Cystic Fibrosis in Australia 2012
15TH ANNUAL REPORT
AUSTRALIAN CYSTIC FIBROSIS
DATA REGISTRY
CYSTIC FIBROSIS IN AUSTRALIA
2012
15th Annual Report from the Australian
Cystic Fibrosis Data Registry
© Cystic Fibrosis Australia 2013
This work is copyright. Apart from any use as permitted under the Copyright Act
1968, no part may be reproduced by any process without prior written
permission from Cystic Fibrosis Australia. Requests and inquiries concerning
reproduction and rights should be addressed to Chief Executive Officer, Cystic
Fibrosis Australia, PO Box 8007, Baulkham Hills NSW 2153.
Published by
Cystic Fibrosis Australia
Unit 26, 5 Inglewood Place
Norwest Business Park
Baulkham Hills NSW 2153
Australia
Website: www.cysticfibrosis.org.au
Email: [email protected]
ISSN 1447–3933
2
Preface
I am very pleased to deliver this 15th annual report from the Australian Cystic
Fibrosis Data Registry (ACFDR), for the year 2012.
First, I must commend the individuals and, in some cases, teams at the cystic
fibrosis treatment centres for their dedication to the task of entering data. Their
tireless commitment has this year enabled the registry to achieve its highest ever
levels of response. And, to their enormous credit, the job was completed within
the shortest ever period. An ambitious timetable was set to allow release of 2012
information ahead of the 10th Australasian Cystic Fibrosis Conference in
Auckland, just 8 months after the reference year. CFA is very proud to be
associated with achievement of this objective, being able to deliver timely
information to inform conference discussions.
CFA acknowledges that the burden of reporting to the Data Registry is real and is
in unfair day-to-day competition with the commitment to patient care.
Nevertheless, as other national CF registries have demonstrated, patient data are
an integral part of care quality management. Indeed, it is not uncommon to hear
overseas achievements with CF care being lauded in quality management circles.
Quality management is a stated priority for Australia's health system, with
associated data collection and analysis resources allocated. It must surely be time
for governments to recognise the outcomes that are possible in CF care in
Australia and take their rightful place as a funding partner for the ACFDR.
We are fortunate to have an excellent data management team involved in the
provision of reports and development of the registry over the past 15 years. Geoff
Sims and Leticia Good of Australian Clinical Registries deserve much credit for
their professional expertise, timely provision of a broad range of quality reports
and the manner with which they engage with our clinic partners, researchers and
other stakeholders.
Fifteen years of reporting have demonstrated the strength of partnership that has
been built between CF Centre Directors, industry sponsors who are
acknowledged in these pages, and CFA. On behalf of the 3156 patients who are
the subjects of this report, I sincerely thank CFA's partners in this important data
project.
David Jack
Chief Executive Officer
Cystic Fibrosis Australia
August 2013
3
Acknowledgements
Many thanks go to the ACFDR Advisory Committee, whose members have
shown a commitment to making the Registry a useful tool for improving health
and standards in the CF community. Members are:
Dr Scott Bell – The Prince Charles Hospital, Brisbane QLD
Dr Peter Bye – Royal Prince Alfred Hospital, Camperdown NSW
Dr Peter Cooper – The Children’s Hospital, Westmead NSW
Mr David Jack - Chief Executive Officer, Cystic Fibrosis Australia
Dr Adam Jaffe – Sydney Children’s Hospital
Dr James Martin – Women’s and Children’s Hospital, Adelaide SA
Dr Sarath Ranganathan – Royal Children’s Hospital. Melbourne VIC
Dr Phil Robinson – Royal Children’s Hospital. Melbourne VIC
Dr Gerard Ryan – Sir Charles Gairdner Hospital, Perth WA
Further acknowledgement must go to the following people and organisations:
Ms Ann-Maree Bosch for logistical arrangements and minutes of proceedings of
the Advisory Committee
Listening Post Pty Ltd, incorporating Prowess Development Pty Ltd for
database design and hosting services
Mr Geoff Sims and Miss Leticia Good of Australian Clinical Registries for
database management and reporting
Thanks also to the following organisations for their generous financial support:
Abbott Laboratories
Novartis
Pharmaxis
Vertex Pharmacuticals
4
Participating Centres
The ACFDR relies on the tireless work of people in the following CF Centres who
enter data and handle edit queries for quality control of the annual collection of
data:
New South Wales
Sydney Children’s Hospital
Royal Prince Alfred Hospital, Sydney
The Children’s Hospital, Westmead
Westmead Hospital – Adults
Gosford Hospital
John Hunter Children’s Hospital, Newcastle
John Hunter Hospital, Newcastle - Adults
Victoria
Royal Children’s Hospital, Melbourne
The Alfred Hospital, Melbourne
Monash Medical Centre, Clayton
Queensland
Royal Children’s Hospital, Brisbane
The Prince Charles Hospital, Brisbane
Mater Hospital – Children, Brisbane
Mater Hospital – Adults, Brisbane
Gold Coast Hospital – Southport
South Australia
Royal Adelaide Hospital
Women’s and Children’s Hospital, Adelaide
Western Australia
Princess Margaret Hospital for Children, Perth
Sir Charles Gairdner Hospital, Perth
Tasmania
Royal Hobart Hospital
Launceston General Hospital
CF Clinic, Burnie
Australian Capital Territory
The Canberra Hospital
5
6
Contents
Preface ....................................................................................................................................... 3
Acknowledgements .................................................................................................................. 4
Participating Centres ................................................................................................................ 5
1
People with cystic fibrosis ............................................................................................... 9
1.1 Overview......................................................................................................................... 9
1.2 Age distribution .............................................................................................................. 9
1.3 Adult marital status, education and activity ............................................................... 11
2
Diagnosis ........................................................................................................................ 13
2.1 Overview....................................................................................................................... 13
2.2 Age at diagnosis ........................................................................................................... 13
2.3 Presentation and diagnosis........................................................................................... 14
2.4 Pancreatic insufficiency ............................................................................................... 14
2.5 Genotyping ................................................................................................................... 14
3
Health and functioning................................................................................................... 17
3.1 Respiratory infections .................................................................................................. 17
3.2 Other medical complications ....................................................................................... 20
3.3 Lung function ............................................................................................................... 21
3.4 Nutrition: body mass index ......................................................................................... 24
4
Treatment of cystic fibrosis ........................................................................................... 29
4.1 Visits to clinics ............................................................................................................. 29
4.2 Therapy for cystic fibrosis patients ............................................................................. 29
4.3 Hospital treatment ........................................................................................................ 32
4.4 Home therapy ............................................................................................................... 33
4.5 Non-transplant surgery ................................................................................................ 34
5
Organ Transplants .......................................................................................................... 35
5.1 Patients assessed for transplant in 2012...................................................................... 35
5.2 Transplants during 2012 .............................................................................................. 35
5.3 Patients ever receiving a transplant ............................................................................. 35
6
Mortality ......................................................................................................................... 37
6.1 Deaths recorded in 2012 .............................................................................................. 37
6.2 Causes of death............................................................................................................. 38
Notes........................................................................................................................................ 39
Supplementary tables and technical notes ........................................................................ 39
Access to registry data ....................................................................................................... 39
Abbreviations...................................................................................................................... 40
7
8
1 People with cystic fibrosis
1.1 Overview
At 31 December 2012 the Australian Cystic Fibrosis Data Registry (ACFDR) held
records of 3,156 people with cystic fibrosis.
