Elisabeth P. Dellon

Barriers to hospice for children as perceived by hospice organizations in North Carolina.

Despite improving organization of hospice for children with life-limiting illnesses, services may be underutilized. We asked representatives of all 76 existing North Carolina hospice organizations about barriers to serving children. Representatives of 61 agencies responded (80%). Hospices serving children differed from hospices not serving children on perception of barriers: 1) Lack of pediatric trained staff (8% vs 42%, p = 0.01); 2) lack of pediatrician consultation (23% vs 50%, p = 0.03); 3) lack of pediatric pharmacy (4% vs 32%, p = 0.006), and inconsistent plan of care between pediatrician and hospice (12% vs 47%, p = 0.01). Lack of pediatric referrals (78%) and families wanting to continue curative therapies while receiving hospice care (77%) were felt to be the most important barriers overall. Enhanced training of pediatric providers and a model of care which blends disease-specific treatment with hospice may improve access to hospice services for children.

Advance care planning in cystic fibrosis: Current practices, challenges, and opportunities.

BACKGROUND Studies in cystic fibrosis (CF) report late attention to advance care planning (ACP). The purpose of this study was to examine ACP with patients receiving care at US adult CF care programs. METHODS Chart abstraction was used to examine ACP with adults with CF dying from respiratory failure between 2011 and 2013. RESULTS We reviewed 210 deaths among 67 CF care programs. Median age at death was 29 years (range 18-73). Median FEV1 in the year preceding death was 33% predicted (range 13-100%); 68% had severe lung disease with FEV1<40% predicted. ACP was documented for 129 (61%), often during hospitalization (61%). Those with ACP had earlier documentation of treatment preferences, before the last month of life (73% v. 35%; p=<0.01). Advance directives were completed by 93% of those with ACP versus 75% without (p<0.01); DNR orders and health care proxy designation occurred more often for those with ACP. Patients awaiting lung transplant had similar rates of ACP as those who were not (67% v. 61%; p=0.55). The frequency of ACP varied significantly among the 29 programs contributing data from four or more deaths. CONCLUSIONS ACP in CF often occurs late in the disease course. Important decisions default to surrogates when opportunities for ACP are missed. Provision of ACP varies significantly among adult CF care programs. Careful evaluation of opportunities to enhance ACP and implementation of recommended approaches may lead to better practices in this important aspect of CF care.

Physician Practices for Communicating With Patients With Cystic Fibrosis About the Use of Noninvasive and Invasive Mechanical Ventilation

BACKGROUND Many patients with advanced cystic fibrosis (CF) lung disease receive intensive treatments such as noninvasive and invasive mechanical ventilation for respiratory failure after little or no communication with physicians. METHODS Using surveys and follow-up interviews, physicians at two major CF care centers reported their practices for discussing intensive treatment preferences with patients with CF and about barriers and facilitators to communication. RESULTS Surveys were completed by 30 (88%) and 26 (76%) of 34 eligible CF physicians who provide care for children (60%), adults (23%), or both (17%). Respondents described variable timing and content of discussions. They identified patient/family factors such as denial of disease severity, optimistic expectations of treatment outcomes, inability of ill patients to participate in discussions, and family disagreements about treatments as primary barriers to discussions. They also acknowledged physician factors, including concern for taking away hope and uncertainty about when to address treatment preferences. Patient/family factors were also the most common facilitators identified, particularly disease severity and inquiry about intensive treatments. They recommended: (1) developing standards for communication, (2) offering training in communication for physicians, (3) creating decision support tools for patients and families, and (4) using the multidisciplinary CF care team to facilitate communication. CONCLUSIONS CF physicians describe numerous patient/family factors barriers to communicating about intensive treatments for respiratory failure. They recommend changing physician and organizational factors to improve practice and promote effective communication. Innovation in clinical training, team roles, and decision support may prompt changes in practice standards.

