Models of Palliative Care Delivery for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Consensus Guidelines

Dio Kavalieratos, Anna M Georgiopoulos, Lara Dhingra, Melissa J Basile, Elliot Rabinowitz, Sarah E Hempstead, Albert Faro, Elisabeth P Dellon, Dio Kavalieratos, Anna M Georgiopoulos, Lara Dhingra, Melissa J Basile, Elliot Rabinowitz, Sarah E Hempstead, Albert Faro, Elisabeth P Dellon

Abstract

Cystic fibrosis (CF) affects more than 70,000 individuals and their families worldwide. Although outcomes for individuals with CF continue to improve, it remains a life-limiting condition with no cure. Individuals with CF manage extensive symptom and treatment burdens and face complex medical decisions throughout the illness course. Although palliative care has been shown to reduce suffering by alleviating illness-related burdens for people with serious illness and their families, little is known regarding the components and structure of various delivery models of palliative care needed to improve outcomes for people affected by CF. The Cystic Fibrosis Foundation (CFF) assembled an expert panel of clinicians, researchers, individuals with CF, and family caregivers, to develop consensus recommendations for models of best practices for palliative care in CF. Eleven statements were developed based on a systematic literature review and expert opinion, and address primary palliative care, specialty palliative care, and screening for palliative needs. These recommendations are intended to comprehensively address palliative care needs and improve quality of life for individuals with CF at all stages of illness and development, and their caregivers.

Keywords: clinical guidelines; cystic fibrosis; healthcare delivery; palliative care; patient-reported outcomes.

Conflict of interest statement

Dr. Dellon reports grants from Cystic Fibrosis Foundation, during the conduct of the study; Dr. Georgiopoulos reports nonfinancial support from Cystic Fibrosis Foundation, during the conduct of the study; grants, personal fees, and nonfinancial support from Cystic Fibrosis Foundation; and grants and personal fees from Vertex Pharmaceuticals, Inc., outside the submitted work. Dr. Goss reports grants from Cystic Fibrosis Foundation, grants from European Commission, grants from NIH (NHLBI), grants from NIH (NIDDK and NCRR), during the conduct of the study; personal fees from Gilead Sciences, personal fees from Novartis, grants from NIH, grants from FDA, other from Boehringer Ingelheim, and personal fees and nonfinancial support from Vertex, outside the submitted work. Mr. Hovater reports having received honoraria for other work with the Cystic Fibrosis Foundation unrelated to this article, outside the submitted work; Dr. McKinzie reports personal fees from Vertex Pharmaceuticals, Inc., outside the submitted work; Dr. Rabinowitz reports grants from Cystic Fibrosis Foundation First and Second Year Clinical Fellowship Training Grant Institutional Training Grant, RABI NO 1680, outside the submitted work. None of the work presented in this opinion piece was influenced by the funding sources noted above. The funding sources that support other ongoing research played no role in writing this article, or in the decision to submit for publication.

Figures

FIG. 1.
FIG. 1.
Integrating primary and specialty palliative care to address palliative needs throughout the CF experience. Palliative care is delivered continuously throughout the illness experience, from diagnosis through bereavement. The intensity of palliative care should match the patient and family's palliative needs, including but not limited to: assessment and management of physical and emotional symptoms; existential and spiritual suffering; provision of information regarding prognosis and treatment options, elicitation of goals of care, and enactment of advance care planning; and support during transplant evaluation and post-transplantation. Palliative care is ideally a partnership, whereby CF care team members provide continuous first-line monitoring and support for palliative needs (“primary palliative care”), while palliative care specialists support the CF care team with expert management of complex or severe concerns. Palliative care needs are illustrated as the variable white band throughout the center of the figure; the width and opacity of the band indicate the fluctuating severity of palliative needs. Importantly, this figure is a crude depiction of palliative needs, which will vary by patient/family; as such, CF care teams should continuously monitor suffering and needs to customize palliative care for each patient. Furthermore, this illustration does not specifically depict certain milestones, such as the transplantation process, which may also affect palliative needs. Color image is available online.
FIG. 2.
FIG. 2.
Palliative care needs assessment strategy for people with CF and their caregivers. Color image is available online.