The mean age of the Registry population was 19.5 years at 31 December 2012.
This mean is slightly higher than previous years (19.1 in 2011, 18.8 in 2010 and
2009). The proportion of the registry population that is adult (18 years and over)
increased from 48.5 per cent in 2011 to 49.3 per cent in 2012, up from 25.6 per cent
when the registry commenced in 1998.
The median age of 17.7 years at 31 December 2012 is slightly higher than
previous years, 17.4 in 2011 and 17.2 in 2010. The median age for males (18.6
years) remained higher than that for females (16.8 years) in 2012.
The growth of 25 in the overall number of registrants in 2012 is about the same as
the excess of new diagnoses (66) over deaths (40) reported for the year. Indicating
that growth in numbers in the registry due to increased coverage has stabilised.
Although individuals who do not attend specialist CF treatment centre are not
included in the statistics in this report, the number of such persons is not large.
1.2 Age distribution
150
100
60
+
59
54
55
-
49
50
-
44
45
-
39
40
-
34
35
-
29
30
-
24
25
-
19
20
-
14
15
-
59
10
-
04
0
50
Number
200
250
ACFDR 2012: Age distribution by sex
Age(years)
Males
Females
9
ACFDR 2012: Age and sex of registrants at 31 December 2012
Age group
Standard demographic
age groups:
Males
Females
Persons
Per cent male
0 - 4 years
207
203
410
50.5
5 - 9 years
243
253
496
49.0
10 - 14 years
240
216
438
54.8
15 - 19 years
208
196
404
51.5
20 - 24 years
211
181
392
53.8
25 - 29 years
196
157
353
55.5
30 - 34 years
135
109
244
55.3
35 - 39 years
83
86
169
49.1
40 - 44 years
65
45
110
59.1
45 - 49 years
43
20
63
68.3
50 - 54 years
27
17
44
61.4
55 - 59 years
6
5
11
54.5
60 + years
4
8
12
33.3
0 - 1 years
71
70
141
50.4
2 - 5 years
193
194
387
49.9
6 - 11 years
290
267
557
52.1
12 - 17 years
257
258
515
49.9
Children and adolescents
811
789
1600
50.7
18 - 29 years
494
409
903
54.7
30 + years
363
290
653
55.6
Alternative CF age
groups and totals:
Adults
Total, all ages
857
699
1556
55.1
1,668
1,488
3,156
52.9
The lower table area shows age dissections that have been recommended for
international comparison of CF data. Many of the tables and charts later in this
report use this age dissection.
The proportion of males in the Australian CF population shows generally better
survival of males in the Australian CF population, which reflects international
experience.
At 31 December 2012, males made up 52.9 per cent and females 47.2 per cent of
the ACFDR population. This has remained a consistent proportion since
establishment of the registry in 1998. The proportion of males is higher amongst
the adult population (55%) than the child and adolescent population (51%).
The proportion of adults in the Registry as a whole was 49.3 per cent at 31
December 2012, up from 25.7 per cent in 1998. A lower proportion shown for the
Australian Capital Territory in 2012 reflects reporting issues.
10
ACFDR 31 December 2012: Adult status by State/Territory of residence
State or Territory of residence
Child/adolescent
Adult
Total
Per cent adult
New South Wales
492
443
935
47.4
Victoria
318
356
674
52.8
Queensland
395
385
780
49.4
Western Australia
178
171
349
49.0
South Australia
145
138
283
48.8
Tasmania
50
51
101
50.5
Australian Capital Territory
19
8
27
29.6
Northern Territory
3
4
7
57.1
Overseas
0
0
0
1,600
1,556
3,156
Total
49.3
ACFDR 1998-2012: Proportion who are adult
60
50
Per cent
40
30
20
10
19
98
19
99
20
00
20
01
20
02
20
03
20
04
20
05
20
06
20
07
20
08
20
09
20
10
20
11
20
12
0
Year
1.3 Adult marital status, education and activity
Management of cystic fibrosis allows an increasing proportion of persons with
the disease to continue with normal activities into their adult life, despite the
degenerative effects of the disease. While reporting of current personal
information is incomplete, gaps in coverage largely reflect lack of information
from the complete CF population at a small number of treatment centres.
Information compiled below is therefore likely to be reasonably representative of
the adult population with cystic fibrosis. Up to three CF centres have been
excluded completely from the analysis.
Around 38 per cent of male adult patients and 46 per cent of adult female
patients for whom marital status is known were in a formal or informal marriage
relationship. Approximately 1 in 7 adult CF patients had children.
11
ACFDR 31 December 2012: Marital status of adults(a)
Males
Marital status
Females
Number
Per cent
Number
Per cent
Married (includes de facto)
233
37.6
240
46.0
Not married
387
62.4
282
54.0
Unknown (includes not recorded)
119
100.0
Total
(a) Two CF centres were excluded from analysis
100.0
100.0
16.1
739
16.1
622
Many people with cystic fibrosis continue with education beyond senior
secondary school level, with 20 per cent of adult CF patients for whom
educational attainment was reported having university qualifications and a
further 19 per cent having completed other study beyond high school.
Approximately 65 per cent were in either full-time or part-time paid employment
during 2012.
ACFDR 31 December 2012: Educational attainment of adults(b)
Number
Per cent
Junior Secondary (Year 10)
122
14.4
Senior Secondary (Year 12)
308
36.5
Tertiary Certificate or Diploma
158
18.7
University Degree
171
20.2
3
0.4
Left school prior to Year 10
Not applicable (a)
Total reported
Unknown/not reported (incl. as % of total below)
Total
(b) Currently studying
(c) Three CF centres were excluded from analysis
22.4
100.0
Number
Per cent
395
36.5
Employed, part time paid
308
28.5
Pensioner
Others not in labour force (a)
Total reported
Unknown/not reported (incl. as % of total below)
Total
(a) includes homemakers, students
(b) One CF centre was excluded from analysis
12
100.0
244
Employed, full time paid
Unemployed
9.8
845
1,089
ACFDR 31 December 2012: Activity status of adults
Voluntary work only
83
5
0.5
138
12.8
88
8.1
147
13.6
1,081
100.0
413
27.6
1,494
100.0
2 Diagnosis
2.1 Overview
The number of new diagnoses of cystic fibrosis (CF) notified to the Registry for
2012 was 74, including 63 diagnosed less than one year of age. This is more than
the 72 overall and 58 infant diagnoses reported in 2011.
