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Dr. Oermann is Assistant Professor of Pediatrics, Baylor College of Medicine and is affiliated with the Pediatric Pulmonary Department, Texas Children’s Hospital, Feigin Center.
Correspondence to: Christopher M. Oermann, MD, Pediatric Pulmonary, TCH Feigin Center, Suite 410, 6621 Fannin MC 3–2571, Houston, TX; e-mail: coermann{at}bcm.tmc.edu
The life expectancy for individuals with cystic fibrosis (CF) has dramatically improved over the past 50 years.1 The median length of survival has increased from roughly 1 year in 1950 to > 30 years today. Children born with CF today have an estimated life expectancy of approximately 40 years.2 There are multiple factors that have contributed to this progress. The development of pancreatic enzyme replacements to treat malabsorption and of effective antipseudomonal antibiotics to suppress airway infection has been crucial. The consolidation of care at Cystic Fibrosis Care Centers sponsored by the US Cystic Fibrosis Foundation, early diagnosis, aggressive disease management, and a host of new therapeutic interventions also have been integral in enhancing quality of life and increasing life expectancy.
The increasing longevity of CF patients has resulted in marked changes
in the epidemiology of CF and an aging CF population.3
The
most recent Cystic Fibrosis Foundation Patient Registry report
indicates a significant increase in the percentage of CF patients
classified as adults (
18 years), with the total percentage rising
from 29.5% in 1988 to 37% in 1998.4
As a result of
aging, CF patients are developing a number of CF-related complications
as well as "adult" medical problems (eg, reproductive
issues) not routinely treated by pediatric pulmonologists. The Cystic
Fibrosis Foundation has long recognized the need for internal
medicine-trained pulmonologists to care for the growing number of adult
CF patients and has recommended, in a 1990 consensus document, the
incorporation of adult-care specialists in center programs, the
establishment of inpatient services in internal medicine wards, and the
creation of transition teams or parallel adult-care
teams.5
Clearly, there is a strong and growing need for
adult-care providers in the nationwide network of CF Care Centers.
Adult CF patients are at increased risk for a number of CF-related complications.6 7 These include CF-related diabetes, ototoxicity and nephrotoxicity due to repeated therapy with aminoglycoside antibiotics, the development of multiresistant Pseudomonas aeruginosa and other organisms, liver disease, osteoporosis and arthropathy, pneumothorax, and major hemoptysis.4 Other CF-related disorders prevalent in the older CF population include a variety of GI and genitourinary problems. Additionally, older CF patients encounter the same types of ailments as age-matched peers. Thus, as McCallum and colleagues point out in this issue of CHEST (see page 1059), CF is now a disease of the adult population with many adult-specific issues. As such, adult CF patients must be treated by an interdisciplinary team of adult-care providers within the environment of the expanding CF Care Center network.
Infertility has long been recognized among men and women with CF.8 Men are almost universally infertile due to the congenital bilateral absence of the vas deferens (CBAVD) with resultant obstructive azoospermia, whereas women show normal anatomy but thick, tenacious cervical mucus that fails to thin during menses.9 Although the specific reasons for these findings are unclear, it may relate to the degree of expression of the CF transmembrane conductance regulator protein in reproductive tissues.10 As the life expectancy for CF patients has risen and greater numbers of patients are reaching adulthood, the issues of fertility and reproductive health have become increasingly important.
The myriad reproductive health and fertility issues facing male and female CF patients have been addressed in numerous publications.9 11 12 Additionally, a substantial body of medical literature addresses the issue of fertility and the approach to infertility in the general population. McCallum and colleagues now provide a timely discussion of male infertility and an update on the current surgical approaches to male infertility in patients with CF. They review their experience with eight couples in which the male partner was infertile due to CBAVD, providing detailed clinical information and therapeutic results (including pregnancy outcomes) of sperm retrieval and intracytoplasmic sperm injection.
This article provides important information for all clinicians caring for adolescent and adult CF patients. Its publication in CHEST represents a growing awareness of the importance of CF among adult-care providers and the significance of the exchange of information across disciplines in medical care.
References
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