Male gender and unemployment are associated with lower levels of perceived social support in adults with cystic fibrosis.

Objective: Adults with cystic fibrosis (CF) face unique challenges with regard to the attainment and maintenance of social support. Although social support has been shown to improve treatment outcomes in other patient-populations, research on social support in adults with CF is limited. In fact, no studies have examined factors associated with less perceived social support in this population.

Social support is associated with fewer reported symptoms and decreased treatment burden in adults with cystic fibrosis.

Background: Although social support has been linked to a variety of health outcomes in those with and without chronic illness, this construct has rarely been studied in adults with cystic fibrosis (CF), who may face a unique set of clinical and psychosocial barriers. The current study explored the relationships between social support, mental health, physical health, treatment activity, and disease-specific quality of life in a sample of adults with CF.

Methods: Participants in the study included 250 adults with CF who took part in a larger longitudinal study.

Death after cessation of treatment by cystic fibrosis patients: An international survey of clinicians.

Background: Little is known about cystic fibrosis patients, who are not considered to be terminally ill, and who die after voluntary cessation of treatment.

Aim: This study was undertaken to provide an international snapshot of this issue.

Design: An online survey was distributed across three continents.

Motivating adherence among adolescents with cystic fibrosis: youth and parent perspectives.

As advances in the care of individuals with cystic fibrosis (CF) have resulted in improved survival, therapeutic regimens for treatment of CF have become increasingly complex. This high treatment burden poses challenges to chronic disease self-management, particularly amongst adolescents. The aim of this qualitative study was to understand the barriers and facilitators of adherence to chronic CF therapies as perceived by adolescents with CF and their parents.

Longitudinal trends in health-related quality of life in adults with cystic fibrosis.

Background: Health-related quality-of-life (HRQOL) measures have been used as patient-reported outcomes in clinical trials in cystic fibrosis (CF), but there are limited data on HRQOL changes over time in adults with CF.

Methods: The Project on Adult Care in Cystic Fibrosis, a prospective, longitudinal panel study of 333 adults with CF at 10 CF centers in the United States, administered a disease-specific HRQOL measure, the Cystic Fibrosis Questionnaire-Revised (CFQ-R), seven times over 21 months. The CFQ-R assesses both physical and psychosocial domains of health.

Watching Boston Med.

The author reflects on the ABC news documentary series Boston Med--both what it achieved, and what it could have achieved.

Palliation of dyspnea in pediatrics.

Dyspnea is a complex physiologic and psychosocial symptom that is difficult to characterize and measure, but essential to treat, as it has a significant effect on quality of life. Although palliation of dyspnea in the child with chronic illness is an under-researched area, children and their families cannot wait for the research to catch up with their current needs. This article addresses several aspects of dyspnea in pediatrics palliative care, with an eye toward practical suggestions for evaluation and management.

Associations between illness perceptions and health-related quality of life in adults with cystic fibrosis.

Objective: The objective of this work was to examine the relationship between illness perception, health status, and health-related quality of life (HRQOL) in a cohort of adults with cystic fibrosis (CF).

Methods: In the Project on Adult Care in Cystic Fibrosis, we administered five subscales (Illness Consequences, Illness Coherence, Illness Timeline--Cyclical, Personal Control, and Treatment Control) of the Illness Perception Questionnaire--Revised (IPQ-R). Multivariable linear regression analyses explored the associations between illness perception, health status, symptom burden, and physical and psychosocial HRQOL, as measured by various domains of the Cystic Fibrosis Questionnaire--Revised (CFQ-R).

NIH Consensus Development Conference statement: inhaled nitric-oxide therapy for premature infants.

Premature birth is a major public health problem in the United States and internationally. Infants born at or before 32 weeks' gestation (2% of all births in the United States in 2007) are at extremely high risk for death in the neonatal period or for pulmonary, visual, and neurodevelopmental morbidities with lifelong consequences including bronchopulmonary dysplasia, retinopathy of prematurity, and brain injury. Risks for adverse outcomes increase with decreasing gestational age.

Research use of leftover newborn bloodspots: attitudes of Canadian geneticists regarding storage and informed consent requirements.

Purpose: Leftover newborn spots can provide a powerful research tool as a population-wide DNA bank. Some provinces/states store them for more than 20 years; however, parents are usually not informed of the retention of leftover newborn spots. To examine the opinions of Canadian geneticists regarding permission for leftover newborn spots storage for research purposes and the associated risks, a web-based survey was distributed to all members of the Canadian College of Medical Geneticists with a valid e-mail address (n = 209) and completed by 78 respondents (37%).

Lung transplantation for cystic fibrosis.

Lung transplantation is a complex, high-risk, potentially life-saving therapy for the end-stage lung disease of cystic fibrosis (CF). The decision to pursue transplantation involves comparing the likelihood of survival with and without transplantation as well as assessing the effect of wait-listing and transplantation on the patient's quality of life. Although recent population-based analyses of the US lung allocation system for the CF population have raised controversies about the survival benefits of transplantation, studies from the United Kingdom and Canada have suggested a definite survival advantage for those receiving transplants.