2.2 Age at diagnosis
Over 80 per cent of infant diagnoses were completed by three months of age,
assisted by neonatal screening programs that operate in all States and Territories
of Australia. A lower proportion of CF patients were diagnosed before one
month of age compared with 2011.
ACFDR 31 December 2012: Infant diagnosis age (months)
40
0
20
Per cent
60
(Per cent distribution)
0m
1m
2m
3m
4m
5m
6m
2012
1998 through 2010
7m
8m
9m
10m 11m
2011
Australian CF centres reported 2 new cases that were diagnosed in early
childhood (1 to 4 years), one aged 5 to 9 years, one aged 10 to 14 years, two aged
20 to 24 years, two aged 30 to 34 years and one aged 35 years or over.
13
2.3 Presentation and diagnosis
Approximately 65 per cent of new cases of CF diagnosed in 2012 included
neonatal screening as a mode of presentation. Respiratory symptoms were
reported in 12 per cent, and gastrointestinal symptoms in 5 per cent.
ACFDR 31 December 2012: Mode of presentation (a) by year of diagnosis
All years
2012
All years
1,602
43
53.2
65.2
Respiratory symptoms
463
8
15.4
12.1
Gastrointestinal symptoms
369
3
12.3
4.5
Meconium ileus
396
1
13.2
1.5
CF sibling
229
3
7.6
4.5
Minor manifestations
30
2
1.0
3.0
Pre-natal diagnosis
39
0
1.3
0.0
Infertility
14
0
0.5
0.0
Other
311
5
10.3
7.6
Unknown (b)
146
0
Number
Neonatal screening
2012
Per cent
Total
3,156
66
100.0
100.0
(a) More than one mode of presentation can be recorded for a patient so numbers in this section
add to more than the total number of registrants and percentage columns add to more than 100.0.
(b) Not known have been excluded when calculating percentages.
2.4 Pancreatic insufficiency
The overall proportion of patients who are pancreatic insufficient is 83 percent,
with no difference evident between the sexes.
2.5 Genotyping
Mutation information was reported for 86 per cent of 2012 patients. More
detailed tables follow in the following two pages.
ACFDR 31 December 2012: Whether patient genotyped, by year of diagnosis
All years
2012
All years
Number
Genotyped
Not genotyped
Unknown/Not recorded
Total
2012
Per cent
2,719
58
86.2
87.9
79
0
2.5
0.0
358
8
11.3
12.1
3,156
66
100.0
100.0
The genetic mutation F508del has been identified as at least one of the paired
mutations responsible for the inheritance of cystic fibrosis in 85 per cent of
patients for whom genotype details have been reported. Over half (52%) are
reported as homozygous for F508del.
14
G551D was the next most prevalent mutation, with 7.4 per cent of the CF
population having this mutation, mostly in combination with F508del or another
mutation.
ACFDR 31 December 2012: Genotype(a)
Mutation 1
F508del
G551D
G542X
R553X
R117H
Other
Total
Per cent
Mutation 2:
F508del
51.8
G551D
6.2
0.2
G542X
2.1
0.2
0.1
R553X
0.5
0.0
0.0
0.0
R117H
2.8
0.1
0.0
0.0
0.1
Other
7.2
0.2
0.2
0.0
0.1
11.3
13.0
0.5
0.3
0.2
0.2
0.8
1.7
0.0
0.0
0.0
0.0
0.3
85.3
1.2
0.6
0.2
0.4
Patients with missing genotype data for both alleles were excluded from analysis
12.3
Unknown / missing
Missing
(a)
100.0
ACFDR 2012: Genotype, major categories
14.7%
51.8%
33.5%
F508del/F508del
Other/Other (Identified)
F508del/Other
15
ACFDR 2012: Patients and alleles by genotype
Patient Number
Patient Per cent
Homozygous
Patient Number
Allele Number
2,316
85.3
1,406
3,722
G551D
200
7.4
5
205
G542X
78
2.9
2
80
R553X
20
0.7
0
20
R117H
91
3.4
3
94
N1303K
46
1.7
3
49
1717-1G>A
34
1.3
0
34
61+1G>T
25
0.9
0
25
W1282X
22
0.8
5
27
9
0.3
0
9
13
0.5
0
13
9
0.3
0
9
12
0.4
0
12
3849+10kbC>T
8
0.3
0
8
R1162X
8
0.3
0
8
1898+1G>A
7
0.3
0
7
3659delC
7
0.3
0
7
R334W
7
0.3
0
7
1078delT
6
0.2
0
6
G85E
7
0.3
0
7
R347P
6
0.2
0
6
E60X
8
0.3
0
8
S549N
5
0.2
0
5
V520F
7
0.3
0
7
2789+5G>A
5
0.2
0
5
Q493X
6
0.2
0
6
394delT
5
0.2
0
5
V470M
2
0.1
0
2
2789+2insA
7
0.3
0
7
2183AA>G
3
0.1
3
6
A455E
7
0.3
0
7
R347H
4
0.1
0
4
1525-1G>A
4
0.1
0
4
R1066C
3
0.1
0
3
1154insTC
3
0.1
0
3
R117C
4
0.1
0
4
S1235R
4
0.1
0
4
I1027T
2
0.1
0
2
S945L
2
0.1
0
2
3120+1G>A
0
0.0
0
0
2184delA
5
0.2
0
5
711+1G>T
0
0.0
0
0
Other
501
18.5
24(a)
525
Unknown
407
15.0
0
407
Missing(b)
54
2.0
0
54
2,715
100.0
1,451
5,430
F508del
I507del
D1152H
R560T
P67L
Total
(a)
(b)
16
Patients with two ‘other’ alleles. May not be homozygous to one another.
Missing values include patients with one missing allele and a second identified allele. Patients with two missing
alleles were excluded from analysis.
3 Health and functioning
Information in this chapter covers respiratory infections, medical complications,
lung function and nutritional measures. For most centres microbiology results
from respiratory samples were taken from data that are now being supplied to
the registry for each test or measurement. Two adult centres did not submit
microbiology information for their patients in 2012 and are excluded from the
analysis. All centres submitted data for multiple occasions where clinical
measures were taken - height, weight and lung function.
