HC Needs Assessment Adult CF

7/28/2019 HC Needs Assessment Adult CF

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CONTENTSExecutive Summary1. Introduction2. Background

2.1. Cystic Fibrosis (CF)2.2. Classification of Cystic Fibrosis2.3. Importance of the problem2.4. Sub-categories

3. Aims and Objectives3.1. Aim3.2. Objectives

4. What is the size of the problem?4.1. South East Coast Population4.2. Methods for estimating incidence and prevalence4.3. Incidence in the South East Coast4.4. Prevalence in the South East Coast4.5. Validation of estimates4.6. Increase in adult CF population

4.7. Transition Care4.8. Limitation of estimates4.9. Mortality and morbidity4.10. Mortality4.11. Morbidity

5. What are the most appropriate and cost effective interventions?5.1.Literature Search5.2.Aetiology5.3.Pulmonary disease management5.4.Extra-pulmonary disease management5.5.Complications

5.6.Advances in treatment5.7.Cost effectiveness

6. What are the existing services, gaps and models of care?6.1.Organisation of CF Care in the UK6.2.CF care in the South East Coast6.3.Inpatient usage of CF services6.4.Comparison with literature on inpatient services6.5.Outpatient utilization of CF services6.6.General practitioners and community care6.7.Access6.8.Stakeholder views

6.9.Literature on patient perspectives6.10. Models of care

7. Discussion and Recommendations7.1.Discussion7.2.Limitations7.3.Recommendations

8. References9. Appendices


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EXECUTIVE SUMMARYThis health needs assessment has been produced for the South East Coast (SEC) CysticFibrosis Steering Group, which is a sub group of the South East Coast SpecialisedCommissioning Group (SEC SCG). The aim of the group is to develop a 5 -10 year strategy forcystic fibrosis services for the South East Coast to address the expected increase in demand forservices, particularly in the adult population.

This healthcare needs assessment has used literature and various data sources locally andnationally to estimate the current and projected number of adult patients with cystic fibrosisacross the South East Coast who have the potential to benefit from cystic fibrosis healthcare.The effectiveness and cost effectiveness of various treatments has been presented. Currentservices have been outlined including baseline service provision, uptake and utilisation ofservices and access. Consultation with stakeholders has identified key issues and gaps incurrent service provision. Guidelines and literature on models of care have been described. Thishas led to the formulation of recommendations that aim to inform the development of the SouthEast Coast Specialised Commissioning Group Cystic Fibrosis Strategy by highlighting theneeds of the population and the gaps in service provision.

KEY FINDINGS

The estimated incidence of CF in SEC was approximately 20 new cases per total live birthsin 2006.

The estimated prevalence of CF in SEC was 530 in all ages with almost half, 260, thought tobe aged16 years or over.

Surrey PCT had the highest estimated number of adult patients (65) whilst Hastings andRother had the lowest number of adult patients (11).

It is estimated that the adult population will increase by about 100 patients from 260 in 2006to 360 patients in 10 years time, an increase of around 10 patients per year.

Inpatient admission rates varied by PCT, and in particular, Surrey PCT had a relatively lownumber of inpatient admissions compared to other PCTs.

There are some gaps in the information on the current adult cystic fibrosis services providedin the South East Coast, particularly around skill mix.

Of the 11 Trusts in the SEC, 5 Trusts do not provide any adult services, whilst 4 trusts statethat they care foradult CF patients and provide shared care. Frimley Park, the only adult CFSpecialist Centre located in the South East Coast area, cares for approximately 100 adultpatients.

In 2006/07, 63% of all adult inpatient admissions were to adult Specialist CF centres,

recognized as such by the Cystic Fibrosis Trust. Inpatient admission rates varied by PCT.The average number of outpatient attendances for all ages in 2004 (the most recent availableoutpatient data) was estimated by PCT and showed noticeable variations between PCT. Itwas also noted that several patients did not have any outpatient attendances in 2004.

There are marked variations in journey times to adult specialist centres. 3 of the 11 locationsin the South East Coast with the highest number of CF residents live more than 50 milesaway from an adult specialist centre. Return train travel times to adult specialist centres vary


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1. INTRODUCTIONThis health needs assessment has been produced for the South East Coast Cystic FibrosisSteering Group, which is a sub group of the South East Coast Specialised CommissioningGroup (SEC SCG), who are responsible for commissioning cystic fibrosis services across theSouth East Coast. The South East Coast Cystic Fibrosis Steering Group was established toensure that a robust and appropriate model of care is in place for all cystic fibrosis patientsacross the South East Coast and to ensure improved health outcomes for people living with

cystic fibrosis in the South East Coast catchment population by effective commissioning ofappropriate and cost effective services. The aim of the group is to develop a 5-10 year strategyfor cystic fibrosis services for the South East Coast (SEC) to address the expected increase indemand for services particularly in the adult population. This health needs assessment willassist in the development of the strategy to address the needs of the population and gaps inservice provision.

Cystic fibrosis is one of the 35 services in the Specialised Services National Definitions Set.1

The Department of Health has stated that specialised services are defined as being high cost,low volume interventions and treatments and provided by relatively few specialist centres.Specialised services are commissioned at a regional level by the 10 Specialised CommissioningGroups in England.

A review of commissioning arrangements for specialised services was carried out in 2006. Itdefined specialised services as those provided in relatively few specialist centres to catchmentpopulations of more than a million people. One of the recommendations of the review was thatSCGs should formally designate specific providers to provide specific specialised servicesbased on nationally agreed patient centered, clinical service, quality and financial criteria and bere-assessed every five years. 2

2. BACKGROUND2.1 Cystic FibrosisCystic fibrosis is the commonest life-limiting autosomal recessive condition to affect theCaucasian UK population.3 The cystic fibrosis gene codes for the cystic fibrosis transmembrane

conductance regulator (CFTR) protein which is involved in the transport of chloride and sodiumions across the membranes of epithelial cells that line the ducts of exocrine glands (sweatglands, pancreas, liver and reproductive system). Dysfunction of this protein results in reducedchloride secretion and excessive sodium absorption. This leads to impaired water secretion,salty sweat, and abnormally thick exocrine secretions.4

Cystic fibrosis is a multi-system disease affecting principally the lungs and digestive system. It isa complex condition involving genetic, nutritional, inflammatory, pulmonary, gastroenterological,psychological, renal, rheumatological and obstetric complications.

In infants the abnormal airway secretion commonly predisposes to chest infections, which aredifficult to eradicate and if not treated early and effectively with antibiotics, often become chronic

and damaging. Viscid secretions obstruct the pancreatic ducts causing severe malabsorption.Signs of severe intestinal malabsorption, failure to thrive and lower respiratory tract infectionscan present at an early age.5 CF has a highly variable presentation and course. Someindividuals with CF have severe pulmonary and/or gastrointestinal disease, while others haverelatively mild disease that presents during adulthood.

3This will become less common with

screening.

2.2 Classification of Cystic FibrosisIn the current edition of the International Classification of Disease (ICD 10), cystic fibrosis isplaced in the section devoted to Endocrine, Nutritional and Metabolic Diseases, Chapter 4.6 It is


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important to identify the classification codes of the disease as they define the disease in termsof the data analysed and allow for the methodology to be reproduced. The classification forcystic fibrosis is subdivided into four parts:

E84 Cystic FibrosisE84.0 CF with pulmonary manifestationsE84.1 CF with intestinal manifestations, meconium illeus

E84.8 CF with other manifestationsE84.9 CF unspecified

2.3 Importance of the problemImproved survivalCystic fibrosis has traditionally been managed by paediatricians. Sufferers of cystic fibrosispreviously died as babies or young children, but with a better understanding of the condition,improved treatment regimes, the application of new antimicrobial therapies, pancreatic enzymereplacement and nutritional knowledge, this has enabled symptoms to be better controlled. Nowsurvival into the fourth and fifth decades is common with specialist care. Continueddevelopments in therapy have resulted in almost yearly improvements in survival in CF. TheCystic Fibrosis Foundation in the US states that median predicted survival was 36.9 years in

2006. The graph below shows the change in mean predicted survival over the past 20 years.

Source: Boyle, M. P. JAMA 20077

Figure 1. Cystic Fibrosis Median Predicted Survival Age from 1985 Through 2006


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Increasing proportion of adultsIt has been said that cystic fibrosis can no longer be referred to as a disease of childhood,instead it has also become a disease of adulthood.8 It is now thought that in the future cysticfibrosis patients will consist of relatively well children and young adults, with an increasingpopulation of sicker and older adults. This means that there are a rapidly increasing number ofadult patients with inevitably more severe problems, whilst the paediatric population will berelatively static. Data from the cystic fibrosis database in 2003 showed that in the UK, 51% of

patients registered with cystic fibrosis were 16 years or over.

8

Between 1965 and 2000 theproportion of adult cystic fibrosis patients in the United States increased dramatically.9 Theincrease is shown in the graph below. Demands on cystic fibrosis health services will increaseas the number of patients surviving into adulthood increases.10 This undoubtedly has an impacton the provision of cystic fibrosis services.