Vaccine induced Hepatitis A and B protection in children at risk for cystic fibrosis associated liver disease

OBJECTIVES Hepatitis A (HAV) and Hepatitis B (HBV) infections can cause serious morbidity in patients with liver disease, including cystic fibrosis associated liver disease (CFALD). HAV and HBV vaccinations are recommended in CFALD, and maintenance of detectable antibody levels is also recommended with chronic liver disease. A better understanding of factors predicting low HAV and HBV antibodies may help physicians improve protection from these viruses in CFALD patients. METHODS We examined HAV and HBV vaccine protection in children at risk for CFALD. Clinical and vaccine histories were reviewed, and HAV and HBV antibody titers measured. Those with no vaccination history or low HAV or HBV titers received primary or booster vaccinations, and responses were measured. RESULTS Thirty-four of 308 children were at risk for CFALD per project criteria. Ten had previous HAV vaccination, of which 90% had positive anti-HAV antibodies. Thirty-three of 34 had previously received primary HBV vaccination (most in infancy), but only 12 (35%) had adequate anti-HBs levels (≥10mIU/mL). Children with adequate anti-HBs levels were older at first HBV vaccine (median 2.3 vs. 0.1 years, p<0.01), and at final HBV vaccine (median 4.0 vs. 0.8 years, p=0.01). Fourteen of 19 (74%) responded to HBV boosters. Z-scores for BMI at HBV booster were significantly lower in booster non-responders (p=0.04). CONCLUSIONS Children at increased risk of CFALD have inadequate HAV and HBV antibody levels, and HBV antibody protection can be enhanced through vaccine boosters. HBV antibody titers should be assessed in CFALD patients with a history of vaccination, particularly in those who received HBV vaccines in infancy or who are malnourished.

Patient and Provider Perspectives on Communication About Body Image With Adolescents and Young Adults With Cystic Fibrosis

Objective This mixed-methods study examined perspectives of adolescents and young adults (AYAs) with cystic fibrosis (CF) and health care providers on body image communication. Methods Interviews and questionnaires were completed by 20 AYAs and 28 providers. Results Although 85% of patients reported they had never had a body image conversation with a health care provider, 74% of providers reported discussing this topic with patients. Patients and providers described body image as an important issue, which should be discussed comfortably and supportively. However, patients often preferred to discuss body image as a distinct topic, separate from physical health, whereas providers preferred integrating body image conversations within weight- and health-based discussions. Conclusions Body image is an important topic for AYAs with CF that often goes unaddressed or addressed in ways that are less preferred by patients. Providers should reduce barriers to effective communication about this important topic, particularly through increased awareness of AYA preferences.

Highlights from the 2015 North American Cystic Fibrosis Conference

The 29th Annual North American Cystic Fibrosis Conference was held in Phoenix, Arizona on October 8–10, 2015. Abstracts were published in a supplement to Pediatric Pulmonology.1 In this review, we summarize presentations in several of the topic areas addressed at the conference. Our goal is to provide an overview of presentations with relevance to emerging or changing concepts in several areas rather than a comprehensive review. Citations from the conference are by first author and abstract number or symposium number, as designated in the supplement. Pediatr Pulmonol. 2016;51:650–657.

Highlights from the 2017 North American Cystic Fibrosis Conference: 2017 NORTH AMERICAN CYSTIC FIBROSIS CONFERENCE

The 31st annual North American Cystic Fibrosis Conference (NACFC) was held in Indianapolis, IN on November 2–4, 2017. Abstracts of presentations from the conference were published in a supplement to Pediatric Pulmonology [2017; Pediatr Pulmonol Suppl. 52: S1‐S776]. The current review summarizes several major topic areas addressed at the conference: the pathophysiology and basic science of cystic fibrosis (CF) lung disease, clinical trials, clinical management issues, and quality improvement (QI). In this review, we describe emerging concepts in several areas of CF research and care.

Difficult conversations: Discussing prognosis with children with cystic fibrosis

Background Despite the chronic, progressive, and life‐threatening nature of cystic fibrosis (CF), there are no guidelines for when and how to communicate prognosis to children with CF.

Defining palliative care in cystic fibrosis: A Delphi study

BACKGROUND The 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. METHODS A 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. RESULTS An 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. CONCLUSION Modified 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.

Exploring knowledge and perceptions of palliative care to inform integration of palliative care education into cystic fibrosis care

Individuals with cystic fibrosis (CF) face the challenges of managing a chronic, progressive disease. While palliative care is a standard of care in serious illnesses, there are no guidelines for its incorporation into CF care. Patients with CF, caregivers, and CF care providers may lack knowledge about palliative care and perceive barriers to integrated care.