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)

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

Caseload (annual report 4/00 - 3/01)

• 36 patients registered

• 3 patients on active transplant list

• 3 patients not suitable for transplant

• 2 deaths

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

• 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

• Bi-lateral lung transplant Sept 2001

Specialities Involved

• Respiratory

• Gastro-Intestinal

• Obs & Gynae

• GP/DN

• Surgery

• Transplant team

• Child & Family Psychiatry

Diabetic Control

• Patients with CFRD - 5

• Glucose Intolerant patients - 2

Intravenous Antibiotics

• 206 in-patient days

• 894 out-patient days

Home Visits

IV's

TIVAD

Support

P.Care

Other

• Total number - 400

• Mileage - 11809

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

Role of CFCNS

• “National consensus standards for the nursing management of cystic fibrosis” march 2001

Responsibilities

• Advocacy

• Clinical Management

• Support and Advice

• Education for patient, families and carers

• Education, research and management

• Psychology

• Dietetics

• Physiotherapy & Community physio

• Social work

• Dr Paterson (bone densitometry)

• Pain clinic

• Rheumatology

CYSTIC FIBROSIS

Mags Pogson CFCNS

Lawrie MacDougall CFCNS

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

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