the self-reported 'Cystic Fibrosis Questionnaire-Revised (CFQ-R + 14)' and a self-report questionnaire about cough suppression, health-related perspectives, and therapy adherence. The status of CF disease was quantified in terms of the percentage of predicted forced expiratory volume in one second (ppFEV 1 ), body mass index (BMI), Pseudomonas aeruginosa, pancreatic status, and CF-related diabetes (CFRD). Additional demographic data for sex, age, graduation, employment, and marital status were assessed.
Results
CS exists in adult CF and is associated with impaired HRQoL but not the overall CF disease status regarding BMI, ppFEV 1 , or health-related perspectives. Despite a higher prevalence of cough suppression in women, no effect of sex regarding either outcome measure was observed.
Conclusion
The results of this study suggest that mental health indicators have an impact on cough suppression.
... Nonetheless, only a very limited number of studies have reported on cough suppression (CS) in pwCF. In two detailed studies with adolescent pwCF, embarrassment about coughing was used as an indicator of CS [3,4]. Sex differences were found in a way that girls were more embarrassed about coughing than boys, with the consequence that girls suppressed coughing in public to avoid negative attention. ...
... With this cross-sectional study, we present for the rst time that CS exists in adult pwCF, is more prevalent in women, and relates to reduced HRQoL. In contrast to previous studies in adolescents, which found that CS negatively correlated with ppFEV 1 and BMI [3,4], these ndings could not be con rmed in adult pwCF. ...
... In the present study, 98% of female subjects suppressed coughing, 40% did so 'frequently' or 'always'. This nding is consistent with previous studies in adolescent pwCF, indicating that females of either age are more self-conscious about the impact of their coughing on others and show a tendency to suppress coughing to avoid negative public attention [3,4]. From a social-cognitive perspective [14], CS undergoes stabilization as development progresses by reinforcement that may include avoiding negative public attention, thus avoiding cough-related negative emotions. ...
Background: Cough suppression assessed by embarrassment about coughing has been shown in adolescents with cystic fibrosis (CF) and negatively affects health-related quality of life (HRQoL) and clinical indicators of disease severity in adolescent females. However, whether cough suppression exists in adults has been studied as little as its effects on clinical and psychological outcomes beyond adolescence.
Methods: 71 subjects completed the self-reported 'Cystic Fibrosis Questionnaire-Revised (CFQ-R +14)' and a self-reported questionnaire about cough suppression, health-related perspectives, and therapy adherence. Status of CF disease was quantified in terms of percentage of predicted forced expiratory volume in one second (ppFEV1), body mass index (BMI), Pseudomonas aeruginosa, and pancreatic status, as well as CF-related diabetes (CFRD). Additional demographic data for sex, age, graduation, employment, and marital status were assessed.
Results: CS exists in adult CF and is associated with impaired HRQoL, but not the overall CF disease status regarding BMI, ppFEV1, or health-related perspectives. Despite a higher prevalence of cough suppression in women, no effect of sex regarding either outcome measure was observed.
Conclusion: The results of this study may suggest that mental health indicators have an impact on cough suppression.
... When a cohort of individuals with CF was followed over a 10-year period, an increased rate of respiratory-related hospitalizations was found to occur in adolescent girls compared to boys (Stephenson et al., 2011). For the females with CF, the decline in lung function occurred earlier (12-14 years of age versus 19-20 in males) and at a steadier rate and was associated with more frequent hospitalizations (Groeneveld et al., 2012;Patterson, Wall, Berge, & Milla, 2008;Stephenson et al., 2011). The purpose of this literature review was to examine the state of the science related to gender differences in HRQoL in those with CF. ...
... This included support from not only friends and family, but also mental health providers (Berge et al., 2007). Concurrently, females have noted more CF-related strains and sought more support than males (Patterson et al., 2008). Conversely, males more easily incorporated CF into their identity as a part of them for which they took full responsibility (Berge et al., 2007). ...
... Females noted more dieting behavior and preoccupation with food when compared to males (Abbott et al., 2007). Females were also more likely to skip the needed high fat food than males (Patterson et al., 2008). Percent of the recommended dietary reference intake was an average of 30 points lower for females compared to males (Simon et al., 2011). ...
... When a cohort of individuals with CF was followed over a 10-year period, an increased rate of respiratory-related hospitalizations was found to occur in adolescent girls compared to boys (Stephenson et al., 2011). For the females with CF, the decline in lung function occurred earlier (12-14 years of age versus 19-20 in males) and at a steadier rate and was associated with more frequent hospitalizations (Groeneveld et al., 2012;Patterson, Wall, Berge, & Milla, 2008;Stephenson et al., 2011). The purpose of this literature review was to examine the state of the science related to gender differences in HRQoL in those with CF. ...
... This included support from not only friends and family, but also mental health providers (Berge et al., 2007). Concurrently, females have noted more CF-related strains and sought more support than males (Patterson et al., 2008). Conversely, males more easily incorporated CF into their identity as a part of them for which they took full responsibility (Berge et al., 2007). ...
... Females noted more dieting behavior and preoccupation with food when compared to males (Abbott et al., 2007). Females were also more likely to skip the needed high fat food than males (Patterson et al., 2008). Percent of the recommended dietary reference intake was an average of 30 points lower for females compared to males (Simon et al., 2011). ...
Background: Depression is a significant concern impacting the health of our maternal populations and directly contributing to maternal mortality. While postpartum depression (PPD) is documented to have significant impacts on maternal child health and maternal mortality, no known study to date has examined the manifestation and trajectory of PPD
in women of childbearing age.
Objective: The purpose of this study was to conduct an integrative literature review to examine the trajectory of PPD. In order to best prevent, treat, and manage PPD, it is important to understand the manifestation of this illness.
Methods: An integrative literature review was conducted utilizing CINAHL, PubMed, and ProQuest Nursing and Allied Health. After the inclusion and exclusion criteria were applied, 14 articles were included in this literature review.
Results: Of the 14 original research articles included in this integrative review, 10 (71%) determined that women who developed PPD also experienced depression either ante- partum or before pregnancy. Through this literature review, the trajectory of PPD was described as being the manifestation of earlier depression, rather than a new onset of a depression episode.
Conclusion: There are several opportunities for addressing the trajectory of PPD. It is essential for the allied health care force to better recognize PPD, its prevention, and its treatment as early as possible in the perinatal period. With the knowledge of this trajectory of PPD in mind, further studies need to explore these health care implications.
Keywords: postpartum depression, perinatal depression, antenatal depression, trajectory, maternal mortality
... Sex differences in adherence have also been demonstrated to be important within the CF population (Abbott, Havermans, & Hart, 2009), with female adolescents with CF displaying more non-adherent behaviours and attitudes than male adolescents with CF (Patterson, Wall, Berge, & Milla, 2008). These differences were most evident for behaviours that were visible to peers (embarrassment about coughing, skipping medications), which could contribute to self-consciousness (Patterson et al., 2008). ...
... Sex differences in adherence have also been demonstrated to be important within the CF population (Abbott, Havermans, & Hart, 2009), with female adolescents with CF displaying more non-adherent behaviours and attitudes than male adolescents with CF (Patterson, Wall, Berge, & Milla, 2008). These differences were most evident for behaviours that were visible to peers (embarrassment about coughing, skipping medications), which could contribute to self-consciousness (Patterson et al., 2008). ...
... The role of identity could explain why the weekend/weekday difference in adherence was most prevalent in adult female CF patients. Patterson et al. (2008) demonstrated that female adolescents with CF display more non-adherent behaviours in comparison to male adolescents with CF. In particular, females were more likely to experience embarrassment when coughing and taking CF medications. ...
Background
Treatment adherence is a major concern in cystic fibrosis (CF), with accumulating evidence that health outcomes are worse in patients with lower levels of adherence. This study investigates how adherence differs for adults with CF during a weekday and a weekend day by examining the roles of sex, anxiety, depression, and lung function as predictors of adherence.
Participants and procedure
Fifty-two adult participants with CF were recruited. Demographics and spirometry results were recorded. Participants completed the Hospital Anxiety and Depression Scale and two daily phone diaries in order to record their adherence to pancreatic enzymes, vitamins, physiotherapy and exercise. Based on previous findings, it was hypothesised that reported adherence would be higher during the weekend in comparison to weekdays, due to lower time pressure during the weekend.
Results
Paired sample t-tests indicated that overall participants had higher reported adherence during the weekend in comparison to weekdays, with sex, anxiety, depression and lung function being predictors of adherence.
Conclusions
Clinical implications and future directions are discussed, with an emphasis on the need for further qualitative research. We are now conducting another research project utilising qualitative interviews with participants to further investigate adherence within the CF population. Our aim is to identify the main adherence barriers and to develop interventions to improve treatment adherence in the CF population.
... Previous studies have reported that compared with boys with CF, adolescent girls with CF have a lower adherence to some aspects of CF management, including coughing as an airway clearance technique, consumption of high-calorie, high-fat foods, and taking medications. 31,32 Differences in caloric intake may be related to perceived body image and self-esteem, with female patients with CF preferring a lower BMI than male patients. 33,34 Girls and women also reported more CF-related emotional and physical strains than boys and men. ...
... 33,34 Girls and women also reported more CF-related emotional and physical strains than boys and men. 32,33 This study has several limitations. It was an observational retrospective study of open-label treatment, without randomization or stratification, and was thus subject to various kinds of bias and confounding factors. ...
We investigated the relationship between self-reported adherence to pancreatic enzyme replacement therapy (PERT), nutritional status, and all-cause hospitalization in cystic fibrosis (CF) patients with a record of PERT use. Association of self-reported annual PERT use rate (adherence) with annual hospital admission rate (HAR) and annual total hospital nights (THNs) were analyzed for 5301 children (2000–2012) and 13,989 adults (2000–2013) from the CF Foundation Patient Registry. Multivariate linear regression was used to determine the association of HAR and THN with mean annual PERT use rate, cumulative PERT use rate, mean body mass index (BMI) (adult) or BMI percentile (pediatric), age, and sex. The median annual PERT use rate was 87% in children and 80% in adults. Statistically, higher annual PERT use, longer cumulative PERT, and higher BMI percentile (children) or BMI (adults) were significantly ( p < 0.0001) associated with lower annual HAR and fewer annual THN in children and adults. Female sex was associated with higher annual HAR and more annual THN in children and adults ( p < 0.05). Results indicate self-reported adherence to PERT, increased BMI, and male sex were associated with fewer hospital admissions and annual hospital nights in CF patients.
... 45 Adherence to therapies is lower in girls and women with CF, who are more likely to skip medications or chest physiotherapy, decrease caloric intake to obtain thinner body stature, and suppress the cough. 46 Female patients may be thinner due to body image issues and exacerbated by praise for an asthenic appearance. In one study, female patients who were underweight were more likely than male patients to consider their weight to be normal. ...
... 48,49 Female patients also have a more emotional impact from their CF than their male counterparts, with higher rates of emotional strain, worry about the future, lower self-esteem, and greater discouragement. 46 3 | BIOLOGY ...
Understanding variability in cystic fibrosis (CF) health outcomes requires an understanding of factors that go far beyond Cystic Fibrosis Transmembrane Receptor (CFTR) function caused by different gene mutations. Social and environmental factors that influence health have a significant influence on the trajectory of health in CF and in other chronic diseases. In this article, we review demographic factors associated with poorer health outcomes in CF, known and postulated biological mechanisms of these outcomes, and interventions that healthcare teams can implement that may reduce outcome disparities.
... However, self-report questionnaires are easy to administer, are inexpensive, and can measure each component of the treatment regimen from the patient's perspective [42]. Currently, no validated questionnaires exist to measure adherence in CF [41,43]. There is also less clarity in the measurement criteria such as the number of doses missed per day or per week that may hold the same degree of clinical significance across the different treatments. ...
... There is also less clarity in the measurement criteria such as the number of doses missed per day or per week that may hold the same degree of clinical significance across the different treatments. Patterson et al. developed a new 'Living with CF Questionnaire' to capture multiple aspects of adherence; however, not all domains had sufficient reliability, and the study had a low response rate [43]. Thus, there is a need to develop appropriate tools to adequately capture patientreported outcomes for CF. ...
