Cystic Fibrosis Caregivers Research Articles

A Partial Picture, Without a Frame: Measuring Quality of Life in Cystic Fibrosis Caregivers

ABSTRACT Background Significant advances in the treatment of Cystic Fibrosis (CF) have extended life expectancy, increasing the number of CF caregivers who deliver demanding daily treatment regimens and manage uncertainty. With the impact these changes have on CF caregivers, it is critical to understand CF caregiver quality of life (QoL). Purpose To examine how QoL is measured in CF caregiver research and identify which QoL domains from the World Health Organization (WHO) framework are represented in CF caregiver QoL outcomes. Methods Literature published from 2003 to the present was searched to identify all relevant articles in online academic databases related to measurement of CF caregiver QoL. Results Findings suggest there is inconsistency within QoL measurement with CF caregivers and no existing scale accounts for all aspects of CF caregiver QoL from the WHO’s framework. Discussion The disconnect between how CF caregiver QoL is measured and how it is defined presents a challenge in utilizing information gleaned to best provide targeted and relevant services and resources to CF caregivers. Translation to Health Education Practice: A better understanding of caregiver QoL across all WHO domains will allow development of targeted programs to adequately address challenges for CF caregivers.

A comparison study: caregiver functioning and family resilience among families of children with cystic fibrosis, asthma, and healthy controls

ABSTRACT Caregivers of children with cystic fibrosis (CF) are at risk for psychosocial difficulties, which has been related to poorer child well-being and medical adherence. The present study utilized the National Survey of Children’s Health (NSCH) to examine self-reported differences in caregiver functioning among a matched sample of caregivers of youth with CF, asthma, and healthy controls (N = 114). CF caregivers reported poorer mental health and more parenting aggravation. Among CF caregivers, poorer caregiver mental health predicted less family resilience, which in turn, predicted more parenting aggravation. Findings have implications for prevention screening and brief interventions within pulmonary medicine.

Tobacco smoke exposure in pediatric cystic fibrosis: A qualitative study of clinician and caregiver perspectives on smoking cessation.

Tobacco smoke exposure has negative impacts on the lung health of children with cystic fibrosis (CF), yet evidence-based strategies for smoking cessation have not been tested with or tailored to CF caregivers. This qualitative study identified barriers and facilitators of smoking cessation in this population and outlined potential interventional approaches. We conducted semi-structured interviews with CF familial caregivers who were current or former smokers, and with members of the CF care team. We asked about experiences, practices, and prerequisites for a successful program. Interviews were recorded, transcribed verbatim, and coded by two investigators. Analysis used a thematic approach guided by the PRECEDE model, which identifies predisposing (intrapersonal), reinforcing (interpersonal), and enabling (structural) factors relevant to health behaviors and programs. Seventeen interviews were conducted-eight with familial caregivers and nine with CF team members. Whereas caregivers provided greater insight into internal difficulties and motivators to quit smoking, clinicians offered more extensive input on barriers and solutions related to the clinical environment. Based on study recommendations, a successful tobacco cessation program should include (a) family education about the harms of smoke exposure for children with CF; (b) screening for exposure, ideally with biochemical verification; (c) access to trained tobacco counselors; (d) affordable pharmacotherapy; and (e) outpatient follow-up of those undergoing tobacco treatment. This qualitative study revealed intrapersonal, interpersonal, and structural barriers to eliminating tobacco smoke exposure in children with CF, outlined opportunities to address these barriers, and made recommendations for a comprehensive tobacco cessation strategy.

WS14-4 The underreported benefits of informal peer-to-peer support amongst cystic fibrosis caregivers

WS14-4 The underreported benefits of informal peer-to-peer support amongst cystic fibrosis caregivers

A prospective analysis of unplanned patient-initiated contacts in an adult cystic fibrosis centre

BackgroundTimely response should be provided when patients contact the cystic fibrosis (CF) centre in between scheduled visits. Little data exist on unplanned patient-initiated contacts in CF adults. MethodsA two-stage prospective study was undertaken from 1 January to 31 December 2015 at Cochin Hospital, Paris (France). The first stage included all adults (≥18 years) who initiated unplanned contacts to the CF centre over four months. Four physicians and three nurses systematically recorded unplanned patient-initiated contacts. The data was analysed to determine why and how patients contacted the CF centre and time spent responding to their request(s). The second stage (one physician, three nurses) lasted twelve months and explored whether high contact frequency was associated with disease severity, using multivariate logistic regression. ResultsIn the first stage, 259 of 410 patients (63%) initiated at least one unplanned contact, corresponding to 1067 contacts over 4 months. Patients favoured email with physicians (61% of contacts) and telephone with nurses (87% of contacts). Total time spent by the 7 caregivers on providing responses was 8 h/work week. Reasons for contacting the CF centre varied greatly, but <20% of contacts were directly related to symptom management. In the second stage, 180 of 212 patients (85%) initiated 1876 contacts over 12 months. Factors associated with ≥5 contacts/year were female sex, FEV1 ≤ 30% predicted, ≥5 physician visits/year, and ≥ 1 hospital admission/year. ConclusionsAnswering unplanned patient-initiated contacts represented a significant workload for CF caregivers. Increased disease severity was associated with high contact frequency.

