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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)
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• Survival is now predicted to be 40 years for those patients born in the 1990’s
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Caseload (annual report 4/00 - 3/01)
• 36 patients registered
• 3 patients on active transplant list
• 3 patients not suitable for transplant
• 2 deaths
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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
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• 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
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• Bi-lateral lung transplant Sept 2001
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Specialities Involved
• Respiratory
• Gastro-Intestinal
• Obs & Gynae
• GP/DN
• Surgery
• Transplant team
• Child & Family Psychiatry
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Diabetic Control
• Patients with CFRD - 5
• Glucose Intolerant patients - 2
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Intravenous Antibiotics
• 206 in-patient days
• 894 out-patient days
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Home Visits
IV's
TIVAD
Support
P.Care
Other
• Total number - 400
• Mileage - 11809
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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
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Role of CFCNS
• “National consensus standards for the nursing management of cystic fibrosis” march 2001
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Responsibilities
• Advocacy
• Clinical Management
• Support and Advice
• Education for patient, families and carers
• Education, research and management
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• Psychology
• Dietetics
• Physiotherapy & Community physio
• Social work
• Dr Paterson (bone densitometry)
• Pain clinic
• Rheumatology
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CYSTIC FIBROSIS
Mags Pogson CFCNS
Lawrie MacDougall CFCNS
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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
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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
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