Cost-benefit analysis of a national screening programme for cystic fibrosis in an Israeli population.

The recently acquired ability to identify 97% of CF carriers in an Israeli Ashkenazi population, prompts an evaluation of a nationwide screening programme. In 1993, the programme would first screen and counsel 9,261 parents, then 396 spouses of carrier parents and finally screen 16.5 fetuses where both parents are carriers. Assuming 92% of screened parents choose abortion of fetus screened positive, 2.33 cases of CF will be prevented in 1993 at a direct cost of $781,000. The $326,000 direct costs of preventing a CF case, exceed the lifetime excess direct costs per case of $297,000. However, benefits of screening also accrue to subsequent pregnancies, resulting in a direct benefit ($14.45 million) to cost ($10.39 million) ratio of 1.39/1 for the period 1993-2032. When benefits and costs resulting from mortality changes, work absences and transport costs are included, the benefit ($15.95 million) to cost ($13.88 million) ratio falls to 1.15/1. Benefit-cost ratios are lower for other ethnic groups in Israel, due to lower carrier rates and lower mutation detection abilities. A CF screening programme will increase the freedom of individuals choice, but should be carried out carefully in order to minimize stigmatization and even discrimination against CF carriers.

[1]  N. Holtzman,et al.  Assessment of risk by pregnant women: implications for genetic counseling and education. , 1986, Social biology.

[2]  R. Williamson,et al.  Screening for carriers of cystic fibrosis through primary health care services. , 1991, BMJ.

[3]  L. Sharples,et al.  Cystic fibrosis: lung transplantation. A new option--a new dilemma! , 1992, Journal of the Royal Society of Medicine.

[4]  C. M. Parkes Planning for the Aftermath , 1991, Journal of the Royal Society of Medicine.

[5]  L. Tenkate Carrier screening in CF. , 1989 .

[6]  Geddes Dm,et al.  Cystic fibrosis and pregnancy. , 1992, Journal of the Royal Society of Medicine.

[7]  L. Tsui,et al.  Identification of the cystic fibrosis gene: chromosome walking and jumping. , 1989, Science.

[8]  L. Cohen,et al.  CYSTIC FIBROSIS AND PREGNANCY A National Survey , 1980, The Lancet.

[9]  Charles R.scriver,et al.  The Metabolic basis of inherited disease , 1989 .

[10]  L. Tsui,et al.  Erratum: Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA , 1989, Science.

[11]  M. Goodman,et al.  The Overselling of Genetic Anxiety , 1982 .

[12]  D. Kalousek,et al.  Chorionic villus sampling: Analysis of fetal losses to delivery, placental pathology, and cervical microbiology , 1991, Prenatal diagnosis.

[13]  P. Farrell,et al.  Parents' Knowledge of Neonatal Screening and Response to False‐Positive Cystic Fibrosis Testing , 1992, Journal of developmental and behavioral pediatrics : JDBP.

[14]  J. Wallwork,et al.  Successful heart-lung transplantation for cystic fibrosis. , 1988, Chest.

[15]  B. Childs,et al.  Tay-Sachs screening: motives for participating and knowledge of genetics and probability. , 1976, American journal of human genetics.

[16]  C. Springer,et al.  Screening for five mutations detects 97% of cystic fibrosis (CF) chromosomes and predicts a carrier frequency of 1:29 in the Jewish Ashkenazi population. , 1992, American journal of human genetics.

[17]  G. Ginsberg Cost-Effectiveness Analysis, Cost-Benefit Analysis and the Value of Life in Health Care and Prevention , 1990 .

[18]  B. Strandvik,et al.  Home intravenous antibiotic treatment of patients with cystic fibrosis , 1992, Acta paediatrica.

[19]  D. Brock,et al.  Prenatal cystic fibrosis carrier testing: designing an information leaflet to meet the specific needs of the target population. , 1992, Journal of medical genetics.

[20]  R. Dinwiddie Cystic fibrosis: yesterday, today and tomorrow. , 1991, Journal of the Royal Society of Medicine.

[21]  B. Wilcken,et al.  REDUCED MORBIDITY IN PATIENTS WITH CYSTIC FIBROSIS DETECTED BY NEONATAL SCREENING , 1985, The Lancet.

[22]  M. King,et al.  Acute and long-term amiloride inhalation in cystic fibrosis lung disease. A rational approach to cystic fibrosis therapy. , 1990, The American review of respiratory disease.

[23]  R. Kane,et al.  Cost savings and economic considerations using home intravenous antibiotic therapy for cystic fibrosis patients , 1988, Pediatric pulmonology.

[24]  L. Sharples,et al.  Heart and lung transplantation for terminal cystic fibrosis: A 4½-year experience , 1991 .

[25]  R. Kaplan,et al.  Functional status as an overall measure of health in adults with cystic fibrosis: further validation of a generic health measure. , 1992, Journal of clinical epidemiology.

[26]  G. Walbroehl Sexual concerns of the patient with pulmonary disease. , 1992, Postgraduate medicine.

[27]  W. Walker,et al.  Nutrition support in cystic fibrosis. , 2009, Nutrition reviews.

[28]  A. Macdonald,et al.  Nutritional strategies in cystic fibrosis: current issues. , 1991, Journal of the Royal Society of Medicine.

[29]  A. Garber,et al.  Costs and benefits of prenatal screening for cystic fibrosis. , 1991, Medical care.

[30]  D. Brahams Liability in negligence as between negligent doctor and dispensing chemist: need for insurance to be made compulsory? , 1983, Lancet.