INTRODUCTION:
Cystic fibrosis (CF) is a life-shortening condition with no cure. Available therapies relieving the symptoms of CF are complex and time-consuming. A comprehensive review assessing adherence to different CF therapies, association of adherence with outcomes, and factors influencing adherence could inform optimal patient management strategies. Areas covered: A targeted literature review of studies published from 2010-2016 assessed adherence to CF therapies. Nineteen studies qualified for inclusion. Adherence to CF therapies was sub-optimal, and varied by treatment, mode of treatment administration, age, season, time and method of adherence measurement. Adherence to ivacaftor and inhaled antibiotics were reported higher than dornase alfa or hypertonic saline, oral pancreatic enzyme and vitamin supplements, and airway clearance therapy. Several patient, healthcare provider and treatment related factors influenced adherence. Sub-optimal adherence was shown to impact clinical and economic burden of the disease. Expert commentary: Higher adherence to CF therapies can lower disease burden, and improve patient outcomes. Healthcare providers and policy makers should devise patient-centered and caregiver-enabled interventions to improve adherence. Research on long-term adherence and outcomes associated with promising oral treatments such as CFTR modulators is needed. Identifying ways to overcome key barriers to adherence can positively affect outcomes associated with CF.
... d only among girls: negative social relations affected FEV 1 results, while positive relations did not cause the improvement of the breathing parameters. Among boys, the worsening of FEV 1 affected only the results of one of LCFQ subscales, namely Physical strains. The findings mentioned above confirmed the outcomes of the teams' previous research (Patterson, Wall et. al. 2007). It revealed that girls carried heavier burden connected with the disease, they had problems with compliance with the treatment, including physiotherapy, diet, and taking medication. Cystic fibrosis, due to its chronic and terminal character, may influence the formation of specific bonds in the families of ill adolescents. The symptoms ...
... The negative influence of cystic fibrosis was observed only in the sphere of emotions: the patients participating in the study experienced sadness, apathy, depression and anger that manifested in the negativist reactions. Older students (11)(12)(13)(14)(15) tended to suppress negative emotions. It was also revealed that the study subjects thought highly of themselves, which, according to the author, served the adaptive function -it helped the patients maintain a positive image of them in the face of serious and incurable disease (Pilecka 1990). ...
The article focuses on the adolescents suffering from cystic fibrosis in the context of biomedical and psychosocial problems. Medical difficulties stem mainly from the rarity of the illness, its multiple organ symptomatology, and complicated treatment, which place a heavy burden on patients and their families. The selected psychological and social aspects concern the emotional sphere of the teenagers, their self-acceptance and social relations, psychosexuality, and the difficulties connected with the transition to adulthood in the context of premature death. In Poland, the studies on non-medical aspects of functioning of the patients with cystic fibrosis are not numerous; hence the article refers to foreign theoretical and empirical studies. The conclusion emphasizes the issue of the insufficient knowledge about cystic fibrosis in Polish educational institutions. It also contains suggestions concerning the directions of future studies that focus on social and personal resources of the ill adolescents that are the source of hope and help them find the meaning of life.
Key words: cystic fibrosis, adolescents, adherence, psychology
... Gender disparity among adolescent patients has significant influence on patient outcomes. Studies have shown that adolescent female patients with a chronic disease are less adherent than their male counterparts [Patterson et al. 2008]. This has been demonstrated in the CF population specifically. ...
... The etiology of this sex difference was not clear. Finally, Patterson and colleagues sent a questionnaire (LCFQ) to patients ages 10-21, both males and females with CF, comparing perceptions of disease-related strains, living with CF, and factors related to treatment feelings and behavior [Patterson et al. 2008]. They found that CF appears to have a greater emotional impact on adolescent females when compared with adolescent males, which may contribute to poorer pulmonary function among females with CF during adolescent years. ...
Bilateral lung transplantation remains a treatment for end-stage cystic fibrosis (CF) lung disease when there is no further medical or surgical treatment available. The aim of lung transplantation is to improve patient life expectancy. The consensus view that lung transplantation extends life in children has been challenged. However, challengers have faced alternative explanations of their finding and it is largely accepted that lung transplantation in the adolescent patient population is an important treatment modality. The goal of this article is to address these concerns and assist with developing creative approaches to this known challenging subset of patients. The main focus of this article will be to discuss the looming question: what existing evidence or best practice insights could improve bilateral lung transplantation outcomes for adolescent patients with end-stage CF?
... While medical parameters, such as percent predicted forced expiratory volume in one second (ppFEV 1 ) are only moderately associated with HRQoL [3], mental health variables such as depression and anxiety play a more critical role in predicting HRQoL [4]. Recent work also demonstrates the importance of previously understudied behaviors in pwCF, such as cough suppression (CS) -a behavior characterized by suppressing coughs in public to avoid negative attention despite the urge to cough [5]. ...
Background
The present study evaluates personality traits in adult patients with cystic fibrosis (CF) and correlates these results with health-related quality of life (HRQoL) and other clinical parameters indicative of disease severity.
Methods
Seventy adults completed the Cystic Fibrosis Questionnaire-Revised (CFQ-R 14+), a CF-specific measure of HRQoL, and a self-administered questionnaire about personality traits and disorders. Mean subscale scores and the prevalence of extreme personality traits on the `Persönlichkeits-Stil- und Störungs-Inventar (PSSI)´ were compared to the norming sample. Moreover, a cluster analysis was conducted to identify personality styles among people with cystic fibrosis (pwCF). The relationship between mean PSSI subscale scores and personality clusters with HRQoL and clinical outcomes, e.g., percent predicted forced expiratory volume in one second (ppFEV1), and body mass index (BMI), was studied by regression analysis considering important confounders.
Results
On several of the subscales of the personality questionnaire, people with cystic fibrosis (pwCF) showed either significantly higher or lower scores than the norm sample. In further analyses, two personality clusters could be identified. PwCF from the cluster with predominantly low scores on the subscales ‘negativistic’, ‘schizoid’, ‘borderline’, ‘depressed’, and ‘paranoid’ showed better HRQoL than pwCF from the other cluster with mainly high normal or elevated scores. The studied health outcomes proved to be independent of the respective personality clusters.
Conclusions
In pwCF, HRQoL is mainly determined by psychological factors, including personality. Since more recent personality theories assume that personality is modifiable, our findings imply that patients with accentuated personality traits may benefit from psychosocial support.
... Hasil penelitian ini juga membuktikan bahwa jenis kelamin mempengaruhi kepatuhan konsumsi mikronutrien (p<0,05). Anak laki-laki dan perempuan mempunyai sifat dasar yang berbeda yaitu anak laki-laki cenderung lebih yakin dan tidak mudah dipengaruhi oleh teman sebayanya (11,12). Penelitian lain juga menjelaskan bahwa anak balita laki-laki 1,38 kali lebih patuh dibandingkan balita perempuan terhadap pemberian mikronutrien dalam bentuk biskuit dan suplemen (10). ...
Compliance of micronutrients supplement consumption was not affected by supplements form
Background: Taburia is micronutrient sprinkle produced by Ministry of Health Indonesia to overcome malnutrition problem in Indonesia. Compliance of Taburia consumption is an important indicator for the success of the supplementation program. Taburia’s compliance in some regions is low (0.05). Several factors affected the compliance level such as mother’s employment status, gender of the children, and age of the children.
Conclusions: The compliance level of Taburia sprinkle and fortified gummy candies was similar. Hence fortified gummy candies could be used as an alternative of micronutrient supplement to overcome nutrition problem in Indonesia.
... The diagnostic lag in in children and adults may be related to decreased recognition of respiratory symptoms and unconscious gender bias by healthcare providers towards females [171][172][173]. Additionally, gender-related factors that disadvantage young females with CF include illness-related worries and poor coping, decreased quality of life, greater depression, discouragement of treatment, and acceptance of being underweight [177][178][179][180][181][182][183][184]. ...
Chronic lung diseases are the third leading cause of death worldwide and are increasing in prevalence over time. Although much of our traditional understanding of health and disease is derived from study of the male of the species – be it animal or human – there is increasing evidence that sex and gender contribute to differences in disease risk, prevalence, presentation, severity, treatment approach, response and outcomes. Chronic obstructive pulmonary disease, asthma and bronchiectasis represent the most prevalent and studied chronic lung diseases and have key sex- and gender-based differences which are critical to consider and incorporate into clinical and research approaches. Mechanistic differences present opportunities for therapeutic development whereas behavioural and clinical differences on the part of patients and providers present opportunities for greater education and understanding at multiple levels. In this review, we seek to summarise the sex- and gender-based differences in key chronic lung diseases and outline the clinical and research implications for stakeholders.
... Poor nutritional status and reduced BMI are associated with lower lung function and increased mortality in CF [9,14,17] , and therefore undoubtedly contribute to the sex disparity in CF survival. Furthermore, CF women have been found to have poorer medication and nutritional adherence, which may also contribute to reduced lung function [18,19] and worse health outcomes [20] . ...
Sex differences in morbidity and mortality have been reported in the cystic fibrosis (CF) population worldwide. However, it is unclear why CF women have worse clinical outcomes than men. In this review, we focus on the influence of female sex hormones on CF pulmonary outcomes and summarise data from in vitro and in vivo experiments on how estrogen and progesterone might modify mucociliary clearance, immunity and infection in the CF airways. The potential for novel sex hormone related therapeutic interventions is also discussed.
... It is worth noting that there were only a small number of males within this study and the aim of qualitative research is not to make generalisations. Living with CF has been evidenced in previous research to have a greater emotional impact on adolescent girls in comparison to boys (Patterson et al., 2008), therefore, there could be a greater emotional impact on adult women. Another possible reason is that the male participants may have downplayed their symptoms and reactions to the diagnosis to maintain their masculine identity, particularly whilst being interviewed by a female researcher. ...
There is a paucity of research investigating what it is like to be diagnosed and to live with cystic fibrosis (CF) in adulthood. Understanding the experiences of these adults and the impact of the condition can provide information to help healthcare professionals deliver appropriate support for their patients. This research aimed to address this gap in the literature. In-depth semi-structured interviews were carried out with sixteen participants (ten females and six males) diagnosed with CF in adulthood. Using thematic analysis, four themes were identified “No, you can’t possibly have CF”, Emotions around diagnosis, “It did kind of take over my life” and “I no longer wish to argue with it”. These themes described participants’ frustrations with their contact with healthcare providers before diagnosis, their ambivalence around their diagnosis, the various impact and challenges faced with day-to-day living, work-life, finances, relationships, fertility, life plans, the future, treatment burden and their acceptance and adjustment to their CF. A key finding was the mismatch between patient need and healthcare provision. The main recommendations made included: 1) raising a greater awareness amongst non-CF specialist healthcare professionals and the general public of the possibility of receiving a CF diagnosis in adulthood, 2) ensuring CF healthcare professionals delivering the news of an adult CF diagnosis have the appropriate training and support and 3) ensuring CF healthcare professionals adequately assess individual’s information needs and provide appropriate and relevant information. The implications for health psychology practice include support for patients to help make sense of the diagnosis, develop adaptive coping strategies and adjust to living with a chronic condition. The role of the psychologist would involve working with specialist CF healthcare professionals through providing educative training, reflective practice and supervision.
Full text available: https://uwe-repository.worktribe.com/output/3321017
... A recent CF study focused on evaluating the lung microbiome as a marker of response to aztreonam did report gender-associated differences in treatment response and lung microbiome diversity [64]. Importantly, treatment adherence differs between genders with females demonstrating poorer overall adherence [65][66][67][68][69][70][71]. This likely contributes to gender-related differences in disease severity in bronchiectasis, for instance, significantly less women use inhalers appropriately and have poorer attendance at follow-up appointments for treatment and pulmonary rehabilitation [1,72,73]. ...