Source: Boyle, M. P. JAMA 20077

2.4 Sub-categoriesDue to the increasing number of adults with CF, the transfer of patients from the care ofpaediatric services to adult services is becoming increasingly important. It is well recognizedthat patients between the age of 16-18 will have different needs to those over 18 and thetransfer between paediatric and adult services needs to be well planned. This health needs

assessment will aim to highlight transition as a distinct subgroup of the adult population.

Figure 2. Percentage and number of adults with CF of the total numberof CF atients in USA over 35 ears


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3. AIM AND OBJECTIVES3.1 AimThe aim of this needs assessment is to assess the healthcare needs of the adult cystic fibrosispopulation resident in the South East Coast to inform the commissioning of appropriate servicesthat will improve the health of this population group in the most efficient way. Theepidemiological approach to HNA has several elements: (i) determining the incidence andprevalence; (ii) identifying the effectiveness and cost-effectiveness of existing interventions (iii)

identifying the current level of service provision. The outcome is to propose servicedevelopments to remedy gaps in services.

3.4 Objectives

To carry out an assessment of the healthcare needs of adult cystic fibrosis patients usingliterature and various data sources locally and nationally

To determine the current and projected number of patients across the SEC area requiringadult cystic fibrosis healthcare

To carry out a literature search to identify evidence on treatment and models of care To determine the level of service provision and geographical location of services To gather evidence on the uptake and access to services To identify gaps in current services and identify key issues by consulting with stakeholders To review current models of care and provide recommendations that will inform the

development of a strategy for an effective model of care for adult cystic fibrosis services


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4. THE SIZE OF THE PROBLEMThis section describes the adult CF population in the South East Coast. Incidence, prevalence,mortality and morbidity data are used to describe the adult cystic fibrosis population in terms ofsize, composition and predicted change to provide an insight into the burden of disease of thecondition.

4.1 South East Coast population

The South East Coast covers a population of about 4.2 million people in Kent, Surrey andSussex. Of these, 3.4 million are aged 16 and over. The South East Coast region has 8 PCTs:Medway, Eastern & Coastal Kent, West Kent, Hastings & Rother, East Sussex Downs & Weald,Brighton & Hove City, West Sussex, and Surrey.

Figure 3. Map of the Primary Care Trusts in South East Coast region11

The following table gives the total number of live births and residents by PCT using dataobtained from the ONS, which was initially shown by local authority and converted intoPCTs.12There were 49,163 live births in the SEC in 2006 and 4.2 million residents in the mid2006 estimates from The Office of National Statistics. Surrey PCT had the highest numberof births and the largest population.

Table 1. Number of live births and number of residents by PCT in the South East Coastregion

Number of residents (mid 2006)PCT

Number of LiveBirths (2006) 16-19 20+ Total 16+ All Ages

Brighton and Hove City 3,100 11,760 196,700 208,460 249,700

East Sussex Downs and Weald 3,162 16,160 253,100 269,260 329,100Eastern and Coastal Kent 8,205 38,480 539,500 577,980 717,100

Hastings and Rother 1,827 8,640 135,600 144,240 176,200

Medway 3,257 14,240 184,000 198,240 251,700

Surrey 13,085 51,360 806,300 857,660 1,063,600

West Kent 8,075 33,840 489,500 523,340 656,100

West Sussex 8,452 36,320 586,700 623,020 766,600

South East Coast 49,163 211,920 3,201,400 3,413,320 4,221,700(Source: Created from ONS data)


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4.2 Methods for estimating Incidence & prevalenceThere are several recognised ways in which incidence and prevalence can be estimated for ahealth needs assessment. These are: Registry or Disease registers for the local population Epidemiological studies in literature for the local/UK population International sources

4.2.1 Registry dataThe UK Cystic Fibrosis Database was started in 1995 by the Cystic Fibrosis Trust and haspublished annual reports. This data is obtained from sixty Specialist CF Centres and CF Clinicsfrom all over the UK. The most recent information available has been published in a report bythe CF Trust in 2004.13 Although the report from the CF Trust does not contain specificincidence or prevalence rates, it does state that in the UK in 2004, there were 164 newlydiagnosed patients with cystic fibrosis and 7064 patients registered with Cystic Fibrosis.

The following table gives the number of patients aged 16+ that were registered with the CysticFibrosis Trust Database in 2004, shown by PCT. The data was provided for all ages and theestimated adult population has been calculated by using a figure from the literature. Thesefigures need to be interpreted with caution as there are several limitations including coverage of

data.

Table 2. Total number of cystic fibrosis patients and estimated adult cystic fibrosispopulation in the South East Coast in 2004 according to PCT

PCT Total PopulationEstimated adult

population aged 16+

Brighton & Hove City PCT 16 8East Sussex Downs & Weald PCT 58 28Eastern & Coastal Kent PCT 78 37Hastings & Rother PCT 28 13Medway PCT 18 9Surrey PCT 180 87West Kent PCT 112 54

West Sussex PCT 81 38Grand Total 571 274

Source: Cystic Fibrosis Trust

4.2.2 LiteratureThe second method of estimating incidence and prevalence is by using recent epidemiologicalstudies of the condition. A literature search was carried out for incidence and prevalence ofcystic fibrosis in published literature. The search strategy was carried out using Medline 1996+and PubMed databases and an internet search was also carried out. There were relatively fewpublished studies that were carried out in the UK, with no studies being specific to the SouthEast Coast population. The following table has been compiled using incidence and prevalencedata cited in the literature.


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Table 3. Summary of UK and international literature showing incidence and prevalence ofCystic Fibrosis

4.3 Incidence in SEC

The average incidence rate (from the pooled UK studies shown in the table above) is 1/2432live births in the UK or 4.1 per 10,000 lives births. The number of live births in the SEC has beenused to estimate the incidence of cystic fibrosis in the SEC.

Table 4. Estimated incidence of cystic fibrosis by PCT using 2006 live births data

PCTIncidence per totallive births in 2006

Brighton and Hove City 1.3

East Sussex Downs and Weald 1.3

Eastern and Coastal Kent 3.4

Hastings and Rother 0.8

Medway 1.3

Surrey 5.4

West Kent 3.3

West Sussex 3.5

South East Coast 20.3

Total Adults >16

SourceIncidence Frequency/

prevalenceIncidence Frequency/

prevalence

UK literature

British Paediatric

Association Survey 1977-8514

1/2500 live births 4976 total UK

prevalence

UK Cystic Fibrosis Survey199515

1/2416 live births 6,657 2,569 aged 16 and over

Cystic fibrosis TrustUKCF database2004

13

164 newlydiagnosed pts(on CF register)

7,046 patientsregistered ondatabase in UK

11 newlydiagnosed pts

3,670 (51.4%)aged 16 and over

Cystic Fibrosis Mortalityand survival in UK 1947-200316

1/2381 live births 8,284 Growth of adultpopulation by145/yr

Lower respiratory diseasein UK - HNA, 200417

140/million residentpopulation

300/million in 15-24yrs35/million in 25+ yrs

International Literature

Italian CF Registry 200418

1 in 4,238

The Cystic FibrosisRegistry of Ireland 200719

1 in 1,353

Key Figures for incidence of CF in SEC

Estimated 20 new cases of CF per total live births in SEC in 2006

Surrey PCT had the highest estimated number of new cases of CF 5.4 cases in 2006

Hastings & Rother PCT had the lowest estimated number of new cases 0.8 cases in2006


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4.3.1. Age at diagnosisIn adults aged 16 and over the number of newly diagnosed CF patients registered on the CFTrust Database was 11. The population aged 16 and over in 2004 was 48.1959 million. Thisgives an incidence rate of newly diagnosed adults of 1 in 4,381,445 in the whole population. Itcould be estimated that there would be approximately 1 newly diagnosed patient aged 16+ inthe SEC per year.

4.4 Prevalence in SEC

The average prevalence in all ages from the pooled UK literature is estimated to be 1 in 7955 or125 per million which will be applied to the total population of the south east coast. Theprevalence in 16+ is estimated to be 1 in 13,132 or 76 per million. Data from the cystic fibrosisdatabase in 2003 showed that in the UK, 51% of patients registered with cystic fibrosis were 16years or over.8

Table 5. Estimated prevalence of cystic fibrosis using resident population data in2006 by PCT in the South East Coast region

PCT 16-19 20+ Total 16+ All Ages

Brighton and Hove City 1 15 16 31

East Sussex Downs and Weald 1 19 20 40

Eastern and Coastal Kent 3 41 44 90

Hastings and Rother 1 10 11 22

Medway 1 14 15 32

Surrey 4 61 65 134

West Kent 3 37 40 82

West Sussex 3 45 47 96

South East Coast 16 244 259 530

The prevalence of CF has been shown by age cohorts as the prevalence differs betweenpaediatric and adult patients. As can be seen. Surrey PCT was estimated to have the highestnumber of cystic fibrosis patients aged 16+ and the highest number of patients of all ages withcystic fibrosis followed by West Sussex. The proportionate distribution of CF patients by agecohort is shown in the graph below.