Cystic Fibrosis Foundation Pulmonary Guidelines. Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients with Cystic Fibrosis.

Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators are a new class of medications targeting the underlying defect in CF. Ivacaftor (IVA) and IVA combined with lumacaftor (LUM; IVA/LUM) have been approved by the U.S. Food and Drug Administration (FDA) for use in patients with CF. However, the FDA label for these medications encompasses patient groups that were not studied as part of the drug approval process. CF clinicians, patients, and their families have recognized a need for recommendations to guide the use of these medications. Develop evidence-based guidelines for CFTR modulator therapy in patients with CF. A multidisciplinary committee of CF caregivers and patient representatives was assembled. A methodologist, an epidemiologist, a medical librarian, and a biostatistician were recruited to assist with the literature search, evidence grading, and generation of recommendations. The committee developed clinical questions using the Patient-Intervention-Comparison-Outcome format. A systematic review was conducted to find relevant publications. The evidence was then evaluated using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach, and recommendations were made based on this analysis. For adults and children aged 6 years and older with CF due to gating mutations other than G551D or R117H, the guideline panel made a conditional recommendation for treatment with IVA. For those with the R117H mutation, the guideline panel made a conditional recommendation for treatment with IVA for 1) adults aged 18 years or older, and 2) children aged 6-17 years with a forced expiratory volume in 1 second (FEV1) less than 90% predicted. For those with the R117H mutation, the guideline panel made a conditional recommendation against treatment with IVA for 1) children aged 12-17 years with an FEV1 greater than 90% predicted, and 2) children less than 6 years of age. Among those with two copies of F508del, the guideline panel made a strong recommendation for treatment with IVA/LUM for adults and children aged 12 years and older with an FEV1 less than 90% predicted; and made a conditional recommendation for treatment with IVA/LUM for 1) adults and children aged 12 years or older with an FEV1 greater than 90% predicted, and 2) children aged 6-11 years. Using the GRADE approach, we have made recommendations for the use of CFTR modulators in patients with CF. These recommendations will be of help to CF clinicians, patients, and their families in guiding decisions regarding use of these medications.

Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine

Gastrointestinal disease in Cystic Fibrosis (CF) is caused by defective chloride and bicarbonate transport in intestinal cells leading to reduced intraluminal fluidity, increased mucous viscosity and consequently development of intestinal inflammation, dysbiosis and often times dysmotility. This triad is also referred to as the “CF gut”. A diagnosis is mainly based on clinical observation and treatment is often times decided empirically. This review of the literature should provide CF caregivers with some tools to identify intestinal inflammation, dysbiosis and dysmotility as possible cause for their patient's gastrointestinal complaints and provide an overview of our current approach to its management.

Defining palliative care in cystic fibrosis: A Delphi study

BackgroundThe goal of palliative care is to improve quality of life for people with serious illness. We aimed to create a cystic fibrosis (CF)-specific definition of palliative care. MethodsA working group of 36 CF care providers, researchers, palliative care providers, quality improvement experts, individuals with CF, and CF caregivers completed a series of questionnaires to rate the value of each of 22 attributes of palliative care, rank top attributes to construct definitions of palliative care, and then rate proposed definitions. ResultsAn average of 28 participants completed each of four questionnaires, with consistent distribution of stakeholder roles across questionnaires. Many identified overlaps in routine CF care and palliative care and highlighted the importance of a definition that feels relevant across the lifespan. ConclusionModified Delphi methodology was used to define palliative care in CF. The definition will be used as the foundation for development of CF-specific palliative care guidelines.

Caring for adults with cystic fibrosis

Adults with cystic fibrosis (CF) will soon outnumber children, thus CF is a disease that adult and pediatric physicians must understand. Adults with CF face a very difficult challenge: to have a career and/or a family while still finding the time to keep up the often demanding treatment regimen required to maintain their health in the face of a complicated multisystem disease, such as CF. It is our duty as physicians to help them meet this challenge by providing effective and compassionate care. More and more effective treatments for CF are gradually becoming available that will markedly enhance the quality of life and improve survival for patients with CF. Intelligently integrating these new therapies with existing therapies to minimize treatment burden while maximizing the benefit of these therapies to maintain lung function and prolong life is perhaps the greatest challenge we face as CF caregivers. It is a wonderful challenge to face for CF patients and their caregivers.