Key points:
CF and non-CF bronchiectasis are complex, multifactorial chronic pulmonary diseases with gender-specific differences in their prevalence, clinical presentation and disease severity.Microbiology and host physiology (immune and inflammatory responses) are essential aspects of bronchiectasis that are influenced by gender.Sex steroid hormones vary in type, fluctuating pattern and concentration throughout life and between the genders with a potential central role in bronchiectasis-related gender differences.Gender-focused clinical and/or therapeutic intervention has the potential to narrow the observed gender gap occurring in bronchiectasis-related lung disease.
Educational aims:
To summarise the existing knowledge base of gender-related differences in CF and non-CF bronchiectasis.To highlight key areas of importance in the diagnosis, monitoring and treatment of bronchiectasis that is amenable to clinical and/or pharmacological intervention to narrow the existing "gender gap".
... The differences were only significant for the measurement of NIBP, SpO 2 , and ECG (Multimedia Appendix 1). These data on gender differences are in good agreement with the literature, which reports reduced adherence by women to telemonitoring of BP [30], antiretroviral therapies [31], management of cystic fibrosis [32], and medications [33]. ...
Background:
In a home telemonitoring trial, patient adherence with scheduled vital signs measurements is an important aspect that has not been thoroughly studied and for which data in the literature are limited. Levels of adherence have been reported as varying from approximately 40% to 90%, and in most cases, the adherence rate usually dropped off steadily over time. This drop is more evident in the first few weeks or months after the start. Higher adherence rates have been reported for simple types of monitoring and for shorter periods of intervention. If patients do not follow the intended procedure, poorer results than expected may be achieved. Hence, analyzing factors that can influence patient adherence is of great importance.
Objective:
The goal of the research was to present findings on patient adherence with scheduled vital signs measurements in the recently completed Commonwealth Scientific and Industrial Research Organisation (CSIRO) national trial of home telemonitoring of patients (mean age 70.5 years, SD 9.3 years) with chronic conditions (chronic obstructive pulmonary disease, coronary artery disease, hypertensive diseases, congestive heart failure, diabetes, or asthma) carried out at 5 locations along the east coast of Australia. We investigated the ability of chronically ill patients to carry out a daily schedule of vital signs measurements as part of a chronic disease management care plan over periods exceeding 6 months (302 days, SD 135 days) and explored different levels of adherence for different measurements as a function of age, gender, and supervisory models.
Methods:
In this study, 113 patients forming the test arm of a Before and After Control Intervention (BACI) home telemonitoring trial were analyzed. Patients were required to monitor on a daily basis a range of vital signs determined by their chronic condition and comorbidities. Vital signs included noninvasive blood pressure, pulse oximetry, spirometry, electrocardiogram (ECG), blood glucose level, body temperature, and body weight. Adherence was calculated as the number of days during which at least 1 measurement was taken over all days where measurements were scheduled. Different levels of adherence for different measurements, as a function of age, gender, and supervisory models, were analyzed using linear regression and analysis of covariance for a period of 1 year after the intervention.
Results:
Patients were monitored on average for 302 (SD 135) days, although some continued beyond 12 months. The overall adherence rate for all measurements was 64.1% (range 59.4% to 68.8%). The adherence rates of patients monitored in hospital settings relative to those monitored in community settings were significantly higher for spirometry (69.3%, range 60.4% to 78.2%, versus 41.0%, range 33.1% to 49.0%, P<.001), body weight (64.5%, range 55.7% to 73.2%, versus 40.5%, range 32.3% to 48.7%, P<.001), and body temperature (66.8%, range 59.7% to 73.9%, versus 55.2%, range 48.4% to 61.9%, P=.03). Adherence with blood glucose measurements (58.1%, range 46.7% to 69.5%, versus 50.2%, range 42.8% to 57.6%, P=.24) was not significantly different overall. Adherence rates for blood pressure (68.5%, range 62.7% to 74.2%, versus 59.7%, range 52.1% to 67.3%, P=.04), ECG (65.6%, range 59.7% to 71.5%, versus 56.5%, range 48.7% to 64.4%, P=.047), and pulse oximetry (67.0%, range 61.4% to 72.7%, versus 56.4%, range 48.6% to 64.1%, P=.02) were significantly higher in males relative to female subjects. No statistical differences were observed between rates of adherence for the younger patient group (70 years and younger) and older patient group (older than 70 years).
Conclusions:
Patients with chronic conditions enrolled in the home telemonitoring trial were able to record their vital signs at home at least once every 2 days over prolonged periods of time. Male patients maintained a higher adherence than female patients over time, and patients supervised by hospital-based care coordinators reported higher levels of adherence with their measurement schedule relative to patients supervised in community settings. This was most noticeable for spirometry.
Trial registration:
Australian New Zealand Clinical Trials Registry ACTRN12613000635763; https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=364030&isReview=true (Archived by WebCite at http://www.webcitation.org/6xPOU3DpR).
... Research to date indicates that lower adherence to prescribed medication regimens may be in part due to difficulties with time management, forgetting, increased complexity of regimens, decreased parental supervision, perceived doubts about the necessity of treatments, stigma and reluctance to disclose CF status, and depression in both patients and their parents. 1,5,9,11,24,[29][30][31][32][33][34][35][36][37][38][39] Adolescent Health, Medicine and Therapeutics 2016:7 submit your manuscript | www.dovepress.com ...
While development of new treatments for cystic fibrosis (CF) has led to a significant improvement in survival age, routine daily treatment for CF is complex, burdensome, and time intensive. Adolescence is a period of decline in pulmonary function in CF, and is also a time when adherence to prescribed treatment plans for CF tends to decrease. Challenges to adherence in adolescents with CF include decreased parental involvement, time management and significant treatment burden, and adolescent perceptions of the necessity and value of the treatments prescribed. Studies of interventions to improve adherence are limited and focus on education, without significant evidence of success. Smaller studies on behavioral techniques do not focus on adolescents. Other challenges for improving adherence in adolescents with CF include infection control practices limiting in-person interactions. This review focuses on the existing evidence base on adherence intervention in adolescents with CF. Future directions for efforts to optimize treatment adherence in adolescents with CF include reducing treatment burden, developing patient-driven technology to improve tracking, communication, and online support, and rethinking the CF health services model to include assessment of individualized adherence barriers.
... Although, to our knowledge, there is no literature documenting sex and/or gender differences in adherence to online training programs, a number of studies have shown sex and/or gender differences in adherence to rehabilitative or therapeutic programs, health guidelines, and medication use. [75][76][77][78][79] Therefore, future studies should also consider sex and/or gender and its influence on adherence. ...
Purpose:
Cognitive environmental enrichment (C-EE) offers promise for offsetting neural decline that is observed in chronic moderate-severe traumatic brain injury (TBI). Brain games are a delivery modality for C-EE that can be self-administered over the Internet without therapist oversight. To date, only one study has examined the feasibility of self-administered brain games in TBI, and the study focused predominantly on mild TBI. Therefore, the primary purpose of the current study was to examine the feasibility of self-administered brain games in moderate-severe TBI. A secondary and related purpose was to examine the feasibility of remote monitoring of any C-EE-induced adverse symptoms with a self-administered evaluation tool.
Method:
Ten patients with moderate-severe TBI were asked to complete 12 weeks (60 min/day, five days/week) of online brain games with bi-weekly self-evaluation, intended to measure any adverse consequences of cognitive training (e.g., fatigue, eye strain).
Results:
There was modest weekly adherence (42.6% ± 4.4%, averaged across patients and weeks) and 70% patient retention; of the seven retained patients, six completed the self-evaluation questionnaire at least once/week for each week of the study.
Conclusions:
Even patients with moderate-severe TBI can complete a demanding, online C-EE intervention and a self-administered symptom evaluation tool with limited therapist oversight, though at daily rate closer to 30 than 60 min per day. Further self-administered C-EE research is underway in our lab, with more extensive environmental support. Implications for Rehabilitation Online brain games (which may serve as a rehabilitation paradigm that can help offset the neurodegeneration observed in chronic TBI) can be feasibly self-administered by moderate-to-severe TBI patients. Brain games are a promising therapy modality, as they can be accessed by all moderate-to-severe TBI patients irrespective of geographic location, clinic and/or therapist availability, or impairments that limit mobility and access to rehabilitation services. Future efficacy trials that examine the effect of brain games for offsetting neurodegeneration in moderate-to-severe TBI patients are warranted.
... Potential explanations for this finding include embarrassment with taking medications in public, the need to take it several times a day and a desire to lose weight, particularly in adolescent females. 12,14,15 However, there were no gender differences in adherence in any group. Furthermore, all groups had a decline in their adherence over the 5 years. ...
Objective
We conducted a retrospective analysis of medication adherence and health outcomes over a 5-year period in children with cystic fibrosis (CF).Methods
Adherence was calculated for several commonly prescribed CF medications by comparing the actual number of times a prescription was filled in a 12-month period to the number of times it should have been filled based on the prescribed supply. We used prescription refill histories as documented by three major specialty pharmacies used by our patients. A binomial mixed effects model was used to investigate the longitudinal association between adherence and age group (0–5, 6–12, and 13–21 years) with gender, year in the study, lung function, body mass index (BMI), and annual hospitalization rate included as potential confounders.ResultsThe 0–5 years group had the highest overall adherence (P = 0.009). The 6–12 years group had significantly better adherence to inhaled medications as compared to oral medications (P = 0.020). Within each group, for any given year in the study, having a higher BMI was associated with greater odds of adherence (P < 0.0001). There were no associations between adherence and gender, lung function or hospitalization rate (P > 0.05).Conclusions
There are significant age differences in adherence. Younger patients have better overall adherence likely secondary to increased parental supervision. Having better nutritional status is associated with improved adherence. Pediatr Pulmonol. © 2015 Wiley Periodicals, Inc.
... Sex and age have an additional bearing on treatment adherence. Young female CF patients encounter significantly more emotional tension and concern about their disease, their degree of self-esteem is lower, they tend to be more readily disappointed about treatment, and their adherence in certain areas of treatment is inferior (56). CF adolescents feel and behave differently from CF adults. ...
There are no European recommendations on issues specifically related to lung transplantation (LTX) in cystic fibrosis (CF). The main goal of this paper is to provide CF care team members with clinically relevant CF-specific information on all aspects of LTX, highlighting areas of consensus and controversy throughout Europe. Bilateral lung transplantation has been shown to be an important therapeutic option for end-stage CF pulmonary disease. Transplant function and patient survival after transplantation are better than in most other indications for this procedure. Attention though has to be paid to pretransplant morbidity, time for referral, evaluation, indication, and contraindication in children and in adults. This review makes extensive use of specific evidence in the field of lung transplantation in CF patients and addresses all issues of practical importance. The requirements of pre-, peri-, and postoperative management are discussed in detail including bridging to transplant and postoperative complications, immune suppression, chronic allograft dysfunction, infection, and malignancies being the most important. Among the contributors to this guiding information are 19 members of the ECORN-CF project and other experts. The document is endorsed by the European Cystic Fibrosis Society and sponsored by the Christiane Herzog Foundation.
... Sex and age have an additional bearing on treatment adherence. Young female CF patients encounter significantly more emotional tension and concern about their disease, their degree of self-esteem is lower, they tend to be more readily disappointed about treatment, and their adherence in certain areas of treatment is inferior [56]. CF adolescents feel and behave differently from CF adults. ...
There are no European recommendations on issues specifically related to lung transplantation (LTX) in cystic fibrosis (CF). The main goal of this paper is to provide CF care team members with clinically relevant CF-specific information on all aspects of LTX, highlighting areas of consensus and controversy throughout Europe. Bilateral lung transplantation has been shown to be an important therapeutic option for end-stage CF pulmonary disease. Transplant function and patient survival after transplantation are better than in most other indications for this procedure. Attention though has to be paid to pretransplant morbidity, time for referral, evaluation, indication, and contraindication in children and in adults. This review makes extensive use of specific evidence in the field of lung transplantation in CF patients and addresses all issues of practical importance. The requirements of pre-, peri-, and postoperative management are discussed in detail including bridging to transplant and postoperative complications, immune suppression, chronic allograft dysfunction, infection, and malignancies being the most important. Among the contributors to this guiding information are 19 members of the ECORN-CF project and other experts. The document is endorsed by the European Cystic Fibrosis Society and sponsored by the Christiane Herzog Foundation.