Key figures for Prevalence of CF in SEC

530 estimated patients with CF in SEC

Estimated 259 patients in 16+ age group (almost 50%)

Surrey PCT had the highest estimated number of patients followed by West Sussex

Hastings & Rother PCT had the lowest number of patients


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Figure 4. Estimated number with cystic fibrosis by age cohort & PCT in South East Coast

4.4.1. Age profilesThe following graph shows the age profile for all patients registered on the UK Cystic FibrosisDatabase in 2004. The age of the patient is calculated as at 31 st December in the year ofanalysis. There were a total of 7046 patients. The greatest percentage of patients were in the10-15 age group followed by 15-


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4.5 Validation of estimatesTo verify the incidence estimates for the South East Coast, a comparison has been made withanother source of data, PEAGASUS.20 This shows the incidence of genetic conditions by PCTin the SEC. In the majority of PCTs, cystic fibrosis makes up the greatest proportion of livebirths of the genetic conditions examined and the incidence compares well to the PCT estimatesin this report. PEGASUS data is shown in the appendices.

A comparison of prevalence of cystic fibrosis of patients aged 16 and over has been made withthe data supplied by the Cystic Fibrosis Trust Registry data (2004) cited previously. This isshown in the graph below. The figures show that in most PCTs the estimated number is fairlysimilar to the CF Registry number.

A systematic survey of European literature carried out recently provided an estimate of theprevalence of rare diseases in Europe. Limitations cited by the methodology of the review statethat the exact prevalence rate of each rare disease is difficult to assess from the available datasources and that there is a low level of consistency between studies, a poor documentation ofmethods used, confusion between terms. The prevalence of cystic fibrosis in all ages is quotedas 12/100,000. This rate is applied to the South East Coast population of approximately 4.2million, this gives an estimate for the total number of cystic fibrosis patients of 504. This alsocompares well to the estimated figure of 530.21

Figure 6. Comparison of data from CF Trust database and estimates for number ofpatients aged 16 and over with cystic fibrosis in the south east coast area

Comparison of CF Trust Data and estimates m ade from literature fo

number of patients 16 and over w ith cystic fibrosis

0

10

20

30

40

50

60

70

80

90

100

Brighton &

Hove City

PCT

East

Sussex

Dow ns &

WealdPCT

Eastern &

Coastal

Kent PCT

Hastings

& Rother

PCT

Medw ay

PCT

Surrey

PCT

West Kent

PCT

West

Sussex

PCT

PCT

Numberofpatients

Data f rom CF Trust Database

Estimates based on literature


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Predicted incre ase in adult CF population in SEC over 10 years

250

260

270

280

290

300

310

320

330

340

350

360

370

2006 2007 2008 2009 2010 2011 2012 2013 2014 2015

Year

NumberofadultswithCF

4.6 Increase in adult CF PopulationIn the UK, although the overall incidence of cystic fibrosis has remained fairy stable, lifeexpectancy is increasing, annual births out-number the annual deaths and so the prevalencehas been increasing.

In 2004, there were approximately 3670 adult patients with CF in 2004 and the CF Trustdatabase estimated that the adult population grew by 145 patients giving an increase of 3.9%. 22

According to one study, the number children with cystic fibrosis was predicted to remain fairlyconstant over the next 10 years, whereas adult numbers will be likely to increase by about 36%.The study concluded that health service provision for children will not need to changesubstantially over the next 10 years whereas services for adults will need to increase by about athird. 23

These estimates have been applied to the estimated SEC adult CF population and are shownon the graph below. The first estimate of an increase of 3.9% per year, would mean an increasefrom 259 to 364 adult patients, an increase of 105 patients over 10 years. The second estimateof an increase of 36% in 10 years would mean an increase of 95 patients from 259 to 354.These give an average increase of about 10 patients per year.

Figure 7. Predicted increase in adult CF population in SEC over 10 years

4.7 Transition CareThe time of transition to an adult CF services is usually between 14-18 years of age. Data fortransition was limited and the only data obtained was for Kent. Of the 31 Kent patients aged 16-18 during 2001-2007, 20 were initially admitted under 16 years but reached 16 years and overin subsequent admissions in the six year period. On average, 3 patients per year reached 16years of age in Kent. The main limitation of this estimate is the number of transition patients isbased on inpatient data.


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4.8 Limitations of estimates of incidence and prevalenceWhile this epidemiological approach has tried to be systematic, there is a lack of existing localepidemiological data. One paper states that carrying out new epidemiological work is costly andtime consuming, therefore there is a reliance on existing data which has the disadvantage that itmay not be timely. 24

It is well known that the CF is much more common in Caucasian populations than in those from

other ethnic backgrounds. It still occurs in these groups but more rarely.

17

Although data onethnicity within Kent, Surrey and Sussex exists, due to the time constraints of manipulating thedata, the incidence and prevalence rate has been calculated using the rates for the UK overalland applied to the local population without taking account of variations in ethnicity in the localpopulation.

Another limitation is that the prevalence rates taken from the literature relate to different yearsand have been averaged and they have been applied to the population in 2006. Caution mustbe taken when using these figures as they are estimates based on many assumptions.


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4.9 Mortality & MorbidityIn addition to incidence and prevalence, to give a whole picture of the burden of disease,health status needs to be included. Ideally local surveys would be undertaken to assess thehealth status of the adult cystic fibrosis population, for example, the number of chest infections,frequency of courses of antibiotics, and type and frequency of complications. As this data is notavailable for the local population and time constraints have meant that primary data collectioncannot be carried out, hospital activity has been used. Therefore, inpatient data and admission

diagnosis has been taken as a proxy of complications and health status.

4. 10 MortalityWhile the condition is a rare cause of death overall (


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4.11 MorbidityCystic fibrosis is a chronic condition that is managed across the health care sector. Overall,cystic fibrosis contributes to a very small proportion of GP consultations, hospital admissionsand hospital bed days (under 1%). The majority of care of cystic fibrosis patients takes place inhospital outpatients including intravenous antibiotic treatment, nebulised antibiotics, mucolytics,physiotherapy and nutritional management. Outpatient care is undertaken by a multidisciplinaryteam including physicians, specialist nurses, physiotherapists, dieticians and social workers.

Patients with cystic fibrosis require life long specialist medical supervision and treatment.

To describe the health status of the population survey data can be used which is particularlyuseful for planning acute services. Often, however, survey data on acute episodes of illness,treatment regimes/uptake or quality of life is limited and a proxy measure of acute illness suchas hospital admissions of GP consultations is used instead. A literature search for survey datawas carried out. No GP data for the local population on CF consultations was available.

4.11.1 Survey DataA literature search identified a survey carried out on adults with CF in the United Kingdom in2000.25 This survey was the third of its kind, the previous two surveys having been carried out in1990 and 1994. A brief summary of some of the findings from the survey has been included to

give an idea of morbidity in CF in adults has been included.

The survey found that the measure of disease severity used in the study, mean symptom scoreand body mass index were closely related to variables such as hospital admissions. In terms offrequency of admission, 48.3% had no admission to hospital in the previous year, a significantrise from 39.7% in 1994. The mean number of admissions per year was 1.5. In terms of homeIVs 41% had no home IV in the previous year. The mean number of courses was 1.92.

Some of the findings of the study are summarised in the table below. From the survey, 67.5% ofresponders reported one or more complications. This was more frequent among women andolder responders.

Quality of Life was also measured in the study using the EuroQOL questionnaire. The studyfound that overall, people with CF have a slightly lower health state than the normal UKpopulation matched by age and sex. However their rating of health state (0.77) and visualanalogue scale (VAS) were comparable to results for other people with chronic diseases suchas asthma (0.73) and low back pain (0.69). Their rating of health state was higher than those inthe general population who reported limiting longstanding illness (0.64), but lower than youngpeople with diabetes (0.88).

One report from the literature search looked at complications and treatments in adults andchildren in the US. The findings from both studies are summarised in the table below. As thereis no local survey data, these national and international survey findings have been applied to theSouth East Coast to provide a crude picture of local morbidity. Resistance and colonisationrates in the US may be different to the UK but this gives a rough idea of the numbers oftreatment uptake and planning of services.


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Table 6. Percentage of adults with infections, complications and treatment with estimatednumbers for SEC

S o u r c e : Data from American CFF Registry data and a UK survey9,25

4.11.2 Local Inpatient dataThe inpatient data has been compiled by the specialist commissioning team at West Kent PCT.These data are for the year of 2006/07. The adult cystic fibrosis patients have been taken as allthose aged 16 and over.

The following table shows the number of admissions and number of bed days by PCT ofresidence in 2006-07 for all recorded cystic fibrosis activity. There were 240 adult cystic fibrosispatients admitted as inpatients during the period with 543 admissions in total. This equates toan average of 2.3 admissions per patient admitted in 2006-07. A health needs assessment ofrespiratory conditions states that admission rates for cystic fibrosis patients vary between 0.3 to2.0 times per person per year.17

West Sussex PCT had the highest number of adult cystic fibrosis patients requiring inpatientadmission. It also had the highest number of actual admissions. Hastings and Rother PCT hadthe lowest number of patients and the lowest number of actual inpatient admissions. West KentPCT had the 3rd highest number of patients and 2nd highest number of admissions but thehighest number of bed days of all the PCTs. There were a total of 3114 inpatient bed days. Thiswas an average of 5.7 days per admission. The inpatient admission data included elective andnon-elective admissions.

