Chest
Cystic Fibrosis Adult Care: Consensus Conference Report
Section snippets
Epidemiology and Survival
Of the 22,301 patients with CF in the 2000 Cystic Fibrosis Foundation Patient Registry Annual Data Report, 8,637 (38.7%) were ≥ 18 years of age.2 This represents a dramatic increase in the number of adults over the past 3 decades, up from about 700 (10% of all CF patients) in 1970 (Fig 1).
These changes are attributable in large part to the significant improvement in survival over the past 30 years. The median predicted survival of only 16 years in 1970 is now up to approximately 32 years. For
Diagnosis
Although CF is usually discovered early in life (70% by the age of 1 year), the diagnosis is being made in adults with increased frequency. Among 22,301 patients in the 2000 CFF Patient Registry, the diagnosis was established at or after the age of 18 in 831 (3.7%).2 Patients diagnosed as adults usually present with chronic respiratory problems. As a group, they have milder lung disease, less pseudomonal infection, and are more likely to be pancreatic-sufficient than patients in whom CF is
Overview
Data comparing the relative effectiveness of various approaches to adult CF care (ie, multidisciplinary vs subspecialty vs primary care-based) are lacking. However, based on the strong association between the establishment of comprehensive CF Care Centers and improved patient outcomes, the committee strongly recommends a multidisciplinary approach modeled on the highly successful pediatric CF care system. The health-care team should include at least a part-time commitment from a physician,
Adolescence
Adolescence is a challenging period, both physically and emotionally, for children and families, even in the absence of chronic illness. The additional challenges of CF may affect normal adolescent development. Similarly, the challenges of adolescence may impact the health of the young person with CF. The pediatric CF team needs to be aware of these challenges, to assess their impact on individual development, and to plan effective interventions that will facilitate the movement of the
Site of Care
Care for teenagers and adults with CF should be provided by personnel who are sensitive and responsive to their medical, developmental, and psychosocial needs. The model of a multidisciplinary team providing care for CF patients has been successful and should be incorporated into the care of adults with CF. Adults generally have more severe pulmonary disease, a higher prevalence of DM, and more complex financial and psychosocial issues. Therefore, a relatively higher intensity of pulmonary,
Definition and Rationale
Obtaining health care in the adult care setting encourages independence and increased self-reliance. Transition should be a planned process over time, as an abrupt transfer to adult care could be unsuccessful. Important meetings and position statements of pediatric and adolescent health professionals in the past decade have brought consensus to the need for both adult care and a smooth transition for young adults with chronic conditions.232233234235236
Models of Transition
National and institutional policies,
Career Planning
Career planning is an important component of the ongoing care provided by the CF center. Ideally, at diagnosis parents should be informed that career planning will be necessary as their child grows older. The CF center team should initiate a discussion of careers with the adolescent patient and may need to assist the patient's school counselor regarding the implications of CF on career planning. The Meyers-Briggs type inventory, a career counseling tool, identifies jobs of interest that a
Employment
According to the 2000 National CF Registry, 2,684 adults with CF (31.0%) were working full time, 867 (10.0%) were working part-time; 1,675 (19.4%) were students, and 299 (3.5%) were homemakers.
Medical Insurance
Obtaining and keeping medical insurance are two of the most important things for the adult with CF. In the United States, 85% of the population obtain heath insurance through their employer, their spouse, or a parent's employer. If possible, changes in employment should be carefully planned to avoid interruption or loss of medical insurance.
Disability Insurance
Benefits for disabled adults with CF may be available under the Social Security Disability Insurance (SSDI), Supplemental Security Income (SSI), Medicaid, and/or Medicare programs.
Fertility
Most men with CF are azoospermic because of anatomic abnormalities of the vas deferens and are functionally sterile, although 1 to 2% may be fertile.249250251 Men in whom CF has been diagnosed in adulthood who have mild mutations are more likely to be fertile.252
In contrast, the woman with CF has a normal reproductive anatomy. It is often stated that women with CF are less fertile than healthy women253254; however, > 100 women with CF become pregnant every year.2 If fertility in women with CF
Bone Disease
Many individuals with CF experience bone and joint disease, including low bone mineral density (BMD). A CFF Consensus Conference on these topics was convened in 2002. Bone disease (ie, osteopenia or osteoporosis) may lead to kyphosis and fractures. These problems occur more commonly in adults and those who have undergone lung transplantation.267268269270271 The prevalence of bone disease in patients with CF depends on the health status of the individual (including severity of lung disease and
End-of-Life Options
Despite substantial therapeutic advances, CF remains uniformly fatal, and little has been written about end-of-life care for patients and their families.298299 Barriers to optimum end-of-life care for these patients include the following:
- 1.
Difficulty predicting the timing of death. The most widely quoted predictive model300 has been called into question.144301302
- 2.
The influence of lung transplantation on decisions about end-of-life care.
- 3.
Personal fears or lack of confidence have prompted many
Appendix: Participants
This list reflects current positions held by participants as of October 2002.
Moira L. Aitken, MD
Director, Adult CF Clinic
Associate Professor
Pulmonary & Critical Care Division
University of Washington
Seattle, WA
Robert Aris, MD
Associate Professor
University of North Carolina at Chapel Hill
Chapel Hill, NC
John D. Armstrong II, MD
Professor and Associate Director
Health Care Ethics, Humanities & Law Program
University of Colorado Health Sciences Center
Denver, CO
Robert J. Beall, PhD
President and CEO
Cystic
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This work was sponsored by the Cystic Fibrosis Foundation, Bethesda, MD. Bruce C. Marshall, MD, was recruited as Director of Clinical Affairs, Cystic Fibrosis Foundation/Cystic Fibrosis Foundation Therapeutics in July 2002.
Each author has stated that he/she has no pertinent involvement in any organization with a direct financial interest in the subject of this manuscript.
- †
Co-first authors.