Advance Care Planning in Adults with Cystic Fibrosis

Abstract

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). We assessed experiences with ACP processes and communication and sought to identify factors associated with completion of an advance directive. Results: The mean age of respondents (n ϭ 234) was 34 years and the mean forced expiratory volume in 1 second (FEV 1 ) was 64% predicted. Seventy-four percent reported that they had spoken to someone, generally a family member, about the care they would want if they became too ill to make decisions for themselves. However, only 30% reported completing an advance directive. Although 79% reported feeling comfortable talking to their clinician about ACP, only 28% said that their CF clinicians have asked about ACP. Having specific wishes about treatment decisions (odds ratio [OR] 7.8, 95% confidence interval [CI] 1.9-32.1) and reporting that a clinician had discussed ACP (OR 4.4, 95% CI 1.5-12.6) were significantly associated with reporting the completion of an advance directive. Discussion: Though the majority of adults with CF report thinking about and communicating with family about advance care wishes, only a minority report completing an advance directive. Few adults with CF report being asked about ACP by their clinicians. Formulating specific wishes and discussing ACP with a clinician are strongly associated with completing an advance directive. Efforts to improve clinician communication with CF adults around ACP are needed to ensure that discussion of advance directives becomes an integral component of adult CF care.

Palliative Care for Patients Dying in the Intensive Care Unit with Chronic Lung Disease Compared with Metastatic Cancer

“…In fact, patients with COPD may experience more severe dyspnea and poorer functional status during a longer and more protracted course of illness than patients with lung cancer (29). Although patients with chronic lung disease face as much, or in some cases more, symptom burden, longer hospital stays, and lower levels of satisfaction of care than patients with cancer (29-32), few discuss their treatment preferences with their physicians or implement advance care planning (7,33,34). These studies document inadequate palliative care before ICU admission for patients with ILD or COPD, and our study documents that these shortcomings continue in the ICU setting.…”

Caregiver Perspectives on Discussions About the Use of Intensive Treatments in Cystic Fibrosis

“…13,14 Advance care planning to foster alignment of medical care with individual patients’ goals and preferences may not be routinely included in the care of patients with advanced CF 13 despite recommendations for its early incorporation into routine CF care. 14,15 Little is known, though, about actual practice and whether it corresponds to these recommendations.…”

Perspectives of adolescent girls with cystic fibrosis and parents on disease‐specific sexual and reproductive health education

“…Adolescent and young adult women with CF report that SRH is rarely discussed as part of their routine healthcare . Similarly, patient‐provider communication around other important, yet sensitive, health topics, such as advance care planning and prognosis, has been shown to be lacking in CF care …”