% Adults inUS study

% Adults inUK study

Estimated numberaged 16+ in SEC

In fe c t io n

P aeruginosa colonization 79% 204

Ciprofloxacin resistance 22% 57Tobramycin resistance 15% 39B capacia colonization 6% 15C o m p l i c a t i o n s

Massive haemoptysis 1.8% 4Pneumothorax 1.4% 3CF related diabetes requiring insulin 16.2% 42CF related diabetes 25.9% 67Arthritis 23.1% 59Sinusitis 19.8% 51Infertility 18.9% 48Treatment

Pancreatic enzyme supplements 91% 235Vitamin supplements 93% 240Nebulised treatment

BronchodilatorsAntibioticsDornase alpha

80%49%63%38%

20712616398

Oral corticosteroids 37% 96On supplemental oxygen continuously 6% 15Home oxygen 8.8% 21Daily physiotherapy 51.2% 133


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Table 9. CF Inpatient admissions according to diagnosis for admissions with a primarydiagnosis of CF in SEC 2006-07

Source: SEC SCG

This data does need to be interpreted with caution due to the limitation of coding the diagnosis.Cystic fibrosis with pulmonary manifestation may be coded as a lower respiratory tract infectionwith a secondary diagnosis of cystic fibrosis or in another instance may have been coded as aprimary diagnosis of cystic fibrosis with pulmonary manifestations but both may be clinicallysimilar.

The following table shows all the remaining admissions that were not coded with a primarydiagnosis of cystic fibrosis. These were all ranked by number of admissions in descending order

and the table displays the highest 60%. As can be seen, some of the diagnoses would beclosely related with CF however, several diagnoses may be unrelated i.e. disturbances in tootheruption/impacted teeth, and headache.

The most frequent admission was for acute lower respiratory infection (17 admissions), followedby pneumothorax (9 admissions), followed by haemoptysis (5) equal to infection/inflammatoryreaction to device, implant & graft (5) other and unspecified intestinal obstruction (5). Theproportionate distribution of inpatient admission diagnoses by PCT are shown on a graph in theappendices.

DiagnosisPrimary(ICD)

BRIGHTONANDHO

VECITYPCT

EASTSUSSEXDOWNS

WEALDPCT

EASTERN&COASTA

LKENTPCT

HASTINGSANDROTHERPCT

M

EDWAYPCT

WESTKENTPCT

WESTSUSSEXPCT

SURREYPCT

GrandTotal

Cystic fibrosis with other manifestations E848 18 14 24 3 17 52 41 5 174

Cystic fibrosis with pulmonary manifestations E840 19 17 17 2 1 27 33 2 118

Cystic fibrosis, unspecified E849 14 14 29 1 2 13 33 1 107

Cystic fibrosis with intestinal manifestations E841 1 3 4

Grand total 52 45 70 6 20 95 107 8 403

Percentage of total inpatient admissions 81.3 74 79 100 95 88 77 15 74


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Table 10. Inpatient admissions according to diagnosis for those admissions not coded ashaving a primary diagnosis of CF in SEC 2006-07

Diagnosis Number of admissions

Unspecified acute lower respiratory infection 17

Pneumothorax, unspecified/other 9

Haemoptysis 5

Inf/inflamm react dev implants & grafts 5Other and unspecified intestinal obstruction 5

Adjustment and management of vascular access device 4

Chronic respiratory failure 4

Bronchiectasis 3

Chest pain, unspecified/other 4

End-stage renal disease 3

Polyp of nasal cavity 3

Acute appendicitis, unspecified/Other specified diseases of appendix 2

Acute pancreatitis 2

Acute renal failure, unspecified 2

Cough 2

Disturbances in tooth eruption/Impacted teeth 2

Headache 2Other and unspecified abdominal pain 2

Other specified functional intestinal disorders 2

Pulmonary embolism without mention of acute cor pulmonale 2

Urinary tract infection, site not specified 2Source: SEC SCG

Inpatient data has been specifically obtained for the three PCTs within Kent to provide data toshow for trends. The following graph shows the trend in inpatient admissions over six yearsfrom 2001-2007 in the three PCTs within Kent and Kent overall. This is the total number ofadmissions not individual patients. The number of inpatient admissions has generally increasedover this time in all the PCTs with the greatest increase in those admitted from West Kent PCT.This is shown in the graph below.


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Figure 9. Trends in inpatient admissions with a primary diagnosis of cystic fibrosisbetween 2001-2007 in Kent by PCT

Source: Compiled from Kent HIS data)

4.11.4 Outpatient and primary care dataPatients with chronic diseases such as cystic fibrosis mainly use outpatients and primary care

health services for their ongoing care, although a major feature of the disease is self-care. Onestudy has produced estimates for service use in 1998-99 in a hypothetical district with apopulation of 500,000 with a similar structure to England & Wales. It estimates that there were150 GP consultations related to cystic fibrosis.17 As the available outpatient data does notprovide a particularly useful picture of morbidity the outpatient data is presented and discussedunder service usage in the models of care section.

Trend in inpatient admissions w ith a primary diagnosis of

cystic fibrosis between 2001-2007 in Kent

0

50

100

150

200

250

2001/02 2002/03 2003/04 2004/05 2005/06 2006/07

Year

Numberofinpatientadmissions

Medw ay PCT

ECK PCT

WK PCT

Kent Total


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5. WHAT ARE THE MOST APPROPRIATE & COST EFECTIVEINTERVENTIONS?

5.1 Literature searchA literature search was conducted in December 2007 and January 2008. The following areaswere searched:

Medline 1996+PubMedNational Library for HealthNICECochrane LibraryGoogle

Several searches were carried out pertaining to evidence for treatment and cost effectiveness,The following MeSH terms were used: Cystic fibrosis AND adults AND evidence ANDtreatment. For cost effectiveness the MeSH term Models economic OR heath care costs,OR cost benefit analysis AND cystic fibrosis were used. Further searches were carried outbased on some of the references from papers already identified. The search had to be widenedfrom adults to all ages as the initial search revealed relatively few results that were specific toadults only. Many of the papers from the database searches were review articles, mainlyliterature reviews rather than systematic reviews. However systematic reviews were directlyfound through the Cochrane Database.

An in-depth description of evidence for each treatment modality is not given in this needsassessment for two reasons: firstly there is a wide breath of information available, and secondlythe aim of this section of needs assessment is intended to provide an overview of evidence fortreatment rather than for each specific modality of treatment.

5.2. AetiologyThere are a wide range of treatments for cystic fibrosis due to the multisystem nature of thedisease. The CFTR (cystic fibrosis transmembrane conductance regulator) acts as a chloridechannel and genetic defects in this gene affect transport of salt and water across cellmembranes. This mainly affects the lungs causing thickening of mucous that can becomeinfected with opportunistic bacteria and the pancreas causing a defective production of exocrineenzymes. Other organs are affected and the complications increase as the patient progressesinto adulthood.

The mainstay of treatment aims to minimise lung damage and maximise lung function andnutritional support. The management of cystic fibrosis is often categorised as pulmonarydisease and extra-pulmonary disease.

5.3. Pulmonary disease managementThe hallmark pulmonary pathology observed in cystic fibrosis is bronchial mucous plugging,inflammation and eventually bronchiectasis. There is a characteristic progressive loss of lungfunction in cystic fibrosis mainly beginning in the teenage years. Individuals often demonstratecharecteristc bacterial flora in their sputum with chronic infection such as S aureus and Hinfluenzae as children and P aeruginosa as adults. Approximately 80% of individuals arechronically infected with P aeruginosa by the age of 18. The clinical course of lung disease incystic fibrosis is marked by periodic exacerbations which are characterised by increasedsputum, dyspnoea, cough, fatigue, weight loss, and a decrease in spirometry measurements,which are in contrast to pneumonias as they are not usually accompanied by fever or positiveblood cultures.7


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The aims of treating the lungs at different stages of disease vary, as does therapy involved inacute and chronic management. The early disease stage aims to encourage mucous clearance,maintain good lung function and eradicate intermittent organisms. The intermediate stage ischaracterised by chronic infection caused by commoner organisms such as Pseudomonasaeruginosa and Staphylococcus aureus and treatment is based on suppression of bacterial loadand treating infective exacerbations. Therapies for the potential eradication of less common

organisms such as Burkholdria cepacia are usually assessed on an individual patient basis. Theend stage of pulmonary disease may result in haemoptysis, pneumothorax or respiratoryfailure.26

There are a wide range of treatments and evidence available.Respiratory treatments representthe greatest challenge to patients and families. One of the primary objectives when treatingpeople who have cystic fibrosis (CF) is to prevent, eradicate or control all types of respiratoryinfection, particularly infection with Staphylococcus aureus, Haemophilus influenzae,Pseudomonas aeruginosa and Burkholderia cepacia. CF Trust Consensus Guidelines state thatvarious regimes of oral, nebulised or intravenous antibiotics can eradicate or delay chronicinfection of the lower airways. This recommendation is based on evidence from at least onerandomised controlled trial.27 A high proportion of antibiotics are required intravenously as the

organisms become resistant. Even so, treatment does not often eradicate the organismcompletely. A high proportion of adult patients are colonised by organisms resistant to severalantibiotics which has important implications for infection control in hospital, outpatients andsocial settings. Several systematic reviews have concluded that nebulised antibiotics are usefulin the treatment of patients colonised with Pseudomonas aeruginosa. This benefit may bedemonstrated in terms of improved lung function, reduced hospital admissions, fewerintravenous antibiotic courses and improved survival.17

Because much of the increased viscosity of pulmonary secretions in patients with cystic fibrosisis due to DNA from neutrophils involved in chronic lung infection, recombinant human DNase(dornase alpha) is often inhaled to decrease sputum viscosity. Daily use of inhaled dornasealpha has been shown in several studies to improve FEV 1.

