Palliative care needs among outpatient adults with cystic fibrosis: Baseline data from the Improving Life with CF trial.

BACKGROUND: Little is known about the burden of illness experienced by people with cystic fibrosis (pwCF) since the advent of CF transmembrane conductance regulator (CFTR) modulator therapies. Studies that characterize the nature of illness burden are needed to inform the development and implementation of palliative care programs that can serve this population and address quality of life concerns. METHODS: Adults with CF treated at five U.S. CF centers were surveyed to obtain baseline data for the Improving Life with CF primary palliative care implementation trial. Consenting patients completed the Integrated Palliative Care Outcome Scale (IPOS), a multidimensional measure of unmet needs for palliative care. Sociodemographic and clinical information was also obtained. The associations among these variables were examined through bivariate and multivariable analyses. RESULTS: Among 256 adults, the most distressing symptoms included not feeling "at peace", communication difficulties with family/friends, anxiety over illness or its treatment, and a lack of energy. In the multivariable analyses, CFTR modulator use was associated with lower IPOS total and physical symptoms scores; female sex and increased hospitalizations were associated with higher scores. Increased age and history of distal intestinal obstructive syndrome were associated with higher IPOS physical symptoms scores. CONCLUSIONS: These findings illuminate the nature of illness burden for pwCF in the era of CFTR modulator therapies. Although illness burden is positively affected by modulator therapy, there is a continuing need for palliative care to address physical, emotional, and spiritual distress, and the communication and practical needs experienced by adults with CF.

Development of a Cystic Fibrosis Primary Palliative Care Intervention: Qualitative Analysis of Patient and Family Caregiver Preferences.

To prevent or mitigate chronic illness burden, people with cystic fibrosis (pwCF) and their family caregivers need primary (generalist-level) palliative care from the time of diagnosis forward. We used qualitative methods to explore their preferences about a screening-and-triage model ("Improving Life with CF") developed to standardize this care. We purposively sampled and interviewed 14 pwCF and caregivers from 5 Improving Life with CF study sites. Thematic analysis was guided by a priori codes using the National Consensus Project's Guidelines for Quality Palliative Care. Participants included 7 adults and 2 adolescents with CF (3 with advanced disease), 4 parents, 1 partner (7 women; 5 people of color). Few were familiar with palliative care. Illness burden was described in multiple domains, including physical (e.g., dyspnea, pain), psychological (e.g., anxiety), and social (e.g., family well-being; impact on work/school). Most preferred survey-based screening with care coordination by the CF team. Preferences for screening approaches varied. PwCF and caregivers experience illness burden and are receptive to a CF-team delivered primary palliative care screening-and-triage model with flexible processes.

Variability in qualifications for principal investigator status in research studies by nurses: A call for clarification.

PURPOSE: To describe existing guidance for qualifications of principal investigator s (PI s) of human subjects research and explore how they are operationalized for pediatric nurse scientists and clinical nurses in children's hospitals. DESIGN AND METHODS: After reviewing federal regulations, accreditation guidelines, and the literature, a convenience sample of members of the National Pediatric Nurse Scientist Collaborative (NPNSC). Participants completed a 33-item survey that included questions about Institutional Review Board (IRB), guidelines, and policies for PI status at their affiliated children's hospitals. RESULTS: The survey was electronically disseminated to 179 members of NPNSC through the Collaborative's listserv. Of the 39 members who responded, 90% hold a PhD and 80% practice in a free-standing children's hospital, nearly all of which (93%) are recognized as Magnet® hospitals. While the majority of respondents indicated that nurse scientists and other nurses were allowed to be PIs of research studies, educational requirements for PI status varied, with 3% requiring a PhD, 15% a baccalaureate degree, and 10% a graduate degree. 54% of respondents reported there was no degree requirement for PI status; however15% reported that even doctorally prepared nurse scientists cannot serve as PIs of research studies at their affiliated children''s hospitals. CONCLUSIONS: The survey identified substantial variability in requirements for PI status and potential barriers to pediatric nurses conducting independent research as PIs at children's hospitals. PRACTICE IMPLICATIONS: Operationalizing existing guidance will expand inclusion of nurse scientist expertise in human subjects research.

"Acquiring the Skills Needed to Communicate What Is in Our Teams' Heart: Love, Compassion and Partnership": Qualitative Analysis of Intact Multidisciplinary Teams' Experience of Relationship-Centered Communication Training.