3.1 Respiratory infections
ACFDR 2012: Number of sputum and BAL/bronchoscopy cultures(a)
0-1
years
2-5
years
6 - 11
years
12 - 17
years
18 - 29
years
30 +
years
All
ages
Per cent of patients tested (b)
Sputum cultures:
None
40.4
31.9
10.5
1.6
0.5
0.6
8.9
1
12.8
18.6
17.1
13.8
22.0
17.8
17.8
2
13.8
10.3
9.6
13.2
16.5
17.2
13.5
3
5.3
14.0
12.6
13.8
14.9
12.9
13.3
4
10.6
8.6
10.9
14.3
10.7
14.1
11.7
5
6.4
5.0
8.7
10.7
10.7
11.0
9.4
6
2.1
2.3
10.0
7.5
7.8
7.7
7.2
7 or more
8.5
9.3
20.7
25.2
16.9
18.7
18.2
100.0
100.0
100.0
100.0
100.0
100.0
100.0
None
61.7
59.8
88.1
96.2
99.3
98.8
89.1
1
33.0
32.9
9.6
3.2
0.7
0.6
8.9
2
4.3
5.3
1.9
0.2
0.0
0.3
1.4
3 or more
1.1
2.0
0.4
0.5
0.0
0.3
0.6
100.0
100.0
100.0
100.0
100.0
100.0
100.0
Total
BAL/bronchoscopy:
Total
Number of patients
Patients tested (b)
Culture not done
94
301
469
441
551
326
2,182
47
86
88
74
146
142
583
128
370
539
505
686
461
2,689
13
17
18
10
11
7
76
Total patients
141
387
557
(a) Two CF centres were excluded from analysis
(b) By any method of obtaining culture.
515
697
468
2,765
Total reported
Not reported
17
The table on the previous page shows the distribution of CF patients according to
the number of both sputum and BAL/bronchoscopy samples examined during
2012. The latter method is used mainly on smaller children. Taking sputum
samples alone, about three quarters (73 per cent) of the patients tested had at
least two sputum samples in 2012. It can also be seen that respiratory cultures
were not reported for around 9 per cent of patients.
Denominators for percentages shown in the tables and chart in this section are
the total number of persons for whom any respiratory culture results were
reported, from any method of testing.
The most commonly identified organisms in respiratory specimens are various
species and forms of Pseudomonas. It can be seen that 53 per cent of patients tested
produced positive Pseudomonas aeruginosa cultures, with the mucoid form
showing in 36 per cent. Its prevalence is greater in adult patients. The table shows
that around 70 per cent of adult CF patients produced samples indicating the
mucoid form of Pseudomonas aeruginosa, two to three times the proportion for
adolescents and much higher than that for children.
ACFDR 2012: Pseudomonas infection by age group(a)(b)
0-1
years
2-5
years
6 - 11
years
12 - 17
years
18 - 29
years
30 +
years
All
ages
Per cent
Pseudomonas aeruginosa:
Mucoid
0.0
1.3
7.7
26.1
65.5
79.1
35.5
Rough/non-mucoid
1.1
8.0
19.4
21.8
32.1
28.5
22.1
Not differentiated
7.4
9.0
15.6
23.1
22.0
23.9
18.7
Any Ps aeruginosa
8.5
16.6
33.9
50.1
79.9
87.4
53.3
Pseudomonas other species
1.1
0.3
1.5
2.7
2.2
2.8
1.9
Patients tested
100.0 100.0 100.0
100.0
100.0
100.0
100.0
(a) Two CF centres were excluded from analysis
(b) Patient may have had more than one type of Pseudomonas infection. Percentages for individual types may add to
more than totals.
While prevalence of Pseudomonas organisms is lower in children than in adults,
though increasing with rising age, young children are more likely than adult
patients to produce cultures showing presence of Staphylococcus aureus. Half of all
child patients aged 6 years and over had this bacterial infection. Haemophilus
influenzae is also evident in relatively high proportions of child patients, highest
in children aged less than 6 years. This organism was present in over one quarter
of children in the 2 to 5 years age group but it is less prevalent at older ages. The
youngest age groups also had the highest proportions with positive cultures of
the bacteria Escherichia coli; 23 per cent for those aged less than 2 years being the
highest.
18
ACFDR 2012: Other respiratory culture by age group(a)
0-1 2-5
years years
6 - 11
years
12 - 17
years
18 - 29
years
30 +
years
Total
Per cent of patients tested (b)
Bacteria:
Staphylococcus aureus
43.6
39.2
50.1
48.8
37.2
34.0
42.4
Haemophilus influenzae
24.5
28.9
18.8
11.1
5.4
3.7
13.2
0.0
0.3
2.1
1.6
4.0
5.5
2.7
Burkholderia cepacia (Ps cepacia)
Stenotrophomonas maltophilia
2.1
3.7
14.1
15.2
3.3
7.7
8.7
23.4
6.6
4.1
3.9
0.5
0.6
3.8
MRSA (c)
3.2
3.0
3.0
3.4
3.3
5.2
3.5
Alcaligenes xylosoxidans
0.0
0.3
1.5
4.1
2.2
3.7
2.3
Serratia marcescens
1.1
1.7
0.6
1.4
0.4
0.0
0.8
Klebsiella (any species)
7.4
0.0
0.9
0.5
0.7
0.3
0.8
Non-tuberculous mycobacterium
0.0
0.3
0.6
2.5
1.8
2.5
1.5
Escherichia coli
Fungi:
Candida
17.0
19.3
29.4
37.0
20.0
19.6
25.2
Aspergillus (any species)
1.1
12.3
26.9
38.1
30.5
25.8
26.8
Scediosporium (any species)
0.0
0.7
5.8
6.8
4.2
4.3
4.4
Other organisms not listed above
28.7
26.6
28.8
20.9
13.4
13.8
20.8
Normal flora only
85.1
77.7
83.6
82.5
43.9
38.7
65.9
No
(a)
(b)
(c)
growth/sterile culture
9.6
8.0
6.8
4.1
2.9
4.3
5.2
Two CF Centres were excluded from analysis
Note: Patients may have multiple infections during the year. Percentages may add to more than 100.0.
Methicillin-resistant Staphylococcus aureus
80
60
40
20
0
Per cent of patients tested
ACFDR 2012: Prevalence of major organisms in lungs
0 to 1
2 to 5
6 to 11
12 to 17
Age group (years)
Ps. aeruginosa (any sp.)
Aspergillis (any sp.)
S. maltophilia
18 to 29
30 +
Staph. aureus
Candida
H. influenzae
19
3.2 Other medical complications
Although better coverage was evident in 2012 compared to 2011, complications
data were subject to about 18 per cent under-reporting in 2012, with six centres
reporting complication data for less than 50 percent of their patients. Prevalence
of medical complications increases with age in CF patients. For instance, over 40
per cent of adult patients aged 30 years or over suffer gastro-oesophageal reflux,
almost 30 percent suffer chronic insulin-dependent diabetes and 40 per cent have
osteoporosis or osteopenia.