7 A Cochrane Review looking atDornase alpha, found that spirometric lung function improved in the treated groups, withsignificant differences at one month, three months, six months and two years but there was anon-significant difference at three years. Insufficient data were available to analyse differencesin antibiotic treatment, inpatient stay and quality of life.28

Physiotherapy is essential to clear secretions from the chest and maintain lung function. FourCochrane systematic reviews have evaluated the effectiveness of physiotherapy. When chestphysiotherapy was compared to no chest physiotherapy, the results of the review showed thatairway clearance techniques have short term effects in terms of increasing mucous transport butno evidence was found on which to draw conclusions regarding long term effects. 29 AnotherCochrane review looked at conventional chest physiotherapy techniques (CCPT) compared toother airway clearance techniques. The authors concluded that there was no demonstratedadvantage of CCPT over other airway clearance techniques in terms of respiratory function. 30

If the chronic decline in lung function results in FEV1 consistently below 30% of predictedfunction, individuals often begin to experience significant limitations in their daily activities andrequire more frequent need for hospitalisation and intravenous antibiotics which both lead toindividuals being referred for initial evaluation for transplant. Exact timing for lungtransplantation surgery depends on the severity of symptoms and rate of clinical decline. It isdocumented that individuals with cystic fibrosis undergoing lung transplantation do well or betterthan individuals with any other disease.7 Patients with end stage disease and those awaitinglung transplantation may also require oxygen or non-invasive positive pressure ventilation. The


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number of patients who can benefit from transplantation is limited by the supply of donororgans. 40 cystic fibrosis patients per year (of all ages in 1990-1992) had lung or hearttransplants in the UK.14

The following table shows various treatment options for pulmonary disease and the level ofevidence underpinning the treatment. This summarises evidence from several sources. Thetype of evidence for the intervention has been included where available. Treatment specifically

in adulthood was difficult to differentiate from much of the data.

Table 11. Summary of evidence for key pulmonary interventions in cystic fibrosis.

Pulmonary Disease Intervention Evidence

Segregation and cohorting of patientsto prevent cross infection

Evidence dependant on organism butconsensus lacking on some strategies.26

Airway clearance techniquesPhysiotherapy

Mucolytics - rhDHaseHypertonic saline

Based on evidence from RCTs andmetanalyses, Cochrane reviews. ForrhDNase and hypertonic saline evidencefor short to medium term only.

Prophylactic antibiotics Evidence for decreased incidence ofStaphylococcus aureus infection is based

on RCTs or metanalyses (long termbenefits and in adulthood less defined)

Early disease stage

Influenza vaccination

Oral or intravenous antibiotics Evidence for elective vs symptomatictreatment is considered to be lackingconsensus.26

Bronchodilators Systematic review showed short and long-acting beta-2 agonists can be beneficialboth in the short and long term in individualswith demonstrable bronchodilatorresponsiveness or bronchial hyper-

responsiveness.31

Anti-inflammatory drugs

Systemic steroids Inhaled steroids

Ibuprofen

Systemic steroids use may havebeneficial effect but significant sideeffects particularly when used in

childhood.32

Systematic review states evidence fromtrials is insufficient to establish if inhaledcorticosteroids have a beneficial orharmful effect in people with CF. Furthertrials are needed.33

Intermediate diseasestage

Nebulised antibiotics

Improve lung functionReduced admissions

Fewer IV antibiotics

3 x systematic reviews including oneCochrane review34

Non-invasive positive pressureventilation

Mainly subjective improvement

Oxygen Robust evidence lacking in CF but vastevidence in COPD35

End stage withcomplications

Lung or heart transplantation Observational studies show increasesurvival and QOL17

Sources: Adapted from several sources7, 17, 26, 36


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5.4 Extra-pulmonary disease managementAlthough the strongest determinant of length and quality of life in cystic fibrosis is pulmonaryhealth, nutritional and gastrointestinal issues also play a significant role.

85-90% of cystic fibrosis patients will have exocrine pancreas insufficiency. 32 Patients who havepancreatic insufficiency, require treatment with pancreatic enzyme supplements with the aim ofnormalising fat absorption and producing normal growth and weight gain. One study showed

that there is evidence that enteric coated microsphere preparations of pancreatic enzymesupplements improve fat absorption when compared to non-enteric coated preparations.However this is dose specific and higher doses may lead to colonic strictures. 17

Malabsorption of fat-soluble vitamins is likely in most patients with cystic fibrosis, particularlypatients who are pancreatic insufficient. Low vitamin levels are associated with poorer clinicalstatus and reduced lung function. The evidence suggests that with modern therapy, onlysupplements of vitamin A, D and E are required in uncomplicated cystic fibrosis.17

The importance of nutritional status in the long-term survival of patients with CF is welldocumented.32 Some patients require additional oral or enteral feeding to maintain their weight.However, one study states that although the use of enteral tube feeding and oral calorie

supplements in cystic fibrosis is widespread, systematic reviews of the evidence show manytrials to be of poor quality and evidence for the use of these forms of treatment is lacking. 17

Liver disease often presents at puberty and the focus of treatment is increasing as lifeexpectancy improves. Hyperviscous bile accumulates in the biliary tree and stimulates focalfibrosis. Clinical signs of liver disease often appear late by which time cirrhosis may beestablished. The table below highlights some gastrointestinal problems and their management.

Table 12. Gastrointestinal problems and management

Extra Pulmonary Disease Intervention Evidence

Exocrineinsufficiency

Enteric coated preparations ofpancreatic enzyme supplementsimprove fat absorption compared tonon-enteric coated preparations

Evidence from RCT but dosespecific37

Pancreas

Pancreatitis Management as for other causes,Pancrex powder

Uncommon, only occurs in patientswith pancreatic exocrine sufficiency

Meconium ileus Gastrograffin enemaSurgery

Smallbowel

Distal intestinalobstruction

Bowel cleaning agents

Fatty liver Ursodeoxycholic acid, taurines Consensus lacking on the use oftaurine

Liver

Cirrhosis Severe cases may needtransplantation

Source: Adapted from a literature review26


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5.5. ComplicationsWith increasing age patients may suffer a variety of complications including diabetes mellitus(DM), osteoporosis, nasal polyposis, inflammatory arthritis and male infertility may also becomeapparent in later life.

Glucose intolerance and CF related diabetes (CFRD) are age-related complications. Evidenceis accumulating that CF-related diabetes contributes to the lung disease and worse outcomes. 38

As survival increases the prevalence of cystic fibrosis related diabetes (CFRD) increases asindividuals are at increasing risk of the complications of diabetes. The principles of insulin andnutritional therapy in CFRD differ to those with type I or type II diabetes.39

People with cystic fibrosis may develop low bone mineral density from either osteoporosis orvitamin D deficiency osteomalacia. The aetiology of osteoporosis in CF is multi-factorial butcentres on an uncoupling in the normal balance between bone formation and resorbtion.Corticosteroid use can accelerate bone loss.7

The majority of men with CF are azoospermic due to anatomical abnormalities of the vasdeferens. It has been quoted that at least 97% of men with cystic fibrosis are infertile.40

However, the management of male infertility has been transformed by the introduction of sperm

aspiration from the epididymis and intracytoplasmic injection into eggs (ISCI). It isrecommended that CF centres should have arrangements for referral of couples where the malehas CF. Some women with CF also have fertility problems however it is recommended thatthese women be managed in the same way as those who do not have CF.41

Many adults with CF, both men and women go on to have children however, issues surroundinginfertility and pregnancy are an important concern to adult patients with cystic fibrosis. There areimportant health issues to consider such as balancing the treatment of CF with spending timeand caring for a child, and issues of life expectancy. 8

Table 13. Common complications of cystic fibrosisCommon Complications Management Evidence

DiabetesInsulinContinue high fat dietOral hypoglycaemic agents rarelyuseful

Nasal polyps Topical SteroidsAntibiotics

Surgery may have medium termbenefit but recurrence is common

Osteopenia PreventionWeight bearing exerciseHigh dairy intakeVitamin D and K therapyBisphosphonates if severe

Some treatments lacking consensus

Bilateral absence of vasdeferens

Sperm aspirationAssisted fertilisation techniques

Source: Adapted from a literature review26

5.6. Advances in treatmentOne review has stated that although conventional treatment has improved greatly over the pastfew decades, current treatments at best, slow the decline in lung function. It is suggested thatnewer approaches such as gene and small molecule based treatments may have more potentialto halt disease progression.26

A recent review focussing on new pulmonary therapies for cystic fibrosis states that improvedknowledge of the molecular mechanisms underlying cystic fibrosis has led to a wide spectrum of


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new treatment approaches. It also suggests that although gene therapy does not appear to be atherapeutic option in the immediate future, cystic fibrosis transmembrane regulatorpharmacotherapy is currently being developed as an alternative to reduce cystic fibrosistransmembrane regulator degradation or improve its function. It concludes by stating that whileclinical efficacy still has to be proven in large clinical trials, there is considerable hope that cysticfibrosis therapy will move from addressing the downstream sequelae of the cystic fibrosistransmembrane regulator defect to a more causal approach in the near future to give greater

spectrum of treatments.

