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Canadian C y s t i c F i b r o s i s Patient Data Registry Report

2009

Canadian CF Patient Data Registry


2009 Report

Highlights from the Canadian CF Patient Data Registry


Approximately 3,800 individuals with CF were seen by one of the 42 specialized CF clinics across Canada More than two children per week were diagnosed with CF in 2009 Nearly 60% of all individuals with CF in Canada are adults CF patients spent over 20,000 days in hospital and attended nearly 14,000 clinic visits in 2009 CF patients underwent 746 courses of home IV therapy in 2009 The median age of survival for Canadians with CF is currently estimated to be 46.7 years of age Of the 63 patients that died in 2009, half were under 28 years old FEV1 (a measure of lung function) is improving for persons with CF; half of all 30-year olds with CF have an FEV1 of approximately 80% predicted in 2009 compared to 75% two decades ago 86% of individuals with CF must take pancreatic enzymes to digest food and absorb nutrients 35% of female adults with CF and 25% of male adults with CF are classified as underweight 44 CF patients received transplants in 2009, more than double compared to a decade ago Nearly half of all patients with CF are infected with harmful bacteria such as Staphylococcus aureus and/or Pseudomonas aeruginosa in their lungs 13% of all individuals with CF have CF-related diabetes, and more than one quarter of CF individuals 35 years of age and older have CF-related diabetes Over 1,800 different mutations in the CFTR gene have been identified, however nearly 90% of individuals with CF in Canada carry at least one copy of the most common CF-causing mutation, F508

Canadian CF Patient Data Registry


2009 Report

Table of Contents
Message & Acknowledgements ....................................................................... 4 Canadian CF Clinics ......................................................................................... 5 The Canadian CF Patient Data Registry (CPDR) ............................................ 5 CPDR Benefits People with CF ........................................................................ 6 Summary Data .................................................................................................. 7 Demographic Data ........................................................................................... 8 Genotype......................................................................................................... 11 Respiratory ..................................................................................................... 12 Nutrition ......................................................................................................... 15 Transplantation .............................................................................................. 19 Microbiology ................................................................................................... 20 CF-Related Diabetes (CFRD) ......................................................................... 22 Hospitalization and Home IV ......................................................................... 22 Survival ........................................................................................................... 23 Commonly Asked Questions........................................................................... 26

Canadian CF Patient Data Registry


2009 Report

Message & Acknowledgements


It is with great pleasure that I present the Canadian Cystic Fibrosis Patient Data Registry (CPDR) 2009 Report. We continue to capture clinical data on essentially all individuals with CF in Canada, making the registry an important national resource. Since our last publication, we have captured back-data on individuals with cystic fibrosis, therefore, there may be some discrepancies between this report and previous reports. I would like to thank Cystic Fibrosis Canada, for their ongoing support and commitment to the registry. Further acknowledgement goes to John Rudson for database design and maintenance, and to the members of the CPDR Working group. Also, I would like to thank all the staff in CF clinics across Canada for their efforts in providing data for the registry and all the individuals with CF in Canada who agree to have their data collected within the CPDR. Sincerely,

Dr. Anne Stephenson, MD, PhD Chair, Canadian CF Patient Data Registry

CPDR Working Group


Dr. Anne Stephenson (St. Michaels Hospital, Toronto) Dr. Mark Chilvers (B.C. Childrens Hospital, Vancouver) Dr. Peter Durie (The Hospital for Sick Children, Toronto) Dr. Larry Lands (Montreal Children's Hospital) Dr. Mark Montgomery (Alberta Children's Hospital, Calgary) Dr. Hans Pasterkamp (Winnipeg Childrens Hospital) Dr. Elizabeth Tullis (St. Michaels Hospital, Toronto) Dr. Ian Waters (Royal Jubilee Hospital, Victoria)

Canadian CF Patient Data Registry


2009 Report

Canadian CF Clinics
British Columbia
Victoria General Hospital Royal Jubilee Hospital, Victoria B.C. Children's Hospital, Vancouver St. Paul's Hospital, Vancouver

Qubec
Centre de sant et des services sociaux de Gatineau, Hull Montreal Children's Hospital Montreal Chest Institute Hpital Ste-Justine, Montral Htel-Dieu de Montral Centre Universitaire de Sant de l'Estrie, Sherbrooke Centre hospitalier de l'Universit Laval, Ste-Foy Institut universitaire de cardiologie et de pneumologie de Qubec, Sainte-Foy Hpital de Chicoutimi Centre hospitalier rgional de Rimouski Centre de sant et des services sociaux de Rouyn-Noranda

