Evidence Based Practice

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Cystic Fibrosis

Scott Powers & Susana Patton, with help from Molly Heidemann, & Racquel Henry

Bartholomew, L. K., Czyzewski, D. I., Parcel, G. S., Swank, P. R., Sockrider, M. M., Mariotto, M. J. et al. (1997). Self-management of cystic fibrosis: Short-term outcomes of the Cystic Fibrosis Family Education Program. Health Education and Behavior, 24, 652-666.

Cheuvront, B., Sorenson, J. R., Callanan, N. P., Stearns, S. C., & DeVellis, B. M. (1998). Psychosocial and educational outcomes associated with home- and clinic-based pretest education and cystic fibrosis carrier testing among a population of at-risk relatives. American Journal of Medical Genetics, 75, 461-468.

de Jong, P. T., Grevink, R. G., Roorda, R. J., Kaptein, A. A., & van der Schans, G. P. (1994). Effect of a home exercise training program in patients with cystic fibrosis. Chest, 5, 463-468.

Delk, K. K., Gevirtz, R., Hicks, D. A., Carden, F., & Rucker, R. (1994). The effects of biofeedback assisted breathing retraining on lung functions in patients with cystic fibrosis. Chest, 105, 23-28.

Goldbeck, L., & Babka, C. (2001). Development and evaluation of a multi-family psychoeducational program for cystic fibrosis. Patient Education & Counseling, 44, 187-192.

Grasso, M. C., Button, B. M., Allison, D. J., & Sawyer, S. M. (2000). Benefits of music therapy as an adjunct to chest physiotherapy in infants and toddlers with cystic fibrosis. Pediatric Pulmonology, 29, 371-381.

Gulmans, V. A., de Meer, K., Brackel, H. J., Faber, J. A., Berger, R., & Helders, P. J. (1999). Outpatient exercise training in children with cystic fibrosis: physiological effects, perceived competence, and acceptability. Pediatric Pulmonology, 28, 39-46.

Hains, A., Behrens, D., Biller, J., & Davies, W. (1997). Cognitive behavioral interventions for adolescents with cystic fibrosis. Journal of Pediatric Psychology, 22, 669-687.

Hernandez-Reif, M., Field, T., Krasnegor, J., Martinez, E., Schwartzman, M., & Mavunda, K. (1999). Children with cystic fibrosis benefit from massage therapy. Journal of Pediatric Psychology, 24, 175-181.

Ireys, H. T., Chernoff, R., DeVet, K. A., & Kim, Y. (2001). Maternal outcomes of a randomized controlled trial of a community-based support program for families of children with chronic illnesses. Archives of Pediatric and Adolescent Medicine, 155, 771-777.

McIlwaine, P. M., Wong, L. T., Peacock, D., & Davidson, A. G. (2001). Long-term comparative trial of positive expiratory pressure versus oscillating positive expiratory pressure (flutter) physiotherapy in the treatment of cystic fibrosis. Journal of Pediatrics, 138, 845-850.

Quan, J. M., Tiddens, H. A., Sy, J. P., McKenzie, S.G., Montgomery, M. D., Robinson, P. J., et al. (2001). The Pulmozyme Early Intervention Trial Study Group. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. Journal of Pediatrics, 139, 813-820.

Quittner, A. L., & Buu, A. (2002). Effects of tobramycin solution for inhalation on global ratings of quality of life in patients with cystic fibrosis and pseudomonas aeruginosa infection. Pediatric Pulmonology, 33, 269-276.

Schneiderman-Walker, J., Pollock, S. L., Corey, M., Wilkes, D. D., Canny, G. J., Pedder, L. et al. (2000). A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. Journal of Pediatrics, 136, 304-310.

Selvadurai, H. C., Blimkie, C. J., Meyers, N., Mellis, C. M., Cooper, P. J., & Van Asperen, P. P. (2002). Randomized controlled study of in-hospital exercise training programs in children with cystic fibrosis. Pediatric Pulmonology, 33, 194-200.

Stark, L. J., Opripari, L.C., Spieth, L.E., Jelalian, E., Quitner, A.Q., Higgins, L., Mackner, L., Byars, K., Lapey, A., Stallings, V.A., & Duggan, C. (2003). Contribution of behavior therapy to dietary treatment in cystic fibrosis: A randomized controlled study with a 2-year follow-up. Behavior Therapy, 34, 237-258.

Wolter, J. M., Bowler, S. D., Nolan, P. J., & McCormack, J. G. (1997). Home intravenous therapy in cystic fibrosis: A prospective randomized trial examining clinical, quality of life and cost aspects. European Respiratory Journal, 10, 896-900.

Addendum (Medication Trials):

Bucuvalas, J. C., Chernausek, S. D., Alfaro, M. P., Krug, S. K., Ritschel, W., & Wilmott, R. W. (2001). Effect of insulinlike growth factor-1 treatment in children with cystic fibrosis. Journal of Pediatric Gastroenterology & Nutrition, 33, 576-581.

Campbell, P. W. (2000). Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. New England Journal of Medicine, 342, 851-859.

Furuya, M. E., Lezana-Fernandez, J. L., Vargas, M. H., Hernandez-Sierra, J. F., & Ramirez-Figueroa, J. L. (2001). Efficacy of human recombinant DNase in pediatric patients with cystic fibrosis. Archives of Medical Research, 32, 30-34.

Hardin, D. S., Ellis, K. J., Dyson, M., Rice. J., McConnell, R., & Seilheimer, D. K. (2001). Growth hormone improves clinical status in prepubertal children with cystic fibrosis: Results of a randomized controlled trial. Journal of Pediatrics, 139, 636-642.

Hutler, M., Schnabel, D., Staab, D., Tacke, A., Wahn, U. & Boning, D. (2002). Effect of growth hormone on exercise tolerance in children with cystic fibrosis. Medicine & Science in Sports & Exercise, 34, 567-572.

Konstan, M. W., Byard, P. J., Hoppel, C. L., & Davis, P. B. (1995). Effect of high-dose ibuprofen in patients with cystic fibrosis. New England Journal of Medicine, 332, 848-854.

Lai, H. C., FitzSimmons, S. C., Allen, D. B., Kosorok, M. R., Rosenstein, B. J., & Campbell, P.W. (2000). Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. New England Journal of Medicine, 342, 851-859.

Marchand, V., Baker, S. S., Stark, T. J., & Baker, R. D. (2000). Randomized, double-blind, placebo-controlled pilot trial of megestrol acetate in malnourished children with cystic fibrosis. Journal of Pediatric Gastroenterology Nutrition, 31, 264-269.

Moss, R. B. (2002). Long-term benefits of inhaled tobramycin in adolescent patients with cystic fibrosis. Chest, 121, 55-63.

Pike, S. E., Prasad, S. A., & Balfour-Lynn, I. M. (2001). Effect of intravenous antibiotics on exercise tolerance (3-min step test) in cystic fibrosis. Pediatric Pulmonology, 32, 38-43.

Ramsey, B. W., Pepe, M. S., Quan, J. M., Otto, K. L., Montgomery, A. B., Williams- Warren, J. et al. (1999). Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group. New England Journal of Medicine, 340, 23-30.

Steinkamp, G., Demmelmair, H., Ruhl-Bagheri, I., von der Hardt, H., & Koletzko, B. (2000). Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients. Journal of Pediatric Gastroenterology Nutrition, 31, 418-23.

Suri, R., Metcalfe, C., Lees, B., Grieve, R., Flather, M., Normand, C. et al. (2001). Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: a randomized trial. Lancet, 358, 1316-1321.