Erratum to “Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis”
... Gender may or may not affect adherence. Some research suggests that adolescent girls are less adherent to CF treatment than adolescent boys [Patterson et al. 2008], while other research has not found an effect of gender on adherence to CF treatment [Masterson et al. 2011]. ...
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.
... Surprisingly, this study did not provide any further clues to this finding as there were few gender differences, except that females reported that overnight feeding is more important and they were more likely to be adherent. In fact, this is the opposite finding to another study on the dietary aspects of CF treatment which reported that females compared to males were less likely to adhere to a high-fat diet [27]. ...
... measured specific areas in CF care, such as health status, knowledge, attitudes, and behaviors, as well as several developed for patient satisfaction for both inpatient and outpatient settings. [5][6][7][8] An example of a widely used survey is the Hospital Consumer Assessment of Healthcare Providers and Systems for patients who had a hospital admission. 9 The UK CF Trust and several US CF care centers shared surveys they use to obtain information on patients' care experiences. ...
Objective:
The purpose of this study was to develop a cystic fibrosis (CF)-specific patient and family experience of care survey that CF care centers could use to inform quality improvement efforts.
Methods:
A literature search and query of CF care centers was conducted to identify existing surveys. Individuals with CF, their families, and health care professionals were also asked what to include. Following this process, a draft survey was developed and then reviewed by focus groups. Finally, a version was piloted at 25 CF care centers to validate and further refine the instrument.
Results:
No CF-specific surveys were found in the literature. Focus group participants stated that they understood the survey questions and that they covered important aspects of care, particularly infection control. The pilot test of the instrument with 485 participants supported its validity by demonstrating significant differences across centers and that most of the 3 care dimensions had acceptable internal consistency (Cronbach α: adults, 0.71-0.85; children, 0.68-0.79).
Conclusion:
A CF-specific patient and family experience of care survey was developed with input from individuals with CF, their families, and health care professionals. The instrument was validated and has been deployed to CF care centers.
... This credibility coupled with its ease of use (about 10 min to complete and of UK origin) means that it has been utilized in several trials for CF (Gee et al., 2003Gee et al., , 2005 Esmond et al., 2006). The CFQoL was selected for this study following careful examination of a number of QoL instruments such as the Cystic Fibrosis Questionnaire (CFQ14+; Henry et al., 2003) developed in France, Living with Cystic Fibrosis Questionnaire (LCFQ; Patterson et al., 2008 ) developed in the USA and EuroQol 5 Dimension for Children and Adolescents (EQ-5D-Y; Wille and Ravens-Sieberer, 2006; Wille et al., 2006a,b). Consequently, the CFQoL was considered to be the most robust, widely used and cross culturally translated CF specific QoL instrument (Debska and Mazurek, 2007; Monti et al., 2008). ...
Background: Health-related quality of life (HRQoL) is a rapidly growing area of expertise and the most commonly used patient-reported outcome (PRO). The impact of cystic fibrosis (CF) on HRQoL is liable to be great, making CF patients ideal candidates for the application of HRQoL instruments. The aims of this study were to assess the affect of CF on HRQoL, to ascertain the reliability and validity of the United Kingdom Sickness Impact Profile (UKSIP) and the Cystic Fibrosis Quality of Life Questionnaire (CFQoL) in the adult CF population, and to examine their role in the management of patients. Methods: Seventy participants were recruited from the All Wales Adult Cystic Fibrosis Centre at Llandough Hospital, UK. There were two stages to the study: self-report of the UKSIP and CFQoL; and completion of the same two questionnaires 7–10 days later. Results: The areas of HRQoL most impaired by CF were employment and concerns regarding the future. The UKSIP and CFQoL showed high internal consistency (rα = 0.89–0.93) and test–retest reliability (rs = 0.57–0.94, p < 0.005) in the CF population. Validity was variable with the UKSIP showing discrimination across socio-demographic factors, whilst the CFQoL showed increased sensitivity to clinical variables. Many parameters influenced patient-reported HRQoL, with the greatest correlations seen with the Borg score (p < 0.005). The use of a HRQoL instrument in CF annual reviews is recommended to provide holistic patient care. The results of this study underpin the value of HRQoL as a patient-reported outcome measure in the management of adult CF.
... Body image issues that may result in nutritional failure as well as differences in resting energy expenditure between males and females may also impact on survival through childhood. [25][26][27][28][29][30][31] This latter observation may be particularly important, and it is interesting to note that the female group that has experienced no change in mortality events is the vulnerable adolescent group aged 10-14 years. Our overall results for 0-14-year-old Australian children showed more marked reductions in mortality from 1998 onwards among girls than among boys, possibly reflecting the fact that male mortality rates had already fallen substantially and any further improvements were likely to be of much lesser magnitude. ...
To assess mortality trends among people with cystic fibrosis (CF) in Australia.
We augmented Australian summary data for deaths from CF registered during 1979-2005 with information from Australian transplant centres on lung transplantation among CF patients for 1989-2005 to allow us to follow trends in all "mortality events" (death or lung transplantation).
Age at death or lung transplantation.
Between 1979 and 2005, the mean age at death increased from 12.2 years to 27.9 years for males and from 14.8 years to 25.3 years for females. Overall, female deaths in childhood (0-14 years) occurred at an age-standardised rate of 0.40 per 100,000 (95% CI, 0.34-0.45) during 1979-2005, which exceeded the corresponding rate for males of 0.24 (95% CI, 0.20-0.28) per 100,000. Among 0-14-year-old boys, event rates declined markedly after 1989, but they declined later and more gradually for girls, with the result that the age-standardised rate for girls was 2.38 times that of boys during 1989-2005 (95% CI, 1.69-3.36).
The pattern of CF mortality in Australia has changed substantially. Mortality rates continue to be higher for girls than for boys, but death in childhood has become uncommon. Survival has increased since 1979, but females continue to have reduced length of life.
... Living with CF has a greater emotional impact on girls. 11 The 'thinness' common in CF can be a positive attribute for young women in their development of a sense of attractiveness and sexual self in western societies. This can also be a problem for men as they are overtaken in height and development by their peers as well as women. ...
The treatment of adolescents with CF is important, as adolescence is often when the CF patient's health deteriorates. They are managed by paediatricians then transitioned to adult teams. For the person with CF, adolescence usually constitutes a major chunk of their life span. The treatment is set on a background of dynamic developmental change. This can cause specific difficulties in management, especially related to eating and adherence level. Developing good relationships with young people, seeing things from their side and seeing them alone every time as well as with their families will help. They may have health-related issues to discuss with you and should be asked HEADS questions regularly. The multidisciplinary team is essential when working with young people. With the right approach, working with young people can be very enjoyable and productive to both parties.
... 10 Studies of adherence have identified important barriers. 11 In children and adolescents with CF, the following variables have been shown to relate to adherence: sociodemographic factors, such as adolescent age (12 years of age and older), 9,[12][13][14][15] gender, 16,17 knowledge of disease management and prescriptions, 12,18 time management, 11 patient-provider communication, 11 disease severity, 15,16 psychiatric/psychological risk factors, such as oppositional behaviors, 11 self-esteem, 13 coping strategies, 12,19 anxiety, 20 and family factors such as parental supervision, 21 quality of family relationships, 16,20 communication, conflict, and stress. 3,[22][23][24] However, little is known about the role depressive symptoms may play in CF adherence. ...
Background:
Little is currently known about the co-morbidity of depression and cystic fibrosis (CF) and there is currently no empirical research on the effects of depressive symptoms on adherence in children and adolescents with CF. The primary aim of this study was to evaluate the extent of depressive symptoms in children and adolescents with CF and their parents, and determine whether depressive symptoms in the child and/or parent was associated with adherence to airway clearance. We also evaluated whether children's perceptions of relational security with a parent were associated with adherence to airway clearance.
Methods:
Participants included 39 children with CF ages 7-17 years and their primary caregivers. Depressive symptoms were measured with the Children's Depression Inventory (CDI) and the Center for Epidemiological Studies-Depression Scale (CES-D). The Relatedness Questionnaire assessed the quality of parent-child relational security. Adherence to airway clearance was measured using the daily phone diary (DPD), an empirically validated adherence measure for youth with CF.
Results:
Rates of depressive symptoms were elevated in children with CF and their parents (29% for children; 35% for mothers; 23% for fathers). Child depressive symptoms were significantly associated with lower rates of adherence to airway clearance, after controlling for demographic variables (r = -0.34, P = 0.02). Child depressive symptoms were associated with worse perceptions of parental relationships (t(35) = 3.2; P = 0.002) and the quality of this relationship was also related to worse adherence (r = 0.42, P = 0.005).
Conclusions:
A large percentage of youth with CF and their parents reported elevated symptoms of depression. Children scoring in the depressed range on a standardized screening measure and those with less secure parent-child relationships were at greatest risk for poor adherence. Thus, depressive symptoms and family relationships are appropriate targets for adherence promotion interventions, which may ultimately improve health outcomes.
... This result differs from those of other studies, in which females displayed a more pessimistic perception of HRQOL. [27][28][29] It is probable that a larger group or a longer follow-up would establish this different view of HRQOL between the genders or would substantiate the data observed in the present study. ...
Health-related quality of life (HRQOL) measurements provide valuable information about the psychological and social impact of treatment on patients with cystic fibrosis (CF). This study evaluated the HRQOL of Brazilian patients with CF and assessed the changes in HRQOL domains over 1 year after dornase alfa (Pulmozyme) introduction.
One hundred fifty-six stable patients with CF and 89 caregivers answered the Portuguese-validated version of the Cystic Fibrosis Questionnaire-Revised (CFQ-R) at baseline (T(0)), and at 3 (T(1)), 6 (T(2)), 9 (T(3)), and 12 (T(4)) months of follow-up. Eighteen patients were excluded because they did not fulfill the inclusion criteria. The patients were analyzed in two groups: those aged 6-11 years and those aged 14 years and older. ANOVA for observed repeated results and the last observation carried forward (LOCF) method for missing data were used for the statistical analysis.
After 1 year of follow-up, there was significant improvement in respiratory symptoms (T(4) - T(0) = 8.1; 95% confidence interval (95% CI) = [2.1;14.0]; effect size (ES) = 0.35; P < 0.001), Emotional Functioning (T(4) - T(0) = 5.6; 95% CI = [1.1;10.1]; ES = 0.31; P < 0.05), Social Functioning (T(4) - T(0) = 6.0; 95% CI = [1.3;11.7]; ES = 0.31; P < 0.05), Body Image (T(4) - T(0) = 11.9; 95% CI = [4.1;19.7]; ES = 0.42; P < 0.05), and Treatment Burden (T(4) - T(0) = 5.3; 95% CI = [0.3;10.3]; ES = 0.24; P < 0.05) domains in the younger group. A significant improvement in Role Functioning (T(4) - T(0) = 6.1; 95% CI = [1.1;11.1]; ES = 0.40; P < 0.05), Body Image (T(4) - T(0) = 12.6; 95% CI = [3.5;21.7]; ES = 0.46; P < 0.05), and Weight (T(4) - T(0) = 11.7; 95% CI = [1.8;21.6]; ES = 0.40; P < 0.05) was obtained in the older group. The caregivers' CFQ-R showed improvements in the Digestive Symptoms (T(4) - T(0) = 5.5; 95% CI = [1.5;9.4]; ES = 0.30; P < 0.05), Respiratory Symptoms (T(4) - T(0) = 7.6; 95% CI = [3.9;11.4]; ES = 0.48; P < 0.05), and Weight (T(4) - T(0) = 10.1; 95% CI = [1.6;18.6]; ES = 0.26; P < 0.05) domains.
The introduction of dornase alfa improved the HRQL of the patients with CF during the first year of treatment.
