Adult Care Clinical Care Guidelines

These clinical care guidelines from the Cystic Fibrosis Foundation provide recommendations on how best to care for adults with CF.

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Cystic Fibrosis Adult Care: Consensus Conference Report Executive Summary
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

Recommendations Evaluation of the Evidence

1. Diagnosis

Diagnosis guidelines were published in 2017.

STANDARD COMPREHENSIVE CARE

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.

  1. Consensus Conference
3. Pulmonary Disease: Evaluation: Sputum Culture
  1. Complete microbiological assessment (including antibiotic susceptibility testing) of expectorated sputum should be performed at least annually but preferably quarterly.
  2. The use of throat swabs has not been validated in adults.
  3. Microbiology laboratories should follow published guidelines for the processing of CF sputum.
  4. Cultures for nontuberculous mycobacteria (NTM) should be performed annually.
  5. Confirmed or suspected B. cepacia isolates should be sent to the CF Foundation-sponsored reference laboratory at the University of Michigan.
  1. Consensus Conference
  2. Consensus Conference
  3. Consensus Conference
  4. Management of Nontuberculous Mycobacteria in Individuals With Cystic Fibrosis guidelines were published in 2016.
  5. Consensus Conference
4. Pulmonary Disease: Evaluation: Imaging
  1. Posterior/anterior and lateral chest X-rays should be obtained every 2-4 years in patients with stable clinical status.
  2. Imaging should be considered for patients with signs or symptoms consistent with an acute pulmonary exacerbation, pneumothorax, lobar atelectasis, or hemoptysis.
  1. Consensus Conference
  2. Pneumothorax and Hemoptysis guidelines were published in 2010.
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
  1. Patients with moderate to severe lung function should be screened for hypoxemia associated with exercise or during sleep.
  2. Oxygen is indicated if:
  3. Exercise oxygen saturation falls below 88 to 90 percent, or
  4. Nocturnal oxygen saturation is <88 to 90 percent for more than 10 percent of total sleep time.
  1. Consensus Conference
  2. Consensus Conference
10. Pulmonary Disease: Management of Complications
  1. Hemoptysis and Pneumothorax
  2. Hepatobiliary Disease
  3. GI Complications
  1. Hemoptysis and Pneumothorax guidelines were published in 2010.
  2. Hepatobiliary Disease guidelines were published in 1998.
  3. Best practices on gastrointestinal aspects of CF were published in 2017.

TRANSITION FROM PEDIATRIC TO ADULT CARE

Recommendations Evaluation of the Evidence
11. Criteria for Successful Transition
  1. Successful transition requires effective communication between pediatric and adult care teams.
  2. A coordinator can help to ensure smooth transition.
  1. Consensus Conference
  2. Consensus Conference
12. Timing
  1. The concept of transition should be introduced early.
  2. Adolescents should gradually be given more responsibility for self-care and decision-making, health education, and training and should be seen alone during clinic visits.
  3. Formal transfer of care typically coincides with graduation from high school but should be individualized to patients.
  4. Strategies to promote smooth transition in the adult care clinic include:
  • Thorough understanding of the patient prior to transfer
  • Inviting parents to the first visit in adult care
  • Introducing any changes in medical treatment gradually
  1. Consensus Conference
  2. Consensus Conference
  3. Consensus Conference
  4. Consensus Conference

EDUCATION AND CAREERS

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

  1. The CF center can assist with information regarding financial assistance.
  2. Students should inquire about local CF care when away from home.
  3. No entity that receives federal funds can discriminate against a person on the basis of a disability (Section 504 of the Rehabilitation Act of 1973).
  4. The CF center may need to provide documentation of the student's disability and need for specific accommodations.
  1. Consensus Conference
  2. Consensus Conference
  3. Consensus Conference
  4. Consensus Conference

EMPLOYMENT

Recommendations Evaluation of the Evidence
15. Protection From Discrimination
  1. Title I of the Americans with Disabilities Act (ADA) prohibits discrimination against a qualified individual with a disability in regard to all terms, conditions, and privileges of employment if the employer employs >15 employees.
  2. The ADA also prohibits discrimination based on a relationship or association with an individual with a disability.
  1. Consensus Conference
  2. Consensus Conference
16. Accommodations for Disability
  1. Under the ADA, people with CF may be entitled to reasonable accommodation if needed.
  2. Reasonable accommodations include:
  • Additional sick time
  • Modification of work schedule
  • Ability to work from home
  • Modification of policies and procedures of the employer
  1. Consensus Conference
  2. Consensus Conference
17. Informing an Employer of a CF Diagnosis
  1. Prospective employers may not ask whether an applicant has a disability during an interview or on an application.
  2. After obtaining a job, the decision to disclose a medical diagnosis is best made by the individual.
  3. The CF center can assist in providing information about CF to the employer.
  1. Consensus Conference
  2. Consensus Conference
  3. Consensus Conference

FAMILY PLANNING AND PREGNANCY

Recommendations Evaluation of the Evidence
18. Fertility/Contraception
  1. Men who desire fertility should undergo semen analysis.
  2. Genetic counseling and CF carrier screening should be offered to individuals with CF and their partners who are contemplating family planning.
  3. Patients should also be counseled regarding the potential effects of parenting on the health of the CF patient and the possibility of early maternal/paternal mortality.
  4. Women with severe lung disease should be advised that panel-reactive antibodies may be increased during pregnancy and some centers consider this a contraindication to lung transplantation.
  1. Consensus Conference
  2. Consensus Conference
  3. Consensus Conference
  4. Consensus Conference
19. Pregnancy
  1. Thresholds of medical contraindication to pregnancy have not been established.
  2. Normal changes that occur during pregnancy may adversely affect the woman with CF.
  3. Good outcomes have been reported in patients with non-severe lung disease.
  1. Consensus Conference
  2. Consensus Conference
  3. Consensus Conference

BONE AND JOINT DISEASE

Recommendations Evaluation of the Evidence
20. Bone and Joint Disease Bone Health and Disease guidelines were published in 2005.

END OF LIFE OPTIONS

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  
Recommendations Evaluation of the Evidence
25. Lung Transplantation Consensus recommendations on lung transplantation in CF were published in 1998.

Future Directions and 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.

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 April 2019 and it was determined that no update is needed at this time.

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