The Partnership Enhancement Program (PEP) is a 6-hour relationship-centered communication training for intact cystic fibrosis (CF) teams. The aim of this study was to analyze qualitative responses from survey participants regarding their takeaways from the training. A total of 210 professionals participated in 20 pilot workshops at 19 care centers in the United States from November 2018 to December 2019. After the workshop, qualitative feedback was captured by PEP facilitators during a feedback gathering session or submitted immediately in writing by participants. The manuscript team used grounded theory and qualitative methods of coding to identify recurring themes across participant responses. Thematic analysis revealed 5 primary themes and a web of interconnected subthemes. Primary themes include the acquisition of skills to improve communication, strengthened patient/provider connection, improved quality of communication, improved team building, and the ability to change and enhance practice. Participants who completed PEP training endorse acquiring communication skills that increase coproduction of care with patients and caregivers as well as improve relationships across the healthcare system.

Formal vs. informal transition in adolescents with cystic fibrosis: A retrospective comparison of outcomes.

BACKGROUND: The aim of this study was to survey young adults who participated in either a formal or semi-formal transition program at one cystic fibrosis (CF) care center to compare their self-perceived transition related anxiety, transition readiness and satisfaction with transition teaching and timing. METHODS: This retrospective cohort study was conducted from 3/1/2015 to 9/30/2016. Study participants met inclusion criteria if they had a diagnosis of CF, received pediatric care from the care center, transitioned to adult care between 1/1/2009 and 3/1/2016 and had at least six months experience in adult care. Participants completed a 43 question Likert-type survey rating their pre-transfer transition related anxiety, transition readiness, and satisfaction with the transition preparation and process. FINDINGS: Participation in a structured transition program was associated with decreased anxiety at transition time (p < .05), increased transition readiness (p < .01) and increased self-perceived healthcare independence (p < .01). Only 48% of participants were satisfied with their chosen transition time, with 18% wishing they had moved to adult care sooner and 34% wishing they could have delayed their transfer to adult care longer. DISCUSSION: This study supports that participation in a formalized transition program was associated with significantly lower pre-transfer anxiety and higher post-transition satisfaction in individuals with CF. Age at transfer initiation was not associated with satisfaction or perceived readiness to transfer. PRACTICE IMPLICATIONS: Disease-specific knowledge acquisition in transition curriculum does not necessarily correlate to task-completion skills. Teams should partner with young adults to choose the right transition time.

Decreased Wait Time and Increased Satisfaction With Bedside Pancreatic Enzyme Dosing for the Inpatient Adolescent With Cystic Fibrosis: A Quality Improvement Project Comparing Enzyme Self-Administration to Nurse Administration.

For children with cystic fibrosis (CF), enzymes are essential with meals to absorb nutrients and ensure adequate growth. When hospitalized, CF patients typically rely on nurse-administered medications. Recently, a pediatric hospital unit began allowing adolescents with CF enzymes at the bedside. Postimplementation, a satisfaction questionnaire was administered to participating patients and nurses measuring patient and nurse satisfaction with access to bedside enzymes versus nurse administration and overall time for enzyme delivery. The survey utilized a 5-point Likert scale. The wait time for pancreatic enzymes decreased for self-administered enzymes when compared to those that were nurse administered. All (11/11) patients and 86% (12/14) of nurses preferred the self-administration of enzymes. Hospitalized pediatric CF patients and nurses had higher levels of satisfaction with enzyme self-administration. Immediate access to enzymes in room safes impact patient autonomy, reflecting home self-care practices. Decreases in wait times optimize nutritional growth and healing while hospitalized. As a result, a new limited scope policy allowing patient-administered enzymes is now in place in the pediatric inpatient CF unit.

Transition Planning for Chronic Illnesses in the Time of COVID-19.

Transition from pediatric to adult care for those with chronic illnesses must have special considerations during the COVID-19 pandemic. The SARS-CoV-2 coronavirus has significantly disrupted social, economic, and health care practices globally. Young adults with special health care needs are at increased risk for poor outcomes during this unprecedented time. We have found that heightened anxiety, health care service disruption, and other logistical complications surrounding the new virus may further confound health care transitions. Increased communication and collaboration with young adults is necessary to provide patient-centered care and ensure they successfully cross the transition chasm.