References

    1. Cystic Fibrosis Foundation: Cystic Fibrosis Foundation Patient Registry 2018 Annual Data Report. Bethesda, MD, 2019
    1. Marmor M, Jonas A, Mirza A, et al. : Opportunities to improve utilization of palliative care among adults with cystic fibrosis: A systematic review. J Pain Symptom Manage 2019;58:1100–1112 e1101.
    1. Trandel ET, Pilewski JM, Dellon EP, et al. : Prevalence of unmet palliative care needs in adults with cystic fibrosis. J Cyst Fibros 2020;19:394–401
    1. Morrison RS, Meier DE: Palliative care. N Engl J Med 2004;350:2582–2590
    1. Quill TE, Abernethy AP: Generalist plus specialist palliative care—Creating a more sustainable model. N Engl J Med 2013;368:1173–1175
    1. Lanken PN, Terry PB, Delisser HM, et al. : An official American Thoracic Society clinical policy statement: Palliative care for patients with respiratory diseases and critical illnesses. Am J Respir Crit Care Med 2008;177:912–927
    1. Sawicki GS, Dill EJ, Asher D, et al. : Advance care planning in adults with cystic fibrosis. J Palliat Med 2008;11:1135–1141
    1. Dellon EP, Basile M, Hobler MR, et al. : Primary palliative care skills in CF: Perspectives of adults with CF, caregivers, and CF care team members. Pediatr Pulmonol 2018;53:410–411
    1. Chen E, Killeen KM, Peterson SJ, et al. : Evaluation of pain, dyspnea, and goals of care among adults with cystic fibrosis: A Comprehensive Palliative Care Survey. Am J Hosp Palliat Care 2017;34:347–352
    1. Trandel ET, Kavalieratos D, Basile M, et al. : Palliative care skills in CF: Perspectives of adults with CF, caregivers, and CF care team members. Pediatr Pulmonol 2020;55:2017–2024
    1. Dellon EP, Goggin J, Chen E, et al. : Defining palliative care in cystic fibrosis: A Delphi study. J Cyst Fibros 2018;17:416–421
    1. Smith TJ, Temin S, Alesi ER, et al. : American Society of Clinical Oncology provisional clinical opinion: The integration of palliative care into standard oncology care. J Clin Oncol 2012;30:880–887
    1. Ferrell BR, Temel JS, Temin S, et al. : Integration of palliative care into standard oncology care: American Society of Clinical Oncology Clinical Practice Guideline Update. J Clin Oncol 2017;35:96–112
    1. Ferrell BR, Twaddle ML, Melnick A, Meier DE: National Consensus Project Clinical Practice Guidelines for Quality Palliative Care Guidelines, 4th Edition. J Palliat Med 2018;21:1684–1689
    1. Aslakson R, Dy SM, Wilson RF, et al. : AHRQ Comparative Effectiveness Technical Briefs. Assessment Tools for Palliative Care. Rockville, MD: Agency for Healthcare Research and Quality, 2017
    1. Harris PA, Taylor R, Thielke R, et al. : Research electronic data capture (REDCap)—A metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inf 2009;42:377–381
    1. Kavalieratos D, Gelfman LP, Tycon LE, et al. : Palliative care in heart failure: Rationale, evidence, and future priorities. J Am Coll Cardiol 2017;70:1919–1930
    1. Kavalieratos D, Corbelli J, Zhang D, et al. : Association between palliative care and patient and caregiver outcomes: A systematic review and meta-analysis. JAMA 2016;316:2104–2114
    1. Dhingra L, Walker P, Berdella M, et al. : Addressing the burden of illness in adults with cystic fibrosis with screening and triage: An early intervention model of palliative care. J Cyst Fibros 2020;19:262–270
    1. Friedman D, Linnemann RW, Altstein LL, et al. : Effects of a primary palliative care intervention on quality of life and mental health in cystic fibrosis. Pediatr Pulmonol 2019;54:984–992
    1. Friedman D, Linnemann RW, Altstein LL, et al. : The CF-CARES primary palliative care model: A CF-specific structured assessment of symptoms, distress, and coping. J Cyst Fibros 2018;17:71–77
    1. Linnemann RW, O'Malley PJ, Friedman D, et al. : Development and evaluation of a palliative care curriculum for cystic fibrosis healthcare providers. J Cyst Fibros 2016;15:90–95
    1. Goggin J, Cohen RI: CF healthcare workers feel unprepared in providing suitable end of life care and desire more education: Results of a nationwide survey. J Cyst Fibros 2016;15:85–89
    1. Gelfman LP, Kavalieratos D, Teuteberg WG, et al. : Primary palliative care for heart failure: What is it? How do we implement it? Heart Fail Rev 2017;22:611–620
    1. Grimley ME, Dellon EP, Yorke D: Health care providers need education and support to provide palliative care to children with CF. Pediatr Pulmonol 2013;48:425–426