The proportion for whom none of the selected complications shown in the
following table have been reported is over 80 per cent for children under 12
years, but declines to 21 per cent in CF patients aged 30 and over.
ACFDR 2012: Medical complications(a)
0-1 2-5
years years
6 - 11
years
12 - 17
years
18 - 29
years
30 +
years
Total
Per cent
Pulmonary:
Major haemoptysis
0.0
0.4
0.0
0.8
4.8
7.9
2.9
Massive haemoptysis
Therapeutic bronchial artery
embolisation
0.0
0.0
0.0
0.5
0.6
1.1
0.4
0.0
0.0
0.0
0.0
3.1
2.3
1.2
Pneumothorax
0.0
0.0
0.0
0.5
1.8
0.8
0.7
Any pulmonary above
0.0
0.4
0.0
1.9
7.7
9.6
4.2
Gastro-oesophageal reflux
4.3
3.3
7.4
11.2
35.6
43.4
21.3
- proven at endoscopy
0.0
1.5
4.1
5.5
5.1
5.6
4.4
Abnormal liver function test
0.0
3.3
8.7
14.2
28.1
26.2
16.9
Cirrhosis or portal hypertension
0.0
1.1
0.5
3.8
5.7
3.1
3.0
Pancreatitis
0.0
0.0
0.3
0.8
1.7
3.4
1.2
Any Gastro-intestinal above
4.3
5.9
15.1
22.1
52.5
55.5
31.8
Endocrine:
Chronic insulin-dependent
diabetes
Intermittent insulin-dependent
diabetes
1.1
0.0
2.0
16.4
16.9
28.5
13.0
0.0
0.7
1.3
1.6
2.4
2.8
1.8
Other glucose abnormality
0.0
0.4
3.8
12.0
20.9
16.9
11.6
Any Endocrine above
1.1
1.1
7.1
28.7
39.4
46.8
25.6
Osteoporosis
0.0
0.0
0.0
3.6
6.1
9.3
3.9
Osteopenia
0.0
0.4
1.3
8.5
23.5
30.7
13.6
Fracture this year
1.1
0.4
0.5
0.5
0.6
1.4
0.7
Any Osteo above
0.0
0.4
1.3
8.5
27.9
39.4
16.3
0.0
0.0
0.0
0.0
0.6
3.1
0.7
94.7
92.6
78.1
54.1
26.8
20.8
52.5
100.0 100.0
100.0
100.0
100.0
100.0
100.0
Gastro-intestinal:
Osteo:
Other:
Cancer
None of the above
Total reported
Number
Total reported
94
269
392
366
545
355
2,021
Unknown or not stated
16
22
24
19
86
81
248
Total patients (b)
110
291
416
385
631
436
2,269
(a) Six CF centres were excluded from analysis
(b) Patient may have had more than one complication. Percentages add to more than 100.0.
20
Although some prevalence of osteoporosis at younger ages is reported in the
table above, this is not displayed on the following chart because of uncertainty
about diagnosis at younger ages.
0
10
20
30
ACFDR 2012: Prevalence of major complications
0 to 1
2 to 5
6 to 11
12 to 17
Age group (years)
Chronic insulin-dependent diabetes
18 to 29
30 +
Osteoporosis
3.3 Lung function
Lung function measures compiled for this report are aligned with methods used
in the United States’ Cystic Fibrosis Foundation’s Patient Registry, that is the
lung function measure included for each patient is the average of the highest
FEV1 per cent predicted value recorded in each quarter of the year. ACFDR
reports prior to 2010 have compiled statistics of the best recorded lung function
(FEV1 per cent predicted) within the year, which are generally higher than those
compiled under the current method. Also since 2010, median values have been
presented in place of means, were appropriate. See ACFDR 2012: Technical Notes
on the Cystic Fibrosis in Australia website (see Notes and References Section at
end).
Table and charts in this section show distributions of lung function summarised
into categories, normal (90% of predicted FEV1 and above), mild impairment
(below 90% but not below 70%), moderate impairment (below 70% but not below
40%) and severe impairment (below 40%).
CF lung function measured as FEV 1 percent predicted is within the normal range
for young children but is lower than 70 per cent of normal, the level at which
moderate lung function impairment is experienced, in adult age ranges. Seven
per cent of children aged 6 to 11 years and fifteen per cent of older children and
adolescents have FEV1 values that are below 70 per cent of predicted values.
21
ACFDR 2012: Median lung function by age
100
80
60
40
20
0
5
10
15
20
25
30
Age (years)
35
40
45
Generally greater proportions of patients have severe lung function impairment
in successive older age groups. The proportion of adult male patients with severe
lung function impairment (21%) is greater than the proportion of female patients
in this severity category (14%).
ACFDR 2012: Lung function impairment by age and by sex
Severe
Moderate
Mild
Normal
Total
Severe
Number
Moderate
Mild
Normal
Total
Per cent
Age/sex group:
6 - 11 years
2
35
153
339
529
0.4
6.6
28.9
64.1
100.0
12 - 17 years
7
70
196
232
505
1.4
13.9
38.8
45.9
100.0
18 - 29 years
104
289
244
163
800
13.0
36.1
30.5
20.4
100.0
30 + years
132
245
127
50
554
23.8
44.2
22.9
9.0
100.0
Total measured
245
639
720
784
2,388
10.3
26.8
30.2
32.8
100.0
Males
159
321
384
407
1,271
12.5
25.3
30.2
32.0
100.0
86
318
336
377
1,117
7.7
28.5
30.1
33.8
100.0
Females
22
Similar proportions of male children and adolescents (56%) and females (54%)
have lung function within the normal range.
ACFDR 2012: Lung function impairment
Children and adolescents
60
Per cent of sex group
50
40
30
20
10
0
Severe
Moderate
Mild
male
Normal
female
However, around 58 per cent of male adults and 56 per cent of female adults
have lung function values below 70 per cent of predicted (moderate or severe
lung function impairment).
ACFDR 2012: Lung function impairment
Adults
Per cent of sex group
40
30
20
10
0
Severe
Moderate
male
Mild
Normal
female
23
3.4 Nutrition: body mass index
Methodological note
This annual report introduces changed methodology for compiling nutrition
outcome data, based on body mass index (BMI). Previous reports showed
median population values calculated from each patient's best measure for the
year. For this report, as has been the case since 2010 reporting of lung function,
median population values have been compiled from each patient's average of
best measures within each quarter of the year, where they are available. As for
lung function, this aligns ACFDR methods with those used in the United States’
Cystic Fibrosis Foundation’s Patient Registry and will facilitate comparisons with
US data.
