title: Developmental and psychosocial issues in cystic fibrosis.
abstract: Cystic fibrosis (CF) is a multisystemic life-limiting genetic disorder, primarily affecting respiratory functioning. Most patients with CF are diagnosed by 2 years of age, and the current median predicted survival rate is 37.4 years old, with 95% of patients dying from complications related to pulmonary infection. Given the chronic, progressive, and disabling nature of CF, multiple treatments are prescribed, most on a daily basis. Thus, this illness requires children, with the aid of their families, to adopt multiple health-related behaviors in addition to managing more typical developmental demands. The morbidity and mortality factors pose cognitive, emotional, and behavioral challenges for many children with CF and their families. This article applies a developmental perspective to describing the psychosocial factors affecting psychological adjustment and health-related behaviors relevant to infants, preschool and school-age children, and adolescents with CF. Topics particularly pertinent to developmental periods and medical milestones are noted, with clinical implications highlighted.
authors.affiliation: Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH 45229-3039, USA. Michelle.Ernst@cchmc.org
authors.forename: Michelle M
authors.initials: MM
authors.lastname: Ernst
authors.forename: Mark C
authors.initials: MC
authors.lastname: Johnson
authors.forename: Lori J
authors.initials: LJ
authors.lastname: Stark
mesh_terms: D000367:Age Factors; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002648:Child; D002657:Child Development; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000235:genetics / Q000523:psychology* / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D002149:Energy Intake; D015444:Exercise; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009769:Object Attachment; D010287:Parent-Child Relations; D011584:Psychology; D011788:Quality of Life / Q000523:psychology; D012919:Social Behavior; D014815:Vitamins / Q000627:therapeutic use
chemical_list: D000900:Anti-Bacterial Agents; D014815:Vitamins; D019005:Cystic Fibrosis Transmembrane Conductance Regulator