TY - JOUR
T1 - Cystic fibrosis in adults
T2 - A changing scene
AU - Blau, Hannah
AU - Livne, Moshe
AU - Mussaffi, Huda
PY - 2003/7/1
Y1 - 2003/7/1
N2 - Background: Cystic fibrosis is no longer a terminal illness of childhood and mean survival is now over 30 years. Adult patients with atypical CF are increasingly being diagnosed. In Israel, all patients are still followed in pediatric centers. Objectives: To describe our experience with adult CF, stressing the importance of adult-related health and psychosocial issues. Methods: Twenty-five CF patients aged 20-50 years, constituting 44% of the 57 patients followed at our center, were analyzed for pulmonary and extrapulmonary features and management. Results: Nineteen were diagnosed as children and 6 as adults. Nineteen were pancreatic-insufficient and 6 were pancreatic-sufficient, including 5 diagnosed as adults. Pulmonary status was usually stable, with forced expiratory volume in 1 second 66.3 ± 21% (mean ± SD) and no difference between pancreatic-sufficient and insufficent patients. The tatter had more hemoptysis, Pseudomonas infection, intestinal obstruction, liver disease and diabetes. Two patients died of malignancy and two of advanced lung disease. A majority received continuous inhaled and oral antibiotics, bronchodilators, Dnase, physiotherapy and periodic home intravenous antibiotics. Psychosocial functioning was excellent: 60% were employed, 36% were married and 40% had children (none with CF). Patients diagnosed as adults had mild multisystem disease or isolated severe lung disease. Conclusions: CF adults generally have a good quality of life. Advances in understanding the CF defect and a plethora of new treatment modalities bode well for the future. Patients must be maintained in optimal condition to reap the benefits, and there is an urgent necessity for adult physicians to develop expertise in CF.
AB - Background: Cystic fibrosis is no longer a terminal illness of childhood and mean survival is now over 30 years. Adult patients with atypical CF are increasingly being diagnosed. In Israel, all patients are still followed in pediatric centers. Objectives: To describe our experience with adult CF, stressing the importance of adult-related health and psychosocial issues. Methods: Twenty-five CF patients aged 20-50 years, constituting 44% of the 57 patients followed at our center, were analyzed for pulmonary and extrapulmonary features and management. Results: Nineteen were diagnosed as children and 6 as adults. Nineteen were pancreatic-insufficient and 6 were pancreatic-sufficient, including 5 diagnosed as adults. Pulmonary status was usually stable, with forced expiratory volume in 1 second 66.3 ± 21% (mean ± SD) and no difference between pancreatic-sufficient and insufficent patients. The tatter had more hemoptysis, Pseudomonas infection, intestinal obstruction, liver disease and diabetes. Two patients died of malignancy and two of advanced lung disease. A majority received continuous inhaled and oral antibiotics, bronchodilators, Dnase, physiotherapy and periodic home intravenous antibiotics. Psychosocial functioning was excellent: 60% were employed, 36% were married and 40% had children (none with CF). Patients diagnosed as adults had mild multisystem disease or isolated severe lung disease. Conclusions: CF adults generally have a good quality of life. Advances in understanding the CF defect and a plethora of new treatment modalities bode well for the future. Patients must be maintained in optimal condition to reap the benefits, and there is an urgent necessity for adult physicians to develop expertise in CF.
KW - Adults
KW - Cystic fibrosis
KW - Multidisciplinary team
KW - Prognosis
KW - Transitioning
UR - http://www.scopus.com/inward/record.url?scp=0043067998&partnerID=8YFLogxK
M3 - ???researchoutput.researchoutputtypes.contributiontojournal.article???
AN - SCOPUS:0043067998
SN - 1565-1088
VL - 5
SP - 491
EP - 495
JO - Israel Medical Association Journal
JF - Israel Medical Association Journal
IS - 7
ER -