Alberta

Alberta Children's Hospital, Calgary Foothills Hospital, Calgary University of Alberta Hospitals, Edmonton

Saskatchewan
Royal University Hospital, Saskatoon Regina General Hospital

Manitoba

Winnipeg Children's Hospital Health Sciences Centre, Winnipeg

Ontario

Hpital rgional de Sudbury Regional Hospital Windsor Regional Hospital London Health Sciences Centre/Children's Hospital of Western Ontario Grand River Hospital, Kitchener St, Marys Hospital, Kitchener Chedoke-McMaster Hospital, Hamilton The Hospital for Sick Children, Toronto St. Michael's Hospital, Toronto Hotel-Dieu Hospital, Kingston Children's Hospital of Eastern Ontario, Ottawa Ottawa General Hospital

Nova Scotia

IWK Health Centre, Halifax QEII Health Sciences Centre, Halifax

New Brunswick
Saint John Regional Hospital

Newfoundland

Janeway Children's Health Centre, St. John's Health Sciences Centre, St. John's

The Canadian CF Patient Data Registry (CPDR)


The CPDR has been in existence since the early 1970s. The goal of the CPDR is to monitor important clinical trends in the Canadian CF population. It has played an invaluable role in helping to improve the quality and length of life of people with CF. Since the majority of CF patients attend one of 42 accredited CF clinics (pediatric and adult) within Canada, it is felt that the Canadian registry is very complete (i.e. it includes data on virtually all Canadians with CF) giving a comprehensive picture of the CF population in this country.

Canadian CF Patient Data Registry


2009 Report

CPDR Benefits People with CF


The CPDR is used both by CF clinicians and researchers to improve their knowledge of disease patterns and care of individuals with CF. CF clinicians can access the CPDR data to better understand their own clinic population and respond to emerging health care issues, including nutritional status, infectious pathogens, pulmonary treatment, and more. The data collected within the CPDR can be used for quality improvement efforts. Clinics can compare the pulmonary and nutritional outcomes of the individuals followed at their clinic to the national median value. Quality improvement initiatives can be developed and clinical outcomes can be tracked over time using the CPDR in order to show improvements. These efforts will ultimately translate into improved outcomes for all individuals with CF. In the past, the CPDR has not only played an important role in directing clinical care but also in clinical research. Investigators can use this information to formulate research questions and to guide the direction of future research. Epidemiologic research examines trends and improvements in a given population over time. Since CF patient data registries study large populations of individuals with CF over time, the CPDR is a powerful research tool. In addition, the CPDR can be used as an educational tool. The summary statistics help to graphically show important clinical outcomes over time. Incorporating these summary statistics into presentations for the public, medical and allied health care professionals, and many other groups can increase knowledge about Canadians with CF. In conclusion, CF patient data registries, and the CPDR in particular, are incredible resources for CF clinicians, researchers, as well as individuals with CF and their families. The CPDR is viewed as a model by other countries interested in developing a patient registry and has helped the Canadian CF medical and research community remain at the forefront of CF care and research. With the continued cooperation and participation of clinical personnel and Canadians with CF, along with the generous support of Cystic Fibrosis Canadas many friends and donors, it will be possible to ensure that data remain available and worthy of study in the future.

The CPDR helps the Canadian CF medical and research community remain at the forefront of CF research and care.

