Cystic fibrosis (CF) is an inherited disease of the mucus glands that affects many body systems. In particular, the disorder causes progressive damage to the respiratory system and results in chronic digestive problems. Many of the issues occur because of mucus, the slippery substance that lubricates and protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. In people with cystic fibrosis, the body produces mucus that is abnormally thick and sticky. This abnormal mucus can obstruct the airways, leading to severe problems with breathing and bacterial infections in the lungs. The infections cause chronic coughing, wheezing, and inflammation. Over time, mucus buildup and infections result in permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs.

Despite the many problems presented by CF, progress is being made in helping patients live and cope with the disease. An illustration of a new approach was reported by Christian and D’Auria in a recent study. The aim of their study was to test the effectiveness of an intervention to improve psychosocial adjustment, functional health, and physiological health in children (8-12 years of age) with CF by teaching them life skills for managing their chronic illness in their everyday lives. An two-group, repeated-measures design was used to compare 116 children with CF randomly assigned to intervention and usual care groups. Assessment occurred at baseline and at 3, 6, and 9 months post-intervention. Children received an individual, tailored intervention during a home visit and a structured group intervention. Four instruments were used to measure psychosocial health status, and functional health status was measured with the Functional Disability Inventory for Children. Physiological status was determined by pulmonary function testing and physical growth. The results indicated that compared with children in the usual care group, the participants in the intervention groups demonstrated decreased perceived impact of illness and decreased loneliness. Improvements were maintained at 9 months for impact of illness and loneliness.

COMMENTS: Christian and D’Auria concluded that the developmentally appropriate, problem-solving, and social skills intervention has promise for decreasing the social consequences of chronic illness (CF) in children's lives. We concur. Although it was not designed as a self-management intervention per se, there are elements from these programs, such as problem solving, that are used in both approaches to CF. A number of promising changes have occurred in the management of CF in the past decade or so. The biggest advance has undoubtedly been the steadily increasing average length of life experienced by patients with the disease. It has been creeping up because of both medical and behavioral changes. Many patients with CF are followed at regional centers established for the disease. The variability of success in increasing life at these centers was the subject of a fascinating article by Atul Gawande, now reprinted in his book, BETTER. Gawande reported how treatment success for CF fell along a bell-shaped curve with outstanding facilities at one end of the spectrum and less successful programs falling at the other end of the spectrum. This was, by itself, a major finding. However, the real hope may be that Gawande later found that facilities later improved their performance with excellent facilities leading the way. As a center’s ranking can be found online, it has prompted all centers to improve their performance. This is great news for those who have CF as they now have the promise of longer and better lives.

B.J. Christian & J.P. D'Auria. Building life skills for children with cystic fibrosis: effectiveness of an intervention. Nursing Research, 2006; 55: 300-307.

A. Gawande. Better: a surgeon’s notes on performance. New York: Metropolitan Books, 2007.