    1. Yankaskas JR, Marshall BC, Sufian B, et al. : Cystic fibrosis adult care: Consensus conference report. Chest 2004;125(1 Suppl):1s–39s
    1. Harrington SE, Rogers E, Davis M: Palliative care and chronic obstructive pulmonary disease: Where the lines meet. Curr Opin Pulmon Med 2017;23:154–160
    1. Dellon EP, Shores MD, Nelson KI, et al. : Caregiver perspectives on discussions about the use of intensive treatments in cystic fibrosis. J Pain Symptom Manage 2010;40:821–828
    1. Dellon EP, Chen E, Goggin J, et al. : Advance care planning in cystic fibrosis: Current practices, challenges, and opportunities. J Cyst Fibros 2016;15:96–101
    1. Chen E, Homa K, Goggin J, et al. : End-of-life practice patterns at U.S. adult cystic fibrosis care centers: A national retrospective chart review. J Cyst Fibros 2017. [Epub ahead of print]; DOI: 10.1016/j.jcf.2017.08.010
    1. Hobler MR, Engelberg RA, Curtis JR, et al. : Exploring opportunities for primary outpatient palliative care for adults with cystic fibrosis: A Mixed-Methods Study of Patients' Needs. J Palliat Med 2018;21:513–521
    1. Karlekar M, Doherty KE, Guyer D, Slovis B: Integration of palliative care into the routine care of cystic fibrosis patients. Palliat Med 2015;29:282–283
    1. Jabbarian LJ, Zwakman M, van der Heide A, et al. : Advance care planning for patients with chronic respiratory diseases: A systematic review of preferences and practices. Thorax 2018;73:222–230
    1. Kazmerski TM, Weiner DJ, Matisko J, et al. : Advance care planning in adolescents with cystic fibrosis: A quality improvement project. Pediatr Pulmonol 2016;51:1304–1310
    1. Wentlandt K, Weiss A, O'Connor E, Kaya E: Palliative and end of life care in solid organ transplantation. Am J Transplant 2017;17:3008–3019
    1. Rosenberger EM, Dew MA, DiMartini AF, et al. : Psychosocial issues facing lung transplant candidates, recipients and family caregivers. Thorac Surg Clin 2012;22:517–529
    1. Collins FS: Realizing the dream of molecularly targeted therapies for cystic fibrosis. N Engl J Med 2019;381:1863–1865
    1. Back AL, Arnold RM, Quill TE: Hope for the best, and prepare for the worst. Ann Intern Med 2003;138:439–443
    1. Faro A, Tallarico E: 2020. Personal communication
    1. Dellon EP, Shores MD, Nelson KI, et al. : Family caregiver perspectives on symptoms and treatments for patients dying from complications of cystic fibrosis. J Pain Symptom Manage 2010;40:829–837
    1. Kumar P, Wright AA, Hatfield LA, et al. : Family perspectives on hospice care experiences of patients with cancer. J Clin Oncol 2017;35:432.
    1. Taylor DH Jr, Ostermann J, Van Houtven CH, et al. : What length of hospice use maximizes reduction in medical expenditures near death in the US Medicare program? Soc Sci Med 2007;65:1466–1478
    1. Aldridge Carlson MD, Barry CL, Cherlin EJ, et al. : Hospices' enrollment policies may contribute to underuse of hospice care in the United States. Health Affairs 2012;31:2690–2698
    1. Colman RE, Curtis JR, Nelson JE, et al. : Barriers to optimal palliative care of lung transplant candidates. Chest 2013;143:736–743
    1. McGuffie K, Sellers DE, Sawicki GS, Robinson WM: Self-reported involvement of family members in the care of adults with CF. J Cyst Fibros 2008;7:95–101
    1. Ernst MM, Johnson MC, Stark LJ: Developmental and psychosocial issues in cystic fibrosis. Pediatr Clin N Am 2011;58:865–885, x.
    1. Quittner AL, Goldbeck L, Abbott J, et al. : Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: Results of The International Depression Epidemiological Study across nine countries. Thorax 2014;69:1090–1097
    1. Glajchen M, Kornblith A, Homel P, et al. : Development of a brief assessment scale for caregivers of the medically ill. J Pain Symptom Manage 2005;29:245–254
    1. Kamal AH, Wolf SP, Troy J, et al. : Policy changes key to promoting sustainability and growth of The Specialty Palliative Care Workforce. Health Aff (Millwood) 2019;38:910–918
    1. Wentlandt K, Dall'Osto A, Freeman N, et al. : The Transplant Palliative Care Clinic: An early palliative care model for patients in a transplant program. Clin Transplant 2016;30:1591–1596
    1. Smyth AR, Bell SC, Bojcin S, et al. : European Cystic Fibrosis Society Standards of Care: Best Practice guidelines. J Cyst Fibros 2014;13 Suppl 1:S23–S42