Canadian CF Patient Data Registry


2009 Report

Summary Data
Table 1: Summary data from CPDR, 1984 to 2009
YEAR Patients with data in reporting year, n Age, mean (yr) % over 18 yrs Age at diagnosis, mean (yr) median (mo) New diagnoses in year, n % with meconium ileus at birth Age at death, mean (yr) median (yr) Total death, n Crude mortality rate, % Median survival age, yr (5-yr window) Males Females Male, of total patients Race, % Caucasian Black Asian First Nations Other Unstated % predicted FEV1, mean 1984 2,191 12.9 29.5 2.3 9 142 9.2 20.3 20 64 2.9 25.2 27.2 22.1 53.4 98.2 0.3 0.3 0.4 0.8 0.1 47.2 1989 2,653 14.3 34.1 2.3 8 130 14.7 23.5 21 37 1.4 31.9 37.1 27.4 53.8 97.5 0.5 0.4 0.5 0.9 0.3 70.9 1994 3,051 15.9 40.3 2.6 8 148 13.6 23.7 21.5 63 2.1 32.9 33.3 32.1 53 97 0.4 0.4 0.7 1.2 0.3 71.9 1999 3,281 17.8 45.6 3 7 118 19.5 29.5 29 61 1.9 34.1 36.1 32.8 53.6 96.5 0.6 0.4 0.7 1.3 0.3 73.2 2004 3,315 19.6 51.4 3.4 7 141 21.2 27.4 23.5 56 1.7 40.3 40.8 38.3 53.6 95.2 0.7 0.5 0.7 1.7 0.2 72.7 2009 3,782 21.4 56.1 3.6 7 135 19.2 32.8 28 63 1.6 46.7 49.8 39.8 53.6 93.6 0.8 0.5 0.8 2 0.2 81.2

% with positive culture: first culture of the year (76-96), starting in 2001 all cultures: Pseudomonas aeruginosa Staphylococcus aureus Haemophilus species Burkholderia cepacia Stenotrophomonas maltophilia Aspergillus fumigatus MRSA --------------40.4 23.6 12.4 8.7 ------39.4 27.9 11.9 8.6 ------40.1 30.5 10.5 5.5 3.2 ----43.7 42.9 14.6 4.6 8.1 11.2 1.3 42.4 45.7 14.4 4.3 12.2 18.4 3.8

Canadian CF Patient Data Registry


2009 Report

Demographic Data
Figure 1: Total Number of CF Patients and New Diagnoses in the CPDR, 1989 to 2009
Number of CF Patients 4,000 3,500 3,000 Number of New Diagnoses 160 140 120 100 80 60 40 20 0 89 90 91 92 93 94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09

Number of New Diagnoses

Number of CF Patients

2,500 2,000 1,500 1,000 500 0

Year

In 2009, a total of 3,782 individuals with CF had clinical records submitted by 39 CF clinics. When an individual was seen at multiple clinics in one year, s/he was only counted once (i.e. unique individuals) for the purpose of generating this graph. The total number of individuals with CF each year represents those individuals who had data reported during the calendar year. The data collected between 2003-2007 were entered at one time by most clinics and some individuals were lost to follow up, resulting in an apparent drop in the total number of CF patients recorded in the CPDR. However, we are now up-to-date with data collection and at the time this graph was generated we had 99% of national data collected for 2009. As seen in Figure 1, the number of new CF diagnoses decreased between 2004 and 2006 but appears to have increased between 2007-2009, perhaps due to the introduction of newborn screening programs across several provinces. In 2009, 135 individuals were newly diagnosed with CF.

Canadian CF Patient Data Registry


2009 Report

Figure 2: Age Distribution of the CF Population, 2009


130 120 110 100 80 Number of CF Patients Cumulative Percent 100 90

Number of CF Patients

90 80 70 60 50 40 30 20 10 0 0 10 20 30 40 50 60 71

70
60 50 40 30 20 10 0

Cumulative Percent

Age (Years)

Figure 2 shows the age distribution of the Canadian CF population for 2009. The median age is 19 years with a range from birth to 78 years. Males account for 53.6% of individuals in the registry in 2009. The proportion of adults with CF continues to increase. In 2009, 56.1% of individuals in the registry are 18 years old or over (Figure 3).
Figure 3: Proportion of individuals with CF 18 years of age or older
60%

Percent of Adult CF Patients

50% 40% 30% 20% 10% 0% 1979 1989 1994 1999 2004 2009

Year

Canadian CF Patient Data Registry


2009 Report

Figure 4: Age at diagnosis, all patients, 2009


Number of CF Patients Cumulative Percent

1,000 900

100

Number of CF Patients

800 75 700 600 500 400 300 25 200 100 0 0 50

Cumulative Percent

Age

Since 2008, several Canadian provinces (Alberta, Ontario, Saskatchewan and British Columbia) have added CF to their newborn screening programs.

As seen in Figure 4, 50% of individuals are diagnosed by six months of age, and 74% by the age of two years. Adults continue to be diagnosed with CF, with 1.5% diagnosed after the age of 40 years. As newborn screening programs for CF are introduced in Canadian provinces, the distribution of this graph is expected to change over time.