42

Another author states that there are many challenges ahead for patients with CF and the clinicalteams caring for them. New ideas and novel therapies will be presented by professionals andpatients alike, with concepts as diverse as telemedicine and complementary therapy.Pharmacological and technological advancements are changing the delivery of health careacross the world of CF and CF teams therefore have a responsibility to stay up to date andensure they work in partnership with patients to provide the best care. 8

5.7 Cost EffectivenessThere are relatively few papers that directly address cost effectiveness in CF, particularly in thecare of adults with CF. One paper explains that the cost effectiveness of treatment for cystic

fibrosis cannot be assessed in the same way as a disease which has a well documented pre-intervention scenario and easy to record end points. In cystic fibrosis there are no clearbeginnings or ends. One must arbitrarily define clinical stability and a patient clinically stabletoday may not be tomorrow. The end point is a change in respiratory function tests, whichseveral independent variables can affect on any particular day, including the incidence ofrespiratory exacerbations, laboratory changes in sputum qualities, which may or may not beclinically relevant, and the subjective observations of patients.

One study recommended that health economic analyses of future therapeutic CF technologiesshould consider indirect costs more often than they have to date and that the perspective ofhealth economic studies should be stated explicitly and always include the societal perspective.They also state that

More economic studies should be based on a controlled and preferably randomized, design. The observation period must be long enough to identify long-term effects of interventions. A greater number of effectiveness studies should be performed to determine costs and

outcomes of therapies applied under everyday life conditions for CF. International comparison studies should identify the influence of different healthcare systems

on the costs and outcomes of interventions. 43

One study carried out was a cost effectiveness analysis of home versus hospital basedtreatment with IV antibiotics for respiratory exacerbations in adult patients with CF in the UK.The study explained that hospital based treatments could result in a greater improvement oflung function compared with home based treatments but that home based treatments mightreduce treatment costs and reduce risks of cross infection. This was a retrospective cohort

study. The primary outcome measure was percentage change in FEV(1); "effectiveness" wasdefined as maintenance of baseline average FEV(1) over the one-year study period. In terms ofeffectiveness of treatment, the mean percentage improvement in FEV1 from the baseline bestwas statistically higher for hospital patients than home patients. At the end of one year, therehad been a mean percentage decline in FEV 1 for home-treated patients but and improvement inhospital treated patients 10.1% (CI 2.9 to 17.2, p=0.003. Treatment was deemed effective inmore hospital (58.8%) than home patients (42.6%).

Clinical outcomes and resources use were combined with unit cost data in an incrementaleconomic analysis for 116 patients that received 454 courses of antibiotics.


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The cost of hospital treatment was higher than home treatment (mean difference of 9,005Confidence Intervals 33,307-14,000 P


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6. WHAT ARE THE EXISTING SERVICES, GAPS & MODELS OF CARE?This is the third of the three main elements of the health needs assessment. This section detailsthe current cystic fibrosis services available in the South East Coast, the usage of the services,access, views and experiences of stakeholders and a discussion of models of care for adultswith cystic fibrosis.

6.1 Organization of CF Care in the UK

Cystic fibrosis is one of 35 services in the Specialised Services National Definitions Set whichare defined as high cost, low volume interventions and treatments that are provided in relativelyfew specialist centres. One source states that these complex and high cost services have oftenbeen established on an ad hoc basis, without planning or co-ordination.46

Cystic fibrosis is a chronic condition and inpatient care is mainly reserved for those withcomplicated disease, severe disease or lacking social support to undertake treatment athome.17 The majority of care of cystic fibrosis patients takes place in hospital outpatients and inthe community and includes intravenous antibiotic treatment, nebulised antibiotics, mucolytics,physiotherapy and nutritional management, for which the evidence has been presented in theprevious section. Outpatient care is usually undertaken by a multidisciplinary team includingphysicians, specialist nurses, physiotherapists, dieticians and social workers.

CF patients were historically looked after by local paediatricians and physicians. The CF Truststates that care improved when specialist CF centres were established, although they were notconsistently available throughout the UK. In additThe CF Trust states that even now, access toCF specialist centres is patchy despite recommending that full adult care should be delivered bythe multidisciplinary team from an adult Specialist CF Centre as this is the only model ofdelivery of care recognised for adults with Cystic Fibrosis by the CF Trust. 47

Adult patients with CF commonly receive their care at a Specialist CF Centre, however there aresome instances where patients choose to attend clinics in a local general hospital. Patientsattending a local CF Clinic may also seen by staff of the Specialist CF Centre who travel out tothe local clinic to provide an outreach service. In addition the CF Trust states that the essential

requirement for routine outpatient and inpatient care and home support should be of the samestandard as those at the specialist CF Centre. 22

The recommended specialist cystic fibrosis team should consist of a paediatrician or adultphysician, a clinical nurse specialist, physiotherapist, dietician, social worker, psychologist andpharmacist. 22


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6.2 Cystic Fibrosis care in the South East Coast AreaThe South East Coast has 8 PCTS: Medway, Eastern & Coastal Kent, West Kent, Hastings &Rother, East Sussex Downs & Weald, Brighton & Hove City, West Sussex, and Surrey. EachPCT has a least one acute trust within the PCT area. The current PCTs with main acuteproviders are shown in the figure below.

Figure 10. Map of South East Coast region by PCT showing Acute Hospitals

Source: South East Coast Strategic Health Authority

Historically, there has been a variation in commissioning of CF services, partly due to the

services being commissioned across a large number of PCTs. Recently, the South East CoastSCG held locality meetings in Kent, Surrey and Sussex to engage with stakeholders in CFservices to record information on current services. The table below is a summary of theresponses from the locality meetings. The term shared care has not been defined and maydiffer from the definition from the CF Trust.

The main providers of Adult Specialist CF care to residents of the South East Coast Region areKings College Hospital in South East London, The Royal Brompton Hospital in South West


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London and Frimley Park Hospital in Surrey. Southampton also has an Adult Specialist CFCentre. There are currently no recognized adult specialist CF centres located in Kent or Sussex.

11 Acute Trusts were included in the data collection in the South East Coast. Of these, 5 Trustsstate that they do not provide any adult services and 4 Trusts state that they care for adult CFpatients. Frimley Park, the only adult CF Specialist Centre in the southeast coast region, caresfor approximately 100 adults. The remaining three trusts all have been 7 and 25 patients: East

Kent (11), Brighton & Sussex (20-25) and East Sussex (7-10).

Kent & Canterbury Hospital has approximately 11 adult cystic fibrosis patients and they areseen by shared care with Kings College Hospital and The Royal Brompton Hospital. Medwayadult patients are seen by shared care provided by Kings College Hospital although the exactnumber of patients is to be confirmed. The Royal Sussex Hospital in Brighton provides care forapproximately 20-25 adult patients. Some adult CF patients are seen at Kings College Hospitaland The Royal Brompton. St Leonards on Sea, East Sussex provides care for 7-10 adultpatients with a shared care arrangement with Kings College Hospital. Frimley Park is aspecialist adult Cystic Fibrosis Centre and provides care for over 100 CF patients.

Approximately half the hospitals in the South East Coast region state that they do not provide

any adult cystic fibrosis services and the patients are seen solely by specialist adult cysticfibrosis centres, which is in line with recommendations from The CF Trust. In Kent, these Trustsare Dartford & Gravesham and Maidstone& Tunbridge Wells, in Surrey these are Ashford andSt Peters and in Sussex these are Royal West Sussex and Worthing & Southlands.

Information from The Royal Brompton Hospital shows that they care for approximately 650 adultcystic fibrosis patients in total, from a wide area. They provide 4 cystic fibrosis outpatient clinicsper week. The CF services at the Royal Brompton include Consultants, Specialist Nurse,Dietician, Physiotherapist, Psychotherapist, Occupational Therapy, Homecare, telephone clinicand 365 days/year 24/7 oncall support. The CF service is also able to provide specialist supportfor CF related liver disease, CF diabetics and specialist transplant assessment services.Information provided from the Stakeholder locality meetings indicates that 70 patients are caredfor through

shared care

arrangements.