Frequency and self-management of pain, dyspnea, and cough in cystic fibrosis.

Cystic fibrosis (CF) has been transformed from a fatal diagnosis in infancy to a chronic disease of children and young adults. Symptom patterns and disease burden in CF may be shifting to reflect the relatively healthier, older population with the disease. Self-management of symptoms is a hallmark of chronic illness, and yet we do not have a good understanding of how CF patients monitor or manage their symptoms.

Palliative and end-of-life care in cystic fibrosis: what we know and what we need to know.

Purpose Of Review: This review will examine what is now known about palliative and end-of-life care for cystic fibrosis (CF) patients, including the changing demographics and context of such care, and then outline a brief research agenda to guide further work in this area.

Recent Findings: We have now entered an era of more invasive end-of-life care in CF, due in part to changes in the demographics of death in CF and in part to the wider pursuit of lung transplantation. Recent single-center studies suggest that lung transplant listing changes the location and intensity of end-of-life care and complicates end-of-life care decision-making for the patient, family, and clinician.

Advance care planning in adults with cystic fibrosis.

Background: Because many patients with cystic fibrosis (CF) continue to survive into adulthood, discussion of end-of-life care decisions between clinicians and patients becomes a crucial part of CF adult care. Advance care planning (ACP) promotes alignment of patient care at the end of life with an individual's goals, however minimal research exists on ACP in CF.

Methods: We surveyed adults enrolled in the Project on Adult Care in Cystic Fibrosis (PAC-CF).

High treatment burden in adults with cystic fibrosis: challenges to disease self-management.

Background: More aggressive management of cystic fibrosis (CF), along with the use of new therapies, has led to increasing survival. Thus, the recommended daily treatment regimens for most CF adults are complex and time consuming.

Methods: In the Project on Adult Care in CF (PAC-CF), an ongoing longitudinal study of CF adults, we assessed self-reported daily treatment activities and perceived treatment burden as measured by the CF Questionnaire-Revised (CFQ-R), a disease-specific quality of life measure.

Reasons for participation in pain research: can they indicate a lack of informed consent?

Objective: To ascertain the self-reported reasons for participation in the clinical research of chronic low back pain and to evaluate those reasons in the context of informed consent and the concept of therapeutic misconception. This is the belief that research participation is equivalent to clinical care.

Design: Qualitative descriptive study with semistructured interviews.

Self-reported physical and psychological symptom burden in adults with cystic fibrosis.

Symptom burden is a key component of health-related quality of life in patients with cystic fibrosis (CF). To examine symptom prevalence and characteristics of adults with CF, we administered the Memorial Symptom Assessment Scale (MSAS), a previously validated measure of symptom burden, to CF patients enrolled in the Project on Adult Care in CF. The mean age of the 303 respondents (response rate 91%) was 32.

Self-reported involvement of family members in the care of adults with CF.

Background: Few empirical studies have examined the role of family caregivers in the lives of adults with CF.

Methods: As part of the Project on Adult Care in CF (PAC-CF), an on-going prospective, longitudinal panel study of adults with CF, 119 family members and friends of adults with CF completed a mail survey in which they reported the frequency of help they provide for their family member with CF during routine care, hospitalization, and home IV treatment.

Results: The 119 caregivers were mainly spouses or unmarried partners (56%) and parents (29%).

Chronic ventilator need in the community: a 2005 pediatric census of Massachusetts.

Objectives: The purpose of this study was to describe the population of children with chronic mechanical ventilation in Massachusetts and their patterns of medical care.

Patients And Methods: Investigators surveyed all of the Massachusetts home ventilator clinics, pediatric pulmonary services, hospital-based pediatric services for special health care needs, insurers, home care vendors, nursing agencies, the Massachusetts Department of Public Health, selected individual providers, and rehabilitation and long-term care facilities providing services to children with chronic respiratory support needs. Support was defined as daily use of noninvasive, negative-pressure, or invasive/transtracheal ventilators.

Integrating palliative care into chronic care for children with severe neurodevelopmental disabilities.

Background: Children with severe neurodevelopmental disabilities and complex medical conditions are a growing and unique segment of the pediatric population. The increasing use of life sustaining technologies has provided the chance at an extended life and increasing inclusion within the broader community. Families work to overcome personal and professional biases, clinical uncertainties, and pragmatic obstacles to improve quality of life.

Role of brain death and the dead-donor rule in the ethics of organ transplantation.

The "dead-donor rule" requires patients to be declared dead before the removal of life-sustaining organs for transplantation. The concept of brain death was developed, in part, to allow patients with devastating neurologic injury to be declared dead before the occurrence of cardiopulmonary arrest. Brain death is essential to current practices of organ retrieval because it legitimates organ removal from bodies that continue to have circulation and respiration, thereby avoiding ischemic injury to the organs.