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Canadian CF Patient Data Registry


2009 Report

Genotype
CF is caused by mutations in a single gene located on chromosome 7, termed the cystic fibrosis transmembrane regulator (CFTR) gene. The CFTR gene codes for a protein called the transmembrane conductance regulator (CFTR) which functions as a chloride channel and is involved in many cellular functions. To date, over 1800 different mutations in the CFTR gene have been identified. The most common mutation worldwide is a three basepair deletion resulting in the deletion of the phenylalanine residue at amino acid position 508, commonly referred to as F508.
Figure 5: Genotype (based on N=3,499)

CF is caused by mutations in the cystic fibrosis transmebrane regulator (CFTR) gene.

12.9%

48.8% 38.3%

Of those individuals with genetic information recorded within the registry, 48.8% carry two F508 mutations (Figure 5) and 88.9% carry at least one F508 mutation (Table 2).

Homozygous deltaF508

Heterozygous deltaF508

Other

Table 2: Frequency of CF mutations (top five) Mutation DeltaF508 621+1G->T G542X G551D A455E n 2825 119 101 76 58 % 88.9 3.7 3.2 2.4 1.8

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Canadian CF Patient Data Registry


2009 Report

Respiratory
Figure 6: Median percent predicted FEV1 vs. age, 1989 and 2009
1989 100 2009

95

Percent Predicted

90

85

80

75

70 6 8 10 12 14 16 18 20 22 24 26 28 30

Age (Years)

Median FEV1 percent predicted has improved over the last two decades. Although this increase is seen across all ages from six to 30 years, it is larger in younger ages compared to older ages. Median FEV1 in 2009 at 30 years of age is 79.4% predicted compared to 74.9% in 1989 (Figure 6).

FEV1 is the maximal amount of air you can forcefully exhale in one second.

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Canadian CF Patient Data Registry


2009 Report

Figure 7: Respiratory Severity of CF Children and Adults (FEV 1 percent predicted), 2009

Normal FEV1 > 90% 60

Mild FEV1 70-89%

Moderate FEV1 40-69%

Severe FEV1 < 40%

50

Percent Predicted

40

30

20

10

Children 6-17

Adults

Lung function is measured from age 6 years and onwards. For children ages 6-17, the majority of individuals with CF (51.5%) have an FEV1 greater than 90% predicted; while for adults, the majority (41%) have lung function classified as moderate severity (Figure 7).
Figure 8: Median FEV1 percent predicted for CF patients 6 to 17 years of age by CF clinic, 2009
110 100 90 National median, 90.9

Percent Predicted

80 70

60
50 40 30 20 10 0

CF Clinics

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Canadian CF Patient Data Registry


2009 Report

In order for a CF clinic to be included in Figure 8, it had to report lung function data on 10 or more CF patients. Twentytwo CF clinics are included in the graph. The number of observations per clinic used to calculate the median FEV1 ranges from 12 to 175. The national median FEV1 is 90.9% predicted with a range from 63.5% to 100.2% (Figure 8).
Figure 9: Median FEV1 percent predicted for CF patients 18 years of age or more by CF clinic, 2009
90 80 70

National median, 64.3

60

Percent Predicted

50 40 30 20

10
0

CF Clinics

Figure 9 shows the median FEV1 percent predicted by CF clinic for those 18 years of age and older. In order to be included in this graph, a CF clinic had to report lung function data on at least 10 CF patients. A total of 24 CF clinics are shown on the graph. The number of observations per clinic used to calculate the median FEV1 ranges from 10 to 342. The national median FEV1 is 64.3% predicted with a range from 45.8% to 89.6%.

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Canadian CF Patient Data Registry


2009 Report

Nutrition
In 2009, 86% of individuals with CF were taking supplemental pancreatic enzymes (pancreatic insufficient), whereas 14% were considered pancreatic sufficient (Figure 10). For those individuals 40 years of age or older, 65.9% are pancreatic insufficient and 34.1% are pancreatic sufficient. This is likely a reflection of the fact that older adults with CF are more likely to have milder mutations that are associated with pancreatic sufficiency (Figure 11).
Figure 10: Pancreatic sufficiency in CF Patients

Many people with CF have difficulty digesting food and absorbing nutrients.