Averages of quarterly best measures will be lower than the best for the year. This
causes a break in series from previous reported data. In the trend charts shown
in this section, the new methodology has been applied back to 2005, where a
series break is indicated by a vertical line. Users should not compare BMI
statistics published for previous years with those in this report. Assistance, if
required, is available from Cystic Fibrosis Austraia.
Data compiled from each patient's best measure for the year, including height
and weight percentiles and z-scores for children and adolescents comparable
with previously published data, will be available from Cystic Fibrosis Australia
on request.
Children and adolescents
ACFDR 2012: Child and adolescent BMI:
median percentiles by age group and sex
Males
2 - 5 years
69.4
6 - 11 years
54.4
12 - 17 years
36.9
Females
2 - 5 years
60.5
6 - 11 years
48.3
12 - 17 years
50.8
24
ACFDR 2012: Median BMI percentile
Children and adolescents
70
Median BMI percentile
60
50
40
30
20
10
0
2-
3-
4-
5-
6-
7-
8-
9- 10- 11- 12- 13- 14- 15- 16- 17-
male
female
BMI percentiles across individual ages show a generally consistent pattern of
lower values at higher ages, but a cohort of adolescent girls have BMIs above the
50th percentile.
Overall, just under half (48 per cent of males and 47 per cent of females) were
below the 50th percentile for BMI in 2012. The distribution is shown in the
following table.
ACFDR 2012: Child and adolescent BMI
percentile distribution by sex
Males
Females
Persons
Per cent
< 3rd
1.9
1.2
1.5
3rd - 4.99th
1.3
0.9
1.1
5th - 9.99th
3.8
3.8
3.8
10th - 24.99th
14.9
11.7
13.3
25th - 49.99th
25.8
29.4
27.6
50th - 74.99th
28.0
31.1
29.5
75th - 89.99th
16.1
15.8
15.9
90th - 94.99th
4.5
3.1
3.8
95th - 96.99th
1.9
2.1
2.0
>= 97th
1.9
1.0
1.5
100.0
100.0
Total
100.0
Number
Total
690
678
1,368
25
Median BMI percentile 1998-2012
Australian Cystic Fibrosis Data Registry
70
60
50
40
19
98
19
99
20
00
20
01
20
02
20
03
20
04
20
05
20
06
20
07
20
08
20
09
20
10
20
11
20
12
30
Year
Males 2-5 years
Females 2-5 years
Males 6-11 years
Females 6-11 years
Males 12-17 years
Females 12-17 years
BMI trend data since the beginning of the registry in 1998 are affected by the break in
series at 2005, described in the methodoligical note at the start of this section. A generally
upward trend is evident in the chart above, with males aged 12 to 17 years being the
exception over the years since 2005.
26
Adults
Adult Body Mass Index scores show 56 per cent of males and 53 percent of
females had an average quarterly BMI score in the range 20 to less than 25. The
proportion of females who had BMI scores below 20 (8 per cent) was more than
double the proportion of males. Over a quarter (27 per cent) of adult males had a
BMI above 25.
0
20
Per cent
40
60
ACFDR 2012: Adult BMI distribution
Under 18
18 to < 20
20 to < 25
25 and over
BMI range
male
female
ACFDR 2012 Adult BMI distribution
BMI range
Less than 18
From 18 to <20
From 20 to <25
25 and over
Total
18 - 29 years
5.0
Males: per cent
16.2
59.7
19.0
100.0
30 + years
1.0
10.0
50.8
38.2
100.0
Male adults measured
3.4
13.7
56.0
26.9
100.0
Females: per cent
18 - 29 years
9.8
24.3
54.3
11.6
100.0
30 + years
5.3
23.5
51.4
19.8
100.0
Female adults measured
7.9
23.9
53.1
15.0
100.0
27
An upward trend in adult BMI has been evident since the registry began in 1998,
as shown in the following chart. Tends earlier than 2005 are based on each
patient's best measure for the year, while those from 2005 to 2012 are based on
the average of quarterly best measures. See the methodological note at the
beginning of this section. The trend is evident, despite this break in series.
Median Body Mass Index 1998-2012
Australian Cystic Fibrosis Data Registry
25
24
23
22
21
19
98
19
99
20
00
20
01
20
02
20
03
20
04
20
05
20
06
20
07
20
08
20
09
20
10
20
11
20
12
20
Year
28
Males 18-29 years
Females 18-29 years
Males 30+ years
Females 30+ years
4 Treatment of cystic fibrosis
This Chapter describes the treatments and therapies recorded for patients in the
Australian Cystic Fibrosis Data Registry.
4.1 Visits to clinics
The average number of visits for both children and adolescents and for adults
was 4.6 in 2012. This figure should be treated with some caution as it may have
been affected by different practices in recording clinic visits at contributing
centres. The median number of visits to clinics is less likely to be so affected, and
stood at 4 for children and adolescents and also the same for adults in 2012.
4.2 Therapy for cystic fibrosis patients
Antibiotic therapy was prescribed for most CF patients, including 89 per cent of
the youngest patients, under age 2 years, and 96 per cent or more in all other age
groups. These proportions, and the numbers and proportions that follow in this
section, were compiled from therapy usage information supplied for 2,382
patients, approx. 75 per cent of all patients in the registry. Missing data was
concentrated more in adult than child and adolescent patients, as 2 major adult
centres, did not supply therapy data for 2012. One major and one smaller
pediatric centre supplied data for less than fifty per cent of their patients.
Oral antibiotic therapy was prescribed for 96 per cent of antibiotics users. Both
PRN (as needed) and continuous usage was prescribed for these patients at some
time during 2012, as shown in the following table. Higher proportions of
adolescents (41%) and very young children (46%) than those in other age groups
were prescribed oral antibiotics for continuous use.
ACFDR 2012: Oral antibiotic therapy - mode of use by age group (a)
0-1
years
2-5
years
6 - 11
years
12 - 17
years
18 - 29
years
30 +
years
All
ages
Per cent
Mode of use:
As needed (PRN)
57.1
76.2
75.1
71.7
85.3
85.5
77.7
Continuous
45.7
26.4
30.5
40.9
28.2
26.1
31.5
Mode of use unknown
Total oral antibiotics users(b)
0.0
1.0
1.1
1.2
0.4
0.3
0.7
100.0
100.0
100.0
100.0
100.0
100.0
100.0
Number
2,160
105
311
469
428
529
318
Four centres are excluded from analysis
More than one mode of use can be recorded so numbers add to more than 100.0. As well, mode of use was not
recorded for all patients where oral antibiotics were reported.
Total oral antibiotics users
(a)
(b)
29
Just over half (54%) of patients used inhaled antibiotics in 2012, with proportions
generally increasing with age.
