Adult Care Clinical Care Guidelines
Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic Fibrosis Adult Care: Consensus Conference Report. Chest. 2004 Jan; 125(1 Suppl):1S-39S.
Improved care has led to a steady increase in the number of adults with cystic fibrosis. The Cystic Fibrosis Foundation Patient Registry demonstrates that, in 2014, the number of adults with CF first exceeded the number of children with CF. With continued therapeutic advances, CF survival continues to improve, and the proportion of adults with CF increases.
Recognizing the unique needs of adults with CF, the CF Foundation convened a consensus conference in 1999 to address care standards for adults with CF. Their findings and recommendations were reported in a consensus conference report. Since that report, there have been many clinical advances and other CF clinical care guideline reports.
CF Foundation-sponsored benchmarking studies of high-performing adult and pediatric CF programs found that patient outcomes were more closely related to care systems, attitudes, practices, patient/family involvement, and improvement projects than to any specific care elements (Boyle MP et al. 2014).
In the Adult Care Consensus Conference Report, we summarize the following topics. CF Foundation guidelines that supersede this report are cited.
- Diagnosis of CF
- Standard care of:
- Pulmonary disease
- Hepatobiliary disease
- Gastrointestinal (GI) complications
- Transition to adulthood
- Family planning and pregnancy
- Bone and joint disease
- End-of-life care
Methodology
In June 1999, the CF Foundation convened a consensus conference to address care of adults with CF. The consensus conference report summarizes the findings of that meeting and incorporates additional data gathered from 1999-2002. All these recommendations were based on consensus opinions. Other CF Foundation and European Cystic Fibrosis Society (ECFS) guidelines are cited in the pertinent sections.
Recommendations
Diagnosis |
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Recommendations | Evaluation of the Evidence |
1. Diagnosis |
Diagnosis guidelines were published in 2017. |
Standard Comprehensive Care |
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Recommendations | Evaluation of the Evidence |
2. Multidisciplinary approach modeled on the pediatric system and provided by a team with training and experience in adult CF care. |
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3. Pulmonary Disease: Evaluation: Sputum Culture
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4. Pulmonary Disease: Evaluation: Imaging
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5. Pulmonary Disease: Management: Pulmonary Exacerbations | Treatment of Pulmonary Exacerbations guidelines were published in 2009. |
6. Pulmonary Disease: Management: Chronic Suppressive Antibiotic Therapy | Chronic Medications for Maintenance of Lung Health guidelines were published in 2013. |
7. Pulmonary Disease: Management: Airway Clearance and Exercise | Airway Clearance Therapies guidelines were published in 2009. |
8. Pulmonary Disease: Management: Mucolytic Agents | Chronic Medications for Maintenance of Lung Health guidelines were published in 2013. |
9. Pulmonary Disease: Management: Oxygen
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10. Pulmonary Disease: Management of Complications
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Transition From Pediatric to Adult Care |
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Recommendations | Evaluation of the Evidence |
11. Criteria for Successful Transition
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12. Timing
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Education and Careers |
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Recommendations | Evaluation of the Evidence |
13. Career Planning The CF center should support patients with career development through initiation of a dialogue about career planning and assisting the patients and their school counselors with regard to the implications of CF on careers and education. |
Consensus Conference |
14. Postsecondary Education
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Employment |
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Recommendations | Evaluation of the Evidence |
15. Protection From Discrimination
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16. Accommodations for Disability
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17. Informing an Employer of a CF Diagnosis
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Family Planning and Pregnancy |
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Recommendations | Evaluation of the Evidence |
18. Fertility/Contraception
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19. Pregnancy
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Bone and Joint Disease |
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Recommendations | Evaluation of the Evidence |
20. Bone and Joint Disease | Bone Health and Disease guidelines were published in 2005. |
End of Life Options |
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Recommendations | Evaluation of the Evidence |
21. Advanced care planning should begin early in the course of a patient's illnesses. | Consensus Conference |
22. Structured discussion and documentation should occur to ensure that a patient's wishes will be respected. | Consensus Conference |
23. Patients should clarify their personal goals and the care they would like to receive in various situations as well as identify the person or persons whom they would like to make health care decisions on their behalf. | Consensus Conference |
24. Palliative care may be beneficial for multidisciplinary management of symptoms that may cause suffering; efforts to maximize quality of life as defined by the patient and family; care provider education; treatment of psychological, social, and spiritual distress; and loss, grief, and bereavement support. | Consensus Conference |
Lung Transplantation |
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Recommendations | Evaluation of the Evidence |
25. Lung Transplantation | Consensus recommendations on lung transplantation in CF were published in 1998. |
Unanswered Questions
- The impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy is unknown. Guidelines for the use of these medications have been published, including in older ages and therapy duration.