... A higher median CD4 cell count for girls than for boys has been reported for US cohorts [5], but another study reported that being female was associated with poorer adherence [24]. Conflicting evidence has been obtained concerning the likelihood of compliance with treatment as a function of sex in adolescents with other chronic conditions [25,26]. In addition to behavioral issues, there may be alternative physiologic explanations not recorded in the EPF for these differences, such as pharmacokinetic differences in drug clearance [27]. ...
BACKGROUND. Increasing numbers of children perinatally infected with human immunodeficiency virus (HIV) are reaching adolescence, largely because of advances in treatment over the past 10 years, but little is known about their current health status. We describe here the living conditions and clinical and immunovirologic outcomes at last evaluation among this pioneering generation of adolescents who were born before the introduction of prophylaxis for vertical transmission and whose infections were diagnosed at a time when treatment options were limited. METHODS. The eligible population consisted of HIV-1-infected children who were born before December 1993 and who were included at birth in the prospective national French Perinatal Cohort (EPF/ANRS CO10). RESULTS. Of the 348 eligible children, 210 (60%; median age, 15 years) were still alive and regularly followed up. Current treatment was highly active antiretroviral therapy (HAART) in 77% and 2 nucleoside analogues in 5.0%; 16% had stopped treatment, and 2% had never been treated. The median CD4 cell count was 557 cells/microL, and 200 cells/microL was exceeded in 94% of patients. The median viral load was 200 copies/mL. Viral load was undetectable in 43% of the adolescents and in 54.5% of those receiving HAART. Median height, weight, and body mass index were similar to French reference values for age, and school achievement was similar to nationwide statistics. Better immunologic status was associated with being younger and with having begun HAART earlier. Undetectable viral load was associated with maternal geographic origin and current HAART. CONCLUSIONS. Given the limited therapeutic options available during the early years of these patients' lives and the challenge presented by treatment adherence during adolescence, the long-term outcomes among this population are encouraging.
This review summarizes the evidence of health disparities in cystic fibrosis (CF), an autosomal recessive genetic disorder with substantial variation in disease progression and outcomes. We review disparities by race, ethnicity, socioeconomic status, geographic location, gender identity, or sexual orientation documented in the literature. We outline the mechanisms that generate and perpetuate such disparities across levels and domains of influence and assess the implications of this evidence. We then recommend strategies for improving equity in CF outcomes, drawing on recommendations for the general population and considering approaches specific to people living with CF.
Background:
Improved understanding and treatment of cystic fibrosis (CF) has led to longer life expectancy, which is accompanied by an increasingly complex regimen of treatments. Suboptimal adherence to the treatment plan, in the context of respiratory disease, has been found to be associated with poorer health outcomes. With digital technology being more accessible, it can be used to monitor adherence to inhaled therapies via chipped nebulisers, mobile phone apps and web-based platforms. This technology can allow monitoring of adherence as well as clinical outcomes, and allow feedback to both the person with CF and their healthcare team.
Objectives:
To assess the effects of using digital technology to monitor adherence to inhaled therapies and health status in adults and children with CF.
Search methods:
We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. Date of last search: 28 October 2021. We also searched Embase and three clinical trial registries and checked references of included studies. Date of last search: 9 November 2021.
Selection criteria:
We searched for randomised controlled trials (RCTs) looking at the effects of a digital technology for monitoring adherence of children and adults with CF to inhaled therapies.
Data collection and analysis:
Two review authors screened the search results for studies eligible for inclusion in the review and extracted their data. We used Risk of Bias 2 for assessing study quality. We assessed the overall certainty of the evidence using GRADE.
Main results:
We included two studies in our review, with 628 participants aged five to 41 years. There was one study each for two different comparisons. Nebuliser target inhalation mode versus standard inhalation mode The included parallel study was carried out over 10 weeks after a run-in period of four to six weeks. The study compared the effects of a digitally enhanced inhalation mode (target inhalation mode) for nebulised antibiotics compared to standard mode in children attending a regional CF clinic in the United Kingdom. The study's primary outcome was the time taken to complete the inhaled treatment, but investigators also reported on adherence to therapy. The results showed that there may be an improvement in adherence with the target inhalation mode when this intervention is used (mean difference (MD) 24.0%, 95% confidence interval (CI) 2.95 to 45.05; low-certainty evidence). The target inhalation mode may make little or no difference to forced expiratory volume in one second (FEV1) % predicted (MD 1.00 % predicted, 95% CI -9.37 to 11.37; low-certainty evidence). The study did not report on treatment burden, quality of life (QoL) or pulmonary exacerbations. eNebuliser with digital support versus eNebuliser without support One large multicentre RCT monitored adherence via data-tracking nebulisers. The intervention group also receiving access to an online web-based platform, CFHealthHub, which offered tailored, flexible support from the study interventionist as well as access to their adherence data, educational and problem-solving information throughout the 12-month trial period. We graded all evidence as moderate certainty. Compared to usual care, the digital intervention probably improves adherence to inhaled therapy (MD 18%, 95% CI 12.90 to 23.10); probably leads to slightly reduced treatment burden (MD 5.1, 95% CI 1.79 to 8.41); and may lead to slightly improved FEV1 % predicted (MD 3.70, 95% CI -0.23 to 7.63). There is probably little or no difference in the incidence of pulmonary exacerbations or QoL between the two groups.
Authors' conclusions:
Digital monitoring plus tailored support via an online platform probably improves adherence to inhaled therapies and reduces treatment burden (but without a corresponding change in QoL) in the medium term (low- and moderate-certainty evidence). In a shorter time frame, technological enhancement of inhaling antibiotics may improve adherence to treatment (low-certainty evidence). There may be little or no effect on lung function with either intervention, and online monitoring probably makes no difference to pulmonary exacerbations. Future research should assess the effect of digital technology on adherence in both children and adults. Consideration of adherence to the total treatment regimen is also important, as an improvement in adherence to inhaled therapies could come at the cost of adherence to other parts of the treatment regimen.
Purpose
Cough is part of the daily life of patients with Cystic fibrosis (CF) and its most common symptom. This study explored the experiences of adolescents with CF in Iran during the COVID-19 pandemic in relation to their cough.
Design and methods
In this qualitative study, we conducted 32 semi-structured interviews with 21 adolescents with CF. We analyzed the data thematically.
Results
We identified three main themes among adolescents with CF in relation to coughing: 1. Cough is a permanent companion; 2. Coughing raises fear of double stigma; 3. Patients' individualized coping strategies to deal with coughing. Participants complained that cough interrupted daily tasks and sleep, drew unwanted attention in public places, and elicited questions about whether they were COVID-19 patients or substance users—both highly stigmatized identities.
Conclusion
Although coughing is a protective mechanism for CF patients, frequent coughing often causes major challenges, particularly during the COVID pandemic, when people were acutely sensitive and aware about coughing. During the COVID-19 pandemic, in addition to taking care of themselves and managing the disease, CF patients therefore had to also overcome issues related to social stigma and isolation.
Practice implications
Healthcare workers play an important role in increasing public awareness about CF and its symptoms, including cough. During the pandemic, healthcare workers can help reduce the stigma of coughing through public education. Healthcare workers can actively communicate with patients to identify severe and ineffective cases of cough due to exacerbation of the disease and refer them to a specialist.
Cystic fibrosis (CF) is an autosomal recessive multiorgan disease that is caused by mutations in a gene, cystic fibrosis transmembrane conductance regulator (CFTR). A host of epidemiology has demonstrated a “gender gap” or sex-based disparity in outcomes of people with CF, where females have more pulmonary exacerbations and a shortened life expectancy relative to males. The etiology of this disparity is not fully elucidated but appears to be multifactorial. In this chapter, we will review the sex-based differences in diagnosis of disease, pulmonary and extrapulmonary manifestations of CF, and host immune response as it relates to puberty, reproductive year, and menopause. We will also review the role of sex hormones on the sex disparity and the proposed mechanisms of how hormones such as estrogen, progesterone, and testosterone contribute to the differing outcomes.
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.
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.
Non-adherence to prescribed treatment is considered the foremost cause of treatment failure in chronic medical conditions. Airway clearance techniques (ACT) play a key role in the management of chronic suppurative lung disease yet, along with inhaled therapies such as nebulised antibiotics, adherence to these is often lower than to other treatments. In this review we discuss methods of monitoring adherence to these therapies and potential barriers and outline suggestions for improving adherence in the paediatric population.
Study Objectives
Infants, children and adolescents are increasingly being prescribed continuous positive airway pressure (CPAP) for treatment of obstructive sleep apnea (OSAS), yet adherence is often poor. The purpose of this study was to examine the relationship between caregiver and patient-reported health cognitions about CPAP prior to starting CPAP, and CPAP adherence at one month. We hypothesized that greater caregiver-reported self-efficacy would be positively associated with CPAP adherence in children. We also evaluated patient-reported self-efficacy, and caregiver- and patient reported risk perception and outcome expectations as they related to adherence, as well as how demographic factors influenced these relationships.
Methods
A pediatric modification of the Self-efficacy Measure for Sleep Apnea Questionnaire was administered to children and adolescents with OSAS prescribed CPAP, and their caregivers, during the clinical CPAP-initiation visit. The primary outcome variable for adherence was the average total minutes of CPAP usage across all days from the date that CPAP was initiated to 31 days later.
Results
Unadjusted ordinary least square regression showed a significant association between caregiver-reported self-efficacy and adherence (p=0.007), indicating that mean daily CPAP usage increased by 48.4 minutes when caregiver-reported self-efficacy increased by 1 point (95% CI of 13.4-83.4 minutes). No other caregiver- or patient-reported cognitive health variables was related to CPAP use.
Conclusions
This study indicates that caregiver CPAP-specific self-efficacy is an important factor to consider when starting youth on CPAP therapy for OSAS. Employing strategies to improve caregiver self-efficacy, beginning at CPAP initiation, may promote CPAP adherence.
The pressures patients with cystic fibrosis (CF) face in managing their disease through diet (i.e., high calorie and fat intake), weight, and exercise may trigger body image and eating concerns. This chapter describes the most recent literature on body image and disordered eating among those with CF. Treatment strategies and interventions aimed at screening for and reducing body dissatisfaction and disordered eating also are discussed. Overall, health care providers and families need to be aware of the body image concerns and disordered eating behaviors sometimes endorsed among those with CF and regularly monitor patient psychosocial well-being in the efforts to improve CF prognosis and survival.
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.
Treatment adherence is crucial in patients with cystic fibrosis, but poor adherence is a problem, especially during adolescence. Identification of barriers to treatment adherence and a better understanding of how context shapes barriers is of great importance in the disease. Adolescent reports of barriers to adherence have been studied, but studies of their parents' experience of such barriers have not yet been carried out. The aim of the present study was to explore barriers to treatment adherence identified by young patients with cystic fibrosis and by their parents.
A questionnaire survey of a cohort of young Danish patients with cystic fibrosis aged 14-25 years and their parents was undertaken.
Barriers to treatment adherence were reported by 60% of the patients and by 62% of their parents. Patients and parents agreed that the three most common barriers encountered were lack of time, forgetfulness, and unwillingness to take medication in public. We found a significant positive correlation between reported number of barriers and perceived treatment burden. We also found a statistically significant relationship between the reported number of barriers and treatment adherence. A significant association was found between the number of barriers and the reactions of adolescents/young adults and those of their mothers and fathers, and between the number of barriers and the way the family communicated about cystic fibrosis.
The present study showed that the majority of adolescents with cystic fibrosis and their parents experienced barriers to treatment adherence. Agreement between adolescents and their parents regarding the level and types of barriers indicates an opportunity for close cooperation between adolescents, their parents, and health care professionals in overcoming adolescent adherence problems.
The goal of the present research was to examine the impact of age and gender on adherence to both infection control (IC) guidelines and traditional medical treatments in a cystic fibrosis (CF) population. Adherence behaviors are consistently suboptimal in chronic illness populations, particularly pulmonary diseases; understanding the factors related to adherence behaviors in CF can aid in the development of interventions to promote adherence.
Participants consisted of 74 individuals with CF ages 9 years and above. Participants were asked to complete questionnaires designed to assess demographic data, treatment adherence, and health beliefs.