Impact of a Child's Chronic Illness on Maternal Sleep and Daytime Functioning

Chronic illness may not only directly impact the sleep of a patient but also indirectly impact the sleep of the family members who provide nighttime care. For parents of children with chronic illnesses, few studies have examined sleep disruptions that may account for elevated rates of depression and fatigue. Our objectives were to examine sleep patterns and causes of sleep disturbances in caregivers of children with and without chronic illnesses and to determine whether sleep mediates the relationship between a child's chronic illness and daytime functioning in caregivers. A total of 118 mothers of children with ventilator dependency (VENT), cystic fibrosis (CF), or healthy children (HEALTHY) completed a series of self-reported measures during a structured telephone interview, including the Pittsburgh Sleep Quality Index, the 24-Hour Sleep Patterns Interview, the Child Health Questionnaire-General Health Scale, the Center for Epidemiological Studies-Depression Scale, and the Iowa Fatigue Scale. Ventilator dependency caregivers reported an earlier morning wake time and shorter total sleep time than both CF and HEALTHY caregivers. In addition, VENT caregivers reported more night wakings and poorer sleep quality than HEALTHY caregivers. Frequent sleep disruptions for VENT caregivers were due to nighttime caregiving, while almost 40% of both VENT and CF caregivers reported sleep disruptions due to stress related to their child's health. Finally, caregiver sleep quality was found to mediate the relationship between child health and caregiver depression and fatigue. Sleep in caregivers of children with chronic illnesses, in particular ventilator dependency, is significantly disrupted, resulting in chronic partial sleep deprivation. Owing to the level of attention and care required by these children, interventions and support for caregivers to improve their sleep quality and quantity are necessary.

Site of care for the treatment of CYSTIC FIBROSIS exacerbations: Does it matter?

Site of care for the treatment of CYSTIC FIBROSIS exacerbations: Does it matter?

Pathways to adult diagnosis of CF: the impact of pre-diagnosis experience on post-diagnosis responses and needs

This phenomenological study explored the pre-diagnosis experiences of 36 men and women who learned during their adult years that they had cystic fibrosis (CF), a disease usually diagnosed in children. Four mutually-exclusive pathways to diagnosis emerged during data analysis, each determined by the extent to which participants experienced symptoms growing up and the extent to which they suspected they had a serious disease. Themes describing the pathways were developed. Pre-diagnosis experiences were found to impact post-diagnosis experiences, as well as individuals’ readiness for education, treatment, and support. Recommendations for CF caregivers, consistent with the expressed wishes of participants, also emerged. Findings revealed that, at least for CF, not all arrive at diagnosis having had similar experiences, suggesting individualized approaches to initiating treatment, education, and support. Although the literature largely conceptualizes chronic illness experience as beginning with diagnosis, this study highlights the pre-diagnosis period as an important focus for additional study.

Lung transplantation and cystic fibrosis: the psychosocial toll.

Lung and heart-lung transplantation have been available as treatment options for cystic fibrosis (CF) since the mid-1980s.1–3 The last decade has seen progress in the understanding of complications such as rejection as well as improvements in surgical and anesthetic techniques that expand the number of CF patients for whom transplantation is an option. As clinical problems such as airway colonization with antibiotic-resistant organisms, sinus disease, and malnutrition yield to continuing research, the potential CF transplant population will increase further. Although it is clear that lung transplantation is of great benefit to some patients with CF, it is also clear that transplantation comes with psychological, social, medical, and financial costs that are different from the burden experienced by patients, families, and CF caregivers in the pretransplant period. Each of the changes from CF patient to CF patient awaiting transplant to CF patient with lung transplant necessitates major alterations in attitudes toward CF care by patients, families, and caregivers. These in turn may result in uncertainty in the areas of control of patient care and continuity. Indeed, our experience leads us to believe that for some CF patients the psychosocial price paid for transplantation coupled with the risks inherent in transplantation together outweigh the potential benefit of the procedure. Patients and families often have the idea that transplantation is uniformly effective and carries a relatively low risk of significant complications, neither of which is the case.3–5 In discussing lung transplantation, Trulock6 has stated, “Lung transplantation is a treatment, not a cure, and it is not a panacea.” A common maxim among transplantation physicians is “Transplantation involves trading one disease for another.” For CF, however, a more correct saying might be “Lung transplantation for CF involves trading part of one disease for an entire second disease.” One trades the lung …

Nutritional assessment and management in cystic fibrosis: a consensus report

This report is a summary of a meeting convened by the Cystic Fibrosis Foundation to develop a consensus among nutrition specialists and cystic fibrosis care givers regarding optimal nutritional management of patients with cystic fibrosis. The first section of the report provides a rationale for emphasizing nutritional management of this genetic disorder. The multiple factors causing malnutrition and a negative energy balance are outlined. The second section provides guidelines for routine assessment of nutrition in these patients. Five categories of nutritional status are defined based on ideal weight for height, age, and gender. These categories are used to formulate a graded response for nutritional intervention. Recommendations are provided for routine dietary supplements, vitamin supplements, and pancreatic enzyme replacement. The primary aim of this report is to educate clinicians as to the importance of frequent assessments and early intervention.

Measurement of hypothetical burnout in cystic fibrosis caregivers.

Three hypothetical phases of staff burnout were measured in a population of cystic fibrosis caregivers and a control population from other areas of specialty pediatrics. The cystic fibrosis caregivers displayed a higher level of emotional exhaustion, lower level of patient depersonalization, and an equal sense of personal accomplishment from the job compared to the controls. We conclude that hypothetical staff burnout can be measured and postulate that these values can be used to quantitate the outcome of anti-burnout programs.