ACFDR 2012: Inhaled antibiotics by age group(a)
0-1
years
2-5
years
6 - 11
years
12 - 17
years
18 - 29
years
30 +
years
All ages
Per cent
Inhaled antibiotics
Yes
11.4
30.7
47.9
59.2
66.3
67.9
53.7
No
62.9
53.0
35.9
27.2
23.2
21.2
32.4
Unknown(b)
0.0
0.0
0.0
0.2
0.5
0.6
0.3
Total antibiotics users
100.0
100.0
100.0
100.0
100.0
100.0
100.0
Total antibiotics users
105
313
474
Number
434
569
349
2,244
100.0
97.9
86.3
75.5
70.3
68.4
76.5
Continuous
0.0
3.1
14.5
24.9
32.1
33.3
24.9
Mode of use unknown
Total inhaled antibiotics
users(c)
0.0
0.0
0.4
1.2
0.3
1.3
0.7
100.0
100.0
100.0
100.0
100.0
100.0
100.0
12
96
227
377
237
1,206
Mode of use
As needed (PRN)
Total inhaled antibiotics users
(a)
(b)
(c)
Number
257
Four CF centres were excluded from analysis
includes not recorded
More than one mode of use can be recorded so numbers add to more than 100.0. As well, mode of use was not
recorded for all patients where oral antibiotics were reported.
Almost all CF patients use a range of other therapies to manage conditions other
than infections, and many take nutritional supplements. Therapies used by the
highest proportion of patients include pancreatic enzymes (85% of
children/adolescents and 82% of adults), vitamin supplements (78% and 76%
respectively), bronchodilators (38% and 69%) and salt tablets (50% and 22%).
ACFDR 2012: Other therapy by type(a)(b)
Child/adolescent
Adult
Number
Per cent
Number
Per cent
486
35.0
476
47.9
Pancreatic enzymes
1,174
84.6
813
81.8
Vitamin supplements
1,080
77.8
758
76.3
Bronchodilators
530
38.2
674
67.8
Corticosteroids inhaled
301
21.7
404
40.6
Corticosteroids oral
107
7.7
119
12.0
Insulin
100
7.2
198
19.9
Macrolides
180
13.0
661
66.5
Salt tablets
688
49.6
216
21.7
7
0.5
57
5.7
252
18.2
397
39.9
735
53.0
336
33.8
1,388
100.0
994
100.0
Dornase alpha
Antihypercalcaemics
Gastric acid secretion reducers
Other
Patients with therapies reported
(a)
(b)
30
Individuals may use more than one type of therapy; percentages by type of therapy add to more than 100.0.
Four CF centres were excluded from analysis
ACFDR 2012: Nutritional supplements by age group(a)(b)
0 to 4
years
5 to 9
years
12.5
29.7
35.3
Per cent
33.1
33.4
27.4
31.3
Nasogastgric
5.2
0.0
2.3
2.0
1.5
2.5
1.9
Total Parenteral Nutrition (TPN)
2.1
0.0
0.0
0.0
0.0
0.0
0.1
Gastrostomy tube/button
2.1
2.5
5.5
11.0
5.3
1.1
5.2
Nutritional supp. type unknown
0.0
0.0
0.0
0.0
0.0
0.0
0.0
Total using nutritional supplements
16.7
30.7
40.2
40.9
37.7
29.9
35.6
Not using nutritional supplements
Patients with nutritional
supplements reported
83.3
69.3
59.8
59.1
62.3
70.1
64.4
100.0
100.0
100.0
100.0
100.0
100.0
100.0
581
358
2,150
Oral (prescribed)
10 to 14 15 to 19 20 to 24 25 to 30
years
years
years
years
All
ages
Number
Total with nutritional supplements.
reported
Nutritional supps. not reported
96
283
433
399
22
41
56
58
40
15
232
Patients with therapies reported
118
324
(a) Four CF centres were excluded from analysis
(b) Individuals may use more than one type
489
457
621
373
2,382
Of the 2,382 patients for whom therapy was reported in 2012, 19 had commenced
oxygen therapy during 2012 and 24 remained on oxygen therapy commenced in
a previous year. The majority (15 and 22 respectively) were adults.
Seventeen patients commenced using non-invasive ventilation in 2012 and 11
had commenced in earlier years. Sixteen of those commencing during 2012 were
aged 18 years and over.
31
4.3 Hospital treatment
The manner of collection of hospitalisation data for the registry does not allow a
clear distinction to be drawn between ‘no hospitalisation’ and non-response in
relation to a patient. Two adult hospitals were excluded from the analysis of
hospitalisation because no data were available, but some under-reporting
elsewhere is possible.
A total of 1,351 patients, or 49 per cent of those attending hospitals that provided
data, recorded at least one hospitalisation during 2012. The tables and charts in
this section describe the hospital experiences of that population.
0
10
20
30
40
50
ACFDR 2012: Hospitalisations
1
2
3
4
5
6
7+
Number of hospitalisations
ACFDR 2012: Hospitalisation related to cystic fibrosis, respiratory causes(a)
0-1
years
2-5
years
6 - 11
years
Persons aged
12 - 17
18 - 29
years
years
30 +
years
All
ages
Per cent of persons in age group
Number of
hospitalisations:
None or none reported
63.1
58.4
55.7
49.3
56.8
61.7
56.6
1
21.3
26.1
27.7
23.9
21.9
18.6
23.4
2
7.8
10.1
7.9
10.5
9.1
7.9
9.0
3
5.7
3.9
5.0
8.5
4.2
6.0
5.5
4
0.7
1.0
2.2
3.5
3.9
3.6
2.9
5
1.4
0.3
0.5
1.4
1.6
1.1
1.1
6
0.0
0.0
0.7
1.4
1.0
0.6
0.8
More than 6
0.0
0.3
0.4
1.6
1.6
0.4
0.9
100.0
100.0
100.0
100.0
100.0
100.0
100.0
467
2,762
Total
Number of persons
Total
141
387
(a) Two CF centres were excluded from analysis
32
557
515
695
s
28
+
27
da
y
da
ys
s
Child/adolescent
14
-
3
71
06
da
y
da
ys
s
da
y
28
+
da
ys
27
da
y
14
-
3
71
06
da
ys
s
0
10
20
30
40
ACFDR 2012: Accumulated Hospital Days
Adult
4.4 Home therapy
20
40
60
80
ACFDR 2012: Home Therapy
0
Percent of patients hospitalised
Of persons known to have been hospitalised during 2012, almost half (48%)
accumulated at least 14 admitted days through the year, similar to the
proportions in recent years. Mean and median days, of 20 and 13 respectively for
these people, underline the fact that some CF patients spend considerable periods
of time hospitalised.