With respect to IC guidelines, chi-square analyses revealed significant age differences in adherence behaviors such that the young adult subsample was least adherent to IC (χ2 = 15.10, df = 6, P = 0.020). Next, a 4 (age: child, adolescent, young adult, adult) × 2 (gender) completely between subjects analysis of variance (ANOVA) was conducted on medical treatment adherence. There was a significant main effect for age [F(3, 65) = 2.940, P = 0.040, ηP2 = 0.119] indicating that the adolescent subsample had the most adherence challenges. Gender was nonsignificant across both adherence types.
Study findings are suggestive of age-related differences in adherence behaviors across both IC and medical regimens and support the use of developmentally sensitive approaches to assessment and interventions addressing adherence. Pediatr Pulmonol. 2011; 46:295–301.
This study examined marital role strain in 33 couples caring for a child with cystic fibrosis (CF) and 33 couples with a healthy child. The relationship between role strain, marital satisfaction, and psychological distress was tested. Couples completed a structured interview, questionnaires, a card sort procedure, and 4 daily diaries assessing activities and mood. Couples in the CF versus comparison group reported greater role strain on measures of role conflict, child-care tasks, and exchanges of affection. They also spent less time in recreational activities, but no reliable group differences were found in marital satisfaction or depression. Regression analyses indicated that role strain was related to marital satisfaction and depression and that recreation time accounted for additional variance. Path analysis suggested that recreation mediated the negative relationship between role strain and distress. The importance of using a contextual, process-oriented approach is discussed.
The development of autonomy is a major goal for individuals with a disabling condition This is particularly true in the transition from adolescence to adulthood The emphasis, in the past, has been upon independence from the assistance especially physical assistance, of others More recent developments in the fields of special education, adolescent development, and family systems suggest a need to redefine autonomy In the model offered here, autonomy is defined in terms of individuals' capacities to take responsibility for their own behavior to make decisions regarding their own lives, and to maintain supportive social relationships The development of autonomy is viewed as being dependent upon development of an internal locus of control, use of a set of coping strategies, maintenance of a support network, and development a reciprocal adult attachment These, in turn, are affected by aspects of the family system This model and its implications for individuals with a disabling condtion are described.
Presents a family stress model, the family adjustment and adaptation response (FAAR) model, and suggests conceptual links to other models of stress. The FAAR model focuses on 3 systems: the individual, the family, and the community. The family system and its efforts to maintain balanced functioning by using its capabilities (resources and coping behaviors) to meet its demands (stressors and strains) are emphasized. This effort to balance demands and capabilities is mediated by the meanings the family ascribes to events. Over time, families go through repeated cycles of adjustment (characterized by relative stability), crisis, and adaptation (characterized by discontinuous, 2nd-order change). (PsycINFO Database Record (c) 2012 APA, all rights reserved)
This study examined marital role strain in 33 couples caring for a child with cystic fibrosis (CF) and 33 couples with a healthy child. The relationship between role strain, marital satisfaction, and psychological distress was tested. Couples completed a structured interview, questionnaires, a card sort procedure, and 4 daily diaries assessing activities and mood. Couples in the CF versus comparison group reported greater role strain on measures of role conflict, child-care tasks, and exchanges of affection. They also spent less time in recreational activities, but no reliable group differences were found in marital satisfaction or depression. Regression analyses indicated that role strain was related to marital satisfaction and depression and that recreation time accounted for additional variance. Path analysis suggested that recreation mediated the negative relationship between role strain and distress. The importance of using a contextual, process-oriented approach is discussed.
This study investigates the role of certain psychosocial variables--sex, age, body image/self-esteem, self-consciousness, stressful life events, and the degree to which an individual identifies with the cultural stereotype of masculinity--as correlates and antecedents to depression in adolescents and explores possible intraindividual mediators of the stress-depression relationship in adolescents. A battery of self-report measures was administered to public high school students in Grades 9-12 in their classrooms at two different times 1 month apart. Female adolescents reported more depressive symptoms, self-consciousness, stressful recent events, feminine attributes, and negative body image and self-esteem; no age effects were obtained. Results suggest a model of adolescent depression in which body/self-esteem and stressful recent events are significant contributors.
Applied Goldfriend and D'Zurilla's (1969) Behavior-Analytic Model to the development of a context-specific measure of stressful situations for adolescents with a serious, chronic illness. In completing the situational analysis phase of the model, 45 adolescents with cystic fibrosis (CF), 20 parents of adolescents with CF, and 8 health care professionals completed structured interviews or daily diaries to obtain the widest range of problematic situations. The adolescent sample was recruited from two different medical centers, and stratified by sex and illness severity. A total of 1,174 problem situations were elicited across all participants and then content-analyzed into 164 nonredundant items in 10 domains (e.g., Medications and Treatment, and School). Few relations were found between demographic variables (e.g., age and illness severity) and the number or difficulty of problematic situations. Both adolescents and parents mentioned the greatest number of problematic situations in the domains of School, Medications and Treatment, and Parent-Teen Relationship. In terms of difficulty, all three respondents (i.e., teens, parents, and health care professionals) rated problems with Clinic and Hospital Visits as very difficult. For the adolescent sample, problems in the Parent-Teen Relationship and Health Concerns were also highly difficult. Significant associations were found between the problematic situations we identified and standardized measures of social and emotional functioning. Adolescents who rated their problems as more difficult also endorsed more symptoms of depression and lower perceptions of social competence. In a future study, the most salient items will be selected to create a role-play measure to elicit adolescents' coping strategies.
To determine sex-specific, age-related changes in physical activity patterns.
We examined cross-sectional data from the National Health Interview Survey, using the 1992 Youth Risk Behavior Survey supplement for adolescents and the 1991 Health Promotion/Disease Prevention supplement for adults. Physical activity patterns were modeled after Healthy People 2000 objectives.
Among adolescents, physical activity patterns generally eroded most from ages 15 through 18. The "regular, vigorous activity" and strengthening patterns declined consistently from ages 12 through 21. Young adulthood (18-29 yr) often marked continuing erosion of activity patterns, whereas middle adulthood (30-64 yr) often revealed relatively stable patterns. At retirement age (65 yr), there was a stabilizing, or even an improving, tendency in activity patterns, usually followed by further erosion through the final period of life. Strengthening behavior eroded dramatically with advancing age among adults, especially among men. Among adolescents, differences between female and male respondents were large for regular, vigorous activity (11.3 percentage points greater for male respondents). In comparison with female adolescents and adults, male respondents reported much higher rates of regular, sustained activity (5.5 and 5.9 percentage points, respectively), and strengthening (18.2 and 11.3 percentage points, respectively). Among adults, levels of physical inactivity among women were moderately greater (5.5 percentage points) than for men. Absolute rates of change per year were mostly large to very large (3.0-8.0 percentage points x yr(-1)) during ages 15-18 yr, but, for adults, they were small (<0.5 percentage points x yr(-1)) for 33 of 40 sex, age, and pattern groupings.
These data suggest that early and continued intervention will be necessary to offset these declines in physical activity throughout adolescence and adulthood.
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.
(1) To compare habitual activity levels in prepubescent and pubescent boys and girls with different degrees of CF lung disease severity and healthy controls. (2) To assess the relation between habitual activity levels and measures of fitness, lung function, nutrition, pancreatic status, and quality of life.
A total of 148 children (75 girls and 73 boys) with CF and matched controls were studied. Regardless of disease severity, there were no differences in habitual activity between prepubescent boys and girls with CF. Pubescent boys with CF were significantly more active than girls with the same degree of disease severity. There were no significant differences in habitual activity between prepubescent children with CF and controls. Pubescent children with mild CF were significantly more active than controls, but those with moderate to severe disease were less active than controls. The best correlates with habitual activity levels were anaerobic power, aerobic capacity, and quality of life. In children with moderate to severe disease, nutrition status correlated significantly with activity levels. The impact of pancreatic status on activity levels and other measures of fitness was most apparent in pubescent girls.
Gender differences in habitual activity were evident only after the onset of puberty. The impact of pancreatic insufficiency on measures of fitness and habitual activity was greatest in pubescent females. The reason for this gender difference may be an interplay of genetic, hormonal, and societal factors and is the focus of a longitudinal study.
The Cystic Fibrosis Questionnaire (CFQ) is a disease-specific instrument that measures health-related quality of life (HRQOL) for adolescents and adults with cystic fibrosis (CF) > or = 14 years, consisting of 44 items on 12 generic and disease-specific scales. Versions of the CFQ are also available for children with CF and their parents. This study evaluated the psychometric properties of the CFQ in a national study at 18 CF centers in the United States.
The CFQ-teen/adult was administered to 212 patients with CF ranging in age from 14 to 53 years. Test-retest reliability was assessed in a subset of patients over a 10- to 14-day interval.
Multitrait analysis indicated a majority of items (95%) correlated more highly with their intended scale than a competing scale, supporting the conceptual model. Internal consistency coefficients indicated the CFQ scales had good reliability (Cronbach alpha = 0.67 to 0.94), and test-retest stability was acceptable (rs = 0.45 to 0.90). Validity was demonstrated by examining relationships between the CFQ, age, pulmonary function, and body mass index. As expected, the CFQ was inversely correlated with age, with older adults reporting lower CFQ scores than younger adults, better nutritional status was positively correlated with several weight-related scales, and the measure differentiated between individuals with varying levels of disease severity. Strong associations were also found between the CFQ and similar scales on the Short Form-36 Health Questionnaire, a well-known generic HRQOL measure.
The results demonstrated that the CFQ-teen/adult is a reliable and valid measure of HRQOL for individuals with CF. It may be utilized in clinical trials to assess the effects of new therapies, to document the progression of disease, and to inform clinical practice.
The purpose of the current study was to evaluate the psychometric properties of the Cystic Fibrosis Questionnaire (CFQ)-Child version, a disease-specific health-related quality of life (HRQOL) measure for children with cystic fibrosis (CF).
The CFQ was administered to 84 children with CF, ranging in age from 7 to 13 years, and their parents.
Multitrait analyses indicated that a majority of items on the CFQ-Child correlated more highly with their hypothesized scale than a competing scale. Internal consistency coefficients were acceptable for all scales (Cronbach's alpha =.60-.76), with the exception of treatment burden (Cronbach's alpha =.44). Results also suggested strong convergence between child and parent-proxy reports on several scales of the CFQ.
Results demonstrated that the CFQ-Child is a reliable and valid measure of HRQOL for children with CF.
Better understanding of evolution of lung function in infants with cystic fibrosis (CF) and its association with pulmonary inflammation and infection is crucial in informing both early intervention studies aimed at limiting lung damage and the role of lung function as outcomes in such studies. To describe longitudinal change in lung function in infants with CF and its association with pulmonary infection and inflammation. Infants diagnosed after newborn screening or clinical presentation were recruited prospectively. FVC, forced expiratory volume in 0.5 seconds (FEV(0.5)), and forced expiratory flows at 75% of exhaled vital capacity (FEF(75)) were measured using the raised-volume technique, and z-scores were calculated from published reference equations. Pulmonary infection and inflammation were measured in bronchoalveolar lavage within 48 hours of lung function testing. Thirty-seven infants had at least two successful repeat lung function measurements. Mean (SD) z-scores for FVC were -0.8 (1.0), -0.9 (1.1), and -1.7 (1.2) when measured at the first visit, 1-year visit, or 2-year visit, respectively. Mean (SD) z-scores for FEV(0.5) were -1.4 (1.2), -2.4 (1.1), and -4.3 (1.6), respectively. In those infants in whom free neutrophil elastase was detected, FVC z-scores were 0.81 lower (P=0.003), and FEV(0.5) z-scores 0.96 lower (P=0.001), respectively. Significantly greater decline in FEV(0.5) z-scores occurred in those infected with Staphylococcus aureus (P=0.018) or Pseudomonas aeruginosa (P=0.021). In infants with CF, pulmonary inflammation is associated with lower lung function, whereas pulmonary infection is associated with a greater rate of decline in lung function. Strategies targeting pulmonary inflammation and infection are required to prevent early decline in lung function in infants with CF.