14%

Figure 11: Pancreatic status by age group

age < 18

age 40

Percentage of patients

86%

100 90 80 70 60 50 40 30 20 10 0 Pancreatic Insufficient Pancreatic Sufficient

Pancreatic Insufficient

Pancreatic Sufficient

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Canadian CF Patient Data Registry


2009 Report

Figure 12: Median BMI Percentile for CF patients 2-17 years by CF clinic, 2009
80

70

Median BMI Percentile

60 50 40 30 20 10 0

National median 48.3

CF Clinics

A total of 25 pediatric CF clinics are included in Figure 12. In order to be included in this graph, a CF clinic had to report data on at least 10 CF patients. In CF patients 2 to 17 years of age, the national median Body Mass Index percentile, as per the Center for Diseases Control (BMI CDC), is 48.3%. The median percentiles range from 29.4 to 70.2%. BMI percentile is not calculated for those under the age of two years. The proportion of individuals age 2-17 whose height is less than the 5th percentile is 6.9% and 6.5% with weight less than the 5th percentile.

BMI percentile indicates the relative position of a child's BMI compared to typical values for other children of the same age.

Weight Status Category Underweight Healthy weight Overweight

Percentile Range

Less than the 5 percentile 5 percentile to less than th the 85 percentile 85 to less than the 95 percentile
th th th

th

Obese

Equal to or greater than the th 95 percentile

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Canadian CF Patient Data Registry


2009 Report

Figure 13: Median BMI for CF patients 18 years of age and older by CF clinic, 2009
National median, 21.9

24

20

16

Median BMI

12

CF Clinics

Figure 13 shows the median BMI by clinic for those CF patients 18 years of age and older. There are 22 CF clinics included in this graph. In order to be included in this graph, a CF clinic had to report data on at least 10 CF patients. The national median BMI is 21.9 kg/m2. The median BMI ranges from 20.1 to 23.1 kg/m2.

Body mass index (BMI) is used to estimate a healthy body weight based on an adult's height.

BMI

Categories: Underweight = <20.0 kg/m2 Adequate weight = 20.0-25.9 kg/m2 Well-nourished = 26-29.9 kg/m2 Obese = >30 kg/m2

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Canadian CF Patient Data Registry


2009 Report

Figure 14: BMI classification for CF patients, by sex, 2009


70 60 50 Males Females

Percent

40 30 20 10 0 Underweight (< 20) Adequate weight (20-25) Well-nourished (26-29) Obese ( 30)

BMI categories

Figure 14 shows the breakdown of BMI categories (see previous page for categories) for adult males and females. A larger proportion of females are considered underweight compared to males (BMI < 20 kg/m2). Often young men who are muscular and fit can have a BMI between 26-29 kg/m2 due to high muscle mass. Overall, 3.3% of the CF adult population has a BMI in the obese category.
Figure 15: Percentage with BMI < 22 kg/m for females and < 23 kg/m for males for CF patients 18 years of age and older by CF clinic, 2009
90 80 70 60 National median, 61.8
2 2

Percent

50 40 30 20 10 0

CF Clinics

The national average percentage of CF patients with BMI < 22 kg/m2 for females 2 and < 23 kg/m for males in patients 18 years of age and older is 61.8% with a range from 46.7% to 84.2%. In order to be included in Figure 15, a CF clinic had to report data on at least 10 CF patients. There are 22 CF clinics included in this graph.

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Canadian CF Patient Data Registry


2009 Report

Transplantation
Figure 16: Number of patients transplanted per year, 1991 to 2009
CPDR 60 Transplant Centre Incentive Grant Applications

Number of Transplants

50

40

30

20

10

0 91 92 93 94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09

Year

Figure 16 shows the number of transplants carried out per year as reported in the CPDR and as reported by the transplant centres on their incentive grant applications. Although the numbers provided represent primarily lung transplants, individuals who received other combinations (e.g. lung-liver, liver, heart-lung, etc.) are also included in the total. Based on the transplant statistics reported in the transplant centres incentive grant applications, the number of CF patients transplanted in 2009 was 44, compared to 39 recorded in the registry. We are working hard with transplant centres to ensure we capture accurate data on transplants within the registry. We hope that in future reports the number transplanted will be the same as that reported on the clinic incentive grant applications.