[31]  C. Whitten Sickle-cell programming--an imperiled promise. , 1973, The New England journal of medicine.

[32]  Matthew P. Anderson,et al.  Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells , 1990, Nature.

[33]  Holtzman,et al.  Proceed With Caution: Predicting Genetic Risks In The Recombinant DNA Era , 1989 .

[34]  N. Wald,et al.  Couple screening for cystic fibrosis , 1993, The Lancet.

[35]  T. Higenbottam,et al.  Heart-lung transplantation for cystic fibrosis. , 1991, Journal of the Royal Society of Medicine.

[36]  L. Tsui,et al.  Erratum: Identification of the Cystic Fibrosis Gene: Genetic Analysis , 1989, Science.

[37]  A. Coates,et al.  Short-term clinical, nutritional, and functional effects of continuous elemental enteral alimentation in children with cystic fibrosis. , 1984, The Journal of pediatrics.

[38]  T. Thelin,et al.  Clinical Follow‐up and Parental Attitudes Towards Neonatal Screening , 1981, Acta paediatrica Scandinavica.

[39]  S. Alemagno,et al.  Carrier screening for cystic fibrosis: a pilot study of the attitudes of pregnant women. , 1992, American journal of public health.

[40]  A. Beaudet,et al.  Linkage disequilibrium, cystic fibrosis, and genetic counseling. , 1989, American journal of human genetics.

[41]  A. Khaghani,et al.  Intermediate-term results of heart-lung transplantation for cystic fibrosis , 1992, The Lancet.

[42]  M R Natowicz,et al.  Discrimination as a consequence of genetic testing. , 1992, American journal of human genetics.

[43]  L. Tsui,et al.  Mutation analysis for heterozygote detection and the prenatal diagnosis of cystic fibrosis. , 1990, The New England journal of medicine.

[44]  A. Smith,et al.  Antibiotic therapy in cystic fibrosis: evaluation of clinical trials. , 1986, The Journal of pediatrics.

[45]  A. Beaudet,et al.  Genotyping errors with the polymerase chain reaction. , 1990, The New England journal of medicine.

[46]  M. King,et al.  A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. , 1990, The New England journal of medicine.

[47]  C. Stoehr,et al.  Cystic fibrosis. Target population for lung transplantation in North America in the 1990s. , 1992, The Journal of thoracic and cardiovascular surgery.

[48]  M. Knowles,et al.  Diabetes and cystic fibrosis: new questions emerging from increased longevity. , 1988, The Journal of pediatrics.

[49]  R. Axton,et al.  A preliminary trial of couple screening for cystic fibrosis: designing an appropriate information leaflet , 1993, Clinical genetics.

[50]  S. Finkelstein,et al.  Diabetes mellitus associated with cystic fibrosis. , 1988, The Journal of pediatrics.

[51]  Z. Miedzybrodzka,et al.  Screening for cystic fibrosis , 1991, The Lancet.

[52]  Z. Miedzybrodzka,et al.  A new approach to prenatal cystic fibrosis carrier screening. , 1993, Journal of medical genetics.

[53]  B. Brambati,et al.  Risk evaluation of CVS , 1993, Prenatal diagnosis.

[54]  S. Fitzsimmons,et al.  The changing epidemiology of cystic fibrosis. , 1993, The Journal of pediatrics.

[55]  B. Bernhardt,et al.  The economics of clinical genetics services. II. A time analysis of a medical genetics clinic. , 1987, American journal of human genetics.

[56]  T. Marteau,et al.  Effects of genetic screening on perceptions of health: a pilot study. , 1992, Journal of medical genetics.

[57]  D. Geddes,et al.  SELF-ADMINISTERED HOME INTRAVENOUS ANTIBIOTIC THERAPY IN BRONCHIECTASIS AND ADULT CYSTIC FIBROSIS , 1984, The Lancet.

[58]  L. Sharples,et al.  Heart and lung transplantation for terminal cystic fibrosis. A 4 1/2-year experience. , 1991, The Journal of thoracic and cardiovascular surgery.

[59]  H. Auerbach,et al.  Prospective controlled study of home and hospital therapy of cystic fibrosis pulmonary disease. , 1987, The Journal of pediatrics.

[60]  R. Rosenfeld,et al.  Short stature and pubertal delay in male adolescents with cystic fibrosis. Androgen treatment. , 1984, American journal of diseases of children.

[61]  J. Bosso,et al.  Feasibility and Cost Savings of Intravenous Administration of Aminoglycosides in Outpatients with Cystic Fibrosis , 1985, Drug intelligence & clinical pharmacy.

[62]  K. Klinger,et al.  Statement from the National Institutes of Health workshop on population screening for the cystic fibrosis gene. , 1990, The New England journal of medicine.

[63]  J. S. Alper,et al.  Genetic Screening: Triumphs, Problems, and Controversies , 1991, Journal of public health policy.

[64]  J. Gustafson,et al.  Ethical and social issues in screening for genetic disease. , 1972, The New England journal of medicine.

[65]  B. Wilfond,et al.  The cystic fibrosis gene: medical and social implications for heterozygote detection. , 1990, JAMA.

[66]  G. Evers‐Kiebooms,et al.  Impact of genetic counseling: A review of published follow‐up studies , 1979, Clinical genetics.

[67]  R. Williamson,et al.  Psychological and social consequences of community carrier screening programme for cystic fibrosis , 1992, The Lancet.