‘This is an excellent book which introduces the underlying concepts and practical issues related to psychosocial measurement and scale development’ - Statistics in Medicine. Effective measurement is a cornerstone of scientific research. Yet many social science researchers lack the tools to develop appropriate assessment instruments for the measurement of latent social-psychological constructs. Scaling Procedures: Issues and Applications examines the issues involved in developing and validating multi-item self-report scales of latent constructs. Distinguished researchers and award-winning educators Richard G Netemeyer, William O Bearden, and Subhash Sharma present a four-step approach for multi-indicator scale development. With these steps, the authors include relevant empirical examples and a review of the concepts of dimensionality, reliability, and validity. Scaling Procedures: Issues and Applications supplies cutting-edge strategies for developing and refining measures. Providing concise chapter introductions and summaries, as well as numerous tables, figures, and exhibits, the authors present recommended steps and overlapping activities in a logical, sequential progression. Designed for graduate students in measurement//psychometrics, structural equation modeling, and survey research seminars across the social science disciplines, this book also addresses the needs of researchers and academics in all business, psychology, and sociology-related disciplines.
Although the methodological literature is replete with advice regarding the development and validation of multi-item scales based on reflective measures, the issue of index construction using formative measures has received little attention. The authors seek to address this gap by (1) examining the nature of formative indicators, (2) discussing ways in which the quality of formative measures can be assessed, and (3) illustrating the proposed procedures with empirical data. The aim is to enhance researchers' understanding of formative measures and assist them in their index construction efforts.
in this chapter, a revision of the FAAR [family adjustment and adaptation response] model is introduced with two objectives in mind
first, there is an effort to revise and clarify concepts based on more recent empirical findings
second, there is an attempt to clarify further the linkages to physiologic, psychologic, and sociologic models of stress so as to render the model more salient for family medicine research
the "family" as one system in a heirarchy of systems (PsycINFO Database Record (c) 2012 APA, all rights reserved)
Adolescence is frequently described as a period of pervasive self-consciousness, but an age-related peak in adolescence is not consistently obtained, and higher self-consciousness in girls is frequently obtained but not predicted by theoretical accounts. Two cohorts of adolescents (N=393), initially assessed at 13 and 15, completed public and private self-consciousness measures 3 times in 4 years. They also reported social comparisons and social engagement. Public self-consciousness decreased and private self-consciousness increased in both cohorts, and girls scored higher on both measures, both in longitudinal and sibling replication samples (n=188). Public self-consciousness appears to be a normative response to adolescent social challenges, with girls' higher levels largely attributable to their closer social engagement. Private self-consciousness emerges as an individual difference in adolescence but is more likely to be salient and predictive of social behavior in adulthood.
This article proposes a multilevel systems model of the interactive biological, psychological, and social processes in childhood illness. This heuristic model can organize the investigation of pathways and mechanisms by which these levels influence one another. A pivotal feature of this biobehavioral family model is the construct of responsivity, which is conceptualized at both family (interpersonal responsivity) and individual (biobehavioral reactivity) levels. Individual biobehavioral reactivity is proposed as a bidirectional pathway by which family patterns and disease processes influence one another. Illustrative clinical material and relevant research are presented with recommendations for future study. This model integrates individual and family-level theory and thus has the capacity to bridge the fields of psychosomatics, pediatrics, behavioral medicine, and family systems medicine.
Limb deficiencies in children are the result of trauma, disease, or congenital causes. The potentially negative impact on psychologic adjustment from the chronic strain of living with limb deficiencies appears to be mediated by perceived social support (interpersonal protective factor), microstressors and daily hassles (socioenvironmental risk factor), and self-esteem (intrapersonal protective factor). These risk and protective factors were simultaneously investigated as potential predictors of depressive symptomatology in 54 children with congenital or acquired limb deficiencies. Hierarchical multiple regression analyses were used to test the main effects and the interaction effects of the predictor variables on depressive symptomatology. None of the interaction terms were statistically significant. An overall simultaneous multiple regression analysis of the main effects model predicted 72% of the variance in depressive symptomatology, with perceived classmate social support emerging as the strongest predictor variable. The findings are discussed in terms of the risk and protective effects of mediating factors on the psychologic and social adaptation of chronically ill and handicapped children.
The changing expectations, therapies and outcomes for young people with cystic fibrosis (CF) necessitate a re-evaluation of the impact of this chronic illness on adolescent development. The aim of this study was to assess the psychological well-being and adjustment of contemporary adolescents with CF.
Forty-nine adolescents with CF (24 males, 25 females) aged 14-18 years were enrolled from the Royal Children's Hospital CF Clinic, Melbourne, Australia. The Offer Self-Image Questionnaire was administered to participating subjects whose growth and pubertal developmental and pulmonary function was assessed.
The mean (SD) FEV1% was 82 (21), and mean (SD) National Institute of Health clinical score was 81 (12). There were no significant differences between males and females in pulmonary function or clinical scores, but growth and pubertal development were delayed in both sexes. Females, but not males, with CF were less well-adjusted than their healthy peers. Two-way analysis of variance was performed and showed significant sex differences in sub-scales of the Offer questionnaire numbers I, II, III, VI, VIII, IX, as well as in the total score. Age was significant only for scales II and VIII, and only scale II displayed an interaction between sex and age.
This study suggests that adjustment and self-esteem are less than ideal in young people with CF, especially females.
Variations in physical activity and aerobic power are examined in relation to age and gender of children and adolescents. Reviews of the world literature show aerobic power relative to body mass remains stable from ages 6 to 16 for males, but for females it declines about 2% per year. Overall, males are about 25% more fit than females. Reviews of nine studies using standardized self-reports or objective measures of physical activity revealed that males are about 15 to 25% more active than females. Over the school age years, a consistent decline in physical activity is seen, with males decreasing about 2.7% per year and females decreasing about 7.4% per year. These data suggest that older youth and females are at increased risk of obesity because of a sedentary lifestyle.
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.
This qualitative study used a grounded theory approach to explore adolescent conceptualizations of their chronic illness experience and related life events. A purposive sample of 20 adolescents (12-18 years of age) with cystic fibrosis were interviewed. Adolescents used three protective strategies for reducing a sense of difference from peers: (1) keeping secrets, (2) hiding visible differences, and (3) discovering a new baseline. "Good friends" were a critical source of support and decreased the importance of differences in their social world. Interventions should focus on strategies for dealing with difficult peer situations and the negative reactions of others.
The authors conducted the largest study to date of survival in cystic fibrosis. The study cohort consisted of all patients with cystic fibrosis seen at Cystic Fibrosis Foundation-accredited care centers in the United States between 1988 and 1992 (n = 21,047), or approximately 85% of all US patients diagnosed with cystic fibrosis. Cox proportional hazards regression analysis was used to compare the age-specific mortality rates of males and females and to identify risk factors serving as potential explanatory variables for the gender-related difference in survival. Among the subjects 1-20 years of age, females were 60% more likely to die than males (relative risk = 1.6, 95% confidence interval 1.4-1.8). Outside this age range, male and female survival rates were not significantly different. The median survival for females was 25.3 years and for males was 28.4 years. Nutritional status, pulmonary function, and airway microbiology at a given age were strong predictors of mortality at subsequent ages. Nonetheless, differences between the genders in these parameters, as well as pancreatic insufficiency, age at diagnosis, mode of presentation, and race, could not account for the poorer survival among females. Even after adjustment for all these potential risk factors, females in the age range 1-20 years remained at greater risk for death (relative risk = 1.6, 95% confidence interval 1.2-2.1). The authors concluded that in 1- to 20-year-old individuals with cystic fibrosis, survival in females was poorer than in males. This "gender gap" was not explained by a wide variety of potential risk factors.
Chronic progressive lung disease is the most prominent cause of morbidity and death in patients with cystic fibrosis (CF), but severity of lung disease and rate of lung function decline are widely variable. Accurate estimates of decline have been difficult to define and compare because the timing of measurements and duration of follow-up differ in various patient groups.
Three hundred sixty-six patients with CF, born from 1960 to 1974, were selected from a CF database birth cohort if they had two or more measurements of pulmonary function, at least one of which was performed before the age of 10 years.
Mixed model regression analysis provided estimates of the average rate of decline of spirometry measurements in subgroups on the basis of survival age, sex, pancreatic status, and genotype.
Patients who died before the age of 15 years had significantly poorer pulmonary function when first tested and a more rapid decline in pulmonary function thereafter than patients who survived beyond the age of 15 years. In the latter, functional levels at the age of 5 years were normal, but average rates of decline were significantly related to survival age. Female patients had significantly steeper decline than male patients, and those with pancreatic insufficiency had much steeper decline than those with pancreatic sufficiency. In the subset of 197 who survived to 1990 and were subsequently genotyped, rate of decline was greater in those homozygous for the delta F508 mutation, compared with those who were heterozygous for delta F508 or those, who had two other mutations.
All but the most severely affected patients, who died before age 15, appear to have had normal pulmonary function when first tested in early childhood. Pancreatic sufficiency, male gender, and some non-delta F508 mutations are associated with a slower rate of pulmonary function decline. Mixed model analysis is a valuable tool for describing and comparing pulmonary function decline in groups of patients with CF.
Length of survival of females with cystic fibrosis is worse than it is in males. Results of current research have shown an important correlation among dietary intake, nutritional status, lung function, and survival. The purpose of this study was to explore gender differences in dietary intake and pancreatic enzyme replacement therapy in males and females with cystic fibrosis.
The study was a cross-sectional measurement of clinical characteristics, energy, and fat intakes in males and females attending the cystic fibrosis outpatients clinics of the John Hunter Hospital, Newcastle, Australia. Twenty-nine subjects, (17 females and 12 males), completed 4-day weighed food records to measure total energy intake and the contribution of macronutrients and to document use of pancreatic enzyme replacement therapy. Energy intake was assessed as the percentage of the recommended energy intake for age and sex.
Females with cystic fibrosis had significantly lower energy and fat intakes than males, whereas the females used significantly more pancreatic enzyme replacement therapy. There were no significant differences in clinical characteristics between groups.
The results support the possibility that gender differences in the energy and fat intakes of older patients may contribute to differential median survival time of males and females with cystic fibrosis.
Although most research linking health disadvantage with gender has focused on women, recent work indicates that hegemonic masculinities can also place the health of men at risk. The importance of comparing the experiences of women and men has been emphasised and this paper focuses on the ways in which the social constructions of femininities and masculinities affect how teenagers live with asthma or diabetes. The majority of girls incorporated these conditions and the associated treatment regimens into their social and personal identities, showing a greater adaptability to living with asthma or diabetes. However, this could have detrimental effects in terms of control, as girls sometimes lowered expectations for themselves. In addition, two aspects of the treatment regimens, diet and exercise, were found to disadvantage girls and advantage boys, because of contemporary meanings of femininities and masculinities. The social construction of femininities meant that these conditions were not seen as the threat that they were by the majority of boys interviewed, who made every effort to keep both conditions outside their personal and social identities by passing. The majority of boys maintained a 'valued' identity by feeling in control of their body and their condition. However, for the small minority of boys who were no longer able to pass the impact of chronic illness led to a 'disparaged' identity. The interaction of gender and health is seen as a complex two-way process, with aspects of contemporary femininities and masculinities impacting on the management of these conditions, and aspects of these conditions impacting in gendered ways upon the constructions of gender.
To identify and compare perceived supportive and nonsupportive behaviors exhibited by family members and friends toward adolescents with cystic fibrosis (CF), and to examine the relationships between supportive and nonsupportive behaviors and adolescents' psychological adjustment.
Participants were 35 adolescents with CF attending the Women's and Children's Hospital in South Australia. Perceived supportive and nonsupportive behaviors were assessed using an adapted version of the Chronic Disease Support Interview. The psychological adjustment of the adolescents was assessed using the Youth Self Report Form. Repeated-measures analyses of variance were performed to compare the support provided by family members and friends. Multiple regression analyses assessed the contribution of supportive and nonsupportive behaviors for the prediction of psychological adjustment.