As can be seen from the table below, due to some of the data being incomplete it is difficult todraw conclusions regarding the level of services being provided and to draw comparisons withthe recommended standards of care particularly those issued by the CF Trust. However it canbe seen that there is a large variation whether the patients are seen at a non-specialist orspecialist adult centre or both. There is variation in the number of patients being seen at eachhospital and also variation in the service provision and presence of an appropriate multi-disciplinary team. Some adult patients in the South East Coast appear to attend adult SpecialistCentres for their care. A few adult patients are seen by the Specialist CF Team in an Outreachclinic by a team from one of the specialist CF Centres in their local hospital. Some adultpatients are seen by a shared care arrangement where they are seen by the specialist CFclinic but also at their local CF clinic. There are also several patients primarily at Brighton &

Sussex Hospitals who are seen solely by their local CF team with no apparent arrangement withspecialist centres. Several district general hospitals stated that they provide shared care with aspecialist centre, however for adults, this model of care is not recognized by the CF Trust. Themodels of care will be discussed in further detail.


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6.3 Inpatient usage of CF servicesThe next section details service uptake for inpatient services. The inpatient data in the followingsection has been shown by county of residence.

The following table and graph shows the hospital type where each admission occurred for theresidents of Kent, Surrey and Sussex. Overall across the SEC, 63% of all adult CF admissionswere to adult Specialist CF centres, recognized as such by the Cystic Fibrosis Trust.48 These

were Kings College Hospital, The Royal Brompton, Papworth Hospital, Frimley Park andSouthampton. Of the three counties, Kent had the highest proportion of patients admitted to aSpecialist CF Centre and residents from Sussex had the highest proportion of admissions tonon-specialist CF centres although the proportion admitted to adult specialist centres for allthree areas are quite similar.

Overall 30% of all inpatient admissions were to hospitals in the South East Coast region thatwere not Specialist CF centres. There is a variation where patients are admitted. Surrey hadalmost 20% of patients admitted to a non-Specialist CF Centre in the South East Coast regionand 20% to London Hospitals whereas Sussex had a nearly 40% of patients admitted to a non-Specialist CF Centre in the South East Coast region.

Table 15. Inpatient activity for adult cystic fibrosis patients (aged 16 and over) accordingto place of admission by county of residence for 2006-07

Sussex Kent Surrey Total

Adult Specialist CF Centres 167 142 35 363

London Hospitals 2 23 10 35

South East Coast Hospitals 100 53 8 161

Other Hospitals 1 0 2 3

Total 270 218 55 543Source: SEC SEG

Figure 11. Proportionate distribution of inpatient admissions according to hospitaltype by county of residence, 2006-07

Source: SEC SEG

Percentage of inpatient admissions by county and hospital location

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Sussex Kent Surrey Total

County of residence

Percentage

Other Hospitals

South East Coast Hospitals

London HospitalsAdult Specialist CF Centres

Percenta e of in atient admissions b count of residence and hos ital location of admission


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The following table provides the inpatient admissions in greater detail by PCT and providerTrust where the admission occurred. West Sussex PCT had the highest number of residentshaving inpatient admissions (139 admissions) and Hastings and Rother PCT had the lowestnumber of residents having inpatient admissions (6).

The two adult Specialist CF Centres that had the highest number of admissions were The RoyalBrompton and Kings College Hospital. The Royal Brompton Hospital had the highest number of

admissions overall and of all the specialist adult cystic fibrosis centres (183). Kings CollegeHospital had the second highest number of admissions (92).

The South East Coast hospitals had a greater number of admissions (161) than the LondonHospitals (35). Brighton & Sussex University Hospitals had the highest number of inpatientadmissions of all the South East Coast hospitals (59), followed by East Kent Hospitals (40).


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Table16. Inpatient admissions in adult cystic fibrosis patients (aged 16 and over) by PCTof residence and place of admission for 2006-07

BRIGHTONANDHOVECITYPCT

EASTSUSSEXDOW

NS&WEALDPCT

HASTINGSANDROT

HERPCT

WESTSUSSEXPCT

EASTERN&COASTALKENTPCT

MEDWAYPCT

WESTKENTPCT

SURREYPCT

Total

Adult Specialist CF Centres

KING'S COLLEGE HOSPITAL NHS TR 14 2 4 16 15 41 92

ROYAL BROMPTON AND HAREFIELD NHS TR 32 11 2 64 21 5 33 15 183

FRIMLEY PARK HOSPITAL NHS TR 4 4 1 1 19 29

PAPWORTH HOSPITAL NHS FOUNDATION TR 4 9 1 14

SOUTHAMPTON UNIVERSITY HOSPITALS NHS TR 9 17 26

Subtotal 45 25 4 93 47 20 75 35 344

London Hospitals

BARTS AND THE LONDON NHS TR 1 13 1 15

CHELSEA AND WESTMINSTER HOSPITAL NHS FOUNDATION TR 1 1

EPSOM AND ST HELIER NHS TR 1 1

EPSOM AND ST HELIER UNIVERSITY HOSPITALS NHS TR 4 4

GUY'S AND ST THOMAS' NHS FOUNDATION TR 1 2 3

HAMMERSMITH HOSPITALS NHS TR 1 1 2

KINGSTON HOSPITAL 2 2

THE LEWISHAM HOSPITAL NHS TR 5 5

UNIVERSITY COLLEGE LONDON HOSPITALS NHS TR 1 1

ST GEORGE'S HEALTHCARE NHS TR 1 1

Subtotal 0 1 0 1 2 0 21 10 35

South East Coast Hospitals

ASHFORD AND ST PETER'S HOSPITALS NHS TR 2 2

BRIGHTON AND SUSSEX UNIVERSITY HOSPITALS NHS TR 16 14 29 59

EAST KENT HOSPITALS NHS TR 40 40

EAST SUSSEX HOSPITALS NHS TR 20 2 22

MAIDSTONE AND TUNBRIDGE WELLS NHS TR 1 11 12

MEDWAY NHS TR 1 1

QUEEN VICTORIA HOSPITAL NHS FOUNDATION TR 1 1

ROYAL SURREY COUNTY HOSPITAL 6 6

ROYAL WEST SUSSEX NHS TR 4 4

SURREY AND SUSSEX HEALTHCARE NHS TR 5 5

WORTHING AND SOUTHLANDS HOSPITALS NHS TR 3 6 9

Subtotal 19 35 2 44 40 1 12 8 161

Other Hospitals

HINCHINGBROOKE HEALTH CARE NHS TR 1 1

PORTSMOUTH HOSPITALS NHS TR 1 1

UNIVERSITY HOSPITAL S. MANCHESTER NHS FOUNDATION TR 1 1

Subtotal 0 0 0 1 0 0 0 2 3

Total 64 61 6 139 89 21 108 55 543

Source: SEC SCG


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6.4 Comparison with literature on Inpatient ServicesA literature search was conducted on usage of CF services in the UK and the proportionattending a specialist adult CF centre. Surveys of adults with CF in the UK were carried out onall adult patients registered with the CF Trust in 1990, 1994 and 2000.25 The survey found thatthe proportion of patients attending a specialist centre for adults increased from 50.4% in 1990to 74.6% in 2000. Approximately 60% of inpatient admissions were to Adult Specialist CFCentres in the South East Coast residents, which is lower than the 74.6% quoted in the

literature.

6.5 Outpatient utilization of CF servicesPatients with chronic diseases mainly use outpatients and primary care services to managetheir ongoing care, although a major feature is self-care. For those with cystic fibrosis a largeproportion occurs on an outpatient basis and requires a multi-disciplinary approach.17

Due to the complex nature of clinical coding and the way data is recorded it had been difficult toobtain detailed outpatient data. Therefore a literature search was also carried out and contactwas made with the CF Trust. Data on outpatient activity was obtained from the Cystic FibrosisTrust Database from 2004 however there are several limitations to this data. The data is unableto be segmented for adults and children, therefore the data is presented for all ages. It is also

not known where the patients attended for their appointment and it would be particularlyimportant to know if they were attending a non specialist or specialist centre. It would also beuseful to know whether any of the attendances were an Annual Review. The following tableshows the number and average number of outpatient attendances of CF patients of all ages byPCT. As can be seen from the table this varies from an average of 4.5 attendances for patientsin Surrey PCT to and average of 1.8 attendances for patients in Hastings & Rother PCT in 2004.