    1. Colman R, Singer LG, Barua R, Downar J: Characteristics, interventions, and outcomes of lung transplant recipients co-managed with palliative care. J Palliat Med 2015;18:266–269
    1. Dellon EP, Shores MD, Nelson KI, et al. : Caregivers' perspectives on decision making about lung transplantation in cystic fibrosis. Progr Transpl 2009;19:318–325
    1. Bourke SJ, Mackley R, Booth Z, et al. : A prospective cohort study of integrated palliative care of cystic fibrosis (CF). Thorax 2015;70:A193
    1. Kavalieratos D, Moreines LT, Hoydich ZP, et al. : Embedded specialty palliative care is feasible, acceptable, and perceived to be effective in cystic fibrosis: Results of a Pilot Randomized Clinical Trial. J Pain Symptom Manage 2019;57:396
    1. Gandesbery B, Dobbie K, Gorodeski EZ: Outpatient Palliative Cardiology Service embedded within a heart failure clinic: Experiences with an emerging model of care. Am J Hosp Palliat Care 2018;35:635–639
    1. Ferrell BR, Temel JS, Temin S, Smith TJ: Integration of palliative care into Standard Oncology Care: ASCO Clinical Practice Guideline Update Summary. J Oncol Pract 2017;13:119–121
    1. Estrada-Veras J, Groninger H: Palliative care for patients with cystic fibrosis #265. J Palliat Med 2013;16:446–447
    1. Bourke S, Anderson A, Doe SJ, et al. : Palliative care as part of the cystic fibrosis team. Pediatr Pulmonol 2014;49:428
    1. Collins ES, Witt J, Bausewein C, et al. : A systematic review of the use of the Palliative Care Outcome Scale and the Support Team Assessment Schedule in Palliative Care. J Pain Symptom Manage 2015;50:842–853.e819.
    1. Quittner AL, Sawicki GS, McMullen A, et al. : Psychometric evaluation of the Cystic Fibrosis Questionnaire-Revised in a national sample. Qual Life Res 2012;21:1267–1278
    1. Quittner AL, Schechter MS, Rasouliyan L, et al. : Impact of socioeconomic status, race, and ethnicity on quality of life in patients with cystic fibrosis in the United States. Chest 2010;137:642–650
    1. Sole A, Perez I, Vazquez I, et al. : Patient-reported symptoms and functioning as indicators of mortality in advanced cystic fibrosis: A new tool for referral and selection for lung transplantation. J Heart Lung Transplant 2016;35:789–794
    1. Kroenke K, Spitzer RL, Williams JB: The PHQ-9: Validity of a brief depression severity measure. J Gen Intern Med 2001;16:606–613
    1. Spitzer RL, Kroenke K, Williams JB, Lowe B: A brief measure for assessing generalized anxiety disorder: the GAD-7. Arch Intern Med 2006;166:1092–1097
    1. Quittner AL, Abbott J, Georgiopoulos AM, et al. : International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety. Thorax 2016;71:26–34
    1. Sharma RK, Astrow AB, Texeira K, Sulmasy DP: The Spiritual Needs Assessment for Patients (SNAP): development and validation of a comprehensive instrument to assess unmet spiritual needs. J Pain Symptom Manage 2012;44:44–51
    1. Dellon EP, Helms SW, Hailey CE, et al. : Exploring knowledge and perceptions of palliative care to inform integration of palliative care education into cystic fibrosis care. Pediatr Pulmonol 2018;53:1218–1224
    1. Dellon EP, Basile M, Hobler MR, et al. : Palliative care needs of individuals with cystic fibrosis: Perspectives of multiple stakeholders. J Palliat Med 2020;23:957–963
    1. Quittner AL, Buu A, Messer MA, et al. : Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis. Chest 2005;128:2347–2354
    1. Alpern AN, Brumback LC, Ratjen F, et al. : Initial evaluation of the Parent Cystic Fibrosis Questionnaire—Revised (CFQ-R) in infants and young children. J Cyst Fibros 2015;14:403–411
    1. Brumback LC, Baines A, Ratjen F, et al. : Pulmonary exacerbations and parent-reported outcomes in children <6 years with cystic fibrosis. Pediatr Pulmonol 2015;50:236–243
    1. Wojtaszczyk A, Glajchen M, Portenoy RK, et al. : Trajectories of caregiver burden in families of adult cystic fibrosis patients. Palliat Support Care 2018;16:732–740
    1. Balzano J, Fresenius A, Walker P, et al. : Web-based symptom screening in cystic fibrosis patients: A feasibility study. J Cyst Fibros 2016;15:102–108
    1. Tsai WI, Kuo SC, Wen FH, et al. : Prolonged grief disorder and depression are distinct for caregivers across their first bereavement year. Psychooncology 2018;27:1027–1034

Source: PubMed

Patrocinadores e Colaboradores

Condições médicas

Intervenções de drogas

3
Se inscrever