Family members provided more tangible support than friends who, conversely, provided more companionship support. Overall, family members scored higher than friends on ratings for supportive behaviors. No differences were observed between family members and friends on ratings for nonsupportive behaviors. Rating of nonsupportive behaviors for family members was found to be the strongest predictor of psychological adjustment.
Family members and friends provide different types of support. Family members provide more tangible help with treatment tasks and adolescents provide more companionship. Overall, this study demonstrated the importance of addressing nonsupportive as well as supportive behaviors when investigating the impact of support on the psychological adjustment of adolescents with a chronic illness.
This study aimed to elucidate the relationship between maternal psychosocial factors related to dietary adherence and growth outcomes in their children with cystic fibrosis (CF). Twenty-five children with CF and mild lung disease, aged 7-12 years, were weighed and measured and completed detailed food records. Mothers completed questionnaires that measured general nutritional knowledge, nutritional knowledge specific to CF, coping strategies, dieting behaviors, self-efficacy about the ability to provide the correct diet, and attitudes and beliefs about CF. Of the cohort, only 16% adhered to the dietary recommendations. Dietary adherence was positively correlated with weight. Maternal nutritional knowledge specific to CF significantly predicted children's dietary adherence score. There was a significant negative correlation between the child's weight and mothers who were dieting successfully. Other psychosocial factors assessed were not related to dietary adherence. Improving the mother's knowledge about nutritional needs in CF appears worthwhile and may have an impact on growth.
This study was a longitudinal examination of the change in both the activities done and the intensity of those activities from childhood to adolescence.
Common activities were assessed by questionnaire initially on 656 subjects from 21 elementary schools; 50.5% were female, 83.4% were Caucasian, 20.6% African-American, and 6.0% were other races.
Girls more often reported sedentary activities overall. Weighted least squares analyses showed boys consistently reported more vigorous activities than girls (P < 0.0008). African-American girls reported fewer vigorous activities than Caucasian or other race girls (P = 0.027). Sedentary activities were more frequently reported with increasing age (X2 P < 0.001). The youngest African-American and Caucasian boys reported similar activity patterns. However, boys from other races reported more intense activities until sixth and seventh grades when African-American boys began reporting more sedentary activities than Caucasians or other races (P = 0.004). During sixth-eighth grades, Generalized Estimating Equations (GEE) models show that girls with more advanced pubertal status reported more sedentary activities than girls who were less developed (P < 0.0001). For high school girls, race was a marginally significant predictor (P = 0.05) of activity status. Neither race nor pubertal status were significant factors in activities chosen by middle school boys. However, for male high school students, Caucasians were more likely than African-Americans to report vigorous activities (P = 0.005).
Variation in activities by race within gender suggests that establishing activity patterns in youth may be race-specific as well as gender-specific and must be accounted for in designing physical activity interventions. Also, pubertal maturation is a factor in activity choices in middle school girls.
Treatment aimed at achieving an ideal nutritional status is an integral part of the management of patients with cystic fibrosis (CF). Emphasis is continually placed upon dietary intake and weight. The effects of this on eating behavior and self-perceptions are unclear. This work compared male and female CF adults with a healthy male and female control population with regard to (a) clinical variables, (b) actual, perceived, and desired body shape/body mass index (BMI), and (c) body satisfaction, eating behaviors and attitudes, and self-esteem. Clinical data were recorded for 221 adults with CF and 148 healthy controls. All subjects completed BMI Charts (perception of body weight/BMI), the Eating Attitudes Test, and scales of body satisfaction and self-esteem. CF patients had poorer lung function and nutritional status than controls. Control males accurately perceived their body shape/BMI and were content with it, whereas CF males viewed their BMI as greater than it actually was and desired to be much heavier. Control females viewed their body shape/BMI as less than it actually was and desired to be even slimmer, in comparison with CF females, who perceived their BMI as less than it actually was but were happy with their perceived shape/weight. Control subjects, especially females, dieted to a greater extent and were more preoccupied with food (with binge eating and intended vomiting) than CF patients. Conversely, those with CF reported greater pressure from others to eat than did controls. More problems with food/eating behavior were associated with less body satisfaction and reduced self-esteem. In comparison with a healthy control population, the perceptions and behaviors of CF adults relating to eating, weight, and body image are not abnormal. Indeed, females with CF have fewer problems than their healthy peers.
Good nutritional status in cystic fibrosis (CF) is associated with improved clinical status and survival. In some conditions where dietary and pharmacological treatment are important (e.g. diabetes), a combination of eating disorders and failure of treatment compliance has been reported. Cases of eating disorders have been reported in CF. Societal pressures on young women to remain slim may compromise optimum clinical management as women are content to remain underweight.
To determine whether women have different perception of their weight than men with CF and whether this manifests in different nutritional behaviour.
Confidential postal questionnaire to 1870 adults with CF in 1994 known to the Association of Cystic Fibrosis Adults (UK). Participants were asked their own weight and height, and their perception of their weight. Very underweight was defined as < 85% ideal body weight, underweight as 85-94%, normal weight as 95-104%, overweight as 105-114% and very overweight as 115% and over.
A significantly higher proportion of women than men who are very underweight (29% vs. 11%) or underweight (41% vs. 15%) saw themselves as being of normal weight or overweight. Conversely, a significantly higher proportion of men than women who were normal weight (42% vs. 19%) saw themselves as underweight. All P-values < 0.01. A significantly higher proportion of people who perceived themselves to be underweight were taking oral or enteral food supplements compared with those who did not (77% vs. 30%, P < 0.05). Multiple logistic regression showed that perception of self as underweight was the strongest predictor of taking oral or enteral food supplements (adjusted odds ratio 2.42), even after adjustment for age, sex, overall severity score, body mass index, and seeing a dietitian in the last year.
Young women with CF tend to overestimate their weight, and young men with CF underestimate their weight when compared with their actual body weight. Perception of self as underweight is reflected in nutritional behaviour, being a significantpredictor of taking oral and enteral food supplements. People working with young patients with CF should be aware of these sex differences in weight perception, and work with young women and young men with CF to achieve a realistic perception of body weight and realistic nutritional goals. There is a need for further research into body image, weight perception, eating behaviour and adherence to pancreatic enzyme supplementation and oral and enteral food supplementation in young patients with CF.
In the context of improvements in both longevity and the quality of life for people with chronic disease, this paper takes as its sense of problem the differential life expectancy for young men as against young women suffering from cystic fibrosis. From a qualitative study of the transition to adulthood for young people with the disease, a theory of gendered embodiment is proposed to explain this differential. The social construction of masculinity and femininity as social practices resulted in the former being more conducive to survival than the latter in this case. There were marked differences between the young women and young men in attitudes to: the meaning of life, death, career and body image; all of which affected adherence to medical regimen.
Cystic fibrosis (CF) is the most common inherited disease of childhood. Caring for a child with CF is stressful and this has implications for the main carer, the parental relationship, well siblings, family functioning as well as the affected child.
This study aimed to explore current stresses and coping strategies used by mothers and to identify roles and strategies that nursing professionals could extend or adopt to support them and families of children with CF.
Semi-structured interviews were carried out with 17 mothers of children with CF who attended the regional cystic fibrosis hospital clinic. Interviews were analysed using the Framework method of analysis.
The study suggests that major stresses for many mothers are feeling in the middle in terms of decision-making particularly concerning the genetic implications of CF, the burden of responsibility for parenting a child with a chronic illness, and coming to terms with a personal change in identity. The most commonly used coping strategy was seeking support from others including nursing professionals. Relationships with health professionals in secondary care were generally positive, with nurses often the first people mothers turned to when they had concerns and also key in interpreting medical information. Relationships with primary care health professionals appeared to be more limited with a role reversal described by some mothers who felt it their responsibility to educate and inform primary care professionals about CF.
This study suggests that nursing professionals in primary and secondary care need to look beyond the care of the child to the needs of the mother. It provides evidence to suggest that nursing professionals in primary and secondary care have a number of important roles to play as holders of hope, bridge builders and in providing continuity of care for such families. It also suggests strategies to strengthen nurse-patient relationship.
To investigate age-specific trends in survival among US patients with cystic fibrosis between 1985 and 1999 and to assess whether survival in female patients with cystic fibrosis has improved relative to survival in male patients.
A retrospective cohort study of 31,012 subjects in the US Cystic Fibrosis Foundation National Patient Registry. Trends in survival outcome were evaluated by the Cox model.
Between 1985 and 1999, mortality fell 61% (95% CI, 36-76) for patients age 2 to 5 years, 70% (60-88) for patients age 6 to 10 years, and 45% (32-66) for patients age 11 to 15 years. Improvements in mortality rates among patients older than 15 years were smaller. Female patients had poorer survival rates than male patients in the age range 2 to 20 years, and this gender gap did not narrow throughout time.
Survival rates of US patients with cystic fibrosis have improved remarkably since 1985. However, most of the improvement was limited to patients 2 to 15 years old. Although both genders benefitted from this trend, female patients have had consistently poorer survival rates than male patients in the age range 2 to 20 years. Further studies are needed to clarify why adult patients with cystic fibrosis had little improvement in survival rates.
Previous studies have shown that female patients with cystic fibrosis (CF) have a significantly poorer prognosis than male patients. Such studies investigating gender-related differences have generally combined data from several centers. The aim of this study was to determine whether with modern aggressive treatment of CF this is still true when care is standardized within a single center.
Retrospective analysis of annual assessment data constructing two cross-sectional studies for the year 1993 (56 female patients, 49 male patients) and 2002 (115 female patients, 94 male patients) and two longitudinal studies, each lasting 5 years, starting in 1993 (21 female patients, 19 male patients) and 1998 (40 female patients, 41 male patients). Outcome measures included mortality, height, and weight SD scores (z scores), and percent predicted for lung function.
In neither cross-sectional study were there significant differences between the sexes for median FEV(1) percent predicted (1993: female patients, 86%; male patients, 84%; 2002: female patients, 93%; male patients, 92%). Female height and weight z scores were at least as good as those of male scores. In the longitudinal studies, there were no clear trends toward declining lung function or growth, but the overall FEV1 percent predicted appeared to be better in female patients than male patients for both cohorts. This was statistically significant for the 1998 cohort (female median FEV1, 91.5% [range, 28 to 134%]; male median FEV1, 84.8% [range, 32 to 145%]; p < 0.05). Female nutritional status was at least as good as male nutritional status, other than the 1998 weight z scores (-0.54 vs -0.21, respectively; p < 0.02). Since 1993, there have been 13 deaths altogether (7 female patients).
During childhood and adolescence, the lung function and nutrition of CF patients should be at least as good in female patients as in male patients. Individual clinic practice should be reviewed if a gender gap persists.
Gender and compliance in the management of a medical regimen for young people with cystic fibrosis. Paper presented at BSA Medical Sociology Conference
- Willis R E Miller
- J Wyn
Miller R, Willis E, Wyn J. Gender and compliance in the management of a medical regimen for young people with cystic fibrosis. Paper presented at BSA Medical Sociology Conference. UK: University of York; 1993.
illness: an application of the Behavior Analytic Model
- M Patterson
M. Patterson et al. / Journal of Cystic Fibrosis 7 (2008) 154–164
illness: an application of the Behavior Analytic Model. J Clin Child
Psychol 1997;26:53–66.
Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis
- M Cory
- L Edwards
- H Sevinson
- M Knowles
Cory M, Edwards L, Sevinson H, Knowles M. Longitudinal analysis of
pulmonary function decline in patients with cystic fibrosis. J Pediatr
1990;131:809-14.
Gender and compliance in the management of a medical regimen for young people with cystic fibrosis
- R Miller
- E Willis
- J Wyn
Miller R, Willis E, Wyn J. Gender and compliance in the management of a
medical regimen for young people with cystic fibrosis. Paper presented at
BSA Medical Sociology Conference. UK: University of York; 1993.
Gender and compliance in the management of a medical regimen for young people with cystic fibrosis
- Miller
Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis
- Cory