Table 17. Number of CF patients of all ages and number of OP visits by PCT of residencein 2004

Source CF Trust

The total number of outpatient attendances, with the frequency of patients for each number of

outpatient attendances is shown in the graph below. The graph shows that for residents inWest Kent PCT, Eastern and Coastal PCT, East Sussex Downs & Weald PCT, Hastings &Rother PCT and Medway PCT had the highest frequency of patients who only had oneoutpatient attendance in the year of 2004. Eastern & Coastal Kent and West Kent PCT bothhad more than 10 patients who did not appear to have any outpatient attendances in 2004.Surrey PCT, West Sussex PCT and Brighton & Hove all had the highest frequency of patientswho had four attendances in 2004 which is inline with the guidance from the CF Trust wherepatient should expect to have a quarterly outpatient review stated in The CF Trust Standards ofCare.22

Brighton&Hov

e

City

EastSussexDo

wns

&Weald

Eastern&Coas

tal

Kent

Hastings&Rother

Medway

Surrey

WestKent

WestSussex

SouthEastCoa

st

total

Number of patients 16 58 79 28 18 180 112 79 570Number of OP visits 61 189 186 51 39 813 308 332 1979

Average number of OP visits 3.8 3.3 2.4 1.8 2.2 4.5 2.8 4.2 3.5


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Figure 12. Number of Outpatient attendances according to PCT of residence for CFpatients of all ages in 2004

Source: CF Trust

6.5.1 Other Outpatient dataA previous piece of work was carried out in Kent regarding the evidence base for cystic fibrosisservices in Kent, Surrey & Sussex. The paper documented outpatient data specifically for Kentin 2006/07. The numbers of adult outpatient attendances were estimated to be an average of4.4 attendances for ECKPCT, 6.5 for Medway and 5.2 for WKPCT. However, there are severallimitations to this data. The methodology used to obtain these figures is based on the patientswho were admitted as inpatients in that year, therefore if a patient had not been an inpatient inthat time period, there would not be any data available on their outpatient activity. It is also notknown which outpatient clinics were used to obtain this data. These figures are also higher than

the figures provided by the CF Trust, however the figures are specifically based on adult data incomparison with that provided by the CF Trust which was for adults of all ages.49

6.5.2. Comparison with literature on Outpatient appointmentsA European consensus on standards of care for patients with cystic fibrosis states that foroutpatient care, patients should be seen every 1-3 months.41 Standards of care from The CysticFibrosis Trust state that cystic fibrosis patients should have an annual review to identifysuboptimal treatment, and that the annual review permits formulation of an individual treatmentplan, provides an update for parents, patients and colleagues and presents an opportunity for

Number of outpatient attendances for cystic fibrosis patients of all ages in 2004 by

PCT - Soruce CFTrust Database

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10

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Number outpatient attendances in 2004

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erofpatients

Brighton & Hove City PCTEast Suss ex Dow ns & Weald PCTEastern & Coastal Kent PCT

Hastings & Rother PCTMedw ay PCT

Surrey PCTWest Kent PCT

West Sussex PCT


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review by the Consultant at the Specialist CF Centre responsible for long term care. Regularroutine outpatient reviews should take place frequently, ideally every 6-8 weeks and at leastevery 3 months.22 Another study states that on average patients of all ages attend outpatients4.6 times annually in the UK.17

It has been shown from the data presented, that there is a large variation in the number ofoutpatient attendance from 4.5 attendances for patients in Surrey PCT to and average of 1.8

attendances for patients in Hastings & Rother PCT in 2004. The majority of PCTs had anaverage number of outpatient attendances below the recommended guidelines.

6.6 General Practitioners and Community CareA survey of adults with cystic fibrosis also looked at general practitioner care of adults withcystic fibrosis. The findings showed that although general practitioners are frequently involved inrepeat prescription supply for people with CF (97%), the proportion relying on the GP for familyplanning and advice fell between 1994 and 2000. Adult patients are having to attend their GPmore frequently for repeat prescriptions since 1990 - a higher proportion now have to attendevery 1 to 2 weeks.25

Although a significant amount of care is provided in the community it has been difficult to

quantify this and therefore has been not been included in this needs assessment.


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AccessThe following map shows the distribution of cystic fibrosis patients of all ages in South East Coast Region by postcode area. This data hasbeen obtained from the CF Trust. As can be seen the population density of CF patients is varied. As would be expected, there are a higherdensity of patients around the major towns of the region.

Figure 13. Distribution of CF patients of all ages in SE Coast by postcode in 2004

Source: CF Trust data mapped by Kent PH HIS


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6.7.1. Journey TimesThe following data looks at access in terms of journey times to the Specialist Adult CF centres.The postcode areas were ranked in descending order according to the number of patientsresident. The locations shown in the table were chosen as they had the highest number ofresidents with CF. This data has been obtained from the CF Trust and is based on CF patientsof all ages. The table also gives the mileage to the nearest Adult Specialist CF Centre andshows that a quarter of the 11 areas with the greatest number of CF patients live over 50 milesaway from their closest adult specialist CF centre by road.

Table 18. Travel distance by road from postcode locations ranked with highest number ofresidents with CF of all ages

PCT Post Code Location No. of pts with CF

ClosestAdult CFcentre byroad inmiles

West Kent PCT ME15 Maidstone 8 36

Brighton & Hove City PCT BN2 Brighton 7 53

West Kent PCT DA12 Gravesend 7 25

Surrey PCT GU15 Camberley 7 4West Sussex PCT PO20 Chichester 7 45

Surrey PCT GU21 Woking 6 13

West Sussex PCT RH11 Crawley 6 31

West Kent PCT TN15 Sevenoaks 6 26

Medway PCT ME8 Gillingham 5 37

Eastern & Coastal Kent PCT CT15 Dover 5 70

East Sussex Downs & Weald PCT BN23 Eastbourne 5 62Source: Data on number of residents obtained from CF Trust)

One survey of cystic fibrosis patients from the literature states the proportion of patients havingto travel less than 50miles to attend a specialist clinic had not increased since 1994 (76.4% in2000 versus 79.6% in 1994).25 One source states that in terms of distance travelled to hospital,4.7% of parents reported all hospitals they attended to be over 50 miles from their home, and15.4% reported all hospitals they attended to be over 25 miles from their home.

The following graphs show an estimate of the return journey times to four Adult Specialist CFCentres: Kings College Hospital, The Royal Brompton, Frimley Park and Southampton. The carjourney estimates are based on a route planner and do not take into account any congestion ortraffic problems. The train times are based on the journey time from the main station with thepostcode area to the station nearest the hospital and exclude any walking time. 50

The first graph shows an estimate of the journey times from locations in Kent to the SpecialistAdult Cystic Fibrosis Centres. The average return journey time from locations in Kent to Kings

College Hospital by train is estimated to be around 2.5 hours, approximately the same averagejourney time to The Royal Brompton. This ranges from around 2 hours from Gravesend andSevenoaks by train to around 4 hours from Dover. Travel times to Frimley Park andSouthampton are much longer.


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Estimate of Return Journey Time from Sussex locations to Adult Specialist CFCentres

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Brighton Chichester Craw ley Eastbourne

Location

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Kings Time on train

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Frimley Park Time on train

Frimley Park Time by c ar

Southampton Time on Train

Southampton Time by car

Figure 14. Estimate of return journey time from Kent locations to Adult SpecialistCF Centres

Source: Travel times estimated using a journey planner

The following graph shows the return journey times from locations in Sussex. The averagejourney time by train to The Royal Brompton and Frimley Park is almost 3 hours. The journeyfrom Chichester to Southampton is approximately 2 hours. The journey time from Eastbourne toKings College Hospital or The Royal Brompton is almost 4 hours.

Figure 15. Estimate of return journey time from Sussex locations to Adult SpecialistCF Centres



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The following graph shows the return journey times from locations in Surrey. The closet AdultSpecialist CF Centre is Frimley Park which is located in Surrey. The average train time is under1 hour.

Figure 16. Estimate of return journey time from Surrey locations to AdultSpecialist CF Centres


As can be seen for the data there is a large variation in the journey times from locations inSouth East Coast. Although Frimley Park is the closest adult centre for patients in Surrey, thejourney times to adult specialist CF Centres from Sussex and Kent are significantly higher.

6.8. Stakeholder viewsThis following section looks at the range of stakeholders views that need to be taken intoaccount. This includes an expert patient representative, professionals, and a parent of a childwith CF who is approaching transition age.

6.8.1. Professional PerspectiveDuring this project I was able attend a meeting with the Associate Director of the South EastCoast Specialised Commissioning Group and three consultants who work at specialist CFcentres. Two of the consultants were adult CF consultants and one was a paediatric CFconsultant. Several of the consultants werea also involved with working with the Cystic FibrosisTrust. During this meeting several themes were discussed including transition care, capacityand travel to adult specialist centres.

All the consultants supported the view that care for adult cystic fibrosis patients should beprovided by dult Specialist CF centres as adult patients have an increasing number ofcomplications which are best managed at a centre with the appropriate skill mix, expertise andcritical mass of patients to give sufficient exposure and training. Clinical specialties are alsoavailable to provide input into the management of the adult CF patients.

They were keen to clarify the differences between full care, shared care, and outreach clinics asthese terms are often used interchangeably but mean very different things in terms of the actualcare received. Full care is where a patient always sees a specialist consultant and multi-disciplinary team from a Specialist Adult CF centre. Outreach care is where staff from thespecialist centre travel to a clinic at the local district general hospital and see patients there but


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on other occasions patients travel to the specialist centres. Shared care is where formalarrangements are made between the specialist CF centre and the district general hospital thatenables travel to be minimized for patients, however, this model of care is not recognized foradults by either the professional group or the Cystic Fibrosis Trust. 51

Although the consultants were fully aware that some patients were not willing to travel tospecialist adult clinics,

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HC Needs Assessment Adult CF

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