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Canadian CF Patient Data Registry


2009 Report

Microbiology
Figure 17: Prevalence of species of bacteria cultured in airways in CF patients (all ages), 2009
50 45 40

Percent Prevalence

35 30 25

20
15 10 5 0

Overall, Pseudomonas aeruginosa and Staphylococcus aureus are the most common pulmonary pathogens in Canadians with CF (Figure 17). The prevalence of Aspergillus fumigatus species, Stenotrophomonas maltophilia, and MRSA have increased between 2008 and 2009 (Figure 18). The largest increase was seen in Aspergillus species. The increased prevalence may be partly due to increase surveillance for these organisms. Conversely, the prevalence of Burkholderia cepacia complex has decreased over the years, to 4.3% in 2009. It is also noteworthy that the prevalence of Pseudomonas aeruginosa has decreased from 45.8% in 2008 to 42.4% in 2009 which may be in part due to more aggressive early eradication strategies in the paediatric population. The prevalence of MRSA in 2009 was 3.8%. MRSA was not captured in the CPDR prior to 2003.

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Canadian CF Patient Data Registry


2009 Report

Figure 18: Prevalence of Microbiology, 2008 vs. 2009


2008 2009

Percentage prevalence

50 45 40 35 30 25 20 15 10 5 0

A. fumigatus

B. cepacia complex

MRSA

P. aeruginosa

S. aureus

S. maltophilia

Figure 19: Age Specific Prevalence of Respiratory Infections in CF Patients, 2009


70 60

P. aeruginosa S. aureus H. influenza B. cepacia complex S. maltophilia

Percent Prevalence

50 40

30
20 10 0 <1 2 to 5 6 to 10 11 to 17 18 to 24 25 to 34 35 to 44 45+

Aspergillus
MRSA

Age Categories

As expected, Staphyloccocus aureus is more common in the pediatric population whereas Pseudomonas aeruginosa is more common in the adult CF population. The prevalence of Stenotrophomonas maltophilia increases until the teen years but then appears to stabilize (Figure 19). Burkholderia cepacia complex is more commonly seen in older individuals with CF which represents the fact that new acquisition of B. cepacia complex in general has decreased substantially over the years making its prevalence in children low. However, those individuals who previously acquired B. cepacia complex are aging making the prevalence of this organism higher in older individuals.

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Canadian CF Patient Data Registry


2009 Report

CF-Related Diabetes (CFRD)


Figure 20: Percentage of patients with CFRD by age category, 2009
30

Percentage of Patients with CFRD

25 20 15 10 5 0

< 6 yrs

6 to 10 yrs

11 to 17 yrs

18 to 24 yrs

25 to 34 yrs

35 yrs

Age Categories

Overall, CFRD was reported in 13.1% of individuals with CF in 2009. In those individuals 35 years of age and older, the prevalence of CFRD is 25.9% in 2009. The prevalence of CFRD increases with age (Figure 20). Of those with CFRD, 51% are female.

Hospitalization and Home IV


Table 3: Number of Hospital days and Home IV courses, 2009

In 2009, 1,525 hospitalizations were recorded in the CPDR. The most common recorded reason for admission was a pulmonary exacerbation. In 2009, 746 courses of home IV therapy were recorded within the registry.

Hospital days Hospitalizations # clinic visits Home IV courses Home IV days

Total # 20,359 1,525 13,965 746 10,308

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Canadian CF Patient Data Registry


2009 Report

Survival
Figure 21: Age at death, 2005 to 2009
Number of Deaths 110 100 90 80 70 60 50 40 30 20 10 0 0 to 5 6 to 10 11 to 20 21 to 30 31 to 40 41 to 50 Over 50 0% 40% 60% Cumulative Percent 100%

Cumulative Percent

Number of Deaths

80%

20%

Age at Death (Years)

Since there are relatively few deaths per year, the sum of all deaths from 2005 to 2009 were included in Figure 21. There were 63 deaths recorded in the registry for 2009. The median age at death for 2009 was 28 years of age. The most common cause of death recorded in the CPDR was pulmonary-related.
Figure 22: Median age of survival for a moving 5-year window by sex
Both Male Female

Median Expected Survival Age

50 45 40 35 30 25 20 15 74 76 78 80 82 84

86

88

90

92

94

96

98

00

02

04

06

08

Last Calendar Year in Window

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Canadian CF Patient Data Registry


2009 Report

Figure 23: Canadian male CF survival by birth cohort 1975 to 2009


100%

90% 1975-79 80% 1980-84 1985-89 70% 1990-94 1995-99

Survival Rate

60%

2000-04
2005-09

50%

40% 1 3 5 7 9 11 13 15 17 19 21 23 25 27 29 31 33 35

Age (years) Figure 24: Canadian female CF survival by birth cohort 1975 to 2009
100%