- The mechanisms of pulmonary exacerbations and their best detection and management are being determined.
- Comprehensive medical insurance coverage and availability to all patients must be developed.
- Provisions are needed for family support during medical treatments and should be sought at the individual centers.
- Detection and treatment of age-related complications, including diabetes, bone disease, cancer, depression, anxiety, and advanced care planning, need further definition.
Further Reading
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Boyle MP, Sabadosa KA, Quinton HB, Marshall BC, Schechter MS. Key Findings of the US Cystic Fibrosis Foundation's clinical practice benchmarking project. BMJ Quality Safety. 2014 23:i15-i22. doi: 10.1136/bmjqs-2013-002369.
Farrell PM, White TB, Ren CL, et al. Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. J of Pediatr. 2017 181:S4-S15.e1. doi: 10.1016/j.jpeds.2016.09.064.
Floto RA, Olivier KN, Saiman L, et al. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of nontuberculous mycobacteria in individuals with cystic fibrosis. Thorax. 2016 Jan; 71(1):88-90. doi: 10.1136/thoraxjnl-2015-207983.
Flume PA, Mogayzel PJ, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Resp Crit Care Med. 2009 Nov 1;180(9):802-8. doi: 10.1164/rccm.200812-1845PP. Epub 2009 Sep 3.
Mogayzel PJ, Naureckas ET, Robinson KA, et al. Cystic Fibrosis Pulmonary Guidelines: Chronic Medications for Maintenance of Lung Health. Am J Resp Crit Care Med. 2013 Apr 1; 187(7):680-9.
Flume PA, Robinson KA, O'Sullivan BP, et al. Cystic Fibrosis Pulmonary Guidelines: Airway Clearance Therapies. Respir Care. 2009 Apr; 54(4):522-37.
Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized trial. JAMA. 2003 290:1749-1756.
Flume PA, Mogayzel PJ, Robinson KA, et al. Cystic Fibrosis Pulmonary Guidelines: Pulmonary Complications: Hemoptysis and Pneumothorax. Am J Respir Crit Care Med. 2010 Aug 1; 182(3):298-306. doi: 10.1164/rccm.201002-0157CI.
Borowitz DS, Grant RJ, Durie PR. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosis colonopathy. J Pediatr. 1995 Nov; 127:681-4.
Stallings VA, Stark LJ, Robinson KA. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008 May; 108(5):832-9. doi: 10.1016/j.jada.2008.02.020.
Schwarzenberg SJ, Hempstead SE, McDonald CM, et al. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J Cyst Fibros. 2016 Nov; 15(6):724-735. doi: 10.1016/j.jcf.2016.08.004. Epub 2016 Sep 3.
Moran A, Brunzell C, Cohen RC, et al. Clinical Care Guidelines for Cystic Fibrosis-Related Diabetes. Diabetes Care. 2010 Dec; 33(12):2697-708. doi: 10.2337/dc10-1768.
Sokol R, Durie PR. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. Recommendations for management of liver and biliary tract disease in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1999 28:S1-S13.
Hadjiliadis D, Khoruts A, Zauber AG, et al. Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendations. Gastroenterology. 2018 Feb; 154(3):736-745.e14. doi: 10.1053/j.gastro.2017.12.012. Epub 2017 Dec 29.
Aris RM, Merkel PA, Bachrach LK, et al. Guide to Bone Health and Disease in Cystic Fibrosis. J Clin Endocrinol Metab. 2005 Mar; 90(3):1888-96. Epub 2004 Dec 21.
Yankaskas JR, Mallory GB. Lung Transplantation in Cystic Fibrosis: Consensus Conference Statement. Chest. 1998 Jan; 113(1):217-26.
Use of These Guidelines
The CF Foundation intends for this executive summary of its guideline to summarize the published guideline. The published guideline summarizes evidence, and provides reasonable clinical recommendations based on that evidence, to clinicians, patients, and other stakeholders. Care decisions regarding individual patients should be made using a combination of these recommendations, the associated benefit-risk assessment of treatment options from the clinical team, the patient's individual and unique circumstances, as well as the goals and preferences of the patients and families that the team serves, as a part of shared decision-making between the patient and clinician.
This executive summary was prepared by:
Holly Keyt, MD, (University of Texas at San Antonio) and James R. Yankaskas, MD, (University of North Carolina at Chapel Hill)
The guidelines were published in January 2004, they were reviewed in July 2021 and it was determined that no update is needed at this time.