21. CF Incidence, Prevalence and Survival Carrier rate of 1 in 25 Incidence of 1 in 2,500 live births By 2002 the number of adult patients will equal or exceed the number of children (Elborn et al 1991) Carrier screening may influence numbers (Cunningham & Marshall 1998)

22. Survival is now predicted to be 40 years for those patients born in the 1990’s

23. Caseload (annual report 4/00 - 3/01) 36 patients registered 3 patients on active transplant list 3 patients not suitable for transplant 2 deaths

29. Case Study Diagnosed at 10 months with steatorrhea and LRTI Stable until 13 when she required increasingly frequent IV’s Pregnancy 1996 - TOP @ 16 weeks Since 1998 she has suffered more frequent exacerbations and now requires IV’s monthly

30. Oxygen dependent Abnormal liver function Occasional episodes of DIOS Button gastrostomy inserted Transplant assessment Dec 2000 Overnight BiPAP from June 2001 Difficulty in controlling pain and nausea

31. Bi-lateral lung transplant Sept 2001

32. Specialities Involved Respiratory Gastro-Intestinal Obs & Gynae GP/DN Surgery Transplant team Child & Family Psychiatry

41. Diabetic Control Patients with CFRD - 5 Glucose Intolerant patients - 2

42. Intravenous Antibiotics 206 in-patient days 894 out-patient days

43. Home Visits Total number - 400 Mileage - 11809

44. Quality Issues Performance indicator tool for home IV’s Risk assessment of patients carrying out home IV’s Introduction of segregated clinics from Jan 01

45. Role of CFCNS “National consensus standards for the nursing management of cystic fibrosis” march 2001

46. Responsibilities Advocacy Clinical Management Support and Advice Education for patient, families and carers Education, research and management

47. Psychology Dietetics Physiotherapy & Community physio Social work Dr Paterson (bone densitometry) Pain clinic Rheumatology

52. CYSTIC FIBROSIS Mags Pogson CFCNS Lawrie MacDougall CFCNS

53. Physiological and Radiological Characterisation of Patients With COPD in Primary Care 110 patients aged 40-80 Acute exacerbation of COPD diagnosed by GP HRCT chest and physiological assessment when stable @ 2 months later –O’Brien et al Thorax 2000;55:635-42

54. Physiological and Radiological Characterisation of Patients With COPD in Primary Care Wide range of impairment of FEV1 Only 5% of patients had a significant bronchodilator response Emphysema present in 51% of patients Bronchiectasis present in 29% of patients –O’Brien et al Thorax 2000;55:635-42