90%

1975-80 1981-84 1985-89

80%

Survival Rate

70%

1990-94 1995-99

60%

2000-04 2005-09

50%

40% 1 3 5 7 9 11 13 15 17 19 21 23 25 27 29 31 33 35

Age (years)

The median age of survival for Canadians with CF is currently estimated to be

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Canadian CF Patient Data Registry


2009 Report

46.7 years of age, which is quite remarkable (Figure 22). Figure 23 and Figure 24 show the survival of males and females by birth cohort. Some key points to highlight from these graphs are: 1. At any given age, the survival is higher for those born more recently suggesting that, over time, there have been advances in CF care that are keeping people healthier. 2. The slope of each line is different. As the birth cohorts become more recent, the slope of the line is more gradual. This means that the survival for someone born in 1975 is not the same for someone born in 2005. 3. The point at which the line crosses the 50% survival mark is the median age of survival. This can only be calculated for those females born between 1975-1980 since this is the only line that cross the 50% survival on the y-axis.

The majority of persons with CF in Canada may now expect to live into their 40s, and even beyond.

Since our last report, we have continued our efforts to ensure we are capturing deaths accurately within the registry. These efforts have yielded some death dates that were previously not recorded in the registry. Because of this, we anticipated that the median survival age would not continue its upward trend but instead, that it would show some adjustment in this most recent analysis. We will continue to work with all the Canadian CF clinics as well as transplant centres to ensure accurate capture of CPDR data including information on deaths and transplants. It is not possible to know for certain the reason for the improvement seen in survival for Canadians with CF and in truth, there are likely multiple factors. Certainly it is a statistic that would not be possible without the hard work and dedication of CF families, Cystic Fibrosis Canada volunteers, partners, donors, researchers and CF clinic teams, and everyone can be very proud of this accomplishment.

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Canadian CF Patient Data Registry


2009 Report

Commonly Asked Questions


Why is the median age at death so different than the median age of survival? Median age at death is calculated simply by taking all those individuals who died in a given year, placing them in ascending order, and determining which age is the middle number. The median age at death is calculated using only those individuals who have died. In other words, of those who died, died before the median age of death and died later than the median age of death. This calculation doesnt tell you anything about the individuals who have survived! You need to know the ages of those still living to get information on median survival.

Is median age of survival the same as life expectancy? No! The life expectancy is the expected average length of life based on current age-specific mortality rates. Life expectancy at birth in Canada for example is 77.3 years. This means that a baby born now will, on average, be expected to live 77.3 years. Median age of survival is the estimated duration of time until 50% of a given population dies. Half of the population are still alive and half have died. The age at which this occurs is the median survival age.

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Canadian CF Patient Data Registry


2009 Report
What is cystic fibrosis? Cystic fibrosis (CF) is the most common fatal genetic disease affecting Canadian children and young adults. CF is a multi-system disease that affects mainly the lungs, and the digestive system. In the lungs, where the effects of the disease are most devastating, CF causes increasingly severe respiratory problems. Mucus and protein also build up in the digestive tract making it difficult to digest and absorb nutrients from food. As improved therapies have helped to address the malnutrition issues in CF, virtually all CF deaths are due to lung disease. What is Cystic Fibrosis Canada? Cystic Fibrosis Canada (formerly known as the Canadian Cystic Fibrosis Foundation) is a national health charity established in 1960, with volunteers in more than 50 chapters across Canada. Cystic Fibrosis Canadas mission is to help people with cystic fibrosis by: funding research towards the goal of a cure or control for cystic fibrosis; supporting high quality CF care; promoting public awareness of cystic fibrosis; and raising and allocating funds for these purposes.

Cystic Fibrosis Canada is one of the largest funders of cystic fibrosis research in the world.

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Canada continues to be a world leader in cystic fibrosis research and care. Thanks to the progress we have made, half of all Canadians with cystic fibrosis are expected to live into their 40s and beyond. While this is remarkable progress, many young Canadians with CF die far too soon, and much work remains to be done. Support from all Canadians will enable us to expand our investment in important programs for people with cystic fibrosis.

Cystic Fibrosis Canada 2221 Yonge St., Suite 601, Toronto, Ontario M4S 2B4 Local: 416-485-9149 Toll free: 1-800-378-2233 www.cysticfibrosis.ca info@cysticfibrosis.ca
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