1
2
3
4
5
6
7+
Number of episodes
33
Charts in this section show the distribution of episode number and days for 402
persons (194 children/adolescents and 208 adults) for whom details were
provided of intravenous antibiotic treatment at home. Three centres, including 1
paediatric and 2 adult centres did not report home therapy treatment in 2012. At
the centres that did report, around 12 per cent of children/adolescents and 13 per
cent of adults received home therapy treatment in 2012, although some underreporting is suspected, particularly in adult centres.
28
+
14
-
Child/adolescent
da
ys
da
ys
s
27
3
da
y
da
ys
71
28
+
06
da
ys
da
ys
14
-
27
da
y
3
71
06
da
ys
s
0
10
20
30
40
50
ACFDR 2012: Accumulated Home Therapy Days
Adult
4.5 Non-transplant surgery
The following table shows the age distribution of persons reported as having
undergone selected non-transplant surgery during 2012. In view of the
incompleteness of reporting, these numbers are likely to be under-estimates.
ACFDR 2012: Non-transplant surgery during the year(a)
IV access devices
0 - 1 years 2 - 5 years
1
4
6 - 11
years
8
12 - 17
years
11
18 - 29
years
19
30 +
years
11
Total
54
Gall bladder disease
0
0
0
1
0
1
2
Gastrostomy
1
2
8
7
7
0
25
Intestinal obstruction
3
0
0
0
2
4
9
Nasal (any surgery)
0
3
3
12
9
5
32
Other
6
11
24
30
44
24
139
(a)
34
Four CF centres were excluded from analysis
5 Organ Transplants
5.1 Patients assessed for transplant in 2012
Cystic fibrosis centres reported 21 patients had been assessed for organ
transplant during 2012. Nineteen were listed as waiting for a bilateral lung
transplant, two unknown. Four of these patients were reported as having
received a transplant during 2012 and are included in the table below. Underreporting of assessments for transplant is suspected.
5.2 Transplants during 2012
With the exception of 2011 the number of transplants reported as received during
2012 was higher than in any previous year of the registry's operation (34). Details
of 32 bilateral lung transplants performed in 2012 are recorded in the following
table. The remaining 2 transplants performed in 2012 were a liver and
heart/lung/liver transplant.
ACFDR 2012: Patients receiving lung transplants in 2012
Age group:
0 - 1 years
Males
0
Females
0
Persons
0
2 - 5 years
0
0
0
6 - 11 years
0
0
0
12 - 17 years
1
1
2
18 - 29 years
9
6
15
30 years and over
7
8
15
17
15
32
All ages
5.3 Patients ever receiving a transplant
In 2012, 119 patients currently recorded in the registry had been recipients of a
transplanted organ. Bilateral lung transplants have been the most common,
comprising of 89 per cent of all transplants ever reported. Patients aged under 18
years of age at time of transplant were most likely to have a liver transplant, with
five of the seven ever reported liver transplants performed on pediatric patients.
Of the four ever recorded other transplant types, three were heart/lung/liver
transplants and one was a kidney transplant.
35
ACFDR 2012: Patients ever receiving a transplant
Age group:
0 - 1 years
Males
0
Females
0
Persons
0
2 - 5 years
0
1
1
6 – 11 years
1
1
2
12 - 17 years
2
9
11
18 - 29 years
23
34
57
30 years and over
23
25
48
All ages
49
70
119
ACFDR 2012: Transplant type, patients ever receiving a transplant
Age group:
Males
Females
Persons
46
60
106
Liver
1
6
7
Heart/lung
0
2
2
Other
2
2
4
49
70
119
Bilateral Lung
All transplants
36
6 Mortality
6.1 Deaths recorded in 2012
The number of deaths reported to the Registry in 2012 was 40. The 2012 figure
was higher than the number of deaths reported in 2011 (27) and 2010 (21). Six of
the 40 deaths reported in 2012 were of people aged less than 18 years.
ACFDR 2012: Deaths, by age and sex
Age group:
0 - 11 years
Males
1
Females
0
12 - 17 years
1
4
5
18 - 29 years
8
7
15
7
12
19
17
23
40
30 + years
All ages
Persons
1
By state and territory of residence, the highest number of deaths was reported for
people residing in New South Wales (16). Ten were reported in Victoria and
Queensland, two in South Australia and one in Western Australia and Tasmania.
The median age at death was 29 years in 2012. A generally upward trend since
1998, when the median age at death was 18 years, is shown in the chart.
ACFDR 1998-2012: Median age at death
40
35
25
20
15
10
5
0
19
98
19
99
20
00
20
01
20
02
20
03
20
04
20
05
20
06
20
07
20
08
20
09
20
10
20
11
20
12
Years of Age
30
Year
37
6.2 Causes of death
Twenty six deaths reported in 2012 were due to pulmonary causes. Both of the
other causes related to CF indicated renal failure.
ACFDR 2012: Cause of death
Males
Females
Persons
10
16
26
Gastrointestinal
0
1
1
Liver failure
0
0
0
Post-transplant
1
3
4
Other
Related to CF:
Pulmonary
1
1
2
Unrelated to CF
3
1
4
Cause not stated
2
1
3
17
23
40
All causes
38
Notes
Supplementary tables and technical notes
A range of supplementary tables, including all table formats published in annual
reports prior to 2011, is available from CFA.
ACFDR 2012: Technical Notes may be accessed online at the Cystic Fibrosis in
Australia website: http://www.cysticfibrosis.org.au/projects/dataregistry/
Technical notes cover:
 Data collection and editing
 Collection instrument
 Identification and resolution of duplicate records
 Derivations for age, lung function and nutrition data
 Registry data quality
Detailed information about data elements, coding schemes and methodology can
be provided on request to CFA.
Access to registry data
Requests for additional information from the Australian Cystic Fibrosis Data
Registry are welcome. Application should be made to Cystic Fibrosis Australia
(CFA). In accordance with a CFA policy on charging for ACFDR data services, a
fee may be charged to recover costs.
Researchers proposing to undertake analysis of unit records may be granted
access to de-identified patient records, subject to approval by Registry's medical
advisory and ethics committees, and to researcher agreement to CFA’s conditions
of use. Interested researchers are advised to contact CFA for details and to
arrange consideration of their research proposal.
All communication about additional data requirements and research access
should be addressed to:
Mr David Jack
Chief Executive Officer
Cystic Fibrosis Australia
PO Box 8007
Baulkham Hills NSW 2153
Phone:
Email:
+61 (0)2 8883 4477
[email protected]
39
Abbreviations
ACFDR
Australian Cystic Fibrosis Data Registry
BAL
Bronchioalveolar lavage
BMI
Body mass index
CF
Cystic fibrosis
CFA
Cystic Fibrosis Australia
FEV1
Forced expiratory volume (litres) in 1 second
MRSA
Methicillin-resistant Staphylococcus aureus
TPN
Total parenteral nutrition
40